Acute respiratory distress syndrome (ARDS), also known as respiratory distress syndrome(RDS) or adult respiratory distress syndrome (in

contrast with IRDS) is a serious reaction to various forms of injuries to the lung. ARDS is a severe lung disease caused by a variety of direct and indirect issues. It is characterized by inflammation of the lung parenchyma leading to impaired gas exchange with concomitant systemic release of inflammatory mediators causing inflammation, hypoxemia and frequently resulting in multiple organ failure. This condition is often fatal, usually requiringmechanical ventilation and admission to an intensive care unit. A less severe form is calledacute lung injury (ALI). ARDS formerly most commonly signified adult respiratory distress syndrome to differentiate it from infant respiratory distress syndrome in premature infants. However, as this type of pulmonary edema also occurs in children, ARDS has gradually shifted to mean acute rather than adult. The differences with the typical infant syndrome remain.

Definition
[edit]Historical

background
[1][2]

Acute respiratory distress syndrome was first described in 1967 by Ashbaugh et al.

Initially there was

no definition, resulting in controversy over incidence and mortality. In 1988 an expanded definition was proposed which quantified physiologic respiratory impairment. In 1994 a new definition was recommended by the American-European Consensus Conference Committee.[1][3] It had two advantages: 1 it recognizes that severity of pulmonary injury varies, 2 it is simple to use.[4] ARDS was defined as the ratio of arterial partial oxygen tension (PaO2) as fraction of inspired oxygen (FiO2) below 200 mmHg in the presence of bilateral alveolar infiltrates on the chest x-ray. These infiltrates may appear similar to those of left ventricular failure, but the cardiac silhouette appears normal in ARDS. Also, the pulmonary capillary wedge pressure is normal (less than 18 mmHg) in ARDS, but raised in left ventricular failure. A PaO2/FiO2 ratio less than 300 mmHg with bilateral infiltrates indicates acute lung injury (ALI). Although formally considered different from ARDS, ALI is usually just a precursor to ARDS. [edit]Consensus

after 1967 and 1994

[1][3]

ARDS is characterized by:  Acute onset

 

Bilateral infiltrates on chest radiograph sparing costophrenic angles Pulmonary artery wedge pressure < 18 mmHg (obtained by pulmonary artery catheterization), if this information is available; if unavailable, then lack of clinical evidence of left ventricular failure suffices

 

if PaO2:FiO2 < 300 mmHg (40 kPa) acute lung injury (ALI) is considered to be present if PaO2:FiO2 < 200 mmHg (26.7 kPa) acute respiratory distress syndrome (ARDS) is considered to be present

To summarize and simplify, ARDS is an acute (rapid onset) syndrome (collection of symptoms) that affects the lungs widely and results in a severe oxygenation defect, but is not heart failure [edit]Patient

presentation and diagnosis

ARDS can occur within 24 to 48 hours of an injury or attack of acute illness. In such a case the patient usually presents with shortness of breath, tachypnea, and symptoms related to the underlying cause, i.e. shock. Long term illnesses can also trigger it, e.g. malaria. ARDS may then occur sometime after the onset of a particularly acute case of the infection. See xray of malarial ARDS. An arterial blood gas analysis and chest X-ray allow formal diagnosis by the aforementioned criteria. Although severe hypoxemia is generally included, the appropriate threshold defining abnormal PaO2 has never been systematically studied. Note though, that a severe oxygenation defect is not synonymous with ventilatory support. Any PaO2 below 100 (generally saturation less than 100%) on a supplemental oxygen fraction of 50% meets criteria for ARDS. This can easily be achieved by high flow oxygen supplementation without ventilatory support. Any cardiogenic cause of pulmonary edema should be excluded. This can be done by placing a pulmonary artery catheter for measuring the pulmonary artery wedge pressure. However, this is not necessary and is now rarely done as abundant evidence has emerged demonstrating that the use of pulmonary artery catheters does not lead to improved patient outcomes in critical illness including ARDS. Plain Chest X-rays are sufficient to document bilateral alveolar infiltrates in the majority of cases. While CT scanning leads to more accurate images of the pulmonary parenchyma in ARDS, it has little utility in the clinical management of patients with ARDS, and remains largely a research tool. [edit]Pathophysiology

A pathohistological image of ARDS.

ARDS is a clinical syndrome associated with a variety of pathological findings. These include pneumonia, eosinophilic pneumonia, cryptogenic organizing pneumonia, acute fibrinous organizing pneumonia, and diffuse alveolar damage (DAD). Of these, the pathology most commonly associated with ARDS is DAD. DAD is characterized by a diffuse inflammation of lung parenchyma. The triggering insult to the parenchyma usually results in an initial release of cytokines and other inflammatory mediators, secreted by local epithelial and endothelial cells. Neutrophils and some T-lymphocytes quickly migrate into the inflamed lung parynchema and contribute in the amplification of the phenomenon. Typical histological presentation involves diffuse alveolar damage and hyaline membrane formation in alveolar walls. Although the triggering mechanisms are not completely understood, recent research has examined the role of inflammation and mechanical stress. [edit]Inflammation Inflammation alone, as in sepsis, causes endothelial dysfunction, fluid extravasation from the capillaries and impaired drainage of fluid from the lungs. Dysfunction of type II pulmonary epithelial cells may also be present, with a concomitant reduction in surfactant production. Elevated inspired oxygen concentration often becomes necessary at this stage, and they may facilitate a 'respiratory burst' in immune cells. In a secondary phase, endothelial dysfunction causes cells and inflammatory exudate to enter the alveoli. This pulmonary edema increases the thickness of the alveolo-capillary space, increasing the distance the oxygen must diffuse to reach blood. This impairs gas exchange leading to hypoxia, increases the work of breathing, eventually induces fibrosis of the airspace. Moreover, edema and decreased surfactant production by type II pneumocytes may cause whole alveoli to collapse, or to completely flood. This loss of aeration contributes further to the right-to-left

shunt in ARDS. As the alveoli contain progressively less gas, more blood flows through them without being oxygenated resulting in massive intrapulmonary shunting. Collapsed alveoli (and small bronchi) do not allow gas exchange. It is not uncommon to see patients with a PaO2 of 60 mmHg (8.0 kPa) despite mechanical ventilation with 100% inspired oxygen. The loss of aeration may follow different patterns according to the nature of the underlying disease, and other factors. In pneumonia-induced ARDS, for example, large, more commonly causes relatively compact areas of alveolar infiltrates. These are usually distributed to the lowerlobes, in their posterior segments, and they roughly correspond to the initial infected area. In sepsis or trauma-induced ARDS, infiltrates are usually more patchy and diffuse. The posterior and basal segments are always more affected, but the distribution is even less homogeneous. Loss of aeration also causes important changes in lung mechanical properties. These alterations are fundamental in the process of inflammation amplification and progression to ARDS in mechanically ventilated patients. [edit]Mechanical

stress

Mechanical ventilation is an essential part of the treatment of ARDS. As loss of aeration (and the underlying disease) progress, the work of breathing (WOB) eventually grows to a level incompatible with life. Thus, mechanical ventilation is initiated to relieve respiratory muscles of their work, and to protect the usually obtunded patient's airways. However, mechanical ventilation may constitute a risk factor for the development, or the worsening, of ARDS.[1] Aside from the infectious complications arising from invasive ventilation with tracheal intubation, positivepressure ventilation directly alters lung mechanics during ARDS. The result is higher mortality, i.e. through baro-trauma, when these techniques are used.[1] In 1998, Amato et al. published a paper showing substantial improvement in the outcome of patients
[1][5] This result was confirmed in a 2000 study ventilated with lower tidal volumes (Vt) (6 mLkg ). -1

sponsored by the NIH.[6] Although both these studies were widely criticized for several reasons, and although the authors were not the first to experiment lower-volume ventilation, they shed new light on the relationship between mechanical ventilation and ARDS. One opinion is that the forces applied to the lung by the ventilator may work as a lever to induce further damage to lung parenchyma. It appears that shear stress at the interface between collapsed and aerated units may result in the breakdown of aerated units, which inflate asymmetrically due to the 'stickiness' of surrounding flooded alveoli. The fewer such interfaces around an alveolus, the lesser the stress.

Indeed, even relatively low stress forces may induce signal transduction systems at the cellular level, thus inducing the release of inflammatory mediators. This form of stress is thought to be applied by the transpulmonary pressure (gradient) (Pl) generated by the ventilator or, better, its cyclical variations. The better outcome obtained in patients ventilated with lower Vt may be interpreted as a beneficial effect of the lower Pl. Transpulmonary pressure, is an indirect function of the Vt setting on the ventilator, and only trial patients with plateau pressures (a surrogate for the actual Pl) were less than 32 cmH2O (3.1 kPa) had improved survival. The way Pl is applied on alveolar surface determines the shear stress to which lung units are exposed. ARDS is characterized by a usually inhomogeneous reduction of the airspace, and thus by a tendency towards higher Pl at the same Vt, and towards higher stress on lessdiseased units. The inhomogeneity of alveoli at different stages of disease is further increased by the gravitational gradient to which they are exposed, and the different perfusion pressures at which blood flows through them. Finally, abdominal pressure exerts an additional pressure on inferoposterior lung segments, favoring compression and collapse of those units. The different mechanical properties of alveoli in ARDS may be interpreted as having varying time constants (the product of alveolarcompliance resistance). A long time constant indicates an alveolus which opens slowly during tidal inflation, as a consequence of contrasting pressure around it, or altered water-air interface inside it (loss of surfactant, flooding). Slow alveoli are said to be 'kept open' using positive end-expiratory pressure, a feature of modern ventilators which maintains a positive airway pressure throughout the whole respiratory cycle. A higher mean pressure cycle-wide slows the collapse of diseased units, but it has to be weighed against the corresponding elevation in Pl/plateau pressure. Newer ventilatory approaches attempt to maximize mean airway pressure for its ability to 'recruit' collapsed lung units while minimizing the shear stress caused by frequent openings and closings of aerated units. The prone position also reduces the inhomogeneity in alveolar time constants induced by gravity and edema. If clinically appropriate, mobilization of the ventilated patient can assist in achieving the same goal. [edit]Progression If the underlying disease or injurious factor is not removed, the amount of inflammatory mediators released by the lungs in ARDS may result in a systemic inflammatory response syndrome (or sepsis if there is lung infection).[1] The evolution towards shock and/or multiple organ failure follows paths analogous to the pathophysiology of sepsis.

This adds up to the impaired oxygenation which is the central problem of ARDS, as well as to respiratory acidosis, which is often caused by ventilation techniques such as permissive hypercapnia which attempt to limit ventilator-induced lung injury in ARDS. The result is a critical illness in which the 'endothelial disease' of severe sepsis/SIRS is worsened by the pulmonary dysfunction, which further impairs oxygen delivery. [edit]Treatment [edit]General Acute respiratory distress syndrome is usually treated with mechanical ventilation in the Intensive Care Unit. Ventilation is usually delivered through oro-tracheal intubation, or tracheostomy whenever prolonged ventilation (2 weeks) is deemed inevitable. The possibilities of non-invasive ventilation are limited to the very early period of the disease or, better, to prevention in individuals at risk for the development of the disease (atypical pneumonias, pulmonary contusion, major surgery patients). Treatment of the underlying cause is imperative, as it tends to maintain the ARDS picture. Appropriate antibiotic therapy must be administered as soon as microbiological culture results are available. Empirical therapy may be appropriate if local microbiological surveillance is efficient. More than 60% ARDS patients experience a (nosocomial) pulmonary infection either before or after the onset of lung injury. The origin of infection, when surgically treatable, must be operated on. When sepsis is diagnosed, appropriate local protocols should be enacted. Commonly used supportive therapy includes particular techniques of mechanical ventilation and pharmacological agents whose effectiveness with respect to the outcome has not yet been proven. It is now debated whether mechanical ventilation is to be considered mere supportive therapy or actual treatment, since it may substantially affect survival. [edit]Mechanical

ventilation

Further information: Pressure Regulated Volume Control The overall goal is to maintain acceptable gas exchange and to minimize adverse effects in its application. Three parameters are used: PEEP (positive end-expiratory pressure, to maintain maximal recruitment of alveolar units), mean airway pressure (to promote recruitment and predictor of hemodynamic effects) and plateau pressure (best predictor of alveolar overdistention). [7] Conventional therapy aimed at tidal volumes (Vt) of 12-15 ml/kg. Recent studies have shown that high tidal volumes can overstretch alveoli resulting in volutrauma (secondary lung injury). The ARDS Clinical

Network, or ARDSNet, completed a landmark trial that showed improvedmortality when ventilated with a tidal volume of 6 ml/kg compared to the traditional 12 ml/kg. Low tidal volumes (Vt) may cause hypercapniaand atelectasis[1] due to their inherent tendency to increase dead space. Low tidal volume ventilation was the primary independent variable associated with reduced mortality in the NIH-sponsored ARDSnet trial of tidal volume in ARDS. Plateau pressure less than 30 cm H2O was a secondary goal, and subsequent analyses of the data from the ARDSnet trial (as well as other experimental data) demonstrate that there appears to be NO safe upper limit to plateau pressure; that is, regardless of plateau pressure, patients fare better with low tidal volumes (see Hager et al., American Journal of Respiratory and Critical Care Medicine, 2005). [edit]APRV

(Airway Pressure Release Ventilation) and ARDS / ALI

No particular ventilator mode is known to improve mortality in ARDS. The landmark ARDSNet trial used a volume controlled mode and showed decrease mortality with smaller volumes. However, other modes of ventilation have not been directly compared to volume controlled ventilation. Some practitioners favor airway pressure release ventilation (APRV). Advantages to APRV ventilation include: decreased airway pressures, decreased minute ventilation, decreased dead-space ventilation, promotion of spontaneous breathing, almost 24 hour a day alveolar recruitment, decreased use of sedation, near elimination of neuromuscular blockade, optimized arterial blood gas results, mechanical restoration of FRC (functional residual capacity), a positive effect on cardiac output (due to the negative inflection from the elevated baseline with each spontaneous breath), increased organ and tissue perfusion, potential for increased urine output secondary to increased renal perfusion. Counterarguments would state that taken to the limit of a paralyzed patient, APRV is essentially a huge tidal volume, low PEEP strategy. While high PEEP is not now felt to improve outcomes in ARDS, high tidal volumes are strongly implicated in worse outcomes. Paradoxically, arterial blood gas results are known to worsen with the ARDSNet low tidal volume strategy, but survival improves, throwing such secondary outcomes usefulness into question. Similarly, in a patient with isolated severe ARDS, other issues such as organ perfusion and cardiac output, or use of sedation are of secondary importance. The patient will live if the lungs heal, or die if the lungs deteriorate. Less severe lung injury in a medically complex patient may entail different trade offs in medical decision making. [edit]Positive

end-expiratory pressure

Positive end-expiratory pressure (PEEP) is used in mechanically-ventilated patients with ARDS to improve oxygenation. In ARDS, three populations of alveoli can be distinguished. There are normal alveoli which are always inflated and engaging in gas exchange, flooded alveoli which can never, under any ventilatory regime, be used for gas enchange, and atelectatic or partially flooded alveoli that can be "recruited" to participate in gas exchange under certain ventilatory regimes. The recruitable aveoli

represent a continuous population, some of which can be recruited with minimal PEEP, and others which can only be recruited with high levels of PEEP. An additional complication is that some or perhaps most alveoli can only be opened with higher airway pressures than are needed to keep them open. Hence the justification for maneuvers where PEEP is increased to very high levels for seconds to minutes before dropping the PEEP to a lower level. Finally, PEEP can be harmful. High PEEP necessarily increases mean airway pressure and alveolar pressure. This in turn can damage normal alveoli by overdistension resulting in DAD. The 'best PEEP' used to be defined as 'some' cmH2O above the lower inflection point (LIP) in the sigmoidal pressure-volume relationship curve of the lung. Recent research has shown that the LIPpoint pressure is no better than any pressure above it, as recruitment of collapsed alveoli, and more importantly the overdistension of aerated units, occur throughout the whole inflation. Despite the awkwardness of most procedures used to trace the pressure-volume curve, it is still used by some to define the minimum PEEP to be applied to their patients. Some of the newest ventilators have the ability to automatically plot a pressure-volume curve. The possibility of having an 'instantaneous' tracing trigger might produce renewed interest in this analysis. PEEP may also be set empirically. Some authors suggest performing a 'recruiting maneuver' (i.e., a short time at a very high continuous positive airway pressure, such as 50 cmH2O (4.9 kPa), to recruit, or open, collapsed units with a high distending pressure) before restoring previous ventilation. The final PEEP level should be the one just before the drop in PaO2 (or peripheral blood oxygen saturation) during a stepdown trial. Intrinsic PEEP (iPEEP), or auto-PEEP, first described by John Marini of St. Paul Regions Hospital, is a potentially unrecognized contributor to PEEP in patients. When ventilating at high frequencies, its contribution can be substantial, particularly in patients with obstructive lung disease. iPEEP has been measured in very few formal studies on ventilation in ARDS patients, and its contribution is largely unknown. Its measurement is recommended in the treatment of ARDS patients, especially when using high-frequency (oscillatory/jet) ventilation. A compromise between the beneficial and adverse effects of PEEP is inevitable. [edit]Prone

position

Distribution of lung infiltrates in acute respiratory distress syndrome is non-uniform. Repositioning into the prone position (face down) might improve oxygenation by relieving atelectasis and improving perfusion. However, although the hypoxemia is overcome there seems to be no effect on overall survival.[1][8] [edit]Fluid

management

Several studies have shown that pulmonary function and outcome are better in patients that lost weight or pulmonary wedge pressure was lowered by diuresis or fluid restriction. [edit]Corticosteroids A Meduri et al. study has found significant improvement in ARDS using modest doses of corticosteroids. The initial regimen consists ofmethylprednisolone 2 mg/kg daily. After 3-5 days a response must be apparent. In 1-2 weeks the dose can be tapered to methylprednisolone 0.5-1.0 mg daily. Patients with ARDS do not benefit from high-dose corticosteroids.[1][9] This was a study involving a small number of patients in one center. A recent NIH-sponsored multicenter ARDSnet LAZARUS study of corticosteroids for ARDS demonstrated that they are not efficacious in ARDS. [edit]Nitric
[1]

oxide

Inhaled nitric oxide (NO) potentially acts as selective pulmonary vasodilator. Rapid binding to hemoglobin prevents systemic effects. It should increase perfusion of better ventilated areas. There are no large studies demonstrating positive results. Therefore its use must be considered individually. Almitrine bismesylate stimulates chemoreceptors in carotic and aortic bodies. It has been used to potentiate the effect of NO, presumably by potentiating hypoxia-induced pulmonary vasoconstriction. In case of ARDS it is not known whether this combination is useful.[1] [edit]Surfactant

therapy

To date no prospective controlled clinical trial has shown a significant mortality benefit of exogenous surfactant in ARDS.[1] [edit]Complications Since ARDS is an extremely serious condition which requires invasive forms of therapy it is not without risk. Complications to be considered are:[1]  Pulmonary: barotrauma (volutrauma), pulmonary embolism (PE), pulmonary fibrosis, ventilatorassociated pneumonia (VAP).     Gastrointestinal: hemorrhage (ulcer), dysmotility, pneumoperitoneum, bacterial translocation. Cardiac: arrhythmias, myocardial dysfunction. Renal: acute renal failure (ARF), positive fluid balance. Mechanical: vascular injury, pneumothorax (by placing pulmonary artery catheter), tracheal injury/stenosis (result of intubation and/or irritation by endotracheal tube.  Nutritional: malnutrition (catabolic state), electrolyte deficiency. [edit]Epidemiology

The annual incidence of ARDS is 1.513.5 people per 100,000 in the general population.[citation needed] Its incidence in the intensive care unit(ICU), mechanically ventilated population is much higher. BrunBuisson et al. (2004) reported a prevalence of acute lung injury (ALI) (see below) of 16.1% percent in ventilated patients admitted for more than 4 hours. More than half these patients may develop ARDS. Mechanical ventilation, sepsis, pneumonia, shock, aspiration, trauma (especially pulmonary contusion), major surgery, massive transfusions,smoke inhalation, drug reaction or overdose, fat emboli and reperfusion pulmonary edema after lung transplantation or pulmonary embolectomy may all trigger ARDS. Pneumonia and sepsis are the most common triggers, and pneumonia is present in up to 60% of patients. Pneumonia and sepsis may be either causes or complications of ARDS. Elevated abdominal pressure of any cause is also probably a risk factor for the development of ARDS, particularly during mechanical ventilation. The mortality rate varies from 30% to 85%.
[citation needed]

Usually, randomized controlled trials in the

literature show lower death rates, both in control and treatment patients. This is thought to be due to stricter enrollment criteria. Observational studies generally report 50%60% mortality.[citation needed]

Adult Respiratory Distress Syndrome

Definition
Adult respiratory distress syndrome (ARDS), also called acute respiratory distress syndrome, is a type of lung (pulmonary) failure that may result from any disease that causes large amounts of fluid to collect in the lungs. ARDS is not itself a specific disease, but a syndrome, a group of symptoms and signs that make up one of the most important forms of lung or respiratory failure. It can develop quite suddenly in persons whose lungs have been perfectly normal. Very often ARDS is a true medical emergency. The basic fault is a breakdown of the barrier, or membrane, that normally keeps fluid from leaking out of the small blood vessels of the lung into the breathing sacs (the alveoli).

Description
Another name for ARDS is shock lung. Its formal name is misleading, because children, as well as adults, may be affected. In the lungs the smallest blood vessels, or capillaries, make contact with the alveoli, tiny air sacs at the tips of the smallest breathing tubes (the bronchi). This is the all-important site where oxygen passes from air that is inhaled to the blood, which carries it to all parts of the body. Any form of lung injury that damages this point of contact, called the alveolo-capillary junction, will allow blood and tissue fluid to leak into the alveoli, eventually filling them so that air cannot enter. The result is the type of breathing distress called ARDS. ARDS is one of the major causes of excess fluid in the lungs, the other being heart failure. Along with fluid there is a marked increase in inflamed cells in the lungs. There also is debris left over from damaged lung cells, and fibrin, a semisolid material derived from blood in the tissues. Typically these materials join together with large molecules in the blood (proteins), to form hyaline membranes. (These membranes are very prominent in premature infants who develop respiratory distress syndrome; it is often called hyaline membrane disease.) If ARDS is very severe or lasts a long time, the lungs do not heal, but rather become scarred, a process known as fibrosis. The lack of a normal amount of oxygen

causes the blood vessels of the lung to become narrower, and in time they, too, may become scarred and filled with clotted blood. The lungs as a whole become very "stiff," and it becomes much harder for the patient to breathe.

Key terms
Alveoli The tiny air sacs at the ends of the breathing tubes of the lung where oxygen normally is taken up by the capillaries to enter the circulation. Aspiration The process in which solid food, liquids, or secretions that normally are swallowed are, instead, breathed into the lungs. Capillaries The smallest arteries which, in the lung, are located next to the alveoli so that they can pick up oxygen from inhaled air. Face mask The simplest way of delivering a high level of oxygen to patients with ARDS or other lowoxygen conditions. Steroids A class of drugs resembling normal body substances that often help control inflammation in the body tissues. Ventilator A mechanical device that can take over the work of breathing for a patient whose lungs are injured or are starting to heal.

Causes and symptoms

A very wide range of diseases or toxic substances, including some drugs, can cause ARDS. They include: y y y y y y y y y y y Breathing in (aspiration) of the stomach contents when regurgitated, or salt water or fresh water from nearly drowning. Inhaling smoke, as in a fire; toxic materials in the air, such as ammonia or hydrocarbons; or too much oxygen, which itself can injure the lungs. Infection by a virus or bacterium, or sepsis, a widespread infection that gets into the blood. Massive trauma, with severe injury to any part of the body. Shock with persistently low blood pressure may not in itself cause ARDS, but it can be an important factor. A blood clotting disorder called disseminated intravascular coagulation, in which blood clots form in vessels throughout the body, including the lungs. A large amount of fat entering the circulation and traveling to the lungs, where it lodges in small blood vessels, injuring the cells lining the vessel walls. An overdose of a narcotic drug, a sedative, or, rarely, aspirin. Inflammation of the pancreas (pancreatitis), when blood proteins, called enzymes, pass to the lungs and injure lung cells. Severe burn injury. Injury of the brain, or bleeding into the brain, from any cause may be a factor in ARDS for reasons that are not clear. Convulsions also may cause some cases.

Usually ARDS develops within one to two days of the original illness or injury. The person begins to take rapid but shallow breaths. The doctor who listens to the patient's chest with a stethoscope may hear "crackling" orwheezing sounds. The low blood oxygen content may cause the skin to appear mottled or

even blue. As fluid continues to fill the breathing sacs, the patient may have great trouble breathing, take very rapid breaths, and gasp for air.

Diagnosis
A simple test using a device applied to the ear will show whether the blood is carrying too little oxygen, and this can be confirmed by analyzing blood taken from an artery. The chest x ray may be normal in the early stages, but, in a short time, fluid will be seen where it does not belong. The two lungs are about equally affected. A heart of normal size indicates that the problem actually is ARDS and not heart failure. Another way a physician can distinguish between these two possibilities is to place a catheter into a vein and advance it into the main artery of the lung. In this way, the pressure within the pulmonary capillaries can be measured. Pressure within the pulmonary capillaries is elevated in heart failure, but normal in ARDS.

Treatment
The three main goals in treating patients with ARDS are: y To treat whatever injury or disease has caused ARDS. Examples are: to treat septic infection with the proper antibiotics, and to reduce the level of oxygen therapy if ARDS has resulted from a toxic level of oxygen. To control the process in the lungs that allows fluid to leak out of the blood vessels. At present there is no certain way to achieve this. Certain steroid hormones have been tried because they can combat inflammation, but the actual results have been disappointing. To make sure the patient gets enough oxygen until the lung injury has had time to heal. If oxygen delivered by a face mask is not enough, the patient is placed on a ventilator, which takes over breathing, and, through a tube placed in the nose or mouth (or an incision in the windpipe), forces oxygen into the lungs. This treatment must be closely supervised, and the pressure adjusted so that too much oxygen is not delivered.

Patients with ARDS should be cared for in an intensive care unit, where experienced staff and all needed equipment are available. Enough fluid must be provided, by vein if necessary, to prevent dehydration. Also, the patient's nutritional state must be maintained, again by vein, if oral intake is not sufficient.

Prognosis
If the patient's lung injury does not soon begin to heal, the lack of sufficient oxygen can injure other organs, such as the kidneys. There always is a risk that bacterial pneumonia will develop at some point. Without prompt treatment, as many as 90% of patients with ARDS can be expected to die. With modern treatment, however, about half of all patients will survive. Those who do live usually recover completely, with little or no long-term breathing difficulty. Lung scarring is a risk after a long period on a ventilator, but it may improve in the months after the patient is taken off ventilation. Whether a particular patient will recover depends to a great extent on whether the primary disease that caused ARDS to develop in the first place can be effectively treated.

Prevention
The only way to prevent ARDS is to avoid those diseases and harmful conditions that damage the lung. For instance, the danger of aspirating stomach contents into the lungs can be avoided by making sure a patient does not eat shortly before receiving general anesthesia. If a patient needs oxygen therapy, as low a level as possible should be given. Any form of lung infection, or infection anywhere in the body that gets into the blood, must be treated promptly to avoid the lung injury that causes ARDS.

Resources
Books Smolley, Lawrence A., and Debra F. Bryse. Breathe RightNow: A Comprehensive Guide to Understanding and Treating the Most Common Breathing Disorders. New York: W. W. Norton & Co., 1998.

Organizations
National Heart, Lung and Blood Institute. P.O. Box 30105, Bethesda, MD 20824-0105. (301) 2511222.http://www.nhlbi.nih.gov. National Respiratory Distress Syndrome Foundation. P.O. Box 723, Montgomeryville, PA 18936.
Gale Encyclopedia of Medicine. Copyright 2008 The Gale Group, Inc. All rights reserved.

syndrome /syndrome/ (sindr m) a set of symptoms occurring together; the sum of signs of any
morbid state; a symptom complex. See also entries under disease.

Aarskog syndrome , Aarskog-Scott syndrome a hereditary X-linked condition characterized by ocular hypertelorism, anteverted nostrils, broad upper lip, peculiar scrotal shawl above the penis, and small hands. acquired immune deficiency syndrome , acquired immunodeficiency syndrome an epidemic, transmissible retroviral disease caused by infection with the human immunodeficiency virus, manifested in severe cases as profound depression of cell-mediated immunity, and affecting certain recognized risk groups. Diagnosis is by the presence of a disease indicative of a defect in cell-mediated immunity (e.g., life-threatening opportunistic infection) in the absence of any known causes of underlying immunodeficiency or of any other host defense defects reported to be associated with that disease (e.g., iatrogenic immunosuppression). acute coronary syndrome a classification encompassing clinical presentations ranging from unstable anginathrough non, sometimes also including Q wave infarction. acute radiation syndrome a syndrome caused by exposure to a whole body dose of over 1 gray of ionizing radiation; symptoms, whose severity and time of onset depend on the size of the dose, include erythema, nausea and vomiting, fatigue, diarrhea, petechiae, bleeding from the mucous membranes, hematologic changes, gastrointestinal hemorrhage, epilation, hypotension, tachycardia, and dehydration; death may occur within hours or weeks of exposure. acute respiratory distress syndrome (ARDS) fulminant pulmonary interstitial and alveolar edema, which usually develops within a few days after the initiating trauma, thought to result from alveolar injury that has led to increased capillary permeability. acute retinal necrosis syndrome necrotizing retinitis with uveitis and other retinal pathology, severe loss of vision, and often retinal detachment; of viral etiology.

Adams-Stokes syndrome episodic cardiac arrest and syncope due to failure of normal and escape pacemakers, with or without ventricular fibrillation; the principal manifestation of severe heart attack. addisonian syndrome the complex of symptoms resulting from adrenocortical insufficiency; see Addison's disease, under disease. Adie's syndrome tonic pupil associated with absence or diminution of certain tendon reflexes. adrenogenital syndrome a group of syndromes in which inappropriate virilism or feminization results from disorders of adrenal function that also affect gonadal steroidogenesis. adult respiratory distress syndrome (ARDS) acute respiratory distress s. AEC syndrome Hay-Wells s. afferent loop syndrome chronic partial obstruction of the proximal loop (duodenum and jejunum) after gastrojejunostomy, resulting in duodenal distention, pain, and nausea following ingestion of food. Ahumada-del Castillo syndrome galactorrhea-amenorrhea syndrome with low gonadotropin secretion. akinetic-rigid syndrome muscular rigidity with varying degrees of slowness of movement; seen in parkinsonism and disorders of the basal ganglia. Alagille syndrome inherited neonatal jaundice, cholestasis with peripheral pulmonic stenosis, unusual facies, and ocular, vertebral, and nervous system abnormalities, due to paucity or absence of intrahepatic bile ducts. Albright's syndrome , Albright-McCune-Sternberg syndrome polyostotic fibrous dysplasia, patchy dermal pigmentation, and endocrine dysfunction. Aldrich's syndrome Wiskott-Aldrich s. Allgrove's syndrome inherited glucocorticoid deficiency with achalasia and alacrima. Alport's syndrome a hereditary disorder marked by progressive nerve deafness, progressive pyelonephritis or glomerulonephritis, and occasionally ocular defects. Alstrm syndrome a hereditary syndrome of retinitis pigmentosa with nystagmus and early loss of central vision, deafness, obesity, and diabetes mellitus. amnestic syndrome a mental disorder characterized by impairment of memory occurring in a normal state of consciousness; the most common cause is thiamine deficiency associated with alcohol abuse. amniotic band syndrome see under sequence. Angelman's syndrome happy puppet s. angular gyrus syndrome a syndrome resulting from an infarction or other lesion of the angular gyrus on the dominant side, often characterized by alexia or agraphia. ankyloblepharonectodermal dysplasiaclefting syndrome Hay-Wells s.

anorexia-cachexia syndrome a systemic response to cancer occurring as a result of a poorly understood relationship between anorexia and cachexia, manifested by malnutrition, weight loss, muscular weakness, acidosis, and toxemia. anterior cord syndrome anterior spinal artery s. anterior interosseous syndrome a complex of symptoms caused by a lesion of the anterior interosseous nerve, usually resulting from a fracture or laceration. anterior spinal artery syndrome localized injury to the anterior portion of the spinal cord, characterized by complete paralysis and hypalgesia and hypesthesia to the level of the lesion, but with relative preservation of posterior column sensations of touch, position, and vibration. Apert's syndrome acrocephalosyndactyly, type I; an autosomal dominant disorder characterized by acrocephaly and syndactyly, often with other skeletal deformities and mental retardation. Asherman's syndrome persistent amenorrhea and secondary sterility due to intrauterine adhesions and synechiae, usually as a result of uterine curettage. Asperger's syndrome a pervasive developmental disorder resembling autistic disorder, being characterized by severe impairment of social interactions and by restricted interests and behaviors; however, patients are not delayed in development of language, cognitive function, and self-help skills. Barrett's syndrome peptic ulcer of the lower esophagus, often with stricture, due to the presence of columnar-lined epithelium, which may contain functional mucous cells, parietal cells, or chief cells, in the esophagus instead of normal squamous cell epithelium. Bartter syndrome a hereditary form of hyperaldosteronism secondary to hypertrophy and hyperplasia of the juxtaglomerular cells, with normal blood pressure and hypokalemic alkalosis in the absence of edema, increased concentration of renin, angiotensin II, and bradykinin; usually occurring in children. basal cell nevus syndrome an autosomal dominant syndrome characterized by the development in early life of numerous basal cell carcinomas, in association with abnormalities of the skin, bone, nervous system, eyes, and reproductive tract. Bassen-Kornzweig syndrome abetalipoproteinemia. battered-child syndrome multiple traumatic lesions of the bones and soft tissues of children, often accompanied by subdural hematomas, willfully inflicted by an adult. Beckwith-Wiedemann syndrome an inherited disorder characterized by exomphalos, macroglossia, and gigantism, often associated with visceromegaly, adrenocortical cytomegaly, and dysplasia of the renal medulla. Behet's syndrome severe uveitis and retinal vasculitis, optic atrophy, and aphtha-like lesions of the mouth and genitalia, often with other signs and symptoms suggesting a diffuse vasculitis; it most often affects young males. Bernard-Soulier syndrome a hereditary coagulation disorder marked by mild thrombocytopenia, giant and morphologically abnormal platelets, hemorrhagic tendency, prolonged bleeding time, and purpura.

Bing-Neel syndrome the central nervous system manifestations of Waldenstrm's macroglobulinemia, possibly including encephalopathy, hemorrhage, stroke, convulsions, delirium, and coma. Birt-Hogg-Dub syndrome an inherited disorder of proliferation of ectodermal and mesodermal components of the pilar system, occurring as multiple trichodiscomas, acrochordons, and fibrofolliculomas on the head, chest, back, and upper limbs. Blackfan-Diamond syndrome congenital hypoplastic anemia. blue toe syndrome skin necrosis and ischemic gangrene manifest as a blue color of the toes, resulting from arterial occlusion, usually caused by emboli, thrombi, or injury. Boerhaave's syndrome spontaneous rupture of the esophagus. Brjeson's syndrome , Brjeson-Forssman-Lehmann syndrome a hereditary syndrome, transmitted as an X-linked recessive trait, characterized by severe mental retardation, epilepsy, hypogonadism, hypometabolism, marked obesity, swelling of the subcutaneous tissues of the face, and large ears. bowel bypass syndrome a syndrome of dermatosis and arthritis occurring some time after jejunoileal bypass, probably caused by immune reponse to bacterial overgrowth in the bypassed bowel. Bradbury-Eggleston syndrome a progressive syndrome of postural hypotension without tachycardia but with visual disturbances, impotence, hypohidrosis, lowered metabolic rate, dizziness, syncope, and slow pulse; due to impaired peripheral vasoconstriction. bradycardia-tachycardia syndrome , brady-tachy syndrome a clinical manifestation of the sick sinus syndrome characterized by alternating periods of bradycardia and tachycardia. Brown-Squard syndrome ipsilateral paralysis and loss of discriminatory and joint sensation, and contralateral loss of pain and temperature sensation; due to damage to one half of the spinal cord. Brown-Vialetto-van Laere syndrome an inherited syndrome of progressive bulbar palsy with any of several cranial nerve disorders. Budd-Chiari syndrome symptomatic obstruction or occlusion of the hepatic veins, causing hepatomegaly, abdominal pain and tenderness, intractable ascites, mild jaundice, and eventually portal hypertension and liver failure. Caffey's syndrome , Caffey-Silverman syndrome infantile cortical hyperostosis. Canada-Cronkhite syndrome Cronkhite-Canada s. capillary leak syndrome extravasation of plasma fluid and proteins into the extravascular space, resulting in sometimes fatal hypotension and reduced organ perfusion; an adverse effect of interleukin-2 therapy. carcinoid syndrome a symptom complex associated with carcinoid tumors, marked by attacks of cyanotic flushing of the skin and watery diarrhea, bronchoconstrictive attacks, sudden drops in blood pressure, edema, and ascites. Symptoms are caused by tumor secretion of serotonin, prostaglandins, and other biologically active substances. carotid sinus syndrome syncope sometimes associated with convulsions due to overactivity of the carotid sinus reflex when pressure is applied to one or both carotid sinuses.

carpal tunnel syndrome pain and burning or tingling paresthesias in the fingers and hand, sometimes extending to the elbow, due to compression of the median nerve in the carpal tunnel.

Median nerve entrapped in carpal tunnel in carpal tunnel syndrome.

Carpenter's syndrome acrocephalopolysyndactyly, type II; an autosomal recessive disorder characterized by acrocephaly, polysyndactyly, brachydactyly, mild obesity, mental retardation, hypogonadism, and other anomalies. central cord syndrome injury to the central part of the cervical spinal cord resulting in disproportionately more weakness or paralysis in the upper limbs than in the lower; pathological change is caused by hemorrhage or edema. cerebrocostomandibular syndrome an inherited syndrome of severe micrognathia and costovertebral abnormalities, with palatal defects, prenatal and postnatal growth deficiencies, and mental retardation. cerebrohepatorenal syndrome a hereditary disorder, transmitted as an autosomal recessive trait, characterized by craniofacial abnormalities, hypotonia, hepatomegaly, polycystic kidneys, jaundice, and death in early infancy. cervical rib syndrome thoracic outlet syndrome caused by a cervical rib. Cestan's syndrome , Cestan-Chenais syndrome an association of contralateral hemiplegia, contralateral hemianesthesia, ipsilateral lateropulsion and hemiasynergia, Horner's syndrome, and ipsilateral laryngoplegia, due to scattered lesions of the pyramid, sensory tract, inferior cerebellar peduncle, nucleus ambiguus, and oculopupillary center. Charcot's syndrome 1. amyotrophic lateral sclerosis. 2. intermittent claudication. Charcot-Marie syndrome Charcot-Marie-Tooth disease.

CHARGE syndrome see under association. Chdiak-Higashi syndrome a lethal, progressive, autosomal recessive, systemic disorder associated with oculocutaneous albinism, massive leukocyte inclusions (giant lysosomes), histiocytic infiltration of multiple body organs, development of pancytopenia, hepatosplenomegaly, recurrent or persistent bacterial infections, and a possible predisposition to development of malignant lymphoma. Chinese restaurant syndrome transient arterial dilatation due to ingestion of monosodium glutamate, which is sometimes used liberally in seasoning Chinese food, marked by throbbing head, lightheadedness, tightness of the jaw, neck, and shoulders, and backache. Chotzen's syndrome acrocephalosyndactyly, type III; an autosomal dominant disorder characterized by acrocephaly and syndactyly in which the latter is mild and by hypertelorism, ptosis, and sometimes mental retardation. Christ-Siemens-Touraine syndrome anhidrotic ectodermal dysplasia. chronic fatigue syndrome persistent debilitating fatigue of recent onset, with greatly reduced physical activity and some combination of muscle weakness, sore throat, mild fever, tender lymph nodes, headaches, and depression, not attributable to any other known causes; it is of controversial etiology. Churg-Strauss syndrome allergic granulomatous angiitis; a systemic form of necrotizing vasculitis in which there is prominent lung involvement. chylomicronemia syndrome familial hyperchylomicronemia. Coffin-Lowry syndrome an X-linked syndrome of incapability of speech, severe mental deficiency, and muscle, ligament, and skeletal abnormalities. Coffin-Siris syndrome hypoplasia of the fifth fingers and toenails associated with growth and mental deficiencies, coarse facies, mild microcephaly, hypotonia, lax joints, and mild hirsutism. compartmental syndrome a condition in which increased tissue pressure in a confined anatomic space causes decreased blood flow leading to ischemia and dysfunction of contained myoneural elements, marked by pain, muscle weakness, sensory loss, and palpable tenseness in the involved compartment; ischemia can lead to necrosis resulting in permanent impairment of function. congenital rubella syndrome transplacental infection of the fetus with rubella, usually in the first trimester of pregnancy, as a consequence of maternal infection, resulting in various developmental anomalies in the newborn infant. Conn's syndrome primary aldosteronism. cri du chat syndrome a hereditary congenital syndrome characterized by hypertelorism, microcephaly, severe mental deficiency, and a plaintive catlike cry, due to deletion of the short arm of chromosome 5. Crigler-Najjar syndrome an autosomal recessive form of nonhemolytic jaundice due to absence of the hepatic enzyme glucuronide transferase, marked by excessive amounts of unconjugated bilirubin in the blood, kernicterus, and severe central nervous system disorders.

syndrome of crocodile tears spontaneous lacrimation occurring parallel with the normal salivation of eating, and associated with facial paralysis; it seems to be due to straying of regenerating nerve fibers, some of those destined for the salivary glands going to the lacrimal glands. Cronkhite-Canada syndrome familial polyposis of the gastrointestinal tract associated with ectodermal defects such as alopecia and onychodystrophy. Crow-Fukase syndrome POEMS s. crush syndrome the edema, oliguria, and other symptoms of renal failure that follow crushing of a part, especially a large muscle mass; see lower nephron nephrosis, under nephrosis. Cruveilhier-Baumgarten syndrome cirrhosis with portal hypertension associated with congenital patency of the umbilical and paraumbilical veins. Cushing's syndrome a condition, more commonly seen in females, due to hyperadrenocorticism resulting from neoplasms of the adrenal cortex or anterior lobe of the pituitary; or to prolonged excessive intake of glucocorticoids for therapeutic purposes (iatrogenic Cushing's s. or Cushing's s. medicamentosus). The symptoms may include adiposity of the face, neck, and trunk, kyphosis caused by softening of the spine, amenorrhea, hypertrichosis (in females), impotence (in males), dusky complexion with purple markings, hypertension, polycythemia, pain in the abdomen and back, and muscular weakness. Da Costa syndrome neurocirculatory asthenia. Dandy-Walker syndrome congenital hydrocephalus due to obstruction of the foramina of Magendie and Luschka. Dejean's syndrome orbital floor s. de Lange's syndrome a congenital syndrome of mental retardation, short stature (Amsterdam dwarf), flat spadelike hands, and other anomalies. dialysis dysequilibrium syndrome symptoms such as headache, nausea, muscle cramps, nervous irritability, drowsiness, and convulsions during or after overly rapid hemodialysis or peritoneal dialysis, resulting from an osmotic shift of water into the brain. disconnection syndrome any neurologic disorder caused by an interruption in impulse transmission along cerebral fiber pathways. Down syndrome mongoloid features, short phalanges, widened space between the first and second toes and fingers, and moderate to severe mental retardation; associated with a chromosomal abnormality, usually trisomy of chromosome 21. Drash syndrome an inherited syndrome of Wilms' tumor with glomerulopathy and male pseudohermaphroditism. Dubin-Johnson syndrome hereditary chronic nonhemolytic jaundice thought to be due to defective excretion of conjugated bilirubin and certain other organic anions by the liver; a brown, coarsely granular pigment in hepatic cells is pathognomonic.

dumping syndrome nausea, weakness, sweating, palpitation, syncope, often a sensation of warmth, and sometimes diarrhea, occurring after ingestion of food in patients who have undergone partial gastrectomy. dyscontrol syndrome a pattern of episodic abnormal and often violent and uncontrollable social behavior with little or no provocation; it may have an organic cause or be associated with abuse of a psychoactive substance. dysmaturity syndrome postmaturity s. Eaton-Lambert syndrome a myasthenia-like syndrome in which the weakness usually affects the limbs and ocular and bulbar muscles are spared; often associated with oat-cell carcinoma of the lung. EEC syndrome ectrodactylyectodermal dysplasiaclefting s.; an inherited congenital syndrome involving both ectodermal and mesodermal tissues, characterized by ectodermal dysplasia with hypopigmentation of skin and hair, and other hair, nail, tooth, lip, and palate abnormalities. Ehlers-Danlos syndrome a group of inherited disorders of connective tissue, varying in clinical and biochemical evidence, in mode of inheritance, and in severity from mild to lethal; major manifestations include hyperextensible skin and joints, easy bruisability, friability of tissues, bleeding, poor wound healing, subcutaneous nodules, and cardiovascular, orthopedic, intestinal, and ocular defects. Eisenmenger's syndrome ventricular septal defect with pulmonary hypertension and cyanosis due to right-to-left (reversed) shunt of blood. Sometimes defined as pulmonary hypertension (pulmonary vascular disease) and cyanosis with the shunt being at the atrial, ventricular, or great vessel area. EMG syndrome Beckwith-Wiedemann s. Escobar syndrome multiple pterygium s. excited skin syndrome nonspecific cutaneous hyperirritability of the back, sometimes occurring when multiple positive reactions are elicited in patch test screening of a battery of substances. exomphalos-macroglossia-gigantism syndrome Beckwith-Wiedemann s. extrapyramidal syndrome any of a group of clinical disorders considered to be due to malfunction in the extrapyramidal system and marked by abnormal involuntary movements; included are parkinsonism, athetosis, and chorea. Faber's syndrome hypochromic anemia. Fanconi syndrome 1. a rare hereditary disorder, transmitted as an autosomal recessive trait, characterized by pancytopenia, hypoplasia of the bone marrow, and patchy brown discoloration of the skin due to the deposition of melanin, and associated with multiple congenital anomalies of the musculoskeletal and genitourinary systems. 2. a general term for a group of diseases marked by dysfunction of the proximal renal tubules, with generalized hyperaminoaciduria, renal glycosuria, hyperphosphaturia, and bicarbonate and water loss; the most common cause is cystinosis, but it is also associated with other genetic diseases and occurs in idiopathic and acquired forms.

Farber syndrome , Farber-Uzman syndrome Farber's disease. Felty's syndrome a syndrome of splenomegaly with chronic rheumatoid arthritis and leukopenia; there are usually pigmented spots on the skin of the lower extremities, and sometimes there is other evidence of hypersplenism such as anemia or thrombocytopenia. fetal alcohol syndrome a syndrome of altered prenatal growth and morphogenesis, occurring in infants born of women who were chronically alcoholic during pregnancy; it includes maxillary hypoplasia, prominence of the forehead and mandible, short palpebral fissures, microophthalmia, epicanthal folds, severe growth retardation, mental retardation, and microcephaly. fetal hydantoin syndrome poor growth and development with craniofacial and skeletal abnormalities, produced by prenatal exposure to hydantoin analogues, including phenytoin. floppy infant syndrome abnormal posture in an infant suspended prone, the limbs and head hanging down; due to any of numerous conditions, particularly perinatal injury to the brain or spinal cord, spinal muscular atrophy, and various genetic disorders. Foix-Alajouanine syndrome a fatal necrotizing myelopathy characterized by necrosis of the gray matter of the spinal cord, thickening of the walls of the spinal vessels, and abnormal spinal fluid. Franceschetti syndrome the complete form of mandibulofacial dysostosis. galactorrhea-amenorrhea syndrome amenorrhea and galactorrhea, sometimes associated with increased levels of prolactin. Ganser syndrome the giving of approximate answers to questions, commonly associated with amnesia, disorientation, perceptual disturbances, fugue, and conversion symptoms. Garcin's syndrome unilateral paralysis of most or all of the cranial nerves due to a tumor at the base of the skull or in the nasopharynx. Gardner's syndrome familial polyposis of the colon associated with osseous and soft tissue tumors. gay bowel syndrome an assortment of sexually transmitted bowel and rectal diseases affecting homosexual males and others who engage in anal intercourse, caused by a wide variety of infectious agents. general adaptation syndrome the total of all nonspecific reactions of the body to prolonged systemic stress, comprising alarm, resistance, and exhaustion. Gerstmann-Strussler syndrome , Gerstmann-Strussler-Scheinker syndrome a group of rare prion diseases of autosomal dominant inheritance, having the common characteristics of cognitive and motor disturbances, ending in death, and the presence of multicentric amyloid plaques in the brain. Gianotti-Crosti syndrome monomorphous, usually nonpruritic, dusky or coppery red, flat-topped, firm papules forming a symmetrical eruption on the face, buttocks, and limbs, including the palms and soles, with malaise and low-grade fever; seen in young children and associated with viral infection. Gilles de la Tourette's syndrome a childhood-onset syndrome comprising both multiple motor and one or more vocal tics, often associated with obsessions, compulsions, hyperactivity, distractibility, and impulsivity; it may diminish or even remit in adolescence or adulthood.

Goodpasture's syndrome glomerulonephritis with pulmonary hemorrhage and circulating antibodies against basement membranes, usually seen in young men and with a course of rapidly progressing renal failure, with hemoptysis, pulmonary infiltrates, and dyspnea. Gradenigo's syndrome sixth nerve palsy and unilateral headache in suppurative disease of the middle ear, due to involvement of the abducens and trigeminal nerves by direct spread of the infection. gray syndrome a potentially fatal condition seen in neonates, particularly premature infants, due to a reaction to chloramphenicol, characterized by an ashen gray cyanosis, listlessness, weakness, and hypotension. Guillain-Barr syndrome acute idiopathic polyneuritis. Gunn's syndrome unilateral ptosis of the eyelid, with movements of the affected eyelid associated with those of the jaw. Hamman-Rich syndrome the acute form of idiopathic pulmonary fibrosis. Hand-Schller-Christian syndrome see under disease. hantavirus pulmonary syndrome a sometimes fatal febrile illness caused by a hantavirus, characterized by variable respiratory symptoms followed by acute respiratory distress, sometimes progressing to respiratory failure. happy puppet syndrome an inherited syndrome of jerky puppetlike movements, frequent laughter, mental and motor retardation, peculiar open-mouthed facies, and seizures. Harada syndrome Vogt-Koyanagi-Harada s. Hay-Wells syndrome an inherited syndrome of ectodermal dysplasia, cleft lip and palate, and adhesions of the margins of the eyelids, accompanied by tooth, skin, and hair abnormalities. HELLP syndrome h emolysis, e levated l iver enzymes, and l ow p latelet count occurring in association with pre-eclampsia. Helweg-Larsen's syndrome an inherited syndrome of anhidrosis present from birth and labyrinthitis occurring late in life. hemolytic uremic syndrome a form of thrombotic microangiopathy with renal failure, hemolytic anemia, and severe thrombocytopenia and purpura. Herrmann's syndrome an inherited syndrome initially characterized by photomyogenic seizures and progressive deafness, with later development of diabetes mellitus, nephropathy, and mental deterioration. HHH syndrome hyperornithinemia-hyperammonemia-homocitrullinuria s. Hinman syndrome a psychogenic disorder seen in children, imitating a neurogenic bladder, consisting ofdetrusor-sphincter dyssynergia without evidence of neural lesion. Horner syndrome , Horner-Bernard syndrome sinking in of the eyeball, ptosis of the upper lid, slight elevation of the lower lid, miosis, narrowing of the palpebral fissure, and anhidrosis and flushing of the affected side of the face; due to a brain stem lesion on the ipsilateral side that interrupts descending sympathetic nerves.

Hughes-Stovin syndrome thrombosis of the pulmonary arteries and peripheral veins, characterized by headache, fever, cough, papilledema, and hemoptysis. Hurler's syndrome an inherited mucopolysaccharidosis due to deficiency of the enzyme -l-iduronidase, characterized by gargoyle-like facies, dwarfism, severe somatic and skeletal changes, severe mental retardation, cloudy corneas, deafness, cardiovascular defects, hepatosplenomegaly, joint contractures, and death in childhood. Hutchinson-Gilford syndrome progeria. hypereosinophilic syndrome any of several diseases characterized by a massive increase in the number ofeosinophils in the blood and bone marrow, with infiltration of other organs. Symptoms vary from mild to the often fatal outcome of eosinophilic leukemia. hyperkinetic syndrome former name for attention-deficit. hyperornithinemia-hyperammonemia-homocitrullinuria syndrome an inherited disorder characterized by elevated levels of ornithine, postprandial hyperammonemia and homocitrullinuria, and aversion to protein ingestion; believed to result from a defect in the transport of ornithine into the mitochondria, which disturbs the cycle of ureagenesis. hyperventilation syndrome a complex of symptoms that accompany hypocapnia caused by hyperventilation, including palpitations, shortness of breath, lightheadedness or giddiness, profuse perspiration, tingling sensations in the fingertips, face, or toes, and vasomotor collapse and loss of consciousness if prolonged. hypoplastic left heart syndrome congenital hypoplasia or atresia of the left ventricle, aortic or mitral valve, and ascending aorta, with respiratory distress, cardiac failure, and death in infancy. impingement syndrome progressive pathologic changes resulting from the impingement of the acromion, coracoacromial ligament, coracoid process, or acromioclavicular joint on the rotator cuff. syndrome of inappropriate antidiuretic hormone (SIADH) persistent hyponatremia, inappropriately elevated urine osmolality, caused by release of vasopressin (antidiuretic hormone) without discernible stimulus. irritable bowel syndrome , irritable colon syndrome a chronic noninflammatory disease with a psychophysiologic basis, characterized by abdominal pain, diarrhea or constipation or both, and no detectable pathologic change. Isaacs' syndrome , Isaacs-Mertens syndrome progressive muscle stiffness and spasms, with continuous muscle fiber activity similar to that seen with neuromyotonia. Jacod's syndrome chronic arthritis after rheumatic fever, with fibrous changes in the joint capsules leading to deformities that may resemble rheumatoid arthritis but lack bone erosion. Jarcho-Levin syndrome an inherited disorder of multiple vertebral defects, short thorax, rib abnormalities, camptodactyly, syndactyly, and sometimes urogenital abnormalities, usually fatal in infancy. Joubert's syndrome inherited, usually fatal, partial to complete agenesis of the cerebellar vermis, with hypotonia, episodic hyperpnea, mental retardation, and abnormal eye movements.

Kartagener's syndrome a hereditary syndrome consisting of dextrocardia, bronchiectasis, and sinusitis. Kimmelstiel-Wilson syndrome intercapillary glomerulosclerosis in which the lesions are nodular. King syndrome a form of malignant hyperthermia accompanied by characteristic physical abnormalities. Klinefelter's syndrome smallness of testes with fibrosis and hyalinization of seminiferous tubules, variable degrees of masculinization, azoospermia, and infertility, and increased urinary gonadotropins. It is associated typically with an XXY chromosome complement although variants include XXYY, XXXY, XXXXY, and various mosaic patterns. Klippel-Feil syndrome shortness of the neck due to reduction in the number of cervical vertebrae or the fusion of multiple hemivertebrae into one osseous mass, with limitation of neck motion and low hairline. Korsakoff's syndrome a syndrome of anterograde and retrograde amnesia with confabulation associated with alcoholic or nonalcoholic polyneuritis, currently used synonymously with the term amnestic syndrome or, more narrowly, to refer to the amnestic component of the Wernicke-Korsakoff syndrome. Kugelberg-Welander syndrome an inherited juvenile form of muscular atrophy due to lesions on the anterior horns of the spinal cord, beginning with the proximal muscles of the lower limbs and pelvic girdle and progressing to the distal muscles. LAMB syndrome a syndrome of familial myomas with cutaneous, cardiac, and endocrine involvement, manifested as l entigines, a trial m yxoma, and b lue nevi. Landau-Kleffner syndrome an epileptic syndrome of childhood with partial or generalized seizures, psychomotor abnormalities, and aphasia progressing to mutism. Launois' syndrome pituitary gigantism. Laurence-Moon syndrome an autosomal recessive disorder characterized by mental retardation, pigmentary retinopathy, hypogonadism, and spastic paraplegia. lazy leukocyte syndrome a syndrome in children, marked by recurrent low-grade infections with a defect in neutrophil chemotaxis and deficient random mobility of neutrophils. Lemieux-Neemeh syndrome an inherited syndrome of Charcot-Marie-Tooth disease with progressive deafness. Leriche syndrome lower limb fatigue on exercising, lack of femoral pulse, impotence, and often pale, cold lower limbs, usually seen in males due to obstruction of the terminal aorta. Lesch-Nyhan syndrome an X-linked disorder of purine metabolism with physical and mental retardation, compulsive self-mutilation of fingers and lips by biting, choreoathetosis, spastic cerebral palsy, and impaired renal function, and by extremely excessive purine synthesis and consequently hyperuricemia and excessive urinary secretion of uric acid. Li-Fraumeni syndrome a familial syndrome of early breast carcinoma associated with soft tissue sarcomas and other tumors. locked-in syndrome quadriplegia and mutism with intact consciousness and preservation of some eye movements; usually due to a vascular lesion of the anterior pons.

long QT syndrome prolongation of the QT interval combined with torsades de pointes and manifest in several forms, either acquired or congenital, the latter with or without deafness; it may lead to serious arrhythmia and sudden death. Lowe syndrome , Lowe-Terrey-MacLachlan syndrome oculocerebrorenal s. Lown-Ganong-Levine syndrome a preexcitation syndrome of electrocardiographic abnormality characterized by a short PR interval with a normal QRS complex, accompanied by atrial tachycardia. Lutembacher's syndrome atrial septal defect with mitral stenosis (usually rheumatic). lymphadenopathy syndrome unexplained lymphadenopathy for 3 or more months at extrainguinal sites, revealing on biopsy nonspecific lymphoid hyperplasia, possibly a prodrome of acquired immunodeficiency syndrome. Maffucci's syndrome enchondromatosis with multiple cutaneous or visceral hemangiomas. malabsorption syndrome a group of disorders marked by subnormal absorption of dietary constituents, and thus excessive loss of nutrients in the stool, which may be due to a digestive defect, a mucosal abnormality, or lymphatic obstruction. male Turner's syndrome Noonan's s. Marfan syndrome a hereditary syndrome of abnormal length of limbs, especially fingers and toes, with subluxation of the lens, cardiovascular abnormalities, and other defects. Marie-Bamberger syndrome hypertrophic pulmonary osteoarthropathy. maternal deprivation syndrome failure to thrive with severe growth retardation, unresponsiveness to the environment, depression, retarded mental and emotional development, and behavioral problems resulting from loss, absence, or neglect of the mother or other primary caregiver. Meckel's syndrome an autosomal recessive syndrome, with sloping forehead, posterior meningoencephalocele, polydactyly, polycystic kidneys, and death in the perinatal period. meconium aspiration syndrome the respiratory complications resulting from the passage and aspiration of meconium prior to or during delivery. median cleft facial syndrome a hereditary form of defective midline development of the head and face, including ocular hypertelorism, occult cleft nose and maxilla, and sometimes mental retardation or other defects. megacystis-megaureter syndrome chronic ureteral dilatation (megaureter) associated with hypotonia and dilatation of the bladder (megacystis) and gaping of ureteral orifices, permitting vesicoureteral reflux of urine, and resulting in chronic pyelonephritis. megacystis-microcolonintestinal hypoperistalsis syndrome (MMIHS) enlarged bladder (megacystis), small colon with decreased or absent peristalsis (microcolon and intestinal hypoperistalsis), and the same abdominal muscle defect as occurs in prune-belly syndrome. Meige syndrome 1. Milroy's disease.

2. dystonia of facial and oromandibular muscles with blepharospasm, grimacing mouth movements, and protrusion of the tongue. MELAS syndrome a maternally-inherited syndrome of m itochondrial e ncephalopathy, l actic a cidosis, ands troke-like episodes. Menkes' syndrome an X-linked recessive disorder of copper absorption marked by severe cerebral degeneration and arterial changes resulting in death in infancy and by sparse, brittle scalp hair. Meretoja's syndrome a type of familial amyloid polyneuropathy. MERRF syndrome a maternally-inherited syndrome of m yoclonus with e pilepsy and with r agged r ed fibers. metabolic syndrome a combination including at least three of the following: abdominal obesity,hypertriglyceridemia, low level of high-density lipoproteins, hypertension, and high fasting glucose level. methionine malabsorption syndrome an inborn aminoacidopathy marked by white hair, mental retardation, convulsions, attacks of hyperpnea, and urine with an odor like an oasthouse (for drying hops) due to alpha-hydroxybutyric acid formed by bacterial action on the unabsorbed methionine. middle lobe syndrome lobar atelectasis in the right middle lobe of the lung, with chronic pneumonitis. Mikulicz's syndrome chronic bilateral hypertrophy of the lacrimal, parotid, and salivary glands, associated with chronic lymphocytic infiltration; it may be associated with other diseases. milk-alkali syndrome hypercalcemia without hypercalciuria or hypophosphatemia and with only mild alkalosis and other symptoms attributed to ingestion of milk and absorbable alkali for long periods. Milkman syndrome a generalized bone disease marked by multiple transparent stripes of absorption in the long and flat bones. Miller syndrome an inherited syndrome of extensive facial and limb defects, sometimes accompanied by heart defects and hearing loss. mitral valve prolapse syndrome prolapse of the mitral valve, often with regurgitation; a common, usually benign, often asymptomatic condition characterized by midsystolic clicks and late systolic murmurs on auscultation. Mbius' syndrome agenesis or aplasia of cranial nerve motor nuclei in congenital bilateral facial palsy, with unilateral or bilateral paralysis of abductors of the eye and sometimes cranial nerve involvement and limb anomalies. Mohr syndrome an autosomal recessive disorder characterized by brachydactyly, clinodactyly, polydactyly, syndactyly, and bilateral hallucal polysyndactyly; by cranial, facial, lingual, palatal, and mandibular anomalies; and by episodic neuromuscular disturbances. Morquio's syndrome two biochemically distinct but clinically nearly indistinguishable forms of mucopolysaccharidosis, marked by genu valgum, pigeon breast, progressive flattening of the vertebral bodies, short neck and trunk, progressive deafness, mild corneal clouding, and excretion of keratan sulfate in the urine.

mucocutaneous lymph node syndrome Kawasaki disease. multiple endocrine deficiency syndrome , multiple glandular deficiency syndrome failure of any combination of endocrine glands, often accompanied by nonendocrine autoimmune abnormalities. multiple pterygium syndrome an inherited syndrome characterized by pterygia of the neck, axillae, and popliteal, antecubital, and intercrural areas, accompanied by facial, skeletal, and genital abnormalites. Munchausen syndrome a subtype of factitious disorder; habitual seeking of hospital treatment for apparent acute illness, the patient giving a plausible and dramatic history, all of which is false. Munchausen syndrome by proxy see factitious disorder by proxy, under disorder. MVP syndrome mitral valve prolapse s. myelodysplastic syndrome any of a group of related bone marrow disorders of varying duration preceding the development of overt acute myelogenous leukemia; characterized by abnormal hematopoietic stem cells, anemia, neutropenia, and thrombocytopenia. myeloproliferative syndromes see under disorder. NAME syndrome a syndrome of familial myxomas with cutaneous, cardiac, and endocrine involvement, manifested as n evi, a trial m yxoma, and neurofibroma e phelides. Negri-Jacod syndrome Jacod's s. Nelson's syndrome the development of an ACTH-producing pituitary tumor after bilateral adrenalectomy in Cushing's syndrome; it is characterized by aggressive growth of the tumor and hyperpigmentation of the skin. nephrotic syndrome any of a group of diseases involving defective kidney glomeruli, with massive proteinuria, lipiduria with edema, hypoalbuminemia, and hyperlipidemia. nerve compression syndrome entrapment neuropathy. Noack syndrome Pfeiffer's s. nonstaphylococcal scalded skin syndrome toxic epidermal necrolysis. Noonan syndrome webbed neck, ptosis, hypogonadism, and short stature, i.e., the phenotype of Turner's syndrome without the gonadal dysgenesis. obesity-hypoventilation syndrome pickwickian syndrome; a syndrome of obesity, somnolence, hypoventilation, and erythrocytosis. occipital horn syndrome the X-linked recessive form of cutis laxa. oculocerebrorenal syndrome an X-linked disorder marked by vitamin Drefractory rickets, hydrophthalmia, congenital glaucoma and cataracts, mental retardation, and renal tubule dysfunction as evidenced by hypophosphatemia, acidosis, and aminoaciduria. oculodentodigital syndrome , ODD syndrome oculodentodigital dysplasia. OFD syndrome oral-facial-digital s.

Omenn's syndrome histiocytic medullary reticulosis. Opitz syndrome , Opitz-Frias syndrome a familial syndrome consisting of hypertelorism and hernias, and in males also characterized by hypospadias, cryptorchidism, and bifid scrotum. Cardiac, laryngotracheal, pulmonary, anal, and renal abnormalities may also be present. oral-facial-digital syndrome any of a group of congenital syndromes characterized by oral, facial, and digital anomalies. Type I, a male-lethal X-linked dominant disorder, is characterized by camptodactyly, polydactyly, and syndactyly; by cranial, facial, lingual, and dental anomalies; and by mental retardation, familial trembling, alopecia, and seborrhea of the face and milia; type II is Mohr s.; type III, an autosomal recessive disorder, characterized by postaxial hexadactyly, by ocular, lingual, and dental anomalies, and by profound mental retardation. orbital floor syndrome exophthalmos, diplopia, and anesthesia in the areas innervated by the trigeminal nerve, occurring with a lesion in the floor of the orbit. organic anxiety syndrome a term used in a former system of classification, denoting an organic mental syndrome marked by prominent, recurrent panic attacks or generalized anxiety caused by a specific organic factor and not associated with delirium. organic brain syndrome organic mental s. organic delusional syndrome a term used in a former system of classification, denoting an organic mental syndrome marked by delusions caused by a specific organic factor and not associated with delirium. organic mental syndrome former term for a constellation of psychological or behavioral signs and symptoms associated with brain dysfunction of unknown or unspecified etiology and grouped according to symptoms rather than etiology. See also under disorder. organic mood syndrome a term used in a former system of classification, denoting an organic mental syndrome marked by manic or depressive mood disturbance caused by a specific organic factor and not associated with delirium. organic personality syndrome a term used in a former system of classification, denoting an organic mental syndrome characterized by a marked change in behavior or personality, caused by a specific organic factor and not associated with delirium or dementia. orofaciodigital syndrome oral-facial-digital s. Ortner syndrome laryngeal paralysis associated with heart disease, due to compression of the recurrent laryngeal nerve between the aorta and a dilated pulmonary artery. ovarian hyperstimulation syndrome mild to severe ovarian enlargement with exudation of fluid and protein, leading to ascites, pleural or pericardial effusion, azotemia, oliguria, and thromboembolism in women undergoing ovulation induction. ovarian vein syndrome obstruction of the ureter due to compression by an enlarged or varicose ovarian vein; typically the vein becomes enlarged during pregnancy.

overlap syndrome any of a group of connective tissue disorders that either combine scleroderma with polymyositis or systemic lupus erythematosus or combine systemic lupus erythematosus with rheumatoid arthritis or polymyositis. overwear syndrome extreme photophobia, pain, and lacrimation associated with contact lenses, particularly nongas permeable hard lenses, usually caused by wearing them excessively. pacemaker syndrome vertigo, syncope, and hypotension, often accompanied by dyspnea, cough, nausea, peripheral edema, and palpitations, all exacerbated or caused by pacemakers that stimulate the ventricle and therefore do not maintain normal atrioventricular synchrony. pacemaker twiddler's syndrome twiddler's syndrome in a patient with an artificial cardiac pacemaker. painful bruising syndrome occurrence of one or more spontaneous, chronic recurring painful ecchymoses without antecedent trauma or after insufficient trauma; sometimes precipitated by emotional stress. Because certain patients exhibit autoerythrocyte sensitization in which intradermal injection of their own erythrocytes produces a painful ecchymosis, some consider the condition to be an autosensitivity to a component of the erythrocyte membrane; others consider it to be of psychosomatic or factitious origin. Pancoast's syndrome 1. neuritic pain and muscle atrophy in the upper limb, and Horner's syndrome, seen with a tumor near the apex of the lung when it involves the brachial plexus. 2. osteolysis in the posterior part of a rib or ribs, sometimes spreading to adjacent vertebrae. paraneoplastic syndrome a symptom complex arising in a cancer-bearing patient that cannot be explained by local or distant spread of the tumor. Parinaud's syndrome paralysis of conjugate upward movement of the eyes without paralysis of convergence; associated with tumors of the midbrain. Parinaud's oculoglandular syndrome a general term applied to conjunctivitis, usually unilateral and of the follicular type, followed by tenderness and enlargement of the preauricular lymph nodes; often due to leptotrichosis but may be associated with other infections. parkinsonian syndrome a form of parkinsonism due to idiopathic degeneration of the corpus striatum or substantia nigra; frequently a sequela of lethargic encephalitis. PEP syndrome POEMS s. Pepper syndrome neuroblastoma with metastases to the liver. persistent mllerian duct syndrome a hereditary syndrome in males of persistence of mllerian structures in addition to male genital ducts. There may be cryptorchidism on just one side with a contralateral inguinal hernia that contains a testis, uterus, and uterine tube (hernia uteri inguinalis). Peutz-Jeghers syndrome familial gastrointestinal polyposis, especially in the small bowel, associated with mucocutaneous pigmentation. Pfeiffer syndrome acrocephalosyndactyly, type V; an autosomal dominant disorder characterized by acrocephalosyndactyly associated with broad short thumbs and big toes.

pickwickian syndrome obesity-hypoventilation s. Pierre Robin syndrome micrognathia with cleft palate, glossoptosis, and absent gag reflex. plica syndrome pain, tenderness, swelling, and crepitus of the knee joint, sometimes with weakness or locking of the joint, caused by fibrosis and calcification of the synovial plicae. Plummer-Vinson syndrome dysphagia with glossitis, hypochromic anemia, splenomegaly, and atrophy in the mouth, pharynx, and upper end of the esophagus. POEMS syndrome p olyneuropathy, o rganomegaly, e ndocrinopathy, M component, and s kin changes, sometimes linked to a dysproteinemia such as the presence of unusual monoclonal proteins and light chains. polyangiitis overlap syndrome a form of systemic necrotizing vasculitis resembling polyarteritis nodosa and allergic angiitis but also showing features of hypersensitivity vasculitis. polycystic ovary syndrome (PCOS) a clinical symptom complex associated with polycystic ovaries and characterized by oligomenorrhea or amenorrhea, anovulation (hence infertility), and hirsutism; both hyperestrogenism and hyperandrogenism are present. polysplenia syndrome a congenital syndrome of multiple splenic masses, abnormal position and development of visceral organs, complex cardiovascular defects, and abnormal, usually bilobate, lungs. postcardiac injury syndrome fever, chest pain, pleuritis, and pericarditis weeks after injury to the heart, including that due to surgery (postpericardiotomy s.) and that due to myocardial infarction (post) . postcardiotomy syndrome postpericardiotomy s. postcardiotomy psychosis syndrome anxiety, confusion, and perception disturbances occurring three or more days after open heart surgery. postcommissurotomy syndrome postpericardiotomy s. postconcussional syndrome physical and personality changes that may occur after concussion of the brain, including amnesia, headache, dizziness, tinnitus, irritability, fatigability, sweating, heart palpitations, insomnia, and difficulty concentrating. postgastrectomy syndrome dumping s. postlumbar puncture syndrome headache in the erect posture, sometimes with nuchal pain, vomiting, diaphoresis, and malaise, all relieved by recumbency, occurring several hours after lumbar puncture; it is due to lowering of intracranial pressure by leakage of cerebrospinal fluid through the needle tract. postmaturity syndrome a syndrome due to placental insufficiency that causes chronic stress and hypoxia, seen in fetuses and neonates in postterm pregnancies, characterized by decreased subcutaneous fat, skin desquamation, and long fingernails, often with yellow meconium staining of the nails, skin, and vernix. postmyocardial infarction syndrome post after myocardial infarction. postpericardiotomy syndrome post after surgery with opening of the pericardium.

Potter's syndrome oligohydramnios sequence. preexcitation syndrome any syndrome with electrocardiographic signs of preexcitation, such as WolffParkinson-White syndrome; sometimes used synonymously with it. premenstrual syndrome some or all of the symptoms of depressed, anxious, angry, or irritable mood, emotional lability, bloating, edema, headache, increased fatigue or lethargy, altered appetite or food cravings, breast swelling and tenderness, constipation, and decreased ability to concentrate occurring in the period between ovulation and the onset of menstruation. prune-belly syndrome a congenital syndrome of deficient or absent anterior abdominal wall musculature, urinary tract anomalies, and undescended testicles. The abdomen is protruding and thinwalled, with wrinkled skin. Putnam-Dana syndrome subacute combined degeneration of the spinal cord. Raeder syndrome , Raeder paratrigeminal syndrome unilateral paroxysmal neuralgic pain in the face associated with Horner's syndrome. Ramsay Hunt syndrome 1. geniculate neuralgia; facial paralysis with otalgia and a vesicular eruption in the external canal of the ear, sometimes extending to the auricle, due to herpes zoster virus infection of the geniculate ganglion. 2. juvenile paralysis agitans (of Hunt). 3. dyssynergia cerebellaris progressiva. Reiter syndrome the triad of nongonococcal urethritis, conjunctivitis, and arthritis, frequently with mucocutaneous lesions. respiratory distress syndrome of the newborn a condition seen in infants born prematurely, by cesarean section, or to diabetic mothers, marked by dyspnea and cyanosis; a common, usually fatal subtype is hyaline membrane disease. Reye's syndrome a rare often fatal encephalopathy of childhood, marked by acute brain swelling with hypoglycemia, fatty infiltration of the liver, hepatomegaly, and disturbed consciousness and seizures, usually seen as a sequel of varicella or an upper airway viral infection. Rh-null syndrome chronic hemolytic anemia affecting individuals who lack all Rh factors (Rhnull); it is marked by spherocytosis, stomatocytosis, and increased osmotic fragility. Riley-Day syndrome familial dysautonomia. Rosenberg-Bergstrom syndrome an inherited syndrome of hyperuricemia, renal insufficiency, ataxia, and deafness. Rukavina's syndrome a type of familial amyloid polyneuropathy. Rundles-Falls syndrome hereditary sideroblastic anemia. Ruvalcaba's syndrome abnormal shortness of the metacarpal or metatarsal bones, hypoplastic genitalia, and mental and physical retardation of unkown etiology, present from birth in males.

Saethre-Chotzen syndrome Chotzen's s. salt-depletion syndrome , salt-losing syndrome vomiting, dehydration, hypotension, and sudden death due to very large sodium losses from the body. It may be seen in abnormal losses of sodium into the urine (as in congenital adrenal hyperplasia, adrenocortical insufficiency, or one of the forms of salt-losing nephritis) or in large extrarenal sodium losses, usually from the gastrointestinal tract. Sanfilippo's syndrome four biochemically distinct but clinically indistinguishable forms of mucopolysaccharidosis, characterized by urinary excretion of heparan sulfate, rapid mental deterioration, and mild Hurler-like symptoms, with death usually occurring before 20 years of age. scalenus syndrome , scalenus anticus syndrome a type of thoracic outlet syndrome due to compression of the nerves and vessels between a cervical rib and the scalenus anticus muscle, with pain over the shoulder, often extending down the arm or radiating up the back. Schaumann's syndrome sarcoidosis. Scheie's syndrome a mild allelic variant of Hurler's syndrome, marked by corneal clouding, clawhand, aortic valve involvement, wide-mouthed facies, genu valgus, and pes cavus; stature, intelligence, and life span are normal. second impact syndrome acute, usually fatal, brain swelling and increased cranial pressure, caused by repeated head trauma in a short space of time, so that a second concussion occurs before recovery from a previous concussion is complete. Sertoli-cellonly syndrome congenital absence of the germinal epithelium of the testes, the seminiferous tubules containing only Sertoli cells, marked by testes slightly smaller than normal, azoospermia, and elevated titers of follicle-stimulating hormone and sometimes of luteinizing hormone. severe acute respiratory syndrome (SARS) an infectious respiratory illness characterized by fever, dry cough, and breathing difficulties, often accompanied by headache and body aches; believed to be caused by a coronavirus. Szary syndrome a form of cutaneous T-cell lymphoma manifested by exfoliative erythroderma, intense pruritus, peripheral lymphadenopathy, and abnormal hyperchromatic mononuclear cells in the skin, lymph nodes, and peripheral blood. Sheehan's syndrome postpartum pituitary necrosis. short-bowel syndrome , short-gut syndrome any of the malabsorption conditions resulting from massive resection of the small bowel, the degree and kind of malabsorption depending on the site and extent of the resection; it is characterized by diarrhea, steatorrhea, and malnutrition. shoulder-hand syndrome reflex sympathetic dystrophy limited to the upper limb. Shprintzen's syndrome velocardiofacial s. Shwachman syndrome , Shwachman-Diamond syndrome primary pancreatic insufficiency and bone marrow failure, characterized by normal sweat chloride values, pancreatic insufficiency, and neutropenia; it may be associated with dwarfism and metaphyseal dysostosis of the hips.

sick sinus syndrome intermittent bradycardia, sometimes with episodes of atrial tachyarrhythmias or periods of sinus arrest, due to malfunction originating in the supraventricular portion of the cardiac conducting system. Silver-Russell syndrome a syndrome of low birth weight despite normal gestation duration, and short stature, lateral asymmetry, and some increase in gonadotropin secretion. Sipple's syndrome multiple endocrine neoplasia, type II. Sjgren's syndrome a symptom complex usually in middle-aged or older women, marked by keratoconjunctivitis sicca, xerostomia, and enlargement of the parotid glands; it is often associated with rheumatoid arthritis and sometimes with systemic lupus erythematosus, scleroderma, or polymyositis. sleep apnea syndrome sleep apnea. Smith-Lemli-Opitz syndrome an autosomal recessive syndrome of microcephaly, mental retardation, hypotonia, incomplete development of male genitalia, short nose with anteverted nostrils, and syndactyly of second and third toes. social breakdown syndrome deterioration of social and interpersonal skills, work habits, and behavior seen in chronically hospitalized psychiatric patients; due to the effects of long-term institutionalization rather than the primary illness. stagnant loop syndrome stasis s. staphylococcal scalded skin syndrome an infectious disease, usually affecting infants and young children, following infection with certain strains of Staphylococcus aureus, characterized by localized to widespread bullous eruption and exfoliation of the skin leaving raw, denuded areas that make the skin look scalded. stasis syndrome overgrowth of bacteria in the small intestine secondary to various disorders causing stasis; it is characterized by malabsorption of vitamin B12, steatorrhea, and anemia. Steele-Richardson-Olszewski syndrome a progressive neurological disorder with onset during the sixth decade, characterized by supranuclear ophthalmoplegia, especially paralysis of the downward gaze, pseudobulbar palsy, dysarthria, dystonic rigidity of the neck and trunk, and dementia. Stein-Leventhal syndrome polycystic ovary s. Stevens-Johnson syndrome a sometimes fatal form of erythema multiforme presenting with a flulike prodrome and characterized by severe mucocutaneous lesions; pulmonary, gastrointestinal, cardiac, and renal involvement may occur. Stewart-Treves syndrome lymphangiosarcoma occurring as a late complication of severe lymphedema of the arm after excision of the lymph nodes, usually in radical mastectomy. stiff-man syndrome a condition of unknown etiology marked by progressive fluctuating rigidity of axial and limb muscles in the absence of signs of cerebral and spinal cord disease but with continuous electromyographic activity. stroke syndrome stroke; a condition with sudden onset due to acute vascular lesions of the brain (hemorrhage, embolism, thrombosis, rupturing aneurysm), which may be marked by hemiplegia or

hemiparesis, vertigo, numbness, aphasia, and dysarthria, and often followed by permanent neurologic damage. Sturge's syndrome , Sturge-Kalischer-Weber syndrome, Sturge-Weber syndrome a congenital syndrome consisting of a port-wine stain type of nevus flammeus distributed over the trigeminal nerve accompanied by a similar vascular disorder of the underlying meninges and cerebral cortex. subclavian steal syndrome cerebral or brain stem ischemia due to vertebrobasilar insufficiency in cases of subclavian steal. sudden infant death syndrome sudden and unexpected death of an infant who had previously been apparently well, and which is unexplained by careful postmortem examination. Swyer-James syndrome acquired unilateral hyperlucent lung, with severe airway obstruction during exhalation, oligemia, and a small hilum. tarsal tunnel syndrome a complex of symptoms resulting from compression of the posterior tibial nerve or of the plantar nerves in the tarsal tunnel, with pain, numbness, and tingling paresthesia of the sole of the foot. Taussig-Bing syndrome transposition of the great vessels of the heart and a ventricular septal defect straddled by a large pulmonary artery. testicular feminization syndrome complete androgen resistance. thoracic outlet syndrome any of several neurovascular syndromes due to compression of the brachial plexus nerve trunks, with pain, paresthesias, vasomotor symptoms, and weakness and small muscle wasting in upper limbs; causes include drooping shoulder girdle, a cervical rib or fibrous band, an abnormal first rib, limb hyperabduction (as during sleep), or compression of the edge of the scalenus anterior muscle. Tolosa-Hunt syndrome unilateral ophthalmoplegia associated with pain behind the orbit and in the area supplied by the first division of the trigeminal nerve; it is thought to be due to nonspecific inflammation and granulation tissue in the superior orbital fissure or cavernous sinus. TORCH syndrome (t oxoplasmosis, o ther agents, r ubella, c ytomegalovirus, h erpes simplex) any of a group of infections seen in neonates as a result of the infectious agent having crossed the placental barrier. Tourette's syndrome Gilles de la Tourette's s. Townes' syndrome an inherited disorder of auricular anomalies, anal defects, limb and digit anomalies, and renal deficiencies, occasionally including cardiac disease, deafness, or cystic ovary. toxic shock syndrome a severe illness with sudden high fever, vomiting, diarrhea, and myalgia, followed by hypotension and, in severe cases, shock; a sunburn-like rash with skin peeling, especially on palms and soles, occurs during the acute phase. It primarily affects menstruating women using tampons, although a few women not using tampons and a few males have been affected. It is thought to be caused by infection withStaphylococcus aureus. Treacher Collins syndrome the incomplete form of mandibulofacial dysostosis.

trisomy 8 syndrome a syndrome due to an extra chromosome 8, usually mosaic (trisomy 8/normal), with mild to severe mental retardation, prominent forehead, deep-set eyes, thick lips, prominent ears, and camptodactyly. trisomy 11q syndrome a variable syndrome due to an extra long arm of chromosome 11, possibly including preauricular fistulas, hypoplasia of the gallbladder, micropenis, bicornuate uterus, microphthalmos, malformations of the heart, lungs, and brain, seizures, and recurrent infection. trisomy 13 syndrome holoprosencephaly due to an extra chromosome 13, in which central nervous system defects are associated with mental retardation, along with cleft lip and palate, polydactyly, and dermal pattern anomalies, and abnormalities of the heart, viscera, and genitalia. trisomy 18 syndrome neonatal hepatitis, mental retardation, scaphocephaly or other skull abnormality, micrognathia, blepharoptosis, low-set ears, corneal opacities, deafness, webbed neck, short digits, ventricular septal defects, Meckel's diverticulum, and other deformities. It is due to an extra chromosome 18. trisomy 21 syndrome Down s. Trousseau's syndrome spontaneous venous thrombosis of upper and lower limbs associated with visceral carcinoma. tumor lysis syndrome severe hyperphosphatemia, hyperkalemia, hyperuricemia, and hypocalcemia after effective induction chemotherapy of rapidly growing malignant neoplasms. Turcot's syndrome familial polyposis of the colon associated with gliomas of the central nervous sytem. Turner's syndrome gonadal dysgenesis with short stature, undifferentiated (streak) gonads, and variable abnormalities such as webbing of neck, low posterior hair line, increased carrying angle of elbow, cubitus valgus, and cardiac defects. The genotype is XO (45, X) or X/XX or X/XXX mosaic. The phenotype is female. twiddler's syndrome dislodgement, breakdown, or other malfunction of an implanted diagnostic device as a result of unconscious or habitual manipulation by the patient. twin transfusion syndrome , twintwin transfusion syndrome one caused by twin-to-twin transfusion (q.v.); the donor twin is small, pale, and anemic, while the recipient is large and polycythemic, with an overloaded cardiovascular system.

Twin transfusion syndrome characterized by arteriovenous shunt at a shared placental cotyledon in diamniotic monochorionic twins.

urethral syndrome symptoms associated with a urethral problem other than infection, including suprapubic aching and cramping, urinary frequency, and bladder complaints such asdysuria, tenesmus, and low back pain.

Usher's syndrome an inherited syndrome of congenital deafness with retinitis pigmentosa, often ending in blindness; mental retardation and gait disturbances may also occur. velocardiofacial syndrome an inherited syndrome of cardiac defects and craniofacial anomalies, often with abnormalities of chromosome 22; learning disabilities often occur, and less often other abnormalities. Vernet's syndrome paralysis of the glossopharyngeal, vagus, and spinal accessory nerves due to a lesion in the region of the jugular foramen. Vogt-Koyanagi-Harada syndrome bilateral uveitis with iridocyclitis, exudative choroiditis, meningism, and retinal detachment, accompanied by alopecia, vitiligo, poliosis, loss of visual acuity, headache, vomiting, and deafness; possibly an inflammatory autoimmune disorder. Waardenburg's syndrome a hereditary, autosomal dominant disorder characterized by wide bridge of the nose due to lateral displacement of the inner canthi and puncta, pigmentary disturbances, including white forelock, heterochromia iridis, white eyelashes, leukoderma, and sometimes cochlear hearing loss. WAGR syndrome a syndrome of W ilms' tumor, a niridia, g enitourinary abnormalities or g onadoblastoma, and mental r etardation, due to a deletion in chromosome 11. Walker-Warburg syndrome , Warburg's syndrome a usually fatal congenital syndrome of hydrocephalus, agyria, various ocular anomalies, and sometimes encephalocele. Waterhouse-Friderichsen syndrome the malignant or fulminating form of epidemic cerebrospinal meningitis, with sudden onset, short course, fever, collapse, coma, cyanosis, petechiae on the skin and mucous membranes, and bilateral adrenal hemorrhage. Weber's syndrome paralysis of the oculomotor nerve on the same side as the lesion, causing ptosis, strabismus, and loss of light reflex and accommodation; also spastic hemiplegia on the side opposite the lesion with increased reflexes and loss of superficial reflexes. Weil's syndrome a severe form of leptospirosis, marked by jaundice usually accompanied by azotemia, hemorrhage, anemia, disturbances of consciousness, and continued fever. Werner's syndrome premature aging of an adult, with early graying and some hair loss, cataracts, hyperkeratinization, muscular atrophy, scleroderma-like changes in the skin of the limbs, and a high incidence of neoplasm. Wernicke-Korsakoff syndrome a neuropsychiatric disorder caused by thiamine deficiency, most often due to alcohol abuse, combining the features of Wernicke's encephalopathy and Korsakoff's syndrome. whiplash shake syndrome subdural hematomas, retinal hemorrhage, and sometimes cerebral contusions caused by the stretching and tearing of cerebral vessels and brain substance, sometimes seen when a very young child is shaken vigorously by the limbs or trunk with the head unsupported; paralysis, visual disturbances, blindness, convulsions, and death may result. Wilson-Mikity syndrome a rare form of pulmonary insufficiency in low-birth-weight infants, with hyperpnea and cyanosis during the first month of life, sometimes ending in death; there are also radiologic abnormalities.

Wiskott-Aldrich syndrome chronic eczema with chronic suppurative otitis media, anemia, and thrombocytopenic purpura, an immunodeficiency syndrome transmitted as an X-linked recessive trait, with poor antibody response to polysaccharide antigens and dysfunction of cell-mediated immunity. withdrawal syndrome substance withdrawal. Wolf-Hirschhorn syndrome a syndrome due to partial deletion of the short arm of chromosome 4, with microcephaly, ocular hypertelorism, epicanthus, cleft palate, micrognathia, low-set ears simplified in form, cryptorchidism, and hypospadias. Wolff-Parkinson-White (WPW) syndrome the association of paroxysmal tachycardia (or atrial fibrillation) and preexcitation, in which the electrocardiogram displays a short PR interval and a wide QRS complex which characteristically shows an early QRS vector (delta wave). Wyburn-Mason's syndrome arteriovenous aneurysms on one or both sides of the brain, with ocular anomalies, facial nevi, and sometimes mental retardation. syndrome X angina pectoris or angina-like chest pain associated with normal arteriographic appearance of the coronary arteries. Zollinger-Ellison syndrome the association of atypical, intractable, sometimes fulminating, peptic ulcers with extreme gastric hyperacidity and benign or malignant gastrinomas in the pancreas.

Dorland's Medical Dictionary for Health Consumers. 2007 by Saunders, an imprint of Elsevier, Inc. All rights reserved.

adult respiratory distress syndrome

n. Abbr. ARDS Interstitial and alveolar edema and hemorrhage of the lungs following injury and associated with hyaline membrane, proliferation of collagen fibers, and swollen epithelium. Also called acute respiratory distress syndrome.
The American Heritage Medical Dictionary Copyright 2007, 2004 by Houghton Mifflin Company. Published by Houghton Mifflin Company. All rights reserved.

adult respiratory distress syndrome (ARDS),

severe pulmonary congestion characterized by diffuse injury to alveolar-capillary membranes. Fulminating sepsis, especially when gram-negative bacteria are involved, is the most common cause. ARDS may occur after trauma; near-drowning; aspiration of gastric acid; paraquat ingestion; inhalation of corrosive chemicals, such as chlorine, ammonia, or phosgene; or the use of certain drugs, including barbiturates,

chlordiazepoxide, heroin, methadone hydrochloride, propoxyphene hydrochloride, and salicylates. Other causes include diabetic ketoacidosis, fungal infections, high altitude, pancreatitis, tuberculosis, and uremia. Also called acute respiratory distress syndrome, congestive atelectasis, hemorrhagic lung, noncardiogenic pulmonary edema, pump lung, shock lung, stiff lung, wet lung.

observations Signs and symptoms include dyspnea, tachypnea, adventitious breath sounds, hypoxemia, and decreased lung compliance. Changes occurring within the lung include damage to the alveolarcapillary membranes, leakage of plasma proteins into the alveoli, dilution of surfactant, cessation of surfactant production, hemorrhage, interstitial edema, impaired gas exchange, and ventilation-perfusion defects.

interventions Treatment includes establishing an airway, administering oxygen, improving the underlying condition, removing the cause of ARDS, suctioning the respiratory passages as necessary, and reducing oxygen consumption. When ventilation cannot be maintained and there is evidence of a rising partial pressure of carbon dioxide in arterial blood, mechanical ventilation is necessary. Positive end-expiratory pressure (PEEP) is widely used in the treatment of ARDS. All interventions for ARDS are supportive; there is no cure.

nursing considerations The patient with ARDS requires constant and meticulous care, reassurance, and observation for changes in respiratory function and adequacy, including signs of hypercapnia, hypoxemia especially confusion), skin flushing, and behavior changes such as agitation and restlessness. Increasing hypoxia may be signaled by tachycardia, elevated blood pressure, increased peripheral resistance, and fulminating respiratory failure. If PEEP is being used, the patient is carefully observed for a sudden disappearance of breath sounds accompanied by signs of respiratory distress-an indication that pneumothorax may be present. Respiratory therapy, sterile suction techniques, and position changes are continued as necessary. The patient's weight is measured frequently, chest x-ray films are evaluated, and bacteriologic cultures of secretions are analyzed for the causative organism. Throughout treatment, ventilation is carefully monitored through analysis of arterial blood gases. ARDS has a 50% mortality rate.

Mosby's Medical Dictionary, 8th edition. 2009, Elsevier.

adult respiratory distress syndrome

Pulmonary medicine A clinical condition consisting of a diffuse infiltrative process in the lung, which affects 150,000/yr US characterized by acute pulmonary edema and respiratory failure, poor oxygenation, functional residual capacity, and compliance; ARDS may accompany various medical and surgical conditions and may be associated with interstitial pneumonitis usual, desquamative and lymphoid types Etiology Gram-negative sepsis, pneumonia, shock, gastric acid aspiration, trauma, drug overdose, toxic gas chlorine, NO2, smoke exposure, severe metabolic derangement, pancreatitis Clinical A 6-24 hr latency period is followed by hypoxia, aeration, dyspnea, severe SOB, 'stiff' lungs, ie pulmonary compliance Radiology Extensive, diffuse bilateral fluffy infiltrates Management Nitric oxide

NO, 18 ppm in one study inhalation therapy results in a mean pulmonary artery pressure37 to 30 mm Hg, intrapulmonary shunting36% to 31%, ratio of partial pressure of arterial O2 to the fraction of inspired O2PaO2/FiO2, an index of arterial oxygenation efficiency152 to 199 PEEP, prayer Prognosis A function of underlying cause Mortality 60%, the cause of death has shifted from hypoxia to multiorgan failure

ARDS (cont.)
In this Article

y y y y y y y y y y y y y y

What is ARDS? How do the lungs work? What are other names for ARDS? What causes ARDS? Who is at risk for ARDS? What are the signs and symptoms of ARDS? How is ARDS diagnosed? How is ARDS treated? Recovering from ARDS What are the complications of ARDS? Information for family and friends Key Points About ARDS ARDS Glossary ARDS Index

How the Lungs Work

To understand acute respiratory distress syndrome (ARDS), it is helpful to understand how your lungs work. Normal Lung Function A slice of normal lung looks like a pink spongefilled with tiny bubbles or holes. Around each bubble is a fine network of tiny blood vessels. These bubbles, which are surrounded by blood vessels, give the lungs a large surface to exchange oxygen (into the blood where it is carried throughout the body) and carbon dioxide (out of the blood). This process is called gas exchange. Healthy lungs do this very well. Here's how normal breathing works:

You breathe in air through your nose and mouth. The air travels down through your windpipe (trachea) through large and small tubes in your lungs called bronchial (BRON-kee-ul) tubes. The larger tubes are bronchi (BRONKeye), and the smaller tubes are bronchioles (BRON-kee-oles). Sometimes, we use the word "airways" to refer to the various tubes or passages that air uses to travel from the nose and mouth into the lungs. The airways in your lungs look something like an upside-down tree with many branches.

At the ends of the small bronchial tubes, there are groups of tiny bubbles called air sacs or alveoli. The bubbles have very thin walls, and small blood vessels called capillaries are next to them. Oxygen passes from the air sacs into the blood in these small blood vessels. At the same time, carbon dioxide passes from the blood into the air sacs.

Effects of ARDS In ARDS, the tiny blood vessels leak too much fluid into the lungs. This results from toxins (poisons) that the body produces in response to the underlying illness or injury. The lungs become like a wet sponge, heavy and stiffer than normal. They no longer provide the effective surface for gas exchange, and the level of oxygen in the blood falls. If ARDS is severe and goes on for some time, scar tissue called fibrosis may form in the lungs. The scarring also makes it harder for gas exchange to occur. People who develop ARDS need extra oxygen and may need a breathing machine to breathe for them while their lungs try to heal. If they survive, ARDS patients may have a full recovery. Recovery can take weeks or months. Some ARDS survivors take a year or longer to recover, and some never completely recover from having ARDS. Other Names for ARDS

y y y y

Adult respiratory distress syndrome Stiff lung Shock lung Wet lung

There is a similar condition in infants called Infant Respiratory Distress Syndrome (also called IRDS, RDS, and hyaline membrane disease). It mainly affects premature infants whose lungs are not well developed when they are born.

What Causes ARDS?

The causes of acute respiratory distress syndrome (ARDS) are not well understood. It can occur in many situations and in persons with or without a lung disease. There are two ways that lung injury leading to ARDS can occur: through a direct injury to the lungs, or indirectly when a person is very sick or has a serious bodily injury. However, most sick or badly injured persons do not develop ARDS. Direct Lung Injury A direct injury to the lungs may result from breathing in harmful substances or an infection in the lungs. Some direct lung injuries that can lead to ARDS include:

y y y y

Severe pneumonia (infection in the lungs) Breathing in vomited stomach contents Breathing in harmful fumes or smoke A severe blow to the chest or other accident that bruises the lungs Indirect Lung Injury Most cases of ARDS happen in people who are very ill or who have been in a major accident. This is sometimes called an indirect lung injury. Less is known about how indirect injuries lead to ARDS than about how direct injuries to the lungs cause ARDS. Indirect lung injury leading to ARDS sometimes occurs in cases of:

y y y y y

Severe and widespread bacterial infection in the body (sepsis) Severe injury with shock Severe bleeding requiring blood transfusions Drug overdose Inflamed pancreas It is not clear why some very sick or seriously injured people develop ARDS, and others do not. Researchers are trying to find out why ARDS develops and how to prevent it.

ARDS (cont.)
In this Article

What is ARDS?

y y y y y y y y y y y y y

How do the lungs work? What are other names for ARDS? What causes ARDS? Who is at risk for ARDS? What are the signs and symptoms of ARDS? How is ARDS diagnosed? How is ARDS treated? Recovering from ARDS What are the complications of ARDS? Information for family and friends Key Points About ARDS ARDS Glossary ARDS Index

Who is At Risk for ARDS?

Acute respiratory distress syndrome (ARDS) usually affects people who are being treated for another serious illness or those who have had major injuries. It affects about 150,000 people each year in the United States. ARDS can occur in people with or without a previous lung disease. People who have a serious accident with a large blood loss are more likely to develop ARDS. However, only a small portion of people who have problems that can lead to ARDS actually develop it. In most cases, a person who develops ARDS is already in the hospital being treated for other medical problems. Some illnesses or injuries that can lead to ARDS include:

y y y y y y y y y

Serious, widespread infection in the body (sepsis) Severe injury (trauma) and shock from a car crash, fire, or other cause Severe bleeding that requires blood transfusions Severe pneumonia (infection of the lungs) Breathing in vomited stomach contents Breathing in smoke or harmful gases and fumes Injury to the chest from trauma (such as a car accident) that causes bruising of the lungs Nearly drowning Some drug overdoses

What Are the Signs and Symptoms of ARDS?

The major signs and symptoms of acute respiratory distress syndrome (ARDS) are:

y y y y

Shortness of breath Fast, labored breathing A bluish skin color (due to a low level of oxygen in the blood) A lower amount of oxygen in the blood Doctors and other health care providers watch for these signs and symptoms in patients who have conditions that might lead to ARDS. People who develop ARDS may be too sick to complain about having trouble breathing or other related symptoms. If a patient shows signs of developing ARDS, doctors will do tests to confirm that ARDS is the problem. ARDS is often associated with the failure of other organs and body systems, including the liver, kidneys, and the immune system. Multiple organ failure often leads to death.

ARDS can be caused by any major swelling (inflammation) or injury to the lung. Some common causes include: y y y y y Breathing vomit into the lungs (aspiration) Inhaling chemicals Pneumonia Septic shock Trauma

ARDS leads to a buildup of fluid in the air sacs. This fluid prevents enough oxygen from passing into the bloodstream. The fluid buildup also makes the lungs heavy and stiff, and decreases the lungs' ability to expand. The level of oxygen in the blood can stay dangerously low, even if the person receives oxygen from a breathing machine (mechanical ventilator) through a breathing tube (endotracheal tube). ARDS often occurs along with the failure of other organ systems, such as the liver or the kidneys. Cigarette smoking and heavy alcohol use may be risk factors. Symptoms y y y Labored, rapid breathing Low blood pressure and organ failure Shortness of breath

Symptoms usually develop within 24 to 48 hours of the original injury or illness. Often, people with ARDS are so sick they are unable to complain of symptoms.

Exams and Tests Listening to the chest with a stethoscope (auscultation) reveals abnormal breath sounds, such as crackles that suggest fluid in the lungs. Often the blood pressure is low. Cyanosis (blue skin, lips, and nails caused by lack of oxygen to the tissues) is often seen. Tests used to diagnose ARDS include: y y y y y y Arterial blood gas Bronchoscopy CBC and blood chemistries Chest x-ray Sputum cultures and analysis Tests for possible infections

Occasionally an echocardiogram or Swan-Ganz catheterization may need to be done to rule out congestive heart failure, which can look similar to ARDS on a chest x-ray. Treatment Typically people with ARDS need to be in an intensive care unit (ICU). The goal of treatment is to provide breathing support and treat the underlying cause of ARDS. This may involve medications to treat infections, reduce inflammation, and remove fluid from the lungs. A breathing machine is used to deliver high doses of oxygen and a continuous level of pressure called PEEP (positive end-expiratory pressure) to the damaged lungs. Patients often need to be deeply sedated with medications when using this equipment. Treatment continues until you are well enough to breathe on your own. Support Groups Many family members of people with ARDS are under extreme stress. Often they can relieve this stress by joining support groups where members share common experiences and problems. See also: Lung disease - support group Outlook (Prognosis) About a third of people with ARDS die from the disease. Survivors usually get back normal lung function, but many people have permanent, usually mild, lung damage. Many people who survive ARDS have memory loss or other problems with thinking after they recover. This is due to brain damage that occurred when the lungs weren't working properly and the brain wasn't getting enough oxygen. Possible Complications y y y y Lung damage (such as pneumothorax) due to use of high settings on the breathing machine needed to treat the disease Multiple organ system failure Pulmonary fibrosis Ventilator-associated pneumonia

When to Contact a Medical Professional Usually, ARDS occurs during another illness, for which the patient is already in the hospital. Occasionally, a healthy person may develop severe pneumonia that gets worse and becomes ARDS. If you have trouble breathing, call your local emergency number (such as 911) or go to the emergency room. Alternative Names Noncardiogenic pulmonary edema; Increased-permeability pulmonary edema; Stiff lung; Shock lung; ARDS; Acute lung injury