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Indian childhood cirrhosis is more common in the age group of 6 month to 4 years.
It is more affected in male child than female child (1 : 4), the first born is at greater risk.
Among twins, the member of the pair on mixed or artificial feeding in predisposed families was
reported to have developed cirrhosis of liver. If the other twin was purely breast-fed for first six
months, he or she escaped the illness.
A definite family predisposition is the hallmark of ICC. Siblings and twins are affected. An
increased prevalence of peptic ulcer, asthma, diabetes and migrane in the pedigrees affected by ICC
has been observed. .
A large majority of cases belongs to middle class families.
Majority of the patients have vegetarian dietetic background.
Recently, a significant decline in the incidence of Indian Childhood Cirrhosis has been observed in all parts
of India since the use of copper and brass utensils for boiling milk is reduced.
ETIOPATHOGENESIS
ICC continues to be a disease of obscure etiology. The following factors may predispose to the illness:
Toxic (copper intoxication): Studies reported that there is an evidence of excess of copper binding
proteins (orcein) in the liver of patient with ICC.
if the baby is weaned earlier and if the milk supplements were added to the breast milk
often before 3 months of age.
use of copper or copper alloy pots for boiling milk and cooking food. During boiling of
milk, copper is released from the copper pots and this is probably absorbed in excess from
the gut by infant.
Viral infection of the liver: It is a consequence of neonatal or infective hepatitis.
To conclude, no single factor seems to be the cause of ICC. It is possible that a genetically prone child
suffers from one or more of the superadded factors (viral, toxic, metabolic and autoimmune) leading to the
overt picture of ICC.
PATHOPHYSIOLOGY
ICC manifests with jaundice, pruritus, lethargy, and hepatosplenomegaly. Histologically, it is
characterized by hepatocyte necrosis, Mallory bodies, intralobular fibrosis, and inflammation. There is an
increased hepatic copper content, usually >700microgram per gram dry weight.
CLINICAL FEATURES
The onset is generally vague and ill defined, ranging from no symptoms to an icteric onset. Some
infants show pre cirrhotic symptoms.
Two modes of presentation are known: a) Insidious which occurs in a large majority of the cases
and b) Acute which is less common.
Insidious onset: In this, the disease will last for 6 months to 3years. Symptoms are grouped under 2
headings.
Pre-cirrhotic symptoms
Cirrhotic symptoms
Irritability
Cirrhotic symptoms are grouped under 3 stages:
Stage I
Disturbed appetite
Chalky, pasty stools and
Slight fever
Liver is enlarged to 3-5cm, edges become sharp
distension of abdomen.
Constipation or diarrhea
and giving an appearance of leafy boarder.
Often slight irregular
Children exhibits jaundice
Poor growth
fever.
Anorexia
Constipation/ diarrhea.
Clay colored stools.
Growth failure
Stage II
Diffuse hepatomegaly
Splenomegaly
Ascites
Esophageal varices
Hematemesis
Anemia
Muscle weakness
Lethargy
GI bleeding.
Stage III: It is the terminal stage of the disease
Restlessness
Confusion
Dyspnea and cyanosis on exertion.
Evidences of hepatocellular failure in the form of
palmar erythema and spider nevi appearance on
the upper torso
A peculiar garlic odor is present in patients with
impending liver cell failure.
Enlarged and hard spleen
Terminally, there is jaundice and hepatic coma
and is often associated with gastro intestinal
bleeding. Child may die at this stage either from
hepatic failure or intercurrent infections.
DIAGNOSTIC EVALUATION
1. History collection
2. Complete physical examination:
Liver can be palpable, very firm in consistency and its boarders will be sharp.
3.
4.
5.
6.
On auscultation hepatic bruit in severe cases. If there is ascites, fluid thrill test can be done.
Liver function test:
increased ALT(alanine transaminase, an enzyme present in hepatocytes.)
increased GGT( gama glutamyl transpeptidase)
Prothrombin time, clotting time and bleeding time should be assessed. PT will be prolonged
Liver biopsy: to find out the sclerosis of liver. It is a reliable method of arriving at a foolproof of
diagnosis.
Cupriuresis: testing the presence of copper in urine after administration of d-penicillamine.
TREATMENT
Until recently, ICC was dubbed as a frustrating situation for which no specific treatment was available. If
the diagnosis is made at an early stage (before the development of jaundice and ascites), ICC is potentially
treated.
1. Initial stage:
Adequate diet with enough of good quality proteins, vitamins and minerals is desirable.
Antibiotics should be given to treat the intercurrent infections / infestations.
The drug of choice is d- pencillamine (which chelate copper) in a dose of 20- 40 mg/kg/day
for 12 to 18 months, leads to marked improvement and even total reversal in the
histopathologic picture.
Symptomatic treatment should be given.
Immuno modulators such as levamisole can be used
Corticosteroids and gammaglobulins are also helpful.
administer IV fluids if there is dehydration
Prevention of infection: follow aseptic techniques
Prophylactic antibiotics can be given to prevent infection
2. Terminal stage:
If the patient has entered precoma or coma, the protein intake should be reduced.
administration of neomycin by gavage and 20% IV glucose drip are helpful.
oxygen can be administered if necessary.
exchange transfusion to remove the circulating toxins.
SURGICAL MANAGEMENT
No specific surgical correction. The only successful treatment for end stage liver disease is liver
transplantation.
If there is portal hypertension with hematemesis, Sengstaken tube may help to control the
esophageal bleed.
A portocaval anastamosis may be done to relieve the portal hypertention and complications of
hypersplenism
NURSING MANAGEMENT
Nursing diagnosis
1.
2.
3.
4.
5.
WILSON DISEASE
INTRODUCTION
Wilsons disease is an inborn error of metabolism due to toxic accumulation of copper in liver,
brain, cornea and other tissues which manifest as neurological or psychiatric symptoms.
Wilson disease (WD), also known as hepatolenticular degeneration, was first described by
American neurologist, Kinnear Wilson, in 1912.
DEFINITION
Wilson disease (hepatolenticular degeneration) is an autosomal recessive disorder that can be
associated with degenerative changes in the brain, liver disease, and Kayser-Fleischer rings in the cornea.
EPIDEMIOLOGY
The incidence is 1/50,000 to 1/100,000 births. It is progressive and potentially fatal if untreated;
specific effective treatment is available.
WD may present with hepatic, hematologic, neurologic, or psychiatric symptoms. WD presents as
liver disease more commonly in children and younger adults than older adults. Its most typical
presentation is that of hepatic or hematologic symptoms in the second decade of life. Neurologic
and psychiatric presentations are more common in the third and fourth decades.
PATHOGENESIS
Copper is an essential metal which acts as a
cofactor for many proteins. In normal human copper
metabolism, the dietary intake of copper exceeds
physiologic needs and is therefore excreted. Copper is
absorbed in the stomach and proximal small intestine
relatively efficiently; therefore, to avoid copper toxicity,
the amount of copper absorbed and stored in the body
must be finely regulated. The main system for achieving
such regulation is the hepatic excretion of copper into
bile. Up to 80% of absorbed copper is actually excreted
in bile to maintain this homeostasis.
copper within hepatocytes. In WD, the absent or reduced function of this ATPase results in the decreased
hepatocellular excretion of copper into bile and defective incorporation of copper into ceruloplasmin. This
in turn leads to the progressive accumulation of copper in the liver, and subsequent hepatic injury. Over
time, copper may be released into the bloodstream and deposited in secondary organs, such as the brain,
cornea, and kidneys. Clinical disease therefore may range over a variable spectrum including abnormal
liver function tests to fulminant hepatic failure and cirrhosis, to seizures, psychosis, and other neurologic
manifestations.
CLINICAL MANIFESTATIONS
Disease presentations are variable. The younger the patient, the more likely hepatic involvement
will be the predominant manifestation. Girls are 3 times more likely than boys to present with acute hepatic
failure. After 20 yr of age, neurologic symptoms predominate. Clinical symptoms of liver involvement in
WD typically do not occur prior to 35 years of life.
Patients less than 20 years old tend to present with liver dysfunction whereas older patients
typically present with psychiatric and neurologic manifestations.
Forms of Wilsonian hepatic disease include asymptomatic hepatomegaly (with or without splenomegaly),
subacute or chronic hepatitis, and acute hepatic failure (with or without hemolytic anemia).
From a hepatic perspective, signs and symptoms may include:
changes in behavior
deterioration in school performance
movement disorders
lack of motor coordination
rigid dystonia
dysarthria
drooling
seizures
migraine
headaches
anxiety
depression
insomnia,
personality changes, and/or psychosis
Kayser-Fleischer rings may be absent in young patients with liver disease but are always present in
patients with neurologic symptoms.
Menstrual irregularities (e.g. amenorrhea, infertility),
Coombs-negative hemolytic anemia (possibly related to the release of large amounts of copper
from damaged hepatocytes)
Copper deposition in the kidneys may lead to nephrocalcinosis, hematuria, or aminoaciduria.
Cardiac manifestations may include cardiomyopathy and arrhythmias.
Unusual manifestations include arthritis, infertility or recurrent miscarriages and endocrinopathies
(hypoparathyroidism).
DIAGNOSIS
Wilson disease should be considered in children and teenagers with unexplained acute or chronic
liver disease, neurologic symptoms of unknown cause, acute hemolysis, psychiatric illnesses,
behavioral changes, Fanconi syndrome, or unexplained bone (osteoporosis, fractures) or muscle
disease (myopathy, arthralgia).
Serum ceruloplasmin: Most patients with Wilson disease have decreased ceruloplasmin levels ( <
20 mg/dL).
Serum copper level: may be elevated in early Wilson disease
Urinary copper excretion: Urinary copper excretion (usually < 40 g/day) is increased to > 100
g/day and often up to 1,000 g or more per day.
Response of urinary copper output to chelation: Estimation of urinary copper after a dpenicillamine challenge (During the 24 hr urine collection patients are given two 500 mg oral doses
of d penicillamine 12 hr apart) help differentiate WD from other causes of raised urinary copper;
excretion >1,600 g/24 hr. is characteristic of WD.
Demonstration of Kayser-Fleischer rings, which might not be present in younger children, requires
a slit-lamp examination by an ophthalmologist.
Liver biopsy is of value for determining the extent and severity of liver disease and for measuring
the hepatic copper content (normally < 10 g/g dry weight). In Wilson disease, hepatic copper
content exceeds 250 g/g dry weight. In later stages of Wilson disease hepatic copper content can
be unreliable because cirrhosis leads to variable hepatic copper distribution and sampling error.
TREATMENT
WD is fatal without treatment. Treatment modalities for WD include diet modification, chelation,
zinc, antioxidants, tetrathiomolybdate, and liver transplantation. Choice of specific therapy is dependent
upon clinical presentation.
Dietary modification
Dietary modification with restriction of consumed copper is nearly universally recommended for
WD patients for at least their first year of therapy.
A major attempt should be made to restrict dietary copper intake to < 1 mg/day. Foods such
as liver, shellfish, nuts, mushroom and chocolate should be avoided. If the copper content of
the drinking water exceeds 0.1 mg/L, it may be necessary to demineralize the water.
Copper-chelating agents
Copper chelation is the mainstay of treatment, which leads to rapid excretion of excess deposited
copper. Currently, D-penicillamine and trientine are the typical chelation medications utilized in treating
WD.
triethylene tetramine dihydrochloride at a dose of 0.5-2.0 g/day for adults and 20 mg/kg/day
for children.
In response to chelation, urinary copper excretion markedly increases, and with continued
administration, urinary copper levels can become normal, with marked improvement in hepatic and
neurologic function and the disappearance of Kayser-Fleischer rings.
Pyridoxine (25 mg/ day) should be co-administered with both D-penicillamine and trientine.
Severe adverse reactions can occur and have been reported to occur at a frequency of approximately 30%
of patients treated with D-penicillamine and 5% of patients treated with trientine. These reactions may
include hypersensitivity reactions, thrombocytopenia, neutropenia, proteinuria, and autoimmune diseases.
Efficacy of treatment is determined by following 24-hour urine copper measurements that is followed
every 3 months for the first 2 years and then annually. Drug toxicity needs to be followed closely with a
complete blood count, hepatic panel, creatinine, and urinalysis serially.
Ammonium tetrathiomolybdate is another alternative chelating agent under investigation for patients
with neurologic disease; initial results suggest that significantly fewer patients experience neurologic
deterioration with this drug compared to penicillamine. The initial dose is 120 mg/day (20 mg between
meals tid and 20 mg with meals tid). Side effects include anemia, leukopenia, thrombocytopenia, and mild
elevations of transaminases.
Zinc therapy
Zinc has also been used as adjuvant therapy, maintenance therapy, or primary therapy in
presymptomatic patients, owing to its unique ability to impair the gastrointestinal absorption of copper.
Zinc acetate is given in adults at a dose of 25-50 mg of elemental zinc 3 times a day, and 25 mg 3
times a day in children > 5 yr of age.
Side effects are mostly limited to gastric upset.
Liver transplantation
In hepatic failure, liver transplantation is the most appropriate therapy. Plasmapheresis or kidney
dialysis may be used as temporizing measures to bridge to transplant. It is also indicated in the absence of
liver failure in patients with neurological WD in whom chelation therapy has proved ineffective.
SCREENING FOR WD
Screening for WD in asymptomatic relatives should begin at 3 years of age. This screen should
include a history and physical examination, hepatic function panel, prothrombin time and INR, slit lamp
examination by a pediatric ophthalmologist, 24-hour urine copper, and consideration of genetic testing.
PROGNOSIS
Untreated patients with Wilson disease can die of hepatic, neurologic, renal, or hematologic
complications. The prognosis for patients receiving prompt and continuous penicillamine is variable and
depends on the time of initiation of and the individual response to chelation. Liver transplantation should
be considered for patients with fulminant liver disease, decompensated cirrhosis, or progressive neurologic
disease; the last indication remains controversial. Liver transplantation is curative, with a survival rate of
85-90%. In asymptomatic siblings of affected patients, early institution of chelation or zinc therapy can
prevent expression of the disease.
REYE SYNDROME
INTRODUCTION
Reye syndrome is characterized by acute non-inflammatory encephalopathy and fatty degenerative
liver failure. The syndrome was first described in 1963 in Australia by RDK Reye and described a few
months later in the United States by GM Johnson. Reye syndrome typically occurs after a viral illness,
particularly an upper respratory tract infection, influenza, varicella, or gastroenteritis, and is associated
with the use of aspirin during the illness. A dramatic decrease in the use of aspirin among children, in
combination with the identification of medication reactions, toxins, and inborn errors of metabolism
(IEMs) that present with Reye syndromelike manifestations, have made the diagnosis of Reye syndrome
exceedingly rare.
DEFINITION
Reye syndrome is a disorder defined as acute encephalopathy associated with other characteristic
organ involvement. It is characterized by fever, profoundly impaired consciousness, and disordered hepatic
function.
ETIOLOGY
The etiology of RS is not well understood, but most cases follow a common viral illness, typically
influenza or varicella. Etiological factors include:
Pathogens
Influenza virus types A and B and varicella-zoster virus are the pathogens most commonly
associated with Reye syndrome. Other pathogens include parainfluenza virus, adenovirus, coxsackievirus,
measles, cytomegalovirus, Epstein-Barr virus, HIV, retrovirus, hepatitis virus types A and B, mycoplasma,
chlamydia, pertussis, shigella, and salmonella.
Salicylates
The association of Reye syndrome with salicylates, particularly aspirin, was demonstrated in
several epidemiologic studies around the world. Less than 0.1% of children who took aspirin developed
Reye syndrome, but more than 80% of patients diagnosed with Reye syndrome had taken aspirin in the past
3 weeks. Recommendations by government health agencies that children not be treated with salicylates led
to an immediate and dramatic decrease in the incidence of Reye syndrome.
Other agents
Acetaminophen, outdated tetracycline, valproic acid, warfarin, zidovudine didanosine, and some
neoplastic drugs have been associated with Reye syndrome or Reye-like syndrome. Nonsteroidal antiinflammatory drugs, including sodium diclofenac and mefenamic acid, are thought to produce or worsen
Reye syndrome. An association with antiemetics, such as phenothiazines, has been postulated but not
substantiated.
Reye syndrome or Reye-like syndrome may also be associated with insecticides; herbicides;
isopropyl alcohol; paint; paint thinner; hepatotoxic mushrooms etc
Inborn errors of metabolism
IEMs that produce Reye-like syndromes include fatty-acid oxidation defects, particularly mediumchain acyl dehydrogenase (MCAD) and long-chain acyl dehydrogenase deficiency (LCAD) inherited and
acquired forms, urea-cycle defects, amino and organic acidopathies, primary carnitine deficiency, and
disorders of carbohydrate metabolism.
EPIDEMIOLOGY
Incidence peaks between age 5 and 14 years; 13.5% were younger than 1 year.
Reye syndrome rarely occurs in newborns or in children older than 18 years.
Reye syndrome is equally distributed between the sexes.
PATHOPHYSIOLOGY
RS is a condition characterized pathologically by cerebral edema and fatty changes of the liver. The
onset of RS is notable for profuse effortless vomiting and varying degrees of neurologic impairment,
including personality changes, seizures, and coma, that lead to increase ICP, herniation, and death
The pathogenesis of Reye syndrome, while not precisely elucidated, appears to involve
mitochondrial injury (induced by various viruses, drugs, exogenous toxins, and genetic factors) resulting
in dysfunction that inhibits oxidative phosphorylation and fatty-acid beta-oxidation in a virus-infected,
sensitized host. The host has usually been exposed to mitochondrial toxins, most commonly salicylates
(>80% of cases).
Histologic changes include cytoplasmic fatty vacuolization in hepatocytes, astrocyte edema and loss
of neurons in the brain, and edema and fatty degeneration of the proximal lobules in the kidneys. All cells
have pleomorphic, swollen mitochondria that are reduced in number, along with glycogen depletion and
minimal tissue inflammation. Hepatic mitochondrial dysfunction results in hyperammonemia (elevated
serum ammonia), which is thought to induce astrocyte edema, resulting in cerebral edema and increased
intracranial pressure (ICP).
CLINICAL PRESENTATION
History:
Most patients had at least 1 viral illness (viral upper respiratory illness or influenza, varicella,
gastroenteritis) in the 3 weeks preceding the onset of Reye syndrome.
Salicylates were detectable in the blood of 82% of patients.
Reye syndrome can occur after vaccination with live viral vaccines.
Physical Examination
Signs and symptoms of Reye syndrome include
protracted vomiting, with or without clinically significant
dehydration; hepatomegaly in 50%; minimal or absent
jaundice; and lethargy progressing to encephalopathy,
Persistent vomiting
Diarrhea
Lethargy
Restlessness and irritability
Hydrocephalus
Hyperammonimia
Hyperglycemia
Stage 0
Stage 1
Stage 2
Stage 3
Stage 4
Stage 5
Stage 6
HURWITZ CLASSIFICATION
Alert, abnormal history and laboratory findings consistent with Reye
syndrome, and no clinical manifestations
Vomiting, sleepiness, and lethargy
Restlessness, irritability, combativeness, disorientation, delirium,
tachycardia, hyperventilation, dilated pupils with sluggish response,
hyperreflexia, positive Babinski sign, and appropriate response to noxious
stimuli
Obtunded, comatose, decorticate rigidity, and inappropriate response to
noxious stimuli
Deep coma, decerebrate rigidity, fixed and dilated pupils, loss of
oculovestibular reflexes, and dysconjugate gaze with caloric stimulation
Seizures, flaccid paralysis, absent deep tendon reflexes (DTRs), no pupillary
response, and respiratory arrest
Patients who cannot be classified because they have been treated with curare
or another medication that alters the level of consciousness
DIAGNOSTIC CRITERIA
Acute
non-inflammatory
encephalopathy with an altered level
of consciousness
DIAGNOSIS
Hepatic dysfunction with a liver
Workup to exclude inborn errors of metabolism (IEMs) mustbiopsy
be performed
include
showingand
fattyshould
metamorphosis
evaluation for defects of fatty-acid oxidation, amino and organic
acidurias,
urea-cycleordefects,
without
inflammation
necrosisand
or a
disorders of carbohydrate metabolism.
greater than 3-fold increase in alanine
Computed tomography (CT) of the head may reveal cerebral aminotransferase
edema, but the results
are usually
(ALT),
aspartate
normal.
aminotransferase (AST), or ammonia
Electroencephalography (EEG) may reveal slow-wave activity in the early stages and flattened
levels
waves in advanced stages.
No other explanation for cerebral
MRI characteristics of Reye syndrome are symmetric thalamic, white matter and basal ganglia
edema or hepatic abnormality
lesions, in children with recent history of salycilates or immunosuppressive
drugs
intake.
Cerebrospinal
fluid
(CSF) with a
white blood cell (WBC) count of 8
Laboratory Studies
cells/L
or
fewer
(usually
lymphocytes); note that lumbar
An ammonia level as high as 1.5 times normal 24-48 hours
puncture should not be performed in
after the onset of mental status changes is the most frequent
patients who are hemodynamically
laboratory abnormality. Ammonia tends to peak 56-60
unstable and/or those in whom
increased intracranial pressure (ICP)
is a concern
Brain biopsy with findings of
cerebral edema without inflammation
Invasive Procedures
The following procedures may be helpful in treatment, workup, and monitoring:
Vascular access (arterial, central venous, or both)
Lumbar puncture if the patient is hemodynamically stable and shows no signs of increased
intracranial pressure (ICP) Opening pressure may or may not be increased; the white blood cell
(WBC) count in the cerebrospinal fluid (CSF) is 8/L or fewer
Intubation to maintain airway and ventilation and to manage ICP
Nasogastric tube placement to decompress the abdomen
Bladder catheterization to monitor urine output
Percutaneous liver biopsy (if indicated) to exclude an IEM or toxic liver disease
Placement of an intracranial device for ICP monitoring in patients with increased ICP
TREATMENT
No specific treatment exists for Reye syndrome; supportive care is based on the stage of the
syndrome.
Continue careful monitoring.
Establish and maintain the patients airway, breathing, and circulation.
Check the glucose level, particularly if the patient is younger than 1 year and/or has altered mental
status. Administer dextrose to correct hypoglycemia.
Admission to the intensive care unit (ICU) is warranted for continued monitoring and treatment.
Consider consultation with a neurologist for electroencephalography (EEG).
Consider consultation with a neurosurgeon for monitoring and treatment of increased intracranial
pressure (ICP).
Consider consultation with a gastroenterologist or surgeon for liver biopsy.
Consider consultation with a metabolic disease specialist if an inborn error of metabolism (IEM) is
a possibility.
Monitor and treat long-term neurologic sequelae. Prescribe outpatient anticonvulsants if ongoing
seizures occur.
Stage-Specific Management
Supportive care is based on the clinical stage of the syndrome, with aggressive treatment provided
to correct or prevent metabolic abnormalities, particularly hypoglycemia and hyperammonemia, and to
prevent or control cerebral edema.
Stages 0-1
Keep the patient quiet.
Frequently monitor vital signs and laboratory values.
Correct fluid and electrolyte abnormalities, hypoglycemia, and acidosis.
Stage 2
The standard of care consists of continuous cardiorespiratory monitoring, placement of central
venous lines or arterial lines to monitor hemodynamic status, urine catheters to monitor urine
output, ECG to monitor cardiac function, and EEG to monitor seizure activity.
Endotracheal intubation may be required at this stage to maintain the airway, control ventilation,
and prevent increased ICP. Use rapid-sequence agents that minimize the chance of increasing ICP.
Place a nasogastric tube to decompress the abdomen.
Hyperammonemia can contribute to cerebral edema and therefore must be corrected aggressively.
sodium phenylacetatesodium benzoate is FDA-approved for the treatment of acute
hyperammonemia and associated encephalopathy in patients with deficiencies in enzymes of the
urea cycle.
Administer ondansetron 1-2 mg IV during the first 15 minutes of the initial dose of sodium
phenylacetatesodium benzoate. If the ammonia level is higher than 500 g/dL or if the patients
condition fails to respond to the initial dose of sodium phenylacetatesodium benzoate, start
dialysis, preferably hemodialysis.
Prevent increased ICP. Elevate the head to 30, keep the head in a midline orientation, use isotonic
rather than hypotonic fluids, avoid overhydration, and administer furosemide 1 mg/kg as often as
every 4-6 hours to control fluid overload.
Stages 3-5
Continuously monitor ICP, central venous pressure, arterial pressure, or end-tidal carbon dioxide.
Perform endotracheal intubation if the patient is not already intubated.
as
possible
to
eliminate
PREVENTION
Salicylates should be avoided in children, except in those who have conditions for which salicylates
are a mainstay of therapy (eg, Kawasaki disease).
It is critical to be alert for and recognize early symptoms of Reye syndrome. It is also important to
be mindful of the possibility that an IEM may be the actual cause of the symptoms and, if this is the
case, to be prepared to treat the IEM. Appropriate management of IEMs dramatically decreases
morbidity and mortality.
Influenza vaccine is recommended by the Centers for Disease Control and Prevention (CDC) for
everyone older than 6 months.
PROGNOSIS
Recovery from RS is rapid and usually without sequelae if the diagnosis is determined early and
therapy is initiated promptly. Patients who survive have full liver function recovery; however,
approximately one third may have subtle neuropsychological deficits.
NURSING CARE MANAGEMENT
Care and observations are implemented as for any child with an altered state of consciousness and
increasing ICP.
Potential Nursing Diagnoses
Families need to be aware that salicylate, the alleged offending ingredient in aspirin, is
contained in other products. They should refrain from administering any product for
influenza-like symptoms without first checking the label for hidden salicylates.
Nursing Outcomes
RESEARCH STUDIES
Role of copper in Indian childhood cirrhosis.
Tanner MS1.
Author information
Abstract
Of the cirrhoses that affect Indian children, Indian childhood cirrhosis (ICC) is a discrete clinical and
histologic entity in which large amounts of copper are deposited in the liver. The evidence linking copper
deposition to increased dietary copper intake in infancy was reviewed. Prevention of this feeding pattern
prevents ICC, and the disease has now largely disappeared from many parts of India. Penicillamine, if
given before the terminal clinical stage of ICC, reduces mortality from 92% to 53%. Long-term survivors
show a sequence of histologic resolution, resulting either in inactive micronodular cirrhosis or in virtually
normal histologic appearance. Twenty-nine treated ICC patients reexamined at 8.8 y of age (range: 6.3-13
y), 5-12 y after diagnosis, were well and had normal results from liver function tests. Clinical and
epidemiologic evidence show that there must be excessive copper ingestion for ICC to develop, but the
lack of an animal model, the inconstant relation between liver copper concentrations and liver damage, and
the rarity of liver disease in adults suggests that other etiologic factors contribute. Two mechanisms are
discussed: 1) that copper may be acting in synergy with a hepatotoxin, or 2) that there may be a genetic
predisposition to copper-associated liver damage, as suggested recently for Tyrollean childhood cirrhosis.
Although ICC is now rare, sporadic cases of an ICC-like disorder in infants continue to occur. There should
be a greater awareness among pediatricians of this disease to enable early diagnosis. Penicillamine should
be used early and adverse prognostic factors recognized as indications for early transplantation and
unregulated water supplies should not be used to prepare infant feeds
Department of Pathology, Global Health City, Chennai, Tamil Nadu 600100, India.
wususama@gmail.com
Abstract
OBJECTIVE:
Indian childhood cirrhosis (ICC), an unique liver disease that has been endemic in most parts of India and
advocated by some to be caused by hepatotoxic effect of excess dietary copper is generally believed now to
have virtually disappeared from the country. In the face of this the authors report here five cases of ICC
encountered in one children hospital over the last 10 y period.
METHODS:
Cases histologically categorized as ICC were initially picked up from the records of the department of
Pathology. Their clinical, investigational and follow up information retrieved from hospital data base along
with pathologic features of liver biopsies were reviewed in detail.
RESULTS:
The age range of the three male and two female children were from 1 to 12 y and on clinical and
investigational features all 5 cases were labeled as non-Wilsonian liver disease of uncertain etiology.
Histopathologic findings in each case however, was characteristic of accepted established phase of ICC.
Three of the five children died in hospital while the other two left the hospital and were lost to follow up.
None of the children had exposure to excess dietary copper.
CONCLUSIONS:
Cases of ICC continue to occur in Andhra Pradesh and very likely in other parts of India. Established and
non-typical cases are possibly being missed because of no histologic confirmation and unawareness of the
protean manifestation and natural history of this disease. Dietary copper overload is unlikely to play a
causal role in ICC.
Dara N1, Imanzadeh F1, Sayyari AA1, Nasri P1, Hosseini AH1.
Author information
Abstract
INTRODUCTION:
Coexistence of Wilson's disease and autoimmune hepatitis has been rarely reported in English literature. In
this group of patients, there exist features of both diseases and laboratory and histopathological studies may
be misleading. Medical treatment for any of these entities, per se, may result in poor response. Therefore,
by considering the acute hepatitis resembling Wilson's disease and autoimmune hepatitis, simultaneous
therapy with immunosuppressive and penicillamine may have a superior benefit.
CASE PRESENTATION:
We present the case of a 10-year-old boy with nausea, vomiting, yellowish discoloration of skin and sclera,
abdominal pain and tea-color urine. Physical examination showed mild hepatomegaly and right upper
quadrant tenderness. Laboratory and histochemical studies and atomic absorption test were done and the
results were highly suggestive of both Wilson's disease and autoimmune hepatitis, in him.
CONCLUSIONS:
This case study highlights, although rare, the coexistence of Wilson's disease and autoimmune hepatitis and
the need to maintain a high level of awareness of this problem. Therefore, it is reasonable to consider this
type of hepatitis in rare patients, with dominant features of both diseases at the same time.
Abstract
Reye syndrome is an extremely rare but severe and often fatal disease. Death occurs in about 30-40% of
cases from brainstem dysfunction. The disease typically is preceded by a viral infection with an
intermediate disease-free interval of 3-5 days. The biochemical explanation for Reye-like symptoms is a
generalized disturbance in mitochondrial metabolism, eventually resulting in metabolic failure in the liver
and other tissues. The etiology of 'classical' Reye syndrome is unknown. Hypothetically, the syndrome may
result from an unusual response to the preceding viral infection, which is determined by host genetic
factors but can be modified by a variety of exogenous agents. Thus, several infections and diseases might
present clinically with Reye-like symptoms. Exogenous agents involve a number of toxins, drugs
(including aspirin [acetylsalicylic acid]), and other chemicals. The 'rise and fall' in the incidence of Reye
syndrome is still poorly understood and unexplained. With a few exceptions, there were probably no new
Reye-like diseases reported during the last 10 years that could not be explained by an inherited disorder of
metabolism or a misdiagnosis. This may reflect scientific progress in the better understanding of cellular
and molecular dysfunctions as disease-determining factors. Alternatively, the immune response to and the
virulence of a virus might have changed by alteration of its genetic code. The suggestion of a defined
cause-effect relationship between aspirin intake and Reye syndrome in children is not supported by
sufficient facts. Clearly, no drug treatment is without side effects. Thus, a balanced view of whether
treatment with a certain drug is justified in terms of the benefit/risk ratio is always necessary. Aspirin is no
exception.
BIBLIOGRAPHY
1. Robert M. Kliegman, Stanton, NELSON TEXTBOOK OF PEDIATRICS, 19 th edn,2011, Elsevier
publishers, Philadelphia, page no: 1390-1395
2. John F. Pohl, Christopher Jolley, Pediatric Gastroenterology A Color Handbook,2014, CRC Press,
USA, Page no 342-350
3. Marilyn J Hockenberry, David Wilson. Wongs Nursing Care of infants and children. 9th edition.
Elsevier publishers.USA.2011
4. www.medscape.com
5. N.C. Nayak & A.R. Chitale, Indian childhood cirrhosis (ICC) & ICC-like diseases: The changing
scenario of facts versus notions, Indian J Med Res 137, June 2013, pp 1029-1042