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LECTURE: BRONCHITIS.

PNEUMONIA BRONCHIAL ASTHMA IN CHILDREN


DEPARTMENT OF PAEDIATRICS CHIEF: PROF. Y.V.PROHOROV LECTURER: ASOC.PR. E.A.TOLSTIKOVA

Bronchitis

is an inflammatory disease of bronchi of various etiology: infectious (mostly viral), allergic, physical and chemical (so-called irritative bronchitis). It may be acute or chronic. There are the following main forms of bronchitis: acute simple bronchitis, acute obstructive bronchitis, bronchiolitis, recurring bronchitis, chronic bronchitis (primary and secondary).

Criteria of acute bronchitis


/. Clinical: - cough - dry and rough at the beginning of disease, gradually becoming productive; - symptoms of intoxication are not expressed greatly and quickly disappear; - symptoms of respiratory insufficiency are absent; - physical signs: on percussion there is a slight tympanic resonance, on auscultation dry and various bubbling rales, heard on both sides of lungs.

Obstructive bronchitis
is a variant of acute bronchitis which proceeds with respiratory tract obstruction because of bronchospasm, mucous edema, hypersecretion and pressure from without. Signs of respiratory tract obstruction: persistent, "spastic" cough, expiratory dyspnea, oral crepitations, dry and various bubbling rales.

Obstructive bronchitis

//. X-ray: - strengthened lung figure, at the same time absence of focal shadow; - signs of disturbances of bronchial permeability: irregular pneumatisation of lungs (focus of hyper- and hypoventilation), lobular atelectasis. ///. Laboratory - hematological: normal leukocyte count or leukopoenia, lymphocytosis, monocytosis. Red blood cells are not changed. ESR is not increased. Differentiatial diagnosis of acute bronchitis is associated with acute bronchiolitis, pneumonia; obstructive bronchitis - with bronchial asthma paroxysm.

Acute bronchiolitis

is mainly a disease of the first months of life. It is attributed mostly to the respiratory syncytial virus, although many other viruses may lead to a similar disease. It is characterized by obstructive respiratory insufficiency and cyclic course.

Criteria of bronchiolitis

/. Clinical: - respiratory insufficiency of obstructive type: cyanosis of nasolabial triangle, expiratory dyspnea with retraction of flexible parts of breast. spastic cough. At the peak of the disease cough is paroxysmal, in productive with scanty viscous sputum (for infants of the first months of the life asphyxial paroxysms are possible); - physical findings: high tympanic resonance on percussion, on auscultation - prolonged breathing out, on inspiration a lot of fine bubbling rales, crepitation on the both sides of lungs; - cyclic course: the disease continues for 6-8 days, and to 8-10,h day it may be completly recovered.

Criteria of bronchiolitis

//. X-ray: - distended lungs and diminished vascular picture. Then intensification of vascular picture as lungs distention is decreasing. ///. Laboratory - normal or decreased white blood cells count, lymphocytosis. ESR is normal or slightly increased. Differential diagnosis is provided with pneumonia and acute bronchitis.

Recurrent bronchitis

Recurrent bronchitis is the disease with relapsing of acute bronchitis 2 and more times a year during 1-2 years. The absence of clinical obstruction and duration of clinical manifestation for 2 weeks and longer every relapse are common. Phases of pathologic process: exacerbation, remission. Chronic bronchitis is a chronic spread inflammatory damage of bronchi with rebuilding of mucous secretory apparatus and sclerotic degeneration of deep layers of bronchial wall. Phases of pathological process: exacerbation, remission.

Criteria of chronic bronchitis


Clinical: - productive cough for several months during 2 years; - permanent various rales; - 2-3 relapses in a year during 2 years; - the signs of lungs ventilation disturbances in remission phase. Note: none of the above mentioned signs may be regarded alone as reliable evidence of chronic bronchitis. The sings must be considered in complex in view of possible development of chronic process. //. X-ray, especially bronchography: - increasing and deformity of lungs figure, the disturbances of the lungs hilus' structure. ///. Bronchoscopy gives information about the character of endobronchitis. It is of great value for differential diagnosis. Primary chronic bronchitis is diagnosed after exclusion of cystic fibrosis, bronchial asthma, lungs and cardiovascular malformations, ciliary dysgenesis.

Chronic bronchitis

Secondary chronic bronchitis - is complication of lungs and cardiovascular malformations, cystic fibrosis, hereditary diseases of lungs and a specific (tuberculosis) bronchial and pulmonary process. Chronic obliterating bronchiolitis (COB) - is chronic inflammatory bronchial disease of viral or immunopathological origin which appears as a result of bronchi or bronchioles obliteration of one or several lungs lobes. It leads to lungs blood circulation disorders and development of emphysema. Phases of pathologic process: relapse, remission. Forms of COB: total one-sided, focal one-sided, focal two-sided, partial.

Criteria of chronic obliterating bronchiolitis

Clinical: - severe respiratory infection with obstructive syndrome in case history; - persistent fine bubbling rales under the weakened breathing. //. X-ray: - one-sided weakening of lung figure, lessening of the lung field size on bronchogramme - unfilling by contrast of bronchi of 5-6th level and lower; prominent decreasing of lung perfusion in zones of pathologic process.

Pneumonias

Pneumonia is an acute infectious inflammatory disease of various nature with involving of respiratory organs into pathologic process and intra-alveolar inflammatory exudation. Classification of pneumonias Groups of pneumonias: 1. Primary 2. Secondary. - In primary pneumonia pathologic process at first develops in lungs tissue and is considered as a basic disease. - In secondary pneumonia pathologic process complicates the course of another pulmonary disease or diseases of other organs (the same concerns the chronic pneumonia).

Classification of pneumonias :

Forms of pneumonias : - focal (focal confluent); - segmental ( mono- or polysegmental); - croupous; - interstitial; Localization of process: lung, lobe, segment, one-sided, double (two sided). Course of pneumonia: - acute; - - lingering (reverse development of pneumonia process not earlier than 6 weeks - 8 months after beginning of disease); - recurrent (under exclusion of reinfection).

Classification of pneumonias : (continuing)


Variety of pneumonias : Out-of-hospital ("home"); Hospital (nosocomial); under perinatal infection - aspirative one; in patients with immune deficiency. The type of infectious agent is connected with the variant of pneumonia : in out-of-hospital pneumonias gram-positive cocci are predominant; in hospital pneumonias - Staphylococci and Gram-negative microflora, in newborns - Chlamidias, Pneumocystas, Ureaplasmas, in aspirative pneumonia - obligate anaerobes combined with Gram-negative bacilli; in case of prominent immune disorders - Pneumocystas, Fungi, Cytomegaloviruses.

Complications

Pulmonary complications: - Synpneumonial pleurisy; - methapneumonial pleurisy; - pulmonary destruction; - pneumothorax, etc. Extrapulmonary complications: - infectious - toxic shock; - DIG - syndrome; - cardiovascular insufficiency; - respiratory distress syndrome of adult type.

Criteria of pneumonia

1. Anamnestic data: - connections with respiratory viral infections; common cold. 2. Clinical: - Syndrome of respiratory insufficiency: - dyspnea, - participation of axillary muscles in breathing act, retraction of flexible parts of thorax, pallor of skin, cyanosis of nasolabial triangle or spread cyanosis, increasing after physical exertion; - Syndrome of intoxication: - worsening of feeling, sleep disturbances, decreasing of appetite, flaccidity, adynamia or hyperexcitability, motor anxiety, hyperthermia, muffled heart sounds, tachycardia. For the infants of the first two months of life marked signs of intoxication are flaccidity, decreasing of appetite down to refusing of breast feeding, weight fall, decreasing of physiological reflexes, appearance of gastrointestinal disorders, development of exicosis; - Focal signs in lungs - they are prominent in segmental (polysegmental) and affluent pneumonias, less marked in focal pneumonias and minimal changes are supervised in interstitial pneumonias: a) on percussion - shortening of resonance on the place of lesion; b) on auscultation - harsh, bronchial or weakened bronchial breathing on the place of lesion; c) fine bubbling rales or crepitation rales above the infiltrative foci.

Criteria of pneumonia (continuing)


3. X-ray: infiltrative changes of lungs of focal or segmental type and reaction of lung's hylus on the side of lesion. Such signs as emphysema, strengthening of hylus' figure (perivascular and peribronchial shadowings) may be supervised either in bronchitis and bronchiolitis or in pneumonia. 4. Laboratory: Blood cell count shows the activity of bacterial inflammation: increasing of ESR, leucocytosis, neutrophilia with shift to the left. In infants of the first months of life it is often seen lymphocyte reaction, early developed anemisation. Differential diagnosis is provided with acute respiratory viral infections, bronchiolitis. In lingering course of pneumonia -with primary tuberculosis, cystic fibrosis.

Bronchiectasis

- congenital or acquired disease of progressing affection of bronchi, which is characterized as infectious inflammatory chronic process which leads to deformation of bronchi, namely their dilatation and disturbances of drainage function, blood and lymph circulation. Some authors regard bronchiectasis combined with pneumosclerosis as "Chronic pneumonia". Thus in various editions we meet the description of the same disease as a "Bronchiectasias" or "Chronic pneumonia". Forms of disease: slight, moderate or severe. Periods of disease: relapse, remission. Localization - one-sided, two-sided, restricted (segment, lobe) and spread, with indication of localization and type of endobronchitis. Types of bronchiectasis: saccular (cyst-like), cylindrical, mixed.

Criteria of bronchiectasis

/.Anamnestic data: - frequent repeated pneumonias with the same localization of lesion with lingering course, which begin at early childhood and infancy; severe pneumonias with lung destruction in the past. I I. Clinical: - permanent productive cough, increasing during exacerbation, with production of mucopurulent sputum, more often in morning; - syndrome of chronic intoxication: pallor, grey skin, quick fatiguability, bad appetite, worsening of feeling; - syndrome of chronic heart-pulmonary failure : cyanosis of naso-labial triangle and acrocyanosis; dyspnea and tachycardia after physical exertion, clubbed fingers, curvature of the nails in their long axis (like "watch glasses"); - thorax deformation: asymmetry of the thorax; retraction or protrusion of separate parts. May be lag of one part of the thorax in the time of breathing; - physical finding are characterized as stable: a) on percussion - shortening of resonance or tympanic tint above the lesion zone; b) on auscultation - changing of breathing depending on the expression of process (harsh, bronchial sometimes amphoral, may be weakened ). Stable various moist and dry rales.

Criteria of bronchiectasis

///. Instrumental: - -ray: on X-ray film made in remission there is a strengthened lung figure, thickening of bronchi walls of one or several segments, diminishing of separate lung segments (pneumosclerosis). - Bronchoscopy: bronchial deformation, saccular or cylindrical bronchiectasis, endobronchitis of different type (catarrhal, purulent etc.). - Bronchography: bronchiectasis of various forms (cylindrical, saccular). - Spirography: decreasing of reserve ability of external breathing, stable respiratory insufficiency. - Pneumotachometry: disturbances of bronchial condition permeability. IV. Laboratory findings: 1) WBC - inflammatory changes are present in relapse and absent on remission. 2) Immunological findings: decreasing of secretory immunoglobulin A and G in bronchial content; - decreasing of serum immunoglobulin A and G. Differential diagnosis is provided with recurrent bronchitis; repeated, lingering pneumonias, tuberculosis of the lungs.

Bronchial Asthma (BA)

According to the modern concept BA is chronic inflammation of respiratory tract with spread but varying obstruction; with hypersensitivity to different agents caused specific immune (sensibilisation, allergy) or nonimmune mechanism. Prominent clinical sign of disease is an attack of difficult breathing (dyspnea) because of bronchial obstruction, hypersecretion of mucous, edema of bronchial walls. Types of obstruction: Acute - spasm of smooth muscles of bronchi. Subacute - edema of mucous of respiratory tract. Chronic - hypersecretion of tenacious mucus, which obturates terminal parts of respiratory tract. Sclerotic changes in bronchial wall. Forms of disease: Atopic (exogenous). Nonatopic (endogenous). Mixed.

Criteria of Bronchial Asthma:


1. Atopic anamnesis. 2. Paroxysms of dyspnea. 3. Spasmatic cough (especially at night and on physical exertion and difficult expiration), accompanied with acute inflammation of lungs. 4. Asthmatic status. 5. Periodic manifestations of symptoms, wheezing in the children of early age more than 3 times. 6. X-ray: at attack there are signs of enrichment of bronchiovascular picture.

A child may have one or more signs, which are used for estimation of the severity of the disease. The patient has to be referred to the most severe group if he reveals even only one sign proper to this group. If the patient with severe course of BA corresponds to more easy forms of disease, but receives drugs treatment corresponding to severe form it is necessary to diagnose the severe BA. Complications: - Atelectasis, - spontaneous pneumothorax, - subcutaneous and mediastinal emphysema, - pulmonary emphysema, - pulmonary heart etc.

Criteria of Severity of BA in children


SignsMildModerateSevere Frequency of attacksNot more than 1 a month3-4 times a monthSeveral times a week or every day Clinical characteristic of attacksEpisodical, quickly disappearing, easyAttacks are accompanied with external expiration disturbances Permanent presence of symptoms, severe attacks, asthmatic statusNight attacksAbsent or easy2-3 times a weekAlmost every 24 h Tolerance to physical exertion, activity, sleep disturbancesNot changedDecreasedEssentially decreasedIndex of FEVI and PEF80% and more (as compared with normal)60%Less than 60%24 hours changes in bronchial permeabilityNot more than 20%20-30%Over 30%Characteristic of remission period3 and more monthsLess than 3 months1-2 monthsPhysical developmentNot disturbedNot disturbedPossible retardation and disproportion of physical developmentThe methods of arrest of the attacksThe attacks disappearSpontaneously or by means of single reception of broncholitics (in the form of inhalation, orally) The attacks arearrested by broncholitics (inthe form of inhalation and parenterally), corticosteroid drugs are administrated parenterally if indicatedThe attacks are arrested at the hospital with the of broncho- and spasmolitics. Simultaneously with corticosteroidsBasic antiinflammatorytreatmentInhalative corticosteroids in the moderate dosesInhalativecorticosteroids in the moderate dosesInhalative and systemic corticosteroids

Criteria of Severity of BA attacks


SignsMildModerateSevereStatus asthmaticus Physical activityPreservedLimitedForced positionAbsentSpeechPreservedLimited. Not fluent speech, speech consists of separate phrasesNot fluent speechAbsentConsciousnessSometimes excitationExcitationExcitation, fear,"breathing panic"Confused unconsciousness hypoxe-michypercapnemic comaRespiration rateRespiration is acceleratedExpressed expiratory dyspneaVery expressed expiratory dyspneaBradypnea, tachypneaParticipation of axillary musclesExpressed a littleExpressedExpressed very muchParadoxal toraco-abdominal respirationWheezingUsually at the end of expirationExpressedExpressed very much"Dumb" lungs, absence of respiratory soundsPulse rateAcceleratedAcceleratedAccelerated very muchBradycardiaFEVI, PEF in % of norms of best patients valueMore than 80%60-80%Less than 60%Pa02NormalMore than 60 mm HgLess than 60 mm HgPaC02NormalLess than 45 mm HgMore than 45 mm Hg

Treatment of BA
BASIC: Ingalation of Glucocorticoides

Symptomatic: B-2 agonists; holinolitics

Nephropathies in children
LECTURE:
DONETSK NATIONAL MEDICAL UNIVERSITY Department of Paediatrics Chief: Prof. Y.V.Prohorov LECTURER: Asociated prof. E.A.Tolstikova

PYELONEPHRITIS (P)

P is an infectious inflammatory disese of tubulointerstitial renal tissue. Diagnostic Criteria: I. CLINICAL Intoxication Syndrome: Weakness, indisposition, bad appetite, loss of weight. In babies: vomiting, meningeal symptoms, toxicosis, exicosis. Painful Syndrome: Pains in abdomen and lumbur region; in the absence of any complaints it is necessary to reveal them actively (palpation of abdomen, jerk on lumbar region Pasternatskiy symptom) Disuria: frequent and painful micturitions.

Renal Syndrome:

leukocyturia - from moderate to significant up to pyuria; leukocyturia with more than 5-7 leukocytes in vision field is demonstrative, neutrophils are prevalent. In the absence of demonstrative leukocyturia it is necessary to carry out the tests of Amburgeau, Addis-Kakowsky, Nechiporenko; bacteriuria - a colony count of over 100 000/ml can be taken as indicating significant bacteriuria. Evaluation of bacteri uria is made by taking into account of the type of a microorganism, its pathogenicity, the form and period of the disease, degree of activity of the process and patient's age. proteinuria - is not typical for pyelonephritis, usually it is mild (1 g/l); hematuria is not typical for pyelonephritis, but it some times occurs in secondary pyelonephritis; restriction of tubular functions as to osmotic concentration (specific gravity is below 1020 while diuresis is less than 1000ml/d). The renal signs of the disease are the most important ones in diagnosing of pyelonephritis, the extrarenal signs in some children may be absent, in others they may be latent.

II. DATA of EXCRETORY UROGRAPHY:


a) in acute pyelonephritis: the kidney is enlarged by more than 1cm on the account of its parenchyma; hypotonia of the ureters; b) in chronic pyelonephritis: - asymmetry of the kidneys; - decrease of renal parenchyma as compared to contralateral; - significant variations of its thickness in the same kidney in different places; - deformation of calyco-pelvic system; - irregular excretion of contrast substance up to adynamia of calicopelvic system. c) in chronic secondary pyelonephritis: - obstruction of the urinary tract; - congenital malformations; - refluxes; - renal dysplasia.

Clasification of pyelonephritis

FORM: 1. Primary. 2. Secondary: - obstructive - dysembriogenetic - dysmetabolic COURSE: 1. Acute 2. Chronic: a) manifest recurrent form b) latent form PERIOD: Active Partial remisson Remission RENAL FUNCTION: Without function s disorders With function s disorders Chronic renal insufficiency

Differential diagnosis:
cystitis, glomerulonephritis, renal tuberculosis, renal tumor, urolithiasis. Pyelonephritis lasting for 6 mo. and longer is regardedas chronic.

TREATMENT OF PYELONEPHRITIS

ANTIBIOTICS:

Amoxicillin; cefalosporins; carbenicillin

NITROFURANS:

1. Furadonin; 2. Furagin 5 8 mg/kg divided in 4 intakes 3. Nalidixic acid (negram, nevigramon) 50-60 mg/kg in 4 intakes 4. 5-NOK, nitroxolin 8-10 mg/kg in 4 intakes

Glomerulonephritis

Glomerulonephritis is an infectious allergic renal disease with primary lesions of glomeruli. Boys suffer twice more often than girls. Diagnostic criteria 1. Clinical: 1) Extrarenal symptoms: a) edemas - at first they occur on eyelids, face; later they may be generalized; b) arterial hypertension - more often hypertension is moderate, in some cases it may be absent.

2) Renal symptoms:

a) oliguria and anuria are present in the initial period of acute glomerulonephritis, in this case urine has high specific gravity (1030-1040 and more); b) hematuria of different degree - moderate (microhematuria) and massive (macrohematuria); c) proteinuria: moderate - up to 1000 mg/l (daily loss is up to 1 g); significant - more than 1000 mg/l, up to 2500-3000 mg/l (daily loss is 2,5-3 g); massive - more than 3000 mg/l (daily loss is more than 3 g). leucocyturia - is nottypicalfor glomerulonephritis; may be transitory leucocyturia of lymphoid character; cylindruria - hyaline, epithelial, granular, waxy casts.

3. Metabolic symptoms:

disorders of water-electrolyte metabolism, which are characterized by different types of hyperhydration intracellular and extracellular; in ionogram there is elevation or decreased level of some electrolytes in plasma and erythrocytes. These disorders are most significant in acute glomerulonephritis in oligoanuric period, in the development of acute and chronic renal insufficiency; disorders of protein metabolism are the most signifcant in high proteinuria and they are characterized by hypoproteinemia (total protein level is less than 60 g/l), hypoalbuminemia (albumins are less than 50%), changes of globulin's fractions ratio; disorders of fat metabolism are present in some cases of glomerulonephritis and they are characterized by hypercholesterinemia (cholesterin is more than 5 mmol/l). Mentioned above renal and extrarenal symptoms and metabolism disorders are seen in different cases of glomerulonephritis in various combinations.

Differential diagnosis :

- glomerulonephritis - pyelonephritis, - renal tuberculosis, - and tumor.

CLASSIFICATION OF GLOMERYLONEPHRITIS

A. FORM: 1. ACUTE GLOMERYLONEPHRITIS (AG): a) with nephritic syndrome; b) with nephrotic syndrome; c) with isolated urinary syndrome; d) with nephrotic syndrome, hematuria and hypertention. 2. CHRONIC GLOMERYLONEPHRITIS: a) nephrotic form; b) hematuric form; c) mixed form; 3. SUBACUTE (malignant) GLOMERYLONEPHRITIS.

B. ACTIVITY OF RENAL PROCESS:

1. ACUTE GLOMELURONEPHRITIS: a) Period of initial manifestations. b) Period of comprehensive manifestations. c) Period of reverse development. d) Transition to chronic glomerulonephritis. 2. CHRONIC GLOMERYLONEPHRITIS: a) period of exacerbation; b) period of part remission; c) period of full clinical and laboratory remission.

C. CONDITION OF RENAL FUNCTIONS:

1. ACUTE and CHRONIC GLOMELURONEPHRITIS: a) Without disorders of renal functions; b) With disorders of renal functions; c) Acute renal insufficiency 2. SUBACUTE (malignant) GLOMERYLONEPHRITIS: a) With disorders of renal functions; b) Chronic renal insufficiency. Glomerulonephritis lasting for 1 year and longer is regarded as to be chronic.

Interstitial nephritis

Interstitial nephritis (IN) is an abacterial inflammation of renal connective tissue with the following involvement of tubules, blood and lymphatic vessels of renal stroma in the pathologic process. Diagnostic criteria: /. Clinical symptoms and signs of acute IN: vomiting; headache; pain in the lumber region; fever; skin eruptions; chill.

//. Laboratory symptoms:


anemia; elevated ESR; acidosis; azotemia; hyperkalemia; hypostenuria, isostenuria; hematuria; leukocyturia of mononuclear or eosinophilic character; high activity of transaminidase in blood and urine; beta-2-microglobuiine in urine; antibodies to the protein of Tamm-Hoursefield.

Clinical signs and symptoms in IN:


flabbiness; bad appetite; abdominal pains (renal colic); osteodystrophy; lag of weight; Laboratory symptoms: hematuria; dysuria-polyuria; tubular acidosis; "salt-toss kidney". Differential diagnosis is provided with pyelonephritis, diffuse and hereditary nephritis, renal tuberculosis, renal tumors, dysmetabolic nephropathies, urolithiasis.

Acute Renal failure (ARF)

Acute renal failure (ARF) denotes an acute impairment of renal function stipulated by sudden and sharp decrease of renal glomerular filtration rate and disorders of excretion of toxic substances, disturbances of water, electrolyte metabolism and acid-alkaline balance. Diagnostic criteria There are pre-renal, renal and post-renal (obstructive) ARF. Important causes of ARF: A. Pre-renal: Acute gastroenteritis. Hemorrhage. Shock.

B. Renal:

1. Acute tubular necrosis: Fluid loss, hemorrhage, shock; Intravascular hemolysis; Sepsis; Nephrotoxic drugs, chemical, radiocontrast substances; Major surgical procedures, road accidents, exten sive burns; Hepatic failure, congestive cardiac failure. 2. Glomerular disease: Acute glomerulonephritis; Hemolitic uremic syndrome. Interstitial nephritis. Acute bacterial pyelonephritis. Miscellaneous: Snakebite; Renal vein thrombosis.

C. Post-renal (obstructive):
Calculus, blood dots, crystals of uric acid, sulphonamides.

Stages of ARF:
Initial (preanuric). Oligoanuric. The stage of diuresis rehabilitation with the development of polyuria. Convalescence. The clinical picture of the initial stage depends on the diseases, which caused ARF.

Oligoanuric stage: Pallor with icteric tint (sometimes hemorrhages, itching); Adynamia, headache, flabbiness; Vomiting, nausea, diarrhea; Tachycardia, decrease of arterial pressure, disorders of rhythm and conduction; Noisy dyspnea; Convulsions; Abdominal pains; Lung's edema. Polyuric stage: Polyuria (up to 10 I) Anemia Infectious complications

Chronic renal failure

Chronic renal failure (CRF) implies permanent, irreversible, severe decrease of renal function. As it is recommended by the 4th European congress of pediatricians-nephrologists it is necessary to diagnose CRF in the children with renal diseases when gtomerular filtration is less than 20 ml/min/1,73m2, creatinine level is more than 0,177 mmol/l and urea level is more than 5,8 mmol/l.

1.

Clinical:
Tiredness, fatigue, headache, loss of appetite, vomiting; Polyuria, nicturia, polydypsia, bone and joint pains, retardation of growth, dryness and itching of skin; Muscular convulsions, paresthesias, signs of sensor or motor neuropathy; Heart failure and hemodynamic disorders. //. Laboratory: Decrease of glomerular filtration rate; Metabolic acidosis; Anemia; Decrease of thrombocytes' adhesion; Hyperkalemia, hyperphosphatemia, hypocalcemia, hypoproteinemia, hyperuricemia; Isostenuria; Renal osteodystrophy; X-ray examination of the chest may reveal cardiomegaly, hypertrophy of the left ventricle, aortectasia, lung's edema, pleural exudate.

Cause of CRF

/. Glomerular diseases a) Glomerulonephritis: of unknown etiology associated with systemic lupus erythematosus (SLE), polyarteriitis nodosa; Henoch-Schbnlein vasculitis. b) Familial nephropathy: nephronophthisis; Alport's syndrome. Hemolytic uremic syndrome; Amyloidosis.

2. Congenital anomalies: bilateral renal dysplasia; Reflux nephropathy. Obstructive nephropathy: pelviureteric junction obstruction; posterior urethral valve; calculi. 3. Miscellaneous: - bilateral Wilms1 tumor.

Dysmetabolic nephropathies

Dysmetabolic nephropathies (DN) are renal lesions because of the severe disorders of metabolism. All the children with dysmetabolic nephropathies have pathology of the bile ducts. Hyperoxaluria may be primary (rare hereditary enzymopathy with elevated synthesis of oxalates) and secondary (hereditable on polygenic type family instability of membranes).

Diagnostic criteria of DN

/. Clinical: recurrent pains in joints and their swelling; abdominal pains (renal colic); lesions of the lungs. //. Laboratory: hematuria; elevated urine level of glycolate, glyoxalates and oxalates in the primary type and D-glyceric acid with oxalates in the secondary type; elevated daily excretion of oxalates.

//. Laboratory findings:

elevated blood and urine level

(more than 3,9 mmol/l and 3,6 mmol/day correspondingly) of uric acid.

LECTURE

CARDIOLOGY Congenital Heart Diseases

DONETSK NATIONAL MEDICAL UNIVERSITY Department of Paediatrics Chief: Prof. Y.V.Prohorov LECTURER: Asociated prof. E.A.Tolstikova

Patent ductus arteriosus (PDA)

Patent ductus arteriosus is a communication between the pulmonary artery and the aorta. The incidence is about 20% among all congenital heart diseases; it occurs more often in girls. Hemodynamics PDA results in left-to-right shunt from the aorta to the pulmonary artery. The increased flow after passing through the lungs reaches the left atrium which enlarges in size. The left ventricle receives the increased volume of blood that causes its dilatation.

Diagnostic criteria

/. Anamnestic. Recurrent pneumonias, respiratory infections. //. Clinical. Continuous systolic and diastolic murmur is audible with maximum in the second left interspace as well as the accentuated 2nd tone over the pulmonary artery and below the left clavicle.

///. Instrumental.

- ECG reveals the signs of overloading of the left ventricle or of both ventricles. - PCG reveals highly amplitude systolic and diastolic murmur in the second left interspace, increase of the amplitude of the second sound. - EchoCG reveals increase of cavities of the left atrium and the left ventricle, increase of motion amplitude of the mitral valve; the diagnosis of PDA may be confirmed by Doppler. - Radiography reveals the lung fields appearing plethoric (hypervolemia), increase of the left ventricle and the left atrium, aortic arch is marked.

Atrial septal defect (ASD)

Atrial septal defect is an abnormal communication between the two atria. The ostium primum type of the defect is located inferior to the fossa ovalis, the ostium secundum type is generally anatomically located at the fossa ovalis. Atrial septal defect constitutes 8-16% of all congenital heart diseases, it occurs twice more often in girls.

Hemodynamics

The right atrium receives blood not only through the superior and inferior vena cava but also the blood, shunted from the left atrium. The right atrium gets larger in size to accommodate the extra volume of blood, so does the right ventricle. The cardiac apex is formed by the enlarged right ventricle. The pulmonary artery and its branches are enlarged as well.

Diagnostic criteria
/. Clinical . Moderate systolic murmur is audible with maximum in the 2nd-3rd left interspace near the sternum; the second sound over the pulmonary artery is widely split and accentuated.

//. Instrumental.

- ECG - this is characterized by right axis deviation and right ventricle hypertrophy, right bundle-branch block; - PCG reveals moderate and low amplitude systolic murmur in the 2nd-3rd left interspace, increase of amplitude of 2nd sound over the pulmonary artery; - EchoCG reveals increase of the right ventricle cavity, paradoxical ventricular septal motion. - Radiography reveals from mild to moderate cardiomegaly, right atrial and right ventricular enlargement, prominent main pulmonary artery segment, a relatively small aortic shadow and plethoric lung fields.

Ventricular septal defect (VSD)

This is a communication between the two ventricles. Anatomically 90% of all ventricular septal defects are located in the membranous part of the ventricular septum with a variable extension into the adjacent muscular part of the septum, others are located in the muscular septum and can be multiple. VSD is the most common congenital cardiac lesion and accounts for 15-25% of all congenital heart diseases, it occurs equally often in boys and girls.

Hemodynamics

Large septal defect (more than 1.5cm) leads to great shunt of blood from the left ventricle to the right one, overloading of vessels of lesser circulation, the left atrium and the left ventricle.

Diagnostic criteria

/. Anamnestic: Recurrent pneumonias, respiratory infections. //. Clinical: a) arrested physical development; b) cardiac humpback; c) systolic thrill in 3rd-5,h left interspace at the sternal border; d) enlargement of borders of relative heart dullness; e) rough systolic murmur over all cardiac regions, accentuated 2nd sound over pulmonary artery.

III.Instrumental

- ECG reveals the signs of overloading of left cardiac parts; later, as pulmonary hypertension develops, the signs of right ventricle hypertrophy appear; - PCG reveals highly amplitude systolic murmur, increase of amplitude of the 2nd sound over the pulmonary artery; - EchoCG reveals increased left atrial and ventricle size as well as exaggerated mitral valve motion. - Radiography reveals the cardiac silhouette of the left ventricle type with the heart size depending on the size of the left-to-right shunt, increased pulmonary vasculature.

Coarctation of the aorta

Congenital coarctation of the aorta is located at the junction of the arch with the descending aorta. It constitutes 15% of all congenital heart diseases and occurs more often in boys. Hemodynamics Arterial pressure above the narrowing is elevated, the left ventricle experiences the overload with pressure. Arterial pressure distal to narrowing is decreased; blood supply is accomplished on account of collateral vessels.

Diagnostic criteria

/. Clinical: a) complaints of a headache, nasal hemorrhages, fall of temperature of lower extremities; b) physical examination shows a good development of shoulder girdle, underdevelopment of lower extremities; c) arterial brachial pressure is elevated, femoral pressure is decreased or is not determined at all; d) moderate systolic murmur over all cardiac region is heard, with the point of maximum intensity over the back in the interscapular area.

//. Instrumental:

- ECG reveals signs of the hypertrophy of the left ventricle; - EchoCG reveals myocardial hypertrophy of the left ventricle; using suprasternal approach coarctation can be visualized and the gradient across the narrowing is obtained with Doppler. - Radiography reveals increase of the left ventricle, enlargement of the ascending aorta: aorta's arch is frequently not differentiated.

Tetralogy of Fallot (TF)

This is the most common cyanotic congenital heart disease in children above the age of two years constituting almost 75% of all blue patients. The overall incidence of TF is 12-14%. Anatomically TF consists of a ventricular septal defect associated with the obstruction of the right ventricular outflow in the form of infundibular or infundibular plus valvular pulmonic stenosis. The four constituents of tetralogy as describea riginally by Fallot are: a) ventricular septal defect, b) pulmonic stenosis, c) overriding or dextraposed aorta, and d) right ventricular hypertrophy.

Hemodynamics

Physiologically the pulmonic stenosis causes concentric right ventricular hypertrophy without cardiac enlargement and an increase of the right ventricular pressure. When the right ventricular pressure is as high as the left ventricular or the aortic pressure, the right-to-left shunt appears to decompress the right ventricle. As soon as the right and the left ventricular pressures become identical, increasing severity of pulmonic stenosis reduces the flow of blood into the pulmonary artery and increases the right-to-left shunt. As the systolic pressures between the two ventricles are identical there is little or no left-to-right shunt, and the ventricular septal defect is silent. The right-to-left shunt is also silent since it occurs at insignificant difference in pressure between the right ventricle and the aorta. The ventricular septal defect of TF is always large enough to allow free exit to the rightto-left shunt. Since the right ventricle is effectively decompressed by the ventricular septal defect, congestive failure never occurs in TF.

Diagnostic

criteria

/. Clinical: a) cyanosis of the skin and the mucous membranes (from light blue to intensive violet colour), which may be present from birth or makes its appearance some months or years after birth; b)anoxic spells (paroxismal attacks of dispnea); c) sitting posture - squatting; d) physical examination discloses rough systolic murmur in the 2nd-3rd left interspace, the flapping 1st sound at the apex, the weakened 2nd sound over the pulmonary artery;

//. Instrumental

- ECG reveals right axis deviation with right ventricular hypertrophy, "P" - pulmonale may be present; - PCG reveals rhombic systolic murmur in the 2nd-3rd left interspace; - EchoCG reveals the large overriding aorta, the right ventricular hypertrophy and outflow obstruction; - Radiography reveals the normal-sized heart with the upturned apex suggestive of the right ventricular hypertrophy, the absence of the main pulmonary artery segment gives it the shape described as "Coren-Sabot"; the pulmonary fields are oligemic.

Classification of congenital heart diseases (by Marder)


Disorders of hemodynamics With enriched pulmonary blood flow With diminished pulmonary blood flow With diminished greater circulation Without disorders of hemodynamics Disorders of hemodynamics Without cyanosis With cyanosis With enriched pulmonary blood flow Patent ductus arteriosus, atrial and ventricular septal defects, atrioventricular communication Eisenmenger's complex, transposition of great vessels, common arterial trunk

- With diminished pulmonary blood flow: Stenosis of the pulmonary artery Fallot's disease, tricuspid atresia, transposition of great vessels with stenosis of the pulmonary artery, Ebstein's anomaly, common false arterial trunk - With diminished greater circulation: Aortal stenosis, coarctation of the aorta

Without cyanosis

Patent ductus arteriosus, atrial and ventricular septal defects, atrioventricular communication Stenosis of the pulmonary artery Aortal stenosis, Coarctation of the aorta True and false dextrocardia, Anomalies of the aorta position and its branches, Small defect of the muscular part of the ventricular septum

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