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Pediatrics 5th year, 18th & 19th lectures (Dr. Jamal)
Pediatrics 5th year, 18th & 19th lectures (Dr. Jamal)
Pediatrics 5th year, 18th & 19th lectures (Dr. Jamal)
Pediatrics 5th year, 18th & 19th lectures (Dr. Jamal)
Pediatrics 5th year, 18th & 19th lectures (Dr. Jamal)
Pediatrics 5th year, 18th & 19th lectures (Dr. Jamal)
Pediatrics 5th year, 18th & 19th lectures (Dr. Jamal)
Pediatrics 5th year, 18th & 19th lectures (Dr. Jamal)
Pediatrics 5th year, 18th & 19th lectures (Dr. Jamal)
Pediatrics 5th year, 18th & 19th lectures (Dr. Jamal)
Pediatrics 5th year, 18th & 19th lectures (Dr. Jamal)
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Pediatrics 5th year, 18th & 19th lectures (Dr. Jamal)

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The lecture has been given on Apr. 14th & 17th, 2011 by Dr. Jamal.

The lecture has been given on Apr. 14th & 17th, 2011 by Dr. Jamal.

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  • 1. Truncus ArteriosusTruncus arteriosus occurs when both aorta and pulmonary arteryarise as one trunk which arises from both right and leftventricles. There is usually an accompanying high VSD & mildto moderate cyanosis and H.F.Treatment involves restructuring the common trunk to createseparate vessels. Ebstein Anomaly "E.A."E.A. is a rare form of neonatal cyanosis, characterized bydownward displacement of the abnormal tricuspid valve into theright ventricle (atrialization of the upper part of the rightventricle) making the right atrium to be hugely dilated.Right ventricle is also dysplastic,and shows some degrees ofoutflow obstruction resulting in right to left shunt through ASDor foramen ovale and various degrees of cyanosis.Mild forms of E.A. may be asymptomatic.Neonates with severe E.A. show; deep cyanosis, hugecardiomegaly, C.C.F. and a pan-systolic murmur.X.ray shows a hugely enlarged box shaped heart.ECG shows characteristic changes of RBBB, P- pulmonale, P-mitrale and prolonged P-R with or without WPW.Echo, catheterization and angiography are confirmatory.Prognosis in severe forms is very bad as there are very fewsurgical options.PGE1 and O2 may help. Hypoplastic left heart syndrome It is also called aortic atresia.This rare form of C.H.D. is characterized by severe hypoplasiaof mitral and aortic valves, left ventricle and the aortic arch, aPDA is the only source of systemic blood flow.Very few survive the 1st week of life. 21
  • 2. Pulmonary stenosis "P.S"P.S may be valvular, subvalvular (infandibular) or supravalvular(main pulmonary artery, the right or left branches).Most cases are mild, asymptomatic and diagnosed accidentally.Severe forms may have symptoms like dyspnea on effort andcyanosis.Ejection systolic murmur at ULSB is heared. X.ray in severeforms shows cardiomegaly, oligemic lungs and prominentpulmonary artery.Treatment is mainly by balloon pulmonary valvuloplasty if theright ventricular pressure is more than 75% of the systemicsystolic pressure. Congestive Cardiac Failure "CCF"CCF means failure of the heart to maintain an output necessaryfor the individuals body.Causes: 1. C.H.D: e.g VSD, PDA, TGA, TAPVD, truncus arteriosus, coarctation of aorta, severe stenosis of A.V, P.V or M.V, hypoplastic left heart, anomalous left coronary origin, arteriovenous malformation; etc. 2. Myocardial diseases: -Viral myocarditis; more common during the 1st two months of life. -Primary myocardial disorders as: *Glycogen storage disease. *Endocardial fibroelastosis. *Ischemic and other cardiomyopathies. -Drugs as Doxorubicin(a chemotherapeutic agent). -Hyperthyroidism. 3.Others:-Severe anemia(less than 5 gm/dl). -Arrhythmia as SVT. -Severe ARI. 22
  • 3. -Chronic upper airway obstruction. -Hypertension. -Overhydration by overloading the circulation.Clinical Picture: This varies to some extent according to the ageand whether the failure is right sided, left sided or both.In general features common in left failure include: -Tachypnea and feeding difficulty -Tachcardia -Cough -Wheezing -Rhonchi and basal cripitation.Right features include: -Hepatomegaly -Facial oedema -Jagular venous engorgement (difficult to be detected in infants) -Leg oedema.In addition the following are seen in failure of either side: -Cardiomegaly -Gallop rhythm (S3) -Peripheral cyanosis -Small volume pulse -Failure to gain weight -Irritability and excessive sweating -Cyanosis and shock are late manifestations.Investigations: 1-CXR helps in -Cardiac size assessment and presence or absence of pulmonary vascularity. -Excluding pulmonary cause. -Evidence of some C.H.D. 2-ECG e.g non specific ST and T changes, general amplitude,arrhythmia, P- pulmonale, etc. 3-Echo in determining functional capacity of the heart and indiagnosis of infective endocarditis. 23
  • 4. Management:A-General: 1-Bed rest in "propped up" position at an angle of 45°. 2-O2 by nasal catheter, head box or in plastic tent. 3-Sedation ; using Morphine, Pethidine, Phenobarbital, Chloral hydrate, Promethazine or Diazepam. 4-Antibiotics in presence of infection. 5-Diet ; low salt.B-Anti failure: 1-Digoxin; increases myocardial efficiency and decreases tachycardia. Digitalization is made during 1st 24 hours, at adose of: Neonates 0.04 mg/kg 1-12 months 0.08 mg/kg 1-3 years 0.06 mg/kg > 3 years 0.04 mg/kg Half the total dose initially, the next half of the dose is dividedinto two and given with 8 hours apart. This is the oral dosage.The parentral dose is 2/3 of the oral dose.Maintenance is given from the 2nd day at a dose of 1/4 to 1/3 ofthe digitalization dose. 2-Diuretics: specially Frusimide at a dose of 1-3 mg/kg orally or 0.5-1.5 mg/kg I.V± Spironolactone (a potassium sparingmildly effective diuretic) 1-4 mg/kg/day in two divided doses. 3-Angiotensin Converting Enzyme Inhibitors (ACEI); asCaptopril reduce work of the heart by causing peripheralvasodilatation and may reduce left to right shunt.ACEI is a potassium sparing drug, hence no need for potassiumsupplements.Both Digoxin and ACEI should be avoided in left heart outflowobstruction. 4-Potassium supplements especially if Digoxin andFrusimide are given. 5-Correction of the underlying cause e.g anemia, arrhythmia,chest infection, circulatory overload, thyrotoxicosis, infectiveendocarditis, hypertension, etc. 24
  • 5. 6-In refractory CCF; vasodilators as Nitroprusside, inotropic(Dopamine, especially in presence of cardiogenic shock), orBetablockers may be tried. 7- Heart transplant or artificial heart is another form oftreating intractable HF. Rheumatic Fever "R.F"R.F. is an immunological manifestation which follows U.R.I. byspecific subtypes of group -A- beta-haemolytic streptococci, ingenetically predisposed individuals. R.F has become very rarenowadays if compared to several decades ago.Streptococcal skin infections do not cause R.F.5-15 year age group is most commonly affected.There is a latent period of 1-3 weeks between sore throat andR.FThere is probably some antigenic similarity betweenstreptococcal antigens and those of joint, heart, skin etc…Diagnosis of R.F. depends upon modified Jones criteria whichinclude: Major criteria: -Carditis -Arthritis -Chorea -Subcutaneous nodules -Erythema marginatum. Minor criteria: -Fever; rarely exceeds 39 Cº -Arthralgia (only joint pain, no objective signs of arthritis) -Elivated E.S.R, C.R.P -Prolonged P-R interval -Previous rheumatic fever. R.F is diagnosed when two major or one major and two minorcriteria are present in addition to an evidence of streptococcalinfection as positive culture, increased A.S.O.T or residualmanifestations of recent scarlet fever. 25
  • 6. In cases when arthritis is taken as a major criteria arthralgia cannot be taken as a minor minor one.As chorea is a late manifestation; other features of R.F may notbe present. Thus in absence of other causes of chorea it can(even in absence of supportive evidences of previousstreptococcal infection) be considered as rheumatic chorea.Similarly insidious or late onset of carditis can be considered asrheumatic carditis provided that other causes are excluded. -Arthritis: affects 75%, clinically migratory (flitting) polyarthritis, mainly affecting large joints and commonly sparing temporomandibular, sternoclavicular, interphalengeal and spine. The affected joint is painful, tender, swallen, shows limitations of movements with very little if any sterile effusion. After 2-5 days the pain and swelling may resolve, only to appear in another joint, without any residual damage. It is very rare to have more than one joint affected at the same time and is also rare to have affection of only one joint.-Carditis: occurs in 50%. It is usually pancarditis (endo, myoand pericarditis).Carditis is the major cause of mortality in R.F. and is the onlyacute manifestation which may leave squelae.Features of endocarditis include valvular affection byregurgitation, most commonly mitral, less commonly aortic andleast commonly tricuspid and extremely rarely pulmonary.In a long term even calcification and stenosis of the affectedvalves may occur.A pansystolic and sometimes a diastolic (Carey comb murmur)are heard.Myocarditis results in soft heart sounds and C.C.F.Pericarditis manifests by chest pain, pericardial rub and mildeffusion.Generally if carditis does not appear during the 1st two weeks ofR.F. it is unlikely to appear later. 26
  • 7. -Chorea (Sydenhams chorea"S.C"): is a late manifestationoccuring about three months after the onset of R.F.S.C is 3-4 times more common in girls.Features of S.C include purposeless, jerky movement resultingin deranged speech, falling things, disturbed handwriting,awkward gait, weakness, emotional liability and sometimesdepression.Clinical tests used to detect S.C. include: – Finger-nose test. – Buttoning the clothes test. – Dinner fork hand positioning. – Pronator test. – Milk maid grip. – Darting tongue. – Audible click during speech. – Clumsy writing. – Sustained hung-up or double knee jerk.S.C is a self limited condition and disappears during 2-6 weekseven without any treatment, but chorea patients are predisposedto have carditis late in life, hence they should be givenprophylaxis to avoid further streptococcal infection.Other causes of chorea are: choreoathetoid C.P., drugs andHuntingtons chorea.-Subcutaneous nodules: occur over bony prominences (elbow,shin, occiput and spine). They vary in size from few mm. to 1-2cm. They are firm, non tender and subcutaneous in location,remain for weeks-months and are found in < 5%.They appear3-6 weeks after the onset of R.F and when present, they almostindicate presence of carditis.-Erythema marginatum: an early major skin sign which appearsvery rarely and is usually undetectable in dark skinned. They areevanescent, non pruretic, non-blanching with raised margin andpale center. They mainly occur over trunk.As subcutaneous nodules these rashes are also common inpatients with carditis. 27
  • 8. Prognosis of R.F.: depends on the severity of R.F. and especiallythe presence of carditis or later development of infectiveendocarditis.Management of R.F: – Bed rest; for 2-3 weeks in all, and larger period and strictly in presence of carditis. – 10 days antibiotics (especially penicillin) to eradicate any streptococcal infection. – Treatment of H.F if present. – Anti-inflammatory drugs (the corner stone of R.F treatment). Aspirin and steroids are the only two anti-inflammatory agents known to suppress rheumatic activity. In absence of carditis Aspirin alone while in presence of carditis use of steroid (Prednisolone) is mandatory. Anti-inflammatory drugs are given for 12 weeks, initially at high doses e.g. 100 mg/kg/day for Aspirin and 2 mg/kg/day for Prednisolone and then gradually reduced. Chorea (in presence of rheumatic activity) when mild needs no drug treatment apart from reassurance. Severe forms can be treated by drugs as; Phenobarbitone, Diazepam, Chlorepromazine, Phenothiazide, Haloperidol and sometimes steroids.Prophylaxis: This is extremely important to prevent recurrenceof R.F.This is provided by using long acting Penicillin which is betterto be continued for life. 28
  • 9. Infective Endocarditis "I.E" I.E is a form of infection (commonly caused by bacteria, rarely fungal or others) of the endothelial surface of the heart and forming vegetations (made of fibrin, inflammatory cells and microorganisms). I.E. predominantly occurs in a diseased heart (congenital or rheumatic valvular diseases or prosthetic valves) or rarely in an otherwise normal heart, especially during septicemic illnesses. I.E may occur after heart surgery, especially shunt operations. I.E. of normal heart is usually acute, and results in acute H.F, while I.E. of a diseased heart is subacute. Predisposing causes include infection anywhere in the body like boils, gingival infection, tooth abscess, ear infection, UTI, osteomylitis, interventions as dental procedures (even tooth brushing), cardiac catheterization, G.U procedures, bronchoscopy and I.V. canulation. Drug addicts using I.V. route are also prone. The commonest organism in the past has been Streptococcus viridance, recently Staph. aurius has also become common, gram negatives and fungi are rare causes.Clinical picture: any fever for more than 7 days in a patient witha known heart disease raises the question of S.A.B.E. Clinical manifestations can be grouped into: 1-Septic signs: fever, chills, rigors, night sweats, general malaise, anorexia, weight loss, anemia, splenomegaly and clubbing. Abscesses may affect brain, kidney or bones. Right side I.E. results in lung abscesses. 2-Cardiovascular signs: left, right or biventricular failure, a new or changing of a pre-existing murmur, embolic phenomena as stroke from CNS embolism, hematuria from renal infarction, left flank pain from splenic infarction, G.I. hemorrhage from mesenteric embolism. 29
  • 10. Evidence of vegitations and rapidly developing regurgitation lesions of the aoric, mitral or rarely tricuspid valves. 3-Features of immune complex deposition: these present as vasculitis, e.g: -Microscopic hematuria, glomerulonephritis and even acute R.F. -Osler nodes (tender erythematous nodules over the pulp of the finger tips). -Roth spots (boat-shaped retinal hemorrhage with pale center). -Splinter hemorrhage under the nails. -Janeway lesions are non tender erythematous patches over the palms and the soles.Investigations: -Blood culture; several samples with 30 min. apart are taken foraerobic, anaerobic and fungal culture. -C.B.C. and E.S.R: increased total W.B.C and total neutrophils(especially in the acute form of I.E.)Decreased platelet, increased ESR and normocyticnormochromic anemia. -Positive C.R.P. -Microscopic hematuria ± albominurea. -Hypergammaglobulinemia. -Positive rheumatoid factor in 50%. -Echo; shows vegitations of > 2 mm. and may detectcomplications as ruptured chordae tendinae or valve cuspperforation and mycotic aneurism.Management of I.E:Large doses of parentral (I.V.) antibiotics should be started inthe form of Penicillin and Gentamycin for 4-6 weeks.Culture and sensitivity results determines the propercombination. Complications may need emergency cardiacsurgery. 30
  • 11. Prophylaxis:All patients with congenital or acquired heart lesions needantibiotic prophylaxis before any surgical procedures that arelikely to initiate bacteremia.Amoxil 50 mg/kg one hour before and 25 mg/kg 6 hours afterthe procedure.In highly risky patients I.V. Ampicillin + Gentamycin is givenbefore and after the procedure. 31

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