Rheumatic fever, TOF, VSD, Nephrotic syndrome, AGN

Jones criteria of Rheumatic fever

Major criteria- 1. Migratory polyarthritis

2. Pancarditis

3. Sydenham chorea

4. Erythema marginatum

5. Subcutaneous nodules

Minor criteria- 1. Clinical features: fever, arthralgia

2. Lab features: a)Elevated acute phase reactants- raised ESR & CRP

b)Prolonged P-R interval( ECG)

2major

1 major + 2 minor

3 minor

Chemoprophylaxis of recurrence of rheumatic fever

Initial and subsequent (recurrent attack)-penicillin prophylaxis

Initial- phenoxymethyl penicillin or erythromycin- 10 days oral at any streptococcal sore throat

Subsequent attack- benzathine penicillin (IM)

Erythromycin (oral)

Penicilin V (oral)

Sulfisoxazole/ sulphadiazine (oral)

Duration-

RF without carditis- 5years/ till 21 years whichever is longer

RF with carditis but no residual heart disease- 10 years/ till 21 years whichever is longer

RF with carditis and residual heart disease- 10 years/ till 40 years whichever is longer/ sometimes lifelong treatment

Investigation of AGN

For nephritis- Urine RME: RBC, RBC cast(hematuria), mild protienuria, leukocytes

CBC- Hb(reduced), ESR(high), DC, TC

For streptococcal infection-

ASO titre- if pharyngeal infection present

Anti DNAase antibody- if skin infection present

Steptozyme test- Ab produced against antigen of bacteria

For complications-

Hb- mild hemolysis

Chest x ray for LVF(cardiomegaly)

Serum creatinine- raised( in acute kidney injury)

Serum electrolytes-hyperkalemia and acidosis

MRI for hypertensive encephalopathy

Management of AGN

Clinical features- puffy face, generalised edema, passage of scanty, high coloured urine

Physical examination- face: puffy around periorbital region and face, mild pallor, edema: present, BP: high

Features of Left ventricular failure-severe respiratory distress, Orthopnea, tachypnea, tachycardia, precordium hyper dynamic, apex beat shifted, lung base- cripitation on auscultation, liver enlarged and tenderness present , JVP: raised

Features of Hypertensive encephalopathy- headache, blurring of vision, confusion, coma, convulsion, unconsciousness, vomiting,

Renal failure- Anura, oligouria, vomiting

Treatment of AGN

1. Counselling of parents

2. Supportive treatment- bed rest, protein intake restricted to (0.5gm/kg/day), Potassium and potassium rich fruits and veggies restricted, fluid intake( 400ml/m2)+previous day output

3. Frusemide( 1-2 mg/kg/day)

4. Phenoxymethyl penicilin (50 mg/kg/day) in 4 separate doses for 10 days

5. Anti htn- nifedipine, labetolol, nitropprusside

Complications of AGN

1. Acute renal failure

2. Electrolyte imbalance- hyperkalemia, hyponatrimia, metabolic acidosis

3. Chronic kidney disease

4. Hypertensive encephalopathy

5. Left ventricular failure

6. Hypokalemia due to severe dehydration

Cardinal features of Nephrotic syndrome

1. Massive proteinuria( more than 1gm/m2/day)

2. Hypoalbuminaemia (less than 2.5gm/dL or 25 gym/L)

3. Generalised edema

4. Hyperlipiduria (more than 200 mg/dL)

Investigation of Nephrotic syndrome

Urine RME- Albuminaeuria, granular and hyaline cast, pus cell( if UTI)

24hr urinary total protein-more than 1gm/m2/day

Spot urinary protein: creatinine ratio- more than 2

Blood- serum albumin

Serum protein

Serum albumin:globulin

Serum urea, BUN, creatinine

Serum cholesterol

Serum c3

Blood for CBC, PBF,ESR

Others- chest X-ray

USG of abdomen

Renal biopsy

Urinary C/S

Management of Nephrotic syndrome

Specific- oral prednisolone

60mg/m2/kg daily in single/2-3 doses for 6 weeks

40 mg/m2/kg in single morning dose for next 6 weeks

Normal family diet with adequate protein but salt restriction

Hypovolumia- normal saline bolus/ albumin infusion prior to diuretics infusion

Mild edema- no diuretics

Or frusemide, spironolactone

Oral penicilin

Ca supplementation if steroid continued for more than 3 months

GI upset- antacid

Follow up- 2 weekly

Complications of nephrotic syndrome

1. Hypovolaemia

2. Shock

3. Acute renal failure

4. Cellulitis

5. Spontaneous peritonitis

6. UTI

7. Arterial and venous thrombosis

TOF management

1. Knee chest position / older children squatting position

2. Calm environment

3. O2 3-5L/min via face mask or head box

If cyanotic spell still persists then,

IV fluid normal saline 10 ml/kg

Morphine- to calm and relax muscle

NaHCO3- IV to correct acidosis

Propranolol- to relax infundibular muscle & reduce spasm

Phenylephrine (IM / SC)

If still persists then- intubation and ventilatory support

TOF advice

1. Knee chest position

2. Calm environment

3. Proper medication( drugs+ diet+ medications)

4. Monitor fluid intake

Components of TOF

1. Ventricular sepal defect

2. Pulmonary stenosis

3. Overriding of aorta

4. Right ventricular hypertrophy

Investigation of TOF

CBC- polycythemia

Chest x ray- boot shaped heart

ECG- right ventricular hypertrophy

Eco- VSD, RVH, overriding quantitative degree of right ventricular outflow tract

Rx of TOF

Anemia- correction

Polycythemia- Venesection/ phlebotomy

Prevention of dehydration

AMB for infective endocarditis

Rx for paroxysmal hypercyanotic shunt

Surgery- palliative shunt procedure

Total corrective surgery

What is cyanotic spell

1. Sudden onset of dyspnea→gasping respiration, syncope

2. Deepening of cyanosis

3. Alteration of conciousness

4. Decreased intensity of systolic murmur

5. Metabolic acidosis

Differentiation of VSD and TOF by clinical evaluation

Clinical evaluation: Cyanosis and clubbing- TOF

Precordium examination:

Inspection: hyperdynamic may be bulged| may be bulged due to rt ventricular hypertrophy

Palpation: apex beat: shifted to Lt, thrusting in nature| not shifted, tapping in nature

Left parasternal heave: may be present| may be absent

Palpable p2: present in pulmonary area| may be absent

Thrill: present in tricuspid area| systolic thrill may be present in upper left intercostal space

Auscultation: heart sound: both s1 & s2 found in 4 areas| s1 normal but s2 pound and single

Added sound: a harsh parasystolic murmur found| a loud ejection murmur found

Investigation of VSD

Chest x-ray: cardiomegaly, pulmonary vascular markings increased| no cardiomegaly but boot shaped heart, lung fields blocked due to decreased pulmonary vascularity

ECG: small VSD- normal, large VSD-left ventricular hypertrophy, VSD associated with pulmonary htn- biventicular hypertrophy, peaked PR interval

Echocardiogram: diagnostic, color Doppler test- blood runs through defected area( location and size of defect), | full diagnosis( VSD, RVH, overriding)

Rx of VSD

Small VSD- spontaneous closure

Moderate to large VSD- diet- high calorie

Frusemide

ACEI

Digoxin

Trans catheter device closure

Surgery

Classify congenital heart disease

Cyanotic- TOF, Transposition of great arteries, tricuspid atresia, persistent truancy’s arteriosus

Acyanotic-with lt to rt shunt- VSD, ASD, PDA

Without shunt -Pulmonary stenosis, aortic stenosis, coarctation of aorta