Nephrotic Syndrome in Pediatrics: Overview and Insights

Nephrotic Syndrome in Pediatrics Presenter: Kessy Julius P. Intern Dr . Supervisor: Christina K. Galabawa, MD, Mmed 2, Pediatrics and Child Health, UDOM. DODOMA REGIONAL REFERRAL HOSPITAL (DRRH)

Outline Introduction Epidemiology Classification & Etiology Pathophysiology Clinical features Investigations & treatment Complications & prognosis 1.

Introduction Syndrome caused by renal diseases that increase the permeability across the glomerular filtration barrier. Characterized by a tetrad of clinical features 2.

Contd 1. Nephrotic range proteinuria Urinary protein excretion > 50 mg/kg/day. 2. Hypoalbuminemia Serum albumin concentration < 3g/dl (30g/l). Hyperlipidemia Edema. 3. 4. 3.

Epidemiology Occurs at all ages but most prevalent in children 1.5-6 years. Boys > girls, 2:1 ratio. Higher in underdeveloped countries (South east Asia and Africa). Incidence worldwide, 2 7 cases per 100,000 children/year. 4.

Classification & Etiology 1. Primary No identifiable systemic disease. These are patients with; I. Idiopathic NS No glomerular inflammation on renal biopsy. II. Primary glomerulonephritis Active sediment and glomerular inflammation on biopsy. 5.

Contd 2. Idiopathic I. Minimal Change Disease (MCD) - 85% II. Focal Segmental Glomeruloscrelosis (FSGSS) III. Membranoproliferative glomerulonephritis IV. Mesangial proliferation V. Membranous nephropathy >80% of children with idiopathic NS are steroid sensitive. 6.

Contd 3. Secondary Infections Viral (HBV, HCV & HIV), Bacterial (Streptococcal) and parasites (Malaria). Others are syphilis and toxoplasmosis. Systemic conditions Autoimmune (SLE & vasculitis), Metabolic (Diabetes & amyloidosis), Malignancies (Leukemia & lymphomas), Hematological (SCA), Obesity. 7.

Contd Allergy bee sting, cow s milk, pollen, house dust. Drugs NSAIDs, Ampicillin, penicillamine, gold, captopril and lithium. 8.

Contd 4. Hereditary NS Below one year. 9.

6.

Clinical features Periorbital puffiness: more marked in the morning and later generalized Scrotal edema Pleural effusion and Ascites are late features Decreased urine output Hypertension and hematuria are absent 10.

Clinical features . 11.

Clinical features . 12.

Clinical features . 13.

Clinical features . 14.

Investigations Urinalysis I. Proteinuria +3 or +4 II. Urinary protein excretion (>40mg/m2/hour) III. 24 hours urinary protein : creatinine > 3 IV. Microscopic hematuria in 10% V. Pus cells in underlying UTI VI. Cellular casts in other forms not MCD 15.

Contd Serum I. II. Others I. II. Albumin < 3g/dl Cholesterol > 250mg/dl CBC usually normal, raised ESR CXR to R/O pleural effusion 16.

Contd Renal biopsy (Indications) I. SRNS II. Frequent relapses III. Steroid toxicity IV. Secondary NS V. Gross hematuria VI. Hypertension VII. Renal insufficiency 17.

T reatment High dose steroids -2mg/kg (max 60) daily for 6 weeks -1.5mg/kg (max 40) alternating days for 6 weeks (If attained remission) Relapse management -Precipitated by minor infections -Treatment of infections often result in remission for +1-2 proteinuria 18.

T reatment Persistent +3-4 needs steroids -2mg/kg (max 60) daily until remission, then 1.5 mg/kg on A/D for 4 weeks. Steroid dependant and frequent relapses -Long term steroid therapy -0.3-0.7 mg/kg on A/D for 9-18 months. 19.

Special definitions Remission: trace/no protein in urine on dipstick for 3 consecutive days. Relapse: urine protein is 3+/4+ for three consecutive days after having attained remission. Frequent relapser: four or more relapses in 12 months. 20.

Special definitions Steroid dependent: two consecutive relapse while on A/D steroids or within 14 days of its discontinuation. Steroid resistant: proteinuria (+2 or more) on daily steroid therapy after 8 weeks. 21.

T reatment Supportive management: Diet - Adequate in proteins and calories - Fluid and salt restriction in edema Hospitalization -Infections -Marked edema - Monitoring BP in hypovolemia 22.

T reatment Diuretics -massive ascites, pleural effusion and severe genital edema Ambulation & Anti coagulants for thromboembolism ACEI in hypertension Calcium carbonate and vitamin D in Osteoporosis 23.

Complications Infections -SBP, Pneumonia, UTI -Sepsis -Cellulitis Arterial and Venous Thrombosis Steroids and other drugs adverse effects 24.

Prognosis Response time: 10% by the end of first week 70% by the end of second week 85% by the end of third week 92% by the end of fourth week 25.

Contd Steroid responders 90% Minimal change disease 50% Mesangial proliferation 20% Focal sclerosis 26.

Contd Poor prognostic factors -Hematuria -Hypertension -Hypocomplementemia -Focal segmental sclerosis -Steroid resistance 27.

THANK YOU!!! To study the phenomenon of disease without books is to sail an uncharted sea while to study books without patients is not to go to the sea at all - William Osler. 28.