Pediatrics | Nephrotic Syndrome | Made Easy By Dr. Shankar Yadav | Pediatric Nephrologist | BPKIHS
A video by: Dr. Bharat Kc (MBBS 2014, BPKIHS) Nephrotic Syndrome Dr. Shankar Pd. Yadav MD (Pediatrics), FPN(IPNA) Assistant Professor BPKIHS Objectives Epidemiology Definition Pathophysiology Aetiology & Clinical Presentation Investigation Clinical Course and Management Epidemiology INCIDENCE: 2-7 per 100,000 children PREVALENCE: 12-16 per 100,000 children Higher in children from south Asia (Eddy AA et al. Lancet 2003) Hospital based occurrence of paediatric renal disease is about 6-8% of which Nephrotic Syndrome constitutes about 35-40% (Bhatta NK 2008, Yadav SP 2016) Definition Manifestation of glomerular disease, characterized by nephrotic range proteinuria and a triad of clinical findings associated with large urinary losses of protein : A. Hypoalbuminaemia (S. Albumin less than 2.5 gm/dl) B. Edema and C. Hyperlipidemia (S. Cholestrol more than 200 mg/dl) Nephrotic Range Proteinuria Protein excretion of more than 40 mg/m2/hr; or, Protein excretion of more than 1 gm/m2/day; or, Spot urine protein : creatinine ratio of more than 2: 1; or, Protein dipstick more than or equal to 3+ Aetiology Primary/Idiopathic (80%) Minimal Change disease ( more than 80 % ) • Mesangial proliferation • Focal segmental Glomerulosclerosis • Membranous Nephropathy Membranoproliferative glomerulonephritis Secondary • Infectious – Hepatitis (B,C) , HIV-1, Malaria, Syphilis, Toxoplasmosis • Inflammatory – Glomerulonephritis • Immunological – Bee sting, Food allergens • Neoplastic – Lymphoma, Leukemia • Traumatic ( Drug induced ) – Penicillamine, Gold, NSAIDS, Pamidronate, Mercury, Lithium Clinical Manifestations Common age of presentation 1-5 yrs Anasarca, Decrease Urine output, Frothy Urine Growth and Blood Pressure Normal Investigations: Initial episode Urine albumin (3+ or 4+) Serum albumin (less than 2.5 g/dL) Serum cholesterol (more than 200 mg/dL) Urinalysis: Urine albumin 3+/4+, Microscopic hematuria – 30%, Few WBCs CBC Blood urea, creatinine C3 in children with gross hematuria Mantoux, Chest X Ray If necessary, HBsAg, ANA TERMINOLOGY REMISSION: Urine protein trace/nil or less than 4mg/m2/hr for 3 days RELAPSE: Urine protein 3+/4+or more than 40mg/m2/hr for 3 days, patient previously being in remission FREQUENT RELAPSER: 2 or more relapses in initial 6 months or 4 or more relapses in any 12 month period STEROID DEPENDENCE: 2 consecutive relapses when on alternate day steroids or within 14 days of its discontinuation STEROID RESISTANCE: absence of remission despite therapy with daily prednisolone (2mg/kg/day) for 4 wks Indications of Renal Biopsy AT ONSET: Age less than 1yr Gross hematuria, persistent microscopic hematuria, low C3 level Sustained hypertension Renal failure not attributable to hypovolemia Suspected secondary cause AFTER INITIAL TREATMENT: -Proteinuria persisting despite 4 wks of daily steroid -Before starting Rx with CsA, Tacrolimus Management of Patients with 1st Episode Nephrotic Syndrome First Episode of NS (No atypical features/Infection). Prednisolone 2 mg/kg daily X 6 weeks Prednisolone 1.5 mg/kg on Alternate Day X 6 weeks Treat any infection. Prednisolone 2 mg/kg/day till remission Prednisolone 1.5mg/kg alternate day X 4 weeks Usual duration of treatment for relapse is 5-6 weeks FRNS and SDNS Not necessary to perform renal biopsy in these cases Alternative agents advised if Prednisolone threshold to maintain remission is higher Features of steroid toxicity are seen ALTERNATIVE THERAPY CHOICE OF AGENT Levamisole – first line for FRNS & SDNS Cyclophosphamide preferred: -Significant steroid toxicity -Severe relapse with episode of hypovolemia/ thrombosis -Poor compliance & difficult to follow up Cyclosporine A– if continues to show steroid dependence/ frequent relapse or in SRNS despite treatment Tacrolimus- preferred in Adolescents, same action as CysA (Pediatric nephrology group , Indian Pediatrics , 2008) CONTROL OF EDEMA Examine for hypovolemia Tab Fursemide 1-3mg/kg/day may add spironolactone 2-4 mg/kg/day Double dose of oral fursemide or maximum daily dose of fursemide 4-6 mg/kg/day is reached Add Hydrochlorthiazide 1-2mg/kg/day or metalazone 0.1-0.3 mg/kg/day Fursemide IV bolus 1-3 mg/kg/dose or infusion 0.1-1mg/kg/hr 20% Albumin 1gm/kg iv followed by iv fursemides Supportive therapy: Diet adequate proteins and calories for age; fats less than 30% of calories Adequate fluid intake Salt restriction in edematous state Immunisation Avoid live vaccines while on steroid therapy; can be give 4 weeks after stopping steroids Monitoring of urine albumin Counseling Relapse is a rule Time to remission Prolonged course of first therapy, compliance Side effects of drugs Risk of FR/SDNS No risk of renal failure Music credit: Aakash Gandhi - lifting dreams @DIP - Medical Videos | 2020 #nephrotic_syndrome #pedia #dip_medical_videos
