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Published on : Aug 02, 2021 | Views : 4260 | Downloads : 25

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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)
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Outline Introduction Epidemiology Classification & Etiology Pathophysiology Clinical features Investigations & treatment Complications & prognosis 1.
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Introduction ▰Syndrome caused by renal diseases that increase the permeability across the glomerular filtration barrier. ▰Characterized by a tetrad of clinical features 2.
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Contd… 1. Nephrotic range proteinuria – Urinary protein excretion > 50 mg/kg/day. 3. Hypoalbuminemia – Serum albumin concentration < 3g/dl (30g/l). Hyperlipidemia Edema.
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Epidemiology ▰Occurs at all ages but most prevalent in children 1.5-6 years. 4. ▰Boys > girls, 2:1 ratio. ▰Higher in underdeveloped countries (South east Asia and Africa). ▰Incidence worldwide, 2 – 7 cases per 100,000 children/year.
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Classification & Etiology 1. Primary No identifiable systemic disease. These are patients with; Idiopathic NS – No glomerular inflammation on renal biopsy. Primary glomerulonephritis – Active sediment and glomerular inflammation on biopsy. 5.
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Contd… 6 . 2. Idiopathic Minimal Change Disease (MCD) - 85% Focal Segmental Glomeruloscrelosis (FSGSS) Membranoproliferative glomerulonephritis Mesangial proliferation Membranous nephropathy >80% of children with idiopathic NS are steroid sensitive.
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Contd… 3. Secondary 7. 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.
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Contd… Allergy – bee sting, cow’s milk, pollen, house dust. Drugs – NSAIDs, Ampicillin, penicillamine, gold, captopril and lithium. 8.
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Contd… 4. Hereditary NS 9. Below one year.
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6.
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Clinical features ▰Periorbital puffiness: more marked in the morning and later generalized 10. ▰Scrotal edema ▰Pleural effusion and Ascites are late features ▰Decreased urine output ▰Hypertension and hematuria are absent
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Clinical features ▰. 11.
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Clinical features ▰. 12.
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Clinical features ▰. 13.
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Clinical features ▰. 14.
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Investigations ▰Urinalysis 15. Proteinuria +3 or +4 Urinary protein excretion (>40mg/m2/hour) 24 hours urinary protein : creatinine > 3 Microscopic hematuria in 10% Pus cells in underlying UTI Cellular casts in other forms not MCD
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Contd… ▰Serum Albumin < 3g/dl Cholesterol > 250mg/dl ▰Others CBC usually normal, raised ESR CXR to R/O pleural effusion 16.
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Contd… 17 . ▰Renal biopsy (Indications) SRNS Frequent relapses Steroid toxicity Secondary NS Gross hematuria Hypertension VII. Renal insufficiency
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Treatment 18 . ▰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
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Treatment ▰Persistent +3-4 needs steroids 19. -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.
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Special definitions ▰Remission: trace/no protein in urine on dipstick for 3 consecutive days. 20. ▰ Relapse: urine protein is 3+/4+ for three consecutive days after having attained remission. ▰Frequent relapser: four or more relapses in 12 months.
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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.
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Treatment 22 . ▰Supportive management: ▰Diet Adequate in proteins and calories Fluid and salt restriction in edema ▰Hospitalization -Infections -Marked edema Monitoring BP in hypovolemia
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Treatment 23 . ▰Diuretics -massive ascites, pleural effusion and severe genital edema ▰Ambulation & Anti coagulants for thromboembolism ▰ACEI in hypertension ▰Calcium carbonate and vitamin D in Osteoporosis
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Complications ▰Infections 24. -SBP, Pneumonia, UTI -Sepsis -Cellulitis ▰Arterial and Venous Thrombosis ▰Steroids and other drugs adverse effects
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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.
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Contd… ▰Steroid responders 90% Minimal change disease 50% Mesangial proliferation 20% Focal sclerosis 26.
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Contd… ▰Poor prognostic factors 27. -Hematuria -Hypertension -Hypocomplementemia -Focal segmental sclerosis -Steroid resistance
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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.