Nephrotic syndrome in children is caused by
Question Category:
Correct Answer:
Minimal change disease
Description:
NEPHROTIC SYNDROME:- Characterised by massive proteinuria, hypoalbuminemua and edema, hyperlipidemia is often associated. Some patients show hematuria and hypeension. Heavy proteinuria( more than 1g/metre square/day) is the underlying abnormality leading to hypoalbuminemia ( serum albumin below 2.5g/dl). The resultant fall in plasma oncotic pressure leads to interstitial edema and hypovolemia. This stimulates the renin angiotensin aldosterone axis and ADH secretion that enhances sodium and water retention. Hypoalbuminemia also induces hepatic synthesis of beta lipoproteins resulting in hypercholesterolemia. Main cause of nephrotic syndrome in children is associated with minimal change disease. Other causes include amyloidosis, vasculitis, SLE, postinfectious glomerulonephritis, and hepatitis B nephropathy. Steroid sensitive nephrotic syndrome:- MCNS accounts for 80% cases of nephrotic syndrome. Electron microscopy shows non specific obliteration of epithelial foot processes. Immunofluorescence studies shows deposits of occassional mesangial IgM. Lab findings- urine examination shows heavy proteinuria. Hyaline and granular casts are present. Serum albumin is low. Hypercholesterolemia impa a milky appearance to plasma. Blood urea and creatinine values within normal range. Blood levels if IgG low and IgM elevated. Low serum calcium level. Steroid resistant nephrotic stndrome:- Homozygous or complete heterozygous mutations in genes encoding podocyte proteins, including podocin(NPHS2), nephrin(NPHS1) and wilms tumor(WT1) genes. Reference: GHAI essential Paediatrics
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