The following are the hallmarks of nephrotic syndrome in children except
High-Yield Explanation
NEPHROTIC SYNDROME:-
Characterised by massive proteinuria, hypoalbuminemia and oedema, hyperlipidemia is often associated.
Some patients show hematuria and hypertension.
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 oedema 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.
The 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 show deposits of occasional mesangial IgM.
Lab findings- urine examination shows heavy proteinuria. Hyaline and granular casts are present. Serum albumin is low. Hypercholesterolemia imparts 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 syndrome:-
Homozygous or complete heterozygous mutations in genes encoding podocyte proteins, including podocin(NPHS2), nephrin(NPHS1) and Wilms a tumour (WT1) genes.