Dietary Management in Slowing Down the Progression of CKDu.

Chronic kidney disease of unknown etiology (CKDu) is an emerging entity in the South Asian region. This predominately affects the farming community belonging to the lower socioeconomic status. CKDu being a progressive condition often leads to end-stage renal failurerequiring renal replacement therapy (RRT).

A rare case of autosomal recessive ATP6V0A4 variant of distal renal tubular acidosis in a young female with recurrent nephrolithiasis.

Homozygous autosomal recessive distal renal tubular acidosis (dRTA) is a rare entity. The intercalated cells in the collecting ducts are defective in apical proton secretion or basolateral bicarbonate reabsorption, due to mutations in genes encoding for proteins in a4 and B1 subunits of the V-ATPase and the anion exchanger Cl/HCO (kAE1). This results in decreased ammonium (NH) excretion and defective urine acidification.