Mitochondrial cytopathy presenting with focal segmental glomerulosclerosis, hypoparathyroidism, sensorineural deafness, and progressive neurological disease

A 6 year old boy who presented with steroid unresponsive nephrotic syndrome is reported. He was found to have focal segmental glornerulosclerosis and associated hypoparathyroidism and sensorineural deafness. The child progres sed to end stage renal failure and was successfully managed by dialysis and cadaveric renal transplantation. He later developed progressive neurologic al deterioration and mitochondrial myopathy and neuropathy was diagnosed.