Cataract caused by myleran toxicity

A case of cataract related to Busulfan [Myleran] was studied with light and electron microscopy. Clinical evaluation revealed bilateral posterior subcapsular cartaracts. Histological abnormalities included the presence of numerous abnormal dense bodies and Morgagnian globules in the anterior subcapsular region with marked cellular destruction and accumulation of granular debris posteriorly. Electron microscopy revealed that abnormal dense inclusions resembled myelinoid bodies. Acid phosphatase activity was localized in the epithelium and was hardly visible in the area of cellular necrosis. It is felt that the myleran - induced disturbance of polar lipid catabolism was responsible for cataract formation this case

DIDMOAD syndrome [Wolfram syndrome] with megacystis, megaureter and hydronephrosis

A case of DIDMOAD syndrome [diabetes insipidus, diabetes mellitus, optic atrophy, and nerve deafness] occurring as an autosomal recessive disorder is described. The patient presented with diabetes mellitus, diabetes insipidus, severe urinary tract dilatation, and asymptomatic involvement of optic and auditory nerves. The diabetes insipidus had both neurogenic and nephrogenic components. A marked reduction in urinary output was demonstrated following the administration of desmopressin. The possible factors that lead to functional obstruction with consequent urinary tract dilatation are discussed. Bladder neck resection was performed and an ileal conduit was constructed. Following the surgery there was an improvement in glomerular function