Lipoid proteinosis in two Indian brothers - a case report from South Bengal

Lipoid proteinosis is a rare autosomal recessive disorder with variable phenotype, caused by defect in extracellular matrix protein-1 and is characterized by deposition of periodic acid-Schiff-positive, diastase resistant material in skin, mucous membrane and internal organs. There are only few reports regarding lipoid proteinosis in literature and in this part of the world. Here, we report a case of lipoid proteinosis in a 29-year-old male with positive family history and widespread distribution involving skin and internal organs. Histopathological finding was consistent with clinical diagnosis of lipoid proteinosis

Effect metformin on urinary VMA levels in type II diabetic subjects

The purpose of this study was to observe the effect of metformin on 24-hours urinary VMA levels in newly diagnosed untreated type 2 diabetic subjects. The study consisted of four weeks for each participant with weekly follow up visits. Samples were collected at 0800-0900 hours after over night fast. Study was conducted in the Department of Pharmacology and Therapeutics, BMSI, JPMC, Karachi. Total duration of study was six months. Fifteen newly diagnosed untreated type 2 diabetics, with fasting plasma glucose levels >/= 126 mg/dl on two occasions and/or postprandial glucose levels >/= 200 mg/dl were enrolled in the study. Patients with concurrent illness or diabetic complications were excluded. Metformin was started from 500 mg/day and titrated at weekly intervals according to glycaemic control and the subjects tolerance to the drug. A 24-hour urinary VMA was assessed at day - 0 [before metformin therapy] and day - 28 [4 weeks after metformin therapy] by using VMA reagent kit of Biosystems Spain on Spectronic -21 spectrophotometer USA. Metformin caused highly significant [P < 0.001] reduction in mean fasting plasma glucose from 233.33 +/- 15.62 mg /dl on day-0 to 151.53 +/- 6.02 mg/dl on day - 28, and a significant [P < 0.01] decrease in 24 - hour urinary VMA levels from 5.18 + 0.50 mg / 24 hours on day-0 to 3.32 + 0.28 mg / 24 hours on day-28. Our results indicate that metformin causes highly significant reduction in fasting plasma glucose and a significant decrease in 24 - hour urinary VMA levels in newly diagnosed untreated type 2 diabetic subjects