Rabson-Mendenhall syndrome

Overview

A very rare genetic disorder involving severe insulin resistance which is where the body fails to be able to use insulin which is needed to control blood sugar levels.

Symptoms

* Thick hair * Severe insulin resistance * Rapid hair growth * Abnormal teeth * Abnormal nails * Acanthosis nigricans * Accelerated growth * Enlarged phallus * Precocious pseudopuberty * Short stature * Thick skin * Dry skin * Insulin dependent diabetes * Hyperglycemia

Causes

* obesity (even an increased percentage of body fat primarily in the abdominal region); risk decreases with weight and drug therapy * lack of physical activity * history of GDM * hypertension

Treatment

Treatment of Rabson-Mendenhall syndrome with pharmacologic doses of human leptin may result in improvement of fasting hyperglycemia, hyperinsulinemia, basal glucose, and glucose and insulin tolerance