Catalog

Rabson-Mendenhall syndrome

No MMS code

Link to WHO Definition

Rabson-Mendenhall syndrome – http://id.who.int/icd/entity/1018973126

Rabson-Mendenhall syndrome belongs to the group of extreme insulin-resistance syndromes. Early onset is marked by intrauterine and postnatal growth retardation, hypotrophy of muscle and adipose tissues, acanthosis nigricans (a skin lesion associated with extreme insulin resistance), dental dysplasia, abnormal hair and nails, hirsutism, and acromegaloid facies. A hypertrophic pineal gland has been reported in some cases. Biologically, infants display fasting hypoglycaemia, postprandial hyperglycaemia and hyperinsulinemia, which progress to permanent hyperglycaemia and recurrent diabetic ketoacidosis.