Preferred Label : Rabson-Mendenhall syndrome;
ICD-11 definition : 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 hypoglycemia, postprandial hyperglycemia and
hyperinsulinemia, which progress to permanent hyperglycemia and recurrent diabetic
ketoacidosis.;
Origin ID : 1018973126;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
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 hypoglycemia, postprandial hyperglycemia and
hyperinsulinemia, which progress to permanent hyperglycemia and recurrent diabetic
ketoacidosis.
