Rabson-Mendenhall syndrome | Zendy

Joel Gupta | Zendy; JonathanM Daniel | Zendy; Vijeev Vasudevan | Zendy

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Rabson-Mendenhall syndrome

Author(s) -

Joel Gupta,

JonathanM Daniel,

Vijeev Vasudevan

Publication year - 2012

Publication title -

journal of indian society of pedodontics and preventive dentistry

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 0.378

H-Index - 33

eISSN - 1998-3905

pISSN - 0970-4388

DOI - 10.4103/0970-4388.105026

Subject(s) - acanthosis nigricans , hyperinsulinemia , medicine , endocrinology , insulin receptor , insulin , insulin resistance

Rabson-Mendenhall syndrome is a rare, autosomal recessive disorder affecting insulin receptor. This disorder is characterized by insulin-resistant diabetes mellitus, hyperinsulinemia, deficiency of subcutaneous fat, acanthosis nigrican, growth retardation, coarse and senile appearance, precocious puberty, and dental prematurity, enlarged genitalia, and pineal hyperplasia. Mutations of the insulin receptor gene affecting insulin action appear to be the basic mechanism underlying this syndrome. Herein, we present a case report on Rabson-Mendenhall syndrome in a 9-year-old girl.

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