Rabson-Mendenhall Syndrome: Case Report
DOI:
https://doi.org/10.61651/rped.2012v65n1p38-41Keywords:
Insulin Resistance, Puberty, Precocious, Fetal Growth RetardationAbstract
The Rabson-Mendenhall Syndrome belongs to a group of syndromes of extreme insulin resistance. This is an extremely rare disorder of unknown prevalence. The syndrome is transmitted as an autosomal recessive trait and affects mainly children of consanguineous parents. It is a syndrome of early onset, characterized by intrauterine growth retardation and postnatal hypotrophy of muscle and adipose tissue, acanthosis nigricans, dental dysplasia, hypertrichosis, and pseudo- precocious puberty, hyperplasia phallic and acromegaloid facies. The prognosis of children with this syndrome is quite daunting. We report the case of a child treated at the Institute of Child Health. Our patient has almost all the features of the syndrome in question and the first report in Peru.
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