Rabson-Mendenhall syndrome

A rare syndrome that belongs to the group of extreme insulin-resistance syndromes (which also includes leprechaunism, the lipodystrophies, and the type A and B insulin resistance syndromes).

Hirsutism

Abnormally increased hair growth referring to a male pattern of body hair (androgenic hair).


Total: 2

                      


(per page)
PMID (PMCID)
26438710
MALE
Case Report: When an Induced Illness Looks Like a Rare Disease.
Rabbone I, Galderisi A, Tinti D, Ignaccolo MG, Barbetti F, Cerutti F.
Pediatrics. 2015;136(5):e1361-5.
This hypothesis was confirmed by a video recording that revealed the administration by the mother of multiple drugs (insulin, glyburide, progesterone, and furosemide) that mimicked most of the features of Rabson-Mendenhall syndrome, including hirsutism and hypoglycemia with coincident, inappropriately normal c-peptide values due to the administration of the insulin secretagogue.
16127220
FEMALE Child
A nonsense mutation in the Arg345 of the insulin receptor gene in a Japanese type A insulin-resistant patient.
Hashiramoto M, Osawa H, Ando M, Murakami A, Nishimiya T, Nakano M, Nishida W, Onuma H, Makino H.
Endocr J. 2005;52(4):499-504.
We have studied a 10-year-old Japanese girl with type A insulin resistance with hirsutism and hyperinsulinemia but without the dysmorphic features characteristic of leprechaunism or Rabson-Mendenhall syndrome.