Rabson-Mendenhall syndrome in a 13 y.o. child (clinical case)
Abstract
We present a case of a teenage boy with a Rabson-Mendenhall syndrome. There are only few recent publications on the topic of Rabson-Mendenhall syndrome in medical literature. This syndrome appears with a same frequency in both sexes. The disease prevalence is still unknown due to many undiagnosed cases linked with high mortality in early childhood. There are no prenatal screening for this disease till now, but it is clear, that in case of positive diagnostic tests, abortion should be recommended.
The typical clinical symptoms of Rabson-Mendenhall syndrome are the following: physical development delay, loss of subcutaneous fat, teeth and nails abnormalities (premature teeth eruption, teeth number doubling, nails thickening). The earliest signs of the syndrome are skin hyperpigmentation and hyperkeratosis at neck, armpits and groin, which are typical for insulin resistance.
Children with Rabson-Mendenhall syndrome usually have early manifestation of diabetes mellitus, characterized by labile disease course and frequent ketoacidosis state.
The present clinical case might be interesting regarding a long follow-up of the child.
The typical clinical symptoms of Rabson-Mendenhall syndrome are the following: physical development delay, loss of subcutaneous fat, teeth and nails abnormalities (premature teeth eruption, teeth number doubling, nails thickening). The earliest signs of the syndrome are skin hyperpigmentation and hyperkeratosis at neck, armpits and groin, which are typical for insulin resistance.
Children with Rabson-Mendenhall syndrome usually have early manifestation of diabetes mellitus, characterized by labile disease course and frequent ketoacidosis state.
The present clinical case might be interesting regarding a long follow-up of the child.
About the Authors
Roza Arturovna Atanesyan
Stavropol State Medical University, Stavropol
Russian Federation
MD, Assistant researcher of the Therapy department
Russian Federation
MD, Assistant researcher of the Therapy department
Leonid Yakovlevich Klimov
Stavropol State Medical University, Stavropol
Russian Federation
MD, PhD, Associate Professor, Cheif of the Pediatric Chair
Russian Federation
MD, PhD, Associate Professor, Cheif of the Pediatric Chair
Tatiana Michailovna Vdovina
Stavropol State Medical University, Stavropol
Russian Federation
Russian Federation
Tatiana Alexeevna Uglova
Stavropol State Medical University, Stavropol
Russian Federation
MD
Russian Federation
MD
Victoria Alexandrovna Kuryaninova
Stavropol State Medical University, Stavropol
Russian Federation
MD, Assistant researcher of the Pediatric department
Russian Federation
MD, Assistant researcher of the Pediatric department
Lilit Samvelovna Alaverdyan
Stavropol State Medical University, Stavropol
Russian Federation
MD
Russian Federation
MD
Elena Ivanovna Andreeva
Stavropol State Medical University, Stavropol
Russian Federation
MD, PhD, Associate professor of the Endocrinology and diabetology department
Russian Federation
MD, PhD, Associate professor of the Endocrinology and diabetology department
Galina Alexandrovna Saneeva
Stavropol State Medical University, Stavropol
Russian Federation
MD, PhD, Associate professor of the Endocrinology and diabetology department
Russian Federation
MD, PhD, Associate professor of the Endocrinology and diabetology department
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For citations:
Atanesyan R.A., Klimov L.Ya., Vdovina T.M., Uglova T.A., Kuryaninova V.A., Alaverdyan L.S., Andreeva E.I., Saneeva G.A. Rabson-Mendenhall syndrome in a 13 y.o. child (clinical case). Diabetes mellitus. 2015;18(1):110-114. (In Russ.)
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