Case Reports

doi: 10.25005/2074-0581-2018-20-1-125-129
RABSON-MENDENHALL SYNDROME IN TWINS

N.F. Niyazova1, N.A. Inoyatova1, Sh. S. Anvarova1, M.N. Ismoilova2, Sh. T. Samieva1, L. Yu. Nuraliev1

1Department of Endocrinology, Avicenna Tajik State Medical University, Dushanbe, Republic of Tajikistan
2Department of Pediatric Endocrinology, City Medical Center № 1 named after Akhmedov Karim, Dushanbe, Republic of Tajikistan

Objective: The article presents a rare clinical case of twins with Rabson-Mendenhall syndrome. To this day, about 55 cases of this syndrome have been described in the literature. The exact incidence of the disease is unknown, which may be due to early prenatal mortality. The syndrome occurs in children of both sexes. The Rabson-Mendenhall syndrome characterized by the delay in physical development, anomaly of the development of teeth and nails, thinning of subcutaneous fat, dry skin. The earliest signs of the syndrome are hyperpigmentation and hyperkeratosis of the skin in the neck, armpits and groin areas, which are signs of severe insulin resistance. Diabetes mellitus develops in childhood, has a labile course, and accompanied by frequent signs of ketoacidosis and specific microvascular complications.

Keywords: Rabson-Mendenhall syndrome, insulin resistance, skin hyperpigmentation, hyperinsulinemia, hyperglycemia.

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Authors informations:

Niyazova Nargis Fazlikhudoevna, Candidate of Medical Sciences, Head of the Department of Endocrinology, Avicenna Tajik State Medical University

Inoyatova Nigina Azizovna, Candidate of Medical Sciences, Associate Professor of the Department of Endocrinology, Avicenna Tajik State Medical University

Anvarova Shakar Saidoromovna, Doctor of Medical Sciences, Full Professor, Professor of the Department of Endocrinology, Avicenna Tajik State Medical University

Ismoilova Mijgona Nodirovna, Head of the Department of Pediatric Endocrinology, City Medical Center № 1 named after Akhmedov Karim

Samieva Shahnoza Timurovna, Clinical Resident of the Department of Endocrinology, Avicenna Tajik State Medical University

Nuraliev Lukmon Yusufovich, Clinical Resident of the Department of Endocrinology, Avicenna Tajik State Medical University

Address for correspondence:

Niyazova Nargis Fazlikhudoevna

Candidate of Medical Sciences, Head of the Department of Endocrinology, Avicenna Tajik State Medical University

734003, Republic of Tajikistan, Dushanbe, Rudaki Ave., 139

Tel.: (+992) 919 022030

E-mail: nargis_7373@mail.ru