Hypergonadotropic Hypogonadism with Partial Alopecia

Alternative Names

  • Al Awadi Farag Teebi Syndrome
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WHO-ICD-10 version:2010

Endocrine, nutritional and metabolic diseases

Disorders of other endocrine glands

OMIM Number

241090

Mode of Inheritance

Autosomal recessive

Description

Hypergonadotropic Hypogonadism with partial alopecia is a very rare condition that is associated with reduced function of the testes/ovaries and patchy hair loss. Insufficient production of sex hormones, in those affected, can result in abnormal sexual development. Affected individuals may also present with variable clinical symptoms such as, ataxia, deafness, diabetes, ocular and skin abnormalities.

Epidemiology in the Arab World

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Other Reports

Jordan

Al-Awadi et al. (1985) reported three siblings (two females and a male) with primary hypogonadism and partial alopecia. Both sisters had hypoplastic internal genitalia and the brother showed results consistent with germinal cell aplasia. Their parents were consanguineous.

Kuwait

See: Jordan > Al-Awadi et al., 1985.

Lebanon

Megarbane et al. (2003) described two sisters from a consanguineous family with primary hypogonadism, partial alopecia and other associated symptoms. The clinical features observed in this study were comparable to those reported by Al-Awadi et al. (1985) and suggested the occurrence of a founder mutation for hypogonadism with alopecia in the Middle-Eastern population.

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