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Hepatic dysfunction in Alström disease.

Abstract

Alström disease is a rare disorder; less than 20 cases have been reported. An 11-year-old girl is described with this condition. She has pigmentary retinopathy, sensory neural deafness, obesity, Type II diabetes mellitus, hyperlipidemia, and acanthosis nigricans. However, in addition she developed hepatic dysfunction, pathologically similar to chronic active hepatitis. This may be a further, previously undescribed systemic manifestation of Alström disease.

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BibTeXRIS

M B Connolly, J E Jan, R M Couch, L T Wong, J E Dimmick, J M Rigg. 1991-09-15. Hepatic dysfunction in Alström disease.. https://doi.org/10.1002/ajmg.1320400408

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Acanthosis nigricans in children is usually associated with obesity. Generalized acanthosis nigricans in childhood is rare. We report a 13-year-old boy with an 8-year history of generalized hyperpigmentation and velvety thickening of the skin. Despite an extensive physical and laboratory examination no evidence of underlying endocrinological or neoplastic disease was found.

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