Adiposogenital dystrophy
| Adiposogenital dystrophy | |
| ICD-10 | E23.6 |
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| ICD-9 | 253.8 |
| DiseasesDB | 29318 |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Phone:617-632-7753
Synonyms and keywords:: Adiposogenital dystrophy; froehlich syndrome; frohlich syndrome; hypothalamic infantilism with obesity syndrome; launois-Cleret syndrome. It has several other names:[1]
- Babinski-Fröhlich syndrome[2] (named after Joseph Babinski[3] and Alfred Fröhlich,[4] but probably first described by Morgagni). (It was given its name by Harvey Cushing.)[5]
- Froelich's syndrome
- Frölich's Syndrome
- Hypothalamic Infantilism-Obesity
- Launois-Cleret Syndrome
- Sexual Infantilism
Presentation
It is characterized by:
- Feminine obesity
- Growth retardation and retarded sexual development, atrophy or hypoplasia of the gonads, and altered secondary sex characteristics,
- headaches
- mental retardation, problems with vision
- polyuria, polydipsia.
It is usually associated with tumours of the hypothalamus, causing increased appetite and depressed secretion of gonadotropin. It seems to affect males mostly.
Many overweight children may appear to have the disorder because of the concurrence of obesity and retarded sexual development; these children have no endocrine disturbances, however, and they mature normally after delayed puberty.
Synonyms
See also
- Prader-Willi syndrome, to which it might resemble. However, this is a genetic disorder.
References
- ↑ National Organisation for Rare Disorders - Froelich's syndrome
- ↑ Template:WhoNamedIt - Babinski-Fröchlich syndrome
- ↑ J. F. Babinski. Tumeur du corps pituitaire sans acromégalie et avec arrêt de développement des organes génitaux. Revue neurologique, Paris, 1900, 8: 531-535.
- ↑ A. Fröhlich. Ein Fall von Tumor der Hypophysis cerebri ohne Akromegalie. Wiener klinische Rundschau, 1901, 15: 833-836; 906-908.
- ↑ Zárate A, Saucedo R (2007). "[The adiposogenital distrophy or Frohlich syndrome and the beginning of the concept of neuroendocrinology]". Gac Med Mex (in Spanish; Castilian). 143 (4): 349–50. PMID 17969845.