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Partial destruction of hypothalamic nuclei resulting in hormonal dysfunction with obesity, growth retardation, and hypogonadism.

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Adiposogenital Syndrome; Dystrophia Adiposo-Genitalis; Neuropituitary Dystrophy; Cerebral Adiposity; Pituitary Infantilism of Adults; Fröhlich Syndrome; Fröhlich Obesity; Morbus Fröhlich Syndrome; Launois-Cléret Syndrome.

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Unknown, but male prevalence.

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Several organic lesions of the hypothalamus may cause this disorder, including tumors (craniopharyngioma), encephalitis, Friedreich ataxia, demyelinating diseases, and microcephaly. Originally described as delayed puberty, hypogonadism, and obesity associated with a tumor impinging on the hypothalamus. Presence of obesity indicates damage to the appetite-regulating regions of the hypothalamus. Hypothalamic dysfunction and hypopituitarism mainly affect the somatotropic hormones and the gonadotropins.

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Obesity with prepubertal onset affecting breasts, abdomen, femoral regions, and hips. Delayed development of secondary sexual characteristics. Headache, visual impairment, mental retardation. Polyuria and polydipsia as a result of diabetes insipidus.

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Based on the clinical and biochemical features (low serum gonadotropin levels; positive vasopressin test for polyuria, if diabetes insipidus is present). Radiologic examinations may show delayed ossification of skeletal structures, suprasellar calcification or destruction, depending on the etiology.

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Dehydration as a consequence of diabetes insipidus requires preoperative fluid replacement and check of serum electrolytes. The patient may be anxious and uncooperative because of mental retardation and visual disturbances. Sedative premedication and/or the presence of the primary care giver for induction of anesthesia may be helpful.

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Venous access can be difficult. Depending on the grade of obesity, a rapid sequence induction should be considered. Obesity and upper airway obstruction may render face-mask ventilation difficult.

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Prader-Labhart-Willi Syndrome: Infantile hypotonia, early childhood-onset obesity, hypogonadism, mental retardation.

Bruch H: The Froehlich's syndrome: Report of the original case. Am J Dis Child 58:1282, 1939.
Reichlin S: Neuroendocrinology, in Williams RH, Larsen RP (eds): Williams' Textbook of Endocrinology. 9th ed. Philadelphia, WB Saunders, 1998, p 165.

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