Disease Information for Adiposogenital dystrophy

Froelich"s Syndrome; Adiposogenital Dystrophy; Babinski-Froelich Syndrome; Dystrophia Adiposogenitalis; Frolich"s Syndrome; Hypothalamic Infantilism-Obesity; Launois-Cleret Syndrome; Sexual Infantilism

Froehlich syndrome is a constellation of endocrine abnormalities believed to result from damage to the hypothalamus, a part of the brain where certain functions such as sleep cycles and body temperature are regulated; Froehlich syndrome appears to be acquired while certain other disorders that resemble it, such as Prader-Willi syndrome, are genetic; This syndrome appears to affect males mostly; The more obvious and frequently encountered characteristics are delayed puberty, small testes, and obesity; Teen-age boys with this disorder must be distinguished from those who have inherited growth delay disorders or Prader Willi syndrome--------[NORD 2005]-------------------------------------------------------------

Possibly disturbed gonadotropin secretion by anterior pituitary; in absence of pituitary lesions, primary disturbance probably in hypothalamus; possibly craniopharyngioma; USUALLY: hypogonadism; male;

SOMETIMES: mental retardation; vitiligo; USUALLY: chronic (> 4 weeks); SOMETIMES: amenorrhea; menstruation decrease; vision impairment; vision impairment, progressive; headache; sexual desire decreased; facial hair loss; hair loss; pubic hair loss; axillary hair loss;

MAKE DIAGNOSIS LESS LIKELY: very brief (< 6 hours);

USUALLY: sexual development retardation; genital abnormality; genital underdevelopment; obesity;

SOMETIMES: pectoral obesity; thigh obesity; genu valgum; joint abnormality; pelvis wide; polydactyly; skeletal deformity; syndactyly; cranial deformity; hypothermia; facial hair scantiness; hair scantiness; nail brittleness; pubic hair scantiness; skin atrophy; skin dryness; axillary hair scantiness; skin wrinkling; skin, delicate;

ADDITIONAL NOTES: Chiefly in prepubertal males; abdominal obesity; decreased pigmentation; USUALLY: bone age, delayed;

ADDITIONAL NOTES: Possibly delayed ossification; suprasellar calcification; STRONGLY SUPPORTS: genu valgum; hypogonadism; sexual development retardation; Prognosis: unfavorable; mental retardation; visual disturbances; possibly diabetes insipidus; usually stunting of growth

-------- [DxPlain/Merck Medicus 2005]-----------

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