Disease Information for Adiposogenital dystrophy

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Clinical Manifestations
Signs & Symptoms
Anorexia in Children
Excessive thirst/polydipsia
Hyperphagia
Daytime Sleepiness
Drowsiness/somnolence
Lethargy/torpor
Diuresis/Large urine volumes
Polyuria
Polyuria and polydipsia
Short stature
Thirst Increased
Weight gain
Clinical Presentation & Variations
Presentation/Juvenile Cataract
Disease Progression
Course/Chronic disorder
Course/Chronic only
Demographics & Risk Factors
Population Group
Child
Population/Pediatrics population
Sex & Age Groups
Population/Child
Population/Child-Infant Only
Population/Children/all
Laboratory Tests
Abnormal Lab Findings - Decreased
LH/Leutinizing Hormone (Lab)
URINE FSH
Associated Diseases & Rule outs
Associated Disease & Complications
Adiposogenital dystrophy
Cataract
Deafness
Deafness, congenital
Deafness, sensorineural
Diabetes mellitus
Hereditary Blindness
Hypogonadism, male
Limb deformities/anomalies
Obesity
Obesity in Children
Retinitis pigmentosa
Syndactyly/Webbing
Diabetes mellitus/non-insulin dep/type II
Hand anomalies
Disease Mechanism & Classification
Class
CLASS/Pediatric disorders (ex)
CLASS/Endocrine gland (category)
CLASS/Genital, male (category)
Pathophysiology
Pathophysiology/Secondary Testicular Failure
Process
PROCESS/Congenital/developmental (category)
Synonyms
Synonym
adiposogenital dystrophy, Adiposogenital dystrophy (disorder), Adiposogenital dystrophy syndrome, adiposogenital syndrome, dystrophy adiposogenital, Froehlich Syndrome, Froehlichs Syndrome, Froehlich's syndrome, Frohlich, Fröhlich, Frohlich's syndrome, Hypothalamic infantilism with obesity syndrome, Launois Cleret syndrome, syndrome adiposogenital, Syndrome Froehlich's, Synonym/Froehlich's syndrome, Synonym/Infantilism, sexual organs (male), Synonym/Launois-Cleret syndrome
Definition

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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External Links Related to Adiposogenital dystrophy
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PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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