同: 脂肪性器性異栄養症

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2017/03/02 02:22:26」(JST)

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Adiposogenital dystrophy is a condition which may be caused by tertiary hypogonadism originating from decreased levels in GnRH. Low levels of GnRH has been associated with defects of the feeding centers of the hypothalamus^{[citation needed]}, leading to an increased consumption of food and thus caloric intake.

Presentation

It is characterized by:

Obesity
Growth retardation and retarded sexual development, atrophy or hypoplasia of the gonads, and altered secondary sex characteristics,
Headaches
Problems with vision
polyuria, polydipsia.

It is usually associated with tumors 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

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).^{[citation needed]} (It was given its name by Harvey Cushing.)^[5] commonly associated with slipped capital femoral epiphysis
Froelich's syndrome
Frölich's Syndrome
Hypothalamic Infantilism-Obesity
Launois-Cleret Syndrome
Sexual Infantilism

References

^ National Organisation for Rare Disorders - Froelich's syndrome(dead link)
^ synd/1792 at Who Named It? - 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). 143 (4): 349–50. PMID 17969845.

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1. 筋強直性ジストロフィー：病因、臨床的特徴、および診断 myotonic dystrophy etiology clinical features and diagnosis
2. 心疾患関連する遺伝性症候群 inherited syndromes associated with cardiac disease
3. 遺伝的変異の概要 overview of genetic variation

English Journal

[Histiocytosis X--a retrospective analysis of 40 cases with localized or disseminated disease].

Kühl J, Kühner U, Ströder J.AbstractA retrospective analysis of 40 cases with histiocytosis X was undertaken to find out the course of primarily localized disease, and the prognosis of children with initially disseminated disease. Bone lesions recurred in nine of 23 children with localized histiocytosis X. In eleven cases other organ manifestations occurred as well; in four cases without bone relapse. After an observation period of 1-14 3/12 years, nine of 22 children in remission suffer from long-term sequelae like diabetes insipidus, convulsion, extrahypothalamic CNS-disease, orthopedic disability, growth retardation, dystrophia adiposogenitalis , and chronic headache. Four of 17 children with disseminated histiocytosis X died. Our results and others from the literature indicate various risk factors to be prognostically significant. 1) age less than 2 years 2) involvement of spleen and/or lung 3) elevated Lahey-score 4) dysfunction of the hematopoietic system, liver, and/or lung 5) histologic feature resembling malignant type 6) no response to therapy 7) severely affected general health. These factors can be evaluated initially. Considering our own experiences and some risk factors we suggest the definition of four risk groups: 1.) localized histiocytosis X of bone, lymph nodes or skin; 2.) disseminated histiocytosis X with benign histologic type and Lahey-score of one or two; 3.) Lahey-score of 3-8; 4.) disseminated histiocytosis X with dysfunction of certain organ systems and/or malignant histology.
Monatsschrift Kinderheilkunde : Organ der Deutschen Gesellschaft für Kinderheilkunde.Monatsschr Kinderheilkd.1984 Feb;132(2):88-95.
A retrospective analysis of 40 cases with histiocytosis X was undertaken to find out the course of primarily localized disease, and the prognosis of children with initially disseminated disease. Bone lesions recurred in nine of 23 children with localized histiocytosis X. In eleven cases other organ
PMID 6610108