Adiposogenital dystrophy


Adiposogenital dystrophy

Description, Causes and Risk Factors:

A disorder characterized primarily by obesity and hypogonadotropic hypogonadism in adolescent boys; dwarfism is rare, and when present is thought to reflect hypothyroidism. Visual loss, behavioral abnormalities, and diabetes insipidus may occur. Adiposogenital dystrophy often is used synonymously for this disorder. Although the original case involved a pituitary tumor, most cases are thought to result from hypothalamic dysfunction in areas regulating appetite and gonadal development. The most common causes are pituitary and hypothalamic neoplasms.

Adiposogenital dystrophy is a condition which may be caused by secondary hypogonadism originating from decreased levels in (Gonadotropin-releasing hormone) GnRH. Low levels of GnRH has been associated with defects of the feeding centers of the hypothalamus, leading to an increase consumption of food and thus caloric intake.

adiposogenital dystrophy

Adiposogenital dystrophy is usually the result of lesions in the hypothalamic gland, the endocrine gland that produces substances that stimulate the pituitary and regulate the appetite. In some cases of adiposogenital dystrophy, the front portion (anterior) of the pituitary gland fails to secrete the hormones that are necessary for the onset of normal puberty. A diseased area (lesion) in the hypothalamus or pituitary is the usual cause of the syndrome. A tumor of the pituitary that is frequently the cause of damage to the hypothalamus in children and adolescents is an expanding hollow (cystic) lesion (craniopharyngioma).

Inflammation from an infection such as tuberculosis or an acute inflammation of the brain (encephalitis) are other injuries that may be responsible for the lesion and, hence, the condition.

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.

Symptoms:

Signs and symptoms of adiposogenital dystrophyinclude obesity, small testes, delay in the onset of puberty, short stature (compared to other family members of the same sex), malformed or undersized fingernails, and headaches. Some children with adiposogenital dystrophymay have mental retardation, difficulties with vision, and in rare cases diabetes. Other symptoms of the syndrome may include excessive thirst, excessive urination, and very delicate skin.

Diagnosis:

Laboratory analysis of the urine from children with adiposogenital dystrophytypically reveals low levels of pituitary hormones, and that finding may suggest the presence of a lesion on the pituitary. Additional tests are needed before a definite diagnosis of adiposogenital dystrophymay be made.

Treatment:

Pituitary extracts may be administered to replace the missing hormones HRT (hormonal replacement therapy) in patients with adiposogenital dystrophy. Tumors of the hypothalamus should be surgically removed if possible. Appetite may be very difficult to manage, although weight control depends on this.

Neurosurgeons in Boston area have reported good results in the treatment of adiposogenital dystrophy by means of relatively new stereotactic-planning (3-dimensional) of a transnasal surgical procedure to remove small tumors in the pituitary gland. Because the number of patients involved was small, much more study of this procedure is needed to determine its safety and usefulness in treating individuals affected by adiposogenital dystrophy.

NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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