Adiposogenital Dystrophy
Encyclopedia
Adiposogenital dystrophy is a condition which may be caused by secondary hypogonadism
originating from decreased levels in 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.
It is usually associated with tumours
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
.
Hypogonadism
Hypogonadism is a medical term for decreased functional activity of the gonads. Low testosterone is caused by a decline or deficiency in gonadal production of testosterone in males...
originating from decreased levels in 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.
Presentation
It is characterized by:- Feminine obesityObesityObesity is a medical condition in which excess body fat has accumulated to the extent that it may have an adverse effect on health, leading to reduced life expectancy and/or increased health problems...
- Growth retardation and retarded sexual development, atrophyAtrophyAtrophy is the partial or complete wasting away of a part of the body. Causes of atrophy include mutations , poor nourishment, poor circulation, loss of hormonal support, loss of nerve supply to the target organ, disuse or lack of exercise or disease intrinsic to the tissue itself...
or hypoplasiaHypoplasiaHypoplasia is underdevelopment or incomplete development of a tissue or organ. Although the term is not always used precisely, it properly refers to an inadequate or below-normal number of cells. Hypoplasia is similar to aplasia, but less severe. It is technically not the opposite of hyperplasia...
of the gonads, and altered secondary sex characteristics, - headaches
- Problems with visionVisual perceptionVisual perception is the ability to interpret information and surroundings from the effects of visible light reaching the eye. The resulting perception is also known as eyesight, sight, or vision...
- polyuriaPolyuriaPolyuria is a condition usually defined as excessive or abnormally large production or passage of urine . Frequent urination is sometimes included by definition, but is nonetheless usually an accompanying symptom...
, polydipsiaPolydipsiaPolydipsia is a medical symptom in which the patient displays excessive thirst. The word derives from the Greek πολυδιψία, which is derived from πολύς + δίψα...
.
It is usually associated with tumours
Tumor
A tumor or tumour is commonly used as a synonym for a neoplasm that appears enlarged in size. Tumor is not synonymous with cancer...
of the hypothalamus
Hypothalamus
The Hypothalamus is a portion of the brain that contains a number of small nuclei with a variety of functions...
, causing increased appetite
Appetite
The appetite is the desire to eat food, felt as hunger. Appetite exists in all higher life-forms, and serves to regulate adequate energy intake to maintain metabolic needs. It is regulated by a close interplay between the digestive tract, adipose tissue and the brain. Decreased desire to eat is...
and depressed secretion
Secretion
Secretion is the process of elaborating, releasing, and oozing chemicals, or a secreted chemical substance from a cell or gland. In contrast to excretion, the substance may have a certain function, rather than being a waste product...
of gonadotropin
Gonadotropin
Gonadotropins are protein hormones secreted by gonadotrope cells of the pituitary gland of vertebrates. This is a family of proteins, which include the mammalian hormones follitropin , lutropin , placental chorionic gonadotropins hCG and eCG and chorionic gonadotropin , as well as at least two...
. 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
Puberty
Puberty is the process of physical changes by which a child's body matures into an adult body capable of reproduction, as initiated by hormonal signals from the brain to the gonads; the ovaries in a girl, the testes in a boy...
.
Synonyms
It has several other names:- Babinski-Fröhlich syndrome (named after Joseph BabinskiJoseph BabinskiJoseph Jules François Félix Babinski was a French neurologist of Polish descent. He is best known for his 1896 description of the Babinski sign, a pathological plantar reflex indicative of corticospinal tract damage....
and Alfred FröhlichAlfred FröhlichAlfred Fröhlich was an Austrian-American pharmacologist and neurologist born in Vienna.In 1895 he graduated from the University of Vienna, and afterwards remained at Vienna as an assistant to Carl Nothnagel...
, but probably first described by MorgagniGiovanni Battista MorgagniGiovanni Battista Morgagni was an Italian anatomist, celebrated as the father of modern anatomical pathology.-Education:...
). (It was given its name by Harvey CushingHarvey CushingHarvey Williams Cushing, M.D. , was an American neurosurgeon and a pioneer of brain surgery, and the first to describe Cushing's syndrome...
.) - Froelich's syndrome
- Frölich's Syndrome
- Hypothalamic Infantilism-Obesity
- Launois-Cleret Syndrome
- Sexual Infantilism
See also
- Prader-Willi syndromePrader-Willi syndromePrader–Willi syndrome is a rare genetic disorder in which seven genes on chromosome 15 are deleted or unexpressed on the paternal chromosome...
, which it might resemble. However, this is a genetic disorderGenetic disorderA genetic disorder is an illness caused by abnormalities in genes or chromosomes, especially a condition that is present from before birth. Most genetic disorders are quite rare and affect one person in every several thousands or millions....
.