What is Lipodystrophy?
Lipodystrophy, otherwise referred to as lipoatrophy, is a medical condition referring to varied groups of metabolic disorders characterized by abnormal distribution or disintegration of adipose tissues in the body leading to partial or generalized loss of adipose tissues. Metabolic abnormalities such as diabetes mellitus, insulin resistance, hypertriglyceridemia, and hepatic steatosis are only some of those regularly observed from a person suffering from this disorder. The disease may lead to complications like acanthosis nigricans, polycystic ovary syndrome, hypertension and proteinuric kidney disease.
Lipodystrophy could either be inherited or acquired. At present, the most familiar type of lipodystrophy is under the acquired category which results from highly active antiretroviral therapy (HAART), which is a treatment used for HIV- infected individuals.
Types of Lipodystrophy
Lipodystrophies are categorized on either etiology – whether the disease was acquired or congenital – or through the pattern of adipose tissue loss which may be generalized (affecting the whole body) or partial (only concentrated in localized portion of the body). Etiological classifications of the disease are shown below:
Congenital Generalized Lipodystrophy (Berardinelli–Seip syndrome) – this kind of lipodystrophy is a rare metabolic condition inherited as an autosomal recessive disorder characterized by total or near absence of fat in the body. This is mostly evident at birth where the infant will seem to manifest a muscular appearance due to loss of adipose tissues in the body. Only the infant’s palms, sole of the feet and scalp are the once spared from fat loss.
Familial Partial LIpodystrophy – is a rare kind of genetic disorder transferred as an autosomal dominant trait. This may affect both males and females with 50% possibility of transferring the trait to their offspring. This may not be present at birth but could be characterized by loss of subcutaneous fat in the extremities but over accumulation of fat in the neck and face during childhood and puberty. Usually patients having this disorder develop buffalo lumps between their shoulder blades.
Acquired Partial Lipodystrophy (Barraquer-Simon’s Syndrome) – this kind of lipodystrophy is a rare form which has the possibility to affect women 3 times more than men. The onset of this condition starts from age 8-10 and is usually a result of a viral infection. This is characterized by loss of subcutaneous tissues from the face then progresses to the neck, shoulders, forearms, thoracic region up to the groin. Patients who suffered from this disorder usually develop kidney problem termed as mambranoploriferative glomerulonephritis 10 years after the onset of the disease. This condition could also be associated to autoimmune diseases such as systemic lupus erythematusus.
Acquired Generalized Lipodystrophy – this is a rare kind of lipodystrophy that is characterized by generalized loss of body fat. In this kind of lipodystrophy, patient may have normal amount of adipose tissues at birth and suddenly disappears during childhood or adolescence. Usually the onset of the disease occurs right after having been infected with varicella, measles, pertussis, diphtheria, pneumonia, osteomyelitis, parotitis, infectious mononucleosis, and hepatitis. Almost all areas of the body may be affected by adipose tissue degeneration. Patients may develop diabetes right after the onset of the disease.
Centrifugal Abdominal Lipodystrophy (Lipodystrophia Cenrtifugalis Abdominalis Infantalis) – this is a lipodystrophy is a disease for infants. This is characterized by abdominal or chest depression of infants which slowly enlarges centrifugally.
Lipoatrophia annularis (Ferriera-Marques lipoatrophia) – this is a lipodystrophy only manifested by women which is characterized by loss of adipose tissues in the upper extremities.
Localized Lipodystrophy – this kind of lipodystrophy usually results from insulin administration characterized by skin depression due to loss of subcutaneous fat in the injection site.
HIV- associated Lipodystrophy (HAL) – this lipodystrophy is common in patients suffering from HIV. There are no known cause of this condition but is associated to the administration of antiretroviral treatments such as HIV-1 protease inhibitors which interferes with lipid metabolism.
Since the disease results from different metabolic disorders, the symptoms of the disease may vary. Listed below are the common clinical manifestations of the disease:
- Partial or generalized loss of subcutaneous fat.
- Higher or over deposition of adipose tissues in other parts of the body usually in the face.
- Loss of body fat.
- Insulin resistance
- Diabetes, in some cases.
- Increased triglyceride levels.
- Fatty liver.
The cause of this disorder is still unknown up to the present but is commonly associated to some metabolic disorders. Due to these disorders fat degeneration follows.
There are no known treatments for the disease as of this moment. Usually, management of the disease focuses on the modification of the patient’s diet and exercise. Though diet modification does not really do much in treating the disease, it helps in prevention of the disease progression. Usually the diet given to patients having this disease are those diets recommended for patients with diabetes mellitus and with heart diseases as the disease usually progresses to DM and some cardiovascular diseases.
Although studies about exercise patterns show small benefits to the treatment of the disease, some exercise patterns provide significant results for patients with HALS. A combination of aerobic and resistance training exercises have shown considerable improvements of cholesterol and triglyceride levels, and body composition.
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