systemic lipodystrophy
Introduction
Introduction to systemic lipodystrophy Total lipodystrophy (also known as adipose atrophic diabetes or Lawrence-Seip syndrome) is congenital or acquired lipoatrophy. Mainly manifested systemic subcutaneous fat and visceral fat atrophy, with visceral diseases or some congenital malformations, skin hairy, acanthosis-like lesions, systemic with liver, bone development, hyperlipidemia, diabetes and so on. basic knowledge The proportion of illness: 0.001% Susceptible people: no specific population Mode of infection: non-infectious Complications: yellow tumor
Cause
Causes of systemic lipodystrophy
(1) Causes of the disease
The cause is not clear, there is family history or genetic influence, it is autosomal recessive inheritance, may result in hyperlipidemia due to defects in fat storage, and some people speculate that it is related to hypothalamic-pituitary dysfunction.
(two) pathogenesis
The pathogenesis is still inaccurate. Some people think that it is an autosomal recessive inheritance, which may result in hyperlipidemia due to defects in fat storage.
Prevention
Systemic lipodystrophy prevention
Early detection, early treatment. Maintain a balanced diet and eat more fruits and vegetables.
Complication
Complications of systemic fat metabolism disorders Complications
Congenital type, usually hepatosplenomegaly, high blood lipids, yellow tumor formation, cardiac hypertrophy, renal function changes, mental insufficiency or hemiplegia, high blood sugar can occur at the age of 3, and can be accompanied by polyuria after 10 years old.
Symptom
Symptoms of systemic fat metabolism disorders Common symptoms Deciding lipid deposition, subcutaneous fat disappearance, cardiac hypertrophy, hepatosplem, most urinary hypertension, water and salt metabolism disorder
Divided into two types of congenital and adult.
Congenital type
Onset at birth or within 2 years of age, the expression of subcutaneous fat disappeared, the skin became dry, but the elasticity is good, extensive pigmentation, underarms, groin folds are most prominent, local skin is linear thickening, similar to acanthosis nigricans, whole body Hairy, hair thick and curly, the forehead hairline can reach the eyebrow bow, the bone develops faster, the height exceeds the same age standard, the hand and foot joints increase, the head becomes longer, the face is thin, the lower forehead, the tibia protrudes, characteristic Face, body muscle shape is strong, stomach muscle thickening, abdominal swelling, often accompanied by umbilical hernia, sexual organ premature hypertrophy, usually hepatosplenomegaly, high blood lipids, skin formation of yellow tumor, cardiac hypertrophy, renal function changes, mental insufficiency or Hemiplegia, hyperglycemia can occur at 3 years of age, and can be accompanied by polyuria after 10 years of age, but does not respond to insulin therapy.
2. Adult type
Symptoms are late or not prominent, height, muscles are large, abdominal swelling is not obvious, before the onset of diabetes, only the growth of the hand and foot bones can be seen, similar to acromegaly, the test can find hyperglycemia, hyperlipidemia, urine Protein and cast, some have high blood pressure, large liver, heart hypertrophy, often died of liver failure or hematemesis.
Examine
Examination of systemic fat metabolism disorders
Histopathology: Subcutaneous and visceral fat disappeared, and electron microscopy showed that fat cells contained many fat droplets.
Diagnosis
Diagnosis and identification of systemic lipodystrophy
According to the characteristics of systemic insulin disease and ketosis-free diabetes, the diagnosis can be confirmed.
Acromegaly needs to be distinguished from the adult type of the disease. The former is caused by pituitary tumor or hyperplasia. In addition to acromegaly, there are progressive pre-headaches, reduced visual field, and decreased vision, which are different from this disease.
