Thin lower body type
Introduction
Introduction Lower body weight loss refers to the clinical symptoms of lower body weight loss caused by diseases such as progressive lipodystrophy. It is one of the clinical symptoms of progressive lipodystrophy. Progressive lipodystrophy (also known as head and chest fat malnutrition; Simons disease; Seip-Laurence syndrome). It is a rare clinical and histological feature of autonomic nervous system disease characterized by adipose tissue metabolic disorders. It is characterized by a slow, progressive bilateral distribution with a substantially symmetrical boundary, atrophy or disappearance of subcutaneous adipose tissue, sometimes combined with limited adipose tissue hyperplasia, hypertrophy. Because of the range of fat atrophy, it can be divided into localized lipodystrophy (Simons disease or head and chest fat malnutrition) and systemic lipodystrophy (Seip-Laurence syndrome).
Cause
Cause
The etiology of this disease is unclear, more common in women, more than 5 to 10 years old before and after onset, often symmetric distribution, slow progress. Strengthen exercise, eat more fruits and vegetables to supplement vitamins to facilitate the recovery of this disease. It is generally considered to be an abnormal disease of fat metabolism associated with autonomic nerves.
Pathogenesis
Mainly related to hypothalamic lesions and post-ganglionic sympathetic neuropathy associated with spinal nerves. The hypothalamus plays a regulatory role in gonadotropins, thyroid stimulating hormones and other endocrine glands, and has an anatomical close relationship with post-ganglionic sympathetic nerve fibers. There seems to be a boundary between the dystrophic adipose tissue disappearance zone and the normal zone or the obese zone. This boundary has a certain relationship with the spinal cord segment. Usually, the body is divided into two parts, the upper body and the lower body, with the waist 1 to 2 as the boundary.
Another study suggests that the hypothalamic and pituitary components constitute a metabolic regulation control system. The fat disappearance and the promotion of the fat transfer factor produced by the system may have a history of acute fever and endocrine defects such as hyperthyroidism, hypopituitarism and Encephalitis, etc., mental factors of injury, early menstruation and pregnancy can be incentives. The current view of the role of genetic factors has not been unified.
Examine
an examination
Related inspection
Testosterone thyroid-binding globulin (TBG) thyroxine (T4) thyroid iodine test urinary testosterone
According to the disappearance of subcutaneous adipose tissue, the normal tissue of muscle and bone is examined for the disappearance of adipose tissue, and the occurrence of subcutaneous fat loss and normalization can be confirmed by combining in different ways. It can be diagnosed according to its clinical symptoms.
Clinical manifestation
1. Most patients are more common in women who are onset from 5 to 10 years old. The onset and progress are slower. Patients with early onset of the disease often have facial or upper extremity adipose tissue disappearing, and then expand downward, involving the buttocks and thighs, showing general symmetry. distributed. The course of the disease lasts for 2 to 6 years and can be stopped by the patient. The facial expression of the patient's cheeks and ankles is loose and loses normal elasticity. The fat around the cheeks and eyelids disappears, so that the patient presents a special face. The patient's buttocks and hips may have obvious subcutaneous. Tissue hyperplasia, hypertrophy, but the hands and feet are often unaffected.
2. The patient can be characterized by the disappearance of adipose tissue, special obesity and normal adipose tissue, which are combined with the basic characteristics of cost sickness in different ways. According to different combinations, it can be expressed as the following types:
1 upper body normal lower body obesity;
2 upper body weight loss lower body obesity;
3 simple upper body weight loss type;
4 upper body obese;
5 lower body weight loss type;
6 body weight loss type;
7 half-length obese.
3. Patients can be combined with skin moisture changes, sweating abnormalities, decreased urine glucose tolerance, tachycardia vascular instability, vascular headache, abdominal pain, vomiting skin and nail nutrition disorders, and other autonomic dysfunction, individual cases can be combined with endocrine function Barriers, such as genital dysplasia, thyroid dysfunction, acromegaly and menstrual disorders. Symptoms tend to be stable within 5 to 10 years after onset.
4. The patient's muscles, bone hair, breast and sweat glands are normal, no muscle disorders, most patients' physical strength is not affected, and the physical and mental development during the course of the disease is not affected. Recently reported that Hodgkin's disease and scleroderma can be complicated.
5. Neonatal or infant patients with congenital systemic and multiple organ lesions, in addition to the head, face and neck, trunk and limbs, subcutaneous and visceral peripheral adipose tissue may also be accompanied by hyperlipidemia Hepatosplenomegaly, skin pigmentation and muscle hypertrophy.
Diagnosis
Differential diagnosis
The disease should be distinguished from the following diseases:
1. On the side of the lateral atrophy, the facial atrophy is progressive, and the skin, subcutaneous tissue and bone are all involved.
2. Localized muscular dystrophy such as face-shoulder-squat type manifests as facial muscle wasting and muscle strength is weakened. EMG indicates muscle damage and subcutaneous fat remains.
3. Excessive weight loss Most cases of excessive weight loss caused by various causes can detect causes such as malignant tumors, chronic infections and long-term gastrointestinal dysfunction.
Laboratory inspection:
Blood biochemistry may have low blood lipids and normal muscle enzymes
Other auxiliary inspections:
1. Skin and subcutaneous tissue biopsy showed subcutaneous adipose tissue atrophy and normal skin.
2. Electromyography shows normal muscle and nerves.
3. B-ultrasound can be found that the affected organs shrink and become smaller.
According to the disappearance of subcutaneous adipose tissue, the normal tissue of muscle and bone is examined for the disappearance of adipose tissue, and the occurrence of subcutaneous fat loss and normalization can be confirmed by combining in different ways. It can be diagnosed according to its clinical symptoms.
Clinical manifestation
1. Most patients are more common in women who are onset from 5 to 10 years old. The onset and progress are slower. Patients with early onset of the disease often have facial or upper extremity adipose tissue disappearing, and then expand downward, involving the buttocks and thighs, showing general symmetry. distributed. The course of the disease lasts for 2 to 6 years and can be stopped by the patient. The facial expression of the patient's cheeks and ankles is loose and loses normal elasticity. The fat around the cheeks and eyelids disappears, so that the patient presents a special face. The patient's buttocks and hips may have obvious subcutaneous. Tissue hyperplasia, hypertrophy, but the hands and feet are often unaffected.
2. The patient can be characterized by the disappearance of adipose tissue, special obesity and normal adipose tissue, which are combined with the basic characteristics of cost sickness in different ways. According to different combinations, it can be expressed as follows: 1 upper body normal lower body obesity; 2 upper body weight loss lower body obesity; 3 simple upper body weight loss type; 4 upper body obesity type; 5 lower body weight loss type; 6 body weight loss type; type.
3. Patients can be combined with skin moisture changes, sweating abnormalities, decreased urine glucose tolerance, tachycardia vascular instability, vascular headache, abdominal pain, vomiting skin and nail nutrition disorders, and other autonomic dysfunction, individual cases can be combined with endocrine function Barriers, such as genital dysplasia, thyroid dysfunction, acromegaly and menstrual disorders. Symptoms tend to be stable within 5 to 10 years after onset.
4. The patient's muscles, bone hair, breast and sweat glands are normal, no muscle disorders, most patients' physical strength is not affected, and the physical and mental development during the course of the disease is not affected. Recently reported that Hodgkin's disease and scleroderma can be complicated.
5. Neonatal or infant patients with congenital systemic and multiple organ lesions, in addition to the head, face and neck, trunk and limbs, subcutaneous and visceral peripheral adipose tissue may also be accompanied by hyperlipidemia Hepatosplenomegaly, skin pigmentation and muscle hypertrophy.
