lipodystrophy
Introduction
Introduction to lipodystrophy In 1885, Weir-Miechell first reported partial lipodystrophy (lipodystrophy), and later reported that the patient had neck, arms, chest and abdomen fat malnutrition, with increased fat deposition in the hips and legs, Lawrence reported in 1946 Complete fat dystrophy, patients with malnutrition are systemic, completely or patchy, and fat deficiency is often accompanied by a series of metabolic disorders. Fatty malnutrition is a rare disease and has only been reported in a few cases. The age of onset of lipodystrophy is between 1 and 40 years old, and occurs in girls aged 5 to 15. The incidence of renal damage is 15% to 30%. Because complete fat malnutrition often occurs simultaneously with diabetes, non-diabetic nephropathy is often found, and its pathological manifestations are indistinguishable from diabetic nephropathy without fat malnutrition. It has been reported in patients without diabetes, pathological manifestations of glomerulonephritis, fat The relationship between malnutrition and glomerular disease is unknown. Tuck et al reported that a small fraction of their capillaries have peripheral mesenteric implants, subepithelial and intramembranous deposits, but in general there is no special Characteristics, patient serum complement C3 levels are normal, no proteinuria, only a slight decrease in renal function. basic knowledge The proportion of illness: 0.015% Susceptible people: no specific population Mode of infection: non-infectious Complications: Diabetes, hirsutism, hepatomegaly, hyperlipidemia
Cause
Causes of lipodystrophy
(1) Causes of the disease
Congenital lipodystrophy is an autosomal recessive inheritance. Patients have a blood relationship; acquired people may have no genetic basis, and often have prodromal symptoms of viral infection. People think that acquired systemic and partial lipodystrophy are autoimmune diseases.
(two) pathogenesis
The pathogenesis of this disease is still unclear. Various hypotheses proposed in epidemiology, genetics and clinical research suggest that systemic lipodystrophy is associated with extensive metabolic and systemic abnormalities, and that sympathetic activity may be enhanced. Strengthen the decomposition of fat; pituitary may secrete fat mobilization substances, but pituitary resection failed to correct fat malnutrition, others found that CRF melanocyte release factor and FSH release factor increased, so the hypothalamus is considered to be the main damage location, there are Humans have proposed the autoimmune destruction theory of adipose tissue, but many patients have patchy lesions that seem difficult to explain with this theory. The relationship between the pathogenesis of renal lesions and lipodystrophy is also unknown.
The most prominent serological abnormality of this disease is a decrease in blood C3, but hypocomplementemia and/or complement activation are not necessary factors for the development of glomerulonephritis.
Renal damage often occurs in patients with lipodystrophy, the incidence rate is 15% to 30%, the main type of renal damage is type II membranous proliferative glomerulonephritis or compact deposition disease (80%), and the other 20% is widely peripheral Mesangial migration, the morphological and histochemical characteristics of these two glomerulonephritis are the same as those of non-fatty dystrophy patients.
A type III variant has been reported that is sensitive to glucocorticoids in patients with this phenotype and low serum complement and nephrotic syndrome.
Because complete fat malnutrition often occurs simultaneously with diabetes, non-diabetic nephropathy is often found, and its pathological manifestations are indistinguishable from diabetic nephropathy without fat malnutrition. It has been reported in patients without diabetes, pathological manifestations of glomerulonephritis, fat The relationship between malnutrition and glomerular disease is unknown. Tuck et al reported that a small fraction of their capillaries have peripheral mesenteric implants, subepithelial and intramembranous deposits, but in general there is no special Characteristics, patient serum complement C3 levels are normal, no proteinuria, only a slight decrease in renal function.
Prevention
Fat malnutrition prevention
Congenital people have no effective preventive measures, and those who acquire sex mainly prevent infection and allergic reactions to prevent the disease from occurring.
Complication
Fat dystrophy complications Complications, diabetes, hirsutism, hepatic hyperlipidemia
Fat malnutrition In addition to kidney disease, patients often have symptoms similar to systemic fat malnutrition, including: diabetes, hirsutism, hepatomegaly, mental retardation, hyperlipidemia and subcutaneous nodules.
Symptom
Fat malnutrition symptoms Common symptoms Nitrogenemia Proteinuria Simple upper body thinning Both sides of the cheeks and ankles concave Muscle hypertrophy Fat metabolism disorders Nodules Giant tongue Abdominal pain Hypertension
1. Kidney performance
20% to 50% of patients with lipodystrophy develop renal disease, and about 10% of patients with dense deposition disease are partial lipodystrophy, and more than 20 years after showing symptoms of fatty dystrophy, significant renal manifestations Patients with impaired kidneys are usually asymptomatic proteinuria and microscopic hematuria, and a few are characterized by nephrotic syndrome. The most prominent serological abnormality of this disease is a decrease in blood C3.
Due to the clinical findings of this disease, there are proteinuria, gross hematuria, microscopic hematuria, pyuria, acute abdominal pain, fever, edema, hyperlipidemia, hypertension and azotemia. In patients with systemic lipodystrophy, kidney disease can also be seen. Syndrome manifestations, so the diagnosis of pyelonephritis, chronic glomerulonephritis or acute glomerulonephritis is often made in different patients.
The nature of renal lesions in this patient is also variable, including acute glomerulonephritis, pyelonephritis, solitary proteinuria and hematuria. Anatomical abnormalities in patients with partial lipodystrophy are common, including hydronephrosis and expansion of the collecting system. .
2. System performance
The occurrence of lipodystrophy is innate or can be gradually manifested in childhood. Congenital patients with lipodystrophy are born at birth, but diabetic patients often appear in adolescence, and acquired patients with lipodystrophy before the onset of diabetes or After the emergence, hyperlipidemia, hyperproteinemia and thyroid hypermetabolism are characteristic, other manifestations include: tall (>9%), muscle hypertrophy, multiple disease, giant tongue, abdominal distension, subcutaneous nodules, liver Large, hardened, clitoral or penile hypertrophy, febrile adenoma, brain atrophy, hemiplegia, thinking, intelligence, stunting and cardiac hypertrophy, a family reported to have hereditary lipodystrophy with multiple hemangioperic bladder disease, but It is speculated that this may be due to the continuation of genes associated with lipodystrophy, rather than some of the symptoms of lipodystrophy.
Examine
Fat malnutrition check
1. Urine examination Visible proteinuria, gross hematuria, microscopic hematuria, pyuria, a small number of nephrotic syndrome in the range of nephrotic syndrome.
2. Blood tests Hyperlipidemia, hyperproteinemia, hyperglycemia, hyperinsulinemia, urea nitrogen, elevated creatinine, and decreased serum complement C3.
Regular X-ray, B-ultrasound, CT, EMG, etc., can be found in kidney size, structural abnormalities and urinary calculi.
Diagnosis
Diagnosis and diagnosis of lipodystrophy
The diagnosis of this disease can be confirmed based on typical clinical manifestations and laboratory tests and other auxiliary tests.
It should be differentiated from scleroderma and other fat-metabolic diseases, and should be differentiated from other causes of nephrotic syndrome.
