What Is Lipodystrophy?

(I) Causes of Onset

Lipodystrophy

Causes of lipodystrophy

(I) Causes of Onset

Congenital lipodystrophy is a blood relationship in patients with autosomal recessive inheritance; acquired people may have no genetic basis and often have prodromal symptoms of viral infection. Acquired systemic and partial lipodystrophy are considered to be autoimmune diseases.

(Two) pathogenesis

The pathogenesis of this disease is unclear. Various hypotheses proposed from epidemiological genetics and clinical studies have suggested that: systemic lipodystrophy and extensive metabolic and systemic abnormalities are related. The enhancement of sympathetic nerve activity may strengthen the fat Decomposition; pituitary may secrete fat mobilization substance but pituitary resection fails to correct fat dystrophy Destruction theory, but many patients with patchy lesions seem difficult to use this theory to explain the pathogenesis of renal disease and the relationship between lipodystrophy is unknown.

The most prominent serological abnormality of this disease is that blood C3 is reduced but hypocomplementemia and / or complement activation are not necessary factors for the occurrence of glomerulonephritis.

The incidence of renal damage in patients with lipodystrophy is 15% to 30%. The main type of renal damage is type membrane proliferative glomerulonephritis or dense deposit disease (80%). The other 20% are migrated by extensive peripheral mesangium. The morphological and histochemical characteristics of these two glomerulonephritis patients are the same as those of non-lipid malnutrition patients.

One patient with a type III variant with this phenotype and low serum complement and nephrotic syndrome was reported to be sensitive to glucocorticoids.

Because complete fatty dystrophy and diabetes occur simultaneously, the pathological manifestations of non-diabetic nephropathy and diabetic nephropathy without fatty dystrophy are often not identified. There have been reports of glomerulonephritis with lipodystrophy and renal nephropathy in patients without diabetes The relationship between globular disease is unknown. Tuck et al. Reported that a small part of their patients had capillaries with implanted subepithelial and intramembranous deposits in the peripheral mesangium, but overall there were no special characteristics. Patients had normal serum complement C3 levels. Proteinuria has only a slight decrease in renal function.

Signs and symptoms of lipodystrophy

1. Renal manifestations of lipodystrophy: 20% to 50% of patients with renal disease manifesting as dense deposit disease. Approximately 10% of patients have partial fatty dystrophy, and most of them show obvious symptoms of lipodystrophy in about 20 years. Renal manifestations In patients with renal impairment, asymptomatic proteinuria and microscopic hematuria are usually manifested as nephrotic syndrome. The most prominent serological abnormality in this disease is decreased blood C3.

Due to clinical findings of proteinuria, gross hematuria, hematuria, pyuria, acute abdominal pain, fever, edema, hyperlipidemia, hypertension, and azotemia, nephrotic syndrome can be seen in patients with systemic lipodystrophy, so it is often separated in different patients Make a diagnosis of chronic glomerulonephritis or acute glomerulonephritis with pyelonephritis.

The nature of renal disease in patients with this disease is also variable. Patients with acute glomerulonephritis, pyelonephritis, solitary proteinuria, and hematuria with partial lipodystrophy. Anatomic abnormalities are very common, including hydronephrosis and expansion of the collecting system.

2. Systemic manifestations The occurrence of lipodystrophy can be congenital or gradually manifest during childhood. Fatty dystrophy in congenital patients occurs at birth, but diabetic patients often have acquired adipose dystrophy in adolescents before the onset of diabetes or Later hyperlipidemia, hyperproteinemia, and thyroid hypermetabolism are characteristic. Other features include: tall (> 9%) muscle hypertrophy, giant tongue abdomen swelling, subcutaneous nodules, liver cirrhosis, or penile hypertrophic adenoma. Cerebral atrophy, hemiplegia, mental retardation, and cardiac hypertrophy. It has been reported that there is a family with heritable lipodystrophy with bladder multiple hemangiomatosis, but it is speculated that this may be due to the continuation of the gene for lipodystrophy rather than lipodystrophy. Some symptoms

The diagnosis of this disease can be determined based on typical clinical manifestations and laboratory tests and other auxiliary tests.

Fatty dystrophy test

1. Urine examination shows proteinuria, gross hematuria, pyuria, and nephrotic syndrome.

2. Blood test Hyperlipidemia Hyperproteinemia Hyperglycemia Hyperinsulinemia Urea nitrogen creatinine increased Serum complement C3 decreased.

Routine X-ray and B-mode CT electromyography can detect abnormalities in the size and structure of the kidney and urinary stones.

Differential diagnosis of lipodystrophy

It should be distinguished from scleroderma and other lipodystrophic diseases. It should also be distinguished from other causes of nephrotic syndrome.

Fat malnutrition complications

In addition to kidney disease, patients with lipodystrophy often have symptoms similar to systemic lipodystrophy, including: diabetic hirsutism, hepatic retardation, hyperlipidemia, and subcutaneous nodules.

Prevention of Fat Malnutrition

Congenital persons have no effective preventive measures. Acquired persons mainly prevent infection and allergic reactions to prevent the occurrence of this disease.

Drugs for the treatment of lipodystrophy

(A) treatment

There is currently no effective method for renin-angiotensin inhibitors to treat this disease.

(B) the prognosis

The duration of the disease usually depends on which clinical manifestations are most prominent. The rate of progression to end-stage renal disease in terms of glomerular damage is quite fast. Dense deposits often occur again after living donor kidney transplantation.