1-antitrypsin deficiency signs and Western medicine treatment

α1-antitrypsin deficiency signs and Western medicine treatment

hepatitis syndrome after the birth of α1-The ATD than heavy children after the birth of the early clinical manifestations of cholestasis. Small number of cases can be gradually improved, liver function returned to normal, but most patients with symptoms of progressive hepatomegaly and liver function deterioration. Children after 1 year of age, ascites, esophageal varices, cirrhosis and portal hypertension levy group. Cirrhosis to late adolescence. PiMS, PiMZ, cirrhosis of the liver caused by pathological α1-AT PiSZ such as the decision of the heterozygous genotype, usually mild, slower development. The small number of cases of neonatal sepsis, bleeding tendency, and umbilical defects, femoral hernia, emphysema and lung performance of recurrent infection.

α1-The ATD laboratory examination methods:

1 protein electrophoresis, alpha globulin, 90% of α1-AT. Serum acetate membrane electrophoresis can be used as routine screening for this disease, such as α1 globulin quantitative <2g / L, can be used as the initial diagnosis of alpha-ATD.

2 trypsin inhibition determination (trypsininhibitor capacity, TIC): the ability of serum to inhibit trypsin indirect quantitative per ml of normal human serum can inhibit the 1.1mg of trypsin, the TIC normal 1-2mg/ml small this value can make a diagnosis.

3antitrypsin antiserum measured serum immunodiffusion or immunofluorescence method. In addition, the increase in this disease serum bilirubin, transaminase increased, alkaline phosphatase was significantly higher, lower serum albumin.

Of the disease there is no effective treatment. Application has tried to promote liver cells to produce α1-AT drugs, such as courtship hormone, Danazol (danazol), methyl chloride copper, etc., in order to improve the blood concentration of α1-AT, to improve the protease and anti-protease balance between, but still in the experimental stage. Orthotopic liver transplantation is also applied to the disease, but did not determine the efficacy of

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