Patients heterozygous for the Z gene (phenotype MZ) also have an increased incidence of emphysema, usually of later onset, but we have observed several cases under 40. The other manifestation of severe 伪-antitrypsin deficiency is liver disease. Three children studied had prolonged neonatal ...
alpha 1-Antitrypsin, the major serum protease inhibitor, is a glycoprotein synthesized in the liver. Severe deficiency results in protease-antiprotease imbalance, which predisposes to severe emphysema at a young age. Reduced serum levels reflect inadequate release of alpha 1-antitrypsin by the liver,...
Alpha-1抗胰蛋白酶缺乏症的英文名字是Alpha-1 antitrypsin deficiency。基因解码表明:Alpha-1抗胰蛋白酶缺乏症是一种遗传性疾病,与基因突变密切相关。Alpha-1抗胰蛋白酶是一种由SERPINA1基因编码的蛋白质,它在肝脏中合成并通过血液循环分布到全身。该蛋白质的主要功能是
Spittle DA, Maier L, Hamzeh N, Silverman EK, Hobbs BD, Hersh CP, DeMeo DL, Cho MH, Bowler RP. Unique and shared systemic biomarkers for emphysema in Alpha-1 Antitrypsin deficiency and chronic obstructive pulmonary disease. EBioMedicine. 2022 Oct;84:104262. doi: 10.1016/j.ebiom.2022.104262....
Alpha-1 antitrypsin(抗胰蛋白酶)deficiency is a genetic disorder(遗传病)in which a protein called alpha-1antitrypsin is defective or absent, and it causes lung and liver disease. α抗胰蛋白酶缺乏症是一种遗传性疾病,患者体内的一种叫做α抗胰蛋白酶的蛋白质出现了问题或者含量减少了,最终导致肺和肝脏...
doi:10.1080/21548331.1971.11706032HarveyUniversityL.UniversitySharpUniversityInformaworldHospital PracticeSharp H. L. Alpha-1-antitrypsin deficiency. Hosp Pract 1971 ; 6: 83 [Taylor & Francis Online]
Alpha-1-antitrypsin deficiency (AATD) is a rare genetic disorder associated with the development of liver and lung disease. AAT is a 52-kD glycoprotein, produced mainly by hepatocytes and secreted into the blood. Agglomeration of the AAT-protein in hepatocytes can result in liver disease. Exposur...
Alpha1-Proteinase Inhibitor (Human), ZEMAIRA® is indicated to raise the plasma level of alpha1-proteinase inhibitor (A1-PI) in patients with A1-PI deficiency and related emphysema.
Lindgren, "The prevalence and clinical significance of 𝛼1-antitrypsin deficiency (PiZ) and ANCA specificities (pro- teinase 3, BPI) in patients with ulcerative colitis," Inflammatory Bowel Diseases, vol. 5, no. 4, pp. 246-252, 1999....
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