Tejwani Vickram, Wang Xiao-Feng, Stoller James K
Internal Medicine, Cleveland Clinic, Ohio.
Quantitative Health Sciences, Cleveland Clinic, Ohio.
Chronic Obstr Pulm Dis. 2015 Sep 10;2(4):290-295. doi: 10.15326/jcopdf.2.4.2015.0141.
PIZZ alpha-1 antitrypsin (AAT) deficiency poses risk for lung disease through 2 different mechanisms: in which deficient AAT levels cause a depleted proteolytic screen and, separately, a proinflammatory effect of Z polymers produced both by alveolar macrophages and by the liver. Ample data support the first mechanism, while the possible contribution of the second 2 proinflammatory mechanisms is currently unknown. Experience with a 74 year-old PIZZ female who underwent single lung transplantation and subsequent orthotopic liver transplantation (OLT) may shed light on the relative contributions of each of the potential mechanisms. Availability of multiple pulmonary function tests (PFT) measurements uniquely permitted calculation of rates of lung function change before and after OLT. The rate of forced expiratory volume in 1 second (FEV) decline normalized post-OLT (from -60 to -21 ml/yr). Her course suggests that restoring the normal serum AAT levels or, alternately, eliminating liver-derived polymers, exerted a greater effect on preventing emphysema progression than local Z polymer production contributed to furthering emphysema.
PIZZ型α-1抗胰蛋白酶（AAT）缺乏症通过两种不同机制引发肺部疾病风险：AAT水平不足导致蛋白水解屏障受损，以及肺泡巨噬细胞和肝脏产生的Z聚合物的促炎作用。大量数据支持第一种机制，而第二种促炎机制的潜在作用目前尚不清楚。一名74岁的PIZZ型女性接受单肺移植及随后的原位肝移植（OLT）的经历，可能有助于阐明每种潜在机制的相对作用。多次肺功能测试（PFT）测量结果的可得性，独特地允许计算OLT前后肺功能变化率。1秒用力呼气量（FEV）下降率在OLT后恢复正常（从-60降至-21 ml/年）。她的病程表明，恢复正常血清AAT水平，或者消除肝脏来源的聚合物，在预防肺气肿进展方面比局部Z聚合物产生对肺气肿进展的影响更大。
