title: Effect of liver transplant on pulmonary functions in adult patients with alpha 1 antitrypsin deficiency: 7 cases.
abstract: OBJECTIVE
  Alpha 1 antitrypsin (A1A) is a 52 kD glycoprotein that is mainly synthesized in the liver. As a major protease inhibitor, it binds to and neutralizes neutrophil elastase, thereby limiting the damage to the normal tissues after an inflammatory response. A deficiency in A1A leads to end-stage liver disease, both in children and in adults. In addition, the deficiency also has a detrimental effect in the lungs of the adult population. Alpha 1 antitrypsin deficiency is corrected with hepatic replacement; however, the changes in pulmonary functions have not been studied before and after liver transplant. The purpose of this study was to observe the changes in the pulmonary functions of patients who underwent liver transplant for the treatment of A1A deficiency.
  
  
  METHODS
  Nine patients underwent liver transplant for A1A deficiency. Seven patients (5 men, 2 women; mean age, 49.95 -/+ 7.09 years) had their pulmonary function tests available before the liver transplant (mean, 5.6 -/+ 3.4; range, 0.9-10.1 months) and after the liver transplant (mean, 30.3 -/+ 18.4, range 7.8-48.1 months) for analysis.
  
  
  RESULTS
  The mean, preliver, transplant, FEV1 was 2.69 -/+ 0.9 L, which was nearly unchanged after the liver transplant to a mean of 2.7 -/+ 1.2 L. During the mean total interval of nearly 3 years, an estimated decline of 250 mL in FEV1 was expected.
  
  
  CONCLUSIONS
  It appears from the results of our study that liver transplant probably prevented the progression of pulmonary disease in A1A-deficient patients. Further study and close, postliver, transplant follow-up is warranted to support our initial findings.
authors.affiliation: Department of Surgery, Division of Abdominal Organ Transplant, Temple University Hospital, Philadelphia, PA 19140, USA. ashokkumar.jain@tuhs.temple.edu
authors.forename: Ashokkumar B
authors.initials: AB
authors.lastname: Jain
authors.forename: Vrishali
authors.initials: V
authors.lastname: Patil
authors.forename: Baber
authors.initials: B
authors.lastname: Sheikh
authors.forename: Michael
authors.initials: M
authors.lastname: Apostolakos
authors.forename: Charlotte
authors.initials: C
authors.lastname: Ryan
authors.forename: Randeep
authors.initials: R
authors.lastname: Kashyap
authors.forename: Mark
authors.initials: M
authors.lastname: Orloff
mesh_terms: D000328:Adult; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D005541:Forced Expiratory Volume / Q000502:physiology; D006801:Humans; D017093:Liver Failure / Q000209:etiology / Q000503:physiopathology / Q000601:surgery*; D016031:Liver Transplantation / Q000502:physiology*; D008168:Lung / Q000503:physiopathology*; D008171:Lung Diseases / Q000209:etiology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D010996:Pleural Effusion / Q000503:physiopathology; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012907:Smoking / Q000503:physiopathology; D019896:alpha 1-Antitrypsin Deficiency / Q000150:complications* / Q000503:physiopathology / Q000601:surgery*