PMID- 35802526
OWN - NLM
STAT- MEDLINE
DCOM- 20220712
LR  - 20220828
IS  - 1473-5687 (Electronic)
IS  - 0954-691X (Linking)
VI  - 34
IP  - 8
DP  - 2022 Aug 1
TI  - Disease progression in patients with PI*ZZ alpha-1 antitrypsin deficiency.
PG  - 852-856
LID - 10.1097/MEG.0000000000002395 [doi]
AB  - BACKGROUND AND OBJECTIVE: Alpha-1 antitrypsin deficiency (AATD) is an uncommon 
      but underdiagnosed cause of cirrhosis and lacks medical treatment options. It is 
      important to recognize risk factors that contribute to disease progression and 
      liver transplantation. We aimed to assess if age, sex, or smoking status was 
      associated with liver or lung disease progression. METHODS: Forty-three patients 
      with ZZ-AATD cirrhosis were consecutively sampled from an Institutional Review 
      Board-approved registry of 240 patients with AATD of any genotype seen as 
      outpatients in the Cleveland Clinic between 1999 and 2019. To determine the 
      association between risk factors and lung or liver disease progression, linear 
      mixed-effects models with fixed effects for linear time, risk factor, and 
      time-by-risk factor interaction, and the random intercepts for intra-patient 
      correlation were used. RESULTS: Based on the mixed-effects model analysis, there 
      was a significant association between liver disease progression and smoking 
      history, and no association with age or sex. There was no association between 
      lung disease progression and age, sex, or smoking history. However, smoking 
      history was significantly associated with lower forced expiratory volume values. 
      CONCLUSION: This study found that in a cohort of patients with PI*ZZ genotype 
      AATD (ZZ-AATD) and cirrhosis, smoking history was associated with liver disease 
      progression, whereas age and sex were not.
CI  - Copyright (c) 2022 Wolters Kluwer Health, Inc. All rights reserved.
FAU - Shen, Katie
AU  - Shen K
AD  - Departments of Internal Medicine.
FAU - Lyu, Ruishen
AU  - Lyu R
AD  - Quantitative Health Sciences, Cleveland Clinic Foundation.
FAU - Sengupta, Shreya
AU  - Sengupta S
AD  - Department of Gastroenterology, Hepatology and Nutrition, Digestive Disease 
      Institute, Cleveland Clinic Foundation, Cleveland, Ohio, USA.
FAU - Wakim-Fleming, Jamile
AU  - Wakim-Fleming J
AD  - Department of Gastroenterology, Hepatology and Nutrition, Digestive Disease 
      Institute, Cleveland Clinic Foundation, Cleveland, Ohio, USA.
LA  - eng
PT  - Journal Article
DEP - 20220627
PL  - England
TA  - Eur J Gastroenterol Hepatol
JT  - European journal of gastroenterology & hepatology
JID - 9000874
RN  - 0 (alpha 1-Antitrypsin)
SB  - IM
MH  - Disease Progression
MH  - Forced Expiratory Volume
MH  - Humans
MH  - Liver Cirrhosis/diagnosis/epidemiology/etiology
MH  - *Lung Diseases
MH  - alpha 1-Antitrypsin/genetics
MH  - *alpha 1-Antitrypsin Deficiency/complications/diagnosis/epidemiology
EDAT- 2022/07/09 06:00
MHDA- 2022/07/14 06:00
CRDT- 2022/07/08 13:23
PHST- 2022/07/08 13:23 [entrez]
PHST- 2022/07/09 06:00 [pubmed]
PHST- 2022/07/14 06:00 [medline]
AID - 00042737-202208000-00006 [pii]
AID - 10.1097/MEG.0000000000002395 [doi]
PST - ppublish
SO  - Eur J Gastroenterol Hepatol. 2022 Aug 1;34(8):852-856. doi: 
      10.1097/MEG.0000000000002395. Epub 2022 Jun 27.