PMID- 24176989
OWN - NLM
STAT- MEDLINE
DCOM- 20140929
LR  - 20140210
IS  - 1532-3064 (Electronic)
IS  - 0954-6111 (Linking)
VI  - 108
IP  - 2
DP  - 2014 Feb
TI  - Disease associations in alpha-1-antitrypsin deficiency.
PG  - 338-43
LID - S0954-6111(13)00416-2 [pii]
LID - 10.1016/j.rmed.2013.10.006 [doi]
AB  - INTRODUCTION: In addition to emphysema alpha-1-antitrypsin deficiency (AATD) has 
      been shown to be associated with several inflammatory conditions, including 
      bronchiectasis, vasculitis, (in particular Wegener's granulomatosis), and 
      panniculitis, suggesting neutrophil proteinases also play a role in their 
      pathophysiology. However, it remains unknown whether other inflammatory diseases 
      are also more prevalent in AATD than the general population. The current study 
      describes the prevalence of other co-morbidities in AATD with particular emphasis 
      on inflammatory bowel disease. METHODS AND RESULTS: The case notes of 651 PiZZ or 
      PiZnull patients attending the UK national centre for AATD between 1996 and 2011 
      were reviewed. The prevalence of inflammatory bowel disease (1.5%) was higher 
      than that predicted in the UK (0.4%). Ten patients had a confirmed diagnosis of 
      ulcerative colitis, and 1 had Crohn's disease. In 2 cases there was a family 
      history of inflammatory bowel disease and all but 1 patient were ex or never 
      smokers. There was also a higher prevalence of hypothyroidism in this patient 
      group than expected for the UK population - 26 cases (7.2% of females and 1.3% of 
      males). CONCLUSIONS: The current study of the UK cohort of patients with AATD 
      confirmed a higher prevalence of ulcerative colitis than would be expected in the 
      general population, providing further evidence of a potential link between these 
      2 conditions. In addition, the data suggested a potential link between 
      hypothyroidism and AATD.
CI  - Copyright (c) 2013 Elsevier Ltd. All rights reserved.
FAU - Stone, H
AU  - Stone H
AD  - ADAPT Project, Lung Function and Sleep Department, Queen Elizabeth Hospital 
      Birmingham, Mindelsohn Way, Edgbaston, Birmingham B15 2WB, UK. Electronic 
      address: hmstone2k@hotmail.com.
FAU - Pye, A
AU  - Pye A
AD  - ADAPT Project, Lung Function and Sleep Department, Queen Elizabeth Hospital 
      Birmingham, Mindelsohn Way, Edgbaston, Birmingham B15 2WB, UK; University of 
      Birmingham, Edgbaston, Birmingham B15 2TT, UK. Electronic address: 
      anita.pye@uhb.nhs.uk.
FAU - Stockley, R A
AU  - Stockley RA
AD  - ADAPT Project, Lung Function and Sleep Department, Queen Elizabeth Hospital 
      Birmingham, Mindelsohn Way, Edgbaston, Birmingham B15 2WB, UK. Electronic 
      address: rob.stockley@uhb.nhs.uk.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20131014
PL  - England
TA  - Respir Med
JT  - Respiratory medicine
JID - 8908438
SB  - IM
MH  - Adult
MH  - Colitis, Ulcerative/*etiology/physiopathology
MH  - Crohn Disease/*etiology/physiopathology
MH  - Female
MH  - Forced Expiratory Volume/physiology
MH  - Humans
MH  - Hypothyroidism/etiology/physiopathology
MH  - Male
MH  - Retrospective Studies
MH  - Vital Capacity/physiology
MH  - alpha 1-Antitrypsin Deficiency/*complications/physiopathology
OTO - NOTNLM
OT  - Alpha-1-antitrypsin deficiency
OT  - Hypothyroidism
OT  - Inflammatory bowel disease
OT  - Neutrophilic inflammation
EDAT- 2013/11/02 06:00
MHDA- 2014/09/30 06:00
CRDT- 2013/11/02 06:00
PHST- 2013/05/08 00:00 [received]
PHST- 2013/10/04 00:00 [revised]
PHST- 2013/10/06 00:00 [accepted]
PHST- 2013/11/02 06:00 [entrez]
PHST- 2013/11/02 06:00 [pubmed]
PHST- 2014/09/30 06:00 [medline]
AID - S0954-6111(13)00416-2 [pii]
AID - 10.1016/j.rmed.2013.10.006 [doi]
PST - ppublish
SO  - Respir Med. 2014 Feb;108(2):338-43. doi: 10.1016/j.rmed.2013.10.006. Epub 2013 
      Oct 14.