PMID- 23527596
OWN - NLM
STAT- MEDLINE
DCOM- 20131115
LR  - 20181202
IS  - 1541-2563 (Electronic)
IS  - 1541-2563 (Linking)
VI  - 10 Suppl 1
DP  - 2013 Mar
TI  - History of the first description of childhood liver disease in AATD.
PG  - 13-6
LID - 10.3109/15412555.2013.766165 [doi]
AB  - This supplement celebrates the 50th anniversary of Alpha-1 antitrypsin deficiency 
      (AATD). Initially AATD was associated with an inherited form of emphysema. This 
      historical article describes the predisposition of AATD to liver disease. The 
      morphologic findings contributed to a better understanding of low serum levels of 
      A1 AT by the finding of AAT accumulating and stuck in the lumen of the rough 
      endoplasmic reticulum of the hepatocyte. Thus, only low levels of PiZ were 
      secreted for the rest of the human body and the only clinical correction was by 
      liver transplantation.
FAU - Sharp, Harvey L
AU  - Sharp HL
AD  - Department of Pediatrics, University of Minnesota, Minneapolis, MN 55455, USA. 
      sharp001@umn.edu
LA  - eng
PT  - Historical Article
PT  - Journal Article
PT  - Personal Narrative
PT  - Review
PL  - England
TA  - COPD
JT  - COPD
JID - 101211769
SB  - IM
MH  - Child
MH  - History, 20th Century
MH  - Humans
MH  - Liver/pathology/ultrastructure
MH  - Liver Transplantation
MH  - alpha 1-Antitrypsin Deficiency/*history/pathology/surgery
EDAT- 2013/04/03 06:00
MHDA- 2013/11/16 06:00
CRDT- 2013/03/27 06:00
PHST- 2013/03/27 06:00 [entrez]
PHST- 2013/04/03 06:00 [pubmed]
PHST- 2013/11/16 06:00 [medline]
AID - 10.3109/15412555.2013.766165 [doi]
PST - ppublish
SO  - COPD. 2013 Mar;10 Suppl 1:13-6. doi: 10.3109/15412555.2013.766165.