PMID- 9630182
OWN - NLM
STAT- MEDLINE
DCOM- 19980630
LR  - 20190822
IS  - 0147-5185 (Print)
IS  - 0147-5185 (Linking)
VI  - 22
IP  - 6
DP  - 1998 Jun
TI  - Liver carcinoma in PiZ alpha-1-antitrypsin deficiency.
PG  - 742-8
AB  - One hundred sixty-four consecutive cases of primary liver carcinoma were 
      evaluated for tumor type, (i.e., hepatocellular carcinoma [HCC], 
      cholangiocarcinoma [CC], and combined hepatocholangiocarcinoma [CHCC]), and for 
      signs of alpha-1-antitrypsin deficiency (AATD) in the surrounding liver tissue. 
      Hepatocellular globular alpha-1-antitrypsin deposits, as detected by a monoclonal 
      antibody to the mutant PiZ alpha-1-antitrypsin (AAT), were seen in 13 cases 
      (7.9%). With regard to tumor type, 4 of 111 HCC cases (3.5%), but 4 of 37 CC 
      cases (10.5%), and even 5 of 16 CHCC cases (30%) were positive for this 
      antitrypsin variant. In all but 1 of 13 cases of alpha-1-antitrypsin deficiency, 
      the carcinoma developed in noncirrhotic liver tissue of elderly people (mean age, 
      62.9 years). In three patients, a heterozygous state of ATT (PiMZ) could be 
      revealed using isoelectric focusing or direct genetic analysis. We conclude from 
      our findings that CHCC and CC especially might be associated with PiZ 
      alpha-1-antitrypsin deficiency. Primary liver carcinoma might develop even in a 
      heterozygote state of PiZ alpha-1-antitrypsin deficiency without concurrent liver 
      disease. Furthermore, liver cirrhosis is not a precondition for these tumors.
FAU - Zhou, H
AU  - Zhou H
AD  - Department of Pathology, University Bonn, Federal Republic of Germany.
FAU - Fischer, H P
AU  - Fischer HP
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Am J Surg Pathol
JT  - The American journal of surgical pathology
JID - 7707904
RN  - 0 (SERPINA1 protein, human)
RN  - 0 (alpha 1-Antitrypsin)
RN  - 68238-35-7 (Keratins)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - Female
MH  - Humans
MH  - Immunohistochemistry
MH  - Isoelectric Focusing
MH  - Keratins/metabolism
MH  - Liver Neoplasms/complications/*etiology/metabolism/pathology
MH  - Male
MH  - Middle Aged
MH  - alpha 1-Antitrypsin/*adverse effects/genetics/metabolism
MH  - alpha 1-Antitrypsin Deficiency/*complications/pathology
EDAT- 1998/06/18 00:00
MHDA- 1998/06/18 00:01
CRDT- 1998/06/18 00:00
PHST- 1998/06/18 00:00 [pubmed]
PHST- 1998/06/18 00:01 [medline]
PHST- 1998/06/18 00:00 [entrez]
AID - 10.1097/00000478-199806000-00012 [doi]
PST - ppublish
SO  - Am J Surg Pathol. 1998 Jun;22(6):742-8. doi: 10.1097/00000478-199806000-00012.