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Liver function in alpha-1-antitrypsin deficient individuals at 37 to 40 years of age.

Abstract Severe alpha-1-antitrypsin (AAT) deficiency (PiZZ) is a risk factor for liver disease, but the prevalence of liver cirrhosis and hepatocellular cancer in PiZZ adults is unknown. The risk of liver disease in adults with moderate AAT deficiency (PiSZ) is also unknown. A cohort of 127 PiZZ, 2 PiZnull, 54 PiSZ, and 1 PiSnull individuals were identified by the Swedish national neonatal AAT screening program between 1972 and 1974, when all 200,000 newborn infants in Sweden were screened for AAT deficiency. The cohort has been followed up since birth. Our aim was to study liver function and signs of liver disease in this cohort at 37 to 40 years of age in comparison with a matched, random sample of control subjects identified from the population registry.Eighty seven PiZZ, 32 PiSZ, and 92 control subjects (PiMM) answered a questionnaire on medication and alcohol consumption and provided blood samples. Liver stiffness was assessed by Acoustic Radiation Force Impulse (ARFI) elastography in 32 PiZZ, 15 PiSZ, and 51 PiMM subjects.The median of liver function tests and procollagen-III-peptide were within the normal range in all Pi subgroups. However, the PiZZ men had significantly higher plasma bilirubin than the PiMM men (P = 0.018). Plasma [Latin Small Letter Gamma]-glutamyl transferase (GGT) was significantly higher in the PiZZ men (P = 0.009) and the PiSZ men (P = 0.021) compared with the PiMM men. The median of liver stiffness was significantly higher in the PiZZ men (P = 0.037) and the PiSZ men (P = 0.032) compared with the PiMM men. The PiZZ women taking medication influencing liver enzymes had significantly higher GGT than the PiMM women on the corresponding treatment (P = 0.023).These AAT-deficient individuals identified by neonatal screening have normal plasma levels of liver function tests, and no clinical signs indicating liver disease at the age of 37 to 40 years. However, bilirubin, GGT, and liver stiffness are significantly higher in PiZZ men than PiMM men.
PMID
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Authors

Mayor MeshTerms
Keywords
Journal Title medicine
Publication Year Start




PMID- 28328804
OWN - NLM
STAT- MEDLINE
DA  - 20170322
DCOM- 20170410
LR  - 20170410
IS  - 1536-5964 (Electronic)
IS  - 0025-7974 (Linking)
VI  - 96
IP  - 12
DP  - 2017 Mar
TI  - Liver function in alpha-1-antitrypsin deficient individuals at 37 to 40 years of 
      age.
PG  - e6180
LID - 10.1097/MD.0000000000006180 [doi]
AB  - Severe alpha-1-antitrypsin (AAT) deficiency (PiZZ) is a risk factor for liver
      disease, but the prevalence of liver cirrhosis and hepatocellular cancer in PiZZ 
      adults is unknown. The risk of liver disease in adults with moderate AAT
      deficiency (PiSZ) is also unknown. A cohort of 127 PiZZ, 2 PiZnull, 54 PiSZ, and 
      1 PiSnull individuals were identified by the Swedish national neonatal AAT
      screening program between 1972 and 1974, when all 200,000 newborn infants in
      Sweden were screened for AAT deficiency. The cohort has been followed up since
      birth. Our aim was to study liver function and signs of liver disease in this
      cohort at 37 to 40 years of age in comparison with a matched, random sample of
      control subjects identified from the population registry.Eighty seven PiZZ, 32
      PiSZ, and 92 control subjects (PiMM) answered a questionnaire on medication and
      alcohol consumption and provided blood samples. Liver stiffness was assessed by
      Acoustic Radiation Force Impulse (ARFI) elastography in 32 PiZZ, 15 PiSZ, and 51 
      PiMM subjects.The median of liver function tests and procollagen-III-peptide were
      within the normal range in all Pi subgroups. However, the PiZZ men had
      significantly higher plasma bilirubin than the PiMM men (P = 0.018). Plasma
      [Latin Small Letter Gamma]-glutamyl transferase (GGT) was significantly higher in
      the PiZZ men (P = 0.009) and the PiSZ men (P = 0.021) compared with the PiMM men.
      The median of liver stiffness was significantly higher in the PiZZ men (P =
      0.037) and the PiSZ men (P = 0.032) compared with the PiMM men. The PiZZ women
      taking medication influencing liver enzymes had significantly higher GGT than the
      PiMM women on the corresponding treatment (P = 0.023).These AAT-deficient
      individuals identified by neonatal screening have normal plasma levels of liver
      function tests, and no clinical signs indicating liver disease at the age of 37
      to 40 years. However, bilirubin, GGT, and liver stiffness are significantly
      higher in PiZZ men than PiMM men.
FAU - Mostafavi, Behrouz
AU  - Mostafavi B
AD  - aDepartment of Respiratory Medicine and Allergology Malmo bDepartment of Clinical
      Radiology Malmo, Skane University Hospital, Lund University, Sweden.
FAU - Diaz, Sandra
AU  - Diaz S
FAU - Tanash, Hanan A
AU  - Tanash HA
FAU - Piitulainen, Eeva
AU  - Piitulainen E
LA  - eng
PT  - Journal Article
PT  - Observational Study
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
RN  - 0 (Collagen Type III)
RN  - 0 (Contraceptive Agents, Female)
RN  - 0 (Prescription Drugs)
RN  - 0 (Procollagen)
SB  - AIM
SB  - IM
MH  - Adult
MH  - Collagen Type III/blood
MH  - Contraceptive Agents, Female/administration & dosage/adverse effects
MH  - Female
MH  - Humans
MH  - Liver/diagnostic imaging/*physiology
MH  - Liver Function Tests
MH  - Male
MH  - Prescription Drugs/administration & dosage/adverse effects
MH  - Procollagen/blood
MH  - Severity of Illness Index
MH  - Sweden
MH  - alpha 1-Antitrypsin Deficiency/*physiopathology
PMC - PMC5371441
EDAT- 2017/03/23 06:00
MHDA- 2017/04/11 06:00
CRDT- 2017/03/23 06:00
AID - 10.1097/MD.0000000000006180 [doi]
AID - 00005792-201703240-00007 [pii]
PST - ppublish
SO  - Medicine (Baltimore). 2017 Mar;96(12):e6180. doi: 10.1097/MD.0000000000006180.

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