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Does the Heterozygous State of Alpha‐1 Antitrypsin Deficiency Have a Role in Chronic Liver Diseases? Interim Results of a Large Case‐Control Study
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| Zeitschriftentitel: | Journal of Pediatric Gastroenterology and Nutrition |
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| Personen und Körperschaften: | , , , , , , , , , |
| In: | Journal of Pediatric Gastroenterology and Nutrition, 43, 2006, S1 |
| Format: | E-Article |
| Sprache: | Englisch |
| veröffentlicht: |
Wiley
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| Schlagwörter: |
| Zusammenfassung: | <jats:title>ABSTRACT</jats:title><jats:sec><jats:title>Background:</jats:title><jats:p>The role of the heterozygous PiZ state of alpha‐1 antitrypsin deficiency (α<jats:sub>1</jats:sub>ATD) in the pathogenesis of chronic liver disease (LD) is still a matter of controversy.</jats:p></jats:sec><jats:sec><jats:title>Aim:</jats:title><jats:p>To determine the prevalence of α<jats:sub>1</jats:sub>ATD heterozygote states in a large population of patients with established LD compared with individuals with no LD, and to determine whether the prevalence of PiZ is increased in patients with more severe LD.</jats:p></jats:sec><jats:sec><jats:title>Methods:</jats:title><jats:p>A cross sectional case‐control study among patients with and without LD. Blood samples were tested for α<jats:sub>1</jats:sub>AT levels and α<jats:sub>1</jats:sub>AT phenotype. The severity of LD was determined by clinical evaluation, lab tests, imaging studies and histopathology.</jats:p></jats:sec><jats:sec><jats:title>Results:</jats:title><jats:p>In total, 1405 patients were enrolled; 651 with, and 754 without LD. Out of them, 173 patients had decompensated cirrhosis requiring liver transplantation. PiMZ was significantly more prevalent in White patients (3.5%) compared with Hispanics (1.7%; <jats:italic>P</jats:italic> = 0.029). There was no difference in PiMZ prevalence between the total LD group and the group with no LD (2.1% vs. 1.7%; <jats:italic>P</jats:italic> = 0.64). Within the LD group, 5.7% of 173 patients with decompensated LD, listed for liver transplantation, had PiMZ, compared with 2.1% of 478 patients with less severe LD (<jats:italic>P</jats:italic> = 0.016). Similarly, there was a disproportionately higher prevalence of PiZ among hepatitis C virus (HCV) patients (5.6%) and patients with nonalcoholic fatty liver disease (NAFLD) (5.0%) with decompensated LD, compared with HCV patients (1.2%) and NAFLD patients (1.9%) with less severe LD (<jats:italic>P</jats:italic> = 0.044 and 0.017, respectively). Patients with cryptogenic cirrhosis, who were not considered NAFLD patients, did not have a higher prevalence of PiMZ compared with patients with LD of known etiologies (1.9% vs. 2.3%; <jats:italic>P</jats:italic> = 0.12).</jats:p></jats:sec><jats:sec><jats:title>Conclusions:</jats:title><jats:p>We found no association between the heterozygous PiZ state of α<jats:sub>1</jats:sub>ATD and the presence of chronic LD in‐general or the presence of cryptogenic cirrhosis. In contrast, patients with decompensated LD of any etiology had a significantly higher prevalence of PiMZ compared with patients with compensated LD. Furthermore, in patients with chronic LD due to HCV or NAFLD there was a significant association between the PiMZ heterozygous state and increased severity of LD and the need for liver transplantation. These interim results suggest that the PiMZ α<jats:sub>1</jats:sub>ATD heterozygous state may have a role in worsening LD due to HCV or NAFLD.</jats:p></jats:sec> |
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| ISSN: |
0277-2116
1536-4801 |
| DOI: | 10.1097/01.mpg.0000226387.56612.1e |