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Wando Mount Pleasant Library
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Hurd/St. Andrews Library
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Alpha-1-Antitrypsin Deficiency in Children—Unmet Needs Concerning the Liver Manifestation.
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- Author(s): Lemke, Joelle; Weigert, Alexander; Bagci, Soyhan; Born, Mark; Ganschow, Rainer; Katzer, David
- Source:
Children; Jun2024, Vol. 11 Issue 6, p694, 10p- Subject Terms:
LIVER abnormalities; BIOPSY; SMALL for gestational age; ACADEMIC medical centers; QUESTIONNAIRES; SEX distribution; SEVERITY of illness index; RETROSPECTIVE studies; ULTRASONIC imaging; DESCRIPTIVE statistics; AGE distribution; LIVER diseases; LONGITUDINAL method; ALPHA 1-antitrypsin; ALPHA 1-antitrypsin deficiency; GENETIC mutation; NEONATAL jaundice; CHOLESTASIS; LIVER transplantation; GENOTYPES; AMINOTRANSFERASES; DISEASE progression; COMORBIDITY; CHILDREN - Source:
- Additional Information
- Subject Terms:
- Abstract: Objectives: This study aimed to analyse the clinical course of 45 children with severe alpha-1-antitrypsin deficiency (AATD) registered in our clinic to detect possible predictors of poor outcomes. Methods: The clinical and biological data of 45 patients with homozygous or compound heterozygous AATD were analysed. The data were collected retrospectively going back to 2005 and prospectively from May 2020 until October 2021. It was based on questionnaires, laboratory values, sonography, and biopsy findings. Liver disease was classified into four grades depending on the grade of liver disease: mild or no liver disease, moderate disease, severe disease, and liver transplantation. Results: Thirty-nine patients (86.7%) had a Pi*ZZ and five (11.1%) a Pi*SZ genotype. One patient showed a new, not-yet-described compound heterozygous genotype (Pi*Z + Asp95Asn). A total of 66.7% of the cohort showed mild or no liver disease, 20% moderate, and 13.3% severe. AATD was diagnosed in most cases because of liver abnormalities, such as the elevation of transaminases (42.2%). A total of 29.4% of the patients with neonatal icterus prolongatus developed severe liver disease, and 25.7% were born small for their gestational age (SGA). Diseases of the atopic type were reported in 47.4% of the cases. Conclusions: The presence of neonatal icterus prolongatus in the first weeks of life was significantly more likely in severe courses of liver disease (r = 0.371, p = 0.012). A tendency toward atopic comorbidity in AAT-deficient children needs to be further evaluated. [ABSTRACT FROM AUTHOR]
- Abstract: Copyright of Children is the property of MDPI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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