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3.8
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Children
Special Issues
Chronic Liver Diseases in Children
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Chronic Liver Diseases in Children

A special issue of Children (ISSN 2227-9067).

Deadline for manuscript submissions: closed (10 February 2024) | Viewed by 1939

Special Issue Editor

Dr. Paul Henderson

E-Mail Website
Guest Editor
Royal Hospital for Children and Young People, Edinburgh, UK
Interests: epidemiology of paediatric gastrointestinal and hepatological diseases; the natural history of these diseases; novel treatments and their efficicacy

Special Issue Information

Dear Colleagues,

Research in all aspects of paediatric liver diseases remains sparse, with much of our current knowledge based on extrapolations from adult studies or data from single centres focusing on single conditions. Given the heterogenous group of hepatic disorders encountered within childhood, more extensive research is required to not only uncover the aetiology of these conditions but also to provide evidence-based treatment pathways and details on the natural history and management of these often-rare diseases into adulthood. We wish to invite you and your colleagues to contribute to our upcoming Special Issue of Children (Impact Factor 2.8), focusing on all aspects of liver disease in children to highlight data and advances on causes and treatment and to help design prospective studies to answer fundamental questions in this field.

Dr. Paul Henderson
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Children is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2400 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • liver diseases
  • child
  • epidemiology
  • causality
  • therapeutics

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Published Papers (1 paper)

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Research

10 pages, 214 KiB  
Article
Alpha-1-Antitrypsin Deficiency in Children—Unmet Needs Concerning the Liver Manifestation
by Joelle Lemke, Alexander Weigert, Soyhan Bagci, Mark Born, Rainer Ganschow and David Katzer
Children 2024, 11(6), 694; https://doi.org/10.3390/children11060694 - 6 Jun 2024
Viewed by 1415
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 [...] Read more.
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. Full article
(This article belongs to the Special Issue Chronic Liver Diseases in Children)
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