Publications by authors named "Hino Bayan"

Article Synopsis
  • Inborn-Errors of Metabolism (IEM) are genetic disorders caused by mutations in genes related to metabolic pathways, with some lacking specific biochemical markers.
  • Integrating next-generation sequencing (NGS), especially whole exome sequencing (WES), into IEM diagnostics enhances accuracy, supports genetic counseling, and broadens treatment options.
  • For example, supplementing amino acids has shown positive effects on patients with deficiencies in aminoacyl-tRNA synthetases (ARSs), improving both biochemical and clinical outcomes.
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Objectives: The glucagon-like peptide-2 analog Teduglutide has been shown to enhance intestinal absorption and decrease parenteral nutrition (PN) requirements in short bowel syndrome (SBS). As data in children is limited, we evaluated nationwide real-life experience and treatment outcome in children with SBS.

Methods: Longitudinal data of children treated with Teduglutide for ≥3 months was collected.

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Article Synopsis
  • A quality improvement program was created in Israel to track important indicators for managing pediatric inflammatory bowel disease (PIBD) over a two-year period, involving outpatient clinics across the country.
  • A Delphi group selected various quality indicators, and data from over 3,000 visits were analyzed, showing significant improvements in specific monitoring practices and clinical outcomes.
  • The study highlighted increased use of biologics versus traditional immunomodulators and demonstrated that even with limited resources, nationwide programs can enhance the standard of care in PIBD.
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Background: Arthrogryposis, renal dysfunction, and cholestasis (ARC) syndrome is a rare and usually fatal metabolic autosomal recessive disorder, which has recently been shown to result from mutations in VPS33B located on chromosome 15q26.1. Neurological signs and ichthyosis almost invariably accompany the disease.

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Background: Cystic fibrosis (CF) is frequently associated with gastrointestinal complaints that can be due to gastrointestinal dysmotility. Electrogastrography (EGG) is an attractive, non-invasive procedure to assess gastric electric activity. The aims of our study were to investigate EGG abnormalities in pancreatic sufficient and pancreatic insufficient CF patients, and to examine whether EGG correlates with gastric emptying as assessed by scintigraphy.

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Objectives: Osteoporosis is the most common manifestation of untreated celiac disease (CD). Bone quantitative ultrasound (QUS) has recently emerged as a new modality for bone status assessment. We evaluated bone status in children with CD using dual-energy x-ray absorptiometry and quantitative ultrasound.

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