Publications by authors named "J P van der Hulst"
Article Synopsis
- Integrin alpha V is crucial for cell adhesion and signaling during development, and mutations in its gene (ITGAV) can lead to serious health issues.
- In three families, biallelic variants were found that caused either dysfunctional protein production or the integrin being misplaced, resulting in severe developmental problems like eye and brain abnormalities, inflammatory bowel disease, and immune issues.
- Studies in patient cells and zebrafish models confirmed these mutations resulted in impaired immune signaling and developmental defects, linking the ITGAV variants to a newly identified human disease.
Purpose Of Review: The aim of this review is to outline recent studies relating to nutritional status and outcomes in pediatric end-stage liver disease.
Main Findings: Pediatric patients with chronic and end-stage liver disease are at high risk of malnutrition. Given additional growth demands in children and the inherent complications of chronic liver disease, achieving adequate nutrition in these patients remains a challenge.
Characteristics and outcomes of home parenteral nutrition among children with severe neurological impairment.
J Pediatr Gastroenterol Nutr
November 2024
Objectives: To describe the characteristics, secular trends, and outcomes of home parenteral nutrition (HPN) use among children with severe neurological impairment (SNI) and non-primary digestive disorders from 2010 to 2023 and compare outcomes to children with primary digestive disorders on HPN.
Methods: A retrospective review of all children with SNI and non-primary digestive disorders, where HPN was initiated between January 2010 and September 2023 at a tertiary care pediatric hospital. The Mann-Kendall trend test was used to assess trends in HPN initiation.
Background: Total energy expenditure (TEE) is the total energy expended by an individual to sustain life, activities, and growth. TEE is formed by four components: resting energy expenditure (REE), activity energy expenditure (AEE), growth-related energy expenditure (GEE), and the thermic effect of feeding (TEF). Some energy expenditure (EE) components may change throughout childhood and cannot be reliably estimated using prediction formulae.
View Article and Find Full Text PDFIntroduction: Undernutrition during pregnancy is linked to adverse pregnancy and birth outcomes and has downstream effects on the growth and development of children. The gut microbiome has a profound influence on the nutritional status of the host. This phenomenon is understudied in settings with a high prevalence of undernutrition, and further investigation is warranted to better understand such interactions.
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