Objective: To analyze and determine the quality of functioning in different components of GHRH-GH-IGF1 axis in children with Down syndrome (DS).
Design: Systematic review and mini meta-analysis of the literature.
Methods: A search was performed in PubMed, Embase, Scopus, and PsycINFO through August 2022. Eligible studies included pediatric patients with DS who had undergone any laboratory evaluation of the GHRH-GH-IGF1 axis. Two reviewers independently screened articles for eligibility. Results of each type of test were weighed together in patients both with and without DS and were pooled using a random effects meta-analysis.
Results: In total, 20 studies assessed the GHRH-GH-IGF1 axis function. A defect in three major components of GHRH-GH-IGF1 axis was found in a significant proportion of pediatric DS patients.
Conclusions: A significant portion of short-stature pathogenesis in children with DS is associated with impaired GHRH-GH-IGF1 axis function.
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http://dx.doi.org/10.3389/fped.2023.1132296 | DOI Listing |
Front Pediatr
February 2023
Pediatric Endocrinology Unit, Soroka University Medical Center, Beer Sheva, Israel.
Objective: To analyze and determine the quality of functioning in different components of GHRH-GH-IGF1 axis in children with Down syndrome (DS).
Design: Systematic review and mini meta-analysis of the literature.
Methods: A search was performed in PubMed, Embase, Scopus, and PsycINFO through August 2022.
Development
October 2020
Walter and Eliza Hall Institute of Medical Research, Melbourne, Victoria 3052, Australia
Börjeson-Forssman-Lehmann syndrome (BFLS) is an intellectual disability and endocrine disorder caused by plant homeodomain finger 6 () mutations. Individuals with BFLS present with short stature. We report a mouse model of BFLS, in which deletion of causes a proportional reduction in body size compared with control mice.
View Article and Find Full Text PDFPediatr Hematol Oncol
March 2020
Department of Pediatrics (Hemat-Oncology Unit), PGIMER Chandigarh, Chandigarh, India.
Imatinib results in growth retardation in children with chronic myeloid leukemia (CML). The study was planned to assess the GHRH-GH-IGF1 axis in children with CML, receiving Imatinib and to evaluate the efficacy of human growth hormone (hGH) therapy. Twenty children with CML, receiving Imatinib for a period exceeding 6 months, with resultant growth retardation were included.
View Article and Find Full Text PDFFront Immunol
September 2019
GIGA-I3 Center of Immunoendocrinology, University of Liège, Liège, Belgium.
The precise impact of the somatotrope axis upon the immune system is still highly debated. We have previously shown that mice with generalized ablation of growth hormone (GH) releasing hormone (GHRH) gene () have normal thymus and T-cell development, but present a marked spleen atrophy and B-cell lymphopenia. Therefore, in this paper we have investigated vaccinal and anti-infectious responses of mice against , a pathogen carrying T-independent antigens.
View Article and Find Full Text PDFMol Metab
May 2017
Department of Physiology, Division of Integrative Physiology, Jichi Medical University, 3311-1 Yakushiji, Tochigi, Shimotsuke, 329-0498, Japan. Electronic address:
Objective: In the hypothalamic arcuate nucleus (ARC), orexigenic agouti-related peptide (AgRP) neurons regulate feeding behavior and energy homeostasis, functions connected to bone metabolism. The 3-phosphoinositide-dependent protein kinase-1 (PDK1) serves as a major signaling molecule particularly for leptin and insulin in AgRP neurons. We asked whether PDK1 in AGRP neurons also contributes to bone metabolism.
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