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- Title
Short Stature in Partially Corrected X-linked Severe Combined Immunodeficiency - Suboptimal Response to Growth Hormone.
- Authors
Suk See De Ravin; Shum, Elaine; Zarember, Kol A.; Rezvani, Geoffrey; Rosenfeld, Ron G.; Stratakis, Constantine A.; Malech, Harry L.
- Abstract
The article discusses a study which aimed to evaluate the growth hormone (GH)/insulin-like growth factor-I (IGF-I) axis in three children with X-linked severe combined immunodeficiency (XSCID) and partial immune reconstitution with profound growth failure. The study hypothesized that the underlying γc defect may be in part responsible for the profoundly short stature observed in many pre-adolescent and adolescent patients with XSCID. Findings suggest that the somatotropic axis revealed profoundly diminished IGF-I production following recombinant GH (rGH) challenge in all three patients.
- Subjects
PEDIATRIC research; GROWTH hormone releasing factor; SOMATOMEDIN; SOMATOTROPIN; SEVERE combined immunodeficiency; SHORT stature; PITUITARY dwarfism
- Publication
Journal of Pediatric Endocrinology & Metabolism, 2008, Vol 21, Issue 11, p1057
- ISSN
0334-018X
- Publication type
Article
- DOI
10.1515/JPEM.2008.21.11.1057