ArticleJournal of pediatric endocrinology & metabolism : JPEM2008
Short stature in partially corrected X-linked severe combined immunodeficiency--suboptimal response to growth hormone.
Article in Journal of pediatric endocrinology & metabolism : JPEM, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
4 citing papers in PubMed, 6 citations in OpenAlex.
- X-linked SCID with a rare mutation.Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology · 2021Article
- Human conditions of insulin-like growth factor-I (IGF-I) deficiency.Journal of translational medicine · 2012Review
- The genetic basis of severe combined immunodeficiency and its variants.The application of clinical genetics · 2012Article
- Partially corrected X-linked severe combined immunodeficiency: long-term problems and treatment options.Immunologic research · 2009Review
Corrections and comments
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Authors and funding
7 authors at 2 institutions in 1 country.
Funding
Abstract
backgroundX-linked severe combined immunodeficiency (XSCID) results from defects in the common cytokine receptor gamma chain (gamma c) required for signaling by receptors for interleukin (IL)-2, -4, -7, -9, -15, and -21. Following haploidentical bone marrow transplant without myelo-conditioning for XSCID, most patients achieve partial reconstitution often limited to T lymphocytes. Many partially corrected patients manifest extreme short stature (<5th percentile). Previous reports have implicated gamma c in growth hormone (GH) receptor signaling, thus severe growth failure in XSCID may be related to the underlying gamma c defect.
aimTo evaluate the GH/insulin-like growth factor-I (IGF-I) axis in three children with XSCID and partial immune reconstitution with profound growth failure.
methodsThe IGF-I generation test was performed by administering recombinant GH subcutaneously for 5 days, and measuring serum levels for IGF-I before GH injection, and on days 5 and 8.
resultsStudy of the somatotropic axis revealed profoundly diminished IGF-I production following rGH challenge in all three patients.
conclusionThe data indicate that the GH/IGF-I axis in these partially corrected XSCID patients with severe short stature is profoundly impaired, and supports previous studies suggesting that the underlying gamma c defect may contribute to the severe growth failure in XSCID. This supports a role for defective gamma c in the extreme short stature of XSCID, and raises the possibility of recombinant IGF-I treatment to bypass this defect.
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