Short stature in partially corrected x-linked severe combined immunodeficiency - Suboptimal response to growth hormone

Suk See De Ravin, Elaine Shum, Kol A. Zarember, Geoffrey Rezvani, Ronald (Ron) Rosenfeld, Constantine A. Stratakis, Harry L. Malech

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

Background: X-linked severe combined immunodeficiency (XSCID) results from defects in the common cytokine receptor γ chain (γc) 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 (

Original languageEnglish (US)
Pages (from-to)1057-1063
Number of pages7
JournalJournal of Pediatric Endocrinology and Metabolism
Volume21
Issue number11
StatePublished - Nov 2008
Externally publishedYes

Fingerprint

X-Linked Combined Immunodeficiency Diseases
Severe Combined Immunodeficiency
Growth Hormone
Interleukin-4 Receptors
Cytokine Receptors
Interleukin-2 Receptors
Bone Marrow
T-Lymphocytes
Transplants

Keywords

  • γc
  • Growth hormone
  • IGF-I
  • Short stature
  • X-linked severe combined immunodeficiency

ASJC Scopus subject areas

  • Endocrinology
  • Endocrinology, Diabetes and Metabolism
  • Pediatrics, Perinatology, and Child Health

Cite this

De Ravin, S. S., Shum, E., Zarember, K. A., Rezvani, G., Rosenfeld, R. R., Stratakis, C. A., & Malech, H. L. (2008). Short stature in partially corrected x-linked severe combined immunodeficiency - Suboptimal response to growth hormone. Journal of Pediatric Endocrinology and Metabolism, 21(11), 1057-1063.

Short stature in partially corrected x-linked severe combined immunodeficiency - Suboptimal response to growth hormone. / De Ravin, Suk See; Shum, Elaine; Zarember, Kol A.; Rezvani, Geoffrey; Rosenfeld, Ronald (Ron); Stratakis, Constantine A.; Malech, Harry L.

In: Journal of Pediatric Endocrinology and Metabolism, Vol. 21, No. 11, 11.2008, p. 1057-1063.

Research output: Contribution to journalArticle

De Ravin, SS, Shum, E, Zarember, KA, Rezvani, G, Rosenfeld, RR, Stratakis, CA & Malech, HL 2008, 'Short stature in partially corrected x-linked severe combined immunodeficiency - Suboptimal response to growth hormone', Journal of Pediatric Endocrinology and Metabolism, vol. 21, no. 11, pp. 1057-1063.
De Ravin, Suk See ; Shum, Elaine ; Zarember, Kol A. ; Rezvani, Geoffrey ; Rosenfeld, Ronald (Ron) ; Stratakis, Constantine A. ; Malech, Harry L. / Short stature in partially corrected x-linked severe combined immunodeficiency - Suboptimal response to growth hormone. In: Journal of Pediatric Endocrinology and Metabolism. 2008 ; Vol. 21, No. 11. pp. 1057-1063.
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AU - Stratakis, Constantine A.

AU - Malech, Harry L.

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