Universität zu Köln

Zivicnjak, M., Schnabel, D., Staude, H., Even, G., Marx, M., Beetz, R., Holder, M., Billing, H., Fischer, D. -C., Rabl, W., Schumacher, M., Hiort, O. and Haffner, D. (2011). Three-Year Growth Hormone Treatment in Short Children with X-Linked Hypophosphatemic Rickets: Effects on Linear Growth and Body Disproportion. J. Clin. Endocrinol. Metab., 96 (12). S. E2097 - 9. WASHINGTON: ENDOCRINE SOC. ISSN 1945-7197

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Abstract

Context: Children with X-linked hypophosphatemic rickets (XLH) are prone to progressive disproportionate stunting despite oral phosphate and vitamin D treatment. Objective: Our objective was to analyze the effects of GH treatment on stature and lengths of linear body segments in short children with XLH. Design, Settings, and Patients: A 3-yr randomized controlled open-label GH study in short prepubertal children with XLH(n = 16) on phosphate and calcitriol treatment was conducted. A cohort of XLH patients (n = 76) on conservative treatment served as an XLH reference population. Main Outcome Measures: Changes in SD scores (SDS) of stature and linear body segments, i.e. sitting height, leg and arm length, and sitting height index (i.e. ratio between sitting height and stature) were the main outcome measures. Results: XLH patients presented at time of enrollment with significant impairments of stature (-3.3 SDS) and linear body segments compared with healthy children. Leg length (-3.8 SDS) was most impaired, whereas sitting height (-1.7 SDS) was best preserved. The markedly elevated mean sitting height index (+3.3 SDS) reflected severe body disproportion. GH resulted in a sustained increase in linear growth (stature, +1.1 SDS; sitting height, +1.3 SDS; leg length, +0.8 SDS; arm length, +1.1 SDS; each P < 0.05 vs. baseline), whereas no significant changes were observed in controls. Mean height SDS at 3 yr did not significantly differ between groups. Sitting height index remained stable in both the GH-treated patients and in study controls but increased further in the XLH-reference population. Conclusions: The 3-yr GH treatment improved linear growth without progression of body disproportion in short children with XLH. (J Clin Endocrinol Metab 96: E2097-E2105, 2011)

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Zivicnjak, M. UNSPECIFIED UNSPECIFIED UNSPECIFIED Schnabel, D. UNSPECIFIED UNSPECIFIED UNSPECIFIED Staude, H. UNSPECIFIED UNSPECIFIED UNSPECIFIED Even, G. UNSPECIFIED UNSPECIFIED UNSPECIFIED Marx, M. UNSPECIFIED UNSPECIFIED UNSPECIFIED Beetz, R. UNSPECIFIED UNSPECIFIED UNSPECIFIED Holder, M. UNSPECIFIED UNSPECIFIED UNSPECIFIED Billing, H. UNSPECIFIED UNSPECIFIED UNSPECIFIED Fischer, D. -C. UNSPECIFIED UNSPECIFIED UNSPECIFIED Rabl, W. UNSPECIFIED UNSPECIFIED UNSPECIFIED Schumacher, M. UNSPECIFIED UNSPECIFIED UNSPECIFIED Hiort, O. UNSPECIFIED UNSPECIFIED UNSPECIFIED Haffner, D. UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-484022
DOI:	10.1210/jc.2011-0399
Journal or Publication Title:	J. Clin. Endocrinol. Metab.
Volume:	96
Number:	12
Page Range:	S. E2097 - 9
Date:	2011
Publisher:	ENDOCRINE SOC
Place of Publication:	WASHINGTON
ISSN:	1945-7197
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language CROATIAN CHILDREN; FINAL HEIGHT; CANCER RISK; FACTOR I; PHOSPHATE; NEPHROCALCINOSIS; CALCITRIOL; THERAPY; PHEX; METABOLISM Multiple languages Endocrinology & Metabolism Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/48402