Universität zu Köln

Hoyer-Kuhn, Heike, Franklin, Jeremy ORCID: 0000-0003-1536-0925, Jones, Christine, Blum, Werner F. and Schoenau, Eckhard (2018). Growth response to growth hormone treatment in patients with SHOX deficiency can be predicted by the Cologne prediction model. J. Pediatr. Endocrinol. Metab., 31 (1). S. 25 - 32. BERLIN: WALTER DE GRUYTER GMBH. ISSN 2191-0251

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Abstract

Background: Growth hormone (GH) treatment in children with short stature homeobox-containing gene (SHOX) deficiency is recognized to increase height velocity (HV) and adult height. Prediction of growth response continues to be a challenge. A comparatively accurate method is the Cologne prediction model developed in children with GH deficiency. The aim was to investigate whether this model also applies to patients with SHOX deficiency. Methods: Included were 48 patients with SHOX deficiency confirmed by DNA analysis and treated with 0.05 mg/kg/day of somatropin. Prediction by the Cologne model uses the following variables: relative bone age (BA) retardation , baseline insulin-like growth factor-I (IGF-I), urinary deoxypyridinoline (DPD) cross-links at 4 weeks and HV at 3 months. Results: HV and height standard deviation scores (SDS) increased significantly during the first year of treatment. Predicted and observed HV (cm/year) showed a Pearson correlation coefficient of 0.50 (p < 0.001; root-mean-square error = 1.63) and for first-year change in height SDS a Pearson correlation coefficient of 0.751 (p < 0.001; root-mean-square error = 0.32). Poor response could be adequately predicted using SDS change, with sensitivity and specificity both above 70% for certain thresholds. Conclusions: The results demonstrate that the Cologne model can be used to predict growth response in patients with SHOX deficiency with reasonable precision in the first treatment year, comparable to prediction in patients with GH deficiency.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Hoyer-Kuhn, Heike UNSPECIFIED UNSPECIFIED UNSPECIFIED Franklin, Jeremy UNSPECIFIED orcid.org/0000-0003-1536-0925 UNSPECIFIED Jones, Christine UNSPECIFIED UNSPECIFIED UNSPECIFIED Blum, Werner F. UNSPECIFIED UNSPECIFIED UNSPECIFIED Schoenau, Eckhard UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-204599
DOI:	10.1515/jpem-2017-0282
Journal or Publication Title:	J. Pediatr. Endocrinol. Metab.
Volume:	31
Number:	1
Page Range:	S. 25 - 32
Date:	2018
Publisher:	WALTER DE GRUYTER GMBH
Place of Publication:	BERLIN
ISSN:	2191-0251
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language IDIOPATHIC SHORT STATURE; LERI-WEILL DYSCHONDROSTEOSIS; CHILDHOOD SHORT STATURE; FOR-GESTATIONAL-AGE; HOMEOBOX GENE SHOX; TURNER-SYNDROME; GH TREATMENT; MAJOR DETERMINANTS; MULTICENTER TRIAL; FINAL HEIGHT Multiple languages Endocrinology & Metabolism; Pediatrics Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/20459