Universität zu Köln

Bonfig, Walter, Roehl, Friedhelm, Riedl, Stefan, Braemswig, Jurgen, Richter-Unruh, Annette, Fricke-Otto, Susanne, Huebner, Angela, Bettendorf, Markus, Schoenau, Eckhard, Doerr, Helmut, Holl, Reinhard W. and Mohnike, Klaus (2018). Sodium Chloride Supplementation Is Not Routinely Performed in the Majority of German and Austrian Infants with Classic Salt-Wasting Congenital Adrenal Hyperplasia and Has No Effect on Linear Growth and Hydrocortisone or Fludrocortisone Dose. Horm. Res. Paediatr., 89 (1). S. 7 - 13. BASEL: KARGER. ISSN 1663-2826

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Abstract

Introduction: Sodium chloride supplementation in saltwasting congenital adrenal hyperplasia (CAH) is generally recommended in infants, but its implementation in routine care is very heterogeneous. Objective: To evaluate oral sodium chloride supplementation, growth, and hydrocortisone and fludrocortisone dose in infants with salt-wasting CAH due to 21-hydroxylase in 311 infants from the AQUAPE CAH database. Results: Of 358 patients with classic CAH born between 1999 and 2015, 311 patients had salt-wasting CAH (133 females, 178 males). Of these, 86 patients (27.7%) received oral sodium chloride supplementation in a mean dose of 0.9 +/- 1.4 mmol/kg/day (excluding nutritional sodium content) during the first year of life. 225 patients (72.3%) were not treated with sodium chloride. The percentage of sodium chloride-supplemented patients rose from 15.2% in children born 1999-2004 to 37.5% in children born 20112015. Sodium chloride-supplemented and -unsupplemented infants did not significantly differ in hydrocortisone and fludrocortisone dose, target height-corrected height-SDS, and BMI-SDS during the first 2 years of life. Conclusion: In the AQUAPE CAH database, approximately one-third of infants with salt-wasting CAH receive sodium chloride supplementation. Sodium chloride supplementation is performed more frequently in recent years. However, salt supplementation had no influence on growth, daily fludrocortisone and hydrocortisone dose, and frequency of adrenal crisis. (C) 2017 S. Karger AG, Basel

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Bonfig, Walter UNSPECIFIED UNSPECIFIED UNSPECIFIED Roehl, Friedhelm UNSPECIFIED UNSPECIFIED UNSPECIFIED Riedl, Stefan UNSPECIFIED UNSPECIFIED UNSPECIFIED Braemswig, Jurgen UNSPECIFIED UNSPECIFIED UNSPECIFIED Richter-Unruh, Annette UNSPECIFIED UNSPECIFIED UNSPECIFIED Fricke-Otto, Susanne UNSPECIFIED UNSPECIFIED UNSPECIFIED Huebner, Angela UNSPECIFIED UNSPECIFIED UNSPECIFIED Bettendorf, Markus UNSPECIFIED UNSPECIFIED UNSPECIFIED Schoenau, Eckhard UNSPECIFIED UNSPECIFIED UNSPECIFIED Doerr, Helmut UNSPECIFIED UNSPECIFIED UNSPECIFIED Holl, Reinhard W. UNSPECIFIED UNSPECIFIED UNSPECIFIED Mohnike, Klaus UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-204051
DOI:	10.1159/000481775
Journal or Publication Title:	Horm. Res. Paediatr.
Volume:	89
Number:	1
Page Range:	S. 7 - 13
Date:	2018
Publisher:	KARGER
Place of Publication:	BASEL
ISSN:	1663-2826
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language 21-HYDROXYLASE DEFICIENCY; CHILDREN; DIAGNOSIS; LIFE Multiple languages Endocrinology & Metabolism; Pediatrics Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/20405