Walter Bonfig1,2, Friedhelm Roehl3, Stefan Riedl4, Jürgen Brämswig5, Annette Richter-Unruh5, Susanne Fricke-Otto6, Angela Hübner7, Markus Bettendorf8, Eckhard Schönau9, Helmut Dörr10, Reinhard W Holl11, Klaus Mohnike12. 1. Department of Pediatrics, Klinikum Wels-Grieskirchen, Wels-Grieskirchen, Austria. 2. Pediatric Endocrinology, Department of Pediatrics, Technische Universität München, München, Germany. 3. Department of Biometrics, Otto von Guericke Universität Magdeburg, Magdeburg, Germany. 4. Pediatric Endocrinology, St. Anna Kinderspital, University of Vienna, Vienna, Austria. 5. Pediatric Endocrinology, Department of Pediatrics, Universitätsklinikum Münster, Westfälische Wilhelmsuniversität Münster, Münster, Germany. 6. Pediatric Endocrinology, Department of Pediatrics, Helios Klinikum Krefeld, Krefeld, Germany. 7. Pediatric Endocrinology, Department of Pediatrics, Universitätsklinikum Dresden, Technische Universität Dresden, Dresden, Germany. 8. Division of Pediatric Endocrinology and Diabetes, Department of Pediatrics, Universitätsklinikum Heidelberg, Ruprecht-Karls-Universität Heidelberg, Heidelberg, Germany. 9. Pediatric Endocrinology, Department of Pediatrics, Universitätsklinikum Köln, Universität zu Köln, Köln, Germany. 10. Pediatric Endocrinology, Department of Pediatrics, Universitätsklinikum Erlangen, Friedrich Alexander Universität Erlangen, Erlangen, Germany. 11. Institute of Epidemiology and Medical Biometry (ZIBMT), University of Ulm, Ulm, Germany. 12. Pediatric Endocrinology, Department of Pediatrics, Otto von Guericke Universität Magdeburg, Magdeburg, Germany.
Abstract
INTRODUCTION: Sodium chloride supplementation in salt-wasting 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 2011-2015. 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.
INTRODUCTION:Sodium chloride supplementation in salt-wasting 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 2011-2015. 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.
Authors: Phyllis W Speiser; Wiebke Arlt; Richard J Auchus; Laurence S Baskin; Gerard S Conway; Deborah P Merke; Heino F L Meyer-Bahlburg; Walter L Miller; M Hassan Murad; Sharon E Oberfield; Perrin C White Journal: J Clin Endocrinol Metab Date: 2018-11-01 Impact factor: 5.958
Authors: Hedi L Claahsen-van der Grinten; Phyllis W Speiser; S Faisal Ahmed; Wiebke Arlt; Richard J Auchus; Henrik Falhammar; Christa E Flück; Leonardo Guasti; Angela Huebner; Barbara B M Kortmann; Nils Krone; Deborah P Merke; Walter L Miller; Anna Nordenström; Nicole Reisch; David E Sandberg; Nike M M L Stikkelbroeck; Philippe Touraine; Agustini Utari; Stefan A Wudy; Perrin C White Journal: Endocr Rev Date: 2022-01-12 Impact factor: 19.871
Authors: Uta Neumann; Annelieke van der Linde; Ruth E Krone; Nils P Krone; Ayla Güven; Tülay Güran; Heba Elsedfy; Sukran Poyrazoglu; Feyza Darendeliler; Tania A S S Bachega; Antonio Balsamo; Sabine E Hannema; Niels Birkebaek; Ana Vieites; Ajay Thankamony; Martine Cools; Tatjana Milenkovic; Walter Bonfig; Eduardo Correa Costa; Navoda Atapattu; Liat de Vries; Guilherme Guaragna-Filho; Marta Korbonits; Klaus Mohnike; Jillian Bryce; S Faisal Ahmed; Bernard Voet; Oliver Blankenstein; Hedi L Claahsen-van der Grinten Journal: Eur J Endocrinol Date: 2022-04-11 Impact factor: 6.558