Contemporary global management of 21-hydroxylase deficiency congenital adrenal hyperplasia in early infancy: a multi-national registry study.
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Lim David B N, Bryce Jillian, Ali Salma R, Tseretopoulou Xanthippi, Birkebaek Niels H, Hannema Sabine E, Campos-Martorell Ariadna, Clemente Maria, Neumann Uta, Flück Christa E, Metzger Sara, Krone Ruth E, German Alina, Baronio Federico, Barat Pascal, Delagrange Marine, Vautier Vanessa, Vieites Ana, Rey Rodolfo, Elsedfy Heba, Atapattu Navoda, Seneviratne Sumudu N, Cools Martine, Guran Tulay, Yavas Abali Zehra, Fu Antony, Janus Dominika, Shenoy Savitha, Wasniewska Malgorzata, Coco Roberto, Russo Gianni, Stancampiano Marianna Rita, Bonfig Walter, Salerno Mariacarolina, Claahsen-van der Grinten Hedi L, Adriaansen Bas P H, Mozzato Chiara, Guazzarotti Laura, Niedziela Marek, Banaszak-Ziemska Magdalena, van Eck Judith, Bachega Tania, Miranda Mirela C, Marginean Otilia, Munarin Jessica, De Sanctis Luisa, Probst-Scheidegger Ursina, Lenherr-Taube Nina, Konrad Daniel, O'Connell Michele, Gawlik-Starzyk Aneta, Sandberg David E, Shnorhavorian Margarett, Krone Nils, Ahmed S Faisal, Davies Justin H
Аннотация
DESIGN AND METHODS: Using the I-CAH Registry, we retrospectively reviewed management over the first 90 days of life of 154 infants with 21-OHD born in 2018-2023, across 33 centers in 18 countries.
RESULTS: Of 154 infants (92 female, 62 male), 136 were diagnosed postnatally, with median (10th centile, 90th centile) presentation age of Day 4 (0, 20.8). At initial hospital discharge, median doses of hydrocortisone (HC), fludrocortisone (FC), and salt were 17 (11.4, 39.6) mg/m2/day, 100 (50, 200) mcg/day and 3.5 (1.6, 8.7) mmol/kg/day, and at Day 90 (D90) 14.5 (8.7, 24.1) mg/m2/day, 100 (50, 200) mcg/day, and 2.1 (1.0, 5.2) mmol/kg/day, respectively. Hyponatremia, hyperkalemia, and hypoglycemia were reported in 70.0%, 71.9%, and 13.0% of infants, respectively. At D90, hyponatremia and hyperkalemia were reported in 7.4% and 28.6%, respectively. At D90, BP measurements were recorded in 30.5%, amongst whom 31.9% had hypertension reported. Median total hospitalization duration over 90 days was 9 days (2, 24). Adrenal crises were associated with 40. 6% of hospitalization episodes. Percentages (males:females) of cases seen by a pediatric endocrinologist, psychologist, pediatric endocrine nurse specialist, and surgeon by D90 were 95.9% (58:84), 33.3% (9:35), 42.1% (20:36), and 23.8% (0:35), respectively.
CONCLUSIONS: Contemporary management of CAH in early infancy varies considerably. Hypertension and hyperkalemia are frequently reported. Our data may help inform development of quality indicators for benchmarking CAH care in infancy.