Tomohiro Ishii1,2, Masanori Adachi1,3, Kei Takasawa1,4, Satoshi Okada1,5, Hotaka Kamasaki1,6, Takuo Kubota1,7, Hironori Kobayashi1,8, Hirotake Sawada1,9, Keisuke Nagasaki1,10, Chikahiko Numakura1,11, Shohei Harada1,12, Kanshi Minamitani1,13, Shigetaka Sugihara1,14, Toshihiro Tajima1,15. 1. The Committee on Mass Screening, Japanese Society for Pediatric Endocrinology, Kyoto, Japan. 2. Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan. 3. Department of Endocrinology and Metabolism, Kanagawa Children's Medical Center, Yokohama, Japan. 4. Department of Pediatrics and Developmental Biology, Tokyo Medical and Dental University, Tokyo, Japan. 5. Department of Pediatrics, Hiroshima University Graduate School of Biomedical and Health Sciences, Hiroshima, Japan. 6. Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Japan. 7. Department of Pediatrics, Osaka University Graduate School of Medicine, Osaka, Japan. 8. Department of Pediatrics, Shimane University Faculty of Medicine, Shimane, Japan. 9. Department of Reproductive and Developmental Medicine, University of Miyazaki, Miyazaki, Japan. 10. Division of Pediatrics, Department of Homeostatic Regulation and Development, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan. 11. Department of Pediatrics, Yamagata University School of Medicine, Yamagata, Japan. 12. Department of Child Studies, Seitoku University, Matsudo, Japan. 13. Department of Pediatrics, Teikyo University Chiba Medical Center, Chiba, Japan. 14. Department of Pediatrics, Tokyo Women's Medical University Medical Center East, Tokyo, Japan. 15. Department of Pediatrics, Jichi Medical University Tochigi Childrens Medical Center, Shimotsuke, Japan.
Abstract
BACKGROUND/AIMS: We aimed to evaluate the incidence and characteristics of adrenal crisis in Japanese children with 21-hydroxylase deficiency (21-OHD). METHODS: We conducted a retrospective nationwide survey for the councilors of the Japanese Society for Pediatric Endocrinology (JSPE) regarding adrenal crisis in children under 7 years with 21-OHD, admitted to hospitals from 2011 through 2016. We defined adrenal crisis as the acute impairment of general health due to glucocorticoid deficiency with at least two of symptoms, signs, or biochemical abnormalities. RESULTS: The councilors of the JSPE in 83 institutions responded to this survey (response rate, 60.1%). Data analyses of 378 patients with 1,101.4 person-years (PYs) revealed that 67 patients (17.7%) experienced at least 1 episode of hospital admission for adrenal crisis at the median age of 2 years. The incidence of adrenal crisis was calculated as 10.9 per 100 PYs (95% confidence interval [CI] 9.6-12.2). Infections were the most common precipitating factors, while no factor was observed in 12.5%. Hypoglycemia occurred concomitantly in 27.4%. One patient died from severe hypoglycemia, resulting in a mortality rate of 0.09 per 100 PYs (95% CI 0.0-0.2). CONCLUSION: Adrenal crisis is not rare and can be accompanied by disastrous hypoglycemia in children with 21-OHD.
BACKGROUND/AIMS: We aimed to evaluate the incidence and characteristics of adrenal crisis in Japanese children with 21-hydroxylase deficiency (21-OHD). METHODS: We conducted a retrospective nationwide survey for the councilors of the Japanese Society for Pediatric Endocrinology (JSPE) regarding adrenal crisis in children under 7 years with 21-OHD, admitted to hospitals from 2011 through 2016. We defined adrenal crisis as the acute impairment of general health due to glucocorticoid deficiency with at least two of symptoms, signs, or biochemical abnormalities. RESULTS: The councilors of the JSPE in 83 institutions responded to this survey (response rate, 60.1%). Data analyses of 378 patients with 1,101.4 person-years (PYs) revealed that 67 patients (17.7%) experienced at least 1 episode of hospital admission for adrenal crisis at the median age of 2 years. The incidence of adrenal crisis was calculated as 10.9 per 100 PYs (95% confidence interval [CI] 9.6-12.2). Infections were the most common precipitating factors, while no factor was observed in 12.5%. Hypoglycemia occurred concomitantly in 27.4%. One patient died from severe hypoglycemia, resulting in a mortality rate of 0.09 per 100 PYs (95% CI 0.0-0.2). CONCLUSION: Adrenal crisis is not rare and can be accompanied by disastrous hypoglycemia in children with 21-OHD.
Authors: Bradley S Miller; Sandra P Spencer; Mitchell E Geffner; Evgenia Gourgari; Amit Lahoti; Manmohan K Kamboj; Takara L Stanley; Naveen K Uli; Brandy A Wicklow; Kyriakie Sarafoglou Journal: J Investig Med Date: 2019-02-28 Impact factor: 2.895
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Authors: Salma R Ali; Jillian Bryce; Houra Haghpanahan; James D Lewsey; Li En Tan; Navoda Atapattu; Niels H Birkebaek; Oliver Blankenstein; Uta Neumann; Antonio Balsamo; Rita Ortolano; Walter Bonfig; Hedi L Claahsen-van der Grinten; Martine Cools; Eduardo Correa Costa; Feyza Darendeliler; Sukran Poyrazoglu; Heba Elsedfy; Martijn J J Finken; Christa E Fluck; Evelien Gevers; Márta Korbonits; Guilherme Guaragna-Filho; Tulay Guran; Ayla Guven; Sabine E Hannema; Claire Higham; Ieuan A Hughes; Rieko Tadokoro-Cuccaro; Ajay Thankamony; Violeta Iotova; Nils P Krone; Ruth Krone; Corina Lichiardopol; Andrea Luczay; Berenice B Mendonca; Tania A S S Bachega; Mirela C Miranda; Tatjana Milenkovic; Klaus Mohnike; Anna Nordenstrom; Silvia Einaudi; Hetty van der Kamp; Ana Vieites; Liat de Vries; Richard J M Ross; S Faisal Ahmed Journal: J Clin Endocrinol Metab Date: 2021-01-01 Impact factor: 5.958