title: Frequency and causes of adrenal crises over lifetime in patients with 21-hydroxylase deficiency.
abstract: OBJECTIVE
  To study adrenal crisis (AC) in patients with congenital adrenal hyperplasia due to classical 21-hydroxylase deficiency (21-OHD). AC was defined as an acute state of health impairment requiring i.v. glucocorticoid administration and hospital admission.
  
  
  METHODS
  In a cross-sectional study with detailed retrospective assessment, AC was studied following two approaches: i) questionnaire based: 122 adult 21-OHD patients (50 men, 72 women, median age 35 years, range 18-69 years) completed a disease-specific questionnaire; and ii) patient chart based: charts of 67 21-OHD patients (32 males, 35 females, median age 31 years, range 20-66 years) were analyzed from diagnosis to last follow-up with regard to frequency and causes of AC since diagnosis.
  
  
  RESULTS
  Evaluation of questionnaires revealed 257 ACs in 4456 patient years (py; frequency 5.8 crises/100 py), while patient charts documented 106 ACs in 2181 py (4.9 crises/100 py). The chart-based evaluation showed that gastrointestinal infections (29%) and salt-wasting crisis (18%) were the main causes of AC. In 14%, the cause remained uncertain. There was no difference in the overall frequency of AC in males and females. AC mostly occurred during childhood, with more than 70% of AC in the first 10 years of life and one-third of AC in the first year of life. Still, 20% of cases of AC were observed in adults (>18 years).
  
  
  CONCLUSIONS
  Our data demonstrate a significant risk of AC in patients with 21-OHD over lifetime. Specific age-adapted and repeated crisis prevention training may help to reduce morbidity due to AC in 21-OHD.
authors.affiliation: Endocrine Research Unit, Medizinische Klinik und Poliklinik IV - Campus Innenstadt, Klinikum der Universität München, Endokrinologie, Ziemssenstrasse 1, D-80336 München, Germany. nicole.reisch@med.uni-muenchen.de
authors.forename: Nicole
authors.initials: N
authors.lastname: Reisch
authors.forename: Marina
authors.initials: M
authors.lastname: Willige
authors.forename: Denise
authors.initials: D
authors.lastname: Kohn
authors.forename: Hans-Peter
authors.initials: HP
authors.lastname: Schwarz
authors.forename: Bruno
authors.initials: B
authors.lastname: Allolio
authors.forename: Martin
authors.initials: M
authors.lastname: Reincke
authors.forename: Marcus
authors.initials: M
authors.lastname: Quinkler
authors.forename: Stefanie
authors.initials: S
authors.lastname: Hahner
authors.forename: Felix
authors.initials: F
authors.lastname: Beuschlein
mesh_terms: D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000150:complications* / Q000188:drug therapy; D000309:Adrenal Insufficiency / Q000175:diagnosis* / Q000188:drug therapy* / Q000209:etiology; D000328:Adult; D000367:Age Factors; D000368:Aged; D003430:Cross-Sectional Studies; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012307:Risk Factors; D011795:Surveys and Questionnaires
chemical_list: D005938:Glucocorticoids