title: Congenital adrenal hyperplasia: management during critical illness.
abstract: BACKGROUND
  Little is known of the optimal dose and administration schedule of hydrocortisone in critically ill patients with congenital adrenal hyperplasia (CAH) caused by 21-hydroxylase deficiency.
  
  
  OBJECTIVE
  To determine plasma cortisol concentrations after intravenous administration of hydrocortisone in children with CAH and to relate these to plasma cortisol concentrations achieved by endogenous secretion in the stress of critical illness in previously healthy children.
  
  
  METHODS
  Plasma cortisol concentrations were measured in 20 patients with classical CAH (median age 11.2 years, range 6.1-16.4) following intravenous administration of hydrocortisone 15 mg/m(2); and in 60 critically ill mechanically ventilated children (median age 2.5 years, range 0.25-16.3) on admission to the paediatric intensive care unit and for 24 hours thereafter.
  
  
  RESULTS
  In the CAH patients, plasma cortisol reached a mean peak of 1648.3 nmol/l (SD 511.9) within 10 minutes of the intravenous bolus, and fell rapidly thereafter; levels remained greater than 450 nmol/l for 2.5 hours only. In critically ill children, mean plasma cortisol on admission to the intensive care unit was 727 nmol/l (SD 426.1). Cortisol concentrations remained raised during the first 24 hours.
  
  
  CONCLUSIONS
  Critically ill patients with classical CAH may be best managed with a single intravenous hydrocortisone bolus followed by a constant rate infusion of hydrocortisone.
authors: London Centre for Paediatric Endocrinology, Great Ormond Street Hospital and The Institute of Child Health, University College London, London WC1N 3JH, UK. charmane@mail.nih.gov
authors: E
authors: E
authors: Charmandari
authors: E J
authors: EJ
authors: Lichtarowicz-Krynska
authors: P C
authors: PC
authors: Hindmarsh
authors: A
authors: A
authors: Johnston
authors: A
authors: A
authors: Aynsley-Green
authors: C G
authors: CG
authors: Brook
mesh_terms: D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000097:blood* / Q000150:complications / Q000188:drug therapy*; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage* / Q000097:blood*; D002648:Child; D002675:Child, Preschool; D016638:Critical Illness; D005260:Female; D006207:Half-Life; D006801:Humans; D006854:Hydrocortisone / Q000008:administration & dosage* / Q000097:blood*; D007275:Injections, Intravenous; D016014:Linear Models; D008297:Male; D011446:Prospective Studies
chemical_list: D000893:Anti-Inflammatory Agents; D006854:Hydrocortisone