Article
Congenital adrenal hyperplasia: management during critical
illness
E Charmandaria, E J Lichtarowicz-Krynskaa, P C Hindmarsha, A Johnstonb, A Aynsley-Greena, C G D Brooka
a London Centre for
Paediatric Endocrinology, Great Ormond Street Hospital and The
Institute of Child Health, University College London, London WC1N
3JH, UK, b Department of Clinical Pharmacology, St
Bartholomew's and the Royal London School of Medicine and Dentistry,
London EC1M 6BQ, UK
Correspondence to: Dr E Charmandari, Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892-1583, USA charmane{at}mail.nih.gov
Accepted 19 February
2001
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.
AIMTo 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.
METHODSPlasma 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/m2; 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.
RESULTSIn 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.
CONCLUSIONSCritically ill patients
with classical CAH may be best managed with a single intravenous
hydrocortisone bolus followed by a constant rate infusion of hydrocortisone.
Keywords: congenital adrenal hyperplasia; critical illness; hydrocortisone; cortisol clearance
© 2001 by Archives of Disease in Childhood
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Arch. Dis. Child. 2001 85: 0.[Extract] [Full Text] [PDF]
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