To determine physicians' beliefs and practices regarding adrenal dysfunction in pediatric critical illness.
Cross-sectional mail survey.
All members of the Canadian Pediatric Endocrine Group and all physicians identified as practicing pediatric intensive care medicine in any of 16 tertiary care teaching centers in Canada.
Three pediatric intensive care physicians and three pediatric endocrinologists reviewed the questionnaire before administration to ensure clarity. We asked participants to report their views on the following: a) the frequency of adrenal insufficiency in pediatric critical illness; b) diagnosis/definition of adrenal insufficiency in pediatric critical illness; and c) the use of empirical glucocorticoids in fluid/vasopressor-resistant hypotension in pediatric critical illness.
Forty-six of 57 (80.7%) endocrinologists responded, with 43 participating (75.4%). Among intensivists, 59 of 70 (84.3%) responded with no refusals. Of intensivists, 81.4% believe that adrenal insufficiency occurs sometimes or often in critically ill pediatric intensive care unit patients, whereas 41.8% of endocrinologists believe adrenal insufficiency occurs never or rarely in these patients. Six definitions of adrenal insufficiency were proposed (varying cortisol level vs. peak/increment of cortisol in response to corticotropin), with no consensus on the definition of adrenal insufficiency from the endocrinologists or intensivists. Half (50.9%) of intensivists said they would sometimes or often empirically treat hypotensive pediatric patients with glucocorticoids, whereas 81.0% of endocrinologists would occasionally or never recommend glucocorticoids on this basis.
There is no consensus among pediatric intensivists or endocrinologists as to how often adrenal insufficiency occurs in pediatric critical illness or how to diagnose this condition. Despite this lack of consensus, however, many pediatric intensivists would empirically treat hypotensive patients who they suspect may have adrenal insufficiency. Prospective studies are required to determine the definition, frequency, and appropriate treatment of adrenal insufficiency in critically ill pediatric patients.
Comment In: Pediatr Crit Care Med. 2007 May;8(3):305-717496522
X-linked congenital adrenal hypoplasia (AHC) is a developmental disorder of the human adrenal gland that results in profound hormonal deficiencies, which are lethal if untreated. Hypogonadotropic hypogonadism (HHG) is frequently associated with this disorder. The gene (DAX-1) responsible for the disease has recently been isolated. It encodes a protein with large similarity to members of the nuclear hormone receptor superfamily. Several different mutations in this gene have been found in patients suffering from AHC. We have identified a missense mutation (N440I) in three patients with AHC and HHG, all belonging to a large Greenlandic family. A total of 42 individuals has been tested for this mutation. We have diagnosed 10 women as carriers, and have excluded 22 women with a 25-50% risk from being carriers, emphasizing the rapid impact of molecular genetic techniques.