Despite increased adherence to glucocorticoid stress dosing guidelines,
patients with adrenal insufficiency are still at risk for acute adrenal
failure, according to data published in the Journal of Pediatrics.
Patients with adrenal insufficiency not only require daily
glucocorticoid therapy, but they must also receive higher doses during stress,
researchers from Sainte-Justine Hospital in Montreal wrote. The Lawson Wilkins
Pediatric Endocrine Society (LWPES) developed guidelines to address these
needs, yet few studies have described the effect of the recommendations.
When the LWPES published its guidelines on stress coverage in
adrenal failure and I tried to find evidence to support what they recommended
and what they also have been doing in our center for more than 30 years
there were very little data in the literature. In particular, we could
find very little documentation of what happens in the hospital with these
patients and if what we do, or do not do, with [glucocorticoid] stress coverage
makes a difference, Cheri L. Deal, PhD, MD, president of the
Canadian Society of Endocrinology and Metabolism, told Endocrine Today.
This lack of information, as well as the death of a patient with
septo-optic dysplasia and panhypopituitarism from septicemia at their center,
prompted Deal and colleagues to investigate the degree to which these
guidelines are followed and what role inappropriate care may play in acute
The researchers conducted a retrospective study involving 102 patients
with primary adrenal insufficiency and 34 patients with secondary adrenal
insufficiency seen at their hospital from 1973 to 2007. None of the patients
had a history of central nervous system tumors or other malignancy. Analysis
was divided into three periods: before 1990; 1990 to 1997; and 1998 to 2007.
Each period represented progress made in hospital and patient care.
Of 247 total hospitalizations, 201 occurred in patients with primary
adrenal insufficiency and 46 in those with secondary adrenal insufficiency.
Sixty-four percent of hospitalizations were classified as urgent, with no
sex-related differences appearing for patients with either primary or secondary
adrenal insufficiency, according to the researchers. Approximately one-third of
all admissions were elective.
Before hospitalization, only 31.2% of patients received increased
glucocorticoid doses at home, despite the guidelines and physician
recommendations to the contrary. In addition, the glucocorticoids were injected
intramuscularly in only five cases resulting in urgent hospitalization.
However, the number of parents using glucocorticoid stress doses before urgent
hospitalization increased from 17.5% during 1973 to 1989 to 47% during 1998 to
It is important to indicate that we did not collect data about
home management or management in the ED when no hospitalization followed, so
that this is, we hope, a worse-case scenario of stress dose usage by
caregivers, Deal said.
Results also revealed a striking improvement in
administration of glucocorticoid stress doses in the ED after 1989, the
researchers said, with the proportion of stress doses received by patients
escalating from 40% before 1990 to 67.6% during 1990 to 1997 and 65.2% during
1998 to 2007. The researchers also noted, however, that evidence of acute
adrenal failure was present in 36.9% of the 157 urgent hospitalizations in the
In some cases, decompensation probably occurred too quickly for
the parents to react, as another smaller study by Japanese investigators
recently pointed out, Deal said.
In 23.5% of all hospitalizations, health care professionals did not
follow stress protocol for glucocorticoid treatment. In 27 cases, the endocrine
service was not consulted. Morbidity, as defined by clinical evidence of acute
adrenal failure after delayed or missed doses, was minimal, with only two cases
of acute adrenal failure that were managed clinically without sequelae.
Our study documents an improvement in patient care with the
introduction of more formal protocols for stress management in patients with
adrenal failure, although affected individuals still show morbidity, which is
Some of this morbidity may be eliminated with a more conscientious
application of glucocorticoid stress doses, but there is still a subset of
patients at higher risk because of their underlying pathology, such as in the
case of septo-optic dysplasia, or because of immunologic and/or genetic factors
influencing glucocorticoid bioavailability, metabolism and action, Deal
said. by Melissa Foster
For more information:
- Leblicq C. J Pediatr. 2011;158:492-498.
Disclosure: Dr. Deal and colleagues report no relevant financial
Janet H. Silverstein
The article by Leblicq et al highlights the fact that despite clear,
well-publicized protocols for stress-management of patients with adrenal
insufficiency, a significant percentage of patients still remain under-treated
when hospitalized for illness or surgery. This literature review was prompted
by the death of a young child with central adrenal insufficiency. In this case,
there was insufficient glucocorticoid administration due to a lack of venous
access when the child was febrile.
This case, however, is not an isolated one, and underscores the need to
repeatedly educate all medical personnel, particularly those who are the first
responders such as the ED staff and hospital physicians about the need for
immediate treatment with high-dose glucocorticoid administration for all
patients ill enough to require ED or hospital care. Endocrinologists are
generally careful to stress the importance of providing parents of children
with adrenal insufficiency handouts and written instructions on how to manage
vomiting or other stressors and to train them in the use of parenteral
glucocorticoids if the child is unable to take medications orally. However, too
often, treatment in the hospital is delayed because medications arent
ordered promptly, because IV access is not available or because there is a long
delay in transfer from the ED to the wards. It is, therefore, extremely
important that no child with adrenal insufficiency be admitted to the hospital
from the ED without first receiving stress doses of glucocorticoids.
Janet H. Silverstein, MD
Endocrine Today Editorial Board Member
Disclosure: Dr. Silverstein reports no