medwireNews: Hypoglycaemia is a relatively frequent and serious finding in patients with congenital adrenal hyperplasia (CAH) during an adrenal crisis, and can be fatal, say Japanese researchers.

The team reports that hypoglycaemia was present in 29% of 53 CAH patients who had an adrenal crisis. These patients were among 378 identified in a nationwide survey; 62 (17.2%) patients had at least one adrenal crisis, and clinical data were available for 53 of these.

The children were aged a median of 2 years at the time of their adrenal crisis, and the majority (62%) had only one crisis, although one child had nine hospital admissions for adrenal crises. Most children were admitted with fever (73%) and/or vomiting (71%). Diarrhoea was also common (28%), and a sizable minority of patients had impaired consciousness (14%) and convulsion (12%).

Hypoglycaemia (29%) was the second-most common biochemical abnormality, following hyponatraemia (36%), and was significantly associated with impaired consciousness and convulsions – patients were respectively around fivefold and eightfold more likely to have these complications if they had hypoglycaemia.

One patient died, at the age of 2 years, as a direct consequence of co-occurring severe hypoglycaemia, which resulted in intractable seizure, leading to brain herniation and death.

Tomohiro Ishii (Keio University School of Medicine) and co-researchers note that this patient had no obvious precipitating factors for adrenal crisis, which was true of 12.5% of patients overall.

“Hyperketonemic hypoglycemia is not only an outcome of adrenal crisis, but also a possible precipitating factor of adrenal crisis during these ages that may be overlooked”, they write in Hormone Research in Paediatrics.

“Hypoglycemia often occurs with nonspecific symptoms and signs and seems to be more common in children than in adults”, says the team. They stress that, although parents should be warned of the dangers of infection, which was the triggering factor in the vast majority of children in this and other studies, clinicians should also emphasise that adrenal crisis may occur in the absence of a clear precipitating factor.

“Based on the results of the survey, pediatric endocrinologists should acknowledge that adrenal crisis is not rare and still potentially associated with fatal outcome”, conclude the researchers.

They add: “It still remains challenging to prevent adrenal crisis and to avoid unnecessary death.”

Eleanor McDermid

Horm Res Paediatr 2018; Advance online publication

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