Hypoglycaemia in congenital adrenal hyperplasia.

Mackinnon, J; Grant, D B

doi:10.1136/adc.52.7.591

Cited by 16 publications

(8 citation statements)

References 3 publications

(4 reference statements)

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“…However, an explanation is more difficult for several cases of symptomatic hypoglycemia, which occurred despite plausible documentation of compliance with treatment and sick day rules. The prevention of severe hypoglycemia seems not to be completely covered by the current treatment recommendations, although there are reports of hypoglycemia (35,36,37,38,39,40) and adrenomedullary dysfunction (2,41,42) in limited CAH study populations. For example, in a French cohort of 45 children diagnosed with classic CAH between 1981 and 1997, at ages 1-5 years and already under treatment, eight patients had severe hypoglycemic events, two of them lethal, while only three patients had a salt-wasting crisis (18).…”

Section: Discussionmentioning

confidence: 99%

Children with classic congenital adrenal hyperplasia experience salt loss and hypoglycemia: evaluation of adrenal crises during the first 6 years of life

Odenwald

Nennstiel-Ratzel

Dörr

et al. 2016

European Journal of Endocrinology

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Objective: To evaluate adrenal crises after the start of treatment up to the age of 6 years in children with classic congenital adrenal hyperplasia (CAH). Design: Analysis of data extracted from a population-based prospective long-term follow-up study of children detected in neonatal screening. Methods: Data of 102 Bavarian children with classic CAH due to 21-hydroxylase deficiency were analyzed, using parental questionnaires and medical reports. Parent-reported hospital admissions of children diagnosed with acute health impairment were included in the analysis if salt loss (hyponatremia) or hypoglycemia was documented in the discharge summary. Results: A total of 74 children (72.5%) had no report of hospital admissions with salt loss or hypoglycemia during the observational period. However, in 27.5% of the children, 22 salt-wasting crises (seven of these also with low blood glucose) and 16 hypoglycemic episodes without salt loss were reported. Furthermore, the cumulative incidence for seizures was elevated; 13 children experienced seizures during hyponatremia or hypoglycemia. Most adrenal crises were triggered by infections, often with inappropriate emergency management, but in 11 cases hypoglycemia occurred unexpectedly, without evidence of severe illness and without any management errors. Frequency of adrenal crises was 6.5 per 100 patient years (95% CI: 4.6-8.8). Conclusions: Crisis prevention remains a permanent challenge for families and physicians caring for children with classic CAH. Expert care and compliance with emergency recommendations are crucial. Further research on the interactions among glucocorticoid deficiency, adrenomedullary dysfunction, and glucose metabolism is necessary for the prevention of hypoglycemia, especially in young CAH patients.

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Section: Discussionmentioning

confidence: 99%

Children with classic congenital adrenal hyperplasia experience salt loss and hypoglycemia: evaluation of adrenal crises during the first 6 years of life

Odenwald

Nennstiel-Ratzel

Dörr

et al. 2016

European Journal of Endocrinology

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“…Hypoglycaemia is a frequent finding in all cortisol deficiency states [1,5,7,8,10,14], suggesting the same pathogenesis of the hypoglycaemia. It is interesting to note that the associated hypoalaninaemia and low catecholamine secretion in our patient (which has not been described previously in isolated glucocorticoid deficiency), have been previously reported in isolated ACTH deficiency [1].…”

Section: Discussionmentioning

confidence: 97%

Isolated glucocorticoid deficiency: metabolic and endocrine studies in a 5-year-old boy

et al. 1985

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A 5-year-old boy is described who presented with episodes of hypoglycaemia triggered by mild infections or fever. Subnormal glucocorticoid production was confirmed by demonstrating low urinary excretion of free cortisol, low plasma cortisol concentrations that did not rise after glucagon and ACTH stimulation, and by elevated plasma ACTH levels. The selective nature of the abnormality was confirmed by demonstrating normal plasma electrolyte concentrations and blood pressure on a salt-restricted diet. Plasma renin activity and plasma aldosterone levels were also normal and responded appropriately to salt restriction and to frusemide-induced diuresis. Starvation-induced hypoglycaemia was associated with raised levels of blood ketone bodies and low blood alanine concentrations. Catecholamine secretion during hypoglycaemia was reduced. Glucocorticoid replacement therapy was effective in restoring normal glucose homeostasis.

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“…There are several reports of hypoglycemia in children with CAH associated with acute illness episodes (Mackinnon & Grant, 1977). Pinto (Pinto et al, 2003) reported the follow-up of 68 children with classic CAH, and noted that hypoglycemia occurred 9 times in children between the ages of 1 to 3 years, and resulted in two deaths.…”

Section: Discussionmentioning

confidence: 99%

Hypoglycemia During Acute Illness in Children With Classic Congenital Adrenal Hyperplasia

Keil

Bosmans

Ryzin

et al. 2010

Journal of Pediatric Nursing

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Congenital adrenal hyperplasia (CAH) describes a group of genetic, autosomal recessive conditions, where there is a block in cortisol biosynthesis. Approximately 95 percent of cases are due to 21-hydroxylase deficiency, which is discussed in this article. Patients with the severe or classic form of CAH have epinephrine deficiency in addition to cortisol deficiency. Both epinephrine and cortisol are important counterregulatory hormones and help prevent hypoglycemia during physical stress. This is the first prospective study to evaluate the incidence of hypoglycemia during acute illness in children with classic CAH. Our objective was to examine blood glucose levels and symptoms of these children during the physical stressor of a typical acute illness managed at home. Twenty patients, ages 3 to 10 years with classic CAH participated. Parents were instructed regarding management of illnesses, home blood glucose monitoring and questionnaire completion. Over 29 months, 20 patients completed questionnaires and 6 patients performed home blood glucose monitoring. A blood glucose of <60mg/dL was documented in 3 out of 8 monitored acute illness episodes, and in 2 out of 6 of monitored children. The acute illness episodes with documented blood glucose <60mg/dL were not associated with vomiting. Our data suggest that children with classic CAH may experience lowering of blood glucose during illnesses, and patient education regarding the management of common childhood illness should include glucose supplementation.

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Hypoglycaemia in congenital adrenal hyperplasia.

Cited by 16 publications

References 3 publications

Children with classic congenital adrenal hyperplasia experience salt loss and hypoglycemia: evaluation of adrenal crises during the first 6 years of life

Children with classic congenital adrenal hyperplasia experience salt loss and hypoglycemia: evaluation of adrenal crises during the first 6 years of life

Isolated glucocorticoid deficiency: metabolic and endocrine studies in a 5-year-old boy

Hypoglycemia During Acute Illness in Children With Classic Congenital Adrenal Hyperplasia

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