Timelines in the management of adrenal crisis – targets, limits and reality

Hahner, Stefanie; Hemmelmann, N; Quinkler, Marcus; Beuschlein, Felix; Spinnler, Christina; Allolio, Bruno

doi:10.1111/cen.12609

Cited by 48 publications

(46 citation statements)

References 20 publications

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“…There were complaints about difficulties in convincing healthcare workers to observe the presented emergency documents. These findings support the need for optimal crisis prevention strategies (14,16), including education, training, empowerment and support of all parents and patients (9,26,29), clear emergency documents (7,30), easily accessible information for professionals (31), and the sensitization of healthcare workers (9,16,27,32). The high number of cases without parenteral application of a glucocorticoid in spite of repeated vomiting might be reduced if -instead of i.m.…”

Section: Discussionmentioning

confidence: 58%

“…The high number of cases without parenteral application of a glucocorticoid in spite of repeated vomiting might be reduced if -instead of i.m. injection -s.c. glucocorticoid injection or a 'hydrocortisone pen' could be established for emergency use in the future (8,32,33). As problems related to a lack of experience among health professionals are characteristic of rare diseases and not unique to CAH, they might be overcome by a generally raised awareness about these so-called 'orphan diseases' in the healthcare system (31).…”

Section: Discussionmentioning

confidence: 99%

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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: 58%

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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“…While 86% of patients were quickly attended to by a health care professional within 45 minutes of a distress call, only 54% received glucocorticoid administration within 30 minutes of arrival. 45 This may be because it is an uncommon condition with which health care professionals are often unfamiliar. The introduction of a pan-European Emergency card with clear instructions on the necessity of early treatment will hopefully empower allied health care providers and physicians to institute treatment.…”

Section: Emergency Helpmentioning

confidence: 99%

Adrenal Crisis: Still a Deadly Event in the 21st Century

Puar

Stikkelbroeck

Smans

et al. 2016

The American Journal of Medicine

143

140

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Adrenal crisis is a life-threatening medical emergency, associated with a high mortality unless it is appropriately recognized and early treatment is rendered. Despite it being a treatable condition for almost 70 years, failure of adequate preventive measures or delayed treatment has often led to unnecessary deaths. Gastrointestinal illness is the most common precipitant for an adrenal crisis. Although most patients are educated about "sick day rules," patients, and physicians too, are often reluctant to increase their glucocorticoid doses or switch to parenteral injections, and thereby fail to avert the rapid deterioration of the patients' condition. Therefore, more can be done to prevent an adrenal crisis, as well as to ensure that adequate acute medical care is instituted after a crisis has occurred. There is generally a paucity of studies on adrenal crisis. Hence, we will review the current literature, while also focusing on the incidence, presentation, treatment, prevention strategies, and latest recommendations in terms of steroid dosing in stress situations.

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“…If possible, the patient should take with them a kit with parenteral hydrocortisone for self-administration. 15,17,19 conclusion…”

Section: Prevention Of a New Addisonian Crisismentioning

confidence: 95%

“…As mentioned, the patient must carry some form of identification of their underlying condition. 15,17 3. The maintenance dose used in cases of mild stress (infections) should be doubled or tripled in periods of more intense stress (surgeries), and may return to the prior dose after 24 to 48 hours.…”

Section: Prevention Of a New Addisonian Crisismentioning

confidence: 99%