☆ 4.5 Review

Emergency and perioperative management of adrenal insufficiency in children and young people: British Society for Paediatric Endocrinology and Diabetes consensus guidance

ARCHIVES OF DISEASE IN CHILDHOOD (2023)

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Congenital Adrenal Hyperplasia-Current Insights in Pathophysiology, Diagnostics, and Management

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Summary: Congenital adrenal hyperplasia is a group of autosomal recessive disorders affecting cortisol biosynthesis, with the most common form caused by steroid 21-hydroxylase deficiency due to mutations in CYP21A2. Recent developments include advancements in understanding steroidogenic pathways, improvements in diagnostic measurements, and exploration of alternative medications and treatments for CAH patients.

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Pediatric Adrenal Insufficiency: Challenges and Solutions

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Summary: Adrenal insufficiency can be initially misdiagnosed as other life-threatening conditions and is particularly challenging in pediatric patients. It requires dynamic testing for diagnosis and long-term glucocorticoid replacement therapy for treatment.

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Fifteen-minute consultation: Emergency management of children presenting with hyperkalaemia

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Management of Acute Adrenal Insufficiency-Related Adverse Events in Children with Congenital Adrenal Hyperplasia: Results of an International Survey of Specialist Centres

Salma Rashid Ali et al.

Summary: While there is variation in the definition and management of AI-related adverse events in children among centres, there is a good level of consensus on specific aspects that can lead to greater benchmarking of care. Over 90% of centres in high-income countries provided steroid management plans, one-to-one patient education, and contact details of health care staff. Additionally, the majority of centres recommended glucocorticoid stress dosing in the event of fever, and vomiting was the most common indication for intramuscular hydrocortisone injection.

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Cortisol Levels During Acute Illnesses in Children and Adolescents A Systematic Review

Mohammad Rezai et al.

Summary: This systematic review examined endogenous cortisol levels in children and adolescents during acute illnesses and found that these levels were higher than those in controls and varied across different acute diseases. This has important implications for optimizing glucocorticoid stress doses for children and adolescents with glucocorticoid deficiency.

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Review Medicine, General & Internal

Adrenal insufficiency

Eystein S. Husebye et al.

Summary: Adrenal insufficiency can be caused by various factors such as primary adrenal disorder, adrenocorticotropic hormone deficiency, or suppression of adrenocorticotropic hormone by certain medications. Clinical features include unintentional weight loss, anorexia, hypotension, fatigue, muscle and abdominal pain, and hyponatremia. Diagnosis is often delayed due to non-specific symptoms, and patient education on managing the condition is crucial to prevent adrenal crisis.

LANCET (2021)

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Acute Illness and Death in Children With Adrenal Insufficiency

Chris Worth et al.

Summary: The study found that parents of children experiencing AC usually follow 'sick day' guidance for adjusting oral hydrocortisone dosage, but rarely use intramuscular hydrocortisone for emergency treatment. Additionally, despite 79% of parents reporting confidence in using intramuscular hydrocortisone, they rarely utilize it in practice, indicating that current training programs are not sufficient to prevent the most serious crises. New strategies need to be developed to encourage the usage of parenteral hydrocortisone.

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Review Endocrinology & Metabolism

Therapy options for adrenal insufficiency and recommendations for the management of adrenal crisis

Hanna Nowotny et al.

Summary: Adrenal insufficiency is a life-threatening condition requiring lifelong glucocorticoid therapy, with a growing number of cases in Europe due to side effects of cancer treatments. Attention should be paid to avoid potential long-term adverse effects during treatment.

ENDOCRINE (2021)

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Guidelines for the management of glucocorticoids during the peri-operative period for patients with adrenal insufficiency Guidelines from the Association of Anaesthetists, the Royal College of Physicians and the Society for Endocrinology UK

T. Woodcock et al.

ANAESTHESIA (2020)

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Prevention of Adrenal Crisis: Cortisol Responses to Major Stress Compared to Stress Dose Hydrocortisone Delivery

Alessandro Prete et al.

JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM (2020)

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Guidance for the prevention and emergency management of adult patients with adrenal insufficiency

Helen Simpson et al.

CLINICAL MEDICINE (2020)

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Adrenal crises in children with adrenal insufficiency: epidemiology and risk factors

Ori Eyal et al.

EUROPEAN JOURNAL OF PEDIATRICS (2019)

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Adrenal Crisis

R. Louise Rushworth et al.

NEW ENGLAND JOURNAL OF MEDICINE (2019)

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Adrenal Crises in Children: Perspectives and Research Directions

R. Louise Rushworth et al.

HORMONE RESEARCH IN PAEDIATRICS (2018)

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Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline

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JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM (2016)

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Variation in absorption and half-life of hydrocortisone influence plasma cortisol concentrations

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Bruno Allolio

EUROPEAN JOURNAL OF ENDOCRINOLOGY (2015)

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Deconvolution analysis of 24-h serum cortisol profiles informs the amount and distribution of hydrocortisone replacement therapy

Catherine J. Peters et al.

CLINICAL ENDOCRINOLOGY (2013)

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Review Pediatrics

Adrenal insufficiency: Still a cause of morbidity and death in childhood

Dorothy I. Shulman et al.

PEDIATRICS (2007)

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