People with Adrenal Insufficiency who are in Adrenal Crisis are Frequently Unable to Self-Administer Rescue Injections

Endocrine Practice, Journal Year: 2025, Volume and Issue: unknown

Published: March 1, 2025

Individuals with adrenal insufficiency (AI) are at risk of acute crisis and death, particularly during illness or trauma, may require rapid treatment parenteral glucocorticoid such as hydrocortisone (HC) to manage a crisis. Current guidelines recommend timely self-injection in an evolving Little is known about the patient experience emergency injections. We surveyed people regarding success In 2022 survey was conducted through National Adrenal Diseases Foundation website individuals AI their caregivers managing They reported on events that required cortisol injection failure injection, context event reasons for failure, RESULTS: Nearly half (41%) insufficient patients were unable self-administer citing effects crisis-associated confusion major barriers. Failed injections led bad outcomes (sicker, need hospitalization, death) 36% cases. Effective, timely, management impending can prevent progression multisystem requiring ICU care, death. Reliance solely result worse outcomes. Treating physicians should include education specific practical instruction technique, well potential assistance FDA approval auto injector, greater engagement EMS clinicians, hospital staff, other healthcare professionals, key future crises.

Language: Английский

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Long-Term Outcomes of Congenital Adrenal Hyperplasia

Endocrinology and Metabolism, Journal Year: 2022, Volume and Issue: 37(4), P. 587 - 598

Published: July 8, 2022

A plethora of negative long-term outcomes have been associated with congenital adrenal hyperplasia (CAH). The causes are multiple and involve supra-physiological gluco- mineralocorticoid replacement, excess androgens both intrauterine postnatal, elevated steroid precursor adrenocorticotropic hormone levels, living a condition as well the proximity cytochrome P450 family 21 subfamily member 2 (<i>CYP21A2</i>) gene to other genes. This review aims discuss different CAH.

Language: Английский

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Transition Readiness in Adolescents and Young Adults Living With Congenital Adrenal Hyperplasia

Endocrine Practice, Journal Year: 2023, Volume and Issue: 29(4), P. 266 - 271

Published: Jan. 21, 2023

Language: Английский

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Approach to the Patient: Preventing Adrenal Crisis Through Patient and Clinician Education

The Journal of Clinical Endocrinology & Metabolism, Journal Year: 2023, Volume and Issue: 108(7), P. 1797 - 1805

Published: Jan. 11, 2023

Abstract Adrenal crisis continues to be an important cause of death despite being a preventable condition. Significant deficits in patient education the prevention adrenal have been identified as contributor risk, importance highlighted international guidelines. Deficits clinician also risk factors for although they not addressed. We use 3 clinical cases highlight role both and crisis, review what is known about related insufficiency provide possible approach addressing with aim reducing through education.

Language: Английский

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Transition from Paediatric to Adult Care in CAH: 20 Years of Experience at a Tertiary Referral Center

Hormone and Metabolic Research, Journal Year: 2024, Volume and Issue: 56(01), P. 45 - 50

Published: Jan. 1, 2024

Abstract Transition medicine aims at the coordinated transfer of young patients with a chronic disease from paediatric to adult care. The present study reflects 20 years experience in transitioning congenital adrenal hyperplasia (CAH) single center setting. Our endocrine transition-clinic was established 2002 and offers joint consultations. Data were evaluated retrospectively 2005 2008 present. Fifty-nine (29 males) transferred. Median age 18.4 (17.6–23.6). Ninety percent presented 21-hydroxlase-deficiency (21-OHD), 38 (23 m) salt-wasting (sw), 7 (1 simple-virilising (sv) 8 (3 non-classic (nc) form. Rarer enzyme deficiencies found 6 cases: 17α-OHD (2 sisters), P450-oxidoreductase-deficiency siblings), 3β-hydroxysteroid-dehydrogenase-deficiency 11β-OHD female). Thirty-four (57.6%, are presently still attending clinic, 1 patient (1.7%, moved away 24 (40.7%, lost follow-up (13 sw-21-OHD, sv-21-OHD, 5 nc-21-OHD). Thirty-seven (62.7%) attended clinic for >2 after transfer, 17 (28.8%) >10 years. In group, median time attendance 16.3 months (0–195.2). Defining successful as two or more visits department initial consultation transition efficient 84.7% cases. A seamless care is essential adolescents CAH. It requires continuous support during period, remains challenging, necessitates adequate funding.

Language: Английский

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Circulatory shock in adults in emergency department

Turkish Journal of Emergency Medicine, Journal Year: 2023, Volume and Issue: 23(3), P. 139 - 139

Published: Jan. 1, 2023

Language: Английский

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Circulatory shock in adults in emergency department

Turkish Journal of Emergency Medicine, Journal Year: 2023, Volume and Issue: 23(3), P. 139 - 148

Published: Jan. 9, 2023

Circulatory shock is a common condition that carries high morbidity and mortality. This review aims to update the critical steps in managing types of adult patients admitted medical emergency intensive care units. A literature was performed by searching PubMed, EMBASE Ovid, Cochrane Library, using following search items: (“shock” OR “circulatory shock” “septic “cardiogenic shock”) AND (“management” “treatment” “resuscitation”). The emphasizes prompt identification with tissue hypoperfusion, knowledge underlying pathophysiological mechanism, initial fluid resuscitation balanced crystalloids, norepinephrine as preferred vasopressor septic profound cardiogenic shock, tailored intervention addressing specific etiologies. Point-of-care ultrasound may help evaluate an undifferentiated determine responsiveness. approach improving; however, confirmatory studies are required for many existing (e.g., amount fluids steroids) emerging angiotensin II) therapies. Knowledge gaps wide variations persist needs urgent improve outcomes.

Language: Английский

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The multiple faces of autoimmune Addison’s disease in children

Frontiers in Endocrinology, Journal Year: 2024, Volume and Issue: 15

Published: Sept. 16, 2024

Primary adrenal insufficiency (PAI) is a rare medical condition, characterized by deficiency in hormones. Although rare, PAI life-threatening disease requiring prompt recognition and treatment. However, symptoms of are often non-specific diagnosis can be challenging, causing frequent diagnostic delays. In adults, autoimmunity the most common cause industrialized countries, whereas children, etiology represented congenital defects steroidogenesis and, particular, hyperplasia (CAH) due to 21-hydroxylase deficiency. A few recent case series from different countries have reported that second pediatric age group. data on autoimmune children still scant exact epidemiology, clinical manifestations, long-term outcomes this condition yet defined. The scope review summarize current knowledge etiology, presentation, treatment childhood increase physicians’ awareness signs should raise an early suspicion condition.

Language: Английский

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Trends in hospital admissions for adrenal insufficiency in adolescents and young adults in the 21st century

Frontiers in Endocrinology, Journal Year: 2022, Volume and Issue: 13

Published: Sept. 20, 2022

Very little is known about the epidemiology of adrenal crises (AC) and insufficiency (AI) in adolescents young adults.Data on all admissions to Australian hospitals between 2000/1 2019/20 for a principal diagnosis AI (including AC) 10-24 year olds were extracted from national repository. Age sex-specific rates age-adjusted compared.Over study, there 3386 AI; 24.0% (n=812) an AC 50·7% (n=1718) secondary AI. Age-adjusted increased 31·70/million 54·68/million (p<0·0001). also increased, most notably second decade (from 5·80/million 2010/11 15·75/million 2019/20) (p<0·00001). Average admission comparable sexes, but significantly females, especially those aged 20 24 years, whose rate (39·65/million) was higher than corresponding (3·15/million) SAI males (23·92/million) females (15·47/million) However, only among 11·81/million 22·12/million 2019/20), with increase 20-24 old 5·07/million 2010 20·42/million adjusted congenital hyperplasia, primary (PAI) drug-induced did not change over study.AC/AI first two decades this century emerging adult population, particularly who experienced marked rates, evident decade. Although uncertain, possible explanations include: dose glucocorticoid replacement; non-adherence therapy; psychosocial factors; difficulty transition services. Admissions while PAI CAH remained constant.

Language: Английский

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Transplantation of porcine adrenal spheroids for the treatment of adrenal insufficiency

Xenotransplantation, Journal Year: 2023, Volume and Issue: 30(5)

Published: Aug. 7, 2023

Abstract Primary adrenal insufficiency is a life‐threatening disorder, which requires lifelong hormone replacement therapy. Transplantation of xenogeneic cells potential alternative approach for the treatment insufficiency. For successful outcome this therapy, transplanted should provide adequate secretion and respond to physiological stimuli. Here, we describe generation characterization primary porcine spheroids capable replacing function glands in vivo. Cells within morphologically resembled adult adrenocortical synthesized secreted steroid hormones regulated manner. Moreover, embedding alginate led formation cellular elongations steroidogenic migrating centripetally towards inner part slab, similar zona Fasciculata intact organ. Finally, transplantation adrenalectomized SCID mice reversed phenotype, significantly improved animals’ survival. Overall, such models could be employed disease modeling drug testing, represent first step toward clinical trials future.

Language: Английский

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