Acute illness in children with secondary adrenal insufficiency.

OBJECTIVE AND BACKGROUND: Secondary adrenal insufficiency (SAI) is a rare condition in childhood which can be associated with high levels of morbidity in some patients. The causes of increased levels of illness are not well defined and warrant further investigation. METHODS: A retrospective cohort of patients with SAI was constructed by examining records of all attendances for acute illness by SAI patients at the emergency department of the two specialist paediatric hospitals in Sydney, Australia between 2004 and 2016. Demographic, clinical, and physiological characteristics together with pre-hospital illness management strategies were assessed. RESULTS: There were 168 presentations for an acute illness by 47 children with SAI. Comorbid diabetes insipidus (DI) was present in 46.8% (n = 22), 77.3% (n = 17) of whom were male (P < .05). Patients with comorbid DI were more likely to be admitted (86.7%, n = 65 vs 60.2%, n = 56 for non-DI, P < .01); had a longer hospital stay (6.5 (8.7) vs 2.5 (2.6) days, P < .001); and higher rates of IV HC administration (56.0%, n = 42 vs 35.5%, n = 33), P < .01). The medically-diagnosed adrenal crisis (AC) rate was 3.68 ACs/100PY. Stress dose use was reported by fewer DI patients (58.7%, n = 44) than non-DI patients (78.5%, n = 73, P < .01). Previous attendance at hospital was positively associated with stress dose use (OR = 1.08, 95% CI 1.00, 1.16). CONCLUSION: Secondary adrenal insufficiency can cause significant morbidity in children. Comorbid DI is associated with higher levels of hospitalisation, longer hospital stays and lower levels of pre-emergent stress dose use. Educational interventions in this subgroup of SAI patients may reduce the burden of morbidity.

Acute illness in patients with concomitant Addison's disease and type 1 diabetes mellitus: Increased incidence of hypoglycaemia and adrenal crises.

BACKGROUND: Patients with Addison's disease (AD) and comorbid type 1 diabetes mellitus (T1DM) are at increased risk of certain acute metabolic disorders relative to patients with one of these conditions only. The reasons for this are unknown. METHODS: All attendances for acute illness by AD patients at the emergency department of a Sydney hospital between 2000 and 2017 were reviewed. Physiological parameters and illness management strategies were compared between AD patients, those with T1DM and AD combined, and a control group of patients with T1DM. RESULTS: There were 39 presentations for an acute medical illness by 20 nondiabetic AD (28 attendances) and 5 diabetic AD patients (11 presentations) and 40 attendances by 10 T1DM controls. In AD patients, 17 (43.6%) attendances were medically diagnosed adrenal crises (AC) (63.6% [n = 7] in diabetic AD and 35.7% [n = 10] in nondiabetic AD). This corresponded to an estimated incidence of 12.5 AC/100 patient-years (PY) for diabetic AD patients compared to 4.7 AC/100PY for nondiabetic AD patients (P < .05). Glucocorticoid stress doses preceded 61.5% (n = 24) of all attendances. Patients who used stress doses had more presentations than those who did not (2.0 ± 1.3 vs 1.2 ± 0.5, P = .01). Diabetic AD patients had a lower mean blood glucose level on presentation (5.6 ± 3.9 mmol/L) than the T1DM control sample (11.6 ± 6.2 mmol/L) P < .001. No T1DM patients had hypoglycaemia in the 3.0-3.9 mmol/L range but 2 (18.2%) of the diabetic AD patients had presenting blood glucose levels in this category (P < .05). Hyperglycaemia was more common among T1DM control patients (62.5%, n = 26) than diabetic AD patients (18.2%, n = 2), P < .01. CONCLUSION: Addison's disease patients with T1DM have a higher incidence of adrenal crisis (AC) and hypoglycaemia than nondiabetic AD patients and a lower incidence of hyperglycaemia than those with T1DM alone. This information may be of value in counselling patients with T1DM and AD about AC and hypoglycaemia prevention.

The use of medical identification jewellery in adults with adrenal insufficiency in Australia.

OBJECTIVE: Hydrocortisone stress dosing during illness can prevent adrenal crises (AC) in patients with adrenal insufficiency (AI). When patients cannot communicate, medical identification jewellery may facilitate parenteral hydrocortisone provision but patient adoption rates are not known. DESIGN: A cross-sectional analysis of Australian medical identification jewellery subscription data. PATIENTS: Patients with AI aged 20 years and over with an active subscription to a large medical jewellery provider. MEASUREMENTS: Subscription rates by AI subtype, geographic area, age and gender. RESULTS: There were 1955 patients with AI and an active subscription in the database, corresponding to a subscription rate of 105.79/million or approximately one-third of the AI population. The subscription rate was substantially higher in primary AI (60.72/million) than secondary AI (23.16/million), corresponding to approximately 60.7% and 11.6% of the estimated population prevalence of each disorder, respectively. There was substantial variation in use by state/territory, with the highest subscribing state having a rate of over four times that of the lowest (P < 0.001). Women comprised 64.8% (n = 1266) of the group. Subscription also varied by age, being highest in the 60-69 year age group (165.15/million) and lowest in those aged 30-39 years (47.23/million) (P < 0.001). Few patients (4.8%, n = 94) mentioned, either in their record or on their jewellery, the need for urgent parenteral hydrocortisone in the event of severe illness. CONCLUSIONS: Medical jewellery is a component of AC risk reduction. However, subscription appears to be underutilised in the Australian AI population, especially among patients with secondary AI. Urgent treatment recommendations should be inscribed on the jewellery.

An analysis of the utilisation of medical identification jewellery among children and young adults with type 1 diabetes mellitus in Australia.

AIMS: People with type 1 diabetes mellitus (T1DM) are at risk of life-threatening illness. Medical jewellery is recommended for emergencies, but its uptake is unknown. This study assessed the use of medical jewellery among people with T1DM aged 0-24 years in Australia. METHODS: A cross sectional analysis of subscription data to the largest medical identification jewellery service in Australia was analysed by age, sex and geographic location using Australian population data from 2018. RESULTS: There were 1599 people with T1DM aged 0-24 in the database, but only 1061 had an active subscription, corresponding to an active subscription rate of 13.28/100,000 population or ~5% of the estimated patient population. Half of the active subscribers were male (543/1061, 51/3%). The average age of active subscribers was 17; very few (n = 12, 1.1%) were aged less than 5; and the highest number (n = 141, 39%) was in the 20-24 age group. Active subscription rates varied significantly by geographic location. 88.4% of active subscribers had a diagnosis of T1DM or equivalent inscribed on their emblem, while engraved instructions for management in an emergency were only included in 1.8% of records (n = 19). CONCLUSIONS: Medical jewellery subscription rates were lower than expected; increased with age; and varied significantly by state/territory. The use of medical identification jewellery may be limited by the lack of suitable engraved instructions for use in an emergency. Factors leading to low use should be addressed. Photo 1 Medical Jewellery with emblem.

Factors underlying a disproportionate increase in hospital admissions for adrenal insufficiency in women aged 20-29 years.

Since the year 2000, admissions for adrenal insufficiency (AI) and adrenal crises (AC) have shown a particular increase in young adult females. We examined data on acute non-surgical hospitalisations for AI/AC from New South Wales, Australia, to determine relevant factors that may have contributed to this increase. Data were analysed to ascertain associations between various comorbid psychosocial issues, identified by relevant ICD-10-AM codes in each record, and ACs. From 2005 to 2021. There were 877 admissions for an acute non-surgical illness in this age group. The average admission rate for females [63.5/million/year] was almost twice that for males [34.0/million/year] (p<0.01), as was the average female AC admission rate [14.7/million/year] relative to that in males [6.75/million/year] (p=NS). Infection was present in 41.6% (n=365) of the admissions and Type 1 diabetes mellitus was present in 12.2% (n=107). Overall, psychosocial factors were considered by the senior clinician to have contributed to the illness episode in 22.1% of all admissions and 29.0% of AC admissions. Having one or more psychosocial problems was associated with an AC in females (37.4%, n=49, in those having an AC, p<0.001) but not males. Females with an AC also had a higher mean composite psychosocial, psychiatric and drug/alcohol score [0.47 (0.67)] than females without an AC [0.32 (0.62) p<0.05]. No comparable associations were found in male patients. An increase in the rates of hospitalisations that included a code for at least one psychosocial problem was highly correlated with increases in admission rates for both ACs (r=0.82, p<0.001) and all AI (r=0.98, p<0.001) in females but there was no such relationship in males. This new evidence suggests that psychosocial factors may play an important role in ongoing rates of ACs in treated AI (incidence approximately 6-8 ACs/100PY) particularly in young adult females. In order to minimize AC episodes, all barriers to self-management need exploration on an individual patient basis and with regard to the patient population as a whole.