AI is a rare disease that requires replacement treatment to restore well-being and to prevent life-threatening events.
AC occurs with an acute deterioration in health status associated with acute hemodynamic imbalance, with hypotension or sinus tachycardia, or marked metabolic disorders, i.e. hyponatremia, hyperkalemia, or hypoglycemia. It represents the most severe complication of AI, which can occur in several stressful situations; its susceptibility varies among patients and unknown risk factors may potentiate the risk of occurrence (16).
The education of the pediatric patient and his family, as well as all the personnel working in the PED is essential to prevent and promptly treat this acute complication at home and upon arrival at the hospital during sick events. Previous findings suggest that physicians are the primary source for obtaining information about the disease and its complications. However, evidence show the need to improve their performances and awareness (16-18).
The incidence of AC in our cohort (3.2 episodes/100 patient-year) was in accordance with literature data. (13,14,16). Admission rates were higher among pre-school patients, as reported by previous studies, which may be explained by the frequent infections in this age group. Literature data describe a second peak of accesses to the ED in adolescents, attributable to the transition period from paediatric to adult services, to greater patient autonomy and the reduced adherence to therapy, exposing them to a greater risk of complications (17-19). The same period of life is often characterized by the need for a therapy adjustment for the pubertal status.
To raise awareness of AC among healthcare professionals, consensus guidelines for patients with AI recommend that patients wear or carry medical alert devices, such as wallet cards, even though currently these alert devices are rarely used. In the present study, the patient informed the triage about the AI condition in 93.5% of accesses to the PED. Future use of a critical information note (CIN), as reported previously (15,17), could provide details on the signs and symptoms to consider and recommendations to follow for the most appropriate management of AI patients in triage settings.
The patients described presented to the Hospital primarily for infectious diseases, mostly gastroenteritis, with no evidence of hemodynamic compromise. Of these, approximately half patients (44%) were later discharged after clinical evaluation, feeling well, without receiving additional steroid therapy during PED access, as most of them had doubled or tripled the habitual steroid dose at home prior to PED access. Literature data confirm that this could be the first approach to a patient with AI during stressful situations (11,15).
In the cohort studied, a lower usual hydrocortisone replacement dose was observed in patients who developed AC; furthermore, home steroid therapy was not increased in 75% of cases. Based on epidemiological data, it has in fact been suggested that the increase in AC rates may be due to the current use of lower doses of glucocorticoids in patients with hypoadrenalism (5,14,18,20).
Previous data report that children with PAI are at higher risk for AC than subjects affected by secondary forms (13,14,18-20), although in our cohort this difference was not significant, probably due to the low number of AC.
Eyal et al. suggested that younger age at diagnosis may be a risk factor for AC, with a higher incidence of AC in the first year of life (13). In our cohort, age did not significantly influence the presentation of AC and there was no difference in the mean age at ED admission between primary and secondary forms.
The most common biochemical feature of AC was hyponatremia, occurring in 53.8% of the subjects, with lower mean sodium value in primary than secondary forms (p= 0.05), followed by hypoglycemia (38.5%), in accordance to previous studies on children and adults, especially on patients with PAI (10,11,18-20).
Treatment of AC is effective when given promptly. In the PED setting, steroid administration was faster in CAI than PAI patients, probably for more severe and acute presentation and the recognized underlying disease in the latter form. Findings from a previously published survey result from AI adult patients in the United Kingdom reported a time limit of 30 min from ED arrival to hydrocortison administration. Additional interventions, such as triage protocols for patients with AI, might be useful to expedite hydrocortison administration after clinical evaluation. (15,18) The importance of paying greater attention to the triage code is further supported by the fact that also in the present cohort this represented the major risk factor for the prediction of an AC.
Other risk factors for the development of AC were dehydration at presentation and the failure to take or increase habitual steroid therapy at home.
Appropriate management requires prompt recognition of the clinical signs, symptoms and biochemical profile of AI and AC triggers. (21)
To our knowledge, this is the first study that reports the clinical features of AI patients accessing a PED and evaluates risk factors for AC. The strengths of the study are that the information is based on the patients’ medical records and not on their self-reports and that the cohort includes patients with both primary and secondary AI.
Limitations are the retrospective nature of the study, with potential under- or over-reporting of AC. Certainly, further prospective and multicenter studies are needed to analyze the accesses of patients with AI in the PED, to establish guidelines for the management of these patients with potentially life-threatening events in emergency settings.