Acute illness in children with secondary adrenal insufficiency

Abstract

Secondary adrenal insufficiency (SAI) is a rare disorder in childhood, which comprises approximately 15%-40% of the total paediatric population with adrenal insufficiency (AI) and is a cause of considerable morbidity and occasional mortality. [1][2][3][4] Aetiologies include congenital malformations, such as septo-optic dysplasia and pituitary aplasia/hypoplasia; cerebral tumours and their treatment; cranial irradiation and trauma. 3,4 Glucocorticoid-induced AI resulting from therapeutic glucocorticoid exposure for treatment of a range of autoimmune, inflammatory and some malignant conditions, can also be included in the SAI category. 3 SAI may be associated with other pituitary hormone deficits, including arginine vasopressin deficiency, which causes diabetes insipidus (DI).

Adrenal crises in adolescents and young adults

“…In contrast, a study of adult patients with AI from Japan identified only 4 ACs in this age group, all of whom had SAI [ 26 ]. As ACs are slightly more common in primary than secondary AI, this result is somewhat unexpected, but may be related to the presence of comorbid diabetes insipidus (DI), which is associated with an increased AC risk [ 31 , 32 ].…”

“…Other factors, such as the presence of comorbid illnesses, are less related to a specific life stage but are nevertheless associated with increased AC risk. Among these are comorbid conditions such as diabetes insipidus (DI), which is relatively common among patients who are survivors of cerebral tumours and some congenital forms of AI [ 31 , 32 ]. Similarly, patients with comorbid diabetes mellitus (DM) may be at increased AC risk, possibly due to the complexities of managing an intercurrent illness in the presence of both disorders, although the evidence supporting this is variable [ 47 – 49 ].…”

“…Continual educational reinforcement about the importance of adherence to recommended replacement schedules and the appropriate use of both oral and parenteral stress dosing is advised for all patients [ 1 , 8 , 13 , 60 ]. However, education alone does not appear to be sufficient for many patients and evidence suggests that direct experience of an episode of acute AI/AC improves competency, especially with regards to use of stress doses [ 13 , 24 , 27 , 28 , 32 ].

Fig.

…”

Una visión general sobre la crisis suprarrenal en el paciente pediátrico

“…Sin embargo, en el caso específico de la IS secundaria, la diabetes insípida se ha identificado como una de las principales comorbilidades para desarrollar CS (10). Una cohorte retrospectiva de 47 pacientes con IS secundaria, donde se examinaron los registros de todas las atenciones por enfermedad aguda en dos servicios de emergencias pediátricas en Australia, identificó que los pacientes con DI tenían más probabilidades de ser ingresados, estancia hospitalaria más prolongada y tasas más altas de administración de hidrocortisona IV (12).…”

Emergency card, emergency medication, and information leaflet for the prevention and treatment of adrenal crisis (Addison crisis): an Austrian consensus document

“…Eine Nebennierenkrise im Erwachsenenalter tritt in etwa bei 5-10 (in einer Studie 24) Patient*innen mit NNI innerhalb von 100 Patient*innenjahren auf und hat vermutlich eine Mortalitätsrate (Sterblichkeitsrate) von etwa 0,5 pro 100 Patient*innenjahren [5,[10][11][12][13][14]. Für das Kindesalter wurden ähnliche Zahlen publiziert [15][16][17][18], wobei rezente Studien bei Kindern marginal niedrigere Inzidenzen mit etwa 3-4 Nebennierenkrisen pro 100 Patient*innenjahren berichtet haben [19][20][21][22]. Patient*innen mit einer NNI haben langfristig ein etwas erhöhtes Mortalitätsrisiko, wobei in einer rezenten Studie ungefähr jeder 10.…”