Clinical and etiological features of primary adrenal insufficiencies in children

The chronic primary adrenal insufficiency or Addison's disease is uncommon in children and belongs generally to a complex syndrome. Study of the clinical and aetiological features of primary adrenal insufficiencies in children. In a retrospective study, we reviewed clinical and diagnostic data of all cases of Addison's disease admitted within a period of 15 years (from january 1991 to December 2006), in a department of paediatrics. Cases due to congenital adrenal hyperplasia were excluded. 6 cases of Addison's disease were diagnosed. Five patients are the product of consanguineous marriage. The age at the diagnosis of adrenal insufficiency varried from 15 months to 9 years 8 months. The adrenal insufficiency was associated to Allgrove syndrome in three cases, to autoimmune polyendocrinopathy type 1 in one patient and to probable peroxisomal disease in another one. The etiological disease was not determined in one patient. A substitutive hormonal therapy was conducted in all patients. During a mean follow-up of 26 months, two adrenal crises were noted. Larger studies about Addison's disease are needed to confirm the preponderance of the Allgrove syndrome.