[Clinical and etiological features of primary adrenal insufficiencies in children].
BACKGROUND
The chronic primary adrenal insufficiency or Addison's disease is uncommon in children and belongs generally to a complex syndrome.


OBJECTIVE
Study of the clinical and aetiological features of primary adrenal insufficiencies in children.


METHODS
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.


RESULTS
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.


CONCLUSIONS
Larger studies about Addison's disease are needed to confirm the preponderance of the Allgrove syndrome.
Matoussi
N
N
Service Pédiatrie, Urgences et Consultations Externes, Hôpital d'enfants de Tunis, Bab Saadoun, Tunisie.
Amdouni
N
N
Fitouri
Z
Z
Makni
S
S
Cheour
M
M
Ben Becher
Sayda
S
D000224:Addison Disease / Q000175:diagnosis* / Q000209:etiology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies