Triple A (Allgrove syndrome)in Children: Clinical study in Children Hospital in Damascus University.

Abstract

Triple-A-syndrome (Allgrove) is a familial form of adrenal insufficiency. It is characterized by classical clinical triad: alacrimia, achalasia and adrenal insufficiency.

Its prevalence may be higher than estimated because of missed diagnosis. The most cases of this autosomal recessive syndrome are associated with neurological degenerative features which seem to be major prognostic factor after correction of adrenal function and achalasia. Management and follow-up of Triple-A-Syndrome requires multidisciplinary center.

This research aims to study cases of Triple A syndrome diagnosed in children in terms of clinical findings and relationship to the severity of adrenal insufficiency and whether there is an improvement in growth or laboratory tests after applying pharmacological or surgical treatment. The absence of previous studies of this case in Syria constitutes a basis for the need for early diagnosis and management in a multidisciplinary center.

Results: the number of participants in the study was 28 (7 females and 21 males), the characteristic triad of the syndrome was present in most cases. The main age of patient was 6 years, and growth failure (44%) and hypoglycemia (55%) dominated the clinical manifestations. Neurological manifestations were seen in 60% of patient. There is no statistical significance between the symptoms and the severity of adrenal insufficiency, the level of ACTH was higher in the groups that underwent surgery, there were no statistical differences between the two treatment regimens by glucocorticoids (two or three dose regimens) on control and on growth variables.