[An association of Addison's disease, achalasia of the cardia and alacrimation. Apropos of 2 cases].
We report two cases of a syndrome combining Addison disease, cardial achalasia, and alacrimation in two girls aged 4 and 5 1/2 years. Moreover, these patients exhibited some unusual features: both also had mineralocorticoid deficiency, and one had loss of tongue papillae and absence of the pupillary reflex. This syndrome is infrequent. Etiology is unknown. Pathophysiology is under debate; autonomic nervous system dysfunction seems likely. We have analyzed the features of this syndrome on the basis of our two observations and the 15 previously reported cases.
Hammami
A
A
Trabelsi
M
M
Bennaceur
B
B
Boukhris
R
R
D000224:Addison Disease / Q000150:complications*; D002675:Child, Preschool; D015352:Dry Eye Syndromes / Q000150:complications*; D004931:Esophageal Achalasia / Q000150:complications*; D005260:Female; D006801:Humans; D013577:Syndrome
Association d'une maladie d'Addison, d'une achalasie du cardia et d'une alacrymation. A propos de deux observations.