Triple A syndrome with ophthalmic manifestations in two siblings.
Indian J Ophthalmol
;
2007 Jul-Aug; 55(4): 304-6
Artículo
en Inglés
| IMSEAR
| ID: sea-70979
ABSTRACT
Triple A syndrome (Allgrove syndrome) is a rare, autosomal recessive disorder characterized by adrenocorticotropic hormone resistant adrenal insufficiency, alacrima, achalasia of the esophageal cardia, progressive neurological degeneration and occasionally autonomic instability. We report the ophthalmic manifestations in 2 siblings from a consanguineous family with this syndrome. A routine ophthalmic examination showed absence of palpebral portion of lacrimal gland, dry eyes and sluggish pupillary reflexes in both eyes of these patients. Both of these patients had achalasia cardia while the boy additionally had increased serum cortisol levels. Topical ocular lubricants were prescribed for both of them and Heller's cardiomyotomy with fundus plication was done for achalasia. These cases would create an awareness of this rare condition in ophthalmology in addition to suggesting its management.
Texto completo:
Disponible
Índice:
IMSEAR (Asia Sudoriental)
Asunto principal:
Femenino
/
Humanos
/
Masculino
/
Enfermedad de Addison
/
Radiografía Torácica
/
Síndromes de Ojo Seco
/
Acalasia del Esófago
/
Niño
/
Hermanos
/
Diagnóstico Diferencial
Tipo de estudio:
Estudio diagnóstico
Idioma:
Inglés
Revista:
Indian J Ophthalmol
Año:
2007
Tipo del documento:
Artículo
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