OBJECTIVE/DESIGN: This study aimed to examine and describe three siblings with @entity20718 , the eldest of whom had associated @entity1318 and @entity5343 . @entity1 : Three affected siblings and four age-matched control subjects participated. INTERVENTION/MAIN OUTCOME MEASURES: The three @entity1 underwent complete ophthalmologic examinations; computed tomographic scanning of brain, orbit, chest, and abdomen; and measurement of serum @entity701 . All three were subjected to a short synacthen challenge. Lacrimal gland biopsies were performed on the two younger subjects, and specimens were studied by light and electron microscopy. RESULTS: All three @entity1 showed virtually absent tear secretion as tested by the Schirmer test. The resulting @entity2372 was most severe in the oldest @entity1 , who developed bilateral corneal melting. The two younger @entity1 showed interpalpebral corneal staining with rose bengal. All three @entity1 improved after @entity432 . @entity5343 was present in the oldest @entity1 . Computed tomographic scanning showed absent lacrimal and shrunken adrenal glands in association with @entity1318 of the @entity418 in the oldest @entity1 . The lacrimal glands were found to be reduced in size in the next eldest @entity1 . When evaluated by electron microscopy, the lacrimal gland biopsy specimens from the two younger @entity1 showed @entity528 associated with depletion of secretory granules in the acinar cells. CONCLUSION: In this disease, radiologic evidence of reducing lacrimal gland size with increasing age could represent a degenerative process. This may be paralleled by other signs and the possibility of @entity5343 and @entity1318 of the @entity418 should be investigated in all @entity1 presenting with @entity936 . These @entity1 appear to have a progressive @entity528 .

Keratoconjunctivitis sicca associated with @entity1318 of the @entity418 , @entity5343 , and lacrimal XXXX : Keratoconjunctivitis sicca secondary to lacrimal @entity528 may parallel degenerative changes in esophageal and adrenocortical function.

@entity1318 :: ('MESH:D004931', 'Disease') :: ['achalasia'] @entity1 :: ('9606', 'Species') :: ['children', 'PARTICIPANTS', 'child'] @entity2372 :: ('MESH:C562399', 'Disease') :: ['keratopathy'] @entity5343 :: ('MESH:D000224', 'Disease') :: ['adrenocortical insufficiency', "Addison's disease"] @entity20718 :: ('MESH:C562827', 'Disease') :: ['alacrima'] @entity701 :: ('MESH:D006854', 'Chemical') :: ['cortisol'] @entity418 :: ('MESH:D004938', 'Disease') :: ['cardia'] @entity936 :: ('MESH:D015352', 'Disease') :: ['dry eyes'] @entity432 :: ('MESH:D001157', 'Disease') :: ['punctal occlusion'] @entity528 :: ('MESH:D009410', 'Disease') :: ['neuronal disease', 'neuronal degeneration']
@entity528 :: (MESH:D009410,Disease) :: ['gland degeneration']
