PMID: 9544638Apr 17, 1998Paper

Keratoconjunctivitis sicca associated with achalasia of the cardia, adrenocortical insufficiency, and lacrimal gland degeneration: Keratoconjunctivitis sicca secondary to lacrimal gland degeneration may parallel degenerative changes in esophageal and adrenocortical function

Ophthalmology
P B MullaneyJ M Risco

Abstract

This study aimed to examine and describe three siblings with alacrima, the eldest of whom had associated achalasia and adrenocortical insufficiency. Three affected siblings and four age-matched control subjects participated. INTERVENTION/MAIN OUTCOME MEASURES: The three children underwent complete ophthalmologic examinations; computed tomographic scanning of brain, orbit, chest, and abdomen; and measurement of serum cortisol. 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. All three children showed virtually absent tear secretion as tested by the Schirmer test. The resulting keratopathy was most severe in the oldest child, who developed bilateral corneal melting. The two younger children showed interpalpebral corneal staining with rose bengal. All three children improved after punctal occlusion. Addison's disease was present in the oldest child. Computed tomographic scanning showed absent lacrimal and shrunken adrenal glands in association with achalasia of the cardia in the oldest child. The lacrimal glands were found to be reduced in size in the next eldest child. When evaluated by electron...Continue Reading

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Citations

Jan 13, 2000·Diseases of the Esophagus : Official Journal of the International Society for Diseases of the Esophagus·S J WalkerN Birbeck
Jul 12, 2001·Clinical Dysmorphology·E TsilouM I Kaiser-Kupfer
Jul 17, 2004·Cornea·Sudesh K AryaSunandan Sood
Jan 1, 2010·BMJ Case Reports·Naveen ShettihalliKokila Lakhoo
Jun 26, 2004·BMC Ophthalmology·Brian P BrooksConstantine A Stratakis
May 22, 2013·Case Reports in Pediatrics·Luigi MazzoneStefano Vicari
Mar 28, 2008·Journal of Clinical Gastroenterology·Ehab ElakkaryRobert Bell
Jan 28, 2009·Ophthalmic Genetics·Cristina Villanueva-MendozaJuan Carlos Zenteno
Jan 19, 2012·PloS One·Shubha TiwariGeeta K Vemuganti
Aug 23, 2019·Journal of Endocrinological Investigation·R PolatT Guran
Jul 24, 2020·European Journal of Ophthalmology·Jéssica Botella GarcíaMaria Fideliz de la Paz
Oct 7, 2006·Current Opinion in Gastroenterology·C Prakash, R E Clouse
Oct 20, 2020·Tropical Doctor·Piyush Manoria
Jun 14, 2020·Protein and Peptide Letters·Gabriele PogliaghiMarco Bonomi
Sep 7, 2021·Orbit·Zhenyang Zhao, Richard C Allen
Jul 5, 2006·Pediatrics·Felicia B AxelrodDebra E Weese-Mayer

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