Alacrima

Updated: Jun 28, 2016
  • Author: Dan D DeAngelis, MD, FRCSC; Chief Editor: Hampton Roy, Sr, MD  more...
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Overview

Background

Alacrima refers to a wide spectrum of lacrimal secretory disorders that are mostly congenital in origin. Symptoms of these disorders can range from a complete absence of tears to hyposecretion of tears; symptoms of rarer disorders include a selective absence of tearing in response to emotional stimulation but a normal secretory response to mechanical stimulation.

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Pathophysiology

Alacrima is usually inherited in an autosomal recessive fashion, but dominant pedigrees have been described. Mutations in the AAAS gene on chromosome band 12q13 have been described in several pedigrees with Allgrove (or triple-A) syndrome. The AAAS gene encodes a 547-amino acid protein named ALADIN (for alacrima-achalasia-adrenal insufficiency-neurologic disorder), which belongs to the family of regulatory proteins that play an important role in regulating intracellular protein transport. [1]

Etiologies can be separated into pathological mechanisms or syndromic associations. See Causes.

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Epidemiology

Frequency

United States

Alacrima is uncommon.

International

Alacrima is uncommon.

Mortality/Morbidity

Corneal sequelae are the most feared complication of alacrima.

Age

Onset of alacrima occurs in infancy.

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