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Keratomalacia

National Organization for Rare Disorders, Inc.

Important
It is possible that the main title of the report Keratomalacia is not the name you expected.

Disorder Subdivisions

  • None

General Discussion

Keratomalacia is an eye (ocular) condition, usually affecting both eyes (bilateral), that results from severe deficiency of vitamin A. That deficiency may be dietary (i.e., intake) or metabolic (i.e., absorption). Vitamin A is essential for normal vision as well as proper bone growth, healthy skin, and protection of the mucous membranes of the digestive, respiratory, and urinary tracts against infection.

Early symptoms may include poor vision at night or in dim light (night blindness) and extreme dryness of the eyes (i.e., xerophthalmia), followed by wrinkling, progressive cloudiness, and increasing softening of the corneas (i.e., keratomalacia). With advancing vitamin A deficiency, dry, "foamy," silver-gray deposits (Bitot spots) may appear on the delicate membranes covering the whites of the eyes. Without adequate treatment, increasing softening of the corneas may lead to corneal infection, rupture (perforation), and degenerative tissue changes, resulting in blindness. In addition, in some cases, vitamin A deficiency may have additional effects, particularly during infancy and childhood.

In some developing countries, vitamin A deficiency in the diet and associated keratomalacia are a major cause of childhood blindness. In such regions, vitamin A deficiency often occurs as part of nonselective general malnutrition in infants and young children. Although rare in developed countries, vitamin A deficiency and keratomalacia may occur secondary to conditions associated with impaired absorption, storage, or transport of vitamin A, such as celiac disease, ulcerative colitis, cystic fibrosis, liver disease, or intestinal bypass surgery and any condition that affects absorption of fat-soluble vitamins.

Resources

Lighthouse International
111 E 59th St
New York, NY 10022-1202
Tel: (800)829-0500
Email: info@lighthouse.org
Internet: http://www.lighthouse.org

Prevent Blindness America
211 West Wacker Drive
Suite 1700
Chicago, IL 60606
Tel: (312)363-6001
Fax: (312)363-6052
Tel: (800)331-2020
Email: info@preventblindness.org
Internet: http://www.preventblindness.org

NIH/National Eye Institute
31 Center Dr
MSC 2510
Bethesda, MD 20892-2510
United States
Tel: (301)496-5248
Fax: (301)402-1065
Email: 2020@nei.nih.gov
Internet: http://www.nei.nih.gov/

NIH/National Institute of Diabetes, Digestive & Kidney Diseases
Office of Communications & Public Liaison
Bldg 31, Rm 9A06
31 Center Drive, MSC 2560
Bethesda, MD 20892-2560
Tel: (301)496-3583
Email: NDDIC@info.niddk.nih.gov
Internet: http://www2.niddk.nih.gov/

World Health Organization (WHO)
Avenue Appia 20
Geneva 27, 1211
Switzerland
Tel: 41227912111
Fax: 41227913111
Internet: http://www.who.int/en/

Blind Children's Center
4120 Marathon Street
Los Angeles, CA 90029-3584
USA
Tel: (323)664-2153
Fax: (323)665-3828
Tel: (800)222-3566
Email: info@blindchildrenscenter.org
Internet: http://www.blindchildrenscenter.org

Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
Tel: (301)251-4925
Fax: (301)251-4911
Tel: (888)205-2311
TDD: (888)205-3223
Internet: http://rarediseases.info.nih.gov/GARD/

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:  4/21/2008
Copyright  1990, 2001, 2003 National Organization for Rare Disorders, Inc.

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