National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Keratomalacia is not the name you expected.
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.
National Association for Visually Handicapped
22 West 21st Street
New York, NY 10010
National Eye Research Foundation
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Northbrook, IL 60062
Prevent Blindness America
211 West Wacker Drive
Chicago, IL 60606
NIH/National Digestive Diseases Information Clearinghouse
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Bethesda, MD 20892-3570
NIH/National Eye Institute
Building 31 Rm 6A32
31 Center Dr MSC 2510
Bethesda, MD 20892-2510
World Health Organization (WHO) Regional Office for the Americas (AMRO)
Pan American Health Organization (PAHO)
525 23rd Street NW
Washington, DC 20037
Blind Children's Center
4120 Marathon Street
Los Angeles, CA 90029-3584
Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
For a Complete Report
For a Complete Report
This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html