Vitamin A is one of the fat-soluble and essential vitamins that is acquired through dietary intake. A normal amount of vitamin A is necessary to maintain a healthy skin, immune system, retina, and ocular surface .
Vitamin A deficiency presents with different signs and symptoms. The ocular presentations are variable and potentially affect all epithelial cells of the eyes. These features may range from simple conjunctival and corneal dryness to xerosis, keratomalacia, scarring of the cornea, and perforation. The resulting visual impairment can lead to dysfunctional dark adaptation, night blindness and damage to the pigment epithelial cells of the photoreceptors .
The direct cause of xerophthalmia is hypovitaminosis A. Active corneal xerophthalmia may be observed in severe famines, otherwise, it is an extremely rare occurrence in older children or adults. This condition is considered a medical emergency and requires prompt management using high doses of vitamin A supplementation  .
Keratomalacia is the most severe form of xerophthalmia. More than one-third of the cornea is involved in this case. The cornea can be thickened and edematous and eventually melt away because of necrosis in the structure of collagen in the cornea. This process may only take a few days.
Children affected by keratomalacia are usually malnourished. However, previously healthy children may develop keratomalacia after episodes of diarrhea or measles infection in which vitamin A stores are rapidly depleted as a result of increased metabolism. Generally, these patients were deficient in vitamin A and the infection triggered further depletion of vitamin A stores  .
Nyctalopia is often the first presenting feature and results from rod dysfunction. Cone dysfunction occurs later and to a lesser extent than rod dysfunction .
Entire Body System
Abstract A retrospective review of 3,490 adminissions to the major pediatric facility in El Salvador was undertaken to determine whether biannual administration of massive doses of vitamin A (200,000 international units) to all availabel 1- to 4-year-old [ncbi.nlm.nih.gov]
The test is subjective but malingering and poor cooperation can easily be detected. Adaptometry is a complement to ERG since it is a focal test and therefore can be more sensitive than an ERG in some instances. [symptoma.com]
Without adequate treatment, increasing softening of all or part of the corneas (keratomalacia) may lead to chronic infection, ulceration, and rupture (perforation) of the corneas and degenerative tissue changes (e.g., corneal protrusion and ocular shrinking [rarediseases.org]
Jaw & Teeth
Anterior chamber was shallow and iris was adherent to the area of descemetocele Dilated Fundus Exam No view OD Normal disk, macula, vessels, and periphery Vitamin A deficiency Infectious keratitis Exposure keratopathy Severe dry eye Hereditary sensory [eyewiki.aao.org]
Laboratory Testing Serum vitamin A: Sweat chloride test 127 mmol/ml (normal range: 0-29 mmol/ml) Corneal cultures grew no organism Molecular genetics: Two cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations; 1078deIT mutation and [eyewiki.aao.org]
Mother and his 2 sisters have eczema. There are cataracts in grandparents but no other eye diseases. Allergies No Known Allergies Medìcations Mylicon as needed for gas. The patient lives at home with his mother, father, and 3 siblings. [eyewiki.aao.org]
In children younger than 12 years of age, a serum retinol of less than 0.7 mg/L is considered low. This test is expensive but a direct measure of serum retinol values. A serum retinol-binding protein (RBP) study is not as costly and easier to perform than the serum retinol test since RBP can be detected by an immunologic assay. RBP is more stable with respect to temperature and light but its levels are less accurate than retinol levels because the RBP types cannot be differentiated and the values are affected by serum protein concentrations    .
Electroretinography (ERG) may be used to confirm and monitor retinopathy. Dark adaptometry is used to evaluate night blindness. The test is subjective but malingering and poor cooperation can easily be detected. Adaptometry is a complement to ERG since it is a focal test and therefore can be more sensitive than an ERG in some instances. Dark adaptometry is useful in the evaluation of cone dysfunction syndromes by showing degrees of cone adaptation.
The treatment was done with vitamin-A to which the patient had responded. The mother of the baby had a history of night blindness throughout the pregnancy for which she was also treated. [ncbi.nlm.nih.gov]
Treatment Treatment Options: Four weeks of treatment with IV human Glu-plasminogen in a small group of children and adults with congenital plasminogen deficiency produced marked clinical improvement in ocular as well as disseminated symptoms according [disorders.eyes.arizona.edu]
Treatment includes vitamin A supplementation depending on severity. Always speak with your doctor about the best personal plan of action. [diseaseinfosearch.org]
What treatments are available? Treatment of Keratomalacia should be started immediately after diagnosis. Keratomalacia is irreversible and can cause permanent corneal scarring. [vitaminsdiary.com]
The division of X3 into X3A and X3B was modified to relate more closely to the degree of damage and the ultimate prognosis for vision. Following is this classification of xerophthalmia. [ijo.in]
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]
Each disorder is presented in a step by step manner, beginning with its etiology and pathology, then clinical features and differential diagnosis. Key learning points are provided at the end of each topic. [books.google.com]
The four cases herewith recorded are published on account of the light which has recently been thrown on the etiology of a disease comparatively rare in this country. [jamanetwork.com]
(Etiology) Keratomalacia is an ocular disorder that is caused by the dietary deficiency of vitamin A. [dovemed.com]
Hill Butterworth-Heinemann, ٢٢/١٠/٢٠١٣ - 352 من الصفحات Scientific Foundations of Ophthalmology focuses on scientific grounds of ophthalmology, including anatomy, genetics, pathology, and epidemiology of blindness and blinding diseases. [books.google.com]
Hill Butterworth-Heinemann, 22 oct. 2013 - 352 pagini Scientific Foundations of Ophthalmology focuses on scientific grounds of ophthalmology, including anatomy, genetics, pathology, and epidemiology of blindness and blinding diseases. [books.google.ro]
The epidemiology of vitamin A deficiency and xerophthalmia. Ann Rev Nutr 1984; 183-205. Google Scholar 15. Sommer A, Sugana T, Djunaedi E, Green WR. Vitamin A responsive pan-ocular xerophthalmia in a healthy adult. Arch Ophthalmol 1978; 96: 1630–4. [link.springer.com]
Vitamin A deficiency associated with cystic fibrosis Vitamin A Deficiency Epidemiology Is one of four leading causes of preventable blindness (the other 3: cataract, trachoma, and onchocerciasis). [eyewiki.aao.org]
EPIDEMIOLOGY DEVELOPING COUNTRIES 125 MILLION PRESCHOOL CHILDREN PEAK AGE 2-3 YEARS BITOT’ S SPOTS PREVALENCE 0.5 % 7. CLINICAL FEATURES WHO CLASSIFICATION : XN NIGHT BLINDNESS X1A CONJUCTIVAL XEROSIS X1B BITOT’S SPOTS X2 CORNEAL XEROSIS X3A COR. [slideshare.net]
Darlow BA, Graham PJ ; Vitamin A supplementation to prevent mortality and short and long-term morbidity Cochrane Database Syst Rev. 2007 Oct 17(4):CD000501. [patient.info]
Prompt therapy with parenteral and topical vitamin A, protein supplementation, and a soft contact lens restored corneal integrity and prevented visual loss in one patient. Keratinization of the conjunctiva did not occur in either patient. [ncbi.nlm.nih.gov]
In addition, eyes damaged by keratomalacia are typically treated with topical antibiotics to prevent or stem the infections common in the damaged cornea. [wisegeek.com]
Topical antibiotics are given to prevent secondary bacterial infection. [online-vitamins-guide.com]
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