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.
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