Excerpt from Scurvy

Synonyms, Key Words, and Related Terms: infantile scurvy, Barlow disease, Barlow’s disease, vitamin C deficiency, ascorbic acid, widower scurvy, chronic malnutrition

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Background: Scurvy is a condition that has been described for at least 500 years. In 1753, Sir James Lind demonstrated that the condition could be prevented in British sailors by adding citrus fruits to their diets. Forty years later, Sir Gilbert Blane convinced the admiralty of the British Navy to approve a preventive dietary regimen for sailors. Prior to that, all sea voyages had to be limited to 10 weeks or less to rehabilitate the crews. Scurvy produces characteristic perifollicular hemorrhages and gingival changes as well as hematologic, joint, and cardiac complications.

Pathophysiology: Scurvy is caused by a prolonged deficiency of vitamin C intake that results in defective collagen synthesis, tissue repair, and synthesis of lipids and proteins. It functions both as a reducing agent and as an antioxidant and is required for many physiologic functions, including metabolism of iron and folic acid, resistance to infection, and integrity of blood vessels.

The clinical manifestations of scurvy are primarily due to abnormal collagen synthesis resulting from a lack of vitamin C. Vitamin C is a cofactor required for the function of several hydroxylases. The absence of vitamin C reduces the function of prolyl hydroxylase, which is required to form hydroxyproline, an amino acid found in collagen but rarely found in other proteins. The presence of hydroxyproline in collagen stabilizes the collagen triple-helix structure by forming interstrand hydrogen bonds. Collagen lacking hydroxyproline is more fragile and contributes to the clinical manifestations of scurvy, including purpura due to vessel wall fragility. In addition, osteoid matrix formation is defective and bone resorption is increased in persons with vitamin C deficiency.

Frequency:

In the US: Scurvy is rare in the United States. Patients at risk include those with chronic malnutrition, such as persons with alcoholism, elderly people, and men who live alone (widower scurvy). Other reported cases include people with monotonous or peculiar diets, including patients undergoing dialysis and those with malabsorption, inflammatory bowel disease, cancer on chemotherapy, Whipple disease, or dyspepsia (those who avoid acidic foods). Scurvy may occur in infants fed evaporated or condensed milk formulas.
Infantile scurvy was fairly common in the United States during the 1950s, but changes in feeding practices in the next decade almost completely eliminated the disorder. If a mother has an adequate diet, breast milk contains sufficient vitamin C for a baby's needs. Commercially available formulas and many prepared fruit juices are fortified with vitamin C.

Internationally: Scurvy is a problem when general malnutrition exists, as in some impoverished, underdeveloped third world countries. Scurvy also occurs in epidemic proportions in international refugee camps and in populations that subsist mainly on cereal grains.

Mortality/Morbidity: Until minimal daily requirements of vitamin C were supplied, scurvy plagued prolonged naval voyages and military campaigns as personnel succumbed to its devastating effects. Lethargy, fatigue, and hemorrhagic manifestations of impaired collagen synthesis affecting oral, ophthalmic, musculoskeletal, cardiac, and gastrointestinal structures and functions incapacitated or killed more people than enemy action in many cases. Manifestations of scurvy tend to dramatically improve, resolving within weeks, if adequate vitamin C is given in daily doses to recoup body stores.

Race: No racial predilection for scurvy has been sited in the literature

Sex: No sex predilection for scurvy has been sited in the literature.