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Scurvy

Background: Scurvy is the clinical state arising from dietary deficiency of vitamin C (ascorbic acid). Historically, scurvy is an ancient disease. The Egyptians recorded the symptoms as early as 1550 BC. Epidemics were witnessed during the Renaissance era (16th-18th century), at which time scurvy was the scourge of the sea explorers. In 1746, James Lind, a British naval surgeon, established the fact that oranges and lemons were effective in curing scurvy. The incidence among the British sailors sharply declined upon routine provision of lemon juice on board. During the 19th century, people who experienced the Great Potato Famine, armies of the Crimean War and American Civil War, Arctic explorers, and California Gold Rush communities were prominent victims of scurvy on land.

Francis Glisson reported the earliest case of infantile scurvy in 1650 after observing it among infants with rickets. After that, infantile scurvy was not reported for another 200 years. By the end of the 19th century, infantile scurvy was readily recognized and frequently observed in Britain and the United States. The increased incidence of infantile scurvy was attributed to the consumption of heated milk and proprietary foods deficient in vitamin C.

In 1912, Holst and Frolisch induced and cured scurvy in guinea pigs through dietary modification. This practice of supplementing the diet of infants receiving heated formulas with fresh fruit or vegetable juices eventually led to the eradication of infantile scurvy in the United States.

Pathophysiology: Vitamin C deficiency results in impaired collagen synthesis. The typical pathological manifestations of vitamin C deficiency are noted in dentine, osteoid, and capillary vessel wall tissues. Pathological changes are a function of the rate of growth of the affected tissues; hence, the bone changes often are observed only in infants during periods of rapid bone growth.

Defective collagen synthesis leads to defective dentine formation, hemorrhaging into the gums, and loss of teeth. Hemorrhaging is a hallmark feature of scurvy and can occur in any organ. Hair follicles are one of the common sites of cutaneous bleeding.

Bone involvement is typical for infantile scurvy. The bony changes occur at the junction between the end of the diaphysis and growth cartilage. Osteoblasts fail to form osteoid (bone matrix), resulting in cessation of endochondral bone formation. Calcification of the growth cartilage at the end of the long bones continues, leading to the thickening of the growth plate. The typical invasion of the growth cartilage by the capillaries does not occur. Preexisting bone becomes brittle and undergoes resorption at a normal rate, resulting in microscopic fractures of the spicules between the shaft and calcified cartilage. With these fractures, the periosteum becomes loosened resulting in the classic subperiosteal hemorrhage at the ends of the long bones. Intra-articular hemorrhage is rare, because the periosteal attachment to the growth plate is very firm.

Frequency:

* In the US: Currently, scurvy occurs very rarely in the United States. Patients who are elderly or alcoholic and who subsist on diets devoid of fresh fruits and vegetables are vulnerable. Infants on restrictive diets because of medical, economic, or social reasons are vulnerable as well. Occurrence of scurvy is uncommon in those younger than 7 months.

* Internationally: International occurrence is unknown.

Mortality/Morbidity:

* Sudden death due to cardiac failure is reported in infants and adults.

* Predominant morbidity is a result of hemorrhage into various tissues. Subperiosteal hemorrhage in the tibia and femur cause excruciating pain.

Race: No racial predilection exists.

Sex: No sexual preponderance exists.

Age: Scurvy can occur at any age. The majority of cases of infantile scurvy occur when the infant is aged 6-24 months. Scurvy is uncommon in the neonatal period.

History:

* Initial symptoms are nonspecific and include the following:

o Loss of appetite

o Peevishness

o Poor weight gain

o Diarrhea

o Tachypnea

o Fever

* Specific symptoms include the following:

o Irritability

o Pain and tenderness of the legs

o Pseudoparalysis

o Swelling over the long bones

o Hemorrhage

Physical:

* The infant is apprehensive, anxious, and progressively irritable. Upon handling and changing of diapers, severe tenderness over the thighs is present. The excruciating pain results in pseudoparalysis. The infant assumes the frog leg posture (ie, keeping hips and knees slightly flexed and externally rotated) for comfort.

* Hemorrhages of the gums usually involve the tissue around the upper incisors. The gums have a bluish-purple hue and feel spongy. Gum hemorrhage occurs only if teeth have erupted.

* Subperiosteal hemorrhage is a typical finding of infantile scurvy. The lower ends of the femur and tibia are the most frequently involved sites. The subperiosteal hemorrhage often is palpable and tender in the acute phase.

* Petechial hemorrhage of the skin and mucous membranes can occur. Rarely, hematuria, hematochezia, and melena are noted.

* Proptosis of the eyeball secondary to orbital hemorrhage is a sign of scurvy.

* Costochondral beading or scorbutic rosary is a common finding. The scorbutic rosary is distinguished from rickety rosary (which is knobby and nodular) by being more angular and having a step-off at the costochondral junction. The sternum typically is depressed.

* Low-grade fever, anemia, and poor wound healing are signs of scurvy.

* Hyperkeratosis, corkscrew hair, and sicca syndrome typically are observed in adult scurvy but rarely occur in infantile scurvy.

Causes:

* Inadequate intake of vitamin C

* Long sea voyages (historically)

* Famines

* Food faddism

* Ignorance (eg, boiling of fruit juices)

* Neglect

Medical Care:

* Vitamin C administered by mouth or parenteral route is effective in curing infantile scurvy.

* Orange juice also is an effective dietary remedy for curing infantile scurvy and was the standard treatment before the discovery of vitamin C.

* Upon instituting dietary or pharmacological treatment, the clinical recovery is impressive. The appetite of the infant is recovered within 24-48 hours. The symptoms of irritability, fever, tenderness upon palpation, and hemorrhage generally resolve within 7 days.

Diet:

* A diet adequate in vitamin C can prevent the development of scurvy. The dietary requirements of vitamin C sufficient to prevent deficiency vary with age of the individual. The following are the Food and Nutrition Board of the National Academy of Sciences, National Research Council's minimum recommended daily dietary allowances of vitamin C:

o Infants: 30-40 mg

o Children and adults: 45-60 mg

o Pregnant women: 70 mg

o Lactating mothers: 90-95 mg

* Food sources rich in vitamin C include the following:

o Citrus fruits

o Berries

o Cantaloupe

o Broccoli

o Cauliflower

o Cabbage

o Spinach

o Potatoes

o Tomatoes


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