Vitamin C Deficiency- Scurvy

Bowing of legs [Ref: Robbins 7/e p458; Nelson 18/c p251; Grainger Radiology 4/e p1942/

• Scurvy results from a long-term deficiency of vitamin C.
• Humans are unable to synthesize vitamin C, due to lack of the enzyme gulonolactone oxidase and therefore depend on dietary sources.
• Vitamin C is not stored in the body, but is taken up by all tissues; the highest levels are found in the pituitary and adrenal glands.
• This disorder occurs more frequently in children (particularly infants) than in adults, and for the most part, results from a diet of pasteurized or boiled milk. (This type of milk is deficient in vitamin C because the vitamin is inactivated by heat).
• For scurvy to develop, the deficiency must be present for a long time of approx. 4 to 6 months. Therefore it is unlikely that the disorder will be seen before the age of 4-6 months.
• The occurrence of scurvy in adults is rare, being seen mostly in patients suffering from chronic severe malnutrition.
• Vitamin C is essential for the hydroxylation of lysine and proline in collagen formation.
• The defect in collagen synthesis results in inadequate support of the walls of capillaries and venules leading to bleeding in the skin and in the gingival mucosa, haemarthroses, and subperiosteal hemorrhages. The gums are swollen and bleed easily. Bleeding in the skin causes petechiae, eccyhmoses, perifollicular hemorrhages.
• Skeletal changes may also develop in infants and children. The pritnaly disturbance is in the formation of osteoid matrix, rather than in mineralization or calcification. (Rickets is characterized by the presence of excess osteoid and lack of calcification; whereas scurvy is associated with deficient osteoid and much calcified cartilage). The potential for skeletal lesions diminish with increasing age.

• The skeletal changes are most pronounced in regions of active endochondral bone growth and therefore are best seen in the distal end of the long bones particularly of the lower extremity. Changes at the sternal ends of the ribs cause scorbutic rosary formation.

Key imaging features of scurvy or Vit. “C” Def

• Osteoporosis
• White line of Frank! (irregular but thickened white line at the growth plate, it represents the zone of well-calcified cartilage).
• Pelkan’s spur (the zone of calcification extends beyond the margins of the metaphyses, resulting in periosteal elevation and inetaphyseal spurs)
• Trummerfield zone or scurvy line (a transverse band of radioluscency subjacent to the white line of Frank!)
• Winiberger’s sign (thin ring of increased density surrounding epiphyses)
• Periosteal elevation (due to subperiosteal hemorrhage)
• The weakened bones yield to the stresses of weight bearing and muscle tension, with bowing of the long bones of the lower legs and abnormal depression of the sternum.
• Wound and fracture healing is impaired due to derangement of collagen synthesis.
• Laboratory diagnosis of vitamin C deficiency is made on the basis of low plasma or leukocyte levels.

James Lind was a Scottish physician. In 1753 he published a treatise of the scurvy was published by James Lind.

Scurvy is a disease now known to be caused by a Vitamin C deficiency, but in Lind’s day, the concept of vitamins was unknown.

Ref: Diseases: Finding the Cure By Robert Mulcah.

A i.e. Lysine

Ans. is ‘a’ i.e., White line in metaphysis

o Ringed epiphysis (Wimburger sign): It is white ring surrounding the epiphyseal centres of ossicification. It is relatively specific for scurvy.

• White line in metaphysis (Freaenkel) can be seen in scurvy, healing rickets, Plumbism, severe PEM, congenital syphilis and acute leukemia

o Option b & d are not specific for scurvy.

A i.e. Epiphyseal widening

Ans. C i.e. Raised serum alkaline phosphatase

Scurvy

• Bleeding into skin & joints,
• Wimberger sign,
• Pseudoparalysis etc.

Ans. C: Hypoglycemia

One of the first signs of scurvy is the development of perifollicular hyperkeratotic papules, often on the shins. These appear as reddish/bluish bruise-like spots surrounding hair follicles. The central hairs are twisted like corkscrews that may break easily. The papules may join together to form large areas of palpable purpura or ecchymoses (bruises).

• Gums may swell and become red, soft and spongy. Any slight friction may cause the gums to bleed. Often this results in poor oral hygiene and dental diseases.
• Bleeding in the joints causes extreme discomfort and pain. Joints may be swollen and tender and the pain can be so severe that patients cannot walk.
• Patients may complain of dryness, irritation, light intolerance, transient visual blurring and stickiness. Haemorrhaging (bleeding) beneath the conjunctiva and within the optic nerve sheath may also occur.
• Anemia develops in 75% of patients as a result of blood loss into tissue, altered absorptions and metabolism of iron and folate, gastrointestinal bleeding and intravascular haemolysis.
• Shortness of breath, low blood pressure, and chest pain leading to shock and death.

Ans. Soap bubble appearance

Ans. is ‘b’ i.e., Pyridoxin

Ans. is ‘b’ i.e., Scurvy

• Vitamin C deficiency causes scurvy.