Updated: 6/18/2021

Scurvy

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Questions
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Evidence
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Topic
Images
https://upload.orthobullets.com/topic/9034/images/scurvy xr1.jpg
https://upload.orthobullets.com/topic/9034/images/scurvy knee.jpg
https://upload.orthobullets.com/topic/9034/images/scurvy wrist.jpg
https://upload.orthobullets.com/topic/9034/images/scorbutic rosary.jpg
https://upload.orthobullets.com/topic/9034/images/rachitic rosary.jpg
https://upload.orthobullets.com/topic/9034/images/wimberger.jpg
  • summary
    • Scurvy is disease caused by severe Vitamin C deficiency which presents with joint effusions, swelling over long bones, bleeding gums, loosening of teeth, hematuria, and susceptibility to hemorrhage.
    • Diagnosis is made based on history, clinical and radiological picture, and resolution of symptoms following vitamin C administration. Lab tests are usually not helpful.
    • Treatment is prompt administration of Vitamin C. 
  • Epidemiology
    • Incidence
      • 8% of men and 6% of women in the US have vitamin C deficiency
    • Demographics
      • male: female ratio is 4:3
      • Bimodal age bracket
        • infants 5-10months
          • uncommon in infants <7mths who are being breast fed as breast milk has vitamin C
        • men >60 years
    • Anatomic location
      • wrists, knees, sternal ends of ribs
        • areas of rapid growth in children
    • Risk factors
      • elderly, especially men who live alone
        • chronic malnutrition
        • overcooking destroys vitamin C
      • alcoholic
      • smokers
      • malabsorptive conditions (Whipple's disease, inflammatory bowel disease, cancer chemotherapy)
  • Etiology
    • Pathophysiology
      • humans are unable to synthesize L-ascorbic acid because the enzyme L-gluconolactone oxidase is nonfunctional
      • Vitamin C deficiency leads to decrease in chondroitin sulfate and collagen synthesis and repair
      • impaired intracellular hydroxylation of collagen peptides
      • net effect is altered bone formatin with the greatest effect occuring in the metaphysis
      • defect in spongiosa of the metaphysis at the growth plate
      • because the demand for type I collagen is greatest during new bone formation
  • Presentation
    • History
      • infant diet consisting of evaporated or condensed milk
      • "tea and toast" diet in elderly
    • Symptoms
      • malaise and fatigue
      • pain
        • bone pain
        • myalgia, because of reduced carnitine production
      • bleeding
        • gum bleeding and loosening of teeth
        • hematuria
        • hematemesis
        • hemorrhage
        • iron deficiency
    • Physical exam
      • petechiae and ecchymosis
      • joint effusions
      • swelling over long bones because of subperiosteal hemorrhage
      • scorbutic rosary (costochondral separation)
        • angular step-off deformity in children
        • differentiated from rachitic rosary, which is rounded and nodular
  • Imaging
    • Radiographs
      • recommended views
        • wrist radiographs
        • knee
        • sternal ends of ribs
      • findings
        • the white line of Frankel
          • widened zone of provisional calcification
          • between epiphysis and metaphysis
        • Trummerfeld zone
          • transvese radiolucent band in the metaphysis adjacent to the Frankel line
          • also known as the scurvy line
        • Wimberger ring
          • ring of increased density surrounding epiphysis
        • Pelkin spur and fracture
          • metaphyseal spurs and fractures
        • corner sign of Park
          • metaphyseal clefts
        • thin cortices ("pencil-point" cortex)
        • decreased trabeculae with ground-glass osteopenia
        • subperiosteal elevation
        • epiphyseal separation
        • fractures and dislocations
  • Studies
    • The diagnosis is usually made based on history, clinical and radiological picture, and resolution of symptoms following vitamin C administration. Lab tests are usually not helpful.
    • Labs
      • fasting serum ascorbic acid level is low
    • Histology
      • replacement of primary trabeculae with granulation tissue
      • areas of hemorhage
      • widening zone of provisional calcification of the physis
  • Treatment
    • Nonoperative
      • vitamin C replacement
      • indications
        • signs and symptoms of scurvy
        • chronic malnutrition
      • techniques
        • oral vitamin C at 250mg qid x 1 week in adults
  • Prognosis
    • Excellent prognosis if treated early
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(OBQ08.26) A 7-year-old male presents to the orthopedic clinic with leg pain that has been present for the past 6 months. The patient recently immigrated to the United States from Somalia. On physical exam, the patient appears malnourished. There is tenderness to palpation of the distal femur. Additionally, the gums have a bluish-purple hue with areas of hemorrhages. A radiograph of the patient's knee is demonstrated in Figure A. Given the most likely diagnosis, which of the following is true regarding this condition?

QID: 412
FIGURES:
1

Result of decreased osteoclast function

6%

(100/1623)

2

Result of Vitamin D deficiency

10%

(167/1623)

3

Result of increased chondroitin sulfate and collagen synthesis

19%

(313/1623)

4

Primarily effects the epiphysis

29%

(468/1623)

5

Causes widening of the zone of provisional calcification

35%

(563/1623)

L 4 D

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