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Information

 Bone and Soft-Tissue Disorders

= BARLOW DISEASE = HYPOVITAMINOSIS C

= vitamin C (= ascorbic acid) deficiency with defective osteogenesis from abnormal osteoblast function

Infantile Scurvy  !!navigator!!

Age: 6–9 months (maternal vitamin C protects for first 6 months)

Predisposed: feeding with pasteurized / boiled milk

Pathogenesis: abnormal collagen formation

  • irritability
  • tenderness + weakness of lower limbs
  • scorbutic rosary of ribs
  • bleeding of gums teething
  • legs drawn up + widely spread = pseudoparalysis

Location: distal femur (esp. medial side), proximal and distal tibia + fibula, distal radius + ulna, proximal humerus, sternal end of ribs

  • “ground-glass” osteoporosis (CHARACTERISTIC)
  • cortical thinning
  • soft-tissue edema (rare)
  • Metaphysis
    • white line = metaphyseal zone of preparatory calcification (DDx: lead / phosphorus poisoning, bismuth treatment, healing rickets)
    • Trümmerfeld zone = radiolucent zone on shaft side of Fränkel white line (site of subepiphyseal infraction)
    • Pelkan spurs = metaphyseal spurs projecting at right angles to shaft axis
      [Karl Francis Pel(i)kan (1890–????), University of California]
    • Park corner sign = subepiphyseal infraction / comminution resulting in mushrooming / cupping of epiphysis (DDx: syphilis, rickets)
      [E. Park, 1935, pathologist]
  • Epiphysis
    • Wimberger ring = sclerotic ring around low-density epiphysis osteopenia of epiphysis
  • Diaphysis
    • subperiosteal hematoma with calcification of elevated periosteum (sure sign of healing)
  • Teeth
    • cyst formation + hemorrhage in enamel

DDx: TORCH infections, leukemia, neuroblastoma

Adult Scurvy  !!navigator!!

Frequency: rare

  • hemarthrosis + bleeding at synchondrosis

 Outline