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
QUESTIONS 1 of 1 1 Previous Next (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: A Type & Select Correct Answer 1 Result of decreased osteoclast function 6% (109/1750) 2 Result of Vitamin D deficiency 11% (198/1750) 3 Result of increased chondroitin sulfate and collagen synthesis 18% (323/1750) 4 Primarily effects the epiphysis 28% (483/1750) 5 Causes widening of the zone of provisional calcification 36% (624/1750) L 4 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic
All Videos (0) Podcasts (1) Basic Science⎪ Scurvy Basic Science - Scurvy Listen Now 7:33 min 12/22/2020 151 plays 3.8 (4)