Scurvy

Scurvy

1. SCURVY

2. INTRODUCTION Vitamin C ( Ascorbic acid ) – water soluble vitamin. Ascorbic acid comes from Latin “ Scorbutus ” meaning scurvy. Scurvy is a nutritional deficiency of vitaminC that results from a few weeks to months of no vitamin C supplementation.

3. History Of Scurvy Discovered by Capt James Cook in 1700s. James Lindt – Pioneer in the field of preventing scurvy.

4. Scurvy is characterised clinically by general haemorrhagic tendency. Severe form of disease is rare Mild and subclinical types are relatively common Main effects are on cells and tissues of mesodermal origin, particularly skeletal system.

5. Etiology Unlike most mammals, humans don’t have the ability to synthesize vitaminC on their own, hence we must obtaion VitaminC through our diet.

6. Occurs most commonly and frequently in artificially fed infants between 5-10 months of age. Since vitaminC is destroyed by heat(heating at 100 degrees centigrate destroys it) , exclusive feeding with processed milk that is lacking VitC will result in latent or symptomatic scurvy. Adult scurvy occurs amongst elderly who are on restricted diet – manifests in the form of subcutaneous haemorrhages with slight trauma and delay in healing of wounds.

7. Patho -physiology. VitC deficiency impairs hydroxylation of lysine and proline which are essential for collagen formation. Impairs the cohesive property of matrix of connective tissue and endothelium. Consequenty capillary haemorrhage beneath mucous menbrane and locations of abundant capillary accumulations.

8. Extra-skeletal sites: Gums Intestine Conjunctiva Skin Bladder Kidney

9. Skeletal Manifestations Most vascular skeletal sites are beneath the periosteum and in the marrow, particularly in the metaphysis especially adjascent to the most actively growing epiphysis : Lower end of femur Upper end of tibia Upper end of humerus

10. Subperiosteal haemorrhage Accumulation of blood Ballooning out of periosteum Clotted blood either re-absorbed or transformed to fibrous tissue. At this point when VitC is supplemented, organised haematoma becomes ossified.

11. Subperiosteal haemorrhage and haematoma .

12. Haemorrhages within the metaphysis interferes with osteoblastic tissue. Endochondral ossification proceeds normally as far as calcified cartilage – accumulates in large amounts Notable deficency of osteoblast and osteoclast Broadened layer of calcified cartilage ( White line of fraenkel )

13. WHITE LINE OF FRAENKEL.

14. Within the epiphysis itself, a zone of calcified cartilage accumulates about the bony centrum . Encircling dense ring is known as Wimberger’s ring.

15. Metaphysis in response to haemorrhage becomes extremely hyperemic. Resultant resorption of bone and failure of laying down of new bones. Extremely defficient ossification – appears as dark zones of radiolucency next to the white line.

16. Lack of bone structure and and accumulation of fragile calcified cartilage weaken epiphysio-metaphyseal junction. Resultant fracture and epiphyseal separation. Epiphysis and epiphyseal plate may be completely displaced from the shaft.

17. Haemorrhage throughout the marrow. Fibrous changes and replacement of haematopoietic tissue. Secondary anaemia . Trabeculations – thinned and poorly visualised (Ground glass appearance) Cortices become slender – may result in pathological fracture – metaphysis in infants and diaphysis in adults.

18. Ground glass appearance.

19. Clinical Picture Infant is restless, pale and febrile. Extremities are held immobile Muscles are in spasm Any attempt to move the limbs, results in the child crying out in excruciating pain. Palpable, tender fixed swelling over a bone – result of sub- periosteal haemorrhage . Recent haemorrhage – swelling soft and fluctuant Later – indurated and less tender.

20. Voluntary immobilisation of lower extremitity - PSEUDOPARALYSIS

21. Clinical Picture Gums – Bluish, spongy swelling, especially above upper central incisor teeth. Teeth are lose and brittle.

22. Petechiae and ecchymosis in skin and submucous membrane.

23. Costo-chondral separations are typical. Sternum with the cartilagenous portion of ribs is displaced posteriorly whilesharp anterior ends of the bony ribs protrude anteriorly . SCORBUTIC ROSARY

24. Haematemesis and haematuria may develop. Disease worsens – anorexia weight loss Progressive anaemia Hyper pyrexia Pneumonia Death

25. LABs Blood ascorbic acid level : </= 0.5 mg/dl – diagnostic of scurvy Normal levels : 1mg/dl CBC – Anaemia .

26. Xray findings White line of Fraenkel Wimberger’s sign Ground glass translucency of bone Thinned out cortices. Sub- periosteal haemorrhage Epiphyseal separation Pelkan spur ( Small bony spur protruding out of the lateral border of the metaphysis at its junction with epiphysis)

27. Differential diagnosis Acute osteomyelitis : Usually one bone is involved. Localised pain and constitutional symptoms are severe. Very high leukocyte counts. Leutic osteochondritis : Common before 3 months of age. (scurvy occurs in late infancy) Mother’s serology is positive H/O repeated spontaneous abortion

28. Differential Diagnosis Osteogenesis Imperfecta : Frcatures are diaphyseal rather than metaphyseal . Liabiloty of fractures lessens with advancing age. Diet is adequate. Blood contains adequate amount of ascorbic acid.

29. Treatment VitC supplementation : Fruit juice, Tablets of cevitamic acid. Required daily intake : 30mg for children. 50mg for adults. Therapeutic dose : Minimum of 200mg per day. Therapeutic dose for adult: 800-1000mg/day for atleast 1 week, then 400mg/day till co mpleete recovery.

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