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.
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.
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)
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.
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.
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
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.
28. 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
29. 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.
30. 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.