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SCURVY
A disease often forgotten!!
Why we should know about Scurvy??
Scurvy is a disease caused by chronic deficiency of vitamin C.
• Being rare ,it is seldom suspected and this frequently leads to delayed
recognition of this disorder.
• Its early diagnosis and appropriate treatment generally has gratifying
results
COOL DUDES BEHIND SCURVY
Captain Cook is credited with solving the
problem of scurvy on his ships by issuing
vitamin C in the form of lemon juice and fresh
fruit and vegetables
James Lind is remembered as the man who
helped to conquer a killer disease. His
reported experiment on board a naval ship
in 1747 showed that oranges and lemons
were a cure for scurvy.
INTRODUCTION- Vitamin C
Vitamin C -known as ascorbic acid - is a water-soluble vitamin.
 Unlike most mammals, humans do not have the ability to make their
own vitamin C. Therefore, we must obtain vitamin C through our diet.
 Fun fact: "ascorbic acid" comes from the New Latin "scorbutus"
meaning scurvy!
Why Vitamin C should be taken in diet ??
“Human beings lack the enzymatic process for conversion of glucose to
ascorbic acid via gluconolactone oxidase unlike other animals (e.g.
rats), therefore vitamin C supplementation in the form of fresh fruits,
vegetables, or dietary supplements is essential
Why do we need Vitamin C??
 Synthesis of collagen, an important
structural component of blood vessels, scar
tissues, tendons, ligaments, and bone.
 Synthesis of the neurotransmitters,
norepinephrine critical to brain function
and are known to affect mood.
 Highly effective antioxidant
 Regenerate other antioxidants such as
vitamin E.
 vitamin C is required for the synthesis of
carnitine, a small molecule that is essential
for the transport of fat to mitochondria, for
conversion to energy.
VITAMIN
C
SOURCES
• Best sources of vitamin C are
citrus fruits (e.g. oranges,
lemons, limes, grapefruits,
gooseberry, black currents,
melons) and vegetables (e.g.
tomato, potatoes, green chilies,
cabbage, broccoli, spinach,
lettuce, cucumber, Brussels
sprouts, red peppers).
• Human milk is richer in vitamin
C than cow's milk.
Many foods can lose their vitamin C content because of cooking,
storage, or oxidation. There is practically no storage in the human
body.
RDA of Vitamin C
The requirement for vitamin
C is increased during
infections inflammatory
states
The new born infant vitamin
C level are related to the
maternal levels – active
placental transfer and
secreted in breast milk.
Who are at Risk of Vitamin C deficiency??
Infants who are fed evaporated
or boiled milk,
 Exclusive meat feeding
Children with dietary restrictions
due to neuropsychiatric or
developmental disorder
 Children with intestinal
malabsorption syndromes
 Patients with end-stage renal
disease undergoing chronic
hemodialysis
Patients on ketogenic diet
HIGH RISK
CLINICAL SYMPTOMS
• Even 8 -12 weeks of irregular or inadequate intake of vitamin C can
result in clinical symptoms.
Early Changes..
• The capillaries are fragile and
there is a tendency to
hemorrhage.
The initial manifestations are
Non-specific such as irritability,
loss of appetite, low grade fever
Dermatological such as
petechiae, ecchymoses,
hyperkeratosis and cork screw
hairs
GUM CHANGES
• Gums become swollen, loosen
and bleed on slightest pressure .
• Gingival disease manifestation
in children is due to poor
dentine formation resulting in
poor teeth formation.
BONE CHANGES
more frequent presentation bringing the child to medical attention.
Deficiency of vitamin C results in a poor formation of the bone
osteoid causing disruption in enchondral bone formation.
The infant may have features of pseudoparalysis and the
presentation may have posture of “pithed frog” with the hips and
knee semiflexed
 The child is frequently irritable and does not like handling.
• Symmetrical involvement –
• Leg swelling (mostly marked at
the knees and the ankles).
Hemorrhages occurring beneath
the periosteum and into the
joints and fractures around
growth plate cause extreme
bone and joints pains. Slipping
of epiphysis around major joints
has been reported
• A “scorbutic rosary” at the
costochondral junctions and
sternum depression are other
characteristic bony features.
DIAGNOSIS
 A low plasma level of
vitamin C - insensitive
laboratory test
 vitamin C level in the
buffy-coat of the
leucocytes - better
estimate of the vitamin
body stores.- not done
routinely
 Leukocyte
concentrations of less
than or equal to 10
mg/108 WBCs are
considered deficient and
indicate latent scurvy
 Urinary excretion
after parenteral
ascorbic acid
infusion.
 After 100 mg of an
intravenous dose
of vitamin C, 80%
should be
excreted within 5
h if the body
stores are not
deficient.
In practice
diagnosis -
clinical +
radiographic
findings.
Radiographic findings
• Most common although nonspecific finding is osteopenia
• A deficient osteoid matrix and loss of trabeculae “ground glass” appearance
• Brittle and fragile bones fracture easily and often heal with abundant callus
formation
• Pencil outlining of the diaphysis and epiphysis due to thin bone cortex
• An irregular thickened white line appears at the metaphysis (White line of
Fraenkel) - the zone of well-calcified cartilage.
• The more definite but late specific radiological feature of scurvy is a zone of
rarefaction beneath Frankelline in the metaphysis - Tru¨mmerfeld (German
word for “field of rubble”) zone.
• beaks found at the periphery of the zone of metaphyseal calcification. They
are associated with periosteal elevation and may be produced by lateral
growth of the calcification zone- Pelkan spurs
• A circular, opaque shadow in the growth centers ioften surrounded by a
white line around the epiphysis, known as Wimberger ring sign.
RADIOGRAPHIC FINDINGS
 Most common although nonspecific finding is
osteopenia
 A deficient osteoid matrix and loss of trabeculae
“ground glass” appearance
 Brittle and fragile bones fracture easily and often heal
with abundant callus formation
 Pencil outlining of the diaphysis and epiphysis due to
thin bone cortex
 An irregular thickened white line appears at the
metaphysis (White line of Fraenkel) - the zone of well-
calcified cartilage.
 The more definite but late specific radiological feature
of scurvy is a zone of rarefaction beneath Frankelline in
the metaphysis - Tru¨mmerfeld (German word for
“field of rubble”) zone.
 beaks found at the periphery of the zone of
metaphyseal calcification. They are associated with
periosteal elevation and may be produced by lateral
growth of the calcification zone- Pelkan spurs
 A circular, opaque shadow in the growth centers often
surrounded by a white line around the epiphysis,
known as Wimberger ring sign.
X RAY OF JOINTS ULTRASOUND MRI
 Osteopenia
 “ground glass”
appearance
 Pencil thin cortex
 White line of
Fraenkel
 Tru¨mmerfeld
(German word for
“field of
rubble”)zone.
 Pelkan spurs
 Wimberger ring
sign.
Bony irregularity,
Bulky subcutaneous
plane,
intramedullary or
periosteal mass,
subperiosteal
hemorrhagic
collections.
 To rule out
malignancy
 Areas of hemorrhage
seen within bones at
the site of fracture and
in the periosteum and
the diffuse marrow
changes
 Multifocal
symmetrical signal
abnormalities
involving the
metaphyses with
associated marrow
enhancements
• A useful mnemonic for remembering many of the common
presentations of scurvy is 4 “H”: hemorrhagic signs, hyperkeratosis,
hematologic abnormalities, and hypochondriasis (delusion of being
sick)
TREATMENT.
Spontaneous bleeding as well as oral and constitutional symptoms are
the foremost to recover (in days) while bone abnormalities and
ecchymoses resolution take longer (in weeks).
Treatment
Infants and children - vitamin C 100 -
300 mg daily
Adults 500- 1000 mg daily for 1 month
or until full recovery of clinical signs
and symptoms occurs
Symptomatic treatment
should also be given in
the form of analgesics
and rest to the part
through splintage
 Complete remodeling
seen
 Residual deformity or
growth disturbance are
seldom reported
CONCLUSION
• Although a rare disease but becoming more commonly in developing
countries where malnutrition is quite prevalent.
• Can present in various forms and can mimic presentation of a
number of common diseases, a high index of suspicion is required
• detailed clinical history including dietary history and comparative
bilateral radiographs diagnosis can be made.
Scurvy in children

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Scurvy in children

  • 2. Why we should know about Scurvy?? Scurvy is a disease caused by chronic deficiency of vitamin C. • Being rare ,it is seldom suspected and this frequently leads to delayed recognition of this disorder. • Its early diagnosis and appropriate treatment generally has gratifying results
  • 3. COOL DUDES BEHIND SCURVY Captain Cook is credited with solving the problem of scurvy on his ships by issuing vitamin C in the form of lemon juice and fresh fruit and vegetables James Lind is remembered as the man who helped to conquer a killer disease. His reported experiment on board a naval ship in 1747 showed that oranges and lemons were a cure for scurvy.
  • 4. INTRODUCTION- Vitamin C Vitamin C -known as ascorbic acid - is a water-soluble vitamin.  Unlike most mammals, humans do not have the ability to make their own vitamin C. Therefore, we must obtain vitamin C through our diet.  Fun fact: "ascorbic acid" comes from the New Latin "scorbutus" meaning scurvy!
  • 5. Why Vitamin C should be taken in diet ?? “Human beings lack the enzymatic process for conversion of glucose to ascorbic acid via gluconolactone oxidase unlike other animals (e.g. rats), therefore vitamin C supplementation in the form of fresh fruits, vegetables, or dietary supplements is essential
  • 6. Why do we need Vitamin C??  Synthesis of collagen, an important structural component of blood vessels, scar tissues, tendons, ligaments, and bone.  Synthesis of the neurotransmitters, norepinephrine critical to brain function and are known to affect mood.  Highly effective antioxidant  Regenerate other antioxidants such as vitamin E.  vitamin C is required for the synthesis of carnitine, a small molecule that is essential for the transport of fat to mitochondria, for conversion to energy. VITAMIN C
  • 7. SOURCES • Best sources of vitamin C are citrus fruits (e.g. oranges, lemons, limes, grapefruits, gooseberry, black currents, melons) and vegetables (e.g. tomato, potatoes, green chilies, cabbage, broccoli, spinach, lettuce, cucumber, Brussels sprouts, red peppers). • Human milk is richer in vitamin C than cow's milk. Many foods can lose their vitamin C content because of cooking, storage, or oxidation. There is practically no storage in the human body.
  • 8. RDA of Vitamin C The requirement for vitamin C is increased during infections inflammatory states The new born infant vitamin C level are related to the maternal levels – active placental transfer and secreted in breast milk.
  • 9. Who are at Risk of Vitamin C deficiency?? Infants who are fed evaporated or boiled milk,  Exclusive meat feeding Children with dietary restrictions due to neuropsychiatric or developmental disorder  Children with intestinal malabsorption syndromes  Patients with end-stage renal disease undergoing chronic hemodialysis Patients on ketogenic diet HIGH RISK
  • 10. CLINICAL SYMPTOMS • Even 8 -12 weeks of irregular or inadequate intake of vitamin C can result in clinical symptoms.
  • 11. Early Changes.. • The capillaries are fragile and there is a tendency to hemorrhage. The initial manifestations are Non-specific such as irritability, loss of appetite, low grade fever Dermatological such as petechiae, ecchymoses, hyperkeratosis and cork screw hairs
  • 12. GUM CHANGES • Gums become swollen, loosen and bleed on slightest pressure . • Gingival disease manifestation in children is due to poor dentine formation resulting in poor teeth formation.
  • 13. BONE CHANGES more frequent presentation bringing the child to medical attention. Deficiency of vitamin C results in a poor formation of the bone osteoid causing disruption in enchondral bone formation. The infant may have features of pseudoparalysis and the presentation may have posture of “pithed frog” with the hips and knee semiflexed  The child is frequently irritable and does not like handling.
  • 14. • Symmetrical involvement – • Leg swelling (mostly marked at the knees and the ankles). Hemorrhages occurring beneath the periosteum and into the joints and fractures around growth plate cause extreme bone and joints pains. Slipping of epiphysis around major joints has been reported • A “scorbutic rosary” at the costochondral junctions and sternum depression are other characteristic bony features.
  • 15. DIAGNOSIS  A low plasma level of vitamin C - insensitive laboratory test  vitamin C level in the buffy-coat of the leucocytes - better estimate of the vitamin body stores.- not done routinely  Leukocyte concentrations of less than or equal to 10 mg/108 WBCs are considered deficient and indicate latent scurvy  Urinary excretion after parenteral ascorbic acid infusion.  After 100 mg of an intravenous dose of vitamin C, 80% should be excreted within 5 h if the body stores are not deficient. In practice diagnosis - clinical + radiographic findings.
  • 16. Radiographic findings • Most common although nonspecific finding is osteopenia • A deficient osteoid matrix and loss of trabeculae “ground glass” appearance • Brittle and fragile bones fracture easily and often heal with abundant callus formation • Pencil outlining of the diaphysis and epiphysis due to thin bone cortex • An irregular thickened white line appears at the metaphysis (White line of Fraenkel) - the zone of well-calcified cartilage. • The more definite but late specific radiological feature of scurvy is a zone of rarefaction beneath Frankelline in the metaphysis - Tru¨mmerfeld (German word for “field of rubble”) zone. • beaks found at the periphery of the zone of metaphyseal calcification. They are associated with periosteal elevation and may be produced by lateral growth of the calcification zone- Pelkan spurs • A circular, opaque shadow in the growth centers ioften surrounded by a white line around the epiphysis, known as Wimberger ring sign.
  • 17. RADIOGRAPHIC FINDINGS  Most common although nonspecific finding is osteopenia  A deficient osteoid matrix and loss of trabeculae “ground glass” appearance  Brittle and fragile bones fracture easily and often heal with abundant callus formation  Pencil outlining of the diaphysis and epiphysis due to thin bone cortex  An irregular thickened white line appears at the metaphysis (White line of Fraenkel) - the zone of well- calcified cartilage.  The more definite but late specific radiological feature of scurvy is a zone of rarefaction beneath Frankelline in the metaphysis - Tru¨mmerfeld (German word for “field of rubble”) zone.  beaks found at the periphery of the zone of metaphyseal calcification. They are associated with periosteal elevation and may be produced by lateral growth of the calcification zone- Pelkan spurs  A circular, opaque shadow in the growth centers often surrounded by a white line around the epiphysis, known as Wimberger ring sign.
  • 18. X RAY OF JOINTS ULTRASOUND MRI  Osteopenia  “ground glass” appearance  Pencil thin cortex  White line of Fraenkel  Tru¨mmerfeld (German word for “field of rubble”)zone.  Pelkan spurs  Wimberger ring sign. Bony irregularity, Bulky subcutaneous plane, intramedullary or periosteal mass, subperiosteal hemorrhagic collections.  To rule out malignancy  Areas of hemorrhage seen within bones at the site of fracture and in the periosteum and the diffuse marrow changes  Multifocal symmetrical signal abnormalities involving the metaphyses with associated marrow enhancements
  • 19. • A useful mnemonic for remembering many of the common presentations of scurvy is 4 “H”: hemorrhagic signs, hyperkeratosis, hematologic abnormalities, and hypochondriasis (delusion of being sick)
  • 20. TREATMENT. Spontaneous bleeding as well as oral and constitutional symptoms are the foremost to recover (in days) while bone abnormalities and ecchymoses resolution take longer (in weeks). Treatment Infants and children - vitamin C 100 - 300 mg daily Adults 500- 1000 mg daily for 1 month or until full recovery of clinical signs and symptoms occurs Symptomatic treatment should also be given in the form of analgesics and rest to the part through splintage  Complete remodeling seen  Residual deformity or growth disturbance are seldom reported
  • 21. CONCLUSION • Although a rare disease but becoming more commonly in developing countries where malnutrition is quite prevalent. • Can present in various forms and can mimic presentation of a number of common diseases, a high index of suspicion is required • detailed clinical history including dietary history and comparative bilateral radiographs diagnosis can be made.