scurvy case scanario and description final.pptx

case based lecture of scurvy

1. A 5 YEAR OLD GIRL WITH Painful LIMBS AND PESUDO-PARALYSIS: DIAGNOSTIC CHALLENGE

2. CASE HISTORY A 5-year-old girl presented in emergency department with the complaints of : Inability to bear weight for 2 months Painful lower limbs for 20 days Generalized body swelling for 10 days

3. CASE HISTORY

4. CASE HISTORY Patient finally reached Sahiwal Teaching Hospital in very miserable condition and was admitted promptly. Apart from above complaints there was no recent history of fever, cough and shortness of breath. There was no significant past medical or surgical history

5. CASE HISTORY Birth history: Birth history was uneventful . She was the third in number of the four children of non-consanguineous marriage. Vaccination History: She was vaccinated according to EPI. Developmental history : Child achieved her mile stones according to age.

6. CASE HISTORY Dietary History: Child was still on mother feed at the age of 5 years. She was mostly fed tea, biscuits and bread. The fruit and vegetables intake was not enough. Socioeconomic History: The child lived in an underprivileged environment. Father was a drug addict and had no permanent source of income. Mother was uneducated and has poor health.

7. EXAMINATION On general physical examination the child was: irritable, asthenic, feverish had skin-mucous paleness. Her weight was below 3 rd centile & height at 25 th centile. There was no lymphadenopathy, jaundice and clubbing.

8. Cutaneous findings include: petechiae, ecchymoses hyperkeratosis and perifollicular hemorrhage gingival hypertrophy with bluish purple discoloration over upper incisors. EXAMINATION

9. Her legs were : extremely tender to touch, extensive non pitting edema on both upper and lower limbs as well as on face and petechial rash all over the lower limbs. LOCAL EXAMINATION OF LIMBS :

10. Joints have a limited range of motion due to extreme tenderness. Her reflexes were diminished, likely due to pain, tenderness and swelling of the limbs. She was non cooperative for detailed neurological examination due to pain and tenderness. NEUROMUSCULAR & JOINT EXAMINATION :

11. SYSTEMIC EXAMINATION :

12. INVESTIGATION Routine investigation Reference range value W.B.C. 5.0-12.1 x 10 3 /uL 3.2 x 10 3 /uL Hb 10.6-13.7gm/dl 6.6 gm/dl Platelets 265-351 x 10 3 / uL 92 x 10 3 /uL ALT 4.8-39.0 U/L 98 U/L Alk po4 97-316 U/L 209 U/L Peripheral film and retics Microcytic hypochromic picture with No abnormal cells. Retics 0.3   ESR 44 mm/hr ESR CRP 110 mg/dl CRP D-Dimers 608 mg/dl D-Dimers

13. Radiological investigation:

14. Can you think of any possible diagnosis ?

15. OUR POSSIBLE DIFFERENTIALS WERE : Multiple Nutritional Deficiencies Leukemia or malignancy DIC Meningococcemia ITP Henoch- Schonlein purpura Hypersensitivity vasculitis

16. Further labs and management : Her bone marrow biopsy was planned. Blood culture sent – no growth found Vitamin D levels sent- turned out to be in sufficienct at 20 Vitamin B 12 and folate levels received normal Broad spectrum IV antibiotics started Fresh frozen plasma and blood transfusion was done

17. Final diagnosis: Meanwhile after reporting from radiology dept her x rays had some diagnostic findings: Generalized osteopenia . Epiphyses are lucent with Wimberger sign . Metaphysis are dense with Trummerfeld zone . Suspected Subperiosteal hemorrhages Possible Torus fracture of femur (MRI needed for further confirmation) SCURVY

18. Further testing require : Plasma vitamin C levels : are not reliable enough to be predictive of scurvy as they can rise or fall with any recent vitamin C intake . Buffy coated Leukocyte vitamin C levels better reflect the body stores however this method is technically more difficult so it was not available in local labs.

19. TREATMENT & FOLLOW UP: She was started on vitamin C and multivitamins. Dietary changes were made. Patients started to respond on the 5th day with reduction in pain and swelling started to subside gradually. Blood line improved . And d-dimers and CRP (As acute phase reactants) had a declining trend. She was discharged on vitamin C therapy. Keeping in mind the child’s socioeconomic background we were Suspecting child neglect in this case , so the grandparents were counselled regarding the grave situation of child’s health and were told to be on regular follow up.

20. SCURVY

21. INTRODUCTION Scurvy is a disease caused by deficiency of Vitamin C. Being benign its seldom suspected and leads to delayed recognition of this disease. Vitamin C –known as ascorbic acid – a water soluble vitamin. Humans lack the enzymatic process for conversion of glucose to ascorbic acid therefore vitamin C supplementation in diet is essential.

22. SOURCE OF VITAMIN C Humans depend on dietary sources for vitamin C. The best food sources of vitamin C are citrus fruits and fruit juices, peppers, berries, melons, guava, kiwifruit, tomatoes, cauliflower, and green leafy vegetables. Human milk is richer in vitamin C than cow’s milk. RECOMMENDED DIETARY ALLOWANCE : 40 mg for ages 0-6 mo 50 mg for 6-12 mo. 15 mg for ages 1-3 yr , 25 mg for 4-8 yr , 45 mg for 9-13 yr.

23. VULNERABLE GROUP FOR SCURVY : Children on highly restrictive diets, devoid of most fruits and vegetables, are at risk of acquiring severe vitamin C deficiency Children with psychiatric illnesses and autism spectrum disorder and eating disorders. The requirement for vitamin C is increased during infectious and diarrheal diseases. Children with intestinal malabsorption syndromes. Patients on ESRD and are on hemodialysis. Infants fed predominantly heat-treated (ultrahigh-temperature or pasteurized) milk or unfortified formulas.

24. Vitamin C is an important antioxidant (electron donor) It is required for hydroxylation of proline residues on procollagen molecules, which support the triple-helix structure of collagen that underpins the integrity of: Skin, Vessel s , Mucous membrane s, Bone . •Vit C contributes to red-cell cytoskeleton protein beta- spectrin , which is crucial to the structure and integrity of the cell. It helps in Neurotransmitter metabolism and Cholesterol metabolism. It maintains the iron and copper atoms in a reduced (active) state. Affect the cellular and immunologic functions of the hematopoietic system. FUNCTION OF VITAMIN C IN BODY

25. Clinical spectrum of scurvy

26. CLINICAL MANIFESTATIONS

27. Clinical photograph showing inflammed marginal gingiva in scurvy. Bluish discolouration of gums

28. Scorbutic Rosary Perifollicular hemorrhages

29. DIAGNOSIS The diagnosis of vitamin C deficiency is usually based on the A history of poor vitamin C intake Characteristic clinical picture Radiographic appearance of the long bones

30. The typical radiographic changes occur at the distal ends of the long bones The shafts of the long bones have a ground-glass appearance because of trabecular atrophy. Pencil outlining of the diaphysis and epiphysis. The white line of Fränkel Trümmerfeld zone Pelkan spur dumbbell or club shape to the affected bone. MRI can demonstrate subperiosteal hematomas along with periostitis, metaphyseal changes, and heterogeneous bone marrow signal intensity.

31. Plasma ascorbate concentration of <0.2 mg/dL usually is considered deficient. A low vitamin C level in plasma is specific for the diagnosis however this is not always reliable indicator as plasma levels may ne normal with recent intake of ascorbic acid. Measuring vitamin C in buffy coat of leukocytes better reflect the body stores but this method is technically more difficult. Saturation of the tissues with vitamin C can be estimated from the urinary excretion of the vitamin after a test dose of ascorbic acid. Generalized nonspecific aminoaciduria is common in scurvy, whereas plasma amino acid levels remain normal.

32. Vitamin C supplements of 100-200 mg/day orally or parenterally ensure rapid and complete cure. The clinical improvement is seen within 1 week in most cases, The treatment should be continued for up to 3 mo for complete recovery. MANAGEMENT

33. PREVENTION Breastfeeding protects against vitamin C deficiency throughout infancy. In children consuming milk formula, fortification with vitamin C must be ensured. Dietary or medicinal supplements are required in children on restrictive diets deficient in vitamin C, severely malnourished children, and those with chronic debilitating conditions (e.g., malignancies, neurologic disorders).

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