Anupa mishra

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Anupa mishra

  1. 1. BY DR ANUPAGUIDED BY: DR MANOJ GHODA DR REKHA H BHAVSAR DR ROHIT R MODI DR DEEPA A BANKER DR PALLAVI P DAGLI
  2. 2. A7 years old male childborn out of a nonconsanguinous marriagepresented with thefollowing complaints:
  3. 3.  An umbilical hernia since age of 6 months which was gradually increasing in size Noisy breathing/ snoring at night Swelling of small joints of fingers with restricted movements of both wrists and fingers
  4. 4. Coarse facies Umbilical hernia and protuberant abdomen Short spade like handsShort stature
  5. 5. A huge hepatosplenomegaly Systolic murmurCNS: mild delay of milestonesOpthalmologic exam: normal
  6. 6.  Hb: 12gm%  S. Mg2+: 2.5 WBC count: 13000  S. phosphorus: 4.7 Differential count:  ALP : 287 71% neutrophils & 22% lymphocytes PS-non specific ESR: 15 CRP - negative S.Ca2: 10.2
  7. 7.  Chest Xray: scoliosis, oar shaped ribs X ray of the wrist and hand: widening of metaphysis of wrist joints, short and widened phalanges..typical features of dysostosis multiplexa. 2 D echo: moderate MR, mild TR, Mild PAH, borderline LVH bicuspid aortic valve
  8. 8. Special investigations done: Liver biopsy: s/o some storage disorder Urine for GAGs: positive ( type???)
  9. 9. For definitive diagnosis:Enzyme assays on culturefibroblalsts or serum leukocytes
  10. 10.  Now that was simple wasn’t it?? A book picture of MUCOPOLYSACCHARIDOSIS!! But what if such was not the case??!!
  11. 11. Very often what we have is only a large spleen!!What to do then?? Lets see!!
  12. 12. HEMATOLOGICAL AUTOIMMUNECONGESTIVE CONDITIONS CHRONIC INFECTIONS PARASITIC INFECTIONS STORAGE DISORDERS
  13. 13.  Age? Residence? Community?
  14. 14. Is there Fever? Duration? Pallor? Bleeding from any site? Rashes? Transfusion of blood or blood products?
  15. 15.  Blood in vomitus? Jaundice? Convulsions? Involuntary movements or developmental delay?
  16. 16.  Familyhistory? Maternal story?
  17. 17.  Facies and skull Pallor Jaundice Rashes/ petehiae/purpura/ echhymosis Lymphadenopathy Eyes Anthropometry
  18. 18.  Distended abdomen? Dilated veins? Hernial sites? Ascites?Now look for the size of the spleen...
  19. 19. Mild splenomegaly:•Infections•CCF•Portalhypertension(earlystage)•Acquired Hemolyticanaemias
  20. 20. Moderatesplenomegaly:•Malaria•Kala Azar•HIV•Portal hypertension
  21. 21. Massivesplenomegaly:•Leukemia•Kala Azar•Chronic hemoyticanaemias•Storage disorders
  22. 22. If not significant.. Malaria Kala azar Portal hypertension Chronic hemolytc anemias
  23. 23. CBC including retic countPeripheral smearLFTsXraysUSG abdomen
  24. 24.  Upper GI Scopy Hb elctrophoresis Bone Marrow examination Urine analysis
  25. 25. The Mathematics of Splenomegaly...
  26. 26. fever pallor PS parasite Kala azar Malaria
  27. 27. Chronic fever Autoimmune Joint rashes disorders pains Lymph malignan nodes cy Anemia/ PS bleeding picture hemolytic ESR
  28. 28. Chronic fever Wt tuberculosis loss/mt / cxrayLymphnodes murmur RHD/IE ESR
  29. 29. Hemetemesis/ jaundice/ Altered edema LFTs Small USG/UGI liver scopy Portal hypertension
  30. 30. Ophthal Skeleton screen abnormalCoarsefacies Neurologic deficits Storage disorder
  31. 31.  To take a detailed clinical history.. Always examine from head to toe.. Do not jump to the spleen directly Even the baseline investigations can give so many clues about the final diagnosis Narrow down to the group of disorders that you are dealing with.

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