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FOLLOW THE ARROWS

INSTRUCTIONS. READ EACH QUESTION . GIVE IT YOUR BEST SHOT . FOLLOW THE ARROWS. GOOD LUCK! . Israel Alfonso, MD. 1. MUCOPOLYSACCHARIDES, ALSO KNOWN AS ______________ ARE THE BY PRODUCT OF COLLAGEN CATABOLISM: A. MUCOLIPIDS B. SPHINGOLIPIDS C. OLIGOSACCHARIDES D. GLYCOSAMINOGLYCANS.

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FOLLOW THE ARROWS

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  1. INSTRUCTIONS READ EACH QUESTION GIVE IT YOUR BEST SHOT FOLLOW THE ARROWS GOOD LUCK! Israel Alfonso, MD

  2. 1. MUCOPOLYSACCHARIDES, ALSO KNOWN AS ______________ ARE THE BY PRODUCT OF COLLAGEN CATABOLISM: • A. MUCOLIPIDS • B. SPHINGOLIPIDS • C. OLIGOSACCHARIDES • D. GLYCOSAMINOGLYCANS

  3. RIGHT! SHOW ME THE QUESTION AGAIN I WANT TO SEE A CLUE, ANYWAY GO TO THE NEXT QUESTION

  4. WRONG! I WANT TO TRY AGAIN GIVE ME A CLUE JUST GIVE ME THE ANSWER

  5. PROTEOLYTIC ENZYMES COLLAGEN FIBER HEPARAN SULFATE DERMATAN SULFATE KERATAN SULFATE CHONDROTIN SULFATE THIS SUBSTANCES ARE CALLED MUCOPOLYSACCHARIDES OR GLYCOSAMINOGLYCANS

  6. 1. MUCOPOLYSACCHARIDES ALSO KNOWN AS ______________ ARE THE BY PRODUCT OF COLLAGEN CATABOLISM: • A. MUCOLIPIDS • B. SPHINGOLIPIDS • C. OLIGOSACCHARIDES • D. GLYCOSAMINOGLYCANS

  7. 2. WHICH OF THE FOLLOWING CONDITIONS IS X-LINKED: A. HURLER SYNDROME B. SCHEIE SYNDROME C. HUNTER SYNDROME D. MORQUIO SYNDROME

  8. RIGHT! SHOW ME THE QUESTION AGAIN I WANT TO SEE A CLUE, ANYWAY GO TO THE NEXT QUESTION

  9. WRONG! I WANT TO TRY AGAIN GIVE ME A CLUE JUST GIVE ME THE ANSWER

  10. HUNTER SYNDROME X-LINKED ONLY BOYS BOYS ARE GOOD HUNTERS THE ONLY OTHER X-LINKED LYSOSOMAL DISORDER IS FABRY

  11. 2. WHICH OF THE FOLLOWING CONDITIONS IS X-LINKED: A. HURLER SYNDROME B. SCHEIE SYNDROME C. HUNTER SYNDROME D. MORQUIO SYNDROME

  12. 3. A DEFICIENCY OF a -L- IDURONIDASE PRODUCES : A. HURLER SYNDROME B. HUNTER SYNDROME C. SANFILIPPO B D. SANFILIPPO A

  13. RIGHT! SHOW ME THE QUESTION AGAIN I WANT TO SEE A CLUE, ANYWAY GO TO THE NEXT QUESTION

  14. WRONG! I WANT TO TRY AGAIN GIVE ME A CLUE JUST GIVE ME THE ANSWER

  15. A-L-IDURONIDASE HURLER COARSE FACE DYSOSTOSIS MULTIPLEX LIVER BIG

  16. 3. A DEFICIENCY OF a -L- IDURONIDASE PRODUCES : A. HURLER SYNDROME B. HUNTER SYNDROME C. SANFILIPPO B D. SANFILIPPO A

  17. 4. WHICH OF THE FOLLOWING IS NOT ASSOCIATED WITH HURLER SYNDROME? • A. BRACHIOCEPHALY • B. HYPERTELORISM • C. HEARING LOSS • D. CORNEA CLOUDING

  18. RIGHT! SHOW ME THE QUESTION AGAIN I WANT TO SEE A CLUE, ANYWAY GO TO THE NEXT QUESTION

  19. WRONG! I WANT TO TRY AGAIN GIVE ME A CLUE JUST GIVE ME THE ANSWER

  20. FACIAL FEATURES OF HURLER SYNDROME CORNEAL CLOUDING FLAT NOSE BRIDGE LARGE NARES THICK LIPS APPEARS FROM 6 TO 24 MONTHS DEAFNESS SCAPHOCEPHALY HEAVY EYE BROWS HYPERTELORISM PROMINENT TONGUE PROMINENT SUPRAORBITAL RIDGES

  21. FACIAL FEATURES OF HUNTER SYNDROME FLAT NOSE BRIDGE LARGE NARES THICK LIPS LESS PROMINENT APPEARS FROM 2 TO 4 YEARS SCAPHOCEPHALY HEAVY EYE BROWS HYPERTELORISM PROMINENT TONGUE PROMINENT SUPRAORBITAL RIDGES

  22. 4. WHICH OF THE FOLLOWING IS NOT ASSOCIATED WITH HURLER SYNDROME? • A. BRACHIOCEPHALY • B. HYPERTELORISM • C. HEARING LOSS • D. CORNEA CLOUDING

  23. 5. WHICH OF THE FOLLOWING IS MORE COMMON IN INFANTS WITH HURLER SYNDROME: • A. CARDIOMYOPATHY • B. LIVER FAILURE • C. FREQUENT BONE FRACTURES • D. ASTHMA

  24. RIGHT! SHOW ME THE QUESTION AGAIN I WANT TO SEE A CLUE, ANYWAY GO TO THE NEXT QUESTION

  25. WRONG! I WANT TO TRY AGAIN GIVE ME A CLUE JUST GIVE ME THE ANSWER

  26. DUE TO DEPOSITION OF DERMATAN AND HEPARAN SULFATE IN THE MYOCARDIUM AND VALVES

  27. 5. WHICH OF THE FOLLOWING IS MORE COMMON IN INFANTS WITH HURLER SYNDROME: • A. CARDIOMYOPATHY • B. LIVER FAILURE • C. FREQUENT BONE FRACTURES • D. ASTHMA

  28. 6. CHILDREN WITH HURLER SYNDROME FREQUENTLY HAVE UPPER RESPIRATORY SYMPTOMS AND OTITIS MEDIA, WHY? • A. BAD IMMUNOLOGICAL SYSTEM • B. ABNORMAL BONY CALVARIUM • C. POOR HYGIENE • D. ALLERGIES

  29. RIGHT! SHOW ME THE QUESTION AGAIN I WANT TO SEE A CLUE, ANYWAY GO TO THE NEXT QUESTION

  30. WRONG! I WANT TO TRY AGAIN GIVE ME A CLUE JUST GIVE ME THE ANSWER

  31. CRANIAL DYSOSTOSIS SAGITTAL SYNOSTOSIS ENLARGE J-SHAPED SELLA THICK PARTIAL LAMBDOIDAL SYNOSTOSIS HYPOPLASIA OF MIDFACIAL BONES

  32. 6. CHILDREN WITH HURLER SYNDROME FREQUENTLY HAVE UPPER RESPIRATORY SYMPTOMS AND OTITIS MEDIA, WHY? • A. BAD IMMUNOLOGICAL SYSTEM • B. ABNORMAL BONY CALVARIUM • C. POOR HYGIENE • D. ALLERGIES

  33. 7. DYSTOSIS MULTIPLEX IN PATIENTS WITH HURLER SYNDROME IS CHARACTERIZED BY ALL OF THE FOLLOWING EXCEPT: A. OAR SHAPED RIBS B. ANTERIOR HYPOPLASIA OF THE LUMBAR VERTEBRAS C. HYPOPLASTIC ILIA D. LARGE FEMORAL HEADS

  34. RIGHT! SHOW ME THE QUESTION AGAIN I WANT TO SEE A CLUE, ANYWAY GO TO THE NEXT QUESTION

  35. WRONG! I WANT TO TRY AGAIN GIVE ME A CLUE JUST GIVE ME THE ANSWER

  36. ANTERIOR HYPOPLASIA OF THE VERTEBRAS AND KYPHOSIS LONG BONES: BROAD AND SHORT HURLER SYNDROME BROAD, BULLET LIKE PHALANGES HYPOBLASTIC ILIA & FEMORAL HEAD

  37. HURLER SYNDROME OAR-SHAPED RIBS ARE NARROW AT THE VERTEBRAL END AND BROAD AT THE STERNAL AGE

  38. 7. DYSTOSIS MULTIPLEX IN PATIENTS WITH HURLER SYNDROME IS CHARACTERIZED BY ALL OF THE FOLLOWING EXCEPT: A. OAR SHAPED RIBS B. ANTERIOR HYPOPLASIA OF THE LUMBAR VERTEBRAS C. HYPOPLASTIC ILIA D. LARGE FEMORAL HEADS

  39. 8. WHICH OF THE FOLLOWING SUBSTANCE IS EXCRETED IN THE URINE OF CHILDREN WITH HURLER SYNDROME? A. HEPARAN AND KERATAN B. KERATAN AND CHONDROTIN C. DERMATAN AND HEPARAN D. HEPARAN AND CHONDROTIN

  40. RIGHT! SHOW ME THE QUESTION AGAIN I WANT TO SEE A CLUE, ANYWAY GO TO THE NEXT QUESTION

  41. WRONG! I WANT TO TRY AGAIN GIVE ME A CLUE JUST GIVE ME THE ANSWER

  42. ENZYMES PROTEOLYTIC ENZYMES GLYCOSAMINOGLYCANS CATABOLISM COLLAGEN FIBER HEPARAN SULFATE HEPARAN SULFATE DERMATAN SULFATE DERMATAN SULFATE HURLER SCHEIE HURLER-SCHEIE a-L-IDURONIDASE SIMPLE SUGAR HEPARAN SULFATE DERMATAN SULFATE KERATAN SULFATE CHONDROTIN SULFATE 4p16.3

  43. 8. WHICH OF THE FOLLOWING SUBSTANCE IS EXCRETED IN THE URINE OF CHILDREN WITH HURLER SYNDROME? A. HEPARAN AND KERATAN B. KERATAN AND CHONDROTIN C. DERMATAN AND HEPARAN D. HEPARAN AND CHONDROTIN

  44. 9. THE GENE ENCODING a –L-IDURONIDASE, THE ENZYME INVOLVED IN HURLER SYNDROME IS LOCATED AT : A. 4p16.3 B. Xq28 C. 17q25.3 D. 7q21.1

  45. RIGHT! SHOW ME THE QUESTION AGAIN I WANT TO SEE A CLUE, ANYWAY GO TO THE NEXT QUESTION

  46. WRONG! I WANT TO TRY AGAIN GIVE ME A CLUE JUST GIVE ME THE ANSWER

  47. ENZYMES PROTEOLYTIC ENZYMES GLYCOSAMINOGLYCANS CATABOLISM COLLAGEN FIBER HEPARAN SULFATE HEPARAN SULFATE DERMATAN SULFATE DERMATAN SULFATE HURLER SCHEIE HURLER-SCHEIE a-L-IDURONIDASE SIMPLE SUGAR HEPARAN SULFATE DERMATAN SULFATE KERATAN SULFATE CHONDROTIN SULFATE 4p16.3

  48. 4p16.3 THE ONLY ONE OF THE MUCOPOLYSACCHARIDOSIS WITH GENE LOCATION IN THE SMALL ARM (p).

  49. 9. THE GENE ENCODING a –L-IDURONIDASE, THE ENZYME INVOLVED IN HURLER SYNDROME IS LOCATED AT : A. 4p16.3 B. Xq28 C. 17q25.3 D. 7q21.1

  50. 10. THE MOST FREQUENT CAUSE OF HYDROCEPHALUS IN PATIENTS WITH HURLER SYNDROME IS? A. AQUEDUCTAL STENOSIS B. OBSTRUCTION OF THE ORIFICE OF MONROE C. NON-COMMUNICATING HYDROCEPHALUS D. COMMUNICATING HYDROCEPHALUS

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