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COLLAGEN DISEASES IN ADOLESCENTS

COLLAGEN DISEASES IN ADOLESCENTS. DR.CHANDRIKA RAO PROFESSOR AND HOD. M.S.RAMAIAH MEDICAL COLLEGE AND HOSPITAL BANGALORE. Definition. Collagen-Greek-`Glue producer` Collagen diseases-A small group of disorders due to structural or metabolic defects in collagen. CASE 6'4",

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COLLAGEN DISEASES IN ADOLESCENTS

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  1. COLLAGEN DISEASES IN ADOLESCENTS DR.CHANDRIKA RAO PROFESSOR AND HOD. M.S.RAMAIAH MEDICAL COLLEGE AND HOSPITAL BANGALORE

  2. Definition • Collagen-Greek-`Glue producer` • Collagen diseases-A small group of disorders due to structural or metabolic defects in collagen.

  3. CASE 6'4", Armspan of 6'7“. He has hypermobile joints in his knees, shoulders and ankles.

  4. CASE

  5. CASE

  6. Collagen-Structure • Generic term for proteins forming a triple helix of three polypeptide chains . • All members of the collagen family form these in the extracellular matrix . • Size, function and tissue distribution vary considerably. • N=28

  7. Collagen vascular disorders • Discoid lupus erythematosus • Systemic lupus erythematosus • Neonatal lupus erythematosus • Juvenile dermatomyositis • Childhood scleroderma

  8. Approach • Detailed history • Progressive • Multiple areas involved • Skin,Musculo-skeletal involvement • Family history • Remmision and relapse

  9. GENETICS Different Types of Mutations in Collagen I Chain Genes Cause Different Disease Severities

  10. Family Tree 45 AAA 28 aortic aneurysm, aneurysm of kidney ? 69 28 AA 31 AA, cerebral hemorrhage 44 28 AA 45 ?valve replacement 13 aortic aneurysm

  11. Ehlers-Danlos syndrome The signs and symptoms of Ehlers-Danlos syndrome vary from mildly loose joints to life-threatening complications

  12. Diseases of Elastic Fiber • Cutis laxa • Williams syndrome • Buschke-Ollendorff syndrome  • Menkes disease  • Pseudoxanthomaelasticum, • Marfan's syndrome 

  13. SLE Revised Diagnostic Criteria • Malar rash • Discoid rash • Photosensitivity • Oral ulcers • Arthritis • Serositis • Renal disorder • Haematologic disorder • Neurologic disoder • Immunologic disoder • ANA 4/11 are present serially or simultaneously.

  14. Preliminary Classification Criteria for Undifferentiated Connective-Tissue Disease

  15. Investigation • CBC,ESR,CRP X-ray • Urinalysis CT Scan • Serum creatinine Early renal biopsy • Rheumatoid factor (RF) • ANA-IFA • Other: • CK,C3, ,TSH,Anti-Ro/SSA,Anti-La/SSB • Anti-Smith,Anti-cardiolipins, • Lupus anticoagulant • Vitamin D - 25(OH)D3

  16. Treatment • Early diagnosis.HEADDSS • May still utilize tried & tested agents initially • Phenytoin, Cyclosporine,Ca Channel blocker-Nifedipine-stimulatory drugs. • Newer immunosuppressive & immunomodulatory-NASAIDS,Cox-2inhibitor, Cyclosporine, Azathioprine, Ca channel blocker.

  17. CONCLUSION • A multidisciplinary approach. • The aim should be to reduce disease activity to a minimum level and to allow treatment free intervals, so that the growth, development, and fertility of these children are ensured.

  18. Thank you • Q:Acquired collagen disorder?

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