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Bronchiectasis 2012 Prof. Dr. Bülent KARADAĞ

Bronchiectasis 2012 Prof. Dr. Bülent KARADAĞ Marmara Uni. Division of Paediatric Pulmonology. Bronchiectasis , children. 2305 articles 2013: 16 2012: 99 Turkey: 71 ( 3). Childhood Pneumonectomies: Two Decades' Experience of a Referral Center.

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Bronchiectasis 2012 Prof. Dr. Bülent KARADAĞ

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  1. Bronchiectasis 2012 Prof. Dr. Bülent KARADAĞ Marmara Uni. Division of Paediatric Pulmonology

  2. Bronchiectasis, children • 2305 articles • 2013: 16 • 2012: 99 • Turkey: 71 ( 3) .

  3. Childhood Pneumonectomies: Two Decades' Experience of a Referral Center. Yalcin S, Ciftci A, Karnak I, Ekinci S, Tanyel FC, Senocak M. Eur J Pediatr Surg.2012Aug 17. . Different features of lung involvement in Niemann-Pick disease and Gaucher disease. Gülhan B, Ozçelik U, Gürakan F, Güçer S, Orhan D, Cinel G, Yalçin E, Ersöz DD, Kiper N, Yüce A, Kale G. Respir Med.2012Sep;106(9):1278-85. Surgical treatment of right middle lobe syndrome in children. Sehitogullari A, Sayir F, Cobanoglu U, Bilici S. Ann Thorac Med.2012Jan;7(1):8-11.

  4. Bronchiectasis 1.PCDDiagnosis-Treatment 2. PCD Genetics 3. PCD LFT 4. BE Diagnosis 5. BE Etiology 6. BE Disease burden 7. BE Surgery

  5. 6th February- Diamond Year of Queen Elisabeth

  6. ERS Task Force 26 countries, 194 centres Pt per center : 4 (2-9) 90% nasal/mucosal biopsy 77% electron microscopy, 57% cilairy function tests Nasal NO 46%, Saccharine 36% Variable management

  7. Arab Spring-27th February

  8. PCD Genetics Exome sequencing for CCDC114 mutations Knowles MR, Am J Hum Genet. 2013;10;92. Mutations causing PCD in 14 genes (60%) Sequence analysis in 6 probands on the ODA Chlamydomonas reinhardtii motility gene (DNAI2, DNAH5)

  9. PCD Genetics HEATR2 mutation Am J Hum Genet. 2012;91:685. Horani A Amish community OR, previously undefined İmmunohistochemistry: HEATR2 in cytoplasm, not in cilia. Its role in the dynein arm transport?

  10. PCD Genetics Mata M, Am J Rhinol Allergy 2012;26:260. Adenylate kinase 7 (AK7) mutation. Mutations described in 5 genes. Gene causing PCD? Lower levels of AK7 gene expression 2 mutations described in AK7 gene

  11. CCDC114 = ODA microtubule-docking complex

  12. CCDC39,40 genes are related to IDA defects and axonemal irregularity Biallelic CCDC39 or CCDC40 mutations Patients with IDA defect 30/34 (%88.2)

  13. Normal ciliary structure and lower levels of NO and PCD symptoms (DNAH11). 163 pts Normal structure=58 23 indefinitive (17 situs inversus or BE, 6 low NO) 13 pts with situs inversus… DNAH11 58pts with PCD 22% DNAH11

  14. Munch- Scream (120 million euro) 2ndMay 2012

  15. Shoemark A, J Clin Pathol 2012;65:267 20 years, 1182 pts 242 (20%) PCD 33 pts.. normal (3%) Electron microscopy does not exclude the diagnosis!

  16. ı URT complications URT Sequelae follow-up ? OM with effusion and hearing loss do not recover after 9 years of age. Treatment increasing the mucociliary clearence ?

  17. Spirometry or Plethysmography? P. aeruginosa inf. 40% , BE 88 % P. aeruginosa and BE (p=0.009; r=0.367) and air trapping (p=0.03; r=0.315) P. aeruginosa and RV >150% (p=0.04) and RV/TLC >140% (p=0.001)

  18. No correlation with P. aeruginosa infection and LFT HRCT and RV (p=0.003; r=0.423), RV/TLC (p<0.001; r =0.513) Plethysmography >spirometry,

  19. Spirometry ve progression of lung disease 20 pts comparison with HRCT (11.6 years)

  20. <15 years, At least 8 years follow-up, 20 pts LFT worsens with age CT scores increases with age, and inversely related with PaO2, FVC, FEV1,and FEF2575%.

  21. 2012 Summer Olympics

  22. Multipl breath inert gas washout (MBW) test- Lung clearance index (LCI) reflects changes better. LCI: PCD %85 abnormal Normal FEV1 %81 abnormal LCI is better correlated with other LFT parameters.

  23. MR vs. HRCT

  24. Swallowing and Endoscopy 66% BE (51% <2 yrs) Severe norological sequelae (OR 9.45, P<0.004) History of GER (OR 3.36, P=0.036)

  25. 77 (%68) +’ve BAL culture 49.5% Infection %63 >1 pathogen Age of diagnosis is important.

  26. BE Infection Hare KM, Int J Antimicrob Agents. 2012;40:365. Long-term effects of antibiotics on nasopharyngeal carrier status ? AZT and β-lactam ab, 104 pts Makrolide …Nazopharyngeal carrier status decreases OR=0.2, (0.1-0.6) β-lactam..OR=0.1, (0.1-0.3) No effect on LRTI! Rate of AB resistant m.o. increases !

  27. BE Infection Hare KM, Int J Antimicrob Agents. 2012; 40:365. Makrolide: Resistant S.pneumonia carrier: OR=4.5 (1.1-21.7) Infection: OR=8.1, (1.4-81.3) β-lactam ab: Resistant H.influenza infection:OR=4.4 (0.8-23.9) Long-term risks ?

  28. Neutrophilia in BAL is correlated with culture but not with PCR

  29. BE Disease Burden Kapur N, Chest 2012 Apr;141:1018-24. Disease burden ? QoL ve parental mental health and the effects of exacerbations Parent-proxy cough-specific quality of life [PC-QOL] Depression, Anxiety, and Stress Scale [DASS])

  30. BE Disease burden Kapur N, Chest 2012 Apr;141:1018-24. Stable state: PC-QOL score:6.5 (5.3-6.9) DASS 21 score: 6 (0-20). Smaller age= worse QOL (P = .04) Worsens with exacerbation

  31. BE Disease Burden Kapur N, Chest 2012 Apr;141:1018-24. PC-QOL score (4.6 [3.8-5.4]; p= .001) DASS score ( 22 [9-42]; P < .001) Anxiety 38%, abnormal depression/stress 54% Especially significant during exacerbations. Prevention of BE, early diagnosis and treatment of exacerbation may decrease the psychological morbidity.

  32. Whitney Houston (1963-2012)

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