Pediatric Year in Review

PEDIATRIC YEAR IN REVIEW 14 4. Persistent pseudomonas infection rates are relatively low in a young PCD cohort, yet these do increase with age, and reasons for this difference from cystic fibrosis cohorts require comparative study of cough clearance, mucus rheology, and innate immune defenses. OTHER ARTICLES OF INTEREST Primary Ciliary Dyskinesia: Diagnosis Bustamante-Marin XM, Yin WN, Sears PR, Werner ME, Brotslaw EJ, Mitchell BJ, Jania CM, Zeman KL, Rogers TD, Herring LE, Refabért L, Thomas L, Amselem S, Escudier E, Legendre M, Grubb BR, Knowles MR, Zariwala MA, Ostrowski LE. Lack of GAS2L2 Causes PCD by Impairing Cilia Orientation and Mucociliary Clearance. Am J Hum Genet. 2019 Feb 7;104(2):229-245. doi: 10.1016/j. ajhg.2018.12.009. Epub 2019 Jan 18. Paff T, Kooi IE, Moutaouakil Y, Riesebos E, Sistermans EA, Daniels HJMA, Weiss JMM, Niessen HHWM, Haarman EG, Pals G, Micha D. Diagnostic yield of a targeted gene panel in primary ciliary dyskinesia patients. Hum Mutat. 2018 May;39(5):653-665. doi: 10.1002/humu.23403. Epub 2018 Mar 9. PMID: 29363216 Primary Ciliary Dyskinesia: Phenotype Best S, Shoemark A, Rubbo B, Patel MP, Fassad MR, Dixon M, Rogers AV, Hirst RA, Rutman A, Ollosson S, Jackson CL, Goggin P, Thomas S, Pengelly R, Cullup T, Pissaridou E, Hayward J, Onoufriadis A, O’Callaghan C, Loebinger MR, Wilson R, Chung EM, Kenia P, Doughty VL, Carvalho JS, Lucas JS, Mitchison HM, Hogg C. Risk factors for situs defects and congenital heart disease in primary ciliary dyskinesia. Thorax. 2019 Feb;74(2):203-205. doi: 10.1136/thoraxjnl-2018-212104. Epub 2018 Aug 30. PMID: 30166424 Primary Ciliary Dyskinesia: Radiology Tadd K, Morgan L, Rosenow T, Schultz A, Susanto C, Murray C, Robinson P. CF derived scoring systems do not fully describe the range of structural changes seen on CT scans in PCD. Pediatr Pulmonol. 2019 Jan 21. doi: 10.1002/ppul.24249. [Epub ahead of print] Nyilas S, Bauman G, Pusterla O, Sommer G, Singer F, Stranzinger E, Heyer C, Ramsey K, Schlegtendal A, Benzrath S, Casaulta C, Goutaki M, Kuehni CE, Bieri O, Koerner-Rettberg C, Latzin P. Structural and Functional Lung Impairment in Primary Ciliary Dyskinesia. Assessment with Magnetic Resonance Imaging and Multiple Breath Washout in Comparison to Spirometry. Ann Am Thorac Soc. 2018 Dec;15(12):1434-1442. doi: 10.1513/AnnalsATS.201712-967OC. PMID: 30290127 Non-CF Bronchiectasis: Review El Boustany P, Gachelin E, Colomban C, Cernoia J, Sudour P, Carsin A, Dubus JC. A review of non-cystic fibrosis bronchiectasis in children with a focus on the role of long-term treatment with macrolides. Pediatr Pulmonol. 2019 Jan 16. doi: 10.1002/ppul.24252. [Epub ahead of print] Review. PMID: 30652424 Non-CF Bronchiectasis: Physical Activity Joschtel B, Gomersall SR, Tweedy S, Petsky H, Chang AB, Trost SG. Objectively measured physical activity and sedentary behaviour in children with bronchiectasis: a cross-sectional study. BMC Pulm Med. 2019 Jan 8;19(1):7. doi: 10.1186/s12890-018-0772-8 Non-CF Bronchiectasis: Preventative Vaccination O’Grady KF, Chang AB, Cripps A, Mulholland EK, Smith- Vaughan H, Wood N, Danchin M, Thornton R, Wilson A, Torzillo PJ, Morris PM, Richmond P, Rablin S, Arnold D, Connor A, Goyal V, Stoney T, Perrett K, Grimwood K. The clinical, immunological and microbiological impact of the 10-valent pneumococcal-Protein D conjugate vaccine in children with recurrent protracted bacterial bronchitis, chronic suppurative lung disease and bronchiectasis: A multi-centre, double-blind, randomised controlled trial. Hum Vaccin Immunother. 2018 Jun 26:1-12. doi: 10.1080/21645515.2018.1488562. [Epub ahead of print] UPDATE ON PCD AND NON-CF BRONCHIECTASIS