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Primary Ciliary Dyskinesia: First Health-related Quality-of-Life Measures for Pediatric Patients.
Dell SD, Leigh MW, Lucas JS, Ferkol TW, Knowles MR, Alpern A, Behan L, Morris AM, Hogg C, DunnGalvin A, Quittner AL. Dell SD, et al. Ann Am Thorac Soc. 2016 Oct;13(10):1726-1735. doi: 10.1513/AnnalsATS.201603-198OC. Ann Am Thorac Soc. 2016. PMID: 27464304 Free PMC article.
RATIONALE: Primary ciliary dyskinesia (PCD) is a rare disease. There are no available data on disease-specific pediatric patient-reported outcomes. ...Psychometric testing is underway to determine their measurement properties for evaluating clinical intervent …
RATIONALE: Primary ciliary dyskinesia (PCD) is a rare disease. There are no available data on disease-specific pediatri …
Variations in infection control practices suggest a need for guidelines in primary ciliary dyskinesia patient care.
Caliskan AB, Horani A, Manion M, Brody SL. Caliskan AB, et al. Pediatr Pulmonol. 2022 Apr;57(4):1072-1075. doi: 10.1002/ppul.25836. Epub 2022 Jan 28. Pediatr Pulmonol. 2022. PMID: 35043594
Primary ciliary dyskinesis (PCD) is an autosomal recessive disorder associated with impaired mucociliary clearance caused by defects in ciliary structure and function. ...After COVID-19 mandates are lifted, 48.1% of centers plan to continue to use masks durin …
Primary ciliary dyskinesis (PCD) is an autosomal recessive disorder associated with impaired mucociliary clearance caused by d …
A systematic review of lung clearance index in non-cystic fibrosis, non-primary ciliary dyskinesia bronchiectasis.
Hine C, Desai M, Davies J, Sapey E, Nagakumar P. Hine C, et al. Respir Med. 2022 Sep;201:106937. doi: 10.1016/j.rmed.2022.106937. Epub 2022 Jul 21. Respir Med. 2022. PMID: 35926429
BACKGROUND: Non cystic fibrosis, non primary ciliary dyskinesia bronchiectasis (nCFnPCD-BE) results in significant morbidity with few evidence-based treatments. ...LCI detected disease in adults, differentiating bronchiectasis from controls (AUC 0.90 to 0.96) …
BACKGROUND: Non cystic fibrosis, non primary ciliary dyskinesia bronchiectasis (nCFnPCD-BE) results in significant morb …
Accuracy of Nasal Nitric Oxide Measurement as a Diagnostic Test for Primary Ciliary Dyskinesia. A Systematic Review and Meta-analysis.
Shapiro AJ, Josephson M, Rosenfeld M, Yilmaz O, Davis SD, Polineni D, Guadagno E, Leigh MW, Lavergne V. Shapiro AJ, et al. Ann Am Thorac Soc. 2017 Jul;14(7):1184-1196. doi: 10.1513/AnnalsATS.201701-062SR. Ann Am Thorac Soc. 2017. PMID: 28481653 Free PMC article. Review.
RATIONALE: Primary ciliary dyskinesia (PCD) is a rare disorder causing chronic otosinopulmonary disease, generally diagnosed through evaluation of respiratory ciliary ultrastructure and/or genetic testing. ...OBJECTIVES: To perform a systematic review …
RATIONALE: Primary ciliary dyskinesia (PCD) is a rare disorder causing chronic otosinopulmonary disease, generally diag …
Using electrical impedance tomography to characterize lung impairment of children with primary ciliary dyskinesia: A pilot cross-sectional study.
Pensabene M, Gambazza S, Carta F, Rocchi A, Lelii M, Madini B, Hassan V, Piotto M, Patria MF. Pensabene M, et al. Pediatr Pulmonol. 2023 Apr;58(4):1051-1058. doi: 10.1002/ppul.26293. Epub 2022 Dec 29. Pediatr Pulmonol. 2023. PMID: 36571235 Free article.
BACKGROUND: In children with primary ciliary dyskinesia (PCD), measures more sensitive than spirometry are needed to characterize underlying pulmonary impairment. ...Using EIT, the global inhomogeneity (GI(TOT) ) index showed moderate to strong correlation wi …
BACKGROUND: In children with primary ciliary dyskinesia (PCD), measures more sensitive than spirometry are needed to ch …
Exacerbations and Pseudomonas aeruginosa colonization are associated with altered lung structure and function in primary ciliary dyskinesia.
Piatti G, De Santi MM, Farolfi A, Zuccotti GV, D'Auria E, Patria MF, Torretta S, Consonni D, Ambrosetti U. Piatti G, et al. BMC Pediatr. 2020 Apr 13;20(1):158. doi: 10.1186/s12887-020-02062-4. BMC Pediatr. 2020. PMID: 32284045 Free PMC article.
BACKGROUND: Recurrent bacterial infections of the respiratory tract are one of the major clinical features of the primary ciliary dyskinesia (PCD), a rare genetic disease due to malfunctioning of motile cilia. ...METHODS: We retrospectively analyzed data from …
BACKGROUND: Recurrent bacterial infections of the respiratory tract are one of the major clinical features of the primary ciliary …
Meckel-Gruber Syndrome: a population-based study on prevalence, prenatal diagnosis, clinical features, and survival in Europe.
Barisic I, Boban L, Loane M, Garne E, Wellesley D, Calzolari E, Dolk H, Addor MC, Bergman JE, Braz P, Draper ES, Haeusler M, Khoshnood B, Klungsoyr K, Pierini A, Queisser-Luft A, Rankin J, Rissmann A, Verellen-Dumoulin C. Barisic I, et al. Eur J Hum Genet. 2015 Jun;23(6):746-52. doi: 10.1038/ejhg.2014.174. Epub 2014 Sep 3. Eur J Hum Genet. 2015. PMID: 25182137 Free PMC article.
In addition to cystic kidneys (97.7%), encephalocele (83.8%) and polydactyly (87.3%), frequent features include other central nervous system anomalies (51.4%), fibrotic/cystic changes of the liver (65.5% of cases with post mortem examination) and orofacial clefts (31.8%). Various …
In addition to cystic kidneys (97.7%), encephalocele (83.8%) and polydactyly (87.3%), frequent features include other central nervous system …
Systematic Analysis of CCNO Variants in a Defined Population: Implications for Clinical Phenotype and Differential Diagnosis.
Amirav I, Wallmeier J, Loges NT, Menchen T, Pennekamp P, Mussaffi H, Abitbul R, Avital A, Bentur L, Dougherty GW, Nael E, Lavie M, Olbrich H, Werner C, Kintner C, Omran H; Israeli PCD Consortium Investigators. Amirav I, et al. Hum Mutat. 2016 Apr;37(4):396-405. doi: 10.1002/humu.22957. Epub 2016 Feb 4. Hum Mutat. 2016. PMID: 26777464
Reduced generation of multiple motile cilia (RGMC) is a novel chronic destructive airway disease within the group of mucociliary clearance disorders with only few cases reported. Mutations in two genes, CCNO and MCIDAS, have been identified as a cause of this disease, both …
Reduced generation of multiple motile cilia (RGMC) is a novel chronic destructive airway disease within the group of mucociliary clearance …
Multicenter analysis of body mass index, lung function, and sputum microbiology in primary ciliary dyskinesia.
Maglione M, Bush A, Nielsen KG, Hogg C, Montella S, Marthin JK, Di Giorgio A, Santamaria F. Maglione M, et al. Pediatr Pulmonol. 2014 Dec;49(12):1243-50. doi: 10.1002/ppul.22984. Epub 2014 Jan 13. Pediatr Pulmonol. 2014. PMID: 24420929
BACKGROUND: No studies longitudinally, simultaneously assessed body mass index (BMI) and spirometry in primary ciliary dyskinesia (PCD). METHODS: We determined BMI and spirometry in 158 PCD children and adolescents from London, UK (n = 75), Naples, Italy (n = …
BACKGROUND: No studies longitudinally, simultaneously assessed body mass index (BMI) and spirometry in primary ciliary dysk …
Magnetic Resonance Imaging of Pulmonary and Paranasal Sinus Abnormalities in Children with Primary Ciliary Dyskinesia Compared to Children with Cystic Fibrosis.
Wucherpfennig L, Wuennemann F, Eichinger M, Schmitt N, Seitz A, Baumann I, Roehmel JF, Stahl M, Hämmerling S, Chung J, Schenk JP, Alrajab A, Kauczor HU, Mall MA, Wielpütz MO, Sommerburg O. Wucherpfennig L, et al. Ann Am Thorac Soc. 2024 Mar;21(3):438-448. doi: 10.1513/AnnalsATS.202305-453OC. Ann Am Thorac Soc. 2024. PMID: 38206973
Rationale: Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are characterized by inherited impaired mucociliary clearance leading to chronic progressive lung disease as well as chronic rhinosinusitis (CRS). ...CRS-MRI sum score and mucopyocele subsco …
Rationale: Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are characterized by inherited impaired mucociliar …