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Nasal Nitric Oxide in Primary Immunodeficiency and Primary Ciliary Dyskinesia: Helping to Distinguish Between Clinically Similar Diseases.
Zysman-Colman ZN, Kaspy KR, Alizadehfar R, NyKamp KR, Zariwala MA, Knowles MR, Vinh DC, Shapiro AJ. Zysman-Colman ZN, et al. Among authors: knowles mr. J Clin Immunol. 2019 Feb;39(2):216-224. doi: 10.1007/s10875-019-00613-8. Epub 2019 Mar 26. J Clin Immunol. 2019. PMID: 30911954 Free PMC article.
Participants underwent nNO testing by standardized protocol using a chemiluminescence analyzer and completed a questionnaire concerning their chronic oto-sino-pulmonary symptoms, including key clinical criteria specific to diagnosed PCD (neonatal respiratory distress at term …
Participants underwent nNO testing by standardized protocol using a chemiluminescence analyzer and completed a questionnaire concerning thei …
Diagnosis, monitoring, and treatment of primary ciliary dyskinesia: PCD foundation consensus recommendations based on state of the art review.
Shapiro AJ, Zariwala MA, Ferkol T, Davis SD, Sagel SD, Dell SD, Rosenfeld M, Olivier KN, Milla C, Daniel SJ, Kimple AJ, Manion M, Knowles MR, Leigh MW; Genetic Disorders of Mucociliary Clearance Consortium. Shapiro AJ, et al. Among authors: knowles mr. Pediatr Pulmonol. 2016 Feb;51(2):115-32. doi: 10.1002/ppul.23304. Epub 2015 Sep 29. Pediatr Pulmonol. 2016. PMID: 26418604 Free PMC article. Review.
These recommendations have been adopted by the governing board of the PCD Foundation to provide guidance for PCD clinical centers for diagnostic testing, monitoring, and appropriate short and long-term therapeutics in PCD patients....
These recommendations have been adopted by the governing board of the PCD Foundation to provide guidance for PCD clinical centers for diagno …
Founder mutation in RSPH4A identified in patients of Hispanic descent with primary ciliary dyskinesia.
Daniels ML, Leigh MW, Davis SD, Armstrong MC, Carson JL, Hazucha M, Dell SD, Eriksson M, Collins FS, Knowles MR, Zariwala MA. Daniels ML, et al. Among authors: knowles mr. Hum Mutat. 2013 Oct;34(10):1352-6. doi: 10.1002/humu.22371. Epub 2013 Aug 6. Hum Mutat. 2013. PMID: 23798057 Free PMC article.
Primary ciliary dyskinesia (PCD) is a rare, autosomal recessive, genetically heterogeneous disorder characterized by ciliary dysfunction resulting in chronic oto-sino-pulmonary disease, respiratory distress in term neonates, laterality (situs) defects, and bronchiectasis. …
Primary ciliary dyskinesia (PCD) is a rare, autosomal recessive, genetically heterogeneous disorder characterized by ciliary dysfunction res …
Genetic causes of bronchiectasis: primary ciliary dyskinesia.
Morillas HN, Zariwala M, Knowles MR. Morillas HN, et al. Among authors: knowles mr. Respiration. 2007;74(3):252-63. doi: 10.1159/000101783. Respiration. 2007. PMID: 17534128 Review.
Primary ciliary dyskinesia (PCD) is a genetically heterogeneous disorder reflecting abnormalities in the structure and function of motile cilia and flagella, causing impairment of mucociliary clearance, left-right body asymmetry, and sperm motility. Clinical manifestations includ …
Primary ciliary dyskinesia (PCD) is a genetically heterogeneous disorder reflecting abnormalities in the structure and function of motile ci …
Mucus clearance and lung function in cystic fibrosis with hypertonic saline.
Donaldson SH, Bennett WD, Zeman KL, Knowles MR, Tarran R, Boucher RC. Donaldson SH, et al. Among authors: knowles mr. N Engl J Med. 2006 Jan 19;354(3):241-50. doi: 10.1056/NEJMoa043891. N Engl J Med. 2006. PMID: 16421365 Free article. Clinical Trial.
Mucus clearance and lung function were measured during 14-day baseline and treatment periods. RESULTS: Long-term inhalation of hypertonic saline without pretreatment with amiloride (i.e., with placebo pretreatment) resulted in a sustained (> or =8 hours) increase in 1-h …
Mucus clearance and lung function were measured during 14-day baseline and treatment periods. RESULTS: Long-term inhalation of hypert …
Nontuberculous mycobacteria. II: nested-cohort study of impact on cystic fibrosis lung disease.
Olivier KN, Weber DJ, Lee JH, Handler A, Tudor G, Molina PL, Tomashefski J, Knowles MR; Nontuberculous Mycobacteria in Cystic Fibrosis Study Group. Olivier KN, et al. Among authors: knowles mr. Am J Respir Crit Care Med. 2003 Mar 15;167(6):835-40. doi: 10.1164/rccm.200207-679OC. Epub 2002 Nov 14. Am J Respir Crit Care Med. 2003. PMID: 12433669
All subjects with three or more positive cultures and exit HRCTs (n = 6) showed progression of HRCT findings, whereas only 17% of subjects with two positive cultures or less had progression (p = 0.0006). In summary, no significant short-term effect on FEV1 was detected in …
All subjects with three or more positive cultures and exit HRCTs (n = 6) showed progression of HRCT findings, whereas only 17% of subjects w …
Pharmacologic modulation of salt and water in the airway epithelium in cystic fibrosis.
Knowles MR, Olivier K, Noone P, Boucher RC. Knowles MR, et al. Am J Respir Crit Care Med. 1995 Mar;151(3 Pt 2):S65-9. doi: 10.1164/ajrccm/151.3_Pt_2.S65. Am J Respir Crit Care Med. 1995. PMID: 7533608 Review.
Phase III studies of amiloride in adults and adolescents are underway, and short-term safety studies in children are under way. Aerosolized uridine triphosphate (UTP) induces Cl- secretion in CF airway epithelia via non-CFTR Cl- channels. ...
Phase III studies of amiloride in adults and adolescents are underway, and short-term safety studies in children are under way. Aeros …
Pharmacologic treatment of abnormal ion transport in the airway epithelium in cystic fibrosis.
Knowles MR, Olivier KN, Hohneker KW, Robinson J, Bennett WD, Boucher RC. Knowles MR, et al. Chest. 1995 Feb;107(2 Suppl):71S-76S. doi: 10.1378/chest.107.2_supplement.71s. Chest. 1995. PMID: 7842817 Review.
Clinical trials of amiloride are currently underway in adults and adolescents, and short-term safety studies have been initiated in children. Aerosolized uridine triphosphate (UTP) induces Cl- (and liquid) secretion in CF airway epithelia via non-CFTR Cl- channels. Short- …
Clinical trials of amiloride are currently underway in adults and adolescents, and short-term safety studies have been initiated in c …
Electrical potential difference and ion transport across nasal epithelium of term neonates: correlation with mode of delivery, transient tachypnea of the newborn, and respiratory rate.
Gowen CW Jr, Lawson EE, Gingras J, Boucher RC, Gatzy JT, Knowles MR. Gowen CW Jr, et al. Among authors: knowles mr. J Pediatr. 1988 Jul;113(1 Pt 1):121-7. doi: 10.1016/s0022-3476(88)80545-6. J Pediatr. 1988. PMID: 3385520
We studied the change in ion transport function by measuring the basal transepithelial potential difference (PD) across the ciliated epithelium of the nose in 85 term neonates during the first 72 hours of life. Differences in PD associated with the mode of delivery or the …
We studied the change in ion transport function by measuring the basal transepithelial potential difference (PD) across the ciliated epithel …
12 results