Published in Eur J Clin Invest on May 01, 2014
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Diagnosis, monitoring, and treatment of primary ciliary dyskinesia: PCD foundation consensus recommendations based on state of the art review. Pediatr Pulmonol (2015) 0.91
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Prevalence of Primary Ciliary Dyskinesia in consecutive referrals of suspect cases and the Transmission Electron Microscopy detection rate: A systematic review and meta-analysis. Pediatr Res (2016) 0.75
Hypovitaminosis D: a novel finding in primary ciliary dyskinesia. Ital J Pediatr (2015) 0.75
Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR. N Engl J Med (2015) 5.51
Characterization of proposed human B-1 cells reveals pre-plasmablast phenotype. Blood (2013) 3.07
Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation. Thorax (2011) 2.84
Lung structure-function correlation in patients with primary ciliary dyskinesia. Thorax (2015) 2.79
Preoperative symptoms of body dysmorphic disorder determine postoperative satisfaction and quality of life in aesthetic rhinoplasty. Plast Reconstr Surg (2013) 2.71
European position paper on endoscopic management of tumours of the nose, paranasal sinuses and skull base. Rhinol Suppl (2010) 2.59
Mutations in the DNAH11 (axonemal heavy chain dynein type 11) gene cause one form of situs inversus totalis and most likely primary ciliary dyskinesia. Proc Natl Acad Sci U S A (2002) 2.21
CCDC39 is required for assembly of inner dynein arms and the dynein regulatory complex and for normal ciliary motility in humans and dogs. Nat Genet (2010) 2.12
Amphotericin B nasal lavages: not a solution for patients with chronic rhinosinusitis. J Allergy Clin Immunol (2006) 1.80
Mutations in the amiloride-sensitive epithelial sodium channel in patients with cystic fibrosis-like disease. Hum Mutat (2009) 1.71
Long-term inhaled dry powder mannitol in cystic fibrosis: an international randomized study. Am J Respir Crit Care Med (2011) 1.69
European position paper on the anatomical terminology of the internal nose and paranasal sinuses. Rhinol Suppl (2014) 1.63
Coexistence of (partial) immune defects and risk of recurrent respiratory infections. Clin Chem (2006) 1.60
High prevalence of body dysmorphic disorder symptoms in patients seeking rhinoplasty. Plast Reconstr Surg (2011) 1.58
Coprescription of antibiotics and asthma drugs in children. Pediatrics (2011) 1.56
The effect of topical amphotericin B on inflammatory markers in patients with chronic rhinosinusitis: a multicenter randomized controlled study. Laryngoscope (2009) 1.46
Noncirrhotic presinusoidal portal hypertension is common in cystic fibrosis-associated liver disease. Hepatology (2011) 1.43
X-linked lymphoproliferative disease due to SAP/SH2D1A deficiency: a multicenter study on the manifestations, management and outcome of the disease. Blood (2010) 1.40
Laboratory diagnosis of specific antibody deficiency to pneumococcal capsular polysaccharide antigens by multiplexed bead assay. Clin Immunol (2009) 1.32
Mutations in CCDC39 and CCDC40 are the major cause of primary ciliary dyskinesia with axonemal disorganization and absent inner dynein arms. Hum Mutat (2013) 1.31
Malignancies of the nasal cavity and paranasal sinuses: long-term outcome with conventional or three-dimensional conformal radiotherapy. Int J Radiat Oncol Biol Phys (2007) 1.19
Intensity-modulated radiotherapy for sinonasal cancer: improved outcome compared to conventional radiotherapy. Int J Radiat Oncol Biol Phys (2010) 1.19
Laboratory diagnosis of specific antibody deficiency to pneumococcal capsular polysaccharide antigens. Clin Chem (2007) 1.17
Human memory B lymphocyte subsets fulfill distinct roles in the anti-polysaccharide and anti-protein immune response. J Immunol (2008) 1.14
Primary ciliary dyskinesia, an orphan disease. Eur J Pediatr (2012) 1.14
Olmsted syndrome: exploration of the immunological phenotype. Orphanet J Rare Dis (2013) 1.11
Is the sensitivity of primary ciliary dyskinesia detection by ciliary function analysis 100%? Eur Respir J (2013) 1.09
Mutant ADA2 in vasculopathies. N Engl J Med (2014) 1.08
Elevated expression of both mRNA and protein levels of IL-17A in sputum of stable Cystic Fibrosis patients. Respir Res (2010) 1.07
Clericuzio type poikiloderma with neutropenia is distinct from Rothmund-Thomson syndrome. Am J Med Genet A (2005) 1.05
Dysbiosis of bifidobacteria and Clostridium cluster XIVa in the cystic fibrosis fecal microbiota. J Cyst Fibros (2012) 1.04
Primary ciliary dyskinesia: critical evaluation of clinical symptoms and diagnosis in patients with normal and abnormal ultrastructure. Orphanet J Rare Dis (2014) 1.03
Cross-sectional and longitudinal comparisons of the predominant fecal microbiota compositions of a group of pediatric patients with cystic fibrosis and their healthy siblings. Appl Environ Microbiol (2011) 1.01
Innate and adaptive interleukin-17-producing lymphocytes in chronic inflammatory lung disorders. Am J Respir Crit Care Med (2010) 1.01
IL-12 contributes to allergen-induced airway inflammation in experimental asthma. J Immunol (2006) 1.01
What's new in cystic fibrosis? From treating symptoms to correction of the basic defect. Eur J Pediatr (2008) 0.99
Vallecular cyst as a cause of congenital stridor: report of five patients. Pediatr Radiol (2009) 0.97
Lung clearance index predicts pulmonary exacerbations in young patients with cystic fibrosis. Thorax (2013) 0.96
Human CD20+CD43+CD27+CD5- B cells generate antibodies to capsular polysaccharides of Streptococcus pneumoniae. J Allergy Clin Immunol (2012) 0.94
Ciliary beating recovery in deficient human airway epithelial cells after lentivirus ex vivo gene therapy. PLoS Genet (2009) 0.93
The importance of local eosinophilia in the surgical outcome of chronic rhinosinusitis: a 3-year prospective observational study. Am J Rhinol Allergy (2014) 0.93
Non-invasive liver elastography (Fibroscan) for detection of cystic fibrosis-associated liver disease. J Cyst Fibros (2009) 0.92
Quality assessment of expert answers to lay questions about cystic fibrosis from various language zones in Europe: the ECORN-CF project. BMC Med Res Methodol (2012) 0.90
Pooled analysis of two large randomised phase III inhaled mannitol studies in cystic fibrosis. J Cyst Fibros (2012) 0.90
Localization of candidate regions for a novel gene for Kartagener syndrome. Eur J Hum Genet (2006) 0.89
Distribution of human beta-defensin polymorphisms in various control and cystic fibrosis populations. Genomics (2005) 0.89
Diagnosis of autoimmune lymphoproliferative syndrome caused by FAS deficiency in adults. Haematologica (2012) 0.89
Granulomatous skin lesions complicating Varicella infection in a patient with Rothmund-Thomson syndrome and immune deficiency: case report. Orphanet J Rare Dis (2010) 0.89
Toll-like receptor 2 and Toll-like receptor 4 polymorphisms in invasive pneumococcal disease. Microbes Infect (2006) 0.89
Impact of air pollution on cystic fibrosis pulmonary exacerbations: a case-crossover analysis. Chest (2013) 0.89
A randomized controlled trial of mometasone furoate nasal spray for the treatment of nasal polyposis. Arch Otolaryngol Head Neck Surg (2006) 0.88
Exhaled nitric oxide corresponds with office evaluation of asthma control. Pediatr Pulmonol (2003) 0.88
Age- and serotype-dependent antibody response to pneumococcal polysaccharides. J Allergy Clin Immunol (2011) 0.87
Clinical practice: treatment of childhood empyema. Eur J Pediatr (2009) 0.87
Laparoscopic Nissen fundoplication in infants and children: analysis of 106 consecutive patients with special emphasis in neurologically impaired vs. neurologically normal patients. Surg Endosc (2007) 0.87
Screening tools for body dysmorphic disorder in a cosmetic surgery setting. Laryngoscope (2011) 0.86
A novel strategy for the isolation and identification of environmental Burkholderia cepacia complex bacteria. FEMS Microbiol Lett (2005) 0.86
Series of extranodal natural killer/T-cell lymphoma, nasal type, with periorbital involvement. Orbit (2014) 0.86
Real life evaluation of intravenous antibiotic treatment in a paediatric cystic fibrosis centre: outcome of home therapy is not inferior. Respir Med (2008) 0.86
High-speed digital imaging method for ciliary beat frequency measurement. J Pharm Pharmacol (2005) 0.86
Prevalence and determinants of allergic diseases in a Congolese population. Int Forum Allergy Rhinol (2012) 0.85
Selective nasal allergen provocation induces substance P-mediated bronchial hyperresponsiveness. Am J Respir Cell Mol Biol (2010) 0.85
Omeprazole, a proton pump inhibitor, improves residual steatorrhoea in cystic fibrosis patients treated with high dose pancreatic enzymes. Eur J Pediatr (2003) 0.85
Tidal off-line exhaled nitric oxide measurements in a pre-school population. Eur J Pediatr (2003) 0.85
Fcgamma-receptor IIA genotype and invasive pneumococcal infection. Clin Immunol (2005) 0.84
Management of macrolide-resistant Mycoplasma pneumoniae infection. Pediatr Infect Dis J (2012) 0.84
Budesonide/formoterol improves lung function compared with budesonide alone in children with asthma. Pediatr Allergy Immunol (2006) 0.84
Long-term follow-up of 44 patients with adenocarcinoma of the nasal cavity and sinuses primarily treated with endoscopic resection followed by radiotherapy. Head Neck (2010) 0.84
Complicated parapneumonic effusion in Belgian children: increased occurrence before routine pneumococcal vaccine implementation. Eur J Pediatr (2008) 0.82
IL-10- and IL-12-independent down-regulation of allergic sensitization by stimulation of CD40 signaling. J Immunol (2006) 0.82
Pain in CF: review of the literature. J Cyst Fibros (2013) 0.80
In vitro polarized transport of L-phenylalanine in human nasal epithelium and partial characterization of the amino acid transporters involved. Pharm Res (2003) 0.80
Nasal corticosteroid treatment reduces substance P levels in tear fluid in allergic rhinoconjunctivitis. Ann Allergy Asthma Immunol (2012) 0.80
Do inhaled corticosteroids impair long-term growth in prepubertal cystic fibrosis patients? Eur J Pediatr (2006) 0.79
Post-operative intensity-modulated radiotherapy for malignancies of the nasal cavity and paranasal sinuses. Radiother Oncol (2007) 0.79
Hyper-immunoglobulin M syndrome caused by a mutation in the promotor for CD40L. Immunology (2007) 0.79
Macrolide therapy targets a specific phenotype in respiratory medicine: from clinical experience to basic science and back. Inflamm Allergy Drug Targets (2008) 0.79
Airway clearance techniques to treat acute respiratory disorders in previously healthy children: where is the evidence? Eur J Pediatr (2008) 0.78
Beneficial effect of antibiotics on ciliary beat frequency of human nasal epithelial cells exposed to bacterial toxins. J Pharm Pharmacol (2008) 0.78
Granulomatous inflammation in cartilage-hair hypoplasia: risks and benefits of anti-TNF-α mAbs. J Allergy Clin Immunol (2011) 0.78
Misdiagnosis as asphyxiating thoracic dystrophy and CMV-associated haemophagocytic lymphohistiocytosis in Shwachman-Diamond syndrome. Eur J Pediatr (2013) 0.78
Determination of IgG subclasses: a need for standardization. J Allergy Clin Immunol (2005) 0.78