Claus Pfannenstiel

Impact of elexacaftor/tezacaftor/ivacaftor on lung function, nutritional status, pulmonary exacerbation frequency and sweat chloride in people with cystic fibrosis: real-world evidence from the German CF Registry

Sivagurunathan Sutharsan, Stefanie Dillenhoefer, Matthias Welsner, Florian Stehling et autres

Treatment with elexacaftor/tezacaftor/ivacaftor (ETI) improves multiple clinical outcomes in people with cystic fibrosis (pwCF) with at least one F508del allele. This study evaluated the real-world impact of ETI on lung function, nutritional status, pulmonary exacerbation frequency, and sweat chloride concentrations in a …

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Ana o 3-specific IgE is a good predictor for clinically relevant cashew allergy in children

Lars Lange, L. Lasota, Antje Finger, Dejan Vlajnic et autres

INTRODUCTION: Component-resolved diagnostics using specific IgE to 2 S albumins has shown to be a valuable new option in diagnostic procedure. Ana o 3 is a 2 S albumin from cashew. The aim of this study was to investigate the role of …

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Genotype alone does not predict the clinical course ofSFTPCdeficiency in paediatric patients

Carolin I. Kröner, Simone Reu, Veronika I. Teusch, Andrea Schams et autres

Patients with interstitial lung disease due to surfactant protein C (SFTPC) mutations are rare and not well characterised. We report on all subjects collected over a 15-year period in the kids-lung register with interstitial lung disease and a proven SFTPC mutation. We …

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Omalizumab: a new treatment option for allergic bronchopulmonary aspergillosis in patients with cystic fibrosis

Sylvia Lehmann, Claus Pfannenstiel, Frank Friedrichs, Kristina Kröger et autres

BACKGROUND: Allergic bronchopulmonary aspergillosis (ABPA) is a severe complication in patients with cystic fibrosis (CF), resulting in deterioration of lung function and impairment of overall prognosis. Standard therapy consists of high dosage, long-term corticosteroid treatment. This carries the risk of serious side …

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Nutzen und Anwendung für den Pädiater

Claus Pfannenstiel, Frank Friedrichs

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Old and novel surfactant protein C (SP-C) mutations in children

Ann-Christin Grimmelt, Michael G. Barker, Frank E. Brasch, Monika Gappa et autres

Genetic variations of SP-C gene are known to cause interstitial lung disease. In previous studies 55 patients have been described focussing on the genetic abnormalities and clinical course. Here we report the data of children with SP-C mutations collected between 1998 and …

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Praxis der oralen Nahrungsmittelprovokation im deutschsprachigen Raum

Lars Lange, Isidor Huttegger, Armin Grübl, Claus Pfannenstiel et autres

Prevalence of thrombophilia and catheter-related thrombosis in cystic fibrosis

Michael G. Barker, Daniel Thoenes, Hiltrud Döhmen, Frank Friedrichs et autres

Venous thrombosis in children and young adults is frequently associated with predisposing conditions and with an indwelling catheter or totally implantable venous access device (TIVAD). These systems are commonly used for the delivery of antibiotic therapy in patients with cystic fibrosis (CF). …

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Volles Programm Fortbildung

Claus Pfannenstiel

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Effect of DNase on exercise capacity in cystic fibrosis

Michael G. Barker, Egbert Franke, Martin Böhle, Claus Pfannenstiel et autres

DNase can reduce viscosity and facilitate expectoration of airway secretions in cystic fibrosis (CF) lung disease. We evaluated its effect on exercise performance in relation to resting pulmonary function. Fifteen sputum-producing CF patients (aged 9-28 years; FEV1 22-83% predicted) performed spirometry, body …

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Aspirin intolerance in boy with primary ciliary dyskinesia

H. Brost, Hagen Ott, Claus Pfannenstiel, T. G. Wenzl et autres

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Interventionelle Radiologie

Rolf W. Günther, Dierk Vorwerk, Claus Pfannenstiel