Affiliation: Hannover Medical School
- Hypertonic saline and recombinant human DNase: a randomised cross-over pilot study in patients with cystic fibrosisManfred Ballmann
Medical School Hannover Paediatric Department, Carl Neubergstr 1, D 30625 Hannover, Germany
J Cyst Fibros 1:35-7. 2002
- Sequential analysis of surfactant, lung function and inflammation in cystic fibrosis patientsMatthias Griese
Children s Hospital, University of Munich, Lindwurmstr 4, 80337 München, Germany
Respir Res 6:133. 2005..So far, longitudinal measurements of surfactant function in CF patients are lacking and it remains unclear how these alterations relate to the progression of airway inflammation as well as decline in pulmonary function over time...
- Low-dose methotrexate for advanced pulmonary disease in patients with cystic fibrosisM Ballmann
Paediatric Department, Medical School Hannover, Hannover, Germany
Respir Med 97:498-500. 2003..The question was: Is an improvement in pulmonary function achievable with low-dose MTX in patients with cystic fibrosis and advanced pulmonary disease.?..
- Functional analysis of F508del CFTR in native human colonAndrea van Barneveld
Klinische Forschergruppe, OE 6710, Medizinische Hochschule Hannover, Hannover, Germany
Biochim Biophys Acta 1802:1062-9. 2010..The finding that some F508del CFTR escapes the ER quality control in vivo substantiates the hope that the defective processing and trafficking of F508del CFTR can be corrected by pharmacological agents...
- Intestinal current measurement for diagnostic classification of patients with questionable cystic fibrosis: validation and reference dataNico Derichs
Cystic Fibrosis Centre, Department of Pediatric Pulmonology and Neonatology, Medizinische Hochschule Hannover, Carl Neuberg Str 1, 30625 Hannover, Germany
Thorax 65:594-9. 2010..CF transmembrane conductance regulator (CFTR)-mediated ion transport can be studied ex vivo in rectal biopsies by intestinal current measurement (ICM)...
- Short-term effect of physiotherapy on variability of the lung clearance index in children with cystic fibrosisSusanne I Fuchs
Children s Hospital and Research Institute, Marien Hospital Wesel gGmbH, Wesel, Germany
Pediatr Pulmonol 45:301-6. 2010..Reproducibility was good and independent on intervention. From our data, we conclude that timing of PT in relation to MBW can be ignored when designing study protocols or when interpreting longitudinal data and treatment effects...
- Size reduction of donor organs in pediatric lung transplantationCarsten Mueller
Department of Pediatric Pneumology and Neonatology, Hannover Medical School, Hannover, Germany
Pediatr Transplant 14:364-8. 2010..This technique might help to reduce waiting list mortality by expanding the donor pool in pediatric LTx...
- Lung and heart-lung transplantation in children and adolescents: a long-term single-center experienceHeidi Görler
Department of Cardiothoracic, Hannover Medical School, Germany
J Heart Lung Transplant 28:243-8. 2009..Therefore, we retrospectively analyzed our experience after 53 lung and heart-lung transplantations (HLTx) in children...
- Heart-lung transplantation in a 14-year-old boy with Alström syndromeHeidi Goerler
Division of Cardiac, Thoracic, Transplantation and Vascular Surgery, Hannover Medical School, Hannover, Germany
J Heart Lung Transplant 26:1217-8. 2007..The boy underwent successful heart-lung transplantation. We conclude from this report that heart-lung transplantation in patients with Alström syndrome is a viable therapeutic option in select cases...
- Genes that determine immunology and inflammation modify the basic defect of impaired ion conductance in cystic fibrosis epitheliaFrauke Stanke
Department of Pediatrics, Hannover Medical School, Germany
J Med Genet 48:24-31. 2011..The cystic fibrosis (CF) basic defect, caused by dysfunction of the apical chloride channel CFTR in the gastrointestinal and respiratory tract epithelia, has not been employed so far to support the role of CF modifier genes...
- Ex vivo biochemical analysis of CFTR in human rectal biopsiesAndrea van Barneveld
Klinische Forschergruppe, OE 6711, Medizinische Hochschule Hannover, Carl Neuberg Str 1, D 30623 Hannover, Germany
Biochim Biophys Acta 1762:393-7. 2006..Interestingly, maturation of CFTR increases substantially in tissue incubated at 26 degrees C. Our data provide a solid basis for future studies on the characterisation of CFTR in pathological cases...
- Cystic fibrosis transmembrane conductance regulator (CFTR)-mediated residual chloride secretion does not protect against early chronic Pseudomonas aeruginosa infection in F508del homozygous cystic fibrosis patientsNico Derichs
Medizinische Hochschule Hannover, Department of Pediatrics, Carl Neuberg Str 1, D 30623 Hannover, Germany
Pediatr Res 55:69-75. 2004..These results demonstrate the presence of some active F508del-CFTR in the apical cell membrane and imply that factors other than the CFTR-mediated residual Cl- secretion determine the age of onset of PA colonization...
- CFTR protein analysis of splice site mutation 2789+5 G-AAndrea van Barneveld
Department of Pediatric Pneumology and Neonatology, OE 6710, Medizinische Hochschule Hannover, D30625 Hannover, Germany
J Cyst Fibros 7:165-7. 2008....
- Ex vivo CF diagnosis by intestinal current measurements (ICM) in small aperture, circulating Ussing chambersHugo R de Jonge
Departments of Paediatric Gastroenterology and Biochemistry, Erasmus University Medical Centre, P O Box 1738, Rotterdam, 3000DR The Netherlands
J Cyst Fibros 3:159-63. 2004..ICM is particularly useful for the classification of individuals with CF-like clinical features with equivocal sweat test values and/or no or one identifiable CFTR mutation...
- Basic protocol for transepithelial nasal potential difference measurementsDaniel Schüler
CF Center, Children Hospital of Justus Liebig University, 35385 Giessen, Germany
J Cyst Fibros 3:151-5. 2004..The article summarizes the objectives and the principle of NPD measurements, describes a hands-on protocol of the procedure and provides quality control measures, practical hints and troubleshooting...
- Pulmonary surfactant, lung function, and endobronchial inflammation in cystic fibrosisMatthias Griese
Children s Hospital, University of Munich, Munich, Germany
Am J Respir Crit Care Med 170:1000-5. 2004....
- Increased mortality after pulmonary fungal infection within the first year after pediatric lung transplantationLara A Danziger-Isakov
Department of Pediatric Infectious Diseases, Cleveland Clinic, Cleveland, Ohio 44195, USA
J Heart Lung Transplant 27:655-61. 2008..Risk factors, morbidity and mortality from pulmonary fungal infections (PFIs) within the first year after pediatric lung transplant have not previously been characterized...
- Effect of treatment with dornase alpha on airway inflammation in patients with cystic fibrosisKarl Paul
Department of Pediatric Pneumology and Immunology, Charite, Germany
Am J Respir Crit Care Med 169:719-25. 2004..We conclude that in patients with CF, an increase in neutrophilic airway inflammation is found that is positively influenced by rhDNase treatment...
- Cystic fibrosis disease-specific centiles in 2000 and 2005Frauke Stanke
Am J Respir Crit Care Med 173:1047; author reply 1047. 2006