Joachim Riethmüller's research while affiliated with Universitätsklinikum Tübingen and other places
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Publications (114)
![Kinetics of D9-Choline and D9-Betaine prior to (visit 2–4 [V2–4], white...](publication/331848463/figure/fig3/AS:11431281211635419@1702468146932/Kinetics-of-D9-Choline-and-D9-Betaine-prior-to-visit-2-4-V2-4-white-symbols-and-from_Q320.jpg)
Background: Choline is essential for the synthesis of liver phosphatidylcholine (PC), parenchymal maintenance, bile formation, and lipoprotein assembly to secrete triglycerides. In choline deficiency, the liver accretes choline/PC at the expense of lung tissue, thereby impairing pulmonary PC homoeostasis. In cystic fibrosis (CF), exocrine pancreas...
Background and aims:
Platelets are critically involved in tissue repair and regeneration, which depend on their inflammatory properties and survival. SDF-1 ligates to CXCR4 and CXCR7 and contributes to the regulation of platelet survival. Platelet CXCR4/CXCR7 are involved in myocardial regeneration after infarction and are associated with outcomes...
Studies over the past several years have demonstrated the important role of sphingolipids in cystic fibrosis (CF), chronic obstructive pulmonary disease and acute lung injury. Ceramide is increased in airway epithelial cells and alveolar macrophages of CF mice and humans, while sphingosine is dramatically decreased. This increase in ceramide result...
Pulmonary infections of cystic fibrosis (CF) patients with Staphylococcus aureus (S. aureus) occur very early in the disease. The molecular details that cause infection-susceptibility of CF patients to and mediate infection with S. aureus are poorly characterized. Therefore, we aimed to identify the role of α-toxin, a major S. aureus toxin, for pul...
Being a classic monogenic disease, gene therapy has always been a promising therapeutic approach for Cystic Fibrosis (CF). However, numerous trials using DNA or viral vectors encoding the correct protein resulted in a general low efficacy. In the last years, chemically modified messenger RNA (cmRNA) has been proven to be a highly potent, pulmonary...
Objectives:
To evaluate the applicability of a semiquantitative MRI scoring system (MR-CF-S) as a prognostic marker for clinical course of cystic fibrosis (CF) lung disease.
Methods:
This observational study of a single-centre CF cohort included a group of 61 patients (mean age 12.9 ± 4.7 years) receiving morphological and functional pulmonary M...
Background
Calcineurin inhibitors (CNI) have significantly improved patient and graft survival in pediatric liver transplantation (pLT). However, CNI toxicity leads to significant morbidity. Moreover, CNIs cannot prevent long-term allograft injury. Mesenchymal stem (stromal) cells (MSC) have potent immunomodulatory properties, which may promote all...
Chronic pulmonary colonization with bacterial pathogens, particularly Pseudomonas aeruginosa, is the primary cause of morbidity and mortality in patients with cystic fibrosis (CF). We observed that β1-integrins accumulate on the luminal membrane of upper-airway epithelial cells from mice and humans with CF. β1-integrin accumulation is due to increa...
Introduction:
Inhaled mannitol has beneficial effects on lung function, mucociliary clearance, quality of life and sputum properties. This trial examined the efficacy of inhaled mannitol in children with cystic fibrosis (CF).
Methods:
The efficacy of inhaled mannitol in children with CF aged 6-17years was assessed in a phase 2, randomised, place...
Specific antistaphylococcal IgG levels associated with clinical condition.
(DOCX)
FEV1% predicted and bacterial density in sputa.
(PDF)
Staphylococcus aureus is an important pathogen in cystic fibrosis (CF). However, it is not clear which factors are associated with worse lung function in patients with persistent S. aureus airway cultures. Our main hypothesis was that patients with high S. aureus density in their respiratory specimens would more likely experience worsening of their...
Clinical characteristics of patients at baseline.
(DOCX)
Background:
Individuals with cystic fibrosis (CF) receive antibiotics continuously throughout their entire life which leads to drug resistant microbial lung infections which are difficult to treat. Nitric oxide (NO) gas possesses antimicrobial activity against a wide variety of microorganisms in vitro, in vivo in animal models and a phase I study...
Background/aims:
In cystic fibrosis (CF), chronic microbial lung infections are difficult to treat and cause morbidity and increased mortality.
Methods:
In a multicentre, open-label, exploratory, non-interventional study, inhaled tobramycin (300 mg twice daily) and colistin (1 million I.U. twice daily) were sequentially combined with the aim to...
Background/aims:
Several recent clinical studies revealed an accumulation of ceramide in bronchial epithelial cells of patients with cystic fibrosis (CF). Degradation of ceramide concentrations in lungs of CF patients employing the functional acid sphingomyelinase inhibitor amitriptyline revealed a benefit in lung function, weight and exacerbation...
Background:
Chronic rhinosinusitis is a hallmark of Cystic fibrosis (CF) impairing the patients' quality of life and overall health. However, therapeutic options have not been sufficiently evaluated. Bronchial inhalation of mucolytic substances is a gold standard in CF therapy. Previously, we found that sinonasal inhalation of dornase alfa as vibr...
Background:
Chitinases have recently gained attention in the field of pulmonary diseases, particularly in asthma and chronic obstructive pulmonary disease, but their potential role in patients with cystic fibrosis (CF)-associated lung disease remains unclear.
Objective:
The aim of this study was to assess chitinase activity systemically and in t...
With intensified antibiotic therapy and longer survival, patients with cystic fibrosis (CF) are colonized with a more complex pattern of bacteria and fungi. However, the clinical relevance of these emerging pathogens for lung function remains poorly defined. The aim of this study was to assess the association of bacterial and fungal colonization pa...
Objectives
Pseudomonas aeruginosa in the airways of CF-patients restricts the efficacy of antibiotics leading to chronic infections which have a large impact on morbidity and mortality. Antibiotic combination therapy might be more efficient than single antibiotics to combat lung infections in CF.
Methods
In an observational pilot study CF patients...
Objectives
Chronic rhinosinusitis significantly impairs CF patients’ quality of life. The PariSinus™ device effectively delivers vibrating aerosols to paranasal sinuses. Previously, we demonstrated a significant reduction of sinonasal symptoms by inhalation of dornase alfa with the device (JCF 2014). Bronchially inhaled hypertonic saline (3–7%) was...
Liver impairment, ranging from steatosis to cirrhosis, is frequent in cystic fibrosis (CF) patients and is becoming increasingly significant due to their improved life expectancy. One aspect of hepatic alterations is caused by increased fecal loss of the essential nutrient choline, following enterohepatic bile phosphatidylcholine (PC) cycle impairm...
Patients with cystic fibrosis (CF) lung disease suffer from chronic airway inflammation, driven by dysbalanced T cell (Th17 and Th2) responses. Regulatory T cells (Tregs) dampen T cell activation, yet their role in CF is incompletely understood.
To characterize numbers, function and clinical impact of Tregs in CF lung disease.
Tregs were quantified...
Cystic fibrosis (CF) lung disease is characterised by chronic Pseudomonas aeruginosa infection and leukocyte infiltration. Chemokines recruit leukocytes to sites of infection. Gene expression analysis identified the chemokine CCL18 as upregulated in CF leukocytes. We hypothesised that CCL18 characterises infection and inflammation in patients with...
Background
Chronic rhinosinusitis significantly impairs CF patients' quality of life and overall health. The Pari-Sinus™ device delivers vibrating aerosol effectively to paranasal sinuses. After a small pilot study to assess sinonasal inhalation of dornase alfa and placebo (isotonic saline) on potential sinonasal outcome measures, we present the su...
In cystic fibrosis (CF), the paranasal sinuses are sites of first and persistent colonization by pathogens such as Pseudomonas aeruginosa. Pathogens subsequently descend to the lower airways, with P. aeruginosa remaining the primary cause of premature death in patients with the inherited disease. Unlike conventional aerosols, vibrating aerosols app...
Cystic fibrosis (CF), the most common autosomal recessive disorder, at least in western countries, is caused by mutations of CFTR. The disease affects the intestine, the pancreas and the liver, but most important for life quality and expectance of CF patients are alterations in lung functions. The present review describes a novel pathway to explain...
Objectives: The aim of this prospective longitudinal multicenter study was to dissect colonization from infection in patients with Staphylococcus aureus cultured from the airways by determining a variety of host-and pathogen-specific parameters. Methods: Inclusion criteria: >6 years, persistent S. aureus cultures from airway specimens. Specimens we...
Cystic fibrosis is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) molecule; these mutations result in a defect in chloride secretion in epithelial cell layers. The disease is characterized by severe gastrointestinal and pulmonary symptoms, but it is the pulmonary symptoms that dominate the clinical course of t...
Background/Aims: Several recent studies revealed an accumulation of ceramide in bronchial, tracheal and intestinal epithelial cells of mice and patients with cystic fibrosis (CF). Normalization of ceramide concentrations in lungs of CF mice employing the functional acid sphingomyelinase inhibitor amitriptyline also normalized mucociliary clearance,...
Background/Aims: Several recent studies revealed an accumulation of ceramide in bronchial, tracheal and intestinal epithelial cells of mice and patients with cystic fibrosis (CF). Normalization of ceramide concentrations in lungs of CF mice employing the functional acid sphingomyelinase inhibitor amitriptyline also normalized mucociliary clearance,...
Pseudomonas aeruginosa persists in patients with cystic fibrosis (CF) and drives CF lung disease progression. P. aeruginosa potently activates the innate immune system, mainly mediated through pathogen-associated molecular patterns, such as flagellin. However, the host is unable to eradicate this flagellated bacterium efficiently. The underlying im...
Background A genome wide association study identified Interferon-related development regulator 1 (IFRD1), a protein expressed by neutrophils, as a key modifier gene for cystic fibrosis (CF) lung disease. Here, we investigated the expression and regulation of IFRD1 in CF neutrophils. Methods IFRD1 expression was quantified in peripheral blood and ai...
Abstract
Background: Infants with extremely low birth weight uniformly
develop anemia of prematurity and frequently require
red blood cell transfusions (RBCTs). Although RBCT is
widely practiced, the indications remain controversial in the
absence of conclusive data on the long-term effects of RBCT.
Objectives: To summarize the current equipoise an...
Objectives: Interferon-related development regulator 1 (IFRD1), a protein highly expressed by neutrophils, was identified as a key modifier gene for cystic fibrosis (CF) lung disease in a genome wide association study. The expression levels and regulation of IFRD1 in neutrophils remained elusive. Here we investigated the protein expression and regu...
Introduction: Data on cystic fibrosis mice demonstrate a change of the sphingolipid metabolism with a massive accumulation of ceramide in the lung, trachea and gut. Ceramide mediates increased cell death, release of DNA in bronchi, chronic inflammation and increased infection susceptibility to pulmonary P. aeruginosa infections. Genetic or pharmaco...
Die mittlere Lebenserwartung der Patienten mit Cystischer Fibrose (CF) liegt bei etwa 30 Jahren. Sie ist im Wesentlichen durch
pulmonale und gastrointestinale Komplikationen eingeschränkt. Die terminale Destruktion der Lunge wird durch chronisch persistierende
Infektionen, v. a. mit Pseudomonas aeruginosa begünstigt. Eine Elimination der Problemkei...
In the majority of patients, secundum atrial septal defects (ASDs) are treated interventionally or surgically, before the onset of clinical symptoms, between 3 and 6 years of age. Because right-ventricular dimensions usually normalize after ASD closure, it has been assumed that cardiac function and exercise performance also normalize at long-term f...
Cystic fibrosis is characterized by severe intestinal and pulmonary symptoms, but also changes in the liver, pancreas and reproductive tract. Since gastrointestinal symptoms can be controlled, the quality of live and longevity of cystic fibrosis patients is mainly determined by pulmonary inflammation and chronic pulmonary infections with Pseudomona...
Ziele: Die Anwendung einer speziellen MRT Technik mit ultrakurzen Echozeiten erlaubt die Darstellung der Lunge mittels Protonendichtemessung. Die Studie untersucht Volumen (LV) und Signalintensitat (SI) der Lunge in der PD-gewichteten MRT und vergleicht die LV mit dem Gasvolumen (ITGV) der Lungenfunktion. Methode: 30 Patienten mit Zystischer Fibros...
PURPOSE
Special MRI technique with ultrashort echo times allows proton density imaging of the lung. This study evaluates the interrelation of lung volume and signal intensity and compares the according lung volumes to the intrathoracic gas volume (ITGV) of body plethysmography.
METHOD AND MATERIALS
10 healthy volunteers and 30 patients with cystic...
Antibiotic combination therapy might be more efficient than single antibiotics to combat Pseudomonas aeruginosa biofilms in the airways of patients with cystic fibrosis. We tested the ability of colistin sulphate-tobramycin combinations and single antibiotics to kill P. aeruginosa biofilms.
P. aeruginosa biofilms were generated in vitro and in rat...
The paranasal sinuses are almost always involved in cystic fibrosis, and chronic rhinosinusitis and nasal polyps are very frequent in the disease. Hereby, the patients' quality of life and their overall health are relevantly impaired. Although dornase alfa, a mucolytic agent, may also be effective in the upper airways, deposition of inhaled drugs i...
Patients with cystic fibrosis (CF) with Pseudomonas aeruginosa lung infections produce endobronchial mucus plugs allowing growth of obligate anaerobes including Prevotella spp. Whether obligate anaerobes contribute to the pathophysiology of CF lung disease is unknown.
The virulence of Prevotella intermedia and Ps aeruginosa was investigated in vitr...
Sphingolipids and in particular ceramide have been shown to be critically involved in the response to many receptor-mediated, but also receptor-independent, mainly stress stimuli. Recent studies demonstrate that ceramide plays an important role in the pathogenesis of cystic fibrosis, a hereditary metabolic disorder caused by mutations of the Cystic...
We hypothesized that a continuous 24-h infusion of 100 mg/kg per day ceftazidime (treatment C) would result in equivalent or even superior anti-infectious efficacy in chronic Pseudomonus aeruginosa (PA) infection in patients with cystic fibrosis (CF) in comparison to the usual application of 200 mg/kg per day ceftazidime in three doses (treatment T...
We hypothesized that a single intravenous (iv) tobramycine infusion (treatment B) would have equivalent anti-infectious efficacy in chronic Pseudomonas aeruginosa (PA) infection in cystic fibrosis (CF) as the commonly performed treatment of three doses (treatment A) . Toxicity and practicability may even be improved in the single-dose regimen.
This...
Employing genetic mouse models we have recently shown that ceramide accumulation is critically involved in the pathogenesis of cystic fibrosis (CF) lung disease. Genetic or systemic inhibition of the acid sphingomyelinase (Asm) is not feasible for treatment of patients or might cause adverse effects. Thus, a manipulation of ceramide specifically in...
Cystic fibrosis patients very often suffer from chronic pulmonary infections, in particular with Pseudomonas aeruginosa. The molecular mechanisms of the very high infection susceptibility of these patients are presently unknown.
Amitriptyline, a blocker of acid sphingomyelinase and acid ceramidase, significantly reduces Pseudomonas aeruginosa lung infection in cystic fibrosis (CF) mice with concurrent increase of survival. Our aim was to establish whether amitriptyline is safe and effective in the treatment of CF patients. In a randomised, double-blinded, placebo-controlle...
At present no evidence-based medical treatment for persistent atelectasis in pediatric non-cystic fibrosis (CF) patients is available.
To evaluate the use of intratracheally instilled recombinant human deoxyribonuclease (rhDNase) in intubated and ventilated pediatric patients, we performed a single-center observational study on 46 pediatric intensi...
Ceramide accumulation in lungs of individuals with CF causes pulmonary inflammation. Normalization of pulmonary ceramide concentrations corrects pathophysiological changes in CF lungs. Thus, ceramide might be a novel target to treat individuals with CF.
There is a paucity of knowledge about prenatal and perinatal risks through maternal amatoxin poisoning. No symptoms of amatoxin intoxication, except for a slight temporary increase in liver enzymes activity, occurred in a term newborn after delivery despite an Amanita phalloides intoxication of the mother 2 days before. Considering previous reports...
Microbial lung infections are the major cause of morbidity and mortality in the hereditary metabolic disorder cystic fibrosis, yet the molecular mechanisms leading from the mutation of cystic fibrosis transmembrane conductance regulator (CFTR) to lung infection are still unclear. Here, we show that ceramide age-dependently accumulates in the respir...
Microbial lung infections are the major cause of morbidity and mortality in the hereditary metabolic disorder cystic fibrosis, yet the molecular mechanisms leading from the mutation of cystic fibrosis transmembrane conductance regulator (CFTR) to lung infection are still unclear. Here, we show that ceramide age-dependently accumulates in the respir...
Inhaled rhDNase may improve sputum viscosity and mucociliary clearance by cleavage of extracellular DNA derived for instance from dead leukocytes in purulent, highly viscous patient sputum.
Here we established a method to quantify rhDNase-mediated DNA fragmentation in sputum using gel electrophoresis. Sputum of Pseudomonas aeruginosa colonized cyst...
Cystic fibrosis (CF), the most common autosomal recessive disorder, at least in western countries, is caused by mutations of the cystic fibrosis transmembranous conductance regulator (CFTR) molecule and affects approximately 80,000 patients in Europe and the USA. Most, if not all, CF patients develop a chronic pulmonary infection with Pseudomonas a...
Ceramide has been shown to be critically involved in many aspects of cellular responses to receptor-dependent and -independent stimuli. For instance, ceramide was demonstrated to be a central component of the signaling cascades mediating apoptosis after death receptor stimulation, treatment with chemotherapy or exposure to gamma-irradiation or UV-A...
Central elements in the infection of mammalian cells with viral, bacterial and parasitic pathogens include the adhesion of the pathogen to surface receptors of the cell, recruitment of additional receptor proteins to the infection-site, a re-organization of the membrane and, in particular, the intracellular signalosome. Internalization of the patho...
Citations
... In addition, YSE promotes the synthesis of LysoPC (20:5(5Z,8Z,11Z,14Z,17Z)), LysoPC (15:0), LysoPC (17:0), and LysoPC (18:0) in the blood, which is conducive to maintaining the balance of glycerophospholipid metabolism, reduces cell damage and inflammation, and ultimately plays a protective role in the liver. More importantly, PC is the main component of bile with biological functions, like promoting bile synthesis and secretion, enhancing the emulsification of fat, and participating in the enterohepatic circulation of bile acids [53]. ...
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... Previously, we found that expression of ACKR3 on the platelet plasma membrane is dynamically regulated upon activation [26,27]. Platelet ACKR3 mediates antiapoptotic effects of platelets [28,29] and loss of the receptor is associated with platelet hyperreactivity and apoptosis [24]. Recently, the hemoglobin degradation product hemin has been shown to induce platelet activation and non-apoptotic cell death by iron, known as ferroptosis [20,21,30]. ...
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... Brodlie et al. found that Cer accumulates in the trachea and epithelial cells of large and small bronchi of various CFTR-deficient mice before any infections and in lungs from CF patients [24]. Moreover, increased Cer levels were described in macrophages in both CF mice models and patients [25]. ...
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... Haemolysins are cytolytic toxins which are secreted by many S. aureus strains. They have been commonly recognised as essential virulence factors in murine CF infection models [54] and previous studies suggest strains downregulate expression of virulence factors during chronic infection [55]. We therefore sought to establish whether our chronic infection isolates retained the ability to lyse ovine red blood cells (RBC), and whether there were any differences in haemolytic activity between the mono-and coinfection strains. ...
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... 18 Further studies used CS-PLGA NP for messenger RNA (mRNA) delivery upon intravenous administration in mice. 19,20 The delivery potential of NP to antigen presenting cells (APC) has gained increased interest due to the function of APC as first line defense against pathogens as well as for their key role in inducing antigen-specific immune responses. 21 NP should allow for APC-selective delivery of immune-modulating molecules as well as antimicrobial compounds without inducing adverse immune reactions. ...
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... Before any examination, consider: a) Are there changes over time that have prognostic or therapeutic relevance for the patient? Multiple scores for each imaging modality are useful for such quantification of longitudinal trends [12][13][14][15]. b) Is there an acute event that requires immediate therapy? ...
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... MSCs are found in various human tissues and secretions such as adipose tissue, peripheral blood, menstrual blood, endometrium, breast milk, and fetal tissues such as amniotic fluid, membranes, chorionic membrane, placenta, umbilical cord, and umbilical cord blood (UCB) [23]. Under specific conditions, MSCs have the potential to differentiate into multiple cell types such as osteoblasts, chondrocytes, and adipocytes [24,25]. UCB is abundantly available, is easy and safe to collect, and can be used to isolate and culture MSCs in huge quantities. ...
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... In total, 18 trials (95 citations) with 3042 participants were included in the review and 76 trials (230 citations) were excluded. Four trials are listed under 'Studies awaiting classification' until more information becomes available (Flume 2015;Herrmann 2017;Nikonova 2010;Ramsey 2017). No trials are currently listed as ongoing. ...
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... Sphingosine, a long-chain sphingoid base, has broad--spectrum antibacterial effects, including Staphylococcus aureus, Escherichia coli, and Porphyromonas gingivalis [153][154][155][156][157][158]. Sphingosine is naturally found in the human body, such as nasal, tracheal, and bronchial epithelial cells [153,154,159]. Sphingosine tightly combines with CL in the bacterial cell membrane. ...
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... However, more research is needed in a developing country setting like India. In children with CF, the use of inhaled mannitol, a hyperosmotic mucoactive drug, also resulted in an improvement in lung function [forced expiratory volume in 1 s (FEV1)] (12,18,19). Clinical evidence supports the use of recombinant human DNAase I (rhDNase; dornase alfa), a mucolytic drug as it improved lung function (FEV1) and reduced pulmonary exacerbations in children with CF (20,21). ...
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