61 research outputs found
sj-docx-1-taj-10.1177_20406223221108627 – Supplemental material for Personalized medicine with drugs targeting the underlying protein defect in cystic fibrosis: is monitoring of treatment response necessary?
No full textSupplemental material, sj-docx-1-taj-10.1177_20406223221108627 for Personalized medicine with drugs targeting the underlying protein defect in cystic fibrosis: is monitoring of treatment response necessary? by Katharina Niedermayr, Verena Gasser, Claudia Rueckes-Nilges, Dorothea Appelt, Johannes Eder, Teresa Fuchs, Lutz Naehrlich and Helmut Ellemunter in Therapeutic Advances in Chronic Disease</p
Guidelines for the diagnosis and management of distal intestinal obstruction syndrome in cystic fibrosis patients
No full textAbstractComplete or incomplete intestinal obstruction by viscid faecal material in the terminal ileum and proximal colon – distal intestinal obstruction syndrome (DIOS) – is a common complication in cystic fibrosis. Estimates of prevalence range from 5 to 12 episodes per 1000 patients per year in children, with higher rates reported in adults. DIOS is mainly seen in patients with pancreatic insufficiency, positive history of meconium ileus and previous episodes of DIOS. DIOS is being described with increasing frequency following organ transplantation. Diagnosis is based on suggestive symptoms with a right lower quadrant mass confirmed on X-ray. The main differential is chronic constipation. Treatment consists of rehydration combined with stool softening laxatives or gut lavage with balanced electrolyte solutions. Rapid fluid shifts have been described following osmotic agents. Avoiding dehydration and optimizing pancreatic enzyme dosage may reduce the chance of further episodes. Prophylactic laxative therapy is widely used, but is not evidence-based
Lung clearance index to detect the efficacy of Aztreonam lysine inhalation in patients with cystic fibrosis and near normal spirometry – A single-centre feasibility study
No full textComparing the efficacy of inhaled antibiotics can be difficult in small groups of patients with cystic fibrosis and mild lung disease. In a feasibility study we compared Aztreonam lysine for inhalation solution (AZLI; Cayston®) to standard inhaled antibiotic therapy in patients with cystic fibrosis and near normal spirometry. To detect treatment responses we used both lung clearance index (LCI) and forced expiratory volume in one second (FEV1). At baseline, median FEV1 was 87% pred. and median LCI was 8.6 (upper limit of normal: 7.0). After 4 weeks, LCI improved by -0.36 after AZLI and deteriorated by +0.12 after tobramycin treatment (p = 0.039). No significant differences between treatments (p = 0.195) were observed using FEV1. These results suggest that lung clearance index can be used to detect treatment induced changes in subjects with mild lung disease.</div
International prospective study of distal intestinal obstruction syndrome in cystic fibrosis: Associated factors and outcome
No full textBackground Distal intestinal obstruction syndrome (DIOS) is a specific complication of cystic fibrosis. Methods A study was performed in 10 countries to prospectively evaluate the incidence, associated factors, and treatment modalities in children and adults. Results 102 patients presented 112 episodes. The incidence of DIOS was similar in children and adults. Medical treatment failed only in cases of complete DIOS (11%). Children with meconium ileus had a higher rate of surgery for DIOS (15% vs. 2%, p = 0.02). Complete DIOS entailed longer hospitalisation (4 [3; 7] days vs. 3 [1; 4], p = 0.002). Delayed arrival at hospital and prior weight loss had a significant impact on the time needed for DIOS resolution. Associated CF co-morbidities for DIOS included meconium ileus (40% vs. 18%, p < 0.0001), exocrine pancreatic insufficiency (92% vs. 84%, p = 0.03), liver disease (22% vs. 12%, p = 0.004), diabetes mellitus (49% vs. 25%, p = 0.0003), and Pseudomonas aeruginosa (68% vs. 52%, p = 0.01); low fibre intake and insufficient hydration were frequently observed. Female gender was associated with recurrent DIOS (75% vs. 52%, p = 0.04), constipation with incomplete episodes (39% vs. 11%, p = 0.03), and poor patient compliance in taking pancreatic enzyme therapy during complete episodes (25% vs. 3%, p = 0.02). Conclusion DIOS is a multifactorial condition having a similar incidence in children and adults. We show that delayed arrival at hospital after the initial symptoms causes significant morbidity. Early recognition and treatment would improve the prognosis
Bridging of host-microbiota tryptophan partitioning by the serotonin pathway in fungal pneumonia
No full textAbstract The aromatic amino acid L-tryptophan (Trp) is essentially metabolized along the host and microbial pathways. While much is known about the role played by downstream metabolites of each pathways in intestinal homeostasis, their role in lung immune homeostasis is underappreciated. Here we have examined the role played by the Trp hydroxylase/5-hydroxytryptamine (5-HT) pathway in calibrating host and microbial Trp metabolism during Aspergillus fumigatus pneumonia. We found that 5-HT produced by mast cells essentially contributed to pathogen clearance and immune homeostasis in infection by promoting the host protective indoleamine-2,3-dioxygenase 1/kynurenine pathway and limiting the microbial activation of the indole/aryl hydrocarbon receptor pathway. This occurred via regulation of lung and intestinal microbiota and signaling pathways. 5-HT was deficient in the sputa of patients with Cystic fibrosis, while 5-HT supplementation restored the dysregulated Trp partitioning in murine disease. These findings suggest that 5-HT, by bridging host-microbiota Trp partitioning, may have clinical effects beyond its mood regulatory function in respiratory pathologies with an inflammatory component
LCI during and after inhalation with TOBI<sup>®</sup> and AZLI.
No full textDecreasing values indicate improvement in lung function.</p
Lung clearance index to detect the efficacy of Aztreonam lysine inhalation in patients with cystic fibrosis and near normal spirometry – A single-centre feasibility study - Fig 3
No full textChanges in LCI (left) or FEV1z-score (right) after inhaled antibiotics. More favourable LCI response to AZLI.</p
Is There a Difference in Immune Response to SARS-CoV-2 Vaccination between Liver and Lung Transplant Patients with Cystic Fibrosis?
No full textPeople with Cystic Fibrosis (CF), especially solid organ transplant recipients, have been prioritized in the SARS-CoV-2 vaccination program. This study assesses antibody response of patients with CF who have undergone liver (CF-LI) or lung (CF-LU) transplantation, and compares results to published data of patients with solid organ transplantation without CF as underlying disease. Antibodies against the spike receptor-binding domain were measured within the routine visits at the CF Centre in Innsbruck, Austria, after the second and third doses of SARS-CoV-2 mRNA vaccines. We report on 13 adult CF patients who are recipients of solid organ transplant, including five CF-LI and eight CF-LU. Overall, 69% had measurable antibody response after two, and 83% after three doses of SARS-CoV-2 vaccines. In CF-LI, positive serological response amounted to 100% after two and three doses, while CF-LU showed only a 50% and a 71% response rate, respectively. Clear differences are seen between the CF-LI and CF-LU groups in our cohort, with worse response rate for lung transplant recipients. Immune response between CF-LI and CF-LU, therefore, must be considered in a differentiated manner, and the importance of booster vaccination is once more emphasized with these data
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