People with Cystic Fibrosis (CF) have problems digesting their food properly. More than 8 in 10 people with CF must take medication to assist their digestion. In spite of this, complications such as bowel blockage occur. Finding out how already licenced drugs for CF work in the gut is the first step in repurposing medications. Tezacaftor/Ivacaftor with Ivacaftor is a drug combination which corrects the basic defect in CF an has shown improvements on lung function. The purpose of this study is to evaluate, using Magnetic Resonance Imaging (MRI) and patient-reported outcomes, whether Tezacaftor/Ivacaftor with Ivacaftor has an effect on improving gastrointestinal problems in CF.

NCT04006873

Conditions

1. Cystic Fibrosis

Interventions

1. Drug: Tezacaftor/Ivacaftor + Ivacaftor
2. Drug: Placebo oral tablet

MeSH:Cystic Fibrosis Fibrosis

According to previous studies, viral pneumonia can develop into pulmonary fibrosis, which can affect patients'lung function and even life health.This study aims to observe the efficacy and safety of Fuzheng Huayu Tablets in the treatment of pulmonary fibrosis after COVID-19.

NCT04279197

Conditions

1. Pulmonary Fibrosis Due to COVID-19

Interventions

1. Drug: Fuzheng Huayu Tablet
2. Drug: Vitamin C tablets
3. Other: Placebo
4. Other: respiratory function rehabilitation training

MeSH:Pulmonary Fibrosis Fibrosis

HPO:Pulmonary fibrosis

COVID-19 pandemic with SARS-CoV-2 infection has become a global challenge. Though most cases of COVID-19 are mild, the disease can also be fatal. Patients with liver cirrhosis are more susceptible to damage from SARS-CoV-2 infection considering their immunocompromised status. The spectrum of disease and factors that influence the disease course in COVID-19 cases with liver cirrhosis are incompletely defined. This muilticentre observational study (COVID-Cirrhosis-CHESS2002) aims to study the clinical characteristics and risk factors associated with specific outcomes in COVID-19 patients with pre-existing liver cirrhosis.

NCT04329559

Conditions

1. COVID-19
2. Liver Cirrhosis

MeSH:Liver Cirrhosis Fibrosis

HPO:Cirrhosis Hepatic fibrosis

This study aims to assess the effects of programmed exercise combined with CFTR protein modulator drugs in the cardiorespiratory fitness, strength, functional capacity and agility in a group of young patients with Cystic Fibrosis.

NCT04415268

Conditions

1. Cystic Fibrosis in Children

Interventions

1. Behavioral: Exercise
2. Behavioral: Unsupervised exercise
3. Drug: CFTR Modulators

MeSH:Cystic Fibrosis Fibrosis

To investigate the efficacy of BI 1015550 compared to placebo based on the change from baseline in Forced Vital Capacity (FVC). To investigate safety and tolerability of BI 1015550 in the overall trial population.

NCT04419506

Conditions

1. Idiopathic Pulmonary Fibrosis

Interventions

1. Drug: BI 1015550
2. Drug: Placebo

MeSH:Pulmonary Fibrosis Idiopathic Pulmonary Fibrosis Fibrosis

HPO:Pulmonary fibrosis

Proton-pump inhibitors (PPI) are commonly prescribed in an uncritical manner to patients with liver cirrhosis without a clear evidence-based indication. Observational studies suggests that PPI use in cirrhotic patients may be a risk factor for the development of infections, especially spontaneous bacterial peritonitis (SBP). A possible explanation are PPI-associated microbiotic shifts leading to small intestinal bacterial overgrowth with subsequently increased bacterial translocation. Furthermore, PPI therapy in cirrhotic patients may lead to an increased risk for pneumonia and Clostridium difficile-infections. However, the evidence is ambiguous, as other published studies found no evidence for an association of PPI use with an increased risk for SBP or pneumonia. Moreover, an association between episodes of hepatic encephalopathy and PPI use has been reported. Infections and hepatic encephalopathy may often lead to a hospitalization of cirrhotic patients and PPI use at discharge has also been associated to early re-hospitalization. While some studies found an association of PPI and increased mortality in cirrhotic patients, other studies could not observe this association. Thus, some of the current evidence suggests an unfavourable risk profile of PPIs in patients with liver cirrhosis. However, this patient population is considered to be at a high risk of gastrointestinal haemorrhage from peptic ulcers. Importantly, patients with liver cirrhosis have an increased mortality after peptic ulcer bleeding as compared to patients without cirrhosis. Therefore, generous PPI use may also have a yet unproven preventive effect against upper gastrointestinal bleeding. The STOPPIT trial is the first prospective, randomized, controlled, double-blind trial investigating the effect of discontinuation of long-term PPI therapy on hospitalized patients with complicated liver cirrhosis with a pre-existing long-term PPI therapy. Importantly, patients with an evidence-based indication for PPI therapy are excluded from the trial. All study participants (n=476) stop their previous PPI treatment and are then randomized (1:1) to receive either placebo (intervention group) or esomeprazole 20mg/day (control group) for 360 days. The primary hypothesis anticipates a delay of re-hospitalisation and/or death (composite endpoint) in patients who discontinue PPI treatment as compared to patients who continue PPI therapy. Secondary objectives include the assessment of mortality, re-hospitalisation rates, infection rates, rate of acute hepatic decompensation and ACLF, as well as rates of upper and lower gastrointestinal bleeding events in both groups. Impact of prolonged or discontinued PPI therapy on the intestinal microbiota and pharmacoeconomics will be studied as a secondary assessment.

NCT04448028

Conditions

1. Liver Cirrhosis

Interventions

1. Drug: Placebo
2. Drug: Esomeprazole 20mg

MeSH:Liver Cirrhosis Fibrosis

HPO:Cirrhosis Hepatic fibrosis

Impacts of the Covid-19 epidemic and associated lockdown measures on the management, health and behaviors of cystic fibrosis patients during the 2020 epidemic

NCT04463628

Conditions

1. Cystic Fibrosis in Children
2. Cystic Fibrosis

Interventions

1. Behavioral: Questionnaire
2. Behavioral: Interview

MeSH:Cystic Fibrosis Fibrosis

This study is investigating the role of allergic (Th2) inflammation in patients with Cystic Fibrosis (CF) and history of fungal infection and/or Allergic Bronchopulmonary Aspergillosis. Little is known about fungal infection in CF and conflicting results exist on whether this results in worse lung function over time. There is concern that persistent fungal infection can result in worse clinical outcome measures in patients with CF. Also, it is unclear how ABPA develops, but may be related to the amount of fungus a patient with CF is infected with. This study looks at inflammatory patterns and allergic responses to fungal elements to help identify biomarkers and signs of allergic disease in fungally infected patients with CF.

NCT04476758

Conditions

1. Cystic Fibrosis
2. Fungal Infection
3. Allergic Bronchopulmonary Aspergillosis

MeSH:Infection Communicable Diseases Mycoses Aspergillosis Pulmonary Aspergillosis Aspergillosis, Allergic Bronchopulmonary Cystic Fibrosis Fibrosis

The main differences observed between SARSCoV-2 pneumonia and other epidemic viral pneumopathies (e.g., seasonal influenza) are the greater infectivity of SARSCoV-2, the clinical severity of the disease, particularly in young patients without co-morbidities, and the observation of radiological images related to significant parenchymal aggression in a large number of patients. The lesions in the acute phase correspond essentially to bilateral ground glass opacity more or less associated with condensations which would be markers of more severe infections. The major scope of the lesions in the acute phase raises the question of whether or not the scanning anomalies are completely resolved over time, and the possible impact on lung function. This risk of sequelae is very important to study given the large number of patients affected by SARSCoV-2, especially since these are often young patients who appear to be "healthy". In the current context of the CoV-2 SARS pandemic, the improved quality and availability of diagnostic scanners provides a wealth of information on the semiology and progression of lung disease with minimal exposure to ionizing radiation. A majority of hospitalized patients with SARSCoV-2 received a CT scan in the early phase of the disease. Indeed, the French Society of Radiology has recommended the performance of a CT scan without injection in thin sections in case of suspicion or for confirmation of the diagnosis in patients presenting initial or secondary clinical signs of severity and justifying hospital management due to the initial lack of reagents for performing biological tests (RT-PCR) and the high sensitivity of the CT scan and its specificity in epidemic periods. The present study aims to study the kinetics of lung involvement in SARS CoV 2, to study the predictive character of the chest CT scan performed at the patient's discharge on the existence of radiological sequelae at 3 months but also at 1 year in order not to misunderstand the constitution of late fibrosis after partial resolution of the CT images. The investigatos will study the correlation between possible radiological abnormalities and the clinical presentation (patient symptoms and lung function). The rigorous follow-up of these patients will allow us to set up, if necessary, early treatment of the detected abnormalities (inhaled corticoids in case of bronchial or bronchiolar damage, study of the place of an anti-fibrosis treatment in case of fibrosis,...).

NCT04483752

Conditions

1. CoV2 SARS Pneumonia

Interventions

1. Other: CHEST CT SCAN

MeSH:Pneumonia Fibrosis

HPO:Pneumonia

SARS-CoV-2 infection induces a hyperinflammatory syndrome, causing the acute respiratory distress syndrome, massive lung cell destruction and, as a plausible sequelae, pulmonary fibrosis in COVID-19 patients. Current focus has been on the development of novel immunosuppressant therapies, in order to control the cytokine storm in COVID-19 patients. Thus, the effect of steroids, intravenous immunoglobulin, non-steroidal immunosuppressants, selective cytokine blockade, JAK/STAT pathway inbhibition, and mesenchymal precursor cells have been evaluated. Based on the above information, we propose COLLAGEN-POLYVINYLPYRROLIDONE (Distinctive name: FibroquelMR, active substance: Collagen-polyvinylpyrrolidone, pharmaceutical form: intramuscular injectable solution, with sanitary registration No. 201M95 SSA IV and SSA code: 010 000 3999) as a potential drug for the downregulation of the cytokine storm. Polymerized type I collagen reduces the expression of IL-1β, IL-8, TNF-alpha, TGF-β1, IL-17, Cox-1, leukocyte adhesion molecules (ELAM-1, VCAM- 1 and ICAM-1), some other mediators of inflammation and increases the levels of IL-10 and the number of regulatory T cells. In addition, it promotes the mechanisms of inhibition of tissue fibrosis, without adverse effects in rheumatoid arthritis and osteoarthritis.

NCT04517162

Conditions

1. Covid19
2. Cytokine Storm
3. Regulation of Inflammatory Response
4. Pulmonary Fibrosis

Interventions

1. Drug: Collagen-Polyvinylpyrrolidone

MeSH:Pulmonary Fibrosis Fibrosis

HPO:Pulmonary fibrosis

Methodology: This is a controlled, randomized, multicenter open-label Phase Ib clinical exploratory trial in patients with fibrosing interstitial lung disease secondary to SARS-CoV-2 infection. Patients who give informed consent will be screened for enrolment in the study. Patients that meet the eligibility criteria will be enrolled and randomly allocated in the control arm (best standard of care) or the experimental arm (best standard of care plus IN01 vaccination). The patients enrolled in the control arm of the study will receive standard of care. The primary endpoint is safety, measured by the Frequency and severity of AEs graded according to Common Terminology Criteria for Adverse Events (CTCAE) Version 5.0 criteria. Biochemical and blood count alterations will be also monitored. Safety will be defined based on the frequency and severity of adverse events (AEs) throughout the patient's participation in the study comparing between control and experimental arms. Efficacy will be measured as function of the annual rate of decline in the Forced Vital Capacity (FVC) at 1 year after patient inclusion in the study and the blood oxygen saturation levels at days 1, 14 (w2), d 28 (w4), 42 (w6) and 92 (w12); week 24, week 36 and week 52. High-resolution Computed Tomography (CT) scans will be taken at at baseline and weeks, 12, 24, and 52 to evaluate the resolution of the fibrosing interstitial lung disease. A translational substudy will be included. Objectives: Primary Objective ● To evaluate the safety and tolerability of IN01 vaccine in diagnosed ex-COVID-19 patients that develop fibrotic lung syndrome after infection. Secondary Objectives - To evaluate the effect of IN01 vaccine on Oxygen saturation, pulmonary function, quality of life and fibrosing status in ex-COVID-19 patients that developed fibrosing lung disease after infection. - To assess biomarkers and molecular markers related to the IN01 vaccine mechanism of action.

NCT04537130

Conditions

1. Pulmonary Fibrosis
2. Covid19

Interventions

1. Biological: IN01 vaccine

MeSH:Pulmonary Fibrosis Fibrosis

HPO:Pulmonary fibrosis

Currently, there is no approved treatment for COVID-19 in France, either for the acute phase, nor for the late chronic phase. the investigator suggest that nintedanib has the potential to block the development of lung fibrosis when initiated early enough to inhibit the activation of mesenchymal cells and the progression of virus-induced pulmonary fibrosis. Computerized Tomography (CT) manifestations of fibrosis or fibrous stripes are described in COVID-19 (Ye, Eur Radiol 2020). Pan et al observed fibrous stripes in 17% patients in the early phase of the disease (Pan, Eur Radiol 2020). Ye et al observed bronchiectasis in 2 patients (15.4%) and evidence of pulmonary fibrosis in 3 patients (23.7%) at HRCT performed at 4 weeks (Ye, Eur Radiol 2020). Long term data are still lacking in patients with COVID-19 and the investigators do not know how many patients will have fibrotic sequelae from the acute illness.

NCT04541680

Conditions

1. SARS-Cov-2 Induced Pulmonary Fibrosis

Interventions

1. Drug: Nintedanib 150 MG [Ofev]
2. Other: Placebo

MeSH:Pulmonary Fibrosis Fibrosis

HPO:Pulmonary fibrosis

A randomized controlled trial to study the efficacy of low dose steroid for 14 days in the treatment of post-covid-19 lung infiltrates

NCT04551781

Conditions

1. Covid19

Interventions

1. Drug: 20 Mg Prednisone for 14 days
2. Drug: control

MeSH:Pulmonary Fibrosis Fibrosis

HPO:Pulmonary fibrosis

The purpose of this study is to validate and utilize a personalized medicine approach to identify potential treatments with current FDA approved CFTR modifiers for non-approved CF gene mutations. The study will perform ex vivo testing of CFTR function and current marketed CFTR modulating drugs on expanded nasal cells at Cincinnati Children's Human Nasal Epithelium (HNE) Core Laboratory. The results will be confirmed and translated into bedside care through an N of 1 trial to determine effectiveness of treatment.

NCT04580368

Conditions

1. Cystic Fibrosis

Interventions

1. Drug: CFTR Modulators

MeSH:Cystic Fibrosis Fibrosis

This is a Phase 1, 3-part, randomized, double-blinded, placebo-controlled study to evaluate the safety, tolerability, pharmacokinetics, and pharmacodynamics of TD-1058 inhaled solution. Part A is a SAD study in healthy subjects, Part B is a MAD study in healthy subjects, and Part C is a multiple-dose study in subjects with IPF.

NCT04589260

Conditions

1. Idiopathic Pulmonary Fibrosis (IPF)

Interventions

1. Drug: TD-1058
2. Drug: Placebo

MeSH:Pulmonary Fibrosis Idiopathic Pulmonary Fibrosis Fibrosis

HPO:Pulmonary fibrosis

The primary objective of this study is to evaluate the effect of brensocatib at 10 mg and 25 mg compared with placebo on the rate of pulmonary exacerbations (PEs) over the 52-week treatment period.

NCT04594369

Conditions

1. Non-Cystic Fibrosis Bronchiectasis

Interventions

1. Drug: Brensocatib 10 mg
2. Drug: Brensocatib 25 mg
3. Drug: Placebo

MeSH:Bronchiectasis Fibrosis

HPO:Bronchiectasis

This is a collaborative study between Icahn School of Medicine at Mount Sinai and Boehringer Ingelheim Pharmaceuticals to determine the effect of Nintedanib on slowing the rate of lung fibrosis in patients who have been diagnosed with COVID-19, and have ongoing lung injury more than 4 weeks out from their diagnosis.

NCT04619680

Conditions

1. Pulmonary Fibrosis
2. Interstitial Lung Disease
3. Respiratory Disease

Interventions

1. Drug: Nintedanib
2. Drug: Placebo

MeSH:Lung Diseases Pulmonary Fibrosis Lung Diseases, Interstitial Respiration Disorders Respiratory Tract Diseases Fibrosis

HPO:Abnormal lung morphology Interstitial pneumonitis Interstitial pulmonary abnormality Pulmonary fibrosis

Cystic fibrosis (CF) affects men and women equally, but after the onset of puberty, women with CF have a lower life expectancy than men with CF. Despite these known differences, the link between CF symptom trends and the menstrual cycle remains critically understudied. To address this gap, this study will investigate changes in CF-specific symptoms among women with CF to evaluate whether and how they correlate with their menstrual cycle. Specifically, the investigators hope to examine whether CF-related symptoms change throughout the menstrual cycle, what the impact of those symptoms is on quality of life, and how feasible it is to use a period tracking app to track CF-related symptoms throughout the menstrual cycle. Investigators are asking women ages 18-45 with CF, who have regular menstrual cycles, to participate. Study procedures, including online surveys, period tracking, and interview, will take approximately 3 months.

NCT04620096

Conditions

1. Cystic Fibrosis

MeSH:Cys Cystic Fibrosis Fibrosis