
Study of LYM-X-SORB™ to Improve Fatty Acid and Choline Status in Children With CF and PI
Cystic FibrosisPancreatic InsufficiencyThe purpose of this phase II research study is to evaluate the effectiveness of the next generation LYM-X-SORB™ in improving the essential fatty acid (EFA) and choline status for children and adolescents with Cystic Fibrosis (CF) and pancreatic insufficiency (PI).

Enteral Nutrition in Liver Cirrhosis
Liver CirrhosisMalnutrition is frequently occurring in patients with liver cirrhosis and is associated with a poorer outcome, as it determines life quality and affects both morbidity and mortality. Long term prognosis after liver transplantation also depends on the nutritional status. Therefore, the early diagnosis and intervention for malnutrition is an important issue in the clinical management of liver cirrhosis. However, oral intake has been shown to be insufficient due to disease related anorexia or concomitant drowsiness and confusion. Specific aims: To investigate the effect of early nutritional intervention via tube feeding on nutritional status, functional status (hand grip strength and peak flow), life quality and disease related complications such as intestinal permeability, and oxidative stress, as well as liver function.

Fibroblast Specific Inhibition of LOXL2 and TGFbeta1 Signaling in Patients With Pulmonary Fibrosis....
Idiopathic Pulmonary FibrosisThis is a two part study. In the first part, the pharmacokinetic profile of Epigallocatechin-3-gallate (EGCG) in normal human volunteers given a single oral dose will be determined to set the dose for the second part of the study. In the second part of this study, lung biopsy fragments and urine samples from patients with interstitial lung disease treated with EGCG will be evaluated in biochemical assays and compared to samples from untreated control patients.

Clinical Impact of MI on Inhaled Antibiotic Adherence in Cystic Fibrosis
Cystic FibrosisAdherence to treatment is a major challenge in cystic fibrosis (CF). Motivational Interviewing (MI) is increasingly used to address this, but its effectiveness is unknown. We investigated the clinical impact of an MI intervention, delivered by a specifically trained pharmacist, on adherence to inhaled antibiotics (IA).

Granulocyte-Colony Stimulating Factor (G-CSF) as Optimizing Therapy for Pediatric Liver Transplantation...
Liver CirrhosisSevere Malnutrition2 moreThis study compares the effect of human recombinant Granulocyte-Colony Stimulating Factor (G-CSF) in pediatric patients with liver cirrhosis with a control group. The study aims to observe improvement of (Pediatric End-stage Liver Disease) PELD score and nutritional status prior to liver transplantation procedure. In addition to the intervention, standard treatments for liver cirrhosis are also given for both groups. G-CSF is administered for 12 times. Condition of disease: Pediatric patient aged 3 months to 12 years old Liver cirrhosis Undernourished / Severe malnutrition PELD score 10-25 Intervention: Drug: Recombinant Human G-CSF Phase: Phase 3

Outcomes in CF Patients Accessing Their Registry Health Records
Cystic FibrosisA multifactorial, parallel group, randomised control study in cystic fibrosis (CF) patients aged 13-30 years over an 18 month period. Patients will be offered read-only access to their CF registry electronic health record (CF View), or educational videos on CF (videos), or videos and CF View, or usual standard of care. The study aims are to examine the effect of patient access to C View on a range of clinical outcomes, health service usage, health literacy and patient reported outcomes.

Study of Circulating Blood Cell Monocytes as Predictive Biomarker of Bone Cystic Fibrosis Disease....
Cystic FibrosisThe aim of the study is to look for risk factors of developing osteoporosis in patients with cystic fibrosis. Blood cells called monocytes will be obtained from a blood sample. These monocytes will make possible to form another class of cells called osteoclasts in the laboratory. These latter cells are responsible for bone degradation and therefore promote the increase of bone fragility. The research will focus on the characterization of these cells to better understand their potential (low, medium or high) to increase bone fragility in patients with cystic fibrosis. Finally the action of CFTR modulators on these bone cells will be tested.

Inspiratory Muscle Training in Patients With Interstitial Lung Disease
Interstitial FibrosisPulmonary Disease5 moreThe aim of this study is to evaluate the effects of inspiratory muscle training program in inspiratory muscle endurance, breathlessness, inspiratory muscle strength, functional capacity and quality of life in patients with interstitial lung disease. Patients are evaluated before the inspiratory muscle training and after 8 weeks of training.

Continuous Cardiorespiratory Monitoring in Cystic Fibrosis
Cystic FibrosisCystic Fibrosis (CF) is a disease without a primary cure that requires lifelong care and is characterized by pulmonary exacerbations (PEx). Wearable devices could provide a way for long-term monitoring of disease progression and early signs of PEx to intervene as early as possible, thereby improving long-term outcomes. This in-hospital feasibility study will assess the ability of Byteflies Sensor Dots to collect relevant cardiorespiratory data in people with CF and its compatibility with clinical workflows, 2) identify candidate digital biomarkers, and 3) collect user feedback from patients and healthcare providers.

MyVoice:CF Decision Aid for Women With Cystic Fibrosis
Cystic FibrosisContraception2 moreThis project is a single-center feasibility study of MyVoice:CF, a patient-facing, web-based decision aid. Aim 1) Assess the acceptability, feasibility, and usability of MyVoice:CF for women with CF and multidisciplinary adult CF providers. Aim 2) Assess the preliminary efficacy of MyVoice:CF related to patient-provider communication, shared decision-making, knowledge, and self-efficacy for women with CF related to reproductive health concerns.