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This research aims to improve the management of exocrine pancreatic insufficiency (EPI), a condition that can develop after pancreatitis, a painful inflammation of the pancreas. EPI occurs when the pancreas does not produce enough enzymes to help the body properly digest food. While pancreatic enzyme replacement therapy (PERT) is commonly used to manage EPI symptoms, it can be challenging for people who rely on feeding tubes. RELiZORB, could help these patients by simplifying the delivery of the enzymes they need. However, RELiZORB has only been studied in people with EPI caused by cystic fibrosis, so its effectiveness in pancreatitis patients remains unknown. This study aims to determine whether RELiZORB is effective for individuals requiring feeding tube support after pancreatitis.
This study aims to evaluate if a 6-month course of pancrelipase (CREON) treatment improves symptoms of exocrine pancreatic insufficiency (EPI) after an attack of acute pancreatitis. Diagnosis of EPI is measured by a fecal elastase value of \<200, and patients must have a qualifying symptom burden based on the EPI symptom tracker survey. Blood and stool will be analyzed as part of this study, and other surveys of health status will be used to track improvement of symptoms.
Pancreas transplantation is currently the most reliable method for glycemic control in insulin dependent diabetic patients. Outcomes of pancreas transplantation have improved significantly over the years due to improved surgical techniques, medical management and immunosuppression. However, weight gain after pancreas transplantation remains a common problem with associated consequences such as development of type 2 diabetes, coronary artery disease, graft loss, metabolic syndrome and increased risk of cardiovascular death. Excessive weight gain is well known after liver and kidney transplantation; however there are very few studies that have looked at weight gain after pancreas transplantation. In a recent study by Knight et al, 26% of the pancreas transplant recipients had excessive weight gain, defined as more than 30% of their baseline weight by 1-year post transplant. The study focused mainly on the endocrine function of the pancreas, explaining that excessive peripheral insulin circulation post-transplant may explain the weight gain. Other factors like immunosuppression, increased oral intake and potentially reduced activity may also have played a role. However no study has looked at the possible role of exocrine secretion from the new pancreatic allograft, combined with exocrine secretion of the old pancreas, leading to excessive availability of digestive juices like trypsin, chymotrypsin, lipase, amylase, gelatinase, elastase etc. Our hypothesis is that the excessive weight gain after pancreas transplant, which is more than in other solid organ transplants, is driven by the excessive digestive juice leading to improved conversion of available food and nutrient into storable energy and subsequently leading to weight gain. The patient will therefore need to either increase physical activity to avoid weight gain post-transplant or significantly reduce caloric intake. Fecal elastase test (FE-1)-elastase is a proteolytic enzyme produced by pancreatic acinar cells. They bind to bile salt and pass through the gut without degradation. These levels correlate well with the other pancreatic enzyme levels. Fecal elastase concentration (FEC) has been used routinely to screen for pancreatic exocrine insufficiency (PEI). Exocrine pancreatic juice has been a target for the management of obesity lately, with the use of drugs like Orlistat (Xenical) that inhibits pancreatic lipase and therefore interfere with the absorption of fat. If our theory of excessive pancreatic juice availability after pancreas transplant can be proven, it can help guide the targeted use and appropriate dosing of such drugs based on the level of the pancreatic juice as measured by the FEC.
The Shwachman-Diamond Syndrome Global Patient Survey and Collaboration Program (SDS-GPS) is an opportunity for patients and their families - from anywhere in the world - to share their experience living with SDS via a safe, secure, and convenient online platform, to * expand the understanding of SDS * improve the lives of people with SDS, and * accelerate the development of new therapies and cures for SDS. By joining, participants will receive early access to relevant information about new clinical trials and other research opportunities (such as clinical registries) based on their profile, accelerating research and increasing clinical trial impact and recruitment success. The platform, consent forms, and surveys are available in five languages: English, Spanish, French, German, and Italian. More languages to come.
The purpose of this research is to determine if the use of RELiZORB™ improves nutrition tolerance and helps critically ill patients meet their nutrition goals. RELiZORB™ is a digestive enzyme cartridge that contains lipase and works as a pancreatic enzyme replacement. It promotes breaking down fat and helps the body absorb it. The device connects with tube feedings to help the body with digestion. RELiZORB™ is approved by the U.S. Food and Drug Administration (FDA) for use with tube feedings in patients 5 years of age or older. While the use of RELiZORB™ in this study is consistent with the FDA approval, the use of RELiZORB™ in patients with multi organ failure is not in the current standard of care practice at Inova Health Care facilities.
Diabetes is a major co-morbidity in pancreatic insufficient cystic fibrosis (PI-CF) and associated with worse outcomes. While reduced β-cell mass contributes to the insulin secretory defects that characterizes cystic fibrosis-related diabetes (CFRD), other modifiable determinants appear operative in the emergence and progression of abnormal glucose tolerance towards diabetes. Identifying interventions to preserve β-cell function are crucial for delaying and potentially preventing CFRD development. In this study, we hypothesize that weekly administration of the long-acting glucagon-like peptide-1 (GLP-1) agonist dulaglutide will improve defective early-phase insulin secretion and improve glucose tolerance during a mixed-meal tolerance test.
In recent years, diabetes has emerged as one of the most significant co-diseases that many Cystic Fibrosis (CF) patients develop. Type 1 (T1D) and Type 2 (T2D) diabetes results when either the body does not make enough insulin or the body does not respond correctly to this insulin, respectively. Insulin is a hormone which is made by cells in the pancreas and helps carry glucose (sugar) from the food we eat to the cells of the body for energy. While cystic fibrosis related diabetes (CFRD) has many features similar to both T1D and T2D, patients with CF may not have the same symptoms as either T1D or T2D patients. Currently, there is little understanding of CFRD and the best options for treatment remain unclear. The purpose of this research study is to examine and understand the various mechanisms that contribute to CFRD and gain a better understanding of potential means to treat CFRD. In particular, we plan to study the effects of incretin hormones that can enhance insulin production in CF patients. Enrollment is complete for the protocol as initially written. In order to further study the role of the incretin hormone on Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) function , we have received approval to extend our investigation to include the following study groups: * Cystic Fibrosis participants with normal glucose tolerance * Non-Cystic Fibrosis controls