Total pancreatectomy with islet autotransplantation in a pancreatic-sufficient cystic fibrosis patient

We present a case of a 4  year old female with pancreatic-sufficient CF, refractory ARP, frequent hospital admissions for abdominal pain, and continued growth failure despite gastrostomy tube and parenteral nutrition.
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Source Type: research

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The objective of this study was to analyze the current evidence for the use of pancreatic enzyme replacement therapy (PERT) in affecting survival and quality of life in patients with pancreatic exocrine insufficiency (PEI). Systematic searches of the literature were performed using the PubMed database. Articles were selected for inclusion if they reported findings from trials assessing the effects of PERT on quality of life, survival, malabsorption, growth parameters (such as height, body weight and body mass index), or gastrointestinal symptoms (such as abdominal pain, stool consistency and flatulence). PERT improved PEI-...
Source: World Journal of Gastroenterology : WJG - Category: Gastroenterology Authors: Tags: World J Gastroenterol Source Type: research
Conclusions: This document presents the first authoritative recommendations on nutritional considerations in pediatric pancreatitis. Future research should address the gaps in knowledge particularly relating to optimal nutrition for AP in children, role of diet or dietary supplements on recurrent attacks of pancreatitis and pain episodes, monitoring practices to detect early growth and nutritional deficiencies in CP and identifying risk factors that predispose children to these deficiencies.
Source: Journal of Pediatric Gastroenterology and Nutrition - Category: Gastroenterology Tags: Society Paper Source Type: research
Conclusion Total pancreatectomy with islet autotransplantation is a safe, effective treatment option for CF patients with CP, giving similar outcomes for those with other CP etiologies.
Source: Pancreas - Category: Gastroenterology Tags: Original Articles Source Type: research
Discussion Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the cystic fibrosis transmembrane receptor (CFTR). It is found in the epithelium of the bronchi, intestine, pancreatic duct and biliary tree. It regulates chloride, bicarbonate and water secretion. The heterozygous state helps prevent against secretory diarrhea, but the homozygous state causes thickened secretions in the hollow tubes of the lungs and digestive tract. There are multiple mutations (> 2000) which have been currently classified into classes depending on their protein production and activity. CF patients generally are l...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news
Date: October 9, 2017 Issue #:  1531Summary:  Pancreatic enzyme replacement products have been used for years to improve digestion in patients with exocrine pancreatic insufficiency (EPI). These products were initially marketed before formal FDA approval was required; in 1991, however, the FDA told all manufacturers of pancreatic enzyme replacement products that they would have to submit a new drug application by April 2010 in order to keep their products on the market.EPI is a chronic disorder characterized by a deficiency of exocrine pancreatic enzymes, which results in malabsorption, steator...
Source: The Medical Letter - Category: Drugs & Pharmacology Authors: Tags: Creon pancreatic Enzyme Products Pancreaze pancrelipase Pertzye Viokace Zenpep Source Type: research
Authors: Nikfarjam M, Wilson JS, Smith RC, Australasian Pancreatic Club Pancreatic Enzyme Replacement Therapy Guidelines Working Group Abstract INTRODUCTION: In 2015, the Australasian Pancreatic Club (APC) published the Australasian guidelines for the management of pancreatic exocrine insufficiency (http://pancreas.org.au/2016/01/pancreatic-exocrine-insufficiency-guidelines). Pancreatic exocrine insufficiency (PEI) occurs when normal digestion cannot be sustained due to insufficient pancreatic digestive enzyme activity. This may be related to a breakdown, at any point, in the pancreatic digestive chain: pancreatic ...
Source: Medical Journal of Australia - Category: General Medicine Tags: Med J Aust Source Type: research
ConclusionRigorous qualitative patient research and expert clinical input supported development of a PEI-specific PRO with the potential to aid management and monitoring of unmet needs among patients with PEI. The next step is to perform psychometric evaluation of the resulting instrument.
Source: The Patient - Patient-Centered Outcomes Research - Category: Global & Universal Source Type: research
Chronic pancreatitis (CP) is a common GI disorder with an incidence of 4/100 000 and a prevalence of 41/100 000.1 Owing to the chronic nature of the disease, patients with CP are often admitted to hospital and represent a serious socioeconomic burden across continents. Patients with CP have an increased mortality by more than threefold compared with the normal population, and the 20-year survival rate is reduced to 45% compared with 65% of the normal population.1 The disease mostly occurs in repeated episodes of acute inflammatory bouts of the pancreas parenchyma that progressively leads to exocrine and endocrine...
Source: Gut - Category: Gastroenterology Authors: Tags: Commentary Source Type: research
Abstract: A workshop was sponsored by the National Institute of Diabetes and Digestive and Kidney Diseases to focus on research gaps and opportunities in chronic pancreatitis (CP) and its sequelae. This conference marked the 20th year anniversary of the discovery of the cationic trypsinogen (PRSS1) gene mutation for hereditary pancreatitis. The event was held on July 27, 2016, and structured into 4 sessions: (1) pathophysiology, (2) exocrine complications, (3) endocrine complications, and (4) pain. The current state of knowledge was reviewed; many knowledge gaps and research needs were identified that require further inves...
Source: Pancreas - Category: Gastroenterology Tags: Conference Report Source Type: research
Purpose of review: To summarize observations in clinical chronic pancreatitis in the past year. Recent findings: A predisposing genetic mutation was identified in 67% of cases of pediatric chronic pancreatitis. A novel susceptibility gene involving a hybrid allele is associated with idiopathic chronic pancreatitis. ABO blood type B and FUT2 nonsecretor status is associated with asymptomatic hyperlipasemia and chronic pancreatitis. Alcohol consumption impairs cystic fibrosis transmembrane conductance regulator (CFTR) activity leading to decreased bicarbonate secretion and patients with susceptible CFTR mutations can develo...
Source: Current Opinion in Gastroenterology - Category: Gastroenterology Tags: PANCREAS: Edited by Fred S. Gorelick Source Type: research
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