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

Related Links:

Publication date: Available online 28 December 2019Source: Journal of Pediatric Surgery Case ReportsAuthor(s): Christina B. Chadwick, Stephen Megison, David M. Troendle, Meghana SatheAbstractIndividuals with cystic fibrosis (CF) who possess milder and less common mutations can have preservation of exocrine pancreatic function (EPF) but are at risk for recurrent pancreatitis, chronic pancreatic damage, and loss of EPF. They may develop sequelae secondary to pancreatic disease including malabsorption, chronic pain, and disordered eating. In severe cases, endoscopic and surgical intervention is necessary. With advancement of ...
Source: Journal of Pediatric Surgery Case Reports - Category: Surgery Source Type: research
Pancreatic exocrine insufficiency occurs when there is insufficient production or delivery of pancreatic enzymes required for the digestion and absorption of food. It is associated with relatively poor outcomes, including reduced quality of life and survival.1 Common causes are cancer of the head/body of the pancreas, chronic pancreatitis, pancreatic resection and cystic fibrosis. It can occur following duodenectomy, gastrectomy or untreated coeliac disease via a reduction in cholecystokinin and thereby post-prandial pancreatic stimulation.
Source: Journal of Pain and Symptom Management - Category: Palliative Care Authors: Source Type: research
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: - 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 ( 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
More News: Children | Chronic Pain | Cystic Fibrosis | Gastroenterology | Hospitals | Nutrition | Pain | Pancreas | Pancreatitis | Parenteral Nutrition | Respiratory Medicine