Genomes and Genes
exocrine pancreatic insufficiency
Summary: A malabsorption condition resulting from greater than 10% reduction in the secretion of pancreatic digestive enzymes (LIPASE; PROTEASES; and AMYLASE) by the EXOCRINE PANCREAS into the DUODENUM. This condition is often associated with CYSTIC FIBROSIS and with chronic PANCREATITIS.
Publications209 found, 100 shown here
- High prevalence of exocrine pancreatic insufficiency in diabetes mellitus. A multicenter study screening fecal elastase 1 concentrations in 1,021 diabetic patientsPhilip D Hardt
Third Medical Department and Policlinic, Giessen University Hospital, Giessen, Germany
Pancreatology 3:395-402. 2003..In this study we had the opportunity to study more than 1,000 diabetic patients to confirm recent observations in smaller populations...
- Screening for pancreatic exocrine insufficiency in patients with diabetes mellitusAmadeu C R Nunes
Department of Gastroenterology, University Hospitals of Coimbra, Avenida Urbano Duarte, Quinta da Estrela, Lote 7, 6o B, Coimbra, Portugal
Am J Gastroenterol 98:2672-5. 2003..The aim of this study was to screen patients with diabetes mellitus (DM) for pancreatic exocrine insufficiency...
- Vegetarian diet alters the assessment of exocrine pancreatic function with the use of fecal testsJaroslaw Walkowiak
Institute of Pediatrics, Karol Marcinkowski University of Medical Sciences, Poznan, Poland
J Pediatr Gastroenterol Nutr 38:224-6. 2004
- Some patients with irritable bowel syndrome may have exocrine pancreatic insufficiencyJohn S Leeds
Department of Gastroenterology, Royal Hallamshire Hospital, Sheffield, United Kingdom
Clin Gastroenterol Hepatol 8:433-8. 2010..We evaluated the prevalence of exocrine pancreatic insufficiency in diarrhea-predominant IBS (D-IBS) and assessed the effects of pancreatic enzyme supplementation.
- Exocrine pancreatic insufficiency, dyserythropoeitic anemia, and calvarial hyperostosis are caused by a mutation in the COX4I2 geneEyal Shteyer
The Metabolic Disease Unit, Hadassah Hebrew University Medical Center, Jerusalem, Israel
Am J Hum Genet 84:412-7. 2009Steatorrhea and malabsorption of lipid-soluble vitamins due to exocrine pancreatic insufficiency are common in patients with cystic fibrosis and are predominant in Shwachman-Bodian-Diamond, Pearson, and Johanson-Blizzard syndromes...
- Impaired ribosomal subunit association in Shwachman-Diamond syndromeNicholas Burwick
Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, WA 98109, USA
Blood 120:5143-52. 2012..In summary, we demonstrate an SBDS-dependent ribosome maturation defect in SDS patient cells. The role of ribosomal subunit joining in marrow failure warrants further investigation...
- Is exocrine pancreatic insufficiency in adult coeliac disease a cause of persisting symptoms?J S Leeds
Department of Gastroenterology, Sheffield Teaching Hospitals Trust, Sheffield, UK
Aliment Pharmacol Ther 25:265-71. 2007..Patients with coeliac disease may have diarrhoea despite being on a gluten-free diet...
- A novel hypomorphic PDX1 mutation responsible for permanent neonatal diabetes with subclinical exocrine deficiencyMarc Nicolino
Division of Pediatric Endocrinology, Hopital Femme Mere Enfant, Lyon University, Lyon, France
Diabetes 59:733-40. 2010..We studied a consanguineous family with two first cousins affected by neonatal diabetes; their four parents had a common ancestor, suggestive of a fully penetrant recessive mutation...
- Mutations in SBDS are associated with Shwachman-Diamond syndromeGraeme R B Boocock
Program in Genetics and Genomic Biology, Room 11 109A, Elm Wing Annex, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada
Nat Genet 33:97-101. 2003....
- Shwachman-Diamond syndrome neutrophils have altered chemoattractant-induced F-actin polymerization and polarization characteristicsClaudia Orelio
Sanquin Research and Landsteiner Laboratory Dept Blood Cell Research Phagocyte Laboratory, The Netherlands
Haematologica 94:409-13. 2009..Thus, Shwachman-Diamond syndrome neutrophils have aberrant chemoattractant-induced F-actin properties which might contribute to the impaired neutrophil chemotaxis...
- Clinical and molecular pathophysiology of Shwachman-Diamond syndrome: an updateKasiani C Myers
Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children s Hospital Medical Center, University of Cincinnati, 3333 Burnet Avenue, MLC 7015, Cincinnati, OH 45229, USA
Hematol Oncol Clin North Am 27:117-28, ix. 2013..This article summarizes the clinical phenotype of SDS, diagnostic and treatment approaches, and novel advances in our understanding of the molecular pathophysiology of this disease...
- Draft consensus guidelines for diagnosis and treatment of Shwachman-Diamond syndromeYigal Dror
The Hospital for Sick Children, University of Toronto, Ontario, Canada
Ann N Y Acad Sci 1242:40-55. 2011..Whenever possible, evidence-based conclusions are made, but as with other rare diseases, the data on SDS are often anecdotal. The authors welcome comments from readers...
- Feline exocrine pancreatic insufficiency: 16 cases (1992-2007)Kelley A Thompson
Department of Veterinary Clinical Sciences, School of Veterinary Medicine, Purdue University, West Lafayette, IN 47907 2026, USA
J Feline Med Surg 11:935-40. 2009Medical records of 16 cats diagnosed with exocrine pancreatic insufficiency (EPI) were reviewed...
- Management of pancreatic exocrine insufficiency: Australasian Pancreatic Club recommendationsJames Toouli
Department of Surgery, Flinders Medical Centre, Adelaide, SA, Australia
Med J Aust 193:461-7. 2010..Medium-chain triglycerides do not provide any clear nutritional advantage over long-chain triglycerides, but can be trialled in patients who fail to gain or to maintain adequate bodyweight in order to increase energy intake...
- Heritability of exocrine pancreatic insufficiency in German Shepherd dogsE Westermarck
Department of Equine and Small Animal Medicine, Faculty of Veterinary Medicine, University of Helsinki, P O Box 57, 00014 Helsinki, Finland
J Vet Intern Med 24:450-2. 2010Several studies have revealed that exocrine pancreatic insufficiency (EPI) is an inherited disease in German Shepherd Dogs (GSDs). Pedigree analyses have suggested an autosomal recessive inheritance model.
- The daily practice of pancreatic enzyme replacement therapy after pancreatic surgery: a northern European survey: enzyme replacement after surgeryEdmée C M Sikkens
Department of Gastroenterology and Hepatology, Erasmus University Medical Center, PO Box 2040, 3015 CE, Rotterdam, The Netherlands
J Gastrointest Surg 16:1487-92. 2012..Data on the practice of enzyme replacement therapy after surgery are lacking, and therefore, we conducted this analysis...
- Efficacy and safety of PANCREAZE® for treatment of exocrine pancreatic insufficiency due to cystic fibrosisBruce C Trapnell
Division of Pulmonary Medicine and Biology, Cincinnati Children s Hospital Medical Center, OH, USA
J Cyst Fibros 10:350-6. 2011Pancreatic enzyme replacement therapy (PERT) is critical for correction of exocrine pancreatic insufficiency (EPI) in patients with cystic fibrosis (CF).
- Randomised clinical trial: the efficacy and safety of pancreatin enteric-coated minimicrospheres (Creon 40000 MMS) in patients with pancreatic exocrine insufficiency due to chronic pancreatitis--a double-blind, placebo-controlled studyV Thorat
Department of Medical Gastroenterology, Poona Hospital and Research Centre, Pune, India
Aliment Pharmacol Ther 36:426-36. 2012..Pancreatic exocrine insufficiency (PEI) results in maldigestion, leading to abdominal pain, steatorrhoea, malnutrition and weight loss...
- Efficacy and tolerability of Creon for Children in infants and toddlers with pancreatic exocrine insufficiency caused by cystic fibrosis: an open-label, single-arm, multicenter studyCarla Colombo
CF Center of Milano, Fondazione IRCCS Ospedale Policlinico, Mangiagalli, Regina Elena, Milano, and University of Milan, Milan, Italy
Pancreas 38:693-9. 2009..To evaluate the efficacy and safety of a pancreatic enzyme preparation specifically developed for infants and small children with cystic fibrosis (CF)...
- Shwachman-diamond syndromeYigal Dror
Division of Haematology and Oncology, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Ontario, Canada
Br J Haematol 118:701-13. 2002
- Successful unrelated umbilical cord blood transplantation in children with Shwachman-Diamond syndromeR Vibhakar
Division of Pediatric Hematology Oncology, University of Iowa Hospitals and Clinics, Iowa City, IA 52242, USA
Bone Marrow Transplant 36:855-61. 2005..Importantly, these cases also suggest that unrelated UCB, in the absence of a matched family member, is an excellent alternative stem cell source for SDS patients undergoing HSCT...
- Nutrition in patients with cystic fibrosis: a European ConsensusM Sinaasappel
Department of Paediatric Gastroenterology, Erasmus Medical Centre, Rotterdam, The Netherlands
J Cyst Fibros 1:51-75. 2002..The purpose of the conference was to develop a consensus document on nutrition in patients with cystic fibrosis based on current evidence...
- Efficacy and tolerability of a new formulation of pancrelipase delayed-release capsules in children aged 7 to 11 years with exocrine pancreatic insufficiency and cystic fibrosis: a multicenter, randomized, double-blind, placebo-controlled, two-period crosGavin R Graff
Penn State Milton S Hershey Medical Center, Penn State Medical School, Department of Pediatrics, Hershey, Pennsylvania 17033 0850, USA
Clin Ther 32:89-103. 2010..enzyme replacement therapy (PERT) is essential for maintaining adequate nutrition in children with exocrine pancreatic insufficiency (EPI) due to cystic fibrosis (CF)...
- Exocrine pancreatic insufficiency in diabetes mellitus: a complication of diabetic neuropathy or a different type of diabetes?Philip D Hardt
Third Medical Department, University Hospital Giessen and Marburg, Giessen Site, Klinikstraße 33, 35392 Giessen, Germany
Exp Diabetes Res 2011:761950. 2011..This paper discusses the currently available studies on this subject and tries to provide an overview of the current concepts of exocrine pancreatic insufficiency in diabetes mellitus.
- A 6-month, open-label clinical trial of pancrelipase delayed-release capsules (Creon) in patients with exocrine pancreatic insufficiency due to chronic pancreatitis or pancreatic surgeryN Gubergrits
Department of Internal Diseases, Donetsk National Medical University, Donetsk, Ukraine
Aliment Pharmacol Ther 33:1152-61. 2011Pancreatic enzyme replacement therapy (PERT) is necessary to prevent severe maldigestion and unwanted weight loss associated with exocrine pancreatic insufficiency (EPI) due to chronic pancreatitis (CP) or pancreatic surgery (PS).
- Pancrelipase delayed-release capsules (CREON) for exocrine pancreatic insufficiency due to chronic pancreatitis or pancreatic surgery: A double-blind randomized trialDavid C Whitcomb
Department of Medicine, University of Pittsburgh, Pennsylvania, USA
Am J Gastroenterol 105:2276-86. 2010..standard of care to prevent maldigestion, malnutrition, and excessive weight loss in patients with exocrine pancreatic insufficiency (EPI) due to chronic pancreatitis (CP) or pancreatic surgery (PS)...
- Effect of the administration schedule on the therapeutic efficacy of oral pancreatic enzyme supplements in patients with exocrine pancreatic insufficiency: a randomized, three-way crossover studyJ E Dominguez-Munoz
Department of Gastroenterology, University Hospital of Santiago de Compostela, C Choupana s n, E 15706 Santiago de Compostela, Spain
Aliment Pharmacol Ther 21:993-1000. 2005..Oral pancreatic enzyme supplements should be properly administered in order to ensure an adequate gastric mixing with the food and simultaneous gastric emptying with the chyme...
- Pancreatic enzyme replacement therapyP Layer
Department of Medicine, Israelitic Hospital, Orchideenstieg 14, D 22297 Hamburg, Germany
Curr Gastroenterol Rep 3:101-8. 2001..Still, in most patients, lipid digestion cannot be completely normalized by current standard therapy, and future developments are needed to optimize treatment...
- Efficacy and safety of Creon 24,000 in subjects with exocrine pancreatic insufficiency due to cystic fibrosisBruce C Trapnell
Division of Pulmonary Biology, Cincinnati Children s Hospital Medical Center, and Division of Pulmonary, Sleep, and Critical Care Medicine, University of Cincinnati, 3333 Burnet Avenue, Cincinnati, OH, 45229 3039, USA
J Cyst Fibros 8:370-7. 2009Pancreatic enzyme replacement therapy is critical for adequate nutrition in cystic fibrosis (CF) patients with exocrine pancreatic insufficiency (EPI).
- Efficacy of a novel pancreatic enzyme product, EUR-1008 (Zenpep), in patients with exocrine pancreatic insufficiency due to chronic pancreatitisPhillip P Toskes
Department of Medicine, University of Florida, Gainesville, FL, USA
Pancreas 40:376-82. 2011..This study evaluated the efficacy and safety of 2 doses of ZENPEP in patients with chronic pancreatitis (CP) and exocrine pancreatic insufficiency (EPI).
- Pancreatic enzyme therapy for pancreatic exocrine insufficiencyJ Enrique Domínguez-Muñoz
Department of Gastroenterology, University Hospital of Santiago de Compostela, C Choupana, S N, E 15706 Santiago de Compostela, Spain
Curr Gastroenterol Rep 9:116-22. 2007..The present article critically reviews current therapeutic approaches to pancreatic exocrine insufficiency...
- Genetic analysis of Shwachman-Diamond syndrome: phenotypic heterogeneity in patients carrying identical SBDS mutationsTakako Kawakami
Department of Pediatrics, Yamagata University School of Medicine, Yamagata, Japan
Tohoku J Exp Med 206:253-9. 2005..Further study will be required to explain the clinical heterogeneity...
- Fecal pancreatic elastase: a reproducible marker for severe exocrine pancreatic insufficiencySatoru Naruse
Department of Gastroenterology, Nagoya University Graduate School of Medicine, Nagoya, Japan
J Gastroenterol 41:901-8. 2006....
- MRI and MRCP findings of the pancreas in patients with diabetes mellitus: compared analysis with pancreatic exocrine function determined by fecal elastase 1Mehmet Bilgin
Department of Radiology, University Hospital Giessen and Marburg, Giessen, Germany
J Clin Gastroenterol 43:165-70. 2009....
- Fecal elastase-1 determination: 'gold standard' of indirect pancreatic function tests?S Luth
Dept of Medicine II Gastroenterology Hepatology, University Hospital of Heidelberg at Mannheim, Germany
Scand J Gastroenterol 36:1092-9. 2001..The aim of our study was to re-evaluate the sensitivity and specificity of elastase-1 and chymotrypsin in stool in the assessment of exocrine pancreatic insufficiency.
- Prevalence and determinants of exocrine pancreatic insufficiency among older adults: results of a population-based studyDietrich Rothenbacher
Department of Epidemiology, German Centre for Research on Ageing, University of Heidelberg, Heidelberg, Germany
Scand J Gastroenterol 40:697-704. 2005The prevalence and main determinants of exocrine pancreatic insufficiency were investigated in a large population-based sample of older adults by measuring pancreatic elastase-1 in stool.
- Giardiasis aggravates malabsorption in cystic fibrosisJ Walkowiak
Institute of Pediatrics, Dept of Gastroenterology and Metabolism, Karol Marcinkowski University of Medical Sciences, Poznan, Poland
Scand J Gastroenterol 39:607-8. 2004
- Follow-up of exocrine pancreatic function in type-1 diabetes mellitusWerner Creutzfeldt
Department of Internal Medicine, Division of Gastroenterology and Metabolism, University of Gottingen, Gottingen, Germany
Digestion 72:71-5. 2005In a previous study, mild to moderate exocrine pancreatic insufficiency, as measured by the secretin-pancreozymin test, was found in 23 (43%) of 53 patients with type-1 diabetes mellitus...
- Fecal chymotrypsin and elastase-1 determination on one single stool collected at random: diagnostic value for exocrine pancreatic statusIsabelle Molinari
Biochemistry Laboratory, Hopital Pellegrin, Bordeaux, France
Clin Biochem 37:758-63. 2004..The aim of our study was to investigate the diagnostic advantage from measuring fecal proteases in stool samples collected for two or three consecutive days in comparison to one single stool sample collected at random...
- [The diagnostic validity of non-invasive pancreatic function tests--a meta-analysis]E Siegmund
Institut fur Klinische Chemie und Pathobiochemie, Medizinische Fakultät der Universität Rostock
Z Gastroenterol 42:1117-28. 2004The paper discusses the non-invasive (tubeless) pancreatic function tests used to diagnose exocrine pancreatic insufficiency (EI)...
- Optimising the therapy of exocrine pancreatic insufficiency by the association of a proton pump inhibitor to enteric coated pancreatic extractsJ E Dominguez-Munoz
Gut 55:1056-7. 2006
- Comparing the urinary pancreolauryl ratio and faecal elastase-1 as indicators of pancreatic insufficiency in clinical practiceD A Elphick
Department of Gastroenterology, Barnsley District General Hospital, Barnsley, UK
Pancreatology 5:196-200. 2005..more recently, the faecal pancreatic elastase-1, are widely used for the noninvasive diagnosis of exocrine pancreatic insufficiency. Both tests have previously been validated against 'gold standard' tests of pancreatic function, but ..
- Update on the evaluation of pancreatic exocrine status in cystic fibrosisDrucy Borowitz
Department of Clinical Pediatrics, State University of New York at Buffalo, New York 14222, USA
Curr Opin Pulm Med 11:524-7. 2005..This paper reviews recent progress in the development of noninvasive, indirect tests of pancreatic function for use as diagnostic tools for patients with cystic fibrosis...
- Diabetes and exocrine pancreatic insufficiency in E2F1/E2F2 double-mutant miceAinhoa Iglesias
Department of Genetics, Physical Anthropology and Animal Physiology, Faculty of Sciences, University of the Basque Country, Bilbao, Spain
J Clin Invest 113:1398-407. 2004..These results suggest that E2F1/E2F2 activity negatively controls growth of mature pancreatic cells and is necessary for the maintenance of differentiated pancreatic phenotypes in the adult...
- Identification of novel mutations in patients with Shwachman-Diamond syndromeElena Nicolis
Laboratory of Molecular Pathology, Azienda Ospedaliera di Verona, Verona, Italy
Hum Mutat 25:410. 2005Shwachman-Diamond syndrome (SDS) is a rare autosomal recessive disease, mainly characterized by exocrine pancreatic insufficiency, hematological dysfunction and skeletal abnormalities...
- Maldigestion associated with exocrine pancreatic insufficiency: implications of gastrointestinal physiology and properties of enzyme preparations for a cause-related and patient-tailored treatmentM J Bruno
Division of Gastroenterology and Hepatology, University of Amsterdam, The Netherlands
Am J Gastroenterol 90:1383-93. 1995Clinical conditions in which secondary maldigestion associated with exocrine pancreatic insufficiency occur include chronic pancreatitis, cystic fibrosis, pancreatic cancer, partial or total gastrectomy, and pancreatic resection...
- A double-blind, randomized, multicentre, crossover study to prove equivalence of pancreatin minimicrospheres versus microspheres in exocrine pancreatic insufficiencyU Halm
Department of Internal Medicine II, University of Leipzig, Germany
Aliment Pharmacol Ther 13:951-7. 1999..There are, however, no clinical trials comparing different sizes of pancreatin microspheres with regard to fat excretion and fat intake...
- Comparative evaluation of a high lipase pancreatic enzyme preparation and a standard pancreatic supplement for treating exocrine pancreatic insufficiency in chronic pancreatitisM Delhaye
Medicosurgical Department of Gastroenterology, Erasme Hospital, Free University of Brussels, Belgium
Eur J Gastroenterol Hepatol 8:699-703. 1996....
- Novel SBDS mutations caused by gene conversion in Japanese patients with Shwachman-Diamond syndromeEiji Nakashima
Laboratory for Bone and Joint Diseases, SNP Research Center, RIKEN, c o Institute of Medical Science, University of Tokyo, 4 6 1 Shirokanedai, 108 8639 Minato ku, Tokyo, Japan
Hum Genet 114:345-8. 2004Shwachman-Diamond syndrome (SDS; OMIM 260400) is an autosomal recessive disorder characterized by exocrine pancreatic insufficiency, bone marrow dysfunction and metaphyseal chondrodysplasia...
- Gastric acid suppression and treatment of severe exocrine pancreatic insufficiencyE P Dimagno
Department of Internal Medicine, Mayo Clinic, 200 First Street S.W, Rochester, MN 55905, USA
Best Pract Res Clin Gastroenterol 15:477-86. 2001..This amount of lipolytic activity taken with an agent that suppresses gastric acid secretion improves fat absorption in most patients and may even abolish steatorrhoea...
- A novel CFTR mutation found in a Chinese patient with cystic fibrosisNan Li
Department of Pulmonary Medicine, Peking University First Hospital, Beijing 100034, China
Chin Med J (Engl) 119:103-9. 2006..Cystic fibrosis (CF) is rare in Chinese. We investigated the mutations in the gene of cystic fibrosis transmembrane conductance regulator (CFTR) in a Chinese CF patient and reviewed the clinical features, gene mutations in Chinese CF cases...
- Effects of diet on clinical signs of exocrine pancreatic insufficiency in dogsElias Westermarck
Department of Clinical Veterinary Sciences, Faculty of Veterinary Medicine, University of Helsinki, Hämeentie 57, 00014 Finland
J Am Vet Med Assoc 228:225-9. 2006To assess the effects of dietary modification on clinical signs of exocrine pancreatic insufficiency (EPI) in dogs.
- Pancreatic enzyme therapy and clinical outcomes in patients with cystic fibrosisSusan S Baker
Department of Pediatrics, Women and Children s Hospital of Buffalo, State University of New York at Buffalo, USA
J Pediatr 146:189-93. 2005..To assess the relationship between pancreatic enzyme therapy (PET) and the clinical outcomes of growth, abdominal pain, constipation, gassiness, and number of stools in cystic fibrosis (CF)...
- Ultrase MT12 and Ultrase MT20 in the treatment of exocrine pancreatic insufficiency in cystic fibrosis: safety and efficacyM W Konstan
Rainbow Babies and Children s Hospital, Cleveland, OH 44106, USA
Aliment Pharmacol Ther 20:1365-71. 2004Cystic fibrosis causes exocrine pancreatic insufficiency, leading to malabsorption. Supplemental pancreatic enzyme therapy alleviates the concomitant malnutrition experienced by cystic fibrosis patients...
- Use of fecal elastase-1 to classify pancreatic status in patients with cystic fibrosisDrucy Borowitz
Department of Pediatrics, Women and Children s Hospital of Buffalo, State University of New York at Buffalo, 219 Bryant Street, Buffalo, NY 14222, USA
J Pediatr 145:322-6. 2004..To test the hypothesis that some patients with cystic fibrosis (CF) are misclassified as pancreatic insufficient, using fecal elastase-1 (FE-1) to define pancreatic status...
- Allogeneic bone marrow transplantation in Shwachman-Diamond syndrome with malignant myeloid transformation. A case reportSo Young Park
Department of Internal Medicine, Inha University Hospital, Inchon, 7 206, 3ga, Shinheung Dong, Choong Gu, Inchon 400 711, Korea
Korean J Intern Med 17:204-6. 2002Shwachman-Diamond syndrome (SDS) is a rare genetic disorder of unknown pathogenesis involving exocrine pancreatic insufficiency and hematological and skeletal abnormalities. About 25% of patients develop hematopoietic malignancies...
- Prognostic factors in canine exocrine pancreatic insufficiency: prolonged survival is likely if clinical remission is achievedDaniel J Batchelor
Small Animal Hospital, Department of Veterinary Clinical Science, University of Liverpool, UK
J Vet Intern Med 21:54-60. 2007Response to therapy in canine exocrine pancreatic insufficiency (EPI) varies considerably, making it difficult to determine prognosis for individual patients.
- Breed associations for canine exocrine pancreatic insufficiencyDaniel J Batchelor
Small Animal Hospital, Department of Veterinary Clinical Science, University of Liverpool, Crown Street, Liverpool L7 7EX, UK
J Vet Intern Med 21:207-14. 2007..Knowledge of breed associations is valuable to clinicians and researchers investigating diseases with a genetic basis...
- Assessment of maldigestion in cystic fibrosisJaroslaw Walkowiak
J Pediatr 145:285-7. 2004
- Systematic review: efficacy and safety of pancreatic enzyme supplements for exocrine pancreatic insufficiencyJ R Taylor
Division of Gastroenterology, University of Michigan Medical Center, Ann Arbor, MI 48105, USA
Aliment Pharmacol Ther 31:57-72. 2010Pancreatic enzyme supplements are standard therapy for fat malabsorption in patients with exocrine pancreatic insufficiency. The FDA determined that published data are insufficient to support the efficacy and safety of these agents.
- Magnetic resonance imaging findings of the pancreas in patients with Shwachman-Diamond syndrome and mutations in the SBDS geneSanna Toiviainen-Salo
Medical Imaging Center, Helsinki University Hospital, Helsinki, Finland
J Pediatr 152:434-6. 2008..The findings suggest that patients with mutations in the SBDS gene have a characteristic magnetic resonance imaging pattern of fat-replaced pancreas and that SBDS mutations are unlikely in patients without this pattern...
- Pancreatic enzyme replacement therapy in chronic pancreatitisE C M Sikkens
Department of Gastroenterology and Hepatology, Erasmus Medical Center, Rotterdam, The Netherlands
Best Pract Res Clin Gastroenterol 24:337-47. 2010b>Exocrine pancreatic insufficiency (EPI) is a serious condition which occurs in several diseases including chronic pancreatitis (CP), cystic fibrosis, pancreatic cancer, and as a result of pancreatic surgery...
- Successful unrelated donor bone marrow transplantation for Shwachman-Diamond syndrome with leukemiaTetsuo Mitsui
Department of Pediatrics, Yamagata University School of Medicine, Yamagata, Japan
Int J Hematol 79:189-92. 2004Shwachman-Diamond syndrome (SDS) is a rare congenital disorder featuring exocrine pancreatic insufficiency, growth retardation, and bone marrow dysfunction. Reports suggest that nearly 25% of all cases are complicated with leukemia...
- Low fecal elastase 1 levels do not indicate exocrine pancreatic insufficiency in type-1 diabetes mellitusJan Uwe Hahn
Clinic for Diabetes and Metabolic Diseases, Klinikum Karlsburg, Karlsburg, Germany
Pancreas 36:274-8. 2008..the basis of very low fecal elastase 1 and very high fecal fat estimations, it has been claimed that exocrine pancreatic insufficiency is frequent in diabetic patients, and that in up to 40% of the patients, pancreatic enzyme ..
- Exocrine pancreas disfunction in severely traumatised patients and early enteral nutritionMetin Senkal
Department of General Surgery, Marien Hospital Witten, Germany
Ulus Travma Acil Cerrahi Derg 14:34-9. 2008We investigated exocrine pancreatic insufficiency in severely traumatised patients with enteral nutrition using the fecal elastase-1 concentration.
- Nutritional management of cystic fibrosis patientsDaina Kalnins
Respiratory Medicine, The Research Institute, Division of Gastroenterology and Nutrition, Department of Pediatrics, Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, Ontario, Canada
Curr Opin Clin Nutr Metab Care 10:348-54. 2007..We critique recent advances in nutritional care of patients with cystic fibrosis focusing on our understanding of the role of fecal elastase as a pancreatic function test, enzyme replacement therapy and bone health...
- Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic reviewVirginia A Stallings
Division of Gastroenterology, Hepatology, and Nutrition, The Children s Hospital of Philadelphia, 3535 Market St, Philadelphia, PA 19104, USA
J Am Diet Assoc 108:832-9. 2008..These Registry data-based recommendations are presented...
- Pancreatic exocrine insufficiency is not common in HNF-1alpha MODYT Klupa
Department of Metabolic Diseases, Jagiellonian University, Medical College, Krakow, Poland
Diabet Med 25:627-30. 2008b>Exocrine pancreatic insufficiency has been described in Type 1 and Type 2 diabetes...
- The effect of zinc supplements in cystic fibrosis patientsS Van Biervliet
Pediatric Department, UZ Ghent, Ghent, Belgium
Ann Nutr Metab 52:152-6. 2008..To study the effect of Zn supplements in cystic fibrosis (CF) on disease evolution...
- Does the pancreas really produce much more lipase than required for fat digestion?Frederic Carriere
Laboratory of Enzymology at Interfaces and Physiology of Lipolysis, CNRS UPR 9025 IBSM, Marseille, France
JOP 6:206-15. 2005....
- Monoclonal versus polyclonal ELISA for assessment of fecal elastase concentration: pitfalls of a new assayArne Schneider
Medical Department I, University Hospital, Frankfurt Main, Germany
Clin Chem 51:1052-4. 2005
- Safety and preliminary clinical activity of a novel pancreatic enzyme preparation in pancreatic insufficient cystic fibrosis patientsDrucy Borowitz
CF Therapeutics Development Network, Seattle, Wash, USA
Pancreas 32:258-63. 2006..We conducted a phase 1 study of a novel pancreatic enzyme product, TheraCLEC-Total (TCT), a proprietary formulation of microbial-derived lipase, protease, and amylase, to determine its safety and preliminary efficacy in cystic fibrosis...
- Biliary versus alcohol-related infected pancreatic necrosis: similarities and differences in the follow-upJacek Reszetow
Department of General, Endocrine, and Transplant Surgery, Medical University of Gdansk, Poland
Pancreas 35:267-72. 2007..Data concerning survivors' quality of life and pancreatic functions are scarce. Follow-up of the patients with alcohol and biliary etiology of IPN treated with open necrosectomy was performed...
- Enteric-coated pancreatic enzyme with bicarbonate is equal to standard enteric-coated enzyme in treating malabsorption in cystic fibrosisDaina Kalnins
Division of Respiratory Medicine, Department of Pediatrics, The Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada
J Pediatr Gastroenterol Nutr 42:256-61. 2006..5 mEq of bicarbonate per capsule with a conventional enteric-coated enzyme (EC-PE) capsule in cystic fibrosis patients with signs or symptoms of moderate to severe malabsorption...
- [Pancreatic exocrine function in diabetes mellitus. Determination of fecal elastase]Carla Mancilla A
Secciones de Gastroenterología, Departamento de Medicina, Hospital Clinico, Universidad de Chile
Rev Med Chil 134:407-14. 2006..One of the complications of diabetes mellitus is the development of pancreatic exocrine insufficiency...
- An enteric-coated high-buffered pancrelipase reduces steatorrhea in patients with cystic fibrosis: a prospective, randomized studyMary Sue Brady
Indiana University School of Health and Rehabilitation Sciences, Indianapolis, IN 46202 5119, USA
J Am Diet Assoc 106:1181-6. 2006..Enteric-coated (EC) high-buffered (2.5 mEq [2.5 mmol] bicarbonate per capsule) pancrelipase microsphere enzymes were compared to EC-nonbuffered pancreatic enzymes for efficacy in reducing steatorrhea in patients with cystic fibrosis...
- Study of a novel pancreatic enzyme replacement therapy in pancreatic insufficient subjects with cystic fibrosisDrucy Borowitz
Women and Children s Hospital of Buffalo, Division of Pediatric Pulmonology, State University of New York at Buffalo, Buffalo, NY 14222, USA
J Pediatr 149:658-662. 2006....
- Re: fecal elastase: pancreatic status verification and influence on nutritional status in children with cystic fibrosisJaroslaw Walkowiak
J Pediatr Gastroenterol Nutr 42:117; author reply 118. 2006
- Comparison of monoclonal and polyclonal ELISAs for fecal elastase in patients with cystic fibrosis and pancreatic insufficiencyDrucy Borowitz
Department of Pediatrics, State University of New York at Buffalo, NY 14222, USA
J Pediatr Gastroenterol Nutr 44:219-23. 2007..We sought to compare these assays in patients with cystic fibrosis and to correlate these with the coefficient of fat absorption (CFA)...
- Pancreatic enzyme therapy and gastrointestinal symptoms in patients with cystic fibrosisJaroslaw Walkowiak
J Pediatr 147:870-1. 2005
- Diagnosis and treatment of intestinal malabsorption in cystic fibrosisJames M Littlewood
Regional Paediatric Cystic Fibrosis Unit, St James s University Hospital, Leeds, UK
Pediatr Pulmonol 41:35-49. 2006....
- Pancreatic enzyme pharmacotherapyMarcus Ferrone
Department of Clinical Pharmacy, Drug Product Services Laboratory, University of California, San Francisco, California 94118 0613, USA
Pharmacotherapy 27:910-20. 2007..Despite the large number of specialty enzyme replacements available commercially, many patients remain dissatisfied with standard therapy, and future developments are needed to optimize treatment in these individuals...
- Gastrointestinal outcomes and confounders in cystic fibrosisDrucy Borowitz
J Pediatr Gastroenterol Nutr 41:273-85. 2005
- [Chronic pancreatitis. Main symptoms: chronic abdominal pain, weight loss in steatorrhea, secondary diabetes mellitus]G Schulthess
Medizinische Poliklinik, Universitatsspital Zurich
Praxis (Bern 1994) 94:1139-45. 2005
- [Fecal elastase-1: a useful test in pediatric practice]L David-Henriau
Unité de Gastroentérologie Pédiatrique, Departement de Pediatrie, Centre Hospitalier Pellegrin, CHU de Bordeaux, Place Amelie Raba Leon, 33076, Bordeaux Cedex, France
Arch Pediatr 12:1221-5. 2005..To study fecal elastase-1 (E1F) and chymotrypsin (ChT) in stools for the diagnosis of pancreatic insufficiency in pediatric practice...
- Fecal elastase: pancreatic status verification and influence on nutritional status in children with cystic fibrosisJacqueline R Cohen
Division of Gastroenterology and Nutrition, The Children s Hospital of Philadelphia, Pennsylvania, USA
J Pediatr Gastroenterol Nutr 40:438-44. 2005..To evaluate fecal elastase 1 (FE) levels in young children with cystic fibrosis and pancreatic insufficiency and to explore the relationship between FE and growth, nutrition, pulmonary status and fat absorption over a 24-month period...
- Fecal elastase-1 concentration: an indirect test of exocrine pancreatic function and a marker of an enteropathy regardless of causeSanda Nousia-Arvanitakis
J Pediatr Gastroenterol Nutr 36:314-5. 2003
- Omeprazole, a proton pump inhibitor, improves residual steatorrhoea in cystic fibrosis patients treated with high dose pancreatic enzymesMarijke Proesmans
Department of Paediatrics, University Hospital Gasthuisberg, Herestraat 49, 3000 Leuven, Belgium
Eur J Pediatr 162:760-3. 2003..5-16.5 g/day) to 5.5 g (quartiles 4.9-8.1 g/day) (P<0.01). The same improvement was noted when fat absorption was calculated: 87% (quartiles 81-89%) without versus 94% (quartiles 90-96%) with omeprazole (P<0.001)...
- Pancreatic elastase 1 in feces of preterm and term infantsK Nissler
Children's Hospital, Technical University of Dresden, Fetscherstr. 74, D-01307 Dresden, Germany
J Pediatr Gastroenterol Nutr 33:28-31. 2001..CONCLUSIONS: The adult reference value for pancreatic E1 of greater than 200 microg/g feces can be applied to infants older than 2 weeks, independent of gestational age, birth weight, and the type of nutrition...
- Comparison of steatocrit and fat absorption in persons with cystic fibrosisMary H Wagner
Department of Pediatrics, University of Florida, Gainesville, Florida 32610, USA
J Pediatr Gastroenterol Nutr 35:202-5. 2002b>Exocrine pancreatic insufficiency is a common problem in persons with cystic fibrosis causing malabsorption and poor growth. The 72-hour fecal fat study is the best qualitative measure of fat malabsorption used in clinical practice...
- Heat wave and acute pancreatitis: very unusual cystic fibrosis presentationAnne Munck
Pediatrics 113:1846. 2004
- Genotype/phenotype correlation of the G85E mutation in a large cohort of cystic fibrosis patientsK Decaestecker
Dept of Pediatrics, University of Leuven, Leuven, Belgium
Eur Respir J 23:679-84. 2004..Therefore, G85E is a class II mutation. Although there is variability in its clinical presentation, G85E mutation results in a severe phenotype...
- Now that fecal elastase is available in the United States, should clinicians start using it?Paul G Lankisch
Clinic of Internal Medicine, Municipal Clinic of Lueneburg, Boegelstrasse 1, D 21339 Lueneburg, Germany
Curr Gastroenterol Rep 6:126-31. 2004Fecal elastase-1 estimation is an alternative fecal enzyme test for the detection of exocrine pancreatic insufficiency, which, in contrast to chymotrypsin estimation, uses monoclonal antibodies against human pancreatic elastase and is ..
- Exocrine pancreatic insufficiency and its consequences on physical development and metabolism in children and adolescents with type 1 diabetes mellitusMartin W Laass
Children s Hospital, Technical University Dresden, Fetscherstrasse 74, 01307, Dresden, Germany
Eur J Pediatr 163:681-2. 2004
- The commercially available ELISA for pancreatic elastase 1 based on polyclonal antibodies does measure an as yet unknown antigen different from purified elastase 1. Binding studies and clinical use in patients with exocrine pancreatic insufficiencyP D Hardt
Third Medical Department and Policlinic, Giessen University Hospital, Giessen, Germany
Z Gastroenterol 41:903-6. 2003..ELISA 2 should be renamed, since obviously it does react with an antigen (antigens) different from elastase 1...
- Fecal elastase-1 as a test for pancreatic function: a reviewRoberto Dominici
Istituto di Scienze Biomediche, Ospedale Luigi Sacco, Milano, Italy
Clin Chem Lab Med 40:325-32. 2002..It shows higher sensitivity and specificity for exocrine pancreatic insufficiency than fecal chymotrypsin determination and is comparable to oral pancreatic function tests such as ..
- Faecal elastase-1: lyophilization of stool samples prevents false low results in diarrhoeaB Fischer
Dept. of Medicine 1, , Erlangen, Germany
Scand J Gastroenterol 36:771-4. 2001BACKGROUND: In patients with diarrhoea, faecal elastase-1 is used to detect exocrine pancreatic insufficiency. Diarrhoea is defined as >85% stool water content...
- Low fecal elastase: potentially related to transient small bowel damage resulting from enteric pathogensSilvia Salvatore
Clinica Pediatrica Universitaria, Universita dell Insubria, Varese, Italy
J Pediatr Gastroenterol Nutr 36:392-6. 2003..However, enteropathy may theoretically cause decreased exocrine pancreatic enzyme secretion through alteration of enteric hormone release...
- Molecular consequences of cystic fibrosis transmembrane regulator (CFTR) gene mutations in the exocrine pancreasN Ahmed
Division of Gastroenterology and Nutrition, The Hospital for Sick Children, Toronto, Canada
Gut 52:1159-64. 2003..We tested the hypothesis that the actual or predicted consequences of mutations in the cystic fibrosis transmembrane regulator gene correlate with the pancreatic phenotype and with measures of quantitative exocrine pancreatic function...
- Fecal elastase-1 is superior to fecal chymotrypsin in the assessment of pancreatic involvement in cystic fibrosisJaroslaw Walkowiak
Institute of Pediatrics, Karol Marcinkowski University of Medical Sciences, Poznan, Poland
Pediatrics 110:e7. 2002..Therefore, the aim of the present study was to compare the sensitivity and the specificity of the fecal elastase-1 (E1) test with the fecal chymotrypsin (ChT) test in a large cohort of CF patients and healthy subjects (HS)...
- [Comparison of faecal lipase test and faecal elastase-1 test in the assessment of exocrine pancreatic function in cystic fibrosis]Jaroslaw Walkowiak
Klinika Gastroenterologii Dzieciecej i ChorÃ³b Metabolicznych, Instytut Pediatrii, Akademia Medyczna, ul Szpitalna 27 33, 60 572 PoznaÅ„, Poland
Med Wieku Rozwoj 7:149-55. 2003..Therefore, the aim of the present study was to compare the sensitivity and the specificity of faecal lipase test to the faecal elastase-1 test in the assessment of exocrine pancreatic function...
- Pathological Evaluation of a Cystic Fibrosis Ferret ModelJohn F Engelhardt; Fiscal Year: 2010..large part due to the lack of a CF animal model that mimics progression of human CF lung disease and exocrine pancreatic insufficiency (CFTR deficient mice lack both these properties)...
- 6th International Conference on SBDSSridhar Mani; Fiscal Year: 2011..Clinical manifestations include exocrine pancreatic insufficiency, neurocognitive dysfunction, bone marrow failure, and leukemia predisposition...
- 4th International Congress on Shwachman-Diamond SyndromeRichard Grand; Fiscal Year: 2007..Characteristic clinical features include exocrine pancreatic insufficiency, bone marrow failure, and leukemia predisposition...
- BORDERLINE PANCREATIC FUNCTION IN CYSTIC FIBROSISMorteza Janghorbani; Fiscal Year: 2000..this proposed project are to develop a noninvasive procedure for accurate measurement of borderline exocrine pancreatic insufficiency in cystic fibrosis (CF) patients and other disorders affecting exocrine pancreatic function...
- NUTRITIONAL STATUS OF CF PULMONARY DISEASEVirginia Stallings; Fiscal Year: 2001..These data will provide the scientific basis for early and effective nutritional intervention to promote normal growth and body composition as standard CF care. ..
- AAT Deficiency & Liver Diseases Caused by ProteinsBruce Trapnell; Fiscal Year: 2006..unreadable] [unreadable]..
- Fourth Annual International Pulmonary Alveolar Proteinosis Research ConferenceBruce Trapnell; Fiscal Year: 2006..unreadable] [unreadable] [unreadable] [unreadable]..
- Pulmonary Alveolar Proteinosis Research ConferenceBruce Trapnell; Fiscal Year: 2005..This conference will provide a forum where thought leaders focused on the molecular and cellular basis or clinical management of PAP will present cutting edge research, integrate concepts, and chart innovative research directions. ..
- Regulation of Alveolar Macrophage PhagocytosisBruce Trapnell; Fiscal Year: 2006..1, and Hck/SFKs in AMs. Results of this research are expected to help establish the feasibility of the therapeutic use of GM-CSF in the treatment of lung infections by a wide variety of microbial pathogens. ..
- Conference--Pulmonary Alveolar Proteinosis ResearchBruce Trapnell; Fiscal Year: 2004..Recent progress in PAP research represents a paradigm of the intersection of basic science, clinical medicine and translational research, which has taken this disease from obscurity to clarity in a decade. ..
- GM-CSF & Alveolar Macrophage Antiviral Lung DefenseBruce Trapnell; Fiscal Year: 2004..abstract_text> ..
- 14th Internatinal Symposium on Inherited DiseasesDavid Whitcomb; Fiscal Year: 2004..The format, venue, and content are designed to maximize participation, education, translation and implementation of the key elements of the many new findings in the genetics of the pancreas. ..
- Intralumental Effect on Cholesterol Absorption/SynthesisJames Heubi; Fiscal Year: 2009..Integration of animal/human techniques will provide tools to characterize the role of modifications of the intraluminal environment on cholesterol solubilization and human cholesterol absorption and synthesis. ..
- Noninvasive biomarkers of proteolytic activity in CFScott Sagel; Fiscal Year: 2007..In addition, he will actively participate and train in the Pediatric GCRC, and frequently interact with his sponsor, mentors, and collaborators. ..
- NAPS2 Continuation - Genome-Wide Association Study of PancreatitisDavid C Whitcomb; Fiscal Year: 2010..Knowledge of the genetic basis of pancreatitis will lead to new treatments, and help remove the stigma that chronic pancreatitis is only a disease of drunkards. ..
- MOLECULAR GENETICS OF HEREDITARY PANCREATITISDavid Whitcomb; Fiscal Year: 2008..The HP and familial pancreatitis studies are especially important because of the statistical power of family studies and the ability to detect modifier genes in a uniform background of susceptibility factors. ..
- Clinical Applications of a Novel Fanconi Anemia AssayAkiko Shimamura; Fiscal Year: 2007..abstract_text> ..
- NOS GENE TRANSFER IN PULMONARY HYPERTENSIONDavid Rodman; Fiscal Year: 2002....
- EXTRAMURAL FACILITIES IMPROVEMENT PROGRAMRobert Golden; Fiscal Year: 2004....
- HYPOXIA AND ET-1 EFFECT ON PA SMC EFFECT ON CHANNELSDavid Rodman; Fiscal Year: 2002....