exocrine pancreatic insufficiency

Summary

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.

Top Publications

  1. ncbi High prevalence of exocrine pancreatic insufficiency in diabetes mellitus. A multicenter study screening fecal elastase 1 concentrations in 1,021 diabetic patients
    Philip D Hardt
    Third Medical Department and Policlinic, Giessen University Hospital, Giessen, Germany
    Pancreatology 3:395-402. 2003
  2. ncbi Screening for pancreatic exocrine insufficiency in patients with diabetes mellitus
    Amadeu 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
  3. ncbi Vegetarian diet alters the assessment of exocrine pancreatic function with the use of fecal tests
    Jaroslaw Walkowiak
    Institute of Pediatrics, Karol Marcinkowski University of Medical Sciences, Poznan, Poland
    J Pediatr Gastroenterol Nutr 38:224-6. 2004
  4. doi Some patients with irritable bowel syndrome may have exocrine pancreatic insufficiency
    John S Leeds
    Department of Gastroenterology, Royal Hallamshire Hospital, Sheffield, United Kingdom
    Clin Gastroenterol Hepatol 8:433-8. 2010
  5. doi 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 cros
    Gavin 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
  6. ncbi 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 study
    Carla Colombo
    CF Center of Milano, Fondazione IRCCS Ospedale Policlinico, Mangiagalli, Regina Elena, Milano, and University of Milan, Milan, Italy
    Pancreas 38:693-9. 2009
  7. ncbi Efficacy and safety of Creon 24,000 in subjects with exocrine pancreatic insufficiency due to cystic fibrosis
    Bruce 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. 2009
  8. doi 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 surgery
    N Gubergrits
    Department of Internal Diseases, Donetsk National Medical University, Donetsk, Ukraine
    Aliment Pharmacol Ther 33:1152-61. 2011
  9. ncbi 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
  10. pmc Exocrine pancreatic insufficiency, dyserythropoeitic anemia, and calvarial hyperostosis are caused by a mutation in the COX4I2 gene
    Eyal Shteyer
    The Metabolic Disease Unit, Hadassah Hebrew University Medical Center, Jerusalem, Israel
    Am J Hum Genet 84:412-7. 2009

Detail Information

Publications204 found, 100 shown here

  1. ncbi High prevalence of exocrine pancreatic insufficiency in diabetes mellitus. A multicenter study screening fecal elastase 1 concentrations in 1,021 diabetic patients
    Philip 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...
  2. ncbi Screening for pancreatic exocrine insufficiency in patients with diabetes mellitus
    Amadeu 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...
  3. ncbi Vegetarian diet alters the assessment of exocrine pancreatic function with the use of fecal tests
    Jaroslaw Walkowiak
    Institute of Pediatrics, Karol Marcinkowski University of Medical Sciences, Poznan, Poland
    J Pediatr Gastroenterol Nutr 38:224-6. 2004
  4. doi Some patients with irritable bowel syndrome may have exocrine pancreatic insufficiency
    John 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.
  5. doi 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 cros
    Gavin 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)...
  6. ncbi 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 study
    Carla 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)...
  7. ncbi Efficacy and safety of Creon 24,000 in subjects with exocrine pancreatic insufficiency due to cystic fibrosis
    Bruce 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. 2009
    Pancreatic enzyme replacement therapy is critical for adequate nutrition in cystic fibrosis (CF) patients with exocrine pancreatic insufficiency (EPI).
  8. doi 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 surgery
    N Gubergrits
    Department of Internal Diseases, Donetsk National Medical University, Donetsk, Ukraine
    Aliment Pharmacol Ther 33:1152-61. 2011
    Pancreatic 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).
  9. ncbi 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...
  10. pmc Exocrine pancreatic insufficiency, dyserythropoeitic anemia, and calvarial hyperostosis are caused by a mutation in the COX4I2 gene
    Eyal Shteyer
    The Metabolic Disease Unit, Hadassah Hebrew University Medical Center, Jerusalem, Israel
    Am J Hum Genet 84:412-7. 2009
    Steatorrhea 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...
  11. doi Pancrelipase delayed-release capsules (CREON) for exocrine pancreatic insufficiency due to chronic pancreatitis or pancreatic surgery: A double-blind randomized trial
    David 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)...
  12. doi Efficacy of a novel pancreatic enzyme product, EUR-1008 (Zenpep), in patients with exocrine pancreatic insufficiency due to chronic pancreatitis
    Phillip 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).
  13. pmc 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.
  14. ncbi Pancreatic enzyme replacement therapy in chronic pancreatitis
    E C M Sikkens
    Department of Gastroenterology and Hepatology, Erasmus Medical Center, Rotterdam, The Netherlands
    Best Pract Res Clin Gastroenterol 24:337-47. 2010
    b>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...
  15. ncbi Mutations in SBDS are associated with Shwachman-Diamond syndrome
    Graeme 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
    ....
  16. ncbi Management of pancreatic exocrine insufficiency: Australasian Pancreatic Club recommendations
    James 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...
  17. ncbi Nutrition in patients with cystic fibrosis: a European Consensus
    M 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...
  18. doi Systematic review: efficacy and safety of pancreatic enzyme supplements for exocrine pancreatic insufficiency
    J R Taylor
    Division of Gastroenterology, University of Michigan Medical Center, Ann Arbor, MI 48105, USA
    Aliment Pharmacol Ther 31:57-72. 2010
    Pancreatic 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.
  19. ncbi Breed associations for canine exocrine pancreatic insufficiency
    Daniel 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...
  20. pmc The daily practice of pancreatic enzyme replacement therapy after pancreatic surgery: a northern European survey: enzyme replacement after surgery
    Edmée 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...
  21. ncbi Prognostic factors in canine exocrine pancreatic insufficiency: prolonged survival is likely if clinical remission is achieved
    Daniel J Batchelor
    Small Animal Hospital, Department of Veterinary Clinical Science, University of Liverpool, UK
    J Vet Intern Med 21:54-60. 2007
    Response to therapy in canine exocrine pancreatic insufficiency (EPI) varies considerably, making it difficult to determine prognosis for individual patients.
  22. ncbi Heritability of exocrine pancreatic insufficiency in German Shepherd dogs
    E 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. 2010
    Several 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.
  23. ncbi Effects of diet on clinical signs of exocrine pancreatic insufficiency in dogs
    Elias 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. 2006
    To assess the effects of dietary modification on clinical signs of exocrine pancreatic insufficiency (EPI) in dogs.
  24. doi Efficacy and safety of PANCREAZE® for treatment of exocrine pancreatic insufficiency due to cystic fibrosis
    Bruce C Trapnell
    Division of Pulmonary Medicine and Biology, Cincinnati Children s Hospital Medical Center, OH, USA
    J Cyst Fibros 10:350-6. 2011
    Pancreatic enzyme replacement therapy (PERT) is critical for correction of exocrine pancreatic insufficiency (EPI) in patients with cystic fibrosis (CF).
  25. doi Magnetic resonance imaging findings of the pancreas in patients with Shwachman-Diamond syndrome and mutations in the SBDS gene
    Sanna 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...
  26. pmc 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 study
    V 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...
  27. ncbi Pancreatic enzyme therapy for pancreatic exocrine insufficiency
    J 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...
  28. ncbi Fecal pancreatic elastase: a reproducible marker for severe exocrine pancreatic insufficiency
    Satoru Naruse
    Department of Gastroenterology, Nagoya University Graduate School of Medicine, Nagoya, Japan
    J Gastroenterol 41:901-8. 2006
    ....
  29. ncbi Genetic analysis of Shwachman-Diamond syndrome: phenotypic heterogeneity in patients carrying identical SBDS mutations
    Takako 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...
  30. doi MRI and MRCP findings of the pancreas in patients with diabetes mellitus: compared analysis with pancreatic exocrine function determined by fecal elastase 1
    Mehmet Bilgin
    Department of Radiology, University Hospital Giessen and Marburg, Giessen, Germany
    J Clin Gastroenterol 43:165-70. 2009
    ....
  31. ncbi Use of fecal elastase-1 to classify pancreatic status in patients with cystic fibrosis
    Drucy 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...
  32. pmc Shwachman-Diamond syndrome neutrophils have altered chemoattractant-induced F-actin polymerization and polarization characteristics
    Claudia 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...
  33. ncbi 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.
  34. ncbi Giardiasis aggravates malabsorption in cystic fibrosis
    J Walkowiak
    Institute of Pediatrics, Dept of Gastroenterology and Metabolism, Karol Marcinkowski University of Medical Sciences, Poznan, Poland
    Scand J Gastroenterol 39:607-8. 2004
  35. ncbi Pancreatic enzyme therapy and clinical outcomes in patients with cystic fibrosis
    Susan 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)...
  36. ncbi Follow-up of exocrine pancreatic function in type-1 diabetes mellitus
    Werner Creutzfeldt
    Department of Internal Medicine, Division of Gastroenterology and Metabolism, University of Gottingen, Gottingen, Germany
    Digestion 72:71-5. 2005
    In 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...
  37. ncbi Prevalence and determinants of exocrine pancreatic insufficiency among older adults: results of a population-based study
    Dietrich Rothenbacher
    Department of Epidemiology, German Centre for Research on Ageing, University of Heidelberg, Heidelberg, Germany
    Scand J Gastroenterol 40:697-704. 2005
    The 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.
  38. ncbi Comparing the urinary pancreolauryl ratio and faecal elastase-1 as indicators of pancreatic insufficiency in clinical practice
    D 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 ..
  39. ncbi [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. 2004
    The paper discusses the non-invasive (tubeless) pancreatic function tests used to diagnose exocrine pancreatic insufficiency (EI)...
  40. ncbi Fecal chymotrypsin and elastase-1 determination on one single stool collected at random: diagnostic value for exocrine pancreatic status
    Isabelle 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...
  41. pmc Optimising the therapy of exocrine pancreatic insufficiency by the association of a proton pump inhibitor to enteric coated pancreatic extracts
    J E Dominguez-Munoz
    Gut 55:1056-7. 2006
  42. ncbi A novel CFTR mutation found in a Chinese patient with cystic fibrosis
    Nan 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...
  43. ncbi Maldigestion associated with exocrine pancreatic insufficiency: implications of gastrointestinal physiology and properties of enzyme preparations for a cause-related and patient-tailored treatment
    M J Bruno
    Division of Gastroenterology and Hepatology, University of Amsterdam, The Netherlands
    Am J Gastroenterol 90:1383-93. 1995
    Clinical 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...
  44. ncbi Identification of novel mutations in patients with Shwachman-Diamond syndrome
    Elena Nicolis
    Laboratory of Molecular Pathology, Azienda Ospedaliera di Verona, Verona, Italy
    Hum Mutat 25:410. 2005
    Shwachman-Diamond syndrome (SDS) is a rare autosomal recessive disease, mainly characterized by exocrine pancreatic insufficiency, hematological dysfunction and skeletal abnormalities...
  45. ncbi Novel SBDS mutations caused by gene conversion in Japanese patients with Shwachman-Diamond syndrome
    Eiji 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. 2004
    Shwachman-Diamond syndrome (SDS; OMIM 260400) is an autosomal recessive disorder characterized by exocrine pancreatic insufficiency, bone marrow dysfunction and metaphyseal chondrodysplasia...
  46. ncbi Successful unrelated donor bone marrow transplantation for Shwachman-Diamond syndrome with leukemia
    Tetsuo Mitsui
    Department of Pediatrics, Yamagata University School of Medicine, Yamagata, Japan
    Int J Hematol 79:189-92. 2004
    Shwachman-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...
  47. pmc Diabetes and exocrine pancreatic insufficiency in E2F1/E2F2 double-mutant mice
    Ainhoa 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...
  48. ncbi Ultrase MT12 and Ultrase MT20 in the treatment of exocrine pancreatic insufficiency in cystic fibrosis: safety and efficacy
    M W Konstan
    Rainbow Babies and Children s Hospital, Cleveland, OH 44106, USA
    Aliment Pharmacol Ther 20:1365-71. 2004
    Cystic fibrosis causes exocrine pancreatic insufficiency, leading to malabsorption. Supplemental pancreatic enzyme therapy alleviates the concomitant malnutrition experienced by cystic fibrosis patients...
  49. ncbi Assessment of maldigestion in cystic fibrosis
    Jaroslaw Walkowiak
    J Pediatr 145:285-7. 2004
  50. ncbi A double-blind, randomized, multicentre, crossover study to prove equivalence of pancreatin minimicrospheres versus microspheres in exocrine pancreatic insufficiency
    U 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...
  51. ncbi Gastric acid suppression and treatment of severe exocrine pancreatic insufficiency
    E 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...
  52. ncbi Update on the evaluation of pancreatic exocrine status in cystic fibrosis
    Drucy 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...
  53. ncbi Allogeneic bone marrow transplantation in Shwachman-Diamond syndrome with malignant myeloid transformation. A case report
    So 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. 2002
    Shwachman-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...
  54. ncbi Comparative evaluation of a high lipase pancreatic enzyme preparation and a standard pancreatic supplement for treating exocrine pancreatic insufficiency in chronic pancreatitis
    M Delhaye
    Medicosurgical Department of Gastroenterology, Erasme Hospital, Free University of Brussels, Belgium
    Eur J Gastroenterol Hepatol 8:699-703. 1996
    ....
  55. ncbi Successful unrelated umbilical cord blood transplantation in children with Shwachman-Diamond syndrome
    R 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...
  56. ncbi Pancreatic enzyme replacement therapy
    P 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...
  57. ncbi 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 study
    J 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...
  58. doi Low fecal elastase 1 levels do not indicate exocrine pancreatic insufficiency in type-1 diabetes mellitus
    Jan 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 ..
  59. ncbi Shwachman-diamond syndrome
    Yigal 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
  60. ncbi Gastrointestinal outcomes and confounders in cystic fibrosis
    Drucy Borowitz
    J Pediatr Gastroenterol Nutr 41:273-85. 2005
  61. ncbi 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
    ....
  62. ncbi Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review
    Virginia 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...
  63. ncbi Exocrine pancreas disfunction in severely traumatised patients and early enteral nutrition
    Metin Senkal
    Department of General Surgery, Marien Hospital Witten, Germany
    Ulus Travma Acil Cerrahi Derg 14:34-9. 2008
    We investigated exocrine pancreatic insufficiency in severely traumatised patients with enteral nutrition using the fecal elastase-1 concentration.
  64. ncbi Monoclonal versus polyclonal ELISA for assessment of fecal elastase concentration: pitfalls of a new assay
    Arne Schneider
    Medical Department I, University Hospital, Frankfurt Main, Germany
    Clin Chem 51:1052-4. 2005
  65. ncbi Pancreatic exocrine insufficiency is not common in HNF-1alpha MODY
    T Klupa
    Department of Metabolic Diseases, Jagiellonian University, Medical College, Krakow, Poland
    Diabet Med 25:627-30. 2008
    b>Exocrine pancreatic insufficiency has been described in Type 1 and Type 2 diabetes...
  66. doi The effect of zinc supplements in cystic fibrosis patients
    S 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...
  67. ncbi Fecal elastase: pancreatic status verification and influence on nutritional status in children with cystic fibrosis
    Jacqueline 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...
  68. ncbi [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...
  69. ncbi Biliary versus alcohol-related infected pancreatic necrosis: similarities and differences in the follow-up
    Jacek 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...
  70. ncbi Pancreatic enzyme pharmacotherapy
    Marcus 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...
  71. ncbi Enteric-coated pancreatic enzyme with bicarbonate is equal to standard enteric-coated enzyme in treating malabsorption in cystic fibrosis
    Daina 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...
  72. ncbi [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...
  73. ncbi Study of a novel pancreatic enzyme replacement therapy in pancreatic insufficient subjects with cystic fibrosis
    Drucy 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
    ....
  74. ncbi Comparison of monoclonal and polyclonal ELISAs for fecal elastase in patients with cystic fibrosis and pancreatic insufficiency
    Drucy 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)...
  75. ncbi Re: fecal elastase: pancreatic status verification and influence on nutritional status in children with cystic fibrosis
    Jaroslaw Walkowiak
    J Pediatr Gastroenterol Nutr 42:117; author reply 118. 2006
  76. ncbi Pancreatic enzyme therapy and gastrointestinal symptoms in patients with cystic fibrosis
    Jaroslaw Walkowiak
    J Pediatr 147:870-1. 2005
  77. ncbi Diagnosis and treatment of intestinal malabsorption in cystic fibrosis
    James M Littlewood
    Regional Paediatric Cystic Fibrosis Unit, St James s University Hospital, Leeds, UK
    Pediatr Pulmonol 41:35-49. 2006
    ....
  78. ncbi An enteric-coated high-buffered pancrelipase reduces steatorrhea in patients with cystic fibrosis: a prospective, randomized study
    Mary 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...
  79. ncbi Nutritional management of cystic fibrosis patients
    Daina 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...
  80. ncbi [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
  81. ncbi Safety and preliminary clinical activity of a novel pancreatic enzyme preparation in pancreatic insufficient cystic fibrosis patients
    Drucy 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...
  82. ncbi Pancreatitis among patients with cystic fibrosis: correlation with pancreatic status and genotype
    Kris De Boeck
    University Hospital, Leuven, Belgium
    Pediatrics 115:e463-9. 2005
    ....
  83. ncbi Omeprazole, a proton pump inhibitor, improves residual steatorrhoea in cystic fibrosis patients treated with high dose pancreatic enzymes
    Marijke 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)...
  84. ncbi How useful is fecal pancreatic elastase 1 as a marker of exocrine pancreatic disease?
    Satti Beharry
    Department of Pediatrics, The University of Toronto, Ontario, Canada
    J Pediatr 141:84-90. 2002
    To evaluate the role of fecal elastase 1 (E1) as a marker of exocrine pancreatic insufficiency (PI).
  85. ncbi Fecal elastase-1 is superior to fecal chymotrypsin in the assessment of pancreatic involvement in cystic fibrosis
    Jaroslaw 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)...
  86. ncbi Exocrine pancreatic insufficiency and its consequences on physical development and metabolism in children and adolescents with type 1 diabetes mellitus
    Martin W Laass
    Children s Hospital, Technical University Dresden, Fetscherstrasse 74, 01307, Dresden, Germany
    Eur J Pediatr 163:681-2. 2004
  87. ncbi Comparison of steatocrit and fat absorption in persons with cystic fibrosis
    Mary H Wagner
    Department of Pediatrics, University of Florida, Gainesville, Florida 32610, USA
    J Pediatr Gastroenterol Nutr 35:202-5. 2002
    b>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...
  88. ncbi 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. 2004
    Fecal 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 ..
  89. ncbi 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 insufficiency
    P 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...
  90. ncbi Genotype/phenotype correlation of the G85E mutation in a large cohort of cystic fibrosis patients
    K 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...
  91. ncbi Heat wave and acute pancreatitis: very unusual cystic fibrosis presentation
    Anne Munck
    Pediatrics 113:1846. 2004
  92. ncbi Lipid, nitrogen, water and energy content of a single stool sample in healthy children and children with cystic fibrosis
    Anita Maria Van den Neucker
    Department of Paediatrics, University Hospital Maastricht, P Debyelaan 25, 6229 HX Maastricht, The Netherlands
    Eur J Pediatr 162:764-6. 2003
    ..Results of single stool sample water and nitrogen were similar in both groups. These results were comparable to those of a 3-day stool collection, as reported in the literature...
  93. ncbi Serum lipase after secretin stimulation detects mild pancreatic involvement in cystic fibrosis
    Jaroslaw Walkowiak
    Department of Gastroenterology, Institute of Pediatrics, Karol Marcinkowski University of Medical Sciences, Poznan, Poland
    J Pediatr Gastroenterol Nutr 38:430-5. 2004
    ..However, identification of mild cases remains a challenge. The aim of this study was to assess the ability of serum lipase after secretin stimulation to identify mild pancreatic insufficiency in patients with CF...
  94. ncbi Faecal elastase-1: lyophilization of stool samples prevents false low results in diarrhoea
    B Fischer
    Dept. of Medicine 1, , Erlangen, Germany
    Scand J Gastroenterol 36:771-4. 2001
    BACKGROUND: In patients with diarrhoea, faecal elastase-1 is used to detect exocrine pancreatic insufficiency. Diarrhoea is defined as >85% stool water content...
  95. ncbi Fecal elastase-1 as a test for pancreatic function: a review
    Roberto 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 ..
  96. ncbi Low fecal elastase: potentially related to transient small bowel damage resulting from enteric pathogens
    Silvia 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...
  97. ncbi [Pancreatic exocrine insufficiency in patients with diabetes mellitus. Current state of our knowledge and practical consequences]
    M L Richter
    Solvay Arzneimittel GmbH, , , D-30173 Hannover
    Fortschr Med Orig 119:77-9. 2001
    ..The diagnosis is confirmed with the aid of simple laboratory tests, for example the chemotrypsin test or the detection of elastase 1 in the stools. By way of treatment, pancreatic enzyme replacement can be applied...
  98. ncbi Early decline of pancreatic function in cystic fibrosis patients with class 1 or 2 CFTR mutations
    Jaroslaw Walkowiak
    Institute of Pediatrics, Department of Gastroenterology and Metabolism, Karol Marcinkowski University of Medical Sciences, 60 572 Poznan, Poland
    J Pediatr Gastroenterol Nutr 40:199-201. 2005
    ..We assessed the decline of pancreatic function in CF infants carrying class 1 or 2 CFTR mutations who were diagnosed in a neonatal screening program...
  99. pmc Molecular consequences of cystic fibrosis transmembrane regulator (CFTR) gene mutations in the exocrine pancreas
    N 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...
  100. ncbi [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 Chorób Metabolicznych, Instytut Pediatrii, Akademia Medyczna, ul Szpitalna 27 33, 60 572 Poznan, 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...
  101. ncbi Evaluation of fecal pancreatic elastase-1 as a measure of pancreatic exocrine function in children with cystic fibrosis
    A Cade
    Regional Paediatric Cystic Fibrosis Unit, St James s University Hospital, Leeds, UK
    Pediatr Pulmonol 29:172-6. 2000
    ..The low values in the first 2 weeks in some normal and premature infants, and the persisting normal values in PS infants, make the fecal EL-1 test unsuitable for neonatal CF screening...

Research Grants92

  1. Shwachman-Diamond Syndrome: Molecular Pathogenesis
    Akiko Shimamura; Fiscal Year: 2009
    ..by applicant): Shwachman-Diamond syndrome (SDS) is an autosomal recessive disease characterized by exocrine pancreatic insufficiency, bone marrow failure, increased risk of acute myeloid leukemia, and other variable developmental ..
  2. Pathological Evaluation of a Cystic Fibrosis Ferret Model
    JOHN ENGELHARDT; Fiscal Year: 2009
    ..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)...
  3. Pathological Evaluation of a Cystic Fibrosis Ferret Model
    John 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)...
  4. ASSESSMENT OF EXOCRINE PANCREATIC FUNCTION IN CHILDREN W/ HIV INFECTION
    ANDREW MULBERG; Fiscal Year: 1999
    Introduction: The aim of our study was to determine the prevalence of steatorrhea and exocrine pancreatic insufficiency (EPI) and their relationship to nutritional status and HIV stage in a population of children HIV infection...
  5. AEROSOLIZED TGAAVCF FOR TREATMENT OF CYSTIC FIBROSIS
    Mark Dovey; Fiscal Year: 2000
    ..The main characteristics are malabsorption due to exocrine pancreatic insufficiency, recurrent bacterial infections of the lower respiratory tract, abnormal regulation of salt ..
  6. AEROSOLIZED TGAAVCF FOR TREATMENT OF CYSTIC FIBROSIS
    Mark Dovey; Fiscal Year: 2001
    ..The main characteristics are malabsorption due to exocrine pancreatic insufficiency, recurrent bacterial infections of the lower respiratory tract, abnormal regulation of salt ..
  7. BIOSYNTHESIS AND STRUCTURE OF RAT LIPASE AND COLIPASE
    Mark Lowe; Fiscal Year: 2000
    ..or colipase activity, such as occurs in the premature infant and in disease states associated with exocrine pancreatic insufficiency, steatorrhea invariably is present...
  8. BIOSYNTHESIS AND STRUCTURE OF RAT LIPASE AND COLIPASE
    Mark Lowe; Fiscal Year: 1999
    ..or colipase activity, such as occurs in the premature infant and in disease states associated with exocrine pancreatic insufficiency, steatorrhea invariably is present...
  9. EXOCRINE PANCREATIC FUNCTION IN CHILD WITH HIV INFECTION
    ANDREW MULBERG; Fiscal Year: 2000
    The role of exocrine pancreatic insufficiency in HIV-associated steatorrhea and malnutrition is undefined. Not infrequently steatorrhea in people with HIV infection remains unexplained...
  10. PHYSIOLOGY AND THE PATHOPHYSIOLOGY OF SECRETIN
    William Chey; Fiscal Year: 1980
    ..The study will be carried out in abnormal states such as patients with achlorhydria and exocrine pancreatic insufficiency. Interaction between secretin in a physiological dose range and octapeptide of cholecystokinin on ..
  11. Genetic Modifiers of CF: Sibling Study
    Garry Cutting; Fiscal Year: 2007
    ..lung disease due to abnormalities in airway surface liquid and chronic malnutrition due to exocrine pancreatic insufficiency. Survival of CF patients is highly correlated with the severity of lung disease and degree of ..
  12. Genetic Modifiers of CF: Sibling Study
    Garry R Cutting; Fiscal Year: 2010
    ..lung disease due to abnormalities in airway surface liquid and chronic malnutrition due to exocrine pancreatic insufficiency. Survival of CF patients is highly correlated with the severity of lung disease and degree of ..
  13. Genetic Modifiers of CF: Sibling Study
    Garry Cutting; Fiscal Year: 2009
    ..lung disease due to abnormalities in airway surface liquid and chronic malnutrition due to exocrine pancreatic insufficiency. Survival of CF patients is highly correlated with the severity of lung disease and degree of ..
  14. 4th International Congress on Shwachman-Diamond Syndrome
    Richard Grand; Fiscal Year: 2007
    ..Characteristic clinical features include exocrine pancreatic insufficiency, bone marrow failure, and leukemia predisposition...
  15. EPITHELIAL CELL LINES WITH CYSTIC FIBROSIS MUTATIONS
    James Yankaskas; Fiscal Year: 1992
    ..Transmembrane Regulator (CFTR) gene and is classically associated with progressive lung disease, exocrine pancreatic insufficiency, and elevated sweat Cl- levels...
  16. Shwachman-Diamond Syndrome: Molecular Pathogenesis
    Akiko Shimamura; Fiscal Year: 2007
    Shwachman-Diamond syndrome (SDS) is an autosomal recessive disease characterized by exocrine pancreatic insufficiency, bone marrow failure, increased risk of acute myeloid leukemia, and other variable developmental abnormalities...
  17. Shwachman-Diamond Syndrome: Molecular Pathogenesis
    Akiko Shimamura; Fiscal Year: 2006
    Shwachman-Diamond syndrome (SDS) is an autosomal recessive disease characterized by exocrine pancreatic insufficiency, bone marrow failure, increased risk of acute myeloid leukemia, and other variable developmental abnormalities...
  18. Shwachman-Diamond Syndrome: Molecular Pathogenesis
    Akiko Shimamura; Fiscal Year: 2004
    Shwachman-Diamond syndrome (SDS) is an autosomal recessive disease characterized by exocrine pancreatic insufficiency, bone marrow failure, increased risk of acute myeloid leukemia, and other variable developmental abnormalities...
  19. Shwachman-Diamond Syndrome: Molecular Pathogenesis
    Akiko Shimamura; Fiscal Year: 2005
    Shwachman-Diamond syndrome (SDS) is an autosomal recessive disease characterized by exocrine pancreatic insufficiency, bone marrow failure, increased risk of acute myeloid leukemia, and other variable developmental abnormalities...
  20. Shwachman-Diamond Syndrome: Molecular Pathogenesis
    Akiko Shimamura; Fiscal Year: 2004
    Shwachman-Diamond syndrome (SDS) is an autosomal recessive disease characterized by exocrine pancreatic insufficiency, bone marrow failure, increased risk of acute myeloid leukemia, and other variable developmental abnormalities...
  21. Molecular genetics, SBDS function, and deficiency in SDS
    Johanna Rommens; Fiscal Year: 2004
    Shwachman-Diamond syndrome (SDS) is a recessive disorder with exocrine pancreatic insufficiency, skeletal abnormalities and bone marrow failure...
  22. Molecular genetics, SBDS function, and deficiency in SDS
    Johanna Rommens; Fiscal Year: 2005
    Shwachman-Diamond syndrome (SDS) is a recessive disorder with exocrine pancreatic insufficiency, skeletal abnormalities and bone marrow failure...
  23. Molecular genetics, SBDS function, and deficiency in SDS
    Johanna Rommens; Fiscal Year: 2006
    Shwachman-Diamond syndrome (SDS) is a recessive disorder with exocrine pancreatic insufficiency, skeletal abnormalities and bone marrow failure...
  24. Molecular genetics, SBDS function, and deficiency in SDS
    Johanna Rommens; Fiscal Year: 2007
    Shwachman-Diamond syndrome (SDS) is a recessive disorder with exocrine pancreatic insufficiency, skeletal abnormalities and bone marrow failure...
  25. STUDY TO COMPARE THE SAFETY AND EFFICACY OF ULTRASE MT12 TO PLACEBO IN CF
    Frank Accurso; Fiscal Year: 1999
    ..is both effective and safe in the treatment of fat malabsorption in patients with CF and exocrine pancreatic insufficiency. The three specific aims include: 1) To evaluate the efficacy of Ultrase MT12 in the treatment of ..
  26. BORDERLINE PANCREATIC FUNCTION IN CYSTIC FIBROSIS
    Morteza 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...
  27. Inflammatory response to pancreatitis in CF mice: sodium & impaired apoptosis
    MATTHEW DIMAGNO; Fiscal Year: 2008
    ..Understanding of the pathophysiology of AP is incomplete & therapies are lacking. The proposed study may provide insight into the pathogenesis of AP & CF & contribute to generation of novel therapies. ..
  28. Pioglitazone Therapy for Endocrine and Exocrine Disease in Alcoholic Pancreatitis
    MATTHEW DIMAGNO; Fiscal Year: 2008
    ....
  29. Inflammatory response to pancreatitis in CF mice: sodium & impaired apoptosis
    MATTHEW DIMAGNO; Fiscal Year: 2007
    ..Understanding of the pathophysiology of AP is incomplete & therapies are lacking. The proposed study may provide insight into the pathogenesis of AP & CF & contribute to generation of novel therapies. ..
  30. Pulmonary Alveolar Proteinosis Research Conference
    Bruce 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. ..
  31. NUTRITIONAL STATUS OF CF PULMONARY DISEASE
    Virginia Stallings; Fiscal Year: 2000
    ..These data will provide the scientific basis for early and effective nutritional intervention to promote normal growth and body composition as standard CF care. ..
  32. Regulation of Alveolar Macrophage Phagocytosis
    Bruce 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. ..
  33. AAT Deficiency & Liver Diseases Caused by Proteins
    Bruce Trapnell; Fiscal Year: 2006
    ..unreadable] [unreadable]..
  34. Rare Lung Disease Clinical Research Network
    Bruce Trapnell; Fiscal Year: 2005
    ..Ongoing clinical, basic and translational studies at the centers chosen have already yielded critical insights into molecular mechanisms underlying lung function and defense in health and disease. ..
  35. Rare Lung Disease Clinical Research Network
    Bruce Trapnell; Fiscal Year: 2006
    ..Ongoing clinical, basic and translational studies at the centers chosen have already yielded critical insights into molecular mechanisms underlying lung function and defense in health and disease. ..
  36. Fourth Annual International Pulmonary Alveolar Proteinosis Research Conference
    Bruce Trapnell; Fiscal Year: 2006
    ..unreadable] [unreadable] [unreadable] [unreadable]..
  37. Role of Anti-GM-CSF Antibodies in Myeloid Cell Function & Innate Immunity
    Bruce Trapnell; Fiscal Year: 2007
    ..Clinical implications exist for PAP therapies, and also for anti-GM-CSF antibody-based therapies to treat serious inflammatory disorders, an approach for which significant commercial interest and development have now emerged. ..
  38. NUTRITIONAL STATUS OF CF PULMONARY DISEASE
    Virginia Stallings; Fiscal Year: 1999
    ..These data will provide the scientific basis for early and effective nutritional intervention to promote normal growth and body composition as standard CF care. ..
  39. Rare Lung Disease Clinical Research Network
    Bruce Trapnell; Fiscal Year: 2006
    ..Ongoing clinical, basic and translational studies at the centers chosen have already yielded critical insights into molecular mechanisms underlying lung function and defense in health and disease. ..
  40. Role of Anti-GM-CSF Antibodies in Myeloid Cell Function & Innate Immunity
    Bruce Trapnell; Fiscal Year: 2008
    ..Clinical implications exist for PAP therapies, and also for anti-GM-CSF antibody-based therapies to treat serious inflammatory disorders, an approach for which significant commercial interest and development have now emerged. ..
  41. Role of Anti-GM-CSF Antibodies in Myeloid Cell Function & Innate Immunity
    Bruce Trapnell; Fiscal Year: 2009
    ..Clinical implications exist for PAP therapies, and also for anti-GM-CSF antibody-based therapies to treat serious inflammatory disorders, an approach for which significant commercial interest and development have now emerged. ..
  42. Safe and Effective Vitamin D Supplementation in HIV
    Virginia Stallings; Fiscal Year: 2009
    ..Inexpensive and easy to administer, vitamin D supplementation may prove to be an effective and feasible treatment for symptoms and prevention of side effects for people of all ages living with HIV/AIDS in the US and around the world. ..
  43. Safe and Effective Vitamin D Supplementation in HIV
    Virginia A Stallings; Fiscal Year: 2010
    ..Inexpensive and easy to administer, vitamin D supplementation may prove to be an effective and feasible treatment for symptoms and prevention of side effects for people of all ages living with HIV/AIDS in the US and around the world. ..
  44. GM-CSF & Alveolar Macrophage Antiviral Lung Defense
    Bruce Trapnell; Fiscal Year: 2004
    ..abstract_text> ..
  45. GM-CSF & Alveolar Macrophage Antiviral Lung Defense
    Bruce Trapnell; Fiscal Year: 2003
    ..abstract_text> ..
  46. Regulation of Alveolar Macrophage Phagocytosis
    Bruce Trapnell; Fiscal Year: 2003
    ..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. ..
  47. GM-CSF & Alveolar Macrophage Antiviral Lung Defense
    Bruce Trapnell; Fiscal Year: 2001
    ..abstract_text> ..
  48. GM-CSF & Alveolar Macrophage Antiviral Lung Defense
    Bruce Trapnell; Fiscal Year: 2002
    ..abstract_text> ..
  49. NUTRITION IN SICKLE CELL DISEASE
    Virginia Stallings; Fiscal Year: 2002
    ..These three projects will be first of their kind in determining whether nutrition intervention improves growth, nutritional status and dietary intake in children with sickle cell disease. ..
  50. Rare Lung Disease Clinical Research Network
    Bruce Trapnell; Fiscal Year: 2003
    ..Ongoing clinical, basic and translational studies at the centers chosen have already yielded critical insights into molecular mechanisms underlying lung function and defense in health and disease. ..
  51. Rare Lung Disease Clinical Research Network
    Bruce Trapnell; Fiscal Year: 2004
    ..Ongoing clinical, basic and translational studies at the centers chosen have already yielded critical insights into molecular mechanisms underlying lung function and defense in health and disease. ..
  52. Role of Anti-GM-CSF Antibodies in Myeloid Cell Function & Innate Immunity
    Bruce C Trapnell; Fiscal Year: 2010
    ..Clinical implications exist for PAP therapies, and also for anti-GM-CSF antibody-based therapies to treat serious inflammatory disorders, an approach for which significant commercial interest and development have now emerged. ..
  53. NUTRITIONAL STATUS OF CF PULMONARY DISEASE
    Virginia 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. ..
  54. Conference--Pulmonary Alveolar Proteinosis Research
    Bruce 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. ..
  55. Mentored Patient-Oriented Research Career Development Award
    Scott Sagel; Fiscal Year: 2005
    ..In addition, he will actively participate and train in the Pediatric GCRC, and frequently interact with his sponsor, mentors, and collaborators. ..
  56. Nonmyeloablative Allografting for Immunodeficiency & Other Nonmalignant Disorders
    Lauri Burroughs; Fiscal Year: 2007
    ..Information gained from these studies will be particularly important for patients with nonmalignant disorders. ..
  57. North American Pancreatitis Study 2 (NAPS2)
    David Whitcomb; Fiscal Year: 2002
    ..The results of these studies may result in the development new diagnostic tools that can be applied at the onset of symptoms, and rational therapy to prevent the development of those pancreatic diseases that we cannot currently cure. ..
  58. NAPS2 Continuation - Genome-Wide Association Study of Pancreatitis
    David Whitcomb; Fiscal Year: 2009
    ..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. ..
  59. NAPS2-AS: Race Dependent Risk for Pancreatitis
    David C Whitcomb; Fiscal Year: 2010
    ....
  60. HYPOXIA AND ET-1 EFFECT ON PA SMC EFFECT ON CHANNELS
    David Rodman; Fiscal Year: 2001
    ....
  61. North American Pancreatitis Study 2 (NAPS2)
    David Whitcomb; Fiscal Year: 2003
    ..The results of these studies may result in the development new diagnostic tools that can be applied at the onset of symptoms, and rational therapy to prevent the development of those pancreatic diseases that we cannot currently cure. ..
  62. NAPS2 Continuation - Genome-Wide Association Study of Pancreatitis
    David Whitcomb; Fiscal Year: 2008
    ..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. ..
  63. MOLECULAR GENETICS OF HEREDITARY PANCREATITIS
    David Whitcomb; Fiscal Year: 1999
    ..Answering this question may provide insight into the pathophysiology of acute and chronic pancreatitis and identify targets for new therapies, and help identify individuals at risk through genetic testing. ..
  64. NAPS2 Continuation - Genome-Wide Association Study of Pancreatitis
    David 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. ..
  65. MOLECULAR GENETICS OF HEREDITARY PANCREATITIS
    David 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. ..
  66. Effects of BMPRII Mutations in Pulmonary Hypertension
    David Rodman; Fiscal Year: 2003
    ..We will have also tested the role of four pulmonary cell types, smooth muscle, endothelium, airway epithelium and macrophages in the link between BMPR2 and pulmonary hypertension. ..
  67. North American Pancreatitis Study 2 (NAPS2)
    David Whitcomb; Fiscal Year: 2005
    ..The results of these studies may result in the development new diagnostic tools that can be applied at the onset of symptoms, and rational therapy to prevent the development of those pancreatic diseases that we cannot currently cure. ..
  68. NOS GENE TRANSFER IN PULMONARY HYPERTENSION
    David Rodman; Fiscal Year: 2000
    ....
  69. MOLECULAR GENETICS OF HEREDITARY PANCREATITIS
    David Whitcomb; Fiscal Year: 2007
    ..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. ..
  70. MOLECULAR GENETICS OF HEREDITARY PANCREATITIS
    David Whitcomb; Fiscal Year: 2004
    ..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. ..
  71. Clinical Applications of a Novel Fanconi Anemia Assay
    Akiko Shimamura; Fiscal Year: 2007
    ..abstract_text> ..
  72. NOS GENE TRANSFER IN PULMONARY HYPERTENSION
    David Rodman; Fiscal Year: 2001
    ....
  73. MOLECULAR GENETICS OF HEREDITARY PANCREATITIS
    David Whitcomb; Fiscal Year: 2001
    ..Answering this question may provide insight into the pathophysiology of acute and chronic pancreatitis and identify targets for new therapies, and help identify individuals at risk through genetic testing. ..
  74. HYPOXIA AND ET-1 EFFECT ON PA SMC EFFECT ON CHANNELS
    David Rodman; Fiscal Year: 2000
    ....
  75. EXTRAMURAL FACILITIES IMPROVEMENT PROGRAM
    Robert Golden; Fiscal Year: 2004
    ....
  76. HYPOXIA AND ET-1 EFFECT ON PA SMC EFFECT ON CHANNELS
    David Rodman; Fiscal Year: 1999
    ....
  77. NOS GENE TRANSFER IN PULMONARY HYPERTENSION
    David Rodman; Fiscal Year: 2002
    ....
  78. NOS GENE TRANSFER IN PULMONARY HYPERTENSION
    David Rodman; Fiscal Year: 1999
    ....
  79. HYPOXIA AND ET-1 EFFECT ON PA SMC EFFECT ON CHANNELS
    David Rodman; Fiscal Year: 2001
    ....
  80. Shwachman-Diamond Syndrome International Registry and Repository
    Akiko Shimamura; Fiscal Year: 2008
    ..These studies will also improve the medical management of non-SDS patients with neutropenia. ..
  81. Shwachman-Diamond Syndrome: Molecular Pathogenesis
    Akiko Shimamura; Fiscal Year: 2008
    Shwachman-Diamond syndrome (SDS) is an autosomal recessive disease characterized by exocrine pancreatic insufficiency, bone marrow failure, increased risk of acute myeloid leukemia, and other variable developmental abnormalities...
  82. Effects of BMPRII Mutations in Pulmonary Hypertension
    David Rodman; Fiscal Year: 2004
    ..We will have also tested the role of four pulmonary cell types, smooth muscle, endothelium, airway epithelium and macrophages in the link between BMPR2 and pulmonary hypertension. ..
  83. HYPOXIA AND ET-1 EFFECT ON PA SMC EFFECT ON CHANNELS
    David Rodman; Fiscal Year: 2002
    ....