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. ncbi 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. 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
  6. ncbi 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
  7. ncbi 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
  8. 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
  9. 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
  10. 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

Research Grants

  1. Shwachman-Diamond Syndrome: Molecular Pathogenesis
    Akiko Shimamura; Fiscal Year: 2009
  2. Genetic Modifiers of CF: Sibling Study
    Garry R Cutting; Fiscal Year: 2010
  3. Genetic Modifiers of CF: Sibling Study
    Garry Cutting; Fiscal Year: 2007
  4. 4th International Congress on Shwachman-Diamond Syndrome
    Richard Grand; Fiscal Year: 2007
  5. Shwachman-Diamond Syndrome: Molecular Pathogenesis
    Akiko Shimamura; Fiscal Year: 2007
  6. Molecular genetics, SBDS function, and deficiency in SDS
    Johanna Rommens; Fiscal Year: 2007
  7. Role of Anti-GM-CSF Antibodies in Myeloid Cell Function & Innate Immunity
    Bruce Trapnell; Fiscal Year: 2007
  8. Safe and Effective Vitamin D Supplementation in HIV
    Virginia A Stallings; Fiscal Year: 2010
  9. Role of Anti-GM-CSF Antibodies in Myeloid Cell Function & Innate Immunity
    Bruce C Trapnell; Fiscal Year: 2010
  10. Regulation of Alveolar Macrophage Phagocytosis
    Bruce Trapnell; Fiscal Year: 2006

Detail Information

Publications177 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
    ..Exocrine insufficiency seems to be correlated to early onset of endocrine failure, long-lasting diabetes mellitus and low body mass index levels...
  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
    ..CONCLUSIONS: Pancreatic exocrine insufficiency occurs more frequently in diabetic patients than in controls. Diabetic individuals with excess weight (BMI >25) may be at increased risk for underlying exocrine pancreatic 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. ncbi 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. 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...
  6. ncbi 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
    ....
  7. ncbi 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...
  8. 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.
  9. 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
  10. 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.
  11. ncbi 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. 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...
  13. 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...
  14. 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...
  15. 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...
  16. 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...
  17. 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...
  18. 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...
  19. 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...
  20. ncbi Assessment of maldigestion in cystic fibrosis
    Jaroslaw Walkowiak
    J Pediatr 145:285-7. 2004
  21. 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...
  22. 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...
  23. ncbi 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...
  24. 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...
  25. 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...
  26. 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
    ....
  27. 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...
  28. ncbi 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 ..
  29. 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...
  30. ncbi Follow-up of exocrine pancreatic function in type-1 diabetes mellitus
    Werner Creutzfeldt
    Department of Internal Medicine, Division of Gastroenterology and Metabolism, , , 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...
  31. 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
    ..CONCLUSION: PET dose is not correlated with growth or gastrointestinal symptoms. More sensitive outcome measures of the effectiveness of PET in patients with CF are needed to guide treatment of PI...
  32. 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).
  33. 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
  34. 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
    ....
  35. 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
    ..The sensitivities were 16.7% for mild, 12.5% for moderate, and 72.2% for severe exocrine pancreatic insufficiency. Forty patients were reexamined after a median interval of 347 days...
  36. ncbi 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)...
  37. 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
    ..Notably, clinical variations are present even among the patients with the identical genotype: compound heterozygotes of the 258+2 T > C and 183-184 TA > CT mutations. Further study will be required to explain the clinical heterogeneity...
  38. ncbi [Chronic pancreatitis. Main symptoms: chronic abdominal pain, weight loss in steatorrhea, secondary diabetes mellitus]
    G Schulthess
    Medizinische Poliklinik,
    Praxis (Bern 1994) 94:1139-45. 2005
  39. 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...
  40. 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
  41. 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...
  42. 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...
  43. ncbi 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...
  44. 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...
  45. ncbi Variability of fecal pancreatic elastase measurements in cystic fibrosis patients
    I Meyts
    Pediatric Department, University Hospital Gasthuisberg, Herestraat 49, 3000 Leuven, Belgium
    J Cyst Fibros 1:265-8. 2002
    ..the fecal pancreatic elastase-1 (EL-1) test is a new non-invasive test for pancreatic function. The aim of the study was to evaluate the intra-patient variability of the fecal EL-1 test in a cystic fibrosis (CF) population...
  46. ncbi Creon 10,000 Minimicrospheres vs. Creon 8,000 microspheres--an open randomised crossover preference study
    C J Patchell
    Birmingham Children s Hospital NHS Trust, Birmingham B4 6NH, UK
    J Cyst Fibros 1:287-91. 2002
    ..Creon 10,000s smaller capsules are easier to take and should aid patient compliance...
  47. ncbi Effects of creatine supplementation in cystic fibrosis: results of a pilot study
    Christian P Braegger
    Divisions of Gastroenterology and Nutrition and Clinical Chemistry and Biochemistry, University Children s Hospital, Steinwiesstrasse 75, Zurich CH 8032, Switzerland
    J Cyst Fibros 2:177-82. 2003
    ..Creatine supplementation has been shown to increase phosphocreatine levels, thus promoting muscle growth and strength in athletes and having protective effects in neuromuscular disorders...
  48. 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
    ....
  49. 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
    ..Children with residual FE had greater fat absorption and improved growth and nutritional status over 24 months. FE assessment should be used to verify pancreatic status in patients with cystic fibrosis...
  50. 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...
  51. 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
    ..3,978 +/- 1,474 U lipase/g fat, P < 0.02). CONCLUSIONS: In the doses used, nutrient absorption of patients taking EC buffered PE preparation offers no advantage over a conventional EC-PE preparation...
  52. 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
    ....
  53. ncbi Pancreatic enzyme therapy and gastrointestinal symptoms in patients with cystic fibrosis
    Jaroslaw Walkowiak
    J Pediatr 147:870-1. 2005
  54. 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
    ....
  55. 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)...
  56. ncbi Gastrointestinal outcomes and confounders in cystic fibrosis
    Drucy Borowitz
    J Pediatr Gastroenterol Nutr 41:273-85. 2005
  57. 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...
  58. 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...
  59. ncbi [Fecal elastase-1: a useful test in pediatric practice]
    L David-Henriau
    , , Centre Hospitalier Pellegrin, CHU de Bordeaux, , 33076, Bordeaux Cedex, France
    Arch Pediatr 12:1221-5. 2005
    ..Nevertheless, low values may be found in diseases with villous atrophy or very liquid stools...
  60. 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...
  61. 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...
  62. 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
    ....
  63. 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 ..
  64. 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
  65. 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.
  66. ncbi [Pancreatic exocrine function in diabetes mellitus. Determination of fecal elastase]
    Carla Mancilla A
    , Departamento de Medicina, , Universidad de Chile
    Rev Med Chil 134:407-14. 2006
    ..Patients with lower levels of elastase have significantly more steatorrhea. Among diabetics it is possible to find a group of patients with non diagnosed chronic pancreatitis...
  67. 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
    ..CONCLUSION: Results from single stool samples yield clinically useful information concerning fat and energy. Single stool sample analysis is therefore useful for frequent monitoring of faecal fat and energy...
  68. 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...
  69. ncbi A prospective evaluation of pancreatic exocrine function in patients with acute pancreatitis: correlation with extent of necrosis and pancreatic endocrine insufficiency
    B Boreham
    Royal Gwent Hospital, Newport, Gwent, Wales, UK
    Pancreatology 3:303-8. 2003
    ....
  70. 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...
  71. ncbi 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...
  72. 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...
  73. ncbi Fecal elastase-1 concentration: an indirect test of exocrine pancreatic function and a marker of an enteropathy regardless of cause
    Sanda Nousia-Arvanitakis
    J Pediatr Gastroenterol Nutr 36:314-5. 2003
  74. 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 ..
  75. 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)...
  76. 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).
  77. 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...
  78. ncbi Pancreatic elastase 1 in feces of preterm and term infants
    K 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...
  79. 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...
  80. 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
    ..001). CONCLUSION: Omeprazole improves fat digestion and absorption in cystic fibrosis patients with residual faecal fat loss despite maximal pancreatic enzyme substitution...
  81. 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 ..
  82. 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
  83. ncbi Polyclonal versus monoclonal ELISA for the determination of fecal elastase 1: diagnostic value in cystic fibrosis and chronic pancreatic insufficiency
    Yvette Miendje
    Departement de Biochimie Medicale, Cliniques Universitaires Saint Luc, Universite Catholique de Louvain, Brussels, Belgium
    Clin Lab 50:419-24. 2004
    ..We report the results of the comparison of this method with a polyclonal based assay (BioServ AG, Rostock, Germany)...
  84. 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...
  85. ncbi Heat wave and acute pancreatitis: very unusual cystic fibrosis presentation
    Anne Munck
    Pediatrics 113:1846. 2004
  86. 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...
  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 Safety and tolerability of a new formulation of pancrelipase delayed-release capsules (CREON) in children under seven years of age with exocrine pancreatic insufficiency due to cystic fibrosis: an open-label, multicentre, single-treatment-arm study
    Gavin R Graff
    Penn State Milton S Hershey Medical Center, Hershey, Pennsylvania, USA
    Clin Drug Investig 30:351-64. 2010
    b>Exocrine pancreatic insufficiency (EPI) is a deficiency of digestive enzymes caused by diseases such as cystic fibrosis (CF)...
  89. ncbi Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome with renal failure: impact of posttransplant immunosuppression on disease activity
    Tim Ulinski
    Department of Pediatric Nephrology, Hopital Trousseau, 75571 Paris Cedex 12, France
    J Clin Endocrinol Metab 91:192-5. 2006
    ..Nonendocrine organ manifestations are autoimmune hepatitis, vitiligo, pernicious anemia, exocrine pancreatic insufficiency, and alopecia...
  90. ncbi CREON (Pancrelipase Delayed-Release Capsules) for the treatment of exocrine pancreatic insufficiency
    Robert J Kuhn
    Division of Pharmacy, Practice and Science, University of Kentucky, Lexington, Kentucky 40536 0596, USA
    Adv Ther 27:895-916. 2010
    b>Exocrine pancreatic insufficiency (EPI) is associated with conditions including cystic fibrosis (CF), chronic pancreatitis (CP), and pancreatic surgery (PS)...
  91. ncbi [Analysis of energy expenditure in adults with cystic fibrosis: comparison of indirect calorimetry and prediction equations]
    Casilda Olveira Fuster
    Unidad de Fibrosis Quística de Adultos, Servicio de Neumologia, Hospital Universitario Carlos Haya, Malaga, Espana
    Arch Bronconeumol 43:366-72. 2007
    ....
  92. ncbi Homozygous (TG)11 allele in intron 8 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene has a protective role against bicarbonate decrease in pure pancreatic juice among Japanese male alcoholics
    Yuji Nakamura
    National Hospital Organization, Kurihama Alcoholism Center, Nobi, Yokosuka, Kanagawa 239 0841
    Intern Med 43:1131-7. 2004
    ..b>Exocrine pancreatic insufficiency exists in about 90% of the patients with cystic fibrosis (CF), which results from an abnormal cystic ..
  93. ncbi Diagnosis of early-stage chronic pancreatitis by secretin-enhanced magnetic resonance cholangiopancreatography
    Laszlo Czako
    First Department of Medicine, University of Szeged, P O Box 427, Szeged, H 6701, Hungary
    J Gastroenterol 42:113-7. 2007
    ..pancreatic T2 signal intensity changes are significantly reduced in patients with a mild exocrine pancreatic insufficiency as compared with healthy volunteers...
  94. ncbi Pancreatin therapy in patients with insulin-treated diabetes mellitus and exocrine pancreatic insufficiency according to low fecal elastase 1 concentrations. Results of a prospective multi-centre trial
    Nils Ewald
    Third Medical Department and Policlinic, Giessen University Hospital, Giessen, Germany
    Diabetes Metab Res Rev 23:386-91. 2007
    ..have only been a few reports on the use of enzyme replacement therapy in diabetic patients with exocrine pancreatic insufficiency. This investigation was designed to evaluate the impact of enzyme-replacement therapy on glucose ..
  95. ncbi Behaviour of serum pancreatic enzymes in chronic pancreatitis
    R Pezzilli
    Emergency Department, Sant Orsola Hospital, Bologna, Italy
    Dig Liver Dis 32:233-7. 2000
    To establish whether serum pancreatic enzyme determination is useful in the identification of patients with chronic pancreatitis and in revealing the presence of exocrine pancreatic insufficiency
  96. ncbi Evaluation of pancreatic exocrine function by secretin-enhanced magnetic resonance cholangiopancreatography
    L Czako
    First Department of Medicine, University of Szeged, Faculty of Medicine, Hungary
    Pancreas 23:323-8. 2001
    ..To assess the feasibility and usefulness of secretin-enhanced magnetic resonance cholangiopancreatography (S-MRCP) for evaluation of pancreatic exocrine function...
  97. ncbi Management of chronic pancreatitis. Focus on enzyme replacement therapy
    G Dobrilla
    Division of Gastroenterology, General Regional Hospital, Bolzano, Italy
    Int J Pancreatol 5:17-29. 1989
    ..with chronic relapsing pancreatitis are twofold: pain relief and control of maldigestion caused by exocrine pancreatic insufficiency. Experience with the use of pancreatic enzymes for analgesic purposes suggests that the less severe ..
  98. ncbi [Steatorrhea and its treatment with pancreatic enzymes in endocrine tumors of the pancreas (pancreatic adenomas)]
    M J Varas
    Hospital Cruz Roja, L Hospitalet de Llobregat, Barcelona
    Rev Esp Enferm Dig 78:27-30. 1990
    ..5 times lower. This pilot study demonstrates that exocrine pancreatic insufficiency can be frequent in endocrine tumors of the pancreas, and although underestimated, it can be ..
  99. ncbi Modern diagnostics of chronic pancreatitis
    Bernhard Glasbrenner
    Department of Gastroenterology, Endocrinology and Infectious Diseases, St Franziskus Hospital, Hohenzollernring 72, 48145 Münster, Germany
    Eur J Gastroenterol Hepatol 14:935-41. 2002
    ..Light to moderate exocrine pancreatic insufficiency is not detectable with adequate accuracy by tubeless function tests...
  100. ncbi Fecal elastase 1 and vitamin D3 in patients with osteoporotic bone fractures
    Sacha T W Mann
    Department of Internal Medicine, Medical Clinic III and Polyclinic of the Justus Liebig University, Giessen, Germany
    Eur J Med Res 13:68-72. 2008
    The aim of the present study was to clarify if patients with osteoporotic bone fractures have exocrine pancreatic insufficiency, especially reduced fecal elastase 1, connected with lowered serum levels of vitamin D3 that could be ..
  101. ncbi Cellular and humoral immune responses in atrophic lymphocytic pancreatitis in German shepherd dogs and rough-coated collies
    M E Wiberg
    Department of Clinical Veterinary Sciences, Faculty of Veterinary Medicine, University of Helsinki, P O Box 57, FIN 00014 Helsinki, Finland
    Vet Immunol Immunopathol 76:103-15. 2000
    The most common cause for the clinical signs of exocrine pancreatic insufficiency (EPI) in dogs is pancreatic acinar atrophy (PAA)...

Research Grants89

  1. Shwachman-Diamond Syndrome: Molecular Pathogenesis
    Akiko Shimamura; Fiscal Year: 2009
    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...
  2. 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 ..
  3. 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 ..
  4. 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...
  5. 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...
  6. 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...
  7. 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. ..
  8. 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. ..
  9. 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. ..
  10. 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. ..
  11. 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. ..
  12. 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. ..
  13. AAT Deficiency & Liver Diseases Caused by Proteins
    Bruce Trapnell; Fiscal Year: 2006
    ....
  14. Fourth Annual International Pulmonary Alveolar Proteinosis Research Conference
    Bruce Trapnell; Fiscal Year: 2006
    ....
  15. 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. ..
  16. 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. ..
  17. 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. ..
  18. 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. ..
  19. 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. ..
  20. GM-CSF & Alveolar Macrophage Antiviral Lung Defense
    Bruce Trapnell; Fiscal Year: 2004
    ..abstract_text> ..
  21. 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. ..
  22. 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. ..
  23. Clinical Applications of a Novel Fanconi Anemia Assay
    Akiko Shimamura; Fiscal Year: 2007
    ..abstract_text> ..
  24. NAPS2-AS: Race Dependent Risk for Pancreatitis
    David C Whitcomb; Fiscal Year: 2010
    ....
  25. 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. ..
  26. 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. ..
  27. 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. ..
  28. NOS GENE TRANSFER IN PULMONARY HYPERTENSION
    David Rodman; Fiscal Year: 2002
    ....
  29. EXTRAMURAL FACILITIES IMPROVEMENT PROGRAM
    Robert Golden; Fiscal Year: 2004
    ....
  30. 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. ..
  31. HYPOXIA AND ET-1 EFFECT ON PA SMC EFFECT ON CHANNELS
    David Rodman; Fiscal Year: 2002
    ....