Published in N Engl J Med on May 01, 1997
Progress in cystic fibrosis and the CF Therapeutics Development Network. Thorax (2012) 1.12
Treatment of Gastrointestinal Problems in Cystic Fibrosis. Curr Treat Options Gastroenterol (2003) 1.06
Enzyme replacement therapy for pancreatic insufficiency: present and future. Clin Exp Gastroenterol (2011) 0.94
Diagnosis and treatment of pancreatic exocrine insufficiency. World J Gastroenterol (2013) 0.90
Exocrine pancreatic insufficiency in adults: a shared position statement of the Italian Association for the Study of the Pancreas. World J Gastroenterol (2013) 0.88
Delayed release pancrelipase for treatment of pancreatic exocrine insufficiency associated with chronic pancreatitis. Ther Clin Risk Manag (2009) 0.84
Systematic review: pancreatic enzyme treatment of malabsorption associated with chronic pancreatitis. Aliment Pharmacol Ther (2008) 0.81
Diagnosing malabsorption with systemic lipid profiling: pharmacokinetics of pentadecanoic acid and triheptadecanoic acid following oral administration in healthy subjects and subjects with cystic fibrosis. Int J Clin Pharmacol Ther (2013) 0.81
In Vitro Comparison of Physical Parameters, Enzyme Activity, Acid Resistance, and pH Dissolution Characteristics of Enteric-Coated Pancreatic Enzyme Preparations: Implications for Clinical Variability and Pharmacy Substitution. J Pediatr Pharmacol Ther (2007) 0.80
Beyond pancreatic insufficiency and liver disease in cystic fibrosis. Eur J Pediatr (2016) 0.79
Delayed release pancrelipase for the treatment of pancreatic exocrine insufficiency associated with cystic fibrosis. Ther Clin Risk Manag (2008) 0.78
Indomethacin and pancreatic enzymes synergistically damage intestine of rats. Dig Dis Sci (1998) 0.78
Uses and abuses of enzyme therapy in cystic fibrosis. J R Soc Med (1998) 0.78
Study design considerations for evaluating the efficacy and safety of pancreatic enzyme replacement therapy in patients with cystic fibrosis. Clin Investig (Lond) (2013) 0.78
Postcibal gastric emptying of pancreatin pellets: effects of dose and meal oil. Dig Dis Sci (2001) 0.78
Maintenance of growth in cystic fibrosis despite reduction in pancreatic enzyme supplementation. Arch Dis Child (1998) 0.77
Pathophysiology of cystic fibrosis and drugs used in associated digestive tract diseases. World J Gastroenterol (2013) 0.77
Is fibrosing colonopathy an immune mediated disease? Arch Dis Child (1997) 0.77
Emergency laparoscopic ileo-colic resection and primary intracorporeal anastomosis for Crohn's acute ileitis with free perforation and faecal peritonitis: first ever reported laparoscopic treatment. Springerplus (2016) 0.76
Calprotectin in cystic fibrosis. BMC Pediatr (2014) 0.76
Successful non-invasive treatment of stricturing fibrosing colonopathy in an adult patient. Eur J Med Res (2011) 0.75
Facing Terminal Ileitis: Going Beyond Crohn's Disease. Gastroenterology Res (2016) 0.75
Oral ulceration and bleeding associated with pancreatic enzyme supplementation in a German shepherd with pancreatic acinar atrophy. Can Vet J (2006) 0.75
Update on intestinal strictures. J R Soc Med (1999) 0.75
Treatment of pancreatic exocrine deficiency. World J Surg (2003) 0.75
Gastric lipase secretion in children with gastritis. Nutrients (2013) 0.75
Practical guide to exocrine pancreatic insufficiency - Breaking the myths. BMC Med (2017) 0.75
Intermittent administration of inhaled tobramycin in patients with cystic fibrosis. Cystic Fibrosis Inhaled Tobramycin Study Group. N Engl J Med (1999) 6.23
Development and validation of a pediatric Crohn's disease activity index. J Pediatr Gastroenterol Nutr (1991) 4.11
Cancer in patients on dialysis for end-stage renal disease: an international collaborative study. Lancet (1999) 4.04
The risk of cancer among patients with cystic fibrosis. Cystic Fibrosis and Cancer Study Group. N Engl J Med (1995) 2.49
Cigarette smoking accelerates progression of alcoholic chronic pancreatitis. Gut (2005) 2.38
Gastrointestinal manifestations of cystic fibrosis: a review. Gastroenterology (1981) 2.38
Diagnosis, screening and management of cystic fibrosis related diabetes mellitus: a consensus conference report. Diabetes Res Clin Pract (1999) 2.32
Hemoconcentration: an early marker of severe and/or necrotizing pancreatitis? A critical appraisal. Am J Gastroenterol (2001) 2.28
Why are small-bowel tumours so rare? Lancet (1973) 2.24
Propranolol in prevention of portal hypertensive hemorrhage in children: a pilot study. J Pediatr Gastroenterol Nutr (1999) 2.22
Identification of the human papilloma virus-1a E4 gene products. EMBO J (1986) 2.21
Review article: epidemiology of male breast cancer. A meta-analysis of published case-control studies and discussion of selected aetiological factors. Int J Cancer (1993) 2.20
Troponin C-like proteins (calmodulins) from mammalian smooth muscle and other tissues. Biochem J (1979) 2.10
Development of the human gastrointestinal tract. A review. Gastroenterology (1976) 1.93
The relationship between biological activity and primary structure of troponin I from white skeletal muscle of the rabbit. Biochem J (1976) 1.84
Microvillus inclusion disease: an inherited defect of brush-border assembly and differentiation. N Engl J Med (1989) 1.77
Nutritional basis of growth failure in children and adolescents with Crohn's disease. Gastroenterology (1979) 1.73
Intraepithelial eosinophils: a new diagnostic criterion for reflux esophagitis. Gastroenterology (1982) 1.73
Correlation of sweat chloride concentration with classes of the cystic fibrosis transmembrane conductance regulator gene mutations. J Pediatr (1995) 1.70
Specific association of activated MAP kinase kinase kinase (Raf) with the plasma membranes of ras-transformed retinal cells. Oncogene (1993) 1.69
Epidemiologic aspects of gallbladder cancer: a case-control study of the SEARCH Program of the International Agency for Research on Cancer. J Natl Cancer Inst (1997) 1.68
Cigarette smoking as a risk factor for pancreatic cancer in patients with hereditary pancreatitis. JAMA (2001) 1.64
Growth failure in children with inflammatory bowel disease: a prospective study. Gastroenterology (1993) 1.62
Chronic diarrhea of childhood and the misuse of elimination diets. J Pediatr (1979) 1.62
Skin signs in acute pancreatitis: frequency and implications for prognosis. J Intern Med (2009) 1.58
Serum and lower respiratory tract drug concentrations after tobramycin inhalation in young children with cystic fibrosis. J Pediatr (2001) 1.56
Homology between a human apoptosis specific protein and the product of APG5, a gene involved in autophagy in yeast. FEBS Lett (1998) 1.55
Hepatobiliary scintigraphy in children with cystic fibrosis and liver disease. J Nucl Med (1994) 1.53
A clinical scoring system for chronic inflammatory bowel disease in children. Dig Dis Sci (1979) 1.52
Comparison of amino acid sequence of troponin I from different striated muscles. Nature (1978) 1.52
Effect of cimetidine and sodium bicarbonate on pancreatic replacement therapy in cystic fibrosis. Gut (1980) 1.50
Acute pancreatitis in childhood. J Pediatr (1988) 1.49
Shwachman syndrome: phenotypic manifestations of sibling sets and isolated cases in a large patient cohort are similar. J Pediatr (1999) 1.47
Hypothermia does not result in more complications after colon surgery. Am Surg (1999) 1.46
Letter: An unusual hernia. JAMA (1976) 1.46
A position paper of the North American Society for Pediatric Gastroenterology and Nutrition. Pediatric gastroenterology Workforce Survey and future supply and demand. J Pediatr Gastroenterol Nutr (1998) 1.45
Bile acid secretion in cystic fibrosis: evidence for a defect unrelated to fat malabsorption. Gut (1986) 1.44
Distribution of primary renal diseases leading to end-stage renal failure in the United States, Europe, and Australia/New Zealand: results from an international comparative study. Am J Kidney Dis (2000) 1.43
Glycoprotein synthesis, transport, and secretion by epithelial cells of human rectal mucosa: normal and cystic fibrosis. Lab Invest (1977) 1.42
Death takes a ride. Alcohol-associated single vehicle fatalities revisited. N Y State J Med (1990) 1.40
Gastric mucosal biopsy in eosinophilic (allergic) gastroenteritis. Gastroenterology (1977) 1.39
Pathogenesis of shigella diarrhea: evidence for a developmentally regulated glycolipid receptor for shigella toxin involved in the fluid secretory response of rabbit small intestine. J Infect Dis (1988) 1.38
Pancreatitis and pancreatic cancer risk: a pooled analysis in the International Pancreatic Cancer Case-Control Consortium (PanC4). Ann Oncol (2012) 1.35
Independent smulation of secretion and protein snthesis in rat parotid gland. The influence of epinephrine and dibutyryl cyclic adenosine 3',5'-monophosphate. J Biol Chem (1969) 1.35
Age-related alterations of immunoreactive pancreatic cationic trypsinogen in sera from cystic fibrosis patients with and without pancreatic insufficiency. Pediatr Res (1986) 1.34
Growth, course, and prognosis after surgery for Crohn's disease in children and adolescents. Pediatrics (1977) 1.31
Effects of long-term nutritional rehabilitation on body composition and clinical status in malnourished children and adolescents with cystic fibrosis. J Pediatr (1985) 1.30
Recommendations for management of liver and biliary tract disease in cystic fibrosis. Cystic Fibrosis Foundation Hepatobiliary Disease Consensus Group. J Pediatr Gastroenterol Nutr (1999) 1.28
Treatment of distal intestinal obstruction syndrome in cystic fibrosis with a balanced intestinal lavage solution. Lancet (1986) 1.28
Risk of pancreatic carcinoma in tropical calcifying pancreatitis: an epidemiologic study. Pancreas (1994) 1.28
Pathology of pancreatic and intestinal disorders in cystic fibrosis. J R Soc Med (1998) 1.28
Skeletal phenotype in patients with Shwachman-Diamond syndrome and mutations in SBDS. Clin Genet (2004) 1.27
Protracted diarrhea of infancy treated by intravenous alimentation. I. Clinical studies of 16 infants. Am J Dis Child (1973) 1.27
Evidence for a primary defect of pancreatic HCO3-secretion in cystic fibrosis. Pediatr Res (1982) 1.26
Temporal patterns in air pollution and hospital admissions. Environ Res (1992) 1.26
Uncertainty in the diagnosis of cystic fibrosis: possible role of in vivo nasal potential difference measurements. J Pediatr (1998) 1.26
Shwachman syndrome: exocrine pancreatic dysfunction and variable phenotypic expression. Gastroenterology (1996) 1.25
Development of the human gastrointestinal tract: twenty years of progress. Gastroenterology (1999) 1.25
Pregnancy in cystic fibrosis of the pancreas. JAMA (1966) 1.23
Shwachman-Diamond syndrome with exocrine pancreatic dysfunction and bone marrow failure maps to the centromeric region of chromosome 7. Am J Hum Genet (2001) 1.21
The amino acid sequence of troponin I from rabbit skeletal muscle. Biochem J (1975) 1.21
Chronic constipation in childhood: a longitudinal study of 186 patients. J Pediatr Gastroenterol Nutr (1984) 1.20
Restriction of lactase gene expression along the proximal-to-distal axis of rat small intestine occurs during postnatal development. Gastroenterology (1994) 1.19
Calmodulin-binding proteins from brain and other tissues. Biochem J (1979) 1.19
Colipase and lipase secretion in childhood-onset pancreatic insufficiency. Delineation of patients with steatorrhea secondary to relative colipase deficiency. Gastroenterology (1984) 1.17
Chronic diarrhea associated with Clostridium difficile in children. Am J Dis Child (1983) 1.17
Congenital hypertrophic pyloric stenosis: the role of gastrin reevaluated. Pediatrics (1978) 1.17
Structural determinants present in the C-terminal binding protein binding site of adenovirus early region 1A proteins. J Biol Chem (1998) 1.17
Cellular localization and T antigen binding of the retinoblastoma protein. Oncogene (1992) 1.16
Use of pancreatic enzyme supplements for patients with cystic fibrosis in the context of fibrosing colonopathy. Consensus Committee. J Pediatr (1995) 1.16
Epidemiology of pancreatic diseases in Lüneburg County. A study in a defined german population. Pancreatology (2002) 1.16
An update on the screening, diagnosis, management, and treatment of vitamin D deficiency in individuals with cystic fibrosis: evidence-based recommendations from the Cystic Fibrosis Foundation. J Clin Endocrinol Metab (2012) 1.15
Genetic polymorphism of N-acetyltransferases, glutathione S-transferase M1 and NAD(P)H:quinone oxidoreductase in relation to malignant and benign pancreatic disease risk. The International Pancreatic Disease Study Group. Eur J Cancer Prev (1998) 1.15
The use of corticosteroids in celiac crisis. J Pediatr (1972) 1.15
Induction of Epstein-Barr virus lytic cycle by tumor-promoting and non-tumor-promoting phorbol esters requires active protein kinase C. J Virol (1991) 1.14
Does bile promote extra-colonic cancer? Lancet (1978) 1.14
Alcohol and trauma. Ann Emerg Med (1984) 1.13
The amino acid sequence of rabbit cardiac troponin I. Biochem J (1976) 1.12
Acute pancreatitis: does gender matter? Dig Dis Sci (2001) 1.12
Coordinate expression of lactase-phlorizin hydrolase mRNA and enzyme levels in rat intestine during development. J Biol Chem (1990) 1.12
Gallstones and risk of gallbladder cancer. J Natl Cancer Inst (1985) 1.12
Dietary intakes of young children with cystic fibrosis: is there a difference? J Pediatr Gastroenterol Nutr (1996) 1.12
Renal failure in infancy, with special reference to the use of peritoneal dialysis. J Pediatr Surg (1966) 1.12
Preliminary studies on the electron microscopic localization of sites of sodium transport in the human eccrine sweat gland. Bibl Paediatr (1967) 1.11
Differential activation of intestinal gene promoters: functional interactions between GATA-5 and HNF-1 alpha. Am J Physiol Gastrointest Liver Physiol (2001) 1.11
Late gastrointestinal bleeding and protein loss after distal small-bowel resection in infancy. J Pediatr Gastroenterol Nutr (1989) 1.11
The amino acid sequence of the delta subunit (calmodulin) of rabbit skeletal muscle phosphorylase kinase. Eur J Biochem (1981) 1.10
Salmonella bacteremia in the first year of life. J Pediatr (1980) 1.10
Hernia-sac cancer. Lancet (1969) 1.10
Regulation of the 26S proteasome by adenovirus E1A. EMBO J (2000) 1.09
Biosynthesis, glycosylation, and intracellular transport of intestinal lactase-phlorizin hydrolase in rat. J Biol Chem (1987) 1.09
Risk of transmission of HIV from surgeon to patient. N Engl J Med (1991) 1.08
Effects of a plasticizer leached from polyvinyl chloride on the subhuman primate: a consequence of chronic transfusion therapy. J Lab Clin Med (1977) 1.08
Kehr's sign--a neglected aid in rupture of the spleen. N Engl J Med (1966) 1.08
The replicative capacities of large E1B-null group A and group C adenoviruses are independent of host cell p53 status. J Virol (1999) 1.07
The preparation of calmodulins from barley (Hordeum sp.) and basidiomycete fungi. Biochem J (1980) 1.06
Pathogenesis of Shigella diarrhea: rabbit intestinal cell microvillus membrane binding site for Shigella toxin. Infect Immun (1986) 1.06
Approaches to inflammatory bowel disease in childhood and adolescence. Pediatr Clin North Am (1975) 1.06