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Zielenski J. Genotype and phenotype in cystic fibrosis. Respiration (Herrlisheim) 2000; 67:117-133.
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Indirect pancreatic function tests in children
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Walkowiak J, Nousia-Arvanitakis S, Henker J, et al. Indirect pancreatic function tests in children. J Pediatr Gastroenterol Nutr 2005; 40:107-114. This is an excellent review of pancreatic function tests in children with a comparison of the pros and cons of each test and an algorithm for assessment of pancreatic exocrine function.
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Now that fecal elastase is available in the United States, should clinicians start using it?
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Lankisch PG. Now that fecal elastase is available in the United States, should clinicians start using it? Curr Gastroenterol Rep 2004; 6:126-131. This is an excellent review of the literature on the sensitivity and specificity of FE-1. It focuses more on FE-1 in chronic pancreatitis, where these parameters are less definitive than in cystic fibrosis, and thus the author does not strongly recommend its use.
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Curr Gastroenterol Rep
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13C-triolein breath test for fat malabsorption in adult patients with cystic fibrosis. J Gastroenterol Hepatol 2004; 19:448-453.
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Analysis of pancreatic elastase-1 concentrations in duodenal aspirates from healthy subjects and patients with chronic pancreatitis
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Use of fecal elastase-1 to classify pancreatic status in patients with cystic fibrosis
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Borowitz D, Baker SS, Duffy L, et al. Use of fecal elastase-1 to classify pancreatic status in patients with cystic fibrosis. J Pediatr 2004; 145:322-326. This reports the largest cohort of subjects with cystic fibrosis ever surveyed using FE-1 and describes the discordance of physician prescribing behavior for pancreatic enzymes and FE-1 results.
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How useful is fecal pancreatic elastase 1 as a marker of pancreatic disease?
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Beharry S, Ellis L, Corey M, et al. How useful is fecal pancreatic elastase 1 as a marker of pancreatic disease? J Pediatr 2002; 141:84-90.
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Safety and preliminary clinical activity of a novel pancreatic enzyme preparation, TheraCLEC-Total, for the treatment of exocrine pancreatic insufficiency
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Borowitz D, Goss C, Stevens C, et al. Safety and preliminary clinical activity of a novel pancreatic enzyme preparation, TheraCLEC-Total, for the treatment of exocrine pancreatic insufficiency. Pediatr Pulmonol 2003; (Suppl 25):339.
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Serum lipase after secretin stimulation detects mild pancreatic involvement in cystic fibrosis
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Walkowiak J, Nousia-Arvanitakis S, Lisowska A, et al. Serum lipase after secretin stimulation detects mild pancreatic involvement in cystic fibrosis. J Pediatr Gastroenterol Nutr 2004; 38:430-435.
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Baker SS, Borowitz D, Duffy L, et al. Pancreatic enzyme therapy and clinical outcomes in patients with cystic fibrosis. J Pediatr 2005; 146:189-193. This cross-sectional analysis describes the discordance between pancreatic functional status based on FE-1 and growth or symptoms. It also describes the lack of concordance between patients' self-described gastrointestinal symptoms and enzyme dose.
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J Pediatr
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Cohen JR, Schall JI, Ittenbach RF, et al. Fecal elastase: pancreatic status verification and influence on nutritional status in children with cystic fibrosis. J Pediatr Gastroenterol Nutr 2005; 40:438-444.
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Faecal elastase-1 test is superior to faecal lipase test in the assessment of exocrine pancreatic function in cystic fibrosis
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Walkowiak J, Lisowska A, Przyslawski J, et al. Faecal elastase-1 test is superior to faecal lipase test in the assessment of exocrine pancreatic function in cystic fibrosis. Acta Paediatr 2004; 93:1042-1045.
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Fecal chymotrypsin and elastase-1 determination on one single stool collected at random: Diagnostic value for exocrine pancreatic status
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Molinari I, Souare K, Lamireau M, et al. Fecal chymotrypsin and elastase-1 determination on one single stool collected at random: diagnostic value for exocrine pancreatic status. Clin Biochem 2004; 37:758-763.
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