메뉴 건너뛰기




Volumn 13, Issue 3, 2016, Pages 175-185

Cystic fibrosis from the gastroenterologist's perspective

Author keywords

[No Author keywords available]

Indexed keywords

ANTIBIOTIC AGENT; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; IVACAFTOR; PROBIOTIC AGENT; URSODEOXYCHOLIC ACID;

EID: 84959358838     PISSN: 17595045     EISSN: 17595053     Source Type: Journal    
DOI: 10.1038/nrgastro.2015.226     Document Type: Review
Times cited : (119)

References (122)
  • 1
    • 0024424270 scopus 로고
    • Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA
    • Riordan, J. R., et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 245, 1066 (1989
    • (1989) Science , vol.245 , pp. 1066
    • Riordan, J.R.1
  • 2
    • 0003766974 scopus 로고    scopus 로고
    • 3rd edn (eds Hodson, M., et al. (Edward Arnold Ltd
    • Walters, S.,&Mehta, A. in Cystic Fibrosis 3rd edn (eds Hodson, M., et al.) 21-45 (Edward Arnold Ltd, 2007
    • (2007) Cystic Fibrosis , pp. 21-45
    • Walters, S.1    Mehta, A.2
  • 5
    • 0028967358 scopus 로고
    • The risk of cancer among patients with cystic fibrosis
    • Neglia, J. P., et al. The risk of cancer among patients with cystic fibrosis. N. Engl. J. Med. 332, 494-499 (1995
    • (1995) N. Engl. J. Med , vol.332 , pp. 494-499
    • Neglia, J.P.1
  • 6
    • 0037420207 scopus 로고    scopus 로고
    • Cancer risk in nontransplanted and transplanted cystic fibrosis patients: A 10 year study
    • Maisonneuve, P., et al. Cancer risk in nontransplanted and transplanted cystic fibrosis patients: a 10 year study. J. Natl Cancer Inst. 95, 381-387 (2003
    • (2003) J. Natl Cancer Inst , vol.95 , pp. 381-387
    • Maisonneuve, P.1
  • 7
    • 84872557485 scopus 로고    scopus 로고
    • Cancer risk in cystic fibrosis: A 20 year nationwide study from the United States
    • Maisonneuve, P., et al. Cancer risk in cystic fibrosis: a 20 year nationwide study from the United States. J. Natl Cancer Inst. 105, 122-129 (2013
    • (2013) J. Natl Cancer Inst , vol.105 , pp. 122-129
    • Maisonneuve, P.1
  • 8
    • 6344231597 scopus 로고    scopus 로고
    • Clinical manifestations of cystic fibrosis among patients diagnosed in adulthood
    • Gilljam, K., et al. Clinical manifestations of cystic fibrosis among patients diagnosed in adulthood. Chest 126, 1215-1224 (2004
    • (2004) Chest , vol.126 , pp. 1215-1224
    • Gilljam, K.1
  • 9
    • 78650477795 scopus 로고    scopus 로고
    • Type of CFTR mutation determines risk of pancreatitis in patients with cystic fibrosis
    • Ooi, C. Y., et al. Type of CFTR mutation determines risk of pancreatitis in patients with cystic fibrosis. Gastroenterology 140, 153-161 (2011
    • (2011) Gastroenterology , vol.140 , pp. 153-161
    • Ooi, C.Y.1
  • 10
    • 84862763252 scopus 로고    scopus 로고
    • Comparing the American and European diagnostic guidelines for cystic fibrosis: Same disease different language?
    • Ooi, C. Y., et al. Comparing the American and European diagnostic guidelines for cystic fibrosis: same disease, different language? Thorax 67, 618-624 (2012
    • (2012) Thorax , vol.67 , pp. 618-624
    • Ooi, C.Y.1
  • 11
    • 84893972760 scopus 로고    scopus 로고
    • Does extensive genotyping and nasal potential difference testing clarify the diagnosis of cystic fibrosis among patients with single-organ manifestations of cystic fibrosis?
    • Ooi, C. Y., et al. Does extensive genotyping and nasal potential difference testing clarify the diagnosis of cystic fibrosis among patients with single-organ manifestations of cystic fibrosis? Thorax 69, 254-260 (2014
    • (2014) Thorax , vol.69 , pp. 254-260
    • Ooi, C.Y.1
  • 12
    • 84866142563 scopus 로고    scopus 로고
    • Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in pancreatitis
    • Ooi, C. Y.,&Durie, P. Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in pancreatitis. J. Cyst. Fibros. 11, 355-362 (2012
    • (2012) J. Cyst. Fibros , vol.11 , pp. 355-362
    • Ooi, C.Y.1    Durie, P.2
  • 13
    • 80455162465 scopus 로고    scopus 로고
    • A CFTR potentiator in patients with cystic fibrosis and the G551D mutation
    • Ramsey, B. W., et al. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N. Engl. J. Med. 365, 1663-1672 (2011
    • (2011) N. Engl. J. Med , vol.365 , pp. 1663-1672
    • Ramsey, B.W.1
  • 14
    • 84937035647 scopus 로고    scopus 로고
    • Lumacaftor-ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR
    • Wainwright, C. E., et al. Lumacaftor-ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR. N. Engl. J. Med. 373, 220-231 (2015
    • (2015) N. Engl. J. Med , vol.373 , pp. 220-231
    • Wainwright, C.E.1
  • 15
    • 0027295745 scopus 로고
    • A suggested nomenclature for designating mutations
    • Beaudet, A. L.,&Tsui, L. C. A suggested nomenclature for designating mutations. Hum. Mutat. 2, 245-248 (1993
    • (1993) Hum. Mutat , vol.2 , pp. 245-248
    • Beaudet, A.L.1    Tsui, L.C.2
  • 16
    • 84926107843 scopus 로고    scopus 로고
    • Prediction of acute pancreatitis risk based on PIP score in children with cystic fibrosis
    • Terlizzi, V., et al. Prediction of acute pancreatitis risk based on PIP score in children with cystic fibrosis. J. Cyst. Fibros. 13, 579-584 (2014
    • (2014) J. Cyst. Fibros , vol.13 , pp. 579-584
    • Terlizzi, V.1
  • 18
    • 0032912589 scopus 로고    scopus 로고
    • Structure and function of the cystic fibrosis transmembrane conductance regulator chloride channel
    • Sheppard, D. N.,&Welsh, M. J. Structure and function of the cystic fibrosis transmembrane conductance regulator chloride channel. Physiol. Rev. 79 (SUPPL 1), S23-S45 (1999
    • (1999) Physiol. Rev , vol.79 , pp. S23-S45
    • Sheppard, D.N.1    Welsh, M.J.2
  • 19
    • 0020700816 scopus 로고
    • Chloride impermeability in cystic fibrosis
    • Quinton, P. M. Chloride impermeability in cystic fibrosis. Nature 301, 421-422 (1983
    • (1983) Nature , vol.301 , pp. 421-422
    • Quinton, P.M.1
  • 20
    • 0028982894 scopus 로고
    • CFTR as a cAMP-dependent regulator of sodium channels
    • Stutts, M. J., et al. CFTR as a cAMP-dependent regulator of sodium channels. Science 269, 847-850 (1995
    • (1995) Science , vol.269 , pp. 847-850
    • Stutts, M.J.1
  • 21
    • 0028982894 scopus 로고
    • CFTR as a cAMP-regulator of Na+ channels
    • Stutts, M. J. CFTR as a cAMP-regulator of Na+ channels. Science 269, 847-850 (1995
    • (1995) Science , vol.269 , pp. 847-850
    • Stutts, M.J.1
  • 22
    • 84881476486 scopus 로고    scopus 로고
    • Intestinal pH and gastrointestinal transit profiles in cystic fibrosis patients measured by wireless motility capsule
    • Gelfond, D., et al. Intestinal pH and gastrointestinal transit profiles in cystic fibrosis patients measured by wireless motility capsule. Dig. Dis. Sci. 58, 2275-2281 (2013
    • (2013) Dig. Dis. Sci , vol.58 , pp. 2275-2281
    • Gelfond, D.1
  • 23
    • 70349223856 scopus 로고    scopus 로고
    • Normal mouse intestinal mucus release requires cystic fibrosis transmembrane regulator-dependent bicarbonate secretion
    • Garcia, M. A., Yang, N.,&Quinton, P. M. Normal mouse intestinal mucus release requires cystic fibrosis transmembrane regulator-dependent bicarbonate secretion. J. Clin. Invest. 119, 2613-2622 (2009
    • (2009) J. Clin. Invest , vol.119 , pp. 2613-2622
    • Garcia, M.A.1    Yang, N.2    Quinton, P.M.3
  • 24
    • 79958122789 scopus 로고    scopus 로고
    • Recommendations for the classification of diseases as CFTR-related disorders
    • Bombieri, C., et al. Recommendations for the classification of diseases as CFTR-related disorders. J. Cyst. Fibros. 10 (SUPPL 2), S86-S102 (2011
    • (2011) J. Cyst. Fibros , vol.10 , pp. S86-S102
    • Bombieri, C.1
  • 25
    • 0038298386 scopus 로고    scopus 로고
    • Molecular consequences of cystic fibrosis transmembrane regulator (CFTR) gene mutations in the exocrine pancreas
    • Ahmed, N., et al. Molecular consequences of cystic fibrosis transmembrane regulator (CFTR) gene mutations in the exocrine pancreas. Gut 52, 1159-1164 (2003
    • (2003) Gut , vol.52 , pp. 1159-1164
    • Ahmed, N.1
  • 26
    • 0026734588 scopus 로고
    • Genetic determination of exocrine pancreatic function in cystic fibrosis
    • Kristidis, P., et al. Genetic determination of exocrine pancreatic function in cystic fibrosis. Am. J. Hum. Genet. 50, 1178-1184 (1992
    • (1992) Am. J. Hum. Genet , vol.50 , pp. 1178-1184
    • Kristidis, P.1
  • 27
    • 0036892322 scopus 로고    scopus 로고
    • Genotype and phenotype correlations in patients with cystic fibrosis and pancreatitis
    • Durno, C., et al. Genotype and phenotype correlations in patients with cystic fibrosis and pancreatitis. Gastroenterology 123, 1857-1864 (2002
    • (2002) Gastroenterology , vol.123 , pp. 1857-1864
    • Durno, C.1
  • 28
    • 39849104146 scopus 로고    scopus 로고
    • The changing face of the exocrine pancreas in cystic fibrosis: The correlation between pancreatic status, pancreatitis and cystic fibrosis genotype
    • Augarten, A., et al. The changing face of the exocrine pancreas in cystic fibrosis: the correlation between pancreatic status, pancreatitis and cystic fibrosis genotype. Eur. J. Gastroenterol. Hepatol. 20, 164-168 (2008
    • (2008) Eur. J. Gastroenterol. Hepatol , vol.20 , pp. 164-168
    • Augarten, A.1
  • 29
    • 0020070795 scopus 로고
    • Improved respiratory prognosis in CF patients with normal fat absorption
    • Gaskin, K., et al. Improved respiratory prognosis in CF patients with normal fat absorption. J. Pediatr. 100, 857-862 (1982
    • (1982) J. Pediatr , vol.100 , pp. 857-862
    • Gaskin, K.1
  • 30
    • 34547164336 scopus 로고    scopus 로고
    • Patterns of GI disease in adulthood associated with mutations in the CFTR gene
    • Wilschanski, M.,&Durie, P. R. Patterns of GI disease in adulthood associated with mutations in the CFTR gene. Gut 56, 1153-1163 (2007
    • (2007) Gut , vol.56 , pp. 1153-1163
    • Wilschanski, M.1    Durie, P.R.2
  • 31
    • 0036095120 scopus 로고    scopus 로고
    • The role of hepatic stellate cells and transforming growth factor b1 in cystic fibrosis liver disease
    • Lewindon, P. J., et al. The role of hepatic stellate cells and transforming growth factor b1 in cystic fibrosis liver disease. Am. J. Pathol. 160, 1705-1715 (2002
    • (2002) Am. J. Pathol , vol.160 , pp. 1705-1715
    • Lewindon, P.J.1
  • 32
    • 0026688954 scopus 로고
    • Bile-duct destruction and collagen deposition: A prominent ultrastructural feature of the liver in cystic fibrosis
    • Linblad, A., Hultcrantz, R.,&Strandvik, B. Bile-duct destruction and collagen deposition: a prominent ultrastructural feature of the liver in cystic fibrosis. Hepatology 16, 372-381 (1992
    • (1992) Hepatology , vol.16 , pp. 372-381
    • Linblad, A.1    Hultcrantz, R.2    Strandvik, B.3
  • 33
    • 84922702335 scopus 로고    scopus 로고
    • Intestinal lesions are associated with altered intestinal microbiome and are more frequent in children and young adults with cystic fibrosis and cirrhosis
    • Flass, T., et al. Intestinal lesions are associated with altered intestinal microbiome and are more frequent in children and young adults with cystic fibrosis and cirrhosis. PLoS ONE 10, e0116967 (2015
    • (2015) PLoS ONE , vol.10 , pp. e0116967
    • Flass, T.1
  • 34
    • 70149106634 scopus 로고    scopus 로고
    • Genetic modifiers of liver disease in cystic fibrosis
    • Bartlett, J. R., et al. Genetic modifiers of liver disease in cystic fibrosis. JAMA 302, 1076-1083 (2009
    • (2009) JAMA , vol.302 , pp. 1076-1083
    • Bartlett, J.R.1
  • 35
    • 78751564502 scopus 로고    scopus 로고
    • Importance of hepatic fibrosis in cystic fibrosis and the predictive value of liver biopsy
    • Lewindon, P. J., et al. Importance of hepatic fibrosis in cystic fibrosis and the predictive value of liver biopsy. Hepatology 53, 193-201 (2011
    • (2011) Hepatology , vol.53 , pp. 193-201
    • Lewindon, P.J.1
  • 36
    • 79952215545 scopus 로고    scopus 로고
    • Noncirrhotic presinusoidal portal hypertension is common in cystic fibrosis-Associated liver disease
    • Witters, P., et al. Noncirrhotic presinusoidal portal hypertension is common in cystic fibrosis-Associated liver disease. Hepatology 53, 1064-1065 (2011
    • (2011) Hepatology , vol.53 , pp. 1064-1065
    • Witters, P.1
  • 37
    • 0032753456 scopus 로고    scopus 로고
    • Natural history of liver disease in cystic fibrosis
    • Lindblad, A., Glaumann, H.,&Strandvik, B. Natural history of liver disease in cystic fibrosis. Hepatology 30, 1151-1158 (1999
    • (1999) Hepatology , vol.30 , pp. 1151-1158
    • Lindblad, A.1    Glaumann, H.2    Strandvik, B.3
  • 38
    • 84855867318 scopus 로고    scopus 로고
    • Ursodeoxycholic acid in cystic fibrosis-Associated liver disease
    • Ooi, C. Y., Nightingale, S., Durie, P.,&Freedman, S. D. Ursodeoxycholic acid in cystic fibrosis-Associated liver disease. J. Cyst. Fibros. 11, 72-73 (2012
    • (2012) J. Cyst. Fibros , vol.11 , pp. 72-73
    • Ooi, C.Y.1    Nightingale, S.2    Durie, P.3    Freedman, S.D.4
  • 39
    • 84857017520 scopus 로고    scopus 로고
    • Diagnostic accuracy of the aspartate aminotransferase-To platelet ratio index for the prediction of hepatitis B related fibrosis: A leading meta-Analysis
    • Jin, W., et al. Diagnostic accuracy of the aspartate aminotransferase-To platelet ratio index for the prediction of hepatitis B related fibrosis: a leading meta-Analysis. BMC Gastroenterol. 12, 14 (2012
    • (2012) BMC Gastroenterol , vol.12 , pp. 14
    • Jin, W.1
  • 40
    • 79952223130 scopus 로고    scopus 로고
    • Performance of the aspartate aminotransferase-To platelet ratio index for the staging of hepatitis C related fibrosis: An updated meta-Analysis
    • Lin, Z. H., et al. Performance of the aspartate aminotransferase-To platelet ratio index for the staging of hepatitis C related fibrosis: an updated meta-Analysis. Hepatology 53, 726-736 (2011
    • (2011) Hepatology , vol.53 , pp. 726-736
    • Lin, Z.H.1
  • 41
    • 84945191347 scopus 로고    scopus 로고
    • Aspartate aminotransferase to platelet ratio and fibrosis 4 as biomarkers in biopsy validated pediatric cystic fibrosis liver disease
    • Leung, D. H., et al. Aspartate aminotransferase to platelet ratio and fibrosis 4 as biomarkers in biopsy validated pediatric cystic fibrosis liver disease. Hepatology 62, 1576-1583 (2015
    • (2015) Hepatology , vol.62 , pp. 1576-1583
    • Leung, D.H.1
  • 42
    • 84864412903 scopus 로고    scopus 로고
    • TIMP 1/-2 and transient elastography allow non invasive diagnosis of cystic fibrosis associated liver disease
    • Rath, T., et al. TIMP 1/-2 and transient elastography allow non invasive diagnosis of cystic fibrosis associated liver disease. Dig. Liver Dis. 44, 780-787 (2012
    • (2012) Dig. Liver Dis , vol.44 , pp. 780-787
    • Rath, T.1
  • 43
    • 79958134189 scopus 로고    scopus 로고
    • Best practice guidance for the diagnosis and management of cystic fibrosis-Associated liver disease
    • Debray, D., Kelly, D., Houwen, R., Strandvik, B.,&Colombo, C. Best practice guidance for the diagnosis and management of cystic fibrosis-Associated liver disease. J. Cyst. Fibros. 10 (SUPPL 2), S29-S36 (2011
    • (2011) J. Cyst. Fibros , vol.10 , pp. S29-S36
    • Debray, D.1    Kelly, D.2    Houwen, R.3    Strandvik, B.4    Colombo, C.5
  • 44
    • 0029899007 scopus 로고    scopus 로고
    • Serum bile acids and ursodeoxycholic acid treatment in cystic fibrosis-related liver disease
    • O'Brien, S. M., et al. Serum bile acids and ursodeoxycholic acid treatment in cystic fibrosis-related liver disease. Eur. J. Gastroenterol. Hepatol. 8, 477-483 (1996
    • (1996) Eur. J. Gastroenterol. Hepatol , vol.8 , pp. 477-483
    • O'Brien, S.M.1
  • 45
    • 0030989703 scopus 로고    scopus 로고
    • Low-dose versus high-dose ursodeoxycholic acid in cystic fibrosis-related cholestatic liver disease Results of a randomized study with 1 year follow up
    • van de Meeberg, P. C., et al. Low-dose versus high-dose ursodeoxycholic acid in cystic fibrosis-related cholestatic liver disease. Results of a randomized study with 1 year follow up. Scand. J. Gastroenterol. 32, 369-373 (1997
    • (1997) Scand. J. Gastroenterol , vol.32 , pp. 369-373
    • Van De Meeberg, P.C.1
  • 46
    • 0026584970 scopus 로고
    • Scintigraphic documentation of an improvement in hepatobiliary excretory function after treatment with ursodeoxycholic acid in patients with cystic fibrosis and associated liver disease
    • Colombo, C., et al. Scintigraphic documentation of an improvement in hepatobiliary excretory function after treatment with ursodeoxycholic acid in patients with cystic fibrosis and associated liver disease. Hepatology 15, 677-684 (1992
    • (1992) Hepatology , vol.15 , pp. 677-684
    • Colombo, C.1
  • 47
    • 70349263453 scopus 로고    scopus 로고
    • High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis
    • Lindor, K. D., et al. High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis. Hepatology 50, 808-814 (2009
    • (2009) Hepatology , vol.50 , pp. 808-814
    • Lindor, K.D.1
  • 48
    • 0038677100 scopus 로고    scopus 로고
    • Transjugular introhepatic portosystemic shunt in five children with cystic fibrosis: Long-Term results
    • Pozler, O., et al. Transjugular introhepatic portosystemic shunt in five children with cystic fibrosis: long-Term results. Hepatogastroenterology 50, 1111-1114 (2003
    • (2003) Hepatogastroenterology , vol.50 , pp. 1111-1114
    • Pozler, O.1
  • 49
    • 77952571394 scopus 로고    scopus 로고
    • A single-institution review of portosystemic shunts in children: An ongoing discussion
    • Lillegard, J. B., et al. A single-institution review of portosystemic shunts in children: an ongoing discussion. HPB Surg. 2010, 964597 (2010
    • (2010) HPB Surg , vol.2010 , pp. 964597
    • Lillegard, J.B.1
  • 50
    • 33748465311 scopus 로고    scopus 로고
    • Does splenectomy in cystic fibrosis related liver disease improve lung function and nutritional status? A case series
    • Linnane, B., Oliver, M. R.,&Robinson, P. J. Does splenectomy in cystic fibrosis related liver disease improve lung function and nutritional status? A case series. Arch. Dis. Child. 91, 771-773 (2006
    • (2006) Arch. Dis. Child , vol.91 , pp. 771-773
    • Linnane, B.1    Oliver, M.R.2    Robinson, P.J.3
  • 51
    • 34147177583 scopus 로고    scopus 로고
    • Partial splenic embolization: Successful treatment of hypersplenism, secondary to biliary cirrhosis and portal hypertension in cystic fibrosis
    • Aslanidou, E., Fotoulaki, M., Tsitouridis, I.,&Nousia Arvanitakis, S. Partial splenic embolization: successful treatment of hypersplenism, secondary to biliary cirrhosis and portal hypertension in cystic fibrosis. J. Cyst. Fibros. 6, 212-214 (2007)
    • (2007) J. Cyst. Fibros , vol.6 , pp. 212-214
    • Aslanidou, E.1    Fotoulaki, M.2    Tsitouridis, I.3    Nousia Arvanitakis, S.4
  • 52
    • 30444458549 scopus 로고    scopus 로고
    • Variceal hemorrhage and cystic fibrosis: Outcomes and implications for liver transplantation
    • Gooding, I., et al. Variceal hemorrhage and cystic fibrosis: outcomes and implications for liver transplantation. Liver Transpl. 11, 1522-1526 (2005
    • (2005) Liver Transpl , vol.11 , pp. 1522-1526
    • Gooding, I.1
  • 53
    • 79952396956 scopus 로고    scopus 로고
    • Liver transplantation in patients with cystic fibrosis: Analysis of United Network for Organ Sharing data
    • Mendizabal, M., et al. Liver transplantation in patients with cystic fibrosis: analysis of United Network for Organ Sharing data. Liver Transpl. 17, 243-250 (2011
    • (2011) Liver Transpl , vol.17 , pp. 243-250
    • Mendizabal, M.1
  • 54
    • 84902314519 scopus 로고    scopus 로고
    • Simultaneous liver-pancreas transplantation for cystic fibrosis-related liver disease: A multicenter experience
    • Bandsma, R. H., et al. Simultaneous liver-pancreas transplantation for cystic fibrosis-related liver disease: a multicenter experience. J. Cyst. Fibros. 13, 471-477 (2014
    • (2014) J. Cyst. Fibros , vol.13 , pp. 471-477
    • Bandsma, R.H.1
  • 55
    • 0042980340 scopus 로고
    • Observations in cystic fibrosis of the pancreas: The gallbladder
    • Esterly, J. R.,&Oppenheimer, E. H. Observations in cystic fibrosis of the pancreas: the gallbladder. Bull. Johns Hopkins Hosp. 110, 247-268 (1962
    • (1962) Bull Johns Hopkins Hosp , vol.110 , pp. 247-268
    • Esterly, J.R.1    Oppenheimer, E.H.2
  • 56
    • 0033039380 scopus 로고    scopus 로고
    • Sclerosing cholangitis in adults with cystic fibrosis: A magnetic resonance cholangiographic prospective study
    • Durieu, I., et al. Sclerosing cholangitis in adults with cystic fibrosis: a magnetic resonance cholangiographic prospective study. J. Hepatol. 30, 1052-1056 (1999
    • (1999) J. Hepatol , vol.30 , pp. 1052-1056
    • Durieu, I.1
  • 57
    • 34548043026 scopus 로고    scopus 로고
    • Primary sclerosing cholangitis in childhood is associated with abnormalities in cystic fibrosis-mediated chloride channel function
    • Pall, H., et al. Primary sclerosing cholangitis in childhood is associated with abnormalities in cystic fibrosis-mediated chloride channel function. J. Pediatr. 151, 255-259 (2007
    • (2007) J. Pediatr , vol.151 , pp. 255-259
    • Pall, H.1
  • 58
    • 0041563992 scopus 로고    scopus 로고
    • Increased prevalence of CFTR mutations and variants and decreased chloride secretion in primary sclerosing cholangitis
    • Sheth, S., et al. Increased prevalence of CFTR mutations and variants and decreased chloride secretion in primary sclerosing cholangitis. Hum. Genet. 113, 286-292 (2003
    • (2003) Hum. Genet , vol.113 , pp. 286-292
    • Sheth, S.1
  • 59
    • 0024599433 scopus 로고
    • Clinical and genetic comparisons of patients with cystic fibrosis, with or without meconium ileus
    • Kerem, E., et al. Clinical and genetic comparisons of patients with cystic fibrosis, with or without meconium ileus. J. Pediatr. 114, 767-773 (1989
    • (1989) J. Pediatr , vol.114 , pp. 767-773
    • Kerem, E.1
  • 60
    • 76349088287 scopus 로고    scopus 로고
    • Meconium ileus in patients with cystic fibrosis is not a risk factor for clinical deterioration and survival: The Israeli Multicenter Study
    • Efrati, O., et al. Meconium ileus in patients with cystic fibrosis is not a risk factor for clinical deterioration and survival: the Israeli Multicenter Study. J. Pediatr. Gastroenterol. Nutr. 50, 173-178 (2010
    • (2010) J. Pediatr. Gastroenterol. Nutr , vol.50 , pp. 173-178
    • Efrati, O.1
  • 61
    • 33749328098 scopus 로고    scopus 로고
    • Relative contribution of genetic and non-genetic modifiers to intestinal obstruction in cystic fibrosis
    • Blackman, S. M., et al. Relative contribution of genetic and non-genetic modifiers to intestinal obstruction in cystic fibrosis. Gastroenterology 131, 1030-1039 (2006
    • (2006) Gastroenterology , vol.131 , pp. 1030-1039
    • Blackman, S.M.1
  • 62
    • 80054884844 scopus 로고    scopus 로고
    • Issues in the management of simple and complex meconium ileus
    • Karimi, A., et al. Issues in the management of simple and complex meconium ileus. Pediatr. Surg. Int. 27, 963-968 (2011
    • (2011) Pediatr. Surg. Int , vol.27 , pp. 963-968
    • Karimi, A.1
  • 63
    • 0031877137 scopus 로고    scopus 로고
    • Increased frequency of cystic fibrosis among infants with jejunoileal atresia
    • Roberts, H. E., et al. Increased frequency of cystic fibrosis among infants with jejunoileal atresia. Am. J. Med. Genet. 78, 446-449 (1998
    • (1998) Am. J. Med. Genet , vol.78 , pp. 446-449
    • Roberts, H.E.1
  • 64
    • 34547099062 scopus 로고    scopus 로고
    • Investigation for cystic fibrosis in infants with jejunoileal atresia in the Netherlands: A 35 year experience with 114 cases
    • Stollman, T. H., Wijnen, R. M.,&Draaisma, J. M. Investigation for cystic fibrosis in infants with jejunoileal atresia in the Netherlands: a 35 year experience with 114 cases. Eur. J. Pediatr. 166, 989-990 (2007
    • (2007) Eur. J. Pediatr , vol.166 , pp. 989-990
    • Stollman, T.H.1    Wijnen, R.M.2    Draaisma, J.M.3
  • 65
    • 84897442150 scopus 로고    scopus 로고
    • Colonic atresia presenting as neonatal bowel obstruction in cystic fibrosis
    • Yap, T. S., Jiwane, A., Belessis, Y.,&Ooi, C. Y. Colonic atresia presenting as neonatal bowel obstruction in cystic fibrosis. J. Pediatr. Gastroenterol. Nutr. 58, e37-e38 (2014
    • (2014) J. Pediatr. Gastroenterol. Nutr , vol.58 , pp. e37-e38
    • Yap, T.S.1    Jiwane, A.2    Belessis, Y.3    Ooi, C.Y.4
  • 66
    • 2542568707 scopus 로고    scopus 로고
    • Distal intestinal obstruction syndrome in adults with cystic fibrosis
    • Dray, X., et al. Distal intestinal obstruction syndrome in adults with cystic fibrosis. Clin. Gastroenterol. Hepatol. 2, 498-503 (2004
    • (2004) Clin. Gastroenterol. Hepatol , vol.2 , pp. 498-503
    • Dray, X.1
  • 67
    • 70349433652 scopus 로고    scopus 로고
    • Distal intestinal obstruction syndrome (DIOS) in patients with cystic fibrosis after lung transplantation
    • Morton, J. R., et al. Distal intestinal obstruction syndrome (DIOS) in patients with cystic fibrosis after lung transplantation. J. Gastrointest. Surg. 13, 1448-1453 (2009
    • (2009) J. Gastrointest. Surg , vol.13 , pp. 1448-1453
    • Morton, J.R.1
  • 68
    • 0036076830 scopus 로고    scopus 로고
    • Emergency colonoscopy for distal intestinal obstruction syndrome in cystic fibrosis patients
    • Shidrawi, R., et al. Emergency colonoscopy for distal intestinal obstruction syndrome in cystic fibrosis patients. Gut 51, 285-286 (2002
    • (2002) Gut , vol.51 , pp. 285-286
    • Shidrawi, R.1
  • 69
    • 0033069576 scopus 로고    scopus 로고
    • Digestive alterations in cystic fibrosis: Retrospective study of a series of 46 adult patients
    • Perez-Aguilar, F., et al. Digestive alterations in cystic fibrosis: retrospective study of a series of 46 adult patients. Gastroenterol. Hepatol. 22, 72-78 (1999
    • (1999) Gastroenterol. Hepatol , vol.22 , pp. 72-78
    • Perez-Aguilar, F.1
  • 70
    • 67650697678 scopus 로고    scopus 로고
    • Lymphocytic leiomyositis and myenteric ganglionitis are intrinsic features of cystic fibrosis: Studies in distal intestinal obstruction syndrome and meconium ileus
    • Smith, V. V., et al. Lymphocytic leiomyositis and myenteric ganglionitis are intrinsic features of cystic fibrosis: studies in distal intestinal obstruction syndrome and meconium ileus. J. Pediatr. Gastroenterol. Nutr. 49, 42-51 (2009
    • (2009) J. Pediatr. Gastroenterol. Nutr , vol.49 , pp. 42-51
    • Smith, V.V.1
  • 71
    • 76149106847 scopus 로고    scopus 로고
    • Defining DIOS and constipation in cystic fibrosis with a multicentre study on the incidence, characteristics, and treatment of DIOS
    • Houwen, R. H., et al. Defining DIOS and constipation in cystic fibrosis with a multicentre study on the incidence, characteristics, and treatment of DIOS. J. Pediatr. Gastroenterol. Nutr. 50, 38-42 (2010
    • (2010) J. Pediatr. Gastroenterol. Nutr , vol.50 , pp. 38-42
    • Houwen, R.H.1
  • 72
    • 33744762758 scopus 로고    scopus 로고
    • Review of the abdominal manifestations of cystic fibrosis in the adult patient
    • Robertson, M. D., Choe, K. A.,&Joseph, P. M. Review of the abdominal manifestations of cystic fibrosis in the adult patient. Radiographics 26, 679-690 (2006
    • (2006) Radiographics , vol.26 , pp. 679-690
    • Robertson, M.D.1    Choe, K.A.2    Joseph, P.M.3
  • 73
    • 0026029073 scopus 로고
    • Appendicitis in cystic fibrosis
    • Shields, M. D., et al. Appendicitis in cystic fibrosis. Arch. Dis. Child. 66, 307-310 (1991
    • (1991) Arch. Dis. Child , vol.66 , pp. 307-310
    • Shields, M.D.1
  • 74
  • 75
    • 84867758702 scopus 로고    scopus 로고
    • Update of faecal markers of inflammation in children with cystic fibrosis
    • Lee, J. M., et al. Update of faecal markers of inflammation in children with cystic fibrosis. Mediators Inflamm. 2012, 948367 (2012
    • (2012) Mediators Inflamm , vol.2012 , pp. 948367
    • Lee, J.M.1
  • 76
    • 84942196547 scopus 로고    scopus 로고
    • Fecal human β-defensin 2 in children with cystic fibrosis: Is there a diminished intestinal innate immune response?
    • Ooi, C. Y., et al. Fecal human β-defensin 2 in children with cystic fibrosis: is there a diminished intestinal innate immune response? Dig. Dis. Sci. 60, 2946-2952 (2015
    • (2015) Dig. Dis. Sci , vol.60 , pp. 2946-2952
    • Ooi, C.Y.1
  • 77
    • 84937517916 scopus 로고    scopus 로고
    • Associations between gut microbial colonization in early life and respiratory outcomes in cystic fibrosis
    • Hoen, A. G., et al. Associations between gut microbial colonization in early life and respiratory outcomes in cystic fibrosis. J. Pediatr. 167, 138-147 (2015
    • (2015) J. Pediatr , vol.167 , pp. 138-147
    • Hoen, A.G.1
  • 78
    • 84876248314 scopus 로고    scopus 로고
    • Cystic fibrosis transmembrane conductance regulator (CFTR) allelic variants relate to shifts in fecal microbiota of cystic fibrosis patients
    • Schippa, S., et al. Cystic fibrosis transmembrane conductance regulator (CFTR) allelic variants relate to shifts in fecal microbiota of cystic fibrosis patients. PLoS ONE 8, e61176 (2013
    • (2013) PLoS ONE , vol.8 , pp. e61176
    • Schippa, S.1
  • 79
    • 84906833163 scopus 로고    scopus 로고
    • Disrupted intestinal microbiota and intestinal inflammation in children with cystic fibrosis and its restoration with Lactobacillus GG: A randomised clinical trial
    • Bruzzese, E., et al. Disrupted intestinal microbiota and intestinal inflammation in children with cystic fibrosis and its restoration with Lactobacillus GG: a randomised clinical trial. PLoS ONE 9, e87796 (2014
    • (2014) PLoS ONE , vol.9 , pp. e87796
    • Bruzzese, E.1
  • 80
    • 83055188802 scopus 로고    scopus 로고
    • Cross-sectional and longitudinal comparisons of the predominant fecal microbiota compositions of a group of pediatric patients with cystic fibrosis and their healthy siblings
    • Duytschaever, G., et al. Cross-sectional and longitudinal comparisons of the predominant fecal microbiota compositions of a group of pediatric patients with cystic fibrosis and their healthy siblings. Appl. Environ. Microbiol. 77, 8015-8024 (2011
    • (2011) Appl. Environ. Microbiol , vol.77 , pp. 8015-8024
    • Duytschaever, G.1
  • 81
    • 84866145985 scopus 로고    scopus 로고
    • Gut dysbiosis in cystic fibrosis
    • Scanlan, P. D., et al. Gut dysbiosis in cystic fibrosis. J. Cyst. Fibros. 11, 454-455 (2012
    • (2012) J. Cyst. Fibros , vol.11 , pp. 454-455
    • Scanlan, P.D.1
  • 82
    • 84865726240 scopus 로고    scopus 로고
    • Serial analysis of the gut and respiratory microbiome in cystic fibrosis in infancy: Interaction between intestinal and respiratory tracts and impact of nutritional exposures
    • Madan, J. C., et al. Serial analysis of the gut and respiratory microbiome in cystic fibrosis in infancy: interaction between intestinal and respiratory tracts and impact of nutritional exposures. mBio 3, e00251-12 (2012
    • (2012) MBio , vol.3 , pp. e00251-e00312
    • Madan, J.C.1
  • 83
    • 34547094857 scopus 로고    scopus 로고
    • Altered transit and bacterial overgrowth in the cystic fibrosis mouse small intestine
    • De Lisle, R. C. Altered transit and bacterial overgrowth in the cystic fibrosis mouse small intestine. Am. J. Physiol. Gastrointest. Liver Physiol. 293, G104-G111 (2007
    • (2007) Am. J. Physiol. Gastrointest. Liver Physiol , vol.293 , pp. G104-G111
    • De Lisle, R.C.1
  • 84
    • 79952270847 scopus 로고    scopus 로고
    • Diagnosis and pharmacological management of small intestinal bacterial overgrowth in children with intestinal failure
    • Malik, B. A., Xie, Y. Y., Wine, E.,&Huynh, H. Q. Diagnosis and pharmacological management of small intestinal bacterial overgrowth in children with intestinal failure. Can. J. Gastroenterol. 25, 41-45 (2011
    • (2011) Can. J. Gastroenterol , vol.25 , pp. 41-45
    • Malik, B.A.1    Xie, Y.Y.2    Wine, E.3    Huynh, H.Q.4
  • 85
    • 84911974049 scopus 로고    scopus 로고
    • Improvement of digestive health and reduction in proteobacterial populations in the gut microbiota of cystic fibrosis patients using a Lactobacillus reuteri probiotic preparation: A double blind prospective study
    • del Campo, R., et al. Improvement of digestive health and reduction in proteobacterial populations in the gut microbiota of cystic fibrosis patients using a Lactobacillus reuteri probiotic preparation: a double blind prospective study. J. Cyst. Fibros. 13, 716-722 (2014
    • (2014) J. Cyst. Fibros , vol.13 , pp. 716-722
    • Del Campo, R.1
  • 86
    • 0020956124 scopus 로고
    • Isolation rate and toxigenic potential of Clostridium difficile isolates from patients with cystic fibrosis
    • Wu, T. C., McCarthy, V. P.,&Gill, V. J. Isolation rate and toxigenic potential of Clostridium difficile isolates from patients with cystic fibrosis. J. Infect. Dis. 148, 176 (1983
    • (1983) J. Infect. Dis , vol.148 , pp. 176
    • Wu, T.C.1    McCarthy, V.P.2    Gill, V.J.3
  • 87
    • 0031893534 scopus 로고    scopus 로고
    • Severe Clostridium difficile-Associated colitis in young patients with cystic fibrosis
    • Rivlin, J., et al. Severe Clostridium difficile-Associated colitis in young patients with cystic fibrosis. J. Pediatr. 132, 177-179 (1998
    • (1998) J. Pediatr , vol.132 , pp. 177-179
    • Rivlin, J.1
  • 88
    • 0023938648 scopus 로고
    • Prevalence of giardiasis in patients with cystic fibrosis
    • Roberts, D. M., et al. Prevalence of giardiasis in patients with cystic fibrosis. J. Pediatr. 112, 555-559 (1988
    • (1988) J. Pediatr , vol.112 , pp. 555-559
    • Roberts, D.M.1
  • 89
    • 4644246657 scopus 로고    scopus 로고
    • Bacterial overgrowth in the cystic fibrosis transmembrane conductance regulator null mouse small intestine
    • Norkina, O., Burnett, T. G.,&De Lisle, R. C. Bacterial overgrowth in the cystic fibrosis transmembrane conductance regulator null mouse small intestine. Infect. Immun. 72, 6040-6049 (2004
    • (2004) Infect. Immun , vol.72 , pp. 6040-6049
    • Norkina, O.1    Burnett, T.G.2    De Lisle, R.C.3
  • 90
    • 0034025852 scopus 로고    scopus 로고
    • Intestinal inflammation in cystic fibrosis
    • Smyth, R. L., et al. Intestinal inflammation in cystic fibrosis. Arch. Dis. Child. 82, 394-399 (2000
    • (2000) Arch. Dis. Child , vol.82 , pp. 394-399
    • Smyth, R.L.1
  • 91
    • 77956439080 scopus 로고    scopus 로고
    • Evidence of intestinal inflammation in patients with cystic fibrosis
    • Werlin, S. L., et al. Evidence of intestinal inflammation in patients with cystic fibrosis. J. Pediatr. Gastrtroenterol. Nutr. 51, 304-308 (2010
    • (2010) J. Pediatr. Gastrtroenterol. Nutr , vol.51 , pp. 304-308
    • Werlin, S.L.1
  • 92
    • 4844226186 scopus 로고    scopus 로고
    • Intestinal inflammation is a frequent feature of cystic fibrosis and is reduced by probiotic administration
    • Bruzzese, E., Raia, V.,&Gaudiello, G. Intestinal inflammation is a frequent feature of cystic fibrosis and is reduced by probiotic administration. Aliment. Pharmacol. Ther. 20, 813-819 (2004
    • (2004) Aliment. Pharmacol. Ther , vol.20 , pp. 813-819
    • Bruzzese, E.1    Raia, V.2    Gaudiello, G.3
  • 93
    • 84927173806 scopus 로고    scopus 로고
    • Intestinal inflammation and impact on growth in children with cystic fibrosis
    • Dhaliwal, J., et al. Intestinal inflammation and impact on growth in children with cystic fibrosis. J. Pediatr. Gastrtroenterol. Nutr. 60, 521-526 (2015
    • (2015) J. Pediatr. Gastrtroenterol. Nutr , vol.60 , pp. 521-526
    • Dhaliwal, J.1
  • 94
    • 84926688564 scopus 로고    scopus 로고
    • Elevated faecal M2 pyruvate kinase in children with cystic fibrosis: A clue to the increased risk of intestinal malignancy in adulthood?
    • Pang, T., et al. Elevated faecal M2 pyruvate kinase in children with cystic fibrosis: a clue to the increased risk of intestinal malignancy in adulthood? J. Gastroenterol. Hepatol. 30, 866-871 (2015
    • (2015) J. Gastroenterol. Hepatol , vol.30 , pp. 866-871
    • Pang, T.1
  • 95
    • 77956023066 scopus 로고    scopus 로고
    • Small intestine bacterial overgrowth does not correspond to intestinal inflammation in cystic fibrosis
    • Lisowska, A., et al. Small intestine bacterial overgrowth does not correspond to intestinal inflammation in cystic fibrosis. Scand. J. Clin. Lab. Invest. 70, 322-326 (2010
    • (2010) Scand. J. Clin. Lab. Invest , vol.70 , pp. 322-326
    • Lisowska, A.1
  • 96
    • 85027934344 scopus 로고    scopus 로고
    • Early colon screening of adult patients with cystic fibrosis reveals high incidence of adenomatous colon polyps
    • Billings, J. L., et al. Early colon screening of adult patients with cystic fibrosis reveals high incidence of adenomatous colon polyps. J. Clin. Gastroenterol. 48, e85-88 (2014
    • (2014) J. Clin. Gastroenterol , vol.48 , pp. e85-88
    • Billings, J.L.1
  • 97
    • 0034838017 scopus 로고    scopus 로고
    • Proliferation not apoptosis, alters epithelial cell migration in small intestine of CFTR null mice
    • Gallagher, A. M.,&Gottlieb, R. A. Proliferation, not apoptosis, alters epithelial cell migration in small intestine of CFTR null mice. Am. J. Physiol. Gastroenterol. 281, G681-G687 (2001
    • (2001) Am. J. Physiol. Gastroenterol , vol.281 , pp. G681-G687
    • Gallagher, A.M.1    Gottlieb, R.A.2
  • 98
    • 4644265292 scopus 로고    scopus 로고
    • Faecal tumour M2 pyruvate kinase: A new, sensitive screening tool for colorectal cancer
    • Hardt, P. D., et al. Faecal tumour M2 pyruvate kinase: a new, sensitive screening tool for colorectal cancer. Br. J. Cancer 91, 980-984 (2004
    • (2004) Br. J. Cancer , vol.91 , pp. 980-984
    • Hardt, P.D.1
  • 99
    • 0029983188 scopus 로고    scopus 로고
    • Mutant cystic fibrosis transmembrane conductance regulator inhibits acidification and apoptosis in C127 cells: Possible relevance to cystic fibrosis
    • Gottlieb, R. A., Dosanjh, A. Mutant cystic fibrosis transmembrane conductance regulator inhibits acidification and apoptosis in C127 cells: possible relevance to cystic fibrosis. Proc. Natl Acad. Sci. USA 93, 3587-3591 (1996
    • (1996) Proc. Natl Acad. Sci. USA , vol.93 , pp. 3587-3591
    • Gottlieb, R.A.1    Dosanjh, A.2
  • 100
    • 50649102821 scopus 로고    scopus 로고
    • Cystic fibrosis as a bowel cancer syndrome and the potential role of CK2
    • Mehta, A. Cystic fibrosis as a bowel cancer syndrome and the potential role of CK2. Mol. Cell. Biochem. 316, 169-175 (2008
    • (2008) Mol. Cell. Biochem , vol.316 , pp. 169-175
    • Mehta, A.1
  • 101
    • 3042599473 scopus 로고    scopus 로고
    • Inflammation and cancer IV Colorectal cancer in inflammatory bowel disease: The role of inflammation
    • Itzkowitz, S. H.,&Yio, X. Inflammation and cancer IV. Colorectal cancer in inflammatory bowel disease: the role of inflammation. Am. J. Physiol. Gastrointest. Liver Physiol. 287, G7-G17 (2004
    • (2004) Am. J. Physiol. Gastrointest. Liver Physiol , vol.287 , pp. G7-G17
    • Itzkowitz, S.H.1    Yio, X.2
  • 102
    • 84855866793 scopus 로고    scopus 로고
    • Acid and non-Acid reflux during physiotherapy in young children with cystic fibrosis
    • Doumit, M., Krishnan, U., Jaffé, A.,&Belessis, Y. Acid and non-Acid reflux during physiotherapy in young children with cystic fibrosis. Pediatr. Pulmonol. 47, 119-124 (2012
    • (2012) Pediatr. Pulmonol , vol.47 , pp. 119-124
    • Doumit, M.1    Krishnan, U.2    Jaffé, A.3    Belessis, Y.4
  • 103
    • 80052480426 scopus 로고    scopus 로고
    • Gastric emptying and different types of reflux in adult patients with cystic fibrosis
    • Pauwels, A., et al. Gastric emptying and different types of reflux in adult patients with cystic fibrosis. Aliment. Pharmacol. Ther. 34, 799-807 (2011
    • (2011) Aliment. Pharmacol. Ther , vol.34 , pp. 799-807
    • Pauwels, A.1
  • 104
    • 25844512172 scopus 로고    scopus 로고
    • Gastroesophageal reflux (symptomatic and silent): A potentially significant problem in patients with cystic fibrosis before and after lung transplantation
    • Button, B. M., et al. Gastroesophageal reflux (symptomatic and silent): a potentially significant problem in patients with cystic fibrosis before and after lung transplantation. J. Heart Lung Transplant. 24, 1522-1529 (2005
    • (2005) J. Heart Lung Transplant , vol.24 , pp. 1522-1529
    • Button, B.M.1
  • 105
    • 0028140481 scopus 로고
    • Strictures of ascending colon in cystic fibrosis and high strength pancreatic enzymes
    • Smythe, R. L., et al. Strictures of ascending colon in cystic fibrosis and high strength pancreatic enzymes. Lancet 343, 85-86 (1994
    • (1994) Lancet , vol.343 , pp. 85-86
    • Smythe, R.L.1
  • 106
    • 0028885145 scopus 로고
    • Use of pancreatic enzyme supplements for patients with cystic fibrosis in the context of fibrosing colonopathy
    • Borowitz, D. S., Grand, R. J.,&Durie, P. R. Use of pancreatic enzyme supplements for patients with cystic fibrosis in the context of fibrosing colonopathy. J. Pediatr. 127, 681-684 (1995
    • (1995) J. Pediatr , vol.127 , pp. 681-684
    • Borowitz, D.S.1    Grand, R.J.2    Durie, P.R.3
  • 107
    • 0036735017 scopus 로고    scopus 로고
    • Consensus report on nutrition for pediatric patients with cystic fibrosis
    • Borowitz, D., Baker, R. D.,&Stallings, V. Consensus report on nutrition for pediatric patients with cystic fibrosis. J. Pediatr. Gastroenterol. Nutr. 35, 246-259 (2002
    • (2002) J. Pediatr. Gastroenterol. Nutr , vol.35 , pp. 246-259
    • Borowitz, D.1    Baker, R.D.2    Stallings, V.3
  • 108
    • 0028324349 scopus 로고
    • Crohn's disease and cystic fibrosis
    • Lloyd-Still, J. D. Crohn's disease and cystic fibrosis. Dig. Dis. Sci. 39, 880-885 (1994
    • (1994) Dig. Dis. Sci , vol.39 , pp. 880-885
    • Lloyd-Still, J.D.1
  • 109
    • 34249028548 scopus 로고    scopus 로고
    • Potential role for the common cystic fibrosis ?F508 mutation in Crohn's disease
    • Bresso, F., et al. Potential role for the common cystic fibrosis ?F508 mutation in Crohn's disease. Inflamm. Bowel Dis. 13, 531-536 (2007
    • (2007) Inflamm. Bowel Dis , vol.13 , pp. 531-536
    • Bresso, F.1
  • 110
    • 77953914912 scopus 로고    scopus 로고
    • Cystic fibrosis gene mutations and gastrointestinal diseases
    • Bahmanyar, S., et al. Cystic fibrosis gene mutations and gastrointestinal diseases. J. Cyst. Fibros. 9, 288-291 (2010
    • (2010) J. Cyst. Fibros , vol.9 , pp. 288-291
    • Bahmanyar, S.1
  • 111
    • 79952455153 scopus 로고    scopus 로고
    • The cystic fibrosis F508del mutation in Crohn's disease
    • Bresso, F., D'Amato, M. The cystic fibrosis F508del mutation in Crohn's disease. J. Cyst. Fibros. 10, 132 (2011
    • (2011) J. Cyst. Fibros , vol.10 , pp. 132
    • Bresso, F.1    D'Amato, M.2
  • 112
    • 84906952377 scopus 로고    scopus 로고
    • Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D mediated cystic fibrosis
    • Rowe, S. M., et al. Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D mediated cystic fibrosis. Am. J. Respir. Crit. Care Med. 190, 175-184 (2014
    • (2014) Am. J. Respir. Crit. Care Med , vol.190 , pp. 175-184
    • Rowe, S.M.1
  • 113
    • 84884517511 scopus 로고    scopus 로고
    • Ivacaftor in a G551D homozygote with cystic fibrosis
    • Harrison, M. J., Murphy, D. M.,&Plant, B. J. Ivacaftor in a G551D homozygote with cystic fibrosis. N. Engl. J. Med. 369, 1280-1282 (2013
    • (2013) N. Engl. J. Med , vol.369 , pp. 1280-1282
    • Harrison, M.J.1    Murphy, D.M.2    Plant, B.J.3
  • 114
    • 84890532573 scopus 로고    scopus 로고
    • The effect of probiotics on fecal calprotectin in patients with cystic fibrosis
    • Fallahi, G., et al. The effect of probiotics on fecal calprotectin in patients with cystic fibrosis. Turk. J. Pediatr. 55, 475-478 (2013
    • (2013) Turk. J. Pediatr , vol.55 , pp. 475-478
    • Fallahi, G.1
  • 115
    • 84890469591 scopus 로고    scopus 로고
    • Effects of probiotics on quality of life in children with cystic fibrosis; A randomized controlled trial
    • Jafari, S. A., et al. Effects of probiotics on quality of life in children with cystic fibrosis; a randomized controlled trial. Iran. J. Pediatr. 23, 669-674 (2013
    • (2013) Iran. J. Pediatr , vol.23 , pp. 669-674
    • Jafari, S.A.1
  • 116
    • 34248588695 scopus 로고    scopus 로고
    • Effect of Lactobacillus GG supplementation on pulmonary exacerbations in patients with cystic fibrosis: A pilot study
    • Bruzzese, E., et al. Effect of Lactobacillus GG supplementation on pulmonary exacerbations in patients with cystic fibrosis: a pilot study. Clin. Nutr. 26, 322-328 (2007
    • (2007) Clin. Nutr , vol.26 , pp. 322-328
    • Bruzzese, E.1
  • 117
    • 77953320256 scopus 로고    scopus 로고
    • Probiotic supplementation affects pulmonary exacerbations in patients with cystic fibrosis: A pilot study
    • Weiss, B., et al. Probiotic supplementation affects pulmonary exacerbations in patients with cystic fibrosis: a pilot study. Pediatr. Pulmonol. 45, 536-540 (2010
    • (2010) Pediatr. Pulmonol , vol.45 , pp. 536-540
    • Weiss, B.1
  • 118
    • 5144232620 scopus 로고    scopus 로고
    • CFTR Cl-channel function in native human colon correlates with the genotype and phenotype in cystic fibrosis
    • Hirtz, S., et al. CFTR Cl-channel function in native human colon correlates with the genotype and phenotype in cystic fibrosis. Gastroenterology 127, 1085-1095 (2004
    • (2004) Gastroenterology , vol.127 , pp. 1085-1095
    • Hirtz, S.1
  • 119
    • 0025772974 scopus 로고
    • Ion transport abnormalities in rectal suction biopsies from children with cystic fibrosis
    • Veeze, H. J., et al. Ion transport abnormalities in rectal suction biopsies from children with cystic fibrosis. Gastroenterology 101, 398-403 (1991
    • (1991) Gastroenterology , vol.101 , pp. 398-403
    • Veeze, H.J.1
  • 120
    • 11044231602 scopus 로고    scopus 로고
    • Assessment of CFTR function in rectal biopsies for the diagnosis of cystic fibrosis
    • Mall, M., Hirtz, S., Gonska, T.,&Kunzelmann, K. Assessment of CFTR function in rectal biopsies for the diagnosis of cystic fibrosis. J. Cyst. Fibros. 3 (SUPPL 2), 165-169 (2004
    • (2004) J. Cyst. Fibros , vol.3 , pp. 165-169
    • Mall, M.1    Hirtz, S.2    Gonska, T.3    Kunzelmann, K.4
  • 121
    • 84880292828 scopus 로고    scopus 로고
    • A functional CFTR assay using primary cystic fibrosis intestinal organoids
    • Dekkers, J. F., et al. A functional CFTR assay using primary cystic fibrosis intestinal organoids. Nat. Med. 19, 939-945 (2013
    • (2013) Nat. Med , vol.19 , pp. 939-945
    • Dekkers, J.F.1
  • 122
    • 0033953729 scopus 로고    scopus 로고
    • Improved assay for fecal calprotectin
    • T?n, H., et al. Improved assay for fecal calprotectin. Clin. Chim. Acta 292, 41-54 (2000
    • (2000) Clin. Chim. Acta , vol.292 , pp. 41-54
    • Tn, H.1


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.