References:
[1]. Elborn J. Cystic fibrosis.
The Lsancet. 2016;388(10059):2519-2531. doi:10.1016/s0140-6736(16)00576-6.
[2]. Gibson-Corley K, Meyerholz
D, Engelhardt J. Pancreatic pathophysiology in cystic fibrosis. The Journal of Pathology.
2015;238(2):311-320. doi:10.1002/path.4634.
[3]. Witt H. Chronic pancreatitis
and cystic fibrosis. Gut. 2003;52(90002):31ii-41. doi: 10.1136/gut.52.suppl_2. ii31.
[4]. W. Park R, J. Grand R. Gastrointestinal
Manifestations of Cystic Fibrosis: A Review. American Gastroenterological Association.
1881;81(6):1143-1161.
[5]. Struyvenberg M, Martin C,
Freedman S. Practical guide to exocrine pancreatic insufficiency – Breaking the
myths. BMC Medicine. 2017;15(1). doi:10.1186/s12916-017-0783-y.
[6]. Haack A. Pathophysiology
of cystic fibrosis and drugs used in associated digestive tract diseases. World
Journal of Gastroenterology. 2013;19(46):8552. doi:10.3748/wjg. v19.i46.8552.
[7]. Davies JC, Alton EW, Bush
A. Cystic fibrosis. BMJ. 2007;335(7632):1255–1259. doi:10.1136/bmj.39391.713229.
[8]. Sheridan MB, Hefferon TW,
Wang N, et al. CFTR transcription defects in pancreatic sufficient cystic fibrosis
patients with only one mutation in the coding region of CFTR. J Med Genet. 2011;48(4):235–241.
doi:10.1136/jmg.2010.083287.
[9]. Soe K, Gregoire-Bottex MM.
A rare CFTR mutation associated with severe disease progression in a 10-year-old
Hispanic patient. Clin Case Rep. 2017;5(2):139–144. doi:10.1002/ccr3.764.
[10]. Farrell PM, Rosenstein BJ,
White TB, et al. Guidelines for diagnosis of cystic fibrosis in newborns through
older adults: Cystic Fibrosis Foundation consensus report. J Pediatr. 2008;153(2):
S4–S14. doi: 10.1016/j.jpeds.2008.05.005.
[11]. Weiss FU, Simon P, Bogdanova
N, et al. Complete cystic fibrosis transmembrane conductance regulator gene sequencing
in patients with idiopathic chronic pancreatitis and controls. Gut. 2005;54(10):1456–1460.
doi:10.1136/gut.2005.064808.
[12]. Navarro S. Historical compilation
of cystic fibrosis. Gastroenterología y Hepatología (English Edition). 2016;39(1):36-42.
doi: 10.1016/j.gastre.2015.12.006.
[13]. FA Ratjen. Cystic fibrosis:
pathogenesis and future treatment strategies. Respir Care. 2009; 54(5): 595-605.
[14]. Derichs N. Targeting a genetic
defect: cystic fibrosis transmembrane conductance regulator modulators in cystic
fibrosis. European Respiratory Review 2013; 22:58-65. doi:10.1183/09059180.00008412.
[15]. J. M, Y. C. Pancreatitis
in Cystic Fibrosis and CFTR-Related Disorder. Acute Pancreatitis. 2012; doi:10.5772/27861.
[16]. Sheppard M, Nicholson A.
The pathology of cystic fibrosis. Current Diagnostic Pathology. 2002;8(1):50-59.
doi: 10.1054/cdip.2001.0088.
[17]. Wilschanski M, Novak I. The
Cystic Fibrosis of Exocrine Pancreas. Cold Spring Harbor Perspectives in Medicine.
2013;3(5): a009746-a009746. doi:10.1101/cshperspect. a009746.
[18]. Soave D, Miller M, Keenan
K, Li W, Gong J, Ip W et al. Evidence for a Causal Relationship Between Early Exocrine
Pancreatic Disease and Cystic Fibrosis–Related Diabetes: A Mendelian Randomization
Study. Diabetes. 2014;63(6):2114-2119. doi:10.2337/db13-1464.
[19]. Madácsy T, Pallagi P, Maleth
J. Cystic Fibrosis of the Pancreas: The Role of CFTR Channel in the Regulation of
Intracellular Ca2+ Signaling and Mitochondrial Function in the Exocrine Pancreas.
Frontiers in Physiology. 2018;9. doi:10.3389/fphys.2018.01585.
[20]. Baker S, Borowitz D, Baker
R. Pancreatic exocrine function in patients with cystic fibrosis. Current Gastroenterology
Reports. 2005;7(3):227-233. doi:10.1007/s11894-005-0039-4.
[21]. Gelfond D, Borowitz D. Gastrointestinal
Complications of Cystic Fibrosis. Clinical Gastroenterology and Hepatology. 2013;11(4):333-342.
doi: 10.1016/j.cgh.2012.11.006.
[22]. Aris RM et al. Guide to bone
health and disease in cystic fibrosis. J Clin Endocrinol Metab. 2005;90(3):1888-96.
doi: 10.1210/jc.2004-1629.
[23]. Blackman, S. M., & Tangpricha,
V. Endocrine Disorders in Cystic Fibrosis. Pediatric clinics of North America.2016;63(4):699–708.
doi: 10.1016/j.pcl.2016.04.009.
[24]. Adler, A. I., Shine, B.,
Haworth, C., Leelarathna, L., & Bilton, D. Hyperglycemia and death in cystic
fibrosis-related diabetes. Diabetes care;2011;34(7):1577–1578. doi:10.2337/dc10-2289.
[25]. Kelly, Andrea et al. Update
on cystic fibrosis-related diabetes. Journal of Cystic Fibrosis.2013;12(4):318 –
331. doi: 10.1016/j.jcf.2013.02.008.
[26]. Kelsey, R., Manderson Koivula,
F. N., McClenaghan, N. H., & Kelly, C. Cystic Fibrosis-Related Diabetes: Pathophysiology
and Therapeutic Challenges. Clinical medicine insights. Endocrinology and diabetes.
2019;12: 1179551419851770. doi:10.1177/1179551419851770.
[27]. Hart, N. J., Aramandla, R.,
Poffenberger, G., Fayolle, C., Thames, A. H., Bautista, A., Powers, A. C. Cystic
fibrosis-related diabetes is caused by islet loss and inflammation. JCI insight.2018;3(8):
e98240. doi: 10.1172/jci.insight.98240.
[28]. Nyirjesy, S. C., Sheikh,
S., Hadjiliadis, D., De Leon, D. D., Peleckis, A. J., Eiel, J. N., … Kelly, A. β-Cell
secretory defects are present in pancreatic insufficient cystic fibrosis with 1-hour
oral glucose tolerance test glucose ≥155 mg/dL. Pediatric diabetes.2018;19(7):1173–1182.
doi:10.1111/pedi.12700.
[29]. Meacham, L. R., Caplan, D.
B., McKean, L. P., Buchanan, C. N., Parks, J. S., & Culler, F. L. Preservation
of somatostatin secretion in cystic fibrosis patients with diabetes. Archives of
disease in childhood.1993;68(1):123–125. doi:10.1136/adc.68.1.123.
[30]. Cory, M., Moin, A., Moran,
A., Rizza, R. A., Butler, P. C., Dhawan, S., & Butler, A. E. An Increase in
Chromogranin A-Positive, Hormone-Negative Endocrine Cells in Pancreas in Cystic
Fibrosis. Journal of the Endocrine Society.2018;2(9):1058–1066. doi:10.1210/js.2018-00143.
[31]. Joseph P. Neglia, M.D., M.P.H.,
Stacey C. FitzSimmons, Ph.D., Patrick Maisonneuve.et,al. The Risk of Cancer among
Patients with Cystic Fibrosis. N Engl J Med 1995;332: 494-499. doi:10.1056/NEJM199502233320803.
[32]. Amy Berrington de González
1, Kwang Pyo Kim 2, and Jonathan M. Samet. Radiation-induced Cancer Risk from Annual
Computed Tomography for Patients with Cystic Fibrosis. American Journal of Respiratory
and Critical Care Medicine. 2007;176(10): 970-973. doi:10.1164/rccm.200704-591OC.
[33]. Cazacu, I. M., Farkas, N.,
Garami, A., Balaskó, M., Mosdósi, B., Alizadeh, H., … Hegyi, P. Pancreatitis-Associated
Genes and Pancreatic Cancer Risk: A Systematic Review and Meta-analysis. Pancreas.
2018;47(9):1078–1086. doi:10.1097/MPA.0000000000001145.
[34]. Sheldon, C. D., Hodson, M.
E., Carpenter, L. M., & Swerdlow, A. J. A cohort study of cystic fibrosis and
malignancy. British journal of cancer. 1993;68(5):1025–1028. doi:10.1038/bjc.1993.474.
[35]. McWilliams, R. R., Petersen,
G. M., Rabe, K. G., Holtegaard, L. M., Lynch, P. J., Bishop, M. D., & Highsmith,
W. E., Jr. Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations
and risk for pancreatic adenocarcinoma. Cancer.2010;116(1):203–209. doi:10.1002/cncr.24697.
[36]. Engjom T, Kavaliauskiene
G, Tjora E, Erchinger F, Wathle G, Lærum BN, et al. Sonographic pancreas echogenicity
in cystic fibrosis compared to exocrine pancreatic function and pancreas fat content
at Dixon-MRI. PLoS ONE. 2018;13(7): e0201019. doi: 10.1371/journal.pone.0201019.
[37]. Toouli J, Biankin A, Oliver
M, Pearce C, Wilson J, Wray N. Management of pancreatic exocrine insufficiency:
Australasian Pancreatic Club recommendations. 2010;193(8):461-467. doi:10.5694/j.1326-5377.
2010.tb04000.x.
[38]. Imrie CW et al. Review article:
enzyme supplementation in cystic fibrosis, chronic pancreatitis, pancreatic and
periampullary cancer. Aliment Pharmacol Ther. 2010;32(1):1-25. doi:10.1111/j.1365-2036.2010.04437.
x.
[39]. Graff, G.R., McNamara, J.,
Royall, J.A., Caras, S.D., & Forssmann, K. 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.
Clinical Drug Investigation. 2010;30(6):351-364. doi:10.2165/11533390-000000000-00000.
[40]. Yankaskas J, Marshall B,
Sufian B, Simon R, Rodman D. Cystic Fibrosis Adult Care. CHEST journal. 2004;125(1):1S–39S.
doi: 10.1378/chest.125.1_suppl.1S.
[41]. Li A, Vigers T, Pyle L, Zemanick
E, Nadeau K, Sagel S et al. Continuous glucose monitoring in youth with cystic fibrosis
treated with lumacaftor-ivacaftor. Journal of Cystic Fibrosis. 2019;18(1):144–149.
doi: 10.1016/j.jcf.2018.07.010.
[42]. Taylor-Cousar JL, Mall MA,
Ramsey BW, et al. Clinical development of triple-combination CFTR modulators for
cystic fibrosis patients with one or two F508del alleles. ERJ Open Res. 2019;5(2):00082-2019.
doi:10.1183/23120541.00082-2019.
[43]. Graff G, Maguiness K, McNamara
J, Morton R, Boyd D, Beckmann K et al. 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 crossover, superiority study. 2010; 32(1):89–103.
doi: 10.1016/j.clinthera.2010.01.012.
[44]. 26. Borowitz D, Goss C, Stevens
C, Hayes D, Newman L, O'Rourke A et al. Safety and Preliminary Clinical Activity
of a Novel Pancreatic Enzyme Preparation in Pancreatic Insufficient Cystic Fibrosis
Patients. Pancreas. 2006;32(3):258-263. doi: 10.1097/01.mpa.0000202952.10612.21.
[45]. Haack A, Aragão GG, Novaes
MR. Pathophysiology of cystic fibrosis and drugs used in associated digestive tract
diseases. World J Gastroenterol. 2013;19(46):8552–8561. doi:10.3748/wjg. v19.i46.8552.