Idiopathic recurrent pancreatitis in a young woman: unexpected clue
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idiopathic recurrent pancreatitis, abdominal pain, cystic fibrosis, diagnosis, enzyme replacement therapy

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Gubergrits, N. B., Monogarova, N. Y., Byelyayeva, N. V., Zeynalova, A. A., Fomenko, P. G., Shpak, S. A., Bakhchevan, E. L., & Burka, A. A. (2020). Idiopathic recurrent pancreatitis in a young woman: unexpected clue. Herald of Pancreatic Club, 48(3), 54-69.

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The article presents a complex clinical case of idiopathic recurrent pancreatitis in a young woman, 23 years old. The main clinical manifestation of the disease was acute, intense, recurrent abdominal pain. Despite ongoing symptomatic therapy, the patient’s condition had been worsened, and the intensity of abdominal pain increased. Adequate dosage of the enzyme replacement therapy allowed increasing the duration of remission. Differential diagnosis, additional laboratory and instrumental studies allowed determining the cause of recurrent pancreatitis and confirming the diagnosis of cystic fibrosis with a primary lesion of the pancreas. A distinctive feature of this clinical case is the diagnosis of a hereditary cystic fibrosis disease in adulthood, the absence of clinical symptoms of lungs’ involvement in the pathological process, as well as the absence of underdevelopment, malnutrition. The diagnosis of cystic fibrosis is confirmed by German specialists; currently, the patient continues treatment in a German clinic.

The article presents current data on idiopathic pancreatitis, discusses a diagnostic algorithm for identification of the cause of the disease. The etiology, pathogenetic mechanisms of cystic fibrosis occurrence are considered, the known types of CFTR gene mutations, causing the development of the disease, as well as the principles of replacement enzyme therapy in patients with cystic fibrosis are described. Evidence-based medicine data are presented, revealing the efficacy and safety of taking enzyme preparations in the correction of the exocrine pancreatic insufficiency.
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1. РОО МО «Помощь больным муковисцидозом». Электронный ресурс. Режим доступа:
2. Ammann R. W. Alcohol- and non-alcohol induced pancreatitis: clinical aspects. NY: McGraw-Hill, 1992. P. 253–272.
3. Ardengh J. C., Malheiros C. A., Rahal F., Pereira V., Ganc A. J. Microlithiasis of the gallbladder: role of endoscopic ultrasonography in patients with idiopathic acute pancreatitis. Rev. Assoc. Med. Bras. 2010. Vol. 56, No 1. P. 27–31.
4. Arif S., Moore F., Marks K., Bouckenooghe T., Dayan C. M., et al. Peripheral and islet interleukin-17 pathway activation characterizes human autoimmune diabetes and promotes cytokine-mediated beta-cell death. Diabetes. 2011. Vol. 60. P. 2112–2119.
5. Augarten A., Ben Tov A., Madgar I., Barak A., Akons H., 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. 2008. Vol. 20. Р. 164–168.
6. Barrio R. Management of endocrine disease: Cystic fibrosis-related diabetes: novel pathogenic insights opening new therapeutic avenues. Eur. J. Endocrinol. 2015. Vol. 172. P. R131–R141.
7. Blanco G., Gesuale С., Varanese М., Monteleone G., Paoluzi O. Idiopathic acute pancreatitis: a review on etiology and diagnostic work-up. Clin. J. Gastroenterol. 2019. Электронный документ. Режим доступа:
8. Borowitz D., Stevens C., Brettman L. R., Campion M., Wilschanski M., Thompson H.; Liprotamase 767 Study Group. Liprotamase long-term safety and support of nutritional status in pancreatic-insufficient cystic fibrosis. J. Pediatr. Gastroenterol. Nutr. 2012. Vol. 54. Р. 248–257.
9. Byrne M. F., Lee J. K., Enns R. Review of idiopathic pancreatitis. World J. Gastroenterol. 2007. Vol. 13, No 47. P. 6296–6313.
10. Carrier Testing for Cystic Fibrosis. Cystic Fibrosis Foundation. 2019. Электронный документ. Режим доступа:
11. Castellani C., Duff А., Bell S., Heijerman H., Munck A., et al. ECFS best practice guidelines: the 2018 revision. J. Cyst. Fibros. 2018. Vol. 17, No 2. Р. 153–178.
12. Chamnan P., Shine B. S., Haworth C. S., Bilton D., Adler A. I. Diabetes as a determinant of mortality in cystic fibrosis. Diabetes Care. 2010. Vol. 33, No 2. Р. 311–316.
13. Coyle W. J., Pineau B. C., Tarnasky P., Knapple W. L., Aabakken L., Hoffman B. J., Cunningham J. T., Hawes R. H., Cotton P. B. Evaluation of unexplained acute and acute recurrent pancreatitis using endoscopic retrograde cholangiopancreatography, sphincter of Oddi manometry and endoscopic ultrasound. Endoscopy. 2002. Vol. 34, No 8. P. 617–623.
14. Cystic Fibrosis Foundation. CFTR structure and regulation. 2019. Электронный документ. Режим доступа:
15. Cystic Fibrosis Foundation. PATIENT REGISTRY HIGHLIGHTS. 2019. Электронный документ. Режим доступа:
16. DiMagno M. J., Lee S. H., Hao Y., Zhou S. Y., McKenna B. J., Owyang C. A proinflammatory, antiapoptotic phenotype underlies the susceptibility to acute pancreatitis in cystic fibrosis transmembrane regulator (-/-) mice. Gastroenterology. 2005. Vol. 129. Р. 665–681.
17. DiMagno M. J., Lee S. H., Owyang C., Zhou S. Y. Inhibition of acinar apoptosis occurs during acute pancreatitis in the human homologue DeltaF508 cystic fibrosis mouse. Am. J. Physiol. Gastrointest. Liver Physiol. 2010. Vol. 299. P. G400–G412.
18. Etemad B., Whitcomb D. C. Chronic pancreatitis: diagnosis, classification, and new genetic developments. Gastroenterology. 2011. Vol. 120. P. 682–707.
19. Galli F., Battistoni A., Gambari R., Pompella A., Bragonzi A., Pilolli F., Iuliano L., Piroddi M., Dechecchi M. C., Cabrini G. Oxidative stress and antioxidant therapy in cystic fibrosis. Biochim. Biophys. Acta. 2012. Vol. 1822, No 5. Р. 690–713.
20. Gibson-Corley K., Meyerholz D., Engelhardt J. Pancreatic Pathophysiology in Cystic Fibrosis. J. Pathol. 2016. Vol. 238, No 2. Р. 311–320.
21. Graff G.R., Maguiness K., McNamara J., Morton R., Boyd D., Beckmann K., Bennett D. 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. Clin. Ther. 2010. Vol. 32, No 1. Р. 89–103.
22. Guo J. H., Chen H., Ruan Y. C., Zhang X. L., Zhang X. H., Fok K. L., Tsang L. L., Yu M. K., Huang W. Q., Sun X., Chung Y. W., Jiang X., Sohma Y., Chan H. C. Glucose-induced electrical activities and insulin secretion in pancreatic islet beta-cells are modulated by CFTR. Nat. Commun. 2014. Vol. 15, No 5. Р. 4420.
23. Hayakawa T., Kondo T., Shibata T., Sugimoto Y., Kitagawa M. Chronic alcoholism and evolution of pain in chronic pancreatitis. Dig. Dis. Sci. 1989. Vol. 34, No 1. P. 33–38.
24. Hegyi P., Rakonczay Z. Jr. The role of pancreatic ducts in the pathogenesis of acute pancreatitis. Pancreatology. 2015. Vol. 15. P. S13–S17.
25. Hewison M. Vitamin D and immune function: an overview. Proc. Nutrit. Soc. 2012. Vol. 71. Р. 50–61.
26. Kaw M., Brodmerkel G. ERCP, biliary crystal analysis and sphincter of Oddi manometry in idiopathic recurrent pancreatitis. Gastrointest. Endosc. 2002. Vol.27. 55. P. 157–162.
27. Keller J., Layer P. Idiopathic chronic pancreatitis. Best Pract. Res. Clin. Gastroenterol. 2008. Vol. 22, No 1. P. 105–113.
28. Ko S. B., Azuma S., Yoshikawa T., Yamamoto A., Kyokane K., Ko M. S., Ishiguro H. Molecular mechanisms of pancreatic stone formation in chronic pancreatitis. Front. Physiol. 2012. Vol. 3. Р. 415.
29. Kopelman H., Corey M., Gaskin K., Durie P., Weizman Z., Forstner G. Impaired chloride secretion, as well as bicarbonate secretion, underlies the fluid secretory defect in the cystic fibrosis pancreas. Gastroenterology. 1988. Vol. 95, No 2. P. 349–355.
30. Kopelman H., Durie P., Gaskin K., Weizman Z., Forstner G. Pancreatic fluid secretion and protein hyperconcentration in cystic fibrosis. New Engl. J. Med. 1985. Vol. 312, No 6. Р. 329–334.
31. Lanng S., Thorsteinsson B., Erichsen G., Nerup J., Koch C. Glucose tolerance in cystic fibrosis. Arch. Dis. Child. 1991. Vol. 66, No 5. Р. 612–616.
32. Lavie M., Fisher D., Vilozni D., Forschmidt R., Sarouk I., Kanety H., Hemi R., Efrati O., Modan-Moses D. Glucose intolerance in cystic fibrosis as a determinant of pulmonary function and clinical status. Diabetes Res. Clin. Pract. 2015. Vol. 110, No 3. Р. 276–284.
33. Layer P., Kashirskaya N., Gubergrits N. Contribution of pancreatic enzyme replacement therapy to survival and quality of life in patients with pancreatic exocrine insufficiency. World J. Gastroenterol. 2019. Vol. 25, No 20. Р. 2430–2441.
34. Layer P., Yamamoto H., Kalthoff L., Clain J. E., Bakken L. J., DiMagno E. P. The different courses of early- and late-onset idiopathic and alcoholic chronic pancreatitis. Gastroenterology. 1994. Vol. 107, No 5. P. 1481–1487.
35. Linsdell P. Functional architecture of the CFTR chloride channel. Mol. Membr. Biol. 2014. Vol. 31. Р. 1–16.
36. Madacsy T., Pallagi P., Maleth J. Cystic fibrosis of the pancreas: the role of CFTR channel in the regulation of intracellular ca2c signaling and mitochondrial function in the exocrine pancreas. Front. Physiol. 2018. Vol.9. P. 1585.
37. Mansour G. Cystic fibrosis. Hospital Medicine. 2019. Электронный документ. Режим доступа:
38. Meyerholz D. K., Stoltz D. A., Pezzulo A. A., Welsh M. J. Pathology of gastrointestinal organs in a porcine model of cystic fibrosis. Amer. J. Pathol. 2010. Vol. 176, No 3. Р. 1377–1389.
39. Milla C. E., Warwick W. J., Moran A. Trends in pulmonary function in patients with cystic fibrosis correlate with the degree of glucose intolerance at baseline. Am. J. Respir. Crit. Care Med. 2000. Vol. 162, No 3, Pt. 1. Р. 891–895.
40. Moran A., Dunitz J., Nathan B., Saeed A., Holme B., Thomas W. Cystic fibrosis-related diabetes: current trends in prevalence, incidence, and mortality. Diabetes Care. 2009. Vol. 32, No 9. Р. 1626–1631.
41. Moran A., Hardin D., Rodman D., Allen H. F., Beall R. J., et al. Diagnosis, screening and management of cystic fibrosis related diabetes mellitus: a consensus conference report. Diabetes Res. Clin. Pract. 1999. Vol. 45, No 1. Р. 61–73.
42. Nathan B. M., Laguna T., Moran A. Recent trends in cystic fibrosis-related diabetes. Curr. Opin. Endocrinol. Diabetes Obes. 2010. Vol. 17, No 4. Р. 335–341.
43. Ode K. L., Moran A. New insights into cystic fibrosis-related diabetes in children. Lancet Diabetes Endocrinol. 2013. Vol. 1, No 1. Р. 52–58.
44. Olivier A. K., Gibson-Corley K. N., Meyerholz D. K. Animal models of gastrointestinal and liver diseases. Animal models of cystic fibrosis: gastrointestinal, pancreatic, and hepatobiliary disease and pathophysiology. Am. J. Physiol. Gastrointest. Liv. Physiol. 2015. Vol. 308. P. G459–G471.
45. Pedersen N.T., Andersen B.N., Pedersen G., Worning H. Chronic pancreatitis in Copenhagen: a retrospective study of 64 consecutive patients. Scand. J. Gastroenterol. 1982. Vol. 17. P. 925–931.
46. Pranke I., Golec A., Hinzpeter A., Edelman A., Sermet-Gaudelus I. Emerging therapeutic approaches for cystic fibrosis. From gene editing to personalized medicine. Front. Pharmacol. 2019. Vol. 10. Р. 121.
47. Quon B. S., Rowe S. M. New and emerging targeted therapies for cystic fibrosis. BMJ. 2016. Vol. 352. P. i859.
48. Robles-Diaz G., Vargas F., Uscanga L., Castilo C. F. Chronic pancreatitis in Mexico City. Pancreas. 1989. Vol. 5. P. 479–483.
49. Somaraju U.R., Solis-Moya A. Pancreatic enzyme replacement therapy for people with cystic fibrosis. Cochrane Database Syst. Rev. 2016. Vol. 11. CD008227.
50. Stecenko A. A., Moran A. Update on cystic fibrosis-related diabetes. Curr. Opin. Pulm. Med. 2010. Vol. 16. Р. 611–615.
51. Stern R. C., Eisenberg J. D., Wagener J. S., Ahrens R., Rock M., doPico G., Orenstein D. M. A comparison of the efficacy and tolerance of pancrelipase and placebo in the treatment of steatorrhea in cystic fibrosis patients with clinical exocrine pancreatic insufficiency. Am. J. Gastroenterol. 2000. Vol. 95. Р. 1932–1938.
52. Thomson H. J. Acute pancreatitis in north and north-east Scotland. J. R. Coll. Surg. Edinb. 1985. Vol. 30. P. 104–111.
53. Trapnell B. C., Maguiness K., Graff G. R., Boyd D., Beckmann K., Caras S. Efficacy and safety of Creon 24,000 in subjects with exocrine pancreatic insufficiency due to cystic fibrosis. J. Cyst. Fibros. 2009. Vol. 8. Р. 370–377.
54. Trapnell B. C., Strausbaugh S. D., Woo M. S., Tong S. Y., Silber S. A., Mulberg A. E., Leitz G. Efficacy and safety of PANCREAZE® for treatment of exocrine pancreatic insufficiency due to cystic fibrosis. J. Cyst. Fibros. 2011. Vol. 10. Р. 350–356.
55. Tucker J. A., Spock A., Spicer S. S., Shelburne J. D., Bradford W. Inspissation of pancreatic zymogen material in cystic fibrosis. Ultrastruct. Pathol. 2003. Vol. 27, No 5. Р. 323–335.
56. Veit G., Avramescu R. G., Chiang A. N., Houck S. A., Cai Z., et al. From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations. Mol. Biol. Cell. 2016. Vol. 27, No 3. Р. 424–433.
57. Wilschanski M., Novak I. The cystic fibrosis of exocrine pancreas. Cold Spring Harb. Perspect. Med. 2013. Vol. 3, No 5. P. a009746.
58. Yaochite J.N., Caliari-Oliveira C., Davanso M. R., Carlos D., Malmegrim K. C., Cardoso C. R., Ramalho L. N., Palma P. V., da Silva J. S., Cunha F. Q., Covas D. T., Voltarelli J. C. Dynamic changes of the Th17/Tc17 and regulatory T cell populations interfere in the experimental autoimmune diabetes pathogenesis. Immunobiology. 2013. Vol. 218, No 3. Р. 338–352.