Pediatric Gastroenterology, Hepatology & Nutrition

The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition (대한소아소화기영양학회)
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Endoscopic Findings of Children with Familial Mediterranean Fever

  • Sag, Elif (Department of Pediatric Gastroenterology, Hepatology and Nutrition, Faculty of Medicine, Karadeniz Technical University) ;
  • Demir, Ferhat (Department of Pediatric Rheumatology, Faculty of Medicine, Karadeniz Technical University) ;
  • Saygin, Ismail (Department of Pathology, Faculty of Medicine, Karadeniz Technical University) ;
  • Kalyoncu, Mukaddes (Department of Pediatric Rheumatology, Faculty of Medicine, Karadeniz Technical University) ;
  • Cakir, Murat (Department of Pediatric Gastroenterology, Hepatology and Nutrition, Faculty of Medicine, Karadeniz Technical University)
  • Received : 2018.02.07
  • Accepted : 2018.04.24
  • Published : 2018.10.15
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Abstract

Purpose: Familial Mediterranean fever (FMF) is an auto inflammatory disease characterized by periodic fever, synovitis and serositis. Patients may be admitted to gastroenterology units due to gastrointestinal symptoms. In this study; we aimed to analyze endoscopic findings and diagnostic utility of endoscopic procedure in children with FMF. Methods: Patient with FMF that was performed endoscopy for the gastrointestinal symptoms were included to the study (39 of 164 patients, 53 procedure). A control group was randomly designed as age and gender matched four endoscopic procedures per one endoscopic procedure of patients with FMF (n=212). Results: No different was found between the patients and control group in esophagogastroscopy findings. However, the diagnosis of gastrointestinal pathology was made by esophagogastroscopy in 46.2% patients. Colonoscopic examination revealed that the frequency of inflammatory bowel disease (IBD) was higher in undiagnosed patients compared to both the control group (50.0% vs. 6.9%, p<0.05, odds ratio [OR]:13.4 and 95% confidence inteval [95% CI]: 2.1-84.3) and the patients under colchicine treatment (50.0% vs. 8.3%, p<0.05, OR: 11 and 95% CI: 0.8-147.8). Colonoscopic procedure that was made after the diagnosis was found to provide contribution by 16.7% in determining the etiology of the additional symptoms. Conclusion: Patients with FMF may be admitted to pediatric gastroenterology outpatient clinic prior to diagnosis or during the follow-up period. The frequency of IBD is high in undiagnosed patients with FMF. Endoscopic procedures may be helpful in these patients for the diagnosis accompanying mucosal lesions.

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References

  1. Wang DQH, Bonfrate L, de Bari O, Wang TY, Portincasa P. Familial Mediterranean fever: from pathogenesis to treatment. J Genet Syndr Gene Ther 2014;5:1-11.
  2. Kitade T, Horiki N, Katsurahara M, Totoki T, Harada T, Tano S, et al. Usefulness of small intestinal endoscopy in a case of adult-onset familial Mediterranean fever associated with Jejunoileitis. Intern Med 2015;54:1343-7. https://doi.org/10.2169/internalmedicine.54.3690
  3. Koshy R, Sivadas A, Scaria V. Genetic epidemiology of familial Mediterranean fever through integrative analysis of whole genome and exome sequences from Middle East and North Africa. Clin Genet 2018;93:92-102. https://doi.org/10.1111/cge.13070
  4. Demir A, Akyuz F, Gokturk S, Evirgen S, Akyuz U, Ormeci A, et al. Small bowel mucosal damage in familial Mediterranean fever: results of capsule endoscopy screening. Scand J Gastroenterol 2014;49:1414-8.
  5. Mor A, Gal R, Livneh A. Abdominal and digestive system associations of familial Mediterranean fever. Am J Gastroenterol 2003;98:2594-604. https://doi.org/10.1111/j.1572-0241.2003.08784.x
  6. Isomoto H, Kamo Y, Chen CC, Nakao K. Clinical management of gastrointestinal amyloidosis. Open J Gastroenterol 2012;2:155-62. https://doi.org/10.4236/ojgas.2012.24030
  7. Hart J, Lewin KJ, Peters RS, Schwabe AD. Effect of long-term colchicine therapy on jejunal mucosa. Dig Dis Sci 1993;38:2017-21. https://doi.org/10.1007/BF01297078
  8. Pras M. Familial Mediterranean fever: from the clinical syndrome to the cloning of the pyrin gene. Scand J Rheumatol 1998;27:92-7.
  9. Tutar E, Kutluk G, Bayrak NA, Celikel CA, Pehlivanoglu E, Ertem D. What is the diagnostic utility of endoscopic scoring systems in children? Turk J Gastroenterol 2013;24:22-9.
  10. Iacono G, Ravelli A, Di Prima L, Scalici C, Bolognini S, Chiappa S, et al. Colonic lymphoid nodular hyperplasia in children: relationship to food hypersensitivity. Clin Gastroenterol Hepatol 2007;5:361-6. https://doi.org/10.1016/j.cgh.2006.12.010
  11. Levine A, Koletzko S, Turner D, Escher JC, Cucchiara S, de Ridder L, et al; European Society of Pediatric Gastroenterology, Hepatology, and Nutrition. ESPGHAN revised porto criteria for the diagnosis of inflammatory bowel disease in children and adolescents. J Pediatr Gastroenterol Nutr 2014;58:795-806.
  12. Gurkan OE, Dalgic B. Gastrointestinal mucosal involvement without amyloidosis in children with familial Mediterranean fever. J Pediatr Gastroenterol Nutr 2013;57:319-23. https://doi.org/10.1097/MPG.0b013e318295fc65
  13. Gurkan OE, Yilmaz G, Aksu AU, Demirtas Z, Akyol G, Dalgic B. Colonic lymphoid nodular hyperplasia in childhood: causes of familial Mediterranean fever need extra attention. J Pediatr Gastroenterol Nutr 2013;57: 817-21. https://doi.org/10.1097/MPG.0b013e3182a9083b
  14. Beşer OF, Kasapcopur O, Cokugras FC, Kutlu T, Arsoy N, Erkan T. Association of inflammatory bowel disease with familial Mediterranean fever in Turkish children. J Pediatr Gastroenterol Nutr 2013;56:498-502. https://doi.org/10.1097/MPG.0b013e31827dd763
  15. Ozcakar ZB, Cakar N, Uncu N, Celikel BA, Yalcinkaya F. Familial Mediterranean fever-associated diseases in children. QJM 2017;110:287-90.
  16. Cakir M, Unal F, Dinler G, Baran M, Yuksekkaya HA, Tumgor G, et al. Inflammatory bowel disease in Turkish children. World J Pediatr 2015;11:331-7. https://doi.org/10.1007/s12519-015-0042-2
  17. Salah S, El-Shabrawi M, Lotfy HM, Shiba HF, Abou-Zekri M, Farag Y. Detection of Mediterranean fever gene mutations in Egyptian children with inflammatory bowel disease. Int J Rheum Dis 2016;19: 806-13. https://doi.org/10.1111/1756-185X.12482
  18. Borekci E, Celikbilek M, Soyturk M, Akar S, Borekci H, Gunaydin I. Functional gastrointestinal disorders in patients with familial Mediterranean fever. Int J Rheum Dis 2017;20:2101-5. https://doi.org/10.1111/1756-185X.12207

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