Year 2019, Volume 3, Issue 1, Pages 91 - 93 2019-01-27

Kistik fibrozlu bir çocukta beklenmedik cerrahi acil: Atipik sunumlu akut apandisit
The unexpected surgical emergency in a child with cystic fibrosis: An acute appendicitis with unusual presentation

Emrah Doğan [1] , Alev Süzen [2] , Marwa Mouline Doğan [3] , Serkan Yaşar Çelik [4] , Neşat Çullu [5]

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Kistik fibrozis ekzokrin glandların yapısal ve metabolik genetik disfonksiyonu ile karakterize otozomal resesif bir hastalıktır. Kistik fibrozisli hastalarda gastrointestinal semptomlar içerisinde ağrı en yaygın semptomdur ve genellikle distal ileal obstrüksiyon sendromuna bağlıdır. Kistik fibrozisli hastalarda apendiks yoğun bir eozinofilik sekresyondan dolayı sıklıkla distandüdür ancak ironik olarak akut apandisit insidansı genel popülasyondan daha düşüktür. Akut apandisit, kistik fibrozisli hastalarda sıklıkla atipik semptomlarla kendini gösterir. Bu yazıda atipik radyolojik bulguları olan kistik fibrozisli bir akut apandisit olgusu sunuldu ve laboratuvar bulguları, tanı ile uyumsuzdu. Hastada sadece defans ve rebound gibi klasik muayene bulguları mevcuttu ve bu temelde hasta tanı aldı. Sonuç olarak, geç tanıya bağlı ciddi komplikasyonları önlemek için, akut apandisitli kistik fibrozis hastalarının atipik klinik ve radyolojik bulgularla başvurabileceği akılda tutulmalıdır.

Cystic fibrosis is an autosomal recessive disease characterized by structural and metabolic genetic dysfunction of the exocrine glands. Abdominal pain is the most common symptom in patients with cystic fibrosis within gastrointestinal symptoms. It is more commonly related to distal ileal obstruction syndrome. In patients with cystic fibrosis, the appendix is frequently distended due to an intense eosinophilic secretion but ironically, the incidence of acute appendicitis has been reported under the general population. Acute appendicitis in patients with cystic fibrosis commonly present with atypical symptoms. We present the case of cystic fibrosis with an acute appendicitis which had atypical radiologic and laboratory findings that were not compatible with the diagnosis. The diagnosis was made on the basis of clinical examination. As a result, it is of great importance to know that acute appendicitis in patients with cystic fibrosis can present with unusual clinical and radiological findings in order to avoid delayed diagnosis and related serious complications.

  • 1. Timuragaoglu L, Sen V, Gurkan MF. Evaluation of the Clinical and Laboratory Characteristics of Children with Cystic Fibrosis. Archives of Pediatrics. 2016;1(1):21-6.
  • 2. Lavelle LP, McEvoy SH, Mhurchu EN, Gibney RG, McMahon CJ, Heffernan EJ, et al. Cystic Fibrosis below the Diaphragm: Abdominal Findings in Adult Patients. RadioGraphics. 2015;35:680-95.
  • 3. Shields MD, Levison H, Reisman JJ, Durie PR, Canny GJ. Appendicitis in cystic fibrosis. Archives of Disease in Childhood. 1990;65:307-10.
  • 4. Davis PB. Therapy for cystic fibrosis: the end of the beginning? N Engl J Med. 2011;365(18):1734-35.
  • 5. Menten R, Lebecque P, Saint-Martin C, Clapuyt P. Outer diameter of the vermiform appendix: not a valid sonographic criterion for acute appendicitis in patients with cystic fibrosis. AJR Am J Roentgenol. 2005;184(6):1901-03.
  • 6. McCarthy VP, Mischler EH, Hubbard VS, Chernick MS, Di Sant'Agnese PA. Appendiceal abscess in cystic fibrosis: a diagnostic challenge. Gastroenterology. 1984;86:564-8.
  • 7. Kilinc MO, Ninis VN, Dağli E, Demirkol M, Ozkinay F, Arikan Z, et al. Highest heterogeneity for cystic fibrosis: 36 mutations account for 75% of all CF chromosomes in Turkish patients. Am J Med Genet. 2002;113(3):250-7
  • 8. Coughlin JP, Gauderer MW, Stern RC, Doershuk CF, Izant RJ Jr, Zollinger RM Jr. The spectrum of appendiceal disease in cystic fibrosis. J Pediatr Surg. 1990;25(8):835–9.
  • 9. Lardenoye SW, Puylaert JB, Smit MJ, Holscher HC. Appendix in children with cystic fibrosis: US features. Radiology. 2004;232(1):187-89.
Primary Language en
Subjects Medicine
Journal Section Case report
Authors

Orcid: 0000-0002-9446-2294
Author: Emrah Doğan (Primary Author)
Institution: Muğla Sıtkı Koçman Üniversitesi Eğitim ve Araştırma Hastanesi
Country: Turkey


Orcid: 0000-0002-0595-3308
Author: Alev Süzen
Institution: Muğla Sıtkı Koçman Üniversitesi Eğitim ve Araştırma Hastanesi
Country: Turkey


Orcid: 0000-0002-3401-895X
Author: Marwa Mouline Doğan
Institution: Universite de Mohammed V
Country: Morocco


Orcid: 0000-0001-6557-1451
Author: Serkan Yaşar Çelik
Institution: Muğla Sıtkı Koçman Üniversitesi, Faculty of Medicine
Country: Turkey


Orcid: 0000-0002-5045-3919
Author: Neşat Çullu
Institution: Muğla Sıtkı Koçman Üniversitesi, Faculty of Medicine
Country: Turkey


Dates

Publication Date: January 27, 2019

Bibtex @case report { josam470193, journal = {Journal of Surgery and Medicine}, issn = {}, eissn = {2602-2079}, address = {Fatih BAŞAK}, year = {2019}, volume = {3}, pages = {91 - 93}, doi = {10.28982/josam.470193}, title = {The unexpected surgical emergency in a child with cystic fibrosis: An acute appendicitis with unusual presentation}, key = {cite}, author = {Doğan, Emrah and Süzen, Alev and Mouline Doğan, Marwa and Çelik, Serkan Yaşar and Çullu, Neşat} }
APA Doğan, E , Süzen, A , Mouline Doğan, M , Çelik, S , Çullu, N . (2019). The unexpected surgical emergency in a child with cystic fibrosis: An acute appendicitis with unusual presentation. Journal of Surgery and Medicine, 3 (1), 91-93. DOI: 10.28982/josam.470193
MLA Doğan, E , Süzen, A , Mouline Doğan, M , Çelik, S , Çullu, N . "The unexpected surgical emergency in a child with cystic fibrosis: An acute appendicitis with unusual presentation". Journal of Surgery and Medicine 3 (2019): 91-93 <http://dergipark.org.tr/josam/issue/42845/470193>
Chicago Doğan, E , Süzen, A , Mouline Doğan, M , Çelik, S , Çullu, N . "The unexpected surgical emergency in a child with cystic fibrosis: An acute appendicitis with unusual presentation". Journal of Surgery and Medicine 3 (2019): 91-93
RIS TY - JOUR T1 - The unexpected surgical emergency in a child with cystic fibrosis: An acute appendicitis with unusual presentation AU - Emrah Doğan , Alev Süzen , Marwa Mouline Doğan , Serkan Yaşar Çelik , Neşat Çullu Y1 - 2019 PY - 2019 N1 - doi: 10.28982/josam.470193 DO - 10.28982/josam.470193 T2 - Journal of Surgery and Medicine JF - Journal JO - JOR SP - 91 EP - 93 VL - 3 IS - 1 SN - -2602-2079 M3 - doi: 10.28982/josam.470193 UR - https://doi.org/10.28982/josam.470193 Y2 - 2018 ER -
EndNote %0 Journal of Surgery and Medicine The unexpected surgical emergency in a child with cystic fibrosis: An acute appendicitis with unusual presentation %A Emrah Doğan , Alev Süzen , Marwa Mouline Doğan , Serkan Yaşar Çelik , Neşat Çullu %T The unexpected surgical emergency in a child with cystic fibrosis: An acute appendicitis with unusual presentation %D 2019 %J Journal of Surgery and Medicine %P -2602-2079 %V 3 %N 1 %R doi: 10.28982/josam.470193 %U 10.28982/josam.470193
ISNAD Doğan, Emrah , Süzen, Alev , Mouline Doğan, Marwa , Çelik, Serkan Yaşar , Çullu, Neşat . "The unexpected surgical emergency in a child with cystic fibrosis: An acute appendicitis with unusual presentation". Journal of Surgery and Medicine 3 / 1 (January 2019): 91-93. https://doi.org/10.28982/josam.470193
AMA Doğan E , Süzen A , Mouline Doğan M , Çelik S , Çullu N . The unexpected surgical emergency in a child with cystic fibrosis: An acute appendicitis with unusual presentation. J Surg Med. 2019; 3(1): 91-93.
Vancouver Doğan E , Süzen A , Mouline Doğan M , Çelik S , Çullu N . The unexpected surgical emergency in a child with cystic fibrosis: An acute appendicitis with unusual presentation. Journal of Surgery and Medicine. 2019; 3(1): 93-91.