Journal of Istanbul Faculty of Medicine

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Case Report


DOI :https://doi.org/10.18017/iuitfd.279885   IUP :https://doi.org/10.18017/iuitfd.279885    Full Text (PDF)

AİLEVİ AKDENİZ ATEŞİ ATAĞI ESNASINDA GİLBERT SENDROMLU HASTADA CİDDİ HİPERBİLİRUBİNEMİ GELİŞİMİ: OLGU SUNUMU

Orkide Kutlu, Muharrem Keskin, Abdülkadir Baştürk, Şamil Ecirli, Yılmaz Bilgiç, Sevil Fişekçi Oktar

Ailevi Akdeniz ateşi - Familial Mediterranean Fever (FMF), ataklar şeklinde ateş ve eşlik eden seröz zarların inflamasyonu ile karakterli otozomal resesif geçişli bir bozukluk olup herediter periyodik ateş sendromlarının en sık rastlananıdır. FMF atağında IL-6, TNF-α, IL-1β gibi sitokinlerin aktif rol oynadığı bilinmektedir. Gilbert sendromu, genetik polimorfizm sonucu UDP-glukronil transferaz aktivitesinde baskılanma sebebi ile ateşli hastalık, cerrahi girişim, kusma ve uzamış açlık dönemlerinde indirekt bilirubinemi ile sonuçlanan bir klinik durumdur. FMF patogenezinde rol alan sitokinlerin UDP-glukronil transferaz aktivitesini baskılaması nedeni ile atak esnasında bilirubin düzeylerinde artış olabileceği düşünülmüştür. Burada FMF atağı sırasında ciddi indirekt bilirubin yüksekliği gözlemlediğimiz bir vaka sunuldu.

Keywords: Familyal Mediterranean fever, indirekt bilirubinemi, Gilbert Sendromu
DOI :https://doi.org/10.18017/iuitfd.279885   IUP :https://doi.org/10.18017/iuitfd.279885    Full Text (PDF)

SEVERE HYPERBILIRUBINEMIA DURING FAMILYAL MEDITERRANEAN FEVER ATTACK IN PATIENTS WITH GILBERT'S SYNDROME

Orkide Kutlu, Muharrem Keskin, Abdülkadir Baştürk, Şamil Ecirli, Yılmaz Bilgiç, Sevil Fişekçi Oktar

Familial Mediterranean fever (FMF) is an autosomal recessive disorder characterized by fever and accompanying attacks of serositis. Cytokines such as IL- 6, TNF-α, IL-1β are known to play an active role in FMF pathogenesis. Gilbert's syndrome, is a clinical condition that results indirect hyperbilirubinemia during febrile illness, surgery, vomiting and prolonged fasting period because of inhibition of UDP-glucuronide transferase activity. Increased bilirubin levels during FMF attack is caused suppression of UDP-glucuronide transferase activity by cytokines involved in the pathogenesis of FMF. Here, we report a patient who had serious indirect bilirubin elevation during an attack of FMF.

Keywords: Familial Mediterranean fever, indirect bilirubinemia, Gilbert’s Syndrome

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APA

Kutlu, O., Keskin, M., Baştürk, A., Ecirli, Ş., Bilgiç, Y., & Fişekçi Oktar, S. (2016). AİLEVİ AKDENİZ ATEŞİ ATAĞI ESNASINDA GİLBERT SENDROMLU HASTADA CİDDİ HİPERBİLİRUBİNEMİ GELİŞİMİ: OLGU SUNUMU. Journal of Istanbul Faculty of Medicine, 79(3), 130-132. https://doi.org/https://doi.org/10.18017/iuitfd.279885


AMA

Kutlu O, Keskin M, Baştürk A, Ecirli Ş, Bilgiç Y, Fişekçi Oktar S. AİLEVİ AKDENİZ ATEŞİ ATAĞI ESNASINDA GİLBERT SENDROMLU HASTADA CİDDİ HİPERBİLİRUBİNEMİ GELİŞİMİ: OLGU SUNUMU. Journal of Istanbul Faculty of Medicine. 2016;79(3):130-132. https://doi.org/https://doi.org/10.18017/iuitfd.279885


ABNT

Kutlu, O.; Keskin, M.; Baştürk, A.; Ecirli, Ş.; Bilgiç, Y.; Fişekçi Oktar, S. AİLEVİ AKDENİZ ATEŞİ ATAĞI ESNASINDA GİLBERT SENDROMLU HASTADA CİDDİ HİPERBİLİRUBİNEMİ GELİŞİMİ: OLGU SUNUMU. Journal of Istanbul Faculty of Medicine, [Publisher Location], v. 79, n. 3, p. 130-132, 2016.


Chicago: Author-Date Style

Kutlu, Orkide, and Muharrem Keskin and Abdülkadir Baştürk and Şamil Ecirli and Yılmaz Bilgiç and Sevil Fişekçi Oktar. 2016. “AİLEVİ AKDENİZ ATEŞİ ATAĞI ESNASINDA GİLBERT SENDROMLU HASTADA CİDDİ HİPERBİLİRUBİNEMİ GELİŞİMİ: OLGU SUNUMU.” Journal of Istanbul Faculty of Medicine 79, no. 3: 130-132. https://doi.org/https://doi.org/10.18017/iuitfd.279885


Chicago: Humanities Style

Kutlu, Orkide, and Muharrem Keskin and Abdülkadir Baştürk and Şamil Ecirli and Yılmaz Bilgiç and Sevil Fişekçi Oktar. “AİLEVİ AKDENİZ ATEŞİ ATAĞI ESNASINDA GİLBERT SENDROMLU HASTADA CİDDİ HİPERBİLİRUBİNEMİ GELİŞİMİ: OLGU SUNUMU.” Journal of Istanbul Faculty of Medicine 79, no. 3 (Aug. 2025): 130-132. https://doi.org/https://doi.org/10.18017/iuitfd.279885


Harvard: Australian Style

Kutlu, O & Keskin, M & Baştürk, A & Ecirli, Ş & Bilgiç, Y & Fişekçi Oktar, S 2016, 'AİLEVİ AKDENİZ ATEŞİ ATAĞI ESNASINDA GİLBERT SENDROMLU HASTADA CİDDİ HİPERBİLİRUBİNEMİ GELİŞİMİ: OLGU SUNUMU', Journal of Istanbul Faculty of Medicine, vol. 79, no. 3, pp. 130-132, viewed 14 Aug. 2025, https://doi.org/https://doi.org/10.18017/iuitfd.279885


Harvard: Author-Date Style

Kutlu, O. and Keskin, M. and Baştürk, A. and Ecirli, Ş. and Bilgiç, Y. and Fişekçi Oktar, S. (2016) ‘AİLEVİ AKDENİZ ATEŞİ ATAĞI ESNASINDA GİLBERT SENDROMLU HASTADA CİDDİ HİPERBİLİRUBİNEMİ GELİŞİMİ: OLGU SUNUMU’, Journal of Istanbul Faculty of Medicine, 79(3), pp. 130-132. https://doi.org/https://doi.org/10.18017/iuitfd.279885 (14 Aug. 2025).


MLA

Kutlu, Orkide, and Muharrem Keskin and Abdülkadir Baştürk and Şamil Ecirli and Yılmaz Bilgiç and Sevil Fişekçi Oktar. “AİLEVİ AKDENİZ ATEŞİ ATAĞI ESNASINDA GİLBERT SENDROMLU HASTADA CİDDİ HİPERBİLİRUBİNEMİ GELİŞİMİ: OLGU SUNUMU.” Journal of Istanbul Faculty of Medicine, vol. 79, no. 3, 2016, pp. 130-132. [Database Container], https://doi.org/https://doi.org/10.18017/iuitfd.279885


Vancouver

Kutlu O, Keskin M, Baştürk A, Ecirli Ş, Bilgiç Y, Fişekçi Oktar S. AİLEVİ AKDENİZ ATEŞİ ATAĞI ESNASINDA GİLBERT SENDROMLU HASTADA CİDDİ HİPERBİLİRUBİNEMİ GELİŞİMİ: OLGU SUNUMU. Journal of Istanbul Faculty of Medicine [Internet]. 14 Aug. 2025 [cited 14 Aug. 2025];79(3):130-132. Available from: https://doi.org/https://doi.org/10.18017/iuitfd.279885 doi: https://doi.org/10.18017/iuitfd.279885


ISNAD

Kutlu, Orkide - Keskin, Muharrem - Baştürk, Abdülkadir - Ecirli, Şamil - Bilgiç, Yılmaz - Fişekçi Oktar, Sevil. “AİLEVİ AKDENİZ ATEŞİ ATAĞI ESNASINDA GİLBERT SENDROMLU HASTADA CİDDİ HİPERBİLİRUBİNEMİ GELİŞİMİ: OLGU SUNUMU”. Journal of Istanbul Faculty of Medicine 79/3 (Aug. 2025): 130-132. https://doi.org/https://doi.org/10.18017/iuitfd.279885



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