Eylül ŞAHİN, Fatma DEVRİM, Büşra KOÇ, Aslı KANTAR, Gülhan ATAKUL, Pınar SEVEN, Hasan AĞIN, Nida DİNÇEL

Myokardit ve STEC Pozitifliği ile Seyreden Atipik HÜS Tanılı Olgu

Hemolitik üremik sendrom (HÜS), intravasküler hemoliz, trombositopeni ve akut böbrek yetmezliği ile karakterize trombotik bir mikroanjiyopatidir. Hastalık genellikle shiga-toksin üreten E. coli (STEC) enfeksiyonu ile birliktelik gösteren tipik HÜS ve kontrolsüz kompleman aktivasyonu ile ortaya çıkan atipik HÜS (aHÜS) olarak sınıflandırılmaktadır. Ancak son zamanlarda yapılan bazı çalışmalara göre diyarenin her iki HUS tipinde de görüldüğü , aHÜS olgularında da STEC pozitifliği ile karşılaşıldığı ve her iki HÜS tipinde de multisistemik tutulumun olduğu görülmüştür. Bazı postmortem incelemelerde miyokardit ile uyumlu histopatolojik bulgular saptanmıştır. Bu raporda kanlı ishal ile başvuran, Shiga toksin 2 pozitifliği göstermesine rağmen atipik HÜS olarak değerlendirilen ve nadiren görülen miyokard tutulumu gözlenen bir olgu sunulmuştur.

Anahtar Kelimeler:

HÜS, Atipik HÜS, STEC, MYOKARDİT

An Atypical Hemolytic Uremic Syndrome Case with STEC Positivity and Myocardial Involvement

Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy characterized by intravascular hemolysis, thrombocytopenia and acute renal failure. HUS is generally classified into two types. While typical HUS follows a gastrointestinal infection with shiga-toxin producing E. coli (STEC), aHUS is associated with autoantibodies or mutations that lead to irregular complement activation. However, according to some recent studies, it was observed that diarrhea and multisystem involvement were seen in both HUS types. STEC positivity was also detected in a little percent of aHUS cases. Histopathological findings compatible with myocarditis were found in postmortem examinations in some cases. Although our case was diagnosed with atypical HUS, typical HUS symptoms such as bloody diarrhea and Shiga toxin 2 positivity were reported and myocardial involvement was observed as a rare complication.

Keywords:

HUS, Atypical HUS, STEC, MYOCARDİTİS,

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