Myokardit ve STEC Pozitifliği ile Seyreden Atipik HÜS Tanılı Olgu

Abstract

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.

Keywords

• HÜS
• Atipik HÜS
• STEC
• MYOKARDİT

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

Abstract

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

References

1. 1. Yürük Yıldırım ZN, Yılmaz A. (2014) Atipik Hemolitik Üremik Sendrom, Çocuk Dergisi 14(3):108-115
2. 2.Davin JC, Majoie C, Groothoff J, Gracchi V, Bouts A, Goodship TH, Loirat C. (2011) Prevention of large-vessel stenoses in atypical hemolytic uremic syndrome associated with complement dysregulation. Pediatr Nephrol. 26:155–157.
3. 3. Neuhaus TJ, Calonder S, Leumann EP. Heterogeneity of atypical hemolytic uremic syndromes. (1997) Arch Dis Child; 76(6): 518-21.
4. 4. Hosler GA, Cusumano AM, Hutchins GM. (2003) Thrombotic Thrombocytopenic Purpura and Hemolytic Uremic Syndrome Are Distinct Pathologic Entities. A Review of 56 Autopsy Cases, Arch Pathol Lab Med; Jul;127(7):834-9.
5. 5. Eckart P, Guillot M, Jokic M, Maragnes P, Boudailliez B, Palcoux JB, Desvignes V. (1999) Cardiac Involvement During Classic Hemolytic Uremic Syndrome, Arch Pediatr, Apr;6(4):430-3.
6. 6. Calvaruso L, Naticchia A, Ferraro PM,Vischini G, Costanzi S. (2019) Deterioration in Clinical Status Is Not Enough to Suspend Eculizumab: A Genetic Complement-Mediated Atypical Hemolytic Uremic Syndrome Case Report. Case Rep Nephrol. doi:10.1155/2019/9264824.
7. 7. Costin M, Cinteza E, Balgradean M. (2019) Hemolytic Uremic Syndrome – Case report. Maedica (Buchar). Sep;14(3):298-300.
8. 8. Mohammed SK, Mubarik A, Nadeem B, Khan K, Muddassir S. (2019) Atypical Hemolytic Uremic Syndrome:A Case Report.Cureus. May 10;11(5):e4634.

9. 9. Basnayake BMDB, Wazil AWM, Nanayakkara N, Samarakoon SMDK, Senavirathne EMSK, Thangarajah BUEWDR, Karunasena N, Mahanama RMBSS. (2020) Atypical hemolytic uremic syndrome: a case report. J Med Case Rep. Jan 13;14(1):11.
10. 10. Campbell CM, Cassol C, Cataland SR, Kahwash R. (2020) Atypical hemolytic uremic syndrome: a case report of a rare cause of reversible cardiomyopathy. Eur Heart J Case Rep. Mar 12;4(2):1-6.