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Deterioration in Clinical Status Is Not Enough to Suspend Eculizumab: A Genetic Complement-Mediated Atypical Hemolytic Uremic Syndrome Case Report
المؤلفون المشاركون
Calvaruso, Luca
Naticchia, Alessandro
Vischini, Gisella
Costanzi, Stefano
Ferraro, Pietro Manuel
المصدر
العدد
المجلد 2019، العدد 2019 (31 ديسمبر/كانون الأول 2019)، ص ص. 1-5، 5ص.
الناشر
Hindawi Publishing Corporation
تاريخ النشر
2019-07-09
دولة النشر
مصر
عدد الصفحات
5
التخصصات الرئيسية
الملخص EN
Background.
Atypical hemolytic uremic syndrome (aHUS) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal failure.
Mutations in CFI gene coding for complement regulation factors and in THBD gene coding for endothelial cell receptor thrombomodulin could predispose to the disease and hypertension can trigger the onset.
Case Presentation.
A 51-year-old female patient who had received kidney transplant eighteen years ago presented with hypertensive peak and hemolysis pattern.
Normal ADAMTS13 levels as well as negative culture and serology for Shiga-toxin excluded, respectively, thrombotic thrombocytopenic purpura (TTP) and typical HUS caused by Shiga toxin-producing Escherichia coli (STEC-HUS).
In suspicion of aHUS, we administered eculizumab and hemodialysis sessions were started as the patient showed severe renal failure.
After an initial response, the patient developed cerebral hemorrhage.
After last eculizumab administration, according to hematological parameters, an unsatisfactory response was observed: given the worsening clinical scenario, we withdrew eculizumab.
Pathogenic mutations in CFI and THBD genes were found.
After eculizumab reinitiation, looking at hemolysis indexes, we observed a suboptimal response as well as an otherwise adequate renal one: renal graft function was recovered despite persistence of hemolysis signs, after 6 months on regular dialysis.
Conclusion.
For the first time, we report an aHUS case in which a peculiar combination of mutations in CFI and THBD is found.
We describe the importance of continuing eculizumab despite deterioration of patient’s clinical conditions.
نمط استشهاد جمعية علماء النفس الأمريكية (APA)
Calvaruso, Luca& Naticchia, Alessandro& Ferraro, Pietro Manuel& Vischini, Gisella& Costanzi, Stefano. 2019. Deterioration in Clinical Status Is Not Enough to Suspend Eculizumab: A Genetic Complement-Mediated Atypical Hemolytic Uremic Syndrome Case Report. Case Reports in Nephrology،Vol. 2019, no. 2019, pp.1-5.
https://search.emarefa.net/detail/BIM-1141251
نمط استشهاد الجمعية الأمريكية للغات الحديثة (MLA)
Calvaruso, Luca…[et al.]. Deterioration in Clinical Status Is Not Enough to Suspend Eculizumab: A Genetic Complement-Mediated Atypical Hemolytic Uremic Syndrome Case Report. Case Reports in Nephrology No. 2019 (2019), pp.1-5.
https://search.emarefa.net/detail/BIM-1141251
نمط استشهاد الجمعية الطبية الأمريكية (AMA)
Calvaruso, Luca& Naticchia, Alessandro& Ferraro, Pietro Manuel& Vischini, Gisella& Costanzi, Stefano. Deterioration in Clinical Status Is Not Enough to Suspend Eculizumab: A Genetic Complement-Mediated Atypical Hemolytic Uremic Syndrome Case Report. Case Reports in Nephrology. 2019. Vol. 2019, no. 2019, pp.1-5.
https://search.emarefa.net/detail/BIM-1141251
نوع البيانات
مقالات
لغة النص
الإنجليزية
الملاحظات
Includes bibliographical references
رقم السجل
BIM-1141251
قاعدة معامل التأثير والاستشهادات المرجعية العربي "ارسيف Arcif"
أضخم قاعدة بيانات عربية للاستشهادات المرجعية للمجلات العلمية المحكمة الصادرة في العالم العربي
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