• Journal of Chest Surgery
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• 제21권4호
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• pp.706-710
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• 1988

Intravascular hemolysis occurs in the majority of patient with mechanical valve prosthesis. The primary cause is mechanical trauma to red cells from turbulent blood flow through the prosthesis. Degree of hemolysis is dependent upon the type, size and material of valve and aggravated by paravalvular leakage. Clinically important hemolytic anemia is required medical management or consideration of reoperation. In severe hemolysis, reoperation is recommended without delay when seems to be renal failure. In this case, postoperative severe mechanical hemolysis was developed immediately after aortic valve replacement with St. Jude medical valve in a 13 year-old male patient. Neither significant paravalvular leakage nor valvular dysfunction was found through redo, but the mechanical valve was strongly suspected the cause of severe hemolysis. The St. Jude Medical valve was changed with Ionescu-Shiley bioprosthesis and any significant clinical problems were not noted through the postoperative course.

• Clinical and Experimental Pediatrics
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• 제59권2호
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• pp.100-103
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• 2016

Patients with hemolytic-uremic syndrome (HUS) can rapidly develop profound anemia as the disease progresses, as a consequence of red blood cell (RBC) hemolysis and inadequate erythropoietin synthesis. Therefore, RBC transfusion should be considered in HUS patients with severe anemia to avoid cardiac or pulmonary complications. Most patients who are Jehovah's Witnesses refuse blood transfusion, even in the face of life-threatening medical conditions due to their religious convictions. These patients require management alternatives to blood transfusions. Erythropoietin is a glycopeptide that enhances endogenous erythropoiesis in the bone marrow. With the availability of recombinant human erythropoietin (rHuEPO), several authors have reported its successful use in patients refusing blood transfusion. However, the optimal dose and duration of treatment with rHuEPO are not established. We report a case of a 2-year-old boy with diarrhea-associated HUS whose family members are Jehovah's Witnesses. He had severe anemia with acute kidney injury. His lowest hemoglobin level was 3.6 g/dL, but his parents refused treatment with packed RBC transfusion due to their religious beliefs. Therefore, we treated him with high-dose rHuEPO (300 IU/kg/day) as well as folic acid, vitamin B12, and intravenous iron. The hemoglobin level increased steadily to 7.4 g/dL after 10 days of treatment and his renal function improved without any complications. To our knowledge, this is the first case of successful rHuEPO treatment in a Jehovah's Witness child with severe anemia due to HUS.

• Childhood Kidney Diseases
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• 제17권2호
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• pp.149-153
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• 2013

응고성 미세혈관병증은 빠른 진단이 예후에 중요한 인자이나, 현재의 진단 기준에 따라서는 thrombotic thrombocytopenic purpura, haemolytic uremic syndrome 외의 비전형적인 응고성 미세혈관병증의 진단이 늦어짐에 따라 나쁜 예후를 초래하게 되는 경우가 많다고 보고되어 있다. 본 저자들은 시행한 혈액 검사상 용혈의 증거가 없는 빈혈, 혈소판 감소증 그리고 급성 신부전을 보인 소아 환아에서 신조직 검사를 통해 비특이적 응고성 미세혈관병증을 진단받은 1증례를 보고하고자 한다. 14세 여자 환아는 3주간 지속된 발열, 구역과 전신 부종을 주소로 본원으로 전원되었다. 내원하여 시행한 혈액 검사상 빈혈과 혈소판 감소증을 보였으나, 용혈의 증거는 없었으며, 혈정 크레아티닌이 증가되어 있었다. 내원 이후 급성 신부전과 발열은 지속적으로 진행되었으며, 소변 검사상 단백뇨가 발생하였다. 환아는 내원 40일경 신고혈압과 동반된 전신 경련이 5분간 있어 뇌 자기 공명 영상을 촬영하였으며, 가역성 후백질 뇌병증 증후군의 양상을 보여 항 경련제 투여를 시작하였다. 이후 지속되는 혈소판 감소증 및 발열은 고용량 스테로이드 치료를 진행한 후 호전되었으나, 급성 신부전 및 단백뇨가 지속되어 신장 조직 검사를 진행하였으며, 검사 결과상 혈전성 미세혈관병증의 소견을 보였다. 이와 같이 조직검사상에서는 응고성 미세혈관병증을 보이나 전형적인 응고성 미세혈관병증의 혈액학적인 진단 기준이 충족되지 않는 비특이적 형태의 응고성 혈관병증의 효과적인 진단을 위하여 보체 기전이나 ADAMTS 13와 같은 유전자 범위의 보다 활발한 연구를 통한 효과적인 진단 기준의 마련이 되어야 할 것으로 보인다.