임신중독증 환자에서 생긴 비전형적인 용혈성요독증후군 1예
A CASE OF ATYPICAL HEMOLYTIC UREMIC SYNDROME IN A PATIENT WITH PREECLAMPSIA
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초록
성인에서 발생하는 비전형적인 용혈성요독증후군은 미세혈관성 용혈성 빈혈, 혈소판감소증, 신장의 미세혈류 혈소판 혈전에 의해 발생하는 급성 신부전증 등의 특징적인 임상증상이 대장균에 의한 독소와 무관하게 나타나는 위험한 질환이다. 원인은 자간증, 태반박리, 양수합병증과 같은 임신 합병증을 포함한 다양한 스트레스이며, 치료는 헤파린과 혈장교환으로 이로 인해 최근 생존율이 급격히 향상되었다. 저자들은 최근 임신 34주에 핍뇨와 혈뇨, 용혈성 빈혈, 혈소판감소증을 보인 임신중독증 임신부에서 태아심박동 저하로 응급 제왕절개수술후 수혈과 알부민 및 헤파린 치료 후 신기능을 회복한 비전형적인 용혈성요독증후군을 경험하였기에 간단한 문헌 고찰과 함께 보고하는바이다.
Abstract
Atypical hemolytic uremic syndrome (HUS) in adults is a life-threatening disorder characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure, which is not related to Escherichia coli . This disease occurs under the condition of certain stresses, including pregnancy related disease like as preeclampsia, placental abruption, and amniotic fl uid embolism. Heparin and plasma exchange has proven to be an effective treatment for HUS and it has decreased the mortality of this illness. We experienced a case of 34 weeks’ pregnant woman with preeclampsia who presented with hematuria, oliguria, hemolytic anemia and thrombocytopenia. We performed emergency cesarean section due to fetal distress. After operation, she was treated with transfusion of packed red blood cell, parenteral albumin, and anticoagulant. After seven days from the operation, the sign of hematuria disappeared and her renal function partially recovered. We describe this case with a brief review of the literature.
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