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Disorders in Hemostasis

  • Sung, Tae-Jung (Department of Pediatrics, College of Medicine, Hallym University)
  • Published : 2011.05.31

Abstract

Neonatal bleeding is a common problem encountered in nursery rooms or neonatal intensive care units, especially among premature infants. Furthermore, owing to recent remarkable improvement of neonatology, survival rates of preterm neonates have increased; hence, neonatal bleeding cannot be emphasized enough. Since the total blood volume of neonates is small, bleeding can be one of the causes of morbidities and mortalities. Therefore, rapid diagnosis and immediate therapy is urgently needed. The patient's medical history including a familial history of a bleeding disorder or of a previously affected infant who suffered from bleeding along with maternal and neonatal drugs can provide important diagnostic clues. Presence of bleeding with or without petechiae and ecchymoses in a healthy term or late preterm infant with thrombocytopenia but normal prothrombin time and activated partial thromboplastin time strongly suggests a congenital bleeding disorder. For a sick infant who is bleeding from multiple sites, an acquired disorder such as disseminated intravascular coagulation is suspected. Intracranial hemorrhage in term or late preterm infants without a history of birth trauma is highly suggestive of coagulation disorders. The purpose of this review is to summarize recent advances in diagnostic methods is as well as basic concepts of neonatal hemostatic disorders. First, an outline of background information will be presented followed by a discussion of primary and secondary hemostatic disorders as well as inherited and acquired disorders.

신생아 출혈은 신생아실에서 흔하게 경험하는 증상 중 하나이며 신생아중환자실에서는 특히 미숙아에게 종종 발생하므로 신속한 진단 및 즉각적인 치료가 필수적이다. 신생아 출혈은 이환율과 사망률의 중요한 원인이 되며 심한 경우 생명을 위협할 수 있다. 특히 최근 들어 비약적인 신생아학의 발달로 인해 초극소저체중출생아를 포함한 미숙아들의 생존율이 높아지고 있어 혈액응고질환의 진단 및 치료의 중요성은 날로 높아져 간다고 하겠다. 출혈이 의심되는 신생아의 정확한 진단을 위해서는 출혈의 가족력, 산모의 병력, 과거 임신력, 신생아 및 산모의 약물복용여부를 포함한 자세한 병력청취가 무엇보다 주의 깊게 시행되어야 하고 특정부위에 국한되어 증상을 보이는지 아니면 광범위한 출혈인지 감별을 해야 한다. 혈소판감소증만 단독으로 보이는 신생아의 경우 자반증(petechiae)과 반상출혈(ecchymoses), 점막출혈 등이 동반될 수 있으나 대부분은 건강해 보이며 비타민 K 결핍출혈, 혈우병 같은 선천성 응고장애를 의심해 볼 수 있으며, 아파 보이는 신생아에서 폐, 위장관, 비뇨생식기계, 천자부위 등에서 출혈이 일어나는 경우는 파종성 혈관내응고증후군을 포함한 후천성 응고이상을 의심해 볼 수 있다. 특별히 외상의 흔적이나 난산의 병력이 없는 만삭아나 준미숙아 등에서 두개 내 출혈이 보이는 경우에도 반드시 혈액응고이상 질환을 의심해봐야 한다. 저자는 본 종설을 통해 신생아출혈을 유발하는 혈액응고질환에 대해 임상유형 및 발생기전에 따라 대해 1차성 및 2차성 응고이상질환으로 나누어 알아보고 이를 다시 선천성 응고장애질환과 후천성 응고장애질환으로 나눠 각각에 대해 최신지견을 토대로 자세히 살펴보고자 한다.

Keywords

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