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首页> 外文期刊>Hong Kong Journal of Paediatrics >The Clinical Features of Chinese Children with von Willebrand Disease: The Experience of a Tertiary Institute
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The Clinical Features of Chinese Children with von Willebrand Disease: The Experience of a Tertiary Institute

机译：中国von Willebrand病患儿的临床特征：高等院校的经验

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The information related to the clinical spectrum of von Willebrand disease (VWD) in Chinese patients remains very limited. We conducted a retrospective chart review on the clinical and haematological features of VWD among Chinese patients at a tertiary paediatric centre in Hong Kong. Ten patients (6 females, 4 males) were diagnosed to have VWD from 1989 to 2005. They underwent treatment in our unit, with a cumulative follow up of 102 patient-years within this 16-year period. Among them, 4 were type 1, 5 were type 2 and 1 was type 3 VWD. Six of the 10 patients had a positive family history of bleeding tendencies. A variety of bleeding manifestations were observed in these patients while mucocutaneous bleeds in the form of frequent epistaxis and easy bruising were the commonest presenting features. Severe bleeding in the form of intracranial haemorrhage occurred in 2 patients. Eight patients underwent desmopressin (DDAVP) test at diagnosis and all were responsive to DDAVP without associated thrombocytopenia. Three patients required frequent DDAVP (intravenous or subcutaneous) and 2 required occasional intermediate purity factor VIII concentrate for bleeding control. In conclusion, majority of Chinese paediatric VWD patients are inherited and acquired form is extremely rare in childhood. Patients with either type 1 or 2 VWD can develop severe bleeding in childhood. In our patient cohort, DDAVP appears to be effective and safe for our patients with either type 1 VWD or non-2B type 2 VWD without inducing thrombocytopenia.
机译：有关中国患者von Willebrand病（VWD）临床谱的信息仍然非常有限。我们对香港一家三级儿科中心的中国患者中VWD的临床和血液学特征进行了回顾性图表审查。从1989年至2005年，有10例患者（6例女性，4例男性）被诊断为VWD。他们在本病房接受了治疗，在这16年中累计随访102年。其中，4型为1型，5型为2型，1型为3型VWD。 10例患者中有6例具有出血倾向的阳性家族史。在这些患者中观察到了多种出血表现，而以频繁鼻frequent和容易瘀伤为形式的粘膜皮肤出血是最常见的表现。 2例患者发生颅内大出血。八名患者在诊断时接受了去氨加压素（DDAVP）测试，所有患者均对DDAVP有反应，而没有相关的血小板减少症。 3例患者需要频繁的DDAVP（静脉内或皮下）治疗，而2例患者需要偶尔的中等纯度VIII因子浓缩以控制出血。总之，大多数中国小儿VWD患者是遗传性的，获得性形式在儿童时期极为罕见。患有1型或2型VWD的患者可能在儿童时期出现严重的出血。在我们的患者队列中，DDAVP对于我们的1型VWD或非2B 2型VWD的患者似乎是有效且安全的，并且不会诱发血小板减少症。

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