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首页> 外文期刊>Pediatric blood & cancer >Clinical features of children, adolescents, and adults with coexisting hypermobility syndromes and von Willebrand disease
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Clinical features of children, adolescents, and adults with coexisting hypermobility syndromes and von Willebrand disease

机译:儿童,青少年和成人共存高管综合征和冯维尔布朗病的临床特征

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Abstract We present five patients with coexistent von Willebrand disease (VWD) and Ehlers‐Danlos syndrome and 21 with VWD and joint hypermobility. Females outnumbered males ten to three, Beighton scores were documented in 58% (15 of 26 patients), and several patients experienced moderately severe bleeding. We believe coexistent hypermobility disorder with VWD potentially affects bleeding severity and want to raise awareness among hematologists. Evaluation by geneticists is recommended because of the varying complexities observed across the disease spectrum, and the availability of new genetic tests should lead to more accurate diagnoses for the various hypermobility disorders.
机译:摘要我们提出了五名共存的von Willebrand疾病(VWD)和Ehlers-Danlos综合征,21例,具有VWD和关节高能力。 女性数少于男性十到三,以58%(26名患者中的15个)记录了Beighton评分,并且有几名患者经历了中度严重的出血。 我们认为与VWD共存的高温性障碍可能会影响出血的严重程度,并希望提高血液学师之间的认识。 由于疾病谱中观察到的不同复杂性,建议使用遗传学家的评估,并且新的遗传测试的可用性应该导致各种高能性疾病的更准确的诊断。

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