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首页> 外文期刊>Pediatrics: Official Publication of the American Academy of Pediatrics >Rare bleeding disorders in children: Identification and primary care management
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Rare bleeding disorders in children: Identification and primary care management

机译：儿童稀有出血障碍：识别和初级保健管理

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摘要

Bleeding symptoms are common in healthy children but occasionally may indicate an underlying congenital or acquired bleeding diathesis. The rare bleeding disorders (RBDs) comprise inherited deficiencies of coagulation factors I (congenital fibrinogen deficiencies), II, V, VII, X, XI, and XIII and combined factor deficiencies, most notably of factors V and VIII and of vitamin K-dependent factors. These disorders often manifest during childhood and may present with recurrent or even serious or life-threatening bleeding episodes, particularly during the neonatal period. Accordingly, primary care and other nonhematologist pediatric providers should be familiar with the clinical presentation and initial evaluation of these rare disorders. Bleeding manifestations generally vary within the same RBD and may be indistinguishable from 1 RBD to another or from other more common bleeding disorders. Serious bleeding events such as intracranial hemorrhage may be heralded by less serious bleeding symptoms. The results of initial coagulation studies, especially prothrombin time and activated partial thromboplastin time, are often helpful in narrowing down the potential factor deficiency, with factor XIII deficiency being an exception. Consultation with a hematologist is advised to facilitate accurate diagnosis and to ensure proper management and follow-up. The approach to bleeding episodes and invasive procedures is individualized and depends on the severity, frequency, and, in the case of procedures, likelihood of bleeding. Prophylaxis may be appropriate in children with recurrent serious bleeding and specifically after life-threatening bleeding episodes. When available, specific purified plasma-derived or recombinant factor concentrates, rather than fresh frozen plasma or cryoprecipitate, are the treatment of choice. Pediatrics 2013;132:882- 892.
机译：出血症状在健康的儿童中常见，但偶尔可能表明潜在的先天性或获得的出血尿液。罕见的出血障碍（RBDs）包括凝血因子I（先天性纤维蛋白原缺陷），II，V，VII，X，XI和XII和联合因子缺陷的遗传缺陷，最值得注目的因素V和VIII和维生素K依赖性因素。这些疾病常常在儿童时表现出来，并且可能存在复发或甚至严重或危及严重或生命的出血集中，特别是在新生儿期间。因此，初级保健和其他乳腺癌小儿科提供者应熟悉这些罕见疾病的临床介绍和初始评估。出血表现通常在相同的RBD内变化，并且可能与另一种或更多常见的出血障碍中的1 RBD无法区分。颅内出血等严重的出血事件可能会使不太严重的出血症状预示着。初始凝血研究的结果，尤其是凝血酶原时间和活化的部分血栓形成时间，通常有助于缩小潜在的因子缺乏，因子XIII缺乏是例外。建议与血液学学者咨询，促进准确的诊断，并确保适当的管理和随访。流血剧集和侵入手术的方法是个性化的，取决于严重程度，频率，以及在程序的情况下，出血的可能性。预防在患有复发性严重出血的儿童中可能适合，并且特别是威胁危及生命的出血发作。当可用时，特异性纯化的血浆衍生或重组因子浓缩物，而不是新鲜的冷冻血浆或冷沉淀物，是选择的治疗方法。 2013年儿科; 132：882-892。

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