妊娠期血栓性血小板减少性紫癜。

IF 2.2 4区 医学 Q2 NURSING
Courtney Sundin, Kaley Rhodes
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引用次数: 0

摘要

摘要:血栓性血小板减少性紫癜(TTP)是一种罕见而严重的疾病,由ADAMTS-13酶缺乏引起,可导致凝血异常和潜在的器官损害。由于凝血的生理变化,妊娠可触发TTP,当ADAMTS-13水平严重缺乏时,TTP的风险显著增加。ADAMTS-13酶可切割vWF多聚体,但在TTP中,其缺乏导致血小板异常聚集和微血管血栓形成,引起器官损伤、溶血性贫血和血小板减少症。这导致血流受损和显著的终末器官灌注问题。TTP的症状从无症状到严重不等,通常与其他与妊娠相关的并发症类似,如HELLP综合征和先兆子痫,这使得诊断具有挑战性。临床症状包括血小板减少、溶血性贫血、神经功能缺损、发热和肾功能不全,反复发生胎儿丢失和宫内生长受限也很常见。诊断依赖于临床评估和实验室结果,同时等待ADAMTS-13活性结果。有效的沟通和及时的多学科合作对TTP的诊断和治疗至关重要。护士在评估、协作、患者和家庭沟通中发挥着至关重要的作用,以解决妊娠期间的复杂并发症。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Thrombotic Thrombocytopenic Purpura in Pregnancy.

Abstract: Thrombotic thrombocytopenic purpura (TTP) is a rare and serious condition caused by a deficiency in the enzyme ADAMTS-13, leading to abnormal clotting and potential organ damage. Pregnancy can trigger TTP due to physiological changes in coagulation, significantly increasing the risk when ADAMTS-13 levels are severely deficient. The enzyme ADAMTS-13 cleaves vWF multimers, but in TTP, its deficiency leads to abnormal platelet aggregation and microvascular thrombi formation, causing organ damage, hemolytic anemia, and thrombocytopenia. This results in impaired blood flow and significant end-organ perfusion issues. TTP symptoms range from asymptomatic to severe, often mimicking other pregnancy-related complications like HELLP syndrome and preeclampsia, making diagnosis challenging. Clinical signs include thrombocytopenia, hemolytic anemia, neurological deficits, fever, and renal dysfunction, with recurrent fetal loss and intrauterine growth restriction also common. Diagnosis relies on clinical evaluation and laboratory findings, while awaiting ADAMTS-13 activity results. Effective communication and prompt multidisciplinary collaboration are crucial in diagnosing and treating TTP. Nurses play a vital role in assessment, collaboration, and patient and family communication to address complex complications during pregnancy.

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来源期刊
CiteScore
2.60
自引率
16.70%
发文量
158
审稿时长
>12 weeks
期刊介绍: MCN''s mission is to provide the most timely, relevant information to nurses practicing in perinatal, neonatal, midwifery, and pediatric specialties. MCN is a peer-reviewed journal that meets its mission by publishing clinically relevant practice and research manuscripts aimed at assisting nurses toward evidence-based practice. MCN focuses on today''s major issues and high priority problems in maternal/child nursing, women''s health, and family nursing with extensive coverage of advanced practice healthcare issues relating to infants and young children. Each issue features peer-reviewed, clinically relevant articles. Coverage includes updates on disease and related care; ideas on health promotion; insights into patient and family behavior; discoveries in physiology and pathophysiology; clinical investigations; and research manuscripts that assist nurses toward evidence-based practices.
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