墨西哥儿童抗磷脂综合征:进化、实验室和临床特征:10年经验

María Teresa Braña-Ruiz
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引用次数: 1

摘要

简介:抗磷脂综合征(APS)是一种自身免疫性多系统疾病,其特征是存在高滴度抗磷脂抗体时,会出现血栓事件、妊娠期发病率以及血液学、皮肤学和神经系统表现。它可以表现为原发性、孤立的临床实体,也可以表现为风湿病的继发性表现,主要是系统性红斑狼疮。在首次描述35年后,对这种病理学的理解仍在发展,在儿童的表现中更是如此。目的:对我院儿科APS人群进行人口学描述,为我们制定诊断标准和有针对性的治疗管理提供依据。它还将允许及时诊断,避免与这种病理学相关的发病率和死亡率。材料和方法:我们进行了一项描述性的横断面研究。我们在档案中搜索了2000年3月至2018年3月在墨西哥步兵医院Federico Gomez进行评估的16岁以下患者,这些患者符合札幌抗磷脂综合征分类标准。结果:通过查阅档案,我们记录了2000年3月至2018年3月在墨西哥步兵医院Federico Gomez的29名患者符合札幌APS分类标准。52%的患者为女性,48%为男性。患者的平均年龄为9.8岁,最低2.2岁,最高16岁。诊断时的平均年龄为14.8岁,标准偏差为4.56(范围为6.2至14.2岁)。在29名患者中,48%被诊断为原发性APS,52%被诊断为继发性APS。在所有继发性APS病例中,系统性红斑狼疮是主要的风湿病。结论:抗磷脂综合征在儿科人群中是一种复杂且描述不清的病理学。其多变的表现和不可预测的性质意味着儿科医生在诊断和治疗方面面临挑战。我们医院对APS患儿的流行病学描述对制定诊断和治疗指南非常有价值。未来的审查,与三级国际风湿病中心的审查相关,是必要的。
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Antiphospholipid syndrome in Mexican children: evolution, laboratory and clinical characteristics: a 10-year experience
Introduction: Antiphospholipid syndrome (APS) is an autoimmune, multisystemic disease characterized by the presence of thrombotic events, gestational morbidity, as well as hematological, dermatological and neurological manifestations, in the presence of high titers of antiphospholipid antibodies. It can present as primary, an isolated clinical entity, or secondary, as a manifestation of a rheumatologic disease, primarily systemic lupus erythematosus. 35 years after its first description, the understanding of this pathology is still evolving and even more so in the presentation in children. Objective: To do a demographic description of pediatric population with APS in our hospital, allowing us to establish diagnostic criteria and targeted therapeutic management. It will also allow a prompt diagnosis, avoiding the morbidity and mortality associated with this pathology. Materials and Methods: We performed a descriptive cross-sectional study. We searched in our archives for patients under 16 years of age, evaluated at the Hospital Infantil de Mexico Federico Gomez from March 2000 to March 2018 that met Sapporo criteria for classification of antiphospholipid syndrome. Results: By reviewing the archives, we documented 29 patients were met Sapporo criteria for classification of APS from March 2000 to March 2018 at Hospital Infantil de Mexico Federico Gomez. 52% of the patients were female, while 48% male. The average age of patients was 9.8 years, with a minimum of 2.2 and a maximum of 16 years. The mean age at diagnosis was 14.8 years, with a standard deviation of 4.56 (range from 6.2 to 14.2 years). Of the 29 patients, 48% were diagnosed with primary APS, while 52% with secondary. Systemic Lupus Erythematosus was the primary rheumatologic disease in all the cases of secondary APS. Conclusion: Antiphospholipid syndrome is a complex and not well described pathology in pediatric population. Its variable presentation and unpredictable nature implies a diagnostic and treatment challenge for pediatricians. Epidemiological description of the pediatric population with APS at our hospital is very valuable for the development of diagnostic and therapeutic guidelines. Future reviews, in correlation with reviews from tertiary international rheumatologic centers are needed.
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