血浆置换是患有危及生命的自身免疫性溶血性贫血的重症儿童的希望所在

IF 0.5 Q4 PEDIATRICS
Noha El-Anwar, Hafez Bazaraa, Fatma Abdel Maksoud, Yasmin Ramadan
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引用次数: 0

摘要

自身免疫性溶血性贫血(AIHA)是一种罕见的儿童疾病,有时会出现急性、危及生命、进展迅速的病程,需要及时治疗。我们的研究旨在描述血浆置换在急性期治疗需要输血且大剂量皮质类固醇治疗无效的自身免疫性溶血性贫血儿科患者中的作用和结果。这是一份描述性回顾性报告,涉及儿童大学医院儿科重症监护室(PICU)自2017年6月至2022年6月期间收治的所有因脉冲类固醇治疗耐药的急性难治性AIHA而接受血浆置换的患者。研究人员收集了入院时和出院时的人口统计学数据、生命体征和实验室检查结果。报告了每位患者所需的 PE 治疗次数、交换用量、置换类型、使用的静脉通路、并发症和结果。该系列包括 19 名患者,其中男性 10 人,女性 9 人,中位年龄和体重分别为 76 个月(IQR 18-121)和 20.9 公斤。平均血红蛋白从出院时的 5.3 ± 1.8 g/dl 提高到 9.9 ± 2.6 g/dl。PE 治疗的平均次数为 2.4 次,未出现不良反应。PICU 平均住院时间为 16.6 天。2名患者(10.5%)因原发病死亡,7名患者(36.8%)需要进一步接受免疫治疗,5名患者(28%)病情复发。对于对类固醇治疗无效或无法输注匹配的 PRBCs 的严重急性危及生命的 AIHA 患儿,PE 可作为一种安全、成功的疗法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Plasma exchange is the hope for critically ill children with life-threatening autoimmune hemolytic anemia
Autoimmune hemolytic anemia (AIHA) is a rare disease in children, sometimes associated with acute, life-threatening, rapidly progressive course requiring prompt management. The aim of our study is to describe the role and outcome of plasma exchange in the acute management of pediatric patients with AIHA requiring transfusion and refractory to high doses of corticosteroids. This was a descriptive retrospective report of all patients admitted to the pediatric intensive care unit (PICU) of Children’s University Hospital who received PE for acute intractable AIHA resistant to management with pulse steroids starting from June 2017 to June 2022. The demographic data, vitals, and laboratory investigations at PICU admission and upon discharge were gathered. The number of PE sessions needed for each patient, volume used for exchange, type of replacement, IV access used, complications, and outcome were reported. This series included 19 patients, 10 males, and 9 females, with a median age and weight of 76 months (IQR 18–121), and 20.9 kg. Improvement of the mean hemoglobin was observed from 5.3 ± 1.8 to 9.9 ± 2.6 g/dl at discharge. The average number of PE sessions was 2.4 sessions with no adverse effects encountered. The mean PICU stay was 16.6 days. Mortality occurred in 2 patients (10.5%) due to their primary illness, while 7 patients (36.8%) were in need of further immunotherapy, and 5 patients (28%) showed relapse. PE may be used as a safe and successful therapy in children with severe acute life-threatening AIHA not responding to steroids, or if well-matched PRBCs are unavailable for transfusion.
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来源期刊
自引率
0.00%
发文量
32
审稿时长
9 weeks
期刊介绍: The Gazette is the official journal of the Egyptian Pediatric Association. The main purpose of the Gazette is to provide a place for the publication of high-quality papers documenting recent advances and new developments in both pediatrics and pediatric surgery in clinical and experimental settings. An equally important purpose of the Gazette is to publish local and regional issues related to children and child care. The Gazette welcomes original papers, review articles, case reports and short communications as well as short technical reports. Papers submitted to the Gazette are peer-reviewed by a large review board. The Gazette also offers CME quizzes, credits for which can be claimed from either the EPA website or the EPA headquarters. Fields of interest: all aspects of pediatrics, pediatric surgery, child health and child care. The Gazette complies with the Uniform Requirements for Manuscripts submitted to biomedical journals as recommended by the International Committee of Medical Journal Editors (ICMJE).
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