Noha El-Anwar, Hafez Bazaraa, Fatma Abdel Maksoud, Yasmin Ramadan
{"title":"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","authors":"Noha El-Anwar, Hafez Bazaraa, Fatma Abdel Maksoud, Yasmin Ramadan","doi":"10.1186/s43054-024-00270-6","DOIUrl":null,"url":null,"abstract":"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.","PeriodicalId":43064,"journal":{"name":"Egyptian Pediatric Association Gazette","volume":null,"pages":null},"PeriodicalIF":0.5000,"publicationDate":"2024-05-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Plasma exchange is the hope for critically ill children with life-threatening autoimmune hemolytic anemia\",\"authors\":\"Noha El-Anwar, Hafez Bazaraa, Fatma Abdel Maksoud, Yasmin Ramadan\",\"doi\":\"10.1186/s43054-024-00270-6\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"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.\",\"PeriodicalId\":43064,\"journal\":{\"name\":\"Egyptian Pediatric Association Gazette\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.5000,\"publicationDate\":\"2024-05-29\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Egyptian Pediatric Association Gazette\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1186/s43054-024-00270-6\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"PEDIATRICS\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Egyptian Pediatric Association Gazette","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1186/s43054-024-00270-6","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"PEDIATRICS","Score":null,"Total":0}
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.
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).