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儿童血液恶性肿瘤患者中的毛霉菌病是一种可挽救的疾病:来自以色列儿童白血病研究组的报告。

Mucormycosis in children with haematological malignancies is a salvageable disease: a report from the Israeli Study Group of Childhood Leukemia.

机构信息

The Rina Zaizov Division of Pediatric Hematology-Oncology, Schneider Children's Medical Center, Petah Tikva, Israel.

Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

出版信息

Br J Haematol. 2020 Apr;189(2):339-350. doi: 10.1111/bjh.16329. Epub 2019 Dec 29.

Abstract

Mucormycosis has emerged as an increasingly important cause of morbidity and mortality in immunocompromised patients, but contemporary data in children are lacking. We conducted a nationwide multicentre study to investigate the characteristics of mucormycosis in children with haematological malignancies. The cohort included 39 children with mucormycosis: 25 of 1136 children (incidence 2·2%) with acute leukaemias prospectively enrolled in a centralized clinical registry in 2004-2017, and an additional 14 children with haematological malignancies identified by retrospective search of the databases of seven paediatric haematology centres. Ninety-two percent of mucormycosis cases occurred in patients with acute leukaemias. Mucormycosis was significantly associated with high-risk acute lymphoblastic leukaemia (OR 3·75; 95% CI 1·51-9·37; P = 0·004) and with increasing age (OR 3·58; 95% CI 1·24-9·77; P = 0·01). Fifteen patients (38%) died of mucormycosis. Rhinocerebral pattern was independently associated with improved 12-week survival (OR 9·43; 95% CI 1·47-60·66; P = 0·02) and relapsed underlying malignancy was associated with increased 12-week mortality (OR 6·42; 95% CI, 1·01-40·94; P = 0·05). In patients receiving frontline therapy for their malignancy (n = 24), one-year cumulative mucormycosis-related mortality was 21 ± 8% and five-year overall survival was 70 ± 8%. This largest paediatric population-based study of mucormycosis demonstrates that children receiving frontline therapy for their haematological malignancy are often salvageable.

摘要

毛霉菌病已成为免疫功能低下患者发病率和死亡率日益增加的重要原因,但目前缺乏儿童方面的相关数据。我们进行了一项全国性多中心研究,旨在调查血液恶性肿瘤患儿毛霉菌病的特征。该队列纳入了 39 例毛霉菌病患儿:25 例(发生率为 2.2%)为前瞻性纳入 2004 年至 2017 年集中临床登记处的 1136 例急性白血病患儿,另外 14 例为通过对 7 个儿科血液病中心数据库的回顾性搜索确定的血液恶性肿瘤患儿。92%的毛霉菌病病例发生在急性白血病患儿中。毛霉菌病与高危急性淋巴细胞白血病显著相关(OR 3.75;95%CI 1.51-9.37;P=0.004),且与年龄增长相关(OR 3.58;95%CI 1.24-9.77;P=0.01)。15 例(38%)患儿死于毛霉菌病。鼻-颅模式与 12 周生存率提高独立相关(OR 9.43;95%CI 1.47-60.66;P=0.02),而基础恶性肿瘤复发与 12 周死亡率增加相关(OR 6.42;95%CI,1.01-40.94;P=0.05)。在接受恶性肿瘤一线治疗的患儿(n=24)中,1 年毛霉菌病相关死亡率为 21±8%,5 年总生存率为 70±8%。这是最大的一项儿童毛霉菌病基于人群的研究,表明接受恶性肿瘤一线治疗的儿童通常可获救。

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