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缺铁和疲劳在出血性疾病青少年中。

Iron deficiency and fatigue among adolescents with bleeding disorders.

机构信息

Division of Pediatric Hematology/Oncology, University of Texas Southwestern Medical Center, Dallas, Texas, USA.

Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, Texas, USA.

出版信息

Am J Hematol. 2022 Jan 1;97(1):60-67. doi: 10.1002/ajh.26389. Epub 2021 Nov 8.

Abstract

Iron deficiency anemia is associated with heavy menstrual bleeding (HMB) and, by extension, a bleeding disorder (BD). It is unknown if iron deficiency without anemia is associated with a BD in adolescents. Moreover, the threshold of ferritin associated with fatigue in adolescents with HMB is unclear. In this multicenter study, we enrolled adolescents with HMB without BD. Participants underwent BD and anemia work-up in Young Women's Hematology Clinics and completed the Peds QL™ fatigue scale. BDs were defined as von Willebrand Disease, platelet function defect, clotting factor deficiencies, and hypermobility syndrome. Two hundred and fifty consecutive adolescents were enrolled, of whom 196 met eligibility criteria. Overall, 43% (95% confidence interval: 36%-50%) were diagnosed with BD. A total of 61% (n = 119) had serum ferritin levels < 15 ng/mL, 23.5% (n = 46) had iron deficiency only, and 37% (n = 73) had iron deficiency anemia. Low ferritin or ferritin dichotomized as < 15 or ≥ 15 ng/mL was not associated with BD on univariable analysis (p = .24) or when accounting for age, race, ethnicity, body mass index, and hemoglobin (p = .35). A total of 85% had total fatigue score below the population mean of 80.5, and 52% (n = 102) were > 2 SD (or < 54) below the mean, the cut-off associated with severe fatigue. A ferritin threshold of < 6 ng/mL had a specificity of 79.8% but a sensitivity of 36% for severe fatigue. In conclusion, iron deficiency without anemia is not a predictor of BD in adolescents with HMB in a specialty setting. Severe fatigue, especially sleep fatigue, is prevalent in adolescents with BD. Ferritin of < 6 ng/mL has ~80% specificity for severe fatigue in adolescents with HMB.

摘要

缺铁性贫血与月经过多(HMB)有关,进而与出血性疾病(BD)相关。目前尚不清楚无贫血的缺铁是否与青少年的 BD 相关。此外,与 HMB 青少年疲劳相关的铁蛋白阈值尚不清楚。在这项多中心研究中,我们招募了无 BD 的 HMB 青少年患者。参与者在青年女性血液诊所进行了 BD 和贫血检查,并完成了 PedsQL™疲劳量表。BD 定义为血管性血友病、血小板功能缺陷、凝血因子缺乏症和高迁移率族蛋白综合征。共纳入 250 名连续的青少年患者,其中 196 名符合入选标准。总体而言,43%(95%置信区间:36%-50%)被诊断为 BD。共有 61%(n=119)的血清铁蛋白水平<15ng/mL,23.5%(n=46)仅存在缺铁,37%(n=73)存在缺铁性贫血。单变量分析(p=0.24)或在考虑年龄、种族、民族、体重指数和血红蛋白后(p=0.35),低铁蛋白或铁蛋白二分位数<15 或≥15ng/mL 与 BD 无关。共有 85%的总疲劳评分低于 80.5 的人群平均值,52%(n=102)的评分低于平均值 2 个标准差(或<54),这是与严重疲劳相关的临界值。铁蛋白<6ng/mL 的特异性为 79.8%,但对严重疲劳的敏感性为 36%。结论,在专科环境中,无贫血的缺铁不是 HMB 青少年 BD 的预测因素。严重疲劳,尤其是睡眠疲劳,在 BD 青少年中很常见。铁蛋白<6ng/mL 对 HMB 青少年严重疲劳的特异性约为 80%。

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