Chinese General Practice ›› 2023, Vol. 26 ›› Issue (30): 3801-3808.DOI: 10.12114/j.issn.1007-9572.2022.0879
Special Issue: 儿科最新文章合辑
• Original Research • Previous Articles Next Articles
Received:
2022-11-23
Revised:
2023-05-17
Published:
2023-10-20
Online:
2023-06-13
Contact:
CHEN Yan, HE Zhixu
通讯作者:
陈艳, 何志旭
作者简介:
基金资助:
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URL: https://www.chinagp.net/EN/10.12114/j.issn.1007-9572.2022.0879
组别 | 例数 | 年龄〔例(%)〕 | 性别(男/女) | 居住环境〔例(%)〕 | 起病时间〔例(%)〕 | |||
---|---|---|---|---|---|---|---|---|
≤12个月 | >12个月 | 农村 | 城市 | ≤7 d | >7 d | |||
T1组 | 43 | 4 (9.3) | 39(90.7) | 20/23 | 34(79.1) | 9(20.9) | 32(74.4) | 11(25.6) |
T2组 | 42 | 12(28.6) | 30(71.4) | 30/12 | 29(69.0) | 13(31.0) | 32(76.2) | 10(23.8) |
T3组 | 43 | 12(27.9) | 31(72.1) | 21/22 | 36(83.7) | 7(16.3) | 29(67.4) | 14(32.6) |
χ2值 | 5.995 | 6.496 | 2.721 | 0.920 | ||||
P值 | 0.050 | 0.039 | 0.257 | 0.631 | ||||
组别 | 进入PICU〔例(%)〕 | 病因〔例(%)〕 | 治疗方式〔例(%)〕 | |||||
是 | 否 | 原发性HLH | EB病毒感染 | 非EB病毒感染 | 不明原因 | 未采用PE/CRRT | 化疗+PE/CRRT | |
T1组 | 16(37.2) | 27(62.8) | 0 | 21(48.9) | 17(39.5) | 5(11.6) | 37(86.0) | 6(14.0) |
T2组 | 30(71.4) | 12(28.6) | 0 | 22(52.4) | 18(42.8) | 2(4.8) | 26(61.9) | 16(38.1) |
T3组 | 36(83.7) | 7(16.3) | 2(4.6) | 24(55.8) | 14(32.6) | 3(7.0) | 28(65.1) | 15(34.9) |
χ2值 | 21.676 | — | 0.420 | 0.997 | 1.376 | 7.152 | ||
P值 | <0.001 | 0.328 | 0.811 | 0.607 | 0.603 | 0.028 |
Table 1 Comparison of general data among the 3 groups of children with HLH
组别 | 例数 | 年龄〔例(%)〕 | 性别(男/女) | 居住环境〔例(%)〕 | 起病时间〔例(%)〕 | |||
---|---|---|---|---|---|---|---|---|
≤12个月 | >12个月 | 农村 | 城市 | ≤7 d | >7 d | |||
T1组 | 43 | 4 (9.3) | 39(90.7) | 20/23 | 34(79.1) | 9(20.9) | 32(74.4) | 11(25.6) |
T2组 | 42 | 12(28.6) | 30(71.4) | 30/12 | 29(69.0) | 13(31.0) | 32(76.2) | 10(23.8) |
T3组 | 43 | 12(27.9) | 31(72.1) | 21/22 | 36(83.7) | 7(16.3) | 29(67.4) | 14(32.6) |
χ2值 | 5.995 | 6.496 | 2.721 | 0.920 | ||||
P值 | 0.050 | 0.039 | 0.257 | 0.631 | ||||
组别 | 进入PICU〔例(%)〕 | 病因〔例(%)〕 | 治疗方式〔例(%)〕 | |||||
是 | 否 | 原发性HLH | EB病毒感染 | 非EB病毒感染 | 不明原因 | 未采用PE/CRRT | 化疗+PE/CRRT | |
T1组 | 16(37.2) | 27(62.8) | 0 | 21(48.9) | 17(39.5) | 5(11.6) | 37(86.0) | 6(14.0) |
T2组 | 30(71.4) | 12(28.6) | 0 | 22(52.4) | 18(42.8) | 2(4.8) | 26(61.9) | 16(38.1) |
T3组 | 36(83.7) | 7(16.3) | 2(4.6) | 24(55.8) | 14(32.6) | 3(7.0) | 28(65.1) | 15(34.9) |
χ2值 | 21.676 | — | 0.420 | 0.997 | 1.376 | 7.152 | ||
P值 | <0.001 | 0.328 | 0.811 | 0.607 | 0.603 | 0.028 |
组别 | 例数 | 发热 | 肝脏肿大 | 脾脏肿大 | 真菌感染 | 弥散性血管内凝血 | 呼吸衰竭 | 中枢神经系统损害 | 急性呼吸窘迫综合征 | 肺炎 | 休克 | 胃肠道出血 | 肺出血 | 多器官功能障碍受累器官数量 | |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
≥3个 | <3个 | ||||||||||||||
T1组 | 43 | 43(100.0) | 28(65.1) | 36(83.7) | 10(23.3) | 7(16.3) | 9(20.9) | 6(14.0) | 4(9.3) | 27(62.8) | 8(18.6) | 5(11.6) | 3 (7.0) | 15(34.9) | 28(65.1) |
T2组 | 42 | 42(100.0) | 31(73.8) | 29(69.1) | 12(28.6) | 11(26.2) | 19(45.2) | 13(31.0) | 3(7.1) | 36(85.7) | 9(21.4) | 7(16.7) | 6(14.3) | 20(47.6) | 22(52.4) |
T3组 | 43 | 43(100.0) | 22(51.2) | 35(81.4) | 4 (9.3) | 12(27.9) | 22(52.4) | 14(32.6) | 2(4.7) | 32(74.4) | 18(41.9) | 10(23.3) | 6(14.0) | 18(41.9) | 25(58.1) |
χ2值 | — | 4.784 | 3.082 | 5.220 | 1.884 | 9.257 | 4.763 | 0.713 | 5.836 | 6.954 | 2.054 | 1.410 | 1.426 | ||
P值 | 1.000 | 0.091 | 0.214 | 0.074 | 0.390 | 0.010 | 0.020 | 0.700 | 0.054 | 0.031 | 0.358 | 0.494 | 0.490 |
Table 2 Comparison of clinical symptoms and signs among the 3 groups of children with HLH
组别 | 例数 | 发热 | 肝脏肿大 | 脾脏肿大 | 真菌感染 | 弥散性血管内凝血 | 呼吸衰竭 | 中枢神经系统损害 | 急性呼吸窘迫综合征 | 肺炎 | 休克 | 胃肠道出血 | 肺出血 | 多器官功能障碍受累器官数量 | |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
≥3个 | <3个 | ||||||||||||||
T1组 | 43 | 43(100.0) | 28(65.1) | 36(83.7) | 10(23.3) | 7(16.3) | 9(20.9) | 6(14.0) | 4(9.3) | 27(62.8) | 8(18.6) | 5(11.6) | 3 (7.0) | 15(34.9) | 28(65.1) |
T2组 | 42 | 42(100.0) | 31(73.8) | 29(69.1) | 12(28.6) | 11(26.2) | 19(45.2) | 13(31.0) | 3(7.1) | 36(85.7) | 9(21.4) | 7(16.7) | 6(14.3) | 20(47.6) | 22(52.4) |
T3组 | 43 | 43(100.0) | 22(51.2) | 35(81.4) | 4 (9.3) | 12(27.9) | 22(52.4) | 14(32.6) | 2(4.7) | 32(74.4) | 18(41.9) | 10(23.3) | 6(14.0) | 18(41.9) | 25(58.1) |
χ2值 | — | 4.784 | 3.082 | 5.220 | 1.884 | 9.257 | 4.763 | 0.713 | 5.836 | 6.954 | 2.054 | 1.410 | 1.426 | ||
P值 | 1.000 | 0.091 | 0.214 | 0.074 | 0.390 | 0.010 | 0.020 | 0.700 | 0.054 | 0.031 | 0.358 | 0.494 | 0.490 |
组别 | 例数 | 总胆红素〔M(P25,P75),μmol/L〕 | 血清尿素氮〔M(P25,P75),mmol/L〕 | 白蛋白(g/L) | 肌酐〔M(P25,P75),μmol/L〕 | 乳酸脱氢酶〔M(P25,P75),U/L〕 | 肌酸激酶同工酶〔M(P25,P75),U/L〕 | 白细胞计数〔M(P25,P75),×109/L〕 |
---|---|---|---|---|---|---|---|---|
T1组 | 43 | 12.1(8.6,27.0) | 3.84(2.79,5.34) | 32.34±5.35 | 37(28,54) | 810 (521,1 339) | 26(16,44) | 1.78(1.05,5.35) |
T2组 | 42 | 12.7(6.7,31.9) | 3.90(2.84,5.70) | 30.13±5.58 | 34(27,42) | 973 (826,1 472) | 35(23,45) | 2.13(1.16,4.71) |
T3组 | 43 | 29.8(8.5,56.6) | 4.36(3.07,8.38) | 28.30±5.40ab | 36(28,59) | 2 017(1 281,4 496)ab | 44(30,90)ab | 2.25(1.45,3.73) |
H(F)值 | 3.971 | 1.632 | 5.930c | 1.956 | 27.796 | 11.059 | 0.373 | |
P值 | 0.137 | 0.442 | 0.003 | 0.376 | <0.001 | 0.004 | 0.829 | |
组别 | 中性粒细胞绝对值〔M(P25,P75),×109/L〕 | 血小板计数〔M(P25,P75),×109/L〕 | 血红蛋白(g/L) | 血清铁蛋白〔M(P25,P75),μg/L〕 | C反应蛋白〔M(P25,P75),mg/L〕 | K+(mmol/L) | Ca2+〔M(P25,P75),mmol/L〕 | Na+(mmol/L) |
T1组 | 0.58(0.33,1.17) | 52(30,99) | 90.16±22.43 | 1 500(956,6 179) | 7.20(2.50,34.50) | 4.19±0.67 | 2.08(1.92,2.23) | 134.87±4.63 |
T2组 | 0.67(0.31,2.30) | 52(21,99) | 92.64±17.89 | 4 795(1 798,11 609) | 32.91(5.59,79.68) | 4.19±0.56 | 2.04(1.91,2.19) | 132.14±3.74 |
T3组 | 0.92(0.38,1.81) | 58(28,82) | 88.95±18.24 | 5 624(1 900,24 134)ab | 25.96(12.80,77.90)ab | 4.15±0.84 | 2.02(1.75,2.16) | 132.54±5.24 |
H(F)值 | 2.108 | 0.257 | 0.389c | 13.155 | 15.266 | 0.052c | 4.679 | 4.426c |
P值 | 0.349 | 0.880 | 0.679 | 0.001 | <0.001 | 0.949 | 0.096 | 0.014 |
组别 | 血浆凝血酶原时间〔M(P25,P75),s〕 | 活化部分凝血活酶时间〔M(P25,P75),s〕 | 纤维蛋白原〔M(P25,P75),g/L〕 | 免疫球蛋白G〔M(P25,P75),mg/L〕 | 免疫球蛋白A〔M(P25,P75),mg/L〕 | 免疫球蛋白M〔M(P25,P75),mg/L〕 | 免疫球蛋白E〔M(P25,P75),mg/L〕 | |
T1组 | 12.2(11.2,15.0) | 39.4(34.9,51.5) | 1.56(1.18,2.10) | 8.27(5.56,12.38) | 1.01(0.34,1.43) | 0.85(0.44,1.39) | 33.90(11.40,103.50) | |
T2组 | 13.3(11.3,16.0) | 41.8(35.1,52.5) | 1.30(0.88,1.92) | 7.99(5.65,12.35) | 0.79(0.44,1.80) | 0.84(0.51,1.19) | 97.00(38.60,201.00) | |
T3组 | 15.4(11.3,20.5) | 55.2(43.2,85.9)ab | 1.06(0.73,1.52)ab | 7.92(5.12,11.55) | 0.74(0.32,1.20) | 0.57(0.32,1.24) | 79.65(13.13,162.50) | |
H(F)值 | 3.051 | 16.612 | 6.874 | 0.513 | 0.652 | 3.052 | 5.488 | |
P值 | 0.218 | <0.001 | 0.032 | 0.774 | 0.722 | 0.217 | 0.064 | |
组别 | 白介素2〔M(P25,P75),μg/L〕 | 白介素4〔M(P25,P75),μg/L〕 | 白介素6〔M(P25,P75),μg/L〕 | 白介素10〔M(P25,P75),μg/L〕 | 肿瘤坏死因子α〔M(P25,P75),μg/L〕 | 干扰素γ〔M(P25,P75),μg/L〕 | 三酰甘油〔M(P25,P75),mmol/L〕 | |
T1组 | 0.98(0.39,1.58) | 0.66(0.11,1.70) | 16.33(5.83,55.20) | 77.18(8.75,429.47) | 0.18(0.08,1.24) | 3.60(0.46,179.81) | 2.7(2.0,4.0) | |
T2组 | 1.34(0.30,2.12) | 0.95(0.14,1.72) | 56.93(5.07,178.40) | 226.81(48.70,513.05) | 0.37(0,1.17) | 20.30(0.47,550.79) | 3.5(2.4,4.7) | |
T3组 | 1.16(0.88,1.60) | 1.00(0.28,1.27) | 56.20(18.08,146.42) | 162.21(23.40,1 035.63) | 0.62(0.21,1.66) | 66.53(4.37,467.12) | 3.4(2.0,4.1) | |
H(F)值 | 1.039 | 0.073 | 2.990 | 0.974 | 2.397 | 1.725 | 3.187 | |
P值 | 0.595 | 0.964 | 0.224 | 0.614 | 0.302 | 0.422 | 0.203 |
Table 3 Comparison of laboratory test results among the 3 groups of children with HLH
组别 | 例数 | 总胆红素〔M(P25,P75),μmol/L〕 | 血清尿素氮〔M(P25,P75),mmol/L〕 | 白蛋白(g/L) | 肌酐〔M(P25,P75),μmol/L〕 | 乳酸脱氢酶〔M(P25,P75),U/L〕 | 肌酸激酶同工酶〔M(P25,P75),U/L〕 | 白细胞计数〔M(P25,P75),×109/L〕 |
---|---|---|---|---|---|---|---|---|
T1组 | 43 | 12.1(8.6,27.0) | 3.84(2.79,5.34) | 32.34±5.35 | 37(28,54) | 810 (521,1 339) | 26(16,44) | 1.78(1.05,5.35) |
T2组 | 42 | 12.7(6.7,31.9) | 3.90(2.84,5.70) | 30.13±5.58 | 34(27,42) | 973 (826,1 472) | 35(23,45) | 2.13(1.16,4.71) |
T3组 | 43 | 29.8(8.5,56.6) | 4.36(3.07,8.38) | 28.30±5.40ab | 36(28,59) | 2 017(1 281,4 496)ab | 44(30,90)ab | 2.25(1.45,3.73) |
H(F)值 | 3.971 | 1.632 | 5.930c | 1.956 | 27.796 | 11.059 | 0.373 | |
P值 | 0.137 | 0.442 | 0.003 | 0.376 | <0.001 | 0.004 | 0.829 | |
组别 | 中性粒细胞绝对值〔M(P25,P75),×109/L〕 | 血小板计数〔M(P25,P75),×109/L〕 | 血红蛋白(g/L) | 血清铁蛋白〔M(P25,P75),μg/L〕 | C反应蛋白〔M(P25,P75),mg/L〕 | K+(mmol/L) | Ca2+〔M(P25,P75),mmol/L〕 | Na+(mmol/L) |
T1组 | 0.58(0.33,1.17) | 52(30,99) | 90.16±22.43 | 1 500(956,6 179) | 7.20(2.50,34.50) | 4.19±0.67 | 2.08(1.92,2.23) | 134.87±4.63 |
T2组 | 0.67(0.31,2.30) | 52(21,99) | 92.64±17.89 | 4 795(1 798,11 609) | 32.91(5.59,79.68) | 4.19±0.56 | 2.04(1.91,2.19) | 132.14±3.74 |
T3组 | 0.92(0.38,1.81) | 58(28,82) | 88.95±18.24 | 5 624(1 900,24 134)ab | 25.96(12.80,77.90)ab | 4.15±0.84 | 2.02(1.75,2.16) | 132.54±5.24 |
H(F)值 | 2.108 | 0.257 | 0.389c | 13.155 | 15.266 | 0.052c | 4.679 | 4.426c |
P值 | 0.349 | 0.880 | 0.679 | 0.001 | <0.001 | 0.949 | 0.096 | 0.014 |
组别 | 血浆凝血酶原时间〔M(P25,P75),s〕 | 活化部分凝血活酶时间〔M(P25,P75),s〕 | 纤维蛋白原〔M(P25,P75),g/L〕 | 免疫球蛋白G〔M(P25,P75),mg/L〕 | 免疫球蛋白A〔M(P25,P75),mg/L〕 | 免疫球蛋白M〔M(P25,P75),mg/L〕 | 免疫球蛋白E〔M(P25,P75),mg/L〕 | |
T1组 | 12.2(11.2,15.0) | 39.4(34.9,51.5) | 1.56(1.18,2.10) | 8.27(5.56,12.38) | 1.01(0.34,1.43) | 0.85(0.44,1.39) | 33.90(11.40,103.50) | |
T2组 | 13.3(11.3,16.0) | 41.8(35.1,52.5) | 1.30(0.88,1.92) | 7.99(5.65,12.35) | 0.79(0.44,1.80) | 0.84(0.51,1.19) | 97.00(38.60,201.00) | |
T3组 | 15.4(11.3,20.5) | 55.2(43.2,85.9)ab | 1.06(0.73,1.52)ab | 7.92(5.12,11.55) | 0.74(0.32,1.20) | 0.57(0.32,1.24) | 79.65(13.13,162.50) | |
H(F)值 | 3.051 | 16.612 | 6.874 | 0.513 | 0.652 | 3.052 | 5.488 | |
P值 | 0.218 | <0.001 | 0.032 | 0.774 | 0.722 | 0.217 | 0.064 | |
组别 | 白介素2〔M(P25,P75),μg/L〕 | 白介素4〔M(P25,P75),μg/L〕 | 白介素6〔M(P25,P75),μg/L〕 | 白介素10〔M(P25,P75),μg/L〕 | 肿瘤坏死因子α〔M(P25,P75),μg/L〕 | 干扰素γ〔M(P25,P75),μg/L〕 | 三酰甘油〔M(P25,P75),mmol/L〕 | |
T1组 | 0.98(0.39,1.58) | 0.66(0.11,1.70) | 16.33(5.83,55.20) | 77.18(8.75,429.47) | 0.18(0.08,1.24) | 3.60(0.46,179.81) | 2.7(2.0,4.0) | |
T2组 | 1.34(0.30,2.12) | 0.95(0.14,1.72) | 56.93(5.07,178.40) | 226.81(48.70,513.05) | 0.37(0,1.17) | 20.30(0.47,550.79) | 3.5(2.4,4.7) | |
T3组 | 1.16(0.88,1.60) | 1.00(0.28,1.27) | 56.20(18.08,146.42) | 162.21(23.40,1 035.63) | 0.62(0.21,1.66) | 66.53(4.37,467.12) | 3.4(2.0,4.1) | |
H(F)值 | 1.039 | 0.073 | 2.990 | 0.974 | 2.397 | 1.725 | 3.187 | |
P值 | 0.595 | 0.964 | 0.224 | 0.614 | 0.302 | 0.422 | 0.203 |
指标 | AUC | 95%CI | 特异度 | 灵敏度 | 最佳截断值 |
---|---|---|---|---|---|
C反应蛋白 | 0.560 | (0.451,0.669) | 0.876 | 0.256 | 82.08 mg/L |
肌酸激酶同工酶 | 0.666 | (0.560,0.772) | 0.697 | 0.615 | 40.5 U/L |
乳酸脱氢酶 | 0.605 | (0.499,0.710) | 0.449 | 0.769 | 927.5 U/L |
活化部分凝血活酶时间 | 0.724 | (0.626,0.822) | 0.798 | 0.590 | 53.95 s |
血清铁蛋白 | 0.648 | (0.551,0.745) | 0.472 | 0.897 | 1 897 μg/L |
纤维蛋白原 | 0.715 | (0.624,0.807) | 0.562 | 0.821 | 1.45 g/L |
Table 4 Predictive value of laboratory test results for death in children with HLH
指标 | AUC | 95%CI | 特异度 | 灵敏度 | 最佳截断值 |
---|---|---|---|---|---|
C反应蛋白 | 0.560 | (0.451,0.669) | 0.876 | 0.256 | 82.08 mg/L |
肌酸激酶同工酶 | 0.666 | (0.560,0.772) | 0.697 | 0.615 | 40.5 U/L |
乳酸脱氢酶 | 0.605 | (0.499,0.710) | 0.449 | 0.769 | 927.5 U/L |
活化部分凝血活酶时间 | 0.724 | (0.626,0.822) | 0.798 | 0.590 | 53.95 s |
血清铁蛋白 | 0.648 | (0.551,0.745) | 0.472 | 0.897 | 1 897 μg/L |
纤维蛋白原 | 0.715 | (0.624,0.807) | 0.562 | 0.821 | 1.45 g/L |
项目 | 单因素Cox比例风险回归分析 | 多因素Cox比例风险回归分析 | ||
---|---|---|---|---|
HR(95%CI) | P值 | HR(95%CI) | P值 | |
男性 | 1.34(0.70,2.56) | 0.375 | ||
进入PICU | 4.88(1.91,12.50) | 0.001 | ||
化疗+PE/CRRT | 0.55(0.25,1.20) | 0.135 | ||
呼吸衰竭 | 5.40(2.68,10.90) | <0.001 | ||
中枢神经系统损害 | 4.48(2.37,8.46) | <0.001 | ||
休克 | 7.03(3.64,13.57) | <0.001 | 4.242(2.09,8.61) | <0.001 |
白蛋白≤30 g/L | 5.09(2.34,11.10) | <0.001 | ||
肌酸激酶同工酶≥40.5 U/L | 3.19(1.67,6.11) | <0.001 | ||
Na+≤130 mmol/L | 2.67(1.15,5.06) | 0.003 | ||
活化部分凝血活酶时间≥53.95 s | 4.58(2.41,8.69) | 0.001 | 2.44(1.24,4.81) | 0.010 |
纤维蛋白原≤1.45 g/L | 2.15(4.87,11.05) | <0.001 | ||
C-反应蛋白≥82.08 mg/L | 1.97(0.96,4.05) | 0.066 | ||
乳酸脱氢酶≥927.5 U/L | 1.23(2.60,5.47) | 0.012 | ||
血清铁蛋白≥1 897 μg/L | 6.13(2.18,17.27) | 0.001 | 3.05(1.02,9.09) | 0.046 |
Table 5 Cox proportional hazard regression model analysis of the influencing factors of death in children with HLH
项目 | 单因素Cox比例风险回归分析 | 多因素Cox比例风险回归分析 | ||
---|---|---|---|---|
HR(95%CI) | P值 | HR(95%CI) | P值 | |
男性 | 1.34(0.70,2.56) | 0.375 | ||
进入PICU | 4.88(1.91,12.50) | 0.001 | ||
化疗+PE/CRRT | 0.55(0.25,1.20) | 0.135 | ||
呼吸衰竭 | 5.40(2.68,10.90) | <0.001 | ||
中枢神经系统损害 | 4.48(2.37,8.46) | <0.001 | ||
休克 | 7.03(3.64,13.57) | <0.001 | 4.242(2.09,8.61) | <0.001 |
白蛋白≤30 g/L | 5.09(2.34,11.10) | <0.001 | ||
肌酸激酶同工酶≥40.5 U/L | 3.19(1.67,6.11) | <0.001 | ||
Na+≤130 mmol/L | 2.67(1.15,5.06) | 0.003 | ||
活化部分凝血活酶时间≥53.95 s | 4.58(2.41,8.69) | 0.001 | 2.44(1.24,4.81) | 0.010 |
纤维蛋白原≤1.45 g/L | 2.15(4.87,11.05) | <0.001 | ||
C-反应蛋白≥82.08 mg/L | 1.97(0.96,4.05) | 0.066 | ||
乳酸脱氢酶≥927.5 U/L | 1.23(2.60,5.47) | 0.012 | ||
血清铁蛋白≥1 897 μg/L | 6.13(2.18,17.27) | 0.001 | 3.05(1.02,9.09) | 0.046 |
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蒋学琴,黄娇娇,黄成双,等. 儿童重型噬血细胞综合征的临床特征及预后相关因素分析[J]. 中华医学杂志,2022,102(28):2181-2188. DOI:10.3760/cma.j.cn112137-20220214-00308.
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