目的 调查重型β地中海贫血患儿照顾者的负担现状，并分析其影响因素，为医护人员制定干预方案提供依据。方法 采用便利抽样法，于2024年1月—12月选取华南地区5家医院就诊的重型β地中海贫血患儿的主要照顾者作为调查对象，采用一般资料调查表、Zarit照顾者负担量表、中文简化版家庭弹性量表和社会支持评定量表进行调查，并采用单因素分析、相关性分析及多重线性回归探究其影响因素。结果 共发放问卷242份，回收232份，回收率为95.87%。重型β地中海贫血患儿主要照顾者照顾负担的总体平均得分为（36.67±18.63）分。多重线性回归分析结果显示，患儿输血频率、输血相关性皮肤过敏和社会支持是患儿照顾负担的影响因素（P＜0.001），可解释总变异的23.9%。结论 重型β地中海贫血患儿照顾者负担程度以中重度为主。患儿曾出现输血不良反应、输血频率更高，其照顾者负担更重；而主要照顾者具有良好的社会支持水平有助于降低其照顾者负担。医护人员应针对以上因素制定干预方案，以减轻重型β地中海贫血患儿照顾者负担水平。

      Objective To investigate the current burden in caregivers of children with β thalassemia major and analyze its influencing factors，so as to provide a basis for medical staff to formulate intervention plans．Methods By using the convenience sampling method，primary caregivers of children with β thalassemia major from five hospitals in South China from January to December 2024 were selected as the survey subjects．The general information questionnaire，Zarit Caregiver Burden Scale，Simplified Chinese Family Resilience Scale and Social Support Rating Scale were used for the survey．And univariate analysis，correlation analysis and multiple linear regression were adopted to explore its influencing factors．Results A total of 242 questionnaires were distributed and 232 were retrieved，with a recovery rate of 95.87%．The overall average score of care burden for primary caregivers of children with β thalassemia major was（36.67±18.63）．The results of multiple linear regression analysis showed that the frequency of blood transfusion in children，transfusion-related skin allergy and social support were the influencing factors of the care burden（P＜0.001），which could explain 23.9% of the total variation．Conclusions The burden level in caregivers of children with β thalassemia major is mainly moderate to severe．The children with adverse reactions to blood transfusion and higher frequency of blood transfusion impose a heavier burden on its caregivers．The primary caregivers who have good social support can help reduce the burden．Medical staff should formulate intervention plans based on the above factors to reduce the burden level of caregivers of children with β thalassemia major．

摘要：目的：分析西双版纳傣族自治州地中海贫血髓外造血组织瘤样增生与胸部髓脂肪瘤的临床与CT影像学特征，筛选出可鉴别地中海贫血胸部髓外造血组织瘤样增生与胸部髓脂肪瘤的独立性影响因素。方法：选择2020年1月至2024年12月我院接诊的40例地中海贫血胸部髓外造血组织瘤样增生患者为病例组，选择同期就诊的40例胸部髓脂肪瘤患者为对照组进行回顾性分析。收集并比较两组患者一般资料及CT影像学特征，以多因素Logisitc回归筛选出独立性影响因素。结果：病例组与对照组性别、年龄、BMI、病灶最大径差异均无统计学意义（P>0.05），两组病灶部位、病灶数量、病灶形态、密度、强化情况差异均具有统计学意义（P<0.05）。多因素Logistic回归分析结果显示：病灶数量（OR=4.526，95%CI=1.258~16.281）、病灶形态（OR=0.310，95%CI=0.104~0.927）、密度（OR=6.704，95%CI=1.145~39.256）、强化情况（OR=4.062，95%CI=1.078~15.308）为地中海贫血髓外造血组织瘤样增生的鉴别两种疾病的独立性影响因素（P<0.05）。结论：病灶数量、病灶形态、密度等CT影像学特征可用于鉴别地中海贫血胸部髓外造血组织瘤样增生与胸部髓脂肪瘤。

目的 探讨四君寿胎汤对地中海贫血孕妇贫血状况的改善效果,以期指导地中海贫血孕妇的中药治疗。方法 选择2019年5月—2020年10月期间我院诊治的200例地中海贫血孕妇,根据随机数字表法将其分为两组,观察组与对照组,各100例,观察组患者给予四君寿胎汤,1剂/d,连续治疗3个月,对照组患者给与安慰剂,1剂/d,连续治疗3个月;治疗前、治疗3个月后,比较两组血液检测指标[血红蛋白(HGB)、红细胞(RBC)、红细胞比容(HCT)、平均红细胞体积(MCV)、平均血红蛋白含量(MCH)]、中医症候积分、肝肾指标[谷丙转氨酶(ALT)、谷草转氨酶(AST)、肌酐(Cre)、尿素(ure)、总胆汁酸(TBA)],记录两组孕妇妊娠结局、新生儿情况并比较。结果 治疗前,两组HGB、RBC、HCT、MCV、MCH比较,差异无统计学意义(P＞0.05);治疗3个月后,两组均升高,且观察组高于对照组(P＜0.05);治疗前,两组食少纳呆、体倦乏力、食后或午后腹胀、大便异常症候积分比较,差异无统计学意义(P＞0.05);治疗结束后,两组症候积分均较治疗前降低,且观察组低于对照组,差异有统计学意义(P＜0.05);治疗前后,两组ALT、AST、TBA、Cre、Ure差异无统计学意义(P＞0.05)。两组胎儿宫内窘迫、宫内生长受限发生率、产妇产后出血率比较,观察组较对照组发生率低,但差异无统计学意义(P＞0.05)。观察组早产发生率较对照组明显下降,差异有统计学意义(P＜0.05);观察组孕妇分娩孕周大于对照组,剖宫产率低于对照组,差异有统计学意义(P＜0.05);两组新生儿窒息率、转PICU率比较,差异无统计学意义(P＞0.05);观察组新生儿出生体重、HGB高于对照组(P＜0.05)。结论 四君寿胎可以改善地中海贫血孕妇的整体贫血状况,对肝肾功能无不良影响,中医证候得到改善,且有利于减少早产发生风险,降低剖宫产率,改善新生儿情况。

Objective To investigate effect of Sijun Shoutai decoction in improving anemia status of pregnant women with thalassemia, and to guide the Chinese medicine treatment of thalassemia in pregnant women in the future. Methods Two hundred pregnant women with thalassemia who were diagnosed in the hospital from May 2019 to October 2020 were divided into observation group and control group randomly, with 100 cases in each group. The observation group was treated with Sijun Shoutai decoction,1 dose/d, with continuous treatment for 3 months. The control group was given placebo,1 dose/d, with continuous treatment for 3 months. The indicators of blood test [hemoglobin (HGB), red blood cell (RBC), hematocrit value (HCT), mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH)], TCM symptom scores, hepatic and renal indicators [alanine aminotransferase (ALT), aspartate aminotransferase (AST), creatinine (Cre), urea (Ure), total bile acid (TBA)] were compared between the two groups before and after treatment for 3 months. The maternal pregnancy outcome and neonatal condition in the two groups were recorded and compared. Results There were no statistical difference in the HGB, RBC, HCT, MCV and MCH between the two groups before treatment (P>0.05); after 3 months the indicators above of two groups increased, and those in observation group were higher than those in control group (P<0.05). There were no statistical differences in the symptom scores of poor appetite, fatigue, abdominal distension after eating or after noon and fecal abnormalities between the two groups before treatment (P<0.05). The levels of ALT, AST and TBA in the two groups decreased after treatment for 3 months, while the Cre and Ure increased, but the differences were not statistically significant (P>0.05). There were no statistical differences in the rates of intrauterine fetal distress, intrauterine growth restriction, and postpartum hemorrhage between the two groups (P>0.05), though the results of observation group were lower than control group.The rate of premature birth was lower than that of control group(P<0.05) ; the gestational week of observation group was more than control group, and the cesarean section rate of observation group was lower than that of control group (P<0.05). There were no statistical differences in the rates of neonatal asphyxia and transfering to PICU between the two groups (P>0.05); the neonatal birth weight and HGB in observation group were higher than those in control group (P<0.05). Conclusion Sijun Shoutai decoction in the treatment of pregnant women with thalassemia can improve anemia status, with no adverse effect on liver or kidney function, improve TCM syndrome, reduce the risk of premature birth,reduce the rate of cesarean,and improve the neonatal condition.

目的 探讨Sysmex-2100全自动血液分析仪检测β-地中海贫血(β-地贫)患儿血小板参数显示不全的原因。方法 收集2017年4月—2020年4月期间本院确诊β-地贫患儿301人,其中重型病例204例,非重型97例。对301例患儿均采用希森美康Sysmex-2100血细胞分析仪进行血细胞分析,标本制作血涂片充分干燥后经瑞氏-吉姆萨染色,由主管技师及以上检验人员进行人工镜检。结果 301例β-地贫患儿血细胞分析结果出现血小板参数不显示者102例,占33.89％;其中有小红细胞、红细胞碎片、血小板聚集97例(95.10％);血小板直方图异常93例(91.18％)。重型β-地贫患儿血小板参数显示不全者75例,非重型β-地贫患儿血小板参数显示不全者27例,两组间血小板参数显示不全比例差异无统计学意义(P= 0.126)。血小板参数全显示病例的平均红细胞体积高于血小板参数显示不全病例,差异有统计学意义(P< 0.0001)。结论 小细胞低色素性红细胞及红细胞碎片化是β-地贫患儿血小板参数显示不全的主要原因,通过血小板直方图正常与否可大致判断血小板参数的可信度,采用PLT-O通道检测及涂片镜检查找原因,可提高结果的准确性。