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．

目的 探讨不同贫血类型患者铁蛋白水平、甲状腺功能检测结果对比分析。方法 前瞻性选取2018年11月—2021年11月我院收治的240例贫血患者作为研究对象。将患者分为小细胞低色素性贫血组（n=75）,正细胞性贫血组（n=100）和大细胞性贫血组（n=65）。检查患者甲状腺功能[甲状腺素（TSH）、游离三碘甲腺原氨酸（FT₃）、游离甲状腺素（FT₄）]和铁蛋白（SF）表达水平。采用Spearman检验进行相关性分析;采用Logistics回归模型进行回归分析。结果 3组患者红细胞（RBC）、血红蛋白（Hb）、平均红细胞容积（MCV）、红细胞平均血红蛋白浓度（MCHC）、平均红细胞血红蛋白含量（MCH）、血清肌酐（Scr）间存在差异（P<0.05）;大细胞性贫血组TSH、FT₃和SF低于正细胞性贫血组和小细胞低色素性贫血组（P<0.05）,而FT₄高于正细胞性贫血组和小细胞低色素性贫血组（P<0.05）;贫血类型与TSH、FT₄、FT₃和SF呈正相关（P<0.05）;多因素Logistics回归分析结果显示,TSH、FT₄、FT₃和SF在3个模型中均为独立危险因素（P<0.05）。结论 不同贫血类型患者间甲状腺功能和铁蛋白表达水平存在显著差异,大细胞性贫血组TSH、FT₃和SF低于正细胞性贫血组和小细胞低色素性贫血组,而FT₄更高,甲状腺功能指标和铁蛋白均是各种类型贫血发生的危险因素。

Objective To explore the comparative analysis of ferritin level and thyroid function detection results in patients with different types of anemia．Methods A total of 240 anemia patients admitted to our hospital from November 2018 to November 2021 were prospectively selected as research objects．The patients were divided into microcytic hypochromic anemia group（n=75）,normocytic anemia group（n=100）,and macrocytic anemia group（n=65）．The expression levels of thyroid function[thyroxine（TSH）,free triiodothyronine（FT₃）,free thyroxine（FT₄）]and ferritin（SF）were examined．Correlation analysis was performed by Sperman test．The logistic regression model was adopted for regression analysis．Results There were significant differences in red blood cell,hemoglobin,mean corpuscular volume,mean corpuscular hemoglobin concentration,mean corpuscular hemoglobin content and serum creatinine among three groups（P<0.05）．TSH,FT₃ and SF in macrocytic anemia group were significantly lower than those in normocytic anemia group and microcytic hypochromic anemia group（P<0.05）,while FT₄ was significantly higher than that in normocytic anemia group and microcytic hypochromic anemia group（P<0.05）．The type of anemia was positively correlated with TSH,FT₄,FT₃ and SF（P<0.05）．The results of multi-factor logistics regression analysis showed that TSH,FT₄,FT₃ and SF were independent risk factors in the three models（P<0.05）．Conclusions There were significant differences in thyroid function and ferritin expression levels among patients with different types of anemia．Macrocytic anemia group TSH,FT₃ and SF were lower than those in normocytic anemia group and microcytic hypochromic anemia group,while FT₄ was higher．Both thyroid function indexes and ferritin were risk factors for various types of anemia．

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。

目的 探讨儿童贫血与血红蛋白等因素的相关性。方法 选取2017年7月——2019年5月于天津市河东区6月龄至5岁以下的儿童作为研究对象参与血红蛋白的检测,剔除重复值及缺失值后,共纳入453例儿童,根据儿童血红蛋白水平将儿童分为贫血组及健康组,对儿童家属进行相关问卷调查,通过Logistic回归分析探讨儿童贫血的影响因素。结果 该社区453例儿童中贫血儿童116例,贫血检出率为25.61%,Hb含量为(125.34±13.75)g/L。贫血组及健康组儿童月龄、主要照顾者、照顾者对喂养知识的知晓率、出生6个月后的喂养方式及添加辅食年龄比较差异具有统计学意义(P＜0.05)。多因素分析结果显示:6~＜12个月、照顾者对喂养知识的知晓＜60%、出生6个月后混合喂养、4~6个月添加辅食均为影响儿童发生贫血的危险因素(OR=2.78、3.82、3.90、3.50)。结论 天津市河东区儿童的贫血情况较为严重,政府相关机构应制定相关的营养改善政策,加大有关儿童正确喂养知识的宣传,同时,医疗机构应对贫血儿童做好相应的健康检查,完善父母的公共卫生教育工作。

目的 分析河源市学龄前儿童发生小细胞低色素性贫血的病因。方法 对我院进行健康体检小细胞低色素性贫血儿童287例进行血常规、血清铁蛋白及地中海贫血基因检测。结果 在所研究的287 例小细胞低色素性贫血儿童病例中,分别检出 α地中海贫血 127例,β地中海贫血 48例,α/β复合地中海贫血2例;铁缺乏 83例 (合并地中海贫血20例, 缺铁性贫血45例),不明原因贫血47例。地中海贫血检出率为61.67%,铁缺乏检出率为21.95% 。结论 地中海贫血是河源市学龄前儿童发生小细胞低色素性贫血最主要的原因,其次是铁缺乏,各年龄段儿童以轻度贫血为主,6月~1岁,1~3岁为铁缺乏高发年龄。α地中海贫血基因型以--^SEA/αα最常见,β地中海贫血以βIVS-II-654/β^N最常见,小细胞低色素症在静止型最常见。

Objective To analyze the causes of microcytic hypochromic anemia in preschool children in Heyuan City. Methods A total of 287 cases with microcytic hypochromic anemia were selected in our hospital. The indexes of hematology, serum ferritin were detected and genetic testing for thalassemia was performed. Results Through genetic analysis, 127 of 287 cases of microcytic hypochromic anemia were confirmed with α-thalassaemia,48 cases with β-thalassaemia,2 cases with compound α/β-thalassaemia and 83 cases with iron deficiency (20 thalassemia cases and 45 iron deficiency anemia cases). Thalassaemia detection rate was 61.67%, iron deficiency detection rate was 21.95%. Conclusion Thalassaemia was the main reason of microcytic hypochromic anemia in preschool children in Heyuan City, followed by iron deficiency. The mild anemia was the main type among all age groups, children aged 0.5-3 had higher iron deficiency rate. The main type of α-thalassaemia was --^SEA/αα, the main type of β-thalassaemia was βIVS-II-654/β^N and the main type of microcytic hypochromic was static type.

目的 探讨多维铁口服液联合维生素AD滴剂治疗婴幼儿缺铁性贫血的疗效。方法 将首次诊断缺铁性贫血的336例婴幼儿依家长意愿分为2个治疗组(A组和B组)与1个对照组,A组予多维铁口服液联合维生素AD滴剂治疗,B组予多维铁口服液联合维生素D滴剂治疗,对照组饮食调理并补充维生素AD,于治疗4周后(3天内)采末梢血行血细胞分析,比较三组患儿贫血治疗显效率、总有效率差异。结果 治疗4周后,A组显效率为71.70%,总有效率为97.17%;B组显效率为41.05%,总有效率为90.30%;对照组显效率为6.25%,总有效率为32.29%。A组贫血治疗显效率、总有效率高于B组和对照组,差异均有统计学意义(P<0.05)。结论 铁剂联合维生素A制剂可显著提高婴幼儿缺铁性贫血的治疗效果。

Objective To observe the curative effect of multivitamin iron oral solution combined with vitamin AD drops on the treatment of iron deficiency anemia in infants. Methods 336 cases of infants who was found with iron deficiency anemia for the first time were divided into two treatment groups (group A and group B) and one control group in accordance with their parents' will. Group A was treated with multivitamin iron oral solution combined with vitamin AD drops. Group B was treated with multivitamin iron oral solution combined with vitamin D drops. The control group was taken just dietary therapy combined with vitamin AD drops. All subjects were tested blood analysis after 4 weeks of treatment (within 3 days). The differences of the treatment excellence rates and the total effective rates were compared in the three groups. Results After 4 weeks of treatment, the excellence rate in group A was 71.70%. The total effective rate in group A was 97.17%. The excellence rate in group B was 41.05%. The total effective rate in group B was 90.30%. The excellence rate in control group was 6.25%. The total effective rate in control group was 32.29%. The differences of the treatment excellence rates and the total effective rates in group A were significantly higher than those in group B or in the control group. The differences were statistically significant (P<0.05). Conclusion Multivitamin iron combined with vitamin A can significantly improve the curative effect of the treatment of iron deficiency anemia in infants.

目的 对比分析巨幼细胞性贫血和难治性贫血患者骨髓形态的异同及其临床意义。方法 选取我院2010年4月—2016年4月收治的巨幼细胞性贫血(MA)35例,难治性贫血(RA)35例为研究对象。收集两组患者临床检验及检查资料,对比分析其临床表现、骨髓检查结果及血液检查等结果。结果 MA组患者红系病态率、粒系病态率、淋巴样小巨核率及PAS阳性率均明显低于RA组,差异明显(P<0.05);两组患者在发热、出血、消化系反应等临床表现率上相比差异不明显(P>0.05);两组患者血象检查中血红蛋白、血小板、及白细胞含量相似,两组差异不明显(P＞0.05)。结论 巨幼细胞性贫血与难治性贫血临床表现及外周血象相似,RA骨髓形态应重点观察淋巴样小巨核细胞;MA组则应强调红系巨幼变的胞体。

目的 探讨生血宁片对缺铁性贫血孕妇的治疗效果。方法 选取90个缺铁性贫血孕妇为研究对象,随机分为A、B、C三组,每组30人,从孕28周开始治疗,A组服用多糖铁复合物,B组口服生血宁片,C组采用营养科建议的补铁食疗菜谱,一直治疗至分娩前,治疗前后各检测血常规,血红蛋白(Hb)、红细胞比容(HCT)、红细胞平均体积(MCV)、红细胞平均血红蛋白浓度(MCHC)对比治疗效果。结果 服药前各组孕妇的血Hb、RBC、HCT、MCV、MCH无统计学差异,A、B组服药后Hb、RBC、HCT、MCV、MCH均高于服药前,且结果有统计学意义(P＜0.05),C组食疗前、后Hb、RBC、HCT、MCV、MCH无统计学差异,A、B、C三组之间Hb、RBC、HCT、MCV、MCH对比结果为,A、B组之间Hb、RBC、HCT、MCV、MCH差异无统计学意义(P＞0.05),但均高于C组,差异有统计学意义(P＜0.05)。结论 生血宁对孕晚期缺铁性贫血的孕妇有治疗作用,且效果与多糖铁复合物相当。

Objective To investigate the therapeutic effect of Shengxuening tablets on iron deficiency anemia in pregnant women. Methods Selected A total of 90 pregnant women with iron deficiency anemia as the research objects, randomly divided into A, B, C three groups. Each group of 30 people was treated since 28 weeks of pregnancy. Group A was treated with polysaccharide iron complex, group B with Shengxuening tablets, group C with the nutritionist recommended iron diet recipes. They have been treated till delivery. Before and after treatment, blood routine examination, the value of Hb,RBC,HCT,MCV,MCH had contrast treatment. Results There was no significant difference of serum Hb,RBC,HCT,MCV,MCH in the pregnant women before treatment. After treatment, Hb,RBC,HCT,MCV,MCH of group A, B were higher than before, and the results were statistically significant(P＜0.05). Before therapy of group C, there was no significant difference, in the three groups, results of Hb,RBC,HCT,MCV,MCH, there was no statistical difference between group A, B (P＞0.05), but both were higher than group C, the difference was statistically significant(P＜0.05). Conclusion There was therapeutic effect of Shengxuening in late pregnant women with iron deficiency anemia, and it is equal to the effect of Polysaccharide iron complex treatment.

       目的   探讨淋巴细胞亚群在鉴别低增生性骨髓增生异常综合征（hypo-MDS）和再生障碍性贫血（AA）中的价值。方法   选取2020年7月—2023年6月在平顶山市第一人民医院治疗的80例hypo-MDS或和AA患者进行回顾性分析，其中hypo-MDS 48例、AA 32例，分析两组患者各类淋巴细胞（CD3⁺ 、CD4⁺ 、CD8⁺ 、CD4⁺ /CD8⁺ 、CD3⁺ CD57⁺ T-大颗粒淋巴细胞、CD3^- CD16/CD56⁺ 自然杀伤细胞、CD19⁺ B淋巴细胞）的差异。结果   hypo-MDS组的CD3⁺ （78.42±8.02）%与AA组的（75.65±8.44）%对比差异无统计学意义（P＞0.05）；hypo-MDS组的CD4⁺ （47.54±6.88）%、CD4⁺ /CD8⁺（2.16±0.61）%高于AA组的CD4⁺ （40.11±5.71）%、CD4⁺ /CD8⁺ （1.49±0.48）%，CD8⁺ （23.12±6.42）%低于AA组CD8⁺ （31.77±6.79）%（P＜0.05）；hypo-MDS患者CD3⁺ CD57⁺ T-大颗粒淋巴细胞（13.45±3.77）%、CD3^- CD16/CD56⁺自然杀伤细胞（12.32±3.97）%高于AA组CD3⁺ CD57⁺ T-大颗粒淋巴细胞（9.77±2.15）%、CD3^- CD16/CD56⁺ 自然杀伤细胞（8.84±2.11）%，CD19⁺ B淋巴细胞（9.75±2.08）%低于AA组（12.65±3.35）%（P＜0.05）。结论   淋巴亚群变化情况可用于AA和hypo-MDS的鉴别诊断。

       Objective  To explore the value of lymphocyte subsets in differentiation between hypoplastic myelodysplastic syndrome（hypo MDS）and aplastic anemia（AA）．Methods  A  retrospective analysis was conducted on 80 patients with hypo MDS and AA who underwent treatment in the First People’s Hospital of Pingdingshan City from July 2020 to June 2023．Among them，there were 48 cases of hypo MDS and 32 cases of AA．The differences in lymphocytes（CD3⁺ ，CD4⁺ ，CD8⁺ ，CD4⁺ /CD8⁺ ，CD3⁺ CD57⁺  T-large granular lymphocytes，CD3^- CD16/CD56⁺  natural killer cells，and CD19⁺  B lymphocytes）between the two groups of patients were analyzed．Results  There was no statistically significant difference in CD3⁺ （78.42±8.02）% between the hypo MDS group and the AA group（75.65±8.44）%（P＞0.05）．The CD4⁺ （47.54±6.88）% and CD4⁺ /CD8⁺ （2.16±0.61）% in the hypo MDS group were higher than those in the AA group（40.11±5.71）% and （1.49±0.48）%，respectively．The CD8⁺（23.12±6.42）% was lower than that in the AA group（31.77±6.79）%（P＜0.05）．The levels of CD3⁺ CD57⁺  T-large granular lymphocytes（13.45±3.77）% and CD3^- CD16/CD56⁺  natural killer cells（12.32±3.97）% in hypo MDS patients were higher than those in the AA group（［9.77±2.15］%，［8.84±2.11］%），and CD19⁺  B lymphoid cells（9.75±2.08）% were lower than that in the AA group（［12.65±3.35］%，P＜0.05）．Conclusions  The changes in lymphatic subpopulations can be used for the differential diagnosis of AA or hypo MDS．