有核红细胞联合Ranson评分及APACHEⅡ评分构建重症急性胰腺炎患者的结局预测模型

目的探讨重症急性胰腺炎(SAP)患者的结局预测模型。方法回顾性病例对照研究。收集温州医科大学附属台州医院2016年1月1日至2018年4月30日102例SAP患者,将入选者按照入院后90天转归情况分为生存组及死亡组,其中生存组88例,男性57例,女性31例,年龄55.5(40.3~69.8)岁;死亡组14例,男性5例,女性9例,年龄63.0(50.8~80.8)岁。通过比较2组间临床资料及实验室指标,筛选出具有统计学差异的指标,采用卡方自动交互检测法(CHAID)构建SAP患者的结局预测模型。收集2018年8月1日至2019年7月1日的50例SAP患者的临床资料及实验室指标结果对得出的结局预测模型进行验证。结果(1)通过卡方检验及Mann-Whitney U检验进行生存组及死亡组临床资料比较得出组间并发心脑血管系统疾病比例、机械通气、感染性休克比例、Charlson合并症指数(CCI)、Ranson评分及APACHEⅡ评分,死亡组均高于生存组,差异具有统计学意义(χ^2=5.554,P=0.018;χ^2=5.585,P=0.018;P=0.008,Z=-3.007,P=0.003;Z=-2.982,P=0.003;Z=-3.257,P=0.001);(2)通过卡方检验及Mann-Whitney U检验进行实验室指标比较得出生存组及死亡组间MPV、CRP、MCHC、pH、PCO2及NRBC阳性率,差异具有统计学意义(Z=-2.466,P=0.014;Z=-2.689,P=0.007;Z=-2.238,P=0.025;Z=-1.977,P=0.048;Z=-2.239,P=0.025;P=0.000),死亡组NRBC阳性率较生存组高,其余指标均较生存组低;(3)决策树CHAID法得出预测方案:当SAP患者满足Ranson评分≤3分时判定存活;当满足Ranson评分>3分且外周血中NRBC呈阴性判定存活;当满足Ranson评分>3分、NRBC呈阳性且APACHEⅡ评分≤21分时判定存活;当满足Ranson评分>3分、NRBC呈阳性且APACHEⅡ评分>21分时,判定预后不良;(4)验证组共收集50例SAP患者,实际生存43例,死亡7例,用方案预测患者结局准确率为94.0%(47/50)。结论有核红细胞联合Ranson评分及APACHEⅡ评分可预测SAP患者的结局。     

Evaluating fetomaternal hemorrhage by alphafetoprotein and Kleihauer following therapeutic abortions

In a survey of 75 patients at 6-11 weeks gestation, fetomaternal hemorrhage (FMH) was detected by significant rises (greater than 2 S.D.) in maternal alphafetoprotein (AFP) levels in 57% of patients, while increased fetal cells were detected by the Kleihauer test in 24% of patients. With increasing gestation, FMH was detected more readily by both tests; however, in evaluating FMH at less than 10 weeks gestation. AFP was found to be a more sensitive and reliable marker than the Kleihauer test. We suggest that there is a gray zone for the Kleihauer test in early gestation, when erythroblasts containing embryonic hemoglobins are gradually replaced in the fetal circulation by erythrocytes containing fetal hemoglobin.     

应用流式细胞术从孕妇外周血分选胎儿有核红细胞并进行DYS14基因扩增与检测

目的 从孕妇外周血分选胎儿有核红细胞，对DYS基因进行扩增，并对扩增准确率进行分析。方法 应用流式细胞术(flow cytometry，FCM)从孕妇外周血分选胎儿有核红细胞(Nucleated red blood cells，NRBCs)，应用套式PCR特异性扩增DYSl4基因，琼脂糖凝胶电泳检测。结果 30例受检中：共有男婴18例，有核红细胞组DYSl4基因的检出率为50．0％(9／18)。结论 NRBCs分选技术的建立使非创伤性产前基因诊断成为可能，但如何进一步改进方法提高分选率，确保诊断的灵敏性，降低漏诊率，仍是需要进一步解决和完善的问题。     

高原实验家兔骨髓各级幼红细胞费莱特直径研究

通过对移居高原实验家兔骨髓内各级红系细胞电镜照片的费莱特直径研究，发现碑红及各级幼红细胞，在低氧环境下有胞体增大趋势，与平原组比较，原红细胞和早幼红细胞变化较大，均Ｐ〈０．０１，有高度显著性意义。提示，造血细胞在急进高原后的前三周内其形态变化最大，以后又逐渐回落。     

Erythroblast Iron Metabolism in Sideroblastic and Sideropenic States

Iron appears to exert self-regulatory control over erythroblast iron uptake, iron storage and its incorporation into haem. It does this via iron regulatory proteins (IRPs) which bind reversibly to the iron responsive elements (IREs) on the mRNA of transferrin receptor (TfR), erythroid 5-aminolaevulinic acid synthase (ALA-S2) and ferritin. Iron deficiency leads to the binding of IRP to IRE. This binding inhibits the translation of mRNA for ALA-S2 and ferritin but stabilizes mRNA for TfR expression.

Sideroblastic erythropoiesis is highly ineffective and characterized by mitochondrial iron loading. The study of X-linked sideroblastic anaemia has shown that the entry of iron into the mitochondria is poorly controlled and able to occur when protoporphyrin production is reduced, as is seen with the ALA-S2 mutations, or when it is increased as has been seen with ABC7 transporter mutations.

Sideropenia characterises both iron deficiency anaemia (IDA) and the anaemia of chronic disease (ACD). Erythroblasts in ACD seem doubly equipped to protect their iron supply with their ability to increase the efficiency of transferrin-iron uptake as well as to activate the IRP/IRE system to increase surface TfR production. This increase in efficiency restricts the need to increase surface TfR production and maintains serum soluble TfR (sTfR) values within the normal range in iron replete ACD. The coexistence of iron deficiency with chronic disease, however, is associated with an increase in both the efficiency and number and a highly significant rise in sTfR values.     

Erythroblastic transformation of chronic myeloid leukemia: Hyperdiploid Ph1-positive karyotypes in primitive PAS-positive erythroblasts

Summary In a case of erythroblastic transformation of chronic myeloid leukemia, morphological, cytochemical, and cytogenetic properties of marrow cells were studied on three occasions. It was investigated whether any correlation could be demonstrated between these cytological parameters. The following observations suggested that early erythroblasts with granular periodic acid-Schiff (PAS) positivity had hyperdiploid Ph¹-positive karyotypes: (1) At the three times of study, the frequencies of Ph¹-positive, hyperdiploid karyotypes varied in phase with the frequencies of erythroblast mitoses and inversely to those of granulopoietic mitoses. (2) In the three consecutive bone marrow samples, the frequencies of early erythroblasts (E₁+E₂) presenting granular PAS positivity corresponded to and showed a statistically significant positive correlation to those of Ph¹-positive hyperdiploid metaphases.Sponsored by the Danish Cancer Society. Aided by grants from Carl Schepler and Wife's Bequest, the Irma Foundation     

Fanconi's Anemia: Ultrastructural Observations on Erythroblasts

A case of Fanconi's anemia is reported. Particular attention was given to the ultrastructure of the erythroblasts. The main abnormalities are summarized as follows: (1) striking polymorphism of all the erythroblasts, (2) multiple nuclear cisternae showing several breaks, (3) admixture of nucleus and cytoplasm, (4) myelinic figures inside nuclear cisternae and cytoplasm, (5) moth-eaten nucleus, and (6) total absence of normal erythroblasts. These abnormalities were still visible after therapy in spite of clinical and hematologic improvement.     

Functional activity of uremic erythroblasts incubated in autologous and homologous plasma

Summary The uptake of³H-thymidine,³H-uridine and³H-leucine in the erythroid precursors of patients with chronic renal failure (CRF) was examined by radioautography. The pattern of incorporation of the radioactive precursors was similar to that observed in erythroblasts of control subjects, i.e., the uptake decreased with cell maturation. CRF erythroblasts incubated with normal, homologous plasma, showed significant increase in the uptake of the radioactive precursors, compared to the activity of these cells incubated in autologous plasma, the only exception being the incorporation of³H-leucine in the proerythroblasts, in which the increase was not statistically significant. These results suggest that the impaired function of CRF erythroblasts related to DNA, RNA and protein synthesis is due not to a defective mechanism in the cells themselves, but most probably to the effect of factors present in uremic plasma, the nature of which remains to be detected.This work was partially supported by a grant from the Chief Scientist's Bureau, Ministry of Health, Israel     

双色共变性荧光原位杂交产前诊断胎儿唐氏综合征

目的 探讨双色共变性荧光原位杂交用于非侵入性产前诊断胎儿唐氏综合征的可行性。方法 对11例孕妇外周血中的胎儿有核红细胞进行抗血型糖蛋白磁珠直接标记,再经磁激活细胞分选法富集,以Y和21号染色体专一探针对分离的胎儿有核红细胞行双色共变性荧光原位杂交,预测胎儿21号染色体倍性和性别,并用羊水染色体核型分析结果,验证预测准确性。结果 11例胎儿21号染色体倍性均正常,与羊水染色体核型分析结果相符。其中5例为男性胎儿,男性胎儿有核红细胞数量为9－65个,平均为25个,男性胎儿有核红细胞纯度为1．4％－18．8％;6例为女性胎儿,孕妇外周血中未见男性胎儿有核红细胞;性别预测结果与羊水染色体型分析结果一致。结论 双色共变性荧光原位杂交用于分析胎儿21号染色体倍性及性别,诊断胎儿唐氏综合征准确、可靠。