大鼠CAG证病结合模型的宏观症征观察

目的:观察大鼠慢性萎缩性胃炎(CAG)证病结合模型宏观症征的改变.方法:Wistar雌性大鼠,随机分为对照组、CAG组、脾虚CAG组、肝郁CAG组和肾虚CAG组.CAG造模采用脱氧胆酸钠和阿斯匹林水溶液交替饮用加免疫损伤法,脾虚造模采用耗气破气加饥饱失常法,肝郁造模采用钳夹激怒加肾上腺素注射法,肾虚造模采用MTU饮用法.总造模时间35～51周.结果:各CAG证病结合模型在症状、舌象、体重、身长、尾长、Lees指数、腹膜后脂肪湿重和指数、体温、食量和饮水量、口腔pH值、胃液pH值等方面出现与衰老、虚证、肝郁、发育、代谢、胃粘膜萎缩等相关的变化.结论:本实验重视慢性造模的证病结合模型宏观症征的观察,具有一定意义.     

Aclarubicin and Low-Dose Cytosine Arabinoside in Combination with Granulocyte Colony-Stimulating Factor in Treating Acute Myeloid Leukemia Patients with Relapsed or Refractory Disease and Myelodysplastic Syndrome: A Multicenter Study of 112 Chinese Patients

One hundred twelve patients with geriatric acute myeloid leukemia (AML), refractory or relapsed AML, or myelodysplastic syndrome and refractory anemia with excess of blasts in transformation (MDS-RAEBt) were entered into this study to receive CAG (aclarubicin and low-dose cytosine arabinoside [Ara-C]in combination with granulocyte colony-stimulating factor [G-CSF]) with the objective of evaluating the efficacy and tolerance of this regimen. Low-dose Ara-C was given subcutaneously at a dosage of 10 mg/m2 every 12 hours on days 1 to 14. Aclarubicin was administered intravenously at a dosage of 14 mg/m2 per day on days 1 to 4 (CAG regimen A) or 7 mg/m2 on days 1 to 8 (CAG regimen B). Recombinant G-CSF was given subcutaneously at a dosage of 200 3g/m2 per day on days 1 to 14. We demonstrated comparable overall complete remission rates for the 4 groups of patients: 30.8% (8/26) in the elderly patients, 48.4% (30/62) in the refractory AML patients, 44.4% (8/18) in the relapsed AML patients, and 38.5% (5/13) in the MDS-RAEBt patients. Of the 52 patients followed up, the 12-month progression-free survival (PFS) and overall survival (OS) rates estimated by the Kaplan-Meier method were 40.73% 3 8.15% and 42.85% 3 8.23%, respectively. The median PFS and OS times were 9.0 3 2.2 months and 11.0 3 1.6 months, respectively. Toxic effects were very rare and mainly consisted of neutropenia and thrombocytopenia due to myelosuppression; approximately 70% to 80% of patients had neutropenia or thrombocytopenia that exceeded National Cancer Institute grade II. Nonhematologic toxicities were not observed in this study.The CAG regimen seems promising, with acceptable toxicity, for the treatment of various categories of poor-prognosis AML and MDS-RAEBt.     

CAG预激方案联合伊马替尼治疗复发/难治性Ph+急性淋巴细胞白血病首例报道

目的:寻找有效治疗复发/难治性Ph染色体阳性急性淋巴细胞白血病(Ph ALL)的疗法。方法:以CAG预激方案联合伊马替尼诱导治疗复发/难治性Ph ALL病例1例。结果:该复发/难治Ph ALL患者经CAG方案联合伊马替尼诱导治疗后达缓解。结论:对于复发/难治性Ph ALL,CAG联合伊马替尼是一个值得尝试的方案。     

CAG方案治疗复发性急性髓细胞白血病疗效观察

目的:探讨CAG方案对复发性急性髓细胞白血病(AML)的临床疗效和不良反应.方法:选择在我院治疗的复发AML 46例,复发后20例选用CAG方案:阿克拉霉素(ACR)10～14 mg·m-2·d-1 (第1～4天,10～14天),静脉滴注;阿糖胞苷(Ara-C)10 mg·m-2·d-1 q12h(第1～14天),皮下注射;粒细胞集落刺激因子(G-CSF)200 μg·m-2·d-1(第1～14天),皮下注射.26例选用对照方案:①DAH:柔红霉素40 mg·m-2·d-1(第1～3天);Ara-c 200 mg·m-2·d-1(第1～7天),高三尖杉酯碱(HHT)3～4 mg·m-2·d-1(第1～7天);②MAE:米托蒽醌(Mito)10 mg·m-2·d-1(第1～3天),Ara-c 200 mg·m-2·d-1(第1～7天),依托泊甙(VP-16)60 mg·m-2·d-1(第1～5天).结果:强化疗组26例,经上述化疗1个疗程后6例获得完全缓解(CR),部分缓解(PR)10例,未缓解(NR)10例.PR和NR 20例患者中6例一般情况差,未能继续强化疗,换用CAG方案;14例予第2个疗强化疗,CR 4例,NR 10例;2疗程CR共10例(38.4%).26例CAG组第1个疗程CR 12例,PR 10例,NR 4例.PR和NR 14例予以CAG第2个疗程治疗,CR 8例,PR 2例,NR 4例,2疗程CR共18例(69.2%).2组CR率差异有统计学意义(P<0.05).CAG组骨髓抑制不明显,不良反应也低.结论:复发AML治疗中,CAG方案是较对照组方案更为有效的且不良应低的治疗方法.     

CAG repeat length in androgen receptor gene and male infertility in Egyptian patients

The CAG repeat and its association with infertility has been debatable. Therefore, this study was planned to assess the distribution of CAG repeat expansion in Egyptian patients and to investigate its association with male infertility. Forty-five infertile men were eligible for the study in addition to 20 aged-matched fertile males as control. Semen analysis, scrotal sonography, assay of serum testosterone, follicle-stimulating hormone (FSH) and luteinising hormone (LH), and determination of the CAG repeat number within exon 1 of the androgen receptor (AR) gene were carried out. Statistically significant difference was found between infertile and control groups regarding sperm count, sperm motility, serum FSH level and CAG repeats (P < 0.05); statistically insignificant difference for the CAG repeats (P = 1.0) was found between oligozoospermic and asthenospermic groups; negative correlation was found between CAG repeat length and sperm count, and a positive correlation was found between CAG repeat length and serum FSH (P < 0.05). Our results validate the concept that long stretches of CAG repeat may be associated with lower AR function with derangement of sperm production, and this may contribute to male infertility in Egyptian men.     

地西他滨联合改良CAG及单倍体相合外周血淋巴细胞回输治疗老年高危恶性血液病

本研究旨在观察地西他滨联合改良CAG及单倍体相合外周血淋巴细胞回输免疫治疗新方案,作为初治老年高危骨髓增生异常综合征（MDS）和急性髓系白血病（AML）的诱导缓解方案的初步疗效及其不良反应。对2012年4月至2012年7月在本院血液科应用地西他滨联合改良CAG及HLA半相合外周血淋巴细胞回输免疫治疗新方案治疗的5例老年高危MDS和AML患者进行前瞻性研究,观察完全缓解率及副反应。结果表明：5例初治老年患者治疗总有效率100%,4例达到完全缓解,1例患者达到部分缓解。既往无MDS病史患者,中性粒细胞数恢复至0.5×109/L的中位时间为15 d,血小板数恢复至20×109/L的中位时间为16 d。主要副作用为IV度骨髓抑制,全部患者治疗中无新发肺部感染等严重并发症。结论：地西他滨联合改良CAG及外周血淋巴细胞回输免疫治疗新方案,治疗老年MDS和AML患者安全有效,值得进一步研究。     

CAG repeat expansions in patients with sporadic cerebellar ataxia

CAG repeat expansions cause spinocerebellar ataxia type 1 (SCA1), SCA2, SCA3, SCA6 and dentatorubral-pallidoluysian atrophy (DRPLA). So far these expansions have been examined mainly in ataxia patients with a family history. However, some sporadic cases with SCA have recently been reported. To elucidate the frequency and characteristics of sporadic SCAs, we screened 85 Japanese ataxia patients without a family history for the SCA1, SCA2, SCA3, SCA6 and DRPLA mutations. As a result, 19 patients (22%) were found to have expanded CAG repeats. Among sporadic SCAs, the SCA6 mutation was most frequently observed. The sporadic SCA6 patients had smaller CAG repeats and a later age of onset than SCA6 patients with an established family history. We also identified one father-child pair in which intermediate sized CAG repeats expanded into the SCA2 disease range during transmission. These findings suggest that patients with ataxia even without a family history should be examined for a CAG repeat expansion.     

冠状动脉造影正常的急性心肌梗死患者的临床特征

目的：对比观察急性心肌梗死(AMI)冠状动脉(冠脉)冠脉造影正常和异常患者临床及血管造影特点。方法：回顾分析1997年6月～2002年12月318例AMI患者的临床特征及血管造影结果。结果：318例AMI患者中冠脉正常者26例占8．2％，多为男性，年龄较轻，发病前有大量吸烟、高度紧张及劳累等明确诱因，多无心绞痛史、高血压病史、糖尿病史及血脂异常者史。结论：AMI可发生于冠脉正常的患者中，多为年龄较轻男性，一般都有明确的吸烟等诱因．冠脉痉挛和急性血栓形成伴自溶可能是其主要发病机制。     

A Japanese family with Machado-Joseph disease characterized by initial emaciation and myoclonus

We describe a Japanese family with hereditary spinocerebellar ataxia characterized by initial emaciation and myoclonus. The proband first noted truncal ataxia, myoclonus in the shoulder and general emaciation at age 24. The other affected members of the family also had such emaciation in the early stage of the disease. The DNA analyses of the family revealed that the patients of the family are associated with the expansions of CAG repeats for Machado–Joseph disease (MJD) on the long arm of chromosome 14. Although the clinical features of MJD are very variable, general emaciation in an early stage of the disease and systemic myoclonus have not been documented. Because it is sometimes difficult to distinguish among hereditary spinocerebellar ataxias such as spinocerebellar ataxia type 1 (SCA1) or dentatorubropallidoluysian atrophy (DRPLA) by clinical features, a genetic examination provides better understanding of such a rare and ambiguous type of hereditary spinocerebellar ataxia.