Bone marrow transplantation for patients with Philadelphia chromosome- positive acute lymphoblastic leukemia

We report the treatment outcome of allogeneic bone marrow transplantation in ten patients with Philadelphia chromosome-positive acute lymphoblastic leukemia. Six patients are alive and well for 6 to 30 months (median 19 months) after transplantation. Four patients died with transplant related complications. In view of the poor prognosis associated with this disease, marrow ablation followed by allogeneic or syngeneic marrow grafting may be the preferred treatment modality if a suitable marrow donor is available.     

Association between pancreatic cystadenocarcinoma, malignant liver cysts, and polycystic disease of the kidney

Polycystic kidney disease is an autosomal dominant disease that may be associated with cystic disease of the liver. In women, the cysts may develop early and be more troublesome than in men. Cystadenocarcinoma of the pancreas is uncommon, comprising 1% of primary pancreatic malignancies. This case report is the first to describe a familial association between polycystic kidney disease and cystadenocarcinoma of the pancreas and liver in the English medical literature. A patient with autosomal dominant polycystic kidney disease (ADPKD) and multiple hepatic cysts developed cystadenocarcinoma of the pancreas with multiple malignant liver cysts. The patient's mother, sister, and niece had ADPKD, and the patient's sister also died of pancreatic cystadenocarcinoma. We believe that the development of these two disease entities in which the primary pathology is cyst formation has a genetic association. (Gastroenterology 1997 Jun;112(6):2104-7)     

甘露聚糖、壳聚糖、α-酸性糖蛋白和姜黄素对脂蛋白(a)和去唾液酸脂蛋白(a)代谢影响的对比分析

分析多糖和姜黄素对脂蛋白 (a)和去唾液酸脂蛋白 (a)代谢的影响 ,从刺猬腋下静脉注入甘露聚糖、壳聚糖、α -酸性糖蛋白和姜黄素 ,2min后注射12 5I-脂蛋白 (a)或12 5I-去唾液酸脂蛋白 (a) ,1h后处死动物 ,测定血、肝、肾、脾、胆汁和肾上腺的同位素含量。结果发现 ,脂蛋白 (a)去唾液酸后能大量进入肝脏 ,加速在体内的分解代谢 ,使血中浓度迅速降低。α -酸性糖蛋白抑制组织对脂蛋白 (a)和去唾液酸脂蛋白 (a)的摄入 ,使血中脂蛋白 (a)和去唾液酸脂蛋白 (a)含量显著增高。壳聚糖和姜黄素增加肝脏和肾上腺对脂蛋白 (a)的摄取 ,使血中脂蛋白 (a)含量略降低 ,但对去唾液酸脂蛋白 (a)代谢无明显影响。甘露聚糖增加脾脏对脂蛋白 (a)的摄取 ,减少胆囊中脂蛋白 (a)含量 ,但增加肾脏和胆囊对去唾液酸脂蛋白 (a)的摄取 ,降低肾上腺对去唾液酸脂蛋白 (a)的摄取。结果提示 ,脂蛋白 (a)去唾液酸后能使脂蛋白 (a)分解代谢加快 ,脂蛋白 (a)分子中的唾液酸在结构稳定中起重要的作用。α -酸性糖蛋白抑制脂蛋白 (a)和去唾液酸脂蛋白 (a)代谢 ,而壳聚糖和姜黄素则促进脂蛋白 (a)代谢     

Intraoperative radiotherapy (IORT) at the Graz University Clinics: development and dosimetry of an applicator system

The use of high energy electron beams for intraoperative radiotherapy necessitates special technical adjustments of the conventional megavolt equipment. At the University Clinic of Radiology, Division of Radiotherapy in Graz, an applicator system for electron energies up to 20 MeV and cone diameters up to 14 cm was developed. A self modified commercial operating table was used instead of the treatment couch to guarantee optimum mobility for the docking maneuver. The technical set-up of this adaptor system as well as the dosimetric considerations and calculations will be presented in detail.     

Extended long-term culture reveals a highly quiescent and primitive human hematopoietic progenitor population

Long-term culture-initiating cells (LTC-IC) are hematopoietic progenitors able to generate colony-forming unit-cells (CFU) after 5 to 8 weeks (35 to 60 days) of culture on bone marrow (BM) stroma and represent the most primitive progenitors currently detectable in vitro. We have recently reported that long-term cultures initiated with CD34+CD38- cells from BM or cord blood are able to continue generating CFU for at least 100 days, ie, beyond the standard LTC-IC period. In this report, single-cell cultures from cord blood and retroviral marking of cord blood and BM were used to study whether the subpopulation of CD34+CD38- cells able to generate CFU beyond 60 days ("extended long-term culture-initiating cells" or ELTC-IC) are functionally distinct from LTC-IC in terms of timing of initial clonal proliferation and generative capacity. All cord blood LTC-IC formed clones of greater than 50 cells by day 30. In contrast, cord blood ELTC- IC proliferated later in culture, 50% forming clones after day 30. Although efficient retroviral marking of LTC-IC was seen (25% to 45%), marking of ELTC-IC was inefficient (< 1%), consistent with a more quiescent progenitor population. There was a positive correlation between time of clonal proliferation and generative capacity. ELTC-IC generated threefold to fourfold more progeny than did LTC-IC (P < .002). These studies show that there is a functional hierarchy of progenitors in long-term culture which correlates with their level of quiescence. By extending the LTC-IC assay, a more primitive progenitor may be studied that may be functionally closer to the human long-term repopulation stem cell in vivo.     

二尖瓣疾病左心室充盈压的多普勒评估

由于在瓣膜区域、左心室松弛性和僵直性等方面易于发生混淆．传统多普勒测量对二尖瓣疾病病人左心房压的预测上受到限制。然而，在犬和临床研究中，组织多普勒成像所测定的充盈早期二尖瓣血流速率起始段和房室环的充盈早期速率之间的时间窗（time interval between the onset of early diastolic mitral inflow velocity and annular early diastolic velocity，TE-Ea）与左心室舒张时间常数（time constant of left ventricular relaxation，t）良好相关，并且不受上述瓣膜区域、左心室舒张和僵直等变量的影响。因此在病人人群中开展这项研究，检验其用途。