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GS Wengler A Lanfranchi T Frusca R Verardi A Neva D Brugnoni S Giliani M Fiorini P Mella F Guandalini E Mazzolari S Pecorelli LD Notarangelo F Porta AG Ugazio 《Canadian Metallurgical Quarterly》1996,348(9040):1484-1487
BACKGROUND: X-linked severe combined immunodeficiency (SCIDXI) is an inherited immune defect which leads to death in infancy from severe infections. The defect is caused by mutations of the IL-2RG gene that encodes for the common gamma chain shared by several cytokine receptors. The disease is characterised by lack of T and NK cells with normal numbers of B cells. SCIDXI can be cured by bone marrow transplantation (BMT) or prevented by abortion after prenatal diagnosis. METHODS: A male fetus was diagnosed as having SCIDXI by molecular, immunophenotypic, and functional analyses. The fetus was injected intraperitoneally under ultrasound guidance with CD34 haematopoietic progenitor cells purified from paternal bone marrow and T-cell depleted by E rosetting. Chimerism analysis was by HLA-DQ alpha typing and gamma-chain staining on cord blood. FINDINGS: A healthy 3.6 kg boy was delivered by caesarean section at 38 weeks of gestation with no clinical or laboratory signs of graft-versus-host disease. Engraftment of donor-derived CD2 cells was found at birth. At 3.5 months of age the infant is well and his T-cell counts and function are normal. INTERPRETATION: In-utero transplantation of haematopoietic progenitor cells allowed immune reconstitution of a fetus with SCIDXI and may be an alternative to elective abortion. Our report should encourage applications of this method to other inherited disorders curable by BMT. 相似文献
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J Booij RJ Knol L Reneman K de Bruin AG Janssen EA van Royen 《Canadian Metallurgical Quarterly》1998,25(12):1666-1669
Flavobacterium aurantiacum NRRL B-184 possesses the ability to degrade aflatoxin B1 in solution and in several food items. Aflatoxin B1 is a potent carcinogen that causes significant economic losses to the agricultural and food industry. The role of trace metal ions (Cu2+, Mn2+, Zn2+, and Co2+) were studied in an effort to understand the enzymatic system involved in aflatoxin B1 degradation by F aurantiacum. The effect of divalent chelators (EDTA and 1,10-phenanthroline [OPT]) in the presence of the trace metal ions was studied as well. Aflatoxin B1 (10 microg/ml) was added to 72-h cultures of F aurantiacum that had been washed and resuspended in phosphate buffer (pH 7.0). HPLC was used to determine aflatoxin B1 concentration in these cultures. Incubating cells at 30 degrees C with 1 and 10 mM Cu2+, Mn2+, and Zn2+ significantly decreased aflatoxin B degradation after 4 and 24 h (P < 0.05). Decreased degradation was also observed with 1 and 10 mM Cu2+ and Zn2+ after 48 h and with 0.1 mM Cu2+ after 24 and 48 h. Co2+ did not have a significant effect on aflatoxin B1 degradation. EDTA and OPT did not counter the inhibition in the presence of Cu2+. The addition of 1 mM EDTA countered the inhibition by 1 mM Mn2+ after 4 and 24 h, but 1 mM OPT did not counter the inhibition by 10 mM Mn2+ after 4 and 24 h. OPT countered the inhibition by 1 mM Zn2+ after 4 and 48 h. These trace elements inhibit aflatoxin B1 degradation by F aurantiacum. In addition, their presence necessitates higher concentrations (>1 mM) of EDTA and OPT for the removal of their inhibitory effect. 相似文献
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Uwe Streller Carlos Mata Qimonda AG 《集成电路应用》2006,(9):38-41
与上一代设计规则相比,亚100nm设计规则DRAM的开发和制造面临着前所未有的挑战。按照新型设计概念,由于器件结构不断缩小,其外形特征的深.宽比增大,这样就给工艺控制增加了难度。因此要求寻找一种能够对带状缺陷和孔洞缺陷进行可靠检测的高深.宽比(HAR)检测方法。另外,由于缺陷尺寸接近小型特征尺寸,许多从前认为不那么严重的缺陷如今变得极具损害力。因而缺陷检测和良率控制技术的改良成为加速开发并实现批量生产的基础。 相似文献
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在片梭和剑杆织机上采用了一种新型的织边装置极大地提高了织造效率和减少了织造时绞边的纱线浪费。这种装置也可改进后安装在旧织机上。 相似文献
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Arterial catheterization for blood gas analysis and direct monitoring of blood pressure in infants and children can be attended by a variety of complications, which limit its use to short periods of time. An improved technique, consisting of elimination of stopcocks, the use of a continuous flushing system adapted for infants and children, and meticulous care of the system, was used in 57 children whose ages ranged from the neonatal period to 16 years. The mean duration of cannulation was 5.5 days (range, 1-31 days). All but one of the catheters were functional at the time of discontinuation. There were no thromboembolic phenomena, local or systemic infections or accidental bleeding. We feel that our approach to prolonged arterial cannulation can be an extremely valuable aid in the management of critically ill children. 相似文献
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On curettage, done in a 39 year old patient because of metorrhagia, a peanut-sized nodule was scraped away from the endometrium. Histologically it consisted of a jumble of irregularly shaped portions of normal appearing embryonal cartilage, of tortuous thick-walled vessels, of twisted bundles of nerves of ducts of respiratory and intestinal epithelium, and of sheets of hornifying skin. The haphazard disarray of tissues supported the notion that the lesion was not a remnant of a malformed embryo. Step-sections through the lesion revealed no traces of placental villi or decidua in the surrounding endometrium. The following were considered as possible origins of the teratoma: 1. displaced germinal cells, 2. retention of pluripotent müllerian epithelium, 3. overaged or blighted ovum. The latter seems most compatible with the histological findings. 相似文献