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胚细胞瘤

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Wilms" tumor is one of the most common solid tumors in children, and the genesis of Wilms" tumor is believed to be associated with delayed differentiation of metanephric blastema in fetus and retaining proliferation in postnatal period.

肾母细胞瘤(Wilms tumor, WT)是儿童最常见的实体瘤之一,其发病与后肾胚基细胞分化受阻并在出生后继续增殖有关。

The other cancers with shorter follow up had three years overall and event free survival as follow: 75% and 62% for Medulloblastoma, 74% and 67% for Non-Hodgkin Lymphoma, 100% and 91% for extracranial Germ Cell Tumour, 30% and 20% for advanced neuroblastoma, 68% and 52% for Ewing's Sarcoma, 87% and 79% for Wilms' Tumour, 82% and 80% for Osteosarcoma, 56% and 45% for Rhabdomyosarcoma, and 60% and 50% for Hepatoblastoma respectively.

其他癌症经三年的短期随访,总存活率及无并发症存活率分别是:成神经管细胞瘤为 75%及 62%;非何杰金氏淋巴瘤为 74%及 67%;颅外的胚细胞瘤为 100%及 91%;已播散成神经细胞瘤为 30%及 20%;尤因氏肉瘤为 68%及 52%;威尔姆斯氏瘤为 87%及 79%;骨肉瘤为 82%及80%;横纹肌肉瘤为 56%及 45%;肝母细胞瘤为 60%及 50%。

Of the 5 patients with available follow-up, 3 were free of disease at 1, 5, and 8 years after orchiectomy (2 necrotic seminomas and 1 germ cell tumor, unclassified). One patient with yolk sac tumor (age 63 y) developed widespread metastases after 15 months and died of disease.

随访了5例患者,其中3例于睾丸肿瘤切除术后1、5和8年任健在且无复发(2例为坏死的精原细胞瘤、1例为未分类的生殖细胞肿瘤)。1例为内胚窦瘤(63岁),15个月后广泛转移,并死于本病。

It also indicates that the expression of WT1 is regulated strictly in time and space, and the aberrant expression of WT1 may result in retarded differentiation of blastemal cell leading NR or nephroblastoma.

提示在散发性肾母细胞瘤发生中除WT1突变外,WT1表达延迟可能是其中起重要作用的机制之一;WT1基因与肾母细胞瘤的组织分化有关,可能诱导胚基细胞向上皮细胞方向分化。

This result indicates that WT1 gene plays an important role in differentiation and development of fetal kidney and may be the factor that promotes metanephric blastemal cell to differentiate into epithelial cell.

结果显示小胎龄肾组织中WT1蛋白在胚基细胞和幼稚肾小球细胞核表达而大胎龄组肾组织中WT1在肾小管细胞胞浆表达,阳性率分别为57.1%(8/14)和46.2%(6/13),提示WT1基因在胚胎肾分化发育的过程中起着重要作用,WT1蛋白可能是促进后肾胚基细胞向上皮细胞分化的调控因子,其表达在时间上和空间上都受到严格的调控,WT1的表达异常可能导致胚基细胞分化停滞。17例肾母细胞瘤WT1蛋白表达阳性率为41.2%(7/17),阳性部位在胚基型和上皮型肿瘤细胞核,表达部位和阳性率与早期胚胎肾相似,其中间质型肾母细胞瘤均为阴性,胚基型和上皮型肾母细胞瘤阳性率70%(7/10),两组间阳性率有显著差异。

CCSK is a rare type of renal neoplasm in childhood and its diagnosis relies mainly on histopathology and immunohistochemistry. It is important to be familiar with the morphological variants of CCSK to avoid confusion with other similar lesions, such as Wilm's tumor, congenital mesoblastic nephroma, malignant rhabdoid tumor of kidney, primitive neuroectodermal tumor.

CCSK是一种罕见的儿童期恶性肾肿瘤,诊断主要依靠组织病理学和免疫组化,熟悉其形态学变异有利于与其它类似病变如肾母细胞瘤、先天性中胚叶肾瘤、肾恶性横纹肌样瘤、原始神经外胚叶肿瘤等鉴别。

PPO gene was not detected in the multiple myelome、 malignant fibrous histiocytoma 、malignant lymphoma、malignant neurosarcoma、solitary plasmacytoma of bone、primitive neuroectodermal tumors、lipoma、 osteosarcoma and sarcoenchondroma.

PPO基因在多发性骨髓瘤、恶性纤维组织细胞瘤、恶性淋巴瘤、恶性神经肉瘤、孤立性浆细胞肉瘤、原始神经外胚瘤、脂肪瘤、骨及软骨肉瘤等肿瘤中没有表达。在骨巨细胞瘤中有微弱表达。

This is a retrospective analysis of 194 patients with primary malignant brain tumor, who registered between January 1, 1970 and December 31, 1979 in cancer registration, Cancer Therapy Center, Veterans General Hospital, Taipei, Taiwan, R.O.C. There were 36 cases of glioblastoma multiforme, 86 cases of malignant astrocytoma (grade Ⅲ and a few grade Ⅱ), 22 of medulloblastoma, 12 of oligodendroglioma, 11 of ependymoma or ependymoblastoma and 27 cases of other types. There were 141 male cases with ages ranging from ito 81 years with a mean of 33.3±19.3 and median of 34 years.

自1970年1月至1979年12月台北荣民总医院癌病治疗中心癌病登记组共登记了194例原发恶性脑瘤其中多形神经胶母细胞瘤有36例,第二级及第三级星细胞瘤有86例,神经管胚细胞瘤有22例,间胶质母细胞瘤有12例,室管膜瘤或室管膜胚细胞瘤(ependy-moblastoma)有11例,其他有27例。

About 48%(90/194) were below 29 years of age. Ninety-nine of 194 cases had either radiotherapy alone or supplementary treatment with radiotherapy following surgery. We analyzed multiple prognostic factors. The factors of importance included postoperative radiotherapy; female in glioblastoma multiforme; the primary tumor dose above 5000 rads and/or whole brain irradiation in malignant astrocytoma and glioblastoma multiforme; the primary tumor dose above 4500 rads in ependymoma, medulloblastoma and oligodendroglioma; and spinal axis irradiation above 2000 rads in ependymoma and medulloblastoma that produced a better prognosis than in other groups.

男与女之比例为2.7比1,年龄分布自1至81岁,平均年龄33.3±19.3岁,中值年龄为34岁,29岁以下占48%(90/194),其中99例单独或手术后辅以放射腺治疗,分析同种脑瘤的预后因素,其中多形神经胶母细胞瘤有手术后放射线治疗或女性,多形神经胶母细胞瘤合并星细胞瘤原发肿瘤剂量5000雷得以上或曾全颅腔照射,神经管胚细胞瘤,间胶质母细胞瘤,及室管膜瘤原发肿瘤剂量4500雷得以上,室管膜瘤及神经管胚细胞瘤有脊轴照射2000雷得以上预后均佳,且统计上有显著的差异。

The triphasic nature of Wilms Tumor is obious here.

Wilms'瘤也称作肾母细胞瘤,是由肾胚芽组织发生的恶性混合型肿瘤,最常见于2~4岁小儿,多单侧发病。

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