不良角化

We did not identify any of the classic features of dyskeratosis congenita in five of the six families.

在6 个家庭中，有5 个家庭未发现先天性角化不良的任何典型特征。

We did not identify any of the classic features of dyskeratosis congenita in five of the six families.

在这6个家族成员中有5个我们并没有证实有任何典型的先天性角化不良特征。

Dyskeratosis congenital is a rare and fatal congenital syndrome characterized by the triad of reticular skin pigmentation, n...

先天性角化不良(Dyskeratosis Congenita，DKC)是一种少见的以皮肤病变为主并累及多器官系统的先天性综合征。

Results There were 52 cases (63%) of LA and 30 cases (37%) of MA, among which 49 cases (60%) presented with pyknotic nucleus of the basal keratinocytes above the amyloid in the upper dermis and 18 cases (22%) presented with pagetoid dyskeratosis cells among their prickle cells. More amyloid in LA and more severe incontinent of pigment in MA.

结果 82例患者中，52例(63%)为LA，30例(37%)为MA；有49例(60%)在淀粉样蛋白沉积上方基底细胞内出现核固缩现象；18例(22%)棘细胞层出现帕杰样角化不良细胞。

It has been proved that TR mutation was related to dyskeratosis congenita, and TR could function as the template in telomere DNA synthesis.

并且对细胞分裂等细胞学事件必不可少，已证明TR 突变与先天性角化不良等疾病相关。

Dyskeratosis congenita is a rare inherited syndrome exhibiting marked clinical and genetic heterogeneity. It is characterised by mucocutaneous abnormalities, bone marrow failure and a predisposition to cancer.

先天性角化不良是一种罕见的遗传综合征，存在显著的临床及遗传异质性，以粘膜皮肤异常，骨髓衰竭及癌症易感性为特征。

Focal acantholytic dyskeratosis with corps rounds and grains were the usual histopathological findings.

局限性棘层松解性角化不良及其产生的圆体和谷粒是最常见的组织病理学改变。

Loss-of-function mutations in telomerase complex genes can cause bone marrow failure, dyskeratosis congenita, and acquired aplastic anemia, both diseases that predispose to acute myeloid leukemia.

端粒末端转移酶的复杂基因丧失功能性突变可导致骨髓衰竭、先天性角化不良及获得性再生障碍性贫血，这些疾病都可以诱发急性髓样白血病。

darier's disease is a rare dermatosis with an autosomal dominant mode of inheritance.greasy papules and plaques arise on the seborrheic areas and in the flexures.acantholysis and dyskeratosis are the typical histological findings.

darier病是一种少见的常染色体显性遗传性皮肤病。常在皮脂溢出部位和屈侧皮肤见油腻性丘疹和斑块。棘层松解和角化不良是典型的病理改变。

Germ-line mutations in the genes hTERT and hTR, encoding telomerase reverse transcriptase and telomerase RNA, respectively, cause autosomal dominant dyskeratosis congenita, a rare hereditary disorder associated with premature death from aplastic anemia and pulmonary fibrosis.

端粒酶逆转录酶的编码基因hTERT和端粒酶RNA的编码基因hTR发生种系突变时，可引起常染色体显性先天性角化不良，这是一种罕见的遗传性疾病，它与再生障碍性贫血和肺纤维变性所致的早产儿死亡具有相关性。

On the other hand, the more important thing is because the urban housing is a kind of heterogeneity products.

另一方面，更重要的是由于城市住房是一种异质性产品。

Climate histogram is the fall that collects place measure calm value, cent serves as cross axle for a few equal interval, the area that the frequency that the value appears according to place is accumulated and becomes will be determined inside each interval, discharge the graph that rise with post, also be called histogram.

气候直方图是将所收集的降水量测定值，分为几个相等的区间作为横轴，并将各区间内所测定值依所出现的次数累积而成的面积，用柱子排起来的图形，也叫做柱状图。