《初中数学人教版八年级上册第十四章14.2乘法公式练习题-普通用卷.docx》由会员分享,可在线阅读,更多相关《初中数学人教版八年级上册第十四章14.2乘法公式练习题-普通用卷.docx(14页珍藏版)》请在文库网上搜索。
1、.2018年国家罕见病目录一览表序号中文名称英文名称121-羟化酶缺乏症21-Hydroxylase Deficiency2白化病Albinism3Alport 综合征Alport Syndrome4肌萎缩侧索硬化Amyotrophic Lateral Sclerosis5Angelman氏症候群(天使综合征)Angelman Syndrome6精氨酸酶缺乏症Arginase Deficiency7热纳综合征(窒息性胸腔失养症)Asphyxiating Thoracic Dystrophy(Jeune Syndrome)8非典型溶血性尿毒症Atypical Hemolytic Uremic S
2、yndrome9自身免疫性脑炎Autoimmune Encephalitis10自身免疫性垂体炎Autoimmune Hypophysitis11自身免疫性胰岛素受体病Autoimmune Insulin Receptopathy(Type B insulin resistance)12-酮硫解酶缺乏症Beta-ketothiolase Deficiency13生物素酶缺乏症Biotinidase Deficiency14心脏离子通道病Cardic Ion Channelopathies15原发性肉碱缺乏症Carnitine Deficiency16Castleman病Castleman Di
3、sease17腓骨肌萎缩症Charcot-Marie-Tooth Disease18瓜氨酸血症Citrullinemia19先天性肾上腺发育不良Congenital Adrenal Hypoplasia20先天性高胰岛素性低血糖血症Congenital Hyperinsulinemic Hypoglycemia21先天性肌无力综合征Congenital Myasthenic Syndrome22先天性肌强直(非营养不良性肌强直综合征)Congenital Myotonia Syndrome (Non-Dystrophic Myotonia, NDM)23先天性脊柱侧弯Congenital Sc
4、oliosis24冠状动脉扩张病Coronary Artery Ectasia25先天性纯红细胞再生障碍性贫血Diamond-Blackfan Anemia26Erdheim-Chester病Erdheim-Chester Disease27法布雷病Fabry Disease28家族性地中海热Familial Mediterranean Fever29范可尼贫血Fanconi Anemia30半乳糖血症Galactosemia31戈谢病Gauchers Disease32全身型重症肌无力Generalized Myasthenia Gravis33Gitelman综合征Gitelman Syn
5、drome34戊二酸血症I型Glutaric Acidemia Type I35糖原累积病(I型、型)Glycogen Storage Disease (Type I、II)36血友病Hemophilia37肝豆状核变性Hepatolenticular Degeneration(Wilson Disease)38遗传性血管性水肿Hereditary Angioedema (HAE)39遗传性大疱性表皮松解症Hereditary Epidermolysis Bullosa40遗传性果糖不耐受症Hereditary Fructose Intolerance41遗传性低镁血症Hereditary H
6、ypomagnesemia42遗传性多发脑梗死性痴呆Hereditary Multi-infarct Dementia (Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy, CADASIL)43遗传性痉挛性截瘫Hereditary Spastic Paraplegia44全羧化酶合成酶缺乏症Holocarboxylase Synthetase Deficiency45同型半胱氨酸血症Homocysteinemia46纯合子家族性高胆固醇血症Homozygous H
7、ypercholesterolemia47亨廷顿舞蹈病Huntington Disease48HHH综合征Hyperornithinaemia-Hyperammonaemia-Homocitrullinuria Syndrome49高苯丙氨酸血症Hyperphenylalaninemia50低碱性磷酸酶血症Hypophosphatasia51低磷性佝偻病Hypophosphatemic Rickets52特发性心肌病Idiopathic Cardiomyopathy53特发性低促性腺激素性性腺功能减退症Idiopathic Hypogonadotropic Hypogonadism54特发性肺
8、动脉高压Idiopathic Pulmonary Arterial Hypertension55特发性肺纤维化Idiopathic Pulmonary Fibrosis56IgG4相关性疾病IgG4 related Disease57先天性胆汁酸合成障碍Inborn Errors of Bile Acid Synthesis58异戊酸血症Isovaleric Acidemia59卡尔曼综合征Kallmann Syndrome60朗格汉斯组织细胞增生症Langerhans Cell Histiocytosis61莱伦氏综合征Laron Syndrome62Leber遗传性视神经病变Leber H
9、ereditary Optic Neuropathy63长链3-羟酰基辅酶A脱氢酶缺乏症Long Chain 3-hydroxyacyl-CoA Dehydrogenase Deficiency64淋巴管肌瘤病Lymphangioleiomyomatosis (LAM)65赖氨酸尿蛋白不耐受症Lysinuric Protein Intolerance66溶酶体酸性脂肪酶缺乏症Lysosomal Acid Lipase Deficiency67枫糖尿症Maple Syrup Urine Disease68马凡综合征Marfan Syndrome69McCune-Albrigh综合征McCune-
10、Albright Syndrome70中链酰基辅酶A脱氢酶缺乏症Medium Chain Acyl-CoA Dehydrogenase Deficiency71甲基丙二酸血症Methylmalonic Academia72线粒体脑肌病Mitochodrial Encephalomyopathy73黏多糖贮积症Mucopolysaccharidosis74多灶性运动神经病Multifocal Motor Neuropathy75多种酰基辅酶A脱氢酶缺乏症Multiple Acyl-CoA Dehydrogenase Deficiency76多发性硬化Multiple Sclerosis77多系统
11、萎缩Multiple System Atrophy78肌强直性营养不良Myotonic Dystrophy79N-乙酰谷氨酸合成酶缺乏症N-acetylglutamate Synthase Deficiency80新生儿糖尿病Neonatal Diabetes Mellitus81视神经脊髓炎Neuromyelitis Optica82尼曼匹克病Niemann-Pick Disease83非综合征性耳聋Non-Syndromic Deafness84Noonan综合征Noonan Syndrome85鸟氨酸氨甲酰基转移酶缺乏症Ornithine Transcarbamylase Deficie
12、ncy86成骨不全症(脆骨病)Osteogenesis Imperfecta (Brittle Bone Disease)87帕金森病(青年型、早发型)Parkinson Disease (Young-onset , Early-onset)88阵发性睡眠性血红蛋白尿Paroxysmal Nocturnal Hemoglobinuria89黑斑息肉综合征Peutz-Jeghers Syndrome90苯丙酮尿症Phenylketonuria91POEMS综合征POEMS Syndrome92卟啉病Porphyria93Prader-Willi综合征Prader-Willi Syndrome94
13、原发性联合免疫缺陷Primary Combined Immune Deficiency95原发性遗传性肌张力不全Primary Hereditary Dystonia96原发性轻链型淀粉样变Primary Light Chain Amyloidosis97进行性家族性肝内胆汁淤积症Progressive Familial Intrahepatic Cholestasis98进行性肌营养不良Progressive Muscular Dystrophy99丙酸血症Propionic Acidemia100肺泡蛋白沉积症Pulmonary Alveolar Proteinosis101肺囊性纤维化P
14、ulmonary Cystic Fibrosis102视网膜色素变性Retinitis Pigmentosa103视网膜母细胞瘤Retinoblastoma104重症先天性粒细胞缺乏症Severe Congenital Neutropenia105婴儿严重肌阵挛性癫痫(Dravet综合征)Severe Myoclonic Epilepsy in Infancy (Dravet Syndrome)106镰刀型细胞贫血病Sickle Cell Disease107Silver-Russell综合征Silver-Russell Syndrome108谷固醇血症Sitosterolemia109脊髓延
15、髓肌萎缩症(肯尼迪病)Spinal and Bulbar Muscular Atrophy (Kennedy Disease)110脊髓性肌萎缩症Spinal Muscular Atrophy111脊髓小脑性共济失调Spinocerebellar Ataxia112系统性硬化症Systemic Sclerosis113四氢生物蝶呤缺乏症Tetrahydrobiopterin Deficiency114结节性硬化症Tuberous Sclerosis Complex115原发性酪氨酸血症Tyrosinemia116极长链酰基辅酶A脱氢酶缺乏症Very Long Chain Acyl-CoA De
16、hydrogenase Deficiency117威廉姆斯综合征Williams Syndrome118湿疹血小板减少伴免疫缺陷综合征Wiskott-Aldrich Syndrome119X-连锁无丙种球蛋白血症X-linked Agammaglobulinemia120X-连锁肾上腺脑白质营养不良X-linked Adrenoleukodystrophy121X-连锁淋巴增生症X-linked Lymphoproliferative Disease;.慲慲质量、安全事故的,视情节处按5-10万元进行违约处罚,并承担法律责任和全部经济损失。7、乙方虚报工程量的,除对虚增部分结算工程款全部扣除外
17、,将按照虚增部分工程款的2倍进行违约处罚。8、乙方承诺按时支付民工工资,否则甲方有权在乙方的工程款中扣除相应费用,用于支付民工工资,不足部分由乙方自行负责进行债务清偿。 9、乙方承诺具备签订和履行本合同所需的法定许可和相应资质,否则由此产生的任何损失和责任由乙方承担,且甲方有权不支付任何费用。 10、对违约金,甲方有权从应付乙方的款项中直接扣除。第九条 争议解决本合同未尽事宜或本合同履行过程中产生的任何争议,双方应友好协商解决。协商不成的,双方同意提交合同签订地法院进行诉讼。律师费、诉讼费由败诉方承担,其中律师费按照单个项目总价的5%进行支付,最低不低于1万元。第十条 其他约定1、乙方在开工前向甲方提交施工计划、承揽施工质量保证书、安全生产责任书。2、乙方应当向甲方提供为施工人员办理人身意外保险的相关凭证。3、本合同期限为一年,自2018年1月1日至2018年12月31日止。4、本合同一式叁份,自双方签章后生效,甲方执贰份,乙方执一份。甲方(盖章): 乙方(盖章): 委托代理人(签字): 委托代理人(签字):联系电话: 联系电话:签订日期: 签订日期:附件1国动科技有限公司建设分公司2018年通信基站施工服务基准单价附件2通信基站质量违约处罚标准附件3 安全生产责任书;.