Chapter 19-C Inheritance
小王子The Little Prince 英文Chapter 19

Chapter 19−the little prince climbs a mountain rangeAfter that, the little prince climbed a high mountain. The only mountains he had ever known were the three volcanoes, which came up to his knees. And he used the extinct volcano as a footstool. "From a mountain as high as this one," he said to himself, "I shall be able to see the whole planet at one glance, and all the people..."But he saw nothing, save peaks of rock that were sharpened like needles. "Good morning," he said courteously."Good morning−−Good morning−−Good morning," answered the echo."Who are you?" said the little prince."Who are you−−Who are you−−Who are you?" answered the echo."Be my friends. I am all alone," he said."I am all alone−−all alone−−all alone," answered the echo."What a queer planet!" he thought. "It is altogether dry, and altogether pointed, and altogether harsh and forbidding. And the people have no imagination. They repeat whatever one says to them... On my planet I had a flower; she always was the first to speak..."。
遗传学课后题1-6

医学研究: 3)医学研究:如建立动物模型研究了解各种调 节心血管功能的因子如转脂蛋白的生理功能及作 用;建立带有肿瘤基因的转基因动物可了解哪些 组织对肿瘤基因转化活性敏感、 组织对肿瘤基因转化活性敏感、肿瘤形成与其基 因的关系、肿瘤基因生长分化影响等等;制备遗 因的关系、肿瘤基因生长分化影响等等; 传性疾病的转基因动物模型, 传性疾病的转基因动物模型,研究和治疗人类遗 传性疾病。 传性疾病。 2、改良和培育动物新品种 3、研制和生产生物活性物质
人类遗传学:又称医学遗传学。主要分支有: 7、1)人类遗传学:又称医学遗传学。主要分支有: 临床细胞遗传学、药物遗传学、免疫遗传学、 临床细胞遗传学、药物遗传学、免疫遗传学、肿瘤 遗传学等。 遗传学等。 动物遗传学:哺乳动物遗传学、鸟类遗传学、 2)动物遗传学:哺乳动物遗传学、鸟类遗传学、 鱼类遗传学、昆虫遗传学、单细胞动物遗传学等。 鱼类遗传学、昆虫遗传学、单细胞动物遗传学等。 植物遗传学:作物遗传学、观赏植物遗传学、 3)植物遗传学:作物遗传学、观赏植物遗传学、 经济植物遗传学、林木遗传学等。 经济植物遗传学、林木遗传学等。 微生物遗传学:细菌遗传学、病毒遗传学等。 4)微生物遗传学:细菌遗传学、病毒遗传学等。
遗传学发展的关键领域是: 6、遗传学发展的关键领域是: 研究人类遗传和变异规律, 1)研究人类遗传和变异规律,通过对人类基因 图谱中功能基因信息的全面解读, 图谱中功能基因信息的全面解读,人类性状的遗传 分析、遗传病的分布和发生机理、遗传病的诊断、 分析、遗传病的分布和发生机理、遗传病的诊断、 基因治疗,按照患者遗传学来选择治疗方法。 基因治疗,按照患者遗传学来选择治疗方法。 干细胞的研究。 2)干细胞的研究。 3)转基因生物方面开展动植物新品种的研究。 转基因生物方面开展动植物新品种的研究。 克隆技术的研究。 4)克隆技术的研究。 5)生物制药方面开展药物分子设计技术的研究。 生物制药方面开展药物分子设计技术的研究。 DNA鉴定技术 鉴定技术。 6)DNA鉴定技术。
《哈利波特与阿兹卡班囚徒》第19章《伏地魔的仆人》中英文对照学习版

中英文对照学习版Harry Potter and the Prisoner of Azkaban《哈利波特与阿兹卡班囚徒》Chapter NineteenThe Servant of Lord Voldemort第19章伏地魔的仆人Hermione screamed. Black l eapt to his feet. Harry jumped as though he'd received a huge el ectric shock.赫敏尖叫起来,布莱克一跃而起,哈利好像突然遭受了强烈的电击。
‘I found this at the base of the Whomping Will ow,’ said Snape, throwing the Cl oak asid e, careful to keep his wand pointing directly at Lupin's chest. ‘Very useful, Potter, I thank you ...’“我在打人柳底下发现了这个。
”斯内普把隐形衣丢到一边,一面仍小心地用魔杖直指卢平的胸膛,“很有用的,波特,我要谢谢你……”Snape was slightly breathl ess, but his face was full of suppressed triumph. ‘You're wond ering, perhaps, how I knew you were here?’he said, his eyes glittering. ‘I’ve just been to your office, Lupin. You forgot to take your Potion tonight, so I took a gobl etful along. And very lucky I did ... lucky for me, I mean. Lying on your d esk was a certain map. One glance at it tol d me all I need ed to know.I saw you running al ong this passageway and out of sight.’斯内普有点气喘,但一脸抑制不住的得意。
英文版简爱 19

Chapter 19THE library looked tranquil enough as I entered it, and the sibyl —if sibyl she were —was seated snugly enough in an easy-chair at the chimney corner. She had on a red cloak and a black bonnet: or rather, a broad- brimmed gipsy hat, tied down with a striped handkerchief under the chin. An extinguished candle stood on the table; she was bending over the fire, and seemed reading in a little black book, like a Prayer Book, by the light of the blaze: she muttered the words to herself, as most old women do, while she read; she did not desist immediately on my entrance: it appeared she wished to finish a paragraph.I stood on the rug and warmed my hands, which were rather cold with sitting at a distance from the drawing- room fire. I felt now as composed as ever I did in my life: there was nothing indeed in the gipsy's appearance to trouble one's calm. She shut her book and slowly looked up; her hat-brim partially shaded her face, yet I could see, as she raised it, that it was a strange one. It looked all brown and black: elf-locks bristled out from beneath a white band which passed under her chin, and came half over her cheeks, or rather jaws: her eye confronted me at once, with a bold and direct gaze.'Well, and you want your fortune told?' she said, in a voice as decided as her glance, as harsh as her features.'I don't care about it, mother; you may please yourself: but I ought to warn you, I have no faith.' 'It's like your impudence to say so: l expected it of you: I heard it in your step as you crossed the threshold.''Did you? You've a quick ear.''I have; and a quick eye, and a quick brain.''You need them all in your trade.''I do: especially when I've customers like you to deal with. Why don't you tremble?''I'm not cold.''Why don't you turn pale?''I am not sick.''Why don't you consult my art?''I'm not silly.'The old crone 'nichered' a laugh under her bonnet and bandage; she then drew out a short black pipe, and lighting it, began to smoke. Having indulged a while in this sedative, she raised her bent body, took the pipe from her lips, and while gazing steadily at the fire, said very deliberately —'You are cold; you are sick; you are silly.''Prove it, ' I rejoined.'I will in a few words. You are cold, because you are alone: no contact strikes the fire from you that is in you. You are sick, because the best of feelings, the highest and the sweetest given to man, keeps far away from you. You are silly, because, suffer as you may, you will not beckon it to approach, nor will you stir one step to meet it where it waits for you.'She again put her short black pipe to her lips, and renewed her smoking with vigour.'You might say all that to almost any one who, you knew, lived as a solitary dependant in a great house.''I might say it to almost any one: but would it be true of almost any one?''In my circumstances.''Yes; just so, in your circumstances: but find me another precisely placed as you are.''It would be easy to find you thousands.''You could scarcely find me one. If you knew it, you are peculiarly situated: very near happiness; yes, within reach of it. The materials are all prepared; there only wants a movement to combine them. Chance laid them somewhat apart; let them be once approached and bliss results.''I don't understand enigmas. I never could guess a riddle in my life.'If you wish me to speak more plainly, show me your palm.''And I must cross it with silver, I suppose?''To be sure.'I gave her a shilling: she put it into an old stocking-foot which she took out of her pocket, and having tied it round and returned it, she told me to hold out my hand. I did. She approached her face to the palm, and pored over it without touching it.'It is too fine, 'said she. 'I can make nothing of such a hand as that; almost without lines; besides, what is in a palm? Destiny is not written there.''I believe you, 'said I.'No, ' she continued, 'it is in the face: on the forehead, about the eyes, and in the eyes themselves, in the lines of the mouth. Kneel, and lift up your head.''Ah! now you are coming to reality, ' I said, as I obeyed her. 'I shall begin to put some faith in you presently.'I knelt within half a yard of her. She stirred the fire, so that a ripple of light broke from the disturbed coal: the glare, however, as she sat only threw her face into deeper shadow: mine, it illumined.'I wonder with what feelings you came to me to-night, ' she said, when she had examined me a while. 'I wonder what thoughts were busy in your heart during all the hours you sit in yonder room with the fine people flitting before you like shapes in a magic-lantern: just as little sympathetic communion passing between you and them as if they were really mere shadows of human forms, and not the actual substance.''I feel tired often, sleepy sometimes, but seldom sad.''Then you have some secret hope to buoy you up and please you with the whispers of the future?''Not I. The utmost I hope is, to save enough money out of my earnings to set up a school some day in a little house rented by myself. ''A mean nutriment for the spirit to exist on: and sitting in that window- seat (you see I know your habits) —''You have learned them from the servants.''Ah! you think yourself sharp. Well, perhaps I have: to speak truth I have an acquaintance with one of them, Mrs Poole —'I started to my feet when I heard the name.'You have —have you?' thought l; 'there is diablerie in the business after all, then!''Don't be alarmed, 'continued the strange being; ' she's a safe hand is Mrs Poole: close and quiet; any one might repose confidence in her. But, as I was saying: sitting in that window-seat, do you think of nothing but your future school? Have you no present interest in any of the company who occupy the sofas and chairs before you? Is there not one face you study? one figure whose movements you follow with at least curiosity?''I like to observe all the faces, and all the figures.''But do you never single one from the rest —or it may be two?''I do frequently; when the gestures or looks of a pair seem telling a tale: it amuses me to watch them.''What tale do you like best to hear?''Oh, I have not much choice! They generally run on the same theme —courtship; and promise to end in the same catastrophe —marriage.'And do you like that monotonous theme?''Positively, I don't care about it: it is nothing to me.''Nothing to you? When a lady, young and full of life and health, charming, with beauty, and endowed with the gifts of rank and fortune, sits and smiles in the eyes of a gentleman you —''I what? ''You know —and perhaps think well of.''I don't know the gentlemen here. I have scarcely interchanged a syllable with one of them; and as to thinking well of them, I consider some respectable, and stately, and middle- aged; and others young, dashing, handsome, and lively: but certainly they are all at liberty to be the recipients of whose smiles they please, without my feeling disposed to consider the transaction of any moment to me.''You don't know the gentlemen here? You have not exchanged a syllable with one of them? Will you say that of the master of the house?''He is not at home.''A profound remark! A most ingenious quibble! He went to Millcote this morning, and will be back here to- night or to-morrow: does that circumstance exclude him from the list of your acquaintance —blot him, as it were, out of existence?''No; but I can scarcely see what Mr Rochester has to do with the theme you had introduced.''I was talking of ladies smiling in the eyes of gentlemen; and of late so many smiles have been shed into Mr Rochester's eyes that they overflow like two cups filled above the brim: have you never remarked that?''Mr Rochester has a right to enjoy the society of his guests.''No question about his right: but have you never observed that, of all the tales told here about matrimony, Mr Rochester has been favoured with the most lively and the most continuous?''The eagerness of a listener quickens the tongue of a narrator.' I said this rather to myself than to the gipsy, whose strange talk, voice, manner, had by this time wrapped me in a kind of dream. One unexpected sentence came from her lips after another, till I got involved in a web of mystification; and wondered what unseen spirit had been sitting for weeks by my heart watching its workings and taking record of every pulse.'Eagerness of a listener!' repeated she: 'yes; Mr Rochester has sat by the hour, his ear inclined to the fascinating lips that took such delight in their task of communicating; and Mr Rochester was so willing to receive and looked so grateful for the pastime given him: you have noticed this?''Grateful! I cannot remember detecting gratitude in his face.''Detecting! You have analysed, then. And what did you detect, if not gratitude?'I said nothing.'You have seen love: have you not? —and, looking forward, you have seen him married, and beheld his bride happy?''Humph! Not exactly. Your witch's skill is rather at fault sometimes.''What the devil have you seen then?''Never mind: I came here to inquire, not to confess. Is it known that Mr Rochester is to be married?''Yes; and to the beautiful Miss Ingram.''Shortly?''Appearances would warrant that conclusion; and, no doubt (though, with an audacity that wants chastising out of you, you seem to question it), they will be a superlatively happy pair. He must love such a handsome, noble, witty, accomplished lady; and probably she loves him, or, if not his person, at least his purse. I know she considers the Rochester estate eligible to the last degree; though (God pardon me! ) I told her something on that point about an hour ago which made her look wondrous grave: the corners of her mouth fell half an inch. I would advise her blackavised suitor to look out: if another comes, with a longer or clearer rent-roll —he's dished —''But, mother, I did not come to hear Mr Rochester's fortune: I came to hear my own; and you have told me nothing of it.''Your fortune is yet doubtful: when I examined your face, one trait contradicted another. Chance has meted you a measure of happiness: that I know. I knew it before I came here this evening. She has laid it carefully on one side for you. I saw her do it. It depends on yourself to stretch out your hand, and take it Up: but whether you will do so is the problem I study. Kneel again on the rug.''Don't keep me long; the fire scorches me.'I knelt. She did not stoop towards me, but only gazed, leaning back in her chair. She began muttering:'The flame flickers in the eye; the eye shines like dew; it looks soft and full of feeling; it smiles at my jargon: it is susceptible, impression follows impression through its clear sphere; where it ceases to smile, it is sad; an unconscious lassitude weighs on the lid: that signifies melancholy resulting from loneliness. It turns from me; it will not suffer further scrutiny: it seems to deny, by a mocking glance, the truth of the discoveries I have already made —to disown the charge both of sensibility and chagrin: its pride and reserve only confirm me in my opinion. The eye is favourable.'As to the mouth, it delights at times in laughter, it is disposed to impart all that the brain conceives; though I dare say it would be silent on much the heart experiences. Mobile and flexible, it was never intended to be compressed in the eternal silence of solitude: it is a mouth which should speak much and smile often, and have human affection for its interlocutor. That feature too is propitious.'I see no enemy to a fortunate issue but in the brow, and that brow professes to say —"I can live alone, if self-respect and circumstances require me so to do. I need not sell my soul to buy bliss. I have an inward treasure born with me, which can keep me alive if all extraneous delights should be withheld, or offered only at a price I cannot afford to give." The forehead declares, "Reason sits firm and holds the reins and she will not let the feelings burst away and hurry her to wild chasms. The passions may rage furiously, like true heathens, as they are; and the desires may imagine all sorts of vain things: but judgement shall still have the last word in every argument, and the casting vote in every decision. Strong wind, earthquake-shock, and fire may pass by: but I shall follow the guiding of that still small voice which interprets the dictates of conscience."'Well said, forehead; your declaration shall be respected. I have formed my plans —right plans I deem them —and in them I have attended to the claims of conscience, the counsels ofreason. I know how soon youth would fade and bloom perish, if, in the cup of bliss offered but one dreg of shame, or one flavour of remorse were detected; and I do not want sacrifice, sorrow, dissolution —such is not my taste. I wish to foster, not to blight —to earn gratitude, not to wring tears of blood —no, nor of brine: my harvest must be in smiles, in endearments, in sweet —That will do. I think I rave in a kind of exquisite delirium. I should wish now to protract this moment ad infinitum, but I dare not. So far I have governed myself thoroughly. I have acted as I inwardly swore I would act; but further might try me beyond my strength. Rise, Miss Eyre: leave me; "the play is played out".'Where was I? Did I wake or sleep? Had I been dreaming? Did I dream still? The old woman's voice had changed: her accent, her gesture, and all, were familiar to me as my own face in a glass —as the speech of my own tongue. I got up, but did not go. I looked; I stirred the fire, and looked again: but she drew her bonnet and her bandage closer about her face, and again beckoned me to depart. The flame illuminated her hand stretched out: roused now, and on the alert for discoveries, I at once noticed that hand. It was no more the withered limb of eld than my own; it was a rounded, supple member, with smooth fingers, symmetrically turned; a broad ring flashed on the little finger, and stooping forward, I looked at it, and saw a gem I had seen a hundred times before. Again I looked at the face; which was no longer turned from me —on the contrary, the bonnet was doffed, the bandage displaced, the head advanced.'Well, Jane, do you know me?' asked the familiar voice.'Only take off the red cloak, sir, and then —''But the string is in a knot —help me.''Break it, sir. ''There, then —"Off, ye lendings!"' And Mr Rochester stepped out of his disguise.'Now, sir, what a strange idea!''But well carried out, eh? Don't you think so?''With the ladies you must have managed well.''But not with you?''You did not act the character of a gipsy with me.''What character did I act? My own?''No; some unaccountable one. In short I believe you have been trying to draw me out —or in; you have been talking nonsense to make me talk nonsense. It is scarcely fair, sir.''Do you forgive me, Jane?''I cannot tell till I have thought it all over. If, on reflection, I find I have fallen into no great absurdity, I shall try to forgive you; but it was not right.''Oh, you have been very correct —very careful, very sensible.'I reflected, and thought, on the whole, I had. It was a comfort-but, indeed, I had been on my guard almost from the beginning of the interview. Something of masquerade I suspected. I knew gipsies and fortune-tellers did not express themselves as this seeming old woman had expressed herself; besides, I had noted her feigned voice, her anxiety to conceal her features. But my mind had been running on Grace Poole —that living enigma, that mystery of mysteries, as I considered her. I had never thought of Mr Rochester.'Well, ' said he, 'what are you musing about? What does that grave smile signify?''Wonder and self-congratulation, sir. I have your permission to retire now, I suppose?''No; stay a moment; and tell me what the people in the drawingroom yonder are doing.''Discussing the gipsy, I dare say.''Sit down! —Let me hear what they said about me.''I had better not stay long, sir; it must be near eleven o-clock. —Oh! are you aware, Mr Rochester, that a stranger has arrived here since you left this morning?''A stranger! —no; who can it be? I expected no one; is he gone?''No; he said he had known you long, and that he could take the liberty of installing himself here till you returned.''The devil he did! Did he give his name?''His name is Mason, sir; and he comes from the West Indies; from Spanish Town, in Jamaica, I think.'Mr Rochester was standing near me; he had taken my hand, as if to lead me to a chair. As I spoke he gave my wrist a convulsive grip; the smile on his lips froze: apparently a spasm caught his breath.'Mason! —the West Indies!' he said, in a tone one might fancy a speaking automaton to enounce its single words; 'Mason! —the West Indies!' he reiterated; and he went over the syllables three times, growing, in the intervals of speaking, whiter than ashes: he hardly seemed to know what he was doing.'Do you feel ill, sir?' I inquired.'Jane, I've got a blow —I've got a blow, Jane!' He staggered.'Oh, lean on me, sir.''Jane, you offered me your shoulder once before; let me have it now.''Yes, sir, yes; and my arm.'He sat down and made me sit beside him. Holding my hand in both his own, he chafed it; gazing on me, at the same time, with the most troubled and dreary look.'My little friend! 'said he, 'I wish I were in a quiet island with only you; and trouble, and danger, and hideous recollections removed from me.'Can I help you, sir? —I'd give my life to serve you.''Jane, if aid is wanted, I'll seek it at your hands; I promise you that.''Thank you, sir. Tell me what to do —I'll try, at least, to do it.''Fetch me now, Jane, a glass of wine from the dining-room: they will be at supper there; and tell me if Mason is with them, and what he is doing.'I went. I found all the party in the dining-room at supper, as Mr Rochester had said; they were not seated at table —the supper was arranged on the sideboard; each had taken what he chose, and they stood about here and there in groups, their plates and glasses in their hands. Every one seemed in high glee; laughter and conversation were general and animated. Mr Mason stood near the fire, talking to Colonel and Mrs Dent, and appeared as merry as any of them. I filled a wine-glass (I saw Miss Ingram watch me frowningly as I did so: she thought I was taking a liberty, I dare say), and I returned to the library.Mr Rochester's extreme pallor had disappeared, and he looked once more firm and stem. He took the glass from my hand.'Here is to your health, ministrant spirit!' he said. He swallowed the contents and returned it to me. 'What are they doing, Jane?''Laughing and talking, sir.''They don't look grave and mysterious, as if they had heard something strange?''Not at all: they are full of jests and gaiety.''And Mason?''He was laughing too.''If all those people came in a body and spat at me, what would you do, Jane?''Turn them out of the room, sir, if I could.'He half-smiled. 'But if I were to go to them, and they only looked at me coldly, and whispered sneeringly amongst each other, and then dropped off and left me one by one, what then? Would you go with them?'I rather think not, sir: I should have more pleasure in staying with you.'To comfort me?''Yes, sir, to comfort you, as well as I could.''And if they laid you under a ban for adhering to me?''I probably should know nothing about their ban; and if I did, I should care nothing about it.''Then you could dare censure for my sake?''I could dare it for the sake of any friend who deserved my adherence; as you, I am sure, do.''Go back now into the room; step quietly up to Mason, and whisper in his ear that Mr Rochester is come and wishes to see him: show him in here, and then leave me.''Yes, sir.'I did his behest. The company all stared at me as I passed straight among them. I sought Mr Mason, delivered the message, and preceded him from the room: I ushered him into the library, and then I went upstairs.At a late hour, after I had been in bed some time, I heard the visitors repair to their chambers; I distinguished Mr Rochester's voice, and heard him say, 'This way, Mason; this is your room.'He spoke cheerfully: the gay tones set my heart at ease. I was soon asleep.。
奥尔波特综合征(Alport syndrome)

奥尔波特综合征(Alport syndrome)奥尔波特综合征(Alport syndrome),又名奥尔波特氏综合征(Alport’s syndrome),也被称作遗传性肾炎(hereditary nephritis)。
可在罕见疾病网上查询:Q87.801,奥尔波特氏综合征(遗传性肾炎和耳聋)。
Alport syndrome or hereditary nephritis is a genetic disorder characterized by glomerulonephritis, endstage kidney disease, and hearing loss. Alport syndrome can also affect the eyes (lenticonus). The presence of blood in the urine (hematuria) is almost always found in this condition.It was first identified in a British family by Dr. Cecil A. Alport in 1927, though William Howship Dickinson is considered by some to have made contributions to the characterization.病因:Alport syndrome is caused by mutations in COL4A3, COL4A4, and COL4A5, collagen biosynthesis genes. Mutations in any of these genes prevent the proper production or assembly of the type IV collagen network, which is an important structural component of basement membranes in the kidney, inner ear, andeye. Basement membranes are thin, sheet-like structures that separate and support cells in many tissues. When mutations prevent the formation of type IV collagen fibers, the basement membranes of the kidneys are not able to filter waste products from the blood and create urine normally, allowing blood and protein into the urine.The abnormalities of type IV collagen in kidney basement membranes cause gradual scarring of the kidneys, eventually leading to kidney failure in many people with the disease. Progression of the disease leads to basement membrane thickening and gives a "basket-weave" appearance from splitting of the lamina densa. Single molecule computational studies of type IV collagen molecules have shown changes in the structure and nanomechanical behavior of mutated molecules, notably leading to a bent molecular shape with kinks.遗传方式:Alport syndrome can have different inheritance patterns that are dependent on the genetic mutation.In most people with Alport syndrome, the condition is inheritedin an X-linked pattern, due to mutations in the COL4A5 gene.A condition is considered X-linked if the gene involved in the disorder is located on the X chromosome. In males, who have only one X chromosome, one altered copy of the COL4A5 gene is sufficient to cause severe Alport syndrome, explaining why most affected males eventually develop kidney failure. In females, who have two X chromosomes, a mutation in one copy of the COL4A5 gene usually results in blood in the urine, but most affected females do not develop kidney failure.Alport syndrome can be inherited in an autosomal recessive pattern if both copies of the COL4A3 or COL4A4 gene, located on chromosome 2, have been mutated. Most often, the parents of a child with an autosomal recessive disorder are not affected but are carriers of one copy of the altered gene.Past descriptions of an autosomal dominant form are now usually categorized as other conditions, though some uses of the term in reference to the COL4A3 and COL4A4 loci have been published.临床诊断:Gregory et al., 1996, gave the following 10 criteria for the diagnosis of Alport syndrome; Four of the 10 criteria must bemet:1. Family history of nephritis of unexplained haematuria in a first degree relative of the index case or in a male relative linked through any numbers of females.2. Persistent haematuria without evidence of another possibly inherited nephropathy such as thin GBM disease, polycystic kidney disease or IgA nephropathy.3. Bilateral sensorineural hearing loss in the 2000 to 8000 Hz range. The hearing loss develops gradually, is not present in early infancy and commonly presents before the age of 30 years.4. A mutation in COL4An (where n = 3, 4 or 5).5. Immunohistochemical evidence of complete or partial lack of the Alport epitope in glomerular, or epidermal basement membranes, or both.6. Widespread GBM ultrastructural abnormalities, in particular thickening, thinning and splitting.7. Ocular lesions including anterior lenticonus, posterior subcapsular cataract, posterior polymorphous dystrophy and retinal flecks.8. Gradual progression to ESRD in the index case of at least two family members.9. Macrothrombocytopenia or granulocytic inclusions, similar to the May-Hegglin anomaly.10. Diffuse leiomyomatosis of esophagus or female genitalia, or both.(The use of eye examinations for screening has been proposed.)免疫组织化学:Immunohistochemical (IHC) evidence of the X-linked form Alport syndrome may be obtained from biopsies of either the skin or the renal glomerulus. In this processes, antibodies are used to detect the presence or absence of the alpha3, alpha4, and alpha5 chains of collagen type 4.All three of these alpha chains are present in the glomerular basement membrane of normal individuals. In individuals expressing the X-linked form of Alport's syndrome, however, the presence of the dysfunctional alpha5 chain causes the assembly of the entire collagen 4 complex to fail, and none of these three chains will be detectable in either the glomerular or the renal tubular basement membrane.Of these three alpha chains, only alpha5 is normally expressed in the skin,[citation needed] so the hallmark of X-linked Alport syndrome on a skin biopsy is the absence of alpha5 staining.治疗:As there is no known cure for the condition, treatments are symptomatic. Patients are advised on how to manage the complications of kidney failure and the proteinuria that develops is often treated with ACE inhibitors, although they are not always used simply for the elevated blood pressure.Once kidney failure has developed, patients are given dialysis or can benefit from a kidney transplant, although this can cause problems. The body may reject the new kidney as it contains normal type IV collagen, which may be recognized as foreign by the immune system.Gene therapy as a possible treatment option has been discussed.另附一篇关于本病的介绍:Diseases of the Kidney: Alport SyndromeVersion of Aug 31, 1999These citations, from the three most recent editions of the large three-volume monograph "Diseases of the Kidney," refer to comprehensive clinical descriptions of Alport syndrome by our University of Utah group.CL Atkin, MC Gregory, WA Border. Alport syndrome. Pp 617-641 (Chapter 19) in RW Schrier, CW Gottschalk (Eds), Diseases of the Kidney, 4th ed, Little, Brown & Co., Boston, 1988.MC Gregory, CL Atkin. Alport syndrome. Pp 571-591 (Chapter 19) in RW Schrier, CW Gottschalk (Eds), Diseases of the Kidney, 5th ed, Little, Brown & Co., Boston, 1993.MC Gregory, CL Atkin. Alport's Syndrome, Fabry's Disease, and Nail-Patella Syndrome, chapter 19 in RW Schrier, CW Gottschalk (Eds), Diseases of the Kidney, 6th ed, Little, Brown & Co.,Boston, pp. 561-590, 1997. NLM Call No.WJ 300 D611 1996, ISBN 0-316-77456-1."Diseases of the Kidney" may be purchased from the publisher, but is readily found in medical school libraries. I, Curtis L. Atkin, have as yet been unable to obtain the publisher's permission to completely reprint here this copyrighted material. The following essay was adapted and condensed from these Chapters by Dr Martin C. Gregory for the HNF Newsletter No. 27, September 1995. My notes and emendations to Dr Gregory's piece are [bracketed].---------------------------------------------------------------------INTRODUCTIONHereditary nephritis is a disparate group of often ill-defined conditions that are similar only in that they run in families and present many diagnostic difficulties. Because the incidence and diversity of such diseases are not generally recognized, opportunities for timely diagnoses and genetic counseling are lost. The most common and best known hereditary nephritis is Alport syndrome. In this chapter, Alport syndrome will be defined as progressive hereditary hematuric nonimmuneglomerulonephritis characterized ultrastructurally by irregular thickening, thinning, and lamellation of the glomerular basement membrane (GBM). In some kindreds nonrenal features occur. These include hearing loss, various ocular defects, abnormalities of platelet number and function, granulocyte inclusions, and esophageal and genital leiomyomatosis (tumors). We regard nonrenal features as helpful diagnostic pointers in some kindreds, although they are not essential to the diagnosis.---------------------------------------------------------------------DISEASE DEFINITIONSTerminology and diagnostic criteria in many reports vary, and it is difficult to define exactly what Alport syndrome or progressive hereditary nephritis should include. Alport did not perform renal histological studies and as his kindred "has rid itself of Alport's disease," no means exist for defining the syndrome he described in modern terms. This kindred had dominantly inherited kidney disease that was characterized in both sexes by hematuria and urinary erythrocyte casts, variable proteinuria, and especially in males by progressive hearingloss and renal failure. Affected males had hearing loss, died in adolescence, and had no offspring. Progressive azotemia (excesses of urea and creatinine in the blood) and ESRD (end stage renal disease) especially in males, complex ultrastructural anomalies of GBM (glomerular basement membrane), and negative glomerular immunofluorescence studies are characteristics of all types of Alport syndrome. Eventual ESRD of nearly all affected males is a central feature. [Now that Type IV disease (below) is better understood, it has become clear that hearing loss essentially always accompanies renal failure in Alport syndrome]. Many reports have expanded the classic dyad of aural and renal symptoms to include other associated nonrenal anomalies and traits [such as] thrombocytopathia (bleeding disorder characterized by defective platelets), ocular abnormalities, or leiomyomatosis. [Anti-basement membrane collagen antisera that bind] normal GBM and epidermal basement membrane (EBM) fail to bind these membranes in many but not all Alport kindreds].---------------------------------------------------------------------AFFECTED INDIVIDUALSChronic hematuria (blood in the urine) is the cardinal sign of Alport syndrome. Persons with hematuria and a gene for Alport syndrome are affected. Clinically normal gene carriers (preponderantly females) should be identified for genetic studies, counseling, and selection of kidney donors; it is, however, misleading to characterize them as affected persons. Our minimal criterion for affectedness is greater than or equal to 3 red cells per high-power field of the centrifuged fresh urine sediment (with rigorous exclusion of menstrual blood) but choice of [either 1 or more, or of 10 or more] erythrocytes per field would change few diagnoses. Urinary erythrocyte casts and proteinuria support the diagnosis of Alport syndrome, but are not necessary for it, whereas other urinary findings (pyuria, positive urine cultures, or proteinuria in the absence of hematuria) are not signs of Alport syndrome. The prime criterion for ascertainment of Alport syndrome in kindreds is the demonstration of a family history of chronic glomerulonephritis in multiple closely related persons.---------------------------------------------------------------------CLASSIFICATIONClinical features regularly displayed by affected persons in a kindred define the characteristic phenotype of Alport syndrome in that kindred. The severity [and timing] of symptoms may vary [amongst relatives] according to age and gender, [yet many kindreds show statistically distinct averages]. Kindreds clearly differ in [rates of progression of renal failure,] typical ages of ESRD, [rates of progression] of hearing loss, [and presence of] ocular abnormalities. Different phenotypes and different modes of inheritance [demonstrate genetic heterogeneity and phenotypic heterogeneity] of Alport syndrome.Juvenile versus Adult Types of Alport Syndrome. Schneider first recognized that males in some kindreds with Alport syndrome experienced ESRD in childhood or adolescence, while in other kindreds, males that had ESRD were middle-aged. Bimodality of age of ESRD has been shown repeatedly; juvenile kindreds are those in which males develop ESRD at a mean age below 31 years; in adult types of Alport syndrome ESRD occurs in males at a mean age greater than 31 years.Major Types of Alport Syndrome. Our analysis of 65 kindredsindubitably suffers from nonuniformity of diagnostic criteria in the original reports, but most fit the following classification well. [The scheme, however, grows ever more obsolete; in particular it does not include autosomal recessive inheritance. Nascent, improved classifications are based on DNA analyses and difficult but gradually improving discrimination of clinical features (phenotype).]Type I Alport Syndrome is dominantly inherited juvenile type nephritis with hearing loss, where affected males have no offspring. Pedigree analyses are uninformative for X-linked vs. autosomal dominant inheritance. Type I is an interim category subject to reclassification because ESRD treatment may now allow the affected males to reproduce, or newer genetic methods may allow chromosomal localization of the nephritis genes. Ocular abnormalities are restricted to the juvenile types (I, II, VI) of Alport syndrome, but may not be present in all kindreds with juvenile disease.Type II Alport Syndrome is X-linked dominant, juvenile type nephritis with hearing loss [caused by mutations of the COL4A5 gene for alpha-5 chain of basement membrane (Type IV) collagen].Types II and VI Alport syndrome may be difficult to distinguish because most of the kindreds have few offspring of affected males.Type III Alport Syndrome is X-linked dominant, adult type nephritis with hearing loss [caused by mutations of the COL4A5 gene].Type IV Alport Syndrome is X-linked dominant, adult type nephritis [caused by mutations of the COL4A5 gene. Until the advent of dialysis and transplantation, families had no marked hearing loss, but now it is clear that hearing loss follows a decade or so after ESRD; see reference 38 in our Bibliography].Type V Alport Syndrome is autosomal dominant nephritis with hearing loss and thrombocytopathia. Type V corresponds to McKusick's category No. 15365, (Epstein Syndrome). This disease has been reported in 12 families and 4 sporadic cases; there were clear instances of male to male transmission. Because ESRD data were limited, the distinction of juvenile- vs. adult-type Alport syndrome could not be made in these kindreds. Prevalence of ESRD of females with type V disease mayapproach that of males, but data are scanty. [See additional article by Dr. Gregory, "Macrothrombocytopathy, Nephritis, and Deafness (Epstein syndrome; Alport syndrome with Macrothrombocytopenia) in the HNF Newsletter No. 27, September 1995. The responsible genes are unknown as of 5/99]Type VI Alport Syndrome is autosomal dominant, juvenile type nephritis with hearing loss [caused in at least some cases by mutations in COL4A3 and COL4A4 genes for alpha-3 and -4 chains of basement membrane (Type IV) collagen. Other autosomal genes are sought].Indeterminate types of Alport Syndrome are unclassifiable as types I-VI in the above scheme. Nineteen of the 65 kindreds in our retrospective study were unclassifiable. Alport syndrome associated with leiomyomatosis is another distinct entity [caused by large deletions spanning the adjacent X-linked COL4A5 and COL4A6 genes and perhaps other genes, making it a "contiguous gene syndrome"].---------------------------------------------------------------------GENE FREQUENCYThe estimated gene frequency [for X-linked Alport syndrome] is 1:5000 in the Intermountain West of the United States. Shaw and Kallen estimated Alport syndrome gene frequency 1:10,000 elsewhere in the United States. We could not estimate worldwide incidence of Alport syndrome. It has been reported in many races and is probably not associated with race or geography. We believe that the observed incidence of Alport syndrome in Utah is about twice that elsewhere, not because of the "founder principle", but because of the unusual extent of our studies. The origins and large founding size of the Utah population, and high rates of gene flow have resulted in gene frequencies that are similar to those in northern Europe.[Dr David Barker tentatively estimates the frequency of autosomal recessive (COL4A3 and COL4A4) mutations at 1:250.]---------------------------------------------------------------------MODES OF INHERITANCEPenetrance and Dominance. Hematuria and ESRD are both manifestations of Alport syndrome, with penetrances thateventually coincide in males, but may be widely disparate in females. Penetrance of hematuria and ESRD is 100% in males with types II, III, and IV disease. For types I, V, and VI hematuria and ESRD likely also approach 100%. For females with types III or IV disease prevalence of hematuria is 90% and eventual prevalence of ESRD in females approximates 15%. ESRD may supervene in close to 100% of females with type V disease. In both males and females the penetrance of hematuria remains constant with age.[X-linked recessive inheritance has in past been implicated from observations of ESRD in most males and much less ESRD in females. In truly X-linked recessive traits such as hemophilia and colorblindness, males are affected and females are clinically normal. In Alport syndrome, however,] hematuria indicates nephritis in most gene-carriers of either [gender. Thus X-linked recessive Alport syndrome is a specious category].X-linked Dominant Inheritance. Starting with the original studies of Utah Kindred P, forms of X-linked, sex-linked, or gender-influenced inheritance have been proposed in a minorityof reports on Alport syndrome. In the large Utah kindreds there were numerous offspring of affected males but no male to male transmission when stringent diagnostic criteria were applied. Classical genetic analysis and likelihood analysis established X-linkage in several kindreds. X-linkage was proven for various kindreds with types II, III, and IV Alport syndrome by findings of close genetic linkage of the Alport locus ATS to restriction fragment length polymorphic markers in or near the Xq22 chromosomal region. Genetic linkage of nephritis with mutations in the COL4A5 gene proves X-linkage in some of the same and other kindreds. Similar linkage to highly polymorphic microsatellite markers within the COL4A5 proves X-linkage in still other families. [X-linkage characterizes roughly 85% of Alport families.][Autosomal Recessive Inheritance characterizes about 15% of Alport families. Regardless of gender, the full array of symptoms are suffered not only by homozygotes of COL4A3 or -4 mutations, but also by double heterozygotes of the COL4A3 and -4 genes. It is becoming clear that heterozygotes of either COL4A3 or COL4A5 may show some but decreased symptoms.]Autosomal Dominant Inheritance. Male to male transmission established autosomal dominant inheritance of Alport syndrome in kindreds which may be categorized as having types V or VI Alport syndrome. [Autosomal dominance characterizes roughly 1% of Alport families. Some but not all of them have mutations of COL4A3 or COL4A4 genes.]---------------------------------------------------------------------GENETIC COUNSELINGDominant inheritance of Alport syndrome may be assumed even with minimal pedigree information. As a group, males with Alport syndrome have about 30% fewer children than normal; many males with juvenile type disease will have no offspring.Incomplete penetrance of Alport syndrome in females must always be kept in mind. In kindreds with X-linkage, daughters of affected males will all be gene carriers regardless of their urinalysis results. Unless there is information from genetic markers or from urinalyses of the next generation, each clinically normal daughter of the three other sorts of gene carrier parents (mothers in kindreds with X-linkage, andparents of either sex in kindreds with autosomal dominance) stands a real probability of having an undetected nephritis gene.---------------------------------------------------------------------CLINICAL FEATURES, TYPES I-VIRenal Symptoms. Hematuria is the cardinal feature, persistent and present from birth in males and in 80-90% of females who have a nephritis gene. The child's mother may note occasionally or persistently red diapers, but hematuria is usually inconspicuous in adult-type disease. Episodes of gross hematuria may follow sore throats or other infections in children and may be the presenting symptom. Macroscopic hematuria is not common in adults and perhaps a feature of juvenile types of disease. Red cell excretion rate is increased by acute infections and by pregnancy.In adult types of Alport syndrome, renal function is typically normal for years and then wanes inexorably to renal failure. The reciprocal of serum creatinine falls linearly with time during this phase (roughly six years from early to end-staterenal failure in adult-type Alport syndrome); hypertension appears, and worsens as renal function deteriorates. Crescentic glomerulonephritis may occur, especially in juvenile types of Alport syndrome, and be accompanied by rapidly progressive renal failure.With either juvenile or adult type Alport syndrome, renal failure is inevitable for affected males, but few females become uremic, and then generally when elderly. ESRD of females in kindreds with type V Alport syndrome may be as frequent as for the males.Sensorineural Hearing Loss. Kindreds with type IV Alport syndrome and some with indeterminate type Alport syndrome have socially normal hearing, whereas progressive and ultimately profound, bilateral, sensorineural hearing loss distinguishes kindreds with all other types. Patients can be unaware of a high frequency loss that is readily shown by audiometry (hearing test). Hearing loss generally occurs later, less severely, and less frequently in females, although some women and girls may have a profound loss. In some families with Alport syndrome and hearing loss, affected members may have apparently normalhearing even after ESRD, but as a rule those family members without hearing loss have less severe renal disease. In Utah Kindred P with type III Alport syndrome, noticeable hearing loss generally coincides with the onset of renal failure.Ocular Features. Eye defects appear limited to kindreds with juvenile type nephritis with hearing loss. In start contrast to hearing loss, which is common in hereditary nephritis, but not specific for it, anterior lenticonus [protrusion of the substance of the crystalline lens] is uncommon though nearly pathognomonic. All cases of anterior lentinconus reported between 1964 and 1982 have been associated with nephritis and/or hearing loss. Lenticonus is more common in males and is usually, but not invariably, bilateral.---------------------------------------------------------------------DIAGNOSISThe path to the correct diagnosis lies through a carefully extended family history and personal examination of the urinary sediment, specifically for hematuria. The proband will commonly be a child with unexplained hematuria or an adolescentto middle-aged male with ESRD, with a vague history of kidney disease in brothers or relatives on the maternal side. Systematic urinalyses may reveal several relatives with hematuria.Of great interest are the forthcoming genetic methods of diagnosis. It appears that probing with cDNAs from COL4A5 will reveal mutations in equal to or less than 10% of kindreds. Emerging techniques with microsatellite markers within COL4A5, exon scanning, single stranded DNA fragment conformational analyses, etc., should soon provide specific genetic tests for gene-carrier status in most families.---------------------------------------------------------------------TREATMENTNo specific treatment is known to affect the underlying pathological process or to alter the clinical course. Antibiotics, anticoagulants, steroids, and immunosuppressives have wrought no benefit. Control of hypertension is mandated on general grounds and protein restriction may prove to be of value once nephron loss gives rise to hyperfiltration.Management of advancing renal failure is along conventional lines. When terminal uremia occurs, dialysis and transplantation pose no particular problems, although the lack of certain GBM antigens invites a slender risk of de novo anti-GBM nephritis after transplantation. Except for one unconvincing example, the glomerular defect of Alport syndrome has not recurred after transplantation. Particular care must be taken in selection of living donors: meticulous and repeated urinalysis for hematuria is the most important step.Great care should be taken to avoid adding insults from drug ototoxicity to the advancing aural injury. Improvement or stabilization of hearing loss has occasionally been noted after transplantation of Alport patients; others have noted no benefit nor have we. Interpretation of these findings is difficult because dialysis or the uremic state have been held culpable for reversible hearing loss. When hearing loss worsens the patient will become more dependent on lip-reading and other visual cues. We have observed poor to fair success with hearing aids. Visual acuity should be monitored at intervals in those with or at risk of lenticonus and consideration given to early lens extraction and intra-ocular lens implantation. Keepsteroid doses low after transplantation and monitor regularly for cataracts; poor vision is a disproportionate handicap to the deaf.关于奥尔波特综合征患者肾移植后排斥的报告奥尔波特综合征患者移植肾失功的主要原因是慢性同种异体移植物肾病(69%)和急性排斥反应(22%)。
WinPE纯净硬盘版【19M】加强硬盘版【25M】整合外置硬盘版【30M】

WinPE纯净硬盘版【19M】&加强硬盘版【25M】&整合外置硬盘版【30M】纯净版不带其他任何软件加强版和盘版适合朋友门硬盘安装做简单维护也可以装入硬盘安装新系统整合外置硬盘还是用小义的版本,1、这个版本在稳定性上比其他小版本的好,2、我只是将这个版本img映像改成了iso映像文件,添加了部分外置软件3、把启动画面改了下,把黑色小人,改成了老毛pe的启动画面。
4、这个版本输入法用的是智能拼音,我比较习惯。
5、当然不代网络功能和和其他的操作系统常用的功能,这3个版本只适合一般的系统维护和安装关于下载页面的说明:下载地方是我的个人网站,纯粹是为朋友服务的,也为深度分流。
在或下载的东西保证其软件的安全性我网上的任何东西都是“ 仅供网友们交流翻版不究”下载页面:/cgi-bin/wsqbbs/wsqbbs.pl?cx=3&hf=1&hfbm=63316511355V45============================回答几个朋友的问题:1、关于我发的PE 安装卸载,我在9楼已经做了说明2、关于光盘版的问题,个人觉得没有必要在做光盘版了,光盘版我首推老九WinPE 老毛桃修改之撒手不管版(Build 070911),这是最好的了。
在稳定性和兼容性上都好于V2、红叶、雨林等知名PE版本。
(只是个人实践证明)3、关于硬盘版推荐使用我的加强硬盘版【25M】&整合外置硬盘版【30M】,对于不太熟悉PE的朋友。
不会出现c盘不能格式等问题。
4、关于ghost,我写了个简单批处理方便不熟悉ghost的朋友实用。
批处理没有编译,抛砖引玉,欢迎大家修改。
ggghost备份恢复工具做的很好,但想到会用ghost的朋友,就直接用ghost了,何必多此一举。
因此就做了这个简单批处理右键迅雷下载:纯净硬盘版/wsqbbs/usr/63316512023_379.rar加强硬盘版/wsqbbs/usr/63316512646_380.rar(英文版)Two regulations promulgated for implementation is in the party in power for a long time and the rule of law conditions, the implementation of comprehensive strictly strategic plan, implementation in accordance with the rules and discipline to manage the party, strengthen inner-party supervision of major initiatives. The two regulations supporting each other, the < code > adhere to a positive advocate, focusing on morality is of Party members and Party leading cadres can see, enough to get a high standard; < rule > around the party discipline, disciplinary ruler requirements, listed as "negative list, focusing on vertical gauge, draw the party organizations and Party members do not touch the" bottom line ". Here, the main from four square face two party rules of interpretation: the first part introduces two party Revised regulations the necessity and the revision process; the second part is the interpretation of the two fundamental principles of the revision of laws and regulations in the party; the third part introduces two party regulations modified the main changes and needs to grasp several key problems; the fourth part on how to grasp the implementation of the two regulations of the party. < code > and < Regulations > revised the necessity and revised history of the CPC Central Committee the amendment to the Chinese Communist Party members and leading cadres honest politics several guidelines > and < Chinese Communist Party discipline and Punishment Regulations > column 1 by 2015 to strengthenparty laws and regulations focus. Two party regulations revision work lasted a Y ears, pooling the wisdom of the whole party, ideological consensus, draw historical experience, respect for the wisdom of our predecessors, which reflects the unity of inheritance and innovation; follow the correct direction, grasp the limited goals, adhere to the party's leadership, to solve the masses of the people reflect a focus on the problem. The new revision of the < code > and < rule >, reflects the party's 18 and the eighth session of the third, the spirit of the fourth plenary session, reflecting the experience of studying and implementing the General Secretary Xi Jinping series of important speech, reflects the party's eighteen years comprehensive strictly practice. (a) revised two regulations of the party need of < the ICAC guidelines > in < in 1997 Leaders as members of the Communist Party of China clean politics certain criteria (Trial) > based on revised, the promulgation and implementation of January 2010, to strengthen the construction of the contingent of leading cadres play an important role. But with the party to manage the party strictly administering the deepening, has not been able to fully meet the actual needs. Content is too complicated, "eight prohibition, 52 are not allowed to" hard to remember, and also difficult to put into practice; the second is concisely positive advocated by the lack of prohibited provisions excessive, no autonomy requirements; the third is banned terms and discipline law, both with the party discipline, disciplinary regulationsrepeat and Criminal law and other laws and regulations repeat; the fourth is to "clean" the theme is not prominent, not for the existing problems, and is narrow, only needle of county-level leading cadres above. < rule > is in 1997 < Chinese Communist Party disciplinary cases (Trial) > based on revision, in December 2003 the promulgation and implementation, to strengthen the construction of the party play very important role. Along with the development of the situation, which many provisions have been unable to fully meet the comprehensive strictly administering the practice needs. One is Ji law, more than half of the provisions and criminal law and other countries laws and regulations Repetition; two is the political discipline regulations is not prominent, not specific, for violation of the party constitution, damage the authority of Party Constitution of misconduct lack necessary and serious responsibility to pursue; third is the main discipline for the leading cadres, does not cover all Party members. Based on the above situation, need to < the criterion of a clean and honest administration > and < rule > the two is likely to be more relevant regulations first amendment. By revising, really put the authority of Party discipline, the seriousness in the party tree and call up the majority of Party members and cadres of the party constitution of party compasses party consciousness. (II) two party regulations revision process the Central Committee of the Communist Party of China attaches great importance to two regulations revision . Xi Jinping, general books recorded in the FifthPlenary Session of the eighth session of the Central Commission for Discipline Inspection, on the revised regulations < > made clear instructions. According to the central deployment, the Central Commission for Discipline Inspection from 2014 under six months begin study two regulations revision. The Standing Committee of the Central Commission for Discipline Inspection 4 review revised. Comrade Wang Qishan 14 times held a special meeting to study two regulations revision, amendment clarifies the direction, major issues of principle, path and target, respectively held a forum will listen to part of the province (area) secretary of the Party committee, Secretary of the Discipline Inspection Commission, part of the central ministries and state organs DepartmentThe first party committee is mainly responsible for people, views of experts and scholars and grassroots party organizations and Party members. Approved by the Central Committee of the Communist Party of China, on 7 September 2015, the general office of the Central Committee of the Party issued a notice to solicit the provinces (autonomous regions, municipalities) Party, the central ministries and commissions, state ministries and commissions of the Party (party), the General Political Department of the military, every 3 people organization of Party of two regulations revision opinion. Central Commission for Discipline Inspection of extensive solicitation of opinions, careful study, attracting, formed a revised sent reviewers. In October 8 and October 12, Central Committee PoliticalBureau Standing Committee and the Political Bureau of the Central Committee After consideration of the two regulations revised draft. On October 18, the Central Committee of the Communist Party of China formally issued two regulations. Can say, two laws amendment concentrated the wisdom of the whole party, embodies the party. Second, < code > and < Regulations > revision of the basic principles of two party regulations revision work and implement the party's eighteen, ten eight plenary, the spirit of the Fourth Plenary Session of the Eleventh Central Committee and General Secretary Xi Jinping important instructions on the revised < low political criterion > and < Regulations >, highlighting the ruling party characteristics, serious discipline, the discipline quite in front of the law, based on the current, a long-term, advance as a whole, with Bu Xiuding independent < rule > and < rule >. Main principle is: first, adhere to the party constitution to follow. The constitution about discipline and self-discipline required specific, awaken the party constitution of party compasses party consciousness, maintaining the authority of the constitution. General Secretary Xi Jinping pointed out that "no rules, no side round. Party constitution is the fundamental law, the party must follow the general rules. In early 2015 held the eighth session of the Central Commission for Discipline Inspection Fifth Plenary Session of the 16th Central Committee, Xi Jinping again pointed out that constitution is the party must follow the general rules, but also the general rules." the revisionof the < code > and < rule > is Method in adhere to the regulations established for the purpose of combining rule of virtue is to adhere to the party constitution as a fundamental to follow, the constitution authority set up, wake up the party constitution and party rules the sense of discipline, the party constitution about discipline and self-discipline specific requirements. 4 second is to adhere to in accordance with the regulations governing the party and the party. The Party of rule of virtue "de", mainly refers to the party's ideals and beliefs, excellent traditional style. The revised the < code > closely linked to the "self-discipline", insisting on the positive initiative, for all members, highlight the "vital few", emphasized self-discipline, focusing on the morality, and the majority of Party members and the ideological and moral standards. The revised < > Ji method separately, Ji, Ji Y an to Method, as a "negative list", emphasizing the heteronomy, focusing on vertical gauge. Is this one high and one low, a positive reaction, the strict party discipline and practice results transformation for the integration of the whole party to observe moral and discipline requirements, for the majority of Party members and cadres provides benchmarking and ruler. Third, insist on to. In view of the problems existing in the party at the present stage, the main problems of Party members and cadres in the aspect of self-discipline and abide by the discipline to make clearly defined, especially the party's eighteen years strict political discipline and political rules, organization and discipline andto implement the central eight provisions of the spirit against the four winds and other requirements into Disciplinary provisions. Not one pace reachs the designated position, focusing on in line with reality, pragmatic and effective. After the revision of major changes, major changes in the < code > and < rule > modified and needs to grasp several key problems (a) < code > < code > adhere to according to regulations governing the party and party with morals in combination, for at the present stage, the leadership of the party members and cadres and Party members in existing main problems of self-discipline, put forward principles, requirements and specifications, showing Communists noble moral pursuit, reflected at all times and in all over the world ethics from high from low 5 common requirements. One is closely linked to the "self-discipline", removal and no direct relation to the provisions of . the second is adhere to a positive advocate, "eight prohibition" 52 are not allowed to "about the content of the" negative list moved into synchronization amendment < cases >. Three is for all the party members, will apply object from the leadership of the party members and cadres to expand to all Party members, fully embodies the comprehensive strictly required. The fourth is prominent key minority, seize the leadership of the party members and cadres is the key, and put forward higher requirements than the ordinary Party members. Five is to simplify, and strive to achieve concise, easy to understand, easy to remember. The revised < code > is the ruling Party since the first insists ona positive advocate forAll Party members and the self-discipline norms, moral declaration issued to all members of the party and the National People's solemn commitment. > < criterion of a clean and honest administration consists of 4 parts, 18, more than 3600 words. After the revision of the < code >, a total of eight, 281 words, including lead, specification and Party member cadre clean fingered self-discipline norms, etc. Part 3 members low-cost clean and self-discipline, the main contents can be summarized as "four must" "eight code". Lead part, reiterated on ideal and faith, fundamental purpose, the fine traditions and work style, noble sentiments, such as "four must" the principle of requirements, strong tone of self-discipline, The higher request for 6 and supervised tenet, the foothold in permanent Bao the party's advanced nature and purity, to reflect the revised standards requirements. Members of self-discipline norms around the party members how to correctly treat and deal with the "public and private", "cheap and rot" thrifty and extravagance "bitter music", put forward the "four norms". Party leader cadre clean fingered self-discipline norms for the leadership of the party members and cadres of the "vital few", around the "clean politics", from civil servant of the color, the exercise of power, moral integrity, a good family tradition and other aspects of the leadership of the party members and cadres of the "four norms" < > < norm norm. "The Party member's self-discipline norms" and "party members and leading cadre clean fingered self-discipline norms," atotal of eight, collectively referred to as the "eight". "Four must" and "eight" of the content from the party constitution and Party's several generation of leaders, especially Xi Jinping, general secretary of the important discussion, refer to the "three discipline and eight points for attention" statements, and reference some embody the Chinese nation excellent traditional culture essence of epigrams. (2) the revised regulations, the main changes in the revised Regulations > to fully adapt to the strictly requirements, reflects the according to the regulations governing the law of recognition of deepening, the realization of the discipline construction and Jin Ju. < rule > is party a ruler, members of the basic line and follow. And the majority of Party members and cadres of Party organizations at all levels should adhere to the bottom line of thinking, fear discipline, hold the bottom line, as a preventive measure, to keep the party's advanced nature and purity. 1, respect for the constitution, refinement and discipline. Revised < rule > from comprehensive comb physical constitution began, the party constitution and other regulations of the Party of Party organizations and Party discipline requirements refinement, clearly defined in violation of the party constitution will be in accordance with regulations to give the corresponding disciplinary action. The original 10 categories of misconduct, integration specification for political discipline, discipline, honesty and discipline masses Ji Law and discipline and discipline and other six categories, the content of < rule >real return to Party discipline, for the majority of Party members and listed a "negative list. 7 2, highlighting the political discipline and political rules. > < Regulations according to the stage of the discipline of outstanding performance, emphasizing political discipline and political rules, organization and discipline, in opposition to the party's leadership and the party's basic theory, basic line, basic program and basic experience, the basic requirement of behavior made prescribed punishment, increase the cliques, against the organization such as violation of the provisions, to ensure that the central government decrees and the Party of centralized and unified. 3, adhere to strict discipline in the law and discipline In front, Ji separated. Revised < Regulations > adhere to the problem oriented, do Ji separated. Any national law existing content, will not repeat the provisions, the total removal of 79 and criminal law, repeat the content of the public security management punishment law, and other laws and regulations. In the general reiterated that party organizations and Party members must conscientiously accept the party's discipline, die van comply with national laws and regulations; at the same time, to investigate violations of Party members and even criminal behavior of Party discipline and responsibility, > < Regulations distinguish five different conditions, with special provisions were made provisions, so as to realize the connection of Party discipline and state law. 4, reflect Wind building and anti-corruption struggle of the latest achievements. < rule > the party's eighteen yearsimplement the spirit of the central provisions of the eight, against the requirements of the "four winds" and transformation for disciplinary provisions, reflecting the style construction is always on the road, not a gust of wind. In the fight against corruption out of new problems, increase the trading rights, the use of authority relatives profit and other disciplinary terms. Prominent discipline of the masses, the new against the interests of the masses and ignore the demands of the masses and other disciplinary terms and make provisions of the disposition and the destruction of the party's close ties with the masses.Discipline to protect the party's purpose. 8 of these regulations, a total of three series, Chapter 15, 178, more than 24000 words, after the revision of the regulations a total of 3 series, Chapter 11, 133, 17000 words, divided into "general" and "special provisions" and "Supplementary Provisions" Part 3. Among them, add, delete, modify the provisions of the proportion of up to nearly 90%. 1, the general general is divided into five chapters. The first chapter to the regulations of the guiding ideology, principles and scope of application of the provisions, highlight the strengthening of the party constitution consciousness, maintenance the authority of Party Constitution, increase the party organizations and Party members must abide by the party constitution, Y an Centralized centralized, would examine at all levels of the amended provisions implementing and maintaining Party discipline, and consciously accept the party discipline,exemplary compliance with national laws and regulations. The second chapter of discipline concept, disciplinary action types and effects of the regulations, will be a serious warning from the original a year for a year and a half; increase the Party Congress representative, by leaving the party above (including leave probation) punishment, the party organization should be terminated its representative qualification provisions. The third chapter of the disciplinary rules of use prescribed in the discipline rectifying process, non convergence, not close hand classified as severely or heavier punishment. "Discipline straighten "At least eighteen years of five years, these five years is to pay close attention to the provisions of the central eight implementation and anti -" four winds ". The fourth chapter on suspicion of illegal party disciplinary distinguish five different conditions, with special provisions were made provisions, to achieve effective convergence of Party and country 9 method. < rule > the provisions of Article 27, Party organizations in the disciplinary review found that party members have committed embezzlement, bribery, dereliction of duty dereliction of duty and other criminal law act is suspected of committing a crime shall give cancel party posts, probation or expelled from the party. The second is < Regulations > Article 28 the provisions of Party organizations in the disciplinary review But found that party members are stipulated in the criminal law, although not involved in a crime shall be investigated for Party discipline and responsibility shouldbe depending on the specific circumstances shall be given a warning until expelled punishment. This situation and a difference is that the former regulation behavior has been suspected of a crime, the feeling is quite strict, and the latter for the behavior not involving crime, only the objective performance of the provisions of the criminal code of behavior, but the plot is a crime to slightly. < Regulations > the 29 provisions, Party organizations in the discipline review found that party members and other illegal behavior, affect the party's image, the damage to the party, the state and the people's interests, we should depend on the situation Seriousness given disciplinary action. The loss of Party members, seriously damaging the party's image of behavior, should be given expelled from the party. At this article is party member is in violation of the criminal law outside the other illegal acts, such as violates the public security administration punishment law, customs law, financial laws and regulations behavior. The fourth is < cases > Article 32 stipulates, minor party members and the circumstances of the crime, the people's Procuratorate shall make a decision not to initiate a prosecution, or the people's court shall make a conviction and exempted from criminal punishment shall be given within the party is removed from his post, probation or expelled from the party. Party members and crime, sheets were fined in accordance with For acts; the principal Ordinance amended the provisions of the preceding paragraph. This is the new content, in order to achieve Ji method effectiveconvergence. Five is < > the thirty third article 10 of the provisions, the Party member due to an intentional crime is sentenced to criminal law (including probation) sheets or additional deprivation of political rights; due to negligence crime and was sentenced to three years or more (excluding three years) a penalty, shall give expelled punishment. Due to negligence crime is convicted and sentenced to three years (including three years) in prison or be sentenced to public surveillance, detention, shall in general be expelled from the party. For the individual may not be expelled from the party, should control Approval. This is followed and retained the original > < Regulations the provisions of punishment party authorization rules and report to a level party organizations. For is "party members with criminal acts, and by the criminal punishment, generally should be expelled from the party". The fifth chapter of probationary Party member of the discipline and discipline after missing members of the treatment and punishment decisions, such as the implementation of the provisions, clear the related party discipline and punishment decision made after, for duties, wages and other relevant alteration formalities for the longest time. 2, sub sub section will the original regulations of10 categories of acts of violation of discipline integration revised into 6 categories, respectively, in violation of the punishments for acts of political discipline "in violation of discipline behavior of punishment" in violation of integrity of disciplinary action points "of violation punishments for actsof mass discipline" "the violation of work discipline, punishment" in violation of discipline of life behavior punishment "6 chapters. 3, annex" Supplementary Provisions "clear authority making supplementary provisions of, cases of interpretative organ, as well as regulations implementation time and retroactivity etc.. 11 (3) learning understanding > < regulations needs to grasp several key problems The first problem -- about the violation of political discipline behavior > < new ordinance chapter 6 the political discipline column for the six disciplines, that is the main opposition to Party leadership and the opposition of the basic theory, basic line, basic program and basic experience, basic requirements of misconduct made provisions of the disposition, especially the eighteen since the CPC Central Committee put forward the Yan Mingzheng treatment of discipline and political rules requirements and practical achievements transformation for Discipline article, increase the false debate central policies, cliques, against the organization review, make no discipline of the principle of harmony terms. These are the party's eighteen years in comprehensive strictly Process combined with the practice of rich content. (1) false debate the central policies and undermine the Party of centralized and unified the problem is made in accordance with the provisions of the party constitution. Constitution in general programme requirements adhere to democratic centralism is one of the requirements of the construction of the party must adhere to the four cardinal. Applicationof this principle is not only the party the basic organization principle and is also the mass line in party life, it requires that we must fully develop inner-party democracy, respect for the dominant position of Party members, safeguarding the Party member democratic rights, give full play to the enthusiasm and creativity of the party organizations at all levels and Party members, at the same time, also must implement the right concentration, ensure the party's mission < the chaos in unity and concerted action to ensure that the party's decision to get quickly and effectively implementing. The Party Central Committee formulated the major principles and policies, through different channels and ways, fully listen to the party organizations and Party members of the opinions and suggestions, but 12 is some people face to face not to say back blather "" will not say, after the meeting said, "" Taiwan does not say, and nonsense ", in fact, not only disrupt the people thought, some causing serious consequences, the damage to the Party of the centralized and unified, hinder the central policy implementation, but also a serious violation of the democratic system of principles. There is no doubt that shall, in accordance with the Regulations > 4 Specified in Article 6 to give the appropriate punishment. For did not cause serious consequences, to give criticism and education or the corresponding tissue processing. (2) about the destruction of the party's unity < New Regulations > the forty eighth to fifty second article, to damage Party's unity unified and violation of political discipline, punishment situationmade explicit provisions. Article 52 of the new "in the party get round group, gangs seek private gain, cliques, cultivate private forces or through the exchange of interests, for their own to create momentum and other activities to gain political capital, given a serious warning or withdraw from their party posts disposition; if the circumstances are serious, to give Leave a party to observation or expelled from the party. (3) on against the organization review of the provisions of the constitution, party loyalty honesty is party members must comply with the obligations. Members must obey the organization decision, shall not violate the organization decided encounters by asking questions to find organization, rely on the organization, shall not deceive the organization, against the organization. For example, after the investigation does not take the initiative to explain the situation, but to engage in offensive and defensive alliance, hiding the stolen money is against survey organization, is a violation of the behavior of political discipline. Article 24 of the original > < Regulations, although the provisions of the interference, hinder group review the behavior of the fabric can be severely or 13 Aggravated punishment, but did not put this kind of behavior alone as a discipline for qualitative amount of discipline. > < new regulations increase the Article 57, "anti organization review, one of the following acts, given a warning or serious warning; if the circumstances are relatively serious, giving removed from or placed on probation within the party post; if the circumstances are serious, give。
骆驼祥子十九章的批注

骆驼祥子十九章的批注(中英文版)Chapter 19 of "Rickshaw Boy" is a pivotal chapter in the novel, as it marks a significant turning point for the protagonist, Xiaoqiang.In this chapter, Xiaoqiang experiences a series of events that shape his character and beliefs.第一十九章是《骆驼祥子》小说中的关键章节,因为它为主人公小强的生活带来了重大转机,一系列事件塑造了他的性格和信仰。
One notable event in this chapter is Xiaoqiang"s encounter with a wealthy businessman who offers him a job.This encounter highlights the stark contrast between the rich and the poor in society, and Xiaoqiang"s reaction to the offer reveals his growing disillusionment with the world.在这一章节中值得注意的是小强与一位富商相遇,这位商人给他提供了一份工作。
这次遭遇突显了社会中贫富之间的鲜明对比,而小强对这份工作的反应则揭示了他对世界的日益失望。
Another important event in this chapter is Xiaoqiang"s encounter with a group of revolutionaries.This encounter exposes him to new ideas and causes him to question his own beliefs.It also serves as a reminder of the political turmoil that exists outside the relatively peaceful world of Xiaoqiang"s rickshaw.在这一章节中另一个重要的事件是小强与一群革命者的相遇。
酒庄中英文名对照

中英文酒庄对照英文名称中文名称国家/产区1 Alianca 阿莲卡酒阁西班牙/圣格欧2 Alois Lageder 拉格德酒园意大利/阿托阿迪3 Alvaro Palacios 阿瓦洛。
帕拉西欧斯酒庄西班牙/普利欧拉多4 Amiral de Beychevelle 龙船将军(或称海军上将,龙船庄副牌)法国/梅多克/圣朱里安5 Antinori 安提诺里酒庄意大利/托斯卡纳6 Astralis 星光园酒(克勒雷登山酒庄旗下品牌)澳大利亚/南河谷7 Aszu Essencia 阿素.艾森西雅酒庄匈牙利/多凯1 Balduzzi 碧桃丝酒庄智利/马利山谷2 Banfi 班菲酒庄意大利/托斯卡纳3 Beringer Vineyards - California 贝灵哲庄园- 加州系列美国/加州/纳帕谷4 Beringer Vineyards - Classic 贝灵哲庄园—经典系列美国/加州/纳帕谷5 Beringer Vineyards — Founders' Estate 贝灵哲庄园—创始者庄园美国/加州/纳帕谷6 Beringer Vineyards - Private Reserve 贝灵哲庄园—庄园特选美国/加州/纳帕谷7 Beringer Vineyards — Stone Cellar 贝灵哲庄园—史东酒窖美国/加州/纳帕谷8 Beringer Vineyards —酒名以产区开头贝灵哲庄园—产区系列美国/加州/纳帕谷9 Bernkasteler Doctor 柏恩卡斯特医生酒园德国/莫索尔10 Biondi Santi 贝昂地酒庄意大利/托斯卡纳11 Blason Timberlay 宝森添百利古堡法国/波尔多12 Bodegas Chivite - Gran Feudo 朱丽安史威特酒庄- 格兰富都系列西班牙/那瓦拉13 Bollinger 柏林格香槟法国/香槟区14 Bricco Roche 罗西峰顶(杰乐托酒庄旗下品牌) 意大利/皮尔蒙特15 Bridgewater Mill 葡萄之路公司(Petaluma) - 水桥酒坊系列澳大利亚16 Brown Brothers 布琅兄弟酒庄澳大利亚/维多利亚1 Cakebread Cellars 卡布瑞酒窖美国/加州/纳帕谷2 Calama 卡拉玛酒庄智利3 Calina 卡利纳酒庄(美国肯德.杰克逊集团成员) 智利4 Cambria - Julia’s Vineyard 坎布瑞—朱丽亚葡萄园美国/圣玛丽亚谷5 Cambria —Katherine’s Vineyard 坎布瑞—凯瑟琳葡萄园美国/圣玛丽亚谷6 Campagnola 坎帕尼奥拉酒园意大利/沃云纳7 Cardinale 卡迪娜美国/纳帕谷8 Carpene Malvolti 卡玛酒园意大利/维纳图9 Carruades de Lafite 拉菲卡罗德(拉菲城堡副牌) 法国/波尔多/波雅克10 Casa Lapostolle 卡莎.拉博丝特酒庄智利11 Castello Di Fonterutoli 风都城堡(马泽世家旗下) 意大利/托斯卡纳12 Caymus — Conundrum 佳慕酒园—谜园美国/加州/纳帕谷13 Caymus - Mer Soleil 佳慕酒园- 海洋之光美国/加州/纳帕谷14 Caymus - Special Selection 佳慕酒园- 特酿系列美国/加州/纳帕谷15 Caymus Vineyards 开木斯酒园美国/加州/纳帕谷16 Ch。
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© Copyright 1992–2004 by Deitel & Associates, Inc. and Pearson Education Inc. All Rights Reserved.
19.4 Casting Base-Class Pointers to DerivedClass Pointere programs in a general fashion – Handle a wide variety of existing (and unspecified) related classes
• Derived class
– Class that inherits data members and member functions from a previously defined base class
Loan Employee Account
Fig. 19.1
Some simple inheritance examples.
© Copyright 1992–2004 by Deitel & Associates, Inc. and Pearson Education Inc. All Rights Reserved.
objects (focus of this chapter) • private: Derived objects are inaccessible by the base class • protected: Derived classes and friends can access protected members of the base class
Base class
Student Shape
Derived classes
GraduateStudent UndergraduateStudent Circle Triangle Rectangle CarLoan HomeImprovementLoan MortgageLoan FacultyMember StaffMember CheckingAccount SavingsAccount
class
© Copyright 1992–2004 by Deitel & Associates, Inc. and Pearson Education Inc. All Rights Reserved.
19.3 Protected Members
• protected inheritance
– Intermediate level of protection between public and private inheritance – Derived-class members can refer to public and protected members of the base class simply by using the member names – Note that protected data “breaks” encapsulation
© Copyright 1992–2004 by Deitel & Associates, Inc. and Pearson Education Inc. All Rights Reserved.
19.2 Base and Derived Classes • Implementation of public inheritance
Chapter 19 - C++ Inheritance
Outline 19.1 19.2 19.3 19.4 19.5 19.6 19.7 19.8 19.9 19.10 19.11 19.12 19.13 19.14 Introduction Inheritance: Base Classes and Derived Classes Protected Members Casting Base-Class Pointers to Derived-Class Pointers Using Member Functions Overriding Base-Class Members in a Derived Class Public, Protected and Private Inheritance Direct Base Classes and Indirect Base Classes Using Constructors and Destructors in Derived Classes Implicit Derived-Class Object to Base-Class Object Conversion Software Engineering with Inheritance Composition vs. Inheritance Uses A and Knows A Relationships Case Study: Point, Circle, Cylinder
– Can be treated as an object of the base class – Reverse not true - base class objects not a derived-class object
• Downcasting a pointer
– Use an explicit cast to convert a base-class pointer to a derived-class pointer – Be sure that the type of the pointer matches the type of object to which the pointer points
– Multiple Inheritance
• Class inherits from multiple base classes
– Three types of inheritance:
• public: Derived objects are accessible by the base class
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21
// Fig. 19.4: point.h // Definition of class Point #ifndef POINT_H #define POINT_H #include <iostream> using std::ostream; class Point { friend ostream &operator<<( ostream &, const Point & ); public: Point( int = 0, int = 0 ); // default constructor int, void setPoint( int, int ); // set coordinates int getX() const { return x; } // get x coordinate int getY() const { return y; } // get y coordinate protected: // accessible by derived classes int x, y; // x and y coordinates of the Point }; // end class Point #endif
class CommissionWorker : public Employee { ... };
Class CommissionWorker inherits from class Employee – friend functions not inherited – private members of base class not accessible from derived
– Circle class derived from the Point base class
– We use pointer of type Point to reference a Circle object, and vice-versa
© Copyright 1992–2004 by Deitel & Associates, Inc. and Pearson Education Inc. All Rights Reserved.
Outline
point.h
© Copyright 1992–2004 by Deitel & Associates, Inc. and Pearson Education Inc. All Rights Reserved.
© Copyright 1992–2004 by Deitel & Associates, Inc. and Pearson Education Inc. All Rights Reserved.
19.1 • Inheritance
– Single Inheritance
Introduction
• Class inherits from one base class
19.2 Base and Derived Classes
© Copyright 1992–2004 by Deitel & Associates, Inc. and Pearson Education Inc. All Rights Reserved.
19.2 Base and Derived Classes
© Copyright 1992–2004 by Deitel & Associates, Inc. and Pearson Education Inc. All Rights Reserved.