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Сряда, Февруари 19, 2025


羊疣龛鲟 磬 孰桧桕囹� 镱 腻蝰赅 绵耱痤屙蝈痤腩汨�



     10th Congres of the European
     Society for Organ Transplantation

                                                                               
                                                                                             
                                                                                             

Transplantation The Official Journal of the

German Transplant Law
EUROTRANSPLANT
Euroliver Foundation

   


衔屡着 抢 着型文形镣酪� 倚劳严死鸵乐冗



    

                                                                                                                        

掩内衅劳扰:

族痦钿痤犴� 囗囹铎� � 蝠囗耧豚眚圉�
软滂赅鲨� � 觐眚疣桧滂赅鲨�
项溽铕 � 铞屙赅 磬 赅礓桎囹栩� 玎 蝠囗耧豚眚圉�
物屦圉�
央邃铒屦囹桠眍 镱忮溴龛�
朽眄� 耠邃铒屦囹桠龛 篑腩骓屙�
竖耥� 耠邃铒屦囹桠龛 篑腩骓屙� (铖蝠� � 躔铐梓眍 铗踱�礤 )
软翦牿梃 � 耠邃铒屦囹桠龛� 镥痂钿
骤觌铖镱痂�, FK- 506, CellCept�
酿筱� 耠邃蝠囗耧豚眚圉桀眄� 镳钺脲扈
�觐� 尻耧屦桁屙蜞腠� 疣琊噌铗觇
橡梓桧� 玎 犏耥囹� 耠邃蝠囗耧豚眚圉桀眄� 耢蝽铖�
褥箜桤圉梃, 理蜩徼铗梓磬 镳铘桦嚓蜩赅
CMV � 蝠囗耧豚眚圉�
EBV � 蝠囗耧豚眚圉�

项皴蝈蝈 晕椅美伺腥咭� 磬 麇痦钿痤犴� 蝠囗耧豚眚桊囗栩� 狷脬囵耜� 溴鲟

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   �痱铗铒眍蝾 镳桉噫溧礤 磬 �� 麇疱� 漯钺 � 觐疱祉囹� 牦蹊磬 磬 恸琊囫蝈� 忮麇 � 痼蜩眄� 镳嚓蜩赅. 翌 钺圜� � 蝠箐眍 疱嚯桤箦祛 � 聱镳钼钿屙� � 祉钽� 镱耱铒屦囹桠龛 篑腩骓屙� 镳� 犏痨圜弪囹� � 镱- 爨腙栩� 溴鲟. � 聱蝾 怵屐� 镳� 篑腩忤�蜞 磬 狷痃� 疣琊囫蜮帙囹� 皴 蝠囗耧豚眚铍钽�, 蝠箫龛�� 麇疱� 漯钺 耱噔� 怦� 镱忮麇 溴翳鲨蝈�. � 祛戾眚� 眢驿栩� 铗 镱漉钿� 玎 蝠囗耧豚眚圉� 铕汔龛 � 牙� � 犭桤� 10 嵇蜩 镱- 泐�爨 铗 磬腓麒弪� 桁.
  茵囗耧豚眚桊囗弪� 磬 镳桉噤赅 � 磬爨脲龛 疣珈屦� � 徉玷疣眍 磬 祛溴痦桤桊囗栩� 蝈蹴桕� 磬 麇痦钿痤犴囹� 蹊痼疸�, 赅牝� � 磬 镱 - 漕狃铗� 铒铉磬忄礤 磬 麇痦钿痤犴囹� 囗囹铎�. 项耠邃磬蜞 镱玮铍�忄 铗 邃桧 麇疱� 漯钺 溧 皴 桤镱腌筲囹 蝠� 疣珉梓龛 皴沆屙蜞- 脲忤�� 麇痦钿痤徨� 腩�, 礤泐� �� 豚蝈疣脲� 皴沆屙� � 铖蜞忄蜞 鬣耱 铗 麇痦� 漯钺 -溴皴� 腩� � 戾滂嚯屙 皴沆屙� 铗 脲忤� 腩�. ( 翳�. 1 � 2 )


澡�. 1    彦沆屙蝽� 囗囹铎� 磬 麇痦� 漯钺 镱 Couinau - 隋忤�� 麇痦钿痤徨� �� 皴 聱耱铊 铗 皴沆屙蜩 2, 3, 4, � 溴耥�� - 铗 皴沆屙蜩 5, 6, 7, 8.

   


澡�. 2    满珈铈眍耱� 玎 觐眈蝠箬疣礤 磬 3 麇痦钿痤犴� 镳桉噤觇 � 磬爨脲龛 疣珈屦�: 溴皴� 腩�, �� 腩�, �� 豚蝈疣脲� 腩�, 觐轵� 祛驽 溧 狷溴 漕嵇腠屙 聱� 皴沆屙�, 铖桡箴�忄� 聱漕忮 � 徼腓囵屙 漯屙噫. IVC- v. cava inf., PV - v. porte, H� - a. hepaica, LHV- �忄 麇痦钿痤犴� 忮磬, BD- 骐屙 赅磬�.
  

务眍忭铗� 钽疣龛麒龛� 玎 桤恸瘌忄礤 磬 蝠囗耧豚眚圉� � 麇疱� 漯钺 铗 骅� 漕眍� 镳� 恸琊囫蝽栩� � 羿牝, 麇 � 钺屐� 耔 忡弪囹� 镳桉噤赅 礤 徼忄 溧 镳梓桧�忄 痂耜 玎 骅忸蜞 磬 漕眍疣 � � 聱蝾 怵屐� 溧 礤 狷溴 镳尻嚯屙� 爨腙� 玎 篑镥� 趔黻鲨铐桊囗� 镳� 疱鲨镨屙蜞. 扬铕邃 H. Bismuth 忡屐囗弪� 磬 75- 80 % 铗 麇痦� 漯钺 礤 眍耔 痂耜 玎 漕眍疣. �觐� 镳铙麾囗� 镱赅玮囹, 麇 镱- 爨膪� 疣珈屦 磬 镳桉噤赅蜞 � 忸溴� 羿牝铕 玎 腩 耠邃铒屦囹桠磬 趔黻鲨�. 令腠�, 镱塍鬣忄 镳桉噤赅, 镱- 爨腙� � 50 % 铗 耦狁蜮屙� 桁 麇疱� 漯钺,  桁囹 磬�- 爨腙� 镳艴钿龛 趔黻鲨铐嚯龛 磬痼龛�. 橡桉噤赅 � 疣珈屦� 镱- 爨腙� 铗 25 %, 钺梓嚅眍 �爨 眈钼� 玎 镳钿骅蝈腠� 趔黻鲨�. 橡� 铒疱溴�礤 磬 桎遴腠� 玎 怦尻� 犷脲� 钺屐 磬 镳桉噤赅蜞, 皴 桤镱腌筲� 箴噔礤龛弪� 磬 Urata ( Urata K. et all.. Calculation of child and adult standard liver volume for liver transplantation. Hepatology, 1995, May, 21: 1317- 1321 ).

k 聱潸疰囗桢蝾

软滂赅鲨� 玎 麇痦钿痤犴� 蝠囗耧豚眚圉�

  橡彗 1993 �. � 相痂� � 镱耱桡磬� 觐眈屙耋� 铗眍耥� 桧滂赅鲨栩� � 觐眚疣桧滂赅鲨栩� 玎 蝠囗耧豚眚圉�.
枢蝾 噌耦膻蝽� 觐眚疣桧滂赅鲨� 皴 镳桢爨�:
1. 义骊� 桤恸眵屦眍漯钺龛 铕汔眄� 磬痼龛�.
2. 湾镱潆彐帙 磬 觐眚痤� 桁箜屙 溴翳鲨�.
3. 湾镱潆彐帙� 磬 觐眚痤� 桤恸眵屦眍漯钺磬 桧翦牿�.
竖� �� �觐� 鲥眚痤忮 镳栳噔�� 桤恸眵屦眍漯钺龛蝈 赅瘀桧铎�, 躔铐梓龛� � 蹂镟蜩�, 嚓蜩忭� 磬痍铎囗�, 蝈骊� 囗囹铎梓龛 囗铎嚯梃.
枢蝾 铗眍耔蝈腠� 觐眚疣桧滂赅鲨� 皴 镱耦麾囹:
1. 袜镳邃磬豚 爨腠篁痂鲨�.
2. 龙狃鬻磬 礤漕耱囹眍耱.
3. 如恸眵屦眍漯钺龛 桦� 蹂镟蝾徼腓囵龛 桧翦牿梃 ( 铖钺屙� 忭桁囗桢 皴 钺瘊 磬 耔耱屐龛蝈 忤痼耥� 桦� 泮徼黜�, 囫鲥礓桊帙 躅豚磴栩 镳� 徼腓囵磬 囹疱玷� ).
4. 茵铎犷玎 磬 v. porte.
5. 砧痼疸梓屙 镱痱铌噔嚯屙 眚.
6. 屿屦屙� 聱痄鬻磬 礤漕耱囹眍耱.
7. 震铐梓磬 � 礤钼豚�忄 皴 屙鲥羿腩镟蜩�.
8. 袜腓麒� 磬 嚯蝈痦囹桠眍 脲麇龛� ( M. Wilson, 诣痤玷礤扈� I 蜩� ).
9. 青犷�忄礤, 觐弪� 皴 铟嚓忄 溧 玎皴沩� � 镳桉噤赅蜞 ( 爨腓沩屙�, 躔铐梓磬 � 忤痼耥� 桧翦牿� ).
   橡� 鬣耱 铗 犷腠栩� 篑镥� 蝠囗耧豚眚圉� 皴 疱嚯桤桊� 耠邃 脲麇龛� 磬 镳�忤蝈, 镳桢爨龛 玎 铗眍耔蝈腠� 觐眚疣桧滂赅鲨� - 磬镳. 囗蜩徼铗梓眍 磬 桧翦牿梃蝈, 蝠铎徨牝铎� 桦� jump grafts 镳� 蝠铎犷玎 磬 镱痱嚯磬蜞 忮磬.
   项耦麇龛蝈 觐眚疣桧滂赅鲨� 礤 皴 镳桢爨� 徨琊彗屦忭�, 忄痂疣� 玎 疣珉梓龛蝈 鲥眚痤忮, 赅蝾 漕 桤忮耱磬 耱屣屙 襦 镱澉桧屙� 磬 鲥腓蝈, 镱耱噔屙� 玎 疱嚯桤桊囗� � 疣珉梓龛蝈 镳钽疣扈, 桦� 磬 铒栩眍耱蜞 磬 尻栾栩�.
  湾怵铍钽梓龛蝈 篑腩骓屙�, 镱��忄 皴 � 礤镱耩邃耱忮龛� 镳邃铒屦囹桠屙 镥痂钿, 桤桉赈囹 蝠箐龛 疱龛�. 袜镳桁屦, 桤忮耱眍 �, 麇 镳� 溴鲟蜞 镱痱铖桉蝈祉囹� 屙鲥羿腩镟蜩� � 钺疣蜩祛 聱耱�龛�, 眍 祛琥黜栩� 磬痼龛� 聱镳钼铈溧 4 耱噤栝 磬 麇痦钿痤犴� 觐爨 磬� - 麇耱� 铒铕铟噔囹 牮嚅磬蜞 镳钽眍玎, 礤玎忤耔祛 篑镥� 桤恸瘌屙囹� 蝠囗耧豚眚圉�. 膨� 玎 礤怵铍钽梓磬蜞 觐眈箅蜞鲨� 蝠�徕� 溧 磬镳噔� 铒栩 溧 疣玢疣龛麒 钺疣蜩扈蝈 戾蜞犷腓蝽� 弭尻蜩 磬 麇痦钿铕钺磬蜞 礤漕耱囹眍耱 铗 蝠嚅龛蝈 (铌铐鬣蝈腠� ) 祛琥黜� 筲疱驿囗�, 觐栩� �爨 溧 狷溧� 觐痂汨疣龛 耠邃 蝠囗耧豚眚圉�蜞. � 镱耠邃龛� 耠篦嚅 镱忮麇蝾 蝠囗耧豚眚圉桀眄� 鲥眚痤忮 襦 疱珏疴桊囗� 玎 桤恸瘌忄礤蝾 �.
   青 溴蝰赅蜞 恸琊囫� 觐眙屦屙鲨�蜞 � 相痂� 镳桢爨, 麇 耠邃龛蝈 玎犷�忄龛� 镳邃耱噔�忄� 礤铖镱痂爨 桧滂赅鲨�: 桤恸眵屦眍漯钺磬 徼腓囵磬 囹疱玷� 耠邃 礤篑镥� 镱痱铄眚屦钹磬耱铎铉� 飋 Kasai, 漯筱� 躅脲耱囹梓龛 玎犷�忄龛�, 怅膻麒蝈腠� 犷脲耱蜞 磬 Byler, 怛铕梓磬 磬 1- 囗蜩蝠栾耔眍� 溴翳鲨� 麇痦钿痤犴� 鲨痤玎, 蹊镱镫噻� 磬 恸蝠鬻屦眍漯钺龛蝈 骐龛 嵇蜩, 怵钿屙� 磬痼龛� 磬 戾蜞犷腓珈�, 趔腱桧囗蝽� 镳铗梓帙� 麇痦钿痤犴� 玎犷�忄龛�.
   枢蝾 桧滂赅鲨� 玎 麇痦钿痤犴� 蝠囗耧豚眚圉� 皴 镱耦麾� � 怆铠屙铗� 赅麇耱忸 磬 骅忸� 镳� 麇痦钿痤犴� 玎犷�忄龛�, 觐栩� 祛汔� 溧 �爨� 玎潸腈栩咫眍 羿蜞脲� 牮嚅:
  疹脲耱囹梓龛:
1. 谚脲�, 礤镱怆�忄� 皴 铗 脲麇龛�, 觐驽� 聱疳彐.
2. 暑耱龛 磬痼龛� � 麴嚓蝮痂.
3. 项怛囵� 皴 屣桤钿� 磬 徼腓囵屙 皴锺桉.
4. 蜀囗蝾爨蝾珥� 礤怵铒囹�.
5. 袜痼龛 疣耱彐 � 疣玮栩桢.
  斟镟蝾鲥塍豚痦�:
1. 暑眈屦忄蜩忭� 礤镱怆�忄� 皴 囫鲨�.
2. 彭鲥羿腩镟蜩�.
3. 竖疴屙� 铗 忄痂鲨 磬 躔囗铒痤忸溧.
   膨� � 邃桧 镱 - 痤� 牮 铗 玎犷�忄龛�, 玎 觐栩� � 溴蝰赅 恸琊囫�, � 疱嚯桤桊囗� 麇痦钿痤犴� 蝠囗耧豚眚圉�:

k 聱潸疰囗桢蝾

   绣龛弪� 玎 桤恸瘌忄礤 磬 麇痦钿痤犴� 蝠囗耧豚眚圉� 皴 忡屐� 耠邃 钺聱驿囗弪� 磬: 1. 膨桀腩汨黜� 滂嚆眍玎. 2. 羊屣屙 磬 麇痦钿痤犴� 礤漕耱囹眍耱. 3. 妙脲扈磬 磬 犷腠� � 牮眍-沭箫钼囹� 祗 镳桧噤脲骓铖�. 4. 念耱 漕 � 铗溧脲麇眍耱 磬 漕眍�. 5. 像桴铄祛鲨铐嚯屙 耱囹篑 磬 皴戾轳蜮铗� � 赅麇耱忸蝾 磬 骅忸蜞.
   如犷瘊� 磬 怵屐弪� 玎 桤恸瘌忄礤 磬 蝠囗耧豚眚圉� � 邃磬 铗 磬� - 蝠箐龛蝈 玎溧麒.翌忄 皴 镳邃铒疱溴� 铗 羿牝�, 麇 羼蝈耱忮龛�� 躅� 磬 麇痦钿痤犴栩� 玎犷�忄龛� � 祛戾眚 磬 磬耱忄礤 磬 礤钺疣蜩�, 镳邃耱�� 脲蜞脲� 桤躅�, � 蝠箐眍 镳邃忤滂�. 埋� 铢� �爨 钺忄腓漤� 牮栩屦梃 玎 铞屙赅 磬 蝾玷 祛戾眚. 亦赅 磬镳桁屦, 礤 怦梓觇 觌桧桷桉蜩 镳桢爨�, 麇 聱耱�龛� 赅蝾 蹂镟蝾疱磬脲� 耔礓痤�, 耧铐蜞礤� 徉牝屦栲脲� 镥痂蝾龛� 桦� 桤疣珏磬 觐嚆箅铒囹�, 镳邃耜噻忄� 礤镳屐屙眍 腩� 桤躅� - 疱耧尻蜩忭� 礤钺躅滂祛耱 铗 耧屮磬 蝠囗耧豚眚圉�. 项 镱漕徨� 磬麒� � 疣珉梓龛蝈 蝠囗耠囗蜞鲨铐龛 鲥眚痤忮 疣玢脲驿囹 嚓蜩忭铖蝌� 磬 玎犷�忄礤蝾 赅蝾 羿牝铕, 镳邃铒疱溴�� 桤躅溧 祗. 青 徼腓囵磬蜞 囹疱玷� 镳钽眍玎蜞 祛驽 脲耥� 溧 狷溴 镳邃忤溴磬 � 疣礻栩� 磬 9 - 18 戾皴鲟. 襄漶赅珞屐铖蝌� 镳� 麇痦钿痤犴� 鲨痤玷 � 漯筱� 弪桀腩汨�, � 溧脲� 镱 - 蝠箐磬.
  漾怵屐屙眍 聱耱忮眍 �, 麇 怵屐弪� 玎 镱�忄蜞 磬 镱漉钿�� 蝠箫屙 漕眍� 祛驽 溧 � 镱忮麇 铗 1 泐滂磬, 觐弪� 皴 铗磬� � 磬� - 泐�爨 耱屣屙 玎 犏痨圜弪囹� 桦� 犷腠栩� � 牮磬 沭箫� �. � 镱忮麇蝾 邂痤镥轳觇 鲥眚痤忮 耢蝽铖蝌� 耩邃 鬣赅蝈 蝠箫屙 铕汔� 赅礓桎囹�, � 戾驿� 20 � 30 %. � 聱蝾 怵屐� 玎泱徉蜞 磬 镳孱犭噤噔帙囹� 鬣耱 犷腠� � 嵇疴栩� 3 戾皴鲟 耠邃 蝠囗耧豚眚圉�蜞, � � 镳�赅 怵赅 � 筲咫梓噔帙囹� 皴 � 怵屐弪� 镳弪疣眈镫囗蜞鲨铐磬 祛疳桎眍耱. 如麒耠屙� �, 麇 赅蝾 �腩, 镳栳腓玷蝈腠� 50 % 铗 赅礓桎囹栩� 玎 麇痦钿痤犴� 蝠囗耧豚眚圉� 玎汨忄� 镳邃� 桦� 耠邃 礤� � 疱珞腧囹 磬 磬镳邃磬腩 祉钽铑疸囗眍 筲疱驿囗�. 橡弼屙赅蜞 磬 痂耜� 玎 犷腠� 铗 汶邃磬 蝾麝� 磬 镳�- � 镱耱疣眈镫囗蜞鲨铐磬蜞 镳彐�屐铖�, � 祉钽� 蝠箐磬.
   橡彗 1987 �. Màlatack 镳邃豚汔 蝾麝钼� 耔耱屐�, 觐�蝾 桁� 玎 鲥� 溧 桎屙蜩翳鲨疣 赅礓桎囹栩� � 磬� - 爨膪� 镳弪疣眈镫囗蜞鲨铐屙 眈 玎 镳彐桠�忄礤, 疱耧尻蜩忭� 眢驿噱 皴 铗 耠邃忄� 漕耱屙 漕眍瘃觇 麇疱� 漯钺. 亦玷 蝾麝钼� 耔耱屐� � 镳邃耱噔屙� 磬 耠邃忄蜞 蜞犭桷�:

脏牝铕� � 蝾麝栩�, 觐栩� 蝈 眍��:
-礼鲨� -   15 �.
-软滂疱牝屙 徼腓痼徼� > 6 煦 % -    13 �.
-软滂疱牝屙 徼腓痼徼� 3- 6 煦 %-    11 �.
-行� > 20 皴�.-    10�.
橡桀痂蝈�:
丸聱� : 镳� 磬狃囗 狃铋 蝾麝� 0 � 28
佯邃屙: 镳� 磬狃囗 狃铋 蝾麝� 29 � 39
妈耦� : 镳� 磬狃囗 狃铋 蝾麝� 40 桦� 镱忮麇

   袜� - 皴痂铉屙 礤漕耱囹 磬 蝾麝钼囹� 耔耱屐� 磬 Malatack � 羿牝, 麇 � 礤 铗麒蜞 恸珈铈眍耱蜞, 疣珉梓龛蝈 弪桀腩汨黜� 邃桧桷� 溧 桁囹 耦狁蜮屙� 桧滂赅鲨� 玎 蝠囗耧豚眚圉�.
   � 耱疱戾驵 溧 皴 磬戾痂 钺尻蜩忮�, 钺忄腓溴� 牮栩屦栝, 觐轵� 溧 铒蜩扈玷疣 祛戾眚� 玎 桤恸瘌忄礤 磬 麇痦钿痤犴� 蜞眈镫囗蜞鲨�, 襦 桤镨蜞龛 恸珈铈眍耱栩� � 磬 �觐� 豚犷疣蝾痦� 蝈耱钼� ( 铒疱溴�礤 磬 觐翦桧钼� 觌桊� � 耠赅, 觌桊� 磬 桧漕鲨囗钼� 珏脲眍 ). 义 桁囹 铒疱溴脲磬 桧纛痨囹桠磬 耱铋眍耱, 眍 镱赅玮囹 � 礤漕耱囹�. 袜镳桁屦, 蝾黜铖蝌� 磬 疱珞腧囹栩� 铗 觐翦桧钼� 蝈耱 皴 镱怆�忄 礤汔蜩忭� 铗 龛忄蜞 磬 徼腓痼徼磬 � 牮蜞.
   Ollerich 镱赅玮�, 麇 蹂镟蝾鲥塍豚痦�� 戾蜞犷腓琥� 磬 腓漕赅桧�, 觐轵� � 耔腠� 玎忤耔� 铗 耱屣屙蜞 磬 麇痦钿痤犴� 牮铗铌, 祛驽 溧 镳邃耜噫� 120 漤邂磬 镳彐桠�屐铖� 镳� 鲨痤玎. 物疱溴�礤蝾 磬 觌桊襦 磬 疣玢疣漤� 镳钿箨� 磬 腓漕赅桧� - monoetylglicinexylidide (MEGX 蝈耱) 皴 镳桦嚆� 痼蜩眄� � 泐脲扈蝈 蝠囗耧豚蜞鲨铐龛 鲥眚痤忮 磬 缅痨囗�, 眍 玎 泐�爨 鬣耱 铗 蝠囗耧豚眚铍铉栩� � 漯筱� 鬣耱� 磬 疋弪�, 镳邃耱铊 礤泐忸蝾 觌桧梓眍 篁恸疰溧忄礤.
   袜蝠箫囗�� 耦狁蜮屙 铒栩 溧忄 铖眍忄龛� 磬 �觐� 蝠囗耧豚眚圉桀眄� 鲥眚痤忮 溧 镳邃镱麒蜞� 疋铊, 镱忮麇 觌桧梓眍 徉玷疣龛 牮栩屦梃 玎 桤恸瘌忄礤 磬 蜞眈镫囗蜞鲨� � 铒蜩爨腠� 怵屐�. 青 铗溴腠� 弪桀腩汨黜� 邃桧桷�, 蝈 桤汶彐溧� 蜞赅:

凌腓囵磬 囹疱玷�
   项漉钿 犏� 礤� � 觐祜脲犟屙 � 怅膻麾�:
1. 满珈铈眍 磬� - 疣眄� 疱嚯桤桊囗� 磬 镱痱�-屙蝈痤囗囫蝾祛玎 镱 Kasai.
2. 族痦钿痤犴� 蝠囗耧豚眚圉� � 耠篦嚅, 麇 铒屦圉�蜞 磬 Kasai 礤 漕忮驿� 漕 桤麇玮囗� 磬 桕蝈疣, 桦� 皴 镱��� 篑腩骓屙� 磬 鲨痤玎蜞.
项磬耱�� 疱珞腧囹栩� 铗 镱痱�-屙蝈痤囗囫蝾祛玎蜞 镱 Kasai 皴 铞屙�忄� 蜞赅:

橡� 溴鲟蜞 � 徼腓囵磬 囹疱玷�, 镳弪镥腓 镱痱�-屙蝈痤囗囫蝾祛玎, 皴 钺瘊 铖钺屙� 忭桁囗桢 磬 恸珈铈眍耱蜞 玎 疣玮栩桢 磬 囫鲥礓桊帙 躅豚磴栩 - 戾驿� 铗溴腠栩� 屣桤钿� 磬 钺铖蝠�礤, 麇痦钿痤犴囹� 趔黻鲨� 祛驽 溧 � 眍痨嚯磬, 眍 镱痱嚯磬蜞 蹊镥痱铐� 镳钽疱耔疣. 湾钺躅滂祛 � 礤镳尻磬蝾 艴钽疣赳觐 耠邃屙� 玎 骐磬 耱噻� 铌铍� �耱铗� 磬 囗囫蝾祛玎, 赅牝� � 玎 纛痨桊囗� 磬 噌聆羼� ad portam hepatis. 洛眍珥� 镳桦嚆囗栩� 囗蜩徼铗桷� 桁囹 怵屐屙屙 篑镥�. 配桧耱忮眍 疣滂赅腠� 疱龛� � 麇痦钿痤犴� 蝠囗耧豚眚圉�.

谚礓痤� 磬 Alagille
   令腠栩� �潢� 漕耱桡囹 耱噤栝 磬 溴觐祜屙耔疣磬 麇痦钿痤犴� 趔黻鲨� 桦� 镱痱嚯磬 蹊镥痱铐�. 务眍忮� 镳钺脲� � 躅脲耱噻囹�. 湾轫栩� 篑腩骓屙�, 赅蝾 礤漕耱囹屙 瘊耱钼 镳桊囫�, 礤蝥痫桁 聱疳彐, 觐耱龛 麴嚓蝮痂, 祛汔� 溧 镱牮�� 牮栩屦梃蝈 玎 怆铠屙� 赅麇耱忸 磬 骅忸�, � 溧 镱耱噔�� 恸镳铖� 玎 麇痦钿痤犴� 蝠囗耧豚眚圉�. 袜� - 麇耱囹� 聱镳钼铈溧 聱痄鬻磬 囗铎嚯� 襦 镥痂翦痦栩� 矬腱铐嚯龛 耱屙铉�. 暑汔蝾 �耥铌囔屦眍蝾 磬�汔礤 � 珥圜栩咫眍 镱忤眍, 耢蝽铖蝌� 镱 怵屐� 磬 蝠囗耧豚眚圉�, � 祉钽� 镱 - 忤耦赅 铗 钺梓嚅磬蜞 - > 58 %. 洛痤�蝽� 镳梓桧� 玎 蝾忄 �, 麇 聱瘀弪� 礤 � � 聱耱�龛� 溧 镱忤 桤螂囫赈帙囹� 耔 麴嚓鲨� 镱 怵屐� 磬 麇痦钿痤犴囹� " 疱镥痿箸� ". 橡噔�� 皴 铒栩� 玎 桎屙蜩翳鲨疣礤 磬 犷腠栩� � 忤耦� 痂耜 玎 聱痄鬻龛 篑腩骓屙�. 埋� 铢� � 磬腓鲥 疱珏疴桊囗铖� 玎 桤恸瘌忄礤 磬 麇痦钿痤犴� 蝠囗耧豚眚圉� 镳� 耔礓痤爨 磬 Alagille, 觐汔蝾 邃桧耱忮磬蜞 桧滂赅鲨� � 赅麇耱忸蝾 磬 骅忸�.
袜犭屙�蜞 磬 �觐� 噔蝾痂 镱赅玮囹 镱 � 疣珉梓磬, 铗 漕耜铕� 镳桢爨磬蜞 犭嚆铒痂�蝽� 镳钽眍玎 玎 蝈玷 犷腠�. 洛痤�蝽铖蝌� 玎 镳彐桠�屐铖� 漕 19 泐滂� 恸琊囫�, 徨� 溧 皴 桤恸瘌� 麇痦钿痤犴� 蝠囗� 镫囗蜞鲨�, 耧铕邃 �� � 邃忄 50 %.

震铐梓屙 � � � 蹂镟蜩�
   销疴铐圜嚯龛�� 屙蝮耔噻 玎 桤恸瘌忄礤 磬 麇痦钿痤犴� 蝠囗耧豚眚圉� 镳� 犷腠� � 躔铐梓磬 HBV 桧翦牿� � 铛豚溴� 铗 忤耦觇� 镳铞屙� ( 80 - 90 % ) 磬 恸玮疣屐铖蝌� � 恸瘐� 镳桉噤赅蜞. 如蝾黜桕 襦 疱珏疴钹痂蝈 磬 HBV � 漯筱� 铕汔龛 � 镥痂翦痦栩� 祛眍眢觌遴痂. 砧耱铍钽梓磬 磬躅潢� 玎 蹂镟蜩� 皴 铗牮桠� 镳� 镱忮麇蝾 疱鲨镨屙蜩.    袜耜铕� 徨 铒桉囗 � 邃桧 囹栾梓屙 蹊耱铍钽梓屙 翦眍戾�, 桤忮耱屙 赅蝾 翳狃铉桊帙� 鲨蝾腓蜩麇�. 锗疣牝屦桤桊� 皴 聱� 疋瘊蹂犟镳羼� 磬 HBV 镳铗彖龛 � 蹂镟蝾鲨蜩蝈, 桤疣珏� 觌弪屙 铗铌 � 躅脲耱噻� 镳� 扈龛爨腠� 恸顼嚯栩咫龛 脲玷�. 袜躅潢囹� 忭篪噔�, 麇 镳� 麇痦钿痤犴� 蝠囗耧豚眚桊囗栩�, HBV 祛驽 溧 皴 镳邂礤 � 滂疱牝眍 鲨蝾镟蝾沐礤�. 孰桧梓磬蜞 桤�忄 磬 翳狃铉桊帙� - 鲨蝾腓蜩黜�� 翦眍戾� � 蜞玷, 磬 狷痃� 镳钽疱耔疣 麇痦钿痤犴� 礤漕耱囹眍耱 � 疣礻栩� 磬 漤� 漕 戾皴鲨. 隋麇龛弪� � 软蝈痿屦铐 镳邃� 蝠囗耧豚眚圉�蜞 礤 忸滂 漕 铗麇螂桠� 磬爨�忄礤 磬 痂耜� 铗 疱桧翦牿�. �觐� 镳铙麾囗� 镱赅玮囹, 麇 镳桦嚆囗弪� 磬 蹂镟蜩� � 蹊镥痂祗礤� 汶钺箅桧 镱 怵屐�, � 耠邃 蝠囗耧豚眚圉�蜞, 祛驽 珥圜栩咫眍 溧 磬爨腓 痂耜� 铗 疱桧翦牿� 磬 镳桉噤赅蜞. 翌玷 镱漉钿 � 耜, � 桧趔玷疣礤蝾 磬 桁箜龛� 蹂镟蜩� � 汶钺箅桧 � 镱忮麇蝾 耠篦噼, � 镱骅珥屙�. 理蜩忤痼耥栩� 嚆屙蜩 ( vidarabin, ganciclovir, lamivudin ) 磬爨�忄� 耱屣屙蜞 磬 忤痼耥� 疱镫桕圉�, 眍 �潢� 忸�� 漕 弭桕囫磬 皴痤觐礅屦耔�.
相疣漕犟嚯眍, 眍 � 镱 - 龛聱� 痂耜 玎 疱桧翦牿� 襦 犷腠栩� � 趔腱桧囗蝽� 镳铗梓帙 HBV 蹂镟蜩�, 赅牝� � 蝈玷, 桁帙� 觐桧翦牿� � D 忤痼�.
   族耱铗囹� � 戾踵龛珈 磬 疱桧翦牿� 磬 镳桉噤赅蜞 镳� 躔铐梓龛� � 蹂镟蜩� 襦 镱漕犴� � 蝈玷, 磬 躔铐梓磬蜞 � 忤痼耥� 桧翦牿�. 橡� 礤泐 钺圜�, 疱桧翦牝桊囗弪� 磬 镳桉噤赅蜞 镱 - �潢� 忸滂 漕 觌桧梓磬 桤�忄, � 潸脬铖痤黜囹� 镳钽眍玎 � 珥圜栩咫眍 镱 - 漕狃�. 配忄 铌铍� 10% 铗 蝠囗耧豚眚桊囗栩� 镱赅玮囹 徨脲玷 磬 嚓蜩忭� 忤痼耥� 桧翦牿� � 镳艴钿 � 鲨痤玎 桦� 铗踱�礤 � 疣礻栩� 磬 1 - 2 泐滂龛.
添屙�蜞 玎 礤钺躅滂祛耱 铗 镳桦嚆囗� 磬 桧蝈痿屦铐 襦 镳铗桠铕鬻桠�. �觐� 鲥眚痤忮 聱钺忄� 玎 珥圜栩咫眍 镱 - 忤耦赅 麇耱铗� 磬 躔铐梓眍 铗踱�礤 磬 镳桉噤赅蜞 镳� 犷腠栩�, 镳� 觐栩� 耠邃 蝠囗耧豚眚圉�蜞 � 镳桦嚆囗 软蝈痿屦铐.
橡钽眍玷蝈 耦鬣� 躔铐梓磬蜞 � 忤痼耥� 桧翦牿� 赅蝾 忮痤�蝽� 磬� - 麇耱囹� 桧滂赅鲨� 玎 麇痦钿痤犴� 蝠囗耧豚眚圉� � 耠邃忄蝈 10 泐滂龛.

离羿- 1 理蜩蝠栾耔眍� 溴翳鲨�
   袜� - 钺 桧滂赅鲨� � 疣玮栩桢蝾 磬 溴觐祜屙耔疣磬 麇痦钿痤犴� 鲨痤玎, 眍 徨脲玷 赅蝾 镱�忄 磬 囫鲨�, 犏疴屙� 铗 忄痂鲨 磬 躔囗铒痤忸溧, 疱鲨滂忤疣� 桕蝈�, 蝠�徕� 溧 狷溧� 疣玢脲驿囗� 赅蝾 嵇疴� 耔沩嚯 玎 桤恸瘌忄礤 磬 蝠囗耧豚眚圉�.

务蝠� 麇痦钿痤犴� 礤漕耱囹眍耱
   令腠栩� � 镱 - 祀噤� 恸琊囫� 桁囹 镱 - 腩 镳钽眍玎. 橡铗痤灬桧钼� 怵屐�, 箐驽眍 镱忮麇 铗 90 皴�., 桦� INR 磬� 4, 皴 囫铞梃疣� 聱� 耢蝈� 桤躅� 徨� 蝠囗耧豚眚圉� 镳� 92 - 95 % 铗 溴鲟蜞. 绣珞腧囹栩� 铗 蝠囗耧豚眚圉�蜞 礤 襦 蜞赅 漕狃�, 赅牝� � 镳� 耠篦噼蝈 � 躔铐梓磬 麇痦钿痤犴� 礤漕耱囹眍耱. 项忮溴龛弪� 镱 镳噔桦� � 撪沭羼桠眍�, 礤 襦祛 镱 铗眍龛� 镳钼彐溧磬蜞 桧蝈礴桠磬 蝈疣镨�, 眍 � 玎 磬扈疣礤 磬 镱漉钿�� 漕眍�. � 耠篦嚅, 麇 聱耱�龛弪� 磬 犷腠� 皴 镱漕狃� 珥圜栩咫眍, 麇痦钿痤犴囹� 蝠囗耧豚眚圉� 祛驽 � 溧 礤 皴 疱嚯桤桊�.
�觐� 弪桀腩汨黜� 邃桧桷� 溧忄� 镱 - 漕狷� 眈 玎 铉漯噔�忄礤 � 耩噔礤龛� � 漯筱�. 泽腱桧囗蝽�� 蹂镟蜩� � 镱赅玮� 耢蝽铖� 铗 95 %, 嚓� 礤 皴 蝠囗耧豚眚桊�. 青 铖蝠囹� 镟疣鲥蜞祛腩忄 桧蝾犟桕圉� � 趔腱桧囗蝽� 蹂镟蜩� �, 聱镱耱噔屙栩� 疱珞腧囹� 铗 桧蝈礴桠眍 觐眈屦忄蜩忭� 脲麇龛� � 麇痦钿痤犴� 蝠囗耧豚眚圉�, 襦 镳栳腓玷蝈腠� 邃磬赈�. 橡� 镟疣鲥蜞祛腩忸 疋瘊蹁铉桊囗� 镳彐桠�忄礤 祛驽 溧 皴 磬犭噔� 镳� INR > 铗 4. 殷� 镱赅玎蝈腓 � 腩 镳钽眍玎 襦: 屙鲥羿腩镟蜩� 3 � 4 耱噤栝, 狷狃鬻磬 礤漕耱囹眍耱 � 戾蜞犷腓蝽� 嚯赅腩玎. 橡噔� 皴 铒栩 玎 脲麇龛� � 桧蝠噔屙铉屙 N � acetylcystein, � 嚓� 蝾� 礤 漕忮溴 漕 镱漕狃屙桢 耱铋眍耱栩� 磬 INR, 蝾忄 镱漶赅玮� 礤钺躅滂祛耱 铗 蝠囗耧豚眚圉�.
   橡� 趔腱桧囗蝽� 镳铗梓帙栩� 麇痦钿痤犴� 磬痼龛� 皴 镳桢爨� 耠邃龛蝈 牮栩屦梃 玎 桤恸瘌忄礤 磬 麇痦钿痤犴� 蝠囗耧豚眚圉�:

  • 龙痃� 磬爨�忄 疣珈屦� 磬 麇痦� 漯钺.
  • INR >4.
  • 彦痼戾� 徼腓痼徼� > 300 mmol / L.
  • 彭鲥羿腩镟蜩�, 礤玎忤耔祛 铗 耱噤� �.
  • 袜腓麒� 磬 狷狃鬻磬 礤漕耱囹眍耱.

诣痤玷礤扈� I 蜩�
   念 铗牮桠囗弪� 磬 NTBC 2- ( 2- nitro- 4- trifluoro- methylbenzoil ) 1,3- cyclo- hexanedione 蝠囗耧豚眚圉�蜞 � 邃桧耱忮眍蝾 疣滂赅腠� 脲麇犴� 耩邃耱忸. NTBC ( 镳桦嚆囗 � 漕玎 0, 6 煦 / 赉.�. ) � 蜩痤玷� - 觇磬珏� 桧蹊徼蝾� � 磬鬣腠栩� 弪囡� 磬 疣玢疣驿囗弪� 磬 蜩痤玷磬. 翌� 弭尻蜩忭� 觐礅屦蜩疣 蜩痤玷礤扈� I 恸� II 蜩�. 族痦钿痤犴囹� � 狷狃鬻磬 滂耵箜牿�, 赅牝� � 镳�忤蝈 磬 礤怵铗铌耔黜铖�, 皴 镳尻疣�忄�. 埋� 铢� 礤 � �耥� 漕觐腙� 犷腠栩� � 磬镳邃磬腩 玎犷�忄礤 皴 眢驿�� 铗 麇痦钿痤犴� 蝠囗耧豚眚圉� � 溧腓 镳� �� 脲麇龛弪� � NTBC 祛驽 溧 镳邃铗怵囹� 疣玮栩桢蝾 磬 蹂镟蝾鲥塍豚疱� 赅瘀桧铎.

令脲耱 磬 Wilson
   悟 Mowat � 镳邃腩驽磬 蝾麝钼� 镳钽眍耱梓磬 铞屙赅 铗麒蜞 镱赅玎蝈腓蝈 皴痼戾� 徼腓痼徼�, 衙我 � 镳铗痤灬桧钼� 怵屐�.

  • 厌铕 镱� 6 镳钽眍玷疣 嵇腠� 恸珩蜞眍�忄礤 镳� 脲麇龛� � Penicillamine � Zinc.
  • 厌铕 铗 10, 11 桦� 12 铉磬鬣忄 礤桤徨骓� 耢� � 礤钺躅滂祛耱 铗 耧屮磬 蝠囗耧豚眚圉�.
  • 厌铕 戾驿� 6 � 10 镱玮铍�忄 桤鬣赈囹咫眍 镱忮溴龛� � 觐眈屦忄蜩忭� 脲麇龛�.
亦玷 蝾麝钼� 铞屙赅 皴 镳钼彐溧 彐邃礤忭�. 橡� 蝈礓屙鲨� 玎 筲咫梓噔囗� 磬 襻铕� 铗 蝾麝�, 皴 镳桉蝥镡� 犏� 麇痦钿痤犴� 蝠囗耧豚眚圉�.

斟祛羿泐鲨蜞痦� 腓祠铞栩磬 蹊耱桀鲨蝾玎
   茵囗耧豚眚圉�蜞 � 觐眚疣桧滂鲨疣磬, 蝥� 赅蝾 礤 镱怆�忄 铖眍忭铗� 玎犷�忄礤, 觐弪� 囹嚓筲� 镳桉噤赅蜞 镱 聱� 磬麒�, 赅牝� � 耦狁蜮屙� 麇疱� 漯钺.

令脲耱 磬 Nieman- Pick 蜩� �
  族痦钿痤犴囹� 蝠囗耧豚眚圉� 礤 镳邃铗怵囹�忄 镳钽疱耔疣礤蝾 磬 礤怵铍钽梓龛蝈 镳�忤. 矽� 礤 � �耥� 溧腓 觐灬桧桊囗囹� 麇痦钿痤犴� � 觐耱眍祛琥黜� 蝠囗耧豚眚圉� 祛汔� 溧 桁囹 脲麇徨� 弭尻�.

描汔眚铌脲蝥麇� 蹂镟蜩� � Coombs 镱玷蜩忭� 囗屐�
  项忮麇蝾 耠篦噼 镳钽疱耔疣� 犏� 麇痦钿痤犴� 礤漕耱囹眍耱. 如忮耱屙 弭尻� 桁囹 桁箜铖箫疱耦痂蝈. 橡� 怦梓觇 麇痦钿痤犴� 蝠囗耧豚眚桊囗�, 玎犷�忄礤蝾 疱鲨滂忤疣.

令脲耱 磬 Caroli
  务眍忭� 镳梓桧� 玎 磬耱忄礤 磬 麇痦钿痤犴� 礤漕耱囹眍耱, � 桧翦牝桊囗弪� 磬 囗铎嚯磬蜞 恸蝠鬻屦眍漯钺磬 骐磬 耔耱屐�. 殷� 疱龛� 玎 蝠囗耧豚眚圉� 皴 忡屐� 镳� 耠篦噼蝈 � 疱鲨滂忤疣, 礤钼豚�屐� 骐龛 桧翦牿梃 � 皴矧桷屐梃.

腆觐忤聆桎铉�
   侮铍� 40 % 铗 犷腠栩� 镱赅玮囹 麇痦钿痤犴� 恸怆梓囗�, 眍 襦祛 镳� 邃桧桷� 铗 �� 皴 磬犭噔� 溴觐祜屙襦鲨� 磬 麇痦钿痤犴囹� 趔黻鲨�. 翌玷 羿牝, 赅牝� � 痂耜钼弪� 铗 镳钿骅蝈腠铗� 桁箜铖箫疱耔忭� 脲麇龛� 耠邃 麇痦钿痤犴� 蝠囗耧豚眚圉�, 磬豚汔� 沭桄腓忸 钺扈耠�礤 磬 礤钺躅滂祛耱蜞 铗 礤�. 青 蝾忄 玎犷�忄礤 怦� 铢� �爨 篁铟礤龛 桧滂赅鲨�, 赅蝾 镱 - 耜铕� 皴 钺瘊 忭桁囗桢 磬 恸珈铈眍耱蜞 玎 镳邃铗怵囹�忄礤 镱耠邃耱忤�蜞 磬 镱痱嚯磬蜞 蹊镥痱铐� � 蝠囗耧豚眚圉�蜞, 铗觐腙铗� 磬 麇痦钿痤犴囹� 趔黻鲨�. �觐� 鲥眚痤忮 聱钺忄� 玎 漕狷� 弭尻� 铗 麇痦钿痤犴囹� 蝠囗耧豚眚圉� 镱 铗眍龛� 钺蝾 聱耱�龛� � 徨腩漯钺龛� 耱囹篑. 暑灬桧桊囗铗� 蝠囗耧豚眚桊囗� 磬 �� 漯钺, 聱瘀� � 麇疱� 漯钺, � 怦� 铢� 忤耦觐 - 痂耜钼�.

斟镟蝾犭囫蝾�
   楞疱耔忭囹� 蹊扈铗屦囡� � 疱珏牿�蜞 磬 蝮祛疣 铖桡箴�忄� 5 泐滂� 镳彐桠�屐铖� 镳� 60 % 铗 犷腠栩�. 族痦钿痤犴� 蝠囗耧豚眚圉� 皴 镳桦嚆�, 觐汔蝾 礤玎忤耔祛 铗 泐痦铗� 脲麇龛�, 蝮祛瘊� 镳钿驵忄 溧 桧翳腧痂疣 爨耔忭� 麇痦� 漯钺, 骐磬蜞 桦� 镱痱嚯磬蜞 耔耱屐�.

斟镟蝾鲥塍豚疱� 赅瘀桧铎
   项漉钿� 玎 麇痦钿痤犴� 蝠囗耧豚眚圉� 襦 蝮祛痂蝈 � 疣珈屦� 漕 5 耢.

棱蝾桁箜屙 蹂镟蜩�
   青 祛戾眚� 皴 镳邃镱麒蜞 桁箜铖箫疱耔忭铗� 脲麇龛�, 觐弪� 祛驽 溧 铗腩骅 蝠囗耧豚眚圉�蜞 � 泐滂龛. � 耠篦嚅, 麇 � 狷溴 桤恸瘌屙�, 麇耱� 皴 铗麒蜞 镥瘃桉蜩疣礤 磬 耧弼梏梓龛蝈 玎 玎犷�忄礤蝾 噔蝾囗蜩蝈豚. 袜犭屙�蜞 磬 �觐� 鲥眚痤忮 镱赅玮囹, 麇 镳� 26 % 铗 耠篦噼蝈 磬耱忄 疱鲨滂� 磬 噔蝾桁箜龛� 镳铞羼 恸瘐� 嚯钽疣羿. 翌黜囹� 铞屙赅 磬 蝾玷 羿牝 � 蝠箐磬, 蝥� 赅蝾 蹊耱铍钽梓龛蝈 磬躅潢� 镳� 噔蝾桁箜龛� 蹂镟蜩�, 铖蝠铗� 铗踱�礤 磬 镳桉噤赅蜞 � 蹂镟蜩� � 襦 祉钽� 聃钿龛.
   橡� 疱� 戾蜞犷腓蝽� 玎犷�忄龛� 礤 皴 疣玮桠� 麇痦钿痤犴� 筲疱驿囗�, 眍 皴 磬犭噔囹 蝈骊� 铗觌铐屙� 恸� 趔黻鲨�蜞 磬 漯筱� 铕汔龛. 橡桁屦� � 蝾忄 铗眍龛� 襦 耔礓痤禚� 磬 Crigler- Najjar I 蜩�, 铌襦塍痂� I 蜩�, 磬痼龛�蜞 � 鲨犏豚 磬 箴彘龛蝈 觇皴腓龛, 羿扈腠囹� 蹊镥瘐铍羼蝈痤脲扈�, 溴翳鲨� 磬 镳铗彖龛 C , S � anti - trombin 3, 镳铒桀眍忄 � 戾蜩� - 爨腩眍忄蜞 圉桎屐�. 橡� 怦�觐 邃眍 铗 �� 蝠囗耧豚眚圉� 蝠�徕� 溧 皴 桤恸瘌� 镳邃� 纛痨桊囗弪� 磬 溴翳龛蜩忭� 铕汔眄� 镱疣驽龛� 赅蝾 鲥疱狃嚯屙 耧囫蜩鲨蝈� 桦� 汶篚铗�. 橡� 嵇疴梓磬蜞 铌襦塍痂�, 麇痦钿痤犴囹� 蝠囗耧豚眚圉� 祛驽 溧 镳邃铗怵囹� 礤钺躅滂祛耱蜞 铗 狷狃鬻磬 蝠囗耧豚眚圉�, 觐汔蝾 汶铎屦箅磬蜞 翳腧疣鲨� 礤 � 磬爨�豚 镱� 25 - 40 mil / min. 添钽� 麇耱� 镳� 蝾玷 蜩� 玎犷�忄龛� 痤滂蝈腓蝈 蝠箐眍 皴 筢彐溧忄� � 礤钺躅滂祛耱蜞 铗 蝠囗耧豚眚圉�, 觐汔蝾 溴蝈蝾 桁 �爨 蝈牦 镳钺脲扈, 桦� 蝈 怦� 铢� 襦 镱眍耔扈.

  央邃 疣琊噌铗忄礤蝾 磬 蝈蹴桕栩� 玎 镳桉噫溧礤 磬 麇疱� 漯钺 � 磬爨脲龛 疣珈屦�, 羿牝铕 聱怙噤屙桢 镱 疣珈屦� 忮麇 �爨 聱蜞 痤� 镳� 铒疱溴�礤 镳桀痂蝈蜞 玎 疣顼疱溴�礤 磬 铕汔磬, 赅牝� � 扈磬腩蝾. 项� 忭桁囗桢 皴 忡屐� � 缆� 聱怙噤屙桢 磬 牮龛蝈 沭箫� - � 镳邃桁耱忸 襦 犷腠栩�, 麒轵� 牮眍-沭箫钼 蜩� � 桎屙蜩麇� � 蝾玷 磬 漕眍疣. 湾聱忪羼蜩扈 镱 缆� 铕汔龛 皴 镳桉噫溧� 襦祛 � 耧屮龛 耠篦噼. 亦眈镫囗蜩疣礤蝾 磬 缆� 礤聱忪羼蜩� 麇疱� 漯钺 � 镱 - 篑镥� 镳� 犏痨圜弪� � 爨腙� 溴鲟, 赅蝾 痂耜 玎 铗踱�礤蝾 祗 磬疣耱忄 � 恸琊囫蝌�. 青 疣珉桕� 铗 狷狃鬻磬蜞 蝠囗耧豚眚圉�, 镳� 麇痦钿痤犴囹� �爨 漕赅玎蝈腭蜮� 玎 蝾忄, 麇 HLA - 蝥赅磬蜞 礤聱忪羼蜩祛耱 铌噻忄 聱耱忮眍 怆�龛� 恸瘐� 镳彐桠�屐铖蜞 磬 镳桉噤赅蜞 桦� 磬 犷腠�. 捻羼 � 礤 皴 桤镱腌忄 赅蝾 镳邃铒疱溴�� 羿牝铕 玎 桤犷� 磬 漕眍瘃觇 麇疱� 漯钺.
  � 镳邃铒屦囹桠龛� 镥痂钿 铖钺屙� 忭桁囗桢 皴 钺瘊 磬 觐痂汨疣礤蝾 镱耠邃桷栩� 铗 忤蜞扈礤� � " 躔囗栩咫屙 溴翳鲨� ". 羊屣屙蜞 磬 蝾玷 溴翳鲨� 觐疱腓疣 � 蹊痼疸梓龛� 桤躅� 铗 麇痦钿痤犴囹� 蝠囗耧豚眚圉�. 项忤忄 皴 徨腧龛�� 忭铖 黟彗 钺钽囹�忄礤 磬 躔囗囹� � 羼屙鲨嚯龛 囔桧铌桉咫桧� � 蜞觇忄, � 疣珀腩礤龛 忮痂汨; 漕徉�� 皴 耩邃眍-忮痂骓� 蝠桡腓鲥痂滂. 翌忄 � 铖钺屙� 忄骓� 玎 犏痨圜弪囹� � 爨腙栩� 溴鲟 � 潸脬铗疣轫� 躅脲耱噻�. 橡� 磬爨脲� 囡弪栩 皴 磬豚汔 躔囗屙� 黟彗 耦礓�. 暑汔蝾 屙蝈疣腠铗� 躔囗屙� 礤 祛驽 溧 镱耱桡礤 驽豚龛� 篑镥�, 皴 镳屐桧噔� 犏� 镟疱眚屦嚯眍.

  褥箜桤圉梃 - 桤�汊囹 皴 骅忤蝈 � 囹屙疣龛蝈 忄犟桧� � 钽脲� 桤�汊囗� 磬 篑腩骓屙� ( 徉牝屦栲腠� 桦� 忤痼耥� 疱嚓蜩忄鲨� ) 镳� 爨耔忭囹� 桁箜铖箫疱耔� 耠邃 蝠囗耧豚眚圉�蜞. 如�耥�忄礤蝾 磬 皴痤腩汨黜� 耱囹篑 磬 疱鲨镨屙� � 漕眍� 镱 铗眍龛� �觐� 忤痼耥� 桧翦牿梃 ( CMV, HSV, EBV, A, B, C - 蹂镟蜩� ) � 祉钽� 聱耱忮眍. 翌忄 皴 铗磬� � 磬�- 泐�爨 耱屣屙 玎 CMV. � 镱忤� 痂耜 玎 疣玮桠囗� 磬 CMV 桧翦牿� 耠邃 蝠囗耧豚眚圉� 襦 皴痤礤汔蜩忭栩� 疱鲨镨屙蜩, 镱塍麒腓 铕汔� 铗 皴痤镱玷蜩忮� 漕眍�, 蝈玷 蝠弪桊囗� � 囗蜩腓祠铞栩囵屙 汶钺箅桧 � 蝈玷, 镳� 觐栩� 皴 � 磬腩骅豚 疱蝠囗耧豚眚圉�.
湾钺躅滂爨 � �腩耱磬 镳弼屙赅 磬 聱耱�龛弪� 磬 怦梓觇 铕汔龛 � 耔耱屐�.
   橡邃镱麒蜞 皴 漕眍痂蝈 溧 狷溧� 镱- 祀噤� 铗 40 泐滂龛, 眍 � 镱 - 恸琊囫蝽栩� 祛汔� 溧 铖桡箴�� 嚯钽疣� � 漕狃� 趔黻鲨�. 勿汔� 礤 祛驽 溧 狷溴 忡弪 铗 漕眍� � 爨腓沩屙� 玎犷�忄礤 ( � 桤觌屙桢 磬 嵇疴梓龛 祛琥黜� 蝮祛痂), 躔铐梓眍 麇痦钿痤犴� 玎犷�忄礤, HIV � 疱蝠钼桊篑磬 桧翦牿�. 悟眍耔蝈腠� 觐眚疣桧滂赅鲨� 襦 蝈骊� 爨耱磬 滂耱餺翳� 磬 麇痦� 漯钺 � 耔耱屐屙 皴锺桉. 尊彗 Doppler 艴钽疣翳�, 囗汨� - � 忮眍沭圄梃 皴 蝥瘃�� 囗铎嚯梃 ( 汶噔眍 聱漕忤 � 磬 骐磬蜞 耔耱屐� ) 赅牝� 镳� 疱鲨镨屙蜞, 蜞赅 � 镳� 骅忤� 漕眍�.

k 聱潸疰囗桢蝾

物屦圉�- 镳� 漕眍疣 麇痦�� 漯钺 皴 桤忄驿� 聱� 玎镟珏龛 篦囫蝥鲨 ( " 爨眸铐� ") 磬 耋镳�- � 桧蝠圊屣囹嚯磬蜞 漕腠� 镳噻磬 忮磬, 麇痦钿痤犴囹� 囵蝈痂� � 蝠箜牦� 鲥腓嚓篑. 义 襦 礤钺躅滂扈 玎 镱耠邃忄蝾 囗囫蝾祛玷疣礤 聱� 聱漕忮蝈 磬 疱鲨镨屙蜞. 族痦�� 漯钺 皴 镥痿箸桊� � UW 疣珧忸疣 黟彗 赅睨豚 � 镱痱嚯磬蜞 忮磬 � 囝痱囹�. 念 祛戾眚� 磬 蝠囗耧豚眚桊囗弪� 祗, 蝾� 皴 镱潆嚆� 磬 耱箐钼� 桉蹂扈�. 族痦�� 漯钺 磬 疱鲨镨屙蜞 皴 皴鲨疣 � 桤忄驿� 赅蝾 皴 玎镟玮囹 聱漕忮蝈 � 骐龛蝈 嵇蜩 玎 镱耠邃忄蜞 囗囫蝾祛玎. 袜�- 磬镳邃 皴 囗囫蝾祛玷疣� 泐痦� � 漕腠� 镳噻磬 忮磬. 央邃忄 镳铎桠囗� 磬 麇痦� 漯钺 � 疣珧忸� 磬 NaCl, 玎 溧 皴 铗耱疣龛 UW 疣珧忸瘊�, 觐轵� � 犷汔� 磬 � � 豚牝囹. 念恸瘌忄 皴 囗囫蝾祛玷疣礤蝾 磬 镱痱嚯龛蝈 聱漕忮, � 觐弪� 皴 恸珩蜞眍�忄 镥痿箸�蜞 磬 镳桉噤赅蜞. 橡� 徼腓囵磬蜞 疱觐眈蝠箨鲨� 襦 恸珈铈龛 溻� 忄痂囗蜞:

  1. 署嚅 犏� 牮嚅 囗囫蝾祛玷疣礤 磬 骐屙 赅磬� 磬 疱鲨镨屙蜞 � 蜞犏� 磬 漕眍疣, 赅蝾 皴 桤镱腌筲� �. 磬�. � - tube stent. 翌玷 忄痂囗� 麇耱� � 蝠箐眍 铖羼蜮桁 镱疣滂 爨腙栩� 疣珈屦� 磬 钺� 骐屙 镳铗铌. ( 澡� � )
  2. 项 - 麇耱� 皴 镳桦嚆� 囗囫蝾祛玷疣礤蝾 磬 漕眍瘃觇� 骐屙 赅磬� 犏� �. 磬�. Roux� en - Y 狃桁赅, 镳邃忄痂蝈腠� 聱玟噤屙� � 潴钿屙箪�. ( 澡� � )

  澡� � � �

亦赅忄 狃桁赅 钺梓嚅眍 聱玟噔囗� 镳� 铒屦圉�蜞 磬 Kasai, 赅蝾 皴 桤镱腌筲� � 镳� 蝠囗耧豚眚圉�蜞. 橡钿骅蝈腠铖蝌� 磬 蹊痼疸梓羼赅蜞 桧蝈疴屙鲨� 镳� 忡屐囗弪� 磬 镳桉噤赅蜞 铗 漕眍疣 � 220- 390 扈�., 玎泱徉蜞 磬 牮 礤 镱忮麇 铗 600 祀. 吾蝾 桉蹂扈黜� 怵屐� 玎 镳桉噤赅蜞 忄痂疣 戾驿� 155 � 613 扈眢蜩. 吗屐弪� 玎 觌囔矧桊囗� 镳� 疱鲨镨屙蜞 � 28- 62 扈眢蜩; 铗 �� 铌铍� 30 扈眢蜩 襦 礤钺躅滂扈 玎 桤恸瘌忄礤 磬 囗囫蝾祛玷蝈, � 铖蜞磬腓蝈 玎 镳屣囵桊囗� 磬 v. cava � 玎蜮囵�礤蝾 磬 怦梓觇 爨腙� 忮眍珥� 聱漕忮.
湾钺躅滂祛蝾 怵屐� 玎 漕耱桡囗� 磬 眍痨嚯龛 疣珈屦� 磬 镳桉噤赅蜞 耠邃 蝠囗� 镫囗蜞鲨�, � 镳栳腓玷蝈腠� 3 戾皴鲟.

k 聱潸疰囗桢蝾

央邃铒屦囹桠眍 镱忮溴龛�
   央邃 铒屦圉�蜞 犷腠�� 皴 磬耱囗�忄 � 铗溴脲龛� 玎 桧蝈礴桠龛 沭桄�, 犏溴蝾 铖忮� 钺梓嚅龛蝈 玎 蜞觇忄 铗溴脲龛� 磬犭屙� � 桤耠邃忄龛�, 镳� 麇痦钿痤犴� 蝠囗耧豚眚桊囗栩� 皴 桤恸瘌忄 � 耠邃� 耠邃眍蝾:
   青镱麾� 皴 桁箜铖箫疱耔忭� 脲麇龛� � 鲨觌铖镱痂� � 戾蜩腼疱漤桤铍铐. 项忮麇蝾 鲥眚痤忮 镳桦嚆囹 噻囹桀镳桧� 赅蝾 蝠弪� 嚆屙�. � 镱耠邃龛蝈 泐滂龛 忪羼蝾 鲨觌铖镱痂�, �觐� 沭箫� 桤镱腌筲囹 FK-506 赅蝾 嵇疴梓屙 桁箜铖箫疱耦�.
   青 镳铘桦嚓蜩赅 蝾灬铉桊囗弪� 磬 麇痦钿痤犴栩� 聱漕忮 蹂祛汶钺桧 � 蹂爨蝾牮栩 皴 镱滗驵� 磬 龛忸 10 - 12 煦%, 疱耧尻蜩忭� <35 %. 青 鲥腧� 皴 镳桦嚆� 蹂镟痂� 桦� 皴 桤恸瘌忄� 忮礤皴牿梃.
   � 耠篦嚅, 麇 � 磬腓鲥 恸眸屙 骐屙 漯屙噫, 皴 耠邃� 骐磬蜞 镳钿箨鲨�. 朽眄铗� � 疣忭铎屦眍 铗溴�礤 磬 骐赅 � 忄驽� 镱赅玎蝈� 玎 漕狃� 磬鬣腠� 趔黻鲨� 磬 镳桉噤赅蜞.
   袜 嵇疴� � 怦尻� 耠邃忄� 镱耱铒屦囹桠屙 溴� 皴 镳钼彐溧 Doppler 艴钽疣翳�, � 觐弪� 皴 耠邃� 镳铛钿桁铖蝌� 磬 镱痱嚯龛蝈 忮龛 � 麇痦钿痤犴栩� 囵蝈痂�.
   配磬 皴潇桷� 耠邃 铒屦圉�蜞, 嚓� 骐磬蜞 囗囫蝾祛玎 � 铖羼蜮屙� � �- tube, 黟彗 礤� 皴 铖羼蜮�忄 躅豚磴桀沭圄� 玎 铞屙赅 镳铛钿桁铖蝌� 磬 骐龛蝈 嵇蜩 � 聱耱�龛弪� 磬 囗囫蝾祛玷蝈 桁.
   念狃� 疣眄� 趔黻鲨� 磬 镳桉噤赅蜞 皴 镳邃镱豚汔 镳�: 耱噌桦磬 蹂祛滂磬扈赅, 疣眄� 镳钿箨鲨� 磬 骐赅, 镱沭羼桠眍 镱漕狃�忄礤 磬 囵蝈痂嚯眍蝾 Ph, 耧噤囗� 磬 皴痼祉� 豚牝囹 � 耱铋眍耱栩� 磬 INR, 眍痨嚯龛 龛忄 磬 汶铉囹�. 袜 嵇疴� 镱耱铒屦囹桠屙 溴� 蝠囗襦扈磬玷蝈 礤 徼忄 溧 镳邂桫噔囹 2000 �, 赅蝾 � 耠邃忄蝈 �觐腙� 漤� 蝈 蝠�徕� 溧 皴 眍痨嚯桤桊囹.
软蝈疴嚯 铗 怵屐� 玎 漕耱桡囗� 磬 眍痨嚯磬 趔黻鲨� 磬 镳桉噤赅蜞 � 祉钽� 桧滂忤潴嚯屙 � 祛驽 溧 漕耱桡礤 62 漤�.
   洛漤嚆� 耠邃 疱镥痿箸�蜞 磬 镳桉噤赅蜞 皴 恸忮驿囹 橡邃龛珙腩� i.v.- 20 煦. / 赉.�., 骤觌铖镱痂� per os- 10 煦 / 赉.�., 褥箴囗 - 2 煦 / 赉.�.
� 耠篦嚅, 麇 忪羼蝾 骤觌铖镱痂� 皴 桤镱腌筲� FK- 506, � 聃屐囹� 礤 镳桉蜮筲� 褥箴囗. 羊囵蜩疣蜞 漕玎 玎 FK - 506 磬 溴� � 耠邃 蝠囗耧豚眚圉�蜞 � 0, 3 煦 / 赉.�.
暑汔蝾 礤 � 恸珈铈眍 溧忄礤蝾 磬 骤觌铖镱痂� 镳彗 篑蜞蜞, 蝾� 皴 镳桦嚆� 忮眍珥�. 洛眍珥囹� 漕玎 皴 铗磬� 犏� 铕嚯磬蜞 1 : 3.
念玎蜞 磬 耱屦铊滂蝈 皴 磬爨�忄 赅牝� 耠邃忄:
1 溴� - 10 煦 / 赉. �.
2 溴� - 8 煦 / 赉.�.
3 溴� - 6 煦 / 赉.�.
4 溴� - 4 煦 / 赉.�.
5 溴� - 2 煦 / 赉.�.
6 溴� - 1煦 / 赉.�.
7 溴� - 1 煦 / 赉.�.
央邃忄 镱 - 徉忭� 磬爨�忄礤 磬 漕玎蜞 漕 0, 5 煦/ 赉.�. � 牮� 磬 嵇疴� 戾皴�; 0,3 煦 / 赉.�. � 牮� 磬 怛铕� 戾皴�. � �觐� 鲥眚痤忮 镳邃龛珙腩睑� 皴 耧桊� 磬 蝠弪� 戾皴�, � 漯筱� - 磬 耱� 戾皴�, � 蝠弪� 1 泐滂磬 耠邃 蝠囗耧豚眚圉�蜞.
念玎蜞 磬 骤觌铖镱痂磬 聱 皴 磬爨�忄, 赅蝾 皴 耠邃� 龛忸蝾 祗 � 牮蜞:

青 FK - 506 皴 镱滗驵� 耠邃龛蝈 龛忄: