Dr. Han's Biliary Atresia Page

 

ȸ Ǿϴ.

ȸ õ 㵵 (Biliary Atresia) ̵ θԵ ڹ Դϴ. ȸ ÷ Ʒ  ȸ Ŭϼ.

ȸ

㵵 Ͻø Ǵ Ѽ Ȩ 湮Ͻʽÿ.

Ǵ Ѽ Ȩ

 

: 1865 Helschl 7 Ʊ ü ΰϿ  㵵 Ǿ ִ 㵵 ̶ ֽϴ. 㵵 ̶ ˷ Ͽ Ư ġ · ġǾ ִ ̴ٰ 1959 Ϻ Tohoku Kasai Ͽ ó Kasai Ǿ ̸ ϰ ִٰ 1970뿡 ͼ ȭ Ǿ 㵵 ȯڰ ְ Ǿϴ. ̽ļ ߴ޷ Kasai Ŀ ȭ Ǵ 㵵 ̷л 100% ġ ȯԴϴ.

㵵̶ ΰ?: 㵵̶ ܴ㵵(extrahepatic bile duct, ׸ 1) Ƽ ȯԴϴ (׸2). ˷ ʾ ̷ ¾ƽñ ۵Ǵ ǰ ȯڿ ó ٰ ð 鼭 Ͼ ϴ (׸ 3) Ƽ Ŀ Ǵ Ǿ ֽϴ.

׸ 1. 㵵 ׸ ܴ㵵(extrahepatic bile duct) ʷϻ ĥϿ Դϴ. ܴ㵵 âڰ () Ѵ.

׸ 2. ׸ ׸ 1 ʷϻ ǥõǾ üܴ㵵 Ƽ   㵵 ְ ֽϴ.

㵵 : 㵵 (׸ 3) ȸ Ȥ Ŀ ų ٰ Ȥ ȸ ۵Ǹ ƱⰡ Ȳ ִ Դϴ. Ʊ Ȳ 2ֱ   ֽϴ(physiologic jaundice). ׷ Ŀ ӵǴ Ȳ 쿡 Ȳ(pathologic jaundice) ݵ Ƿ ãư Ḧ ޾ƾ մϴ. ⼭ ٽ ϰ ƱⰡ Ȳ ְ Ͼ Ƶ Ʊ ڶ ǰϰ ̱ Ҿư Բ ̸ ó ʰ Ͽ ġ ñ⸦ ġ 찡 ϴ.   Ͽ Ÿ ̳ ̷ Ͽ 谡 ҷ Ⱑ Ͽ Ȳ ° ˴ϴ. ȭ Ǹ ĵƷ簡 ܼ Ǹ ϰų 뺯 ǰ Ǿ Ե˴ϴ. ġḦ ʰ Ǹ ᱹ Ʊ 2 ϰ ˴ϴ.

׸. 3   ׸ 㵵 ȯڰ ȥյ ȸ ְ ֽϴ. 㵵 ̷ ⵵ ־ ȥյ ȸ Ȥ ⵵ ϸ Ȳ 쿡 Ȳ޷ â ⵵ մϴ.

" Ͽ ƱⰡ ̷ Ϳ Ͽ ʴ´" ̴".

ٲپ Կų Ͽ Ͽ ܰ ġ ñ⸦ ġ 찡 Ʊ ȸ̰ų 쿡 ѷ ǻ縦 ãƺ⸦ 帳ϴ.

㵵 Ż ߿伺: Ȳް Ȥ ȸ ٰ 㵵 ƴմϴ. Ż 쳪 Ѵ(choledochal cyst) Ϻ ȯڿ ̷ ֽϴ.   Ѵ 쿡  㵵 Ż 㵵 찡 ϴ. Ż ̶ ־ ʴ ġ ƴմϴ. ׷ 㵵 쿡 8ֳ Ƿ ʿմϴ.

㵵 ܹ(Ż ) :

1. ӻ : ƱⰡ ȸ Ȥ 鼭 Ȳ ִ ȯ ֽϴ. ׷ Ż 쿡 ƱⰡ ̸ µ ʽϴ. ׷ 㵵 ȭ ȭDZ Ʊ ſ ǰ ̸ ڶϴ. ̷ ȯ ̴ ƴϰ Ϲ̶ ñ ٶϴ.

2. ˻: ȯ ϴ ù ° ˻ İ ǰڽϴ. ̸ Ͽ Ѵ Ȯ ˴ϴٸ Ż 㵵 ̸ ķ Ȯ Ͽ ϴ 찡 ϴ.(׸ 4)

 

 

 

 

 

 

׸ 4. 㵵 ȯ 㳶 ܴ㵵 . ׷ 2-3 Ʊ 㵵 ʹ ۾Ƽ Ƴ Ż ȯڿ ķ ̸ Ȯ ϴ 찡 Ƿ ȴ.

3. 缱Կ: кǴ 缱 Ҹ Ʊ ƿ ֻϿ Ұ âڷ кǴ ˻Դϴ. Ұ âڷ ̸ Ʊ 㵵 ƴ϶ ֽϴ.(׸ 5) ׷ Ұ âڷ ʴ´ٰ ؼ ݵ 㵵̶ ϴ.(׸ 6) ֳϸ Ż 쿡 ʰų к ʴ 쿡 㵵 ִٰ Ͽ Ұ âڷ Դϴ.

׸ 5. Ż ȯڿ 缱 Կ Ұ ִ ˴ϴ.(ȭǥ)

׸ 6. 㵵ȯڿ Կ 缱 Կ ð Ұ âڷ ϴ. ׷ ̷ Ұ Ż ȯڿ ֱ ˻ ƴմϴ.

4. ˻: ٴ÷ 񷯼 Ϻθ  Ͼ ̰ ϴ Դϴ. Ż 㵵 ʱ⳪ ⿡ Ұ ̱ ȯ ϸ ٴ÷  ֽϴ

5. ڱ 㵵Կ: (Ҿƿܰ, Ҿư, 缱) ܹ ذ ʾҳ ϰ ϰ ֽϴ. Ʊ 㵵 þ ػ󵵰 ڱ ̿Ͽ 㵵 Ƿ ˷ ϴٰ մϴ. ٸ õǰ ġ 迡 ϰ ںϰ ֽϴ. ֱ ̸ Ͽ Ϻδ ̹ ̹ ǥϿϴ. ڱ㵵Կ ܱ ǰ ִ ϴ. ׷ ڱ Կ⸸ ִٰ Ͽ ִٰ ʽϴ. ƹ ⸦ ´ٰ Ͽ ʺڰ ۰ ̰ Ͱ ġԴϴ. ϴ ü  , , Ұ ȭ ̷ ϴ Ͱ ٰ ֽϴ. Ÿ Կ  񿡰 Ǿ ȯڰ  ڱ㵵Կ ̷ Ҹ ʰ Ͽ ź ٴ մϴ.

    Ȯϰ(2001 1 , ΰ; 98%, Ư̵;100%), ħ( )̶ Դϴ. ſ Ȯ ܹ ʿ ޾Ҵ Ż ȯڰ ϰ ʹ ʿ ʰ Ǿϴ. 㵵 ȯ 쿡 Ȯϰ ϰ Ǿ ñ⸦ մ ־ϴ. Ʒ ׸(׸ 7, 8, 9, 10) ϰ ִ ڱ 㵵Կ Ͽ ְ ֽϴ.

ǥ ڱ㵵Կ Ʒ ٿ ֽϴ.

ȸ ǥ

Seok Joo Han, Myung-Joon Kim, Airi Han, Ki Sup Chung, Choon Sik Yoon, Dojoong Kim, Eui Ho Hwang: Magnetic Resonance Cholangiography for the Diagnosis of Biliary Atresia. Journal of Pediatric Surgery 37(4): 599-604, 2002

 

Myung-Joon Kim, Young Nyun Park, Seok Joo Han, Choon Sik Yoon, Hyung Sik Yoo, Eui Ho Hwang, Ki Sup Chung: Biliary Atresia in Neonates and Infants: Triangular Area of High Signal Intensity in the Porta Hepatis at T2-weighted MR Cholangiography with US and Histopathologic Correlation. Radiology 215: 395-401, 2000  

ȸ ǥ

Ѽ, ⼷, , Ѿָ,Ȳȣ:㵵 ܿ ־ ڱ㵵Կ 뼺. Ҿư 44: 899-907, 2001 ( ڱ㵵Կ ߾ 㵵   ϰ Դϴ.)

 

 

 

 

 

 

 

׸ 7. Ż ȯ ڱ 㵵Կ 㳶(GB) ܴ㵵(CHD, CBD) ̸ 㵵(BHD, 2nd) ˴ϴ. ȯڴ ޾ƾ ߴ ʿ ڱ 㵵 Կ ϹǷν ־ϴ.

 

 

 

 

 

 

 

׸ 8. 㵵 ȯ ڱ 㵵Կ 㳶(GB) ̳ ׸ 7 ̴ 㵵 ϴ. ȯڴ ڱ 㵵 Kasai ־ϴ.

 

 

 

 

 

 

 

 

׸ 9. 㵵 ȯ ڱ 㵵Կ 㳶(GB) ũ ̳ ׸ 7 ̴ 㵵 ϴ. ̷ 㳶 ū 㵵 쿡 Ұ ˴ϴ. ȯڿ Kasai ־ϴ.

 

 

 

 

 

 

 

 

׸ 10. ׸ 9 㵵 ȯ 㵵Կ 㳶(GB) ̸ 㵵 ֽϴ. ׸ 9 Կ ڱ 㵵Կ ϸ ڱ 㵵 Կ Դϴ.

㵵  ġϴ°?.: ε 㵵 ʴ 쿡 ᱹ Ͽ 㵵Կ(operative cholangiography) 㵵 Ȯ ī ϰ ˴ϴ. ī ϴٰ Ʊ ʰ ᱹ Ǹ  ̽ ʿϰ DZ⵵ մϴ.  ᱹ 㵵 Ʊ ī ġǰų Ϻδ ī ̽ ġ ֽϴ. δ 㵵 ġ ƴϸ ġᰡ Դϴ.  ׷ (ī , ü κ ̽) Ͽ ˾ƺ ϰڽϴ.

1) ī (Kasai hepatic portoenterostomy): ī ̶ 1959 Ϻ Ҿƿܰ ǻ ī Ͽ ߵ 㵵 θ ǰ ִ ̴. 㵵 ȯڿ 㵵 κ ƺ ̰ ȮεǴ 㵵 ִٴ Ȯϰ κп ̿Ͽ ο 㵵 ִ ̴. ߿ Ʈ Ȯ ʴ 㵵 κп Ͽ ִ 䱸Ѵ. 㵵 κп Ͽ ִ ȿ ϰ ȴ. ̷ ǻ簡 쿡 ϸ Psuedo-Kasai operation̶ Ѵ. Ʒ ׸ .

ī 㵵 ȯ 㵵  㳶 㵵 Ƽ ִ ִ.

̰ ִ ̼ 㵵 ִ κ( ) ϰ ִ ī ߿ ̴.

ܵ ̿Ͽ 㵵 ְ ִ ̴.

 ī : ī Ǿ ȯ ̰ ʹ 쿡 кǸ鼭 Ȳ ȴ. ׷ ȯ ̰ ʹ Ƽ ̹ 㵵 ų 쿡 ī Ǿٰ к ʰ ȴ. Ʒ ׸ ī ȸ 뺯 ְ ׸ Ŀ Ȳ 뺯 ְ ִ.

 ī ȯڿ ̰ ū Ǵ 㵵̴. 㵵 2 Ŀ ʴ´. 㵵 غϸ ̽ ī ǰϰ ְ ȴ.ī ó õǾ Ϻ ȣť(, Tohoku) . 1971 1994 ī ȯ 35% ȯڴ к ռϿ Ȳ ҽǵǰ պ Ͽ , ߴ޵ ƿ ̰ . 36% ȯڴ պ(㵵, ƾ ) 14% ȯڴ к Ǿ ʾƼ Ȳ ҽǵ ʾҴ. 15% ȯڿ Ŀ к ٰ Ѵ.

 ī ϴ : 幰 ī ȯڿ ڱ 찡 ȴ. ̷ 쿡 κ 㵵 찡 幰Դ ǥ鿡 ó ڶ ̼ 㵵 Ƿμ 찡 ִ. 쿡 Ͽ 㵵 ִ ó Ͽ ָ к 簳ȴ. ׷ ̸  Ǵϱ Ͽ ſ Ͽ ϸ 1 ȯڴ ϰ ִ.

㵵 պ

㵵(cholangitis): 㵵 㵵 ȯڿ ɰ ̴. 㵵 ǿ ߻ ڸ ٸ 40%-60% ǰ ִ. 㵵 Ȯ Ը ʾ Ϲ ޾ 鿩 ִ ī Ŀ 㵵 Ǹ 忡 ִ 㵵 ö󰡼 ٴ ̴. ޹ħϿ ִ ӻ, Ű ε ̸ ߿ ϳ ϰ ִ. ׷ ̸ ܿ ü ٸ ߿ ȴٰ Ѵ. 㵵 Ÿ 鼭 ٰ ȴ( Ͼ ). ̶ ˻縦 ϸ ġ ϸ ġ(C-protein) ϰ ȴ. 㵵 ӵǸ ᱹ ջ ް ǹǷ  ȯڿԴ ſ ġ̴. ī 㵵 ǽɵǸ ü ġḦ ޾ƾ Ѵ. ġ ް ׻ ̷ . ེ к ǰ ִ 쿡 6 9ʹ 㵵 󵵰 2 Ŀ ʴ´ٴ ̴.

 㵵 : 㵵  ϱ õǾ Դ.   ׸ Ʒ . ׸ A ο 㵵 ִ ̸ Ͽ ( 60-70 cm) 빰 㵵 ö󰡴 ִ ̸ ׸ B 㵵 Ͻ ̾Ƴ 빰 ö ϰ ϴ ̸ ׸ C 忡 긦 ־ 빰 㵵 ö ϰ ϴ ̴. B,C ʱ ʹ ٸ 㵵 ȿ Ͽ ϰ պ ʷϴ ˷ Ϻο ǰ ִ. Ϲ A Ѵ.

 

  (Portal Hypepertension): 㵵 ı⿡ պμ Ȳ ȯڿ ߻ϰ ȴ. 󵵴 ڸ ٸ 34% 76% ǰ ִ. ƾ ߻ 㵵 谡 ִ ˷ 㵵 ġᰡ ߿ϴٰ ϰڴ. ƾ Ǵ ĵ Ʒ(esophageal varices) ̸ ̴ ĵƷ ִ ȯ 20-60% ٰ Ѵ. ܿ (splenomegaly) Ͽ (thrombocytopenia) ʷ ִ.   㵵 쿡 Ұ 쿡 ̰  ȭǴ Ǿ ִ.

  պ: ȭ 뼺 Ÿ ʿ ̹Ƿ ̵ ʷ ִ. 㵵 ȯڿԴ 뼺 Ÿ Ͽ ִ medium chain triglyceride Ե Ư ̴ Ѵ.

 

2005 4 ȸ 㵵 պ Ʈ ÷ ŬϽʽÿ.

 

  : ī ̽ θ Ͽ ִ , ġ ɼ δ Ȳ ҽ Ǵ³ ƴϸ ׷ ϴİ ߿ϴ.   ó ī Ͽ ť ڷḦ 캸 Ʒ ׸ . ׷ ֵ Ȳ ҽ 쿡 ī   ׷ 쿡 ̽ ʿϰ ɼ ٰ ϰڴ.

 ̽

 ̽ļ ޼ ߴϴ о߷ 㵵 ȯڿ ־ ġ Ͽ. ̴ ߴް Բ cyclosporin̳ FK-506 鿪 ߴ޿ ũ. տ Ͽ 㵵 ȯڴ ī ޴ ̽ ޴ ̷л Ǿ. Ŀٶ Ҿƿ Ϻθ  ̽ϴ ̽ļ ֱ ߵǾ ذǾ.

.1) ī ޱ⿡ ʹ ̰ . 2) ī к . 3) ī պ(㵵, ) Ȱ ų ջ Ͽ ɼ ִ ̴.

׷ ̽ Ŀ ذǴ ƴϴ. źι Ͼ 쿡 ȯڴ Ұ Ǹ ź ϱ Ͽ Ǵ 鿪 ۿ Ͽ ġ ִ. ̻ ϴ ̳ ȯڿ ᱹ ̽ ʿϰ ȴ. Ʒ ׸ ̽ļ Ͽ ְ ִ.

 

 δ : 㵵̶ ȯƿ ȣڸ Ѻ鼭 ǻμ Ÿ ҽϴ. δ ̷ Ǿ ϴ ȸ ⵵ Ͽϴ. δ ּ ϰ ִٰ ϳ ذ ϴ ϴ. ׷ ϰ ϸ鼭 ̸ ذϰ մϴ. Ʈ 㵵 ް Ʊ⸦ ϴ 찡 ҽϴ. ÿ ̽ ̶ ħ ̸ ī ̾ϴ. ׷ ֱ ϳ 㵵 Ģ ĥ ִ ̴ ȵȴٰ  մϴ. 游 ִٸ ǻ ذ մ ֽϴ. ϳ 㵵 ȯڿ Ǿ մϴ.

ȸ ż ɵ ϰ ֽ Ͽ ֽñ ٶϴ. ȯ ȣ 츦 ȯմϴ. ȭ  Ŀ Դϴ.

ȸ 2005 Ϻ δ Ư ȯ 㵵Ⱑ ö󰬽ϴ.

ش ÷ Ʒ Ŭ ϼ.

ش

2011 ѿܰȸ ܰ 㵵⿡ chapterԴϴ.

ǰ л Ӹ ƴ϶ ٸ ǻ絵 ϴ ̱ 㵵⿡ Ͽ ȭ Ͽ ← ª ȿ Դϴ.  (ش ٷΰ) 

 

Ҿƿܰǻ Ȩ Դϴ.

ϴ å ֽϴ.

븸 ̸ ȯڿ ϴ ֽϴ.

ݵ ǻ ñ ٶϴ.

㵵 Ͽ ñϽ ø Ȩ  湮Ͽ ֽʽÿ.

Ȩ 湮 ư home ŬϽø ˴ϴ.

ø Ʒ г ˻ ŬϽʽÿ.

㵵 г ˻

ֵޱ(2003.7. Ǻ, HWP file)