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Budd-Chiari syndrome

= Hepatic venous obstruction syndrome

hepatic vein obstructionÀ¸·Î ÀÎÇÏ¿© hepatomegaly, abd pain, ascites, zone 3 sinusoidal

distensionÀÌ »ý±â´Â º´ÀÌ´Ù.

1. ¿øÀÎ

¨ç myeloproliferative disease ¿¹) polycythemia vera : 60%¿¡¼­ °ü·Ã, young female

¨è SLE, antiphospholipid syndrome(primary or secondary to SLE)

¨é circulating anticoagulant

¨ê DIC

¨ë Idiopathic granulomatous venulitis

¨ì PNH

¨í anticoagulant factor deficiency: AT III deficiency

¨î Behcet's disease Cx

¨ï oral contraceptive, pregnancy·Î ÀÎÇÑ hepatic vein thrombosis

¨ð trauma

¨ñ polycystic liver disease¿¡¼­ mechanical compression

¨ò IVC obstruction d/t malignant ds

¨ó RA myxoma

¨ô alcoholic & veno-occlusive ds¿¡¼­ central hepatic vein involve

¨õ liver transplantationÈÄ veno-occlusive disease

2. º´¸®

large hepatic vein : thrombotic

Isolated IVC or small hepatic vein obstruction : ÈçÈ÷ non-thrombotic

Liver : enlarge, purplish, smooth

venous congestion - cut surface "nutmeg" change

* chronic- caudate lobe enlarge, IVC compress

splenomegaly, portal-systemic collateral circulation¹ß»ý

mesenteric vessel thrombosis

* histology : zone3 venous dilatation

congestion with hemorrhage, necrosis

3. ÀÓ»óƯ¡

fulminant course¶§ encephalopathy¹ß»ýÇϸç 2-3ÁÖ³» »ç¸Á

chronic hepatocellular disease¹ß»ý

-> slowly develp -> confusion, cirrhosis

1) acute form : ill patient

abdominal pain, vomiting, liver enlargement

ascites, mild icterus

watery diarrehea(mesenteric venous obstruction)

-> terminal, inconstant feature

total hepatic vein occlusion¹ß»ý½Ã delirium, coma with hepatocellular failure

-> ¼öÀϳ» »ç¸Á

2) chronic form

1-6°³¿ù¿¡ °ÉÃļ­ ¹ß»ý

pain, enlarged tender liver, ascites

portal hypertension -> spleen palpation

caudate lobe enlargement

IVC block -> leg edema

3) LFT

Bil 2 mg/dLÀÌ»óÀº Àß ³ÑÁö ¾Ê´Â´Ù.

s-ALP, Alb°¨¼Ò

PT prolongation, protein¡é(¡ñ protein-losing enteropathy)

4) liver Bx: ÇʼöÀû, zone 3 congestion

5) hepatic venography : tortous, lase-like spider-web pattern

6) IVC venography : IVC patencyÈ®ÀÎ

side-to-side narrowing, IVC distortion, extrinsic compression from Lt

7) USG : hepatic vein abnormality

caudate lobe hypertrophy

IVC compression

PW doppler : hepatic vein, IVC blood flow(-)

Color doppler : hepatic vein abnormality(+)

8) CT : liver enlargement, diffuse hypodensity

patchy enhancement after contrast

* hepatic metastasis¿Í È¥µ¿µÊ

4. Áø´Ü

liver cirrhosis¿Í °¨º°À§ÇØ liver Bx½ÃÇà

»ó±â ¿µ»óÁø´Ü½ÃÇà

5. ¿¹ÈÄ

¿øÀο¡ µû¶ó ´Ù¸£´Ù.

6. Ä¡·á

Acute : fibrinolytic therapy - uncertain

Chronic : anticoagulant - underlying thrombosing diseaseȯÀÚ¿¡¼­ »ç¿ë

¿øÀο¡ µû¸¥ Ä¡·á¸¦ ÇÑ´Ù.