Progression from non-alcoholic steatohepatitis(NASH)to decompensated liver cirrhosis during the course of crohn’s disease : a case report

Crohn病の経過中に非アルコール性脂肪性肝炎(NASH)から非代償性肝硬変へと進展した一例

Progression from non-alcoholic steatohepatitis(NASH)to decompensated liver cirrhosis during the course of crohn’s disease : a case report

Creators Tanabe Norikazu

Creators Harima Yumiko

Creators Hashimoto Shinichi

Creators Terai Shuji

Creators Yamasaki Takahiro

Creators Sakaida Isao

症例は31歳の女性.18歳時にCrohn病と診断され,当科での治療を開始された.経腸栄養療法や5-ASA製剤,ステロイドや抗TNFα抗体製剤等の内科的治療を行うも効果不十分であり,消化管合併症の悪化から24歳時に回腸部分切除術,26歳時に回盲部切除術,28歳時に回腸および上行結腸切除術を施行し,残存小腸は約280cmとなった.その後も症状は安定せず,成分栄養剤による経腸栄養療法を勧めるも患者の理解が得られず,長期の絶食および中心静脈栄養を施行していた.31歳時頃より,見当識障害および活動性低下が認められたため当科入院となった.腹部骨盤単純CT検査上,肝萎縮を伴う肝硬変の状態と考えられ,血液生化学検査にて著明な肝機能障害およびアンモニア値の上昇を認めたため,非代償性肝硬変症による肝性脳症と診断された.血液検査上HBVおよびHCV感染は否定され,飲酒歴もなく,以前より脂肪肝が認められ,肝胆道系酵素の上昇も認められていたことから,非アルコール性脂肪性肝炎(NASH)による非代償性肝硬変と診断した.年齢と肝機能から肝移植を考慮したが,適したドナーがいなかったことと,患者が肝移植を希望しなかったことから対症療法を継続した.その後もCrohn病や肝硬変の加療で入退院を繰り返し肝不全により死亡した.重症Crohn病の経過中に複数の要因からNASHを併発し非代償性肝硬変症へ進展した,極めてまれな症例を経験したため報告する.

The patient was a 31-year-old woman who had been diagnosed with Crohn’s disease at age 18 years. Enteral nutrition and medical treatment were given, but their effect was insufficient. Gastrointestinal complications worsened, and ileocecal resection was performed three times. The remaining small intestine was 280 cm. But, her symptoms were unstable. Thus long-term fasting and central venous nutrition were adopted. At 31 years old, she was hospitalized with cognitive dysfunction and decreased activity. CT scan showed liver cirrhosis(LC),and hepatic dysfunction and an elevated ammonia level were seen on blood tests. Therefore, she was diagnosed as hepatic encephalopathy from decompensated LC. Blood tests were negative for HBV and HCV infections, she had no history of drinking alcohol, fatty liver had been presented for some time, and elevated transaminase were seen. This led to a diagnosis of LC resulting from non-alcoholic steatohepatitis(NASH).Liver transplantation was considered, but there was no appropriate donor, and the patient did not desire. Therefore, symptomatic therapy was continued. She was repeatedly hospitalized and finally died from liver failure. We report that the rare case in which NASH occurred due to multiple factors during the course of severe Crohn’s disease and progressed to decompensated LC.

[ISSN]0513-1731

[NCID]AN00243156