難治性腸管合併症の特徴と治療戦略―Crohn病に伴う慢性腸管不全患者の特徴─在宅中心静脈栄養療法例を中心に

Home Parenteral Nutrition for Crohn's Disease with Intestinal Failure

要旨　本邦において腸管不全を伴うCrohn病（CD）に対するHPN長期施行症例の有用性や合併症に関する検討は少ないため，CD患者29例（全患者718例中4.0％に相当）における長期在宅中心静脈栄養療法（HPN）施行例の現状とその有用性や問題点を検討した．対象29例中，男性9例，女性20例，HPN導入時の平均年齢は33.5歳であった．CDの診断からHPN導入までの平均経過期間は11.9±6.3年で，HPN施行の平均期間は6.6±4.0年であった．CDでの平均手術回数は3.3±1.9回，平均残存小腸長は210.7±98.0cm，人工肛門造設例は19例（65.5％）であった．残存腸管に活動性があるものは27例（93.1％）で，HPN導入前に狭窄22例（75.9％），瘻孔23例（79.3％），痔瘻癌1例（3.4％），アミロイドーシス1例（3.4％）の腸管合併症を認めた．HPN導入の主な理由は小腸大量切除後が16例と最も多く，次いで難治性瘻孔11例，難治性の肛門病変7例であった．HPN導入前後の入院頻度は導入前平均0.98±1.0回/年に対し，導入後0.52±0.39回/年と有意に減少していた（p＝0.036）．主なポート合併症は，カテーテル感染症や閉塞で0.63回/年であった．観察期間中5例死亡例があり，肝不全2例，心室細動，腎不全，突然死がそれぞれ1例であった．初回ポート留置後の入れ替え不要者の割合は6か月で78％，12か月で71％，18か月で56％であった．HPNはCD患者に対し，入院頻度の低下など，患者のQOL向上に有用である一方，ポート・カテーテル感染も多く，敗血症や血栓症，肝障害など重篤な合併症もみられた．HPN導入は慎重にすべきであり，原疾患とともに注意深い経過観察が必要である．Crohn病の腸管不全は4％に生じ，多くはHPN管理が必要となるが，残存腸病変に対する治療も重要である．

　Few studies have been conducted in Japan regarding the utility and complications of long-term HPN（home parenteral nutrition）for CD（Crohn's disease）with intestinal failure. The present study investigated the current state, utility and issues of long-term HPN in 29 CD patients, equivalent to 4.0％ of 718 CD patients. Patient characteristics were as follows : 9 men and 20 women ; mean age at start of HPN, 33.5 years ; mean period between CD diagnosis and start of HPN, 11.9±6.3 years ; mean HPN duration, 6.6±4.0 years ; and, mean frequency of CD-related surgeries, 3.3±1.9 times, including ileostomy and colostomy in 19 patients（65.5％）with a mean residual small intestine length of 210.7±98.0 cm. Activity in the residual intestinal tract was present in 27 patients（93.1％）. Intestinal tract complications prior to the start of HPN comprised stenosis, n＝22（75.9％）; fistula, n＝23（79.3％）; anal fistula cancer, n＝1（3.4％）; and, amyloidosis, n＝1（3.4％）. The most common primary reasons for HPN were massive small bowel resection（n＝16）, uncontrollable fistula（n＝11）and refractory anal lesions（n＝7）. The mean frequency of hospitalization significantly decreased following the start of HPN, from 0.98±1.0 times/year before starting to 0.52±0.39 times/year after starting（p＝0.036）. The main port-related complications comprised catheter infection and blockage, occurring at a mean of 0.63 times/year. During follow-up, 5 patients died, 2 patients experienced liver failure, and ventricular fibrillation, renal failure and sudden death were each suffered by 1 patient, respectively. Substitution following initial port placement was unnecessary for 78％ of patients at 6 months, 71％ at 12 months, and 56％ at 18 months. Although HPN decreased hospitalization frequency and improved quality of life for CD patients, a high incidence of catheter infection and serious complications such as septicemia, thrombosis and hepatic dysfunction were also observed. HPN should be introduced with caution and careful follow-up of both the primary disease and HPN is required. Intestinal failure arises in 4％ of CD cases and although HPN management is often necessary, treatment for residual bowel lesions is also important.