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胆道閉鎖症(小児科)

著者: 田中秀明 福島県立医科大学附属病院 小児外科

監修: 五十嵐隆 国立成育医療研究センター

著者校正/監修レビュー済:2019/07/10
参考ガイドライン:
胆道閉鎖症診療ガイドライン
患者向け説明資料

概要・推奨   

  1. 胆道閉鎖症(biliary atresia、BA)の患者は葛西手術により胆汁流出が得られても、長期的には肝障害が進行する場合が少なくないといえる。葛西手術後も肝移植の選択肢を視野に入れた綿密なフォローが長期にわたり必要である(推奨度1)
  1. BAの患児に対しては早期に葛西手術を行うほど術後の成績がよい傾向がある(推奨度1)
  1. 便色カラーカードによるスクリーニングはBAの早期発見、早期手術を促し、術後成績を向上させる可能性があるOJ、推奨度1)
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薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、林太祐、渡邉裕次、井ノ口岳洋、梅田将光による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、
著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適用の査定において保険適用及び保険適用外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適用の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
田中秀明 : 特に申告事項無し[2021年]
監修:五十嵐隆 : 特に申告事項無し[2021年]

改訂のポイント:
  1. 胆道閉鎖症診療ガイドラインに基づき改訂を行った。

病態・疫学・診察

疾患情報(疫学・病態)  
  1. BAは胎児期から出生前後に肝外胆管が完全に閉塞し、肝内胆管にも病変が及ぶ疾患で、適切な外科的治療を行わなければ患児は救命できない。
  1. 1万出生に約1人の割合で出生し、男女比は0.6:1と女児に多い。
  1. 10%のBAの患者に内臓逆位、多(または無)脾症、下大静脈欠損、心奇形などを合併するbiliary atresia splenic malformation(BASM)が認められることがある。
  1. 原因は単一ではなく、遺伝的素因があるところにウイルス感染、なんらかのtoxinなどの環境因子が複数影響して発症するとの説が有力である。
  1. BAの肝組織像はさまざまな程度の門脈域の線維化や浮腫、胆管増生、胆汁うっ滞を示すが、組織像のみでは他の閉塞性黄疸を来す疾患との鑑別は難しく、画像診断が必須である。
  1. 胆道閉鎖症は、指定難病であり、重症度2以上の場合などでは申請し認定されると保険料の自己負担分の一部が公費負担として助成される。([平成27年7月施行])
  1.  難病法に基づく医療費助成制度 
問診・診察のポイント  
  1. BAの主な症状は、黄疸、灰白色便、褐色尿、肝腫大である。

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文献 

著者: Terry M Humphrey, Mark D Stringer
雑誌名: Radiology. 2007 Sep;244(3):845-51. doi: 10.1148/radiol.2443061051.
Abstract/Text PURPOSE: To evaluate prospectively the sensitivity of ultrasonography (US) in the diagnosis of biliary atresia (BA), with surgery as the reference standard.
MATERIALS AND METHODS: After institutional ethical approval and with informed parental consent, 90 consecutive fasting infants with conjugated hyperbilirubinemia underwent detailed US studies performed by a single operator with a 7.5-MHz curvilinear transducer and a 13.5-MHz linear-array transducer. The following features were prospectively recorded: gallbladder morphology, triangular cord sign, presence of a common bile duct, liver size and echotexture, splenic appearance, and vascular anatomy. The operator was blinded to results of other investigations. Sensitivity, specificity, and positive and negative predictive values were calculated for each US variable. BA and non-BA groups were compared by means of the Fisher exact test for categorical variables and an unpaired t test for continuous variables.
RESULTS: Thirty infants (13 male, 17 female) had surgically confirmed BA, and 60 (35 male, 25 female) had other documented causes of neonatal jaundice; the mean ages at US assessment were 48.5 and 52.4 days, respectively (P>.5). Eight US features showed a significant difference between BA and non-BA groups (P<.001, Fisher exact test). The features with the greatest individual sensitivity and specificity, respectively, in the diagnosis of BA were triangular cord sign (73% and 100%), abnormal gallbladder wall (91% and 95%) and shape (70% and 100%), and an absent common bile duct (93% and 92%). The hepatic artery diameter was significantly larger in infants with BA than in those without BA (mean+/-standard deviation, 2.2 mm+/-0.59 vs 1.6 mm+/-0.40, P<.001), but portal vein diameters were not significantly different. By means of all these US features, 88 of 90 infants were correctly classified as having or not having BA, for an overall accuracy of 98%.
CONCLUSION: BA can be distinguished with US from other causes of conjugated hyperbilirubinemia in 98% of infants if multiple US features are carefully evaluated.

Copyright (c) RSNA, 2007.
PMID 17709832  Radiology. 2007 Sep;244(3):845-51. doi: 10.1148/radiol.・・・
著者: Woo Sun Kim, Jung-Eun Cheon, Byung Jae Youn, So-Young Yoo, Wha Young Kim, In-One Kim, Kyung Mo Yeon, Jeong Kee Seo, Kwi-Won Park
雑誌名: Radiology. 2007 Nov;245(2):549-55. doi: 10.1148/radiol.2452061093. Epub 2007 Sep 21.
Abstract/Text PURPOSE: To prospectively evaluate the accuracy of hepatic artery diameter and hepatic artery diameter-to-portal vein diameter ratio for ultrasonographic (US) diagnosis of biliary atresia, with cholangiographic or clinical information as reference standard.
MATERIALS AND METHODS: Institutional review board approval and informed consent were obtained. US was performed in 68 neonates and infants with cholestatic jaundice (mean age, 61 days; male-to-female ratio, 38:30). Biliary atresia (n = 38) was confirmed with cholangiography, and hepatitis (n = 30) was diagnosed with clinical (n = 24) or cholangiographic (n = 6) findings. Diameter of the right hepatic artery was measured with US. Right hepatic artery diameter-to-right portal vein diameter ratio was measured to determine relative enlargement of the hepatic artery. As a control group, 17 neonates and infants (mean age, 67 days; male-to-female ratio, 12:5) without jaundice underwent US of the porta hepatis. Statistical analysis was performed to compare US parameters among three groups with one-way analysis of variance. Optimal cutoff values of the hepatic artery diameter and hepatic artery diameter-to-portal vein diameter ratio for biliary atresia diagnosis were obtained with receiver operating characteristic analysis.
RESULTS: The diameter of the right hepatic artery in biliary atresia group (1.9 mm +/- 0.4 [standard deviation]) was significantly larger than that in the hepatitis (1.4 mm +/- 0.3) and control (1.2 mm +/- 0.2) groups (P < .001). Hepatic artery diameter-to-portal vein diameter ratio in the biliary atresia group (0.52 +/- 0.12) was larger than that in hepatitis (0.40 +/- 0.07) and in control (0.40 +/- 0.10) groups (P < .001). Optimum cutoff values for diagnosis of biliary atresia were 1.5 mm (sensitivity, 92%; specificity, 87%; accuracy, 89%) for hepatic artery diameter and 0.45 for hepatic artery diameter-to-portal vein diameter ratio (sensitivity, 76%; specificity, 79%; accuracy, 78%).
CONCLUSION: Measurement of hepatic artery diameter can be helpful in the US diagnosis of biliary atresia.

PMID 17890351  Radiology. 2007 Nov;245(2):549-55. doi: 10.1148/radiol.・・・
著者: Richard A Schreiber, Collin C Barker, Eve A Roberts, Steven R Martin, Fernando Alvarez, Lesley Smith, J Decker Butzner, Iwona Wrobel, David Mack, Stanley Moroz, Mohsin Rashid, Rabin Persad, Dominique Levesque, Herbert Brill, Garth Bruce, Jeff Critch, Canadian Pediatric Hepatology Research Group
雑誌名: J Pediatr. 2007 Dec;151(6):659-65, 665.e1. doi: 10.1016/j.jpeds.2007.05.051. Epub 2007 Oct 22.
Abstract/Text OBJECTIVE: To determine the outcomes of Canadian children with biliary atresia.
STUDY DESIGN: Health records of infants born in Canada between January 1, 1985 and December 31, 1995 (ERA I) and between January 1, 1996 and December 31, 2002 (ERA II) who were diagnosed with biliary atresia at a university center were reviewed.
RESULTS: 349 patients were identified. Median patient age at time of the Kasai operation was 55 days. Median age at last follow-up was 70 months. The 4-year patient survival rate was 81% (ERA I = 74%; ERA II = 82%; P = not significant [NS]). Kaplan-Meier survival curves for patients undergoing the Kasai operation at age < or = 30, 31 to 90, and > 90 days showed 49%, 36%, and 23%, respectively, were alive with their native liver at 4 years (P < .0001). This difference continued through 10 years. The 2- and 4-year post-Kasai operation native liver survival rates were 47% and 35% for ERA I and 46% and 39% for ERA II (P = NS). A total of 210 patients (60%) underwent liver transplantation; the 4-year transplantation survival rate was 82% (ERA I = 83%, ERA II = 82%; P = NS).
CONCLUSIONS: This is the largest outcome series of North American children with biliary atresia at a time when liver transplantation was available. Results in each era were similar. Late referral remains problematic; policies to ensure timely diagnosis are required. Nevertheless, outcomes in Canada are comparable to those reported elsewhere.

PMID 18035148  J Pediatr. 2007 Dec;151(6):659-65, 665.e1. doi: 10.1016・・・
著者: Mark Davenport, Enrica Caponcelli, Emily Livesey, Nedim Hadzic, Edward Howard
雑誌名: Ann Surg. 2008 Apr;247(4):694-8. doi: 10.1097/SLA.0b013e3181638627.
Abstract/Text OBJECTIVE: The exact nature of the relationship between age at Kasai portoenterostomy (KP) for biliary atresia (BA) and outcome is controversial.
METHODS: Single-center retrospective analysis of consecutive cases (January 1994-December 2005) undergoing KP, using 2 dichotomous measures of outcome (clearance of jaundice to <20 micromol/L and native liver survival at 2 years post-KP). Outcome was analyzed by age cohort (<30 days, 30-40 days, etc) and cumulatively. Data are quoted as median (interquartile range). Statistical analysis was by extended Fisher r x c analysis. P < 0.05 was regarded as significant.
RESULTS: Two hundred twenty-five infants with BA were divided into 3 groups based on possible etiology: isolated BA (n = 177), biliary atresia splenic malformation (BASM) syndrome (n = 28), and cystic BA (n = 23). Three infants had both BASM and a cyst. Age at KP was significantly greater in those with isolated BA [58 (48-61)] compared with both BASM [47 (39-59); P = 0.02] and cystic BA [47 (39-54); P = 0.02]. Overall, 127 (56%) cleared their jaundice and 145 (65%) survived to 2 years with their native liver. Children with isolated BA showed no statistical difference by age cohort for clearance of jaundice (P = 0.75) or for native liver survival (P = 0.14). In contrast, there was a marked detrimental effect of age at KP for both BASM and cystic BA groups (P = 0.02).
CONCLUSION: There is a marked detrimental effect of age at KP on groups with a presumed "developmental" origin, not seen in the majority with isolated BA.

PMID 18362634  Ann Surg. 2008 Apr;247(4):694-8. doi: 10.1097/SLA.0b013・・・
著者: F M Karrer, J R Lilly, B A Stewart, R J Hall
雑誌名: J Pediatr Surg. 1990 Oct;25(10):1076-80; discussion 1081.
Abstract/Text The Registry provides information about 904 children with biliary atresia from more than 100 institutions. There was a 1.4 to 1 female predominance; racial distribution was 62% caucasian, 20% black, 11% Hispanic, 4.2% asian, and 1.5% American Indian. Eight hundred sixteen (90%) underwent corrective surgery (median age at operation, 69 days). Intraoperatively, 70% had totally obliterated extrahepatic bile ducts, 22% had patency of the gallbladder and distal common duct, whereas only 8% had "correctable" biliary atresia (proximal duct patency). A variety of reconstructions were used, but the majority of patients had a Roux-en-Y portoenterostomy with or without exteriorization. Follow-up was available for 670 children (74%) with average length of follow-up of 5 years (range, 1 to 16 years). Five-year actuarial survival was 48% following Kasai's operation, but was less than 10% (at 3 years) if no operative correction was done. Survival was unaffected by sex, type of reconstruction, or cholangitis. Predictors of a bad outcome were (1) caucasian race; (2) operative age greater than 60 days; (3) presence of cirrhosis at initial biopsy; (4) totally nonpatent extrahepatic ducts; (5) absent ducts at the level of transection in the liver hilus; and (6) subsequent development of varices or ascites. Identification of factors predictive of the ultimate outcome provide a basis for either continued efforts with management of Kasai's operation or for early referral for liver transplantation.

PMID 2262862  J Pediatr Surg. 1990 Oct;25(10):1076-80; discussion 108・・・
著者: Masato Shinkai, Youkatsu Ohhama, Hiroshi Take, Norihiko Kitagawa, Hironori Kudo, Kyoko Mochizuki, Tomoko Hatata
雑誌名: J Pediatr Gastroenterol Nutr. 2009 Apr;48(4):443-50.
Abstract/Text OBJECTIVE: Sequential strategies combining the Kasai operation as a first-line treatment and liver transplantation as a second-line option, if necessary, have been accepted for patients with biliary atresia (BA). To understand the role of the Kasai operation in the treatment of BA, it is necessary to analyze the long-term outcome of the operation alone and to evaluate the present status of survivors retaining their native livers.
MATERIALS AND METHODS: A retrospective chart review was carried out for a group of 80 patients who had undergone the Kasai operation between 1970 and 1986 at the Kanagawa Children's Medical Center.
RESULTS: The 5-, 10-, and 20-year survival rates of patients with their native livers were 63%, 54%, and 44%, respectively. The survival rates varied significantly depending on the type of BA, age at initial Kasai operation, era of surgery, and surgical method. By age 20, nearly half of the adult survivors had already developed liver cirrhosis and its sequelae. Episodes of cholangitis and gastrointestinal bleeding occurred after 20 years of age in 37% and 17% of the adult patients, respectively, and 20% of the adult patients died of liver failure or underwent living-related partial liver transplantation in their 20s. Five female patients gave birth to a total of 9 children, and 1 male patient fathered a child.
CONCLUSIONS: Although increasing numbers of patients with BA survive 20 years or more after the Kasai operation, meticulous lifelong postoperative care should be continued for the survivors because of the possibility of hepatic deterioration.

PMID 19330933  J Pediatr Gastroenterol Nutr. 2009 Apr;48(4):443-50.
著者: Cheng-Hui Hsiao, Mei-Hwei Chang, Huey-Ling Chen, Hung-Chang Lee, Tzee-Chung Wu, Chieh-Chung Lin, Yao-Jong Yang, An-Chyi Chen, Mao-Meng Tiao, Beng-Huat Lau, Chia-Hsiang Chu, Ming-Wei Lai, Taiwan Infant Stool Color Card Study Group
雑誌名: Hepatology. 2008 Apr;47(4):1233-40. doi: 10.1002/hep.22182.
Abstract/Text UNLABELLED: Biliary atresia is the most common cause of death from liver disease in children. Although the Kasai operation before 60 days of age can significantly improve prognosis, delay in referral and surgery remains a formidable problem worldwide because of difficulties in differentiating it from benign prolonged neonatal jaundice. We established a universal screening system using an infant stool color card to promote the early diagnosis and treatment of biliary atresia. After a pilot regional study in 2002-2003, a national stool color screening system was established by integrating the infant stool color card into the child health booklet given to every neonate in Taiwan since 2004. Within 24 hours of the discovery of an abnormal stool color, this event is reported to the registry center. The annual incidence of biliary atresia per 10,000 live births in 2004 and 2005 was 1.85 (40/216,419) and 1.70 (35/205,854), respectively. The sensitivity of detecting biliary atresia using stool cards before 60 days of age was 72.5% in 2004, which improved to 97.1% in 2005. The national rate of the Kasai operation before 60 days of age increased from 60% in 2004 to 74.3% in 2005. The jaundice-free rate (<2 mg/dL) at 3 months after the Kasai operation among infants with biliary atresia in 2004-2005 was 59.5% (44 of 74), significantly higher than the historical data of 37.0% in 1976-2000 before the stool card screening program (P = 0.002).
CONCLUSION: Universal screening using the stool color cards can enhance earlier referral, which may ultimately lead to timely performance of the Kasai operation and better postoperative outcome in infants with biliary atresia.

PMID 18306391  Hepatology. 2008 Apr;47(4):1233-40. doi: 10.1002/hep.22・・・

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