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Published in: Pediatric Surgery International 12/2017

01-12-2017 | Review Article

Screening for biliary atresia

Author: Akira Matsui

Published in: Pediatric Surgery International | Issue 12/2017

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Abstract

Early diagnosis followed by proper KP is essential for the improvement of long-term prognosis for patients with BA. It is increasingly accepted that KP at ≤ 30 days of age significantly improves native liver survival rate. Published analyses in English and Japanese indicate that screening by SCC and DB/CB is potentially feasible. Screening with SCC has been implemented in Tochigi Prefecture, Japan, since 1994. The concept of SCC was introduced from Japan to Taiwan and resulted in nationwide screening with SCC for the first time in Taiwan in 2004, followed by Japan in 2012. Home-based screening using SCC is easy and cost-effective; however, it may cause some difficulties for families in case of stools with intermediate colors. Laboratory-based screening using DB/CB may detect the suspected cases earlier, resulting in an increase in the number of patients with BA who undergo KP at ≤ 30 days of age; however, the recall rate is 1% and may be beyond an acceptable range. Further studies are needed to assess the feasibility and cost-effectiveness of both home-based (SCC) and laboratory-based (DB/CB) screening for BA.
Literature
1.
go back to reference Gu YH, Yokoyama K, Mizuta K, Tsuchioka T, Kudo T, Sasaki H, Nio M, Tang J, Ohkubo T, Matsui A (2015) Stool color card screening for early detection of biliary atresia and long-term native liver survival: a 19-year cohort study in Japan. J Pediatr 166:897–902.e1. doi:10.1016/j.jpeds.2014.12.063 CrossRefPubMed Gu YH, Yokoyama K, Mizuta K, Tsuchioka T, Kudo T, Sasaki H, Nio M, Tang J, Ohkubo T, Matsui A (2015) Stool color card screening for early detection of biliary atresia and long-term native liver survival: a 19-year cohort study in Japan. J Pediatr 166:897–902.e1. doi:10.​1016/​j.​jpeds.​2014.​12.​063 CrossRefPubMed
2.
go back to reference Hsiao CH, Chang MH, Chen HL, Lee HC, Wu TC, Lin CC, Yang YJ, Chen AC, Tiao MM, Lau BH et al (2008) Universal screening for biliary atresia using an infant stool color card in Taiwan. Hepatology 47:1233–1240. doi:10.1002/hep.22182 CrossRefPubMed Hsiao CH, Chang MH, Chen HL, Lee HC, Wu TC, Lin CC, Yang YJ, Chen AC, Tiao MM, Lau BH et al (2008) Universal screening for biliary atresia using an infant stool color card in Taiwan. Hepatology 47:1233–1240. doi:10.​1002/​hep.​22182 CrossRefPubMed
4.
go back to reference Lykavieris P, Chardot C, Sokhn M, Gauthier F, Valayer J, Bernard O (2005) Outcome in adulthood of biliary atresia: a study of 63 patients who survived for over 20 years with their native liver. Hepatology 41:366–371. doi:10.1002/hep.20547 CrossRefPubMed Lykavieris P, Chardot C, Sokhn M, Gauthier F, Valayer J, Bernard O (2005) Outcome in adulthood of biliary atresia: a study of 63 patients who survived for over 20 years with their native liver. Hepatology 41:366–371. doi:10.​1002/​hep.​20547 CrossRefPubMed
5.
go back to reference Kasahara M, Umeshita K, Inomata Y, Uemoto S, Japanese Liver Transplantation Society (2013) Long-term outcomes of pediatric living donor liver transplantation in Japan: an analysis of more than 2200 cases listed in the registry of the Japanese Liver Transplantation Society. Am J Transplant 13:1830–1839. doi:10.1111/ajt.12276 CrossRefPubMed Kasahara M, Umeshita K, Inomata Y, Uemoto S, Japanese Liver Transplantation Society (2013) Long-term outcomes of pediatric living donor liver transplantation in Japan: an analysis of more than 2200 cases listed in the registry of the Japanese Liver Transplantation Society. Am J Transplant 13:1830–1839. doi:10.​1111/​ajt.​12276 CrossRefPubMed
8.
go back to reference Superina R, Magee JC, Brandt ML, Healey PJ, Tiao G, Ryckman F, Karrer FM, Iyer K, Fecteau A, West K et al (2011) The anatomic pattern of biliary atresia identified at time of Kasai hepatoportoenterostomy and early postoperative clearance of jaundice are significant predictors of transplant-free survival. Ann Surg 254:577–585. doi:10.1097/SLA.0b013e3182300950 CrossRefPubMedPubMedCentral Superina R, Magee JC, Brandt ML, Healey PJ, Tiao G, Ryckman F, Karrer FM, Iyer K, Fecteau A, West K et al (2011) The anatomic pattern of biliary atresia identified at time of Kasai hepatoportoenterostomy and early postoperative clearance of jaundice are significant predictors of transplant-free survival. Ann Surg 254:577–585. doi:10.​1097/​SLA.​0b013e3182300950​ CrossRefPubMedPubMedCentral
10.
go back to reference Schreiber RA, Masucci L, Kaczorowski J, Collet JP, Lutley P, Espinosa V, Bryan S (2014) Home-based screening for biliary atresia using infant stool colour cards: a large-scale prospective cohort study and cost-effectiveness analysis. J Med Screen 21:126–132. doi:10.1177/0969141314542115 CrossRefPubMed Schreiber RA, Masucci L, Kaczorowski J, Collet JP, Lutley P, Espinosa V, Bryan S (2014) Home-based screening for biliary atresia using infant stool colour cards: a large-scale prospective cohort study and cost-effectiveness analysis. J Med Screen 21:126–132. doi:10.​1177/​0969141314542115​ CrossRefPubMed
13.
go back to reference Sasaki H (1984) Development of bile acid metabolism in neonates during perinatal period. Part 2: mass screening of congenital biliary atresia by radioimmunoassay using dried blood spot. Acta Paediatr Jpn 26:161–168 Sasaki H (1984) Development of bile acid metabolism in neonates during perinatal period. Part 2: mass screening of congenital biliary atresia by radioimmunoassay using dried blood spot. Acta Paediatr Jpn 26:161–168
15.
go back to reference Madlon-Kay DJ (1997) Recognition of the presence and severity of newborn jaundice by parents, nurses, physicians, and icterometer. Pediatrics 100:E3CrossRefPubMed Madlon-Kay DJ (1997) Recognition of the presence and severity of newborn jaundice by parents, nurses, physicians, and icterometer. Pediatrics 100:E3CrossRefPubMed
16.
go back to reference Mowat AP, Davidson LL, Dick MC (1995) Earlier identification of biliary atresia and hepatobiliary disease: selective screening in the third week of life. Arch Dis Child 72:90–92CrossRefPubMedPubMedCentral Mowat AP, Davidson LL, Dick MC (1995) Earlier identification of biliary atresia and hepatobiliary disease: selective screening in the third week of life. Arch Dis Child 72:90–92CrossRefPubMedPubMedCentral
18.
19.
go back to reference Witt M, Lindeboom J, Wijnja C, Kesler A, Keyzer-Dekker CM, Verkade HJ, Hulscher JB (2016) Early detection of neonatal cholestasis: inadequate assessment of stool color by parents and primary healthcare doctors. Eur J Pediatr Surg 26:67–73. doi:10.1055/s-0035-1566101 PubMed Witt M, Lindeboom J, Wijnja C, Kesler A, Keyzer-Dekker CM, Verkade HJ, Hulscher JB (2016) Early detection of neonatal cholestasis: inadequate assessment of stool color by parents and primary healthcare doctors. Eur J Pediatr Surg 26:67–73. doi:10.​1055/​s-0035-1566101 PubMed
21.
go back to reference Kong YY, Zhao JQ, Wang J, Qiu L, Yang HH, Diao M, Li L, Gu YH, Matsui A (2016) Modified stool color card with digital images was efficient and feasible for early detection of biliary atresia—a pilot study in Beijing, China. World J Pediatr 12:415–420. doi:10.1007/s12519-016-0061-7 CrossRefPubMed Kong YY, Zhao JQ, Wang J, Qiu L, Yang HH, Diao M, Li L, Gu YH, Matsui A (2016) Modified stool color card with digital images was efficient and feasible for early detection of biliary atresia—a pilot study in Beijing, China. World J Pediatr 12:415–420. doi:10.​1007/​s12519-016-0061-7 CrossRefPubMed
22.
go back to reference Morinville V, Ahmed N, Ibberson C, Kovacs L, Kaczorowski J, Bryan S, Collet JP, Schreiber R (2016) Home-based screening for biliary atresia using infant stool color cards in Canada: Quebec feasibility study. J Pediatr Gastroenterol Nutr 62:536–541. doi:10.1097/MPG.0000000000001042 CrossRefPubMed Morinville V, Ahmed N, Ibberson C, Kovacs L, Kaczorowski J, Bryan S, Collet JP, Schreiber R (2016) Home-based screening for biliary atresia using infant stool color cards in Canada: Quebec feasibility study. J Pediatr Gastroenterol Nutr 62:536–541. doi:10.​1097/​MPG.​0000000000001042​ CrossRefPubMed
24.
28.
go back to reference Matsui A, Kasano Y, Yamauchi Y, Momoya T, Shimada T, Ishikawa T, Abukawa D, Kimura A, Adachi K, Tazuke Y (1996) Direct enzymatic assay of urinary sulfated bile acids to replace serum bilirubin testing for selective screening of neonatal cholestasis. J Pediatr 129:306–308CrossRefPubMed Matsui A, Kasano Y, Yamauchi Y, Momoya T, Shimada T, Ishikawa T, Abukawa D, Kimura A, Adachi K, Tazuke Y (1996) Direct enzymatic assay of urinary sulfated bile acids to replace serum bilirubin testing for selective screening of neonatal cholestasis. J Pediatr 129:306–308CrossRefPubMed
30.
go back to reference Mushtaq I, Logan S, Morris M, Johnson AW, Wade AM, Kelly D, Clayton PT (1999) Screening of newborn infants for cholestatic hepatobiliary disease with tandem mass spectrometry. BMJ 319:471–477CrossRefPubMedPubMedCentral Mushtaq I, Logan S, Morris M, Johnson AW, Wade AM, Kelly D, Clayton PT (1999) Screening of newborn infants for cholestatic hepatobiliary disease with tandem mass spectrometry. BMJ 319:471–477CrossRefPubMedPubMedCentral
Metadata
Title
Screening for biliary atresia
Author
Akira Matsui
Publication date
01-12-2017
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Surgery International / Issue 12/2017
Print ISSN: 0179-0358
Electronic ISSN: 1437-9813
DOI
https://doi.org/10.1007/s00383-017-4175-3

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