Simple biliary atresia score-a validated diagnostic aid for infantile cholestasis.
Biliary atresia
Cholestatic jaundice
Diagnostic score
Journal
Pediatric surgery international
ISSN: 1437-9813
Titre abrégé: Pediatr Surg Int
Pays: Germany
ID NLM: 8609169
Informations de publication
Date de publication:
31 Jul 2024
31 Jul 2024
Historique:
accepted:
10
07
2024
medline:
1
8
2024
pubmed:
1
8
2024
entrez:
31
7
2024
Statut:
epublish
Résumé
The workup of jaundiced infants may be variable and protracted, thereby delaying the diagnosis and timely intervention for biliary atresia (BA). This potentially leads to inferior outcomes. We developed a practical score to stratify infantile cholestasis according to the risk of having BA. The score (0-7) [gallbladder length ≤ 15 mm (+ 1), common bile duct (CBD) diameter < 0.5 mm(+ 1), pre-portal vein (PV) echogenicity(+ 1), direct-to-total bilirubin ratio (D/T) ≥ 0.7(+ 2), and gamma-glutamyl transferase (GGT) ≥ 200 IU/L(+ 2)] are derived from logistic regression of data from a retrospective cohort of cholestatic infants (n = 58, 41 BA) in our institution. It was then validated with a separate retrospective cohort (n = 28, 17 BA) from another institution. Final diagnoses were as per intraoperative cholangiogram (IOC) and liver histopathology. A cutoff score of ≥ 3 diagnosed BA with 100% and 94% sensitivity in the derivative cohort (area under receiver operating characteristic curve, AUROC 0.869) and validation cohort (AUROC 0.807), respectively. D/T ratio was the most sensitive (93%) and CBD diameter was the most specific (88%) parameter. The score accurately predicted non-BA in 11(65%) and 7(63%) infants in the derivative and validation cohorts, respectively, with one missed BA in the latter. We propose a validated, simple, yet sensitive diagnostic score to risk-stratify cholestatic infants, aiming to expedite definitive management of BA.
Identifiants
pubmed: 39085697
doi: 10.1007/s00383-024-05785-y
pii: 10.1007/s00383-024-05785-y
doi:
Substances chimiques
Bilirubin
RFM9X3LJ49
gamma-Glutamyltransferase
EC 2.3.2.2
Types de publication
Journal Article
Validation Study
Langues
eng
Sous-ensembles de citation
IM
Pagination
212Informations de copyright
© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.
Références
Feldman AG, Sokol RJ (2013) Neonatal cholestasis. NeoReviews 14(2):e63–e73
doi: 10.1542/neo.14-2-e63
Chardot C (2006) Biliary atresia. Orphanet J Rare Dis 1:1–9
doi: 10.1186/1750-1172-1-28
Scottoni F, Davenport M (2020) Biliary atresia: potential for a new decade. Semin Pediatr Surg 29(4):150940
doi: 10.1016/j.sempedsurg.2020.150940
pubmed: 32861444
Chiu CY, Chen PH, Chan CF, Chang MH, Wu TC, Group T. I. S. C. C. S. (2013) Biliary atresia in preterm infants in Taiwan: a nationwide survey. J Pediatr 163(1):100-103.e1
doi: 10.1016/j.jpeds.2012.12.085
pubmed: 23414661
Nio M, Wada M, Sasaki H, Tanaka H (2015) Effects of age at Kasai portoenterostomy on the surgical outcome: a review of the literature. Surg Today 45:813–818
doi: 10.1007/s00595-014-1024-z
pubmed: 25212566
Kwong AJ, Ebel NH, Kim WR, Lake JR, Smith JM, Schladt DP, Skeans MA, Foutz J, Gauntt K, Cafarella M (2022) OPTN/SRTR 2020 annual data report: liver. Am J Transpl 22:204–309
doi: 10.1111/ajt.16978
Wang L, Yang Y, Chen Y, Zhan J (2018) Early differential diagnosis methods of biliary atresia: a meta-analysis. Pediatr Surg Int 34:363–380
doi: 10.1007/s00383-018-4229-1
pubmed: 29397405
Collins GS, Reitsma JB, Altman DG, Moons KG (2015) Transparent reporting of a multivariable prediction model for individual prognosis or diagnosis (TRIPOD): the TRIPOD statement. Ann Intern Med 162(1):55–63
doi: 10.7326/M14-0697
pubmed: 25560714
Kanegawa K, Akasaka Y, Kitamura E, Nishiyama S, Muraji T, Nishijima E, Satoh S, Tsugawa C (2003) Sonographic diagnosis of biliary atresia in pediatric patients using the “triangular cord” sign versus gallbladder length and contraction. Am J Roentgenol 181(5):1387–1390
doi: 10.2214/ajr.181.5.1811387
Choi S-O, Park W-H, Lee H-J, Woo S-K (1996) ‘Triangular cord’: a sonographic finding applicable in the diagnosis of biliary atresia. J Pediatr Surg 31(3):363–366
doi: 10.1016/S0022-3468(96)90739-3
pubmed: 8708904
Roquete ML, Ferreira AR, Fagundes ED, Castro LP, Silva RA, Penna FJ (2008) Accuracy of echogenic periportal enlargement image in ultrasonographic exams and histopathology in differential diagnosis of biliary atresia. J de Pediatr 84:331–336
Zhou L, Shan Q, Tian W, Wang Z, Liang J, Xie X (2016) Ultrasound for the diagnosis of biliary atresia: a meta-analysis. Am J Roentgenol 206(5):W73–W82
doi: 10.2214/AJR.15.15336
Napolitano M, Franchi-Abella S, Damasio MB, Augdal TA, Avni FE, Bruno C, Darge K, Ključevšek D, Littooij AS, Lobo L (2021) Practical approach to imaging diagnosis of biliary atresia, part 1: prenatal ultrasound and magnetic resonance imaging, and postnatal ultrasound. Pediatr Radiol 51:314–331
doi: 10.1007/s00247-020-04840-9
pubmed: 33201318
Shneider BL, Moore J, Kerkar N, Magee JC, Ye W, Karpen SJ, Kamath BM, Molleston JP, Bezerra JA, Murray KF (2017) Initial assessment of the infant with neonatal cholestasis—Is this biliary atresia? PLoS ONE 12(5):e0176275
doi: 10.1371/journal.pone.0176275
pubmed: 28493866
pmcid: 5426590
Brahee DD, Lampl BS (2022) Neonatal diagnosis of biliary atresia: a practical review and update. Pediatr Radiol 52(4):685–692
doi: 10.1007/s00247-021-05148-y
pubmed: 34331566
Bezerra JA, Wells RG, Mack CL, Karpen SJ, Hoofnagle JH, Doo E, Sokol RJ (2018) Biliary atresia: clinical and research challenges for the twenty-first century. Hepatology 68(3):1163–1173
doi: 10.1002/hep.29905
pubmed: 29604222
El-Guindi MA-S, Sira MM, Sira AM, Salem TA-H, El-Abd OL, Konsowa HA-S, El-Azab DS, Allam AA-H (2014) Design and validation of a diagnostic score for biliary atresia. J Hepatol 61(1):116–123
doi: 10.1016/j.jhep.2014.03.016
pubmed: 24657403
Dong R, Jiang J, Zhang S, Shen Z, Chen G, Huang Y, Zheng Y, Zheng S (2018) Development and validation of novel diagnostic models for biliary atresia in a large cohort of Chinese patients. EBioMedicine 34:223–230
doi: 10.1016/j.ebiom.2018.07.025
pubmed: 30077722
pmcid: 6116426
Kim JR, Hwang J-Y, Yoon HM, Jung AY, Lee JS, Kim JS, Namgoong J-M, Kim DY, Oh SH, Kim KM (2018) Risk estimation for biliary atresia in patients with neonatal cholestasis: development and validation of a risk score. Radiology 288(1):262–269
doi: 10.1148/radiol.2018172390
pubmed: 29634437
Zhao D, Gu S, Gong X, Li Y, Sun X, Chen Y, Deng Z, Zhang Y (2021) Web-based calculator for biliary atresia screening in neonates and infants with cholestasis. Transl Pediatr 10(2):225
doi: 10.21037/tp-20-170
pubmed: 33708508
pmcid: 7944186
Ali KM, Zalata KR, Barakat T, Elzeiny SM (2024) Pathologic approach to Neonatal cholestasis with a simple scoring system for biliary atresia. Virchows Arch 484(1):93–102
doi: 10.1007/s00428-023-03704-5
pubmed: 38008855
Hsiao C-H, Chang M-H, Chen H-L, Lee H-C, Wu T-C, Lin C-C, Yang Y-J, Chen A-C, Tiao M-M, Lau B-H (2008) Universal screening for biliary atresia using an infant stool color card in Taiwan. Hepatology 47(4):1233–1240
doi: 10.1002/hep.22182
pubmed: 18306391
Matsui A (2017) Screening for biliary atresia. Pediatr Surg Int 33:1305–1313
doi: 10.1007/s00383-017-4175-3
pubmed: 28983697
Lee SM, Cheon J-E, Choi YH, Kim WS, Cho H-H, Kim I-O, You SK (2015) Ultrasonographic diagnosis of biliary atresia based on a decision-making tree model. Korean J Radiol 16(6):1364
doi: 10.3348/kjr.2015.16.6.1364
pubmed: 26576128
pmcid: 4644760
Tang K-S, Huang L-T, Huang Y-H, Lai C-Y, Wu C-H, Wang S-M, Hwang K-P, Huang F-C, Tiao M-M (2007) Gamma-glutamyl transferase in the diagnosis of biliary atresia. Acta Paediatr Taiwan 48(4):196
pubmed: 18265540
Rendón-Macías ME, Villasís-Keever MA, Castañeda-Muciño G, Sandoval-Mex AM (2008) Improvement in accuracy of gamma-glutamyl transferase for differential diagnosis of biliary atresia by correlation with age. Turk J Pediatr 50(3):253
pubmed: 18773671
Chen X, Dong R, Shen Z, Yan W, Zheng S (2016) Value of gamma-glutamyl transpeptidase for diagnosis of biliary atresia by correlation with age. J Pediatr Gastroenterol Nutr 63(3):370–373
doi: 10.1097/MPG.0000000000001168
pubmed: 26963938
Liao, F.-M., Chang, K.-C., Wu, J.-F., Chen, H.-L., Ni, Y.-H., Chang, M.-H. (2022) Direct bilirubin and risk of biliary atresia. Pediatrics 149(6). www.pediatrics.org/cgi/doi/10.1542/peds.2021-053073