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International Journal of General Medicine Volume 16, 2023 - Issue
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ORIGINAL RESEARCH

Clinical and Laboratory Characteristics in Children with Alagille Syndrome: Experience of a Single Center

Dalei LiDepartment of Pediatric Internal Medicine, The First People’s Hospital of Lianyungang & Xuzhou Medical University Affiliated Hospital of Lianyungang & The First Affiliated Hospital of Kangda College of Nanjing Medical University, Lianyungang, 222061, People’s Republic of ChinaView further author information
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Kangwei MaoDepartment of Pediatric Internal Medicine, The First People’s Hospital of Lianyungang & Xuzhou Medical University Affiliated Hospital of Lianyungang & The First Affiliated Hospital of Kangda College of Nanjing Medical University, Lianyungang, 222061, People’s Republic of ChinaCorrespondence[email protected]
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Jun SunDepartment of Pediatric Internal Medicine, The First People’s Hospital of Lianyungang & Xuzhou Medical University Affiliated Hospital of Lianyungang & The First Affiliated Hospital of Kangda College of Nanjing Medical University, Lianyungang, 222061, People’s Republic of ChinaView further author information
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Jiangyang LiuDepartment of Pediatric Internal Medicine, The First People’s Hospital of Lianyungang & Xuzhou Medical University Affiliated Hospital of Lianyungang & The First Affiliated Hospital of Kangda College of Nanjing Medical University, Lianyungang, 222061, People’s Republic of ChinaView further author information
&
Chenxi ZhangDepartment of Pediatric Internal Medicine, The First People’s Hospital of Lianyungang & Xuzhou Medical University Affiliated Hospital of Lianyungang & The First Affiliated Hospital of Kangda College of Nanjing Medical University, Lianyungang, 222061, People’s Republic of ChinaView further author information
Pages 77-83 | Received 19 Jul 2022, Accepted 09 Nov 2022, Published online: 06 Jan 2023

References

  • Benabed Y, Chaillou E, Denis MC, et al. Alagille syndrome: a case report. Ann Biol Clin. 2018;76(6):675–680.
  • Chen HL, Wu SH, Hsu SH, et al. Jaundice revisited: recent advances in the diagnosis and treatment of inherited cholestatic liver diseases. J Biomed Sci. 2018;25(1):75. doi:10.1186/s12929-018-0475-8
  • Mitchell E, Gilbert M, Loomes KM. Alagille syndrome. Clin Liver Dis. 2018;22(4):625–641. doi:10.1016/j.cld.2018.06.001
  • Diaz-Frias J, Kondamudi NP. Alagille syndrome. In: StatPearls. Treasure Island (FL): StatPearls Publishing; 2021.
  • Kamath BM, Baker A, Houwen R, et al. Systematic review: the epidemiology, natural history, and burden of alagille syndrome. J Pediatr Gastroenterol Nutr. 2018;67(2):148–156. doi:10.1097/MPG.0000000000001958
  • Bray SJ. Notch signalling in context. Nat Rev Mol Cell Biol. 2016;17(11):722–735. doi:10.1038/nrm.2016.94
  • Grochowski CM, Loomes KM, Spinner NB. Jagged1 (JAG1): structure, expression, and disease associations. Gene. 2016;576(1 Pt 3):381–384. doi:10.1016/j.gene.2015.10.065
  • Leonard LD, Chao G, Baker A, et al. Clinical utility gene card for: alagille Syndrome (ALGS). Eur J Hum Genet. 2014;22(3):435. doi:10.1038/ejhg.2013.140
  • Kamath BM, Suchy FJ, Sokal RJ, et al. Liver Disease in Children. 3rd ed. New York: CambridgeUniversity Press; 2007:326–345.
  • Guru Murthy GS, Rana BS, Das A, et al. Alagille syndrome: a rare disease in an adolescent. Dig Dis Sci. 2012;57(11):3035–3037. doi:10.1007/s10620-012-2226-0
  • Kohut TJ, Gilbert MA, Loomes KM. Alagille syndrome: a focused review on clinical features, genetics, and treatment. Semin Liver Dis. 2021;41(4):525–537. doi:10.1055/s-0041-1730951
  • Gilbert MA, Loomes KM. Alagille syndrome and non-syndromic paucity of the intrahepatic bile ducts. Transl Gastroenterol Hepatol. 2021;6:22. doi:10.21037/tgh-2020-03
  • Kamath BM, Munoz PS, Bab N, et al. A longitudinal study to identify laboratory predictors of liver disease outcome in Alagille syndrome. J Pediatr Gastroenterol Nutr. 2010;50(5):526–530. doi:10.1097/MPG.0b013e3181cea48d
  • Saleh M, Kamath BM, Chitayat D. Alagille syndrome: clinical perspectives. Appl Clin Genet. 2016;9:75–82. doi:10.2147/TACG.S86420
  • Jesina D. Alagille Syndrome: an Overview. Neonatal Netw. 2017;36(6):343–347. doi:10.1891/0730-0832.36.6.343
  • Rovner AJ, Schall JI, Jawad AF, et al. Rethinking growth failure in Alagille syndrome: the role of dietary intake and steatorrhea. J Pediatr Gastroenterol Nutr. 2002;35(4):495–502. doi:10.1097/00005176-200210000-00007
  • Zhang W, Zhao X, Huang J, et al. Alagille syndrome: an uncommon cause of intrahepatic cholestasis in adults. Rev Esp Enferm Dig. 2019;111(4):323–326. doi:10.17235/reed.2019.5679/2018
  • Ayoub MD, Kamath BM. Alagille syndrome: diagnostic challenges and advances in management. Diagnostics. 2020;10(11):907. doi:10.3390/diagnostics10110907
  • Turnpenny PD, Ellard S. Alagille syndrome: pathogenesis, diagnosis and management. Eur J Hum Genet. 2012;20(3):251–257. doi:10.1038/ejhg.2011.181
  • Yang WH, Zhang L, Xue FS, Riaz A, Zhu ZJ. Pediatric liver transplantation for alagille syndrome: anesthetic evaluation and perioperative management. Ann Transplant. 2020;25:e924282. doi:10.12659/AOT.924282
  • Berniczei-Royko A, Chałas R, Mitura I, Nagy K, Prussak E. Medical and dental management of Alagille syndrome: a review. Med Sci Monit. 2014;20:476–480. doi:10.12659/MSM.890577
  • Sanderson E, Newman V, Haigh SF, et al. Vertebral anomalies in children with Alagille syndrome: an analysis of 50 consecutive patients. Pediatr Radiol. 2002;32(2):114–119. doi:10.1007/s00247-001-0599-x
  • Fischetto R, Palmieri VV, Tripaldi ME, et al. Alagille syndrome: a novel mutation in JAG1 gene. Front Pediatr. 2019;7:199. doi:10.3389/fped.2019.00199
  • Andersson ER, Chivukula IV, Hankeova S, et al. Mouse model of alagille syndrome and mechanisms of jagged1 missense mutations. Gastroenterology. 2018;154(4):1080–1095. doi:10.1053/j.gastro.2017.11.002

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