Abstract
The association between Down syndrome and congenital portosystemic shunts, most commonly caused by patent ductus venosus, remains relatively unknown. The authors present a girl with Down syndrome with patent ductus venosus, pancreaticobiliary maljunction and paucity of interlobular bile ducts, presenting with neonatal cholestasis and transient abnormal myeloproliferative disorder. To the best of authors’ knowledge, no report of the concurrent presence of the above in Down syndrome has been published.
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References
Jain M, Singh A, Mantan M, Kapoor S. Evaluation of structural anomalies of kidney and urinary tract in children with Down syndrome. Indian J Pediatr. 2014;81:734.
Narayanan DL, Yesodharan D, Kappanayil M, et al. Cardiac spectrum, cytogenetic analysis and thyroid profile of 418 children with Down syndrome from South India: a cross-sectional study. Indian J Pediatr. 2014;81:547–51.
Greene AK, Kim S, Rogers GF, Fishman SJ, Olsen BR, Mulliken JB. Risk of vascular anomalies with Down syndrome. Pediatrics. 2008;121:e135–40.
Arnell H, Fischler B. Population-based study of incidence and clinical outcome of neonatal cholestasis in patients with Down syndrome. J Pediatr. 2012;161:899–902.
Chiaie LD, Neuberger P, Von KT. Congenital intrahepatic portosystemic shunt:prenatal diagnosis and possible influence on fetal growth. Ultrasound Obstet Gynecol. 2008;32:233–5.
Sood V, Khanna R, Alam S, Rawat D, Bhatnagar S, Rastogi A. Ductal paucity and Warkany syndrome in a patient with congenital extrahepaticportocaval shunt. World J Hepatol. 2014;6:358–62.
Bernard O, Franchi-Abella S, Branchereau S, Pariente D, Gauthier F, Jacquemin E. Congenital portosystemic shunts in children: recognition, evaluation, and management. Semin Liver Dis. 2012;32:273–87.
Sokollik C, Bandsma RH, Gana JC, van den Heuvel M, Ling SC. Congenital portosystemic shunt: characterization of a multisystem disease. J Pediatr Gastroenterol Nutr. 2013;56:675–81.
Franchi-Abella S, Branchereau S, Lambert V, et al. Complications of congenital portosystemic shunts in children: therapeutic options and outcomes. J Pediatr Gastroenterol Nutr. 2010;51:322–30.
Acknowledgments
The authors thank Dr. Christine Kwan for editing and revising this case report and Dr. Kanako Hatanaka for providing pathological specimens.
Contributions
HY is the attending doctor and wrote the article; KK is the sub-attending doctor and helped to perform the examination; SH performed patient’s surgery; OT and AT also performed patient’s surgery and helped to draft the manuscript; SI is a chief of NICU in Teine Keijinkai Hospital and helped to perform examinations for the patient and helped to draft the manuscript. SI will act as guarantor for this paper.
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Yamaguchi, H., Kosugiyama, K., Honda, S. et al. Down Syndrome with Patent Ductus Venosus and Hepato-Biliary-Pancreatic Abnormalities. Indian J Pediatr 83, 78–80 (2016). https://doi.org/10.1007/s12098-015-1797-0
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DOI: https://doi.org/10.1007/s12098-015-1797-0