Clinical Parameters and Outcomes of Biliary Atresia in Children: A Single Centre Study

Authors

  • Raazia Nawaz Department of Pediatric, Combined Military Hospital/National University of Medical Sciences (NUMS) Rawalpindi Pakistan
  • Farooq Ikram Department of Pediatric, Combined Military Hospital/National University of Medical Sciences (NUMS) Rawalpindi Pakistan
  • Bilal Department of Pediatric, Combined Military Hospital/National University of Medical Sciences (NUMS) Rawalpindi Pakistan
  • Saba Idrees Department of Pediatric, Combined Military Hospital/National University of Medical Sciences (NUMS) Rawalpindi Pakistan
  • Ifnan Shamraiz Department of Pediatric, Combined Military Hospital/National University of Medical Sciences (NUMS) Rawalpindi Pakistan
  • Samina Tabassum Department of Pediatric, Combined Military Hospital/National University of Medical Sciences (NUMS) Rawalpindi Pakistan

DOI:

https://doi.org/10.51253/pafmj.v74i4.9280

Keywords:

Biliary atresia, Gamma glutamyl transferase, Hepatic portoenterostomy, Jaundice, Kasai.

Abstract

Objective: To assess the clinical and laboratory parameters, interventions and their outcomes in children presenting with biliary atresia at a tertiary care hospital.

Study Design: Prospective longitudinal study.

Place and Duration of study: Pediatric Gastroenterology and Hepatology Department of Combined Military Hospital, Rawalpindi Pakistan, from Feb 2021 to Jan 2022.

Methodology: Patients diagnosed with extrahepatic biliary atresia on the basis of liver histopathology and intra operative cholangiogram were enrolled consecutively during this period. The clinical parameters, liver function tests, radiological investigations and histopathology findings were recorded and correlated. The impact of Kasai procedure on outcome and prognosis of biliary atresia patients was noted along with an interval analysis at 1 year of age.

Results: Forty patients were studied including 26 males and 14 females. Thirty (75%) patients underwent Kasai at a median age of 3.14±1.2 months with a success rate of 66.6% (p-value<0.01). No significant correlation was established between age at Kasai Hepatic Portoenterostomy and success of Kasai Hepatic Portoenterostomy (p-value>0.05). A statistically significant relation was established between one-year outcomes of patients undergoing Kasai Hepatic Portoenterostomy and without Kasai Hepatic Portoenterostomy (p-value 0.01). A significant difference in survival of patients undergoing Kasai Hepatic Portoenterostomy and those without Kasai Hepatic Portoenterostomy (p< 0.001) was observed.

Conclusion:  Intervention in the form of Kasai Hepatic Portoenterostomy significantly contributes towards an improved outcome in patients with Biliary Atresia.

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References

Ortiz-Perez A, Donnelly B, Temple H, Tiao G, Bansal R, Mohanty SK. Innate immunity and pathogenesis of biliary atresia. Front Immunol 2020; 11:329.

https://doi.org/10.3389/fimmu.2020.00329

Liu J, Chen W, Zhou M, Li W, Tang J, Zhou Q. A Nomogram Predicting the Prognosis of Children with Biliary Atresia After Hepatoportoenterostomy. Front Pediatr 2021; 9: 641318.

https://doi.org/10.3389/fped.2021.641318

Malik A, Thanekar U, Mourya R, Shivakumar P. Recent developments in etiology and disease modeling of biliary atresia: a narrative review. Dig Med Res 2020; 3: 59.

https://doi.org/10.21037%2Fdmr-20-97

Wehrman A, Waisbourd-Zinman O, Wells RG. Recent advances in understanding biliary atresia. F1000Res 2019: 8: F1000 https://doi.org/10.12688%2Ff1000research.16732.1

Fawaz R, Baumann U, Ekong U. Guideline for the evaluation of cholestatic jaundice in infants: joint recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr 2017; 64(1): 154-168.

Wildhaber BE. Biliary atresia: 50 years after the first Kasai. Int Schol Res Notices 2012; 2012(1): 132089.

https://doi.org/10.5402/2012/132089

Sanchez-Valle A, Kassira N, Varela VC, Radu SC, Paidas C, Kirby RS. Biliary atresia: epidemiology, genetics, clinical update, and public health perspective. Adv Pediatr 2017; 64(1): 285-305.

https://doi.org/10.1016/j.yapd.2017.03.012

Bezerra JA, Wells RG, Mack CL, Karpen SJ, Hoofnagle JH, Doo E, et al. Biliary atresia: clinical and research challenges for the twenty‐first century. Hepatology 2018; 68(3): 1163-1173.

https://doi.org/10.1002/hep.29905

Liu J, Dai S, Chen G, Sun S, Jiang J, Zheng S, et al. Diagnostic Value and Effectiveness of an Artificial Neural Network in Biliary Atresia. Front Pediatr 2020; 8: 409.

https://doi.org/10.3389/fped.2020.00409

Mohamadien NR, Makboul R, Galal SM, Mostafa NM. Role of hepatobiliary scintigraphy with different semi-quantitative parameters along with histopathological scoring in differentiating biliary atresia from neonatal hepatitis. Egypt J Radiol Nucl Med 2021; 52: 111.

https://doi.org/10.1186/s43055-021-00482-5

Neto B, Borges-Dias M, Trindade E, Estevão-Costa J, Campos JM. Biliary atresia-clinical series. GE-Portuguese. J Gastroenterol 2018; 25(2): 68-73. https://doi.org/10.1159/000480708

Chen SY, Lin CC, Tsan YT, Chan WC, Wang JD, Chou YJ, et al. Number of cholangitis episodes as a prognostic marker to predict timing of liver transplantation in biliary atresia patients after Kasai portoenterostomy. BMC Pediatr 2018; 18(1): 1-7.

https://doi.org/10.1186/s12887-018-1074-2

Nightingale S, Stormon MO, O’Loughlin EV, Shun A, Thomas G, Benchimol EI, et al. Early posthepatoportoenterostomy predictors of native liver survival in biliary atresia. J Pediatr Gastroenterol Nutr 2017; 64(2): 203-209.

https://doi.org/10.1097/MPG.0000000000001289

Khayat A, Alamri AM, Saadah OI. Outcomes of late Kasai portoenterostomy in biliary atresia: a single-center experience. J Int Med Res 2021; 49(5): 03000605211012596.

https://doi.org/10.1177/03000605211012596

Nio M. Japanese biliary atresia registry. Pediatr. Surg. Int 2017; 33(12): 1319-1325.

https://doi.org/10.1007/s00383-017-4160-x

Yassin NA, El-Tagy G, Abdelhakeem ON, Asem N, El-Karaksy H. Predictors of short-term outcome of Kasai portoenterostomy for biliary atresia in infants: a single-center study. Pediatr Gastroenterol Nutr 2020; 23(3): 266.

https://doi.org/10.5223/pghn.2020.23.3.266

Kumar R, Lal BB, Sood V, Khanna R, Kumar S, Bharathy KG, et al. Predictors of successful Kasai portoenterostomy and survival with native liver at 2 years in infants with biliary atresia. J Clin Exp Hepatol 2019; 9(4): 453-459.

https://doi.org/10.1016/j.jceh.2018.09.008

Noorulla F, Dedon R, Maisels MJ. Association of early direct bilirubin levels and biliary atresia among neonates. JAMA Netw Open 2019; 2(10): e1913321.

https://doi.org/10.1001/jamanetworkopen.2019.13321

Ho A, Sacks MA, Sapra A, Khan FA. The Utility of Gallbladder Absence on Ultrasound for Children with Biliary Atresia. Front Pediatr 2021; 9: 530. https://doi.org/10.3389/fped.2021.685268

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Published

30-08-2024

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Original Articles

How to Cite

1.
Raazia Nawaz, Farooq Ikram, Bilal, Saba Idrees, Ifnan Shamraiz, Samina Tabassum. Clinical Parameters and Outcomes of Biliary Atresia in Children: A Single Centre Study. Pak Armed Forces Med J [Internet]. 2024 Aug. 30 [cited 2024 Dec. 22];74(4):956-60. Available from: https://pafmj.org/PAFMJ/article/view/9280