Analysis of health care delivery in patients with biliary atresia in a tertiary hospital in Northeast Brazil
DOI:
https://doi.org/10.11606/issn.1679-9836.v101i1e-159916Keywords:
Biliary atresia, Hepatic portoenterostomy, Diagnosis, Epidemiology, Prognosis, ChildAbstract
Introduction: Biliary atresia (BA) is a rare pediatric pathology, but it is the main cause of liver transplantation in children, if not reversed with the realization of the Roux-en-Y portoentorostomy (Kasai surgery) in adequate time. There is a tendency for the surgical procedure to be delayed throughout Brazil and an investigation of its cause is necessary. Objective: to determine the epidemiological profile of the patient with BA and to evaluate the health care focused on it at a tertiary hospital. Methods: a descriptive, retrospective, cross-sectional study was performed using medical records of patients with a diagnosis of BA followed up at a tertiary hospital between 1996 and 2015. Results: the initial sample had 72 patients, and only 52 patients had complete records available from the hospital´s archive. Among the 26 patients included, the mean age at admission was 87,9 days (± 57,9), the ultrasonography (USG) was performed in 24 cases, and it was repeated in 10. The Kasai surgery were performed in 50% of the patients, 38.4% of which were at the appropriate time. Of the patients who were operated on late, 25% were transplanted and 50% died, while among the 4 operated on time, only one was transplanted and there were no deaths. Conclusion: the diagnostic difficulties, in particular, failure to suspect BA in primary or tertiary care, in addition to a percentage of 41.6% of USG with false-negative results, delayed the surgical moment, confirming the worst outcome of the late-onset patient.
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Carvalho E de, Ivantes CAP, Bezerra JA. Extrahepatic biliary atresia: current concepts and future directions. J Pediatr (Rio J). 2007;83(2):105–20. doi: https://doi.org/10.2223/JPED.1608.
Santos JL, Carvalho E, Bezerra JA. Advances in biliary atresia: from patient care to research. Brazilian J Med Biol Res. 2010;43(6):522–7. doi: https://doi.org/10.1590/S0100-879X2010007500035
Hadzić N, Davenport M, Tizzard S, Singer J, Howard ER, Mieli-Vergani G. Long-term survival following Kasai portoenterostomy: is chronic liver disease inevitable? J Pediatr Gastroenterol Nutr. 2003;37(4):430–3. doi: https://doi.org/10.1097/00005176-200310000-00006.
Moreira RK, Cabral R, Cowles RA, Lobritto SJ. Biliary Atresia: a multidisciplinary approach to diagnosis and management. Arch Pathol Lab Med. 2012;136(7):746–60. doi: https://doi.org/10.5858/arpa.2011-0623-RA
Cauduro SM. Extrahepatic biliary atresia: diagnostic methods. J Pediatr (Rio J). 2003;79(2):107-14. https://doi.org/10.1590/S0021-75572003000200004.
Yoon PW, Bresee JS, Olney RS, James LM, Khoury MJ. Epidemiology of biliary atresia: a population-based study. Pediatrics. 1997;99(3):376-82. doi: https://doi.org/10.1542/peds.99.3.376.
Nio M, Ohi R, Miyano T, Saeki M, Shiraki K, Tanaka K. Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese Biliary Atresia Registry. J Pediatr Surg. 2003;38(7):997-1000. doi: https://doi.org/10.1016/s0022-3468(03)00178-7.
McKiernan PJ, Baker a J, Kelly D a. The frequency and outcome of biliary atresia in the UK and Ireland... including commentary by Rothwell PM. Lancet. 2000;355(9197):25-30. doi: https://doi.org/10.1016/S0140-6736(99)03492-3.
Narkewicz MR. Biliary atresia: an update on our understanding of the disorder. Curr Opin Pediatr. 2001;13(5):435-40. doi: https://doi.org/10.1097/00008480-200110000-00009.
Sokol RJ, Mack C, Narkewicz MR, Karrer FM. Pathogenesis and outcome of biliary atresia: current concepts. J Pediatr Gastroenterol Nutr. 2003;37(1):4-21. doi: https://doi.org/10.1097/00005176-200307000-00003.
Davenport M. Biliary atresia. Semin Pediatr Surg. 2005;14:42–8. doi: https://doi.org/10.1053/j.sempedsurg.2004.10.024.
Bassett MD, Murray KF. Biliary atresia: recent progress. J Clin Gastroenterol. 2008;42(6):720–9. doi: https://doi.org/10.1097/MCG.0b013e3181646730.
Khalil BA, Perera MTPR, Mirza DF. Clinical practice: Eur J Pediatr. 2010;169(4):395-402. doi: https://doi.org/10.1007/s00431-009-1125-7.
Nakamura K, Tanoue A. Etiology of biliary atresia as a developmental anomaly: Recent advances. J Hepatobiliary Pancreat Sci. 2013;20(5):459–64. doi: https://doi.org/10.1007/s00534-013-0604-4.
Kobayashi H, Yamataka A, Lane GJ, Miyano T. Levels of circulating antiinflammatory cytokine interleukin-1 receptor antagonist and proinflammatory cytokines at different stages of biliary atresia. J Pediatr Surg. 2002;37(7):1038–41. doi: 10.1053/jpsu.2002.33840.
Bezerra JA, Tiao G, Ryckman FC, Alonso M, Sabla GE, Shneider B, et al.. Genetic induction of proinflammatory immunity in children with biliary atresia. Lancet. 2002;360(9346):1653–9. DOI: https://doi.org/10.1016/S0140-6736(02)11603-5.
Davenport M, Gonde C, Redkar R, Koukoulis G, Tredger M, Mieli-Vergani G, et al.. Immunohistochemistry of the liver and biliary tree in extrahepatic biliary atresia. J Pediatr Surg. 2001;36(7):1017–25. DOI: 10.1053/jpsu.2001.24730.
Mack CL, Tucker RM, Sokol RJ, Karrer FM, Kotzin BL, Whitington PF, et al.. Biliary atresia is associated with CD4+ Th1 cell-mediated portal tract inflammation. Pediatr Res. 2004;56(1):79–87. doi: https://doi.org/10.1203/01.PDR.0000130480.51066.FB.
Fung KP, Lau SP, Name F, Lau S. P. Differentiation between extrahepatic and intrahepatic cholestasis by discriminant analysis. J Paediatr Child Heal. 1990;26(3):132–5. doi: https://doi.org/10.1111/j.1440-1754.1990.tb02409.x.
Choi SO, Park WH, Lee HJ, Woo SK. “Triangular cord”: A sonographic finding applicable in the diagnosis of biliary atresia. J Pediatr Surg. 1996;31(3):363–6. doi: https://doi.org/10.1016/s0022-3468(96)90739-3.
Park W-H, Choi S-O, Kim S-P, Zeon S-K, Lee S-L. A New Diagnostic Approach to Biliary Atresia With Emphasis. J Pediatr Surg. 1997;32(11):1555–9. doi: https://doi.org/10.1016/s0022-3468(97)90451-6.
Jaw TS, Kuo YT, Liu GC, Chen SH, Wang CK. MR cholangiography in the evaluation of neonatal cholestasis. Radiology. 1999;212(1):249–56. doi: https://doi.org/10.1148/radiology.212.1.r99jl13249.
Chardot C, Carton M, Spire-Bendelac N, Le Pommelet C, Golmard JL, Reding R, et al.. Is the Kasai operation still indicated in children older than 3 months diagnosed with biliary atresia? J Pediatr. 2001;138(2):224–8. doi: https://doi.org/10.1067/mpd.2001.111276.
Serinet M-O, Wildhaber BE, Broué P, Lachaux A, Sarles J, Jacquemin E, et al.. Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screening. Pediatrics. 2009;123(5):1280–6. doi: https://doi.org/10.1542/peds.2008-1949.
Lazaridis KN. The Cholangiopathies Konstantinos. Mayo Clin Proc. 2015;90(6):791–800. doi: https://doi.org/10.1016/j.mayocp.2015.03.017.
de Carvalho E, dos Santos JL, da Silveira TR, Kieling CO, Silva LR, Porta G, et al.. Biliary atresia: the Brazilian experience. J Pediatr (Rio J). 2010;86(6):473–9. doi: https://doi.org/10.2223/JPED.2054.
Kieling CO, Santos JL Dos, Vieira SMG, Ferreira CT, Linhares ARR, Lorentz AL, et al.. Biliary atresia: we still operate too late. J Pediatr (Rio J). 2008;84(5):436–41. doi: https://doi.org/10.2223/JPED.1825.
Oliveira J De, Silva LR. Atresia das vias biliares : perfil clínico e epidemiológico dos pacientes pediátricos em serviço de referência do estado da Bahia. Rev Cien Med Biol. 2012;48–53. doi: https://doi.org/10.9771/cmbio.v11i1.5799.
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