Colestasis neonatal: Un abordaje desafiante para los pediatras ante los principales diagnósticos diferenciales y tratamientos

Autores/as

DOI:

https://doi.org/10.33448/rsd-v13i5.45796

Palabras clave:

Colestasis; Neonatal; Hiperbilirrubinemia; Atresia biliar; Colestasis intra-hepática.

Resumen

Los signos clínicos de la colestasis neonatal, como ictericia, acolia y coluria, son fácilmente perceptibles. Sin embargo, corresponde al pediatra diferenciar rápidamente la causa de la hiperbilirrubinemia colestásica debido a la situación de urgencia pediátrica cuyo diagnóstico tardío puede ser potencialmente grave y letal. Este estudio tuvo como objetivo identificar las principales causas de ictericia colestásica en recién nacidos y lactantes y cómo los pediatras pueden realizar una aproximación diagnóstica temprana e iniciar el tratamiento. Al tratarse de un artículo de revisión integradora, se propone ayudar prácticamente a abordar los principales diagnósticos diferenciales de la ictericia colestásica y las terapias disponibles. Las bases bibliográficas investigadas: Cocrhane, Embase, Medline y Pubmed. Los criterios de selección: artículos gratuitos completos entre 2019 y 2024, en portugués, inglés o español, y la presencia de al menos dos de los descriptores. Por lo tanto, en el caso de un recién nacido con ictericia mayor de dos semanas, el pediatra debe solicitar una medición de bilirrubina, ya que la colestasis siempre es patológica con valores directos mayores a 1,0 mg/dl y las causas pueden ser extra o intrahepáticas con un cuadro desafiante de diagnóstico diferencial, en el extrahepático tenemos la atresia biliar cuyo abordaje quirúrgico precoz conlleva un pronóstico más favorable, por lo que al investigar la colestasis neonatal el pediatra debe partir de las enfermedades más comunes, priorizando aquellas con tratamiento quirúrgico por el riesgo de mortalidad, siendo fundamental en la investigación la realización de anamnesis del binomio madre-bebé, examen físico detallado, evaluación de las características de las heces, pruebas de laboratorio y de imagen, si es necesario, biopsia con histopatología y derivación al especialista.

Citas

Abbey, P., Kandasamy, D., & Naranje, P. (2019). Neonatal Jaundice. Indian journal of pediatrics, 86(9), 830–841. https://doi.org/10.1007/s12098-019-02856-0

Antala, S., & Taylor, S. A. (2022). Biliary Atresia in Children: Update on Disease Mechanism, Therapies, and Patient Outcomes. Clinics in liver disease, 26(3), 341–354. https://doi.org/10.1016/j.cld.2022.03.001

Bigio, J. Z. D., Tannuri, A. C. A., Falcão, M. C., Matsushita, F. Y., & de Carvalho, W. B. (2024). Factors associated with cholestasis in newborns with gastroschisis. Revista Paulista De Pediatria, 42, e2022152. https://doi.org/10.1590/1984-0462/2024 /42/ 2022152

Chen, G., Liu, J., Huang, Y., Wu, Y., Lu, X., Dong, R., Shen, Z., Sun, S., Jiang, J., & Zheng, S. (2021). Preventive effect of prophylactic intravenous antibiotics against cholangitis in biliary atresia: a randomized controlled trial. Pediatric surgery international, 37(8), 1089–1097. https://doi.org/10.1007/s00383-021-04916-z

Cho, S. J., & Kim, G. E. (2019). A practical approach to the pathology of neonatal cholestatic liver disease. Seminars in diagnostic pathology, 36(6), 375–388. https://doi.org/10.1053/j.semdp.2019.07.004

Di Serafino, M., Gioioso, M., Severino, R., Esposito, F., Vezzali, N., Ferro, F., Pelliccia, P., Caprio, M. G., Iorio, R., & Vallone, G. (2020). Ultrasound findings in paediatric cholestasis: how to image the patient and what to look for. Journal of ultrasound, 23(1), 1–12. https://doi.org/10.1007/s40477-019-00362-9

Feldman, A. G., & Sokol, R. J. (2019). Neonatal cholestasis: emerging molecular diagnostics and potential novel therapeutics. Nature reviews. Gastroenterology & hepatology, 16(6), 346–360. https://doi.org/10.1038/s41575-019-0132-z

Feldman, A. G., & Sokol, R. J. (2020). Recent developments in diagnostics and treatment of neonatal cholestasis. Seminars in pediatric surgery, 29(4), 150945. https://doi.org/10.1016/j.sempedsurg.2020.150945

Feldman, A. G., & Sokol, R. J. (2021). Neonatal Cholestasis: Updates on Diagnostics, Therapeutics, and Prevention. NeoReviews, 22(12), e819–e836. https://doi.org/10.1542/neo.22-12-e819

Fligor, S. C., Hirsch, T. I., Tsikis, S. T., Adeola, A., & Puder, M. (2022). Current and emerging adjuvant therapies in biliary atresia. Frontiers in pediatrics, 10, 1007813. https://doi.org/10.3389/fped.2022.1007813

Gunda, S. T., Chambara, N., Chen, X. F., Pang, M. Y. C., & Ying, M. T. (2022). Diagnostic Efficacy of Advanced Ultrasonography Imaging Techniques in Infants with Biliary Atresia (BA): A Systematic Review and Meta-Analysis. Children (Basel, Switzerland), 9(11), 1676. https://doi.org/10.3390/children9111676

Halder, A., Patra, S., Mandal, B., Ray, G., Ghosh, R., Mukherjee, S., & Chatterjee, U. (2023). Differentiating biliary atresia from other causes of infantile cholestasis: An appraisal of the histomorphological changes on liver biopsy. Indian journal of pathology & microbiology, 66(4), 790–798. https://doi.org/10.4103/ijpm.ijpm_215_22

Heinz, N., & Vittorio, J. (2023). Treatment of Cholestasis in Infants and Young Children. Current gastroenterology reports, 25(11), 344–354. https://doi.org/10.1007/s11894-023-00891-8

Kapoor, V., Malviya, M. N., & Soll, R. (2019). Lipid emulsions for parenterally fed preterm infants. The Cochrane database of systematic reviews, 6(6), CD013163. https://doi.org/10.1002/14651858.CD013163.pub2

Kriegermeier, A., & Green, R. (2020). Pediatric Cholestatic Liver Disease: Review of Bile Acid Metabolism and Discussion of Current and Emerging Therapies. Frontiers in medicine, 7, 149. https://doi.org/10.3389/fmed.2020.00149

Latorraca, C. D. O. C., Rodrigues, M., Pacheco, R. L., Martimbianco, A. L. C., & Riera, R. (2019). Busca em bases de dados eletrônicas da área da saúde: por onde começar. Diagn Tratamento, 24(2), 59-63.

Lew-Tusk, A., Pęksa, M., & Stachowicz-Stencel, T. (2023). Recent studies on non-invasive biomarkers useful in biliary atresia - a literature review. Acta biochimica Polonica, 70(3), 475–480. https://doi.org/10.18388/abp.2020_6858

Li, Y., Jiang, J., & Wang, H. (2022). Ultrasound elastography in the diagnosis of biliary atresia in pediatric surgery: a systematic review and meta-analysis of diagnostic test. Translational pediatrics, 11(5), 748–756. https://doi.org/10.21037/tp-22-159

Liu, S., Yang, Q., Ji, Q., Wang, Z., Sun, R., & Zhan, J. (2024). Effect of Kasai procedure on liver transplantation in children with biliary atresia: a systematic review and updated meta-analysis. Translational pediatrics, 13(1), 10–25. https://doi.org/10.21037/tp-23-504

Li, T., Zheng, Q., Zhang, R., Liu, S., Lin, Y., & Zhan, J. (2022). A novel model based on immune-related genes for differentiating biliary atresia from other cholestatic diseases Pediatric surgery international, 39(1), 45 https://doi.org/10.1007/s00383-022-05322-9

Lustosa,A.M.P;Nogueira,H.B.R. et.al,Gastroenterologia Pediatrica.Fortaleza.Apex ,2019,132p

Mancell, S., Islam, M., Dhawan, A., & Whelan, K. (2022). Fat-soluble vitamin assessment, deficiency and supplementation in infants with cholestasis. Journal of human nutrition and dietetics : the official journal of the British Dietetic Association, 35(2), 273–279. https://doi.org/10.1111/jhn.12957

Mysore, K. R., Shneider, B. L., & Harpavat, S. (2019). Biliary Atresia as a Disease Starting In Utero: Implications for Treatment, Diagnosis, and Pathogenesis. Journal of pediatric gastroenterology and nutrition, 69(4), 396–403. https://doi.org/10.1097/MPG.0000000000002450

Nguyen, H. P. A., Ren, J., Butler, M., Li, H., Qazi, S., Sadiq, K., Dao, H. T., & Holterman, A. (2022). Study protocol of Phase 2 open-label multicenter randomized controlled trial for granulocyte-colony stimulating factor (GCSF) in post-Kasai Type 3 biliary atresia. Pediatric surgery international, 38(7), 1019–1030. https://doi.org/10.1007/s00383-022-05115-0

Orłowska, E., Czubkowski, P., Wołochowska, K., Jarzębicka, D., Motyl, I., & Socha, P. (2021). Assessment of Lactobacillus casei rhamnosus (LGG) therapy in children with biliary atresia - Randomized placebo controlled trial. Clinics and research in hepatology and gastroenterology, 45(6), 101753. https://doi.org/10.1016/j.clinre.2021.101753

Page, M. J., McKenzie, J. E., Bossuyt, P. M., Boutron, I., Hoffmann, T. C., Mulrow, C. D., Shamseer, L., Tetzlaff, J. M., Akl, E. A., Brennan, S. E., Chou, R., Glanville, J., Grimshaw, J. M., Hróbjartsson, A., Lalu, M. M., Li, T., Loder, E. W., Mayo-Wilson, E., McDonald, S., McGuinness, L. A., … Moher, D. (2022). A declaração PRISMA 2020: diretriz atualizada para relatar revisões sistemáticas. Revista panamericana de salud publica, 46, e112. https://doi.org/10.26633/RPSP.2022.112

Pietrobattista, A., Mosca, A., Liccardo, D., Alterio, T., Grimaldi, C., Basso, M., Saffioti, M. C., Corte, C. D., Spada, M., & Candusso, M. (2020). Does the Treatment After Kasai Procedure Influence Biliary Atresia Outcome and Native Liver Survival?. Journal of pediatric gastroenterology and nutrition, 71(4), 446–451. https://doi.org/10.1097/MPG.0000000000002837

Quelhas, P., Jacinto, J., Cerski, C., Oliveira, R., Oliveira, J., Carvalho, E., & Dos Santos, J. (2022). Protocols of Investigation of Neonatal Cholestasis-A Critical Appraisal. Healthcare (Basel, Switzerland), 10(10), https://doi.org/10.3390/healthcare10102012

Rabbani, T., Guthery, S. L., Himes, R., Shneider, B. L., & Harpavat, S. (2021). Newborn Screening for Biliary Atresia: a Review of Current Methods. Current gastroenterology reports, 23(12), 28. https://doi.org/10.1007/s11894-021-00825-2

Solanki, S., Kanojia, R. P., Gupta, P. K., Singhai, P., Lal, S. B., & Mahajan, J. K. (2023). A Randomized Control Trial to Evaluate the Effect of Local Instillation of Mitomycin-C at the Porta after Kasai Portoenterostomy in Patients of Biliary Atresia. Journal of Indian Association of Pediatric Surgeons, 28(4), 307–313.

Shirley M. (2022). Maralixibat: First Approval. Drugs, 82(1), 71–76. https://doi.org/10.1007/s40265-021-01649-0

Sutton, H., Karpen, S. J., & Kamath, B. M. (2024). Pediatric Cholestatic Diseases: Common and Unique Pathogenic Mechanisms. Annual review of pathology, 19, 319–344. https://doi.org/10.1146/annurev-pathmechdis-031521-025623

Tarro, T. M., Song, L., Carter, B. A., Yeh, M., Pannaraj, P. S., & Mohandas, S. (2020). 579. Prophylactic Antibiotics Did Not Decrease Recurrent Cholangitis in Patients with Biliary Atresia After Kasai Portoenterostomy. Open Forum Infectious Diseases, 7(Suppl 1), S354–S355. https://doi.org/10.1093/ofid/ofaa439.773

Wu, L. N., Zhu, Z. J., & Sun, L. Y. (2022). Genetic Factors and Their Role in the Pathogenesis of Biliary Atresia. Frontiers in pediatrics, 10, 912154. https://doi.org/10.3389/fped.2022.912154

Wang, P., Zhang, H. Y., Yang, J., Zhu, T., Wu, X., Yi, B., Sun, X., Wang, B., Wang, T., Tang, W., Xie, H., Tou, J., Han, Y., Liu, X., Zhan, J., Liu, Y., Li, Y., Lv, Z., Lu, L., Zhao, B., & Feng, J. (2023). Severity assessment to guide empiric antibiotic therapy for cholangitis in children after Kasai portoenterostomy: a multicenter prospective randomized control trial in China. International journal of surgery (London, England), 109(12), 4009–4017. https://doi.org/10.1097/JS9.0000000000000682

Publicado

17/05/2024

Cómo citar

FERREIRA, R. D. .; RESENDE, L. R. . Colestasis neonatal: Un abordaje desafiante para los pediatras ante los principales diagnósticos diferenciales y tratamientos. Research, Society and Development, [S. l.], v. 13, n. 5, p. e6213545796, 2024. DOI: 10.33448/rsd-v13i5.45796. Disponível em: https://rsdjournal.org/index.php/rsd/article/view/45796. Acesso em: 30 jun. 2024.

Número

Sección

Revisiones