Use of intrathecal drug infusion pump with combined morphine and bupivacaine medication for the treatment of abdominal pain due to Acute Intermittent Porphyria (AIP)

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Roger Wilson Gonçalves de Oliveira
Roberta Fernandes Souza
Rômulo Sarrazin de Andrade
Larissa de Oliveira Campelo
Fabiana Moreira Leite Dantas
Thalyta Almeida Lopes Soares
Luciano Barroso de Albuquerque Filho
Emanuel Inácio dos Santos
Enzo Lima Alcântara Parente
Rafael do Nascimento Muniz
André Alcântara Parente
Wirio Igor Morais Moura
Jéssica Maria Bezerra Braga
Franklin Reis
Júlio César Claudino dos Santos

Abstract

Porphyrias consist of nine genetic disorders, acquired or congenital, resulting from failures in the biosynthesis process of the heme group, resulting in the accumulation of metabolic intermediates in the human body, causing toxicity. We describe a case of a 32-years-old Woman presented with severe and continual abdominal pain, presenting a visual analog scale (VAS) score of 9-10, described by the patient as the worst pain she has ever felt in her life, colic type in the mesogastric area and that radiated throughout the entire abdomen for six months. Due to the presented condition, she underwent appendectomy and cholecystectomy procedures, being later diagnosed with acute intermittent porphyria (AIP). It is important to highlight that some manifestations resulting from AIP are similar to Acute Abdomen. This is a clinical condition characterized by sudden or progressive abdominal pain, which usually requires an emergency surgical procedure to resolve the condition. The diagnosis of AIP is quite complex, and it can easily cause misdiagnoses, as in the present case report, in which the patient was mistakenly submitted to appendectomy and cholecystectomy procedures, due to the symptom of severe abdominal pain, which could lead to exposure her to iatrogenic damage.

Article Details

How to Cite
de Oliveira, R. W. G., Souza, R. F. ., de Andrade, R. S. ., Campelo, L. de O. ., Dantas, F. M. L., Soares, T. A. L., de Albuquerque Filho, L. B., dos Santos, E. I., Parente, E. L. A. ., Muniz, R. do N. ., Parente, A. A. ., Moura, W. I. M. ., Braga, J. M. B. ., Reis, F., & dos Santos, J. C. C. . (2022). Use of intrathecal drug infusion pump with combined morphine and bupivacaine medication for the treatment of abdominal pain due to Acute Intermittent Porphyria (AIP). Brazilian Journal of Case Reports, 3(1), 4–9. https://doi.org/10.52600/2763-583X.bjcr.2023.3.1.4-9
Section
Clinical Case Reports
Author Biographies

Roger Wilson Gonçalves de Oliveira, Centro Universitário Fametro, Manaus, AM, Brazil.

Centro Universitário Fametro, Manaus, AM, Brazil.

Roberta Fernandes Souza, Centro Universitário Fametro

Centro Universitário Fametro, Manaus, AM, Brazil.

 

Rômulo Sarrazin de Andrade, Centro Universitário Fametro

Centro Universitário Fametro, Manaus, AM, Brazil.

Larissa de Oliveira Campelo, Centro Universitário Fametro

Centro Universitário Fametro, Manaus, AM , Brasil.

Fabiana Moreira Leite Dantas, Centro Universitário Fametro, Manaus

Centro Universitário Fametro, Manaus, AM , Brasil.

Thalyta Almeida Lopes Soares, Centro Universitário Fametro

Centro Universitário Fametro, Manaus, AM , Brasil.

Luciano Barroso de Albuquerque Filho, Faculdade de Medicina, Centro Universitário Christus, UNICHRISTUS

Faculdade de Medicina, Centro Universitário Christus, UNICHRISTUS, Fortaleza, CE, Brasil.

Emanuel Inácio dos Santos, Faculdade de Enfermagem, Centro Universitário Estácio de Sá

Faculdade de Enfermagem, Centro Universitário Estácio de Sá, Fortaleza, CE, Brasil.

Enzo Lima Alcântara Parente, Faculdade de Medicina, Centro Universitário Christus, UNICHRISTUS

Faculdade de Medicina, Centro Universitário Christus, UNICHRISTUS, Fortaleza, CE, Brasil.

Rafael do Nascimento Muniz, Centro Universitário Augusto Motta, UNISUAM

Centro Universitário Augusto Motta, UNISUAM, Rio de Janeiro, RJ, Brazil.

André Alcântara Parente, Faculdade de Medicina, Centro Universitário Christus, UNICHRISTUS

Faculdade de Medicina, Centro Universitário Christus, UNICHRISTUS, Fortaleza, CE, Brazil.

Wirio Igor Morais Moura, Faculdade de Medicina, Centro Universitário Christus, UNICHRISTUS

Faculdade de Medicina, Centro Universitário Christus, UNICHRISTUS, Fortaleza, CE, Brazil.

Jéssica Maria Bezerra Braga, Faculdade de Medicina, Centro Universitário Christus, UNICHRISTUS

Faculdade de Medicina, Centro Universitário Christus, UNICHRISTUS, Fortaleza, CE, Brazil.

Franklin Reis, Centro Universitário Fametro

Centro Universitário Fametro, Manaus, AM , Brasil.

Júlio César Claudino dos Santos, Faculdade de Medicina, Centro Universitário Christus, UNICHRISTUS

Faculdade de Medicina, Centro Universitário Christus, UNICHRISTUS, Fortaleza, CE, Brasil.

Universidade Federal de São Paulo, São Paulo, SP, Brasil. 

Laboratório de Neurociências, Departamento de Neurologia e Neurocirurgia, Universidade Federal de São Paulo, São Paulo, SP, Brasil.

References

Wylie, K., Testai, F.D. Neurological Manifestations of Acute Porphyrias. Curr Neurol Neurosci Rep 22, 355–362 (2022). https://doi.org/10.1007/s11910-022-01205-7.

Granata, F.; Nicolli, A.; Colaiocco, A.; Di Pierro, E.; Graziadei, G. Psychological Aspect and Quality of Life in Porphyrias: A Review. Diagnostics 2022, 12, 1193. https://doi.org/10.3390/ diagnostics12051193.

Amador AP, Cordero AS, Risco PS. Acute intermittent porphyria: is oseltamivir safe in these patients? Clin Med (Lond). 2022 May;22(3):280-281. doi: 10.7861/clinmed.2022-0100.

Ortega AJ, Cherukuri S, Kalas MA, et al. A Perfect Storm: Abdominal Pain and Ileus Explained by Acute Intermittent Porphyria Caused by Prehospitalization and Intrahospitalization Factors. Journal of Investigative Medicine High Impact Case Reports. January 2022. doi:10.1177/23247096221109206.

Parreira Bizinoto, G., Gomes de Campos, F., Alexandre Lins Mourão, Y., & de Gregório Faria, M. (2020). Diagnóstico e tratamento da Porfiria Aguda: relato de caso. Health Residencies Journal - HRJ, 1(8), 20–25. https://doi.org/10.51723/hrj.v1i8.127.

Li P, Maitra D, Kuo N, Bonkovsky HL, Omary MB. Geographic prevalence variation and phenotype penetrance in porphyria: insights from a Chinese population database. Blood Adv. 2021 Jan 12;5(1):12-15. doi: 10.1182/bloodadvances.2020003150.

Gonzalez-Mosquera LF, Sonthalia S. Acute Intermittent Porphyria. [Updated 2022 May 8]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK547665/.

Spiritos Z, Salvador S, Mosquera D, Wilder J. Acute Intermittent Porphyria: Current Perspectives And Case Presentation. Ther Clin Risk Manag. 2019 Dec 16;15:1443-1451. doi: 10.2147/TCRM.S180161.

Tinoco J, Eloy A, Regufe R, Rosinha D, Taleco T, Silva-Duarte J. Use of sugammadex in acute intermittent porphyria. Rev Mex Anestesiol. 2021; 44 (3): 229-232. https://dx.doi.org/10.35366/99671.

Spiritos Z, Salvador S, Mosquera D, Wilder J. Acute Intermittent Porphyria: Current Perspectives And Case Presentation. Ther Clin Risk Manag. 2019 Dec 16;15:1443-1451. doi: 10.2147/TCRM.S180161.

Tinoco J, Eloy A, Regufe R, Rosinha D, Taleco T, Silva-Duarte J. Use of sugammadex in acute intermittent porphyria. Rev Mex Anestesiol. 2021; 44 (3): 229-232. https://dx.doi.org/10.35366/99671.

Indika, N.L.R., Kesavan, T., Dilanthi, H.W. et al. Many pitfalls in diagnosis of acute intermittent porphyria: a case report. BMC Res Notes 11, 552 (2018). https://doi.org/10.1186/s13104-018-3615-z.

Ramanujam VS, Anderson KE. Porphyria Diagnostics-Part 1: A Brief Overview of the Porphyrias. Curr Protoc Hum Genet. 2015 Jul 1;86:17.20.1-17.20.26. doi: 10.1002/0471142905.hg1720s86.

Prisi S, Banerjee P, Mishra TK. Acute Intermittent Porphyria (AIP): A Difficult Diagnosis. MAMC J Med Sci 2021;7:86-9.

A Li, Wood’s lamp urinary examination in acute intermittent porphyria, QJM: An International Journal of Medicine, 2022;, hcac100, https://doi.org/10.1093/qjmed/hcac100.

Sharma AG, Pandit K, Gupta S, Kumar V. Acute Intermittent Porphyria in Prepubertal Child-diagnostic and Therapeutic Challenges in India: A Case Report and Literature Review. Indian J Crit Care Med. 2022;26(3):390-394. doi:10.5005/jp-journals-10071-24133.

Zhao, L., Wang, X., Zhang, X., Liu, X., Ma, N., Zhang, Y., et al. (2020). Therapeutic strategies for acute intermittent porphyria. Intractable Rare Dis. Res. 9, 205–216. doi: 10.5582/irdr.2020.03089.

Bonkowsky HL, Tschudy DP, Collins A, Doherty J, Bossenmaier I, Cardinal R, Watson CJ. Acute hepatic porphyrias: Rec-ommendations for evaluation and longterm management. Hepatology. 2017; 66:1314-1322.

Wang, B., Rudnick, S., Cengia, B. and Bonkovsky, H.L. (2019), Acute Hepatic Porphyrias: Review and Recent Progress. Hepatol Commun, 3: 193-206. https://doi.org/10.1002/hep4.1297.

Crooke ST, Witztum JL, Bennett CF, Baker BF. RNA-Targeted Therapeutics. Cell Metab. 2018 Apr 3;27(4):714-739. doi: 10.1016/j.cmet.2018.03.004.

Shi, Y.; Lu, A.; Wang, X.; Belhadj, Z.; Wang, J.; Zhang, Q. Acta Pharmaceutica Sinica B 2021, 11 (8), 2396–2415.

Brian M. Ilfeld, James C. Eisenach, Rodney A. Gabriel; Clinical Effectiveness of Liposomal Bupivacaine Administered by Infiltration or Peripheral Nerve Block to Treat Postoperative Pain: A Narrative Review. Anesthesiology 2021; 134:283–344 doi: https://doi.org/10.1097/ALN.0000000000003630.

Ha HK, Lee KG, Choi KK, Kim WS, Cho HR. Effect of bupivacaine on postoperative pain and analgesics use after sin-gle-incision laparoscopic appendectomy: double-blind randomized study. Ann Surg Treat Res. 2020 Feb;98(2):96-101. doi: 10.4174/astr.2020.98.2.96. Epub 2020 Jan 31.

Moradkhani MR, Karimi A. Effect of Artemisia Aucheri.L and Bupivacaine Encapsulated Nanoparticles on Nociceptive Pain. Drug Res (Stuttg). 2019 Jul;69(7):401-405. doi: 10.1055/a-0825-6487. Epub 2019 Jan 7. PMID: 30616248.

Aoki Y, Atsumi K, Kora M, Koh N, Yokoyama J. Sugammadex and amino acid infusion can contribute to safe anesthetic management of variegate porphyria. JA Clin Rep. 2018 Jun 18;4(1):49. doi: 10.1186/s40981-018-0187-9.

Tinoco, Joana et al. Use of sugammadex in acute intermittent porphyria. Rev. mex. anestesiol., Ciudad de México, v. 44, n. 3, p. 229-232, sept. 2021.

Shim JW, Cho YJ, Moon HW, Park J, Lee HM, Kim YS, Moon YE, Hong SH, Chae MS. Analgesic efficacy of intrathecal morphine and bupivacaine during the early postoperative period in patients who underwent robotic-assisted laparoscopic prostatec-tomy: a prospective randomized controlled study. BMC Urol. 2021 Feb 26;21(1):30. doi: 10.1186/s12894-021-00798-4.

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