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Hepatic Disease with Portal Hypertension and Acute Juvenile Paracoccidioidomycosis: A Report of Two Cases and Literature Review

Abstract

Paracoccidioidomycosis (PCM) is a neglected systemic mycosis endemic to Latin America caused by dimorphic fungi of the genus Paracoccidioides. The acute juvenile PCM is a severe type of presentation that usually affects young vulnerable patients and rarely progresses to portal hypertension. Here, two cases of liver disease and portal hypertension as complications of acute juvenile PCM are reported. Diagnosis of PCM was performed by isolation of the fungus and molecular identification of the strains provided through partial sequencing of two protein encoding genes, arf and gp43. Genotypic analysis revealed that Paracoccidioides brasiliensis S1 was the phylogenic species involved in both cases. Patients presented a good clinical response to amphotericin B and sulfamethoxazole–trimethoprim. These results highlight the importance of the interdisciplinary approach in patients with severe forms of PCM to avoid and treat complications, and the necessity of further investigations focusing on host-pathogen interaction in order to explain the broad clinical spectrum in PCM as well as the severity and poor outcome in some clinical cases.

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Acknowledgements

The authors thank the sequencing platform team at Oswaldo Cruz Foundation—PDTIS/FIOCRUZ for automated nucleotide sequencing of the strains and also the staff of Diagnostic Section of the Mycology Laboratory for the strain maintenance. The authors are also very grateful to Dr. Jose Mauro Peralta for kindly providing the serological evaluation for schistosomiasis.

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Correspondence to Priscila Marques de Macedo.

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The authors declare that they have no competing interests.

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The Research Ethics Committee of Evandro Chagas National Institute of Infectious Diseases (INI)/Fiocruz has approved this study protocol under the register CAAE 42590515.0.0000.5262.

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de Macedo, P.M., Almeida-Paes, R., Freitas, D.F.S. et al. Hepatic Disease with Portal Hypertension and Acute Juvenile Paracoccidioidomycosis: A Report of Two Cases and Literature Review. Mycopathologia 182, 915–919 (2017). https://doi.org/10.1007/s11046-017-0152-6

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  • DOI: https://doi.org/10.1007/s11046-017-0152-6

Keywords

  • Paracoccidioides brasiliensis S1
  • Acute juvenile paracoccidioidomycosis
  • Hepatic disease
  • Portal hypertension
  • Complications