Budd-Chiari Syndrome Associated with Porto-Mesenteric Venous Thrombosis: an Unusual Revealing Mode of Paroxystic Nocturnal Hemoglobinuria

Authors

  • S. Khedher Unité de soins intensifs, service de gastroentérologie, hôpital Charles-Nicolle
  • Y. Said Unité de soins intensifs, service de gastroentérologie, hôpital Charles-Nicolle
  • N. Foudhaili Unité de soins intensifs, service de gastroentérologie, hôpital Charles-Nicolle
  • K. Ben Ismail Unité de soins intensifs, service de gastroentérologie, hôpital Charles-Nicolle
  • M. Salem Unité de soins intensifs, service de gastroentérologie, hôpital Charles-Nicolle

DOI:

https://doi.org/10.3166/rea-2019-0108

Keywords:

Septic shock, Pediatrics, Resuscitation, Fluid therapy, Monocytes/pathology

Abstract

Budd-Chiari syndrome (BCS) is a vascular disease of the liver, mainly caused by hepatic venous thrombosis. Paroxysmal nocturnal hemoglobinuria (PNH), acquired clonal hematopoietic stem cell disorder, is one of the prothrombotic conditions predisposing to the development of primary BCS. BCS–PNH combination is rare, often reported as isolated clinical cases or small series, and has prognostic and therapeutic specificities. We report the case of a 27-year-old girl with PNH revealed as part of the etiological assessment of a BCS, associated with portal venous thrombosis, mesenteric thrombosis, and pulmonary embolism.

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Published

2019-11-01

How to Cite

Khedher, S., Said, Y., Foudhaili, N., Ben Ismail, K., & Salem, M. (2019). Budd-Chiari Syndrome Associated with Porto-Mesenteric Venous Thrombosis: an Unusual Revealing Mode of Paroxystic Nocturnal Hemoglobinuria. Médecine Intensive Réanimation, 28(6), 451–455. https://doi.org/10.3166/rea-2019-0108

Issue

Vol. 28 No. 6 (2019): Novembre-Décembre 2019 / Néphrologie et métabolisme

Section

Commented case report