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Canadian Journal of Cardiology

Successful Total Pericardiectomy for Constrictive Pericarditis in the First Series of Japanese Patients With Mulibrey Nanism

Published:February 13, 2018DOI:https://doi.org/10.1016/j.cjca.2018.02.008

      Abstract

      Patients with Mulibrey nanism (MUL) present with growth failure and multiple organ manifestations, and MUL is caused by mutations in TRIM37. In this article, we report on the first case series of Japanese patients with MUL who developed congestive heart failure due to constrictive pericarditis. Our case series suggests that early diagnosis and total pericardiectomy before adherence of the pericardium might provide clinical benefit and better prognosis for MUL.

      Résumé

      Le nanisme Mulibrey se caractérise par un retard de croissance et des manifestations multiorganiques. Il est attribuable à des mutations du gène TRIM37. Dans la présente communication, nous faisons état de la première série de cas de patients japonais atteints de nanisme Mulibrey chez qui une insuffisance cardiaque congestive s’est constituée par suite d’une péricardite constrictive. Notre série de cas donne à penser qu’un diagnostic précoce et une péricardectomie totale pratiquée avant l’adhésion des feuillets du péricarde peuvent apporter un bénéfice clinique et se traduire par un meilleur pronostic dans les cas de nanisme Mulibrey.
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