Successful pericardiectomy in Hennekam syndrome

Objective & Hypothesis:Hennekam syndrome is an autosomal recessive disorder, characterized by lymphangiectasia in internal organs with lymphedema.

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Key Findings & Data:Although a poor long-chain fatty diet is given for treatment, its success rate is low.

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Conclusion & Impact:In this article, we report the results of a successful pericardiectomy performed on a 21-month-old boy who was diagnosed with Hennekam syndrome.

🔬Design:Research Article

📍Scope:Comprehensive Sample

⚙️Variables:Domain Parameters

Abstract

Hennekam syndrome is an autosomal recessive disorder, characterized by lymphangiectasia in internal organs with lymphedema. Pleural or pericardial effusions may present with lymphedema on extremities in the course of the disease. Although a poor long-chain fatty diet is given for treatment, its success rate is low. In this article, we report the results of a successful pericardiectomy performed on a 21-month-old boy who was diagnosed with Hennekam syndrome.

Özet

Hennekam sendromu, lenfödem ile birlikte iç organlarda lenfanjiyektazinin görüldüğü otozomal resesif geçişlibir hastalıktır. Hastalığın seyri sırasında ekstremite- lerdeki lenfödeme eşlik eden plevral veya perikardiyal efüzyonlar görülebilmektedir. Tedavide uzun zincirliyağdan fakir diyet uygulansa da, başarı oranı düşüktür.Bu yazıda, Hennekam sendromu tanısı konulan 21 aylıkerkek olguda başarı ile uygulanan perikardiyektominin sonucu sunuldu.

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Cite This Article

SARIGÜL, A., ALP, H., BAYSAL, T., KARATAŞ, Z., KARAARSLAN, S. (2022). Successful pericardiectomy in Hennekam syndrome. Türk Göğüs Kalp Damar Cerrahisi Dergisi, Cilt: 21 - Sayı: 4, 1110–1113. https://ajindex.com/articles/424527

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