Hipertansiyonu, Renal İnfarktı ve Morgagni Hernisi Olan Marfan Sendromlu Bir Olgu

Öz

Marfan sendromu 15. kromozomda mutasyonlar ile oluşan konnektif dokunun otozomal dominant kalıtım ile geçen bir hastalığıdır. Kardiyovasküler hastalıklar ve diafragmatik hernileri de içeren yumuşak doku herniasyonları Marfan sendromunda görülebilen durumlardır. Olgu: 22 yaşında erkek hasta, hipertansiyonun klinik bulguları ile nefroloji polikliniğine başvurdu. Hastanın değerlendirmesinde Morgagni diyafragmatik herni ve renal infarkt tespit edildi. Hastanın yapılan muayenesinde Marfan sendromunu düşündüren fiziksel görünümüne sahipti. Radyolojik değerlendirmeler sağ konjenital diyafragmatik Morgagni hernisi ve renal infarktı ortaya çıkardı. Sonuç: Marfan sendromunda akselere hipertansiyon varlığında renal infarkt akla gelmelidir. Ayrıca hastalarda diyafragma yüksekliği varsa Morgagni hernisi olabileceği unutulmamalıdır. Anahtar kelimeler: Marfan Sendromu, Renal İnfarkt, Morgagni Hernisi

Anahtar Kelimeler

• Marfan Sendromu, Renal İnfarkt, Morgagni Hernisi

A Case of Marfan Syndrome with Hypertansion, Renal Infarct and Morgagni Hernia

Öz

Backround: Marfan syndrome is an autosomal dominant disorder of connective tissue caused by mutations in the chromosome 15. Cardiovascular disorders and soft tissue hernias including diaphragmatic hernias may seen in Marfan syndrome Objective: A 22 years-old male patient admitted to our clinic with clinical features of hypertension and we found Morgagni diaphragmatic hernia and renal infarct at the evaluation. He had the clinical outlook of Marfan syndrome. Radiological investigations demonstrated a right congenital diaphragmatic Morgagni hernia and renal infarct. Conclusion: Because, Marfan syndrome is presented with several clinical manifestations which can be seen at different ages from neonatal age to elder ages; diaphragmatic hernia should remind Marfan syndrome and further clinical evaluations should be performed. We report a patient with Morgagni diaphragmatic hernia which may seen as a rare complication of Marfan syndrome and renal infarct without aort or renal dissection and aneurysm. Key words: Marfan Syndrome, Renal Infarct, Morgagni Hernia

Anahtar Kelimeler

• Marfan Syndrome, Renal Infarct, Morgagni Hernia

Kaynakça

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