Mustafa BASAK, Deniz AVCI, Samet KARAHAN, Mehmet ERDOGMUS, Kadir BULUT, Ali ÇETİNKAYA

Wilson disease with situs inversus totalis

Situs inversus abdominal organların rotasyonuyla giden pozisyonel bir anomali olup torasik organ transpozisyonu da eklendiğinde hastalık situs inversus totalis olarak adlandırılmaktadır. Wilson hastalığı (hepatolentiküler dejenerasyon) hepatik ve nöropsiki- atrik semptomlara neden olan otozomal resesif kalıtılan bakır metabolizması hastalığıdır. Bu olgu sunumunda Wilson hastalığı ile situs inversus totalis birlikteliği gösteren ve literatüre göre ilk vaka olan bir olgu tanımladık.

Wilson hastalığı ile situs inversus totalis birlikteliği

Situs inversus is a positional anomaly that rotates the abdominal internal visceria and when it is associated with transposition of the thoracic organs it is called situs inversus totalis. Wilson disease (or hepatolenticular degeneration) is an autosomal recessive hereditary disease of human copper metabolism, which causes hepatic and neuropsychiatric symptoms. We describe a case report of situs inversus totalis with Wilson disease, which is the first case to our knowledge.

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