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ASEMPTOMATİK BİR VVİLSON HASTASININ KARACİĞERİNİN ELEKTRON MİKROSKOPİK ÖZELLİKLERİ

Electron Microscopic Findings Patient's Liver Of An Asymptomatic Wilson's Disease

Journal Name:

  • İnönü Üniversitesi Tıp Fakültesi Dergisi

Publication Year:

  • 2000

Key Words:

Keywords (Original Language):

Author NameUniversity of AuthorFaculty of Author
Abstract (2. Language): 
Wilson's disease is a disorder of copper metabolism that is transmitted as an autosomal recessive trait. Light microscopic findings in symptomatic Wilson's disease may be normal. Electron microscopy may show characteristic changes in the hepatocytes. In the present study the liver of a patient with asymptomatic Wilson's disease was investigated. Many lipid droplets varying in size were observed within the hepatocyte cytoplasm. Nucleus and cytoplasm were rich in glycogen. Mitochondria showed marked pleomorphism. Cristae of some of the mitochondria were increased and dilatated, intercristal spaces were narrowed. Cristae of some of the mitochondria were decreased and thin, intercristal spaces were enlarged. Cristae were absent in some of the mitochondria. Tubules of agranular endoplasmic reticulum were sometimes enlarged. Many lysosomes and lipofuscin pigment were observed within the cytoplasm. Many lysosomes were also observed within the cytoplasm of Kupffer's cells. The mitochondria and lysosomes displayed the most prominent changes. It is suggested that electron microscopy may be helpful in early diagnosis of Wilson's disease even in the asyptomatic stage of the disease.
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Abstract (Original Language): 
Wilson hastalığı, otozomal resesif kalıtımsal geçişli bir bakır metabolizması hastalığıdır. Semptomatik Wilson hastalığında bile ışık mikroskopik bulgular tanı için spesifik olmayabilir. Elektron mikroskopik olarak hepatositlerde spesifik bulgular saptanabilir. Çalışmamızda asemptomatik bir Wilson hastasının karaciğeri elektron mikroskopik düzeyde incelendi Hepatosit sitoplazmasında irili ufaklı çok sayıda lipid damlaları izlendi Sitoplazma ve nükleoplazma glikojenden zengindi Mitokondrilerde belirgin pleomorfizm saptandı. Bazı mitokondrilerde kristalar çok sayıda, kalın ve yoğun, interkristal mesafeler dardı. Bazılarında ise kristalar seyrek ve ince, interkristal mesafeler genişti Bazı mitokondrilerde krista yapısının tamamen ortadan kalktığı gözlendi. Agranüler endoplazma retikulumu tubullerinde yer yer genişlemeler saptandı. Sitoplazmada çok miktarda lizozomlar ve lipofusin granülleri izlendi Kupffer hücrelerinde de çok miktarda lizozoma rastlandı. Wilson hastalığında hepatosit sitoplazmasında en belirgin değişikliklerin mitokondrilerde ve lizozomlarda ortaya çıktığı gözlendi Elektron mikroskopik bulguların semptomların ortaya çıkmasından önce saptanabileceği düşünülerek elektron mikroskopik incelemenin hastalığın erken tanısında önemli rolü olabileceği kanısına varıldı.
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  • 3
276-280
  • Turkish

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