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Tuberous Sclerosis’de Böbrek Tutulumu: Klinik ve Radyolojik Bulgular

Renal İnvolvement of Tuberous Sclerosis: Clinical and Radiologic Findings

Journal Name:

  • Uludağ Üniversitesi Tıp Fakültesi Dergisi

Publication Year:

  • 2003

Keywords (Original Language):

Author NameUniversity of Author
Abstract (2. Language): 
Tuberous Sclerosis (TS) is an autosomal dominantly inherited disorder characterized by the presence of hamartomas in multiple organ systems. The classic clinical triad is epilepsy, mental retardation and skin lesions named as adenoma sebaceum. The most common lesion in kidney is angiomyolipoma in TS. It is seen in 40 to 80 % of patients. These lesions are tend to grow and may led to severe haemorhage. In our study, 14 patients with tuberous sclerosis diagnosed by clinical findings and radiologic examinations (cranial CT and MR) were investigated for renal involvement of tuberous sclerosis. History, complaints, physical examinations, laboratory examinations and renal US and BT examinations of patients were evaluated retrospectively. Multiple renal angiomyolipomas were detected in eight of fourteen patients by US and CT. Two of these patients had findings of renal insufficiency. These two are older patients and their AMLs were larger. In tuberous sclerosis, angiomyolipomas are common renal lesions and are tend to increase with age. They may lead to renal insufficiency. But it is not yet known why and how AML grows. Further research is needed in this regard.
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Abstract (Original Language): 
Tuberous sclerosis (TS) otozomal dominant geçişli, birçok organ sisteminde hamartomlarla karakterize bir hastalıktır. Klasik klinik triadı epilepsi, mental retardasyon ve adenoma sebaseum olarak adlandırılan deri lezyonlarıdır. Böbreklerde en sık görülen lezyon angiomyolipomdur (AML). AML hastaların % 40-80’inde saptanır. Bu lezyonlar büyümeye eğilimlidirler ve ciddi hemorajiye yol açabilirler. Çalışmamızda klinik bulgular ve radyolojik tetkiklerle (kranial BT-MR) TS tanısı alan 14 hasta böbrek tutulumu yönünden incelendi. Olguların yakınmaları, fizik muayeneleri, laboratuar bulguları, renal US ve BT bulguları retrospektif olarak değerlendirildi. Ondört olgunun 8’inde (%57) US ve BT tetkikleri ile renal AML saptandı. Bunlardan ikisinde böbrek fonksiyonları belirgin şekilde bozulmuştu. Bu iki olgu da ileri yaşta ve büyük AML’lar bulunan olgulardı. TS da böbrekte sıklıkla izlenen AML’lar yaş arttıkça boyut ve sayı olarak artmaya eğilimlidir. Böbrek yetmezliğine neden olabilirler. Ancak AML’ların nasıl ve neden büyüdüğü hala bilinmemektedir. Bu konuda ileri araştırmalara gerek duyulmaktadır.
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  • 1
7-10
  • Turkish

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