Dergiler / Üroonkoloji Bülteni / 2018 / Cilt: 17 - Sayı: 2

Wunderlich Syndrome, Tuberous Sclerosis-Related Giant Renal Angiomyolipoma Rupture: Case Report

Wunderlich Sendromu, Tüberoskleroz ile İlişkili Dev Renal Anjiyomiyolipom Rüptürü: Olgu Sunumu

Sayfa
76–78
DOI
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⚡ TL;DR(1 Cümlede Hızlı Çıkarım)

He was discharged from the intensive care unit on postoperative day 1 and from the hospital on day 5.

📌 3 Maddede Bu Makale

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Amaç & Hipotez:Angiomyolipoma (AML) is a mesenchymal tumor of the kidney that is composed of morphologically abnormal smooth muscle cells, blood vessels, and adipose-like foci.
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Temel Bulgular & Veri:A 39-year-old man with tuberous sclerosis was admitted to the emergency room with left flank pain, hematuria, and nausea.
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Sonuç & Katkı:He was discharged from the intensive care unit on postoperative day 1 and from the hospital on day 5.
🔬Tür:Araştırma Makalesi
📍Kapsam:Genişletilmiş Akademik Örneklem
⚙️Değişkenler:Alan Parametreleri

Özet

Angiomyolipoma (AML) is a mesenchymal tumor of the kidney that is composed of morphologically abnormal smooth muscle cells, blood vessels, and adipose-like foci. Renal AML is usually clinically asymptomatic and detected incidentally during imaging. Rarely, renal AML can cause life-threatening spontaneous massive retroperitoneal hemorrhage, known as Wunderlich syndrome. A 39-year-old man with tuberous sclerosis was admitted to the emergency room with left flank pain, hematuria, and nausea. On physical examination, there was a hard sensitive mass extending from the upper left half of the abdomen to the midline. Left renal AML and extensive retroperitoneal hematoma measuring about 360x220x195 mm were detected on abdominal computed tomography. The patient exhibited signs of hypovolemic shock and emergency total nephrectomy was performed. He was discharged from the intensive care unit on postoperative day 1 and from the hospital on day 5.

Abstract

Anjiyomiyolipom (AML), morfolojik olarak anormal kan damarları, düz kas hücreleri ve yağ dokusu benzeri odaklardan oluşan böbreğin mezenkimal bir tümörüdür. Renal AML klinik olarak çoğunlukla asemptomatiktir ve insidental olarak görüntüleme yöntemleri sırasında saptanabilir. Renal AML nadiren “Wunderlich sendromu” olarak bilinir ve hayatı tehdit edebilen, retroperitoneal spontan masif kanamalara sebep olabilir. Tüberoskleroz hastalığı bulunan 39 yaşındaki erkek hasta; sol yan ağrısı, hematüri ve bulantı ile acil servise başvurdu. Fizik muayenede karın sol üst yarısından orta hatta uzanan sert hassas kitle mevcuttu. Çekilen abdominal bilgisayarlı tomografide 360x220x195 mm boyutlarında sol renal AML ve yaygın retroperitoneal hematom saptandı. Hipovolemik şok tablosunda olan hastaya acil radikal nefrektomi yapıldı. Hasta postoperatif 1. gün yoğun bakım ünitesinden, 5. gün hastaneden taburcu edildi.

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Bu Makaleye Atıf Yap

BARAN, Ö., AYKAÇ, A., ÖNER, S., AKTÜMEN, A., BÜRLUKKARA, S., SUNAY, M. M., ÖZOK, H. U. (2018). Wunderlich Syndrome, Tuberous Sclerosis-Related Giant Renal Angiomyolipoma Rupture: Case Report. Üroonkoloji Bülteni, Cilt: 17 - Sayı: 2, 76–78. https://ajindex.com/articles/334344

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Kaynakça (13)

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