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Renal Agenîzisi Olan Bir Beckwith-Wiedemann Olgusu

Beckwith-Wiedemann Syndrome With Renal Agenesis

Journal Name:

  • Türk Nefroloji, Diyaliz ve Transplantasyon Dergisi

Publication Year:

  • 2007

Key Words:

Keywords (Original Language):

Author NameUniversity of AuthorFaculty of Author
Abstract (2. Language): 
Beckwith-Wiedemann syndrome is a congenital disorder characterized by somatic overgrowth, macroglossia, anterior abdominai wall defects and macrosomia. Additional features include ear lobe creases or pits, facial nevus flammeus, hemihyperplasia, neonatal hypoglicemia, organomegaly and renal abnormalities. The most common renal abnormalities are nephromegaly, simple cysts, hydronephrosis, and medullary cysts.Other less common findings include nephrocalcinosis, increased cortical and pyramidal echogenicity, 3 double collecting system, and caliceal diverticula. Renal agenesis has not been reported before. Our case is first BWS with renal agenesis.
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Abstract (Original Language): 
Beckwith-Wiedemann sendromu (BWS); somatik aşırı büyüme, makroglossi, ön karın duvarı defekti ve makrosomi ile karakter e bir konjenital anamalıdır. Bu olgularda kulak Sobu anomalileri, yüzde nevus flammeus, hemihipertrofi, neonatal hipoglisemi, organomegaly renal anomaliler görülebilir. En sık renal anomaliler nefromegali, basit kistler, hidronefroz, medüller kistlerdir, daha az •randa da nefrokalsinozis, artmış kortikal ve piramidal ekojenite, çift toplayıcı sistem ve kalisiel divertikül olarak belirlenmiştir. Renal agenezi daha önce BWS'de bildirilmemiştir. Olgumuz renal agenezi saplanan ilk BWS olması sebebiyle sunulmuştur.
FULL TEXT (PDF): 
PDF icon arastrmx_172977_16_pp_196-197.pdf
  • 4
196-197
  • Turkish

REFERENCES

References: 

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