The phenotypic and pathological features of prune-belly syndrome

ŞAHİN D., Çetiner H., GÜVEN Ş.

TURKISH JOURNAL OF PATHOLOGY, cilt.26, sa.2, ss.114-119, 2010 (SCI-Expanded) identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 26 Sayı: 2
  • Basım Tarihi: 2010
  • Doi Numarası: 10.5146/tjpath.2010.01007
  • Dergi Adı: TURKISH JOURNAL OF PATHOLOGY
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, TR DİZİN (ULAKBİM)
  • Sayfa Sayıları: ss.114-119
  • Anahtar Kelimeler: Prune-belly syndrome, Uretral obstruction, Urinary tract anomalies, Fetus, Fetal pathology
  • Acıbadem Mehmet Ali Aydınlar Üniversitesi Adresli: Hayır

Özet

Objective: Prune-belly syndrome is a rare congenital disorder characterized by musculature deficiency in the abdominal wall, lower urinary tract obstruction, other urinary tract anomalies, and bilateral cryptorchidism. The syndrome is commonly associated with pulmonary, skeletal, cardiac, and gastrointestinal defects. Over 95% of patients are male. Urinary tract disease is the major prognostic factor with the complications of pulmonary hypoplasia and end stage renal disease. The aim of this study was to determine phenotypic and pathologicalfeatures of fetuses with this syndrome.