Hepatoblastoma and prune belly syndrome: a potential association
- نوع فایل : کتاب
- زبان : انگلیسی
- مؤلف : Brian Becknell & Priya Pais & Grace Onimoe & Hemalatha Rangarajan & Andrew L. Schwaderer & Kirk McHugh & Mark A. Ranalli & David S. Hains
- چاپ و سال / کشور: 2011
Description
Prune belly syndrome (PBS) is a congenital anomaly characterized by the clinical triad of lax abdominal musculature, bilateral cryptorchidism, and abnormalities of the kidney and urinary tract. Previous reports of malignancy in patients with PBS have been limited to germ cell tumors. Hepatoblastoma (HBL) is the most common hepatic malignancy of childhood, affecting approximately 100 children each year in the USA. We describe a set of 4 pediatric patients with PBS and HBL. All individuals were born after 2002. These subjects lacked genetic, natal, or environmental factors known to confer risk of HBL. The occurrence of PBS and HBL in these patients constitutes a novel potential association.
Pediatr Nephrol (2011) 26:1269–1273 DOI 10.1007/s00467-011-1874-1 Received: 31 January 2011 / Revised: 28 February 2011 / Accepted: 3 March 2011 / Published online: 20 May 2011