Hepatoblastoma and prune belly syndrome: a potential association

被引：8

作者：

Becknell, Brian ^{[1
,7
]}

Pais, Priya ^{[2
,7
]}

Onimoe, Grace ^{[1
,3
,7
]}

Rangarajan, Hemalatha ^{[4
,7
]}

Schwaderer, Andrew L. ^{[1
,5
,7
]}

McHugh, Kirk ^{[6
,7
]}

Ranalli, Mark A. ^{[1
,3
,7
]}

Hains, David S. ^{[1
,5
,8
]}

机构：

[1] Ohio State Univ, Dept Pediat, Coll Med, Columbus, OH 43205 USA

[2] Childrens Hosp Wisconsin, Div Nephrol, Med Coll Wisconsin, Milwaukee, WI 53201 USA

[3] Nationwide Childrens Hosp, Div Hematol & Oncol, Columbus, OH 43205 USA

[4] Childrens Hosp Wisconsin, Div Hematol & Oncol, Med Coll Wisconsin, Milwaukee, WI 53201 USA

[5] Nationwide Childrens Hosp, Div Nephrol, Columbus, OH 43205 USA

[6] Nationwide Childrens Hosp, Res Inst, Ctr Mol & Human Genet, Columbus, OH 43205 USA

[7] Ctr Clin & Translat Res, Columbus, OH 43205 USA

[8] Nationwide Childrens Hosp, Res Inst, Ctr Clin & Translat Res, Columbus, OH 43205 USA

来源：

PEDIATRIC NEPHROLOGY | 2011年 / 26卷 / 08期

关键词：

Prune belly syndrome; Hepatoblastoma; Malignancy; BECKWITH-WIEDEMANN-SYNDROME; BIRTH CHARACTERISTICS; RISK-FACTORS; CHILDREN; CANCER; DISEASE;

D O I：

10.1007/s00467-011-1874-1

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

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.

引用

页码：1269 / 1273

页数：5

共 27 条

[1] Hypoplastic Glomerulocystic Kidney Disease and Hepatoblastoma A Potential Association not Caused by Mutations in Hepatocyte Nuclear Factor 1β [J].