Loss of imprinting at insulin-like growth factor II (IGFII), in association with H19 silencing, has been described previously in a subgroup of Beckwith-Wiedemann syndrome (BWS) patients who have an elevated risk for Wilms' tumor. An equivalent somatic mutation occurs in sporadic Wilms' tumor. We describe a family with overgrowth in three generations and Wilms' tumor in two generations, with pat...

how to cite this article: talebian a, goudarzi rm, mohammadzadeh m , mirzadeh as. vincristine-induced cranial neuropathy. iran j child neurol. 2014 winter; 8(1):66-68.   abstract vincristine (vcr) is a vinca alkaloid that is used for treatment of many malignancies. the vinca alkaloids are neurotoxic, usually causing a peripheral neuropathy, but cranial neuropathies are rare as side effects.  de...

epidermal nevus syndrome is a rare congenital disorder, characterized by epidermal nevi and multiple organ involvement. multicystic kidney disease has been very rarely reported in this syndrome. here is the report of a boy presented with multiple epidermal nevi, cardiac anomaly, seizure attack, hemi hypertrophy, and multicystic dysplastic kidney complicated with wilms' tumor. according to this ...

Book details Willemijn Wilms Floet URBAN OASES Dutch Hofjes as Hidden Architectural Gems . nai010 publishers, 2021. ISBN 978-94-6208-660-9. € 39.95, paperback (Also available in and e-book English Dutch). pp. 208, with illustrations (220 full color).

The case of a child with nephroblastoma, bilateral radial aplasia, and a de novo (1;7) translocation is reported. The association between abnormal karyotypes, dysmorphic syndromes, and childhood malignancy is well known.' We report the presence of a nephroblastoma in a boy with bilateral radial aplasia and a balanced de novo (1;7) translocation. Case report The patient was the first child of he...

at an early age. Furthermore, a genetic predisposition to develop the tumour is associated with aniridia, genitourinary abnormalities and mental retardation (the WAGR syndrome) (1). Children with this rare syndrome typically carry a germline deletion involving band p13 on one of the two (parentally-derived) chromosome 11 homologues (2). The inherited 11 p deletion in WAGR and hereditary Wilms' ...

Wilms' tumor (WT) accounts for 90% of all pediatric renal malignant tumors. The most common postoperative complication based on the National Wilms' Tumor Study is small bowel obstruction. We report on a 2-year-old girl with postoperative bowel obstruction following a right nephrectomy for WT. The patient was reintervened 48 hours after surgery and a cecal volvulus was found. Here, we will descr...

Introduction: Wilms’ tumor or nephroblastoma is an embryonal that develops from the remaining immature kidney and fourth most common primary renal malignancy in children. An asymptomatic abdominal mass present more than 90% hematuria 30% of patients. Preoperative workup includes complete laboratory blood tests imaging to ensure intrarenal mass. This study aimed describe anesthetic management pa...

Wilms' tumor (WT) is associated with loss of heterozygosity at chromosome 11p13, the site of the Wilms' tumor suppressor gene, WT1. Although the preferential loss of maternal alleles suggested that differential allelic expression of WT1 might occur, this has not been evident in normal fetal tissues or WTs. In this study, we show that the WT1 antisense regulatory region is differentially methyla...

Extrarenal Wilms' tumor of the ovary is a very rare tumor likely derived from embryonic mesonephros. We present the first reported case of a teratoid extrarenal Wilms' tumor of the ovary with a short review of the existing literature. In the case, a 26-year-old woman presented with back pain and was found to have a dermoid cyst; three years later, she presented again, now pregnant, with severe ...