© The Canadian Journal of UrologyTM; 21(5); October 2014 Address correspondence to Dr. Lane S. Palmer, Pediatric Urology, Cohen Children’s Medical Center of New York, 269-01 76th Avenue, New Hyde Park, NY 11040 USA Voiding cystourethrogram in multicystic dysplastic kidney: the pendulum has swung Ariella A. Friedman, MD, Lane S. Palmer, MD Cohen Children’s Medical Center, North Shore-Long Island...

Wolcott-Rallison syndrome (WRS) is a rare autosomal recessive disorder due to mutations in the EIF2AK3 gene. It is characterized by permanent neonatal diabetes mellitus, skeletal dysplasia, liver impairment, neutropenia and renal dysfunction. Liver is the most commonly affected organ and liver failure is the commonest cause of death in this syndrome. The EIF2AK3 gene encodes a transmembrane pro...

The aim of the study was to summarize the preliminary experience of minimally invasive open nephrectomy operation on children with multicystic dysplastic kidney (MCDK). A retrospective review was performed on the clinical materials of the 15 children that had accepted consecutive minimally invasive open nephrectomies during the previous 2 years. The enrolled children were diagnosed with unilate...

We analyzed 17 cases of multicystic dysplastic kidney (MCDK) to document the natural history of MCDK and its management. One patient was nephrectomied for respiratory failure associated with MCDK. Follow-up studies of 14 kidneys revealed that 5 kidneys (36%) did not change in size, 7 kidneys (50%) decreased in size. Two kidneys (14%) increased in size during the follow up periods and were nephr...

INTRODUCTION Wnt-1 is capable of inducing metanephric mesenchyme to undergo tubulogenesis. A relationship between the degree of cystogenesis and reduced E-cadherin (E-cad) expression was described. Syndecan-1 (Sdc-1) has a critical role in kidney development. MATERIALS AND METHODS Ten multicystic dysplastic kidneys (MCDKs) were stained with hematoxylin and eosin and immunohistochemistry was p...

Paramesonephric duct remnants are an infrequent cause of abdominal symptoms in childhood. Preoperative diagnosis is often difficult and diagnosis is usually made at surgery. We report a rare presentation of an acute abdomen in a child with bilateral inflamed fimbrial cysts. Ultrasound revealed the presence of a multicystic lesion behind bladder. It was only at laparotomy the diagnosis of bilate...

Multicystic dysplastic kidney (MCDK) is a relatively common developmental anomaly in infants and children and has a good prognosis. In contrast, a malignant rhabdoid tumor of the kidney (MRTK) is one of the most lethal neoplasms of early life. However, the presentation of such a lethal tumor combined with multicystic dysplasia has not been reported to date. In this report, we describe a case of...