Glossary - W

Wilms' tumor

Wilms' tumor is an embryonal renal neoplasm containing blastema, stromal or epithelial cell types usually affecting children before the fifth year. Several congenital anomalies are known to be associated with this disease. A two stage mutational model has been proposed: occurrence in either hereditary or sporadic form. Patients with aniridia have a deletion of the short arm of chromosome 11. It affects the Renal/urological system. 0.69 per 100,000 people in the United States, and 8/100,000 children under the age of 15. The median age of incidence is 36.5 months. Females have greater susceptibility and the disease is usually asymptomatic. Symptoms include, palpable upper abdominal mass, abdominal pain, fever, anemia, signs of acute abdomen with free intraperitoneal rupture (very rare), cardiac murmur, hepatosplenomegaly, ascites, prominent abdominal wall veins and/or varicocele and gonadal metastases. Causes include hereditary or sporadic forms of genetic mutation, in its familial form: autosomal dominant trait with incomplete penetrance (1 %) and/or potential of paternal occupational exposure (eg. autobody repairman). Those at risk may have aniridia, hemihypertrophy, hypospadias, duplicated renal collecting systems. Wiedmann-Beckwith, syndrome, Drash's syndrome, Klippel-Trenaunay syndrome and familial occurrence.

Wilson's disease

Wilson's disease is an inherited metabolic disorder characterized by excessive amounts of copper in the liver, brain, kidneys and corneas. It can lead to tissue necrosis and fibrosis, which in turn can cause hepatic disease and neurologic changes. Without treatment, it leads to fatal hepatic failure. Its usual course is chronic.

 

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