n. congenital absence of the iris (of the eye). This may be a hereditary condition, associated with macular dysplasia, sensory nystagmus, and congenital cataract. See also WAGR syndrome.
(Wilms’ tumour) n. a malignant tumour arising from the embryonic kidney and occurring in young children, usually below the age of three and rarely over the age of eight. In approximately 5% of cases it involves both kidneys. Treatment consists of removing the kidney (see nephrectomy) and giving chemotherapy and sometimes radiotherapy. Although almost half the cases have spread by the time diagnosis is made, this does not prevent a cure: the number of children that survive at least five years after diagnosis is improving, being currently around 75%. In some children the tumour is associated with an abnormality of chromosome number 13; in these cases other features, such as absence of the iris in the eye (see aniridia) and *hemihypertrophy, are present. In other cases there is an association with congenital nephropathy and intersex disorders (see Denys-Drash syndrome).... nephroblastoma
Wilms’ tumour (see nephroblastoma), aniridia, genitourinary abnormalities, and mental retardation: a condition due to a deletion of part of the short arm of chromosome 11.... wagr syndrome
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