In which of the following conditions, Aniridia and Hemihyperophy are most likely present ?
Correct Answer: Wilms' tumour
Description: Ans. is 'b' i.e.,Wilm's tumour Wilm's tumour is associated with three groups of congenital malformations. o The risk of Wilm's tumour is increased in these conditions. o These are ? WAGR syndrome - It consists of o Aniridia o Mental retardation o Genital anomalies The risk of Wilm's tumour is increased by 33% in this syndrome. Denys - Drash syndrome - It consists of o Gonadal dysgenesis (male pseudohermaphroditism) o Nephropathy leading to renal failure. Majority of patients with this syndrome have ranal failure. Beckwith - Wiedeman syndrome - It consists of o Enlargement of body organs o Renal medullary cysts. o Hemihyperophy o Abnormal large cells in adrenal coex.
Category:
Pathology
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