What major complication is associated with WAGR syndrome?

WAGR syndrome is a genetic disorder that is characterized by a specific set of features, with the most significant complication being the increased risk of Wilms tumor, a type of kidney cancer. This syndrome is caused by a deletion on chromosome 11p13, which affects several genes, including the WT1 gene that plays a crucial role in kidney and gonadal development and function. Individuals with WAGR syndrome have a significantly higher incidence of Wilms tumor compared to the general population, making it a major concern for healthcare providers monitoring affected individuals.

While neurofibromas, cardiovascular defects, and intellectual disability can be seen in various genetic disorders or syndromes, they are not defining features or major complications specifically associated with WAGR syndrome. Intellectual disability is indeed common in individuals with WAGR syndrome, but the most pressing medical issue in terms of complications and the need for surveillance is the risk for Wilms tumor. Therefore, the association between WAGR syndrome and Wilms tumor is well-documented, emphasizing the necessity for early detection and management of this potential complication in affected individuals.