The topic 11p Deletion Syndrome you are seeking is a synonym, or alternative name, or is closely related to the medical condition WAGR Syndrome.

Quick Summary:

• WAGR Syndrome is a rare genetic disorder in which different conditions co-exist within a single individual. The 4 main features of this syndrome are Wilms’ tumor, Aniridia, Genitourinary abnormalities, and Retardation (or intellectual disability); hence the acronym WAGR
• The signs and symptoms of WAGR Syndrome generally involve the kidneys (Wilms’ tumor), reduction or lack of colored part of the eyes (aniridia), malformation of the urinary tract, problems with the genitalia (genitourinary abnormalities), and diminished mental acuity (intellectual disability)
• Kidney failure and conditions, such as diabetes and hyperlipidemia (in obese individuals), are complications that are commonly observed in children with WAGR Syndrome
• WAGR Syndrome is caused by genetic mutational abnormalities. Specific gene mutations have been identified. The common gene mutations include the ‘deletion of a portion of the short arm (p) of chromosome 11’. This deletion leads to the loss of specific genes called PAX6 and WT1. Both PAX6 and WT1 genes are important to the development of the eyes, brain, genitalia, kidneys, and the urinary tract
• Additionally, deletion of the BDNF gene can cause WAGRO Syndrome. The BDNF gene regulates eating, drinking, and body weight in individuals. The loss of the BDNF gene on chromosome 11 leads to obesity, the “O” in WAGRO Syndrome (in addition to other characteristic signs and symptoms of WAGR Syndrome)
• A very small proportion of those with WAGR Syndrome have a family history of the condition, which may be a risk factor for development of this disorder
• The diagnosis for WAGR Syndrome can involve a physical examination, assessment of symptoms, genetic analysis of the chromosome for large deletions, and the use of specific genetic techniques. Genetic analysis of the parents prior to pregnancy, as well as prenatal analysis of the fetus, can potentially yield important information regarding WAGR Syndrome
• Treatment options for WAGR Syndrome are dictated by the symptoms that are observed. Children affected by WAGR Syndrome may require coordinated and long-term medical care. Those with Wilms’ tumor due to WAGR Syndrome may require chemotherapy, radiotherapy, and/or surgery
• Periodic and life-long health monitoring is a requisite for those with WAGR Syndrome. Supportive care from the family is also an important aspect of managing symptoms of the disorder. Specifically, children may need special education and therapy in order to aid normal development
• Individuals with WAGR Syndrome are reported to have generally good prognosis (long-term survival), if provided with periodic health check-ups, which is followed by prompt symptomatic treatment. Likewise, the prognosis for Wilms’ tumor is also reported to be excellent with adequate treatment
• Due to the fact that WAGR Syndrome is caused by the loss of genetic material; there are currently no standard means of prevention. If a family history of the condition is known, genetic testing of the parents prior to pregnancy may help identify the specific risks. If prenatal testing reveals that the child likely has WAGR Syndrome, counselling may help the parents understand the disease better

Please find comprehensive information on WAGR Syndrome regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention, prognosis, and additional useful information HERE.