Caring for Patients with WAGR Syndrome

WAGR syndrome (WAGRS) is a rare genetic disorder that affects both males and females. It is named after its associated clinical symptoms: Wilms' tumor (i.e., a type of kidney cancer), aniridia (i.e., underdevelopment or absence of the iris of the eye), genitourinary anomalies (e.g., undescended testicles or hypospadias [i.e., abnormal placement of the urinary opening in males along the shaft of the penis], underdeveloped ovaries in females), and mental retardation.

WAGRS is caused by a deletion of varying lengths of genes along the short arm of chromosome 11. The deletion can be inherited or can occur spontaneously during egg, sperm, or early fetal development. WAGRS is most often identified in newborns following an unremarkable pregnancy and birth. The presence of aniridia combined with genital anomalies warrants evaluation for the syndrome. Diagnosis is made based on results of genetic testing confirming deletions on chromosome 11. 

Disease presentation varies: children with WAGRS may have only one or two of the characteristic physical and/or mental conditions signified by the syndrome initials. Treatment is individualized according to the patient presentation. Patients with Wilms' tumor may require surgical intervention, radiation, or chemotherapy. Corrective lenses, medications, and surgery may be necessary for patients with ophthalmic involvement. Patients with genitourinary anomalies (e.g., undescended testicles) often require urological surgical repair. In the event of renal failure, dialysis or kidney transplantation may be necessary. Special education services and speech, physical, and/or occupational therapy may be appropriate for patients with cognitive impairments. Nurses should reinforce clinician's explanation of treatment options, and provide emotional/psychological support and educate.

Please login to your Nursing Reference Center subscription to read the Quick Lesson on "WAGR Syndrome."

Recently, the evidence-based care sheet Depression and Spirituality was revised following review under the systematic literature surveillance program. Information of value to nursing practice regarding depression and spirituality was a research study.

The results of a research study indicate that anxiety and depression frequently occur associated with chronic and debilitating conditions, such as cardiovascular diseases, chronic obstructive pulmonary disease, diabetes mellitus, arthritis, and cancer, reducing the quality of life and increasing the burden of the underlying condition. Spirituality can help improve or alleviate symptoms of depression.

We invite you to login to the Nursing Reference Center to read new and updated content as it becomes available.