Browsing by Subject "Spinal Dysraphism"
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Item The Adult Spina Bifida Patient: Does a Delay in Referral Impact Urodynamic Findings and Clinical Outcomes? Recommendations for Transition of Care(2017-01-17) Eastman, Jessica; Lemack, Gary; Harris, Catherine; Howard, CatherineINTRODUCTION: Improvements in the management of children with myelomeningocele have resulted in an influx of such patients, many of whom have complex neurogenic bladder conditions, to adult urologists. We reviewed the presenting symptoms, urodynamic findings, and changes in clinical management of adults with spina bifida, specifically focusing on the relationship between the delay in urological follow-up and clinical outcomes. METHODS: All patients with neurological conditions that presented for urologic evaluation at a tertiary referral center have been prospectively entered into a database since 2000. Data from patients with spina bifida including, bladder management, chief complaint, urodynamic findings (UDS), surgical interventions and upper tract imaging were analyzed. RESULTS: Of the 1110 patients in the database, 60 patients with spina bifida were identified (51.7% male, 48.3% female). Median age at presentation was 33 (16-64). The majority of patients presented for symptom evaluation (75%) vs. establishing care (25%). The most common presenting symptoms were incontinence (n=18, 30%) and urinary tract infection (UTI) (n=15, 25%). Patients who had documented prior urologic evaluation were assessed for the interval to presentation (n=53). Patients were classified as having their last evaluation within the preceding 12 months (n=23, 43.3%), between 12 and 24 months (n=17, 32%), between 2 and 5 years (n=11, 20.8%) or greater than 5 years prior (n=2, 3.8%). Patients were significantly more likely to present within 12 months of their last evaluation if they were symptomatic (p=0.022). Patients presenting more than one year from their last evaluation were more likely to have DO (p=0.0215), though neither altered compliance nor DESD were associated with delay in diagnosis. As seen with children, the UDS diagnosis of impaired compliance was significantly associated with abnormal imaging findings (p=0.0328). Overall, 42% of this cohort required intervention following referral, and urologic workup including urodynamics altered clinical management in 58.9% of patients. CONCLUSION: Spina Bifida patients continue to require close surveillance into adulthood, and this evaluation must include urodynamic testing. Additionally, there is indication that patients who delay care are more likely to have UDS abnormalities that might necessitate changes in management strategies. We advocate follow-up of less than 12 months between adult urology clinics or within one year after pediatric surveillance has terminated.Item PDGFRalpha Signaling Requirements in the Development of Tissues Derived From Sclerotome and Dermatome(2007-05-23) Pickett, Elizabeth Ann; Tallquist, Michelle D.The Platelet-Derived Growth Factor Receptors (PDGFR) play roles in the development of multiple mesenchymal cell types. Upon ligand binding, the receptors signal through a number of intracellular signaling molecules and elicit many cellular responses including migration, proliferation, and differentiation. Studies utilizing tissuespecific deletion permit the elucidation of cell autonomous phenotypes, while analysis of signaling point mutants permits identification of the function of specific downstream effectors. My studies use both methods to uncover the role of PDGFRalpha signaling in the development of skin and bone. An unexplained phenotype of PDGFRalpha null embryos is a defect in the axial skeletal that leads to spina bifida. Using conditional gene deletion I determined that the population of cells responsible for this phenotype is not the chondrocyte but is instead another derivative of the sclerotome. These results demonstrate that signals from adjacent tissues can play important roles in the differentiation of bone populations and identifies an etiology for spina bifida that is not directly caused by neural tube or bone defects. Interestingly, loss of PI3K signaling downstream of PDGFRalpha also results in spina bifida. I found that the sclerotome population described above requires PDGFRalpha signaling to migrate dorsal to the neural tube via PI3K activation. Without this signaling event, downstream effectors including Akt, p70S6K, and PAK1 are not activated and the cells fail to form Rac-associated membrane ruffles. This is the first in vivo evidence that PI3K driven pathways are required for migration downstream of the PDGFRalpha .