Intraocular Medulloepithelioma: AIRP Best Cases in Radiologic-Pathologic Correlation.

Editor's Note.-RadioGraphics continues to publish radiologic-pathologic case material selected from the American Institute for Radiologic Pathology (AIRP) "best case" presentations. The AIRP conducts a 4-week Radiologic Pathology Correlation Course, which is offered five times per year. On the penultimate day of the course, the best case presentation is held at the American Film Institute Silver Theater and Cultural Center in Silver Spring, Md. The AIRP faculty identifies the best cases, from each organ system, brought by the resident attendees. One or more of the best cases from each of the five courses are then solicited for publication in RadioGraphics. These cases emphasize the importance of radiologic-pathologic correlation in the imaging evaluation and diagnosis of diseases encountered at the institute and its predecessor, the Armed Forces Institute of Pathology (AFIP).

Ectopic cerebellum in the posterior cranial fossa.

Isolated, well-differentiated ectopic cerebellar tissue is extremely rare, with only eight cases in the literature. We describe a unique case of histopathologically proven ectopic cerebellar tissue presenting as a discrete extra-axial mass in the posterior cranial fossa. We describe the clinical, CT and MRI findings, as well as the surgical and histopathological findings and review the relevant literature.

Immediate functional progression program in adolescent athletes with a spondylolysis.

OBJECTIVE: To assess the preliminary evidence for the efficacy and safety of an immediate functional progression program to treat adolescent athletes with an active spondylolysis. DESIGN: Prospective single-arm trial. SETTING: Hospital-based sports medicine and physical therapy clinic. PARTICIPANTS: Twelve adolescent athletes (14.2 ± 2 years, 25% female) with an active spondylolysis. MAIN OUTCOME MEASURES: Clinical outcomes included time out of sport, Micheli Functional Scale (Function and Pain) and adverse reactions. Clinical outcomes were assessed at baseline, 1 month, 3 months and 6 months. Magnetic resonance imaging was performed at baseline and 3 months to confirm diagnosis and assess healing of lesion. RESULTS: Eleven participants (92%) fully returned to sport in a median time of 2.5 months (75 days; interquartile range 55 days, 85 days). All participants demonstrated marked improvements in pain and function by the end of the program. One participant (8%) had an adverse reaction during care with a significant recurrence of LBP and had not returned to sport by 6 months. Magnetic resonance imaging demonstrated improvement of the spondylolytic lesion in all but one participant. CONCLUSION: The immediate functional progression program appears a viable method for treating active spondylolysis and warrants future research.

Impact of Nurse Certification Rates on Patient Satisfaction and Outcomes: A Literature Review.

The Institute of Medicine's report The Future of Nursing: Leading Change, Advancing Health states that continued competence requires lifelong learning. Certification measures lifelong learning by validating the expertise of nurses in specialty areas beyond those required by licensure examinations. Current research provides limited quantitative evidence to support a positive correlation between nurse certification rates and patient satisfaction and outcomes. The health care industry and affiliated professionals are experiencing increased public scrutiny and accountability through mandated quality of care measures that impact monetary reimbursement. Increased public scrutiny and accountability highlights the need for research to substantiate the quantitative benefits of nurse certification on patient satisfaction and outcomes. The Hospital Consumer Assessment of Healthcare Providers and Systems Survey scores, the Hospital Value-Based Purchasing program, and the National Database of Nursing Quality Indicators(®) are potential sources for producing the reliable and valid evidence needed to convince RNs, hospital administrators, and all other stakeholders that nurse certification has a quantifiable correlation with patient satisfaction and outcomes.

m.8993T>G-Associated Leigh Syndrome with Hypocitrullinemia on Newborn Screening.

Citrulline is among the metabolites measured by expanded newborn screening (NBS). While hypocitrullinemia can be a marker for deficiency of proximal urea cycle enzymes such as ornithine transcarbamylase (OTC), only a handful of state newborn screening programs in the United States officially report a low citrulline value for further work-up due to low positive predictive value. We report a case of a male infant who was found to have hypocitrullinemia on NBS. After excluding proximal urea cycle disorders by DNA sequencing, his NBS result was felt to be a false positive. At 4 months of age, he developed poor feeding, failure to thrive, apnea and infantile spasms with a progression to intractable seizures, as well as persistent hypocitrullinemia. He was diagnosed with Leigh syndrome due to a maternally inherited homoplasmic m.8993T>G mutation in the ATPase 6 gene. His mother, who had previously been diagnosed with cerebral palsy, was concurrently diagnosed with neuropathy, ataxia, and retinitis pigmentosa (NARP) due to heteroplasmy of the same mutation. She had progressive muscle weakness, ataxia, and speech dyspraxia. The m.8993T>G mutation causes mitochondrial ATP synthase deficiency and it is hypothesized to undermine the synthesis of citrulline by CPS1. In addition to proximal urea cycle disorders, the evaluation of an infant with persistent hypocitrullinemia should include testing for the m.8993T>G mutation and other disorders that cause mitochondrial dysfunction.