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1.
J Pediatr Hematol Oncol ; 45(3): 152-154, 2023 04 01.
Article in English | MEDLINE | ID: mdl-36897628

ABSTRACT

Opsoclonus myoclonus ataxia syndrome (OMAS) is a rare disorder that causes significant neurodevelopmental sequelae in children. Approximately half of pediatric OMAS cases are paraneoplastic, typically associated with localized neuroblastic tumors. Since early persistence or relapse of OMAS symptoms is common even after tumor resection, OMAS relapses may not routinely prompt reevaluation for recurrent tumors. We report a 12-year-old girl with neuroblastic tumor recurrence associated with OMAS relapse a decade after initial treatment. Providers should be aware of tumor recurrence as a trigger for distant OMAS relapse, raising intriguing questions about the role of immune surveillance and control of neuroblastic tumors.


Subject(s)
Opsoclonus-Myoclonus Syndrome , Female , Humans , Child , Opsoclonus-Myoclonus Syndrome/etiology , Opsoclonus-Myoclonus Syndrome/therapy , Neoplasm Recurrence, Local , Ataxia/therapy , Ataxia/complications
2.
Transplant Cell Ther ; 29(3): 207.e1-207.e5, 2023 03.
Article in English | MEDLINE | ID: mdl-36610491

ABSTRACT

Institutions that perform hematopoietic cell transplantation (HCT) are required by law to report standardized, structured data on transplantation outcomes. A key post-transplantation outcome is engraftment, the time between HCT infusion and reemergence of circulating neutrophils and platelets. At our center, we found that manual chart abstraction for engraftment data was highly error-prone. We developed a custom R/Shiny application that automatically calculates engraftment dates and displays them in an intuitive format to augment the manual chart review. Our hypothesis was that use of the application to assist with calculating and reporting engraftment dates would be associated with a decreased error rate. The study was conducted at a single tertiary care institution. The application was developed in a collaborative, multidisciplinary fashion by members of an embedded cellular therapy informatics team. Retrospective validation of the application's accuracy was conducted on all malignant HCTs from February 2016 to December 2020 (n = 198). Real-world use of the application was evaluated prospectively from April 2021 through April 2022 (n = 53). The Welch 2-sample t test was used to compare error rates preimplementation and postimplementation. Data were visualized using p charts, and standard special cause variation rules were applied. The accuracy of reported data postdeployment increased dramatically; the engraftment error rate decreased from 15% to 3.8% for neutrophils (P = .003) and from 28% to 1.9% for platelets (P < .001). This study demonstrates the effective deployment of a custom R/Shiny application that was associated with significantly reduced error rates in HCT engraftment reporting for operational, research, and regulatory purposes. Users reported subjective satisfaction with the application and that it addressed difficulties with the legacy manual process. Identifying and correcting erroneous data in engraftment reporting could lead to a more efficient and accurate nationwide assessment of transplantation success. Furthermore, we show that it is possible and practical for academic medical centers to create and support embedded informatics teams that can quickly build applications for clinical operations in a manner compliant with regulatory requirements.


Subject(s)
Hematopoietic Stem Cell Transplantation , Retrospective Studies , Transplantation, Homologous , Registries , Automation
3.
Psychiatr Q ; 89(4): 1007-1018, 2018 12.
Article in English | MEDLINE | ID: mdl-30145770

ABSTRACT

Medical students often become involved as post-disaster emergency responders despite incomplete training, and in doing so may suppress their immediate experiences as victims and survivors. This experience, however, may lead them to increase their motivation to help others. We examined how cognitive and emotional reactions to disaster correlated with posttraumatic growth (PTG) in medical students in Fukushima, Japan after the Great East Japan Earthquake of March 11, 2011. To date, Fukushima continues to suffer from radiation concerns following the nuclear power plant meltdown. In a survey three years after the onset of a long-term disaster, with a cross-sectional research design, medical students (N = 494) reported their negative post-disaster reactions, desire to help, and demonstrations of capability, and completed the Posttraumatic Growth Inventory (PTGI). We conducted hierarchical regression analyses and found that the addition of variables pertaining to negative post-disaster reactions (e.g. confusion, anger, and sadness) led to the largest increase in predictive value for PTGI scores; students reporting a past traumatic experience were also more likely to experience PTG. Our results indicate that weathering stressful disaster circumstances created opportunities for positive personal growth and reinforcement at a crucial time in medical students' professional development.


Subject(s)
Disasters , Earthquakes , Emotions/physiology , Posttraumatic Growth, Psychological , Stress Disorders, Post-Traumatic/psychology , Students, Medical/psychology , Adult , Cross-Sectional Studies , Female , Humans , Japan , Male , Young Adult
4.
J Clin Neurosci ; 34: 259-263, 2016 Dec.
Article in English | MEDLINE | ID: mdl-27476892

ABSTRACT

Prior studies, using systemic hypertension and elastase infusion, have induced cerebral aneurysm (CA) formation in mice. However, the CAs induced were rapidly formed, relatively large, and often ruptured. These features are not completely representative of human CAs. We set out to develop a mouse model representative of the early pathological features of human CA. Twenty male C57/BL6 mice were placed in a stereotactic frame. Low dose elastase solution (2µl/min) was manually injected into the right basal cistern. Human angiotensin II (0.11µl/h) was infused subcutaneously. Mice were observed for 2-3weeks prior to euthanasia. Early CA histopathological features including endothelial change (EC) and internal elastic lamina degeneration (IELD) were systematically sought at major cerebral arterial bifurcations. Brains were harvested from 11 of 15 mice, yielding 27 bifurcations. Sub-arachnoid haemorrhage (SAH) without CA formation was observed in one brain. Macroscopic CA without SAH was observed in another brain. Early CA features were observed in 8/11 (73%) brains. All bifurcations with IELD demonstrated EC: where EC was absent, IELD was also absent. EC severity appeared to correlate with IELD severity. EC and IELD were both severe within the CA. Using lower dose elastase solution than previously employed, we developed a model of early CA pathology. Our model demonstrated that the spectrum of known early CA pathology can be created at multiple bifurcations in mice, with EC severity appearing to correlate with IELD severity. This model permits the study of factors which potentially advance or retard the progression of CA formation.


Subject(s)
Disease Models, Animal , Intracranial Aneurysm/chemically induced , Intracranial Aneurysm/pathology , Pancreatic Elastase/pharmacology , Animals , Male , Mice , Mice, Inbred C57BL , Pancreatic Elastase/administration & dosage
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