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How often is Klippel-Feil Syndrome associated with congential heart disease presentation of five cases and a review of the literature

Published on: 3rd September, 2019

OCLC Number/Unique Identifier: 8270717914

Introduction: Klippel-Feil syndrome (KFS), is a bone disorder characterized by the abnormal joining (fusion) of two or more spinal bones in the neck (cervical vertebrae), which is present from birth. Three major features result from this abnormality: a short neck, a limited range of motion in the neck, and a low hairline at the back of the head. In some individuals, KFS can be associated with a variety of additional symptoms and physical abnormalities which contribute in the deterioration and complication of the condition of the child. Aim of presentation: Here, we report five children from Kosovo with KFS associated with different heart abnormalities, clinical presentation, diagnosis, management, and outcomes of selected conditions in resources-limited settings. Methods: Retrospectively we analysed medical reports of five children, diagnosed at different age with congenital disease and clinical and lab signs of Klippel-Feil syndrome. Conclusion: Basing on our cases, all diagnosed in a small country as a Kosovo, we can conclude that KFS is not such a rare condition. In addition, such syndrome is not so rarely associated with different congenital heart disease. In four cases cardiac surgery was indicated and successfully was done abroad Kosovo in the lack of such services in Kosovo.
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Biological dose calculation using variable RBE in Single- and Multi-field Optimization IMPT plans for 3 Brain Tumor patients

Published on: 14th February, 2019

OCLC Number/Unique Identifier: 8022197964

Purpose: The purpose of this study was to evaluate biological dose in single-field optimization (SFO) and multi-field optimization (MFO) intensity-modulated proton therapy (IMPT) plans for brain tumor patients that used a fixed relative biological effectiveness (FRBE) and those that used a variable RBE (VRBE). Materials and methods: SFO and MFO IMPT plans were planned by the Varian Eclipse treatment planning system for three brain tumor patients. Dose and linear energy transfer (LET) distributions for each plan were recomputed using an in-house fast Monte Carlo dose calculator system, and then biological dose distributions were calculated with a FRBE of 1.1 or with a previously published VRBE model. We then compared biological dose distributions obtained by the VRBE with those obtained by the FRBE. Results: Doses obtained by the VRBE for the gross tumor volume and clinical target volume in all plans were 1%-2% larger than those obtained by the FRBE. The minimum dose obtained by the VRBE for the brainstem in the SFO IMPT of one patient was 140% larger than that obtained by the FRBE, but the difference was only 5.3 cGy (RBE). The difference in maximum dose for the optic chiasm in the MFO IMPT of another patient was less than 3.2%, but the dose difference was 149.2 cGy (RBE). We also found that no major differences were seen between the biological dose differences in the SFO IMPT plans and those in the MFO IMPT plans. Conclusion: We could observe biological dose differences between the FRBE and the VRBE in the SFO and the MFO IMPT plans for brain tumor patients.
Cite this ArticleCrossMarkPublonsHarvard Library HOLLISGrowKudosResearchGateBase SearchOAI PMHAcademic MicrosoftScilitSemantic ScholarUniversite de ParisUW LibrariesSJSU King LibrarySJSU King LibraryNUS LibraryMcGillDET KGL BIBLiOTEKJCU DiscoveryUniversidad De LimaWorldCatVU on WorldCat