We report a case of hepatoblastoma in a 10-year-old girl with mosaic-type trisomy 18. A comprehensive literature review reveals only 2 cases involving mosaic trisomy 18 patients. Our patient underwent an abbreviated chemotherapy course before complete surgical resection. Her hepatoblastoma did not contain cells with trisomy 18. The conservative management approach resulted in a successful outcome; she remains disease free >2 years after surgery. Along with presenting a literature review, this report demonstrates a favorable outcome in a mosaic trisomy 18 child with hepatoblastoma where tumor cells lacked a trisomy 18 karyotype.
Background: No recognized standards exist for teaching patients and their caregivers ventricular assist device (VAD) self-care skills. We compared the effectiveness of a VAD simulation-based mastery learning (SBML) self-care training curriculum with usual VAD self-care training. Methods and Results: VAD patients and their caregivers were randomized to SBML or usual training during their implant hospitalization. The SBML group completed a pretest on 3 VAD self-care skills (controller, power source, and dressing change), then viewed videos and participated in deliberate practice on a simulator. SBML participants took a posttest and were required to meet or exceed a minimum passing standard for each of the skills. The usual training group completed the existing institutional VAD self-care teaching protocol. Before hospital discharge, the SBML and usual training groups took the same 3 VAD self-care skills tests. We compared demographic and clinical information, self-confidence, total participant training time, and skills performance between groups. Forty participants completed the study in each group. There were no differences in demographic and clinical information, self-confidence, or training time between groups. More participants in the SBML group met the minimum passing standard compared with the usual training group for controller (37/40 [93%] versus 25/40 [63%]; P =0.001), power source (36/40 [90%] versus 9/40 [23%]; P <0.001), and dressing change skills (19/20 [95%] versus 0/20; P <0.001). Conclusions: SBML provided superior VAD self-care skills learning outcomes compared with usual training. This study has important implications for patients due to the morbidity and mortality associated with improper VAD self-care. Clinical Trial Registration: URL: http://www.clinicaltrials.gov . Unique identifier: NCT03073005.
This study demonstrates that secondary orbital reconstruction can achieve excellent functional and cosmetic results with high patient satisfaction and minimal complications. Secondary reconstruction of previously repaired orbital fractures should be considered when clinically indicated.
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