RESUMO
Barth syndrome is an X-linked disorder characterized by dilated cardiomyopathy, cyclic neutropenia, skeletal myopathy, abnormal mitochondria, and growth deficiency. The primary defect is a mutation in the TAZ gene on the X chromosome at Xq28, resulting in abnormal phospholipid biosynthesis and cardiolipin deficiency. To date, there has been no systematic evaluation of the cardiac phenotype. We report five cases of cardiac arrest and/or placement of an internal cardiac defibrillator with documented ventricular arrhythmia. We suggest that ventricular arrhythmia is part of the primary phenotype of the disorder and that patients should be screened accordingly.
Assuntos
Cardiomiopatia Dilatada , Desfibriladores Implantáveis , Doenças Genéticas Ligadas ao Cromossomo X , Taquicardia Ventricular , Fibrilação Ventricular , Aciltransferases , Adolescente , Cardiomiopatia Dilatada/genética , Cardiomiopatia Dilatada/fisiopatologia , Cardiomiopatia Dilatada/terapia , Criança , Eletrocardiografia , Doenças Genéticas Ligadas ao Cromossomo X/genética , Doenças Genéticas Ligadas ao Cromossomo X/fisiopatologia , Doenças Genéticas Ligadas ao Cromossomo X/terapia , Predisposição Genética para Doença , Parada Cardíaca/etiologia , Parada Cardíaca/terapia , Humanos , Masculino , Mutação , Fenótipo , Proteínas/genética , Taquicardia Ventricular/genética , Taquicardia Ventricular/fisiopatologia , Taquicardia Ventricular/terapia , Fatores de Transcrição/genética , Fibrilação Ventricular/genética , Fibrilação Ventricular/fisiopatologia , Fibrilação Ventricular/terapiaRESUMO
A survey was conducted of the graduates of a medical technology program to assess their feelings about their jobs. Specifically, the study attempted to determine the relationship between a "sense of accomplishment" and "career commitment." The relationship between job satisfaction and sense of accomplishment was also studied. Analysis using chi-square revealed a relationship between the respondent's sense of accomplishment and career commitment. Responses on accomplishment were then compared in terms of the graduates' ratings of selected job characteristics. These findings are compatible with those of other studies on job satisfaction of medical technologists. That is, medical technologists are a group whose motivational factors tend to be intrinisic to the job. These factors can be identified as achievement, recognition for achievement, the work itself, responsibility, and growth or advancement. The findings have implications for determining components related to job satisfaction in administrative practices.
