One hundred eighteen cases of nevoid basal cell carcinoma syndrome (NBCCS, Gorlin's syndrome or basal cell nevus syndrome) are presented in this study. In aiming to ascertain all the affected families in Australia, we have examined the largest series to date. Relative frequencies of associated complications are presented and compared with those of the recent English survey by Evans et al. [J Med Genet 30:460-464, 1993]. The frequencies of most manifestations are similar. However, one major difference is that the multiple basal cell carcinomas are manifest from an earlier age in the Australian population, which probably reflects greater exposure to ultraviolet radiation. Of the 64 families ascertained, 37 represented simplex cases, and, accordingly, the apparent new mutation rate is surprisingly high (14-81%) given the lack of impact of NBCCS on reproductive capabilities. There is some evidence to suggest that this may be attributable to anticipation.

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PurposeTeacher attrition is a significant international concern facing administrators. Although a considerable amount of literature exists related to the causes of job dissatisfaction and teachers leaving the profession, relatively few theoretical models test the complex interrelationships between these variables. The goal of this paper is to partially fill this gap.Design/methodology/approachUsing a sample of 479 certified teachers who taught either at elementary (55.3 percent), middle (33.0 percent), or high (10.6 percent) school levels, three competing theoretical models with variables related to teacher stress or support were tested using structural equation modeling to predict job dissatisfaction and eventual intention to quit.FindingsThe most parsimonious model revealed that student stressors completely mediated the relationship between teacher efficacy related to student engagement and job dissatisfaction, with social support superiors and student stressors being best predictors of job dissatisfaction. Although important within the school system, teacher workload stressors and social support from colleagues did not contribute significantly to the models.Originality/valueTheoretical models are needed to assist school administrators and researchers in developing programs to improve teacher retention and to predict those teachers who will struggle within the profession. Moreover, developing and testing comprehensive models associated with variables related to teacher and student success is critical for a well functioning school system.