Late-Onset Erythropoietic Porphyria Caused by a Chromosome 18q Deletion in Erythroid Cells

The erythropoietic porphyrias, erythropoietic protoporphyria and congenital erythropoietic porphyria, result from germline mutations in the ferrochelatase gene and uroporphyrinogen III synthase gene, respectively. Both conditions normally present in childhood but rare cases with onset past the age of 40 y have been reported. Here we show that late-onset erythropoietic protoporphyria can be caused by deletion of the ferrochelatase gene in hematopoietic cells with clonal expansion as part of the myelodysplastic process. This is the first direct demonstration of porphyria produced by an acquired molecular defect restricted to one tissue. Some other cases of late-onset erythropoietic porphyria may be explained by a similar mechanism

Participatory Action Research: Reflections on Critical Incidents in a PAR Project

This article describes a participatory action research (PAR) project designed to evaluate Parent to Parent progra ms in f ive states in the United States. The PAR team consisted of two groups: 1) parent leaders of programs that create one-to-one matches between help-seeking parents of children with disabilities and volunteer supporting parents and 2) university-based researchers. Based on a narrative record of the project, critical incidents are presented along with reflections of factors that contributed to the success of the project overall. The project successfully gathered evaluative data about Parent to Parent self-help programs. Results of both the PAR project and the s tudy i t created are presented. A process of developing a shared understanding of the programs and of the purposes for evaluating them, along with an on-going willingness of parents and researchers to compromise, led to creative solutions t

The eye contact effect: mechanisms and development

The ‘eye contact effect’ is the phenomenon that perceived eye contact with another human face modulates certain aspects of the concurrent and/or immediately following cognitive processing. In addition, functional imaging studies in adults have revealed that eye contact can modulate activity in structures in the social brain network, and developmental studies show evidence for preferential orienting towards, and processing of, faces with direct gaze from early in life. We review different theories of the eye contact effect and advance a ‘fast-track modulator’ model. Specifically, we hypothesize that perceived eye contact is initially detected by a subcortical route, which then modulates the activation of the social brain as it processes the accompanying detailed sensory information

Content analysis of elite articulations

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/67140/2/10.1177_002200276400800406.pd

Work-Unit Absenteeism: Effects of Satisfaction, Commitment, Labor Market Conditions, and Time

Prior research is limited in explaining absenteeism at the unit level and over time. We developed and tested a model of unit-level absenteeism using five waves of data collected over six years from 115 work units in a large state agency. Unit-level job satisfaction, organizational commitment, and local unemployment were modeled as time-varying predictors of absenteeism. Shared satisfaction and commitment interacted in predicting absenteeism but were not related to the rate of change in absenteeism over time. Unit-level satisfaction and commitment were more strongly related to absenteeism when units were located in areas with plentiful job alternatives