Malignant B-cell lymphoma in an infant with severe combined immunodeficiency with short-limbed skeletal dysplasia

Abstract In an infant with skeletal anomalies and haemolytic disease, intestinal perforation was caused by necrosis of an as yet undetected B-cell lymphoma. Severe combined immunodeficiency with short-limbed skeletal dysplasia was diagnosed. This is the first published report of a patient with this syndrome in combination with haemolytic disease and B-cell-lymphoma.     

Changes in gap junctional intercellular communication in mouse skin carcinogenesis

Gap junction intercellular communication (GJIC) has been measured in cell lines that represent different stages of chemically induced mouse skin carcinogenesis. No significant difference in GJIC, as measured by dye spread, was found in cultures of normal keratinocyte, papilloma or squamous carcinoma cell lines. There was no correlation, in this system, between the presence of a mutant Ha-ras gene and down- regulation of communication. There was, however, a marked decrease in GJIC (80-90%) on progression from squamous to spindle carcinoma cells. Measurement of GJIC in somatic cell hybrids shows that the genetic defect responsible for this down-regulation is recessive and is common to two independently isolated spindle cell lines. No abnormalities were found in the spindle cells in expression of connexin 43, a cell component involved in gap junction formation and permeability. However, expression of E-cadherin, a cell-cell adhesion molecule implicated in the process of gap junction formation, was missing in the spindle carcinoma cells. Introduction of an E-cadherin cDNA into the spindle cells partially restored junctional communication without causing any noticeable alterations in cell morphology. During the study a non- tumourigenic keratinocyte line, a sub-clone of a normal keratinocyte line, was also found to have a low level of GJIC. However, the defect in this line was shown, by genetic complementation in somatic cell hybrids, to be different from that in the spindle carcinoma cell lines. Consistent with these data, analysis by immunofluorescence shows an abnormal distribution of connexin 43 in these cells.      

Exposure and peritraumatic response as predictors of posttraumatic stress in children following the 1995 Oklahoma City bombing

Studies have demonstrated a positive relationship between exposure and posttraumatic stress, but one's subjective appraisal of danger and threat at the time of exposure may be a better predictor of posttraumatic stress than more objective measures of exposure. We examined the role of peritraumatic response in posttraumatic stress reactions in over 2,000 middle school children 7 weeks after the 1995 Oklahoma City, Oklahoma, bombing. While many children reported hearing and feeling the blast and knowing direct victims, most were in school at the time of the explosion and therefore were not in direct physical proximity to the incident. Physical, interpersonal, and television exposure accounted for 12% of the total variance in our measure of posttraumatic stress when peritraumatic response was ignored. Peritraumatic response and television exposure accounted for 25% of the total variance, and physical and interpersonal exposure were not significant in this context. These findings suggest the importance of peritraumatic response in children's reactions to terrorism. These carly responses can be used to help determine which children may experience difficulty over time.