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Background. Chronic granulomatous disease (CGD) is due to defective nicotinamide adenine dinucleotide phosphate oxidase activity and characterized by recurrent infections with a limited spectrum of bacteria and fungi as well as inflammatory complications. To understand the impact of common severe infections in CGD, we examined the records of 268 patients followed at a single center over 4 decades. Methods. All patients had confirmed diagnoses of CGD, and genotype was determined where possible. Medical records were excerpted into a standard format. Microbiologic analyses were restricted to Staphylococcus, Burkholderia, Serratia, Nocardia, and Aspergillus. Results. Aspergillus incidence was estimated at 2.6 cases per 100 patient-years; Burkholderia, 1.06 per 100 patient-years; Nocardia, 0.81 per 100 patient-years; Serratia, 0.98 per 100 patient-years, and severe Staphylococcus infection, 1.44 per 100 patient-years. Lung infection occurred in 87% of patients, whereas liver abscess occurred in 32%. Aspergillus incidence was 55% in the lower superoxide-producing quartiles (quartiles 1 and 2) but only 41% in the higher quartiles (rate ratio, <0.0001). Aspergillus and Serratia were somewhat more common in lower superoxide producing gp91phox deficiency. The median age at death has increased from 15.53 years before 1990 to 28.12 years in the last decade. Fungal infection carried a higher risk of mortality than bacterial infection and was the most common cause of death (55%).Gastrointestinal complications were not associated with either infection or mortality. Conclusions. Fungal infections remain a major determinant of survival in CGD. X-linked patients generally had more severe disease, and this was generally in those with lower residual superoxide production. Survival in CGD has increased over the years, but infections are still major causes of morbidity and mortality.

Pulmonary nontuberculous mycobacterial (PNTM) disease is increasing, but predisposing features have been elusive. To prospectively determine the morphotype, immunophenotype, and cystic fibrosis transmembrane conductance regulator genotype in a large cohort with PNTM. We prospectively enrolled 63 patients with PNTM infection, each of whom had computerized tomography, echocardiogram, pulmonary function, and flow cytometry of peripheral blood. In vitro cytokine production in response to mitogen, LPS, and cytokines was performed. Anthropometric measurements were compared with National Health and Nutrition Examination Survey (NHANES) age- and ethnicity-matched female control subjects extracted from the NHANES 2001-2002 dataset. Patients were 59.9 (+/-9.8 yr [SD]) old, and 5.4 (+/-7.9 yr) from diagnosis to enrollment. Patients were 95% female, 91% white, and 68% lifetime nonsmokers. A total of 46 were infected with Mycobacterium avium complex, M. xenopi, or M. kansasii; 17 were infected with rapidly growing mycobacteria. Female patients were significantly taller (164.7 vs. 161.0 cm; P < 0.001) and thinner (body mass index, 21.1 vs. 28.2; P < 0.001) than matched NHANES control subjects, and thinner (body mass index, 21.1 vs. 26.8; P = 0.002) than patients with disseminated nontuberculous mycobacterial infection. A total of 51% of patients had scoliosis, 11% pectus excavatum, and 9% mitral valve prolapse, all significantly more than reference populations. Stimulated cytokine production was similar to that of healthy control subjects, including the IFN-gamma/IL-12 pathway. CD4(+), CD8(+), B, and natural killer cell numbers were normal. A total of 36% of patients had mutations in the cystic fibrosis transmembrane conductance regulator gene. Patients with PNTM infection are taller and leaner than control subjects, with high rates of scoliosis, pectus excavatum, mitral valve prolapse, and cystic fibrosis transmembrane conductance regulator mutations, but without recognized immune defects.

The pervasiveness of child maltreatment is a global issue, although its impact on the United States is markedly severe. The mortality rate for children four years of age and younger continues to rise annually, with an especially alarming increase present for children with disabilities. Although inservice and preservice educators, including speech-language pathologists, are mandated to report suspected maltreatment, several obstacles appear to hinder their actions. Understanding the factors that predict an individuals' likelihood of reporting malfeasance may help minimize maltreatment occurrence and child mortality. Preservice speech-language pathologists attending accredited programs in the United States were surveyed regarding their attitudes (e.g., commitment, confidence, and concern) toward child maltreatment, including the likelihood they will report alleged abuse and neglect. The study controlled for ethnicity and gender. The results of the survey were analyzed using hierarchical multiple regression and showed the strength of the relationship between the predictor variables (e.g., commitment, confidence, and concern), including the covariates (e.g., gender and ethnicity) on the criterion variable (e.g., reporting practices). The results of the study suggested that the attitudinal dimensions of commitment and concern, including the covariate gender, have a statistically significant contribution to the likelihood that preservice speech-language pathologists will report alleged child maltreatment.

Fallout is the radioactive glass formed from a mixture of vaporized anthropogenic materials with proximate environmental material. These glassy byproducts constitute a compositional record of environmental materials such as soil and vapor precursors that are responsible for chemical heterogeneity in the glasses. The work of this dissertation is to untangle these different sources of compositional heterogeneity, distinguishing low-abundance vaporized anthropogenic-rich material from natural compositions, in order examine soil behavior and chemical evolution during fallout formation. Unfortunately, mixing convolutes the multivariate elemental relationships in these melts due to overlapping element abundances that might distinguish different source materials and chemical behaviors, making bivariate analysis difficult or impossible to interpret. For these reasons historical and modern studies do not thoroughly investigated how soil affects deposition and incorporation of vaporized material in fallout. This work employs a two-step strategy to overcome these challenges and more effectively understand preserved heterogeneity. First (1), three prospective multivariate approaches are compared: classical least squares (CLS) and principal component analysis (PCA), which are commonly employed in the literature, with Multivariate Curve Resolution Alternating Least Squares (MCR-ALS) that is novel in this application. This analysis shows that a closure and equality constrained MCR-ALS approach succeeds in identifying unrecognized precursor components and is a suitable alternative to PCA, or CLS-style approaches in situations distinguished by limited a priori information of precursor compositions. Second (2), the MCR-ALS method is applied to unbiased spatial analyses from 13 Trinity fallout glasses to resolve the contribution and composition of the environmental or vaporized sources. This numerical approach reveals the importance of environmental precursors such as alkali feldspar, calcite, and quartz, to chemical heterogeneity in most glasses. In contrast to previous studies, however, the work resolves a high Al-Si-rich precursor constituting evidence for volatile loss in the melts. Likewise, an Ca- Fe-Mg-rich composition is also resolved, constituting possible evidence for a vapor source term. Correlation of spatial activity distributions, measured by autoradiography, with the compositional precursors from MCR-ALS in clear evidence supporting surface-driven condensation, wherein molten soil components serve as heterogeneous nucleation sites for the vapor source, agglomeration of small melts onto the surface of larger melts, and physical mixing of the vapor source into the interior of molten objects. This is the first report to provide quantitative model-supported evidence for key mechanisms affecting the composition of the vapor phase in a nuclear event and highlighting the impact of local materials on resultant fallout compositions.

A ripple of speculation ran through the audience at the National Press Club in Canberra every time the self-styled Dean of Gonzo Journalism rose from the top table to go to the lavatory before his scheduled address.

For excellence in writing about dogs', the Dog Writers As- sociation of America offers annual awards in journalism. IT has not come to that yet in Australia but suddenly everyone from governments to private enterprise Wants to endow an annual award for Journalistic merit.