Late Onset of Chronic Granulomatous Disease Revealed by Paecilomyces lilacinus Cutaneous Infection

Chronic granulomatous disease (CGD) is an inherited immunodeficiency due to defective leukocyte NADPH responsible for recurrent infections and aberrant inflammation. Mutations in the CYBB gene are responsible for the X-linked CGD and account for approximately 70% of the cases. CGD is diagnosed during childhood in males. Female carriers may have biased X-inactivation and may present with clinical manifestations depending on the level of residual NADPH oxidase activity. We report the case of a previously asymptomatic female carrier who was diagnosed at age 67 with a skin infection with the rare fungus Paecilomyces lilacinus as the first manifestation of CGD. Dihydrorhodamine 123 (DHR) activity was below 10%. Next-generation sequencing (NGS) revealed mutations in DNMT3A, ASXL1, and STAG2 suggesting that clonal hematopoiesis could be responsible for a progressive loss of NADPH oxidase activity and the late onset of X-linked CGD in this patient. Long-term follow-up of asymptomatic carrier women seems to be essential after 50 years old.

     

Lung cancer incidence in young women vs. young men: A systematic analysis in 40 countries

Previous studies have reported converging lung cancer rates between sexes. We examine lung cancer incidence rates in young women vs. young men in 40 countries across five continents. Lung and bronchial cancer cases by 5-year age group (ages 30–64) and 5-year calendar period (1993–2012) were extracted from Cancer Incidence in Five Continents. Female-to-male incidence rate ratios (IRRs) and 95% confidence intervals (95%CIs) were calculated by age group and birth cohort. Among men, age-specific lung cancer incidence rates generally decreased in all countries, while in women the rates varied across countries with the trends in most countries stable or declining, albeit at a slower pace compared to those in men. As a result, the female-to-male IRRs increased among recent birth cohorts, with IRRs significantly greater than unity in Canada, Denmark, Germany, New Zealand, the Netherlands and the United States. For example, the IRRs in ages 45–49 year in the Netherlands increased from 0.7 (95% CI: 0.6–0.8) to 1.5 (95% CI: 1.4–1.7) in those born circa 1948 and 1963, respectively. Similar patterns, though nonsignificant, were found in 23 additional countries. These crossovers were largely driven by increasing adenocarcinoma incidence rates in women. For those countries with historical smoking data, smoking prevalence in women approached, but rarely exceeded, those of men. In conclusion, the emerging higher lung cancer incidence rates in young women compared to young men is widespread and not fully explained by sex differences in smoking patterns. Future studies are needed to identify reasons for the elevated incidence of lung cancer among young women.     

Effectiveness of High Power Ultrasound for Surimi-Based Preparation of Lionfish (Pterois volitans) Patties by Textural,Sensory and Shape Preference

Lionfish is an invasive and predatory species with low local consumption due to a lack of culinary knowledge of its preparation. A new way to prepare lionfish was proposed to increase variety and provide added value. The aim of this study was to evaluate the effect of high power ultrasound application on the textural properties measured by the texture profile analysis, sensory attributes, and shape preference of surimi patties (rounded and square) made from lionfish. Patties were prepared using lionfish surimi processed with ultrasound (37 kHz; 150 W), sodium citrate (0.3%), sucrose (1%), and salt (2%). Ultrasound exposure increased the hardness of the patties by 35.1%, decreased cohesiveness by 89.7%, and decreased the chewiness by 49.1%. Results show that high power ultrasound showed potential as an environmentally friendly technology to create surimi as a base for patties with sensory and textural appropriateness, increasing the potential culinary applications of lionfish.