An uncommon presentation of chronic myeloid leukemia

Annals of Hematology -     

Embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma,and medulloepithelioma share molecular similarity and comprise a single clinicopathological entity

Three histological variants are known within the family of embryonal rosette-forming neuroepithelial brain tumors. These include embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma (EBL), and medulloepithelioma (MEPL). In this study, we performed a comprehensive clinical, pathological, and molecular analysis of 97 cases of these rare brain neoplasms, including genome-wide DNA methylation and copy number profiling of 41 tumors. We identified uniform molecular signatures in all tumors irrespective of histological patterns, indicating that ETANTR, EBL, and MEPL comprise a single biological entity. As such, future WHO classification schemes should consider lumping these variants into a single diagnostic category, such as embryonal tumor with multilayered rosettes (ETMR). We recommend combined LIN28A immunohistochemistry and FISH analysis of the 19q13.42 locus for molecular diagnosis of this tumor category. Recognition of this distinct pediatric brain tumor entity based on the fact that the three histological variants are molecularly and clinically uniform will help to distinguish ETMR from other embryonal CNS tumors and to better understand the biology of these highly aggressive and therapy-resistant pediatric CNS malignancies, possibly leading to alternate treatment strategies.     

Pediatric transplantation in Europe during the COVID-19 pandemic: Early impact on activity and healthcare

The current pandemic SARS-CoV-2 has required an unusual allocation of resources that can negatively impact chronically ill patients and high-complexity procedures. Across the European Reference Network on Pediatric Transplantation (ERN TransplantChild), we conducted a survey to investigate the impact of the COVID-19 outbreak on pediatric transplant activity and healthcare practices in both solid organ transplantation (SOT) and hematopoietic stem cell transplantation (HSCT). The replies of 30 professionals from 18 centers in Europe were collected. Twelve of 18 centers (67%) showed a reduction in their usual transplant activity. Additionally, outpatient visits have been modified and restricted to selected ones, and the use of telemedicine tools has increased. Additionally, a total of 14 COVID-19 pediatric transplanted patients were identified at the time of the survey, including eight transplant recipients and six candidates for transplantation. Only two moderate-severe cases were reported, both in HSCT setting. These survey results demonstrate the limitations in healthcare resources for pediatric transplantation patients during early stages of this pandemic. COVID-19 disease is a major worldwide challenge for the field of pediatric transplantation, where there will be a need for systematic data collection, encouraging regular discussions to address the long-term consequences for pediatric transplantation candidates, recipients, and their families.     

Risk of Respiratory Cancer around a Sewage Plant in Prato,Italy

In this study, the authors evaluated the risk of respiratory cancer related to environmental pollutants among a population that resided near a sewage plant in Prato, Italy. Subjects included lung cancer deaths (1987-1996) and incident cases of lung and laryngeal cancers (1987-1994) among residents of Prato. The authors used the mortality or incidence rates for the entire population of Prato (by gender and by 5-yr age group) to calculate the expected cases in each census unit. Data were analyzed and adjusted for an index of social deprivation (Stone test). Among males, the excess risk of lung cancer mortality decreased as distance from the plant increased for 2 time periods (1987-1996 [p = .008] and 1990-1996 [p = .030]) and for lung cancer incidence during 1987-1994 (p = .011). Similar results were obtained when sewage plant workers were excluded from the analysis. A similar, but not statistically significant, trend was observed among female incident lung cancer cases, as well as among male incident laryngeal cancer cases. Despite methodological limitations common to geographic studies, the results were consistent with those previously published on mortality excesses for lung cancer among plant workers under study. The role of environmental pollutants as a risk for respiratory cancer must be further clarified with additional epidemiological studies and an environmental monitoring program.     

Oxidative stress and uremia

Oxidative stress is a pathogenic element of great importance in uremic patients, with a great impact on their survival. The cause of oxidative stress in patients on hemodialysis is traditionally attributed to the recurrent activation of polymorphonucleate neutrophils and monocytes. The effects of oxidative stress are evident on all biochemical components of biological tissues: lipids, proteins, carbohydrates, and nucleic acids. This study briefly reviews the effects of different dialytic techniques and of kidney transplant on several parameters of oxidative stress. Many different modalities of pharmaceutical intervention are then analyzed, and the clinical evidences reported.     

Deciphering the TLR transcriptome and downstream signaling pathway in cerebrospinal fluid in pediatric meningitis

Inflammation Research - Pediatric meningitis is characterized by a colossal inflammatory response to the pathogen in the central nervous system (CNS). This unabated inflammatory response persists...     

"It's a hair-dryer…No, it's a drill": misidentification-related false recognitions in younger and older adults

Memory for visual objects, although typically highly accurate, can be distorted, especially in older adults. Here we asked whether also erroneous identifications of visual objects subsequently corrected and replaced by a correct identification might induce false recognitions, and whether this is more likely to occur in older people. For this aim a new paradigm was developed. In the first phase, participants performed a visual object identification task with degraded pictures of objects and produced correct and false but subsequently corrected identifications. In the second phase, participants performed a surprise recognition task in which also false identifications were presented. False identifications elicited false recognitions, with a stronger and more reliable effect in elderly participants, suggesting that correcting the initial visual error is not sufficient to correct the memory for the experience. Moreover, misidentification-related false recognitions coexisted in memory along with correct recognitions of correct identifications. These findings are discussed in relation with age-related deficits in memory updating and strategic retrieval.