Reversible posterior leukoencephalopathy syndrome: report of 2 simultaneous cases in children

Reversible posterior leukoencephalopathy syndrome (RPLS) is a rare complication of cancer chemotherapy. We have recently observed two cases occurred simultaneously in children receiving different chemotherapy regimens, for hepatoblastoma and acute lymphoblastic leukaemia, respectively. Both children presented with altered mental status, severe visual disturbances, headache, seizures, backpain and hypertension. Magnetic resonance imaging showed cortical and subcortical lesions especially in the occipital and parietal regions, strongly consistent with RPLS. Both patients completely recovered from their neuropsychologic deficits in about ten days only with anticonvulsant and antihypertensive therapy, and chemotherapy regimen was promptly restarted according to the planned protocol, without any neuropsychological sequela. A mild left midriasis was the only neurologic defect that persisted in the patient with acute lymphoblastic leukemia.     

Emotional and behavioral problems after pediatric liver transplantation: a quantitative assessment

Several uncertainties regarding psychological problems in children who underwent liver transplantation and the need to differentiate these disturbances from those related to the underlying previous chronic liver disease itself exist. This background triggered the present pilot study to investigate, using quantitative assessment methods, the incidence and the type of emotional and behavioral disturbances after liver transplantation. Sixteen liver transplant recipients (aged 5.7-14.4 yr) and 12 age-matched controls with stable chronic liver disease were assessed through the parent report form of Child Behavior Checklist/ 4-18. The mean time elapsed since transplantation was 8.1 yr. No patient or family had received psychological support during chronic liver disease or at any phase of the transplantation process. Transplanted children scored within borderline range for Internalizing and Total Behavioral Problems and within pathological range for Competences, except for the Activity Scale. Transplanted children showed more Total Behavioral (p = 0.005) and Externalizing Problems (p = 0.0005) than controls. Both groups scored within the pathological range for Total Competences with no significant differences between the two groups. Our findings suggest that in the absence of support programs a psychological risk does exist for a long period of time, after transplantation. Regarding Total Behavioral Problems and Externalizing Problems, this risk is higher than in children with chronic liver disease.     

Early-Onset Ventilator-Associated Pneumonia in Severe Traumatic Brain Injury: is There a Relationship with Prehospital Airway Management?

BackgroundPrehospital airway management in severe traumatic brain injury (TBI) is widely recommended by international guidelines for the management of trauma. Early-onset ventilator-associated pneumonia (EOVAP) is a common occurrence in this population and can worsen mortality and functional outcome.ObjectivesIn this retrospective observational study, we aimed to evaluate the association between different prehospital airway management variables and the occurrence of EOVAP. Secondarily we evaluated the correlation between EOVAP and mortality and neurological outcome.MethodsThe study retrospectively evaluated 223 patients admitted from 2010 to 2017 in our trauma intensive care unit for severe TBI. The population was divided into three groups on the basis of the airway management technique adopted (bag mask ventilation, laryngeal tube, orotracheal intubation). Uni- and multivariate logistic regression analyses were performed using the occurrence of EOVAP as the dependent variable, to investigate potential associations with prehospital airway management.ResultsA total of 131 episodes (58.7%) of EOVAP were registered in the study population (223 patients). Laryngeal tube and orotracheal intubation were used in patients with significantly lower Glasgow Coma Scale score on scene and a higher Face Abbreviated Injury Scale; advanced airway management significantly increased the total rescue time. The prehospital airway management technique adopted, prehospital type of sedation or use of muscle relaxants, type of transport, and rescue times were not associated with the occurrence of EOVAP.ConclusionsPrehospital airway management does not have a significant impact on the occurrence of EOVAP in severe TBI patients. Similarly, it does not have a significant impact on mortality or long-term neurological outcome despite increasing duration of mechanical ventilation, intensive care unit, and hospital stay.     

Next generation sequencing in sporadic retinoblastoma patients reveals somatic mosaicism

In about 50% of sporadic cases of retinoblastoma, no constitutive RB1 mutations are detected by conventional methods. However, recent research suggests that, at least in some of these cases, there is somatic mosaicism with respect to RB1 normal and mutant alleles. The increased availability of next generation sequencing improves our ability to detect the exact percentage of patients with mosaicism. Using this technology, we re-tested a series of 40 patients with sporadic retinoblastoma: 10 of them had been previously classified as constitutional heterozygotes, whereas in 30 no RB1 mutations had been found in lymphocytes. In 3 of these 30 patients, we have now identified low-level mosaic variants, varying in frequency between 8 and 24%. In 7 out of the 10 cases previously classified as heterozygous from testing blood cells, we were able to test additional tissues (ocular tissues, urine and/or oral mucosa): in three of them, next generation sequencing has revealed mosaicism. Present results thus confirm that a significant fraction (6/40; 15%) of sporadic retinoblastoma cases are due to postzygotic events and that deep sequencing is an efficient method to unambiguously distinguish mosaics. Re-testing of retinoblastoma patients through next generation sequencing can thus provide new information that may have important implications with respect to genetic counseling and family care.     

Beyond a “Woman’s Problem”: The Role of Relationship Processes in Female Genital Pain

Female genital pain is a prevalent condition that can disrupt the psychosexual and relational well-being of affected women and their romantic partners. Despite the intimate context in which the pain can be elicited (i.e., during sexual intercourse), interpersonal correlates of genital pain and sexuality have not been widely studied in comparison to other psychosocial factors. This review describes several prevailing theoretical models explaining the role of the partner in female genital pain: the operant learning model, cognitive-behavioral and communal coping models, and intimacy models. The review includes a discussion of empirical research on the interpersonal and partner correlates of female genital pain and the impact of genital pain on partners’ psychosexual adjustment. Together, this research highlights a potential reciprocal interaction between both partners’ experiences of female genital pain. The direction of future theoretical, methodological, and clinical research is discussed with regard to the potential to enhance understanding of the highly interpersonal context of female genital pain.     

miRNA and long non-coding RNA transcriptional expression in hepatocellular carcinoma cell line-secreted extracellular vesicles

Clinical and Experimental Medicine - Extracellular vesicles (EVs) are membrane-released vesicles acting as transporters of proteins, lipids and short/long non-coding RNA (miRNAs and lncRNAs). They...