Richter's syndrome presenting as primary central nervous system lymphoma. Transformation of an identical clone

The development of a central nervous system (CNS) large cell lymphoma in a patient simultaneously diagnosed with chronic lymphocytic leukemia (CLL) is reported. Although differences in phenotypic expression were demonstrated in study of the peripheral blood and CNS disease, identical immunoglobulin gene rearrangements were identified, providing evidence for evolution of two morphologically distinct neoplasms from the same clone. Beyond histologic transformation, acquisition of an aneuploid cell population in the CNS tumor was demonstrated by analysis of DNA content. Isolated parenchymal involvement of the CNS by large cell transformation of CLL has not been previously described; its relationship to CNS lymphoma and Richter's syndrome are reviewed.     

Tubeless Laryngotracheal Surgery in Infants and Children via Jet Ventilation Laryngoscope

We present the first use of tubeless superimposed combined high- and low-frequency jet ventilation (SHFJV) with a jet laryngoscope in laryngotracheal surgery in infants and children. Twenty-eight patients underwent 53 operative procedures. The average age of the patients was 7.3 years. The most common diagnoses were laryngeal papillomatosis and subglottic stenosis. The duration of jet ventilation averaged 33 min. The gas exchange was sufficient in each case. The advantages of SHFJV in the surgery of the laryngotracheal area in infants and children are optimal view at the larynx and trachea, maximum space for the handling, application of the laser without risks, no time limitation, suitability for stenosis, and neither anesthetic nor surgical complications.     

Circulating Fibronectin Controls Tumor Growth

Fibronectin is ubiquitously expressed in the extracellular matrix, and experimental evidence has shown that it modulates blood vessel formation. The relative contribution of local and circulating fibronectin to blood vessel formation in vivo remains unknown despite evidence for unexpected roles of circulating fibronectin in various diseases. Using transgenic mouse models, we established that circulating fibronectin facilitates the growth of bone metastases by enhancing blood vessel formation and maturation. This effect is more relevant than that of fibronectin produced by endothelial cells and pericytes, which only exert a small additive effect on vessel maturation. Circulating fibronectin enhances its local production in tumors through a positive feedback loop and increases the amount of vascular endothelial growth factor (VEGF) retained in the matrix. Both fibronectin and VEGF then cooperate to stimulate blood vessel formation. Fibronectin content in the tumor correlates with the number of blood vessels and tumor growth in the mouse models. Consistent with these results, examination of three separate arrays from patients with breast and prostate cancers revealed that a high staining intensity for fibronectin in tumors is associated with increased mortality. These results establish that circulating fibronectin modulates blood vessel formation and tumor growth by modifying the amount of and the response to VEGF. Furthermore, determination of the fibronectin content can serve as a prognostic biomarker for breast and prostate cancers and possibly other cancers.     

Rapid antidepressant effect of S-ketamine in schizophrenia

Rapid anti-suicidal and antidepressant effects of ketamine have repeatedly been confirmed in unipolar and bipolar depression. Although meaningful antidepressant efficacy of ketamine has also been shown in depressed patients with a history of psychotic symptoms, its administration in psychotic disorders has largely been neglected due to its potential to exacerbate dissociative or psychotic symptoms. Presenting a case of a young female inpatient suffering from schizophrenia with a severe post-psychotic depression, we demonstrate a robust anti-suicidal and antidepressant effect of S-ketamine infusions administered thrice weekly for 3 weeks in total. Importantly, no relevant psychotic or dissociative symptoms occurred during the whole augmentation treatment period leading to a sustained remission of depressive symptoms and suicidality. Our safe and effective experience with intravenous S-ketamine might encourage researchers and clinicians to widen its administration range beyond the diagnosis of depression to enrich the current knowledge of ketamine effects in psychotic disorders.     

Effects of dopamine and opioid receptor antagonism on the neural processing of social and nonsocial rewards

Rewards are a broad category of stimuli inducing approach behavior to aid survival. Extensive evidence from animal research has shown that wanting (the motivation to pursue a reward) and liking (the pleasure associated with its consumption) are mostly regulated by dopaminergic and opioidergic activity in dedicated brain areas. However, less is known about the neuroanatomy of dopaminergic and opioidergic regulation of reward processing in humans, especially when considering different types of rewards (i.e., social and nonsocial). To fill this gap of knowledge, we combined dopaminergic and opioidergic antagonism (via amisulpride and naltrexone administration) with functional neuroimaging to investigate the neurochemical and neuroanatomical bases of wanting and liking of matched nonsocial (food) and social (interpersonal touch) rewards, using a randomized, between-subject, placebo-controlled, double-blind design. While no drug effect was observed at the behavioral level, brain activity was modulated by the administered compounds. In particular, opioid antagonism, compared to placebo, reduced activity in the medial orbitofrontal cortex during consumption of the most valued social and nonsocial rewards. Dopamine antagonism, however, had no clear effects on brain activity in response to reward anticipation. These findings provide insights into the neurobiology of human reward processing and suggest a similar opioidergic regulation of the neural responses to social and nonsocial reward consumption.     

Cross-informant agreement between adolescents with myelomeningocele and their parents

The development of self-management skills by adolescents with myelomeningocele is an ongoing process. Previous studies lack consensus about what data can be accurately obtained from adolescents. This cross-sectional study using a convenience sample of 66 adolescent-parent dyads evaluated whether adolescents with myelomeningocele and their parents are interchangeable reporters of data. Adolescents' ages ranged from 12 to 21 years with a mean of 16 years 2 months (SD 2y 8mo); 38 were female, 28 were male; 30% had thoracic lesions, 32% had lumbar lesions, 15% had lumbosacral lesions, 23% had sacral lesions, and 85% had ventriculoperitoneal shunts. In this analysis, participants reported activities (decision-making, household responsibility, and friendship activities) and select outcomes (functional status, self-management, and social competence) similarly. However, differences emerged in reports of beliefs (adolescent future expectations, family variables) and select developmental competencies (school, job, athletic, behavioral, attractiveness, and romantic appeal). Analysis using t-test and interclass correlations supported a pattern of adolescent-parent agreement in areas of observable behavior and differences in more subjective domains such as perception of developmental competencies.