Craniofacial bone atrophy in Parry Romberg syndrome demonstrated using a Bayesian hierarchical model

PurposeParry Romberg syndrome (PRS) is a condition characterized by progressive hemifacial atrophy, predominantly affecting the soft tissues. Associated bone retraction is a common clinical feature of PRS but has never been assessed. Here we used 3D imaging and Bayesian statistics in order to demonstrate and quantify bone atrophy in PRS.Materials and methodsTen non-operated patients with PRS (4/10 males) and 12 age-matched controls (7/12 males) were included into the study. The average age at CT-scan was 9.67 ± 4.13 years for PRS patients and 12.5 ± 4.37 years for controls. Soft and hard tissue atrophy levels were quantified using computed tomography scans, based on the distances between surfaces of the affected side and the non-affected contralateral side, both for the skin and the bone. We used a hierarchical Bayesian model with clinical priors in order to assess the relationship between hard and soft tissue atrophies.ResultsPRS patients had significant hard tissue atrophy, and atrophy extents were similar for soft and hard tissues. There was a trend for a correlation between the extent of hard tissue retraction and the extent of soft tissue retraction, and we could not demonstrate that the relationship between hard and soft tissue retractions was different in PRS and controls.ConclusionOur results indicated that bone atrophy was most probably a primary process rather than a phenomenon secondary to soft tissue retraction. We have provided the first assessment of bone atrophy in PRS patients using Bayesian statistics.     

Tcl1 protein functions as an inhibitor of de novo DNA methylation in B-cell chronic lymphocytic leukemia (CLL)

B-cell chronic lymphocytic leukemia (CLL) is the most common human leukemia. Deregulation of the T-cell leukemia/lymphoma 1 oncogene (TCL1) in mouse B cells causes a CD5(+) leukemia similar to aggressive human CLL. To examine the mechanisms by which Tcl1 protein exerts its oncogenic activity in B cells, we performed proteomics experiments to identify its interacting partners. We found that Tcl1 physically interacts with de novo DNA methylthansferases Dnmt3A and Dnmt3B. We further investigated the effects of Tcl1 up-regulation on the enzymatic activity of Dnmt3A and found that Tcl1 overexpression drastically inhibits Dnmt3A function. In addition, B cells from TCL1 transgenic mice showed a significant decrease in DNA methylation compared with WT controls. Similarly, CLL samples with high Tcl1 expression showed a decrease in DNA methylation compared with CLL samples with low Tcl1 expression. Given the previous reports of inactivating mutations of DNMT3A in acute myelogenous leukemia and myelodysplastic syndrome, our results suggest that inhibition of de novo DNA methylation may be a common oncogenic mechanism in leukemogenesis.     

Gastric dysmotility after liquid bolus ingestion in systemic sclerosis: an ultrasonographic study

Gastric involvement appears quite commonly in systemic sclerosis (SSc). The aim of this study was to evaluate gastric wall motility using ultrasonography, a noninvasive method able to track both filling and emptying of fundus and antrum. The study was performed in 20 SSc patients and 20 healthy control subjects. Gastric filling and emptying were evaluated by transabdominal ultrasonography, measuring changes in fundus and antral areas over a 1-h period after ingestion of a liquid bolus (500 ml of mineral water). Areas of both gastric fundus and antrum at basal evaluation were found to be smaller in SSc patients than in healthy controls. Gastric filling was significantly reduced after ingestion of liquid bolus. Gastric emptying was delayed both in fundus and antrum. No significant differences of gastric wall motility have been observed in different subsets of SSc patients. Our findings show that gastric dysmotility is frequent and severe in SSc patients, contributing to the gastrointestinal disturbances which are very common in this disease.     

Psychosocial correlates of sleep quality and architecture in women with metastatic breast cancer

Background

Sleep disturbance is prevalent among women with metastatic breast cancer (MBC). Our study examined the relationship of depression and marital status to sleep assessed over three nights of polysomnography (PSG).

Methods

Women with MBC (N = 103) were recruited; they were predominately white (88.2%) and 57.8 ± 7.7 years of age. Linear regression analyses assessed relationships among depression, marital status, and sleep parameters.

Results

Women with MBC who reported more depressive symptoms had lighter sleep (e.g., stage 1 sleep; P < .05), less slow-wave sleep (SWS) (P < .05), and less rapid eye movement (REM) sleep (P < .05). Single women had less total sleep time (TST) (P < .01), more wake after sleep onset (WASO) (P < .05), worse sleep efficiency (SE) (P < .05), lighter sleep (e.g., stage 1; P < .05), and less REM sleep (P < .05) than married women. Significant interactions indicated that depressed and single women had worse sleep quality than partnered women or those who were not depressed.

Conclusion

Women with MBC and greater symptoms of depression had increased light sleep and reduced SWS and REM sleep, and single women had worse sleep quality and greater light sleep than married counterparts. Marriage was related to improved sleep for women with more depressive symptoms.     

Somatic underpinnings of perceived empathy: The importance of psychotherapy training

Abstract

This study investigated the somatic underpinning of empathy using an interpersonal physiology approach. Thirty-nine dyads were formed by a “pseudo-patient” and a “listener” (a therapist, a psychologist, or a non-therapist). Dyadic physiological concordance in electrodermal responses and listeners' empathy were evaluated during simulations of clinical sessions. A significant positive correlation between empathy as perceived by pseudo-patients and physiological concordance was found, providing empirical evidence of a somatic underpinning of empathy. Moreover, therapists showed higher levels of physiological concordance and empathy, confirming the importance of psychotherapy training in managing clinical interactions.