A systematic review of the oral and craniofacial manifestations of cri du chat syndrome

Cri du chat syndrome is an autosomal disorder. Because it affects few people in the population it is considered a rare disease, yet it is one of the most common autosomal chromosomal syndromes in humans. It entails pathognomonic alterations that affect the craniofacial and oral anatomy of patients. The aim of this study is to review these craniofacial and oral abnormalities in patients with Cri du chat syndrome. The PubMed Medline database was searched using two different strategies. First, we used “Dentistry” and “Cri du chat” as keywords; second, we used “Cri du chat” and “craniofacial.” Seven articles in which the main orofacial and cranio‐skeletal characteristics of patients with Cri du chat syndrome were described were selected according to the inclusion and exclusion criteria. Cri du Chat syndrome entails pathognomonic characteristics in the craniofacial area (epicanthus, short philtrum, and wide nasal bridge), the oral area (mandibular retrognathism and anterior open bite) and the cranial region (alterations at the cranial base angle and a small upper airway). However, more studies on larger samples are needed to specify the orofacial and craniofacial characteristics of patients with Cri du chat syndrome more accurately. Clin. Anat. 29:555–560, 2016. © 2015 Wiley Periodicals, Inc.     

Periods of rest in fMRI contain individual spontaneous events which are related to slowly fluctuating spontaneous activity

fMRI studies of brain activity at rest study slow (<0.1 Hz) intrinsic fluctuations in the blood‐oxygenation‐level‐dependent (BOLD) signal that are observed in a temporal scale of several minutes. The origin of these fluctuations is not clear but has previously been associated with slow changes in rhythmic neuronal activity resulting from changes in cortical excitability or neuronal synchronization. In this work, we show that individual spontaneous BOLD events occur during rest, in addition to slow fluctuations. Individual spontaneous BOLD events were identified by deconvolving the hemodynamic impulse response function for each time point in the fMRI time series, thus requiring no information on timing or a‐priori spatial information of events. The patterns of activation detected were related to the motor, visual, default‐mode, and dorsal attention networks. The correspondence between spontaneous events and slow fluctuations in these networks was assessed using a sliding window, seed‐correlation analysis, where seed regions were selected based on the individual spontaneous event BOLD activity maps. We showed that the correlation varied considerably over time, peaking at the time of spontaneous events in these networks. By regressing spontaneous events out of the fMRI signal, we showed that both the correlation strength and the power in spectral frequencies <0.1 Hz decreased, indicating that spontaneous activation events contribute to low‐frequency fluctuations observed in resting state networks with fMRI. This work provides new insights into the origin of signals detected in fMRI studies of functional connectivity. Hum Brain Mapp, 2013. © 2012 Wiley Periodicals, Inc.     

Factors linked to disease severity and time to remission in patients with chronic spontaneous urticaria

Biomarkers useful for the evaluation and management of patients with chronic spontaneous urticaria (CSU) are not currently available. A review of various clinical and laboratory markers that have been studied to assess their value for determining the severity or predicting the evolution of disease in adult patients with CSU was carried out. A search of the medical literature on PubMed and MEDLINE including the terms urticaria, chronic urticaria, chronic idiopathic urticaria, CSU, severity, prognosis and treatment was performed. Based on our review of the literature, among the clinical markers studied, higher age at onset, being female, long disease duration and aspirin/NSAID hypersensitivity may be linked to both severe CSU and a long time to spontaneous remission. In addition, a positive autologous serum skin test (ASST) may be associated with severe CSU, and comorbidity of inducible urticaria and concomitant recurrent angio‐oedema may be linked to longer CSU duration. Potential biomarkers of CSU severity and/or duration include basophil numbers and susceptibility to activation, inflammatory markers, markers of activation of the extrinsic coagulation pathway, immunoglobulin E and vitamin D. Although the described markers are promising, further studies on representative and well‐characterized patient populations are needed to determine the value of these clinical and biological markers for predicting the severity and course of disease in patients with CSU.     

Associations of HIV Testing,Sexual Risk and Access to Prevention Among Female Sex Workers in the Dominican Republic

The Caribbean region has one of the highest proportions of HIV in the general female population attributable to sex work. In 2008 (n = 1256) and 2012 (n = 1525) in the Dominican Republic, HIV biological and behavioral surveys were conducted among female sex workers (FSW) in four provinces using respondent driven sampling. Participants were ≥15 years who engaged in intercourse in exchange for money in the past 6 months and living/working in the study province. There were no statistically significant changes in HIV and other infections prevalence from 2008 to 2012, despite ongoing risky sexual practices. HIV testing and receiving results was low in all provinces. FSW in 2012 were more likely to receive HIV testing and results if they participated in HIV related information and education and had regular checkups at health centers. Further investigation is needed to understand barriers to HIV testing and access to prevention services.