The prevalence of priapism in children and adolescents with sickle cell disease in Brazil

To evaluate priapism rates in individuals <18 years of age with sickle cell disease (SCD) at a referral center. An evaluation was made of 599 consecutive male patients with SCD, separated according to type of hemoglobinopathy (HbSS, HbSC and HbS-β-thalassemia). Age at first episode and number of episodes were recorded. Cases of sickle cell trait were excluded. Mean age was similar in all groups. Overall, priapism occurred in 3.6 % of patients (5.6 % of those with HbSS and 1.1 % of those with HbSC; P = 0.01). In HbSS patients, the prevalence rate of priapism was from 3.5 (CI 95 % 0.94-13.4) when compared with patients with HbSC. No patient with β-thalassemia had priapism. Mean follow-up was 39.7 months (range 1-202 months). Since 91 % of patients with priapism had HbSS, this group was evaluated separately, revealing a rate of priapism of 1.6 % in patients <10 years and 8.3 % in those ≥ 10 years of age (P = 0.002). Regarding priapism in HbSS patients ≥ 10 years (8.3 %) when compared with patients <10 years (1.6 %), the prevalence rate was from 3.3 (CI 95 % 1.1-9.5). Duration of follow-up was not correlated with priapism (P = 0.774). Forty-seven patients were lost to follow-up. Telephone contact was successful with 14/22 patients with priapism, 50 % of whom had required hospital treatment. Most episodes (86 %) occurred at night, always during sleep. Medical interventions were required in 13 cases as follows: intravenous hydration (n = 4), corpora cavernosa puncture and drainage (n = 7) and corpus cavernosum-corpus spongiosum shunts (n = 2). The prevalence of priapism in children <18 years of age with SCD was 3.6 %, lower than previously reported. Prevalence was higher in HbSS patients, increasing in patients >10 years of age. Most episodes occurred at night and half of the patients required some form of urological procedure.     

《英国非酒精性脂肪性肝炎肝移植指南》导读

非酒精性脂肪性肝炎（non—alcoholicsteatohepatitis,NASH）现已成为肝移植愈来愈重要的基础肝病。鉴于晚期NASH患者常并存多种影响肝移植转归的临床问题,而至今尚无针对NASH患者进行肝移植的评估和治疗指南,为此英国移植学会（British Transplant Society,BTS）邀请相关专家制定了指南,以指导肝移植前后NASH患者的处理。     

Microsurgical reconstruction of maxillectomy defects: experience of 24 cases

Introduction

The defect complexity and reconstructive options make the maxillary reconstruction a controversial theme and in a constant debate. The maxilla is a fundamental aesthetic and functional structure of the face. Microsurgical vascularized flaps replaced the usage of prosthetic material and pedicled flaps as a “gold standard” for the reconstruction of complex defects following maxillectomy.

Methods

The authors report their experience of 24 maxillectomies with immediate microsurgical reconstruction, performed by the senior author (H.C.) between 1998 and 2011. They evaluate and classify the defects and the reconstructive options according to the classification system as proposed by Cordeiro and Santamaria in 2000, by a patient questionnaire and post-operative surgeon follow-up for the functional (diet, speech, and vision) and aesthetic end results.

Results

There were no flap failures. The main etiology was squamous cell carcinoma and the most used flap was rectus abdominis free flap. Classes I and II were responsible for the cases in which the reconstructive algorithm was not followed. Most patients responded as having a normal diet, a nearly normal speech and unaffected vision. In a score of 1 to 5, the mean score in esthetic given by the patient was 3.62, while the mean score given by the surgeons was 4.13.

Conclusions

Microsurgical reconstruction of maxillectomy defects with free flaps is the best reconstructive option, being the osteomyocutaneous flaps as the gold standard. Although with limited rehabilitation, good functional and aesthetic results are to be expected with myocutaneous flaps. The existence of an algorithm facilitates the classification and systematization of maxillary reconstruction. However, due to defect complexity and large number of reconstructive options, a perfect solution does not exist. The individual assessment of the patient and the defect always provides the best method for the reconstructive planning, mainly when choosing free flaps. Level of Evidence: Level IV, therapeutic study.     

Extensive Cytoreductive Surgery for Appendiceal Carcinomatosis: Morbidity, Mortality, and Survival

Background

Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemoperfusion (HIPEC) are frequently used to treat appendiceal carcinomatosis. Some patients require multivisceral resection because of the volume of disease. It is unclear whether extent of CRS impacts survival in appendiceal carcinomatosis.

Methods

We analyzed 282 patients undergoing attempted CRS/HIPEC for appendiceal carcinomatosis. Patients were defined as having undergone Extensive CRS (n = 60) if they had >3 organ resections or >2 anastomoses; a subgroup of Extreme CRS patients (n = 10) had ≥5 organ resections and ≥3 anastomoses. Kaplan–Meier survival curves and multivariate Cox-regression models were used to identify prognostic factors affecting outcomes.

Results

Relative to the comparison group, patients undergoing Extensive CRS had a higher median peritoneal carcinomatosis index, operative duration, blood loss, and length of stay. No difference in completeness of cytoreduction, severe morbidity, or 60-day mortality was evident. Subgroup analysis of 10 patients undergoing extreme CRS likewise revealed no increase in severe morbidity or mortality. Median progression-free (PFS) and overall survival (OS) were 23.5 and 74 months in the comparison group; 18.5 (p = 0.086) and 51 (p = 0.85) months in the Extensive CRS group; and 40 months and not reached in the Extreme CRS subgroup. In a multivariable analysis, extent of CRS was not independently associated with PFS or OS.

Conclusions

Extensive CRS is associated with greater OR time, blood loss, and length of stay, but is not associated with higher morbidity, mortality, or inferior oncologic outcomes in patients with appendiceal carcinomatosis.     

Effect of Intrathecal Baclofen Concentration on Spasticity Control: Case Series

Background/Objective: Intrathecal baclofen (ITB) has been shown to be an effective treatment for severe spasticity of spinal or cerebral origin. Although most patients respond well to an ITB trial, there are often difficulties in achieving and/or maintaining such effectiveness with ITB pump treatment. There are few published guidelines for dosing efficacy and no studies looking at the effect of concentration of ITB on spasticity management.

Methods: Case series of 3 adults with severe spasticity treated with ITB pump: a 44-year-old man with C7 tetraplegia using a 40-mL Medtronic SynchroMed II pump with 500-μg/mL concentration; a 35-year-old woman with traumatic brain injury with right spastic hemiplegia using a 18-mL Medtronic SynchroMed EL pump with 2,000-μg/mL concentration; and a 43-year-old woman with spastic diplegic cerebral palsy using a 40-mL Medtronic SynchroMed II pump with 2,000-μg/mL concentration.

Results: After reducing ITB concentrations in the pump, either as part of a standard protocol for dye study to assess the integrity of pump and catheter system or secondary to plateau in therapeutic efficacy, patients experienced temporary, significant reduction in spasticity based on range of motion, Modified Ashworth scores, and verbal feedback.

Conclusions: Decreasing the concentration of ITB seems to affect spasticity control. Further research in this area is needed for those patients with refractory spasticity to optimize efficacy of ITB therapy.     

MC3T3‐E1 Cells Behavior on Surfaces Bombarded by Argon Ions in Planar Cathode Discharge

To evaluate the effect of topography in nanoscale, titanium surfaces were bombarded by argon ions (a chemically inert gas), in an atmosphere of plasma. The effects of surface parameters on morphology, adhesion, proliferation, and MC3T3‐E1 preosteoblasts differentiation were analyzed. Nontreated (smooth) surfaces were used as a control. The levels of average roughness (Ra) observed in bombarded and smooth titanium surfaces were of 95 and 14 nm, respectively. The wettability increased on treated surfaces. The number of attached cells (30 and 60 min) was significantly higher on the bombarded surface. The cell proliferation after 3 and 7 days was also significantly higher on the ion‐bombarded surface. In addition, the ALP activity and expression of osteocalcin were higher in cells grown on the treated surface. The results showed that bombardment with argon ions increased the roughness and the wettability of the Ti surface, promoting a significant increase in the adhesion, proliferation, and differentiation of preosteoblasts.     

A lasting post-stimulus activation on dorsolateral prefrontal cortex is produced when processing valence and arousal in visual affective stimuli

This paper introduces a new paradigm in the study of emotional processes through functional neuroimaging. We study whether the valence and arousal of visual stimuli influence neuroimaging of the evoked hemodynamic changes. Using functional near-infrared spectroscopy (fNIRS), we investigate evoked-cerebral blood oxygenation (CBO) changes in dorsolateral prefrontal cortex (DLPFC) during direct exposure to different emotion-eliciting stimuli ('on' period), and during the period directly following stimulus cessation ('off' period). We hypothesize that the evoked-CBO, rather than return to baseline after stimulus cessation, would show either overshoot or undershoot. The study includes 30 healthy subjects and a total of 9 stimuli, which consist of video-clips with different emotional content. The total sample of trials studied (270) is classified according to the valence and arousal ratings given by the subjects. Results show a more robust activation in DLPFC during the 'off' period than during the 'on' period, depending on the subjective degree of arousal given to the stimulus. Our findings provide the first fNIRS evidence showing that an increment in subjective arousal leads to activation in DLPFC which persists after stimulus cessation and this does not occur with non-arousing stimuli. Neuroimaging studies must consider the duration and affective dimensions of the stimulus as well as the duration of the scanning to specify how much of the recorded response is analyzed. Not accounting for this difference may contribute to confusion in the data interpretation.     

In or out? Methodological considerations for including and excluding findings from a meta‐analysis of predictors of antiretroviral adherence in HIV‐positive women

AIM: This paper is a discussion detailing the decisions concerning whether to include or exclude findings from a meta-analysis of report of quantitative studies of antiretroviral adherence in HIV-positive women. BACKGROUND: Publication constraints and the absence of reflexivity as a criterion for validity in, and reporting of, quantitative research preclude detailing the many judgements made in the course of a meta-analysis. Yet, such an accounting would assist researchers better to address the unique challenges to meta-analysis presented by the bodies of research they have targeted for review, and to show the subjectivity, albeit disciplined, that characterizes the meta-analytic process. DATA SOURCES: Data were 29 published and unpublished studies on antiretroviral adherence in HIV-positive women of any race/ethnicity, class, or nationality living in the United States of America. The studies were retrieved between June 2005 and January 2006 using 40 databases. REVIEW METHODS: Findings were included if they met the statistical assumptions of meta-analysis, including: (1) normal distribution of observations; (2) homogeneity of variances; and (3) independence of observations. RESULTS: Relevant studies and findings were excluded because of issues related to differences in study design, different operationalizations of dependent and independent variables, multiple cuts from common longitudinal data sets, and presentation of unadjusted and adjusted findings. These reasons led to the exclusion of 73% of unadjusted relationships and 87% of adjusted relationships from our data set, leaving few findings to synthesize. CONCLUSION: Decisions made during research synthesis studies may result in more information losses than gains, thereby obliging researchers to find ways to preserve findings that are potentially valuable for practice.     

Self-inflicted oral trauma in a baby with Moebius syndrome

Moebius syndrome (MS) is a rare disorder mainly characterized by bi- or unilateral palsy of the facial and abducens nerves. Among the various orofacial characteristics related to this syndrome, the following are pointed out, difficulty with sucking, tongue malformation, mandibular hypoplasia, saliva drooling, arched palate and micrognathia. In spite of the diversity of oral features described for these patients, self-inflicted oral trauma has not yet been reported. Therefore, the purposes of the present report were to describe an unusual case of a baby with Moebius syndrome who had a parafunctional habit that resulted in a persistent traumatic lesion of the lower lip, and discuss the treatment performed.