Connectivity measures suggest a sub-cortical generator of myoclonus in Angelman syndrome

ObjectiveThe clinical and neurophysiological characteristics of myoclonus in Angelman syndrome (AS) have been evaluated in single case or small cohorts, with contrasting results. We evaluated the features of myoclonus in a wide cohort of AS patients.MethodsWe performed polygraphic EEG-EMG recording in 24 patients with genetically confirmed AS and myoclonus. Neurophysiological investigations included jerk-locked back-averaging (JLBA), cortico-muscular coherence (CMC) and generalised partial directed coherence (GPDC). CMC and GPDC analyses were compared to those obtained from 10 healthy controls (HC).ResultsTwenty-four patients (aged 3–35 years, median 20) were evaluated. Sequences of quasi-continuous rhythmic jerks mostly occurred at alpha frequency or just below (mean 8.4 ± 1.4 Hz), without EEG correlate. JLBA did not show any clear transient preceding the jerks. CMC showed bilateral over-threshold CMC in alpha band that was prominent on the contralateral hemisphere in the patient group as compared to HC group. GPDC showed a significantly higher alpha outflow from both hemispheres toward activated muscles in the patient group, and a significantly higher beta outflow from contralateral hemisphere in the HC group.ConclusionsThese neurophysiological findings suggest a subcortical generator of myoclonus in AS.SignificanceMyoclonus in AS has not a cortical origin as previously hypothesised.     

PDCA循环在消化道肿瘤伴糖尿病患者营养全程管理中的效果观察

目的 探讨PDCA 循环在消化道肿瘤伴糖尿病患者营养全程管理中的效果观察。方法 运用PDCA循环对80例不同程度营养不良的消化道肿瘤伴糖尿病患者实施营养全程管理,比较干预前后患者的体重、BMI指数、糖化血红蛋白、血红蛋白、总蛋白、白蛋白及球蛋白水平。结果 患者血红蛋白、总蛋白、白蛋白及球蛋白水平均有提高,比较差异均有统计学意义(P＜0.05)。结论 PDCA循环管理在消化道肿瘤伴糖尿病患者营养全程管理中有较大的优势, 值得推广。     

Surgical Technique Notes of Arterial Vascular Reconstruction During Kidney Transplantation: Personal Experience and Literature Review

Background

Arterial vascular anomalies in patients undergoing kidney transplantation (KT) are correlated with a higher incidence of early surgical complications, potentially causing graft loss. Arterial reconstruction allows patients to overcome these surgical challenges, thus minimizing the risk of poor outcomes. The aim of the present study is to retrospectively investigate the safety and effectiveness of the multiple arterial reconstruction technique with a Teflon patch in case of an unavailable aortic patch: to do so, surgical complications, graft function, and patient survival were evaluated.

Family-centered Care for Sick Newborns: A Thumbnail View

Family-centered care (FCC) for sick newborns is emerging as a paradigmatic shift in the practice of facility-based newborn care. It seeks to transforming a provider-centered model into a client-centered one and thus build a new therapeutic alliance. FCC is the cornerstone of continuum of care, imparting caregiving competencies to parents/caregivers both within institutions as well as after the discharge. This has potential gains for the newborn, family members, and facility-level staff. The initial model piloted in tertiary-care settings is now undergoing translation at five sites across the country; the outcomes are keenly awaited.     

中国精神分裂症患者不同家庭干预措施效果比较的meta分析

目的对中国精神分裂症患者采取家庭干预的研究文献进行综合回顾和系统评价, 比较不同条件下家庭干预效果的差异。方法在中国知网、维普、万方、中国生物医学文献数据库四大中文数据库及OVID Medline、Science Direct、Web of Science、EBSCO四大英文数据库中, 检索各数据库建库至2015年1月为止使用社会功能缺陷筛选量表(SDSS)、简明精神病(科)量表(BPRS)、阳性与阴性症状量表(PANSS)研究中国精神分裂症患者家庭干预效果的文献, 以标准化加权均数差( SMD)作为效应量, 采用meta分析比较不同干预时间、不同干预类型、对不同病程和不同严重程度的精神分裂症患者的家庭干预效果差异。 结果共纳入57篇符合标准的文献。SDSS、PANSS分析结果显示:① 干预时间越长干预效果越好( P < 0.0001、 P=0.0025);② 单独家庭干预比多个家庭合并单独家庭干预的效果更明显( P < 0.0001、 P=0.0131);③ 干预对于病情较重患者效果较好( P < 0.0001、 P=0.0280)。SDSS量表还显示家庭干预对于病程短的患者效果更好( P < 0.0001)。 结论家庭干预更适合病程较短的精神分裂症患者, 干预应实施较长时间; 单独家庭干预更有利于患者阴性症状的改善和社会功能的康复, 且对于病情较轻患者的阴性症状改善效果更好。     

Effect of lower leg compression during cesarean section on post-spinal hypotension and neonatal hemodynamic parameters: nonrandomized controlled clinical trial

ObjectivesThis study aimed to determine the effect of lower leg compression during cesarean section (CS) on post-spinal hypotension (PSH) and neonatal hemodynamic parameters.MethodsThis study is a nonrandomized controlled clinical trial conducted in the cesarean delivery unit of the National Medical institute, Damanhour, Egypt. The sample included 120 parturients (60 intervention and 60 control). The researchers developed three tools for data collection: sociodemographic data and reproductive history interview schedule, electronic monitoring of maternal hemodynamic parameters, and neonatal hemodynamic assessment sheet. All parturients received ordinary pre-operative care. For the intervention group, a long elastic stocking (ordinary pressure 20–30 mmHg, 1 mmHg = 0.133 kPa) was applied on both legs during cesarean section. The control group received the same care without the elastic stocking.ResultsSystolic blood pressure, diastolic blood pressure, and mean arterial blood pressure were significantly higher in the intervention group throughout the entire operation period except in the last 5–15 min. Heart rate was significantly lower in the intervention group. Only 13.3% of the intervention group took ephedrine compared with 45% of the control group. Apgar score was higher among neonates of intervention group compared with the control group at 1 min. Neonatal acidosis was significantly higher in the control group than in the contral group.ConclusionLower leg compression technique can effectively reduce PSH and neonatal acidosis.     

Effectiveness of the 10-valent pneumococcal conjugate vaccine against radiographic pneumonia among children in rural Bangladesh: A case-control study

BackgroundPneumococcal conjugate vaccine (PCV) effectiveness against radiographic pneumonia in South Asia is unknown. Bangladesh introduced PCV10 in 2015 using a three dose primary series (3 + 0). We sought to measure PCV10 effectiveness for two or more vaccine doses on radiographic pneumonia among vaccine-eligible children in rural Bangladesh.MethodsWe conducted a matched case-control study over two years from 2015 to 2017 using clinic and community controls in three subdistricts of Sylhet, Bangladesh. Cases were vaccine eligible 3–35 month olds at Upazila Health Complex outpatient clinics with World Health Organization-defined radiographic primary endpoint pneumonia (radiographic pneumonia). Clinic controls were matched to cases within a one week time window by age, sex, and clinic and had an illness unlikely to be Streptococcus pneumoniae; community controls were healthy and similarly matched within a one week time window by age and sex, and distance from the clinic. We estimated adjusted vaccine effectiveness (aVE) using conditional logistic regression.ResultsWe matched 1262 cases with 2707 clinic and 2461 community controls. Overall, aVE using clinic controls was 21.4% (95% confidence interval, −0.2%, 38.4%) for ≥2 PCV10 doses and among 3–11 month olds was 47.3% (10.5%, 69.0%) for three doses. aVE increased with higher numbers of doses in clinic control sets (p = 0.007). In contrast, aVE using community controls was 7.6% (95% confidence interval, −22.2%, 30.0%) for ≥2 doses. We found vaccine introduction in the study area faster and less variable than expected with 75% coverage on average, which reduced power. Information bias may also have affected community controls.ConclusionsClinic control analyses show PCV10 prevented radiographic pneumonia in Bangladesh, especially among younger children receiving three doses. While both analyses were underpowered, community control enrollment – compared to clinic controls – was more difficult in a complex, pluralistic healthcare system. Future studies in comparable settings may consider alternative study designs.     

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: Diagnoses and Surgical Results in 12 Pediatric Patients

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital condition. It responds well to early diagnosis and treatment, but otherwise the prognosis is poor. We present our case series of 12 patients (mean age, 2 ± 2.58 yr; age range, 2 mo–8 yr), emphasizing the diagnostic process and discussing our surgical results. The diagnosis of ALCAPA should be suspected in infants who have dilated cardiomyopathy with electrocardiographic changes that suggest ischemia, and in older children who have isolated mitral regurgitation. When clinical suspicion is high, the results of 2-dimensional echocardiography combined with color-flow Doppler studies in expert hands can establish the diagnosis, thus avoiding angiography in critically ill infants. The treatment of choice in our patients was transfer and reimplantation of the left coronary artery onto the ascending aorta. There were 2 deaths: both were infants in extremis who underwent emergency surgery. An older child with severe ventricular dysfunction was given mechanical ventricular assistance and then heart transplantation. As of this report, all 10 survivors remained well and asymptomatic.