胎儿无脾综合征的产前超声诊断

目的 探讨胎儿无脾综合征的声像图表现.方法 在21751例20～40周胎儿系统超声检查中,超声检查确诊4例无脾综合征,回顾性分析4例无脾综合征胎儿的声像图表现.结果 胎儿无脾综合征声像图特征为:①多伴有复杂而严重的心脏及大血管畸形;②内脏转位;③脾缺如.结论 胎儿系统超声检查发现复杂心血管畸形时,应常规观察胎儿脾及内脏位置,以便早期诊断胎儿无脾综合征.     

Disposable laryngeal tube suction: standard insertion technique versus two modified insertion techniques for patients with a simulated difficult airway

Objective

The disposable laryngeal tube suction (LTS-D) is a supraglottic airway device that can be used as an alternative to tracheal tube to provide ventilation. We tested the hypothesis that, with a frontal jaw thrust insertion technique (FIT/JT), the rate of correct placement attempts in patients with a simulated difficult airway by means of a rigid cervical immobilization collar could be significantly increased compared to the standard insertion technique (SIT) recommended by the manufacturer.

Methods

70 adult patients undergoing trauma surgery under general anaesthesia had an LTS-D inserted, randomly assigned to the SIT or FIT/JT. In the FIT/JT, the operator was standing in front of the patient's head, and forced chin lift to create sufficient retropharyngeal space was performed. The rate of successful tube placements within 180 s and with a maximum of two attempts was the main outcome variable. To distinguish between the effects of the frontal approach and the jaw thrust manoeuvre, a third group was studied after completion of the SIT and FIT/JT groups. The standard insertion technique, but with a jaw thrust manoeuvre (SIT/JT), was employed in another 35 consecutive patients.

Results

Overall placement success was 49% (SIT, 17/35 patients, P < 0.001), 91% (SIT/JT, 32/35 patients) and 100% (FIT/JT). The time required for successful insertion was shortest in the FIT/JT group (23 ± 6 s), and significantly longer in the SIT/JT (42 ± 29 s, P < 0.001) and SIT groups (51 ± 29 s, P < 0.0001).

Conclusion

In anaesthetised patients with a simulated difficult airway created with a rigid cervical collar, the overall LTS-D placement success was significantly higher when a jaw thrust manoeuvre was performed, regardless of the particular technique used to introduce the LTS-D. Therefore, an intense jaw thrust manoeuvre should be performed whenever an LTS-D is being inserted.     

胎儿无脾综合征的超声诊断及尸解对照

目的 探讨无脾综合征胎儿的超声声像图及尸解特征。方法 在48186例孕16-40周的胎儿系统超声检查中，发现胎儿心脏畸形88例，其中超声检查及尸解确诊4例无脾综合征，回顾性分析4例无脾综合征胎儿的超声声像图及尸解资料。结果 胎儿无脾综合征超声声像图及尸解特点为：（1）复杂而严重的心脏及大血管畸形（永存动脉干、心内膜垫缺损及单心室等）；（2）内脏转位；（3）脾脏缺如；（4）对称性发育倾向（双侧3叶肺、肝左右叶大小对称、双上腔静脉等）。结论 产前系统胎儿超声检查发现胎儿有复杂心血管畸形时，应常规观察胎儿脾脏及内脏位置，以早期诊断胎儿无脾综合征。     

Immune reconstitution inflammatory syndrome in a large multicenter cohort study: case definition and comparability

Evaluation of: Novak RM, Richardson JT, Buchacz K et al.; HIV Outpatient Study (HOPS) Investigators. Immune reconstitution inflammatory syndrome: incidence and implications for mortality. AIDS 26(6), 721–730 (2012).

This study was nested within the HIV Outpatient Study cohort and investigated the incidence and risk factors for immune reconstitution inflammatory syndrome (IRIS) and the impact of IRIS on mortality. IRIS was defined as a new type B or C AIDS-defining condition or one of a range of mucocutaneous or autoimmune conditions diagnosed within 180 days of starting a new combination antiretroviral therapy regimen, provided there was a documented HIV viral load or CD4 response. IRIS occurred in 10.6% of the 2610 patients. Risk factors independently associated with IRIS included high HIV viral load and low CD4 count. IRIS related to type B or C AIDS-defining conditions was associated with subsequent mortality. Deaths among IRIS cases occurred over 3 years (median) after the IRIS event, making it unlikely that these deaths were directly attributable to IRIS. The IRIS case definition used has important differences when compared with previously published IRIS case definitions thereby affecting comparability of these findings, but this was a pragmatic definition for a large multicenter cohort study utilizing a central database.     

心脾综合征合并肺静脉回流异常的超声心动图诊断

目的探讨超声心动图对心脾综合征合并肺静脉回流异常的诊断价值,分析其漏诊及误诊原因,提高诊断的准确率。方法本文总结了190例心脾综合征患儿肺静脉回流情况的超声检测结果,并与其心血管造影或磁共振检查结果进行对照。结果190例心脾综合征患儿中,无脾综合征159例,其中61例合并肺静脉回流异常(占38.3%),多脾综合征31例,其中5例合并肺静脉回流异常(占16.1%)。66例患儿合并肺静脉回流异常,超声诊断符合者17例(占25.7%),漏误诊49例(占74.3%);66例肺静脉回流异常患儿中,伴有明显异常回流途径者17例,超声诊断15例(占88.2%)。结论无脾综合征患儿常合并肺静脉回流异常。超声心动图可以较准确地诊断心脾综合征伴有明显异常回流途径的肺静脉异位引流,其他类型肺静脉回流异常检出的准确率尚待进一步提高。     

JT Dispersion in Wolff-Parkinson-White Syndrome: Effect of Eccentric Ventricular Depolarization on the Dispersion of Repolarization

There is much interest in QT dispersion for noninvasive risk stratification of patients at risk of arrhythmias. However, little is known about the genesis of abnormal QT dispersion. In particular, whether eccentric ventricular depolarization, as seen in preexcitation, can lead to abnormal dispersion of repolarization is unknown. We studied 24 children aged 1–19 years (mean ± SD, 11 ± 5 years) with manifest preexcitation due to Wolff-Parkinson-White syndrome who had successful catheter ablation. Standard ECGs done preablation, early postablation (< 1 week), mid postablation (> 1 week, < 2 months), and late postablation (> 2 months) were reviewed. The QRS duration prior to ablation ranged from 90–160 ms (mean ± SD, 123 ±21 ms). On the preablation ECG, the JT and JTc dispersions showed no relationship to the QRS duration (r = 0.04 and 0.07, respectively). There was no change in JT dispersion when the preablation (42 ±15 ms) ECG was compared to early (43 ±15 ms), mid (44 ±13 ms), and late postablation (48 ± 19 ms) ECGs. There was no significant change in JTc dispersion as well. Thus, JT dispersion is unrelated to QRS duration and unaffected by catheter ablation in patients with Wolff-Parkinson-White syndrome. Eccentric ventricular depolarization does not lead to abnormal dispersion of repolarization.     

Postural control in patients with Down syndrome

Purpose. To assess postural control in individuals with Down syndrome.

Method. Sixty young adults with Down syndrome were assessed and compared to 10 non-handicapped young. The subjects were asked to stay on a force platform for 30 seconds. Postural control was evaluated in two conditions: open and closed eyes. The kinetic data carried out from the force platform (i.e., ground reaction forces and Center of Pression (COP) displacements) were both evaluated in time domain and in frequency domain.

Results. Patients with Down syndrome are characterized by instable postural control. In particular the data evaluation in frequency domain underlined for Down syndrome subjects versus control group an increase in frequency oscillation both in anterior-posterior and in medio-lateral direction, that are confirmed in time domain analysis only for medio-lateral direction. In DS no changes are evident between eyes open and eyes closed condition.

Conclusion. This study finds that subjects with Down syndrome included in this research demonstrate that deficits in postural control system that may provide a partial explanation for function balance problems that are common in these subjects.     

Septic patients with mitochondrial DNA haplogroup JT have higher respiratory complex IV activity and survival rate

ObjectiveThe influence of mitochondrial deoxyribonucleic acid (mtDNA) haplogroup or oxidative phosphorylation system (OXPHOS) function on survival of septic patients has been scarcely studied. However, the association between mtDNA haplogroup, OXPHOS capacity at diagnosis of severe sepsis, and survival has been not previously reported, and that was the objective of the present study.MethodsThis was a prospective, multicenter, observational study. Blood samples from 198 patients at diagnosis of severe sepsis were analyzed to determine mtDNA haplogroup and platelet respiratory complex IV (CIV) specific activity. The end point of the study was 30-day survival.ResultsSeptic patients with mtDNA haplogroup JT showed higher 30-day survival than those with mtDNA haplogroup non-JT (31/38 [81.6%] vs 99/160 [61.9%]; P= .02). Septic patients with mtDNA haplogroup JT showed higher platelet CIV specific activity than those with mtDNA haplogroup non-JT (P= .002).ConclusionsThe main novel finding of our study, including the largest series providing data on platelet CIV specific activity according to mtDNA haplogroup in severe septic patients, was that those with mtDNA haplogroup JT showed higher survival and higher platelet CIV specific activity at diagnosis of severe sepsis than patients with mtDNA haplogroup non-JT.     

超声心动图对无脾综合征腔静脉回流的诊断价值

目的探讨超声心动图对无脾综合征腔静脉回流的诊断价值,分析其漏误诊原因,以提高诊断准确率。方法回顾分析277例无脾综合征患儿腔静脉回流的超声心动图表现,并与其心血管造影或磁共振检查结果进行对照分析。结果 277例无脾综合征患儿中,双侧上腔静脉151例(54.5%),右侧上腔静脉79例(28.5%),左侧上腔静脉47例(17.0%)。上腔静脉回流超声诊断符合者268例(96.8%),下腔静脉回流超声诊断符合者243例(87.7%)。277例患儿中,34例肝静脉直接回流至心房(12.3%),超声心动图均未予诊断。结论超声心动图可以较准确地诊断无脾综合征腔静脉的回流,但对于肝静脉回流的诊断尚待进一步提高。     

Different Effects of Amiodarone and Quinidine on the Homogeneity of Myocardial Refractoriness in Patients With Intraventricular Conduction Delay

BACKGROUND: Increases in QT and JT dispersion have been suggested as indicative of a proarrhythmic potential as a result of heterogeneity in myocardial refractoriness, the reduction of which by antiarrhythmic agents might be associated with a beneficial effect on the development of serious ventricular arrhythmias. METHODS: To test the hypothesis that amiodarone reduces the heter-ogeneity of ventricular refractoriness to a significantly greater extent than quinidine in patients with intraventricular conduction defects under treatment for ventricular arrhythmias, the corrected and uncorrected QT and JT intervals and dispersions from 12-lead surface electrocardiograms were determined in 120 patients with intraventricular conduction defects with cardiac arrhythmias before and during treatment with amiodarone (n = 60) and quinidine (n = 60). RESULTS: Amiodarone increased QT from 403 +/- 50 ms to 459 +/- 47 ms (P <.001), with a similar increase in the corrected QT interval (QTc) (P <.001). Amiodarone reduced QT dispersion by 40% (P <.001), whereas quinidine increased by 18% (P <.001). The net effects of both drugs were similar for OTc. Amiodarone, but not quinidine, reduced heart rate significantly; amiodarone had no effect on the QRS; but quinidine increased if (P <.001). Quinidine as well as amiodarone increased the JT and JTc intervals significantly, but the effect of quinidine was qualitatively less striking. Amiodarone decreased the JT dispersion by 33% (P <.001) and JTc dispersion by 37% (P <.001). On the other hand, quinidine increased the corresponding values for JT and JTc by 18% (P <.001) and 21% (P <.001), respectively. The overall data on QT and JT dispersion indicate an improvement in the homogeneity of myocardial refractoriness with amiodarone treatment and the converse with quinidine treatment; this observation is consistent with a lower proarrhythmic propensity and mortality with amiodarone than with quinidine. Quinidine increased the QRS interval more than amiodarone, and the data indicate that in patients with intraventricular conduction defects, the monitoring of the JT interval might more accurately reflect changes in myocardial repolarization. CONCLUSIONS: Amiodarone and quinidine both increased the corrected and uncorrected QT and JT intervals; amiodarone decreased and quinidine increased the dispersion of these intervals, and these results suggested an improvement in the homogeneity of myocardial refractoriness as a result of amiodarone treatment and the converse as a result of quinidine treatment. Quinidine increased the QTS interval more than amiodarone, and the data indicate that in patients with intraventricular conduction defects, the monitoring of the JT interval might more accurately reflect changes in myocardial repolarization.     

Survival and mitochondrial function in septic patients according to mitochondrial DNA haplogroup

Introduction

We recently found that platelet cytochrome c oxidase (COX) activities and quantities in 6-month-survival septic patients are significantly higher than those of patients who died before 6 months. Other studies suggested that the mitochondrial DNA (mtDNA) genotype could play a major role in sepsis survival. Given that COX catalytic subunits are encoded by mtDNA, the objective of the present study was to explore whether mtDNA population genetic variation could affect COX activity and quantity and favors sepsis survival.

Methods

A prospective, multicenter, observational study was carried out in six Spanish ICUs. We included 96 patients with severe sepsis. We determined the mtDNA haplogroup, the COX specific activity/citrate synthase specific activity (COXa/CSa) ratio and the COX quantity/citrate synthase specific activity (COXq/CSa) ratio in circulating platelets at the time of diagnosis, day 4 and day 8. We used survival at 1 and 6 months as endpoints.

Results

Patients with the JT mtDNA haplogroup (n = 15) showed higher COXq/CSa ratio at day 4 (P = 0.04) and day 8 (P = 0.02) than those with other haplogroups (n = 81). Logistic regression analysis showed that the JT mtDNA haplogroup (odds ratio = 0.18; 95% confidence interval = 0.04 to 0.94; P = 0.04) and COXq/CSa ratio (odds ratio = 0.53; 95% confidence interval = 0.30 to 0.93; P = 0.03) were associated with 1-month survival after controlling for age and lactic acid levels.

Conclusions

The novel findings of our study are that 1-month surviving septic patients showed higher COXq/CSa ratio than nonsurviving individuals, that patients from the JT mtDNA haplogroup showed a higher COXq/CSa ratio and that JT patients had a higher 1-month survival than patients from other mtDNA haplogroups.     

Esophageal varices in congenital heart disease with total anomalous pulmonary venous connection

Total anomalous pulmonary venous connection (TAPVC) is an uncommon congenital anomaly in which the anatomical presentations vary widely among patients. We hereby present two newborns with TAPVC associated with asplenia syndrome; both had severe esophageal varices due to infradiaphragmatic pulmonary venous drainage. Ultrafast computed tomography (CT) scanning was superior to color Doppler echocardiography and cardiac catheterization as it provided a detailed portrait of the pulmonary drainage. The remarkable radiographic manifestations are presented.     

心脾综合征中肺静脉回流的影像学诊断

目的:分析76例心脾综合征中肺静脉回流特点对手术的影响,并评价其影像学诊断方法。方法:76例均做了心血管造影。其中38例做了MRI检查。结果:56例无脾综合征中33例伴肺静脉异位引流(APVC),28例为完全性肺静脉异位引流(TAPVC),12例为心上型,3例为心下型。20例多脾综合征中,5例伴APVC。结论:心脾综合征肺静脉回流特点多样,完全性肺静脉异位引流发生率高,术前正确诊断对先天性心脏病的手术治疗很重要。心血管造影仍有很高的诊断价值,造影增强磁共振血管成像(CE-MRA)是最好的辅助诊断方法。     

Haemophilus influenzae type b capsular polysaccharide antibody levels in Japanese young patients with hematological malignancies and asplenia

Individuals with immunosuppressive condition have a high risk of invasive Haemophilus influenzae type b (Hib) infection. In Japan, routine Hib vaccination program for children under 5 years old was introduced in December 2008. However, the national policy does not make provision for individuals aged ≥5 years who have medical conditions associated with a high risk of invasive Hib disease to receive Hib vaccine. We measured serum anti-polyribosylribitol phosphate specific (anti-PRP) antibodies to Hib in patients aged ≥5 years with hematological malignancies and asplenia and evaluated their levels of anti-PRP antibodies in post administration of Hib vaccine era. A total of 65 patients (48 with hematological malignancies, and 17 with asplenia) were included in this study, of which 84% had not received Hib vaccine. In addition, 95.4% had short-term protective levels of anti-PRP antibodies (defined as ≥0.15 μg/mL) and 41.5% had long-term protective levels of anti-PRP antibodies (defined as ≥1.0 μg/mL). Five patients had low anti-PRP antibody levels despite a history of Hib vaccination. Our results suggest that young patients with underlying diseases such as hematological malignancies and asplenia may be at risk of invasive Hib disease. Hence, we recommend they should receive Hib vaccines even if they are over the age limit for routine Hib vaccination program.     

血管内超声评估不同中医证型冠心病动脉粥样硬化斑块特征

目的 基于血管内超声(IVUS)观察不同中医证型冠心病(CHD)动脉粥样硬化斑块特征。方法 回顾性分析116例CHD患者,根据中医辨证分为气虚血瘀证(n=33)、痰浊阻滞证(n=42)与痰瘀互结证(n=41),比较不同证型CHD患者病变血管及斑块IVUS特征。结果 气虚血瘀证CHD脂质池面积、血管外弹力膜面积、重构指数、斑块偏心性指数均大于痰浊阻滞证及痰瘀互结证(P均<0.05);气虚血瘀证斑块内纤维脂质成分比例大于痰瘀互结证(P<0.05);痰浊阻滞证脂质池面积、血管外弹力膜面积、斑块偏心性指数均小于、而斑块内坏死成分比例大于痰瘀互结证(P均<0.05)。结论 不同证型CHD动脉粥样硬化斑块特征存在一定差异,以气虚血瘀证斑块稳定性最差。     

Ventricular Fibrillation and Ventricular Tachycardia Triggered by Late‐Coupled Ventricular Extrasystoles in a Brugada Syndrome Patient

Premature ventricular complexes (PVC) falling after the end of the T wave triggered ventricular fibrillation (VF) at night and monomorphic ventricular tachycardia (MVT) during daytime, in a recipient of implantable cardioverter defibrillator with Brugada syndrome. Treatment with bepridil (1) decreased the height of ST segment elevation in leads V1‐V3, (2) completely eliminated VF, and (3) markedly decreased the incidence of PVC and MVT. Albeit rare, VF can be triggered by late‐coupled PVC, due to a mechanism other than phase 2 reentry in some patients with Brugada syndrome. (PACE 2011; e1–e5)     

Pain in Children with Down Syndrome: Assessment and Intervention by Parents

The aims of this study were: 1) to describe how parents assess pain in their child with Down syndrome; and 2) to examine the relationship between pain assessment and decisions by parents to intervene to relieve pain in the child. Twelve parents of school-age children with Down syndrome (ages 6-12 years) were interviewed. Data analysis followed Spradley's Developmental Research Sequence (1979). This included domain analysis, taxonomic analysis, componential analysis, and theme development. Four themes emerged from the data analysis. Theme 1: Being sensitive to verbal and behavioral attributes of pain in the child. Parents reported that they assessed pain in their child with Down syndrome through the child's use of words to express pain, pointing or showing pain location, crying, changes in usual activities, and seeking closeness to the parent. Theme 2: Recognizing emotional and social responses to pain. Parents reported that emotional responses (e.g., anger, fear, frustration, acting out) were part of the pain expressions of their child with Down syndrome. Theme 3: Identifying differences in pain expressions between child and siblings. Parents used strategies to assess pain based on their beliefs that the child was less verbal, slower to complain, and less bothered by pain than siblings. Strategies included questioning the child to elicit self-reporting of pain and observing the child's behaviors. Theme 4: Making decisions to intervene. Parents reported that actions to relieve pain in their child with Down syndrome included more psychologic measures than physical measures, but otherwise no differences were noted in the actions taken for the child and the child's siblings.     

Effect of individualized physical rehabilitation programs on respiratory function in women with post-mastectomy syndrome

Purpose: The purpose of this study was to determine the effectiveness of an individualized physical rehabilitation programs aimed at improving respiratory function in women with post-mastectomy syndrome. Methods: In a randomized controlled trial 50 women with post-mastectomy syndrome were enrolled in the experimental group (EG, n = 25) or the comparison group (CG, n = 25). The program for the EG included: aqua aerobics (i.e. aqua jogging, aqua building, and aqua stretching); conditional swimming; and recreational aerobics. The program for the CG included: conditional swimming and Pilates exercises. Both intervention groups attended individualized physical rehabilitation programs three times per week for 48 weeks. The primary outcome measure was spirometry of the patients measured before, 6 and 12 months after the intervention. Results: This study demonstrated that most of the respiratory function parameters increased significantly in both groups over the year of exercise training. After the year of training the individualized physical rehabilitation program for the EG was significantly better (p < 0.01) as compared with the CG, except for inspiratory reserve volume and maximal voluntary ventilation, which were not statistically different. Conclusions: The results of the study suggest that individual programs of physical rehabilitation could be considered effective for the improvement of respiratory function of the patients with post-mastectomy syndrome. The results obtained could serve as a basis for more widespread clinical program development.