完全性房室间隔缺损合并肺动脉高压术后75例预后分析

探讨完全性房室间隔缺损（CAVSD）合并肺动脉高压术后的疗效。方法　对2003年1月至2007年3月在上海儿童医学中心行CAVSD合并肺动脉高压修补术的75例患儿进行随访分析。患儿术前行心导管检查,了解肺动脉压力及肺小动脉阻力（PAR）。通过超声心动图心尖四腔切面评估术前,术后第2天、1个月、6个月及1年肺动脉高压的情况。记录术后监护时间、呼吸机使用时间及肺动脉高压危象发生情况。结果　75例CAVSD患儿术后监护（4.9±2.1） d,呼吸机使用（2.1±0.9） d,出现肺动脉高压危象12例（16%）。PAR > 8 Wood 单位患儿与PAR≤8 Wood 单位患儿比较,年龄较大,术后监护时间、呼吸机维持时间较长,肺动脉高压危象发生率较高 （P均 < 0.05）。院内死亡5例,其中4例的共同瓣严重发育不良。75例患儿中,术后第2天彩超评估39例（52%）肺动脉压力降至正常,51例（68%）于术后1个月肺动脉压力降至正常,64例（85%）于术后6个月肺动脉压力降至正常;68例（91%）于术后1年肺动脉压力降至正常,余7例术后1年仍合并肺动脉高压。结论　CAVSD早期修补术安全、有效。术前PAR > 8 Wood 单位患儿术后易出现肺动脉高压危象。     

Cerebral hemiatrophy associated with hippocampal sclerosis following a single prolonged febrile seizure

The etiological relation of prolonged febrile seizures with hippocampal sclerosis and cerebral hemiatrophy is controversial. Causal relationship is mainly adopted from retrospective statistical analysis and data from epilepsy surgery. We report a 17-month-old boy who had a prolonged febrile seizure with a transient postictal flaccid hemiparesis and anisocoria. Family history was unremarkable. Magnetic resonance imaging (MRI) revealed abnormal results in the right hippocampal area where diffusion-weighted sequences showed increased signal intensity consistent with acute neuronal edema. Repeat MRI 5 months later demonstrated sclerosis and atrophy of the right hippocampus in association with an increased T2-weighted signal and atrophy of the right frontal, temporal, and parietal lobe. In addition, 18-fluorodeoxyglucose positron emission tomography and 99mTc-ECD single-photon emission computed tomography revealed glucose hypometabolism and decreased perfusion in the right hemisphere, respectively. A final MRI, 12 months following the seizure, was widely unchanged. Interestingly, during a follow-up of 42 weeks, only minor motor deficits were observed. This case uniquely presents the acute onset of hippocampal sclerosis and, consecutively, cerebral hemiatrophy after a single febrile seizure. This suggests that a single prolonged febrile seizure may cause global morphological changes of the brain, not only affecting hippocampal formation.     

A role for Pten in paediatric intestinal dysmotility disorders

Purpose  

The enteric nervous system (ENS) is a network of neurons and glia that lies within the gut wall. It is responsible for the normal regulation of gut motility and secretory activities. Hirschsprung’s disease (HD) is a congenital defect of the ENS, characterised by an absence of ganglia in the distal colon. Intestinal neuronal dysplasia (IND) is a condition that clinically resembles HD, characterised by hyperganglionosis, giant and ectopic ganglia, resulting in intestinal dysmotility. Intestinal ganglioneuromatosis is characterised by hyperplasia and hypertrophy of enteric neuronal cells and causes chronic intestinal pseudo-obstruction (CIPO). Phosphatase and tensin homolog deleted on chromosome 10 (Pten) is a phosphatase that is critical for controlling cell growth, proliferation and cell death. A recent study of Pten knockout mice showed evidence of ganglioneuromatosis in the ENS suggesting a role for this protein in ENS development. Ganglioneuromatosis patients have also been shown to have a decreased level of Pten expression in the colon. The aim of our study was to investigate Pten expression in the ENS of HD and IND patients compared to normal controls.     

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??Abstract??Objective??To understand the prevalence of Epstein-Barr virus in hospitalized children. Methods??All results of anti-VCA-IgM and anti-VCA-IgG to Epstein-Barr virus detected by ELISA in Chongqing Children’s Hospital from January to December in 2009 were collected. The data was counted using statistical analysis of Chi-Square Test by gender?? age group and season. Results??The total infection rate of Epstein-Barr virus for hoapitalized children under 18 years old was 35.27% in 2009 and the recent infection rate was 12.66%.The accumulation infection rate gradually increased with ages?? and nearly half of children had been infected by Epstein-Barr virus in pre-school age group. The period from 3 to 5 years old was the peak age of infection. The accumulation rate among 0??3 months?? 3??6 months?? 6??12 months?? 1??2 years?? 3??5 years?? 6??12 years and 13??18 years old was 27.70%?? 10.48%?? 14.13%?? 27.69%?? 44.61%?? 51.99% and 57.34%?? respectively. And the recent infection rate was 1.15%?? 1.25%?? 2.52%?? 12.55%?? 22.21%?? 18.55% and 20.3%?? respectively. The recent infection rates in March?? September and October were higher than those of others in 2009?? and the difference was statistically significant ??P < 0.05??. Except the 1??2 years group ??P < 0.05???? there was no difference in other age groups about the cumulative and the recent infection rate by gender ??P > 0.05??. Conclusion??1. The infection rate of Epstein-Barr virus for childen is lower than that reported before in China. Nearly half of children have been infected by Epstein-Barr virus in pre-school age group?? the peak period of infection being 3??5 years old.2. There is no gender difference of cumulative and recent infection rate in Chongqing in 2009?? the infection rate in March and September-October were higher than those of others.     

Malignant Struma Ovarii: A Case Report

We present a case of a 40-yr-old woman diagnosed with a primary malignant struma ovarii. The patient was admitted with the complaint of pelvic pain and a large pelvic mass in the mid-portion of lower abdomen on gynecological examination. Pre-operative tumor markers and routine biochemistry were unremarkable. She was treated with total abdominal hysterectomy and right salpingo-oopherectomy. Post-operatively, she was diagnosed with a malignant struma ovarii through the usage of histopathological criteria similar to the guidelines for primary thyroid gland disease. The patient was subsequently performed left salpingo-oopherectomy and retroperitoneal pelvic lympadenectomy for re-staging. Although, left ovary and lymph nodes were histopathologically normal, she was offered thyroidectomy but she refused to accept the offer. Thyroglobulin level was monitored in the post-operative period. She is free of the disease for 18 months.     

Acute lung injury: apoptosis in effector and target cells of the upper and lower airway compartment

Apoptotic cell death has been considered an underlying mechanism in acute lung injury. To evaluate the evidence of this process, apoptosis rate was determined in effector cells (alveolar macrophages, neutrophils) and target cells (tracheobronchial and alveolar epithelial cells) of the respiratory compartment upon exposure to hypoxia and endotoxin stimulation in vitro. Cells were exposed to 5% oxygen or incubated with lipopolysaccharide (LPS) for 4, 8 and 24 h, and activity of caspase‐3, ‐8 and ‐9 was determined. Caspase‐3 of alveolar macrophages was increased at all three time‐points upon LPS stimulation, while hypoxia did not affect apoptosis rate at early time‐points. In neutrophils, apoptosis was decreased in an early phase of hypoxia at 4 h. However, enhanced expression of caspase‐3 activity was seen at 8 and 24 h. In the presence of LPS a decreased apoptosis rate was observed at 8 h compared to controls, while it was increased at 24 h. Tracheobronchial as well as alveolar epithelial cells experienced an enhanced caspase‐3 activity upon LPS stimulation with no change of apoptosis rate under hypoxia. While increased apoptosis rate is triggered through an intrinsic and extrinsic pathway in alveolar macrophages, intrinsic signalling is activated in tracheobronchial epithelial cells. The exact pathway pattern in neutrophils and alveolar epithelial cells could not be determined. These data clearly demonstrate that upon injury each cell type experiences its own apoptosis pattern. Further experiments need to be performed to determine the functional role of these apoptotic processes in acute lung injury.     

The effects of volatile induction and maintenance of anesthesia and selective spinal anesthesia on QT interval, QT dispersion, and arrhythmia incidence

OBJECTIVE:

The effects of sevoflurane general anesthesia and bupivacaine selective spinal anesthesia on QT dispersion (QTd) and corrected QT (QTc) interval were investigated.

METHODS AND MATERIALS:

This prospective, randomized, double-blind study was conducted between July and September 2009 in the Urology and General Surgery operating rooms. Forty ASA I–II patients undergoing noncardiac surgery were randomized into two groups: Group R (n = 20) and Group V (n = 20). In Group R, 5 mg bupivacaine was administered into the spinal space. Anesthesia induction in Group V was established with sevoflurane + 0.1 mg/kg vecuronium using the maximum vital capacity technique. Anesthesia was maintained with 2–3% sevoflurane + 50% N₂O/O₂ inhalation. All patients were tested with a 24-hour Holter ECG device. QT, QTc, and QTd intervals were measured using 12-lead ECG records at 1 and 3 minutes during preinduction, postinduction, postincision and postextubation periods. Mean arterial pressure (MAP), heart rate and ECG records were measured simultaneously.

RESULTS:

None of the patients displayed arrhythmia. There was no significant difference between the groups with regard to QTd values (p>0.05). However, QTc was longer in Group V than in Group R after the induction of anesthesia at 3 minutes, after the intubation at 1 and 3 minutes, and after the incision at 1 and 3 minutes. MAP and heart rate were generally higher in Group V (p<0.05).

CONCLUSION:

Although Volatile Induction and Maintenance of Anesthesia (VIMA) with sevoflurane might prolong the QTc interval and did not result in arrhythmia, selective spinal anesthesia with bupivacaine was not associated with alterations in the QT interval or arrhythmia.     

Array-based comparative genomic hybridization identifies a high frequency of copy number variations in patients with syndromic overgrowth

Overgrowth syndromes are a heterogeneous group of conditions including endocrine hormone disorders, several genetic syndromes and other disorders with unknown etiopathogenesis. Among genetic causes, chromosomal deletions and duplications such as dup(4)(p16.3), dup(15)(q26qter), del(9)(q22.32q22.33), del(22)(q13) and del(5)(q35) have been identified in patients with overgrowth. Most of them, however, remain undetectable using banding karyotype analysis. In this study, we report on the analysis using a 1-Mb resolution array-based comparative genomic hybridization (CGH) of 93 patients with either a recognizable overgrowth condition (ie, Sotos syndrome or Weaver syndrome) or an unclassified overgrowth syndrome. Five clinically relevant imbalances (three duplications and two deletions) were identified and the pathogenicity of two additional anomalies (one duplication and one deletion) is discussed. Altered segments ranged in size from 0.32 to 18.2 Mb, and no recurrent abnormality was identified. These results show that array-CGH provides a high diagnostic yield in patients with overgrowth syndromes and point to novel chromosomal regions associated with these conditions. Although chromosomal deletions are usually associated with growth retardation, we found that the majority of the imbalances detected in our patients are duplications. Besides their importance for diagnosis and genetic counseling, our results may allow to delineate new contiguous gene syndromes associated with overgrowth, pointing to new genes, the deregulation of which may be responsible for growth defect.     

Down-regulation of p38 mitogen-activated protein kinase activation and proinflammatory cytokine production by mitogen-activated protein kinase inhibitors in inflammatory bowel disease

Crohn's disease and ulcerative colitis are inflammatory bowel diseases (IBD) characterized by chronic relapsing mucosal inflammation. Tumour necrosis factor (TNF)‐α, a known agonist of the mitogen‐activated protein kinase (MAPK) pathway, is a key cytokine in this process. We aimed first to determine whether p38 MAPK is activated in IBD inflamed mucosa, and then studied the effect of four different p38α inhibitory compounds on MAPK phosphorylation and secretion of proinflammatory cytokines by IBD lamina propria mononuclear cells (LPMCs) and organ culture biopsies. In vivo phospho‐p38α and p38α expression was evaluated by immunoblotting on intestinal biopsies from inflamed areas of patients affected by Crohn's disease and ulcerative colitis, and from normal mucosa of sex‐ and age‐matched control subjects. Both mucosal biopsies and isolated LPMCs were incubated with four different p38α selective inhibitory drugs. TNF‐α, interleukin (IL)‐1β and IL‐6 were measured in the organ and cell culture supernatants by enzyme‐linked immunosorbent assay. We found higher levels of phospho‐p38α in the inflamed mucosa of IBD patients in comparison to controls. All the p38α inhibitory drugs inhibited p38α phosphorylation and secretion of TNF‐α, IL‐1β and IL‐6 from IBD LPMCs and biopsies. Activated p38α MAPK is up‐regulated in the inflamed mucosa of patients with IBD. Additionally, all the p38α selective inhibitory drugs significantly down‐regulated the activation of the MAPK pathway and the secretion of proinflammatory cytokines.