Saturation compensation for visible light communication with off-the-shelf detectors.

A photodetector may get saturated if the incident optical power approaches the detector saturation threshold. In such a case, a visible light communication (VLC) system fails to recover the useful transmitted signals. We introduce a saw waveform based response characterization technique when an off-the-shelf avalanche photo-diode (APD) is deployed. Subsequently, we present two saturation compensation methods, namely the optical attenuation and gain reduction. We analytically conclude that the latter outperforms the former in terms of signal to noise ratio. Experimental results demonstrate the effectiveness of the proposed methods in improving the bit error rate (BER) and error vector magnitude (EVM) performances in the presence of strong ambient radiation and detector noise. In addition, optimization of the APD internal gain and joint employment of the two compensation methods are discussed.

Comparison of interpolation-based sampling frequency offset compensation schemes for practical OFDM-VLC systems.

Sampling frequency offset (SFO) is an important issue in the orthogonal frequency-division multiplexing (OFDM)-based visible light communication (VLC) systems with low-cost analog-to-digital or digital-to-analog converters (ADCs/DACs). A digital interpolation or resampling filter can be used to effectively compensate the SFO. In such case, oversampling at the receiver ADC is required to mitigate the aliasing effect due to imperfect DACs and nonlinearity of visible light sources that cause extra frequency components inside/outside the OFDM signal spectrum. The oversampling factor (rate) is mainly determined by the order of the digital interpolation filter and nonlinear VLC links. The design of the OFDM-VLC receiver incorporating the digital interpolation filter is vital as it affects not only the transmission performance but also the complexity of digital signal processing (DSP). To evaluate the feasibility of the digital interpolation-based SFO compensation schemes for cost-sensitive VLC applications, in this paper, a real-time OFDM-VLC receiver incorporating the 2nd/3rd/4th order interpolation filters is experimentally demonstrated. An OFDM frame structure is designed for the synchronization including SFO estimation and compensation, in which the precision and latency of DSP are considered. On the basis of the real-time OFDM-VLC receiver, the comparison in the VLC transmission performance and DSP complexity between different interpolation-based SFO compensation schemes is discussed.

Graph-based multi-user scheduling for indoor cooperative visible light transmission.

Recent progress in the indoor visible light communication (VLC) has shown promising signs of alleviating an increasing strain on the radio frequency spectrum and enhancing transmission capacity. Nevertheless, the indoor VLC usually suffers from inter-channel interference (ICI) due to the dense light-emitting diode (LED) deployment. The ICI is considered as a key factor affecting signal to interference and noise ratio (SINR) and spectral efficiency. To address this challenge, an efficient multi-user scheduling framework that employs interference coordination and cooperative transmission is investigated based on the graph theory. To effectively mitigate ICI and maximize benefit of the cooperative transmission, the cell-centric (CC) and user-centric (UC) clustering are introduced for cooperative transmission. For the CC clustering, the multi-user scheduling problem under the proportional fairness criterion is formulated to maximize spectral efficiency while ensuring user fairness. Such a problem is solved by linear programming and greedy algorithms after transforming it into a maximum weighted independent set problem with the aid of a modified interference graph. For the UC clustering, the multi-user scheduling problem under the max-min criterion is formulated and solved by a proposed heuristic approach based on the bipartite graph theory. Numerical results show that the proposed graph-based scheduling is capable of providing up to 7.7 dB gain in SINR over the non-cooperative transmission. The bipartite graph scheduling offers high spectral efficiency and service fairness index. In the worst case with an occlusion probability of 1, a small SINR penalty of up to 1 dB is observed with the greedy algorithm. It is shown that the graph-based scheduling is robust to receiver rotation and occlusion in terms of spectral efficiency, SINR, and user fairness.

Real-time investigation of CAP transceivers with hybrid digital equalization for visible light communication.

In a practical light emitted diodes (LEDs)-based visible light communication (VLC) system, high-speed transmission is generally limited by the LED bandwidth. To address the bandwidth limitation, a hybrid digital linear and decision-feedback equalization (DFE) is investigated to improve the transmission performance (or spectral efficiency) in the carrier-less amplitude phase modulation (CAP)-based VLC systems. A real-time CAP-VLC transceiver with the hybrid digital equalization is designed, based on which 200 Mb/s transmission is successfully demonstrated over a 15 m VLC link with the commercial red LEDs (bandwidth: 6.5 MHz). In the real-time CAP-VLC system, the baseline wander (BLW) is observed, due to the removal of the low-frequency components with a direct current (DC) block. The BLW effect can be mitigated by increasing the roll-off factor. However, this roll-off factor affects the equalization performance, due to an increased loss in the signal spectrum beyond the system bandwidth. Optimization of the roll-off factor and filter length is required. Experimental results show that, with the optimized real-time transceiver design, the hybrid Wiener/recursive least squares (RLS) and DFE significantly improves the error vector magnitude (EVM) performance compared with the DFE. In addition, the digital signal processing (DSP) complexity is discussed.

All-optical mode-group multiplexed transmission over a graded-index ring-core fiber with single radial mode.

We present a design of graded-index ring-core fiber (GI-RCF) supporting 3 linearly polarized (LP) mode-groups (i.e. LP01, LP11 and LP21) with a single radial index of one for mode-division multiplexed (MDM) transmission. Reconfigurable spatial light modulator (SLM) based spatial (mode) (de)multiplexers are used to systematically characterize spatial/temporal modal properties of the GI-RCF. We also demonstrate all-optical mode-group multiplexed transmissions over a 360m fabricated GI-RCF without using multiple-input multiple-output digital signal processing (MIMO DSP).

Expression of the novel all-trans retinoic acid-related resistance gene HA117 in pediatric solid tumors.

This study aimed to detect the protein expression of HA117 in pediatric solid tumors. Immunohistochemistry was performed to detect the expression of HA117 and P-gp in pediatric solid tumors. In Hodgkin lymphoma (HL), non-Hodgkin lymphoma (NHL), nephroblastoma (WT), and neuroblastoma (NB), the positive expression rate of HA117 was 65.4%, 58.3%, 81.3%, and 74.1%, and that of P-gp was 57.7%, 70.8%, 65.6%, and 66.7%, respectively. HA117 expression was closely related to the clinical stage of HL (P=0.004) and to the International Prognostic Index score, mediastinal lesions, and clinical stages of NHL (P=0.01, 0.03, and 0.01). The expression of HA117 in WT was higher than in adjacent normal tissues, but there was no statistical significance (P=0.21). The positive expression of HA117 in NB was markedly higher than that in normal tissues (P=0.002), which closely associated with histologic type and lymph node metastasis (P=0.03 and 0.001). Spearman correlation analysis revealed that HA117 expression was not correlated with P-gp in these 4 tumors. This suggests that HA117 might be an important resistance gene in pediatric solid tumors. The mechanism underlying the resistance to all-trans retinoic acid conferred by HA117 is different from that of P-gp.

Thirty-five consecutive pediatric living donor liver transplantation: experiences and lessons learned from a single center.

BACKGROUND/AIMS: In the last 10 years, the early patient outcome of liver transplantation in children have significantly improved. Now the overall outcomes of pediatric LT are promising. METHODOLOGY: In this study, we review the outcome of all pediatric liver transplants performed at our center and analyze our experiences with pediatric liver transplant. Of the 34 liver transplant recipients, 26 were highly urgent (19.7%). RESULTS: Actuarial patient survival rates at 6, 12, and 36 months was 82.9%, 79.8% and 72.2%, respectively. Indications for liver transplant were biliary atresia (n = 22), Wilson's disease (n = 4), glycogen storage disease (n = 3), portal vein cavernous transformation (PVCT) (n = 3), fulminant liver failure (n = 1), and cryptogenic cirrhosis (n = 1). The main complications were surgical complications (including biliary complications, portal vein or arterial complications, intestinal perforation, postoperative bleeding, of which 20% required reoperation) and infections. Cyclosporine was the primary immunosuppressive agent used in 70.6% of patients, with a 26.5% incidence of acute allograft rejection within the first six months. One children underwent re-transplant as a result of hepatic artery thrombosis. Nine children died during followup. They were related to portal vein thrombosis (one), chronic rejection (one), sepsis (one), post-transplant lymphoproliferative disease (one) and so on. CONCLUSIONS: The overall outcomes of pediatric liver transplantation at our center are promising. Advances in post-transplant care and monitoring of the recipients, technical refinements enable these results.

Expression patterns of CXCR4 in different colon tissue segments of patients with Hirschsprung's disease.

C-X-C chemokine receptor type 4 (CXCR4) plays a crucial role in a wide range of physiological and pathological processes, including the migration of stem cells, such as neural crest-derived cells. Hirschsprung's disease (HSCR), a developmental disorder characterized by the absence of ganglion cells, is regarded as the consequence of the premature arrest of the craniocaudal migration of neural crest-derived cells (NCDCs) in the gastrointestinal tract during the development of the enteric nervous system (ENS). In this study, colon tissue samples from 61 HSCR patients were surgically collected and divided into aganglionic, oligoganglionic and normal ganglionic segments. Quantitative real-time polymerase chain reactions (PCR), Western blotting, and immunohistochemical and immunofluorescence staining were performed to analyze the expression levels and patterns of CXCR4 in different colon tissue segments. The expression levels of CXCR4 mRNA and protein in the aganglionic segments were decreased compared to the normal ganglionic and oligoganglionic colon segments (p<0.01). Immunohistochemical staining showed that intensive CXCR4 staining was detected in the ganglion cells and the supporting glial cells in the ganglion in control colon specimens and normal ganglionic and oligoganglionic colon segments from the HSCR patients; however, CXCR4 staining was significantly decreased in the aganglionic colon segments. Immunofluorescence staining showed that CXCR4 staining was mainly detected in the ganglia where RET-positive ganglion cells were observed. Elucidating CXCR4 expression patterns in colon segments could be the basis for further investigations of the potential role of CXCR4 in ENS development.

Usage of 64-detector-row spiral computed tomography volumetry in preoperative volume prediction in living donor liver transplantation in children.

PURPOSE: To investigate the correlation between the graft volume calculated by 64-detector-row spiral computed tomography (CT) and the graft weight measured during the living donor liver transplantation (LDLT) operation, and try to get an equation to help determine the possible weight of graft before operation. METHODS: 23 donors with left lateral lobe LDLT were enrolled to undergo 64-detector-row spiral CT and the imaging data at the hepatic venous phase was used for whole and partial liver volumetric measurement on a dedicated image postprocessing workstation. The resected part of donor liver was weighed during the operation. Statistical analysis with SPSS15.0 was used to analyze the correlation between the estimated liver volume by CT and the actual graft weight. RESULTS: The graft volume calculated preoperatively by CT (293.35 ± 53.43 ml) was significantly larger than measured graft weight during the operation (252.82 ± 50.96 g) (P < 0.05). All corresponding pre- and intraoperative data correlated significantly (R = 0.885) (P < 0.001). Intraoperatively expected weight (W (intraop)) in grams and volume calculated preoperatively by CT (V (preop)) in milliliters can be calculated with the equation W (intraop) (g) = 0.844 × V (preop) (ml) + 5.271. CONCLUSION: Liver volume calculated by 64-detector-row spiral CT preoperatively can predict the actual graft weight, which is very useful in donor selection in LDLT.

Chemosensitization in non-small cell lung cancer cells by IKK inhibitor occurs via NF-kappaB and mitochondrial cytochrome c cascade.

In this study, we demonstrated with mechanistic evidence that parthenolide, a sesquiterpene lactone, could antagonize paclitaxel-mediated NF-kappaB nuclear translocation and activation by selectively targeting I-kappaB kinase (IKK) activity. We also found that parthenolide could target IKK activity and then inhibit NF-kappaB; this promoted cytochrome c release and activation of caspases 3 and 9. Inhibition of caspase activity blocked the activation of caspase cascade, implying that the observed synergy was related to caspases 3 and 9 activation of parthenolide. In contrast, paclitaxel individually induced apoptosis via a pathway independent of the mitochondrial cytochrome c cascade. Finally, exposure to parthenolide resulted in the inhibition of several NF-kappaB transcript anti-apoptotic proteins such as c-IAP1 and Bcl-xl. These data strengthen the rationale for using parthenolide to decrease the apoptotic threshold via caspase-dependent processes for treatment of non-small cell lung cancer with paclitaxel chemoresistance.

Successful treatment of Epstein-Barr Virus-associated haemophagocytic syndrome arising after living donor liver transplantation.

A 6-year-old girl received an orthotopic liver transplant secondary to liver failure of unknown etiology. Several days after liver transplantation, she developed pancytopenia and Epstein -Barr Virus (EBV)-positive seroconvert after transplant. Taking of medical history to provide evidence of pancytopenia correlated with EBV infection after transplant. Bone-marrow biopsy was carry out to confirm haemophagocytic syndrome (HPS). Laboratory investigations, including blood routine count, microscopic blood film examination, serum ferritin concentration, and liver function tests were carried out starting from the first day of suspected HPS infection, continuing until 15 weeks post-infection. Liver transplantation followed with a combination of cyclosporin A, corticosteroids, VP-16, and supportive strategies.

Adult to pediatric living donor liver transplantation for portal cavernoma.

AIM: Portal cavernoma (PC) is an important cause of non-cirrhotic portal hypertension with severe complications, such as variceal hemorrhage in pediatric patients. With the development of new surgical techniques, living donor liver transplantation (LDLT) has recently been recognized as a viable but challenging treatment option for PC. The purpose of the present study was to summarize the efficacy of LDLT in PC patients and to carry out a follow-up study of pediatric recipients. METHODS: The primary indication for LDLT in our research was PC with severe variceal bleeding and liver function decompensation. Three patients were diagnosed with PC following evaluation with computed tomography angiography and abdominal color Doppler ultrasonography (CDU). RESULTS: Various surgical techniques, including jump bypass grafting for portal vein anastomosis, were carried out according to the range and degree of cavernous transformation within the splenic vein and superior mesenteric vein. Postoperative CDU confirmed the early integrity of the portal vein (PV) in each patient. PV rethrombosis occurred in one patient 7 days after LDLT, despite anticoagulation therapy with coumadin. Two of the three patients had no further episodes of variceal hemorrhage during the 2-year follow-up period. CONCLUSIONS: The present study is the first report of the successful use of LDLT to treat pediatric PC patients. We conclude that LDLT is effective for the majority of pediatric patients with PC.

[Retrospective analysis of maternal and infant birth features of hepatoblastoma patients].

OBJECTIVE: To explore the risk factors for hepatoblastoma. METHODS: A case-cohort study using Logistic regression multiple variables analysis of medical record data sets was conducted to examine infant and perinatal risk factors for hepatoblastoma. RESULTS: Birth weight less than 1,000 g was associated with a strongly increased risk of hepatoblastoma (odds risk, OR = 26.0, 95% confidence interval, CI: 14.0 to 65.7). After adjustment of birth weight, a moderately increased risk of hepatoblastoma was found for older maternal age ( > 35 years vs. 20 to 34 years: OR = 2.6, 95% CI: 0.9 to 5.9), maternal smoking (OR = 2.9, 95% CI: 1.1 to 4.2) and higher maternal pregnancy body mass index (OR = 3.2, 95% CI: 1.0 to 6.7). CONCLUSION: Very low birth weight and maternal characteristics including overweight, smoking are associated with hepatoblastoma risk.

BMP2 Is Related to Hirschsprung's Disease and Required for Enteric Nervous System Development.

The enteric nervous system (ENS) is derived from neural crest cells (NCCs). Defects in ENS NCCs colonizing in the intestines lead to an absence of enteric ganglia in the colon and results in Hirschsprung's disease (HSCR). Bone morphogenetic proteins (BMPs) play diverse roles in the proliferation, migration and survival of ENS NCCs; however, whether BMPs are involved in HSCR and the underlying mechanism remains largely unknown. In this study, we found that BMP2 expression is significantly decreased in HSCR patients. Further experiments demonstrated that BMP2 is involved in the regulation of NCC proliferation, migration and differentiation. In a detailed analysis of the role of BMP2 in HSCR development in vivo, we demonstrated that BMP2b regulates the proliferation, migration and differentiation of vagal NCCs in zebrafish and that BMP2b is required for intestinal smooth muscle development. In addition, we showed that BMP2b is involved in regulating the expression of glial cell line-derived neurotrophic factor (GDNF) in the intestine, which mediates the regulation of ENS development by BMP2b in zebrafish. These results highlight a central role of the BMP-GDNF cascade in intestinal patterning and ENS development. Our results further demonstrate the key role of BMP2 in the etiology of HSCR in vitro and in vivo.

Behavioural disorders in children with pectus excavatum in China: a retrospective cohort study with propensity score matching and risk prediction model.

OBJECTIVES: Surgical repair of pectus excavatum is typically carried out immediately prior to puberty. However, at the time of surgery, some psychosocial issues, such as behavioural disorders may already have developed and the likelihood of these psychosocial disorders resolving after surgery is unclear. For this reason, some surgeons choose to perform surgical repair at an earlier age in some children. The study retrospectively compares the rate of behavioural disorders in children undergoing the Nuss procedure at 4 vs. 10 years of age. We also attempted to develop a model to predict the risk of behavioural disorders in 10 year-old patients. METHODS: The current study included children receiving Nuss procedure for pectus excavatum at either 4 or 10 years of age. The presence/absence of behavioural disorder was assessed preoperatively, and in the third year, after removal of the bar. A propensity score matching (PSM) analysis was conducted to reduce the potential for confounding by baseline factors. Multivariable logistic regression was conducted to establish a model to predict the risk of behavioural disorders in the third year after the removal of the bar. The model was accessed by discrimination and calibration. A formula and a nomogram were developed based on the results. RESULTS: The number of patients in each group was 45 after PSM. The rate of behavioural disorders at the baseline was significantly higher in the children undergoing Nuss procedure at 10 years of age [36% vs 20%, odds ratio (OR) 2.21, 95% confidence interval (CI) 0.85-5.72; P = 0.157]. The rate of behavioural disorders in the third year after the removal of the bar was 36% and 18% in children undergoing surgery at 10 and 4 years of age, respectively (OR 2.55, 95% CI 0.96-6.79; P = 0.094). The rate of persistent behavioural disorders, defined as continuing to have behavioural disorders in the third year after the removal of the bar in those with behavioural disorders at the baseline, was 88% vs 56% (OR 3.47, 95% CI 0.56-21.36; P = 0.204). Two patients (4%) relapsed in each group. A risk prediction model by variables of gender, Haller index, pulmonary function and score of Child Behaviour Checklist at the baseline was provided. CONCLUSIONS: The rate of behavioural disorders was considerably lower in children who underwent the Nuss procedure at 4 years of age than at 10 years of age. Behavioural disorders may not readily resolve after surgery. Performing surgery at an early age rather than just before puberty may be better for psychosocial development. Psychosocial aid is necessary in addition to surgery to address behavioural disorders.

Potential biomarkers involving IKK/RelA signal in early stage non-small cell lung cancer.

The clinical relevance of nuclear factor kappaB (NF-kappaB) and its regulatory molecules on prognosis of patient with early stages of non-small cell lung cancer (NSCLC), remains unclear. Therefore, we conducted biomarker analyses with survival in patients with stages I and II NSCLC. Tumor samples were collected from 88 patients with early-stage NSCLC (stages I, II). A minimum follow-up period of 5 years was required. RelA, phosphorylated I kappaB (pI kappaB alpha), pIKK alpha/beta were detected by immunostaining. NF-kappaB DNA binding activity was assessed by electrophoretic mobility shift assay. Association of clinical and pathologic variables (e.g. sex, age, pathologic stage) with relevant molecules was determined by Pearson's chi(2) test or Fisher's exact test. Survival analysis based on single expression of RelA, pI kappaB alpha, pIKK alpha/beta as well as composite expressions were evaluated using Cox proportional hazards regression models, and log rank test followed Kaplan-Meier estimates. RelA, pI kappaB alpha, pIKK alpha/beta were observed as increased expression in NSCLC tissues compared with adjacent normal tissues and normal lung tissues. These molecules were associated with tumor-node-metastasis stages, T stages and histological status, respectively. Among the molecules analyzed, RelA and pI kappaB alpha-positive were statistically significant predictors of patient death in the entire patient population adjusted by age, gender and smoking status; furthermore both RelA and pI kappaB alpha-positive was the strongest prognostic indicators of poor prognosis by univariate and multivariate analyses. Borderline positive correlations were observed between RelA and pI kappaB alpha or pIKK alpha/beta expression. In this cohort of early-stage NSCLC patients, molecular markers, especially composite application of multiple biomarkers (both nuclear RelA and cytoplasmic pI kappaB-alpha expression) that independently predict overall survival have been identified.

[Living-related liver transplantation for cavernous transformation of portal vein: a clinical study of 3 cases].

OBJECTIVE: To review the outcomes of living-related liver transplantation (LRLT) in treating 3 cases of cavernous transformation of portal vein (CTPV) with severe portal hypertension. METHODS: Three children (two boys and one girl) were presented to our hospital with recurring esophageal variceal bleeding, decompensating ascites, splenomegaly and refractory anemia. CTPV was confirmed by intravenous computed tomographic portography using a helical computed tomography scanner and 3-dimensional image reconstruction. LRLT were performed in these 3 patients from July 2006 to January 2007. The evaluation of the outcomes was made by referring to their clinical features and laboratory and imaging examination findings. RESULTS: Although one patient died from early graft thrombosis, the other two patients showed excellent prognoses. They lived and stayed well during a follow-up period of 12-14 months. Following the transplantations, there had been no esophageal variceal hemorrhage, the ascites disappeared and the portal hypertension vanished. Their hemoglobin, blood platelets count, and serum albumin reached normal values. CONCLUSION: LRLT is an effective procedure in treating CTPV with severe portal hypertension. The reconstruction of the portal vein is the difficult part of the LRLT procedure.

Changes in resting pulmonary function testing over time after the Nuss procedure: A systematic review and meta-analysis.

BACKGROUND: Pectus excavatum, the most common congenital chest wall deformity in pediatric patients, leads to pulmonary dysfunction. There is no consensus regarding the effectiveness of the Nuss procedure for recovering pulmonary function. In this meta- analysis, we focused on the changes that occur in pulmonary function after the Nuss procedure. METHODS: We performed a literature search in the MEDLINE, Embase, Cochrane library and PubMed databases. The included studies were required to contain pulmonary function tests with results adjusted to predicted values both before and after the Nuss procedure. The key outcomes of interest in this analysis were pulmonary function measured as forced expiratory volume in 1s (FEV1) and forced vital capacity (FVC). Subgroup analyses were performed based on time since surgery and the mean ages of the patients when they underwent surgery by forest plots and meta-regressions. RESULTS: Thirteen studies involving 465 participants were included in this review. The standard mean difference (SMD) observed in FEV1 and FVC after surgery were 0.17 (95% CI, 0.01-0.33, p=0.04) and -0.18 (95% CI, -0.41-0.06, p=0.14), respectively. The overall meta-regression SMD of FEV1 and FVC by time since surgery were 1.21 (95% CI, 1.04-1.41, p=0.020) and 1.38 (95% CI, 1.05-1.83, p=0.027), respectively. We found evidence of a temporal relationship between time at which pulmonary function tests were performed after surgery and predicted FEV1 and FVC values. The SMD of FEV1 (0.26, p=0.012) was slightly higher in group evaluated more than 2year after initial surgery. CONCLUSIONS: Abnormal resting pulmonary functions tests performed prior to surgery showed an initial depression after surgery. The FEV1 of patients slightly increased at 2year post surgery compared with the baseline. Further studies with longer term follow-up are still needed to determine if pulmonary function could improve to normal after surgery. LEVELS OF EVIDENCE: Level of evidence: 4 (based on lowest level of article analyzed in meta-analysis/systematic review).

BMP4 knockdown of NCSCs leads to aganglionosis in the middle embryonic stage.

Transplacental bone morphogenetic protein (BMP)4 RNA interference (RNAi) is a technique used to knockdown genes in embryos. BMP4 are essential for the development of nervous system in the differentiation of neural crest stem cells (NCSCs). The failure of differentiation and migration of NCSCs may lead to aganglionosis. In the present study, pregnant mice were divided into three groups: Ringer's group, pSES group and RNAi­BMP4 group. In order to silence the BMP4 gene in the first generation (F1), 11.5 day pregnant mice were injected with the small interfering RNA BMP4 plasmid, pSES or Ringer's solution via the tail vein. Semi­quantitative reverse transcriptase­polymerase chain reaction (RT­PCR)and western blotting were employed to ensure the downregulation of BMP4. Finally, X­rays were performed following a barium enema. Aganglionosis was diagnosed by general anatomy and immunohistochemistry. Compared with the control group, transplacental RNAi was able to downregulate the BMP4­Smad4 of 11.5 day embryos, as determined by semi­quantitative RT­PCR and western blotting. The megacolons of the mice were demonstrated by X­ray and confirmed by general anatomy. Aganglionosis of colonic mucosa and submucosa were diagnosed by pathology, and immunohistochemistry. Knockdown of BMP4 in pregnant mice at the middle embryonic stage led to aganglionosis. It was therefore demonstrated that BMP­Smad was essential to the NCSCs of middle stage embryos. BMP­Smad served important roles in the generation of aganglionosis. This technique of knockdown BMP4 gene may be used to establish an aganglionosis mouse model.

Potential association of long noncoding RNA HA117 with tetralogy of Fallot.

Tetralogy of Fallot (TOF) is a congenital heart disease characterized by abnormal cardiomyocyte differentiation in the right ventricular outflow tract (RVOT), and HA117 is a novel long noncoding RNA (lncRNA) with anti-differentiation roles. To investigate the potential association of HA117 with TOF, we collected 84 RVOT tissues from patients with TOF. We determined the expression of HA117 in RVOT samples from TOF patients and collected clinical data to conduct a cross-sectional and short-term follow-up study. McGoon ratio, Nakata index, and left ventricular end-diastolic volume index (LVEDVI) were negatively correlated with the expression of HA117 based on subgroup analysis, correlation analysis and logistic regression analysis. Additionally, cardiopulmonary bypass (CPB) time and ICU stay were longer in patients with higher expression of HA117 than in patients with lower expression of HA117. Furthermore, percentage improvement in SPO2 was significantly reduced in patients with increased HA117 expression at 6 months after surgery. Our results suggested that the increased expression of the novel lncRNA HA117 is a risk factor for unfavorable McGoon ratio, Nakata index and LVEDVI in TOF patients. Additionally, an increased expression of HA117 might lead to adverse short-term outcomes in TOF patients.