Fatty liver disease protective MTARC1 p.A165T variant reduces the protein stability of MTARC1.

Non-alcoholic fatty liver disease (NAFLD) is one of the most common causes of liver disease worldwide. MTARC1, encoded by the MTARC1 gene, is a mitochondrial outer membrane-anchored enzyme. Interestingly, the MTARC1 p.A165T (rs2642438) variant is associated with a decreased risk of NAFLD, indicating that MTARC1 might be an effective target. It has been reported that the rs2642438 variant does not have altered enzymatic activity so we reasoned that this variation may affect MTARC1 stability. In this study, MTARC1 mutants were generated and stability was assessed using a protein stability reporter system both in vitro and in vivo. We found that the MTARC1 p.A165T variant has dramatically reduced the stability of MTARC1, as assessed in several cell lines. In mice, the MTARC1 A168T mutant, the equivalent of human MTARC1 A165T, had diminished stability in mouse liver. Additionally, several MTARC1 A165 mutants, including A165S, A165 N, A165V, A165G, and A165D, had dramatically decreased stability as well, suggesting that the alanine residue of MTARC1 165 site is essential for MTARC1 protein stability. Collectively, our data indicates that the MTARC1 p.A165T variant (rs2642438) leads to reduced stability of MTARC1. Given that carriers of rs2642438 show a decreased risk of NAFLD, the findings herein support the notion that MTARC1 inhibition may be a therapeutic target to combat NAFLD.

Assessment of right ventricular diastolic function in pediatric patients with repaired tetralogy of Fallot by cardiovascular magnetic resonance and echocardiography.

OBJECTIVES: Cardiovascular magnetic resonance (CMR) imaging is routinely performed for assessing right ventricular (RV) systolic but not diastolic function. We aimed to investigate CMR-based assessment of RV diastolic function in pediatric patients with repaired tetralogy of Fallot (rTOF), compared to transthoracic echocardiography (TTE) measurements. METHODS: A total of 130 consecutive pediatric patients with rTOF who underwent clinically indicated CMR and same-day TTE were included. Forty-three controls were recruited. Phase-contrast images were used to measure trans-tricuspid valve flow velocities during early (E) and late diastolic (A) phases (measured in cm/s). Feature tracking of the tricuspid annulus was performed to derive early (e') and late diastolic (a') myocardial velocities (measured in cm/s). RV diastolic function was evaluated by E/A ratio, E/e' ratio, and E-wave deceleration time (measured in milliseconds). Regression analyses were utilized to identify potential variables associated with RV diastolic dysfunction (DD). The performance of CMR-derived parameters in diagnosing RV DD was assessed using receiver-operating characteristic analyses. RESULTS: Good agreement was found between CMR and TTE measurements (ICC 0.70-0.89). Patients with RV DD (n = 67) showed significantly different CMR-derived parameters including E and e' velocities, and E/A and E/e' ratio, compared to patients without DD (n = 63) (all p < 0.05). CMR-derived E and e' velocities and E/e' ratio were independent predictors of RV DD. E/e' of 5.8 demonstrated the highest discrimination of RV DD (AUC 0.76, sensitivity 70%, specificity 86%). CONCLUSIONS: CMR-derived parameters showed good agreement with TTE parameters in determining RV DD. CMR-derived E/e' was proved to be the most effective in identifying RV DD. CLINICAL RELEVANCE STATEMENT: This study demonstrated the feasibility and efficacy of CMR in assessing diastolic function in pediatric patients. RV DD was presented in over half of patients according to current TTE guidelines, highlighting the need for assessing RV diastolic function during follow-up. KEY POINTS: • Routinely acquired cine and phase-contrast cardiovascular magnetic resonance (CMR) images yielded right ventricular (RV) diastolic parameters which demonstrated good agreement with transthoracic echocardiography (TTE) measurements. • There was a high prevalence of RV diastolic function impairment in pediatric patients with repaired tetralogy of Fallot (rTOF). • CMR is a reliable complementary modality of TTE for RV diastolic function evaluation.

Leaf grey spot caused by Arcopilus aureus on tobacco in China.

Tobacco (Nicotiana tabacum L.) is one of the most widely cultivated industrial crops worldwide. From April to July 2023, about 40% of tobacco seedlings in the greenhouse exhibited irregular taupe lesions in Zhengzhou, Henan Province, China. At an early stage of the lesion development, light grey spots with the diameter of 1-2 mm were observed, these spots gradually expanded and connected into large irregular lesions causing leaf wrinkling or withered. A total of 12 infected leaf tissues were sterilized with 75% ethanol for 45 s, rinsed three times in sterilized water and then plated on potato dextrose agar (PDA) medium for 10 days at 28°C in darkness. Seven fungal colonies that show the similar appearance were isolated and three of them (MB-1, MB-2 and MB-3) were used for subsequent identification. Colonies of these strains on PDA with loose mycelium and orange-red pigment on the underside, white aerial in the center and light yellow hyphae near the periphery, formed in the shape of a concentric ring pattern. Ascomata appeared from the 14th day, were black, spherical or ellipsoid with walls of textura angularis, and size was 53.8-101.1 µm × 50.3-104.3 µm (n=30). Terminal hairs were brown and straight, gradually tapering toward the tips. Asci clavate or fusiform, spore bearing part 16.2-29.2 × 7.3-11.4 µm (n=21), with 8 irregularly arranged ascospores, evanescent. Ascospores are brown at maturity, biapiculate, navicular or fusiform shapes with size of 8.7-12.8 µm × 4.8-6.9 µm (n=100), and more or less inaequilateral. Single spore strains derived from these strains exhibited the morphological features consistent with the original strains. The morphological characteristics of the fungus were consistent with the description of Arcopilus aureus (Chivers) X.W. Wang & Samson (= Chaetomium aureum Chivers) (Lee et al. 2019). Furthermore, the sequences of RPB2 region were amplified from these strains and the result sequences (GenBank accession no. OR513105-OR513108) all showed a 100.00% identity with A. aureus strain CBS 538.73 (GenBank accession no. KX976807.1). It was reported that the RPB2 gene was efficient in discriminating Arcopilus species (Tavares et al. 2022), thus a maximum likelihood (ML) phylogenetic tree based on the RPB2 gene sequences were constructed using MEGA 7.0 with 1000 replications of bootstrapping (Kumar et al. 2016), which revealed that these strains formed a well-supported clade with A. aureus strains of (CBS 153.52 and CBS538.73) (Wang et al. 2022). Pathogenicity analysis were performed on healthy flue-cured tobacco seedlings leaves (cv Y85) by using mycelial agar plugs (5 mm in diameter) and spore suspension (1×106 spores/mL), and the PDA plugs and sterile water were used for control group, respectively. Tobacco seedlings were incubated in a 25°C and 70% RH growth chamber. After seven days, the leaves showed obvious symptoms, with taupe lesions and yellow halos on the periphery, whereas no symptoms were found on the control leaves. The A. aureu was then reisolated from inoculated diseased leaves. Previously, A. aureus has been only reported to cause leaf black disease on Pseudostellaria heterophylla in China (Yuan et al. 2021). To our knowledge, this is the first reported of A. aureus causing tobacco leaf grey spot worldwide. Arcopilus aureus has been reported as a plant biocontrol fungus (Wang et al. 2013). However, due to the potential serious damage in tobacco seedlings caused by this fungus, the use of A. aureus as a plant biocontrol agent needs to be given more attention, and disease control measures of this pathogen should be developed.

Data-driven discovery of a universal indicator for metallic glass forming ability.

Despite the importance of glass forming ability as a major alloy characteristic, it is poorly understood and its quantification has been experimentally laborious and computationally challenging. Here, we uncover that the glass forming ability of an alloy is represented in its amorphous structure far away from equilibrium, which can be exposed by conventional X-ray diffraction. Specifically, we fabricated roughly 5,700 alloys from 12 alloy systems and characterized the full-width at half-maximum, Δq, of the first diffraction peak in the X-ray diffraction pattern. A strong correlation between high glass forming ability and a large Δq was found. This correlation indicates that a large dispersion of structural units comprising the amorphous structure is the universal indicator for high metallic glass formation. When paired with combinatorial synthesis, the correlation enhances throughput by up to 100 times compared to today's state-of-the-art combinatorial methods and will facilitate the discovery of bulk metallic glasses.

Hierarchical Structures Composed of Cu(OH)2 Nanograss within Directional Microporous Cu for Glucose Sensing.

Cu(OH)2 nanomaterials are widely investigated for non-enzymatic glucose sensors due to their low-cost and excellent performance. Cu(OH)2 nanomaterials usually grow on substrates to form sensor electrodes. Reported works mainly focus on structure adjusting of the Cu(OH)2 nanostructures, while the optimization of substrates is still lacking. In the present work, directional porous Cu (DPC) was applied as the substrate for the growth of Cu(OH)2 nanograss (NG), and hierarchical structures of Cu(OH)2@DPC were prepared by alkaline oxidation. The morphology and microstructure evolution of the prepared hierarchical structures was investigated, and the non-enzymatic glucose sensing performance was evaluated. Cu(OH)2@DPC exhibits enhanced comprehensive non-enzymatic glucose sensing performance compared to the reported ones, which may benefit from both the effective adsorption of the Cu(OH)2 NG with a relatively high surface area and the high solute exchange of the DPC by a channel effect. This work provides new insights into the further improvement of the non-enzymatic glucose sensing performance of Cu(OH)2 nanostructures by optimizing the substrate structure.

Ventricular flow analysis and its association with exertional capacity in repaired tetralogy of Fallot: 4D flow cardiovascular magnetic resonance study.

BACKGROUND: Four-dimensional (4D) flow cardiovascular magnetic resonance (CMR) allows quantification of biventricular blood flow by flow components and kinetic energy (KE) analyses. However, it remains unclear whether 4D flow parameters can predict cardiopulmonary exercise testing (CPET) as a clinical outcome in repaired tetralogy of Fallot (rTOF). Current study aimed to (1) compare 4D flow CMR parameters in rTOF with age- and gender-matched healthy controls, (2) investigate associations of 4D flow parameters with functional and volumetric right ventricular (RV) remodelling markers, and CPET outcome. METHODS: Sixty-three rTOF patients (14 paediatric, 49 adult; 30 ± 15 years; 29 M) and 63 age- and gender-matched healthy controls (14 paediatric, 49 adult; 31 ± 15 years) were prospectively recruited at four centers. All underwent cine and 4D flow CMR, and all adults performed standardized CPET same day or within one week of CMR. RV remodelling index was calculated as the ratio of RV to left ventricular (LV) end-diastolic volumes. Four flow components were analyzed: direct flow, retained inflow, delayed ejection flow and residual volume. Additionally, three phasic KE parameters normalized to end-diastolic volume (KEiEDV), were analyzed for both LV and RV: peak systolic, average systolic and peak E-wave. RESULTS: In comparisons of rTOF vs. healthy controls, median LV retained inflow (18% vs. 16%, P = 0.005) and median peak E-wave KEiEDV (34.9 µJ/ml vs. 29.2 µJ/ml, P = 0.006) were higher in rTOF; median RV direct flow was lower in rTOF (25% vs. 35%, P < 0.001); median RV delayed ejection flow (21% vs. 17%, P < 0.001) and residual volume (39% vs. 31%, P < 0.001) were both greater in rTOF. RV KEiEDV parameters were all higher in rTOF than healthy controls (all P < 0.001). On multivariate analysis, RV direct flow was an independent predictor of RV function and CPET outcome. RV direct flow and RV peak E-wave KEiEDV were independent predictors of RV remodelling index. CONCLUSIONS: In this multi-scanner multicenter 4D flow CMR study, reduced RV direct flow was independently associated with RV dysfunction, remodelling and, to a lesser extent, exercise intolerance in rTOF patients. This supports its utility as an imaging parameter for monitoring disease progression and therapeutic response in rTOF. Clinical Trial Registration https://www.clinicaltrials.gov . Unique identifier: NCT03217240.

A novel victorivirus isolated from the tobacco spot blight fungus Stagonosporopsis cucurbitacearum in China.

Stagonosporopsis cucurbitacearum is an important plant-pathogenic fungus that causes stem and leaf blight diseases in a variety of crops. Here, we report the characterization of a novel victorivirus, tentatively named "Stagonosporopsis cucurbitacearum victorivirus 1" (ScVV-1), isolated from the S. cucurbitacearum isolate M-7. The ScVV-1 genome is 5,165 bp in length with a predicted GC content of 60.1% and contains two large open reading frames (ORF 1 and ORF2) encoding putative proteins that share significant sequence similarity with coat proteins (CPs) and RNA-dependent RNA polymerases (RdRps) of mycoviruses of the family Totiviridae. The ScVV-1 RdRp appears to be translated using a stop-initiation pentanucleotide UAAUG sequence. Phylogenetic analysis based on CP and RdRp amino acid (aa) sequences both indicated that ScVV-1 belongs to the genus Victorivirus in the family Totiviridae. To our knowledge, this is the first full-length genome sequence of a victorivirus infecting S. cucurbitacearum.

Highly sensitive time-resolved fluoroimmunoassay for the quantitative onsite detection of Alternaria longipes in tobacco.

AIMS: Alternaria longipes is a causal agent of brown spot of tobacco, which remains a serious threat to tobacco production. Herein, we established a detection method for A. longipes in tobacco samples based on the principle of time-resolved fluoroimmunoassay, in order to fulfil the requirement of rapid, sensitive and accurate detection in situ. METHODS AND RESULTS: A monoclonal antibody against A. longipes was generated, and its purity and titration were assessed using western blot and ELISA. The size of europium (III) nanospheres was measured to confirm successful antibody conjugation. The method described here can detect A. longipes protein lysates as low as 0.78 ng ml-1 , with recovery rates ranging from 85.96% to 99.67% in spiked tobacco. The specificity was also confirmed using a panel of microorganisms. CONCLUSIONS: The fluorescent strips allow rapid and sensitive onsite detection of A. longipes in tobacco samples, with high accuracy, specificity, and repeatability. SIGNIFICANCE AND IMPACT OF THE STUDY: This novel detection method provides convenience of using crude samples without complex procedures, and therefore allows rapid onsite detection by end users and quick responses towards A. longipes, which is critical for disease control and elimination of phytopathogens.

Detection of persistent systolic and diastolic abnormalities in asymptomatic pediatric repaired tetralogy of Fallot patients with preserved ejection fraction: a CMR feature tracking study.

OBJECTIVES: A fast cardiovascular magnetic resonance (CMR) feature tracking was applied to assess ventricular systolic and diastolic function. This study sought to detect right ventricular (RV) systolic and diastolic abnormalities in asymptomatic pediatric repaired tetralogy of Fallot (rTOF) patients with preserved RV ejection fraction (EF). METHODS: One hundred asymptomatic pediatric rTOF patients with preserved RVEF ≥ 45% and 52 control subjects underwent cine CMR examinations. Tricuspid annular plane systolic excursion (TAPSE); peak tricuspid annular systolic (Sm), early diastolic (Em), and late diastolic (Am) velocities; and biventricular global radial (GRS), circumferential (GCS), and longitudinal strains (GLS) were analyzed using CMR feature tracking. RESULTS: TAPSE, Sm, Em, Am, and RV GLS were significantly lower in rTOF patients compared with controls (all p < 0.01). The lower limits (mean-2·standard deviations) of TAPSE, Sm, Em, and Am among controls were 10.9 mm, 6.3 cm/s, 8.9 cm/s, and 2.4 cm/s, respectively, and 78%, 75%, 75%, and 19% of rTOF patients had corresponding measurements below these thresholds. Among rTOF patients, RV GLS was significantly lower in females than in males (p < 0.05). CONCLUSIONS: Despite preserved RVEF, there was a high prevalence of RV systolic and diastolic dysfunction among pediatric rTOF patients, which was detected using fast CMR feature tracking. KEY POINTS: • There was high prevalence of systolic and diastolic dysfunction in asymptomatic pediatric repaired tetralogy of Fallot (rTOF) patients despite preserved right ventricular (RV) ejection fraction (EF). • Significant correlations were observed between right ventricular (RV) measurements (strains, tricuspid annular plane systolic excursion (TAPSE), peak tricuspid annular early diastolic velocity (Em), peak tricuspid annular late diastolic velocity (Am)), and left ventricular (LV) strain measurements, which indicates ventricular-ventricular interactions at systolic and diastolic function level. • Right ventricular (RV) global longitudinal strain (GLS) was lower in female repaired tetralogy of Fallot (rTOF) patients than in males, suggesting females with rTOF may be at a higher risk of developing RV systolic dysfunction than males.

Feasibility of Cardiac Computed Tomography for the Evaluation of Ventricular Function in Postoperative Children With Congenital Heart Disease: Comparison With Cardiac Magnetic Resonance Imaging.

OBJECTIVE: We explored the feasibility of cardiac computed tomography (CCT) to evaluate postoperative ventricular function in children with congenital heart disease (CHD) and evaluated the accuracy and reproducibility of CCT using cardiac magnetic resonance (CMR) as a reference. METHODS: Thirty-two postoperative children with CHD (20 boys and 12 girls) who underwent CMR and CCT were enrolled. Left and right ventricular ejection fraction, end-diastolic volume, end-systolic volume, stroke volume, and cardiac index were measured using cardiac function analysis software. Cardiac function data were compared between CMR and CCT. The agreement between the 2 modalities was assessed using a Bland-Altman analysis. Intraclass correlation coefficients were used to assess intraobserver and interobserver reproducibility in CCT functional measurements. RESULTS: All functional parameters showed no significant difference (P > 0.05) and were well-correlated (r > 0.5, P < 0.05) between CMR and CCT. The mean values of all ventricular function parameters in CCT were higher compared with CMR. As indicated by 95% limits of agreement, left ventricular function parameters showed a better level of agreement compared with right ventricular function parameters between the 2 modalities. Intraobserver and interobserver reproducibility were excellent in CCT measurements for all functional parameters (intraclass correlation coefficient > 0.9). CONCLUSIONS: Compared with the criterion standard of CMR, CCT is feasible for assessing postoperative ventricular function with sufficient diagnostic accuracy and reproducibility in children with CHD. In addition to its important role regarding anatomical characterization, CCT is a suitable alternative and convenient follow-up tool that can be used to functional evaluation in children who are intolerant with CMR or have contraindications to CMR.

Right and left ventricular function and flow quantification in pediatric patients with repaired tetralogy of Fallot using four-dimensional flow magnetic resonance imaging.

BACKGROUND: To assess the accuracy and reproducibility of right ventricular (RV) and left ventricular (LV) function and flow measurements in children with repaired tetralogy of Fallot (rTOF) using four-dimensional (4D) flow, compared with conventional two-dimensional (2D) magnetic resonance imaging (MRI) sequences. METHODS: Thirty pediatric patients with rTOF were retrospectively enrolled to undergo 2D balanced steady-state free precession cine (2D b-SSFP cine), 2D phase contrast (PC), and 4D flow cardiac MRI. LV and RV volumes and flow in the ascending aorta (AAO) and main pulmonary artery (MPA) were quantified. Pearson's or Spearman's correlation tests, paired t-tests, the Wilcoxon signed-rank test, Bland-Altman analysis, and intraclass correlation coefficients (ICC) were performed. RESULTS: The 4D flow scan time was shorter compared with 2D sequences (P < 0.001). The biventricular volumes between 4D flow and 2D b-SSFP cine had no significant differences (P > 0.05), and showed strong correlations (r > 0.90, P < 0.001) and good consistency. The flow measurements of the AAO and MPA between 4D flow and 2D PC showed moderate to good correlations (r > 0.60, P < 0.001). There was good internal consistency in cardiac output. There was good intraobserver and interobserver biventricular function agreement (ICC > 0.85). CONCLUSIONS: RV and LV function and flow quantification in pediatric patients with rTOF using 4D flow MRI can be measured accurately and reproducibly compared to those with conventional 2D sequences.

Preoperative Morphological Prediction of Early Reoperation Risk After Primary Repair in Tetralogy of Fallot: A Contemporary Analysis of 83 Cases.

This study was conducted to investigate the pulmonary artery (PA) variations in tetralogy of Fallot (TOF) and preoperative morphological predictors for early reoperation. Eighty-three TOF patients and 20 children with normal PA were included. The TOF group was divided into two subsets according to whether or not reoperation was performed within 3 years postoperatively. Clinical information was obtained, along with computed tomography (CT)-based three-dimensional geometry of the PA. Morphological measurements of the length of the main PA branches, the angles between them, and the cross-sectional area of each segment of the PAs were acquired using computer software. Logistic regression and receiver operating characteristic curves were applied to analysis. The TOF group showed a significantly smaller PA size and irregular PA shape, with lower Nakata and McGoon indices, than the control group. The median bifurcation angle (angle-γ) was greater than 100° in the TOF group, as compared to 66.70° in the control group (P < 0.000). Residual obstruction of the infundibulum or PAs was the main reason for early reoperation in this series. The development of the main PA and left PA was poorer in the reoperation subset than in the non-reoperation subset (P ≤ 0.01). The preoperative angle-γ in the reoperation subset was larger than that in the non-reoperation subset (median, 117.8° vs. 112.0°, P = 0.026). Higher weight (OR = 0.372) and McGoon index (OR = 0.122) were protective factors, while larger angle-γ (> 114.8°, OR = 5.040) and angle-γ normalized by body surface area (BSA) (γ/BSA > 297.9, OR = 18.860) were risk factors. This study provides an intuitive perspective of PA anatomical variations in TOF. Larger preoperative PA bifurcation angle and γ/BSA were morphological risk predictors of postoperative reoperation in patients with TOF.

Right and left ventricular interactions, strain, and remodeling in repaired pulmonary stenosis patients with preserved right ventricular ejection fraction: A cardiac magnetic resonance study.

BACKGROUND: Right ventricular dilation and dysfunction is a common long-term complication in patients with repaired pulmonary stenosis (rPS). Additionally, abnormal right and left ventricular interactions have been reported in right-sided heart defect after intervention, including in pulmonary stenosis. PURPOSE: To analyze ventricular strain, remodeling, and left and right ventricular interactions in rPS patients with preserved right ventricular ejection fraction (RVEF) compared with healthy children using cardiac magnetic resonance. STUDY TYPE: A cross-sectional study. POPULATION: In all, 34 rPS patients and 10 healthy children volunteers (controls). FIELD STRENGTH/SEQUENCE: 3.0T/2D balanced steady-state free precession (2D b-SSFP) cine, late gadolinium enhancement (LGE), and 2D phase contrast (2D-PC). ASSESSMENT: Pulmonary regurgitation (PR) fractions of the main pulmonary artery, biventricular volumes, masses, function, and cardiac strain. STATISTICAL TESTS: Mann-Whitney U-test, t-test, Pearson correlation coefficients, Spearman's correlation coefficients, and intraclass correlation coefficients analysis were performed. RESULTS: For group analysis, the right ventricular (RV) global circumferential strain and radial strain were significantly increased in patients when compared with controls (-13.57 ± 2.69 vs. -5.91 ± 3.16, P < 0.001; 25.31 ± 8.12 vs. 9.87 ± 5.32, P < 0.001, respectively). The fraction of PR displayed moderate correlation with right ventricular end-diastolic volume index (RVEDVi) (r = 0.452, P = 0.022). RVEDVi and mass index were larger in patients vs. control (104.92 ± 27.46 vs. 85.15 ± 11.98, P = 0.016; 18.28 ± 4.95g/m2 vs. 11.67 ± 2.14 g/m2 , P < 0.001, respectively). Patients presented with preserved left ventricular ejection function, but was lower than healthy controls (60.89% ± 4.89% vs. 65.95% ± 4.56%, P = 0.006). Regional circumferential strain of segment 3 of left ventricle (LV) were significantly decreased in patients (-7.79 ± 6.52 vs. -13.56 ± 3.22, P = 0.003). DATA CONCLUSION: Compensated increased RV strain, myocardial remodeling of RV, and adverse right and left ventricular interactions occur in rPS patients with preserved RVEF. The decreased interventricular septum strain may lead to impaired LV function due to RV dilation as a result of PR. LEVEL OF EVIDENCE: 3 Technical Efficacy Stage: 3 J. Magn. Reson. Imaging 2020;52:129-138.

Quantitative comparison of two-dimensional and three-dimensional strain measurement using MRI feature tracking in repair Fontan patients and normal child volunteers.

BACKGROUND: The accuracy of 2D and 3D strain analyses was evaluated by comparing strain and cardiac function parameters in Fontan repair patients and normal child volunteers. METHODS: We retrospectively enrolled 32 patients with Fontan circulation and 32 child volunteers who had undergone clinical cardiac magnetic resonance (CMR) assessment of the dominant ventricle with a 1.5-Tesla MRI scanner. Global and regional strain (2D and 3D) of the dominant ventricle in both groups was assessed using CMR feature-tracking. Correlations between cardiac function and strain data were assessed using Pearson's correlation coefficient values. The intraclass correlation coefficient (ICC) and coefficient of variation (CoV) were determined to evaluate repeatability and agreement. RESULTS: The 2D GLS showed significant differences between the Fontan repair patients and volunteers (- 16.49 ± 5.00 vs. -19.49 ± 2.03; p = 0.002). The 2D GRS and 2D GCS showed no significant differences between two groups. 2D GRS: 38.96 ± 14.48 vs. 37.46 ± 7.77; 2D GCS: - 17.64 ± 5.00 vs. -16.89 ± 2.96, respectively; p > 0.05). The 3D global radial strain (GRS), global circumferential strain (GCS), and global longitudinal strain (GLS) showed significant differences between the Fontan repair patients and volunteers (3D GRS: 36.35 ± 16.72 vs. 44.96 ± 9.98; 3D GLS: - 8.86 ± 6.84 vs. -13.67 ± 2.44; 3D GCS: - 13.70 ± 7.84 vs. -18.01 ± 1.78; p < 0.05, respectively). The ejection fraction (EF) and 3D GCS were significantly associated (r = - 0.491, p = 0.004). The 3D GCS showed correlations with the indexed end-diastolic volume (EDV) (r = 0.523, p = 0.002) and indexed end-systolic volume (ESV) (r = 0.602, p < 0.001). 3D strain showed good reproducibility, with GCS showing the best inter-observer agreement (ICC = 0.87 and CoV = 5.15), followed by GLS (ICC = 0.84 and CoV = 5.36). CONCLUSIONS: 3D GCS is feasible, highly reproducible, and strongly correlated with conventional cardiac function measures. 3D GCS assessments may be useful for monitoring abnormal myocardial motion in patients with Fontan circulation.

Early open reduction of dislocated hips using a modified Smith-Petersen approach in arthrogyposis multiplex congenita.

BACKGROUND: Arthrogryposis multiplex congenita (AMC) is a rare syndrome with multiple joint contractures. Within the medical community, there is controversy surrounding AMC in terms of the ideal surgical approach and age for performing a reduction of dislocated hips. The purpose of this retrospective study was to evaluate the clinical outcomes of early open reduction of infant hip dislocation with arthrogryposis multiplex congenita following a modified Smith-Petersen approach that preserves the rectus femoris. METHODS: From 2010 to 2017, we performed this procedure on 28 dislocated hips in 20 infants under 12 months of age with AMC. The clinical and radiology data were reviewed retrospectively. The mean age at surgery was 6.9 ± 5.1 months, with a mean follow-up of 42.4 ± 41.1 months. RESULTS: After open reduction, the average hip acetabular index (AI), the international hip dysplasia institute classification (IHDI), and the hip range of motion significantly improved (all P < 0.001). After the surgery, 16 patients were community walkers, and four patients were home walkers. Three hips in two patients required secondary revision surgery for residual acetabular dysplasia with combined pelvic osteotomy and femoral osteotomy. Seven of the hips that had been operated on showed signs of avascular necrosis (AVN). Among them, four were degree II, two were degree III, and one was degree IV. Multiple linear regression analysis demonstrated that greater age (in months) heightened the risk for secondary revision surgery (P = 0.032). CONCLUSIONS: The modified Smith-Petersen approach preserving the rectus femoris is an encouraging and safe option for treating hip dislocation in young AMC patients (before 12 months). If surgery takes place at less than 12 months of age for patients with AMC, this earlier open reduction for hip dislocation may reduce the chances of secondary revision surgery. LEVEL OF EVIDENCE: IV, retrospective non-randomized study.

Hip morphology in mucopolysaccharidosis type IVA through radiograph, magnetic resonance imaging and arthrogram assessment.

PURPOSE: This study examined the hip morphology of paediatric patients with mucopolysaccharidosis (MPS) type IVA (MPS IVA). METHODS: This was a retrospective chart review of 42 hips in 21 children with MPS IVA. Pelvic radiographs and magnetic resonance imaging (MRI) scans of 42 hips and arthrograms of 13 hips were analysed. The bony, cartilaginous and labral coverage of the acetabulum was determined by acetabular index (AI), centre edge angle (CEA) and femoral head coverage (FHC). RESULTS: The mean age at the time of radiography was 66.3 ± 21.7 months. The bony, cartilaginous and labral AI in the MRI assessment were 36.3 ± 5.3, 18.3 ± 4.7 and 12.1 ± 4.6 degrees, respectively. The inter-class correlation coefficients (ICCs) for the bony AI, CEA and FHC measurements on radiographs and MRI were 0.936, 0.879 and 0.810, respectively. In the MRI assessment, labrum in 12 of 42 hips appeared as a regular triangle, and it was flat on 30/42 hips. The average arthrographic AI (AAI) was 11.1 ± 2.7 degrees. The ICCs value of AAI versus cartilaginous and labral AI on MRI indicates good agreement but higher in labral AI. CONCLUSION: Hips in MPS IVA exhibited obvious cartilage and labrum compensation in response to abnormal ossification of bony acetabulum. Cartilage in MPS IVA hip increases the thickness in the longitudinal direction, while the labrum becomes flatten in the horizontal direction. The AAI may represent intraoperative labrum coverage. The femora-acetabular harmony is difficult to determine using radiography only, and pre-operative MRI and an intraoperative arthrogram are very important in a hip assessment in MPS IVA.

Diagnostic accuracy of multi-slice computed tomography in children with Abernethy malformation.

BACKGROUND: Abernethy malformation is a rare congenital abnormality. Imaging examination is an important method for the diagnosis. The purpose of this study was to demonstrate manifestations of multi-slice computed tomography (MSCT) in Abernethy malformation and its diagnostic accuracy. METHODS: Fourteen children with Abernethy malformation were admitted to our center in China between July 2011 and January 2018. All 14 patients (eight males and six females) received MSCT and digital subtraction angiography (DSA) while eight patients also received ultrasound. The patients' age ranged from 1 to 14 (median age 8 years old). The clinical records of the patients were retrospectively reviewed. MSCT raw data were transferred to an Advantage Windows 4.2 or 4.6 workstation (General Electric Medical Systems, Waukesha, WI). We compared the findings of MSCT with DSA and surgical results in order to ascertain diagnostic accuracy. RESULTS: Three cases had type Ib Abernethy malformation and eleven cases had type II. Two cases of type II Abernethy malformation were misdiagnosed as type Ib using MSCT. Comparing the findings of MSCT with DSA and surgical results, the accuracy of MSCT was 85.7% (12/14), in which 100.0% (3/3) were type Ib and 81.8% (9/11) were type II. Clinical information included congenital heart disease, pulmonary hypertension, diffuse pulmonary arteriovenous fistula, abnormal liver function, hepatic nodules, elevated blood ammonia, and hepatic encephalopathy. Eleven cases were treated after diagnosis. One patient with Abernethy malformation type Ib (1/3) underwent liver transplantation. Seven patients with Abernethy malformation type II (7/11) were treated by shunt occlusion, received laparoscopy, or were treated with open surgical ligation. Another three patients (3/11) with Abernethy malformation type II were treated by interventional portocaval shunt occlusion under DSA. CONCLUSION: MSCT attains excellent capability in diagnosing type II Abernethy malformation and further shows the location of the portocaval shunt. DSA can help when it is hard to determine diagnosis between Abernethy type Ib and II using MSCT.

The changes in, and relationship between, plasma nitric oxide and corticotropin-releasing hormone in patients with major depressive disorder.

There is strong evidence of roles of the hypothalamus-pituitary-adrenal axis and nitric oxide (NO) synthase-NO system in depression, but the relationship between them is unknown. The aim of this study, therefore, was to elucidate whether there is any correlation between NO and corticotropin-releasing hormone (CRH) in major depressive disorder (MDD) patients. In 16 outpatients with MDD and 18 healthy controls, the plasma amino acids citrulline (Cit) and arginine (Arg) were determined by high-performance liquid chromatography, and CRH levels was measured by radioimmunoassay. The Cit/Arg ratio was calculated as an index of NO synthesis. Correlations between NO and CRH were examined with the Spearman test. Before treatment, no significant correlation was observed between the plasma NO level and CRH levels in MDD patients. The plasma NO levels were significantly higher in MDD patients. A significant correlation was found between NO levels and Hamilton Depression Rating Scale (HAMD) scores in MDD patients. The plasma CRH levels were significantly higher in MDD patients than in controls. After monotherapy for 2 months, the NO levels had dramatically declined but were also higher than those in the controls. This study is the first report of the absence of a significant correlation between plasma NO and CRH levels, although both levels are elevated in MDD patients. Furthermore, the strong links between the plasma NO levels and the HAMD scores, as well as the increased NO reduction after remission, suggest that NO plays a key role in depression and may be an indicator of therapeutic success.

Carbon Monoxide Potentiates High Temperature-Induced Nicotine Biosynthesis in Tobacco.

Carbon monoxide (CO) acts as an important signal in many physiological responses in plants, but its role in plant secondary metabolism is still unknown. Nicotine is the main alkaloid generated in tobacco and the plant hormone jasmonic acid (JA) has previously been reported to efficiently induce its biosynthesis. Whether and how CO interacts with JA to regulate nicotine biosynthesis in tobacco remains elusive. In this study, we demonstrate that high temperature (HT) induces quick accumulation of nicotine in tobacco roots, combined with an increase in CO and JA concentration. Suppressing CO generation reduced both JA and nicotine biosynthesis, whereas exogenous application of CO increased JA and nicotine content. CO causes an increased expression of NtPMT1 (a key nicotine biosynthesis enzyme), via promoting NtMYC2a binding to the G-box region of its promoter, leading to heightened nicotine levels under HT conditions. These data suggest a novel function for CO in stimulating nicotine biosynthesis in tobacco under HT stress, through a JA signal.

Multislice Computed Tomography Assessment of Tracheobronchial Patterns in Partial Anomalous Left Pulmonary Artery.

OBJECTIVE: The aim of this study was to present relationship between partial anomalous left pulmonary artery (PALPA) and the tracheobronchial tree and patterns of the tracheobronchial tree assessed by multislice computed tomography (MSCT). METHODS: Nine patients were assessed by MSCT. The relationships between the tracheobronchial tree and PALPA and different tracheobronchial patterns, location of tracheobronchial stenosis, severity of stenosis, and associated cardiac defects were evaluated. The results of MSCT for these patients were compared with the operative findings. RESULTS: The anatomy of PALPA was clearly identified by MSCT in all 9 patients. Three relationships between PALPA and the tracheobronchial tree were noted. In addition, 3 patterns of tracheobronchial tree anatomy were also demonstrated. The PALPA arose from the right pulmonary artery, forming a pulmonary sling (n = 2). The PALPA, which arose from the proximal right pulmonary artery, went below the tracheal bifurcation and passed anterior (n = 1) or inferior-anterior (n = 6) to the proximal left main bronchus. Three patterns of the tracheobronchial tree were presented with normal (n = 5), normal pattern with right tracheal bronchus (n = 3), and bridging bronchus (n = 1). The rate of tracheobronchial stenosis was 56% (5/9).Five patients underwent operation, and at that time, the relationship between PALPA and the tracheobronchial tree defined on MSCT was confirmed. CONCLUSIONS: The PALPA can be associated with tracheobronchial anomalies and airway compression depending on its orientation to the airway. Noninvasive imaging modalities such as MSCT will be helpful for making further management decisions.