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All tested patients with X-linked retinoschisis (XLRS) had the same possibly unique RS1 pathogenic variant, c.441G > A (p.Trp147X). Pathologic variants and genes for IRDs in Iceland did not resemble those described in ancestral North-Western European nations. Four variants were reclassified as likely pathogenic. One novel pathogenic variant defined a genetically homogenous XLRS patient group.

As a specialized intraparenchymal vascular conduit, hepatic sinusoids play a key role in liver microcirculation. This study aimed to explore the three-dimensional (3D) morphological changes of cirrhotic sinusoids by serial histological sections.

Cirrhosis was induced by tail vein injection of albumin in Wistar rats with a positive antibody. A total of 356 serial histological sections were prepared from liver tissue blocks of normal and cirrhotic rats. The optical microscope images were registered and reconstructed, and 3D reconstructions of the fine structures of fibrous tissues and sinusoids were subsequently visualized.

The fibrosis area of the cirrhotic sample was 6-16 times that of the normal sample (P<0.001). Cirrhosis led to obvious changes in the distribution and morphology of sinusoids, which were mainly manifested as dilation, increased quantity and disordered distribution. Compared with normal liver, cirrhotic liver has a significantly increased volume ratio, number and volume of sinusoids (1.63-, 0.53-, and 1.75-fold, respectively, P<0.001). Furthermore, the samples were further divided into three zones according to the oxygen supply, and there were significant differences in the morphology of the sinusoids in the normal and cirrhotic samples (P<0.05). In particular, morphological parameters of the cirrhotic sinusoids near the portal area were obviously greater than those in the normal liver (P<0.05).

3D morphological structures of hepatic sinusoids were reconstructed, and the adaptive microstructure changes of cirrhotic sinusoids were accurately measured, which has an important implications for the study of hepatic microcirculation and pathological changes of cirrhosis.

3D morphological structures of hepatic sinusoids were reconstructed, and the adaptive microstructure changes of cirrhotic sinusoids were accurately measured, which has an important implications for the study of hepatic microcirculation and pathological changes of cirrhosis.

 Air leak is the most common complication after lung resection and leads to increased length of hospital (LOH) stay or patient discharge with a chest tube. Management by autologous blood patch pleurodesis (ABPP) is controversial because few studies exist, and the technique has yet to be standardized.

 We retrospectively reviewed patients undergoing ABPP for prolonged air leak (PAL) following lobectomy in three centers, between January 2014 and December 2019. They were divided into two groups Group A, 120 mL of blood infused; Group B, 60 mL. Propensity score-matched (PSM) analysis was performed, and 23 patients were included in each group. Numbers and success rates of blood patch, time to cessation of air leak, time to chest tube removal, reoperation, LOH, and complications were examined. Univariate and multivariate analysis of variables associated with an increased risk of air leak was performed.

 After the PSM, 120 mL of blood is statistically significant in reducing the number of days before chest tube removal after ABPP (2.78 vs. 4.35), LOH after ABPP (3.78 vs. 10.00), and LOH (8.78 vs. 15.17). Complications (0 vs. 4) and hours until air leak cessation (6.83 vs. 3.91, range 1-13) after ABPP were also statistically different (

 < 0.05). Air leaks that persisted for up to 13 hours required another ABPP. No patient had re-operation or long-term complications related to pleurodesis.

 In our experience, 120 mL is the optimal amount of blood and the procedure can be repeated every 24 hours with the chest tube clamped.

 In our experience, 120 mL is the optimal amount of blood and the procedure can be repeated every 24 hours with the chest tube clamped.

 Though transfemoral (TF) access has emerged as a gold standard access for patients with aortic stenosis who undergo transcatheter aortic valve implantation (TAVI), there has been no study that has characterized patients who cannot undergo TF access in detail. We aim to evaluate the contraindications for TF access, their incidence, classify them, and provide the outcomes of patients who failed to be TF candidates.

 From 925 patients who underwent TAVI between February 2014 and May 2020 at our heart center, 130 patients failed to be TF candidates and underwent transapical-transcatheter aortic valve implantation (TA-TAVI). In this study, we included all those patients who failed to be TF candidates and underwent TA-TAVI using the third-generation balloon expandable valve (Edwards SAPIEN 3 valve [S3]) (116 patients; STS score 6.07 ± 4.4; age 79.4 ± 7).

 The incidence of patients unsuitable for TF access at our heart center was 14%. We classified this TAVI population into absolute contraindication for TF access

 = 84 (72.5%) and increased interventional risk for TF access

 = 32 (27.5%). Caspase activation After TA-TAVI of this specific population using S3, the in-hospital mortality and stroke were 1.7 and 1.7%, respectively. The vascular injury rate was 1.7%. We registered no paravalvular leakage ≥2. The pacemaker rate was 7.4%. The mean transvalvular pressure gradient was 8.7 mm Hg.

 The incidence of patients who cannot undergo TF access or who are at high interventional risk is considerably high. TA-TAVI, supported with sufficient interventional experience and appropriate valve system, represents an excellent alternative for patients with distinct vasculopathy.

 The incidence of patients who cannot undergo TF access or who are at high interventional risk is considerably high. TA-TAVI, supported with sufficient interventional experience and appropriate valve system, represents an excellent alternative for patients with distinct vasculopathy.

 Surgical correction of an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) has been associated with excellent survival during recent years. The purpose of this study was to evaluate the effectiveness of reimplantation of the coronary artery and to investigate the recovery of postoperative cardiac and mitral valve (MV) function.

 From 2005 to 2015, 80 patients who had ALCAPA received surgical correction. Among them, 49 were infants. The median patient age was 7.8 months. Operative strategies included reimplantation of the coronary artery in 71 patients, the Takeuchi procedure in another 7 patients, and coronary artery ligation in the remaining 2 patients.

 There were 11 hospital deaths and 2 late deaths. Six patients required intraoperative or postoperative mechanical circulatory support. A significant improvement in the ejection fraction (EF) and shortening fraction (SF) was present in all surviving patients at discharge, at a 3-month follow-up and at a 1-year follow-up. MV function improved gradually after surgical repair with no late secondary intervention.

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