Discordant renal progression of Fabry disease in male monozygotic twins: a case report.

Fabry disease (FD) is a rare X-linked lysosomal storage disease caused by mutations in the gene that encodes α-galactosidase A (α-GAL). Clinical phenotypes tend to vary in monozygotic female twins because mutations are located on the X-chromosome, whereas similar phenotypes are found in male monozygotic twins. Here we report the case of male monozygotic twins with FD presenting with distinguishable renal phenotypes.

Complete plastid genome of (Ceramiales, Rhodophyta).

We report the complete plastid genome of also known as . The plastid genome was 174,192 bp in size. Annotation showed there were 193 protein coding genes, three ribosomal RNAs, and 29 transfer RNAs.

Comparative Outcome Analysis of N-Butyl Cyanoacrylate Embolization of the False Lumen Versus Thoracic Endovascular Aortic Repair in Aortic Dissection.

Purpose: To evaluate the feasibility, safety, and effectiveness of N-butyl cyanoacrylate (NBCA) embolization for the treatment of aortic dissection.

Materials And Methods: In this single-center retrospective study conducted from February 2003 to June 2019, NBCA embolization of an aortic false lumen was attempted in 12 patients (median age, 59 y; range, 41-68 y) and thoracic endovascular aortic repair (TEVAR) was performed in 53 patients (median age, 59 y; range, 37-70 y) for aortic dissection with one or more indications of persisting pain, malperfusion, rupture or impending rupture, maximal aortic diameter ≥ 55 mm, and/or rapid aortic enlargement. The main exclusion criterion for embolization was the presence of fast blood flow in the aortic false lumen on aortography.

Aortic Remodeling and Clinical Outcomes in Type B Aortic Dissection According to the Timing of Thoracic Endovascular Aortic Repair.

Background: The objective of the study was to investigate aortic remodeling and clinical outcomes after thoracic endovascular aortic repair (TEVAR) for type B aortic dissection (AD) according to timing of the procedure.

Methods: A total of 87 patients with type B AD who underwent TEVAR at 2 centers were included in this retrospective analysis. Patients were divided into acute/subacute (≤6 weeks, n = 35), early chronic (6 weeks to 1 year, n = 20), and late chronic (>1 year, n = 32) groups according to the timing of TEVAR after symptom onset.

Development and validation of a prognostic model for patients with hepatocellular carcinoma undergoing radiofrequency ablation.

Background: There are large variations in prognosis among hepatocellular carcinoma (HCC) patients undergoing radiofrequency ablation (RFA). However, current staging or scoring systems hardly discriminate the outcome of HCC patients treated with RFA.

Methods: A total of 757 treatment-naïve HCC patients undergoing RFA (derivation cohort) were analyzed to establish a nomogram for disease-free survival (DFS) based on Cox proportional hazard regression model.

Safety and Efficacy of Percutaneous Cryoablation for Small Hepatocellular Carcinomas Adjacent to the Heart.

Purpose: This study evaluated the safety and efficacy of percutaneous cryoablation for treatment of the left subdiaphragmatic small hepatocellular carcinomas (HCCs) adjacent to the heart.

Materials And Methods: Between September 2013 and March 2018, 189 consecutive patients underwent cryoablation for small HCCs (≤3 cm); 70 patients (mean: 61.3 ± 10.

Late complications after hybrid aortic arch repair.

Objective: Although hybrid arch repair has gained widespread application as an alternative option for high-risk patients, concerns about its long-term durability remain an important issue. The aim of this study was to investigate late complications after hybrid arch repair.

Methods: From January 2002 to December 2017, hybrid arch repair was performed in 65 patients with aortic arch disease (median age, 66.

The Effect of Fibronectin-Immobilized Microgrooved Titanium Substrata on Cell Proliferation and Expression of Genes and Proteins in Human Gingival Fibroblasts.

Background: We aimed to determine the effect of fibronectin (FN)-immobilized microgrooved titanium (Ti) on human gingival fibroblast proliferation, gene expression and protein expression.

Methods: Photolithography was used to fabricate the microgrooved Ti, and amine funtionalization (silanization) was used for FN immobilization on titanium surfaces. Cell proliferation, gene expression and protein expression were analyzed, followed by multiple regression analysis for determining the influential factors on cell proliferation.

Five-year clinical outcomes of uterine artery embolization for symptomatic leiomyomas: An analysis of risk factors for reintervention.

Objectives: The objective of this study is to investigate the five-year reintervention rate of uterine artery embolization (UAE) for symptomatic leiomyomas and to identify potential predictive factors of reintervention.

Methods: From March 2011 to February 2012, 92 consecutive women (mean age 41.9 years, range 28-55 years) underwent bilateral UAE.

Clinical Results of Transarterial Embolization to Control Postoperative Vascular Complications after Partial Nephrectomy.

Purpose: We evaluated the safety and clinical efficacy of transarterial embolization of vascular complications after partial nephrectomy.

Materials And Methods: This retrospective study included 1,187 patients who underwent partial nephrectomy between January 2006 and December 2017. A total of 36 patients were referred to the interventional radiology department for vascular complications after partial nephrectomy.

Ablation Volume Measurement After Percutaneous Cryoablation Using a Two-cryo-probe Technique for Small Hepatocellular Carcinomas.

Purpose: To calculate the ablation volume of percutaneous cryoablation (PCA) with a two-cryo-probe technique for small hepatocellular carcinomas (HCCs) and to assess risk factors for local tumor recurrence (LTR).

Materials And Methods: Between January 2013 and June 2017, 96 patients (mean age, 60.7 years; range, 37-83 years) with 106 small HCCs were retrospectively analyzed.

Catheter-directed Sclerotherapy for Ovarian Endometrioma: Short-term Outcomes.

Purpose To evaluate the effectiveness of catheter-directed sclerotherapy (CDS) with 95% ethanol in patients with primary or recurrent ovarian endometriomas. Materials and Methods In this prospective study, 14 participants (mean age, 32 years; range, 20-44 years) who underwent CDS for ovarian endometrioma from March 2015 to December 2017 were evaluated. Diagnosis was based on symptoms and imaging studies.

Comparison of open surgical versus hybrid endovascular repair for descending thoracic aortic aneurysms with distal arch involvement.

Background: Our aim was to compare the efficacies of conventional open thoracotomy and hybrid endovascular technique in patients with descending thoracic aortic aneurysms involving the distal arch.

Methods: Between January 2005 and December 2015, 125 consecutive patients with descending aneurysms involving distal arch underwent open repair via thoracotomy (n=79) or zone 1/2 hybrid endovascular repair (n=46). Surgeries entailing total arch replacement by elephant trunk technique (with sternotomy) and Zone 0 hybrid arch repairs were excluded.

Peripheral artery disease is associated with poor clinical outcome in patients with abdominal aortic aneurysm after endovascular aneurysm repair.

Background: We investigated the effects of coronary artery disease (CAD) or peripheral artery disease (PAD) on clinical outcomes of patients with abdominal aortic aneurysm (AAA) treated with endovascular aortic aneurysm repair (EVAR).

Methods: We retrospectively evaluated a total of 475 patients with AAA treated with EVAR at a single center. Patients were divided into three groups: group A (n = 166), patients without CAD or PAD; group B (n = 196), patients with CAD but without PAD; and group C (n = 113), patients with PAD regardless of CAD.

Transcatheter arterial embolisation for acute lower gastrointestinal haemorrhage: a single-centre study.

Objective: To investigate the safety and efficacy of transcatheter arterial embolisation (TAE) in the management of lower gastrointestinal bleeding (LGIB) and to identify predictors of clinical outcomes.

Methods: Between December 2005 and April 2017, 274 patients underwent diagnostic angiography for signs and symptoms of LGIB; 134 patients with positive angiographic findings were retrospectively analysed. The technical success of TAE and clinical outcomes, including recurrent bleeding, major complications, and in-hospital mortality were evaluated.

Uterine Artery Embolization for Pedunculated Subserosal Leiomyomas: Evidence of Safety and Efficacy.

Purpose: To evaluate safety and efficacy of uterine artery embolization (UAE) for pedunculated subserosal (PS) leiomyomas.

Materials And Methods: Of 1,069 patients who underwent UAE for symptomatic leiomyomas or adenomyosis from 2007 to 2016, 55 patients (mean age 40.3 y ± 4.

Mesocaval Shunt Creation for Jejunal Variceal Bleeding with Chronic Portal Vein Thrombosis.

The creation of transjugular intrahepatic portosystemic shunt (TIPS) is a widely performed technique to relieve portal hypertension, and to manage recurrent variceal bleeding and refractory ascites in patients where medical and/or endoscopic treatments have failed. However, portosystemic shunt creation can be challenging in the presence of chronic portal vein occlusion. In this case report, we describe a minimally invasive endovascular mesocaval shunt creation with transsplenic approach for the management of recurrent variceal bleeding in a portal hypertension patient with intra- and extrahepatic portal vein occlusion.

Safety and Efficacy of Transarterial Radioembolization Combined with Chemoembolization for Bilobar Hepatocellular Carcinoma: A Single-Center Retrospective Study.

Background: Radioembolization induced liver disease (REILD) is a possible sequela of transarterial radioembolization (TARE), particularly in cases of whole-liver treatment. To mitigate this problem, the safety and efficacy of combined transarterial chemoembolization (TACE) and TARE were evaluated for patients with bilobar hepatocellular carcinoma (HCC).

Materials And Methods: Nineteen patients (mean age 60 years; range 27-82 years) treated for HCC between June 2012 and September 2014 were included in the analysis.

Safety and effectiveness of transarterial embolization for splenic artery hemorrhage in patients undergoing radical gastrectomy.

Background Perigastric lymph nodes are dissected during gastrectomy, potentially resulting in life-threatening postoperative bleeding. Purpose To evaluate the safety and effectiveness of transarterial embolization (TAE) for bleeding from the splenic artery in patients who underwent gastrectomy. Material and Methods Between January 2004 and December 2016, 14,523 patients underwent gastrectomy at our institution, and ten patients (nine men; mean age = 64.

Randomized Controlled Trial Comparing Radiologic Pigtail-Retained Gastrostomy and Radiologic Mushroom-Retained Gastrostomy.

Purpose: To prospectively compare 2 different types of percutaneous fluoroscopic gastrostomy procedures (pigtail-retained gastrostomy [PG] vs mushroom-retained gastrostomy [MG]).

Materials And Methods: Between March 2014 and February 2016, 100 patients were randomly assigned to receive 14-F PG or 20-F MG. Block randomization (block size 4) was performed, and sample size was calculated to assess the difference in minor complications.

Effects of anatomical characteristics as factors in abdominal aortic aneurysm rupture: CT aortography analysis.

The aim of this study was to analyze the anatomical characteristics of patients with ruptured abdominal aortic aneurysms (AAAs) using computed tomography (CT) aortography in order to determine the risk factors for rupture.We retrospectively reviewed the CT aortography findings and medical records of patients with ruptured AAAs who underwent CT aortography between February 2002 and December 2014. Age, sex, blood pressure at the time of rupture, treatment methods used for the ruptured AAAs, and treatment outcomes were analyzed.

Safety and Efficacy of Uterine Artery Embolization in Ectopic Pregnancies Refractory to Systemic Methotrexate Treatment: A Single-Center Study.

Background: To investigate the use of uterine artery embolization (UAE) to manage ectopic pregnancies that are refractory to systemic methotrexate (MTX) therapy and plagued by persistently high serum β-human chorionic gonadotrophin (hCG) levels and vaginal bleeding. The safety and efficacy of UAE were addressed.

Materials And Methods: A retrospective review was conducted for thirteen patients (mean age 35.

Clinical Outcomes for Endovascular Repair of Thoracic Aortic Disease Using the Seal Thoracic Stent Graft: A Korean Multicenter Retrospective Study.

Purpose: To investigate the midterm outcomes of thoracic endovascular aneurysm repair (TEVAR) with the use of the Seal stent graft for four categories of thoracic aortic disease.

Materials And Methods: This retrospective multicenter study evaluated the records of 216 Korean patients who underwent TEVAR with the Seal stent graft during 2007-2010. The study outcomes were (i) perioperative death, (ii) endoleak, (iii) repeat intervention, (iv) aortic-related death, and (v) all sudden unexplained late deaths.

Risk factors for stent graft-induced new entry after thoracic endovascular aortic repair for Stanford type B aortic dissection.

Objective: Stent graft-induced new entry (SINE) has been increasingly observed after thoracic endovascular aortic repair (TEVAR) for Stanford type B aortic dissection. SINE is often life threatening, and reintervention is required. This study investigated risk factors for SINE after TEVAR.

Clinical outcome of transarterial embolization for postgastrectomy arterial bleeding.

Background: The aim of this study was to retrospectively investigate the feasibility and safety of transcatheter arterial embolization in the management of postgastrectomy arterial bleeding.

Methods: Between January 2004 and July 2015, 13,246 patients underwent total or subtotal gastrectomy at our institution, and 24 patients (18 men; mean age 66.8 years; range 42-80 years) underwent transcatheter arterial embolization for postoperative arterial bleeding identified on angiography.