Hepatic sinusoidal obstruction syndrome after allogeneic hematopoietic stem cell transplantation in adult patients with idiopathic aplastic anemia

We retrospectively investigated the incidence, risk factors, and outcomes of SOS (sinusoidal obstruction syndrome; previously veno-occlusive disease [VOD]) after allogeneic hematopoietic stem cell transplantation (alloHSCT) in aplastic anemia. Two hundred and sixty patients were included in the analysis. SOS developed in 7.3% (n = 19/260) of patients. Classical Cy (200 mg/m²)-ATG was the most common conditioning regimen (84.2%) in the SOS group. The SOS mortality rate was 4/19 (21.1%). Univariate analyses revealed that Cy 200 mg/m² conditioning (p = 0.035), classical Cy-ATG conditioning (p = 0.007), and horse ATG conditioning (p < 0.001) were significant risk factors for developing SOS. Multivariate analysis revealed that only horse ATG conditioning was a poor prognostic factor (HR = 3.484; 95% CI 1.226–9.904; p = 0.002). Rabbit ATG (HR 12.719; 95% CI 2.332–69.373; p = 0.003) and weight gain > 10% (HR 35.655; 95% CI 2.208–575.805; p = 0.012) were risk factors in the overall SOS group. Both rabbit ATG conditioning and weight gain of more than 10% were associated with poor overall survival with a median of 1.2 months (5Y survival rate, any risk factor vs. none: 74.6% vs. 0.0%; p < 0.001; Fig. 2) in the SOS group.     

食管癌术后放疗的临床分析

目的评价食管癌术后放疗的价值。方法1990-1992年，行根治性切除胸段食管瘤106例，其中单纯手术58例，术后放疗48例，同时合并化疗10例。结果单纯手术与术后放疗和／或化疗的2年局部控制率分别为59％和91％，5年生存率为21％和30％。2年局部控制率术后放疗组显著高于单纯手术组（P＜0．01）。5年生存率术后放疗组虽优于单纯手术组，但无显著性差异（P＜0．05）。结论术后放疗是提高食管癌局部控制率的重要手段。     

Clinical and Genetic Characteristics of Korean Patients Diagnosed with Chronic Enteropathy Associated with SLCO2A1 Gene: A KASID Multicenter Study

Background/AimsChronic enteropathy associated with SLCO2A1 gene (CEAS), an inherited disease characterized by nonspecific intestinal ulcers, has emerged in the Japanese population via loss-of-function mutations in the SLCO2A1 gene. We aimed to investigate the clinical and genetic characteristics of Korean patients diagnosed with CEAS.MethodsFrom July 2018 to July 2021, we performed Sanger sequencing of the SLCO2A1 gene in 46 patients with chronic intestinal ulcers. CEAS was confirmed based on known SLCO2A1 mutations. We summarized the clinical characteristics of patients with confirmed CEAS.ResultsFourteen out of 46 patients (30.4%) had genetically confirmed CEAS, and two SLCO2A1 variants were detected (splicing site variant c.940+1G>A and nonsense mutation [p.R603X] in SLCO2A1). Twelve patients (85.7%) were females and the median age at diagnosis of CEAS was 44.5 years. All patients presented with abdominal pain, and 13 patients (92.9%) presented with anemia (median hemoglobin, 9.6 g/dL). Ten patients (71.4%) had hypoalbuminemia (median, 2.7 g/dL). The most commonly involved site was the ileum (13/14, 92.9%). Manifestations of primary hypertrophic osteoarthropathy (PHO), such as digital clubbing, pachydermia, and periostosis were observed in five patients (28.6%) and two male patients and one female patient satisfied all major PHO diagnostic criteria.ConclusionsThe clinical and genetic characteristics of Korean patients with confirmed CEAS were similar to those reported in the literature. CEAS should be considered in the differential diagnosis for patients with unexplained chronic nonspecific ulcers of the small intestine.     

Population Prevalence,Cancer Risk,and Mortality Risk of Turner Syndrome in South Korean Women Based on National Health Insurance Service Data

PurposeIn South Korea, investigations into Turner syndrome (TS) prevalence and TS-associated cancer and mortality are lacking. Accurate data were estimated from the National Health Insurance Service (NHIS) and the Rare Diseases Registry (RDR) records.Materials and MethodsData on patients with TS who were registered in the RDR between 2007 and 2017 were collected. To estimate TS-associated cancer and mortality risk, the data were compared with data of 1:3 age-matched controls.ResultsIn 2017, 2054 patients with TS were identified from a total population of 26186952 South Korean women; therefore, the prevalence was 7.84 per 100000 persons. TS prevalence across 10-year interval age groups were 11.82, 23.17, 18.37, 10.49, 4.09, and 0.38 for age under 10 years, teenagers, 20s, 30s, 40s, and older than 50, respectively (per 100000 persons). The cancer risk in patients with TS was higher than that of age-matched controls over 5.3 person-years [hazard ratio (HR)=1.82, 95% confidence interval (CI) 1.01–3.27, p=0.045]. Among different types of cancer, thyroid cancer risk in patients with TS was significantly higher than that of age-matched controls (HR=2.78, 95% CI 1.06–7.26, p=0.037). We also observed that TS-associated all-cause mortality risk was higher than that of age-matched controls (HR=3.36, 95% CI 1.59–7.10, p=0.002).ConclusionNational prevalence of TS was suggested, and an increased risk of TS-associated thyroid cancer and mortality were observed in this study.     

Pattern Analysis of Laser Fiber Degradation According to the Laser Setting: In Vitro Study of the Double-Firing Phenomenon

BackgroundIt is essential to understand the mechanism of the various causes of laser fiber damage and an ideal method of reducing endoscope damage induced by laser emission in multiple sites. This study classified the different patterns of laser fiber degradation according to laser settings and analyzed the role of cavitation bubbles to find a desirable way of minimizing endoscope damage.MethodsA total of 118 laser fibers were analyzed after 1-,3-, and 5-min laser emission to artificial stones under the settings of 1 J-10 Hz, 1 J-20 Hz, 1 J-30 Hz, and 2 J-10 Hz. Every 3 cm from the fiber tip was marked and examined with a digital microscope and a high-speed camera. The images of the fibers and the movement of cavitation bubbles were taken with a distance of 1 to 5 mm from the gel.ResultsSeven types of fiber damage (charring, limited and extensive peeled-off, bumpy, whitish plaque, crack, and break-off) coincided during laser emission. Damages rapidly increased with emission time > 3 minutes regardless of the laser settings. The damaged lengths covered 5 mm on average, and the fibers at 5-min emission were significantly shorter than others. The fiber durability of 1J-10Hz setting was better than other settings after 3-min laser emission. Backward movement of the cavitation bubbles was found at the 1-mm distance from the gel, and the damaged lengths were longer than the diameters of the cavitation bubbles because of their proximal movement.ConclusionThe damage patterns of the laser fiber tips were classified into seven types. The heat damage around the surface of the laser fiber can be increased according to the high-energy or high-frequency laser setting, a short distance to the stone, a short distance from the tips of flexible ureteroscopes, no cutting laser fiber procedures, and the inappropriate use of irrigation fluid or laser fiber jacket.     

Modulation of Reactive Oxygen Species to Overcome 5-Fluorouracil Resistance

5-Fluorouracil (5-FU) remains to be an important chemotherapeutic drug for treating several cancers when targeted therapy is unavailable. Chemoresistance limits the clinical utility of 5-FU, and new strategies are required to overcome the resistance. Reactive oxygen species (ROS) and antioxidants are balanced differently in both normal and cancer cells. Modulating ROS can be one method of overcoming 5-FU resistance. This review summarizes selected compounds and endogenous cellular targets modulating ROS generation to overcome 5-FU resistance.     

Agreement and Reliability between Clinically Available Software Programs in Measuring Volumes and Normative Percentiles of Segmented Brain Regions

ObjectiveTo investigate the agreement and reliability of estimating the volumes and normative percentiles (N%) of segmented brain regions among NeuroQuant (NQ), DeepBrain (DB), and FreeSurfer (FS) software programs, focusing on the comparison between NQ and DB.Materials and MethodsThree-dimensional T1-weighted images of 145 participants (48 healthy participants, 50 patients with mild cognitive impairment, and 47 patients with Alzheimer’s disease) from a single medical center (SMC) dataset and 130 participants from the Alzheimer’s Disease Neuroimaging Initiative (ADNI) dataset were included in this retrospective study. All images were analyzed with DB, NQ, and FS software to obtain volume estimates and N% of various segmented brain regions. We used Bland–Altman analysis, repeated measures ANOVA, reproducibility coefficient, effect size, and intraclass correlation coefficient (ICC) to evaluate inter-method agreement and reliability.ResultsAmong the three software programs, the Bland–Altman plot showed a substantial bias, the ICC showed a broad range of reliability (0.004–0.97), and repeated-measures ANOVA revealed significant mean volume differences in all brain regions. Similarly, the volume differences of the three software programs had large effect sizes in most regions (0.73–5.51). The effect size was largest in the pallidum in both datasets and smallest in the thalamus and cerebral white matter in the SMC and ADNI datasets, respectively. N% of NQ and DB showed an unacceptably broad Bland–Altman limit of agreement in all brain regions and a very wide range of ICC values (-0.142–0.844) in most brain regions.ConclusionNQ and DB showed significant differences in the measured volume and N%, with limited agreement and reliability for most brain regions. Therefore, users should be aware of the lack of interchangeability between these software programs when they are applied in clinical practice.     

Feasibility of Single-Shot Whole Thoracic Time-Resolved MR Angiography to Evaluate Patients with Multiple Pulmonary Arteriovenous Malformations

ObjectiveTo evaluate the feasibility of single-shot whole thoracic time-resolved MR angiography (TR-MRA) to identify the feeding arteries of pulmonary arteriovenous malformations (PAVMs) and reperfusion of the lesion after embolization in patients with multiple PAVMs.Materials and MethodsNine patients (8 females and 1 male; age range, 23–65 years) with a total of 62 PAVMs who underwent percutaneous embolization for multiple PAVMs and were subsequently followed up using TR-MRA and CT obtained within 6 months from each other were retrospectively reviewed. All imaging analyses were performed by two independent readers blinded to clinical information. The visibility of the feeding arteries on maximum intensity projection (MIP) reconstruction and multiplanar reconstruction (MPR) TR-MRA images was evaluated by comparing them to CT as a reference. The accuracy of TR-MRA for diagnosing reperfusion of the PAVM after embolization was assessed in a subgroup with angiographic confirmation. The reliability between the readers in interpreting the TR-MRA results was analyzed using kappa (κ) statistics.ResultsFeeding arteries were visible on the original MIP images of TR-MRA in 82.3% (51/62) and 85.5% (53/62) of readers 1 and 2, respectively. Using the MPR, the rates increased to 93.5% (58/62) and 95.2% (59/62), respectively (κ = 0.760 and 0.792, respectively). Factors for invisibility were the course of feeding arteries in the anteroposterior plane, proximity to large enhancing vessels, adjacency to the chest wall, pulsation of the heart, and small feeding arteries. Thirty-seven PAVMs in five patients had angiographic confirmation of reperfusion status after embolization (32 occlusions and 5 reperfusions). TR-MRA showed 100% (5/5) sensitivity and 100% (32/32, including three cases in which the feeding arteries were not visible on TR-MRA) specificity for both readers.ConclusionSingle-shot whole thoracic TR-MRA with MPR showed good visibility of the feeding arteries of PAVMs and high accuracy in diagnosing reperfusion after embolization. Single-shot whole thoracic TR-MRA may be a feasible method for the follow-up of patients with multiple PAVMs.