Publications by authors named "Dae-Yeon Kim"

256 Publications

PLPostnatal Imaging for Prediction of Outcome in Patients with Left-sided Congenital Diaphragmatic Hernia.

J Pediatr 2022 Aug 6. Epub 2022 Aug 6.

Department of Radiology and Research Institute of Radiology, University of Ulsan College of Medicine, Asan Medical Center, 88, Olympic-ro 43-gil, Songpa-Gu, Seoul 05505, Republic of Korea.

Objective: To evaluate associations between postnatal imaging features and outcome of left-sided congenital diaphragmatic hernia (L-CDH) defined by overall survival and a requirement for extracorporeal membrane oxygenation (ECMO).

Study Design: Newborns prenatally diagnosed with L-CDH between January 2013 and September 2021 were studied retrospectively. Esophageal deviation index was newly defined as the largest diameter from the midline to deviated gastric tube divided by the largest transverse diameter of the thoracic cavity on the radiograph. Regression analyses were performed to identify postnatal imaging features associated with overall survival and a requirement for ECMO. Predictive power, i.e., area under the curve (AUC) of a time-dependent receiver operating characteristic curve, of prenatal, postnatal, and intraoperative findings for predicting survival were calculated.

Results: 97 patients (54 male; mean gestational age, 38.3 ± 1.9 weeks; mean birth weight, 2956.5 ± 540.0 g) were analyzed. Esophageal deviation index (adjusted hazard ratio [HR]: moderate [≥0.19, <0.24], 6.427 [P = 0.029]; severe [≥0.24], 33.007 [P < 0.001]) and right pneumothorax (adjusted HR, 8.763; P = 0.002) were associated with overall survival, and also associated with a requirement for ECMO. Liver herniation on postnatal ultrasound (US) was also associated with overall survival (P <0.001) and a requirement for ECMO (P = 0.001). In addition, AUC for prediction of 1 year survival from postanatal US was comparable with prenatally or intraoperatively detected liver herniation (0.93; 95% CI, 0.88-0.97).

Conclusion: Esophageal deviation index, right pneumothorax, and liver herniation observed by postnatal imaging have prognostic value in patients with L-CDH.
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http://dx.doi.org/10.1016/j.jpeds.2022.07.037DOI Listing
August 2022

Living donor liver transplantation in an infant patient with progressive familial intrahepatic cholestasis along with hepatocellular carcinoma: a case report.

Korean J Transplant 2022 Mar;36(1):73-78

Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Progressive familial intrahepatic cholestasis (PFIC) is an autosomal recessive inherited disease requiring liver transplantation (LT). Hepatocellular carcinoma (HCC) is very rare in infants. We present a case of living donor LT using a left lateral section graft performed in a 7-month-old female infant diagnosed with PFIC type II and HCC. No mutation on gene was identified. Because of progressive deterioration of liver function, living donor LT with her mother's left lateral section graft was performed. Pretransplant serum alpha-fetoprotein (AFP) level was increased to 2,740 ng/mL, but HCC was not taken into account because of its rarity. The explant liver showed micronodular liver cirrhosis, multiple infantile hemangiomas and two HCCs of 0.7 cm and 0.3 cm in size. The patient recovered uneventfully from the LT operation. This patient has been regularly followed up with abdomen ultrasonography and AFP measurement every 6 months. The patient has been continually doing well for 8 years after the LT. In conclusion, LT is currently the only effective treatment for PFIC-associated end-stage liver diseases. HCC can develop at the cirrhotic liver of any cause, thus elevation of HCC tumor markers in pediatric patients is an important clue to perform further investigation before LT.
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http://dx.doi.org/10.4285/kjt.21.0007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9235533PMC
March 2022

Comparison of splenic vessel preserving distal pancreatectomy and the Warshaw technique for solid pseudopapillary neoplasm in children.

Pediatr Surg Int 2022 Aug 28;38(8):1143-1148. Epub 2022 May 28.

Division of Pediatric Surgery, Department of Surgery, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, Korea.

Purpose: Spleen-preserving distal pancreatectomy (SPDP) such as splenic vessel preservation (SVP) for solid pseudopapillary neoplasms (SPNs) in the body or tail of the pancreas in children prevents post-splenectomy infection and hematologic disorders. However, SVP could be technically challenging, and the Warshaw technique (WT) could be an alternative technique that has been reported to be safe and effective in adults. We, therefore, compared the perioperative outcomes of SVP and WT in pediatric patients.

Methods: We retrospectively reviewed the medical records of pediatric patients with SPN who underwent SPDP using SVP or WT between November 2002 and November 2018 at a large-sized tertiary referral center.

Results: Twenty-eight patients were included. Sixteen (57.1%) patients underwent SVP and 12 (42.9%) patients underwent WT. There were no significant differences in the baseline characteristics between the two groups. Postoperative complications occurred in 8 patients each in the SVP (50%), and the WT (66.7%) groups. Two (12.5%) in the SVP group and 1 (8.3%) in the WT group required additional intervention. During a median follow-up duration of 49 months, there were no significant differences in the incidence of splenic infarctions or perigastric varices between the two groups.

Conclusions: There were no significant differences in the surgical outcomes between WT and SVP in pediatric patients with SPN. WT could be a safe and feasible alternative technique for SVP in challenging cases.
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http://dx.doi.org/10.1007/s00383-022-05134-xDOI Listing
August 2022

Effect of Colloidal Interactions and Hydrodynamic Stress on Particle Deposition in a Single Micropore.

Langmuir 2022 May 4;38(19):6013-6022. Epub 2022 May 4.

School of Chemical and Biological Engineering, and Institute of Chemical Processes, Seoul National University, Seoul 08826, Korea.

Clogging is ubiquitous. It happens in a wide range of material processing and causes severe performance degradation or process breakdown. In this study, we investigate clogging dynamics in a single micropore by controlling the surface property of the particle and processing condition. Microfluidic observation is conducted to investigate particle deposition in a contraction microchannel where polystyrene suspension is injected as a feed solution. The particle deposition area is quantified using the images taken using a CCD camera in both upstream and downstream of the microchannel. Pressure drop across the microchannel is also measured. When the particle interaction is repulsive, the deposition occurs mostly in downstream, while an opposite tendency is identified when the particle interaction is attractive. More complex deposition characteristics are found as the flow rate is changed. Particle flux density and the ratio of lift force to colloidal force are introduced to explain the clogging dynamics. This study provides a useful insight to alleviate clogging issues by controlling the colloidal interaction and hydrodynamic stress.
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http://dx.doi.org/10.1021/acs.langmuir.2c00237DOI Listing
May 2022

Transcriptome Analysis of MYB Genes and Patterns of Anthocyanin Accumulation During Seed Development in Wheat.

Evol Bioinform Online 2022 13;18:11769343221093341. Epub 2022 Apr 13.

Department of Plant Biotechnology, Korea University, Seoul, Korea.

Plants accumulate key metabolites as a response of biotic/abiotic stress conditions. In seed coats, anthocyanins, carotenoids, and chlorophylls can be found. They have been associated as important antioxidants that affect germination. In wheat, anthocyanins can impart the seed coat color which have been recognized as health-promoting nutrients. Transcription factors act as master regulators of cellular processes. Transcription complexes such as MYB-bHLH-WD40 (MBW) regulate the expression of multiple target genes in various plant species. In this study, the spatiotemporal accumulation of seed coat pigments in different developmental stages (10, 20, 30, and 40 days after pollination) was analyzed using cryo-cuts. Moreover, the accumulation of phenolic, anthocyanin, and chlorophyll contents was quantified, and the expression of flavonoid biosynthetic genes was evaluated. Finally, transcriptome analysis was performed to analyze putative MYB genes related to seed coat color, followed by further characterization of putative genes. , an MYB gene, was cloned and sequenced. It was determined that contains a SANT domain, which is often present in proteins participating in the response to anthocyanin accumulation. Moreover, transcript levels were shown to be influenced by anthocyanin accumulation during grain development. Interaction network analysis showed interactions with GL2 (HD-ZIP IV), EGL3 (bHLH), and TTG1 (WD40). The findings of this study elucidate the mechanisms underlying color formation in L. seed coats.
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http://dx.doi.org/10.1177/11769343221093341DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9014723PMC
April 2022

Assessment of pathogens and risk factors associated with bloodstream infection in the year after pediatric liver transplantation.

World J Gastroenterol 2022 Mar;28(11):1159-1171

Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul 05505, South Korea.

Background: Bloodstream infection (BSI) is one of the most significantly adverse events that can occur after liver transplantation (LT) in children.

Aim: To analyze the profile of BSI according to the postoperative periods and assess the risk factors after pediatric LT.

Methods: Clinical data, collected from medical charts of children ( = 378) who underwent primary LT, were retrospectively reviewed. The primary outcome considered was BSI in the first year after LT. Univariate and multivariate analyses were performed to identify risk factors for BSI and respective odds ratios (ORs).

Results: Of the examined patients, 106 (28%) experienced 162 episodes of pathogen-confirmed BSI during the first year after LT. There were 1.53 ± 0.95 episodes children (mean ± SD) among BSI-complicated patients with a median onset of 0.4 mo post-LT. The most common pathogenic organisms identified were , followed by and About half (53%) of the BSIs were of unknown origin. Multivariate analysis demonstrated that young age (≤ 1.3 year; OR = 2.1, = 0.011), growth failure (OR = 2.1, = 0.045), liver support system (OR = 4.2, = 0.008), and hospital stay of > 44 d (OR = 2.3, = 0.002) were independently associated with BSI in the year after LT.

Conclusion: BSI was frequently observed in patients after pediatric LT, affecting survival outcomes. The profile of BSI may inform clinical treatment and management in high-risk children after LT.
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http://dx.doi.org/10.3748/wjg.v28.i11.1159DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8985487PMC
March 2022

Clinical characteristics of neonatal cholestasis in a tertiary hospital and the development of a novel prediction model for mortality.

EBioMedicine 2022 Mar 25;77:103890. Epub 2022 Feb 25.

Department of Pediatrics, Asan Medical Center Children's Hospital, University Ulsan College of Medicine, 88, Olympic-ro 43-gil Songpa-Gu, Seoul 05505, South Korea.

Background: Few studies have described the aetiologies of neonatal cholestasis, and the overall neonatal cholestasis-related mortality (NCM) rate is unclear. We investigated the aetiology and outcome of neonatal cholestasis in a tertiary hospital and developed an NCM prediction model for these patients.

Methods: Patients aged <100 days with serum direct bilirubin (DB) levels of >1.0 mg/dL were retrospectively screened. Diagnostic and laboratory data during the 8-week follow-up period after enrolment between 2005 and 2020 were extracted digitally, and medical charts were reviewed manually by clinicians. Logistic regression was used to derive a prediction model for the 1-year mortality outcome of neonatal cholestasis, and performance evaluation and external validation were conducted for the NCM prediction model.

Findings: We enrolled 4028 neonates with DB of >1.0 mg/dL at least once. Prematurity and birth injury (35.4%), complex heart anomalies (18.6%), liver diseases (11.4%), and gastrointestinal anomalies (9.2%) were the most common aetiologies; 398 (9.9%) patients died before one year of age. The peak value of DB was positively correlated to the 1-year mortality rate. In the multivariate analysis, simple laboratory indices, including platelet, prothrombin time, aspartate aminotransferase, albumin, direct bilirubin, creatinine, and C-reactive protein, were independent predictors of 1-year mortality outcome of complete-case subjects. Using these laboratory indices, a logistic regression-based NCM prediction model was constructed. It showed acceptable performances on discrimination (area under the curve, 0.916), calibration (slope, 1.04) and Brier scoring (0.072). The external validation of the sample (n = 920) from two other centres also revealed similar performance profiles of the NCM model.

Interpretation: Various aetiologies of neonatal cholestasis were identified in a tertiary hospital, resulting in unfavourable outcomes of a large proportion. The NCM prediction model may have the potential to help clinicians to be aware of high-risk neonatal cholestasis.

Funding: Ministry of Health & Welfare, Republic of Korea.
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http://dx.doi.org/10.1016/j.ebiom.2022.103890DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8889106PMC
March 2022

Molecular Characterization of U-box E3 Ubiquitin Ligases (TaPUB2 and TaPUB3) Involved in the Positive Regulation of Drought Stress Response in .

Int J Mol Sci 2021 Dec 20;22(24). Epub 2021 Dec 20.

Department of Plant Biotechnology, Korea University, Seoul 02841, Korea.

Plant U-box E3 ubiquitin ligase (PUB) is involved in various environmental stress conditions. However, the molecular mechanism of U-box proteins in response to abiotic stress in wheat remains unknown. In this study, two U-box E3 ligase genes ( and ), which are highly expressed in response to adverse abiotic stresses, were isolated from common wheat, and their cellular functions were characterized under drought stress. Transient expression assay revealed that TaPUB2 was localized in the cytoplasm and Golgi apparatus, whereas TaPUB3 was expressed only in the Golgi apparatus in wheat protoplasts. Additionally, TaPUB2 and TaPUB3 underwent self-ubiquitination. Moreover, TaPUB2/TaPUB3 heterodimer was identified in yeast and the cytoplasm of wheat protoplasts using a pull-down assay and bimolecular fluorescence complementation analysis. Heterogeneous overexpression of and conferred tolerance to drought stress. Taken together, these results implied that the heterodimeric form of U-box E3 ubiquitin ligases (TaPUB2/TaPUB3) responded to abiotic stress and roles as a positive regulator of drought stress tolerance.
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http://dx.doi.org/10.3390/ijms222413658DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8704797PMC
December 2021

Energy harvesting performance of an EDLC power generator based on pure water and glycerol mixture: analytical modeling and experimental validation.

Sci Rep 2021 Dec 6;11(1):23426. Epub 2021 Dec 6.

School of Mechanical Engineering, Pusan National University, Busan, 46241, Republic of Korea.

A liquid droplet oscillating between two plane electrodes was visualized, and the electrical power generation based on the reverse-electrowetting-on-dielectric (REWOD) phenomenon was measured. For the upper plate, a hydrophobic surface treated by PTFE was used, and the lower plate was tested using the hydrophilic surface properties of ITO glass. To analyze the dynamic behavior of an oscillating liquid bridge, a modeling study was carried out using the phase field method based on the finite element method. The dynamic contact angle of the oscillating liquid bridge was modeled based on advancing and receding contact angles. The variable interfacial areas between the liquid and solid surfaces were calculated and agreed well with the experimental results within a 10% error band. Furthermore, experimental and analytical studies were carried out to examine the REWOD energy harvesting characteristics of the glycerol-water mixtures in various concentrations. As a result, the peak voltage output was obtained at a specific concentration of the glycerol mixture, and the power density of the oscillating liquid bridge at this point was up to 2.23 times higher than that of pure water.
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http://dx.doi.org/10.1038/s41598-021-02964-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8648766PMC
December 2021

Near-Infrared Self-Powered Linearly Polarized Photodetection and Digital Incoherent Holography Using WSe/ReSe van der Waals Heterostructure.

ACS Nano 2021 Oct 22. Epub 2021 Oct 22.

Center of Optoelectronic Materials and Devices, Post-Silicon Semiconductor Institute, Korea Institute of Science and Technology (KIST), Seoul 02792, Republic of Korea.

Polarization-sensitive photodetection has attracted considerable attention as an emerging technology for future optoelectronic applications such as three-dimensional (3D) imaging, quantum optics, and encryption. However, traditional photodetectors based on Si or III-V InGaAs semiconductors cannot directly detect polarized light without additional optical components. Herein, we demonstrate a self-powered linear-polarization-sensitive near-infrared (NIR) photodetector using a two-dimensional WSe/ReSe van der Waals heterostructure. The WSe/ReSe heterojunction photodiode with semivertical geometry exhibits excellent performance: an ideality factor of 1.67, a broad spectral photoresponse of 405-980 nm with a significant photovoltaic effect, outstanding linearity with a linear dynamic range wider than 100 dB, and rapid photoswitching behavior with a cutoff frequency up to 100 kHz. Strongly polarized excitonic transitions around the band edge in ReSe lead to significant 980 nm NIR linear-polarization-dependent photocurrent. This linear polarization sensitivity remains stable even after exposure to air for longer than five months. Furthermore, by leveraging the NIR (980 nm)-selective linear polarization detection of this photodiode under photovoltaic operation, we demonstrate digital incoherent holographic 3D imaging.
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http://dx.doi.org/10.1021/acsnano.1c06234DOI Listing
October 2021

Prenatal prognostic factors for isolated right congenital diaphragmatic hernia: a single center's experience.

BMC Pediatr 2021 10 20;21(1):460. Epub 2021 Oct 20.

Department of Obstetrics and Gynecology, University of Ulsan College of Medicine, Asan Medical Center, 88 Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, Korea.

Background: Right-sided congenital diaphragmatic hernia (RCDH) is relatively rare compared with left-sided congenital diaphragmatic hernia (LCDH). Clinical data of RCDH, especially with respect to antenatal prediction of neonatal outcome, are lacking. The aim of this study was to report the treatment outcomes of patients with antenatally diagnosed RCDH and to evaluate the predictability of observed-to-expected lung area-to-head circumference ratio (O/E LHR) for perinatal outcomes, focused on mortality or extracorporeal membrane oxygenation (ECMO) requirement.

Methods: We retrospectively reviewed the medical records of newborn infants with isolated RCDH. We analyzed and compared the clinical and prenatal characteristics including the fetal lung volume, which was measured as the O/E LHR, between the survivors and the non-survivors.

Results: A total of 26 (66.7%) of 39 patients with isolated RCDH survived to discharge. The O/E LHR was significantly greater in survivors (64.7 ± 21.2) than in non-survivors (40.5 ± 23.4) (P =.027). It was greater in survivors without ECMO requirement (68.3 ± 15.1) than non-survivors or those with ECMO requirement (46.3 ± 19.4; P = .010). The best O/E LHR cut-off value for predicting mortality in isolated RCDH was 50.

Conclusions: The findings in this study suggest that O/E LHR, a well-characterized prognostic indicator in LCDH, could be applied to a fetus with antenatally diagnosed RCDH. A large cohort study is required to verify the association between O/E LHR values and the graded severity of RCDH.
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http://dx.doi.org/10.1186/s12887-021-02931-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8526355PMC
October 2021

Recent Improvement in Survival Outcomes and Reappraisal of Prognostic Factors in Pediatric Living Donor Liver Transplantation.

Liver Transpl 2022 06 22;28(6):1011-1023. Epub 2021 Oct 22.

Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.

Living donor liver transplantation (LDLT) is a significant advancement for the treatment of children with end-stage liver disease given the shortage of deceased donors. The ultimate goal of pediatric LDLT is to achieve complete donor safety and zero recipient mortality. We conducted a retrospective, single-center assessment of the outcomes as well as the clinical factors that may influence graft and patient survival after primary LDLTs performed between 1994 and 2020. A Cox proportional hazards model was used for multivariate analyses. The trends for independent prognostic factors were analyzed according to the following treatment eras: 1, 1994 to 2002; 2, 2003 to 2011; and 3, 2012 to 2020. Primary LDLTs were performed on 287 children during the study period. Biliary atresia (BA; 52%), acute liver failure (ALF; 26%), and monogenic liver disease (11%) were the leading indications. There were 45 graft losses (16%) and 27 patient deaths (7%) in this population during the study period. During era 1 (n = 81), the cumulative survival rates at 1 and 5 years after LDLT were 90.1% and 81.5% for patients and 86.4% and 77.8% for grafts, respectively. During era 2 (n = 113), the corresponding rates were 92.9% and 92% for patients and 89.4% and 86.7% for grafts, respectively. During era 3 (n = 93), the corresponding rates were 100% and 98.6% for patients and 98.9% and 95.4% for grafts, respectively. In the multivariate analyses, primary diagnosis ALF, bloodstream infection, posttransplant lymphoproliferative disease, and chronic rejection were found to be negative prognostic indicators for patient survival. Based on generalized care guidelines and center-oriented experiences, comprehensive advances in appropriate donor selection, refinement of surgical techniques, and meticulous medical management may eventually realize a zero-mortality rate in pediatric LDLT.
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http://dx.doi.org/10.1002/lt.26308DOI Listing
June 2022

Single-Incision versus Multiport Robotic Myomectomy: A Propensity Score Matched Analysis of Surgical Outcomes and Surgical Tips.

J Clin Med 2021 Aug 31;10(17). Epub 2021 Aug 31.

Department of Obstetrics and Gynecology, Seoul Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea.

We aimed to compare the perioperative outcomes of single-incision robotic myomectomy (SIRM) and multiport robotic myomectomy (MPRM) and provide surgical tips. We retrospectively analyzed the medical records of 462 patients with symptomatic leiomyoma who underwent MPRM or SIRM between March 2019 and April 2021. Demographic characteristics and surgical outcomes, including the total operative time (OT), estimated blood loss (EBL), and surgical complication rate, were compared between the two groups. Patients in the SIRM group had lower a body mass index and rate of previous pelvic surgery and were younger than those in the MPRM group. The myoma type was not different between groups; however, the MPRM group had larger, and more myomas than the SIRM group. After propensity score matching, these variables were not significantly different between the groups. The total OT, EBL, difference in hemoglobin levels, transfusion rate, and postoperative fever were not different between the groups. No postoperative complications occurred in the SIRM group. In the MPRM group, one patient needed conversion to laparotomy, and two patients had postoperative complications (umbilical incisional hernia and acute kidney injury). In conclusion, both MPRM and SIRM are feasible and effective surgical options for symptomatic myomas with cosmetic benefits and minimal risk of laparotomy conversion.
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http://dx.doi.org/10.3390/jcm10173957DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8432212PMC
August 2021

Regulation of Glycosylphosphatidylinositol-Anchored Protein (GPI-AP) Expression by F-Box/LRR-Repeat (FBXL) Protein in Wheat ( L.).

Plants (Basel) 2021 Aug 5;10(8). Epub 2021 Aug 5.

Institute of Animal Molecular Biotechnology, Korea University, 145 Anam-Ro, Seongbuk-Gu, Seoul 02841, Korea.

F-box proteins are substrate recognition components of the Skp1-Cullin-F-box (SCF) complex, which performs many important biological functions including the degradation of numerous proteins via the ubiquitin-26S proteasome system. In this study, we isolated the gene encoding the F-box/LRR-repeat (FBXL) protein from wheat ( L.) seedlings and validated that the TaFBXL protein is a component of the SCF complex. Yeast two-hybrid assays revealed that TaFBXL interacts with the wheat glycosylphosphatidylinositol-anchored protein (TaGPI-AP). The green fluorescent protein (GFP) fusion protein of TaFBXL was detected in the nucleus and plasma membrane, whereas that of TaGPI-AP was observed in the cytosol and probably also plasma membrane. yeast two-hybrid and bimolecular fluorescence complementation (BiFC) assays revealed that TaFBXL specifically interacts with TaGPI-AP in the nucleus and plasma membrane, and TaGPI-AP is targeted by TaFBXL for degradation via the 26S proteasome system. In addition, TaFBXL and TaGPI-AP showed antagonistic expression patterns upon treatment with indole-3-acetic acid (IAA), and the level of TaGPI-AP was higher in tobacco leaves treated with both MG132 (proteasome inhibitor) and IAA than in leaves treated with either MG132 or IAA. Taken together, our data suggest that TaFBXL regulates the TaGPI-AP protein level in response to exogenous auxin application.
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http://dx.doi.org/10.3390/plants10081606DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8397982PMC
August 2021

Effect of Metal Foam Insert Configurations on Flow Boiling Heat Transfer and Pressure Drop in a Rectangular Channel.

Materials (Basel) 2021 Aug 17;14(16). Epub 2021 Aug 17.

School of Mechanical Engineering, Pusan National University, Busan 46241, Korea.

Heat transfer under flow boiling is better in a rectangular channel filled with open-cell metal foam than in an empty channel, but the high pressure drop is a drawback of the empty channel method. In this study, various types of metal foam insert configurations were tested to reduce the pressure drop while maintaining high heat transfer. Specifically, we measured the boiling heat transfer and pressure drop of a two-phase vertical upward flow of R245fa inside a channel. To measure the pressure and temperature differences of the metal foam, differential pressure transducers and T-type thermocouples were used at both ends of the test section. While the saturation pressure was kept constant at 5.9 bar, the steam quality at the inlet of the test section was changed from 0.05 to 0.99. The channel height, moreover, was 3 mm, and the mass flux ranged from 133 to 300 kg/ms. The two-phase flow characteristics were observed through a high-speed visualization experiment. Heat transfer tended to increase with the mean vapor quality, and, as expected, the fully filled metal foam channel offered the highest thermal performance. The streamwise insert pattern model had the lowest heat transfer at a low mass flux. However, at a higher mass flux, the three different insert models presented almost the same heat transfer coefficients. We found that the streamwise pattern model had a very low pressure drop compared to that of the spanwise pattern models. The goodness factors of the flow area and the core volume of the streamwise patterned model were higher than those of the full-filled metal foam channel.
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http://dx.doi.org/10.3390/ma14164617DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8401193PMC
August 2021

Pediatric deceased donor liver transplantation with size reduction for recipient-graft size matching.

Ann Hepatobiliary Pancreat Surg 2021 Aug;25(3):431-435

Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

We present a case of pediatric deceased donor liver transplantation using a reduced whole liver graft in a 25-month-old boy weighing 12.7 kg. After he had undergone Kasai portoenterostomy for biliary atresia, his general condition deteriorated progressively. He was enrolled on the waiting list for liver transplantation with Pediatric End-stage Liver Disease score of 15. The donor was a 51-monthold boy with body weight of 20 kg. The donor-to-recipient body weight ratio was 158%. The liver graft appeared to be larger than the recipient's abdominal cavity. Thus, we planned to do size reduction. Recipient surgery was performed following standard procedures. We performed graft outflow vein reconstruction using a modified piggyback technique like the double inferior vena cava method. Since the portal vein was hypoplastic, a side-to-side anastomosis technique was used. We also performed intraoperative portogram to embolize venous collaterals. After completing the graft implantation, we found that the liver graft was too large to be accommodated within the abdomen. After resection of the left lateral section parenchyma, we successfully performed primary closure of the abdominal wound. This patient experienced episodes of acute rejection. He has been doing well for four years after the transplantation.
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http://dx.doi.org/10.14701/ahbps.2021.25.3.431DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8382866PMC
August 2021

The Clinical Significance and Utility of HPV-DNA Testing in Korean Women with Atypical Glandular Cells in Cervical Pap Tests: An Analysis of 311 Cases at a Single Institution.

Cancer Invest 2021 Nov 3;39(10):885-892. Epub 2021 Sep 3.

Department of Obstetrics and Gynecology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

The aim of this study is to analyze the correlation between clinically significant histologic results and HPV in women with AGC in pap test. Of the 311 women confirmed as AGC, 111 women (35.7%) was identified as positive for HPV. In the AGC analysis, cervical lesions were significantly more common in HPV positive group compared to HPV negative group (61.2 vs. 10.5%,  < 0.001). In contrast, endometrial lesions were not associated with HPV infection (8.1 vs. 4.5%,  = 0.12). The HPV-DNA testing in women with AGC may be a useful tool for predicting clinically significant cervical lesions.
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http://dx.doi.org/10.1080/07357907.2021.1952593DOI Listing
November 2021

Clinical Characteristics and Long-term Outcomes of Pediatric Ulcerative Colitis: A Single-Center Experience in Korea.

Gut Liver 2022 Mar;16(2):236-245

Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.

Background/aims: : Although pediatric ulcerative colitis (UC) has a different phenotype and clinical course than adult UC, its clinical features and outcomes are poorly defined, especially in Asian populations. This study investigated the clinical features and long-term outcomes of pediatric UC in a Korean population.

Methods: We retrospectively analyzed 208 patients aged <18 years diagnosed with UC between 1987 and 2013. The patient characteristics at diagnosis according to the Paris classification and the clinical course were analyzed.

Results: The male-to-female ratio was 1.3:1, and the median patient age was 15.5 years. At diagnosis, 28.8% of patients had proctitis (E1), 27.8%, left-sided colitis (E2); 5.2%, extensive colitis (E3); and 38.2%, pancolitis (E4). The cumulative probabilities of extension after 5, 10, 15, and 20 years were 32.7%, 40.4%, 52.5%, and 65.8%, respectively. Eighteen patients underwent colectomy, and three patients had colorectal cancer. The cumulative probabilities of colectomy after 5, 10, 15, and 20 years were 7.1%, 8.9%, 12.6%, and 15.6%, and those of colorectal cancer after 10, 15, and 20 years were 0%, 2.1%, and 12.0%, respectively. The disease extent, Pediatric Ulcerative Colitis Activity Index severity, and systemic corticosteroid therapy were significant risk factors for colectomy. The development of primary sclerosing cholangitis was significantly associated with colorectal cancer.

Conclusions: This study provides detailed information on the disease phenotype and long-term clinical outcomes in a large cohort of Korean children with UC. They have extensive disease at diagnosis, a high rate of disease extension, and a low rate of cumulative colectomy.
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http://dx.doi.org/10.5009/gnl20337DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8924810PMC
March 2022

Efficacy and safety of autologous adipose tissue-derived stem cell therapy for children with refractory Crohn's complex fistula: a Phase IV clinical study.

Ann Surg Treat Res 2021 Jul 30;101(1):58-64. Epub 2021 Jun 30.

Department of Pediatric Surgery, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.

Purpose: Autologous adipose tissue-derived stem cells (ASCs) have been proposed for patients with refractory Crohn disease, but research is lacking in pediatric patients. This Phase IV study evaluated the efficacy and safety of ASCs in children with refractory Crohn's fistulae.

Methods: Patients with a refractory Crohn's fistula who did not have conventional therapy for more than 3 months or with a recurrent complex Crohn's fistula were included. All patients were at least 14 years old. Patients with infection, poor condition, or active Crohn disease with a disease activity index of 450 and above were excluded. Five patients were treated with ASCs from 2014 to 2015 in Asan Medical Center. ASC administration was adjusted according to fistula size (1 mL per cm). We evaluated the efficacy and safety 8 weeks after injection and followed patients for 6 months.

Results: Fistulae were healed in 4 patients by 8 weeks after ASC injection. Of these 4 patients, 1 had complete fistula closure and sustainability after 6 months. The other 3 with healing effects had less than 50% fistula closure by 6 months. None of these 4 patients have persistent fistulae. One patient had no healing effect, and seton ligation was performed 8 months after ASC injection. There were no adverse effects related to ASC administration.

Conclusion: ASC therapy is a simple and well-tolerated therapeutic option for children with refractory Crohn's complex fistulae. Complete closure was well-sustained. However, more data from a larger number of patients are needed.
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http://dx.doi.org/10.4174/astr.2021.101.1.58DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8255577PMC
July 2021

Urinary exosomal microRNA profiling in type 2 diabetes patients taking dipeptidyl peptidase-4 inhibitor compared with sulfonylurea.

Kidney Res Clin Pract 2021 Sep 7;40(3):383-391. Epub 2021 Jul 7.

Department of Internal Medicine, Soonchunhyang University Cheonan Hospital, Cheonan, Republic of Korea.

Background: Dipeptidyl peptidase-4 (DPP-4) inhibitor has been reported to have kidney-protective benefits. To elucidate how antidiabetic agents prevent diabetic kidney disease progression, it is important to investigate their effect on the kidney environment in type 2 diabetes mellitus (DM) patients. Herein, we investigated the expression pattern of urinary exosome-derived microRNA (miRNA) in patients taking a combination of DPP-4 inhibitor and metformin (DPP-4 inhibitor group) and compared them with patients taking a combination of sulfonylurea and metformin (sulfonylurea group).

Methods: This was a prospective study involving 57 patients with type 2 DM (DPP-4 inhibitor group, n = 34; sulfonylurea group, n = 23) and healthy volunteers (n = 7). We measured urinary exosomal miRNA using the NanoString nCounter miRNA array (NanoString Technologies) across the three groups (n = 4 per each group) and validated findings using real-time polymerase chain reaction.

Results: Twenty-one differentially expressed candidate miRNAs were identified, and six (let-7c-5p, miR-23a-3p, miR-26a-3p, miR-30d, miR-205, and miR-200a) were selected for validation. Validation showed no significant difference in miRNA expression between the DPP-4 inhibitor and sulfonylurea groups. Only miR-23a-3p was significantly overexpressed in the diabetes group compared with the control group (DPP-4 inhibitor vs. control, p = 0.01; sulfonylurea vs. control, p = 0.007). This trend was consistent even after adjusting for age, sex, and body mass index.

Conclusion: There was no significant difference in urine exosome miRNA expression between diabetic participants taking DPP-4 inhibitor and those taking sulfonylurea. The miR-23a levels were higher in diabetic participants than in nondiabetic controls.
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http://dx.doi.org/10.23876/j.krcp.21.015DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8476296PMC
September 2021

Effect of chilling acclimation on germination and seedlings response to cold in different seed coat colored wheat (Triticum aestivum L.).

BMC Plant Biol 2021 Jun 2;21(1):252. Epub 2021 Jun 2.

Department of Plant Biotechnology, Korea University, Seoul, 02841, Korea.

Background: Flavonoids can protect plants against extreme temperatures and ROS due to their antioxidant activities. We found that deep-purple seed coat color was controlled by two gene interaction (12:3:1) from the cross between yellow and deep-purple seed coat colored inbreds. F seeds were grouped in 3 by seed coat color and germinated under chilling (4 °C) and non-acclimated conditions (18 °C) for a week, followed by normal conditions (18 °C) for three weeks and a subsequent chilling stress (4 °C) induction. We analyzed mean daily germination in each group. Additionally, to study the acclimation in relationship to the different seed coat colors on the germination ability and seedling performances under the cold temperatures, we measured the chlorophyll content, ROS scavenging activity, and expression levels of genes involved in ROS scavenging, flavonoid biosynthetic pathway, and cold response in seedlings.

Results: The results of seed color segregation between yellow and deep purple suggested a two-gene model. In the germination study, normal environmental conditions induced the germination of yellow-seed, while under chilling conditions, the germination ratio of deep purple-seed was higher than that of yellow-colored seeds. We also found that the darker seed coat colors were highly responsive to cold acclimation based on the ROS scavenging enzymes activity and gene expression of ROS scavenging enzymes, flavonoid biosynthetic pathway and cold responsive genes.

Conclusions: We suggest that deep purple colored seed might be in a state of innate pre-acquired stress response state under normal conditions to counteract stresses in a more effective way. Whereas, after the acclimation, another stress should enhance the cold genes expression response, which might result in a more efficient chilling stress response in deep purple seed seedlings. Low temperature has a large impact on the yield of crops. Thus, understanding the benefit of seed coat color response to chilling stress and the identification of limiting factors are useful for developing breeding strategies in order to improve the yield of wheat under chilling stress.
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http://dx.doi.org/10.1186/s12870-021-03036-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8173842PMC
June 2021

Identification of a Splice Variant (c.5074+3A>C) of by RNA Sequencing and TOPO Cloning.

Genes (Basel) 2021 05 26;12(6). Epub 2021 May 26.

Department of Laboratory Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul 05505, Korea.

Grading the pathogenicity of 1/2 variants has great clinical importance in patient treatment as well as in the prevention and screening of hereditary breast and ovarian cancer (HBOC). For accurate evaluation, confirming the splicing effect of a possible splice site variant is crucial. We report a significant splicing variant (c.5074+3A>C) in in a patient with recurrent ovarian cancer. Next-generation sequencing (NGS) of from patient's peripheral blood identified the variant, which was strongly suspected of being a splicing mutation based on in silico predictions. Direct RNA analysis yielded multiple transcripts, and TOPO cloning of the complementary DNA (cDNA) and Sanger sequencing revealed an aberrant transcript with an insertion of the first 153 bp of intron 17, and another transcript with the 153 bp insertion along with an exon 18 deletion. A premature termination codon was presumed to be formed by the 153 bp partial intron retention common to the two transcripts. Therefore, c.5074+3A>C was classified as a likely pathogenic variant. Our findings show that active use of functional studies of variants suspected of altered splicing are of great help in classifying them.
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http://dx.doi.org/10.3390/genes12060810DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8229931PMC
May 2021

Efficacy of Living Donor Liver Transplantation in Patients with Methylmalonic Acidemia.

Pediatr Gastroenterol Hepatol Nutr 2021 May 4;24(3):288-294. Epub 2021 May 4.

Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.

Purpose: Despite aggressive medical and nutritional management, patients with methylmalonic acidemia (MMA) often suffer from multi-organ damage. Early deceased donor liver transplantation (DDLT) has emerged as an intervention to prevent disease progression. We investigated the efficacy of living donor LT (LDLT) with a potential carrier of MMA and a small volume of graft in patients with MMA as an alternative to DDLT.

Methods: Of five patients (three male, two female; median age 5.7 years; range, 1.3-13.7 years), four underwent carrier LDLT, while one underwent non-carrier auxiliary LDLT. All patients received pre- and post-LT continuous renal replacement therapy and were provided with minimal restriction diet according to serum MMA level after LT. MMA levels in the serum and urine, the incidence of metabolic crisis, and clinical findings before and after LT were compared.

Results: The survival rate was 100% during 2.2 years of follow up period after LT. In all five cases, MMA titer in the serum after transplantation decreased with less restrictive diet. Metabolic crisis was not observed during the follow-up period. In addition, no patient showed progression of severe renal impairment requiring hemodialysis. Progression of delayed cognitive development was not observed. Social functioning with improved neuropsychiatric development was observed.

Conclusion: This study showed that LDLT achieved improved quality of life with less restrictive diet, therefore it could be a feasible alternative option to DDLT for the treatment of patients with MMA, even with an auxiliary LT.
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http://dx.doi.org/10.5223/pghn.2021.24.3.288DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8128783PMC
May 2021

Outcome of staging chest CT and identification of factors associated with lung metastasis in children with hepatoblastoma.

Eur Radiol 2021 Dec 25;31(12):8850-8857. Epub 2021 May 25.

Department of Pediatric Surgery, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Republic of Korea.

Objectives: To evaluate the outcome of staging chest CT and to identify clinicoradiological factors predictive of lung metastasis in patients with hepatoblastoma based on the 2017 PRE-Treatment EXTent of tumor (PRETEXT) system.

Methods: This bi-center study retrospectively identified patients diagnosed with hepatoblastoma between January 1998 and September 2019 in two tertiary hospitals. The primary outcome was the proportion of the patients who had lung metastasis at staging chest CT. The diagnostic accuracy of staging chest CT was calculated based on the 2017 PRETEXT criteria. The secondary outcome was the identification of factors predictive of lung metastasis using multivariable logistic regression.

Results: In total, 123 patients (median age, 1 year; interquartile range, 0-4 years; 59 female) were included. Among those, 28% (35/123; 95% confidence interval [CI], 21-37%) had lung metastasis at staging chest CT. The overall accuracy of staging chest CT was 96.8%. The proportion of lung metastasis in patients with stage I, II, III, and IV was 0%, 24% (12 of 49; 95% CI, 14-38%), 23% (9 of 40; 95% CI, 12-38%), and 56% (14 of 25; 95% CI, 37-73%), respectively. Multifocality (adjusted odds ratio, 6.7; 95% CI, 2.7-17.5; p < .001) and male sex (adjusted odds ratio, 3.1; 95% CI, 1.2-8.6; p = .02) were associated with the presence of lung metastasis.

Conclusions: Twenty-eight percent of the patients with hepatoblastoma had lung metastasis at staging chest CT. Multifocality and male sex were predictive factors for lung metastasis on staging chest CT.

Key Points: • The proportion of lung metastasis in patients with hepatoblastoma was 28%. • The overall accuracy of staging chest CT was 97% based on the 2017 PRETEXT system. • Hepatic tumor multifocality and male sex were predictors of lung metastasis.
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http://dx.doi.org/10.1007/s00330-021-08047-wDOI Listing
December 2021

Recent improvement in survival outcomes and reappraisal of prognostic factors in hepatoblastoma.

Cancer Med 2021 05 3;10(10):3261-3273. Epub 2021 May 3.

Divison of Pediatric Hematology/Oncology, Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.

Background: Prognostic factors in hepatoblastoma need to be reevaluated considering the advances in treatment modalities. The study aimed to evaluate current outcomes of hepatoblastoma and reappraise the association of prognostic factors, including pre-treatment extent of tumor (PRETEXT) stage with annotation factors and Children's Hepatic tumors International Collaboration-Hepatoblastoma Stratification (CHIC-HS) system, with survival outcomes.

Methods: We evaluated 103 consecutive patients with hepatoblastoma retrospectively according to the treatment period based on the introduction of a liver transplantation program.

Results: The 5-year overall survival (OS), event-free survival (EFS), and transplant-free survival rates were 80.2%, 74.2%, and 61.8%, respectively. EFS and OS were improved significantly from 58.6% to 81.6% (P = 0.024) and from 58.6% to 90.8% (P < 0.001), respectively, in the late period (N = 74) compared with the early period (N = 29). The PRETEXT stage was significant or marginally significant for EFS and OS in the early period but not in the late period. The P, F, R, and C factors were significant for OS and EFS in the early period. However, in the late period, only the P factor was significant for OS, and the F and M factors were significant for EFS. The CHIC-HS system was significant or marginally significant for EFS in both the early and late periods; however, it was significant for OS only in the early period.

Conclusion: Survival rates were significantly improved in children with hepatoblastoma, especially in those with advanced PRETEXT stages with positive annotation factors and in a high-risk CHIC-HS group. Prognostic factors had different clinical implications with evolved treatment modalities.
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http://dx.doi.org/10.1002/cam4.3897DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8124118PMC
May 2021

Real-world experience of olaparib as maintenance therapy in BRCA-mutated recurrent ovarian cancer.

Arch Gynecol Obstet 2021 10 19;304(4):1055-1063. Epub 2021 Apr 19.

Department of Obstetrics and Gynecology, University of Ulsan College of Medicine, Asan Medical Center, 88 Olympic-ro, 43-gil, Songpa-gu, Seoul, 05505, Republic of Korea.

Purpose: The primary objective of our study was to investigate the effectiveness and safety of olaparib maintenance therapy in patients with BRCA-mutated recurrent ovarian cancer in daily practice. The secondary objective of this study was to identify prognostic factors associated with prolonged progression-free survival (PFS) in such patients.

Methods: We conducted a retrospective analysis of 40 patients who received olaparib maintenance treatment. Data on clinicopathological factors, oncological outcomes, and adverse events were obtained from medical records and analyzed.

Results: All patients had high-grade serous recurrent ovarian cancer with BRCA mutation and achieved complete or partial response to the most recent platinum-based chemotherapy. After a median follow-up of 14.3 months, the median PFS was 23.7 months (95% confidence interval, 14.1-33.4); however, the median overall survival was not reached. In the log-rank test, the PFS was significantly longer for patients with most recent platinum-free interval (PFI) ≥ 12 months, complete response to the last platinum-based chemotherapy, and less than three lines of previous chemotherapy (p = 0.005, p = 0.016, and p = 0.023, respectively). Most hematologic and non-hematologic adverse events were of grade 1 or 2, and the common adverse events were mostly related to myelosuppression.

Conclusion: Olaparib maintenance treatment in BRCA-mutated recurrent ovarian cancer is effective and safe in clinical practice. Most recent PFI, response to the last platinum-based chemotherapy, and the number of previous chemotherapy lines were associated with PFS in patients with BRCA-mutated recurrent ovarian cancer.
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http://dx.doi.org/10.1007/s00404-021-06013-xDOI Listing
October 2021

Effect of 5-azacytidine (5-aza) on UCP2 expression in human liver and colon cancer cells.

Int J Med Sci 2021 19;18(10):2176-2186. Epub 2021 Mar 19.

College of Veterinary Medicine & Institute of Veterinary Science, Kangwon National University, Chuncheon 24341, Korea.

The function of the uncoupling protein 2 (UCP2) is different for each cancer cell. However, the mechanism of expression is still unclear. DNA methylation affects protein expression and is one factor that transforms normal cells into cancer cells. In this study, the hepatocellular carcinoma Hep3B and HepG2 cells and colorectal cancer HT-29 cells were treated with 5-azacytidine (5-aza), a DNA demethylation agent, to observe the modification of UCP2 expression and the methylation degree in the UCP2 promoter region. Promoter basal activity and degree of UCP2 expression were measured in Hep3B, HepG2, and HT-29 cells. In addition, methylation-specific PCR (MSP) was performed to investigate the degree of methylation in the UCP2 promoter region. The methylation region in the UCP2 promoter was confirmed based on bisulfite sequencing. In Hep3B cells in which UCP2 mRNA was not transcribed, the promoter basal activity was significantly higher than in HT-29 or HepG2 cells in which UCP2 mRNA was transcribed. Treatment with 5-aza increased UCP2 expression in Hep3B and HT-29 cells; however, the expression in HepG2 cells was unchanged. The UCP2 promoter in Hep3B cells has numerous methylated regions compared with HT-29 and HepG2 cells. The results of the present study revealed that inhibition of UCP2 expression in Hep3B cells was due to methylation of the promoter region. Investigating the mechanism that induces UCP2 expression in cancer cells is important to understand the function of UCP2, which could aid in cancer treatment.
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http://dx.doi.org/10.7150/ijms.56564DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8040421PMC
November 2021

Role of minimally invasive surgery in early ovarian cancer.

Gland Surg 2021 Mar;10(3):1252-1259

Department of Obstetrics and Gynecology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.

Despite having revolutionized the management of multiple types of gynecologic cancers laparoscopy and robotic surgery have had limited utility in ovarian cancer until recently. The development in medical technology allows surgeons to perform minimally invasive surgery (MIS) not only in early ovarian cancer, but also in advanced ovarian cancer. Thus far, most prospective studies showed feasible results of MIS in ovarian cancer. Even with many proven advantages of the MIS, there is no concrete evidence of the disparity in survival rate between laparoscopic, robotic surgery and laparotomy surgery. We reviewed the results of MIS in ovarian cancer thus far and suggest how the gynecologists can apply MIS in ovarian cancer in the future. Until the further prospective studies show solid evidence of safety in the MIS in ovarian cancer, comprehensive discussion about the benefits and risk with the patient and the level of surgical skill of the gynecologist should be considered in determining the type of surgery.
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http://dx.doi.org/10.21037/gs-2019-ursoc-07DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8033075PMC
March 2021

Effects of sirolimus in the treatment of unresectable infantile hemangioma and vascular malformations in children: A single-center experience.

J Vasc Surg Venous Lymphat Disord 2021 11 6;9(6):1488-1494. Epub 2021 Apr 6.

Department of Pediatric Surgery, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.

Objective: Recently, sirolimus has emerged as a safe and effective treatment modality for unresectable vascular lesions. In the present study, we investigated the effectiveness and safety of sirolimus from our early experience with patients with unresectable vascular anomalies.

Methods: The medical records and radiologic images of all patients with unresectable vascular anomalies treated with sirolimus at our center from January 2018 to November 2019 were retrospectively reviewed. All patients were administered oral doses of sirolimus 0.8 mg/m every 12 hours as the initial dose, followed by maintenance of a target serum concentration (5-10 ng/mL) with therapeutic drug monitoring.

Results: Six patients with unresectable vascular anomalies were treated with sirolimus for ≥10 months. Their median age at the initiation of sirolimus treatment was 17 months (range, 8-67 months). The median duration of treatment was 13 months (range, 10-16 months). One patient had a good response, four had an intermediate response, and one had no response to sirolimus therapy. None of the patients had discontinued sirolimus therapy because of adverse effects.

Conclusions: Sirolimus can be used effectively and safely for patients with unresectable vascular anomalies. However, further prospective studies are warranted to evaluate the long-term effects of sirolimus and clarify the indications for early intervention.
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http://dx.doi.org/10.1016/j.jvsv.2021.03.014DOI Listing
November 2021

Risk Factors for Disease Behavior Evolution and Efficacy of Biologics in Reducing Progression in Pediatric Patients with Nonstricturing, Nonpenetrating Crohn's Disease at Diagnosis: A Single-Center Experience in Korea.

Gut Liver 2021 11;15(6):851-857

Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.

Background/aims: : Recently, the treatment of Crohn's disease (CD) has changed to a treat-to-target strategy, in which disease progression is prevented with early intervention. We analyzed the long-term evolution of nonstricturing, nonpenetrating (B1) disease at diagnosis and factors related to disease evolution in pediatric CD.

Methods: We retrospectively analyzed 402 patients between 2000 and 2013 who were younger than 18 years and had B1 disease at CD diagnosis. The median follow-up was 6.1 years (range, 1 to 13 years). The cumulative probabilities of developing stricturing (B2) or penetrating (B3) disease and associations between risk factors and disease behavior evolution were evaluated.

Results: Among the 402 patients, 75 (18.7%) had B2 or B3 disease by the final follow-up. The cumulative probabilities of disease behavior evolution were 18.3%, 34.3%, and 50.9% at 5, 10, and 13 years, respectively. Patients whose disease progressed had an increased risk of intestinal resection (hazard ratio [HR], 3.61; 95% confidence interval [CI], 2.25 to 6.03; p<0.001). First-degree family history of inflammatory bowel disease (HR, 2.38; 95% CI, 1.07 to 5.28; p=0.032), isolated ileal involvement at diagnosis (HR, 7.55; 95% CI, 1.04 to 15.57; p=0.045), and positive anti-Saccharomyces cerevisiae antibody titers (HR, 2.10; 95% CI, 1.03 to 4.25; p=0.040) were associated with disease behavior evolution. Early treatment with biologics significantly reduced disease progression (HR, 0.46; 95% CI, 0.79 to 3.39; p=0.042).

Conclusions: This study suggests that early aggressive therapy should be considered in B1 behavior pediatric CD patients with risk factors of disease evolution to improve long-term outcomes.
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http://dx.doi.org/10.5009/gnl20279DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8593514PMC
November 2021
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