Background/Aims: Patients with postural tachycardia syndrome (POTS) commonly experience gastrointestinal (GI) symptoms. We aim to assess the feasibility and preliminary efficacy data for DA-9701, a prokinetic agent targeting 5-hy-droxytryptamine 1A, 5-hydroxytryptamine 4, and dopamine D 2 receptors, in patients with POTS. Methods: In a randomized, double-blind, placebo-controlled, single-center crossover trial, patients with POTS were given either 30 mg of DA-9701 or a placebo 3 times daily for eight weeks in a 1:1 ratio. After a 4-week washout, patients received the alternate treatment for another 8 weeks. The primary endpoint focused on assessing the change in GI symptoms (total Nepean Dyspepsia Index-Korean version [NDI-K] symptom score) from baseline over the 8 week-treatment period. Endpoints were assessed in all enrolled and randomized patients (intention-to-treat), and in those who completed the trial (per-protocol analysis). Results: Between January 2022 and August 2023, 24 patients were randomized (n = 12 per group), with 3 discontinuing after randomization. DA-9701 did not significantly improve primary endpoints for total NDI-K symptom scores in either the intention-to-treat (least-squares means, –13.9 vs. –9.5, P = 0.326) or per-protocol analyses (–17.2 vs –12.0, P = 0.242).Notably, a trend toward improvement in specific GI symptoms, such as upper abdominal pain, was observed in both intention-to-treat (–0.6 vs 0.7; P = 0.066) and per-protocol analyses (–0.9 vs 0.6; P = 0.045). No serious adverse events were observed. Conclusion DA-9701 did not improve GI symptoms in this crossover trial; however, its potential effect on specific GI symptoms merits further investigation.

Infectious disease constitutes on one of the main causes of death that is identified on forensic autopsies. However, the postmortem diagnosis of these diseases and the assessment of their severity constitute major challenges. Procalcitonin (PCT), which is used as a biomarker for sepsis in clinical settings, has been applied in the context of forensic autopsies. In this study, we aimed to evaluate the utility of PCT testing in the postmortem diagnosis of infectious disease. This retrospective analysis included cases wherein autopsies with PCT testing were performed at the National Forensic Service between November 2023 and December 2024. Relevant data were collected through a comprehensive review of autopsy reports. Although the PCT concentrations in the infectious disease group were significantly higher than those in the control group, clinical sepsis criteria showed limited sensitivity in the postmortem diagnosis of infectious diseases. The comparison of clinical medical records and postmortem laboratory results revealed that PCT concentrations decreased after death. Thus, despite the usefulness of PCT testing for the postmortem diagnosis and determination of death from infectious diseases, the results should be interpreted comprehensively in conjunction with other autopsy findings rather than being used as a standalone criterion.

Purpose: Biliary atresia (BA) is a rare but progressive cholangiopathy and the leading cause of pediatric liver transplantation worldwide. The Korean Association of Pediatric Surgeons (KAPS) has conducted three national surveys (2001, 2011, and 2023) to assess long-term trends in the diagnosis, treatment, and outcomes of BA. This study provides a comparative analysis of the 2nd and 3rd national surveys, with reference to selected findings from the 1st survey. Methods: This study included 453 patients from the 3rd national survey (2011–2021) and 435 patients from the 2nd survey (2001–2010), all of whom underwent Kasai portoenterostomy. Data were collected via electronic case report forms from pediatric surgical centers nationwide. Comparisons were made regarding demographics, clinical features, diagnostic patterns, operative details, follow-up outcomes, and survival. Kaplan–Meier analysis was used to evaluate long-term survival. Results: The mean number of BA patients per year remained stable between surveys (43.5 in the 2nd, 41.18 in the 3rd), though centralization of care increased, with 61.5% of cases managed by two major institutions in the 3rd survey. The median age at surgery decreased, and the use of preoperative imaging (especially magnetic resonance cholangiopancreatography) increased. The 10-year native liver survival rate declined from 59.8% to 53.7%, while overall 10-year survival improved slightly (92.9% to 93.2%). Postoperative complications, such as cholangitis and liver failure, persisted but were better categorized. The 3rd survey also reported improved mortality (4.9%) and reduced follow-up loss (11.5%) compared to the 2nd survey. Conclusion While overall survival after Kasai operation has remained high and even improved, native liver survival has slightly declined. The findings reflect earlier diagnosis, more consistent diagnostic imaging, and increasing centralization of care. These trends underscore the importance of long-term nationwide data collection in guiding future strategies for BA management in Korea.

Pediatric intestinal perforation is a surgical emergency that must be promptly addressed regardless of the specific cause. Here we report a case of colon perforation caused by indeterminate inflammatory bowel disease (IBD) in an autistic 13-year-old boy. Ulcerative colitis (UC) and lymphoma were first suspected but subsequently ruled out. The patient was previously hospitalized locally for 8 days due to diarrhea. He was diagnosed with UC and colon perforation in the emergency room. He then underwent subtotal colectomy with end ileostomy. Pathological examination of the colon showed multiple perforations with absence of chronic crypt change (a characteristic of UC), presence of undermining ulcers, and atypical lymphocyte infiltrations. Lymphoma was ruled out from immunohistochemistry and blood tests. Indeterminate colitis was finally suggested as the cause of perforation. Genetic analysis confirmed KBG syndrome, but no abnormalities otherwise known to be relevant to colitis. This case demonstrates that spontaneous colon perforation might occur in KBG syndrome patients suffering from severe enteritis without IBD, malignancy, or other conditions known to cause perforation, supporting the necessity of close monitoring when such patients present with severe symptoms including fever and abdominal distension without showing improvement.

Purpose: This study aims to investigate and compare the incidence, demographic characteristics, clinical manifestations, preoperative diagnostic methods, anatomical classifications, associated anomalies, operative treatments, and postoperative outcomes of patients with intestinal atresia treated by the members of the Korean Association of Pediatric Surgeons (KAPS) through three nationwide surveys. Methods: KAPS conducted 3 national surveys in 1998, 2010, and 2024 to examine the patients diagnosed with intestinal atresia. In preparation for the survey, we developed a customized case registration form to obtain data on patient sex, birth weight, gestational age, clinical manifestations, preoperative diagnostic methods, anatomical types, associated anomalies, operative treatments, and postoperative outcomes. Authorized KAPS members completed the case registration form. Results: The first, second, and third national surveys included 218, 222, and 236 individuals diagnosed with intestinal atresia, respectively. The male-to-female ratios were 1.5:1, 1.1:1, and 1.1:1, respectively. The first, second, and third national surveys revealed that 34.3%, 43.3%, and 53.4% of patients were born before 37 weeks of gestation, respectively. Additionally, 28.7%, 32.0%, and 40.7% of patients had a birth weight under 2,500 g. In the third national survey, duodenoduodenostomy was the most common procedure, performed in 70 out of 82 patients diagnosed with duodenal atresia. Resection and anastomosis were the main surgical procedures conducted in 47 out of 54 cases of jejunal atresia and 74 out of 92 cases of ileal atresia. The mortality rates in the first, second, and third national surveys were 13.8%, 3.6%, and 1.3% respectively, with the lowest rate observed in the third national survey. Conclusion These national surveys offer valuable insights into the current state of intestinal atresia, including specific surgical interventions and postoperative outcomes in South Korea. For pediatric surgeons aiming to enhance their understanding of intestinal atresia and its treatment options, these surveys could be an indispensable resource and guide.

Purpose: This study aimed to compare clinical outcomes between thoracoscopic surgery (TR) and open surgery (OR) for esophageal atresia with distal tracheoesophageal fistula (EA with distal TEF) and to evaluate the feasibility of TR. Methods: We retrospectively analyzed the clinical data of 42 patients who underwent primary surgery for EA with distal TEF from January 2012 to December 2020. We compared the OR and TR groups based on patient characteristics, intraoperative outcomes, and early and late postoperative outcomes. Results: The TR group had longer operation times and a higher risk for intraoperative hypoxic events. Anastomosis leakage and stricture tended to occur more frequently in the TR group, although not statistically significant. The TR group required a significantly higher number of esophageal balloon dilatations (P = 0.006). Conclusion Successful TR for EA with distal TEF requires advanced anesthesia and efforts to overcome the learning curve of surgical skill with limited thoracoscopic instruments for neonates. Despite challenges, TR is considered a feasible method for EA with distal TEF patients when performed by experienced pediatric surgeons with appropriate anesthesia support.

Purpose: We evaluated factors predicting the recurrence of macular edema in patients with branch retinal vein occlusion using optical coherence tomography (OCT). Methods: This study enrolled 55 patients diagnosed with branch retinal vein occlusion who presented to the outpatient clinic between March 2022 and March 2023. A retrospective analysis categorized patients into non-recurrence and recurrence groups. Data on visual acuity, spherical equivalent, intraocular pressure, number of injections, and follow-up duration were collected from medical records. OCT images were obtained before and 6 months after intravitreal injection to measure and analyze central retinal thickness, subfoveal choroidal thickness, and disorganization of retinal inner layers. Results: No statistically significant difference was observed in the central retinal thickness change before and after treatment between the groups. Although no significant differences were observed in visual acuity between the two groups before treatment, significant improvement in visual acuity was observed in the non-recurrence group after 6 months of treatment. The non-recurrence group was younger compared to the recurrence group; moreover, the intraocular pressure in non-recurrence patients decreased significantly during the 6-month treatment period. In addition, a decrease in subfoveal choroidal thickness before and after treatment, the difference in subfoveal choroidal thickness between the affected eye and the fellow eye before treatment, and the reduction in disorganization of retinal inner layers before and after treatment were associated with a favorable prognosis without recurrence. Conclusions Changes in subfoveal choroidal thickness before and after treatment, variations in subfoveal choroidal thickness between the affected and fellow eyes before treatment, and the degree of disorganization of retinal inner layers exhibited significant associations with the recurrence of macular edema. This is significant because it allows for predictions based on baseline OCT images.

Purpose: To identify initial factors contributing to the resistance against intravitreal aflibercept treatment in polypoidal choroidal vasculopathy (PCV) patients. Methods: This study included PCV patients initially treated with aflibercept. Resistance was defined when treatment did not reduce subretinal fluid (SRF) or intraretinal fluid (IRF) by 100 μm or more after three consecutive 4-week intervals, and the treatment interval could not be extended beyond 8 weeks. To identify initial factors associated with resistance to aflibercept treatment, we examined visual acuity and central choroidal thickness before initial treatment and after three treatments. Choroidal thickness was divided into the thickness of the large choroidal vessel layer and the layer that includes choriocapillaris and medium choroidal vessel thickness (termed medium choroidal vessel/choriocapillaris layer thickness, MCCT). Additionally, the volume of SRF, IRF, subretinal hyperreflective material, and pigment epithelial detachment in optical coherence tomography (OCT) images was investigated. The statistical significance of each factor was assessed through logistic regression analysis. Results: The study included 39 eyes showing no resistance to aflibercept and 37 eyes that exhibited resistance. Multiple logistic regression analysis, adjusted for age and sex, indicated that a lower initial ratio of MCCT to choroidal thickness at the fovea was associated with resistance to aflibercept. Conclusions In patients with PCV treated with aflibercept, early OCT anatomical structures, such as the initial MCCT-choroidal thickness ratio at the fovea, may predict response to treatment injections.

Purpose: This study provides insights into the prevalence at birth, clinical characteristics, and outcomes of gastroschisis and omphalocele in Korea over the past decade, addressing the lack of localized data despite advanced healthcare capabilities. Methods: The study retrospectively analyzed data from 20 pediatric surgical centers in Korea from January 2012 to December 2021, including 269 patients diagnosed with gastroschisis or omphalocele. Data variables included gender, gestational age, birth weight, associated anomalies, type of defect, surgical interventions, and outcomes. Results: The study covered 269 patients, with 80 gastroschisis and 189 omphalocele cases. Gastroschisis prevalence at birth remained stable at 2.15 per 100,000 live births, while omphalocele increased to 5.08 per 100,000. Both conditions had similar gender ratios (0.95). Gastroschisis patients had lower birth weights (2,463.90±505.50 g) and smaller head circumferences (31.97±1.86 cm) compared to omphalocele patients (2,757.65±761.24 g, 32.78±2.64 cm). Omphalocele cases had more associated anomalies, especially cardiovascular issues. Prenatal diagnosis rates were high: 93.7% for gastroschisis and 86.4% for omphalocele. About 96.3% of gastroschisis and 84.1% of omphalocele patients were born in their treatment hospitals. Gastroschisis patients underwent surgery sooner (average 3.5 days) and started feeding later (16.5 days) than omphalocele patients (average 56.5 days to surgery, 6.6 days to start feeding). Hospital stays and follow-up durations were similar, averaging around 782.6 days for gastroschisis and 800.3 days for omphalocele patients. Survival rates were 89.7% for gastroschisis and 87.1% for omphalocele. Conclusion The study highlights the need for early diagnosis, centralized care, and specialized surgical approaches to optimize outcomes for gastroschisis and omphalocele patients in Korea. Enhanced prenatal screening and surgical protocols are recommended to improve these patients' prognosis.

Background: Actinidia polygama (silver vine) has been used in oriental medicine to treat gout, rheumatoid arthritis, and inflammation. Actinidia polygama water extract (APWE) is named PB203. Objective: To investigate whether PB203 has anti-photoaging effects and to understand the molecular mechanism underlying such effects. Methods: The antioxidant effect was assessed by 1,1-diphenyl-2-picrylhydrazyl assay and 2′,7′-dichlorodihydrofluorescein diacetate staining in ultraviolet B (UVB)-irradiated HaCaT cells with or without PB203 treatment. Type I collagen, matrix metalloproteinase-1 (MMP-1), tissue inhibitor of metalloproteinase (TIMP-1), hyaluronic acid (HA), hyaluronan synthase 1 (HAS1) and HAS2 levels were measuring by enzyme-linked immunosorbent assay or reverse transcription quantitative polymerase chain reaction. Also, we investigate the effects of PB203 on wrinkle formation, and the potential mechanisms underlying such effects were investigated in UVB-induced wrinkle mouse model mice. Results: PB203 alleviated the UVB-induced reactive oxygen species production, phosphorylation of JNK, ERK, and p38, and formation of AP-1. In addition, PB203 inhibited the decreases in type I collagen and TIMP-1 levels, and the increase in MMP-1 levels in UVBexposed HaCaT cells. In UVB-induced wrinkle mouse model, PB203 inhibited the decreases in elastin and type I collagen levels as well as the increases in MMP-1 expression, wrinkle formation, and skin dehydration. Furthermore, PB203 increased the expression of filaggrin, HAS1, and HAS2, improving the skin barrier function. Conclusion Taken together, we found that PB203 is as a potent candidate to serve as a functional ingredient or therapeutic agent to improve UVB-mediated skin aging.