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.

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.

OBJECTIVE: Acupotomy is a modern acupuncture method that includes modern surgical methods. Since acupotomy is relatively more invasive than filiform acupuncture treatment, it is important to establish the safety profile of this practice. To justify further large-scale prospective observational studies, this preliminary study was performed to assess the feasibility of the approach and investigate the safety profile and factors potentially associated with adverse events (AEs). METHODS: This was a prospective pilot study that assessed the feasibility of a large-scale forthcoming safety study on acupotomy treatment in a real-world setting. The feasibility (call response rate, drop-out rate, response rate for each variable and recruitment per month) and safety profile (incidence, type, severity and causality of AEs, and factors potentially associated with AEs) were measured. RESULTS: A total of 28 participants joined the study from January to May 2018. A follow-up assessment was achieved in 258 (1185 treatment points) out of 261 sessions (1214 treatment points). The response rate via telephone on the day after treatment was 87.3%. There were 8 systemic AEs in all the sessions (8/258; 3.11%) and 27 local AEs on the total points treated (27/1185; 2.28%). Severe AEs did not occur. Total AE and local AE occurrence were associated with blade width and the number of needle stimulations per treatment point. CONCLUSION: The findings suggest that it could be feasible to analyze the safety of acupotomy in a real-world setting. Moreover, the primary data on some relevant AEs could be determined. We are planning large-scale prospective studies based on these findings. TRIAL REGISTRATION Clinical Research Information Service (CRIS) KCT0002849 (https://cris.nih.go.kr/cris/search/detailSearch.do/11487).
Humans ; Feasibility Studies ; Prospective Studies ; Pilot Projects ; Acupuncture Therapy/methods* ; Research Design ; Treatment Outcome

Background/Aims: Pheochromocytoma and paraganglioma (PPGL) are catecholamine-producing tumors that can cause blood pressure (BP) elevation and cardiovascular complications. Clinical presentation of these tumors may be changed through widespread use of imaging studies, which enables detection of PPGLs before onset of symptoms. We investigated clinical profiles of patients with surgically resected PPGLs. Methods: From 2005 to 2017, 111 consecutive patients with surgically resected PPGLs in two tertiary hospitals in Korea were studied. Results: Mean age was 52 ± 16 years, 57 patients (51.4%) were male and 54 (48.6%) were hypertensive. Twenty-nine PPGLs (26.1%) were extra-adrenal paragangliomas. Sixteen (14.4%) and seven patients (6.3%) (Group 1, n = 23) were diagnosed during work-up of hypertension and transient cardiomyopathy respectively, and the remainder (Group 2, n = 88) were incidentalomas detected during routine abdominal imaging. Patients in the Group 1 were younger and more frequently symptomatic, and had higher BPs, heart rates and levels of urinary catecholamines than those in the Group 2. Paragangliomas were less frequent and secretion of epinephrine and metanephrine was more predominant in the Group 1 than in Group 2. After the surgical resections, 18.2% of patients still needed antihypertensive medications. Conclusions Out of 111 patients with surgically resected PPGLs, 88 (79.3%) were diagnosed as incidentalomas. Seven patients presented with transient cardiomyopathy and 16 with hypertension. Tumor location and secretion of catecholamine may vary depending on the presence of symptoms.

PURPOSE: Nutritional therapy (NT), such as enteral nutrition (EN) or parenteral nutrition (PN), is essential for the malnourished patients. Although the complications related to NT has been well described, multicenter data on symptoms in the patients with receiving NT during hospitalization are still lacking. METHODS: Nutrition support team (NST) consultations, on which NT-related complications were described, were collected retrospectively for one year. The inclusion criteria were patients who were (1) older than 18 years, (2) hospitalized, and (3) receiving EN or PN at the time of NST consultation. The patients' demographics (age, sex, body mass index [BMI]), type of NT and type of complication were collected. To compare the severity of each complication, the intensive care unit (ICU) admission, hospital stay, and type of discharge were also collected. RESULTS: A total of 14,600 NT-related complications were collected from 13,418 cases from 27 hospitals in Korea. The mean age and BMI were 65.4 years and 21.8 kg/m2. The complications according to the type of NT, calorie deficiency (32.4%, n=1,229) and diarrhea (21.6%, n=820) were most common in EN. Similarly, calorie deficiency (56.8%, n=4,030) and GI problem except for diarrhea (8.6%, n=611) were most common in PN. Regarding the clinical outcomes, 18.7% (n=2,158) finally expired, 58.1% (n=7,027) were admitted to ICU, and the mean hospital days after NT-related complication were 31.3 days. Volume overload (odds ratio [OR]=3.48) and renal abnormality (OR=2.50) were closely associated with hospital death; hyperammonemia (OR=3.09) and renal abnormality (OR=2.77) were associated with ICU admission; “micronutrient and vitamin deficiency” (geometric mean [GM]=2.23) and volume overload (GM=1.61) were associated with a longer hospital stay. CONCLUSION: NT may induce or be associated with several complications, and some of them may seriously affect the patient's outcome. NST personnel in each hospital should be aware of each problem during nutritional support.
Adult ; Body Mass Index ; Demography ; Diarrhea ; Enteral Nutrition ; Hospitalization ; Humans ; Hyperammonemia ; Intensive Care Units ; Korea ; Length of Stay ; Multicenter Studies as Topic ; Nutrition Therapy ; Nutritional Support ; Parenteral Nutrition ; Referral and Consultation ; Retrospective Studies ; Vitamins

PURPOSE: Laparoscopy-assisted distal gastrectomy for early gastric cancer has gained acceptance and popularity worldwide. However, laparoscopy-assisted distal gastrectomy for advanced gastric cancer is still controversial. Therefore, we propose this prospective randomized controlled multi-center trial in order to evaluate the safety and feasibility of laparoscopy assisted D2-gastrectomy for advanced stage gastric cancer. MATERIALS AND METHODS: Patients undergoing distal gastrectomy for advanced gastric cancer staged cT2/3/4 cN0/1/2/3a cM0 by endoscopy and computed tomography are eligible for enrollment after giving their informed consent. Patients will be randomized either to laparoscopy-assisted distal gastrectomy or open distal gastrectomy. Sample size calculation revealed that 102 patients are to be included per treatment arm. The primary endpoint is the non-compliance rate of D2 dissection; relevant secondary endpoints are three-year disease free survival, surgical and postoperative complications, hospital stay and unanimity rate of D2 dissection evaluated by reviewing the intraoperative video documentation. DISCUSSION: Oncologic safety is the major concern regarding laparoscopy-assisted distal gastrectomy for advanced gastric cancer. Therefore, the non-compliance rate of clearing the N2 area was chosen as the most important parameter for the technical feasibility of the laparoscopic procedure. Furthermore, surgical quality will be carefully reviewed, that is, three independent experts will review the video records and score with a check list. For a long-term result, disease free survival is considered a secondary endpoint for this trial. This study will offer promising evidence of the feasibility and safety of Laparoscopy-assisted distal gastrectomy for advanced gastric cancer.Trial Registration: NCT01088204 (international), NCCCTS-09-448 (Korea).
Arm ; Disease-Free Survival ; Endoscopy ; Gastrectomy ; Humans ; Informed Consent ; Laparoscopy ; Length of Stay ; Lymph Node Excision ; Lymph Nodes ; Postoperative Complications ; Prospective Studies ; Sample Size ; Stomach Neoplasms

Churg-Strauss syndrome (CSS) is an uncommon disorder that is characterized by asthma or allergic rhinitis, fever, systemic vasculitis, extravascular granulomas and hypereosinophilia. Cutaneous manifestations of CSS are one of the most common extra-pulmonary findings, and they occur in approximately 60% of the cases. Other manifestations include pulmonary infiltrates and heart, kidney, gastrointestinal tract, peripheral and central nervous system involvement and the patients also complain of systemic symptoms, including malaise, fever, myalgia and weight loss. The diagnosis of CSS is difficult because the manifestations vary with the disease stage and delayed treatment can lead to fatal major organ damage. We report here on 3 cases of CSS that were first diagnosed by the dermatologic department and with consultation from other departments. So, we want to remind dermatologists that if the patients of asthma or other allergic diseases have purpuric skin lesion, then CSS should be included in the differential diagnosis.
Asthma ; Central Nervous System ; Churg-Strauss Syndrome ; Diagnosis, Differential ; Fever ; Gastrointestinal Tract ; Granuloma ; Heart ; Humans ; Kidney ; Rhinitis ; Rhinitis, Allergic, Perennial ; Skin ; Systemic Vasculitis ; Vasculitis ; Weight Loss

Pigmented spindle cell nevus (PSCN) is a solitary heavily pigmented macule or papule that usually occurs on the lower legs of women in the second decade of life. Histologically, fascicles of uniform, elongated, pigmented nevoid cells at the dermo-epidermal junction tend to fuse with neighboring nests. PSCN should be differentiated from Spitz nevus, dysplastic nevus, and malignant melanoma. For making the differential diagnosis of PSCN from malignant melanoma of nevoid cells, the overall symmetry, relative uniformity, maturation with depth and no nuclear atypia are important clues. A 3-year-old girl presented with a black papule on the anterior aspect of the ankle. We diagnosed PSCN by the histologic findings and the specific "starburst" dermoscopic pattern.
Animals ; Ankle ; Dermoscopy ; Diagnosis, Differential ; Dysplastic Nevus Syndrome ; Female ; Humans ; Leg ; Melanoma ; Nevus, Epithelioid and Spindle Cell ; Nevus, Spindle Cell ; Preschool Child

BACKGROUND: It has been known that alveolar macrophage exposed to bacterial lipopolysaccharide (LPS) induces a lots of nitric oxide (NO) and inducible nitric oxide synthase (iNOS) mRNA expression. The Authors elucidated the effects of iNOS mRNA expression by inhalational anesthetics (isoflurane) on endotoxemic rats and positive pressure ventilation with and without LPS. METHODS: Fifty Sprague-Dawley rats (250 - 270 g) were anesthesized with urethane injected in the peritoneal cavity. Then the expression of iNOS mRNA in the alveolar macrophages of the rats were measured after injection of LPS, 2 hours of isoflurane (0.5 - 2.5%) anesthesia, and 2 hours of positive pressure ventilation. The activities of iNOS in macrophages were measured by analysing iNOS mRNA expression by Northern blot analysis with autoradiography using the polymerase chain reaction (PCR) method. RESULTS: The size and patterns of the iNOS mRNA band in the 0.5 - 2% isoflurane group were almost same as with the control group. The size of the iNOS mRNA band in the 2.5% isoflurane group increased more than in the control group. In the continous positive-pressure ventilation with LPS group, the iNOS mRNA band was slightly increased compared to the normal lung and the continous positive-pressure ventilation without LPS group. CONCLUSIONS: Higher concentrations of isoflurane anesthesia may evoke the expression of iNOS mRNA in a septic model. Positive pressure ventilation in sepsis may induce iNOS mRNA production.
Anesthesia ; Anesthetics ; Animals ; Autoradiography ; Blotting, Northern ; Isoflurane* ; Lung ; Macrophages ; Macrophages, Alveolar ; Nitric Oxide ; Nitric Oxide Synthase Type II ; Peritoneal Cavity ; Polymerase Chain Reaction ; Positive-Pressure Respiration ; Rats* ; Rats, Sprague-Dawley ; RNA, Messenger* ; Sepsis ; Urethane

BACKGROUND: In Korean traditional medicine, human beings are classified into 4 groups accordance with the characteristics of physical constitution, named; Taeum, Taeyang, Soum, and Soyang. Most of Korean traditional doctors believe that the back ground of pathophysiology is differ among each group in many diseases. HLA is the most polymorphic gene and related with human immune response. It has been used for determination of individual identification, genetic diversity and a study of disease association in different ethnic group. It has well known that the most human inflammatory disease which thought to have some autoimmune basis are in some way promoted by genes of the HLA region. The purpose of this study is to observe the distribution of HLA-DR genotypes in 4 types of physical constitution in Korean traditional medicine. METHODS: Three hundred and ten Korean subjects(Soyang : 58cases, Soum :142cases, Taeum : 92cases, Taeyang : 18cases) were enrolled for this study. Genomic DNAs extracted by phenol/chroloform were amplified with allele specific primers and determined by 2% agarose gel electrophoresis with ethidium bromide staining for analysis of HLA-DR polymorphism. RESULTS: The genotype frequency of HLA-DR in a total or 310 Koreans was DR1 ; 7.2%, DR 2; 10.5%, DR 3;1.7%, DR 4; 19.9%, DR 5; 11.9%, DR 6;19.9%, DR 7; 7.0%, DR 8; 10.6%, DR 9; 8.7%, DR10;1.9%. There was no significant diffrence of HLA-DR genotype distribution among four physical constitution groups classified by Korean traditional medicine. CONCLUSION: Our results suggest that the classification of physical constitution in Korean traditional medicine is not related to HLA-DR genotypes.
Alleles ; Classification ; Constitution and Bylaws* ; DNA ; Electrophoresis, Agar Gel ; Ethidium ; Ethnic Groups ; Genetic Variation ; Genotype ; HLA-DR Antigens* ; Humans ; Medicine, Korean Traditional*