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.

No abstract available.
Carcinoid Tumor* ; Proctitis*

No abstract available.
Carcinoid Tumor* ; Proctitis*

Emphysematous gastritis is a rare disorder characterized by emphysematous change of the gastric wall due to infection with a gas-forming organism. Acute necrotizing esophagitis is a rare disorder with an unknown pathogenesis. Above two disorders rarely occur together, only three global cases have been reported to date. Such a case has never been reported in Korea, we report a novel case of severe emphysematous gastritis with concomitant portal venous air and acute necrotizing esophagitis in type 1 diabetes presenting with diabetic ketoacidosis. A 24-year-old man known to have type 1 diabetes and pulmonary tuberculosis was brought to the emergency room for epigastric pain with vomiting. His body mass index was 14.7, and the laboratory findings demonstrated leukocytosis and acidosis, as well as elevated serum glucose, ketone, and C-reactive protein levels. Enhanced computed tomography showed portal vein gas and edematous wall thickening without enhancement in the stomach wall, with air density along the stomach and esophageal wall. The patient required surgical intervention of total gastrectomy and cervical esophagostomy followed by esophagocolostomy and esophageal reconstruction. Early radiologic diagnosis and clinical suspicion of this disease and prompt intervention including antibiotics, decompression, and surgery are important for a good prognosis.
Acidosis ; Anti-Bacterial Agents ; Blood Glucose ; Body Mass Index ; C-Reactive Protein ; Decompression ; Diabetic Ketoacidosis* ; Diagnosis ; Emergency Service, Hospital ; Esophagitis* ; Esophagostomy ; Gastrectomy ; Gastritis* ; Humans ; Korea ; Leukocytosis ; Portal Vein ; Prognosis ; Stomach ; Tuberculosis, Pulmonary ; Vomiting ; Young Adult

Development of transfusion-related acute lung injury (TRALI), a non-cardiogenic pulmonary edema, after blood transfusion, is a rare but potentially leading cause of mortality from blood transfusion. We report on a case of TRALI in a 51-year male with acute calculous cholecystitis and liver cirrhosis. As preoperative treatment, he was given ten units of fresh frozen plasma (FFP) for 3 days before the operation. During the transfusion of the 10th unit of FFP, he experienced a sudden onset of hemoptysis, tachypnea, tachycardia, and cyanosis. Bilateral pulmonary infiltration not observed on the chest X-ray at the visit was newly developed. There was no evidence of volume overload but severe hypoxemia. Blood transfusion was stopped and he recovered fully after 8 days of oxygen therapy through a nasal cannula. Although HLA and HNA antibodies were not detected in the donor's blood, HLA antibodies (A2, B57, B58) were detected in the patient's blood. We reported this meaningful case of TRALI that occurred after transfusion of only fresh frozen plasma which did not contain human leukocyte antibody in a patient with HLA antibody.
Acute Lung Injury* ; Anoxia ; Antibodies ; Blood Transfusion ; Catheters ; Cholecystitis ; Cyanosis ; Hemoptysis ; Humans ; Leukocytes ; Liver Cirrhosis ; Male ; Mortality ; Oxygen ; Plasma* ; Pulmonary Edema ; Tachycardia ; Tachypnea ; Thorax

A pleurobiliary fistula is an abnormal communication between the biliary system and the pleural space. It has rarely been reported after percutaneous transhepatic gallbladder drainage (PTGBD). Here, we report the case of an 88-year-old man with bilious pleural infection via pleurobiliary fistula following PTGBD. The patient had a fever, dyspnea and right pleuritic chest pain. The PTGBD was performed 2 months prior to treat acute cholecystitis with large gallstones. Chest radiography demonstrated a right pleural effusion and a computed tomography of the abdomen showed a pleurobiliary fistula tract associated with the previous PTGBD. A drainage tube was inserted into the right pleural effusion, and the bilious pleural fluid infected with Escherichia coli was drained. Careful approach to PTGBD procedure and reducing duration of catheter placement should prevent fistula formation. As a rare complication of PTGBD, practitioners should be aware of the potential of pleural infection by a pleurobiliary fistula tract.
Abdomen ; Aged, 80 and over ; Biliary Fistula ; Biliary Tract ; Catheters ; Chest Pain ; Cholecystitis, Acute ; Drainage* ; Dyspnea ; Escherichia coli ; Fever ; Fistula* ; Gallbladder* ; Gallstones ; Humans ; Pleural Effusion ; Radiography ; Thorax

PURPOSE: The aim of this study was to evaluate changes in expressions of neuregulin (NRG)1 and erbB2 tyrosine kinase (ErbB2) in bladders of rats with cyclophosphamide (CYP)-induced interstitial cystitis (IC). METHODS: Twenty-four Sprague-Dawley rats were divided into the IC group (n=16) and the control group (n=8). After inducing IC with intraperitoneal CYP injection, expressions of NRG1 and ErbB2 were analyzed using western blotting and reverse transcriptase-polymerase chain reaction. RESULTS: In Western blotting, relative intensities and distributions of both NRG1 and ErbB2 were approximately 1.5- and 3.2-fold higher, respectively, in the IC group than in the control group (mean+/-standard deviation: 1.42+/-0.09 vs. 0.93+/-0.15 and 0.93+/-0.16 vs. 0.29+/-0.08, P<0.05). In the rat bladder samples, mRNA expression levels of NRG1 and ErbB2 were higher in the IC group than in the control group (P<0.05). CONCLUSIONS: Our study has demonstrated significant changes in mRNA expression and immunoreactivity of NRG1 and ErbB2 receptors in the urinary bladder after CYP-induced IC. These results suggest that the up-regulated NRG1 may play a role in inducing an overactive bladder and promoting regeneration in the inflammatory bladder with CYP-induced IC.
Animals ; Blotting, Western ; Cyclophosphamide ; Cystitis, Interstitial* ; Neuregulin-1* ; Protein-Tyrosine Kinases* ; Rats* ; Rats, Sprague-Dawley ; Receptor, erbB-2 ; Regeneration ; RNA, Messenger ; Tyrosine* ; Urinary Bladder* ; Urinary Bladder, Overactive

Propofol is an intravenous hypnotic agent that is generally used for sedation in the intensive care unit and for induction of anesthesia during minimally invasive surgery, endoscopy, and plastic surgery in local clinics. Low blood pressure and transient apnea might occur under propofol sedation, whereas stress-induced cardiomyopathy is a very rare complication. We herein describe a case involving a 25-year-old woman without cardiovascular risk factors who developed stress-induced cardiomyopathy after propofol injection for anesthesia and was treated with conservative treatment. This case reminds us that clinicians should consider the possible occurrence of stress-induced cardiomyopathy after anesthesia using propofol, even in patients without cardiovascular risk factors.
Adult ; Anesthesia* ; Apnea ; Cardiomyopathies* ; Endoscopy ; Female ; Humans ; Hypotension ; Intensive Care Units ; Propofol* ; Risk Factors ; Surgery, Plastic ; Surgical Procedures, Minimally Invasive

PURPOSE: We have performed both open partial nephrectomy (OPN) and laparoscopic radiofrequency ablation (RFA) on selected patients since January 2007 and have been following these patients through serial laboratory assessments and computed tomography (CT). The purpose of the present study was to evaluate long-term oncologic outcomes and renal function status for laparoscopic RFA versus OPN at a minimum follow-up of 3 years. MATERIALS AND METHODS: A total of 55 patients with exophytic, single small renal masses were treated with either OPN (n=14) or laparoscopic RFA (n=41) by a single surgeon. The indications for laparoscopic RFA were as follows: 1) cases with the greatest dimension of the renal mass <3 cm, and 2) cases in which the collecting system, renal calyx, and great vessels were free from the tumor margins by 1 cm. RESULTS: The estimated blood loss (EBL), the operation time, and the mean number of hospital days was significantly lower in the laparoscopic RFA group than in the OPN group. Oncologic data did not differ significantly between the two groups. Creatine clearance levels did not differ significantly compared with those before the operation in either group. CONCLUSIONS: Our data suggest excellent therapeutic outcomes with laparoscopic RFA with achievement of effective operative times, hospital stays, and EBL compared with OPN. According to our indications for laparoscopic RFA, laparoscopic RFA is an effective minimally invasive therapy for the treatment of small renal masses, yielding oncologic outcomes and renal function equivalent to those of OPN.
Catheter Ablation ; Creatine ; Follow-Up Studies ; Humans ; Kidney Neoplasms ; Length of Stay ; Nephrectomy ; Operative Time