Hepatocellular carcinoma (HCC) is a major cause of cancer-related mortality worldwide. The recent introduction of immune checkpoint inhibitors (ICIs) has transformed the therapeutic landscape for advanced HCC. Combination regimens such as atezolizumab plus bevacizumab, durvalumab plus tremelimumab, and nivolumab plus ipilimumab have demonstrated significant survival improvements over conventional tyrosine kinase inhibitors and have become the new standard of care. However, ICIs can trigger immune-related adverse events (irAEs) through overactivation of the immune system, affecting multiple organs including the skin, gastrointestinal tract, liver, endocrine system, lungs, and heart. Patients with HCC frequently have underlying liver diseases such as chronic hepatitis or cirrhosis, placing them at higher risk of hepatic irAEs compared to that with other cancer types, which can markedly influence prognosis. The pathophysiology of irAEs is driven by a series of interconnected immune mechanisms, including excessive T-cell activation, disruption of immune tolerance, cytokine dysregulation, complement-mediated injury, and innate immune activation. Clinical decisions regarding the continuation, interruption, or discontinuation of ICIs, as well as the administration of corticosteroids or immunosuppressants, should be guided by the severity of toxicity. Organ-specific management strategies and multidisciplinary collaboration are essential, particularly for severe presentations. This review summarizes the incidence, mechanisms, and management strategies for ICI-related irAEs in advanced HCC, and provides practical insights for clinical decision-making.

Purpose: To report a nationwide survey on neuroblastoma conducted by the Korean Association of Pediatric Surgeons (KAPS) in 2020. Methods: The clinical data of pediatric patients diagnosed with and treated for neuroblastoma from 2005 to 2019 in 19 hospitals of KAPS members were collected. Survival and prognostic factor analyses were performed using the log rank test and Cox proportional hazard analysis. A p-value <0.05 was considered significant. Results: A total of 669 patients with neuroblastoma were registered for the study. The results were presented and discussed at the 36th annual meeting of the KAPS, which was held in Seoul on August 21, 2020. Conclusion This study provides information on patient demographics, prognostic outcomes, and comprehensive treatment outcomes for neuroblastoma. The study is expected to be an important reference for improving pediatric surgeons’ understanding and treatment of neuroblastoma.

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.

Gitelman syndrome is a condition caused by a mutation of the thiazide sensitive Na-Cl cotransporter gene on the distal convoluted tubule. It results in a variety of clinical features, including hypokalemia, hypomagnesemia, hypocalciuria, and metabolic alkalosis. It is often diagnosed in asymptomatic adults presented with unexplained hypokalemia; however, it is sometimes associated with muscular cramps, numbness, fatigue, weakness, or paralysis. We experienced a case of rheumatoid arthritis accompanied by Gitelman syndrome, presented with hand tremor. We diagnosed her using renal clearance study and genetic analysis. Here, we report our experiences regarding this case along with a literature review.
Adult ; Alkalosis ; Arthritis, Rheumatoid* ; Fatigue ; Furosemide ; Genetic Testing ; Gitelman Syndrome* ; Hand ; Humans ; Hypesthesia ; Hypokalemia ; Muscle Cramp ; Paralysis ; Solute Carrier Family 12, Member 3 ; Thiazides ; Tremor

No abstract available.
Carcinoid Tumor* ; Proctitis*

No abstract available.
Carcinoid Tumor* ; Proctitis*

Gitelman syndrome is a condition caused by a mutation of the thiazide sensitive Na-Cl cotransporter gene on the distal convoluted tubule. It results in a variety of clinical features, including hypokalemia, hypomagnesemia, hypocalciuria, and metabolic alkalosis. It is often diagnosed in asymptomatic adults presented with unexplained hypokalemia; however, it is sometimes associated with muscular cramps, numbness, fatigue, weakness, or paralysis. We experienced a case of rheumatoid arthritis accompanied by Gitelman syndrome, presented with hand tremor. We diagnosed her using renal clearance study and genetic analysis. Here, we report our experiences regarding this case along with a literature review.
Adult ; Alkalosis ; Arthritis, Rheumatoid ; Fatigue ; Furosemide ; Genetic Testing ; Gitelman Syndrome ; Hand ; Humans ; Hypesthesia ; Hypokalemia ; Muscle Cramp ; Paralysis ; Solute Carrier Family 12, Member 3 ; Thiazides ; Tremor

Serotonin syndrome, an adverse drug reaction, is a consequence of excess serotonergic agonism of central nervous system receptors and peripheral serotonergic receptors. Serotonin syndrome has been associated with large numbers of drugs and drug combinations, and serotonin-norepinephrine reuptake inhibitor-induced serotonin syndrome is rare. It is often described as a sign of excess serotonin ranging from tremor in mild cases to delirium, neuromuscular rigidity, and hyperthermia in life-threatening cases. Diagnosis is based on the symptoms and patient's history, and several diagnostic criteria have been developed. We experienced a rare case of fibromyalgia accompanied by tremor, hyperreflexia, spontaneous clonus, muscle rigidity, and diaphoresis after 10 days of single use of duloxetine 30 mg. Only one case of serotonin syndrome resulting from administration of duloxetine has been reported in Korea, however that case resulted from co-administration of fluoxetine. We report here on this case along with a review of the relevant literature.
Central Nervous System ; Delirium ; Diagnosis ; Drug Combinations ; Drug-Related Side Effects and Adverse Reactions ; Duloxetine Hydrochloride* ; Felodipine ; Fever ; Fibromyalgia* ; Fluoxetine ; Humans ; Korea ; Muscle Rigidity ; Reflex, Abnormal ; Serotonin Syndrome* ; Serotonin* ; Tremor

OBJECTIVE: Although previous trials suggested a relationship between neutrophil-lymphocyte ratio (NLR), platelet-lymphocyte ratio (PLR), and systemic inflammatory response, clinical utility of NLR and PLR in rheumatoid arthritis (RA) is not well defined. This study was conducted to assess the efficiency of NLR and PLR as an inflammatory index in patients with RA. METHODS: A total of 107 patients with newly diagnosed RA who had never used steroid and a control group of 50 age- and gender-matched healthy subjects whose high sensitive C-reactive protein (hsCRP) was within normal range were included. Those with cerebrovascular diseases, diabetes, malignancies, or any cardiovascular diseases were excluded from both groups. The patients were divided into two groups according to the Disease Activity Score of 28 joints (DAS28). Group 1 included patients with a DAS28 score of 3.2 and lower (low disease activity) and group 2 included patients with a score higher than 3.2 (moderate to high disease activity). RESULTS: NLR and PLR in the patient group were 2.99±2.04, 170.90±86.49, significantly higher than that of the control group. NLR and PLR in group 2 were 4.16±2.50, 225.23±93.21, significantly higher than those of group 1 patients (2.26±1.22, 137.15±61.92). NLR and PLR both showed correlation with rheumatoid factor, hsCRP, serum albumin, Korean Heath Assesment Questionnaire, and DAS28. CONCLUSION: These data showed a positive correlation between NLR or PLR level and RA disease activity, suggesting that NLR or PLR can be used as an additional inflammatory marker in patients with RA.
Arthritis, Rheumatoid* ; C-Reactive Protein ; Cardiovascular Diseases ; Equidae ; Humans ; Joints ; Reference Values ; Rheumatoid Factor ; Serum Albumin

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