Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder primarily affecting children, typically presenting as self-limiting skin lesions. However, extracutaneous JXG (extra-JXG) or systemic JXG (sJXG) can involve various organs, including the central nervous system (CNS), leading to high morbidity and mortality, especially in cases of CNS involvement. Treatment approaches for CNS–JXG, including surgical resection, chemotherapy, and radiation therapy, lack consensus, with reports of relapsed or refractory cases being rare. Here, we present the case of a 2-year-old girl with refractory extra-JXG involving the CNS, characterized by multiple intracranial lesions.Initial chemotherapy with prednisolone and vinblastine showed no response, and second-line therapy, including cytarabine and vincristine combined with surgical resection, was also ineffective. However, a 6-cycle third-line chemotherapy regimen incorporating vincristine and carboplatin led to a positive response, with stable disease status, and normal growth and neurodevelopment observed. Our findings suggest that combined chemotherapy with carboplatin and vincristine is a feasible and well-tolerated treatment strategy for patients with CNS–JXG.

Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder primarily affecting children, typically presenting as self-limiting skin lesions. However, extracutaneous JXG (extra-JXG) or systemic JXG (sJXG) can involve various organs, including the central nervous system (CNS), leading to high morbidity and mortality, especially in cases of CNS involvement. Treatment approaches for CNS–JXG, including surgical resection, chemotherapy, and radiation therapy, lack consensus, with reports of relapsed or refractory cases being rare. Here, we present the case of a 2-year-old girl with refractory extra-JXG involving the CNS, characterized by multiple intracranial lesions.Initial chemotherapy with prednisolone and vinblastine showed no response, and second-line therapy, including cytarabine and vincristine combined with surgical resection, was also ineffective. However, a 6-cycle third-line chemotherapy regimen incorporating vincristine and carboplatin led to a positive response, with stable disease status, and normal growth and neurodevelopment observed. Our findings suggest that combined chemotherapy with carboplatin and vincristine is a feasible and well-tolerated treatment strategy for patients with CNS–JXG.

Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder primarily affecting children, typically presenting as self-limiting skin lesions. However, extracutaneous JXG (extra-JXG) or systemic JXG (sJXG) can involve various organs, including the central nervous system (CNS), leading to high morbidity and mortality, especially in cases of CNS involvement. Treatment approaches for CNS–JXG, including surgical resection, chemotherapy, and radiation therapy, lack consensus, with reports of relapsed or refractory cases being rare. Here, we present the case of a 2-year-old girl with refractory extra-JXG involving the CNS, characterized by multiple intracranial lesions.Initial chemotherapy with prednisolone and vinblastine showed no response, and second-line therapy, including cytarabine and vincristine combined with surgical resection, was also ineffective. However, a 6-cycle third-line chemotherapy regimen incorporating vincristine and carboplatin led to a positive response, with stable disease status, and normal growth and neurodevelopment observed. Our findings suggest that combined chemotherapy with carboplatin and vincristine is a feasible and well-tolerated treatment strategy for patients with CNS–JXG.

Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder primarily affecting children, typically presenting as self-limiting skin lesions. However, extracutaneous JXG (extra-JXG) or systemic JXG (sJXG) can involve various organs, including the central nervous system (CNS), leading to high morbidity and mortality, especially in cases of CNS involvement. Treatment approaches for CNS–JXG, including surgical resection, chemotherapy, and radiation therapy, lack consensus, with reports of relapsed or refractory cases being rare. Here, we present the case of a 2-year-old girl with refractory extra-JXG involving the CNS, characterized by multiple intracranial lesions.Initial chemotherapy with prednisolone and vinblastine showed no response, and second-line therapy, including cytarabine and vincristine combined with surgical resection, was also ineffective. However, a 6-cycle third-line chemotherapy regimen incorporating vincristine and carboplatin led to a positive response, with stable disease status, and normal growth and neurodevelopment observed. Our findings suggest that combined chemotherapy with carboplatin and vincristine is a feasible and well-tolerated treatment strategy for patients with CNS–JXG.

Background: and Purpose Patients with dementia are particularly vulnerable to coronavirus disease 2019 (COVID-19) because they tend to be older and often have concomitant diseases. Previous studies have investigated the impact of dementia on COVID-19 outcomes, but the evidence is not robust for Asian populations. We aimed to determine the relationship between dementia and COVID-19 outcomes using data from a large-scale nationwide public database. Methods: Data on patients with COVID-19 who were released from quarantine between January 1, 2020 and April 30, 2020, published by the Korea Disease Control and Prevention Agency, were divided into two groups based on the dementia status. Propensity-score matching was used to adjust for multiple confounders between the dementia and no-dementia groups. Binary, ordinal logistic regression and multivariate Cox proportional-hazards models were used to compare mortality, quarantine duration, and clinical deterioration according to the dementia status in the two groups. Results: Males and older individuals (age ≥60 years) constituted 41.5% and 32.9%, respectively, of the 5,299 patients. The prevalence of dementia was 4.2%, and 4.5% of the participants died during hospitalization. In multivariate analysis, dementia was significantly associated with increased mortality (odds ratio [OR]=2.80, 95% confidence interval [CI]=1.60–4.60), longer duration of quarantine (hazard ratio=1.69, 95% CI=1.16–2.45), and larger shift to a worse clinical severity (common OR=1.74, 95% CI=1.18–2.61). Conclusions After adjusting for important clinical predictors, dementia was associated with increased in-hospital mortality, duration of quarantine, and clinical deterioration during hospitalization in COVID-19 patients.

Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder characterized by heterogenous lesions infiltrated with CD1a+/CD207+ cells. Although LCH has a relatively good prognosis, the prognosis for patients with LCH refractory to standard chemotherapy is poor. Neurodegenerative LCH (ND-LCH) is a central nervous system complication of LCH that is characterized by progressive radiological and clinical abnormalities. Symptomatic ND-LCH is difficult to treat and therefore has a poor prognosis. A two-year-old boy presented with a scalp mass. Biopsy confirmed LCH. Whole-body imaging revealed LCH involvement in multiple bones of the skull, facial bones, and lungs. Prednisolone and vinblastine chemotherapy was initiated. One-year post-treatment, most of the lesions in the bones and lung nodules disappeared, and chemotherapy was discontinued. New neurodegenerative lesions appeared 4 months after chemotherapy was discontinued. Second-line chemotherapy using cytarabine, vincristine, and prednisolone was initiated. However, neurological manifestations of ND-LCH worsened post second-line treatment, and the treatment was switched to cytarabine and cladribine. Despite third-line chemotherapy, the lesions progressed, and neurological deficits worsened. After identifying BRAF V600E mutation in the tumor tissue using next-generation sequencing, cytotoxic chemotherapy was discontinued and vemurafenib treatment was initiated. One-year post-vemurafenib therapy, ND-LCH manifestations regressed, and the patient experienced neurological improvement.

Purpose: Additional surgery is recommended for patients after a non-curative endoscopic submucosal dissection (ESD) to prevent residual cancer (RC) or lymph node metastasis (LNM). We aimed to evaluate the clinicopathologic characteristics of patients who underwent an additional gastrectomy after a non-curative ESD procedure and identify the risk factors of RC and LNM. Methods: We retrospectively assessed the clinicopathological factors of 73 patients who underwent additional gastrectomy following a non-curative ESD between January 2009 and December 2019 at our center. Results: RC and LNM rates after additional gastrectomy were 9.6% and 8.2%, respectively. Invasion deeper than 500 μm (P = 0.045), positive horizontal resection margin (P < 0.001), and positive ESD margin (P = 0.001) were identified as statistically significant factors in univariate analysis for RC, but not in multivariate analysis. Lymphatic invasion was the only risk factor found to be significant in both univariate and multivariate analyses (P = 0.005 and P = 0.012). Conclusion Additional gastrectomy is necessary to prevent RC or LNM after non-curative ESD. Lymphatic invasion was also associated with LNM in patients who underwent an additional gastrectomy after a non-curative ESD, and in such cases, active treatment is required.

PURPOSE: Recently, a nomogram predicting overall survival after gastric resection was developed and externally validated in Korea and Japan. However, this gastric cancer nomogram is derived from large-volume centers, and the applicability of the nomogram in smaller centers must be proven. The purpose of this study is to externally validate the gastric cancer nomogram using a dataset from a medium-volume center in Korea. MATERIALS AND METHODS: We retrospectively analyzed 610 patients who underwent radical gastrectomy for gastric cancer from August 1, 2005 to December 31, 2011. Age, sex, number of metastatic lymph nodes (LNs), number of examined LNs, depth of invasion, and location of the tumor were investigated as variables for validation of the nomogram. Both discrimination and calibration of the nomogram were evaluated. RESULTS: The discrimination was evaluated using Harrell's C-index. The Harrell's C-index was 0.83 and the discrimination of the gastric cancer nomogram was appropriate. Regarding calibration, the 95% confidence interval of predicted survival appeared to be on the ideal reference line except in the poorest survival group. However, we observed a tendency for actual survival to be constantly higher than predicted survival in this cohort. CONCLUSIONS: Although the discrimination power was good, actual survival was slightly higher than that predicted by the nomogram. This phenomenon might be explained by elongated life span in the recent patient cohort due to advances in adjuvant chemotherapy and improved nutritional status. Future gastric cancer nomograms should consider elongated life span with the passage of time.
Calibration ; Chemotherapy, Adjuvant ; Cohort Studies ; Dataset* ; Discrimination (Psychology) ; Gastrectomy ; Humans ; Japan ; Korea ; Lymph Nodes ; Nomograms* ; Nutritional Status ; Retrospective Studies ; Stomach Neoplasms*

In Republic of Korea, a 7-valent pneumococcal conjugated vaccine (PCV7) was licensed for use in infants in 2003, and 13-valent PCV (PCV13) replaced it since 2010. We investigated trends in serotype distribution and antibiotic susceptibility of pneumococcal isolates from adult patients with invasive pneumococcal diseases (IPD). Invasive pneumococcal isolates from adult patients of ≥ 16 years of age were collected from 1997 to 2012. Serotypes of the isolates were determined by the Quellung reaction. Distribution of serotypes was analyzed according to the vaccine types. Antibiotic susceptibility was tested by using E-test strips. A total of 272 invasive pneumococcal isolates were included. The most common serotypes were serotype 19F (8.5%, 23/272), and serotype 3 (8.1%, 22/272), and 24.6% (67/272) of the isolates were of non-vaccine serotypes. Of the 272 isolates, 2.6% (7/272) were penicillin MICs of ≥ 4 µg/mL. The proportion of the PCV13 serotypes decreased from 63.3% (50/79) in 1997-2003 to 48.6% (17/35) in 2011-2012, whereas that of non-vaccine serotypes was 26.6% (21/79) and 25.7% (9/35), respectively, for the same periods. The proportion of the PCV13 serotypes showed a decreasing trend among adult patients with IPD over the study period.
Adolescent ; Adult ; Aged ; Anti-Infective Agents/*pharmacology/therapeutic use ; Ceftriaxone/pharmacology/therapeutic use ; Female ; Humans ; Male ; Microbial Sensitivity Tests ; Middle Aged ; Penicillins/pharmacology/therapeutic use ; Pneumococcal Infections/drug therapy/*microbiology/mortality ; Republic of Korea ; Serogroup ; Serotyping ; Streptococcus pneumoniae/*drug effects/isolation & purification ; Young Adult

PURPOSE: Several recent studies have reported on the clinical importance and prognostic significance of neutrophil-to-lymphocyte ratio (NLR) in gastric cancer. The objective of this study was to identify the subgroups of patients with gastric cancer for which the preoperative NLR was prognostically significant.METHODS: Data from 870 patients who were among those who had undergone surgery for gastric cancer between August 2005 and December 2013 were evaluated. Receiver operating characteristic curve analysis was used to determine the cut-off value for NLR. The patients were classified into high-NLR (NLR≥1.7) and low-NLR (NLR<1.7) groups, and survival analysis of subgroups of gastric cancer patients was performed.RESULTS: Univariate analysis identified age, gender, tumor location, tumor histology, tumor, node, metastasis (TNM) stage, and NLR as significant prognostic factors. Multivariate analysis identified age, TNM stage, and NLR as significant prognostic factors. In subgroup analysis, NLR was a significant prognostic factor except group of TNM stage I, II with age younger than 70 years.CONCLUSION: Except group of TNM stage I, II with age younger than 70 years, careful postoperative follow-up is warranted for those patients with elevated NLR.
Follow-Up Studies ; Humans ; Multivariate Analysis ; Neoplasm Metastasis ; ROC Curve ; Stomach Neoplasms