Changes in the characteristics of pediatric emergency practice following the introduction of pediatric specialist care

Purpose@#We aimed to evaluate whether pediatric emergency practice has improved since the introduction of pediatric specialist care (PSC). @*Methods@#Retrospective observational study was conducted using the data retrieved from the emergency department (ED) of a tertiary university hospital in Cheongju, Korea. Patients younger than 19 years who visited the ED from January 2019 through December 2023 were enrolled in this study. Hospitalization (overall and intensive care unit [ICU]), in-hospital mortality, and return visit within 24 hours were compared between the periods before (January 2019-January 2021) and after (June 2021-December 2023) the introduction of PSC. Adjusted odds ratios with 95% confidence intervals were calculated for the outcomes using multivariable logistic regression. @*Results@#During the study period, a total of 36,162 patients visited the ED. The visits increased from 12,196 before to 22,387 after the introduction of PSC (increase by 83.6%). Annual numbers of the visits have increased since 2020 and reached 10,942 in 2023. After the introduction of PSC, decreases were noted in the hospitalization (adjusted odds ratio, 0.67; 95% confidence interval, 0.62-0.72) and return visit within 24 hours (0.73; 0.61-0.88). Hospitalization to the ICU increased (2.90; 2.29-3.69), while there was no significant difference in the in-hospital mortality (1.31; 0.77-2.25). @*Conclusion@#After the introduction of PSC, overall hospitalization and return visit decreased, while hospitalization to the ICU increased without a difference in the in-hospital mortality. Multidisciplinary efforts are needed to continue providing the pediatric specialist-centered emergency practice.

Comparing Montreal Cognitive Assessment Performance in Parkinson’s Disease Patients: Age- and Education-Adjusted Cutoffs vs. Machine Learning

Objective@#The Montreal Cognitive Assessment (MoCA) is recommended for general cognitive evaluation in Parkinson’s disease (PD) patients. However, age- and education-adjusted cutoffs specifically for PD have not been developed or systematically validated across PD cohorts with diverse education levels. @*Methods@#In this retrospective analysis, we utilized data from 1,293 Korean patients with PD whose cognitive diagnoses were determined through comprehensive neuropsychological assessments. Age- and education-adjusted cutoffs were formulated based on 1,202 patients with PD. To identify the optimal machine learning model, clinical parameters and MoCA domain scores from 416 patients with PD were used. Comparative analyses between machine learning methods and different cutoff criteria were conducted on an additional 91 consecutive patients with PD. @*Results@#The cutoffs for cognitive impairment decrease with increasing age within the same education level. Similarly, lower education levels within the same age group correspond to lower cutoffs. For individuals aged 60–80 years, cutoffs were set as follows: 25 or 24 years for those with more than 12 years of education, 23 or 22 years for 10–12 years, and 21 or 20 years for 7–9 years. Comparisons between age- and education-adjusted cutoffs and the machine learning method showed comparable accuracies. The cutoff method resulted in a higher sensitivity (0.8627), whereas machine learning yielded higher specificity (0.8250). @*Conclusion@#Both the age- and education-adjusted cutoff methods and machine learning methods demonstrated high effectiveness in detecting cognitive impairment in PD patients. This study highlights the necessity of tailored cutoffs and suggests the potential of machine learning to improve cognitive assessment in PD patients.

Comparison of clinical features of anaphylaxis patients according to epinephrine administration

Objective@#Epinephrine is a first-line drug for anaphylaxis, but a poor prognosis can occur if not administered properly. This study compared the clinical features of patients with anaphylaxis in the emergency department (ED) according to epinephrine administration. @*Methods@#This was a retrospective cross-sectional study using ED-based data retrieved from a tertiary university hospital. Patients diagnosed with anaphylaxis between 2018 and 2020 were enrolled in this study. The patients with anaphylaxis were classified according to epinephrine administration, and the clinical features were compared. The adjusted odds ratios (AORs) with a 95% confidence interval (CI) were calculated for the main factors associated with epinephrine use. @*Results@#Among 205 eligible patients with anaphylaxis, 157 (76.6%) were treated with epinephrine. The main contributing factors influencing epinephrine use were patients with cardiovascular symptoms (AOR=2.97; 95% CI, 1.26-7.01) and patients transferred from other hospitals (AOR=0.37; 95% CI, 0.16-0.85). @*Conclusion@#The major factors influencing epinephrine use in the ED when patients with anaphylaxis presented with cardiovascular symptoms were identified. It is essential to prevent potentially fatal consequences in patients with anaphylaxis through appropriate epinephrine administration.

Real-time observation of neutrophil extracellular trap formation in the inflamed mouse brain via two-photon intravital imaging / 한국실험동물학회지

Intravital imaging via two-photon microscopy (TPM) is a useful tool for observing and delineating biological events at the cellular and molecular levels in live animals in a time-lapse manner. This imaging method provides spatiotemporal information with minimal phototoxicity while penetrating a considerable depth of intact organs in live animals.Although various organs can be visualized using intravital imaging, in the field of neuroscience, the brain is the main organ whose cell-to-cell interactions are imaged using this technique. Intravital imaging of brain disease in mouse models acts as an abundant source of novel findings for studying cerebral etiology. Neutrophil infiltration is a wellknown hallmark of inflammation; in particular, the crucial impact of neutrophils on the inflamed brain has frequently been reported in literature. Neutrophil extracellular traps (NETs) have drawn attention as an intriguing feature over the last couple of decades, opening a new era of research on their underlying mechanisms and biological effects.However, the actual role of NETs in the body is still controversial and is in parallel with a poor understanding of NETs in vivo. Although several experimental methods have been used to determine NET generation in vitro, some research groups have applied intravital imaging to detect NET formation in the inflamed organs of live mice. In this review, we summarize the advantages of intravital imaging via TPM that can also be used to characterize NET formation, especially in inflamed brains triggered by systemic inflammation. To study the function and migratory pattern of neutrophils, which is critical in triggering the innate immune response in the brain, intravital imaging via TPM can provide new perspectives to understand inflammation and the resolution process.

Burnout and Resilience among Emergency Physicians at Korean University Hospitals during the COVID-19 Pandemic: A Cross-Sectional Analysis

Purpose@#This study aimed to investigate burnout and resilience among emergency physicians (EPs) at university teaching hospitals during the coronavirus disease (COVID-19) pandemic. @*Materials and Methods@#In April to May 2021, a survey was administered to 331 and 309 emergency medicine specialists and residents, respectively, from 31 university teaching hospitals in Korea. Data on the respondents’ age, sex, designation, working area, experience with treating COVID-19 patients, and personal experience with COVID-19 were collected. Based on the participants’ characteristics, quality of life (compassion satisfaction, burnout, and secondary traumatic stress), resilience, emotional content, and self-image were analyzed. @*Results@#A total of 247 responses were analyzed. Compared to specialists, compassion satisfaction and resilience in residents were not good, burnout was severe, and emotional content and self-image were less positive. Experiences with treating COVID-19 patients did not cause any difference in quality of life, resilience, emotional content, and self-image among participant subgroups. Personal COVID-19 experiences were associated with poor compassion satisfaction, resilience, less positive emotional content and self-image, and severe burnout. Compassion satisfaction, secondary traumatic stress, and resilience can definitively affect burnout. @*Conclusion@#The quality of life and resilience of EPs in university teaching hospitals in Korea during the COVID-19 pandemic have been low. Supportive measures to improve resilience can prevent burnout among emergency staff, particularly residents and EPs, with personal experiences related to COVID-19.

Multidetector computed tomography-based evaluation of gastric volumes in patients with out-of-hospital cardiac arrest

Objective@#Resuscitation-related gastric inflation is associated with inadequate ventilation and the risk of gastric regurgitation in out-of-hospital cardiac arrest (OHCA) patients. This study aims to estimate resuscitation-related gastric inflation values by using multi-detector computed tomography (MDCT) scanning. @*Methods@#MDCT imaging data were obtained from OHCA patients undergoing resuscitation from January 2014 to December 2020. Thirty age- and sex-matched healthy controls that underwent an MDCT scan were included. Gastric air volume (GAV), total gastric volume (TGV), and GAV/gastric content volume (GCV) ratio values were estimated. @*Results@#In healthy controls (n=30), GAV and TGV values were in the range 5.0-35.0 mL, and 202.0-1,002.0 mL, respectively. The mean GAV and TGV values of OHCA patients (n=97) were 251.0 mL (range, 55.5-896.0) and 878.0 mL (range, 430.5-1,696.0), respectively. Significant between-group differences were determined in the mean GCV, GAV, and GAV/GCV ratio values. In OHCA patients, the cut-off value for abnormal GAV was defined as 56.5 mL (mean value plus two times standard deviation). Patients with abnormal GAV findings on MDCT scans had a longer duration from arrest to the return of spontaneous circulation, low body mass index, and increased rates of lactic acidosis. @*Conclusion@#Our results indicate an association between gastric air accumulation after resuscitation with longer recovery from arrest to return of spontaneous circulation, low body mass index, and increased lactic acidosis.

Altered Intestinal Permeability and Drug Repositioning in a Post-operative Ileus Guinea Pig Model

Background/Aims@#The aim of this study is to identify the alteration in intestinal permeability with regard to the development of post-operative ileus (POI).Moreover, we investigated drug repositioning in the treatment of POI. @*Methods@#An experimental POI model was developed using guinea pigs. To measure intestinal permeability, harvested intestinal membranes of the ileum and proximal colon was used in an Ussing chamber. To identify the mechanisms associated with altered permeability, we measured leukocyte count and expression of calprotectin, claudin-1, claudin-2, and mast cell tryptase. We compared control, POI, and drug groups (mosapride [0.3 mg/kg and 1 mg/kg, orally], glutamine [500 mg/kg, orally], or ketotifen [1 mg/kg, orally] with regard to these parameters. @*Results@#Increased permeability after surgery significantly decreased after administration of mosapride, glutamine, or ketotifen. Leukocyte counts increased in the POI group and decreased significantly after administration of mosapride (0.3 mg/kg) in the ileum, and mosapride (0.3 mg/kg and 1 mg/kg), glutamine, or ketotifen in the proximal colon. Increased expression of calprotectin after surgery decreased after administration of mosapride (0.3 mg/kg), glutamine, or ketotifen in the ileum and proximal colon, and mosapride (1 mg/kg) in the ileum. The expression of claudin-1 decreased significantly and that of claudin-2 increased after operation. After administration of glutamine, the expression of both proteins was restored. Finally, mast cell tryptase levels increased in the POI group and decreased significantly after administration of ketotifen. @*Conclusions@#The alteration in intestinal permeability is one of the factors involved in the pathogenesis of POI. We repositioned 3 drugs (mosapride, glutamine, and ketotifen) as novel therapeutic agents for POI.

Altered Intestinal Permeability and Drug Repositioning in a Post-operative Ileus Guinea Pig Model

Background/Aims@#The aim of this study is to identify the alteration in intestinal permeability with regard to the development of post-operative ileus (POI).Moreover, we investigated drug repositioning in the treatment of POI. @*Methods@#An experimental POI model was developed using guinea pigs. To measure intestinal permeability, harvested intestinal membranes of the ileum and proximal colon was used in an Ussing chamber. To identify the mechanisms associated with altered permeability, we measured leukocyte count and expression of calprotectin, claudin-1, claudin-2, and mast cell tryptase. We compared control, POI, and drug groups (mosapride [0.3 mg/kg and 1 mg/kg, orally], glutamine [500 mg/kg, orally], or ketotifen [1 mg/kg, orally] with regard to these parameters. @*Results@#Increased permeability after surgery significantly decreased after administration of mosapride, glutamine, or ketotifen. Leukocyte counts increased in the POI group and decreased significantly after administration of mosapride (0.3 mg/kg) in the ileum, and mosapride (0.3 mg/kg and 1 mg/kg), glutamine, or ketotifen in the proximal colon. Increased expression of calprotectin after surgery decreased after administration of mosapride (0.3 mg/kg), glutamine, or ketotifen in the ileum and proximal colon, and mosapride (1 mg/kg) in the ileum. The expression of claudin-1 decreased significantly and that of claudin-2 increased after operation. After administration of glutamine, the expression of both proteins was restored. Finally, mast cell tryptase levels increased in the POI group and decreased significantly after administration of ketotifen. @*Conclusions@#The alteration in intestinal permeability is one of the factors involved in the pathogenesis of POI. We repositioned 3 drugs (mosapride, glutamine, and ketotifen) as novel therapeutic agents for POI.

Understanding the Host Innate Immune Responses against SARS-CoV-2Infection and COVID-19 Pathogenesis

The emergence of a new severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic has become a significant health concern worldwide. Undoubtedly, a better understanding of the innate and adaptive immune responses against SARS-CoV-2 and its relationship with the coronavirus disease 2019 (COVID-19) pathogenesis will be the sole basis for developing and applying therapeutics. This review will summarize the published results that relate to innate immune responses against infections with human coronaviruses including SARS-CoV-1 and SARS-CoV-2 in both humans and animal models. The topics encompass the innate immune sensing of the virus to the dysregulation of various innate immune cells during infection and disease progression.

Peri-operative Inflammatory Marker as a Predictive Factor for Prolonged Post-operative Ileus After Gastrectomy for Gastric Cancer

Background/Aims@#Although prolonged post-operative ileus (PPOI) is an important factor for the prolonged length of post-operative hospital stay, there is still a lack of effective predictive and therapeutic methods for PPOI. Previous studies reported that increased inflammatory markers, such as C-reactive protein (CRP) level and neutrophil to lymphocyte ratio (NLR), are associated with malignancies. The aim of our study is to elucidate the association between peri-operative inflammatory markers and PPOI after gastrectomy for gastric cancer. @*Methods@#We enrolled patients who received gastrectomy for gastric cancer from June 2013 to January 2016 at a single tertiary referral center in Seoul, Korea. We evaluated peri-operative inflammatory markers, including CRP level, NLR, and platelet to lymphocyte ratio (PLR) of enrolled patients. We compared these data between control group and PPOI group. @*Results@#A total of 390 subjects were enrolled in this study, and 132 patients (33.8%) showed PPOI. In univariate analysis, preoperative CRP level and NLR, post-operative day (POD) 1 CRP level, NLR, and PLR, and POD3 CRP level, NLR, and PLR were significantly associated with PPOI. In multivariate analysis, preoperative NLR (P = 0.014), POD1 NLR (P = 0.019), POD3 CRP (P = 0.004), and POD3 NLR (P = 0.008) were independent risk factors for PPOI. @*Conclusions@#Peri-operative inflammatory markers, such as CRP level and NLR, are useful predictive factors for PPOI who received gastrectomy for gastric cancer. Moreover, prophylactic antibiotics and anti-inflammatory drugs can be preventive and therapeutic agents for PPOI.

Peri-operative Inflammatory Marker as a Predictive Factor for Prolonged Post-operative Ileus After Gastrectomy for Gastric Cancer

Background/Aims@#Although prolonged post-operative ileus (PPOI) is an important factor for the prolonged length of post-operative hospital stay, there is still a lack of effective predictive and therapeutic methods for PPOI. Previous studies reported that increased inflammatory markers, such as C-reactive protein (CRP) level and neutrophil to lymphocyte ratio (NLR), are associated with malignancies. The aim of our study is to elucidate the association between peri-operative inflammatory markers and PPOI after gastrectomy for gastric cancer. @*Methods@#We enrolled patients who received gastrectomy for gastric cancer from June 2013 to January 2016 at a single tertiary referral center in Seoul, Korea. We evaluated peri-operative inflammatory markers, including CRP level, NLR, and platelet to lymphocyte ratio (PLR) of enrolled patients. We compared these data between control group and PPOI group. @*Results@#A total of 390 subjects were enrolled in this study, and 132 patients (33.8%) showed PPOI. In univariate analysis, preoperative CRP level and NLR, post-operative day (POD) 1 CRP level, NLR, and PLR, and POD3 CRP level, NLR, and PLR were significantly associated with PPOI. In multivariate analysis, preoperative NLR (P = 0.014), POD1 NLR (P = 0.019), POD3 CRP (P = 0.004), and POD3 NLR (P = 0.008) were independent risk factors for PPOI. @*Conclusions@#Peri-operative inflammatory markers, such as CRP level and NLR, are useful predictive factors for PPOI who received gastrectomy for gastric cancer. Moreover, prophylactic antibiotics and anti-inflammatory drugs can be preventive and therapeutic agents for PPOI.

Effects of Changes in Inspiratory Time on Inspiratory Flowrate and Airway Pressure during Cardiopulmonary Resuscitation: A Manikin-Based Study / 고신대학교의과대학학술지

Objectives@#Given that cardiopulmonary resuscitation (CPR) is an aerosol-generating procedure, it is necessary to use a mechanical ventilator and reduce the number of providers involved in resuscitation for in-hospital cardiac arrest in coronavirus disease (COVID-19) patients or suspected COVID-19 patients. However, no study assessed the effect of changes in inspiratory time on flowrate and airway pressure during CPR. We herein aimed to determine changes in these parameters during CPR and identify appropriate ventilator management for adults during CPR. @*Methods@#We measured changes in tidal volume (Vt), peak inspiratory flow rate (PIFR), peak airway pressure (Ppeak), mean airway pressure (Pmean) according to changes in inspiratory time (0.75 s, 1.0 s and 1.5 s) with or without CPR. Vt of 500 mL was supplied (flowrate: 10 times/min) using a mechanical ventilator. Chest compressions were maintained at constant compression depth (53 ± 2 mm) and speed (102 ± 2/min) using a mechanical chest compression device. @*Results@#Median levels of respiratory physiological parameters during CPR were significantly different according to the inspiratory time (0.75 s vs. 1.5 s): PIFR (80.8 [73.3 – 87.325] vs. 70.5 [67 – 72.4] L/min, P < 0.001), Ppeak (54 [48 – 59] vs. 47 [45 – 49] cmH2O, P < 0.001), and Pmean (3.9 [3.6 – 4.1] vs. 5.7 [5.6 – 5.8] cmH2O, P < 0.001). @*Conclusions@#Changes in PIFR, Ppeak, and Pmean were associated with inspiratory time. PIFR and Ppeak values tended to decrease with increase in inspiratory time, while Pmean showed a contrasting trend. Increased inspiratory time in low-compliance cardiac arrest patients will help in reducing lung injury during adult CPR.

Extract from Zanthoxylum piperitum Induces Apoptosis of AGS Gastric Cancer Cells Through Akt/MDM2/p53 Signaling Pathway / 中国结合医学杂志

OBJECTIVE@#To determine the effect of Zanthoxylum piperitum extracet (ZPE) on apoptosis and analyze anticancer substances in ZPE, changes in proteins related to apoptosis, and pathological changes in tumors in mouse.@*METHODS@#Fifteen 4-week-old female BALB/c nu/nu mice were divided into 3 groups depending on ZPE dose, with 5 in each group. AGS gastric carcinoma cells (1 × 10@*RESULTS@#High performance liquid chromatography (HPLC) analysis showed that ZPE contained organic sulfur compounds such as alliin and S-allylcysteine. MTT assay results revealed that ZPE (10-85 µ g/mL) could effectively inhibit the growth of AGS gastric cancer cells at higher concentrations (P<0.05, P<0.01). The annexin V & dead cell staining assay and cell cycle arrest assay confirmed a dose-dependent increase in the apoptosis rate and G@*CONCLUSION@#ZPE decreases AGS cell proliferation and induces apoptosis by inhibiting Akt and MDM2 expression.

Annual Report of the Korean Association of External Quality Assessment Service on Routine Hematology (2019)

Two trials involving proficiency tests for complete blood counts (CBCs) and blood cell morphology were conducted as part of the 2019 Routine Hematology Program of the Korean Association of External Quality Assessment Service. Three different control samples were subjected to CBC testing, and two blood cell morphology images were posted on the laboratory’s website during each trial. The mean response rates of the 1,791 participating laboratories were 97.2% and 34.9% for CBC and blood cell morphology, respectively. The distribution of equipment for CBC testing was comparable to that in 2018. The coefficient of variation (CV) ranges were 2.9%–4.1%, 1.6%–2.4%, 1.4%–2.8%, 4.7%–5.3%, and 4.8%–7.3% for white blood cell counts, red blood cell counts, hemoglobin, hematocrit, and platelet counts, respectively. The concordance rate ranged from 83.5% to 99.7% in the blood cell morphology tests. In 2019, the number of participating laboratories was higher than that in 2018, and the CVs of CBC results were similar to those in 2018.

Effect of the indoor environment on atopic dermatitis in children

Atopic dermatitis (AD) is a chronically relapsing inflammatory disorder of the skin. It is characterized by severe pruritus, sleep disturbance, and poor quality of life. In addition, AD frequently occurs in childhood and is considered the first manifestation of atopic march, which progresses into respiratory allergic diseases, such as asthma and allergic rhinitis, as the patient grow older. In this regard, the early diagnosis and active treatment of AD are required to lower the severity of disease and to prevent the development of atopic march. The proper management of AD includes avoidance of aggravating factors, barrier repair by skin care, and control of inflammation and infection with appropriate medications. Among them, identification and elimination of environmental risk factors responsible for worsening skin symptoms are essential. They include temperature, humidity, food and inhalant allergens, microorganisms, air pollutants, ultraviolet radiation, water hardness, and so forth. In particular, there is growing concern about indoor environment because time spent indoors and exposure to various kinds of pollutants is increasing with modernization and industrialization. In this review, we summarize the hazardous effect of temperature, humidity, cigarette smoke, volatile organic compounds, formaldehyde, and phthalates on the development or aggravation of AD. Current epidemiological studies and animal experiments suggest possible contributions of various indoor environmental factors to the development and aggravation of AD. However, further studies are needed to demonstrate a causal relationship between indoor environmental factors and AD.

Similarity of the cut score in test sets with different item amounts using the modified Angoff, modified Ebel, and Hofstee standard-setting methods for the Korean Medical Licensing Examination / 보건의료교육평가

Purpose@#The Korea Medical Licensing Exam (KMLE) typically contains a large number of items. The purpose of this study was to investigate whether there is a difference in the cut score between evaluating all items of the exam and evaluating only some items when conducting standard-setting. @*Methods@#We divided the item sets that appeared on 3 recent KMLEs for the past 3 years into 4 subsets of each year of 25% each based on their item content categories, discrimination index, and difficulty index. The entire panel of 15 members assessed all the items (360 items, 100%) of the year 2017. In split-half set 1, each item set contained 184 (51%) items of year 2018 and each set from split-half set 2 contained 182 (51%) items of the year 2019 using the same method. We used the modified Angoff, modified Ebel, and Hofstee methods in the standard-setting process. @*Results@#Less than a 1% cut score difference was observed when the same method was used to stratify item subsets containing 25%, 51%, or 100% of the entire set. When rating fewer items, higher rater reliability was observed. @*Conclusion@#When the entire item set was divided into equivalent subsets, assessing the exam using a portion of the item set (90 out of 360 items) yielded similar cut scores to those derived using the entire item set. There was a higher correlation between panelists’ individual assessments and the overall assessments.

Similarity of the cut score in test sets with different item amounts using the modified Angoff, modified Ebel, and Hofstee standard-setting methods for the Korean Medical Licensing Examination / 보건의료교육평가

Purpose@#The Korea Medical Licensing Exam (KMLE) typically contains a large number of items. The purpose of this study was to investigate whether there is a difference in the cut score between evaluating all items of the exam and evaluating only some items when conducting standard-setting. @*Methods@#We divided the item sets that appeared on 3 recent KMLEs for the past 3 years into 4 subsets of each year of 25% each based on their item content categories, discrimination index, and difficulty index. The entire panel of 15 members assessed all the items (360 items, 100%) of the year 2017. In split-half set 1, each item set contained 184 (51%) items of year 2018 and each set from split-half set 2 contained 182 (51%) items of the year 2019 using the same method. We used the modified Angoff, modified Ebel, and Hofstee methods in the standard-setting process. @*Results@#Less than a 1% cut score difference was observed when the same method was used to stratify item subsets containing 25%, 51%, or 100% of the entire set. When rating fewer items, higher rater reliability was observed. @*Conclusion@#When the entire item set was divided into equivalent subsets, assessing the exam using a portion of the item set (90 out of 360 items) yielded similar cut scores to those derived using the entire item set. There was a higher correlation between panelists’ individual assessments and the overall assessments.

Pseudohypoaldosteronism Type 1 with a NovelMutation in the NR3C2 Gene: A Case Report

Pseudohypoaldosteronism type 1 (PHA1) is a rare salt-wasting disorder caused byresistance to mineralocorticoid action. PHA1 is of two types with different levelsof disease severity and phenotype as follows: systemic type with an autosomalrecessiveinheritance (caused by mutations of the epithelial sodium channel)and renal type with an autosomal dominant inheritance (caused by mutations inthe mineralocorticoid receptor). The clinical manifestations of PHA1 vary widely;however,PHA1 commonly involves hyponatremia, hyperkalemia, metabolicacidosis and elevated levels of renin and aldosterone. The earliest signs of bothtype of PAH1 also comprise insufficiency weight gain due to chronic dehydrationand failure to thrive during infancy. Here, we report a case of renal PAH1 in a28-day-old male infant harboring a novel heterozygous mutation in NR3C2 gene(c.1341_1345dupAAACC in exon 2), showing only failure to thrive without thecharacteristic of dehydration.

Programmed death-ligand 1 expression and its correlation with clinicopathological parameters in gallbladder cancer

Background@#Immunomodulatory therapies targeting the interaction between programmed cell death protein 1 and programmed death-ligand 1 (PD-L1) have become increasingly important in anticancer treatment. Previous research on the subject of this immune response has established an association with tumor aggressiveness and a poor prognosis in certain cancers. Currently, scant information is available on the relationship between PD-L1 expression and gallbladder cancer (GBC). @*Methods@#We investigated the expression of PD-L1 in 101 primary GBC cases to determine the potential association with prognostic impact. PD-L1 expression was immunohistochemically assessed using a single PD-L1 antibody (clone SP263). Correlations with clinicopathological parameters, overall survival (OS), or progression- free survival (PFS) were analyzed. @*Results@#PD-L1 expression in tumor cells at cutoff levels of 1%, 10%, and 50% was present in 18.8%, 13.8%, and 7.9% of cases. Our study showed that positive PD-L1 expression at any cutoff was significantly correlated with poorly differentiated histologic grade and the presence of lymphovascular invasion (p < .05). PD-L1 expression at cutoff levels of 10% and 50% was significantly positive in patients with perineural invasion, higher T categories, and higher pathologic stages (p < .05). Additionally, there was a significant association noted between PD-L1 expression at a cutoff level of 50% and worse OS or PFS (p = .049 for OS, p = .028 for PFS). Other poor prognostic factors included histologic grade, T category, N category, pathologic stage, lymphovascular invasion, perineural invasion, growth pattern, and margin of resection (p < .05). @*Conclusions@#The expression of PD-L1 in GBC varies according to cutoff level but is valuably associated with poor prognostic parameters and survival. Our study indicates that the overexpression of PD-L1 in GBC had a negative prognostic impact.

Primary hepatic extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue

Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), is one of the specific type of low-grade B-cell lymphoma not infrequently found worldwide. It typically involves mucosal sites such as stomach and conjunctiva; however, primary hepatic MALT lymphoma has been extremely rarely reported. We describe a case of hepatic MALT lymphoma in a 70-year-old male patient who underwent left hepatectomy due to the incidentally detected liver masses at a medical checkup. The resected specimen revealed multinodular masses consisting of small-to-intermediate-sized lymphoid cells with serpentine pattern and focal lymphoepithelial lesions. The tumor cells were diffusely positive for CD20 and Bcl-2 but negative for CD3, CD10, CD5, CD23, CD43, and cyclinD1. The Ki-67 labeling index was 10% and immunoglobulin heavy chain gene rearrangement study confirmed monoclonal proliferation. In this paper, we discuss several unique clinicopathologic characteristics which will be helpful to the differential diagnosis of hepatic MALT lymphoma.