Purpose: NUT carcinoma (NC) is a solid tumor caused by the rearrangement of NUTM1 that usually develops in midline structures, such as the thorax. No standard treatment has been established despite high lethality. Thus, we investigated whether targeting the junction region of NUTM1 fusion breakpoints could serve as a potential treatment option for NC. Materials and Methods: We designed and evaluated a series of small interfering RNAs (siRNAs) targeting the junction region of BRD4-NUTM1 fusion (B4N), the most common form of NUTM1 fusion. Droplet digital polymerase chain reaction using the blood of patients was also tested to evaluate the treatment responses by the junction sequence of the B4N fusion transcripts. Results: As expected, the majority of NC fusion types were B4N (12 of 18, 67%). B4N fusion-specific siRNA treatment on NC cells showed specific inhibitory effects on the B4N fusion transcript and fusion protein without affecting the endogenous expression of the parent genes, resulting in decreased relative cell growth and attenuation of tumor size. In addition, the fusion transcript levels in platelet-rich-plasma samples of the NC patients with systemic metastasis showed a negative correlation with therapeutic effect, suggesting its potential as a measure of treatment responsiveness. Conclusion This study suggests that tumor-specific sequences could be used to treat patients with fusion genes as part of precision medicine for a rare but deadly disease.

Purpose: Mycoplasma pneumoniae pneumonia (MP) is a major cause of community-acquired pneumonia (CAP) in children and is associated with extrapulmonary manifestations (EPM). The incidence and risk factors for EPM in children are unknown. Methods: This was a retrospective study involving 65,243 pediatric patients with CAP between 2010 and 2015 at 23 nationwide hospitals in South Korea. Medical records were reviewed to collect information regarding the clinical characteristics, radiological results, and laboratory findings. Logistic regression with multivariate analysis was performed to evaluate the risk factors associated with EPM in MP. Results: The incidence of EPM was 23.9%, including elevation of liver enzymes (18.1%), mucocutaneous manifestations (4.4%), proteinuria (4.1%), cardiovascular and neurological manifestations (0.4%), hematologic manifestations (0.2%), and arthritis (0.2%). Statistical analysis showed that mucocutaneous manifestations significantly increased with elevated alanine aminotransferase (adjusted odds ratio [aOR], 3.623; 95% confidence interval [CI], 1.933-6.790) and atopic sensitization (aOR, 2.973; 95% CI, 1.615–5.475) and decreased with respiratory virus coinfection (aOR, 0.273; 95% CI, 0.084–0.887). Elevated liver enzymes were significantly associated with elevated lactate dehydrogenase (aOR, 3.055; 95% CI, 2.257–4.137), presence of pleural effusion (aOR, 2.635; 95% CI, 1.767–3.930), and proteinuria with respiratory virus coinfection (aOR, 2.245; 95% CI, 1.113–4.527). Conclusion Approximately 24% of pediatric patients with MP had various EPM. As the risk factors associated with each EPM were different, it is necessary to evaluate the various clinical aspects and findings of MP to predict and prepare for the occurrence of EPM.

In case of gingival recession or bone defect in maxillary anterior implant treatment, it is not easy to obtain satisfactory clinical results. In this case, loss of the labial alveolar plate was diagnosed in the maxillary right central incisor, so after tooth extraction, soft tissue was secured and implant placement with bone graft was planned. In addition, digital guide surgery was performed for the ideal implant position, and GBR (Guided Bone Regeneration) was accompanied with the xenogeneic bone and the autologous bone collected from the mandibular ramus since alveolar bone defects were extensive. After a sufficient period of osseointegration of the implant, a temporary prosthesis was fabricated through secondary stage surgery and impression taking, and through periodic external adjustment, the shape of soft tissue was improved. In the final prosthesis fabrication, a color tone of natural teeth was induced by an gold anodized customized abutment, and an aesthetic and functional zirconia prosthesis with reproducing the shape of the temporary prosthesis through intraoral scan was delivered.

Purpose: Patients who have undergone gastrectomy have unique symptoms that are not appropriately assessed using currently available tools. This study developed and validated a symptom-focused quality of life (QoL) questionnaire for patients who have received gastrectomy for gastric cancer. Materials and Methods Based on a literature review, patient interviews, and expert consultation by the KOrean QUality of life in Stomach cancer patients Study group (KOQUSS), the initial item pool was developed. Two large-scale developmental studies were then sequentially conducted for exploratory factor analyses for content validity and item reduction. The final item pool was validated in a separate cohort of patients and assessed for internal consistency, test-retest reliability, construct validity, and clinical validity. Results The initial questionnaire consisted of 46-items in 12 domains. Data from 465 patients at 11 institutions, followed by 499 patients at 13 institutions, were used to conduct item reduction and exploratory factor analyses. The final questionnaire (KOQUSS-40) comprised 40 items within 11 domains. Validation of KOQUSS-40 was conducted on 413 patients from 12 hospitals. KOQUSS-40 was found to have good model fit. The mean summary score of the KOQUSS-40 was correlated with the EORTC QLQ-C30 and STO22 (correlation coefficients, 0.821 and 0.778, respectively). The KOQUSS-40 score was also correlated with clinical factors, and had acceptable internal consistency (> 0.7). Test-retest reliability was greater than 0.8. Conclusion The KOQUSS-40 can be used to assess QoL of gastric cancer patients after gastrectomy and allows for a robust comparison of surgical techniques in clinical trials.

Purpose: Patients who have undergone gastrectomy have unique symptoms that are not appropriately assessed using currently available tools. This study developed and validated a symptom-focused quality of life (QoL) questionnaire for patients who have received gastrectomy for gastric cancer. Materials and Methods Based on a literature review, patient interviews, and expert consultation by the KOrean QUality of life in Stomach cancer patients Study group (KOQUSS), the initial item pool was developed. Two large-scale developmental studies were then sequentially conducted for exploratory factor analyses for content validity and item reduction. The final item pool was validated in a separate cohort of patients and assessed for internal consistency, test-retest reliability, construct validity, and clinical validity. Results The initial questionnaire consisted of 46-items in 12 domains. Data from 465 patients at 11 institutions, followed by 499 patients at 13 institutions, were used to conduct item reduction and exploratory factor analyses. The final questionnaire (KOQUSS-40) comprised 40 items within 11 domains. Validation of KOQUSS-40 was conducted on 413 patients from 12 hospitals. KOQUSS-40 was found to have good model fit. The mean summary score of the KOQUSS-40 was correlated with the EORTC QLQ-C30 and STO22 (correlation coefficients, 0.821 and 0.778, respectively). The KOQUSS-40 score was also correlated with clinical factors, and had acceptable internal consistency (> 0.7). Test-retest reliability was greater than 0.8. Conclusion The KOQUSS-40 can be used to assess QoL of gastric cancer patients after gastrectomy and allows for a robust comparison of surgical techniques in clinical trials.

Objective: The aim of this study was to investigate differentially expressed genes and their functions in the hippocampus and striatum after heroin administration in cynomolgus macaques of different ages. Methods: Cynomolgus monkeys were divided by age as follows: 1 year (A1, n = 2); 3 to 4 years (A2, n = 2); 6 to 8 years (A3, n = 2); and older than 11 years (A4, n = 2). After heroin was injected intramuscularly into the monkeys (0.6 mg/kg), we performed large-scale transcriptome profiling in the hippocampus (H) and striatum (S) using RNA sequencing technology. Some genes were validated with real-time quantitative PCR. Results: In the hippocampus, the gene expression of A1H was similar to that of A4H, while the gene expression of A2H was similar to that of A3H. Genes associated with the mitogen-activated protein kinase signaling pathway (STMN1, FGF14, and MAPT) and -aminobutyric acid-ergic synapses (GABBR2 and GAD1) were differentially expressed among control and heroin-treated animals. Differential gene expression between A1S and A4S was the least significant, while differential gene expression between A3S and A2S was the most significant. Genes associated with the neurotrophin signaling pathway (NTRK1 and NGFR), autophagy (ATG5), and dopaminergic synapses (AKT1) in the striatum were differentially expressed among control and heroin-treated animals. Conclusion These results suggest that even a single heroin exposure can cause differential gene expression in the hippocampus and striatum of nonhuman primates at different ages.

PURPOSE: Croup is known to have epidemics in seasonal and biennial trends, and to be strongly associated with epidemics of parainfluenza virus. However, seasonal and annual epidemics of croup have not been clearly reported in Korea. This study aimed to examine the seasonal/annual patterns and etiologies of childhood croup in Korea during a consecutive 6-year period. METHODS: Pediatric croup data were collected from 23 centers in Korea from 1 January 2010 to 31 December 2015. Electronic medical records, including multiplex reverse transcription polymerase chain reaction (RT-PCR) results, demographics and clinical information were cross-sectionally reviewed and analyzed. RESULTS: Overall, 2,598 childhood croup patients requiring hospitalization were identified during the study period. Among them, a total of 927 who underwent RT-PCR were included in the analysis. Males (61.5%) predominated, and most (63.0%) of them were younger than 2 years of age (median, 19 months; interquartile range, 11–31 months). Peak hospitalization occurred in 2010 and 2012 in even-numbered years, and parainfluenza virus (PIV, 39.7%) was the most common cause of childhood croup requiring hospitalization, followed by respiratory syncytial virus (14.9%), human rhinovirus (12.5%), Mycoplasma pneumonaie (10.6%), and human coronavirus (7.3%). CONCLUSION: It is concluded that croup hospitalization has a biennial pattern in even-numbered years. PIV may be the most common cause of childhood croup; however, croup epidemics could be attributed to other viruses.
Child ; Coronavirus ; Croup ; Demography ; Electronic Health Records ; Hospitalization ; Humans ; Korea ; Male ; Mycoplasma ; Paramyxoviridae Infections ; Polymerase Chain Reaction ; Respiratory Syncytial Viruses ; Retrospective Studies ; Reverse Transcription ; Rhinovirus ; Seasons

OBJECTIVE: Research on calcium use as an adjunctive rescue therapy for refractory septic shock is limited. In this study, we aimed to investigate the short-term hemodynamic response after calcium supplementation in patients with refractory septic shock. METHODS: This was a single-center, retrospective study of patients who presented to the Emergency Department from October 2014 through February 2018. Patients with refractory septic shock requiring norepinephrine-equivalent (NE) dose ≥ 0.5 µg/kg/min and receiving calcium supplementation, were included in the analysis. Patients were categorized into response group and no-response group, based on the changes in NE doses and mean arterial pressure after calcium administration. A multivariable logistic regression model was used to evaluate the association between hemodynamic response and 28-day survival. RESULTS: A total of 66 patients were included in the study. Of these, 34 patients (52%) were classified as the response group, and 32 patients (48%) encompassed the no-response group (17 patients who deteriorated and 15 patients who had no significant changes in the clinical status). The 28-day mortality was determined to be 44% in the response group and 63% in the no-response group (P=0.14). Multivariable analysis revealed that the response group had a significant association with lower 28-day mortality (adjusted odds ratio, 0.23; 95% confidence interval, 0.06–0.89; P=0.03). CONCLUSION Short-term hemodynamic changes after calcium administration were variable in patients with refractory septic shock. Hemodynamic improvement after calcium supplementation may be associated with better survival. Further studies are required to determine when calcium supplementation needs to be considered, and how it impacts patientcentered outcomes in refractory septic shock.

We report on a first case of bone marrow metastasis by dedifferentiated liposarcoma. A 39-year-old male diagnosed with retroperitoneal dedifferentiated liposarcoma underwent surgery and postoperative radiotherapy. In spite of radiotherapy, his whole-body positron emission tomography showed high uptake in multiple bone metastasis. With thrombocytopenia, bone scan suggested bone marrow involvement. After bone marrow biopsy, bone marrow metastasis by dedifferentiated liposarcoma was finally confirmed. He was administered with systemic chemotherapy with doxorubicin. But he died 3 months after chemotherapy due to disease progression. This case revealed that in a patient of unexplained cytopenia with dedifferentiated liposarcoma, bone marrow metastasis should be in consideration.
Adult ; Biopsy ; Bone Marrow* ; Disease Progression ; Doxorubicin ; Drug Therapy ; Humans ; Liposarcoma* ; Male ; Neoplasm Metastasis ; Positron-Emission Tomography ; Radiotherapy ; Thrombocytopenia

PURPOSE: Although influenza vaccine contains some residual egg protein (ovalbumin), recent studies have been reported that the influenza vaccine is even safe for patients with egg allergy. The object of this study was to assess the safety of influenza vaccination and estimate the risk factors of allergic reactions to influenza vaccination in children with egg allergy. METHODS: The medical records of 108 children were reviewed retrospectively, those were diagnosed as egg allergy at Department of Pediatrics in Severance Children's Hospital between January 2006 and December 2011. All of them were vaccinated with very low ovalbumin concentration (< or =0.12 microg/mL). Patients were vaccinated in graded doses by the international guideline. Subjects without allergic reactions to influenza vaccine among egg allergy patients were recruited as control subjects. RESULTS: Only 12 subjects of patients had adverse reactions to influenza vaccination. There were no significant relationships between adverse reactions to influenza vaccine and their own history of other allergy, history of breast feeding or graded vaccination. The egg allergy symptoms or egg-specific IgE levels were not associated with adverse reactions to influenza vaccination. CONCLUSION: These results show that patients with egg allergy may have adverse reactions with influenza vaccine, but severe adverse reactions are rare as general population. Therefore, the patients with egg allergy can be safe vaccinated with influenza vaccine, regardless of severity of allergic reaction after egg ingestion or methods of vaccination.
Breast Feeding ; Child* ; Eating ; Egg Hypersensitivity* ; Humans ; Hypersensitivity ; Immunoglobulin E ; Influenza Vaccines ; Influenza, Human* ; Medical Records ; Methods ; Ovalbumin ; Ovum* ; Pediatrics ; Retrospective Studies ; Risk Factors ; Vaccination*