Background/Aims: The prospective Crohn’s Disease Clinical Network and Cohort Study is a nationwide multicenter cohort study of patients with Crohn’s disease (CD) in Korea, aiming to prospectively investigate the clinical features and long-term prognosis associated with CD. Methods: Patients diagnosed with CD between January 2009 and September 2019 were prospectively enrolled. They were divided into two cohorts according to the year of diagnosis: cohort 1 (diagnosed between 2009 and 2011) versus cohort 2 (between 2012 and 2019). Results: A total of 1,175 patients were included, and the median follow-up duration was 68 months (interquartile range, 39.0 to 91.0 months). The treatment-free durations for thiopurines (p<0.001) and anti-tumor necrosis factor agents (p=0.018) of cohort 2 were shorter than those of cohort 1. Among 887 patients with B1 behavior at diagnosis, 149 patients (16.8%) progressed to either B2 or B3 behavior during follow-up. Early use of thiopurine was associated with a reduced risk of behavioral progression (adjusted hazard ratio [aHR], 0.69; 95% confidence interval [CI], 0.50 to 0.90), and family history of inflammatory bowel disease was associated with an increased risk of behavioral progression (aHR, 2.29; 95% CI, 1.16 to 4.50). One hundred forty-one patients (12.0%) underwent intestinal resection, and the intestinal resection-free survival time was significantly longer in cohort 2 than in cohort 1 (p=0.003). The early use of thiopurines (aHR, 0.35;95% CI, 0.23 to 0.51) was independently associated with a reduced risk of intestinal resection. Conclusions The prognosis of CD in Korea appears to have improved over time, as evidenced by the decreasing intestinal resection rate. Early use of thiopurines was associated with an improved prognosis represented by a reduced risk of intestinal resection.

Chronic spontaneous urticaria (CSU) is defined as the occurrence of spontaneous wheals, angioedema, or both for >6 weeks in the absence of specific causes. It is a common condition associated with substantial disease burden both for affected individuals and societies in many countries, including Korea. CSU frequently persists for several years and requires high-intensity treatment; therefore, patients experience deteriorations in quality of life and medication-associated complications. During the last decade, there have been major advances in the pharmacological treatment of CSU and there is an outstanding need for evidence-based guidelines that reflect clinical practice in Korea. The guidelines reported here represent a joint initiative of the Korean Academy of Asthma, Allergy and Clinical Immunology and the Korean Dermatological Association, and aim to provide evidence-based guidance for the management of CSU in Korean adults and children. In Part 1, disease definition, guideline scope and development methodology as well as evidence-based recommendations on the use of antihistamines and corticosteroids are summarized.

Chronic spontaneous urticaria (CSU) is defined as the occurrence of spontaneous wheals, angioedema, or both for >6 weeks in the absence of specific causes. It is a common condition associated with substantial disease burden both for affected individuals and societies in many countries, including Korea. CSU frequently persists for several years and requires high-intensity treatment; therefore, patients experience deteriorations in quality of life and medication-associated complications. During the last decade, there have been major advances in the pharmacological treatment of CSU and there is an outstanding need for evidence-based guidelines that reflect clinical practice in Korea. The guidelines reported here represent a joint initiative of the Korean Academy of Asthma, Allergy and Clinical Immunology and the Korean Dermatological Association, and aim to provide evidence-based guidance for the management of CSU in Korean adults and children. In Part 1, disease definition, guideline scope and development methodology as well as evidence-based recommendations on the use of antihistamines and corticosteroids are summarized.

BACKGROUND/OBJECTIVES: Dietary Reference Intakes (DRI) for energy are derived from total energy expenditure (TEE) measured using the doubly labelled water (DLW) method. The objective of this study was to assess the validity of DRI for predicting the energy requirements of elementary school-age children. SUBJECTS/METHODS: The present study involved 25 elementary school-age children aged between 9 and 11 years. TEE was assessed by the DLW method, and the results were compared with the TEE predicted by the DRI equations in order to evaluate accuracy. RESULTS: The subjects' TEE measured by the DLW method was 1,925.2 ± 380.9 kcal/day in boys and 1,930.0 ± 279.4 kcal/day in girls, whereas resting energy expenditure was 1,220.2 ± 176.9 kcal/day in boys and 1,245.9 ± 171.3 kcal/day for girls. The physical activity level was 1.58 ± 0.20 in boys and 1.55 ± 0.13 in girls. The mean bias between the predicted and measured TEE was 12.6% in boys and −1.6% in girls, and the percentage of accurate predictions was 28.6% and 63.6%, respectively. In boys, the equation resulted in underprediction of TEE among the subjects having low TEE values, whereas there was overprediction among subjects having high TEE values as shown by the Bland-Altman plot. On the contrary, this proportional bias was not observed in girls. CONCLUSIONS: The findings of this study suggest that the DRI equation for energy could result in the overestimation of energy requirements in elementary school-age boys. In the case of girls, the equations could be accurate at the group level. However, the DRI appears to be invalid for individual girls, as more than one third of girls had their TEE inaccurately predicted. We recommend more studies for confirmation of these results.
Bias (Epidemiology) ; Child* ; Energy Metabolism ; Female ; Humans ; Methods ; Motor Activity ; Nutritional Requirements ; Recommended Dietary Allowances* ; Water

PURPOSE: This study compared the functional fitness and brake response of elderly and young drivers to confirm the correlation between the functional fitness and brake response. METHODS: This study was a cross sectional observational design. Older drivers (>65age, n=21) and young adult drivers (20–40aged, n=20) were enrolled as subjects. The functional fitness of the subjects was measured using a senior fitness test consisting of a back scratch (BS), chair sit and reach (CSR), arm curl (AC), chair stand up (CSU), foot up and go (8-FUG), and 2-minute step (2-MS). The brake response used the virtual driving simulator to measure the brake reaction time (BRT) and braking distance (BD) according to the pedestrian protection and traffic signal compliance. RESULTS: The older drivers had a lower BS (p < 0.000), CSU (p=0.040), and 8-FUG (p=0.011) than the young adult drivers. BS and 8-FUG showed a significant positive correlation with the BRT and BD of pedestrian protection and traffic signal compliance. CSU showed a significant negative correlation with the BRT of pedestrian protection and traffic signal compliance. CONCLUSION: These findings suggest that the flexibility of the upper extremity, lower extremity strength, and agility are strongly correlated with the driving performance of elderly drivers.
Aged* ; Arm ; Compliance ; Foot ; Humans ; Lower Extremity ; Pliability ; Reaction Time ; Upper Extremity ; Young Adult

No abstract available.

BACKGROUND: Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is a standard procedure to evaluate suspicious lymph node involvement of lung cancer because computed tomography (CT) and 18F-fluorodeoxyglucose positron emission tomography-CT (PET-CT) have limitations in their sensitivity and specificity. There are a number of benign causes of false positive lymph node such as anthracosis or anthracofibrosis, pneumoconiosis, old or active tuberculosis, interstitial lung disease, and other infectious conditions including pneumonia. The purpose of this study was to evaluate possible causes of false positive lymph node detected in chest CT or PET-CT. METHODS: Two hundred forty-seven patients who were initially diagnosed with lung cancer between May 2009 and December 2012, and underwent EBUS-TBNA to confirm suspicious lymph node involvement by chest CT or PET-CT were analyzed for the study. RESULTS: Of 247 cases, EBUS-TBNA confirmed malignancy in at least one lymph node in 189. The remaining 58 patients whose EBUS-TBNA results were negative were analyzed. Age ≥65, squamous cell carcinoma as the histologic type, and pneumoconiosis were related with false-positive lymph node involvement on imaging studies such as chest CT and PET-CT. CONCLUSION: These findings suggest that lung cancer staging should be done more carefully when a patient has clinically benign lymph node characteristics including older age, squamous cell carcinoma, and benign lung conditions.
Anthracosis ; Carcinoma, Squamous Cell ; Electrons ; Humans ; Lung Diseases, Interstitial ; Lung Neoplasms* ; Lung* ; Lymph Nodes* ; Needles* ; Pneumoconiosis ; Pneumonia ; Sensitivity and Specificity ; Thorax* ; Tomography, X-Ray Computed* ; Tuberculosis

Floating-Harbor syndrome is a rare autosomal dominant genetic disorder associated with SRCAP mutation. To date, approximately 50 cases of Floating-Harbor syndrome have been reported, but none have been reported in Korea yet. Floating-Harbor syndrome is characterized by delayed bony maturation, unique facial features, and language impairment. Here, we present a 6-year-old boy with a triangular face, deep-set protruding eyes, low-set ears, wide nose with narrow nasal bridge, short philtrum, long thin lips, clinodactyly, and developmental delay that was transferred to our pediatric clinic for genetic evaluation. He showed progressive delay in the area of language and cognition-adaption as he grew. He had previously undergone chromosomal analysis at another hospital due to his language delay, but his karyotype was normal. We performed targeted exome sequencing, considering several syndromes with similar phenotypes. Library preparation was performed with the TruSight One sequencing panel, which enriches the sample for about 4,800 genes of clinical relevance. Massively parallel sequencing was conducted with NextSeq. An identified variant was confirmed by Sanger sequencing of the patient and his parents. Finally, the patient was confirmed as the first Korean case of Floating-Harbor syndrome with a novel SRCAP (Snf2 related CREBBP activator protein) mutation (c.7732dupT, p.Ser2578Phefs*6), resulting in early termination of the protein; it was not found in either of his healthy parents or a control population. To our knowledge, this is the first study to describe a boy with Floating-Harbor syndrome with a novel SRCAP mutation diagnosed by targeted exome sequencing in Korea.
Child ; Ear ; Exome* ; High-Throughput Nucleotide Sequencing ; Humans ; Karyotype ; Korea ; Language Development Disorders ; Lip ; Male ; Nose ; Parents ; Phenotype

Nontuberculous mycobacteria (NTM) have been increasingly recognized as an important cause of chronic pulmonary infections. The Mycobacterium avium complex (MAC), which is composed of two species, Mycobacterium avium and Mycobacterium intracelluare, is the most commonly encountered pathogen associated with NTM lung disease. MAC pulmonary infection typically presents in a fibrocavitary form or a nodular bronchiectatic form. However, there have been atypical presentations of MAC pulmonary infections, including solitary pulmonary nodules (SPN). There have been several previous reports of SPN due to MAC infection in the United States, Japan, and Korea. In 2009, Sekine and colleagues reported a case of MAC pulmonary infection presenting with multiple nodules. To date, however, there have been no cases of NTM lung infection with multiple cavitary pulmonary nodules, and neither a fibrotic change nor nodular bronchiectasis. The present case showed a multiple cavitating nodular lung infection due to MAC, which is very rare and different from the typical presentation of MAC pulmonary infections. We also showed that percutaneous transthoracic needle aspiration can be a useful diagnostic tool to evaluate a case of multiple cavitary nodules.
Bronchiectasis ; Japan ; Korea ; Lung ; Lung Diseases ; Mycobacterium avium ; Mycobacterium avium Complex* ; Mycobacterium* ; Needles ; Nontuberculous Mycobacteria ; Solitary Pulmonary Nodule ; United States

PURPOSE: Infliximab is currently used for the treatment of active Crohn's disease (CD). We aimed to assess the efficacy and safety of infliximab therapy and to determine the predictors of response in Korean patients with CD. MATERIALS AND METHODS: A total of 317 patients who received at least one infliximab infusion for active luminal CD (n=198) and fistulizing CD (n=86) or both (n=33) were reviewed retrospectively in 29 Korean referral centers. Clinical outcomes of induction and maintenance therapy with infliximab, predictors of response, and adverse events were evaluated. RESULTS: In patients with luminal CD, the rates of clinical response and remission at week 14 were 89.2% and 60.0%, respectively. Male gender and isolated colonic disease were associated with higher remission rates at week 14. In week-14 responders, the probabilities of sustained response and remission were 96.2% and 93.3% at week 30 and 88.0% and 77.0% at week 54, respectively. In patients with fistulizing CD, clinical response and remission were observed in 85.0% and 56.2% of patients, respectively, at week 14. In week-14 responders, the probabilities of sustained response and remission were 94.0% and 97.1%, respectively, at both week 30 and week 54. Thirty-nine patients (12.3%) experienced adverse events related to infliximab. Serious adverse events developed in 19 (6.0%) patients including seven cases of active pulmonary tuberculosis. CONCLUSION: Infliximab induction and maintenance therapy are effective and well tolerable in Korean patients with luminal and fistulizing CD. However, clinicians must be aware of the risk of rare yet critical adverse events.
Colonic Diseases ; Crohn Disease* ; Humans ; Infliximab* ; Male ; Phenobarbital ; Referral and Consultation ; Retrospective Studies ; Tuberculosis ; Tuberculosis, Pulmonary