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.

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

Sodium-glucose cotransporter 2 (SGLT2) inhibitors are a new class of oral antihyperglycemic drugs; inhibition of the cotransporter allows for increased renal glucose excretion that consequently leads to reduced plasma glucose level. We report a rare case of a 73-year-old woman with type 2 diabetes mellitus who developed severe ketoacidosis caused by an SGLT2 inhibitor. At admission, severe ketoacidosis with arterial PH 6.99 was observed, though her serum glucose level of 232 mg/dL was not excessively high. It is necessary to pay attention to patients with type 2 diabetes being treated with an SGLT2 inhibitor, as anorexia, diarrhea, dehydration, and weight loss can develop in conjunction with a high fat/protein diet.
Aged ; Anorexia ; Blood Glucose ; Dehydration ; Diabetes Mellitus ; Diabetes Mellitus, Type 2 ; Diabetic Ketoacidosis* ; Diarrhea ; Diet ; Female ; Glucose ; Humans ; Hydrogen-Ion Concentration ; Ketosis ; Weight Loss

In order to investigate the implication of viral replication in acute, subacute, and chronic infections of coxsackievirus B3 (CVB3), we examined the histopathological changes and plus- and minus-strand viral RNA dynamics in heart, pancreas, brain, and liver of CVB3-infected A/J mice. Mice were inoculated intraperitoneally with CVB3 and sacrificed on 1, 2, 3, 4, 7, 10, 14, 21, 30, 60, and 90 days post infection (p.i.). Plus- and minus-strand viral RNAs in the organs were quantitated and the organs were additionally evaluated histopathologically for inflammation. No inflammatory infiltrates were observed in the liver, brain, and heart. In contrast, massive lymphocyte infiltration and fat replacement were shown in the pancreas with loss of acinar cells. Both plus- and minus-strand viral RNA levels were detected by 21 days p.i. in heart, 90 days p.i. in pancreas, 4 days p.i. in liver, and 10 days p.i. in brain. The plus-strand RNA was found at least fifty fold higher than the minus-strand RNA by 4 days p.i. in heart and pancreas and by 3 days p.i. in liver. The plus- to minus-strand RNA ratio in brain was found less than 1:20. Our data indicate that viral replication was actively occurred in heart, pancreas, and liver during acute CVB3 infection, whereas viral replication was limited in brain. Furthermore, chronic persistent viral RNA was observed in pancreas. In conclusion, CVB3 at low dose of virus induces severe pancreatitis but marginal or no inflammatory changes in the heart, liver, and brain.
Acinar Cells ; Animals ; Brain ; Heart ; Inflammation ; Liver ; Lymphocytes ; Mice ; Pancreas ; Pancreatitis ; RNA ; RNA, Viral ; Viruses

BACKGROUND/AIMS: Although polyethylene glycol (PEG) solution is safe and effective for bowel preparation for colonoscopy, its salty taste as well as the required large volume limits its use. Sulfate free PEG (SF-PEG) solution was developed to reduce the salty taste by removing the sodium sulfate. The aim of this study was to compare the SF-PEG solution with the standard PEG solution in regard to the quality of bowel preparation, the degree of patients' satisfaction and the side effects in Korean patients. METHODS: From July through September in 2006, 100 patients who underwent colonoscopic polypectomy were prospectively enrolled. They were randomly assigned to receive either PEG or SF-PEG solution. The quality of bowel preparation was assessed by one colonoscopist who was blinded with regard to the type of preparation used. Detailed questionnaires were used to assess the patients' tolerance and satisfaction. The serum biochemical parameters were measured before and after bowel cleansing. RESULTS: The numbers of patients whose quality of bowel preparation was excellent, good, fair and poor were 2, 43, 5 and 0 in PEG group, while this was 0, 41, 9 and 0 in SF-PEG group, respectively (p=0.118). The side effects were not different between the groups. The visual analog scale score of the patients' satisfaction was 6.4+/-2.4 in the PEG group and 5.8+/-2.8 in the SF-PEG group (p=0.237). The changes of serum biochemical parameters were also not different. CONCLUSIONS: The SF-PEG solution shows similar effectiveness and safety for bowel preparation for colonoscopy when compared to the standard PEG solution. The SF-PEG solution is a good solution to use for colonoscopy preparation.
Colonoscopy ; Humans ; Polyethylene ; Polyethylene Glycols ; Prospective Studies ; Sodium ; Sulfates ; Surveys and Questionnaires

The purpose of this study was to compare the nutrient intakes, blood pressure, and blood lipids in Korean adults according to BMI. Subjects were recruited and divided into three groups according to BMI, which included normal group (18.5< or =BMI<23), overweight group (23< or =BMI<25), obese group (25< or =BMI). The average age, height, weight, BMI of the subjects were 53.1years, 159.2cm, 54.5kg, 21.4kg/m2 for normal group, 52.3years, 159.4cm, 61.4kg, 24.1kg/m2 for overweight group, 55.9years, 158.7cm, 68.5kg, 27.2kg/m2 for obese group, respectively. There were not significant differences in energy and food intakes by BMI groups. The blood pressure, serum cholesterol, serum triglyceride, serum LDL-cholesterol, and AI were significantly increased and serum HDL-cholesterol decreased with increment of BMI. The age and BMI were significantly positively correlated with blood pressure and serum lipids except HDL-cholesterol. There was significantly negative correlation between percentage of protein energy intake and AI. To summarize these results, energy, nutrient, and food intakes were not significantly correlated with BMI, and blood pressure and blood lipids increased with increment of BMI. Further systematic study of relation among nutrient intakes including various life factors, BMI, blood pressure, and blood lipid profile is needed.
Adult* ; Blood Pressure* ; Body Mass Index* ; Cholesterol ; Energy Intake ; Humans ; Overweight ; Triglycerides

Minerals play important roles in biological processes. Hair mineral analysis has the advantages of conveniences in sampling and observing the profiles of multiple minerals simultaneously. The purpose of this study was to compare hair mineral contents between urban and local middle school students, and to find out the relation with nutrient intakes and clinical symptoms. Ten nutritional minerals and 7 environmental mineral contents were analyzed in the hair samples of 44 and 37 male students who resided in urban and local area, respectively. And their nutrient intake and clinical symptom score were estimated from questionnaire. The average age, height, and weight were 15.1 years, 168.4 cm, 56.8 kg in urban subjects and 15.4 years, 169.1 cm, 61.9 kg in local subjects, respectively. The residence types were apartment (63.6%) and small-sized apartment (22.7%) in urban subjects and apartment (51.4%) and house (37.8%) in local subjects. The primary water sources were purified water (38.3%), running water (18.2%) in urban subjects and purified water (32.4%), underground water (27.0%) in local subjects, respectively. Daily energy and nutrient intakes were not significantly different between two groups. Among the surveyed 17 clinical symptoms, the scores of constipation, cold, anxieties and total score of clinical symptoms in urban subjects were significantly lower than those in local subjects. Hair contents of Na, Mg, Ca, Cu, V, Al, As were significantly higher, while U was lower in urban subjects compared to those in rural subjects. Hair contents of Ni and U were significantly correlated with food intake, and Cr, V, Al, Sb, As, U showed a correlation with energy intake. Hair Mg level showed significantly negative correlation with total clinical symptom score. In conclusion, hair mineral profile between urban and local students was significantly different. And some of these minerals were significantly correlated with food and nutrient intakes. Especially hair magnesium was significantly correlated with total clinical symptom score. Therefore, hair mineral levels would be needed for more systematic study elucidating potentiality as a useful clinical tool.
Anxiety ; Biological Processes ; Constipation ; Eating ; Energy Intake ; Groundwater ; Hair* ; Humans ; Magnesium ; Male* ; Minerals ; Running ; Water ; Surveys and Questionnaires

PURPOSE: Respiratory motion is a considerable inhibiting factor for precise treatment with stereotactic radiosurgery using the CyberKnife (CK). In this study, we developed a moving phantom to simulate three-dimensional breathing movement and investigated the distortion of dose profiles between the use of a moving phantom and a static phantom. MATERIALS AND METHODS: The phantom consisted of four pieces of polyethylene; two sheets of Gafchromic film were inserted for dosimetry. Treatment was planned to deliver 30 Gy to virtual tumors of 20, 30, 40, and 50 mm diameters using 104 beams and a single center mode. A specially designed robot produced three-dimensional motion in the right-left, anterior-posterior, and craniocaudal directions of 5, 10 and 20 mm, respectively. Using the optical density of the films as a function of dose, the dose profiles of both static and moving phantoms were measured. RESULTS: The prescribed isodose to cover the virtual tumors on the static phantom were 80% for 20 mm, 84% for 30 mm, 83% for 40 mm and 80% for 50 mm tumors. However, to compensate for the respiratory motion, the minimum isodose levels to cover the moving target were 70% for the 30~50 mm diameter tumors and 60% for a 20 mm tumor. For the 20 mm tumor, the gaps between the isodose curves for the static and moving phantoms were 3.2, 3.3, 3.5 and 1.1 mm for the cranial, caudal, right, and left direction, respectively. In the case of the 30 mm tumor, the gaps were 3.9, 4.2, 2.8, 0 mm, respectively. In the case of the 40 mm tumor, the gaps were 4.0, 4.8, 1.1, and 0 mm, respectively. In the case of the 50 mm diameter tumor, the gaps were 3.9, 3.9, 0 and 0 mm, respectively. CONCLUSION: For a tumor of a 20 mm diameter, the 80% isodose curve can be planned to cover the tumor; a 60% isodose curve will have to be chosen due to the tumor motion. The gap between these 80% and 60% curves is 5 mm. In tumors with diameters of 30, 40 and 50 mm, the whole tumor will be covered if an isodose curve of about 70% is selected, equivalent of placing a respiratory margin of below 5 mm. It was confirmed that during CK treatment for a moving tumor, the range of distortion produced by motion was less than the range of motion itself.
Polyethylene ; Radiosurgery* ; Range of Motion, Articular ; Respiration

It has been suggested that isoflavones protect the cardiovascular system, in part by attenuating blood pressure. The purpose of the present research was to compare the isoflavone intake and blood lipids between hypertensive and normotensive and to examine dietary management for prevention of hypertension. Anthropometrical measurements, blood pressures, nutrients and isoflavone intakes using the 24-hour recall method, and serum lipids of 81 hypertensives and 77 normotensives were estimated. The average age, height, weight, and BMI were 58.2 years, 158.5 cm, 64.3 kg, and 25.6 kg/m2 for the hypertensive and 58.0 years, 159.4 cm, 63.0 kg, and 24.8 kg/m2 for the normotensive, respectively. Systolic and diastolic blood pressures of the hypertensive were significantly higher than those of the normotensive (p<0.001, p<0.001). The daily food intake of the hypertensive was significantly lower than that of the normotensive (p<0.01). The daily energy intakes of hypertensive and normotesive were 1479.8 kcal and 1590.9 kcal and there was no significant difference between the two groups. Carbohydrate intake of the hypertensive was significantly higher than that of the normotensive (p<0.05). However, daily intakes of plant protein, fiber, ash, calcium, sodium, potassium, and vitamin B1 of the hypertensive were significantly lower than those of the normotensive (p<0.05, p<0.01, p<0.01, p<0.01, p<0.05, p<0.05, p<0.05). The daily intakes of daidzein, genistein, isoflavone of the hypertensive were significantly lower than those of the normotensive (p<0.05, p<0.05, p<0.05). However, isoflavone intakes per 1000 kcal showed no significant difference between the two groups. Serum lipids of the hypertensive and normotensive were 189.6 mg/dL and 187.2 mg/dL for total cholesterol, 157.3 mg/dL and 161.9 mg/dL for triglyceride, 42.9 mg/dL and 43.5 mg/dL for HDL-cholesterol, 115.2 mg/dL and 111.4 mg/dL for LDL-cholesterol, and 3.5 and 3.4 for atherogenic index. In the total subjects, there was a significantly negative correlation between systolic blood pressure and genistein intake (p<0.05). Based on these results, we concluded that the daily intakes of food, energy, and isoflavones of hypertensives were lower than normotensives. Therefore, it should be emphasized that proper dietary management considering these dietary factors for prevention of hypertension.
Blood Pressure ; Calcium ; Cardiovascular System ; Cholesterol ; Eating ; Genistein ; Hypertension ; Isoflavones ; Plants ; Potassium ; Sodium ; Thiamine ; Triglycerides