1.Assessment of the Clinical Heterogeneity of Kawasaki Disease Using Genetic Variants of BLK and FCGR2A
Bo Kyung SIM ; Hyein PARK ; Jae Jung KIM ; Sin Weon YUN ; Jeong Jin YU ; Kyung Lim YOON ; Kyung Yil LEE ; Hong Ryang KIL ; Gi Beom KIM ; Myung Ki HAN ; Min Seob SONG ; Hyoung Doo LEE ; Kee Soo HA ; Sejung SOHN ; Young Mi HONG ; Gi Young JANG ; Jong Keuk LEE ;
Korean Circulation Journal 2019;49(1):99-108
BACKGROUND AND OBJECTIVES: Patients with Kawasaki disease (KD) are clinically heterogeneous because its diagnosis is based solely on clinical observation and there are no definitive biomarkers. We dissected the clinical heterogeneity of KD patients using the KD-associated genetic variants. METHODS: We performed a genetic association analysis in several KD subgroups categorized by clinical characteristics using the KD-associated variants of the B lymphoid tyrosine kinase (BLK; rs6993775) and Fc gamma receptor II a (FCGR2A; rs1801274) in a large number of case (n=1,011) and control (n=4,533) samples. RESULTS: BLK and FCGR2A were very significantly associated with KD in Korean KD patients (odds ratio [OR],1.48; p=4.63×10⁻¹¹ for BLK, and OR, 1.26; p=1.42×10⁻⁴ for FCGR2A). However, in KD subgroup analysis, we found that neither BLK nor FCGR2A were associated with either incomplete Kawasaki disease (iKD) type patients or those older than 5 years of age (p>0.2), suggesting that patients with iKD or those older than 5 years of age are a unique subgroup of KD. In genetic association analysis after excluding iKD patients and those older than 5 years old, we found that BLK was associated with all KD subgroups, whereas FCGR2A was specifically associated with male KD patients younger than 1 year of age (OR, 2.22; p=2.35×10⁻⁵). CONCLUSIONS: KD is a clinically and genetically heterogeneous disease. These findings will provide new insights into the clinical and genetic heterogeneity of KD.
Biomarkers
;
Diagnosis
;
Genetic Heterogeneity
;
Genome-Wide Association Study
;
Humans
;
Male
;
Mucocutaneous Lymph Node Syndrome
;
Polymorphism, Single Nucleotide
;
Population Characteristics
;
Protein-Tyrosine Kinases
2.Assessment of the Clinical Heterogeneity of Kawasaki Disease Using Genetic Variants of BLK and FCGR2A
Bo Kyung SIM ; Hyein PARK ; Jae Jung KIM ; Sin Weon YUN ; Jeong Jin YU ; Kyung Lim YOON ; Kyung Yil LEE ; Hong Ryang KIL ; Gi Beom KIM ; Myung Ki HAN ; Min Seob SONG ; Hyoung Doo LEE ; Kee Soo HA ; Sejung SOHN ; Young Mi HONG ; Gi Young JANG ; Jong Keuk LEE ;
Korean Circulation Journal 2019;49(1):99-108
BACKGROUND AND OBJECTIVES:
Patients with Kawasaki disease (KD) are clinically heterogeneous because its diagnosis is based solely on clinical observation and there are no definitive biomarkers. We dissected the clinical heterogeneity of KD patients using the KD-associated genetic variants.
METHODS:
We performed a genetic association analysis in several KD subgroups categorized by clinical characteristics using the KD-associated variants of the B lymphoid tyrosine kinase (BLK; rs6993775) and Fc gamma receptor II a (FCGR2A; rs1801274) in a large number of case (n=1,011) and control (n=4,533) samples.
RESULTS:
BLK and FCGR2A were very significantly associated with KD in Korean KD patients (odds ratio [OR],1.48; p=4.63×10â»Â¹Â¹ for BLK, and OR, 1.26; p=1.42×10â»â´ for FCGR2A). However, in KD subgroup analysis, we found that neither BLK nor FCGR2A were associated with either incomplete Kawasaki disease (iKD) type patients or those older than 5 years of age (p>0.2), suggesting that patients with iKD or those older than 5 years of age are a unique subgroup of KD. In genetic association analysis after excluding iKD patients and those older than 5 years old, we found that BLK was associated with all KD subgroups, whereas FCGR2A was specifically associated with male KD patients younger than 1 year of age (OR, 2.22; p=2.35×10â»âµ).
CONCLUSIONS
KD is a clinically and genetically heterogeneous disease. These findings will provide new insights into the clinical and genetic heterogeneity of KD.
3.Failure of Conservative Treatment in Two Cases of Polyarteritis Nodosa with Superior Mesenteric Artery Involvement Presenting with Abdominal Pain.
Ho Chan SIM ; Ju Hyoung LEE ; Ji Woon LEE ; Seung Hyun PARK ; Ju Won LEE ; Heui Jeong JEONG ; Jin LEE ; Sung Yeun YANG
The Korean Journal of Helicobacter and Upper Gastrointestinal Research 2017;17(3):154-158
Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis affecting medium- or small-sized arteries. Its diagnosis may be delayed because it is a rare disease, and patients presenting with PAN demonstrate variable clinical manifestations and non-specific laboratory abnormalities. Gastrointestinal involvement occurs in 14~65% of patients with PAN and is a significant cause of morbidity and mortality. Thus, early diagnosis is very important in PAN with gastrointestinal involvement. We report two cases of rapidly progressive PAN presenting with abdominal pain, having failed conservative treatment.
Abdominal Pain*
;
Arteries
;
Diagnosis
;
Early Diagnosis
;
Humans
;
Mesenteric Artery, Superior*
;
Mortality
;
Polyarteritis Nodosa*
;
Rare Diseases
;
Vasculitis
4.Specific Expression of Aplysia Phosphodiesterase 4 in Bag Cells Revealed by in situ Hybridization Analysis.
Deok Jin JANG ; Hyoung F KIM ; Jae Hoon SIM ; Chae Seok LIM ; Bong Kiun KAANG
Experimental Neurobiology 2015;24(3):246-251
Phosphodiesterases (PDEs) play a key role in the regulation of cyclic adenosine monophosphate (cAMP), which in turn mediates various cellular functions including learning and memory. We previously cloned and characterized three PDE4 isoforms (ApPDE4) from Aplysia kurodai. Using reverse transcription polymerase chain reaction (RT-PCR), we found that ApPDE4 isoforms are primarily expressed in the central nervous system. However, the detailed distribution of ApPDE4 mRNA in Aplysia individual ganglions was not evident. In this study, to determine the distribution of ApPDE4 mRNAs in Aplysia ganglions, we performed in situ hybridization (ISH) using a probe targeting ApPDE4, including the PDE catalytic domain. Interestingly, we found the strongest ISH-positive signals in the symmetrical bag cell clusters of the abdominal ganglion. The R2, R14, L7, L2 and L11 neurons in the abdominal ganglion, LP1 neuron in pleural ganglion, and metacerebral (MCC) neurons were ISH-positive. Mechanosensory neurons of the sensory cluster were also stained on the ventral aspect of the right and left pleural ganglia. Taken together, we found the detailed distribution of ApPDE4 mRNA in Aplysia ganglion and support their roles in serotonin (5-HT)-induced synaptic facilitation of Aplysia mechanosensory neurons.
Adenosine Monophosphate
;
Aplysia*
;
Catalytic Domain
;
Central Nervous System
;
Clone Cells
;
Cyclic Nucleotide Phosphodiesterases, Type 4*
;
Ganglia
;
Ganglion Cysts
;
In Situ Hybridization*
;
Learning
;
Memory
;
Neurons
;
Phosphoric Diester Hydrolases
;
Polymerase Chain Reaction
;
Protein Isoforms
;
Reverse Transcription
;
RNA, Messenger
;
Serotonin
5.Spontaneous Bacterial Peritonitis with Sepsis Caused by Enterococcus hirae.
Jong Seop SIM ; Hyoung Su KIM ; Ki Jong OH ; Myung Soo PARK ; Eun Ju JUNG ; Youn Joo JUNG ; Dae Gil KANG ; Seung In SEO ; Won Jin KIM ; Myoung Kuk JANG
Journal of Korean Medical Science 2012;27(12):1598-1600
Selective intestinal decontamination (SID) with norfloxacin has been widely used for the prophylaxis of spontaneous bacterial peritonitis (SBP) because of a high recurrence rate and preventive effect of SID for SBP. However, it does select resistant gut flora and may lead to SBP caused by unusual pathogens such as quinolone-resistant gram-negative bacilli or gram-positive cocci. Enterococcus hirae is known to cause infections mainly in animals, but is rarely encountered in humans. We report the first case of SBP by E. hirae in a cirrhotic patient who have previously received an oral administration of norfloxacin against SBP caused by Klebsiella pneumoniae and presented in septic shock.
Administration, Oral
;
Ampicillin/therapeutic use
;
Anti-Bacterial Agents/therapeutic use
;
Ascitic Fluid/microbiology
;
Enterococcus/*isolation & purification
;
Gram-Positive Bacterial Infections/complications/drug therapy/*microbiology
;
Humans
;
Male
;
Microbial Sensitivity Tests
;
Middle Aged
;
Peritonitis/*diagnosis/drug therapy/microbiology
;
Sepsis/*etiology
6.Comparison of Clinical Manifestations of RSV, Rhinovirus and Bocavirus Infections in Children with Acute Wheezing.
Hyun Wook SHIN ; Ha Lim CHO ; Ju Hee YOU ; Eun Jung YOU ; Eun Young KIM ; Kyoung Sim KIM ; Yong Wook KIM ; Jin Jong SEO ; Hyoung Min CHO
Pediatric Allergy and Respiratory Disease 2011;21(4):334-343
PURPOSE: The purpose of this study was to compare clinical manifestations of human bocavirus (hBoV), respiratory syncytial virus (RSV), and human rhinovirus (hRV) in children with acute wheezing. METHODS: We retrospectively investigated 549 virus-positive throat swabs obtained between January 2006 and December 2008 from pediatric inpatients with acute respiratory tract disease at Kwangju Christian Hospital. Among them, 109 patients, who had lower respiratory tract infections with wheezing, were enrolled in this study. The medical records of patients with positive results were reviewed for clinical data. RESULTS: The mean age of the patients with RSV was 7.15 months, 15.66 months in those with hRV, and 15.04 months in those with hBoV. The mean fever duration and frequency of patients with fever was 2.43 days and 47.9% for RSV, 2.86 days and 51.7% for hRV, and 3.75 days and 69.6% for hBoV. The frequency of patients with acute otitis media was 20.8% in the RSV, 20.7% in the hRV, and 13.0% in the hBoV groups. The frequency of lung infiltration on chest X-ray was 12.5% in the RSV, 20.7% in the hRV, and 47.8% in the hBoV groups. CONCLUSION: We compared the clinical manifestations of respiratory viral infections in infants and children with wheezing. However, further surveillance will be necessary to clarify the clinical manifestations of the viruses.
Bocavirus
;
Child
;
Fever
;
Human bocavirus
;
Humans
;
Infant
;
Inpatients
;
Korea
;
Lung
;
Medical Records
;
Otitis Media
;
Pharynx
;
Respiratory Sounds
;
Respiratory Syncytial Viruses
;
Respiratory Tract Diseases
;
Respiratory Tract Infections
;
Retrospective Studies
;
Rhinovirus
;
Thorax
7.Effects of Combined Therapy with Ezetimibe Plus Simvastatin After Drug-Eluting Stent Implantation in a Porcine Coronary Restenosis Model.
Jung Sun CHO ; Myung Ho JEONG ; Doo Sun SIM ; Young Joon HONG ; Kyung Seob LIM ; Jung Ha KIM ; Hyoung Doo KIM ; Ju Yeal BAEK ; Hee Jeoung YOON ; Sung Ho HER ; Seung Won JIN ; Ju Han KIM ; Youngkeun AHN ; Jeong Gwan CHO ; Jong Chun PARK ; Jung Chaee KANG
Journal of Korean Medical Science 2010;25(5):716-722
The aim of this study was to examine the anti-proliferative and anti-inflammatory effects of ezetimibe/simvastatin (E/S) after drug-eluting stent (DES) implantation in a porcine coronary restenosis model. Pigs were randomized into two groups in which the coronary arteries (23 pigs) had DES. Stents were deployed with oversizing (stent/artery ratio 1.3:1) in porcine coronary arteries. Fifteen pigs were taken 10/20 mg of E/S and eight pigs were not taken E/S. Histopathologic analysis was assessed at 28 days after stenting. In neointima, most inflammatory cells were lymphohistiocytes. Lymphohistiocyte count was not different between two groups (337+/-227 vs. 443+/-366 cells, P=0.292), but neointima area was significantly smaller (1.00+/-0.49 mm2 vs. 1.69+/-0.98 mm2, P=0.021) and percent area stenosis was significantly lower (23.3+/-10% vs. 39+/-19%, P=0.007) in E/S group compared with control group. There were no significant differences in fibrin score (1.99+/-0.79 vs. 1.81+/-0.88, P=0.49), endothelial score (1.75+/-0.66 vs. 1.80+/-0.59, P=0.79), and the percent of endothelium covered lumen (43+/-21% vs. 45+/-21%, P=0.84) between E/S group and control group. Combined therapy with ezetimibe and simvastatin inhibits neointimal hyperplasia, but does not inhibit inflammatory infiltration and arterial healing after DES implantation in a porcine coronary restenosis model.
Animals
;
Anticholesteremic Agents/administration & dosage
;
Azetidines/*administration & dosage
;
Coronary Restenosis/diagnosis/drug therapy/*etiology
;
*Disease Models, Animal
;
Drug Combinations
;
Drug Implants/administration & dosage
;
Drug-Eluting Stents/*adverse effects
;
Female
;
Graft Occlusion, Vascular/diagnosis/*drug therapy/*etiology
;
Humans
;
Hydroxymethylglutaryl-CoA Reductase Inhibitors/administration & dosage
;
Simvastatin/*administration & dosage
;
Swine
;
Treatment Outcome
8.Diet and Airway Obstruction: A Cross Sectional Study from the Second Korean National Health and Nutrition Examination Survey.
Jin Hwa LEE ; Yun Su SIM ; Gee Young SUH ; Jeong Seon RYU ; Dong Ho SHIN ; Kyung Haeng KOH ; Yeon Jae KIM ; Wan PARK ; Hyoung Kyu YOON ; Man Jae LEE ; Jung Hyun CHANG
The Korean Journal of Internal Medicine 2010;25(2):132-139
BACKGROUND/AIMS: Several dietary factors, such as antioxidant vitamins, have potential roles in the development of obstructive lung diseases. However, the results of studies on the relationships between dietary factors and obstructive lung diseases are inconsistent. The aim of this study was to determine which nutrients are related to airway obstruction (AO) in the Korean population. METHODS: We used data obtained as part of the Korean National Health and Nutrition Examination Survey (NHANES II) in 2001. Analysis was restricted to 1,005 adults who were 18 years of age and older, who had two or more acceptable spirometry curves, and who had participated in the nutrition examination survey. AO was defined as the ratio of forced expiratory volume in one second (FEV1) to forced vital capacity (FVC) of less than 0.7. RESULTS: Of the 1,005 study subjects, 78 (7.8%) had AO. Statistically significant factors associated with AO were 55 years of age or older (p = 0.032), central obesity (p = 0.047), hypertension (p < 0.001), smoking of 20 pack-years or more (p < 0.001), low income (p < 0.001), and low dietary protein intake expressed as a ratio of protein to recommended dietary allowance for Koreans (p = 0.037). Multiple logistic regression analyses revealed four factors that were independently associated with AO: smoking of 20 pack-years or more (odds ratio [OR], 5.801; p < 0.001), hypertension (OR, 3.905; p < 0.001), low protein intake (OR, 0.992; p = 0.004), and low income (OR, 1.962; p = 0.018). CONCLUSIONS: In the Korean NHANES, smoking, hypertension, and low income were related to AO. Among dietary factors, only low protein intake was associated with AO.
Adult
;
Airway Obstruction/*epidemiology
;
Antioxidants/administration & dosage
;
Body Mass Index
;
Cross-Sectional Studies
;
Dietary Proteins/administration & dosage
;
Female
;
Forced Expiratory Volume
;
Humans
;
Logistic Models
;
Male
;
Malnutrition/*epidemiology
;
Middle Aged
;
Minerals/administration & dosage
;
Nutrition Surveys
;
Obesity/epidemiology
;
Prevalence
;
Republic of Korea/epidemiology
;
Risk Factors
;
Smoking/*epidemiology
;
Spirometry
;
Vital Capacity
;
Vitamins/administration & dosage
9.Recurrent Wheezing After Human Bocavirus Lower Respiratory Tract Illnesses in Early Life.
Se Rin NA ; Bong Chil IM ; Ju Hee YOU ; Eun Jung YOU ; Eun Young KIM ; Kyoung Sim KIM ; Yong Wook KIM ; Jin Jong SEO ; Yoon Seok CHUNG ; Hyoung Min CHO
Pediatric Allergy and Respiratory Disease 2010;20(4):247-255
PURPOSE: Excluding RSV, the relationship between bronchiolitis caused by viruses and the development of wheezing and atopy in childhood has not been well studied. We studied this relationship in children who had bronchiolitis caused by human bocavirus before 2 years of age. METHODS: We retrospectively investigated 2,430 throat swab obstained between January 2005 and December 2007 from pediatric in-patients with acute respiratory tract disease at the Kwangju Christian Hospital. Human bocavirus was detected in 112 patients. A total of 61 patients less than 2 years of age were finally enrolled in this study. Patients were followed up between April and June of 2008. We measured the frequency of wheezing and atopic status using (allergy skin-prick tests, CAP tests and MAST tests). RESULTS: Of the 61 patients, 16 (26.2%) had recurrent wheezing. Of these 16 patients, 8 (13.1%) had Infrequent wheezing (1-2 wheezing episodes) and 8 (13.1%) had frequent wheezing (3 and over wheezing episodes). Of the total 61 patients, 18 (29.5%) completed allergy tests. Of the 18 patients, 10 (55.6%) were sensitized to at least 1 allergen. Recurrent wheezing was significantly associated with the severity of bronchiolitis (mild vs. moderate vs. severe; 9.1% vs. 22.2% vs. 66.7%). CONCLUSION: Human bocavirus-induced bronchiolitis in childhood are an independent risk factor for development of wheezing in childhood and may be associated with an increased risk of allergic sensitization. The most important risk factor for recurrent wheezing is the severity of lower respiratory tract illnesses.
Bronchiolitis
;
Child
;
Human bocavirus
;
Humans
;
Hypersensitivity
;
Korea
;
Pharynx
;
Respiratory Sounds
;
Respiratory System
;
Respiratory Tract Diseases
;
Retrospective Studies
;
Risk Factors
10.Clinical Spectrum of Norovirus Gastroenteritis Compared to Rotavirus Gastroenteritis at a Single Center in Gwanju, Korea during 2005-2006; Compared to Rotaviral Gastroenteritis.
Yang Jin LEE ; Seong Nam JEONG ; Ju Hee YOO ; Hyoung Min CHO ; Eun Jung YOO ; Eun Young KIM ; Yong Wook KIM ; Kyoung Sim KIM ; Sun Hee KIM
Korean Journal of Pediatric Infectious Diseases 2009;16(1):61-72
PURPOSE:We evaluated the clinical features of Norovirus gastroenteritis compared with Rotavirus gastroenteritis in hospitalized children. METHOD:We detected causative agents in 3,261 samples of children hospitalized with gastroenteritis symptoms at a single center of pediatrics between 2005 and 2006. Among 266 and 303 samples which tested positive for Norovirus and Rotavirus, we selected 73 and 182 samples of children with relatively pure gastroenteritis symptoms and retrospectively analyzed the corresponding medical records. RESULTS:The male-to-female ratio of the Norovirus (+) and Rotavirus (+) groupswas 1.43:1 and 1.56:1 both groups were predominantly in males. The mean age of the Norovirus (+) and Rotavirus (+) groups was 36.7 and 24.4 months, respectively the children in the former group were older than the children in the latter group. The incidence in the Norovirus (+) group was more concentrated in the winter. The symptoms in the Norovirus (+), in decreasing order, included vomiting, diarrhea, and fever. The duration of vomiting, diarrhea, and fever was 2.1, 1.2, and 1.2 days. The maximum number of episodes of vomiting and diarrhea per day was 3.5 and 4.5, respectively. The severity score was 10.16. The symptoms inthe Rotavirus (+) group, in decreasing order, included diarrhea, vomiting, and fever. The duration of diarrhea, vomiting, and fever was 2.2, 4.3, and 2.2 days, respectively. The maximum number of episodes of vomiting and diarrhea per day was 3.3 and 6.5, respectively. The severity score was 11.9. The severity in the Norovirus (+) group was somewhat lower than the Rotavirus (+) group. The younger the child, the more severe the symptoms in the Norovirus (+) group. There was no difference between mono-and co-infection in severity and between the two groups regarding the hematologic findings. CONCLUSION:Based on the findings reported herein, additional studies about prophylaxis, as well as the epidemiology and clinical features of pediatric Norovirus gastroenteritis, are required.
Child
;
Child, Hospitalized
;
Coinfection
;
Diarrhea
;
Fever
;
Gastroenteritis
;
Humans
;
Incidence
;
Korea
;
Male
;
Norovirus
;
Pediatrics
;
Retrospective Studies
;
Rotavirus
;
Vomiting

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