Your browser doesn't support javascript.
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 107
Filtrar
Mais filtros










Base de dados
Intervalo de ano de publicação
1.
J Hum Genet ; 65(4): 421-426, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31965063

RESUMO

Kawasaki disease (KD) is an acute, self-limited vasculitis, mainly affecting children younger than 5 years old, with accompanying fever and signs of mucocutaneous inflammation. Intravenous immunoglobulin (IVIG) is the standard treatment for KD; however, ~15% of patients are resistant to IVIG treatment. To identify protein coding genetic variants influencing IVIG resistance, we re-analyzed our previous genome-wide association study (GWAS) data from 296 patients with KD, including 101 IVIG non-responders and 195 IVIG responders. Five nonsynonymous SNPs (nsSNPs) in five immune-related genes, including a previously reported SAMD9L nsSNP (rs10488532; p.Val266Ile), were associated with IVIG non-response (odds ratio [OR] = 1.89-3.46, P = 0.0109-0.0035). In a replication study of the four newly-identified nsSNPs, only one in the interleukin 16 (IL16) gene (rs11556218, p.Asn1147Lys) showed a trend of association with IVIG non-response (OR = 1.54, P = 0.0078). The same IL16 nsSNP was more significantly associated with IVIG non-response in combined analysis of all data (OR = 1.64, P = 1.25 × 10-4). Furthermore, risk allele combination of the IL16 CT and SAMD9L TT nsSNP genotypes exhibited a very strong effect size (OR = 9.19, P = 3.63 × 10-4). These results implicate IL16 as involved in the mechanism of IVIG resistance in KD.

2.
Pharmacogenomics J ; 20(1): 80-86, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30971808

RESUMO

Kawasaki disease (KD) is a systemic vasculitis affecting infants and children; it manifests as fever and signs of mucocutaneous inflammation. Intravenous immunoglobulin (IVIG) treatment effectively attenuates the fever and systemic inflammation. However, 10-20% patients are unresponsive to IVIG. To identify genetic variants influencing IVIG non-response in KD, a genome-wide association study (GWAS) and a replication study were performed using a total of 148 IVIG non-responders and 845 IVIG-responders in a Korean population. rs28662 in the sterile alpha motif domain-containing protein 9-like (SAMD9L) locus showed the most significant result in the joint analysis of GWAS and replication samples (odds ratio (OR) = 3.47, P = 1.39 × 10-5). The same SNP in the SAMD9L locus was tested in the Japanese population, and it revealed a more significant association in a meta-analysis with Japanese data (OR = 4.30, P = 5.30 × 10-6). These results provide new insights into the mechanism of IVIG response in KD.

3.
Artigo em Inglês | MEDLINE | ID: mdl-31851089

RESUMO

BACKGROUND AND AIMS: All published meta-analyses failed to demonstrate that preoperative transarterial chemoembolization improves the clinical outcomes of patients with resectable hepatocellular carcinoma. The present study aimed to investigate the utility of systemic inflammatory cells as a tumor biology marker predicting therapeutic benefit of neoadjuvant transarterial chemoembolization in patients with resectable hepatocellular carcinoma. MATERIALS AND METHODS: We retrospectively investigated 441 hepatocellular carcinoma patients who underwent curative resection. Among 441 patients, 73 patients underwent preoperative transarterial chemoembolization, and 368 patients did not. We compared recurrence-free survival and overall survival between transarterial chemoembolization plus sequential resection group and resection only group. We analyzed whether pretreatment neutrophil-lymphocyte ratio demonstrates survival benefit in each groups. RESULTS: No significant difference was observed in recurrence-free or overall survival between both groups. In the transarterial chemoembolization plus sequential resection group, the 5-year overall survival in patients with high neutrophil-lymphocyte ratio (≥1.6) was significantly lower than that in patients with low neutrophil-lymphocyte ratio (78.4% and 100%, P = 0.027). High neutrophil-lymphocyte ratio was associated with vascular invasion (P = 0.033). CONCLUSION: Neutrophil-lymphocyte ratio can be considered as a predictive factor of long-term survival and used to identify patients with resectable hepatocellular carcinoma who benefit from neoadjuvant transarterial chemoembolization.

4.
Artigo em Inglês | MEDLINE | ID: mdl-31723976

RESUMO

CONTEXT: No universal waist circumference (WC) percentile cut-offs used have been proposed for screening central obesity in children and adolescents. OBJECTIVE: To develop international WC percentile cut-offs for children and adolescents with normal weight based on data from eight countries in different regions and examine the relation with cardiovascular risk. DESIGN AND SETTING: We used pooled data on WC in 113,453 children and adolescents (males, 50.2%) aged 4-20 years from eight countries in different regions (Bulgaria, China, Iran, Korea, Malaysia, Poland, Seychelles, and Switzerland). We calculated WC percentile cut-offs in samples including or excluding children with obesity, overweight or underweight. WC percentiles were generated using the general additive model for location scale and shape (GAMLSS). We also estimated the predictive power of the WC 90th percentile cut-offs to predict cardiovascular risk using receiver operator characteristics curve analysis based on data from three countries that had available data (China, Iran and Korea). We also examined which WC percentiles connected with WC cut-offs for central obesity in adults (at age of 18 years). MAIN OUTCOME MEASURE: WC measured based on recommendation by the World Health Organization. RESULTS: We validated the performance of the age- and sex- specific 90th percentile WC cut-offs calculated in children and adolescents (6-18 years of age) with normal weight (excluding youth with obesity, overweight or underweight) by linking it with cardiovascular risk (AUC: 0.69 for boys; 0.63 for girls). In addition, WC percentile among normal weight children linked relatively well with established WC cut-offs for central obesity in adults (e.g., AUC in US adolescents: 0.71 for boys; 0.68 for girls). CONCLUSION: The international WC cut-offs developed in this study could be useful to screen central obesity in children and adolescents aged 6-18 years and allow direct comparison of WC distributions between populations and over time.

5.
Hypertension ; 74(6): 1343-1348, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31630571

RESUMO

In 2017, the American Academy of Pediatrics (AAP) updated the clinical practice guideline for high blood pressure (BP) in the pediatric population. In this study, we compared the difference in prevalence of elevated and hypertensive BP values defined by the 2017 AAP guideline and the 2004 Fourth Report and estimated the cardiovascular risk associated with the reclassification of BP status defined by the AAP guideline. A total of 47 200 children and adolescents aged 6 to 17 years from 6 countries (China, India, Iran, Korea, Poland, and Tunisia) were included in this study. Elevated BP and hypertension were defined according to 2 guidelines. In addition, 1606 children from China, Iran, and Korea who were reclassified upward by the AAP guideline compared with the Fourth Report and for whom laboratory data were available were 1:1 matched with children from the same countries who were normotensive by both guidelines. Compared with the Fourth Report, the prevalence of elevated BP defined by the AAP guideline was lower (14.9% versus 8.6%), whereas the prevalence of stages 1 and 2 hypertension was higher (stage 1, 6.6% versus 14.5%; stage 2, 0.4% versus 1.7%). Additionally, comparison of laboratory data in the case-control study showed that children who were reclassified upward were more likely to have adverse lipid profiles and high fasting blood glucose compared with normotensive children. In conclusion, the prevalence of elevated BP and hypertension varied significantly between both guidelines. Applying the new AAP guideline could identify more children with hypertension who are at increased cardiovascular risk.

6.
Korean Circ J ; 49(12): 1167-1180, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31456368

RESUMO

BACKGROUND AND OBJECTIVES: Hypertension is becoming one of the most common health conditions in children and adolescents due to increasing childhood obesity. We aimed to provide the auscultatory blood pressure (BP) normative reference values for Korean non-overweight children and adolescents. METHODS: BP measurements in children and adolescents aged 10 to 18 years were performed in the Korean National Health and Nutrition Examination Survey (KNHANES) from 1998 to 2016. BP was measured using a mercury sphygmomanometer. Sex-, age- and height-specific systolic BP (SBP) and diastolic BP (DBP) percentiles were calculated in the non-overweight children (n=10,442). We used the General Additive Model for Location Scale and Shape method to calculate BP percentiles. RESULTS: The 50th, 90th, 95th, and 99th percentiles of SBP and DBP tables and graphs of non-overweight children and adolescents aged 10 to 18 years were presented by age and height percentiles. We found that the SBP and DBP at the 95th percentile were well correlated with height. The BP tables presented by height contained BP values from 124 cm to 190 cm for boys and from 120 cm to 178 cm for girls. Boys had higher SBP and DBP. CONCLUSIONS: We provided the sex-, age- and height-specific auscultatory BP values using the KNHANES big data. These may be useful in diagnosis and treatment of hypertension in Korean children and adolescents.

7.
J Clin Hypertens (Greenwich) ; 21(9): 1335-1342, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31389662

RESUMO

Pediatric elevated blood pressure (BP) and hypertension are usually defined using traditional BP tables at the 90th and 95th percentiles, respectively, based on sex, age, and height, which are cumbersome to use in clinical practice. The authors aimed to assess the performance of the static cut-points (120/80 mm Hg and 130/80 mm Hg for defining elevated BP and hypertension for adolescents, respectively; and 110/70 mm Hg and 120/80 mm Hg for children, respectively) in predicting increased arterial stiffness. Using data from five population-based cross-sectional studies conducted in Brazil, China, Korea, and New Zealand, a total of 2546 children and adolescents aged 6-17 years were included. Increased arterial stiffness was defined as pulse wave velocity ≥sex-specific, age-specific, and study population-specific 90th percentile. Compared to youth with normal BP, those with hypertension defined using the 2017 American Academy of Pediatrics guideline (hereafter referred to as "percentile-based cut-points") and the static cut-points were at similar risk of increased arterial stiffness, with odds ratios and 95% confidence intervals of 2.35 (1.74-3.17) and 3.07 (2.20-4.28), respectively. Area under the receiver operating characteristic curve and net reclassification improvement methods confirmed the similar performance of static cut-points and percentile-based cut-points (P for difference > .05). In conclusion, the static cut-points performed similarly well when compared with the percentile-based cut-points in predicting childhood increased arterial stiffness. Use of static cut-points to define hypertension in childhood might simplify identification of children with abnormal BP in clinical practice.

8.
Korean Circ J ; 49(9): 866-876, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31165592

RESUMO

BACKGROUND AND OBJECTIVES: Elevated endothelin (ET)-1 level is strongly correlated with the pathogenesis of pulmonary arterial hypertension (PAH). Expression level of nicotinamide adenine dinucleotide phosphate oxidase (NOX) 4 is increased in the PAH patients. Ambrisentan, a selective endothelin receptor A (ERA) antagonist, is widely used in PAH therapy. The current study was undertaken to evaluate the effects of ambrisentan treatment in the monocrotaline (MCT)-induced PAH rat model. METHODS: Rats were categorized into control group (C), monocrotaline group (M) and ambrisentan group (Am). The M and Am were subcutaneously injected 60 mg/kg MCT at day 0, and in Am, ambrisentan was orally administered the day after MCT injection for 4 weeks. The right ventricle (RV) pressure was measured and pathological changes of the lung tissues were observed by Victoria blue staining. Protein expressions of ET-1, ERA, endothelial nitric oxide synthase (eNOS) and NOX4 were confirmed by western blot analysis. RESULTS: Ambrisentan treatment resulted in a recovery of the body weight and RV/left ventricle+septum at week 4. The RV pressure was lowered at weeks 2 and 4 after ambrisentan administration. Medial wall thickening of pulmonary arterioles and the number of intra-acinar arteries were also attenuated by ambrisentan at week 4. Protein expression levels of ET-1 and eNOS were recovered at weeks 2 and 4, and ERA levels recovered at week 4. CONCLUSIONS: Ambrisentan administration resulted in the recovery of ET-1, ERA and eNOS protein expression levels in the PAH model. However, the expression level of NOX4 remained unaffected after ambrisentan treatment.

10.
Eur J Gastroenterol Hepatol ; 31(10): 1250-1255, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30925530

RESUMO

BACKGROUND AND AIM: Although sorafenib is the first systemic therapy to show survival benefit for advanced hepatocellular carcinoma (HCC), its survival benefit is variable for HCC. Systemic inflammation may be associated with survival in HCC. We investigated the use of systemic inflammation markers, including neutrophil-to-lymphocyte ratio (NLR), in the prognosis of sorafenib-treated HCC patients. PATIENTS AND METHODS: We retrospectively analyzed data of 82 patients with advanced HCC who received sorafenib as the first-line treatment. Data on pretreatment and post-treatment (2-3 months after initiating sorafenib therapy, first tumor response evaluation day) clinical, laboratory, and tumor characteristics were collected. Survival-related prognostic factors were analyzed. RESULTS: Patients were mostly in the intermediate (12.2%) or advanced (87.8%) Barcelona Clinic Liver Cancer stages. Fifty-six (68.3%) patients had vascular invasion and 34 (41.5%) patients had extrahepatic disease. The median progression-free survival (PFS) and overall survival (OS) were 4.7 months [95% confidence interval (CI): 2.8-6.5 months] and 4.7 months (95% CI: 2.8-6.5 months). In multivariate analysis for OS, diarrhea (hazard ratio: 0.588; 95% CI: 0.348-0.993) and NLR decline (decreased compared with pretreatment) (hazard ratio: 0.479; 95% CI: 0.300-0.765) were independent factors of good OS. In the NLR decline group, the median PFS and OS were 7.1 and 7.3 months, respectively. In the NLR nondecline group, the median PFS and OS were 3.0 and 3.2 months, respectively. The difference in OS between the two groups was significant (P = 0.004). CONCLUSION: A change in NLR after sorafenib therapy was associated with a better prognosis in patients with advanced HCC.

11.
Korean Circ J ; 49(3): 223-237, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30808073

RESUMO

It has been known for a long time that elevated blood pressure (BP) in the young may persist and progress into adult hypertension (HTN). Multiple studies have revealed the predicted BP trajectory lines starting from childhood and related them to later cardiovascular (CV) risks in adulthood. As a small baby grows into a tall adult, BP will also naturally increase. Among early-life predictors of adult HTN, birth history, such as prematurity, and low birth weight have been popular subjects in research on pediatric HTN, because body size at birth has been reported to be inversely related to the risk of adulthood HTN. The hypothesis of HTN in prematurely born adolescents has been postulated as a physiological predisposition to postnatal excessive weight gain. Current body weight is a well-known independent predictor of HTN in children, and some studies showed that children demonstrating upward crossing of their weight percentiles while growing into adolescents have significantly increased risk for elevated BP later in life. Recently, reports focused on the adverse effect of excessive catch-up growth in this population are gradually drawing attention. Accordingly, children born prematurely or with intrauterine growth restriction who show rapid changes in their weight percentile should be under surveillance with BP monitoring. Prevention of childhood obesity, along with special care for premature infants or infants small for their gestational age, by providing healthy nutritional guidelines should be cardinal strategies for the prevention of adult HTN and CV risks later in life.

12.
Pediatr Cardiol ; 40(3): 483-488, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30267110

RESUMO

Kawasaki disease (KD) is a self-limiting systemic vasculitis of unknown etiology. KD is often complicated by coronary artery aneurysms (CAAs), which develop in about 20-25% of untreated children and 3-5% of children treated with intravenous immunoglobulin therapy. To identify the risk loci for CAA susceptibility in patients with KD, we performed a genome-wide association study (GWAS) using our previous Illumina HumanOmni1-Quad BeadChip data (296 KD patients) and a new replication study in an independent sample set (713 KD patients) by grouping KD patients without CAA (control) versus KD patients with extremely large aneurysms (diameter ≥ 5 mm) (case). Among 44 candidate single -nucleotide polymorphisms (SNPs) selected from the initial GWAS data (33 cases vs. 215 controls), a SNP (rs899162) located 7 kb upstream of the TIFAB gene on chromosome five was replicated in an independent sample (12 cases vs. 532 controls). In the combined analysis (45 cases vs. 747 controls), the SNP (rs899162) showed a highly significant association with CAA formation (diameter ≥ 5 mm) in patients with KD (odds ratio = 3.20, 95% confidence interval = 2.02-5.05, Pcombined = 1.95 × 10-7). These results indicate that the TIFAB gene may act as a CAA susceptibility locus in patients with KD.


Assuntos
Aneurisma Coronário/genética , Síndrome de Linfonodos Mucocutâneos/complicações , Fator 6 Associado a Receptor de TNF/genética , Estudos de Casos e Controles , Aneurisma Coronário/etiologia , Vasos Coronários/patologia , Predisposição Genética para Doença , Estudo de Associação Genômica Ampla , Genótipo , Humanos , Síndrome de Linfonodos Mucocutâneos/genética , Polimorfismo de Nucleotídeo Único
13.
Korean J Pediatr ; 62(3): 95-101, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30360036

RESUMO

PURPOSE: Increased apoptosis was recently found in the hypertrophied left ventricle of spontaneously hypertensive rats (SHRs). Although the available evidence suggests that apoptosis can be induced in cardiac cells by various insults including pressure overload, cardiac apoptosis appears to result from an exaggerated local production of angiotensin in adult SHRs. Altered expressions of Bcl associated X (Bax), Bcl-2, chemokine receptor (CCR)-2, monocyte chemoattractant protein (MCP)-1, transforming growth factor (TGF)-ß1, phosphorylated extracellular signal-regulated kinases (PERK), and connexin 43 proteins, and kallikrein mRNA were investigated to explore the effects of losartan on the SHR model. METHODS: Twelve-week-old male rats were grouped as follows: control (C), SHR (hypertension: H), and losartan (L; SHRs were treated with losartan [10 mg/kg/day] for 5 weeks). Western blot and reverse transcription polymerase chain reaction assays were performed. RESULTS: Expression of Bax, CCR-2, MCP-1, TGF-ß1, PERK, and connexin 43 proteins, and kallikrein mRNA was significantly increased in the H group compared to that in the C group at weeks 3 and 5. Expression of Bax, CCR-2, MCP-1, TGF-ß1, and connexin 43 proteins and kallikrein mRNA was significantly decreased after losartan treatment at week 5. PERK protein expression was significantly decreased after losartan treatment at weeks 3 and 5. Bcl-2 protein expression was significantly decreased in the H group compared to that in the C group at weeks 3 and 5. CONCLUSION: Losartan treatment reduced expression of Bax, CCR-2, MCP-1, TGF-ß1, PERK, and connexin 43 proteins, and kallikrein mRNA in SHRs, along with decreased inflammation and apoptosis.

14.
Korean Circ J ; 49(1): 99-108, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30468029

RESUMO

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.

15.
Hypertens Res ; 42(6): 845-851, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30587855

RESUMO

Pediatric blood pressure (BP) reference tables are generally based on sex, age, and height and tend to be cumbersome to use in routine clinical practice. In this study, we aimed to develop a new, height-specific simple BP table according to the international child BP reference table based on sex, age and height and to evaluate its performance using international data. We validated the simple table in a derivation cohort that included 58,899 children and adolescents aged 6-17 years from surveys in 7 countries (China, India, Iran, Korea, Poland, Tunisia, and the United States) and in a validation cohort that included 70,072 participants from three other surveys (China, Poland and Seychelles). The BP cutoff values for the simple table were calculated for eight height categories for both the 90th ("elevated BP") and 95th ("high BP") percentiles of BP. The simple table had a high performance to predict high BP compared to the reference table, with high values (boys/girls) of area under the curve (0.94/0.91), sensitivity (88.5%/82.9%), specificity (99.3%/99.7%), positive predictive values (93.9%/97.3%), and negative predictive values (98.5%/97.8%) in the pooled data from 10 studies. The simple table performed similarly well for predicting elevated BP. A simple table based on height only predicts elevated BP and high BP in children and adolescents nearly as well as the international table based on sex, age, and height. This has important implications for simplifying the detection of pediatric high BP in clinical practice.

16.
Genomics Inform ; 16(2): 36-41, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30304924

RESUMO

Kawasaki disease (KD) is an acute febrile vasculitis predominately affecting infants and children. The dominant incidence age of KD is from 6 months to 5 years of age, and the incidence is unusual in those younger than 6 months and older than 5 years of age. We tried to identify genetic variants specifically associated with KD in patients younger than 6 months or older than 5 years of age. We performed an age-stratified genome-wide association study using the Illumina HumanOmni1-Quad BeadChip data (296 cases vs. 1,000 controls) and a replication study (1,360 cases vs. 3,553 controls) in the Korean population. Among 26 candidate single nucleotide polymorphisms (SNPs) tested in replication study, only a rare nonsynonymous SNP (rs4365796: c.1106C>T, p.Thr369Met) in the lymphoid enhancer binding factor 1 (LEF1) gene was very significantly associated with KD in patients younger than 6 months of age (odds ratio [OR], 3.07; pcombined = 1.10 × 10-5), whereas no association of the same SNP was observed in any other age group of KD patients. The same SNP (rs4365796) in the LEF1 gene showed the same direction of risk effect in Japanese KD patients younger than 6 months of age, although the effect was not statistically significant (OR, 1.42; p = 0.397). This result indicates that the LEF1 gene may play an important role as a susceptibility gene specifically affecting KD patients younger than 6 months of age.

17.
Korean J Pediatr ; 61(9): 271-278, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30274504

RESUMO

PURPOSE: Abnormal potassium channels expression affects vessel function, including vascular tone and proliferation rate. Diverse potassium channels, including voltage-gated potassium (Kv) channels, are involved in pathological changes of pulmonary arterial hypertension (PAH). Since the role of the Kv1.7 channel in PAH has not been previously studied, we investigated whether Kv1.7 channel expression changes in the lung tissue of a monocrotaline (MCT)-induced PAH rat model and whether this change is influenced by the endothelin (ET)-1 and reactive oxygen species (ROS) pathways. METHODS: Rats were separated into 2 groups: the control (C) group and the MCT (M) group (60 mg/kg MCT). A hemodynamic study was performed by catheterization into the external jugular vein to estimate the right ventricular pressure (RVP), and pathological changes in the lung tissue were investigated. Changes in protein and mRNA levels were confirmed by western blot and polymerase chain reaction analysis, respectively. RESULTS: MCT caused increased RVP, medial wall thickening of the pulmonary arterioles, and increased expression level of ET-1, ET receptor A, and NADPH oxidase (NOX) 4 proteins. Decreased Kv1.7 channel expression was detected in the lung tissue. Inward-rectifier channel 6.1 expression in the lung tissue also increased. We confirmed that ET-1 increased NOX4 level and decreased glutathione peroxidase-1 level in pulmonary artery smooth muscle cells (PASMCs). ET-1 increased ROS level in PASMCs. CONCLUSION: Decreased Kv1.7 channel expression might be caused by the ET-1 and ROS pathways and contributes to MCT-induced PAH.

18.
Korean J Pediatr ; 61(5): 167-173, 2018 May.
Artigo em Inglês | MEDLINE | ID: mdl-29853942

RESUMO

Purpose: Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome with many causes, including Kawasaki disease (KD). The purpose of this study was to identify the laboratory tests needed to easily differentiate KD with HLH from incomplete KD alone. Methods: We performed a retrospective study on patients diagnosed with incomplete KD and incomplete KD with HLH (HLH-KD) between January 2012 and March 2015. We compared 8 secondary HLH patients who were first diagnosed with incomplete KD with all 247 incomplete KD diagnosed patients during the study period. The complete blood count, erythrocyte sedimentation rate, platelet count, and serum total protein, albumin, triglyceride, C-reactive protein, N-terminal pro-brain natriuretic peptide (NT-proBNP), and ferritin levels were compared. Clinical characteristics and echocardiography findings were also compared between the 2 groups. Results: The total duration of fever was longer in the HLH-KD group than in the KD group. White blood cell and platelet counts were higher in the KD group. Alanine aminotransferase, ferritin, and coronary artery diameter were increased in the HLH-KD group compared with those in the KD group. The median of NT-proBNP was significantly higher in the HLH-KD group than in the KD group at 889.0 (interquartile range [IQR], 384.5-1792.0) pg/mL vs. 233.0 (IQR, 107.0-544.0) pg/mL. Conclusion: The NT-proBNP level may be helpful in distinguishing incomplete KD from KD with HLH. The NT-proBNP level should be determined in KD patients with prolonged fever, in addition to the white blood cell count, platelet count, and ferritin level, to evaluate secondary HLH.

19.
Korean J Pediatr ; 61(1): 12-16, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-29441107

RESUMO

Purpose: To differentiate adenoviral pharyngoconjunctival fever (PCF) from acute Kawasaki disease (KD) using laboratory tests before results of virus-real time polymerase chain reaction and ophthalmologic examination are obtained. Methods: Baseline patient characteristics and laboratory measurements were compared between 40 patients with adenovirus infection and 123 patients with KD. Results: The patients with adenovirus infection were generally older than those with KD (median: 3.9 years vs. 2 years, P=0.000). White blood cell and, platelet count, and aspartate aminotransferase, alanine aminotransferase, and N-terminal pro-brain natriuretic peptide (NT-proBNP) levels showed significant differences between the 2 groups, but the C-reactive protein (CRP) levels did not (6.8±3.0 mg/dL vs. 8.3±5.8 mg/dL, P=0.126). In the adenovirus infection group, the CRP levels were <1, <3, <10, and ≥10 mg/dL in 2 (5%), 3 (7.5%), 30 (75%), and 5 patients (12.5%), respectively. The cutoff NT-proBNP level was 265 pg/mL. Discrepancy was defined as CRP and NT-proBNP levels of ≥3 or <3 mg/dL, and <265 or ≥265 pg/mL, respectively. Among the 35 patients with adenovirus infection whose CRP levels were ≥3 mg/dL, 29 (82.9%) showed a discrepancy. Conversely, of the 103 patients with KD whose CRP levels were ≥3 mg/dL, 83 (80.6%) showed no discrepancy. Between the groups, a significant difference in discrepancy rate was observed (P=0.000). None of the patients with adenovirus infection had CRP and NT-proBNP levels of <3 mg/dL and ≥265 pg/mL, respectively. Conclusion: With a sensitivity of 82.9% and a specificity of 80.6%, CRP and NT-proBNP levels may differentiate between adenoviral PCF and acute KD.

SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA