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1.
Artigo em Inglês | MEDLINE | ID: mdl-38664989

RESUMO

Objective: Non-alcoholic fatty liver disease (NAFLD) is defined as chronic hepatic steatosis and is becoming prevalent along with the increasing trend of obesity in children and adolescents. A non-invasive and reliable tool is needed to differentiate non-alcoholic steatohepatitis (NASH) from simple steatosis. This study evaluates the association between the triglyceride glucose (TyG) index and the ultrasonographic fatty liver indicator (US-FLI), and the possibility of using the TyG index for prediction of severity of pediatric NAFLD. Methods: One hundred twenty one patients who were diagnosed with NAFLD by ultrasonography were included. They were categorized into 3 groups according to body mass index (BMI). Ninety two were obese, and 19 and 10 were overweight and normal weight, respectively. Results: The homeostatic model assessment for insulin resistance (HOMA-IR) was highest in the group with obesity (P=0.044). The TyG index and US-FLI did not differ significantly among the 3 BMI groups (P=0.186). Fourteen (11.6 %) of the 121 patients had US-FLI  6, in whom the BMI-SDS and TyG index were higher (P=0.017, P=0.004), whereas HOMA-IR did not differ significantly from the group with US-FLI < 6 (P=0.366). US-FLI was associated with BMI-SDS and the TyG index. TyG index was significantly associated with US-FLI after adjustment for BMI-SDS. The cut-off value for the TyG index for predicting US-FLI  6 was 8.91, with an area under the curve of 0.785. Conclusion: TyG index was associated with the degree of hepatic steatosis, suggesting that it might be a useful tool for predicting the severity of pediatric NAFLD.

2.
Sci Rep ; 13(1): 10062, 2023 06 21.
Artigo em Inglês | MEDLINE | ID: mdl-37344574

RESUMO

Two-dimensional shear wave elastography (2D-SWE) evaluates liver stiffness using a non-invasive method, but studies in the paediatric population are rare. This study evaluated the role of 2D-SWE in the diagnosis and severity of paediatric non-alcoholic fatty liver disease (NAFLD). In total, 131 patients with NAFLD and 25 healthy controls were enrolled in this study. The diagnosis and severity of NAFLD were initially assessed using the ultrasound fatty liver index (US-FLI), and all participants underwent 2D-SWE. US-FLI semi-quantitatively measures the severity of NAFLD on a scale of 2-8. The assessment of liver stiffness measurement (LSM) by 2D-SWE is presented in kilopascals (kPa). The NAFLD group was characterised by significantly higher LSM (4.40 ± 0.90 kPa) than the control group (3.76 ± 0.28 kPa) (P < 0.001). 2D-SWE significantly correlated with age, height, weight, body mass index, glucose, aspartate aminotransferase, alanine aminotransferase, high-density lipoprotein cholesterol, US-FLI, and triglyceride-glucose index (P < 0.001). In the receiver operating characteristic curve analysis, the area under the curve of LSM for predicting US-FLI ≥ 2 and ≥ 6 was 0.784 (P < 0.001) and 0.819 (P < 0.001), respectively. In conclusion, we suggest that 2D-SWE can be used as a non-invasive diagnostic tool for diagnosing and assessing the severity of paediatric NAFLD.


Assuntos
Técnicas de Imagem por Elasticidade , Hepatopatia Gordurosa não Alcoólica , Humanos , Adolescente , Criança , Hepatopatia Gordurosa não Alcoólica/diagnóstico por imagem , Hepatopatia Gordurosa não Alcoólica/patologia , Cirrose Hepática/patologia , Técnicas de Imagem por Elasticidade/métodos , Testes de Função Hepática , Curva ROC , Fígado/diagnóstico por imagem , Fígado/patologia
3.
PLoS One ; 18(2): e0280309, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36749747

RESUMO

Impulse oscillometry system (IOS) is a simple, and less invasive method for assessing small to total airway resistance in children. We analyzed the correlation between IOS, spirometry, and plethysmographic parameters performed for the diagnosis of pediatric BO patients. A total of 89 IOS assessments of pediatric BO patients or children without lung disease were included, and the relationship between pulmonary function tests (PFTs) and diagnostic performance was analyzed. R5, R5-20, X5, and AX were statistically significantly worse in the BO group. In general linear correlation analysis, R5% (adjusted ß [aß], -0.59; p < 0.001) and AX % (aß, -0.9; p < 0.001) showed the strongest correlation with conventional PFT parameters. R5% and AX % also showed the highest correlation with FEF25-75% (aß, -0.48; p < 0.001 and aß, -0.83; p < 0.001), and sRaw % (aß, -0.73; p = 0.003 and aß, -0.59; p = 0.008, respectively). Multivariate logistic regression analysis showed that R5 Z-score showed the highest ORs with FEV1 (OR = 3.94, p = 0.006), FEF25-75% (OR = 5.96, p = 0.005), and sRaw % (OR = 4.85, p = 0.022). Receiver operating curve analysis suggested AX % and R5% as the most optimal IOS parameters for BO diagnostic performance with the area under the curve of 0.915 and 0.882, respectively. In conclusion, R5 and AX are the parameters that can independently identify the severity of airway obstruction in pediatric BO patients without conventional lung function tests. IOS is an easy-to-perform, and reliable diagnostic method capable of detecting pathological obliteration of the small airways in children with BO.


Assuntos
Bronquiolite Obliterante , Pulmão , Humanos , Criança , Oscilometria/métodos , Testes de Função Respiratória/métodos , Espirometria , Volume Expiratório Forçado
4.
Front Endocrinol (Lausanne) ; 13: 1025005, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36531458

RESUMO

Background: Serum uric acid (UA) within appropriate levels is reported to be beneficial in patients with idiopathic short stature (ISS). This study aimed to evaluate the association between serum UA levels and height standard deviation scores (SDS) in patients with ISS during growth hormone (GH) therapy. Methods: A longitudinal study (LG Growth Study) of 182 children (mean age: 7.29±2.60 years) with ISS was performed. All participants were in the prepubertal stage and treated with GH, and the data within a treatment period of 30 months were analyzed. Results: In the adjusted Pearson's correlation, UA was significantly correlated with height SDS after controlling for sex, age, and body mass index (BMI) SDS (r=0.22, p=0.007). In the adjusted multiple regression analyses, the height SDS was significantly associated with UA after controlling for sex, age, and BMI SDS (ß=0.168, p=0.007). Within the 30-month treatment period, the UA levels significantly increased as the height SDS increased, and the mean UA levels at baseline and 30 months after treatment were 3.90±0.64 mg/dL and 4.71±0.77 mg/dL, respectively (p=0.007). Discussion: In conclusion, UA is related to height SDS, and GH treatment leads to a significant increase in UA without hyperuricemia. Elevated UA is considered a favorable outcome of GH therapy, and further studies are needed to determine its role as a monitoring tool.


Assuntos
Hormônio do Crescimento , Hormônio do Crescimento Humano , Humanos , Criança , Pré-Escolar , Ácido Úrico , Transtornos do Crescimento/tratamento farmacológico , Estudos Longitudinais , Estatura
5.
Genes (Basel) ; 13(6)2022 05 24.
Artigo em Inglês | MEDLINE | ID: mdl-35741700

RESUMO

Objective: The mechanism underlying postnatal growth failure and catch-up growth in small-for-gestational-age (SGA) children is poorly understood. This study investigated the exosomal miRNA signature associated with catch-up growth in SGA children. Methods: In total, 16 SGA and 10 appropriate-for-gestational-age (AGA) children were included. Serum exosomal miRNA was analyzed using next-generation sequencing (NGS). Exosomal miRNA was profiled for five SGA children with catch-up growth (SGA-CU), six SGA children without CU growth (SGA-nCU), and five AGA children. Results: Exosomal miRNA profiles were clustered into three clear groups. The exosomal miRNA expression profiles of the SGA-nCU group differed from those of the SGA-CU and AGA groups. In all, 22 miRNAs were differentially expressed between SGA-nCU and AGA, 19 between SGA-nCU and SGA-CU, and only 6 between SGA-CU and AGA. In both SGA-nCU and SGA-CU, miR-874-3p was upregulated and miR-6126 was downregulated. Therefore, these two miRNAs could serve as biomarkers for SGA. Compared with SGA-CU and AGA, miR-30c-5p, miR-363-3p, miR-29a-3p, and miR-29c-3p were upregulated in SGA-nCU, while miR-629-5p and miR-23a-5p were downregulated. These six miRNAs could be associated with growth failure in SGA-nCU children. Conclusions: SGA children without CU have a distinct exosomal miRNA expression profile compared with AGA and SGA children with CU. Exosomal miRNAs could serve as novel biomarkers for CU.


Assuntos
MicroRNAs , Biomarcadores/metabolismo , Criança , Feminino , Retardo do Crescimento Fetal , Humanos , Recém-Nascido , Recém-Nascido Pequeno para a Idade Gestacional/metabolismo , MicroRNAs/genética
6.
Pediatr Int ; 64(1): e15016, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-34606653

RESUMO

BACKGROUND: With the coronavirus disease 2019 (COVID-19) pandemic lasting for more than a year, it is imperative to identify the associated changes in the use of emergency medical care for efficient operation of the pediatric emergency department (PED). This study was conducted to determine the long-term impact of the COVID-19 pandemic on patterns of PED visits. METHODS: This is a retrospective observational study of visits to the PED of six hospitals, between January 1, 2017, and December 31, 2020. We compared changes in the characteristics of patients before and during the COVID-19 pandemic. RESULTS: A total of 245 022 visits were included in this analysis. After the first case of COVID-19 was reported in Korea, we observed a significant decrease (54.2%) in PED visits compared with the annual average number of visits in the previous 3 years. Since then, the weekly number of PED visits decreased by 11.9 person/week (95% CI: -15.3--8.4, P < 0.001), which included an increase of 0.21% (95% CI: 0.15%-0.26%, P < 0.001) per week in high acuity patients. From 2017 to 2020, the proportion of infectious respiratory diseases by year was 25.9%, 27.0%, 28.6%, and 16.3%, respectively, demonstrating a significant decrease in 2020 (P < 0.001). CONCLUSIONS: During the COVID-19 pandemic, the number of patient visits to PEDs continues to decline, especially among those with infectious diseases. However, the disease severity of patients has gradually increased. There has been a change in the characteristics of visits to PEDs after COVID-19 which will require an appropriate response from a long-term perspective.


Assuntos
COVID-19 , COVID-19/epidemiologia , Criança , Serviço Hospitalar de Emergência , Hospitais Pediátricos , Humanos , Pandemias , Estudos Retrospectivos , SARS-CoV-2
7.
Pediatr Int ; 64(1): e15003, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-34582093

RESUMO

BACKGROUND: Auscultation is an easy way to evaluate and diagnose patients with lung conditions but has the shortcoming of being subjective. Using the spectrogram, it is possible to visualize wheezing. We therefore conducted a study to compare the efficacy of diagnosing wheezing by auscultation versus diagnosing wheezing by spectrogram. METHODS: This was an investigation of interrater reliability and agreement in which the subject population consisted of children, and the rater population consisted of pediatric pulmonologists. We recorded 55 respiratory sound files from June to November 2019. Three pediatric pulmonologists listened to the respiratory sound files and assessed whether wheezing was present. All respiratory sound files were also converted into spectrograms; the same pulmonologists viewed these and assessed whether wheezing was present. We tested for interrater reliability and agreement between the auscultation results and spectrographic results and investigated the diagnostic reliability of auscultation versus spectrogram. RESULTS: Agreement among the three raters of our auscultation respiratory recordings was 88% and reliability was good (κ = 0.76, P < 0.001). Agreement among the three raters of our spectrograms was 83% and reliability was good (κ =0.66, P < 0.001). The level of agreement between each rater's spectrographic findings and diagnosed wheezing was 91%, 75%, and 93%, respectively. Reliability was accordingly very good, moderate, and very good (κ = 0.82, 0.49, 0.85, P < 0.001), respectively. CONCLUSIONS: A spectrogram may be a valuable tool for evaluating wheezing in children. It may also be used to improve a young clinician's ability to accurately diagnose wheezing in the future.


Assuntos
Auscultação , Sons Respiratórios , Humanos , Criança , Sons Respiratórios/diagnóstico , Reprodutibilidade dos Testes , Auscultação/métodos
8.
J Clin Res Pediatr Endocrinol ; 14(4): 481-484, 2022 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-34210114

RESUMO

ACAN variants can manifest as various clinical features, including short stature, advanced bone age (BA), and skeletal defects. Here, we report rare clinical manifestations of ACAN defects in a 9 year, 5 month-old girl born small for gestational age (SGA), who presented with short stature, and was initially diagnosed with idiopathic growth hormone deficiency. She displayed several dysmorphic features, including genu valgum, cubitus valgus, and recurrent patellar dislocations. She presented with progressive advancement of BA compared with chronological age. Whole exome sequencing confirmed the presence of a novel heterozygous nonsense variant, c.1968C>G, p.(Tyr656*), in ACAN. ACAN variants should be considered in short stature patients born SGA with joint problems, particularly those with recurrent patellar dislocation and genu valgum.


Assuntos
Nanismo , Geno Valgo , Doenças do Recém-Nascido , Recém-Nascido , Feminino , Humanos , Lactente , Agrecanas/genética , Idade Gestacional , Heterozigoto
9.
Endocr J ; 69(5): 559-565, 2022 May 30.
Artigo em Inglês | MEDLINE | ID: mdl-34924455

RESUMO

The triglyceride-glucose (TyG) index is associated with predicting type 2 diabetes mellitus (T2DM), but its relationship with homeostatic model assessment of insulin resistance (HOMA-IR) in T2DM is not established. We aimed to investigate the role of TyG index for detection of T2DM in children and adolescents and compare it with HOMA-IR. A cross sectional study was performed in 176 overweight or obese children and adolescents with mean age of 11.34 ± 3.24 years. TyG index was calculated as ln (fasting triglyceride (TG) [mg/dL] × fasting glucose [mg/dL]/2). Of a total of 176 subjects, 57 (32%) were diagnosed with T2DM. Significant differences were observed in the TyG index between T2DM and non-T2DM (p < 0.001). The TyG index had a positive correlation with fasting glucose (r = 0.519, p < 0.001), HOMA-IR (r = 0.189, p < 0.017), HbA1c (r = 0.429, p < 0.001), total cholesterol (TC) (r = 0.257, p = 0.001), TG (r = 0.759, p < 0.001), and low-density lipoprotein cholesterol (LDL-C)(r = 0.152, p < 0.001), and a negative correlation with high-density lipoprotein cholesterol (HDL-C)(r = -0.107, p < 0.001) after controlling for sex, age and BMI standard deviation scores (SDS). In multiple regression analyses, 91.8% of the variance in TyG index was explained by age, glucose, HOMA-IR, TG, LDL-C, and HDL-C (p < 0.001). In the receiver operating characteristic (ROC) analysis, the TyG index [area under the curve (AUC) 0.839)] showed a better performance compared to HOMA-IR (AUC 0.645) in identifying patients with T2DM (p < 0.001). In conclusion, the TyG index had significant association with insulin resistance in T2DM and was superior to HOMA-IR in predicting T2DM in children and adolescents.


Assuntos
Diabetes Mellitus Tipo 2 , Resistência à Insulina , Obesidade Infantil , Adolescente , Biomarcadores , Glicemia/análise , Criança , HDL-Colesterol , LDL-Colesterol , Estudos Transversais , Diabetes Mellitus Tipo 2/complicações , Diabetes Mellitus Tipo 2/diagnóstico , Glucose , Humanos , Obesidade Infantil/complicações , Obesidade Infantil/diagnóstico , Triglicerídeos
10.
Sci Rep ; 11(1): 23660, 2021 12 08.
Artigo em Inglês | MEDLINE | ID: mdl-34880367

RESUMO

The purpose of this study was to present age- and sex-specific distributions of the triglyceride-glucose (TyG) index and to evaluate their relationship with cardiometabolic risk factors in children and adolescents. A total of 7404 participants aged 10-18 years from the Korean National Health and Nutrition Survey were included as the reference population. The TyG index was calculated as ln(fasting triglyceride [mg/dL] × fasting glucose [mg/dL]/2). The percentile of the TyG index exhibited a steady linear relationship with age for both sexes. TyG index significantly correlated with waist circumference (WC) standard deviation score (SDS; r = 0.110, p < 0.001), systolic blood pressure (SBP; r = 0.104, p < 0.001), diastolic blood pressure (DBP; r = 0.083, p < 0.001), glucose (r = 0.220, p < 0.001), high-density lipoprotein cholesterol (HDL-C; r = - 0.325, p < 0.001), and triglycerides (TG; r = 0.926, p < 0.001). Multiple linear regression analysis revealed that the TyG index was significantly associated with WC SDS (ß = 0.116, p < 0.001), SBP (ß = 2.009, p < 0.001), DBP (ß = 1.464, p < 0.001), glucose (ß = 3.376, p < 0.001), HDL-C (ß = - 6.431, p < 0.001), and TG (ß = 85.518, p < 0.001). Our results suggest that the TyG index has a steady linear distribution for sex and age in children and adolescents and constitutes an indicator for predicting metabolic disorders that could lead to cardiovascular disease later in life.


Assuntos
Glicemia/metabolismo , Fatores de Risco Cardiometabólico , Triglicerídeos/sangue , Adolescente , Pressão Sanguínea , Criança , Fatores de Confusão Epidemiológicos , Feminino , Humanos , Masculino , Inquéritos Nutricionais , Circunferência da Cintura
11.
Micromachines (Basel) ; 12(7)2021 Jul 14.
Artigo em Inglês | MEDLINE | ID: mdl-34357235

RESUMO

Airborne metal particles (MPs; particle size > 10 µm) in workplaces result in a loss in production yield if not detected in time. The demand for compact and cost-efficient MP sensors to monitor airborne MP generation is increasing. However, contemporary instruments and laboratory-grade sensors exhibit certain limitations in real-time and on-site monitoring of airborne MPs. This paper presents a microfluidic MP detection chip to address these limitations. By combining the proposed system with microcirculation-based particle-to-liquid collection and a capacitive sensing method, the continuous detection of airborne MPs can be achieved. A few microfabrication processes were realized, resulting in a compact system, which can be easily replaced after contamination with a low-priced microfluidic chip. In our experiments, the frequency-dependent capacitive changes were characterized using MP (aluminum) samples (sizes ranging from 10 µm to 40 µm). Performance evaluation of the proposed system under test-bed conditions indicated that it is capable of real-time and continuous monitoring of airborne MPs (minimum size 10 µm) under an optimal frequency, with superior sensitivity and responsivity. Therefore, the proposed system can be used as an on-site MP sensor for unexpected airborne MP generation in precise manufacturing facilities where metal sources are used.

12.
Sci Rep ; 11(1): 9524, 2021 05 04.
Artigo em Inglês | MEDLINE | ID: mdl-33947900

RESUMO

This study was performed to evaluate the waist-to-height ratio (WHtR) distribution and assess its relationship with cardiometabolic risk in children and adolescents. A total of 8091 subjects aged 10-18 years were included from a nationally representative survey. Participants were classified into three groups: (1) < 85th, (2) ≥ 85th and < 95th, and (3) ≥ 95th percentile of WHtR. The WHtR distribution varied with sex and age. Whereas WHtR decreased from age 10-15 years in boys and from age 10-12 years in girls, it slightly increased thereafter. Compared to the < 85th percentile group, the WHtR ≥ 85th and < 95th percentile group had an odds ratio (OR) of 1.2 for elevated blood pressure (BP), 1.89 for elevated triglycerides (TGs), 1.47 for reduced high-density lipoprotein cholesterol (HDL-C) and 4.82 for metabolic syndrome (MetS). The ≥ 95th percentile group had an OR of 1.4 for elevated BP, 2.54 for elevated glucose, 2.22 for elevated TGs, 1.74 for reduced HDL-C, and 9.45 for MetS compared to the < 85th percentile group. Our results suggest that sex- and age-specific WHtR percentiles can be used as a simple clinical measurement to estimate cardiometabolic risk.


Assuntos
Estatura/fisiologia , Doenças Cardiovasculares/etiologia , Doenças Cardiovasculares/fisiopatologia , Circunferência da Cintura/fisiologia , Adolescente , Pressão Sanguínea/fisiologia , Índice de Massa Corporal , Doenças Cardiovasculares/metabolismo , Criança , HDL-Colesterol/metabolismo , Feminino , Humanos , Hipertensão/metabolismo , Hipertensão/fisiopatologia , Masculino , Síndrome Metabólica/complicações , Síndrome Metabólica/metabolismo , Síndrome Metabólica/fisiopatologia , Obesidade Abdominal/complicações , Obesidade Abdominal/metabolismo , Obesidade Abdominal/fisiopatologia , Fatores de Risco , Triglicerídeos/metabolismo , Razão Cintura-Estatura
13.
Bone Marrow Transplant ; 56(8): 1929-1936, 2021 08.
Artigo em Inglês | MEDLINE | ID: mdl-33782549

RESUMO

This study aimed to evaluate the pretransplant diffusing capacity as a predictor of outcomes in pediatric allogeneic hematopoietic stem cell transplantation (allo-HSCT). Retrospective cohort study of 176 children followed outcomes for 5 years after allo-HSCT. We conducted an analysis of PFTs include spirometry, body plethysmography, and diffusing capacity prior to allo-HSCT. We analyzed the probabilities of overall survival (OS), disease-related mortality (DRM), and non-relapse mortality (NRM). Of all carbon monoxide diffusing capacity (DLCO) parameters obtained using the Global Lung Function Initiative (GLI)-2017, univariate analysis showed that the grade 3, 4 of DLCOadj and Category III, IV of LFS significantly increase NRM (p = 0.003 and p = 0.008). Multivariate analysis indicated that a significant increase in the risk of NRM is associated with grades 3, 4 DLCOadj (hazard ratio [HR] = 4.90, p = 0.020). Kaplan-Meier analyses showed that a significant stepwise increase in NRM was observed with both worse pretransplant DLCOadj grades and LFS categories (p < 0.001 and p = 0.003). A compromised pretransplant diffusing capacity and a high LFS significantly increase the risk of NRM. Especially, DLCOadj before transplantation can be used as an important predictor of NRM after allo-HSCT in children with malignancy.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Recidiva Local de Neoplasia , Criança , Humanos , Estimativa de Kaplan-Meier , Estudos Retrospectivos , Transplante Homólogo
14.
Endocr J ; 68(8): 889-895, 2021 Aug 28.
Artigo em Inglês | MEDLINE | ID: mdl-33762520

RESUMO

This study aimed to investigate the relationships between genetic polymorphisms of leptin/receptor genes and clinical/biochemical characteristics in children with growth hormone deficiency (GHD). Ninety-three GHD children and 69 age-matched normal controls were enrolled. Anthropometric measurements, bone age, and laboratory test results were obtained. Polymorphisms in the LEP gene promoter locus (LEP-2548, rs7799039) and LEPR genes (K109R, rs1137100 and Q223R, rs1137101) were analyzed using PCR-RFLP. The serum leptin levels were measured using an ELISA kit. The median height and BMI z-scores of all GHD subjects were -2.20 and -0.26, respectively, and those of normal controls were -0.30 and -0.13, respectively. The serum leptin levels were similar between GHD subjects and normal controls (p = 0.537), but those were different between the complete GHD (6.97 ng/mL) and partial GHD (4.22 ng/mL) groups (p = 0.047). There were no differences in the genotypic distributions of LEP-2548, LEPR K109R, and Q223R between GHD subjects and normal controls. However, GHD subjects with the G allele at LEP-2548 showed higher IGF-1 (p = 0.047) and IGFBP-3 SDSs (p = 0.027) than GHD subjects with the A allele. GHD subjects with the G allele at LEPR Q223R showed lower stimulated GH levels (p = 0.023) and greater height gain after 1 year of GH treatment (p = 0.034) than GHD subjects with the A allele. In conclusion, leptin/leptin receptor genes are suggested to have the role of growth-related factors, which can affect various growth responses in children who share the same disease entity.


Assuntos
Transtornos do Crescimento/tratamento farmacológico , Terapia de Reposição Hormonal , Hormônio do Crescimento Humano/uso terapêutico , Leptina/genética , Polimorfismo de Nucleotídeo Único , Receptores para Leptina/genética , Adolescente , Alelos , Estatura/efeitos dos fármacos , Criança , Pré-Escolar , Feminino , Frequência do Gene , Genótipo , Transtornos do Crescimento/genética , Hormônio do Crescimento Humano/administração & dosagem , Humanos , Proteína 3 de Ligação a Fator de Crescimento Semelhante à Insulina/genética , Fator de Crescimento Insulin-Like I/genética , Leptina/sangue , Masculino , Resultado do Tratamento
15.
J Pediatr Endocrinol Metab ; 34(2): 255-259, 2021 Feb 23.
Artigo em Inglês | MEDLINE | ID: mdl-33544498

RESUMO

OBJECTIVES: Defects in the IGF-1 receptor gene (IGF1R) induce IGF-1 resistance, characterized by intrauterine and postnatal growth retardation, normal or elevated serum IGF-1 levels, and feeding problems. Obesity, idiopathic growth hormone deficiency (IGHD), bone age advancement, and serum IGF-1 level in the lower half of the reference range are very rare clinical features in patients with IGF1R defects. CASE PRESENTATION: In this study, we report the atypical clinical manifestations of IGF1R defects. Short stature girl born small for gestational age were initially diagnosed with IGHD. No catch-up growth was achieved despite sufficiently elevated IGF-1 levels after recombinant human growth hormone (rhGH) treatment. Single nucleotide polymorphism microarray analysis finally confirmed terminal deletion of 15q26.2q26.3 in the subject. CONCLUSION: Intrauterine growth retardation, postnatal growth failure, and IGF-1 resistance during rhGH treatment are homologous features exhibited by affected patients, and may be predictive of IGF1R defects.


Assuntos
Nanismo/patologia , Deleção de Genes , Obesidade/patologia , Receptor IGF Tipo 1/genética , Criança , Nanismo/complicações , Nanismo/genética , Feminino , Humanos , Masculino , Obesidade/complicações , Obesidade/genética , Linhagem , Prognóstico
16.
Sci Rep ; 11(1): 1263, 2021 01 13.
Artigo em Inglês | MEDLINE | ID: mdl-33441845

RESUMO

The aim of this study was to develop a predictive model of pediatric mortality in the early stages of intensive care unit (ICU) admission using machine learning. Patients less than 18 years old who were admitted to ICUs at four tertiary referral hospitals were enrolled. Three hospitals were designated as the derivation cohort for machine learning model development and internal validation, and the other hospital was designated as the validation cohort for external validation. We developed a random forest (RF) model that predicts pediatric mortality within 72 h of ICU admission, evaluated its performance, and compared it with the Pediatric Index of Mortality 3 (PIM 3). The area under the receiver operating characteristic curve (AUROC) of RF model was 0.942 (95% confidence interval [CI] = 0.912-0.972) in the derivation cohort and 0.906 (95% CI = 0.900-0.912) in the validation cohort. In contrast, the AUROC of PIM 3 was 0.892 (95% CI = 0.878-0.906) in the derivation cohort and 0.845 (95% CI = 0.817-0.873) in the validation cohort. The RF model in our study showed improved predictive performance in terms of both internal and external validation and was superior even when compared to PIM 3.


Assuntos
Mortalidade da Criança , Mortalidade Hospitalar , Mortalidade Infantil , Unidades de Terapia Intensiva Pediátrica , Aprendizado de Máquina , Modelos Biológicos , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Admissão do Paciente , Estudos Retrospectivos
17.
J Pediatr Hematol Oncol ; 43(1): e90-e94, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-32427706

RESUMO

Obstructive lung disease (OLD) that develops after hematopoietic stem cell transplantation (HSCT) has a significant impact on morbidity and mortality. We investigated the role of pulmonary function tests (PFTs) in the prediction of prognosis of OLD in children who have undergone HSCT. We retrospectively reviewed 538 patients who underwent allogenic HSCT in the Department of Pediatrics, Seoul St. Mary's Hospital, South Korea, from April 2009 to July 2017. OLD was identified on PFTs or chest computed tomography scans obtained from 3 months after HSCT onwards. OLD developed after HSCT in 46 patients (28 male individuals, median age: 11.2 y). The group that developed OLD with an unfavorable prognosis (n=23) had a lower forced vital capacity (FVC) (% of predicted, 78.53±24.00 vs. 97.71±16.96, P=0.01), forced expiratory volume in 1 second (FEV1) (% of predicted, 52.54±31.77 vs. 84.44±18.59, P=0.00), FEV1/FVC (%, 59.28±18.68 vs. 79.94±9.77, P=0.00), and forced expiratory flow at 25% to 75% of forced vital capacity (FEF25-75) (% of predicted, 30.95±39.92 vs. 57.82±25.71, P=0.00) at diagnosis than the group that developed OLD with a favorable prognosis (n=23). The group that developed OLD with an unfavorable prognosis had significant reductions in FVC, FEV1, FEV1/FVC, and FEF25-75 at 2 years after diagnosis. Children who develop OLD with an unfavorable prognosis after HSCT already have poor lung function at the time of diagnosis. Additional treatment should be considered in patients who develop OLD after HSCT according to their PFTs at diagnosis.


Assuntos
Neoplasias Hematológicas/terapia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Pneumopatias Obstrutivas/mortalidade , Pulmão/fisiopatologia , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Volume Expiratório Forçado , Neoplasias Hematológicas/patologia , Humanos , Pneumopatias Obstrutivas/diagnóstico , Pneumopatias Obstrutivas/etiologia , Masculino , Prognóstico , Testes de Função Respiratória , Estudos Retrospectivos , Taxa de Sobrevida , Capacidade Vital
18.
J Pediatr Endocrinol Metab ; 34(1): 59-63, 2021 Jan 27.
Artigo em Inglês | MEDLINE | ID: mdl-33180049

RESUMO

OBJECTIVES: Makorin ring finger protein 3 (MKRN3) is associated with the initiation of puberty, and loss of function mutation of MKRN3 is the most common genetic cause of central precocious puberty (CPP). A recent study reported that MKRN3 interacts with and suppresses neural pentraxin-1 precursor (NPTX1) activity via polyubiquitination during early puberty in the mouse hypothalamus. This study investigated the correlation between serum NPTX1 and MKRN3 in CPP girls and predicted the potential role of NPTX1 in pubertal progression. METHODS: In this case-control study, we examined 34 girls diagnosed with CPP and 34 healthy prepubertal girls. Anthropometric and hormonal parameters were measured and serum levels of NPTX1 and MKRN3 were evaluated with commercial enzyme-linked immunosorbent assay kits. RESULTS: Serum MKRN3 level decreased significantly in CPP patients compared to controls (344.48 ± 333.77 and 1295.21 ± 780.80 pg/mL, respectively, p<0.001). Serum MKRN3 tended to decrease as Tanner breast stage increased. However, no significant difference was observed in serum NPTX1 levels between patients and controls (20.14 ± 31.75 ng/mL and 12.93 ± 8.28 ng/mL, respectively, p=0.248). The serum level of NPTX1 did not change significantly with the Tanner breast stage. Serum NPTX1 was correlated with the height standard deviation score (r=0.255; p<0.05), but was not correlated with serum MKRN3 level or the others. Conclusion: Although serum NPTX1 level was independent of serum MKRN3 level, the possibility they might be involved in the progression of puberty or CPP remains. Further research is needed to determine their role in the hypothalamus.


Assuntos
Biomarcadores/sangue , Proteínas do Tecido Nervoso/sangue , Puberdade Precoce/epidemiologia , Ubiquitina-Proteína Ligases/sangue , Proteína C-Reativa , Estudos de Casos e Controles , Criança , Feminino , Seguimentos , Humanos , Mutação , Prognóstico , Puberdade Precoce/sangue , Puberdade Precoce/patologia , República da Coreia/epidemiologia
19.
J Microbiol Immunol Infect ; 53(4): 640-646, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30424947

RESUMO

BACKGROUND/PURPOSE: Differences exist among racial and ethnic groups in the prevalence and severity of allergic diseases. However, influence of population admixing on allergic disease has not been studied. We examined the effect of population admixing on the occurrence of allergic disease. METHODS: We reviewed the data of 68,043 adolescents who participated in the 11th Korea Youth Risk Behavior Web-based Survey, which provides a sample that is representative of the entire Korean middle school and high school student population. Multi-ethnic status was determined by using parental country of birth and prevalence of asthma, allergic rhinitis (AR), and atopic dermatitis (AD) was determined by questionnaire. RESULTS: Multi-ethnic adolescents accounted for approximately 0.9% of the total adolescents. Prevalence of asthma was significantly higher in multi-ethnic group than non multi-ethnic group while that of AR and AD was significantly higher in non multi-ethnic group than multi-ethnic group. Parental region of country at birth showed a significant difference in prevalence of allergic disease. Univariate analysis found that urbanity, perceived economic status (PES), parental region of country at birth, and environmental tobacco smoke (ETS) showed a significant odds ratio (OR) in asthma, AR, and AD. Body mass index (BMI) showed a significant OR in asthma and AD. After adjusting for urbanity, PES, BMI and ETS, multiethnicity showed significantly lower OR in AR and AD. CONCLUSION: Population admixing appears to have significant effect on the prevalence of allergic disease. Further study will be needed to clarify the effect of population admixing on prevalence of allergic disease.


Assuntos
Asma/epidemiologia , Dermatite Atópica/epidemiologia , Etnicidade , Hipersensibilidade , Rinite Alérgica/epidemiologia , Adolescente , Asma/etnologia , Dermatite Atópica/etnologia , Feminino , Humanos , Hipersensibilidade/epidemiologia , Hipersensibilidade/etnologia , Masculino , Razão de Chances , Prevalência , República da Coreia/epidemiologia , Rinite Alérgica/etnologia , Fatores de Risco , Inquéritos e Questionários
20.
J Pediatr Hematol Oncol ; 42(4): 266-270, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-31599854

RESUMO

OBJECTIVE: This study aimed to determine prognostic factors associated with mortality in pediatric oncology patients admitted to the intensive care unit (ICU) with pulmonary complications. MATERIALS AND METHODS: This retrospective cohort study included patients 21 years of age with underlying oncologic diseases admitted to the ICU of a Korean Tertiary Referral Hospital with pulmonary complications from April 2009 to March 2017. Patients admitted for perioperative management or nonpulmonary complications were excluded. Demographic, laboratory, and clinical parameters (eg, Glasgow Coma Scale [GCS], pediatric Sequential Organ Failure Assessment [pSOFA], and Pediatric Logistic Organ Dysfunction [PELOD] scores) were reviewed. RESULTS: Overall, 110 patients (62 male, 56.3%) with a median age of 13 years (interquartile range: 8 to 16 y) were studied. The median ICU stay was 8 days (interquartile range: 4.25 to 16 d). Forty-five (40.9%) patients required mechanical ventilation. The overall mortality rate was 59.1% (65/110 patients). A multivariate logistic regression identified a low GCS score, peripheral oxygen saturation/fraction of inspired oxygen ratio, and hematocrit and increased total bilirubin as significantly associated with increased mortality. The pSOFA and PELOD scores on days 1 and 3 postadmission predicted in-ICU mortality, with corresponding areas under the curve of 0.80/0.76 and 0.87/0.83, respectively. CONCLUSION: Several clinical scores and factors may predict mortality in pediatric oncology patients with pulmonary complications.


Assuntos
Mortalidade Hospitalar , Unidades de Terapia Intensiva , Tempo de Internação , Pneumopatias , Neoplasias , Adolescente , Criança , Intervalo Livre de Doença , Feminino , Humanos , Pneumopatias/etiologia , Pneumopatias/mortalidade , Pneumopatias/terapia , Masculino , Neoplasias/mortalidade , Neoplasias/terapia , Valor Preditivo dos Testes , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida
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