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1.
Asia Pacific Allergy ; (4): 9-2020.
Artigo em Inglês | WPRIM (Pacífico Ocidental) | ID: wprim-785456

RESUMO

BACKGROUND: Recently, the prevalence of food allergies during childhood is increasing, with fruits being common allergens. However, data on allergens that cause fruit and vegetable allergies and pollen-food allergy syndrome (PFAS) in childhood are relatively few. This study aimed to examine the allergens in fruit and vegetable allergies in pediatric patients and to determine the association between fruit and vegetable allergies and PFAS.OBJECTIVE: This study aimed to examine the current status of fruit and vegetable allergies in Japanese children.METHODS: This was a multicenter case series observational study. The participants included children aged <15 years who developed allergic symptoms after eating fruits and vegetables and subsequently received treatment in the Pediatric Department of 6 hospitals in the Osaka Prefecture in Japan during the study period from August 2016 to July 2017. Participants' information was obtained using a questionnaire, and data were obtained by performing several types of allergy tests using blood samples.RESULTS: A total of 97 children (median age, 9 years; 56 males) were included in the study. Apple was the most common allergen, followed by peach, kiwi, cantaloupe, and watermelon. A total of 74 participants (76%) exhibited allergic symptoms due to PFAS; moreover, pathogenesis-related protein-10 (PR-10) was the most common allergen superfamily. On the contrary, in the group where neither PR-10 nor profilin was sensitized, kiwi and banana were the most common allergens, and the age of onset was lower than that in the PFAS group. Specific antibody titer was significantly associated with Birch for Bet v1 and latex for Bet v2 (r = 0.99 and r = 0.89).CONCLUSION: When we examine patients with fruit and vegetable allergies, we should first consider PFAS even in childhood specifically for children greater than 4 years old.


Assuntos
Idade de Início , Alérgenos , Grupo com Ancestrais do Continente Asiático , Betula , Criança , Citrullus , Estudo Clínico , Cucumis melo , Ingestão de Alimentos , Hipersensibilidade Alimentar , Frutas , Humanos , Hipersensibilidade , Japão , Látex , Musa , Estudo Observacional , Prevalência , Profilinas , Prunus persica , Rinite , Rinite Alérgica Sazonal , Verduras
2.
Artigo em Chinês | WPRIM (Pacífico Ocidental) | ID: wprim-781704

RESUMO

At present, non-standard use of antibiotics remains a common phenomenon in the treatment of preterm infants with early-onset sepsis (EOS) in China. The expert panel of neonatologists in Hunan Province formulated a consensus on the diagnosis and use of antibiotics for EOS in preterm infant [Chin J Contemp Pediatr, 2020, 22(1): 1-6], which has a positive effect on the rational use of antibiotics. Based on this consensus, this article points out that in order to use antibiotics accurately, it is necessary to accurately identify EOS in preterm infants, accurately understand their clinical manifestations and medical history, and accurately evaluate the laboratory test results. Also, this article offers suggestions for the use of antibiotics in preterm infants with EOS.


Assuntos
Idade de Início , Antibacterianos , China , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Doenças do Prematuro , Fatores de Risco , Sepse , Tratamento Farmacológico
3.
J. pediatr. (Rio J.) ; 95(5): 593-599, Sept.-Oct. 2019. tab, graf
Artigo em Inglês | LILACS (Américas) | ID: biblio-1040360

RESUMO

Abstract Objective: The purpose of this study was to illustrate the association between vascular endothelial growth factor level and pulmonary artery hypertension in children with β-thalassemia major. Method: This case-control study was conducted on 116 children with β-thalassemia major; 58 of them had pulmonary artery hypertension. They were compared to 58 healthy children who were age and sex-matched (control group). Serum levels of vascular endothelial growth factor and echocardiographic assessment were done for all children. Results: Vascular endothelial growth factor serum level was significantly higher in children with β-thalassemia major with pulmonary artery hypertension than in those without pulmonary artery hypertension, as well as in control groups (p < 0.001). Vascular endothelial growth factor serum level had a significant positive correlation with pulmonary artery pressure and serum ferritin, as well as a significant negative correlation with the duration of chelation therapy. Logistic regression analysis revealed that elevated vascular endothelial growth factor (Odd Ratio = 1.5; 95% Confidence Interval, 1.137-2.065; p = 0.005) was an independent risk factor of pulmonary artery hypertension in such children. Vascular endothelial growth factor serum level at a cutoff point of >169 pg/mL had 93.1% sensitivity and 93.1% specificity for the presence of pulmonary artery hypertension in children with β-thalassemia major. Conclusion: Elevated vascular endothelial growth factor serum level is associated with pulmonary artery hypertension in children with β-thalassemia.


Resumo: Objetivo: A finalidade deste estudo foi exemplificar a associação entre o nível de fator de crescimento endotelial vascular e a hipertensão arterial pulmonar em crianças com talassemia beta maior. Método: Este estudo caso-controle foi realizado em 116 crianças com talassemia beta maior; 58 das quais apresentaram hipertensão arterial pulmonar em comparação com 58 crianças saudáveis pareadas por idade e sexo (grupo de controle). Os níveis séricos do fator de crescimento endotelial vascular e a avaliação ecocardiográfica foram realizados em todas as crianças. Resultados: O nível sérico do fator de crescimento endotelial vascular foi significativamente maior em crianças com talassemia beta maior com hipertensão arterial pulmonar que as crianças sem hipertensão arterial pulmonar e os grupos de controle (p < 0,001). O nível sérico do fator de crescimento endotelial vascular apresentou uma correlação positiva significativa com a pressão arterial pulmonar e a ferritina sérica e correlação negativa significativa com a duração da terapia de quelação. A análise de regressão logística revelou que o fator de crescimento endotelial vascular elevado (RC = 1,5; IC de 95%: 1,137-2,065; p = 0,005) foi um fator de risco independente de hipertensão arterial pulmonar nessas crianças. O nível sérico do fator de crescimento endotelial vascular no ponto de corte > 169 (pg/mL) apresentou 93,1% de sensibilidade e 93,1% de especificidade na presença de hipertensão arterial pulmonar em crianças com talassemia beta maior. Conclusão: O nível sérico do fator de crescimento endotelial vascular elevado está associado à hipertensão arterial pulmonar em crianças com talassemia beta.


Assuntos
Humanos , Masculino , Feminino , Criança , Adolescente , Talassemia beta/sangue , Fator A de Crescimento do Endotélio Vascular/sangue , Hipertensão Pulmonar/sangue , Valores de Referência , Esplenectomia , Fatores de Tempo , Ecocardiografia Doppler , Estudos de Casos e Controles , Fatores de Risco , Curva ROC , Análise de Variância , Talassemia beta/fisiopatologia , Idade de Início , Estatísticas não Paramétricas , Hipertensão Pulmonar/fisiopatologia
4.
Trends psychiatry psychother. (Impr.) ; 41(3): 292-296, July-Sept. 2019. tab
Artigo em Inglês | LILACS (Américas) | ID: biblio-1043529

RESUMO

Abstract Objectives To assess the sociodemographic, psychiatric and criminal profile of adolescent offenders complying with temporary custody for homicide/homicide attempt and to compare it to that of the population of adolescents in custody for other crimes. Methods This cross-sectional study was based on the review of the medical records of 74 juvenile offenders in temporary custody at socioeducational agency Fundação de Atendimento Sócio-Educativo do Rio Grande do Sul. For the analysis, variables that presented p < 0.2 were included in multivariate adjustment through logistic regression. Results The sample comprised males only, mostly with white skin color (55.6 vs. 57.9% for homicidal and non-homicidal, respectively) and with a high prevalence of school failure (77.8 vs. 91.2%). There was a high prevalence of family history of delinquency (88 vs. 81%). Only years of study and belonging or not to a criminal organization remained statistically significant in the multivariate model. Conclusion The results show that having fewer years of study and denying belonging to a criminal organization are predictive factors of homicidal behavior in adolescent offenders (both with statistical relevance). The other variables were not statistically significant for this outcome. The present study may serve as a basis for further research, which may improve our understanding of risk factors for juvenile homicide.


Resumo Objetivos Avaliar o perfil sociodemográfico, psiquiátrico e criminal de adolescentes infratores que cumprem internação provisória por homicídio ou tentativa de homicídio e compará-los aos adolescentes privados de liberdade por outros atos infracionais. Métodos Este estudo transversal baseou-se na revisão dos prontuários médicos de 74 adolescentes infratores em internação provisória na Fundação de Atendimento Sócio-Educativo do Rio Grande do Sul. Para a análise, variáveis que apresentaram p <0,2 foram incluídas no ajuste multivariado por meio de regressão logística. Resultados A amostra foi composta apenas por homens, a maioria de pele branca (55,6 versus 57,9% para homicidas e não-homicidas, respectivamente) e com alta prevalência de reprovações escolares (77,8 vs. 91,2%). Houve alta prevalência de antecedentes familiares de delinquência (88 versus 81%). Apenas anos de estudo e pertencimento ou não a uma organização criminosa permaneceram estatisticamente significantes no modelo multivariado. Conclusão Os resultados mostram que ter menos anos de estudo e negar pertencer a uma organização criminosa foram fatores preditivos de comportamento homicida em adolescentes infratores (ambos com relevância estatística). As demais variáveis não foram estatisticamente significativas para esse desfecho. O presente estudo pode servir como base para futuras pesquisas, o que pode melhorar nossa compreensão dos fatores de risco para o homicídio juvenil.


Assuntos
Humanos , Masculino , Adolescente , Criminosos/psicologia , Homicídio/psicologia , Controle Social Formal , Brasil , Estudos Transversais , Idade de Início , Transtorno da Conduta/psicologia , Transtorno da Conduta/epidemiologia , Criminosos/legislação & jurisprudência , Criminosos/estatística & dados numéricos , Homicídio/legislação & jurisprudência , Homicídio/estatística & dados numéricos , Delinquência Juvenil/legislação & jurisprudência , Delinquência Juvenil/psicologia , Delinquência Juvenil/estatística & dados numéricos
6.
Artigo em Coreano | WPRIM (Pacífico Ocidental) | ID: wprim-760318

RESUMO

OBJECTIVES: Late-onset schizophrenia (LOS, age at first onset ≥40 years) is characterized by including predominance of women, better premorbid social adjustment and lower severity of positive/negative symptoms. However, few studies have been conducted on LOS, especially in Asian countries. This study aimed to examine the clinical features of LOS in comparison with early-onset schizophrenia (EOS). METHODS: By retrospectively reviewing medical records, we assessed demographic data and clinical features of 76 LOS (20 males) and 357 EOS (144 males) who admitted to the psychiatric ward of a general hospital. RESULTS: The mean ages of onset were 47.3±5.1 (LOS) and 25.7±6.5 (EOS) years. There were significantly more women in LOS (73.7%) than EOS (59.6%). Significantly more LOS patients had a marital (88.2% vs. 25.8%) and employment history (28.9% vs. 13.1%) than EOS. Patients with LOS had fewer negative (14.3±9.0 vs. 19.9±9.3), general psychopathology score (36.9±11.1 vs. 42.3±13.9) than EOS patients. CONCLUSION: In line with previous studies, this study demonstrated that LOS patients have better premorbid social adjustment. Our finding also replicates previous findings that LOS patients differ from EOS in predominance of women and relative lack of negative symptoms. These results suggest that LOS may be a distinct subtype of schizophrenia.


Assuntos
Idade de Início , Grupo com Ancestrais do Continente Asiático , Emprego , Feminino , Hospitais Gerais , Humanos , Coreia (Geográfico) , Registros Médicos , Psicopatologia , Estudos Retrospectivos , Esquizofrenia , Razão de Masculinidade , Ajustamento Social
7.
Artigo em Inglês | WPRIM (Pacífico Ocidental) | ID: wprim-759786

RESUMO

BACKGROUND: The occurrence of cutaneous malignant tumors has been increasing worldwide due to changes in various environmental factors. OBJECTIVE: Our study aimed to analyze the overall tendency of clinical characteristics in single-center patients with cutaneous malignant tumors according to sex, age, duration, size, anatomic site, treatment, and concomitant diseases. METHODS: We retrospectively reviewed the medical records and clinical photographs of 319 patients diagnosed with cutaneous malignant tumors (basal cell carcinoma, squamous cell carcinoma, melanoma, lymphoma, Paget's disease, metastatic skin cancer, mycosis fungoides, angiosarcoma, dermatofibrosarcoma protuberans, Kaposi sarcoma, malignant fibrous histiocytoma, Merkel cell carcinoma) and 109 patients diagnosed with premalignant tumors (Bowen's disease) between January 2007 and January 2017. RESULTS: The average annual incidence of malignant cutaneous tumors was 2.4%. In total, 158 males (36.9%) and 270 females (63.1%) were included with a mean age of onset of 66 years. Among the malignant tumors, basal cell carcinoma (30.8%) was the most common, while the incidences of squamous cell carcinoma (18.9%), melanoma (6.3%), and lymphoma (4.4%) were also high. The predominantly involved anatomic sites were the face (67.3%), trunk (11.5%), and legs (10.9%). Most cases involved solitarily existing tumors (77.3%), and pruritus (21.7%) was the most common accompanying symptom. Among the 69.6% of patients who received treatment for their tumors, the most common method was surgical removal. No significant increase in concomitant diseases or environmental factors was detected. CONCLUSION: Our study is meaningful as an overall and recent review of various types of cutaneous malignancies that provides preliminary data for further large-scale nationwide studies.


Assuntos
Idade de Início , Carcinoma Basocelular , Carcinoma de Células Escamosas , Dermatofibrossarcoma , Feminino , Hemangiossarcoma , Histiocitoma Fibroso Maligno , Humanos , Incidência , Perna (Membro) , Linfoma , Masculino , Registros Médicos , Melanoma , Métodos , Micose Fungoide , Prurido , Estudos Retrospectivos , Sarcoma de Kaposi , Neoplasias Cutâneas , Estatística como Assunto
8.
Artigo em Inglês | WPRIM (Pacífico Ocidental) | ID: wprim-758957

RESUMO

Calf diarrhea caused by infectious agents is associated with economic losses in the cattle industry. The purpose of this study was to identify the causative agents and epidemiological characteristics of diarrhea in Korean native calves (KNC). In total, 207 diarrheal KNC aged less than 7 months were investigated. Fecal samples collected from the rectum were examined for causative agents using polymerase chain reaction (PCR) or real-time PCR and the number of oocysts were counted. Fourteen causative agents were detected from 164 of the 207 diarrheal KNC. Rotavirus was the most common agent (34.8%), followed by Eimeria spp. (31.7%), Escherichia coli (22.0%), Giardia spp. (14.0%), Clostridium difficile (9.8%), bovine viral diarrhea virus (8.5%), coronavirus (7.9%), Cryptosporidium spp. (7.3%), torovirus (6.7%), parvovirus (5.5%), norovirus (4.9%), kobuvirus (1.8%), adenovirus (1.2%), and Salmonella spp. (0.6%). About 95 (57.9%) of 164 calves were infected with a single causative agent and 42.1% were infected by multiple agents. No significant difference was observed in mortality between calves infected with a single agent and multiple agents. The occurrence of diarrhea caused by rotavirus, Eimeria spp., kobuvirus, and Giardia spp. was significantly different based on onset age, and the prevalence of diarrhea caused by rotavirus or C. difficile was significantly different between seasons. This study help the understanding of KNC diarrhea for the development of an effective strategy for disease prevention and control, especially in Eastern provinces of South Korea.


Assuntos
Adenoviridae , Idade de Início , Animais , Bovinos , Clostridium difficile , Coronavirus , Cryptosporidium , Diarreia , Eimeria , Epidemiologia , Escherichia coli , Giardia , Kobuvirus , Coreia (Geográfico) , Mortalidade , Norovirus , Oocistos , Parvovirus , Reação em Cadeia da Polimerase , Prevalência , Reação em Cadeia da Polimerase em Tempo Real , Reto , Rotavirus , Salmonella , Estações do Ano , Torovirus
9.
Journal of Breast Cancer ; : 297-310, 2019.
Artigo em Inglês | WPRIM (Pacífico Ocidental) | ID: wprim-764261

RESUMO

PURPOSE: Numerous previous studies have reported inconsistent results about the differences between synchronous contralateral breast cancer (sCBC) and metachronous contralateral breast cancer (mCBC). This study aimed to compare the clinical characteristics and outcomes between sCBC and mCBC and determine predictive factors for the survival of sCBC and mCBC patients. METHODS: Using the Surveillance, Epidemiology, and End Results Program database, we identified sCBC or mCBC patients from 2000 to 2010. The Kaplan-Meier method and Cox proportional hazards regression analysis were used to analyze overall survival and breast cancer-specific survival (BCSS) rates of sCBCs and mCBCs, respectively. RESULTS: Overall, 14,057 sCBC (n = 8,139, 57.9%) and mCBC (n = 5,918, 42.1%) patients were included. The first tumors of sCBC were more likely to have higher stage and more lymph and distant metastases, whereas those of mCBC were more often infiltrating ductal carcinoma (IDC), had localized stage, were estrogen receptor (ER) and progesterone receptor (PR) negative, and had less axillary nodal involvement. The second tumors of mCBC tended to be IDC and have higher grade, adverse stage, ER and PR-negativity; and more axillary nodal involvement, compared to the second tumors of sCBC. mCBC patients had significantly favorable 5-year BCSS but worse long-term BCSS compared with sCBC patients. Moreover, subgroup analysis revealed no significant difference of BCSS between sCBC and mCBC among patients aged 18–60 years. Multivariate analysis indicated that age, grade, and stage of 2 tumors; surgery for second tumor; and ER status of the second tumor were independent prognostic factors for BCSS of contralateral breast cancer (CBC). CONCLUSION: The characteristics and outcomes of sCBCs and mCBCs were substantially different. sCBC and mCBC patients may have different prognosis, and the prognosis of CBC depends on the first and second tumors.


Assuntos
Idade de Início , Neoplasias da Mama , Mama , Carcinoma Ductal , Estrogênios , Humanos , Métodos , Análise Multivariada , Metástase Neoplásica , Prognóstico , Receptores de Progesterona , Fatores de Risco , Programa de SEER
10.
Artigo em Inglês | WPRIM (Pacífico Ocidental) | ID: wprim-763572

RESUMO

OBJECTIVE: Alcohol-induced blackout (blackout) is a typical early symptom of cognitive impairment caused by drinking. However, the first onset age of blackout or the duration after onset of blackout has not been directly compared in previous studies. The purpose of this study was to investigate the differences in cognitive function to the first start age of blackouts and their duration. METHODS: Thirty-one male subjects were included in this study. Their age at the first blackout and the duration after the onset of blackout were investigated. Neuropsychological tests were conducted to determine their attention, memory, and executive function. Subjects were divided into three groups according to their age of the first onset blackout (group O1, 40 years). Subjects were also divided into three groups by duration after the onset of blackout (P1, 30 years). We then examined differences in neurocognitive function among these groups. RESULTS: O1 tended to have a lower memory score than O2 (F = 3.28, p = 0.053). Significant differences were observed in attention and executive function between groups P1 and P3 (Digit Span_backward: F = 6.07, p < 0.05; visual span_forward: F = 4.19, p < 0.05; executive intelligence quotient: F = 3.55, p < 0.05). CONCLUSION: Greater memory impairment was detected in subjects having an earlier age of the first blackout. The longer the duration after the onset of blackout, the more impaired their attention and executive function skills.


Assuntos
Idade de Início , Transtornos Induzidos por Álcool , Amnésia Retrógrada , Transtornos Cognitivos , Cognição , Ingestão de Líquidos , Função Executiva , Humanos , Inteligência , Masculino , Memória , Testes Neuropsicológicos
11.
Artigo | WPRIM (Pacífico Ocidental) | ID: wprim-763557

RESUMO

OBJECTIVE: Genetics factors are likely to play a role in the risk, clinical presentation and treatment outcome in major depressive disorder (MDD). In this study, we investigated the role of three candidate genes for MDD; calcium voltage-gated channel subunit alpha1 C (CACNA1C), cholinergic receptor nicotinic alpha 7 subunit (CHRNA7), and mitogen-activated protein kinase 1 (MAPK1). METHODS: Two-hundred forty-two MDD patients and 326 healthy controls of Korean ancestry served as samples for the analyses. Thirty-nine single nucleotide polymorphisms (SNPs) within CACNA1C, CHRNA7, and MAPK1 genes were genotyped and subsequently tested for association with MDD (primary analysis) and other clinical features (symptoms’ severity, age of onset, history of suicide attempt, treatment outcome) (secondary analyses). Single SNPs, haplotypes and epistatic analyses were performed. RESULTS: Single SNPs were not associated with disease risk and clinical features. However, a combination of alleles (haplotype) within MAPK1 was found associated with MDD-status. Secondary analyses detected a possible involvement of CACNA1C haplotype in resistance to antidepressant treatment. CONCLUSION: These data suggest a role for MAPK1 and CACNA1C in MDD risk and treatment resistance, respectively. However, since many limitations characterize the analysis, the results must be considered with great caution and verified.


Assuntos
Idade de Início , Alelos , Cálcio , Depressão , Transtorno Depressivo Maior , Genética , Haplótipos , Humanos , Proteína Quinase 1 Ativada por Mitógeno , Plasticidade Neuronal , Polimorfismo de Nucleotídeo Único , Suicídio , Resultado do Tratamento
12.
Artigo em Inglês | WPRIM (Pacífico Ocidental) | ID: wprim-741366

RESUMO

PURPOSE: Myotonic dystrophy, also known as dystrophia myotonica (DM), is an autosomal dominant disorder with 2 genetically distinct forms. DM type 1 (DM1) is the more common form and is caused by abnormal expansion of cytosine/thymine/guanine (CTG) repeats in the DM protein kinase (DMPK) gene. Our study aimed to determine whether the age of onset is correlated with CTG repeat length in a population of pediatric patients with DM1. METHODS: We retrospectively identified 30 pediatric patients with DM1 that underwent DMPK testing, of which the clinical data of 17 was sufficient. The cohort was divided into 2 subgroups based on the clinical phenotype (congenital-onset vs. late-onset) and number of CTG repeats ( < 1,000 vs. ≥1,000). RESULTS: We found no significant difference between the age of onset and CTG repeat length in our pediatric patient population. Based on clinical subgrouping, we found that the congenital-onset subgroup was statistically different with respect to several variables, including prematurity, rate of admission to neonatal intensive care unit, need for respiratory support at birth, hypotonia, dysphagia, ventilator dependence, and functional status on last visit, compared to the late-onset subgroup. Based on genetic subgrouping, we found a single variable (poor feeding in neonate) that was significantly different in the large CTG subgroup than that in the small CTG subgroup. CONCLUSION: Clinical variables exhibiting statistically significant differences between the subgroups should be focused on prognosis and designing tailored management approaches for the patients; our findings will contribute to achieve this important goal for treating patients with DM1.


Assuntos
Idade de Início , Estudos de Coortes , Transtornos de Deglutição , Estudos de Associação Genética , Genótipo , Humanos , Recém-Nascido , Terapia Intensiva Neonatal , Hipotonia Muscular , Distrofia Miotônica , Miotonina Proteína Quinase , Parto , Fenótipo , Prognóstico , Estudos Retrospectivos , Ventiladores Mecânicos
13.
J. bras. pneumol ; 45(5): e20180079, 2019. tab, graf
Artigo em Inglês | LILACS (Américas) | ID: biblio-1012575

RESUMO

ABSTRACT Objective: To describe the clinical, functional, and radiological features of index cases of familial pulmonary fibrosis (FPF) in Brazil. Methods: We evaluated 35 patients with FPF - of whom 18 (51.4%) were women - with a median age of 66.0 years (range, 35.5-89.3 years). All of the patients completed a standardized questionnaire, as well as undergoing pulmonary function tests and HRCT of the chest. In 6 cases, lung tissue samples were obtained: from surgical biopsies in 5 cases; and from an autopsy in 1 case. Results: A history of smoking and a history of exposure to birds or mold were reported in 45.7% and 80.0% of the cases, respectively. Cough and marked dyspnea were reported by 62.8% and 48.6% of the patients, respectively. Fine crackles were detected in 91.4% of the patients. In 4 patients, the findings were suspicious for telomere disease. The median FVC and DLCO, as percentages of the predicted values, were 64.9% (range, 48.8-105.7%) and 38.9% (range, 16.0-60.0%), respectively. Nine patients had reduced DLCO despite having normal spirometry results. Regarding HRCT, patterns typical of usual interstitial pneumonia were found in 6 patients (17.1%). In 25 cases (71.5%), the HRCT features were consistent with a diagnosis other than idiopathic pulmonary fibrosis. In 11 cases (31.4%), the radiological patterns were uncharacteristic of interstitial lung disease. Of the six lung tissue samples analyzed, four showed interstitial pneumonia with bronchiolocentric accentuation, and, on the basis of the clinical and radiological data, the corresponding patients were diagnosed with hypersensitivity pneumonitis. Conclusions: Patients with FPF can present with a wide variety of clinical features. Most HRCT scans of these patients exhibit patterns not typical of usual interstitial pneumonia. The family history of fibrotic lung diseases should be investigated in all patients under suspicion, regardless of their age.


RESUMO Objetivo: Descrever as características clínicas, funcionais e radiológicas de um grupo de casos índice diagnosticados com fibrose pulmonar familiar (FPF) no Brasil. Métodos: Trinta e cinco pacientes com FPF (18 mulheres; 51,4%), com mediana de idade de 66,0 anos (variação: 35,5-89,3 anos), responderam a um questionário padronizado e foram submetidos a testes de função pulmonar e TCAR de tórax. Tecido pulmonar foi obtido para revisão em 6 casos: a partir de biópsias cirúrgicas em 5 e de autópsia em 1. Resultados: Antecedentes de tabagismo e de exposição a aves ou mofo foram referidos por 45,7% e 80,0% dos casos, respectivamente. Tosse e dispneia significante foram referidas por 62,8% e 48,6% dos pacientes, respectivamente. Estertores finos foram detectados em 91,4% dos indivíduos. Em 4 pacientes, os achados levantaram suspeitas de doença dos telômeros. As medianas da CVF e da DLCO foram, respectivamente, de 64,9% (variação: 48,8-105,7%) e 38,9% (variação: 16,0-60,0%) em porcentagem dos valores previstos. Apesar de espirometria normal, 9 pacientes exibiram DLCO reduzida. Em relação às TCAR, padrões típicos de pneumonia intersticial usual foram encontrados em 6 pacientes (17,1%). Em 25 casos (71,5%) os achados tomográficos foram mais consistentes com um diagnóstico de não relacionado a fibrose pulmonar idiopática. Em 11 pacientes (31,4%) o padrão radiológico foi incaracterístico para doença pulmonar intersticial. Das seis amostras de tecido pulmonar analisadas, quatro mostraram pneumonias intersticiais com acentuação bronquiolocêntrica e, em função de outros dados clínicos e radiológicos, pneumonite de hipersensibilidade foi diagnosticada. Conclusões: Pacientes com FPF podem apresentar características clínicas diversas. A maioria das TCAR desses pacientes exibe padrões não típicos de pneumonia intersticial usual. A pesquisa da história clínica de outros casos de pneumopatias fibrosantes na família deve ser feita em todos os pacientes em investigação, independentemente da idade.


Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Fibrose Pulmonar/patologia , Doenças Pulmonares Intersticiais/patologia , Fibrose Pulmonar/epidemiologia , Fibrose Pulmonar/diagnóstico por imagem , Testes de Função Respiratória , Biópsia , Brasil/epidemiologia , Tomografia Computadorizada por Raios X , Distribuição por Sexo , Doenças Pulmonares Intersticiais/epidemiologia , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Idade de Início , Distribuição por Idade
14.
Artigo em Inglês | WPRIM (Pacífico Ocidental) | ID: wprim-765850

RESUMO

OBJECTIVE: Ample evidence has suggested that age at onset of Parkinson's disease (PD) is associated with heterogeneous clinical features in individuals. We hypothesized that this may be attributed to different patterns of nigrostriatal dopamine loss. METHODS: A total of 205 consecutive patients with de novo PD who underwent 18F-FP-CIT PET scans (mean follow-up duration, 6.31 years) were divided into three tertile groups according to their age at onset of parkinsonian motor symptoms. Striatal dopamine transporter (DAT) availability was compared between the old- (n = 73) and young-onset (n = 66) groups. In addition, the risk of developing freezing of gait (FOG) and longitudinal requirements for dopaminergic medications were examined. RESULTS: The old-onset PD group (mean age at onset, 72.66 years) exhibited more severe parkinsonian motor signs than the young-onset group (52.58 years), despite comparable DAT availability in the posterior putamen; moreover, the old-onset group exhibited more severely decreased DAT availability in the caudate than the young-onset group. A Cox regression model revealed that the old-onset PD group had a higher risk for developing FOG than the young-onset group [hazard ratio 2.523, 95% confidence interval (1.239–5.140)]. The old-onset group required higher doses of dopaminergic medications for symptom control than the young-onset group over time. CONCLUSION: The present study demonstrated that the old-onset PD group exhibited more severe dopamine loss in the caudate and were more likely to develop gait freezing, suggesting that age at onset may be one of the major determinants of the pattern of striatal dopamine depletion and progression of gait disturbance in PD.


Assuntos
Idade de Início , Proteínas da Membrana Plasmática de Transporte de Dopamina , Dopamina , Seguimentos , Congelamento , Marcha , Humanos , Doença de Parkinson , Tomografia por Emissão de Pósitrons , Putamen , Tempo (Meteorologia)
15.
Artigo em Inglês | WPRIM (Pacífico Ocidental) | ID: wprim-765349

RESUMO

Brain tumors are the second most common type of structural brain lesion that causes chronic epilepsy. Patients with low-grade brain tumors often experience chronic drug-resistant epilepsy starting in childhood, which led to the concept of long-term epilepsy-associated tumors (LEATs). Dysembryoplastic neuroepithelial tumor and ganglioglioma are representative LEATs and are characterized by young age of onset, frequent temporal lobe location, benign tumor biology, and chronic epilepsy. Although highly relevant in clinical epileptology, the concept of LEATs has been criticized in the neuro-oncology field. Recent genomic and molecular studies have challenged traditional views on LEATs and low-grade gliomas. Molecular studies have revealed that low-grade gliomas can largely be divided into three groups : LEATs, pediatric-type diffuse low-grade glioma (DLGG; astrocytoma and oligodendroglioma), and adult-type DLGG. There is substantial overlap between conventional LEATs and pediatric-type DLGG in regard to clinical features, histology, and molecular characteristics. LEATs and pediatric-type DLGG are characterized by mutations in BRAF, FGFR1, and MYB/MYBL1, which converge on the RAS-RAF-MAPK pathway. Gene (mutation)-centered classification of epilepsy-associated tumors could provide new insight into these heterogeneous and diverse neoplasms and may lead to novel molecular targeted therapies for epilepsy in the near future.


Assuntos
Idade de Início , Astrocitoma , Biologia , Encéfalo , Neoplasias Encefálicas , Classificação , Epilepsia , Ganglioglioma , Glioma , Humanos , Terapia de Alvo Molecular , Neoplasias Neuroepiteliomatosas , Convulsões , Lobo Temporal
16.
Artigo em Inglês | WPRIM (Pacífico Ocidental) | ID: wprim-765014

RESUMO

BACKGROUND: Panic disorder (PD) and major depressive disorder (MDD) can occur concurrently, despite different clinical manifestations. Because MDD and PD patients tend to have more complicated conditions, understanding the co-occurrence and pattern of these conditions is important. Here, we investigated the influence of PD and MDD on each other, with respect to time interval. METHODS: Data from three national representative surveys were pooled (total 18,807 respondents), and the age of onset (AOO) of PD and MDD was analyzed. We performed Kaplan-Meier analysis to estimate separate survival functions, using the AOO of MDD and PD as the outcome. To understand the temporal effect of other disorders, we used a Cox proportional hazard model to estimate the hazard ratios for the onset of MDD/PD with other comorbidities as time-dependent covariates. RESULTS: PD elevated the risk of subsequent MDD by 1.5-fold, whereas MDD elevated the risk of subsequent PD by 3.8-fold. The effect of such an elevation risk was significant for up to 2 years. CONCLUSION: The results revealed a bidirectional relationship between MDD and PD. Each disease represents a risk of a subsequent occurrence of the other, which lasts for a considerable duration.


Assuntos
Idade de Início , Comorbidade , Transtorno Depressivo Maior , Humanos , Estimativa de Kaplan-Meier , Coreia (Geográfico) , Transtorno de Pânico , Pânico , Modelos de Riscos Proporcionais
17.
Intestinal Research ; : 486-495, 2019.
Artigo em Inglês | WPRIM (Pacífico Ocidental) | ID: wprim-785867

RESUMO

BACKGROUND/AIMS: Information about familial aggregation of inflammatory bowel disease (IBD) in Asia is limited. We aimed to analyze the prevalence and risk of familial IBD in an Indian cohort and compare familial and sporadic cases.METHODS: Familial IBD cases were identified from a large prospectively maintained IBD registry. The prevalence of IBD in first- and seconddegree relatives of index cases was evaluated. The disease behavior was compared to that of sporadic cases.RESULTS: Total 3,553 patients (ulcerative colitis [UC], 2,053; Crohn’s disease [CD], 1,500) were included. Familial IBD was noted in 4.13% of CD and 4.34% of UC patients. Family history was commoner in pediatric group (< 18 years) (P= 0.0002; odds ratio [OR], 2.8; 95% confidence interval [CI], 1.6–4.8). Majority had paternal transmission (UC, 67.42%; CD, 70.97%). Concordance of disease type was higher in UC (79.7%) compared to CD (37.1%). Familial IBD was associated with higher cumulative relapse rate (CD, P< 0.001; UC, P< 0.001), higher cumulative rate of surgery (CD, P< 0.001; UC, P< 0.001) and higher rate of biologic use (CD, P= 0.010; UC, P= 0.015). Pan-colitis was higher in familial UC (P= 0.003; OR, 1.935; 95% CI, 1.248–3.000). Fistulizing disease was commoner in familial CD (P= 0.041; OR, 2.044; 95% CI, 1.030–4.056).CONCLUSIONS: The prevalence of familial IBD in India appears comparable to rest of Asia but lower than the West. It is associated with a younger age of onset, higher incidence of pan-colitis in UC and fistulizing complications in CD. Familial IBD has higher cumulative relapse, surgery and biologic use rates. Hence, family history of IBD could have important prognostic implications.


Assuntos
Idade de Início , Ásia , Estudos de Coortes , Colite , Colite Ulcerativa , Doença de Crohn , Humanos , Incidência , Índia , Doenças Inflamatórias Intestinais , Razão de Chances , Prevalência , Estudos Prospectivos , Recidiva
18.
Artigo em Inglês | WPRIM (Pacífico Ocidental) | ID: wprim-785812

RESUMO

PURPOSE: This study was performed to analyze the clinical and imaging features of contemporary osteomyelitis (OM) and to investigate differences in these features on panoramic radiography according to patients' history of use of medication affecting bone metabolism.MATERIALS AND METHODS: The records of 364 patients (241 female and 123 male, average age 66.8±14.9 years) with OM were retrospectively reviewed. Panoramic imaging features were analyzed and compared between patients with medication-related OM (m-OM) and those with conventional, medication-unrelated OM (c-OM).RESULTS: The age of onset of OM tended to be high, with the largest number of patients experiencing onset in their 70s. The 2 most frequent presumed causes were antiresorptive medication use (44.2%) and odontogenic origin (34.6%). On panoramic radiographs, a mix of osteolysis and sclerosis was the most common lesion pattern observed (68.6%). Sequestrum, extraction socket, and periosteal new bone formation were found in 143 (42.1%), 79 (23.2%), and 24 (7.1%) cases, respectively. The m-OM group exhibited sequestrum and extraction socket more frequently and displayed significantly higher mandibular cortical index values than the c-OM group.CONCLUSION: We observed some differences in imaging features as shown on panoramic radiography according to the history of antiresorptive medication use. This study may help elucidate the predictive imaging features of medication-related osteonecrosis of the jaw.


Assuntos
Idade de Início , Feminino , Humanos , Arcada Osseodentária , Masculino , Metabolismo , Osteogênese , Osteólise , Osteomielite , Osteonecrose , Radiografia Panorâmica , Estudos Retrospectivos , Esclerose
19.
Artigo em Inglês | WPRIM (Pacífico Ocidental) | ID: wprim-785637

RESUMO

Mucopolysaccharidosis type II (MPS II) is a rare X-linked recessive lysosomal storage disease caused by mutation of the iduronate-2-sulfatase gene. The mutation results in iduronate-2-sulfatase deficiency, which causes the progressive accumulation of heparan sulfate and dermatan sulfate in cellular lysosomes. The phenotype, age of onset, and symptoms of MPS II vary; accordingly, the disease can be classified into either the early-onset type or the late-onset type, depending on the age of onset and the severity of the symptoms. In patients with severe MPS II, symptoms typically first appear between 2 and 5 years of age. Patients with severe MPS II usually die in the second decade of life although some patients with less severe disease have survived into their fifth or sixth decade. Here, we report the establishment of a preimplantation genetic diagnosis (PGD) strategy using multiplex nested polymerase chain reaction, direct sequencing, and linkage analysis. Unaffected embryos were selected via the diagnosis of a single blastomere, and a healthy boy was delivered by a female carrier of MPS II. This is the first successful application of PGD in a patient with MPS II in Korea


Assuntos
Idade de Início , Blastômeros , Dermatan Sulfato , Diagnóstico , Estruturas Embrionárias , Feminino , Heparitina Sulfato , Humanos , Coreia (Geográfico) , Doenças por Armazenamento dos Lisossomos , Lisossomos , Masculino , Mucopolissacaridoses , Mucopolissacaridose II , Reação em Cadeia da Polimerase Multiplex , Parto , Fenótipo , Reação em Cadeia da Polimerase , Diagnóstico Pré-Implantação , Prostaglandinas D
20.
Artigo em Inglês | WPRIM (Pacífico Ocidental) | ID: wprim-785582

RESUMO

BACKGROUND: Primary hyperoxaluria (PH), a rare inborn error of glyoxylate meta bolism causing overproduction of oxalate, is classified into three genetic subgroups: type 1–3 (PH1–PH3) caused by AGXT, GRHPR , and HOGA1 gene mutations, respectively. We performed a retrospective case series study of Korean pediatric patients with PH.METHODS: In total, 11 unrelated pediatric patients were recruited and their phenotypes and genotypes were analyzed by a retrospective review of their medical records.RESULTS: Mutational analyses revealed biallelic AGXT mutations (PH1) in nine patients and a single heterozygous GRHPR and HOGA1 mutation in one patient each. The c.33dupC was the most common AGXT mutation with an allelic frequency of 44%. The median age of onset was 3 months (range, 2 months-3 years), and eight patients with PH1 presented with end stage renal disease (ESRD). Patients with two truncating mutations showed an earlier age of onset and more frequent retinal involvement than patients with one truncating mutation. Among eight PH1 patients presenting with ESRD, five patients were treated with intensive dialysis followed by liver transplantation (n=5) with/without subsequent kidney transplantation (n=3).CONCLUSION: Most patients presented with severe infantile forms of PH. Patients with two truncating mutations displayed more severe phenotypes than those of patients with one truncating mutation. Sequential liver and kidney transplantation was adopted for PH1 patients presenting with ESRD. A larger nation-wide multicenter study is needed to confirm the genotype-phenotype correlations and outcomes of organ transplantation.


Assuntos
Idade de Início , Diálise , Estudos de Associação Genética , Genótipo , Humanos , Concentração de Íons de Hidrogênio , Hiperoxalúria Primária , Falência Renal Crônica , Transplante de Rim , Fígado , Transplante de Fígado , Registros Médicos , Transplante de Órgãos , Fenótipo , Retinaldeído , Estudos Retrospectivos , Transplantes
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