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1.
Sci Rep ; 10(1): 11868, 2020 07 17.
Artigo em Inglês | MEDLINE | ID: mdl-32681105

RESUMO

A new method to predict coronary artery lesions (CALs) in Kawasaki disease (KD) was developed using a mean structure equation model (SEM) and neural networks (Nnet). There were 314 admitted children with KD who met at least four of the six diagnostic criteria for KD. We defined CALs as the presence of a maximum z score of ≥ 3.0. The SEM using age, sex, intravenous immunoglobulin resistance, number of steroid pulse therapy sessions, C-reactive protein level, and urinary ß2-microglobulin (u-ß2MG/Cr) values revealed a perfect fit based on the root mean square error of approximation with an R2 value of 1.000 and the excellent discrimination of CALs with a sample score (SS) of 2.0 for a latent variable. The Nnet analysis enabled us to predict CALs with a sensitivity, specificity and c-index of 73%, 99% and 0.86, respectively. This good and simple statistical model that uses common parameters in clinical medicine is useful in deciding the appropriate therapy to prevent CALs in Japanese KD patients.


Assuntos
Doença da Artéria Coronariana/diagnóstico , Doença da Artéria Coronariana/etiologia , Suscetibilidade a Doenças , Síndrome de Linfonodos Mucocutâneos/complicações , Redes Neurais de Computação , Adulto , Biomarcadores , Feminino , Humanos , Masculino , Modelos Estatísticos , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/terapia , Prognóstico , Estudos Retrospectivos , Sensibilidade e Especificidade
2.
CEN Case Rep ; 9(2): 95-100, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-31707643

RESUMO

The oculocerebrorenal disorder of Lowe syndrome is an X-linked mutation in the gene oculocerebrorenal syndrome of Lowe 1 (OCRL), characterized by the triad of congenital cataracts, severe intellectual impairment, and renal tubular dysfunction. Manifestations of phenotype in female carriers and patients are extremely rare. We present a female case with congenital cataracts, severe intellectual impairment, sensorineural hearing loss, and renal tubular dysfunction as Lowe syndrome. A 9-year-old Japanese girl visited our hospital due to prolonged proteinuria. Her renal biopsy revealed diffuse mesangium proliferation, sclerosis and dilatation of renal tubules, and mild IgA deposition in the mesangial region. Furthermore, she had congenital cataracts, severe intellectual impairment, and sensorineural hearing loss. Genetic screening did not identify mutations of the ORCL gene encoding inositol polyphosphate 5-phosphatase (IPP-5P) (46 XX, female). However, we found the reduction of enzyme activity of IPP-5P to 50% of the normal value. Furthermore, her renal function had deteriorated to renal failure within a decade. Finally, she received peritoneal dialysis and renal transplantation. We present the oculocerebrorenal phenotype of Lowe syndrome in a female patient with reduced activity of IPP-5P without OCRL gene mutation.


Assuntos
Inositol Polifosfato 5-Fosfatases/metabolismo , Síndrome Oculocerebrorrenal/diagnóstico , Síndrome Oculocerebrorrenal/genética , Insuficiência Renal/terapia , Povo Asiático/etnologia , Povo Asiático/genética , Catarata/congênito , Criança , Progressão da Doença , Feminino , Glomerulonefrite por IGA/complicações , Perda Auditiva Neurossensorial/congênito , Humanos , Deficiência Intelectual/diagnóstico , Transplante de Rim/métodos , Túbulos Renais Proximais/patologia , Mutação , Síndrome Oculocerebrorrenal/enzimologia , Diálise Peritoneal/métodos , Fenótipo , Proteinúria/diagnóstico , Proteinúria/etiologia , Índice de Gravidade de Doença
3.
Medicine (Baltimore) ; 97(43): e12930, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30412100

RESUMO

Enterovirus and human parechovirus (HPeV) are RNA viruses belonging to the family Picornaviridae that frequently infect infants. These infections show a wide variety of clinical manifestations, from mild to severe. However, there are no known early clinical markers for diagnosis and prediction of disease severity. The aim of this study was to examine the clinical utility of urinary beta 2-microglobulin (ß2MG) for the early detection and prognosis of infantile enterovirus and HPeV infections.This retrospective study included 108 full-term infants younger than 60 days of age, including 15 with enterovirus or HPeV-3 (enterovirus/HPeV-3), 22 with respiratory syncytial virus (RSV), and 24 with bacterial infections. Laboratory data and clinical characteristics were compared among these 3 groups. Of the 15 patients with enterovirus/HPeV-3, 6 were treated with intravenous immunoglobulin (IVIG subgroup) because of severe clinical conditions.Urinary ß2MG to creatinine ratio (ß2MG/Cr) was significantly higher in the enterovirus/HPeV-3 group compared to bacterial and RSV infection groups (both P < .001). In the enterovirus/HPeV-3 group, mean peak urinary ß2MG/Cr was observed on day 1 or 2. Urinary ß2MG/Cr values were significantly higher in the IVIG subgroup than the non-IVIG subgroup (P < .001).Increased urinary ß2MG/Cr in early-stage infection may be a useful clinical marker for the detection and prediction of infantile enterovirus and HPeV infection severity.


Assuntos
Infecções por Enterovirus/diagnóstico , Enterovirus/isolamento & purificação , Parechovirus/isolamento & purificação , Infecções por Picornaviridae/diagnóstico , Microglobulina beta-2/urina , Biomarcadores/urina , Creatina/urina , Infecções por Enterovirus/fisiopatologia , Infecções por Enterovirus/urina , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Infecções por Picornaviridae/fisiopatologia , Infecções por Picornaviridae/urina , Estudos Retrospectivos , Índice de Gravidade de Doença
4.
J Neurol Sci ; 368: 109-12, 2016 Sep 15.
Artigo em Inglês | MEDLINE | ID: mdl-27538611

RESUMO

The magnetic resonance imaging findings of reversible isolated lesions with transiently reduced diffusion in the splenium of corpus callosum of patients with a wide spectrum of pathological conditions are referred to as reversible splenial lesion syndrome (RESLES). Clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) is probably included within the spectrum of RESLES; however, its exact pathophysiology is not known. Here, we describe three patients with MERS and one patient with RESLES, all of whom showed elevated urinary ß2-microglobulin regardless of diagnosis and presence of pathogens. Elevated urinary ß2-microglobulin suggested that an excessive immune response might play a role in the pathophysiology of reversible splenial lesions.


Assuntos
Encefalopatias/diagnóstico por imagem , Encefalopatias/urina , Corpo Caloso/diagnóstico por imagem , Microglobulina beta-2/urina , Biomarcadores/urina , Encefalopatias/tratamento farmacológico , Criança , Pré-Escolar , Imagem de Difusão por Ressonância Magnética , Feminino , Humanos , Masculino
5.
Pediatr Int ; 55(6): 714-21, 2013 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-23745743

RESUMO

BACKGROUND: Neuraminidase inhibitors have been reported to decrease mortality in patients infected with influenza A (H1N1) pdm 2009 (H1N1 pdm09), but it is not clear whether they are effective against H1N1pdm09 in apparently healthy children. METHODS: The effect of early treatment with neuraminidase inhibitors on 70 otherwise healthy children with possible H1N1 pdm09 (pH1N1pdm09) infection was investigated. The children were simultaneously treated with a neuraminidase inhibitor (oseltamivir or zanamivir) and maoto, a Japanese traditional herbal medicine, which had been reported to be effective against seasonal influenza. Clinical severity was assessed using patient history, namely the worst values for clinical vital signs and laboratory data on admission. After refining these parameters with univariate, decision tree and multiple regression analysis, mean covariance structure equation analysis was used to investigate the association of estimated clinical severity to the selected parameters. RESULTS: Total path analysis using a Bayesian method indicated that the estimated clinical severity of pH1N1pdm09 was positively associated with maximum body temperature, pulse rate, respiration rate, duration necessary for defervescence, admission duration and log urinary ß2-microglobulin/creatinine level, and negatively associated with age and the presence and duration of treatment with the neuraminidase inhibitor in the outpatient clinic. CONCLUSIONS: This study provides the first clinical evidence that early treatment with neuraminidase inhibitors in outpatient clinic decreased the estimated clinical severity of pH1N1pdm09 in apparently otherwise healthy pediatric inpatients.


Assuntos
Inibidores Enzimáticos/uso terapêutico , Vírus da Influenza A Subtipo H1N1 , Influenza Humana/tratamento farmacológico , Neuraminidase/antagonistas & inibidores , Oseltamivir/uso terapêutico , Zanamivir/uso terapêutico , Criança , Intervenção Médica Precoce , Feminino , Humanos , Masculino
6.
J Infect Chemother ; 19(5): 833-42, 2013 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-23467791

RESUMO

The clinical severity of the 2009 pandemic H1N1 influenza (H1N1 pdm09) was thought to be related to the difference between the amount of viral load and condition of the host immune response. We investigated the role of serum levels of IgG and its subclasses in clinical severity using the data from 45 child inpatients suffering from bronchitis or mild pneumonia caused by possible H1N1 pdm09 (pH1N1 pdm09) infection. After selecting parameters for serum IgG subclasses and logarithmically transformed urinary beta-2 microglobulin/creatinine (b2MG/Cr) values and admission duration, we performed path analysis using a mean covariance structure equation analysis to investigate the relationship between the clinical severity and the foregoing selected parameters. Total path analyses using a Bayesian method revealed that the estimated clinical severity caused by pH1N1 pdm09 was positively associated with maximal respiration rates, admission duration, and log urinary b2MG/Cr levels, whereas negatively associated with serum IgG, IgG1, IgG2, and IgG3 levels, duration of neuraminidase inhibitor therapy in outpatient clinics, and age. Serum IgG and its subclasses (IgG1-IgG3) reduced estimated clinical severity in children with pH1N1 pdm09 infection.


Assuntos
Imunoglobulina G/sangue , Vírus da Influenza A Subtipo H1N1/isolamento & purificação , Influenza Humana/sangue , Análise de Variância , Criança , Feminino , Humanos , Influenza Humana/epidemiologia , Influenza Humana/imunologia , Japão/epidemiologia , Masculino , Modelos Biológicos , Índice de Gravidade de Doença
7.
Pediatr Int ; 54(6): 758-61, 2012 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-22726275

RESUMO

BACKGROUND: The severity of the 2009 pandemic H1N1 influenza (H1N1 pdm 09) in immune deficient children is unknown. The aim of the present study was to investigate this in a case of complete IgG3 deficiency complicated by pneumonia and asthma attack. METHODS: The clinical parameters of the IgG3 deficiency patient were compared with those of four control patients using 95% confidence intervals. These control patients were selected from 71 patients admitted due to pneumonia or bronchitis caused by H1N1 pdm 09, and were chosen according to age, absence of pretreatment with oseltamivir before admission, presence of a past history of asthma, use of antibiotics, and combination of inhalation of a beta2 agonist and treatment with i.v. methylprednisolone for asthma attack. RESULTS: The IgG3 deficiency patient had significantly longer duration of admission and period of oseltamivir, with a significantly decreased pulse oxygen saturation and increased maximum serum C-reactive protein, creatine kinase and urinary excretion of ß2-microglobulin/creatinine, compared with the controls (P < 0.05). CONCLUSIONS: Complete IgG3 deficiency is possibly associated with severity of the clinical course of pneumonia and asthma attack in children suffering from H1N1 pdm 09.


Assuntos
Asma/etiologia , Deficiência de IgG/complicações , Vírus da Influenza A Subtipo H1N1 , Influenza Humana/epidemiologia , Pandemias , Pneumonia Viral/etiologia , Adolescente , Asma/epidemiologia , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Deficiência de IgG/epidemiologia , Deficiência de IgG/metabolismo , Incidência , Influenza Humana/complicações , Influenza Humana/diagnóstico , Japão/epidemiologia , Masculino , Pneumonia Viral/epidemiologia , Estudos Retrospectivos , Índice de Gravidade de Doença , Taxa de Sobrevida/tendências
8.
Clin Pediatr Endocrinol ; 16(1): 11-6, 2007.
Artigo em Inglês | MEDLINE | ID: mdl-24790339

RESUMO

A 10-yr-old boy visited Minoh City Hospital complaining of gross hematuria. Laboratory investigations revealed hypercalcemia, hypophosphatemia, and elevated serum levels of parathyroid hormone. A stone was found in the right ureter with drip infusion pyelography. A parathyroid adenoma was successfully diagnosed with computed tomography, ultrasonography, and methoxy-2-isobutyl isonitrile (MIBI) scintigraphy. Multiple endocrine neoplasia was ruled out by normal results of endocrine laboratory examinations. Extracorporeal shock wave lithotripsy was performed to treat the urolithiasis, and the parathyroid adenoma was surgically removed. Primary hyperparathyroidism is rare in childhood; however, this case suggests that gross hematuria is an important sign of hyperparathyroidism.

9.
Clin Calcium ; 15 Suppl 1: 80-2, 2005 Sep.
Artigo em Japonês | MEDLINE | ID: mdl-16272636

RESUMO

We experienced a suspected case of pseudohypoparathyroidism type II. The patient came to our emergency room with no thermal convulsion. The Ellsworth-Howard test was applied to the patient to determine the type of PHP.


Assuntos
Pseudo-Hipoparatireoidismo/diagnóstico , Biomarcadores/urina , Criança , AMP Cíclico/urina , Feminino , Humanos , Hidroxicolecalciferóis/uso terapêutico , Hormônio Paratireóideo , Fósforo/urina , Pseudo-Hipoparatireoidismo/tratamento farmacológico , Pseudo-Hipoparatireoidismo/fisiopatologia , Valores de Referência , beta-N-Acetil-Hexosaminidases/urina
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