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INTRODUCTION: Children with severe motor and intellectual disabilities (SMID) are susceptible to severe lower respiratory tract infection (LTRI). As SMID patients are prone to develop recurrent wheezing and are often diagnosed with bronchial asthma, they frequently receive systemic corticosteroids as an adjunctive treatment for LRTIs. However, the efficacy of corticosteroid therapy for LTRIs in SMID children is unclear. We investigated whether or not corticosteroid therapy was associated with better clinical outcomes for SMID children with LRTIs. METHODS: Our retrospective study enrolled 217 SMID children 1-15 years old hospitalized for LTRIs. We compared the clinical characteristics and outcomes between patients with and without corticosteroid therapy. RESULTS: Of the 217 patients, 29 (13.3%) received corticosteroid therapy. The proportion of patients with a history of bronchial asthma was higher and LRTI was more severe in patients with corticosteroid therapy than in those without the therapy. The length of hospital stay (LOHS) was significantly longer in patients with corticosteroid therapy (median 13 days) than in those without corticosteroid therapy (median 9 days) (P = 0.02). The same tendency was shown for the LOHS in patients with severe or moderate LRTI, although not to a significant extent. CONCLUSION: Systemic corticosteroid therapy was not associated with better clinical outcomes in SMID children with LRTIs, even if the patients suffer from severe LRTIs. Corticosteroids should be used cautiously for LRTIs in SMID children because bronchial asthma is likely to be overdiagnosed in these children.
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Deficiência Intelectual , Infecções Respiratórias , Adolescente , Criança , Pré-Escolar , Hospitalização , Humanos , Lactente , Deficiência Intelectual/complicações , Deficiência Intelectual/tratamento farmacológico , Tempo de Internação , Infecções Respiratórias/complicações , Infecções Respiratórias/tratamento farmacológico , Estudos RetrospectivosRESUMO
Estradiol has an important role in the brain, such as in neuronal development and protection, but estradiol levels in the human brain have not been well investigated. In this study, we measured the estradiol concentration in the cerebrospinal fluid (CSF) of infants to reveal the relationships between the estradiol concentrations in the serum and the CSF and further determined exosomal microRNAs in serum. Estradiol in the CSF was strongly correlated with serum estradiol and moderately correlated with miR-126-5p in the serum exosomes. This report is the first to determine the estradiol concentration in CSF from infants and showed that the levels of miR-126-5p as well as serum estradiol can be candidates to predict brain estrogen status.
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Estradiol/sangue , Estradiol/líquido cefalorraquidiano , Exossomos/metabolismo , MicroRNAs/metabolismo , Biomarcadores/sangue , Biomarcadores/líquido cefalorraquidiano , Pré-Escolar , Feminino , Humanos , Lactente , MasculinoRESUMO
BACKGROUND: The therapeutic range of topiramate (TPM) blood level is not set because the efficacy and safety are not considered to be related to the level. However, the therapeutic target without side effects is necessary, so the optimal range of TPM blood level was analyzed in this study. STUDY QUESTION: This study was conducted to evaluate the efficacy of TPM over 2 years and the utility of measuring blood levels of TPM during the follow-up of epileptic patients. STUDY DESIGN: Thirty patients (18 males, 12 females; age range, 6 months-15 years) were treated with TPM for epilepsy. The initial dosage of TPM was 1-3 mg·kg·d. If the effect proved insufficient after 2 weeks, the dosage was increased to 4-9 mg·kg·d. MEASURES AND OUTCOMES: Blood levels of TPM were measured by liquid chromatography-tandem mass spectrometry at 1, 6, 12, and 24 months after levels reached steady state. The efficacy of TPM was evaluated by the reduction in epileptic seizure rate (RR) at the time of blood sampling. Statistical analysis was performed using the Mann-Whitney U test. RESULTS: A positive correlation was seen between blood levels and maintenance dosages, but no correlation was observed between blood levels and RR. Any significant difference was not identified in TPM levels between the effective group (RR ≥50%) and the ineffective group (RR <50%; P = 0.159). In the subgroup of patients who did not use valproic acid, a significant difference in TPM levels was apparent between the effective and ineffective groups (P = 0.029). The optimal range of TPM was advocated 3.5-5.0 µg/mL. The optimal range was set, so that ranges did not overlap between the effective and ineffective groups. No patients experienced any side effects. CONCLUSIONS: Measuring blood levels of TPM based on the classification of concomitant drugs and adjusting the dosage to reach the optimal range were recommended.
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Anticonvulsivantes/farmacologia , Epilepsia/sangue , Convulsões/sangue , Topiramato/farmacologia , Adolescente , Anticonvulsivantes/sangue , Anticonvulsivantes/uso terapêutico , Criança , Pré-Escolar , Interações Medicamentosas , Quimioterapia Combinada/efeitos adversos , Quimioterapia Combinada/métodos , Epilepsia/tratamento farmacológico , Feminino , Seguimentos , Humanos , Lactente , Masculino , Taxa de Depuração Metabólica/efeitos dos fármacos , Estudos Prospectivos , Convulsões/tratamento farmacológico , Topiramato/sangue , Topiramato/uso terapêutico , Resultado do Tratamento , Ácido Valproico/farmacologia , Ácido Valproico/uso terapêuticoRESUMO
BACKGROUND: Video-assisted thoracoscopic surgery for patent ductus arteriosus (VATS-PDA) is an alternative surgical procedure to open chest surgery, even in premature infants. This study investigated whether the timing of VATS-PDA has a prognostic impact in premature infants whose operative indication was determined according to the symptomatic PDA and the ineffectiveness of or contraindication to indomethacine therapy. METHODS: We studied 49 infants born at or before 28 weeks of gestation who were admitted to the neonatal intensive care unit between January 2004 and June 2016, and who underwent VATS-PDA. The patients were divided into two groups according to median age at the time of surgery (early group, 24 infants who underwent surgery at ≤ 24 days of life; late group, 25 infants who underwent surgery at ≥ 25 days of life). RESULTS: No significant differences were found in bodyweight at 30 days of age and 40 weeks of corrected gestational age between the groups. The timing of surgery did not affect the operative procedure or postoperative complications. In addition, no differences were observed between the early and late groups in terms of complications associated with prematurity, including intraventricular hemorrhage, incidence and severity of bronchopulmonary dysplasia, and necrotizing enteropathy. CONCLUSION: Video-assisted thoracoscopic surgery for patent ductus arteriosus can be safely performed in premature infants without a preferential timing for the intervention, suggesting that this procedure allows for an elective basis approach after heart failure management with conservative and/or drug therapy in premature infants with PDA.
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Permeabilidade do Canal Arterial/cirurgia , Lactente Extremamente Prematuro , Doenças do Prematuro/cirurgia , Cirurgia Torácica Vídeoassistida/métodos , Fatores Etários , Fármacos Cardiovasculares/uso terapêutico , Permeabilidade do Canal Arterial/tratamento farmacológico , Feminino , Humanos , Indometacina/uso terapêutico , Recém-Nascido , Doenças do Prematuro/tratamento farmacológico , Masculino , Prognóstico , Estudos Retrospectivos , Resultado do TratamentoRESUMO
Here we report a case of a 12-year-old girl who was referred to our department because of marked short stature of more than -5 SD below the median. Although her growth failure began suddenly at 6 years of age, she never had an examination because she had no other symptoms. Brain MRI examination suggested a tumor in the suprasellar region, and endocrine examination revealed combined pituitery hormone deficiency due to the tumor. Before surgery, the supplementation with hydrocortisone and levothyroxine was initiated. The pathological diagnosis of the surgically removed tumor was xanthogranuloma. The pattern of her growth curve showed a growth failure with sudden onset, which is a typical pattern of short stature secondary to pituitary disfunction including growth hormone deficiency associated with brain tumors. This case suggests that growth failure could be the only symptom in pediatric cases with brain tumors. Improved awareness regarding the association of growth failure with brain tumors is needed for earlier diagnosis and treatment. Furthermore, the growth curves should be carefully evaluated in regular health examinations at school.
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Estatura , Insuficiência de Crescimento , Transtornos do Crescimento/etiologia , Doenças da Hipófise/complicações , Xantogranuloma Juvenil/complicações , Anormalidades Múltiplas/etiologia , Fatores Etários , Criança , Diagnóstico Precoce , Fácies , Feminino , Transtornos do Crescimento/patologia , Transtornos do Crescimento/prevenção & controle , Humanos , Hipotireoidismo/etiologia , Imageamento por Ressonância Magnética , Exame Físico , Doenças da Hipófise/diagnóstico por imagem , Doenças da Hipófise/patologia , Doenças da Hipófise/cirurgia , Hormônios Adeno-Hipofisários/deficiência , Instituições Acadêmicas , Índice de Gravidade de Doença , Fator de Transcrição Pit-1/deficiência , Xantogranuloma Juvenil/diagnóstico por imagem , Xantogranuloma Juvenil/patologia , Xantogranuloma Juvenil/cirurgiaRESUMO
Neonatal sepsis continues to be a global problem with significant morbidity and mortality, because of the difficulty in predicting its onset with clinical symptoms alone. Thus, the presence of biomarkers is useful for the diagnosis of neonatal sepsis. Presepsin is a 13-kDa truncated form of soluble CD14 that is produced through proteolytic cleavage on activated monocytes. Presepsin, consisting of 64 amino acid residues, has been proposed as a reliable biomarker for the early diagnosis of sepsis in neonates. However, some biomarkers for the diagnosis of sepsis are elevated during the early neonatal period due to physiological variation, whereas such variation in presepsin levels is uncertain. The objective of this study is to investigate the physiological variation in plasma presepsin levels during the early neonatal period. This prospective study included 30 full-term healthy neonates, including 15 neonates delivered by cesarean section. Plasma presepsin levels were examined at birth and on the first day and the fifth day of life in neonates, and the levels on the 5th day of life were lower than those at any other points (P < 0.001). Moreover, there was no significant difference of plasma presepsin levels between neonates delivered vaginally and by cesarean section. The physiological variation in plasma presepsin levels was observed during the early neonatal period. Attention needs to be paid when measuring plasma presespsin levels for the screening of sepsis during the early neonatal period.
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Receptores de Lipopolissacarídeos/sangue , Fragmentos de Peptídeos/sangue , Biomarcadores/sangue , Proteína C-Reativa/metabolismo , Calcitonina/sangue , Demografia , Feminino , Humanos , Recém-Nascido , Contagem de Leucócitos , MasculinoRESUMO
OBJECTIVE: To assess the clinical utility and safety of a strategy for refractory Kawasaki disease, defined by Egami score ≥3. STUDY DESIGN: First-line treatment was with intravenous methylprednisolone (30 mg/kg, 2 hours, 1 dose) plus intravenous immunoglobulin (2 g/kg, 24 hours) treatment. Patients resistant to first-line treatment received additional intravenous immunoglobulin as a second-line treatment. Patients resistant to second-line treatment who had received Bacillus Calmette-Guérin vaccination 6 months earlier were treated with infliximab; otherwise, plasma exchange was performed. A total of 71 refractory patients with Kawasaki disease (median age: 2.4 years) of 365 patients with Kawasaki disease were treated according to our strategy from April 2007 to April 2016. Treatment resistance was defined as a persistent fever at 36 hours after treatment. We evaluated coronary artery lesions at the time of the diagnosis, at 1 month, and at 1 year after the diagnosis in accordance with the American Heart Association guidelines and the criteria of the Japanese Ministry of Health, Labour, and Welfare. RESULTS: First-line therapy was effective for 58 of 71 patients (81.6%), and second-line therapy was effective for 9 of 13 patients (69.2%). At third line, 3 patients were treated by infliximab, and 1 was treated with plasma exchange. Of the 18 patients with coronary artery abnormalities at diagnosis, 13 patients at 1 month and 6 patients at 1 year had coronary artery dilatation (median z score 3.0, 2.6, and 1.4, respectively). There were no patients with coronary artery aneurysm (CAA). CONCLUSIONS: Our strategy for refractory Kawasaki disease was safe and effective in preventing CAA.
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Anti-Inflamatórios/uso terapêutico , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Infliximab/uso terapêutico , Metilprednisolona/uso terapêutico , Síndrome de Linfonodos Mucocutâneos/terapia , Troca Plasmática , Doença Aguda , Criança , Pré-Escolar , Protocolos Clínicos , Terapia Combinada , Aneurisma Coronário/etiologia , Aneurisma Coronário/prevenção & controle , Esquema de Medicação , Quimioterapia Combinada , Feminino , Humanos , Lactente , Injeções Intravenosas , Masculino , Síndrome de Linfonodos Mucocutâneos/complicações , Resultado do TratamentoRESUMO
This study was conducted to evaluate the effectiveness of lamotrigine (LTG) over 2 years and the usefulness of measuring its blood levels during the follow-up of patients with epilepsy. We measured peak blood LTG levels of 32 patients with epilepsy (9.16 ± 3.34 years old; mean ± SD). The blood levels were measured at 6 months, 1 year, and 2 years after reaching the LTG maintenance dosage. The effectiveness of LTG was evaluated to determine the seizure reduction rate. The patients were classified as effective cases (mean of own seizure reduction rates >50%) and ineffective cases (≤50%). The results were that the dosage and blood level showed positive correlations in the case of combination use with sodium valproate (VPA) (r = 0.690), carbamazepine and/or phenobarbital (r = 0.940), and others (r = 0.548). In several groups, the blood levels and efficacies did not show any positive correlations. In the cases of combination use with VPA, the blood levels of effective cases and ineffective cases were significantly different (P = 0.001). The optimal range was 8-11.5 µg/mL based on the average and SD values in the effective cases. No patients had any side effects. In conclusion, no precise definition of the therapeutic range was possible because of the incomplete correlation between the blood level and seizure frequency. We recommend the optimal range of LTG as a therapeutic target without any side effects, and it was established that the range in the combination with VPA was 8-11.5 µg/mL.
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Anticonvulsivantes/uso terapêutico , Epilepsia/tratamento farmacológico , Convulsões/tratamento farmacológico , Triazinas/uso terapêutico , Adolescente , Anticonvulsivantes/sangue , Anticonvulsivantes/farmacologia , Carbamazepina/farmacologia , Carbamazepina/uso terapêutico , Criança , Pré-Escolar , Relação Dose-Resposta a Droga , Interações Medicamentosas , Quimioterapia Combinada/métodos , Epilepsia/sangue , Epilepsia/epidemiologia , Feminino , Humanos , Lamotrigina , Masculino , Fenobarbital/farmacologia , Fenobarbital/uso terapêutico , Estudos Prospectivos , Convulsões/sangue , Convulsões/epidemiologia , Resultado do Tratamento , Triazinas/sangue , Triazinas/farmacologia , Ácido Valproico/farmacologia , Ácido Valproico/uso terapêuticoRESUMO
Clobazam (CLB) is an antiepileptic drug that is metabolized to the major metabolite N-desmethylclobazam (N-CLB). Our aim was to evaluate the utility of corrected urinary concentrations of CLB and N-CLB in Japanese children and adolescents with epilepsy. Blood and urinary concentrations of CLB and N-CLB were evaluated in 42 patients. The urinary and peak blood concentrations were measured 2-3 h after the last dose. The ratio of the blood and urinary creatinine concentrations was used to calculate the corrected urinary concentrations. A moderate correlation was found between the CLB dose and the CLB serum concentration, but this correlation was not found for N-CLB. Patients were dichotomized based on two regression lines, which were detected by statistical analyses with a cumulative distribution function: the lower ratio group (CLB/N-CLB < 0.275) and the higher ratio group (≥0.275). Moderate correlations were observed between the CLB dose and the serum concentration or the corrected value of CLB for the lower ratio group, and moderate to strong correlations were observed for the higher ratio group. The corrected urinary concentration of CLB correlates to the CLB dose when stratified by the CLB/N-CLB ratio and may prove practical for clinical estimation of the CLB serum concentration.
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Benzodiazepinas/sangue , Benzodiazepinas/urina , Epilepsia Resistente a Medicamentos/sangue , Epilepsia Resistente a Medicamentos/urina , Administração Oral , Adolescente , Anticonvulsivantes/administração & dosagem , Anticonvulsivantes/sangue , Anticonvulsivantes/urina , Povo Asiático , Benzodiazepinas/administração & dosagem , Benzodiazepinas/farmacocinética , Criança , Pré-Escolar , Clobazam , Epilepsia Resistente a Medicamentos/tratamento farmacológico , Feminino , Humanos , Masculino , Adulto JovemRESUMO
BACKGROUND: A Japanese nationwide survey has reported that Down syndrome (DS) is a less-frequently occurring comorbidity in Kawasaki disease (KD). Although altered immune responses are frequently observed in DS, no studies have focused on the treatment response and risk for coronary artery abnormalities (CAA) in DS patients with KD. The aim of this study was therefore to evaluate the clinical manifestations, treatment response and prevalence of CAA in DS with KD. METHODS: We retrospectively reviewed the medical records of DS patients with KD from 2005 through 2012. The survey questionnaires were sent to facilities nationwide, and clinical data regarding KD in DS were collected. A control group consisted of non-DS patients with KD who were managed at Toho University. RESULTS: Of the 94 233 children diagnosed with acute KD from 2005 to 2012, 16 children with acute KD also had DS (0.017%). The DS-KD patients were significantly older than the non-DS patients (median, 8 years vs 1 year, P < 0.05, respectively). Half of the DS patients had incomplete KD. Although 50% of the DS children were at high risk of immunoglobulin resistance, all children responded to initial treatment and none had CAA. CONCLUSIONS: All DS-KD patients responded to initial i.v. immunoglobulin (IVIG) or aspirin despite having a high risk of IVIG resistance, and none of the DS patients had CAA. This suggests that the risk of treatment resistance and development of CAA may be not higher in DS patients with acute KD.
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Anomalias dos Vasos Coronários/epidemiologia , Síndrome de Down/epidemiologia , Resistência a Medicamentos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Síndrome de Linfonodos Mucocutâneos/tratamento farmacológico , Criança , Pré-Escolar , Comorbidade , Anomalias dos Vasos Coronários/diagnóstico , Feminino , Humanos , Lactente , Japão/epidemiologia , Masculino , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/epidemiologia , Prevalência , Estudos Retrospectivos , Fatores de Risco , Resultado do TratamentoRESUMO
Recent studies revealed that obesity is a low-grade, chronic inflammatory state that is accompanied by the enhanced production of multiple chemokines. In particular, metabolic syndrome (MS) and visceral adipose tissue (VAT) accumulation are significantly associated with certain chemokines in adults. However, little is known regarding this association in obese children. The aim of this study was to investigate the relationship between circulating chemokine levels and both MS and VAT accumulation in obese children. Forty-four obese schoolchildren (26 boys) with a percentage of overweight (POW) exceeding 20 were evaluated. The median age was 11.4 years (range: 6.8-16.5 years). Blood samples were drawn after overnight fasting, and serum chemokine levels (CCL2, CCL5 and CXCL10) were quantitated. Visceral fat area (VFA) determinations were conducted using computed tomography. The results showed that the median BMI Z-score, POW, waist circumference and VFA of the subjects were 2.24 SD, 49.8%, 88.3 cm and 80.8 cm2, respectively. Eighteen were diagnosed with MS. CCL2 was significantly increased in MS subjects compared with non-MS subjects (p<0.05). CXCL10 was positively correlated with VFA (r=0.425, p<0.01). There were no significant correlations between age and chemokine levels. We showed that CCL2 levels were elevated in MS and CXCL10 levels were associated with VFA in obese children. Our results suggest that CCL2 and CXCL10 play important roles in the progression of obesity-related metabolic complications in children.
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Quimiocina CCL2/sangue , Quimiocina CXCL10/sangue , Gordura Intra-Abdominal/patologia , Síndrome Metabólica/sangue , Obesidade Infantil/sangue , Adolescente , Estudos de Casos e Controles , Criança , Progressão da Doença , Feminino , Humanos , Masculino , Síndrome Metabólica/complicações , Síndrome Metabólica/patologia , Tamanho do Órgão , Obesidade Infantil/complicações , Obesidade Infantil/patologiaRESUMO
We herein describe a fetal case of arrhythmogenic right ventricular cardiomyopathy (ARVC) with double mutations in transmembrane protein 43 (TMEM43). RV aneurysm and ventricular arrhythmia were detected during the fetal period. After birth, electrocardiogram showed frequent premature ventricular contractions (PVC) of left bundle branch block morphology and epsilon waves in the right-sided chest leads. Echocardiography also indicated RV aneurysm with regionally decreased systolic function. PVC disappeared after treatment with amiodarone and mexiletin. Mutations in TMEM43, which was recently identified as the causative gene of ARVC type 5, were also confirmed in the present patient and in the patient's mother, and they were therefore diagnosed with ARVC. The present case confirms that symptoms of ARVC can emerge during the fetal period. Pediatricians need to keep in mind the possibility of ARVC when they encounter patients with RV aneurysm and arrhythmia.
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BACKGROUND: The best time for vaccination in infants with congenital heart disease (CHD) after cardiopulmonary bypass (CPB) surgery is unclear, but it is important to prevent Haemophilus influenzae type b (Hib) infection in infants with CHD after CPB surgery. To identify the best time for Hib vaccination in infants with CHD after CPB surgery, we investigated the immunological status, and the efficacy and safety of Hib vaccination after CPB surgery. METHODS: Sixteen subjects who underwent surgical correction of ventricular septal defect with CPB were investigated. Immunological status and cytokines were analyzed before surgery, 2 months after surgery, and before Hib booster vaccination. Hib-specific IgG was also measured to evaluate the effectiveness of vaccination. RESULTS: Immunological status before and 2 months after surgery (e.g. whole blood cells and lymphocyte subset profile) was within the normal range and no subjects had hypercytokinemia. Additionally, all subjects who received Hib vaccination at 2-3 months after CPB surgery had effective serum Hib-specific IgG level for protection against Hib infection without any side-effects. CONCLUSIONS: CPB surgery does not influence acquired immunity and Hib vaccination may be immunologically safe to perform at 2 months after CPB surgery. Hib vaccination at 2-3 months after CPB surgery was effective in achieving immunization for infants with simple left-right shunt-type CHD.
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Procedimentos Cirúrgicos Cardíacos , Infecções por Haemophilus/prevenção & controle , Haemophilus influenzae tipo b/imunologia , Cardiopatias Congênitas/cirurgia , Imunidade Inata , Feminino , Seguimentos , Infecções por Haemophilus/imunologia , Humanos , Lactente , Recém-Nascido , Masculino , Prognóstico , Estudos Retrospectivos , Fatores de Tempo , VacinaçãoRESUMO
Pseudohypoparathyroidism type 1b (PHP-1b) is usually diagnosed on various symptoms of hypocalcemia. Previous studies reported a few cases of autosomal dominant pattern PHP-1b identified on familial analysis with asymptomatic hypocalcemia. Herein we report the case of a 6-year-old male patient with sporadic PHP-1b incidentally detected on preoperative examination. He had neither characteristic findings of Albright hereditary osteodystrophy nor evidence of tetany. Sporadic PHP-1b was diagnosed on the basis of clinical observation and laboratory examination. In addition, genetic testing using methylation-specific multiplex ligation-dependent probe amplification indicated broad methylation abnormalities and confirmed the sporadic form of PHP-1b. Sporadic PHP-1b might often be overlooked when diagnosis is done simply on definitive clinical features. To avoid this, DNA sequencing and methylation analysis should be performed even in the absence of definitive clinical features.
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Cromograninas/genética , DNA/genética , Subunidades alfa Gs de Proteínas de Ligação ao GTP/genética , Hipocalcemia/etiologia , Pseudo-Hipoparatireoidismo/diagnóstico , Criança , Cromograninas/metabolismo , Análise Mutacional de DNA , Subunidades alfa Gs de Proteínas de Ligação ao GTP/metabolismo , Testes Genéticos , Humanos , Hipocalcemia/sangue , Hipocalcemia/diagnóstico , Masculino , Pseudo-Hipoparatireoidismo/complicações , Pseudo-Hipoparatireoidismo/genética , Pseudo-HipoparatireoidismoRESUMO
Background Several biomarkers for the diagnosis of sepsis are elevated during the early neonatal period due to physiological variations. The aim of this study was to investigate the physiological variation in neutrophil CD64 (nCD64) expression during the early neonatal period and the change in nCD64 expression in neonates with noninfectious diseases. Methods Of 71 neonates enrolled in this prospective study, 5 and 51 were diagnosed as having bacteremia and noninfectious diseases, respectively. Fifteen healthy neonates were enrolled as normal controls. Peripheral white blood cell counts, serum C-reactive protein and procalcitonin levels, and nCD64 expression were examined at birth and on the first and fifth day of life in neonates with noninfectious diseases and healthy neonates. In neonates with bacteremia, these markers were measured at onset. Results nCD64 expression was significantly higher in neonates with bacteremia (median, 1,992) than in those with noninfectious diseases (1,823, p < 0.001) and healthy neonates (1,848, p = 0.002). Unlike other biomarkers, no differences in nCD64 expression were observed on the same days between neonates with noninfectious diseases and healthy neonates. Conclusion nCD64 expression may be a useful marker for the diagnosis of bacterial infection in the early neonatal period, because it does not show any physiological variations.
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Bacteriemia/sangue , Sepse Neonatal/diagnóstico , Neutrófilos/metabolismo , Receptores de IgG/fisiologia , Biomarcadores/sangue , Proteína C-Reativa/análise , Calcitonina/sangue , Estudos de Casos e Controles , Feminino , Humanos , Recém-Nascido , Japão , Contagem de Leucócitos , Masculino , Sepse Neonatal/sangue , Doenças não Transmissíveis , Estudos ProspectivosRESUMO
The introduction of foetoscopic laser photocoagulation has dramatically improved the prognosis of patients with severe twin-to-twin transfusion syndrome. We present the case of a donor who exhibited right-heart failure with a high echoic wall change of the right ventricle after the foetoscopic laser photocoagulation procedure. The prenatal and 1-year postnatal follow-up revealed the gradual recovery of the right ventricular function.
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Transfusão Feto-Fetal/complicações , Fetoscopia/efeitos adversos , Insuficiência Cardíaca/etiologia , Fotocoagulação a Laser/efeitos adversos , Ultrassonografia Pré-Natal , Disfunção Ventricular Direita/etiologia , Feminino , Insuficiência Cardíaca/diagnóstico por imagem , Humanos , Gravidez , Disfunção Ventricular Direita/diagnóstico por imagemRESUMO
The use of measured data as boundary conditions renders hemodynamic simulations more patient-specific. However, synchronized acquisition of data at multiple locations is often difficult in clinical practice. This study proposes a method for resynchronizing measured data for use as boundary conditions for flow simulations using frequency analyses, and discusses the optimal cut-off frequency for differentiating cardiac and respiratory variation in hemodynamic data during resynchronization. To demonstrate the utility of the method, a Fontan circulation, which is the final palliative result with single-ventricle physiology, was used. The results suggest that it is optimal to set a cut-off frequency that gives a local minimum in the power spectrum that is slightly lower than the peak frequency of the heartbeat. Additionally, the total energy loss depended on the cut-off frequency, although the overall flow patterns appeared to be similar. The method is applicable to cardiovascular systems other than the Fontan circulation, where hemodynamic data with multifactorial fluctuations are required at various locations but simultaneous measurements are not possible.
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Simulação por Computador , Técnica de Fontan , Hemodinâmica , Ventilação Monopulmonar , Desenho de Equipamento , Humanos , Modelos Cardiovasculares , Ventilação Monopulmonar/métodosRESUMO
Clinicians are currently encountering an increasing number of patients in the long-term period after tetralogy of Fallot (TOF) repair presenting with pulmonary valve regurgitation (PR) or right ventricular (RV) dysfunction. The purpose of this study was to evaluate the clinical utility of the plasma brain natriuretic peptide (BNP) level and consider surgical indications and timing of pulmonary valve replacement (PVR). We examined 33 patients (21 males, 12 females, mean age 14.5 ± 2.8 years) who underwent TOF repair at Kitasato University Hospital. All patients were evaluated using echocardiography and blood sampling. The mean age at the time of initial repair was 1.3 ± 0.7 years. The patients with moderate-severe PR exhibited significantly higher plasma BNP levels than the patients with trivial-mild PR (mean 37.5 ± 33.1 vs. 17.3 ± 6.6 pg/ml, p = 0.013). The mean plasma BNP level with cardiac symptoms was higher than that observed in the patients without any symptoms (71.4 ± 46.1 vs. 25.0 ± 14.0 pg/ml, p = 0.005). The mean BNP level was significantly decreased after PVR (71.3 ± 46.1-26.1 ± 13.2 pg/ml, p = 0.009), and the plasma BNP level was found to be positively correlated with the RV end-diastolic pressure (r = 0.851; p = 0.008). The optimal BNP cut-off value for considering PVR was 32.15 pg/ml (sensitivity, 85.7 %; specificity, 83.3 %). The plasma BNP level may become a useful diagnostic tool for considering the indications and optimal timing of PVR over the long term after TOF repair.
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Peptídeo Natriurético Encefálico/sangue , Insuficiência da Valva Pulmonar/sangue , Valva Pulmonar/cirurgia , Tetralogia de Fallot/sangue , Tetralogia de Fallot/cirurgia , Adolescente , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/métodos , Criança , Ecocardiografia , Feminino , Implante de Prótese de Valva Cardíaca/efeitos adversos , Implante de Prótese de Valva Cardíaca/métodos , Humanos , Masculino , Valva Pulmonar/fisiopatologia , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Insuficiência da Valva Pulmonar/fisiopatologia , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/fisiopatologia , Fatores de Tempo , Resultado do TratamentoRESUMO
It has been established that a cationic rhodium(I)/H8-binap complex is able to catalyze the [2+2+2] cycloaddition of diynes with carbodiimides and carbon dioxide under ambient conditions. Enantio- and/or regioselective variants of these reactions are also disclosed.
RESUMO
BACKGROUND: Infliximab (IFX), a known monoclonal antibody against tumor necrosis factor-α (TNF-α), is used to treat Kawasaki disease (KD) patients with intravenous immunoglobulin (IVIG) resistance. The transcriptional modulation of inflammation following IFX therapy has not been reported in KD patients. METHODS: We investigated the transcript abundance profiles in whole blood obtained from eight IVIG-resistant KD subjects treated with IFX therapy using microarray platforms and compared them with those in initially IVIG-responsive subjects. A pathway analysis was performed using WikiPathways to search for the biological pathways of the transcript profiles. Four transcripts changed by IFX therapy were subsequently validated using quantitative real-time polymerase chain reaction. RESULTS: The pathway analysis showed the reduced abundance of transcripts in the nucleotide-binding oligomerization domain, matrix metalloproteinase (MMP), and inflammatory cytokine pathways and the increased abundance of transcripts in the T-cell receptor, apoptosis, TGF-ß, and interleukin-2 pathways. Additionally, the levels of four transcripts (peptidase inhibitor-3, MMP-8, chemokine receptor-2, and pentraxin-3) related to KD vasculitis and IVIG resistance decreased after IFX therapy. CONCLUSION: The administration of IFX was associated with both the signaling pathways of KD inflammation and several transcripts related to IVIG resistance factors. These findings provide strong theoretical support for the use of IFX in KD patients with IVIG resistance.