RESUMO
Botrytis cinerea is a necrotrophic pathogen that infects across a broad range of plant hosts, including high-impact crop species. Its generalist necrotrophic behavior stems from its ability to detoxify structurally diverse phytoalexins. The current study aims to provide evidence of the ability of B. cinerea to tolerate the sesquiterpenoid phytoalexin rishitin, which is produced by potato and tomato. While the growth of potato pathogens Phytophthora infestans (late blight) and Alternaria solani (early blight) was severely inhibited by rishitin, B. cinerea was tolerant to rishitin. After incubation of rishitin with the mycelia of B. cinerea, it was metabolized to at least six oxidized forms. Structural analysis of these purified rishitin metabolites revealed a variety of oxidative metabolism including hydroxylation at C7 or C12, ketone formation at C5, and dihydroxylation at the 10,11-olefin. Six rishitin metabolites showed reduced toxicity to P. infestans and A. solani, indicating that B. cinerea has at least 5 distinct enzymatic reactions to detoxify rishitin. Four host-specialized phytopathogenic Botrytis species, namely B. elliptica, B. allii, B. squamosa, and B. tulipae also had at least a partial ability to metabolize rishitin as B. cinerea, but their metabolic capacity was significantly weaker than that of B. cinerea. These results suggest that the ability of B. cinerea to rapidly metabolize rishitin through multiple detoxification mechanisms could be critical for its pathogenicity in potato and tomato.
Assuntos
Botrytis , Fitoalexinas , Phytophthora infestans , Doenças das Plantas , Sesquiterpenos , Botrytis/metabolismo , Botrytis/genética , Botrytis/efeitos dos fármacos , Sesquiterpenos/metabolismo , Doenças das Plantas/microbiologia , Phytophthora infestans/metabolismo , Phytophthora infestans/genética , Phytophthora infestans/crescimento & desenvolvimento , Phytophthora infestans/efeitos dos fármacos , Solanum lycopersicum/microbiologia , Inativação Metabólica , Alternaria/metabolismo , Alternaria/genética , Redes e Vias Metabólicas , Solanum tuberosum/microbiologiaRESUMO
BACKGROUND: Endoscopic monitoring of disease activity in patients with ulcerative colitis (UC) is important. However, frequent colonoscopic examinations are difficult to perform because of their invasiveness, especially in children. Bowel wall thickness (BWT) measurement using intestinal ultrasonography and fecal calprotectin (FC) measurement are useful noninvasive evaluation methods. METHODS: We retrospectively analyzed BWT and FC levels and evaluated the Mayo endoscopic subscore (MES) using colonoscopy in pediatric patients with UC during the same period. The BWT was evaluated using the maximum BWT (mBWT), which was the maximum value of each colonic BWT; the sum of BWT (sBWT), which was the sum of each colonic BWT; and the sum of the adjusted BWT (saBWT), which was corrected using sBWT. RESULTS: In 54 procedures from 40 patients, FC, mBWT, sBWT, and saBWT were significantly different between MES 0-1 and MES 2. The agreement between BWT and MES 2 was 193 out of 216 segments (89.4%). Sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of FC were 68.8%, 84.2%, 64.7%, 86.5%, and 79.6% respectively, while those of saBWT were 81.2%, 89.5%, 76.5%, 91.9%, 87.0%, respectively. CONCLUSIONS: BWT in each colonic segment, particularly saBWT, was more useful than FC for detecting moderate colonic inflammation (MES 2) in pediatric patients with UC. Therefore, intestinal ultrasonography may be helpful in the less invasive management of pediatric patients with UC.
Assuntos
Colite Ulcerativa , Humanos , Criança , Colite Ulcerativa/diagnóstico por imagem , Estudos Retrospectivos , Biomarcadores , Colonoscopia , Fezes/química , Ultrassonografia , Índice de Gravidade de Doença , Mucosa IntestinalRESUMO
BACKGROUND: Glucocorticoids (GCs) are highly effective yet problematic agents against bronchopulmonary dysplasia (BPD). The dimeric trans-activation of GCs induces unfavorable effects, while monomeric trans-repression suppresses inflammation-related genes. Recently, non-steroidal-selective glucocorticoid-receptor agonists and modulators (SEGRAMs) with only the trans-repressive action have been designed. METHODS: Using a bleomycin (Bleo)-induced alveolar simplification newborn rat model (recapitulating arrested alveolarization during BPD), we evaluated the therapeutic effects of compound-A (CpdA), a SEGRAM. Sprague-Dawley rats were administered Bleo from postnatal day (PD) 0 to 10 and treated with dexamethasone (Dex) or CpdA from PD 0 to 13. The morphological changes and mRNA expression of inflammatory mediators, including interleukin (IL)-1ß, C-X-C motif chemokine ligand 1 (CXCL1), and C-C motif chemokine 2 (CCL2) were investigated. RESULTS: Similar to the effects of Dex, CpdA exerted protective effects on morphological derangements and inhibited macrophage infiltration and production of pro-inflammatory mediators in Bleo-treated animals. The effects of CpdA were probably mediated by GC receptor (GR)-dependent trans-repression, because unlike the Dex-treated group, anti-inflammatory genes specifically induced by GR-dependent trans-activation (such as "glucocorticoid-induced leucine zipper, GILZ") were not upregulated. CONCLUSIONS: CpdA improved lung inflammation, inhibited the arrest of alveolar maturation, and restored histological and biochemical changes in a Bleo-induced alveolar simplification model. IMPACT: SEGRAMs have attracted widespread attention because they are expected to not exhibit unfavorable effects of GCs. Compound A, one of the SEGRAMs, improved lung morphometric changes and decreased lung inflammation in a bleomycin-induced arrested alveolarization, a newborn rat model representing one of the main features of BPD pathology. Compound A did not elicit bleomycin-induced poor weight gain, in contrast to dexamethasone treatment. SEGRAMs, including compound A, may be promising candidates for the therapy of BPD with less adverse effects compared with GCs.
Assuntos
Glucocorticoides , Receptores de Glucocorticoides , Ratos , Animais , Glucocorticoides/farmacologia , Receptores de Glucocorticoides/genética , Animais Recém-Nascidos , Dexametasona/farmacologia , Bleomicina , Ratos Sprague-Dawley , QuimiocinasRESUMO
BACKGROUND: Eculizumab was approved for atypical haemolytic uraemic syndrome (aHUS) in Japan in 2013. Post-marketing surveillance (PMS) was mandated by regulatory authorities to assess the safety and effectiveness of eculizumab in patients with aHUS in a real-world setting. METHODS: Paediatric patients in the PMS cohort who were <18 years of age at the first administration of eculizumab and diagnosed with aHUS [excluding Shiga toxin-producing Escherichia coli HUS, thrombotic thrombocytopaenic purpura and secondary thrombotic microangiopathy (TMA)] were included in the effectiveness and safety analysis. Clinical endpoints of effectiveness [complete TMA response, TMA event-free status, platelet (PLT) count and lactate dehydrogenase (LDH) normalization, serum creatinine (sCr) decrease and estimated glomerular filtration rate (eGFR) improvement] were analysed in patients treated with at least one dose of eculizumab. Serious adverse events (SAEs) were also evaluated. RESULTS: A total of 40 paediatric patients (median age 5 years) were included. The median eculizumab treatment duration was 66 weeks. PLT count, LDH and eGFR significantly improved at 10 days post-treatment. Complete TMA response, haematologic normalization, sCr decrease, eGFR improvement and TMA event-free status were achieved by 73.3%, 73.3%, 70.0%, 78.3% and 77.5% of patients, respectively. Discontinuation criteria were met by 18 patients: 13 patients maintained treatment discontinuation at the end of observation and 5 patients, including 1 patient with aHUS relapse, continued the treatment but extended the treatment interval. During eculizumab treatment, 59 SAEs (0.66/person-year) were reported. Although four deaths were reported, none of them were related to eculizumab. CONCLUSION: Eculizumab was well tolerated and effective for paediatric patients with aHUS in the real-world setting in Japan.
Assuntos
Síndrome Hemolítico-Urêmica Atípica , Microangiopatias Trombóticas , Humanos , Criança , Pré-Escolar , Síndrome Hemolítico-Urêmica Atípica/tratamento farmacológico , Síndrome Hemolítico-Urêmica Atípica/diagnóstico , Japão , Anticorpos Monoclonais Humanizados/efeitos adversos , Microangiopatias Trombóticas/complicações , Vigilância de Produtos Comercializados , Inativadores do Complemento/efeitos adversosRESUMO
BACKGROUND: Limited data are available on the survival and causes of death in pediatric patients with chronic kidney disease (CKD) stage 5 receiving kidney replacement therapy (KRT) in Asia. METHODS: Data were obtained from the Japanese nationwide cross-sectional CKD stage 5 survey on pediatric patients (<20 years of age) who started KRT from 2006 to 2013. The cohort was divided into three groups according to age at the start of KRT: <1, 1-5, and 6-19 years. RESULTS: Among the 701 children who were included, 59.3% were boys. Peritoneal dialysis was the most common initial modality of KRT (60.3%). Median age at KRT initiation was 10.2 years. Infants (<1 year old) accounted for 16.0% of the total cohort. Overall survival at 1 and 5 years was 97.2% and 92.5%, respectively. Infants had significantly lower survival rates than the other groups (hazard ratio, 5.35; 95% CI, 2.60-11.03; P < 0.001). In contrast, after the age of 1 year, the survival rate improved and did not differ from that of other age groups. The most common causes of death were infection (35.9%) and sudden death (15.4%). CONCLUSIONS: The overall survival rate of pediatric patients with CKD stage 5 in Japan is like that in other high-income countries. Age at initiation of KRT is an important factor affecting survival since the poorest survival rate was observed in infants. Further improvement in infant dialysis therapy is still needed to improve survival of the youngest children. A higher resolution version of the Graphical abstract is available as Supplementary information.
Assuntos
Falência Renal Crônica , Transplante de Rim , Lactente , Masculino , Humanos , Criança , Feminino , Estudos de Coortes , Estudos Transversais , População do Leste Asiático , Transplante de Rim/efeitos adversos , Terapia de Substituição Renal , Falência Renal Crônica/etiologia , Análise de SobrevidaRESUMO
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is an ultra-rare and life-threatening disease. For decades, plasma therapy was used to manage patients with aHUS. Since eculizumab, a recombinant humanized anti-C5 monoclonal antibody, was approved for treatment of aHUS, it has been used to treat patients with aHUS. Here, we examined the effectiveness of eculizumab and plasma therapy, respectively in the treatment of pediatric patients with aHUS. METHODS: Data were collected from questionnaires sent to 75 institutions known to be treating thrombotic microangiopathy (TMA). RESULTS: A total of 24 patients were evaluable, in which no recurrence of TMA was reported at last observation. There were four therapy groups: two patients receiving supportive therapy, one receiving plasma therapy alone, 17 switching from plasma therapy to eculizumab (therapy switched), and four receiving eculizumab alone. Among 17 patients of therapy-switched group, only one patient achieved complete remission at the end of plasma therapy, 15 patients achieved complete remission after eculizumab initiation, and two patients reached end-stage renal disease. Adverse events were reported in nine cases; among these, meningococcal infection, anaphylaxis, and eculizumab-related infusion reaction were reported among those treated with eculizumab. CONCLUSION: This study provided substantial evidence from a Japanese population that the conversion from plasma therapy to eculizumab therapy should be considered in patients with aHUS who show an incomplete response to plasma therapy. In addition, although no new safety events were detected, careful attention should be paid to meningococcal infection, eculizumab-related infusion reactions and allergic reactions with administration of eculizumab.
Assuntos
Síndrome Hemolítico-Urêmica Atípica , Falência Renal Crônica , Microangiopatias Trombóticas , Humanos , Criança , Síndrome Hemolítico-Urêmica Atípica/tratamento farmacológico , Anticorpos Monoclonais Humanizados/efeitos adversos , Microangiopatias Trombóticas/induzido quimicamenteRESUMO
Atherosclerosis can develop in adult patients with congenital heart disease (CHD) and should be given attention. Endothelial function is well known as a predictor of the development of atherosclerosis but has not been well investigated in patients with repaired CHD. This study aimed to clarify the endothelial function and its relationship with clinical backgrounds and parameters in adolescents with various types of repaired CHDs. Endothelial function was evaluated using peripheral arterial tonometry (PAT). The reactive hyperemia index (RHI) was evaluated and compared between adolescents with repaired CHD and those in the control group. The relationship between the clinical background and parameters was also investigated in patients with repaired CHD. Forty-eight patients with repaired CHD (age 14.0 ± 3.3 years) and 114 healthy volunteers were included in this study. Patients with repaired CHD comprised 16 with repaired non-cyanotic CHD, 14 with repaired tetralogy of Fallot, and 18 who underwent the Fontan procedure. RHI in the repaired CHD group was significantly lower than in the control group. There was no significant correlation between the RHI and blood biochemical markers, such as uric acid, creatine, and brain natriuretic peptide levels. The RHI was significantly higher in patients taking angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs) than in those not taking them. Endothelial function was impaired in adolescents with repaired CHD compared to that in the control group. Microvascular endothelial dysfunction developed even in adolescents with simple non-cyanotic CHD.
RESUMO
We present a case of Gorlin-Goltz syndrome (GGS) in a patient who developed medulloblastoma, osteosarcoma, myelodysplastic syndrome, basal cell carcinoma, and odontogenic keratocyst by the age of 19 years. He had no known family history and no characteristic physical features of GGS. A frameshift mutation in the PTCH1 gene was found in the oral mucosa as a low-frequency mosaicism, basal cell carcinoma, and normal skin by whole exome sequencing of cancer susceptibility genes. Setting a therapeutic strategy with regard to second cancer development is important for pediatric cancer patients who have a background of cancer predisposition. Advances in comprehensive multigenetic analysis are anticipated to aid in developing such a strategy.
Assuntos
Síndrome do Nevo Basocelular , Carcinoma Basocelular , Neoplasias Cerebelares , Meduloblastoma , Neoplasias Cutâneas , Adulto , Síndrome do Nevo Basocelular/diagnóstico , Síndrome do Nevo Basocelular/genética , Carcinoma Basocelular/genética , Carcinoma Basocelular/patologia , Criança , Humanos , Masculino , Adulto JovemRESUMO
BACKGROUND: Ritodrine and magnesium sulfate are administered to prevent preterm labor. Magnesium sulfate is also administered to prevent preeclampsia. These drugs have been reported to increase potassium levels in pregnant women and neonates. The aim of this study was to investigate the relationship between potassium levels in preterm infants and antenatal treatment. METHODS: This prospective cohort study was conducted at Saiseikai Suita Hospital. Preterm infants born at <35 weeks' gestation between October 2012 and September 2014 were recruited and divided into four groups based on the antenatal treatment their mothers received. Serum and urine electrolyte levels at birth and serum potassium levels 1 day after birth were measured. RESULTS: The mothers of 16 infants received no antenatal treatment (condition C); the mothers of 29 infants received antenatal ritodrine (R); the mothers of seven infants received magnesium sulfate (M); and the mothers of 15 infants received both magnesium sulfate and ritodrine (M + R). At birth, potassium levels were similar among the four groups. However, potassium levels a day after birth were significantly higher in the M + R group than in the other groups: median (min.-max.) mEq/L 4.8 (3.8-6.2), 4.8 (3.6-6.0), and 4.4 (3.8-5.9) vs. 5.8 (4.9-7.2), in the C, R, and M groups versus the M + R group, respectively (P < 0.01). Significantly more infants in the M + R group exhibited a fractional excretion of potassium of <10% compared with those in the other groups. CONCLUSION: The increased potassium levels we observe in preterm infants of mothers who received antenatal magnesium sulfate and ritodrine administration on postnatal day 1 warrant monitoring by neonatologists.
Assuntos
Ritodrina , Lactente , Recém-Nascido , Feminino , Gravidez , Humanos , Ritodrina/uso terapêutico , Recém-Nascido Prematuro , Sulfato de Magnésio/uso terapêutico , Sulfatos , Estudos de Coortes , Estudos Prospectivos , PotássioRESUMO
BACKGROUND: This study investigated the impact of coronavirus disease 2019 pandemic school closures on the mental health of school students with chronic diseases. METHODS: Questionnaires were distributed to students from 4th-9th grade diagnosed with chronic diseases at Osaka Medical College Hospital and their parents or caregivers. Questionnaires from 286 families were returned by mail after the schools reopened. The students were divided into the "psychosomatic disorder" group (group P, n = 42), the "developmental disorder" group (group D, n = 89), and the "other disease" group (group O, n = 155). Using students' self-reports on the Questionnaire for Triage and Assessment with 30 items, we assessed the proportion of students with a high risk of psychosomatic disorder in three groups. We investigated how the students requiring the support of somatic symptom (SS) felt about school during school closure. Further, using parents' and caregivers' answers, SS scores were calculated before and during school closure and after school reopening. RESULTS: The proportion of students with a high risk of developing psychosomatic disorder increased in all groups. For the students in Group P, the response "I did not want to think about school" indicated an increase in SS scores, and for the students in Group O, "I wanted to get back to school soon" indicated a decrease. Furthermore, especially in Group P students, SS scores decreased transiently during school closure and increased after school reopening. CONCLUSIONS: The impact of school closure on mental health differed depending on students' diagnoses and feelings about school during school closures.
Assuntos
COVID-19/epidemiologia , Saúde Mental/estatística & dados numéricos , Instituições Acadêmicas , COVID-19/prevenção & controle , Criança , Doença Crônica , Deficiências do Desenvolvimento/epidemiologia , Humanos , Japão/epidemiologia , Pandemias , Transtornos Psicofisiológicos/epidemiologia , Fatores de Risco , Inquéritos e QuestionáriosRESUMO
BACKGROUND: We aimed to compare the profile of the Wechsler Intelligence Scale for Children Fourth Edition (WISC-IV) between Japanese schoolchildren born very preterm (VP) and with very low birthweight (VLBW) and those with attention deficit hyperactivity disorder (ADHD), and to identify the specific neurocognitive characteristics of VLBW/VP children. METHODS: The VLBW/VP group in the present study included 50 (19 male, 31 female) first- to third-grade elementary school children born between January 2008 and February 2013 at Osaka Medical and Pharmaceutical University Hospital and Saiseikai Suita Hospital with birthweights <1,500 g and <32 gestational weeks. The ADHD group included 18 (13 male, 5 female) first- to third-grade elementary school children who visited Osaka Medical and Pharmaceutical University Hospital between January 2019 and October 2021. Full-scale intelligence quotient scores, four indices, and 12 subtests of the WISC-IV were calculated for all participants and compared between the VLBW/VP and ADHD groups. We assessed whether the patients' clinical history was associated with a low score on the cancellation task in the VLBW/VP group. RESULTS: The WISC-IV profiles showed similar between-group patterns, and the VLBW/VP group had lower cancellation task scores than the ADHD group. CONCLUSIONS: This is the first study to compare WISC-IV profiles between VLBW/VP children and those with ADHD. Further investigation is needed on the association between academic performance and the score of the cancellation task, and the neural mechanism of low performance for cancellation tasks in VLBW/VP children.
Assuntos
Transtorno do Deficit de Atenção com Hiperatividade , Transtorno do Deficit de Atenção com Hiperatividade/diagnóstico , Transtorno do Deficit de Atenção com Hiperatividade/psicologia , Peso ao Nascer , Criança , Feminino , Humanos , Recém-Nascido , Recém-Nascido de muito Baixo Peso , Masculino , Preparações Farmacêuticas , Escalas de WechslerRESUMO
BACKGROUND: Several studies have demonstrated that non-osmotic antidiuretic hormone activity contributes to the development of hyponatremia in children with common febrile diseases. However, the relationship between hyponatremia and body temperature has remained unclear. We therefore examined this relationship in children with common diseases. METHODS: In this retrospective case study based on a chart review, 1,973 children presenting with acute illnesses at Hirakata City Hospital between November 2008 and October 2009, and for whom blood test data were available, were enrolled. The median age of this cohort was 2.7 years and the mean serum sodium concentration was 136.4 mEq/L; 454 patients showed hyponatremia (<135 mEq/L). The patients were classified into four groups on the basis of body temperature, <37 °C, 37 °C (37.0-37.9 °C), 38 °C (38.0-38.9 °C) and ≥39 °C, and their serum sodium concentration was compared. RESULTS: The mean sodium level was significantly lower in febrile (135.9 mEq/L) than in non-febrile (138.5 mEq/L) patients. The mean serum sodium levels in the four temperature groups were, in ascending order, 138.5 mEq/L (95% CI, 138.3-138.8 mEq/L), 137.3 mEq/L (137.1-137.5 mEq/L), 136.1 mEq/L (135.8-136.3 mEq/L) and 134.6 mEq/L (134.4-134.9 mEq/L), respectively. The serum sodium level in each individual temperature range became significantly lower as body temperature increased (P < 0.001). CONCLUSIONS: There is a clear inverse correlation between serum sodium level and body temperature in children with common febrile diseases, and fever may play an important role in this relationship.
Assuntos
Temperatura Corporal , Hiponatremia , Criança , Pré-Escolar , Estudos de Coortes , Humanos , Hiponatremia/epidemiologia , Hiponatremia/etiologia , Estudos Retrospectivos , SódioRESUMO
BACKGROUND: The prevalence of overweight and obesity has increased, not just in adults but also in children. In Japan, the percentage of overweight (POW) is widely used for the estimation and assessment of percentage body fat (PBF) for children. We examine whether there is a difference between normal height and short stature children in terms of the relationship between POW and PBF. METHODS: A total of 321 children of normal height (163 boys, 158 girls) and 106 children with short stature (78 boys, 28 girls) were assessed according to sex. Percentage of overweight was used to represent the degree of obesity. The PBF of the total body was examined by dual energy X-ray absorptiometry. RESULTS: According to their PBF, normal height subjects of both sexes were classified as obese if they had a POW of more than 20%. However, short-stature subjects of both sexes were not classified as obese until the boys had a POW of 40%, and girls had a POW of 35%, respectively. Thus, overweight or obesity is overestimated in short stature children in both males and females with the rule "POW above 20% is recognized as obesity." CONCLUSIONS: Percentage of overweight is commonly considered as a good surrogate measure for determining overweight and obesity in children of both sexes. However, this study has clarified that body fat accumulation is overestimated in children with short stature when using the POW measurement.
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Sobrepeso , Obesidade Infantil , Masculino , Adulto , Feminino , Humanos , Criança , Sobrepeso/epidemiologia , Obesidade Infantil/epidemiologia , Índice de Massa Corporal , Tecido Adiposo , Japão/epidemiologiaRESUMO
BACKGROUND: Small bowel capsule endoscopy (SBCE) for Crohn's disease is useful; however, its use has some limitations, such as invasiveness when endoscopic assistance is required in patients who cannot swallow the capsule, and the burden of interpretation on a physician. In contrast, intestinal ultrasonography (IUS) is a non-invasive modality for children. The purpose of this study is to evaluate the accuracy of IUS for pediatric patients with established Crohn's disease. METHODS: Small bowel capsule endoscopy and IUS findings from the same period in pediatric patients with established Crohn's disease were analyzed retrospectively. First, we compared the Lewis score (LS), small bowel endoscopic activity, and IUS findings by small bowel wall thickness (SBWT) and mesenteric lymph node size (MLNS). Second, we compared the performance of IUS findings with those of some biomarkers. RESULTS: In 22 procedures, SBWT and MLNS were correlated with LS (r = 0.52, P < 0.05, and r = 0.45, P < 0.05, respectively). Small bowel wall thickness, erythrocyte sedimentation rate, and fecal calprotectin levels had the highest accuracy (81.8%, 81.8%, and 81.8%, respectively). The combination of SBWT and MLNS had the highest positive predictive value and negative predictive value (100% and 83.3%, respectively). CONCLUSIONS: Intestinal ultrasonography findings, including SBWT and MLNS, are useful for monitoring small bowel lesions in pediatric patients with established Crohn's disease. We suggest first evaluating small bowel inflammation by IUS in pediatric patients with Crohn's disease before SBCE because IUS is less invasive than SBCE.
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Endoscopia por Cápsula , Doença de Crohn , Endoscopia por Cápsula/métodos , Criança , Doença de Crohn/diagnóstico por imagem , Humanos , Estudos Retrospectivos , UltrassonografiaRESUMO
BACKGROUND: The aim of this study was to assess the treatment response to conventional antiepileptic drugs and low-dose adrenocorticotropic hormone therapy for infantile spasms in children with Down syndrome. METHODS: We retrospectively investigated the response and relapse rates, electroencephalography findings, patient characteristics during drug withdrawal, and developmental outcome in 10 children with Down syndrome treated for infantile spasms in our hospital. RESULTS: All patients showed cessation of infantile spasms and achieved electroencephalographic normalization. Spasm relapse occurred in one of 10 patients (10%). Antiepileptic drugs have been withdrawn for seven of 10 patients (70%), none of whom have experienced seizure relapse since drug withdrawal. The median developmental quotient (n = 8) was 20.5, which shows that the developmental outcome was unfavorable. Low-dose adrenocorticotropic hormone therapy achieved a low seizure remission rate of 28.6%. CONCLUSIONS: Elucidation of the optimal treatment for infantile spasms in children with Down syndrome is needed to reduce the duration of infantile spasms and improve the developmental outcome.
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Síndrome de Down , Espasmos Infantis , Anticonvulsivantes/uso terapêutico , Criança , Síndrome de Down/complicações , Síndrome de Down/tratamento farmacológico , Eletroencefalografia , Humanos , Lactente , Japão/epidemiologia , Estudos Retrospectivos , Espasmo/tratamento farmacológico , Espasmos Infantis/diagnóstico , Espasmos Infantis/tratamento farmacológico , Espasmos Infantis/etiologia , Resultado do TratamentoRESUMO
The safety and efficacy of landiolol have not been fully elucidated in pediatric patients. This study aimed to clarify the safety and efficacy of landiolol in a pediatric cohort. We retrospectively assessed the clinical features of 21 pediatric patients who were administered landiolol at our hospital. We also investigated the rates of sinus rhythm conversion and heart rate response. The median patient age was 7 months (interquartile range 1-13 months). The etiology of tachyarrhythmia was junctional ectopic tachycardia in 10 patients (47.6%), atrial tachycardia in 10 patients (47.6%), and ventricular tachycardia in 1 patient (4.8%). Of the 21 children, 18 (85.7%) had congenital heart defects, including 14 (77.8%) in whom a landiolol infusion was performed perioperatively. The landiolol infusion was effective in 18 pediatric patients (85.7%), as measured by the conversion to sinus rhythm or a reduced heart rate. Atrial tachycardia in the perioperative period was terminated in all patients. Of 7 patients with tachyarrhythmias unrelated to the perioperative period, landiolol was effective in 5. No adverse effects were reported in any patient. Landiolol infusion is effective and safe in pediatric patients with tachyarrhythmia of various etiologies, especially those with atrial tachyarrhythmia during the perioperative period.
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Fibrilação Atrial , Morfolinas , Criança , Humanos , Lactente , Morfolinas/efeitos adversos , Estudos Retrospectivos , Taquicardia/tratamento farmacológico , Taquicardia/etiologia , Ureia/análogos & derivadosRESUMO
BACKGROUND: Cardiac dysfunction, arrhythmia, and hepatic fibrosis are well-known complications after right heart bypass surgery in patients with single-ventricle physiology. However, little is known about coronary arterial fistulae, and only a few reports have been published. This study aimed to elucidate the clinical characteristics of these rare coronary arterial fistulae that developed as complications in cases of single-ventricle physiology after right heart bypass surgery. METHODS: We retrospectively investigated the clinical features and courses of patients who developed acquired and progressive coronary arterial fistulae after right heart bypass surgery in our hospital. RESULTS: We identified three cases of coronary arterial fistulae out of 21 patients who underwent right heart bypass surgery. All three cases underwent cardiac catheterisation for post-operative evaluation and were administered pulmonary vasodilators of phosphodiesterase type V inhibitors, antiplatelet, anticoagulation, and diuretics. Moreover, they had common clinical features such as right-dominant single ventricle and long-term exposure to chronic hypoxia. Serial angiograms revealed acquired and progressive coronary arterial fistulae. In addition, coronary arterial fistulae contributed to their symptoms of heart failure. CONCLUSION: Patients with chronic hypoxia and dominant right ventricle, who are treated with phosphodiesterase type V inhibitors, should be followed up after right heart bypass surgery to monitor the possible development of coronary arterial fistulae. Moreover, the indication for pulmonary vasodilators in single-ventricle physiology after right heart bypass surgery should be optimised to avoid adverse effects.
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Cardiopatias Congênitas , Atresia Pulmonar , Ventrículos do Coração/diagnóstico por imagem , Humanos , Estudos Retrospectivos , Resultado do Tratamento , Vasodilatadores/uso terapêuticoRESUMO
BACKGROUND: More than 90% of gastric cancer cases are caused by Helicobacter pylori infections. To prevent gastric cancer, an H pylori test-and-treat strategy targeting young people has been implemented in various places in Japan. In this study, we evaluated the effectiveness of an H pylori test-and-treat strategy for second-year junior high school students in Takatsuki City. MATERIALS AND METHODS: In 2014-2017, a urine-based H pylori test was used for initial screening. The final infection status was determined by a 13 C-urea breath test (13 C-UBT). Successful H pylori eradication was confirmed by 13 C-UBT 3 months after treatment. First-line eradication therapy was changed from 10 mg of rabeprazole, 750 mg of amoxicillin, and 200 mg of clarithromycin twice daily for 7 days in 2014 to 20 mg of vonoprazan, 750 mg of amoxicillin, and 200 mg of clarithromycin twice daily for 7 days in 2015-2017. Second-line eradication therapy included 10 mg of rabeprazole, 750 mg of amoxicillin, and 250 mg of metronidazole twice daily for 7 days. RESULTS: In total, 8067 of 13 055 students participated this project and 206 students were diagnosed with H pylori infection. The success rate of first-line therapy was 45.9% in 2014 and 83.8% after the revised first-line therapy was administered. The final eradication rate was 98.5%. There were no severe side effects. CONCLUSION: Our results support the use of the H pylori test-and-treat strategy for junior high school students as a safe approach for the prevention of gastric cancer. H pylori eradication therapy with vonoprazan could be a standard therapy in children.
Assuntos
Infecções por Helicobacter/diagnóstico , Infecções por Helicobacter/tratamento farmacológico , Helicobacter pylori/efeitos dos fármacos , Serviços de Saúde Escolar/estatística & dados numéricos , Neoplasias Gástricas/prevenção & controle , Adolescente , Antibacterianos/administração & dosagem , Testes Respiratórios , Participação da Comunidade/estatística & dados numéricos , Feminino , Helicobacter pylori/isolamento & purificação , Humanos , Japão/epidemiologia , Masculino , Neoplasias Gástricas/microbiologia , Resultado do Tratamento , Ureia/análiseRESUMO
BACKGROUND: Although there is debate regarding the timing of initiation of renal replacement therapy (RRT) in adults with end-stage renal disease, there is a paucity of reliable epidemiological data on pediatric patients. The present study was performed to investigate current practice in Japan with regard to the timing of initiation of RRT in children based on estimated glomerular filtration rate (eGFR). METHODS: A total of 649 pediatric patients < 20 years old with eGFR at the initiation of RRT between 1 January 2006 and 31 December 2013 were included in the study. Baseline eGFR was calculated for each patient using the Schwartz formula. RESULTS: eGFR at the start of RRT was 12.1 mL/min/1.73 m2 [interquartile range (IQR) 8.4-16.3]. A total of 209 children (32.2%) had high eGFR (eGFR > 15 mL/min/1.73 m2) at the initiation of RRT. Initiation of RRT was more likely in those undergoing preemptive transplantation (PEKT) with high eGFR [odds ratio (OR) 4.16; 95% confidence interval (CI) 1.95-8.90, P < 0.001]. There were 31 deaths of various causes during follow-up, with infections representing the leading causes of death. CONCLUSIONS: The median eGFR at the initiation of RRT in children showed a wide range of variation. Further studies are needed to investigate the impact of the decision regarding when to initiate RRT in individual pediatric patients.
Assuntos
Taxa de Filtração Glomerular , Disparidades em Assistência à Saúde/tendências , Falência Renal Crônica/terapia , Rim/fisiopatologia , Nefrologistas/tendências , Padrões de Prática Médica/tendências , Terapia de Substituição Renal/tendências , Adolescente , Fatores Etários , Criança , Pré-Escolar , Tomada de Decisão Clínica , Estudos Transversais , Feminino , Pesquisas sobre Atenção à Saúde , Humanos , Lactente , Recém-Nascido , Japão/epidemiologia , Falência Renal Crônica/diagnóstico , Falência Renal Crônica/mortalidade , Falência Renal Crônica/fisiopatologia , Masculino , Terapia de Substituição Renal/efeitos adversos , Terapia de Substituição Renal/mortalidade , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores de Tempo , Resultado do TratamentoRESUMO
SIGNIFICANCE: Our study revealed that the validity of a new Japanese visual-perceptual test was acceptable. Visual-perceptual abilities are important to activities of daily living; thus, accurate assessment of visual perception is especially important for children with neurodevelopmental disorders and particularly so for those with learning problems. PURPOSE: Visual perception refers to the process by which one receives visual information through sensory impulses and then translates those impulses into meaning based on a previously developed view of the environment. A problem in Japan is the paucity of visual perception tests that use normative data from children who are native Japanese. The Wide-range Assessment of Vision-related Essential Skills (WAVES), which measures visual perception and eye-hand coordination skills and is based on Japanese normative data, was recently published in Japan. The validity of this test has not been comprehensively established. METHODS: To investigate the validity of the WAVES, we used the Pearson correlation coefficient to calculate the degrees of association among WAVES scaled and index scores compared with Developmental Test of Visual Perception, Third Edition, scores from 108 elementary school children with symptoms of learning problems. Participants were recruited at Osaka Medical College Learning Disability Center and Sakai Seikeikai Learning Disability Center. RESULTS: The concurrent validity of the WAVES was supported by moderate correlation (r = 0.67, P < .01) between the total scores for visual perception and eye-hand coordination index from the WAVES and general visual perception index from the Developmental Test of Visual Perception, Third Edition, even though a correlation analysis of subtests found differences between the two tests. CONCLUSIONS: Our results showed that the indices from the two tests measured nearly the same underlying visual-perceptual constructs and indicated that the WAVES had acceptable levels of concurrent validity.