RESUMO
BACKGROUND: Children awaiting heart transplant (Tx) have a high risk of death due to donor organ scarcity. Historically, ventricular assist devices (VADs) reduced waitlist mortality, prompting increased VAD use. We sought to determine whether the VAD survival benefit persists in the current era. METHODS: Using the Scientific Registry of Transplant Recipients, we identified patients listed for Tx between 3/22/2016 and 9/1/2020. We compared characteristics of VAD and non-VAD groups at Tx listing. Cox proportional hazards models were used to identify risk factors for 1-year waitlist mortality. RESULTS: Among 5054 patients, 764 (15%) had a VAD at Tx listing. The VAD group was older with more mechanical ventilation and renal impairment. Unadjusted waitlist mortality was similar between groups; the curves crossed ~90 days after listing (p = .55). In multivariable analysis, infant age (HR 2.77, 95%CI 2.13-3.60), Black race (HR 1.57, 95%CI 1.31-1.88), congenital heart disease (HR 1.23, 95%CI 1.04-1.46), renal impairment (HR 2.67, 95%CI 2.19-3.26), inotropes (HR 1.28, 95%CI 1.09-1.52), and mechanical ventilation (HR 2.23, 95%CI 1.84-2.70) were associated with 1-year waitlist mortality. VADs were not associated with mortality in the first 90 waitlist days but were protective for those waiting ≥90 days (HR 0.43, 95%CI 0.26-0.71). CONCLUSIONS: In the current era, VADs reduce waitlist mortality, but only for those waitlisted ≥90 days. The differential effect by race, size, and VAD type is less clear. These findings suggest that Tx listing without VAD may be reasonable if a short waitlist time is anticipated, but VADs may benefit those expected to wait >90 days.
Assuntos
Transplante de Coração , Coração Auxiliar , Sistema de Registros , Listas de Espera , Humanos , Listas de Espera/mortalidade , Masculino , Feminino , Lactente , Criança , Pré-Escolar , Adolescente , Fatores de Risco , Bases de Dados Factuais , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Insuficiência Cardíaca/mortalidade , Insuficiência Cardíaca/cirurgia , Insuficiência Cardíaca/terapia , Estados Unidos/epidemiologiaRESUMO
Obstructive sleep apnea (OSA) has been associated with negative cardiovascular effects and negative outcomes following cardiovascular surgery in the adult population. Our objective was to evaluate if there is a similar association in children. Single center retrospective matched cohort study, we collected data on patients aged 1-18 who were admitted to the cardiac intensive care unit (CICU) between Jan 2012 and Dec 2021. We used a 3:1 propensity score matching for cases not diagnosed with OSA. Primary outcome was a composite variable: "adverse outcome," consisting of prolonged hospital and CICU stay, prolonged duration of mechanical ventilation, need for extracorporeal membrane oxygenation, and death. The study comprised 80 patients diagnosed with OSA and 240 patients without a diagnosis of OSA before cardiac surgery. The median age was 5.3 years (IQR 2.7-11.2). There were 184 (57.5%) males, and 102 (31.9%) had chromosomal and genetic abnormalities. There was a difference in "adverse outcome" between the OSA and non-OSA groups [34 (42.50%) vs 68 (28.33%), p = 0.027]. Moreover, bivariate analysis revealed that CICU length of stay, and chromosomal anomalies were statistically different between the groups. By logistic regression the composite variable "adverse outcome" remained associated to the OSA group (p = 0.009) with an adjusted odds ratio (OR) of 4.09 (1.83-9.18), p < 0.001. Children diagnosed with OSA had a higher risk of "adverse outcome" following cardiac surgery. The risk disappeared if the patient had Tonsillectomy and Adenoidectomy before cardiac surgery. Further studies should explore a proactive treatment for OSA in pediatric patients who need cardiac surgery.
RESUMO
Wheat yellow (stripe) rust, caused by Puccinia striiformis f. sp. tritici (Pst), is one of the most devastating diseases of wheat worldwide. Pst populations are composed of multiple genetic groups, each carrying one or more races characterized by different avirulence/virulence combinations. Since the severe epidemics in 2017, yellow rust has become the most economically important wheat foliar disease in Uruguay. A set of 124 Pst isolates collected from wheat fields in Uruguay between 2017 and 2021 were characterized phenotypically, and 27 of those isolates were subsequently investigated in-depth by additional molecular genotyping and race phenotyping analyses. Three genetic groups were identified, PstS7, PstS10, and PstS13, with the latter being the most prevalent. Two races previously reported in Europe, Warrior (PstS7) and Benchmark (PstS10), were detected in four and two isolates, respectively. A third race, known as Triticale2015 (PstS13), that was first detected in Europe in 2015 and in Argentina in 2017 was detected at several locations. Additional virulence to Yr3, Yr17, Yr25, Yr27, or Yr32 was detected in three new race variants within PstS13. The identification of these new races, which have not been reported outside South America, provides strong evidence of the local evolution of virulence in Pst during the recent epidemic years.
Assuntos
Doenças das Plantas , Puccinia , Triticum , Virulência/genética , Doenças das Plantas/microbiologia , Puccinia/patogenicidade , Puccinia/genética , Triticum/microbiologia , Uruguai , Genótipo , Evolução Biológica , Fenótipo , Basidiomycota/genética , Basidiomycota/patogenicidade , Basidiomycota/classificação , Basidiomycota/fisiologiaRESUMO
OBJECTIVE: Limited data exists regarding prostanoid (PGI2) use in critically ill patients with pulmonary hypertension. (PH) in the pediatric cardiac intensive care unit (CICU) setting. MATERIALS AND METHODS: Single center, retrospective study of patients with diagnosis of PH who received continuous PGI2 and were admitted to CICU from January/2015 to April/2022. Data collected included patient demographics and clinical characteristics including diagnosis, etiology of PH, vasoactive and ventilatory support, length of stay, and survival. Type, initial, maximum, and final dose of PGI2 as well as hemodynamic data was obtained. Data reported as mean ± standard deviation. Significance taken p value < 0.05. RESULTS: 24 patients received PGI2 therapy at a mean age of 3.1 years, range (0-16.6 years). PGI2 was in the form of IV epoprostenol in 12 patients, IV treprostinil in 6, and SQ treprostinil in 6 patients. Mean initial dose was 2.79 ng/kg/min, max dose 18.75 ng/kg/min, and mean duration of therapy was 38.5 days. At PGI2 initiation, 21 (87.5%) were on vasoactive infusions, 19 (79.2%) mechanically ventilated (MV), and 6 (25%) were on extracorporeal membrane oxygenation (ECMO). The in-hospital mortality rate was 37.5% (n = 9). Patients MV and on ECMO support had higher risk of death (p = 0.04, and < 0.01, respectively). CONCLUSION: PGI2 therapy was tolerated in approximately 50% of patients with the most common side effect being hypotension leading to discontinuation in 1/3rd of patients. Ongoing evaluation of the benefits of PGI2 for patients in the CICU setting will help better identify patient selection, type, and dosing of PGI2.
RESUMO
Dilated cardiomyopathy is the most common presentation of cardiomyopathy in children with 20-35% of patients having an identified genetic component. There are more than 30 genes implicated in the pathogenesis of dilated cardiomyopathy. We present the first report of a female infant with dilated cardiomyopathy with a genetic variant in the dispatched RND transporter family member 1 gene.
Assuntos
Cardiomiopatia Dilatada , Cardiomiopatia Dilatada/genética , Família , Feminino , Humanos , Lactente , Mutação , LinhagemRESUMO
Background: Multisystem inflammatory syndrome (MIS-C) is a clinical presentation reported in children related to Coronavirus-19 infection who present with a toxic shock like syndrome. Vitamin D deficiency has been postulated to play a role with severity of coronavirus infection in adult patients and other viral respiratory infections. Objective: This study aims to investigate if severe vitamin D deficiency was associated with increased disease severity and cardiac involvement in MIS-C. Methods: This is a retrospective and single center study. We included hospitalized patients less than 18 years of age with diagnosis of MIS-C between March and July 2020. Severe vitamin D deficiency was defined as 25-OH vitamin D level < 10 ng/ml within 48 h of admission. The composite outcome severe disease included patients requiring inotropes, mechanical ventilation, and extracorporeal membrane oxygenation. Results: Of the 31 patients with MIS-C, 45% were male and 58% were African American. The median age was 8 (1-13) years. Ten patients had severe vitamin D deficiency with a mean level of 7.2 ng/ml. Ninety percent of patients with severe vitamin D deficiency had severe disease (P < 0.001). Patients with severe vitamin D deficiency had an increased risk of cardiac involvement (P < 0.001). Conclusions: We describe a potential association between severe vitamin D deficiency and severe disease in children presenting with MIS-C. Severe vitamin D deficiency predisposes patients for cardiovascular involvement and may play a critical role in the host immune response to COVID-19 infection. Future prospective studies at the basic science and clinical level should be pursued to better delineate this association.
RESUMO
Flowering is a key agronomic trait that influences adaptation and productivity. Previous studies have indicated the genetic complexity associated with the flowering response in a photoinsensitive weedy rice accession PSRR-1 despite the presence of a photosensitive allele of a key flowering gene Hd1. In this study, we used whole-genome and RNA sequencing data from both cultivated and weedy rice to add further insights. The de novo assembly of unaligned sequences predicted 225 genes, in which 45 were specific to PSRR-1, including two genes associated with flowering. Comparison of the variants in PSRR-1 with the 3K rice genome (RG) dataset identified unique variants within the heading date QTLs. Analyses of the RNA-Seq result under both short-day (SD) and long-day (LD) conditions revealed that many differentially expressed genes (DEGs) colocalized with the flowering QTLs, and some DEGs such as Hd1, OsMADS56, Hd3a, and RFT1 had unique variants in PSRR-1. Ehd1, Hd1, OsMADS15, and OsMADS56 showed different alternate splicing (AS) events between genotypes and day length conditions. OsMADS56 was expressed in PSRR-1 but not in Cypress under both LD and SD conditions. Based on variations in both sequence and expression, the unique flowering response in PSRR-1 may be due to the high-impact variants of flowering genes, and OsMADS56 is proposed as a key regulator for its day-neutral flowering response.
Assuntos
Perfilação da Expressão Gênica/métodos , Oryza/crescimento & desenvolvimento , Locos de Características Quantitativas , Sequenciamento Completo do Genoma/métodos , Mapeamento Cromossômico , Produtos Agrícolas/classificação , Produtos Agrícolas/genética , Produtos Agrícolas/crescimento & desenvolvimento , Flores/genética , Flores/crescimento & desenvolvimento , Regulação da Expressão Gênica de Plantas , Sequenciamento de Nucleotídeos em Larga Escala , Oryza/classificação , Oryza/genética , Fotoperíodo , Proteínas de Plantas/genética , Polimorfismo de Nucleotídeo Único , RNA-SeqRESUMO
The inferior vena cava collapsibility index (IVCCI) is an ultrasound method used to assess central venous pressure (CVP). Our objective was to evaluate the correlation between IVCCI and CVP in children during the early period following surgery for congenital heart disease (CHD). Prospective study performed in a single tertiary care center. Patients ≤ 18 years old, who underwent cardiopulmonary bypass surgery for CHD, were enrolled. Ultrasound images of the inferior vena cava (IVC) were obtained at two time points; the first was within 2 h of arrival to the CICU and the second was 12-18 h from the first measurement. CVP measured by catheter placed during surgery was recorded within minutes of performing ultrasound. Maximum and minimum IVC diameters were measured by 2D images and M mode method. Seventy patients (47.1% males), with median age 7 months (IQR 4-47 months) and weight 6.9 kg (IQR 4.8-13.5 kg), were evaluated. The 2D IVCCI had inverse correlation with CVP in patients breathing spontaneously; r = - 0.76 (p < 0.01) and r = - 0.73 (p < 0.01), during the first and second measurements, respectively. The 2D IVCCI ≤ 0.24 had sensitivity, specificity, and negative predictive value of 94%, 79%, and 88.9% , respectively, to detect CVP ≥ 10 mmHg. No correlation was found between IVCCI and CVP during positive pressure ventilation. There is a significant inverse correlation between 2D IVCCI and CVP in spontaneously breathing children after surgery for CHD. Use of 2D IVCCI for monitoring CVP could reduce the frequency and duration of CVP catheters and their inherent complications.
Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Pressão Venosa Central , Cardiopatias Congênitas/cirurgia , Veia Cava Inferior/diagnóstico por imagem , Ponte Cardiopulmonar/métodos , Pré-Escolar , Feminino , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/fisiopatologia , Humanos , Lactente , Masculino , Período Perioperatório , Valor Preditivo dos Testes , Estudos Prospectivos , Respiração , Ultrassonografia/métodos , Veia Cava Inferior/fisiopatologiaRESUMO
Although rare, children with active coronavirus disease 2019 are at risk of developing malignant arrhythmia. Herein, we present the first paediatric case of refractory ventricular tachycardia from acute fulminant myocarditis secondary to acute COVID-19 infection. This 5-year-old boy required venoarterial extracorporeal membrane oxygenation support, but made a complete recovery without significant morbidity.
Assuntos
COVID-19 , Oxigenação por Membrana Extracorpórea , Miocardite , Taquicardia Ventricular , Criança , Pré-Escolar , Humanos , Masculino , Miocardite/complicações , Miocardite/diagnóstico , SARS-CoV-2 , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/etiologiaRESUMO
INTRODUCTION: The number of adults requiring surgeries for CHD is increasing. We sought to evaluate the utility of the vasoactive-ventilation-renal (VVR) score as a predictor of prolonged length of stay in adults following CHD surgery. METHODS: This is a retrospective review of 158 adult patients who underwent CHD surgery involving cardiopulmonary bypass. VVR score was calculated upon arrival to ICU and every 6 hours for the first 48 hours post-operatively. Our primary outcome was prolonged length of stay defined as hospital length of stay greater than 75th percentile for the cohort (≥8 days). RESULTS: The study cohort had a median age of 25.6 years (18-60 years), and 83 (52.5%) were male. The groups with and without prolonged length of stay were comparable in age, gender, race, and surgical severity score. VVR score was significantly higher at all time points in the group with prolonged length of stay. The first post-operative day peak VVR score ≥13 had a sensitivity of 81% and specificity of 75% for predicting prolonged length of stay (p = 0.0001). On regression analysis, peak VVR score during the first day was independently associated with prolonged length of stay. CONCLUSIONS: Peak VVR score during the first post-operative day was a strong predictor of prolonged length of stay in adults following CHD surgery.
Assuntos
Procedimentos Cirúrgicos Cardíacos , Cardiopatias Congênitas , Adulto , Ponte Cardiopulmonar , Feminino , Cardiopatias Congênitas/cirurgia , Humanos , Tempo de Internação , Masculino , Prognóstico , Estudos RetrospectivosRESUMO
BACKGROUND: Obesity is a modifiable, independent risk factor for mortality and morbidity after cardiovascular surgery in adults. Our objective was to evaluate the impact of obesity on short-term outcomes in adolescents undergoing surgery for congenital heart disease (CHD). METHODS: This retrospective chart review included patients 10-18 years of age who underwent CHD surgery. Our exclusion criteria were patients with a known genetic syndrome, heart transplantation, and patients with incomplete medical records. The clinical data collected included baseline demographics and multiple perioperative variables. Charting the body mass index in the Centers for Disease Control and Prevention growth curves, the entire cohort was divided into three categories: obese (>95th percentile), overweight (85th-95th percentile), and normal weight (<85th percentile). The composite outcome included survival, arrhythmias, surgical wound infection, acute neurologic injury, and acute kidney injury. RESULTS: The study cohort (n = 149) had a mean standard deviation (SD), body mass index (BMI) of 22.6 ± 6.5 g/m2, and 65% were male. There were 27 obese (18.1%), 24 overweight (16.1%), and 98 normal weight (65.8%) patients. Twenty-seven (18%) patients had composite adverse outcomes. Overweight and obese patients had significantly higher adverse outcomes compared with normal weight patients (odds ratio (OR): 2.9; confidence interval (CI): 1-8.5, p = 0.04 and OR: 3; CI: 1-8.5, p = 0.03, respectively). In multivariate analysis, obesity was an independent predictor of adverse outcome in our cohort (p = 0.04). CONCLUSIONS: Obesity is associated with short-term adverse outcome and increased health resource utilisation in adolescents following surgery for CHD. Further studies should evaluate if intervention in the preoperative period can improve outcomes in this population.
Assuntos
Índice de Massa Corporal , Procedimentos Cirúrgicos Cardíacos/mortalidade , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/cirurgia , Obesidade Infantil/complicações , Adolescente , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Criança , Feminino , Humanos , Modelos Logísticos , Masculino , Michigan/epidemiologia , Análise Multivariada , Sobrepeso/complicações , Aceitação pelo Paciente de Cuidados de Saúde , Estudos Retrospectivos , Fatores de Risco , Resultado do TratamentoRESUMO
Plant architecture is critical for enhancing the adaptability and productivity of crop plants. Mutants with an altered plant architecture allow researchers to elucidate the genetic network and the underlying mechanisms. In this study, we characterized a novel nal1 rice mutant with short height, small panicle, and narrow and thick deep green leaves that was identified from a cross between a rice cultivar and a weedy rice accession. Bulked segregant analysis coupled with genome re-sequencing and cosegregation analysis revealed that the overall mutant phenotype was caused by a 1395-bp deletion spanning over the last two exons including the transcriptional end site of the nal1 gene. This deletion resulted in chimeric transcripts involving nal1 and the adjacent gene, which were validated by a reference-guided assembly of transcripts followed by PCR amplification. A comparative transcriptome analysis of the mutant and the wild-type rice revealed 263 differentially expressed genes involved in cell division, cell expansion, photosynthesis, reproduction, and gibberellin (GA) and brassinosteroids (BR) signaling pathways, suggesting the important regulatory role of nal1. Our study indicated that nal1 controls plant architecture through the regulation of genes involved in the photosynthetic apparatus, cell cycle, and GA and BR signaling pathways.
Assuntos
Regulação da Expressão Gênica de Plantas , Mutação , Oryza/anatomia & histologia , Fotossíntese , Folhas de Planta/anatomia & histologia , Proteínas de Plantas/genética , Mapeamento Cromossômico , Redes Reguladoras de Genes , Oryza/genética , Oryza/metabolismo , Folhas de Planta/genética , Folhas de Planta/metabolismo , TranscriptomaRESUMO
The indiscriminate use of nitrogenous fertilizers continues unabated for commercial crop production, resulting in air and water pollution. The development of rice varieties with enhanced nitrogen use efficiency (NUE) will require a thorough understanding of the molecular basis of a plant's response to low nitrogen (N) availability. The global expression profiles of root tissues collected from low and high N treatments at different time points in two rice genotypes, Pokkali and Bengal, with contrasting responses to N stress and contrasting root architectures were examined. Overall, the number of differentially expressed genes (DEGs) in Pokkali (indica) was higher than in Bengal (japonica) during low N and early N recovery treatments. Most low N DEGs in both genotypes were downregulated whereas early N recovery DEGs were upregulated. Of these, 148 Pokkali-specific DEGs might contribute to Pokkali's advantage under N stress. These DEGs included transcription factors and transporters and were involved in stress responses, growth and development, regulation, and metabolism. Many DEGs are co-localized with quantitative trait loci (QTL) related to root growth and development, chlorate-resistance, and NUE. Our findings suggest that the superior growth performance of Pokkali under low N conditions could be due to the genetic differences in a diverse set of genes influencing N uptake through the regulation of root architecture.
Assuntos
Nitrogênio/metabolismo , Oryza/genética , Oryza/fisiologia , Raízes de Plantas/fisiologia , Estresse Fisiológico/genética , Transcriptoma/genética , Processamento Alternativo/genética , Biomassa , Cloratos/metabolismo , Clorofila/metabolismo , Regulação da Expressão Gênica de Plantas/efeitos dos fármacos , Ontologia Genética , Genes Controladores do Desenvolvimento , Genótipo , Anotação de Sequência Molecular , Oryza/efeitos dos fármacos , Reguladores de Crescimento de Plantas/farmacologia , Raízes de Plantas/efeitos dos fármacos , Raízes de Plantas/genética , Raízes de Plantas/crescimento & desenvolvimento , Brotos de Planta/efeitos dos fármacos , Brotos de Planta/metabolismo , Locos de Características Quantitativas/genética , Transdução de Sinais/genética , Estresse Fisiológico/efeitos dos fármacos , Fatores de Transcrição/metabolismoRESUMO
Background: Intravascular papillary endothelial hyperplasia (IPEH) is a benign vascular lesion that usually involves the head and neck or extremities. Involvement of the coronary arterial system is unreported. Case: A 1-month-old patient born with hypoplastic left heart syndrome died from a massive myocardial infarction shortly after first stage palliation with Norwood/Sano. Autopsy demonstrated a massive univentricular hemorrhagic infarction with complete occlusion of the left main coronary artery and its intramural branches by intraluminal papillary endothelial hyperplasia (IPEH). Immunostaining with CD34 and CD31 confirmed the diagnosis. The inferior and superior mesenteric artery branches also had IPEH. Conclusion: IPEH can involve the coronary vasculature, can be multifocal and can occur in the newborn.
Assuntos
Vasos Coronários/patologia , Hiperplasia/patologia , Síndrome do Coração Esquerdo Hipoplásico/patologia , Infarto do Miocárdio/patologia , Diagnóstico Diferencial , Endotélio Vascular/patologia , Feminino , Cabeça/patologia , Humanos , Hiperplasia/diagnóstico , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico , Lactente , Recém-Nascido , Infarto do Miocárdio/diagnóstico , Pescoço/patologiaRESUMO
OBJECTIVE: The aim of this study was to evaluate the prevalence of acute kidney injury after first-stage surgical palliation in patients with a single ventricle and to explore associated risk factors and outcomes. Design and patients This single-centre retrospective study included neonates who underwent either Norwood or Hybrid procedure from 2008 to 2015 for a single ventricle. Postoperative acute kidney injury was defined using the paediatric risk, injury, failure, loss, end-stage renal disease (pRIFLE), criteria within 72 hours of the procedure. Main results Our cohort (n=48) underwent surgical palliation at a mean (SD) age of 12 (11) days. Postoperative acute kidney injury was diagnosed in 14 (29%) patients. The prevalence of acute kidney injury in the Hybrid group was 16% and 53% in the Norwood group. Infants who developed acute kidney injury underwent surgery at younger ages [6 (5-10) versus 10 (8-16) days, p=0.016], and had a higher peak lactate level in the initial 24 hours [5.9 (4.2-9.1) versus 3.4 (2.4-6.7), p=0.007]. Norwood procedure was significantly associated with acute kidney injury [odds ratio 11.7 (95% confidence interval 1.3-101.9), p=0.03]. ICU stay [38 (21-84) versus 16 (6-45) days, p=0.038] and time to extubation [204 (120-606) versus 72 (26-234) hours, p=0.014] were longer in those with acute kidney injury. The two patients who developed early postoperative renal failure as per pRIFLE died before discharge from associated comorbidities. CONCLUSIONS: Acute kidney injury occurs in a third of the patients with single ventricle after surgical palliation but is mostly transient. Norwood, compared with Hybrid procedure, is a risk factor for postoperative acute kidney injury, which, in turn, is associated with longer ICU stay and time to extubation.
Assuntos
Injúria Renal Aguda/etiologia , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Procedimentos de Norwood/efeitos adversos , Cuidados Paliativos/métodos , Complicações Pós-Operatórias/etiologia , Injúria Renal Aguda/epidemiologia , Feminino , Humanos , Recém-Nascido , Tempo de Internação/tendências , Masculino , Procedimentos de Norwood/métodos , Razão de Chances , Complicações Pós-Operatórias/epidemiologia , Estudos Retrospectivos , Fatores de Risco , Estados Unidos/epidemiologiaRESUMO
BACKGROUND: Adults with congenital heart disease (CHD) in the United States now outnumber children with CHD, due in part to the improvement in surgical and medical management. This growing population may present postoperatively to the emergency department (ED) with nonspecific complaints from unforseen complications secondary to cardiac intervention. CASE REPORT: We describe a 39-year-old male who presented to the ED with hematuria and dysuria after he underwent percutaneous device ventricular septal defect (VSD) closure 10 days before. Upon initial evaluation, laboratory results confirmed a urinary tract infection and hematuria. Given persistent red discoloration of urine and easy fatigability, further investigation and re-evaluation found him to be anemic secondary to intravascular hemolysis. Cardiac catheterization showed residual shunting through the VSD device margins causing the hemolysis. Although this is a rare complication of VSD device closure, the patient's initial presentation of hematuria and dysuria presented a unique diagnostic challenge. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Patients with underlying CHD require emergency physicians to consider a multidisciplinary approach to properly diagnose and facilitate treatment.
Assuntos
Anemia/etiologia , Comunicação Interventricular/complicações , Adulto , Anemia/complicações , Anemia/diagnóstico , Procedimentos Cirúrgicos Cardíacos/métodos , Disuria/etiologia , Comunicação Interventricular/cirurgia , Hematúria/etiologia , Humanos , Masculino , Complicações Pós-Operatórias/sangueRESUMO
BACKGROUND: Pruritus is a cardinal symptom of atopic dermatitis, and an increased cutaneous sensory network is thought to contribute to pruritus. Although the immune cell-IL-31-neuron axis has been implicated in severe pruritus during atopic skin inflammation, IL-31's neuropoietic potential remains elusive. OBJECTIVE: We sought to analyze the IL-31-related transcriptome in sensory neurons and to investigate whether IL-31 promotes sensory nerve fiber outgrowth. METHODS: In vitro primary sensory neuron culture systems were subjected to whole-transcriptome sequencing, ingenuity pathway analysis, immunofluorescence, and nerve elongation, as well as branching assays after IL-31 stimulation. In vivo we investigated the cutaneous sensory neuronal network in wild-type, Il31-transgenic, and IL-31 pump-equipped mice. RESULTS: Transgenic Il31 overexpression and subcutaneously delivered IL-31 induced an increase in the cutaneous nerve fiber density in lesional skin in vivo. Transcriptional profiling of IL-31-activated dorsal root ganglia neurons revealed enrichment for genes promoting nervous system development and neuronal outgrowth and negatively regulating cell death. Moreover, the growth cones of primary small-diameter dorsal root ganglia neurons showed abundant IL-31 receptor α expression. Indeed, IL-31 selectively promoted nerve fiber extension only in small-diameter neurons. Signal transducer and activator of transcription 3 phosphorylation mediated IL-31-induced neuronal outgrowth, and pharmacologic inhibition of signal transducer and activator of transcription 3 completely abolished this effect. In contrast, transient receptor potential cation channel vanilloid subtype 1 channels were dispensable for IL-31-induced neuronal sprouting. CONCLUSIONS: The pruritus- and TH2-associated novel cytokine IL-31 induces a distinct transcriptional program in sensory neurons, leading to nerve elongation and branching both in vitro and in vivo. This finding might help us understand the clinical observation that patients with atopic dermatitis experience increased sensitivity to minimal stimuli inducing sustained itch.
Assuntos
Interleucinas/metabolismo , Prurido/imunologia , Prurido/metabolismo , Células Receptoras Sensoriais/metabolismo , Células Th2/imunologia , Células Th2/metabolismo , Animais , Análise por Conglomerados , Gânglios Espinais/citologia , Gânglios Espinais/metabolismo , Expressão Gênica , Perfilação da Expressão Gênica , Humanos , Interleucinas/genética , Camundongos , Camundongos Knockout , Camundongos Transgênicos , Fibras Nervosas/metabolismo , Fosforilação , Prurido/genética , Fator de Transcrição STAT3/metabolismo , Pele/imunologia , Pele/inervação , Pele/metabolismoRESUMO
The specification of vascular patterning in plants has interested plant biologists for many years. In the last decade a new context has emerged for this interest. Specifically, recent proposals to engineer C(4) traits into C(3) plants such as rice require an understanding of how the distinctive venation pattern in the leaves of C(4) plants is determined. High vein density with Kranz anatomy, whereby photosynthetic cells are arranged in encircling layers around vascular bundles, is one of the major traits that differentiate C(4) species from C(3) species. To identify genetic factors that specify C(4) leaf anatomy, we generated ethyl methanesulfonate- and γ-ray-mutagenized populations of the C(4) species sorghum (Sorghum bicolor), and screened for lines with reduced vein density. Two mutations were identified that conferred low vein density. Both mutations segregated in backcrossed F(2) populations as homozygous recessive alleles. Bulk segregant analysis using next-generation sequencing revealed that, in both cases, the mutant phenotype was associated with mutations in the CYP90D2 gene, which encodes an enzyme in the brassinosteroid biosynthesis pathway. Lack of complementation in allelism tests confirmed this result. These data indicate that the brassinosteroid pathway promotes high vein density in the sorghum leaf, and suggest that differences between C(4) and C(3) leaf anatomy may arise in part through differential activity of this pathway in the two leaf types.
Assuntos
Brassinosteroides/metabolismo , Sistema Enzimático do Citocromo P-450/genética , Folhas de Planta/genética , Proteínas de Plantas/genética , Sorghum/genética , Sistema Enzimático do Citocromo P-450/metabolismo , Folhas de Planta/metabolismo , Proteínas de Plantas/metabolismo , Sorghum/metabolismoRESUMO
Our aim was to evaluate the Vasoactive Inotropic Score (VIS) as a prognostic marker in adolescents following surgery for congenital heart disease. This single-center retrospective chart review included patients 10-18 years of age, who underwent cardiac surgery from 2009 to 2014. Hourly VIS was calculated for the initial 48 postoperative hours using standard formulae and incorporating doses of six pressors. The composite adverse outcome was defined as any one of death, resuscitation or mechanical support, arrhythmia, infection requiring antibacterial therapy, acute kidney injury or neurologic injury. Surgeries were risk-stratified by the type of surgical repair using the validated STAT score. Statistical analysis (SPSS 19.0) included Mann-Whitney U test, Chi-square test, ROC curves, and binary regression analysis. Our cohort (n = 149) had a mean (SD) age of 13.9 (2.4) years and included 97 (65.1 %) males. Maximal VIS at 24 and 48 h following surgery was significantly higher in subjects (n = 27) who suffered an adverse outcome. Subjects with adverse outcome had longer bypass and cross-clamp times, durations of stay in the hospital, and a higher rate of acute kidney injury, compared to those (n = 122) without postoperative adverse outcomes. The area under the ROC for maximum VIS at 24-48 h after surgery was 0.76, with sensitivity, specificity, and positive and negative predictive values with 95 % CI of 67 (48-82) %, 74 (70-77) %, and 36 (26-44) % and 91 (86-95) %, respectively, at a cutoff >4.75. On binary logistic regression, maximum VIS on second postoperative day remained significantly associated with adverse outcome (OR 1.35; 95 % CI> 1.12-1.64, p = 0.002). Maximal VIS at 24 and 48 h correlated significantly with length of stay and time to extubation. Maximal VIS on the second postoperative day predicts adverse outcome in adolescents following cardiac surgery. This simple yet robust prognostic indicator may aid in risk stratification and targeted interventions in this population.