RESUMO
INTRODUCTION: Establishing straightforward and reproducible steps to describe the technique performed with the aid of the robotic system for complex hernia surgery is key for good outcomes. Even using the description of open surgery as a parameter for performing the robotic technique, it is important to stress the particularities of this access. To describe the steps to perform robotic-assisted TAR (r-TAR) in a standardized technique, with a critical and safe view of all the anatomical structures. DESCRIPTION OF THE TECHNIQUE: We defined 8 landmarks for the critical view of safety in r-TAR which include: (1) patient position, trocar and docking; (2) posterior rectus sheath mobilization; (3) transversus abdominis release (TAR)-Top-down technique; (4) transversus abdominis release (TAR)-bottom-up technique and mesh insertion; (5) contralateral trocar insertion and redocking, 6) posterior sheath closure; (7) final mesh positioning; and (8) anterior defect closure and drains. DISCUSSION: Complex hernia surgery using a robotic-assisted posterior component separation requires well-established steps so the procedure can be reproducible and achieve better results.
Assuntos
Parede Abdominal , Hérnia Ventral , Procedimentos Cirúrgicos Robóticos , Músculos Abdominais/cirurgia , Parede Abdominal/cirurgia , Hérnia Ventral/cirurgia , Herniorrafia/métodos , Humanos , Procedimentos Cirúrgicos Robóticos/métodos , Telas CirúrgicasRESUMO
The increase in the consumption of fresh produce has correlated with a rise in the number of reported foodborne illnesses. To identify potential risk factors associated with postharvest practices, the present study employed multilocus sequence typing (MLST) for the genotypic classification of Escherichia coli isolates recovered from three sources sampled at seven operational stages in a cantaloupe packinghouse in Northwestern Mexico. The MLST analysis results indicated that the E. coli isolates were classified into 18 different sequence types (ST), and 11 of these STs were found to be novel. ST-171 was the predominant type and was found in 19% (7/36) of the recovered isolates. Interestingly, the novel ST-827 was found to be significantly associated with isolates recovered from workers' hands, sampled during final postwash stages. Further phylogenetic analyses to examine the relatedness of the STs revealed genetic heterogeneity. Fourteen of the identified STs were assigned to known clonal groups, while the remaining four novel STs were distinct and did not cluster with any clonal group. The present study has provided the first evidence indicating that several sources from distinct operational stages in a cantaloupe packinghouse may contribute to a genotypic and phylogenetic diverse set of E. coli isolates. SIGNIFICANCE AND IMPACT OF THE STUDY: Packinghouses can be considered as a potential source of microbial contamination. Using multilocus sequence typing, this study identified a genotypic and phylogenetic diverse set of Escherichia coli isolates recovered from the surfaces of cantaloupes, workers' hands and processing equipment at a cantaloupe packinghouse. A total of 61% of the sequence types identified were novel, and a distinct sequence type, ST-827, was significantly associated with worker's hands, sampled during the final postwash operational stages in the packinghouse. These findings serve as a baseline to identify potential sources of microbial contamination at distinct operational stages in a cantaloupe packinghouse.
Assuntos
Cucumis melo/microbiologia , Escherichia coli/genética , Contaminação de Alimentos , Embalagem de Alimentos , Variação Genética , Técnicas de Tipagem Bacteriana , Escherichia coli/isolamento & purificação , Genótipo , Humanos , México , Tipagem de Sequências Multilocus , FilogeniaRESUMO
The Wolffian duct (WD) undergoes morphological changes induced by androgens to form the epididymis, which is an organ essential for sperm maturation. Androgen action in WD epithelium involves paracrine factors of mesenchymal origin that function by still poorly understood mechanisms. Here we studied the antimicrobial ß-defensin SPAG11C as a new player in duct morphogenesis, localized prenatally in the WD mesenchyme. Organotypic culture of rat WDs and tissues from Androgen Receptor (AR) knockout mice (ARKO) were used. Our results show that androgen/AR signaling differentially regulated SPAG11C expression at mRNA and protein levels in the developing WD. WDs incubated with recombinant human SPAG11C were shorter and less coiled as a result of reduced epithelial cell proliferation, but not increased apoptosis. Our results suggested ß-defensin SPAG11C as an androgen-target required for WD morphogenesis. This highlights the multifunctional repertoire of the ß-defensin protein family and their potential contribution to the in utero environment that determines male reproductive success.
Assuntos
Androgênios/farmacologia , Anti-Infecciosos/farmacologia , Morfogênese/efeitos dos fármacos , Ductos Mesonéfricos/efeitos dos fármacos , beta-Defensinas/farmacologia , Animais , Antígenos de Superfície/metabolismo , Apoptose/efeitos dos fármacos , Proliferação de Células/efeitos dos fármacos , Epididimo/efeitos dos fármacos , Epididimo/metabolismo , Epitélio/efeitos dos fármacos , Epitélio/metabolismo , Feminino , Regulação da Expressão Gênica no Desenvolvimento/efeitos dos fármacos , Humanos , Masculino , Camundongos , Camundongos Knockout , Organogênese/efeitos dos fármacos , Ratos , Ratos Wistar , Receptores Androgênicos/metabolismo , Ductos Mesonéfricos/metabolismoRESUMO
Abies koreana is an endemic and rare species from Korea and is classified as endangered by the International Union for Conservation of Nature. Although the genetic diversity assessment for current population of A. koreana needs to be performed urgently, no microsatellite markers have been developed for this species. In the present study, we developed 22 novel polymorphic microsatellite loci and the characteristics of these loci were determined in A. koreana as well as in Abies nephrolepis, the most closely related species, and these loci were compared with previously reported microsatellite markers developed for the Abies genus. Genomic sequence (161 Mbp; 325,776 reads) was obtained from one individual of A. koreana using Roche 454 GS-FLX Titanium sequencing and 19,258 repeat motifs were identified from it. A total of 288 primer pairs with high copy numbers of di-repeat motifs were evaluated for amplification in A. koreana and A. nephrolepis. A total of 71 primer pairs successfully amplified fragments, of which 22 showed polymorphisms in A. koreana and A. nephrolepis. The average expected diversity was 0.767 and 0.717 in A. koreana and A. nephrolepis, respectively; these heterozygosity levels were moderate compared to the previously reported microsatellite loci from Abies species. This is the first set of microsatellite markers developed for A. koreana as well as A. nephrolepis and further population genetic studies of both species and genetic delimitation can be carried out for the species conservation and management.
Assuntos
Abies/genética , Repetições de Microssatélites , Polimorfismo Genético , Espécies em Perigo de ExtinçãoRESUMO
In total, 216 Hy-Line brown laying hens (40-week-old), were used in a 5-week experiment to evaluate the effects of ionized or chelated water-soluble mineral mixture supplementation on live performance, nutrient digestibility, blood characteristics, egg quality, and excreta microbiota. Layers were randomly assigned to one of three dietary treatments with 12 replicates of six adjacent cages each. The dietary treatments consisted of: 1) CON (basal diet + normal tap water), 2) T1 (CON+0.5% ionized mineral mixture in tap water, pH 3.0); and 3) T2 (CON+ 0.5% chelated mineral mixture in tap water, pH 3.0). Egg production tended to increase in week 1, week 3 and week 4 in the birds supplemented with T1 and T2 diet compared with CON. Moreover, the dietary supplementation of water-soluble mineral mixture improved (p=0.02) eggshell thickness in week 4 and tended to improve in week 5 of the experimental period in T2 hens compared with CON. The layers fed the T1 diet presented higher (p 0.05) Ca digestibility than CON hens, while the T2 diet promoted a numerical increase in Ca digestibility. Blood calcium concentration increased (p 0.05) with T1 and T2 treatments compared with CON. The laying hens fed the T1 and T2 diets tended to present lower Salmonella and E. coli counts isolated from excreta compared with CON. In conclusion, ionized and chelated mineral mixtures had a positive impact on production performance and eggshell quality, improved Ca digestibility and blood Ca level.
Assuntos
Feminino , Animais , Fenômenos Fisiológicos da Nutrição do Lactente , Ovos , Galinhas/metabolismo , Galinhas/sangueRESUMO
In total, 216 Hy-Line brown laying hens (40-week-old), were used in a 5-week experiment to evaluate the effects of ionized or chelated water-soluble mineral mixture supplementation on live performance, nutrient digestibility, blood characteristics, egg quality, and excreta microbiota. Layers were randomly assigned to one of three dietary treatments with 12 replicates of six adjacent cages each. The dietary treatments consisted of: 1) CON (basal diet + normal tap water), 2) T1 (CON+0.5% ionized mineral mixture in tap water, pH 3.0); and 3) T2 (CON+ 0.5% chelated mineral mixture in tap water, pH 3.0). Egg production tended to increase in week 1, week 3 and week 4 in the birds supplemented with T1 and T2 diet compared with CON. Moreover, the dietary supplementation of water-soluble mineral mixture improved (p=0.02) eggshell thickness in week 4 and tended to improve in week 5 of the experimental period in T2 hens compared with CON. The layers fed the T1 diet presented higher (p 0.05) Ca digestibility than CON hens, while the T2 diet promoted a numerical increase in Ca digestibility. Blood calcium concentration increased (p 0.05) with T1 and T2 treatments compared with CON. The laying hens fed the T1 and T2 diets tended to present lower Salmonella and E. coli counts isolated from excreta compared with CON. In conclusion, ionized and chelated mineral mixtures had a positive impact on production performance and eggshell quality, improved Ca digestibility and blood Ca level.(AU)
Assuntos
Animais , Feminino , Fenômenos Fisiológicos da Nutrição do Lactente , Ovos , Galinhas/sangue , Galinhas/metabolismoRESUMO
Coronary artery disease (CAD), a multifactorial disease, is a common cause of mortality in humans. Polymorphisms in the endothelial nitric oxide synthase (eNOS) gene (-786T>C, 4a4b, and 894G>T) have been previously associated with increased CAD risk. However, the sample size of this previous study was too small and limited to comprehensively define an association between eNOS polymorphisms and CAD; therefore, this analysis was duplicated with a larger population. The study was conducted on 559 patients with CAD and 574 healthy controls. Genetic DNA was extracted using the commercial G-DEX blood extraction kit and statistical analyses were performed on the GraphPad prism 4.0 and MedCalc 12.0 statistical software platforms. No single variant of the eNOS polymorphism was associated with CAD risk. The combination genotypes of eNOS -786TT/4a4b+4a4a [adjusted odds ratio (AOR) = 0.122; 95% confidence interval (CI): 0.042-0.358] and eNOS -786TC+CC/4b4b (AOR = 0.379; 95%CI: 0.147-0.979) were associated with decreased CAD incidence. Haplotype analysis revealed that the T-4a haplotype of eNOS -786T>C and 4a4b exerted a protective effect against CAD. The association between eNOS -786T>C and increased CAD risk was not replicated in this (larger) population. However, some combined genotypes showed a meaningful association with CAD risk.
Assuntos
Povo Asiático/genética , Doença da Artéria Coronariana/genética , Estudos de Associação Genética , Predisposição Genética para Doença , Óxido Nítrico Sintase Tipo III/genética , Polimorfismo Genético , Idoso , Alelos , Estudos de Casos e Controles , Comorbidade , Doença da Artéria Coronariana/sangue , Doença da Artéria Coronariana/epidemiologia , Feminino , Ácido Fólico/sangue , Frequência do Gene , Genótipo , Haplótipos , Homocisteína/sangue , Humanos , Desequilíbrio de Ligação , Masculino , Pessoa de Meia-Idade , Razão de Chances , Polimorfismo de Nucleotídeo Único , República da Coreia/epidemiologia , Risco , Fatores de RiscoRESUMO
The red fox, Vulpes vulpes (Canidae), is the most widely distributed terrestrial carnivore worldwide, but this species is classified as endangered in Korea. In this study, we developed 25 polymorphic microsatellite markers that included 3-13 (mean = 6.32) alleles per locus using 22 red fox individuals. The most polymorphic locus was FR(59)TG (13 alleles) and the least polymorphic loci were FR(70)TG and FR(182)AG (3 alleles each). No significant deviation from Hardy-Weinberg equilibrium (P < 0.05) was observed for the 25 markers. Observed (HO) and expected (HE) heterozygosity varied from 0.182 to 1.000 and from 0.175 to 0.929, respectively. These newly developed microsatellite markers will be useful for investigating the genetic diversity and population genetic structure of V. vulpes and will aid in developing conservation strategies for this species.
Assuntos
Raposas/genética , Variação Genética , Genética Populacional , Sequenciamento de Nucleotídeos em Larga Escala/veterinária , Repetições de Microssatélites/genética , Alelos , Animais , Sequência de Bases , Conservação dos Recursos Naturais , Espécies em Perigo de Extinção , Heterozigoto , Sequenciamento de Nucleotídeos em Larga Escala/métodos , Dados de Sequência Molecular , República da Coreia , Análise de Sequência de DNA/veterináriaRESUMO
Os modelos de apoio à decisão são importantes ferramentas que podem ser utilizadas para analisar alternativas tecnológicas na gestão e tratamento de resíduos sólidos urbanos (RSU) frente a diversas situações envolvendo aspectos políticos, econômicos, ambientais e sociais. Este estudo teve como objetivo propor tecnologias e arranjos tecnológicos para o tratamento adequado de RSU para a Região Sul do Brasil, por meio do levantamento de informações e uso de dois modelos de apoio à decisão: AHP (Analytic Hierarchy Process) e Promethee II (Preference Ranking Organization Method for Enrichment Evaluations). As tecnologias analisadas foram: reciclagem, compostagem, tratamento mecânico-biológico, digestão anaeróbia, incineração com geração de energia elétrica em ciclo combinado (energia elétrica e térmica), aterro sanitário com e sem geração de energia. Essas tecnologias foram hierarquizadas nos modelos com base em quatro critérios: ambientais, sociais, econômicos e políticos. Como resultado, foram propostos quatro arranjos tecnológicos possíveis para a Região Sul e que podem ser utilizados como referência para estudos nas demais regiões geográficas do Brasil. .
The decision support models are important tools that can be used to analyze technological alternatives in the management and treatment of municipal solid waste (MSW) against several situations involving political, economic, environmental and social aspects. This study aimed to propose alternatives for appropriate treatment of MSW in the South Region of Brazil by surveying information and using two decision support models: AHP (Analytic Hierarchy Process) and Promethee II (Preference Ranking Organization Method for Enrichment Evaluations). The studied technologies were: recycling, composting, mechanical-biological treatment, anaerobic digestion, incineration with electricity generation and combined cycle (electricity and heat) and landfill with and without power generation. These technologies were hierarchically ranked in the models based on four criteria: environmental, social, economic and political. As a result, we proposed four possible technological arrangements for the South Region and these can be used as reference for studies in other geographic regions of Brazil. .
RESUMO
There is comparatively little information on health-related quality of life (HRQoL) in subjects with allergic rhinitis (AR) or allergic rhinoconjunctivitis (AR/C) in countries beyond western Europe and North America. The primary aim of this investigation was therefore to review and assess the information in the public domain on HRQoL in AR/C patients from diverse regions of the world, represented by different countries, including Argentina, Australia, Brazil, Russia, Singapore, South Africa and Turkey. Second, in view of the absence of a standardized definition for 'AR control', the review aimed to determine whether a working definition of AR/C can be inferred from validated tests or other instruments documented to date. Despite the comparatively low number of studies, this review demonstrated that overall the symptoms of AR/C impair the HRQoL of patients in these regions by adversely impacting sleep, daily activities, physical and mental status and social functioning, similar to that demonstrated in much larger numbers of studies of AR/C patients in Europe and the United States. Furthermore, the findings of the review suggest that 'overall' control of the disease should encompass reduction of nasal and ocular symptoms, as well as improvements in HRQoL, comorbid conditions and cognition. Although some instruments are currently available for measuring control of AR, none are capable of assessing all these aspects, emphasizing the need to develop appropriate new instruments.
Assuntos
Conjuntivite Alérgica/fisiopatologia , Qualidade de Vida , Rinite Alérgica Perene/fisiopatologia , Argentina , Austrália , Brasil , Conjuntivite Alérgica/epidemiologia , Conjuntivite Alérgica/prevenção & controle , Humanos , Rinite Alérgica , Rinite Alérgica Perene/epidemiologia , Rinite Alérgica Perene/prevenção & controle , Federação Russa , Singapura , África do Sul , TurquiaRESUMO
An interspecific artificial hybrid was produced between two economically important aquaculture flatfish: olive flounder (Paralichthys olivaceus) and starry flounder (P. stellatus). This hybrid displays the rapid growth characteristic of the former and tolerance to low temperatures and low salinity of the latter, but the genetics of inheritance in this hybrid have not been elucidated. Polymorphic microsatellite markers developed for P. olivaceus and P. stellatus were tested to determine if these markers can be used for analysis of parentage and genetic inheritance. Multiplex PCR using two primer sets that were specific to each species produced PCR products of different sizes; these could be used for the identification of interspecific hybrids. Among the 192 primers derived from olive flounder, 25.5% of the primer sets successfully amplified genomic DNA from starry flounder, and 23% of the 56 primer sets originating from starry flounder amplified DNA from olive flounder. Analysis of genetic inheritance in the hybrid using seven of the 62 microsatellite markers common to both species demonstrated classic Mendelian inheritance of these markers in the hybrid progeny, with the exception of one locus identified as a null allele in the hybrid. These results demonstrate that cross-specific microsatellite markers can be used tools for parentage analysis of hybrid flatfish, for mapping quantitative trait loci, for marker-assisted selective breeding, and for studies of the evolution of fish.
Assuntos
Adaptação Fisiológica/genética , Cruzamento , Quimera/genética , DNA/genética , Linguado/genética , Repetições de Microssatélites , Animais , Quimera/crescimento & desenvolvimento , Primers do DNA/química , Primers do DNA/genética , Linguado/crescimento & desenvolvimento , Reação em Cadeia da Polimerase/métodos , Salinidade , Especificidade da EspécieRESUMO
BACKGROUND: In 2005, 80% of cardiovascular disease (CVD) deaths occurred in low- to middle-income countries (i.e., developing nations). Cardiovascular imaging, such as myocardial perfusion SPECT, is one method that may be applied to detect and foster improved detection of at-risk patients. This document will review the availability and utilization for nuclear cardiology procedures worldwide and propose strategies to devise regional centers of excellence to achieve quality imaging around the world. METHODS: As a means to establish the current state of nuclear cardiology, International Atomic Energy Agency member and non-member states were queried as to annual utilization of nuclear cardiology procedures. Other sources for imaging statistics included data from medical societies (American Society of Nuclear Cardiology, European Society of Cardiology, and the European Association of Nuclear Medicine) and nuclear cardiology working groups within several nations. Utilization was calculated by dividing annual procedural volume by 2007 population statistics (/100,000) and categorized as high (>1,000/100,000), moderate-high (250-999/100,000), moderate (100-249/100,000), low-moderate (50-99/100,000) and low (<50/100,000). RESULTS: High nuclear cardiology utilization was reported in the United States, Canada, and Israel. Most Western European countries, Australia, and Japan reported moderate-high utilization. With the exception of Argentina, Brazil, Colombia and Uruguay, South America had low usage. This was also noted across Eastern Europe, Russia, and Asia. Utilization patterns generally mirrored each country's gross domestic product. However, nuclear cardiology utilization was higher for developing countries neighboring moderate-high "user" countries (e.g., Algeria and Egypt); perhaps the result of accessible high-quality training programs. CONCLUSIONS: Worldwide utilization patterns for nuclear cardiology vary substantially and may be influenced by physician access to training and education programs. Development of regional training centers of excellence can guide utilization of nuclear cardiology through the application of guideline- and appropriateness-driven testing, training, continuing education, and quality assurance programs aiding developing nations to confront the epidemics of CVD.
Assuntos
Cardiologia/estatística & dados numéricos , Diagnóstico por Imagem/estatística & dados numéricos , Acessibilidade aos Serviços de Saúde/estatística & dados numéricos , Avaliação das Necessidades , Medicina Nuclear/estatística & dados numéricos , Países DesenvolvidosRESUMO
BACKGROUND: Differences in the IgE response to isoallergens could have clinical implications; therefore, its analysis will contribute to the design of better strategies for the diagnosis and treatment of allergic respiratory diseases. Several isoforms have been described from mites but there is no information about the clinical impact of Blomia tropicalis isoallergens. OBJECTIVE: To evaluate the differences in the IgE response against two Blo t 12 isoallergens. METHODS: The IgE-binding properties of Blo t 12 isoallergens were analysed by ELISA, a skin prick test and ELISA cross-inhibition. Epitope mapping was performed using synthetic overlapping peptides. Fold recognition methods were used to model the chitin-binding domain of the two isoallergens. RESULTS: The frequency and strength of the IgE response were greater for Blo t 12.0101 than for Blo t 12.0102. Three IgE-binding areas were identified in Blo t 12.0101; one of them included two residues that are different in Blo t 12.0102. Modelling of the chitin-binding domains of these allergens predicted that they have structural differences that could influence antibody recognition of one of these epitopes. CONCLUSION: In silico structural analysis and immunological characterization of Blo t 12 reveals that allergen polymorphism influences IgE reactivity. Blo t 12.0101 is the most IgE-reactive isoform in Cartagena. The identified IgE epitopes could be mutated to obtain hypoallergenic molecules of potential use for immunotherapy.
Assuntos
Alérgenos/imunologia , Asma/imunologia , Imunoglobulina E/sangue , Pyroglyphidae/imunologia , Adolescente , Alérgenos/química , Sequência de Aminoácidos , Animais , Criança , Clonagem Molecular , Reações Cruzadas/imunologia , Mapeamento de Epitopos , Humanos , Dados de Sequência Molecular , Peptídeos/imunologia , Isoformas de Proteínas/química , Isoformas de Proteínas/imunologia , Pyroglyphidae/química , Alinhamento de Sequência , Testes CutâneosRESUMO
We report a 25 years old woman with a four years history of end stage renal disease on hemodialysis. During the last two years of follow up, multiple tender nodules appeared in the left clavicle and ribs. These were diagnosed as brown tumors, secondary to osteitis fibrosa cystica, in the context of a secondary hyperparathyroidism. A subtotal parathyroidectomy was performed and a homologous parathyroid implant was done in the right forearm. The pathological study of the surgical pieces confirmed the presence of the brown tumors and parathyroid hyperplasia. Nine months after surgery, parathormone levels and brown tumor calcifications decreased significantly and bone mineral density increased by 20 per cent.
Assuntos
Humanos , Feminino , Adulto , Neoplasias Primárias Múltiplas/complicações , Distúrbio Mineral e Ósseo na Doença Renal Crônica/complicações , Glândulas Paratireoides/patologia , Hiperparatireoidismo Secundário/complicações , Hiperparatireoidismo Secundário/diagnóstico , Hiperparatireoidismo Secundário/cirurgia , Hiperplasia/complicações , Hiperplasia/diagnóstico , Neoplasias Primárias Múltiplas/diagnóstico , Distúrbio Mineral e Ósseo na Doença Renal Crônica/diagnóstico , Osteíte Fibrosa Cística/complicações , Osteíte Fibrosa Cística/diagnósticoRESUMO
OBJECTIVE: We tested the hypothesis that adding montelukast to budesonide would improve asthma control in children with inhaled glucocorticoid-dependent persistent asthma. STUDY DESIGN: In a multicenter, randomized, double-blind, crossover study, we compared the benefit of adding montelukast, 5 mg, or placebo once daily to budesonide, 200 microg, twice daily. RESULTS: After a 1-month run-in with budesonide, 200 microg, twice daily, 279 children were randomized to montelukast or placebo. The mean +/- SD age was 10.4 +/- 2.2 years, the mean forced expiratory volume in 1 second (FEV(1)) was 77.7% +/- 10.6% predicted, and reversibility was 18.1% +/- 12.9%. Compared with adding placebo to budesonide, adding montelukast produced significant improvements in mean percent change from baseline FEV(1) (P =.062 [P =.010 for per-protocol analysis]), mean absolute change from baseline FEV(1) (P =.040), mean increase from baseline in morning (P =.023) and evening (P =.012) peak expiratory flows, decrease in exacerbation days by approximately 23% (P <.001), decreased beta2-agonist use (P =.013), and reduced blood eosinophil counts (P <.001). The treatments did not differ significantly with regard to safety. CONCLUSIONS: Montelukast, 5 mg, added to budesonide improved asthma control significantly, indicated by a small additive effect on lung function and a clinically relevant decrease in asthma exacerbation days.
Assuntos
Acetatos/uso terapêutico , Antiasmáticos/uso terapêutico , Asma/tratamento farmacológico , Quinolinas/uso terapêutico , Acetatos/administração & dosagem , Acetatos/efeitos adversos , Antiasmáticos/administração & dosagem , Antiasmáticos/efeitos adversos , Asma/fisiopatologia , Budesonida/administração & dosagem , Budesonida/efeitos adversos , Budesonida/uso terapêutico , Ciclopropanos , Método Duplo-Cego , Quimioterapia Combinada , Volume Expiratório Forçado/fisiologia , Humanos , Valor Preditivo dos Testes , Qualidade de Vida , Quinolinas/administração & dosagem , Quinolinas/efeitos adversos , Sulfetos , Resultado do TratamentoRESUMO
Long-term correction of urea cycle disorders is achieved by correction of the enzymatic defect in hepatocytes. Currently, orthotopic liver transplantation is the primary means of achieving this correction. In the United States most liver transplantations for urea cycle disorders have been restricted to patients with ornithine transcarbamylase deficiency and argininosuccinic aciduria. However, patients with citrullinemia have also received transplants, but more so in Europe and Japan. Recent advances in organ procurement, surgical technique, and immunosuppression have significantly decreased morbidity and mortality. However, unique short-term complications associated with surgery and long-term complications associated with chronic immunosuppression have spurred continued efforts to develop gene replacement therapies for management of acute metabolic decompensations as intercurrent therapy until liver transplantation, and ultimately, for long-term correction. The pathophysiology of urea cycle disorders requires gene vector delivery systems that are highly efficient for liver transduction and transgene expression. To date, adenoviral vectors are unique in fulfilling these criteria, and significant data have been gained in both animal and human studies with early versions of adenoviral vectors. Ultimately, the development of helper-dependent adenoviral vectors may offer the long-term expression and increased margin of safety necessary for adjunctive therapies.
Assuntos
Erros Inatos do Metabolismo dos Aminoácidos/terapia , Transplante de Fígado , Ureia/metabolismo , Adenoviridae/genética , Erros Inatos do Metabolismo dos Aminoácidos/metabolismo , Erros Inatos do Metabolismo dos Aminoácidos/fisiopatologia , Erros Inatos do Metabolismo dos Aminoácidos/cirurgia , Animais , Criança , Contraindicações , Vetores Genéticos , HumanosRESUMO
The Chinese delicacy "bird's nest" is the most common cause of food-induced anaphylaxis requiring hospitalization among Chinese children in Singapore. This investigation has established an immunoglobulin E-mediated cause and has characterized the major putative allergens.