RESUMO
Global energy demand is increasing rapidly and due to intensive consumption of different forms of fuels, there are increasing concerns over the reduction in readily available conventional energy resources. Because of the deleterious atmospheric effects of fossil fuels and the uncertainties of future energy supplies, there is a surge of interest to find environmentally friendly alternative energy sources. Hydrogen (H2) has attracted worldwide attention as a secondary energy carrier, since it is the lightest carbon-neutral fuel rich in energy per unit mass and easy to store. Several methods and technologies have been developed for H2 production, but none of them are able to replace the traditional combustion fuel used in automobiles so far. Extensively modified and renovated methods and technologies are required to introduce H2 as an alternative efficient, clean, and cost-effective future fuel. Among several emerging renewable energy technologies, photobiological H2 production by oxygenic photosynthetic microbes such as green algae and cyanobacteria or by artificial photosynthesis has attracted significant interest. In this short review, we summarize the recent progress and challenges in H2-based energy production by means of biological and artificial photosynthesis routes.
Assuntos
Clorófitas/fisiologia , Cianobactérias/fisiologia , Hidrogênio/metabolismo , Oxigênio/metabolismo , Fotossíntese , Metabolismo Energético , Nanotecnologia , FotobiologiaRESUMO
The aim of this study was to compare the prognosis of separated and non-separated tooth autotransplantation of the upper first and second molars with complete root formation undertaken at dental clinics. The participating dentists were requested to provide information on transplantations they had undertaken from 1 January 1990 to 31 December 2010. Data on a total of 708 teeth from 637 patients were collected. This study analysed 35 separated teeth and 22 non-separated teeth of 47 participants ranging from 27 to 76 years of age (mean age: 55·0 years) after data screening and elimination. The cumulative post-transplantation survival rate at 10 years was 77·1% for separated teeth and 63·6% for non-separated teeth as calculated with the Kaplan-Meier method. There were no significant differences between separated teeth and non-separated teeth in a log rank test (P = 0·687). Separated-tooth autotransplantation can help fill narrow recipient sites and increase occlusal supporting zones, but the clinical success rate was only 48·6%. Although transplantation of teeth with complete root formation has limited prognosis, transplantation of upper first and second molars, whether separated or non-separated, is a viable option to replace missing teeth.
Assuntos
Arcada Parcialmente Edêntula/cirurgia , Dente Molar/transplante , Procedimentos Cirúrgicos Bucais/métodos , Raiz Dentária/transplante , Adulto , Idoso , Feminino , Humanos , Masculino , Maxila/cirurgia , Pessoa de Meia-Idade , Prognóstico , Transplante Autólogo/métodosRESUMO
Gender-related risk factors in the survival of transplanted teeth with complete root formation have not yet been identified. The purpose of this study was to investigate gender differences in tooth autotransplantation at dental clinics. We asked participating dentists to provide information on transplantations they had undertaken from 1 January 1990 to 1931 December 2010. The data were screened to exclude patients who underwent more than one transplantation, smokers or those whose smoking habits were unknown, patients under 30 or who were 70 years old and over, cases where the transplanted teeth had incomplete root formation or multiple roots and those with fewer than 20 present teeth post-operation. We analysed 73 teeth of 73 males (mean age, 47.2 years) and 106 teeth of 106 females (mean age, 45.3 years) in this study. The cumulative survival rate and mean survival time were calculated using the Kaplan-Meier method. The cumulative survival rate for males was 88.3% at the 5-year mark, 64.8% at 10 years and 48.6% at 15 years; for females, it was 97.2% at the 5-year mark, 85.9% at 10 years and 85.9% at 15 years. A log-rank test indicated the difference between males and females to be significant (P = 0.011). There was also a significant difference in the main causes for the loss of transplanted teeth: males lost more transplanted teeth due to attachment loss than females (P < 0.05). These results indicate that males require more attention during the autotransplantation process, particularly at the stage of pre-operation evaluation and that of follow-up maintenance.
Assuntos
Raiz Dentária/anatomia & histologia , Dente/transplante , Adulto , Idoso , Dente Pré-Molar/patologia , Dente Pré-Molar/transplante , Feminino , Seguimentos , Sobrevivência de Enxerto , Humanos , Masculino , Pessoa de Meia-Idade , Dente Molar/patologia , Dente Molar/transplante , Odontogênese/fisiologia , Perda da Inserção Periodontal/complicações , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Fatores Sexuais , Fatores de Tempo , Perda de Dente/etiologia , Transplante Autólogo , Resultado do TratamentoRESUMO
The aim of this study was to investigate risk factors with age in the long-term prognosis of autotransplantation of teeth with complete root formation at dental clinics. Participating dentists were asked to provide information on transplantations they had undertaken from 1 January 1990 to 31 December 2010. Data on a total of 708 teeth from 637 patients were collected. The data were screened to exclude patients who were under 25 or 70 years of age and over, those who were smokers or whose smoking habits were unknown, those whose transplanted teeth had incomplete root formation or multiple roots and those with fewer than 25 present teeth post-operation. The participants in this study were 71 men (74 teeth) and 100 women (107 teeth) ranging from 25 to 69 years of age. Third molars were used as donor teeth in 89·0% of the cases. The participants were divided into three age groups of 25-39, 40-54 and 55-69. Survival analysis was conducted using the Kaplan-Meier method, and a log-rank test revealed that there were no significant differences in age groups for men or women. Cox regression analysis indicated that the survival of transplanted teeth was not influenced by age. However, although not statistically significant, the clinical success rate was lower in the 55-69-year-old group than that in the younger groups. These results indicate that if suitable donor teeth are available and the conditions are right, autotransplantation is a viable treatment for missing teeth regardless of the age of the patient.
Assuntos
Raiz Dentária/crescimento & desenvolvimento , Dente/transplante , Adulto , Fatores Etários , Idoso , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Dente Serotino/transplante , Prognóstico , Modelos de Riscos Proporcionais , Transplante AutólogoRESUMO
BACKGROUND: Mesothelin is expressed in various types of malignant tumour, and we recently reported that expression of mesothelin was related to an unfavourable patient outcome in pancreatic ductal adenocarcinoma. In this study, we examined the clinicopathological significance of the mesothelin expression in gastric cancer, especially in terms of its association with the staining pattern. METHODS: Tissue specimens from 110 gastric cancer patients were immunohistochemically examined. The staining proportion and intensity of mesothelin expression in tumour cells were analysed, and the localisation of mesothelin was classified into luminal membrane and/or cytoplasmic expression. RESULTS: Mesothelin was positive in 49 cases, and the incidence of mesothelin expression was correlated with lymph-node metastasis. Furthermore, luminal membrane staining of mesothelin was identified in 16 cases, and the incidence of luminal membrane expression was also correlated with pT factor, pStage, lymphatic permeation, blood vessel permeation, recurrence, and poor patient outcome. Multivariate analysis showed that luminal membrane expression of mesothelin was an independent predictor of overall patient survival. CONCLUSION: We described that the luminal membrane expression of mesothelin was a reliable prognostic factor in gastric cancer, suggesting the functional significance of membrane-localised mesothelin in the aggressive behaviour of gastric cancer cells.
Assuntos
Proteínas Ligadas por GPI/metabolismo , Neoplasias Gástricas/metabolismo , Idoso , Biomarcadores Tumorais/metabolismo , Células Epiteliais/metabolismo , Feminino , Humanos , Imuno-Histoquímica , Linfonodos/metabolismo , Metástase Linfática , Masculino , Mesotelina , Pessoa de Meia-Idade , Neoplasias Gástricas/mortalidade , Neoplasias Gástricas/patologia , Análise de SobrevidaAssuntos
Carcinoma Basocelular/química , Carcinoma Basocelular/patologia , Inibidor de Quinase Dependente de Ciclina p27/análise , Quinases Ciclina-Dependentes/análise , Neoplasias Cutâneas/química , Neoplasias Cutâneas/patologia , Idoso , Proliferação de Células , Feminino , Humanos , Imuno-Histoquímica , Coloração e RotulagemRESUMO
The aim of this study was to investigate the usage of tooth autotransplantation in dental clinics which offer the treatment and evaluate its practicality. Participating dentists were requested to provide information on transplantations they had undertaken from 1 January 1990 to 31 December 2010. A total of 614 teeth from 552 patients (37 dentists) ranging in age from 17 to 79 (mean age: 44·1) were examined. Cumulative survival rate and mean survival time were calculated using the Kaplan-Meier method, and log rank test was used for analysis of factors. The mean number of autotransplantation patients per clinic per year was 1·4. Upper third molars constituted 36·8% of donor teeth, while 37·1% were lower third molars. The lower first molar region was the most common recipient site at 32·6%, followed by the lower second molar region (28·0%). Prosthodontic treatment of transplanted teeth involved coverage with a single crown (72·5%) and abutment of bridge (18·9%). A total of 102 transplanted teeth were lost owing to complications such as attachment loss (54·9%) and root resorption (25·7%). The cumulative survival rate in cases where donor teeth had complete root formation was 90·1% at 5 years, 70·5% at 10 years and 55·6% at 15 years. The mean survival time was 165·6 months. Older age was a significant risk factor (P < 0·05) for survival. In cases where suitable donor teeth are available, autotransplantation of teeth may be a plausible treatment option for dealing with missing teeth in dental clinics.
Assuntos
Procedimentos Cirúrgicos Bucais/estatística & dados numéricos , Dente/transplante , Adolescente , Adulto , Idoso , Clínicas Odontológicas , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Transplante Autólogo/estatística & dados numéricos , Resultado do Tratamento , Adulto JovemRESUMO
The aim of this study was to investigate the risk factors affecting long-term prognosis of autotransplantation of third molars with complete root formation in males at dental clinics. Participating dentists were requested to provide information on transplantations they had undertaken from 1 January 1990 to 31 December 2010. Data on a total of 708 teeth from 637 patients were collected. After data screening and elimination, participants of this study consisted of 183 teeth of 171 males ranging from 20 to 72 years of age (mean age, 44·8 years). The cumulative survival rate was 86·0% at the 5-year mark, 59·1% at 10 years and 28·0% at 15 years. The mean survival time was 134·5 months, as calculated by the Kaplan-Meier method. Single factor analysis using the log-rank test showed that the following factors had significant influence (P < 0·05) on survival of transplanted teeth: periodontal disease as the reason for recipient site tooth extraction, fewer than 25 present teeth and Eichner index Groups B1 to C. Cox regression analysis examined five factors: age, smoking habit, recipient site extraction caused by periodontal disease, fewer than 25 present teeth and Eichner index. This analysis showed that two of these factors were significant: fewer than 25 present teeth was 2·63 (95% CI, 1·03-6·69) and recipient site extraction caused by periodontal disease was 3·80 (95% CI, 1·61-9·01). The results of this study suggest that long-term survival of transplanted teeth in males is influenced not only by oral bacterium but also by occlusal status.
Assuntos
Dente Serotino/transplante , Adulto , Fatores Etários , Idoso , Coroas , Dente Suporte , Cárie Dentária/etiologia , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Perda da Inserção Periodontal/etiologia , Periodontite/complicações , Complicações Pós-Operatórias , Estudos Retrospectivos , Fatores de Risco , Tratamento do Canal Radicular , Reabsorção da Raiz/etiologia , Fatores Sexuais , Fumar , Análise de Sobrevida , Anquilose Dental/etiologia , Extração Dentária , Fraturas dos Dentes/etiologia , Raiz Dentária/lesões , Alvéolo Dental/cirurgia , Transplante Autólogo , Resultado do Tratamento , Adulto JovemRESUMO
Zeolite-templated carbons (ZTCs) are ordered microporous carbons synthesized by using zeolite as a sacrificial template. Unlike well-known ordered mesoporous carbons obtained by using mesoporous silica templates, ZTCs consist of curved and single-layer graphene frameworks, thereby affording uniform micropore size (ca. 1.2 nm), developed microporosity (â¼1.7 cm3 g-1), very high surface area (â¼4000 m2 g-1), good compatibility with chemical modification, and remarkable softness/elasticity. Thus, ZTCs have been used in many applications such as hydrogen storage, methane storage, CO2 capture, liquid-phase adsorption, catalysts, electrochemical capacitors, batteries, and fuel cells. Herein, the relevant research studies are summarized, and the properties as well as the performances of ZTCs are compared with those of other materials including metal-organic frameworks, to elucidate the intrinsic advantages of ZTCs and their future development.
RESUMO
Development of the anterior pituitary proceeds via spatiotemporal patterning of transcription factors and signalling molecules. Among them, retinoic acid (RA) functions as an important signalling molecule for vertebrate organogenesis in many tissues. However, little is known regarding the target genes in the developing pituitary. The present study aimed to clarify the relationship between endogenous RA signalling and mRNA expression of the pituitary-specific transcription factor Prop1 in the pituitary primordium of Rathke's pouch. Gene expression analysis and in situ hybridisation demonstrated that retinaldehyde dehydrogenases (Raldhs) and all types of RA receptors (Rars) are expressed at the level of transcription in the rat Rathke's pouch. Ex vivo organ culture using Rathke's pouch and an in vitro reporter assay demonstrated that RA signalling increases the expression level of Prop1 via RARα. Moreover, a reporter assay using serial truncated constructs of the 5'-upstream region of mouse Prop1 revealed a predicted cis-regulatory element of RARα. This is the first report of a relationship between RA signalling and Prop1-expression during early pituitary development.
Assuntos
Regulação da Expressão Gênica no Desenvolvimento , Proteínas de Homeodomínio/metabolismo , Hipófise/metabolismo , Transdução de Sinais/fisiologia , Tretinoína/metabolismo , Animais , Proteínas de Homeodomínio/genética , Camundongos , Hipófise/embriologia , Ratos , Ratos Wistar , Receptores do Ácido Retinoico/genética , Receptores do Ácido Retinoico/metabolismo , Receptores X de Retinoides/genética , Receptores X de Retinoides/metabolismoRESUMO
NAD+ (oxidized form of NAD:nicotinamide adenine dinucleotide)-reducing soluble [NiFe]-hydrogenase (SH) is phylogenetically related to NADH (reduced form of NAD+):quinone oxidoreductase (complex I), but the geometrical arrangements of the subunits and Fe-S clusters are unclear. Here, we describe the crystal structures of SH in the oxidized and reduced states. The cluster arrangement is similar to that of complex I, but the subunits orientation is not, which supports the hypothesis that subunits evolved as prebuilt modules. The oxidized active site includes a six-coordinate Ni, which is unprecedented for hydrogenases, whose coordination geometry would prevent O2 from approaching. In the reduced state showing the normal active site structure without a physiological electron acceptor, the flavin mononucleotide cofactor is dissociated, which may be caused by the oxidation state change of nearby Fe-S clusters and may suppress production of reactive oxygen species.
Assuntos
Proteínas de Bactérias/química , Hidrogenase/química , NAD/química , Sítios de Ligação , Oxirredução , Conformação Proteica , Subunidades Proteicas/química , SolubilidadeRESUMO
Synovial sarcoma accounts for almost 10% of all soft tissue sarcomas, and its prognosis is poor with 5-year survival rates at 36%. Thus, new treatments and therapeutic targets for synovial sarcoma are required. Tumor-initiating cells have been defined by the ability for self-renewal and multipotent differentiation, and they exhibit higher tumorigenic capacity, chemoresistance and radiation resistance, expecting to be a new therapeutic target. In synovial sarcoma, the presence of such stemness remains largely unclear; thus, we analyzed whether synovial sarcoma possessed tumor-initiating cells and explored specific markers, and we discovered that synovial sarcoma cell lines possessed heterogeneity by way of containing a sphere-forming subpopulation highly expressing NANOG, OCT4 and SOX2. By expression microarray analysis, CXCR4 was identified to be highly expressed in the sphere subpopulation and correlated with stem-cell-associated markers. Inhibition of CXCR4 suppressed the cell proliferation of synovial sarcoma cell lines in vitro. The tumor-initiating ability of CXCR4-positive cells was demonstrated by xenograft propagation assay. CXCR4-positive cells showed higher tumorigenicity than negative ones and possessed both self-renewal and multipotent differentiation ability. Immunohistochemical analysis of 39 specimens of synovial sarcoma patients revealed that CXCR4 strongly correlated with poor prognosis of synovial sarcoma. Thus, we conclude that CXCR4 is the marker of synovial sarcoma-initiating cells, a new biomarker for prognosis and a new potential therapeutic target.
Assuntos
Células-Tronco Neoplásicas/química , Receptores CXCR4/análise , Sarcoma Sinovial/patologia , Animais , Biomarcadores Tumorais/análise , Linhagem Celular Tumoral , Feminino , Humanos , Camundongos , Proteínas de Fusão Oncogênica/fisiologia , Prognóstico , Receptores CXCR4/fisiologia , Sarcoma Sinovial/imunologiaRESUMO
Human DOCK180, which was originally identified as a major protein bound to the Crk oncogene product, is an archetype of the CDM family of proteins, including Ced-5 of Caenorhabditis elegans and Mbc of Drosophila melanogaster. After DOCK180, at least three putative human proteins that manifest high amino acid sequence similarity to DOCK180 have been registered in the GenBank/EMBL database. We have designated one of them, KIAA0209, as DOCK2 and characterize here. DOCK2 mRNA was expressed mostly in peripheral blood cells, followed by slight expression in the spleen and thymus, whereas DOCK180 was expressed in all tissues tested except in peripheral blood cells. Immunostaining of human cadaver tissues revealed that the expression of DOCK2 was limited to the lymphocytes and macrophages of various organs. DOCK2 bound to and activated Rac1, as did DOCK180; however, DOCK2 did not bind to CrkII, which transduces signals at focal adhesions. Thus, DOCK180 and DOCK2 are regulators of Rac and function in adherent and non-adherent cells, respectively.
Assuntos
Proteínas de Caenorhabditis elegans , Proteínas de Transporte/metabolismo , Fatores de Troca do Nucleotídeo Guanina , Proteínas/metabolismo , Proteínas Proto-Oncogênicas , Sequência de Aminoácidos , Proteínas de Transporte/química , Proteínas de Transporte/isolamento & purificação , DNA Complementar/química , Proteínas Ativadoras de GTPase , Humanos , Imuno-Histoquímica , Leucócitos/metabolismo , Proteínas de Membrana/química , Dados de Sequência Molecular , Proteínas Quinases/metabolismo , Proteínas/química , Proteínas Proto-Oncogênicas c-crk , RNA Mensageiro/análise , RNA Mensageiro/metabolismo , Homologia de Sequência de Aminoácidos , Baço/metabolismo , Timo/metabolismo , Proteínas rac de Ligação ao GTP/metabolismo , Proteínas rac1 de Ligação ao GTP/metabolismo , Proteína RAC2 de Ligação ao GTPRESUMO
Crk belongs to the adapter proteins that participate in many signalling pathways from cell surface receptors. We have characterised the CrkII-23 mutant that inhibits the transformation of NRK cells induced by epidermal growth factor (EGF) and transforming growth factor (TGF)-beta. To study the biochemical difference, cDNAs of the wild-type CrkII and the CrkII-23 mutant were introduced stably into NIH 3T3 cells expressing EGF receptor (EGFR). Both CrkII and CrkII-23 were phosphorylated on tyrosine upon EGF simulation with similar time course and dose dependency. Whereas the wild-type CrkII bound to EGFR only after EGF stimulation, CrkII-23 bound to EGFR from before stimulation. Mutation in the Src homology (SH) 2 or amino-terminal SH3 domain did not abolish the binding of CrkII-23 to EGFR in the quiescent cells, suggesting that the binding is mediated by a novel mechanism. These CrkII-23-derived mutants, however, did not suppress transformation of NRK cells by EGF and TGF-beta. Hence, both the SH2 and amino-terminal SH3 domains are required to inhibit transformation of NRK cells. These results suggest that persistent signalling from CrkII-23 bound to EGFR suppresses transformation by EGF and TGF-beta in NRK23 cells.
Assuntos
Proteínas Adaptadoras de Transdução de Sinal , Proteínas Adaptadoras de Transporte Vesicular , Transformação Celular Neoplásica/genética , Fator de Crescimento Epidérmico/farmacologia , Receptores ErbB/metabolismo , Mutação , Proteínas Quinases/genética , Proteínas Quinases/metabolismo , Fator de Crescimento Transformador beta/farmacologia , Ubiquitina-Proteína Ligases , Domínios de Homologia de src/genética , Células 3T3 , Animais , Células COS , Diferenciação Celular/efeitos dos fármacos , Transformação Celular Neoplásica/metabolismo , Análise Mutacional de DNA , Fator de Iniciação 2 em Eucariotos , Fatores de Troca do Nucleotídeo Guanina , Humanos , Camundongos , Neurônios/citologia , Neurônios/efeitos dos fármacos , Células PC12 , Fosforilação , Ligação Proteica , Proteínas Quinases/fisiologia , Estrutura Terciária de Proteína , Proteínas/metabolismo , Proteínas Proto-Oncogênicas/metabolismo , Proteínas Proto-Oncogênicas c-abl/metabolismo , Proteínas Proto-Oncogênicas c-cbl , Proteínas Proto-Oncogênicas c-crk , Ratos , Proteínas Adaptadoras da Sinalização Shc , Proteína 1 de Transformação que Contém Domínio 2 de Homologia de Src , Tirosina/metabolismoRESUMO
RGD (arg-gly-asp)-containing proteins have been shown to be components of cartilage matrix. In the present study, the role of interactions of cells with RGD-containing cartilage matrix proteins in rat costal epiphyseal chondrocyte differentiation was examined using a pelleted culture system as an in vitro model of endochondral ossification. Cell attachment assays showed the presence of integrins which mediated the binding of chondrocytes to fibronectin, a member of RGD-containing cartilage matrix proteins, in an RGD-dependent manner. In the early culture period, when chondrocytes had nonhypertrophic morphology with low levels of alkaline phosphatase, the exogenous addition of synthetic peptide GRGDSP (gly-arg-gly-asp-ser-pro) caused an increase in alkaline phosphatase levels and enlargement of chondrocytes in pelleted cultures. Treatment with GRGDSP from the early to late culture periods in association with the transition of chondrocytes from prehypertrophic to hypertrophic phenotypes followed by matrix mineralization resulted in suppression of mineral growth without significant effects on alkaline phosphatase levels or cellular morphology in the cultures. Similarly, addition of the synthetic peptide during the late culture period with the advance of cartilage mineralization suppressed mineral growth in pelleted cultures. These data indicate an important role of interactions of chondrocytes with RGD-containing cartilage matrix proteins through integrins in the regulation of epiphyseal chondrocyte differentiation in pelleted cultures.
Assuntos
Proteínas da Matriz Extracelular/metabolismo , Lâmina de Crescimento/citologia , Oligopeptídeos/farmacologia , Receptores Imunológicos/metabolismo , Sequência de Aminoácidos , Análise de Variância , Animais , Adesão Celular , Diferenciação Celular , Células Cultivadas , Fibronectinas/metabolismo , Masculino , Oligopeptídeos/metabolismo , Ratos , Ratos Wistar , CostelasRESUMO
Metal-metal bond formation by a cobaltadithiolene complex was observed for the first time in the reaction of [Co(η(5) -C5 H5 )(S2 C6 H4 )] with [Mo(CO)3 (py)3 ] and BF3 to give the Co-Mo-Co cluster 1. Cyclic voltammetry reveals that 1 undergoes two one-electron reduction steps at the Co centers, which is indicative of transmission of the Co-Co electronic interaction through the Mo center.
RESUMO
A myocardial 201TI SPECT examination was conducted both prior to and following surgery of two adult patients with an anomalous left coronary artery originating from the pulmonary artery (Bland-White-Garland syndrome). In this syndrome, the anterior wall is usually affected first. Preoperative examination demonstrated an inferoposterior perfusion defect. In both patients, the right coronary artery was markedly dilated, and a significant left-to-right shunt formation was observed. Both patients were diagnosed as possibly having coronary steal syndrome. A prolonged inadequate blood supply to the right coronary region may cause inferoposterior cardiomyopathy. In the postoperative examination, myocardial perfusion markedly improved in one patient whose preoperative SPECT showed redistribution in a delayed scan. Thallium-201 SPECT was therefore found to be useful in assessing both the preoperative and postoperative myocardial perfusion.
Assuntos
Circulação Coronária , Anomalias dos Vasos Coronários/diagnóstico por imagem , Radioisótopos de Tálio , Tomografia Computadorizada de Emissão de Fóton Único , Adulto , Idoso , Anomalias dos Vasos Coronários/fisiopatologia , Anomalias dos Vasos Coronários/cirurgia , Feminino , Coração/diagnóstico por imagem , Humanos , Masculino , SíndromeRESUMO
A 15-month-old boy with severe aplastic anemia developed veno-occlusive disease (VOD) after allogeneic bone marrow transplantation (BMT), in which the preparative regimen included 50 mg/kg/day cyclophosphamide and anti-lymphocyte globulin for 4 consecutive days. The diagnosis was made based on clinical symptoms and data including, hepatomegaly, right upper quadrant abdominal pain, jaundice, ascites, coagulopathy and thrombocytopenia which was refractory to transfusions of platelet concentrate. We gave 2, 3, 5 and 5 mg/day/body of recombinant tissue plasminogen activator (tPA) followed by heparin and prostaglandin E1 (PGE1) effectively and without significant side effect on days 9, 10, 13 and 14, respectively. Clinical and biochemical improvement was steady and dramatic. We suggest that tPA following continuous heparin and PGE1 infusion may be useful in the treatment of VOD even in infantile cases.
Assuntos
Alprostadil/uso terapêutico , Transplante de Medula Óssea/efeitos adversos , Heparina/uso terapêutico , Hepatopatia Veno-Oclusiva/tratamento farmacológico , Ativador de Plasminogênio Tecidual/uso terapêutico , Anemia Aplástica/terapia , Purging da Medula Óssea , Quimioterapia Combinada , Hepatopatia Veno-Oclusiva/etiologia , Humanos , Lactente , Masculino , Transplante HomólogoRESUMO
A 10-year-old male with severe aplastic anemia following allogeneic BMT developed a hemolytic crisis on post-BMT day 67. The diagnosis of cold hemagglutinin disease was made based on the findings of anemia, reticulocytosis, positive direct Coombs test, and markedly elevated cold agglutinins. Anti-nuclear antibody and anti-DNA antibody were also increased. Plasmapheresis was effective as an emergency measure. Corticosteroid after plasmapheresis had a transient effect. At the second episode of hemolysis 6 months after BMT, immunosuppressive therapy with cyclophosphamide plus corticosteroid was successfully administered without negative effect on engraftment.
Assuntos
Anemia Hemolítica Autoimune/terapia , Doenças Autoimunes/terapia , Ciclofosfamida/uso terapêutico , Imunossupressores/uso terapêutico , Plasmaferese , Prednisona/uso terapêutico , Anemia Aplástica/terapia , Autoanticorpos/sangue , Criança , Feminino , Seguimentos , Humanos , Imunoglobulina M/sangue , Masculino , Recidiva , Terapia de Salvação , Transplante HomólogoRESUMO
A 4-year-old boy with Diamond-Blackfan anemia and a history of multiple transfusions underwent umbilical cord blood transplantation from his HLA-identical female sibling born by vaginal delivery at 38 weeks. The patient was prepared with busulfan, cyclophosphamide and antilymphocyte globulin. Methotrexate and cyclosporin A were given for the prophylaxis of GVHD. Regimen-related toxicity was not observed and successful engraftment occurred, including the erythroid series. No evidence of acute or chronic GVHD has been observed for 14 months after transplantation. This is the first case of successful umbilical cord blood transplantation to a patient with Diamond-Blackfan anemia.