RESUMO
A chronic myelogenous leukemia patient with a masked Ph chromosome due to a new type of translocation, t(9;11;22)(q34;p11;q11), is reported.
Assuntos
Leucemia Mieloide/genética , Cromossomo Filadélfia , Translocação Genética , Adolescente , Medula Óssea/patologia , Bandeamento Cromossômico , Cromossomos Humanos Par 11 , Cromossomos Humanos Par 22 , Cromossomos Humanos Par 9 , Humanos , Cariotipagem , Leucócitos/citologia , MasculinoAssuntos
5-Hidroxitriptofano/uso terapêutico , Biopterinas/análogos & derivados , Distonia/genética , Neurônios/metabolismo , Serotonina/metabolismo , Adulto , Biopterinas/uso terapêutico , Criança , Dopamina/metabolismo , Distonia/tratamento farmacológico , Feminino , Humanos , Levodopa/uso terapêuticoRESUMO
We investigated the effects of long-term oral and intramuscular vitamin E repletion in children with chronic cholestasis. Clinical improvement or suppression of neuromuscular involvement after adequate vitamin E repletion was demonstrated. Light and electron microscopic abnormalities of the skeletal muscle, however, did not completely disappeared despite the correction of the biochemical abnormalities for more than 3 years. The muscle fibers showed less variety of pathologic features than before vitamin E repletion. Inclusions observed in the skeletal muscle fibers before vitamin E treatment were still observed in subsarcolemmal cytoplasm and the perinuclear regions. They were more irregularly curved and consisted of various substances. Similar inclusions were also observed in Schwann cells, perineural cells, fibroblasts, pericytes, endothelial cells and smooth muscle cells of intramuscular vessels. Although the external lamina was not disrupted, separation of the external lamina from the plasma membrane and multilayered external lamina were often observed. The nerves among muscle fibers still showed degenerative features. Morphological changes of the skeletal muscle during vitamin E therapy have not so far been reported in cases of chronic cholestasis. We discuss the relationship of these findings to vitamin E replacement in children with chronic cholestasis.
Assuntos
Colestase Intra-Hepática/complicações , Músculos/ultraestrutura , Deficiência de Vitamina E/tratamento farmacológico , Vitamina E/uso terapêutico , Biópsia , Pré-Escolar , Doença Crônica , Feminino , Humanos , Lactente , Masculino , Músculos/anormalidades , Fatores de Tempo , Deficiência de Vitamina E/etiologiaAssuntos
Encéfalo/metabolismo , Glucose/metabolismo , Leucemia Linfoide/tratamento farmacológico , Metotrexato/efeitos adversos , Adolescente , Encéfalo/diagnóstico por imagem , Encéfalo/efeitos dos fármacos , Criança , Feminino , Humanos , Leucemia Linfoide/diagnóstico por imagem , Masculino , Metotrexato/uso terapêutico , Tomografia Computadorizada de EmissãoAssuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Leucemia/tratamento farmacológico , Doença Aguda , Criança , Pré-Escolar , Ensaios Clínicos como Assunto , Doxorrubicina/administração & dosagem , Humanos , Lactente , Leucemia Linfoide/tratamento farmacológico , Metotrexato/administração & dosagem , Vincristina/administração & dosagemAssuntos
Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Leucemia Linfoide/tratamento farmacológico , Adolescente , Criança , Pré-Escolar , Ensaios Clínicos como Assunto , Terapia Combinada , Feminino , Humanos , Lactente , Leucemia Linfoide/radioterapia , Masculino , Metotrexato/administração & dosagem , Vincristina/administração & dosagemRESUMO
The presence of neutrophils and their precursors with peroxidase-positive and sudanophilic giant granules, which are characteristic of Chédiak-Higashi syndrome (CHS), in the fetal liver and blood of beige (CH), but not of the control C 57 Black, mice was observed. This finding supports the view that the prenatal diagnosis could be possible, if the fetal blood specimen is obtainable safely, assuming the occurrence of similar marker cells in fetal hematopoiesis in CHS of human.
Assuntos
Síndrome de Chediak-Higashi/embriologia , Grânulos Citoplasmáticos/patologia , Sangue Fetal/citologia , Fígado/citologia , Neutrófilos/ultraestrutura , Animais , Compostos Azo , Síndrome de Chediak-Higashi/patologia , Hematopoese , Fígado/embriologia , Camundongos , Camundongos Endogâmicos C57BL , Naftalenos , Peroxidases , Coloração e RotulagemRESUMO
Flavine adenine dinucleotide (FAD) may inhibit not only the aggregation but also ATP release of platelets in vitro. FAD did not inhibit the synthesis of thromboxane from 1-14C-arachidonic acid by platelets. FAD was found to be hydrolyzed to FMN and AMP by a plasma enzyme, the activity of which in normal adults being estimated as 32.6 +/- 9.1 m microM/ml plasma/min and AMP, thus produced, and/or FAD might compete for the receptor sites on the platelet surface. This could be the mechanism of the inhibitory effect of FAD upon the platelet functions. The intravenous drip infusion of FAD in a dose of 0.2 mg/kg within 30 min was followed by no marked decrease of platelet aggregation, probably because the plasma concentration of FAD and/or AMP did not reach the levels high enough to suppress the platelet functions.
Assuntos
Plaquetas/fisiologia , Flavina-Adenina Dinucleotídeo/fisiologia , Trifosfato de Adenosina/sangue , Adulto , Ácido Araquidônico , Ácidos Araquidônicos/sangue , Plaquetas/enzimologia , Flavina-Adenina Dinucleotídeo/metabolismo , Humanos , Hidrólise , Agregação PlaquetáriaAssuntos
Leucemia Linfoide/sangue , Monócitos/imunologia , Fagocitose , Adolescente , Atividade Bactericida do Sangue , Criança , Pré-Escolar , Feminino , Humanos , MasculinoAssuntos
Leucemia Linfoide/sangue , Leucemia Mieloide/sangue , Neutrófilos/fisiologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , MasculinoRESUMO
Both riboflavin-2',3',4',5'-tetrabutyrate and flavin adenine dinucleotide (FAD), especially the latter, could inhibit H2O2-induced platelet aggregation. FAD enhanced the glutathione reductase activity of platelets. FAD might exert its inhibitory effect on the H2O2-induced platelet aggregation via the glutathione reductase and peroxidase system.
Assuntos
Flavina-Adenina Dinucleotídeo/farmacologia , Peróxido de Hidrogênio/antagonistas & inibidores , Agregação Plaquetária/efeitos dos fármacos , Riboflavina/análogos & derivados , Adolescente , Plaquetas/enzimologia , Butiratos/farmacologia , Glutationa Peroxidase/metabolismo , Glutationa Redutase/metabolismo , Humanos , Técnicas In Vitro , Riboflavina/farmacologiaRESUMO
Hydrogen peroxide can either induce or inhibit or enhance the platelet aggregation in vitro depending upon the experimental conditions. Vitamin E and vitamin E-nicotinate are found to be effective to inhibit, to some extent, the platelet aggregation induced by combined adenosine diphosphate (ADP) and hydrogen peroxide (H2O2), added simultaneously, to the platelet rich plasma (PRP). Vitamin E and vitamin E-nicotinate seemed, however, to be unable to prevent the reduction of platelet response to ADP, which was brought about by the pretreatment of PRP with H2O2 of a lower concentration.
Assuntos
Difosfato de Adenosina/antagonistas & inibidores , Peróxido de Hidrogênio/antagonistas & inibidores , Agregação Plaquetária/efeitos dos fármacos , Vitamina E/farmacologia , Adolescente , Humanos , Técnicas In Vitro , Ácidos Nicotínicos/farmacologiaRESUMO
The inhibitory effect of vitamin E-nicotinate upon the hydrogen peroxide (H2O2)-induced platelet aggregation was found to be greater than that of either vitamin E alone or the combination of vitamin E and nicotinic acid. Nicotinic acid showed no inhibitory effect. It was suggested that the effect of vitamin E-nicotinate was not due to the additive effect of vitamin E and nicotinic acid produced by hydrolysis, but to the unique and distinctive property of vitamin E-nicotinate itself.