RESUMEN
Serum level of alpha-1-antitrypsin is significantly reduced in newborns with idiopathic respiratory distress when compared to matched controls. This decrease was predominantly due to the group of infants who died from I.R.D., provided that alpha-1-antitrypsin determination was carried out on the first day of life. Alpha-1-antitrypsin was demonstrable by indirect immunofluorescence in hyaline membranes whereas fluorescence was negative in the alveolae of infants who had no hyaline membranes.
Asunto(s)
Pulmón/análisis , Síndrome de Dificultad Respiratoria del Recién Nacido/metabolismo , Deficiencia de alfa 1-Antitripsina , Edad Gestacional , Humanos , Recién Nacido , Síndrome de Dificultad Respiratoria del Recién Nacido/sangreRESUMEN
Different HT have been studied with the electron microscope and the findings compared to those in the literature. Five patients with thrombasthenia and three with Willebrand's diseases showed various disorders, usually anisocytosis and hypertrophic open canalicular system, in relation to regenerative states. Thirteen young children with 21-trisomy (2 with leukemia) and 13/15 trisomy (1 case) had enlarged platelets with abnormalities of membranes and vacuoles indicating some metabolic disorders and necrotic lesions. One child with Cooley's anemia showed a few granules and necrotic lesions. All these symptoms were related to regenerative or primitive megakaryocytopathias. The lesion of these latter cell appears in other HT: aberrations of membranes in giant platelets syndroms (J. Bernard and J. P. Soulier syndrome), disorders in membranes and/or granules in thrombopenic thrombopathias, the short platelet life span in May-Hegglin anomaly, storage pool disease in Hermansky-Pudlak syndrome. Finally the ultrastructural abnormalities of the platelets mainly help to distinguish several diseases of megakaryocytes.
Asunto(s)
Plaquetas/ultraestructura , Megacariocitos/ultraestructura , Púrpura Trombocitopénica/genética , Trastornos de las Plaquetas Sanguíneas/sangre , Adhesión Celular , Membrana Celular/ultraestructura , Niño , Cromosomas Humanos 13-15 , Gránulos Citoplasmáticos , Síndrome de Down/sangre , Glucógeno/metabolismo , Trastornos Hemorrágicos/genética , Humanos , Leucemia/sangre , Metabolismo de los Lípidos , Microscopía Electrónica , Talasemia/sangre , Vacuolas , Enfermedades de von Willebrand/sangreRESUMEN
Blood platelets from infant and new born mongolians were examined and compared taking into consideration existing or non existing haematologic anomalies, such as transitory leucoblastosis with or without hyperthrombocytosis and acute leucosis without thrombopenia. These abnormalities were correlated with the analysis of control samples from haematologically normal new-born and premature children as well as samples from adult mongolian, patients with other chromosomal alterations (13-15, phi) and new-born or adults with thrombocytosis. The presence of dysmorphic platelets could be observed in variable degrees and numbers in all the mongolian samples. The characters of the morphologic abnormalities, constantly observed with variations due to pathological causes, denote either metabolic or structural thrombopathy.