Artículo

Sassetti, B.; Lajmanovich, A.; Vázquez, A.; Vizcarguénaga, M.I.; Berthier, R.; Aversa, L.; Bustelo, P.; Kordich, L. "Glanzmann thrombasthenia in children from Argentina" (1996) Journal of Pediatric Hematology/Oncology. 18(1):23-28
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Abstract:

Purpose: Glanzmann thrombasthenia is a well-defined inherited disorder of platelet function characterized by a decrease or absence of functional platelet glycoprotein (GP) GPIIbIIIa. The diagnosis must be considered in patients presenting with mucocutaneous bleeding, purpura, a normal platelet count, abnormal platelet aggregation, and a prolonged bleeding time. In most of the patients, the presence of small amounts of either GPIIb or GPIIIa was detected in their platelets. These observations could provide a basis for determining the clinical and laboratory heterogeneity of the disease. Patients and Methods: We studied 10 patients of seven unrelated families with the usual methods and an immunoalkaline phosphatase technique (APAAP) to analyze the biosynthesis of GP in megakaryocytes. Results: The results allowed us to classify six patients as GT type I, three as type II, and one as a variant. Conclusion: The nature and severity of the bleeding manifestations, in our patients, were not predictible by the laboratory findings. These confirm the clinical and laboratory heterogeneity of the disease.

Registro:

Documento: Artículo
Título:Glanzmann thrombasthenia in children from Argentina
Autor:Sassetti, B.; Lajmanovich, A.; Vázquez, A.; Vizcarguénaga, M.I.; Berthier, R.; Aversa, L.; Bustelo, P.; Kordich, L.
Filiación:Depto. de Quim. Biológica, Fac. de Ciencias Exactas y Naturales, Universidad Nacional de Buenos Aires, Argentina
INSERM U-217, Grenoble, France
Unidad Hematología, Div. Hematol.-Oncología, Hosp. de Niños de Buenos Aires, Argentina
Primera Junta 3724, Dpto. 3, 1407 Buenos Aires, Argentina
Palabras clave:Bleeding disorder; GPIIbIIIa; GT; Pediatrics; fibrinogen receptor; adolescent; argentina; article; autosomal recessive disorder; bleeding; bleeding time; child; clinical article; disease classification; female; glanzmann disease; human; male; priority journal; purpura; thrombocyte aggregation; Adolescent; Argentina; Blotting, Southern; Child; Child, Preschool; Female; Humans; Immunoenzyme Techniques; Infant; Male; Megakaryocytes; Platelet Glycoprotein GPIIb-IIIa Complex; Thrombasthenia
Año:1996
Volumen:18
Número:1
Página de inicio:23
Página de fin:28
DOI: http://dx.doi.org/10.1097/00043426-199602000-00005
Título revista:Journal of Pediatric Hematology/Oncology
Título revista abreviado:J. PEDIATR. HEMATOL. ONCOL.
ISSN:10774114
CODEN:JPHOF
CAS:Platelet Glycoprotein GPIIb-IIIa Complex
Registro:https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_10774114_v18_n1_p23_Sassetti

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Citas:

---------- APA ----------
Sassetti, B., Lajmanovich, A., Vázquez, A., Vizcarguénaga, M.I., Berthier, R., Aversa, L., Bustelo, P.,..., Kordich, L. (1996) . Glanzmann thrombasthenia in children from Argentina. Journal of Pediatric Hematology/Oncology, 18(1), 23-28.
http://dx.doi.org/10.1097/00043426-199602000-00005
---------- CHICAGO ----------
Sassetti, B., Lajmanovich, A., Vázquez, A., Vizcarguénaga, M.I., Berthier, R., Aversa, L., et al. "Glanzmann thrombasthenia in children from Argentina" . Journal of Pediatric Hematology/Oncology 18, no. 1 (1996) : 23-28.
http://dx.doi.org/10.1097/00043426-199602000-00005
---------- MLA ----------
Sassetti, B., Lajmanovich, A., Vázquez, A., Vizcarguénaga, M.I., Berthier, R., Aversa, L., et al. "Glanzmann thrombasthenia in children from Argentina" . Journal of Pediatric Hematology/Oncology, vol. 18, no. 1, 1996, pp. 23-28.
http://dx.doi.org/10.1097/00043426-199602000-00005
---------- VANCOUVER ----------
Sassetti, B., Lajmanovich, A., Vázquez, A., Vizcarguénaga, M.I., Berthier, R., Aversa, L., et al. Glanzmann thrombasthenia in children from Argentina. J. PEDIATR. HEMATOL. ONCOL. 1996;18(1):23-28.
http://dx.doi.org/10.1097/00043426-199602000-00005