Hematological findings in Noonan syndrome
Rev. Hosp. Clin. Fac. Med. Univ. Säo Paulo
;
58(1): 5-8, Jan.-Feb. 2003. tab
Artigo
em Inglês
| LILACS
| ID: lil-335223
ABSTRACT
OBJECTIVE:
Noonan syndrome is a multiple congenital anomaly syndrome, and bleeding diathesis is considered part of the clinical findings. The purpose of this study was to determine the frequency of hemostatic abnormalities in a group of Noonan syndrome patients.METHOD:
We studied 30 patients with clinical diagnosis of Noonan syndrome regarding their hemostatic status consisting of bleeding time, prothrombin time, activated partial thromboplastin time and thrombin time tests, a platelet count, and a quantitative determination of factor XI.RESULTS:
An abnormal laboratory result was observed in 9 patients (30 percent). Although coagulation-factor deficiencies, especially factor XI deficiency, were the most common hematological findings, we also observed abnormalities of platelet count and function in our screening.CONCLUSIONS:
Hemostatic abnormalities are found with some frequency in Noonan syndrome patients (30 percent in our sample). Therefore, we emphasize the importance of a more extensive hematological investigation in these patients, especially prior to an invasive procedure, which is required with some frequency in this disorder
Texto completo:
DisponíveL
Índice:
LILACS (Américas)
Assunto principal:
Transtornos da Coagulação Sanguínea
/
Síndrome de Noonan
Tipo de estudo:
Estudo diagnóstico
Limite:
Adolescente
/
Adulto
/
Criança
/
Criança, pré-escolar
/
Feminino
/
Humanos
/
Lactente
/
Masculino
Idioma:
Inglês
Revista:
Rev. Hosp. Clin. Fac. Med. Univ. Säo Paulo
Assunto da revista:
Medicina
Ano de publicação:
2003
Tipo de documento:
Artigo
País de afiliação:
Brasil
Instituição/País de afiliação:
University of São Paulo/BR
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