Novel Insights from Clinical Practice

Portal Vein Thrombosis and High Factor VIII in Turner Syndrome
Cristiane Kopacek Zilz^a, Juliana Keller Brenner^b, Regina Helena Elnecave<sup>a

a</sup>Serviço de Endocrinologia, Hospital de Clínicas de Porto Alegre, and
^bUniversidade Federal do Rio Grande do Sul, Faculdade de Medicina, Porto Alegre, Brazil

Address of Corresponding Author

Horm Res 2006;66:89-93 (DOI: 10.1159/000093693)

  Key Words

• Turner syndrome
• Portal vein thrombosis
• Factor VIII
• Von Willebrand factor
• Thrombosis

  Abstract

Backgrounds/Aims: Turner syndrome is not usually associated with thrombotic events. The aim of this study is to report 3 Turner syndrome patients with portal vein thrombosis and, in 2 of them, high factor VIII. These findings are compared to values in Turner syndrome patients without thrombosis and controls. Methods: In different years, 3 patients with Turner syndrome were initially seen at the Gastroenterology Clinic of Hospital de Clínicas de Porto Alegre, Brazil, for portal vein thrombosis. After the most common causes of portal vein thrombosis and thrombophilias had been excluded, the 2 surviving patients were studied for clotting factors VIII, IX and von Willebrand factor. The same factors were also assessed in 25 Turner syndrome patients without thrombosis and 25 normal girls. Results: One of the patients with portal vein thrombosis died before the study. In the 2 surviving patients, factors VIII and von Willebrand levels were >150 IU/dl, which is considered to be high. In Turner syndrome patients without thrombosis, the mean factor VIII level was 127.2 ± 41.1 IU/dl and for von Willebrand factor 101.2 ± 26.9 IU/dl, while in control girls these were 116.0 ± 27.6 and 94.28 ± 27.5 IU/dl, respectively. Factor VIII and von Willebrand factor were not different between these 2 groups. When non-O blood group Turner syndrome patients and normal girls were compared, the former had significantly higher levels of factor VIII. Conclusions: This is the first report on the unusual finding of portal thrombosis in patients with Turner syndrome in whom high levels of factor VIII and von Willebrand factor were found. Factor VIII is higher in the non-O blood group Turner syndrome patients without thrombosis when compared to normal girls.

Copyright © 2006 S. Karger AG, Basel

  Author Contacts

Regina Helena Elnecave, MD, PhD
Serviço de Endocrinologia, Hospital de Clínicas de Porto Alegre
Rua Ramiro Barcelos 2350 - Prédio 12, 4° andar
CEP: 90035-003, Porto Alegre, RS (Brazil)
Tel. +55 51 2101 8127, Fax +55 51 2101 8777, E-Mail rhe@portoweb.com.br

  Article Information

Received: October 19, 2005
Accepted: April 19, 2006
Published online: May 31, 2006
Number of Print Pages : 5
Number of Figures : 2, Number of Tables : 2, Number of References : 36