Inherited haemorrhagic disease with abnormal prothrombin consumption

The propositus is a 4-year-old boy who presented with a history of excessive bleeding after surgical procedures as well as haematomas and epistaxis. The defect in haemostasis consisted in an anomaly of the prothrombin consumption tests as the only abnormality while all the other conventional coagula...

ver descrição completa

Detalhes bibliográficos
Autores: Rocha, E. (Eduardo)|||/items/96d5cf13-d967-4252-8888-36a4956209d6, Páramo-Fernández, J.A. (José Antonio)|||/items/8c56baa6-2a43-4836-b91b-4eed15be3c96, Cuesta, B. (Braulia)|||/items/346a2bd1-5f42-4853-bf55-b53e424b53c6, Fernandez, J. (Javier)|||/items/60cfb594-0850-42a3-97ae-4b29abfd6b56
Formato: artículo
Fecha de publicación:1985
País:España
Recursos:Universidad de Navarra
Repositorio:Dadun. Depósito Académico Digital de la Universidad de Navarra
Idioma:inglés
OAI Identifier:oai:dadun.unav.edu:10171/22925
Acesso em linha:https://hdl.handle.net/10171/22925
Access Level:acceso abierto
Palavra-chave:Blood Coagulation Disorders/genetics
Prothrombin/metabolism
id ES_37d6c64742aa0f8ea5af04d51062da6c
oai_identifier_str oai:dadun.unav.edu:10171/22925
network_acronym_str ES
network_name_str España
repository_id_str
spelling Inherited haemorrhagic disease with abnormal prothrombin consumptionRocha, E. (Eduardo)|||/items/96d5cf13-d967-4252-8888-36a4956209d6Páramo-Fernández, J.A. (José Antonio)|||/items/8c56baa6-2a43-4836-b91b-4eed15be3c96Cuesta, B. (Braulia)|||/items/346a2bd1-5f42-4853-bf55-b53e424b53c6Fernandez, J. (Javier)|||/items/60cfb594-0850-42a3-97ae-4b29abfd6b56Blood Coagulation Disorders/geneticsProthrombin/metabolismThe propositus is a 4-year-old boy who presented with a history of excessive bleeding after surgical procedures as well as haematomas and epistaxis. The defect in haemostasis consisted in an anomaly of the prothrombin consumption tests as the only abnormality while all the other conventional coagulation and fibrinolysis tests as well as platelet function tests were normal. The father of the propositus had no previous history of excessive bleeding but was found to have an abnormal prothrombin consumption index. The reaction to prothrombin conversion, normal at onset, slowed down to less than normal and did not reach completion until 24 h. The in vivo studies suggest that the effect does not act on the interaction between platelet phospholipid and plasma. The factor II dosage and the electrophoretic mobility of prothrombin of the plasma were normal; nevertheless when studying the purified prothrombin by means of crossed immunoelectrofocusing there appeared an anomaly of pI. This result suggests the possible existence of an abnormal prothrombin molecule responsible for a slow prothrombin conversion.Blackwell PublishingDadun. Depósito Académico Digital Universidad de Navarra20122012-07-1919851985-01-0119851985-01-01journal articlehttp://purl.org/coar/resource_type/c_6501info:eu-repo/semantics/articleapplication/pdfhttps://hdl.handle.net/10171/22925reponame:Dadun. Depósito Académico Digital de la Universidad de Navarrainstname:Universidad de NavarraInglésengopen accesshttp://purl.org/coar/access_right/c_abf2info:eu-repo/semantics/openAccessoai:dadun.unav.edu:10171/229252026-06-21T12:47:57Z
dc.title.none.fl_str_mv Inherited haemorrhagic disease with abnormal prothrombin consumption
title Inherited haemorrhagic disease with abnormal prothrombin consumption
spellingShingle Inherited haemorrhagic disease with abnormal prothrombin consumption
Rocha, E. (Eduardo)|||/items/96d5cf13-d967-4252-8888-36a4956209d6
Blood Coagulation Disorders/genetics
Prothrombin/metabolism
title_short Inherited haemorrhagic disease with abnormal prothrombin consumption
title_full Inherited haemorrhagic disease with abnormal prothrombin consumption
title_fullStr Inherited haemorrhagic disease with abnormal prothrombin consumption
title_full_unstemmed Inherited haemorrhagic disease with abnormal prothrombin consumption
title_sort Inherited haemorrhagic disease with abnormal prothrombin consumption
dc.creator.none.fl_str_mv Rocha, E. (Eduardo)|||/items/96d5cf13-d967-4252-8888-36a4956209d6
Páramo-Fernández, J.A. (José Antonio)|||/items/8c56baa6-2a43-4836-b91b-4eed15be3c96
Cuesta, B. (Braulia)|||/items/346a2bd1-5f42-4853-bf55-b53e424b53c6
Fernandez, J. (Javier)|||/items/60cfb594-0850-42a3-97ae-4b29abfd6b56
author Rocha, E. (Eduardo)|||/items/96d5cf13-d967-4252-8888-36a4956209d6
author_facet Rocha, E. (Eduardo)|||/items/96d5cf13-d967-4252-8888-36a4956209d6
Páramo-Fernández, J.A. (José Antonio)|||/items/8c56baa6-2a43-4836-b91b-4eed15be3c96
Cuesta, B. (Braulia)|||/items/346a2bd1-5f42-4853-bf55-b53e424b53c6
Fernandez, J. (Javier)|||/items/60cfb594-0850-42a3-97ae-4b29abfd6b56
author_role author
author2 Páramo-Fernández, J.A. (José Antonio)|||/items/8c56baa6-2a43-4836-b91b-4eed15be3c96
Cuesta, B. (Braulia)|||/items/346a2bd1-5f42-4853-bf55-b53e424b53c6
Fernandez, J. (Javier)|||/items/60cfb594-0850-42a3-97ae-4b29abfd6b56
author2_role author
author
author
dc.contributor.none.fl_str_mv Dadun. Depósito Académico Digital Universidad de Navarra
dc.subject.none.fl_str_mv Blood Coagulation Disorders/genetics
Prothrombin/metabolism
topic Blood Coagulation Disorders/genetics
Prothrombin/metabolism
description The propositus is a 4-year-old boy who presented with a history of excessive bleeding after surgical procedures as well as haematomas and epistaxis. The defect in haemostasis consisted in an anomaly of the prothrombin consumption tests as the only abnormality while all the other conventional coagulation and fibrinolysis tests as well as platelet function tests were normal. The father of the propositus had no previous history of excessive bleeding but was found to have an abnormal prothrombin consumption index. The reaction to prothrombin conversion, normal at onset, slowed down to less than normal and did not reach completion until 24 h. The in vivo studies suggest that the effect does not act on the interaction between platelet phospholipid and plasma. The factor II dosage and the electrophoretic mobility of prothrombin of the plasma were normal; nevertheless when studying the purified prothrombin by means of crossed immunoelectrofocusing there appeared an anomaly of pI. This result suggests the possible existence of an abnormal prothrombin molecule responsible for a slow prothrombin conversion.
publishDate 1985
dc.date.none.fl_str_mv 1985
1985-01-01
1985
1985-01-01
2012
2012-07-19
dc.type.none.fl_str_mv journal article
http://purl.org/coar/resource_type/c_6501
dc.type.openaire.fl_str_mv info:eu-repo/semantics/article
format article
dc.identifier.none.fl_str_mv https://hdl.handle.net/10171/22925
url https://hdl.handle.net/10171/22925
dc.language.none.fl_str_mv Inglés
eng
language_invalid_str_mv Inglés
language eng
dc.rights.none.fl_str_mv open access
http://purl.org/coar/access_right/c_abf2
dc.rights.openaire.fl_str_mv info:eu-repo/semantics/openAccess
rights_invalid_str_mv open access
http://purl.org/coar/access_right/c_abf2
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv Blackwell Publishing
publisher.none.fl_str_mv Blackwell Publishing
dc.source.none.fl_str_mv reponame:Dadun. Depósito Académico Digital de la Universidad de Navarra
instname:Universidad de Navarra
instname_str Universidad de Navarra
reponame_str Dadun. Depósito Académico Digital de la Universidad de Navarra
collection Dadun. Depósito Académico Digital de la Universidad de Navarra
repository.name.fl_str_mv
repository.mail.fl_str_mv
_version_ 1869406063049048064
score 15,198674

Registos relacionados