Homozygous protein C deficiency presenting as neonatal purpura fulminans: management with fresh frozen plasma, low molecular weight heparin and protein C concentrate.
J Thromb Thrombolysis
; 45(2): 315-318, 2018 Feb.
Article
em En
| MEDLINE
| ID: mdl-29270858
ABSTRACT
Purpura fulminans in neonates is a rapidly progressive thrombotic disorder manifesting as hemorrhagic skin infarction and disseminated intravascular coagulation. Being inherited in an autosomal dominant manner, it is a medical emergency. Clinical presentations of patients may vary depending on the genetic mutations. Retinal and intracranial hemorrhages are the worst clinical scenarios with persistent morbidity. During acute phase, fresh frozen plasma, protein C concentrates and anticoagulant therapy should be administered rapidly. Here we report a patient with homozygous protein C deficiency.
Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Deficiência de Proteína C
Tipo de estudo:
Diagnostic_studies
Limite:
Humans
/
Newborn
Idioma:
En
Ano de publicação:
2018
Tipo de documento:
Article