Fulminant sepsis/meningitis due to Haemophilus influenzae in a protein C-deficient heterozygote treated with activated protein C therapy.
Eur J Pediatr
; 168(6): 673-7, 2009 Jun.
Article
en En
| MEDLINE
| ID: mdl-18751723
ABSTRACT
A 13-month-old Japanese female with Haemophilus influenzae type b meningitis presented with unusually severe septic shock and cerebral infarction in half a day of fever. The initial therapy of plasma-derived activated protein C (Anact C) led to an impressive effect on the aggressive condition. However, purpura fulminans and the consistent decline of plasma protein C activity (<20%) required prolonged activated protein C therapy and gene analysis. The patient carried a novel heterozygous mutation of PROC (exon 4; 335 GAC>TAC, Asp46Tyr). This is the first report of infectious purpura fulminans in a protein C-deficient heterozygote. The clinical onset and treatment course adequately corroborated the aggravated immune/hemostatic reactions and the cytoprotective effects of activated protein C replacement in human heterozygous protein C deficiency. The monitoring of plasma protein C activity and sufficient administration of activated protein C product could improve the outcome of severe sepsis in children.
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Proteína C
/
Haemophilus influenzae tipo b
/
Deficiencia de Proteína C
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Púrpura Fulminante
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Meningitis por Haemophilus
Límite:
Female
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Humans
/
Infant
Idioma:
En
Revista:
Eur J Pediatr
Año:
2009
Tipo del documento:
Article
País de afiliación:
Japón