Fulminant sepsis/meningitis due to haemophilus influenzae in a protein C-deficient heterozygote treated with activated protein C therapy

masataka ishimura, Mitsumasa Saito, Shoichi Ohga, Takayuki Hoshina, Haruhisa Baba, Michiyo Urata, Ryutaro Kira, Hidetoshi Takada, Koichi Kusuhara, Dongchon Kang, Toshiro Hara

Research output: Contribution to journalArticle

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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.

Original languageEnglish
Pages (from-to)673-677
Number of pages5
JournalEuropean Journal of Pediatrics
Volume168
Issue number6
DOIs
Publication statusPublished - Jun 1 2009

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Haemophilus influenzae
Heterozygote
Protein C
Meningitis
Sepsis
Purpura Fulminans
Blood Proteins
Therapeutics
Protein C Deficiency
Haemophilus influenzae type b
Cerebral Infarction
Hemostatics
Septic Shock
Fever

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health

Cite this

Fulminant sepsis/meningitis due to haemophilus influenzae in a protein C-deficient heterozygote treated with activated protein C therapy. / ishimura, masataka; Saito, Mitsumasa; Ohga, Shoichi; Hoshina, Takayuki; Baba, Haruhisa; Urata, Michiyo; Kira, Ryutaro; Takada, Hidetoshi; Kusuhara, Koichi; Kang, Dongchon; Hara, Toshiro.

In: European Journal of Pediatrics, Vol. 168, No. 6, 01.06.2009, p. 673-677.

Research output: Contribution to journalArticle

ishimura, masataka ; Saito, Mitsumasa ; Ohga, Shoichi ; Hoshina, Takayuki ; Baba, Haruhisa ; Urata, Michiyo ; Kira, Ryutaro ; Takada, Hidetoshi ; Kusuhara, Koichi ; Kang, Dongchon ; Hara, Toshiro. / Fulminant sepsis/meningitis due to haemophilus influenzae in a protein C-deficient heterozygote treated with activated protein C therapy. In: European Journal of Pediatrics. 2009 ; Vol. 168, No. 6. pp. 673-677.
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