TY - JOUR
T1 - Fulminant sepsis/meningitis due to haemophilus influenzae in a protein C-deficient heterozygote treated with activated protein C therapy
AU - Ishimura, Masataka
AU - Saito, Mitsumasa
AU - Ohga, Shouichi
AU - Hoshina, Takayuki
AU - Baba, Haruhisa
AU - Urata, Michiyo
AU - Kira, Ryutaro
AU - Takada, Hidetoshi
AU - Kusuhara, Koichi
AU - Kang, Dongchon
AU - Hara, Toshiro
N1 - Funding Information:
Acknowledgments This work was supported by a Grant-in-Aid for Scientific Research from the Ministry of Education, Science, Sports and Culture of Japan, and by a research grant from the Japan Society for the Promotion of Science (JSPS).
PY - 2009/6
Y1 - 2009/6
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=67349221334&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=67349221334&partnerID=8YFLogxK
U2 - 10.1007/s00431-008-0816-9
DO - 10.1007/s00431-008-0816-9
M3 - Article
C2 - 18751723
AN - SCOPUS:67349221334
SN - 0340-6199
VL - 168
SP - 673
EP - 677
JO - Acta Paediatrica Hungarica
JF - Acta Paediatrica Hungarica
IS - 6
ER -