TY - JOUR
T1 - Staphylococcal endocarditis as the first manifestation of heritable protein S deficiency in childhood
AU - Hoshina, Takayuki
AU - Nakashima, Yasutaka
AU - Sato, Daisuke
AU - Nanishi, Etsuro
AU - Nishio, Hisanori
AU - Nagata, Hazumu
AU - Yamamura, Kenichiro
AU - Doi, Takehiko
AU - Shiokawa, Yuichi
AU - Koga, Yui
AU - Kang, Dongchon
AU - Ohga, Shouichi
AU - Hara, Toshiro
N1 - Funding Information:
We thank Prof. Yoshinao Oda and Dr. Kenichi Kohashi and Yuichi Yamada (Department of Pathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan) and Dr. Hatsue Ueda (Department of Pathology, National Cerebral and Cardiovascular Center, Osaka, Japan) for helpful discussion. This work was supported in part by a Grant-in-Aid for Scientific Research from the Ministry of Education, Culture, Sports, Science and Technology of Japan and by a grant from the Ministry of Health, Labour and Welfare of Japan .
PY - 2014/2
Y1 - 2014/2
N2 - A 12-year-old Japanese girl developed infective endocarditis and central nervous system disease. The previously healthy girl showed altered consciousness and abnormal behaviors along with the classical signs of septic emboli. Staphylococcus aureus was isolated from peripheral blood, but not, the pleocytotic cerebrospinal fluid. Diagnostic imaging studies revealed a vegetative structure in the morphologically normal heart, and multiple thromboembolisms in the brain and spleen. Low plasma activity of protein S (12%) and thrombophilic family history allowed the genetic study, demonstrating that she carried a heterozygous mutation of PROS1 (exon 13; 1689C > T, p.R474C). Surgical intervention of the thrombotic fibrous organization and subsequent anticoagulant therapy successfully managed the disease. There are no reports of infective endocarditis in childhood occurring as the first presentation of heritable thrombophilia. Protein S deficiency might be a risk factor for the development or exacerbation of infective endocarditis in children having no pre-existing heart disease.
AB - A 12-year-old Japanese girl developed infective endocarditis and central nervous system disease. The previously healthy girl showed altered consciousness and abnormal behaviors along with the classical signs of septic emboli. Staphylococcus aureus was isolated from peripheral blood, but not, the pleocytotic cerebrospinal fluid. Diagnostic imaging studies revealed a vegetative structure in the morphologically normal heart, and multiple thromboembolisms in the brain and spleen. Low plasma activity of protein S (12%) and thrombophilic family history allowed the genetic study, demonstrating that she carried a heterozygous mutation of PROS1 (exon 13; 1689C > T, p.R474C). Surgical intervention of the thrombotic fibrous organization and subsequent anticoagulant therapy successfully managed the disease. There are no reports of infective endocarditis in childhood occurring as the first presentation of heritable thrombophilia. Protein S deficiency might be a risk factor for the development or exacerbation of infective endocarditis in children having no pre-existing heart disease.
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U2 - 10.1016/j.jiac.2013.08.002
DO - 10.1016/j.jiac.2013.08.002
M3 - Article
C2 - 24462440
AN - SCOPUS:84903558337
SN - 1341-321X
VL - 20
SP - 128
EP - 130
JO - Journal of Infection and Chemotherapy
JF - Journal of Infection and Chemotherapy
IS - 2
ER -
