Staphylococcal endocarditis as the first manifestation of heritable protein S deficiency in childhood

Takayuki Hoshina, Yasutaka Nakashima, Daisuke Sato, Etsuro Nanishi, Hisanori Nishio, Hazumu Nagata, Kenichiro Yamamura, Takehiko Doi, Yuichi Shiokawa, Yui Koga, Dongchon Kang, Shouichi Ohga, Toshiro Hara

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1 Citation (Scopus)


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.

Original languageEnglish
Pages (from-to)128-130
Number of pages3
JournalJournal of Infection and Chemotherapy
Issue number2
Publication statusPublished - Feb 2014

All Science Journal Classification (ASJC) codes

  • Microbiology (medical)
  • Infectious Diseases
  • Pharmacology (medical)


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