Hisaki Naito, Hidenobu Kamohara, Toshinori Oshima, Junji Yamashita, Kentaro Tokunaga, Daisuke Niimori, Atsushi Kotera, Katsuyuki Sagishima, Hideki Nakayama and Yoshihiro Kinoshita
Osteogenesis imperfecta is main symptom in an autosomal recessive Jeune syndrome. Odontogenic infection by dentinogenesis imperfecta and poor oral hygiene is not known and Infective Endocarditis (IE) as a life threatening complication is the first report in Jeune syndrome. Case: A 13-year-old female patient presented with fever, disturbed consciousness, and convulsion. She had Jeune syndrome with mitral regurgitation and mental retardation. Cerebral hemorrhage, vegetation of mitral valve and poor oral hygiene demonstrated clinical definite diagnosis of IE by Duke Criteria. Bacterial aneurysm would cause to be cerebral hemorrhage that was no worsening by Computed Tomography. Vancomycin or Linezolid was treated after MRSA was detected in blood culture. Decayed teeth were removed as source of IE. Consciousness level and inflammation response were improved and no sign of infection was confirmed by frequent echocardiogram and blood culture. Outcome: Patient was discharged the hospital after she had no symptoms with appropriate treatment. Conclusion: Observation of oral hygiene and consideration of IE as the differential diagnosis are beneficial in a severe infectious disease of unknown origin with osteogenesis imperfecta, Jeune syndrome. Antibiotics therapy, oral hygiene and teeth extraction are effective for IE caused by decayed teeth with dentinogenesis imperfecta.
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