Sekerci Ahmet Ercan, Sisman Yildiray, Ertas Elif Tarim, Sahman Halil, Aydinbelge Mustafa
Department of Oral and Maxillofacial Radiology, Faculty of Dentistry, Erciyes University, Kayseri, Turkey.
J Dent Child (Chic). 2012 May-Aug;79(2):93-9.
Infantile malignant osteopetrosis (IMO) is an uncommon autosomal recessive disease characterized by dense, brittle, sclerotic, radiopaque bones, which generally results in neurological and hematological abnormalities. In general, IMO is fatal, as patients fail to grow and expire due to complications of the disease such as severe chronic anemia, bleeding, and/or infections. In children with IMO, the development of the dentition is seriously disturbed. Patients with infantile osteopetrosis have a variety of signs and symptoms, including osteomyelitis of the jaws, which frequently and generally resists treatment. Infections may eventually become lethal; hence, jaw infection control is an important aspect of the disease for dentists and oral surgeons to consider. The purpose of this case report was to present 2 rare cases of pediatric patients (one of them died due to respiratory failure at 10 years old) diagnosed with osteopetrosis complicated by osteomyelitis of the jaw.
婴儿恶性骨硬化症(IMO)是一种罕见的常染色体隐性疾病,其特征是骨骼致密、脆弱、硬化且在X射线下呈不透光性,通常会导致神经和血液学异常。一般来说,IMO是致命的,因为患者会因严重慢性贫血、出血和/或感染等疾病并发症而无法生长并最终死亡。在患有IMO的儿童中,牙齿发育受到严重干扰。婴儿骨硬化症患者有多种体征和症状,包括颌骨骨髓炎,这种病症通常频繁出现且难以治疗。感染最终可能会致命;因此,控制颌骨感染是牙医和口腔外科医生在治疗该疾病时需要考虑的一个重要方面。本病例报告的目的是呈现2例罕见的儿科患者(其中1例在10岁时因呼吸衰竭死亡),他们被诊断为患有骨硬化症并伴有颌骨骨髓炎。