論文の公開元へCase of osteopetrosis with multiple impacted primary and permanent teeth diagnosed at eight years old
概要
Osteopetrosis, an inherited skeletal disorder initially reported by a German radiologist in
1904 [1], is characterized by increased bone density due to a defect in remodeling caused
by malfunction of osteoclasts [2]. Based on its inheritance form and clinical severity,
osteopetrosis is classified into three distinct types; (1) severe/malignant infantile type
with autosomal recessive inheritance, (2) intermediate type with dominant or autosomal
recessive inheritance, and (3) mild/late onset/adult type with autosomal dominant
inheritance [3, 4]. As for the main systemic manifestations, bone osteosclerosis develops,
resulting in a short stature and likelihood of bone fractures [5]. Osteosclerosis in the skull
base causes narrowing of the neural foramen, which leads to cranial nerve damage, as
well as vision and hearing loss [3]. Furthermore, deficiency in all types of blood cells due
to reduced bone marrow space has been reported to lead to anemia, hemorrhage, and
immunodeficiency [6, 7].
Dental manifestations in patients with osteopetrosis include missing teeth, delayed
tooth eruption, ankylosed teeth, impacted teeth, crown and root abnormalities, enamel
hypoplasia, periodontal membrane defects, narrowing of dental pulp chambers, and
mandibular prognathism [8-10]. In addition, disruption of the calcium-phosphorus ratio,
enamel defects, and crown abnormalities have been reported to increase retention of
dental plaque, making the patient more susceptible to dental caries [11]. In addition, a
jawbone affected by osteosclerosis is highly susceptible to fracture [12]. Due to
compromised vascular supply, osteomyelitis can occur as a complication of tooth
extraction, dental caries, and periodontitis [13]. Jawbone fractures and osteomyelitis are
generally treated with symptomatic therapy, thus it is very important for osteopetrosis
patients to maintain good oral hygiene for preventing these complications [13, 14]. ...
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