Hypophosphatasia 환아의 치료 증례
HYPOPHOSPHATASIA : CASE REPORT
소속 상세정보
박수정/Soo-Jung Park
이제호/최형준/김기덕/최병재/Jae-Ho Lee/Hyung-Jun Choi/Kee-Deog Kim/Byung-Jai Choi
KMID : 0358919980250030555
Abstract
요약
1. Hypophosphatasia는 골조직 및 치아조직의 비정상적인 석회화를 나타내는 대사장애로,
특징적 구강내 소견 인 유치의 조기탈락 양상으로 치과에서 조기진단이 가능하다.
2. 본 증례에서 상악궁 확장을 동반한 의치 제작으로 심미적, 기능적으로 양호한 결과를 얻
었으며, 영구치열기 까지 장기적 관찰 및 치료가 필요할 것으로 사료된다.
#초록#
Hypophosphatasia is a rare metabolic disorder which manifests characteristics such as
abnormal mineralization of bone and dental tissues, diminished serum and tissue alkaline
phosphatase, and increased urinary secretion of PEA. It inherited as an autosomal
recessive or dominant trait and occurs in all races. In general, hypophosphatasia can be
classified in 4 subtypes which are the perinatal, infantile, childhood, adult type depending
upon the age at presentation and severity. In young children with Hypophosphatasia the
long bones show irregular defects, and the skull showes poor calcification. In older
children with premature closure of the skull sutures there may be multiple lucent area
called gyral or convolutional markings, described as resembling beaten copper,
presumably resulting from increased intracranial pressure. Examination of the jaws
reveals a generalized lucency of the maxilla and mandible. the cortical bone and lamina
dura are thin, and the alveolar bone may be deficient. Clinical features of
Hypophosphatasia include premature loss of deciduous teeth, especially incisors,
hypoplasia or aplasia of root cementum, enamel hypoplasia, irregular calcification of
dentin, large pulp chamber, and resorption of marginal alveolar bone and roots. Our
report involves a patient with a chief complaint of early loss of both Mx. and Mn.
deciduous incisors. After conducting a through clinical and radiographic examination this
patient was referred to pediatrics under the suspicion of hypophosphatasia, the diagnosis
proved to be correct and successful results were accomplished through a denture made
to improve esthetics and function.
키워드
alkaline phosphatase; early exfoliation; hypophosphatasia;
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