Hypophosphatemia rickets 환아의 증례보고 : A CASE REPORT = HYPOPHOSPHATEMIA RICKETS

국문 초록 (Abstract)

Hypophosphatemia rickets는 비타민 D의 치료량에 내성을 갖기 때문에 Vitamin D-resistant rickets(VDRR)라고도 명명되며, 이는 요세관으로부터 무기질 인산의 재흡수가 감소됨으로써 혈청 칼슘농도는 정상이나 인산농도가 낮고 alkaline phosphatase가 약간 증가되기 때문에 쉽게 진단되는 구루병의 한 형태이다.
이러한 Hypophosphatemia rickets의 임상적인 소견으로 양 다리가 휘는 것, 작은 키, 척추측만, 손목과 발목부위의 팽대가 나타나며 구강내 소견으로는 자발적인 치성농양의 높은 발생률, 맹출 지연, 근단공의 폐쇄지연, 얇고 저형성된 법랑질, 명확히 인지하기 힘든 치조백선, 확대된 치수강, 법랑상아경계까지 연장된 치수각 등이 있다.
본 증례는 유치의 상실과 그에 따른 치료를 위해 본원 소아과에서 의뢰된 비타민 D 저항성 구루병 환아의 임상소견과 그 치과적 치료에 대해 보고하는 바이다.

다국어 초록 (Multilingual Abstract)

Hypophosphatemia rickets, also known as Vitamin D-resistant rickets(VDRR) and refractory rickets, is a form of rickets which is resistant to the usual doses of vitamin D. VDRR is characterized by decreased renal tubular reabsorption of inorganic phosphate and is easily diagnosed by a normal blood calcium, hypophosphatemia, and slightly elevated alkaline phosphatase.
Clinical features of Hypophosphatemia rickets included lateral bowing deformities of the legs, short stature, scoliosis, and enlargement of wrist and ankles.
Dental finding in patient with VDRR were spontaneous dental abscesses in caries free teeth and other dental findings included delayed eruption, delayed apical closure, thin and hypoplastic enamel, absent or poorly defined lamina dura, enlarged pulp chambers, and numerous accessory canals and pulp horns that extend up and into the dentinoenamel junction.
we reported the clinical feature and treatment of VDRR child who was referred from the department of pediatrics for early loss of primary teeth and its treatment.

참고문헌 (Reference)

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