• Title/Summary/Keyword: 저인산혈증성 구루병

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HYPOPHOSPHATEMIC RICKETS : CASE REPORT (저인산혈증성 구루병 환아의 증례 보고)

  • Park, Yoon-Hee;Choi, Byung-Jai;Lee, Jong-Gap
    • Journal of the korean academy of Pediatric Dentistry
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    • v.27 no.1
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    • pp.108-112
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    • 2000
  • Hypophosphatemic rickets is lack of reponse to physiologic doses of vitamin D, different from the vitamin D-dependent rickets. It is inherited in an X-linked dominant fashion. The prime features of this disorder are lowered serum phosphate levels, elevated serum alkaline phosphatase and normal serum calcium levels. The dental manifestation often include apical radiolucencies, abscess and fistulas of clinically sound teeth. Dental radiographs show ricketic bone trabeculations, abscent or abnormal lamina dura and abnormal cementum. This case which was diagnosed to hypophosphatemic rickets, showed multiple spontaneous periapical abscess and gingival fistula enlarged pulp chambers, extension of the pulp horns into the cusp tips and delayed eruption.

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A Case of Hypophosphatemic Rickets associated with Epidermal Nevus Syndrome (저인산혈증성 구루병을 동반한 표피모반증후군 1례)

  • Lee Yong Joo;Kang Ju Hyung;Lee Soo Jin;Park Ho Jin;Shin Choong Ho;Cheong Hae Il
    • Childhood Kidney Diseases
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    • v.9 no.2
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    • pp.263-268
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    • 2005
  • Epidermal nevus syndrome is a rare disease consisting of epidermal nevus and multisystem pathologic conditions associated with anomalies in the central nervous system, bone, eye, heart, vasculature and genito-urinary system. Hypophosphatemic rickets has been rarely observed in association with this syndrome. We report a case of hypophosphatemic rickets as sociated with epidermal nevus syndrome with review of the literature. (J Korean Soc Pediatr Nephrol 2005;9:263-268)

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X-LINKED DOMINANT HYPOPHOSPHATAEMIC RICKETS: 13 YEARS FOLLOW-UP STUDY (X 염색체 우성 저인산혈증성 구루병: 13년간의 치험례)

  • Ha, Na;Kim, Youngjin;Kim, Hyunjung;Nam, Soonhyeun
    • The Journal of Korea Assosiation for Disability and Oral Health
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    • v.13 no.1
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    • pp.14-18
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    • 2017
  • X-linked dominant hypophosphataemic rickets(XLHR) is a hereditary disease generally transmitted as an X-linked dominant trait. Oral manifestations include poorly mineralized dentin, enlarged pulp chambers and root canals, early tooth loss. Also, spontaneous dental abscesses could occur in the absence of a history of trauma or caries. This report describes the clinical finding and dental treatment of a child, who came to the hospital for treatment caries but was refered to pediatrics because the child showed clinically short of status, bow-leg and radiographically enlarged pulp chamber and canal, there as diagnosed as XLHR. After the diagnosis, 13 years follow-up and treatment were performed.

X-LINKED HYPOPHOSPHATEMIC RICKETS : CASE REPORT (성염색체 연관 저인산혈증성 구루병 환자의 증례 보고)

  • Lee, Su-Jin;Kim, Young-Jae;Jang, Ki-Taeg;Lee, Sang-Hoon;Kim, Chong-Chul;Hahn, Se-Hyun;Kim, Jung-Wook
    • Journal of the korean academy of Pediatric Dentistry
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    • v.36 no.2
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    • pp.298-304
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    • 2009
  • XLH (X-linked hypophosphatemic rickets) is a form of rickets which is resistant to the usual dose of vitamin D and inherited in a X-linked dominant manner. It is also known as vitamin D-resistant rickets or familial hypophosphatemic rickets. Here we report a 6-year-and-6-month-old female patient of XLH who is diagnosed with in SNUB. She was referred from local clinic for impaction of maxillary left permanent incisor and its treatment. She presents bowing deformities of the legs, short stature, enlargement of wrist and ankles and spontaneous dental abscesses of clinically sound teeth delayed eruption, taurodontism, delayed apical closure, enlarged pulp chambers, and absent or poorly defined lamina dura. The purpose of this case is to review the literatures of XLH and report the dental and medical characteristics of this patient.

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