Dental Alterations Associated with X-Linked Hypophosphatemic Rickets

Pereira, Cláudio Maranhão; Andrade, Cléverton Roberto de; Vargas, Pablo Agustı́n; Coletta, Ricardo D.; Almeida, Oslei Paes de; Lopes, Márcio Ajudarte

doi:10.1097/00004770-200404000-00015

Cited by 54 publications

(56 citation statements)

References 8 publications

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“…Some authors advocate extracti on of teeth that present periradicular abscesses and eventual restorati on with CONCLUSION REFERENCES implants; however, endodonti c and restorati ve treatment may not be able to maintain asepsis. The incompletely mineralized denti n exists in the form of calcospherites, which trap microorganisms and also impede mechanical endodonti c cleaning 47,48 . The practi ti oner must conclude that the occurrence of spontaneous abscesses following a shallow cavity preparati on necessitates aggressive preventi ve dental procedures.…”

Section: Prognosis and Therapymentioning

confidence: 99%

Types of Rickets, Dental and Histologic Findings: Review of the Literature

Tümen¹

2009

Pesq Bras Odontoped Clin Integr

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Introducti on: A disease that occurs during childhood, rickets is the failure of growing bone to mineralize. Many skeletal and radiographic changes can ocur because of the lack of calcifi ed osteoid and the buildup of unossifi ed carti lage. Proper bone formati on requires a complex interplay of several organs and chemicals, and vitamin D deserves special menti on because any disturbance in its producti on, absorpti on, or metabolism is paramount in the development of rickets. The pathophysiology of the disease is thought to be impaired phosphate transport, especially decreased phosphate resorpti on in the renal proximal tubule, as well as in the intesti ne. In most cases, the diagnosis is established with a thorough history and physical examinati on and confi rmed by laboratory evaluati on. Early diagnosis is essenti al because morbidity can be minimized if children are treated before eight months of age. Objecti ve: The aim of this literature review is to present various types of rickets with clinical features and the dental fi ndings, preventi ve measurements and treatments. Conclusion: The denti st as well as the pediatrician should be made aware of the features of this disorder so that early interventi on can prevent subsequent serious and more invasive dental procedures. KEYWORDS DESCRITORES Rickets;Hypophosphataemia; Oral manifestati ons; Therapeuti cs. RESUMO ABSTRACTIntrodução: O raquiti smo, uma doença que ocorre durante a infância, é a falta de crescimento ósseo necessário para a mineralização. Diversas alterações radiográfi cas esqueléti cas podem ocorrer devido a ausência de osteóide calcifi cado e a formação de carti lagem. A formação óssea adequada requer uma complexa interação de vários órgãos e produtos químicos, e a vitamina D merece uma menção especial, pois qualquer alteração na sua produção, absorção ou no seu metabolismo é fundamental para o desenvolvimento do raquiti smo. Acredita-se que a fi siopatologia da doença está no transporte inadequado do fosfato, especialmente na diminuição da reabsorção de fosfato no túbulo renal proximal bem como no intesti no. Na maioria dos casos, o diagnósti co é estabelecido com uma história completa e exame fí sico, e confi rmado por exames laboratoriais. O diagnósti co precoce é essencial, pois a morbidade pode ser minimizada se as crianças forem tratadas antes dos oito meses de idade. Objeti vo: Apresentar os vários ti pos de raquiti smo com suas característi cas clínicas, aspectos dentários, medidas preventi vas e tratamento. Conclusão: O cirurgião-denti sta, bem como o pediatra, devem ser informados das característi cas desta desordem para que a intervenção precoce possa evitar seqüelas subsequentes e procedimentos odontológicos mais invasivos.

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Section: Prognosis and Therapymentioning

confidence: 99%

Types of Rickets, Dental and Histologic Findings: Review of the Literature

Tümen¹

2009

Pesq Bras Odontoped Clin Integr

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“…Differently from vitamin D dependent rickets or from other causes of hypophosphatemic rickets (ADHR, TIO and HHRH), in XHR muscle weakness and hypotonia are absent (50). XHR is also characterized by dental damages due to dentin under-mineralization and tendency to teeth fall and dental abscesses (51). These dental alterations are more severe in adolescents and in male adults (48).…”

Section: X-linked Hypophosphatemic Ricketsmentioning

confidence: 99%

“…Early treatment of XHR can minimize growth impairment, limb deformities, and teeth anomalies (49,51,60). However, the growth deficit accumulated in the first years of life is usually permanent, especially when treatment has begun at a later age.…”

Section: Treatmentmentioning

confidence: 99%

Hypophosphatemic rickets and osteomalacia

Filho

Castro²,

Damiani

2006

Arq Bras Endocrinol Metab

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The hypophosphatemic conditions that interfere in bone mineralization comprise many hereditary or acquired diseases, all of them sharing the same pathophysiologic mechanism: reduction in the phosphate reabsorption by the renal tubuli. This process leads to chronic hyperphosphaturia and hypophosphatemia, associated with inappropriately normal or low levels of calcitriol, causing osteomalacia or rickets in children and osteomalacia in adults. X-linked hypophosphatemic rickets, autosomaldominant hypophosphatemic rickets, and tumor-induced osteomalacia are the main syndromes involved in the hypophosphatemic rickets. Although these conditions exhibit different etiologies, there is a common link among them: increased activity of a phosphaturic factor, being the fibroblast growth factor 23 (FGF-23) the most studied one and to which is attributed a central role in the pathophysiology of the hyperphosphaturic disturbances. Activating mutations of FGF-23 and inactivating mutations in the PHEX gene (a gene on the X chromosome that codes for a Zn-metaloendopeptidase proteolytic enzyme which regulates the phosphate) involved in the regulation of FGF-23 have been identified and have been implicated in the pathogenesis of these disturbances. Genetic studies tend to show that the phosphorus homeostasis depends on a complex osteo-renal metabolic axis, whose mechanisms of interaction have been poorly understood so far. This paper reviews the current knowledge status concerning the pathophysiology of phosphate metabolism regulation and the pathophysiologic basis of hypophosphatemic rickets. It also analyzes the clinical picture and the therapeutic aspects of these conditions as well. RESUMORaquitismo Hipofosfatêmico e Osteomalácia. Os distúrbios hipofosfatêmicos que comprometem a mineralização óssea englobam várias doenças, hereditárias e adquiridas, as quais compartilham um mesmo mecanismo fisiopatológico: a diminuição da reabsorção de fosfato nos túbulos renais. Este processo promove hiperfosfatúria e hipofosfatemia crônicas, associadas a níveis inapropriadamente normais ou baixos de 1,25 (OH) 2 D 3 , com conseqüente desordem do metabolismo ósteo-mineral, resultando em raquitismo e osteomalá-cia na faixa etária pediátrica e em osteomalácia nos adultos. O raquitismo hipofosfatêmico ligado ao X, o raquitismo hipofosfatêmico autossômico dominante e a osteomalácia induzida por tumor são as principais síndromes que constituem os raquitismos hipofosfatêmicos. Apesar de estas doenças apresentarem etiopatogenias distintas, as evidências bioquímico-moleculares indicam uma base fisiopatológica em comum: maior atividade de um agente fosfatúrico, sendo o fator de crescimento do fibroblasto 23 (FGF-23) o mais estudado e ao qual é atribuído um papel central na fisiopatologia destes distúrbios. Várias mutações ativadoras do gene do FGF-23 e mutações inativadoras do

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“…Low levels of vitamin D can trigger the body to release hormones that lead to the eventual loss of calcium and phosphate from bones, which leads to insufficient bone mineralization. Vitamin D Resistant Rickets (VDRR) is also known as X-linked hypophosphatemia with a prevalence of 1:20.000 people (1)(2)(3)(4). It is characterized by a calcium and phosphate metabolism disorder affecting mineralized tissues, bone and teeth (1,5).…”

Section: Introductionmentioning

confidence: 99%

“…Vitamin D Resistant Rickets (VDRR) is also known as X-linked hypophosphatemia with a prevalence of 1:20.000 people (1)(2)(3)(4). It is characterized by a calcium and phosphate metabolism disorder affecting mineralized tissues, bone and teeth (1,5). VDRR was first documented by Albright et al (6) (1937) and mineralization defects of hard tissues in these cases are derived from renal transepithelial transport disturbance resulting in decreased tubular reabsorption of phosphate and hypophosphatemia (1,4,7).…”

Section: Introductionmentioning

confidence: 99%