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Dento-maxillo-facial phenotype and implants-based oral rehabilitation in Ectodermal Dysplasia with WNT10A gene mutation: report of a case and literature review.

[oligodontia]

To report the dento-craniofacial phenotype of a family affected by a WNT10A HED and to describe the implant-based oral rehabilitation of a patient presenting a severe oligodontia linked to this mutation. A molecular hypothesis concerning the involvement of Wnt-β-catenin pathway in implant osteointegration will be proposed.Patients affected by a WNT10A mutation were included from a large group of HED patients. WNT10A gene was sequenced in second intention for patients negative for EDA-EDAR-EDARADD mutations. Dento-craniofacial phenotype was described based on clinical and radiological data.Severe oligodontia was observed in the patient affected by a compound heterozygous mutation of WNT10A gene. CT exams showed marked maxillary bone hypoplasia in the posterior areas with a sub-normal mandible treatment consisted in the placement of 4 mandibular implants and in 2 implant-supported bridges. In the maxilla, an autogenous bone graft was indicated. The post-operative radiological follow-up showed partial bone resorption of the grafts, treated with ramus bone shaving and a membrane, followed by the placement of 4 maxillary implants.Patients affected by WNT10A HED require multi-disciplinary dental diagnosis and treatment. A close post-operative radiological follow-up appears necessary given the biological functions of Wnt-β-catenin in bone repair.

Diseases presenting "treatment" symptom

  • 22q11.2 deletion syndrome
  • achondroplasia
  • acute rheumatic fever
  • aniridia
  • congenital toxoplasmosis
  • dentinogenesis imperfecta
  • erdheim-chester disease
  • esophageal carcinoma
  • homocystinuria without methylmalonic aciduria
  • lymphangioleiomyomatosis
  • megacystis-microcolon-intestinal hypoperistalsis syndrome
  • neuralgic amyotrophy
  • oligodontia
  • pleomorphic liposarcoma
  • primary hyperoxaluria type 1
  • pyomyositis
  • scrub typhus
  • severe combined immunodeficiency
  • von hippel-lindau disease
  • wiskott-aldrich syndrome

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