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Radiographic study of patients with ectodermal dysplasia and partial
(2012-11-01)
Aim: To investigate the association between gender and type of cleft with hypodontia and to verify if the presence of the cleft interferes with hypodontia of one or more type of teeth in patients with ectodermal dysplasia ...
AEC syndrome and CHAND syndrome: Further evidence of clinical overlapping in the ectodermal dysplasias
(Blackwell Science Inc, 2000-05-01)
Among the ectodermal dysplasias, there are several examples of overlapping phenotypes in disorders that are considered distinct. We report a 5-year-old boy born to nonconsanguineous parents and presenting with ectodermal ...
Overlay removable partial dentures for a patient with ectodermal dysplasia: A clinical report
(Mosby, Inc, 2001-12-01)
The orofacial characteristics of ectodermal dysplasia include anodontia or hypodontia, hypoplastic conical teeth, underdevelopment of the alveolar ridges, frontal bossing, a depressed nasal bridge, protuberant lips, and ...
Use of dental implants in children: A literature review
(2001-05-01)
The aim of this literature review is to discuss the use of dental implants in growing patients and the influence of maxillary and mandibular skeletal and dental growth on the stability of those implants. It is recommended ...
Ectodermal dysplasia, ectrodactyly, clefting, anophthalmia microphthalmia, and genitourinary anomalies: nosology of goltz-gorlin syndrome versus eec syndrome
(Wiley-Blackwell, 1992-02-01)
We report on two unrelated Brazilian girls born to normal and nonconsanguineous parents and presenting ectodermal dysplasia, ectrodactyly, clefting, tear duct anomalies, and micro/anophthalmia. The clinical picture presented ...
Blepharocheilodontic (BCD) syndrome: Expanding the phenotype?
(2003-09-01)
We describe affected individuals in three generations of a family and another sporadic case, all Brazilian patients, with a combination of signs that diagnose the BCD syndrome. In addition to the cardinal signs, the sporadic ...
Regional odontodysplasia: Case report
(2007-11-01)
Regional odontodysplasia (RO) is a rare developmental anomaly involving both mesodermal and ectodermal dental components in a group of contiguous teeth. It affects the primary and permanent dentitions in the maxilla and ...
Incontinentia pigmenti (Bloch-Sulzberger syndrome): a case report.
(1993-06-01)
A syndrome--incontinentia pigmenti--of probable genetic etiology, is discussed and a new case report presented. The syndrome presents systemic and dental manifestations, which are separate although similar to those found ...
Hematopoietic stem cell transplantation in 29 patients hemizygous for hypomorphic IKBKG/NEMO mutations
(Amer Soc Hematology, 2017)
X-linked recessive ectodermal dysplasia with immunodeficiency is a rare primary immunodeficiency caused by hypomorphic mutations of the IKBKG gene encoding the nuclear factor kappa B essential modulator (NEMO) protein. ...