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PubMed1977

Autosomal recessive hypohidrotic ectodermal dysplasia with subclinical manifestation in the heterozygote.

Sweat pores on the epidermal ridges are hypoplastic and reduced in number in three sisters affected with autosomal recessive hypohidrotic dysplasia. The heterozygote state is expressed by a reduced number of qualitatively normal sweat pores (14.07 +/- 8.59 as compared to 22.27 +/- 2.33 in controls). Clinical and genetic considerations suggest that this may be a distinct type that has to be differentiated from other autosomal recessive hypohidrotic ectodermal dysplasias. Heterozygote manifestation may contribute to delineate this groups of disorders further.

Adolescent↗
PubMed1996

Syringomas, natal teeth and oligodontia: a new ectodermal dysplasia?

A 26-year-old female is described with multiple facial syringomas, neonatal teeth and oligodontia. Her father has similar dental findings but no reliable evidence of syringomas. This combination of findings probably represents a new autosomal dominant ectodermal dysplasia with variable expression between the sexes.

Adult↗
PubMed1977

Reduction of the rate of outgrowth, cell density, and cell division following removal of the apical ectodermal ridge of the chick limb-bud.

Removal of the apical ectodermal ridge causes a reduction in the rate of outgrowth of the wing-bud and the loss of distal parts. More specifically it causes a short-term increase in cell density and cell death and a decrease in the rate of cell proliferation. The evidence supports the hypothesis of density-dependent control of cell division and suggests that there may also be a mechanism regulating skeletal length at the time of differentiation. An informal model is presented to explain the observations.

Animals↗
PubMed1994

Early treatment considerations for oligodontia in ectodermal dysplasia: a case report.

Two brothers diagnosed with ectodermal dysplasia were treated for 5 years during their mixed-dentition periods for problems arising from absence of teeth (oligodontia) and abnormally formed teeth. An individualized approach to restorative and prosthodontic management allowed dynamic and evolving treatment attuned to their concern about their appearance.

Anodontia↗
PubMedDate not supplied

[The morphogenetic reactions of the ectoderm in the early gastrula of the clawed toad to mechanical stretching].

The ventral ectoderm explants of the Xenopus laevis early gastrula were stretched on an elastic substrate 1.5- to twofold for 30 min, then fixed in the stretched state and incubated for up to 6 h. The stretching of explants generated their two-phase active reaction, which was estimated using the criterion of eccentricity of cells and whole explants developed in this study. At the first phase, during the external force application, the explant cells were elongated towards its stretching to a markedly greater extent than the whole explant: eccentricity of the whole cells exceeded that of the whole explants. Cell processes elongated in the same direction appeared, cell contacts and apical cytoskeleton structures developed, and mechanical tensions increased. At the second phase, within 5 h after the stretching, the shape of individual cells, rather than the whole explant, returned to the initial one (approximately isotropic), mechanical tensions decreased, and visible morphogenesis of the explant proceeded: it acquired a complex shape with longitudinal groove and polar thickenings. We conclude that intercalary movements of the cells are present at the second phase of the active explant reaction and morphogenesis is realized at the phase of relaxation of mechanical tensions.

Animals↗
PubMed1997

[Ectodermal dysplasia: multiple manifestations of a hereditary disease].

Ectodermal dysplasia is a hereditary disease that can manifest itself in many ways. Four cases, including two cases involving heterozygote twins, are presented. The clinical signs of the disease, as observed are compared to the knowledge offered in the classic and modern literature. Special attention is given to the intraoral manifestations of the disease, and to the solutions that a multidisciplinary dental team could consider to improve the masticatory function and the facial esthetics of patients affected by this disease.

Anodontia↗
PubMed1997

Ectodermal dysplasia, cleft lip/palate, and severe cutaneous and osseous syndactyly in a mentally retarded girl: a new multiple malformation syndrome.

A 13-year-old mentally retarded girl with severe cutaneous and osseous syndactyly of the hands and feet, cleft lip/palate, and ectodermal dysplasia is presented. We conclude that the pattern of malformations described represents a new multiple malformation syndrome. A comparison with Zlotogora-Ogür syndrome is presented.

Abnormalities, Multiple↗
PubMed1997

Overdenture prosthesis for oral rehabilitation of hypohidrotic ectodermal dysplasia: a case report.

The dentition of a patient with ectodermal dysplasia was restored with a modified hollowed maxillary overdenture opposing a conventional mandibular overdenture. Lingualized occlusion was used because it was the ideal occlusal scheme for this patient to achieve denture stability. The lingual cusps of the maxillary posterior teeth contacted the fossae of the mandibular teeth to create freedom of movement and to prevent lateral interference.

Child, Preschool↗
PubMedDate not supplied

Craniofacial changes following early prosthetic treatment in a case of hypohidrotic ectodermal dysplasia with complete anodontia.

The authors describe early prosthetic treatment in a case of hypohidrotic ectodermal dysplasia with complete anodontia. Craniofacial changes were analyzed by means of Ricketts' cephalometric analysis and superimposition methods. Early prosthetic treatment for complete anodontia determined a backward-downward rotation of the mandible with consequent normal positioning of the chin in the space, normalization in lower facial height, reduction in convexity of the face, and improvement in facial esthetics. Superimposition methods demonstrated that the direction of growth of both the maxilla and the mandible was progressing favorably. Improvements in esthetics and in the function of masticatory and perioral muscles, together with their positive psychological impact, were also discussed.

Age Factors↗
PubMed1976

Incorporation of tritiated cytochalasin B into ectodermal explants of the amphibian gastrula.

Ectodermal explants from gastrulae of Triturus alpestris were exposed to tritiated cytochalasin B (5 and 10 mug/ml) for 1/2-2 1/2 h. The flat explants failed to heal into compact spheres and the component cells gradually rounded up. Radioactivity in the fixed and sectioned material was analysed by light and electron microscopic autoradiography. Silver grains were found predominantly over the yolk platelets, but were also scattered over other cellular components. Areas containing pigment granules or the feltwork material, present in the apices of dissociating cells of the superficial layer, exhibited higher activity than areas containing mitochondria or cytoplasmic matrix. Lipid droplets and nuclei showed comparatively little activity. The results are discussed in relation to previous findings on the uptake of tritiated cytochalasin D by cell fractions.

Animals↗
PubMedDate not supplied

Pattern of permanent teeth present in individuals with ectodermal dysplasia and severe hypodontia suggests treatment with dental implants.

PURPOSE: The objective of this study was to assess the pattern of permanent teeth present in a self-selected sample of 17 female and 35 male patients with ectodermal dysplasia presenting for treatment with dental implants. METHODS: Mean age of sample: 18.7 years, age range: 5.9 to 60.9 years. Panoramic radiographs were examined independently by two investigators to determine the permanent teeth present. None of the sample reported extractions of permanent teeth prior to presenting for implants. RESULTS: Permanent teeth most likely to be present, reported as a percentage of the patient sample with that tooth, were: maxillary central incisors (42%), maxillary first molars (41%), mandibular first molars (39%), maxillary canines (22%), mandibular second molars (17%), maxillary second premolars (15%), and mandibular premolars (12%). Comparing dentition by quadrants, mandibular anterior teeth (canines and incisors) were least likely to be present. CONCLUSIONS: Results support previous findings that the maxillary central incisors, maxillary first molars, mandibular first molars, and maxillary canines are the most conserved teeth in hypodontia. Successful use of osseointegrated implants in the anterior mandibles of most of these patients suggests that habilitation of the mandible with dental implant-supported prostheses is a reasonable option.

Adolescent↗
PubMed1998

Management of ectodermal dysplasia: a literature review.

Oral rehabilitation is commonly difficult in cases of ectodermal dysplasia because the affected individuals are quite young when they are evaluated for treatment. Most affected children require extensive dental treatment in order to restore their appearance and help them develop a positive self-image. This article reviews the dental management of the disorder, including its psychosocial aspects and timing of different modalities.

Child↗
PubMed1997

Ectodermal abnormalities in Kabuki syndrome.

We describe a girl with Niikawa-Kuroki (Kabuki) syndrome (NKS) with conical incisors, hypodontia, hypoplastic nails, and brittle hair. Abnormal teeth are common in NKS and support a hypothesis of autosomal dominant inheritance of the syndrome [Halal et al., 1989; Silengo et al., 1996]. Hair abnormalities have never been investigated in NKS. The ectodermal involvement in NKS could represent an important clue for the understanding of the pathogenesis of this syndrome.

Abnormalities, Multiple↗
PubMed1996

Tumorigenicity of cerebellar primitive neuro-ectodermal tumors in athymic mice correlates with poor prognosis in children.

The histogenesis of medulloblastoma, also described as a cerebellar primitive neuro-ectodermal tumor (PNET), remains controversial and unresolved. In addition, genetic markers which characterize cerebellar PNETs with poor prognosis in children have not been identified. Since xenografts can be valuable tools for better understanding the genetic events involved in cerebellar PNETs, small fragments of tumor samples from 17 children with newly diagnosed cerebellar PNETs were transplanted s.c. into female athymic Swiss mice. Eleven were non-metastatic and 6 were metastatic PNETs. Eight tumors (47%) were tumorigenic. Histological analysis showed 6 typical medulloblastomas, 1 PNET with melanin pigment and 1 PNET with a rhabdoid phenotype. Wide heterogeneity was observed in tumor growth, with a doubling time ranging from 8 to 81 days after the first passage. Tumorigenicity was correlated with the metastatic phenotype of the tumor (p < 0.001). All the patients but one with a tumorigenic tumor relapsed and died. The survival of patients with a non-tumorigenic PNET (67%) was significantly higher than that of patients with a tumorigenic PNET (13%) (p < 0.02). None of the xenografts or tumors from patients exhibited N-myc-gene alteration. Only one xenograft showed c-myc amplification, with an abnormal 15-kilobase fragment. None of the 17 tumors from patients showed amplification or c-myc-gene rearrangement. In conclusion, tumorigenicity of cerebellar PNETs strongly correlates both with the metastatic phenotype of the tumors and with the disease-free survival of the patients. In addition, genetic events other than c-myc-gene amplification might be involved in cerebellar PNETs with poor prognosis.

Animals↗
PubMed2000

Further evidence for autosomal dominant inheritance and ectodermal abnormalities in Kabuki syndrome.

Most cases with Kabuki syndrome (KS) were reported sporadically. Recently, a few familial cases of KS were reported. This report provides further evidence that the KS is inherited as a dominant trait with variable expressivity. The proposita is an 18-month-old girl with facial findings characteristic of Kabuki syndrome, prominent fingertip-pads, a midsagittal cleft of vertebral body D4, hypotonia, and psychomotor retardation. Her mother had a similar facial appearance, prominent, cup-shaped ears, an abnormal dentition, early breast development, and low-normal intelligence. Because mother and daughter both had evident Kabuki syndrome, we conclude that KS in this family is inherited as a dominant trait. Further family history supports this finding. Microscopic examination of the hair of the proposita shows abnormalities consisting of trichorrhexis nodosa, twisting of the hairshafts, and irregularity of the diameter of the hair, as was described recently in a patient with KS. This could be another occasional finding in this syndrome, but further studies are required. The presence of abnormal hair, nails, and the commonly described tooth abnormalities in KS further suggest ectodermal involvement in this syndrome.

Abnormalities, Multiple↗
PubMed1988

Myosin, tubulin and laminin immunoreactivity in the ectoderm of the growing area opaca of the chick embryo.

Distribution of myosin, tubulin and laminin immunoreactive cells in the area opaca of the young chick embryo (Stages 4-8 HH) was studied using immunofluorescence technique. For the three markers, the number of stained cells increased with the age of the blastoderm. Cells stained for tubulin and laminin, were distributed throughout the area opaca, showing no supracellular organization. On the contrary, the cells stained for myosin became organized in a ring surrounding the area pellucida. This pattern appeared at the stage 6. Such an heterogenous distribution of the markers suggests a functional diversification of the ectodermal cell monolayer forming at these early developmental stages the area opaca. This idea is also supported by the results of autoradiography for tritiated thymidin which showed that the edge cells did not synthetize DNA and consequently did not divide.

Animals↗
PubMed1996

Gangliosides modulate the growth rate and cell phenotype of a murine primitive neuro-ectodermal tumour.

Intratumoural administration of gangliosides (GSD) into a murine primitive neuro-ectodermal tumour, growing in the epicranial subcutaneous tissue of syngenic rats, results in a decrease in the tumour growth rate and causes a more differentiated phenotype of tumour cell, with immunohistochemical expression of neuronal markers. These findings suggest that the potential usefulness of intralesional administration of GSD for the control of tumours of primitive neuro-epithelial character could be considered.

Animals↗