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Surface topography of isolated gastrula ectoderm of Triturus vulgaris.

The surface topography of the outer cell layer of the two-layered gastrula ectoderm of Triturus vulgaris resembles that of the other amphibians: the cells are tightly packed and devoid of pseudopodia. The cells of the inner layer are spherical and interconnected with pseudopodia of various lengths. After excision of the ectoderm the peripheral cells of the outer layer elongate perpendicularly to the edge of the explant, while the number of pseudopodia on the inner layer greatly increases. The results show that the curling-up of the explant and formation of a sphere results from locomotor activity of the cells in both layers.

Animals↗

Premature cataracts in a family with hidrotic ectodermal dysplasia.

In a family with hidrotic ectodermal dysplasia affecting five members in three generations, bilateral premature cataracts have developed in four of the five affected individuals. To our knowledge, this represents the first report of a family in which bilateral premature cataracts appear to be inherited with hidrotic ectodermal dysplasia.

Adult↗

Hypohidrotic ectodermal dysplasia: characteristics and treatment.

Hypohidrotic ectodermal dysplasia is a rare congenital disease that affects several ectodermal structures. The disease is usually transmitted as an X-linked recessive trait in which the gene is carried by the female and manifested in the male. Manifestations of the disease differ in severity and may involve teeth, skin, hair, nails, and sweat and sebaceous glands. Most affected children require extensive dental treatment to restore their appearance and help the development of a positive self-image.

Adolescent↗

Hypohydrotic ectodermal dysplasia affecting a female patient.

Ectodermal dysplasia syndromes are extremely rare. Hypohydrotic Ectodermal Dysplasia (HED) is classically an X-linked recessive condition affecting males, although a clinically identical autosomal recessive form of this condition has been described. The patient described in this article is female, who was initially thought to be a manifesting carrier for X-linked HED. However, the pedigree and examination of other family members suggest that she has the rarer autosomal recessive form. The patient is also edentulous and requires complete dentures, a concept regarded by many as a novelty in young children.

Anodontia↗

Type II congenital dyserythropoietic anemia in a patient with ectodermal dysplasia. Distinction from dyskeratosis congenita.

PURPOSE: We describe a patient who presented with severe anemia and ectodermal dysplasia. PATIENTS AND METHODS: This is a case report of a patient whose anemia was evaluated at New York Hospital and then returned to Australia where further testing was performed. RESULTS: The history indicated that this was a chronic anemia. Bone marrow examination showed binucleated late normoblasts consistent with congenital dyserythropoietic anemia type II (CDA II) and not dyskeratosis congenita. Paroxysmal nocturnal hemoglobinuria was excluded despite the presence of a positive sucrose hemolysis test. Other types of acquired and congenital anemias were excluded by testing. CONCLUSIONS: This is the first patient reported with coincident CDA II and ectodermal dysplasia.

Anemia, Dyserythropoietic, Congenital↗

Hypohidrotic (anhidrotic) ectodermal dysplasia: molecular genetic research and its clinical applications.

X-linked hypohidrotic (anhidrotic) ectodermal dysplasia (EDA) results in abnormal morphogenesis of the teeth, hair, and eccrine sweat glands. The disorder is inherited as an X-linked recessive trait with significant morbidity and mortality in affected males, but with little to no clinical expression in many carrier females. Therefore, despite much effort, carrier detection based on clinical findings has been problematic. The locus for the disorder has now been localized to a region of less than one million base pairs on the X-chromosome, permitting DNA based carrier, prenatal, and early neonatal testing for many families. The isolation and sequencing of the EDA gene itself should be forthcoming by the application of the techniques of positional cloning. The isolation of the gene will allow direct mutation detection in even sporadic cases, and will further improve genetic counseling. It will also permit analysis of how the gene functions in the normal development of the epidermal and oral ectoderm, which may result in improved therapies for the disorder.

Chromosome Mapping↗

Repair of saddle nose deformity in Wegener's granulomatosis and ectodermal dysplasia.

Two cases are reported involving surgical treatment of a saddle nose deformity due to Wegener's granulomatosis and ectodermal dysplasia, respectively. The association of ozena with both diseases requires special consideration for any type of transplants because of a high risk of infectious complications. By extranasal incisions the nasal dorsum has been successfully reconstructed by transplanting autogenic conchal cartilage. There has been no resorption or displacement of the transplant after twelve months in the case of ectodermal dysplasia, and after 25 months in the patient with Wegener's granulomatosis despite a severe recurrence of this disease.

Adult↗

Clinical management of ectodermal dysplasia.

Children with ectodermal dysplasia may have hypodontia or anodontia and are often treated dentally with conventional adult appearing prosthesis which are focused only on the oral manifestations of the syndrome. This case report describes the management of an ectodermal dysplasia patient to provide improved aesthetics, function, and emotional development.

Anodontia↗

The ectrodactyly-ectodermal dysplasia-clefting syndrome: a literature review and case report.

Ectrodactyly, atypical anhidrotic ectodermal dysplasia, and cleft lip and palate are the principal manifestations of ectrodactyly-ectodermal dysplasia-clefting syndrome. Intraorally, the common manifestations of the syndrome are hypodontia, enamel hypoplasia, and generalized microdontia. In addition, the patient may suffer from xerostomia and have a deeply fissured tongue. The oral mucosa appears, in some patients, to have an increased susceptibility to candidiasis. This case report describes a patient who exhibits many of the manifestations of the syndrome and explains ways in which many of the anomalies of the dentition can be esthetically and functionally corrected.

Abnormalities, Multiple↗

Ectodermal dysplasia with associated double tooth.

The case describes a double molar tooth in a seven-year-old girl who has ectodermal dysplasia. The most characteristic dental findings in ectodermal dysplasia are hypodontia and conically shaped crowns. In our case a double tooth was also present in the primary molar region, in addition to these characteristic findings.

Anodontia↗

Effect of dorsal and ventral limb ectoderm on the development of the limb of the embryonic chick.

Limb ectoderm plus a small amount of subjacent mesoderm obtained from the dorsal or the ventral surface of each of the four limbs of the chick embryo were grafted to the dorsal surface of the right wing in either reversed or normal orientation. The host wings developed in abnormal humerus in all cases except when the graft was obtained from the dorsal surface of the right wing and was in normal orientation. However, the nature of the abnormalities varied with the source of the graft and with the orientation of the graft. It is unlikely that the abnormalities were related solely to the small amount of mesoderm grafted since previous experiments have demonstrated that the mesoderm would regulate. Flank ectoderm plus a small amount of flank mesoderm did not cause limb abnormalities when grafted in the same manner.

Animals↗

New form of hidrotic ectodermal dysplasia in a Lebanese family.

We report a sister and brother born to consanguineous parents presenting with severe hypodontia, fine hair, and onychodysplasia. Five other relatives are similarly affected. The comparison with other ectodermal dysplasias is presented and discussed. The possibility of a new autosomal recessive form of ectodermal dysplasia is raised.

Adult↗

Ectodermal dysplasia in adulthood: the restorative difficulties and management.

Ectodermal dysplasia can result in a number of oral problems. As the condition is usually diagnosed in childhood, multidisciplinary specialist dental care at an early age may minimize the long-term dental complications. This article describes the restorative management of an adult patient suffering from ectodermal dysplasia for whom such support was not available, resulting in the provision of complex and highly invasive restorative treatment in adult life to provide a functional and aesthetically acceptable dentition. The various treatment options that were considered are discussed together with the details of the treatment provided.

Adult↗

Hidrotic ectodermal dysplasia in a black mother and daughter.

Hidrotic ectodermal dysplasia is a rare genodermatosis with clinical hallmarks of alopecia, nail dystrophy, and palmoplantar hyperkeratosis. This report calls attention to the first reported cases, to our knowledge, of hidrotic ectodermal dysplasia in a black mother and daughter.

Adolescent↗

FGF-2 alters the fate of mouse epiblast from ectoderm to mesoderm in vitro.

We have developed an in vitro differentiation assay to characterize the ability of peptide growth factors to induce differentiation in mouse epiblast. We report that culturing explants of mouse anterior epiblast, a tissue normally fated to give rise to neuroectoderm and surface ectoderm, in a serum-free, chemically defined medium with 10-50 ng/ml of FGF-2 induced gross changes in cell morphology. Treated cells adopted an elongated, flattened morphology but did not migrate from the explant. Instead, FGF-2-treated cells condensed into multicellular mounds or ridges. Immunocytochemistry showed that cells in treated explants expressed vimentin and in situ hybridization demonstrated that FGF-2 induced the expression of brachyury, goosecoid, and myo-D in regions of treated explants displaying morphological differentiation. Control explants cultured with platelet-derived growth factor AA (PDGF AA), transforming growth factor-beta 1 (TGF-beta 1), or in defined medium alone showed no morphological or biochemical differentiation. These results indicate that FGF-2 altered the fate of mouse anterior epiblast from ectoderm to mesoderm in vitro. Cell migration, which is characteristic of primitive streak mesoderm in vivo, was not induced by FGF-2 in these assays. However, the changes in morphology and the expression of mesodermal genes in vitro do support an early role for FGF signaling in the induction of mouse primitive streak mesoderm, as well as in later patterning events during embryogenesis.

Animals↗

Cytological and microspectrophotometric analysis of mesodermalized explants of Triturus gastrula ectoderm.

Using isolated presumptive ectoderm of the newt (Triturus pyrrhogaster) embryos as a reactor and extract of rat bone marrow as a mesodermal inductor, changes of cell number and mitotic index of the reactor cells were studied. In early stages of cultivation the increase in cell number in the mesodermalized ectodermal piece was slower than in the non-mesodermalized epidermal piece; but after 24 h it showed abrupt increase and reached a cell population equal to that of the control at 48 h of cultivation. In the experimental series, the mitotic index was 0 at 4 h after the application of the inducing stimulus, but increased precipitously in the next 8 h and reached a level of 4-4% at 12 h and thereafter decreased gradually. The cell cycle stopped at the S phase and stayed in it for several hours after the application of inductor. A sudden fall in cell number, observed in the mesodermalized epidermal piece between the 4th and the 8th h after the application of inducing stimulus, seems to be attributable to cell death which was brought about by the inducing stimulus. In the histogenetic process phases of repression on mitosis by an inducing stimulus, cell proliferation and nonproliferation seem to succeed each other.

Animals↗

Interdigital chondrogenesis and extra digit formation in the duck leg bud subjected to local ectoderm removal.

In the chick embryo the interdigital tissue in the stages previous to cell death exhibits in vitro a high chondrogenic potential, and forms extra digits when subjected in vivo to local ectodermal removal. In the present work we have analyzed the chondrogenic potential both in vivo and in vitro of the interdigital mesenchyme of the duck leg bud. As distinct from the chick, the interdigital mesenchyme of the duck leg bud exhibits a low degree of degeneration, resulting in the formation of webbed digits. Our results show that duck interdigital mesenchyme exhibits also a high chondrogenic potential in vitro until the stages in which cell death starts. Once cell death is finished chondrogenesis becomes negative and the interdigital mesenchyme forms a fibroblastic tissue. In vivo the interdigital mesenchyme of the duck leg bud subjected to ectoderm removal forms ectopic foci of chondrogenesis with a range of incidence similar to that in the chick. Unlike those of the chick the ectopic cartilages of the duck are rounded and smaller, and appear to be located at the distal margin of the interdigital mesenchyme. Formation of extra digits in the duck occurs with a lower incidence than in the chick. It is concluded that ectopic chondrogenesis and formation of extra digits is related to the intensity of interdigital cell death. The non-degenerating interdigital mesenchymal cells destined to form the interdigital webs of the duck appear to contribute very little to the formation of interdigital cartilages.

Animals↗