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PubMed1980

[Congenital atrichia associated with keratin cysts - Variant of partial ectodermal dysplasia].

This is a report of three male siblings suffering from almost complete Atrichia, dental and osseous anomalies and also multiple disseminated horny papules. These latter lesions are caused by follicular plugging with a horny substance possibly due to a disorder of keratinisation as seen in patients with partial ectodermal dysplasia. Clinical manifestations and possible variations of this syndrome are described and the hereditary patterns are discussed.

Abnormalities, Multiple↗
PubMed1977

[Cross-reacting epithelial antigen of ectodermal origin in mouse tumors].

Cross-reacting antigen (CR-antigen) previously found by indirect immunofluorescence in cell elements of murine multilayer epithelial cells (except corneal epithelium) to play the role of antigenic label of cover epithelia of ectodermal origin was found in mice tumor cells, related histogenetically with multilayer epithelium (transplantable proventricular cancer, two strains of squamous-cell cervical cancer in mice). CR-antigen was observed in transplantable breast adenocarcinoma and in transplantable mice teratocarcinoma.

Adenocarcinoma↗
PubMed1980

A genetic study of anodontia in X-linked hypohidrotic ectodermal dysplasia.

Dental examinations and tooth measurements were conducted on 16 mothers, 10 fathers, and 23 affected males in 15 families with X-linked hypohidrotic ectodermal dysplasia. Small teeth and congenital missing teeth were sufficiently consistent findings in obligate heterozygotes to suggest that carriers can usually be recognized by clinical criteria.

Anodontia↗
PubMed1994

Dental and craniofacial findings in hypohidrotic ectodermal dysplasia during the primary dentition phase.

A more detailed knowledge of dental and craniofacial features in hypohidrotic ectodermal dysplasia (HED) during childhood is needed in order to include these characteristics among diagnostic criteria. The present study comprised 5 HED children, 4 males and 1 female, during the primary dentition phase. Clinical and radiological dental findings consisted of multiple congenitally missing primary teeth, conoid primary incisors, moderate to severe taurodontic second primary molars. Supernumerary cusps and diastema were found as well. A pattern of symmetry was assessed for hypodontia in the primary dentition. The cephalometric study compared the HED sample to a matched non-syndrome sample and revealed abnormally short maxillary depth (p < 0.05), strongly reduced lower facial height (p < 0.01) and a reduction in facial soft tissue thickness (p < 0.05 - p < 0.01) in HED children. The importance of an early diagnosis and treatment of HED dento-facial malformations so as to improve esthetics and function is stressed.

Anodontia↗
PubMed1994

A neonate with ectodermal dysplasia ectrodactyly clefting syndrome and ventricular septal defect.

A 37-week gestation male boy was born to a gravida seven para six mother by spontaneous vertex delivery at home. The baby cried at birth. On day 3 of life, he was admitted for respiratory distress. Physical examination revealed ectrodactyly, thin dry skin, anomalous tear duct with cardiomegaly. X-ray revealed absent radii, cardiomegaly and hemivertebra at L1. Echocardiogram revealed perimembranous type of ventricular septal defect. A diagnosis of Ectodermal Dysplasia Ectrodactyly Clefting Syndrome with ventricular septal defect was made. He was managed conservatively in the nursery. However, he expired on day 27 of life following short spell of fever apnoeic episode due to neonatal sepsis.

Abnormalities, Multiple↗
PubMed1994

[Anodontia and hidrotic ectodermal dysplasia, hyporeactive to heat. Effect of acitretin].

We observed an adult female patient presenting an autosomal recessive ectodermal dysplasia difficult to classify among the various reported forms of this disease. She had the major signs of the hidrotic type of the disease (palmo-plantar hyperkeratosis, nail and hair dystrophies, and typical sweat glands) and anodontia as reported in the hypohidrotic form of the syndrome. We evaluated the function of the sweat glands and found them hyporeactive to a heat stimulus. Most of the epithelial alterations have improved under therapy by acitretin.

Acitretin↗
PubMed1994

Mosaic expression of hypohidrotic ectodermal dysplasia in an isolated affected female child.

BACKGROUND: Hypohidrotic ectodermal dysplasia (HED) is characterized by hypotrichosis, hypodontia, onychodysplasia and, as the most striking feature, hypohidrosis. The X-linked recessive form of HED, also known as Christ-Siemens-Touraine syndrome, is the most frequent and widely documented form. A clinically identical autosomal recessive form of HED has also been described. Because of the X-linked mode of inheritance, nearly all observations have concerned pedigrees of predominantly male affected patients. We present a rare isolated affected female child with a mosaic expression of HED. We attempted to assess the mode of inheritance in our case. OBSERVATIONS: We documented the characteristic clinical appearance in our proband, as well as the scanning electron microscopic findings regarding the hair. The starch-iodine test results in this patient revealed the clinical expression of HED in a mosaic fashion, running along the Blaschko lines. CONCLUSIONS: The starch-iodine test results proved to be useful in the assessment of carriers of X-linked HED, and our proband was considered to an isolated affected female with a mosaic expression of HED.

Child, Preschool↗
PubMed1993

Dentures for a 3-yr-old child with ectodermal dysplasia: case report.

A case of a 3-year-old boy with X-linked hypohidrotic ectodermal dysplasia is presented. Complete maxillary and mandibular dentures were made at 3 years and 2 months of age. The process for making the dentures is described. Obtaining enough patient cooperation and proper application of behavioral management techniques are critical factors for the procedure described in the article.

Anodontia↗
PubMed1976

[Organization and differentiation of the somite mesoderm cultured in the presence of adjacent tissues (notocord, spinal cord, ectoderm, endoderm) in anurans].

The explantation of pieces of somitic mesoderm alone or in association with neighbouring tissues (notochord, spinal cord, ectoderm, endoderm) shows slearly the power of self-differentiation of the somitic tissue. Only, the spinal cord does not stimulate the differentiation of the somitic mesoderm into muscular tissue. Only the notochord does not stimulate the formation of typical myotomes. It seems that ii does not possess biochemical activity, its effect if reduced to a mechanical role.

Age Factors↗
PubMedDate not supplied

[The translation symmetry in cell mosaics of amphibian embryonic ectoderm as a manifestation of their modular structure].

A theoretical proposition that the cell mosaics possess translation symmetry has been experimentally confirmed by scanning electron microscopy of the common frog embryonic ectoderm. This symmetry is a manifestation of the tissues modular structure and it provides new data for quantitative estimation of composition and structure of tissue morphofunctional element as well as for diagnostics and prediction of tissue development.

Amphibians↗
PubMed1993

[Familial ectodermal dysplasia with agenesis of the breasts and the external urethral sphincter. Description of a case].

The Authors report a case of athelia and amastia observed in a 28-year-old woman. The association of severe dental alterations, nail dystrophies and irregular cutaneous hyperpigmentation with normal sweating, make it possible to catalogue the anomaly in the hydrotic ectodermal dysplasia, an autosomal dominant hereditary disease. The syndrome, transmitted by the father, is also present in two brothers of the patient: the association of sphincter urethrae agenesis, responsible for urinary incontinence unsuccessful treated with two surgical operations, is to be considered quite fortuitous.

Abnormalities, Multiple↗
PubMed1993

Detection of a molecular deletion at the DXS732 locus in a patient with X-linked hypohidrotic ectodermal dysplasia (EDA), with the identification of a unique junctional fragment.

X-linked hypohidrotic ectodermal dysplasia (EDA) has been localized to the Xq12-q13.1 region. A panel of genomic DNA samples from 80 unrelated males with EDA has been screened for deletions at seven genetic loci within the Xq12-13 region. A single individual was identified with a deletion at the DXS732 locus by hybridization with the mouse genomic probe pcos169E/4. This highly conserved DNA probe is from locus DXCrc169, which is tightly linked to the Ta locus, the putative mouse homologue of EDA. The proband had the classical phenotype of EDA, with no other phenotypic abnormalities, and a normal cytogenetic analysis. A human genomic DNA clone, homologous to pcos169E/4, was isolated from a human X-chromosome cosmid library. On hybridization with the cosmid, the proband was found to be only partially deleted at the DXS732 locus, with a unique junctional fragment identified in the proband and in three of his maternal relatives. This is the first determination of carrier status for EDA in females, by direct mutation analysis. Failure to detect deletion of the other loci tested in the proband suggests that the DXS732 locus is the closest known locus to the EDA gene. Since the DXS732 locus contains a highly conserved sequence, it must be considered to be a candidate locus for the EDA gene itself.

Adult↗
PubMed1977

Hidrotic ectodermal dysplasia with diffuse eccrine poromatosis.

Eccrine poromatosis (EP) of remarkable severity has been observed in a patient with hidrotic ectodermal dysplasia (HED), a member of the kindred originally described by Clouston. Neither the EP nor its association with HED has been previously reported.

Adult↗
PubMed1977

[Anhidrotic ectodermal dysplasia--Identification of heterocygote (carrier) females (author's transl)].

We report on a newborn male infant suffering from anhidrotic ectodermal dysplasia. This x-linked recesive disorder has a high letality during the first year of life. Survivors are psychologically grossly impaired. This necessitates identification of carrier females. Characteristics of heterocygotes (e.g. palmar ridge flattening, paucety of pores, dermoglyphic pattern) are described. In pregnancy amniocentesis and chromosome analysis for sex determination are to be recommended.

Adult↗
PubMed1996

Activin treated urodele ectoderm: a model experimental system for cardiogenesis.

The tissue interactions which comprise the inductive phenomena associated with urodele heart morphogenesis are relatively well understood. In order to take full advantage of the experimental potential of this system formulation of an in vitro tissue culture system would be very helpful. Herein are described conditions for culturing Cynops pyrrhogaster early gastrula ectoderm tissue in the presence of the peptide growth factor activin. Two-week old explant cultures frequently displayed beating heart-like rudiments within. The beating frequency was measured and the extent to which cytodifferentiation mimicked normal heart differentiation assessed. Both measurements provided optimistic assessments which should encourage further exploitation of this model system.

Activins↗