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At least 613 records · Page 34Linked to original sources
PubMed2002

Clinical management of hypohidrotic ectodermal dysplasia with anodontia: case report.

Ectodermal dysplasia is a rare hereditary disorder with a characteristic physiognomy. The case of a 5-year-old child with hypohidrotic ectodermal dysplasia and complete anodontia is presented. Because of the anodontia and the need for treatment at an early age, the prosthetic management of such a young child can be difficult. Complete dentures were provided to encourage a normal psychological development and to improve the function of the stomatognathic system.

Anodontia↗
PubMed1995

Ectodermal dysplasia syndrome in siblings with true keloids, stenosis of the esophagus after operations for congenital achalasia and renovascular hypertension due to stenosis of renal artery.

Ectodermal dysplasia syndrome (EDS) is a rare hereditary disease, with symptoms brought about by dysplasia of ectodermal tissue (such as skin, teeth, nails, and hair). This report details the cases of two siblings (41 and 43 year old sisters) with autosomal recessive and hydrotic EDS complicated by esophageal achalasia, postoperative stenosis of esophagus, true keloids, renovascular hypertension, incomplete malrotation of the bowel, and demyelination of the brain.

Adult↗
PubMed2006

Palatal size and shape in 6-year olds affected by hypohidrotic ectodermal dysplasia.

OBJECTIVE: To analyze the size and shape of the hard tissue palate of Italian subjects with hypohidrotic ectodermal dysplasia (HED). MATERIALS AND METHODS: The morphology and the dimensions of the hard tissue palate were analyzed in eight 6-year-old boys affected by HED. Four of the boys were completely edentulous and four partially dentate. Palatal landmarks were identified on stone casts and digitized with three-dimensional computerized electromagnetic instrumentation. Palatal length, slope, width, and maximum palatal height in both the sagittal and frontal planes were measured. From the coordinates of palatal landmarks, a mathematical equation of palatal shape was constructed, independent of size. HED palatal data were compared with reference data obtained from 12 healthy boys with a complete deciduous dentition. RESULTS: Palatal length and height in both the sagittal and frontal planes were significantly reduced in HED as compared with control individuals. A less steep (not significant) palatal slope was found in HED than in reference subjects, whereas similar palatal width values were observed. All palatal measurements were larger in partially dentate than in edentulous patients. Both HED and edentulousness influenced palatal shape. The HED boys had a relatively lower palate than the reference boys. In the edentulous HED boys, the hard tissue palate was relatively lower than in partially dentate HED subjects. CONCLUSIONS: Palatal size and shape were significantly modified by the presence of hypohidrotic ectodermal dysplasia, and the major alterations were found in edentulous HED subjects.

Anodontia↗
PubMed1997

Atopic dermatitis may be a genetically determined dysmaturation of ectodermal tissue, resulting in disturbed T-lymphocyte maturation. A hypothesis.

Although atopic dermatitis is a skin disorder, it includes immune deviations such as T-cell accumulation and activation in the skin, resulting in chronic, relapsing eczema. The T-lymphocyte activation in the skin is not accompanied by specific allergies in up to two thirds of the patients. It has been shown that T-cell lines and clones can be established from skin biopsies of patients with atopic dermatitis showing cytokine-dependent, but antigen-independent, continuous growth in vitro. This indicates the existence of skin-homing T-lymphocytes with growth requirements different from those of mature T-lymphocytes in the blood. We suggest that atopic dermatitis is a genetically determined change of ectodermal tissue. The thymic epithelium is derived from the ectoderm, and because of that we hypothesize that the maturation of the T-cell immune system of persons who develop atopic dermatitis is disturbed due to a faulty selection of T-lymphocytes in the thymus. "Dys"-matured T-cells leave the thymus as a consequence of faulty selection and continue their growth in the skin. The cells are eventually eradicated by the immune surveillance conducted by the normal part of the patients' immune system and as a consequence of diminished output of faulty selected T-lymphocytes during maturation. Because of the increased proliferation capacity of the aberrant T-cells, a cytokine imbalance occurs and in some patients this leads to the development of type I allergies due to a skewing of the humoral immune system towards IgE production.

Dermatitis, Atopic↗
PubMed1984

Xanthoma tendinosum in a normolipemic ectodermal dysplasia patient.

A female patient with congenital ectodermal dysplasia is described, who developed xanthoma tendinosum in her hands simulating rheumatoid arthritis. The serum lipids of this patient were normal. Both ectodermal dysplasia and xanthoma tendinosum in a normolipemic patient are rather uncommon and to our knowledge have not been previously reported in one and the same patient.

Adult↗
PubMed1994

Epithelial cystic lesions of the sellar and parasellar region: a continuum of ectodermal derivatives?

Cystic epithelial lesions of the sellar and parasellar region are classified on the basis of histology and location into Rathke's cleft cysts, epithelial cysts, epidermoid cysts, dermoid cysts, and craniopharyngiomas. A retrospective review of the clinical presentation, radiological findings, and histology was performed on 19 such lesions, and a survey of the literature pertinent to the classification, clinical presentation, and embryology of these lesions was conducted. Presentation was nonspecific and not predictive of histology. Imaging studies were generally useful in distinguishing these tumors, with the exception of Rathke's cleft cysts, suprasellar epidermoid cysts, and craniopharyngiomas, which frequently could not be differentiated. On microscopic examination, most lesions fit into distinct categories; however, overlap was common among all and some could not be definitively categorized by histological criteria. Evidence supportive of an ectodermal ancestry for sellar and parasellar epithelial-lined cystic lesions is presented. Based on the current findings and a review of the literature, it is suggested that these lesions represent a continuum of ectodermally derived cystic epithelial lesions.

Adolescent↗
PubMed2005

Subtotal amelia in a child with autosomal recessive hypohidrotic ectodermal dysplasia.

We report an inbred Tunisian family, in which the proband manifested signs of hypohidrotic ectodermal dysplasia, subtotal amelia, scoliosis and left renal agenesis. Two other family members had the full clinical criteria of hypohidrotic ectodermal dysplasia, characterized by deficient sweat glands, hypodontia, hypoplasia of the mucous glands, and fine hair. Nine family subjects had variable clinical expression of the disorder.

Child↗
PubMed2000

Ectrodactyly-ectodermal dysplasia-clefting syndrome.

Ectrodactyly-ectodermal dysplasia-clefting syndrome is a rare congenital anomaly that affects tissues of mesodermal and ectodermal origin. Musculoskeletal involvement frequently requires orthopedic intervention. The authors present a review of the literature pertaining to this rare syndrome as well as a case report of a female patient who exhibited the complete clinical triad. A description of the surgical management of her condition is also presented.

Abnormalities, Multiple↗
PubMed2000

Hypohidrotic ectodermal dysplasia.

Hypohidrotic ectodermal dysplasia was first described by Thurnam in 1848. It is a rare, X-linked, recessive disorder characterized by anhidrosis or hypohidrosis, hypotrichosis, dental hypoplasia and characteristic facial features. Herein, we report a typical case of hypohidrotic ectodermal dysplasia. A 20-year-old male presented with the above symptoms at birth. When a family history was taken, it was discovered that his uncle (mother's brother) had the same characteristic facial features and hypotrichosis.

Adult↗
PubMed2000

Two lineage boundaries coordinate vertebrate apical ectodermal ridge formation.

Proximal-distal outgrowth of the vertebrate limb bud is regulated by the apical ectodermal ridge (AER), which forms at an invariant position along the dorsal-ventral (D/V) axis of the embryo. We have studied the genetic and cellular events that regulate AER formation in the mouse. In contrast to implications from previous studies in chick, we identified two distinct lineage boundaries in mouse ectoderm prior to limb bud outgrowth using a Cre/loxP-based fate-mapping approach and a novel retroviral cell-labeling technique. One border is transient and at the limit of expression of the ventral gene En1, which corresponds to the D/V midline of the AER, and the second border corresponds to the dorsal AER margin. Labeling of AER precursors using an inducible Cre showed that not all cells that initially express AER genes form the AER, indicating that signaling is required to maintain an AER phenotype. Misexpression of En1 at moderate levels specifically in the dorsal AER of transgenic mice was found to produce dorsally shifted AER fragments, whereas high levels of En1 abolished AER formation. In both cases, the dorsal gene Wnt7a was repressed in cells adjacent to the En1-expressing cells, demonstrating that signaling regulated by EN1 occurs across the D/V border. Finally, fate mapping of AER domains in these mutants showed that En1 plays a part in positioning and maintaining the two lineage borders.

Animals↗
PubMed2001

Sequence polymorphisms of the EDA and the DL genes in the patients with an X-linked and an autosomal forms of anhidrotic ectodermal dysplasia.

Oligodontia, sparse hair and deficiency of eccrine sweat glands are the features characteristic for the phenotype of the patients with anhidrotic ectodermal dysplasia (EDA). This syndrome is caused by mutations in the EDA or DL (downless) genes, encoding members of the TNF ligand and TNF receptor families, involved in the communication between the cells during embryonic life. We investigated both the coding and noncoding regions of the EDA and the DL genes in the patients exhibiting clinical symptoms of ectodermal dysplasia. Sequence analysis of the amplified fragments of the EDA gene revealed polymorphisms in introns three, four and five. The polymorphism in intron four was found in about 60% of the patients and was no more frequent than in the normal individuals. The two other polymorphisms were rare. Polymorphisms were also observed in exons 9 and 12 of the DL gene, but they did not alter the sequence of the protein product of the gene. Our results indicate that in order to accelerate screening for the mutations of the EDA gene and reduce the costs, the amplified fragments should not contain intronic sequences. However, in the case of the DL gene, where polymorphic sites are located in exons, restriction analysis with the use of appropriate enzyme should be conducted, but usually sequencing analysis could not be avoided.

Ectodermal Dysplasia↗
PubMed2001

Oligodontia is associated with extra-oral ectodermal symptoms and low whole salivary flow rates.

INTRODUCTION: Ectodermal dysplasias (EDs) involve abnormal development of hair, teeth, nails, and sweat glands. OBJECTIVES: (1) To investigate the frequency of extraoral ectodermal symptoms in persons with oligodontia (>6 congenitally missing teeth). (2) To examine whole salivary flow rates in oligodontia patients, with special emphasis on persons with EDs. SURVEY GROUP AND METHODS: Sixty-eight persons with oligodontia and 39 healthy control persons were included. All participants underwent an interview, general and oral examinations, and whole salivary secretory tests. RESULTS: Thirty-nine (57%) of the oligodontia patients had disturbances in either hair, nails and/or sweat production in addition to teeth and were classified as the ED group. The remaining 29 oligodontia patients had no obvious signs of EDs (non-ED group). The prevalences of dry skin, asthma and eczema were higher, and whole salivary secretory rates lower, in the ED group as compared to the control group. Intermediate values were observed in the non-ED group. Incisors, canines and molars were more frequently missing in the ED group than in the non-ED group, whereas no differences were observed in the frequency of missing premolars. Negative correlations between the number of missing teeth and unstimulated and chewing stimulated whole salivary secretory rates were found.

Adolescent↗
PubMed2000

Management of ectodermal dysplasia in children--an overview.

Oral rehabilitation is often difficult for the young child with ectodermal dysplasia. Most affected children require extensive dental treatment in order to restore their appearance and function. Early intervention also helps children develop a positive self-image. This paper will review the dental management and timing of different treatment modalities for children with ectodermal dysplasia.

Adolescent↗
PubMedDate not supplied

Growth analysis of a patient with ectodermal dysplasia treated with endosseous implants: a case report.

Congenital absence of multiple teeth and poorly developed alveolar ridges are associated with ectodermal dysplasia. Affected patients often require dental prosthetic treatment during their developmental years. Maxillofacial growth and development in a preadolescent female patient with ectodermal dysplasia following oral rehabilitation with maxillary and mandibular endosseous dental implants is reported. Four maxillary and 4 mandibular implants were successfully integrated and restored at 8 years of age. Growth analysis 12 years later revealed that the implants followed maxillary and mandibular growth displacement. Minor impaction of the maxillary implants was observed, and mandibular implants were affected by the mandibular growth rotation, which led to a change in implant inclination. The treatment outcome is compared to similar previously reported studies and cases.

Anodontia↗
PubMed2001

Recurrent fever and lack of tooth buds. a case of ectodermal dysplasia in a 9 months old boy.

We present the case of a 9 months old boy with an X-linked form of anhidrotic (hypohidrotic) ectodermal dysplasia. Several chest X-rays had been performed to rule out pneumonia because of recurrent episodes of high fever. The child's lack of tooth buds (hypodontia), which could be encountered on the margins of the chest X-rays, are suggestive for ectodermal dysplasia.

Anodontia↗
PubMed1995

[Ectodermal dysplasia: a heterogenic deviation].

The 'problem' of ectodermal dysplasia is discussed in conjunction with a case report and a review of the literature. In a range of hereditary disorders all ectodermal structures can be effected in a variable way: reduction of the amount of hair, sweat glands and sebaceous glands, and a complete or partial anodontia of the deciduous and permanent dentition with malformation of the erupted teeth.

Adult↗
PubMedDate not supplied

EEC syndrome (ectrodactyly-ectodermal dysplasia-clefting): a clinical case report.

The EEC syndrome (ectrodactyly-ectodermal dysplasia -clefting) is defined as a multiple congenital anomaly syndrome characterized by ectodermal dysplasia, distal limb anomaly, cleft lip/palate, and lacrimal duct anomalies. This case report reviews and presents clinical symptoms and treatment of the EEC syndrome.

Child↗
PubMedDate not supplied

The role of prosthodontists in habilitation and rehabilitation in rare disorders: the ectodermal dysplasia experience.

PURPOSE: The aim of this article is to highlight the strong worldwide trend of enhancing the opportunities for individuals with rare disorders to get adequate information and treatment. Hypohidrotic ectodermal dysplasia is used as an example. MATERIALS AND METHODS: The development in recent years of databases for rare disorders, the establishment of resource centers in dentistry, and the strong movement of user influence by the formation of patient organizations and exchange of information through the Internet are described. Reference is made to the recent Scandinavian consensus conference on ectodermal dysplasia. RESULTS: Motives for the centralization of treatment planning to resource centers for persons with rare disorders, the compilation of treatment outcomes in databases, and the advocation of a multidisciplinary team approach in dentistry are presented. CONCLUSION: The prosthodontic profession has a key position in the habilitation and rehabilitation of many different diagnoses of rare disorders.

Adolescent↗