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PubMed2004

Neural induction in Xenopus: requirement for ectodermal and endomesodermal signals via Chordin, Noggin, beta-Catenin, and Cerberus.

The origin of the signals that induce the differentiation of the central nervous system (CNS) is a long-standing question in vertebrate embryology. Here we show that Xenopus neural induction starts earlier than previously thought, at the blastula stage, and requires the combined activity of two distinct signaling centers. One is the well-known Nieuwkoop center, located in dorsal-vegetal cells, which expresses Nodal-related endomesodermal inducers. The other is a blastula Chordin- and Noggin-expressing (BCNE) center located in dorsal animal cells that contains both prospective neuroectoderm and Spemann organizer precursor cells. Both centers are downstream of the early beta-Catenin signal. Molecular analyses demonstrated that the BCNE center was distinct from the Nieuwkoop center, and that the Nieuwkoop center expressed the secreted protein Cerberus (Cer). We found that explanted blastula dorsal animal cap cells that have not yet contacted a mesodermal substratum can, when cultured in saline solution, express definitive neural markers and differentiate histologically into CNS tissue. Transplantation experiments showed that the BCNE region was required for brain formation, even though it lacked CNS-inducing activity when transplanted ventrally. Cell-lineage studies demonstrated that BCNE cells give rise to a large part of the brain and retina and, in more posterior regions of the embryo, to floor plate and notochord. Loss-of-function experiments with antisense morpholino oligos (MO) showed that the CNS that forms in mesoderm-less Xenopus embryos (generated by injection with Cerberus-Short [CerS] mRNA) required Chordin (Chd), Noggin (Nog), and their upstream regulator beta-Catenin. When mesoderm involution was prevented in dorsal marginal-zone explants, the anterior neural tissue formed in ectoderm was derived from BCNE cells and had a complete requirement for Chd. By injecting Chd morpholino oligos (Chd-MO) into prospective neuroectoderm and Cerberus morpholino oligos (Cer-MO) into prospective endomesoderm at the 8-cell stage, we showed that both layers cooperate in CNS formation. The results suggest a model for neural induction in Xenopus in which an early blastula beta-Catenin signal predisposes the prospective neuroectoderm to neural induction by endomesodermal signals emanating from Spemann's organizer.

Animals↗
PubMed1976

Considerations in dental treatment of children with ectodermal dysplasia.

Characteristics of ectodermal dysplasia and agammaglobulinemia have been presented and a case in a 6 1/2-year-old boy having both genetic diseases is reported. The child had been mocked by his peers at school. A maxillary overlay denture and mandibular cast denture were constructed using a Baker bar and cast gold thimble crowns. The parents reported that the patients personality changed favorably after dental treatment was completed. He is now 13 years of age, has had his dentures replaced once, and enjous a rather active life with the aid of antibiotics and supplemental globulins.

Agammaglobulinemia↗
PubMed2005

A costs analysis of dental treatment for ectodermal dysplasia.

OVERVIEW: Dental treatment modalities for ectodermal dysplasia (ED) vary markedly depending on the clinical manifestations, but to date there have been no studies exploring the potential economic impact of ED. On the basis of anecdotal and clinical reports, the authors postulate that costs of dental treatment for this condition can have a substantial financial impact on patients and their families. OBJECTIVE: The purpose of the authors' pilot study was to develop an economic model for various treatment modalities for ED with severe hypodontia. METHODS: The authors first used a comprehensive review of the literature and expert consensus to establish a treatment modalities model for ED. Next, they completed chart reviews to validate the model with sample treatment and costs information. Using these data, they then constructed a model of treatment options and associated costs. RESULTS: The sample included 24 patients with ED who had severe hypodontia. Forty-two percent were female; patients' ages ranged from 4 years, 11 months to 31 years, 1 month. Forty-two percent had dental insurance coverage, while more than one-half paid for services out of pocket. An estimated 84 percent had undergone prosthodontic treatment, 37 percent orthodontic treatment and 19 percent implant surgery. Depending on the age of the patient and types of dental treatment, there was a broad variation in costs. This ranged from $2,038 to $3,298 for those who had received prosthodontic treatment only; it ranged from $12,632 to $41,146 for those who had received a combination of prosthodontic, orthodontic and implant treatment. CONCLUSIONS: Dental treatment for ED had a marked financial impact on patients and their families and varied depending on the type and duration of treatment.

Adolescent↗
PubMed2004

Variations in facial soft tissues of italian individuals with ectodermal dysplasia.

OBJECTIVE: To supply quantitative information about the facial soft tissues of patients with ectodermal dysplasia (ED). DESIGN, SETTING, AND PATIENTS: The three-dimensional coordinates of soft tissue facial landmarks were obtained using an electromagnetic digitizer in 20 Caucasian patients with ED (11 male and 9 female patients aged 7 to 41 years) and 391 healthy controls (163 female and 228 male subjects of comparable ages). From the landmarks, 15 facial dimensions and two angles were calculated. Data were compared with those collected in healthy individuals by computing z-scores. Two summary anthropometric measurements for quantifying craniofacial variations were assessed in both the patients and reference subjects: the mean z-score (an index of overall facial size) and its standard deviation, called the craniofacial variability index (an index of facial harmony). RESULTS: In patients with ED, a large variability was found for both the mean z-score and the craniofacial variability index. On average, the patients had a somewhat smaller facial size than the normal individuals, with a global disharmonious appearance. Overall, 70% of patients fell outside the quantitative definitions for normal facial size, harmony or both. When examining the distribution of the single z-scores of the 17 selected measurements, variable patterns were found, without consistent effects of age or sex. CONCLUSIONS: The facial soft tissue structures of patients with ED differed both in size and shape from those of normal controls of the same age, sex, and ethnic group.

Adolescent↗
PubMed2001

Clinical management of ectodermal dysplasia with long term follow up: two case reports.

The present study describes the characteristics and clinical management of two patients with ectodermal dysplasia with long term follow-up. Dental treatments depend on the severity of disorder, therefore, treatment varies according to the age, growth and development of the stomatognathic system of the patient. It is important that the patient and dentist understand continued monitoring for dental problems is necessary. This provides improved aesthetics, function and emotional development.

Adult↗
PubMed2001

Hypohidrotic ectodermal dysplasia: dental, clinical, genetic and dermatoglyphic findings of three cases.

Patients with hypohidrotic ectodermal dysplasia (HED) are characterized by the clinical manifestations of hypodontia, hypohidrosis, hypotrichosis and a highly characteristic facial physiognomy. This disorder is inherited as an X-linked trait. This report presents three cases with HED in which the clinical evaluation (intraoral and radiological), genetic findings and SEM examination of hair. Boys 6 to 14 year old and a 11 year old girl were referred to the Marmara University, Faculty of Dentistry, complaining of oligodontia in the maxillary and mandibular arches and delay in eruption of other teeth. Peg-shaped teeth have been observed. The dermatoglyphs of the patients were striking. SEM examination of hair demonstrated a distinctly abnormal longitudinal grooving along the entire length of each hair and a desquamation of the surface cuticles. The treatment was planned in a multidisciplinary odontological group involving pediatric dentistry, orthodontics, prosthodontics and oral surgery and maxillofacial radiology of future dental habilitation. A specially designed overdenture, a removable prosthesis and osseointegrated implants were constructed. Periodic recall visits were advised, to monitor the dentures and implants during periods of growth and development, and eruption of the permanent teeth.

Anodontia↗
PubMed1997

Apical ectodermal ridge-dependent expression of the chick 67 kDa laminin binding protein gene (cLbp) in developing limb bud.

Apical ectodermal ridge (AER)-mesoderm interaction is important for morphogenesis in the developing chick limb bud. Genes whose expression is dependent upon the presence of AER, are likely to play important roles in the AER-mesoderm interaction. We report here the gene expression pattern of the chick homolog of the 67 kDa laminin binding protein (LBP), which is a non-integrin laminin receptor whose function relates to cell attachment, spreading, and polarization. Northern analysis showed that a single 1.4 kb transcript exists in stage 20 limb buds and which is dramatically reduced 24 hr after removal of AER. In situ hybridization analysis revealed that the chick 67 kDa laminin binding protein gene (cLbp) was expressed in the mesodermal region overlapping the Msx1-expressing domain and in the AER in early stage limb buds. Expression in the mesoderm was gradually restricted to the distal region underneath the AER as development proceeds. The expression in the limb mesoderm could be induced by local application of FGF-2 which could thus mimic the AER functions. These results indicated that the expression of cLbp depends on AER signals and that the 67 kDa non-integrin receptor binding to laminin plays a role in the AER-mesoderm interaction.

Animals↗
PubMed1996

Ectrodactyly, ectodermal dysplasia, and cleft lip syndrome. Case report.

We describe five sporadic cases of the EEC syndrome (ectrodactyly, ectodermal dysplasia, and cleft lip). There are only a few reports of the syndrome being inherited in Japan. Two of the five patients had genitourinary anomalies and one mental retardation. Four of the five patients had clefts in the primary and secondary palate. All had deformities of the hands, polydactyly, syndactyly, and camptodactyly. Syndactyly could be a component of the syndrome.

Abnormalities, Multiple↗
PubMed1989

Association of ectodermal dysplasia, ectrodactyly and macular dystrophy: EEM syndrome (case report).

The authors reported a 41-year-old female patient with EEM (ectodermal dysplasia, ectrodactyly and macular dystrophy) syndrome with hypotrichosis, teeth anomaly, split hand complex and retinal changes with prominent pigmentations located in the posterior pole of the retina. Retinal degeneration had shown minimal progression during 11 years. A longer follow-up period was necessary to make a definite diagnosis of these fundus changes. This is an isolated case born from a consanguineous marriage.

Adult↗
PubMed1997

Two families with dyshidrotic ectodermal dysplasia associated with ingrowth of corneal vessels, limbal hair growth, and Bitôt-like conjunctival anomalies.

Five cases from two unrelated families with a hitherto unknown combination of dyshidrotic ectodermal dysplasia with corneal vessel ingrowth, limbal hair follicles with hairs, and Bitôt-like spots in the conjunctiva are described. The corneal lesions were slowly progressive. In one pedigree, autosomal recessive inheritance is most likely, in the other there is uncertainty about the mode of inheritance. According to the criteria of Pinheiro and Freire-Maya, the mentioned cases can be classified into subgroup 1-2-4. The cases under investigation showed no palisades of Vogt like those seen in aniridia and after radiation therapy. We also found an absence of goblet cells in the affected individuals. We suggest therefore that the corneal and conjunctival anomalies are possibly caused by a stem cell disorder.

Adult↗
PubMed1996

Ectodermal dysplasia with hypodontia in a set of Nigerian twins--a case report.

A set of Nigerian male twins with features of ectodermal dysplasia born to apparently normal parents are presented. The main findings in both children were hypodontia, abnormally shaped teeth and hypotrichosis. The general treatment modalities of the condition, especially in a tropical environment, are discussed.

Abnormalities, Multiple↗
PubMed1999

[Anesthetic management of a patient with hypohidrotic ectodermal dysplasia].

Hypohidrotic ectodermal dysplasia (HED) is a rare congenital anomaly complex characterized by hypodontia, hypotricosis and hypohidrosis. There have been only a few reports of anesthetic management of patients with HED. We managed a 20-year-old man with HED, who underwent debridement and skin grafting under epidural anesthesia, without untoward events. Potential problems in anesthetic management of patients with HED are also discussed.

Adult↗
PubMed1999

A girl with ectodermal dysplasia, choanal atresia and polysyndactyly.

We present a 3-year-old child with clinical features of ectodermal dysplasia comprising sparse hair, dystrophic and ridged nails and bilateral obstruction of the nasolacrimal ducts. Additional findings were unilateral choanal atresia, bilateral syndactyly of the feet and polydactyly. We discuss the differential diagnosis of these clinical findings.

Abnormalities, Multiple↗
PubMed1975

The syndrome of ectrodactyly, ectodermal dysplasia, and clefting (EEC).

Early recognition of ectrodactyly, ectodermal dysplasia, and clefting of the lip and palate as a syndrome could result in more beneficial treatment for the patient. Patients with the EEC syndrome often have ocular and auricular deficiencies that progressively become more severe. These patients are often seen first by cleft palate teams who make the diagnosis. The patient's dental status requires frequent evaluation after corrective procedures for cleft lip and cleft palate.

Adolescent↗
PubMedDate not supplied

[Ectodermal dysplasia. Clinical case].

A case of ectodermal dysplasia with anodontia, and severe hypotrichosis is described. Clinical aspects and differential diagnosis with Huth-chinson-Gildorf syndrome are also discussed.

Anodontia↗
PubMed1999

Hypohidrotic ectodermal dysplasia--a case report.

A case presented here is that of a nine year old male patient with total anodontia. Findings of this case as regards to the orofacial, radiographic and other general manifestations were suggestive of hypohidrotic ectodermal dysplasia. The dental problems were best managed by prosthetic replacement of dentition taking into consideration a design which would provide adequate relief for the preservation of the ridges which were thin and underdeveloped to the absence of teeth.

Anodontia↗
PubMed2000

Oral rehabilitation of a hypohidrotic ectodermal dysplasia patient: a 6-year follow-up.

This case report describes the oral rehabilitation of a female child with hypohidrotic ectodermal dysplasia over a 6-year time period. It demonstrates the need for periodic modification and replacement of a prosthesis, an orthodontic appliance, and a gingivoplasty. Although the initial treatment plan was considered to be a compromise due to limited cooperation, an improvement was observed in the patient's social behavior as a consequence of her dental treatment. The effects of unavoidable changes in the dental team over 6 years are also discussed.

Anodontia↗