Lastly there is consideration of the role of the paediatric dentist in interdisciplinary management of the affected child and adolescent patient. [nature.com]

The dentist must bear in mind the high prevalence of cardiac defects in these patients and implement established procedures for the prevention of bacterial endocarditis when necessary. [omicsonline.org]

A dentist can identify and diagnose the condition by the clinical signs and symptoms supported with a history of causative factor. Radiographs may be used to rule out other conditions of the teeth. [thehealthsite.com]

A team approach that includes input from a pediatric dentist, an orthodontist, a prosthodontist, and an oral and maxillofacial surgeon is necessary for a successful outcome. [amsdottorato.unibo.it]

Dentists while encountering with such patients with oligodontia, a detailed case history, physical examination to rule out syndromic form of oligodontia, and radiographs should be done to diagnose the case. [jioh.org]

Paraparesis (partial paralysis of the lower extremities) Quadriparesis (Weakness of all four limbs, both arms and both legs) Seizures Spasticity (unusual “tightness”, stiffness, or “pull” of muscles.) [nfed.org]

Teeth are weak and prone to rapid wear and tear. Early loss of enamel due to defective dentin causes the tooth to be sensitive to temperature changes. [thehealthsite.com]

[…] had been reported by Seow and Lai. [14] Oligodontia in many patients has been reported to have a strong impact on declining the level of confidence, especially in high schoolchildren and at workplace, due to unsatisfied appearance of smile owing to weak [jioh.org]

Oculodentodigitaldysplasia Micropthalmos, microcornea, epicanthal folds, fine sparse hair, enamel hypoplasia, MN with wide alveolar ridge, syndactyly of fingers and toes, muscle weakness, nystagmus. [brainscape.com]

Treatment & prognosis  Enamel pearl – area of weak point of periodontal attachment.  Oral hygiene maintained to prevent loss of periodontal support.  Flattening / removal of enamel & furcation plasty. 80. [slideshare.net]

Nails: Most people with Ectodermal Dysplasia do not have nail abnormalities, although the nails are frequently dry and rough. [dentalimplants-usa.com]

The clinical manifestations observed were- dry skin(94.74%); scaly skin(42.11%); sparse hair on scalp, eyebrows and eyelashes(100%); frontal bossing(63.18%); saddle nose (57.89%); hypertelorism (47.37%); nail abnormality(52.63%); normal sweat glands(21.05% [jnsbm.org]

Abnormal number of teeth Dystrophic fingernails Renal insufficiency Dystrophic toenail Soft skin Taurodontia Non-midline cleft lip Protruding ear Prominent supraorbital ridges Brittle hair Abnormality of the voice Dysphonia Hoarse voice Abnormality of [mendelian.co]

Because this condition is relatively benign, people are treated only for the correction of their teeth and little attention is given to the nail abnormalities. [thefreelibrary.com]

abnormalities - - - - - 2 Dry skin - 6 No. of affected ectodermal appendages 3 3 2 3 2 3 4 mean 2.9 Additional ectodermal abnormalities Ectodermal symptoms other than tooth agenesis were in some cases unnoticed, neglected or considered normal variants [bmcmedgenet.biomedcentral.com]

The clinical features include sparse, fine hair, missing or conical-shaped teeth, decreased sweat and mucous glands, hypoplastic skin, and heat intolerance with exercise or increased ambient temperature. [amsdottorato.unibo.it]

Heat intolerance at increased ambient temperature or during exercise might rise high fever, which is one of the most important and dangerous aspects of patients affected by HED. 3 6 Dental abnormalities are the most common patients complaint; complete [iedn.org]

Patients with Fever and Sparse scalp hair. may also develop some of the following symptoms: Uncommon Symptoms - Between 30% and 50% cases Intellectual disability Microcephaly Sparse and thin eyebrow Conical tooth Fine hair Dry skin Hypodontia Seizures Heat [mendelian.co]

X-linked hypohidrotic ectodermal dysplasia is characterized in males by thin sparse hair, dry skin, absence of sweating (and therefore heat intolerance), and multiple missing teeth. [pocketdentistry.com]

In-Depth Information The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. [rarediseases.info.nih.gov]

Miscellaneous Variable clinical symptoms Cardiac features are observed in 3% of cases Neurologic features have been diagnosed in 30% of cases Diagnosing ODDD Syndrome A physician can diagnose ODD syndrome on the basis of physical features. [nfed.org]

Furthermore, the dentist will play an important role in the integral approach regarding health care needs of the child, in collaboration with the physician and the parents. Good dental health goes far beyond having good teeth. [thefreelibrary.com]

tooth agenesis. [4] According to different authors, the frequency of hypodontia is 1-10% and oligodontia 0.1-0.9%. [5], [6] Anodontia occurs very rarely (17 cases were described over the last 50 years). [7] Oligodontia may occur as a part of a genetic [jidonline.com]

Collegioum Antropol 1998; 22 : 291–298. 11 Vastardis H, Karimbux N, Guthua SW, Seidman JG, Seidman CE A human MSZ1 homeodomain missence mutation causes selective tooth agenesis. [nature.com]

The WNT10A gene in ectodermal dysplasias and selective tooth agenesis. Am J Med Genet A. 2014;164A:2455–60. View Article PubMed Google Scholar Tziotzios C, Petrof G, Liu L, Verma A, Wedgeworth EK, Mellerio JE, McGrath JA. [bmcmedgenet.biomedcentral.com]

In anterior dentition, how does premature tooth loss affect successor eruption? Less than 3 years, delayed. More than 5 years, accelerated. In posterior dentition, how does premature tooth loss affect successor eruption? Less than 7 years, delayed. [brainscape.com]

Emphasis should be placed on dental care aiming at prevention of tooth loss from caries or periodontal disease. During the extraction, elevators should be used with caution so as to protect the adjacent tooth from inadvertent mobilization. [indjos.com]

Prevention - Taurodontia absent teeth sparse hair Not supplied. [checkorphan.org]

MalaCards based summary : Teeth, Congenital Absence of, with Taurodontia and Sparse Hair, is also known as taurodontia, absent teeth, sparse hair syndrome. Affiliated tissues include skin, and related phenotypes are taurodontia and sparse hair [malacards.org]

Name Teeth, Congenital Absence Of, With Taurodontia And Sparse Hair Synonyms TAURODONTIA, ABSENT TEETH, AND SPARSE HAIR Classification developmental, genetic, odontological, skin Phenotypes Autosomal recessive inheritance ; Oligodontia ; Sparse hair ; [mousephenotype.org]

Title Other Names: Teeth, congenital absence of, with taurodontia and sparse hair Categories: Taurodontia, absent teeth, sparse hair syndrome is a rare condition that, as the name suggests, is primarily characterized by malformations of the primary and [rarediseases.info.nih.gov]

Baby had large forehead, flat nasal bridge, absence of eye lashes and eye brows, thin and hypo-pigmented skin, abnormality of nails, thin and sparse hairs, absent tears, and sweating [Figure 1]. [digitmedicine.com]

Sweat Glands: Diminished or absent sweating is a common problem. The sweat glands are absent, reduced in number, or may not function normally. Reduced sweating may result in very high fevers, because the body regulates its temperature by sweating. [dentalimplants-usa.com]

The first step in the algorithm for making a specific diagnosis of an ED is to determine the presence of sweating (hidrotic) or absence of sweating (hypohidrotic/anhidrotic). [mhmedical.com]

[…] to lack of sweat glands (anhidrosis or hypohidrosis). [digitmedicine.com]

[…] due to lack of sweat glands (anhidrosis or hypohidrosis) this causes intolerance to heat and over heating can rarely be a cause of death fingernails and toenails may be absent, thick, abnormally shaped, discoloured, ridged, slow growing, or brittle lack [radiopaedia.org]

Vadodara, Gujarat India Source of Support: None, Conflict of Interest: None DOI: 10.4103/0976-9668.117012 Abstract Background: Ectodermal dysplasia (ED) is a group of rare, inherited disorders characterized by sparse hair, missing teeth and inability to sweat [jnsbm.org]

Researchers estimate that about 1 per 100 diagnoses of alopecia totalis are incorrect. See introduction to alopecia totalis. [checkorphan.org]

Ectodermal dysplasia alopecia preaxial polydactyly 0 *Alopecia *Ectodermal Dysplasia *Polydactyly *Facies. Ectodermal dysplasia adrenal cyst 0 *Adrenal Gland Diseases *Cysts *Ectodermal Dysplasia. [reference.md]

Clinical differential considerations include: alopecia areata aplasia cutis congenita focal dermal hypoplasia syndrome incontinentia pigmenti Naegeli-Franceschetti-Jadassohn syndrome pachyonychia congenita Promoted articles (advertising) [radiopaedia.org]

[…] contractures Partial agenesis of the corpus callosum Cortical gyral simplification Femoral bowing Preaxial polydactyly Diabetes insipidus Agenesis of cerebellar vermis Colpocephaly Absent facial hair Epidermal acanthosis Palmar hyperkeratosis Patchy alopecia [mendelian.co]

Patients with severe alopecia can wear wigs to improve their appearance. Use of topical minoxidil with or without a topical tretinoin has been shown to improve hair growth in a small number of patients. [digitmedicine.com]

Some of the overlooked hair/scalp conditions include cicatrial alopecia, traction folliculitis and sebhorrheic dermatitis Prognosis - Taurodontia absent teeth sparse hair Not supplied. Treatment - Taurodontia absent teeth sparse hair Not supplied. [checkorphan.org]

Familial, autosomal recessive Claire Smith 00000164 0000000191 Atopic dermatitis (eyelids, the arms and legs) and tangled hair since early childhood. Repeated dental problems, most recently a gum abscess. [dna2.leeds.ac.uk]

Xerosis or eczematous dermatitis should be treated with topical emollients. Patients with severe alopecia can wear wigs to improve their appearance. [digitmedicine.com]

[…] excessively brittle abnormal peg-shaped or pointed teeth, particularly the upper incisors and cuspids which are typically conical missing teeth (anodontia or hypodontia) and taurodontism of deciduous molars inability to sweat due to lack of sweat glands (anhidrosis [radiopaedia.org]

[…] glands) without other systemic findings.Hypohidrotic, or anhidrotic, ectodermal dysplasia (HED/EDA) is characterized by a triad of signs comprising sparse hair (hypotrichosis), abnormal or missing teeth (anodontia or hypodontia), and inability to sweat (anhidrosis [mendelian.co]

Babies with anhidrosis/hypohidrosis should be advised air conditioning at home, school, and work, frequent consumption of cool liquids, and wear cool clothing to maintain hydration and temperature. [digitmedicine.com]

NIH Rare Diseases : 54 Taurodontia, absent teeth, sparse hair syndrome is a rare condition that, as the name suggests, is primarily characterized by malformations of the primary and/or secondary molars (taurodontia); the absence of several teeth; and [malacards.org]

Title Other Names: Teeth, congenital absence of, with taurodontia and sparse hair Categories: Taurodontia, absent teeth, sparse hair syndrome is a rare condition that, as the name suggests, is primarily characterized by malformations of the primary and [rarediseases.info.nih.gov]

SELECTED GENES FOR YOUR SLICE SUGGESTED CUSTOM SLICES Customize below OR enter Suggested Slice ID on printed requisition form (e.g. 706 XomeDx Slice - Slice ID: CS-Albinism). [genedx.com]

We have called the condition Recessive Incisor Hypodontia (RIH) and suggest that it belongs to the Finnish Disease Heritage, the enrichment of some 40 rare disorders. [jmg.bmj.com]

Freire-Maia and Pinheiro laid claim to more than 150 ectodermal disorders in their most recent compilation. 2 Several other classification schemes have been suggested. [mhmedical.com]

Atypical absence seizures Esodeviation Happy demeanor Broad eyebrow Drowsiness Deep plantar creases Prominent nasal tip Broad columella Bruxism Low hanging columella Tracheal stenosis Broad hallux phalanx Chronic constipation Large earlobe Atrophic, [mendelian.co]

Paraparesis (partial paralysis of the lower extremities) Quadriparesis (Weakness of all four limbs, both arms and both legs) Seizures Spasticity (unusual “tightness”, stiffness, or “pull” of muscles.) [nfed.org]