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Taurodontia Absent Teeth Sparse Hair

Taurodontia - Absent Teeth - Sparse Hair Syndrome


Presentation

  • Treatment is based on the signs and symptoms present in each person. MalaCards based summary : Teeth, Congenital Absence of, with Taurodontia and Sparse Hair, is also known as taurodontia, absent teeth, sparse hair syndrome.[malacards.org]
  • Treatment is based on the signs and symptoms present in each person. [1] [2] [3] Last updated: 10/13/2016 This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person.[rarediseases.info.nih.gov]
  • The enamel was present only in some areas of the tooth and absent in some. Generalized attrition was observed on the incisal and occlusal surfaces with open contacts.[ijdr.in]
  • Incisors and canines are often conical-shaped while primarily second molars, if present, are mostly affected by taurodontism.[amsdottorato.unibo.it]
  • Bilateral postaxial polydactyly of hands were present (Figure 2a). Hair was sparse especially in the eyebrows (Figure3). The left testis was not fully descended.[omicsonline.org]
Nail Abnormality
  • 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]
Heat Intolerance
  • 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]
Physician
  • 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]
Selective Tooth Agenesis
  • 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]
Small Teeth
  • Synonyms ODDD ODD Syndrome Oculotdentoosseous dysplasias (ODOD) ODDD Syndrome is a Rare Genetic Condition Characterized by A typical facial appearance Variable eye, tooth and finger involvement Teeth Enamel hypoplasia Selective missing teeth Small teeth[nfed.org]
  • Missing teeth and small teeth often present together, and so masking conical or similarly distinctive teeth with composite is strongly advised.[pocketdentistry.com]
  • Possible teeth abnormalities include congenitally missing teeth, conically shaped teeth, and small teeth (microdontia).[thefreelibrary.com]
Tooth Loss
  • 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]
Sparse Hair
  • Prevention - Taurodontia absent teeth sparse hair Not supplied.[checkorphan.org]
  • More Symptoms of Taurodontia, absent teeth, sparse hair syndrome » Causes See also causal information: Causes of Taurodontia Causes of Sparse hair Similar Topic Articles Taurodontia Absent teeth Sparse hair Names and Terminology References Source: GARD[familydiagnosis.com]
  • 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]
Sparse Hair
  • Prevention - Taurodontia absent teeth sparse hair Not supplied.[checkorphan.org]
  • More Symptoms of Taurodontia, absent teeth, sparse hair syndrome » Causes See also causal information: Causes of Taurodontia Causes of Sparse hair Similar Topic Articles Taurodontia Absent teeth Sparse hair Names and Terminology References Source: GARD[familydiagnosis.com]
  • 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]
Alopecia
  • 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]
Alopecia
  • 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]
Dermatitis
  • 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]
Facial Hemihypertrophy
  • Facial hemihypertrophy, otodental syndrome. Constriction of CEJ and elongated pulp chamber with apically displaced pulp. HYPODONTIA associated. Elongated pulp chamber with short stunted roots. Failure of Hertwig's epithelial root sheath.[brainscape.com]
Suggestibility
  • 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]
Seizure
  • 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]

Treatment

  • Stay at the forefront of your field with updated treatment methods throughout, as well as an increased focus on patients with skin of color.[books.google.com]
  • Treatment is based on the signs and symptoms present in each person. MalaCards based summary : Teeth, Congenital Absence of, with Taurodontia and Sparse Hair, is also known as taurodontia, absent teeth, sparse hair syndrome.[malacards.org]
  • You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.[rarediseases.info.nih.gov]
  • Treatment - Taurodontia absent teeth sparse hair Not supplied. Resources - Taurodontia absent teeth sparse hair Not supplied.[checkorphan.org]

Prognosis

  • 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]
  • Treatment and prognosis  Done for aesthetic purpose  Peg laterals- full size porcelain crowns. 14. In number of teeth 15.[slideshare.net]
  • Diagnosis is also possible using chorionic villi or amniotic fluid using linkedâ microsatellite markers if a previously affected sibling has been identified [ 9 ].The prognosis of EvCsyndrome is linked to the presence of congenital heart defects and respiratory[omicsonline.org]
  • ED per se has not only a poor prognosis, but it also needs constant care for temperature and other problems associated with this condition. Genetic counseling is recommended for families who have family history of ED.[digitmedicine.com]
  • Thus, individuals with bi-allelic WNT10A mutations may be recognized early in life by combining information of both dentitions as well as other ectodermal features of importance for diagnosis, prognosis and counselling.[bmcmedgenet.biomedcentral.com]

Etiology

  • This article reports rare three cases of congenitally missing permanent teeth with over-retained deciduous dentition and review the literature citing its etiology, clinical implications, and management.[jidonline.com]
  • Taurodontism – A review on its etiology, prevalence and clinical considerations. J Clin Exp Dent 2010;2:187-90. 15. Rathee M, Mahesh G, Arun K, Richa M, Renu K.[jioh.org]
  • Etiology unknown. Possibly familial, common for more than 1 tooth. MN primary molars are the most common. Should exfoliate normally. Want to monitor and not extract as to not damage the successors. Minor eruption variation is normal.[brainscape.com]
  • Hypodontia–a retrospective review of prevalence and etiology. Part I. Quintessence Int 2005 ; 36 : 263 – 70 . 18. Lexner MO , B ardow A, Hertz JM, e t al. A nomalies of tooth formation in hypohidrotic ectodermal dysplasia.[iedn.org]

Pathophysiology

  • Another pathophysiological method seen in the background of PSS is that of meagre craft vasculitis that can less commonly muddle PSS but all but certainly accounts benefit of some of the neurological manifestations, in particular mononeuropathy multiplex[sarcomacancer.org]

Prevention

  • Prevention - Taurodontia absent teeth sparse hair Not supplied.[checkorphan.org]
  • The goal of the treatment is aesthetic, preventive and restorative. Bonding may be done for aesthetic reasons. Crowns can restore the structure and function of the teeth as well as prevent further attrition.[thehealthsite.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]
  • […] people with hypodontia and so prevention of dental disease is paramount.[nature.com]
  • Parents were counseled regarding the disease and preventive care needed.[digitmedicine.com]

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