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Sample records for anodontia

  1. A case report of anodontia

    Energy Technology Data Exchange (ETDEWEB)

    Park, Tae Won; Kwon, Hyuk Choon [School of Dentistry, Seoul National University, Seoul (Korea, Republic of)

    1971-11-15

    Authors observed a rare case of total anodontia. The patient was 4 years old boy whose roentgenologic aspects of bone pattern was decreased in trabeculation and there was not tooth and toothlike substances. It was supposed that the cause of this disease was endocrine dysfunctions in his childhood.

  2. Ectodermal Dysplasia with Anodontia: A Report of Two Cases

    Science.gov (United States)

    Bani, Mehmet; Tezkirecioglu, Ali Melih; Akal, Nese; Tuzuner, Tamer

    2010-01-01

    Ectodermal dysplasia is a hereditary disorder that occurs as a consequence of disturbances in the ectoderm of the developing embryo. The triad of nail dystrophy, alopecia or hypotrichosis and palmoplantar hyperkeratosis is usually accompanied by a lack of sweat glands and a partial or complete absence of primary and/or permanent dentition. Two case reports illustrating the prosthetic rehabilitation of 2 young boys with anhidrotic ectodermal dysplasia associated with severe anodontia are presented. Since the oral rehabilitation of these cases is often difficult; particularly in pediatric patients, treatment should be administered by a multidisciplinary team involving pediatric dentistry, orthodontics, prosthodontics and oral-maxillofacial surgery. PMID:20396456

  3. Hypohidrotic ectodermal dysplasia with anodontia: A rare case-rehabilitation by prosthetic management

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    M Naveen Kumar

    2012-01-01

    Full Text Available Ectodermal dysplasia is a hereditary disorder characterized by developmental dystrophies of ectodermal derivatives- It is characterized by triad of signs comprising sparse hair, abnormal or missing teeth and inability to sweat. This case of 12-year-old boy with hypohidrotic ectodermal dysplasia and complete anodontia of both primary and permanent dentition is presented. Owing to the need for treatment at an early age for anodontia and due to the ill-formed maxillary and mandibular residual ridges, the prosthetic management can be difficult. Complete dentures with soft liners and hollow maxillary denture were provided to encourage normal psychological development and to improve the function of the stomatognathic system.

  4. “Maxillary lateral incisor partial anodontia sequence”: a clinical entity with epigenetic origin

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    Consolaro, Alberto; Cardoso, Maurício Almeida; Consolaro, Renata Bianco

    2017-01-01

    ABSTRACT The relationship between maxillary lateral incisor anodontia and the palatal displacement of unerupted maxillary canines cannot be considered as a multiple tooth abnormality with defined genetic etiology in order to be regarded as a “syndrome”. Neither were the involved genes identified and located in the human genome, nor was it presumed on which chromosome the responsible gene would be located. The palatal maxillary canine displacement in cases of partial anodontia of the maxillary lateral incisor is potentially associated with environmental changes caused by its absence in its place of formation and eruption, which would characterize an epigenetic etiology. The lack of the maxillary lateral incisor in the canine region means removing one of the reference guides for the eruptive trajectory of the maxillary canine, which would therefore, not erupt and /or impact on the palate. Consequently, and in sequence, it would lead to malocclusion, maxillary atresia, transposition, prolonged retention of the deciduous canine and resorption in the neighboring teeth. Thus, we can say that we are dealing with a set of anomalies and multiple sequential changes known as sequential development anomalies or, simply, sequence. Once the epigenetics and sequential condition is accepted for this clinical picture, it could be called “Maxillary Lateral Incisor Partial Anodontia Sequence.” PMID:29364376

  5. “Maxillary lateral incisor partial anodontia sequence”: a clinical entity with epigenetic origin

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    Alberto Consolaro

    Full Text Available ABSTRACT The relationship between maxillary lateral incisor anodontia and the palatal displacement of unerupted maxillary canines cannot be considered as a multiple tooth abnormality with defined genetic etiology in order to be regarded as a “syndrome”. Neither were the involved genes identified and located in the human genome, nor was it presumed on which chromosome the responsible gene would be located. The palatal maxillary canine displacement in cases of partial anodontia of the maxillary lateral incisor is potentially associated with environmental changes caused by its absence in its place of formation and eruption, which would characterize an epigenetic etiology. The lack of the maxillary lateral incisor in the canine region means removing one of the reference guides for the eruptive trajectory of the maxillary canine, which would therefore, not erupt and /or impact on the palate. Consequently, and in sequence, it would lead to malocclusion, maxillary atresia, transposition, prolonged retention of the deciduous canine and resorption in the neighboring teeth. Thus, we can say that we are dealing with a set of anomalies and multiple sequential changes known as sequential development anomalies or, simply, sequence. Once the epigenetics and sequential condition is accepted for this clinical picture, it could be called “Maxillary Lateral Incisor Partial Anodontia Sequence.”

  6. Early functional, esthetic, and psychological rehabilitation of preschool child with nonsyndromic oligodontia and anodontia in mixed dentition stage through conservative systematic approach: A case report with 5-year follow-up

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    Manu Rathee

    2016-01-01

    Full Text Available Missing teeth are a common developmental abnormality in humans. It may manifest as absence of varying numbers of primary and/or secondary teeth. Early treatment and follow-up are the key to successful rehabilitation of young patients with congenitally missing teeth. It is critical that oral rehabilitation is started early to maintain and correct the oral functions. Mucosa borne removable prostheses are the commonly selected treatment options for the young patients who present with oligodontia or anodontia. This clinical report describes esthetic, functional, and psychological rehabilitation of a young boy with severe oligodontia in maxillary arch and anodontia in mandibular arch. The individualized conservative graded approach in prosthetic rehabilitation with removable acrylic prosthesis helped to achieve esthetics, functionality, and psychological benefits.

  7. Early functional, esthetic, and psychological rehabilitation of preschool child with nonsyndromic oligodontia and anodontia in mixed dentition stage through conservative systematic approach: A case report with 5-year follow-up.

    Science.gov (United States)

    Rathee, Manu; Malik, Poonam; Dua, Madhuri; Yadav, Vikas

    2016-01-01

    Missing teeth are a common developmental abnormality in humans. It may manifest as absence of varying numbers of primary and/or secondary teeth. Early treatment and follow-up are the key to successful rehabilitation of young patients with congenitally missing teeth. It is critical that oral rehabilitation is started early to maintain and correct the oral functions. Mucosa borne removable prostheses are the commonly selected treatment options for the young patients who present with oligodontia or anodontia. This clinical report describes esthetic, functional, and psychological rehabilitation of a young boy with severe oligodontia in maxillary arch and anodontia in mandibular arch. The individualized conservative graded approach in prosthetic rehabilitation with removable acrylic prosthesis helped to achieve esthetics, functionality, and psychological benefits.

  8. Prosthetic rehabilitation of a preschooler with induced anodontia - A clinical report

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    Shivayogi M Hugar

    2011-01-01

    Full Text Available A case of prosthetic rehabilitation with complete dentures for a three year nine months old child is presented. Dental prosthesis are frequently used to avoid psychologic, speech, or swallowing problems in preschooler children. The case was followed up for a period of three years. These prosthesis were modified as the child grew and as the permanent teeth erupted into the oral cavity the dentures were trimmed from the areas of erupting teeth to facilitate their eruption.

  9. [Ectodermal Capdepont syndrome and oral prosthetic rehabilitation. About a clinical case].

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    Kumpanya, P; Matshumba, M; Sekele, I B; Mayunga, M; Lutula, P S; Ntumba, M K

    2015-03-01

    The authors describe the ectodermal Capdepont syndrome as an anomaly characterized by anhidrosis, hypotrichosis and anodontia diagnosed in a 22 year-old adult. In front of this anodontia, oral prosthetic rehabilitation remains the only solution.

  10. Prosthetic Rehabilitation in Children: An Alternative Clinical Technique

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    Nádia Carolina Teixeira Marques

    2013-01-01

    Full Text Available Complete and partial removable dentures have been used successfully in numerous patients with oligodontia and/or anodontia. However, there is little information in the literature regarding the principles and guidelines to prosthetic rehabilitation for growing children. This case report describes the management of a young child with oligodontia as well as the treatment planning and the prosthetic rehabilitation technique.

  11. Implant-Retained Overdentures for Young Children With Severe Oligodontia : A Series of Four Cases

    NARCIS (Netherlands)

    Filius, Marieke A. P.; Vissink, Arjan; Raghoebar, Gerry M.; Visser, Anita

    Purpose: The treatment need is high in children with severe oligodontia and anodontia, because they often have functional and esthetic problems owing to missing teeth. Because the intraforaminal region barely grows after eruption of the permanent mandibular incisors, dental implant treatment should

  12. Christ siemens touraine syndrome: A rare case report

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    N Retnakumari

    2016-01-01

    Full Text Available Christ-Siemens-Touraine (CST is a rare hereditary disorder of X-linked recessive trait, characterized by abnormal development of two or more structures or tissues of ectodermal origin. The common clinical findings include hypodontia, hypohydrosis, hypotrichosis, and onychodysplasia. Although hypodontia is common, anodontia is a rare feature. Most of the patients are suffering from social rejection and consequent psychological trauma because of the facial dysmorphism and absence of multiple teeth. Oral rehabilitation is of prime importance for such patients. This article presents a case in a 5½-year-old boy presenting with altered manifestations affecting almost all the ectodermal structures like skin, hair, nails, teeth, sebaceous glands, sweat glands, salivary glands, mammary glands, and tear glands. He also had complete anodontia and dry mouth. A multidisciplinary treatment was given to the patient with the collaboration of various health professionals. The child gained confidence and was relieved from the psychological impact following the prosthetic rehabilitation.

  13. Prosthodontic management of anhidrotic ectodermal dysplasia

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    Shilpy Gupta

    2011-01-01

    Full Text Available Ectodermal dysplasia is characterized by the absence or defects of two or more ectodermally derived structures. Anodontia or hypodontia is the most striking dental manifestation. In severe hypodontia, there is lack of alveolar development with consequent protrusion and eversion of the lips. Patients with anhidrotic forms suffer from heat intolerance due to lack of sweat glands and mild infections may lead to death in infancy from hyperthermia. A case of a 4-year-old child with anhidrotic ectodermal dysplasia with partial anodontia is presented. Dental, oral, and physical features were taken into consideration in diagnosis and treatment planning for this patient. Clinical management consisted of removable partial prosthesis in maxillary arch and complete denture prosthesis in mandibular arch. The main aim of the treatment was to improve psychological development and to promote better functioning of the stomatognathic system.

  14. Multidisciplinary approach in the management of ectodermal dysplasia: A case report

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    Brahmananda Dutta

    2016-01-01

    Full Text Available Ectodermal dysplasias are rare hereditary disorders characterized by abnormal development of certain tissues and structures of ectodermal origin. The condition is of importance to dentists as it affects the teeth resulting in hypodontia or anodontia, and a dentist plays an important role in the rehabilitation of the patient. Apart from having difficulties in eating and speaking, young affected individuals can also feel that they look different from their contemporaries resulting in low self-esteem. Well-fitting and functioning prosthesis could be a great help during their schooling years, as it will improve appearance and thus boost their self-confidence. We report a case of hypohidrotic ectodermal dysplasia in a 12-year-old girl who exhibited partial anodontia, and a multidisciplinary approach comprising restorative, orthodontic, surgical, and prosthetic treatment was planned to rehabilitate the girl.The aim of the treatment was to improve psychological development apart from promoting better functioning of the stomatognathic system.

  15. Christ Siemens Touraine syndrome: A rare case report.

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    Retnakumari, N; Varghese, Manuja; Kannan, V P

    2016-01-01

    Christ-Siemens-Touraine (CST) is a rare hereditary disorder of X-linked recessive trait, characterized by abnormal development of two or more structures or tissues of ectodermal origin. The common clinical findings include hypodontia, hypohydrosis, hypotrichosis, and onychodysplasia. Although hypodontia is common, anodontia is a rare feature. Most of the patients are suffering from social rejection and consequent psychological trauma because of the facial dysmorphism and absence of multiple teeth. Oral rehabilitation is of prime importance for such patients. This article presents a case in a 5½-year-old boy presenting with altered manifestations affecting almost all the ectodermal structures like skin, hair, nails, teeth, sebaceous glands, sweat glands, salivary glands, mammary glands, and tear glands. He also had complete anodontia and dry mouth. A multidisciplinary treatment was given to the patient with the collaboration of various health professionals. The child gained confidence and was relieved from the psychological impact following the prosthetic rehabilitation.

  16. Hennekam lymphangiectasia syndrome

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    Lakshminarayana, G.; Mathew, A.; Rajesh, R.; Kurien, G.; Unni, V. N.

    2011-01-01

    Hennekam lymphangiectasia syndrome is a rare disorder comprising of intestinal and renal lymphangiectasia, dysmorphic facial appearance and mental retardation. The facial features include hypertelorism with a wide, flat nasal bridge, epicanthic folds, small mouth and small ears. We describe a case of a multigravida with bad obstetric history and characteristic facial and dental anomalies and bilateral renal lymphangiectasia. To our knowledge this is the first case of Hennekam lymphangiectasia syndrome with anodontia to be reported from India. PMID:22022089

  17. Hypohidrotic ectodermal dysplasia with ankylosis of temporomandibular joint and cleft palate: A rare presentation

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    Goyal, Manisha; Pradhan, Gaurav; Gupta, Sunita; Kapoor, Seema

    2015-01-01

    The ectodermal dysplasias are a heterogenous group of diseases, which have one or more anomalies of the hair, teeth, nails, and sweat glands. Hypohidrotic ectodermal dysplasia (HED) is the most common type and is usually transmitted as an X-linked recessive trait. It is characterized by classical triad of hypotrichosis, anhidrosis/hypohidrosis, and hypodontia/anodontia. Here, we describe an Indian boy affected with HED and rare features including ankylosis of temporomandibular joint and cleft palate. PMID:25684924

  18. A Novel Mutation in the EDAR Gene Causes Severe Autosomal Recessive Hypohidrotic Ectodermal Dysplasia

    DEFF Research Database (Denmark)

    Henningsen, Emil; Svendsen, Mathias Tiedemann; Lildballe, D. L.

    2014-01-01

    We report on a 2-year-old girl presenting with a severe form of hypohidrotic ectodermal dysplasia (HED). The patient presented with hypotrichosis, anodontia, hypohidrosis, frontal bossing, prominent lips and ears, dry, pale skin, and dermatitis. The patient had chronic rhinitis with malodorous......-mediated NF-kB signalling. This complete loss-of-function mutation likely accounts for the severe clinical abnormalities in ectodermal structures in the described patient. (C) 2014 Wiley Periodicals, Inc....

  19. Hypohidrotic ectodermal dysplasia with ankylosis of temporomandibular joint and cleft palate: A rare presentation

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    Manisha Goyal

    2015-01-01

    Full Text Available The ectodermal dysplasias are a heterogenous group of diseases, which have one or more anomalies of the hair, teeth, nails, and sweat glands. Hypohidrotic ectodermal dysplasia (HED is the most common type and is usually transmitted as an X-linked recessive trait. It is characterized by classical triad of hypotrichosis, anhidrosis/hypohidrosis, and hypodontia/anodontia. Here, we describe an Indian boy affected with HED and rare features including ankylosis of temporomandibular joint and cleft palate.

  20. Valutazione dello spostamento tridimensionale degli impianti in giovani pazienti affetti da EDs riabilitati con overdenture implanto-supportati

    OpenAIRE

    Battelli, Filippo

    2013-01-01

    Ectodermal Dysplasias syndrome (EDs) are a heterogeneous group of inherited disorders characterized by dysplasia of tissues of ectodermal origin. Complete or partial anodontia are the most frequent dental findings. Prosthetic rehabilitation is recommended from functional, esthetic, and psychological points of view. Because of the anatomical abnormalities of existing teeth and alveolar ridges, conventional prosthetic rehabilitation in young patient is often difficult. Five growing patients (ag...

  1. Dental implants for severely atrophied jaws due to ectodermal dysplasia

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    Preetha Balaji

    2015-01-01

    Full Text Available The aim was to present the successful esthetical and functional rehabilitation of partial anodontia in a case of severe ectodermal dysplasia with complete atrophy of the jaws. A 17-year-old male with Class III malocclusion with partial anodontia sought dental implant treatment. His expectation was that of Class I occlusion. The challenge in the case was to match the expectation, reality, and the clinical possibilities. Ridge augmentation was performed with a combination of rib graft and recombinant human bone morphogenetic protein-2. Simultaneously, 6 implants (Nobel Biocare™ - Tapered Groovy were placed in maxillary arch and 10 in the mandible. Simultaneous placement ensured faster and better osseointegration though a mild compromise of the primary stability was observed initially. After adequate healing, Customized Zirconia Procera™ system was used to build the framework. Zirconia crown was cemented to the framework. Radiological and clinical evidence of osseointegration was observed in all 16 dental implants. Successful conversion of Class III to Class I occlusion was achieved with the combination of preprosthetic alveolar ridge augmentation, Procera™ Implant Bridge system. Abnormal angulations and or placement of dental implants would result in failure of the implant. Hence conversion of Class III to Class I occlusion needs complete and complex treatment planning so that the entire masticatory apparatus is sufficiently remodeled. Planning should consider the resultant vectors that would otherwise result in failure of framework or compromise the secondary stability of the dental implant during function. A successful case of rehabilitation of complex partial anodontia is presented.

  2. Ectodermal dysplasia

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    Sonia Saggoo

    2009-01-01

    Full Text Available Hereditary hypohidrotic ectodermal dysplasia, also called the Christ-Siemens-Touraine Syndrome is characterized by congenital dysplasia of one or more ectodermal structures and is manifested by hypohidrosis, hypotrichosis and hypodontia. It is usually an X-linked recessive mendelian character which is rarely seen in males. It results from abnormal morphogenesis of cutaneous and oral embryonic ectoderm. Patients with this disorder exhibit smooth , thin and dry skin, fine and blond scanty hair. Intra-orally anodontia or hypodontia, with impaired development of alveolar process is seen. A case report of a rare case of this disorder in a female patient aged 18 years is hereby presented.

  3. Hypohidrotic ectodermal dysplasia: a felicitous approach to esthetic and prosthetic management.

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    Singh, Tapan; Singh, Ronauk; Singh, Gurendra Pal; Singh, Jitender Pal

    2013-05-01

    Ectodermal dysplasia is a hereditary disease characterized by congenital dysplasia of one or more ectodermal structure and other accessory appendages. The oral manifestations are anodontia and poor bony foundation which impairs both esthetic as well as the masticatory function. The prosthodontic management of patients with such dysplastic condition necessitates a multidisciplinary approach. This case report describes the prosthodontic oral rehabilitation of a 16 years old female pediatric patient with ectodermal dysplasia. How to cite this article: Singh T, Singh R, Singh GP, Singh JP. Hypohidrotic Ectodermal Dysplasia: A Felicitous Approach to Esthetic and Prosthetic Management. Int J Clin Pediatr Dent 2013;6(2):140-145.

  4. OVERLAY DENTURES: A REVIEW AND REPORT OF FOUR CASES

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    Deeksha SHARMA

    2013-12-01

    Full Text Available Patients with ectodermal dysplasia or having under‑ gone cleft surgery with anodontia or hypodontia, hypo‑ plastic conical teeth and patients with severely worn dentition are difficult to treat because of the poor remaining tooth structure. These patients often exhibit loss of vertical dimension of occlusion and aesthetic problems and usually need complex prosthetic treatments. Financial constraints or other priorities often restrict one from choosing the most desirable treatment. The overlay removable denture is a covering prosthesis partially supported by natural teeth, tooth roots, or dental implants, providing an efficient alter‑ native of treatment. Clinical reports describe the various applications of overlay dentures.

  5. Diagnosis and Management of a Patient with Congenitally Missing Maxillary First Permanent Molars: A Rare Case Report

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    Megha Gupta

    2016-01-01

    Full Text Available Congenitally missing teeth are the most commonly seen dental anomalies. Agenesis of the permanent first molar has the least frequency of all the tooth types, and it usually occurs in association with oligodontia or anodontia. Thus, agenesis of the bilateral maxillary first permanent molar is an extremely rare occurrence, and no such case has been reported in ethnic Saudi Arabian population. We hereby report a case of nonsyndromic bilateral congenitally missing maxillary first permanent molar in an eight-year-old Saudi female patient. Comprehensive oral rehabilitation was done for the patient. The implications of the tooth agenesis are also discussed. The prognosis of this case is presented.

  6. Dental Management of Ectodermal Dysplasia: Two Clinical Case Reports

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    Somayeh Hekmatfar

    2012-06-01

    Full Text Available Ectodermal dysplasia is a hereditary disorder associated with abnormal development of embryonic ectodermally-derived organs including teeth, nails, hair and sweat glands. Hypodontia of the primary and permanent dentition is the most common oral finding. Therefore, affected patients need dental prosthetic treatments during their developmental years. This report presents two cases of children affected by ectodermal dysplasia with anodontia. Oral rehabilitation was accomplished with removable acrylic prostheses. Treatment had major impacts on self-esteem, masticatory function, speech and facial esthetic.

  7. Prosthetic rehabilitation of patients with hypohidrotic ectodermal dysplasia: A systematic review.

    Science.gov (United States)

    Schnabl, D; Grunert, I; Schmuth, M; Kapferer-Seebacher, I

    2018-04-21

    Hypohidrotic ectodermal dysplasia (HED) comprises a large group of inherited disorders of ectodermal structures, characterised by hypo- or anhidrosis, hypotrichosis and hypo- or oligo- or anodontia. We aimed to systematically assess the spectrum of prosthodontic approaches with regard to the patients' age and to provide clinical implications for practicing dentists. An electronic and manual search was conducted in four databases (Medline, LIVIVO, Cochrane Library, Web of Science Core Collection). Publications of multiple study designs written in English or German without data restrictions, reporting on prosthodontic treatment of patients diagnosed with HED and afflicted with oligo- or anodontia, were included. In total, 75 articles on 146 patients were analysed according to the patients' age. In children aged 2-17 years, removable full or partial (over)dentures represented standard treatment. In the mandible, implant-supported removable dentures on two interforaminal implants presented an alternative, already in young childhood. In cases with more than six teeth per jaw, also fixed (resin) bridges were used, frequently after orthodontic treatment. In adults, fixed or removable reconstructions with the help of up to eight implants per jaw, usually placed after bone augmentation procedures, were standard. Ten case reports/series with long-term follow-up illustrated the need for consistent maintenance including denture renewals. Prosthodontic rehabilitation should start in early childhood and needs to be revised in accordance with the patients' growth. Treatment should be carried out by a multidisciplinary team addressing variable demands in different age groups. © 2018 John Wiley & Sons Ltd.

  8. Implant-retained overdentures for young children with severe oligodontia: a series of four cases.

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    Filius, Marieke A P; Vissink, Arjan; Raghoebar, Gerry M; Visser, Anita

    2014-09-01

    The treatment need is high in children with severe oligodontia and anodontia, because they often have functional and esthetic problems owing to missing teeth. Because the intraforaminal region barely grows after eruption of the permanent mandibular incisors, dental implant treatment should be considered a treatment option for these children. The purpose of our study was to assess the treatment outcomes regarding satisfaction and the care and aftercare of implant-retained mandibular overdentures in a series of 4 young children without erupted mandibular teeth from either severe oligodontia (n = 3) or anodontia (n = 1). Four children without erupted mandibular teeth, aged 6 to 13 years, were provided with an implant-retained overdenture on 2 implants. The surgical and prosthetic care and aftercare were scored by the clinicians. Also, the patients and their parents were queried about how satisfied they were with the overdenture. The median follow-up of the patients was 5.2 years (range 3.2 to 8.4). No implants were lost, no cases of peri-implantitis occurred, and the need for treatment and aftercare was low. Patient and parent satisfaction with this treatment was high. A 2-implant, retained overdenture in children with no erupted mandibular teeth is a safe treatment modality when appropriate treatment and aftercare can be provided. Copyright © 2014 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.

  9. Osseointegrated Supported Prosthesis and Interdisciplinary Approach for Prosthodontic Rehabilitation of a Young Patient with Ectodermal Dysplasia

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    Karthik M. Sadashiva

    2013-01-01

    Full Text Available Anhidrotic ectodermal dysplasia is a triad of hypodontia or anodontia, hypotrichosis, and hypohydrosis, associated with other problems that result from the defective development of structures of ectodermal origin (Freire-Maia, Pinheiro (1988. Early and extensive dental treatment is needed keeping in mind the effect on the craniofacial growth. Due to rapid growth of the jaws, the patients are rehabilitated using removable prostheses (Tarjan et al. (2005. Hence for a young patient in this case report, the placement of endosseous osseointegrated implants was delayed till adulthood. Finally a definitive fixed tooth-supported and osseointegrated implant supported fixed partial denture therapy was used to rehabilitate the patient satisfactorily after she had completed her growth (Sweeney et al. (2005. A review of the current literature relevant to several aspects of syndromic hypodontia, patient selection, and implant planning is discussed.

  10. A novel mutation in the EDAR gene causes severe autosomal recessive hypohidrotic ectodermal dysplasia.

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    Henningsen, Emil; Svendsen, Mathias Tiedemann; Lildballe, Dorte Launholt; Jensen, Peter Kjestrup Axel

    2014-08-01

    We report on a 2-year-old girl presenting with a severe form of hypohidrotic ectodermal dysplasia (HED). The patient presented with hypotrichosis, anodontia, hypohidrosis, frontal bossing, prominent lips and ears, dry, pale skin, and dermatitis. The patient had chronic rhinitis with malodorous nasal discharge. The girl was the second born child of first-cousin immigrants from Northern Iraq. A novel homozygous mutation (c.84delC) in the EDAR gene was identified. This mutation most likely causes a frameshift in the protein product (p.S29fs*74). This results in abolition of all ectodysplasin-mediated NF-kB signalling. This complete loss-of-function mutation likely accounts for the severe clinical abnormalities in ectodermal structures in the described patient. © 2014 Wiley Periodicals, Inc.

  11. Pemakaian Gigi Tiruan Sebagian Pada Penderita Ektodermal Displasia dengan Anondonsia Parsial

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    Rastia Indriyati

    2015-09-01

    Full Text Available Ectodermal dysplasia is a hereditary disease characterized by a congenital dysplasia of one or more ectodermal structure and their accessory appendeges. The main feature is hypohidrosis, hypotrichosis and hypodontia. Ectodermal dysplasia appear to be ingeritaed as an x-linked recessive trait. Common manifestations include frontal bossing with prominent supraorbital ridges, nasal bridge depression, protuberant lips and defective hair follicles and eyebrows. Intraorally, common findings are hypodontia or anodontia and conical teeth. The treatment is to improve the appearance and oral function included a removable prosthesis. A 5-year-old and 9-year-old male in the same family, described in the case report of ectodermal dysplasia with removable partial dentures

  12. REHABILITASI PASIEN GIGI TIRUAN PENUH DENGAN EKTODERMAL DYSPLASIA (LAPORAN KASUS

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    Susi R. Puspita Dewi

    2015-07-01

    Full Text Available Ectodermal dysplasia is a rare congenital disease that affects several ectodermal structures. This disease is usually transmitted as an x-linked recessive trait in which the gene is carried by female and manifested in male. The orofacial characteristics of ectodermal dysplasia include anodontia or hypodontia, congenital teeth, underdevelopment of alveolar ridges and it is not uncommon for the face of an affected child to take on the appearance characteristic of old age, a prominent forehead, protuberant lips, a depressed nasal bridge, hypotricosis, and hypohidrosis. The treatment to manage orogacial disfigurement may afford the patient some measure of confidence, esthetics, function and speech. This case report describes the diagnosis and treatment of ectodermal dysplasia in an 18 year patient. The treatment included removable complete denture.

  13. Using implants for prosthodontic rehabilitation of a 4-year-old with ectodermal dysplasia.

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    Toomarian, Lida; Ardakani, Mohammad Reza Talebi; Ramezani, Jamileh; Adli, Amin Rezaei; Tabari, Zahra Alizadeh

    2014-01-01

    Ectodermal dysplasia (ED) is an inherited disorder that affects ectodermally derived organs, such as teeth. Pathogenesis is thought to involve an altered epithelium-mesenchymal interaction. ED patients have oligodontia (or sometimes anodontia) in addition to other abnormalities involving the skin, sweat glands, or hair. Many different subtypes have been introduced in the literature. This article describes the case of a 4-year-old patient who, after being diagnosed with ED, was put on a treatment plan that involved mandibular implants, reshaping of the maxillary primary central incisors, and prosthetic dental rehabilitation. Due to the child's rapid growth, both dentures were changed 9 months post-treatment. Two years post-treatment, the maxillary denture was changed again and the child was placed under close supervision.

  14. Anesthetic management of a pediatric patient with hypohidrotic ectodermal dysplasia undergoing emergency surgery.

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    Ahiskalioglu, Elif Oral; Ahiskalioglu, Ali; Firinci, Binali; Dostbil, Aysenur; Aksoy, Mehmet

    2015-01-01

    Ectodermal dysplasias are rare conditions with a triad of hypotrichosis, anodontia and anhidrosis. In literature review there have been only a few reports of anesthetic management of patients with ectodermal dysplasias. Hyperthermia is a very serious risk which may occur due to the defect of sweat glands. The present case involves a 10-year-old child with ectodermal dysplasia who presented with an acute abdomen and was considered for an emergency surgery. Our aim was to demonstrate the successful management of this case using a combination of general and epidural anesthesia. It is important for anesthesiologist to have information about this syndrome in case of emergency operations, since it can prevent serious complications and even save lives. Copyright © 2013 Sociedade Brasileira de Anestesiologia. Published by Elsevier Editora Ltda. All rights reserved.

  15. Prosthodontic Rehabilitation of Hereditary Ectodermal Dysplasia in an 11-Year-Old Patient with Flexible Denture: A Case Report

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    Neha Jain

    2012-01-01

    Full Text Available Hereditary ectodermal dysplasia is a rare group of inherited disorders characterized by aplasia or dysplasia of two or more tissues of ectodermal origin such as hair, nails, teeth, and skin. The dental characteristics of this syndrome include anodontia or hypodontia of the primary and/or permanent teeth, hypoplastic conical teeth, and underdevelopment of the alveolar ridges. The options for a definitive treatment plan include fixed, removable or implant-supported prostheses, singly or in combination. This clinical report describes the prosthetic rehabilitation of an 11-year-old boy with hereditary ectodermal dysplasia. Maxillary flexible removable partial denture and mandibular conventional complete denture were fabricated to establish an acceptable masticatory function, speech, and esthetics for the patient.

  16. Dental anomalies in panoramic radiographs of pediatric patients.

    Science.gov (United States)

    Marsillac, Mirian de Waele Souchois de; Andrade, Marcia Rejane Thomas; Fonseca, Raquel de Oliveira; Marcal, Sonia Lucia Macedo; Santos, Vera Lucia Campos

    2013-01-01

    A panoramic radiograph is more likely utilized in children with high caries risk and mixed dentition, and it can be complemented by other X-rays (such as periapical and/or bitewings). This study analyzed 1359 panoramic radiographs taken over 33 years at the Pedodontics Clinic of the State University of Rio de Janeiro in order to determine the prevalence of dental anomalies in mixed dentition children. The population evaluated had 670 (49.3%) boys and 689 (50.7%) girls, ranging in age from 5-12 years, with a median age of 8 years. The total prevalence of anomalies detected was 11.72%; anodontia and supernumerary teeth were the most reported (4.63% and 3.31%, respectively). Statistical differences noted were in the presence of supernumary teeth in males (4.9%, P dentition, as well as complement the clinical examination at the first dental visit of a pediatric patient with a high risk for caries.

  17. The role of partial denture in management of hypohidrotic ectodermal dysplasia

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    Tania Saskianti

    2008-06-01

    Full Text Available Ectodermal dysplasia is a rare hereditary disorder with a characteristic physiognomy. The ectodermal dysplasia constitutes a group of hereditary disorders whose clinical manifestation can be defects in ectodermal structures. The case of a 11-year-old child with hypohidrotic ectodermal dysplasia and partial anodontia is presented. Affected children require extensive dental treatment to restore appearance and help the development of a positive self image. Partial denture was provided to encourage a normal psychological development and to improve the function of the stomatognatic system. It is important for the patient and the dentist to understand that continued monitoring for dental problems is necessary. This paper had an objective to relate and discuss a case of hypohidrotic ectodermal dysplasia, with the approach of the influence of an esthetic rehabilitation and functional alternative in the improvement of the quality of life.

  18. Ectodermal Dysplasia: Report and Analysis of Eleven South Indian Patients with Review of Literature

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    Renuka Ammanagi

    2010-01-01

    Full Text Available Ectodermal dysplasia represents a rare syndrome affecting two or more ectodermally derived structures. The condition is thought to occur in approximately 1 in every 100,000 live births. It affects men more frequently and severely, while women being the carriers and heterozygote usually show minor defects. There are more than 150 different variants of ectodermal dysplasia (ED reported in the literature. Most commonly encountered among them is hypohidrotic ED which frequently exhibits the most severe dental anomalies like hypodontia or anodontia along with hypohidrosis and hypotrichosis. Here we make an attempt to collectively report and discuss eleven South Indian patients who reported to our department during the year 1998 to 2004. An added emphasis is laid on family history of consanguineous marriage among the parents of these patients.

  19. Anesthetic management of a pediatric patient with hypohidrotic ectodermal dysplasia undergoing emergency surgery

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    Elif Oral Ahiskalioglu

    2015-11-01

    Full Text Available Ectodermal dysplasias are rare conditions with a triad of hypotrichosis, anodontia and anhidrosis. In literature review there have been only a few reports of anesthetic management of patients with ectodermal dysplasias. Hyperthermia is a very serious risk which may occur due to the defect of sweat glands. The present case involves a 10-year-old child with ectodermal dysplasia who presented with an acute abdomen and was considered for an emergency surgery. Our aim was to demonstrate the successful management of this case using a combination of general and epidural anesthesia. It is important for anesthesiologist to have information about this syndrome in case of emergency operations, since it can prevent serious complications and even save lives.

  20. Anesthetic management of a pediatric patient with hypohidrotic ectodermal dysplasia undergoing emergency surgery

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    Elif Oral Ahiskalioglu

    2015-12-01

    Full Text Available ABSTRACT Ectodermal dysplasias are rare conditions with a triad of hypotrichosis, anodontia and anhidrosis. In literature review there have been only a few reports of anesthetic management of patients with ectodermal dysplasias. Hyperthermia is a very serious risk which may occur due to the defect of sweat glands. The present case involves a 10-year-old child with ectodermal dysplasia who presented with an acute abdomen and was considered for an emergency surgery. Our aim was to demonstrate the successful management of this case using a combination of general and epidural anesthesia. It is important for anesthesiologist to have information about this syndrome in case of emergency operations, since it can prevent serious complications and even save lives.

  1. Peran Gigi pada Tumbuh Kembang Dentofasial (Laporan Kasus

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    Retno Hayati

    2015-11-01

    Full Text Available The function of dentition is not only for mastication, the other function is to coordinate the growth of maxilla, midface and mandible. This important function was done through occlusal contact during occlusion. The occlusal surfaces become a link of stomato-gnathic system. This case report showed the midface growth was affected by agenesis. The first case was 10-years old girl with congenital missing 16 and 26. The relationship of upper and lower arch erior was edge to edge and crossbite. In posterior, the occlusal contact were found between upper second primary molars and lower first permanent molars. The second and third cases were 5 and 9-years old boys. They were siblings with ectodermal dysplasias and partial anodontia. The facial profile of these children were concave. The relationship of maxilla and mandible was class III. The manifestation of growth retardation was shown by cephalometric measurement, SN, Ans-Pns, Ptm-A, N-Me, Ans-Me, S-Go were smaller than standard normal children.

  2. Prosthodontic management of hypohidrotic ectodermal dysplasia: a case report

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    Emilija Bajraktarova Valjakova

    2015-09-01

    Full Text Available Introduction: Ectodermal dysplasia (ED is a hereditary disorder associated with developmental disorders of two or more structures of ectodermal embryonic origin. Hypodontia or anodontia of the primary and permanent dentition, poorly developed alveolar ridges and improper maxillo – mandibular relations, are the most common oral manifestations. Management of patients with ectodermal dysplasia requires a multidisciplinary team approach. Case presentation: A 6.5 year-old boy with hypohidrotic ectodermal dysplasia (HED presented in this article, had typical features of HED: hypohidrosis, hypotrichosis, severe hypodontia, atrophic alveolar ridges, old-looking facial expression. According to the patient’s age and clinical findings, removable complete over-denture prosthesis in both arches was the treatment of choice. Conclusion: In patients with ED, it is important to establish correct maxillo – mandibular relations and normal function of the dento-facial system (chewing, swallowing, and speaking. Prosthodontic treatment has a major impact on aesthetics and functions,facilitates psychological development and improves emotional condition and social life of the patient.

  3. Clinical outcomes of implant therapy in ectodermal dysplasia patients: a systematic review.

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    Wang, Y; He, J; Decker, A M; Hu, J C; Zou, D

    2016-08-01

    The purpose of this review was to determine the outcome of oral function reconstruction in ectodermal dysplasia (ED) patients who have received dental implant therapy. A search was made of the PubMed and Web of Science databases; key words used were "(ectodermal dysplasia) AND (implant OR implants)", with supplementary retrieval key words "dental implant", "zygomatic implant", "anodontia", and "edentulous". Patient age, use of bone graft, implant site, type of implant, and survival rate of the implants were included in the subsequent data analysis. Forty-five articles published between 1988 and October 2015 were included in this analysis. The cases of a total of 96 patients were retrieved (22 children and 74 adults); these patients received a total of 701 implants. Fourteen implants were removed during a median follow-up time of 24 months. The 24-month implant survival rate was 97.9% in adult subjects and 98.6% in children. Sixty-eight percent of adult patients underwent bone augmentation prior to implant placement. Based on this review, dental implants are commonly used in the oral reconstruction of ED patients. However, long-term data on bone augmentation and implant success are needed, as well as additional clinical evidence on bone resorption, the esthetic outcomes of implant therapy, and physiological considerations in ED patients. Copyright © 2016 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

  4. Ambras Syndrome: First Reported Case in Bangladesh and its Oral Rehabilitation.

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    Khan, M H; Ashrafuzzaman, S M; Taib, A N; Alam, M T; Khan, S H; Goldstein, S K; Rahman, R

    2015-10-01

    People with rare hypertrichosis syndromes became crowd-drawing money-making phenomena in many 19th century sideshow acts. These individuals have been referred to as dog-men, hair-men, and werewolves. In 1993, Baumister et al. described congenital hypertrichosis lanuginose or Ambras syndrome: a distinct form of congenital hypertrichosis characterized by excessive hair growth over the body and face associated with facial and occasional dental anomalies. Much is not known about this syndrome since fewer than 50 cases have been documented worldwide. In this case report, a nine year old girl presented with excessive hair growth throughout her body that was denser along her midline. Furthermore, her face displayed the typical dysmorphic features characteristic of Ambras syndrome: a round tip nose, thickened nasal cartilage, antiverted nares, prominent philtrum with deep groove, and a trapezoid mouth. Oral examination revealed normal oral mucosa with completely missing and unerupted decidious and permanent teeth. Panoramic radiographs confirmed unerupted deciduous teeth. Previous case reports have mentioned the presence of occasional dental anomalies such as retarded first and second dentition and absence of some teeth. However, this is the first reported case of Ambras syndrome presenting with complete anodontia. Prior cytogenetic studies performed on persons with Ambras syndrome have implicated a balanced pericentric inversion of chromosome 8. However, it is likely that dental anomalies are likely a result of a different genetic rearrangement. Further studies are needed to explore the cause of this rare phenotype of Ambras syndrome with complete unerupted dentition.

  5. Oral findings in a patient with Sebaceous Nevi - A Case Report.

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    Baliga, Vidya; Gopinath, V P K; Baliga, Sudhindra; Chandra, Umesh

    2013-10-01

    The presentation herewith as scripted is to describe a case with Nevus sebaceous with oral manifestations. Nevus Sebaceous or Jadassohn's nevus is an epidermal nevus with predominant sebaceous glands seen histologically. Reports of oral involvement have been few ranging from papillomatous growths of the tongue, gingiva, palate to dental abnormalities such as anodontia and dysodontia. The present case describes a nevus sebaceous present on the right half of the face and neck, showing intraoral papillomatous growth on the lateral part of the tongue on the right side. The patient was healthy and did not report involvement of any other organ systems. Intraoral involvement may be seen in patients with Nevus Sebaceous, hence proper screening is important. In patients presenting with large nevi on the head and neck such as ours, involvement of other systems such as ocular, neurologic and oral lesions may be seen, therefore screening of such patients is of importance. Patients with nevus sebaceous may be predisposed to the occurrence of tumours. Therefore, careful screening of such patients is necessary. How to cite this article: Baliga V, Gopinath VP, Baliga S, Chandra U. Oral findings in a patient with Sebaceous Nevi - A Case Report. J Int Oral Health 2013; 5(5):139-42.

  6. Oculodentodigital dysplasia

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    Dharmil C Doshi

    2016-01-01

    Full Text Available Oculodentodigital dysplasia is a rare, autosomal dominant disorder with high penetrance and variable expressivity, caused by mutations in the connexin 43 or gap junction protein alpha-1 gene. It has been diagnosed in fewer than 300 people worldwide with an incidence of around 1 in 10 million. It affects many parts of the body, particularly eyes (oculo, teeth (dento, and fingers and/or toes (digital. The common clinical features include facial dysmorphism with thin nose, microphthalmia, syndactyly, tooth anomalies such as enamel hypoplasia, anodontia, microdontia, early tooth loss and conductive deafness. Other less common features are abnormalities of the skin and its appendages, such as brittle nails, sparse hair, and neurological abnormalities. To prevent this syndrome from being overlooked, awareness of possible symptoms is necessary. Early recognition can prevent blindness, dental problems and learning disabilities. Described here is the case of a 21-year-old male who presented to the ophthalmology outpatient department with a complaint of bilateral progressive loss of vision since childhood.

  7. Implant Supported Prosthesıs in a Patıent wıth Progerıa: Case Report

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    Gözlem Ceylan

    2009-08-01

    Full Text Available Prosthodontic rehabilitation can be accomplished with fixed, overdenture, complete, or implant-retained prostheses. Dental treatment overcomes the patient’s functional, psychological, esthetic and phonation problems. Remaining healthy teeth may allow the dentist to fabrícate a removable partial overdenture, fixed partial prosthesis or implant - supported prosthesis. The retention of a number of abutments helps maintain a positive ridge form with greater height and volume of the alveolar bone, improving masticatory performance, as well as providing a more stable prostheses. Dental patients who have medical problems need many treatment procedures. Multidisciplinary treatment planning is invaluable for patient’s dental health. Progeria is a rare genetic condition where symptoms resembling aspects of aging are manifested at an early age. characteristic clinical findings of Progeria disease include abnormalities of the skin and hair in conjunction with char-acteristic facial features and skeletal abnormalities. The characteristic facies show protruding ears, beaked nose, thin lips with centrofacial cyanosis, prominent eyes, frontal and parietal bossing with pseudohydrocephaly, midface hypoplasia with micrognathia and large anterior fontanel. The other reported anomalies are dystrophic nails, hypertrophic scars and hypoplastic nipples. The findings that are nearly interested in dentistry are delayed dentition, anodontia, hypodontia, or crowding of teeth. This article presents the multidisciplinary dental treatment planning includes surgical, endodontic and prosthetic treatment of a patient with a history of progeria. In this case complete-arch fixed prostheses in both maxilla and mandible, supported by a combination of im-plants and teeth are reported.

  8. The isotopic biosignatures of photo- vs. thiotrophic bivalves: are they preserved in fossil shells?

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    Dreier, A; Loh, W; Blumenberg, M; Thiel, V; Hause-Reitner, D; Hoppert, M

    2014-09-01

    Symbiont-bearing and non-symbiotic marine bivalves were used as model organisms to establish biosignatures for the detection of distinctive symbioses in ancient bivalves. For this purpose, the isotopic composition of lipids (δ13C) and bulk organic shell matrix (δ13C, δ34S, δ15N) from shells of several thiotrophic, phototrophic, or non-symbiotic bivalves were compared (phototrophic: Fragum fragum, Fragum unedo, Tridacna maxima; thiotrophic: Codakia tigerina, Fimbria fimbriata, Anodontia sp.; non-symbiotic: Tapes dorsatus, Vasticardium vertebratum, Scutarcopagia sp.). ∆13C values of bulk organic shell matrices, most likely representing mainly original shell protein/chitin biomass, were depleted in thio- and phototrophic bivalves compared to non-symbiotic bivalves. As the bulk organic shell matrix also showed a major depletion of δ15N (down to -2.2 ‰) for thiotrophic bivalves, combined δ13C and δ15N values are useful to differentiate between thio-, phototrophic, and non-symbiotic lifestyles. However, the use of these isotopic signatures for the study of ancient bivalves is limited by the preservation of the bulk organic shell matrix in fossils. Substantial alteration was clearly shown by detailed microscopic analyses of fossil (late Pleistocene) T. maxima and Trachycardium lacunosum shell, demonstrating a severe loss of quantity and quality of bulk organic shell matrix with time. Likewise, the composition and δ13C-values of lipids from empty shells indicated that a large part of these compounds derived from prokaryotic decomposers. The use of lipids from ancient shells for the reconstruction of the bivalve's life style therefore appears to be restricted. © 2014 John Wiley & Sons Ltd.

  9. Hereditary ectodermal dysplasia: A retrospective study

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    More, Chandramani B.; Bhavsar, Khusbhu; Joshi, Jigar; Varma, Saurabh N.; Tailor, Mansi

    2013-01-01

    Background: Ectodermal dysplasia (ED) is a group of rare, inherited disorders characterized by sparse hair, missing teeth and inability to sweat. Objective: To review and analyze cases of ED with an emphasis on clinical manifestations and parent's marriage history. Methodology: The present retrospective study was conducted by assessing the clinical records of nineteen cases of ED, available in the archives of the department; for age, gender, family history of consanguineous marriage and clinical manifestations. Results: It was observed that ED was more prevalent in males, with a ratio of 1.7:1. The hypohydrotic type was more common (78.95%) than hydrotic type (21.05%). The marriage history of parents revealed that 66.67% had consanguineous marriage and had 68.42% offspring's affected with ED; whereas 33.33% had history of non-consanguineous marriage and had 31.58% offspring's affected with ED. 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%); abnormal sweat glands(78.95%); hypoplastic maxilla(52.63%); protuberant lips (57.89%); palmo-plantar keratosis(21.05%); wrinkled & hyper pigmented facial skin(84.21%); partial anodontia(94.74%); conical shaped teeth(84.21%); high arched palate(68.42%); thin alveolar bone(100.00%); taurodontism(21.05%) and cleft lip & cleft palate(05.26%). The number of teeth present in all the cases ranged from 0 to 19. Conclusion: ED patients suffer from social problems and poor psychological and physiological development as a result of unacceptable esthetics and abnormal function of orofacial structures. Oral rehabilitation thus becomes mandatory, although it is often difficult; particularly in pediatric patients. PMID:24082749

  10. Cenozoic Methane-Seep Faunas of the Caribbean Region.

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    Steffen Kiel

    Full Text Available We report new examples of Cenozoic cold-seep communities from Colombia, Cuba, the Dominican Republic, Trinidad, and Venezuela, and attempt to improve the stratigraphic dating of Cenozoic Caribbean seep communities using strontium isotope stratigraphy. Two seep faunas are distinguished in Barbados: the late Eocene mudstone-hosted 'Joes River fauna' consists mainly of large lucinid bivalves and tall abyssochrysoid gastropods, and the early Miocene carbonate-hosted 'Bath Cliffs fauna' containing the vesicomyid Pleurophopsis, the mytilid Bathymodiolus and small gastropods. Two new Oligocene seep communities from the Sinú River basin in Colombia consist of lucinid bivalves including Elongatolucina, thyasirid and solemyid bivalves, and Pleurophopsis. A new early Miocene seep community from Cuba includes Pleurophopsis and the large lucinid Meganodontia. Strontium isotope stratigraphy suggests an Eocene age for the Cuban Elmira asphalt mine seep community, making it the oldest in the Caribbean region. A new basal Pliocene seep fauna from the Dominican Republic is characterized by the large lucinid Anodontia (Pegophysema. In Trinidad we distinguish two types of seep faunas: the mudstone-hosted Godineau River fauna consisting mainly of lucinid bivalves, and the limestone-hosted Freeman's Bay fauna consisting chiefly of Pleurophopsis, Bathymodiolus, and small gastropods; they are all dated as late Miocene. Four new seep communities of Oligocene to Miocene age are reported from Venezuela. They consist mainly of large globular lucinid bivalves including Meganodontia, and moderately sized vesicomyid bivalves. After the late Miocene many large and typical 'Cenozoic' lucinid genera disappeared from the Caribbean seeps and are today known only from the central Indo-Pacific Ocean. We speculate that the increasingly oligotrophic conditions in the Caribbean Sea after the closure of the Isthmus of Panama in the Pliocene may have been unfavorable for such large

  11. Fragile X syndrome: panoramic radiographic evaluation of dental anomalies, dental mineralization stage, and mandibular angle

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    Aida Sabbagh-Haddad

    Full Text Available ABSTRACT Fragile X syndrome (FXS is a disorder linked to the chromosome X long arm (Xq27.3, which is identified by a constriction named fragile site. It determines various changes, such as behavioral or emotional problems, learning difficulties, and intellectual disabilities. Craniofacial abnormalities such as elongated and narrow face, prominent forehead, broad nose, large and prominent ear pavilions, strabismus, and myopia are frequent characteristics. Regarding the oral aspects, deep and high-arched palate, mandibular prognathism, and malocclusion are also observed. Objective: The purpose of this study was to evaluate the dental radiographic characteristics as described in 40 records of patients with panoramic radiography. Material and Methods: The patients were in the range of 6–17 years old, and were divided into two groups (20 subjects who were compatible with the normality standard and 20 individuals diagnosed with the FXS, which were matched for gender and age. Analysis of the panoramic radiographic examination involved the evaluation of dental mineralization stage, mandibular angle size, and presence of dental anomalies in both deciduous and permanent dentitions. Results: The results of radiographic evaluation demonstrated that the chronology of tooth eruption of all third and second lower molars is anticipated in individuals with FXS (p<0.05. In this group, supernumerary deciduous teeth (2.83%, giroversion of permanent teeth (2.31%, and partial anodontia (1.82% were the most frequent dental anomalies. In addition, an increase was observed in the mandibular angle size in the FXS group (p<0.05. Conclusion: We conclude that knowledge of dental radiographic changes is of great importance for dental surgeons to plan the treatment of these individuals.

  12. Prevalence and distribution of selected developmental dental anomalies in an Indian population.

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    Gupta, Saurabh K; Saxena, Payal; Jain, Sandhya; Jain, Deshraj

    2011-06-01

    The purpose of this study was to determine the prevalence of developmental dental anomalies in an Indian population and to statistically analyze the distribution of these anomalies. The study was based on clinical examination, evaluation of dental casts, and panoramic radiographs of 1123 Indian subjects (572 males, 551 females), who visited the outpatient clinic at Government Dental College, Indore between November 2009 and September 2010, after obtaining their informed consent. These patients were examined for the following developmental dental anomalies: shape anomalies (microdontia, talon cusp, dens evaginatus, fusion, taurodontism), number anomalies (hypodontia, oligodontia, anodontia), structural anomalies (amelogenesis imperfecta, dentinogenesis imperfecta) and positional anomalies (ectopic eruption, rotation, impaction). The percentages of these anomalies were assessed for the whole group and compared using statistical analysis. Among the 1123 subjects, a total of 385 individuals (34.28%) presented with the selected developmental dental anomalies. The distribution by sex was 197 males (34.44%), and 188 females (34.06%). Out of the total 1123 individuals, 351 (31.26%) exhibited at least one anomaly, 28 (2.49 %) showed two anomalies and 6 (0.53%) displayed more than two anomalies. P values indicated that the dental anomalies were statistically independent of sex. On intergroup comparison, positional anomalies were significantly most prevalent (P dental anomaly was rotation (10.24%), followed by ectopic eruption (7.93%). The next common group was number anomalies. The most common number anomaly was hypodontia (4.19%), which had a higher frequency than hyperdontia (2.40%). Analyzing the next prevalent group of shape anomalies, microdontia (2.58%) was found to be the most common, followed by taurodontism (2.49%), dens evaginatus (2.40%) and talon cusp (0.97%). Dentinogenesis imperfecta (0.09%) was the rarest, followed by amelogenesis imperfecta (0.27%) and fusion

  13. Displasia ectodérmica: Presentación de un caso ECTODERMAL DYSPLASIA: A CASE REPORT

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    Jaime Diez Betancourt

    2005-04-01

    Full Text Available La displasia ectodérmica es una enfermedad genética muy rara, la cual se encuentra caracterizada por manifestaciones odontoestomatológicas imponentes; presenta aspectos interesantes desde el punto de vista del diagnóstico, así como la rehabilitación conjunta ortodóntica, protésica y estética. En el Servicio de Ortodoncia de la Clínica Estomatológica INPE concurrió una paciente de 9 años de edad con manifestaciones clínicas de displasia ectodérmica, principalmente anodoncia, hipoplasia de dientes anteriores y malposición dentaria, con dientes de aspecto de conos y anomalías de las cúspides, y con afectación sicológica de la paciente. Con un tratamiento conjunto de ortodoncia y del estomatólogo general integra,l se logró restituir la maloclusión, y al mismo tiempo, con el tratamiento estético, se devolvió a la paciente a la vida socialmente útil y afectiva en vísperas de cumplir los 15 años.The ectodermic dysplasia is a very rare genetic disease that is characterized by impressive odontostomatological manifestations. It presents interesting aspects regarding the diagnosis and the joined orthodontic, prosthetic and aesthetic rehabilitation. A 9-year-old patient with clinical manifestations of ectodermic dysplasia, mainly anodontia, hypoplasia of the anterior tooth and tooth malposition, with cone-like tooth, abnormalities of the cusps and psychological affectation, presented for care at the Orthodontics Service of the INPE Dental Clinic. By a joint treatment of orthdontics and the general stomatologist, it was possible to restore malocclusion and, at the same time, with the aesthetic treatment the patient was returned to the socially useful and affective life before being 15.

  14. Hereditary ectodermal dysplasia: A retrospective study.

    Science.gov (United States)

    More, Chandramani B; Bhavsar, Khusbhu; Joshi, Jigar; Varma, Saurabh N; Tailor, Mansi

    2013-07-01

    Ectodermal dysplasia (ED) is a group of rare, inherited disorders characterized by sparse hair, missing teeth and inability to sweat. To review and analyze cases of ED with an emphasis on clinical manifestations and parent's marriage history. The present retrospective study was conducted by assessing the clinical records of nineteen cases of ED, available in the archives of the department; for age, gender, family history of consanguineous marriage and clinical manifestations. It was observed that ED was more prevalent in males, with a ratio of 1.7:1. The hypohydrotic type was more common (78.95%) than hydrotic type (21.05%). The marriage history of parents revealed that 66.67% had consanguineous marriage and had 68.42% offspring's affected with ED; whereas 33.33% had history of non-consanguineous marriage and had 31.58% offspring's affected with ED. 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%); abnormal sweat glands(78.95%); hypoplastic maxilla(52.63%); protuberant lips (57.89%); palmo-plantar keratosis(21.05%); wrinkled & hyper pigmented facial skin(84.21%); partial anodontia(94.74%); conical shaped teeth(84.21%); high arched palate(68.42%); thin alveolar bone(100.00%); taurodontism(21.05%) and cleft lip & cleft palate(05.26%). The number of teeth present in all the cases ranged from 0 to 19. ED patients suffer from social problems and poor psychological and physiological development as a result of unacceptable esthetics and abnormal function of orofacial structures. Oral rehabilitation thus becomes mandatory, although it is often difficult; particularly in pediatric patients.

  15. Biomonitoring on Integrated Multi-Thropic Aquaculture (IMTA) activities using macrobenthic mollusks on Tembelas Island, Kepulauan Riau Province

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    Abyan Syuja’, M.; Putro, Sapto P.; Muhammad, Fuad

    2018-05-01

    Macrobenthic mollusks are considered a good aquatic bioindicator for environmental biomonitoring. This study was conducted in Tembelas Island, Karimun District, Kepulauan Riau Province, at 103°29’47’ - 103°29’90’ BT and 0.991°16’63’ - 0.989°06’37’ LS. The objective was to compare the quality of water and sediment at Integrated Multitrophic Aquaculture (IMTA) sites, monocultural sites and reference sites using the community structure of mollusks, and to assess the potential of mollusks as bioindicator. The research was conducted in June and October of 2016. Data of abiotic parameters included the composition of sediment substrate and DO, pH, salinity, temperature and turbidity. The sampling procedure was performed with the use of an Ekman Grab. The animals obtained were 1 mm size-sieved and were fixed using 10% formalin for further analysis. In total there were 49 species and 2 classes obtained (Gastropods and Bivalves). The most prevalent genus were Costoanachis sp (Fam. Collumbellidae) and Anodontia sp (Fam. Lucinidae). The values of outcomes from indices used were considered low, ranging from 1.34 to 2.54 for diversity (H’), from 0.86 to 0.96 for evenness, and from 0.11 to 0.31 for dominance (C). Further analysis consisting of a multivariate approach and graphical methods of cluster analysis and Non- Metric Multidimensional Scaling (NMDS) ordination showed differences between the assemblages in the aquacultural areas and the reference area. However, the tendency of grouping the stations between IMTA and monocultural sites did not occur, implying both sites have relatively similar structures of macrobenthic mollusks. Based on the data obtained, sediment composition at the KJABB IMTA, monocultural areas and the reference area were dominated by silt, with the highest value being 92.06% found in the KJABB IMTA area. Clay, silt and nitrogen levels were the abiotic factors influencing structure of macrobenthic mollusks (BIO-ENV-Primer 6.1.5; r = 0.571).