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1.
Arch Oral Biol ; 162: 105961, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38547646

RESUMO

OBJECTIVE: Dental agenesis data in modern and premodern sub-Saharan Africans are presented by region, West, Central, East, and South, and by sex. Beyond characterizing the anomaly, comparisons are made with other populations and future work is encouraged. The findings should be of use to dental clinicians and anthropologists. METHODS: Agenesis of the UI2, LI1, UP2, LP2, UM3, and LM3 was recorded in 52 discrete samples of mainly skeletal dentitions (n = 2162) from across the subcontinent. After dividing into temporal categories, regional pooling was effected for adequate sample sizes across the vast geographic area. Only adults were included to record M3 status. Analyses included 95% confidence intervals and chi-square comparisons by region and sex. RESULTS: Of 1668 modern individuals 2.3% have UI2-LP2 agenesis (CI 1.6-3.1%). Regional and sex differences are non-significant, though females are most affected. For M3s it is 7.0% (5.7-8.4%), with the Central region sample differing significantly from the East and South. Females again have greater prevalence, with the difference in the West significant. UI2-LP2 agenesis affects 0.6% of 494 premodern individuals (0.1-1.8%), while M3 agenesis is 8.5% (6.1-11.5%). None of these differences are significant. CONCLUSIONS: Rates are toward the low end of global ranges, including 0.0-12.6% for UI2-LP2 from case reports, and 5.3-56.0% for M3 agenesis. With exceptions, generally insignificant inter-region differences imply that rates reasonably represent sub-Saharan peoples overall. Results will be of interest to anthropologists, but those related to risk factors, patterning, and prevalence may assist clinicians in tailoring treatment, while informing patients how this anomaly differs by population ancestry.


Assuntos
Anodontia , Adulto , Humanos , Masculino , Feminino , Prevalência , Anodontia/epidemiologia , Dentição Permanente , Caracteres Sexuais , África Subsaariana/epidemiologia
2.
Cureus ; 15(10): e46961, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-38021739

RESUMO

Tooth agenesis, one of the most common developmental defects in humans, not only impairs oral function but can also lead to craniofacial deformities. Bibliometric analysis can reveal significant shifts in research and publishing trends within specific fields. This study aims to provide a comprehensive overview of the research hotspots in tooth agenesis and predict future trends through bibliometric analysis. We searched for English-language publications related to tooth agenesis from 2001 to 2021 on the Web of Science. The publications were limited to original and review articles, and bibliometric parameters such as publication year, country, institution, author, journal, citations, and keywords were extracted and analyzed using VOSviewer, Microsoft Excel 2010, and CiteSpace. A total of 2,287 papers were ultimately selected. The results show that the USA holds a leading position in the field of tooth agenesis research. A total of 9,803 authors participated in these studies, with Alexandre R Vieira from the USA being the most prolific and most cited author. This study indicates that multidisciplinary management has become the consensus first choice for treating dental agenesis. Gene mutations related to tooth agenesis continue to be a research hotspot attracting scholarly attention. Exploring the relationship between tooth agenesis and cancer may be a future research direction. These findings contribute to potential collaborations among experts in future research on the genetic causes of tooth agenesis and tumor development and to assist the scientific community by identifying research gaps in this field.

3.
J Pharm Bioallied Sci ; 15(Suppl 1): S795-S796, 2023 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-37654255

RESUMO

Ectopic eruption of the tooth is a developmental disturbance where the tooth does not erupt in a normal position. This may be due to arch length deficiency or due to, delayed or premature exfoliation of deciduous teeth, or a combination of the above. A total or complete absence of teeth is called true anodontia; if one or more teeth are missing, it is called partial anodontia. It can be syndromic or non-syndromic in occurrence. Here, we present a rare case of non-syndromic partial anodontia and ectopic eruption of permanent mandibular canine in a female patient.

4.
Int J Clin Pediatr Dent ; 16(4): 649-655, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37731806

RESUMO

Background: Pediatric dentists face significant challenges when treating a child with anodontia. Early intervention is essential to ensure the normal physiological and psychological development while also promoting normal jaw development. Case Report: This report describes a 4-year follow-up case of a child with complete anodontia. Prosthetic rehabilitation during the primary and mixed dentition period improved the facial esthetics, self-esteem, speech, and masticatory function of the child. Conclusion: This case report summarizes the treatment procedure, challenges faced in clinical management and the various prosthetic options with its advantages and disadvantages for a child with anodontia. How to cite this article: Kaikure MK, Shetty K, Menezes R, et al. Esthetic and Functional Rehabilitation of a Child with Complete Anodontia in Primary and Mixed Dentition Stage: A Case Report with 4-year Follow-up. Int J Clin Pediatr Dent 2023;16(4):649-655.

5.
Clin Cosmet Investig Dent ; 15: 133-141, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37576744

RESUMO

Ectodermal dysplasia (ED) is an inherited disorder that affects the ectoderm of a developing embryo and impacts structures that originate from it. It typically presents as a triad of missing teeth (anodontia/hypodontia), sparse hair (atrichosis/hypotrichosis), and lack of sweat glands (hypohidrosis), often accompanied by nail dystrophy and palmoplantar hyperkeratosis. There are two main types of this condition: X-linked anhidrotic or hypohidrotic and hidrotic (autosomal type). The oral manifestation of ED may include anodontia or hypodontia, with or without cleft lip and palate. Tooth loss leads to a decrease in the height of the alveolar ridges, resulting in a reduction of the vertical dimension of the lower face, disappearance of the vermilion border, and prominent lips. As a result, the affected person's face may resemble that of an elderly individual. The current case report aims to illustrate the prosthodontic rehabilitation of 16-year-old male patient with ectodermal dysplasia and complete anodontia, visiting Dentistry Teaching Hospital, Kabul, Afghanistan. Fortunately, this patient was acceptably managed with the collaboration of several disciplines.

6.
BMC Pediatr ; 23(1): 322, 2023 06 24.
Artigo em Inglês | MEDLINE | ID: mdl-37355575

RESUMO

BACKGROUND: Dental agenesis (DA) in the permanent dentition is one of the most common dental anomalies, with a prevalence up to 2-10%. Therefore, the aim of this retrospective study was to investigate the prevalence and therapeutic treatment of DA in healthy children (HC) compared to children with systemic disease or congenital malformation (SD/CM). METHODS: Out of 3407 patients treated at the Department of Paediatric Dentistry of the Justus Liebig University Giessen (Germany) between January 2015 and December 2020, a total of 1067 patients (594 female, 473 male) aged between 4.5 and 18 years were included in this study due to DA. Besides the patients' general medical history and therapeutic treatments, panoramic radiographs were analysed. RESULTS: In contrast to the HC group with 9.7% DA, the SD/CM group showed a significantly higher prevalence of DA (19.8%; p < 0.05). The latter group was further classified into children with ectodermal dysplasia (4.4%), down syndrome (8.2%), cleft lip and palate (4.4%), intellectual disability/developmental delay (16.4%), and other genetic/organic diseases without intellectual disability (45.9%). Regarding therapeutic treatments, the HC group (59.5%) was significantly more often treated with an orthodontic gap opening compared to the SD/CM group (42.6%; p < 0.05), followed by orthodontic gap closing 36.5% in the HC group and 22.9% in the SD/CM group (p < 0.05), whereas no treatment was predominantly performed in the SD/CM group (37.7%) compared to the HC group (4%; p < 0.05). Furthermore, 50% in the SD/CM group required general anaesthesia for therapeutic treatment (vs. 8.1% in the HC group; p < 0.05). CONCLUSIONS: Children with SD/CM suffered more often from DA compared to HC that underlines multi- and interdisciplinary treatment of utmost importance. Furthermore, due to intellectual disability, common treatment methods can be complicated by insufficient compliance. This fact underlines the importance of an early attempt to establish the necessary cooperation enabling children with SD/CM to receive therapy.


Assuntos
Anodontia , Fenda Labial , Fissura Palatina , Deficiência Intelectual , Humanos , Masculino , Criança , Feminino , Pré-Escolar , Adolescente , Fenda Labial/epidemiologia , Estudos Retrospectivos , Anodontia/epidemiologia , Anodontia/terapia , Fissura Palatina/complicações , Prevalência , Deficiência Intelectual/complicações
7.
Int J Clin Pediatr Dent ; 16(1): 147-152, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37020764

RESUMO

Hartsfield syndrome is a rare and unique clinical combination of ectrodactyly and holoprosencephaly (HPE) with or without cleft lip and palate, as well as various additional characteristics. Although several genes responsible for HPE and ectrodactyly have been identified, the genetic origin of Hartsfield syndrome remains unknown, as there are few reports in the literature. The objective of this case report is to present dentofacial abnormalities in an 11-year-old boy with Hartsfield syndrome, who presented mental retardation, hearing loss, bilateral hand and foot ectrodactyly, HPE, and solitary median maxillary central incisor (SMMCI) besides 12 dental ageneses. How to cite this article: P Reis PM, Faber J, O Rosa JS, et al. Solitary Median Maxillary Central Incisor in Hartsfield Syndrome: A Case Report. Int J Clin Pediatr Dent 2023;16(1):147-152.

8.
J Stomatol Oral Maxillofac Surg ; 124(4): 101425, 2023 09.
Artigo em Inglês | MEDLINE | ID: mdl-36796638

RESUMO

INTRODUCTION: the aim of this study was to report and assess the management and implant rehabilitation of oligodontia patients since its recognition in 2012 by french authorities in the nomenclature. MATERIAL AND METHODS: we conducted a retrospective study in the Maxillofacial Surgery and Stomatology Department of the Lille University Hospital between January 2012 and May 2022. Patients had to present an oligodontia recognized under the ALD31 in adulthood, and to have benefited from a pre-implant/implant surgical treatment in the unit. RESULTS: a total of 106 patients were included in the study. The mean number of agenesis was 12 per patient. The most missing teeth are the ones at the end of the series. After a pre-implant surgery phase including orthognathic surgery and/or bone grafting, 97 patients benefited from implant placements. The mean age for this phase was 19.38. A total of 688 implants were placed. The median number of implants placed was 6 per patient and 5 patients presented implant failures after or during the osseointegration phase (16 implants lost). The implant success rate was 97.6%. 78 patients benefited from rehabilitations by fixed implant-supported prostheses and 3 by implant-supported mandibular removable prostheses. DISCUSSION: The described care pathway seems to us adapted to the patients followed in our department, with good functional and esthetic results. It would be necessary to evaluate on a national scale to adapt the management process.


Assuntos
Anodontia , Implantes Dentários , Humanos , Implantação Dentária Endóssea/métodos , Estudos Retrospectivos , Prótese Dentária Fixada por Implante , Anodontia/epidemiologia , Anodontia/cirurgia , Anodontia/reabilitação
9.
BMC Oral Health ; 23(1): 37, 2023 01 23.
Artigo em Inglês | MEDLINE | ID: mdl-36691053

RESUMO

BACKGROUND: Sex dimorphism has been implicated in oral health differences and the pathogenesis of oral diseases, such as tooth agenesis, periodontal disease, dental caries, and tooth loss. Tooth agenesis (TA) is one of the most common developmental anomalies in humans, and its prevalence and patterns are different across ethnic groups. The aim of this study was to investigate the phenotypes and sex-associated patterns of nonsyndromic tooth agenesis (TA) in Thai dental patients. METHODS: One thousand ninety panoramic radiographs were examined. One hundred and one subjects (37 males, 64 females, 15-20 years-old) with nonsyndromic TA were evaluated. Differences in TA prevalence between groups were analyzed using the chi-square or Fisher exact test. RESULTS: The TA prevalence, excluding third molars, was 9.3% and more frequently found in the mandible compared with the maxilla. The maxilla demonstrated a higher prevalence of first premolar agenesis than the mandible (P = 0.012), while the mandible had a higher prevalence of second premolar agenesis than the maxilla (P = 0.031). There were significantly more males missing one tooth than females, however, there were more females missing two or more teeth than males (P = 0.042). A missing maxillary left lateral incisor was significantly more frequent in males (P = 0.019), while a missing mandibular right lateral incisor was more frequent in females (P = 0.025). In females, the pattern of two mandibular lateral incisors agenesis was the most common and significantly present in females more than males (P = 0.015). In contrast, the pattern of one mandibular left lateral incisor agenesis was only observed in males and significantly found in males more than females (P = 0.047). CONCLUSIONS: We demonstrate sex-associated differences in nonsyndromic tooth agenesis. The prevalence of single tooth agenesis was higher in males, while that of two or more teeth agenesis was higher in females. We found different patterns of lateral incisor agenesis between males and females.


Assuntos
Anodontia , Cárie Dentária , Anormalidades Dentárias , Adolescente , Adulto , Feminino , Humanos , Masculino , Adulto Jovem , Anodontia/epidemiologia , Cárie Dentária/patologia , Dentição Permanente , Maxila/patologia , Prevalência , Caracteres Sexuais , Anormalidades Dentárias/epidemiologia , Má Oclusão
10.
BMC Oral Health ; 23(1): 10, 2023 01 09.
Artigo em Inglês | MEDLINE | ID: mdl-36624442

RESUMO

BACKGROUND: Progressive familial intrahepatic cholestasis is a heterogeneous group of disorders, leading to intrahepatic cholestasis, with the possibility of chronic liver failure and biliary cirrhosis. Oligodontia is either the manifestation of a specific syndrome or is non-syndromic. To the best of our knowledge, this is the first case report of type 3 progressive familial intrahepatic cholestasis and concurrent oligodontia, craniosynostosis, dens in dente, taurodontism, and delayed permanent dentition in the medical and dental literature. CASE PRESENTATION: We present the dental and medical histories and comprehensive dental management of a girl with type 3 progressive familial intrahepatic cholestasis and several dental anomalies, who was referred to a dental clinic due to severe dental caries and pain. CONCLUSION: Our findings suggest that PFIC with manifestations as oligodontia, craniosynostosis, dens in dente, taurodontism, and delayed permanent dentition, might indicate an unknown syndrome; otherwise, the craniofacial anomalies are the manifestations of an independent disease coinciding with PFIC. Moreover, our case is a good example of the importance of timely medical and dental care in confining further health-related complications. The patient was able to ingest without any pain or discomfort after receiving proper dental management.


Assuntos
Colestase Intra-Hepática , Dens in Dente , Cárie Dentária , Feminino , Humanos , Criança , Cárie Dentária/complicações , Cárie Dentária/terapia , Colestase Intra-Hepática/complicações , Colestase Intra-Hepática/genética , Assistência Odontológica
11.
J West Afr Coll Surg ; 13(4): 106-110, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-38449548

RESUMO

Dental agenesis is the most common developmental aberration in humans, and it is typically linked with a variety of other oral anomalies. Oligodontia, anodontia, and hypodontia are the words used in the literature to denote missing teeth. Although tooth agenesis is not a serious public health problem, it can cause speech, masticatory, aesthetic, and functional problems. A multidisciplinary approach is necessary to establish comprehensive treatment planning, counselling sessions, and frequent follow-ups, to obtain stable, functional, and psychological rehabilitation, which will eventually improves the patient's quality of life. In this case study, after considering the young age and growth of the patient and also his financial status, a conservative approach to rehabilitate the non-syndromic oligodontia patient with a 12-year follow-up and fabrication of maxillary overlay denture and mandibular conventional complete denture has been discussed.

12.
Braz. j. oral sci ; 22: e237699, Jan.-Dec. 2023. ilus
Artigo em Inglês | LILACS, BBO - Odontologia | ID: biblio-1437668

RESUMO

Aim: Dental number anomalies are a group of congenital developmental disorders divided into two groups supernumerary and missing teeth. This study was conducted to investigate the prevalence of numeric dental anomalies using panoramic images in patients referred to the Hamadan Dental Faculty. Methods: In this cross-sectional study, 2,197 panoramic radiographs of patients aged 6-49 years were evaluated. These anomalies are divided into two groups: 1) Supernumerary teeth, including Mesiodens, Distodens, and Peridens, and 2) Missing teeth, including Hypodontia, Oligodontia, and Anodontia. A Chi-square test was performed to assess the relationship between the anomalies. Data analysis was performed using SPSS 16, in which P-value < 0.05 was considered the statistical significance level. Results: Of 736 males (32.2%) and 1548 females (67.8%) in this study, 32 (4.3%) and 55 cases (3.8%) had supernumerary teeth, respectively. The prevalence of supernumerary teeth was 0.3%, 0.5%, and 0.6% in males and 0.2%, 1% and 1.2% in females for mesiodens, distodens, and peridens, respectively. Also, 243 males (10.6%) and 655 females (28.6%) had missing teeth anomalies. Hypodontia in the maxilla was the most common anomaly in both genders, while mesiodens was the least common. Conclusion: Hypodontia was the most common anomaly, followed by peridens; the least common anomaly was mesiodens. The prevalence of supernumerary teeth was greater in males, though the difference was not statistically significant. In comparison, females had a greater prevalence of missing teeth


Assuntos
Humanos , Masculino , Feminino , Criança , Adolescente , Adulto , Pessoa de Meia-Idade , Anormalidades Dentárias/epidemiologia , Radiografia Panorâmica , Anodontia
13.
J Ayub Med Coll Abbottabad ; 34(4): 895-897, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36566424

RESUMO

Ectodermal Dysplasia (ED) is a rare genetic condition characterized by the involvement of ectoderm derivatives such as hair, nail, sweat glands, and teeth. It has many variants, but the two most common ones are hypohidrotic/anhidrotic ectodermal dysplasia and hidrotic ectodermal dysplasia. Herein, we present a case of a 20-year-old female with hypohidrotic ectodermal dysplasia who had anodontia, hypohidrosis, and hypotrichosis, and her condition went unrecognized until she was seen for gastroenteritis at a tertiary care center. This case report will help spread education and awareness regarding such a rare and under-recognized condition. Early diagnosis and intervention help improve the quality of life.


Assuntos
Anodontia , Displasia Ectodérmica Anidrótica Tipo 1 , Displasia Ectodérmica , Humanos , Feminino , Adulto Jovem , Adulto , Displasia Ectodérmica Anidrótica Tipo 1/complicações , Displasia Ectodérmica Anidrótica Tipo 1/diagnóstico , Qualidade de Vida , Displasia Ectodérmica/complicações , Displasia Ectodérmica/diagnóstico , Anodontia/etiologia
14.
Diagnostics (Basel) ; 12(12)2022 Nov 30.
Artigo em Inglês | MEDLINE | ID: mdl-36553004

RESUMO

Pseudo-anodontia consists in the clinical, not radiographic, absence of teeth, due to failure in their eruption. It has been reported as part of an extremely rare syndrome, named GAPO syndrome. Pseudo-hypoparathyroidism type 1a (PHPT-1a) is a rare condition, characterized by resistance to the parathyroid hormone (PTH), as well as to many other hormones, and resulting in hypocalcemia, hyperphosphatemia, and elevated PTH. We report here the case of a 32-year-old woman with a long-standing history of non-treated hypocalcemia, in the context of an undiagnosed PHPT-1a. She had an intellectual disability, showed clinical features of the Albright hereditary osteodystrophy (AHO) and presented signs of multiple hormone resistances. She received treatment for seizures since the age of six. Examination of her mouth revealed a complete absence of teeth. Treatment of hypocalcemia and hormone deficiencies were started only at 29 years of age. Genetic testing demonstrated the presence of a frameshift variant in the GNAS gene in the proband as well as in her mother. A Single Nucleotide Polymorphism (SNP) array analysis failed to demonstrate pathogenic copy number variants (CNVs) but showed several regions with loss of heterozygosity (LOHs) for a final percentage of 1.75%, compatible with a fifth degree of relationship. Clinical exome sequencing (CES) ruled out any damaging variants in all the teeth agenesis-related genes. In conclusion, although we performed an extensive genetic analysis in search of possible additional gene alterations that could explain the presence of the peculiar phenotypic characteristics observed in our patient, we could not find any additional genetic defects. Our results suggest that the association of genetically confirmed PHPT-1a and complete pseudo-anodontia associated with persistent patchy alopecia areata is a new additional nonclassical feature related to the GNAS pathogenic variant.

15.
J Indian Prosthodont Soc ; 22(3): 305-309, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36511063

RESUMO

Treatment of pediatric patients with partial anodontia is a challenge requiring interdisciplinary approach. Growth period, reduced vertical dimension, microdontia, and unacceptable esthetics present difficulties at various stages of prosthetic rehabilitation. Congenital absence of teeth impairs the nutritional status of the growing child and causes a psychological setback. This article describes the prosthetic management of a patient suffering from partial anodontia done over a period of 10 years. Considering the age and psychological and financial requirements of the patient, removable and fixed prostheses were fabricated at different phases of the treatment. The ultimate aim was restoration of function, improvement of esthetics, and overall psychological upliftment of the patient which was achieved by maxillary metal ceramic bridge and mandibular implant retained hybrid prosthesis.


Assuntos
Anodontia , Prótese Parcial Removível , Anormalidades Dentárias , Humanos , Criança , Estética Dentária , Anodontia/cirurgia , Anodontia/reabilitação , Dimensão Vertical
16.
Orphanet J Rare Dis ; 17(1): 376, 2022 10 17.
Artigo em Inglês | MEDLINE | ID: mdl-36253866

RESUMO

OBJECTIVE: The objective of this systematic review was to determine the orthodontic and dentofacial orthopedic treatments carried out in patients with ectodermal dysplasia to facilitate functional and aesthetic rehabilitation. METHODS: The systematic review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-analysis statement. We systematically searched PubMed, Web of Science, Scopus, Scielo, LILACS, EBSCOhost and Embase databases up to 6 January 2022. We included articles describing patients with any type of ectodermal dysplasia who received orthodontic or dentofacial orthopedic treatment to facilitate functional and aesthetic oral rehabilitation. The search was not restricted by language or year of publication. The quality of the studies was assessed using the Joanna Briggs Institute Quality Assessment Scale of the University of Adelaide for case series and case reports. The review was registered at the University of York Centre for reviews (CRD42021288030). RESULTS: Of the initial 403 studies found, 29 met the inclusion criteria. After applying the quality scale, 23 were left for review-21 case reports and 2 case series. The initial age of patients ranged from 34 months to 24 years. Thirteen studies were on hypohidrotic and/or anhidrotic ectodermal dysplasia, of which two were X-chromosome linked. In one study, the patient had Wiktop syndrome, and in nine the type of ectodermal dysplasia was not specified. The duration of treatment was 7 weeks to 10 years. The treatments described were: fixed orthodontic appliances or simple acrylic plates designed for tooth movement, including leveling and aligning, closing of diastemata, retraction of impacted teeth in the dental arch; clear aligners; fixed and/or removable appliances for the correction of skeletal and/or dentoalveolar relationships; palatal expanders in combination with face masks for orthopedic traction of the maxilla; and orthognathic surgery. Only three studies provided cephalometric data. CONCLUSION: The level of evidence of the articles reviewed was low and most orthopedic and dentofacial orthodontic treatments described were focused on correcting dental malpositioning and jaw asymmetries and not on stimulating growth from an early age. Studies with greater scientific evidence are needed to determine the best treatment for these patients.


Assuntos
Displasia Ectodérmica Anidrótica Tipo 1 , Displasia Ectodérmica , Pré-Escolar , Displasia Ectodérmica/terapia , Humanos , Técnicas de Movimentação Dentária/efeitos adversos
17.
J. oral res. (Impresa) ; 11(5): 1-8, nov. 23, 2022. ilus, tab
Artigo em Inglês | LILACS | ID: biblio-1437179

RESUMO

Introduction: Ectodermal dysplasia is a rare genetic disorder that affects structures derived from ectoderm such as teeth, hair, nails, and sweat glands. Oligodontia is a common finding that affects the chewing, smiling and self- esteem of these pediatric patients. Case Report: We present a case report of a 7-years-old pediatric patient who consulted with his mother, showing the same condition. The intervention consisted of a removable prosthesis with satisfactory results. Conclusion: The X-linked hypohidrotic ectodermal dysplasia represents a challenge for a pediatric dentist who offers rehabilitation to the patient according to craniofacial development, age, socioeconomic status, and the number of missing teeth.


Introducción: La oligodoncia es un hallazgo común que afecta la masticación, la sonrisa y la autoestima de estos pacientes pediátricos. Case Report: Presentamos un reporte de caso de un paciente pediátrico de 7 años que consultó con su madre presentando la misma condición. La intervención consistió en una prótesis de remoción con resultados satisfactorios. Conclusión: La displasia ectodérmica hipohidrótica ligada al cromosoma X representa un desafío para un odontopediatra que ofrece rehabilitación al paciente de acuerdo con el desarrollo craneofacial, la edad, el nivel socioeconómico y el número de dientes faltantes.


Assuntos
Humanos , Masculino , Criança , Anormalidades Dentárias/terapia , Prótese Dentária/métodos , Displasia Ectodérmica Anidrótica Tipo 1/terapia , Anodontia/terapia , Qualidade de Vida , Resultado do Tratamento , Prótese Parcial Removível
18.
Acta Vet Scand ; 64(1): 23, 2022 Sep 06.
Artigo em Inglês | MEDLINE | ID: mdl-36068608

RESUMO

BACKGROUND: Hypohidrotic ectodermal dysplasia (HED) is a congenital syndrome of mammals affecting organs and tissues of ectodermal origin characterized by absence or hypoplasia of hair, teeth, and eccrine glands. The disorder has been reported in several species, including humans, mice, dogs and cattle, associated with variants in genes affecting the ectodysplasin pathway, including the X-linked ectodysplasin A (EDA) gene. Until now, nine pathogenic variants have been found in the bovine EDA gene. Here we report a novel variant in EDA in a crossbreed male Belgian Blue calf with HED, and provide an overview of the phenotypic and allelic heterogeneity of EDA-related forms of HED in cattle. CASE PRESENTATION: A 45-day-old male crossbreed British Blue calf was referred with congenital hypotrichosis, oligodontia and omphalitis. On histopathological examination of the nasal planum, nasolabial glands and ducts were not observed. The density of hair follicles was low, and they were small, with a predominance of telogen-phase hairs, and some serocellular crusts. The phenotype of the calf resembled that of HED. Whole-genome sequencing (WGS) was performed and revealed a 21,899 base-pair deletion encompassing the coding exon 2 of EDA, predicted to result in an altered transcript and aberrant protein. CONCLUSIONS: The clinicopathological and genetic findings were consistent with a case of X-linked HED. A very similar EDA deletion has been previously reported in a family of Holstein cattle with HED. The newly identified hemizygous EDA loss-of-function variant is certainly pathogenic and therefore is the genetic cause for the observed phenotype. This case report provides an additional example of the potential of WGS-based precise diagnostics in livestock species such as cattle to increase the diagnostic yield in rare diseases.


Assuntos
Doenças dos Bovinos , Displasia Ectodérmica Anidrótica Tipo 1 , Displasia Ectodérmica , Animais , Bovinos , Doenças dos Bovinos/genética , Displasia Ectodérmica/genética , Displasia Ectodérmica/veterinária , Displasia Ectodérmica Anidrótica Tipo 1/genética , Displasia Ectodérmica Anidrótica Tipo 1/patologia , Displasia Ectodérmica Anidrótica Tipo 1/veterinária , Ectodisplasinas/genética , Éxons , Masculino , Fenótipo
19.
J Prosthodont ; 31(9): 738-743, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-35946867

RESUMO

Children with ectodermal dysplasia and complete anodontia experience difficulties in oral rehabilitation because of the small arch size. A case of a 7-year-old boy, whose arch size (length and width) was 30-40% smaller than that of a male adult and who presented with difficulties in jaw relation recording using commercially available devices is described. A digital workflow involving a mini arch tracer was introduced. Primary impressions were made using three-dimensionally (3D) printed mini trays produced based on the patient's computed tomography images, and digital primary casts were obtained based on the scanned and reversed primary impressions. The final custom impression trays with mini tracing plates were designed based on the primary casts. In addition, the hand shank, retention plate, and retainers were placed on the designed custom trays and 3D-printed to produce an individual arch tracer system. In addition, two height-checking buckles were designed to help adjust the height of a tracing screw. Finally, the jaw relation of the patient was recorded and transferred, and a set of complete dentures were delivered, satisfying both the patient and his family.


Assuntos
Anodontia , Displasia Ectodérmica , Humanos , Criança , Masculino , Anodontia/reabilitação , Prótese Total , Displasia Ectodérmica/complicações , Técnica de Moldagem Odontológica
20.
J. health sci. (Londrina) ; 24(2): 119-122, 20220704.
Artigo em Inglês | LILACS-Express | LILACS | ID: biblio-1412624

RESUMO

Tooth agenesis or hypodontia is a developmental anomaly, where there is absence of one or more dental elements. The absence of maxillary lateral incisor can be uni or bilateral, has an incidence of 20% and is more frequent in females. Is directly related to the establishment of malocclusions and aesthetic dissatisfaction, so it is important to evaluate multidisciplinary parameters for a treatment decision. The objective of the present study is to establish, according to the present literature, clinical criteria for the therapeutic approach of opening or closing dental space of the Maxillary Lateral Incisor Agenesis. A systematized literature review was carried out in online databases: PubMed, Embase and Lilacs. Health descriptors and free terms were combined with Boolean operators to search: "Tooth Agenesis" or "Anodontia" and "Lateral Incisors" and "Treatment" and "Opening Space" or "Space Closure". Clinical studies that presented diagnostic criteria and therapeutic possibilities for Maxillary Lateral Incisor Agenesis, in English and without publication time frame, were included. During the clinical examination, skeletal, dental and periodontal parameters must be carefully observed. The intervention will always consist of a multidisciplinary approach, which may involve orthodontics, prosthetics, dentistry, periodontics and implantology for functional and aesthetic rehabilitation. The interceptive approach is based on early diagnosis and maintenance of the dental space, so orthodontic corrective treatment can be minimized later on. Late intervention includes closing the space with the reanatomization of adjacent dental elements or opening the space for implant and prosthesis installation. (AU)


A agenesia dentária ou hipodontia é uma anomalia de desenvolvimento, onde há ausência de um ou mais elementos dentários. A ausência do incisivo lateral superior possui uma incidência de 20%, mais frequente no sexo feminino e pode ser unilateral ou bilateral e estão diretamente relacionadas ao estabelecimento de maloclusões e insatisfação estética, assim, é importante que os profissionais saibam avaliar os parâmetros multidisciplinares para uma tomada de decisão sobre seu tratamento. O objetivo do presente estudo é estabelecer, segundo a literatura presente, os critérios clínicos para a tomada de decisão terapêutica de abertura ou fechamento de espaço para a Agenesia de Incisivo Lateral Superior. Foi realizada uma Revisão Sistematizada da Literatura nas bases de dados online: PubMed, Embase e Lilacs. Foram combinados descritores em saúde e termos livres com operadores boleanos para realizar a pesquisa nas bases de dados: "Tooth Agenesis" or "Anodontia" and "Lateral Incisors" and "Treatment" and "opening space" or "space closure". Foram incluídos estudos clínicos que apresentaram critérios para o diagnóstico e possibilidades terapêuticas para a Agenesia do Incisivo Lateral Superior, na língua inglesa e sem recorte temporal de publicação. Durante o exame clínico devem ser observados minuciosamente os parâmetros esqueléticos, dentários e periodontais. A intervenção sempre consistirá na abordagem multidisciplinar, podendo envolver ortodontia, prótese, dentística, periodontia e implantodontia para a reabilitação funcional e estética. A abordagem interceptativa consiste em diagnosticar precocemente e manter o espaço dentário, para que possa minimizar terapias ortodônticas corretivas de maior magnitude posteriormente. A intevenção tardia engloba o fechamento do espaço com a reanatomização dos elementos dentários adjacentes e abertura do espaço para instalação de implante e prótese. (AU)

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