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Hypodontia

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https://www.readbyqxmd.com/read/28447407/wiedemann-rautenstrauch-syndrome-a-phenotype-analysis
#1
Stefano Paolacci, Debora Bertola, José Franco, Shehla Mohammed, Marco Tartaglia, Bernd Wollnik, Raoul C Hennekam
Wiedemann-Rautenstrauch syndrome (WRS) is a neonatal progeroid disorder characterized by growth retardation, lipodystrophy, a distinctive face, and dental anomalies. Patients reported to date demonstrate a remarkable variability in phenotype, which hampers diagnostics. We performed a literature search, and analyzed 51 reported patients, using the originally reported patients as "gold standard." In 15 patients sufficient information and photographic evidence was available to confirm the clinical diagnosis. In 12 patients the diagnosis was suggestive but lack of data prevented a definite diagnosis, and in 24 patients an alternative diagnosis was likely...
April 26, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28430890/acceptability-comparison-between-hawley-retainers-and-vacuum-formed-retainers-in-orthodontic-adult-patients-a-single-centre-randomized-controlled-trial
#2
Mohammed Saleh, Mohammad Y Hajeer, Dieter Muessig
Background: Hawley retainers (HRs) and vacuum formed retainers (VFRs) are the most commonly used removable retainers in the orthodontic practice. Patients' cooperation in wearing these appliances is affected by the levels of discomfort and oral impairment. The evidence regarding their acceptably among orthodontic patients is limited. Aims: To compare the acceptability of HRs and VFRs over a 6-month period in a group of fixed orthodontic patients. Trial Design: Two-arm parallel-group randomized controlled trial...
April 19, 2017: European Journal of Orthodontics
https://www.readbyqxmd.com/read/28413597/concurrent-manifestation-of-clinical-hypodontia-and-blindness-a-case-report
#3
Jeremiah Robert Moshy, Karpal Singh Sohal, Mark Chindia
A case is reported of a 26-year-old blind man with hypodontia and multiple apparently underdeveloped impacted teeth. The patient reported that he had progressively developed visual impairment at the age of 11 years whence he became totally blind when he turned 12 years. The aim of this report is to open an academic and professional debate on the challenges of its definitive diagnosis and appropriate intervention.Blindness is not reported in any of the previously described syndromes; therefore, concurrent manifestation of "hypodontia, blindness, failure of eruption and digital lesions" can be proposed as a syndrome...
2017: Journal of Dental Research, Dental Clinics, Dental Prospects
https://www.readbyqxmd.com/read/28412234/premolar-autotransplantation-in-juvenile-dentition-quantitative-assessment-of-vertical-bone-and-soft-tissue-growth
#4
Inessa Michl, Dirk Nolte, Claudia Tschammler, Martin Kunkel, Robert Linsenmann, Johannes Angermair
OBJECTIVE: Premolar autotransplantation represents an effective therapeutic option for the treatment of juvenile dentition with either aquired or congenital hypodontia. The objective of this prospective clinical study was to quantitatively assess bone and soft tissue levels after autogenous premolar transplantation by clinical and radiographic parameters. STUDY DESIGN: In the study, 26 premolars were transplanted in 20 patients after traumatic tooth loss (n = 16) or congenital aplasia (n = 10) in the anterior maxilla...
February 22, 2017: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
https://www.readbyqxmd.com/read/28410841/contemporary-surgical-management-of-hypodontia
#5
REVIEW
J Breeze, M S Dover, R W Williams
Hypodontia is the term most commonly applied to the condition in which teeth congenitally fail to develop. Such cases differ from teeth that have been lost early or that have failed to erupt, although their initial presentation may be similar and therefore not recognised. The range of missing teeth and their physical and psychological results is large, and the difference in complexity in the management of a patient with isolated hypodontia compared with one with oligodontia or anodontia together with skeletal and orthognathic discrepancies should not be underestimated...
April 11, 2017: British Journal of Oral & Maxillofacial Surgery
https://www.readbyqxmd.com/read/28401166/hypodontia-an-update-on-its-etiology-classification-and-clinical-management
#6
REVIEW
Azza Husam Al-Ani, Joseph Safwat Antoun, William Murray Thomson, Tony Raymond Merriman, Mauro Farella
Hypodontia, or tooth agenesis, is the most prevalent craniofacial malformation in humans. It may occur as part of a recognised genetic syndrome or as a nonsyndromic isolated trait. Excluding third molars, the reported prevalence of hypodontia ranges from 1.6 to 6.9%, depending on the population studied. Most affected individuals lack only one or two teeth, with permanent second premolars and upper lateral incisors the most likely to be missing. Both environmental and genetic factors are involved in the aetiology of hypodontia, with the latter playing a more significant role...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28387468/sella-turcica-bridging-and-dental-anomalies-is-there-an-association
#7
Andrea Scribante, Maria Francesca Sfondrini, Marco Cassani, Danilo Fraticelli, Sergio Beccari, Paola Gandini
BACKGROUND: Sella turcica bridging (STB), or calcification of the interclinoid ligament of sella turcica, has been reported to be associated with some dental anomalies (palatal canine impaction and transposition). HYPOTHESIS OR AIM: The aim of the study was to find any association between canine impaction, hyperdontia or hypodontia and sellar dimensions or bridging. DESIGN: Lateral cephalometric radiographs from 78 patients with impacted canines, 68 with dental agenesis and 17 with hyperdontia were collected...
April 7, 2017: International Journal of Paediatric Dentistry
https://www.readbyqxmd.com/read/28376965/multidisciplinary-management-of-hypodontia
#8
Michael O'Sullivan, Brian O'Connell
Patients with hypodontia require a wide range of treatment, ranging from single tooth replacement to the restoration of multiple edentulous spaces in both arches. Treatment should involve an interdisciplinary team, as no dental speciality possesses the range of expertise required to optimally treat this patient population. This paper presents principles of treatment, key factors of assessment and multidisciplinary approaches to management of the hypodontia patient, including contributions from conservative dentistry...
February 28, 2017: Primary Dental Journal
https://www.readbyqxmd.com/read/28356682/dental-implants-in-children-a-multidisciplinary-perspective-for-long-term-success
#9
REVIEW
Nidhi Agarwal, Dipanshu Kumar, Ashish Anand, Surendrakumar Kaluram Bahetwar
Congenital hypodontia or trauma is a frequent cause of loss of teeth in children. The absence of teeth leads to loss of function and lack of normal alveolar growth, along with unpleasant esthetics that hamper the psychosocial development of the young child. Traditionally, the management of tooth loss in the young child is done by conservative means. None of those methods of treatment are completely satisfactory and have their own drawbacks. Dental implants in a young child would be an ideal mode of treatment for the absence of teeth...
July 2016: National Journal of Maxillofacial Surgery
https://www.readbyqxmd.com/read/28298625/periodontal-disease-and-fam20a-mutations
#10
Piranit Nik Kantaputra, Chotika Bongkochwilawan, Mark Lubinsky, Supansa Pata, Massupa Kaewgahya, Huei Jinn Tong, James R Ketudat Cairns, Yeliz Guven, Nipon Chaisrisookumporn
Enamel-renal-gingival syndrome (ERGS; OMIM #204690), a rare autosomal recessive disorder caused by mutations in FAM20A, is characterized by nephrocalcinosis, nephrolithiasis, amelogenesis imperfecta, hypoplastic type, gingival fibromatosis and other dental abnormalities, including hypodontia and unerupted teeth with large dental follicles. We report three patients and their families with findings suggestive of ERGS. Mutation analysis of FAM20A was performed in all patients and their family members. Patients with homozygous frameshift and compound heterozygous mutations in FAM20A had typical clinical findings along with periodontitis...
March 16, 2017: Journal of Human Genetics
https://www.readbyqxmd.com/read/28293528/intermediate-phenotype-between-adult-syndrome-and-eec-syndrome-caused-by-r243q-mutation-in-tp63
#11
Yuki Otsuki, Koichi Ueda, Chisei Satoh, Ryuta Maekawa, Koh-Ichiro Yoshiura, Sachiko Iseki
A patient who had ectrodactyly, dry skin, exfoliative dermatitis, and hypodontia with peg-shaped teeth, but not cleft lip and palate, is described. Ectrodactyly with a tooth anomaly is recognized in both acro-dermato-ungual-lacrimal-tooth (ADULT) syndrome and ectrodactyly-ectodermal dysplasia-cleft (EEC) syndrome. These 2 syndromes are caused by heterozygous mutations in the transcriptional factor gene p63. Mutation analysis of p63 gene showed a heterozygous mutation c.728G>A, p.Arg243Gln (previously referred to as R204Q) in the patient, but not in his parents...
December 2016: Plastic and Reconstructive Surgery. Global Open
https://www.readbyqxmd.com/read/28284207/dental-anomalies-prevalence-and-associations-between-them-in-a-large-sample-of-non-orthodontic-subjects-a-cross-sectional-study
#12
G Laganà, N Venza, A Borzabadi-Farahani, F Fabi, C Danesi, P Cozza
BACKGROUND: To analyze the prevalence and associations between dental anomalies detectable on panoramic radiographs in a sample of non-orthodontic growing subjects. METHODS: For this cross-sectional study, digital panoramic radiographs of 5005 subjects were initially screened from a single radiographic center in Rome. Inclusion criteria were: subjects who were aged 8-12 years, Caucasian, and had good diagnostic quality radiographs. Syndromic subjects, those with craniofacial malformation, or orthodontic patients were excluded and this led to a sample of 4706 subjects [mean (SD) age = 9...
March 11, 2017: BMC Oral Health
https://www.readbyqxmd.com/read/28249300/4h-leukodystrophy-a-brain-magnetic-resonance-imaging-scoring-system
#13
Suzanne Vrij-van den Bos, Janna A Hol, Roberta La Piana, Inga Harting, Adeline Vanderver, Frederik Barkhof, Ferdy Cayami, Wessel N van Wieringen, Petra J W Pouwels, Marjo S van der Knaap, Geneviève Bernard, Nicole I Wolf
4H (hypomyelination, hypodontia and hypogonadotropic hypogonadism) leukodystrophy (4H) is an autosomal recessive hypomyelinating white matter (WM) disorder with neurologic, dental, and endocrine abnormalities. The aim of this study was to develop and validate a magnetic resonance imaging (MRI) scoring system for 4H. A scoring system (0-54) was developed to quantify hypomyelination and atrophy of different brain regions. Pons diameter and bicaudate ratio were included as measures of cerebral and brainstem atrophy, and reference values were determined using controls...
March 1, 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28220267/chimpanzee-down-syndrome-a-case-study-of-trisomy-22-in-a-captive-chimpanzee
#14
Satoshi Hirata, Hirohisa Hirai, Etsuko Nogami, Naruki Morimura, Toshifumi Udono
We report a case of chimpanzee trisomy 22 in a captive-born female. Because chromosome 22 in great apes is homologous to human chromosome 21, the present case is analogous to human trisomy 21, also called Down syndrome. The chimpanzee in the present case experienced retarded growth; infantile cataract and vision problems, including nystagmus, strabismus, and keratoconus; congenital atrial septal defect; and hypodontia. All of these symptoms are common in human Down syndrome. This case was the second reported case of trisomy 22 in the chimpanzee...
February 21, 2017: Primates; Journal of Primatology
https://www.readbyqxmd.com/read/28207178/dental-development-in-down-syndrome-and-healthy-children-a-comparative-study-using-the-demirjian-method
#15
M S van der Linden, S Vucic, D J F van Marrewijk, E M Ongkosuwito
OBJECTIVE: In children with Down syndrome, the timing of dental eruption is important for orthodontics treatment planning. Aim of this study was to determine whether tooth eruption and development of the dentition in children with Down syndrome are impaired. MATERIAL AND METHODS: Dental development was scored on orthopantomograms (OPTs) of 95 children with Down syndrome. The dental age was determined at the left mandibular side according to the Demirjian method and by converting the assigned scores to the dental maturity score...
May 2017: Orthodontics & Craniofacial Research
https://www.readbyqxmd.com/read/28155232/pax9-gene-mutations-and-tooth-agenesis-a-review
#16
REVIEW
O Bonczek, V J Balcar, O Šerý
Paired box 9 (PAX9) is one of the best-known transcription factors involved in the development of human dentition. Mutations in PAX9 gene could, therefore, seriously influence the number, position and morphology of the teeth in an affected individual. To date, over 50 mutations in the gene have been reported as associated with various types of dental agenesis (congenitally missing teeth) and other inherited dental defects or variations. The most common consequence of PAX9 gene mutation is the autosomal-dominant isolated (non-syndromic) oligodontia or hypodontia...
February 2, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28139484/prevalence-of-dental-anomalies-in-deciduous-dentition-and-its-association-with-succedaneous-dentition-a-cross-sectional-study-of-4180-south-indian-children
#17
G Shilpa, Niraj Gokhale, Sreekanth Kumar Mallineni, Sivakumar Nuvvula
OBJECTIVE: The objective of this study was to estimate the prevalence of dental anomalies in primary dentition of Indian population. MATERIALS AND METHODS: This cross-sectional study was conducted on 4180 children in the age of 2-6 years. Anomalies were classified based on Kreiborg criteria. The term "double tooth" was used to avoid misinterpretation between gemination. and fusion. Patients having radiographs were also examined for associated dental anomalies in permanent dentition...
January 2017: Journal of the Indian Society of Pedodontics and Preventive Dentistry
https://www.readbyqxmd.com/read/28118512/prosthodontic-rehabilitation-for-a-patient-with-down-syndrome-a-clinical-report
#18
Nasser M Alqahtani, Hussain D Alsayed, John A Levon, David T Brown
Patients with Down syndrome can present with a variety of oral manifestations such as hypodontia, periodontal disease, premature tooth loss, reduced salivary flow, crowding of teeth in both arches, and decreased occlusal vertical dimension. The intellectual ability of people with Down syndrome varies widely. They present with a mild-to-moderate intellectual disability that restricts their ability to communicate and adjust to their environment, which can add complexity in the overall dental treatment. There is little information in the literature regarding the prosthodontic rehabilitation for patients with Down syndrome in combination with dental implant placement...
January 24, 2017: Journal of Prosthodontics: Official Journal of the American College of Prosthodontists
https://www.readbyqxmd.com/read/28105635/dental-and-extra-oral-clinical-features-in-41-patients-with-wnt10a-gene-mutations-a-multicentric-genotype-phenotype-study
#19
C Tardieu, S Jung, K Niederreither, M Prasad, S Hadj-Rabia, N Philip, A Mallet, E Consolino, E Sfeir, B Noueiri, N Chassaing, H Dollfus, M-C Manière, A Bloch-Zupan, F Clauss
WNT10A gene encodes a canonical wingless pathway signaling molecule involved in cell fate specification as well as morphogenetic patterning of the developing ectoderm, nervous system, skeleton, and tooth. In patients, WNT10A mutations are responsible for ectodermal-derived pathologies including isolated hypo-oligodontia, tricho-odonto-onycho-dermal dysplasia (TOODD) and Schöpf-Schulz-Passarge Syndrome (SSPS). Here we describe the dental, ectodermal, and extra-ectodermal phenotypic features of a cohort of 41 patients from 32 unrelated families...
January 20, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28097853/-clinical-and-molecular-study-in-a-family-with-autosomal-dominant-hypohidrotic-ectodermal-dysplasia
#20
Michele Callea, Francisco Cammarata-Scalisi, Colin E Willoughby, Sabrina R Giglio, Ilaria Sani, Sara Bargiacchi, Giovanna Traficante, Emanuele Bellacchio, Gianluca Tadini, Izzet Yavuz, Angela Galeotti, Gabriella Clarich
Hypohidrotic ectodermal dysplasia (HED) is a rare disease characterized by deficiency in development of structure derived from the ectoderm and is caused by mutations in the genes EDA, EDAR, or EDARADD. Phenotypes caused by mutations in these three may exhibit similar clinical features, explained by a common signaling pathway. Mutations in EDA gene cause X linked HED, which is the most common form. Mutations in EDAR and EDARADD genes cause autosomal dominant and recessive form of HED. The most striking clinical findings in HED are hypodontia, hypotrichosis and hypohidrosis that can lead to episodes of hyperthermia...
February 1, 2017: Archivos Argentinos de Pediatría
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