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https://www.readbyqxmd.com/read/28709677/functional-and-esthetic-restorative-treatment-with-preheated-resins-in-a-patient-with-ectodermic-dysplasia-a-clinical-report
#1
José M Olivares, Alonso Hidalgo, Juan P Pavez, Dafna Benadof, Roberto Irribarra
Ectodermal dysplasia is a heterogeneous genetic condition affecting 1.6 to 22 per 100000 people. Oral manifestations associated with this condition include hyperdontia, hypodontia, microdontia, and conical teeth. Traditional treatment consists of a combination of orthodontic and rehabilitation therapies. The initial treatment stage uses removable prostheses and interim crowns for long periods, thus increasing risks for developing secondary caries. This clinical report describes the use of direct composite resin bonding with preheated compactable resins applied to vacuum-formed trays filled with clear silicone...
July 11, 2017: Journal of Prosthetic Dentistry
https://www.readbyqxmd.com/read/28691455/adult-syndrome-dental-features-of-a-very-rare-condition
#2
L Azzi, V Maurino, R Vinci, F Croveri, A Boggio, A Tagliabue, J Silvestre-Rangil
The Acro-Dermato-Ungual-Lacrimal-Tooth syndrome (ADULT syndrome) is one of the rarest ectodermal dysplasias and it is associated with several malformations involving especially the limbs. The most clinical features are the presence of ectrodactyly, syndactyly, hypermelanosis or multiple lentigines, onhycodysplasia, abnormalities in the lacrimal duct, recurrent conjuntivitis, photophobia, mammarian hypoplasia, hypotrichosis and frontal alopecia, hypohydrosis, cutaneous photosensitivity, nasal bridge prominence, exfoliative dermatitis and xerosis...
April 2017: Journal of Biological Regulators and Homeostatic Agents
https://www.readbyqxmd.com/read/28612428/outcomes-and-prognostic-factors-that-influence-the-success-of-tooth-autotransplantation-in-children-and-adolescents
#3
Vasiliki Kafourou, Huei Jinn Tong, Peter Day, Nadine Houghton, R James Spencer, Monty Duggal
BACKGROUND/AIM: Tooth autotransplantation has been advocated for replacement of missing teeth or teeth that are unsuitable for restoration. The aim of this study was to investigate the outcomes and prognostic factors that influenced the success of tooth transplantation in a paediatric population. MATERIALS AND METHODS: Data were extracted from the records of 75 patients (89 teeth). Demographic and prognostic factors were recorded and analysed for the clinical and radiographic outcomes for periodontal ligament (PDL) and pulp healing of transplanted teeth...
June 14, 2017: Dental Traumatology: Official Publication of International Association for Dental Traumatology
https://www.readbyqxmd.com/read/28589944/cerebellar-hypoplasia-with-endosteal-sclerosis-is-a-polr3-related-disorder
#4
Jamal Ghoumid, Florence Petit, Odile Boute-Benejean, Frédéric Frenois, Maryse Cartigny, Clémence Vanlerberghe, Thomas Smol, Roseline Caumes, Nicolas de Roux, Sylvie Manouvrier-Hanu
CHES (cerebellar hypoplasia with endosteal sclerosis) syndrome (OMIM#213002) associates hypomyelination, cerebellar atrophy, hypogonadism and hypodontia. So far, only five patients have been described. The condition is of neonatal onset. Patients have severe psychomotor delay and moderate to severe intellectual disability. Inheritance is assumed to be autosomal recessive due to recurrence in sibs, consanguinity of parents and absence of vertical transmission. CHES syndrome is reminiscent of 4H-leukodystrophy, a recessive-inherited affection due to variations in genes encoding subunits of the RNA polymerase III (POLR3A-POLR3B-POLR1C)...
August 2017: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/28584496/marfan-syndrome
#5
T Sivasankari, Philips Mathew, Ravi David Austin, Sakthi Devi
Marfan syndrome (MFS) is the autosomal dominant-inherited multisystem connective-tissue disorder, with a reported incidence of 1 in 10,000 individuals and equal distribution in both genders. The main clinical manifestation of this disorder consists of an exaggerated length of the upper and lower limbs, hyperlaxity, scoliosis, alterations in the cardiovascular and pulmonary systems, and atypical bone overgrowth. Orofacial manifestations such as high-arched palate, hypodontia, long narrow teeth, bifid uvula, mandibular prognathism, and temporomandibular disorders are also common...
January 2017: Journal of Pharmacy & Bioallied Sciences
https://www.readbyqxmd.com/read/28569451/treatment-outcomes-and-assessment-of-oral-health-related-quality-of-life-in-treated-hypodontia-patients
#6
Motasum Abu-Awwad, Kenneth Hemmings, Saja Mannaa, Daljit Gill, Akil Gulamali, Aviva Petrie
OBJECTIVES: 1. Assess treatment outcomes and oral health related quality of lifeM (OHRQoL) of hypodontia patients after completing dental treatment at the Eastman Dental Hospital and compare it with British public norms. 2. Assess factors which have an association with patients' reported OHRQoL. METHODS: Hypodontia patients who finished their treatment completed a 16-item questionnaire (UK oral health related quality-of-life measure), a list of four questions about their satisfaction with treatment received and a clinical assessment using modified USPHS criteria...
March 2017: European Journal of Prosthodontics and Restorative Dentistry
https://www.readbyqxmd.com/read/28561206/4h-leukodystrophy-a-brain-magnetic-resonance-imaging-scoring-system
#7
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...
June 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28553001/hypohidrotic-ectodermal-dysplasia-breastfeeding-complications-due-to-impaired-breast-development
#8
Mandy Wahlbuhl-Becker, Florian Faschingbauer, Matthias W Beckmann, Holm Schneider
Background X-linked hypohidrotic ectodermal dysplasia (XLHED), the most common form of ectodermal dysplasia, is caused by mutations in the gene EDA. While only affected men develop the full-blown clinical picture, females who are heterozygous for an EDA mutation often also show symptoms such as hypodontia, hypotrichosis and hypohidrosis. These women may also suffer from malformations of the mammary gland which represent not just a cosmetic problem but can limit their breastfeeding capability. This paper summarizes the findings of the first systematic study on the impact of hypohidrotic ectodermal dysplasia on breastfeeding...
April 2017: Geburtshilfe und Frauenheilkunde
https://www.readbyqxmd.com/read/28535361/maternal-smoking-during-pregnancy-is-associated-with-offspring-hypodontia
#9
A H Al-Ani, J S Antoun, W M Thomson, T R Merriman, M Farella
Little is known about environmental risk factors for hypodontia. The objective of this study was to investigate the association between hypodontia and common environmental risk factors, such as maternal smoking and alcohol and caffeine consumption during pregnancy. Eighty-three hypodontia cases with 1 or more missing permanent lateral incisors and/or 1 or more missing premolars were enrolled in this clinic-based case-control study. Some 253 controls with no missing teeth were frequency matched to cases by age and sex...
May 1, 2017: Journal of Dental Research
https://www.readbyqxmd.com/read/28509361/prevalence-of-dental-developmental-anomalies-of-permanent-teeth-in-children-and-their-influence-on-esthetics
#10
Anita Fekonja
OBJECTIVE: The aim of this study was to determine the prevalence of dental developmental anomalies in permanent teeth and their influence on esthetics. MATERIALS AND METHODS: The records of 473 subjects, which comprised of orthopantomograms, clinical examination, and anamnestic data, were explored for dental developmental anomalies. Subjects with dental anomalies completed the modified questionnaire. Data on reasons for seeking the treatment as well as factors affecting the patients' satisfaction were collected...
May 16, 2017: Journal of Esthetic and Restorative Dentistry
https://www.readbyqxmd.com/read/28459997/hypomorphic-mutations-in-polr3a-are-a-frequent-cause-of-sporadic-and-recessive-spastic-ataxia
#11
Martina Minnerop, Delia Kurzwelly, Holger Wagner, Anne S Soehn, Jennifer Reichbauer, Feifei Tao, Tim W Rattay, Michael Peitz, Kristina Rehbach, Alejandro Giorgetti, Angela Pyle, Holger Thiele, Janine Altmüller, Dagmar Timmann, Ilker Karaca, Martina Lennarz, Jonathan Baets, Holger Hengel, Matthis Synofzik, Burcu Atasu, Shawna Feely, Marina Kennerson, Claudia Stendel, Tobias Lindig, Michael A Gonzalez, Rüdiger Stirnberg, Marc Sturm, Sandra Roeske, Johanna Jung, Peter Bauer, Ebba Lohmann, Stefan Herms, Stefanie Heilmann-Heimbach, Garth Nicholson, Muhammad Mahanjah, Rajech Sharkia, Paolo Carloni, Oliver Brüstle, Thomas Klopstock, Katherine D Mathews, Michael E Shy, Peter de Jonghe, Patrick F Chinnery, Rita Horvath, Jürgen Kohlhase, Ina Schmitt, Michael Wolf, Susanne Greschus, Katrin Amunts, Wolfgang Maier, Ludger Schöls, Peter Nürnberg, Stephan Zuchner, Thomas Klockgether, Alfredo Ramirez, Rebecca Schüle
Despite extensive efforts, half of patients with rare movement disorders such as hereditary spastic paraplegias and cerebellar ataxias remain genetically unexplained, implicating novel genes and unrecognized mutations in known genes. Non-coding DNA variants are suspected to account for a substantial part of undiscovered causes of rare diseases. Here we identified mutations located deep in introns of POLR3A to be a frequent cause of hereditary spastic paraplegia and cerebellar ataxia. First, whole-exome sequencing findings in a recessive spastic ataxia family turned our attention to intronic variants in POLR3A, a gene previously associated with hypomyelinating leukodystrophy type 7...
June 1, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28447407/wiedemann-rautenstrauch-syndrome-a-phenotype-analysis
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
REVIEW
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
#20
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
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