scholarly journals The Wiedemann-Rautenstrauch or Neonatal Progeroid Syndrome: Report of a Patient with Gingival Hyperplasia and Severe Anterior Open Bite

2020 ◽  
Vol 22 (1) ◽  
Author(s):  
Parisa Derakhshan ◽  
Farin Kiany

Introduction: The Wiedemann-Rautenstrauch syndrome (WRS) is a rare progeroid syndrome with an autosomal recessive pattern of inheritance. The main clinical features include severe intrauterine and postnatal growth failure, distinctive facial appearance, hydrocephaly, prominent scalp veins, absence of subcutaneous fat, sparse hair of the scalp, eyebrows, and eyelashes, generalized lipoatrophy, psychomotor delay, progressive neurological deterioration, and short life expectancy. Natal teeth and micrognathia are reported as the oral manifestations of this syndrome. Case Presentation: Here is the report of a 6.5-year-old female patient with clinical signs of WRS referred to the Department of Periodontology of Shiraz School of Dentistry with the chief complaint of toothache and gingival hyperplasia. Carious teeth were extracted, and excess gingival tissue was removed through a surgical procedure under general anesthesia. The patient was the first case with a concomitant severe anterior open bite. Conclusions: Therapies for WRS are symptomatic, requiring the coordinated efforts of a team of specialists. Dentists and oral surgeons can be recruited by pediatricians and family physicians to improve the quality of life of such patients.

2014 ◽  
Vol 19 (1) ◽  
pp. 19-25 ◽  
Author(s):  
Helder Baldi Jacob ◽  
Ary dos Santos-Pinto ◽  
Peter H. Buschang

INTRODUCTION: Due to the lack of studies that distinguish between dentoalveolar and basal changes caused by the Thurow appliance, this clinical study, carried out by the School of Dentistry - State University of São Paulo/Araraquara, aimed at assessing the dental and skeletal changes induced by modified Thurow appliance. METHODS: The sample included an experimental group comprising 13 subjects aged between 7 and 10 years old, with Class II malocclusion and anterior open bite, and a control group comprising 22 subjects similar in age, sex and mandibular plane angle. Maxillary/mandibular, horizontal/vertical, dental/skeletal movements (ANS, PNS, U1, U6, Co, Go, Pog, L1, L6) were assessed, based on 14 landmarks, 8 angles (S-N-ANS, SNA, PPA, S-N-Pog, SNB, MPA, PP/MPA, ANB) and 3 linear measures (N-Me, ANS-Me, S-Go). RESULTS: Treatment caused significantly greater angle decrease between the palatal and the mandibular plane of the experimental group, primarily due to an increase in the palatal plane angle. ANB, SNA and S-N-ANS angles significantly decreased more in patients from the experimental group. PNS was superiorly remodeled. Lower face height (ANS-Me) decreased in the experimental group and increased in the control group. CONCLUSIONS: The modified Thurow appliance controlled vertical and horizontal displacements of the maxilla, rotated the maxilla and improved open bite malocclusion, decreasing lower facial height.


2017 ◽  
Vol 41 (2) ◽  
pp. 161-165 ◽  
Author(s):  
Fundagül Bilgiç ◽  
İbrahim Erhan Gelgör

Introduction: Malocclusion is one etiological factor of temporomandibular joint disorder (TMD). This study investigates the prevalence of TMD and the relationship between TMD and the type of occlusion. Study design: A sample of 923 children (463 girls and 460 boys, ages 7–12 years old) was grouped not only by chronological age but also by gender. The information was collected on functional occlusion (anterior and lateral sliding, interferences), dental wear, mandibular mobility (maximal opening, deflection, deviation), and temporomandibular joint and muscular pain recorded by palpation. Results: Headache was the only symptom of temporomandibular dysfunction (TMD) reported by the children. The results showed that one or more clinical signs were recorded in 25% of the subjects, most of which were mild in character. The prevalence increased during the developmental stages. Girls were in general more affected than boys. Conclusions: In this study, many subjects with TMD had malocclusions. Early treatment may be important in the prevention of severe TMD. Significant associations were found between different signs, and TMD was associated with posterior crossbite, anterior open bite, Angle Class II and III malocclusions, and extreme maxillary overjet.


2022 ◽  
Vol 11 (1) ◽  
pp. e35211120077
Author(s):  
Karen Katlein Dolenkei ◽  
Lucas do Nascimento Tavares ◽  
Denildo de Magalhães ◽  
Luís Henrique Araújo Raposo

The aim of this clinical report is to present the replacement of unsatisfactory metal-ceramic crowns of elements 12, 11, 21 and 22, by lithium disilicate glass-reinforced ceramic crowns in a patient with skeletal anterior open bite. A patient sought care at the Dental Hospital at the School of Dentistry of UFU, complaining of odor between the metal-ceramic crowns of the antero-superior teeth. After clinical and radiographic examination, invasion of the biological space was noticed, surgery was indicated in order to restore the biological space. After healing, the pre-existing cast metal posts were masked using an opaque composite resin and the teeth were reprepared for full all-ceramic crowns. Impressions were taken in two steps, using PVS associated to retraction cords. The ceramic copings were obtained in lithium disilicate ceramic, and the veneering was performed by stratification. After testing the ceramics crowns in relation to, fit, function and aesthetic results, cementation was completed using modified absolute isolation, followed by prophylaxis of preparations with pumice and saline, surface treatment of the ceramic restorations and luting using self-adhesive resin cement. Finally, an occlusal splint was produced to control the effects of bruxism and orofacial pain symptoms. The anterior open skeletal bite was a challenging factor for the rehabilitation of this patient; however, one must consider the entire process that the patient would undergo if choosing for orthognathic surgery, and the patient should be aware of the case limitations. In addition, the use of lithium disilicate glass-reinforced ceramics proved the versatility of this material for anterior aesthetic restorations.


2020 ◽  
Vol 44 (4) ◽  
pp. 262-267
Author(s):  
Semra Gürsoy ◽  
Filiz Hazan ◽  
Bülent Kaderli ◽  
Timur Meşe ◽  
Ajlan Tükün

Objective: To evaluate orodental, facial, clinical and molecular characteristics of the patients with Noonan Syndrome (NS).Study Design: The orodental, clinical and molecular characteristics of 29 mutation-positive patients with NS were recorded. Orodental examination was performed in 17 patients. All exons and exonintron boundries of PTPN11 and SOS1 genes were analyzed by Sanger sequencing. Results: A total of 29 patients with NS from 27 unrelated families were included in the study. Seventeen patients were examined by a specialist in oral medicine. The most common orodental findings were high-arched palate (n=13), gingivitis (n=6) and severe caries (n=6). Anterior open bite, posterior cross bite, Class II malocclusion, hypodontia, prognathism (maxillary or mandibular), macroglossia and gingival hyperplasia were also detected. Thirteen different mutations were observed in PTPN11 gene and exon 3 was the hotspot region. Hypodontia was detected in two patients who had the same mutation in PTPN11 gene, c.181G>A, p.D61N. Conclusion: This study indicated a high prevalance of orodental problems including high-arched palate, severe dental caries and gingivitis in patients with mutation-positive NS. The mutation in PTPN11 gene, c.181G>A, p.D61N, may be associated with hypodontia in patients with NS.


Pathogens ◽  
2021 ◽  
Vol 10 (3) ◽  
pp. 298
Author(s):  
Esther Dirks ◽  
Phebe de Heus ◽  
Anja Joachim ◽  
Jessika-M. V. Cavalleri ◽  
Ilse Schwendenwein ◽  
...  

A 23-year-old pregnant warmblood mare from Güssing, Eastern Austria, presented with apathy, anemia, fever, tachycardia and tachypnoea, and a severely elevated serum amyloid A concentration. The horse had a poor body condition and showed thoracic and pericardial effusions, and later dependent edema and icteric mucous membranes. Blood smear and molecular analyses revealed an infection with Theileria equi. Upon treatment with imidocarb diproprionate, the mare improved clinically, parasites were undetectable in blood smears, and 19 days after hospitalization the horse was discharged from hospital. However, 89 days after first hospitalization, the mare again presented to the hospital with an abortion, and the spleen of the aborted fetus was also PCR-positive for T. equi. On the pasture, where the horse had grazed, different developmental stages of Dermacentor reticulatus ticks were collected and subjected to PCR, and one engorged specimen was positive for T. equi. All three amplicon sequences were identical (T. equi genotype E). It is suspected that T. equi may repeatedly be transmitted in the area where the infected mare had grazed, and it could be shown that transmission to the fetus had occurred. Due to the chronic nature of equine theileriosis and the possible health implications of infection, it is advised to include this disease in the panel of differential diagnoses in horses with relevant clinical signs, including horses without travel disease, and to be aware of iatrogenic transmission from inapparent carrier animals.


2021 ◽  
pp. 1-16
Author(s):  
Zoë Thijs ◽  
Laura Bruneel ◽  
Guy De Pauw ◽  
Kristiane M. Van Lierde

<b><i>Background:</i></b> Relationships between malocclusion and orofacial myofunctional disorders (OMD), as well as malocclusions and articulation disorders (AD) have been described, though the exact relationships remain unclear. Given the high prevalence of these disorders in children, more clarity is needed. <b><i>Summary:</i></b> The purpose of this study was to determine the association between OMD (specifically, bruxism, deviate swallowing, caudal resting tongue posture, and biting habits), AD, and malocclusions in children and adolescents aged between 3 and 18 years. To conduct a systematic review, 4 databases were searched (MEDLINE, Embase, Web of Science, and Scopus). The identified articles were screened for the eligibility criteria. Data were extracted from the selected articles and quality assessment was performed using the tool of Munn et al. [Int J Health Policy Manag. 2014;3:123–81] in consensus. Using the search strategy, the authors identified 2,652 articles after the removal of duplicates. After reviewing the eligibility criteria, 17 articles were included in this study. One of the included articles was deemed to have an unclear risk of bias, whereas all other articles were considered to have a low risk of bias. The articles showed a relationship between anterior open bite and apico-alveolar articulatory distortions, as well as between anterior open bite and deviate swallowing. For the biting habits, bruxism, and low tongue position no clear conclusions could be drawn. <b><i>Key Messages:</i></b> The current review suggests a link between specific types of malocclusion and OMD and AD. However, more high-quality evidence (level 1 and level 2, Oxford Levels of Evidence) is needed to clarify the cooccurrence of other OMD, AD, and malocclusions.


2009 ◽  
Vol 79 (4) ◽  
pp. 804-811 ◽  
Author(s):  
Kenichi Sasaguri ◽  
Rika Ishizaki-Takeuchi ◽  
Sakurako Kuramae ◽  
Eliana Midori Tanaka ◽  
Takashi Sakurai ◽  
...  

Abstract A 32-year-old Japanese female patient consulted the authors' dental clinic with a 4.5-year history of rheumatoid arthritis (RA). She complained of pain during mouth opening and difficulty in eating due to masticatory dysfunction caused by an anterior open bite. Imaging showed severe erosion and flattening of both condyles. RA stabilized after pharmacological therapy and became inactive during the orthodontic therapy aimed at reconstructing an optimal occlusion capable of promoting functional repositioning of the mandible. At present, 4 years and 2 months postretention, the reconstructed occlusion remains stable, and both condyles continue to be remodeled. The distance from reference position to intercuspal position has gradually decreased throughout the 4-year posttreatment and postretention periods. Orthodontic therapy that comprehensively reconstructs occlusion and enhances the functioning of the mandible can induce remodeling of eroded condyles, even those with a history of rheumatoid arthritis.


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