scholarly journals Management of Amelogenesis Imperfecta in Childhood: Two Case Reports

2021 ◽  
Vol 18 (13) ◽  
pp. 7204
Author(s):  
Mirja Möhn ◽  
Julia Camilla Bulski ◽  
Norbert Krämer ◽  
Alexander Rahman ◽  
Nelly Schulz-Weidner
Keyword(s):  
Case Reports ◽  
Treatment Options ◽  
Permanent Dentition ◽  
Amelogenesis Imperfecta ◽  
Structural Defects ◽  
Type I ◽  
Oral Rehabilitation ◽  
Self Confidence ◽  
Health Quality ◽  
Masticatory System

Amelogenesis imperfecta (AI) is defined as an interruption of enamel formation due to genetic inheritance. To prevent malfunction of the masticatory system and an unaesthetic appearance, various treatment options are described. While restoration with a compomer in the anterior region and stainless steel crowns in the posterior region is recommended for deciduous dentition, the challenges when treating such structural defects in mixed or permanent dentition are changing teeth and growing jaw, allowing only temporary restoration. The purpose of this case report is to demonstrate oral rehabilitation from mixed to permanent dentition. The dentition of a 7-year-old patient with AI type I and a 12-year-old patient with AI type II was restored under general anesthesia to improve their poor aesthetics and increase vertical dimension, which are related to problems with self-confidence and reduced oral health quality of life. These two cases show the complexity of dental care for structural anomalies of genetic origin and the challenges in rehabilitating the different phases of dentition.

scholarly journals Amelogenesis imperfecta: therapeutic strategy from primary to permanent dentition across case reports

2018 ◽  
Vol 18 (1) ◽  
Author(s):  
Steve Toupenay ◽  
Benjamin Philippe Fournier ◽  
Marie-Cécile Manière ◽  
Chantal Ifi-Naulin ◽  
Ariane Berdal ◽  
...  
Keyword(s):  
Therapeutic Strategy ◽  
Case Reports ◽  
Permanent Dentition ◽  
Amelogenesis Imperfecta

scholarly journals Celebrating one year anniversary of Contemporary Pediatric Dentistry and introducing the new Congress

2021 ◽  
Vol 2 (3) ◽  
pp. 134-134
Author(s):  
Burak Buldur
Keyword(s):  
Case Reports ◽  
Treatment Options ◽  
Pediatric Dentistry ◽  
Good News ◽  
International Congress ◽  
Original Research ◽  
Virtual Meeting ◽  
Health Quality ◽  
One Year

Hi Dear Friends and Colleagues, I am pleased to inform you some of good news regarding the development and process of the Contemporary Pediatric Dentistry (CPD). Since its establishment, CPD has received 80 submissions from all over world within sixteen months, and published 25 articles including review, original research and case reports. CPD has also moved to be indexed in reputed databases. Another achievement was establishment of 1st International Congress of Contemporary Pediatric Dentistry (ICCPD), organized by the CPD, which was taken place on 1- 3 November 2021 as a fully virtual meeting to celebrate the first anniversary of the launch of CPD. The ICCPD hosted 257 registered colleagues from 47 countries. The website of the ICCPD had been viewed more than 5000 visitors from 102 countries in only 2 months. As an exciting action, CPD is now getting ready to initiate the 2nd International Congress of Contemporary Pediatric Dentistry (www.iccpd.org). I feel confident that the ICCPD 2022 will serve as a place of learning where we can all share the latest knowledge and learn the contemporary view, approaches, techniques and treatment options from highly eminent clinicians and/or academicians from different countries. The theme of the ICCPD 2022 is “Contemporary Clinical Techniques in Pediatric Dentistry”. The 2nd ICCPD will also host oral and poster presentation. All abstracts will be published in the Supplementary Issue of Contemporary Pediatric Dentistry. It is my honor to invite all colleagues, who have sincere enthusiasm to help children to let them have better oral health, quality of life, smiling and development, to attend 2nd International Congress of Contemporary Pediatric Dentistry to be held on 2-4 September 2022 as a fully virtual meeting. Please follow website and social media accounts of CPD to get latest information regarding Congress and the Journal.

scholarly journals Oral Rehabilitation Using Noninvasive Restorative Approach for Late Mixed Dentition of Preterm Birth Child with Amelogenesis Imperfecta

2020 ◽  
Vol 2020 ◽  
pp. 1-5
Author(s):  
Abdulfatah Alazmah
Keyword(s):  
Preterm Birth ◽  
Structural Integrity ◽  
Amelogenesis Imperfecta ◽  
Mixed Dentition ◽  
Treatment Modalities ◽  
Oral Rehabilitation ◽  
Child Treatment ◽  
Medical Needs ◽  
Self Confidence ◽  
Invasive Method

Preterm birth children comprise about 6% of live births around the world. It is known that premature children exhibit oral anomalies that could affect the function and/or appearance of their dentition in addition to their medical needs. A diagnosis of amelogenesis imperfecta (AI) can present a challenge for both the patient and the treating clinician. This can be more complicated in the case of child treatment, where cooperation and some of the treatment modalities for adults can not be considered. Conventional management of such children is not possible due to the ongoing process of growth and development and the ability of the child to cope with the extensive and lengthy treatment procedure. This article highlights a minimally invasive method for managing AI using adhesive and full-coverage restoration that requires no tooth preparation; this allows the structural integrity of the teeth to be maintained, along with their vitality. As a result, the child will have teeth with better function and aesthetic, to improve eating, appearance, and self-confidence.

scholarly journals P.057 Distal hereditary motor neuropathy type I due to the GARS: c.1415A>G, p.His472Arg mutation

2016 ◽  
Vol 43 (S2) ◽  
pp. S34-S34
Author(s):  
AG Florendo-Cumbermack ◽  
K Kimpinski ◽  
S Venance
Keyword(s):  
Genetic Characterization ◽  
Case Reports ◽  
Treatment Options ◽  
Type I ◽  
Motor Neuropathy ◽  
Juvenile Onset ◽  
Hereditary Motor ◽  
Clinical Presentations ◽  
Hereditary Motor Neuropathy ◽  
Exon 11

Background: The distal hereditary motor neuropathies (dHMN) have been characterized through case reports and family studies. Their genetic characterization remains a work in progress. An appreciation of the genetic underpinnings may lead to treatment options in the future. Hence reports, like this one, which add to this understanding, remain extremely important. Methods: The clinical presentations, electrophysiology and genetics of two patients with the dHMN I phenotype are described. Results: A mother and son presented with slowly progressive distal weakness of the lower extremities with onset in the first and second decades. Distal weakness of the upper extremities developed later. Examination 20 and 50 years after onset revealed wasting and weakness of distal upper and lower extremity muscles with absent distal and preserved proximal deep tendon reflexes. Sensory examination was normal. Electrodiagnostic studies demonstrated a motor axonopathy. Genetics testing revealed a missense mutation on chromosome 7, exon 11 of the GARS gene: c.1415A>G (p.His472Arg). Conclusions: GARS mutations have been identified in patients with the dHMN I (juvenile onset distal weakness and wasting) and dHMN V (upper limb predominant) phenotypes. However, this mutation has not previously been directly linked to the dHMN I phenotype.

scholarly journals Clinical Diagnosis and Oral Rehabilitation of a Patient with Amelogenesis imperfecta: A Case Report

2008 ◽  
Vol 9 (4) ◽  
pp. 92-98 ◽  
Author(s):  
Veena Hegde ◽  
Amar A. Sholapurkar ◽  
Robin Mathia Joseph ◽  
Jothi M. Varghese ◽  
Krishna Neelagiri ◽  
...  
Keyword(s):  
Case Report ◽  
Female Patient ◽  
Clinical Diagnosis ◽  
Economic Status ◽  
Systemic Disease ◽  
Treatment Options ◽  
Amelogenesis Imperfecta ◽  
Clinical Report ◽  
Oral Rehabilitation ◽  
Tooth Structure

Abstract Aim This clinical report describes the oral rehabilitation of a young female patient diagnosed with the hypocalcified, autosomal recessive type of Amelogenesis imperfecta (AI). A brief discussion on diagnosis of AI is also included. Background AI has been defined as a group of hereditary enamel defects not associated with evidence of systemic disease. It can be characterized by enamel hypoplasia and/or hypomaturation or hypocalcification of the existing teeth. Restoration for patients with this condition should be oriented toward the functional and esthetic rehabilitation and the protection of these teeth. Report A 31-year-old female patient presented with concerns including extreme sensitivity; dissatisfaction with size, shape, and shade of teeth; and poor masticatory efficiency. She was very conscious about the appearance of her teeth and reported that her primary dentition was affected in the same manner. The specific objectives of this treatment were to eliminate tooth sensitivity, enhance esthetics, and restore masticatory function. Treatment included crown lengthening procedures and placement of anterior and posterior metalceramic crowns. A 12-month follow-up with clinical and radiographic examinations revealed no evidence of any untoward effects of the treatment on the restored teeth or their supporting structures. Summary Management of a patient with AI is a challenge for the clinician. The treatment options vary considerably depending on several factors such as the age of the patient, socio-economic status, periodontal condition, loss of tooth structure, severity of the disorder, and, most importantly, the patient's cooperation. The clinician has to consider the long-term prognosis of the treatment outcome. This clinical report describes the fabrication of metal ceramic and all metal crowns for the restoration of severely worn teeth in a patient with AI which requires meticulous maintenance of oral hygiene and patient co-operation. Citation Sholapurkar AA, Joseph RM, Varghese JM, Neelagiri K, Acharya SRR, Hegde V, Pai KM, Bhat M. Clinical Diagnosis and Oral Rehabilitation of a Patient with Amelogenesis imperfecta: A Case Report. J Contemp Dent Pract 2008 May; (9)4:092-098.

scholarly journals Characteristics and Treatment Challenges of Non-Clear Cell Renal Cell Carcinoma

Cancers ◽  
2021 ◽  
Vol 13 (15) ◽  
pp. 3807
Author(s):  
Pierangela Sepe ◽  
Arianna Ottini ◽  
Chiara Carlotta Pircher ◽  
Andrea Franza ◽  
Melanie Claps ◽  
...  
Keyword(s):  
Renal Cell ◽  
Kinase Inhibitors ◽  
Clear Cell ◽  
Case Reports ◽  
Treatment Options ◽  
Single Agent ◽  
Growth Factor Receptor ◽  
Mtor Inhibitors ◽  
Cell Renal Cell Carcinoma ◽  
Clear Cell Rcc

Non-clear cell renal cell carcinomas (RCC) comprise several rare and poorly described diseases, often characterized by bad prognosis and with no standard treatments available. The gap in their clinical management is linked to the poor molecular characterization in handling the treatment of non clear-cell RCC with untailored therapies. Due to their rarity, non-clear RCC are in fact under-represented in prospective randomized trials. Thus, treatment choices are based on extrapolating results from clear cell RCC trials, retrospective data, or case reports. Over the last two decades, various options have been considered as the mainstay for the treatment of metastatic RCC (mRCC), including angiogenesis inhibitors, vascular endothelial growth factor receptor inhibitors, other tyrosine kinase inhibitors (TKIs), as well as MET inhibitors and mammalian targeting of rapamycin (mTOR) inhibitors. More recently, the therapeutic armamentarium has been enriched with immunotherapy, alone or in combination with targeted agents that have been shown to significantly improve outcomes of mRCC patients, if compared to TKI single-agent. It has been widely proven that non-clear cell RCC is a morphologically and clinically distinct entity from its clear cell counterpart but more knowledge about its biology is certainly needed. Histology-specific collaborative trials are in fact now emerging to investigate different treatments for non-clear cell RCC. This review summarizes pathogenetic mechanisms of non-clear cell RCC, the evolution of treatment paradigms over the last few decades, with a focus on immunotherapy-based trials, and future potential treatment options.

Gelatin-Containing Vaccines for Varicella, Zoster, Measles, Mumps, and Rubella Induce Basophil Activation in Patients with Alpha-Gal Syndrome

2021 ◽  
pp. 1-7
Author(s):  
Paul Schmidle ◽  
Jana Mehlich ◽  
Knut Brockow ◽  
Ulf Darsow ◽  
Tilo Biedermann ◽  
...  
Keyword(s):  
Case Reports ◽  
Meat Products ◽  
Live Vaccine ◽  
Basophil Activation ◽  
Type I ◽  
Mmr Vaccine ◽  
Varicella Zoster ◽  
Additional Tool ◽  
Severe Anaphylaxis ◽  
New Type

Background: The alpha-gal syndrome (AGS) describes a new type I allergy entity to the carbohydrate epitope galactose-α-1,3-galactose (alpha-gal), which is mainly found in mammalian food products (e.g., beef, pork, and venison). Apart from meat products, alpha-gal can also be found in products containing gelatin of bovine or porcine origin. Recent case reports pointed to severe anaphylaxis in patients suffering from AGS after vaccination with vaccines containing hydrolyzed gelatin. It was the objective of this study to evaluate if basophil activation tests (BATs) performed with such vaccines were positive in patients with AGS. Methods: BAT was performed with different dilutions of a gelatin-containing measles, mumps, and rubella (MMR) live vaccine; an attenuated varicella (V) vaccine; an attenuated V-zoster (VZ) vaccine; a MMR live vaccine not containing gelatin (non-gelatin MMR vaccine) in 2 patients with confirmed AGS, 2 patients highly suspicious for AGS, and 2 healthy individuals without any previous medical history for allergies. Results: All patients showed strongly positive results for all gelatin-containing vaccines (MMR vaccine, V vaccine, and VZ vaccine). Non-gelatin MMR vaccine was negative. The 2 healthy controls did not show any basophil activation. Conclusions: Gelatin-containing vaccines should be administered with caution or avoided in patients with AGS because of their high potential to activate basophils indicating a risk for anaphylaxis. Also, BAT is a useful additional tool when it comes to screening for potentially high-risk alpha-gal-containing drugs.

scholarly journals PARE0002 IMPACT OF SERVICE DOGS ON THE BURDEN OF ARTHRITIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1287.1-1287
Author(s):  
P. Wegscheider
Keyword(s):  
Positive Impact ◽  
Case Reports ◽  
Service Dogs ◽  
Mobility Impairments ◽  
Assistance Dogs ◽  
Self Confidence ◽  
Team Assessment ◽  
Personal Assistants ◽  
Austrian Population ◽  
Short Time

Background:Assistance dogs support humans with different physical disabilities: 1. Service dogs for people with diverse mobility impairments 2. signaling dogs for humans diagnosed with diabetes, hearing impairments, seizure, or posttraumatic stress disorder 3. guide dogs for people with visual impairments. Definitions and terms are not consistent over Europe; Austrian terminology is used in the following. These specially trained dogs support people in their everyday lives and make it possible that less help is required from personal assistants or caregivers. Diverse studies show this positive impact of assistance dogs on the quality of life of disabled people. There are just a few case reports from United States about service dogs for people diagnosed with any inflammatory rheumatic disorder. Dogs are trained individually for about 1.5 years before team training and the concluding team assessment through Austrian authorities take place. Since 2015, there has been an adapted legislation for service dogs in Austria which brings significant improvements in many areas. In comparison to the model set by Austria, there is no corresponding legal basis at EU level or in other European countries.Objectives:Case-report about my own situation diagnosed with juvenile idiopathic arthritis (JIA) in 2001 and my service dog May. May supports my every day live in private and business affairs since 2019Methods:May was trained for less than two years before team assessment. May is able to pick up things I dropped. These include coins, my key, my mobile phone, clothes, towels and lots of other things I want her to pick it up for me. May opens and closes doors, empties the washing machine, pulls the laundry basket and even helps me put on and take off clothes. In general, she carries many things which I instruct her to carry. I am able to learn her more new things in a short time. Furthermore May acts safe on public transport and even airplanes. Due to special training May is allowed to move without dog leash or muzzle. A muzzle or leash would handicap the dog’s work. As a result of May’s help I need less personal assistance.Results:May’s physical and psychological support gives me greater independence and increases my self-confidence. She was trained to specifically meet the needs of my disability. Beside her skills, May helps to reduce pain and burden of arthritis.Conclusion:My aim is to spotlight the great support of service dogs to severely affected arthritis patients. Austria had realized legislation for assistance dogs to guarantee certain permissions the owner’s needs (e.g. access to working place, hospital). I would love to raise awareness about assistance dogs to improve the knowledge about those animals to implement European legislation.References:[1]Arbeitskreis für Hygiene in Gesundheitseinrichtungen des Magistrats der Stadt Wien, MA 15, Richtlinie für den Umgang mit Assistenzhunden und Therapiehunden in Gesundheitseinrichtungen, 29.3.2017; access 1.10.2019[2]AK Krankenhaushygiene OÖ, Umgang mit Assistenzhunden in Gesundheitseinrichtungen, Version 1; access 1.2019[3]Bremhorst, A et al, Spotlight on Assistance Dogs- Legislation, Welfare and Research, Animals 2018, 8, 129; doi:10.3390/ani8080129[4]Deutsche Gesellschaft für Krankenhaushygiene (DGKH), Empfehlungen zum hygienegerechten Umgang mit Therapiehunden in Krankenhäusern und vergleichbaren Einrichtungen, Hyg Med 2017; 42-10[5]Glenk, LM et al, Perceptions on Helath Benefits of Guide Dog Ownership in an Austrian Population of Blind People with and without a Guide Dog, Animals 2019, 9, 428; doi:10.3390/ani9070428[6]Lundqvist, M et al, Certified service dogs- A cost- effectiveness analysis appraisal, Plos ones 12.9.2019, doi:10.1371/journal.pone.0219911[7]Sozialministeriumservice, Richtlinie Therapiehunde des Bundesministers für Arbeit, Soziales und Konsumentenschutz, 01.01.2015; access 1.10.2019Acknowledgments:I am very grateful to Kati Kohoutek, May’s trainer and the efforts of Austrian’s long lasting dog trainers and Karl Weissenbacher, the leader of Messerli Institute/ department of Veterinary University Vienna.Disclosure of Interests:None declared

Efficiency of diffusion-weighted MRI for differentiating radiologically similar simple and type I hydatid cysts of the liver

2021 ◽  
pp. 028418512098813
Author(s):  
Ilyas Dundar ◽  
Mesut Ozgokce ◽  
Fatma Durmaz ◽  
Sercan Ozkacmaz ◽  
Saim Turkoglu ◽  
...  
Keyword(s):  
Treatment Options ◽  
Type I ◽  
Hydatid Cysts ◽  
Liver Cysts ◽  
Diffusion Weighted ◽  
Significant Difference ◽  
Mean Diameter ◽  
Apparent Diffusion ◽  
Adc Values ◽  
Hepatic Lesions

Background Determining the nature of purely cystic hepatic lesions is essential because different kinds have different follow-ups, treatment options, and complications. Purpose To explore the potential of apparent diffusion coefficient (ADC) values of diffusion-weighted imaging (DWI) for the differentiation of type I hydatid cysts (HC) and simple liver cysts (SLC), which have similar radiological appearances. Material and Methods This single-center prospective study was conducted during 2016–2019. Round, homogenous, anechoic liver cysts >1 cm were classified according to at least two years of imaging follow-up, radiological features, serology, as well as puncture aspiration injection reaspiration procedure and pathology results. ADC values of 95 cysts (50 type I HCs and 45 SLCs) were calculated on DWI. The differences in ADC values were analyzed by independent t-test. Results Of 51 patients, 28 were female, 23 were male (mean age 32.07 ± 22.95 years; age range 5–82 years). Mean diameter of 45 SLCs was 2.59 ± 1.23 cm (range 1.2–7.6 cm) and ADCmean value was 3.03 ± 0.47 (range 2.64–5.85) while mean diameter of 50 type I HCs was 7.49 ± 2.95 cm (range 2.8–14 cm) and ADCmean value was 2.99 ± 0.29 (range 2.36–3.83). There was no statistically significant difference in ADC values between type I HCs and SLCs Conclusion Some studies report that ADC values of type I HCs are statistically significantly lower than those of SLCs. Others suggest no significant difference. In our study with a higher number of cases, using ADC parameters similar to those in previous studies, we did not find any statistically significant difference.

scholarly journals Squamous cell carcinoma of the scrotum in HIV: two case reports

2021 ◽  
Vol 27 (1) ◽  
Author(s):  
Jeff John ◽  
Ken Kesner ◽  
John Lazarus
Keyword(s):  
Squamous Cell Carcinoma ◽  
Cell Carcinoma ◽  
Squamous Cell ◽  
Case Reports ◽  
Treatment Options ◽  
Punch Biopsy ◽  
Delayed Treatment ◽  
Clinical Presentations ◽  
T1 N0 M0

Abstract Background Squamous cell carcinoma (SCC) of the scrotum was the first malignancy known to be associated with exposure to an occupational carcinogen—in this case, soot trapped in the breeches of chimney sweeps. Better civil rules and regulations and the replacement of hearths with other forms of heating have rendered SCC of the scrotum a rarity. We report two cases of scrotal SCC with vastly differing clinical presentations and management. Case presentation Case 1 had T1 N0 M0 disease and presented with a small (< 2 cm), innocuous-looking, non-healing ulcer of eight years duration. A punch biopsy revealed a superficially invasive SCC confirmed on immunohistochemical profiling. A wide local excision of the lesion was subsequently performed. Follow-up at three years showed no signs of recurrence. Case 2 presented with T4 N1 M1 disease and rapidly progressing locally destructive mass. A punch biopsy of the scrotal lesion confirmed invasive moderately differentiated focally keratinising SCC. The metastatic evaluation confirmed the presence of metastatic, extensive para-aortic lymphadenopathy. He was managed with cisplatin-based chemoradiotherapy. Conclusion Early detection and management of patients with SCC of the scrotum are essential. If the diagnosis is delayed, treatment options become limited, and the prognosis is poor. Notwithstanding the rarity of this disease, multicentre trials are needed to provide more precise guidelines as to the optimal management of these patients.

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