scholarly journals Rare Genetic Disorders Affecting the Periodontal Supporting Tissues in Adolescence

2021 ◽  
Vol 2 ◽  
Author(s):  
Ines Kapferer-Seebacher ◽  
Lena Foradori ◽  
Johannes Zschocke ◽  
Reinhard Schilke

In adolescents periodontal destruction may be the primary manifestation of an as yet unrecognized rare systemic disease, and it may be up to the periodontist to make the correct tentative diagnosis. Many genetic diseases that present with primary periodontal manifestations in adolescence affect immune function, sometimes with only mild or absent systemic features. They include periodontal Ehlers-Danlos syndrome (lack of attached gingiva, various connective tissue abnormalities), Papillon-Lefèvre syndrome (palmoplantar hyperkeratosis), and plasminogen deficiency (fibrin deposition within mucous membranes). Other immune disorders with severe periodontitis manifesting in adolescence are usually diagnosed in early childhood due to unmistakeable systemic features. They include Cohen syndrome (developmental disorder, truncal obesity, and microcephaly), Hermansky-Pudlak Syndrome (oculocutaneous albinism, bleeding diathesis, and other systemic manifestations), glycogen storage disease type 1b, and Chediak-Higashi syndrome (pyogenic infections, albinism, and neuropathy). The structural integrity of periodontal tissue is affected in genodermatoses such as Kindler syndrome, a type of epidermolysis bullosa. In primary hyperoxaluria, inflammatory periodontal destruction is associated with renal calculi. Breakdown of periodontal tissues independent of dental plaque biofilm-induced periodontitis is found in hypophosphatasia (highly variable skeletal hypomineralization) or isolated odontohypophosphatasia, hypophosphatemic rickets and primary hyperparathyroidism. Finally, alveolar osteolysis mimicking localized periodontitis may be due to neoplastic processes, e.g., in neurofibromatosis type 1 (typical skin features including café au lait macules and neurofibromas), Langerhans cell histiocytosis (locally destructive proliferation of bone marrow-derived immature myeloid dendritic cells), and Gorham-Stout disease (diffuse cystic angiomatosis of bone).

Healthcare ◽  
2021 ◽  
Vol 9 (6) ◽  
pp. 744
Author(s):  
Pradeep S. Anand ◽  
Supriya Mishra ◽  
Deepti Nagle ◽  
Namitha P. Kamath ◽  
Kavitha P. Kamath ◽  
...  

Background: Findings of studies testing the association between smokeless tobacco (SLT) use and periodontal health have shown varying results in different populations. Considering the high prevalence of SLT use in India, the present study was conducted to understand the pattern of periodontal destruction within different areas of the dentition among SLT users. Methods: Age, gender, oral hygiene habits, the frequency and duration of SLT consumption, the type of SLT product used, and the site of retention of the SLT product in the oral cavity were recorded among 90 SLT users. Probing depth (PD), recession (REC), and clinical attachment loss (CAL) at SLT-associated and non SLT-associated teeth of the mandibular arch were compared based on the site of retention of the SLT product, the type of product used, and the duration of the habit. Results: REC and CAL were significantly higher at the SLT-associated zones compared to non SLT-associated zones and at both interproximal and mid-buccal sites of SLT-associated teeth. Among individuals who had the habit for more than 5 years and also among those who had the habit for 5–10 years, PD, REC, and CAL were significantly higher at SLT-associated teeth than at non SLT-associated teeth. Significantly greater periodontal destruction was observed at SLT-associated teeth among khaini users and gutkha users. Conclusions: Smokeless tobacco consumption resulted in greater destruction of periodontal tissues. The severity of periodontal destruction at SLT-associated sites differed depending on the type of smokeless tobacco used, the site of retention of the SLT, and the duration of the habit.


2010 ◽  
Vol 1 (3) ◽  
pp. 151
Author(s):  
Herawati Herawati ◽  
Jenny Sunariani

Tobacco contains thousands of chemical substances which known to be harmful to periodontal tissues. Nicotine was considered as the most toxic substances to periodontal tissues. The datas in this review indicate that smoking may have a significant role in the initiation and progression of periodontal destruction. The conclusion of this and the other studies indicate that smokers have a less favorable response to periodontal therapy than non smoker. Nicotine is potentially toxic substances that have a detrimental effect on periodontal tissue, by altering the host response or directly damage the cells of normal periodontium.


2008 ◽  
Vol 33 (2) ◽  
pp. 155-160 ◽  
Author(s):  
Camila Palma Portaro ◽  
Yndira Gonzalez Chópite ◽  
Abel Cahuana Cárdenas

Destructive forms of periodontal disease in children are uncommon. Severe periodontal destruction can be a manifestation of a systemic disease; however, in some patients, the underlying cause of increased susceptibility and early onset is still unknown. Objective: To describe an effective therapeutic approach to Generalized Aggressive Periodontitis (GAgP) in children, based on a 3-1/2 year-old male patient referred to the Hospital due to early loss of incisors, gingivitis,and tooth mobility in his primary dentition. Intraoral examination revealed severe gingival inflammation,dental abscesses, pathological tooth mobility, bleeding upon probing and attachment loss around several primary teeth. Dental radiographs revealed horizontal and vertical bone loss. Treatment consisted on the extraction of severely affected primary teeth, systemic antibiotics, deep scaling of remaining teeth and strict oral hygiene measures. Once the patient's periodontal condition was stabilized, function and esthetics were restored with "pedi-partials." After a follow-up period of nearly 4 years, the patient's periodontal status remains healthy, facilitating the eruption of permanent teeth. Conclusion: Prompt diagnosis and good treatment regimen may provide an effective therapeutic management of Generalized Aggressive Periodontitis.


2007 ◽  
Vol 19 (3) ◽  
Author(s):  
Aprilia Adenan ◽  
Nunung Rusminah

The objectives of prostheses are to restore mastication force, improve esthetics and maintain gingival health. The construction and function of prosthesis restoration are mutually interdependent with condition of periodontal tissues. A properly constructed prosthesis is an integral phase of complete treatment of periodontal disease in order to maintain periodontal tissues health. This paper reports case of a man aged 47 years who came to Dental Specialist Clinic in Oral and Dental Hospital Faculty of Dentistry Universitas Padjadjaran with chief complaint of mobility in almost all his teeth and they seems to look longer. The patient has no systemic disease and did not want his teeth to be extracted. Clinical and panoramic radiographic and laboratoris examinations has been done. During treatment, oclusal adjustment and splinting had been done on tooth 33,34,35 and tooth 44,43 also tooth 43,42 splinting with composite. The following treatment was the acrylic removable partial denture for upper jaw while mandible was fitted a frame denture which functioned as a semi permanent splint. One month post treatment, patien felt comfort and the denture was well functioning.


Author(s):  
Manfred Nilius ◽  
MInou Nilius ◽  
Charlotte Mueller ◽  
Guenter Lauer ◽  
Berit Koch ◽  
...  

Periodontal Ehlers-Danlos syndrome type VIII (pEDS) is rare and caused by mutation in complement 1 subunit. Lack of attached gingiva, pretibial plaques, joint mobility, skin fragility, and easy bruising consolidate the diagnosis. We describe a case of de novo-mutation of pEDS with generalized periapical cemento dysplasia (PCD) and leukoencephalopathy.


2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Edoardo Ferrari Cagidiaco ◽  
Simone Grandini ◽  
Cecilia Goracci ◽  
Tim Joda

Abstract Background Lithium disilicate is now a well accepted material for indirect restorations. The aim of this trial was to evaluate two lithium disilicate systems using a novel prosthodontic Functional Index for Teeth (FIT). Methods Partial adhesive crowns on natural abutment posterior teeth were made on sixty patients. Patients were divided into two groups: Group 1 IPS e.max press (Ivoclar-Vivadent, Schaan, Liecthestein), and Group 2 Initial LiSi press (GC Co., Tokyo, Japan). The restorations were followed-up for 3 years, and the FIT evaluation was performed at last recall. The FIT is composed of seven variables (Interproximal, Occlusion, Design, Mucosa, Bone, Biology and Margins), each of them are evaluated using a 0–1-2 scoring scheme, and is investigated by an oral radiograph and occlusal and buccal pictures. More in details, three variables have the three scores made on the presence or not of major, minor or no discrepancy (for ‘Interproximal’, ‘Occlusion’ and ‘Design’), presence or not of keratinized and attached gingiva (‘Mucosa’), presence of bone loss > 1.5 mm, < 1.5 mm or not detectable (‘Bone’), presence or not of Bleeding on Probing and or Plaque Index (‘Biology’), presence of detectable gap and marginal stain or not (‘Margins’). The Mann-Whitney ‘U’ test was used and the level of significance was set at p < 0.05. Also, “success” of the crowns (restoration in place without any biological or technical complication) and “survival” (restoration still in place with biological or technical complication) were evaluated. Results Regarding FIT scores, all partial crowns showed a stable level of the alveolar crest without detectable signs of bone loss in the radiographic analysis. All other evaluated parameters showed a high score, between 1.73 and 2. No statistically significant difference emerged between the two groups in any of the assessed variables (p > 0.05). All FIT scores were compatible with the outcome of clinical success and no one restoration was replaced or repaired and the success rate was 100%. Conclusions The results showed that it is possible to evaluate the clinical performance of partial crowns using FIT. The FIT proved to be an effective tool to monitor the performance of the restorations and their compatibility with periodontal tissues at the recall. The FIT can be really helpful for a standardized evaluation of the quality of the therapy in prosthodontic dentistry. The two lithium disilicate materials showed similar results after 3 years of clinical service. Trial registration The study protocol was approved by the Ethical Committee of University of Siena (clinicaltrial.gov # NCT 01835821), ‘retrospectively registered’.


2013 ◽  
Vol 14 (4) ◽  
pp. 762-765
Author(s):  
Vidya Baliga ◽  
VPK Gopinath ◽  
Sudhindra Baliga ◽  
Srinivas Sulugodu Ramachandra ◽  
KD Jithendra ◽  
...  

ABSTRACT Aim To report rare findings of oral and periodontal manifestations in a patient with Epidermal nevus syndrome (ENS). Background The ENS describes the rare association of an epidermal nevus with abnormalities of central nervous system, ocular and skeletal abnormalities. Reports of oral involvement have been few. Also, most of the intraoral lesions have been reported in patients with nevi that do not fulfill the criteria for the diagnosis of ENS. Case description This report describes a case of ENS that, in addition to cutaneous manifestations showed skeletal involvement and intraoral manifestations such as the extension of the nevi on the face intraorally involving the labial mucosa, hypoplasia, hypodontia of teeth and severe periodontal destruction. Conclusion Patients with extensive epidermal nevi and systemic abnormalities should be suspected of having the ENS. Evaluation and management of patients with ENS requires a multidisciplinary team approach involving the dermatologist, pediatrician, ophthalmologist, neurologist, genetist, plastic surgeon and orthopedic services. Although uncommonly described in association with ENS, significant intraoral lesions do occur. Periodontal manifestations as in our patient, which to our knowledge has not been described in association with ENS so far, may also be present. Clinical relevance Alteration of the response of periodontal tissues to dental plaque in the presence of certain systemic diseases has been reported, but not in association with ENS. Severe periodontal destruction due to exaggerated response to dental plaque was seen in the present case. Hence, emphasis on oral hygiene maintenance in such patients is essential. Patients with ENS must be evaluated periodically as they show a persistent predisposition for the development of tumors How to cite this article Baliga V, Gopinath VPK, Baliga S, Ramachandra SS, Jithendra KD, Shetty S. Intraoral Manifestations in a Patient with Epidermal Nevus Syndrome . J Contemp Dent Pract 2013;14(4):762-765.


2021 ◽  
Author(s):  
Juan Wu ◽  
Wai Keung Leung ◽  
Weibin Sun

The object of this chapter was to provide an overview including relevant research progress of some genetic disorders with periodontal manifestations. A number of genetic disorders increase patient susceptibility to periodontal disease, with the latter exhibit rather rapid and aggressive presentations. Periodontal disease, perhaps could be the first detectable sign of an undiagnosed genetic disorder. It is therefore important for dental practitioners to be familiar with genetic disorders and their impact on the periodontal tissues. This chapter reviews several genetic disorders that exhibit periodontal manifestations, including hereditary gingival fibromatosis, Papillon-Lefèvre syndrome, cyclic neutropenia, Ehlers-Danlos syndrome and hypophosphatasia.


2020 ◽  
Vol 23 (2) ◽  
pp. 168-176
Author(s):  
Mahmure A. Tayman ◽  
İsmail Koyuncu ◽  
Nimet Ö. Köklü

Background: ADAMTS (A disintegrin-like metalloproteinase with thrombospondin motifs) is a group of 19 zinc-dependent metalloproteases known to function in many pathological and physiological processes, such as adhesion, cell fusion, signaling, proteolysis and ECM degradation. Objectives: The aim of this study was to demonstrate the levels of ADAMTS-4 and -5 in gingival tissues with Stage III-Grade B generalized periodontitis (SIII-GB), Stage III-Grade C generalized periodontitis (SIII-GC) and healthy-control (C) groups. Methods: The clinical measurements were recorded for each patient. A total of 63 gingival biopsy specimens were obtained from the C (n:20), SIII-GB (n:23) and SIII-GC (n:20) groups. Polymerase chain reaction (Rt-PCR) and immunohistochemical (IHC) examinations were used to determine gene and protein levels. Results: According to the results of all methods, ADAMTS-4 and -5 expressions existed in periodontitis and C groups (P> 0.05). Immunostaining for ADAMTS-4 was found to be higher in patients with periodontitis than for ADAMTS-5 (P>0.05). Gene expression levels for ADAMTS-4 and -5 seemed to be up-regulated in subjects diagnosed with periodontitis, but the results were not statistically significant (P>0.05). A positive correlation was observed between PPD and ADAMTS-4 mRNA in SIII-GC (p=0.035) and SIII-GB (p=0.015). A positive correlation was determined between ADAMTS-4 mRNA and ADAMTS-5 mRNA in SIII-GC (p=0.037) and SIII-GB (p=0.00). Conclusion: ADAMTS expression may take part in both pathological and physiological processes in the periodontal tissues, and periodontal destruction may be the result of a complex interaction of several pathways with many participants, such as ADAMTS-4 and -5, thus facilitating the exaggeration of periodontal disease.


Sign in / Sign up

Export Citation Format

Share Document