Journal of Dental Medicine

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One patient with Ellis Von Creveld syndrome contains: dwarfism, congenital heart disease, ectodermal dysplasia, polyductyly, an abnormally wide labial renum and maxillary molars with single root.

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The most important craniofacial dysostosis & syndromes are Crouzon, Apret, Pfeiffer.Carpenter Saether- Chotzen. The common characteristic is premature closure of cranial sutures, which result in Maxillofacial deformities. Perfect recognition and carefully evaluation are the most important points in their reconstruction surgery.Because the prevalence & surgical treatment of Crouzon syndrome is low, I present a case report include surgical treatment of a patient with crouzon which was a accomplished in Shariati Hospital.

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Muscular pain is one of the most common causes for facial pain. Myofacial pain dysfunction syndrome (MPDS) is one of the most important disorders of facial area that affected patients suffer from pain, tenderness of one or more masticatory muscles and limitation of movements. Lack of specific and effective therapeutic method, makes it necessary to find a treatment to decrease pain. Considering the analgesic and anti- inflammatory effects of low-level laser, some investigators have recommended it for MPDS patient's treatment. In this study, the effects of low level laser (Ga-Al-As) on MPDS patients referred to dental faculty of Tehran University of Medical Sciences, were investigated. Twenty- two MPDS patents, with mean age 33.32 years, were treated for 4 weeks (12 visits). Clinical examinations were performed in 6 stages. The results of this study showed that variables such as pain severity, cheek pain, pain frequency and tenderness of masseter, temporal, internal and external pterygoid muscles, had a statistically significant improvement at the end of treatment with low level laser (GA-A1-AS) which lasted for three months, (follow- up period). Results of this study showed that, low-level laser therapy has a sedative effect on the pain and tenderness of masticatory muscles in MPDS. Patients and having an accurate and regular program to complete treatment period, has an important role in MPDS improvement.

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Diagnosis and treatment of patients with periodontitis as a manifestation of systemic diseases is of especial concern to the periodontist, especially those associated with genetic disorders, which have poor prognosis. With aggressive progression of periodontal bone and attachment loss, a patient could be a partial or total edentulous early in life.The aim of this article was to report a case of Papillon-Lefevre syndrome (PLS) with generalizedprepubertal periodontitis (GPPP). A ten-year old boy for whom active periodontal treatment and subsequent maintenance recalls was performed for five years since the diagnosis of PLS. Treatment procedures included: precise mechanical instrumentation at several visits, periodontal surgery, adjunctive serial systemic antibiotic therapy, professional irrigation of pockets with 0/2% chlorhexidine solution and periodic maintenance recall visits. In spite of all of these, progressive course of the disease continued until the patient was fifteen and edentulous. Unfortunately association of GPPP with systemic unmanageable condition or diseases has caused refractory periodontitis, which yet has no proven and reliable treatment protocol. Besides, this article has discussed more successful treatment modalities for PLS with GPPP and the genetic aspects of host susceptibility, which is a complicated and challenging field.

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Papillon Lefevre Syndrome (PLS) is a rare disease, associated with the early onset periodontal break down in deciduous and permanent dentition.The etiology of PLS is not exactly determined but recently, mutation in catespin C gene, as a genetic basis for the disease, has been established. The aim of this study was to investigate the HLA status in patients affected with PLS. In this research, the frequency of HLA-I and HLA-DR in 9 PLS patients (Belonged to seven unrelated families) were compared with 37 healthy people (controls). Fisher exact test was used for data analysis. The results indicated that the frequency of HLA-B18 was significantly higher in the patients’ group, compared to the control group(P<0.05). The present study, based on the serological methods, is the only existing controlled study regarding PLS. However, in order to get accurate results, a molecular technology with more samples is required.

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Sjogren syndrome is one of the autoimmune diseases which is characterized by lymphocytic infiltration to exocrine glands and causes keratoconjunctivitis sicca and xerostomia. Today, a large population, with a majority of women over 40, suffer from this disease and have several complications regarding oral health and reduced life quality such as severe dental caries, painful eyes, olfactory and gustatory deficiency, speech, mastication and swallowing discomforts. Unfortunately, these patients do not respond to the conventional therapies. Nowadays in medical world, which its target is basic therapy and not symptomatic one, several gene therapy approaches, have gained importance in treatment of this apparently incurable diseases. Due to the facts that this disease is the second prevelant autoimmune disease, after rheumatoid arthritis, and the conventional therapies of the disease are all relative and symptomatic, researchers have insisted on the basic and causative therapy through gene transfer more than before. In the Present article, through reviewing 58 references containing recent scientific and investigatory findings it has been tried, to consider the pathogenesis and conventional therapies of this syndrome. Another purpose of this study was to investigate several and potentially very effective gene transfer systems and different theraputic genes (mainly membrane water channels, ione transporter molecules, transcription factors, antifungal proteins and free radical scavengers).

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Papillon Lefevre Syndrome (PLS) is a rare recessive autosomal disease, which is associated with palm and sole hyperkeratosis and early periodontium break down of deciduouse and permanent dentition. In the differential diagnosis of PLS, congenital form of palm and sole hyperkeratosis should be considered.Recently, mutation in catepesin C gene has been recognized as a genetic basis for PLS disease. In this disease, complete lack of catepesin C enzyme activity, due to the mutations on both allels, are observed. Actinobacillous actinomycetemcomitans (A.a) bacteria are presented as the main periodontal pathogene in PLS disease. The treatment results have been contradictory and no agreement has been obtained in this regard. Elimination of A.a bacteria and administration of synthetic retinoids are reported as a suitable treatment.

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Background and Aim: One of the most common causes of facial pain, is muscular pain. MPDS is one of the most important disorders of facial area that many of patients suffer from pain, tenderness of one or more masticatory muscles and limitation of movement. Psychological factors play an important role in MPDS. Anxiety and depression by increasing muscle tension, can cause myofacial pain syndrome They can also accompany the syndrome as comorbidities or develop as sequelae of chronic pain and disability. Many approaches can be used to treat myofacial pain syndrome. The least invasive and least traumatic approach should be se.lected. Often, treatment for myofacial pain syndrome fails, because underlying problems go untreated. Progressive Muscle Relaxation (PMR) is mainly used for treating anxiety and depression. The aim of this study was to evaluate the effect of PMR in improving signs and symptoms of patients with MPDS.

Materials and Methods: In this before and after clinical trial study, 33 patients with MPDS that had anxiety and or mild depression(were assessed by Beck Inventory)were treated by PMR in a period of a month (3 visits)and were followed up until 2 months. Analysis was done using paired t-test/ wilcoxon-sign-rank test.

Results: The results of this study showed that variants like intensity of pain, tenderness of masticatory muscles, maximum opening of mouth with and without pain, anxiety (p<0.001) and depression (p=0.001) improved significant 16 after treatment in comparison with before treatment.

Conclusion: In conclusion, PMR is effective in improving signs and symptoms in patients with MPDS. Therefore psychological status should be considered in treatment plan of these patients.

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Background and Aims: Multiple supernumerary teeth are rare and often found in association with syndromes such as Gardner, Cleidocranial dysplasia and cleft lip and palate, with a much less chance for isolated non-syndromic cases. The aim of this study was to report a case with 6 supernumerary teeth without syndromic association.
Case Report: The patient was a 33 year-old female, referred to oral diseases and diagnosis department with chief complaint of sensitivity to cold and hot food in right upper premolar region. Oral examination revealed 5 erupted lingually supernumerary teeth (four in mandibular and one in maxillary premolar region, respectively). Further panoramic radiography clarified an extra impacted tooth in the palatal region of left premolar maxillary area. All extra teeth had been appeared since the age of 17 during one year, as the patient claimed. Medical history and thorough clinical and paraclinical examinations were not significant except for the hypothyroidism, since 5 years ago. No other family member noticed to be the case. Based on our findings, a diagnosis of non-syndromic multiple supernumerary teeth was established.
Conclusion: A thorough examination of each patient presented with supernumerary teeth, including panoramic and intraoral radiographic images may provide valuable information regarding accompanying syndromes and unerupted teeth. Early diagnosis is an essential step for orthodontic or surgical decisions making, preventing or avoiding worsening complications such as malocclusion, adjacent normal teeth delayed eruption or rotation, diasthema, cystic lesions and resorption of contiguous teeth.

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