激素替代疗法治疗女性灼口综合征的临床观察

目的 :观察尼尔雌醇治疗女性灼口综合征的临床效果。方法 :采用尼尔雌醇辅以孕激素的雌激素替代疗法治疗已停经的女性灼口综合征患者。结果 :激素治疗组与维生素治疗组相比有显著性差异 (P <0 .0 1)。结论 :舌感觉异常多发生在妇女更年期 ,内分泌紊乱起着重要作用 ,激素替代疗法可以明显缓解症状     

Genetic studies of syndromes with severe periodontitis and palmoplantar hyperkeratosis

The Papillon-Lefevre and Haim Munk syndromes are characterized by the presence of both palmoplantar hyperkeratosis (PPK) and severe early onset periodontitis. It is the early onset periodontal disease component that distinguishes these from other more common forms of PPK. It has been proposed that the periodontal disease component may be a casual association in individuals with PPK. Genetic syndromes with palmoplantar keratosis and severe early onset periodontitis may be due to specific bacterial infections in individuals with PPK. Recently, keratin gene mutations have been identified in several conditions typified by palmoplantar keratosis. The present study sought to test the hypothesis that a keratin gene defect similar to those previously identified in other PPK conditions is responsible for the Haim Munk and the Papillon-Lefevre syndromes. We have performed genetic linkage studies to test for linkage between polymorphic DNA loci within 2 cytokeratin gene families and the disease phenotype in Haim Munk syndrome and Papillon-Lefevre syndrome. Families with individuals segregating for the Haim Munk syndrome and the Papillon-Lefevre syndrome were examined to determine disease status, and genotyped for microsatellite DNA markers closely linked to the acidic (type I) and the basic (type II) cytokeratin genes on chromosomes 12 and 17. Genotype data were evaluated for microsatellite allele homozygosity in affected individuals. Results of these preliminary genetic studies suggest that the gene defect in Haim Munk syndrome is not due to a gene defect in either the type I or the type II keratin gene clusters. These findings suggest that Haim Munk syndrome may be genetically distinct from other more common forms of PPK that have been linked to the cytokeratin gene families, and suggest that mutations in genes other than keratin genes are responsible. Additional family studies are needed to confirm these preliminary findings.     

Metabolic syndrome: pathogenesis, medical care and dental implications

BACKGROUND: The dental literature contains little information about metabolic syndrome (MetS) and its dental implications. TYPES OF STUDIES REVIEWED: The authors conducted a MEDLINE search for the period 2000 through 2005, using the term "metabolic syndrome" to define its pathophysiology, medical treatment and dental implications. RESULTS: MetS is the co-occurrence of abdominal obesity, hyper-triglyceridemia, reduced high-density lipoprotein cholesterol levels, hypertension and impaired fasting glucose, which results from consumption of a high-calorie diet and decreased levels of physical activity superimposed on the appropriate genetic setting. Components of MetS synergistically promote the development of atherosclerosis, resulting in myocardial infarction and stroke. CLINICAL IMPLICATIONS: Deteriorating oral health status is associated with worsening of the atherogenic profile. Tooth loss often results in chewing difficulties because of inadequate occlusive surfaces and may lead to alterations in food selection and dietary quality. This, in turn, adversely affects body composition and nutritional status, both of which are related to vascular health. Dentists should develop treatment plans that preserve and restore the dentition, thus ensuring maximum masticatory efficiency and affording patients the optimum opportunity to consume food that will not foster atherogenesis.     

Auto‐inflammatory syndromes and oral health

Auto‐inflammatory diseases (periodic syndromes) are rare childhood‐onset disorders which are characterized by fluctuating or recurrent episodes of fever and inflammation affecting serosal surfaces, joints, eyes and/or skin without significant autoantibody production or an identifiable underlying infection. They are disorders of innate immunity and the underlying genetic defect has been identified in most of the syndromes. Diagnosis relies on clinical symptoms and evidence of an elevated acute phase response during attacks, supported by finding mutations in the relevant genes. Several syndromes can lead to systemic AA amyloidosis. Aphthous‐like oral ulceration has been reported as one manifestation in several of the syndromes, including periodic fever, aphthous‐stomatitis, pharyngitis, adenitis (PFAPA) familial Mediterranean fever (FMF), hyperimmunoglobulinaemia D and periodic fever syndrome, tumour necrosis factor receptor associated periodic syndrome and pyogenic sterile arthritis, pyoderma gangrenosum, acne (PAPA). Chronic jaw recurrent osteomyelitis has been recorded in chronic recurrent multifocal osteomyelitis. Advances in the molecular pathogenesis of these syndromes and the regulation of innate immunity have enhanced diagnosis, and rationalized therapies. This article reviews the periodic fever syndromes relevant to oral health and the suggested association of FMF with Behçet’s disease.     

Widespread radiopacity of jaw bones in familial adenomatosis coli

Abnormalities of jaw bones and teeth were evaluated in 37 patients with familial adenomatosis coli (FAC) by means of orthopantomography. Osteomatous radiopaque lesions were evident in 75.7 percent of FAC patients (focal type: 62.2% and wide spread type: 13.5%), odontomes in 29.7% peripheral osteomas in 13.5%, unerupted teeth in 16.2% and supernumerary unerupted teeth in 16.2%. Five of the 37 FAC patients had an unusual widespread type of radiopacity with other abnormalities. Combined abnormalities were most frequent in patients with this type in comparison with patients with the focal type. Osteomas and peripheral osteomas were revealed in this type with statistical significance. Biopsy performed in one patient revealed an osteoma. These results suggest that the multiple abnormalities seen in oral X-ray examination may predict the occult existence of familial adenomatosis coli and these widespread radiopaque lesions probably represent the most extensive manifestation of FAC.     

茎突综合征与翼钩综合征

手术治疗茎突综合征２４例和翼钩综合征１１例。前者总有效率和治愈率分别为９１．６％和５８．３％，后者为１００％和６３．６％。对两综合征的病因、病理机理、诊断和治疗作了临床比较分析     

Rieger综合征-附1例家系病例报告

目的：分析1例Rieger综合征的典型家系病例，对临床医师认识和诊断这一与口腔发育异常密切相关的罕见遗传病提供线索和信息。方法：对1例Rieger综合征患进行家系调查，对家系成员进行临床检查，修复治疗和表型分析。结果：临床诊断1例Rieger综合征病例家系，显示常染色体显性的遗传方式，该综合征可导致严重的上颌骨发育不足和多数恒牙先天缺失。结论：Rieger综合征是导致先天性牙齿缺失的重要遗传性疾病之一，由于修复治疗的需要，可能对口腔医师在这一疾病的发现和诊断中起到重要作用。     

Detection and quantification of herpesviruses in Kostmann syndrome periodontitis using real‐time polymerase chain reaction: a case report

Background/aims: Kostmann syndrome, or severe congenital neutropenia, is an autosomal recessive disease of neutrophil production and is associated with severe periodontal pathology. The aim of this study was to determine whether human cytomegalovirus (HCMV) and Epstein‐Barr virus (EBV) contribute to the pathogenesis of Kostmann syndrome periodontitis. Methods: Supragingival plaque and saliva samples were taken from a 6‐year‐old boy and his 3‐year‐old sister suffering from Kostmann syndrome, and from two age‐ and gender‐matched healthy children serving as controls. The samples were taken before and 24 months after periodontal treatment. Real‐time polymerase chain reaction (TaqMan Real‐Time PCR) assay was used to quantify HCMV and EBV DNA. Results: EBV was detected in baseline samples from the Kostmann syndrome patients but not in samples from the healthy control subjects. HCMV was only detected in the saliva of the boy with Kostman syndrome at baseline. Herpesviruses numbers decreased dramatically in the post‐treatment samples. Conclusion: EBV and HCMV were detected in the two subjects with Kostmann syndrome periodontitis. The results of the study indicate that nonsurgical treatment of Kostmann syndrome periodontitis can reduce supragingival and salivary herpes viral loads.     

Papillon-Lefèvre syndrome: analysis of HLA antigens

OBJECTIVES: Papillon-Lefèvre syndrome (PLS) is a rare disease associated with prepubertal periodontitis. Our previous studies demonstrated that three unrelated patients with PLS showed the similar antigen-specific immune responses to Actinobacillus actinomycetemcomitans. The initiation of antigen-specific immune responses was involved with human leukocyte antigens (HLA) on antigen-presenting cells. The aim of this study was to examine HLA haplotypes in the three patients with PLS. SUBJECTS AND METHODS: The three PLS patients, their mothers and the father of one patient participated in this study. HLA class I and class II antigens were determined serologically and DNA typing for DRB1 and DQB1 was performed using the restriction fragment length polymorphism-polymerase chain reaction method. RESULTS: The distribution of serologic HLA haplotypes, in two of three patients, was found to be quite similar. The DNA typing revealed that DRB1*0406, DRB1*08032, DQB1*0302, DQB1*06011 genotypes were shared in the two patients. The probability of sharing these four DNA types in unrelated individuals was nearly 1:40,000 in the Japanese population. CONCLUSION: Our results suggest that HLA antigen may be included as a possible host factor in the pathogenesis of PLS and that a genetically controlled immune response may account for an increased susceptibility to periodontal infection.     

Open mandibular condylotomy (osteotomy) with precision scaphoid staple osteosynthesis and meniscopexy. A description of the operation

The technical minutiae of the operation of open mandibular condylotomy and precision scaphoid staple osteosynthesis with meniscoplasty or meniscopexy is described and illustrated. The operation is regarded as an evolution of closed condylotomy described by Ward in 1961 and is indicated for those cases of severe and persistent temporomandibular joint pain and crepitus unresponsive to skilled conservative treatment over a reasonable time. The success of the procedure is probably the sequel of a partial denervation of the joint capsule associated with arthrotomy, the interruption of venous hypertension, the relocation of the mandibular condyle and enlargement of the joint space. The operation is also applicable to cases of active (and inactive) mandibular condylar hyperplasia wherein a condylar neck ostectomy and staple is used to ablate an active growth centre or correct a lesser degree of mandibular asymmetry.