Ehlers⁃Danlos综合征的口腔颌面部表现

Ehlers-Danlos综合征是一组罕见的临床和遗传异质性的结缔组织疾病,表现为广泛的不同程度的皮肤、韧带、血管和内部器官结缔组织脆性增加,主要的临床特征包括皮肤脆弱,易擦伤,皮肤延展性过度和关节活动过度.尽管目前有关Ehlers-Danlos综合征口腔颌面部表现的临床数据并不多,但文献检索发现几乎Ehlers-Da...     

Oral manifestations of Ehlers-Danlos syndrome: report of case

First described in the beginning of this century, Ehlers-Danlos syndrome is a hereditary connective tissue disorder. This case report describes an 11-year-old girl who had frequent unexplainable bruises. During an initial orthodontic evaluation, characteristic symptoms were spotted, and a subsequent diagnosis made.     

Ehlers-Danlos syndrome: classifications, oral manifestations, and dental considerations

Ehlers-Danlos syndrome (EDS) is the name given for 6 types of connective tissue disorders. While the prevalence of this disease is small, it is seen on every continent and affects both sexes and all races. The various types of EDS are reviewed with reference given to both the older Berlin nosology and the newer Villefranche nosology. Phenotypes of EDS vary depending upon which type of collagen is altered, leading the practitioner to the diagnosis before biochemical confirmation is obtained. In this regard, because collagen is present throughout the head and neck, oral and maxillofacial manifestations of the disease are discussed and are readily noticeable to the astute dentist. Specialists in several fields of dentistry are made aware of the complications EDS can pose on treatment, healing, and follow-up care.     

Oral manifestations of Beckwith-Wiedemann syndrome

A report of case of a patient with Beckwith-Wiedemann syndrome is presented. The macroglossia associated with the syndrome necessitated long-term dental follow-up to prevent and treat problems with occlusion, speech, and esthetic appearance that might develop. Early intervention might be required if feeding or respiratory difficulties, resulting from the macroglossia, arise. The diagnosis of the syndrome should alert the dentist to the increased incidence of intra-abdominal malignancies and the need for periodic observation.     

Oral manifestations of primary immunological diseases

BACKGROUND: Primary immunodeficiencies have many oral manifestations. The clinical presentation of these diseases demonstrates the roles of different immune cells for the maintenance of oral health. METHODS: The authors reviewed selected literature describing systemic and oral manifestations of the primary immunodeficiencies published between 1966 and 1999. RESULTS: The authors found that oral candidiasis and herpetic infections are seen frequently in patients with T-cell deficiencies, while patients with B-cell deficiencies are most susceptible to bacterial infections. Periodontitis and oral candidiasis are found in some, but not all, phagocyte deficiencies. CONCLUSIONS: These findings demonstrate that T cells, B cells and phagocytes all have roles in oral immunity. CLINICAL IMPLICATIONS: Acquired conditions that affect the immune system such as diabetes, alcoholism and acquired immunodeficiency syndrome, as well as certain medications, will affect oral defense mechanisms. The effects that acquired immunodeficiencies will have on oral health can be predicted from the oral manifestations of primary immunodeficiencies.     

Oral manifestations of patients with Marfan syndrome: a case-control study

OBJECTIVE: The purpose of this study was to conduct a complete analysis of the oral abnormalities of patients with Marfan syndrome. STUDY DESIGN: Twenty three patients with Marfan syndrome and 69 healthy controls were studied. The subjects were screened for cariologic and periodontal alterations, as well as structural defects of enamel and dentin. Data analysis was performed by using the t test, the chi-square test, and regression models. RESULTS: Patients aged 0 to 17 years were significantly at risk for caries. Local hypoplastic enamel spots were more frequent in Marfan syndrome and could be related to caries history of the deciduous dentition. Root deformity, abnormal pulp shape, and pulpal inclusions were a frequent finding in patients with Marfan syndrome. Calculus and gingival indices were significantly higher in the study group as well. CONCLUSIONS: This study shows the importance of early diagnosis of oral anomalies and timely treatment of dental problems in Marfan syndrome. A series of therapeutic guidelines to be integrated in treatment strategies is proposed.     

Oral manifestations of a possible new periodic fever syndrome

Periodic fever syndrome is composed of a group of disorders that present with recurrent predictable episodes of fever, which may be accompanied by: (1) lymphadenopathy; (2) malaise; (3) gastrointestinal disturbances; (4) arthrolgia; (5) stomatitis; and (6) skin lesions. These signs and symptoms occur in distinct intervals every 4 to 6 weeks and resolve without any residual effect, and the patient remains healthy between attacks. The evaluation must exclude: (1) infections; (2) neoplasms; and (3) autoimmune conditions. The purpose of this paper is to report the case of a 41/2- year-old white female who presented with a history of periodic fevers accompanied by: (1) joint pain; (2) skin lesions; (3) rhinitis; (4) vomiting; (5) diarrhea; and (6) an unusual asymptomatic, marked, fiery red glossitis with features evolving to resemble geographic tongue and then resolving completely between episodes. This may represent the first known reported case in the literature of a periodic fever syndrome presenting with such unusual recurring oral findings.     

Orthodontic treatment protocol of Ehlers-Danlos syndrome type VI

Ehlers-Danlos syndrome (EDS) type VI is an autosomal recessive disorder of the connective tissue characterized by joint hypermobility, muscle hypotonia, scoliosis, and ocular fragility. In this case report, an EDS type VI patient with a skeletal and dental Class III malocclusion is presented and the clinical approach to his orthodontic problem is emphasized. A 17-year-old male patient presenting some major and minor symptoms of the syndrome was referred to our orthodontic department for diagnosis and treatment. The typical clinical signs confirmed the diagnosis of EDS type VI. He was a skeletal and dental Class III malocclusion patient (both mandibular protrusion and maxillary retrusion) with a noncontributory family history. He had severe crowding in the lower and upper dental arches with retruded incisors. His first treatment plan included orthognathic surgery, but because of the risks of bleeding and poor healing, we elected to treat the patient without surgery.     

Ehlers-Danlos syndrome: a case report.

A 13-year-old male patient classified as Type VIII, Ehlers-Danlos syndrome, in which the typical periodontal findings are not seen, is presented. The patient has many partially erupted deciduous teeth and partially erupted mandibular permanent incisors. It is suggested that Type VIII has two subdivisions. In subdivision A there is periodontal involvement with a normal eruption pattern; subdivision B has no periodontal involvement, but the teeth do not erupt.     

Oral manifestations of patients with Turner syndrome

OBJECTIVE: The purpose of this study was to conduct a complete analysis of orofacial abnormalities of patients with Turner syndrome. Study Design: Twenty nine patients with Turner syndrome and 30 without Turner syndrome (control group) were studied. The subjects were screened for cariological and periodontal alterations, orthodontic anomalies, tooth crown morphologic condition, alveolar arch dimensions, and temporomandibular joint function. RESULTS: Significantly lower mean values for decayed, missing, and filled teeth were found for patients with Turner syndrome as compared with the control group. Plaque and gingival indices were significantly higher in the study group. Orthodontic anomalies were more frequent and severe in patients with Turner syndrome. Our investigation showed reduced tooth crown size in the study group. The alveolar arch of the maxilla was narrower and of normal length, but the mandibular arch was shorter and broader. There was no significant alteration in temporomandibular joint function in the 2 examined groups. CONCLUSIONS: Our examination showed the importance of early diagnosis of oral anomalies and timely treatment of dental problems for patients with Turner syndrome.     

Oral manifestations of the congenital insensitivity-to-pain syndrome

The congenital insensitivity-to-pain syndrome is a sensory syndrome in which pain is impaired. It has been variably classified under a variety of terms, on occasion leading to some confusion. The condition is present at birth. The patient is usually, but not always, normal with respect to intelligence, development, and psychological adjustments. Other sensory perceptions are normal. Traumatic lesions as a result of self-mutilative acts are not uncommon, especially at an early age. The condition may not be apparent clinically until the time of initial tooth eruption. As the primary teeth erupt, the patient acquires the necessary apparatus for self-infliction of wounds to oral structures, skin, and fingernails. A case of congenital indifference to pain is presented, with clinical documentation of tooth-related problems occurring over a 2-year period and of the steps taken to correct or minimize the traumatic effects of chewing.     

Oral manifestations of Menkes' kinky hair syndrome

Menkes' Kinky Hair Syndrome (MKHS) comprises an array of clinical manifestations including hair shaft abnormalities, epidermal hypopigmentation, and progressive cerebral degeneration that are transmitted as an X-linked recessive disorder affecting copper transport pathways in primarily young males. The oral manifestations of MKHS are scantly reported to include the presence of gingival enlargement and delayed eruption of primary teeth. The purpose of this report is to present a case of MKHS describing the intraoral clinical findings.