少汗型外胚层发育不全1例

本文报道1例外胚层发育不全病例,并分析外胚层发育不全的临床表现、发病原因及遗传方面的影响因素等.     

少汗性外胚叶发育不全1例报告

报告1例少汗性外胚叶发育不全病例,患者女,17岁,出生至今口腔内仅有1颗牙,家庭中有近三代近亲结婚史。全身皮肤汗毛少,干燥,光滑,头发稀疏,眉毛和睫毛稀少,淡黄色,腋毛和阴毛缺如。双唇外翻,突出,干燥,上、下牙槽嵴呈未发育状,仅有左上第三乳牙,畸形,无松动。采用全口义齿修复,咀嚼功能恢复。     

Ectodermal dysplasia. A seven-year case report

This case report describes the manifestations of ectodermal dysplasia and tracks the seven-year management of a male ED patient. It demonstrates the benefits of early intervention, describes restorative and orthodontic treatment rendered, and a range of future treatment options available when early intervention is employed.     

Ectodermal dysplasia: a heterogenic deviation

The 'problem' of ectodermal dysplasia is discussed in conjunction with a case report and a review of the literature. In a range of hereditary disorders all ectodermal structures can be effected in a variable way: reduction of the amount of hair, sweat glands and sebaceous glands, and a complete or partial anodontia of the deciduous and permanent dentition with malformation of the erupted teeth.     

Orthodontic management of a dental concrescence: a case report

Dental concrescence is a rare dental abnormality resulting in the joining of two teeth at the level of the cementum. This is the first reported case of the orthodontic management of a dental concrescence and the options for patient treatment are discussed. In this case, a compromised occlusal result was accepted with restorative masking of the affected teeth.     

Cleidocranial dysplasia: a case report

Cleidocranial dysplasias is an autosomal dominant disorder that presents with skeletal dysplasia. The dental manifestations are mainly delayed exfoliation of primary teeth and delayed eruption of permanent teeth, with multiple impacted supernumeraries. This report addresses the complex nature of the treatment modalities. In our patient, surgical exposure of unerupted teeth was done with orthodontic traction. Post-surgical follow-up was uneventful.     

Chondroectodermal dysplasia: a case report

Chondroectodermal Dysplasia is a disease complex consisting of bilateral manual polydactyly, chondrodysplasia of long bones resulting in acromelic dwarfism, hydroitic ectodermal dysplasia affecting principally the nails, teeth and hair and congenital heart malformations. It is necessary to identify this disease at its early stage in order to render prompt treatment. The oral manifestations are characteristic for this particular disease so that a dental surgeon can identify this condition and refer the case to a cardiologist and orthopedician for corrective surgeries. In this article, a case of a 2 1/2-year old child with chondro-ectodermal dysplasia is presented along with radiographic investigations and treatment plan.     

Surgical management of a patient with cleidocranial dysplasia: A case report

Cleidocranial dysplasia is associated with the formation of many supernumerary teeth which usually fall to erupt. In later life, cysts may form around the embedded teeth. The following report describes the management of such a case with a method which promotes satisfactory prosthodontic rehabilitation.     

Ectodermal dysplasia. A case with impacted permanent teeth

An unusual case of ectodermal dysplasia is presented. Besides the main symptoms of ectodermal dysplasia, congenitally missing teeth and the impaction of all the other existing permanent teeth make this case interesting.     

Hereditary ectodermal dysplasia: a case report

Hereditary ectodermal dysplasia is an inherited disorder involving skin, hair, nails and teeth. Two main clinical forms have been described--hypohidrotic type and hidrotic type. A case of ectodermal dysplasia with absence of hypohidrosis and defective nails has been reported. The importance of early prosthetic management has been discussed.     

Florid cemento-osseous dysplasia: a case report

Cemento-osseous dysplasias are a group of disorders known to originate from periodontal ligament tissues and involve, essentially, the same pathological process. They are usually classified, depending on their extent and radiographic appearances, into three main groups: periapical (surrounds the periapical region of teeth and are bilateral), florid (sclerotic symmetrical masses) and focal (single lesion) cemental dysplasias. Florid cemento-osseous dysplasia clearly appears to be a form of bone and cemental dysplasia that is limited to jaws. Patients do not have laboratory or radiologic evidence of bone disease in other parts of the skeleton. For the asymptomatic patient, the best management consists of regular recall examinations with prophylaxis and reinforcement of good home hygiene care to control periodontal disease and prevent tooth lose. Management of the symptomatic patient is more difficult. At this stage, there is an inflammatory component to the disease and the process is basically a chronic osteomyelitis involving dysplastic bone and cementum. Antibiotics may be indicated but may not be effective. A case of florid cemento-osseous dysplasia occurring in a 47-year-old Caucasian female is reported which was rare in regard to race and sex.     

Hypohidrotic ectodermal dysplasia: a unique approach to esthetic and prosthetic management: a case report

Hypohidrotic ectodermal dysplasia is a rare congenital disease that affects several ectodermal structures. The condition is usually transmitted as an x-linked recessive trait, in which gene is carried by the females and manifested in males. Manifestations of the disease differ in severity and involve teeth, skin, hair, nails and sweat and sebaceous gland. Ectodermal dysplasia is usually a difficult condition to manage. Prosthodontically, because of the typical oral deficiencies, and afflicted individuals are quite young to receive extensive prosthodontic treatment, which restores their appearance and helps them, for the development of positive self-image. This case report describes the management of upper jaw with over denture with copings on existing teeth i.e. two permanent peg shaped centrals as well as lateral incisors. However with adequate of retainer lower denture was provided with a new treatment modality.     

Craniometaphyseal dysplasia: case report

Craniometaphyseal dysplasia is a rare genetic bone remodeling disorder characterized by undertubulation of the long bones, especially in the lower extremities, causing deformities of the metaphyses of the long bones, and sclerosis of the skull base or cranial bone hyperostosis. The authors report a case of craniometaphyseal dysplasia in an 8-year-old Brazilian child, emphasizing the importance of precocious diagnosis of this rare genetic disorder.