Long-term dental outcomes in patients with fibrodysplasia ossificans progressiva: a report of three cases of tooth extraction.

Fibrodysplasia ossificans progressiva (FOP) is an extremely rare autosomal dominant disorder characterized by congenital skeletal malformation and progressive heterotopic ossification. In the oral and maxillofacial region, deformity of the temporomandibular joint is a common feature of FOP, as well as restricted mouth opening derived from heterotopic ossification in the masticatory muscles. Since surgical procedures are generally not recommended because of the risk of flare-ups and increased heterotopic ossification, reports of tooth extractions and their outcomes in patients with FOP are limited.

Dental Arch Relationship Outcomes Following 2-Stage Palatoplasty for Japanese Patients With Complete Unilateral Cleft Lip and Palate: A 3-Center Study.

Objective: To compare dental arch relationship outcomes following 3 different 2-stage palatal repair protocols.

Design: Retrospective, cross sectional.

Setting: Three cleft palate centers (A, B, C) in Japan.

A Survey of Orthodontic Treatment in Team Care for Patients With Syndromic Craniosynostosis in Japan.

Objective: To understand the actual condition of orthodontic treatment in team care for patients with syndromic craniosynostosis (SCS) in Japan.

Design: A nationwide collaborative survey.

Setting: Twenty-four orthodontic clinics in Japan.

Radiographic characteristics of the hand and cervical spine in fibrodysplasia ossificans progressiva.

Fibrodysplasia ossificans progressiva (FOP) is a disabling heritable disorder of connective tissue characterized by progressive heterotopic ossification in various extraskeletal sites. Early correct diagnosis of FOP is important to prevent additional iatrogenic harm or trauma. Congenital malformation of the great toes is a well-known diagnostic clue, but some patients show normal-appearing great toes.

Dental arch relationship outcomes in one- and two-stage palatoplasty for Japanese patients with complete unilateral cleft lip and palate.

Objective: To compare dental arch relationship outcomes following one- and two-stage palatal repair.

Design: Nonrandomized, clinical trial with concurrent control.

Setting: Hokkaido University Hospital.

Maxillary protraction in patients with cleft lip and palate in mixed dentition: cephalometric evaluation after completion of growth.

Objective : To clarify the short- and long-term effects of maxillary protraction (MP) in mixed dentition in patients with unilateral cleft lip and palate (UCLP). Design : Retrospective study. Setting : University of Tokyo Hospital.

Facial morphology and occlusion of a patient with fibrodysplasia ossificans progressiva (FOP): a case report.

Fibrodysplasia ossificans progressiva (FOP) is a rare disease characterized by postnatal heterotopic ossification (HO). When HO affects the masticatory muscles, mouth opening becomes restricted. This paper presents the changes in facial morphology and occlusion of a patient with FOP who was followed from the age of 8 to age 21.

Evaluation of mandibular hypoplasia in patients with hemifacial microsomia: a comparison between panoramic radiography and three-dimensional computed tomography.

Objective: To compare the accuracy of three-dimensional computed tomography (3D-CT) and panoramic radiography in the evaluation of mandibular hypoplasia in patients with hemifacial microsomia (HFM).

Design: Retrospective study of imaging data. Setting : Images selected from the archives of the University of Tokyo Hospital.

A novel mutation of ALK2, L196P, found in the most benign case of fibrodysplasia ossificans progressiva activates BMP-specific intracellular signaling equivalent to a typical mutation, R206H.

Fibrodysplasia ossificans progressiva (FOP) is a rare autosomal dominant congenital disorder characterized by progressive heterotopic ossification in muscle tissues. Constitutively activated mutants of a bone morphogenetic protein (BMP) receptor, ALK2, have been identified in patients with FOP. Recently, a novel ALK2 mutation, L196P, was found in the most benign case of FOP reported thus far.

Primary treatment for cleft lip and/or cleft palate in children in Japan.

Objective: To investigate current trends in primary treatment for children with cleft lip and/or cleft palate in Japan.

Design: Nationwide, retrospective study under the direction of the Academic Survey Committee of the Japanese Cleft Palate Association based on analysis of data obtained via a booklet-style questionnaire completed by institutions providing primary treatment for cleft lip and/or palate patients. PARTICIPANTS, PATIENTS: Patients were 4349 children undergoing primary repair for cleft lip and/or palate at 107 participating institutions between 1996 and 2000.

Deformity of the great toe in fibrodysplasia ossificans progressiva.

Background: As invasive medical procedures can induce permanent heterotopic ossification in fibrodysplasia ossificans progressiva (FOP), caution should be exercised in clinical practice. The present study was conducted to examine the characteristics of the great toe deformity in patients with FOP, which may lead to an early diagnosis of this condition.

Methods: The subjects consisted of 31 feet from 16 FOP patients (8 males, 8 females) with an average age of 17.

Treatment of ankylosed maxillary central incisors by single-tooth dento-osseous osteotomy and alveolar bone distraction.

When tooth ankylosis occurs in growing children, the ankylosed tooth fails to erupt and gradually positions itself below the occlusal plane. This causes functional and esthetic problems, and orthodontic treatment is often impossible. To clarify this problem, we developed a new treatment protocol for the movement of ankylosed teeth.

[Cytokines in bone diseases. BMP signaling and fibrodysplasia ossificans progressiva].

Fibrodysplasia ossificans progressiva (FOP) is a rare autosomal dominant disorder characterized by progressive heterotopic bone formation in skeletal muscle tissue. In 2007, FOP was authorized as one of the Intractable Disorders by the Ministry of Health, Labour and Welfare of Japan. The Research Committee on FOP is working on the molecular mechanisms underlying heterotopic bone formation and the development of new treatments for FOP.

Novel mutations of the HOXD13 gene in hand and foot malformations.

Homeobox genes encode a set of transcription factors of fundamental importance for body patterning during embryogenesis. Hoxa9-a13 and Hoxd9-d13 play an especially important part in vertebrate limb development. Synpolydactyly (SPD) is characterized by various malformations of the limbs.

Segmental alveolar distraction for the correction of unilateral open-bite caused by multiple ankylosed teeth: a case report.

Ankylosed teeth fail to erupt to meet their counterparts in the opposite jaw. In cases where ankylosis occurs in multiple teeth, the occlusion shows an open bite. This article describes a case of unilateral open bite caused by multiple ankylosed teeth, where treatment involved segmental alveolar bone distraction.

Assessment of dental arch relationships in Japanese patients with unilateral cleft lip and palate.

Objective: Evaluation of the dental arch relationships of Japanese patients with unilateral cleft lip and palate (UCLP) from the orthodontic clinic of the University of Tokyo Hospital (UTH) compared with patients treated by the Oslo Cleft Team, Norway.

Design: Retrospective study and comparison with previous reports.

Materials: Dental models of 24 patients with UCLP in UTH taken before orthodontic treatment and before alveolar bone grafting were included.

Reconstruction necessitated by severe hypoplasia or a columella defect.

In reconstruction necessitated by severe hypoplasia or a columella defect, the surgeon must consider various factors in each case, such as the characteristic columellar shape, color match, texture, patient age, original disease, and surrounding scars. In these cases, reconstruction of both the cartilaginous strut and the overlying skin is necessary and important to obtain good results. The authors report three cases of reconstruction of the columella with satisfactory results.

Targeting of iNOS with antisense DNA plasmid reduces cytokine-induced inhibition of osteoblastic activity.

Proinflammatry cytokines, tumor necrosis factor-alpha combined with interleukin-1beta, induce excessive production of nitric oxide (NO) and its cytotoxic metabolite peroxynitrite (ONOO-) via inducible nitric oxide synthase (iNOS) in murine osteoblasts. In this study, to properly estimate the effects of antisense DNA of iNOS on osteoblastic activity, we produced transformed cell lines with antisense plasmid that specifically targets the iNOS gene for potential long-lasting inhibition. Transformed antisense cell lines were identified by 1) the detection of antisense transcripts, 2) the attenuated expression of iNOS protein, 3) the reduction of NO synthase activity, and 4) the level of NO production.

The treatment of hemimandibular hyperplasia preserving enlarged condylar head.

Objective: To present a case of hemimandibular hyperplasia (HH) treated with orthognathic surgery that preserves the condyle without disturbing mandibular function.

Methods: A 27-year-old woman with HH was treated with orthognathic surgery preserving the enlarged condylar head. Radiographic examination showed typical enlargement of the right condyle, elongation of the right ascending ramus and mandibular body, and tilted occlusal plane.

A case of a Japanese patient with cleidocranial dysplasia possessing a mutation of CBFA1 gene.

Cleidocranial dysplasia (CCD) is an autosomal dominant human bone disease characterized by hypoplastic or aplastic clavicles, wide cranial sutures, supernumerary teeth, short stature, and other skeletal disorders. Recently, various mutations of the core binding factor (CBFA1) gene have been detected in CCD patients. The CBFA1 gene is a member of the runt family of transcription factors.