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Objective: IgG4-related sclerosing cholangitis (IgG4-SC) is recognized as a benign steroid-responsive disease; however, little is known about the risk of development of cancer in patients with IgG4-SC and about how to counter this risk. Design: We conducted a retrospective review of the data of 924 patients with IgG4-SC selected from a Japanese nationwide survey. The incidence, type of malignancy, and risk of malignancy in these patients were examined. Then, the standardized incidence ratio (SIR) of cancer in patients with IgG4-SC was calculated. Results: Relapse was recognized in 19.7% (182/924) of patients, and cancer development was noted in 15% (139/924) of patients. Multivariate analysis identified only relapse as an independent risk factor for the development of cancer. In most of these patients with pancreato-biliary cancer, the cancer developed within 8 years after the diagnosis of IgG4-SC. The SIR for cancer after the diagnosis of IgG4-SC was 12.68 (95% confidence interval [CI] 6.89-8.79). The SIRs of cancers involving the biliary system and pancreas were 27.35 and 18.43, respectively. The cumulative survival rate was significantly better in the group that received maintenance steroid treatment (MST) than in the group that did not; thus, MST influenced the prognosis of these patients. Conclusion: Among the cancers, the risk of pancreatic and biliary cancers is the highest in these patients. Because of the elevated cancer risk, surveillance after the diagnosis and management to prevent relapse are important in patients with IgG4-SC to reduce the risk of development of cancer.K Kubota et al.IgG4-related sclerosing cholangitis and cancer 557

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Patients with cleft lip and palate (CLP) often present relative skeletal mandibular prognathism. The main reason for this is the scars from lip and palatoplasty which inhibit maxillary anterior growth. The amount of forward movement of the entire maxilla by Le Fort Ⅰ osteotomy is limited to preserve the velopharyngeal function, making it difficult to achieve the ideal forward movement of the maxilla. If velopharyngeal insufficiency occurs after an ideal forward movement, pharyngoplasty is required. Because MASDO does not move the soft

It is known that stable orthodontic treatment for skeletal Class Ⅱ open-bite cases caused by retrognathia can attain over bite by mandibular counterclockwise rotation due to intrusion of upper and lower molars. Evaluation of the temporomandibular joint is essential in these cases, as there may be progressive resorption of the condyle. Furthermore, it is important to plan surgical treatment based on restoration of the mandibular position as close to the centric relation (CR) as possible. When there is disharmony in the arch width between both jaws due to a narrowed maxillary arch in adult patients, dental or skeletal expansion may be used to correct it. For skeletal expansion, distraction osteogenesis or segmental Le Fort I osteotomy is widely used. Compared to skeletal