Intracystic injection of pingyangmycin(PYM) might be a more favorable option for treatment of oral and plunging ranulas

Ranulas are mucoceles that develop as a result of mucous extravasation from the sublingual gland and typically present in the floor of mouth. The treatment of ranulas are various, mainly including surgical and nonsurgical methods. The preferred treatment of oral and plunging ranulas is still uncertain. According to the complications associated with surgical therapy, nonsurgical sclerotherapy has been advocated by clinicians for its advantages of less injury, no scar,less suffering, etc. Recently, it was reported that OK-432 was a relatively effective sclerosing agent for both lymphatic malformations and ranulas, although it has a high rate of recurrence after treating ranulas. Pingyangmycin is another reported conventional sclerosing agent for lymphatic malformations. Herein, we hypothesize that intracystic injection of pingyangmycin may be an optimal method for the treatment of ranulas.

The clinical significance of bimodal growth pattern in the treatment of hemangiomas in infancy and children

Hemangiomas are the most common benign vascular neoplasms of infancy and children, the life cycle of which involves a phase of rapid proliferation in the neonatal period, followed by spontaneous involution, usually during childhood [1]

Dlx2 over-expression:a possible mechanism for first branchial arch malformation

The first branchial arch malformation(FBAM) is a rare congenital defect associated with anomalous development of the first and second branchial arches.Cause of FBAM still remains unknown,and is thought in most cases to be multifactorial,involving both genetic and enviromental factors.Dlx2 as a member of the Dlx homeobox gene family,plays a crucial role in the development of the first branchial arch.The tissues regulated mainly by Dlx2 are coincident with the tissues mainly involved in FBAM.Dlx2 over-expression generated by electroporation transfection can disturb the migration and differentiation of cranial neural crest cells(CNCCs),which migrate to the branchial arches and in turn give rise to much of the facial skeleton and connective tissues.Furthermore,Dlx2 over-expression can be found in the first branchial arch spontaneous mutant mice.So we hypothesize that Dlx2 over-expression mutation causes FBAM due to an increase in cell-cell adhesion and inhibiting the migration of CNCC to the first branchial arch in the early stage,or migrating to an incorrect position and can't differentiate into normal tissues.What an exact role of Dlx2 over-expression in FBAM remains to be investigated and Dlx2 over-expression transgenic mouse will be a nice model for further research in FBAM.

An ectopic study of tissue-engineered bone with Nell-1 gene modified rat bone marrow stromal cells in nude mice

Background Tissue engineering techniques combined with gene therapy have been recently used to improve osteogenesis. NEL-like molecule-1 （Nell-1）, a novel growth factor, has been reported to have specificity for osteochondral lineage. The study assessed the osteogenic differentiation of rat bone marrow stromal cells （bMSCs） after Nell-1 gene modification and examined its ectopic bone formation ability in a nude mice model with tissue engineering technique. Methods bMSCs obtained from Fischer 344 rats were transduced with either AdNell-1 （Nell-1 group） or Ad-β-galactosidase （AdLacZ, LacZ group） or left untransduced （untransduced group）. The expression of Nell-1 protein was determined by Western blotting and transfer efficiency was assessed, mRNA expressions of osteopontin （OP）, bone sialoprotein （BSP） and osteocalcin （OC） were assessed by real-time PCR 0, 3, 7, 14, and 21 days after gene transfer. Alkaline phosphatase （ALP） activity was measured and von Kossa test was also conducted. Finally, with a tissue engineering technique, gene transduced bMSCs, combining with β-tricalcium phosphate （β-TCP） at a concentration of 2×10^7 cells/ml, were implanted at subcutaneous sites on the back of nude mice. Four weeks after surgery, the implants were evaluated with histological staining and computerized analysis of new bone formation. Results Under current transduction conditions, gene transfer efficiency reached （57.9±6.8）%. Nell-1 protein was detected in Nell-1 group but not in untransduced group and LacZ group. Induced by Nell-1, BSP and OP expression were increased at intermediate stage and OC expression was increased at later stage. ALP activity and the number of calcium nodules were highest in Nell-1 group. Four weeks after implanted into nude mice subcutaneously, the percentage of new bone area in Nell-1 group was （18.1±5.0）%, significantly higher than those of untransduced group （11.3±3.2）% and LacZ group （12.3±3.1）% （P〈0.05）. Conclusions This study has demonstrated the ability of Nell-1 to induce osteogenic differentiation of rat bMSCs in vitro and to enhance bone formation with a tissue engineering technique. The results suggest that Nell-1 may be a potential osteogenic gene to be used in bone tissue engineering.

A new mutation of PTCH gene in a Chinese family with nevoid basal cell carcinoma syndrome

Background Nevoid basal cell carcinoma syndrome （NBCCS） is a rare autosomal dominant disease characterized by a combination of development anomalies and a predisposition to tumour formation. Mutation of patched gene （PTCH）, considered the molecular defect of NBCCS, in a Chinese NBCCS family was investigated in this study. Methods Genomic DNA was isolated from blood samples of all 12 members of this family. The mutated PTCH gene was screened by polymerase chain reaction amplification and direct sequencing. Results A new mutation of 3 bp （GAT deletion） was found in all seven affected members of this family. This mutation caused one aspartate deletion in the fourth transmembrane domain of the PTCH protein located within the sterol sensing domain （SSD）. This deletion was not found in any unaffected members of this family nor in 200 control samples.Conclusions Our findings suggest that one 3-bp deletion in PTCH gene was the cause of nevoid basal cell carcinoma in a Chinese family through affecting the conformation and function of PTCH protein.