Frontometaphyseal dysplasia is an uncommon genetic syndrome affecting bone and connective tissue. This condition is characterized by the prominence of supraorbital ridges, hyperostosis of the skull, mandibular hypoplasia and malocclusion with antegonial notching, underdeveloped paranasal flaring of the long bone, and a widened iliac wing. Frontometaphyseal dysplasia has an X-linked dominant trait. Clinical manifestations are more severe in males and have extreme variability in females. We present an 11-year-old boy who had frontometaphyseal dysplasia with disfigured face due to bilaterally urinary tract malformation and chronic urinary tract infection. To improve facial appearance, his prominent supraorbital ridges were contoured by ostectomy and burring through bicoronal incision. Urologic operation was performed simultaneously. Histologically, resected bony specimen revealed bony tissue with normal trabecular pattern suggesting exostotic bone. The patient healed without any postoperative complications and he and his parents were satisfied with his final facial morphology.
Child ; Connective Tissue ; Female ; Humans ; Hyperostosis ; Male ; Malocclusion ; Parents ; Postoperative Complications ; Skull ; Urinary Tract ; Urinary Tract Infections

Restoration of anteroposterior projection of the zygomatic body and facial width are key elements in treatment of complex zygomatic fractures. Traditionally, it has been understood that for most fractures of the zygoma, three-point fixation of the zygomaticofrontal suture, inferior orbital rim, and zygomaticomaxillary buttress generally produces exact structural restoration. Recently, the importance of accurate reduction of the zygomatic arch through coronal incision has been favored in complex zygomatic fractures. Though coronal incision has the advantages of accurate reduction and fixation as a result of extensive exposure of the fractured area, this procedure also has many disadvantages including injury to the facial nerve, paresthesia, alopecia, scar formation, longer operating time, protracted hospitalization, etc. As well, there have been no objective data to prove the fact that four-point fixation through coronal incision is superior to traditional three-point fixation. From May, 1994 to December, 1998, the authors treated 45 patients by traditional three-point (n=20) and coronal four-point fixation (n=25) with random sampling. To assess the difference between the two methods the authors measured the axial angle of the zygoma, the axial angle of the zygomatic arch, and the degree of zygomatic arch inclination on submentovertex X-ray, and then analyzed the measurements by the paired T-test(p < 0.05). As a result, the axial angle of the zygoma and zygomatic arch assessed by anteroposterior projection, as well as the facial width showed no statistical difference between the two groups, respectively(p = 0.26, p = 0.18). Mean while, the degree of zygomatic arch inclination representing the local contour of the fractured zygomatic arch was statistically significant between the two groups(p < 0.05). Thus, the traditional three-point fixation method may be widely acceptable in treatment of complex zygomatic fractures with anterior three-point or zygomatic arch comminution.
Alopecia ; Cicatrix ; Facial Nerve ; Hospitalization ; Humans ; Orbit ; Paresthesia ; Sutures ; Zygoma ; Zygomatic Fractures

The tibial pilon fracture has been described as difficult fracture to manage. We have reviewed 23 cases of tibial pilon fractures from Mar. 1987 to Feb. 1993 at our hospital. 1. The fractures were classified into five types according to the system of Ovadia and Beals and the methods of treatment were divided into two groups; 9 cases were treated with Ilizarov device(Group I). 6 cases out of Group I were type 3, 4 and 5. Other methods were performed in 14 cases(Group II). 8 cases out of Group II were type 3, 4, and 5. 2. In type 3, 4 and 5 fractures, there were 86 per cent good and fair radiographic results in Group I and 63 per cent good and fair results in Group II. 3. Satisfactory results were obtained by the treatment of Ilizarov method especially in type 3, 4 and 5 fractures. The advantages of Ilizarov device were its primary reduction with ligamentotaxis, easy open reduction due to proximal and distal stabilization, minimal soft tissue injury and minimal internal fixation.
Ilizarov Technique ; Soft Tissue Injuries

Hyper-activated depressor septi nasi is an important factor caused a nasal tip drooping and many studies have been carried out, however it still remains controversial. This study presents a surgical modality for its intervention, based on the anatomical study. Eleven fixed and six fresh cadavers were used for the study. We found that the depressor septi nasi was consisted of three fascicles. Medial fascicles were inserted into the dermocartilaginous ligament, and deep fibers of the medial fascicles were attached to the anterior nasal spine. After superficial fibers were interdigitated with the orbicularis oris, they were attached to the alveolar bone. Intermedial fascicles were inserted to the footplates of the medial cruses and the caudal septum. After they were interdigitated with the medial fascicle and the orbicularis oris, they were attached to the alveolar bone. From April to August 2008, five patients had surgical intervention for hyper-activated depressor septi nasi. Medial and intermedial fascicles were detached completely from the anterior nasal spine and the septum through intranasal approach. Tip droopings were improved in all cases. Specific complications were not found. We believe that surgical intervention through intranasal approach is a useful method for correction of tip drooping.

A symmetric approach, using external rhinoplasty, is presented to aid the plastic surgeon in obtaining improved aesthetic and functional results in patients with postoperative nasal deformities. The external approach yields a full visualization of the underlying nasal framework and intraoperative evaluation of the deformities to be corrected subsequently. The nasal septal cartilage is unequivocally one of the best graft sources for reconstruction of the dorsum, columella or tip. It has fairly even surface and pliability in carving and shaping the graft. The graft can be obtained during the surgery with less morbidity and prepared easily for need of the shape. The only real disadvantage is the limited amount of cartilage that can be obtained from the septum. The dorsal and caudal rims, one or more cm in width, of the nasal septum should not be disturbed to maintain the nasal frame during harvesting the septal graft. Authors invented novel instruments, J & D knife and Flat (Spatula) suction tip, and have employed the devices for harvesting the septal cartilage. We were unable to gain enough amount of the cartilage by using a swivel knife or cartilage scissors. The septal cartilage can be resected as much as needed with newly invented instruments which facilitate a separation(method) technique.
Cartilage* ; Congenital Abnormalities ; Humans ; Nasal Septum ; Pliability ; Rhinoplasty* ; Suction ; Transplants

Background and Objectives: Human CD34＋hematopoietic stem cells can reconstitute the human hematopoietic system when transplanted into immunocompromised mice after irradiation. Human leukapheresis peripheral blood (LPB)-and cord blood (CB)-derived CD34＋ cells have a similar capacity to reconstitute myeloid lineage cells in a humanized mice (hu-mice) model. However, potent stem cells, such as CB-CD34＋ cells, efficiently reconstitute the lymphoid system in vivo compared to LPB-CD34 ＋ cells. Modeling the human hematolymphoid system is vital for studying immune cell crosstalk in human xenografted mice, with CB-CD34＋ cells used as an optimized cell source because they are essential in reconstituting lymphoid lineage cells. Methods: and Results: In this study, we established hu-mice that combined human characteristics with long-term survival and investigated the efficiency of the engraftment of lymphoid lineage cells derived from LPB- and CB-CD34＋cells in the bone marrow, spleen, and LPB. We found an overall increase in the transcriptional activity of lymphoid lineage genes in CB-CD34＋ cells. Our results revealed that potent CB-CD34＋ cells displaying a general upregulation of the expression of genes involved in lymphopoiesis could contribute to the hematolymphoid system in the humanized mice model with longevity. Conclusions Our data suggest that humanized mouse model by usage of CB-CD34 ＋ cells displaying high expression of TFs for lymphoid lineage cells can contribute to study the immune response against lymphocytes.

Background and Objectives: Human CD34＋hematopoietic stem cells can reconstitute the human hematopoietic system when transplanted into immunocompromised mice after irradiation. Human leukapheresis peripheral blood (LPB)-and cord blood (CB)-derived CD34＋ cells have a similar capacity to reconstitute myeloid lineage cells in a humanized mice (hu-mice) model. However, potent stem cells, such as CB-CD34＋ cells, efficiently reconstitute the lymphoid system in vivo compared to LPB-CD34 ＋ cells. Modeling the human hematolymphoid system is vital for studying immune cell crosstalk in human xenografted mice, with CB-CD34＋ cells used as an optimized cell source because they are essential in reconstituting lymphoid lineage cells. Methods: and Results: In this study, we established hu-mice that combined human characteristics with long-term survival and investigated the efficiency of the engraftment of lymphoid lineage cells derived from LPB- and CB-CD34＋cells in the bone marrow, spleen, and LPB. We found an overall increase in the transcriptional activity of lymphoid lineage genes in CB-CD34＋ cells. Our results revealed that potent CB-CD34＋ cells displaying a general upregulation of the expression of genes involved in lymphopoiesis could contribute to the hematolymphoid system in the humanized mice model with longevity. Conclusions Our data suggest that humanized mouse model by usage of CB-CD34 ＋ cells displaying high expression of TFs for lymphoid lineage cells can contribute to study the immune response against lymphocytes.

Myotonic dystrophy 1 (DM1) is a multi-system disorder characterized by endocrine defects that include testicular and tubular atrophy, oligospermia and azoospermia, and increased follicle-stimulating hormone levels. We describe a rare case of DM1 presenting as infertility in a 29-year-old man.
Adult ; Atrophy ; Azoospermia ; Follicle Stimulating Hormone ; Humans ; Infertility ; Infertility, Male ; Male ; Myotonic Dystrophy ; Oligospermia

BACKGROUND: In contrast to fillers made from artificial substances, platelet-rich fibrin matrix (PRFM) filler does not cause hypersensitivity reactions or foreign body reactions. PRFM is also highly accessible in terms of cost. Hence, in this study, the efficacy of PRFM for soft tissue augmentation and volume maintenance was evaluated in an animal experiment. METHODS: Twenty nude mice were injected with hyaluronic acid filler, fibrin glue, PRFM filler, and normal saline (control). The remaining volume was measured 4 times over the course of 8 weeks using the volumetric taping bowl method and magnetic resonance imaging. RESULTS: All nude mice survived and showed no signs of infection, such as erythema or edematous changes, during the study period. Migration of the injected substance was not detected at 2, 4, or 8 weeks after the procedure. The remaining volumes of normal saline at 2, 4, and 8 weeks were 10.50%, 2.00%, and 0.00%; fibrin glue, 20.50%, 9.00%, and 2.50%; hyaluronic acid filler, 82.00%, 35.00%, and 17.33%; and PRFM filler, 70.31%, 26.75%, and 14.37%, respectively. CONCLUSIONS: PRFM filler had a high soft-tissue filling capacity compared with the control. It also showed a similar effect to hyaluronic acid filler. Thus, PRFM filler could be a good alternative for correcting soft-tissue deficits.
Animal Experimentation ; Animals ; Cosmetic Techniques ; Erythema ; Fibrin Tissue Adhesive ; Fibrin* ; Foreign Bodies ; Hyaluronic Acid ; Hypersensitivity ; Magnetic Resonance Imaging ; Methods ; Mice ; Mice, Nude* ; Platelet-Rich Plasma

Isolation of induced pluripotent stem cells (iPSCs) from fully differentiated somatic cells has revolutionized existing concepts of cell differentiation and stem cells. Importantly, iPSCs generated from somatic cells of patients can be used to model different types of human diseases. They may also serve as autologous cell sources that can be used in transplantation therapy. In this study, we investigated the neuronal properties of an iPSC line that is derived from human neonatal foreskin fibroblasts (FS-1). We initially examined the morphology and marker expression of FS-1 cells at undifferentiated stage. We then spontaneously differentiated FS-1 cells in suspension culture and examined the expression of markers representing three germ layers. We finally differentiated FS-1 cells into neuronal lineages by co-culturing them with PA6 stromal cells, and found that, under the conditions we used, they have a tendency to differentiate into more forebrain-type neurons, suggesting that FS-1 iPSC-derived neural cells will be useful to be used in cell therapy of stroke or Huntington's disease, among others. Taken together, FS-1 cells derived from human neonatal fibroblasts exhibit very similar properties with human ES cells, and can provide useful sources for cell therapy and various other applications.
Cell Differentiation ; Fibroblasts ; Foreskin ; Germ Layers ; Humans ; Huntington Disease ; Induced Pluripotent Stem Cells ; Infant, Newborn ; Neurons ; Pluripotent Stem Cells ; Stem Cells ; Stroke ; Stromal Cells ; Tissue Therapy ; Transplants