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

No abstract available.
Heart Defects, Congenital*

No abstract available.
Child* ; Heart Arrest, Induced* ; Humans ; Oxygen*

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

BACKGROUND: In this study, we investigated the early time course of expression of the major histocompatibility (MHC) antigens, intercellular adhesion molecule-1 (ICAM-1), vascular cell adhesion molecule-1 (VCAM-1), interleukin-6 and the histopathological changes in the coronary arteries of cardiac allografts exchanged between inbred mice strains that differ in one loci of class I major histocompatibility antigen (B10.BR to B10.A). MATERIAL AND METHOD: No immunosuppressive therapy was used. Both allografts and the hearts of the recipients were harvested at 7 (group 1, n=6), 15 (group 2, n=6), 21 (group 3, n=6), and 30 (group 4, n=6) days after transplantation. They were examined by immunohistochemistry, microscopy and morphometry. All allografts had contractions at the time of harvest. RESULT: A strong MHC class I antigen expression was present on the endothelial and medial cells of the coronary arteries in group 1 and remained unchanged in the rest of the groups. However, MHC class II reactivity was none or very little at any time. Mild to moderate ICAM-1 expression was observed on the endothelial cells, but not on the medial cells at any time by 30 days. VCAM-1 expression was strong both on the endothelial and medial cells at any time. Moderate degree expression of interleukin-6 was observed from 7 to 30 day specimens. Histopathologically, percentage of affected vessels (vessels with intimal thickening) was less than 10 % in 7 day group and increased up to 50 % at 30 days. Mean percent narrowing of the lumen of the affected vessels revealed less than 20 % at 7 days and 40 % at 30 days. The area occupied by tropomyosin positive cells in the intimal lesion, graded from 0 to 3, showed gradual increase but remained between grade 0 to 1 by 30 days. Medial integrity was also well preserved at any time. Moderate perivascular mononuclear cell infiltration was observed at 7 days and it was progressively increased upto 30 days. Recipients' heart revealed no positive immunopathologic findings. CONCLUSION: In this study, the early time course of progression of the transplantation vasculopathy was demonstrated in the murine heterotopic heart transplant model.
Allergy and Immunology ; Allografts ; Animals ; Atherosclerosis ; Coronary Vessels* ; Endothelial Cells ; Heart* ; Histocompatibility ; Histocompatibility Antigens ; Immunohistochemistry ; Intercellular Adhesion Molecule-1 ; Interleukin-6 ; Mice* ; Microscopy ; Transplantation ; Tropomyosin ; Vascular Cell Adhesion Molecule-1

No abstract available.
Arteries* ; Heart*

No abstract available.
Adenocarcinoma, Clear Cell* ; Vagina*

No abstract available.
Cachexia* ; Ductus Arteriosus, Patent* ; Heart Failure* ; Heart* ; Humans ; Infant*

No Abstract available.
Cardiopulmonary Bypass* ; Heart Defects, Congenital* ; Hydrogen-Ion Concentration*

No abstract available.
Tetralogy of Fallot*