Romberg's disease is an uncommon condition manifested by progressive hemifacial atrophy of the skin, soft tissue and bone. Facial asymmetry with soft tissue deficiency in Romberg's disease causes a significant disability affecting the social life and can bring about many psychological problems. The aim of surgical treatment is cosmetic amelioration of the defect. Several conventional reconstructive procedures have been used for correcting facial asymmetry. They include fat injections, dermal fat grafts, silicone injections, cartilage and bone grafts, pedicled flaps and free flaps. We report our experience with 6 patients involving 6 free flaps with a minimun of 1 year follow-up who were treated from October 1989 to March 1998. All patients were classified as having moderate to severe atrophy. The average age of disease onset was 4.5 years. The average duration of atrophy was 5.2 years. No patient was operated on with a quiescent interval of less than 1 year. The average age at operation was 14.1 years, ranging from 10 to 24 years. Follow-up ranged from 1 to 9 years. Reconstruction was performed using 2 groin dermofat free flaps and 4 latissimus dorsi muscular free flaps. To achieve the finest symmetrical and aesthetic results, several ancillary procedures were performed in 4 patients. These procedures included Le-Fore 1 1/2 leveling osteotomy, sagittal split ramus osteotomy, reduction malarplasty and angleplasty, rib and calvarial bone graft, correction of alopecia and additional dermofat graft. All patients were satisfied with the results. We believe that free flap is one of the best choices for contoured restoration of facial asymmetry in Romberg's disease.
Alopecia ; Atrophy ; Cartilage ; Facial Asymmetry ; Facial Hemiatrophy ; Follow-Up Studies ; Free Tissue Flaps* ; Groin ; Humans ; Osteotomy ; Osteotomy, Sagittal Split Ramus ; Ribs ; Silicones ; Skin ; Superficial Back Muscles ; Surgical Flaps ; Transplants

No abstract available.
Goldenhar Syndrome*

No abstract available.
Ear* ; Humans ; Tissue Donors* ; Transplants*

No abstract available.
Animals ; Interleukin-2 ; Mice*

No abstract available.
Citrobacter* ; Enterobacter*

Pleomorphic (undifferentated) carcinoma is a rare histologic type of carcinomas of the gallbladder and an atypical carcinoid is thought to be an intermediated type between carcinoid tumor and small cell carcinoma. Dense core "neurosecretory" granules can be found in the above mentioned tumors. We experienced a case of a double tumor of the gallbladder in a 51-year old male patient. Grossly, a large solid mass, about 5.0 cm in diameter, was found in the fundic portion and the neck portion also had a small 1.5 cm-sized polypoid mass. Microscopically, these lesions had features of pleomorphic carcinoma and atypical carcinoid, respectively. Immunohistochemically, they manifested reactivity for neuron specific enolase. Ultrastructural study revealed neurosecretory granules in the cytoplasms of tumor cells of the fundic and neck masses. Although light microscopic features of these tumor masses are quite different, we consider that these tumors represent a spectrum of neuroendocrine differentiation.
Male ; Humans

No abstract available.
Drug Resistance, Microbial* ; Plasmids* ; Pneumonia*

Cancer is considered to occur through abnormal growth and differentiation processes, in which oncogenes and tumor suppressor genes are deeply related. Cellular responses to DNA-damaging agents are believed to be critical determinants of human tumorigenesis. Cell cycle arrests and DNA repair following DNA damage require the coordination of multiple gene products that, as a whole, serve to maintain the integrity of the genome. Within the cell cycle, both G1-S and G2-M phase transitions are under constant surveillance by checkpoint genes for the protection of cells from either exogenous or endogenous DNA-damaging agents. p53 tumor suppressor gene mediates cell cycle perturbations in response to DNA damage, and play a role in cell death, genetic stability, and cancer susceptibility. Recently, gene therapy with p53 tumor suppressor gene is expected as a new effective therapeutic strategy in many kinds of cancer. By using retroviral vector system, we transduced p53 tumor suppressor gene into human osteosarcoma cells, and analysed its growth suppression and apoptosis inducing effects. Combined effects of p53 gene therapy with chemotherapeutic agent or radiation were also analysed. Titer of ecotrophic p53 retrovirus was 5.0x10/ml, and that of amphotrophic p53 retrovirus was 2.0x10/ml when NIH3T3 cells were used as target cells. Human osteosarcoma cells infected with amphotrophic p53 retroviruses showed increased p21waf1 gene expression, which acts as a major cyclin-dependent kinase inhibitor in DNA damage responses. In normal DMEM media, human skin fibroblasts infected with amphotrophic p53wt retroviruses showed very slow growing (1.7 fold increase in doubling time) and very low saturation density (50% decrease in cell density). In media containing chemotherapeutic agent, human osteosarcoma cells infected with p53wt retroviruses died rapidly; 75% of them died within 4 days and all of them died within 10 days of incubation with chemotherapeutic agent. Their DNAs were extracted and electrophoresed in agarose gel, and we identified DNA ladders characteristic of apoptotic cell death. When human osteosarcoma cells infected with p53 retroviruses were irradiated with ultraviolet light, more than 95% of cancer cells died within 1 day; whereas mock infected cells showed only less than 5% of cell death. These findings suggest that retroviral vector mediated p53 tumor suppressor gene transfer into cancer cells can suppress tumor cell growth and decrease tumor cell density effectively. These findings also suggest that effective induction of tumor cell apoptosis can be obtained when p53 gene therapy is used in combination with chemotherapy or radiotherapy.
Apoptosis ; Carcinogenesis ; Cell Count ; Cell Cycle ; Cell Cycle Checkpoints ; Cell Death ; DNA ; DNA Damage ; DNA Repair ; Drug Therapy ; Fibroblasts ; Gene Expression ; Genes, p53 ; Genes, Tumor Suppressor* ; Genetic Therapy* ; Genome ; Humans ; Oncogenes ; Osteosarcoma ; Phase Transition ; Phosphotransferases ; Radiotherapy ; Retroviridae* ; Sepharose ; Skin ; Ultraviolet Rays ; Zidovudine

No abstract available.
Hepatitis B virus* ; Hepatitis B* ; Hepatitis*

No abstract available.
Hepatitis B virus* ; Hepatitis B* ; Hepatitis*