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.
Ear* ; Humans ; Tissue Donors* ; Transplants*

No abstract available.
Goldenhar Syndrome*

Luque introduced new posterior spinal instrumentation by segmental sublaminar wiring for spinal deformity in 1976. This system initially developed for the treatment of paralytic scoliosis, but now its application was extended to treatment of other types of scolisis, spinal fractures and spondylolysthesis. Advantages of this system in scoliosis are better correction force and secure internal fixation enabling early ambulation without external support. Disadvantages include longer operation time, possibilities of epidural bleeding and dural laceration. Luque instrumentation in thoracic and lumbar spine fracture is an effective means of obtaining following goals: provide reduction, maintenance of alignment, restoration of stability, prevention of deformity, low pseudarthrosis rate and early mobilization without external support. Luque instrumentation were carried out in 5 cases at Dept. of O.S. at SNUH from Oct. 1983 to Feb. 1984 with excellent results. The average follow-up period was 7.4 months and there was no complication. Two cases of unstable thoracic and lumbar spine fractures were treated with Luque instrumentation with fusion. The use of double sublaminar wiring with Luque rods, two levels above and two levels below on area of fracture provided early stabilization to allow rehabilitation without external immobilization. Three cases of paralytic scoliosis underwent Luque instrumentation by a modification of the Galveston technique with fusion. Average preoperative curve was 110°(ranged from 101° to 126°). Immediate postoperative correction was 58.7°(46.6%) and average 7 months-following result was 56.3°(48.3%). Operation time averaged 6hr 7min and blood loss was averaged 12 pints. Those patients required instrumentation from the pelvis to middorsal segment, in an effort to control the curve and associated pelvic tilting. In early follow-up the author obtained remarkable correction in paralytic curves, and the pelvic obliquity were well corrected with a pelvis and provid ing with better sitting balance.
Congenital Abnormalities ; Early Ambulation ; Follow-Up Studies ; Hemorrhage ; Humans ; Immobilization ; Lacerations ; Pelvis ; Pseudarthrosis ; Rehabilitation ; Scoliosis ; Spinal Fractures ; Spine

PURPOSE: This retrospective study was done to evaluate the status of nosocomial urinary tract infections and to determine the risk factors andtransmission route of causal IRPA through molecular epidemiology. METHOD: Two hundred ninety-nine of 423 patients admitted to the internal medicine and surgery ICU at a university hospital incity B had a positiveurine culture. Twelve of the 299 patients who had a urinary tract infection had IRPA strains. The data was collected from November 1, 2004 to January 31, 2005. The following results were obtained after the data was analyzed using percentile and UPGMA. RESULT: The rate of nosocomial urinary tract infections in the ICU was 10.8%. Therewere 16.8 cases of infection based on the period of hospitalization. There were 16.9 cases of infection based on the use of a foley catheter. The rate of nosocomial urinary tract infection in the ICU and urinary tract infections related to IRPA were higher in patients with the following characteristics: men, old age, admission through the emergency room, longer than seven days admission, severity of admitting causes, disturbance of consciousness, hydration less than 300cc in 24hours, a long course of antibiotics, a long period of foley catheterization and perineal care. Most of the microorganisms that caused the urinary tract infection were gram negative bacilli, among which P. aeruginosa was found in 70 patients (18.5%) and IRPA in 12 (4.0%). Among the 12 IRPA strains that were tested with PFGE, eight showed a dice coefficient higher than 80%, suggesting a genetic relationship. They were related with the period of hospitalization in the same ICU. These patients all received direct care for a urinary tract infection. CONCLUSION: Through these results, IRPA can be consideredas a contributing factors to urinary tract infections thus, active preventative measures are needed by the medical staff.
Adult ; Aged ; Aged, 80 and over ; Anti-Bacterial Agents/*pharmacology ; Cross Infection/*epidemiology/etiology/microbiology ; Drug Resistance, Bacterial ; Female ; Humans ; Imipenem/*pharmacology ; Intensive Care Units ; Male ; Middle Aged ; Pseudomonas Infections/drug therapy/*epidemiology ; Pseudomonas aeruginosa/classification/drug effects/*genetics ; Retrospective Studies ; Risk Factors ; Urinary Catheterization ; Urinary Tract Infections/*epidemiology/etiology/microbiology

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

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.
Emergencies* ; Emergency Medical Services*

BACKGROUND: Many physicians hesitate to discuss do-not-resuscitate (DNR) orders with patients or family members in critical situations. In the intensive care unit (ICU), delayed DNR decisions could cause unintentional cardiopulmonary resuscitation, patient distress, and substantial cost. We investigated whether the timing of DNR designation affects patient outcome in the medical ICU. METHODS: We enrolled retrospective patients with written DNR orders in a medical ICU (13 bed) from June 1, 2014 to May 31, 2015. The patients were divided into two groups: early DNR patients for whom DNR orders were implemented within 48 h of ICU admission, and late DNR patients for whom DNR orders were implemented more than 48 h after ICU admission. RESULTS: Herein, 354 patients were admitted to the medical ICU and among them, 80 (22.6%) patients had requested DNR orders. Of these patients, 37 (46.3%) had designated DNR orders within 48 hours of ICU admission and 43 (53.7%) patients had designated DNR orders more than 48 hours after ICU admission. Compared with early DNR patients, late DNR patients tended to withhold or withdraw life-sustaining management (18.9% vs. 37.2%, p = 0.072). DNR consent forms were signed by family members instead of the patients. Septic shock was the most common cause of medical ICU admission in both the early and late DNR patients (54.1% vs. 37.2%, p = 0.131). There was no difference in in-hospital mortality (83.8% vs. 81.4%, p = 0.779). Late DNR patients had longer ICU stays than early DNR patients (7.4 ± 8.1 vs. 19.7 ± 19.2, p < 0.001). CONCLUSIONS: Clinical outcomes are not influenced by the time of DNR designation in the medical ICU. The late DNR group is associated with a longer length of ICU stay and a tendency of withholding or withdrawing life-sustaining treatment. However, further studies are needed to clarify the guideline for end-of-life care in critically ill patients.
Advance Directives ; Cardiopulmonary Resuscitation ; Consent Forms ; Critical Illness* ; Hospital Mortality ; Humans ; Intensive Care Units ; Resuscitation Orders* ; Retrospective Studies ; Shock, Septic

Huntington's Disease (HD), an autosomal dominant disorder of mid-life onset, is characterized by progressive involuntary choreiform movement, psychological change and dementia. We present here two cases of Huntington's disease. One case has family history, but the other has none.
Chorea ; Dementia ; Humans ; Huntington Disease*