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*

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

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

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*

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

Aphakic or pseudophakic glau-oma is known to be at high risk for failure after filtration surgery. The authors retrospectively investigated the results of trabeculectomy in 14 eyes of 14 patients with glaucoma, seven aphakic and seven pseudophakic eyes, which were followed from six months to three years with an average ot 16.5 months. A conventional trabeculectomy without using antifibrotic agents was performed in all the eyes. The site of conjunctival incision was prepared on the virgin area of the conjunctiva; 8 superonasal, 4 inferonasal and 2 inferotemporal approaches. An average postoperative intraocular pressure reduction was 13.4 mmHg. The number of medications decreased in 11 eyes. A spontaneously developed functioning bleb was found in seven eyes. In other four eyes, the filtering bleb was formed with an aid of massage or needling. The remallllllg three eyes did not show a functioning bleb. Postoperative complications included hyphema in 3 eyes, overfiltration in 2 eyes, choroidal detachment in 1 eye, corneal edema in 1 eye and endophthalmitis in 1 eye.
Aphakia* ; Blister ; Choroid ; Conjunctiva ; Corneal Edema ; Endophthalmitis ; Filtering Surgery* ; Glaucoma ; Humans ; Hyphema ; Intraocular Pressure ; Massage ; Postoperative Complications ; Pseudophakia* ; Retrospective Studies ; Trabeculectomy

No abstract available.
Citrobacter* ; Enterobacter*