Metaphyseal Chondrodysplasia is a disease characterized by metaphyseal ossification leading to shortening of stature. The extremities, especially lower, and mainly affected, resulting in a disproportionate drarfing which spares the trunk. The first case was reported in a patient with irregular metaphysis of lower extremities and hands, and severe shorteness of stature by Murk Jansen in 1934. A milder form of Metaphyseal Chondrodysplasia was noted in 1949 by Schmid, which is more common and transmitted in autosomal dominant. Mckusick recently reported another form of Metaphseal Chondrodysplasia, and other types were reported, but they are extremly rare, and of little clinical significance. In the Schmid type, patient is normal at birth, but characteristically shortness of stature, bowed leg, and waddling gait developed at biginning of walking. Ali labratory findings including serum calcium level, phosphorous, alkaline phosphatase, renal function test is normal in Metaphyseal Chondrodysplasia. The most difficult differential diagnosis is Vitamin D-resistant rickets in clinical and x-ray findings. The only treatment is careful observation and properly timed corrective surgery. Authors experienced on case of Schmid type of Metaphyseal Chondrodysplasia which had been treated with Vitamin D under impression of rickets for 1 year before the case was consulted to Orthopedic Department. Proximal tibial osteotomy and subtrochanteric valgus osteotomy were performed for the correction of genu varum and coxa vara deformity with satisfactory results for one year follow up.
Alkaline Phosphatase ; Calcium ; Congenital Abnormalities ; Coxa Vara ; Diagnosis, Differential ; Extremities ; Follow-Up Studies ; Gait ; Genu Varum ; Hand ; Humans ; Leg ; Lower Extremity ; Orthopedics ; Osteotomy ; Parturition ; Rickets ; Rickets, Hypophosphatemic ; Vitamin D ; Walking

Apert described acrocephalosyndactyly as a clinical entity in 1906. At first he included a patient with craniofacial deformities combined with syndactyly of both the upper and lower extremities. This is a rare congenital disturbance in the growth of bone and soft tissue affecting principally the head, the hands and the feet. More than 200 cases have been reported in the world literature, and three cases in Korea. Authors experienced one case of the classic pattern of Apert's syndrome and treated surgically for syndsctyly.
Acrocephalosyndactylia ; Congenital Abnormalities ; Foot ; Hand ; Head ; Humans ; Korea ; Lower Extremity ; Syndactyly

Congenital scoliosis is defined as a lateral curvature of the spine caused by congenital anomalies of the vertebral development. In this study seventeen congenital scoliosis patients seen and treated by either Milwaukee brace or surgical intervention at Department of Orthopedic Surgery, Seoul National University Hospital from December 1972 to April 1978 were evaluated according to frequency of the curve pattern, character of the deformity, method of treatment and its correctability. The following results were obtained in this study. 1. Age distribution was from 2 to 39 years and mean age was 13.4 years. 2. The most common deformity pattern was hemivertebra in 8 cases (47.1%), unilateral unsegmented bar in 4 (23.6%) and mixed type in 3 (17.6%). 3. The most common curve level was thoracic in 6 cases (35.3%), followed by thoracolumbar in 4 (23.5%) and lumbar in 4 (23.5%). 4. The best corrected type by Milwaukee brace was trapezoid vertebra (29.4%) and then mixed type(26.1%), followed by block type (20%) and hemivertebra(2.3%). 5. The operative treatment was performed in the cases of unilateral bar and progressive type with Milwaukee brace. In terms of the correctability of the curvature, the surgical intervention was better than the conservative treatment, with the result of its average correction of 33.5% that is compared with 15.9% of Milwaukee brace. 6. Halofemoral traction was effectively applied in the cases of rigid and severe curve; the initial curve 76.3 degress with 21.3% of flexibility. 7. Loss of correction in operative treatment was 2.3 degrees (6.5%).
Age Distribution ; Braces ; Congenital Abnormalities ; Humans ; Methods ; Orthopedics ; Pliability ; Scoliosis ; Seoul ; Spine ; Traction

No abstract available.
Deoxyglucose* ; Shock*

Authors examined 40,000 minifilms(boys and girls each 10,000 in junior and senior high schools) which were taken for survey of pulmonary tuberculosis for junior and senior high school students and checked the prevalence of scoliosis. It was considered lateral curvature of spine more than 10 degrees as nonpostural scoliosis, which were analyzed prevalence rate, degree of curvature, distribution of size of curvature, pattern of curve and direction of curvature. Following results were obtained. 1. The overall prevalence rate was 1.56; male 1.38. and female 1.70%(Junior high school 1.06%, Senior high school 2.05%). 2. Scoliosis secondary due to bony abnormality was found in 5 cases. 3. The most common pattern of curvature was the thoracic curve. 4. In sihgle curve, right to left ratio was 1.7, especially senior high school girls had more right curve and junior high school boys had more left curve. 5. The prevalence of senior high school students was greater than that of junior high school students and the degree of curvature was also severe in senior high school students than in junior high school students.
Busan ; Female ; Humans ; Korea ; Male ; Prevalence ; Scoliosis ; Spine ; Tuberculosis, Pulmonary

BACKGROUND: In patients with systemic lupus erythematosus (SLE), serum factors play a role in the apoptosis and necrosis of neutrophils. We intended to verify that autoantibodies including anti-dsDNA antibody are one of the factors. We also investigated the potential usefulness of simultaneous flow cytometric measurement of cytotoxicity and autoantibody binding to neutrophils in SLE sera for evaluation of disease activity. METHODS: A total of 228 sera from 48 patients with lupus nephritis (LN), 19 patients with SLE without LN, 35 patients with rheumatoid arthritis (RA) and 40 healthy males were studied. Whole blood from healthy males mixed with test sera was incubated. Autoantibody binding, apoptosis and necrosis of neutrophils were measured by flow cytometry using IgG-FITC and 7-aminoactinomycin D after a 20-hour incubation period or after adjustments of incubation conditions. The results were expressed as the test/healthy control ratio of measured values, and the correlations between these results and anti-dsDNA antibody levels were investigated. RESULTS: IgG mean fluorescence intensity (MFI) ratio was 1.0+/-0.3, 1.6+/-1.6, 2.0+/-1.5 and 4.8+/-7.5 in the healthy, RA, SLE and LN group, respectively, and showed a significant increase in the LN group when compared with the healthy group (N=20 in each group, P<0.05). Apoptosis & necrosis ratio was 1.0+/-0.2, 1.0+/-0.5, 1.6+/-1.0 and 2.6+/-1.9 in each of the above 4 groups, and showed a significant increase in the LN group when compared with the healthy (P<0.005) and RA group (P< 0.01). By immunofluorescence microscopy, increased nuclear reactivities on neutrophils by autoantibody binding were observed in 12 (60%) of 20 LN sera. All three correlations between anti-dsDNA antibody level, apoptosis & necrosis ratio and IgG MFI ratio were significant (P<0.0005). Preincubation with DNA extracts decreased both IgG MFI ratio and apoptosis & necrosis ratio significantly (N=25, P<0.05). CONCLUSIONS: Our findings confirm the previous reports of increased neutrophil apoptosis in the peripheral blood of patients with SLE. This study indicates that anti-dsDNA antibody or other antinuclear antibodies in sera are associated with an active increase in the apoptosis and necrosis of neutrophils as well as simple binding to neutrophils. This may suggest that autoantibodies increase the exposure of autoantigen DNA and exacerbate autoimmunity in the pathogenesis. Although further studies are needed, we suggest that measuring the cytotoxicity and autoantibody binding to neutrophils in SLE serum simultaneously by flow cytometry should be a useful test for evaluation of disease activity.
Antibodies, Antinuclear ; Apoptosis* ; Arthritis, Rheumatoid ; Autoantibodies ; Autoimmunity ; DNA ; Flow Cytometry ; Fluorescence ; Humans ; Immunoglobulin G ; Lupus Erythematosus, Systemic* ; Lupus Nephritis ; Male ; Microscopy, Fluorescence ; Necrosis ; Neutrophils*

BACKGROUND: This study was undertaken to reduce the side effects of epidural morphine through the addition of nalbuphine in 37 cesarean delivery. METHODS: Forty patients were divided into 2 groups; M(control) group: bolus administration of morphine 2 mg in 0.5% bupivacaine and continuous epidural 41 hour-infusion of morphine 7mg, N(experimental) group: bolus administration of morphine 2 mg in 0.5% bupivacaine combined with nalbuphine 10mg and continuous epidural 41 hour-infusion of morphine 7mg combined with nalbuphine 10mg via the Paragon infusor. RESULTS: During the postoperative 48 hours, their pain scores and side effects were recorded at 6, 12, 18, 24, 30, 36, 42 and 48 hours. The analgesic effects were good in two groups(mean VAS <3.0) and pain scores were statistically significant at 18 and 30 hour. The incidence of pruritus, nausea, vomiting and urinary retention was decreased in group N(p<0.05). CONCLUSIONS: We concluded that continuous epidural morphine combined with nalbuphine was one of recommendable methods to reduce side effects of morphine.
Bupivacaine ; Cesarean Section* ; Female ; Humans ; Incidence ; Infusion Pumps ; Morphine* ; Nalbuphine* ; Nausea ; Pregnancy ; Pruritus ; Urinary Retention ; Vomiting

No abstract available.
Neuroma*

BACKGROUND: The purpose of carotid endarterectomy is to prevent stroke regardless of past neurologic events. Major concern in the carotid endarterectomy is the inadequate blood flow of ipsilateral hemisphere during clamping of the carotid artery. It is well known that internal carotid artery back pressure means collateral cerebral blood flow. Our study is intended to determine the guideline of shunt placement according to the carotid back pressure and electroencephalographic finding. MATERIAL AND METHOD: The study population comprised of 16 consecutive patients who underwent carotid endarterectomy for carotid stenosis in our institution between from February 1996 to March 1999. There were 14 men and 2 women between the ages of 56 and 78 years(mean age 66.25+/-6.53 years). The carotid stenosis in the operative site was ranged from 61% to 95%(mean 73.8+/-12.33%) and the mean carotid stenosis of the contralateral side was 60.99+/-25.03%. During the operation, electroencephalographic monitoring was taken in all cases. The internal carotid artery back pressure was measured to estimate the collateral cerebral blood flow, and in all patients with back pressure below 40 mmHg(11 patients) and patients with complete occulusion of contralateral carotid artery(2 patients), an internal shunt was installed. RESULT: One postoperative death occurred in a patient with large evolving cerebral infarction and severe ipsilateral carotid stenosis, who underwent emergent carotid endarterectomy. The cause of death was hemorrhagic infarction in the corresponding cerebral territory. We observed that immediate operation after a major stroke negatively influenced the postoperative outcome. No intraoperative ischemic neurologic complication developed. During the follow-up upto now(mean follow-up 21.5+/-11.85 months), there has been no early or late recurrence of stroke except one patient ,in whom cerebral infarction developed in the contralateral side on the first postoperative day. CONCLUSION: At least 4-6 weeks stabilization after a stroke is recommended for surgical management. The carotid endarterectomy is an effective surgical intervention for prevention of anticipated stroke and can be performed safely if an internal shunt is used in patients whose internal carotid arterial back pressure is below 40 mmHg.
Carotid Arteries ; Carotid Artery, Internal ; Carotid Stenosis* ; Cause of Death ; Cerebral Infarction ; Constriction ; Endarterectomy ; Endarterectomy, Carotid* ; Female ; Follow-Up Studies ; Humans ; Infarction ; Male ; Recurrence ; Stroke

Iliac vein compression syndrome is a rare clinical condition in which left common iliac vein is anatomically compressed between right common iliac artery and the fifth lumbar vertebrae or pelvic brim. Patients usually have marked edema of the left leg. Although the actual incidence is unknown, this syndrome most affects women in the second to fourth decade of life. We report 4 cases of iliac compression syndrome treated by surgical intervention during the last fifteen-year period. Among them, two women have suffered from chronic intermittent swelling and pain of left leg, and two men had acute or subacute symptoms. The fibrous web in the entrance of left iliac vein into vena cava existed in one case. Including this one, two were treated by transposition of iliac artery or vein with thrombectomy. One was decompressed the arterial compression by mobilization or adhesiolysis of the surrounding vessles with thrombectomy. To the last one in which the distal common iliac vein was compressed by tortous dilated left internal iliac artery, transsection and ligation of the internal iliac artery was performed. All patients have experienced improved left leg condition after operation. except the last one in which suffered from thrombosis of left popliteal vein after the iliac decompression.
Decompression ; Edema ; Female ; Humans ; Iliac Artery ; Iliac Vein ; Incidence ; Leg ; Ligation ; Lumbar Vertebrae ; Male ; May-Thurner Syndrome ; Popliteal Vein ; Thrombectomy ; Thrombosis ; Veins