Cystic fibrosis (CF) is an autosomal recessive disease that is very rare in Asians: only a few cases have been reported in Korea. We treated a female infant with CF who had steatorrhea and failure to thrive. Her sweat chloride concentration was 102.0 mM/L. Genetic analysis identified two novel mutations including a splice site mutation (c.1766+2T>C) and a frameshift mutation (c.3908dupA; Asn1303LysfsX6). Pancreatic enzyme replacement and fat-soluble vitamin supplementation enabled the patient to get a catch-up growth. This is the first report of a Korean patient with CF demonstrating pancreatic insufficiency. CF should therefore be considered in the differential diagnosis of infants with steatorrhea and failure to thrive.
Alternative Splicing ; Base Sequence ; Cystic Fibrosis/complications/diagnosis/*genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/*genetics ; Diagnosis, Differential ; Exocrine Pancreatic Insufficiency/complications/diagnosis/*genetics ; Female ; Frameshift Mutation ; Humans ; Infant ; Republic of Korea ; Steatorrhea/diagnosis

No abstract available.

No abstract available.
Empyema*

No abstract available.
Child* ; Humans ; Lung Diseases, Interstitial*

130 cases of the suprarcondylar fractures of the humerus in children were analysed. 60 cases were immobilized with plaster splint without closed reduction. 22 cases were closedly reduced and immobilized with plaster splint. 47 cases were closedly reduced and were fixed percutaneously with K-wires. 39 cases were operatively reduced and were fixed with K-wires. The following results were obtained. 1. The average age was 7.9 years, and the male and female ratio was 2.7: 1. 2. The extension type was 96.2% and the flexion type was 3.8%. 3. The left side (64.6%) was more frequently involved. 4. As associated complications, nerve injury (4.6%), vascular injury (2.3%), open fracture (2.3%), and associated fracture (2.3%). were developed. During treatment incomplete ulnar nerve palsy (2.3%), pin tract superficial infection(3.1%), severe cubitus varus deformity(4.6%), severe limitation of elbow motion(11.6%) were also developed. 5. Final results in the Holmberg's 4 Groups according to Mitchell & Adams' criteria showed that the more the displacement of fracture is increased, the more the poor result is also increased. 6. In spite of the selection of the more severe cases for surgery, the results of the operative cases were better than those achieved by closed reduction in the easier cases. 7. In the displaced fractures, immediate open reduction brings the satisfactory results and minimizies the sequellae.
Child ; Elbow ; Female ; Fractures, Open ; Humans ; Humerus ; Male ; Splints ; Ulnar Neuropathies ; Vascular System Injuries

In spite of numerous dinical and patho-anatomical studies made in the past, there are still different opinions concerning the mechanism of low back pain. The sciatic symptoms in degenerative lumbar disc disease may arise from the direct mechanical effect of pressure exerted by protruding disc material on nerve roots, and that such symptoms may therefore be relieved by surgical decompression of the nerve roots, has been widely accepted for nearly 40 years. There is, however, a good deal of evidence, both from operative and histological findings as well as from indirect sources, which suggests that inflammatory changes present in and around affected nerve roots may also be of importance in the production of sciatic symptoms in lumbar disc disease. The possibility that, at least in some cases, these inflammatory changes could contribute significantly or even predominantly to the production and persistence of sciatic symptoms, has stimulated many attempts to treat sciatica by means of local antiinflammatory measures in particular, corticosteroid injections. We have studied the effect of the injection of mixture triamcinolone and local anesthetic (1% xylocaine or 0.5% marcaine) comparing with the effect of usual conservative treatment of low back pain and sciatica. Of the 92 patients suffered from lumbago & sciatica, who were treated from 1979. 7 to 1981. 7 in Korea and Jae Il general hospital, 44 patients were treated with intermittent epidural block, 12 patients with continuous epidural block and 36 patients were treated only with usual conservative treatment. The results of clinical observation were as follows: 1. The causative disease were herniated nucleus pulposus in 60 patients (64%), lumbar sprain in 12 (13%), degenerative joint disease of spine in 11 (12%),… 2. The epidural block cases were divided into acute group (symptoms less than 3 months) and chronic group (symptoms longer than 3 months). The over all improvement of the acute group was 22 of 27(81%) and of the chronic group was 22 of 29(76%). The duration of symptoms didnt influenced significantly the effect of epidural block. 3. Therapeutic effect of epidural block was as follows; complete relief 22 of 56(39%), marked relief 13 of 56(23%), slight relief 9 of 56(16%), no improvement 12 of 56(22%). The over all improvement was resulted in 44 of 56(78%): intermittent epidural block 34 of 44(77%), continuous epidural block 10 of 12(83%). 4. Therapeutic effect of conservative treatment was as follows; complete relief 10 of 36(28%), marked relief 7 of 36(19%), slight relief 4 of 36(11%), no improvement 15 of 36(42%). The over all improvement of symptoms was resulted in 21 of 36(58%). 5. Follow up period of epidural block patients were 3 months in average, and persistent relief of symptom were obtained in 12 out of 20 patients(60%), and recurrence of symptom were in 6 of 20 patients(30%). It is considered that epidural block will shorten the time of recovery from severe pain, avoid hospitalization or long periods of bed rest and allow earlier physiotherapy. In addition it may avoid the need for surgical intervention to produce rapid relief of acute pain and give to patient economic, psychologic benefit.
Acute Pain ; Bed Rest ; Decompression, Surgical ; Follow-Up Studies ; Hospitalization ; Hospitals, General ; Humans ; Joint Diseases ; Korea ; Lidocaine ; Low Back Pain ; Recurrence ; Sciatica ; Spine ; Sprains and Strains ; Triamcinolone

The purpose of this study is to assess the differences of the lumbosacral angles between the lying and standing position among Iumbago patients. Among Iumbago patients the following diseases with known causes were exclused in this study; spondylolisthesis, spondylolysis, pure herniated nucleus pulposus, tuberculous spondylitis, congenltal anomalies, trauma and psychosis etc. Only the following with unknown causes were included; idlopatic lumbago, hernlated nucleus pulposus with bone and joint changes, instability and degenerative osteoarthritis. The following lumbosacral angles were measured and evaluated in 460 lumbago patients; 271 patients in the lying position and 189 patients in the standing position. 1. Ferguson angle 2. Sacrovertebral angle (Mitchell angle) 3. LS horlzontal angle 4. Promonotorium angle 5. Line of weight bearing The results were summarized as follows; 1. Ferguson angle Lying position 38.4 Standing positon 35.3 Sacrovertebral angle 140.7 142 L5 horizontal angle 20.4 17.4 Promontorlum angle 134.4 134.9 2. Ferguson and L5 horizontal angles in lumbago patients were increased in the lying position and decreased in the standing position as compared with the angles in normal individuals (control group). 3. Sacrovertebral angle In lumbago patients was decreased in the lying position. 4. Promonotorium angle in lumbago patients was increased in the lying and standing position. 5. Judging by the Iine of weight bearing, Iumbago patients in the:lying position showed more instability as compared with that in the standing position group.
Deception ; Humans ; Joints ; Low Back Pain ; Osteoarthritis ; Posture ; Psychotic Disorders ; Spondylitis ; Spondylolisthesis ; Spondylolysis ; Statistics as Topic ; Weight-Bearing

10 cases of femoral shaft fractures were treated by cast brace during the past 5 years from May, 1975 to August, 1979 at the Department of Orthopedic Surgery, Korea General and Jae II Hospital, The results were summarized as follows; 1. Of the 10 cases, 8 cases were male and 2 were female, and age distribution was between 14 and 65. 2. 5 cases were treated by cast brace from the start as the primary treatment and the other 5 were by other method initially and followed by cast brace secondarily. 3. One of 5 cases treated by early cast brace developed delayed union and the other one of 5 cases treated by late cast brace secondarily developed non-union, which were treated by open reduction and internal fixation with cancellous bone graft. 4. The cast brace was applied within the first 6 weeks after fracture and average application time was 9. 7 weeks in the cases treated by early cast brace, which was compared with 12.5 weeks in the late cast brace group. 5. Average time of fracture healing was 13.5 weeks in the early cast brace group and 18. 5 weeks in the late cast brace group. 6. In three of 10 cases, counter traction was applied to reduce the limb shortening during bed rest.
Age Distribution ; Bed Rest ; Braces ; Extremities ; Female ; Fracture Healing ; Humans ; Korea ; Male ; Methods ; Orthopedics ; Traction ; Transplants

Malignant fibrous histiocytama is currently defined as a malignant primary bone tumor, and that may contain fibromatoua, xanthomatous, or histiocytic elementa, varing widely in the degree of anaplasia that is present. The principal lesions to be considered in the differential diagnoais of malignant fibrous histiocytoma are osteogenic sarcoma and fibrosarcoma. Malignant fibrous histiocytoma is more sensitive to chemotherapeutic ageata than are osteosarcama and fibrosarcama. In this paper 2 cases sre reported, including the one in which the tumor arose in a midahaft of the femur and the other one arosein the upper metaphyseal portion of the tibla.
Anaplasia ; Femur ; Fibrosarcoma ; Histiocytoma, Malignant Fibrous ; Osteosarcoma

Absence of the thumb, either traumatic or congenical, causes a severe deficiency in hand function; in fact grasp and pinch are almost impossible. Thus when the thumb is partially or totally absent, reconstructive surgery is appealing. Usually the thumb should be reconstructed only when amputation has been at the metacarpophal-angeal joint or at a more proximal level. The procedures for reconstruction of the thumb are well known. They include transposition of a digit, procedures to lengthen the thumb metacarpal and methods of total reconstruction including a pedicle graft to obtain sensibility. Although pollicization (transposition of a finger to replace an absent thumb) endangers the finger, that is worthwhile, especially in complete bilateral absence of the thumb or in bilateral traumatic amputation. The each finger with a part or the whole of the metacarpal, has been transplanted to the stump of the metacarpal of the thumb or the trapezium. When all of the fingers are normal, the index finger is the best choice for thumb replacement because of its circumferential size, phalangeal length, independent motion and proximity to the thenar eminence. Recently this operation has been done with conservation of all nerves, vessels, tendons and muscles. We have had two pollicization operations (transposition of index finger) in the patient of bilateral traumatic amputation of the thumbs. Four months later excellent pinch and grasp with normal sensibility in the reconstructed thumbs were obtained with all remaining digits.
Amputation ; Amputation, Traumatic ; Fingers ; Hand ; Hand Strength ; Humans ; Joints ; Muscles ; Tendons ; Thumb ; Transplants