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

No abstract available.
Pylorus*

Many kinds of lumbar diseases cause lumbar segmental instability and/or limitation of motion. But, there is no reliable method for measuring accurate trunk motion. In order to measure relative trunk motion respect to the pelvis in vitro, external linkage type of spine motion analyzer was developed. Special programs for calculation of the relative angular motion and graphical display were also developed. The developed device was tested to compare spine motion pattern between 15 normal volunteers and 18 lumbar fusion patients in flexion, extension, lateral bending and axial rotation. It was rather simple and reliable method for spine motion analysis. And it was possible to measure not only angular displacement but also angular velocity and angular acceleration. There were more coupling motions in lateral bending and axial rotation than in flexion and extension. Compared to the normal subjects, patients showed different angular motion, especially in the angular acceleration. Optimal trajectory of the trunk motion derived from mathematical model in flexion and extension matched well with measurement for normal subjects.
Acceleration ; Healthy Volunteers ; Humans ; Models, Theoretical ; Pelvis ; Spine*

The mineral content and width of bone can be determined noninvasively by “bone densitometer”, which measures the absorption by bone of a monoenergetic photon beam that originates in a radioactive source(Iodine-125 at 27.3 Kev). The intensity of the beam transmitted by the bone is measured by a scintillation detector. The bone mineral density is obtained from dividing the bone mineral content by bone width. Since Cameron and Sorenson, in 1963, first described the photon absorptiometry, many investigators have studied this method and applied it clinically. In order to determine the bone density of normal koreans, and compare it with that of fracture risk group, we measured the bone density of the distal one third of the nondominant radius in 152 normal persons(55 male, 97 female), and 54 patients(23 male, 31 female) having the risk of spontaneous fracture from the third to seventh decades. This data were also compared with those of normal Caucasians. The results were as follows. 1. The average bone densities(gm/cm2) of normal men from the third to the seventh decades were 0.773 ±0.055, 0.749 ±0.070, 0.770 ±0.060, 0.797 ±0.053, 0.664 ±0.126, respectively and those of normal women were 0.680 ±0.058, 0.680 ±0.036, 0.674 ±0.052, 0.608 ±0.084, 0.523 ±0.093, respectively. 2. The average bone densitied(gm/cm2) of fracture risk men from the third to seventh decades were 0.647 ±0.072, 0.719 ±0.050, 0.729 ±0.085, 0.699 ±0.064, 0.562 ±0.049, respectively and those of fracture risk women were 0.603 ±0.049, 0.061 ±0.021, 0.326 ±0.034, 0.494 ±0.045, 0.430 ±0.035, respectively. 3. There were statistically significantly differences in the bone densities between the normal population group and the fracture risk group. 4. The average bone densities(gm/cm2) of normal koreans were lower than those of normal Caucasians by 0.115 ±0.023 in male, and 0.091 ±0.005 in female. 5. We belive that bone densitometer is an effective tool in early detection and treatment in metabolic bone deseass including osteoporosis.
Absorptiometry, Photon ; Absorption ; Bone Density ; Female ; Fractures, Spontaneous ; Humans ; Male ; Methods ; Miners ; Osteoporosis ; Population Groups ; Radius ; Research Personnel

Limb lengthening is now an accepted form of correcting limb length inequality. It is, however, associated not infrequently with a variety of complications including pheripheral nerve palsies. Such nerve dysfunction is obviously dependent on the velocity and amount of bone lengtening. In this experiment, the author attempted to determine the safe limits of the velocity and amount of bone lengthening by measuring somatosensory evoked potential (SSEP). The tibiae of 96 rabbits, weighing from 1.5 to 1.6kg, were osteotomized; in Group I, the tibiae were lengthened 0.35mm per day once daily;in Group II, 0.7mm per day; in Group III, 1.05mm per day; and in Group IV, 1.4mm per day. Several rabbits were subjected to the study weekly until, six weeks postoperatively in Group I, five weeks postoperatively in Grup II, four weeks postoperatively in Group III, and three weeks postoperatively in Group IV. The following results were obtained: 1. Preliminarily, in order to verify the possible difference between the right and left tibiae, Pl latency and amplitude were measured in 18 rabbits. Paired t-test revealed no statistically significant difference between the two (p>0.05). The amplitude manifested more individual variation than the Pl latency. 2. As lengthening progressed, the Pl latency gradually increased, whereas the amplitude decreased. The higher the percentage of lengthening to the original tibial length, the more marked the changes in both the Pl latency and amplitude. 3. As lengthening progressed, significant changes were observed earlier in the amplitude than in the Pl laterncy. 4. Significant changes in the amplitude were observed when lengthening reached 12.2% of the original tibial length in Group I (0.35mm/day) and 10.5% in Group IV (1.4mm/day). Significant changes in the Pl laterncy were observed when lengthening reached 17.8% of the original length. It is concluded that somatosensory evoked potential is an effective method of detecting early and preventing neurological complications in bone lengthening. Somatosensory evoked potential is a clinically feasible technique. It is expected that the finding of study may serve as a useful guideline for detecting the safe limits of velocity and amount of bone lengthening.
Bone Lengthening ; Evoked Potentials, Somatosensory ; Extremities ; Methods ; Paralysis ; Rabbits ; Socioeconomic Factors ; Tibia

A case of systemic coccidioidomycosis with cutaneous lesions has been found in Dec. 1974, for the first time in Korea, which is proved through cutaneous biopsy .and mycologic culture from skin lesion. The patient was 57-year-old Korean male. Skin lesions on left extremities showed several skin colored or ligbt brownish papules and surrounding circular erythematous scaly patches with slightly elevated borders. The patient was also found to have moderate diabetes mellitus, hypokalemia and resultant neuropathy. Histopathology revealed granulomatous lesions in the upper dermis, and numerous spherules both inside and outside of giant cells. Mycologic culture from grind skin specimen on Sabourauds glulose agar showed snow-white colony and ather typical arthrospores. Ampbotericin B intravenous injection was started and continued for 16 days. During a,dministration of the drug, side effects consisting of chills, fever, headache, chest pain and dyspnea developed. Therefore, further administration of the drug could not be done. During tbe several months following last discharge from the hospital, the patient has been, with no further specific therapy, in relatively good general condition, and developed no new lesions. The old papules have diminished in size, but did not disappear.
Agar ; Biopsy ; Chest Pain ; Chills ; Coccidioidomycosis* ; Dermis ; Diabetes Mellitus ; Dyspnea ; Extremities ; Fever ; Giant Cells ; Headache ; Humans ; Hypokalemia ; Injections, Intravenous ; Korea ; Male ; Middle Aged ; Skin

For young children with scoliosis before growth spurt, suhcutaneous lengthening without fusion was designed by Harrington and modified by Moe and Luque. However, many problems including spontaneous fusion, rod breakage, and hook disloclgement have been ohserved. CotrelDubousset(CD) instrumentation was sometimes used, but it usually resulted in failure due to soft tissue adhesion around the rough surface of ordinary CD rod. We tried to use the smooth CD rod, transvcrse-pedicle clawing on the upper part, and pedicle screw inscrtion on upper and lower part of the curve to reduce the hardware failures. Among 8 patients in whom suhcutaneous lengthening with smooth CD rod was carried out hetween October l992 and Suly 1996. 4 cases perfomed with final spinal fusion were analysed. There were I central core disease, 1 multicore disease and 2 idiopathic scoliosis(infantile and juvenile type). Mean age at the first operation was l0.0(8.8-11.8) years, and the Risser sign was all grade 0 except one with grade 1. Suhcutaneous lengthening was performed every 5 or 6 months Mean lengthening duration was 22(9-39) months and mean age at spinal fusion was 11.7(9.6-13.8) years. Mean Cobb angle decreased from 7ldegrees (55degrees-88degrees) at preoperative stage to 32 (10degrees-59degrees) at the last follow-up. There were 5 complications during 21 operations, and three hardware failures comprised 2 hook dislodgcment and 1 screw pull-out. Crankshaft phenomenon happened in I case who had had a posterior fusion in young age(9.6 years) due to laminar fracture. The suhcutaneous lengthening with smooth CD rod can he another option of treatment for young children with severe scoliosis. prescrving the powth potential of involved vertebrae with few complications.
Animals ; Child* ; Follow-Up Studies ; Hoof and Claw ; Humans ; Myopathy, Central Core ; Scoliosis* ; Spinal Fusion ; Spine ; Tissue Adhesions

A Case of pigmented choroid plexus carcinoma is reported. The patient was a 35-year-old woman who had headache of 3-month duration, followed by ataxia for 1month before admission. Brain CT revealed a well enhanced mass at the left cerebello-pontine angle area as well as hydrocephalus. Subtotal removal of the tumor was performed via suboccipital craniectomy under the impression of meningioma because there was a mass only in the extraventricular area. The tumor was diagnosed by light microscopic examination. Pigmented choroid plexus carcinoma, which has been reported as low grade malignancy, is very rare and located always in the ventricles. In the case the tumor was subtotally removed and ventriculo-peritoneal shunt surgery and radiation therapy underwent later. The patient has been followed-up without recurrence for 1 year postoperatively.
Adult ; Ataxia ; Brain ; Choroid Plexus Neoplasms* ; Choroid Plexus* ; Choroid* ; Female ; Headache ; Humans ; Hydrocephalus ; Meningioma ; Recurrence ; Ventriculoperitoneal Shunt

This paper describes the basic date measurements for total body irradiation with 6 Mv photon beam including compensators designs. The technique uses bilateral opposing field with tissue compensators for the head, neck, lungs, and legs from the hip to toes. In vivo dosimetry was carried out for determining absorbed dose at various regions in 7 patients using diode detectors (MULTIDOSE, Model 9310, MULTIDATA Co., USA). As a results, the dose uniformity of+/-3.5%(generally, within+/-10%) can be achieved with our total body irradiation technique.
Head ; Hip ; Humans ; Leg ; Lung ; Neck ; Toes ; Whole-Body Irradiation*

We present our recent experience of two rare cases of ruptured aneurysm of the azygos anterior cerebral together with a review of the literature. On admission, the patients were in clinical Grade 4 of Hunt and Hess classification. Cerebral angiograms showed a giant saccular aneurysm(25x10x10mm in size) on the distal bifurcation portion of the azygos anterior cerebral artery in one patient, and a medium sized saccular aneurysm(7x9mm in size) on the proximal portion of azygos anterior cerebral artery in the other patient. Both the patients underwent a successful, direct clipping of the aneurysmal neck through an interhemispheric and left parietal approach, respectively. One month later after the operations, one patient showed mutism and spastic paraparesis and the other remained in a drowsy mental status with left side hemiparesis. The pre-operative neurologic status and further neurologic deficits attributed to cerebral vasospasm are thought to be the most important prognostic factors in deciding the post-operative outcome in these patients.
Aneurysm ; Aneurysm, Ruptured* ; Anterior Cerebral Artery* ; Classification ; Humans ; Mutism ; Neck ; Neurologic Manifestations ; Paraparesis, Spastic ; Paresis ; Rabeprazole ; Vasospasm, Intracranial