No abstract available.
Asthma* ; Child* ; Humans

Lowe et al. have described a disease characterized by organic aciduria, decreased renal ammonia production, hydrophthalmos, and mental retardation, this disease is now called as Lowe's syndrome or oculo-cerebro-renal syndrome which manifests growth plates, metabolic acidosis, and characteristic eye changes, including bilateral cataract, glaucoma and nystagmus. It is a congenital hereditary affectation. Most of the patients with this syndrome are male sex. We report a new case of Lowe's syndrome in a 5-year old girl.
Acidosis ; Ammonia ; Cataract ; Child ; Female ; Glaucoma ; Growth Plate ; Humans ; Hydrophthalmos ; Intellectual Disability ; Male

PURPOSE: The intermediate filament proteins, desmin and vimentin, are specific components of the cytoskeleton of striated muscle fibers and of mononuclear cells of mesenchymal origin including myoblasts, respectively. Desmin has also been found in presumptive myoblasts of mammals. The aim of this experiment was attempted to observe the phenotypic changes of intermediate filaments in skeletal muscle fibers during early stages of sciatic nerve crushing injury. MATERIALS AND METHODS: The sciatic nerves of rats were surgically crushed by hemostat and serial cryosections of soleus and extensor digitorum longus(EDL) muscles were prepared at 2, 4, 6, 8, 10, 15, 20 and 27 days after nerve injury. Serial cryosections were immunolabelled with desmin, vimentin and laminin and were histochemically reacted with NADH-TR. RESULTS: 1) Firstly, desmin positive fibers were appeared in fast-twitch type C fibers of both muscles at 6 days after nerve crushing, but were not reacted for vimentin. 2) Co-expressions of desmin and vimentin were firstly detected in fast-twitch type A fibers of EDL muscles at 8 days after nerve injury. In soleus muscles, co-expressions of desmin and vimentin were firstly seen in slow-twitch type B fibers at 10 days after nerve injury. Many atrophic fibers, that contained several central nuclei like myotubes and co-expressed desmin and vimentin, were appeared in EDL muscles at 10 days after nerve injury. Although whole regions of fibers were regenerated in EDL muscles, only peripheral regions of fibers were regenerated in soleus muscles at 15 days after nerve injury. Many atrophic fibers, co-expressed of desmin and vimentin, were appeared in EDL muscles at 20 days after nerve injury. These whole fibers represented various degrees of regenerating stages. Most of mature fibers containing several central nuclei, only expressed vimentin slightly, were seen in soleus muscles at 20 days after nerve injury. Most fibers of both muscles were matured at 27 days after nerve injury, but some fibers in EDL muscles were still in processing of degeneration and regeneration. No expressions of desmin and vimentin indicated that muscle fibers were almostly matured in soleus muscles at 27 days after nerve injury. 3) Targetoid or target fibers which informed reinnervation, were appeared firstly in soleus muscles at 20 days and were seen in both muscles at 27 days after nerve injury. All targetoid and target fibers were type B fibers. CONCLUSION: Desmin was revealed in processes of degeneration and regeneration and vimentin was appealed in regeneration process. At the same time, positive immunoreactivity of desmin and vimentin showed specific differences in degree of degeneration and regeneration according to different muscles and muscle fibers.
Animals ; Cytoskeleton ; Desmin ; Intermediate Filament Proteins* ; Intermediate Filaments* ; Laminin ; Leg* ; Mammals ; Muscle Fibers, Skeletal ; Muscle, Striated ; Muscles* ; Myoblasts ; Nerve Crush ; Nerve Fibers, Myelinated ; Nerve Fibers, Unmyelinated ; Rats* ; Regeneration* ; Sciatic Nerve* ; Vimentin

PURPOSE: The intermediate filament proteins, desmin and vimentin, are specific components of the cytoskeleton of striated muscle fibers and of mononuclear cells of mesenchymal origin including myoblasts, respectively. Desmin has also been found in presumptive myoblasts of mammals. The aim of this experiment was attempted to observe the phenotypic changes of intermediate filaments in skeletal muscle fibers during early stages of sciatic nerve crushing injury. MATERIALS AND METHODS: The sciatic nerves of rats were surgically crushed by hemostat and serial cryosections of soleus and extensor digitorum longus(EDL) muscles were prepared at 2, 4, 6, 8, 10, 15, 20 and 27 days after nerve injury. Serial cryosections were immunolabelled with desmin, vimentin and laminin and were histochemically reacted with NADH-TR. RESULTS: 1) Firstly, desmin positive fibers were appeared in fast-twitch type C fibers of both muscles at 6 days after nerve crushing, but were not reacted for vimentin. 2) Co-expressions of desmin and vimentin were firstly detected in fast-twitch type A fibers of EDL muscles at 8 days after nerve injury. In soleus muscles, co-expressions of desmin and vimentin were firstly seen in slow-twitch type B fibers at 10 days after nerve injury. Many atrophic fibers, that contained several central nuclei like myotubes and co-expressed desmin and vimentin, were appeared in EDL muscles at 10 days after nerve injury. Although whole regions of fibers were regenerated in EDL muscles, only peripheral regions of fibers were regenerated in soleus muscles at 15 days after nerve injury. Many atrophic fibers, co-expressed of desmin and vimentin, were appeared in EDL muscles at 20 days after nerve injury. These whole fibers represented various degrees of regenerating stages. Most of mature fibers containing several central nuclei, only expressed vimentin slightly, were seen in soleus muscles at 20 days after nerve injury. Most fibers of both muscles were matured at 27 days after nerve injury, but some fibers in EDL muscles were still in processing of degeneration and regeneration. No expressions of desmin and vimentin indicated that muscle fibers were almostly matured in soleus muscles at 27 days after nerve injury. 3) Targetoid or target fibers which informed reinnervation, were appeared firstly in soleus muscles at 20 days and were seen in both muscles at 27 days after nerve injury. All targetoid and target fibers were type B fibers. CONCLUSION: Desmin was revealed in processes of degeneration and regeneration and vimentin was appealed in regeneration process. At the same time, positive immunoreactivity of desmin and vimentin showed specific differences in degree of degeneration and regeneration according to different muscles and muscle fibers.
Animals ; Cytoskeleton ; Desmin ; Intermediate Filament Proteins* ; Intermediate Filaments* ; Laminin ; Leg* ; Mammals ; Muscle Fibers, Skeletal ; Muscle, Striated ; Muscles* ; Myoblasts ; Nerve Crush ; Nerve Fibers, Myelinated ; Nerve Fibers, Unmyelinated ; Rats* ; Regeneration* ; Sciatic Nerve* ; Vimentin

Synovial sarcoma is a very rare malignant tumor which arising from the synovial lining connective tissue, tendon sheaths and bursae. However, there are a few case reports of this tumor arising from the bone. We have experienced 3 cases of the tumor from the bone, i. e. the head of the left fibula, the sacrum and the right ileum, and the distal end of the left femur. Microscopically they showed typical slit like spaces, lined by epitheloid cells or cuboidal cells and one could easily find out spindle, polygonal, or giant cells. The proximal third of the left fibula was excised, and the other case arising from the sacrum & right ilium was treated with radiation therapy. Another case refused hip disarticulation, The effectiveness of the treatment is uncertain.
Connective Tissue ; Disarticulation ; Femur ; Fibula ; Giant Cells ; Head ; Hip ; Ileum ; Ilium ; Sacrum ; Sarcoma, Synovial ; Tendons

Reduction in the extremity length of more than three to four centimeters has significant effect on both gait disturbance and aesthetic change. The method of Anderson has been one of the most successful procedures of surgical leg elongation but requires long period of bed rest and immobilization. Also. the lengthening occurs the tibia, although shortening occurs twice as frequently in the femur as in the tibia. Wagner in 1971 reported his experience with the technique that allows correction in both the femur and tibia yet does not require prolonged immobilizaton. This involves a two stage procedure with rigid external fixation and lengthening of 1.5mm per day. The second stage is for osteosynthesis and may be suppleniented by bone graft. We began using the Wagner's technique of leg lengthening at Chonju Presbyterian Medical Center about 18 months age. Two patients are presented with 7 and 9 month follow-ups after femoral lengthening of 5 cm each. Both femurs united well and the leg length discrepancy was corrected. These cases indicate that this method is a good addition to the armamentarium of treating leg length discrepancy. A subsequent review of more cases and their follow-up using this tretment program shall be forthcoming.
Bed Rest ; Extremities ; Femur ; Follow-Up Studies ; Gait ; Humans ; Immobilization ; Jeollabuk-do ; Leg ; Methods ; Protestantism ; Tibia ; Transplants

The skeleton is a frequent site of bone tumor metastasis. Radiographic examination Is not sufficiently reliable in early detection since an abnormality is unlikely to be observed until more than 50% of the bone material has been lost. Therefore there was much effort to discover radiographic materials for use in scanning. At the present tirne, 99m Tc-labeled diphosphonate is the best available material for bone scanning. We applied whole body scanning with 99m Tc-to 40 patients who had bone tumors (primary or secondary), infection and other cases. The results obtained are as follow: 1. The scan can detect the extensiveness of the tumor better than plain X-ray in the early stage. 2. In metastatic disease, 60% of all patients were positive in the scan and all of there were negative in X-ray. 3. In cases of cancer, definitive treatment planning can be facilitated by the information obtained from whole body scanning. 4. In inflammatory disease, we can differentiate osteomyelltis from cellulitis or pyogenic arthritis in the early stage. 5. In fibrous dysplasia, it was possible to differentiate monostatic from polyostotic disease. 6. In nonunion of bone, scan was positive but X-ray was eouivocal.
Arthritis ; Cellulitis ; Humans ; Neoplasm Metastasis ; Skeleton ; Whole Body Imaging

The incidence of spinal tuberculosis has been decreased because of the development of anti-tuberculosis drugs, the use of radical surgical procedures and the improvement in the nations general hygiene. The anterior fusion is the most frequently used surgical measure in the treatment of spinal tuberculosis. One-hundred-eighty cases of spinal tuberculosis were treated by surgery during the 5 years from 1976 to 1980 at Presbyterian Medical Center with the following results: 1. The age distribution showed the peak incidence to be in the third decade. 2. The predilection site was the lumbar area and the percentage was 43.3. 3. In 17.2% of the cases, anti-tuberculosis drug was ingested before surgery. 4. Paraplegia was present in 16.1% of spinal tuberculosis. 5. In paraplegia, most of the location of the involved bodies was in the thoracic and the thoracolumbar vertebrae and the percentage was 79.3. 6. In paraplegia, complete recovery was 82.8%, and incomplete recovery was 10.3%. 7. In 90% of the patients, anterior fusion was done and the union rate was 91%. 8. Kyphosis was the most common in late complication.
Age Distribution ; Clinical Study ; Humans ; Hygiene ; Incidence ; Kyphosis ; Paraplegia ; Protestantism ; Spine ; Tuberculosis, Spinal

Metastatic malignant tumors involving the spine cause destruction of vertebral body, kyphosis and neural deficit. Irradiation relieves the pain and decreases the tumor mass, but sometimes the spine is progressively destroyed and becomes unstable. Traeatment of metastatic tumor involving the spine is difficult. Replacement of the vertebral body with bone cement and high dosage of radiation therapy was recommended by Harrington. The advantages are excision of the tumor mass, firm immobilization and enough irradiation not to recur. Two cases of metastatic carcinoma involving cervical spine were treated by anterior decompression, bone cement fixation and irradiation. One case died seventeen days after surgery due to severe pleural effusion. Another case was treated with irradiation (6,000 rads) after surgery and the pain was relieved. The spine is stable one year after surgery. There is no local recurrence.
Decompression ; Immobilization ; Kyphosis ; Pleural Effusion ; Recurrence ; Spine

Authors reviewed 21 cases of unstable intra-articular fractures of the distal radius treated with open reduction and internal fixation to improve anatomical and functional results during March 1990 to May 1993 at Dong-A University Hospital. The following results were obtained; 1. The most common injury type was type VIII(9 cases) according to Frykman's classification, type C,(8 cases) according to AO classification, and open fractures were 4 cases. 2. Twelve cases were treated with open reduction and internal fixation with external fixator and 9 cases were treated with open reduction and internal fixation with cast. 3. According to demerit point rating system, excellent to good results were obtaned in 76.2%. In cases of external fixator application, excellent and good results were 88.3%, compared to 66.7% in cases of cast application. 4. In last follow-up examination, the mean loss of radial length was 0.9mm(10.0%) in cases of cast, but 0.3mm(3.3%) in cases of external fixator, the mean loss of radial inclination was 2.7° (16.7%) and 1.8° (11.0%) respectively, the mean loss of volar tilt was 2.9° (19.6%) and 2.4° (16.4%) respectively, and there were statistical significances in all three parameters between the external fixator group and the cast group(p < 0.05). 5. Open reduction and internal fixation, combined with external fixation was thought to be a better method than open reduction and internal fixation with cast for the treatment of unstable comminuted fractures of the distal radius, because that was more comfortable and convenient to patients and useful to prevent late displacement or collapse of fractures.
Classification ; External Fixators ; Follow-Up Studies ; Fractures, Comminuted ; Fractures, Open ; Humans ; Intra-Articular Fractures ; Methods ; Radius