No abstract available.
Hematology* ; Humans ; Thyroid Gland*

Limb salvage operations for osteosarcoma of the extremity usually consist of wide excision and skeletal reconstruction. Most osteosarcoma patients are anemic prior to the surgery as majority of them undergo preoperative neo-adjuvant chemotherapy; thus, it is necessary to treat anemia before and after the surgery since limb salvage operation tends to accompany significant blood loss. Despite the fact that blood transfusion has bad influence on prognosis, complication, and postoperative outcome of cancer patients, it is still considered as a standard management to fix anemia for limb salvage operations. We would like to present a case report in which the authors succeeded in performing limb salvage operations on patients with distal femur osteosarcoma without transfusion.
Anemia ; Blood Transfusion* ; Drug Therapy ; Extremities ; Femur* ; Humans ; Limb Salvage* ; Osteosarcoma* ; Prognosis

PURPOSE: Ataxia Telangiectasia (AT) is a hereditary multi-systemic disease resulting from mutations of AT gene and is characterized by progressive neurodegeneration, cancer, immune system defects, and hypersensitivity to ionizing radiation. AT gene has a homologue sequence of PI3-kinase. The activity and cellular function of PI3-kinase in AT gene remains unclear. This study was undertaken to evaluate the function of AT gene through the effect on cell survival and differentiation by the inhibition of AT gene expression. MATERIALS AND METHODS: NH2-terminal portion of AT gene was isolated from MCF-7 cells by RT-PCR. The isolated DNA fragment was ligated in reverse orientation in pcDNA3. This antisense ATM expression vector was transfected to PC-12 cells by calcium phosphate method, and the transformed cells were selected using G418 and immunohisto- chemistry. To analyze the cell survival and differentiation, cells were cultured in serum free medium supplemented with/without NGF. We performed the immunoprecipitation for the p53 induction of cells after ionizing radiation, and the FACS for the apoptosis of cells after the exposure of wortmanin. RESULTS: PC-12 cells which down-regulated AT gene (like ATM, AT mutated) showed decreased survival and ceased differentiation with NGF. Also, PC-12 (ATM) cells showed increased apoptosis with wortmanin and reduced or delayed p53 induction after ionizingradiation. CONCLUSION: Results obtained from these studies suggest that AT gene regulates survival and differentiation of PC-12 cells through PI3-kinase activity. It seems that apoptosis is induced by the inhibition of AT gene expression.
Apoptosis ; Ataxia Telangiectasia ; Calcium ; Cell Survival ; Chemistry ; DNA ; Gene Expression ; Hypersensitivity ; Immune System ; Immunoprecipitation ; MCF-7 Cells ; Nerve Growth Factor ; Phenotype* ; Phosphatidylinositol 3-Kinases ; Radiation, Ionizing

Glycogen storage diseases(GSD) are inherited disorders affecting glycogen metabolism and type I GSD is due to the absence or deficiency of glucose-6-phosphatase(G6Pase) enzyme in the liver, kidney, and intestinal mucosa. The defect leads to inadequate hepatic conversion of G6P to glucose and thus make affected individuals susceptible to fasting hypoglycemia, and the accumulation of glycogen occurs in the liver and other organs. Type Ia is the most common form of GSD and clinically growth retardation may manifest of GSD itself rather than growth hormone deficiency(GHD), but we experienced a case of type I GSD with GHD in a 14-year-o1d male. The height was 125 cm, compatible with 50 th percentile of height of 8 years of age. He has doll-like face with fat cheek, relatively thin extremities, and metabolic acidosis, hyperuricemia, hypoglycemia, hyperlipidemia. GH stimulation test with clonidine and L-dopa revealed that the patient had decreased GH secretion. After laboratory work up including liver biopsy, he was diagnosed as type I GSD. Hypoglycemia was managed with frequent feeding with high starch diet(uncooked cornstarch). Metabolic acidosis and hyperuricemia were treated with sodium bicarbonate, allopurinol and probenecid. The patient is being followed at out-patient clinic with clinical improvement after of diet therapy and GH administration.
Acidosis ; Allopurinol ; Biopsy ; Cheek ; Clonidine ; Diet Therapy ; Extremities ; Glucose ; Glycogen Storage Disease* ; Glycogen* ; Growth Hormone* ; Humans ; Hyperlipidemias ; Hyperuricemia ; Hypoglycemia ; Intestinal Mucosa ; Kidney ; Levodopa ; Liver ; Male ; Metabolism ; Outpatients ; Probenecid ; Sodium Bicarbonate ; Starch

A case of carcinoma of the parathyroid gland causing hyperparathyroidism was experienced at the department of orthopaedic surgery, C.A.F.G.H. This patient, a 23 years old male, had complained of a left shoulder pain during the past 3months. And weakness of the lower extremities causing walking disturbance, multiple bone pain, and polyuria had occurred sinoe one month before admission. Physical examination revealed a hard palpable mass in the right anterior region of the neck. Radiologic examination revealed subperiosteal bone resorption in phalanges of the hands and feet, generalized osteoporosis and cystic lesions, and pathologic fracture of the ribs, left humerus, and left femur. Laboratory studies demonstrated hypercalcemia, hypophosphatemia, increased alkaline phosphatase and PTH, hypercalciuria, and hyperphophaturia. Sonogram and C-T scan of the neck revealed a mass in the right inferior portion of the thyroid. Rib biopsy revealed the findings of ostitis fibrosa cystica, and parathyroid gland and regional lymph node biopsy revealed a parathyroid carcinoma with regional lymph node metastasis.
Alkaline Phosphatase ; Biopsy ; Bone Resorption ; Femur ; Foot ; Fractures, Spontaneous ; Hand ; Humans ; Humerus ; Hypercalcemia ; Hypercalciuria ; Hyperparathyroidism ; Hypophosphatemia ; Lower Extremity ; Lymph Nodes ; Male ; Neck ; Neoplasm Metastasis ; Osteoporosis ; Parathyroid Glands ; Parathyroid Neoplasms ; Physical Examination ; Polyuria ; Ribs ; Shoulder Pain ; Thyroid Gland ; Walking

The suprascapular nerve entrapment syndrome is a rare neuropathy. Clein described the frist case report of this syndrome and operative procedure in 1975. The cardinal sign is pain in and around the shoulder, weakness of abduction and external rotation and atrophy of the supraspinatus and infraspinatus muscles, This syndrome can be diagnosed by clinical symptoms, physical examination, special roentgenogram and electromyogram. We experienced a case of the suprascapular nerve entrapment syndrome. We treated it by resection of the superior transverse ligament of suapula, and obtained good result.
Atrophy ; Ligaments ; Muscles ; Nerve Compression Syndromes ; Physical Examination ; Shoulder ; Surgical Procedures, Operative

No abstract available.
Blood Cell Count* ; Blood Cells* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Prognosis* ; Remission Induction*

No abstract available.
Humans ; Kidney Transplantation* ; Tissue Donors*

The Tolosa-Hunt syndrome, first described by Hunt, et al., as painful ophthalmoplegia in 1961 is characterized by recurrent unilateral retro-orbital pain and dramatic response of steroid therapy. We have recently experienced one case of this syndrome. A 41 year old man was admitted to our hospital because of diplopia, ptosis of the right upperlid and right orbital pain which developed 2 months prior to admission. On examination, right EOM was markedly limited to all direction and sensation was decreased in the 1st and 2nd trigeminal division on the right. Simple skull films and carotid angiogram showed no abnormality. In orbital venography, 3rd portion of the right ophthalmic vein was not visualized. Steroid was administered and improvement of EOM was noted in the next day of therapy. On discharge, previous symptoms disappeared completely.
Adult ; Diplopia ; Humans ; Ophthalmoplegia ; Orbit ; Phlebography ; Sensation ; Skull ; Tolosa-Hunt Syndrome* ; Veins

The Tolosa-Hunt syndrome, first described by Hunt, et al., as painful ophthalmoplegia in 1961 is characterized by recurrent unilateral retro-orbital pain and dramatic response of steroid therapy. We have recently experienced one case of this syndrome. A 41 year old man was admitted to our hospital because of diplopia, ptosis of the right upperlid and right orbital pain which developed 2 months prior to admission. On examination, right EOM was markedly limited to all direction and sensation was decreased in the 1st and 2nd trigeminal division on the right. Simple skull films and carotid angiogram showed no abnormality. In orbital venography, 3rd portion of the right ophthalmic vein was not visualized. Steroid was administered and improvement of EOM was noted in the next day of therapy. On discharge, previous symptoms disappeared completely.
Adult ; Diplopia ; Humans ; Ophthalmoplegia ; Orbit ; Phlebography ; Sensation ; Skull ; Tolosa-Hunt Syndrome* ; Veins