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*

Carcinomatous meningitis is a relatively common late complication of systemic cancer but there is difficulty in diagnosis when there is no clinical evidence of primary malignant lesion. We have experienced a patient who entered our hospital with complaints of headache, vomiting and deteriorated consciousness. On neurological examination, both optic fundi showed hemorrhagic papilledema and central type facial palsy was observed on the right side. All routine laboratory and radiological examination revealed no abnormality except suspiciously dilated ventricles on Conray ventriculogram. She had been treated under the impression of pseudotumor cerebri. She went downhill course and expired on the 18 th. Hospital day. Autopsy was performed. Pathological findings were limited to the central nervous system. The brain was diffusely swollen and weighed 1510 gms. The cerebral hemispheres were cloudy throughout with dusky gray appearance. Sulci were tight and gyri became flat. No evidence of herniation was present. Coronal sections disclosed two foci of yellow and granular lesions in the left parieto-occipital cortex just under the menengeal coverings. These lesions were less than 0.5 cm in maximum dimensions. Microscopically almost entire cerebral, cerebellar and spinal cord leptomeninges were infiltrated by neoplastic cells that often formed glandular structures. These tumorous growths in the meninges were continuous with the subjacent tumor masses found in the cortex. No other foci of tumor were seen in the CNS. The ventricular system was mild to moderately dilated with slightly cloudy surface. Microscopically no tumor was seen along the ependyma. Search for the primary site of meningeal carcinomatosis included multiple sections of entire visceral organs with particular emphasis on the lung. There was a focus of metastatic papillary carcinoma in one of the left hilar lymph node. Meticulous gross dissection and multiple sections of the lungs, however, failed to show any tumor.
Autopsy ; Brain ; Carcinoma, Papillary ; Central Nervous System ; Cerebrum ; Consciousness ; Diagnosis ; Ependyma ; Facial Paralysis ; Headache ; Humans ; Lung ; Lymph Nodes ; Meningeal Carcinomatosis* ; Meninges ; Neurologic Examination ; Papilledema ; Pseudotumor Cerebri ; Spinal Cord ; Vomiting

We had experienced a case of congenital lobar emphysema in a 3 months old male infant. Chief symptoms included tachypenea, respiratory difficulty, cyanosis, Chest X-ray or chest CT scan revealed extensive emphysematous changes of the right upper and middle lobes, compression of the right lower lobe and shifted of mediastinum to the left side. This condition was appeared in the absence of infection and foreign body in the bronchus and its failure to respond to conservative treatment. This patient was treated by the right upper and right middle lobes pneumonectomy. A brief review of literature was made.
Bronchi ; Cyanosis ; Emphysema* ; Foreign Bodies ; Humans ; Infant ; Male ; Mediastinum ; Pneumonectomy ; Thorax ; Tomography, X-Ray Computed