Glycogen storage disease is a rare metabolic disorder of significant to the anesthesiologist. The term "glycogen storage disease" is applied to a group of congenital and familial disorders characterised by depostion of abnormally large or small quantities of glycogen in the tissues. 13 types of glycogen storage diseases have been described, classified on the basis of enzyme deficiencies. Type l glycogen storage disease (von Gierke's Disease) is the most common of this constellation of syndromes. The basic defect is a deficiency of enzyme, glucose-6-phosphatase. The patient has hepatomegaly, renomegaly, stunted growth, a tend toward severe hypoglycemia and acidoais. The adverse effect of the combined anesthetic and surgical procedure during operation was reflected in a deterioration of the patients's biochemical parameters. A cardiac arrest after tonsillectomy of the patient with Von Gierke's disease was reported and this fact cmphasizes serious anesthetic problems during operation. Anesthetic management of these patients should focus on prevention of hypoglycemia and lactic acidosis. The careful frequent measuring of the acid-base status is highly recommended and is essential prior to and during andy surgical procedure. We report a case of anesthetic management for a patient with Von Gierke's desease ane review anesthetic problems for these patients.
Acidosis, Lactic ; Glucose-6-Phosphatase ; Glycogen ; Glycogen Storage Disease ; Glycogen Storage Disease Type I ; Heart Arrest ; Hepatomegaly ; Humans ; Hypoglycemia ; Tonsillectomy

Malignant germ cell tumors occur in children and young women in reproductive age, of all the germ cell malignancies, only pure dysgerminomas had a high cure rate prior to 1970. This was due to the exquisite radiosensitivity of these tumors. Multiple-agent chemotherapy has dramatically improved the pmgnosis of patients with malignant ovarian germ cell tumors. Clinicopathological analysis was performed on 21 cases of malignant germ cell tumors of the ovary, 9 cases at the department of Obstetrics & Gynecology, Kyung-pook National University Hospital, 4 cases, at the department of Obstetrics and Gynecology, Dong-guk University Kyungju Hospital and 8 cases, at the department of Obstetrics and Gynecology, Dae-gu Fatima Hospital during the period 10 years from July. 19S8 to June. 1997 The results were summarized as follows: 1. The mean age of the patients was 21.8 years old, ranging from 10 to 61 years old. 2. Main initial symptoms were abdominal distension(33.3%), abdominal mass palpation(33.3%), abdominal pain(28.6%), amenorrhea(4.8%) in order. 3. Histologically, the tumors were classified as immature teratoma(57.1%), dysgerminoma(19%), mixed germ cell tumor(9.5%), endodermal sinus tumor(9.5%), embryonal cell carcinoma(4.8%). 4, According to FIGO classification Stage I was the most prevalent(57.1%) at the time of diagnosis while Stage III was forward in 48.6%, Stage II in 9.5%, and no Stage IV, 5. The 5-year survival rate was 80.4% and three patients have died of malignancy and the other 18 patients live without disease from 11 to 102 months, with a median follow-up of 34.2 months. 6. Prognostic factors of malignant germ cell tumors in this study were the clinical stage, the presence of ascites and the residual tumor mass.
Ascites ; Child ; Classification ; Daegu ; Diagnosis ; Drug Therapy ; Dysgerminoma ; Endoderm ; Female ; Follow-Up Studies ; Germ Cells* ; Gyeongsangbuk-do ; Gynecology ; Humans ; Middle Aged ; Neoplasm, Residual ; Neoplasms, Germ Cell and Embryonal* ; Obstetrics ; Ovary* ; Radiation Tolerance ; Survival Rate

The study was designed to analyse the reorganization of the rabbit TMJ meniscus which was anteriorly displaced by surgery. The author compared the anteriorly displaced groups with control group. After surgical opening of the left rabbit TMJ space, cut the posterior attachment of the meniscus, and pushed it under the undercut area of the condyle head. Experimental groups were sacrificed by 1,2,4,8 weeks after surgery. The samples were analysed with light microscope under T-B stain and electron microscope. The results were as follows: 1) The rabbit TMJ meniscus consisted of thick anterior and posterior band running different way, and comparative thin intermediate band runining antero-posteriorly. 2) Round oval shape chondrocyte-like cells were imbeded between the collagen fiber bundles and composed of proteoglycan granules, that showed metachromasia with toluidine blue, around the cell matrix. 3) Type II collagen fiber bundles in experimental group occured degenerative changes in organic patterns at 8 weeks, but those of type I collagen fiber bundles sustained longer. 4) The typical fibrocartilage of the rabbit TMJ meniscus was changed into fibrotic mode in process of time and showed the degenerative changes, which contained hyperplasia, calcification, resorption and hyalinization in the connective tissue. 5) The hyperplastic change of the synovial membrane in 4 week group and transitional change from fibrocyte to chondrocyte in cell type in 8 week group were observed. 6) The diameters of collagen fibers were diminished with the degenerative changes, the shape of the fibers became wavier and more nonorganic in running pattern and fiber bundle spaces widened.
Chondrocytes ; Collagen ; Collagen Type I ; Collagen Type II ; Connective Tissue ; Fibrocartilage ; Head ; Hyalin ; Hyperplasia ; Proteoglycans ; Running ; Synovial Membrane ; Temporomandibular Joint* ; Tolonium Chloride

Vasopressor effects of epinephrine, norepinephrine, ephedrine, phenylephrine, methoxamine and of mephentermine were compared in resrpinized dogs with or without nitrous oxide-oxygen-halothane anesthesia. The results are as follows: (1) Epinephrine and norepinephrine were shown to have the most potent pressor effect in reserpinized and nitrous oxide-oxygen-halothane-anesthetized dogs. Phenylephrine, methoxamine, mephen-termine and ephedrine were less potent in decreasing order. (2) Decrease in mean arterial pressure was less marked in dogs reserpinized and anesthetized with nitrous oxide-oxygen-halothane than in reserpinized but unanesthetized dogs. (3) It revealed that nitrous oxide-oxygen-halothane anesthesia potentiated the vasopressor effects of the sympathomimetic amines studied. Nitrous oxide-oxygen-halothane anesthesia is implicated to exert an sympathomimetic effect.
Anesthesia* ; Animals ; Arterial Pressure ; Dogs* ; Ephedrine ; Epinephrine ; Mephentermine ; Methoxamine ; Norepinephrine ; Phenylephrine ; Sympathomimetics*

To determine the effects of hydrcxyethyl starch upon liver, kidney, serm electrolytes and particularly upon blood coagulation, 500 ml of 6% HES in saline solution was administered intravenously to 15 patients during elective minor surgery. In all cases weighted blood loss was less than 600 ml without replacement. The following laboratory tests were performed immediately before infusion and again 1 hour, 24 hours, 48 hours, and 1 week after the infusion: RBC, WBC, hemoglobin, hematocrit, ESR, platelet, bleeding time, coagulation time, prothrombin time, total protein, albumin, total bilirubin, direct bilirubin, SGOT, SGPT, alkaline phosphatase, BUN, sodium, potassium, and chloride. The results are as follows: 1) No anaphylactic shock or bleeding tendency characteristic of colloids was encountered. 2) No functional disturbance of liver or kidney directly attributable to HES was identified. 3) All laboratory parameters except WBC and ESR decreased after infusion. The decrease was, however, within the normal range and believed secondary to dilational effect of infusion. WBC increased somewhat, but returned to the preinfusion level in a week. 4) Exceptionally erythrocyte sedimentation rate increased notably during the 24 hour period following infusion. As with dextran, this was interpreted not due to direct effect of HES, but due to increased adsorption of fibrinogen, alpha-beta-gamma-glcbulin to red cell surface with changes in electric charge between red cells. 5) Clinical applicability, metabolic aspect, degree of subtitution of hydroxyethyl group and safety with multiple infusion of HES must be carefully determined.
Adsorption ; Alanine Transaminase ; Alkaline Phosphatase ; Anaphylaxis ; Aspartate Aminotransferases ; Bilirubin ; Bleeding Time ; Blood Coagulation ; Blood Platelets ; Blood Sedimentation ; Colloids ; Dextrans ; Electrolytes ; Fibrinogen ; Hematocrit ; Hemorrhage ; Humans ; Kidney ; Liver ; Potassium ; Prothrombin Time ; Reference Values ; Selective Estrogen Receptor Modulators ; Sodium ; Sodium Chloride ; Starch* ; Surgical Procedures, Minor

Glycogen storage diseases are inherited disorders of carbohydrate metabolism caused by a deficiency of enzymes that are involved in degradation of glycogen in the liver. The accumulation of glycogen occurs in the liver and other organs. Type Ia is the most common form and clinically may manifest of glycogen storage disease itself rather than growth hormone deficiency. But in this case the patient showed exceptional extreme growth retardation. Growth hormone stimulation test with clonidine and L-dopa revealed that the patient had growth hormone deficiency. Therefore, we report of a case of glycogen storage disease type Ia with the presence of GH deficiency with review of literature. A 16-year-old male was admitted for the evaluation of hepatomegaly and extreme short stature. The height was 113.5cm, less than third percentile of same age group, and compatible with fiftieth percentile of height of 6 years of age. After laboratory work up including liver biopsy, he was diagnosed with type I glycogen storage disease. The patient was presented with metabolic acidosis, hyperuricemia, and hypoglycemia. Hypoglycemia was managed with frequent feeding with high starch diet and intravenous glucose infusion. Metabolic acidosis was treated with sodium bicarbonate. Secondary hyperuricemia was treated with allopurinol. The patient is being followed at out-patient clinic with clinical improvement after of GH administration.
Acidosis ; Adolescent ; Allopurinol ; Biopsy ; Carbohydrate Metabolism ; Clonidine ; Diet ; Glucose ; Glycogen Storage Disease* ; Glycogen* ; Growth Hormone ; Hepatomegaly ; Humans ; Hyperuricemia ; Hypoglycemia ; Levodopa ; Liver ; Male ; Outpatients ; Sodium Bicarbonate ; Starch

The authors have experienced two cases of general anesthesia for operation of congenital tracheo-esophageal fistula and the results were obtained as follows. 1. The anesthetic agent should be selected as considering of lesser irritating to respiratory system and smaller amount of tracheobronchial secretion. 2. The infant circle absorber system has been preferred to the Ayres T-piece apparatus, because maintaining normal body temperature and removal of carbon dioxide easily. 3. Removal of secretion from trreheobronchial tree, intermittent positive pressure breathing, maintaining normal body temperature and humidity were thought to be necessary for the prevention and treatment of postogerative pneumonia or atelectasis.
Anesthesia, General* ; Body Temperature ; Carbon Dioxide ; Esophageal Atresia* ; Fistula* ; Humans ; Humidity ; Infant ; Intermittent Positive-Pressure Breathing ; Pneumonia ; Pulmonary Atelectasis ; Respiratory System

The authors have experienced five cases of general anesthesia for repair of diaphragmatic hernia in children, and following results were obtained. Whether it was congenital or traumatic in origin, prompt reduction and repair is desirable if accurate diagnosis was made and patients condition was acceptable, for increased postoperative survival rate and prevention of possible complications. When the intermittent positive pressure breathing was needed during preoperative, intraoperative or postoperative period, the greatest concern of anesthetist is that excessive positive ventilation should be avoid. The most important factors in this situation probably are the use of chest drainage, daily Roentgen-ray examination, and better use of postoperative ventilatory assistance, for prevention of postoperative atelectasis and other pulmonary complications.
Anesthesia, General* ; Child* ; Diagnosis ; Drainage ; Hernia, Diaphragmatic* ; Humans ; Intermittent Positive-Pressure Breathing ; Postoperative Period ; Pulmonary Atelectasis ; Survival Rate ; Thorax ; Ventilation

It may be the most important problem to keep the patent airway during a general anesthetic procedure or when emergency patient care is necessary. Endotracheal intubation facilitates to keep the airway. But unfortunately, we are confronted with complications of endotracheal intubation, occasionally. The authors experienced a case of segmental bronchial obstruction with mucus plug and successfully remedied by fiberoptic bronchoscopic aspiration through the endotracheal tube.
Anesthesia, General* ; Emergencies ; Intubation, Intratracheal* ; Mucus* ; Patient Care

PURPOSE: To gain insight on transcriptional repression of Topo II a in HL-60 cells arrested to G2/M and M phase, the levels of Topo IIa mRNA and the binding activity of ATF have been investigated with Northern blot hybridization and DNA mobility shift assay, respectively. MATERIALS AND METHODS: HL-60 cells were grown in RPMI 1640 medium supplemented with 10% heat-mactivated fetal bovine serum and antibiotics in a humidified 5% CO2 at 37C degree. Total RNA was prepared by a modification of the method of Karlinsey et al. Northern blot hybridization was performed by the method of Virca et al. A Xho I-Mlu I fragment of phTOP2 was used as probe for Northern blot analysis of Topo II a mRNA. DNA mobility shift assay was performed by the method of Lim et al. End labeled DNA oligomer (upper strand, 5-TCTCCGCTATGACGCCGAGTGGTG-3) for ATF binding activity was mixed with nuclear extracts in a 20 pl reaction volume containing 60 mM KC1, 12 mM HEPES, pH 7.9, 5 mM MgCl2, 0.2 mM EDTA, 0.2 mM DTT, 12% glycerol, and 2 ug of poly [dI-dC]. RESULTS: HL-60 cells were arrested at G2/M phase and M phase after taxol or nocodazole treatment. The levels of Topo II a mRNA were reduced at 24 hours after exposure with nocodazole or taxol but the unknotting activities were not changed. DNA mobility shift assay using oligonucleotide containing the ATF binding site showed that ATF binding activity was reduced after pretreatment of nododazole or taxol. CONCLUSIONS: These results suggest that the reduction of ATF binding activity may be important to transcriptional repression of Topo II a gene by nocodazole and taxol in HL- 60 cells.
Anti-Bacterial Agents ; Binding Sites ; Blotting, Northern ; Cell Division* ; DNA Topoisomerases, Type I* ; DNA Topoisomerases, Type II* ; DNA* ; Edetic Acid ; Electrophoretic Mobility Shift Assay ; Genes, vif ; Glycerol ; HEPES ; HL-60 Cells ; Humans ; Hydrogen-Ion Concentration ; Magnesium Chloride ; Nocodazole ; Paclitaxel ; Repression, Psychology ; RNA ; RNA, Messenger