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

OBJECTIVE: The Positive and Negative Affect Schedule (PANAS) was developed as a measure of positive affect (PA) and negative affect (NA). The aim here is to examine the factor structure and concurrent validity of the Korean version of the Positive and Negative Affect Schedule (K-PANAS) in a clinical sample in Korea. METHODS: K-PANAS was administered to a clinical sample in Korea. Internal consistency, test-retest reliability, and confirmatory factor analysis (CFA) were undertaken to examine the factorial structure and reliability of the K-PANAS. RESULTS: The reliability of K-PANAS is satisfactory. CFA showed that several of the models commonly used in Western populations provided an insufficient fit. The modified model provided a more adequate fit to the data. CONCLUSION: The authors demonstrate that the K-PANAS has adequate psychometric properties, and that findings obtained in the West using PANAS were partially replicated.
Appointments and Schedules ; Korea ; Psychometrics

Although laparoscopic surgery has been adopted for the treatment of peritonitis since the time laparoscopy was introduced for emergency operations, a laparoscopic Hartmann's procedure has not been described. We report on a Hartmann's procedure and its reversal for the treatment of peritonitis from colonic perforation by laparoscopic surgery. A 43-year-old female was diagnosed with fecal peritonitis due to a colonic perforation in the rectosigmoid colon. Five trocars were used and a Lapdisc(R) (Hakko medical, Japan) was placed at the previous Phannelstiel incision site. One hundred sixty minutes were needed for the laparoscopic Hartmann's procedure following peritoneal lavage. An oral diet was started at the postoperative 6th day and the patient was discharged on the 20th day with complete recovery. Hartmann's reversal was laparoscopically performed 3 months later. A Lapdisc was placed at the colostomy site following colostomy take-down. Four trocars were inserted at the same area as the first operation. The operative time was 180 minutes. There were no postoperative complications.
Adult ; Colon ; Colostomy ; Diet ; Emergencies ; Female ; Humans ; Laparoscopy ; Operative Time ; Peritoneal Lavage ; Peritonitis ; Postoperative Complications ; Surgical Instruments

Secondary mania associated with focal brain lesion has only rarely been reported. The authors report a case of secondary mania artier postoperative radiation therapy for primary brain tumor. Localized necrotic lesion was found in the right basotemporal lobe involving hippocampus, which might be the result of late delayed damage of the postoperative radiation therapy. This case suggests that secondary mania is closely related to focal, right basotemporal lesions. Knowing the relationship between the location of brain lesions and manic syndrome may be helpful to us In understanding the neuroanatomical and neurobiological mechanism of primary.
Bipolar Disorder* ; Brain ; Brain Neoplasms* ; Hippocampus

Cytogenetic analysis of 4 cases of meningiomas from 3 male and 1 female patients is reported. One of male patients suffered from neurofibromatosis type 2. Histologically, the meningiomas were meningotheliomatous (1), transitional (2), and psammomatous (1). Chromosomal abnormalities were found in all cases with a karyotype 45,XY,-22, 45,XY,-16, 45,XX,-2, and 45,XY,t (15p;22q), respectively. Monosomy of chromosome 22 was detected only in the patient with neurofibromatosis type 2. These cytogenetic analysis demonstrates that variable clonal karyotype aberrations exist in meningiomas.
Adolescent ; Adult ; *Chromosome Aberrations ; Female ; Humans ; Male ; Meningeal Neoplasms/*genetics ; Meningioma/*genetics ; Neurofibromatosis 2/genetics

NAG activity has been measured in the urine of hypertension, diabetes mellitus and renal disease subjects. Results have shown significantly higher levels of Urinary NAG in hypertension, diaetes and renal disease subjects compared to normal control Subjects group(P<0.001). Urinary NAG measurement is simple and accurate and it provides a valuable information in the early detection of renal dysfunction in hypertension and diabetes subjects and also in the follow up of these patients.
Acetylglucosaminidase* ; Diabetes Mellitus* ; Follow-Up Studies ; Humans ; Hypertension*

OBJECTIVES: Both electroconvulsive shock(ECS) and kainic acid-induced seizures activate mitogenactivated protein kinases(MAPKs)in rat hippocampus. They can also induce the expression of MAPK phosphatase-1(MKP-1)in rat hippocampus. MKP-1 is known as a specific MAPK deactivator. This study aimed to elucidate the role of MKP-1 in the deactivation of MAPKs in rat hippocampus. METHODS: In order to induce MKP-1 in the hippocampus, ECS was given to the rats. At the time points when MKP-1 was sufficiently induced, the second ECS was given to them and the subsequent phosphorylation or activation of MAPKs were measured in the hippocampus. A second group of rats were injected with kainic acid and the relationship between MKP-1 expression and MAPK phosphorylation was examined in their hippocampi. RESULTS: The expression of MKP-1 did not influence the phosphorylation or activation of MAPKs following ECS in rat hippocampus. Kainic acid-induced expression of MKP-1 did not significantly reduce the phosphorylation of MAPKs. CONCLUSION: MKP-1 did not play a significant role in the deactivation of MAPKs which were activated by ECS or kainic acid in rat hippocampus.
Animals ; Electroshock ; Hippocampus* ; Kainic Acid ; Phosphorylation ; Rats* ; Seizures

A 64-year-old man was admitted for gross hematuria. Preoperative study revealed right renal cell carcinoma with inferior vena cava(IVC) tumor thrombus. Right radical nephrectomy was performed, and deep hypothermic circulatory arrest(DHCA) with retrograde cerebral perfusion(RCP) was used for extraction of tumor thrombus in the IVC. The thrombus originated from the right kidney, which extended the orifice of the gonadal vein in the left renal vein laterally, the hepatic vein superiorly, and 3 cm below the right renal vein inferiorly. The thrombus was removed completely without caval wall injury under DHCA with RCP, and the postoperative course was uneventful. He received immunotherapy with interferon, and followed up without any surgical problem.
Carcinoma, Renal Cell* ; Circulatory Arrest, Deep Hypothermia Induced* ; Gonads ; Hematuria ; Hepatic Veins ; Humans ; Immunotherapy ; Interferons ; Kidney ; Middle Aged ; Nephrectomy ; Renal Veins ; Thrombosis ; Veins ; Vena Cava, Inferior

Stromal tumors of the gastrointestinal tract, especially of the esophagus, are rare. We had a case of malignant gastrointestinal stromal tumor(GIST) of the esophagus. A 46 years old woman was admitted for abnormal mass shadow in the chest radiograph. The mass was originated from the lower thoracic esophagus, and compressed the right lower pulmonary vein and the inferior vena cava. We removed the tumor externally without injuring of the esophageal mucosa via right posterolateral thoracotomy. The tumor was positive for CD 34 and CD 117, and diagnosed malignant GIST of the esophagus.
Esophagus* ; Female ; Gastrointestinal Stromal Tumors* ; Gastrointestinal Tract ; Humans ; Middle Aged ; Mucous Membrane ; Pulmonary Veins ; Radiography, Thoracic ; Thoracotomy ; Vena Cava, Inferior

OBJECTIVE: This is a prospective clinical study on survivors of the collapse accident of a major department store building occurred in Seoul in lune 1995 to investigate the incidence of acute PTSD, to identify risk factors affecting the severity of PTSD symptoms, and to evaluate the pattern of symptom changes over time. METHOD: Thirty-two victims were interviewed with modified Korean version of the PTSD-I(DSM-III-R) to determine the severity of symptoms and diagnosis of PTSD. Degrees of anxiety and depression were measured with Hamilton's Anxiety Scale(HAS) and Depression Scales(HDS) respectively. Subjects were assessed in series at one month(time point 1) and three month(time point 2) from the time the accident occurred. RESULTS: The incidence of PTSD in the subjects over the 3 months was 41% using by full criteria and 48% by partial criteria. Regression analysis at the time point 1, the HDS score, which was partially related with death of accompanied persons(friends/relatives/colleagues), contributed significantly to the PTSD-I variances. At the time point 2, loss of consciousness contributed significantly to the variance of the PTSD-1, HDS and HAS scores. Between tire point 1 and time point 2, the overall severity of PTSD symptoms far all the subjects has remained almost unchanged. However, re-experience and hyperarousal symptoms were improved in comparison with avoidance symptoms, although not statistically significant. CONCLUSIONS: This study result show a high risk of developing PTSD and partial PTSD among the building collapse victims. The death of accompanied person, severity of depression and loss of consciousness are all regarded as major factors affecting the severity of PTSD. Of PTSD symptom clusters, avoidance symptoms are likely to be less changeable than reexperience and hyperarousal symptoms over time.
Anxiety ; Depression ; Diagnosis ; Disasters ; Humans ; Incidence* ; Prospective Studies ; Risk Factors ; Seoul* ; Stress Disorders, Post-Traumatic* ; Structure Collapse* ; Survivors* ; Unconsciousness