Glycogen storage disease(GSD) type lis due to defect of glycose-6-phosphatase at the membrane of the endoplasmic reticulum in liver. Clinical presentations of GSD 1 are massive hepatomegaly without splenomegaly, failure to thrive, bleeding tendency, hypoglycemia, fasting ketosis and hyperlipidemia. The appearance of patient is short and fat with particularly fat cheeks. Mental development is usually normal. It was diagnosed by liver biopsy and cofirmend as GSD type lby enzyme analysis. We have experienced a case of GSD type land reported with brief review of literatures
Biopsy ; Cheek ; Endoplasmic Reticulum ; Failure to Thrive ; Glycogen Storage Disease* ; Glycogen* ; Hemorrhage ; Hepatomegaly ; Humans ; Hyperlipidemias ; Hypoglycemia ; Ketosis ; Liver ; Membranes ; Splenomegaly

PURPOSE: The aim of this study was to determine the advantage of adequate PTGBD in acute complicated cholecystitis patients. METHODS: We performed a retrospective review of a collected database from September 2001 to July 2008. Acute cholecystitis with gangrene or perforation was defined as acute complicated cholecystitis. A PTGBD was performed for these patients immediately after the diagnosis using US or CT and then a tubogram was performed after 5~7 days. After evaluating the gallbladder (GB) and common bile duct (CBD) with a tubogram, we removed the drainage tube and the patients underwent a LC after readmission. RESULTS: Three hundred seventy four of the 893 patients who were diagnosed with acute cholecystitis underwent PTGBD. While 19 (3.2%) of the total acute cholecystitis patients were converted to open cholecystectomy due to severe inflammation, 14 (3.7%) of the acute complicated patients were converted to open cholecystectomy. In 79 patients, the pre-operative tubogram showed the presence of CBD stone and so ERCP was performed. There were no post-operative deaths. CONCLUSION: PTBGD in acute complicated cholecystitis patients allows the early relief of acute cholecystitis symptoms. This allows sufficient evaluation and treatment for CBD during the PTGBD state. Furthermore, this decreases the mortality and morbidity in the high-risk patients due to sufficient evaluation and management of the underlying critical disease, which allows elective cholecystectomy when the patients is in better condition for surgery. Therefore, PTGBD can be useful for acute complicated cholecystitis.
Cholangiopancreatography, Endoscopic Retrograde ; Cholecystectomy ; Cholecystectomy, Laparoscopic ; Cholecystitis ; Cholecystitis, Acute ; Common Bile Duct ; Drainage ; Gallbladder ; Gangrene ; Humans ; Imidazoles ; Inflammation ; Nitro Compounds ; Retrospective Studies

The present study was conducted to analyze the status of food and nutrients intakes of the colorectal cancer patients in the Daegu.Kyungpook area and to find dietary risk factors related to the occurrence of colorectal cancer in this community. The case subjects (123) were selected from the patients recently diagnosed as colorectal cancer at Kyungpook National University Hospital, the control subjects (182) were selected from the patients of the Department of Orthopedic Surgery at the same hospital and from the healthy volunteers who did not have any gastrointestinal diseases. The food consumption survey was done by individual interviews using semi-quantitative food frequency questionnaire and nutrients intakes were analysed by CAN program. The results of the study suggested that dietary factors which are speculated as the risk factors of colorectal cancer in Daegu.Kyungpook area were high consumption of cereals and oils, low consumption of fruits and mushrooms, high consumption of energy and fat, especially animal fat, low consumption of dietary fiber, high percentage of energy intake from cereals and potatoes, high intakes of protein, fat, vitamin A and cholesterol from egg, low intake of calcium from vegetables, and high intake of iron from meats and eggs. These findings might be useful for the nutrition education to prevent colorectal cancer in the community. However it is recommended to conduct more extensive and systematic survey to reconfirm these dietary risk factors under taking into consideration of the dietary characteristics in this region.
Agaricales ; Animals ; Calcium ; Edible Grain ; Cholesterol ; Colorectal Neoplasms* ; Daegu* ; Dietary Fiber ; Education ; Eggs ; Energy Intake ; Fruit ; Gastrointestinal Diseases ; Gyeongsangbuk-do* ; Healthy Volunteers ; Humans ; Iron ; Korea* ; Meat ; Oils ; Orthopedics ; Ovum ; Risk Factors ; Solanum tuberosum ; Surveys and Questionnaires ; Vegetables ; Vitamin A

The purposes of this study were to investigate the characteristics of lifestyle and dietary habits of the colorectal cancer patients in Daegu and Kyungpook areas and to collect the data useful for nutrition education for the prevention of colorectal cancer in this community. The case subjects of the study were 123 patients diagnosed recently as colorectal cancer at Kyungpook National University Hospital. The control subjects were 182 persons who did not have any gastrointestinal diseases, including the patients from the department of orthopedic surgery and healthy volunteers. The survey covering general characteristics, life style, dietary habits, eating behaviors, and food intake frequency was administered by individual interviews using questionnaires. The results of the study suggest that high BMI, daily life stress, pessimistic personality, lack of physical activities, and familial cancer history might be the possible risk factors for the incidence of colorectal cancer. Dietary factors suspected as risk factors for colorectal cancer in the present study included strong preferences to meats, salty and fatty taste foods, low intake of water, alcohol drinking, smoking, coffee intake and irregular eating habits. A high consumption of seaweeds, green-yellow vegetables, light-colored vegetables, and green tea was suggested as a preventive factor for colorectal cancer. It is recommended to conduct more extensive and systematic surveys in the near future to reconfirm the risk factors of colorectal cancer in consideration of the characteristic food culture in this community. The results of the present study may be applied to nutritional education for the prevention of colorectal cancer for the local residents.
Alcohol Drinking ; Coffee ; Colorectal Neoplasms* ; Daegu ; Eating* ; Education ; Feeding Behavior* ; Food Habits ; Gastrointestinal Diseases ; Gyeongsangbuk-do ; Healthy Volunteers ; Humans ; Incidence ; Life Style* ; Meat ; Motor Activity ; Orthopedics ; Risk Factors* ; Smoke ; Smoking ; Stress, Psychological ; Surveys and Questionnaires ; Tea ; Vegetables ; Water

OBJECTIVE: The purpose of this study was to evaluate the effects of localized brain cooling on blood-brain barrier (BBB) permeability following transient middle cerebral artery occlusion (tMCAO) in rats, by using dynamic contrast-enhanced (DCE)-MRI. MATERIALS AND METHODS: Thirty rats were divided into 3 groups of 10 rats each: control group, localized cold-saline (20℃) infusion group, and localized warm-saline (37℃) infusion group. The left middle cerebral artery (MCA) was occluded for 1 hour in anesthetized rats, followed by 3 hours of reperfusion. In the localized saline infusion group, 6 mL of cold or warm saline was infused through the hollow filament for 10 minutes after MCA occlusion. DCE-MRI investigations were performed after 3 hours and 24 hours of reperfusion. Pharmacokinetic parameters of the extended Tofts-Kety model were calculated for each DCE-MRI. In addition, rotarod testing was performed before tMCAO, and on days 1-9 after tMCAO. Myeloperoxidase (MPO) immunohisto-chemistry was performed to identify infiltrating neutrophils associated with the inflammatory response in the rat brain. RESULTS: Permeability parameters showed no statistical significance between cold and warm saline infusion groups after 3-hour reperfusion 0.09 ± 0.01 min-1 vs. 0.07 ± 0.02 min-1, p = 0.661 for K(trans); 0.30 ± 0.05 min-1 vs. 0.37 ± 0.11 min-1, p = 0.394 for kep, respectively. Behavioral testing revealed no significant difference among the three groups. However, the percentage of MPO-positive cells in the cold-saline group was significantly lower than those in the control and warm-saline groups (p < 0.05). CONCLUSION: Localized brain cooling (20℃) does not confer a benefit to inhibit the increase in BBB permeability that follows transient cerebral ischemia and reperfusion in an animal model, as compared with localized warm-saline (37℃) infusion group.
Animals ; Behavior Rating Scale ; Blood-Brain Barrier* ; Brain* ; Infarction, Middle Cerebral Artery* ; Ischemia ; Ischemic Attack, Transient ; Magnetic Resonance Imaging* ; Middle Cerebral Artery* ; Models, Animal ; Neutrophils ; Permeability* ; Peroxidase ; Rats* ; Reperfusion ; Rotarod Performance Test

Nesidioblastosis is a rare disorder, and it usually considered as a cause of neonatal hyperinsulinemic hypoglycemia. A 35 year-old-woman with hyperinsulinemic hypoglycemia was admitted in an unconscious condition. Abdominal CT, pancreas MRI and celiac angiography with an intra-arterial calcium stimulation test revealed a suspicious insulin-producing tumorous lesion in the head of pancreas. The patient underwent enucleation of the pancreas head tumor under the initial diagnosis of insulinoma. However, the tumor was confirmed histologically as nesidioblastosis that showed ductoendocrine proliferations and numerous small endocrine cell groups. Nesidioblastosis is classified into a focal type and a diffuse type, which are characterized by different clinical outcomes. The patient in our case showed a normal blood glucose level after operation, which is often the case for the focal type. Herein, we report this very rare case of adult nesiodioblastosis that was successfully treated by surgical resection.
Adult ; Angiography ; Blood Glucose ; Calcium ; Endocrine Cells ; Head ; Humans ; Hyperinsulinism ; Hypoglycemia ; Insulinoma ; Nesidioblastosis ; Pancreas ; Unconscious (Psychology)

PURPOSE: Current pediatric cancer research requires an organized pediatric tumor tissue bank with standardized guidelines for preparation and storage of human tumor tissue samples, white cells, serum, genomic DNA, RNA, cDNA and proteins.. Our institution established and managed pediatric tumor tissue bank for the last one year, and we want to present an overview of our experiences and guidelines. METHODS: From leukemia patients, peripheral blood and bone marrow aspirates were collected at initial diagnosis. Leukemic cells were prepared by Ficoll density-gradient centrifugation and stored at 196oC liquid nitrogen. For solid tumors, tissue cultures were performed as soon as possible after surgical excision or needle biopsy. Serum free media and primary cultured cells were collected and stored at 20degrees C and at 196degrees C, respectively. Genomic DNA, RNA and cDNA were isolated from leukemic cells and cultured solid tumor cells, and stored at 20degrees C. We also isolated genomic DNA from white blood cells of solid tumor patients and stored at 20degrees C. Finally we collected serum samples from all pediatric cancer patients at diagnosis and stored at 20degrees C. RESULTS: Among the 41 cases of leukemia and 100 cases of solid tumor patients who were diagnosed at department of pediatrics, Samsung Medical Center, from August 2000 to July 2001, 26 cases (63%) of leukemia and 59 cases (59%) of solid tumor patients were registered to Pediatric Tumor Tissue Bank. Primary cell cultures were performed in 21 cases of solid tumors and were successful in 19 cases (90%). The isolated genomic DNA, RNA and cDNA were all in high quality confirmed by electrophoresis in agarose gel. CONCLUSION: The problem of tissue sample size obtained by needle biopsy could be overcome by primary cell cultures. For the effective management of pediatric tumor tissue bank, fresh tissue collection with active cooperation of surgeons, organized personnel structure, and multidisciplinary standardized guidelines are necessary.
Biopsy, Needle ; Bone Marrow ; Cell Culture Techniques ; Cells, Cultured ; Centrifugation ; Culture Media, Serum-Free ; Diagnosis ; DNA ; DNA, Complementary ; Electrophoresis ; Ficoll ; Humans ; Leukemia ; Leukocytes ; Nitrogen ; Pediatrics ; Primary Cell Culture ; RNA ; Sample Size ; Sepharose ; Tissue Banks*

OBJECTIVE: To retrospectively evaluate the features of undiagnosed breast cancers on prior screening breast magnetic resonance (MR) images in patients who were subsequently diagnosed with breast cancer, as well as the potential utility of MR-computer-aided evaluation (CAE). MATERIALS AND METHODS: Between March 2004 and May 2013, of the 72 consecutive pairs of prior negative MR images and subsequent MR images with diagnosed cancers (median interval, 32.8 months; range, 5.4-104.6 months), 36 (50%) had visible findings (mean size, 1.0 cm; range, 0.3-5.2 cm). The visible findings were divided into either actionable or underthreshold groups by the blinded review by 5 radiologists. MR imaging features, reasons for missed cancer, and MR-CAE features according to actionability were evaluated. RESULTS: Of the 36 visible findings on prior MR images, 33.3% (12 of 36) of the lesions were determined to be actionable and 66.7% (24 of 36) were underthreshold; 85.7% (6 of 7) of masses and 31.6% (6 of 19) of non-mass enhancements were classified as actionable lesions. Mimicking physiologic enhancements (27.8%, 10 of 36) and small lesion size (27.8%, 10 of 36) were the most common reasons for missed cancer. Actionable findings tended to show more washout or plateau kinetic patterns on MR-CAE than underthreshold findings, as the 100% of actionable findings and 46.7% of underthreshold findings showed washout or plateau (p = 0.008). CONCLUSION: MR-CAE has the potential for reducing the number of undiagnosed breast cancers on screening breast MR images, the majority of which are caused by mimicking physiologic enhancements or small lesion size.
Adult ; Aged ; Breast/*pathology ; Breast Neoplasms/*diagnosis/pathology/radiography ; Diagnosis, Computer-Assisted/*methods ; False Negative Reactions ; Female ; Humans ; Magnetic Resonance Imaging/*methods ; Mammography/*methods ; Middle Aged ; Retrospective Studies

A 57-year-old man with severe abdominal pain was admitted to our hospital. Chest PA and simple abdominal X-ray revealed no specific findings, but the abdominal-pelvis CT scan showed a 5cm sized multiloculated cystic tumor originating from the pancreatic head and a 3.2cm sized hepatic lesion that was suspected to be a metastic lesion. A radical operation was not able to be performed because of peritoneal metastasis and gastrocolonal infiltration. Only open lymph node biopsy was done and it revealed metastatic small cell carcinoma. Long-acting octreotide and gemcitabine was administered to the patient, but there was no therapeutic response. The tumor grew very rapidly to 26cm in size and the patient died 2 months later from his first hospital day. Necropsy was performed, and the pathologic finding of the resected mass was confirmed to be small cell carcinoma, the same as the result of the previous lymph node biopsy.
Abdominal Pain ; Biopsy ; Carcinoma, Small Cell ; Deoxycytidine ; Head ; Humans ; Lymph Nodes ; Middle Aged ; Neoplasm Metastasis ; Octreotide ; Pancreas ; Pancreatic Neoplasms ; Thorax

OBJECTIVE: To evaluate the benefits of maternal corticosteroid therapy in infants delivered before 33 weeks of gestation after premature rupture of membranes (PROM). METHODS: This retrospective study included the pregnant women complicated by preterm delivery within 32 weeks of gestation after PROM at the Asan Medical Center between 1997 to 1999. Patients were divided into 2 groups according to the gestational age at delivery, i.e., one group who delivered within 28 weeks of gestation and the other group who delivered between 29 and 32 weeks of gestation. Within each group, we evaluated the effect of maternal dexamethasone therapy on the perinatal and neonatal outcomes based on the medical records. Data were analyzed with pearson's chi-square test, Fisher's exact test, and two sample t-test. p<0.05 was considered statistically significant. RESULTS: One hundred and fifteen pregnancies complicated by preterm delivery within 32 weeks of gestation after premature rupture of membranes were included. Preterm deliveries occurred within 28 weeks of gestation in 48 cases (41.7%) and between 28 and 32 weeks of gestations in 67 cases (58.3%). Antenatal dexamethasone therapy was done in 27 out of 48 mothers (56.3%) who delivered within 28 weeks of gestation and in 47 out of 67 mothers (70.1%) who delivered between 29 and 32 weeks of gestation. Antenatal dexamethasone therapy did not affect the selected perinatal outcome variables (gestational age at delivery, birth weight, Apgar scores, cesarean section rate, and maternal and neonatal WBC counts and serum C-reactive protein concentrations). Incidences of neonatal complications (respiratory distress syndrome, intraventricular hemorrhage, retinopathy of prematurity, and periventricular leukomalacia) between the groups who did and did not received antenatal dexamethasone were not significantly different in pregnancies who delivered within 28 weeks of gestation. However, incidences of respiratory distress syndrome and intraventricular hemorrhage were significantly lower in a group who received antenatal dexamethasone than in a group who did not in pregnancies who delivered between 29 and 32 weeks of gestation (p<0.05). CONCLUSION: Antenatal corticosteroid therapy may be beneficial to the infants delivered between 29 and 32 weeks of gestation after PROM. However, it may have no therapeutic advantage to the group who delivered within 28 weeks of gestation after PROM.
Birth Weight ; C-Reactive Protein ; Cesarean Section ; Chungcheongnam-do ; Dexamethasone ; Female ; Gestational Age ; Hemorrhage ; Humans ; Incidence ; Infant* ; Medical Records ; Membranes* ; Mothers ; Pregnancy* ; Pregnant Women ; Premature Birth ; Retinopathy of Prematurity ; Retrospective Studies ; Rupture*