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

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)

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

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*

The number of HIV-infected individuals susceptible to Hepatitis A virus (HAV) infection is increasing in Korea; however, it has proven difficult to devise a vaccination policy therefore because limited seroepidemiologic data exists for them. Accordingly, anti-HAV IgG was measured in 188 HIV-infected adults between July 2008 and July 2010. The nadir CD4+ T lymphocyte counts were not different between the HAV-positive and -negative groups (197 +/- 138 vs 202 +/- 129, P = 0.821). The only factor independently associated with seropositive status was age under 40 yr old (OR 0.017, P < 0.001). Our findings suggest that HAV vaccination in HIV-infected adults should be targeted at persons under the age of 40 yr.
Adult ; Age Factors ; CD4 Lymphocyte Count ; Female ; HIV Infections/*complications ; Hepatitis A/complications/*epidemiology ; Hepatitis A Antibodies/blood ; Hepatitis A virus/immunology ; Humans ; Male ; Middle Aged ; Odds Ratio ; Republic of Korea/epidemiology ; Seroepidemiologic Studies

Autoimmune chronic pancreatitis (AIP) typically manifests as diffuse pancreatic swelling and diffuse irregular narrowing of the main pancreatic duct. Recently, mass-forming focal-type AIP, which shows focal pancreatic swelling with mass and focal narrowing of the main pancreatic duct, has been reported. Since this type of AIP is difficult to differentiate from pancreatic cancer, the greater part of these cases have been treated surgically, with the diagnosis confirmed thereafter. Here we report a case of mass-forming focal-type AIP detected in the head of the pancreas; it has been successfully treated with steroids.
Head ; Pancreatic Ducts ; Pancreatic Neoplasms ; Pancreatitis ; Pancreatitis, Chronic

OBJECTIVES: Premature ovarian failure (POF) is a syndrome defined as the cessation of ovarian function before the age of 40 years that is characterized by amenorrhoea associated with elevated gonadotropin levels. The aim of this study was to compare clinical manifestation of primary amenorrhea and secondary amenorrhea group. METHODS: This study was designed as a retrospective multicenter study of 262 women with premature ovarian failure. Sixty eight women with primary amenorrhea and 194 women with secondary amenorrhea were evaluated and hormonal level, lipid profile, bone mineral density, and pregnancy rates were compared. RESULTS: The estradiol level was markedly lower in primary amenorrhea than secondary amenorrhea. The pregnancy rate of 43.3% before the diagnosis in secondary amenorrhea was markedly higher than the rate of 0% in primary amenorrhea. The pregnancy rates after treatment was 5.9% in primary amenorrhea, but 1.0% after diagnosis and 2.8% after treatment in secondary amenorrhea. The pregnancy rate after hormonal treatment was 3.7% in total, 8.3% in primary amenorrhea, and 2.8% in secondary amenorrhea. In nine cases of pregnancy, seven cases were after estrogen-progestin (EP), one case was after clomiphene citrate and one case was after EP/human menopausal gonodotropin (hMG). And In nine cases of pregnancy, six cases resulted from oocyte donation. The prevalence of osteopenia/osteoporosis was markedly higher in primary amenorrhea than in secondary amenorrhea. CONCLUSION: Premature ovarian failure has negative influences on the physical and psychological health of young patients. Effective management should include earlier diagnosis and intensive medical intervention to relieve symptoms of estrogen deficiency and to treat long-term disease such as osteoporosis and in assisted pregnancy by oocyte donation.
Amenorrhea ; Bone Density ; Clomiphene ; Estradiol ; Estrogens ; Female ; Gonadotropins ; Humans ; Oocyte Donation ; Osteoporosis ; Pregnancy ; Pregnancy Rate ; Prevalence ; Primary Ovarian Insufficiency ; Retrospective Studies