No abstract available.
Leukoencephalopathies*

No abstract available.
Anencephaly* ; Humans ; Pregnancy, Twin* ; Twins*

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

No abstract available.
Doxorubicin* ; Membrane Potentials* ; Membranes* ; Papillary Muscles*

We describe the case of a 45-year-old woman who had self-regressing livedo reticularis on the lower extremities. Examination of a biopsy specimen from the mottled area revealed myxomatous emboli in the deep dermal arterioles. Echocardiography showed a myxoma in the left atrium. After the tumor was surgically excised, the patient had no further evidence of the disorders during the 3-year period of follow-up. Livedo reticularis caused by an peripheral arterial embolism, which in turn was caused by the tumor fragments in our patient with left atrial myxoma, is considered to be unusual.
Arterioles ; Biopsy ; Echocardiography ; Embolism ; Female ; Follow-Up Studies ; Heart Atria ; Humans ; Livedo Reticularis* ; Lower Extremity ; Middle Aged ; Myxoma*

OBJECTIVE: As an electronic health record system is implementing in Korean health care sectors, concerns about key functionalities of electronic health record systems are increasing. The purpose of this study was to identify core functions and set the priority in electronic health record systems under the Korean contexts in order to assure and improve the quality of the systems. METHODS: A survey was conducted using questionnaire developed by the study team based on literature review. The subjects were medical record administrators working at medical record department in general hospitals. RESULTS: The response rate was 59.8%(55/92). The functions which more than ninety percent of subjects responded as necessary right now and/or in near future related to 'drug alert', 'clinical guideline', 'chronic disease management', 'automated real-time surveillance', 'coded data', 'result reporting', 'de-identifying data', 'disease registry', and 'provider-provider communication and connectivity'. CONCLUSION: The results showed the high prioritized functions were decision support and health information/data management.
Electronic Health Records* ; Health Care Sector ; Hospitals, General* ; Humans ; Medical Record Administrators ; Medical Records ; Needs Assessment* ; Surveys and Questionnaires

BACKGROUND: Prior reports of umbilical vein variation were strongly associated with several congenital anomalies. This suggests that the incidence of umbilical vein anomaly was more frequently seen in dead fetuses than in liveborn infants because the leading causes of stillbirth are chromosomal and/or congenital anomalies. The developing liver exerts a profound influence in modifying the primitive vitelline and umbilical veins. This study was undertaken to identify the umbilical vein variations and the associated hepatic and perihepatic structural anomalies in dead fetuses. METHODS: Dissection was done in eighteen dead fetuses who had undergone delivery at Chonbuk National University Hospital between December 1996 and February 1998. The weight ratios of the liver to the body and the right to the left hepatic lobes and the distal attachment of umbilical vein, including the presence of hepatic fissures and ligaments, were examined. According to the gestational age, the cases were divided into two groups (group 1: < 32 weeks, 13 cases; group 2: > or = 32 weeks, 5 cases). RESULTS: The gestational age of the fetuses ranged from 19 to 39 weeks. The fetal liver constituted 3.7% and 4.0% of the total fetal body weight for group 1 and 2, respectively. The mean weight ratio of the right to the left hepatic lobe was between 1:1.4 and 1:1.5. There were no gross morphologic abnormalities in 9 cases. Eighteen kinds of chromosomal and congenital anomalies, including omphalocele, anomalous hepatic segmentation, hypogenesis of the hand and the foot, syndactyly, polycystic kidney, etc., were observed in 9 dead fetuses. A case of an abnormal distal attachment of the umbilical vein was identified. The umbilical vein drained into the dilated extrahepatic portal vein directly. The hepatic segments and fissures were completely normal, but ligamentum teres was not identified in this case. A peculiar hepatic segmental anomaly due to whole organ herniation through a defect in an omphalocele was observed. CONCLUSIONS: The weight of the liver of the dead fetuses was relatively small in both groups. No weight shift of left to right was recognized. The incidence of congenital anomalies was much higherin the dead fetuses (50%) than in the live births (0.7-1.98%). There were two hepatic anomalies (11.1%), including segmentation and distal attachment of the umbilical vein. The authors reviewed all reports of umbilical vein anomalies and propose a classification for umbilical vein variations focused on the distal points of attachment. All variations of the umbilical vein tend to fall into two main groups. In the first group, the veins have distal points of attachment into portal vein systems. In the second group, the veins have distal points of attachment into systemic veins.
Classification* ; Fetal Weight ; Fetus* ; Foot ; Gestational Age ; Hand ; Hernia, Umbilical ; Humans ; Incidence ; Infant ; Jeollabuk-do ; Ligaments ; Live Birth ; Liver ; Polycystic Kidney Diseases ; Portal Vein ; Stillbirth ; Syndactyly ; Umbilical Veins* ; Veins ; Vitellins

No abstract available.
Adrenoleukodystrophy*

Prevention of fatal postoperative complications and improved management of patients with complications are important means for achieving increased survival in gastric cancer patients. A study of 390 patients undergoing gastrectomies at the Department of Surgery, Chonbuk National University Hospital, during 6 years from 1990 to 1995 was performed to examine factors related to a high rate of postoperative complications and to compare specific and non-specific complications. Postoperative complications developed in 185 (47.4%) out of the total 390 cases undergoing gastrectomies for gastric cancer; including 107 (27.4%) specific and 78 (20.0%) non-specific. Typical complications were 46 cases of diarrhea, 20 cases of intestinal obstruction including an adhesive and paralytic ileus, 16 cases of vomiting, 7 cases of anastomotic leakage, 7 cases of hemorrhage, 6 cases of anastomosis stenosis, 2 cases of duodenal stump leakage, 1 case of acute pancreatitis, 1 case of pancreatic fistula, 1 case of enterocutaneous fistula within the category of specific complications and 22 cases of elevated hepatic enzymes, 21 cases of pulmonary disease, 2 cases of abnormal EKG, 2 cases of sepsis, 2 cases of cerebral infarction, and 1 case of renal failure within the category of non-specific complications. Multivariate analysis identified combined resection, radical resection in stage IV in operative factors, blood(PRC) transfusion of over 4 units, elevation of sGOT and sGPT, age between 50 and 65, diabetic mellitus of coexistic diseases in non-operative factors as detrimental factors with an independent influence on the postoperative complications for gastric cancer. We believe that the postoperative complications for gastric cancer did not develope because of local and operative factors, but were significantly common when general and non-operative factors were combined with gastrectomy.
Adhesives ; Alanine Transaminase ; Anastomotic Leak ; Aspartate Aminotransferases ; Cerebral Infarction ; Constriction, Pathologic ; Diarrhea ; Electrocardiography ; Gastrectomy ; Hemorrhage ; Humans ; Intestinal Fistula ; Intestinal Obstruction ; Intestinal Pseudo-Obstruction ; Jeollabuk-do ; Lung Diseases ; Multivariate Analysis ; Pancreatic Fistula ; Pancreatitis ; Postoperative Complications ; Renal Insufficiency ; Sepsis ; Stomach Neoplasms* ; Vomiting

OBJECTIVE: To compare the efficacy of suprapapillary and transpapillary meth-ods of transhepatic biliary metallic stent placement in malignant biliary strictures and to specify the indications of each method applied. MATERIALS AND METHODS: Stents were placed in 59 patients. Strictures were cat-egorized as type A (within 3 cm of the ampulla, n = 27), type B (over 3 cm from ampulla, n = 7), type C (within 3 cm of the bending portion, n = 9), or type D (over 3 cm above the bending portion, n=16). The stenting method was suprapapillary in 34 cases and transpapillary in 25. The rates of initial and long-term patency and of early recurrence were compared. RESULTS: Initial patency rates for the suprapapillary and transpapillary methods were 1/7 (14.3%) and 20/20 (100%) respectively for type A (p < 0.0001), 4/5 (80.0%) and 2/2 for type B, 3/7 (42.9%) and 2/2 for type C, and 15/16 (93.8%) and 0/0 for type D. Early recurrence rates were 7/30 (23.3%) using the suprapap-illary method and 4/29 (13.8%) using the transpapillary method (p = 0.51). The long-term patency rate did not differ significantly according to either type (p =0.37) or method (p = 0.62). CONCLUSION: For good initial patency, the transpapillary method is recommended for strictures of the distal extrahepatic duct near the ampulla and just above the bending portion. Long-term patency is not influenced by the stenting method employed.
Bile Duct Neoplasms/complications ; Bile Duct Obstruction, Extrahepatic/etiology/*therapy ; Cholangiography ; Comparative Study ; Digestive System Neoplasms/complications ; Female ; Human ; Male ; Middle Age ; Palliative Care/*methods ; *Radiology, Interventional ; Retrospective Studies ; *Stents ; Tomography, X-Ray Computed