No abstract available.
Leukoencephalopathies*

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

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.
Anencephaly* ; Humans ; Pregnancy, Twin* ; Twins*

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*

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

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

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

CD10, a marker of immature B lymphocytes, is expressed in the developing epithelium of mammary glands, hair follicles, and renal tubules of human fetuses. To assess mesenchymal and stromal expression of CD10, we performed immunohistochemical assays in whole body sections from eight fetuses of gestational ages 15-20 weeks. In addition to expression in urinary tract and intestinal epithelium, CD10 was strongly expressed at both gestational ages in fibrous tissues surrounding the airways from the larynx to lung alveoli, in the periosteum and ossification center, and in the glans of external genitalia. CD10 was not expressed, however, in other cavernous tissues. These findings suggest that mesenchymal, in addition to epithelial cells at specific sites, are likely to express CD10. The glomeruli, alveoli, and glans are all end products of budding or outgrowth processes in the epithelium or skin. However, in contrast to the CD34 marker of stromal stem cells, CD10 was not expressed in vascular progenitor cells and in differentiated vascular endothelium. The alternating pattern of CD10 and CD34 expression suggests that these factors play different roles in cellular differentiation and proliferation of the kidneys, airway and external genitalia.
Endothelium, Vascular ; Epithelial Cells ; Epithelium ; Fetus* ; Genitalia ; Gestational Age ; Hair Follicle ; Humans* ; Intestinal Mucosa ; Kidney ; Larynx ; Lung ; Mammary Glands, Human ; Mesoderm ; Periosteum ; Precursor Cells, B-Lymphoid ; Skin ; Stem Cells ; Urinary Tract