Edwards syndrome is first introduced by Edwards and characterized by facial anomalies, multiple cardiovascular, gastrointestinal, urogenital, and skeletal malformations. It results from triplication of part or all of chromosome 18 in some or all of the patient's cells. It has an incidence of 1 in 4,500 live births or less and short life expectancy. Recently we experienced a case of 3-day-old female new born infant with this syndrome. Post mortem examination showed progeric face with prominent occiput, large flabby ears, microphthalmia, and micrognathia, bilateral clenched hands with flexion contraction of middle fingers, and bilateral rockerbottom feet. Internal examination revealed horseshoe kidney, esophageal atresia with tracheoesophageal fistula, two accessory spleens, and multiple cardiac anomalies. A trisomy 18 was confirmed by the cytogenetic study.
Infant ; Male ; Female ; Infant, Newborn ; Humans ; Incidence

BACKGROUND: Recently, clinical use of fresh frozen plasma(FFP) has increased in all blood transfusion activity. But inappropriate uses of FFP may be serious problem due to adverse transfusion reactions, viral transmission, and economic aspects. The aim of this study is to justify clinical indication of FFP by receiving the inappropriate use of them. METHODS: Analyzing hospital records of 115 patients transfused with FFP at Kyung Hee Medical Center from January to April 1998, retrospectively, we investigated the purpose of transfusions, the volume of transfused FFP, and the results of pre- and post-coagulation tests. RESULTS: The most common purpose of transfusions of FFP was plasma volume replacement after surgical operation. Coagulation tests of pre- and post-transfusion were performed 85% and 47%, respectively. Fifty-seven patients(88%) transfused FFP have shown normal results in pre-coagulation tests, and the volume replacement with FFP was done inadequately in 62 cases(54%). CONCLUSION: We conclude that, in order to eliminate side effects due to inappropriate use of FFP, and protect patients, it is necessary to make guidelines of FFP transfusion, educate hospital staffs regularly, and perform pre- and post-coagulation tests routinely for the patients who need FFP transfusion. (Korean J Blood Transfusion 10(1): 5-12, 1999)
Blood Group Incompatibility ; Blood Transfusion ; Hospital Records ; Humans ; Plasma Volume ; Plasma* ; Retrospective Studies

No abstract available.
Encephalomyelitis, Acute Disseminated*

Factor VIII inhibitors are produced during or after coagulation factor VIII (FVIII) therapy in hemophilia A patients. These inhibitors are usually detected by a modified Bethesda assay or an enzymelinked immunosorbent assay (ELISA). In this study, we used the Bethesda assay to determine the incidence of FVIII inhibitors in 75 fresh plasma samples obtained from 50 hemophilia A patients, and then used ELISA and the Bethesda assay to determine the titres of these inhibitors after the samples had been frozen and thawed. The samples from the screening Bethesda assay were centrifuged and stored at -70degrees C in accordance with the assay guidelines. Subsequently, these samples were thawed and analyzed using ELISA and the Bethesda assay. The incidence of inhibitors in hemophilia A patients was 20.0%. Among the 35 inhibitor-positive samples identified in the screening Bethesda assay, 16 were positive in ELISA while only 4 were positive in the repeated Bethesda assay. In this study, the ELISA technique showed a higher sensitivity than the Bethesda assay in the detection of FVIII inhibitors in samples that were subjected to freezing and thawing procedures; this was because the Bethesda assay could not identify the FVIII inhibitors that were degraded after freezing and thawing.
Blood Coagulation Factor Inhibitors/*analysis ; *Enzyme-Linked Immunosorbent Assay ; Factor VIII/*antagonists &inhibitors/metabolism ; Hemophilia A/*blood/diagnosis ; Humans ; Immunologic Tests ; Male

Astrovirus is frequently associated with diarrhea in children. It can not be readily isolated by cell culture, and an electronmicroscope is usually used for detection of this agent. Recently in 1995 a combined method of reverse transcription-polymerase chain reaction (RT-PCR) was designed for easier detection of astrovirus, which is based on the conserved sequence in 3'-end of genomes of the 7 known serotypes of human astrovirus. As of yet there has not been any report of astrovirus data in Korea using the RT-PCR methods. The purpose of this study was to detect astrovirus incidence, severity of symptoms, seasonal variation and coinfection rate with rotavirus in Korean children inpatients with diarrhea. Fecal specimens from 61 young children hospitalized with gasteroenteritis Korea from Jan. 1996 through Mar. 1997. They were examined for astroviurs infection by RT-PCR method. Results are as follows: 1. Astrovirus was detected at 9.8% (6/61) from fecal specimens of children with severe diarrhea by EIA using monoclonal antibody coated plates. 2. Astorvirus was detected at 29.5% (18/61) from fecal specimens of children with severe diarrhea by RT-PCR. 3. The age of the 18 children affected by astrovirus ranged from 2 monthes to 7 years with mean of 3.0 years. 4. Mean hospital stay of the 1S children was 6.1 days. 5. Five (27.8%) astrovirus RT-PCR positive strains were confirmed in November and in December, respectively out of 18 specimens in total. 6. Astrovirus coinfection with rotavirus type G1 was confirmed in 15/16 specimens (93.8%), and with type G2 was in 1/16 specimens (6.3%).
Cell Culture Techniques ; Child* ; Coinfection ; Conserved Sequence ; Diarrhea ; Feces* ; Genome ; Humans ; Incidence ; Inpatients ; Korea ; Length of Stay ; Mamastrovirus ; Polymerase Chain Reaction* ; Reverse Transcription* ; Rotavirus ; Seasons

Human rotavirus has now been established as the leading cause of gastroenteritis in young children worldwide. At least fourteen serotypes of group A rotavirus have been identified on the basis of antibody responses to major neutralizing glycoprotein, VP7 (G type for glycoprotein), present in the outer capsid of the virus. Serotype 1, 2, 3 and 4 are the most highly prevalent in human. In Korea, rotavirus is also the principal cause of severe nonbacterial diarrhea requiring hospitalization in infants and young children, which is commonly detected by EIA method. The epidemiology of rotavirus infection has been monitored by only serologic methods without electropherotyping in Korea. This study shows seasonal and age related variations .of rotavirus infection in Korea according to the genotype using the reverse transcription polymerase chain reaction (RT-PCR). Fecal specimens were obtained from 39 children hospitalized with acute watery diarrhea and gastroenteritis in Ewha Womans University MokDong Hospital in Seoul from Jan. to Dec. of 1996. All four (1, 2, 3, 4) major G serotypes were identified by amplification of segment of the gene for VP7 using RT-PCR. Rotavirus Gl 749 bp, G2 653 bp, G3 374 bp and G4 583bp were shown on 2.9 or 3.3% NuSieve agar gel. Results were as follows: 1) Rotavirus was detected at 53.8% (21/39) by EIA and 89.7% (35/39) by RT-PCR. 2) Serotype Gl, G2, G3, G4 when detected by RT-PCR accounted for 80.0% (28/35), 14.3% (5/35), 2.9% (1/35) and 2.9% (1/35), respectively. 3) Thirty five strains of rotavirus were detected at the frequency of 17.1% (6/35) in Oct., 20.0% (7/35) in Nov. and 20.0% (7/35) in Dec. 4) As for the age range, children affected by rotavirus were mostly under 1 years.
Agar ; Antibody Formation ; Capsid ; Child ; Child, Hospitalized* ; Diarrhea* ; Epidemiology ; Female ; Gastroenteritis ; Genotype* ; Glycoproteins ; Hospitalization ; Humans* ; Infant ; Korea ; Polymerase Chain Reaction* ; Reverse Transcription* ; Rotavirus Infections ; Rotavirus* ; Seasons ; Seoul

No abstract available.
Liver* ; Radioactivity* ; Radionuclide Imaging*

No abstract available.
Brain Injuries* ; Brain* ; Disability Evaluation* ; Humans ; Perfusion* ; Technetium Tc 99m Exametazime* ; Tomography, Emission-Computed, Single-Photon*

No abstract available.
Neonatology

The omentum is unique in that it is the body tissue specially developed for the resolution of local inflammatory processes because of combination of its blood supply and abundant lymphatic drainage. So, the use of omental pedicle graft is well established for the more complex repairs and reconstruction of the urinary tract. From March, 1982 to February, 1988, we have experienced twelve cases of the urinary tract repairs or reconstruction using omental pedicle graft, which include six cases of multiple renal laceration, two cases of renal staghorn calculi and four cases of complicated vesicovaginal fistula and the result were excellent.
Calculi ; Drainage ; Lacerations ; Omentum ; Transplants* ; Urinary Tract* ; Vesicovaginal Fistula