No abstract available.
Hemorrhage*

Herpes simplex esophagitis can occur in those with normal immune function, but is more often seen in those who are immunocompromised. In one series, 5 percent of post-kidney transplant recipients had herpes esophagitis. We experienced a case of herpes simplex esophagitis, following renal transplantation in a 9 year old male. He complained of epigastric pain, nausea and blood-tinged vomiting. Endoscopic examination showed volcano ulcer, mucosal friability and multiple confluent ulcers covered by whitish exudates on elevated margin in the middle and lower esophagus. Microscopic findings revealed multinucleated giant cells, margination of chromatin, intense nonspecific inflammation and strong positive for herpes simplex virus immunohistochemical staining. Esophageal lesions and symptoms improved after acyclovir therapy.
Acyclovir ; Child* ; Chromatin ; Esophagitis ; Esophagus ; Exudates and Transudates ; Giant Cells ; Herpes Simplex* ; Humans ; Inflammation ; Kidney Transplantation ; Male ; Nausea ; Simplexvirus* ; Transplantation ; Ulcer ; Vomiting

Serological prenatal screening tests are widely used to detect fetal chromosomal abnormalities such as Down and Edward syndromes. Amniocentesis is conducted as a confirmatory test in the screening-positive case. After discovering of presence of fetal cell-free DNA in maternal blood, non-invasive prenatal test (NIPT) coupled with next generation sequencing are performed in abroad. Results of genomics-based NIPT results supplied to Labgenomics laborotory from June, 2013 to August, 2014 were analyzed. Maternal blood samples were collected into specific Cell-Free DNA BCT tube and were transported. The samples were then delivered to Ariosa Diagnostics by FEDEX. Fetal cell-free DNA samples were analyzed using the Harmony test with sequencing of relevant chromosomes and by using the FORTE (fetal-fraction optimized risk of trisomy evaluation) algorism at Ariosa Diagnostics. In all, 149 cases from 28 medical clinics were analyzed. Six subjects were required recollection of samples because of a low fetal DNA fraction in the initially obtained samples. Of these 6 subjects, no sample could be collected from one. Of the remaining 148 cases, 144 had a low risk of trisomy, and 4 had a high risk for Down syndrome, thus providing a positivity percentage of 2.7%. Fetal DNA fraction in the maternal blood samples ranged from 4.2% to 23.7% with a mean value of 12.0%. We have experienced cases with a high risk for Down syndrome with genomics-based NIPT referred to abroad.
Amniocentesis ; Chromosome Aberrations ; DNA ; Down Syndrome ; Prenatal Diagnosis ; Trisomy

No abstract available.
Animals ; Linoleic Acid* ; Macrophages, Peritoneal* ; Mice*

BACKGROUND: Unexpected deaths of the patients at ED are surprising to the medical staffs as well as the families. It may also increase the possibilities of medico-legal problems. This study was conducted to review the unexpected death in ED and find the predictive factors leading patients to unexpected death. METHODS: A retrospective study with reviewing available medical records of 183 patients who were expired at ED of Shinchon Severance Hospital from Mar. 1997 to Feb. 1998 and 103 patients of Youngdong Severance Hospital from Mar. 1996 to Feb. 1998 was done. Analyses were 4one on 30 variables of physical findings, laboratory results, procedures and therapies. We used univariate analysis, such as t-test and x2-test and multiple logistic regression analysis. RESULTS: Of the total 286 deaths in ED, 153 cases were enrolled in this analysis. The number of unexpected deaths were 76. Age, mental status, fraction of neutrophil count, BUN level, whether endotracheal incubation was done, use of inotropics were the factors with statistical ,significance on the univariate analysis, but only comatose mentality, endotracheal incubated case, and use of the inotropics were significant on logistic regression analysis. The longer the ED length of stay, the higher the rate of mortality and unexpected death. CONCLUSION: This result suggest that medical attention is needed on the patients with comatose decreased mental stylus, endotracheally intubated case and needing inotropic agents to prevent unexpected deaths. Shortening the ED length of stay may also be a factor for decrease the unexpected death rate.
Coma ; Emergencies* ; Emergency Service, Hospital* ; Humans ; Length of Stay ; Logistic Models ; Medical Records ; Medical Staff ; Mortality ; Neutrophils ; Retrospective Studies

A twenty four-year-old female patient had suffered progressive dyspnea for 6 years until death. She denied any symptoms suggestive of connective tissue disease, or deep vein thrombosis. She suffered an episode of pontine infarct in 1995. Four years after diagnosis of primary pulmonary hypertension, she died of sudden death during hospitalization. Gross features of pulmonary arteries at autopsy were as follows: left main pulmonary artery showed dilation of the lumen and thickening of the wall, and right main pulmonary artery was markedly dilated and contained fresh thrombus. Hematoxylin and eosin-stained sections of lung tissue showed plexiform lesions of pulmonary arteries, complete luminal obliteration of pulmonary arterioles and dilated lesion of pulmonary arterioles, and capillaries. This patient represents a typical case with a primary pulmonary arteriopathy with plexiform lesions with thrombotic lesion, demonstrating the importance of thrombosis in situ in the pathogenesis of primary pulmonary hypertension. To our knowledge, this is the first autopsy report on the primary pulmonary hypertension in Korea.
Arterioles ; Autopsy ; Capillaries ; Connective Tissue Diseases ; Death, Sudden ; Diagnosis ; Dyspnea ; Female ; Hematoxylin ; Hospitalization ; Humans ; Hypertension, Pulmonary* ; Korea ; Lung ; Phenobarbital ; Pulmonary Artery ; Thrombosis ; Venous Thrombosis

BACKGROUND/AIMS: Intestinal lymphangiectasia is a disease characterized by dilated lymphatics of the intestinal mucosa and excessive enteric loss of plasma proteins. Instead of multiple blind peroral jejunal biopsy, duodenal endoscopy and endoscopic small bowel biopsy were performed. We evaluated the significance of endoscopic small bowel biopsy and the usefulness of the other diagnostic methods in the diagnosis of intestinal lymphangiectasia in children. METHODS: Fourteen children seen between August 1989 and August 1997 with clinically suggestive intestinal lymphangiectasia were analysed. The median age at onset of symptoms was 4 years. Primary intestinal lymphangiectasia occurred in 10 children and secondary intestinal lymphangiectasia occurred in 4 children, of whom two had Fontan operation, one had constrictive pericarditis, and one had Crohn's disease. Low fat, high protein diet with medium chain triglycerides was the mainstay of treatment. RESULTS: 1) Diarrhea was present in 14 patients, and edema in 11 patients. Hypocalcemic tetany occurred in 6 children and vomiting in 5 children. Dight children had ascites and three of these had chylous ascites. Growth retardation was present in four patients, chylothorax in one, and lymphedema in one. The initial serum albumin concentration was 1.8 g/dl, the serum calcium level 6.7 mg/dl, and the total lymphocyte count 623 /mm(3). 2) Dilated lymphatics in the small bowel mucosa was confirmed by endoscopic biopsy in 14 children(100%). The sensitivity of alpha1-antitrypsin clearance was 100%. Duodenal endoscopy showed scattered white spots covering mucosa in 11 children(79%). Small bowel series revealed thickened mucosal folds in 10 children(77%). Four(31%) had positive finding of 99mTc-antimony lymphoscintigraphy. 3) Responses to treatment in children with primary intestinal lymphangiectasia were graded as good if the symptoms resolved, and poor if there was no lasting resolution of symptoms and repeated albumin administration. Response to therapy was good in four and poor in six patients. The mean age at onset of symptoms was 8 years in good resonse group, and 2 years in poor response group (p<0.05). CONCLUSION: The diagnosis of intestinal lymphangiectasia is confirmed by duodenoscopy and endoscopic small bowel biopsy in a child with diarrhea, edema, hypoalbuminemia, and lymphocytopenia. As compared with other diagnostic methods such as small bowel series and lymphoscintigraphy, duodenoscopy and endoscopic small bowel biopsy are very sensitive and should be performed early.
Ascites ; Biopsy* ; Blood Proteins ; Calcium ; Child* ; Chylothorax ; Chylous Ascites ; Crohn Disease ; Dental Caries ; Diagnosis* ; Diarrhea ; Diet ; Duodenoscopy ; Edema ; Endoscopy ; Fontan Procedure ; Humans ; Hypoalbuminemia ; Intestinal Mucosa ; Lymphedema ; Lymphocyte Count ; Lymphopenia ; Lymphoscintigraphy ; Mucous Membrane ; Pericarditis, Constrictive ; Serum Albumin ; Tetany ; Triglycerides ; Vomiting

No abstract available.
Paralyses, Familial Periodic*

PURPOSE: The Pediatric Risk of Mortality(PRISM) III score was developed from the Physiologic Stability Index(PSI) to assess pediatric ICU mortality and Provide an objective data as a severity index. Although the PRISM score has been applied to many comparisions and analyses in previous studies, there are few reports applied to pediatric intensive care patients in Korea. To evaluate the effectiveness of the PRISM III score as a severity index for expecting mortality and find important variables influencing mortality, we applied this scoring scale to pediatric neurologic patients admitted to the ICU and analyzed the data statistically. METHODS: Data collection was done by careful review of medical records and scored each clinical variable. The outcome at discharge was determined as non-survival, survival, and hopeless discharge. Determination of mortality in the hopeless discharge group was done within 48 hours after discharge by telephone interview. The study populations were classified into four groups; CNS infection(26 patients), acute encephalopathy(31 patients), status epilepticus(35 patients) and cerebrovascular disorder(4 patients). The difference of the PRISM III score between the survival group and non-survival group was compared by using the nonparametric Mann~Whitney test in the entire study population and for each diagnostic group. To confirm the degree of fitness between the actual mortality and Predicted mortality, the Hosmer-Lemeshow goodness-of-fit test, a multiple logistic regression model was used. All clinical variables used for scoring were compared for survivals and non-survivals by the Chi-square test. f values <0.05 were considered significant. RESULTS: The PRISM III score was significantly higher in the non-survival groups than in the survival group. Predicted mortality from the PRISM III score has fitted to actual mortality According to the results of analyses in each diagnostic groups, the PRISM III score was higher in non-survivals of the acute encephalopathy and CNS infection groups, but statistically insignificant in the cerebrovascular disorders and status epilepticus groups. The important variables of the PRISM III score associated with mortality were mental state, Pupil reflex, systolic blood pressure, acidosis, blood sodium level blood creatinine level, blood glucose level, and PT/PTT. , CONCLUSION: The PRISM III score is helpful in predicting mortality in pediatric intensive care neurologic patients, especially those in the acute encephalopathy or the CNS infection groups. However, this score was not useful in the status epilepticus group, and insignificant in cerebrovascular group. Due to the smallness of the study group, more massive and comprehensive studies are needed as a follow up to this study.
Acidosis ; Blood Glucose ; Blood Pressure ; Cerebrovascular Disorders ; Creatinine ; Data Collection ; Follow-Up Studies ; Humans ; Critical Care* ; Interviews as Topic ; Korea ; Logistic Models ; Medical Records ; Mortality* ; Pupil ; Reflex ; Sodium ; Status Epilepticus

No abstract available.