Congenital hepatic fibrosis is an inherited, congenital disorder of the liver characterized by portal hypertension and hepatic fibrosis. We experienced a case of congenital hepatic fibrosis with esophageal varix in a 9-year-old male. He complained hematemesis, hematochezia, dizziness. In laboratory examination, AST/ALT was slightly increased. Esophageal varix was noted by an endoscopic examination. Hepatosplenomegaly and hypoechoic lesion of periportal area were seen by abdominal CT scanning. Histologic finding of liver biopsy showed fibrous tracts containing dilated bile ductules connecting adjacent portal spaces that were widened by mature fibrosis. Endocopic sclerotherpy and ligation was done. We summarized a case with review of literatures.
Bile ; Biopsy ; Child ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Dizziness ; Esophageal and Gastric Varices* ; Fibrosis* ; Gastrointestinal Hemorrhage ; Hematemesis ; Humans ; Hypertension, Portal ; Ligation ; Liver ; Male ; Tomography, X-Ray Computed

PURPOSE: We have done this retrospective study to know the relative incidence and clinical manifestations of organic acidopathies in Korea during 8 years(from Jul. 1997 to May 2005). This results of organic acid analysis of 1,787 patients were compared with the results of organic acid analysis that were published three years ago. METHODS: The results of quantitative organic acid analysis of samples of 1788 patients, referred from Jul. 1997 to May 2005, were analyzed retrospectively according to four age group(-2 mon, 3 mon-2 years, 3-12 years) and major clinical manifestations. Quantification of 83 organic acids was done with gas chromatography and mass spectometry. RESULTS: We diagnosed 470 patients with 27 diseases of organic acid metabolism during this study period. Diseases found more than 10 cases are cytosolic 3-ketothiolase deficiency, mitochondrial respiratory chain disorders, PDHC deficiency, mitochondrial 3-ketothiolase deficiency, glutaric aciduria type II, biotinidase deficiency, methylmalonic aciduria and propionic aciduria. Other diseases were diagnosed in less than 10 cases. CONCLUSION: Though the incidence of individual organic acidemia is low, the overall incidence of organic acidemia as a whole seems to be relatively high in Korea. Compared with the results of organic acid analysis that were reported three years ago, we couldn't find a new disease and the difference of the relative incidences of high incident diseases. We were apprehensive of the errors that was owing to the short study period(3 years), but the relative incidences of our study(8 years) were similar to the results of organic acid analysis that were reported three years ago.
Acetyl-CoA C-Acyltransferase ; Biotinidase Deficiency ; Chromatography, Gas ; Cytosol ; Electron Transport ; Humans ; Incidence ; Korea* ; Metabolism ; Multiple Acyl Coenzyme A Dehydrogenase Deficiency ; Neurologic Manifestations ; Propionic Acidemia ; Pyruvate Dehydrogenase Complex Deficiency Disease ; Retrospective Studies

PURPOSE: To evaluate the effects of an intravitreal amniotic membrane (AM) injection on a rat model of laser-induced choroidal neovascularization (CNV). METHODS: Intravitreal injection of AM (12.5 microgram/mL) into the left eyes and phosphate-buffered saline into the fellow eyes of 15 rats was performed immediately after laser injury. At week 2, after angiography, the rats were sacrificed, and histopathologic analysis was performed. Angiographic and histopathologic assessments of the effects of AM injection on CNV growth were compared between the two groups. RESULTS: CNV thickness was found to have increased more in the AM treatment group (141.54+/-8.12 micrometer) than in the experimental control group (65.23+/-16.90 micrometer) (p=0.010). CONCLUSIONS: Intravitreal AM injection demonstrated histopathologic pro-angiogenic properties in a rat model of CNV.
Amnion ; Angiography ; Animals ; Choroid ; Choroidal Neovascularization ; Eye ; Intravitreal Injections ; Rats

BACKGROUND: This research is to determine the chief complaints and related features of elderly patients who are presented to emergency department with medical problems. METHODS: Medical records of patients, 65 years or above, who visited Uijeongbu Hospital Emergency Center between January 1, 2012 and June 30, 2012 were reviewed retrospectively. Age, gender, mode of transportation to the hospital, chief complaints, and diagnosis were among the subjects analyzed. RESULTS: Elderly patients with medical problems, 3,468 visited the emergency department, constituting 12.66% from 27,396 patients in total during the research period. Patients aged 70 to 74 were 28.45%, composing the most among the age groups. Ambulance was the mode of transportation used by 43.06% of the patients and 42.96% of them stayed overnight, while 11.13% stayed in the intensive care units. The most frequent chief complaints were abdominal pain (16.81%), dyspnea (13.96%), and fever (11.16%). The most common diagnosis for patients with abdominal pain was gastritis (20.75%), chronic heart failure (26.03%) for dyspnea, and pneumonia (28.96%) for fever. The main diagnoses of in-patients according to the order of frequency were cerebral vascular accident (16.38%), pneumonia (12.48%), and chronic heart failure (6.04%). CONCLUSION: The number of elderly patients who stayed overnight and stayed in the intensive care units have increased comparing to younger patients. The top 10 most frequent chief complaints accounted for 78.92% by medical elderly patients. The results of this research could be used for the development of geriatric emergency medicine training programs and critical pathway for interns and residents.
Abdominal Pain ; Aged ; Ambulances ; Critical Pathways ; Dyspnea ; Emergencies ; Emergency Medicine ; Fever ; Gastritis ; Heart Failure ; Humans ; Intensive Care Units ; Medical Records ; Pneumonia ; Retrospective Studies ; Transportation

In 22 end-stage renal disease patients on regular hemodialysis, echocardiographic study including 2-D Doppler echocardiogrphy was performed to evaluate the effect of hemodialysis on cardiac performance and the degree of valvular regurgitation. After hemodialysis, there were significant improvement of ventricular contractility and reduction of circulating volume in accordance with the reduction of degree of valvular regurgitation in comparison with before hemodialysis. We postulate that the reduction of valvular regurgitation after hemodialysis is caused by relief of hypervolemic state.
Echocardiography* ; Echocardiography, Doppler ; Humans ; Kidney Failure, Chronic ; Renal Dialysis*

Arotinolol, a new alpha and beta receptor antagonist, was administered in 27 essential hypertensive patients for 8 weeks in order to evaluate the antihypertensive effect and side effects. The dose were 10mg to 15mg given twice a day. The results are as follows : 1) Before medication, systolic and diastolic blood pressure in sitting, supine and erect position were 173.1+/-3.2/105.8+/-1.8, 171.1+/-3.6/86.7+/-2.0 and 169.3+/-2.6/97.2+/-2.1mmHg. 2) After 8 weeks treatment moderated to marked antihypertensive effect was observed in 74.0%(in systolic blood pressure) and 81.4%(in diastolic blood pressure) respectively in sitting position. 3) There was a significant reduction of pulse rate from 73.0+/-2.5 beats per minute on the beginning of the treatment to 63.4+/-5.2 beats per minute after 8 weeks of medication. 4) There was no significant change in hematocrit, WBC, serum lipid, GOT, GPT, BUN and creatinine. But fasting blood sugar was reduced from 95.3mg% to 81.5mg% with treatment. 5) The side effects of arotinolol were gastrointestinal symptoms(15%), fatigue(11%), dizziness(7%) and insomnia(3%). But these side effects were not severe enough to discontinue medication. In summary, arotinolol seemed to be an effective antihypertensive drug in treating mild to moderate hypertension without significant side effects.
Blood Glucose ; Blood Pressure ; Creatinine ; Fasting ; Heart Rate ; Hematocrit ; Humans ; Hypertension

OBJECTIVE: To investigate the effect of treadmill aerobic exercise for eight weeks on cardiopulmonary fitness and functional mobility in stroke patients METHOD: Twenty one stroke patients who could walk independently and had MMSE score of 24 or higher were included. We used maximal exercise test by means of modified Harbor protocol before and after eight weeks' treadmill aerobic exercise. We monitored patients' status with EKG, heart rate, blood pressure and Borg rating of perceived exertion, and measured maximal O2 consumption, maximal heart rate, blood pressure, minute ventilation, rate pressure product and respiratory exchange ratio during maximal exercise test. We also assessed functional mobility by Fugl-Meyer assessment of lower extremity, Berg balance scale, five times sit to stand test, timed up & go test, dynamic gait index scale, activities-specific balance confidence scale and Korean-modified Barthel index before and after 8 weeks' training. RESULTS: Twenty one patients completed treadmill aerobic exercise training. After eight weeks' treadmill aerobic exercise, the cardiopulmonary fitness, measured by maximal O2 consumption, maximal heart rate, minute ventilation, systolic blood pressure, functional mobility measured by Fugl-Meyer assessment of lower extremity, Berg balance scale, five times sit to stand test, timed up & go test, dynamic gait index scale, activities-specific balance confidence scale and Korean-modified Barthel index showed statistically significant improvement (p<0.05). CONCLUSION: Eight weeks' treadmill aerobic exercise improved the cardiopulmonary fitness and functional mobility in ambulatory stroke patients.
Blood Pressure ; Electrocardiography ; Exercise ; Exercise Test ; Gait ; Heart Rate ; Humans ; Lower Extremity ; Stroke ; Ventilation

PURPOSE: This study was undertaken to recognize the relapse rate and risk factors of relapse after discontinuation of antiepileptic drugs in patients with benign epilepsy of childhood with centro-temporal spikes(BECT). METHODS: The subjects were 57 patients with BECT, who visited our hospital from January 1990 to December 2004. They were followed up for more than 24 months after discontinuation of antiepileptic drugs. And they were analysed on the relapse rate and the factors that were presumed to influence the relapse. RESULTS: Seizures were relapsed in 7 of 57 patients(12.2%) after discontinuation of antiepileptic drugs. Relapses were more frequent in patients who took antiepileptic drugs for seizure control more than 12 months(15.0%) than those who didn't(10.8%). Moreover, more relapses occurred to those who were at higher age at onset and who took antiepileptic drugs less than 36 months(100%). Furthermore, those who had abnormalities in EEG experienced more relapses(33.3%) than others. The relapse rate was not significantly different in the aspect of and sex seizure frequency after discontinuation of medication between relapsed and non-relapsed patients. CONCLUSION: It is concluded that we should consider the risk factors of relapse in patients with BECT in order to avoid relapse when discontinuing antiepileptic drugs. Moreover, further studies are needed to clarify the risk factors of relapse.
Anticonvulsants* ; Electroencephalography ; Epilepsy* ; Humans ; Recurrence ; Risk Factors ; Seizures

Background: Data on severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2) delta variant virulence are insufficient. We retrospectively compared the clinical features of adult coronavirus disease 2019 (COVID-19) patients without risk factors for severe COVID-19 who entered residential treatment centers (RTCs) before and after the delta variant outbreak. Methods: We collected medical information from two RTCs in South Korea. On the basis of nationwide delta variant surveillance, we divided the patients into two groups: 1) the delta-minor group (diagnosed from December 2020–June 2021, detection rate < 10%) and 2) the delta-dominant group (diagnosed during August 2021, detection rate > 90%). After propensity-score matching, the incidences of pneumonia, hospital transfer and need for supplemental oxygen were compared between the groups. In addition, risk factors for hospital transfer were analysed. Results: A total of 1,915 patients were included. The incidence of pneumonia (14.6% vs.9.2%, P = 0.009), all-cause hospital transfer (10.4% vs. 6.3%, P = 0.020) and COVID-19-related hospital transfer (7.5% vs. 4.8%, P = 0.081) were higher in the delta-dominant group than those in the delta-minor group. In the multivariate analysis, the delta-dominant group was an independent risk factor for all-cause (adjusted odds ratio [aOR], 1.91; 95% confidence interval [CI], 1.16–3.13; P = 0.011) and COVID-19-related hospital transfer (aOR, 1.86; 95% CI, 1.04–3.32; P = 0.036). Conclusion Hospitalization rates were increased in the adult COVID-19 patients during the delta variant nationwide outbreak. Our results showed that the delta variant may be more virulent than previous lineages.

OBJECTIVE: The objective of this study was to determine whether postoperative conventional angiography conducted during the early stage after aneurysm clipping is useful in patients with ruptured aneurysm. METHODS: Between May 2008 and November 2009, 57 patients who presented with ruptured cerebral aneurysms were treated with surgical clipping. Among them, a consecutive series of 45 patients who underwent postoperative angiography was analyzed retrospectively. Parameters of the postoperative angiography were categorized as incomplete clippings, vasospasms, vascular compromises, or other aneurysms. RESULTS: The average age of the patients was 49.2 years and ranged from 18 to 72 years. The average timing of the postoperative angiography after the onset of hemorrhage was 11.1 (+/- 5.6) days. Complete aneurysm closure was achieved in 43 (95%) patients. A neck remnant aneurysm was discovered in two patients and a fundus remnant was revealed in one patient. Twelve (27%) patients showed angiographic vasospasms and seven needed angioplasty. Four cases (9%) revealed either parent artery stenosis or branch occlusions, and two of them (4%) were clinically significant. Two cases showed aneurysms at another location, one of which was a ruptured aneurysm. Overall, 21 (47%) patients exhibited significant findings on the postoperative angiography. CONCLUSIONS: Our retrospective analysis revealed that postoperative angiography might be valuable in patients with ruptured aneurysms, especially in the acute stage, in order to determine the presence of vasospasms, incomplete clippings, vascular compromises, or other aneurysms that were missed at the initial cerebral angiography.
Aneurysm ; Aneurysm, Ruptured ; Angiography ; Angioplasty ; Arteries ; Cerebral Angiography ; Constriction, Pathologic ; Hemorrhage ; Humans ; Intracranial Aneurysm ; Neck ; Parents ; Retrospective Studies ; Subarachnoid Hemorrhage ; Surgical Instruments