PURPOSE: Kawasaki disease(KD) manifests a systemic vasculitis of unknown etiology in young children. Vascular endothelial growth factor(VEGF), vascular cell adhesion molecule-1(VCAM-1) and interleukin-1 beta(IL-1beta) may play important roles in the pathogenesis of KD. Intravenous immunoglobulin(IVIG) and methylprednisolone(MP) are therapeutically effective for KD, however, the precise mechanisms of the two drugs are still unknown. We investigated the therapeutic efficacy of IVIG and/or MP for KD in vitro. METHODS: Human umbilical vein endothelial cells(HUVEC) obtained from umbilical cords of healthy newborns were cultured. After HUVEC were treated with IL-1beta, the effect of IVIG and/or MP on the in vitro activation of HUVEC were assessed by cell proliferation and reverse transcription-polymerase chain reaction-detected expression of mRNA coding for VEGF, VCAM-1, and IL-1beta. RESULTS: IVIG and MP down-regulated the expression of VEGF mRNA induced by IL-1beta(P<0.05, respectively) significantly. The combination of both showed a synergistic effect on the expressions with a dose dependent manner of MP compared to the IVIG or MP alone respectively(P<0.05). IVIG and MP down-regulated the expression of VCAM-1 mRNA induced by IL-1beta(P<0.05, respectively). The combination of both showed a synergistic effect on the expressions with a dose dependent manner of MP(P<0.05). IVIG and MP down-regulated the expression of IL-1beta mRNA induced by IL-1beta(P<0.001, P<0.05, respectively). The combination of both showed a synergistic effect on the expressions with a dose dependent manner of MP(P<0.001). CONCLUSION: These results suggested that IVIG and MP are therapeutically effective for KD in vitro as well as in vivo.
Cell Adhesion ; Cell Proliferation ; Child ; Clinical Coding ; Human Umbilical Vein Endothelial Cells ; Humans* ; Immunoglobulins, Intravenous ; Infant, Newborn ; Interleukin-1 ; Interleukin-1beta ; Methylprednisolone* ; Mucocutaneous Lymph Node Syndrome ; RNA, Messenger* ; Systemic Vasculitis ; Umbilical Cord ; Umbilical Veins* ; Vascular Cell Adhesion Molecule-1* ; Vascular Endothelial Growth Factor A*

PURPOSE: To assess CT findings of pulmonary sarcoidosis and correlate these with sACE level and PFT. MATERIALS AND METHODS: Between 1989 and 1995, 14 patients (4 men and 10 women, aged between 28 and 55 years) with histologically confirmed pulmonary sarcoidosis were consecutively selected. HRCT scans were performed in 12 patients and conventional CT scans in two. CT findings were reviewed by three radiologists, and were correlated with the index of disease activity based on sACE level and pulmonary function test. RESULTS: Pulmonary parenchymal abnormalities were seen in all patients ; small nodules of less than 3 mm in diameter were seen in eight. Other abnormalities were nodules of more than 3 mm in diameter (n=7), confluent nodules (n=5), ground glass opacity (n=5), patchy areas of consolidation with air bronchogram (n=5), and architectural distortion (n=3). The upper lung zone was more frequently involved than the middle or lower zone. In ten patients, the peripheral interstitum was predominantly involved, while only three patients showed predominant peribronchovascular involvement. Lymphadenopathy was noted in 13. There was no correlation between sACE level, the results of a pulmonary function test and the extent of parenchymal involvement. CONCLUSION: HRCT is valuable for the identification, characterization, and determination of the extent to which parenchymal lung is involved in sarcoidosis. The extent of this involvement does not correlate with sACE level and pulmonary function test results.
Female ; Glass ; Humans ; Lung ; Lymphatic Diseases ; Male ; Respiratory Function Tests ; Sarcoidosis ; Sarcoidosis, Pulmonary* ; Tomography, X-Ray Computed

Congenital hemolytic anemia is mainly developed due to intrinsic defects of erythrocytes, but in some cases the cause of hemolytic anemia is unclear. Gilbert's syndrome shows mild, chronic unconjugated hyperbilirubinemia that is due to reduced UDP glucuronosyltransferase (UGT-1A1) activity and this develops because of UGT-1A1 gene mutation. We report here on a case of severe hyperbilirubinemia in a 17-year-old male who was diagnosed with congenital hemolytic anemia of an unknown cause combined with Gilbert's syndrome.
Adolescent ; Anemia, Hemolytic ; Anemia, Hemolytic, Congenital ; Erythrocytes ; Gilbert Disease ; Glucuronosyltransferase ; Humans ; Hyperbilirubinemia ; Male

PURPOSE: To establish the incidence, type and significance of knee instability in patients with ipsilateral femoral and tibial shaft fracture, comparing with the patients with femoral shaft or tibial shaft fracture alone. MATERIALS AND METHODS: Two hundreds and seventy-nine consecutive patients were retrospectively reviewed from February 2000 to April 2004. They were composed of 80 patients with femoral shaft fracture alone, 176 patients with tibial shaft fracture alone and 23 patient with ipsilateral femoral and tibial shaft fracture. We evaluate the instability of knee based on physical examinations, plain stress films and MRI. We analyze incidence and period to diagnosis of instability, period to complete bony union and Hospital for Special Surgery (HSS) knee score respectively. RESULTS: There were 6.3% of knee instability in femoral shaft fracture alone, 9.7% in tibial shaft fracture alone and 30.4% in ipsilateral femoral and tibial shaft fracture. The average period to diagnosis of instability, average period to complete bony union and average HSS knee score were 9.2 months, 4.7 months and 65 points in femoral shaft fracture alone, 9.1 months, 4.2 months and 69 points in tibial shaft fracture alone, 8.7 months, 5.3 months (femur), 4.7 months (tibia) and 57 points in ipsilateral femoral and tibial shaft fracture respectively. CONCLUSION: We should consider MRI to evaluate the knee instability in patient with ipsilateral femoral and tibial shaft fracture at the time of injury and make a plan early about the treatment of knee instability.
Diagnosis ; Humans ; Incidence ; Knee* ; Magnetic Resonance Imaging ; Physical Examination ; Retrospective Studies

Small bowel volvulus in adults is very rare and occasionally occurs due to congenital midgut malrotation. The most common cause of small bowel volvulus is adhesions (74%), and other causes are Crohn's disease (7%), neoplasia (5%), hernia (2%), radiation (1%), and miscellaneous (11%). Presenting symptoms may be acute or present periodically during a longer period of time with a condition that is intermittent or recurrent because of spontaneous detorsion of the volvulus. Diagnostic imaging studies are plain abdominal film, ultrasonography, abdominal CT, and angiography. Abdominal CT is the most accurate. The most frequent CT finding is "Whirlpool sign." Other findings are bowel-loop dilatation, bowel-wall thickening, beak signs, mesenteric alterations, and extraluminal fluid. The mortality rate associated with small bowel volvulus in adults is 10~67%. We report a case of small bowel volvulus in adults.
Adult* ; Angiography ; Animals ; Beak ; Crohn Disease ; Diagnostic Imaging ; Dilatation ; Hernia ; Humans ; Intestinal Volvulus* ; Mortality ; Tomography, X-Ray Computed ; Ultrasonography

BACKGROUND AND OBJECTIVES: This was a multicenter study to evaluate the usefulness of the tumor necrosis factor-alpha (TNF-alpha) blocker infliximab for treatment of Korean pediatric patients with refractory Kawasaki disease (KD). SUBJECTS AND METHODS: Data from 16 patients throughout Korea who were diagnosed with refractory KD and received infliximab were collected retrospectively. RESULTS: Complete response to therapy with cessation of fever occurred in 13 of 16 patients. C-reactive protein (CRP) concentrations decreased following infliximab infusion in all 14 patients in whom it was measured before and after treatment. There were no infusion reactions or complications associated with infliximab except in 1 case with acute hepatitis occurring during treatment followed by calculous cholecystitis 4 months later. Fifteen patients had coronary artery (CA) abnormalities before infliximab therapy. Three had transient mild dilatation and 9 had CA aneurysms, with subsequent normalization in 4 patients, persistent mild dilatation in 3, persistent aneurysm in 2, and there were 3 cases (2 with CA aneurysm, 1 with mild CA dilatation) without follow-up echocardiography. CONCLUSION: The results of this study suggest that infliximab may be useful in the treatment of refractory KD, and it appears that there is no significant further progression of CA lesions developing after infliximab treatment. Multicenter trials with larger numbers of patients and long-term follow-up are necessary to assess the clinical efficacy and safety of infliximab in refractory KD.
Aneurysm ; Antibodies, Monoclonal ; C-Reactive Protein ; Child ; Cholecystitis ; Coronary Vessels ; Dilatation ; Echocardiography ; Fever ; Follow-Up Studies ; Hepatitis ; Humans ; Korea ; Mucocutaneous Lymph Node Syndrome ; Retrospective Studies ; Tumor Necrosis Factor-alpha ; Infliximab

In acute colonic pseudo-obstruction(Ogilvie's syndrome, 1948), there is no distal obstruction but colonic obstruction symptom and distended colon is shown radiologicaly and clinically. The etiology of this syndrome are complex of any medical and surgical problem. Elderly patients who are undergoing CAPD have multiple medical problems. But among them only one case which was diagnosed with this syndrome was reported in Korea. Neostigmine is unstable medicine due to muscarinic effects if neostigmine(anticholinesterase inhibitor) has side effects to the CAPD patients with multiple medical problems, it can be fatal. We use pyridostigmine, which has less muscarinic effect, and has similiar potency compared to neostigmine to acute colonic pseudo-obstruction, and thus achieved radiological improvement.
Aged ; Cholinergic Agents ; Colon* ; Colonic Pseudo-Obstruction ; Humans ; Korea ; Neostigmine ; Peritoneal Dialysis, Continuous Ambulatory* ; Pyridostigmine Bromide*

OBJECTIVE: Our purpose was to determine whether abnormal triple marker in the second trimester may be associated with adverse pregnancy outcomes. METHODS: Between November 1996 and April 1998, we evaluated 1,158 pregnant women undergoing second trimester triple marker screening tests who delivered at our hospital. The pregnancy outcomes of 48 women with false positive screens were compared with 1,158 screen negative controls. The pregnancy outcomes were obtained from hospital delivery records. RESULTS: Women with abnormal triple marker showed increased risks for low birth weight(p<0.01). But there was no significant differences between study and control groups with respect to preterm labor, pregnancy induced hypertension, oligohydroamnios, premature rupture of membrane, placenta previa, abruptio placenta, fetal death in utero. CONCLUSION: Abnormal triple marker in the second trimester was associated with low birth weight.
Female ; Fetal Death ; Humans ; Hypertension, Pregnancy-Induced ; Infant, Low Birth Weight ; Infant, Newborn ; Mass Screening* ; Membranes ; Obstetric Labor, Premature ; Parturition ; Placenta ; Placenta Previa ; Pregnancy ; Pregnancy Outcome ; Pregnancy Trimester, Second ; Pregnancy, High-Risk* ; Pregnant Women ; Rupture

Severe fever with thrombocytopenia syndrome (SFTS) is an emerging tick-borne disease caused by the newly discovered SFTS Bunyavirus, and there have been no case reports of SFTS patients presenting with hemophagocytic lymphohistiocytosis (HLH) in the English literature. We report a case of SFTS presenting with HLH in a 73-year-old immunocompetent male farmer. Although the patient had poor prognostic factors for SFTS, such as old age and central nervous system symptoms, he recovered fully with supportive care.
Aged ; Central Nervous System ; Farmers ; Fever* ; Humans ; Lymphohistiocytosis, Hemophagocytic* ; Male ; Orthobunyavirus ; Phlebovirus ; Thrombocytopenia* ; Tick-Borne Diseases

PURPOSE: The aim of this study was to determine the effect of alcohol on the expression of VEGF-A, PEDF, and VEGFR-2 in human osteoblasts. MATERIALS AND METHODS: Human osteoblasts primarily derived from the intertrochanteric region of the femur with osteonecrosis and fracture (control) were cultured with alcohol (0, 20, 100, 150 mM). The level of cell proliferation and the expression levels of VEGF-A mRNA, PEDF mRNA, and VEGFR-2 mRNA was evaluated according to the alcohol concentrations and the culture periods. RESULTS: Osteoblasts with the added alcohol showed an early increase in cell population, and a subsequent decrease or steady level thereafter compared with those without alcohol (p<0.05). The osteoblasts in the osteonecrosis group showed an increase in VEGF-A mRNA and PEDF mRNA expression at high alcohol concentrations (100, 150 mM), resulting in an decreased VEGF-A/PEDF ratio, while those in the control group showed an increase in VEGF-A mRNA expression and a decrease in PEDF mRNA expression, resulting in an increase in the VEGF-A/PEDF ratio (p<0.05). CONCLUSION: Alcohol stops the proliferation of osteoblasts and can cause an imbalance between VEGF-A and PEDF, thereby inhibiting the neovascularization of osteonecrosis.
Cell Proliferation ; Femur ; Head* ; Humans ; Necrosis* ; Osteoblasts* ; Osteonecrosis ; RNA, Messenger ; Vascular Endothelial Growth Factor A ; Vascular Endothelial Growth Factor Receptor-2*