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

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

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

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

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*

PURPOSE: There are concerns that administration of bisphosphonate (BP) can substantially suppress bone turnover, potentially interfering with fracture healing. We investigated the effects of preoperative BP administration before internal fixation of intertrochanteric femoral fractures using fracture healing and clinical outcomes. MATERIALS AND METHODS: We retrospectively analyzed data from 130 patients who underwent internal fixation for osteoporotic intertrochanteric femoral fractures between March 2012 and July 2016. Patients previously treated with BPs for at least 3 months (BP group; n=29) were compared with the remaining patients (BP-naïve group; n=101). Radiographs were used to assess and compare fracture healing 3 months and 1 year postsurgery. The primary clinical outcome measure assessed was change in Koval score. RESULTS: Fracture union at 3 months after surgery was verified in 72.4% of patients (21/29) in the BP group and 90.1% of patients (91/101) in the BP-naïve group (P=0.027). Fracture union at 1 year postsurgery (BP group, 93.1% [27/29] vs. BP-naïve group, 97.0% [98/101], P=0.310) and change in Koval score (1.1 vs. 1.0, P=0.694) were not significantly different between the groups. Multivariable logistic regression analysis revealed that a history of BP administration was associated with an increased risk of delayed union at 3 months postsurgery (P=0.014). CONCLUSION: Preoperative administration of BP was associated with a decreased fracture healing rate 3 months after internal fixation, compared with BP-naïve patients. Therefore, patients previously treated with a BP should be carefully allowed to wean off walking aids and transition to full weight-bearing in the early postoperative period.
Bone Remodeling ; Diphosphonates ; Femoral Fractures ; Fracture Healing ; Hip Fractures ; Humans ; Logistic Models ; Osteoporosis ; Outcome Assessment (Health Care) ; Postoperative Period ; Retrospective Studies ; Walking ; Weight-Bearing

Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is rare disease which is steroid-responsive and often associated with IgG4 related systemic disease such as autoimmune pancreatitis. It is characterized by increased serum IgG4 and IgG4-positive lymphoplasmacytic infiltration in bile ducts. It is often difficult to distinguish IgG4-SC to hilar cholangiocarcinoma if it manifests as an isolated bile duct. We report a case of 79-year-old woman with IgG4-SC which was difficult to distinguish hilar cholangiocarcinoma due to similar clinical and radiologic findings, showing good therapeutic effect after a 2-week steroid trial.
Aged ; Bile Ducts ; Cholangiocarcinoma* ; Cholangitis, Sclerosing* ; Female ; Humans ; Immunoglobulin G ; Immunoglobulins ; Klatskin Tumor ; Pancreatitis ; Rare Diseases