• Clinical and Experimental Pediatrics
• /
• v.50 no.12
• /
• pp.1180-1187
• /
• 2007

Systemic lupus erythematosus (SLE) is an episodic, multi-system, autoimmune disease characterized by widespread inflammation of blood vessels and connective tissues and by the presence of antinuclear antibodies (ANAs), especially antibodies to native (double-stranded) DNA (dsDNA). Its clinical manifestations are extremely variable, and its natural history is unpredictable. Untreated, SLE is often progressive and has a significant fatality rate. The most widely used criteria for the classification of SLE are those of the American College of Rheumatology (ACR), which were revised in 1982 and modified in 1997. The presence of four criteria have been diagnosed as a SLE. Rashes are common at onset and during active disease. The oral mucosa is the site of ulceration with SLE. Arthralgia and arthritis affect most children and these symptoms are short in duration and can be migratory. Lupus nephritis may be more frequent and of greater severity in children than in adults. The initial manifestation of nephritis is microscopic hematuria, followed by proteinuria. The most common neuropsychiatric symptoms are depression, psychosis(hallucination and paranoia) and headache. CNS disease is a major cause of morbidity and mortality. Pericarditis is the most common cardiac manifestation. Libman-Sacks endocarditis is less common in children. The most frequently described pleuropulmonary manifestations are pleural effusions, pleuritis, pneunonitis and pulmonary hemorrhage. During the active phase ESR, CRP, gamma globulin, ferritin and anti-dsDNA are elevated. Antibodies to dsDNA occur in children with active nephritis. Antibodies to the extractable nuclear antigens (Sm, Ro/SS-A, La/SS-B) are strongly associated with SLE. Specific treatment should be individualized and based on the severity of the disease. Sepsis has replaced renal failure as the most common cause of death.

• Tuberculosis and Respiratory Diseases
• /
• v.69 no.5
• /
• pp.375-380
• /
• 2010

Lymphoid interstitial pneumonia (LIP) is a rare benign lymphoproliferative interstitial lung disease. LIP has been associated with autoimmune disorders, HIV, viral infections, and so on. Once underlying systemic diseases have been excluded, a diagnosis of idiopathic LIP can be made. Although 6 cases of pathologically confirmed LIP have occurred in Korea, thus far none has been associated with primary Sjogren's syndrome. A 44-year-old man was admitted to hospital due to a dry cough and dypsnea on exertion that had been ongoing for 2 months. A chest radiography showed multiple and variable-sized cystic lesions, on both lungs and both interstitial infiltration and consolidation in both lower lung fields. Tests for autoantibody showed positive results of anti-nuclear antibody and anti-Ro/La antibody. The patient underwent a video assisted thoracoscopic surgery biopsy and pathologically confirmed LIP. We report the first known case of LIP-associated with primary Sjogren's syndrome in Korea.

• Tuberculosis and Respiratory Diseases
• /
• v.43 no.5
• /
• pp.786-791
• /
• 1996

Systemic sclerosis is a multisystemic disease of unknown origin charicterized by degenerative fibrotic and inflammatory changes in the skin, vessels, joints, muscles, and visceral organs. Involvement of the lung in systemic sclerosis is common, but pleural effusion is rare. Although vasculitis commonly accompanies many connective tissue disorders, it has been rarely reported in systemic sclerosis. A 43-year-old woman, with a 10-year history of Raynaud's phenomenon, was admitted due to right chest pain. Her hands showed diffuse thickening and swelling of skin. Chest X-ray showed pleural effusions and esophageal manometry showed hypotonic peristalsis and low lower esophageal sphincter tone compatible with scleroderma esophagus. Antinuclear antibodies were present (titer>1 : 160) with a speckled pattern. She was positive for rheumatoid factor, anti scl-70 and RNP antibodies, but negative for anti-Ro, La, and Sm antibodies. Histology of the pleura revealed the presence of leukocytoclastic vasculiti. After adminisrration of prednisolone 30 mg/day, her chest symptom was improved. We report a case of systemic sclerosis with pleural effusions due to leukocytoclastic vasculitis with review of the literatures.

• Tuberculosis and Respiratory Diseases
• /
• v.64 no.3
• /
• pp.230-235
• /
• 2008

We described herein the first case of primary $Sj{\ddot{o}}gren^{\prime}s$ syndrome in Korea which presented with multiple cysts caused by only peribronchiolar lymphocytic infiltration, a rare pulmonary manifestation in $Sj{\ddot{o}}gren^{\prime}s$ syndrome, and was confirmed by surgical lung biopsy. A brief review of the literature has been included.

• Korean Journal of Food Science and Technology
• /
• v.53 no.4
• /
• pp.463-469
• /
• 2021

Bioactivities of curcumin, a major pigment of Curcuma longa L., have been widely investigated. In this study, the antioxidant and cytotoxic properties of curcumin and its analogs including ferulic acid, dibenzoylmethane (DBM), and tetrahydrocurcumin (THC), and their structure-activity relationships were assessed. Ferulic acid, THC, and curcumin showed strong scavenging activities against several radicals and exhibited considerable lipid peroxidation inhibitory activity. Curcumin showed the strongest cytotoxic activities against HeLa, HCT-116, IEC-6 and INT 407 cells, whereas ferulic acid did not show any cytotoxic effect up to 100 µM against these cell lines. Cytotoxicity of curcumin and THC was significantly enhanced by superoxide dismutase and diminished by N-acetylcysteine. The combination treatment of curcumin and ferulic acid enhanced the cytotoxicity, whereas the combination of curcumin and DBM offset their toxicity. These results suggest that methoxy phenolic and β-diketon moieties are crucial for the antioxidant- and cytototoxic activities of curcumin, respectively.

• Kosin Medical Journal
• /
• v.33 no.3
• /
• pp.307-317
• /
• 2018

Background: Detection of antibodies to extractable nuclear antigens (ENAs) is needed for the diagnosis in systemic autoimmune diseases. In this study, we compared three reagents using line immunoblot assay (LIA) or multiplex bead immunoassay for detecting the anti-ENAs. Methods: A total of 89 sera were tested by 3 different assays: EUROASSAY Anti-ENA Profile (Euroimmune, Germany), Polycheck Autoimmune Test (Biocheck GmbH, Germany), and $FIDIS^{TM}$ Connective Profile (Biomedical Diagnostics, France). The following individual ENAs were investigated: Sm, SS-A (Ro), SS-B (La), Scl-70, Jo-1 and RNP. We reviewed medical records to investigate the discrepant results among three methods. Results: Overall percent agreements were 96.1% between EUROASSAY Anti-ENA Profile and $FIDIS^{TM}$ Connective profile; 90.4% between EUROASSAY Anti-ENA Profile and Polycheck Autoimmune Test using the manufacturers' cutoff; 96.4% between EUROASSAY Anti-ENA Profile and Polycheck Autoimmune Test using a upward cutoff; 90.4% between $FIDIS^{TM}$ Connective profile and Polycheck Autoimmune Test the manufacturers' cutoff; and 96.4% between $FIDIS^{TM}$ Connective profile and Polycheck Autoimmune Test a upward cutoff. Conclusions: The three assays showed excellent agreement with each other. With appropriate cutoff, the all three assays for six of the anti-ENA tests investigated in this study can be used in clinical laboratories for detecting the anti-ENAs.

• Tuberculosis and Respiratory Diseases
• /
• v.52 no.2
• /
• pp.179-185
• /
• 2002

A bronchus-associated lymphoid tissue(BALT) lymphoma of the lung is a rare disorder of patients with Sj$\ddot{o}$gren's syndrome. A 49-year-old woman was admitted for an evaluation of exertional dyspnea and general weakness which had persisted for two years. The patient had suffered from dry mouth and dry eyes for five years. The physical examinations showed a coarse breath sound with inspiratory crackles on the whole lung field, particularly on the both basal lungs. The laboratory data disclosed high titers of anti-nuclear antibodies, and anti-SSA (Ro), and anti-SSB (La) antibodies. Chest radiographs demonstrated the presence of bilateral, diffuse, reticulonodular densities in both lungs. Thin-section CT scans showed diffusely distributed mosaic pattern of an inhomogeneous attenuation extending over the entire lung zone. The histological findings from an open-lung biopsy specimen revealed an accumulation of lymphoid cells around the bronchioles and an extension of malignant lymphoma cells from the bronchiolar epithelium toward the alveolar space. Immunohistochemically, the neoplastic cells reacted positively to the CD 20 antigen and were focally positive for the UCHL 1 antigen. The histological diagnosis was consistent with a low grade marginal zone B-cell lymphoma originating in the BALT. Here, we present a case of a histologically proven BALT lymphoma of the lung in a patient with primary Sj$\ddot{o}$gren's Syndrome.

• The Korean Journal of Nuclear Medicine
• /
• v.33 no.6
• /
• pp.527-536
• /
• 1999

Purpose: Radiotracers that bind to the central benzodiazepine receptor are useful for the investigation of various neurological and psychiatric diseases. [C-11]Flumazenil, a benzodiazepine antagonist, is the most widely used radioligand for central benzodiazepine receptor imaging by PET. We synthesized 3-(2-[F-18]fluoro)flumazenil, a new fluorine-18 ($t_{1/2}$= 110 min) labeled analogue of benzodiazepine receptor imaging agent, and evaluated in vivo for biodistribution in mice. Materials and Methods: Flumazenil (Ro 15-1788) was synthesized by a modification of the reported method. Precursor of 3-(2-[F-18]fluoro)flumazenil, the tosylated flumazenil derivative was prepared by the tosylation of the ethyl ester by ditosylethane. [F-18] labeling of tosyl substitued flumazenil precursor was performed by adding F-18 ion at $85^{\circ}C$ in the hot ceil for 20 min. The reaction mixture was trapped by C18 cartridge, washed with 10% ethanol, and eluted by 40% ethanol. Bidistribution in mice was determined after intravenous injection. Results: The total chemical yield of tosylated flumazenil derivative was ${\sim}40%$. The efficiency of labeling 3-(2-[F-18]fluoro)flumazenil was 66% with a total synthesis time of 50 min. Brain uptakes of 3-(2-[F-18]fluoro)flumazenil at 10, 30, 60 min after injection, were $2.5{\pm}0.37,\;2.2{\pm}0.26,\;2.1{\pm}0.11$ and blood activities were $3.7{\pm}0.43,\;3.3{\pm}0.07,\;3.3{\pm}0.09%ID/g$, respectively. Conclusion: We synthesized a tosylated flumazenil derivative which was successfully labeled with no-carrier-added F-18 by nucleophilic substitution.

• Clinical and Experimental Pediatrics
• /
• v.49 no.4
• /
• pp.381-387
• /
• 2006

Purpose : Neonatal lupus is characterized by congenital complete heart block(CCHB), cutaneous rash, and laboratory abnormalities in infants born to mothers with systemic lupus erythematosus(SLE). This study aims to examine the incidence of CCHB and clinical outcome in neonates born to mothers with SLE. Methods : The study group consisted of 49 neonates, born from 57 pregnancies of 55 women with SLE, diagnosed at Hanyang University Hospital for the period between January 1997 and January 2005. Clinical and laboratory data were retrospectively identified from medical record. Results : There were 5(8.8 percent) spontaneous abortions and one(1.8 percent) still births among 57 pregnancies of 55 mothers. Of 49 live births, 15(26.3 percent) were premature and eight(12.3 percent) were small for their gestational age. There was one(1.8 percent) CCHB suspected during pregnancy on fetal echocardiograpy in a fetus of mother with systemic lupus erythematosus and the fetus was not born by artificial abortion because of mother. There was no CCHB among EKG findings of 49 newborns. Laboratory testing showed hematologic abnormalities among 25.6 percent(10/39) of the babies. 5.1 percent(2/39) and 7.7 percent(3/39) of them were diagnosed as neutropenia, and thrombocytopenia was seen respectively. Anti-SSA(Ro) and antiphospholipid antibodies were predictive factors for prematurity(P=0.003, P=0.049). Anticardiolipin antibodies were predictive factors for ventilatory care(P=0.018). Conclusion : The incidence of CCHB among neonates born to mothers with SLE, which was measured in this study, was lower than that in earlier studies. A high incidence of hematologic abnormalities was found in our study. It is suggested that careful examination should be made of skin for the diagnosis of neonatal lupus.

• Journal of the Korean Applied Science and Technology
• /
• v.31 no.4
• /
• pp.719-730
• /
• 2014

The hot water extract from Artemisia capillaris fermented with Hericium erinaceum mycelium (AC-HE) were assessed for the protection against oxidative modification of biological macromolecules and cell death. Antioxidant activity of AC-HE evaluated using 2,2-diphenyl-1-picrylhydrazyl radical, 2,2'-azino-bis(3-ethylbenzothiazoline-6-sulfonic acid) radical and peroxyl radical scavenging assays. AC-HE showed 61.73% DPPH radical scavenging activity at $500{\mu}g/mL$, 97.39% ABTS radical scavenging activity at $250{\mu}g/mL$, and 44.18% peroxyl radical scavenging activity at $100{\mu}g/mL$. AC-HE were shown to significantly inhibited DNA strand breakage induced by peroxyl radical. AC-HE also prevented peroxyl radical-mediated human serum albumin modification. AC-HE effectively inhibited $H_2O_2$ induced cell death and significantly increased of the 11.47% cell survival at $100{\mu}g/mL$. AC-HE also decreased intracellular reactive oxygen species (ROS) levels in $H_2O_2$-treated cells. The results suggested that AC-HE can contribute to antioxidant and protected cells from oxidative stress-induced cell injury.