Infective endocarditis by Hemophilus species is very rare:there are only 22 reported-cases of Hemophilus paraphrophilus endocarditis. We report a case of Hemophilus paraphrophilus endocarditis in a middle-aged woman with cerebral embolism and hemorrhage.
Endocarditis* ; Female ; Haemophilus ; Haemophilus paraphrophilus* ; Hemorrhage* ; Humans ; Intracranial Embolism*

OBJECTIVE: The purpose of this study is to assess the clinical characteristics and the serial changes of high resolution CT (HRCT) findings and to correlate those with the results of clinical parameters in biopsy proven nonspecific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP) with connective tissue diseases (CTD). METHODS: Retrospective analysis was made of forty patients with CTD diagnosed of NSIP and UIP from a single tertiary hospital between January 1996 and February 2006. RESULTS: UIP was common in rheumatoid arthritis, systemic sclerosis and Sjogren's syndrome, while NSIP was frequent in polymyositis/dermatomyositis. No significant difference was found in the clinical characteristics of patients with NSIP and UIP. In initial HRCT findings, extents of honeycombing and reticulation pattern were significantly more in UIP-CTD than in NSIP-CTD. In bronchoalveolar lavage (BAL) results, proportion of alveolar macrophages was significantly higher in NSIP-CTD than in UIP-CTD. In NSIP-CTD, significant increment in the extent of reticulation and honeycombing was noted in the serial HRCT findings despite the aggressive treatment. Significant correlation was found between leukocytosis and honeycombing change in NSIP-CTD. Despite no significant difference of survival between two groups, patients with UIP-CTD seem to have a higher mortality than those with NSIP-CTD. CONCLUSION: It is suggested that chest HRCT and BAL fluid analysis may be helpful in the differential diagnosis of NSIP- and UIP-CTD and leukocytosis in initial blood test might be predictive of honeycombing progression in NSIP-CTD. Further study will be required to compare with the prognosis of NSIP- and UIP-CTD.
Arthritis, Rheumatoid ; Biopsy ; Bronchoalveolar Lavage ; Connective Tissue Diseases* ; Connective Tissue* ; Diagnosis, Differential ; Hematologic Tests ; Humans ; Idiopathic Pulmonary Fibrosis* ; Leukocytosis ; Lung Diseases, Interstitial ; Macrophages, Alveolar ; Mortality ; Prognosis ; Retrospective Studies ; Scleroderma, Systemic ; Sjogren's Syndrome ; Tertiary Care Centers ; Thorax ; Tomography, X-Ray Computed

Adult-onset Still's disease (AOSD) is a systemic inflammatory disorder with variable clinical features. The interleukin (IL)-1 receptor antagonist anakinra has been proposed as an alternative effective treatment in refractory AOSD. We report, for the first time in Korea, two cases of refractory AOSD in which anakinra treatment produced a clinical response. The first patient had frequent clinical flare-ups with fever, sore throat, myalgia, and pleuritic chest pain despite treatment with methotrexate and etanercept. In the second patient, treatments with various immunosuppressive agents failed to control the disease activity. Treatment with anakinra 100 mg/day was initiated in both cases. A complete clinical remission and improvement in the laboratory parameters were observed. The steroid dose was tapered without further clinical flare-ups. Anakinra appears to be an effective alternative treatment modality in patients with AOSD refractory to conventional disease-modifying anti-rheumatic drugs and corticosteroid therapy.
Antirheumatic Agents ; Chest Pain ; Fever ; Humans ; Immunoglobulin G ; Immunosuppressive Agents ; Interleukin 1 Receptor Antagonist Protein ; Interleukins ; Korea ; Methotrexate ; Pharyngitis ; Receptors, Tumor Necrosis Factor ; Still's Disease, Adult-Onset ; Etanercept

OBJECTIVES: Paraoxonase is a high density lipoprotein (HDL)-associated enzyme, which has been implicated in preventing low density lipoprotein-cholesterol (LDL-C) from oxidation. The human paraoxonase gene is codominantly expressed as allele A and B. The A allele codes for glutamine (A subtype) and the B allele for arginine (B subtype) at codon 192 of the paraoxonase enzyme. This genetic polymorphism divides the enzyme into high and low activity form. It has been believed that this difference of specific activity might change the metabolism of cholesterol and the prevalence of coronary artery disease. The present study investigated the association among the paraoxonase gene polymorphism and the level of plasma lipoprotein and coronary artery disease. METHODS: The 416 subjects who have undergone coronary angiography in SNUH were recruited. The patients (n=251) had >50% stenosis of at least one of the major coronary arteries. To identify the genotype of paraoxonase, we amplified the target region in the paraoxonase gene by PCR ( polymerase chain reaction) and electrophoresed the products. RESULTS: There was no difference between the two groups in the allele frequency (A : B = 0.41 : 0.59 in patients, A : B = 0.37 : 0.63 in controls; p=0.21) or in the genotype frequency (AA:AB:BB= 45:116:90 in patients, AA:AB:BB=22:77:66 in controls; p=0.41). There was no association of the paraoxonase genotype with serum lipoprotein level and acute coronary syndrome in this study. The B allele was not an independent risk factor for coronary artery disease in this study. CONCLUSION: The paraoxonase gene 192 polymorphism was not an independent risk factor for coronary artery disease in this study.
Acute Coronary Syndrome ; Alleles ; Arginine ; Aryldialkylphosphatase* ; Cholesterol ; Codon ; Constriction, Pathologic ; Coronary Angiography ; Coronary Artery Disease* ; Coronary Vessels* ; Gene Frequency ; Genotype ; Glutamine ; Humans ; Lipoproteins ; Metabolism ; Plasma ; Polymerase Chain Reaction ; Polymorphism, Genetic ; Prevalence ; Risk Factors

Adult-onset Still's disease (AOSD) is a systemic inflammatory disorder with variable clinical features. The interleukin (IL)-1 receptor antagonist anakinra has been proposed as an alternative effective treatment in refractory AOSD. We report, for the first time in Korea, two cases of refractory AOSD in which anakinra treatment produced a clinical response. The first patient had frequent clinical flare-ups with fever, sore throat, myalgia, and pleuritic chest pain despite treatment with methotrexate and etanercept. In the second patient, treatments with various immunosuppressive agents failed to control the disease activity. Treatment with anakinra 100 mg/day was initiated in both cases. A complete clinical remission and improvement in the laboratory parameters were observed. The steroid dose was tapered without further clinical flare-ups. Anakinra appears to be an effective alternative treatment modality in patients with AOSD refractory to conventional disease-modifying anti-rheumatic drugs and corticosteroid therapy.
Antirheumatic Agents ; Chest Pain ; Fever ; Humans ; Immunoglobulin G ; Immunosuppressive Agents ; Interleukin 1 Receptor Antagonist Protein ; Interleukins ; Korea ; Methotrexate ; Pharyngitis ; Receptors, Tumor Necrosis Factor ; Still's Disease, Adult-Onset ; Etanercept

PURPOSE: The aim of this study was to asses the fetal loss rate after mid-trimester amniocentesis. MATERIALS AND METHODS: This was a retrospective cohort study including singleton pregnant women who underwent mid-trimester amniocentesis at Cheil General Hospital from January 2008 through December 2010. The procedure-related fetal loss was defined as miscarriage within 2 weeks after amniocentesis. We evaluated the fetal loss rate within 2 weeks after amniocentesis and fetal loss rate before 24 gestational weeks. RESULTS: During the study period, a total of 4,356 singleton pregnant women underwent mid-trimester amniocentesis. A total of Five hundred ninety six women were excluded owing to follow up loss and termination of pregnancy due to abnormal karyotype or major anomaly. At our institute, the fetal loss rate within 2 weeks was 0.1% and before 24 gestational weeks was 0.3% after amniocentesis. CONCLUSION: The fetal loss rate after mid-trimester amniocentesis in our study is lower than previously reported rate. We suggest that amniocentesis is a safe procedure.
Abnormal Karyotype ; Abortion, Spontaneous ; Amniocentesis ; Cohort Studies ; Equidae ; Female ; Follow-Up Studies ; Hospitals, General ; Humans ; Pregnancy ; Pregnant Women ; Retrospective Studies

PURPOSE: Increased maternal age is a major risk factor for chromosomal abnormalities. The maternal age-specific risk of fetal trisomy was theoretically calculated. We investigated the actual frequency of fetal trisomy between 16 and 24 gestational weeks in pregnant women over the age of 34 at delivery. MATERIALS AND METHODS: We retrospectively, over a four-year period, reviewed the medical records of women with singleton pregnancies that started their antenatal care before the 10th week of pregnancy. Pregnant women aged 34 to 45 years at the time of delivery were enrolled and divided into groups of one-year intervals. We investigated the frequency of Down syndrome and all trisomies as a function of the maternal age and compared with the theoretical maternal-age-specific risk. RESULTS: Of the 5,858 pregnant women enrolled in the study, the rate of trisomy 21 was 0.29% (17 cases). The observed frequencies of trisomy 21 in women with maternal ages of 35 years and 40 years were 1:1,116 and 1:141, respectively. The rate of all trisomies was 0.39% (23 cases). The observed frequencies of all trisomies in women with maternal ages of 35 years and 40 years were 1:372 and 1:56, respectively. CONCLUSION: The frequencies of Down syndrome and all trisomies were proportional to the maternal age. However, the observed frequencies of Down syndrome and all trisomies between the 16 and 24 gestational weeks were lower than the theoretical rates.
Chromosome Aberrations ; Down Syndrome ; Epidemiology ; Female ; Humans ; Maternal Age ; Medical Records ; Pregnancy ; Pregnant Women* ; Retrospective Studies ; Risk Factors ; Trisomy*

BACKGROUND/AIMS: Tuberculous meningitis is a common, occasionally fatal infectious disease of the central nervous system. We evaluated the diagnostic validity of weighted diagnostic index scores (WDIS), which have been suggested to aid in the diagnosis of adult patients with tuberculous meningitis, on the basis of simple clinical and laboratory findings. METHODS: The microbiological and clinical data of adult patients with either tuberculous or bacterial meningitis were reviewed retrospectively. Diagnostic validity and WDIS cut-off values were evaluated by receiver operating characteristic (ROC) curve analysis. RESULTS: A total of 77 cases were included: 47 with tuberculous meningitis and 30 with bacterial meningitis. For the diagnosis of tuberculous meningitis, the sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of WDIS suggested by Thwaites (cut-off value of < or =4) were 100, 90, 94 and 100%, respectively. Based on ROC curve analysis (area under ROC curve=0.993), a cut-off value of < or =3 was most appropriate for the diagnosis of tuberculous meningitis. The sensitivity and specificity of WDIS with a cut-off value of < or =3 were 100 and 93.3%, respectively. CONCLUSIONS: Our study demonstrated that WDIS are highly predictable and acceptable for the early diagnosis of tuberculous meningitis in Korea, based on our analysis of clinical and laboratory findings. A cut-off value of < or =3 showed the best diagnostic validity.
Adult ; Central Nervous System ; Communicable Diseases ; Early Diagnosis ; Humans ; Korea ; Meningitis, Bacterial ; Retrospective Studies ; ROC Curve ; Sensitivity and Specificity ; Tuberculosis, Meningeal

Purpose: Real-world experience with tocilizumab in combination with dexamethasone in patients with severe coronavirus disease (COVID-19) needs to be investigated. Materials and Methods: A retrospective cohort study was conducted to evaluate the effect of severity-adjusted dosing of dexamethasone in combination with tocilizumab for severe COVID-19 from August 2020 to August 2021. The primary endpoint was 30-day clinical recovery, which was defined as no oxygen requirement or referral after recovery. Results: A total of 66 patients were evaluated, including 33 patients in the dexamethasone (Dexa) group and 33 patients in the dexamethasone plus tocilizumab (DexaToci) group. The DexaToci group showed a statistically significant benefit in 30-day clinical recovery, compared to the Dexa group (p=0.024). In multivariable analyses, peak FiO2 within 3 days and tocilizumab combination were consistently significant for 30-day recovery (all p<0.05). The DexaToci group showed a significantly steeper decrease in FiO2 (-4.2±2.6) than the Dexa group (−2.7±2.6; p=0.021) by hospital day 15. The duration of oxygen requirement was significantly shorter in the DexaToci group than the Dexa group (median, 10.0 days vs. 17.0 days; p=0.006). Infectious complications and cellular and humoral immune responses against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in the convalescence stage were not different between the two groups. Conclusion A combination of severity-adjusted dexamethasone and tocilizumab for the treatment of severe COVID-19 improved clinical recovery without increasing infectious complications or hindering the immune response against SARS-CoV-2.

PURPOSE: In Korea, pregnancy termination is frequently reported among women who took medications for an acute or chronic disease during pregnancy, for fear of teratogenic risk. We have previously shown that a service providing evidence-based information is helpful for women who week counseling to make a rational decision regarding their pregnancies. This study aimed to evaluate whether termination of pregnancy based on such perceptions, is justified using the 'DRug Exposure and risk Assessment in Moms' (DREAM) registry. METHODS: The study included 5,032 consenting pregnant women from the clinic and call center at the Korean Motherisk Program, from November 1999 to October 2008. The DREAM registry recorded the pregnancy outcomes (preterm birth, low birth weight, intrauterine fetal death, and congenital anomaly) of 3,328 women. RESULTS: Among women exposed to medications, time of exposure ranged from 3.5-4.6 weeks of gestation. There were 1,308 different drugs prescribed to these women. The drug most frequently prescribed was acetaminophen followed by chlorpheniramine maleate, and pseudoephedrine. There were 4.7% (n=156/3,328) women who underwent a voluntary abortion for fear of birth defects. We compared frequency of birth defects between exposed women and unexposed pregnant women in our institution during gestation. The frequency of major congenital malformations was 2.5% (n=74/2,977) in exposed group and 2.9% (n=75/2,573) in unexposed group (P=0.32). There was no statistically significant difference between exposed and control group in the rate of preterm births, intrauterine fetal death and low-birth weight babies. CONCLUSION: We did not observe increased risk of congenital malformations and adverse pregnancy outcomes in a population of pregnant women exposed to a variety of medications. Therefore these medications are not considered teratogen.
Acetaminophen ; Chlorpheniramine ; Chronic Disease ; Congenital Abnormalities ; Counseling ; Female ; Fetal Death ; Humans ; Infant, Low Birth Weight ; Infant, Newborn ; Korea ; Maleates ; Parturition ; Pregnancy ; Pregnancy Outcome ; Pregnant Women ; Premature Birth ; Pseudoephedrine ; Risk Assessment