Chronic granulomatous disease is a group of rare disorders characterized by a disease of oxidative metabolism of phagocytes with deficit of antimicrobial activity. Also, it has clinical features of chronic pyogenic infection, granuloma formation, hypergammaglobulinemia, anemia and leukocytosis.We experienced a case of chronic granulomatous disease with chronic recurrent infection, hypergammaglobulinemia and liver abscess. It was confirmed by modified NBT test. The result of patient's NBT test was less than 11%. The patient received subcutaneous injection of interferon-gamma, 200 million unit (0.1mg/m2 of body surface area per dose), on three consecutive days per week for 3 weeks and her symptom was improved. Then, we report a case of chronic granulomatous disease which is improved by interferon- therapy with brief review of literature.
Anemia ; Body Surface Area ; Granuloma ; Granulomatous Disease, Chronic* ; Humans ; Hypergammaglobulinemia ; Injections, Subcutaneous ; Interferon-gamma ; Liver Abscess ; Metabolism ; Phagocytes

A 67-year-old male patient was admitted with an abrupt sudden cardiac death. He represented with an extreme electrical storm of 30 times of ventricular fibrillation (VF) episodes on one day. External shocks were performed to terminate VF. Transient J-wave in the inferior-lateral leads and Brugada electrocardiography pattern on the right precordial leads appeared during the electrical storm. And J-wave disappeared after the termination of electrical storm. We report a case of the appearance of J-wave during electrical storm in a patient with Brugada syndrome.
Brugada Syndrome ; Death, Sudden, Cardiac ; Electrocardiography ; Humans ; Male ; Shock ; Ventricular Fibrillation

Behcet? disease is a recurring illness characterized by the triple symptom complex of aphthous stomatitis, genital ulceration, and uveitis. The disease is multisystemic disorder with involvement of skin, gastrointestinal tract, blood vessels, central nervous system, joints, and epididymis. Vascular involvement of Behcet? disease affects both arteries and veins and blood vessels of all sizes. Periosteal new bone formation is one feature of hypertrophic osteoarthropathy. Other features are clubbing and arthritis. Periosteal new bone formation on the bones of the lower extremities without other features of hypertrophic osteoarthropathy has been reported in patients with varicose veins, vitamin A intoxication, infantile cortical hyperostosis, tuberous sclerosis, congenital syphilis and chronic renal failure with hyperparathyroidism, and chronic venous insufficiency. However, periosteal new bone formation has not, to our knowledge, been described in patients with Behcet? disease yet although similar findings are occasionally noted in polyarteritis nodosa. We report a case of Behcet? disease complicated by leg swelling with periosteal new bone formation of right lower leg.
Arteries ; Arthritis ; Behcet Syndrome ; Blood Vessels ; Central Nervous System ; Epididymis ; Gastrointestinal Tract ; Humans ; Hyperostosis, Cortical, Congenital ; Hyperparathyroidism ; Joints ; Kidney Failure, Chronic ; Leg ; Lower Extremity ; Male ; Osteogenesis* ; Polyarteritis Nodosa ; Skin ; Stomatitis, Aphthous ; Syphilis, Congenital ; Tuberous Sclerosis ; Ulcer ; Uveitis ; Varicose Veins ; Veins ; Venous Insufficiency ; Vitamin A

PURPOSE: Matrix metalloproteinase(MMP)-9 is known to breakdown the blood-brain barrier by degrading the extracellular matrix of the subendothelial basement membrane in meningitis. Tissue inhibitor of metalloproteinase(TIMP)-1, a known inhibitor of MMP-9, has been postulated to inhibit the proteolytic activity of MMP-9 by bindng to MMP-9, but their interaction has not been fully understood yet. So far, there have been some reports on the relationship of MMP-9 and TIMP-1 in bacterial meningitis, but few reports in viral meningitis. Furthermore, there has been no report on this in Korea. We investigated the concentrations of MMP-9 and TIMP-1 in cerebrospinal fluid (CSF) and serum of patients with viral meningitis and control subjects, and evaluated their relationship with other clinical parameters of meningitis. METHODS: CSF and blood were obtained from 25 subjects with viral meningitis and 14 control subjects. After centrifugation, supernatants were stored at -20 degrees C and we assayed concentrations of MMP-9 and TIMP-1 by the sandwich ELISA method. RESULTS: Concentrations of CSF MMP-9 and TIMP-1 were significantly elevated in patients with viral meningitis, when compared with those in control subjects. Their serum levels showed no differences between the two groups. MMP-9 levels were closely correlated with TIMP-1 levels in the CSF(rs=0.42, P<0.05). CSF MMP-9/TIMP-1 ratios were significantly higher in patients with viral meningitis than those in the control subjects(P<0.05). Both CSF MMP-9 and TIMP-1 levels positively correlated with CSF total leukocyte counts(rs=0.43, P<0.05, rs=0.48, P<0.05). TIMP-1 levels positively correlated with total protein concentrations in the CSF(rs=0.43, P<0.05). CONCLUSION: MMP-9 and TIMP-1 may play an important role in the breakdown and maintenance of BBB in viral meningitis, respectively.
Basement Membrane ; Blood-Brain Barrier ; Centrifugation ; Cerebrospinal Fluid* ; Child* ; Enzyme-Linked Immunosorbent Assay ; Extracellular Matrix ; Humans ; Korea ; Leukocytes ; Matrix Metalloproteinase 9* ; Meningitis ; Meningitis, Aseptic* ; Meningitis, Bacterial ; Meningitis, Viral ; Tissue Inhibitor of Metalloproteinase-1*

PURPOSE: In this randomized, double-blind study, we investigated the analgesic efficacy and side effects of continuous constant-dose infusions of remifentanil after total abdominal hysterectomy and compared it to fentanyl. MATERIALS AND METHODS: Fifty-six adult female patients scheduled for elective total abdominal hysterectomy were enrolled in this study. Patients were randomly assigned to two groups according to fentanyl (group F, n=28) or remifentanil (group R, n=28) for postoperative analgesia. Patients in group F were given fentanyl intravenously with an infusion rate of fentanyl 0.5 microgram/kg/hr; group R was given remifentanil with an infusion rate of remifentanil 0.05 microgram/kg/min for 2 days. Pain intensity at rest, occurrence of postoperative nausea and vomiting (PONV), dizziness, pruritus, and respiratory depression were assessed 1 hr after arrival at the post-anesthesia care unit, at 6; 12; 24; and 48 hr post-operation and 6 hr post-infusion of the study drug. Pain was evaluated by using visual analogue scale (VAS; 0-10). The time that patients first requested analgesics was recorded as well as additional analgesics and antiemetics. RESULTS: There were no significant differences in VAS, time to first postoperative analgesics, and additional analgesics between the 2 groups. The incidences and severities of PONV and opioid related side effects were not different between the groups; however, there were 3 episodes (10.7%) of serious respiratory depression in group R. CONCLUSION: Continuous infusion technique of remifentanil did not reveal any benefits compared to fentanyl. Furthermore, it is not safe for postoperative analgesia in the general ward.
Adolescent ; Adult ; Aged ; Analgesics, Opioid/administration & dosage/therapeutic use ; Drug Administration Schedule ; Female ; Fentanyl/administration & dosage/*therapeutic use ; Humans ; Hysterectomy/*adverse effects ; Infusions, Intravenous ; Middle Aged ; Pain, Postoperative/*drug therapy/etiology ; Piperidines/administration & dosage/*therapeutic use ; Treatment Outcome

PURPOSE: Respiratory syncytial virus (RSV) is the most common etiologic agent of acute bronchiolitis in infancy, and has been regarded as a possible cause of hyperreactive airway disease later in childhood. This study was performed to compare the immunologic responses between infantile asthma and bronchiolitis and to evaluate whether these immunologic responses can be useful markers to predict the development of subsequent wheezing. METHODS: We analyzed the serum concentrations of interleukin-4 (IL-4) and soluble intercellular adhesion molecule-1 (sICAM-1) from 29 infantile asthmatics, 13 infants with bronchiolitis, and 19 non-atopic controls. RSV infection was confirmed with indirect immunofluorescent staining and viral culture. RESULTS: Serum concentrations of IL-4 and sICAM-1 were significantly higher in infants with asthma compared with infants with those of controls. Peripheral blood eosinophils were significantly decreased in RSV positive bronchiolitis, but there were no differences in the serum concentrations of IL-4 and sICAM-1 between the infants with and without RSV infection. Serum concentrations of IL-4 and sICAM-1 in the first wheezing episode were higher in infants with persistent wheezing than those without. CONCLUSION: There are significant differences in immunologic responses between infantile asthma and bronchiolitis, and the serum IL-4 and sICAM-1 levels in the first wheezing episode may be used as a marker to predict the development of subsequent wheezing.
Asthma* ; Bronchiolitis* ; Eosinophils ; Humans ; Infant* ; Intercellular Adhesion Molecule-1 ; Interleukin-4 ; Respiratory Sounds ; Respiratory Syncytial Viruses

Spontaneous coronary artery dissection is a rare cause of acute coronary syndrome and sudden cardiac death. The incidence, causes, pathogenesis, and treatment have not been defined clearly, but spontaneous coronary artery dissection should be considered in young patients without major cardiovascular risk factors or in patients in the peripartum period who present with acute coronary syndrome. The treatment is often challenging. Medical treatment is usually considered, and percutaneous coronary intervention or coronary artery bypass surgery may be possible in some patients. We herein report two cases of middle-aged males with myocardial infarction who were treated with percutaneous coronary angioplasty.
Adult ; Coronary Artery Disease/*complications/surgery/ultrasonography ; Humans ; Male ; Middle Aged ; Myocardial Infarction/*etiology/surgery/ultrasonography ; *Percutaneous Coronary Intervention ; Rupture, Spontaneous/surgery/ultrasonography ; Ultrasonography, Interventional

OBJECTIVE: To compare the advantages and disadvantages between total vaginal hysterectomy (VTH) and laparoscopically assisted vaginal hysterectomy (LAVH) including the indications and safety. METHODS: We reviewed the medical records of patients who underwent VTH from July 1998 to December 1999 and those who underwent LAVH from January 2000 to April 2002. We evaluated age, parity, previous abdominal operations, indications for hysterectomy, combined operations, operation time, bleeding amount, hemoglobin change, weight of uterus, and postoperative complications. RESULTS: 1. Age was not a notable factor but parity was significantly lower in LAVH group. 2. In VTH group, 48% of patients had previous operations compared with 46% in LAVH group. 3. The most common indication for hysterectomy of both group was uterine myoma. 4. The weight of hysterectomized specimen was 256 g in VTH group and 237 g in LAVH group. 5. In VTH group, 38% received concurrent surgical procedures of which colporrhaphy was the most common (14 cases). In LAVH group, 72.5% received concurrent surgical procedures of which salpingoo- phorectomy was most common. 6. The operation time showed a notable difference; 78.6 min. in VTH group and 105.4 min. in LAVH. 7. There was no significant difference in bleeding amount and hemoglobin change. 8. Postoperative complication was higher in VTH group (14%) than LAVH group (7.5%). However all the patients recovered with conservative treatment and close observation. CONCLUSION: Both VTH and LAVH had the following advantages compared with abdominal hysterectomy: less pain, shorter hospital stay, cosmetic advantages, lower prevalence. In this study we found out that in VTH, the procedure could be done safely even if the uterus was big or with previous abdominal operations. Limited operation field and the fact that we couldn't check the abdominal cavity were some disadvantages. In comparison, LAVH offered a view of the abdominal cavity which make easy adnexal operation but because of expensive operative tools, cost was a problem. In order to satisfy the patient and lower the cost, appropriate study on the indications and training on procedures will be necessary.
Abdominal Cavity ; Bleeding Time ; Female ; Hemorrhage ; Humans ; Hysterectomy ; Hysterectomy, Vaginal ; Leiomyoma ; Length of Stay ; Medical Records ; Parity ; Postoperative Complications ; Prevalence ; Uterine Prolapse* ; Uterus

PURPOSE: The pathogenesis of atopic dermatitis is not clearly defined yet, but the pathogenetic role of Th2 cells has been supposed. CD30 is a membrane-bound glycoprotein that may be expressed on activated T cells with a sustained expression in Th2 cells and can be released as a soluble form(sCD30). This study was done to document the changes of serum sCD30 and it's clinical significance in atopic dermatitis. METHODS: We analyzed serum sCD30, serum soluble IL-2 receptor (sIL-2R), total serum IgE and total eosinophil counts from 18 children with atopic dermatitis(AD), 15 atopic asthmatics without AD (AA), 15 atopic asthmatics with AD(AD+AA), and 14 healthy non atopics(control). We investigated the correlation of serum sCD30 levels with disease severity assessed by clinical scoring(SCORAD index) in the group of AD and AD+AA. RESULTS: The serum levels of sCD30 were significantly higher in the group of AD and AD+AA than the group of AA and control. There were no differences in serum sCD30 levels between the group of AA and control and between the group of AD and AD+AA. The serum sIL-2R levels showed no significant differences among the four groups. There was significant positive correlation between serum sCD30 and serum sIL-2R levels(P<0.05). Both serum sCD30 and serum sIL-2R levels showed no correlation with total serum IgE, total eosinophil counts, and disease severity, respectively. CONCLUSION: Serum sCD30 is elevated only in atopic dermatitis irrespective of presence of asthma. The results suggest that Th2 immune responses may involved the pathogenesis of atopic dermatitis and sCD30 may be the possible marker of atopic dermatitis.
Asthma ; Child* ; Dermatitis, Atopic* ; Eosinophils ; Glycoproteins ; Humans ; Immunoglobulin E ; Receptors, Interleukin-2 ; T-Lymphocytes ; Th2 Cells

OBJECTIVE: To compare cervical dilatation efficacy and safety of intravaginal misoprostol with that of cervical laminaria tent insertion in endometrial curettage due to abnormal uterine bleeding METHOD: Patients requesting endometrial curettage due to abnormal uterine bleeding were randomized into two group. In group I (n=53), 100 g tablet of misoprostol was placed in the posterior vaginal fornix at midnight. In Group II (n=54), laminaria tent number 5 was inserted in cervical canal at midnight. Endometrial curettage was performed on the next morning. The degrees of cervical dilatation were measured by Hegar dilator just before endometrial curettage. Both groups were compared about cervical dilatation efficacy and side effect. RESULT: The degrees of cervical dilatation were 8.8+/-1.1 mm in group I and 9.3+/-1.9 mm in group II. There was no significant statistical difference. (P=0.134) The mean intervals from the use of misoprostol or laminaria to endometrial curettage were 704+/-140 minutes in group I and 829+/-156 in group II. In group I, mild fever (less than 38 degrees) was observed in four women and one complained mild abdominal pain. In group II, five women revealed mild fever and fifty women complained abdominal discomfort. All didn't need any medication or special therapy. CONCLUSION: Intravaginal misoprostol was appeared as efficacious as laminaria tent in cervical dilatatory effect before endometrial curettage in abnormal uterine bleeding patients. It was considered more convenient, safer and more cost effective method compared than cervical laminaria tent in cervical preparation before endometrial curettage.
Abdominal Pain ; Curettage* ; Female ; Fever ; Humans ; Labor Stage, First ; Laminaria* ; Misoprostol* ; Pregnancy ; Uterine Hemorrhage*