No abstract available.
Blood Transfusion* ; Lymphocytes*

No abstract available.
Cervix Uteri* ; Female ; Health Expenditures*

Congenital obstuction of pulmonary vein without anomalous drainage can cause long-standing pulmonary congestion and pulmonary arterial hypertension, and it may include stenosis of individual pulmonary veinsor total pulmonary vein atresia. We reviewed seven cases of pulmonary vein obstruction, five of which accompanied other cardiac anomalies. Right pulmonary veins were involved in all seven cases including one bilateral case. Pulmonary veins were occluded totally in five and partially in three lungs. Pumonary catheterization and angiography were done for diagnosis. Chest radiographs of total occlusion cases showed decreased lung volume, features of pulmonary edema, interstitial lesions, and pleural thickening, which were quite specific, whereas pulmonry venous dilatation was dominant findings in partial obstruction cases. Pulmonary perfusion scans (n=3) showed total perfusion defects in the cases of total occlusion of veins. MR imaging (n=2) demonstrated total occlusion of pulmonary veins in the venoatrial junction in two, and membranous focal obstruction in one lung. Two patients had pneumonectomy and histological confirmation, Although catheterization and angiography are essential for the diagnosis, MR imaging is thought to be useful for the diagnosis of pulmonary vein obstruction.
Angiography ; Catheterization ; Catheters ; Constriction, Pathologic ; Diagnosis ; Dilatation ; Drainage ; Estrogens, Conjugated (USP) ; Humans ; Hypertension ; Lung ; Magnetic Resonance Imaging ; Perfusion ; Pneumonectomy ; Pulmonary Edema ; Pulmonary Veins* ; Radiography, Thoracic ; Veins

The Sturge Weber syndrome is an uncommon disorder characterized by port-wine nevus of the face with angiomatosis of the ipsilateral cerebral leptomeninges and extensive calcification in the underlying cerebral cortex. Associated with this syndrome are mental retardation, choroidal angioma, buphthalmus or glaucoma, seizure and hemiplegia. We studied a case of incomplete form of Sturage Weber syndrome in a 12 year-old boy, who had generalized seizure, a homonymous hemianopsia, intracranial calcification and leptomeningeal angiomatosis. There are no characteristic facial nevus, mental retardation, occular changes and hemiplegia.

OBJECTIVES: It is well known that pneumoconiotic patients experience impairments of macrophage function, as well as poor penetration of drugs into the fibrotic nodules and the immune system. Resultantly, pneumonia is frequently involved in pneumoconiotic patients and its treatment is not easy. Therefore, we conducted a clinical evaluation of immunoglobulin G which is known to be effective in severe infectious diseases. METHODS: We randomly selected 45 pneumoconiotic patients with pneumonia and classified them into 2 groups. The experimental group (IgG group) was scheduled to receive antibiotics and IgG (5 g I.V./day for 7 days). The control group was treated with antibiotics alone. Sputum gram stain (counts of WBCs and microorganisms), body temperature, arterial oxygen tension, and counts of peripheral venous blood leukocytes and band neutrophils were used as markers to assess the response effect therapy at time periods of 0, 2, 4, 6, and 8 days after completion of therapy. We compared the clinical scores between the two groups. RESULTS: The experimental IgG treated group was composed of 27 patients, and the control group comprised 18 patients. There was no statistical differences between two groups in terms of age, pneumoconiotic profusion, impairment degree of pulmonary function, or frequency of pathogen isolation in the sputum before medication. The experimental IgG treated group showed lower clinical scores as compared with the control group (p=0.083). CONCLUSIONS: These results suggest that IgG infusion with antibiotics will have an effect on pneumonia therapy in pneumoconiosis patients that are under 60 years and exhibit simple pneumoconiosis.
Anti-Bacterial Agents ; Body Temperature ; Communicable Diseases ; Humans* ; Immune System ; Immunoglobulin G* ; Immunoglobulins* ; Leukocytes ; Macrophages ; Neutrophils ; Oxygen ; Pneumoconiosis ; Pneumonia* ; Sputum

Localized granulomatosis with polyangiitis (loc-GPA) is a milder disease state of GPA restricted to the respiratory tract. Transition from localized form to systemic/generalized disease is predicted to occur in approximately 10% of the patients. We report an unusual case of loc-GPA involving multiple cranial nerves, which in 3 years progressed into systemic disease involving pituitary gland. Initially antineutrophil cytoplasmic antibody (ANCA) was negative, but as symptoms of diabetes insipidus started, ANCA became positive. Clinical course of ANCA negative loc-GPA should be carefully monitored for development of systemic disease. ANCA may be a useful marker for detecting transition from localized to systemic disease.
Antibodies, Antineutrophil Cytoplasmic ; Cranial Nerves ; Diabetes Insipidus ; Granulomatosis with Polyangiitis* ; Humans ; Pituitary Gland ; Respiratory System

Purpose: Transforming growth factor (TGF)-beta is a member of the superfamily of polypeptides, which control cell cycle progression and a variety of other cellular activities. TGF-beta1 has been implicated as an effector of the induction of apoptosis in response to 5alpha-reductase inhibitor (5ARI) and; therefore, causes a decrease in the prostate volume. We investigated the effect of 5ARI in the expression of TGF-beta1 in benign prostatic hyperplasia (BPH). Materials and Methods: 50 patients diagnosed with BPH were divided into two groups. The control group (n=30), in which a transurethral resection of the prostate (TURP) was performed without medication, and the 5ARI group (n=20), who were administrated with 5 mg of 5ARI daily for at least 3 months, followed by TURP. The resected specimens were stained with anti-rabbit TGF-beta1 polyclonal antibody using immunofluoroscent staining. The expression of TGF-beta1 was analyzed with a confocal laser scanning microscope and an image analyzer. The mRNA level of TGF-beta1 was determined by reverse transcriptase-polymerase chain reaction (RT-PCR). Results: There were no statistical differences in the patient characteristics, including age, serum prostate-specific antigen (PSA) level and prostate volume, between the two groups. The expression of TGF-beta1 was demonstrated in the luminal epithelium and smooth muscle cells in BPH. TGF-beta1 was more strongly expressed in the luminal epithelium of both groups, and in the 5ARI group than the control (p<0.001). Conclusions: These results suggest that 5ARI up-regulates the expression of TGF-beta1 in BPH patients, and may a play role as an inhibitor in the proliferation of BPH through the TGF-beta1 signal pathway.
Apoptosis ; Cell Cycle ; Epithelium ; Humans ; Myocytes, Smooth Muscle ; Peptides ; Phenobarbital ; Prostate ; Prostate-Specific Antigen ; Prostatic Hyperplasia* ; RNA, Messenger ; Signal Transduction ; Transforming Growth Factor beta1 ; Transforming Growth Factors ; Transurethral Resection of Prostate

No abstract available.
Hodgkin Disease*

Background/Aims: Veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is one of the most fatal complications of hematopoietic cell transplantation (HCT), and defibrotide is the only curative drug. We conducted this study to confirm the survival rate of VOD/SOS patients diagnosed in Korea and assess the efficacy of defibrotide. Methods: Patients diagnosed with VOD/SOS after allogenic HCT between 2003 and 2020 were enrolled. We investigated day +100 survival rates and associated risk factors in patients who satisfied the modified Seattle criteria within 50 days of HCT. Results: A total of 110 patients satisfied the modified Seattle criteria, of which 65.5% satisfied the Baltimore criteria. Thirty-seven patients were treated with defibrotide. The day +100 survival rate of the 110 patients was 65.3%. The survival rates in patients who did not meet the Baltimore criteria and in those who did were 86.8% and 53.7%, respectively (p = 0.001). The day +100 survival rate of patients treated with defibrotide was 50.5%. Among the patients receiving defibrotide, those whose creatinine levels were more than 1.2 times the baseline had a significantly lower survival rate at 26.7% (p = 0.014). On multivariate regression analysis, the hazard ratio of satisfaction of the Baltimore criteria was 4.54 (95% confidence interval [CI], 1.69 to 12.21; p = 0.003). In patients treated with defibrotide, the hazard ratio was 8.70 (95% CI, 2.26 to 33.45; p = 0.002), when creatinine was more than 1.2 times the baseline on administration. Conclusions The day +100 survival rate was significantly lower when the Baltimore criteria were satisfied, and when there was an increase in creatinine at the time of defibrotide administration.

Follicular helper T (TFH) cells are recently highlighted as their crucial role for humoral immunity to infection as well as their abnormal control to induce autoimmune disease. During an infection, naive T cells are differentiating into TFH cells which mediate memory B cells and long-lived plasma cells in germinal center (GC). TFH cells are characterized by their expression of master regulator, Bcl-6, and chemokine receptor, CXCR5, which are essential for the migration of T cells into the B cell follicle. Within the follicle, crosstalk occurs between B cells and TFH cells, leading to class switch recombination and affinity maturation. Various signaling molecules, including cytokines, surface molecules, and transcription factors are involved in TFH cell differentiation. IL-6 and IL-21 cytokine-mediated STAT signaling pathways, including STAT1 and STAT3, are crucial for inducing Bcl-6 expression and TFH cell differentiation. TFH cells express important surface molecules such as ICOS, PD-1, IL-21, BTLA, SAP and CD40L for mediating the interaction between T and B cells. Recently, two types of microRNA (miRNA) were found to be involved in the regulation of TFH cells. The miR-17-92 cluster induces Bcl-6 and TFH cell differentiation, whereas miR-10a negatively regulates Bcl-6 expression in T cells. In addition, follicular regulatory T (TFR) cells are studied as thymus-derived CXCR5+PD-1+Foxp3+ Treg cells that play a significant role in limiting the GC response. Regulation of TFH cell differentiation and the GC reaction via miRNA and TFR cells could be important regulatory mechanisms for maintaining immune tolerance and preventing autoimmune diseases such as systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA). Here, we review recent studies on the various factors that affect TFH cell differentiation, and the role of TFH cells in autoimmune diseases.
Arthritis, Rheumatoid ; Autoimmune Diseases ; Autoimmunity* ; B-Lymphocytes ; CD40 Ligand ; Cell Differentiation ; Cytokines ; Germinal Center ; Immune Tolerance ; Immunity, Humoral ; Interleukin-6 ; Lupus Erythematosus, Systemic ; Memory ; MicroRNAs ; Negotiating ; Plasma Cells ; Recombination, Genetic ; T-Lymphocytes ; T-Lymphocytes, Helper-Inducer* ; T-Lymphocytes, Regulatory ; Transcription Factors