No abstract available.
Humans ; Mucocutaneous Lymph Node Syndrome* ; Onycholysis*

Background: This study was designed to determine whether presynaptic receptor blockade could be differentiated from postsynaptic receptor blockade by examining the effect of increasing frequencies of indirect stimulation on partial twitch depression in vitro rat phrenic nerve hemidiaphragm preparations. Methods: After isolating rat phrenic nerve hemidiaphragm preparation, T200/T1 ratio (twitch height of the 200th stimuli divided by that of the 1st stimuli) at frequencies of 0.2, 0.5, 1.0, and 2.0 Hz using a drug concentration which provided approximately 20% twitch depression at 0.1 Hz was calculated. To compare T200/T1 ratios with TOF ratios, 2.0 Hz TOF response was measured immediately after 200th stimuli at either frequency of stimulation. Results: Hexamethonium caused a marked decrease in T200/T1 ratio at 0.5~2.0 Hz of stimulation, whereas alpha-bungarotoxin caused no change in T200/T1 ratios at up to 2.0 Hz of stimulation. The T200/T1 ratios produced by d-tubocurarine, vecuronium, mivacurium, and rocuronium located intermediate between alpha-bungarotoxin and hexamethonium, however significant differences among four drugs were found at 2.0 Hz. The propensity for decrease in T200/T1 ratios at 2.0 Hz might differ from this study: hexamethonium >d-tubocurarine >rocuronium >mivacurium = vecuronium >alpha-bungarotoxin. T200/T1 ratios at 2.0 Hz were not different from TOF ratios. Conclusions: When the observed effects in this study were provided with result of alpha-bungarotoxin acting predominantly at postsynaptic receptors and hexamethonium acting predominantly at presynaptic receptors, the effects of nondepolarizing muscle relaxants at each binding site could be differentiated by examining the T200/T1 ratios at 2.0 Hz.
Animals ; Binding Sites ; Bungarotoxins ; Depression* ; Hexamethonium ; Phrenic Nerve* ; Rats* ; Receptors, Presynaptic ; Tubocurarine ; Vecuronium Bromide

Ganglioneuroma is a rare benign tumor which originates in the neural crest, and is found along the path of the sympathetic chain, from the base of the skull to the pelvic cavity. Due to the slow growth of this type of tumor, it may be detected incidentally, or detected by virtue of the attendant pressure effects on adjacent structures. We report one case of ganglioneuroma arising in the pelvic cavity.
Ganglioneuroma ; Neural Crest ; Skull ; Virtues

PURPOSE: We report an unusual case of Leber hereditary optic neuropathy presenting with optic disc hyperfluorescence. CASE SUMMARY: A 17-year-old male with sequential painless visual loss 3 weeks apart affecting first the left and then the right eye presented to our neuro-ophthalmology clinic. His best-corrected visual acuity was counting fingers in the right eye and 0.32 in the left eye. Fundus examination showed mild optic disc edema and hyperemia in both eyes, which were worse in the right eye. Fluorescein angiography revealed dye leakage from the right optic disc in the late phase. The results of magnetic resonance imaging of the brain and spinal cord were normal, and lumbar puncture study was unremarkable. Mitochondrial DNA sequencing revealed a pathognomonic 11778 mutation for Leber hereditary optic neuropathy. His vision deteriorated to 0.03 in both eyes 6 months later, but slowly started to improve 11 months after onset. At 2 years, his corrected visual acuity was 0.2 in both eyes. CONCLUSIONS: To our knowledge, this is the first report of optic disc hyperfluorescence in Leber hereditary optic neuropathy. This finding suggests that this mitochondrial optic neuropathy can masquerade as optic neuritis.
Adolescent ; Brain ; DNA, Mitochondrial ; Edema ; Fingers ; Fluorescein Angiography ; Humans ; Hyperemia ; Magnetic Resonance Imaging ; Male ; Optic Atrophy ; Optic Atrophy, Hereditary, Leber ; Optic Nerve Diseases ; Optic Neuritis ; Spinal Cord ; Spinal Puncture ; Visual Acuity

BACKGROUND AND OBJECTIVES: Extracellular uridine 5'-triphosphate (UTP) regulates a variety of biological functions in the airway epithelium including chloride and fluid transport, mucociliary clearance, and mucin secretion via P2Y purinergic receptors. This study was undertaken to investigate which P2Y purinergic receptors are expressed in the normal human middle ear epithelial (NHMEE) cells. We also determined the levels of mucin secretion and its mRNA expressions following stimulation with UTP. MATERIALS AND METHOD: The level of P2Y (P2Y1, P2Y2, P2Y4, P2Y6, P2Y11 and P2Y12) receptors and mucin gene 5AC (MUC5AC), MUC5B, MUC8 messenger RNA (mRNA)s were measured by reverse transcription (RT)-polymerase chain reaction (PCR). We also determined the levels of mucin secretion following stimulation with UTP by dot-blotting method in NHMEE cells. RESULTS: Middle ear epithelial cells expressed P2Y1, P2Y2, P2Y6, P2Y11 and P2Y12 receptors but not the P2Y4 receptor. Apically applied UTP induced increased the mucin secretion. On the other hand, UTP did not enhance the mucin mRNA expression until 72 h had lapsed after treatment. CONCLUSION: Our study suggests that UTP acts as a secretogogue on mucin secretion in NHMEE cells.
Ear ; Ear, Middle* ; Epithelial Cells* ; Epithelium ; Hand ; Humans* ; Mucins* ; Mucociliary Clearance ; Mucous Membrane ; Receptors, Purinergic ; Reverse Transcription ; RNA, Messenger ; Uridine Triphosphate ; Uridine*

Phase-partitioning with Triton X-114 (TX114) was applied to the TSP antigen, which may be preferentially associated with the cell wall of M. tuberculosis. The hydrophilic protein components of the TSP antigen were successfully separated from integral hydrophobic macromolecules. To further characterize and examine the cellular immune response of the aqueous fraction of the TSP antigen (TSPa), the in vitro properties of the antigen were measured by lymphoproliferation; surface expression of IL-2 Ra on T lymphocytes was analyzed by flow cytometry; and the cytokine mRNA expression pattern was determined by RT-PCR. Significant lymphoproliferative responses to the TSPa antigen were observed in healthy tuberculin reactive donors after a 5 day in vitro stimulation. TSPa treatment of PBMCs from healthy tuberculin positive subjects for 5 days resulted in progressive augmentation of IFN-r, II 2, and IL-2Ra mRNA expression, as measured by RT-PCR, but considerably reduced IL-4 mRNA expression. In addition, the TSPa antigen stimulated more IL-12 p40 mRNA production than did the PPD antigen, and graduaBy suppressed IL- 10 mRNA expression. Moreover, the CD3' T cells of tuberculin positive subjects displayed a profound increase in their expression of the II 2Ru protein (39.0%) in response to the TSPa antigen. Proliferation was correlated with IL-2 and IL-2Ra mRNAs, but not correlated with distinct IFN-r or IL-12 p40 mRNA production. These findings strongly suggest that the TSPa antigen preferentially evokes the generation of a Thl-like immune response in healthy tuberculin reactors.
Cell Wall ; Flow Cytometry ; Humans* ; Immunity, Cellular* ; Interleukin-12 ; Interleukin-2 ; Interleukin-4 ; Mycobacterium tuberculosis* ; Mycobacterium* ; Neptune ; RNA, Messenger ; T-Lymphocytes ; Tissue Donors ; Tuberculin ; Tuberculosis

Today, the duodenal tuberculosis is a very rare disease. Final diagnosis of duodenal tuberculosis is difficult due to inconsistent symptoms and nonspecific duodenal lesions. We found 2 cases of duodenal tuberculosis by gastrascopy. In gastroscopic findings, there were undetermined ulcers and irregular nodular mucosal changes.
Diagnosis ; Rare Diseases ; Tuberculosis* ; Ulcer

BACKGROUND: In patients with mitral stenosis, the degree of pulmonary hypertension is expected to be related to the severity of mitral valve obstruction. However, some patients with severe mitral stenosis do not develop reactive pulmonary hypertension. MATERIALS AND METHODS: We evaluated 34 patients with symptomatic mitral stenosis undergoing percutaneous mitral valvuloplasty by clinical, echocardiographic, and invasive hemodynamic(cardiac cathrterization) data. Prevalvuloplasty data were available in 34 subjects[mean age 38+/-9 year ; women 74% ; NYHA class 1 (6 patients), class 2 (17 patients), class 3 (7 patients), class 4 (4 patients) ; in electrocardiography, NSR(23 patients), Atrial fibrillation(11 patients)]. RESULTS: 1) The pulmonary vascular bed gradient was significantly correlated with pulmonary vascular resistance(r=0.91), mean pulmonary artery pressure(r=0.82), transmitral mean pressure gradient(r=0.64) and mitral valve area(r=-0.48). The pulmonary vascular resistance was significantly correlated with mena pulmonary artery pressure(r=0.77), transmiral mean pressure gradient(r=0.61) and mitral valve area(r=-0.54), NYHA functional classification(r=0.36). However, the pulmonary vascular bed gradient and pulmonary vascular resistance was not significantly correlated with age, sex, cardiac output, the severity of mitral regurgitation and mean left atrial pressure. 2) The mean pulmonary artery pressure was significantly correlated with mean left atrial pressure(r=0.80), transmitral mean pressure gradient(r=0.72) and mitral valve area(r=-0.47). 3) When patients were divided into those with a pulmonary vascular bed gradient > 12mmHg and Atrial Pressure ; Cardiac Output ; Echocardiography ; Electrocardiography ; Female ; Heart Diseases ; Humans ; Hypertension, Pulmonary* ; Mitral Valve ; Mitral Valve Insufficiency ; Mitral Valve Stenosis* ; Pulmonary Artery ; Vascular Resistance

The cause of cyclic strabismus is not clear but is assumed to be an abnormality of the biological clock mechanism. Secondary factors, combined with surgery or trauma as well as unknown causes stimulate its occurrence and may be followed by the periodicity of strabismus. We report postoperative follow-up results of 4 cases of cyclic esotropia following surgery. Two cases (case 1 and 2), who developed cyclic esotropia after surgery for intermittent exotropia, show good postoperative alignment without periodicity. But in case 3, originally treated for intermittent exotropia after receiving treatment for consecutive esotropia, the patient reverted to original condition. In case 4, originally treated for congenital glaucoma and receiving secondary treatment for cyclic esotropia, the cyclic esotropia was recurred. After surgery for consecutive cyclic strabismus, it is necessary to follow up for a long time.
Biological Clocks ; Esotropia* ; Exotropia ; Follow-Up Studies ; Glaucoma ; Humans ; Periodicity ; Strabismus

The Kidd (Jk) system is one of the most important blood group systems in transfusion medicine due to immediate or delayed hemolytic transfusion reactions as well as hemolytic disease of newborn (HDN). We experienced a case of jaundice and hemolytic anemia in a newborn due to anti-Jk(b) incompatibility appearing within the first 24 hours of life. The infant's direct and indirect antiglobulin tests were positive. There were no ABO and Rh (D) incompatibilities between the mother and the baby. Direct Coomb's IgG was strongly positive but C3d was negative. We started the exchange transfusion with the whole blood and had a favorable outcome. We report this case with a brief review of relevant literature.
Anemia, Hemolytic ; Blood Group Incompatibility ; Coombs Test ; Erythroblastosis, Fetal ; Humans ; Immunoglobulin G ; Infant, Newborn ; Jaundice ; Jaundice, Neonatal* ; Mothers ; Transfusion Medicine