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Biomedical subjects

C H Tsai

Publications and source records attributed to C H Tsai.

At least 19 recordsLinked to original sources

Two novel mutations of the glycine receptor gene in a Taiwanese hyperekplexia family.

The authors report a Taiwanese family with autosomal recessive hyperekplexia. Two novel mutations, W96C (from the paternal allele) and R344X (from the maternal allele), which are located in exon 4 and exon 7 of the GLRA1 gene, were identified in this family. A series of electrophysiologic investigations were conducted in one of the probands, and the results suggest that the "startle center" is located subcortically.

Acoustic Stimulation↗

Increase in laminin expression in allergic airway remodelling and decrease by dexamethasone.

Lung expression of the extracellular matrix protein, laminin, and its receptor, laminin-1 receptor, were examined in a mouse model of asthma with airway remodelling. Ovalbumin (OVA) was administered to BALB/c mice, intraperitoneally on days 0 and 14, and intranasally periodically between days 14 and 75. The mice developed airway eosinophil and mononuclear inflammatory cell infiltration and fibrosis. On day 76, a marked increase in total laminin was seen in the airways of OVA-treated mice compared to controls by Western blot analysis. The increased laminin expression was detected immunocytochemically in the thickened subepithelial basement membrane and around airways and blood vessels. The OVA-treated mice showed increased expression of the alpha1, beta1, and gamma1 chains of the laminin-1 isoform in monocytes, macrophages and eosinophils infiltrating the airways. Laminin-1 receptor expression was increased in inflammatory and endothelial cells in the lungs of OVA-treated mice compared to controls. Treatment of OVA-sensitised/challenged mice with dexamethasone reduced airway expression of laminin and laminin-1 receptor in OVA-treated mice but not airway hyperresponsiveness to methacholine. Laminin deposition may be an important component of the airway remodelling observed in chronic allergic lung inflammation and is a process modulated by corticosteroids.

Airway Resistance↗

Combined therapy with deferiprone and desferrioxamine successfully regresses severe heart failure in patients with beta-thalassemia major.

Cardiac complications caused by iron deposition are major causes of death in patients with beta-thalassemia major. Deferiprone (L1) was found to have greater efficacy at depleting myocardial iron than desferrioxamine (DFX). Furthermore, combined therapy with L1 and DFX produced an additive or synergistic iron chelating effect. We report the successful treatment of severe heart failure in two patients with beta-thalassemia major with the combined therapy. Magnetic resonance images showed a marked recovery of signal intensity in the heart, indicating a significant reduction of iron load in the heart. No significant adverse effects were noted. Therefore, combined therapy with L1 and DFX should be considered in patients with beta-thalassemia major and cardiac complications.

Adult↗

Detecting abnormal regional cerebral blood flow in patients with primary Sjögren's syndrome by technetium-99m ethyl cysteinate dimer single photon emission computed tomography of the brain--a preliminary report.

Technetium-99m ethyl cysteinate dimer (Tc-99m ECD) brain single photon emission computed tomography (SPECT) was used to detect abnormal regional cerebral blood flow (rCBF) in 32 female patients with primary Sjögren's syndrome (PSS) showing definite neuropsychiatric symptoms/signs and normal brain magnetic resonance imaging (MRI) findings. It demonstrated hypoperfusion brain lesions in 18 (56.3%) of the patients, most frequently in the parietal lobes, and appears to be a sensitive tool for this clinical application.

Adult↗

Simultaneously evaluating the effects of one-week fluticasone propionate inhalation therapy on lung ventilation and permeability in children with asthma.

This study evaluated the effects of fluticasone propionate inhalation therapy on lung ventilation and alveolar permeability by quantitative Tc-99m DTPA radioaerosol inhalation lung scintigraphy in 15 children with asthma. Lung ventilation was evaluated as the distribution percentage (D%) of Tc-99m DTPA radioaerosols in the central, intermediate and peripheral regions of the right lung. Alveolar permeability was measured by the rate of Tc-99m DTPA radioaerosol clearance curve from the peripheral alveoli of the right lung and represented as slope. The D% and slopes were calculated before and after one-week inhalation therapy (100 microg fluticasone propionate two times daily for one-week) to evaluate the effects of inhalation therapy on lung ventilation and alveolar permeability. The preliminary results revealed statistically significantly improved lung ventilation but no significant change of alveolar permeability in the right lung after one-week fluticasone propionate inhalation therapy in children with asthma. We suggest that the widely available and noninvasive Tc-99m DTPA radioaerosol inhalation lung scintigraphy can simultaneously evaluate lung ventilation and alveolar permeability in one study and should contribute to any disorder involving both alveoli and airways.

Adolescent↗

Usefulness of 99mTc ethyl cysteinate dimer brain SPECT to detect abnormal regional cerebral blood flow in patients with acute carbon monoxide poisoning.

99mTc ethyl cysteinate dimer (99mTc-ECD) brain single photon emission computed tomography (SPECT) was used to detect abnormal regional cerebral blood flow (rCBF) in patients with acute carbon monoxide (CO) poisoning. Ten patients with acute CO poisoning and no past histories of psychoneurological disorders were enrolled in this study. After oxygen treatment, all of the 10 patients were investigated using 99mTc-ECD brain SPECT and brain computed tomography (CT) scan. Brain CT scan findings were normal in all of the 10 patients. 99mTc-ECD brain SPECT showed the hypoperfusion lesions of the basal ganglia and brain cortex in five and seven patients, respectively. Only three of the 10 patients had normal 99mTc-ECD brain SPECT findings. This study suggests that, in comparison with brain CT scan, 99mTc-ECD brain SPECT is a better tool for the early detection of hypoperfusion brain lesions in acute CO poisoning in patients with normal brain CT findings.

Adolescent↗

Comparison of quantitative 99mTc-HMPAO and 67Ga citrate lung scans in patients with active diffuse infiltrative lung disease.

Diffuse infiltrative lung disease (ILD) is a heterogeneous group of disorders that predominantly affect the lung parenchyma and spare the airway. To objectively assess the degree of pulmonary vascular endothelium damage in active ILD, lung/liver uptake ratios (L/L ratios) on 99mTc hexamethylpropylene amine oxime (99mTc-HMPAO) lung scans were determined in 21 patients with active ILD. Meanwhile, the 67Ga citrate uptake index (GUI) on 67Ga lung scans was measured in order to evaluate the severity of lung inflammation in active ILD. The results show there were statistically significant differences between normal controls and patients with active ILD, as shown in the L/L ratio and GUI. However, when the patients were divided into two groups: (1) eight patients with normal chest X-ray findings, and (2) 13 patients with abnormal X-ray findings, there was no significant difference between groups 1 and 2 for the results of L/L ratio and GUI. In addition, no correlation between the degree of damage to the pulmonary vascular endothelium and the severity of lung inflammation was found. In conclusion, L/L ratios on 99mTc-HMPAO lung scans and GUI on 67Ga lung scans are different to the findings of chest X-rays and have the potential to objectively detect the degree of damage to the pulmonary vascular endothelium and the severity of lung inflammation in active IDL. However, the relationship between L/L ratio and GUI in active ILD is not significant.

Acute Disease↗

Objective evidence of abnormal regional cerebral blood flow in patients with systemic lupus erythematosus on Tc-99m ECD brain SPECT.

Technetium-99m ethyl cysteinate dimer (Tc-99m ECD) brain single photon emission computed tomography (SPECT) was used to detect abnormal regional cerebral blood flow (rCBF) in 78 SLE patients with neuropsychiatric manifestations. These patients were separated into two subgroups: group 1 including 48 cases with definite neuropsychiatric symptoms/signs and group 2 with 30 cases having no neuropsychiatric symptoms/signs. Tc-99m ECD brain SPECT demonstrated hypoperfusion brain lesions in 90% and 20% of patients in groups 1 and 2, respectively. In both groups, parietal lobe and cerebellum are the most and least common areas with hypoperfusion lesions, respectively. This study suggests that Tc-99m ECD brain SPECT may provide objective information for detection of hypoperfusion brain lesions in SLE patients.

Adult↗

Environmental risk factors of young onset Parkinson's disease: a case-control study.

While the cause of Parkinson's disease (PD) remains unknown, recent evidence suggests certain environmental factors, such as well water drinking, herbicides and pesticides exposure, and neurotoxins, may trigger the chain of oxidative reactions culminating in the death of dopaminergic neurons in substantia nigra to cause parkinsonism. Most studies to date focused on PD with old age onset. However, there is a peculiar group of parkinsonian patients, the young onset Parkinson's disease (YOPD), in whom the age of onset is before 40. It is intriguing to know whether earlier exposure to the putative neurotoxin(s) may contribute to the earlier onset. We therefore conducted this case-control study in which 60 PD patients, 30 YOPD patients and the same number of age- and sex-matched young controls were included. Using logistic regression, we found well water drinking and head injury were risk factors for the development of YOPD. When YOPD patients were compared with PD, we found head injury and exercise were the significant predictors. Keeping all other variables constant, head injury was a risk factor and exercise appeared to be a protective factor. We conclude early exposure to well water drinking and head trauma may trigger and expedite the appearance of parkinsonian features, but such acceleration may be prevented through regular exercise.

Adult↗

Hydrops foetalis caused by anti-Mur in first pregnancy--a case report.

Anti-'Mia' is the most common alloantibody of potential clinical significance in the Taiwanese population. The Mi.III phenotype is rare among Caucasians but has a high incidence in various Oriental populations. We describe a nulliparous woman with no history of transfusions, who had hydrops foetalis at 28 weeks gestation. Foetal haemoglobin was 4.4 g dL-1, and a positive direct antiglobulin test was positive in the foetal blood. Intrauterine intravascular transfusion was given, and the baby was discharged healthy. Anti-'Mia' was identified in the maternal serum, the cord blood serum and the eluate from red cells of the cord blood. Anti-'Mia' in the maternal serum was confirmed to be anti-Mur. The polymerase chain reaction-restriction fragment length polymorphism method confirmed that both the baby and her father had the Mi.III gene. Therefore, our report documents that anti-Mur has the potential to cause hydrops foetalis.

ABO Blood-Group System↗

Heterogeneity of myocardial sleeve morphology and gap junctions in canine superior vena cava.

BACKGROUND: The myocardial sleeve of the superior vena cava (SVC) has been identified as a potential initiating focus in atrial fibrillation, but information on cell-to-cell linkage at this site is lacking. METHODS AND RESULTS: We examined the SVC in 8 dogs by immunoconfocal and electron microscopy. Cardiomyocytes outlined with vinculin and bearing striations positive for alpha-actinin are found in the proximal segment of the SVC. These cells, grouped in bundles of various orientations according to location, extend cephalically as far as 3 cm from the right atrium (RA)-SVC junction. Comparison between the junctional level and the level 2 cm distal shows that the myocardial layer in the latter is thinner and not as compact and is composed of longer cells (87.3+/-15.7 versus 71.6+/-14.4 micrometer, P<0.01). Gap junctions made of connexin43 (Cx43), Cx40, and Cx45 are aggregated mainly at the intercalated disks, and colocalization of connexins is a common feature throughout the myocardial sleeve. Areas of atypical expression exist, however, characterized by a center of abundant Cx43 labels surrounded by a periphery of scattered tiny Cx40-labeled spots. Although in the ventral subluminal compact myocardial layer, individual cells at both levels are surrounded by similar numbers of cells, the number of aggregation of labeled gap junctions at the distal level is less (2.3+/-0.6 versus 3.7+/-0.9, P<0.01). In addition, electron-microscopic examination demonstrates that the gap junctions at the distal level are smaller in size (0.37+/-0.30 versus 0.55+/-0.34 micrometer, P<0.01). CONCLUSIONS: The myocardial sleeve in the canine SVC is a heterogeneous structure, which could potentially form a substrate for heterogeneity of electrical coupling.

Actinin↗