ABSTRACT
Fibrotic disease is a type of disease in which the body is affected by various stimulating factors, which causes the activation or phenotypic transformation of myofibroblasts, increases the expression of collagen and excessive deposition of extracellular matrix, resulting in damage to the structure and function of organs. Transforming growth factor-β (TGF-β) is currently recognized as a signal transduction pathway involved in the occurrence of fibrotic diseases, and plays an important role in regulating the occurrence and development of fibrotic diseases such as lung, liver, myocardium, kidney and pancreas. In recent years, many studies have explored the role of TGF-β signaling pathway in the occurrence and development of fibrotic diseases, while to inhibit the activation of TGF-β signaling pathway might have anti-fibrosis effects, but the mechanism has not been fully understood. Therefore, the research progress of the role of TGF-β signaling pathway in such fibrotic diseases as lung, liver, myocardium, kidney and pancreas, and so on, has mainly been reviewed in present paper, so as to provide new strategies for the selection of therapeutic targets for fibrotic diseases.
ABSTRACT
<p><b>INTRODUCTION</b>The nationwide neonatal screening for glucose-6-phosphate dehydrogenase (G6PD) deficiency in Taiwan was started on 1 July 1987. A network of G6PD referral hospitals distributed all around Taiwan was organised for follow-up, confirmatory testing, medical care and genetic counselling. To assess the reliability of confirmatory and screening tests, an external quality assurance (QA) programme for G6PD assay was developed.</p><p><b>MATERIALS AND METHODS</b>Lyophilised quality control (QC) materials and dried blood spots were prepared from erythrocytes and whole blood for confirmatory and screening tests, respectively. The external QA surveys were carried out every 1 to 2 months. The QA results were evaluated and compared to the consensus result and reference value. The test results were submitted through internet by participating laboratories and the summary reports were published on a webpage (http:// www.g6pd.tw) within 2 weeks.</p><p><b>RESULTS</b>Twenty-one referral laboratories in Taiwan and 16 screening laboratories in Germany, Lebanon, Mainland China, Philippines, Thailand, Taiwan, Turkey, and Vietnam have been participating in the QA programme. From 1988 to 2007, 144 QA surveys for confirmatory testing were sent to referral laboratories. Among the 2,622 reports received, 292 (11.1%) were found to be abnormal. Interlaboratory coefficient of variation (CV) for the confirmatory test has reached below 10% in recent years. The significant improvement in interlaboratory CV was found to be correlated with the preventive site visits to the referral laboratories since November 2004. From 1999 to 2007, 52 external QA surveys for the screening test were performed. Among 504 reports received, 97 (19.2%) were found to be abnormal. From the 5040 blood spots tested by the screening laboratories, 95 false negative (1.9%) and 187 false positive (3.7%) results were reported.</p><p><b>CONCLUSIONS</b>The external QA programme has been useful for monitoring the performance of the referral hospitals and screening laboratories and helpful for the participating laboratories to improve their test quality.</p>