Spontaneous pneumothorax due to Birt-Hogg-Dube syndrome in a Chinese family

A Chinese woman presented with a spontaneous pneumothorax and a family history suggestive of the autosomal dominant transmission of pneumothorax. The patient also had skin fibrofolliculomas and folliculin gene deletion, compatible with Birt-Hogg-Dube (BHD) syndrome. The importance of BHD syndrome and other familial spontaneous pneumothoraces is discussed.     

Cancer Prevention through Legislation-Hong Kong Experience

Cancer is the major cause of death worldwide and in the local community. Due to the aging population and changes in lifestyle of the citizens, it is expected that the incidence of cancer will continue to increase. In fact, according to the World Health Organization, about 30% of cancer death can be prevented. The fight against cancer relies on support from the government, together with collaborations with the policymakers, healthcare professionals, and the public. Legislation can act as a tool for cancer prevention. The purpose of this paper is to provide an overview of the global cancer burden and to describe how cancer legislation acts as a tool for cancer prevention in the Hong Kong region.     

Patient satisfaction with out-of-hours primary care in the Netherlands

Background  

In recent years out-of-hours primary care in the Netherlands has changed from practice-based to large-scale cooperatives. The purpose of this study is to determine patient satisfaction with current out-of-hours care organised in general practitioner (GP) cooperatives, and gain insight in factors associated with this satisfaction.     

T cell receptor-negative thymocytes from SCID mice can be induced to enter the CD4/CD8 differentiation pathway

In order to investigate the role of T cell receptor (TcR) expression in thymocyte maturation, we have analyzed thymocytes from C.B-17/SCID mice, which are unable to productively rearrange their antigen receptor genes and fail to express TcR. Despite this defect, SCID thymocytes are functional as they produce lymphokines and proliferate in response to a variety of stimuli. Phenotypic analysis revealed that thymocyte populations from young adult SCID mice resemble thymocyte populations from normal embryonic mice in that they are large, Thy-1.2+, CD4-, CD8-, TcR- and enriched in CD5lo, IL2R+ and Pgp1+ cells. However, other TcR- populations normally present in adult mice (i.e., CD4-CD8+ cells and CD4+CD8+ cells) are absent from the thymus of TcR- adult SCID mice. To understand the basis of the developmental arrest of TcR- SCID thymocytes at the CD4-CD8- stage of differentiation, we analyzed thymi from the occasional "leaky" SCID mouse which possesses small numbers of TcR+ thymocytes. We found that the presence of TcR+ cells within a SCID thymus was invariably associated with the presence of CD4+ and/or CD8+ SCID thymocytes. Interestingly, however, the CD4+/CD8+ SCID thymocytes were not themselves necessarily TcR+. That is, emergence of SCID thymocytes expressing CD4/CD8 was tightly linked to the presence of TcR+ cells within that SCID thymus, but the SCID thymocytes that expressed CD4/CD8 were not necessarily the same cells that expressed TcR. Finally, we found that the introduction into TcR- SCID mice of normal bone marrow cells that give rise to TcR+ cells within the SCID thymus promoted the differentiation of SCID thymocytes into CD4-CD8+ and CD4+CD8+ TcR- cells. These data indicate that TcR+ cells within the thymic milieu provide critical signals which promote entry of CD4-CD8-TcR- precursor T cells into the CD4/CD8 differentiation pathway. When applied to differentiation of normal thymocytes, these findings may imply a critical role for early appearing CD4-CD8- TcR (gamma/delta)+ cells in initiating normal thymic ontogeny.     

黄芩汤及其肠道菌群的代谢产物对D-半乳糖胺诱导的肝损伤的保护作用的比较研究

目的 :对黄芩汤及其经肠道菌群作用后的代谢产物的体内保肝作用进行比较研究。方法 :使用普通和伪无菌小鼠 ,用D-半乳糖胺造成肝脏损伤模型 ,分别给予黄芩汤和黄芩汤的代谢产物 ,测定给药 3d后血清中丙氨酸氨基转换酶 (ALT)和天门冬氨酸氨基转换酶 (AST)的活性。结果 :普通小鼠口服黄芩汤后 ,高、中剂量组小鼠血清中ALT水平明显下降 ,与模型组产生显著差异 ;伪无菌小鼠口服黄芩汤代谢产物之后 ,高、中剂量组血清中ALT水平显著下降 ,但黄芩汤 3个剂量组对其无相似的药理作用。结论 :黄芩汤经过肠道菌群作用后的代谢产物是体内产生保肝降酶作用的物质基础。     

Proliferation signal inhibitors in the treatment of lupus nephritis: Preliminary experience

Aim: Proliferation signal inhibitors (PSI) have demonstrated efficacy in prevention and treatment in an animal model of lupus nephritis (LN) but there are no data regarding the use of PSI in human LN. We report here our experience of using PSI treatment in seven patients with severe proliferative lupus nephritis. Methods: This is a retrospective study on patients with proliferative lupus nephritis who had received PSI treatment. Results: Seven patients were included. Two patients had concomitant membranous lupus nephropathy. The indications for PSI included mycophenolate mofetil intolerance (n = 4), history of malignancy (n = 2) and leucopoenia (n = 1). Five patients were given PSI when disease was active. Two had treatment discontinued because of acute cholecystitis and leucopoenia, respectively. In the other three patients combined steroid and PSI treatment as induction therapy led to improvements in serology, renal function and proteinuria. In two patients treated with PSI and low‐dose steroid as maintenance immunosuppression, both maintained stable lupus serology, renal function and proteinuria over 18 months. Side‐effects included dyslipidemia and oral ulcers. Conclusion: Proliferation signal inhibitors warrants further investigation as an alternative immunosuppressive treatment in lupus nephritis.