Multiple Hereditary Exostoses. Clinical problems and therapeutic options

Multiple Hereditary Exostoses (MHE) is a rare autosomal dominant disorder characterized by the presence of multiple osteochondromas. Patients often present with growth disturbances and angular deformities of the long bones, and a limited range of motion of the joints. In adults, the risk of malignant transformation of osteochondroma into chondrosarcoma exists, with an estimated incidence of 3–6%. In addition to the physical deformities, pain is a significant problem for patients with MHE and the disease has a profound negative influence on activities of daily living, occupation and school performance.In the treatment of patients with MHE, knowledge about the various disorders encountered and the natural course of the disease, as well as appropriate timing of interventions are essential. Surgical treatment may not only include the excision of osteochondromas but also limb reconstruction procedures to correct deformities. During childhood, however, this may be limited to procedures such as hemiepiphysiodesis for valgus deformity of the knee and ankle to prevent more extensive surgery at older age. Furthermore, early recognition of malignant degeneration of osteochondroma and the immediate treatment of chondrosarcoma are of the utmost importance for patient survival. Therefore, periodic screening in children as well as adults is highly recommended.     

Lymphadenopathy associated with IgG4-related disease: Diagnosis & differential diagnosis

IgG4-related sclerosing disease, which now encompasses diverse organ-related disorders with various prior eponymic designations, may also present with solitary or multifocal lymph node enlargement. This review considers the histopathologic features of IgG4 lymphadenopathy (IgG4LAD), which has been subdivided by Cheuk & Chan into 5 microscopic subtypes. Those include variants that are typified by multicentric Castleman disease (MCD)-like changes, follicular hyperplasia, interfollicular lymphoplasmacytic proliferation, progressive transformation of germinal centers, and formation of inflammatory pseudotumor (IPT)-like lesions. All of them demonstrate an excess of IgG4-immunoreactive plasma cells in the inflammatory cell population. Differential diagnostic considerations for IgG4LAD include true MCD, true IPT, luetic lymphadenitis, Rosai-Dorfman disease, and inflammatory myofibroblastic tumor, among others. An interpretative distinction between malignant lymphoma and IgG4LAD is also crucial.     

血管平滑肌细胞表型转换对动脉粥样硬化的作用研究进展

在动脉粥样硬化的进程中,血管中膜平滑肌细胞发生表型转换、迁移、增殖,进入血管内膜,参与动脉粥样硬化斑块纤维帽及新生血管的生成。本文就当前关于血管平滑肌细胞表型转换对动脉粥样硬化作用的研究进展作一综述。     

医院科研基地的节能措施与成效-以四川大学华西医院科研基地为例

文章以四川大学华西医院科研基地为例，分析了科研基地的既有设施设备用能设计指标与当前实际环境需求的差异，提出了对公共区域照明、围墙灯、绿地照明以及中央空调系统末端等方面改进的相应措施，并分析了实施效果。     

Endocrine systems in juvenile anadromous and landlocked Atlantic salmon (Salmo salar): seasonal development and seawater acclimation

The present study compares developmental changes in plasma levels of growth hormone (GH), insulin-like growth factor I (IGF-I) and cortisol, and mRNA levels of their receptors and the prolactin receptor (PRLR) in the gill of anadromous and landlocked Atlantic salmon during the spring parr-smolt transformation (smoltification) period and following four days and one month seawater (SW) acclimation. Plasma GH and gill GH receptor (GHR) mRNA levels increased continuously during the spring smoltification period in the anadromous, but not in landlocked salmon. There were no differences in plasma IGF-I levels between strains, or any increase during smoltification. Gill IGF-I and IGF-I receptor (IGF-IR) mRNA levels increased in anadromous salmon during smoltification, with no changes observed in landlocked fish. Gill PRLR mRNA levels remained stable in both strains during spring. Plasma cortisol levels in anadromous salmon increased 5-fold in May and June, but not in landlocked salmon. Gill glucocorticoid receptor (GR) mRNA levels were elevated in both strains at the time of peak smoltification in anadromous salmon, while mineralocorticoid receptor (MR) mRNA levels remained stable. Only anadromous salmon showed an increase of gill 11beta-hydroxysteroid dehydrogenase type-2 (11beta-HSD2) mRNA levels in May. GH and gill GHR mRNA levels increased in both strains following four days of SW exposure in mid-May, whereas only the anadromous salmon displayed elevated plasma GH and GHR mRNA after one month in SW. Plasma IGF-I increased after four days in SW in both strains, decreasing in both strains after one month in SW. Gill IGF-I mRNA levels were only increased in landlocked salmon after 4days in SW. Gill IGF-IR mRNA levels in SW did not differ from FW levels in either strain. Gill PRLR mRNA did not change after four days of SW exposure, and decreased in both strains after one month in SW. Plasma cortisol levels did not change following SW exposure in either strain. Gill GR, 11beta-HSD2 and MR mRNA levels increased after four days in SW in both strains, whereas only the anadromous strain maintained elevated gill GR and 11beta-HSD2 mRNA levels after one month in SW. The results indicate that hormones and receptors of the GH and cortisol axes are present at significantly lower levels during spring development and SW acclimation in landlocked relative to anadromous salmon. These findings suggest that attenuation of GH and cortisol axes may, at least partially, result in reduced preparatory upregulation of key gill ion-secretory proteins, possibly a result of reduced selection pressure for marine adaptations in landlocked salmon.