Hernia fibroblasts lack β-estradiol induced alterations of collagen gene expression

BACKGROUND: Estrogens are reported to increase type I and type III collagen deposition and to regulate Metalloproteinase 2 (MMP-2) expression. These proteins are reported to be dysregulated in incisional hernia formation resulting in a significantly decreased type I to III ratio. We aimed to evaluate the β-estradiol mediated regulation of type I and type III collagen genes as well as MMP-2 gene expression in fibroblasts derived from patients with or without history of recurrent incisional hernia disease. We compared primary fibroblast cultures from male/female subjects without/without incisional hernia disease. RESULTS: Incisional hernia fibroblasts (IHFs) revealed a decreased type I/III collagen mRNA ratio. Whereas fibroblasts from healthy female donors responded to β-estradiol, type I and type III gene transcription is not affected in fibroblasts from males or affected females. Furthermore β-estradiol had no influence on the impaired type I to III collagen ratio in fibroblasts from recurrent hernia patients. CONCLUSION: Our results suggest that β-estradiol does not restore the imbaired balance of type I/III collagen in incisional hernia fibroblasts. Furthermore, the individual was identified as an independent factor for the β-estradiol induced alterations of collagen gene expression. The observation of gender specific β-estradiol-dependent changes of collagen gene expression in vitro is of significance for future studies of cellular response

Blau syndrome : cross-sectional data from a multicentre study of clinical, radiological and functional outcomes

OBJECTIVE: To report baseline articular, functional and ocular findings of the first international prospective cohort study of Blau syndrome (BS). METHODS: Three-year, multicentre, observational study on articular, functional (HAQ, Childhood HAQ and VAS global and pain), ophthalmological, therapeutic and radiological data in BS patients. RESULTS: Baseline data on the first 31 recruited patients (12 females and 19 males) from 18 centres in 11 countries are presented. Of the 31 patients, 11 carried the p.R334W NOD2 mutation, 9 the p.R334Q and 11 various other NOD2 missense mutations; 20 patients were sporadic and 11 from five BS pedigrees. Median disease duration was 12.8 years (1.1-57). Arthritis, documented in all but one patient, was oligoarticular in 7, polyarticular in 23. The median active joint count was 21. Functional capacity was normal in 41%, mildly impaired in 31% and moderate-severe in 28% of patients. The most frequently involved joints at presentation were wrists, ankles, knees and PIPs. On radiographs, a symmetrical non-erosive arthropathy was shown. Previously unknown dysplastic bony changes were found in two-thirds of patients. Ocular disease was documented in 25 of 31 patients, with vitreous inflammation in 64% and moderate-severe visual loss in 33%. Expanded manifestations (visceral, vascular) beyond the classic clinical triad were seen in 52%. CONCLUSION: BS is associated with severe ocular and articular morbidity. Visceral involvement is common and may be life-threatening. Bone dysplastic changes may show diagnostic value and suggest a previously unknown role of NOD2 in bone morphogenesis. BS is resistant to current drugs, suggesting the need for novel targeted therapies

On the Coverage of All-Risk Cargo Insurance——Focus on Carrier`s Unauthorized Disposal of Cargo

海洋运输货物一切险（以下简称“海运货物一切险”）在分摊海上货物运输风险方面发挥着重大作用。然而，由于我国海上保险制度的不完善，使得海运货物一切险在应对海上风险，尤其是承运人私自处分货物等外来风险时存在较大不足。本文以承运人私自处分货物风险为中心，剖析我国海运货物一切险承保范围的不足，借鉴英国相关制度的最新发展，结合海运货物一切险承保范围识别标准的法理基础，论证我国海运货物一切险应加强对承运人私自处分货物风险的承保，并提出相应的保险条款完善建议。 本文除了引言和结语外，正文共分四章： 第一章评析我国海运货物一切险承保范围应对承运人私自处分货物风险的现状与不足。由于我国海运货物一切险条款中对“...All-risk cargo insurance plays an important role in sharing the risk of carriage of goods by sea. However, owing to the imperfection of Chinese marine insurance system, there exists great deficiency for all-risk cargo insurance in confronting with marine risks, especially external risks such as carrier's unauthorized disposal of cargos (hereinafter referred to as CUDC). Focusing on the risks of CU...学位：法学硕士院系专业：法学院法律系_民商法学(含劳动法学、社会保障法学)学号：1362008115068