Exaggerated elastin turnover in young individuals with Marfan syndrome: new insights from the AIMS trial.

Aims: The fragmentation and loss of elastic fibre in the tunica media of the aorta are pathological hallmarks of Marfan syndrome (MFS) but the dynamics of elastin degradation and its relationship to aortic size and physiological growth remain poorly understood.

Methods And Results: In this analysis of the AIMS randomized controlled trial, the association of plasma desmosine (pDES)-a specific biomarker of mature elastin degradation-with age and aortic size was analysed in 113 patients with MFS and compared to 109 healthy controls. There was a strong association between age and pDES in both groups, with higher pDES levels in the lower age groups compared to adults.

Biomarkers of Aortopathy in Marfan Syndrome.

Marfan Syndrome (MFS) is an autosomal dominant, genetically inherited connective tissue disorder which primarily affects the cardiovascular system, but can also have systemic manifestations. First described in 1896, MFS has a prevalence of around 1/5000 in the general population. It is becoming increasingly common to see patients with MFS in a clinical setting due to the improved care of patients with adult congenital heart disease and general improvement in survival.

Plasma Desmosine and Abdominal Aortic Aneurysm Disease.

Background It is recognized that factors beyond aortic size are important in predicting outcome in abdominal aortic aneurysm (AAA) disease. AAA is characterized by the breakdown of elastin within the aortic tunica media, leading to aortic dilatation and rupture. The aim of this study was to investigate the association of plasma desmosine (pDES), an elastin-specific degradation product, with disease severity and clinical outcome in patients with AAA.

Characteristics of subjects counseled and tested for HIV in an Indonesian hospital; factors associated with HIV-status and CD4 cell-count.

Aim: to identification the predictors of HIV-seropositivity and level of immunosuppression.

Methods: in a cross-sectional study, we examined HIV-testing practice, HIV-knowledge, perceived risk of being HIV-positive, and health status among out-patients enrolled in voluntary counseling and testing (VCT), and in-patients who underwent provider-initiated testing (PITC) in the largest teaching hospital in West Java, Indonesia.

Results: among 202 respondents, 47% were HIV+, and the median CD4 of HIV+ was 193/mm³ among VCT (n=124) and 11/mm³ among PITC (n=78).

The expression of p53 in invasive ductal carcinoma of the breast: a study in the North-East States of Malaysia.

The p53 gene is a tumour suppressor gene that encodes a 393-amino-acid nuclear DNA-binding phosphoprotein. The significance of p53 detection is that p53 mutation is linked with chemo-resistance and transformation to more aggressive disease in a large number of tumour types and it was confirmed that mutant p53 is involved in neoplastic transformations. In addition, the expression of p53 has been closely correlated with clinicopathological findings.