Five-year follow-up of left ventricular diastolic function in systemic sclerosis patients: Determinants of mortality and disease progression

Objectives

In systemic sclerosis (SSc), left ventricular diastolic dysfunction reflects primary myocardial involvement of the disease. We aimed to assess the abnormalities of the diastolic function, analyze the characteristics of the disease progression, and investigate the prognostic value of diastolic dysfunction in SSc patients.

Patients and methods

A total of 34 SSc patients (57 ± 12 years, 31 female) were involved in the study. The following traditional or tissue Doppler parameters of left ventricular diastolic function were obtained: E/A, lateral E?, E/E?, left ventricular mass index (LVM index), and maximal left atrial (LA) volume index. Measurements were repeated after 5.5 years.

Results

At baseline, diastolic dysfunction was found in 62% of the SSc patients. Follow-up time was 5.4 ± 1.2 years. A total of 6 patients died of heart failure. In univariate Cox regression analysis, age (HR = 1.08, p < 0.05), LVM index (HR = 1.07, p < 0.01), lateral E? (HR = 1.57, p = 0.05), and LA volume index (HR = 1.11, p < 0.01) were predictors of survival. During the follow-up, significant increase in LA volume index (27.5 ± 9.7 vs. 35.4 ± 10.6 cm³/m², p < 0.001) and E/E? was found (7.6 ± 2.5 vs. 8.7 ± 3.8, p < 0.05) while E? did not change (9.6 ± 2.6 vs. 9.2 ± 1.9 cm/s, NS). The increase in LA volume index showed positive correlation (r = 0.46, p < 0.05) while the decrease in E? values showed negative correlation (r = −0.54, p < 0.01) with the duration of the SSc.

Conclusion

In SSc patients, left ventricular diastolic dysfunction is highly prevalent and is associated with increased risk of mortality. Our data suggest that in the advanced phase of the disease, the myocardial fibrotic processes burns out while the increase of the filling pressure progresses continuously.     

Genetic factors associated with the presence and progression of nonalcoholic fatty liver disease: a narrative review

Nonalcoholic fatty liver disease (NAFLD) is the most common chronic liver disease in the world. Whereas insulin resistance and obesity are considered major risk factors for the development and progression of NAFLD, the genetic underpinnings are unclear. Before 2008, candidate gene studies based on prior knowledge of pathophysiology of fatty liver yielded conflicting results. In 2008, Romeo et al. published the first genome wide association study and reported the strongest genetic signal for the presence of fatty liver (PNPLA3, patatin-like phospholipase domain containing 3; rs738409). Since then, two additional genome wide scans were published and identified 9 additional genetic variants. Whereas these results shed light into the understanding of the genetics of NAFLD, most of associations have not been replicated in independent samples and, therefore, remain undetermined the significance of these findings. This review aims to summarize the understanding of genetic epidemiology of NAFLD and highlights the gaps in knowledge.     

Angiotensin-Converting Enzyme Gene Polymorphism Is Correlated to the Progression of Disease in Patients with IgA Nephropathy: A Metaanalysis

Angiotensin-converting enzyme (ACE) displays potent vasoconstrictive effects, attenuation of fibrinolysis, and platelet activation and aggregation. Recently it was mentioned that the insertion/deletion polymorphism of the ACE gene is associated with ESRD. The lengthy course of IgA Nephropathy (IgAN) and the possibility of good outcomes without therapy suggest nontoxic therapies such as ACE inhibitors and angiotensin receptor blockers (ARBs). However, the correlation between the ACE gene polymorphism and progression of IgAN still requires further approval. Here, the author performs a summative analysis on the recent previous reports on the ACE gene polymorphism and its correlation to progression of IgAN. The meta-analysis was performed to assess the correlation between the pattern of ACE gene polymorphism and progression of IgAN. From five available studies, 346 and 555 patients with (group 1) and without (group 2) the progression of disease are evaluated. According to this study, the frequency of DD genotype in group 1 is significant higher than group 2 (p < 0.05). In addition, the author first reports a non-significant correlation between the ethnicity and the ACE gene polymorphism.     

Progression of Kidney Damage in Balkan Endemic Nephropathy: A 15-Year Follow-Up of Patients with Kidney Biopsy Examination

Progression of kidney damage was studied in 18 patients with Balkan endemic nephropathy (BEN), with a mean 15-year follow-up after renal biopsy. According to kidney function, estimated by ^99mTc-DTPA clearance, patients were divided into three groups: with apparently normal kidney function (clearance 103.5 ± 21.3 mL/min/1.73 m²), with incipient renal failure (clearance 65.5 ± 11.3), and with advanced renal failure (clearance 28.0 ± 6.2). The mean yearly decrease of glomerular filtration rate was 2.74 mL/min. In two patients, an increase of kidney function was recorded. Six patients become dialysis dependent, two from the group with incipient renal failure, but all four from the group with advanced renal failure. Three patients died after 8 to 12 years of follow-up, one from causes unrelated to kidney disease and two from end-stage renal failure. This study has shown that BEN is characterized by a slow course and prolonged evolution, modified by medical supervision and treatment.     

Progression in Untreated Idiopathic Scoliosis After end of Growth

Seventy patients more than 16 years of age with untreated idiopathic scoliosis were followed-up for a mean period of 8.5 years. The majority of curves increased; 3° per year before 20 years of age and 1° per year after 20 years. Curves beiow 40° increased significantly less than bigger curves and curves from 60–80° increased the most. Single curves increased significantly more than double curves; thoracic curves increased significantly more than lumbar curves. It is concluded that surgery should be recommended to adolescent patients with thoracic or thoraco-lumbar single curves larger than 40°, and that patients not treated operatively should be followed-up through early adult life.