No abstract

Aortic aneurysm is a complex multifactorial disease, and its molecular mechanism is not understood. In thoracic aortic aneurysm (TAA), the expansion of the aortic wall is lead by extracellular matrix (ECM) degeneration in the medial layer, which leads to weakening of the aortic wall. This dilatation may end in rupture and-if untreated-death. The aortic media is composed of vascular smooth muscle cells (VSMCs) and proteins involved in aortic elasticity and distensibility. Delineating their functional and quantitative decrease is critical in elucidating the disease causing mechanisms as well as the development of new preventive therapies. Laser microdissection (LMD) is an advanced technology that enables the isolation of the desired portion of tissue or cells for proteomics analysis, while preserving their integrity. In our study, the aortic media layers of 36 TAA patients and 8 controls were dissected using LMD technology. The proteins isolated from these tissue samples were subjected to comparative proteomic analysis by nano-LC-MS/MS, which enabled the identification of 352 proteins in aortic media. Among these, 41 proteins were differentially expressed in the TAA group with respect to control group, and all were downregulated in the patients. Of these medial proteins, 25 are novel, and their association with TAA is reported for the first time in our study. Subsequent analysis of the data by ingenuity pathway analysis (IPA) shows that the majority of differentially expressed proteins were found to be cytoskeletal-associated proteins and components of the ECM which are critical in maintaining aortic integrity. Our results indicate that the protein expression profile in the aortic media from TAA patients differs significantly from controls. Further analysis of the mechanism points to markers of pathological ECM remodeling, which, in turn, affect VSMC cytosolic structure and architecture. In the future, the detailed investigation of the differentially expressed proteins may provide insight into the elucidation of the pathological processes underlying aneurysms.

Background: It is difficult to predict the complications and prognosis of ECMO, which is gaining widespread use in patients with pediatric surgery. Platelet-to-lymphocyte ratio (PLR) and neutrophil-to-lymphocyte ratio (NLR) are emerging inflammatory markers that can be calculated from complete blood count, which is a cheap and easily accessible laboratory analysis. The ratios between cellular elements in peripheral blood have been demonstrated to provide information on inflammation, infection, and immune response. Methods: Sixty-seven patients who needed ECMO application after undergoing pediatric cardiovascular surgery in our clinic, between May 2005 and April 2020, were included in this study. The age of patients varied between 4 days and 17 years with a mean of 30.59 ± 147.26 months. Results: The relationships between PLR or NLR values and various blood parameters and blood gas results were found to be statistically nonsignificant in our group of pediatric ECMO recipients. Even if the effect of PLR and NLR values on mortality and prognosis is statistically nonsignificant in patients who need ECMO after congenital heart surgery, PLR and NLR are typically elevated in the postoperative period. An increase in these values above a certain threshold may be a statistically significant indicator for the prediction of mortality. Conclusions: There are few studies in the literature concerning PLR and NLR values in patients with pediatric heart surgery. We consider this study will make way for new studies in the future.

Background The number of pediatric patients who survive open-heart surgery has increased in recent years and the complications seen in this patient group continue to decrease with each technological advance, including new surgical and neuroprotective techniques and the improvement in surgeons’ experience with this patient population. However, neurological complications, which are the most feared and difficult to manage, require long-term follow-up, and increase hospital costs remain a leading cause of mortality and morbidity in this cohort. Results We evaluated the neurological physical examination, cranial computed tomography (CT), and magnetic resonance (MRI) records of 162 pediatric patients with neurological symptoms lasting more than 24 h after undergoing heart surgery in our clinic between June 2012 and May 2020. The patients’ ages ranged from 0 to 205 months, with a mean of 60.59 ± 46.44 months. Of the 3849 pediatric cardiac surgery patients we screened, 162 had neurological complications in the early period (the first 10 days after surgery). The incidence was calculated as 4.2%; 69 patients (42.6%) experienced seizures, 17 (10.5%) experienced confusion, 39 (24.1%) had stupor, and 37 (22.8%) had hemiparesis. Of the patients who developed neurological complications, 54 (33.3%) died. Patients with neurological complications were divided into 3 groups: strokes (n = 90), intracranial bleeding (n = 37), and no radiological results (n = 35). Thirty-four patients (37.8%) in the stroke group died, as did 15 (40%) in the bleeding group, and 5 (14.3%) in the no radiological results group. Conclusions Studies on neurological complications after pediatric heart surgery in the literature are currently insufficient. We think that this study will contribute to a more detailed discussion of the issue. Responses to neurological events and treatment in the pediatric group may differ compared to the adult age group. Primary prevention methods should be the main approach in combating neurological complications; their formation mechanisms should be carefully monitored and preventive treatment strategies should be developed.

Pulmonary artery aneurysms are rare entities which are usually associated with other congenital defects. While surgery is the preferred treatment for symptomatic aneurysms, controversy exists as to the indications for repair in asymptomatic patients.

Pulmonary pressure is one of the most important parameters in the postoperative follow-up of patients who have undergone the Glenn procedure. Platelet activation markers, which are inexpensive and easily accessible blood count parameters, have been shown to be associated with the aetiology and pathogenesis of primary pulmonary artery hypertension. We examined the relationship between platelet activation markers and pulmonary pressures in the early postoperative period of patients who underwent the Glenn procedure. Eighty-five patients who underwent the Glenn procedure in our clinic between January 2011 and March 2020 were included in the study retrospectively. Fifty-one patients were male and 34 were female, and age varied from 4 to 416 months, with a mean of 28.64 ± 51 months. Patients with increased pulmonary blood flow on palliation before Glenn surgery had higher mean platelet volume values. However, no correlation was found between pulmonary pressures and platelet activation markers in the early postoperative period. There was not similar study evaluating platelet activation markers in the paediatric age group before and after postoperative Glenn surgery in the literature. Therefore, even if platelet activation markers provide information about the pulmonary bed, they may be misleading due to other reasons that trigger bleeding and inflammatory processes in the early postoperative period.

No abstract

Ailesel homozigot hiperkolesterolemi düşük yoğunluklu lipoprotein reseptör gen mutasyonlarından kaynaklanan otozomal dominant bir hastalıktır. Hastalık erken yaşta koroner arter hastalığı gelişme riskinde artış ile ilişkilidir. Bu yazıda, yaygın koroner arter hastalığı ve ailesel homozigot hiperkolesterolemisi olan ve başarılı koroner arter baypas greft cerrahisi yapılan 13 yaşında bir erkek olgu sunuldu. Anah tar söz cük ler: Koroner arter hastalığı; koroner baypas arter greftleme; ailesel hiperkolesterolemi; cerrahi. Homozygous familial hypercholesterolemia is an autosomal dominant disorder caused by mutations in the low density lipoprotein receptor gene. The disease is associated with an increased risk of coronary artery disease early in life. In this article, we present a 13-year-old boy with familial homozygous hypercholesterolemia who had an extensive coronary artery disease and underwent successful coronary artery bypass graft surgery.