Tetralogy of Fallot (ToF) is one of the most common cyanotic congenital heart defects. We sought to summarize all available data regarding the epidemiology and perioperative outcomes of syndromic ToF ...patients. A PRISMA-compliant systematic literature review of PubMed and Cochrane Library was performed. Twelve original studies were included. The incidence of syndromic ToF was 15.3% (
n
= 549/3597). The most prevalent genetic syndromes were 22q11.2 deletion (47.8%; 95% CI 43.4–52.2) and trisomy 21 (41.9%; 95% CI 37.7–46.3). Complete surgical repair was performed in 75.2% of the patients (
n
= 161/214; 95% CI 69.0–80.1) and staged repair in 24.8% (
n
= 53/214; 95 CI 19.4–30.9). Relief of RVOT obstruction was performed with transannular patch in 64.7% (
n
= 79/122; 95% CI 55.9–72.7) of the patients, pulmonary valve-sparing technique in 17.2% (
n
= 21/122; 95% CI 11.5–24.9), and RV-PA conduit in 18.0% (
n
= 22/122; 95% CI 12.1–25.9). Pleural effusions were the most common postoperative complications (
n
= 28/549; 5.1%; 95% CI 3.5–7.3). Reoperations were performed in 4.4% (
n
= 24/549; 95% CI 2.9–6.4) of the patients. All-cause mortality rate was 9.8% (
n
= 51/521; 95% CI 7.5–12.7). Genetic syndromes are seen in approximately 15% of ToF patients. Long-term survival exceeds 90%, suggesting that surgical management should be dictated by anatomy regardless of genetics.
Background
No consensus currently exists regarding the optimal approach for peritoneal dialysis catheter placement. We aimed to compare the outcomes of percutaneous and surgical peritoneal dialysis ...catheter placement.
Methods
A systematic review of the literature was performed using the MEDLINE, Cochrane Library, and Scopus databases (end-of-search date: August 29th, 2020). We included studies comparing percutaneous (blind, under fluoroscopic/ultrasound guidance, and “half-perc”) and surgical peritoneal dialysis catheter placement (open and laparoscopic) in terms of their infectious complications (peritonitis, tunnel/exit-site infections), mechanical complications (leakage, inflow/outflow obstruction, migration, hemorrhage, hernia, bowel perforation) and long-term outcomes (malfunction, removal, replacement, surgery required, and mortality).
Results
Thirty-four studies were identified, including thirty-two observational studies (twenty-six retrospective and six prospective) and two randomized controlled trials. Percutaneous placement was associated with significantly lower rates of tunnel/exit-site infection relative risk (RR) 0.72, 95% confidence interval (CI) 0.56–0.91, catheter migration (RR 0.68, 95% CI 0.49, 0.95), and catheter removal (RR 0.73, 95% CI 0.60–0.88). The 2-week and 4-week rates of early tunnel/exit-site infection were also lower in the percutaneous group (RR 0.45, 95% CI 0.22–0.93 and RR 0.41, 95% CI 0.27–0.63, respectively). No statistically significant difference was observed regarding other outcomes, including catheter survival and mechanical complications.
Conclusion
Overall, the quality of published literature on the field of peritoneal dialysis catheter placement is poor, with a small percentage of studies being randomized clinical trials. Percutaneous peritoneal dialysis catheter placement is a safe procedure and may result in fewer complications, such as tunnel/exit-site infections, and catheter migration, compared to surgical placement.
Protocol registration
PROSPERO CRD42020154951.
Graphic abstract
Objectives
Our aim was to compare the safety and efficacy of proximal repair (PR) versus extensive repair (ER) for acute type A aortic dissection (ATAAD).
Methods
A literature search in three ...databases was performed according to the PRISMA statement. Studies comparing PR versus ER for ATAAD were included. Random-effects meta-analyses were performed.
Results
A total of 27 studies incorporating 7113 patients (PR: 5080; ER: 2033) were included. Patients undergoing PR presented decreased in-hospital mortality (odds ratio OR: 0.67 95% Confidence Interval (95% CI) 0.53–0.85;
p
< 0.01) and post-operative bleeding (OR 0.75 95% CI 0.60–0.95;
p
= 0.02) compared to ER. Meta-regression analysis revealed that in-hospital mortality was not influenced by differences regarding the extent of dissection (
p
= 0.43). Cardiopulmonary bypass time (SMD:-0.93 95% CI − 1.22, − 0.66;
p
< 0.01) and length of hospital stay (SMD:-0.19 95% CI − 0.34, − 0.05;
p
= 0.01) were also lower in the PR group, while there was no difference in terms of renal failure and permanent neurological deficit. The ER approach demonstrated a lower post-discharge mortality compared to PR (OR 1.46 95% CI 1.09, 1.97;
p
= 0.01), while the post-discharge reoperation rate was comparable between the two groups. 1 and 3-year overall survival (OS) were comparable between PR and ER (OR 1.05, 95% CI 0.77–1.44;
p
= 0.76) and (OR 1.27 95% CI 0.86–1.86;
p
= 0.23), respectively. The 5-year OS (OR 1.67 95% CI 1.16–2.41;
p
= 0.01) was in favor of the PR arm.
Conclusions
In patients with ATAAD, PR was associated with lower odds of in-hospital mortality but higher odds of late mortality. ER and PR demonstrated similar post-operative complication and reoperation rates.
Esophageal and gastro-esophageal junction cancer is a major cause of cancer-related mortality, with poor prognosis. Toll-like receptors (TLRs) play a significant role in the innate immune system; ...their increased expression has been associated with esophageal adenocarcinoma. This study aimed to determine the association between TLR-3 and TLR-4 expression with clinical and oncological outcomes of patients that underwent esophagectomy for cancer.
This is a retrospective analysis of prospectively collected data from consecutive patients within a 2-year period. Primary endpoints of the study were the assessment of the expression of TLR-3 and TLR-4 in primary tumors as well as in metastatic lymph nodes. Secondary endpoints were the correlation of TLR-3 and TLR-4 values with the clinical, pathological, and oncological outcomes.
A significantly higher expression of TLR-3 and TLR-4 in primary tumors and metastatic-lymph nodes was observed. There was a significant association between TLR-3 expression and T-stage, as well as TLR-4 expression and grade of differentiation in the primary site. Additionally, metastatic-lymph node TLR-4 expression was significantly correlated with N-stage. A strong correlation between TLR-4 expression and overall or progression-free survival rates was detected.
This study found a significantly increased TLR expression in malignant tissue/metastatic lymph nodes, as well as a significant positive correlation between TLRs and worse clinical outcomes. TLRs have a pivotal role in the inflammation pathway in the esophagus and during esophageal carcinogenesis. This study highlights the need for further investigation into TLR-mediated signaling pathways and their potential role as diagnostic and therapeutic targets.
Despite ongoing oncological advances, pancreatic ductal adenocarcinoma (PDAC) continues to have an extremely poor prognosis with limited targeted and immunotherapeutic options. Its genomic background ...has not been fully characterized yet in large-scale populations all over the world. Methods: Replicating a recent study from China, we collected tissue samples from consecutive Greek patients with pathologically-confirmed metastatic/unresectable PDAC and retrospectively investigated their genomic landscape using next generation sequencing (NGS). Findings: From a cohort of 409 patients, NGS analysis was successfully achieved in 400 cases (56.50% males, median age: 61.8 years). Consistent with a previous study,
was the most frequently mutated gene in 81.50% of tested samples, followed by
(50.75%),
(8%), and
(7.50%).
variants with on-label indications were detected in 2%, and 87.50% carried a variant associated with off-label treatment (
,
,
, or HRR-genes), while 3.5% of the alterations had unknown/preliminary-studied actionability (
). Most of HRR-alterations were in intermediate- and low-risk genes (
,
,
,
,
,
,
,
), with controversial actionability: 8% harbored a somatic non-
alteration, 6 cases had a high-risk alteration (
,
), and one co-presented a
alteration. Elevated LOH was associated with HRR-mutated status and
mutations while lowered LOH was associated with
alterations. Including TMB/MSI data, the potential benefit from an NGS-oriented treatment was increased from 1.91% to 13.74% (high-MSI: 0.3%, TMB > 10 muts/MB: 12.78%). TMB was slightly increased in females (4.75 vs. 4.46 muts/MB) and in individuals with age > 60 (4.77 vs. 4.40 muts/MB). About 28.41% showed PD-L1 > 1% either in tumor or immune cells, 15.75% expressed PD-L1 ≥ 10%, and only 1.18% had PD-L1 ≥ 50%. This is the largest depiction of real-world genomic characteristics of European patients with PDAC, which offers some useful clinical and research insights.
Background
Following the publication of reports from landmark international consensuses (Louisville 2008 and Morioka 2014), minimally invasive hepatectomy became widely accepted as a legitimate ...alternative to open surgery. We aimed to compare the operative, hospitalization, and total economic costs of open (OLR) vs. laparoscopic (LLR) vs. robotic liver resection (RLR).
Methods
We performed a systematic literature review (end-of-search date: July 3, 2020) according to the PRISMA statement. Random-effects meta-analyses were conducted. Quality assessment was performed with the Cochrane Risk of Bias tool for randomized controlled trials, and the Newcastle–Ottawa Scale for non-randomized studies.
Results
Thirty-eight studies reporting on 3847 patients (1783 OLR; 1674 LLR; 390 RLR) were included. The operative costs of LLR were significantly higher than those of OLR, while subgroup analysis also showed higher operative costs in the LLR group for major hepatectomy, but no statistically significant difference for minor hepatectomy. Hospitalization costs were significantly lower in the LLR group, with subgroup analyses indicating lower costs for LLR in both major and minor hepatectomy series. No statistically significant difference was observed regarding total costs between LLR and OLR both overall and on subgroup analyses in either major or minor hepatectomy series. Meta-analyses showed higher operative, hospitalization, and total costs for RLR vs. LLR, but no statistically significant difference regarding total costs for RLR vs. OLR.
Conclusion
LLR’s higher operative costs are offset by lower hospitalization costs compared to OLR leading to no statistically significant difference in total costs, while RLR appears to be a more expensive alternative approach.
Background: Hereditary cancer predisposition syndromes are responsible for approximately 5–10% of all diagnosed cancer cases. In order to identify individuals at risk in a cost-efficient manner, ...family members of individuals carrying pathogenic alterations are tested only for the specific variant that was identified in their carrier relative. The purpose of this study was to investigate the clinical use and implementation of cascade family testing (CFT) in families of breast cancer patients with pathogenic/likely pathogenic variants (PVs/LPVs) in cancer-related predisposition genes. Methods: Germline sequencing was carried out with NGS technology using a 52-gene panel, and cascade testing was performed by Sanger sequencing or MLPA. Results: In a cohort of 1785 breast cancer patients (families), 20.3% were found to have PVs/LPVs. Specifically, 52.2%, 25.1%, and 22.7% of patients had positive findings in high-, intermediate-, and low-penetrance breast cancer susceptibility genes, respectively. Although CFT was recommended to all families, only 117 families (32.3%) agreed to proceed with genetic testing. Among the first-degree relatives who underwent CFT, 70.3% were female, and 108 of 121 (89.3%) were cancer free. Additionally, 42.7%, 36.7%, and 20.6% were offspring, siblings, and parents of the subject, respectively. Our data suggest that CFT was mostly undertaken (104/117, 88.8%) in families with positive findings in high-risk genes. Conclusions: Cascade family testing can be a powerful tool for primary cancer prevention by identifying at-risk family members. It is of utmost importance to implement genetic counseling approaches leading to increased awareness and communication of genetic testing results.
Metabolic syndrome (MS) is defined as the constellation of obesity, insulin resistance, high serum triglycerides, low high-density lipoprotein cholesterol, and high blood pressure. It increasingly ...affects more and more people and progressively evolves into a serious issue with widespread healthcare, cost, and quality of life associated consequences. MS is associated with increased morbidity and mortality due to cardiovascular or chronic liver disease. Conservative treatment, which includes diet, exercise, and antidiabetic agents, is the mainstay of treatment, but depends on patient compliance to medical treatment and adherence to lifestyle modification recommendations. Bariatric surgery has recently emerged as an appropriate alternative treatment with promising long-term results. Sleeve gastrectomy and Roux-en-Y gastric bypass constitute the most commonly performed procedures and have been proven both cost-effective and safe with low complication rates. Liver transplantation is the only definitive treatment for end-stage liver disease and its utilization in patients with non-alcoholic steatohepatitis has increased more than fivefold over the past 15 years. In this review, we summarize current state of evidence on the surgical treatment of MS.
Septate uterus is the most common congenital uterine malformation. It has been associated with poor reproductive outcomes, such as infertility and recurrent miscarriage, in the context of both ...assisted and non-assisted reproduction, though the exact underlying pathophysiological reasons remain unclear. Diagnosis is based on two-dimensional and three-dimensional ultrasound, magnetic resonance imaging, or laparoscopic/hysteroscopic findings. Hysteroscopic repair of the uterine septum has been shown to confer several benefits to reproductive outcomes, though this fact remains in question, due to inconsistent and or low-quality evidence in the medical literature. An individualized approach to the treatment of infertility patients with septate uteri is imperative, given the plethora of possible underlying factors that may complicate management. In this report, we present the case of a patient with a subseptate uterus and a history of infertility, who, following hysteroscopic metroplasty, managed to conceive and ultimately successfully deliver a healthy child.
Diffuse esophageal leiomyomatosis (DEL) is a rare disorder characterized by benign hypertrophy of esophageal smooth muscle cells. No rigorous summary of available evidence on how to best manage these ...patients exists.
To define the clinical features and outcomes of pediatric patients with DEL.
A systematic literature search of the PubMed and Cochrane databases was performed with respect to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement (end-of-search date: October 6, 2018). The algorithm: "esophageal leiomyomatosis AND (children OR pediatric*)" was implemented.
Thirty-five studies including a total of 58 patients were analyzed. The female:male ratio was 1.45:1. Mean patient age was 8.54 ± 4.67 years. The most common disease manifestations were dysphagia and gastrointestinal symptoms (90.0%, 95% confidence interval CI: 78.2-96.1), followed by failure to thrive (57.9%, 95% CI: 36.2-76.9) and pulmonary symptoms (56.4%, 95% CI: 41.0-70.7). Alport syndrome (AS) was seen in 57.7% (95% CI: 44.2-70.1) of the patients. The most commonly implemented procedure was esophagectomy (85.2%;
= 46/54; 95% CI: 73.1-92.6) with gastric transposition (37.8%;
= 17/45; 95% CI: 25.1-52.4). Postoperative complications developed in 33.3% (
= 15/45; 95% CI: 21.3-48) of the patients. All-cause mortality was 7.0% (95% CI: 2.3-17.2) and disease-specific mortality was 3.5% (95% CI: 0.3-12.6).
DEL is an uncommon condition that typically occurs in the setting of AS. Esophagectomy with gastric transposition is the mainstay of treatment. Although complications develop in one-third of the patients, mortality rates are low.
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