ADVANCED LYPOSARCOMA MYXOIDES OF THE EXTREMITY

Sarcomas are soft tissue tumors arising from primitive mesenchyme. Small incidence (4-5/100 000 in Europe) is the reason their pathogenesis is relatively unknown. Patient (38) complained of a growth on the upper part of right thigh, the size of a child’s head. A magnetic resonance imaging (MRI) scan was done and tumefaction was described: oval shape, 13.5 x 7.5 x 11cm in diameter, without infiltration of surrounding tissue. The tumor was surgically removed and was pathohistologically verified as low grade liposarcoma myxoides. After initial presentation the patient presented a series of recurrences and metastases in the abdominal wall, extremities and  liver. Soft tissue metastasis from the lower extremities to the liver occur in 0.5% of cases and they are usually small and multiple, as in the presented patient. The European Sarcoma Medical Oncologist’s guide recommends that every  extremity tumor larger than 5cm and suspicious of malignancy should be evaluated using biopsy and imaging methods. A biopsy should be done before excision under ultrasound or computerized tomography (CT) guidance with the goal of planning the best therapy protocol and prevention of a generalized disease with metastases. The recommended imaging method is an MRI scan, although sarcomas can have a benign presentation.  Standard therapy includes surgical resection with local radiotherapy.  Liposarcoma myxoides, a rare soft tissue tumor, demands biopsy and complete surgical removal with detailed and continuous postoperative imaging follow-up and oncological therapy. The therapeutic goal is to increase survival and preserve extremity function

Presentation of pericardial hydatid cyst as acute cardiac tamponade

SummaryWe report a case of a 47-year-old man with isolated pericardial hydatid cyst (without myocardial involvement) that presented as acute pericardial tamponade. After initial investigation and transthoracic echocardiography, emergent pericardial drainage was performed for downgrading the urgency of a definitive treatment for a hydatid cyst. A computed tomography examination after the pericardial drainage showed a pericardial cyst without heart muscle involvement, making the treatment possible through anterior thoracotomy and without performing cardiopulmonary bypass. Complete surgical removal of the cyst was performed. The postoperative course was uneventful. The patient received postoperative albendazole treatment. He remained asymptomatic and no recurrence was observed during a 1-year follow-up period

HYPERLIPIDEMIA IN ACUTE PANCREATITIS: CONCOMITANT DISORDER OR A CAUSE?

Acute pancreatitis is a common condition with alcohol and gallstones being the most frequent etiologies. The aim of our study was to determine the prevalence of hyperlipidemia, its etiopathogenetic role and influence on outcomes in patients with acute pancreatitis.The study included 47 patients admitted to our clinic for acute pancreatitis during one year period. On admission patients with hyperlipidemia were compared to those without it, regarding following parameters: body mass index, Glasgow score, organ failure occurrence, local complications occurrence (pancreatic necrosis, pseudocyst, abscess, jaundice, gastric outlet syndrome), intensive care unit stay and death. The results of the study revealed high incidence of hyperlipidemia in 51% of examined acute pancreatitis patients with the prevalence of severe forms in more than half of these patients. Dominant lipid disorder was hypertriglyceridemia, followed by hypercholesterolemia. It was clearly demonstrated that patients with hyperlipidemia, especially hypertriglyceridemia, had more severe acute pancreatitis, higher incidence of complications and poorer outcome compared to normolipemic patients. Hyperlipidemia in patients with acute pancreatitis should be considered and treated by a clinician as a separate serious problem, both when being a cause and a concomitant disorder. Hypolipidemic therapy should be administered both in urgent acute pancreatitis settings and as a long-term treatment aimed to prevent inflammation recurrence by successful persistent serum lipid levels control.Key words: Hyperlipidemia, acute pancreatitis

Regional differences among female patients with heart failure from the Cardiac Insufficiency BIsoprolol Study in ELDerly (CIBIS-ELD)

Background: The aim of our study was to examine regional differences in the demographics, etiology, risk factors, comorbidities and treatment of female patients with heart failure (HF) in the Cardiac Insufficiency BI soprolol Study in ELDerly (CIBIS-ELD) clinical trial.Methods and results: One hundred and fifty-nine female patients from Germany and 169 from Southeastern (SE) Europe (Serbia, Slovenia and Montenegro) were included in this subanalysis of the CIBIS-ELD trial. Women comprised 54% of the study population in Germany and 29% in SE Europe. German patients were significantly older. The leading cause of HF was arterial hypertension in German patients, 71.7% of whom had a preserved ejection fraction. The leading etiology in SE Europe was the coronary artery disease; 67.6% of these patients had a reduced left ventricular ejection fraction (34.64 ± 7.75%). No significant differences were found in the prevalence of traditional cardiovascular risk factors between the two regions (hypertension, diabetes, hypercholesterolemia, smoking and family history of myocardial infarction). Depression, chronic obstructive pulmonary disease and malignancies were the comorbidities that were noted more frequently in the German patients, while the patients from SE Europe had a lower glomerular filtration rate. Compared with the German HF patients, the females in SE Europe received significantly more angiotensin converting enzyme inhibitors, loop diuretics and less frequently angiotensin receptor blockers and mineralocorticoid receptor antagonists.Conclusions: Significant regional differences were noted in the etiology, comorbidities and treatment of female patients with HF despite similar risk factors. Such differences should be considered in the design and implementation of future clinical trials, especially as women remain underrepresented in large trial populations.

Genes that determine immunology and inflammation modify the basic defect of impaired ion conductance in cystic fibrosis epithelia

BACKGROUND: The cystic fibrosis (CF) basic defect, caused by dysfunction of the apical chloride channel CFTR in the gastrointestinal and respiratory tract epithelia, has not been employed so far to support the role of CF modifier genes. METHODS: Patients were selected from 101 families with a total of 171 F508del-CFTR homozygous CF patients to identify CF modifying genes. A candidate gene based association study of 52 genes on 16 different chromosomes with a total of 182 genetic markers was performed. Differences in haplotype and/or diplotype distribution between case and reference CF subpopulations were analysed. RESULTS: Variants at immunologically relevant genes were associated with the manifestation of the CF basic defect (0.01<Praw<0.0001 at IL1B, TLR9, TNFalpha, CD95, STAT3 and TNFR). The intragenic background of F508del-CFTR chromosomes determined disease severity and manifestation of the basic defect (Praw=0.0009). Allele distributions comparing transmitted and non-transmitted alleles were distorted at several loci unlinked to CFTR. CONCLUSIONS: The inherited capabilities of the innate and adaptive immune system determine the manifestation of the CF basic defect. Variants on F508del-CFTR chromosomes contribute to the observed patient-to-patient variability among F508del-CFTR homozygotes. A survivor effect, manifesting as a transmission disequilibrium at many loci, is consistent with the improvement of clinical care over the last decades, resulting in a depletion of risk alleles at modifier genes. Awareness of non-genetic factors such as improvement of patient care over time is crucial for the interpretation of CF modifier studies

Identification of a rare p.G320R alpha-1-antitrypsin variant in emphysema and lung cancer patients

The alpha-1-antitrypsin (A1AT) gene is highly polymorphic, with more than 100 genetic variants identified of which some can affect A1AT protein concentration and/or function and lead to pulmonary and/or liver disease. This study reports on the characterization of a p.G320R variant found in two patients, one with emphysema and the other with lung cancer. This variant results from a single base-pair substitution in exon 4 of the A1AT gene, and has been characterized as P by isoelectric focusing. Functional evaluation of the A1AT p.G320R variant was through comparing specific trypsin inhibitory activity in two patients with pulmonary disorders, carriers of the p.G320R variant, and 19 healthy individuals, carriers of normal A1AT M variants. Results showed that specific trypsin inhibitory activity was lower in both emphysema (2.45 mU/g) and lung cancer (2.07 mU/g) patients than in carriers of the normal variants (range 2.51-3.71 mU/g). This rare A1AT variant is associated with reduced functional activity of A1AT protein. Considering that it was found in patients with severe pulmonary disorders, this variant could be of clinical significance

Surgical site infection after gastrointestinal surgery in high-income, middle-income, and low-income countries: a prospective, international, multicentre cohort study

Background: Surgical site infection (SSI) is one of the most common infections associated with health care, but its importance as a global health priority is not fully understood. We quantified the burden of SSI after gastrointestinal surgery in countries in all parts of the world. Methods: This international, prospective, multicentre cohort study included consecutive patients undergoing elective or emergency gastrointestinal resection within 2-week time periods at any health-care facility in any country. Countries with participating centres were stratified into high-income, middle-income, and low-income groups according to the UN's Human Development Index (HDI). Data variables from the GlobalSurg 1 study and other studies that have been found to affect the likelihood of SSI were entered into risk adjustment models. The primary outcome measure was the 30-day SSI incidence (defined by US Centers for Disease Control and Prevention criteria for superficial and deep incisional SSI). Relationships with explanatory variables were examined using Bayesian multilevel logistic regression models. This trial is registered with ClinicalTrials.gov, number NCT02662231. Findings: Between Jan 4, 2016, and July 31, 2016, 13 265 records were submitted for analysis. 12 539 patients from 343 hospitals in 66 countries were included. 7339 (58·5%) patient were from high-HDI countries (193 hospitals in 30 countries), 3918 (31·2%) patients were from middle-HDI countries (82 hospitals in 18 countries), and 1282 (10·2%) patients were from low-HDI countries (68 hospitals in 18 countries). In total, 1538 (12·3%) patients had SSI within 30 days of surgery. The incidence of SSI varied between countries with high (691 [9·4%] of 7339 patients), middle (549 [14·0%] of 3918 patients), and low (298 [23·2%] of 1282) HDI (p < 0·001). The highest SSI incidence in each HDI group was after dirty surgery (102 [17·8%] of 574 patients in high-HDI countries; 74 [31·4%] of 236 patients in middle-HDI countries; 72 [39·8%] of 181 patients in low-HDI countries). Following risk factor adjustment, patients in low-HDI countries were at greatest risk of SSI (adjusted odds ratio 1·60, 95% credible interval 1·05–2·37; p=0·030). 132 (21·6%) of 610 patients with an SSI and a microbiology culture result had an infection that was resistant to the prophylactic antibiotic used. Resistant infections were detected in 49 (16·6%) of 295 patients in high-HDI countries, in 37 (19·8%) of 187 patients in middle-HDI countries, and in 46 (35·9%) of 128 patients in low-HDI countries (p < 0·001). Interpretation: Countries with a low HDI carry a disproportionately greater burden of SSI than countries with a middle or high HDI and might have higher rates of antibiotic resistance. In view of WHO recommendations on SSI prevention that highlight the absence of high-quality interventional research, urgent, pragmatic, randomised trials based in LMICs are needed to assess measures aiming to reduce this preventable complication

Recycling and Reinforcing Intimomedial Flap of the infrarenal Aorta using Anterior Longitudinal Ligament in Patients with acute Trauma with Bowel Injuries

We present a patient with blunt abdominal trauma with severe acute right limb ischemia and clinical signs of diffuse peritonitis. Computed tomography angiography showed circumferential dissection of the infrarenal aorta with occlusion of the right common iliac artery. We opted for simultaneous abdomen exploration and open repair of injured aorta. Critical weakening of the aortic wall with imminent rupture was identified intraoperatively. Aortotomy cranially from bifurcation showed circumferential intimomedial dissection. The fixation of fragile intimomedial flap of aortic dissection was achieved with reinforcement using an anterior longitudinal ligament. The long aortoiliac arteriotomy was repaired using a great saphenous vein patch. Patient had uneventful postoperative course and was discharged after 7 days. In patients with abdominal polytrauma with peritonitis, and no available endovascular tools, open surgery for circumferential aortic dissection is possible and successful. Described reinforcement of the posterior aortic wall to the anterior longitudinal ligament should be added to the armamentarium of aortic injury treatment