Isolated Primary Pancreatic Wegener’s Granulomatosis: Report of Two Cases

Context Chronic inflammatory vasculitis, namely Wegener's granulomatosis, is an autoimmune disease predominantly affecting the respiratory tract and rarely the mucosa of the upper or lower gastrointestinal tract. We report two cases of isolated pancreatic manifestation of Wegener’s granulomatosis, mimicking a tumor. Case Reports Two patients, a 57-year-old man and a 68-year-old woman, presented with isolated lesions thought to be pancreatic adenocarcinoma and both underwent surgical resection. On pathology, the lesions were found to be a granulomatous vasculitis and were considered to represent isolated pancreatic Wegener’s granulomatosis, in the absence of other clinical features of the disease. Conclusions Benign diseases such as Tuberculosis may mimic pancreatic tumours diagnosed on cross-sectional imaging. Wegener’s granulomatosis should be included in the differential diagnosis of an isolated pancreatic mass, in the absence of other cardinal symptoms for malignancy, due to the extensive surgery and risks involved in resection of pancreas.Image: CT image depicting a bulky “soft tissue density” mass of the tail of the pancreas

What is the best timing of surgery in patients with post-infarct ventricular septal rupture?

A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was ‘in which patients with a post-infarct ventricular septal rupture (PIVSR) might immediate surgery give better results than delayed surgery in terms of mortality’? Altogether, 88 papers were found using the reported search criteria, of which 6 represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated. The recommendations are based on outcomes from 3238 patients undergoing surgery for PIVSR. Mean age was 67.5 ± 8.8 (40–88 years). Left ventricular function was compromised in most patients with mean ejection fraction of 40%. All papers carried out univariate and/or multivariate analyses of variables that contributed to different in-hospital mortalities. Early surgery, i.e. from >3 days to within 4 weeks after MI, had an overall in-hospital mortality of 52.4%; delayed surgery, typically from 1 week to after 4 weeks post-myocardial infarction, had an overall operative in-hospital mortality of 7.56%. Most authors observe that a shorter time between rupture and surgery is an unfavourable predictor of outcome independent of haemodynamic status. The consensus was that nearly all patients with PIVSR, particularly if >15 mm diameter with a significant shunt and resultant haemodynamic deterioration, should undergo early surgical repair. The precise timing of surgery depends on patients' haemodynamic status. Exclusion from surgery should be considered if life expectancy or quality is severely limited by another limiting underlying pathology. If the patient is in cardiogenic shock, due to pulmonary to systemic blood flow ratio shunt rather than infarct size, immediate surgery should follow resuscitation measures and cardiac support. If the patient is haemodynamically stable, surgery could be performed after 3–4 weeks of medical optimization with inotropic and mechanical cardiac support. If there is clinical deterioration, immediate surgery is indicated

Physical activity and liver disease affect the fat free mass in adolescents with cystic fibrosis

Cystic fibrosis (CF) is predominantly a lung disease but is also characterised by impaired skeletal muscularity and a reduction in fat-free mass. We aimed to test the hypothesis that clinical and anthropometric parameters would determine fat-free mass impairment in adolescents with CF. We measured the fat-free mass index (FFMI) using bioelectrical impedance, the lung function using spirometry, the number of shuttles as a measure of exercise tolerance and the reported physical activity in children and young people with CF in a tertiary centre at King’s College Hospital, London, UK. CF-related liver disease was diagnosed by abnormal liver enzymes and/or ultrasonography. We studied 28 children and young people (11 male) with a median (interquartile range (IQR)) age of 15 (13–17) years. They had a median (IQR) FFMI of 13.5 (11.6–15.1) kg/m(2). The FFMI significantly correlated with age (rho = 0.568, p = 0.002), number of shuttles (rho = 0.691, p < 0.001) and reported hours of activity per day (rho = 0.426, p = 0.024). The median (IQR) FFMI was significantly higher in male [15.1 (13.1–18.6) kg/m(2)] compared to female participants [12.7 (11.6–14.1) kg/m(2), p = 0.008]. The median (IQR) FFMI was significantly lower in the 10 (36%) participants with liver disease [11.9 (11.5–13.4) kg/m(2)] compared to the FFMI in the remaining 18 participants without liver disease [14.4 (12.5–15.9) kg/m(2), p = 0.027]. Conclusion: Fat-free mass increases with increasing age and growth in adolescents with CF. Physical activity exerts a beneficial effect on fat-free mass, and CF-related liver disease negatively affects fat-free mass in adolescents with CF