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/m2. 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/m2] compared to female participants [12.7 (11.6-14.1) kg/m2, 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/m2] compared to the FFMI in the remaining 18 participants without liver disease [14.4 (12.5-15.9) kg/m2, 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. WHAT IS KNOWN: • Health behaviours in adolescence influence lifelong health in cystic fibrosis (CF). • A normal body mass index in CF might fail to reveal a low fat-free mass (FFM), and quality of life in CF is strongly associated with a reduced FFM. WHAT IS NEW: • FFM increases with increasing age and growth in adolescents with CF. • Physical activity exerts a beneficial effect, and liver disease negatively affects FFM in adolescents with CF.

Nutritional status and pulmonary outcome in children and young people with cystic fibrosis.

BACKGROUND: Nutrition is closely related to mortality and pulmonary and respiratory muscle function in cystic fibrosis (CF) patients. We initially validated results from a bioelectrical impedance device against dual energy x-ray absorptiometry (DEXA). We then determined whether fat free mass assessed by a portable impedance device rather than body mass index (BMI) better correlated with pulmonary function, respiratory muscle strength and exercise capacity in CF patients. METHODS: Eighteen young people and adults (median age 19, range 12-39 years) with CF had dual energy X-ray absorptiometry and direct segmental multi-frequency impedance analysis. Body composition, pulmonary function, respiratory muscle function and exercise tolerance using the impedance device were measured in 29 young people with CF with median age 15 (range 12-19) years. MAIN FINDINGS: There was a significant correlation between impedance and absorptiometry results (r2 = 0.947). Fat free mass correlated with the forced vital capacity z-score (r = 0.442, p = 0.016), maximal inspiratory pressure (r = 0.451, p = 0.014) and exercise tolerance (r = 0. 707, p < 0.001). BMI z-scores did not significantly correlate with pulmonary or respiratory muscle function. Subjects with a fat free mass z-score of ≤2 had a lower forced expiratory volume in 1 s z-score (p = 0.007), lower forced vital capacity z-score (p = 0.001), higher residual volume z-score (p = 0.042), lower maximal inspiratory pressure (p = 0.039), more days of intravenous antibiotics per year (p = 0.016) and a higher rate of chronic infections (p = 0.006). PRINCIPAL CONCLUSIONS: Fat-free mass measured by impedance correlated better with pulmonary and respiratory muscle function and exercise capacity than BMI.

The roles of drug therapy given via the endotracheal tube to neonates.

Many drugs are given to intubated neonates by the inhalation route. The optimum aerosol delivery system, however, has not been identified and there are many challenges in delivering drugs effectively to the lower airways of intubated neonates. The effectiveness of surfactant in prematurely born infants and nitric oxide has been robustly investigated. Other drugs are being used on very limited evidence.

The treatment of combined trigeminal and glossopharyngeal neuralgia by glycerol rhizolysis of the trigeminal ganglion.

A 78-year-old man with combined trigeminal and glossopharyngeal neuralgia underwent glycerol rhizolysis of the trigeminal ganglion. The treatment led to the immediate relief of both neuralgias. We discuss the potential mechanism of this unexpected therapeutic effect with reference to the pathophysiology of trigeminal and glossopharyngeal neuralgia.

Successful treatment of failing biological prosthesis because of "Stent creep" with valve-in-valve transcatheter aortic valve implantation.

INTRODUCTION: Stent creep is an uncommon mode of structural deterioration of bioprosthetic heart valves defined by a permanent inward deflection of the stent posts. This may occur because of valve over-sizing and leads to intrinsic valve stenosis. It has been described in older generation of bioprosthesis and was thought not to occur in modern devices. METHODS: We describe three patients who were referred for bioprosthetic valve degeneration with presumed aortic stenosis. Investigations demonstrated mid valvular gradient predominantly because of stent creep. We performed valve-in-valve TAVI with Edward SAPIEN prosthesis. RESULTS: Median age was 84 and logistic EuroSCORE 34.4. All patients had degenerated bioprosthesis with mean implant duration of 5.6 years. Two patients had Carpentier Edwards Perimount prosthesis (19 and 23 mm) and one patient had a Mitroflow (21 mm). Mean gradients were 33, 54, and 22 mm Hg. About 23 mm Edward SAPIEN valve was implanted in all cases with immediate improvement in haemodynamics with mean gradient reduction to 10, 17, and 8 mm Hg, respectively. The mean aortic valve area increased from 0.63 to 1.76cm(2) . There were no serious adverse events. The patients improved from NYHA III/IV to I/II post procedure and remain well at median follow-up of 24-months. DISCUSSION: Stent creep is an uncommon mode of structural deterioration in bioprosthetic heart valves. It has been described in the previous generation of bioprosthesis. It is important to distinguish leaflet dysfunction and stent creep. By forcing the stent posts outwards a balloon expandable TAVI device can be used to treat this condition.

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.

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.