A cross-sectional nationwide survey on esophageal atresia and tracheoesophageal fistula.

BACKGROUND: Our study aims at disclosing epidemiology and most relevant clinical features of esophageal atresia (EA) pointing to a model of multicentre collaboration. METHODS: A detailed questionnaire was sent to all Italian Units of pediatric surgery in order to collect data of patients born with EA between January and December 2012. The results were crosschecked by matching date and place of birth of the patients with those of diagnosis-related group provided by the Italian Ministry of Health (MOH). RESULTS: A total of 146 questionnaires were returned plus a further 32 patients reported in the MOH database. Basing on a total of 178 patients with EA born in Italy in 2012, the incidence of EA was calculated in 3.33 per 10,000 live births. Antenatal diagnosis was suspected in 29.5% patients. 55.5% showed associated anomalies. The most common type of EA was Gross type C (89%). Postoperative complications occurred in 37% of type C EA and 100% of type A EA. A 9.5% mortality rate was reported. CONCLUSIONS: This is the first Italian cross-sectional nationwide survey on EA. We can now develop shared guidelines and provide more reliable prognostic expectations for our patients.

Inflammatory bowel disease in children and adolescents in Italy: data from the pediatric national IBD register (1996-2003).

BACKGROUND: The purpose was to assess in Italy the clinical features at diagnosis of inflammatory bowel disease (IBD) in children. METHODS: In 1996 an IBD register of disease onset was established on a national scale. RESULTS: Up to the end of 2003, 1576 cases of pediatric IBD were recorded: 810 (52%) ulcerative colitis (UC), 635 (40%) Crohn's disease (CD), and 131 (8%) indeterminate colitis (IC). In the period 1996-2003 an increase of IBD incidence from 0.89 to 1.39/10(5) inhabitants aged <18 years was observed. IBD was more frequent among children aged between 6 and 12 years (57%) but 20% of patients had onset of the disease under 6 years of age; 28 patients were <1 year of age. Overall, 11% had 1 or more family members with IBD. The mean interval between onset of symptoms and diagnosis was higher in CD (10.1 months) and IC (9 months) versus UC (5.8 months). Extended colitis was the most frequent form in UC and ileocolic involvement the most frequent in CD. Upper intestinal tract involvement was present in 11% of CD patients. IC locations were similar to those of UC. Bloody diarrhea and abdominal pain were the most frequent symptoms in UC and IC, and abdominal pain and diarrhea in CD. Extraintestinal symptoms were more frequent in CD than in UC. CONCLUSIONS: The IBD incidence in children and adolescents in Italy shows an increasing trend for all 3 pathologies. UC diagnoses exceeded CD.

Lethal and behavioural effects of three synthetic repellents (DEET, IR3535 and KBR 3023) on Aedes aegypti mosquitoes in laboratory assays.

The knock-down, mortality and 'irritancy' effects of three synthetic repellents (DEET, IR3535 and KBR 3023) on Aedes aegypti (L) (Diptera: Culicidae) were evaluated in the laboratory in the absence of animal bait. Filter paper tests were carried out to assess the knock-down effect (KDt(50) and KDt(95)) and mortality (LC(50) and LC(95)) induced by each repellent. 'Irritancy' tests were carried out to compare the flight response (time to first take-off, or FT) to increasing concentrations of repellents (2-7%) and at five distances from the treated surface (0-40 mm). DEET had an insecticidal effect (KDt(50) = 9.7 min at 7%; CL(50)= 1165 mg/m(2)), whereas IR3535 and KBR 3023 did not. Relative to an untreated control, IR3535 was an irritant (relative irritancy or RI > 1) at doses of 5% and 7% (RI = 17.7 and 9.9, respectively), whereas DEET was an irritant at lower concentrations (RI = 12.3 at 2% DEET). KBR 3023 was the weakest irritant over the same range of concentrations (RI(max) = 3.6 at 6%). DEET was more of an irritant (RI(20) = 9.4) than IR3535 (RI(20) = 2.9) over a range of distances (0-20 mm), and KBR 3023 was not an irritant unless mosquitoes made contact with the treated surface. All three repellents had a significant effect on mosquitoes, but DEET exhibited a more complex mode of action than the others due to its insecticidal properties. The repellents do not behave as a single class of compounds with a common mode of action, but most probably affect different physiological systems in insects. The physiological and molecular mechanisms of repellents, especially DEET, should be investigated to ensure a better use of these molecules for skin applications and/or for treating materials against mosquitoes.

The inflammatory abdominal aortic aneurysm and coronary artery disease. Case report and review.

Inflammatory abdominal aortic aneurysm (IAAA) is defined as an unusually thickened aneurysmatic wall, encircled by a wide dense perianeurysmal and/or retroperitoneal fibrosis with adjacent tissues adhesion, and is now considered as an extreme shape of the common phlogistic process involved in atherosclerotic plaque formation. Latest studies demonstrated that inflammation plays an important role in coronary disease and in other atherosclerosis manifestations. We introduce the clinical case of a patient with IAAA who developed an acute myocardial infarction 6 months after the surgical procedure on the aorta. Through a literature review about IAAA we stress the clinical usefulness of the inflammatory markers as independent predictors in management of patients with coronary disease and we present the hypothesis, related to the introduced case, of an advanced coronary disease, aggravated or clinically revealed after the cytokine storm related to important localized inflammatory engagements or great vascular surgery treatments.

Electrostatic properties of bovine beta-lactoglobulin.

Bovine beta-Lactoglobulin (BLG) has been studied for many decades, but only recently structural data have been obtained, making it possible to simulate its molecular properties. In the present study, electrostatic properties of BLG are investigated theoretically using Poisson-Boltzmann calculations and experimentally following pH titration via NMR. Electrostatic properties are determined for several structural models, including an ensemble of NMR structures obtained at low pH. The changes in electrostatic forces upon changes in ionic strength, solvent dielectric constant, and pH are calculated and compared with experiments. pK(a)s are computed for all titratable sites and compared with NMR titration data. The analysis of theoretical and experimental results suggests that (1) there may be more than one binding sites for negatively charged ligands; (2) at low pH the core of the molecule is more compact than observed in the structures obtained via restrained molecular dynamics from NMR data, but loop and terminal regions must be disordered.

[Double-chamber right ventricle: a case report]. / Ventricolo destro a doppia camera: descrizione di un caso clinico.

The double-chamber right ventricle is a congenital cardiac malformation usually associated with other cardiac defects, seldom isolated and in adult subject. It is characterized by the presence of an anomalous bundle that divides the right ventricle into two chambers. The clinical and electrocardiographic signs of isolated double-chamber right ventricle are few and not specific. An echocardiographic diagnosis of isolated double-chamber right ventricle is reported. In a 18-year-old asymptomatic male with systolic murmur 2/6 at third space over the left sternal border, right ventricular hypertrophy and intraventricular conduction delay at ECG, two-dimensional echo showed an anomalous transversal muscle bundle that divided the right ventricle into two chambers, superior and inferior. Color Doppler showed a diastolic tricuspidal-like flow through a paraseptal discontinuity of the bundle and a systolic jet that reached the right atrium, with a pressure gradient of 30.9 mmHg. The absence of symptoms and other cardiopathy, without significant right outflow tract obstruction, was considered as an index of a good prognosis; therefore cardiac catheterization was not advised.

[Diastolic flow in the left ventricle of healthy subjects: a study using processing of intracardiac color-Doppler maps]. / Il flusso diastolico del ventricolo sinistro nei soggetti normali: studio mediante elaborazione delle mappe color-Doppler intracardiache.

UNLABELLED: Aim of the study was to evaluate the left ventricular (LV) diastolic flow propagation. Ten healthy subjects (28 +/- 6 years) were examined. Two-dimensional echocardiography and color-Doppler at mitral valve were performed. A cine-loop was transferred on-line to a color image processor. Selected digitized images were stored on microdisk. LV end-diastolic, end-systolic and early filling (EF) volumes were measured. Doppler measurements across the mitral valve were obtained: peak E, peak A, E/A, the integral (VTI) of E and A, acceleration and filling time. Extension of color and time base to apex, time of EF and atrial filling (AF) were obtained by M-color, along the base-apex axis. On frames of EF and AF of 2D-color, diastolic maps were processed: surface area and pixel velocity, mean (mv) and peak (pv), were obtained of the entire color area and of 4 selected areas, 2 mm wide: 1 along the annulus-apex axis (LAx), and 3 transverse, planes, at the annulus, at papillary (1/3) and at the apex (2/3 of LAx). Three-dimensional plot and velocity distribution were obtained. Values of pixel of the entire color area were stored on microdisk and processed using a Macintosh PC. For each transverse section, 1 pixel wide, mv and pv were obtained, and data were referred to normalized axis and the mean was calculated. RESULTS: end-diastolic volume index (EDVI): 66 +/- 7, end-systolic volume index (ESVI): 26 +/- 5 ml.(ABSTRACT TRUNCATED AT 250 WORDS)

[Pathophysiologic classification of heart failure: contribution of echocardiography]. / Classificazione fisiopatologica dell'insufficienza cardiaca: contributo dell'ecocardiografia.

Aim of the study was to examine the role of echocardiography to classify patients with heart failure. Fifty-seven subjects (32 dilated cardiomyopathy (DCM), 9 aortic regurgitation (AR), 16 hypertensives (HT)--4 in Class (CI) NYHA I, 24 II, 15 III, 14 IV--were studied by M-2D echo. Eighty-seven normals (N) were the control group; 11 were controlled after clinical improvement (3.4 +/- 3.8 months); 11 after worsening (12 +/- 17). We have evaluated: left ventricular diastolic dimension (LVIDd), wall thickness/radius ratio (H/R), diastolic (D) and systolic volume (S Vol), ejection fraction (EF), systolic arterial pressure/end-systolic volume ratio (P/V), and stress. LVIDd and stress were increased in all groups; H/R reduced, except in HT and in Cl I; EF and P/V reduced except in Cl I. Between I and II LVIDd was different; between II and III all parameters were different, between III and IV only EF and P/V. According to regression S-D Vol, EF-P/V and EF-stress we identify the reduction of EF and the related mechanisms, ie reduced contractility or increased afterload. Thus, according to P/V and stress, we classify the patients in 4 pathopysiologic classes: 1 and 2 with P/V within 2 SD N: 1 with stress within 2SD N, EF and H/R normal; 2 with stress greater than +2SD, H/R normal and EF reduced; 3 and 4 with P/C less than -2 SD N: 3 with normal, 4 with stress greater than +2 SD. In the 1 and 2, 1 out 14 is in III Cl NYHA, none in IV; in 3,6 out 8 are in II, in 4, 9 out 35 are in Cl less than III. In the follow-up, in 8 of the improved patients, EF and P/V were increased and stress reduced; in 3, EF was reduced. In 8 of the worsened, EF and P/V were reduced, LVIDd and stress increased; in 3 EF and LVIDd were increased, P/V reduced. This study demonstrates discordance between Cl NYHA and echo, and how classification of NYHA does not give information about the several components of heart failure. However LVID and EF are not sufficient. By a correlation of echo-parameters of contractility, afterload and pump performance, we may suggest a classification of heart failure in pathophysiologic classes.

Comparison of muzolimine and furosemide in heart failure.

Comparative and randomized evaluation of 30 mg muzolimine and 40 mg furosemide was assessed in 18 patients with CHF. Muzolimine is slightly more effective than furosemide with regard to total 24-hour urine excretion, and a significant difference was found in the time-response curve. In fact the maximum rate of diuresis occurred at the second hour with muzolimine and at fourth hour with furosemide. Both drugs were well tolerated and no side-effects were observed.