The FODRAT study (FOod addiction, DRugs, Alcohol and Tobacco): first data on food addiction prevalence among patients with addiction to drugs, tobacco and alcohol.

OBJECTIVES: The main focus of this study was to evaluate the prevalence of food addiction (FA) in a population of 575 subjects, all affected by drugs, alcohol and/or tobacco addiction. METHODS: Patients were enrolled in Addiction Service Centers and 25 items YFAS questionnaire was administered. Prevalence of FA was studied among patients who already have an addiction and then this prevalence was compared between groups of abusers (by type of substance), comparing mono abusers with polyabusers, as well as regressions by age, BMI, sex, through multiple regression analysis. RESULTS: Prevalence of FA in the sample is 20.17%. Risk of FA increases with the number of substances used (polyabuse). Results show a positive correlation, in addicted people, between BMI values and FA, with significant values (OR 1.08; 95% CI 1.04-1.13; p = 0.006). Age is inversely correlated with FA (OR 0.97; 95% CI 0.95-0.99; p = 0.01). Female sex is positively associated (OR 2.60; 95% CI 1.59-4.27, p = 0.000). No significant association appears with any substance, even if the highest prevalence is recorded among cannabis users (31.03%), and heroin (21.07%), followed by cocaine (18.53%), alcohol (14.49%) and tobacco (11.61%). A comparison between the FA prevalence in our study and that from another study in the Italian general population (11%) shows a significant difference (p < 0.001). CONCLUSIONS: Prevalence of FA among addicted people is greater than in the general population. Risk of FA increases with the increase in number of used substances (polyabuse). Age is inversely correlated with FA. There is a positive and significant correlation between BMI and FA among substance/tobacco abusers. LEVEL OF EVIDENCE: Level V, observational cross-sectional descriptive study.

Real-time cellular impedance monitoring and imaging of biological barriers in a dual-flow membrane bioreactor.

The generation of physiologically relevant in-vitro models of biological barriers can play a key role in understanding human diseases and in the development of more predictive methods for assessing toxicity and drug or nutrient absorption. Here, we present an advanced cell culture system able to mimic the dynamic environment of biological barriers while monitoring cell behaviour through real-time impedance measurements and imaging. It consists of a fluidic device with an apical and a basal flow compartment separated by a semi-permeable membrane. The main features of the device are the integration of sensing through transepithelial electrical impedance (TEEI) measurements and transparent windows for optical monitoring within a dual flow system. Caco-2 cells were cultured in the TEEI bioreactor under both flow and static conditions. Although no differences in the expression of peripheral actin and occludin were visible, the cells in dynamic conditions developed higher impedance values at low frequencies, indicative of a higher paracellular electrical impedance with respect to the static cultures. TEEI measurements at high frequency also enabled monitoring monolayer formation, which can be correlated with the observation of an RC behaviour in the impedance spectra. In particular, the cells subject to flow showed accelerated barrier formation and increased vitality with respect to the static controls, again highlighting the importance of dynamic conditions for epithelial cells.

Aging phenotype(s) in kidneys of diabetic mice are p66ShcA dependent.

The p66ShcA protein controls cellular responses to oxidative stress, senescence, and apoptosis. Here, we test the hypothesis that aging phenotype(s) commonly associated with the broad category of chronic kidney disease are accelerated in diabetic kidneys and linked to the p66ShcA locus. At the organ level, tissue stem cells antagonize senescent phenotypes by replacing old dysfunctional cells. Using established methods, we isolated a highly purified population of stem cell antigen-1-positive mesenchymal stem cells (Sca-1+ MSCs) from kidneys of wild-type (WT) and p66 knockout (p66 KO) mice. Cells were plated in culture medium containing normal glucose (NG) or high glucose (HG). Reactive oxygen species (ROS) metabolism was substantially increased in WT MSCs in HG medium in association with increased cell death by apoptosis and acquisition of the senescent phenotype. DNA microarray analysis detected striking differences in the expression profiles of WT and p66 KO-MSCs in HG medium. Unexpectedly, the analysis for p66 KO-MSCs revealed upregulation of Wnt genes implicated in self-renewal and differentiation. To test the in vivo consequences of constitutive p66 expression in diabetic kidneys, we crossed the Akita diabetic mouse with the p66KO mouse. Homozygous mutation at the p66 locus delays or prevents aging phenotype(s) in the kidney that may be precursors to diabetic nephropathy.

Hypoxia-induced apoptotic cell death is prevented by oestradiol via oestrogen receptors in the developing central nervous system.

The neuroprotective effects of oestrogens have been demonstrated against a variety of insults, including excitotoxicity, oxidative stress and cerebral ischemia under certain conditions. However, the molecular mechanisms underlying oestrogen neuroprotection are still unclear. We aimed to determine whether 17beta-oestradiol (E(2)) administration post-hypoxia (p-hx) was neuroprotective and whether these actions were mediated through oestrogen receptors (ER). For this purpose, 12-embyonic day-old chickens were subjected to acute hypoxia [8% (O(2)), 60 min], followed by different reoxygenation periods. To test the neuroprotective effect of E(2) and its mechanism, embryos were injected 30 min after the end of hypoxia with E(2) alone or with ICI 182 780, a competitive antagonist of ER. Cytochrome c (cyt c) release, an indicator of mitochondrial apoptotic pathway, was measured by western blot in optic lobe cytosolic extracts. DNA fragmentation by TUNEL fluorescence and caspase-3 fragmentation by immunofluorescence were detected on optic lobe sections. Acute hypoxia produces a significant increase in cyt c release from mitochondria at 4 h p-hx, followed by an increase in TUNEL positive cells 2 h later (6 h p-hx). Administration of E(2) (0.5 mg/egg) produced a significant decrease in cytosolic cyt c levels at 4 h p-hx, in caspase-3 activation and in TUNEL positive cells at 6 h p-hx compared to vehicle treated embryos. In the E(2)-ICI 182 780 treated embryos, cyt c release, caspase-3 fragmentation and TUNEL positive cells were similar to the hypoxic embryos, thus suggesting the requirement of an E(2)-ER interaction for E(2) mediated neuroprotective effects. In conclusion, E(2) prevents hypoxia-induced cyt c release and posterior cell death and these effects are mediated by oestrogen receptors.

Value of image fusion in the staging of prostatic carcinoma.

PURPOSE: We assessed the value of image fusion in the staging of prostatic cancer in a series of 32 patients who underwent preoperative evaluation with transrectal colour-Doppler ultrasonography (TRUS) and magnetic resonance imaging (MRI). MATERIALS AND METHODS: Colour-Doppler TRUS exams were performed using a 7.5-MHz biplanar probe. MRI exams were done with a scanner operating at 1.5 Tesla (T) using an endorectal coil. All patients underwent radical prostatectomy within 2 weeks from the imaging assessment. Whole-mount sections were prepared from the surgical specimens and were subsequently digitised by using a high-resolution scanner. The Digital Imaging and Communications in Medicine (DICOM) TRUS and MR images as well as the digitised pathological images were transferred to a graphic workstation to perform image fusion. RESULTS: Image fusion was technically possible in 25/32 cases in which axial TRUS images were available. The following fusion images were obtained: TRUS + pathological sections; MRI + pathological sections; TRUS + MRI + pathological sections. The final pathological staging concerning the T status was: four pT2b, fourteen pT2c, three pT3a and four pT3b. The three types of image fusion led to the following results: TRUS + pathological sections, correct staging in 20/25 cases (accuracy 80%); MRI + pathological sections, correct staging in 22/25 cases (accuracy 88%); TRUS + MRI + pathological sections, correct staging in 23/25 cases (accuracy 92%). CONCLUSIONS: Our study suggests that by using image fusion between colour-Doppler TRUS and endorectal MRI, it is possible to improve the accuracy of pathological staging in patients who are candidates for radical prostatectomy.

Pneumatosis cystoides intestinalis: imaging findings with colonoscopy correlation.

We report on a case of pneumatosis cystoides intestinalis in a 32-year-old woman affected by coeliac disease with stool cultures positive for salmonella. After plain film of the abdomen, patient underwent computed tomography and magnetic resonance colonography studies that demonstrated numerous, diffuse bubblelike intramural gas collections into the ascending, transverse and descending colonic wall. CT and MR findings were correlated with colonoscopy.

Dyspnoea and hypoxaemia after lung surgery: the role of interatrial right-to-left shunt.

After lung surgery, some patients complain of unexplained increased dyspnoea associated with hypoxaemia. This clinical presentation may be due to an interatrial right-to-left shunt despite normal right heart pressure. Some of these patients show postural dependency of hypoxaemia, whereas others do not. In this article, the pathogenesis and mechanisms involved in this post-surgical complication are discussed, and the techniques used for confirmation and localisation of shunt are reported. An invasive technique, such as right heart catheterisation with angiography, was often used in the past as the diagnostic procedure for the visualisation of interatrial shunt. As to noninvasive techniques, a perfusion lung scan may be used as the first approach as it may detect the effect of the right-to-left shunt by visualising an extrapulmonary distribution of the radioactive tracer. The 100% oxygen breathing test could also be used to quantify the amount of right-to-left shunt. Particular emphasis is given to newer imaging modalities, such as transoesophageal echocardiography, which is minimally invasive but highly sensitive in clearly visualising the atrial septum anatomy. Finally, the approach to closure of the foramen ovale or atrial septal defect is discussed. Open thoracotomy was the traditional approach in the past. Percutaneous closure has now become the most used and effective technique for the repair of the interatrial anatomical malformation.

Hepatocellular adenoma: imaging findings and pathological correlation.

A 45-year-old man presented with a 1-week history of gastrooesophageal reflux, epigastric discomfort and abdominal pain especially in the right hypochondrium. Abdominal ultrasonography, contrast-enhanced spiral computed tomography and magnetic resonance imaging of the abdomen were performed. Eventually, imaging findings were correlated with histopathological analysis, which confirmed the diagnosis of a 18 cm x 16 cm hepatocellular adenoma.

Tyrosine phosphorylation of focal adhesion kinase by PDGF is dependent on ras in human hepatic stellate cells.

Focal adhesion kinase (FAK) is a widely expressed nonreceptor tyrosine kinase found in focal adhesions. FAK has been indicated as a point of convergence of other signaling pathways including platelet-derived growth factor (PDGF) receptors, and recently, FAK tyrosine phosphorylation has been shown to be stimulated by PDGF. In the present study we assessed the role of Ras as a possible intermediate protein regulating PDGF-induced FAK tyrosine phosphorylation in human hepatic stellate cells (HSCs), liver-specific pericytes primarily involved in the pathogenesis of liver fibrosis. For this purpose, cells were first subjected to retroviral-mediated gene transfer with a dominant-negative mutant of Ras (N17Ras). This resulted in a marked inhibition of PDGF-induced FAK tyrosine phosphorylation together with the expected reduction of PDGF-induced extracellular signal-regulated kinase activity (ERK). Afterward, the effects of pharmacological agents potentially affecting Ras isoprenylation were evaluated. PDGF-induced FAK tyrosine phosphorylation, ERK activity and intracellular calcium increase, as well as the biological effects of this growth factor, (i.e., mitogenesis and cell migration) were effectively blocked by GGTI-298, an inhibitor of geranylgeranyltransferase I. Inhibition of Ras processing obtained with FTI-277, an inhibitor of farnesyltransferase, resulted in detectable effects only at high doses. Taken together, these results establish that Ras operates as a protein-linking PDGF-beta receptor to FAK in human HSCs, and that signaling molecules requiring geranylgeranylation may also be involved in this process.

Long-term follow-up of stents implanted to relieve peripheral pulmonary arterial stenosis: hemodynamic findings and results of lung perfusion scanning.

In recent years, percutaneous placement of stents has been used as an alternative to surgery or balloon angioplasty for the treatment of adults with peripheral pulmonary arterial stenosis. This therapy has also been proposed for children, but questions still remain about its indications in this group of patients. We describe here the results of intravascular placement of stents in a group of 29 patients, with a mean age of 12+/-7 (range 3-31) years and weighing 35+/-19 (range 11-74) kg. All were affected by postsurgical or congenital isolated pulmonary arterial stenosis, and have now been followed for 38+/-19 (range 6-65) months. The early hemodynamic results have been excellent, with a significant reduction of the pulmonary arterial systolic pressure, the systolic pressure gradient, and the ratio of systolic pressures in the pulmonary and systemic circuits, and with a significant increase of the diameter of the stented vessels in all the patients. Of the 29 patients, 24 have been recatheterized 18+/-10 months after the procedure, demonstrating the stability of the results, with a low incidence of late restenosis, this seen in only 1 patient (2%). Lung perfusion scanning, performed in 17 patients each year after the follow-up catheterization, has showed that the results are maintained at long-term follow-up (51+/-9 months).

[20 years of biological monitoring (1976-1995) of occupational lead exposure in various Tuscan provinces]. / Venti anni di sorveglianza biologica (1976-1995) dell'esposizione professionale a piombo in alcune provincie toscane.

We report the results of 24,475 blood lead determinations (PbB) performed by the Occupational Toxicology Laboratory of Florence between 1976 and 1995. Biological monitoring was carried out in 383 factories localized in the districts of Florence, Prato, Pistoia, Lucca and Pisa. The most represented production fields were colouring ceramic factories, artistic ceramics, transfer-pictures for ceramics, glass factories, typographies, glass decoration factories, chemical factories. In twenty years, the median values of PbB decreased from 48 to 17 micrograms/100 ml in males and from 40 to 8 micrograms/100 ml in females. The 95 degrees centile ranged from 84 to 46 micrograms/100 ml for males and from 63 to 42 micrograms/100 ml for females. In the 1988-95 period, we observed an increase of both the 95 degrees centile and the maximal values because of the inclusion of data from workers employed in factories where the "decorazione a scavo" technique, which is characterized by elevated environmental lead concentrations, was used. In the period 1991-93, the ranges of PbB observed in glass decoration factories were 13-160 micrograms/100 ml for males and 4-80 micrograms/100 ml for females, respectively.

Multiple exposure to arsenic, antimony, and other elements in art glass manufacturing.

Art glass manufacturing is one of the most interesting examples of exposure to complex mixtures. Among the raw materials used are silica sand, borax, carbonates, nitrates of Ca, Na, K, and a great number of compounds that are mainly oxides of As, Sb, Al, Zn, Cr, Ni, Sn, Se, Cd, Mn, Cu, Co, Fe, Nd, Er, Eu, and La. In six art glass factories that use As or Sb as fining agents, the exposure to these elements was investigated in 32 workers by means of environmental and biological monitoring. Analysis was conducted by atomic absorption spectrometry (AAS) and inductively coupled plasma mass spectrometry (ICP-MS). The results confirmed that As, which is the main carcinogen in glass production, reaches high air concentrations and is generally above the American Conference of Governmental Industrial Hygienists (ACGIH) threshold limit value-time-weighted average (TLV-TWA) of 10 micrograms/m3. When partly substituted by antimonial compounds, As air concentrations dropped dramatically, while the air levels of Sb (which is considered less toxic but is classified as a class 2B carcinogen by IARC) were relatively low and below the ACGIH TLV-TWA of 500 micrograms/m3. Exposure to As and Sb also differed in the three types of jobs investigated: As was high in oven chargers, Sb was higher in batch mixers, and both elements were low in makers-formers. Eleven to 18 elements were detected: arsenic, Al, Ba, Sb, Pb, and Zn were the elements most frequently measured (in tens, and in some cases hundreds, of micrograms/m3), followed by B, Li, Mn, Se, Sn, Sr, Ce, La, and Nd (in micrograms or in some cases tens of micrograms/m3. The results of biological monitoring for As, Sb, and other elements were in agreement with environmental monitoring data. We concluded that multiple detection of elements is a useful tool (or the evaluation of exposure to complex mixtures such as those used in the art glass industry and that such detection also allows a more accurate evaluation of related epidemiological data.

[A cohort study of art glass workers in the Empoli area]. / Studio di coorte dei lavoratori del vetro artistico nel territorio empolese.

The investigation aimed at studying cause-specific mortality of art glass workers employed in 17 industrial facilities in Tuscany, Italy. A cohort of 3390 workers employed for at least 1 year was obtained from company payrolls. Follow-up was between the year each factory started operations, mostly in the mid-fifties, and the end of 1993. The cause specific expected mortality was computed relative to Tuscany rates, specified for gender, 5-year age groups and calendar year. Separate analyses were carried out for the job titles of makers, batch mixers and grinders. For males, 3180 individuals, the observed mortality for cancer causes was above the expected for the lung [standardized mortality ratio (SMR) 123, 10 observed (Obs)], larynx (SMR 166, 10 Obs), stomach (SMR 105, 30 Obs) and brain (SMR 150, 7 Obs). For non-cancer causes observed mortality was above expected for hypertensive diseases (SMR 178, 10 Obs) and diseases of the genitourinary system (SMR 169, 11 Obs). Increases for the above listed causes were shown also among makers. Mortality for larynx and lung cancer increased with time since first exposure and significantly increased SMRs were observed for 21 or more years since first exposure: this pattern was still present with smoking adjustment. The results showed consistently increased mortality for lung and larynx cancer in the overall cohort and among makers. Stomach cancer, brain cancer, hypertensive diseases and diseases of the genitourinary system were also increased in the overall cohort and among makers.

Surgical closure of muscular ventricular septal defects using double umbrella devices (intraoperative VSD device closure).

OBJECTIVES: Surgical closure of some muscular ventricular septal defects has been proven to be difficult. In order to simplify the surgical technique we have used intraoperatively Rashkind double umbrella devices to occlude muscular ventricular septal defects. METHODS: On the basis of haemodynamic and echocardiographic study five children aged 4, 6, 7, 14 and 41 months were considered suitable candidates for intraoperative closure of muscular ventricular septal defects (midmuscular in three cases, apical in two) by Rashkind devices. Three of them had previously undergone pulmonary artery banding at 10, 11 and 41 days, respectively. During hypothermic cardiopulmonary by pass a delivery system was introduced across the tricuspid valve into the right ventricle and then passed through the ventricular septal defect; the distal umbrella of a 17 mm device was opened in the left ventricular cavity; a traction was applied to the introducer and the proximal umbrella was opened on the right side straddling the interventricular septum; the device was then secured on the right side by few stitches. In one case because of the wide diameter of the ventricular septal defect two umbrellas were used. The surgical procedure was completed with debanding and/or closure of other defects close to the aortic or tricuspid valve. RESULTS: Immediate results, tested by epicardial or transesofageal echo, showed a minimal residual shunt in 4 patients and a moderate shunt in one. No early deaths occurred. A complete atrioventricular block developed in 1 patient who had an additional perimembranous defect closed with a prosthetic patch: a permanent pace maker was inserted 3 months after the operation. There was a late death for untractable right ventricular failure in 1 patient who had a large residual shunt erroneously considered moderate. In this patient, the size of the defect was underestimated both preoperatively then intraoperatively. The four survivors are doing well with no signs of hemodynamically significant residual shunts. CONCLUSIONS: The use of Rashkind umbrella devices for closing intraoperatively muscular defects can be helpful to standard surgical techniques when technical problems make patch closure difficult. Its use avoid the need of left ventriculotomy. Careful definition of the size of the defect is mandatory to select suitable candidates.

[Radiofrequency perforation in pulmonary valve atresia and intact ventricular septum]. / Perforazione con radiofrequenza della atresia valvolare polmonare a setto interventricolare intatto.

METHODS: Four neonates with Pulmonary Atresia and intact Ventricular Septum underwent Radiofrequency Valvotomy in our institution from October 1994 to June 1995. All patients had "membranous" atresia with confluent and normal size pulmonary branches; one of them had abnormal tricuspid tensor apparatus and severe right ventricular outflow tract hypertrophy. The right ventricle was considered of sufficient size to support biventricular circulation in all cases. In three patients the pulmonary arteries were supplied by the arterial duct maintained open with intravenous infusion of Prostaglandins. The other patient had previously undergone a modified left Blalock Taussig shunt in another institution. The cardiac catheterization was performed by the femoral venous and arterial routes, under general anesthesia. After delineating the atretic valve with angiography, a 5 French right Judkins catheter was placed in the right ventricular outflow tract just beneath the membrane. A 2 french radiofrequency catheter (Cerablate PA 120 Osypka) was then passed through the right Judkins; with a mean energy of 5-10 watts over 3-5 seconds the valve was perforated. After a predilation with a 2,5 mm balloon catheter (New Probe USCI or Cobra SCIMED), the valve was dilated with balloon catheters 20 to 30% larger than the anulus (Cristal Balloon BALT). RESULTS: In all cases the procedure was successful without any complication; a significant decrease of right ventricular pressure and an excellent antegrade flow across the valve were achieved. Two patients were weaned from prostaglandins by sixth and eighth day after the procedure. The patient with previous Bialock Taussig shunt showed a complete recovery of the right ventricle, but four months later underwent surgery for shunt closure and enlargement of the left pulmonary branch. These three patients are well at latest follow-up (5-10 months), with normal biventricular circulation. The other patient remained duct-dependent and was operated on of outflow patch and shunt nine days after the procedure, with good result; he unfortunately died the tenth postoperative day for complications of hemopericardium related to epicardial wires removal. CONCLUSIONS: In our opinion radiofrequency valvotomy is the first choice procedure in cases suitable for biventricular repair. In selected patients it may represent a definitive treatment.

[Percutaneous occlusion of patent ductus arteriosus by the Rashkind double-umbrella device]. / Fermeture percutanée du canal artériel par la double ombrelle de Rashkind.

BACKGROUND: The transcatheter option consisting of implanting and releasing an occlusive device designed as a double-umbrella is an interesting alternative to surgery aimed to close persistently patent ductus arteriosus. POPULATION AND METHODS: Closure of a duct with the Rashkind device had been planned in 113 children. The procedure was abandoned in 12 with inadequately sized ducts (too large or too small). This study therefore included 101 attempts in patients aged 2.3 months to 18.5 years (m +/- 1 SD = 45.9 +/- 43.2 months) whose weights ranged from 3.3 to 87 kg (m +/- 1 SD = 15.7 +/- 11.7 kg). The narrowest dimension of the duct on the aortograms ranged from 1.2 to 6.2 mm (m +/- 1 SD = 2.9 +/- 0.9 mm). RESULTS: The procedure failed in seven patients because of a too large and/or tubular vessel, causing removal of the device prior to release in five patients, or surgical extraction after it had embolized into a pulmonary artery branch in two patients. An early acute hemolysis requiring again the surgical removal of an instable device in a tubular duct was seen in one case. Two patients had femoral artery occlusion successfully treated with thrombolytic agents. Complete occlusion was immediately proven in 32 (35%) of the 92 successful and stable implantations. These figures raised to 64% (59 cases) prior to discharge. At final follow-up (0.3-59 months, m +/- 1 SD = 13.8 +/- 14.4 months), another 16 total occlusions were observed and one patient was successfully managed by a second implantation. The final occlusion rate was 83% (76 cases). Of the 16 residual shunts, five were surgically suppressed and the remaining were minimal. CONCLUSION: Transcatheter occlusion of the patent ductus arteriosus is safe in children weighing more than 5 kg, having ducts with a narrowing ranging from 1 to 6 mm. It is efficient in five out of six cases and has less disadvantages than surgery.

[The closure with a double-umbrella prosthesis of recanalized ducts after surgical ligation]. / Chiusura mediante protesi a doppio ombrellino di dotti ricanalizzati dopo legatura chirurgica.

Four patients of 5, 27, 27 and 62 years of age, who previously underwent surgical ligation of ductus arteriosus (respectively 3, 17, 19 and 17 years before), came to our observation because of recanalization of the duct. In three cases the duct was an isolated finding, whereas in the oldest patient the duct ligation had been accompanied by implantation of a Starr prosthesis in aortic position. The diagnosis of residual shunt was based on the auscultation of a continuous murmur at the left subclavicular space and on typical signs of patent ductus evidenced by Doppler interrogation. In all cases the diagnosis was confirmed by angiography; the minimal diameter of the ducts varied between 2.5 and 4 mm. Three umbrellas of 17 mm and one of 12 mm diameter were employed for the closure of the residual shunt. The implantation of the double-umbrella device was obtained with the routine transfemoral venous approach. In the youngest patient, in order to facilitate the progression of the transseptal catheter through the duct, it was necessary to create a circuit with the guide wire from the femoral vein to the contralateral femoral artery. In all four patients the shunt was completely abolished. The oldest patient underwent replacement of a malfunctioning aortic prosthesis three days after implantation of the double-umbrella device; the surgical approach was this way significantly simplified. The implantation of a Rashkind double-umbrella device is the method of choice for closure of residual shunts through the duct after surgical ligation.

Hypertrophic obstructive cardiomyopathy. Late follow-up after ventricular myotomy-myectomy.

A series of 25 patients with hypertrophic obstructive cardiomyopathy, isolated or associated with minor anomalies, were operated upon using a modified Morrow's procedure. The indication for operation was based on either the presence of severe symptoms despite treatment with beta-blocking agents, or a significant peak gradient of over 50 mmHg even in asymptomatic patients. All patients survived and none was lost to follow-up. Long-term results were evaluated with 2-dimensional echocardiography. Surgical versus pharmacological treatment is discussed, with particular consideration given to paediatric patients.