Aim This study determined cardiovascular impairment in young children with obstructive respiratory disease who were assessed using the opening interrupter technique (RINT). Methods This pilot study enrolled 41 children who had been referred to pulmonology and allergology specialists at the University of Catania, Italy, from March to July 2017: 23 (mean age 4.13 +/- 0.62 years) had chronic coughs and wheezing and 18 controls (mean age 4.27 +/- 0.66 years) had obstructive chest disease, but were otherwise healthy. Airway resistance was evaluated using RINT and cardiac function by studying the ejection fraction, pulmonary artery systolic pressure (PASP), tricuspid annular plane systolic excursion and tricuspid flow propagation velocity (TFPV). Results The RINT and PASP values were significantly higher in the patient group when compared to the controls, but the TFPV values were lower. A direct and significant Spearman's correlation coefficient (r) between RINT and PASP values was observed (r = 0.81). We found a significant inverse correlation between RINT and TFPV (r = -0.83), as well as TFPV and PASP (r = -0.78). Conclusion This study showed that children with obstructive respiratory diseases had a major risk of cardiovascular impairment. Impaired diastolic function of the right ventricle occurred very early when airway resistance was abnormally increased.

Background: Chronic cough and wheezing represent the most common causes of respiratory disability among children, also showing an increased prevalence in cardiovascular impairment. Aim: To assess the cardiovascular impairment in preschool children with chronic cough and wheezing and to correlate the echocardiographic indices related to the right ventricular function with airway resistance evaluated using RINT. Methods: 23 children with obstructive lung diseases (12 males, mean age 4.13±0.62 years) and 18 healthy children (9 males, mean age 4.27±0.66 years) were enrolled. For each patient, airway resistance and cardiac functional parameters were evaluated. Results: RINT and Pulmonary Artery Pressure (PAP) values were significantly higher in patients group compared with controls (p<0.001). Tricuspid Flow Propagation Velocity (TFPV) values resulted lower in patients group compared with controls (p<0.001). A direct and significant correlation between RINT and PAP values was observed (r= 0.79; p<0.001). RINT correlated with TFPV in an inverse and significant manner (r= -0.71; p<0.001) as well as TFPV and PAP (r= 0.66; p<0.001). Conclusions: Preschool children with chronic cough and wheezing have a major risk of cardiovascular impairment. The impairment of diastolic function of right ventricle occurs very early in preschool children with respiratory diseases in which airway resistances resulted abnormally increased. We recommend a cardiological follow-up, using also TFPV and PAP parameters, in preschool children with chronic cough and wheezing.

Le mucopolisaccaridosi (MPS) rappresentano un gruppo di rari disordini lisosomiali, con una eterogenea presentazione clinica in termini di ereditarietà (autosomica recessiva ed X-linked), età di presentazione (neonatale, bambini ed adulti), manifestazioni sistemiche e cardiache (da forma lieve fino a quella severa). Le raccomandazioni basate sull’evidenza circa la diagnosi e la gestione della malattia cardiovascolare nelle MPS sono scarse. Il Gruppo Italiano Cardiologi Esperti Malattie Metaboliche (GICEM) è un gruppo di cardiologi, cardiochirurghi e pediatri con un’esperienza specifica nelle malattie metaboliche ed in particolare nelle MPS. In questa rassegna viene riportata la nostra esperienza e le nostre raccomandazioni sulla diagnosi e gestione della malattia cardiovascolare nelle MPS, con un approccio personalizzato basato sull’evidenza tenendo in considerazione i diversi fenotipi delle MPS (in particolare I, II, IVa, VI), età di presentazione e gravità delle manifestazioni sistemiche e cardiache.

Mucopolysaccharidoses (MPS) represent a group of rare lysosomal storage disorders, with a heterogeneous clinical presentation in terms of inheritance (autosomal and X-linked recessive), age of onset (infants, children, and adults), systemic and cardiac manifestations (mild to severe disease forms). Evidence-based recommendations on the diagnosis and management of cardiovascular disease in MPS are scarce. GICEM (Gruppo Italiano Cardiologi Esperti Malattie Metaboliche) is a group of cardiologists, cardiac surgeons and pediatricians with a specific expertise in metabolic diseases including MPS. In this paper, we report our experience and recommendations on the diagnosis and management of cardiovascular aspects in MPS, with a tailored approach based on current evidence, and taking into account MPS phenotype (particularly, I, II, IVa, VI), age at presentation, and severity of systemic and cardiac manifestations.

Probiotics are defined as live microorganisms which when administered in adequate amounts, confer a health benefit to the host. They can positively influence the intestinal microbiota, are safe, and do not show any adverse reactions or side effects even in preterm infants. They have both direct and indirect effects: enhancement of the epithelial barrier, increased adhesion to intestinal mucosa, concomitant inhibition of pathogen adhesion, competitive exclusion of pathogenic microorganisms, production of antimicroorganism substances, and modulation of the immune system.

Kawasaki disease is a vasculitis of the medium sized arteries, widely spread in developed countries and of unknown etiology. The diagnosis is clinical and is based on the criteria established by the American Heart Association. The most redoubtable cardiovascular finding of the disease is the coronary aneurysm; its incidence can be drastically reduced by a timely first line therapy (intravenous immunoglobulin and aspirin at antinflammatory dosage). The resistant cases are treated with a second dose of IVIG or, secondarily, with infliximab, methylprednisolone, cyclosporine or abciximab. Several studies have demonstrated the association between childhood Kawasaki disease and adulthood atherosclerosis; moreover is attested that the intimal thickening of the left main and left anterior descending coronary artery is a predictor of subclinical atherosclerosis. On this basis, we conducted an observational cross-sectional case control double blind study about the

Adolescents with type 1 diabetes and obesity present higher cardiovascular risk and ambulatory blood pressure measurements (ABPM) has been shown to predict vascular events, especially by identifying the nondipper status. The aim of our observational cross-sectional study conducted in adolescents with type 1 diabetes, overweight subjects and healthy controls was to assess mean blood pressure parameters to identify subclinical cardiovascular risk.

BACKGROUND:Cardiovascular involvement in Cystic Fibrosis (CF) is a not rare condition, although the prevalence of subclinical pulmonary hypertension (PH) and cardiac dysfunction is not known in the early stages of CF progression. The aim of our study was to assess cardiac involvement in children and adults affected by cystic fibrosis compared with healthy subjects of same age using echocardiography.METHODS:Fifty-five patients, 25 adults and 30 children completed the study. We assessed FEV1 (Forced Expiratory Volume in one second), and carried out colour Doppler-echocardiography evaluating ejection fraction (EF) measurement of left ventricle, tricuspid annular plane systolic excursion (TAPSE) of right ventricle and pulmonary artery pressure (PAP). We compared the auxological, respiratory and cardiologic data with those of 16 adults and 34 children of the same age.RESULTS:We discovered significantly different values of PAP between patients and controls in both children (p = 0.0001, r=- 0.62) and adults (p=0.0001, r=- 0.63), whereas the EF and TAPSE showed significantly different values in only adults (p=0.0023 and p=0.0194 respectively). We found in both children and adults with CF an inverse correlation between PAP and FEV1 (p=0.000, p=0.001), Erythrocyte Sedimentation Rate (ESR) and FEV 1 (p=0.015, r=- 0.43; p=0.009, r=- 0.51), and highly sensitive C-reactive protein (hs-CRP) and FEV 1 (p=0.007, r=- 0.48; p=0.001, r=- 0.60). In adults we also detected direct correlation between PAP and hs-CRP (p=0.008, r=0.51) and PAP and ESR (p=0.009, r=0.51).CONCLUSIONS:In paediatric-aged CF patients there are already early signs of potential heart impairment, represented by an increase of pulmonary blood pressure, and in adult age the systolic function of right ventricle may be impaired. We hypothesise that such cardiac impairments may gradually arise due to preceding chronic inflammation related to prior degeneration of lung function and thus it is very important to keep patients clinically stable and address chronic inflammation as early as possible in the progression of CF.

Background: Troponin is a protein of the troponin-tropomyosin complex in myocardium and it is considered an highly sensitive marker of myocardial necrosis both in adults and newborns with perinatal asphyxia. Some studies assume that high troponin levels in adults without signs of myocardial necrosis could be conditioned by some factors, such as hemolysis, turbidity, and hyperbilirubinemia. Case presentation: We report a case of a term female newborn of Caucasian race, with high troponin levels in absence of neonatal asphyxia and without clinical or instrumental signs of myocardial ischaemia. Conclusion: Hyperbilirubinemia might be a factor that could interfere with assessment of sieric troponin T. This might suggest that levels of troponin T should be trusted only when they are associated with signs of other conditions suggesting heart damage or respiratory distress.

Fazit Die Durchführung einer SLI im Kreißsaal sei bei Frühgeborenen eine einfache und wirksame Methode, um die Lungenfunktion der Kinder zu verbessern und die Notwendigkeit invasiver Beatmungsmaßnahmen zu vermindern, meinen Grasso et al. Dabei traten in der hier untersuchten Gruppe Komplikationen nicht häufiger auf als bei den konventionell behandelten Kindern. Der Unterschied zu den Ergebnissen anderer Gruppen könnte u. a. dadurch zustande kommen, dass die SLI hier für 15 s aufrechterhalten wurde im Gegensatz zu den 5 s, die einige Autoren beschrieben haben. Negative Auswirkungen auf die Hämodynamik wurden hier nicht beobachtet, allerdings sollte dieser mögliche Effekt weiter untersucht werden.

Persistent pulmonary hypertension of the newborn (PPHN) is a cyanogenic plurifactorial disorder characterized by failed postnatal drop of pulmonary vascular resistance and maintenance of right-to-left shunt across ductus arteriosus and foramen ovale typical of intrauterine life. The pathogenesis of PPHN is very complex and can result from functional (vasoconstriction) or structural (arteriolar remodeling, reduced pulmonary vessels density) anomalies of pulmonary circulation. Etiopathogenetic factors heterogeneity can strongly condition therapeutical results and prognosis of PPHN that is particularly severe in organic forms that are usually refractory to selective pulmonary vasodilator therapy with inhaled nitric oxide. This paper reports the more recent acquisitions on molecular physiopathogenetic mechanisms underlying functional and structural forms of PPHN and illustrates the bases for adoption of new potential treatment strategies for organic PPHN. These strategies aim to reverse pulmonary vascular remodeling in PPHN with arteriolar smooth muscle hypertrophy and stimulate pulmonary vascular and alveolar growth in PPHN associated with lung hypoplasia.In order to restore lung growth in this severe form of PPHN, attention is focused on the results of studies of mesenchymal stem cells and their therapeutical paracrine effects on bronchopulmonry dysplasia, a chronic neonatal lung disease characterized by arrested vascular and alveolar growth and development of pulmonary hypertension.

Background: Rhabdomyomas are the most common type of cardiac tumors in children. Anatomically, they can be considered as hamartomas. They are usually randomly diagnosed antenatally or postnatally sometimes presenting in the neonatal period with haemodynamic compromise or severe arrhythmias although most neonatal cases remain asymptomatic. Typically rhabdomyomas are multiple lesions and usually regress spontaneously but are often associated with tuberous sclerosis complex (TSC), an autosomal dominant multisystem disorder caused by mutations in either of the two genes, TSC1 or TSC2. Diagnosis of tuberous sclerosis is usually made on clinical grounds and eventually confirmed by a genetic test by searching for TSC genes mutations.Methods: We report our experience on 33 cases affected with rhabdomyomas and diagnosed from January 1989 to December 2012, focusing on the cardiac outcome and on association with the signs of tuberous sclerosis complex. We performed echocardiography using initially a Philips Sonos 2500 with a 7,5/ 5 probe and in the last 4 years a Philips IE33 with a S12-4 probe. We investigated the family history, brain, skin, kidney and retinal lesions, development of seizures, and neuropsychiatric disorders.Results: At diagnosis we detected 205 masses, mostly localized in interventricular septum, right ventricle and left ventricle. Only in 4 babies (12%) the presence of a mass caused a significant obstruction. A baby, with an enormous septal rhabdomyoma associated to multiple rhabdomyomas in both right and left ventricular walls died just after birth due to severe heart failure. During follow-up we observed a reduction of rhabdomyomas in terms of both number and size in all 32 surviving patients except in one child. Eight patients (24,2%) had an arrhythmia and in 2 of these cases rhabdomyomas led to Wolf-Parkinson-White Syndrome. For all patients the arrhythmia spontaneously totally disappeared or was reduced gradually. With regarding to association with tuberous sclerosis, we diagnosed tuberous sclerosis clinically in 31 babies (93,9%).Conclusion: Rhabdobyomas are tumors with favorable prognosis because they frequently do not cause symptoms and they often regress in numbers and size. Nevertheless, due to frequent association with tuberous sclerosis complex and the resulting neurological impairment, the prognosis can result unfavorable.

Background and aim Adolescents with type 1 diabetes (T1DM) and obesity present higher cardiovascular risk and ambulatory blood pressure measurements (ABPM) can identify the non-dipper status, often the first sign of hypertension. The aim of our observational cross-sectional case-control study conducted in T1DM, overweight and healthy adolescents was to assess blood pressure (BP) parameters to identify subclinical cardiovascular risk. Methods We performed ABPM for 24 h in 39 adolescents (26 male and 13 female) with T1DM followed in our Paediatric Department between January 2011 and December 2012. We compared the data of patients with those of overweight subjects and healthy controls. Results ABPM revealed no significant difference between T1DM patients and overweight subjects except for night-time diastolic BP values (p = 0.000). Instead we found significant difference between type 1 diabetic patients and healthy controls in all 24 h Systolic (p = 0.000), 24 h Diastolic (p = 0.002), Day-time Systolic (p = 0.005), Night-time Systolic (p = 0.000), Day-time diastolic (p = 0.038) and Night-time Diastolic (0.000) BP values. We detected hypertension in 10/39 (25.6%) T1DM patients and in 8/39 (20.5%) overweight subjects (p = 0.591), whereas no-one of healthy controls presented hypertension (p = 0.001). We observed non-dipper pattern in 32/39 (82%) T1DM patients, in 26/39 (66.6%) overweight subjects (p = 0.120), and in 13/39 (33.3%) of healthy controls (p = 0.000). Conclusions ABPM studies allows to identify patients at risk for the development of hypertension, who might benefit from the early introduction of anti-hypertensive therapies. In evaluating BP in T1DM and overweight subjects, ABPM should be used since a reduced dipping can indicate incipient hypertension.