Publications by authors named "Ugo Giordano"

25 Publications

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How to Apply European and American Guidelines on High Blood Pressure in Children and Adolescents. A Position Paper Endorsed by the Italian Society of Hypertension and the Italian Society of Pediatrics.

High Blood Press Cardiovasc Prev 2020 Jun 13;27(3):183-193. Epub 2020 Mar 13.

Arterial hypertension unit Pediatric Cardiology and cardiac Surgery Department, Bambino Gesù Children's Hospital, Rome, Italy.

Children are defined as hypertensive when their blood pressure values equal or exceed the 95th percentile of the blood pressure value distribution in a pediatric population, according to gender, age and height. The population on which reference tables are based is of fundamental importance to establish the threshold values for the diagnosis of hypertension in pediatric age. Before 2017, both American and European guidelines used nomograms created in the same reference population which included children of all weight classes. Given the close and well-known association between hypertension and excess weight in childhood, the 2017 American guidelines proposed new reference nomograms excluding subjects with overweight and obesity from the "historical" reference population. Furthermore, the new American guidelines suggested a fixed cut-off of 130/80 mmHg, starting from 13 years and regardless of gender and height, to make the diagnosis of hypertension. In this document, the Italian Hypertension Society (SIIA) and the Italian Pediatric Society (SIP) jointly discuss a number of issues raised by the new American guidelines that involve the entire medical community, and also address the definition of arterial hypertension in the transition phase between childhood and adulthood.
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http://dx.doi.org/10.1007/s40292-020-00369-yDOI Listing
June 2020

Kawasaki disease: guidelines of the Italian Society of Pediatrics, part I - definition, epidemiology, etiopathogenesis, clinical expression and management of the acute phase.

Ital J Pediatr 2018 Aug 30;44(1):102. Epub 2018 Aug 30.

Bambino Gesù Children's Hospital, Rome, Italy, Piazza S. Onofrio n. 4, 00165, Rome, Italy.

The primary purpose of these practical guidelines related to Kawasaki disease (KD) is to contribute to prompt diagnosis and appropriate treatment on the basis of different specialists' contributions in the field. A set of 40 recommendations is provided, divided in two parts: the first describes the definition of KD, its epidemiology, etiopathogenetic hints, presentation, clinical course and general management, including treatment of the acute phase, through specific 23 recommendations.Their application is aimed at improving the rate of treatment with intravenous immunoglobulin and the overall potential development of coronary artery abnormalities in KD. Guidelines, however, should not be considered a norm that limits treatment options of pediatricians and practitioners, as treatment modalities other than those recommended may be required as a result of peculiar medical circumstances, patient's condition, and disease severity or complications.
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http://dx.doi.org/10.1186/s13052-018-0536-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6116535PMC
August 2018

Kawasaki disease: guidelines of Italian Society of Pediatrics, part II - treatment of resistant forms and cardiovascular complications, follow-up, lifestyle and prevention of cardiovascular risks.

Ital J Pediatr 2018 Aug 30;44(1):103. Epub 2018 Aug 30.

Bambino Gesù Children's Hospital, Piazza S. Onofrio n. 4, 00165, Rome, Italy.

This second part of practical Guidelines related to Kawasaki disease (KD) has the goal of contributing to prompt diagnosis and most appropriate treatment of KD resistant forms and cardiovascular complications, including non-pharmacologic treatments, follow-up, lifestyle and prevention of cardiovascular risks in the long-term through a set of 17 recommendations.Guidelines, however, should not be considered a norm that limits the treatment options of pediatricians and practitioners, as treatment modalities other than those recommended may be required as a result of peculiar medical circumstances, patient's condition, and disease severity or individual complications.
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http://dx.doi.org/10.1186/s13052-018-0529-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6116479PMC
August 2018

Impact of complex congenital heart disease on the prevalence of arterial hypertension after aortic coarctation repair.

Eur J Cardiothorac Surg 2019 Mar;55(3):559-563

Pediatric Cardiology and Cardiac Surgery Department, Bambino Gesù Children's Hospital, Institute for Treatment and Research, Rome, Italy.

Objectives: This study was designed to evaluate the difference in the prevalence of long-term arterial hypertension among patients with corrected aortic coarctation according to the existence of associated cardiac congenital lesions.

Methods: We identified 235 patients who had undergone surgery for aortic coarctation and classified them into 2 groups: patients with isolated coarctation of the aorta (CoA) and patients with aortic coarctation associated with complex congenital heart disease. Data were retrospectively analysed.

Results: There were 148 subjects with isolated CoA and 87 with complex CoA (CoA-c). Patients were defined as hypertensive if they required antihypertensive treatment and/or when blood pressure was above 95th percentile. Patients with isolated aortic coarctation were significantly younger than patients with CoA-c (P < 0.001) and a markedly higher prevalence of arterial hypertension (44% vs 24%) was documented in the isolated coarctation group. The difference in the prevalence of hypertension in the 2 groups was still significant after correcting for differences in age (P < 0.001), confirming that the prevalence of arterial hypertension in patients with CoA-c was half of that of patients with isolated CoA.

Conclusions: We conclude that complex congenital heart disease in patients who have undergone surgical correction for aortic coarctation results in a significantly lower prevalence of late-onset hypertension. Reduced systemic flow and pressure before surgery in patients with CoA-c might be associated with a lower rate of arterial hypertension.
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http://dx.doi.org/10.1093/ejcts/ezy257DOI Listing
March 2019

Use of a Pediatric Syncope Unit Improves Diagnosis and Lowers Costs: A Hospital-Based Experience.

J Pediatr 2018 10 28;201:184-189.e2. Epub 2018 Jun 28.

Clinical Care and Management, Innovation Research Area, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.

Objectives: To assess the effect of a dedicated pediatric syncope unit on the diagnostic and therapeutic management of children with suspected syncope. We also evaluated the effectiveness of the pediatric syncope unit model in decreasing unnecessary tests and hospitalizations, minimizing social costs, and improving diagnostic yield.

Study Design: This single-center cohort observational, prospective study enrolled 2278 consecutive children referred to Bambino Gesù Children's Hospital from 2012 to 2017. Characteristics of the study population, number and type of admission examinations, and diagnostic findings before the pediatric syncope unit was implemented (2012-2013) and after the pediatric syncope unit was implemented (2014-2015 and 2016-2017) were compared.

Results: The proportion of undefined syncope, number of unnecessary diagnostic tests performed, and number of hospital stay days decreased significantly (P < .0001), with an overall decrease in costs. A multivariable logistic regression analysis, adjusted for confounding variables (age, sex, number of diagnostic tests), the period after pediatric syncope unit (2016-2017) resulted as the best independent predictor of effectiveness for a defined diagnosis of syncope (P < .0001).

Conclusions: Pediatric syncope unit organization with fast-tracking access more appropriate diagnostic tests is effective in terms of accuracy of diagnostic yield and reduction of costs.
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http://dx.doi.org/10.1016/j.jpeds.2018.05.032DOI Listing
October 2018

Prevalence of Hypertension in Children with Early-Stage ADPKD.

Clin J Am Soc Nephrol 2018 06 19;13(6):874-883. Epub 2018 Apr 19.

Division of Nephrology, Department of Pediatric Subspecialties, and

Background And Objectives: Autosomal dominant polycystic kidney disease is the most common inheritable kidney disease, frequently thought to become symptomatic in adulthood. However, patients with autosomal dominant polycystic kidney disease may develop signs or symptoms during childhood, in particular hypertension. Although ambulatory BP monitoring is the preferred method to diagnose hypertension in pediatrics, data in children with autosomal dominant polycystic kidney disease are limited.

Design, Setting, Participants, & Measurements: Our retrospective multicenter study was conducted to collect ambulatory BP monitoring recordings from patients with autosomal dominant polycystic kidney disease age <18 years old. Basic anthropometric parameters as well as data on kidney function, BP treatment, and kidney ultrasound were also collected.

Results: Data from 310 children with autosomal dominant polycystic kidney disease with a mean age of 11.5±4.1 years old were collected at 22 European centers. At the time when ambulatory BP monitoring was performed, 95% of children had normal kidney function. Reference data for ambulatory BP monitoring were available for 292 patients. The prevalence rates of children with hypertension and/or those who were treated with antihypertensive drugs were 31%, 42%, and 35% during daytime, nighttime, or the entire 24-hour cycle, respectively. In addition, 52% of participants lacked a physiologic nocturnal BP dipping, and 18% had isolated nocturnal hypertension. Logistic regression analysis showed a significant association between a categorical cyst score that was calculated on the basis of the number of cysts >1 cm per kidney and daytime hypertension (odds ratio, 1.70; 95% confidence interval, 1.21 to 2.4; =0.002), nighttime hypertension (odds ratio, 1.31; 95% confidence interval, 1.05 to 1.63; =0.02), or 24-hour hypertension (odds ratio, 1.39; 95% confidence interval, 1.08 to 1.81; =0.01). Kidney length, expressed as SD score, was also significantly associated with nighttime hypertension (odds ratio, 1.23; 95% confidence interval, 1.06 to 1.42; =0.10).

Conclusions: These data indicate high prevalence of hypertension in children with autosomal dominant polycystic kidney disease starting at young ages.
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http://dx.doi.org/10.2215/CJN.11401017DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5989684PMC
June 2018

The Benefit of Sleeve Gastrectomy in Obese Adolescents on Nonalcoholic Steatohepatitis and Hepatic Fibrosis.

J Pediatr 2017 01 30;180:31-37.e2. Epub 2016 Sep 30.

Hepatometabolic Unit, Bambino Gesù Children's Hospital, Rome, Italy; Liver Research Unit, Bambino Gesù Children's Hospital, Rome, Italy. Electronic address:

Objective: To determine whether bariatric surgery is effective for the treatment of nonalcoholic steatohepatitis (NASH) in adolescence, we compared the efficacy of laparoscopic sleeve gastrectomy (LSG) with that of lifestyle intervention (nonsurgical weight loss [NSWL]) for NASH reversal in obese adolescents.

Study Design: Obese (body mass index ≥ 35 kg/m) adolescents (13-17 years of age) with biopsy-proven NAFLD underwent LSG, lifestyle intervention plus intragastric weight loss devices (IGWLD), or only NSWL. At baseline and 1 year after treatment, patients underwent clinical and psychosocial evaluation, blood tests, liver biopsy, polysomnography, and 24-hour ambulatory blood pressure estimation.

Results: Twenty patients (21%) underwent LSG, 20 (21%) underwent IGWLD, and 53 (58%) received lifestyle intervention alone (NSWL). One year after treatment, patients who underwent LSG lost 21.5% of their baseline body weight, whereas patients who underwent IGWLD lost 3.4%, and patients who underwent NSWL increase 1.7%. In patients who underwent LSG, NASH reverted completely in all patients and hepatic fibrosis stage 2 disappeared in 18 patients (90%). After IGWLD, NASH reverted in 6 patients (24%) and fibrosis in 7 (37%). Patients who received the NSWL intervention did not improve significantly. Hypertension resolved in all patients who underwent LSG with preoperative hypertension (12/12) versus 50% (4/8) of the patients who underwent IGWLD (P = .02). The cohort-specific changes in impaired glucose metabolism were similar: 100% (9/9) of affected patients who underwent LSG versus 50% (1/2) of patients who underwent IGWLD (P = .02). LSG was also more affective in resolving dyslipidemia (55% [7/12] vs 26% [10/19]; P = .05) and sleep apnea (78% [2/9] vs 30% [11/20]; P = .001).

Conclusion: LSG was more effective than lifestyle intervention, even when combined with intragastric devices, for reducing NASH and liver fibrosis in obese adolescents after 1 year of treatment.
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http://dx.doi.org/10.1016/j.jpeds.2016.08.101DOI Listing
January 2017

Transient global ventricular dysfunction in an adolescent affected by pancreatic adenocarcinoma.

BMC Pediatr 2016 07 19;16:99. Epub 2016 Jul 19.

Pediatric Cardiology, Bambino Gesù Children's Hospital, Rome, Italy.

Background: Takotsubo cardiomyopathy (TC) is characterized by a transient decrease in ejection fraction and a reversible left ventricular dysfunction. The pathophysiology of TC is not completely understood. Heterogeneous and multifactorial mechanisms are involved: drugs, emotional and physical stress, genetic and hormonal factors.

Case Presentation: A 17 year-old male with metastatic pancreatic adenocarcinoma, under chemotherapy containing 5-fluorouracil, presented severe left ventricular dysfunction requiring mechanical ventilation and inotropes administration. He completely recovered in 2 weeks.

Conclusion: To our knowledge this is the first report of transient form of ventricular dysfunction, mimicking TC, in an adolescent. We believe that children and adolescents receiving 5-fluorouracil should be closely monitored and referred for investigation if they develop cardiac symptoms.
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http://dx.doi.org/10.1186/s12887-016-0628-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4952277PMC
July 2016

Novelty in hypertension in children and adolescents: focus on hypertension during the first year of life, use and interpretation of ambulatory blood pressure monitoring, role of physical activity in prevention and treatment, simple carbohydrates and uric acid as risk factors.

Ital J Pediatr 2016 Jul 16;42(1):69. Epub 2016 Jul 16.

Dipartimento di Medicina e Chirurgia, Università di Milano Bicocca, Monza, Italy.

The present article intends to provide an update of the article "Focus on prevention, diagnosis and treatment of hypertension in children and adolescents" published in 2013 (Spagnolo et al., Ital J Pediatr 39:20, 2013) in this journal. This revision is justified by the fact that during the last years there have been several new scientific contributions to the problem of hypertension in pediatric age and during adolescence. Nevertheless, for what regards some aspects of the previous article, the newly acquired information did not require substantial changes to what was already published, both from a cultural and from a clinical point of view. We felt, however, the necessity to rewrite and/or to extend other parts in the light of the most recent scientific publications. More specifically, we updated and extended the chapters on the diagnosis and management of hypertension in newborns and unweaned babies, on the use and interpretation of ambulatory blood pressure monitoring, and on the usefulness of and indications for physical activity. Furthermore, we added an entirely new section on the role that simple carbohydrates (fructose in particular) and uric acid may play in the pathogenesis of hypertension in pediatric age.
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http://dx.doi.org/10.1186/s13052-016-0277-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4947361PMC
July 2016

The availability and the adherence to pediatric guidelines for the management of syncope in the Emergency Department.

J Pediatr 2014 Nov 6;165(5):967-72.e1. Epub 2014 Aug 6.

Pediatric Emergency Department, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

Objective: To evaluate the impact of the 2009 Italian pediatric clinical guidelines on the management of syncope.

Study Design: A retrospective study of patients who presented to the Emergency Department (ED) of Bambino Gesù Children's Hospital with syncope during the 2 years before and then for 2 years after the establishment of the Italian pediatric clinical guidelines. Implementation of the clinical guidelines included educational seminars, additional training of health care workers, and the availability of clinical guidelines and its algorithms on ED examination rooms.

Results: We studied a population of 1073 patients (n = 470 vs n = 603). Most patients had neurocardiogenic syncope with a greater increase in postimplementation period (n = 241, 51.3% vs n = 454, 49.8%); we also noticed a parallel reduction of the number of patients with undefined syncope (30% vs 8.3%). We observed an increase in electrocardiogram (n = 328, 69.8% vs n = 512, 85.1%; P < .001), a reduction in electroencephalogram (n = 54, 11.5% vs n = 25, 4.1%; P < .001), and computed tomography scan/magnetic resonance imaging utilization (n = 26, 5.5% vs n = 8, 1.3%; P < .001). In addition, there was a significant reduction of hospital admission rates (n = 195; 41.5% vs n = 116, 19.2%; P < .001). The time period was significantly associated with improvements in all procedures at the multivariate analysis.

Conclusions: Providing practitioners in the ED with age-oriented clinical guidelines increased the efficiency of clinical management of pediatric syncope. Our study demonstrated that the implementation of pediatric clinical guidelines on syncope improve diagnosis, reduce hospital admissions, and decrease the use of unnecessary diagnostic tests.
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http://dx.doi.org/10.1016/j.jpeds.2014.06.064DOI Listing
November 2014

Association between nocturnal blood pressure dipping and insulin resistance in children affected by NAFLD.

Eur J Pediatr 2014 Nov 17;173(11):1511-8. Epub 2014 Jun 17.

Sport Medicine Unit, Bambino Gesù Children's Hospital-IRCCS, Rome, Italy,

Unlabelled: The aim of this study was to analyse the relationship between insulin-glucose metabolism, nocturnal blood pressure dipping and nonalcoholic fatty liver disease (NAFLD) in obese adolescents without diabetes. One hundred one consecutive children, with biopsy-proven NAFLD, were included in this study. Blood samples were drawn for the analyses of liver function tests, insulin-glucose metabolism and lipid profile appraisal. An ambulatory blood pressure measurement (ABPM) was performed. Seventy-six children (75.3 %) were systolic nondippers, and 23 of them were diastolic nondippers (30.3 %). No differences were found in the anthropometric parameters between the two groups. When compared to the systolic dippers, the systolic nondippers had higher medians of mean nocturnal blood pressure, glucose at 0, 60 and 120 min in the oral glucose tolerance test (OGTT), OGTT insulin at all time points and insulin-resistance values. No correlation of histopathological features with dipping/nondipping statuses was found.

Conclusions: We found an association between a nocturnal blood pressure fall and measures of insulin levels, independent of obesity, or daytime blood pressure levels, among the obese patients with NAFLD. Although no association between nondipping profiles and NAFLD was observed in our study, further studies with a longer term follow-up are needed, to better elucidate the complex link between these particular entities.
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http://dx.doi.org/10.1007/s00431-014-2342-2DOI Listing
November 2014

Heart rate variability arterial hypertension in young heart-transplanted recipients: association progression of cardiac allograft vasculopathy?

Pediatr Transplant 2013 Aug 5;17(5):441-4. Epub 2013 Jun 5.

CardioRespiratory and Sport Medicine Unit, Bambino Gesù Children's Hospital, Rome, Italy.

Hypertension and reduced HRV are frequent in heart-transplanted recipients. We studied 26 young recipients to investigate the relationship between BP and HRV during simultaneous 24-h monitoring. Presence of CAV was considered. All HRV measures were significantly lower than normal values. Significant correlations were found between mean daytime systolic BP and the rMSSD (p = 0.04), and mean daytime DBP and SDANN for all 5-min segments (p = 0.03) and between rMSSD and mean nighttime DBP (p = 0.03). Four patients were hypertensive during daytime, seven had a reduced nocturnal fall and two had a nocturnal rise in BP. Eight patients showed severe CAV grade (grade IV) on the Stanford scale, 13 moderate (grade III) and five mild (grade I-II). After a follow-up time of 30 months, four of the 13 patients (30%) with CAV grade III showed an increase to grade IV and all showed abnormalities of both HRV and ABPM patterns. The relationship between HRV abnormalities and arterial hypertension and CAV should be further explored.
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http://dx.doi.org/10.1111/petr.12105DOI Listing
August 2013

Fatty liver and insulin resistance in children with hypobetalipoproteinemia: the importance of aetiology.

Clin Endocrinol (Oxf) 2013 Jul 25;79(1):49-54. Epub 2013 Mar 25.

Hepato-Metabolic Department, Bambino Gesù Children's Hospital-IRCCS, Rome, Italy.

Objective: Hepatic steatosis is strongly associated with insulin resistance, but causative mechanisms that link these conditions are still largely unknown. Nowadays, it is difficult to establish whether fatty liver is the cause of insulin resistance or instead the complex metabolic derangements of insulin resistance determine hepatic steatosis and its progression to fibrosis. In patients with familial hypobetalipoproteinemia (FHBL), hepatic steatosis is because of the genetically determined defective form of apolipoprotein B, independently of metabolic derangements. Therefore patients with FHBL represent a good in vivo model to evaluate the relationships between fatty liver and insulin sensitivity.

Methods: We evaluated insulin resistance through HOMA-IR in 60 children with echografic and histological features of steatosis; 30 of whom had nonalcoholic fatty liver disease (NAFLD) and 30 had FHBL.

Results: All patients had histological features of hepatic steatosis. Patients with FHBL were hypolipidemic, as expected. No significant differences between two groups were observed in liver function tests. IRI and HOMA-IR were statistically higher in NAFLD subjects compared to the FHBL group.

Conclusion: In our study, we demonstrated that in children with FHBL, hepatic steatosis is dissociated from insulin resistance. This finding suggests that fat accumulation per se may be not a sufficient causal factor leading to insulin resistance, and that other mediators may be involved in the development of alteration in glucose metabolism and metabolic syndrome in patients with NAFLD.
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http://dx.doi.org/10.1111/j.1365-2265.2012.04498.xDOI Listing
July 2013

Heart rate variability abnormalities in young patients with dilated cardiomyopathy.

Pediatr Cardiol 2012 Oct 13;33(7):1171-4. Epub 2012 Mar 13.

Pediatric Cardiology & Cardiac Surgery Department, Bambino Gesù Children's Hospital, P.zza Sant'Onofrio 4, 00165, Rome, Italy.

Heart rate variability (HRV) has become the conventionally accepted term for describing variations in both instantaneous heart rate and R-R intervals. In the pediatric age group, HRV has been investigated in healthy children, diabetics, respiratory distress syndrome of the newborn, and sudden infant death syndrome. This study aimed to evaluate HRV in pediatric patients with dilated cardiomyopathy and to compare it with that of age-matched normal subjects. The study evaluated 21 patients with dilated cardiomyopathy: 11 females (mean age, 7 ± 4 years; range, 2-17 years) and 10 males (mean age, 10 ± 6 years; range, 2-18 years). Dilated cardiomyopathy (DCM) was diagnosed according to commonly accepted criteria after a noninvasive cardiologic examination, echocardiography, and 24-h Holter monitoring (MR45 and MR45-3 Oxford recorder). The patients were divided into six groups according to age, sex, and type of cardiomyopathy. Heart rate variability was recorded and analyzed in the time domain. The patients with DCM showed an abnormal HRV pattern. Particularly in the 5-6-year-old male patient group, the HRV values all were significantly increased (p = 0.05). In the 2-6-year-old female patient group, the mean cycle length, the standard deviation of all normal sinus R-R intervals during 24 h (SDNN), and the standard deviation of the average normal sinus R-R intervals for all 5-min segments (SDANN) were significantly increased (p = 0.05). The 13-18-year-old female patient group showed a significant reduction in SDNN and the mean of the standard deviation of all normal sinus R-R intervals for all 5-min segments (SDNNi) (p = 0.05). The modification of the HRV pattern in the time domain, partially age- and gender-dependent modification, may reflect an imbalance of the autonomic nervous system in children who show a delayed or reduced activity, such as pediatric patients with DCM.
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http://dx.doi.org/10.1007/s00246-012-0277-9DOI Listing
October 2012

Physical activity, fitness, and dyspnea perception in children with congenital diaphragmatic hernia.

Pediatr Pulmonol 2011 Oct 25;46(10):1000-6. Epub 2011 Apr 25.

Cardiorespiratory and Sport Medicine Unit, Cardiology and Cardiac Surgery Department, Bambino Gesù Children's Hospital, Rome, Italy.

We assessed whether physical activity could influence the performance and perception of dyspnea in children who were operated on for high risk congenital diaphragmatic hernia (CDH). We hypothesized that CDH children with normal activity would have better lung function and exercise performance level when compared to sedentary CDH subjects. We studied 18 children (11 males and 7 females, mean age 6.6 ± 2.6 years) who were surgically corrected. All children underwent physical examination, ECG at rest, and a maximal exercise stress test on a treadmill to measure the duration of exercise, maximal heart rate and blood pressure, maximal oxygen uptake (VO(2)  max and VO(2)  ml/kg/min). Lung function testing to measure forced vital capacity (FVC), forced expiratory volume in 1 sec (FEV(1) ), and peak expiratory flow (PEF) was also performed. Following the stress test, the Dalhousie dyspnea and effort scale was shown to children as a pictorial panel with three groups of increasing (from 1 to 7) levels of effort perception, throat discomfort, and chest dyspnea. Children were divided into group A (sedentary) and group B (regular physical participation). There was no difference in CDH severity between the two groups. Group A had a statistically significant lower duration of exercise (P < 0.01), maximal oxygen consumption (VO(2)  max P < 0.0001), VO(2)  ml/kg/min (P < 0.001), higher throat closing feeling (P < 0.004), chest dyspnea (P < 0.001), and effort perception (P < 0.04) compared to group B. No differences were found in lung function tests. In conclusion, our data may suggest that children with a history of CDH who are active maintain a higher level of performance with less perception of dyspnea and effort.
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http://dx.doi.org/10.1002/ppul.21471DOI Listing
October 2011

Metabolic syndrome in italian obese children and adolescents: stronger association with central fat depot than with insulin sensitivity and birth weight.

Int J Hypertens 2011 Feb 22;2011:257168. Epub 2011 Feb 22.

Endocrinology and Diabetes Unit, University Department of Paediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Piazza S.Onofrio 4, 00165 Rome, Italy.

Aim. To evaluate whether body fat distribution, birth weight, and family history for diabetes (FHD) were associated with metabolic syndrome (MetS) in children and adolescents. Methods. A total of 439 Italian obese children and adolescents (5-18 years) were enrolled. Subjects were divided into 2 groups: prepubertal and pubertal. MetS was diagnosed according to the adapted National Cholesterol Education Program criteria. Birth weight percentile, central obesity index (measured by dual-energy X-ray absorptiometry), insulin sensitivity (ISI), and disposition index were evaluated. Multivariate logistic regression models were used to determine variables associated with MetS. Results. The prevalence of MetS was 17%, with higher percentage in adolescents than in children (21 versus 12%). In the overall population, central obesity index was a stronger predictor of MetS than insulin sensitivity and low birth weight. When the two groups were considered, central fat depot remained the strongest predictor of MetS, with ISI similarly influencing the probability of MetS in the two groups and birth weight being negatively associated to MetS only in pubertal individuals. Neither FHD nor degree of fatness was a significant predictor of MetS. Conclusion. Simple clinical parameters like increased abdominal adiposity and low birth weight could be useful tools to identify European obese adolescents at risk for metabolic complications.
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http://dx.doi.org/10.4061/2011/257168DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3057027PMC
February 2011

Usefulness of ambulatory blood pressure monitoring and head-up tilt test in the evaluation of paediatric syncope.

Cardiol Young 2011 Feb 16;21(1):89-93. Epub 2010 Nov 16.

Cardiology and Cardiac Surgery Department, Cardiorespiratory and Sport Medicine Unit, Bambino Gesù Children's Hospital - Research Institute, Rome, Italy.

Objectives: The aim of this study was to investigate the usefulness of ambulatory blood pressure monitoring versus head-up tilt test in the evaluation of children with a history of syncope.

Study Design: We considered 146 consecutive children with more than one episode of syncope. All patients had a normal electrocardiogram at rest and were otherwise considered to be healthy. Forty-six patients,19 male, with a mean age of 13.6 plus or minus 5.6 years, were studied with a head-up tilt test and 100 patients, 41 male with a mean age of 9.4 plus or minus 5.6 years, were studied with ambulatory blood pressure monitoring. Twelve patients underwent both procedures. Hypotension during ambulatory blood pressure monitoring was defined when mean blood pressure values were lower than the 50th centile and the head-up tilt test was positive when syncope occurred. All patients were followed for 10 plus or minus 2 months.

Results: Ambulatory blood pressure monitoring showed postural hypotension in 91% children, while head-up tilt test was positive for 54%. In the group of children having both tests, two of them were negative for both, 10 of 12 children had a positive ambulatory blood pressure monitoring while only five of 10 children had a positive response to head-up tilt test.

Conclusions: When a child with a normal resting electrocardiogram is referred with a typical history of syncope, the use of ambulatory blood pressure monitoring as a non-invasive first step for diagnosis of postural hypotension may be more sensitive than the head-up tilt test. Behavioural adjustments resolved the continued syncope in most cases. If episodes persist then the head-up tilt test is indicated.
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http://dx.doi.org/10.1017/S1047951110001551DOI Listing
February 2011

Long-term cardiovascular effects of pre-transplant native kidney nephrectomy in children.

Pediatr Nephrol 2010 Dec 25;25(12):2523-9. Epub 2010 Sep 25.

Division of Pediatric Nephrology, Bambino Gesú Hospital, Rome, Italy.

Left ventricular (LV) hypertrophy (H) and hypertension are prevalent in children with end-stage renal disease (ESRD) and after renal transplantation. Severe hypertension prior to renal transplantation has traditionally been an indication for native kidney nephrectomy. The impact of nephrectomy on cardiovascular disease has not been well documented. We retrospectively evaluated echocardiographic and ambulatory blood pressure monitoring (ABPM) data in 67 young adults who had undergone transplantation in the pediatric age with a mean follow-up of 10.4 years. Unilateral or bilateral nephrectomies had been performed in 32 patients. The number of antihypertensive drugs used prior to transplantation was significantly higher in the nephrectomized groups. At follow-up the amount of antihypertensive medications was similar between groups and no significant differences were observed in mean arterial blood pressure (MAP) or LV mass index (LVMi). LVH was observed in 50% of non-nephrectomized patients, 45.4% of patients with unilateral nephrectomy, and 44.4% of patients without native kidneys (p = n.s.). In conclusion, unilateral or bilateral nephrectomies prior to transplantation do not appear to influence blood pressure control or the prevalence of LVH after renal transplantation. Longitudinal studies with repeated assessment of LVMi, before and after renal transplantation, are needed to assess the impact of residual activity of native kidneys on arterial blood pressure and cardiac structural changes, even in normotensive patients, to evaluate cardiovascular morbidity.
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http://dx.doi.org/10.1007/s00467-010-1638-3DOI Listing
December 2010

Mid-term results, and therapeutic management, for patients suffering hypertension after surgical repair of aortic coarctation.

Cardiol Young 2009 Sep 13;19(5):451-5. Epub 2009 Aug 13.

Cardiorespiratory and Sports Medicine Unit, Department of Pediatrics, Bambino Gesù Children's Hospital, Rome, Italy.

We designed our study to investigate the efficacy of a new therapeutic approach to late onset hypertension in patients after surgical repair of aortic coarctation. Several studies have shown a higher incidence of hypertension during daily activities, and during exercise, in patients after surgical correction of coarctation. To the best of our knowledge, however, no data exists concerning haemodynamics, the response of arterial pressures, and the effects of medications for lowering blood pressure during exercise or during daily activities.We studied 128 patients, aged 15.6 +/- 4.3 years, to determine the response of blood pressure as we administered treatment in the attempt to achieve a normotensive state. We excluded patient with associated cardiac abnormalities, apart from those with bicuspid aortic valves. We evaluated blood pressure at rest in both the right arm and leg to establish presence of any gradient, as well as the blood pressure in the arm during exercise testing, and by 24-hour ambulatory monitoring.Atenolol was prescribed for those with elevated values of blood pressure but with a normal increment of heart rate during exercise. We prescribed Candesartan for those with elevated levels of blood pressure but with reduced increments of heart rate, specifically maximal heart rates of less than 85% of their predicted value. Both drugs were used when one alone was not effective. We found that, in young patients, candesartan provided better control of blood pressure with no side-effects, especially as demonstrated using 24-hour ambulatory monitoring, while atenolol was less effective, with more side-effects. Our experience suggests that both drugs should be used in patients who are non-responsive to monotherapy.
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http://dx.doi.org/10.1017/S1047951109990734DOI Listing
September 2009

Insulin resistance and exercise capacity in male children and adolescents with non-alcholic fatty liver disease.

Acta Diabetol 2009 Jun 7;46(2):97-104. Epub 2008 Oct 7.

Scientific Directorate, Bambino Gesù Children's Hospital and Research Institute, S. Onofrio 4 square, 00165, Rome, Italy.

Insulin resistance (IR) and obesity may be associated with impaired response to physical exercise. We aimed at assessing physical capacity in obese children with biopsy proven non-alcoholic fatty liver disease (NAFLD) as compared to normal weight and obese children without fatty liver disease. All male subjects, 20 NAFLD and 31 control individuals (20 obese, without NAFLD and 11 normal weight children) took part in the study. We evaluated changes in cardiovascular parameters during a bicycle-ergometer exercise test (James' test). Duration, power of exercise, heart rate (HR), blood pressure (BP), pulse pressure, cardiac output ((I)CO) and total peripheral vascular resistance indexed for height ((I)TPVR) were recorded at rest ((r)) and peak ((p)) exercise. The homeostatic model assessment was used to determine insulin resistance (HOMA-IR) and beta-cell action (HOMA-beta cell). In NAFLD and obese subjects, fasting leptin, insulin secretion, insulinogenic index (IGI), muscle insulin sensitivity (MISI) and hepatic insulin resistance index (HIRI) were assayed. Children with NAFLD were the most insulin-resistant (P = 0.001), and showed higher HIRI than obese controls (P = 0.05). At rest, they had the lowest values of SBP(r) (P = 0.001 vs. controls and P < or = 0.05 vs. obese controls); during the test, the highest values of (I)CO(p) (P = 0.005), Delta(I)CO (P = 0.003) and DeltaTRVP(p) (P < or = 0.0001). NAFLD and obese controls both had impaired DeltaHR(p) (P < or = 0.0001). However, obese controls were not able to reduce peripheral resistance during the test. HOMA-IR explained 28% of variance in Delta(I)CO of the whole sample, (P < or = 0.0001). In obese children with or without NAFLD, increased IR and body weight may induce cardiovascular compensatory changes in response to physical exercise with fairly different pathogenetic mechanisms, which are likely to be dependent on the different degree of IR.
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http://dx.doi.org/10.1007/s00592-008-0063-6DOI Listing
June 2009

The influence of different surgical procedures on hypertension after repair of coarctation.

Cardiol Young 2005 Oct;15(5):477-80

Cardiorespiratory Medicine and Sports Unit, Bambino Gesù Children's Hospital - Research Institute, Rome, Italy.

We measured resting and exercise haemodynamics, as well as 24-hour ambulatory blood pressure, so as to study the influence on development of hypertension in children after repair of coarctation by either construction of a subclavian flap or end-to-end anastamosis. The patients in both groups were studied a mean time of 13 years after surgery. Thus, we divided 43 children who had undergone surgical repair of coarctation, and who were not on antihypertensive therapy, into a group of 22 patients who had undergone subclavian flap repair, with a mean age of 14 plus or minus 2.6 years, and another group of 21 patients undergoing end-to-end anastomosis, with a mean age of 13.5 plus or minus 3.9 years. We examined blood pressure at rest and during exercise, along with the measurement of cardiac output using impedance cardiography, and during 24-hour ambulatory monitoring. We recorded systolic and diastolic blood pressures, pulse pressure, cardiac output and total peripheral vascular resistance at rest and at peak exercise. During ambulatory monitoring, we measured mean pressures over 24 hours, in daytime and nighttime, 24-hour pulse pressure, and 24-hour mean arterial pressure. Student's t test was used to judge significance, accepting this when p was less than 0.05. The group repaired using the subclavian flap showed significantly disadvantageous differences for diastolic blood pressure at rest, systolic blood pressure at peak exercise and for 24-hour systolic and diastolic blood pressure, 24-hour mean arterial pressure, and daytime and nighttime systolic blood pressure during ambulatory monitoring. Our findings suggest that, after repair using the subclavian flap in comparison to end-to-end anastomosis, patients show a higher incidence of late hypertension, both during exercise and ambulatory monitoring. The data indicate different residual aortic stiffnesses, these being lower after end-to-end anastomosis, which may be due to the greater resection of the abnormal aortic tissue when coarctation is repaired using the latter technique.
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http://dx.doi.org/10.1017/S1047951105001332DOI Listing
October 2005

Blood pressure in the long-term follow-up of children with hemolytic uremic syndrome.

Pediatr Nephrol 2004 Nov;19(11):1241-4

Department of Nephrology and Urology, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.

The hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure (ARF) in young children. Most patients recover from the acute phase of the illness but they may develop arterial hypertension(AH) after many years, even in the absence of signs of renal impairment during short-term follow-up. In this study, we performed casual blood pressure (BP) measurement, 24-h blood pressure monitoring (ABPM), and a Bruce walking treadmill study (ET) in 24 children (aged 5-15 years, 13 males, 11 females) with a history of HUS and normal renal function during follow-up (median 5.8 years, range 1.8-12.4 years). There were 22 children(91%) with prodromal diarrhea associated with HUS and 20 (83%) underwent dialysis during the acute illness. All children had normal casual BP measurement. Of 13 children (54%) with normal ABPM, 5 patients (38%) had an abnormal BP response during the ET study. There were 4 (58%) of the 7 patients with AH by ABPM (29%)and an abnormal BP response during ET. These findings suggest that ET could be a useful means of identifying children with a history of HUS that could be at risk of future AH even if they had normal renal function, casual BP, and ABPM during long-term follow-up. These results should be confirmed with a large prospective clinical study.
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http://dx.doi.org/10.1007/s00467-004-1582-1DOI Listing
November 2004

Exercise-induced left bundle branch block in a young female athlete.

Cardiol Young 2003 Aug;13(4):367-9

Sports Medicine Unit, Department of Pediatrics, Bambino Gesù Children's Hospital, Research Institute, Rome, Italy.

An 11-year-old female, in apparent good health, was referred to our Institute because of exercise-induced left bundle branch block detected during a Master's step test performed to obtain the clearance for competitive sport. The tests that we performed to investigate the possible causes were all negative. We suspect, however, that the left bundle branch block is the initial sign of cardiomyopathy. If our suspicions are correct, close follow-up is important to chart the possible progression of the disease.
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August 2003

Exercise blood pressure response, cardiac output and 24-hour ambulatory blood pressure monitoring in children after aortic coarctation repair.

Ital Heart J 2003 Jun;4(6):408-12

Sports Medicine Unit, Department of Pediatrics, Bambino Gesù Children's Hospital, Research Institute, Rome, Italy.

Background: The aim of this study was to assess blood pressure at rest, the response to exercise and the 24-hour ambulatory blood pressure monitoring (ABPM) profile in children operated for aortic coarctation.

Methods: Twenty children were operated upon for aortic coarctation. The patients' data were compared with those obtained from 19 healthy controls of the same age. Treadmill exercise testing was performed and cardiac output was determined using the acetylene-rebreathing method and indexed for the body surface area; ABPM was performed only in the patients group. The main outcome measures were the time of exercise, systolic (SBP) and diastolic (DBP) blood pressure both at rest and at peak exercise, maximal heart rate, total peripheral vascular resistance at rest and at peak exercise, and the pulse pressure (PP = SBP-DBP) at rest, at peak exercise and at ABPM. The Mann-Whitney test (non-parametric) and linear regression analysis were used when appropriate.

Results: Patients compared with healthy controls showed significant differences in SBP and PP at rest, and in DBP, cardiac index, total peripheral vascular resistance and PP at peak exercise. In the patients group only, linear regression analysis showed a significant correlation between PP and cardiac output, both at rest and at peak exercise, and between the arm-leg gradient at rest and PP at ABPM.

Conclusions: These findings suggest that blood pressure abnormalities could be due both to the altered baroceptor reflex control mechanism, resulting in cardiac output and total peripheral vascular resistance abnormalities, and to the progressive increase in resistance during exercise at the site of the repair, resulting in the higher PP, that may be related to a local loss of the natural aortic elasticity.
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June 2003

Total peripheral vascular resistance in pediatric renal transplant patients.

Kidney Int 2002 Nov;62(5):1870-4

Department of Pediatric Nephrology and Unit of Sports Medicine, Bambino Gesù Children Research Hospital, Rome, Italy.

Background: Abnormal cardiovascular reactivity at rest and during physical exercise may be a risk factor for left ventricular hypertrophy (LVH) in pediatric renal transplanted (Tx) patients. Data on total peripheral vascular resistance (TPR) are not available.

Methods: Eleven renal Tx patients treated with cyclosporine (7 females and 4 males; mean age 14.6 +/- 3.3 years; mean time since transplantation 43 +/- 35 months) were evaluated for 24-hour blood pressure (BP), TPR and echocardiographic left ventricular mass (LVM). TPR values of patients were compared with data of a group of 11 healthy controls matched for sex and age.

Results: Twenty-four-hour ambulatory blood pressure monitoring showed that all but one patient had normal daytime BP values and six patients showed a reduced or inverse nocturnal dip. LVH was found in 72% of the patients. In comparison with healthy controls, patients showed significantly elevated TPR at rest and during exercise suggesting an increased vascular tone. The degree of LVH in these patients is severe and appears disproportionate to the BP values.

Conclusion: The high incidence of LVH can reflect an augmented cardiovascular reactivity associated with a disturbed circadian pattern. The increase in TPR and the reduction of the nocturnal fall of BP also might contribute to the development of LVH in young renal Tx patients.
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http://dx.doi.org/10.1046/j.1523-1755.2002.00639.xDOI Listing
November 2002