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1.
Eur J Pediatr ; 181(1): 83-90, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-34196792

RESUMO

The measurement of sodium intake may be important for the management of hypertension. Dietary surveys and 24-h urinary collection are often unreliable and/or impractical. We hypothesized that urinary sodium excretion can be accurately estimated through multiple spot urine samples from different days. All enrolled subjects were children of the coauthors of the study. Fifty-two 24-h urinary collections (4 per subject) for measuring sodium excretion and the 297 related urinary samples (1 per voiding) were collected for calculating the urinary sodium/urinary creatinine ratio in 13 children. The mean of 4 measured sodium excretions served as the individual "gold standard". Twenty-four urinary collections were used to generate the equation predicting the mean measured sodium excretion from the mean of 4 urinary sodium/urinary creatinine [= 0.016 × urinary sodium (mmol/L) / urinary creatinine (mmol/L) ratio + 3.3)]; the remaining 28 urinary collections and 153 urinary samples were used for the external validation. All subjects underwent an additional validation procedure involving 12 urinary samples randomly collected on different days 6 months apart. The performance of sodium excretion calculated from a total of over 22,000 possible means of 4 out of all the available urinary samples, randomly taken on different days, was analyzed as to precision (by means of the coefficient of variation) and as to accuracy (by means of the P30). The coefficients of variations of measured vs. calculated sodium excretion were 25.3% vs. 25.8%, and the P30 of calculated sodium excretion was 100%. The excellent performance of calculated sodium excretion was confirmed both by external validation and by samples collected 6 months apart with mean P30s, all between 86 and 100%.Conclusion: In the described experimental conditions, urinary sodium excretion was estimated with equal precision and more accurately (and practically) by the mean of 4 urinary sodium/urinary creatinine ratios from random samples from different days than by a single urinary collection. In real life, with several errors systematically affecting urinary collection, the superiority of calculated sodium excretion is likely to be even greater. What is Known: • The measurement of sodium intake with the current standards of care (dietary survey or 24-h urinary collection) is laborious and can be inaccurate. What is New: • The study provides evidence that sodium intake can be estimated equally precisely, more accurately and more practically with the urinary sodium-to-urinary creatinine ratio from 4 urine samples taken on different days than with a single urinary collection.


Assuntos
Hipertensão , Sódio na Dieta , Criança , Creatinina , Dieta , Humanos , Sódio , Urinálise , Coleta de Urina
2.
Pediatr Nephrol ; 35(4): 687-693, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-31900633

RESUMO

BACKGROUND: This contribution aims to report and analyze a novel approach for office blood pressure measurement in children. METHODS: Healthy children 5 to 8 years of age were eligible. After 5 minutes rest, 10 unattended blood pressure readings were taken at 3-minute intervals using a validated automated oscillometric device. After discarding outlier values (< 5th or > 95th percentile of the recorded values), the coefficient of variation and the mean of the 10 readings were calculated. The single readings #1 to #10 were compared with this elaborated average of the 10 measurements. RESULTS: Two hundred eighty-one healthy, non-obese children (137 females, 49%), median age 5.7 (IQR 5.3-6.1) years, were analyzed. The median coefficients of variation were 7% (IQR 5-9) for systolic and 4% (IQR 3-6) for diastolic blood pressure. The first 3 measurements were significantly different from the average, while the readings #4 to #10 were not. Based on the average, only nine subjects had a systolic or diastolic blood pressure > 90th centile (n = 3 > 95th percentile). CONCLUSIONS: Although most guidelines advise three blood pressure readings, these findings suggest that in children, office blood pressure measurement might be improved by including ten measurements. In situations of time constraints, the fourth blood pressure reading might be used as a reliable approximation.


Assuntos
Determinação da Pressão Arterial/métodos , Pressão Sanguínea/fisiologia , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Visita a Consultório Médico/estatística & dados numéricos , Hipertensão do Jaleco Branco/prevenção & controle
4.
Eur J Pediatr ; 177(9): 1383-1388, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29387982

RESUMO

It is well known that the nephron endowment of healthy subjects is highly variable and that individual nephron mass has potentially important implications both in health and disease. However, nephron count is technically impossible in living subjects. Based on the observation of an increase in serum creatinine (sCr) in otherwise healthy newborns with solitary kidney during the physiological perinatal dehydration, we hypothesized that perinatal sCr might be helpful in identifying healthy subjects with a reduced nephron mass. In the framework of a study on blood pressure in babies (NeoNeph), sCr of normal Caucasian neonates was determined 48-96 h after birth and their association with a family history of arterial hypertension (AH) was analyzed. SCr was determined in 182 normal newborns (90 males) at a mean of 61 ± 8 h after birth (range 46-82). Newborns with paternal AH had a higher mean sCr (0.97 + 0.28 mg/dL) then newborns without paternal AH (0.73 + 0.28 mg/dL; p = 0.006). No differences in mean sCr were found in relation with mother or grandparent's history of AH. CONCLUSION: The association between parental AH and high sCr during perinatal dehydration supports the hypothesis that the latter is a promising tool for identifying normal subjects with a reduced nephron mass with potential important implications in prevention and in understanding the individual outcome of renal and extrarenal diseases (including AH). What is Known: • Nephron endowment of healthy subjects is highly variable and individual nephron mass has potentially important implications both in health and disease however nephron count is not feasible in living subjects. What is New: • Serum creatinine during perinatal dehydration is a possible biomarker for identifying normal subjects with a reduced nephron mass.


Assuntos
Creatinina/sangue , Desidratação/sangue , Hipertensão/complicações , Néfrons/fisiopatologia , Biomarcadores/sangue , Desidratação/complicações , Feminino , Humanos , Recém-Nascido , Masculino , Gravidez
5.
Pharmacology ; 99(1-2): 75-78, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-27756073

RESUMO

Propranolol is becoming the treatment of choice for complicated infantile hemangioma. We report here data on peripheral blood flow, O2-saturation, electrocardiographic PR-interval, left ventricular function, blood pressure and heart rate that were assessed before and during treatment for ≥4 weeks with propranolol 2 mg/kg of body weight daily in 67 infants <12 months of age in normal sinus rhythm and with structurally normal hearts. Management with propranolol was well tolerated in all and did not modify peripheral blood flow, O2-saturation, electrocardiographic PR-interval and left ventricular fractional shortening or ejection fraction. Absolute blood pressure levels were similar without and with propranolol. However, age-adjusted centile levels for both systolic and diastolic levels were significantly lower while on propranolol. The heart rate was significantly lower both when expressed as absolute value and when expressed as age-adjusted centile on treatment with propranolol. In conclusion, propranolol 2 mg/kg of body weight daily causes a statistically though not clinically relevant decrease in blood pressure and heart rate in cardially healthy infants affected by infantile hemangioma. Temporary discontinuation during acute febrile illnesses and during diarrheal diseases should be considered to prevent excessive hypotension.


Assuntos
Antagonistas Adrenérgicos beta/farmacologia , Pressão Sanguínea/efeitos dos fármacos , Frequência Cardíaca/efeitos dos fármacos , Hemangioma/tratamento farmacológico , Propranolol/farmacologia , Função Ventricular Esquerda/efeitos dos fármacos , Antagonistas Adrenérgicos beta/uso terapêutico , Pressão Sanguínea/fisiologia , Relação Dose-Resposta a Droga , Monitoramento de Medicamentos/métodos , Feminino , Frequência Cardíaca/fisiologia , Hemangioma/fisiopatologia , Humanos , Lactente , Masculino , Propranolol/uso terapêutico , Função Ventricular Esquerda/fisiologia
7.
Pediatr Cardiol ; 37(8): 1581-1589, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-27573216

RESUMO

Children affected by hemodynamically significant congenital heart disease (HSCHD) experience severe respiratory complications that can increase the frequency of hospitalizations. The aim of the SINERGY study was to describe the incidence of respiratory diseases and to collect information on active and passive immunoprophylaxis in the first 2 years of life. In this retrospective, multicenter, and epidemiologic study, children with HSCHD were enrolled across 11 Italian sites. Children born between December 31, 2007, and December 31, 2012, were observed during their first 2 years of life. Data were collected through hospital database searches and parent interviews. Four hundred twenty children were enrolled: 51.7 % were female, 79.5 % were born full-term (≥37 weeks), and 77.6 % weighed >2500 g at birth. The most frequent heart defects were ventricular septal defect (23.1 %) and coarctation of the aorta (14.3 %). The incidence of respiratory diseases was 63.1 %. Frequent respiratory diseases not requiring hospitalization were upper respiratory tract infections (76.4 %), acute bronchitis (43.3 %), and influenza (22.1 %), while those requiring hospitalization were bronchitis and bronchiolitis (8.3 % each one). While active immunoprophylaxis was applied with wide compliance (diphtheria/pertussis/tetanus, 99.5 %; Haemophilus influenzae type b, 72.5 %; pneumococcus, 79.9 %; meningococcus, 77.4 %), only 54 % of children received respiratory syncytial virus (RSV) passive prophylaxis (palivizumab). Of the 35 hospitalizations due to bronchiolitis, 27 (77.1 %) did not receive prophylaxis against RSV, compared with 8 (22.9 %) who received prophylaxis (P < 0.0001). Children with HSCHD are at major risk of respiratory diseases. Passive immunoprophylaxis can help to prevent hospitalizations for bronchiolitis.


Assuntos
Cardiopatias Congênitas , Anticorpos Monoclonais , Anticorpos Monoclonais Humanizados , Antivirais , Criança , Feminino , Hospitalização , Humanos , Incidência , Itália , Masculino , Infecções por Vírus Respiratório Sincicial , Estudos Retrospectivos
8.
Pediatr Nephrol ; 30(2): 345-52, 2015 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-25149851

RESUMO

BACKGROUND: Shigatoxin-associated hemolytic uremic syndrome (STEC-HUS) is a common thrombotic microangiopathy (TMA) in which central nervous system (CNS) involvement is responsible for the majority of deaths and for severe long-term sequelae. We have analyzed the role of hemoconcentration in disease severity. METHODS: This was a retrospective review of the records and laboratory data at presentation of all patients with STEC-HUS cases (n = 61) over a 10-year period. The patients were grouped into three severity classes: group A, comprising patients who did not require dialysis; group B, patients who were dialyzed without CNS involvement; group C, patients with CNS involvement. RESULTS: Patients with CNS involvement (group C) had a higher mean hemoglobin level (11.2 ± 2.3 g/dL) than those of group A or B ( 9.4 ± 2.1 and 7.5 ± 1.9 g/dL, respectively; p < 0.0001). We also observed that the higher the initial hemoglobin level, the more severe the long-term renal damage (p < 0.007). CONCLUSIONS: In patients with STEC-HUS, hemoconcentration and hypovolemia may be responsible for more severe ischemic organ damage (both short and long term) at disease onset, and these signs should be regarded as risk factors for CNS damage and for more severe TMA. Therefore, we recommend that hydration status should be actively monitored in HUS patients and that dehydration, when diagnosed, should be promptly corrected.


Assuntos
Síndrome Hemolítico-Urêmica/sangue , Síndrome Hemolítico-Urêmica/complicações , Doenças do Sistema Nervoso/etiologia , Adolescente , Criança , Pré-Escolar , Feminino , Hidratação/efeitos adversos , Hematócrito , Humanos , Lactente , Masculino , Estudos Retrospectivos , Fatores de Risco
9.
J Hum Hypertens ; 36(6): 554-560, 2022 06.
Artigo em Inglês | MEDLINE | ID: mdl-33935283

RESUMO

A reduced nephron number may play a role in the pathogenesis of arterial hypertension (AH), and it is well recognized that individual nephron endowment is widely variable. However, nephrons count is technically impossible in vivo. Based on the observation that subjects with a reduced nephron mass exhibit an increase in renal functional biomarkers during acute dehydration, we hypothesized that cystatin C concentration during neonatal physiological dehydration could identify subjects with reduced nephron endowment. This is a prospective, observational, cohort study enrolling healthy, caucasian, term neonates born after an uneventful pregnancy. Two groups of newborns were compared: neonates born to fathers on antihypertensive treatment (HF) versus those born to proven normotensive fathers older than 40 years of age (NF). Enrolled newborns underwent cystatin C determination at the time of newborn screening. Forty newborns with HF and 80 with NF were enrolled. No differences in baseline characteristics were observed between the two groups except for the number of hypertensive grandparents higher among newborns to HF (47.8% vs. 21.1%; p: 0.001). Cystatin C was significantly higher in newborns with HF (1.62 ± 0.30 mg/L vs 1.41 ± 0.27 mg/L; p < 0.001). Linear regression analysis corrected for confounders confirmed that paternal hypertension was the only variable significantly associated with high cystatin C level during post-natal dehydration. Besides offering new insights on the pathogenesis of familial hypertension, our results support the specific role of nephron endowment and suggest the possibility of identifying subjects at risk for reduced nephron endowment as early as at birth.


Assuntos
Cistatina C , Hipertensão , Estudos de Coortes , Desidratação/complicações , Desidratação/patologia , Feminino , Humanos , Hipertensão/etiologia , Recém-Nascido , Néfrons/patologia , Gravidez
10.
J Hypertens ; 40(10): 1902-1908, 2022 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-35983863

RESUMO

OBJECTIVE: Percutaneous transluminal renal angioplasty (PTRA), the recommended treatment in children with renovascular hypertension (RVH), often has unsatisfactory outcomes. Cutting balloons may improve the results of angioplasty in different vascular beds with complex and resistant lesions. We retrospectively analysed the effects of percutaneous cutting balloon angioplasty (PCBA) on blood pressure, cardiac mass and renal artery acceleration time in children/adolescents referred to our centre for RVH. PATIENTS AND METHODS: Thirteen patients (aged 9-19 years) with renal artery stenosis (RAS) and severe hypertension were identified. RASs were focal fibromuscular (FMD) or FMD-like dysplasia (in six cases bilateral, in five associated with mid aortic syndrome). Ten patients had uncontrolled hypertension, in nine cases associated with left ventricular hypertrophy (LVH). Acceleration time was abnormal in all stenotic arteries. Eighteen PCBA were performed, in three arteries associated with stent implantation. RESULTS: PCBA was technically successful in all individuals without major complications. In one patient, an intra-stent restenosis occurred, successfully redilated with conventional angioplasty without recurrence at 4 years distance. One year after PCBA, mean SBP and DBPs were markedly reduced from 146 ±â€Š25 to 121 ±â€Š10 mmHg and from 87 ±â€Š11 to 65 ±â€Š12 mmHg, respectively ( P  < 0.001 for both). At that time, hypertension was cured in seven children and controlled in five individuals. This favourable outcome was confirmed with ambulatory blood pressure measurement in four patients. At the latest follow-up, left ventricular mass and acceleration time were normal in all patients. CONCLUSION: PCBA proved to be a well tolerated and effective procedure that can be considered as an alternative to PTRA to treat hypertensive children/adolescents with recurrent or resistant RAS.


Assuntos
Angioplastia com Balão , Hipertensão Renovascular , Hipertensão , Obstrução da Artéria Renal , Adolescente , Angioplastia/efeitos adversos , Angioplastia com Balão/efeitos adversos , Angioplastia com Balão/métodos , Monitorização Ambulatorial da Pressão Arterial , Criança , Humanos , Hipertensão/complicações , Hipertensão Renovascular/etiologia , Hipertensão Renovascular/terapia , Artéria Renal , Obstrução da Artéria Renal/complicações , Obstrução da Artéria Renal/cirurgia , Estudos Retrospectivos
11.
J Hypertens ; 40(1): 171-179, 2022 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-34857709

RESUMO

BACKGROUND: Sodium intake is known to contribute to the development of hypertension, thus intake reduction is a cornerstone in the prevention and management of hypertension. The increase in renal sodium excretion might represent a further potential preventive and/or therapeutic opportunity. OBJECTIVE: To explore the working hypothesis that an increased fluid intake can improve renal sodium handling towards a decrease in blood pressure. METHODS: The SPA Project is a multicenter, observational, cross-sectional, cohort study investigating healthy children, aged 5-8 years as to sodium and fluid intake by means of urinary sodium and creatinine from multiple samples taken in different days in order to characterize them in lower/higher sodium and lower/higher fluid intake. Both SBP and DBP (by multiple office blood pressure measurements) were used as outcome measures. RESULTS: Three hundred and thirty-nine healthy, nonoverweight children (51.6% boys) with a median age of 5.7 years old (IQR: 5.3-6.2) participated in the study but only 223 could be analyzed. Among children with higher sodium intake, those introducing more fluids, showed a significantly lower blood pressure (both systolic and diastolic) compared with those with lower fluid intake: systolic 86.0 ±â€Š8.5 vs. 90.0 ±â€Š8.1 mmHg; P = 0.014 and diastolic: 53.8 ±â€Š4.9 vs. 58.6 ±â€Š6.6 mmHg; P < 0.0001. CONCLUSION: An increased fluid intake is associated with a reduced blood pressure possibly by increasing renal sodium excretion. We speculate that this simple, highly acceptable, inexpensive, and harmless measure might have a role in preventing and/or minimizing the epidemics of hypertension and of its related morbidities both in children and in adults.


Assuntos
Hipertensão , Adulto , Pressão Sanguínea , Criança , Pré-Escolar , Estudos de Coortes , Estudos Transversais , Feminino , Humanos , Hipertensão/prevenção & controle , Masculino , Sódio
12.
Ital J Pediatr ; 47(1): 16, 2021 Jan 25.
Artigo em Inglês | MEDLINE | ID: mdl-33494789

RESUMO

Aim of these revised recommendations for the general management of Kawasaki disease is to encourage its prompter recognition and warrant the most appropriate therapy, based on ascertained scientific data, raising awareness of the complications related to misdiagnosis or delayed treatment. A set of 20 synthetic operative statements is herein provided, including the definition of Kawasaki disease, its protean presentations, clinical course and seminal treatment modalities of all disease phases. The application of these recommendations should improve prognosis of Kawasaki disease and prevent the progression to permanent vascular abnormalities, thereby diminishing morbidity and mortality.


Assuntos
Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/terapia , Criança , Diagnóstico Diferencial , Progressão da Doença , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Itália , Prognóstico
13.
Circulation ; 120(18): 1761-7, 2009 Nov 03.
Artigo em Inglês | MEDLINE | ID: mdl-19841298

RESUMO

BACKGROUND: The prevalence of genetic arrhythmogenic diseases is unknown. For the long-QT syndrome (LQTS), figures ranging from 1:20 000 to 1:5000 were published, but none was based on actual data. Our objective was to define the prevalence of LQTS. METHODS AND RESULTS: In 18 maternity hospitals, an ECG was performed in 44 596 infants 15 to 25 days old (43 080 whites). In infants with a corrected QT interval (QTc) >450 ms, the ECG was repeated within 1 to 2 weeks. Genetic analysis, by screening 7 LQTS genes, was performed in 28 of 31 (90%) and in 14 of 28 infants (50%) with, respectively, a QTc >470 ms or between 461 and 470 ms. A QTc of 451 to 460, 461 to 470, and >470 ms was observed in 177 (0.41%), 28 (0.06%), and 31 infants (0.07%). Among genotyped infants, disease-causing mutations were found in 12 of 28 (43%) with a QTc >470 ms and in 4 of 14 (29%) with a QTc of 461 to 470 ms. One genotype-negative infant (QTc 482 ms) was diagnosed as affected by LQTS on clinical grounds. Among family members of genotype-positive infants, 51% were found to carry disease-causing mutations. In total, 17 of 43 080 white infants were affected by LQTS, demonstrating a prevalence of at least 1:2534 apparently healthy live births (95% confidence interval, 1:1583 to 1:4350). CONCLUSIONS: This study provides the first data-based estimate of the prevalence of LQTS among whites. On the basis of the nongenotyped infants with QTc between 451 and 470 ms, we advance the hypothesis that this prevalence might be close to 1:2000. ECG-guided molecular screening can identify most infants affected by LQTS and unmask affected relatives, thus allowing effective preventive measures.


Assuntos
Síndrome do QT Longo/epidemiologia , Síndrome do QT Longo/genética , Mutação , Análise Mutacional de DNA , Eletrocardiografia , Saúde da Família , Genótipo , Humanos , Recém-Nascido , Programas de Rastreamento , Prevalência , Estudos Prospectivos
14.
BMC Infect Dis ; 10: 305, 2010 Oct 24.
Artigo em Inglês | MEDLINE | ID: mdl-20969802

RESUMO

BACKGROUND: Although the most frequent extra-pulmonary manifestations of respiratory syncytial virus (RSV) infection involve the cardiovascular system, no data regarding heart function in infants with bronchiolitis associated with RSV infection have yet been systematically collected. The aim of this study was to verify the real frequency of heart involvement in patients with bronchiolitis associated with RSV infection, and whether infants with mild or moderate disease also risk heart malfunction. METHODS: A total of 69 otherwise healthy infants aged 1-12 months with bronchiolitis hospitalised in standard wards were enrolled. Pernasal flocked swabs were performed to collect specimens for the detection of RSV by real-time polymerase chain reaction, and a blood sample was drawn to assess troponin I concentrations. On the day of admission, all of the infants underwent 24-hour Holter ECG monitoring and a complete heart evaluation with echocardiography. Patients were re-evaluated by investigators blinded to the etiological and cardiac findings four weeks after enrollment. RESULTS: Regardless of their clinical presentation, sinoatrial blocks were identified in 26/34 RSV-positive patients (76.5%) and 1/35 RSV-negative patients (2.9%) (p < 0.0001). The blocks recurred more than three times over 24 hours in 25/26 RSV-positive patients (96.2%) and none of the RSV-negative infants. Mean and maximum heart rates were significantly higher in the RSV-positive infants (p < 0.05), as was low-frequency power and the low and high-frequency power ratio (p < 0.05). The blocks were significantly more frequent in the children with an RSV load of ≥100,000 copies/mL than in those with a lower viral load (p < 0.0001). Holter ECG after 28 ± 3 days showed the complete regression of the heart abnormalities. CONCLUSIONS: RSV seems associated with sinoatrial blocks and transient rhythm alterations even when the related respiratory problems are mild or moderate. Further studies are needed to clarify the mechanisms of these rhythm problems and whether they remain asymptomatic and transient even in presence of severe respiratory involvement or chronic underlying disease.


Assuntos
Bronquiolite/complicações , Bronquiolite/patologia , Frequência Cardíaca , Infecções por Vírus Respiratório Sincicial/complicações , Infecções por Vírus Respiratório Sincicial/patologia , Vírus Sincicial Respiratório Humano/isolamento & purificação , Bloqueio Sinoatrial/diagnóstico , Bronquiolite/virologia , Ecocardiografia , Feminino , Coração/fisiopatologia , Humanos , Lactente , Masculino , Nariz/virologia , Infecções por Vírus Respiratório Sincicial/virologia , Troponina/sangue
15.
Am J Med Genet A ; 149A(6): 1268-72, 2009 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-19449412

RESUMO

Congenital heart defects (CHDs) have been estimated to occur in approximately 20% of patients with Brachmann-de Lange syndrome (BDLS, also known as Cornelia de Lange syndrome, OMIM 122470). We report on the results of a prospective echocardiographic evaluation of a cohort of 87 Italian BDLS patients with longitudinal follow-up from 5 to 12 years. A cardiac anomaly was identified in 29/87 (33.3%) including 28 (32.2%) patients with a structural CHD, and an additional patient (1.2%) with isolated non-obstructive hypertrophic cardiomyopathy (HCM). Of the 28 patients with a CHD, 12 (42.9%) had an isolated obstructive CHD, 10 of which were pulmonary stenosis (36%), 8 (28.6%) had an isolated left to right shunt, and the remainder showed a combination of structural anomalies. Overall incidence of pulmonary stenosis was 39% (11/28). Isolated late-onset mitral or tricuspid valve dysplasia, albeit hemodynamically insignificant, was detected at follow-up examination in 4 (14.3%) patients older than 10 years, previously known to be normal. In contrast to previous studies, only two patients required surgery, one for closure of a large perimembranous ventricular septal defect (VSD) and associated ASD closure (1), and another for VSD closure and relief of pulmonary valve stenosis (1). The remainder are receiving medical follow-up. We believe that the overall frequency (33.3%) and evidence of 4 late onset dysplastic valves anomalies justifies both echocardiographic assessment in all BDLS patients at the first diagnostic assessment, and later on during medical follow-up.


Assuntos
Síndrome de Cornélia de Lange/terapia , Cardiopatias Congênitas/terapia , Comunicação Interatrial/terapia , Doenças das Valvas Cardíacas/terapia , Estenose da Valva Pulmonar/terapia , Adolescente , Adulto , Criança , Estudos de Coortes , Síndrome de Cornélia de Lange/complicações , Ecocardiografia/métodos , Feminino , Seguimentos , Cardiopatias Congênitas/complicações , Comunicação Interatrial/complicações , Doenças das Valvas Cardíacas/complicações , Humanos , Lactente , Itália/epidemiologia , Estudos Longitudinais , Masculino , Estudos Prospectivos , Estenose da Valva Pulmonar/complicações , Obstrução do Fluxo Ventricular Externo/complicações , Obstrução do Fluxo Ventricular Externo/terapia , Adulto Jovem
16.
Ital J Pediatr ; 44(1): 103, 2018 Aug 30.
Artigo em Inglês | MEDLINE | ID: mdl-30157893

RESUMO

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.


Assuntos
Anti-Inflamatórios não Esteroides/uso terapêutico , Doenças Cardiovasculares/tratamento farmacológico , Resistência a Medicamentos , Imunoglobulinas Intravenosas/uso terapêutico , Síndrome de Linfonodos Mucocutâneos/tratamento farmacológico , Guias de Prática Clínica como Assunto , Doenças Cardiovasculares/diagnóstico , Doenças Cardiovasculares/epidemiologia , Criança , Pré-Escolar , Doença da Artéria Coronariana/diagnóstico , Doença da Artéria Coronariana/tratamento farmacológico , Doença da Artéria Coronariana/epidemiologia , Feminino , Seguimentos , Humanos , Masculino , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/epidemiologia , Pediatria , Medição de Risco , Índice de Gravidade de Doença , Sociedades Médicas
17.
Ital J Pediatr ; 44(1): 102, 2018 Aug 30.
Artigo em Inglês | MEDLINE | ID: mdl-30157897

RESUMO

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.


Assuntos
Imunoglobulinas Intravenosas/administração & dosagem , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/terapia , Guias de Prática Clínica como Assunto , Doença Aguda , Gerenciamento Clínico , Progressão da Doença , Feminino , Humanos , Itália , Masculino , Síndrome de Linfonodos Mucocutâneos/epidemiologia , Pediatria/normas , Prognóstico , Ensaios Clínicos Controlados Aleatórios como Assunto , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Sociedades Médicas , Resultado do Tratamento
18.
Ital J Pediatr ; 42(1): 69, 2016 Jul 16.
Artigo em Inglês | MEDLINE | ID: mdl-27423331

RESUMO

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.


Assuntos
Monitorização Ambulatorial da Pressão Arterial/métodos , Exercício Físico/fisiologia , Hipertensão/diagnóstico , Hipertensão/tratamento farmacológico , Estilo de Vida , Adolescente , Fatores Etários , Determinação da Pressão Arterial/métodos , Criança , Pré-Escolar , Dieta com Restrição de Carboidratos , Humanos , Hipertensão/prevenção & controle , Lactente , Pediatria , Prevenção Primária/métodos , Prognóstico , Fatores de Risco , Papel (figurativo) , Resultado do Tratamento , Ácido Úrico/efeitos adversos
19.
Pediatrics ; 137(1)2016 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-26644486

RESUMO

BACKGROUND: Hemolytic uremic syndrome associated with Shiga toxin-producing Escherichia coli (STEC-HUS) is a severe acute illness without specific treatment except supportive care; fluid management is concentrated on preventing fluid overload for patients, who are often oligoanuric. Hemoconcentration at onset is associated with more severe disease, but the benefits of volume expansion after hemolytic uremic syndrome (HUS) onset have not been explored. METHODS: All the children with STEC-HUS referred to our center between 2012 and 2014 received intravenous infusion targeted at inducing an early volume expansion (+10% of working weight) to restore circulating volume and reduce ischemic or hypoxic tissue damage. The short- and long-term outcomes of these patients were compared with those of 38 historical patients referred to our center during the years immediately before, when fluid intake was routinely restricted. RESULTS: Patients undergoing fluid infusion soon after diagnosis showed a mean increase in body weight of 12.5% (vs 0%), had significantly better short-term outcomes with a lower rate of central nervous system involvement (7.9% vs 23.7%, P = .06), had less need for renal replacement therapy (26.3% vs 57.9%, P = .01) or intensive care support (2.0 vs. 8.5 days, P = .02), and needed fewer days of hospitalization (9.0 vs 12.0 days, P = .03). Long-term outcomes were also significantly better in terms of renal and extrarenal sequelae (13.2% vs 39.5%, P = .01). CONCLUSIONS: Patients with STEC-HUS had great benefit from early volume expansion. It is speculated that early and generous fluid infusions can reduce thrombus formation and ischemic organ damage, thus having positive effects on both short- and long-term disease outcomes.


Assuntos
Hidratação , Síndrome Hemolítico-Urêmica/terapia , Criança , Pré-Escolar , Intervenção Médica Precoce , Feminino , Síndrome Hemolítico-Urêmica/microbiologia , Humanos , Lactente , Masculino , Estudos Retrospectivos , Índice de Gravidade de Doença , Escherichia coli Shiga Toxigênica , Resultado do Tratamento
20.
Pediatr Infect Dis J ; 23(6): 559-63, 2004 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-15194840

RESUMO

The impact of highly active antiretroviral therapy (HAART) on cardiovascular involvement was evaluated in 38 vertically human immunodeficiency virus-infected children followed up for 5 years. This study demonstrates for the first time in a cohort of children the resolution of previous dilated cardiomyopathy after the start of HAART and the absence of cardiovascular events related to metabolic abnormalities during the period of its administration.


Assuntos
Terapia Antirretroviral de Alta Atividade/efeitos adversos , Doenças Cardiovasculares/induzido quimicamente , Infecções por HIV/tratamento farmacológico , Distribuição por Idade , Análise de Variância , Terapia Antirretroviral de Alta Atividade/métodos , Doenças Cardiovasculares/diagnóstico , Doenças Cardiovasculares/epidemiologia , Criança , Pré-Escolar , Estudos de Coortes , Relação Dose-Resposta a Droga , Esquema de Medicação , Ecocardiografia , Eletrocardiografia , Feminino , Infecções por HIV/congênito , Humanos , Incidência , Itália/epidemiologia , Estudos Longitudinais , Masculino , Probabilidade , Prognóstico , Medição de Risco , Índice de Gravidade de Doença , Distribuição por Sexo
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