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2.
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
3.
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
4.
Ital J Pediatr ; 43(1): 111, 2017 Dec 12.
Artigo em Inglês | MEDLINE | ID: mdl-29233182

RESUMO

Five years after the first edition, we have revised and updated the guidelines, re-examining the queries and relative recommendations, expanding the issues addressed with the introduction of a new entity, recently proposed by the American Academy of Pediatrics: BRUE, an acronym for Brief Resolved Unexplained Events. In this manuscript we will use the term BRUE only to refer to mild, idiopathic cases rather than simply replace the acronym ALTE per se.In our guidelines the acronym ALTE is used for severe cases that are unexplainable after the first and second level examinations.Although the term ALTE can be used to describe the common symptoms at the onset, whenever the aetiology is ascertained, the final diagnosis may be better specified as seizures, gastroesophageal reflux, infection, arrhythmia, etc. Lastly, we have addressed the emerging problem of the so-called Sudden Unexpected Postnatal Collapse (SUPC), that might be considered as a severe ALTE occurring in the first week of life.


Assuntos
Apneia/diagnóstico , Causas de Morte , Cianose/diagnóstico , Doenças do Recém-Nascido/diagnóstico , Guias de Prática Clínica como Assunto , Morte Súbita do Lactente/prevenção & controle , Apneia/mortalidade , Cianose/mortalidade , Emergências , Medicina Baseada em Evidências , Feminino , Humanos , Lactente , Recém-Nascido , Doenças do Recém-Nascido/mortalidade , Itália , Masculino , Medição de Risco , Análise de Sobrevida
5.
JAMA ; 316(18): 1906-1912, 2016 Nov 08.
Artigo em Inglês | MEDLINE | ID: mdl-27825009

RESUMO

Importance: Anakinra, an interleukin 1ß recombinant receptor antagonist, may have potential to treat colchicine-resistant and corticosteroid-dependent recurrent pericarditis. Objective: To determine the efficacy of anakinra for colchicine-resistant and corticosteroid-dependent recurrent pericarditis. Design, Setting, and Participants: The Anakinra-Treatment of Recurrent Idiopathic Pericarditis (AIRTRIP) double-blind, placebo-controlled, randomized withdrawal trial (open label with anakinra followed by a double-blind withdrawal step with anakinra or placebo until recurrent pericarditis occurred) conducted among 21 consecutive patients enrolled at 3 Italian referral centers between June and November 2014 (end of follow-up, October 2015). Included patients had recurrent pericarditis (with ≥3 previous recurrences), elevation of C-reactive protein, colchicine resistance, and corticosteroid dependence. Interventions: Anakinra was administered at 2 mg/kg per day, up to 100 mg, for 2 months, then patients who responded with resolution of pericarditis were randomized to continue anakinra (n = 11) or switch to placebo (n = 10) for 6 months or until a pericarditis recurrence. Main Outcomes and Measures: The primary outcomes were recurrent pericarditis and time to recurrence after randomization. Results: Eleven patients (7 female) randomized to anakinra had a mean age of 46.5 (SD, 16.3) years; 10 patients (7 female) randomized to placebo had a mean age of 44 (SD, 12.5) years. All patients were followed up for 12 months. Median follow-up was 14 (range, 12-17) months. Recurrent pericarditis occurred in 9 of 10 patients (90%; incidence rate, 2.06% of patients per year) assigned to placebo and 2 of 11 patients (18.2%; incidence rate, 0.11% of patients per year) assigned to anakinra, for an incidence rate difference of -1.95% (95% CI, -3.3% to -0.6%). Median flare-free survival (time to flare) was 72 (interquartile range, 64-150) days after randomization in the placebo group and was not reached in the anakinra group (P <.001). During anakinra treatment, 20 of 21 patients (95.2%) experienced transient local skin reactions: 1 (4.8%) herpes zoster, 3 (14.3%) transaminase elevation, and 1 (4.8%) ischemic optic neuropathy. No patient permanently discontinued the active drug. No adverse events occurred during placebo treatment. Conclusion and Relevance: In this preliminary study of patients with recurrent pericarditis with colchicine resistance and corticosteroid dependence, the use of anakinra compared with placebo reduced the risk of recurrence over a median of 14 months. Larger studies are needed to replicate these findings as well as to assess safety and longer-term efficacy. Trial Registration: clinicaltrials.gov Identifier: NCT02219828.


Assuntos
Desenvolvimento Infantil , Cognição , Fórmulas Infantis , Recém-Nascido de muito Baixo Peso/crescimento & desenvolvimento , Leite Humano , Método Duplo-Cego , Feminino , Humanos , Lactente , Recém-Nascido , Recém-Nascido Prematuro , Desenvolvimento da Linguagem , Masculino , Ontário
6.
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
7.
Ital J Pediatr ; 40: 65, 2014 Oct 24.
Artigo em Inglês | MEDLINE | ID: mdl-25344148

RESUMO

Acute bronchiolitis is the leading cause of lower respiratory tract infection and hospitalization in children less than 1 year of age worldwide. It is usually a mild disease, but some children may develop severe symptoms, requiring hospital admission and ventilatory support in the ICU. Infants with pre-existing risk factors (prematurity, bronchopulmonary dysplasia, congenital heart diseases and immunodeficiency) may be predisposed to a severe form of the disease. Clinical diagnosis of bronchiolitis is manly based on medical history and physical examination (rhinorrhea, cough, crackles, wheezing and signs of respiratory distress). Etiological diagnosis, with antigen or genome detection to identify viruses involved, may have a role in reducing hospital transmission of the infection. Criteria for hospitalization include low oxygen saturation (<90-92%), moderate-to-severe respiratory distress, dehydration and presence of apnea. Children with pre-existing risk factors should be carefully assessed.To date, there is no specific treatment for viral bronchiolitis, and the mainstay of therapy is supportive care. This consists of nasal suctioning and nebulized 3% hypertonic saline, assisted feeding and hydration, humidified O2 delivery. The possible role of any pharmacological approach is still debated, and till now there is no evidence to support the use of bronchodilators, corticosteroids, chest physiotherapy, antibiotics or antivirals. Nebulized adrenaline may be sometimes useful in the emergency room. Nebulized adrenaline can be useful in the hospital setting for treatment as needed. Lacking a specific etiological treatment, prophylaxis and prevention, especially in children at high risk of severe infection, have a fundamental role. Environmental preventive measures minimize viral transmission in hospital, in the outpatient setting and at home. Pharmacological prophylaxis with palivizumab for RSV bronchiolitis is indicated in specific categories of children at risk during the epidemic period. Viral bronchiolitis, especially in the case of severe form, may correlate with an increased incidence of recurrent wheezing in pre-schooled children and with asthma at school age.The aim of this document is to provide a multidisciplinary update on the current recommendations for the management and prevention of bronchiolitis, in order to share useful indications, identify gaps in knowledge and drive future research.


Assuntos
Bronquiolite/terapia , Antagonistas de Receptores Adrenérgicos beta 2/uso terapêutico , Antibacterianos/uso terapêutico , Anticorpos Monoclonais Humanizados/uso terapêutico , Antivirais/uso terapêutico , Bronquiolite/diagnóstico , Broncodilatadores/uso terapêutico , Tomada de Decisões , Exposição Ambiental/prevenção & controle , Epinefrina/uso terapêutico , Glucocorticoides/uso terapêutico , Hospitalização , Humanos , Umidade , Recém-Nascido , Unidades de Terapia Intensiva Neonatal , Nebulizadores e Vaporizadores , Oxigenoterapia , Palivizumab , Alta do Paciente , Atenção Primária à Saúde , Terapia Respiratória , Solução Salina Hipertônica/administração & dosagem , Índice de Gravidade de Doença , Vitamina D/uso terapêutico , Vitaminas/uso terapêutico
8.
Int J Dermatol ; 52(9): 1140-6, 2013 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-23829783

RESUMO

OBJECTIVE: To assess propranolol efficacy and safety in complicated infantile hemangiomas in two different age groups. PATIENTS AND METHODS: We report on 68 infants with infantile hemangiomas treated with oral propranolol at the lowest effective dose at different ages for a period of six months. Inclusion criteria were life-threatening hemangiomas, function-threatening hemangiomas, facial hemangiomas with risk for disfigurement, and extensive and ulcerated hemangiomas. A previously designed safety protocol was applied to all patients. The evolution of all hemangiomas since baseline (pre-therapy) until the end of follow-up was assessed on the basis of clinical features (color, palpable softening, size, and volume) and taken at follow-up visits. RESULTS: Our results showed that propranolol was effective in arresting the proliferative phase and in accelerating the involution of infantile hemangiomas in 92.6% of cases. Propranolol efficacy was clear even when it was started after 12 months of life at low dose; after discontinuation of therapy there was a moderate-to-severe regrowth in 9.3% of cases and a mild regrowth in 22.5%. No adverse events were observed. CONCLUSIONS: Propranolol should be used as first-line medical treatment in all cases of complicated infantile hemangiomas.


Assuntos
Dermatoses Faciais/tratamento farmacológico , Hemangioma/tratamento farmacológico , Propranolol/administração & dosagem , Neoplasias Cutâneas/tratamento farmacológico , Administração Oral , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Masculino , Propranolol/efeitos adversos , Resultado do Tratamento , Vasodilatadores/administração & dosagem , Vasodilatadores/efeitos adversos
9.
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
10.
Audiol Neurootol ; 13(1): 1-6, 2008.
Artigo em Inglês | MEDLINE | ID: mdl-17715463

RESUMO

Facioscapulohumeral muscular dystrophy (FSHD) is an autosomal dominant progressive myopathy, characteristically associated with a 4q35 deletion. In the unusual infantile-onset form of this degenerative disease, sensorineural hearing loss is a frequent clinical manifestation, whereas in patients with typical late-onset FSHD, investigations regarding hearing impairment yielded controversial results. We describe the findings of a multicenter investigation on possible auditory impairment in a series of 73 FSHD patients with a genetically confirmed diagnosis. Among them, 49 cases with no risk factors for deafness, aside from the disease, were identified by a clinical questionnaire and otoscopic examination (mean age 37.8 years, 31 males and 18 females). These subjects were evaluated by pure-tone audiometry. None were aware of hearing loss, while 4 had raised unilateral or bilateral pure-tone audiometric thresholds at 4000 and 8000 Hz, when evaluated by standardized tables. However, the mean raw pure-tone audiometric threshold values for these 49 cases were not significantly different from those of 55 controls (mean age 37.1 years, 32 males and 23 females). Moreover, by statistical analysis, age of onset, degree of muscular weakness and 4q35 EcoRI fragment size made no significant difference to auditory thresholds in our FSHD patients. Overall, the results of our multicenter study suggest that hearing loss in typical FSHD is not more prevalent than in the normal population.


Assuntos
Cromossomos Humanos Par 4 , Perda Auditiva Neurossensorial/epidemiologia , Perda Auditiva Neurossensorial/genética , Distrofia Muscular Facioescapuloumeral/epidemiologia , Distrofia Muscular Facioescapuloumeral/genética , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Audiometria de Tons Puros , Feminino , Rearranjo Gênico , Predisposição Genética para Doença/epidemiologia , Perda Auditiva Neurossensorial/diagnóstico , Humanos , Masculino , Pessoa de Meia-Idade , Prevalência , Fatores de Risco
11.
Clin Dysmorphol ; 15(2): 65-70, 2006 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-16531730

RESUMO

Tetralogy of Fallot associated with the atrioventricular canal defect has been usually reported in association with Down syndrome. The aim of the present study was to describe the cardiac aspects and the genetic anomalies in children with this association of heart defects. We identified 64 patients with atrioventricular canal defect tetralogy of Fallot. All children underwent complete cardiovascular, clinical phenotypic and genetic evaluation. A genetic syndrome or extracardiac anomalies were found in 56 patients (87.5%). Down syndrome (43 patients, 67.2%) was the most frequent genetic diagnosis. Other syndromes were 8p deletion, trisomy 13, duplication 5p, cranio-cerebello-cardiac syndrome, Cantrell syndrome, CHARGE association, VACTERL association, and DiGeorge syndrome related to maternal diabetes. No patients in our series had 22q11 deletion. Tetralogy of Fallot with extreme dextroposition of the aorta was found in seven patients (only one with Down syndrome). Additional cardiac malformations were present in 23 patients (only 11 with Down syndrome). The association between atrioventricular canal defect and tetralogy of Fallot represents a cardiac phenotype with strong genetic characteristics. For this reason, a careful genetic examination is required. Our study confirms the high prevalence of Down syndrome, but also reveals a significant genetic heterogeneity. Additional cardiac defects are prevalent in patients without Down syndrome.


Assuntos
Comunicação Atrioventricular/complicações , Comunicação Atrioventricular/genética , Heterogeneidade Genética , Tetralogia de Fallot/complicações , Tetralogia de Fallot/genética , Adolescente , Adulto , Criança , Pré-Escolar , Comunicação Atrioventricular/epidemiologia , Feminino , Humanos , Incidência , Lactente , Masculino , Fenótipo
12.
J Interv Cardiol ; 17(3): 183-7, 2004 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-15209582

RESUMO

Unilateral pulmonary vein (PV) atresia is a rare congenital cardiac malformation with evolution toward irreversible pulmonary hypertension. Pneumonectomy or lung transplant is currently the treatment of choice for such a disease. We describe an unusual case of right PV atresia and major aorto-pulmonary collaterals treated with percutaneous angioplasty, stent implantation, and aorto-pulmonary collateral embolization.


Assuntos
Angioplastia com Balão , Circulação Colateral/fisiologia , Embolização Terapêutica , Veias Pulmonares/anormalidades , Stents , Pré-Escolar , Feminino , Humanos
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