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1.
Pediatrics ; 144(4)2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31481415

RESUMO

Diagnostic point-of-care ultrasound (POCUS) is a growing field across all disciplines of pediatric practice. Machine accessibility and portability will only continue to grow, thus increasing exposure to this technology for both providers and patients. Individuals seeking training in POCUS should first identify their scope of practice to determine appropriate applications within their clinical setting, a few of which are discussed within this article. Efforts to build standardized POCUS infrastructure within specialties and institutions are ongoing with the goal of improving patient care and outcomes.

2.
Lancet Diabetes Endocrinol ; 7(9): 695-706, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31377265

RESUMO

BACKGROUND: Deficiency of the thyroid hormone transporter monocarboxylate transporter 8 (MCT8) causes severe intellectual and motor disability and high serum tri-iodothyronine (T3) concentrations (Allan-Herndon-Dudley syndrome). This chronic thyrotoxicosis leads to progressive deterioration in bodyweight, tachycardia, and muscle wasting, predisposing affected individuals to substantial morbidity and mortality. Treatment that safely alleviates peripheral thyrotoxicosis and reverses cerebral hypothyroidism is not yet available. We aimed to investigate the effects of treatment with the T3 analogue Triac (3,3',5-tri-iodothyroacetic acid, or tiratricol), in patients with MCT8 deficiency. METHODS: In this investigator-initiated, multicentre, open-label, single-arm, phase 2, pragmatic trial, we investigated the effectiveness and safety of oral Triac in male paediatric and adult patients with MCT8 deficiency in eight countries in Europe and one site in South Africa. Triac was administered in a predefined escalating dose schedule-after the initial dose of once-daily 350 µg Triac, the daily dose was increased progressively in 350 µg increments, with the goal of attaining serum total T3 concentrations within the target range of 1·4-2·5 nmol/L. We assessed changes in several clinical and biochemical signs of hyperthyroidism between baseline and 12 months of treatment. The prespecified primary endpoint was the change in serum T3 concentrations from baseline to month 12. The co-primary endpoints were changes in concentrations of serum thyroid-stimulating hormone (TSH), free and total thyroxine (T4), and total reverse T3 from baseline to month 12. These analyses were done in patients who received at least one dose of Triac and had at least one post-baseline evaluation of serum throid function. This trial is registered with ClinicalTrials.gov, number NCT02060474. FINDINGS: Between Oct 15, 2014, and June 1, 2017, we screened 50 patients, all of whom were eligible. Of these patients, four (8%) patients decided not to participate because of travel commitments. 46 (92%) patients were therefore enrolled in the trial to receive Triac (median age 7·1 years [range 0·8-66·8]). 45 (98%) participants received Triac and had at least one follow-up measurement of thyroid function and thus were included in the analyses of the primary endpoints. Of these 45 patients, five did not complete the trial (two patients withdrew [travel burden, severe pre-existing comorbidity], one was lost to follow-up, one developed of Graves disease, and one died of sepsis). Patients required a mean dose of 38.3 µg/kg of bodyweight (range 6·4-84·3) to attain T3 concentrations within the target range. Serum T3 concentration decreased from 4·97 nmol/L (SD 1·55) at baseline to 1·82 nmol/L (0·69) at month 12 (mean decrease 3·15 nmol/L, 95% CI 2·68-3·62; p<0·0001), while serum TSH concentrations decreased from 2·91 mU/L (SD 1·68) to 1·02 mU/L (1·14; mean decrease 1·89 mU/L, 1·39-2·39; p<0·0001) and serum free T4 concentrations decreased from 9·5 pmol/L (SD 2·5) to 3·4 (1·6; mean decrease 6·1 pmol/L (5·4-6·8; p<0·0001). Additionally, serum total T4 concentrations decreased by 31·6 nmol/L (28·0-35·2; p<0·0001) and reverse T3 by 0·08 nmol/L (0·05-0·10; p<0·0001). Seven treatment-related adverse events (transiently increased perspiration or irritability) occurred in six (13%) patients. 26 serious adverse events that were considered unrelated to treatment occurred in 18 (39%) patients (mostly hospital admissions because of infections). One patient died from pulmonary sepsis leading to multi-organ failure, which was unrelated to Triac treatment. INTERPRETATION: Key features of peripheral thyrotoxicosis were alleviated in paediatric and adult patients with MCT8 deficiency who were treated with Triac. Triac seems a reasonable treatment strategy to ameliorate the consequences of untreated peripheral thyrotoxicosis in patients with MCT8 deficiency. FUNDING: Dutch Scientific Organization, Sherman Foundation, NeMO Foundation, Wellcome Trust, UK National Institute for Health Research Cambridge Biomedical Centre, Toulouse University Hospital, and Una Vita Rara ONLUS.

6.
Pediatr Res ; 84(Suppl 1): 68-77, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30072805

RESUMO

Pulmonary hypertension contributes to morbidity and mortality in both the term newborn infant, referred to as persistent pulmonary hypertension of the newborn (PPHN), and the premature infant, in the setting of abnormal pulmonary vasculature development and arrested growth. In the term infant, PPHN is characterized by the failure of the physiological postnatal decrease in pulmonary vascular resistance that results in impaired oxygenation, right ventricular failure, and pulmonary-to-systemic shunting. The pulmonary vasculature is either maladapted, maldeveloped, or underdeveloped. In the premature infant, the mechanisms are similar in that the early onset pulmonary hypertension (PH) is due to pulmonary vascular immaturity and its underdevelopment, while late onset PH is due to the maladaptation of the pulmonary circulation that is seen with severe bronchopulmonary dysplasia. This may lead to cor-pulmonale if left undiagnosed and untreated. Neonatologist performed echocardiography (NPE) should be considered in any preterm or term neonate that presents with risk factors suggesting PPHN. In this review, we discuss the risk factors for PPHN in term and preterm infants, the etiologies, and the pathophysiological mechanisms as they relate to growth and development of the pulmonary vasculature. We explore the applications of NPE techniques that aid in the correct diagnostic and pathophysiological assessment of the most common neonatal etiologies of PPHN and provide guidelines for using these techniques to optimize the management of the neonate with PPHN.

7.
Pediatr Res ; 84(Suppl 1): 1-12, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30072808

RESUMO

Cardiac ultrasound techniques are increasingly used in the neonatal intensive care unit to guide cardiorespiratory care of the sick newborn. This is the first in a series of eight review articles discussing the current status of "neonatologist-performed echocardiography" (NPE). The aim of this introductory review is to discuss four key elements of NPE. Indications for scanning are summarized to give the neonatologist with echocardiography skills a clear scope of practice. The fundamental physics of ultrasound are explained to allow for image optimization and avoid erroneous conclusions from artifacts. To ensure patient safety during echocardiography recommendations are given to prevent cardiorespiratory instability, hypothermia, infection, and skin lesions. A structured approach to echocardiography, with the same standard views acquired in the same sequence at each scan, is suggested in order to ensure that the neonatologist confirms normal structural anatomy or acquires the necessary images for a pediatric cardiologist to do so when reviewing the scan.

8.
Pediatr Res ; 84(Suppl 1): 57-67, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30072807

RESUMO

One of the major challenges of neonatal intensive care is the early detection and management of circulatory failure. Routine clinical assessment of the hemodynamic status of newborn infants is subjective and inaccurate, emphasizing the need for objective monitoring tools. An overview will be provided about the use of neonatologist-performed echocardiography (NPE) to assess cardiovascular compromise and guide hemodynamic management. Different techniques of central blood flow measurement, such as left and right ventricular output, superior vena cava flow, and descending aortic flow are reviewed focusing on methodology, validation, and available reference values. Recommendations are provided for individualized hemodynamic management guided by NPE.

9.
Pediatr Res ; 84(Suppl 1): 13-17, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30072809

RESUMO

There is a growing interest worldwide in using echocardiography in the neonatal unit to act as a complement to the clinical assessment of the hemodynamic status of premature and term infants. However, there is a wide variation in how this tool is implemented across many jurisdictions, the level of expertise, including the oversight of this practice. Over the last 5 years, three major expert consensus statements have been published to provide guidance to neonatologists performing echocardiography, with all recommending a structured training program and clinical governance system for quality assurance. Neonatal practice in Europe is very heterogeneous and the proximity of neonatal units to pediatric cardiology centers varies significantly. Currently, there is no overarching governance structure for training and accreditation in Europe. In this paper, we provide a brief description of the current training recommendations across several jurisdictions including Europe, North America, and Australia and describe the steps required to achieve a sustainable governance structure with the responsibility to provide accreditation to neonatologist performed echocardiography in Europe.

10.
Front Pediatr ; 6: 140, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29868528

RESUMO

The hemodynamic changes during the first few breaths after birth are probably the most significant and drastic adaptation in the human life. These changes are critical for a smooth transition of fetal to neonatal circulation. With the cord clamping, lungs take over as the source of oxygenation from placenta. A smooth transition of circulation is a complex mechanism and primarily depends upon the drop in pulmonary vascular resistance (PVR) and increase in systemic vascular resistance (SVR). Understanding the normal transition physiology and the adverse adaptation is of utmost importance to the clinicians looking after neonates. It may have a significant influence on the presentation of congenital heart defects (CHDs) in infants. Bedside echocardiography may help in understanding the transition physiology, especially the hemodynamic changes and shunting across ductus arteriosus and foramen ovale, and it may play an important role in making judicious clinical decisions based upon the altered physiology.

11.
Indian Pediatr ; 55(5): 417-424, 2018 May 15.
Artigo em Inglês | MEDLINE | ID: mdl-29845957

RESUMO

The role of functional echocardiography in neonatal intensive care unit is rapidly evolving, and increasingly neonatologists are using it in making clinical decisions in sick infants. Functional echocardiography can provide a direct assessment of hemodynamics on bedside, and may be considered as an extension of the clinical examination to evaluate cardiovascular wellbeing in the critically-ill infant. The physiological information may be used in targeting specific intervention based upon the underlying pathophysiology. Functional echocardiography is being used for diagnosis of pulmonary hypertension, patent ductus arteriosus, hemodynamic evaluation, assessment of cardiac function, and recognition of pericardial effusion and cardiac tamponade in the neonatal intensive care setting. Despite of its increasing popularity, there is a paucity of structured training programs for neonatologists to acquire skills in echocardiography. This review article discusses clinical applications of functional echocardiography in the neonatal intensive care unit.


Assuntos
Ecocardiografia/métodos , Cardiopatias/diagnóstico por imagem , Hipertensão Pulmonar/diagnóstico por imagem , Cardiopatias/fisiopatologia , Hemodinâmica , Humanos , Hipertensão Pulmonar/fisiopatologia , Recém-Nascido , Unidades de Terapia Intensiva Neonatal , Neonatologia/métodos , Sistemas Automatizados de Assistência Junto ao Leito
13.
Front Pediatr ; 6: 79, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29670871

RESUMO

In the neonatal and pediatric intensive care setting, bedside cardiac ultrasound is often used to assess ventricular dimensions and function. Depending upon the underlying disease process, it is necessary to be able to evaluate the systolic and diastolic function of left and or right ventricles. The systolic function of left ventricle is mostly assessed qualitatively on visual inspection "eye-balling" and quantitatively by measuring circumferential fraction shortening or calculating the ejection fraction by Simpson's planimetry. The assessment of left ventricular diastolic function relies essentially on the mitral valve and pulmonary venous Doppler tracings or tissue Doppler evaluation. The right ventricular particular shape and anatomical position does not permit to use the same parameters for measuring systolic function as is used for the LV. Tricuspid annular plane systolic excursion (TAPSE) and S' velocity on tissue Doppler imaging are more often used for quantitative assessment of right ventricle systolic function. Several parameters proposed to assess right ventricle systolic function such as fractional area change, 3D echocardiography, speckle tracking, and strain rate are being researched and normal values for children are being established. Diastolic function of right ventricle is evaluated by tricuspid valve and hepatic venous Doppler tracings or on tissue Doppler evaluation. The normal values for children are pretty similar to adults while normal values for the neonates, especially preterm infants, may differ significantly from adult population. The normal values for most of the parameters used to assess cardiac function in term neonates and children have now been established.

14.
Front Pediatr ; 6: 2, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29404312

RESUMO

Shock in newborn infants has unique etiopathologic origins that require careful assessment to direct specific interventions. Early diagnosis is key to successful management. Unlike adults and pediatric patients, shock in newborn infants is often recognized in the uncompensated phase by the presence of hypotension, which may be too late. The routine methods of evaluation used in the adult and pediatric population are often invasive and less feasible. We aim to discuss the pathophysiology in shock in newborn infants, including the transitional changes at birth and unique features that contribute to the challenges in early identification. Special emphasis has been placed on bedside focused echocardiography/focused cardiac ultrasound, which can be used as an additional tool for early, neonatologist driven, ongoing evaluation and management. An approach to goal oriented management of shock has been described and how bed side functional echocardiography can help in making a logical choice of intervention (fluid therapy, inotropic therapy or vasopressor therapy) in infants with shock.

15.
Arch Dis Child Educ Pract Ed ; 103(3): 137-140, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29162633

RESUMO

Congenital heart disease (CHD) remains a leading cause of infant mortality, which is even higher in infants with undiagnosed duct-dependent CHDs. Up to 39%-50% of infants with critical CHD are being discharged undiagnosed from the hospital. Infants with duct-dependent critical CHD remain well during the fetal period and may deteriorate when the ductus arteriosus (commonly called 'duct') closes after birth. It is critical to open or maintain ductus arteriosus patent in infants with duct-dependent CHDs. Prostaglandin E1 (alprostadil marketed as 'Prostin VR ') and prostaglandin E2 (dinoprostone) are used to maintain a patent ductus arteriosus and the dose of medication depends on the clinical presentation. Delay in starting prostaglandin infusion can have deleterious effects on infants and can even lead to death. These infants often present as an emergency, and professionals caring for these infants need to have a good understanding of these conditions and medications used for ductal patency.

16.
Front Pediatr ; 5: 201, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28966921

RESUMO

Hemodynamic instability and inadequate cardiac performance are common in critically ill children. The clinical assessment of hemodynamic status is reliant upon physical examination supported by the clinical signs such as heart rate, blood pressure, capillary refill time, and measurement of the urine output and serum lactate. Unfortunately, all of these parameters are surrogate markers of cardiovascular well-being and they provide limited direct information regarding the adequacy of blood flow and tissue perfusion. A bedside point-of-care echocardiography can provide real-time hemodynamic information by assessing cardiac function, loading conditions (preload and afterload) and cardiac output. The echocardiography has the ability to provide longitudinal functional assessment in real time, which makes it an ideal tool for monitoring hemodynamic assessment in neonates and children. It is indispensable in the management of patients with shock, pulmonary hypertension, and patent ductus arteriosus. The echocardiography is the gold standard diagnostic tool to assess hemodynamic stability in patients with pericardial effusion, cardiac tamponade, and cardiac abnormalities such as congenital heart defects or valvar disorders. The information from echocardiography can be used to provide targeted treatment in intensive care settings such as need of fluid resuscitation versus inotropic support, choosing appropriate inotrope or vasopressor, and in providing specific interventions such as selective pulmonary vasodilators in pulmonary hypertension. The physiological information gathered from echocardiography may help in making timely, accurate, and appropriate diagnosis and providing specific treatment in sick patients. There is no surprise that use of bedside point-of-care echocardiography is rapidly gaining interest among neonatologists and intensivists, and it is now being used in clinical decision making for patients with hemodynamic instability. Like any other investigation, it has certain limitations and the most important limitation is its intermittent nature. Sometimes acquiring high quality images for precise functional assessment in a ventilated child can be challenging. Therefore, it should be used in conjunction with the existing tools (physical examination and clinical parameters) for hemodynamic assessment while making clinical decisions.

18.
Eur J Pediatr ; 175(2): 281-7, 2016 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-26362538

RESUMO

UNLABELLED: Targeted echocardiographic assessments of haemodynamic status are increasingly utilised in many settings. Application in the neonatal intensive care units (NICU) is increasingly demanded but challenging given the risk of underlying structural lesions. This statement follows discussions in UK led by the Neonatologists with an Interest in Cardiology and Haemodynamics (NICHe) group in collaboration with the British Congenital Cardiac Association (BCCA) and the Paediatricians with Expertise in Cardiology Special Interest Group (PECSIG). Clear consensus was agreed on multiple aspects of best practice for neonatologist-performed echocardiogram (NoPE)-rigorous attention to infection control and cardiorespiratory/thermal stability, early referral to paediatric cardiology with suspicion of structural disease, reporting on standardised templates, reliable image storage, regular skills maintenance, collaboration with a designated paediatric cardiologist, and regular scan audit/review. It was agreed that NoPE assessments should confidently exclude structural lesions at first scan. Practitioners would be expected to screen and establish gross normality of structure at first scan and obtain confirmation from paediatric cardiologist if required, and subsequently, functional echocardiography can be performed for haemodynamic assessment to guide management of newborn babies. To achieve training, NICHe group suggested that mandatory placements could be undertaken during core registrar training or neonatal subspecialty grid training with a paediatric cardiology placement for 6 months and a neonatology placement for a minimum of 6 months. In the future, we hope to define a precise curriculum for assessments. Technological advances may provide solutions-improvements in telemedicine may have neonatologists assessing haemodynamic status with paediatric cardiologists excluding structural lesions and neonatal echocardiography simulators could increase exposure to multiple pathologies and allow limitless practice in image acquisition. CONCLUSION: We propose developing training places in specialist paediatric cardiology centres and neonatal units to facilitate training and suggest all UK practitioners performing neonatologist-performed echocardiogram adopt this current best practice statement. WHAT IS KNOWN: Neonatologist-performed echocardiogram (NoPE) also known as targeted neonatal echocardiography (TNE) or functional ECHO is increasingly recognised and utilised in care of sick newborn and premature babies. There are differences in training for echocardiography across continents and formal accreditation processes are lacking. WHAT IS NEW: This is the first document of consensus best practice statement for training of neonatologists in neonatologist-performed echocardiogram (NoPE), jointly drafted by Neonatologists with interest in cardiology & haemodynamics (NICHe), paediatric cardiology and paediatricians with expertise in cardiology interest groups in UK. Key elements of a code of practice for neonatologist-performed echocardiogram are suggested.


Assuntos
Acreditação/normas , Cardiologia/educação , Ecocardiografia/normas , Neonatologia/educação , Consenso , Cardiopatias Congênitas/diagnóstico por imagem , Humanos , Recém-Nascido , Unidades de Terapia Intensiva Neonatal , Garantia da Qualidade dos Cuidados de Saúde , Reino Unido
19.
Cardiol Young ; 26(3): 528-31, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-25990755

RESUMO

UNLABELLED: Following the Safe and Sustainable review of Paediatric Services in 2012/2013, National Health Service England recommended that local paediatric cardiology services should be provided by specially trained paediatricians with expertise in cardiology in all non-specialist hospitals. AIM: To understand the variation in local paediatric cardiology services provided across district general hospitals in the United Kingdom. STUDY DESIGN AND METHODS: An internet-based questionnaire was sent out via the Paediatrician with Expertise in Cardiology Special Interest Group and the Neonatologists with Interest in Cardiology and Haemodynamics contact databases and the National Health Service directory. Non-responders were followed-up via telephone. RESULTS: The response rate was 80% (141 of 177 hospitals), and paediatricians with expertise in cardiology were available in 68% of those. Local cardiology clinics led by paediatricians with expertise in cardiology were provided in 96 hospitals (68%), whereas specialist outreach clinics were held in 123 centres (87%). A total of 11 hospitals provided neither specialist outreach clinics nor any local cardiology clinics led by paediatricians with expertise in cardiology. Paediatric echocardiography services were provided in 83% of the hospitals, 12-lead electrocardiogram in 96%, Holter electrocardiogram in 91%, and exercise testing in only 47% of the responding hospitals. Telemedicine facilities were established in only 52% of the centres, where sharing echocardiogram images via picture archiving and communication system was used most commonly. CONCLUSION: There has been a substantial increase in the availability of paediatricians with expertise in cardiology since 2008. Most of the hospitals are well-supported by specialist cardiology centres via outreach clinics; however, there remains significant variation in the local paediatric cardiology services provided across district general hospitals in the United Kingdom.


Assuntos
Cardiologia , Hospitais Gerais/estatística & dados numéricos , Pediatras/provisão & distribução , Pediatria , Ecocardiografia , Eletrocardiografia , Hospitais Gerais/organização & administração , Humanos , Inquéritos e Questionários , Telemedicina , Reino Unido , Recursos Humanos
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