scholarly journals State-of-the art review: Noncompaction cardiomyopathy in pediatric patients

2021 ◽  
Author(s):  
Sofie Rohde ◽  
Rahatullah Muslem ◽  
Emrah Kaya ◽  
Michel Dalinghaus ◽  
Jaap I. van Waning ◽  
...  
Keyword(s):  
Pediatric Patients ◽  
American Heart ◽  
State Of The Art ◽  
Heart Diseases ◽  
Pediatric Population ◽  
Adult Population ◽  
Neuromuscular Diseases ◽  
Heart Association ◽  
Noncompaction Cardiomyopathy

AbstractNoncompaction cardiomyopathy (NCCM) is a disease characterized by hypertrabeculation, commonly hypothesized due to an arrest in compaction during fetal development. In 2006, NCCM was classified as a distinct form of cardiomyopathy (CMP) by the American Heart Association. NCCM in childhood is more frequently familial than when diagnosed in adulthood and is associated with other congenital heart diseases (CHDs), other genetic CMPs, and neuromuscular diseases (NMDs). It is yet a rare cardiac diseased with an estimated incidence of 0.12 per 100.000 in children up to 10 years of age. Diagnosing NCCM can be challenging due to non-uniform diagnostic criteria, unawareness, presumed other CMPs, and presence of CHD. Therefore, the incidence of NCCM in children might be an underestimation. Nonetheless, NCCM is the third most common cardiomyopathy in childhood and is associated with heart failure, arrhythmias, and/or thromboembolic events. This state-of-the-art review provides an overview on pediatric NCCM. In addition, we discuss the natural history, epidemiology, genetics, clinical presentation, outcome, and therapeutic options of NCCM in pediatric patients, including fetuses, neonates, infants, and children. Furthermore, we provide a simple classification of different forms of the disease. Finally, the differences between the pediatric population and the adult population are described.

scholarly journals MR-Proadrenomedullin as biomarker of renal damage in urinary tract infection in children

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Rafael Peñalver Penedo ◽  
Marta Rupérez Lucas ◽  
Luis Antonio Álvarez-Sala Walther ◽  
Alicia Torregrosa Benavent ◽  
María Luisa Casas Losada ◽  
...  
Keyword(s):  
Urinary Tract Infection ◽  
Urinary Tract ◽  
Tract Infection ◽  
Pediatric Patients ◽  
Pediatric Population ◽  
Adult Population ◽  
Roc Curves ◽  
Renal Scintigraphy ◽  
Reactive Protein ◽  
Sensitivity Specificity

Abstract Background Midregional-proadrenomedullin (MR-proADM) is a useful prognostic peptide in severe infectious pathologies in the adult population. However, there are no studies that analyze its utility in febrile urinary tract infection (fUTI) in children. An accurate biomarker would provide an early detection of patients with kidney damage, avoiding other invasive tests like renal scintigraphy scans. Our objective is to study the usefulness of MR-proADM as a biomarker of acute and chronic renal parenchymal damage in fUTI within the pediatric population. Methods A prospective cohort study was conducted in pediatric patients with fUTI between January 2015 and December 2018. Plasma and urine MR-proADM levels were measured at admission in addition to other laboratory parameters. After confirmation of fUTI, renal scintigraphy scans were performed during the acute and follow-up stages. A descriptive study has been carried out and sensitivity, specificity and ROC curves for MR-proADM, C-reactive protein, and procalcitonin were calculated. Results 62 pediatric patients (34 female) were enrolled. Scintigraphy showed acute pyelonephritis in 35 patients (56.5%). Of those patients, the median of plasmatic MR-proADM (P-MR-proADM) showed no differences compared to patients without pyelonephritis. 7 patients (11.3%) developed renal scars (RS). Their median P-MR-proADM levels were 1.07 nmol/L (IQR 0.66–1.59), while in patients without RS were 0.48 nmol/L (0.43–0.63) (p < 0.01). The AUC in this case was 0.92 (95% CI 0.77–0.99). We established an optimal cut-off point at 0.66 nmol/L with sensitivity 83.3% and specificity 81.8%. Conclusion MR-ProADM has demonstrated a poor ability to diagnose pyelonephritis in pediatric patients with fUTI. However, P-MR-proADM proved to be a very reliable biomarker for RS prediction.

scholarly journals Management of Cardiac Involvement Associated With Neuromuscular Diseases: A Scientific Statement From the American Heart Association

Circulation ◽  
2017 ◽  
Vol 136 (13) ◽  
Author(s):  
Brian Feingold ◽  
William T. Mahle ◽  
Scott Auerbach ◽  
Paula Clemens ◽  
Andrea A. Domenighetti ◽  
...  
Keyword(s):  
American Heart Association ◽  
Cardiac Involvement ◽  
American Heart ◽  
Neuromuscular Diseases ◽  
Heart Association ◽  
Scientific Statement

Traumatic brain injury in pediatric patients: evidence for the effectiveness of decompressive surgery

2011 ◽  
Vol 31 (5) ◽  
pp. E5 ◽  
Author(s):  
Geoffrey Appelboom ◽  
Stephen D. Zoller ◽  
Matthew A. Piazza ◽  
Caroline Szpalski ◽  
Samuel S. Bruce ◽  
...  
Keyword(s):  
Traumatic Brain Injury ◽  
Brain Injury ◽  
Pediatric Patients ◽  
Pediatric Population ◽  
Adult Population ◽  
Pediatric Trauma ◽  
Anatomical Variations ◽  
Decompressive Surgery ◽  
Pediatric Populations ◽  
The Impact

Traumatic brain injury (TBI) is the current leading cause of death in children over 1 year of age. Adequate management and care of pediatric patients is critical to ensure the best functional outcome in this population. In their controversial trial, Cooper et al. concluded that decompressive craniectomy following TBI did not improve clinical outcome of the analyzed adult population. While the study did not target pediatric populations, the results do raise important and timely clinical questions regarding the effectiveness of decompressive surgery in pediatric patients. There is still a paucity of evidence regarding the effectiveness of this therapy in a pediatric population, and there is an especially noticeable knowledge gap surrounding age-stratified interventions in pediatric trauma. The purposes of this review are to first explore the anatomical variations between pediatric and adult populations in the setting of TBI. Second, the authors assess how these differences between adult and pediatric populations could translate into differences in the impact of decompressive surgery following TBI.

Classification of cardiomyopathies

2018 ◽  
Author(s):  
Eloisa Arbustini ◽  
Valentina Favalli ◽  
Alessandro Di Toro ◽  
Alessandra Serio ◽  
Jagat Narula
Keyword(s):  
New York ◽  
American Heart Association ◽  
American Heart ◽  
Heart Association ◽  
Genetic Mutation ◽  
Phenotypic Data ◽  
Septal Thickness ◽  
New York Heart Association ◽  
European Society Of Cardiology

For over 50 years, the definition and classification of cardiomyopathies have remained anchored in the concept of ventricular dysfunction and myocardial structural remodelling due to unknown cause. The concept of idiopathic was first challenged in 2006, when the American Heart Association classification subordinated the phenotype to the aetiology. Cardiomyopathies were classified as genetic, acquired, and mixed. In 2008, the European Society of Cardiology proposed a phenotype-driven classification that separated familial (genetic) from non-familial (non-genetic) forms of cardiomyopathy. Both classifications led the way to a precise phenotypic and aetiological description of the disease and moved away from the previously held notion of idiopathic disease. In 2013, the World Heart Federation introduced a descriptive and flexible nosology—the MOGE(S) classification—describing the morphofunctional (M) phenotype of cardiomyopathy, the involvement of additional organs (O), the familial/genetic (G) origin, and the precise description of the (a)etiology including genetic mutation, if applicable (E); reporting of functional status such as American College of Cardiology/American Heart Association stage and New York Heart Association classification (S) was left optional. MOGE(S) is a bridge between the past and the future. It allows description of comprehensive phenotypic data, all genetic and non-genetic causes of cardiomyopathy, and incorporates description of familial clustering in a genetic disease. MOGE(S) is the instrument of precision diagnosis for cardiomyopathies. The addition of the early and unaffected phenotypes to the (M) descriptor outlines the clinical profile of an early affected family member; the examples include non-dilated hypokinetic cardiomyopathy in dilated cardiomyopathy and septal thickness (13–14 mm) in hypertrophic cardiomyopathy classes.

Feasibility and safety of transradial access for pediatric neurointerventions

2020 ◽  
Vol 12 (9) ◽  
pp. 893-896 ◽  
Author(s):  
Visish M Srinivasan ◽  
Caroline C Hadley ◽  
Marc Prablek ◽  
Melissa LoPresti ◽  
Stephanie H Chen ◽  
...  
Keyword(s):  
Pediatric Patients ◽  
Pediatric Population ◽  
Adult Population ◽  
Safety Data ◽  
Related Complication ◽  
Femoral Access ◽  
Transradial Access ◽  
Potential Benefits ◽  
Clinically Significant ◽  
Procedural Outcomes

BackgroundDiagnostic cerebral angiograms are increasingly being performed by transradial access (TRA) in adults, following data from the coronary literature supporting fewer access-site complications. Despite this ongoing trend in neuroangiography, there has been no discussion of its use in the pediatric population. Pediatric TRA has scarcely been described even for coronary or other applications. This is the first dedicated large study of transradial access for neuroangiography in pediatric patients.MethodsA multi-institutional series of consecutively performed pediatric transradial angiograms and interventions was collected. This included demographic, procedural, outcomes, and safety data. Data was prospectively recorded and retrospectively analyzed.ResultsThirty-seven diagnostic angiograms and 24 interventions were performed in 47 pediatric patients. Mean age, height, and weight was 14.1 years, 158.6 cm, and 57.1 kg, respectively. The radial artery measured 2.09+/-0.54 mm distally, and 2.09+/-0.44 mm proximally. Proximal and distal angiography were performed for both diagnostic and interventional application (17 distal angiograms, two distal interventions). Clinically significant vasospasm occurred in eight patients (13.1%). Re-access was successfully performed 11 times in seven patients. Conversion to femoral access occurred in five cases (8.2%). The only access-related complication was a small asymptomatic wrist hematoma after TR band removal.ConclusionsTransradial access in pediatric patients is safe and feasible. It can be performed successfully in many cases but carries some unique challenges compared with the adult population. Despite the challenge of higher rates of vasospasm and conversion to femoral access, it is worth exploring further, given the potential benefits.

Gamma Knife surgery for cerebral arteriovenous malformations in children: a 13-year experience

2008 ◽  
Vol 1 (4) ◽  
pp. 296-304 ◽  
Author(s):  
David Hung-Chi Pan ◽  
Yu-Hung Kuo ◽  
Wan-Yuo Guo ◽  
Wen-Yuh Chung ◽  
Hsiu-Mei Wu ◽  
...  
Keyword(s):  
Gamma Knife ◽  
Pediatric Patients ◽  
Pediatric Population ◽  
Statistical Significance ◽  
Adult Population ◽  
Gamma Knife Surgery ◽  
Morbidity Rate ◽  
Obliteration Rate

Object Studies on the efficacy of arteriovenous malformation (AVM) radiosurgery have largely been conducted in the adult population. Clinically, the results may not always be applicable to pediatric patients. Moreover, studies involving the pediatric population have largely comprised small- (< 3 cm3) and medium-sized (3–10 cm3) AVMs. For large (> 10 cm3) AVMs in children, sparse radiosurgical results are available. The current study was conducted to further clarify the role of radiosurgery in the treatment of pediatric AVMs. Methods A retrospective analysis was performed of data obtained in 105 pediatric patients (< 18 years of age) with cerebral AVMs treated by Gamma Knife surgery (GKS) between 1993 and 2006. For statistical comparison the authors studied data acquired in 458 adult patients with AVMs treated during the same period. The patients underwent follow-up magnetic resonance imaging at 6-month intervals. Cerebral angiography was used to confirm the obliteration of the AVM. Results In pediatric patients, the AVM obliteration rate at 48 months after a primary GKS was 65%. Repeated GKS in those in whom primary treatments failed further ablated some AVMs, for an overall obliteration rate of 81%. The efficacy of GKS correlated with the size of the AVM: 91% for small, 86% for medium, and 64% for large AVMs. The treatments were associated with an 8% morbidity rate and < 1% mortality rate. Posttreatment hemorrhage occurred in 4 (4%) of 105 patients. Obliteration rates at 48 months of small and extremely large (> 20 cm3) AVMs were similar in the pediatric and adult groups, whereas AVMs between 3 and 10 cm3 responded less efficaciously in children (p = 0.042). The AVMs with volumes ranging from 10 to 20 cm3 were also associated with a lower obliteration rate in children at 48 months, but statistical significance was not reached (p = 0.279). Conclusions Gamma Knife surgery is an effective and safe treatment alternative for pediatric AVMs. The medium (3–10-cm3) and large (10–20-cm3) AVMs tend to respond less efficaciously than those of comparable size in adults.

scholarly journals Transesophageal Echocardiography for Ross-Konno Procedure

2018 ◽  
Vol 02 (01) ◽  
pp. 036-038
Author(s):  
Shivani Aggarwal ◽  
Probal Basumatary ◽  
V. Devagourou
Keyword(s):  
Transesophageal Echocardiography ◽  
Pediatric Patients ◽  
American Heart ◽  
Heart Association ◽  
Left Ventricular ◽  
Aortic Annulus ◽  
Ventricular Outflow Obstruction ◽  
Aortic Homograft ◽  
Left Ventricular Outflow ◽  
The Right

AbstractRoss procedure involves replacing diseased aortic valve with pulmonary autograft and placing a pulmonary/aortic homograft between the right ventricle and pulmonary artery. In case of small aortic annulus in pediatric patients or in patients with multilevel left ventricular outflow obstruction, aortic annulus enlargement might be required concurrently. Konno type of aortic ventriculoplasty is most commonly done. The American Heart Association/American College of Cardiology (AHA/ACC) has classified transesophageal echocardiography (TEE) as class I indication for congenital heart disease

Stroke as Presenting Feature of COVID-19 in a Pediatric Patient

2021 ◽  
Author(s):  
Shanna Swartwood ◽  
Gary R. Nelson ◽  
Audie C. Espinoza
Keyword(s):  
Pediatric Patients ◽  
Pediatric Population ◽  
Adult Population ◽  
Acute Onset ◽  
Cerebrovascular Complications ◽  
Novel Virus ◽  
Polymerase Chain ◽  
Diagnostic Work ◽  
Novel Coronavirus ◽  
Work Up

AbstractNeurologic manifestations of severe acute respiratory syndrome coronavirus 2, the virus responsible for novel coronavirus 2019 (COVID-19) infection, have been frequently reported in the adult population but remain relatively rare in pediatric patients, specifically in regard to cerebrovascular accidents (CVAs). We present the case of a previously healthy 16-year-old adolescent boy with no preceding infectious symptoms who developed acute onset of left-sided weakness and slurred speech subsequently diagnosed with acute ischemic stroke (AIS). After performing a thorough diagnostic work-up, no clear etiology for AIS was identified. He was found to be COVID-19 positive by reverse transcription polymerase chain reaction upon admission. Accumulating evidence supports a link between COVID-19 and a systemic prothrombotic state suggesting pediatric patients who present with AIS and no other risk factors should be screened for this novel virus and potentially for extracranial sources of thrombi. As the rates of positive COVID-19 infection increase in the pediatric population, pediatricians and other pediatric subspecialists should be aware of the potential neurological and cerebrovascular complications of this novel virus to avoid delays in evaluation and intervention.

THE PEDIATRICIAN AND ATHEROSCLEROSIS

PEDIATRICS ◽  
1972 ◽  
Vol 49 (2) ◽  
pp. 165-168
Author(s):  
Shiela Mitchell ◽  
S. Gilbert Blount ◽  
Sidney Blumenthal ◽  
Mary Jane Jesse ◽  
William H. Weidman
Keyword(s):  
American Heart Association ◽  
Congenital Heart ◽  
American Heart ◽  
Heart Diseases ◽  
Heart Association ◽  
The United States ◽  
Review Of The Literature ◽  
The Past ◽  
Foreign Countries ◽  
Future Risk

The incidence of premature disability and death from complications of atherosclerosis in the adult American is so high that pediatricians must accept the responsibility of finding the threatened child and, insofar as possible, reducing the future risk. The Council of Rheumatic Fever and Congenital Heart Diseases of the American Heart Association formed a Committee which, during the past 2½ years, has met with a number of different experts in the field. These presentations have been supplemented by a selected review of the literature, and a workshop made up of experts in several related fields selected from the United States and a number of foreign countries to complement the above presentations.

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