BACKGROUND Myocarditis is an important cause of morbidity and mortality in children, leading to long-term sequelae including chronic congestive heart failure, dilated cardiomyopathy, heart transplantation, and death. The initial diagnosis of myocarditis is usually based on clinical presentation, but this widely ranges from the severe sudden onset of a cardiogenic shock to asymptomatic patients. Early recognition is essential in order to monitor and start supportive treatment prior to the development of severe adverse events. Of note, many cases of fulminant myocarditis are usually misdiagnosed as otherwise minor conditions during the weeks before the unexpected deterioration. AIM To provide diagnostic clues to make an early recognition of pediatric myocarditis. To investigate early predictors for poor outcomes. METHODS We conducted a retrospective cross-sectional single-center study from January 2008 to November 2017 at the Pediatric Department of our institution, including children < 18-years-old diagnosed with myocarditis. Poor outcome was defined as the occurrence of any of the following facts: death, heart transplant, persistent left ventricular systolic dysfunction or dilation at hospital discharge (early poor outcome), or after 1 year of follow-up (late poor outcome). We analyzed different clinical features and diagnostic test findings in order to provide diagnostic clues for myocarditis in children. Multivariable stepwise logistic regression analysis was performed using all variables that had been selected by univariate analysis to determine independent factors that predicted a poor early or late outcome in our study population. RESULTS A total of 42 patients [69% male; median age of 8 (1.5-12) years] met study inclusion criteria. Chest pain (40%) was the most common specific cardiac symptom. Respiratory tract symptoms (cough, apnea, rhinorrhea) (38%), shortness of breath (35%), gastrointestinal tract symptoms (vomiting, abdominal pain, diarrhea) (33%), and fever (31%) were the most common non-cardiac initial complaints. Tachycardia (57%) and tachypnea (52%) were the most common signs on the initial physical exam followed by nonspecific signs of respiratory tract infection (44%) and respiratory distress (35%). Specific abnormal signs of heart failure such as heart murmur (26%), systolic hypotension (24%), gallop rhythm (20%), or hepatomegaly (20%) were less prevalent. Up to 43% of patients presented an early poor outcome, and 16% presented a late poor outcome. In multivariate analysis, an initial left ventricular ejection fraction (LVEF) < 30% remained the only significant predictor for early [odds ratio (OR) (95%CI) = 21 (2-456), P = 0.027) and late [OR (95%CI) = 8 (0.56-135), P = 0.047) poor outcome in children with myocarditis. LVEF correlated well with age ( r = 0.51, P = 0.005), days from the...

Lateral ventricular volumes of preterm infants with PHVD could be reliably determined using 3DUS. Ventricular volume could be accurately estimated using three lineal measurements. More studies are needed to address the importance of volume determination in PHVD.

Dystrophin-deficient cardiomyopathy (DDC) is currently the leading cause of death in patients with dystrophinopathies. Targeting myocardial fibrosis (MF) has become a major therapeutic goal in order to prevent the occurrence of DDC. We aimed to review and summarize the current evidence about the role of the renin–angiotensin–aldosterone system (RAAS) in the development and perpetuation of MF in DCC. We conducted a comprehensive search of peer-reviewed English literature on PubMed about this subject. We found increasing preclinical evidence from studies in animal models during the last 20 years pointing out a central role of RAAS in the development of MF in DDC. Local tissue RAAS acts directly mainly through its main fibrotic component angiotensin II (ANG2) and its transducer receptor (AT1R) and downstream TGF-b pathway. Additionally, it modulates the actions of most of the remaining pro-fibrotic factors involved in DDC. Despite limited clinical evidence, RAAS blockade constitutes the most studied, available and promising therapeutic strategy against MF and DDC. Conclusion: Based on the evidence reviewed, it would be recommendable to start RAAS blockade therapy through angiotensin converter enzyme inhibitors (ACEI) or AT1R blockers (ARBs) alone or in combination with mineralocorticoid receptor antagonists (MRa) at the youngest age after the diagnosis of dystrophinopathies, in order to delay the occurrence or slow the progression of MF, even before the detection of any cardiovascular alteration.

RESUMENLeclercia adecarboxylata y Raoultella ornithinolytica constituyen bacterias Gram-negativas emergentes. Los casos descritos son excepcionales. En los últimos años, las mejoras en las técnicas de diagnóstico microbiológico han permitido su detección y conocimiento. Se presenta el caso de un niño de 11 años con enfermedad mitocondrial, portador de catéter venoso central de larga duración, que desarrolló dos episodios de sepsis por L. adecarboxylata y R. ornithinolytica, respectivamente. En los casos de infección asociada al uso de catéter, es posible, en ocasiones, el tratamiento sin su retirada con evolución favorable. Es importante reconocer L. adecarboxylata y R. ornithinolytica como patógenos de diagnóstico cada vez más frecuentes, sobre todo, en pacientes inmunodeprimidos o con patologías crónicas asociadas. Palabras clave: sepsis, Enterobacteriaceae, infecciones relacionadas con catéteres, enfermedades mitocondriales, pediatría.ABSTRACT Leclercia adecarboxylata and Raoultella ornithinolytica are emergent Gram-negative bacteria. Infections caused by these microorganisms are exceptional. Improvement of microbiologist techniques in the last years has enabled their detection and more accurate knowledge. We present the case of an 11-year-old boy with mitochondrial disease with a longterm central catheter who suffered from two sepsis caused by L. adecarboxylata and R. ornithinolytica, respectively. In catheter-related infections, sometimes it is possible to provide antimicrobial treatment without removal of catheter with good results, as in our patient. It is important to recognize L. adecarboxylata and R. ornithinolytica like increasingly frequent pathogenic bacteria, mostly in immunocompromised or chronic patients.

Objectives: The aim of this study is to explore if manually segmented total brain volume (TBV) from 3D ultrasonography (US) is comparable to TBV estimated by magnetic resonance imaging (MRI). We then wanted to test 2D based TBV estimation obtained through three linear axes which would enable monitoring brain growth in the preterm infant during admission.Methods: We included very low birth weight preterm infants admitted to our neonatal intensive care unit (NICU) with normal neuroimaging findings. We measured biparietal diameter, anteroposterior axis, vertical axis from US and MRI and TBV from both MRI and 3D US. We calculated intra- and interobserver agreement within and between techniques using the intraclass correlation coefficient and Bland-Altman methodology. We then developed a multilevel prediction model of TBV based on linear measurements from both US and MRI, compared them and explored how they changed with increasing age. The multilevel prediction model for TBV from linear measures was tested for internal and external validity and we developed a reference table for ease of prediction of TBV.Results: We used measurements obtained from 426 US and 93 MRI scans from 118 patients. We found good intra- and interobserver agreement for all the measurements. US measurements were reliable when compared to MRI, including TBV which achieved excellent agreement with that of MRI [ICC of 0.98 (95% CI 0.96–0.99)]. TBV estimated through 2D measurements of biparietal diameter, anteroposterior axis, and vertical axis was comparable among both techniques. We estimated the population 95% confidence interval for the mean values of biparietal diameter, anteroposterior axis, vertical axis, and total brain volume by post-menstrual age. A TBV prediction table based on the three axes is proposed to enable easy implementation of TBV estimation in routine 2D US during admission in the NICU.Conclusions: US measurements of biparietal diameter, vertical axis, and anteroposterior axis are reliable. TBV segmented through 3D US is comparable to MRI estimated TBV. 2D US accurate estimation of TBV is possible through biparietal diameter, vertical, and anteroposterior axes.

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