Pediatric takotsubo cardiomyopathy: A review and insights from a National Multicentric Registry.

Takotsubo syndrome (TTS) in the pediatric population is an infrequent but relevant cause of morbidity and mortality, with limited studies addressing its clinical course and prognosis. We aimed to analyze the clinical features and prognosis of pediatric TTS in a nation-wide multicenter registry and considering the published literature. We included a total of 54 patients from 4 different hospitals in Spain, as well as pediatric TTS patients from the published literature. Comparisons between groups were performed in order to assess for statistically and clinically relevant prognostic differences between pediatric and adult population features. Patients with pediatric TTS are more commonly male and exhibit a higher prevalence of physical triggers. The left ventricular ejection fraction (LVEF) was significantly lower in the pediatric population (30.5 + 10.4 vs 36.9 + 16.9, p < 0.05), resulting in more than fivefold rates of cardiogenic shock on admission compared to the general adult TTS population (Killip IV 74.1% vs 10.5%, p < 0.001) with similar rates of death and recurrence between groups. TTS in the pediatric population presents a distinctive clinical profile, with higher prevalence of atypical symptoms and physical triggers, as well as higher rates of cardiogenic shock on admission and similar mortality and recurrence rates than those of the adult population. This study provides valuable insights into understanding pediatric TTS and underscores the necessity for further research in this age group.

The relevance of electrocardiogram filtering in the differential diagnosis of Brugada syndrome: An illustrative case report.

We present a clinical case of a teenager in whom a Brugada syndrome electrical pattern was found which was finally diagnosed as a Brugada phenocopy due to a wrong setup of the high-pass filter on the electrocardiogram.

Correlation between urinary and serum NT-proBNP in acute bronchiolitis: A pilot study.

BACKGROUND AND AIMS: We aimed to analyze the correlation of urinary with serum N-terminal pro-brain natriuretic peptide (NT-proBNP) concentrations and its association with severity in acute bronchiolitis. MATERIAL AND METHODS: A pilot observational study was conducted between October 1, 2021 and March 31, 2022 including acute bronchiolitis cases who attended our institution. Serum and urinary NT-proBNP concentrations were determined using the Alere i NT-proBNP assay in time-matched urine and blood samples. The Mann-Whitney U test, Spearman's correlations, and simple linear regression were utilized to analyze the association of urine NT-proBNP levels with serum NT-proBNP and with variables indicative of severe bronchiolitis. RESULTS: Seventeen infants (median age 68 [IQR: 36-91] days) with 36 time-matched samples were included. The urine NT-proBNP was positively and strongly correlated with the serum NT-proBNP concentrations (Spearman's ρ = 0.81 & R2  coefficient = 0.751; p < 0.001), and increased with higher C-reactive protein, (p = 0.004), procalcitonin (p = 0.001), and pCO2 (p = 0.029) levels. The initial urinary NT-proBNP concentrations were higher in those infants that required ventilatory support compared with those without this outcome (1.85 [IQR: 1.16-2.44] vs. 0.63 [IQR: 0.45-0.84] pg/mg); p < 0.001); and resulted positively and strongly correlated with the duration of the ventilatory support (Spearman's ρ = 0.76; p < 0.001) and the length of stay hospitalization (Spearman's ρ = 0.84; p < 0.001). CONCLUSION: The urinary NT-proBNP concentrations could be a reliable surrogate for serum NT-proBNP levels and resulted elevated in cases of acute bronchiolitis with complicated evolution, suggesting a potential as a noninvasive tool to assess severity in this setting.

Age-adjusted NT-proBNP could help in the early identification and follow-up of children at risk for severe multisystem inflammatory syndrome associated with COVID-19 (MIS-C).

BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C) has emerged as a new disease associated with COVID-19 that presents in acute critically ill children with acute cardiovascular dysfunction. AIM: To determine whether the age-adjusted N-terminal pro-brain natriuretic peptide (NT-proBNP) value (Z-log-NT-proBNP) is associated with severe MIS-C and myocardial dysfunction. METHODS: A retrospective study was conducted which included children with MIS-C managed at our institution between April 1, 2020, and February 28, 2022. We divided the population into groups depending on severity based on pediatric intensive care unit (PICU) admission. We compared Z-log-NT-proBNP values across these groups and analyzed Z-log-NT-proBNP dynamics during the one-month follow-up. RESULTS: We included 17 participants [median age 3 (2-9) years] and seven (41%) required PICU admission. All (100%) of these cases presented very high (Z-log > 4) levels of NT-proBNP at the time of admission compared to only 5 (50%) patients with non-severe MIS-C (P = 0.025). NT-proBNP was significantly correlated with high-sensitive Troponin I levels (P = 0.045), Ross modified score (P = 0.003) and left ventricle ejection fraction (P = 0.021). CONCLUSION: Raised NT-proBNP, specifically very high values (Z-log-NT-proBNP > 4) could help in the early identification of MIS-C patients with myocardial dysfunction requiring inotropic support and PICU admission.

Síndrome de encefalopatía posterior reversible como comienzo de glomerulonefritis postinfecciosa / Posterior reversible encephalopathy syndrome as a debut of postinfectious glomerulonephritis

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Vitamin D Levels and Cardiopulmonary Status in Infants with Acute Bronchiolitis.

BACKGROUND: To assess association of Vitamin D deficiency with cardiac and pulmonary status in infants with acute bronchiolitis. METHODS: Infants hospitalized with acute bronchiolitis were enrolled and classified as those with serum 25 hydroxyvitamin D (25-OHD) below or equal and above 20 ng/mL. The primary outcomes were cardiopulmonary involvement defined by elevation of NT-ProBNP, alteration of echocardiographic parameters and respiratory support requirements. The secondary outcomes were the need for PICU admission and duration of hospitalization. RESULTS: Ninety two (50 males) infants with median (IQR) age 1 (0.5 - 3) months were included with median (IQR) serum 25 - OHD level 27.4 (11.4 - 40.3) ng/mL. Forty-three (47%) patients had serum below 20 ng/mL with left ventricle dysfunction (P=0.008), right ventricle dysfunction (P=0.008) and pulmonary hypertension (P=0.007) on echocardiography than those with serum 25 - OHD > 20 ng/mL. The median (IQR) serum NT - ProBNP levels were higher in those with low 25 - OHD levels than normal 25 - OHD levels [2232.2 (461.4 - 4313.3) and 830.4 (312.7 - 2579.5)], respectively; P=0.003. Low 25-OHD levels were associated with increased risk for PICU admission (OR 3.9 (95% CI 1.5-10.1); P=0.004), higher rates of non-invasive ventilation, (P= 0.048), mechanical ventilation (P=0.005), and longer duration of hospitalization (P=0.015). CONCLUSION: Low serum vitamin D level was associated with clinical severity and impaired cardiac and pulmonary status in infants with acute bronchiolitis.

Routine laboratory test to assess the need of respiratory support in acute bronchiolitis.

BACKGROUND AND OBJECTIVES: Accurate and readily available biomarkers to predict the clinical course of bronchiolitis would enable enhanced decision-making in this setting. We explored the relationship of several biochemical parameters available at the pediatric emergency care setting with the need of advanced respiratory support (ARS): continuous positive airway pressure (CPAP), biphasic positive airway pressure (BiPAP), or invasive mechanical ventilation (MV) in bronchiolitis. METHODS: Single-center, prospective, observational, including infants aged less than 12 months diagnosed with acute bronchiolitis at the Pediatric Emergency Department. Determination of plasmatic values of several laboratory tests was performed at the time of hospital admission. Multivariate logistic analysis identified independent predictors for need of ARS. RESULTS: From October 1, 2018 to May 1, 2020, we recruited 149 infants (58% males; median age of 1 [0.5-2.5] month). Thirty-seven (25%) cases required ARS. After adjusting by age, bacterial superinfection, and comorbidities in the multivariate analysis, only higher levels of glycemia (p = 0.001), C-reactive protein (CRP) (p = 0.028), CRP/albumin ratio (p = 0.032), and NT-proBNP (p = 0.001) remained independently associated with ARS. These biomarkers reached moderate prediction accuracy with area under the curve of receiver operator curve curves ranging from 0.701 to 0.830 (p = 0.001). All they presented relatively high specificity (0.75-0.84) and negative predictive values (0.77-0.89) with low sensitivity and positive predictive values. They also correlated significantly with length of stay hospitalization (p = 0.001). CONCLUSION: Increased plasmatic levels of CRP, CRP/albumin ratio, glycemia, and NT-proBNP at hospital admission are associated with the need for ARS in infants with acute bronchiolitis.

Different Phenotypes of Schimke Immuno-Osseous Dysplasia (SIOD) in Two Sisters with the Same Mutation in the SMARCAL1 Gene.

BACKGROUND: Schimke immuno-osseous dysplasia (SIOD) is a very rare autosomal recessive genetic disease caused by mutations in the SMARCAL1 gene. It is characterized by spondyloepiphyseal dysplasia, T-cell immunodeficiency, hypercromic nevi, hypercholestero-lemia, and steroid-resistant nephrotic syndrome with progressive renal failure to end-stage kidney disease. CASE PRESENTATION: We report two cases of SIOD in sisters, diagnosed after the debut of nephrotic syndrome. Both had a personal history of short stature, acetabular hip dysplasia, and hypercholesterolemia. The first case, a 6-year-old girl, presented peripheral refractory edema, severe arterial hypertension, and progressive decrease of the glomerular filtration rate. Steroid-resistance of nephrotic syndrome was confirmed, treated with tacrolimus without response. Renal function worsened over the following 4 months, so haemodialysis was started. Her sister, a 5-year-old girl, had the steroid-resistant nephrotic syndrome and normal blood pressure and renal function under enalapril treatment. In view of the suspicion of SIOD, genetic studies were carried out, revealing the same mutation in homozygosis. CONCLUSION: SIOD has a variable expression with multi-systemic involvement with a short life expectancy. Early diagnosis is important, which can encourage the early start of treatment and anticipation of complications that may be life-threatening.

Characterization of Cardiopulmonary Interactions and Exploring Their Prognostic Value in Acute Bronchiolitis: A Prospective Cardiopulmonary Ultrasound Study.

We aimed to delineate cardiopulmonary interactions in acute bronchiolitis and to evaluate the capacity of a combined cardiopulmonary ultrasonography to predict the need for respiratory support. This was a prospective observational single-center study that includes infants <12 month of age admitted to a hospital due to acute bronchiolitis. All the included patients underwent clinical, laboratory and cardiopulmonary ultrasonographic evaluation at the same time point within 24 h of hospital admission. The existence of significant correlation between cardiac and respiratory parameters was the primary outcome. The association of different cardiopulmonary variables with the need of respiratory support higher than O2, the length of stay hospitalization, the PICU stay and the duration of respiratory support were a secondary outcome. We enrolled 112 infants (median age 1 (0.5-3) months; 62% males) hospitalized with acute bronchiolitis. Increased values of the pulmonary variables (BROSJOD score, pCO2 and LUS) showed moderate correlations with NT-proBNP and all echocardiographic parameters indicative of pulmonary hypertension and myocardial dysfunction (Tei index). Up to 36 (32%) infants required respiratory support during the hospitalization. This group presented with higher lung ultrasound score (p < 0.001) and increased values of NT-proBNP (p < 0.001), the Tei index (p < 0.001) and pulmonary artery pressures (p < 0.001). All the analyzed respiratory and cardiac variables showed moderate-to-strong correlations with the LOS of hospitalization and the time of respiratory support. Lung ultrasound and echocardiography showed a moderate-to-strong predictive accuracy for the need of respiratory support in the ROC analysis, with the AUC varying from 0.74 to 0.87. Those cases of bronchiolitis with a worse pulmonary status presented with a more impaired cardiac status. Cardiopulmonary ultrasonography could be a useful tool to easily identify high-risk populations for complicated acute bronchiolitis hospitalization.

Left Ventricular Hypertrophy in Patients with X-Linked Hypophosphataemia

X-linked hypophosphatemia (XLH) is a rare genetic disorder with X-linked dominant inheritance. Mutations in the PHEX gene increase fibroblast growth factor 23 (FGF23) concentrations, causing loss of phosphorus at the proximal tubule. Most pediatric patients debut in the first two years with short stature and bowed legs. Conventional treatment consists of oral supplements with phosphorus and calcitriol. Since 2018, burosumab has been approved as a novel therapeutic option for XLH, with promising results. The purpose of this study was to share our experience with two cases of XLH treated with burosumab. These patients presented with a broad phenotypical differences. One had the most severe radiological phenotype and developed left ventricular hypertrophy (LVH) and left ventricular dysfunction with preserved ejection fraction. Treatment with burosumab was well-tolerated and was followed by radiological stability and a striking improvement in both blood biochemistry and quality of life. The LVH was stable and left ventricular function normalized in the patient with cardiac involvement. In recent years many studies have been carried out to explain the role of FGF23 in cardiovascular damage, but the exact pathophysiological mechanisms are as yet unclear. The most intensively studied populations are patients with XLH or chronic kidney disease, as both are associated with high levels of FGF23. To date, cardiovascular involvement in XLH has been described in patients treated with conventional treatment, so it would be of interest to investigate if early use of burosumab at the time of diagnosis of XLH would prevent the occurrence of cardiovascular manifestations.

Early elevated NT-proBNP but not troponin I is associated with severe bronchiolitis in infants.

BACKGROUND: We aimed to explore and to compare the association between the NT-proBNP and high-sensitivity troponin I (hs-cTnI) at early stages of acute bronchiolitis with echocardiographic alterations, clinical severity and outcomes. METHODS: A single centre, prospective observational study including previously healthy infants aged 1-12 months with bronchiolitis admitted to a tertiary hospital from April 2019 to March 2020. All patients underwent clinical, laboratory and echocardiographic evaluation at the same time point within 12 h of hospital admission. NT-proBNP > 1121 pg/ml and hs-cTnI > 26 ng/L were considered elevated. The primary outcome measure was the association of raised cardiac biomarkers with the need for PICU admission. RESULTS: We enrolled 40 infants with median levels of NT-proBNP of 1176 (520-3030) pg/ml and hs-cTnI of 11.5 (5-21) ng/L at the time of hospital admission. Raised levels of NT-proBNP and hs-cTnI in 50% and 20% of cases, respectively. Of them, 15 (37%) required PICU admission during the hospitalization. Increased NT-proBNP was associated with PICU admission (adjusted OR 9.5 (CI95% 1.4-64); p = 0.020), prolonged hospitalization (ß = 2.7; p = 0.012) and duration of oxygen administration (ß = 2.7; p = 0.004) in the multivariate analysis. There were no differences in hs-cTnI levels regarding PICU admission (p = 0.866). Increased hs-cTnI levels were only associated with oxygen administration duration (Spearman rho = 0.38; p = 0.017), but this association disappeared in the multivariate analysis. Only NT-proBNP was associated with echocardiographic parameters of myocardial dysfunction (p < 0.001), and pulmonary hypertension (p < 0.001) CONCLUSION: Early elevated NT-proBNP but not hs-cTnI could be used as a biomarker for myocardial strain and disease severity in bronchiolitis.

Lipomatous atrial septal hypertrophy associated with adrenocorticotropin hormone administration in an infant with West syndrome.

We present the rare case of lipomatous atrial septal hypertrophy associated with adrenocorticotropin hormone therapy in an infant with West syndrome, highlighting their relatively benign nature and good prognosis in children, and the relevance of the differential diagnosis with more dangerous cardiac masses in order to avoid aggressive diagnostic and therapeutic interventions.

Glomerulonefritis aguda postinfecciosa por Streptococcus equi en paciente pediátrico / Acute post infectious glomerulonephritis by Streptococcus equi in a pediatric patient

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