Hypovitaminosis D and Leukocytosis to Predict Cardiovascular Abnormalities in Children with Kawasaki Disease: Insights from a Single-Center Retrospective Observational Cohort Study.

An aberrant immune response involving yet unidentified environmental and genetic factors plays a crucial role in triggering Kawasaki disease (KD). The aim of this study was to assess general and laboratory data at the onset of KD in a single-center cohort of children managed between 2003 and 2023 and retrospectively evaluate any potential relationship with the development of KD-related cardiovascular abnormalities (CVAs). We took into account a total of 65 consecutive children with KD (42 males, median age: 22 months, age range: 2-88 months) followed at the Department of Life Sciences and Public Health in our University; demographic data, clinical signs, and laboratory variables at disease onset, before IVIG infusion, including C-reactive protein, hemoglobin, white blood cell (WBC) count, neutrophil count, platelet count, aminotransferases, natremia, albumin, total bilirubin, and 25-hydroxyvitamin D were evaluated.

Congenital heart defects in CTNNB1 syndrome: Raising clinical awareness.

CTNNB1 [OMIM *116806] encodes β-catenin, an integral part of the cadherin/catenin complex, which functions as effector of Wnt signaling. CTNNB1 is highly expressed in brain as well as in other tissues, including heart. Heterozygous CTNNB1 pathogenic variations are associated with a neurodevelopmental disorder characterized by spastic diplegia and visual defects (NEDSDV) [OMIM #615075], featuring psychomotor delay, intellectual disability, behavioral disturbances, movement disorders, visual defects and subtle facial and somatic features.

The heart in RASopathies.

The cardiovascular phenotype associated with RASopathies has expanded far beyond the original descriptions of pulmonary valve stenosis by Dr Jaqueline Noonan in 1968 and hypertrophic cardiomyopathy by Hirsch et al. in 1975. Because of the common underlying RAS/MAPK pathway dysregulation, RASopathy syndromes usually present with a typical spectrum of overlapping cardiovascular anomalies, although less common cardiac defects can occur.

Brugada pattern revealed during SARS-COV-2-related multi-systemic inflammatory syndrome in a 6-year-old child.

Rhythm abnormalities are rare during COVID-19-related multisystem inflammatory syndrome in children (MIS-C). We are reporting the detection of type I Brugada pattern in a 6-year-old child with MIS-C. Following the start of treatment (systemic steroids and immunoglobulins), a gradual evolution of cardiac rhythm up to normalisation was observed, concomitantly with a progressive reduction of inflammatory markers.

Genotype-cardiac phenotype correlations in a large single-center cohort of patients affected by RASopathies: Clinical implications and literature review.

Congenital heart disease (CHD) and hypertrophic cardiomyopathy (HCM) are common features in patients affected by RASopathies. The aim of this study was to assess genotype- phenotype correlations, focusing on the cardiac features and outcomes of interventions for cardiac conditions, in a single-center cohort of 116 patients with molecularly confirmed diagnosis of RASopathy, and compare these findings with previously published data. All enrolled patients underwent a comprehensive echocardiographic examination.

Neonatal Life-Threatening Nonoliguric Hyperkalemia Under Therapeutic Hypothermia.

To illustrate our experience with two cases of neonatal life-threatening hyperkalemia during therapeutic hypothermia (TH) despite a normal acid-base status, urine output, and preserved renal function. Clinical cases are presented from Pediatric Intensive Care Unit (PICU) admission to the onset of the hyperkalemia, with related complications and after resolution. Similar cases were not retrieved from a critical review of pertinent literature.

Clinical applications of Lung Ultrasound in children in Pediatric Emergency Setting: a lesson from a child with severe heart disease.

Background And Aim: Rheumatic Heart Disease (RHD) often evolves in congestive heart failure with development of pulmonary edema after a period of asymptomatic, latent phase. In the last years, Lung Ultrasound (LUS) has gained a primary role in the diagnosis and management of pleuropulmonary disorders, also in pediatric practice and in the diagnosis and follow-up of pulmonary edema through the qualitative analysis of ultrasound B-lines.Aim of this case report is that to keep high clinicians' attention to the diagnosis that of Rheumatic Heart Disease also in high-income countries and to deepen the role and importance of lung ultrasound, in clinical practice, in diagnosis and follow-up of pediatric lung diseases, especially in emergency settings as happened in our case.

Complications associated with myocardial bridging in four children without underlying cardiac disease: a case series.

Background: Myocardial bridging is largely considered to be a benign, symptomless congenital anomaly of the coronary arteries in which the intramyocardial coronary course is partially 'tunnelled' and leads to vessel compression during ventricular systole. There are few data regarding children.

Objective: To report on myocardial bridging observed in children seeking medical help in the paediatric emergency room.

Smith-Magenis syndrome: Report of morphological and new functional cardiac findings with review of the literature.

Smith-Magenis syndrome (SMS) is a genetic disorder characterized by multiple congenital anomalies, sleep disturbance, behavioral impairment, and intellectual disability. Its genetic cause has been defined as an alteration in the Retinoic Acid-Induced 1 gene. Cardiac anomalies have been reported since the first description of this condition in patients with 17p11.

Role of Troponin Determination to Diagnose Chest Pain in the Pediatric Emergency Department.

Objectives: Chest pain is a common cause to admission to the pediatric emergency department and often leads to an extensive cardiac evaluation. The objective of this study was to evaluate the usefulness of the troponin (TN) plasma level determination in the initial phase of the differential diagnosis of chest pain in children.

Methods: This is a retrospective observational study on 107 patients, aged 0 to 19 years, admitted for chest pain to the pediatric emergency department of our institution.

Resolution of Giant Coronary Aneurisms in a Child With Refractory Kawasaki Disease Treated With Anakinra.

Kawasaki disease (KD) is an acute, febrile illness of unknown etiology that mainly affects children under 5 years of age. intravenous immunoglobulin (IVIG), the standard treatment, has reduced coronary involvement to <5%. Patients who do not improve after an initial IVIG have a higher risk of developing coronary arteries aneurysms, and its optimal treatment remains controversial.

Outcome of Neonates with Vein of Galen Malformation Presenting with Severe Heart Failure: A Case Series.

Background: Neonatal presentation of vein of Galen aneurysmal malformations (VGAMs) complicated by cardiac failure and pulmonary hypertension is frequently associated with a poor prognosis. Interventional neuroradiology with embolization can offer a chance for survival, although neurological damage can represent a limitation.

Objective: This article determines if aggressive intensive care and drug management of cardiac failure before urgent embolization can influence morbidity and mortality.

Autoimmunity and autoinflammation as the yin and yang of idiopathic recurrent acute pericarditis.

Autoimmunity and autoinflammation are generally considered as mutually exclusive mechanisms of diseases but may concur to specific syndromes. Idiopathic recurrent acute pericarditis (IRAP) is defined as the recurrence of pericardial symptoms at any point following the prior cessation of acute pericarditis, and the latency is generally 6 weeks. Manifestations of pericarditis such as pericardial friction rub, electrocardiographic changes, and pericardial effusion are less frequent in the subsequent episodes compared to the index attack, and in some cases the only clinical sign is represented by a suggestive chest pain.

Correlation analysis between echocardiographic flow pattern and N-terminal-pro-brain natriuretic peptide for early targeted treatment of patent ductus arteriosus.

Objective: Echocardiographic flow patterns of patent ductus arteriosus (PDA) are useful to predict the development of hemodynamically significant ductus in premature infants. N-terminal pro-brain natriuretic peptide (NT-proBNP) concentrations seem to be useful to detect PDA. We investigated how NT-proBNP levels change on the basis of different flow patterns during the first day of life, and whether NT-proBNP might represent a reliable decision tool in PDA management.

Myocardial performance index and biochemical markers for early detection of doxorubicin-induced cardiotoxicity in children with acute lymphoblastic leukaemia.

Background: Despite significant improvements in the prognosis of childhood acute lymphoblastic leukaemia (ALL), the risk of anthracycline-induced cardiovascular disease remains a major concern. This study was designed to investigate the role of the myocardial performance index (MPI) and serum concentrations of biomarkers (cTnT and NT-pro-BNP) in the early detection of subclinical anthracycline-induced functional alterations in children with ALL.

Methods: All children consecutively admitted to our Pediatric Oncologic Department from January 2009 to October 2010 with a diagnosis of ALL were enrolled in this study.

Isolated pericardial agenesis revealed by bradycardia and heart MRI in a healthy 5-year-old child.

We present a five-year-old boy with an unremarkable medical history who was incidentally found to have bradycardia and electrocardiographic signs of right axial deviation. Initial echocardiogram showed left displacement of the cardiac apex with slight enlargement of the right ventricle, while frontal chest radiograph showed a lucent area between the aorta and pulmonary artery. Cardiac magnetic resonance imaging finally revealed a partial left pericardial agenesis and abnormal displacement of the heart into the left hemithorax.

Nebulized iloprost and noninvasive respiratory support for impending hypoxaemic respiratory failure in formerly preterm infants: a case series.

Objective: To describe a series of ex-preterm infants admitted to pediatric intensive care unit due to impending hypoxaemic respiratory failure complicated by pulmonary hypertension (PH) who were treated electively combining noninvasive ventilation (NIV) and nebulized iloprost (nebILO).

Design: Open uncontrolled observational study.

Setting: Pediatric Intensive Care Unit, University Hospital.

Levosimendan in two neonates with ischemic heart failure and pulmonary hypertension.

We report the successful and safe use of levosimendan, a new calcium-sensitizing agent with positive inotropic and vasodilatory action, in 2 critically ill term newborns with acute heart failure and pulmonary hypertension in the absence of any underlying heart malformation and/or previous cardiosurgical procedures. During the neonatal period, levosimendan may represent an ideal drug for immature myocardium characterized by a higher calcium-dependent contractility than in adults.

Is the prophylaxis of patent ductus arteriosus useful in extremely premature infants?

This study was aimed to verify the efficacy and safety of ibuprofen prophylaxis of patent ductus arteriosus in very preterm infants, in order to select infants receiving higher benefits from this intervention. Two hundred neonates with gestational age (GA) < or = 28 weeks receiving ibuprofen within the first two hours of life were included. Ductus closure rate was 68%, and results were significantly dependent on GA (48.