Gabriella De Rosa

Prognostic factors and outcome of children with severe head injury: an 8-year experience

Abstract.Objectives: Our aim was to analyze prognostic factors and their association with outcome among children with severe head injury. Methods: We conducted a retrospective study among children with severe head injury admitted to our Pediatric Intensive Care Unit (PICU) from November 1992 to December 2000. The patients were immediately evaluated for the severity of head injury (Glasgow Coma Score, GCS), clinical presentation, cerebral axial tomography, early complications (hypoxia and hypotension), metabolic and hematological alterations and early post-traumatic seizures. Six months after injury we applied the Glasgow Outcome Score (GOS). Correlations with GOS were evaluated using univariate and multivariate logistic models. Results: In all, 122 children with severe head injury were identified. The patients presented the following scores: 18 (14.7.0%) children had a GOS of 1; 2 had a GOS of 2 (1.6%); 27 (22.2%) a GOS of 3 and 75 (61.5%) a GOS of 4 or 5. A low GOS was significantly and independently associated with low GCS, multiple trauma, the presence of hypoxia and hypotension, disseminated intravascular coagulation (DIC), hyperglycemia and early post-traumatic seizures. Hematological alterations (white blood cells) were also associated with a low GOS, though not significantly. Conclusion: In addition to GCS, types of trauma and brain lesion, hypoxia and hypotension, hemocoagulative disorders (DIC), hyperglycemia and early post-traumatic seizures are predictors of GOS. A knowledge of these prognostic factors and the correct management of children with severe head injury helps clinicians to improve outcome and to reduce morbidity and mortality.

Transcatheter closure of atrial septal defect in young children: Results and follow-up

OBJECTIVES This study sought to analyze the safety, efficacy, and follow-up results of percutaneous closure of secundum atrial septal defect (ASD) in young children. BACKGROUND Results of ASD transcatheter closure in adults are widely reported but there are no large published series concerning young children. METHODS Between December 1996 and February 2002, 48 of 553 patients percutaneously treated at our institution were children age <or=5 years. Indications for closure were: elective closure in 32 patients; frequent respiratory infections in 8; failure to thrive in 2; liver transplantation in 5; and a fenestrated Fontan in 1. The procedure was carried out under general anesthesia with fluoroscopy and transesophageal control. Two different devices were used: 1) the CardioSEAL/StarFLEX (CS/SF) and 2) the Amplatzer septal occluder (ASO). Basal physical examinations and echocardiograms were performed prior to the procedure and at follow-ups (1, 6, and 12 months, and yearly thereafter). RESULTS The mean age at closure was 3.6 +/- 1.3 years. A CS/SF was used in 10 subjects; an ASO was used in 38 patients. No deaths or immediate major complications occurred. The total occlusion rate was 87% at procedure, rising to 94% at discharge. The mean follow-up was 18 +/- 14 months. No midterm major or minor complications occurred. The occlusion rate rose to 100% at 12 months of follow-up. Symptomatic patients improved significantly. CONCLUSIONS In the current era and in experienced hands, ASD closure can be performed safely and successfully, even in very young children.

Role of Heart Rate Variability in the Early Diagnosis of Diabetic Autonomic Neuropathy in Children

Background: Diabetic autonomic neuropathy (DAN) is a major complication of diabetes. DAN has been shown to be closely related to glycemic control. To contribute significantly to the morbidity and mortality of the disease, and to be indicative of an increased risk of cardiovascular events. Tests asssing the function of the autonomic nervous system, such as the response of heart rate and blood pressure to maneuvers stimulating the autonomic nervous system, including deep breathing. Valsalva maneuver and standing, allowed to detect signs of DAN in adolescents; however, the sensitivity of such tests in revealing an early impairment of the autonomic nervous system proved low. Several studies found heart rate variability (HRV) to be useful in assessing the dysfunction of the autonomic nervous system in diabetic children and adolescents, but only few HRV parameters were evaluated in most of them. Objective: To study cardiac autonomic nervous system in diabetic children, and to investigate whether the duration of diabetes and the degree of metabolic control are determinants for the development of DAN in children. Patients and Methods: We analyzed HRV in 50 asymptomatic patients with insulin-dependent diabetes mellitus (IDDM) and 30 healthy children matched for age and sex. Results: Patiens with a history of diabetes > 8 years showed significant alterations of the autonomic nervous systemm (significant reduction of r-MSSD, pNN50, HF and increase in LF/HF). Conversely, only a reduction in pNN50 was found in patients with a disease duration < 8 years. Furthermore, we also observed significant HRV abnormalities in patients with an impaired metabolic control of diabetes. Compared to controls, patients with glycosylated hemoglobin blood levels (HbA1C) > 8% showed a significant reduction of r-MSSD, pHH50 and total power spectrum, whereas no HRV abnormalities were detected in patients with an HbA1C < 8%. Conclusions: HRV analysis can detect early subclinical alterations of the autonomic nervous system in asymptomatic patients with IDDM, which seem to consist mainly in a parasympathetic impairment. Autonomic dysfunction is associated both with the duration and an inadequate metabolic control of the disease.Hintergrund: Die diabetische Polyneuropathie (DAN) ist eine Hauptkomplikation des Diabetes mellitus. Die DAN zeigt eine enge Beziehung zur Kontrolle der Blutzuckerwerte und trägt signifikant zur Morbidität und Letalität der Erkrankung bei und weist auf eine erhöhte kardiovaskuläre Ereignisrate hin. Die DAN kann bei Erwachsenen mittels spezieller Teste geprüft werden, die die Herzfrequenzänderung und das Blutdruckverhalten untersuchen, wenn Provokationsverfahren zum Beispiel mit Valsalva-Manöver, tiefer Atmung und Stehversuch durchgeführt werden. Die Teste weisen aber eine geringe Sensitivität auf. Die Herzfrequenzvaviabilität (HRV) selbst ist aber ein gutes Verfahren, um eine autonome Dysregulation aufzudecken. Ziel: Prüfung des autonomen Nervensystems bei Kindern mit Diabetes mellitus in Abhängigkeit von der Dauer und Schwere der gestörten Glucosestoffwechselsituation. Patienten und Methoden: Bei 50 Patienten mit insulinpfichtigem Diabetes mellitus und 30 gesunden Kindern wurde die HRV geprüft. Ergebnisse: Patienten mit einer Diabetesdauer über 8 Jahre zeigten eine signifikante Störung des autonomen Nervensystems mit Reduktion der HRV. War die Dauer des Diabetes weniger als 8 Jahre, war nur der Parameter pNN 50 erniedrigt. Eine gestörte HRV wurde nur bei Patienten mit geströter metabolischer Situation gefunden. Bei Patienten mit einem HbA1C Wert > 8% fanden sich erniedrigte Werte für r-MSSD, pNN50 und das Gesamtpowerspektrum im Vergleich zu Kontrollpersonen. Patienten mit HbA1C-Werten unter 8% blieben demgegenüber unauffällig. Schlussfolgerung: Die HRV-Analyse bei insulinplfichtigen Kindern mit Diabetes mellitus kann genutzt werden, um im subklinischen Bereich eine Störung der autonomen Funktion des Nervensystems zu überprüfen. Im Wesentlichen liegt eine Parasymathikusstörung vor. Die autonome Dysregulation ist korreliert zur Dauer und zum metabolischen Status der Kinder.

Cardiac Adverse Effects of Early Dexamethasone Treatment in Preterm Infants: A Randomized Clinical Trial

This study evaluates the effects of early administration of dexamethasone on left ventricle dimensions and their clinical significance in preterm infants. Fifty preterm infants with birth weight ≤ 1250 g and gestational age ≤ 30 weeks were randomly assigned after 72 hours of life to the dexamethasone group (n = 25) or to the control group (n = 25). The treated infants received dexamethasone intravenously from the 4th day of life for 7 days (0.5 mg/kg/day for the first 3 days, 0.25 mg/kg/day for the next 3 days, and 0.125 mg/kg/day for the 7th day). Serial echocardiographic measurements of end systolic interventricular septum thickness, end diastolic interventricular septum thickness, end systolic left ventricle posterior wall thickness, end diastolic left ventricle posterior wall thickness, left ventricle end diastolic diameter, and left ventricle end systolic diameter were taken before starting dexamethasone, on days 3 and 7 of treatment, 7 days after the interruption of treatment, and at the 28th day of life. Five infants of each group were excluded by the final analysis because of the lack of a complete cardiac evaluation, leaving 20 treated and 20 control infants. Infants receiving dexamethasone had a significantly larger increase in mean septal and left posterior wall thickness during the treatment and 7 days after the dexamethasone weaning. The mean left ventricle diameter of treated infants was significantly lower than that of control infants from the 7th day of treatment to the 28th day of life. Four neonates (20%) in the dexamethasone group developed left ventricular myocardial hypertrophy without left ventricle outflow tract obstruction, showing signs of decreased cardiac output and ischemic changes on ECG. The daily fluid intake was increased to 200 ml/kgto ensure an adequate preload volume, and the complete resolution of left ventricle hypertrophy was obtained within the 2nd to 3rd week after dexamethasone weaning. Preterm infants receiving an early (< 96 hours of life) short course of dexamethasone develop a left ventricular myocardial hypertrophy that can be symptomatic and clinically significant. Preterm infants included in future studies with the goal to find the minimum dose and duration of dexamethasone treatment should be strictly monitored echocardiographically for this side effect.

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. Several autoimmune diseases may manifest with pericarditis which is often related to viral infections, while postviral pericarditis may in turn display a nonspecific autoimmune background. Similarly, autoinflammatory syndromes such as familial Mediterranean fever and tumor necrosis factor receptor-associated periodic syndrome are characterized by self-limiting pericardial symptoms. Corticosteroids are generally effective, thus supporting the autoimmune nature of IRAP, but dramatic results are obtained with interleukin-1 blocking agents in corticosteroid-dependent cases, pointing to a pathogenic role for the inflammasome. Based on these observations, we submit that IRAP represents a paradigmatic example of the putative coexistence of autoimmunity and autoinflammation: the main aim of this review is to critically discuss the hypothesis as well as the current understanding of this enigmatic clinical condition.

What do parents know about the malformations afflicting the hearts of their children

Traditionally, medical professionals have entrusted the parents of children with chronic illness with the task of imparting knowledge about the illness to the children. This practice assumes that parents understand the illness, and that they pass on their knowledge in a manner appropriate for the individual child. The aim of our study was to assess the knowledge that parents of children with a cardiac defect have about the malformation in the heart of their child. We sent a modified version of the Leuven questionnaire to 350 families. The questionnaire was filled in and sent back to our centre by 148 families. Parents showed a good knowledge of the name and anatomical characteristics of the cardiac disease suffered by their child. Parents with a child taking drugs were not very knowledgeable about the regime, side effects, and interaction with other drugs or food. Only one-quarter of the parents knew the definition of endocarditis, although almost two-fifths were aware that unexplained fever for more than 5 days was the most typical symptom. Less than half of the parents knew that endocarditis could recur. About two-fifths of parents knew the real possibility of their child being involved in competitive sports; but almost half of parents were unable to answer this question. The poor knowledge about particular aspects of the disease, treatment, and preventive measures revealed by our parents may have major consequences. The results of our study are relevant to general daily clinical practice.

Neurogenic stunned myocardium presenting as left ventricular hypertrabeculation in childhood: a variant of Takotsubo cardiomyopathy?

Objective: To report the first case of neurogenic stunned myocardium presenting with heart left ventricle noncompaction requiring intensive care in the perioperative period of tension tumor-induced hydrocephalus. Methods and Design: Case report and literature review. Our Institutional Review Board waived the need for consent. Patient: A 12-yr-old female with intracranial astrocytoma and hypertensive hydrocephalus presented with severe heart dysfunction and life-threatening ventricular ectopies intraoperatively. A severe heart failure developed requiring hemodynamic and ventilatory support for 10 days. Echocardiography showed a transient noncompaction aspect of the left ventricular wall, further confirmed by a cardiac magnetic resonance image. The noncompaction aspect lasted until 15 days postadmission, as was the case for the QT interval prolongation; no life-threatening ectopies were demonstrated on the subsequent Holter electrocardiogram monitoring. Conclusions: This report describes a unique presentation of myocardial stunning in association with an intracranial illness, namely, a hypertensive hydrocephalus complicating an intracranial neoplasm.

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

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).

Neonatal emergencies associated with cardiac rhabdomyomas: an 8-year experience.

During the foetal-neonatal period, rhabdomyomas represent the majority of cardiac tumours and are closely associated with tuberous sclerosis. Cardiac rhabdomyomas may be completely asymptomatic and are incidentally discovered during an echocardiogram, or may cause cardiac dysfunctions requiring medical and/or surgical intervention. During foetal life and the early neonatal period, life-threatening conditions, mostly due to arrhythmias, cardiac failure or obstruction, do occur on rare occasions. We reviewed the medical records of all cases of cardiac rhabdomyomas diagnosed prenatally or postnatally over an 8-year period. The present study reviews 7 cases of life-threatening conditions. Arrhythmic episodes were described in 5 patients, and blood flow obstruction was reported in 2 cases. Antiarrhythmic agents successfully controlled the clinical and electrophysiological conditions. Obstructive conditions were associated with poor outcomes. In conclusion, when prenatal diagnosis of rhabdomyoma is made, appropriate planning at delivery for the management of potential haemodynamic complications may prevent adverse neonatal outcomes. The clinical outcome is more influenced by obstructive rather than by dysrhythmic complications. Appropriate antiarrhythmic treatment is of primary importance. In all cases discovered through prenatal and/or neonatal life-threatening conditions, an accurate follow-up should always be performed to anticipate the development of tuberous sclerosis.

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

Abstract 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. Methods: Neonates with gestational age <32 weeks were assessed prospectively, using paired Doppler-echocardiographic evaluation and NT-proBNP values, at T0 (6–24 h of life), and daily until ductal closure. Results: At T0, NT-proBNP concentrations of 41 neonates correlated to the kind of pattern (p = 0.018) with the highest values in neonates with pulsatile or growing patterns. A value <9854 pg/ml identified neonates with spontaneous closure (sensitivity 71.8%, specificity 100%). Overall, 32 infants needed treatment. Pre-treatment NT-proBNP values increased compared to those at T0, significantly in neonates with growing pattern at T0 (p = 0.001). After treatment, NT-proBNP concentrations decreased compared to pre-treatment values (p = 0.0024), more markedly in the responders than in the non-responders (p = 0.042). Conclusions: NT-proBNP concentrations at T0 show a good agreement with different flow patterns and represent a useful tool to identify neonates at risk of developing hemodynamically significant PDA.