Sandro Dessardo

Tako-Tsubo Syndrome in a 12-Year-Old Girl: Exhausted Heart, Not Broken Heart

We report the case of a 12-year-old girl with Tako-tsubo cardiomyopathy. She was successfully treated in our Pediatric Intensive Care Unit for acute left ventricular failure, which occurred after a brief submersion in sea water. At 2-month follow-up she showed an almost complete recovery of cardiac function. We consider Tako-tsubo syndrome, which is rarely reported in the pediatric population, to be the most likely diagnosis.

Chronic lung disease of prematurity and early childhood wheezing: Is foetal inflammatory response syndrome to blame?

BACKGROUND Long-lasting respiratory symptoms have a huge impact on the quality of life in prematurely born children. AIMS We aimed to investigate the perinatal and maternal risk factors involved in the development of chronic respiratory morbidity in preterm infants, with an emphasis on the importance of Foetal Inflammatory Response Syndrome (FIRS). STUDY DESIGN Prospective cohort study. SUBJECTS Demographic, antenatal, delivery and outcomes data were collected from 262 infants with less than 32 completed weeks of gestational age, over a 10-year period. OUTCOME MEASURES Presence of chronic lung disease of prematurity and early childhood wheezing. RESULTS In multivariate logistic regression analysis the presence of FIRS appears to be the most important risk factor for both, chronic lung disease of prematurity (OR 31.05, 95% CI 10.7-87.75, p<0.001) and early childhood wheezing (OR 5.63, 95% CI 2.42-13.05, p=0.01). In the alternative regression model for early childhood wheezing, with chronic lung disease included as a variable, the statistical significance of FIRS completely vanished (OR 1.15, 95% CI 0.39-3.34, p=0.79), whilst chronic lung disease became the most important risk factor (OR 23.45, 95% CI 8.5-63.25, p<0.001). CONCLUSIONS Prenatal and early neonatal events are of utmost importance in the development of chronic respiratory symptoms in children. The influence of FIRS on the development of chronic respiratory symptoms goes far beyond its impact on gestational age and may be related to direct inflammation-mediated lung tissue damage. CLD appears to be an intermittent step on the way from FIRS to ECW.

Down syndrome and Crohn's disease: an extremely rare association.

trisomy 21, but normally concerns developmental abnormalities of the gastrointestinal tract, Hirschsprung disease or intestinal pathologies with verosimilar genetic basis, such as gluten enteropathy.1 Clustering of cases of Crohn’s disease according to family, race and ethnicity, as well as increased incidence of some human leukocyte antigens (HLA) loci in these patients supports the role of genetic factors in the disease.2 Further indirect evidence for a genetic basis for Crohn’s disease is the reported association with Ullrich– Turner syndrome, Hermansky–Pudlak syndrome, mucoviscidosis and other disorders with known genetic etiologies.3–5 Since we found only one previous report of the presence of Crohn’s disease in a patient with trisomy 21,6 we would like to report on this extremely rare association (trisomy 21, anal atresia and Crohn’s disease) found in our patient.

Cardiopulmonary bypass in a child with hereditary angioedema

The article describes the perioperative and postoperative management of a child with hereditary angioedema needing cardiac surgery using cardiopulmonary bypass.

Lethal respiratory failure due to a surfactant protein C gene mutation

A case of a full-term male infant with recurrent episodes of „obstructive bronchitis” and „bilateral pneumonias”, refractory to conventional asthma therapy, is reported. When referred to the paediatric clinic at the age of 15 months, his clinical presentation was of acute respiratory distress syndrome with severe hypoxemia, which was easily corrected by the inhalation of oxygen. A series of further investigations, including lung biopsy, suggested the diagnosis of childhood interstitial lung disease (ILD). Due to progressive respiratory failure, the boy died after 3 months of mechanical ventilator support, at the age of 19 months. Subsequently performed genetic studies revealed that he was heterozygous for the I73T mutation of the surfactant protein C precursor protein. Thus, suspicion of childhood ILD due to genetic mutations that influence surfactant metabolism should be raised in children who present with unexplained respiratory distress or chronic respiratory symptoms. Less invasive testing, such as surfactant protein genetics, may be diagnostic in such cases.

Laryngeal cyst and tracheomalacia in neonate: coincidence or complication?

The authors are presenting a case of a female infant with a large congenital laryngeal cyst. After operation, tracheomalacia with inspiratory stridor has been noticed.

Paths of causal influence from prenatal inflammation and preterm gestation to childhood asthma symptoms

Abstract Introduction: Long-lasting respiratory symptoms have a huge impact on the quality of life in prematurely born children. The aim was to investigate paths of assumed causality leading from foetal inflammatory response syndrome (FIRS) to asthma symptoms in preterms. Methods: Demographic, antenatal, delivery and outcome data were collected from 262 infants with less than 32 completed weeks of gestational age over a 10-year period in a prospective cohort study. The presence of symptoms of asthma beyond the age of 5 years was the primary outcome measure. The causal effect of FIRS on childhood asthma was tested with three different logistic regression models and two structural equation models (SEM). Results: FIRS (OR = 4.7) and subsequent chronic lung disease of prematurity (OR = 7.7) and early childhood wheezing (OR = 9.5) are the most important risk factors for development of asthma symptoms in children born with less than 32 weeks of gestational age. The path analysis showed that FIRS has a large direct (0.59), medium indirect (0.11) and large overall (0.70) effect on CLD; large negative direct effect on ECW (−0.34) and a large positive indirect effect (0.74), mediated by CLD. On the occurrence of asthma symptoms, FIRS has a medium negative direct effect (−0.18) and a medium positive indirect effect (0.26), mediated by CLD and ECW. Conclusion: Prenatal inflammation plays an important role in the development of chronic respiratory disturbances in preterm infants. This influence is mainly related to structural and developmental lung abnormalities initiated in utero as consequences of FIRS, resulting in CLD of prematurity, and overcoming the protective mechanisms of chorioamnionitis.

CRIB II score versus gestational age and birth weight in preterm infant mortality prediction: who will win the bet?

Introduction. In neonatology, various illness severity scores have been developed to predict mortality and morbidity risk in neonates. The aim of our study was to validate the ability of the ‘Clinical Risk Index for Babies’ (CRIB) II score to predict mortality in neonates born before 32 weeks’ gestation in a level 3 neonatal intensive care unit (NICU), setting.Materials and Methods. Prospective birth cohort study including all live-born neonates of 32 weeks’ gestation or less. . CRIB II score was calculated and the predicted mortality was compared with the observed mortality. Discrimination (the ability of the score to correctly predict survival or death) was assessed by calculating the receiver operating characteristic curve (ROC curve) and its associated area under the curve (AUC).Results. The ROC curve analysis in our study showed that the AUC was 0.9008 suggesting that mortality prediction was 90% accurate for all infants. Sensitivity and specificity were 77% and 88% respectively. In our study population, the CRIB II score appears to be more accurate than gestational age and birth weight in predicting mortality.Conclusions. The CRIB II scoring system is a useful tool for predicting mortality and morbidity in NICUs, and also a useful tool for evaluating the variations in mortality and other outcomes seen between different NICUs.

Mediastinal tube placement in a premature infant with cardiorespiratory derangement due to ventilator associated pneumomediastinum

While mediastinal free air in the ventilated newborn is usually benign, tension pneumomediastinum can lead to further cardiorespiratory compromise due to the compression of mediastinal structures, including the heart and large blood vessels. The authors present a case of life-threatening pneumomediastinum in a ventilated preterm leading to abrupt onset of cardiorespiratory failure. An 8 French (Fr) drainage catheter was placed in the anterior mediastinum using the 2nd right intercostal space as an insertion site, with prompt hemodynamic improvement. A brief description of the drainage technique and a literature review is presented. SANDRO DESSARDO • KRISTINA LAH TOMULI • NEVEN FRLETA • NADA SINDI I DESSARDO

Preoperative management of hypoplastic left heart syndrome

Pediatricians are frequently involved in the care of cyanotic newborns in the labor and delivery room, as well as in the well baby nursery. Causes of hypoxia and cyanosis in the term newborn can be found within all physiological systems. Congenital heart structural diseases account for the largest diagnostic category. There have been significant advances during the past years in the diagnosis and treatment of neonates with critical congenital heart disease, especially in the field of pre- and post-operative intensive care. The term hypoplastic left heart syndrome (HLHS) describes a spectrum of cardiac structural abnormalities characterized by marked hypoplasia of the left ventricle and ascending aorta. Prenatal diagnosis, initial resuscitation and optimal preoperative management are key elements that allow the best opportunity for low mortality and normal neurodevelopment in affected newborns. Preoperatively, the goal is to achieve adequate systemic oxygen delivery. Patency of the ductus arteriosus (DA) is critical for survival until surgery. Blood flow to the pulmonary and systemic circulations should be nearly balanced (goal Q p /Q s ratio of 1). The immediate therapy for all infants with HLHS is an intravenous infusion of prostaglandin E 1 (PGE 1 ) in order to manipulate the DA and maintain ductal patency. Oxygen saturations of 75% to 85% by pulse oximetry suggest an adequate balance between systemic and pulmonary blood flow. Judicious use of inotropic support is initiated if evidence of low cardiac output is detected. Diuretics may be necessary to help alleviate the increased volume load on the right ventricle. The goal of respiratory management is to increase pulmonary vascular resistance and decrease systemic vascular resistance. Infants with HLHS who are born with a severely restricted or no inter-atrial communication, a rare occurrence, have profound hypoxemia. The severe restriction of blood flow across the atrial septum results in a life-threatening situation and these patients, which present with severe cyanosis and hemodynamic instability, require urgent postnatal cardiac catheterization to relieve the septal obstruction and improve oxygenation. Special attention should be paid to the prevention of brain injury and poor neurodevelopmental outcome. Care for infants with HLHS is complex, and often multiple specialists are involved. Despite an increase in the number of newborns with complex congenital heart disease and a growing percentage of patients with single-ventricle physiology, it is possible to care for this particular group of patients and achieve acceptable mortality risks, even in centres with no pediatric cardiac surgery facilities, if good preoperative management protocols are followed.