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 Qp/Qs ratio of 1). The immediate therapy for all infants with HLHS is an intravenous infusion of prostaglandin E1 (PGE1) 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

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

Pediatric Idiopathic Intracranial Hypertension: Clinical and Demographic Features

Idiopathic intracranial hypertension (IIH) is a clinical condition characterized by elevated intracranial pressure and absence of clinical, laboratory or radiographic evidence of central nervous system infection, vascular malformation, intracranial space occupying lesion or hydrocephalus. In the last years the raising understanding of pediatric IIH, especially concerning its demographics and epidemiology, has brought up to a redefinition of diagnostic criteria and reevaluation of pathogenesis and treatment. The authors reviewed the records of nineteen consecutive children with newly diagnosed IIH in order to compare demographic characteristics, clinical pictures and ophthalmologic aspects as optic disc evaluation and visual field evaluation, as well as treatment modalities and follow up. Beside obesity and female gender, potential alternative risk factors remains to be investigated, which need a good collaboration between neuroophthalmologists and pediatric neurologists

Postinfectious Glomerulonephritis and Epstein-Barr Virus Co-Infection

Contrary to group A b-hemolytic streptococcus as the most common cause of postinfectious glomerulonephritis (PIGN), Epstein-Barr virus (EBV) is only occasionally associated with acute renal involvement. We describe an 11-year-old boy who presented with clinical signs of infective mononucleosis and acute glomerulonephritis characterized by edema, hypertension and dark colored urine with diminished renal function. Serology tests confirmed streptococcal infection and acute EBV infection. Persistently depressed C3 complement and gross hematuria indicated renal biopsy which shows PIGN-type picture and, in addition, acute interstitial nephritis, both conclusive of streptococcal infection. We performed tissue DNA extraction by polymerase chain reaction (PCR) and demonstrated EBV-DNA from the kidney specimen supporting EBV involvement in renal tissue. This is the first reported case of PIGN with serologically-proven streptococcal and simultaneously, acute EBV co-infection. EBV-DNA extraction supported the EBV involvement in renal tissue suggesting that both etiologic agents might have contributed to renal inflammation. Adding serology evaluation for EBV in cases with typical clinical signs of infective mononucleosis and renal symptoms, EBV might be more commonly associated with PIGN than is currently appreciated

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 Qp/Qs ratio of 1). The immediate therapy for all infants with HLHS is an intravenous infusion of prostaglandin E1 (PGE1) 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

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

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

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

Preživljenje do otpusta iz bolnice novorođenčadi vrlo niske porođajne težine u dvije hrvatske perinatalne regije: retrospektivna studija vremena i uzroka smrti

We investigated mortality, causes, timing and risk factors for death until hospital discharge in very-low-birth-weight (VLBW) infants born in two Croatian perinatal care regions. This retrospective study included 252 live born VLBW infants. The mortality rate until hospital discharge was 30.5% (77/252). VLBW in-fants who died had by 4 weeks lower gestational age (GA) than surviving infants (median GA, 25 vs. 29 weeks), lower birth weight (BW) (mean BW, 756.4 vs. 1126.4 g), lower 5-minute Apgar score (median 5 vs. 8) and were more often resuscitated at birth (41.6 vs. 19.4%; p12 hours to necrotizing enterocolitis.Istraživali smo smrtnost, uzroke, vrijeme i rizične čimbenike za smrt do otpusta iz bolnice u novorođenčadi vrlo niske porođajne težine (VNPT) rođene u dvije hrvatske perinatalne regije. Ova retrospektivna studija je uključila 252 živo-rođena novorođenčeta VNPT. Smrtnost do otpusta iz bolnice bila je 30.5% (77/252). Novorođenčad VNPT koja su umrla bila su 4 tjedna manje gestacijske dobi od novorođenčadi koja je preživjela (medijan gestacijske dobi 25 prema 29 tjedana; p12 sati glavni uzrok smrti je nekrotizirajući enterokolitis

What Associates Charles Bonnet Syndrome with Age-Related Macular Degeneration?

Charles Bonnet syndrome (CBS) is a condition related to patients with visual loss due to age related macular degeneration or glaucoma that are having complex visual hallucinations. The CBS was first described by Swiss physician Charles Bonnet in 1760. Affected patients, who are otherwise mentally healthy people with significant visual loss, have vivid, complex recurrent visual hallucinations (VHs). One characteristic of these hallucinations is that they usually are »Lilliputian hallucinations« as patients experience micropsia (hallucinations in which the characters or objects are distorted and much smaller than normal). The prevalence of Charles Bonnet Syndrome has been reported to be between 10% and 40%; a recent Australian study has found the prevalence to be 17.5%. The high incidence of non-reported CBS is thought to be as a result of patient’s fear to report the symptoms as they could be labeled as mentally insane since those type of visual hallucinations could be found in variety of psychiatric and neurological disorders such as drug or alcohol abuse (delirium tremens), Alice in Wonderland syndrome (AIWS), psychosis, schizophrenia, dementia, narcolepsy, epilepsy, Parkinson disease, brain tumors, migraine, as well as, in long term sleep deprivation. VHs can also be presented as the initial sign of the Epstein-Barr virus infection in infectious mononucleosis. Patients who suffer from CBS usually possess insight into the unreality of their visual experiences, which are commonly pleasant but may sometimes cause distress. The hallucinations consist of well-defined, organized, and clear images over which the subject has little control. It is believed that they represent release phenomena due to deafferentiation of the visual association areas of the cerebral cortex, leading to a form of phantom vision. Cognitive defects, social isolation, and sensory deprivation have also been implicated in the etiology of this condition. This study was conducted on 350 patients diagnosed with Age-Related Macular Degeneration (AMD) and shows incidence of CBS in 13% of patients with AMD. Furthermore, we have found higher incidence of CBS in patients with massive loss of vision in peripheral visual field which is not age related