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Topic Review
Blood Transfusion Donor Sex Influences Newborn Outcomes
The risk of both short- and long-term mortality increases with each red blood cell (RBC) transfusion exposure in critically ill patients. In the extremely preterm newborn, one of the most heavily transfused patient groups, observational data has proposed that the association between RBC transfusion and adverse neonatal outcomes may be influenced by donor sex, although the data remains limited.
  • 683
  • 27 Dec 2022
Topic Review
Gut Microbiota and Chronic Kidney Disease
The human intestinal microbiota is a highly intricate structure with a crucial role in promoting health and preventing disease. It consists of diverse microbial communities that inhabit the gut and contribute to essential functions such as food digestion, nutrient synthesis, and immune system development. The composition and function of the gut microbiota are influenced by a variety of factors, including diet, host genetics, and environmental features. In pediatric patients, the gut microbiota is particularly dynamic and vulnerable to disruption from endogenous and exogenous factors.
  • 679
  • 23 Aug 2023
Topic Review
The Treatment of Enthesitis-Related Arthritis
Enthesitis-related arthritis (ERA) represents 5–30% of all cases of juvenile idiopathic arthritis (JIA) and belongs to the spectrum of the disorders included in the group of juvenile spondyloarthritis. In the last decade, there have been considerable advances in the classification, diagnosis, monitoring, and treatment of ERA. New provisional criteria for ERA have been recently proposed by the Paediatric Rheumatology INternational Trials Organisation, as part of a wider revision of the International League of Associations for Rheumatology criteria for JIA. The therapeutic recommendations for ERA are comparable to those applied to other non-systemic JIA categories, unless axial disease and/or enthesitis are present. In such cases, the early use of a TNF-alpha inhibitor is recommended. Novel treatment agents are promising, including IL-17/IL-23 or JAK/STAT pathways blockers.
  • 679
  • 24 Oct 2023
Topic Review
Butterflies and Ribbons in Multiple Gestation
In neonatology, multiple pregnancies are common. Unfortunately, it is not rare for one baby to die. Communication with parents in these circumstances has been demonstrated to be sub-optimal. Formal training programs for clinicians in NICUs, using either the Butterfly or Ribbon approaches, should be routinely offered. 
  • 678
  • 04 Sep 2023
Topic Review
Clinical Manifestations of Kawasaki Disease
Kawasaki disease is a systemic idiopathic febrile vasculitis that often affects children under 5 years. In Japan back in 1961, KD was initially reported and subsequently recognised based on the profiling of 50 patients with a similar phenotype by Dr Tomisaku Kawasaki in 1967. Kawasaki disease was then identified as the most common cause of acquired heart disease in paediatric populations as it would subsequently result in coronary artery aneurysms if left untreated.
  • 673
  • 10 Jun 2022
Topic Review
Genomic Aberrations in Neurodevelopmental Disorders
Genomic studies are increasingly revealing that neurodevelopmental disorders are caused by underlying genomic alterations. Chromosomal microarray testing has been used to reliably detect minute changes in genomic copy numbers. The genes located in the aberrated regions identified in patients with neurodevelopmental disorders may be associated with the phenotypic features. In such cases, haploinsufficiency is considered to be the mechanism, when the deletion of a gene is related to neurodevelopmental delay. The loss-of-function mutation in such genes may be evaluated using next-generation sequencing. On the other hand, the patients with increased copy numbers of the genes may exhibit different clinical symptoms compared to those with loss-of-function mutation in the genes. In such cases, the additional copies of the genes are considered to have a dominant negative effect, inducing cell stress. In other cases, not the copy number changes, but mutations of the genes are responsible for causing the clinical symptoms. 
  • 671
  • 28 Mar 2022
Topic Review
Human Breast Milk for Very Preterm Neonates
Prematurity is the leading cause of death in children younger than 5 years old and a main reason for morbidity in the pediatric population. Annually, almost 15 million neonates are born prematurely (before 37 week of gestation), and the prevalence is increasing every year. According to World Health Organization (WHO), among 184 countries, the percentage of preterm birth ranges between 5% and 18%. In Greece, 4.7% of total births were premature between the years 1980 and 2008, with the percentage of prematurity reaching 9.6% in 2008. Very preterm neonates (<32 weeks of gestation) and extremely preterm neonates (<28 weeks of gestation) account for about 10% and 5%, respectively, of all premature neonates, with their morbidity and mortality being inversely related with gestation age.
  • 671
  • 26 Sep 2022
Topic Review
Lipoprotein(a) as a Marker of Cardiovascular Health Risk
Lipoprotein(a) [Lp(a)] is made up of apoprotein(a) [apo(a)] and LDL-like particle. The proportion of Lp(a) in each individual is genetically determined and is only minimally modifiable by environment or diet. Lp(a) has important pro-atherosclerotic and pro-inflammatory effects. For these reasons, high Lp(a) values are an important independent risk factor for cardiovascular disease and calcific aortic valve stenosis. Numerous studies have been performed in adults about the pathophysiology and epidemiology of Lp(a), much less information is available regarding Lp(a) in children and adolescents. Gaining information on these points is particularly important for deciding whether Lp(a) assay may be useful for defining the cardiovascular risk in children, in order to plan a prevention program early.
  • 666
  • 03 Jul 2023
Topic Review
Prevention of Obesity in Prader–Willi Syndrome
Prader–Willi syndrome (PWS) is a complex genetic disorder which involves the endocrine and neurologic systems, metabolism, and behavior. It is to summarize current knowledge on dietary management and treatment of PWS and, in particular, to prevent excessive weight gain. Growth hormone (GH) therapy is the recommended standard treatment for PWS children, because it improves body composition (by changing the proportion of body fat and lean body mass specifically by increasing muscle mass and energy expenditure), linear growth, and in infants, it promotes psychomotor and IQ development.
  • 661
  • 27 May 2022
Topic Review
Free Radical Disease in Newborn
Oxygen toxicity is mainly due to the production of oxygen radicals, molecules normally produced in humans and involved in a myriad of physiological reactions. In the neonatal period, an imbalance between oxidants and antioxidant defenses, the so-called oxidative stress, might occur, causing severe pathological consequences.
  • 659
  • 27 Mar 2023
Topic Review
Sleep–Wake Rhythm Influence Neurodevelopmental Disorders in Children
Development of the sleep–wake rhythm has a significant effect on the physical and mental development of children. The sleep–wake rhythm is controlled by aminergic neurons in the brainstem’s ascending reticular activating system, which is associated with synaptogenesis and the promotion of brain development. Before evaluating a sleep disorder in a child, it is of paramount importance to evaluate, as a prerequisite, whether ‘the parent or guardian has a stable sleep rhythm’ or whether ‘the parent or guardian pays attention to the adequate environment in which the child sleeps well at night’. The sleep rhythm of the child changes with that of his or her guardian.
  • 655
  • 13 Jun 2023
Topic Review
Primary Stabbing Headache in Children and Adolescents
Primary Stabbing Headache (PSH) is characterized by brief, focal, and paroxysmal pain (“stab”), occurring sporadically or in clusters. Data on pediatric cases are poor. The prevalence of PSH and probable PSH varies from 2.5 to 10% among children with primary headaches and it is higher among children aged less than 6 years old. The mean age of onset is between 7 and 11 years of age. Attack duration greatly varies, ranging from a few seconds to several minutes. The intensity of pain is usually from moderate to severe. Associated symptoms are infrequent but may be observed (mainly photophobia, vertigo, nausea, and vomiting). Neuroradiological findings are usually unremarkable; Electroencephalographic (EEG) may show sporadic epileptiform abnormalities (up to 30% of cases). Preventive therapy is anecdotal, including treatment with indomethacin, trazodone, valproate, and amitriptyline.
  • 655
  • 26 Feb 2024
Topic Review
Treatment of X-Linked Hypophosphatemia in Children
The conventional treatment for X-linked hypophosphatemia (XLH), consisting of phosphorus supplementation and a biologically active form of vitamin D (alfacalcidol or calcitriol), is used to treat rickets and leg deformities and promote growth. However, patients’ adult height often remains less than −2 SD. Moreover, adverse events, such as renal calcification and hyperparathyroidism, may occur. The main pathology in XLH is caused by excessive production of fibroblast growth factor 23 (FGF23). Treatment with burosumab, a blocking neutralizing antibody against FGF23, is better than conventional therapy for severe XLH and has no serious, short-term side effects. Thus, treatment with burosumab may be an option for severe XLH. 
  • 652
  • 27 Oct 2022
Topic Review
Diabetes Mellitus and COVID-19 in Children
Coronavirus disease 2019 (COVID-19) is a complex infectious disease caused by the SARS-CoV-2 virus, and it currently represents a worldwide public health emergency. The pediatric population is less prone to develop severe COVID-19 infection, but children presenting underlying medical conditions, such as diabetes mellitus, are thought to be at increased risk of developing more severe forms of COVID-19.
  • 647
  • 25 Jul 2023
Topic Review
SARS-CoV-2 Vertical Infection of Newborns
Severe acute respiratory syndrome virus 2 (SARS-CoV-2), the virus that causes 2019 coronavirus disease (COVID-19), has been isolated from various tissues and body fluids, including the placenta, amniotic fluid, and umbilical cord of newborns. Much scientific effort has been directed toward studying SARS-CoV-2, focusing on the different features of the virus, such as its structure and mechanisms of action. In addition, a great deal of emphasis has been placed on creating reliable diagnostic techniques and different drugs or vaccinations for treating COVID-19 disease.
  • 639
  • 06 Feb 2023
Topic Review
Diet and Nutritional Interventions in Early Life
The infant gut microbiome plays a key role in the healthy development of the human organism and appears to be influenced by dietary practices through multiple pathways. First, maternal diet during pregnancy and infant nutrition significantly influence the infant gut microbiota. Moreover, breastfeeding fosters the proliferation of beneficial bacteria, while formula feeding increases microbial diversity. The timing of introducing solid foods also influences gut microbiota composition. In preterm infants the gut microbiota development is influenced by multiple factors, including the time since birth and the intake of breast milk, and interventions such as probiotics and prebiotics supplementation show promising results in reducing morbidity and mortality in this population.
  • 628
  • 27 Feb 2024
Topic Review
Wilms Tumor (Nephroblastoma)
Wilms tumor (or nephroblastoma) is a malignant and solid neoplasm that derives from the primitive renal bud. It represents the most frequent primary tumor of the urogenital tract in childhood, and treatment consists of surgery and chemo-radiotherapy. 
  • 626
  • 19 Dec 2023
Topic Review
Triggers of Pediatric Migraine
There are three triggers or risk factors for migraines in children and adolescents: stress, sleep poverty, and alimentation (including diet and obesity). Clinicians should advise patients to avoid certain triggers, such as stress and sleep disorders, and make a few conservative dietary changes.
  • 624
  • 26 Oct 2021
Topic Review
Pancreatic Enzyme Replacement Therapy in the Pediatric Population
Pancreatic enzyme replacement therapy, the actual treatment for exocrine pancreatic insufficiency, is essential in children with cystic fibrosis to prevent malabsorption and malnutrition and needs to be urgently initiated. This therapy presents many considerations for physicians, patients, and their families, including types and timing of administration, dose monitoring, and therapy failures. Based on clinical trials, pancreatic enzyme replacement therapy is considered effective and well-tolerated in children with cystic fibrosis.
  • 624
  • 20 Feb 2023
Topic Review
Ketogenic Diet and Polycystic Ovary Syndrome
The ketogenic diet (KD) has attracted significant interest for the treatment of insulin resistance (IR) and for the control of carbohydrate metabolism, which has proven to be beneficial for several dysmetabolic conditions, including polycystic ovary syndrome (PCOS). The goal of the KD is to induce a fasting-like metabolism with production of chetonic bodies. Ketosis is a good regulator of calorie intake and mimics the starvation effect in the body, leading to body weight control and consequent metabolism. Additionally, during ketogenesis, insulin receptor sensitivity is also promoted. 
  • 623
  • 28 Aug 2023
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