Henoch-Schönlein Purpura Nephritis in Childhood

An Update on Glomerulopathies - Clinical and Treatment Aspects is a systemic overview of recent advances in clinical aspects and therapeutic options in major syndromes of glomerular pathology. The book contains twenty four chapters divided conveniently into five sections. The first section deals with primary glomerulopathies, and the second section is devoted to glomerulopathies complicating infectious conditions. The third section deals with systemic autoimmune disorders and vasculitides which constitute major causes of glomerular disease and often renal failure. The fourth section includes chapters discussing the glomerular involvement in some major metabolic and systemic conditions. The final section has chapters which relate to some general aspects of glomerular diseases. This book will form an excellent reference tool for practicing and academic nephrology community

Current factor IX replacement options for hemophilia B and the challenges ahead

Introduction: Therapy for hemophilia B is aimed at replacing the congenital deficiency of coagulation factor IX (FIX). For replacement therapy, several FIX concentrates derived from donated human plasma or engineered by recombinant DNA technology are currently commercially available. The use of these products is well established and permit patients a relatively normal life. To further improve treatment efficacy, recombinant FIX products with a prolonged half-life have been developed, allowing relaxed prophylactic dosing and reducing treatment burden. Areas covered: In this review, we explore the current FIX replacement options for hemophilia B patients by analyzing the outcomes of their main clinical trials. We cover advances in the FIX molecules with extended half-life (EHL). Published literature on products for replacement of hemophilia B was retrieved using PubMed with no temporal limits. Expert opinion: The recent introduction of recombinant EHL FIX products has represented a major advance in the therapeutic management of hemophilia B patients, permitting both a reduction of treatment burden and improving patients' compliance to prophylaxis and, ultimately, quality of life

Prospective in the medical treatment of reduced renal growth and function by high grade vesicoureteral reflux in children

Renal dysplasia associated with grade IV/V of VUR has its own natural progression independent of pyelonephritis recurrences. The incidence of renal failure has been stable even in years of aggressive treatment approach. Interestingly, microalbumin excretion rate in children with high-grade of VUR (IV-V) was significantly increased than those with low-grade VUR and control

Pediatric Sleep-Disordered Breathing and Long-Term Complications: Clinical and Health Implications

Transitional medicine is defined as the branch of medicine which deals with the transition from the pediatric to adult healthcare system [...]

The growing interest in vitamin D is positively related to that of its kidney complications and is negatively related to that of bone benefit: an analysis based on Google Trends (Preprint)

Background. The benefits of vitamin D relate to muscle strength, cardiovascular health, and prevents osteoporosis. Objective. This study aimed to explore trends of global interest on vitamin D, hypercalcaemia, adverse kidney effects (stones and kidney failure) and osteoporosis. Methods. An electronic search was conducted with Google Trends, limiting searches based on the "health" criterion. Results. Worldwide interest in vitamin D3 (cholecalciferol), vitamin D2 (ergocalciferol or calciferol), kidney stones and kidney failure is progressively growing over time. On the other hand, vitamin D was found to be negatively correlated with hypercalcaemia and bone density. Another result of our analysis is the distribution of the popularity of searches across countries. In particular, the global popularity for vitamin D3 seems higher than that of vitamin D2 and also shows different geographical preferences. The growing interest in vitamin D parallels that of kidney stones and kidney failure, while decreasing popularity has been noted for hypercalcaemia and bone density. Conclusions. The research volumes help to clarify the changes in the trends of use of supplements and the development of their complications, according to the different geographical areas, socioeconomic status and online literac

Sleep Disordered Breathing in Children with Autism Spectrum Disorder: An In-Depth Review of Correlations and Complexities

Sleep-disordered breathing is a significant problem affecting the pediatric population. These conditions can affect sleep quality and children’s overall health and well-being. Difficulties in social interaction, communication, and repetitive behavioral patterns characterize autism spectrum disorder. Sleep disturbances are common in children with ASD. This literature review aims to gather and analyze available studies on the relationship between SDB and children with autism spectrum disorder. We comprehensively searched the literature using major search engines (PubMed, Scopus, and Web of Science). After removing duplicates, we extracted a total of 96 records. We selected 19 studies for inclusion after a thorough title and abstract screening process. Seven articles were ultimately incorporated into this analysis. The research findings presented herein emphasize the substantial influence of sleep-disordered breathing on pediatric individuals diagnosed with autism spectrum disorder (ASD). These findings reveal a high incidence of SDB in children with ASD, emphasizing the importance of early diagnosis and specialized treatment. Obesity in this population further complicates matters, requiring focused weight management strategies. Surgical interventions, such as adenotonsillectomy, have shown promise in improving behavioral issues in children with ASD affected by OSA, regardless of their obesity status. However, more comprehensive studies are necessary to investigate the benefits of A&T treatment, specifically in children with ASD and OSA. The complex relationship between ASD, SDB, and other factors, such as joint hypermobility and muscle hypotonia, suggests a need for multidisciplinary treatment approaches. Physiotherapy can play a critical role in addressing these intricate health issues. Early sleep assessments and tailored weight management strategies are essential for timely diagnosis and intervention in children with ASD. Policy initiatives should support these efforts to enhance the overall well-being of this population. Further research is crucial to understand the complex causes of sleep disturbances in children with ASD and to develop effective interventions considering the multifaceted nature of these conditions

Thromboembolism in childhood nephrotic syndrome: a rare but serious complication

The main clinical features of nephrotic syndrome (NS) are heavy proteinuria, hypoalbuminemia, hyperlipidemia and edema. In addition, multiple abnormalities in the coagulation pathway may be a consequence of the NS. Both arterial and venous thromboembolic complications (TEC) are relatively common and serious consequences of NS. In addition, arterial and venous thrombosis might be unexpected events during an exacerbation of NS. Embolic episodes may manifest in different regions of the body such as the brain or the lung. Hence, predisposing factors, personal and family history of TEC, thrombosis location and evolution should be always investigated in children with NS

Clinical perspective on renal elasticity quantification by acoustic radiation force impulse: Where we are and where we are going

Recent interests have focused on the exploration of the mechanical properties (elasticity, stiffness and deformity) of parenchymatous organs using tissue strain imaging techniques and elastosonography. Many factors can affect measurements and an accurate technique is necessary in order to avoid mistakes and to obtain reproducible data. The use of tissue strain imaging techniques to predict only renal fibrosis or progression of chronic kidney disease is of dubious value because other variables - blood perfusion and urinary pressure - can contribute to a given measure, even with a standardized method able to minimize intra- and inter-operator variability

Efficacy and safety of tranexamic acid in acute haemorrhage

Tranexamic acid is a synthetic haemostatic drug that inhibits fibrinolysis. It is effective in reducing bleeding and mortality, although to different extents in trauma, peripartum, and surgical settings. It is generally safe and inexpensive with few adverse reactions, although further studies are needed to assess its safety in settings of high thromboembolic risk

The Impact of Growth Hormone Therapy on Sleep-Related Health Outcomes in Children with Prader–Willi Syndrome: A Review and Clinical Analysis

This literature review of growth hormone (GH) therapy and sleep-related health outcomes in children diagnosed with Prader–Willi syndrome (PWS) assembles evidence for the consequences of sleep deprivation and poor sleep quality: difficulty concentrating and learning at school, behavioral problems, diminished quality of life, and growth impairment. Sleep-disordered breathing (SDB) is another factor that impacts a child’s well-being. We searched the electronic databases Medline PubMed Advanced Search Builder, Scopus, and Web of Science using MeSH terms and text words to retrieve articles on GH deficiency, recombinant human growth hormone (rhGH) therapy, sleep quality, SDB, and PWS in children. The censor date was April 2023. The initial search yielded 351 articles, 23 of which were analyzed for this review. The study findings suggest that while GH may have a role in regulating sleep, the relationship between GH treatment and sleep in patients with PWS is complex and influenced by GH dosage, patient age, and type and severity of respiratory disorders, among other factors. GH therapy can improve lung function, linear growth, and body composition in children with PWS; however, it can also trigger or worsen obstructive sleep apnea or hypoventilation in some. Long-term GH therapy may contribute to adenotonsillar hypertrophy and exacerbate sleep apnea in children with PWS. Finally, GH therapy can improve sleep quality in some patients but it can also cause or worsen SDB in others, leading to diminished sleep quality and overall quality of life. The current evidence suggests that the initial risk of worsening SDB may improve with long-term therapy. In conclusion, rhGH is the standard for managing patients with PWS. Nonetheless, its impact on respiratory function during sleep needs to be thoroughly evaluated. Polysomnography is advisable to assess the need for adenotonsillectomy before initiating rhGH therapy. Close monitoring of sleep disorders in patients with PWS receiving GH therapy is essential to ensure effective and safe treatment