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

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

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

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

5.75 Prevalence of Connective Tissue–Related Symptoms in Children With Neurodevelopmental Disorders: Insights Supporting the Connectivome Theory

Objectives ASD, ADHD, and Tourette’s disorder (TD) exhibit overlapping neuropathological mechanisms, such as impaired brain connectivity. A more complete view of the alteration of connectivity that characterizes neurodevelopmental disorders both at the central and peripheral levels has recently been formulated through the “Connectivome Theory,” which is based on the role of connective tissue in the different organs. The objective of this study is to investigate the prevalence of symptoms arising from connective tissue alterations in individuals diagnosed with ASD, ADHD, or TD and to compare it with a sample of healthy controls. Methods A questionnaire investigating connective tissue–related symptoms was administered from December 2019 to January 2022 to the families of 120 children diagnosed with ASD (n = 48), ADHD (n = 36), and TD (n = 36) attending the Child Neuropsychiatry Outpatient Clinics of the University Hospital of Verona. The questionnaire was also administered to the families of a control group, composed of 44 typically developing children. The symptoms assessed by the questionnaire were: 1) striae rubre/skin irregularities/flushed skin; 2) excessive sweating of hands/feet; 3) back pain/transient limb muscle aches/chronic fatigue; 4) hip dysplasia/scoliosis/hunched back; 5) flat feet; 6) constipation/diarrhea/alternating bowel; 7) heartburn/gastroesophageal reflux/hiatus hernia; 8) use of orthodontic appliances; 9) tactile/visual/auditory/olfactory/gustatory hypersensitivity; and 10) myopia/drooping eyelids/eyelid ptosis. Results Mean ages of cases and controls were 10.1 (SD: 3.6) and 9.5 (SD: 2.5) years, respectively. Most of the cases (110 subjects, 91.7%) were males; controls were distributed in 22 (50.0%) males and 22 (50.0%) females. Of the 10 symptoms assessed, 7 were more prevalent in cases than controls. Despite the small sample, the difference in prevalence reaches statistical significance with regards to flat feet (cases 47.97% vs controls 13.64%; p = .001), hypersensitivity (cases 54.47% vs controls 18.18%; p = .001) and myopia (cases 16.26% vs controls 2.27%; p = .016). Conclusions This exploratory study indicates a likely association of connective tissue–related disorders in children with neurodevelopmental disorders. Further studies will aim to confirm this hypothesis and evaluate ASD, ADHD, and TD separately

Assessing Response Rates and Sleep Disorder Prevalence: Insights from a Propranolol Treatment Study for Infantile Haemangiomas

Background: Infantile haemangiomas (IHs) sometimes require treatment with propranolol. Sleep disturbances are the most frequently reported side effects. Monitoring adverse drug events necessitates repeated hospital visits, which can be challenging during a pandemic. Objectives: To explore the effectiveness of a new electronic questionnaire in identifying sleep disturbances related to treatment with propranolol and potential confounding factors. To evaluate the response rate to the questionnaire. To report the proportion of patients on propranolol with sleep disturbances. Methods: In an observational, prospective cohort study, caregivers provided clinical information during ambulatory visits and via an electronic questionnaire after an 8-week treatment course with propranolol and at the time of treatment interruption. Adverse drug reaction reporting forms were assessed for causality. Results: The questionnaire response rate was 91%, and the completion rate was 100%. A total of 59% of patients experienced sleep disturbances during propranolol treatment, which were considered adverse reactions. Sleep disorders were frequent during sleep regression phases and in subjects who fell asleep during physical contact with caregivers or bed-sharing with parents. Conclusion: The application of this questionnaire allows for identifying adverse sleep events associated with propranolol in IHs and potential confounders. Counselling on sleep hygiene is recommended before treatment onset

A New Method to Evaluate Joint Hypermobility in Paediatric Patients with Neurodevelopmental Disorders: A Preliminary Study

Background/Objectives: Neurodevelopmental disorders (NDDs) include a wide range of conditions that develop during the formation of the central nervous system, such as autism spectrum disorder (ASD) and attention-deficit/hyperactivity disorder (ADHD). Tourette syndrome (TS) is another neurodevelopmental disorder characterised by motor and vocal tics, which often co-occurs with ASD and ADHD. This study explores the feasibility of assessing joint hypermobility in children with specific neurodevelopmental conditions by measuring both ankles' passive range of motion (pROM). Methods: This study involved children diagnosed with ASD, ADHD, and TS, aged 5 to 15 years, who were compared with a control group of healthy children. The Beighton and Brighton scores and the pROM of the left and right ankles were measured. Data were analysed using SPSS version 22.0 for Windows (IBM SPSS Statistics, Chicago, IL, USA). A total of 102 subjects participated in this study (72.52% male, with a mean age of 10.7 ± 2.2 years). The sample included 24 children with ASD, 27 with ADHD, 26 with TS, and 25 healthy controls. Results: The pROM of the right and left ankles showed a significant positive correlation with the Beighton and Brighton scores in children with NDDs (ASD, ADHD, and TS combined). A trend towards higher Beighton scores (≥6) was observed in the ADHD and TS groups, with significance found in the TS group (p = 0.013). The pROM of the right ankle was significantly higher in the ADHD (p = 0.021) and TS (p = 0.013) groups compared to the controls. Although the left ankle followed a similar trend in the TS group, the difference was not statistically significant (p = 0.066). Controlling for age, the diagnosis of ASD, ADHD, and TS does not appear to impact any of the variables examined. Conclusions: There is a trend towards a higher prevalence of individuals with elevated Beighton scores in the ADHD and TS groups, suggesting greater general flexibility or hypermobility in these patients. However, the pROM of the right ankle is significantly higher in the ADHD and TS groups, with solid evidence in the TS group. These findings were not observed in children with ASD. However, it is necessary to consider the measurements obtained in relation to the patients' age. Finally, given that the pROM of the ankles correlates with the Beighton and Brighton scores, it could be utilised for the initial screening, monitoring, and follow-up of JH in some children with NDDs. Further investigations are required

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