47 results match your criteria: "Children's Health Institute[Affiliation]"

Background: Despite the use of behavioral interventions and psychotropic medications, many individuals with autism spectrum disorder (ASD) who engage in severe aggression remain refractory to conventional treatment. Propranolol, a beta-blocker, has accumulated much anecdotal evidence as a promising option. However, well-designed studies are rare, and the apprehension about cardiovascular side effects from large doses continues to exist.

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Calcium signalling and transport in the kidney.

Nat Rev Nephrol

August 2024

Department of Physiology, Medical College of Georgia, Augusta University, Augusta, GA, USA.

The kidney plays a pivotal role in regulating calcium levels within the body. Approximately 98% of the filtered calcium is reabsorbed in the nephron, and this process is tightly controlled to maintain calcium homeostasis, which is required to facilitate optimal bone mineralization, preserve serum calcium levels within a narrow range, and support intracellular signalling mechanisms. The maintenance of these functions is attributed to a delicate balance achieved by various calcium channels, transporters, and calcium-binding proteins in renal cells.

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Dystonia, typically characterized by slow repetitive involuntary movements, stiff abnormal postures, and hypertonia, is common among individuals with cerebral palsy (CP). Dystonia can interfere with activities and have considerable impact on motor function, pain/comfort, and ease of caregiving. Although pharmacological and neurosurgical approaches are used clinically in individuals with CP and dystonia that is causing interference, evidence to support these options is limited.

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Objective: The present study, conducted with a population of military families, examined the comparative effectiveness of three program formats of Adaptive Parenting Tools (ADAPT), a parenting program for families of school-aged children in which a National Guard or Reserve (NG/R) parent had returned from deployment to the post-9/11 conflicts. Despite well-documented need, parenting programs for NG/R families are scarce and often inaccessible. We predicted that both facilitator-delivered conditions (i.

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Molecular mechanisms of loop diuretics on renal calcium and magnesium transport.

Acta Physiol (Oxf)

May 2024

Department of Cardiovascular and Renal Research, Institute of Molecular Medicine, University of Southern Denmark, Odense, Denmark.

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Objective: To ascertain patient-reported, modifiable barriers to prenatal diagnosis of congenital heart defects (CHDs).

Methods: This was a mixed-methods study among caretakers of infants who received congenital heart surgery from 2019 to 2020 in the Chicagoland area. Quantitative variables measuring sociodemographic characteristics and prenatal care utilization, and qualitative data pertaining to patient-reported barriers to prenatal diagnosis were collected from electronic health records and semi-structured phone surveys.

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Predictors of biliary atresia outcome: Saudi National Study (2000 - 2018).

Saudi J Gastroenterol

November 2023

College of Medicine, Alfaisal University; Division of Pediatric Gastroenterology, Children's Specialized Hospital, Prince Abdullah Bin Khaled Celiac Disease Research Chair, Department of Pediatrics, Faculty of Medicine, King Saud University, Riyadh, Saudi Arabia.

Background: Outcomes in biliary atresia (BA) have been well-documented in large national cohorts from Europe, North America, and East Asia. Understanding the challenges that preclude success of the Kasai portoenterostomy (KPE) is the key to improve the overall outcomes of BA and implementing intervention strategies. Here, we analyzed the data from the Saudi national BA study (204 BA cases diagnosed between 2000 and 2018) to identify the prognostic factors of BA outcomes.

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Association Between Prenatal Diagnosis and Age at Surgery for Noncritical and Critical Congenital Heart Defects.

Circ Cardiovasc Qual Outcomes

September 2023

Division of Advanced General Pediatrics and Primary Care, Department of Pediatrics (M.M.D.), Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL.

Background: The relationship between the prenatal diagnosis of congenital heart defects (CHDs) and age at CHD surgery is poorly understood, despite the known relationships between age at surgery and long-term outcomes. The objective of this study was to determine the associations between prenatal diagnosis of CHD and age at surgery, and whether these associations differ for critical and noncritical CHDs.

Methods: This is a cohort analysis of patients aged 0 to 9 years who received their initial cardiac surgery at Ann & Robert H.

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Article Synopsis
  • This study investigates the role of autonomic dysfunction in children and adolescents with persistent post-concussive symptoms (PPCS) after experiencing a concussion.
  • Data were collected from participants in the Emergency Department and analyzed at 4 and 12-week intervals to assess symptoms and cardiac autonomic reflexes.
  • Results indicated that while both PPCS and non-PPCS groups experienced similar autonomic responses, a significant percentage of participants in both groups exhibited symptoms typical of orthostatic tachycardia.
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Article Synopsis
  • The International Parkinson and Movement Disorders Society (MDS) formed a task force to develop guidelines for transitioning pediatric patients with movement disorders to adult healthcare systems.
  • A formal consensus was established through a multi-round Delphi survey, informed by literature reviews and surveys of MDS members on transition practices, with input from 23 expert neurologists worldwide.
  • The task force produced 15 consensus recommendations focusing on team structure, planning, care goals, and research needs, while highlighting challenges in implementation due to healthcare resource distribution and the need for further research on transitional care outcomes.
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The immune response elicited by the current COVID-19 vaccinations declines with time, especially among the immunocompromised population. Furthermore, the emergence of novel SARS-CoV-2 variants, particularly the Omicron variant, has raised serious concerns about the efficacy of currently available vaccines in protecting the most vulnerable people. Several studies have reported that vaccinated people get breakthrough infections amid COVID-19 cases.

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Trimethoprim/sulfamethoxazole-induced methemoglobinemia in pediatric patient: A case report.

Clin Case Rep

March 2023

Pharmacy Services Administration King Saud Medical City, Riyadh First Cluster, MOH Riyadh Saudi Arabia.

In this report, a case of high dose of sulfamethoxazole/trimethoprim-induced methemoglobinemia and is reported in a young boy with ventilator-associated pneumonia and was treated successfully with methylene blue and cessation of the offending agent.

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Background: ASEAN (Association of Southeast Asian Nations) is composed of ten Southeast Asian countries bound by socio-cultural ties that promote regional peace and stability. South Asia, located in the southern subregion of Asia, includes nine countries sharing similarities in geographical and ethno-cultural factors. Chikungunya is one of the most significant problems in Southeast and South Asian countries.

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Metabolic patterns in brain 18F-fluorodeoxyglucose PET relate to aetiology in paediatric dystonia.

Brain

June 2023

Children's Neurosciences, Complex Motor Disorders Service (CMDS), Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust (GSTT), London SE1 7EH, UK.

There is a lack of imaging markers revealing the functional characteristics of different brain regions in paediatric dystonia. In this observational study, we assessed the utility of [18F]2-fluoro-2-deoxy-D-glucose (FDG)-PET in understanding dystonia pathophysiology by revealing specific resting awake brain glucose metabolism patterns in different childhood dystonia subgroups. PET scans from 267 children with dystonia being evaluated for possible deep brain stimulation surgery between September 2007 and February 2018 at Evelina London Children's Hospital (ELCH), UK, were examined.

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The 21 Century Cures Act requires FDA to expand its use of real-world evidence (RWE) to support approval of previously approved drugs for new disease indications and post-marketing study requirements. To address this need in neonates, the FDA and the Critical Path Institute (C-Path) established the International Neonatal Consortium (INC) to advance regulatory science and expedite neonatal drug development. FDA recently provided funding for INC to generate RWE to support regulatory decision making in neonatal drug development.

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The spectrum of functional tic-like behaviours: Data from an international registry.

Eur J Neurol

February 2023

Department of Clinical Neurosciences, Psychiatry, Pediatrics and Community Health Sciences, University of Calgary, Calgary, Alberta, Canada.

Article Synopsis
  • A significant increase in adolescents and young adults seeking help for functional tic-like behaviors (FTLBs) was observed from 2019 to 2022, prompting an international registry study to better understand this phenomenon.
  • Data was collected from 10 tertiary centers, involving 294 patients primarily aged 12-25, revealing high rates of complex movements and vocalizations, with a notable link to anxiety, depression, and autism spectrum disorders.
  • Treatment outcomes showed that most patients did not benefit from tic-suppressing medications, highlighting the need for further research on effective treatments and the impact of social media exposure on FTLBs.
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Molecular mechanisms underlying paracellular calcium and magnesium reabsorption in the proximal tubule and thick ascending limb.

Ann N Y Acad Sci

December 2022

Department of Cardiovascular and Renal Research, Institute of Molecular Medicine, University of Southern Denmark, Odense, Demark.

Calcium and magnesium are the most abundant divalent cations in the body. The plasma level is controlled by coordinated interaction between intestinal absorption, reabsorption in the kidney, and, for calcium at least, bone storage and exchange. The kidney adjusts urinary excretion of these ions in response to alterations in their systemic concentration.

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Clinical Reasoning: A Teenage Girl With Progressive Hyperkinetic Movements, Seizures, and Encephalopathy.

Neurology

January 2023

From the Paediatric Neurology Department, Evelina London Children's Hospital, Guy's & St Thomas' NHS Foundation Trust, London, UK; Metabolic Medicine Department, Evelina London Children's Hospital, London, UK; Clinical Neurophysiology Department, Evelina London Children's Hospital, London, UK; Clinical Genetics Department, Guys and St Thomas Hospital, London, UK; Neuroradiology Department, Evelina London Children's Hospital, London, UK; Women and Children's Health Institute, Faculty of Life Sciences & Medicine, King's College London, UK; Randall Centre for Cell and Molecular Biophysics, Muscle Signalling Section, Faculty of Life Sciences and Medicine (FoLSM), King's College London, UK; and Molecular Neurosciences, Developmental Neurosciences Programme, UCL Great Ormond Street Institute of Child Health, London, UK.

Article Synopsis
  • The "epilepsy-dyskinesia" spectrum is a complex and challenging area in diagnosing neurogenetic and neurometabolic conditions due to variability in symptoms and lack of specific biomarkers.
  • A detailed case study is presented of a teenage girl with unusual neurological symptoms starting from 14 months, showing progressive motor and cognitive issues, and multiple diagnostic tests returning unremarkable results.
  • The article highlights the importance of differential diagnosis and management in cases with neurological regression and emphasizes the role of next-generation sequencing for rapidly identifying underlying causes of these conditions.
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Genome characterization of a uropathogenic isolate PA_HN002 with cyclic di-GMP-dependent hyper-biofilm production.

Front Cell Infect Microbiol

August 2022

Guangdong Province Key Laboratory of Microbial Signals and Disease Control, Integrative Microbiology Research Centre, South China Agricultural University, Guangzhou, China.

can cause various types of infections and is one of the most ubiquitous antibiotic-resistant pathogens found in healthcare settings. It is capable of adapting to adverse conditions by transforming its motile lifestyle to a sessile biofilm lifestyle, which induces a steady state of chronic infection. However, mechanisms triggering the lifestyle transition of strains with clinical significance are not very clear.

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The Epidemiology and Outcome of Biliary Atresia: Saudi Arabian National Study (2000-2018).

Front Pediatr

July 2022

Department of Biostatistics, Research Services Administration, Research Center, King Fahad Medical City, Riyadh, Saudi Arabia.

Background: The epidemiology and outcomes of biliary atresia (BA) have been well-documented in national cohorts from two main ethnicities, namely, the Asian Orientals and Caucasians, with incidence ranging from 1 in 5,000 to 1 in 9,000 live births in East Asia and 1 in 15,000 to 19,000 live births in Europe and North America.

Objective: We report the first nationwide BA study outside North America, Europe, and East Asia to describe the epidemiology and outcomes of BA in Saudi Arabia.

Methods: A national database of BA cases diagnosed between 2000 and 2018 was analyzed.

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Calcium and phosphate are critical for numerous physiological processes. Consequently, the plasma concentration of these ions are tightly regulated. Calcitriol, the active form of vitamin D, is a positive modulator of mineralization as well as calcium and phosphate metabolism.

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The contribution of regulated colonic calcium absorption to the maintenance of calcium homeostasis.

J Steroid Biochem Mol Biol

June 2022

Department of Physiology, University of Alberta, Canada; Women's and Children's Health Institute, Alberta, Canada; Department of Paediatrics, University of Alberta, Canada. Electronic address:

Calcium absorption and secretion can occur along the length of the small and large intestine. To date, the focus of research into intestinal calcium absorption has been the small intestine, the site contributing the majority of intestinal calcium absorption. However, evidence that the colon contributes as much as 10% of enteral calcium transport has been available for decades.

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Background: Treatment with the aminoglycoside antibiotic gentamicin can be associated with severe adverse effects, including renal Ca wasting. The underlying mechanism is unknown but it has been proposed to involve activation of the Ca-sensing receptor (CaSR) in the thick ascending limb, which would increase expression of claudin-14 (CLDN14) and limit Ca reabsorption. However, no direct evidence for this hypothesis has been presented.

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Mental health and behaviour in children with dystonia: Anxiety, challenging behaviour and the relationship to pain and self-esteem.

Eur J Paediatr Neurol

November 2021

Complex Motor Disorders Service, Evelina London Children's Hospital, Guy's & St Thomas' NHS Foundation Trust, Westminster Bridge Road, London, SE17 7EH, UK. Electronic address:

Purpose: To ascertain whether young people with dystonia are more likely than the general population to have mental health and/or behavioural difficulties, and to explore factors that may contribute to these difficulties.

Method: Using a quasi-experimental design, 50 young people with dystonia aged 7-17 and their carers were recruited from the Evelina London Children's Hospital. Young people completed the Beck Youth Inventories and the Strengths and Difficulties Questionnaire.

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