The endocrine system plays an intricate role in the regulation and modulation of cardiovascular function. Several hormones including thyroid, mineralocorticoid, glucocorticoid, arginine-vasopressin (AVP), and growth hormone (GH) have been investigated as adjunctive therapies in pediatric cardiac disease. Thyroid hormone supplementation appears to be safe in neonatal and pediatric post-operative cardiac patients, but the benefits have been modest and inconsistent. Glucocorticoids appear to decrease the inflammatory response associated with cardiopulmonary bypass in children, but have little effect on clinical outcomes. The role of AVP in pediatric shock remains limited due to inconsistent trial results and its potential side effect profile. Although mineralcorticoids are commonly used to treat neurocardiogenic syncope, little to no benefit has been demonstrated in controlled trials. GH normalizes altered cardiac function in children who are GH deficient, but its effectiveness in the treatment of heart failure has been variable. Overall, the use of these hormones in a variety of pediatric cardiac conditions generally appears to be safe, but their efficacy for relieving symptoms, improving cardiac function, and improving clinical outcomes remains unclear.
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http://dx.doi.org/10.2741/S31 | DOI Listing |
Pediatr Nephrol
January 2025
Department of Anesthesiology, West China Hospital, Sichuan University, No. 37 Guoxue Lane, Wuhou District, Chengdu, 610000, Sichuan, China.
Background: Cardiac surgery-associated acute kidney injury (CSA-AKI) is a notably common complication in pediatrics, with an incidence rate ranging from 15 to 64%. This rate is significantly higher than that observed in adults. Currently, there is a lack of substantial evidence regarding the association between intraoperative blood pressure variability (BPV) during cardiac surgery with cardiopulmonary bypass (CPB) and the development of AKI in pediatric patients.
View Article and Find Full Text PDFEur J Hum Genet
January 2025
Center for Medical Genetics, Ghent University Hospital, Ghent, Belgium.
The etiology of congenital heart disease (CHD) is complex, comprising both genetic and environmental factors. Despite documented familial occurrences, the genetic etiology remains largely elusive. Trio exome sequencing identified a heterozygous FLT4 splice site variant in two families with respectively tetralogy of Fallot (TOF), and variable CHD comprising both the TOF spectrum and aortic coarctation.
View Article and Find Full Text PDFCommun Med (Lond)
January 2025
Rare Disease Translational Center, The Jackson Laboratory, Bar Harbor, ME, USA.
Background: Multiple Sulfatase Deficiency (MSD) is a rare inherited lysosomal storage disorder characterized by loss of function mutations in the SUMF1 gene that manifests as a severe pediatric neurological disease. There are no available targeted therapies for MSD.
Methods: We engineered a viral vector (AAV9/SUMF1) to deliver working copies of the SUMF1 gene and tested the vector in Sumf1 knock out mice that generally display a median lifespan of 10 days.
Int J Cardiol
January 2025
Department of Cardiology, Cardiovascular Institute, Thorax Center, Erasmus MC, Rotterdam, the Netherlands; European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN GUARD-Heart), Amsterdam, the Netherlands.
Background: Little is known about the very long-term outcome in Tetralogy of Fallot (ToF) patients.
Objectives: To prospectively evaluate clinical outcome and quality-of-life after surgical repair of ToF.
Methods: Single-centre, longitudinal cohort-study evaluating every decade 144 ToF patients who underwent surgical repair <15 years of age between 1968 and 1980.
Lancet Diabetes Endocrinol
January 2025
LIB Therapeutics, Cincinnati, OH, USA. Electronic address:
Background: Lerodalcibep, a small binding anti-PCSK9 protein (adnectin), showed effective LDL cholesterol reduction in heterozygous familial hypercholesterolaemia. We aimed to assess the safety and efficacy of lerodalcibep and evolocumab in a globally diverse homozygous familial hypercholesterolaemia population.
Methods: This phase 3, randomised, open-label, crossover, non-inferiority study consisted of two 24-week treatment periods separated by an 8-week washout.
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