Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029713 (immaturity)
 4,335 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Every year in the United States approximately 40,000 infants are born with congenital heart disease. Several of these infants require corrective or palliative surgery in the neonatal period. Mortality rates after cardiac surgery are highest amongst neonates, particularly those born prematurely. There are several reasons for the increased surgical mortality risk in neonates. This review outlines these risks, with particular emphasis on the relative immaturity of the organ systems in the term and preterm neonate.
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2013
PMID:Neonatal cardiac care, a perspective. 2356 14

During the last two decades, significant progress has been made in the identification of genetic defects underlying inherited arrhythmia syndromes, which has provided some clinical benefit through elucidation of gene-specific arrhythmia triggers and treatment. However, for most arrhythmia syndromes, clinical management is hindered by insufficient knowledge of the functional consequences of the mutation in question, the pro-arrhythmic mechanisms involved, and hence the most optimal treatment strategy. Moreover, disease expressivity and sensitivity to therapeutic interventions often varies between mutations and/or patients, underlining the need for more individualized strategies. The development of the induced pluripotent stem cell (iPSC) technology now provides the opportunity for generating iPSC-derived cardiomyocytes (CMs) from human material (hiPSC-CMs), enabling patient- and/or mutation-specific investigations. These hiPSC-CMs may furthermore be employed for identification and assessment of novel therapeutic strategies for arrhythmia syndromes. However, due to their relative immaturity, hiPSC-CMs also display a number of essential differences as compared to adult human CMs, and hence there are certain limitations in their use. We here review the electrophysiological characteristics of hiPSC-CMs, their use for investigating inherited arrhythmia syndromes, and their applicability for identification and assessment of (novel) anti-arrhythmic treatment strategies.
Cardiovasc Drugs Ther 2017 Jun
PMID:Human iPSC-Derived Cardiomyocytes for Investigation of Disease Mechanisms and Therapeutic Strategies in Inherited Arrhythmia Syndromes: Strengths and Limitations. 2872 24

The management of cardiopulmonary bypass for pediatric cardiac surgery is more challenging than that in adults due to the smaller size, immaturity, and complexity of the anatomy in children. Despite major improvements in cardiopulmonary bypass, there remain many subjects of debate. This review article discusses the physiology of cardiopulmonary bypass for pediatric and congenital heart surgery, including topics related to hemodilution, hypothermia, acid-base strategies, inflammatory response, and myocardial protection.
Gen Thorac Cardiovasc Surg 2018 Feb
PMID:Cardiopulmonary bypass for pediatric cardiac surgery. 2918 63

Cardiomyocytes derived from human pluripotent stem cells (hPSCs) are emerging as an invaluable alternative to primarily sourced cardiomyocytes. The potentially unlimited number of hPSC-derived cardiomyocytes (hPSC-CMs) that may be obtained in vitro facilitates high-throughput applications like cell transplantation for myocardial repair, cardiotoxicity testing during drug development, and patient-specific disease modeling. Despite promising progress in these areas, a major disadvantage that limits the use of hPSC-CMs is their immaturity. Improvements to the maturity of hPSC-CMs are necessary to capture physiologically relevant responses. Herein, we review and discuss the different maturation strategies undertaken by others to improve the morphology, contractility, electrophysiology, and metabolism of these derived cardiomyocytes.
J Cardiovasc Transl Res 2018 10
PMID:Maturation of Pluripotent Stem Cell-Derived Cardiomyocytes: a Critical Step for Drug Development and Cell Therapy. 2955 52


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