Dataset Information

Evaluation of Drug-Related Receptors in Children With Dilated Cardiomyopathy.

ABSTRACT: Background: Effective treatments for pediatric dilated cardiomyopathy (DCM) are limited. Currently, pediatric DCM therapy mainly includes supportive heart failure (HF) treatment. While the treatment for child DCM patients is generally the same as that for adult DCM patients, few randomized prospective studies on the clinical efficacy of treatments for pediatric DCM have been published. We explored the appropriate treatments for child patients. Methods: The ultrastructure of pediatric DCM and control hearts was analyzed by electron microscopy and HE staining. Left ventricular tissues from children in the DCM and control groups were subjected to quantitative RT-PCR (qRT-PCR) to study the mRNA expression of receptors related to various treatments, including drugs targeting the renin-angiotensin-aldosterone system (RAAS) system, digoxin, milrinone, and ?-receptor blockers, in child patients in the clinic. Furthermore, the differences in drug receptors in heart tissues between children and adults with DCM were analyzed. Results: Compared with the control children, the children in the DCM group showed marked abnormalities in structure and organelles. The mRNA levels of angiotensin-converting enzyme (ACE), REN, prorenin receptor (PRR), NEP, ATP1A1, and phosphodiesterase3 (PDE3A) were higher in the pediatric DCM group than the control group. Interestingly, the mRNA expression of these treatment-related receptors was much higher in children than in adults. Conclusion: ACE inhibitors, PRR or REN receptor inhibitors, PDE3 inhibitors and LCZ696 may be effective in children with DCM. However, ?-receptor blockers are not valid treatments for pediatric DCM. Moreover, high receptor expression was observed in children. These data will improve the selection of drugs for DCM patients, enhance treatment, and increase the survival rate.

SUBMITTER: Guo Q

PROVIDER: S-EPMC6779825 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature

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Evaluation of Drug-Related Receptors in Children With Dilated Cardiomyopathy.

Guo Qing Q Liu Jie J Zhu Peng P Liu Yali Y Dong Nianguo N Shi Jiawei J Peng Hua H

Frontiers in pediatrics 20191001

<b>Background:</b> Effective treatments for pediatric dilated cardiomyopathy (DCM) are limited. Currently, pediatric DCM therapy mainly includes supportive heart failure (HF) treatment. While the treatment for child DCM patients is generally the same as that for adult DCM patients, few randomized prospective studies on the clinical efficacy of treatments for pediatric DCM have been published. We explored the appropriate treatments for child patients. <b>Methods:</b> The ultrastructure of pediatr ...[more]

PMID: 31632936

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Project description:Dilated cardiomyopathy (DCM) is a heart muscle disease characterized by ventricular dilatation and impaired systolic function. Patients with DCM suffer from heart failure, arrhythmia, and are at risk of premature death. DCM has a prevalence of one case out of 2500 individuals with an incidence of 7/100,000/year (but may be under diagnosed). In many cases the disease is inherited and is termed familial DCM (FDC). FDC may account for 20-48% of DCM. FDC is principally caused by genetic mutations in FDC genes that encode for cytoskeletal and sarcomeric proteins in the cardiac myocyte. Family history analysis is an important tool for identifying families affected by FDC. Standard criteria for evaluating FDC families have been published and the use of such criteria is increasing. Clinical genetic testing has been developed for some FDC genes and will be increasingly utilized for evaluating FDC families. Through the use of family screening by pedigree analysis and/or genetic testing, it is possible to identify patients at earlier, or even presymptomatic stages of their disease. This presents an opportunity to invoke lifestyle changes and to provide pharmacological therapy earlier in the course of disease. Genetic counseling is used to identify additional asymptomatic family members who are at risk of developing symptoms, allowing for regular screening of these individuals. The management of FDC focuses on limiting the progression of heart failure and controlling arrhythmia, and is based on currently accepted treatment guidelines for DCM. It includes general measures (salt and fluid restriction, treatment of hypertension, limitation of alcohol intake, control of body weight, moderate exercise) and pharmacotherapy. Cardiac resynchronization, implantable cardioverter defibrillators and left ventricular assist devices have progressively expanding usage. Patients with severe heart failure, severe reduction of the functional capacity and depressed left ventricular ejection fraction have a low survival rate and may require heart transplant.

Dataset Information

Evaluation of Drug-Related Receptors in Children With Dilated Cardiomyopathy.

Publications

Evaluation of Drug-Related Receptors in Children With Dilated Cardiomyopathy.

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