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DOI 10.4103/jpgm.jpgm_468_17

Медицинская библиотека · Journal of postgraduate medicine · 2018

Moyamoya syndrome in hemoglobin E-beta thalassemia: A rare presentation and association.

Doctor P N, Choudhari A, Verma M, Merchant R H

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Аннотация

During early embryonic development, the heart undergoes a remarkable and complex transformation, acquiring its iconic four-chamber structure whilst concomitantly contracting to maintain its essential function. The emergence of cardiac form and function involves intricate interplays between molecular, cellular, and biomechanical events, unfolding with precision in both space and time. The dynamic morphological remodelling of the developing heart renders it particularly vulnerable to congenital defects, with heart malformations being the most common type of congenital birth defect (∼35% of all congenital birth defects). This mini-review aims to give an overview of the morphogenetic processes which govern early heart formation as well as the dynamics and mechanisms of early cardiac function. Moreover, we aim to highlight some of the interplay between these two processes and discuss how recent findings and emerging techniques/models offer promising avenues for future exploration. In summary, the developing heart is an exciting model to gain fundamental insight into the dynamic relationship between form and function, which will augment our understanding of cardiac congenital defects and provide a blueprint for potential therapeutic strategies to treat disease.

Для цитирования: Doctor P N, Choudhari A, Verma M, Merchant R H. Moyamoya syndrome in hemoglobin E-beta thalassemia: A rare presentation and association.. Journal of postgraduate medicine. 2018. DOI: 10.4103/jpgm.jpgm_468_17.

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