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大头畸形和手指改变:一篇叙述性综述。

Macrocephaly and Finger Changes: A Narrative Review.

机构信息

1st Department of Pediatrics, "Iuliu Hațieganu" University of Medicine and Pharmacy Cluj-Napoca, 400370 Cluj-Napoca, Romania.

1st Pediatrics Clinic, Emergency Pediatric Clinical Hospital, 400370 Cluj-Napoca, Romania.

出版信息

Int J Mol Sci. 2024 May 20;25(10):5567. doi: 10.3390/ijms25105567.

Abstract

Macrocephaly, characterized by an abnormally large head circumference, often co-occurs with distinctive finger changes, presenting a diagnostic challenge for clinicians. This review aims to provide a current synthetic overview of the main acquired and genetic etiologies associated with macrocephaly and finger changes. The genetic cause encompasses several categories of diseases, including bone marrow expansion disorders, skeletal dysplasias, ciliopathies, inherited metabolic diseases, RASopathies, and overgrowth syndromes. Furthermore, autoimmune and autoinflammatory diseases are also explored for their potential involvement in macrocephaly and finger changes. The intricate genetic mechanisms involved in the formation of cranial bones and extremities are multifaceted. An excess in growth may stem from disruptions in the intricate interplays among the genetic, epigenetic, and hormonal factors that regulate human growth. Understanding the underlying cellular and molecular mechanisms is important for elucidating the developmental pathways and biological processes that contribute to the observed clinical phenotypes. The review provides a practical approach to delineate causes of macrocephaly and finger changes, facilitate differential diagnosis and guide for the appropriate etiological framework. Early recognition contributes to timely intervention and improved outcomes for affected individuals.

摘要

大头畸形,以头围异常增大为特征,常伴有特征性手指改变,给临床医生带来诊断挑战。本综述旨在对大头畸形伴手指改变的主要获得性和遗传性病因进行全面概述。遗传病因包括骨髓扩张障碍、骨骼发育不良、纤毛病、遗传性代谢疾病、RAS 病和过度生长综合征等几类疾病。此外,还探讨了自身免疫和自身炎症性疾病是否可能与大头畸形和手指改变有关。颅骨和四肢形成的复杂遗传机制是多方面的。生长过度可能源于调节人类生长的遗传、表观遗传和激素因素之间复杂相互作用的中断。了解潜在的细胞和分子机制对于阐明导致观察到的临床表型的发育途径和生物学过程非常重要。该综述提供了一种实用方法来明确大头畸形和手指改变的病因,有助于鉴别诊断和指导适当的病因框架。早期识别有助于为受影响的个体提供及时的干预和改善的结果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5402/11122644/a0a6ef0c9440/ijms-25-05567-g001.jpg

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