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先天性肾上腺皮质增生症患儿的生长评估与营养状况——来自越南一家三级儿科中心的横断面研究

Growth Assessment and Nutritional Status in Children with Congenital Adrenal Hyperplasia-A Cross-Sectional Study from a Vietnamese Tertiary Pediatric Center.

作者信息

Nguyen Thi Thuy Hong, Le Khanh Minh, Tran Thi Anh Thuong, Nguyen Khanh Ngoc, Can Thi Bich Ngoc, Bui Phuong Thao, Tran Dat Tien, Vu Chi Dung

机构信息

Department of Paediatrics, Hanoi Medical University, Hanoi 11521, Vietnam.

Center for Endocrinology, Metabolism, Genetic/Genomics and Molecular Therapy, Vietnam National Children's Hospital, Hanoi 11512, Vietnam.

出版信息

Diagnostics (Basel). 2025 Jun 16;15(12):1534. doi: 10.3390/diagnostics15121534.

DOI:10.3390/diagnostics15121534
PMID:40564853
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12192552/
Abstract

Children with congenital adrenal hyperplasia (CAH) face significant risks of impaired growth and metabolic disturbances despite standard glucocorticoid therapy. This cross-sectional study aimed to evaluate growth outcomes, nutritional status, and associated factors among children with CAH treated in a Vietnamese tertiary pediatric center. We assessed 201 children aged 1.1-16.5 years in a tertiary pediatric center in Vietnam for anthropometric parameters, biochemical markers (calcium, phosphate, 25-hydroxyvitamin D), and clinical features. Growth status was evaluated using WHO standards, and bone age was assessed radiographically. Statistical analyses explored associations between growth outcomes and clinical, biochemical, and treatment-related factors. : Stunting was present in 16.4% of children, while 53.3% were overweight or obese. Bone age advancement occurred in 51.7% of cases. Vitamin D insufficiency or deficiency was detected in 85.6% of patients, and hypocalcemia was present in 85.1%. Overweight/obesity, vitamin D deficiency, and bone age advancement were associated with older age, prolonged corticosteroid therapy, higher androgen levels, and clinical features of treatment imbalance (e.g., Cushingoid appearance, hyperpigmentation). Female sex was significantly associated with higher rates of stunting. : Growth impairment, nutritional deficiencies, and skeletal maturation disturbances are prevalent among children with CAH in Vietnam. Early identification of risk factors and the implementation of tailored management strategies that address both endocrine and nutritional health are crucial for optimizing long-term outcomes.

摘要

尽管接受了标准的糖皮质激素治疗,但先天性肾上腺皮质增生症(CAH)患儿仍面临生长发育受损和代谢紊乱的重大风险。这项横断面研究旨在评估越南一家三级儿科中心接受治疗的CAH患儿的生长结局、营养状况及相关因素。我们在越南一家三级儿科中心对201名年龄在1.1至16.5岁的儿童进行了人体测量参数、生化指标(钙、磷、25-羟维生素D)及临床特征评估。使用世界卫生组织标准评估生长状况,并通过X光片评估骨龄。统计分析探讨了生长结局与临床、生化及治疗相关因素之间的关联。16.4%的儿童存在发育迟缓,而53.3%的儿童超重或肥胖。51.7%的病例出现骨龄提前。85.6%的患者检测出维生素D不足或缺乏,85.1%的患者存在低钙血症。超重/肥胖、维生素D缺乏和骨龄提前与年龄较大、长期使用皮质类固醇治疗、雄激素水平较高以及治疗失衡的临床特征(如库欣样面容、色素沉着)有关。女性发育迟缓率明显较高。越南CAH患儿中普遍存在生长发育障碍、营养缺乏和骨骼成熟紊乱。早期识别危险因素并实施针对内分泌和营养健康的定制管理策略对于优化长期结局至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a47f/12192552/70d681714ffe/diagnostics-15-01534-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a47f/12192552/70441f8c37cf/diagnostics-15-01534-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a47f/12192552/70d681714ffe/diagnostics-15-01534-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a47f/12192552/70441f8c37cf/diagnostics-15-01534-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a47f/12192552/70d681714ffe/diagnostics-15-01534-g002.jpg

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本文引用的文献

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Congenital Adrenal Hyperplasia.先天性肾上腺皮质增生症
Pediatr Ann. 2025 Feb;54(2):e74-e77. doi: 10.3928/19382359-20241203-02. Epub 2025 Feb 1.
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Barriers to the Management of Classic Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency.21-羟化酶缺乏所致经典型先天性肾上腺皮质增生症管理中的障碍
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Current Status of Newborn Bloodspot Screening Worldwide 2024: A Comprehensive Review of Recent Activities (2020-2023).《2024年全球新生儿血斑筛查现状:2020 - 2023年近期活动综合回顾》
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Growth and Treatment in Congenital Adrenal Hyperplasia: An Observational Study from Diagnosis to Final Height.先天性肾上腺皮质增生症的生长和治疗:从诊断到最终身高的观察性研究。
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Characteristics of Growth in Children With Classic Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency During Adrenarche and Beyond.青春期和青春期后患有 21-羟化酶缺陷的经典先天性肾上腺皮质增生症儿童生长特点。
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