范维克 - 格鲁巴赫综合征和淋巴切除术
Abril Adriana Arellano-Llamas1, Alvaro Hernandez-Caballero2, Efren Delgado-Mendoza3
1Instituto Mexicano del Seguro Social, UMAE Hospital de Gineco Obstetricia No. 3 La Raza, Mexico City 02990, Mexico.
Children (Basel, Switzerland)
|July 27, 2024
概括
范维克-格鲁巴赫综合征 (VWGS) 涉及早熟青春期,甲状腺功能低下症和淋巴腺质量. 在患有这些症状的儿童中,早期甲状腺功能低下症查至关重要,以防止不必要的手术.
科学领域:
- 儿科内分泌学 儿科内分泌学
- 生殖内分泌学 生殖内分泌学
- 遗传学 是一个遗传学.
背景情况:
- 范·维克-格鲁巴赫综合征 (VWGS) 的特点是周围早熟青春期,甲状腺功能低下症和淋巴腺质量.
- 延迟诊断可能导致不必要的淋巴切除术.
- 唐氏综合征在一些VWGS病例中是一个显著的并发症.
研究的目的:
- 突出显示甲状腺功能低下症查在患有外围早期青春期和生殖腺质量的儿童中的重要性.
- 介绍一个案例研究,说明患有唐氏综合征的儿童中VWGS的诊断挑战和管理.
- 为了强调Levothyroxine治疗相关症状的潜在好处.
主要方法:
- 一个10岁女孩的案例介绍,她患有唐氏综合症,身高矮,白风.
- 诊断工作包括超声波,地形检查和内分泌研究 (TSH,LH,雌激素).
- 审查关于范维克-格鲁巴赫综合征的现有文献.
主要成果:
- 患者出现了阴道出血和双侧卵巢质量,最初接受了淋巴切除术.
- 手术后的内分泌评估显示了严重的甲状腺功能低下和外围青春期,证实了VWGS.
- 利沃甲氧治疗改善了肥胖和矮身;在13岁开始接受激素替代治疗.
结论:
- 患有外围早期青春期和淋巴体质量的儿童在淋巴切除术前需要彻底评估甲状腺功能低下症.
- 早期诊断和治疗甲状腺功能低下症对于管理VWGS和预防并发症至关重要.
- 管理VWGS可能是复杂的,特别是在患有诸如唐氏综合征等并发症的患者中.
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