一种罕见的甲状腺功能减退儿童内分泌急症:以黏液性水肿昏迷为表现的临床评估。

IF 1.7 Q3 PEDIATRICS
Kürşat Çetin, Zeynep Donbaloğlu, Yasemin Funda Bahar, Aslıhan Karakurum, Özlem Tolu Kendir, Hale Tuhan, Mesut Parlak
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引用次数: 0

摘要

目的:黏液水肿昏迷(MC)是一种严重而罕见的甲状腺功能减退症,可导致多器官功能衰竭和意识改变。报告本病例系列的目的是评估诊断为MC的儿科患者的临床表现、诊断结果和结局。材料和方法:本文报告了作者所在医院儿科内分泌科在2020年1月1日至2024年10月31日期间诊断为MC的8例患者的病例系列。病例的临床和实验室资料来源于医院档案系统,采用MC诊断评分系统对病例进行评分。结果:平均年龄为10.36±3.56岁,男女比例为1:3。其中4例患者入院时诊断为甲状腺功能减退,其余4例患者有甲状腺功能减退病史,但因治疗不遵医嘱而出现甲状腺功能减退。大多数患者表现为水肿、体重迅速增加、嗜睡和其他甲状腺功能减退症状。桥本甲状腺炎6例,甲状腺发育不全1例,甲状腺发育不全1例。实验室检查显示促甲状腺激素(TSH)严重升高,游离甲状腺素(fT4)和游离三碘甲状腺原氨酸(fT3)水平低。6例患者CK和肌红蛋白水平升高,提示继发性横纹肌溶解。左旋甲状腺素(LT4)治疗后,肌肉力量、甲状腺功能和其他临床参数均有显著改善。所有患者均无需重症监护,生存率均为100%。结论:早期诊断和适当的甲状腺激素替代治疗对逆转代谢异常和预防危及生命的并发症至关重要。本研究强调了及时干预的重要性,并强调了甲状腺功能减退儿童严格坚持甲状腺激素治疗的必要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

A Rare Endocrinological Emergency in Children with Hypothyroidism: Clinical Evaluation of Cases Presenting with Myxedema Coma.

A Rare Endocrinological Emergency in Children with Hypothyroidism: Clinical Evaluation of Cases Presenting with Myxedema Coma.

A Rare Endocrinological Emergency in Children with Hypothyroidism: Clinical Evaluation of Cases Presenting with Myxedema Coma.

Objective: Myxedema coma (MC) is a severe and rare clinical form of hypothyroidism that causes multiple organ failure and altered consciousness. The aim of reporting this case series is to evaluate the clinical presentation, diagnostic findings, and outcomes in pediatric patients diagnosed with MC. Materials and Methods: This article presents a case series of 8 patients diagnosed with MC between January 1, 2020, and October 31, 2024, at pediatric endocrinology department of the authors' hospital. The clinical and laboratory data of the cases were obtained from the hospital records system, and the cases were scored using the diagnostic scoring system for MC. Results: The mean age was 10.36 ± 3.56 years, with a male-to-female ratio of 1:3. Four of the patients were diagnosed with hypothyroidism upon admission with MC, while the remaining 4 had a history of hypothyroidism but presented with MC due to non-compliance with treatment. The majority of patients presented with edema, rapid weight gain, lethargy, and other symptoms of hypothyroidism. Six patients had Hashimoto's thyroiditis, 1 had thyroid hypoplasia, and 1 had thyroid aplasia. Laboratory tests revealed severely elevated thyroid-stimulating hormone (TSH) and low free thyroxine (fT4) and free triiodothyronine (fT3) levels. Six patients had elevated CK and myoglobin levels, indicating secondary rhabdomyolysis. Following levothyroxine (LT4) therapy, significant improvements were observed in muscle strength, thyroid function, and other clinical parameters. None of the patients required intensive care, and all recovered with 100% survival rate. Conclusion: Early diagnosis and appropriate thyroid hormone replacement therapy are crucial for reversing the metabolic abnormalities and preventing life-threatening complications. This study highlights the importance of timely intervention and emphasizes the need for strict adherence to thyroid hormone therapy in children with hypothyroidism.

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