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肾上腺素能危象作为神经母细胞瘤的首发形式。

Adrenergic crisis as a debut form of a neuroblastoma.

机构信息

Servicio de Urgencias, Hospital Infantil Universitario Niño Jesús, Madrid, España.

Servicio de Cuidados Intensivos, Hospital Infantil Universitario Niño Jesús, Madrid, España.

出版信息

Rev Chil Pediatr. 2020 Oct;91(5):767-772. doi: 10.32641/rchped.vi91i5.1828.

DOI:10.32641/rchped.vi91i5.1828
PMID:33399643
Abstract

INTRODUCTION

The most common clinical presentation of neuroblastoma is an abdominal mass, but it can present with uncommon symptoms, such as adrenergic storm due to catecholamine release.

OBJECTIVE

To describe an unusual presentation of neuroblastoma and the wide differential diagnosis that exists in an infant with adrenergic symptoms.

CLINICAL CASE

A 7-week old female infant was evaluated due to a 3-week history of sweating and irritability associated with a 24-hour fever and respiratory distress. At admission, she presented poor general condition, irritability, sweating, facial redness, tachypnea and skin paleness, extreme sinus tachycardia, and high blood pressure (HBP), interpreted as adrenergic symptoms. The study was completed with abdominal ultrasound and magnetic reso nance imaging that showed a large retroperitoneal mass compatible with neuroblastoma. Plasma and urinary catecholamines tests showed high levels of dopamine, adrenaline, and noradrenaline, probably of tumor origin. We started antihypertensive treatment with alpha-blocker drugs, showing a good blood pressure control. The tumor was surgically resected without incidents and adequate subsequent recovery. The patient presented a favorable evolution after three years of follow-up. Con clusions: In an infant with adrenergic symptoms such as irritability, redness, sweating associated with HBP, it should be ruled out pathology heart or metabolic (hypoglycemia) pathology, intoxications, and/or adrenal pathology. Within this last one, neuroblastoma is the first diagnostic possibility, since it is one of the main tumors in childhood and, although this presentation is not usual, it can produce these symptoms.

摘要

介绍

神经母细胞瘤最常见的临床表现是腹部肿块,但它也可能表现出不常见的症状,如由于儿茶酚胺释放引起的肾上腺素能危象。

目的

描述神经母细胞瘤的一种不常见表现以及婴儿出现肾上腺素能症状时存在广泛的鉴别诊断。

临床病例

一名 7 周大的女性婴儿因 3 周前出现出汗和烦躁症状,伴有 24 小时发热和呼吸窘迫而接受评估。入院时,她表现出一般状况不佳、烦躁不安、出汗、面部发红、呼吸急促和皮肤苍白、极度窦性心动过速和高血压(HBP),被解释为肾上腺素能症状。完成了腹部超声和磁共振成像检查,显示出与神经母细胞瘤相符的大型腹膜后肿块。血浆和尿液儿茶酚胺测试显示多巴胺、肾上腺素和去甲肾上腺素水平升高,可能来自肿瘤。我们开始使用α受体阻滞剂药物进行降压治疗,血压得到了很好的控制。肿瘤在无并发症的情况下进行了手术切除,随后恢复良好。患者在 3 年的随访后表现出良好的预后。结论:对于出现烦躁、发红、出汗和 HBP 等肾上腺素能症状的婴儿,应排除心脏或代谢(低血糖)疾病、中毒和/或肾上腺疾病。在后者中,神经母细胞瘤是首先要考虑的诊断可能性,因为它是儿童期的主要肿瘤之一,尽管这种表现并不常见,但它可能会引起这些症状。

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