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[一名学龄前女童发热2个月,意识障碍1周]

[Fever for 2 months and disturbance of consciousness for 1 week in a preschool-aged girl].

作者信息

Tian Mao-Qiang, Lei Wen-Ting, Lang Chang-Hui, Li Juan, Tan Jun-Mei, Shu Xiao-Mei

机构信息

Department of Pediatric, Affiliated Hospital of Zunyi Medical University, Zunyi, Guizhou 563003, China.

出版信息

Zhongguo Dang Dai Er Ke Za Zhi. 2021 May;23(5):519-523. doi: 10.7499/j.issn.1008-8830.2101101.

Abstract

Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation syndrome (ROHHADS) is a rare multi-system disease, and delayed diagnosis and treatment may lead to catastrophic cardiopulmonary complications. As far as we know, no patient with ROHHADS has been reported in China, and this article reports a child with ROHHADS to improve the awareness of this disease among clinicians. A girl, aged 3 years, had the clinical manifestations of rapid weight gain, fever, disturbance of consciousness, and convulsion. The physical examination showed a body weight of 20 kg, somnolence, irregular breathing, and stiff neck. She had increased blood levels of prolactin and follicle-stimulating hormone and hyponatremia. The lumbar puncture showed an increased intracranial pressure. The brain MRI and magnetic resonance venography showed symmetrical lesions in the periventricular region and venous thrombosis in the right transverse sinus and the superior sagittal sinus. The sleep monitoring showed hypopnea. The girl was finally diagnosed with ROHHADS and intracranial venous thrombosis. She recovered after symptomatic treatment including decreasing intracranial pressure, anticoagulation, and respiratory support. The possibility of ROHHADS should be considered for patients with unexplained obesity, fever, and hypoventilation, with or without central nervous system symptoms. Early diagnosis and standardized follow-up can improve the prognosis of children with ROHHADS.

摘要

伴有下丘脑功能障碍、通气不足和自主神经调节异常综合征的快速进展性肥胖症(ROHHADS)是一种罕见的多系统疾病,诊断和治疗延迟可能导致灾难性的心肺并发症。据我们所知,中国尚无ROHHADS患者的报道,本文报告一名ROHHADS患儿,以提高临床医生对该病的认识。一名3岁女孩出现体重快速增加、发热、意识障碍和惊厥等临床表现。体格检查显示体重20kg,嗜睡,呼吸不规则,颈部僵硬。她的催乳素和促卵泡生成素水平升高,并有低钠血症。腰椎穿刺显示颅内压升高。脑部MRI和磁共振静脉血管造影显示脑室周围区域对称性病变,右侧横窦和上矢状窦静脉血栓形成。睡眠监测显示呼吸不足。该女孩最终被诊断为ROHHADS和颅内静脉血栓形成。经过包括降低颅内压、抗凝和呼吸支持在内的对症治疗后康复。对于不明原因肥胖、发热和通气不足的患者,无论有无中枢神经系统症状,均应考虑ROHHADS的可能性。早期诊断和规范随访可改善ROHHADS患儿的预后。

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