Brighouse James Robert, Shepherd Kathryn, Santos Rui, Shivamurthy Vinay
Paediatric Rheumatology, Evelina London Children's Hospital, London, UK
Paediatric Rheumatology, Evelina London Children's Hospital, London, UK.
Arch Dis Child Educ Pract Ed. 2022 Apr;107(2):113-115. doi: 10.1136/archdischild-2020-319887. Epub 2020 Sep 15.
Two previously well Caucasian teenage girls with no significant travel or family history were admitted with sinusitis refractory to treatment with antibiotics. Both had progressive symptoms despite broad-spectrum antibiotics and developed involvement of other systems, ultimately requiring admission to the paediatric intensive care unit (PICU). They were subsequently diagnosed with the same condition and made an excellent recovery.The first girl, 14 years old, originally presented to her general practitioner with a sore throat, nasal congestion, and fever. Despite multiple courses of antibiotics over the following month she presented again multiple times with progressive symptoms including epistaxis, widespread myalgia and arthralgia, difficulty in breathing, haemoptysis, fatigue, and weight loss. Examination on admission found minimal discharge from her right tympanic membrane, ulcerated inferior turbinates with dried blood and yellow mucus in both nasal cavities, swollen tonsils without exudate, and an isolated aphthous ulcer on the tip of her tongue. She had small bilateral cervical lymph nodes. Examination of the cardiovascular system was unremarkable but on respiratory examination there was reduced air entry on the right side. Her abdomen was generally tender but soft with no organomegaly. A week into her admission she developed an oxygen requirement and increased work of breathing requiring intubation and ventilation.The second girl, 13 years old, presented to her local hospital with a 1-day history of epistaxis, right ear pain, and pain and swelling to the right periorbital tissues. Again, despite multiple courses of antibiotics, she had persistent symptoms and subsequently developed fever, fatigue, haemoptysis, and had a syncopal episode. She was admitted to her local district general hospital and, despite initial treatment, deteriorated over the following 2 weeks with persistent fever, new oxygen requirement, deteriorating renal function, and anaemia. She was transferred to a tertiary centre for respiratory support which escalated from Optiflow, through continuous then biphasic positive airway pressure, intubation and ventilation, and ultimately VV-ECMO.The investigations for both patients prior to admission to PICU at our centre are shown in table 1.
两名此前健康的白种少女，没有显著的旅行史或家族病史，因鼻窦炎接受抗生素治疗无效而入院。尽管使用了广谱抗生素，两人的症状仍不断进展，并累及其他系统，最终需要入住儿科重症监护病房（PICU）。她们随后被诊断为相同病症，并康复良好。
第一名女孩，14岁，最初因喉咙痛、鼻塞和发热就诊于全科医生。在接下来的一个月里，尽管多次使用抗生素疗程，她仍多次因症状进展而就诊，包括鼻出血、广泛的肌痛和关节痛、呼吸困难、咯血、疲劳和体重减轻。入院检查发现右鼓膜有少量分泌物，双侧下鼻甲溃疡，鼻腔内有干血和黄色黏液，扁桃体肿大但无渗出物，舌尖有一个孤立的阿弗他溃疡。她双侧颈部有小淋巴结。心血管系统检查无异常，但呼吸检查发现右侧呼吸音减弱。她的腹部普遍压痛但柔软，无脏器肿大。入院一周后，她出现了吸氧需求，呼吸做功增加，需要插管和通气。
第二名女孩，13岁，因鼻出血、右耳疼痛以及右眶周组织疼痛和肿胀1天就诊于当地医院。同样，尽管多次使用抗生素疗程，她仍有持续症状，随后出现发热、疲劳、咯血，并发生了一次晕厥发作。她入住当地的区综合医院，尽管进行了初始治疗，但在接下来的2周内病情恶化，持续发热、出现新的吸氧需求、肾功能恶化和贫血。她被转到一家三级中心接受呼吸支持，从Optiflow逐渐升级为持续气道正压通气、双相气道正压通气、插管和通气，最终使用静脉-静脉体外膜肺氧合（VV-ECMO）。
在我们中心这两名患者入住PICU之前的检查结果见表1。
