Background/Aims Childhood sarcoidosis is a rare multisystem inflammatory granulomatous disease. The prevalence of sarcoidosis in children is unknown due to its rarity. A review of sarcoidosis in Denmark found an incidence in children < 15 years of 0.22-0.27 per 100 000/year. Diagnosis can be challenging given the varied presentations due to multisystem involvement. There is no one laboratory test that is diagnostic for sarcoidosis. Angiotensin converting enzyme (ACE) has been shown to be elevated in 50% of late-onset childhood sarcoidosis. Reference levels are laboratory and age dependent, and healthy children can have levels that are 40-50% higher than in adults. Our aim was to gain a better understanding of the use of ACE in clinical practice and to review its role in diagnosis and management of sarcoidosis. Methods We collated the list of all patients under 18 years who had ACE levels done at our hospital between 1/12/2021 and 30/11/2022 via clinical pathology. Electronic patient records were reviewed for all patients to gather information on demographics, and clinical symptoms. Results We had a total of 98 ACE blood tests done for 58 patients. Clinical notes and results were available for 34 of these patients with a total of 48 ACE results. Age range of patients was 3-17 years with a mean age of 12 years. 19 (56%) patients were female, 15 (44%) were male. 50% were of white ethnicity, 12% of black ethnicity, 3% Indian ethnicity, 3% Pakistani ethnicity, 3% Chinese ethnicity and 29% ethnicity not recorded. 45 were requested by in hospital specialties, 2 from other hospitals and 1 from a GP. Of those requested in hospital 73% had been requested by ophthalmology, rheumatology or dermatology, with the remainder requested by a range of other paediatric specialties. Results ranged from 11-195 U/L, with a median of 76 U/L. Hospital reference range is 13-64 U/L and 18 patients had results >64 U/L. Reference ranges for paediatric patients in literature is 29-112 U/L. 6 patients had a result>112U/L. One patient had a diagnosis of sarcoidosis. Following the initial results a further 4 years of results were analysed from 02/01/2018-30/11/2022. 10 further patients had ACE results >112U/L. Of these, none had sarcoidosis. Conclusion These results support that the diagnosis of sarcoidosis is rare. ACE results need to be interpreted being mindful that normal childhood ACE levels may be higher than the reference ranges given by hospitals, which may be based on adult ranges. There is need for more research in this area to ascertain diagnostic ACE levels and role of ACE in disease monitoring. I plan to lead a trainee multicentre retrospective project looking at the diagnosis of paediatric sarcoidosis, and within this review the role of ACE levels. Disclosure M.C. Mackay: None. S. Deepak: None. K. Warrier: None. S. Rangaraj: None.

中文翻译：

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E050 血管紧张素转换酶用于诊断儿童结节病

背景/目标儿童结节病是一种罕见的多系统炎性肉芽肿性疾病。由于其罕见，儿童结节病的患病率尚不清楚。对丹麦结节病的回顾发现，儿童中的发病率<10%。 15 年每 10 万人每年 0.22-0.27。由于涉及多系统，诊断可能具有挑战性。没有一种实验室检查可以诊断结节病。 50% 的迟发性儿童结节病中血管紧张素转换酶 (ACE) 升高。参考水平取决于实验室和年龄，健康儿童的水平可能比成人高 40-50%。我们的目的是更好地了解 ACE 在临床实践中的应用，并回顾其在结节病诊断和治疗中的作用。方法我们通过临床病理学整理了2021年12月12日至2022年11月30日期间在我院进行ACE水平测定的所有18岁以下患者的名单。对所有患者的电子病历进行了审查，以收集有关人口统计和临床症状的信息。结果 我们总共为 58 名患者进行了 98 次 ACE 血液检测。其中 34 名患者的临床记录和结果共有 48 项 ACE 结果。患者年龄范围为3-17岁，平均年龄12岁。 19 名患者（56%）为女性，15 名患者（44%）为男性。 50%为白人，12%为黑人，3%为印度族，3%为巴基斯坦族，3%为华人，29%为未记录族裔。 45 份是医院内科室要求的，2 份来自其他医院，1 份来自全科医生。在医院提出的请求中，73% 是眼科、风湿科或皮肤科提出的请求，其余是一系列其他儿科专科提出的请求。结果范围为 11-195 U/L，中位数为 76 U/L。医院参考范围是13-64 U/L，18名患者的结果>64 U/L。文献中儿科患者的参考范围是 29-112 U/L。 6名患者的结果＞112U/L。一名患者被诊断为结节病。继初步结果之后，又对 2018 年 1 月 2 日至 2022 年 11 月 30 日期间的 4 年结果进行了分析。另外10名患者的ACE结果＞112U/L。其中，没有人患有结节病。结论 这些结果支持结节病的诊断很少见。解释 ACE 结果时需要注意，正常儿童 ACE 水平可能高于医院给出的参考范围，而医院给出的参考范围可能基于成人范围。该领域需要更多的研究来确定诊断性 ACE 水平以及 ACE 在疾病监测中的作用。我计划领导一个实习生多中心回顾性项目，研究儿童结节病的诊断，并在本次回顾中探讨 ACE 水平的作用。披露 MC Mackay：无。 S.迪帕克：没有。 K. Warrier：没有。 S. Rangaraj：没有。