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贝赫切特病的罕见儿科心肺表现。

Rare paediatric cardiopulmonary presentation of Behcet's disease.

机构信息

Oliver Fisher Neonatal Unit, Medway Maritime Hospital, Gillingham, UK

Neonatal Transfer Service, The Royal London Hospital, London, UK.

出版信息

BMJ Case Rep. 2022 Mar 9;15(3):e248134. doi: 10.1136/bcr-2021-248134.

Abstract

This case discusses a 10-year-old boy who presented in significant respiratory distress with cardiac tamponade with associated gross ascites and hepatomegaly, requiring urgent transfer for pericardiocentesis. On further investigation, he was found to have multiple pulmonary emboli and evidence of panserositis. An underlying rheumatological cause was suspected in the absence of any evidence of infection or malignancy, and blood tests were positive for anti-double stranded DNA, anticardiolipin and antiglycoprotein antibodies as well as HLA B51. In his medical history, he has previously had mouth ulcers, chronic anaemia of undetermined cause and erythema multiforme. These symptoms, along with clinical presentation, mean a diagnosis of Behcet's disease and associated antiphospholipid syndrome was felt to be most likely. Anticoagulation therapy was commenced for treatment of the emboli, and colchicine was started for management of Behcet's disease. The patient was discharged clinically well from the hospital and continues under specialist rheumatological and haematological follow-up.

摘要

本病例讨论了一名 10 岁男孩,他因心脏压塞伴有大量腹水和肝肿大而出现严重呼吸窘迫,需要紧急转介进行心包穿刺。进一步检查发现他有多发性肺栓塞和腹膜炎的证据。由于没有感染或恶性肿瘤的证据,怀疑存在潜在的风湿性病因,血液检查抗双链 DNA、抗心磷脂和抗糖蛋白抗体以及 HLA B51 呈阳性。在他的病史中,他以前曾有口腔溃疡、原因不明的慢性贫血和多形红斑。这些症状以及临床表现表明,贝赫切特病和相关抗磷脂综合征的诊断最有可能。为了治疗栓塞,开始进行抗凝治疗,同时开始使用秋水仙碱治疗贝赫切特病。该患者临床状况良好出院,并继续接受专科风湿学和血液学随访。

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本文引用的文献

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Atypical cardiopulmonary manifestations in pediatric Behçet's disease.儿童贝赫切特病的非典型心肺表现。
Pediatr Pulmonol. 2020 Dec;55(12):3407-3413. doi: 10.1002/ppul.25068. Epub 2020 Sep 25.
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Juvenile-onset Behçet's syndrome and mimics.幼年发病型贝赫切特综合征及其伴发疾病。
Clin Immunol. 2020 May;214:108381. doi: 10.1016/j.clim.2020.108381. Epub 2020 Mar 10.
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Diagnostic/classification criteria in pediatric Behçet's disease.儿童白塞病的诊断/分类标准。
Rheumatol Int. 2019 Jan;39(1):37-46. doi: 10.1007/s00296-018-4208-9. Epub 2018 Nov 14.
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Behçet's disease in children, an overview.儿童贝赫切特病概述
Pediatr Rheumatol Online J. 2016 Feb 18;14(1):10. doi: 10.1186/s12969-016-0070-z.

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