摘要
目的对1例以胸闷、乏力、水肿及少尿等心力衰竭症状为主要表现的患儿进行诊断及治疗。方法根据患儿症状、体征、既往病史、辅助检查结果及文献资料分析进行确诊,并予相应治疗。结果患儿以心力衰竭的症状为主要表现,查体有体、肺循环淤血体征,超声心动图及心脏磁共振检查发现患儿存在心脏扩大及心功能低下,且存在心肌纤维化,可确诊为扩张型心肌病,结合既往有烟雾病史,且多次血常规检查存在血小板减少,且抗心磷脂抗体、狼疮抗凝物及抗-β2糖蛋白Ⅰ抗体均为阳性,患儿可确诊为抗磷脂综合征(APS)。可排除存在系统性红斑狼疮、肿瘤及常见病毒感染,因此考虑患儿同时为原发性抗磷脂抗体综合征(PAPS)患者。予免疫抑制剂及抗凝治疗后患儿病情有好转。结论PAPS可并扩张型心肌病,虽然比较少见,但是应当提高认识,以防漏诊。
Objective Primary antiphospholipid syndrome （PAPS） is a rare syndrome in children, and dilated cardiomyopathy in PAPS is extremely rare. At present,only 1 child was reported as antiphospholipid syndrome（APS） with dilated cardiomyopathy. A child suffering from heart failure was diagnosed. Methods According to the symptoms, signs, past history, laboratory examination and the previous published papers of other authors,child＇s disease was diagnosed and treated, and related literature was reviewed. Results A 12 - year old boy presented with heart failure, fatigue, swollen limbs and oliguria, His echocardiogram and heart MRI showed that the left extended ventricular diameter, low systolic function, and mild mitral, pulmonary valve and aortic valve regulation. His heart MRI also showed myocardial fibrosis, .so the dilated cardiomyopathy was diagnosed.Laboratory stuides showed the presence of mild thrombosytopenia, high tilters of anticardiolipin antibodies, anti - β2- glycoprotein Ⅰ antibodies and a lupus anticogulant. A diagnosis of PAPS was made because we could not document any underlying causes of the APS. He was treated with high dose intravenous immunoglobulin, methylprednisolone, and stroll dose of aspirin. The symptoms and the signs were improved. Conclusion PAPS is a rare disorder and less recognized in children,and the dilated cardiomyopathy might result from PAPS, and attention should be paid to misdiagnosis.
出处
《实用儿科临床杂志》
CAS
CSCD
北大核心
2006年第9期538-539,共2页
Journal of Applied Clinical Pediatrics
关键词
抗磷脂综合征
心肌病
充血性
儿童
antiphospholipid syndrome
cardiomyopathy, congestive
child
