伴有心外分流的多系统肉样瘤病病例报告

Faris K. Shamsudeen
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摘要

一名 61 岁的女性患者主诉干咳和逐渐加重的呼吸困难,病程 3 个月,体重明显下降超过 14 公斤。就诊时,患者身体不适、低氧血症、呼吸急促,系统检查显示听诊时有细微的基底吱吱声。胸部 CT 显示肺部随机结节,伴有支气管扩张病变。鉴于呼吸困难和 d-二聚体升高,做了 CT 肺血管造影,结果正常。对比超声心动图检查提示有明显的心外右向左分流。放射学证据显示,该患者患有慢性肝病,伴有门静脉高压,在造影后的图像中,肝脏和脾脏实质散布着许多界限不清的结节性不透明斑。ANA 蛋白呈核糖体-p 蛋白阳性,血清 ACE 水平升高。最终在超声引导下进行了肝活检,组织病理学检查发现肝脏肉芽肿病变与肉样瘤病相符。
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A case report of multisystemic sarcoidosis with extracardiac shunt
A 61-year-old female patient, presented with complaints of dry cough and gradually worsening dyspnea of 3 months duration, associated with significant weight loss of >14 kg. At presentation, patient was sick, hypoxemic, and tachypneic and system examination revealed fine basal crepitations on auscultation. CT chest showed random pulmonary nodules with bronchiectatic changes. In view of respiratory distress and elevated d-dimer, CT pulmonary angiogram was done and was normal. A contrast echocardiography study hinted towards a significant right to left extracardiac shunt. There was radiological evidence of chronic liver disease with portal hypertension and numerous poorly defined nodular opacities scattered in hepatic and splenic parenchyma in post contrast images. ANA profile was positive for ribosomal-p protein along with elevated serum ACE levels. An ultrasound guided liver biopsy was ultimately performed and histopathology revealed granulomatous lesion in liver compatible with sarcoidosis.
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