Dilated Cardiomyopathy Concomitant with Acute Rheumatic Fever and Sporadic Hereditary Spherocytosis

Fitrisia Amelin, D. Hariyanto, Amizah Zatil Izzah
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Abstract

Dilated cardiomyopathy (DCM) is a heart muscle disorder defined by the presence of a dilated and poorly functioning left ventricle in the absence of abnormal loading conditions (hypertension, valve disease) or ischaemic heart disease sufficient to cause global systolic impairment. A 5 years old and 6 months old girl with unremarkable cardiac history hospitalized for congestive heart failure due to dilated cardiomyopathy concomitant with acute rheumatic feverandsporadic hereditary spherocytosis.Acute rheumatic fever diagnosed based on the presentation of carditis, fever, high erythrocyte sedimentation rate, C-Reactive Protein (+), and antistreptolysin titer O (+).DCM was diagnosed after echocardiography. Sporadic hereditary spherocytosis was diagnosed based on anamnesis of pale and jaundice, splenomegaly on physical examination, hemolytic anemia, reticulocytosis, spherosit (+). Both parents reveals normal hematologic finding and the osmotic resistance test showed increasing of osmotic fragility. Prognosis of this patient remains worse because of inadequateleft ventricle-right ventricle (LV-RV) function and highly increasing The N-terminal prohormone of brain natriuretic peptide (NT-proBNP) level.
扩张型心肌病伴发急性风湿热和散发的遗传性球形细胞增多症
扩张型心肌病(DCM)是一种心肌疾病,在没有异常负荷条件(高血压、瓣膜疾病)或缺血性心脏病足以引起整体收缩损伤的情况下,左心室扩张和功能不良。一例5岁零6个月大的女婴,心脏病史一般,因扩张型心肌病合并急性风湿热和散发遗传性球形细胞增多症引起的充血性心力衰竭住院。急性风湿热诊断基于表现为心炎,发烧,高红细胞沉降率,c反应蛋白(+)和抗溶血素滴度O(+)。超声心动图诊断DCM。散发性遗传性球形红细胞增多症的诊断依据是苍白和黄疸的记忆,体格检查脾肿大,溶血性贫血,网状红细胞增多症,球状红细胞增多症(+)。双亲血液学检查正常,渗透阻力试验显示渗透脆性增加。由于左-右心室(LV-RV)功能不全,且脑利钠肽n端原激素(NT-proBNP)水平增高,患者预后仍较差。
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