A case of lipoprotein glomerulopathy with thrombotic microangiopathy due to malignant hypertension.
A case of lipoprotein glomerulopathy with thrombotic microangiopathy due to malignant hypertension.
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恶性高血压脂蛋白肾小球病伴血栓性微血管病一例
DOI：
10.1186/1471-2369-14-53
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发表时间：
2013-02-28
期刊：
影响因子：
2.3
通讯作者：
Hu Z
中科院分区：
文献类型：
--
作者：
Wu Y;Chen X;Yang Y;Wang B;Liu X;Tao Y;Fu P;Hu Z
脂蛋白肾小球病变(LPG)是一种罕见的遗传性肾病，其特征是肾小球毛细血管严重扩张的管腔内的脂蛋白。LPG的常见临床表现为蛋白尿或肾病综合征。在液化石油气中，高血压和贫血被认为是轻微的。液化石油气血栓性微血管病(TMA)此前未见报道。在这份报告中，我们介绍了一位发生TMA的液化石油气患者。4年前(2005年)，一位19岁的中国女性被诊断为肾病综合征，并接受了泼尼松治疗。泼尼松和环磷酰胺的组合没有任何效果，六个月后停止使用。尽管她对类固醇耐药，但在接下来的三年里，她保持了正常的肾功能，没有贫血和血小板减少。2009年2月，她出现严重头痛和视力模糊，并因严重高血压在当地一家医院就诊。血压为2 2 0/16 0 毫米汞。实验室资料显示：血红蛋白3.8 g/dL；血小板29×109/L；尿蛋白7.9 g/d；总胆红素29.9 umol/L；间接胆红素28.2 umol/L；乳酸脱氢酶1172U/L；白蛋白2.66 g/dL；尿素氮52 mg/dL；血肌酐3.2 mg/dL；甘油三酯253 mg/dL；总胆固醇273 mg/dL。ANA、ds-DNA、ANCA、抗GBM抗体、抗心磷脂均为阴性。肾活检显示LPG合并TMA。基因评估显示，患者携带APOE京都突变。血压的适当控制改善了微血管病理性贫血和血小板减少，但肾功能没有改善，最终发展为尿毒症，并成为血液透析依赖。结论我们报告了一例罕见的TMA病例，可能是由于液化石油气恶性高血压所致。早期降脂和降压治疗可能会改善预后。LPG与TMA的病理生理关系有待进一步研究。
BackgroundLipoprotein glomerulopathy (LPG) is a rare inherited renal disease characterized by intraglomerular lipoprotein within the lumina of severely dilated glomerular capillaries. The common clinical presentation of LPG includes proteinuria or nephrotic syndrome. Hypertension and anemia were thought to be mild in LPG. Thrombotic microangiopathy (TMA) in LPG has not been previously reported. In this report, we present a patient with LPG that developed TMA. To the best of our knowledge, this is the first report of TMA in LPG.Case presentationFour years ago (2005), a 19-year-old Chinese woman was diagnosed with nephrotic syndrome and provided prednisone treatment. A combination of prednisone and cyclophosphamide did not have any effect and was discontinued after six months. Although she was steroid-resistant, over the next subsequent three years, she maintained normal renal function without anemia and thrombocytopenia. In February 2009, she had a severe headache and blurry vision and presented at a local hospital with severe hypertension. Blood pressure was 220/160 mmHg. Laboratory data showed hemoglobin 3.8 g/dL; platelet counts 29×109/L; urinary protein 7.90 g/d; total bilirubin 29.9 umol/L; indirect bilirubin 28.2 umol/L; LDH 1172 U/L; ALB 2.66 g/dL; urea nitrogen 52 mg/dL; serum creatinine 3.2 mg/dL; triglyceride 253 mg/dL; total cholesterol 273 mg/dL. ANA, ds-DNA, ANCA, anti-GBM antibody and anticardiolipin were all negative. A renal biopsy revealed LPG with TMA. Genetic evaluation showed the patient carried theAPOEKyoto mutation. Adequate control of blood pressure improved microangiopathic anemia and thrombocytopenia, however, renal function did not improve and she eventually developed uremia and became hemodialysis dependent.ConclusionWe report on a rare case of TMA probably due to malignant hypertension in LPG. Early lipid-lowering and antihypertensive treatment may improve outcome. The pathophysiologic relationship between LPG and TMA should be investigated further.
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影响因子：
6.1
作者：
Zhang Xin;Liu Zhihong;Li Leishi
通讯作者：
Li Leishi
影响因子：
5.3
作者：
Arai, T;Yamashita, S;Matsuzawa, Y
通讯作者：
Matsuzawa, Y
影响因子：
4.9
作者：
LIP, GYH;BEEVERS, M;BEEVERS, DG
通讯作者：
BEEVERS, DG
影响因子：
3.4
作者：
Yokomine T;Hirakawa H;Ozawa E;Shibata K;Nakayama T
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Nakayama T
影响因子：
13.2
作者：
Ieiri, N;Hotta, O;Taguma, Y
通讯作者：
Taguma, Y