Abstract:
:Lipoprotein glomerulopathy (LPG) is a rare hereditary disease characterized by the accumulation of much thrombi material consisting of lipoproteins at the glomerular capillary lumen. Most patients show nephrotic syndrome; nearly half progress to chronic renal failure. Intensive therapy with lipid-lowering agents reportedly engenders clinical remission with histological resolution. We report the case of a 14-year-old Japanese female patient who had been in a nephrotic condition with hematuria from 4 years old and who had been diagnosed based on pathological and molecular examination at 7 years old. We initially treated the patient with probucol, enalapril (angiotensin-converting enzyme inhibitor: ACEI), and dipyridamole from age 7, but achieved no improvement in her nephrotic status. Subsequently, we replaced probucol with bezafibrate at age 11 and added atorvastatin calcium hydrate and valsartan (angiotensin II receptor blocker: ARB) the following year. The next 3 years' treatment improved her nephrotic status, decreased serum apolipoprotein E, and markedly decreased intraglomerular lipoprotein thrombi. Early and intensive therapy with antilipidemic drugs combined with ACEI and ARB is inferred to be effective for LPG.
journal_name
Clin Exp Nephroljournal_title
Clinical and experimental nephrologyauthors
Matsunaga A,Furuyama M,Hashimoto T,Toyoda K,Ogino D,Hayasaka Kdoi
10.1007/s10157-009-0207-1subject
Has Abstractpub_date
2009-12-01 00:00:00pages
659-62issue
6eissn
1342-1751issn
1437-7799journal_volume
13pub_type
杂志文章abstract::A 61-year-old man infected with hepatitis C virus developed urinary protein. Two-dimensional electrophoresis and immunoblotting of sera revealed no monoclonal proteins. Light microscopy and immunofluorescence of a kidney biopsy specimen demonstrated bubbling appearance and formation of spikes, associated with predomin...
journal_title:Clinical and experimental nephrology
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journal_title:Clinical and experimental nephrology
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journal_title:Clinical and experimental nephrology
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journal_title:Clinical and experimental nephrology
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更新日期:2002-09-01 00:00:00
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doi:10.1007/s10157-011-0521-2
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journal_title:Clinical and experimental nephrology
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journal_title:Clinical and experimental nephrology
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journal_title:Clinical and experimental nephrology
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