title: Improvement of nephrotic syndrome by intensive lipid-lowering therapy in a patient with lipoprotein glomerulopathy.
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.
authors: Department of Pediatrics, Yamagata University School of Medicine, Iida-Nishi, Yamagata, Japan. matsunagattuso@yahoo.co.jp
authors: Akira
authors: A
authors: Matsunaga
authors: Masayuki
authors: M
authors: Furuyama
authors: Taeko
authors: T
authors: Hashimoto
authors: Kentaro
authors: K
authors: Toyoda
authors: Daisuke
authors: D
authors: Ogino
authors: Kiyoshi
authors: K
authors: Hayasaka
mesh_terms: D000293:Adolescent; D047228:Angiotensin II Type 1 Receptor Blockers / Q000627:therapeutic use; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D001057:Apolipoproteins E / Q000097:blood; D001629:Bezafibrate / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D004656:Enalapril / Q000627:therapeutic use; D005260:Female; D006417:Hematuria / Q000188:drug therapy; D006801:Humans; D000960:Hypolipidemic Agents / Q000627:therapeutic use*; D007674:Kidney Diseases / Q000188:drug therapy; D007676:Kidney Failure, Chronic / Q000188:drug therapy; D007678:Kidney Glomerulus / Q000187:drug effects; D009404:Nephrotic Syndrome / Q000188:drug therapy*; D011341:Probucol / Q000627:therapeutic use; D013777:Tetrazoles / Q000627:therapeutic use; D014633:Valine / Q000031:analogs & derivatives / Q000627:therapeutic use; D000068756:Valsartan
chemical_list: D047228:Angiotensin II Type 1 Receptor Blockers; D000806:Angiotensin-Converting Enzyme Inhibitors; D001057:Apolipoproteins E; D000960:Hypolipidemic Agents; D013777:Tetrazoles; D004656:Enalapril; D000068756:Valsartan; D014633:Valine; D011341:Probucol; D001629:Bezafibrate