Successful treatment of lipoprotein glomerulopathy in a daughter and a mother using niceritrol

Hamatani, Hiroko; Hiromura, Keiju; Kobatake, Keiko; Yoshida, Hiroaki; Kobayashi, Shohei; Yoneda, Naohiro; Kayakabe, Ken; Matsumoto, Takayuki; Kuroiwa, Takashi; Ueki, Kazue; Nojima, Yoshihisa

doi:10.1007/s10157-010-0333-9

Cited by 11 publications

(12 citation statements)

References 16 publications

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“…Protein A immunoadsorption [20] and LDL-apheresis [21] could induce remission of LPG, but are very expensive. Treatment with intensive lipid-lowering agents, including fibrates, has been reported to lead to clinical remission along with histological resolution of lipoprotein thrombi in serial biopsies [22-25]. Clinical remission of LPG may reduce the risks of malignant hypertension with TMA.…”

Section: Discussionmentioning

confidence: 99%

A case of lipoprotein glomerulopathy with thrombotic microangiopathy due to malignant hypertension

Chen

Yang

et al. 2013

BMC Nephrol

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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 the APOE Kyoto 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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Section: Discussionmentioning

confidence: 99%

A case of lipoprotein glomerulopathy with thrombotic microangiopathy due to malignant hypertension

Chen

Yang

et al. 2013

BMC Nephrol

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“…Z Hu et al: APOE Kyoto mutation assembled in China c l i n i c a l i n v e s t i g a t i o n Ninety LPG cases have been reported worldwide, 4,9,10,17,[19][20][21][24][25][26][27][28][29][30][31][32][33] nine with the APOE Kyoto mutation. 8,14,[16][17][18][19][20][21] Herein, we presented 35 LPG patients carrying the APOE Kyoto allele, making it the most common mutation related to LPG.…”

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confidence: 99%

Hereditary features, treatment, and prognosis of the lipoprotein glomerulopathy in patients with the APOE Kyoto mutation

Huang

et al. 2014

Kidney International

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Lipoprotein glomerulopathy is a rare inherited renal disease, caused by mutation of the APOE gene, characterized by proteinuria and nephrotic syndrome with elevated serum apoE. Since its treatment and outcome are unknown, we retrospectively studied 35 patients within 31 unrelated Han families with biopsy-proven lipoprotein glomerulopathy residing in the same county in southwest China. DNA sequencing detected the APOE Kyoto mutation (p. Arg25Cys) in all patients and 28 asymptomatic relatives. All shared the same ɛ3 allele. The patients presented with proteinuria, higher total triglyceride, and serum apoE levels relative to non-carriers. The serum apoE and triglyceride levels of asymptomatic carriers were between those of the patients and non-carriers. Sixteen patients received fenofibrate treatment for over 12 months. Six reached complete remission (proteinuria under 0.3 g/day with stable serum creatinine) with intensive control of their lipid profile (normalized serum apoE and triglycerides under 100 mg/dl). Eight reached partial remission. At 3 years of follow-up, patients treated with fenofibrate had superior survival and stable renal function. Thus, fenofibrate can induce lipoprotein glomerulopathy remission and the fibrate effects depend on the degree of lipid control and baseline proteinuria. Moreover, normalization of serum apoE and triglycerides can be used to judge the efficacy of lipid-lowering treatment.

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“…Although LPG is known to have a poor renal prognosis, case reports of successful treatment with lipid-lowering therapy have been increasing [17][18][19][20][21][22][23]. Interestingly, the course of the urinary protein level in our patient closely paralleled his TG and cholesterol levels ( Fig.…”

Section: Discussionmentioning

confidence: 62%

Five-year follow-up of a case of lipoprotein glomerulopathy with APOE Kyoto mutation

et al. 2016

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We report the case of a 34-year-old Japanese male with lipoprotein glomerulopathy (LPG). Renal biopsy showed LPG, and followed by a genetic analysis revealed a mutation in apolipoprotein E gene (APOE Kyoto; Arg25-Cys). We started treatment with probucol, bezafibrate, losartan, and allopurinol. Urinary protein decreased in response to treatment but has remained at about 1.27 ± 0.71 g/gCr, and a repeat biopsy which was performed 1 year after the first biopsy showed no clear evidence of pathological remission and complication of other glomerular disease. After 5 years of follow-up after the start of treatment, renal function has almost maintained without apparent deterioration. Interestingly, the course of the urinary protein level closely paralleled his triglyceride and cholesterol levels in a long-term. This observation suggests the importance of tight control of lipid profiles as a means of renoprotection in LPG patient.

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Successful treatment of lipoprotein glomerulopathy in a daughter and a mother using niceritrol

Cited by 11 publications

References 16 publications

A case of lipoprotein glomerulopathy with thrombotic microangiopathy due to malignant hypertension

A case of lipoprotein glomerulopathy with thrombotic microangiopathy due to malignant hypertension

Hereditary features, treatment, and prognosis of the lipoprotein glomerulopathy in patients with the APOE Kyoto mutation

Five-year follow-up of a case of lipoprotein glomerulopathy with APOE Kyoto mutation

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