Therapeutic management of a new case of LCAT deficiency with a multifactorial long-term approach based on high doses of angiotensin II receptor blockers (ARBs)

Aranda, Pedro J.; Valdivielso, Pedro; Pisciotta, L; Garcia, I; C, Garcã A-Arias; Bertolini, S; G, Martã N-Reyes; Lez-Santos, Gonzã; Calandra, S.

doi:10.5414/cnp69213

Cited by 30 publications

(21 citation statements)

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“…Treatment with angiotensin converting enzyme inhibitors and angiotensin receptor blockers has been reported to reduce proteinuria and progression of renal disease. 90 Severe renal disease requires haemodialysis and, eventually, kidney transplantation, although the pathology often rapidly reappears. 91 Progression of corneal opacities could require corneal transplantation to restore vision.…”

Section: Current and Future Therapymentioning

confidence: 99%

Rare dyslipidaemias, from phenotype to genotype to management: a European Atherosclerosis Society task force consensus statement

Hegele

Borén

Ginsberg

et al. 2020

The Lancet Diabetes & Endocrinology

137

101

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Genome sequencing and gene-based therapies appear poised to advance the management of rare lipoprotein disorders and associated dyslipidaemias. However, in practice, underdiagnosis and undertreatment of these disorders are common, in large part due to interindividual variability in the genetic causes and phenotypic presentation of these conditions. To address these challenges, the European Atherosclerosis Society formed a task force to provide practical clinical guidance focusing on patients with extreme concentrations (either low or high) of plasma low-density lipoprotein cholesterol, triglycerides, or high-density lipoprotein cholesterol. The task force also recognises the scarcity of quality information regarding the prevalence and outcomes of these conditions. Collaborative registries are needed to improve health policy for the care of patients with rare dyslipidaemias.

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Section: Current and Future Therapymentioning

confidence: 99%

Rare dyslipidaemias, from phenotype to genotype to management: a European Atherosclerosis Society task force consensus statement

Hegele

Borén

Ginsberg

et al. 2020

The Lancet Diabetes & Endocrinology

137

101

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“…Eventually these patients can develop hypertension and end-stage renal disease, which can be treated by renal transplantation, but the disease can reoccur in the renal allograft[95]. A recent report has suggested that angiotensin-converting enzyme inhibitors, which reduces proteinuria, may be useful in these patients for delaying the progression of the renal disease[96]. …”

Section: Human Genetic Disorders Of Lcatmentioning

confidence: 99%

Lecithin: cholesterol acyltransferase – from biochemistry to role in cardiovascular disease

Rousset

Vaisman²,

Amar³

et al. 2009

Current Opinion in Endocrinology, Diabetes &Amp; Obesity

168

136

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Purpose of review We discuss the latest findings on the biochemistry of lecithin:cholesterol acyltransferase (LCAT), the effect of LCAT on atherosclerosis, clinical features of LCAT deficiency, and the impact of LCAT on cardiovascular disease from human studies. Recent findings Although there has been much recent progress in the biochemistry of LCAT and its effect on HDL metabolism, its role in the pathogenesis of atherosclerosis is still not fully understood. Studies from various animal models have revealed a complex interaction between LCAT and atherosclerosis that may be modified by diet and by other proteins that modify lipoproteins. Furthermore, the ability of LCAT to lower apoB appears to be the best way to predict its effect on atherosclerosis in animal models. Recent studies on patients with LCAT deficiency have shown a modest but significant increase incidence of cardiovascular disease consistent with a beneficial effect of LCAT on atherosclerosis. The role of LCAT in the general population, however, have not revealed a consistent association with cardiovascular disease. Summary Recent research findings from animal and humans studies have revealed a potential beneficial role of LCAT in reducing atherosclerosis but additional studies are necessary to better establish the linkage between LCAT and cardiovascular disease.

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“…Treatment with a combination of nicotinic acid and fenofibrate [24] or a high dose of angiotensin II receptor blockers [25] showed benefits in lipid abnormalities, proteinuria and kidney function. In proband WX we observed efficacy of proteinuria treatment with corticosteroids.…”

Section: Discussionmentioning

confidence: 98%

Corticosteroid treatment of kidney disease in a patient with familial lecithin-cholesterol acyltransferase deficiency

et al. 2011

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Familial lecithin-cholesterol acyltransferase (LCAT) deficiency (FLD) is a rare genetic disorder of lipid metabolism, characterised by low plasma HDL cholesterol, proteinuria, haemolytic anaemia and corneal opacities. Usually renal disease progresses during the third decade of life to renal failure; however the pathogenesis of renal disease is not well understood. In this study we describe treatment of renal disease in two siblings with FLD. The proband WX at the age of 31 years presented proteinuria and ankle oedema during her third pregnancy. Diagnosis of FLD was based on a renal biopsy with characteristic serpiginous fibrillar deposits under electron microscopy, markedly decreased HDL cholesterol, esterified cholesterol levels and LCAT activity, confirmed by molecular analysis. After 3 years her proteinuria increased and she received an ACE inhibitor to which she responded well. During further increases of proteinuria she additionally received methylprednisolone and her proteinuria decreased. This long-term observation indicates the efficacy of corticosteroids and renin-angiotensin-aldosterone system blockers in the treatment of proteinuria in patients with FLD. The results suggest the role of inflammatory processes as well as dyslipidemia in the pathogenesis of glomerular disorders in LCAT-deficient patients.

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Therapeutic management of a new case of LCAT deficiency with a multifactorial long-term approach based on high doses of angiotensin II receptor blockers (ARBs)

Cited by 30 publications

References 0 publications

Rare dyslipidaemias, from phenotype to genotype to management: a European Atherosclerosis Society task force consensus statement

Rare dyslipidaemias, from phenotype to genotype to management: a European Atherosclerosis Society task force consensus statement

Lecithin: cholesterol acyltransferase – from biochemistry to role in cardiovascular disease

Corticosteroid treatment of kidney disease in a patient with familial lecithin-cholesterol acyltransferase deficiency

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