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Premature Coronary Atherosclerosis in a 5-Year-Old With Corticosteroid-Refractory Nephrotic Syndrome FREE

Ronald J. Kallen, MD; Russell K. Brynes, MD; Andrew J. Aronson, MD; Chaim Lichtig, MD; Benjamin H. Spargo, MD
[+] Author Affiliations

Reprint requests to Cleveland Clinic Foundation, Desk 87W, 9500 Euclid Av, Cleveland, OH 44106 (Dr Kallen.)


Arch Pediatr Adolesc Med. 1977;131(9):976-980. doi:10.1001/archpedi.1977.02120220042006
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• We report the case of a 5-year-old girl who died two years after onset of the idiopathic nephrotic syndrome, which failed to respond to treatment with corticosteroid and cyclophosphamide. Severe atherosclerotic changes were noted in both coronary arteries. Prolonged hyperlipidemia in patients with long-standing nephrotic syndrome may represent a major risk factor predisposing to premature coronary atherosclerosis in children who are also destined to develop chronic renal failure.

(Am J Dis Child 131:976-980, 1977)

REFERENCES

Churg J, Habib R, White RHR:  A report of the international study of kidney disease in children: Pathology of the nephrotic syndrome in childhood . Lancet 1:1299-1302, 1970;.
White RHR, Glasgow EF, Mills RJ:  Clinicopathologic study of nephrotic syndrome in childhood . Lancet 1:1353-1359, 1970;.
Habib R:  Focal glomerular sclerosis . Kidney Int 4:355-361, 1973;.
Siegel NJ, Kashgarian M, Spargo BH, et al:  Minimal change and focal sclerotic lesions in lipoid nephrosis . Nephron 13:125-137, 1974;.
Habib R, Kleinknecht C, Gubler M-C:  Extramembranous glomerulonephritis in children: Report of 50 cases . J Pediatr 82:754-766, 1973;.
Berlyne GM, Mallick NP:  Ischaemic heart-disease as a complication of nephrotic syndrome . Lancet 1:399-400, 1969;.
Alexander JH, Schapel GJ, Edwards KDG:  Increased incidence of coronary heart disease associated with combined elevation of serum triglyceride and cholesterol concentrations in the nephrotic syndrome in man . Med J Aust 2:119-122, 1974;.
Stern MP, Kolterman OG, Fries JF, et al:  Adrenocortical steroid treatment of rheumatic diseases . Arch Intern Med 132:97-101, 1973;.
Bagdade JD, Porte D Jr, Bierman EL:  Hypertriglyceridemia: A metabolic consequence of chronic renal failure . N Engl J Med 279:181-185, 1968;.
Ibels LS, Simons LA, King JO, et al:  Studies on the nature and causes of hyperlipidemia in uraemia, maintenance dialysis and renal transplantation . Q J Med 44:601-614, 1975;.
Kannel WB, Dawber TR:  Atherosclerosis as a pediatric problem . J Pediatr 80:544-554, 1972;.
Schwarz H, Kohn JL:  Lipoid nephrosis: A clinical and pathologic study based on 15 years' observation with special reference of prognosis . Am J Dis Child 49:579-593, 1935;.
Gofman JW, Jones HB, Lindgren FT, et al:  Blood lipids and human atherosclerosis . Circulation 2:161-178, 1950;.
Berlyne GM, Tavill AS, Baker SBD:  Renal artery stenosis and the nephrotic syndrome . Q J Med 33:325-335, 1964;.
Kallen RJ, Lee S-K, Aronson AJ, et al:  Idiopathic membranous glomerulopathy preceeding the emergence of systemic lupus erythematosus in two children . J Pediatr 90:72-76, 1977;.
Matsuda I:  Incidence of atherosclerosis in nephrosis . J Pediatr 67:152, 1965;.
Fredrickson DS, Levy RI, Lees RS:  Medical progress: Fat transport in lipoproteins—an integrated approach to mechanisms and disorders . N Engl J Med 276:32-44, 94-103, 148-156, 215-226, 273-281, 1967;.
Baxter JH, Goodman HC, Havel RJ:  Serum lipid and lipoprotein alterations in nephrosis . J Clin Invest 39:455-465, 1960;.
Chopra JS, Mallick NP:  Hyperlipoproteinaemias in nephrotic syndrome . Lancet 1:317-321, 1971;.
Newmark SR, Anderson CF, Donadio JV, et al:  Lipoprotein profiles in adult nephrotics . Mayo Clinic Proc 50:359-364, 1975;.
Slack J:  Risks of ischaemic heart-disease in familial hyperlipoproteinaemic states . Lancet 2:1380-1382, 1969;.
Glueck CJ, Fallat RW, Tsang R, et al:  Hyperlipemia in progeny of parents with myocardial infarction before age 50 . Am J Dis Child 127:70-75, 1974;.
Council on Foods and Nutrition:  Diet and coronary heart disease: A council statement . JAMA 222:1647, 1972;.
Kannel WB, Castelli WP, Gordon T, et al:  Serum cholesterol, lipoproteins, and the risk of coronary heart disease: The Framingham study . Ann Intern Med 74:1-12, 1971;.
International Study of Kidney Disease in Children:  Prospective, controlled trial of cyclophosphamide therapy in children with the nephrotic syndrome . Lancet 2:423-427, 1974;.
Siegel NJ, Goldberg B, Krassner LS, et al:  Long-term follow-up of children with steroid-responsive nephrotic syndrome . J Pediatr 81:251-258, 1972;.
Schwartz MW, Schwartz GJ, Cornfeld D:  A 16-year follow-up study of 163 children with nephrotic syndrome . Pediatrics 54:547-552, 1974;.
Lindner A, Charra B, Sherrard DJ:  Accelerated atherosclerosis in prolonged maintenance hemodialysis . N Engl J Med 290:697-701, 1974;.
Pennisi AJ, Fiedler J, Lipsey A, et al:  Hyperlipidemia in pediatric renal allograft recipients . J Pediatr 87:249-251, 1975;.
Ibels LS, Stewart JH, Mahony JF, et al:  Deaths from occlusive arterial disease in renal allograft recipients . Br Med J 3:552-554, 1974;.
Lees RS, Wilson DE:  Medical Progress—The treatment of hyperlipidemia . N Engl J Med 284:186-195, 1971;.
National Institutes of Health Conference:  Dietary and drug treatment of primary hyperlipoproteinemia . Ann Intern Med 77:267-294, 1972;.
Bridgman JF, Rosen SM, Thorp JM:  Complications during clofibrate treatment of nephrotic-syndrome hyperlipoproteinaemia . Lancet 1:506-509, 1972;.
Fabre J, Denizot M, Micheli H, et al:  Correction of hyperlipemia of the nephrotic syndrome by clofibrate: Muscular side-effect of the drug , abstract ed. Kidney Int 3:273, 1973;.
Larsen R, Glueck CJ, Tsang R:  Special diet for familial type II hyperlipoproteinemia . Am J Dis Child 128:67-72, 1974;.

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Churg J, Habib R, White RHR:  A report of the international study of kidney disease in children: Pathology of the nephrotic syndrome in childhood . Lancet 1:1299-1302, 1970;.
White RHR, Glasgow EF, Mills RJ:  Clinicopathologic study of nephrotic syndrome in childhood . Lancet 1:1353-1359, 1970;.
Habib R:  Focal glomerular sclerosis . Kidney Int 4:355-361, 1973;.
Siegel NJ, Kashgarian M, Spargo BH, et al:  Minimal change and focal sclerotic lesions in lipoid nephrosis . Nephron 13:125-137, 1974;.
Habib R, Kleinknecht C, Gubler M-C:  Extramembranous glomerulonephritis in children: Report of 50 cases . J Pediatr 82:754-766, 1973;.
Berlyne GM, Mallick NP:  Ischaemic heart-disease as a complication of nephrotic syndrome . Lancet 1:399-400, 1969;.
Alexander JH, Schapel GJ, Edwards KDG:  Increased incidence of coronary heart disease associated with combined elevation of serum triglyceride and cholesterol concentrations in the nephrotic syndrome in man . Med J Aust 2:119-122, 1974;.
Stern MP, Kolterman OG, Fries JF, et al:  Adrenocortical steroid treatment of rheumatic diseases . Arch Intern Med 132:97-101, 1973;.
Bagdade JD, Porte D Jr, Bierman EL:  Hypertriglyceridemia: A metabolic consequence of chronic renal failure . N Engl J Med 279:181-185, 1968;.
Ibels LS, Simons LA, King JO, et al:  Studies on the nature and causes of hyperlipidemia in uraemia, maintenance dialysis and renal transplantation . Q J Med 44:601-614, 1975;.
Kannel WB, Dawber TR:  Atherosclerosis as a pediatric problem . J Pediatr 80:544-554, 1972;.
Schwarz H, Kohn JL:  Lipoid nephrosis: A clinical and pathologic study based on 15 years' observation with special reference of prognosis . Am J Dis Child 49:579-593, 1935;.
Gofman JW, Jones HB, Lindgren FT, et al:  Blood lipids and human atherosclerosis . Circulation 2:161-178, 1950;.
Berlyne GM, Tavill AS, Baker SBD:  Renal artery stenosis and the nephrotic syndrome . Q J Med 33:325-335, 1964;.
Kallen RJ, Lee S-K, Aronson AJ, et al:  Idiopathic membranous glomerulopathy preceeding the emergence of systemic lupus erythematosus in two children . J Pediatr 90:72-76, 1977;.
Matsuda I:  Incidence of atherosclerosis in nephrosis . J Pediatr 67:152, 1965;.
Fredrickson DS, Levy RI, Lees RS:  Medical progress: Fat transport in lipoproteins—an integrated approach to mechanisms and disorders . N Engl J Med 276:32-44, 94-103, 148-156, 215-226, 273-281, 1967;.
Baxter JH, Goodman HC, Havel RJ:  Serum lipid and lipoprotein alterations in nephrosis . J Clin Invest 39:455-465, 1960;.
Chopra JS, Mallick NP:  Hyperlipoproteinaemias in nephrotic syndrome . Lancet 1:317-321, 1971;.
Newmark SR, Anderson CF, Donadio JV, et al:  Lipoprotein profiles in adult nephrotics . Mayo Clinic Proc 50:359-364, 1975;.
Slack J:  Risks of ischaemic heart-disease in familial hyperlipoproteinaemic states . Lancet 2:1380-1382, 1969;.
Glueck CJ, Fallat RW, Tsang R, et al:  Hyperlipemia in progeny of parents with myocardial infarction before age 50 . Am J Dis Child 127:70-75, 1974;.
Council on Foods and Nutrition:  Diet and coronary heart disease: A council statement . JAMA 222:1647, 1972;.
Kannel WB, Castelli WP, Gordon T, et al:  Serum cholesterol, lipoproteins, and the risk of coronary heart disease: The Framingham study . Ann Intern Med 74:1-12, 1971;.
International Study of Kidney Disease in Children:  Prospective, controlled trial of cyclophosphamide therapy in children with the nephrotic syndrome . Lancet 2:423-427, 1974;.
Siegel NJ, Goldberg B, Krassner LS, et al:  Long-term follow-up of children with steroid-responsive nephrotic syndrome . J Pediatr 81:251-258, 1972;.
Schwartz MW, Schwartz GJ, Cornfeld D:  A 16-year follow-up study of 163 children with nephrotic syndrome . Pediatrics 54:547-552, 1974;.
Lindner A, Charra B, Sherrard DJ:  Accelerated atherosclerosis in prolonged maintenance hemodialysis . N Engl J Med 290:697-701, 1974;.
Pennisi AJ, Fiedler J, Lipsey A, et al:  Hyperlipidemia in pediatric renal allograft recipients . J Pediatr 87:249-251, 1975;.
Ibels LS, Stewart JH, Mahony JF, et al:  Deaths from occlusive arterial disease in renal allograft recipients . Br Med J 3:552-554, 1974;.
Lees RS, Wilson DE:  Medical Progress—The treatment of hyperlipidemia . N Engl J Med 284:186-195, 1971;.
National Institutes of Health Conference:  Dietary and drug treatment of primary hyperlipoproteinemia . Ann Intern Med 77:267-294, 1972;.
Bridgman JF, Rosen SM, Thorp JM:  Complications during clofibrate treatment of nephrotic-syndrome hyperlipoproteinaemia . Lancet 1:506-509, 1972;.
Fabre J, Denizot M, Micheli H, et al:  Correction of hyperlipemia of the nephrotic syndrome by clofibrate: Muscular side-effect of the drug , abstract ed. Kidney Int 3:273, 1973;.
Larsen R, Glueck CJ, Tsang R:  Special diet for familial type II hyperlipoproteinemia . Am J Dis Child 128:67-72, 1974;.

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