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An Unusual Variation of Familial Nephrosis

An Unusual Variation of Familial Nephrosis Abstract This is a report of an unusual occurrence of cases of nephrotic syndrome in a family in which a mother, her brother, and her child had the steroidsensitive relapsing form of the disease. The nephrotic syndrome in children is usually idiopathic and rarely familial. Its peak incidence is in children ages 1 to 6 years. It generally follows a relapsing course, but is steroid sensitive and demonstrates minimal glomerular changes on pathologic study. It is very unusual to see a familial pattern to the disease, except in the rare autosomal recessive syndrome or in association with certain systemic metabolic disorders of which the nephrotic syndrome is only one component. Report of Cases.—Case 1.—Nephrosis developed in the index patient at age 8 years. Her clinical course and laboratory values were consistent with the diagnosis of idiopathic nephrotic syndrome of childhood. She has always been steroid sensitive. A needle biopsy References 1. Bader PL, Grove J, Trygstad CW, et al: Familial nephrotic syndrome . Am J Med 56:34-43, 1974.Crossref 2. White RH: The familial nephrotic syndrome: I. A European survey . Clin Nephrol 1:215-219, 1973. 3. Moncrieff MW, White RHR, Glascow EF, et al: The familial nephrotic syndrome: II. A clinicopathological study . Clin Nephrol 1:220-229, 1973. 4. Huttunen NP: Congenital nephrotic syndrome of Finnish type: Study of 75 patients . Arch Dis Child 51:344-348, 1976.Crossref 5. Burke EC, Holley KE, Stickler GB: Familial nephrotic syndrome with nephrocalcinosis and tubular dysfunctions . J Pediatr 82:202-206, 1973.Crossref 6. Makker SP, Heymann W: Pregnancy in patients who have the idiopathic nephrotic syndrome in childhood . J Pediatr 81:1140-1144, 1972.Crossref 7. Johnson CL, Johnson CR, Reader R: The nephrotic syndrome in pregnancy . Aust Ann Med 12:342-345, 1963. 8. Studd JWW, Blainey JD: Pregnancy and nephrotic syndrome . Br Med J 1:276-280, 1969.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

An Unusual Variation of Familial Nephrosis

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References (8)

Publisher
American Medical Association
Copyright
Copyright © 1979 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1979.02130020108022
Publisher site
See Article on Publisher Site

Abstract

Abstract This is a report of an unusual occurrence of cases of nephrotic syndrome in a family in which a mother, her brother, and her child had the steroidsensitive relapsing form of the disease. The nephrotic syndrome in children is usually idiopathic and rarely familial. Its peak incidence is in children ages 1 to 6 years. It generally follows a relapsing course, but is steroid sensitive and demonstrates minimal glomerular changes on pathologic study. It is very unusual to see a familial pattern to the disease, except in the rare autosomal recessive syndrome or in association with certain systemic metabolic disorders of which the nephrotic syndrome is only one component. Report of Cases.—Case 1.—Nephrosis developed in the index patient at age 8 years. Her clinical course and laboratory values were consistent with the diagnosis of idiopathic nephrotic syndrome of childhood. She has always been steroid sensitive. A needle biopsy References 1. Bader PL, Grove J, Trygstad CW, et al: Familial nephrotic syndrome . Am J Med 56:34-43, 1974.Crossref 2. White RH: The familial nephrotic syndrome: I. A European survey . Clin Nephrol 1:215-219, 1973. 3. Moncrieff MW, White RHR, Glascow EF, et al: The familial nephrotic syndrome: II. A clinicopathological study . Clin Nephrol 1:220-229, 1973. 4. Huttunen NP: Congenital nephrotic syndrome of Finnish type: Study of 75 patients . Arch Dis Child 51:344-348, 1976.Crossref 5. Burke EC, Holley KE, Stickler GB: Familial nephrotic syndrome with nephrocalcinosis and tubular dysfunctions . J Pediatr 82:202-206, 1973.Crossref 6. Makker SP, Heymann W: Pregnancy in patients who have the idiopathic nephrotic syndrome in childhood . J Pediatr 81:1140-1144, 1972.Crossref 7. Johnson CL, Johnson CR, Reader R: The nephrotic syndrome in pregnancy . Aust Ann Med 12:342-345, 1963. 8. Studd JWW, Blainey JD: Pregnancy and nephrotic syndrome . Br Med J 1:276-280, 1969.Crossref

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: Feb 1, 1979

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