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Studies on Familial Nephrosis: I. Clinical and Pathologic Study of Four Cases in a Single Family

Studies on Familial Nephrosis: I. Clinical and Pathologic Study of Four Cases in a Single Family Abstract The nephrotic syndrome has rarely been observed in more than one member of a family and is not generally considered to be a familial disease. Prader1 and Goettsch,2 however, reported the occurrence of the nephrotic syndrome in twins. Barnett3 has observed nephrosis in two siblings of three families and Werner4 in siblings of two additional families. Recently Fanconi5,6 summarized the experience of his clinic with regard to the familial incidence of the nephrotic syndrome in 17 children of seven families. He concluded that nephrosis may occur as a familial disease and that the disease tends to be severer in older members of the family. He also stated that the prognosis of familial nephrosis seemed to be worse than that usually accorded the sporadic form of nephrosis. This report presents the results of a study of a family in which all four children suffered from the References 1. Prader, A.: Lipoidnephrose bei eineiigen Zwillingen , Helvet. pediat. acta 5:392, 1950. 2. Goettsch, E.: Nephritis and Nephrosis , in Brenneman's Practice of Pediatrics , Vol. 3, Chap. 28, 1948, p. 14. 3. Barnett, H. L.; Forman, C. W., and Lauson, H. D.: The Nephrotic Syndrome in Children , in Advances in Pediatrics , Vol. 5, Edited by S. Z. Levine, Chicago, The Year Book Publishers, Inc., 1952. 4. Werner, M.: Handbuch der Erbbiologie , Berlin, Springer, 1942, p. 886. 5. Fanconi, G.; Kousmine, C., and Frischknecht, W.: Die konstitutionelle Bereitschaft zum Nephrosesyndrom , Helvet. pediat. acta 6:199, 1951. 6. Friderich, J.; Prader, A., and Fanconi, G.: Häufigkeit, Prognose und Behandlung des Nephrosesyndroms , Helvet. pediat. acta 9:109, 1954. 7. McQuarrie, I.: Experiments of Nature , Lawrence, Kan., University of Kansas Press, 1944. 8. Emerson, K., Jr.; Futcher, P. H., and Farr, L. E.: The Relation of High and Low Urea Clearances to the Inulin and Creatinine Clearances in Children with the Nephrotic Syndrome , J. Clin. Invest. 20:361, 1941.Crossref 9. Rubin, M. I.; Bruck, E., and Rapoport, M.: Maturation of Renal Function in Childhood: Clearance Studies , J. Clin. Invest. 28:1144, 1949.Crossref 10. Metcoff, J.; Kelsey, W. M., and Janeway, C. A.: The Nephrotic Syndrome in Children: An Interpretation of Its Clinical, Biochemical, and Renal Hemodynamic Features as Variations of a Single Type of Nephron Disease , J. Clin. Invest. 30:471, 1951.Crossref 11. Randolf, T. G.: Blood Studies in Allergy: I. Direct Counting Chamber Determination of Eosinophiles in Propylene Glycol-Aqueous Stains , J. Allergy 15:89, 1944.Crossref 12. Ely, R.; Raile, R. B.; Bray, P. F., and Kelley, V. C.: Studies of 17-Hydroxycorticosteroids: IV. Evaluation of a Standard ACTH-17-Hydroxycorticosteroid Response Test in Children , Pediatrics 13:403, 1954. 13. McCall, M. F., and Singer, B.: Studies in Nephrosis: Chemical Corticoids, Salt-Retaining Factor, and Effect of ACTH , J. Clin. Endocrinol. 13:1157, 1953.Crossref 14. Farnsworth, E. B.: Studies on Adrenocortical Function in Relation to the Nephrotic Syndrome , J. Clin. Endocrinol. 13:1169, 1953.Crossref 15. Leutscher, J. A., Jr.; Neher, R., and Wettstein, A.: Isolation of Crystalline Aldosterone from the Urine of a Nephrotic Patient , Experientia 10:456, 1954.Crossref 16. Unpublished observations. 17. Kelley, V. C.; Good, R. A., and McQuarrie, I.: Serum Mucoproteins in Children in Health and Disease with Special Reference to Rheumatic Fever , Pediatrics 5:824, 1950. 18. Longsworth, L. G., and MacInnes, D. A.: An Electrophoretic Study of Nephrotic Sera and Urine , J. Exper. Med. 71:77, 1940.Crossref 19. Kelley, V. C.; Good, R. A., and Glick, D.: Mucolytic Enzyme Systems: XI. Hyaluronidase Inhibitor and Serum Mucoproteins in Patients with Lipoid Nephrosis and Acute Glomerulonephritis , J. Clin. Invest. 29:1500, 1950.Crossref 20. Rytand, D. A., and Randall, E.: Titers of Heterophile Antibodies and Antistreptolysin "O" in Acute Glomerular Nephritis and in the Nephrotic Syndrome , Am. J. Med. 8:524, 1950.Crossref 21. Rytand, D. A.; Rantz, L. A., and Randall, E.: Antistreptolysin "O" in Urine and Serum of Patients with the Nephrotic Syndrome, Proceedings of the Society for Clinical Investigation , J. Clin. Invest. 29:843, 1950. 22. Earle, D. P., Jr., and others: The Serum Antistreptolysin Titer in Chronic Glomerulonephritis , J. Clin. Invest. 21:483, 1942.Crossref 23. Allen, A. C.: Clinicopathologic Meaning of the Nephrotic Syndrome , Am. J. Med. 18:277, 1955.Crossref 24. Heymann, W., and Lund, H. Z.: Nephrotic Syndrome in Rats , Pediatrics 7:691, 1951. 25. Seegal, B. C.; Hasson, M. W.; Gaynor, E. C., and Rothenberg, M. S.: Glomerulonephritis Produced in Dogs by Specific Antisera: I. The Course of the Disease Resulting from Injection of Rabbit Antidog-Placenta Serum or Rabbit Antidog-Kidney Serum , J. Exper. Med. 102:789, 1955.Crossref 26. Bevans, M.; Seegal, B. C., and Kaplan, R.: Glomerulonephritis Produced in Dogs by Specific Antisera: II. Pathologic Sequences Following the Injection of Rabbit Antidog-Placenta Serum or Rabbit Antidog-Kidney Serum , J. Exper. Med. 102:807, 1955.Crossref 27. Coons, A. H.; Leduc, E. H., and Connolly, J. M.: Studies on Antibody Production: I. A Method for the Histochemical Demonstration of Specific Antibody and Its Application to a Study of the Hyperimmune Rabbit , J. Exper. Med. 102: 49, 1955.Crossref 28. Muehrcke, R. C.; Kark, R. M., and Pirani, C. L.: Biopsy of the Kidney in the Diagnosis and Management of Renal Disease , New England J. Med. 253:537, 1955.Crossref 29. Rennie, J. B.: The Oedematous Syndrome of Nephritis with Special Reference to Prognosis , Quart. J. Med. 16:21, 1947. 30. Heymann, W., and Alperin, L. J.: Lipemic Nephrosis With and Without Nephritis , Am. Pract. 3:680, 1949. 31. Bell, E. T.: Renal Diseases , Ed. 6, Philadelphia, Lea & Febiger, 1950. 32. Heymann, W., and Startzman, V.: Lipemic Nephrosis , J. Pediat. 28:117, 1946.Crossref 33. Block, W. M.; Jackson, R. L.; Stearns, G., and Butsch, M. P.: Lipoid Nephrosis: Clinical and Biochemical Studies of 40 Children with 10 Necropsies , Pediatrics 1:733, 1948. 34. Murphy, F. D.; Warfield, L. M.; Grill, J., and Annis, E. R.: Lipoid Nephrosis: A Study of 9 patients, with Special Reference to Those Observed over a Long Period , Arch. Int. Med. 62: 355, 1938.Crossref 35. Pantin, C. G.: A Study of the Relation Between Nephritis and Nephrosis , Guy's Hosp. Rep. 18:456, 1938. 36. Dunn, J. S.: Nephrosis or Nephritis? J. Path. & Bact. 39:1, 1934. 37. Farquhar, M.; Vernier, R. L., and Good, R. A.: Studies of Familial Nephrosis: II. The Renal Lesion as Observed with the Electron Microscope, Am. J. Path., to be published. 38. Unpublished observations. 39. Fanconi, G.: Tubular Insufficiency and Renal Dwarfism , Arch. Dis. Childhood 29:1, 1954. 40. Babson, S. G.: Renal Glycosuria , J. Pediat. 17:781, 1940. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png A.M.A. Journal of Diseases of Children American Medical Association

Studies on Familial Nephrosis: I. Clinical and Pathologic Study of Four Cases in a Single Family

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Publisher
American Medical Association
Copyright
Copyright © 1957 American Medical Association. All Rights Reserved.
ISSN
0096-6916
DOI
10.1001/archpedi.1957.02060040471001
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Abstract

Abstract The nephrotic syndrome has rarely been observed in more than one member of a family and is not generally considered to be a familial disease. Prader1 and Goettsch,2 however, reported the occurrence of the nephrotic syndrome in twins. Barnett3 has observed nephrosis in two siblings of three families and Werner4 in siblings of two additional families. Recently Fanconi5,6 summarized the experience of his clinic with regard to the familial incidence of the nephrotic syndrome in 17 children of seven families. He concluded that nephrosis may occur as a familial disease and that the disease tends to be severer in older members of the family. He also stated that the prognosis of familial nephrosis seemed to be worse than that usually accorded the sporadic form of nephrosis. This report presents the results of a study of a family in which all four children suffered from the References 1. Prader, A.: Lipoidnephrose bei eineiigen Zwillingen , Helvet. pediat. acta 5:392, 1950. 2. Goettsch, E.: Nephritis and Nephrosis , in Brenneman's Practice of Pediatrics , Vol. 3, Chap. 28, 1948, p. 14. 3. Barnett, H. L.; Forman, C. W., and Lauson, H. D.: The Nephrotic Syndrome in Children , in Advances in Pediatrics , Vol. 5, Edited by S. Z. Levine, Chicago, The Year Book Publishers, Inc., 1952. 4. Werner, M.: Handbuch der Erbbiologie , Berlin, Springer, 1942, p. 886. 5. Fanconi, G.; Kousmine, C., and Frischknecht, W.: Die konstitutionelle Bereitschaft zum Nephrosesyndrom , Helvet. pediat. acta 6:199, 1951. 6. Friderich, J.; Prader, A., and Fanconi, G.: Häufigkeit, Prognose und Behandlung des Nephrosesyndroms , Helvet. pediat. acta 9:109, 1954. 7. McQuarrie, I.: Experiments of Nature , Lawrence, Kan., University of Kansas Press, 1944. 8. Emerson, K., Jr.; Futcher, P. H., and Farr, L. E.: The Relation of High and Low Urea Clearances to the Inulin and Creatinine Clearances in Children with the Nephrotic Syndrome , J. Clin. Invest. 20:361, 1941.Crossref 9. Rubin, M. I.; Bruck, E., and Rapoport, M.: Maturation of Renal Function in Childhood: Clearance Studies , J. Clin. Invest. 28:1144, 1949.Crossref 10. Metcoff, J.; Kelsey, W. M., and Janeway, C. A.: The Nephrotic Syndrome in Children: An Interpretation of Its Clinical, Biochemical, and Renal Hemodynamic Features as Variations of a Single Type of Nephron Disease , J. Clin. Invest. 30:471, 1951.Crossref 11. Randolf, T. G.: Blood Studies in Allergy: I. Direct Counting Chamber Determination of Eosinophiles in Propylene Glycol-Aqueous Stains , J. Allergy 15:89, 1944.Crossref 12. Ely, R.; Raile, R. B.; Bray, P. F., and Kelley, V. C.: Studies of 17-Hydroxycorticosteroids: IV. Evaluation of a Standard ACTH-17-Hydroxycorticosteroid Response Test in Children , Pediatrics 13:403, 1954. 13. McCall, M. F., and Singer, B.: Studies in Nephrosis: Chemical Corticoids, Salt-Retaining Factor, and Effect of ACTH , J. Clin. Endocrinol. 13:1157, 1953.Crossref 14. Farnsworth, E. B.: Studies on Adrenocortical Function in Relation to the Nephrotic Syndrome , J. Clin. Endocrinol. 13:1169, 1953.Crossref 15. Leutscher, J. A., Jr.; Neher, R., and Wettstein, A.: Isolation of Crystalline Aldosterone from the Urine of a Nephrotic Patient , Experientia 10:456, 1954.Crossref 16. Unpublished observations. 17. Kelley, V. C.; Good, R. A., and McQuarrie, I.: Serum Mucoproteins in Children in Health and Disease with Special Reference to Rheumatic Fever , Pediatrics 5:824, 1950. 18. Longsworth, L. G., and MacInnes, D. A.: An Electrophoretic Study of Nephrotic Sera and Urine , J. Exper. Med. 71:77, 1940.Crossref 19. Kelley, V. C.; Good, R. A., and Glick, D.: Mucolytic Enzyme Systems: XI. Hyaluronidase Inhibitor and Serum Mucoproteins in Patients with Lipoid Nephrosis and Acute Glomerulonephritis , J. Clin. Invest. 29:1500, 1950.Crossref 20. Rytand, D. A., and Randall, E.: Titers of Heterophile Antibodies and Antistreptolysin "O" in Acute Glomerular Nephritis and in the Nephrotic Syndrome , Am. J. Med. 8:524, 1950.Crossref 21. Rytand, D. A.; Rantz, L. A., and Randall, E.: Antistreptolysin "O" in Urine and Serum of Patients with the Nephrotic Syndrome, Proceedings of the Society for Clinical Investigation , J. Clin. Invest. 29:843, 1950. 22. Earle, D. P., Jr., and others: The Serum Antistreptolysin Titer in Chronic Glomerulonephritis , J. Clin. Invest. 21:483, 1942.Crossref 23. Allen, A. C.: Clinicopathologic Meaning of the Nephrotic Syndrome , Am. J. Med. 18:277, 1955.Crossref 24. Heymann, W., and Lund, H. Z.: Nephrotic Syndrome in Rats , Pediatrics 7:691, 1951. 25. Seegal, B. C.; Hasson, M. W.; Gaynor, E. C., and Rothenberg, M. S.: Glomerulonephritis Produced in Dogs by Specific Antisera: I. The Course of the Disease Resulting from Injection of Rabbit Antidog-Placenta Serum or Rabbit Antidog-Kidney Serum , J. Exper. Med. 102:789, 1955.Crossref 26. Bevans, M.; Seegal, B. C., and Kaplan, R.: Glomerulonephritis Produced in Dogs by Specific Antisera: II. Pathologic Sequences Following the Injection of Rabbit Antidog-Placenta Serum or Rabbit Antidog-Kidney Serum , J. Exper. Med. 102:807, 1955.Crossref 27. Coons, A. H.; Leduc, E. H., and Connolly, J. M.: Studies on Antibody Production: I. A Method for the Histochemical Demonstration of Specific Antibody and Its Application to a Study of the Hyperimmune Rabbit , J. Exper. Med. 102: 49, 1955.Crossref 28. Muehrcke, R. C.; Kark, R. M., and Pirani, C. L.: Biopsy of the Kidney in the Diagnosis and Management of Renal Disease , New England J. Med. 253:537, 1955.Crossref 29. Rennie, J. B.: The Oedematous Syndrome of Nephritis with Special Reference to Prognosis , Quart. J. Med. 16:21, 1947. 30. Heymann, W., and Alperin, L. J.: Lipemic Nephrosis With and Without Nephritis , Am. Pract. 3:680, 1949. 31. Bell, E. T.: Renal Diseases , Ed. 6, Philadelphia, Lea & Febiger, 1950. 32. Heymann, W., and Startzman, V.: Lipemic Nephrosis , J. Pediat. 28:117, 1946.Crossref 33. Block, W. M.; Jackson, R. L.; Stearns, G., and Butsch, M. P.: Lipoid Nephrosis: Clinical and Biochemical Studies of 40 Children with 10 Necropsies , Pediatrics 1:733, 1948. 34. Murphy, F. D.; Warfield, L. M.; Grill, J., and Annis, E. R.: Lipoid Nephrosis: A Study of 9 patients, with Special Reference to Those Observed over a Long Period , Arch. Int. Med. 62: 355, 1938.Crossref 35. Pantin, C. G.: A Study of the Relation Between Nephritis and Nephrosis , Guy's Hosp. Rep. 18:456, 1938. 36. Dunn, J. S.: Nephrosis or Nephritis? J. Path. & Bact. 39:1, 1934. 37. Farquhar, M.; Vernier, R. L., and Good, R. A.: Studies of Familial Nephrosis: II. The Renal Lesion as Observed with the Electron Microscope, Am. J. Path., to be published. 38. Unpublished observations. 39. Fanconi, G.: Tubular Insufficiency and Renal Dwarfism , Arch. Dis. Childhood 29:1, 1954. 40. Babson, S. G.: Renal Glycosuria , J. Pediat. 17:781, 1940.

Journal

A.M.A. Journal of Diseases of ChildrenAmerican Medical Association

Published: May 1, 1957

References