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December 1959, Vol 98, No. 6 >
Article | December 1959

Infantile Nephrosis:  Clinical, Biochemical, and Morphologic Studies of the Syndrome FREE

HOWARD G. WORTHEN, M.D.; ROBERT L. VERNIER, M.D.; ROBERT A. GOOD, M.D., Ph.D.
[+] Author Affiliations

Submitted for publication Feb. 25, 1959.

Aided by grants from the American Heart Association, Minnesota Heart Association, U. S. Public Health Service and the Hill Family Foundation.

Research Fellow of the American Heart Association (Dr. Vernier). American Legion Memorial Heart Research Professor of Pediatrics (Dr. Good).


AMA Am J Dis Child. 1959;98(6):731-748. doi:10.1001/archpedi.1959.02070020733009.
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The nephrotic syndrome occurs infrequently prior to the second year of life. When nephrosis does develop during the first year, the course differs from that of older children with nephrosis, being characterized by an extremely poor prognosis and an almost complete refractoriness to therapy. Despite its low incidence, infantile nephrosis is important, not only because of the severity of the disorder itself but also because the occurrence of nephrosis in this age group raises questions regarding the etiology of the disease.

Since 1952, we have studied 12 patients who developed nephrosis during the first year of life. These cases illustrate the characteristic clinical and laboratory features of the disease, the difficulties in management, and the inordinately high mortality rate of infantile nephrosis. The purpose of this paper is to present a clinical and laboratory analysis of these cases, together with morphologic observations obtained by kidney biopsy and autopsy material, studied

REFERENCES

1 +
Farquhar, M. G.; Vernier, R. L., and Good, R. A.:  The Application of Electron Microscopy in Pathology: Study of Renal Biopsy Tissues , Schweiz. med. Wchnschr. 87:501, 1957;.
2 +
Vernier, R. L.; Farquhar, M. G.; Brunson, J. G., and Good, R. A.:  Chronic Renal Disease in Children: Correlation of Clinical Findings with Morphologic Characteristics Seen by Light and Electron Microscopy , A. M. A. J. Dis. Child. 96:306, 1958;.
3 +
Farquhar, M. G.:  Preparation of Ultrathin Tissue Sections for Electron Microscopy , Lab. Invest. 5:317, 1956;.
4 +
Giles, H. M.; Pugh, R. C. B.; Darmady, E. M.; Stranack, F., and Woolf, L. I.:  The Nephrotic Syndrome in Early Infancy: A Report of 3 Cases , Arch. Dis. Childhood 32:167, 1957;.
5 +
Oliver, J.: Architecture of the Kidney in Chronic Bright's Disease , New York, Paul B. Hoeber, Inc., 1939;.
6 +
Vernier, R. L.; Brunson, J. G., and Good, R. A.:  Studies on Familial Nephrosis: I. Clinical and Pathologic Study of 4 Cases in a Single Family , A. M. A. J. Dis. Child. 93:469, 1957;.
7 +
Sandell, E. B.: Colorimetric Determination of Traces of Metal , New York, Interscience Publishers, 1944;, p. 324.
8 +
Farquhar, M. G.; Vernier, R. L., and Good, R. A.:  Studies on Familial Nephrosis: II. Gomerular Changes Observed with the Electron Microscope , Am. J. Path. 33:791, 1957;.
9 +
Spiro, D.:  The Structural Basis of Proteinuria in Man: Electron Microscopic Studies of Renal Biopsy Specimens from Patients with Lipoid Nephrosis, Amyloidosis, and Subacute and Chronic Glomerulonephritis , Am. J. Path. 35:47, 1959;.
10 +
Vernier, R. L.; Worthen, H. G., and Good, R. A.: Unpublished observations.
11 +
Hallman, N.; Hjelt, L., and Ahvenainen, E. K.:  Nephrotic Syndrome in Newborn and Young Infants , Ann. paediat. fenn. 2:227, 1956;.
12 +
Collins, R. D.:  Chronic Glomerulonephritis in a Newborn Child , A. M. A. J. Dis. Child. 87: 478, 1954;.
13 +
Fanconi, G.; Kousmine, C., and Frischknecht, W.:  Die konstitutionelle Bereitschaft zum Nephrosesyndrom , Helvet. paediat. acta. 6:199, 1951;.
14 +
Kunstadter, R. H., and Rosenblum, L.:  Neonatal Glomerulonephritis and the Nephrotic Syndrome in a 1,320-Gm. Prematurely Born Infant , A. M. A. J. Dis. Child. 88:611, 1954;.
15 +
Eiben, R. M.; Kleinerman, J., and Cline, J. C.:  Nephrotic Syndrome in a Neonatal Premature Infant , J. Pediat. 44:195, 1954;.
16 +
Gruskay, F. L., and Turano, A.:  Nephrosis in a Newborn Infant: A Syndrome Difficult to Explain by Existing Theories of Etiology , A. M. A. J. Dis. Child. 94:117, 1957;.
17 +
Riley, C., in Proceedings of the Tenth Conference on the Nephrotic Syndrome, to be published.
18 +
Baxter, J. H., and Goodman, H. C.:  Nephrotoxic Serum Nephritis in Rats: I. Distribution and Specificity of the Antigen Responsible for the Production of Nephrotoxic Antibodies , J. Exper. Med. 104:467, 1956;.
19 +
Mellors, R. C., and Ortega, L. G.:  Analytical Pathology: III. Observations on the Pathogenesis of Glomerulonephritis, Lipid Nephrosis, Periarteritis Nodosa, and Secondary Amyloidosis in Man , Am. J. Path. 32:455, 1956;.
20 +
Lange, K.; Slobody, L., and Strang, R.:  Prolonged Intermittent ACTH and Cortisone Therapy in the Nephrotic Syndrome: Immunologic Basis and Results , Pediatrics 15:156, 1955;.
21 +
Good, R. A., in Host-Parasite Relationships in Living Cells , edited by H. M. Felton, Springfield, Ill., Charles C Thomas, Publisher, 1957;, p. 130.
22 +
Heymann, W., and Lund, H. Z.:  Nephrotic Syndrome in Rats , Pediatrics 7:691, 1951;.
23 +
Bacala, J. C., and Burke, R. J.:  Generalized Cytomegalic Inclusion Disease: Report of Case and Review of Literature , J. Pediat. 43:712, 1953;.

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

References

1 +
Farquhar, M. G.; Vernier, R. L., and Good, R. A.:  The Application of Electron Microscopy in Pathology: Study of Renal Biopsy Tissues , Schweiz. med. Wchnschr. 87:501, 1957;.
2 +
Vernier, R. L.; Farquhar, M. G.; Brunson, J. G., and Good, R. A.:  Chronic Renal Disease in Children: Correlation of Clinical Findings with Morphologic Characteristics Seen by Light and Electron Microscopy , A. M. A. J. Dis. Child. 96:306, 1958;.
3 +
Farquhar, M. G.:  Preparation of Ultrathin Tissue Sections for Electron Microscopy , Lab. Invest. 5:317, 1956;.
4 +
Giles, H. M.; Pugh, R. C. B.; Darmady, E. M.; Stranack, F., and Woolf, L. I.:  The Nephrotic Syndrome in Early Infancy: A Report of 3 Cases , Arch. Dis. Childhood 32:167, 1957;.
5 +
Oliver, J.: Architecture of the Kidney in Chronic Bright's Disease , New York, Paul B. Hoeber, Inc., 1939;.
6 +
Vernier, R. L.; Brunson, J. G., and Good, R. A.:  Studies on Familial Nephrosis: I. Clinical and Pathologic Study of 4 Cases in a Single Family , A. M. A. J. Dis. Child. 93:469, 1957;.
7 +
Sandell, E. B.: Colorimetric Determination of Traces of Metal , New York, Interscience Publishers, 1944;, p. 324.
8 +
Farquhar, M. G.; Vernier, R. L., and Good, R. A.:  Studies on Familial Nephrosis: II. Gomerular Changes Observed with the Electron Microscope , Am. J. Path. 33:791, 1957;.
9 +
Spiro, D.:  The Structural Basis of Proteinuria in Man: Electron Microscopic Studies of Renal Biopsy Specimens from Patients with Lipoid Nephrosis, Amyloidosis, and Subacute and Chronic Glomerulonephritis , Am. J. Path. 35:47, 1959;.
10 +
Vernier, R. L.; Worthen, H. G., and Good, R. A.: Unpublished observations.
11 +
Hallman, N.; Hjelt, L., and Ahvenainen, E. K.:  Nephrotic Syndrome in Newborn and Young Infants , Ann. paediat. fenn. 2:227, 1956;.
12 +
Collins, R. D.:  Chronic Glomerulonephritis in a Newborn Child , A. M. A. J. Dis. Child. 87: 478, 1954;.
13 +
Fanconi, G.; Kousmine, C., and Frischknecht, W.:  Die konstitutionelle Bereitschaft zum Nephrosesyndrom , Helvet. paediat. acta. 6:199, 1951;.
14 +
Kunstadter, R. H., and Rosenblum, L.:  Neonatal Glomerulonephritis and the Nephrotic Syndrome in a 1,320-Gm. Prematurely Born Infant , A. M. A. J. Dis. Child. 88:611, 1954;.
15 +
Eiben, R. M.; Kleinerman, J., and Cline, J. C.:  Nephrotic Syndrome in a Neonatal Premature Infant , J. Pediat. 44:195, 1954;.
16 +
Gruskay, F. L., and Turano, A.:  Nephrosis in a Newborn Infant: A Syndrome Difficult to Explain by Existing Theories of Etiology , A. M. A. J. Dis. Child. 94:117, 1957;.
17 +
Riley, C., in Proceedings of the Tenth Conference on the Nephrotic Syndrome, to be published.
18 +
Baxter, J. H., and Goodman, H. C.:  Nephrotoxic Serum Nephritis in Rats: I. Distribution and Specificity of the Antigen Responsible for the Production of Nephrotoxic Antibodies , J. Exper. Med. 104:467, 1956;.
19 +
Mellors, R. C., and Ortega, L. G.:  Analytical Pathology: III. Observations on the Pathogenesis of Glomerulonephritis, Lipid Nephrosis, Periarteritis Nodosa, and Secondary Amyloidosis in Man , Am. J. Path. 32:455, 1956;.
20 +
Lange, K.; Slobody, L., and Strang, R.:  Prolonged Intermittent ACTH and Cortisone Therapy in the Nephrotic Syndrome: Immunologic Basis and Results , Pediatrics 15:156, 1955;.
21 +
Good, R. A., in Host-Parasite Relationships in Living Cells , edited by H. M. Felton, Springfield, Ill., Charles C Thomas, Publisher, 1957;, p. 130.
22 +
Heymann, W., and Lund, H. Z.:  Nephrotic Syndrome in Rats , Pediatrics 7:691, 1951;.
23 +
Bacala, J. C., and Burke, R. J.:  Generalized Cytomegalic Inclusion Disease: Report of Case and Review of Literature , J. Pediat. 43:712, 1953;.

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