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ENDOCARDIAL FIBROELASTOSIS: Report of Unusual Case with Impaired Ability to Fabricate Serum Proteins

ENDOCARDIAL FIBROELASTOSIS: Report of Unusual Case with Impaired Ability to Fabricate Serum Proteins Abstract ENDOCARDIAL fibroelastosis or endocardial sclerosis until recently has been looked upon as a pathologic diagnosis.1 With the growing interest in this condition, attempts have been made to define this as a clinical entity and one which in many cases can be diagnosed ante mortem.2 The postmortem findings of endocardial fibroelastosis have been described in infants from 3 days to 5 years of age; the majority of the deaths have been of infants between 4 and 9 months. Generally speaking, three clinical patterns have been noted, characterized best by their course: (1) sudden death occurring 24 to 48 hours after the onset of symptoms of respiratory distress; (2) prolonged progressive increase in dyspnea, anorexia, irritability, occasional vomiting, cardiac failure, and death; (3) repeated episodes of respiratory distress and cardiac failure which respond well to oxygen and digitalis and which may last months or even years. The purpose of this References 1. Pototschnig, G.: Über die kongenitale diffuse Endokardhyperplasia des linken Ventrikels , Ztschr. ang. Anat. 4:234, 1918. 2. Gross, P.: Concept of Fetal Endocarditis: General Review with Report of Illustrative Case , Arch. Path. 31:163, 1941. 3. Sano, M. E., and Anderson, N. A.: Elastic Tissue Hyperplasia of Endocardium , Arch. Path. 33:533, 1942. 4. Weinberg, T., and Himmelfarb, A. J.: Endocardial Fibroelastosis (So-Called Fetal Endocarditis); Report of 2 Cases Occurring in Siblings , Bull. Johns Hopkins Hosp. 72:299, 1943. 5. Cosgrove, G. E., Jr., and Kaump, D. H.: Endocardial Sclerosis in Infants and Children , Am. J. Clin. Path. 16:322, 1946. 6. Craig, J. M.: Congenital Endocardial Sclerosis , J. Tech. Methods 30: 15, 1949. 7. Mahon, G. S.: Idiopathic Hypertrophy of the Heart with Endocardial Fibrosis: Report of 2 Cases , Am. Heart J. 12:608, 1936.Crossref 8. Collier, F. C., and Rosahn, P. D.: Endocardial Fibroelastosis , Pediatrics 7:175, 1951. 9. Edmonds, H. W., and Seelye, W. B.: Endocardial Sclerosis: Review of Changing Concepts with Report of 6 Cases , Pediatrics 7:651, 1951. 10. Hill, W. T., and Reilly, W. A.: Endocardial Fibroelastosis , A. M. A. Am. J. Dis. Child. 82:579, 1951. 11. Adams, F. H., and Katz, B.: Endocardial Fibroelastosis: Case Reports with Special Emphasis on the Clinical Findings , J. Pediat. 41:141, 1952.Crossref 12. Total protein was determined by the Biuret method. The globulin was precipitated out, 26% sodium sulfate being used. 13. Platt, B. S., and Lu, G. D.: Studies on Metabolism of Pyruvic Acid in Normal and Vitamin B1-Deficient States: Accumulation of Pyruvic Acid and Other Carbonyl Compounds in Beri-Beri and Effect of Vitamin Bi , Biochem. J. 33:1525, 1939. 14. Friedemann, T. E., and Haugen, G. E.: Pyruvic Acid: Determination of Keto Acids in Blood and Urine , J. Biol. Chem. 147:415, 1943. 15. Footnotes 1 b, c, d, e, f, g, h, i, j, and 2: 16. Adams and Katz.2 17. Lewis, K. C.: Cardiac Enlargement of Unknown Etiology in Infancy and Childhood , J. Pediat. 39:698, 1951.Crossref 18. McQuarrie, I.: Impaired Ability to Fabricate Serum Proteins the Chief Cause of Edema in Chronic Constrictive Pericarditis , Journal-Lancet 62:199,, 1942. 19. Stadler, H., and Stinger, D. I.: Case of Pick's Syndrome as Basis for Study of Hypoproteinemia , J. Pediat. 18:84, 1941.Crossref 20. Sherlock, S.: The Liver in Heart Failure: Relation of Anatomical, Functional and Circulatory Changes , Brit. Heart J. 13:273, 1951.Crossref 21. Westerfeld, W. W.; Stotz, E., and Berg, R. L.: Role of Pyruvate in Metabolism of Ethyl Alcohol , J. Biochem. 144:657, 1942. 22. Stotz, E.: Pyruvates , Advances in Enzymology 5:129, 1945. 23. Michel, H. O.: Electrometric Method for the Determination of Red Blood Cell and Plasma Cholinesterase Activity , J. Lab. & Clin. Med. 34:1564, 1949. 24. McCance, R. A.: Serum Cholinesterases in Undernutrition , Proc. Royal Soc. Med. 43: 272, 1950. 25. Kunkel, H. G., and Ward, S. M.: Plasma Esterase Activity in Patients with Liver Disease and the Nephrotic Syndrome , J. Exper. Med. 86:325, 1947.Crossref 26. Vorhaus, L. J., II; Scudamore, H. H., and Kark, R. M.: Measurement of Serum Cholinesterase Activity in the Study of Diseases of the Liver and Biliary System , Gastroenterology 15:304, 1950. 27. Davies, J. N. P.: Nutrition and Nutritional Diseases , Annual Review of Medicine, Stanford, Calif. Annual Review Inc., 1952, Vol. 3, p. 99. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png A.M.A. Journal of Diseases of Children American Medical Association

ENDOCARDIAL FIBROELASTOSIS: Report of Unusual Case with Impaired Ability to Fabricate Serum Proteins

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Publisher
American Medical Association
Copyright
Copyright © 1953 American Medical Association. All Rights Reserved.
ISSN
0096-6916
DOI
10.1001/archpedi.1953.02050080195007
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Abstract

Abstract ENDOCARDIAL fibroelastosis or endocardial sclerosis until recently has been looked upon as a pathologic diagnosis.1 With the growing interest in this condition, attempts have been made to define this as a clinical entity and one which in many cases can be diagnosed ante mortem.2 The postmortem findings of endocardial fibroelastosis have been described in infants from 3 days to 5 years of age; the majority of the deaths have been of infants between 4 and 9 months. Generally speaking, three clinical patterns have been noted, characterized best by their course: (1) sudden death occurring 24 to 48 hours after the onset of symptoms of respiratory distress; (2) prolonged progressive increase in dyspnea, anorexia, irritability, occasional vomiting, cardiac failure, and death; (3) repeated episodes of respiratory distress and cardiac failure which respond well to oxygen and digitalis and which may last months or even years. The purpose of this References 1. Pototschnig, G.: Über die kongenitale diffuse Endokardhyperplasia des linken Ventrikels , Ztschr. ang. Anat. 4:234, 1918. 2. Gross, P.: Concept of Fetal Endocarditis: General Review with Report of Illustrative Case , Arch. Path. 31:163, 1941. 3. Sano, M. E., and Anderson, N. A.: Elastic Tissue Hyperplasia of Endocardium , Arch. Path. 33:533, 1942. 4. Weinberg, T., and Himmelfarb, A. J.: Endocardial Fibroelastosis (So-Called Fetal Endocarditis); Report of 2 Cases Occurring in Siblings , Bull. Johns Hopkins Hosp. 72:299, 1943. 5. Cosgrove, G. E., Jr., and Kaump, D. H.: Endocardial Sclerosis in Infants and Children , Am. J. Clin. Path. 16:322, 1946. 6. Craig, J. M.: Congenital Endocardial Sclerosis , J. Tech. Methods 30: 15, 1949. 7. Mahon, G. S.: Idiopathic Hypertrophy of the Heart with Endocardial Fibrosis: Report of 2 Cases , Am. Heart J. 12:608, 1936.Crossref 8. Collier, F. C., and Rosahn, P. D.: Endocardial Fibroelastosis , Pediatrics 7:175, 1951. 9. Edmonds, H. W., and Seelye, W. B.: Endocardial Sclerosis: Review of Changing Concepts with Report of 6 Cases , Pediatrics 7:651, 1951. 10. Hill, W. T., and Reilly, W. A.: Endocardial Fibroelastosis , A. M. A. Am. J. Dis. Child. 82:579, 1951. 11. Adams, F. H., and Katz, B.: Endocardial Fibroelastosis: Case Reports with Special Emphasis on the Clinical Findings , J. Pediat. 41:141, 1952.Crossref 12. Total protein was determined by the Biuret method. The globulin was precipitated out, 26% sodium sulfate being used. 13. Platt, B. S., and Lu, G. D.: Studies on Metabolism of Pyruvic Acid in Normal and Vitamin B1-Deficient States: Accumulation of Pyruvic Acid and Other Carbonyl Compounds in Beri-Beri and Effect of Vitamin Bi , Biochem. J. 33:1525, 1939. 14. Friedemann, T. E., and Haugen, G. E.: Pyruvic Acid: Determination of Keto Acids in Blood and Urine , J. Biol. Chem. 147:415, 1943. 15. Footnotes 1 b, c, d, e, f, g, h, i, j, and 2: 16. Adams and Katz.2 17. Lewis, K. C.: Cardiac Enlargement of Unknown Etiology in Infancy and Childhood , J. Pediat. 39:698, 1951.Crossref 18. McQuarrie, I.: Impaired Ability to Fabricate Serum Proteins the Chief Cause of Edema in Chronic Constrictive Pericarditis , Journal-Lancet 62:199,, 1942. 19. Stadler, H., and Stinger, D. I.: Case of Pick's Syndrome as Basis for Study of Hypoproteinemia , J. Pediat. 18:84, 1941.Crossref 20. Sherlock, S.: The Liver in Heart Failure: Relation of Anatomical, Functional and Circulatory Changes , Brit. Heart J. 13:273, 1951.Crossref 21. Westerfeld, W. W.; Stotz, E., and Berg, R. L.: Role of Pyruvate in Metabolism of Ethyl Alcohol , J. Biochem. 144:657, 1942. 22. Stotz, E.: Pyruvates , Advances in Enzymology 5:129, 1945. 23. Michel, H. O.: Electrometric Method for the Determination of Red Blood Cell and Plasma Cholinesterase Activity , J. Lab. & Clin. Med. 34:1564, 1949. 24. McCance, R. A.: Serum Cholinesterases in Undernutrition , Proc. Royal Soc. Med. 43: 272, 1950. 25. Kunkel, H. G., and Ward, S. M.: Plasma Esterase Activity in Patients with Liver Disease and the Nephrotic Syndrome , J. Exper. Med. 86:325, 1947.Crossref 26. Vorhaus, L. J., II; Scudamore, H. H., and Kark, R. M.: Measurement of Serum Cholinesterase Activity in the Study of Diseases of the Liver and Biliary System , Gastroenterology 15:304, 1950. 27. Davies, J. N. P.: Nutrition and Nutritional Diseases , Annual Review of Medicine, Stanford, Calif. Annual Review Inc., 1952, Vol. 3, p. 99.

Journal

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

Published: Aug 1, 1953

References