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A Fatal Granulomatous Disease of Childhood: The Clinical, Pathological, and Laboratory Features of a New Syndrome

A Fatal Granulomatous Disease of Childhood: The Clinical, Pathological, and Laboratory Features... Abstract In 1950, a 12-month-old child was referred to the University of Minnesota Hospitals with a syndrome consisting of chronic suppurative lymphadenitis, hepatosplenomegaly, pulmonary infiltrations, and an eczematoid dermatitis about the eyes, nose, and mouth. Since then we have seen three other children with an almost identical clinical picture. This syndrome appears to be a distinct clinical entity not previously described in the medical literature. This syndrome follows a malignant course and at this time has been responsible for the death of all four of these patients. In the management of these patients we have utilized a wide variety of regimens. No satisfactory therapy has been found, however, and the disease has relentlessly progressed through severe debilitation to ultimate death over a period of several years. The clinical syndrome presented by these patients is similar to various disease states that have been previously well characterized. The most striking similarity is with References 1. Fahey, J. L.; Leonard, E.; Churg, J., and Godman, G.: Wegener's Granulomatosis , Am. J. Med. 17:168, 1954.Crossref 2. Godman, G., and Churg, J.: Wegener's Granulomatosis: Pathology and Review of Literature , A. M. A. Arch. Path. 58:533, 1954. 3. Walton, E. W., and Leggat, P. O.: Wegener's Granulomatosis , J. Clin. Path. 9:31, 1956.Crossref 4. Lane, H. C., and Dodd, K.: Idiopathic Lethal Granuloma of the Nose and Face , Pediatrics 16: 461, 1955. 5. Howell, R. G.: Median Lethal Granuloma of the Face Treated by Radiotherapy , Proc. Roy. Soc. Med. 48:447, 1955. 6. Friedmann, I.: The Pathology of Malignant Granuloma of the Nose , J. Laryng. & Otol. 69:331, 1955. 7. McGovern, J. P., and Merritt, D. H.: Sarcoidosis in Childhood , Advances Pediat. 8:97, 1956. 8. Theodos, P. A.; Brieger, H., and Cathcart, R. T.: Chronic Pulmonary Beryllosis , J. A. M. A. 158:1428, 1955. 9. Chesner, C.: Chronic Pulmonary Granulomatosis in Residents Near Beryllium Plant , Ann. Int. Med. 32:1028, 1950. 10. Warwick, W. J., and Good, R. A.: Cat Scratch Disease , in Brennemann's Practice of Pediatrics , edited by I. McQuarrie, Hagerstown, Md., W. F. Prior Company, Inc., 1956, Vol. 3, Ch. 19, pp. 39-49. 11. Kabat, E. A., and Mayer, M. M.: Experimental Immunochemistry , Springfield, Ill., Charles C Thomas, Publisher, 1948. 12. Zak, S. J.: Immunochemical Studies of Human Serum Gamma Globulins , Univ. Minnesota Hosp. Staff Meet. , (April) 26, 1957, p. 17. 13. Uhr, J. W.; Salvin, S. B., and Pappenheimer, A. M., Jr.: Delayed Hypersensitivity: II. Induction of Hypersensitivity in Guinea Pigs by Means of Antigen-Antibody Complexes , J. Exper. Med. 105: 11, 1957. 14. Good, R. A., and Bridges, R. A.: Development of Delayed Hypersensitivity in Agammaglobulinemia, Henry Ford Symposium on the Mechanism of Hypersensitivity, to be published. 15. Martin, S. P.; Pierce, C. H.; Middlebrook, G., and Dubos, R. J.: The Effect of Tubercle Bacilli on the Polymorphonuclear Leukocytes of Normal Animals , J. Exper. Med. 91:381, 1950. 16. Wood, W. B., Jr.: Studies on Cellular Immunology of Acute Bacterial Infections , Harvey Lect. 47:72, 1953. 17. Rebuck, J. W., and Crowley, J. H.: A Method of Studying Leukocytic Functions in Vivo , Ann. New York Acad. Sc. 59:757, 1955. 18. Janeway, C. A.; Craig, J.; Davidson, M.; Downey, W.; Gitlin, D., and Sullivan, J.: Hypergammaglobulinemia Associated with Severe Recurrent and Chronic Nonspecific Infections , A.M.A. Am. J. Dis. Child. 88:388, 1954. 19. Kostman, R.: Hereditary Agranulocytosis , Acta pædiat . 45( (Suppl. 105) ):6, 1956. 20. Landing, B. H., and Shirkey, H. S.: A Syndrome of Recurrent Infection and Infiltration of Viscera by Pigmented Lipid Histiocytes , Pediatrics 20:431, 1957. 21. Dameshek, W.: Acquired Hemolytic Anemia, in Proceedings of the Third International Congress of the International Society of Hematology , Cambridge, England, (Aug.) 21-25, 1951, edited by C. V. Moore, New York, Grune & Stratton, Inc., 1951, p. 270. 22. Chase, M. W.: Further Observations on the Development of Granulomatous Nodules in Tuberculin-Sensitized Guinea Pigs , Rockefeller Inst. Bull. Ann. Report 1956-1957, pp. 72. 23. Voisin, G. A., and Maurer, P.: Studies on the Role of Antibodies in the Failure of Homografts , Ann. New York Acad. Sc. 64:1053, 1957. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png A.M.A. Journal of Diseases of Children American Medical Association

A Fatal Granulomatous Disease of Childhood: The Clinical, Pathological, and Laboratory Features of a New Syndrome

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References (28)

Publisher
American Medical Association
Copyright
Copyright © 1959 American Medical Association. All Rights Reserved.
ISSN
0096-6916
DOI
10.1001/archpedi.1959.02070010389004
Publisher site
See Article on Publisher Site

Abstract

Abstract In 1950, a 12-month-old child was referred to the University of Minnesota Hospitals with a syndrome consisting of chronic suppurative lymphadenitis, hepatosplenomegaly, pulmonary infiltrations, and an eczematoid dermatitis about the eyes, nose, and mouth. Since then we have seen three other children with an almost identical clinical picture. This syndrome appears to be a distinct clinical entity not previously described in the medical literature. This syndrome follows a malignant course and at this time has been responsible for the death of all four of these patients. In the management of these patients we have utilized a wide variety of regimens. No satisfactory therapy has been found, however, and the disease has relentlessly progressed through severe debilitation to ultimate death over a period of several years. The clinical syndrome presented by these patients is similar to various disease states that have been previously well characterized. The most striking similarity is with References 1. Fahey, J. L.; Leonard, E.; Churg, J., and Godman, G.: Wegener's Granulomatosis , Am. J. Med. 17:168, 1954.Crossref 2. Godman, G., and Churg, J.: Wegener's Granulomatosis: Pathology and Review of Literature , A. M. A. Arch. Path. 58:533, 1954. 3. Walton, E. W., and Leggat, P. O.: Wegener's Granulomatosis , J. Clin. Path. 9:31, 1956.Crossref 4. Lane, H. C., and Dodd, K.: Idiopathic Lethal Granuloma of the Nose and Face , Pediatrics 16: 461, 1955. 5. Howell, R. G.: Median Lethal Granuloma of the Face Treated by Radiotherapy , Proc. Roy. Soc. Med. 48:447, 1955. 6. Friedmann, I.: The Pathology of Malignant Granuloma of the Nose , J. Laryng. & Otol. 69:331, 1955. 7. McGovern, J. P., and Merritt, D. H.: Sarcoidosis in Childhood , Advances Pediat. 8:97, 1956. 8. Theodos, P. A.; Brieger, H., and Cathcart, R. T.: Chronic Pulmonary Beryllosis , J. A. M. A. 158:1428, 1955. 9. Chesner, C.: Chronic Pulmonary Granulomatosis in Residents Near Beryllium Plant , Ann. Int. Med. 32:1028, 1950. 10. Warwick, W. J., and Good, R. A.: Cat Scratch Disease , in Brennemann's Practice of Pediatrics , edited by I. McQuarrie, Hagerstown, Md., W. F. Prior Company, Inc., 1956, Vol. 3, Ch. 19, pp. 39-49. 11. Kabat, E. A., and Mayer, M. M.: Experimental Immunochemistry , Springfield, Ill., Charles C Thomas, Publisher, 1948. 12. Zak, S. J.: Immunochemical Studies of Human Serum Gamma Globulins , Univ. Minnesota Hosp. Staff Meet. , (April) 26, 1957, p. 17. 13. Uhr, J. W.; Salvin, S. B., and Pappenheimer, A. M., Jr.: Delayed Hypersensitivity: II. Induction of Hypersensitivity in Guinea Pigs by Means of Antigen-Antibody Complexes , J. Exper. Med. 105: 11, 1957. 14. Good, R. A., and Bridges, R. A.: Development of Delayed Hypersensitivity in Agammaglobulinemia, Henry Ford Symposium on the Mechanism of Hypersensitivity, to be published. 15. Martin, S. P.; Pierce, C. H.; Middlebrook, G., and Dubos, R. J.: The Effect of Tubercle Bacilli on the Polymorphonuclear Leukocytes of Normal Animals , J. Exper. Med. 91:381, 1950. 16. Wood, W. B., Jr.: Studies on Cellular Immunology of Acute Bacterial Infections , Harvey Lect. 47:72, 1953. 17. Rebuck, J. W., and Crowley, J. H.: A Method of Studying Leukocytic Functions in Vivo , Ann. New York Acad. Sc. 59:757, 1955. 18. Janeway, C. A.; Craig, J.; Davidson, M.; Downey, W.; Gitlin, D., and Sullivan, J.: Hypergammaglobulinemia Associated with Severe Recurrent and Chronic Nonspecific Infections , A.M.A. Am. J. Dis. Child. 88:388, 1954. 19. Kostman, R.: Hereditary Agranulocytosis , Acta pædiat . 45( (Suppl. 105) ):6, 1956. 20. Landing, B. H., and Shirkey, H. S.: A Syndrome of Recurrent Infection and Infiltration of Viscera by Pigmented Lipid Histiocytes , Pediatrics 20:431, 1957. 21. Dameshek, W.: Acquired Hemolytic Anemia, in Proceedings of the Third International Congress of the International Society of Hematology , Cambridge, England, (Aug.) 21-25, 1951, edited by C. V. Moore, New York, Grune & Stratton, Inc., 1951, p. 270. 22. Chase, M. W.: Further Observations on the Development of Granulomatous Nodules in Tuberculin-Sensitized Guinea Pigs , Rockefeller Inst. Bull. Ann. Report 1956-1957, pp. 72. 23. Voisin, G. A., and Maurer, P.: Studies on the Role of Antibodies in the Failure of Homografts , Ann. New York Acad. Sc. 64:1053, 1957.

Journal

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

Published: Apr 1, 1959

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