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Aldrich's Syndrome (Thrombocytopenia, Eczema, and Infection in Infants): Studies of the Defense Mechanisms

Aldrich's Syndrome (Thrombocytopenia, Eczema, and Infection in Infants): Studies of the Defense... Abstract Aldrich, Steinberg, and Campbell,1 in 1954, described a sex-linked recessive condition in infants which was characterized by draining ears, eczematoid dermatitis, and bloody diarrhea. Huntely and Dees,2 and Wolff and Bertucio3 reported 11 additional infants and children who have had the unusual triad of severe eczema, thrombocytopenia, and early death resulting from overwhelming infection. This disease appeared to be a definite entity which, although uncommon, is not extremely rare. Because of the distressing morbidity and unfavorable prognosis despite all current therapy and because the etiology of this interesting syndrome remains an enigma, continued investigation of this disease was considered necessary. It is the purpose of the present paper to report seven additional instances of this syndrome in which extensive morphological, immunological, hematological, endocrinological, and clinical investigations were completed. Report of Cases Case 1.—This child was admitted to the University of Minnesota Hospitals at the age of 3 References 1. Aldrich, R. A.; Steinberg, A. G., and Campbell, D. C.: Pedigree Demonstrating a Sex-Linked Recessive Condition Characterized by Draining Ears, Eczematoid Dermatitis, and Bloody Diarrhea , Pediatrics 13:133-139, 1954. 2. Huntely, C. C., and Dees, S. C.: Eczema Associated with Thrombocytopenia Purpura and Purulent Otitis Media , Pediatrics 19:351-361, 1957. 3. Wolff, J. A., and Bertucio, M.: A Sex-Linked Genetic Syndrome in a Negro Family Manifested by Thrombocytopenia, Eczema, Bloody Diarrhea, Recurrent Infection, Anemia, and Epistaxis , A. M. A. J. Dis. Child. 93:74, 1957. 4. Wood, W. B.: Intercellular Surface Phagotosis , Science 106:86-87, 1947.Crossref 5. Martin, S. P.; Pierce, C. H.; Middlebrook, G., and Dubos, R. J.: The Effect of Tubercule Bacilli in the Polymorphonuclear Leucocytes of Normal Anemia , J. Exper. Med. 91:381, 1950.Crossref 6. Rebuck, J. W.: Cytology of Acute Inflammation in Man as Demonstrated by Two Original Technical Procedures, with Particular Reference to the Role of Lymphocytes, Ph.D. Thesis, University of Minnesota, 1947. 7. Giedion, V. A., and Scheidegger, J. J.: Kongenitale Immunoparese die Fehlen Specifischer β2 Globuline und Quantitiv Normeden Gamma Globulin , Helvet. paediat. acta 12:241-259, 1957. 8. Page, A. R., and Good, R. A.: Studies on Cyclic Neutropenia , A. M. A. J. Dis. Child 94: 623-661, 1957.Crossref 9. Hitzig, W. H.: Die Physiologische Entiwichlung der Immunoglobuline (Gamma- und Bete2-Globulin) , Helvet. paediat. acta 12:596-605, 1957. 10. Good, Thomas, in Proceedings of Western Society for Pediatric Research, 1957. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png A.M.A. Journal of Diseases of Children American Medical Association

Aldrich's Syndrome (Thrombocytopenia, Eczema, and Infection in Infants): Studies of the Defense Mechanisms

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References (9)

Publisher
American Medical Association
Copyright
Copyright © 1959 American Medical Association. All Rights Reserved.
ISSN
0096-6916
DOI
10.1001/archpedi.1959.02070010139001
Publisher site
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Abstract

Abstract Aldrich, Steinberg, and Campbell,1 in 1954, described a sex-linked recessive condition in infants which was characterized by draining ears, eczematoid dermatitis, and bloody diarrhea. Huntely and Dees,2 and Wolff and Bertucio3 reported 11 additional infants and children who have had the unusual triad of severe eczema, thrombocytopenia, and early death resulting from overwhelming infection. This disease appeared to be a definite entity which, although uncommon, is not extremely rare. Because of the distressing morbidity and unfavorable prognosis despite all current therapy and because the etiology of this interesting syndrome remains an enigma, continued investigation of this disease was considered necessary. It is the purpose of the present paper to report seven additional instances of this syndrome in which extensive morphological, immunological, hematological, endocrinological, and clinical investigations were completed. Report of Cases Case 1.—This child was admitted to the University of Minnesota Hospitals at the age of 3 References 1. Aldrich, R. A.; Steinberg, A. G., and Campbell, D. C.: Pedigree Demonstrating a Sex-Linked Recessive Condition Characterized by Draining Ears, Eczematoid Dermatitis, and Bloody Diarrhea , Pediatrics 13:133-139, 1954. 2. Huntely, C. C., and Dees, S. C.: Eczema Associated with Thrombocytopenia Purpura and Purulent Otitis Media , Pediatrics 19:351-361, 1957. 3. Wolff, J. A., and Bertucio, M.: A Sex-Linked Genetic Syndrome in a Negro Family Manifested by Thrombocytopenia, Eczema, Bloody Diarrhea, Recurrent Infection, Anemia, and Epistaxis , A. M. A. J. Dis. Child. 93:74, 1957. 4. Wood, W. B.: Intercellular Surface Phagotosis , Science 106:86-87, 1947.Crossref 5. Martin, S. P.; Pierce, C. H.; Middlebrook, G., and Dubos, R. J.: The Effect of Tubercule Bacilli in the Polymorphonuclear Leucocytes of Normal Anemia , J. Exper. Med. 91:381, 1950.Crossref 6. Rebuck, J. W.: Cytology of Acute Inflammation in Man as Demonstrated by Two Original Technical Procedures, with Particular Reference to the Role of Lymphocytes, Ph.D. Thesis, University of Minnesota, 1947. 7. Giedion, V. A., and Scheidegger, J. J.: Kongenitale Immunoparese die Fehlen Specifischer β2 Globuline und Quantitiv Normeden Gamma Globulin , Helvet. paediat. acta 12:241-259, 1957. 8. Page, A. R., and Good, R. A.: Studies on Cyclic Neutropenia , A. M. A. J. Dis. Child 94: 623-661, 1957.Crossref 9. Hitzig, W. H.: Die Physiologische Entiwichlung der Immunoglobuline (Gamma- und Bete2-Globulin) , Helvet. paediat. acta 12:596-605, 1957. 10. Good, Thomas, in Proceedings of Western Society for Pediatric Research, 1957.

Journal

A.M.A. Journal of Diseases of Children – American Medical Association

Published: Feb 1, 1959

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