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L. Strauss, R. Platt (1957)
Endocardial sclerosis in infancy associated with abnormal storage (gargoylism); report of a case in an infant, aged five months and review of the literature.Journal of the Mount Sinai Hospital, New York, 24 6
L. Thomas (1956)
REVERSIBLE COLLAPSE OF RABBIT EARS AFTER INTRAVENOUS PAPAIN, AND PREVENTION OF RECOVERY BY CORTISONEThe Journal of Experimental Medicine, 104
G. Hurler (1920)
�ber einen Typ multipler Abartungen, vorwiegend am SkelettsystemZ. Kinderheilk., 24
V. A. McKusick (1960)
Heritable Disorders of Connective Tissue
T. Usui, K. Akaishi, F. Fukuhara, T. Kuroda, T. Kurose, K. Tabata (1961)
[Polysaccharide changes in 2 cases of gargoylism treated with dexamethasone--referred to electron microscopic findings of Reilly's body].Shonika kiyo. Annales paediatrici Japonici, 7
N. Diferrante, C. Rich (1956)
The determination of acid aminopolysaccharide in urine.The Journal of laboratory and clinical medicine, 48 3
Albert Dorfman (1958)
Studies on the biochemistry of connective tissue.Pediatrics, 22 3
T. Usui , F. Fukuhara, T. Kurose, K. Akaishi, T. Kuroda (1961)
Polysaccharide Changes in 2 Cases of Gargoylism Treated with Dexamethasone: Referred to Electron Microscopic Findings of Reilly's BodyAnn. Paediat. Jap., 7
E. A. Davidson , W. Small (1961)
Hormonal Control of Mucopolysaccharide MetabolismFed. Proc., 20
A. Dorfman, S. Schiller (1958)
Effects of hormones on the metabolism of acid mucopolysaccharides of connective tissue.Recent progress in hormone research, 14
Karl Meyer, Philip Hoffman (1961)
Hurler's syndrome.Arthritis and rheumatism, 4
J. Potter (1961)
On papain and flop-eared rabbits.Arthritis and rheumatism, 4
C. Hunter (1917)
A Rare Disease in 2 BrothersProc. Roy. Soc. Med., 10
M. M. Grumbach (1958)
Urinary Excretion and Tissue Storage of Sulfated Mucopolysaccharides in Hurler's SyndromeA.M.A. J. Dis. Child., 96
Horrigan Wd, Baker Dh (1961)
Gargoylism: a review of the roentgen skull changes with a description of a new finding.The American journal of roentgenology, radium therapy, and nuclear medicine, 86
David Brown (1957)
TISSUE STORAGE OF MUCOPOLYSACCHARIDES IN HURLER-PFAUNDLER'S DISEASE.Proceedings of the National Academy of Sciences of the United States of America, 43 9
K. Meyer, M. Grumbach, A. Linker, P. Hoffman (1958)
Excretion of Sulfated Mucopolysaccharides in Gargoylism. (Hurler's Syndrome)∗Proceedings of the Society for Experimental Biology and Medicine, 97
Sara Schiller, Albert Dorfman (1957)
The metabolism of mucopolysaccharides in animals: the effect of cortisone and hydrocortisone on rat skin.Endocrinology, 60 3
R. McCluskey, L. Thomas (1959)
The removal of cartilage matrix in vivo by papain; prevention of recovery with cortisone, hydrocortisone and prednisoione by a direct action on cartilage.The American journal of pathology, 35 4
A. Dorfman (1957)
Occurrence of Urinary Acid Mucopolysaccharides in the Hurler SyndromeProc. Nat. Acad. Sci., 43
John Bryant, Irwin Leder, DeWitt Statten (1958)
The release of chondroitin sulfate from rabbit cartilage following the intravenous injection of crude papain.Archives of biochemistry and biophysics, 76 1
K. Meyer, E. Davidson, A. Linker, P. Hoffman (1956)
The acid mucopolysaccharides of connective tissue.Biochimica et biophysica acta, 21 3
G. Brante (1952)
Gargoylism; a mucopolysaccharidosis.Scandinavian journal of clinical and laboratory investigation, 4 1
K. Meyer, P. Hoffman, A. Linker, M. Grumbach, P. Sampson (1959)
Sulfated Mucopolysaccharides of Urine and Organs in Gargoylism (Hurler's Syndrome) II. Additional Studies.∗Proceedings of the Society for Experimental Biology and Medicine, 102
L. Layton (1951)
Cortisone inhibition of mucopolysaccharide synthesis in the intact rat.Archives of biochemistry and biophysics, 32 1
Abstract Introduction The syndrome referred to as the Hurler or Hunter-Hurler syndrome (lipochondrodystrophy or "gargoylism") was first described as a clinical entity in 1917.1-3 In 1952 Brante 4 identified the "storage substance" in the liver and meninges of three such autopsied individuals to have the characteristics of an acid mucopolysaccharide. He believed this material to be chemically similar or identical to chondroitinsulfuric acid. Dorfman and Lorincz,5 Brown,6 and Meyer et al.7-9 isolated the acid mucopolysaccharide com- ponents from the urine and tissues of individuals with this syndrome. Excessive quantities of chondroitinsulfuric acid-B (B-heparin) and heparitin sulfate were found to be present.In recent years interest has been focused on the relationship of the adrenal corticosteroids to diverse aspects of connective tissue metabolism. Layton10 found that sulfate fixation in the skin of intact rats was inhibited by cortisone administration. Schiller and Dorfman 11,12 reported that the uptake References 1. Hunter, C.: A Rare Disease in 2 Brothers , Proc. Roy. Soc. Med. 10:104, 1917. 2. Hurler, G.: Über einen Typ multipler Abartungen, vorwiegend am Skelettsystem , Z. Kinderheilk. 24:220, 1920.Crossref 3. McKusick, V. A.: Heritable Disorders of Connective Tissue , Ed. 2, St. Louis, C. V. Mosby Company, 1960. 4. Brante, G.: Gargoylism A Mucopolysaccharidosis , Scand. J. Clin. Lab. Invest. 4:43, 1952.Crossref 5. Dorfman, A., and Lorincz, A. E.: Occurrence of Urinary Acid Mucopolysaccharides in the Hurler Syndrome , Proc. Nat. Acad. Sci. 43:443, 1957.Crossref 6. Brown, D. H.: Tissue Storage of Mucopolysaccharides in Hurler-Pfaundler's Disease , Proc. Nat. Acad. Sci. 43:783, 1957.Crossref 7. Meyer, K.; Grumbach, M. M.; Linker, A., and Hoffman, P.: Excretion of Sulfated Mucopolysaccharides in Gargoylism (Hurler's Syndrome) , Proc. Soc. Exp. Biol. Med. 97:275, 1958.Crossref 8. Meyer, K.; Hoffman, P.; Linker, A.; Grumbach, M. M., and Sampson, P.: Sulfated Mucopolysaccharides of Urine and Organs in Gargoylism (Hurler's Syndrome): II. Additional Studies , Proc. Soc. Exp. Biol. Med. 102:587, 1959.Crossref 9. Meyer, K., and Hoffman, P.: Hurler's Syndrome , Arthritis Rheum . 4:552, 1961.Crossref 10. Layton, L. L.: Cortisone Inhibition of Mucopolysaccharide Synthesis in Intact Rat , Arch. Biochem. 32:224, 1951.Crossref 11. Schiller, S., and Dorfman, A.: The Metabolism of Mucopolysaccharides in Animals: The Effect of Cortisone and Hydrocortisone on Rat Skin , Endocrinology 60:376, 1957.Crossref 12. Dorfman, A., and Schiller, S.: Effects of Hormones on the Metabolism of Acid Mucopolysaccharides of Connective Tissue , Recent Progr. Hormone Res. 14:427, 1958. 13. Thomas, L.: Reversible Collapse of Rabbit Ears After Intravenous Papain, and Prevention of Recovery by Cortisone , J. Exp. Med. 104:245, 1956.Crossref 14. Bryant, J. H.; Leder, I. G., and Stetten, D., Jr.: The Release of Chondroitin Sulfate from Rabbit Cartilage Following the Intravenous Injection of Crude Papain , Arch. Biochem 76:122, 1958.Crossref 15. McCluskey, R. T., and Thomas, L.: The Removal of Cartilage Matrix in Vivo by Papain: Prevention of Recovery with Cortisone, Hydrocortisone and Prednisolone by a Direct Action on Cartilage , Amer. J. Path. 35:819, 1959. 16. Potter, J. L.: Current Comments: On Papain and Flop-Eared Rabbits , Arthritis Rheum . 4:389, 1961.Crossref 17. Davidson, E. A.; Small, W., and Perchemlides, P.: Hormonal Control of Mucopolysaccharide Metabolism , Fed. Proc. 20:162, 1961. 18. Grumbach, M. M., and Meyer, K.: Urinary Excretion and Tissue Storage of Sulfated Mucopolysaccharides in Hurler's Syndrome , A.M.A. J. Dis. Child. 96:467, 1958. 19. Usui, T.; Fukuhara, F.; Kurose, T.; Akaishi, K.; Kuroda, T., and Tabata, K.: Polysaccharide Changes in 2 Cases of Gargoylism Treated with Dexamethasone: Referred to Electron Microscopic Findings of Reilly's Body , Ann. Paediat. Jap. 7:78, 1961. 20. DiFerrante, N., and Rich, C.: The Determination of Acid Aminopolysaccharide in Urine , J. Lab. Clin. Med. 48:491, 1956. 21. Dorfman, A.: Studies on the Biochemistry of Connective Tissue , Pediatrics 22:576, 1958. 22. Meyer, K.; Davidson, E. A.; Linker, A., and Hoffman, P.: Acid Mucopolysaccharides of Connective Tissue , Biochim. Biophys. Acta 21:506, 1956.Crossref 23. Horrigan, W. D., and Baker, D. H.: Gargoylism: A Review of the Roentgen Skull Changes with a Description of a New Finding , Amer. J. Roentgenol. 86:473, 1961. 24. Strauss, L., and Platt, R.: Endocardial Sclerosis in Infancy Associated with Abnormal Storage (Gargoylism): Report of a Case in an Infant, Aged 5 Months and Review of the Literature , J. Mount Sinai Hosp. N.Y. 24:1258, 1957.
American Journal of Diseases of Children – American Medical Association
Published: Jul 1, 1963
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