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CYSTINE NEPHROLITHIASIS FREE

GEORGE H. EWELL, M.D.
[+] Author Affiliations

Read before the Section on Urology at the Eighty-Third Annual Session of the American Medical Association, New Orleans, May 11, 1932.


MADISON, WIS.

From the Section of Urology, the Jackson Clinic.


JAMA. 1932;99(26):2160-2166. doi:10.1001/jama.1932.02740780012004
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Cystine nephrolithiasis is a comparatively rare and exceedingly interesting pathologic finding. It probably occurs more often than is reported, as it is not at all unreasonable to suppose that some of the cases of recurrent renal calculi are of this type, as well as single instances of passed or surgically removed calculi not subjected to chemical analysis.

Simon,1 in 1900, collected from the literature 103 cases of cystine calculi. In 1912, Link2 collected 146 cases. In 1925, Seeger and Kearns3 noted 181 reported cases of cystinuria, 124 of which were complicated with calculus formation. Lewis4 found seventy-one reported cases of cystinuria from 1921 to 1932, although calculus formation was not observed in all of these. Henline,5 in 1930, reported three cases of cystine nephrolithiasis. About 70 per cent of the reported cases have been in males. The disease is quite widespread, cases having been reported

REFERENCES

Simon, C. E.:  Cystinuria and Its Relation to Diaminuria , Am. J. M. Sc. 119: 39, 1900;.
Link, R. E. A.: Beitrag zur Kenntnis der Cystinurie und der Cystinsteine , Leipzig, Sturm & Koppe, 1912;.
Seeger, S. J., and Kearns, W. M.:  Cystinuric Lithiasis , J. A. M. A. 85:4 ( (July 4) ) 1925;.
Lewis, H. B.:  Cystinuria: A Review of Some Recent Investigations , Yale J. Biol. & Med. 4:437 ( (March) ) 1932;.
Henline, R. B.:  Cystine Calculi: Report of Three Cases , Am. J. Surg. 8:581 ( (March) ) 1930;.
Wollaston, W. H.:  On Cystic Oxide: A New Species of Urinary Calculus , Phil. Tr. , London, 1810;, p. 223.
Prout, W.: On the Nature and Treatment of Stomach and Urinary Diseases , ed. 3, London, J. & A. Churchill, 1840;.
Lassaigne, J. L.:  Observation sur l'existence de l'oxide cystique dans un calcul vesical du chien, et essai analytique sur la composition élémentaire de cette substance particulière , Ann. de chim. et phys. 23: 328, 1823;.
Ward, F. W.:  The Absorption Spectra of Some Indole Derivatives , Biochem. J. 17:891, 1923;.
Lewis, H. B., and Lough, S. A.:  Metabolism of Sulphur: Metabolic Study of a Case of Cystinuria , J. Biol. Chem. 81:285 ( (Feb.) ) 1929;.
Robson, W.:  Protein Metabolism in Cystinuria , Biochem. J. 23: 138, 1929;.
Alsberg, C., and Folin, O.:  Protein Metabolism in Cystinuria , Am. J. Physiol. 14: 54, 1905;.
Cox, G. J.; Smythe, C. V., and Fishback, C. F.:  The Nephropathogenic Action of Cystine , J. Biol. Chem. 82:95 ( (April) ) 1929;.
Lignac, G. O. E.:  Nephrosis and Nephritis Caused by Cystine , Nederl. tijdschr. v. geneesk. 2:2187 ( (Nov. 4) ) 1925;
abstr., J. A. M. A. 86:318 ( (Jan. 23) ) 1926;.
Curtis, A. C., and Newburgh, L. H.:  The Toxic Action of Cystine on the Kidney , Arch. Int. Med. 39:817 ( (June) ) 1927;.
Cox, G. J., and Hudson, Leona:  The Nephropathogenic Action of Cystine and the Dietary Control of Cystine Nephrosis , J. Nutrition 2:271 ( (Jan.) ) 1930;.
Tennant, C. E.:  Cystine Calculi: A Complex Surgical Problem , J. A. M. A. 80:305 ( (Feb. 3) ) 1923;.
Mörner, C. T.:  Cystinuria in Sweden , Upsala läkaref. förh. 31: 171 ( (Sept.) ) 1926;.
Toel, F.:  Beobachtungen über Cystinbildung , Ann. d. Pharm. 96:247, 1855;.
Abderhalden, Emil:  Familiare Cystindiathese , Ztschr. f. physiol. Chem. 38: 557, 1903;.
Kretschmer, H. L.:  Cystinuria and Cystine Stones, with a Report of a New Family of Cystinurics , Urol. & Cutan. Rev. 20: 1, 1916;.
Thin, Robert:  Familial Cystinuria , Edinburgh M. J. 36:490 ( (Aug.) ) 1929;.
Blum, L.:  Ueber das Schicksal des Cystins in Tierkorper , Beitr. z. chem. Phys. u. Path. 5: 1, 1903;.
Moreigne, H.:  Étude sur la cystinurie , Arch. de méd. expér. et d'anat. path. 11:254, 1899;.
Wasserthal:  Beitrag zur Kasuistik und Aetiologie der Cystinurie , Centralbl. f. d. Krankh. d. Harn- u. Sex.-Org. 15:121, 1904;.
Thévenot, M.:  Calcul renal bilateral de cystine pure chez une fillete , J. d'urol. 27:231 ( (March) ) 1929;.
Harris, A.:  Cystinuria: A Comprehensive Study with Report of an Interesting Case , Surg., Gynec. & Obst. 38:640 ( (May) ) 1924;.
Thomas, G. J., and Rodda, F. C.:  Multiple Cystine Calculi in the Left Kidney with Obstruction at the Uretero-Pelvic Juncture and Multiple Cystine Calculi in the Bladder in a Boy of Four years , Tr. Am. A. Genito-Urin. Surgeons , 23:451, 1930;.
Beer, E.:  Cystine Nephrolithiasis: Colon Bacillemia , Internat. J. Surg. 33:219 ( (July) ) 1920;.
Culligan, J. M.:  Renal Stones Permeable to the X-Ray , J. Urol. 11: 559 ( (June) ) 1924;.
Cantani, A.: Cystinurie, Fettsucht, Gallensteine, Specielle Pathologie und Therapie der Stoffwechselkrankheiten , Leipzig, Denicke, 1881;, vol. 3.
Beale, L. S.:  Cystine in the Urine Successfully Treated by Large Doses of Carbonate of Ammonia , Lancet 2:363, 1884;.
Klemperer, G., and Jacoby, M.:  Zur Behandlung der Cystinurie , Therap. d. Gegenw. 55:101, 1914;
abstr., J. A. M. A. 62:1207 ( (April 11) ) 1914;.
Edwards, H. C.:  Cystinuria with Calculous Formation , Brit. J. Surg. 12: 345 ( (Oct.) ) 1924;.
Crowell, A. J.:  Cystine Nephrolithiasis , J. Urol. 11:545 ( (June) ) 1924;
Surg., Gynec. & Obst. 38:87 ( (Jan.) ) 1924;.
Reaves, J. U.:  Treatment of Cystine Stones , South. M. J. 18: 599 ( (Aug.) ) 1925;.
Mörner, C. T.:  Can Anything be Done to Diminish Stone Formation in Cystinuria? Upsala läkaref. förh. 32: 1 ( (Feb.) ) 1927;.

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Simon, C. E.:  Cystinuria and Its Relation to Diaminuria , Am. J. M. Sc. 119: 39, 1900;.
Link, R. E. A.: Beitrag zur Kenntnis der Cystinurie und der Cystinsteine , Leipzig, Sturm & Koppe, 1912;.
Seeger, S. J., and Kearns, W. M.:  Cystinuric Lithiasis , J. A. M. A. 85:4 ( (July 4) ) 1925;.
Lewis, H. B.:  Cystinuria: A Review of Some Recent Investigations , Yale J. Biol. & Med. 4:437 ( (March) ) 1932;.
Henline, R. B.:  Cystine Calculi: Report of Three Cases , Am. J. Surg. 8:581 ( (March) ) 1930;.
Wollaston, W. H.:  On Cystic Oxide: A New Species of Urinary Calculus , Phil. Tr. , London, 1810;, p. 223.
Prout, W.: On the Nature and Treatment of Stomach and Urinary Diseases , ed. 3, London, J. & A. Churchill, 1840;.
Lassaigne, J. L.:  Observation sur l'existence de l'oxide cystique dans un calcul vesical du chien, et essai analytique sur la composition élémentaire de cette substance particulière , Ann. de chim. et phys. 23: 328, 1823;.
Ward, F. W.:  The Absorption Spectra of Some Indole Derivatives , Biochem. J. 17:891, 1923;.
Lewis, H. B., and Lough, S. A.:  Metabolism of Sulphur: Metabolic Study of a Case of Cystinuria , J. Biol. Chem. 81:285 ( (Feb.) ) 1929;.
Robson, W.:  Protein Metabolism in Cystinuria , Biochem. J. 23: 138, 1929;.
Alsberg, C., and Folin, O.:  Protein Metabolism in Cystinuria , Am. J. Physiol. 14: 54, 1905;.
Cox, G. J.; Smythe, C. V., and Fishback, C. F.:  The Nephropathogenic Action of Cystine , J. Biol. Chem. 82:95 ( (April) ) 1929;.
Lignac, G. O. E.:  Nephrosis and Nephritis Caused by Cystine , Nederl. tijdschr. v. geneesk. 2:2187 ( (Nov. 4) ) 1925;
abstr., J. A. M. A. 86:318 ( (Jan. 23) ) 1926;.
Curtis, A. C., and Newburgh, L. H.:  The Toxic Action of Cystine on the Kidney , Arch. Int. Med. 39:817 ( (June) ) 1927;.
Cox, G. J., and Hudson, Leona:  The Nephropathogenic Action of Cystine and the Dietary Control of Cystine Nephrosis , J. Nutrition 2:271 ( (Jan.) ) 1930;.
Tennant, C. E.:  Cystine Calculi: A Complex Surgical Problem , J. A. M. A. 80:305 ( (Feb. 3) ) 1923;.
Mörner, C. T.:  Cystinuria in Sweden , Upsala läkaref. förh. 31: 171 ( (Sept.) ) 1926;.
Toel, F.:  Beobachtungen über Cystinbildung , Ann. d. Pharm. 96:247, 1855;.
Abderhalden, Emil:  Familiare Cystindiathese , Ztschr. f. physiol. Chem. 38: 557, 1903;.
Kretschmer, H. L.:  Cystinuria and Cystine Stones, with a Report of a New Family of Cystinurics , Urol. & Cutan. Rev. 20: 1, 1916;.
Thin, Robert:  Familial Cystinuria , Edinburgh M. J. 36:490 ( (Aug.) ) 1929;.
Blum, L.:  Ueber das Schicksal des Cystins in Tierkorper , Beitr. z. chem. Phys. u. Path. 5: 1, 1903;.
Moreigne, H.:  Étude sur la cystinurie , Arch. de méd. expér. et d'anat. path. 11:254, 1899;.
Wasserthal:  Beitrag zur Kasuistik und Aetiologie der Cystinurie , Centralbl. f. d. Krankh. d. Harn- u. Sex.-Org. 15:121, 1904;.
Thévenot, M.:  Calcul renal bilateral de cystine pure chez une fillete , J. d'urol. 27:231 ( (March) ) 1929;.
Harris, A.:  Cystinuria: A Comprehensive Study with Report of an Interesting Case , Surg., Gynec. & Obst. 38:640 ( (May) ) 1924;.
Thomas, G. J., and Rodda, F. C.:  Multiple Cystine Calculi in the Left Kidney with Obstruction at the Uretero-Pelvic Juncture and Multiple Cystine Calculi in the Bladder in a Boy of Four years , Tr. Am. A. Genito-Urin. Surgeons , 23:451, 1930;.
Beer, E.:  Cystine Nephrolithiasis: Colon Bacillemia , Internat. J. Surg. 33:219 ( (July) ) 1920;.
Culligan, J. M.:  Renal Stones Permeable to the X-Ray , J. Urol. 11: 559 ( (June) ) 1924;.
Cantani, A.: Cystinurie, Fettsucht, Gallensteine, Specielle Pathologie und Therapie der Stoffwechselkrankheiten , Leipzig, Denicke, 1881;, vol. 3.
Beale, L. S.:  Cystine in the Urine Successfully Treated by Large Doses of Carbonate of Ammonia , Lancet 2:363, 1884;.
Klemperer, G., and Jacoby, M.:  Zur Behandlung der Cystinurie , Therap. d. Gegenw. 55:101, 1914;
abstr., J. A. M. A. 62:1207 ( (April 11) ) 1914;.
Edwards, H. C.:  Cystinuria with Calculous Formation , Brit. J. Surg. 12: 345 ( (Oct.) ) 1924;.
Crowell, A. J.:  Cystine Nephrolithiasis , J. Urol. 11:545 ( (June) ) 1924;
Surg., Gynec. & Obst. 38:87 ( (Jan.) ) 1924;.
Reaves, J. U.:  Treatment of Cystine Stones , South. M. J. 18: 599 ( (Aug.) ) 1925;.
Mörner, C. T.:  Can Anything be Done to Diminish Stone Formation in Cystinuria? Upsala läkaref. förh. 32: 1 ( (Feb.) ) 1927;.
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