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Renal disease in type I glycogen storage disease.

Publication ,  Journal Article
Chen, YT; Coleman, RA; Scheinman, JI; Kolbeck, PC; Sidbury, JB
Published in: N Engl J Med
January 7, 1988

Although kidney enlargement occurs in Type I glycogen storage disease, renal disease has not been considered a major problem. Death from renal failure in three patients known to us prompted a study of renal function in this disorder. Of the 38 patients with Type I glycogen storage disease under our care, the 18 children under 10 years old had normal renal function. Fourteen of the 20 older patients (13 to 47 years) had disturbed renal function, manifested by persistent proteinuria; many also had hypertension, hematuria, or altered creatinine clearance. Progressive renal insufficiency developed in 6 of these 14 patients, leading to three deaths from renal failure. At the onset of proteinuria, creatinine clearance was increased in seven patients (3.05 +/- 0.68 ml per second per 1.73 m2 of body-surface area; range, 2.47 to 4.13 [normal range, 1.33 to 2.33 ml per second per 1.73 m2]). Renal biopsies were performed in three patients after an average of 10 years of proteinuria. All three biopsies demonstrated focal segmental glomerulosclerosis in various stages of progression. Our data suggest that chronic renal disease is a frequent and potentially serious complication of Type I glycogen storage disease. In addition to treating hypoglycemia vigorously, physicians should monitor renal function carefully in patients with this disorder.

Duke Scholars

Published In

N Engl J Med

DOI

ISSN

0028-4793

Publication Date

January 7, 1988

Volume

318

Issue

1

Start / End Page

7 / 11

Location

United States

Related Subject Headings

  • Proteinuria
  • Male
  • Kidney Failure, Chronic
  • Kidney Diseases
  • Kidney
  • Infant
  • Hypertension, Renal
  • Humans
  • Glycogen Storage Disease Type I
  • Glomerulosclerosis, Focal Segmental
 

Citation

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Chen, Y. T., Coleman, R. A., Scheinman, J. I., Kolbeck, P. C., & Sidbury, J. B. (1988). Renal disease in type I glycogen storage disease. N Engl J Med, 318(1), 7–11. https://doi.org/10.1056/NEJM198801073180102
Chen, Y. T., R. A. Coleman, J. I. Scheinman, P. C. Kolbeck, and J. B. Sidbury. “Renal disease in type I glycogen storage disease.N Engl J Med 318, no. 1 (January 7, 1988): 7–11. https://doi.org/10.1056/NEJM198801073180102.
Chen YT, Coleman RA, Scheinman JI, Kolbeck PC, Sidbury JB. Renal disease in type I glycogen storage disease. N Engl J Med. 1988 Jan 7;318(1):7–11.
Chen, Y. T., et al. “Renal disease in type I glycogen storage disease.N Engl J Med, vol. 318, no. 1, Jan. 1988, pp. 7–11. Pubmed, doi:10.1056/NEJM198801073180102.
Chen YT, Coleman RA, Scheinman JI, Kolbeck PC, Sidbury JB. Renal disease in type I glycogen storage disease. N Engl J Med. 1988 Jan 7;318(1):7–11.
Journal cover image

Published In

N Engl J Med

DOI

ISSN

0028-4793

Publication Date

January 7, 1988

Volume

318

Issue

1

Start / End Page

7 / 11

Location

United States

Related Subject Headings

  • Proteinuria
  • Male
  • Kidney Failure, Chronic
  • Kidney Diseases
  • Kidney
  • Infant
  • Hypertension, Renal
  • Humans
  • Glycogen Storage Disease Type I
  • Glomerulosclerosis, Focal Segmental