Abstract • Nephropathlc cystinosis causes renal death by approximately age 10 years. With increased life span due to kidney transplantation, ten to 25 years of cystine accumulation has resulted in pancreatic complications in individuals with cystinosis. We noted severe hyperglycemia in five posttransplant patients, three of whom remained insulin-dependent diabetics several years after transPlant. The clinical findings were not consistent with steroid-dependent or insulin-resistant diabetes. Pancreatic cystine deposition was detected histologically and biochemically on postmortem examination of two other patients. We conclude that hyperglycemia may be anticipated in the immediate posttransplant period in cystinotic patients and that some patients will require insulin therapy years later. The use of cystine-depleting agents should be considered in posttransplant cystinosis as an attempt to prevent potential damage to the pancreas and other organs from cystine deposition. (AJDC 1987;141:1087-1089) References 1. Schneider JA, Schulman JD: Cystinosis , in Stanbury JB, Wyngaarden JB, Fredrickson DS, et al (eds): The Metabolic Basis of Inherited Disease , ed 5. New York, McGraw-Hill International Book Co, 1983, pp 1844-1866. 2. Gahl WA: Cystinosis . Adv Pediatr 1986;33: 95-126. 3. Gahl WA, Tietze F, Bashan N, et al: Defective cystine exodus from isolated lysosome-rich fractionsfractions of cystinotic leucocytes . J Biol Chem 1982;257:9570-9575. 4. Gahl WA, Bashan N, Tietze F, et al: Cystine transport is defective in isolated leukocyte lysosomes from patients with cystinosis . Science 1982;217:1263-1265.Crossref 5. Gretz N, Manz F, Augustin R, et al: Survival time in cystinosis: A collaborative study . Proc Eur Dial Transplant Assoc Eur Ren Assoc 1982;19:582-589. 6. Chantler C, Carter JE, Bewick M, et al: Ten years' experience with regular haemodialysis and renal transplantation . Arch Dis Child 1980;55: 435-445.Crossref 7. Broyer M, Guillot M, Gubler MC, et al: Infantile cystinosis: A reappraisal of early and late symptoms. Adv Nephrol 1981;10:137-166. 8. Lucky AW, Howley PM, Megyesi K, et al: Endocrine studies in cystinosis: Compensated primary hypothyroidism . J Pediatr 1977;91: 204-210.Crossref 9. Kaiser-Kupfer MI, Caruso RC, Minckler D, et al: Long-term ocular findings in cystinosis post-renal transplant . Arch Ophthalmol 1986;104:706-711.Crossref 10. Green OC: Endocrinologic complications associated with cystic fibrosis , in Lloyd-Still JD (ed): Textbook of Cystic Fibrosis . Littleton, Mass, John Wright–PSG Inc, 1983, pp 329-350. 11. Vinik AI, Jackson WPU: Endocrine secretions in chronic pancreatitis , in Podolsky S, Viswanathan M (eds): Secondary Diabetes: The Spectrum of the Diabetic Syndromes . New York, Raven Press, 1980, pp 165-189. 12. Viswanathan M: Pancreatic diabetes in India , in Podolsky S, Viswanathan M (eds): Secondary Diabetes: The Spectrum of the Diabetic Syndromes . New York, Raven Press, 1980, pp 105-116. 13. Gahl WA, Schneider JA, Thoene JG, et al: Course of nephropathic cystinosis after age 10 years . J Pediatr 1986;109:605-608.Crossref 14. Gahl WA, Reed GF, Thoene JG, et al: Cysteamine therapy for children with nephrotic cystinosis . N Engl J Med 1987;316:971-977.Crossref
American Journal of Diseases of Children – American Medical Association
Published: Oct 1, 1987
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