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Assessment of glucose tolerance in nephropathic cystinosis patients /

Marwa Mohammed Elsayed Abdelmonaem

Assessment of glucose tolerance in nephropathic cystinosis patients / تقييم تفاوت مستوى السكر في الدم في مرضى داء السيستين Marwa Mohammed Elsayed Abdelmonaem ; Supervised Neveen Abdelmonem Soliman , Noha Arafa Mohammed , Ghada Maher Mohamed - Cairo : Marwa Mohammed Elsayed Abdelmonaem , 2019 - 138 P. : charts , facsimiles ; 25cm

Thesis (M.Sc.) - Cairo University - Faculty of Medicine - Department of Pediatrics

Background: Nephropathic cystinosis is a rare genetic disease characterized by accumulation of the amino acid cystine in lysosomes throughout the body. Diabetes mellitus remain a major complication of nephropathic cystinosis. Methods: Assessment of glucose metabolic status among sixteen patients with confirmed nephropathic cystinosis aged three years or more using OGTT and HbA1C. Evaluation of pancreatic Ý cell functions using 2-hour postprandial c-peptide values. Results: The study included 16 patients their age ranged from 4 to 21 years. Their age at diagnosis of nephropathic cystinosis ranged from 5 to 96 months. The incidence of abnormal glucose metabolism among our cohort of patients with nephropathic cystinosis was 4 patients (25%). One patient (6.2%) had both impaired fasting glucose (IFG) and IGT; one patient (6.2%) had only impaired glucose tolerance (IGT). Another two patients (12.5%) showed diabetic levels blood glucose during OGTT. Four patients had impaired HbA1C > 6%. Ten patients had elevated 2-hour postprandial c-peptide > 7.1 ng/ml. Conclusion: All patients with nephropathic cystinosis are at risk of developing diabetes mellitus as a complication of CTN during adolescence or adulthood if they are not compliant to treatment, or 10 years after renal transplantation due to steroid and tacrolimus use. Nevertheless, there is a pressing need enhance patient compliance for oral cysteamine and steroid sparing protocol of immunosuppressive drugs and use cyclosporine instead of tacrolimus to improve outcome



Impaired fasting glucose Nephropathic cystinosis Oral glucose tolerance test