Blood glucose profile and insulin levels during the oral glucose tolerance test were retrospectively analyzed in 52 cystic fibrosis (CF) patients aged 10–33 years (mean 18 years). Thirty-seven subjects (71.1%) had normal glucose tolerance; 6 patients (11.5%) had impaired glucose tolerance and 9 patients (17.3%), with fasting blood glucose levels below 7.2 mmol/l were classified as diabetic, according to WHO diagnostic criteria. No significant differences in the total amount of secreted insulin (expressed as area under the curve) were detected in CF patients with or without glucose intolerance, but a significant delay in the insulin peak time appeared to be related to a compromised nutritional status. Our data indicate a significantly increased risk of glucose metabolism impairment related to poor nutritional condition (RR 5.40; 95% CI: 1.5–19.7) and worse clinical status (RR 4.27; 95% CI: 1.05–17.2). In particular, glucose tolerance abnormalities were found in 50% of CF patients with an unsatisfactory nutritional condition versus 15.6% of CF patients with good nutritional status. Conclusions: Since CF-related diabetes is often underdiagnosed and associated with deterioration in patients’ overall clinical status, it is very important to identify subjects at risk of developing diabetes by strict monitoring of glucose metabolism when deterioration in nutritional and clinical conditions is seen which cannot be otherwise explained.

Keywords:
Cystic fibrosis, Glucose tolerance, Diabetes, Insulin secretion
1.
Stacey C, Fitzsimmons PD: The changing epidemiology of cystic fibrosis. J Pediatr 1993;122:1–5.
2.
Krueger LJ, Lerner A, Kats SM, Mack R,Holsclaw DS Jr, Lebenthal E: Cystic fibrosis and diabetes mellitus: Interactive or idiopathic? J Pediatr Gastroenterol Nutr 1991;2:209–219.
3.
Hodson ME: Diabetes mellitus and cystic fibrosis. Clin Endocrinol Metab 1992;6:797–805.
4.
Koch C, Lanng S: Other organ systems; in Hodson ME, Geddes DM (eds): Cystic Fibrosis. London, Chapman & Hall, 1995.
5.
WHO Study Group: Diabetes Mellitus. WHO Tech Rep Ser 1985;727:1–113.
6.
National Diabetes Data Group: Classification and diagnosis of diabetes mellitus and other categories of glucose intolerance. Diabetes 1979;28:1039–1075.
7.
Reisman J, Corey M, Canny G, Levison H: Diabetes mellitus in patients with cystic fibrosis. Pediatrics 1988;86:374–377.
8.
Finkelstein SJ, Wielinski CL, Elliott GR, Warwick WJ, Barbosa J, Wu SC, Klein DJ: Diabetes mellitus associated with cystic fibrosis. J Pediatr 1988;112:373–377.
9.
Consensus Conference on CF related Diabetes mellitus. Bethesda, Md., Cystic Fibrosis Foundation, 1990, vol 1, pp 1–5.
10.
Shwachman H, Kulczycki LL: Long-term study of 105 patients with cystic fibrosis. Am J Dis Child 1958;96:6–15.
11.
Consensus Conference on Nutritional Assessment and Management in Cystic Fibrosis. Bethesda, Md., Cystic Fibrosis Foundation, 1990, vol 1, pp 1–15.
12.
Cystic Fibrosis Genetic Analysis Consortium: Population variation of common cystic fibrosis mutations. Hum Mutat 1994;1:1–15.
13.
Epidemiological graphics, estimation, testing package ANALYSIS MODULE (PECAN), version 0.26.6; EPIXAT (R), version 0.03 (C) Copyright 1985–1991, SERC and CYTEL.
14.
Arrigo T, Cucinotta D, Conti Nibali S, Di Cesare E, Di Benedetto A, Magazzù G, De Luca F: Longitudinal evaluation of glucose tolerance and insulin secretion in nondiabetic children and adolescents with cystic fibrosis: results of two-year follow-up. Acta Paediatr 1993;82:249–253.
15.
De Luca F, Arrigo T, Di Benedetto A, Tedeschi A, Sferlazzas C: Four year follow-up of glucose tolerance and beta-cell function in nondiabetic cystic fibrosis patients. Horm Res 1995;44:45–50.
16.
Lanng S, Thorsteinsson B, Erichsen G, Neruo J, Koch C: Glucose tolerance in cystic fibrosis. Arch Dis Child 1991;66:612–616.
17.
De Schepper J, Dab MP, Lolb H: OGTT in cystic fibrosis: Correlation with clinical parameters and glycosylated haemoglobin determinations. Eur J Pediatr 1991;150:403–406.
18.
Lanng S, Thorsteinsson B, Roeder M, Orskov C, Holst J, Nerup J, Koch C: Pancreas and gut hormone responses to oral glucose and intravenous glucagon in cystic fibrosis patients with normal, impaired and diabetic glucose tolerance. Acta Endocrinol Copenh 1993;128:207–214.
19.
Leslie RDG, Lo S, Millward BA, Honour J, Pyke DA: Decreased growth velocity before IDDM onset, Diabetes 1991;40:211–216.
20.
Mohan V, Alagappan V, Snehalatha C, Ramachandran A, Thiruvengadam KV, Viswanathan M: Insulin and C-peptide responses to glucose load in cystic fibrosis. Diabetes Metab 1985;11:376–379.
21.
Holl RW, Heinze E, Wolf A, Rank M, Teller WM: Reduced pancreatic insulin release and reduced peripheral insulin sensitivity contribute to hyperglycaemia in cystic fibrosis. Eur J Pediatr 1995;154:356–361.
22.
Ahmad T, Nelson R, Taylor R: Insulin sensitivity and metabolic clearance rate of insulin in cystic fibrosis. Metabolism 1994;43:163–167.
23.
Robert JJ, Grasset E, De Montalembert M, Chevenne D, Deschamps I, Boitard C, Lenoir G: Recherche de facteurs d’intolérance au glucose dans la mucoviscidose. Arch Fr Pediatr 1992;49:17–22.
24.
Moran A, Diern P, Klein DJ, Levitt MD, Robertson RP: Pancreatic endocrine function in cystic fibrosis. J Pediatr 1991;118:715–723.
25.
Cucinotta D, De Luca F, Arrigo T, Di Benedetto A, Sferlazzas C, Gigante A, Rigoli L, Magazzu G: First-phase insulin response to intravenous glucose in cystic fibrosis patients with different degrees of glucose tolerance. J Pediatr Endocrinol 1994;7:13–17.
26.
Stahl M, Girard J, Rutishauser M, Nars PW, Zuppinger K: Endocrine function of the pancreas in cystic fibrosis: Evidence for an impaired glucagon and insulin response following arginine infusion. J Pediatr 1976;84:821–824.
27.
Reisman J, Corey M, Canny G, Levison H: Diabetes mellitus in patients with cystic fibrosis: Effect on survival. Pediatrics 1990;86:374–377.
28.
Lanng S, Thorseinsson B, Nerup J, Koch C: Influence of the development of diabetes mellitus on clinical status in patients with cystic fibrosis. Eur J Pediatr 1992;151:684–687.
1998
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