[Endocrine complications of cystic fibrosis in childhood]. / Les complications endocriniennes de la mucoviscidose chez l'enfant.

Since the 20 last years, the median age of survival has dramatically improved in children suffering from cystic fibrosis and complications such as growth retardation, pubertal delay and low bone mineral density are now more often than not observed in affected adolescents. The severity of the disease and the poor nutritional status due to pancreatic insufficiency and malabsorption are commonly implicated but recent data suggest that the disease could also play a role though the alteration of the chlore chanel (CFTR). Furthermore an increase prevalence of glucose intolerance and diabetes due to the progressive ß cells destruction is observed in these children that make the life sometimes difficult for these adolescents already affected by an heavy chronic disease. The monitoring of the children should thus now become pluridisciplinary and include regular clinical evaluation of height and pubertal status, mineral bone density by DEXA and OGTT every two years since 10 years of age. Therefore, in addition to the standard treatment of cystic fibrosis is now added the vitamin D supplementation, the subcutaneous insulin therapy and may be the growth hormone that could be a new therapeutic demonstrating beneficial effects in these chronic disease. However further studies need to be performed to improve the management of these new endocrine complications more and more frequent in children and adolescents suffering from cystic fibrosis.

Factors associated with glycemic control. A cross-sectional nationwide study in 2,579 French children with type 1 diabetes. The French Pediatric Diabetes Group.

OBJECTIVE: To determine on a large scale the multiple medical and nonmedical factors that influence glycemic control in the general population of children with diabetes, we performed a nationwide French cross-sectional study. RESEARCH DESIGN AND METHODS: We enrolled 2,579 patients aged 1-19 years with type 1 diabetes of > 1 year's duration. The study was center based: 270 centers were identified, 206 agreed to participate, and 147 included at least 90% of their patients. Questionnaires were completed by physicians interviewing patients and family, and HbA1c measurements were centralized. To identify explanatory variables for HbA1c level and frequency of severe hypoglycemia, we performed multiple regression analysis using all the quantitative variables collected and stepwise logistic regression for the qualitative variables. RESULTS: Mean HbA1c value for the whole population was 8.97 +/- 1.98% (normal 4.7 +/- 0.7% [SD]). Only 19 children (0.7%) had ketoacidosis during the 6 months before the study, whereas 593 severe hypoglycemia events occurred in 338 children (13.8%). Control was better in university-affiliated hospitals and centers following > 50 patients, reflecting the importance of access to experienced diabetologists. Children had a mean of 2.3 injections, allegedly performed 2.8 glucose measurements per day, and were seen an average of 4.6 times per year at the center. In the multiple regression analysis, 94% of the variance of HbA1c was explained by our pool of selected variables, with the highest regression coefficient between HbA1c and age (Rc = 0.43, P < 0.0001), then with daily insulin dosage per kilogram (Rc = 0.28, P < 0.0001), mother's age (Rc = 0.26, P < 0.0001), frequency of glucose measurements (Rc = 0.21, P < 0.0001), and diabetes duration (Rc = 0.14, P < 0.0001). Logistic regression identified quality of family support and dietary compliance, two related qualitative and possibly subjective variables, as additional explanatory determinants of HbA1c. The frequency of severe hypoglycemia was 45 per 100 patient-years and correlated with diabetes duration, but not with HbA1c levels or other variables. CONCLUSIONS: Although overall results remain unsatisfactory, 33% of studied French children with type 1 diabetes had HbA1c < 8%, the value obtained in Diabetes Control and Complications Trial adolescents treated intensively. Diabetes management in specialized centers should be encouraged.

Improvement of diabetic control and acceptability of a three-injection insulin regimen in diabetic adolescents. A multicenter controlled study.

OBJECTIVE: To compare the effectiveness and acceptability of a three-injection insulin regimen with the conventional two-injection therapy in an unselected population of diabetic adolescents. RESEARCH DESIGN AND METHODS: Some 205 patients aged 10-18 yr with IDDM, who were previously treated with two daily insulin injections, were included without any selection into a randomized trial. They were either switched to three (regular prebreakfast, regular prelunch, and [regular+ultralente] predinner) or remained on two ([regular+intermediary] prebreakfast and predinner) subcutaneous injections. They were evaluated after 1 yr of treatment. The major criteria of outcome of efficacy were the concentration of GHb, the frequency of severe hypoglycemia and DKA, and body weight. RESULTS: Of the patients, 82% accepted the three-injection regimen, and 83% accepted the two-injection regimen. At entry into the trial, no significant differences appeared between the two treatment groups nor among patients refusing the allocated regimen. Significant explanatory variables predicting initial diabetes control were duration of disease and adherence to diet. GHb, decreased from 9.8 +/- 0.1 to 9.3 +/- 0.2% (P < 0.05) in the three-injection group, whereas it increased from 9.5 +/- 0.3 to 9.8 +/- 0.3% (P < 0.05) in the two-injection group, resulting in a modest (0.75%) but significant difference (P < 0.05) between GHb change in the two groups. The difference reached 1.4% (P < 0.0002) in patients with GHb > 11.2% at entry. The frequency of hypoglycemia and DKA was similar in the two groups. None of the parameters known to potentially influence glycemic control changed during the trial, and, therefore, the improvement of GHb could be attributed to the pattern of daily insulin distribution per se. CONCLUSIONS: In the general diabetic adolescent population, the efficacy of a three-injection regimen is somewhat superior to that of a conventional two-injection regimen, particularly in patients previously poorly controlled. The acceptability of this regimen being excellent, its increased use should be considered in this age-group.

[Insulin therapy using a portable pump in children. Apropos of a pediatric experience]. / Insulinothérapie par pompe portable chez l'enfant. A propos d'une expérience pédiatrique.

Experience accumulated with 17 pediatric patients with insulin-dependent diabetes mellitus treated with an insulin pump is reported. Follow-up exceeds four years in seven patients. A significant improvement in metabolic control was seen in only four patients and was confined to the first year of use of the pump. Overall, the improvement in control was not significant. Severe hypoglycemia was uncommon (0.27/patient-year). Ketoacidosis was rare (0.08/patient-year). Transient ketosis was the most common adverse event and was often related to technical problems which should immediately lead to appropriate action by patients who have received proper education. Use of an insulin pump remains an alternative which deserves to be considered when conventional therapies fail.

[The participation in sports of diabetic children. A survey in the Rouen region]. / La pratique du sport chez l'enfant diabétique. Enquête dans la région rouennaise.

The purpose of this survey conducted during the 1987 academic year was to collect information on the participation in sports, whether at school or in athletic clubs, of 63 children followed on an outpatient basis for diabetes mellitus and to determine what steps are effectively taken by these children to prevent hypoglycemia. Simultaneously, the participation in sports of 200 children seen in a surgical outpatient clinic for any reason was determined. The data collected demonstrated that the diabetic children participated as actively as non-diabetic children in athletics at school and, in addition, were likely to participate in out-of-school non-compulsory sports (40% versus 27%). Approximately two out of three diabetic children reported attempting to prevent hypoglycemia during sports at school by an intake of carbohydrates before or after the activity and/or by a reduction in the dose of insulin acting during the activity, with variable results. Results suggested that hypoglycemic episodes tended to be overtreated, perhaps in part because the teachers were apprehensive about the possible negative effects of hypoglycemia. During out-of-school non-compulsory athletic activities, especially competitive sports, preventive measures against hypoglycemia were consistently taken and diary results seemed to improve steadily in patients who trained regularly several times a week. Lastly, in the population studied no correlation was found between the time spent every week participating in sports and glycated hemoglobin levels.