Do children and adolescents with type 1 diabetes suffer from a lack of resources in France? Results from a benchmark study in the New Aquitaine region.

BACKGROUND: A benchmark study was conducted in the southwest of France, in the New Aquitaine region, to investigate metabolic outcomes and availability of resources in pediatric diabetes units. We assessed whether the level of care was in accordance with the International Society for Pediatric and Adolescent Diabetes recommendations. METHODS: Demographic and clinical data were collected, as were all HbA1c tests for the 2017 calendar year. Pediatricians specialized in diabetes care were invited to complete an online survey concerning means allocated to the management of type 1 diabetes in their centers. RESULTS: Sixteen centers provided data for 1277 patients and 3873 clinical visits. A total of 1115 children suffering from diabetes for more than 1 year were studied. Median HbA1c was 8% (7.4-8.6) for the whole region. Only 29.2% of children had good metabolic control in accordance with the <7.5% target. We identified slight but significant variation in glycemic control among centers (P=0.029). The use of an insulin pump varied greatly among centers but did not explain HbA1c differences. We did not identify a correlation between medical or paramedical time dedicated to the follow-up of diabetic patients and the mean HbA1c of each center. For 100 diabetic patients, follow-up was provided by 0.42 physicians (0.23-1.50), 0.15 nurses (0-0.56), 0.12 dietitians (0-0.48), and 0.07 psychologists (0-0.30). CONCLUSION: This study demonstrates a lack of human resources allocated to the management of type 1 diabetes in the region that is far below international recommendations. The proportion of children achieving the international glycemic target is low. There is a clear need to improve glycemic control in children, which will only be possible with improved professional practices, encouraged by benchmark studies, and by increasing the size of our multidisciplinary teams.

The growing incidence of type 1 diabetes in children: the 17-year French experience in Aquitaine.

BACKGROUND: While the incidence of type 1 diabetes in children has increased in various parts of the world, in France, no actual figures have been available since 1997. OBJECTIVE: The aim of this study was to determine whether or not the pattern of increase in the incidence of type 1 diabetes in children aged less than 15 years varies with age at onset in Aquitaine (France) over a 17-year period. PATIENTS AND METHODS: From 1988 to 1997, all newly diagnosed cases of type 1 diabetes were confirmed by registration into the French Registry of Incidence of Diabetes. Subsequently, all cases registered from 1998 to 2004 were collected within paediatric centres in Aquitaine as part of their hospital-based prospective records. RESULTS: In the overall population, the age- and gender-adjusted incidence rate increased from 8.86 per 100,000 per year (95% CI: 6.27-11.45) in 1988 to 13.47 per 100,000 per year (95% CI: 10.29-16.65) in 2004, indicating an annual increase in incidence of 3.34% (95% CI: 3.33-3.34). Median age at diabetes onset for cases in the first registration period (1988-1996) was significantly higher than that in the second registration period (1997-2004): 10.04 years (range: 6.64-12.53) versus 8.83 years (range: 5.48-11.73), respectively (P=0.01). The annual increase in incidence rate was highest in the youngest children and varied significantly with age (0-4 years: 7.59%; 5-9 years: 4.06%; 10-14 years: 1.28%). CONCLUSION: These results indicate a doubling of the incidence of type 1 diabetes in children every 30 years in Aquitaine, with an even steeper increase among younger children, thus underscoring the need for appropriate adaptation of the system of healthcare provision.

Gastroesophageal reflux and Cornelia de Lange syndrome: typical and atypical symptoms.

As previously reported, gastroesophageal reflux (GER) is a frequent and severe medical complication of Cornelia de Lange syndrome (CDLS). The incidence of GER and the correlation between its presence and degree, and the clinical phenotype of CDLS (mild/classical according to Van Allen classification) were evaluated in a series of 43 patients. The pattern of presenting symptoms and their clinical evolution after medical or surgical treatment were also studied. A pathological GER was evident in 28/43 (65%) CDLS patients. The incidence of the complication was not significantly different in patients with classical (93.3%) vs. mild phenotype (82.3%), whereas a strong correlation was present between the degree of the esophageal damage and the clinical phenotype. A behavioral symptom (hyperactivity) was the most frequent sign associated with the condition (85%). Our data confirm the high occurrence of GER in CDLS patients, independently from the CDLS clinical phenotype. Regarding the severity of the esophageal lesions, a significant difference between the two clinical CDLS phenotypes was found. The evaluation of the presenting symptoms and of their evolution during the treatment emphasizes the importance of behavioral symptoms as major signs of esophageal damage in CDLS.

[Fertility in the operated on monolateral cryptorchism patient]. / Studio sulla fertilità del criptorchide monolaterale operato.

Fertility of operated monolateral Cryptorchides has been evaluated in a long term follow up comparing the patients who underwent orchidopexy with patients who underwent orchiectomy. From our experience, as well as from various Author's reports, patients who underwent orchiectomy demonstrated a two fold of fertility compared to the group who underwent orchidopexy.

Renal tubular acidosis in a case of Shwachman's syndrome.

We describe metabolic acidosis in a 15-month-old girl with clinical features of Shwachman's syndrome. Renal function tests indicated that the patient had type 1 renal tubular acidosis. Based on our findings and other reports of renal tubular dysfunction in patients with Shwachman's syndrome, we conclude that it is important to look for a possible renal tubular defect in this syndrome.