Impact of continuous glucose monitoring on everyday life of young children with type 1 diabetes and their parents: An evaluation of 114 families.

INTRODUCTION: The prevalence of type 1 diabetes is increasing worldwide. The advent of new monitoring devices has enabled tighter glycemic control. AIM: To study the impact of glucose monitoring devices on the everyday life of young children with type 1 diabetes (T1D) and their parents. METHODS: A questionnaire was addressed to parents of children with T1D under the age of 6 years with an insulin pump treated in one of the hospitals of the ADIM network in France between January and July 2020. RESULTS: Among the 114 families included in the study, 53% of parents (26/49) woke up every night to monitor blood glucose levels when their child had flash glucose monitoring (FGM), compared with 23% (13/56) of those whose child had continuous glucose monitoring (CGM). Overall, 81% of parents (86/108) found that glucose monitoring improved their own sleep and parents whose child had CGM were significantly more likely to report improved sleep (86% vs 73%, p = 0.006). Forty-nine percent of parents (55/113) declared that they (in 87% of cases, the mother only) had reduced their working hours or stopped working following their child's T1D diagnosis. Maternal unemployment was significantly associated with the presence of siblings (p = 0.001) but not with glycemic control (p = 0,87). Ninety-eight percent of parents (105/107) think that glucose monitoring improves school integration. CONCLUSION: In these families of children with T1D, new diabetes technologies reduced the burden of care but sleep disruption remained common. Social needs evaluation, particularly of mothers, is important at initial diagnosis of T1D in children.

[Childhood steroid-dependent idiopathic nephrotic syndrome: Predictive factors for the need of immunosuppressive treatment]. / Syndrome néphrotique idiopathique corticodépendant de l'enfant : facteurs prédictifs de recours à un traitement immunosuppresseur.

BACKGROUND AND AIMS: More than half of the children with idiopathic nephrotic syndrome become steroid-dependent (or frequent relapsers) and will later require the use of complementary treatment aiming to reduce steroids' side effects and to limit the number of proteinuria relapses. It appears important to identify these children as early as possible in order to adapt their treatment. The aim of this study was to analyze the population of children, under 18 years of age, diagnosed between 1/01/2000 and 31/05/2015 with an idiopathic nephrotic syndrome and followed at the Montpellier University Hospital to search for criteria predictive of steroid-sparing agent use. METHODS: In this retrospective study of children with idiopathic nephrotic syndrome, the exclusion criteria were primary steroid resistance and children with no proteinuria relapse after diagnosis. RESULTS: Eighty-four children (54 boys) were included in this study. The mean follow-up duration was 5.5 years (0.75-16). The mean age at diagnosis was 4.6 years. Sixty-five children (77%) received at least one steroid-sparing agent during their follow-up, within a mean 10 months after diagnosis. In these patients, the first relapse of the disease occurred earlier when compared with the children who were maintained on steroid alone (4 months vs 7 months; P<0.001). The use of methylprednisolone pulses to obtain a remission, the cumulative dose of steroid treatment, and the number of proteinuria relapses were also significantly correlated with the use of complementary immunosuppressive therapy. CONCLUSION: We found no predictive criteria of the use of steroid-sparing agents at diagnosis in our population of children. Nevertheless, with the steroid regimen used, the time within which the first proteinuria relapse occurred appears to be a significant criterion for the secondary use of a steroid-sparing agent. These data should be taken into account when choosing the treatment regimen.

[Epidemiology of diabetes in children in Languedoc-Roussillon (France)]. / Registre épidémiologique du diabète chez l'enfant en Languedoc-Roussillon.

Since 1st January 2000, the PEDIAB-LR registry has listed new cases of diabetes in children under 16 years of age in the Languedoc-Roussillon region of France, in order to assess the incidence and epidemiological characteristics of children affected by diabetes. At the end of December 2010, 745 children had been registered. The characteristics of these children included an identical proportion of girls and boys, a mean age of 8 years at diagnosis, and a family history of type 1 diabetes in 8.4% of the cases. Inaugural ketoacidosis was observed in 69.5% of the patients and was severe in 23.7% of these cases. To replace intravenous insulin, a two-injection dosing regimen (in the morning and before dinner) was prescribed in 76.5% of cases, multi-injections of basal-bolus in 16%, and subcutaneous insulin infusion (insulin pump therapy) in 7.5% of cases. Between 2000 and 2010, there was no increase in overall incidence. It is noteworthy that age at diagnosis tended to decrease, but this was not statistically significant. In conclusion, the PEDIAB-LR registry is currently the only French registry on diabetes in children, analyzing many factors related to its incidence, such as age at diagnosis and the existence of ketoacidosis.

[Hemolytic uremic syndrome with severe neurological involvement: how should it be managed?]. / Syndrome hémolytique et urémique avec atteinte neurologique sévère: quelle prise en charge?

INTRODUCTION: The management of diarrhea-associated hemolytic and uremic syndrome (D(+) HUS) with severe acute neurological involvement continues to be debated. We report on 2 cases and discuss the treatment. CASE REPORT 1: A 2.5-year-old girl presented with generalized seizures during gastroenteritis. Blood tests revealed features of HUS and a pyramidal syndrome was found on physical examination. Brain MRI, 24h after admission, showed lesions in the periventricular and subcortical area. She was started on peritoneal dialysis (PD) and daily plasma exchanges (PE) for 10 days. Her neurological condition improved quickly as well as the findings of the second brain MRI performed after PE. One year later she had no apparent neurological or renal sequelae. CASE REPORT 2: A 2.5-year-old boy presented with generalized seizures during gastroenteritis for 3 days, leading to a diagnosis of HUS. He also had a severe pyramidal syndrome with spastic tetraparesis and aphasia. Brain MRI, 48 h after admission, revealed severe bilateral and symmetric lesions involving the thalami, internal and external capsules, lenticular nuclei, and brainstem. He was started on PD and daily PE for 10 days. Brain MRI performed after PE was unchanged. Clinically, his neurological condition improved slowly with regression of spastic tetraparesis and progressive recovery of motor skills. Nine months later, his renal function is normal but he is still having intensive physiotherapy. DISCUSSION: Both children have received similar management including 10 PEs started within 48 h after the diagnosis of D(+) HUS with severe neurological involvement, but their neurological outcome appeared to be significantly different. There is no clear proof in the literature concerning the effects of PE in such patients, even when performed very early. Eculizumab, an antibody that inhibits complement factor 5a and the formation of the membrane attack complex, has recently been used in such cases and seems to provide a more specific therapeutic action. Control studies are needed to specify its use in this disease.

[Posterior urethral valves: prenatal diagnosis, neonatal data and outcome]. / Les valves de l'urètre postérieur. Etat des lieux en Languedoc-Roussillon de 1998 à 2007 : données anténatales, néonatales et devenir à moyen terme.

AIM OF THE STUDY: To evaluate the impact of prenatal diagnosis on the epidemiology and outcome of children with posterior urethral valves (PUV), considering that today termination of pregnancy may be proposed in the most severe cases. PATIENTS AND METHODS: Forty-three cases of patients with PUV were diagnosed between 1998 and 2007 in the Languedoc-Roussillon region. In this study, we detailed the prenatal data and postnatal outcome of those patients with a mean follow-up period of 7.6 years. RESULTS: Medical interruption of pregnancy was performed in 15 severe cases. One death in utero was also observed. Twenty-seven patients were managed postnatally and one child died during the neonatal period because of lung hypoplasia. For the 26 remaining patients at the end of the follow-up period, 5 had mild renal insufficiency with a creatinine clearance calculated with the Schwartz formula of less than 80 ml/min/1.73 m2. One reached end-stage renal failure at the age of 7 years. Proteinuria and hypertension were rare. DISCUSSION AND CONCLUSION: Today, because of the increase in medical termination of pregnancy in the most severe cases, most of the children born alive with PUV experienced a better renal prognosis when compared with previous data. Prenatal diagnosis and therapeutic progress significantly modified the epidemiology and outcome of this disease. Nevertheless, the best predictor of renal outcome remains renal function at 1 year of age.

[Trisomy 20 mosaicism revealed by pigmentary mosaicism of the Ito-type]. / Mosaïcisme pigmentaire de type Ito révélant une trisomie 20 en mosaïque.

INTRODUCTION: Ito hypomelanosis-type pigmentary mosaicism is characterized by congenital pigmentation disorders along Blaschko's lines. We report a case of Ito-type pigmentary mosaicism associated with a congenital growth hormone deficiency having revealed trisomy 20 mosaicism. OBSERVATION: A 4 year-old boy presented with congenital pigmentation disorders. His history was marked by: inter-uterine delayed growth of unknown etiology, a dysmorphic syndrome, psychomotor retardation with speech problems, right cryptorchidia and an isolated, idiopathic, congenital growth hormone deficiency that had been treated with recombinant somatropine since the age of three. The clinical examination revealed alternating hypo and hyper-pigmented maculae with linear distribution on the limbs and in "twirls" on the trunk following Blaschko's lines. The blood karyotype was normal, the karyotype on fibroblasts of hypopigmented skin revealed trisomy 20 mosaicism. DISCUSSION: The occurrence of pigmentary mosaicism related to trisomy 20 mosaicism is exceptional. The combination of Ito hypomelanosis-type pigmentary mosaicism and delayed growth due to growth hormone deficiency has never been reported before. Our observation, unusual because of such an association, raises the question of the eventual existence of associated genes located on the chromosome 20 implied in the secretion of growth hormone and/or melanogenesis. It also underlines the interest of conducting cytogenic explorations on fibroblasts of damaged skin in the case of Ito-type pigmentary mosaicism, even if the blood karyotype is normal or in the absence of a patent phenotype abnormality.

Comparison of ultrasound and dimercaptosuccinic acid scintigraphy changes in acute pyelonephritis.

The strategy for morphological investigations in children with acute pyelonephritis (APN) remains debatable. We studied 70 children (median age 2.0 years) admitted with a first episode of pyelonephritis using a high-resolution ultrasound technique (RUS) and compared the results with 99m technetium-dimercaptosuccinic acid (DMSA) renal scintigraphy. The DMSA scan was abnormal in 62 children (89%). However, using a high-frequency transducer we found abnormal sonogram changes in 61 children (87%), consisting of an increased kidney volume in 42, and/or a thickening of the wall of the renal pelvis in 42, and/or a focal hyper- or hypoechogenicity in 36, and/or a diffuse hyperechogenicity in 31 children. Micturating cystourethrography was performed in all children, revealing vesicoureteral reflux (VUR) in 22 (31%). Among those children with VUR, 4 had a normal DMSA scan, 2 an abnormal RUS, and 2 a normal DMSA scan and RUS. Our data suggest that B-mode RUS performed with a high-frequency transducer by a trained radiologist is nearly as sensitive as the DMSA scan in diagnosing renal involvement in children with unobstructed APN and in predicting VUR.

[Hematuria revealing primary hyperparathyroidism in a child]. / Hématurie révélatrice d'une hyperparathyroïdie primaire chez l'enfant.

BACKGROUND: Hematuria rarely reveals primary hyperparathyroidism in the child. CASE REPORT: A 10-year-old boy presented with gross hematuria and urolithiasis. Biological findings showed hypercalcemia and hypercalciuria. Initially, parathormone plasma levels remained within normal range, but raised after a few months and at that time, ultrasound scan examination showed a parathyroid adenoma. Nevertheless, two surgical explorations were necessary to cure hypercalcemia and hyperparathyroidism. Histological aspects of parathyroid adenoma were not found on pathological examination. CONCLUSION: Symptomatic hypercalciuria may be the first symptom revealing primary hyperparathyroidism in children. Surgical treatment remains difficult in such a case.