Archives De Pediatrie最新文献

The urological complications of junctional epidermolysis bullosa (JEB) are difficult to manage because of the fragility of the skin and mucosa, and are not the subject of any recommendations. We report on 2 cases of chronic lower urinary tract dysfunction causing chronic kidney disease, treated by bladder enlargement with sigmoidocystoplasty and Mitrofanoff appendicovesicostomy after failure of medical treatment and suprapubic catheterisation several times a day. Follow-up was favourable in both children, with kidney function preservation and significant improvement of quality of life.

Background: Epilepsy is the main complication of febrile status epilepticus (FSE) in children. The association between FSE and the development of hippocampal sclerosis (HS) and mesial temporal lobe epilepsy (MTLE) is controversial.

Objectives: This study primarily aimed to evaluate the role of magnetic resonance imaging (MRI) in diffusion-weighted imaging (DWI) sequence during the acute phase of FSE in predicting long-term development of HS and epilepsy. The secondary objective was to assess the value of arterial spin labeling (ASL) perfusion MRI in the acute phase of FSE.

Methods and settings: Patients with a first episode of FSE between 6 months and 5 years of age who underwent early MRI were retrospectively included. MRI analysis focused on the DWI signal and the presence of perfusion abnormalities on ASL imaging. We also examined the first electroencephalogram (EEG) during the acute phase. Long-term follow-up analysis assessed the occurrence of HS on MRI scans and the development of epilepsy.

Results: A total of 15 children were included. The presence of restricted diffusion areas on MRIs performed in the acute phase after FSE was significantly associated with the later development of HS. However, the association between restricted diffusion areas on early MRIs and the development of pharmaco-resistant epilepsy on follow-up, found in 3 patients, was not statistically significant. There was a trend for an association between early ASL perfusion MRI changes and EEG findings when both examinations were performed closely.

Conclusion: Early DWI-MRI seems to play a major role in the prognostic evaluation of FSE in children. It may help to determine hippocampal involvement and assess the risk of subsequent HS. However, the study data are insufficient to conclude on the association between diffusion abnormalities and the development of MTLE. Although ASL perfusion may provide additional insight, more data are needed.

Introduction: Type 1 diabetes (T1D), particularly diabetic ketoacidosis (DKA), is believed to induce a prothrombotic state, mainly due to activation of the coagulation system, platelets, and relative hypofibrinolysis.

Observations and discussion: A central venous catheter (CVC) can be necessary in cases of severe inaugural DKA in the pediatric intensive care unit (PICU). Children with a CVC are at increased risk of thrombosis, particularly before the age of three. Following a literature review and our experience in Toulouse (4 cases) of CVC-related thrombosis, we propose that CVC placement should be avoided as much as possible and alternative venous access should be preferred to minimize the risk of thrombosis.

Conclusion: If CVC placement cannot be avoided, preventive anticoagulation should be considered, but further studies are needed to establish a proper protocol.

Subacute sclerosing panencephalitis (SSPE), a progressive inflammatory neurodegenerative disease of the central nervous system caused by the measles virus, is experiencing a significant resurgence following recent measles epidemics. This study aimed to investigate the pathology of SSPE in early childhood and determine its clinical, electrophysiological, and radiological characteristics.

Materials and methods: This retrospective, descriptive study was conducted at the Pediatric Department of Nafissa Hamoud University Hospital in Algiers. The study included children aged 2-6 years who were hospitalized for subacute neurological disorders. Data collection spanned a 3-year period from January 1, 2020, to December 31, 2022.

Results and discussion: A total of 47 patients were examined, comprising 37 boys and 10 girls. The average age was 45.9 months (range: 24-72 months). Notably, 41 (87 %) of the patients had not received measles vaccination, and 37 (80 %) had contracted the measles virus, with 31 cases (83.8 %) occurring in children under 1 year of age. SSPE manifested approximately 3 years after infection (range: 1-5 years). Clinical events primarily included myoclonic seizures (26 cases), tonic-clonic seizures (6 cases), atonic seizures (6 cases), and focal seizures (3 cases); movement disorders were observed in 25 patients, and cognitive decline in 28 patients. Encephalitis manifestations, such as alterations in consciousness or behavior and psychiatric symptoms, were frequently observed. Electroencephalograms (EEG) revealed characteristic periodic complexes in only 22 patients. Magnetic resonance imaging (MRI) scans were normal in 24 patients but showed characteristic anomalies in 21 patients, primarily consisting of white matter hyperintensity and cortico-subcortical atrophy.

Conclusion: SSPE is not rare in early childhood. The latency period between virus infection and disease onset can be very short. Clinical, electroencephalographic, and radiological signs of the disease in young children may be atypical. Genetic studies are necessary to establish a genetic predisposition to the disease. Measles vaccination remains the most effective preventive measure against SSPE.

Introduction: Vitamin D is essential for children's growth and for the prevention of rickets; moreover, it has an essential role in phosphocalcic homeostasis. In 2022, new recommendations for vitamin D supplementation were established in France. In this study, we sought to evaluate the modalities of supplementation in children up to the age of 16 years and to determine the factors that might be related to compliance with the recommendations.

Materials and methods: This was an observational descriptive study. Over two distinct periods, questionnaires were distributed at three pediatric emergency departments in Basse-Normandie (Lower Normandy) to children up to 16 years of age.

Results: Overall, 710 questionnaires were analyzed. Adherence to vitamin D supplementation among children aged 0-16 years was low, with only 17 % of children following the supplementation recommendations, but it increased to 38 % in children under 2 years old. The main factors associated with adherence to the recommendations were younger age (odds ratio [OR]: 0.35, 95 % confidence interval [CI] [0.19-0.62], p < 0.001), medical follow-up by a pediatrician (OR: 0.34, 95 % CI [0.21-0.55], p < 0.001), and a higher socioeconomic status of the parents (OR: 2.43, 95 % CI [1.23-5.16], p = 0.014).

Conclusion: Adherence to the 2022 vitamin D supplementation recommendations was low, with only 17 % of children complying. However, these data need to be verified by conducting further large-scale research to confirm the findings and identify the most effective strategies for improving long-term adherence to the recommendations.

Background: Fetal alcohol spectrum disorder (FASD) is the leading cause of non-genetic intellectual disabilities. Many healthcare professionals in France have benefited from initial and continuing training programs on this public health theme. The objective of our study is to describe and compare knowledge of FASD among health professionals in two different french regions with a health prevention and information system (Indian Ocean (IO)) or without (Nouvelle-Aquitaine (NA)).

Methods and settings: A free and anonymous electronic survey related to perinatality, childhood and adolescence was sent by email or social networks to various health professionals in the IO and NA regions. Responses were analyzed by comparing the function and affiliation of healthcare professionals to each region.

Results: We obtained 193 replies from the IO and 265 replies from NA. On the one hand, 79 % of healthcare professionals reported receiving training on FASD, and most of them were aware that there is no threshold of maternal alcohol intake without risk to the fetus. It appears that 91 % of them are aware of the cognitive-behavioral disorders associated with the diagnosis of FASD. On the other hand, barely 19 % were aware that maternal age is a risk factor, and only 39 % had knowledge of the three cardinal signs of FAS dysmorphia. Only 30 % described a neurological anomaly related to the diagnosis. Regarding alcohol-related neurodevelopmental disorders, 30 % wrongly believed that growth retardation and facial dysmorphia were necessary for diagnosis. Data analysis reveals more variability in inter-professional knowledge in the region without a platform. While there is no direct significant difference between regions regarding knowledge of the FASD, among the NA respondents, only 32 % reported that paternal alcohol consumption was a risk factor, whereas 51 % of the IO respondents were already aware of it.

Conclusion: This study points out the heterogeneity of healthcare professionals' knowledge of FASD within each region. It highlights the challenge of informing, training and orienting mothers and their children in an appropriate manner. The establishment of diagnostic platforms seems essential to provide early, optimised and adapted care in response to this particular public health problem.

Objective: Our aim was to assess the use of a low FODMAP (fermentable oligosaccharides, disaccharides, monosaccharides and polyols) diet in children with different types of functional abdominal pain disorders (FAPD) and to identify predictive factors of response to this diet.

Methods: This was a multicenter, experimental, uncontrolled, prospective trial. Patients with irritable bowel syndrome (IBS), functional dyspepsia, and functional abdominal pain followed a low FODMAP diet for 2 weeks. We collected data on abdominal pain before and after the diet. Patients who showed a ≥ 50 % reduction in abdominal pain frequency were considered responders.

Results: A total of 48 patients with FAPD participated in this trial. They all showed a significant decrease in the frequency (p < 0.05) and intensity (p < 0.05) of abdominal pain after the diet. We considered 41.7 % of patients to be responders. Among children with functional dyspepsia, 66.6 % responded to the diet, and so did 71.4 % of those with IBS. On the contrary, 71.8 % of children with functional abdominal pain were non-responders, and this diagnosis was considered a predictive factor of poor response to the diet (OR: 9.87, CI [1.52; 63.97], p = 0.016).

Conclusion: In children with FAPD, a diagnosis of functional abdominal pain is a predictive factor of poor response to a low FODMAP diet. Better results were achieved with this diet in children with IBS or functional dyspepsia.

Hutchinson-Gilford syndrome, also known as progeria, is a rare genetic disorder that causes premature and accelerated ageing from the neonatal period. The disease is caused by de novo mutations in the LMNA gene. Patients present with a range of symptoms, including skin, bone, joint and cardiac disorders, as well as characteristic facial dysmorphia. Intracranial hypertension is not a known symptom of this disease. To the best of our knowledge, no case of a patient with Hutchinson-Gilford syndrome presenting with intracranial hypertension without a traumatic context has been reported in the literature to date. This report presents the case of a child diagnosed with Hutchinson-Gilford syndrome who presented with intracranial hypertension at the age of three years, with no secondary cause identified. Long-term treatment with acetazolamide was required to control the intracranial hypertension. We hypothesise that the intracranial hypertension may be related to vascular abnormalities observed in Hutchinson-Gilford syndrome, which may cause a venous drainage dysfunction. To support the hypothesis of a non-random association between intracranial hypertension and Hutchinson-Gilford syndrome, further reports of similar cases in children with the syndrome are required.

Background: Minimally invasive surgery seems particularly suited to adolescents in view of the cosmetic improvements it provides. This study reports on our first experience of single-incision laparoscopic surgery for adjustable gastric banding (SILS-AGB) using a single-trocar approach and evaluates its safety and efficacy as compared with conventional four-trocar laparoscopy.

Methods: The data of adolescent patients who underwent SILS-AGB or conventional laparoscopy for adjustable gastric banding (CL-AGB) between 2014 and 2019 in our center were reviewed. Pre- and postoperative data on weight- and obesity-associated comorbidities were used to assess the efficacy of the surgical intervention. Perioperative and postoperative data on the duration of surgery, use of analgesics, hospital stay, and complications were compared between the SILS and CL groups along with an assessment of scar quality.

Results: Overall, 12 patients, with a mean body mass index (BMI) of 43.3 kg/m^-² (37.0-55.5) at surgery were included in the SILS-AGB group and compared with 14 patients who underwent classic laparoscopy (CL-AGB group) and had an initial BMI of 39.5 kg/m^-² (32.0-49.8). Median surgery time was 82 min (55-140) in the CL-AGB group and 106 min (75-159) in the SILS-AGB group (p = 0.04). Postoperative recovery was better in the SILS-AGB group with a mean duration of level-3 intravenous analgesia of 1.8 ± 0.4 days (vs. 2.4 ± 0.6 days, p = 0.02) and a median duration of hospitalization of 2 days (2-3) versus 3 days (2-5) (p = 0.0005). Mid-term follow-up showed equivalent efficacy in terms of weight loss, with a mean BMI at 12 months of 38.5 ± 6.0 kg m^-1kg m^-1² in both groups, and resolution of hyperinsulinism (92.3 % before surgery vs. 48 % at 6 months).

Conclusion: SILS for AGB placement appears to be as safe and effective as CL despite a slightly longer operative time. SILS was associated with faster recovery and better cosmetic results with a single scar.

Background: Abusive Head Trauma (AHT) is a leading cause of morbidity and mortality in infants requiring rapid neuroimaging performance and prognostic rapid diagnosis. The Pittsburgh Infant Brain Injury Score (PIBIS) clinical prediction rule (CPR) was derived to identify infants most likely to present brain injury, whose diagnosis would benefit from head CT. Our study aimed to externally validate the PIBIS CPR in a pediatric French population.

Methods: A retrospective study was conducted in a French pediatric emergency department between 2015 and 2017. We included all consecutive infants who underwent a neurological imaging. Medical data were collected, and PIBIS score was determined, both retrospectively.

Results: We included 129 infants among which 33 cases (including 20 with a diagnosis of AHT). The sensitivity and specificity of the PIBIS CPR were 75.8 % (95 % CI 57.7-88.9) and 61.4 % (51.0-71.2) and negative and positive predictive values 88.1 % (77.8-94.7) and 40.3 % (33.0-48.2). Among the 20 infants with a diagnosis of AHT, 19 (95.0 %) were correctly identified by the PIBIS CPR.

Conclusion: Our external validation study found a lower diagnostic value of the PIBIS CPR than in the original study. This argues for adding biomarkers to improve its performance, notably in the context of suspected AHT.