Archives De Pediatrie最新文献

Background: Data are lacking for lung evaluation after hematopoietic cell stem transplantation (HSCT) in children under the age of six, as this population cannot be included in respiratory function monitoring protocols.

Methods and settings: The RESPPEDHEM cohort included individuals under the age of 18 who underwent HSCT between January 2014 and November 2017. The eligible population for this study consisted of children from the RESPEDHEM cohort, who underwent HSCT before the age of 6, were still alive in October 2023 and had pulmonary function tests (PFTs) performed more than 3 years after HSCT. The primary objective of our multicenter study was to describe long-term PFT outcomes in children who received HSCT before the age of six, as included in the RESPEDDHEM cohort. The secondary objective was to identify clinical, radiological and transplant-related factors associated with abnormal lung function. Pulmonary abnormalities were defined according to the guidelines of the American Thoracic Society (ATS), the European Respiratory Society (ERS), and Global Lung Initiative (GLI).

Results: Among the 40 children, the mean (SD) age at transplantation was 3.7 ± 1.4 years; 50 % had undergone PFT before HSCT. The last follow-up lung function test was performed at 10.2 ± 2.2 years. Twelve individuals (30 %; 95 %CI: 17-47 %) had abnormal lung function at the end of the study, based on the recent ERS/ATS technical standard on interpretive strategies for routine lung function tests. The main anomalies were obstructive defect (n=4) and restrictive defect (n=4).

Conclusion: This study is the first lung function analysis of children undergoing HSCT before the age of six. Abnormalities persist in about one-third of the population, and 42 % of these children were asymptomatic. Therefore, systematic and long-term respiratory monitoring is needed even if the absence of clinical symptoms. However, half of the cohort did not undergo pre-HSCT pulmonary function testing, which limits the ability to attribute abnormalities solely to the transplant.

Background: The clinical value of thrombophilia testing in pediatric patients remains debated, especially in the absence of clear, age-specific guidelines. We aimed to assess the real-life indications, results, and clinical impact of thrombophilia testing in a French tertiary pediatric center.

Methods: We conducted a retrospective, single-center study of all children (<18 years) who underwent hereditary thrombophilia testing at Armand Trousseau Hospital (AP-HP.Sorbonne Université) during 2019. Clinical indications, test results, thrombotic events, and their consequences for clinical management were analyzed.

Results: A total of 129 patients (median age 6.7 years; 39.5% female) were included. The five most frequent indications were hematologic malignancy (55.0%), suspected or confirmed thrombotic events (18.6%), pre-kidney transplant evaluation (6.2%), family history of thrombophilia or thrombosis (6.2%), and preoperative assessment (3.9%). At least one abnormality was identified in 29.5% of patients, but only 10 had confirmed hereditary thrombophilia: protein S deficiency (n = 1), factor V Leiden mutation (homozygous n = 2; heterozygous n = 4), and heterozygous prothrombin G20210A mutation (n = 3). Most natural anticoagulant deficiencies were observed in children with leukemia and were not retested, thus remaining unconfirmed. Eleven patients (8.5%) experienced a venous thromboembolism (VTE); ten had at least one transient risk factor. Thrombophilia testing led to a change in clinical management in five patients (3.9%). Among patients with confirmed thrombophilia, preventive counseling was inconsistently documented.

Conclusion: Thrombophilia testing in children should be carefully targeted, guided by established recommendations, and reserved for situations with clear clinical relevance. Testing should be deferred during acute illness, repeated to confirm lifelong diagnoses, and, if confirmed, accompanied by appropriate preventive counseling. Efforts should also focus on providing standardized, practical advice when a thrombophilia is identified.

Background: Mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) is an uncommon clinico-radiological entity, mostly described in children in association with viral or atypical bacterial infections. Mycoplasma pneumoniae (MP) is a rare but recognized trigger. Kawasaki disease (KD) may also present with neurological involvement.

Observation and discussion: We report the case of a 14-year-old boy with acute encephalopathy, Mycoplasma pneumoniae infection, and features of incomplete KD. Brain MRI showed a reversible lesion in the splenium of the corpus callosum consistent with MERS. The patient developed mucocutaneous signs and transient coronary artery dilatation. He improved with antibiotic and anti-inflammatory therapy.

Conclusion: This case highlights a rare overlap between MERS, Mycoplasma pneumoniae, and incomplete KD, suggesting shared inflammatory pathways. Clinicians should consider this association when encountering pediatric encephalopathy with systemic inflammatory features.

Background: Motor control of the upper limb is very important for play, personal care, and daily living activities. Losing the motor function of the hand limits the daily life activities, educational, and social participation of children with cerebral palsy (CP).

Objective: To compare the effects of constraint-induced movement therapy (CIMT) with virtual reality (CIMT+VR) use, only CIMT use, and traditional therapy (TT) on hand functions in children with hemiparetic Cerebral Palsy (h-CP).

Material and methods: Children who were h-CP would be divided into three groups randomly (TT (tradit, CIMT, CIMT+VR groups). Hand function and performance were evaluated with the Jebsen-Taylor and Moberg pick-up tests.

Results: Forty-five children participated in the present study. There were no differences in sex, affected side, age, hand function, or performance between groups. After six weeks of therapy, a difference in hand function was observed between groups. However, there were no differences in the functional performance of the hemiparetic hand after therapy.

Conclusion: Using CIMT and VR together improved hand functions, while there was no change in the functional performance of the hand.