[Erythrapheresis in children with sickle cell disease, experience of a paediatric apheresis unit]. / Érythraphérèses chez l'enfant drépanocytaire, expérience d'une unité d'aphérèse pédiatrique.

Erythrocyte exchanges on cell separators can be used in children with sickle cell disease and are effective in lowering the level of haemoglobin S. Of the 938 aphereses performed in 2020 in our unit, we observed a low rate of failure of procedures and few complications. Ninety-six percent of erythraphereses were performed in the context of chronic exchange programs, in more than 80% of cases for cerebral vasculopathy or after the occurrence of ischemic strokes. Less than 4% of the procedures were performed for specific indications (preparation for cholecystectomy most often). The vascular access is rarely an obstacle to the realisation of the apheresis. In case of insufficient venous capital, installing an arteriovenous fistula may be considered. Depending on the child's weight, haemoglobin level, and the severity of the sickle cell anaemia, precautions may be necessary when priming the procedure. Nurses experienced in paediatric apheresis and a good medical knowledge of sickle cell disease allowed us to use this technique from the age of 3years and the weight of 15kg.

[Perception and assessment of pain by caregiver and adolescent with sickle cell disease: The impact of the patient's anxiety]. / Perception et évaluation de la douleur par le soignant et l'adolescent drépanocytaire : impact de l'anxiété du patient.

Pain is the main symptom in sickle cell disease and is a major issue in its management. Its complexity often makes assessment difficult for both patients and caregivers. This study looks for a link between anxiety in children with sickle cell disease at the beginning of their hospital stay and the difficulties experienced by caregivers to assess their pain. Forty teenagers hospitalized for a vaso-occlusive crisis were included in this prospective study. To determine which patients were "difficult to assess," a self-assessment of pain combined with a nursing assessment were proposed to patients (NS [numerical scale] and FLACC [Face Legs Activity Cry Consolability]). Feedback from specialized physicians was collected. In this study, no objective criteria allowed us to determine whether patients were "difficult to assess." At the beginning of the hospital stay, self-assessment for pain and nurse assessment with the NS matched. In this context, the FLACC scale did not assist in determining the pain score accurately. Patients identified as difficult to assess by physicians are more anxious than others.

[Severe acute pancreatitis in children receiving asparaginase: multicenter retrospective study]. / Pancréatites aiguës sévères à l'asparaginase chez l'enfant: étude rétrospective multicentrique.

UNLABELLED: Asparaginase is frequently used in the treatment of lymphoblastic malignancies in children and is a major cause of drug-induced acute pancreatitis. Severe cases of iatrogenic pancreatitis are uncommon but potentially lethal, and represent a diagnostic and therapeutic challenge. PATIENTS AND METHOD: We have retrospectively collected pediatric cases of severe acute pancreatitis induced by asparaginase, having occurred since January 1996 in participating centers from France and Belgium. RESULTS: Eleven patients, between four and 15 years old, have been included. Pancreatitis has been observed in all treatment phases, after 6 to 21 doses of asparaginase, 2 to 16 days after the last injection. Circulatory collapse (5/11), insulin-dependent diabetes (6/11) and pancreatic pseudokysts (7/11) were the major complications. Non-surgical treatment mainly included digestive rest, broad-spectrum antibiotic therapy and prolonged use of morphine. Asparaginase has been eventually reintroduced in three cases, and has caused a recurrence of pancreatitis in two of them. CONCLUSION: Intensive supportive management should enable a favourable outcome in most cases of acute pancreatitis induced by asparaginase in children. There is no way to predict the occurrence of this adverse event. Re-use of asparaginase should probably be ruled out.

[Development of psychological and intellectual performance in transplanted sickle cell disease patients: a prospective study from pretransplant period to 5 years after HSCT]. / Développement psycho-intellectuel des enfants drépanocytaires ayant reçu une allogreffe de cellules souches hématopoïétiques.

RATIONALE: Allogeneic hematopoietic stem-cell transplantation (HSCT) is the only curative treatment for sickle cell disease (SCD). Cerebral vasculopathy was the principal indication for transplantation. These children could present impaired neuropsychological development related to different causes, hence the value of exploring their intellectual capacities before and after transplantation. MATERIAL AND METHODS: Prospective longitudinal study from 1992 to 2006 in all transplanted SCD patients. The patients were assessed using Wechsler scales with four different indices: verbal comprehension, perceptual reasoning, working memory, and processing speed (PSI), providing a full-scale intellectual quotient (IQ). RESULTS: Fifteen SCD patients (8 females and 7 males; mean age, 8.9 years) were evaluated before and 36 and 60 months after transplantation. All were from Africa and lived in France. All patients except 2 had experienced ischemic stroke before HSCT. The median full-scale IQ was 87, 94, and 94 before transplantation and 36 months and 60 months after HSCT, respectively. DISCUSSION: At pre-HSCT evaluation, full-scale IQ was considered as "low average". This relatively poor result could be related to impairment of PSI, which reflects frequent graphic and motor abnormalities related to the previous stroke experienced by almost all patients. At 3 years after HSCT, all indices including IQ had increased. Only the PSI had decreased, this observation being potentially related to previous stroke and to the depression frequently experienced by the transplant recipient patient after the acute phase, when the disease is cured. At 5 years after HSCT, the median full-scale IQ was stable and the PSI had increased. CONCLUSION: At the end of follow-up, the patients improved their physical and psychological well-being. This allowed them to build projects for the future and to manifest the desire of becoming an adult. Bone marrow transplantation in this cohort of children with SCD and severe cerebral vasculopathy is associated with improved performance as measured by the Wechsler scale.