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This prospective study aimed to analyze determinants that can influence bone mineral density evolution in childhood acute leukemia survivors. Patients included were selected from the long-term follow-up LEA cohort and had dual energy radiograph absorptiometry scan between 10 and 18 years and after the age of 18. All scans were centrally reviewed. Bone mineral density was measured at the lumbar spine, femoral neck, total hip, and whole body, and expressed as z-score. Eighty-nine patients (female 39, lymphoblastic leukemia 68, relapse 25, hematopoietic stem cell transplantation 44, and mean age 15.4 and 20.1 years at the first and second scans, respectively) were studied. The first and second scan z-scores were significantly correlated ( P < 10 -3 ). Mean femoral neck and total hip z-scores improved significantly between the first and second scans, whereas no significant evolution occurred at the lumbar spine and whole-body level. On the second evaluation, 14.6% of patients had z-score <-2 at the lumbar spine and 4.3% at the femoral neck level. Gender, type of leukemia, transplantation, relapse, cumulative corticosteroid doses, or growth hormone deficiency did not have any significant impact on z-score variation. Younger age at diagnosis (≤8.5 years) proved an unfavorable risk factor for z-score evolution at the lumbar spine ( P = 0.041); the trend did not reach statistical significance for metabolic syndrome ( P = 0.054). At the femoral neck, both were associated with unfavorable z-score evolution ( P = 0.003 and 0.025, respectively). Patients treated at a younger age and those with metabolic syndrome seem to be at higher risk of bone mineral density decline and should benefit from specific interventions., Competing Interests: The authors declare that they have no conflicts of interest with regard to the content of this report., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the European Hematology Association.)

Introduction: Our objectives were to assess the quality of life (QoL) of parents of childhood leukemia survivors compared with population norms and to identify the determinants of parents' long-term QoL., Methods: Parents of minors who had survived childhood leukemia participating in the French LEA cohort (Leucémie de l'Enfant et de l'Adolescent-French Childhood Cancer Survivor Study for Leukemia) were asked to complete the French version of the WHOQOL-BREF. Results were compared with age- and sex-matched values from a French reference population. Parents' and survivors' characteristics likely to be associated with QoL, long after the child's leukemia diagnosis, were explored using multivariate analysis., Results: We included 487 parents (mean age 42.9 ± 6.0 years, mean follow-up time from diagnosis 7.3 ± 3.3 years). Compared with the reference population, scores for physical health and social relationships for parents of childhood leukemia survivors were significantly lower (P < 0.001, effect size = 0.24 and P < 0.001, effect size = 0.29, respectively) contrary to scores for psychological health which were significantly higher (P < 0.001, effect size = 0.29). Even if health- and cancer-related characteristics were associated with parents' QoL in some dimensions, the only factor associated with each of the three dimensions (social relationships, physical health, and psychological) in the multivariate analysis was the parent's financial situation., Conclusions: Long after leukemia diagnosis, the parents reported lower scores in the physical health and social relationship domains. Despite the difficulties of actually influencing socioeconomic characteristics, it is important to consider the social situation of each family in the long-term care of survivors and their families., (© 2020 Wiley Periodicals, Inc.)

Objective: To evaluate the association between medical and social environmental factors and the risk of repeating a grade in childhood leukemia survivors., Study Design: A cross-sectional study of childhood leukemia survivors, recruited through the LEA cohort (Leucémie de l'Enfant et de l'Adolescent [French Childhood Cancer Survivor Study for Leukemia]) in 2014. An adjusted logistic regression model was used to identify variables linked to repeating a grade after the diagnosis among the survivors, and the rates of repeating a grade were compared between the survivors and their siblings using a multilevel logistic regression model., Results: The mean age at inclusion of the 855 participants was 16.2 ± 7.0 years, and the mean duration of follow-up from diagnosis to evaluation was 10.2 ± 6.2 years. After disease onset, 244 patients (28.5%) repeated a grade, with a median interval of 4 years (IQR, 2-8 years). Independent factors associated with repeating a grade were male sex (OR, 1.78; 95% CI, 1.21-2.60), adolescence (OR, 2.70; 95% CI, 1.63-4.48), educational support during the treatment period (OR, 3.79; 95% CI, 2.45-5.88), low parental education level (OR, 2.493; 95% CI, 1.657-3.750), and household financial difficulties (OR, 2.62; 95% CI, 1.607-4.28). Compared with siblings, survivors were at greater risk of repeating a grade (OR, 1.87; 95% CI, 1.48-2.35)., Conclusions: The most vulnerable patients seemed to be adolescents and those with parents of low socioeconomic status. Improving the schooling career of leukemia survivors will require that the medical community more carefully consider the social status of patients., (Copyright © 2018 Elsevier Inc. All rights reserved.)

The prevalence of the metabolic syndrome among adults from the French LEA childhood acute leukemia survivors' cohort was prospectively evaluated considering the type of anti-leukemic treatment received, and compared with that of controls. The metabolic profile of these patients was compared with that of controls. A total of 3203 patients from a French volunteer cohort were age- and sex-matched 3:1 to 1025 leukemia survivors (in both cohorts, mean age: 24.4 years; females: 51%). Metabolic syndrome was defined according to the National Cholesterol Education Program's Adult Treatment Panel III criteria. Metabolic syndrome was found in 10.3% of patients (mean follow-up duration: 16.3±0.2 years) and 4.5% of controls, (OR=2.49; P <0.001). Patients transplanted with total body irradiation presented the highest risk (OR=6.26; P <0.001); the other treatment groups also showed a higher risk than controls, including patients treated with chemotherapy only. Odd Ratios were 1.68 ( P =0.005) after chemotherapy only, 2.32 ( P =0.002) after chemotherapy and cranial irradiation, and 2.18 ( P =0.057) in patients transplanted without irradiation. Total body irradiation recipients with metabolic syndrome displayed a unique profile compared with controls: smaller waist circumference (91 vs 99.6 cm; P =0.01), and increased triglyceride levels (3.99 vs 1.5 mmol/L; P <0.001), fasting glucose levels (6.2 vs 5.6 mmol/L; P =0.049), and systolic blood pressure (137.9 vs 132.8 mmHg; P =0.005). By contrast, cranial irradiation recipients with metabolic syndrome had a larger waist circumference (109 vs 99.6 cm; P =0.007) than controls. Regardless of the anti-leukemic treatment, metabolic syndrome risk was higher among childhood leukemia survivors. Its presentation differed depending on the treatment type, thus suggesting a divergent pathophysiology. This study is registered at clinicaltrials.gov identifier: 01756599 ., (Copyright© 2018 Ferrata Storti Foundation.)

Objective: To gain insight into how primary immunodeficiencies (PIDs) affect children's health status and quality of life., Study Design: The French Reference Center for PIDs conducted a prospective multicenter cohort that enrolled participants who met all criteria: patients included in the French Reference Center for PIDs registry, children younger than18 years, and living in France. Participants were asked to complete both a health questionnaire and a health-related quality of life (HR-QoL) questionnaire. A severity score was assigned to each health condition: grade 1 (mild) to grade 4 (life-threatening). HR-QoL in children was compared with age- and sex-matched French norms., Results: Among 1047 eligible children, 656 were included in the study, and 117 had undergone hematopoietic stem cell transplantation; 40% experienced at least one grade 4 condition, and 83% experienced at least one grade 3 or 4 condition. Compared with the French norms, children with PID scored significantly lower for most HR-QoL domains. Low HR-QoL scores were associated strongly with burden of poor conditions., Conclusions: Our results quantify the magnitude of conditions in children with PID and demonstrate that the deleterious health effects borne by patients already are evident in childhood. These results emphasize the need to closely monitor this vulnerable population and establish multidisciplinary healthcare teams from childhood., Trial Registration: ClinicalTrials.gov: NCT02868333 and EudraCT 2012-A0033-35., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Most children with primary immunodeficiencies (PIDs) now reach adulthood. However, few studies have evaluated their health status and health-related quality of life (HRQoL)., Objective: To investigate long-term morbidity, the French Reference Center for PIDs initiated a prospective multicenter cohort: the French Childhood Immune Deficiency Long-term Cohort. The data collected were used to assess the physical health condition of patients who reached adulthood and the effect on their quality of life., Methods: Patients were asked to complete health status questionnaires. A severity score (grade 1 [mild] to grade 4 [life-threatening]) was assigned to each health condition. The HRQoL of patients was compared with age- and sex-matched French normal values by using the 36-item Short-Form Survey (SF-36) HRQoL questionnaire., Results: Among 329 participants, the mean age at evaluation was 27.6 years, with a 21-year mean follow-up after diagnosis; 43% reported at least 1 grade 4 health condition, and 86% reported at least 1 grade 3 (severe) or 4 health condition. Twenty-five (7.6%) patients had been treated for cancer. Compared with the French normal values, adults with PIDs scored significantly lower for all HRQoL domains. HRQoL was strongly associated with the burden of health conditions. The association with grade 4 or grade 3-4 health conditions was highly significant for all physical and mental domains., Conclusion: Adults with PIDs diagnosed during childhood experienced a heavy burden of health conditions, which affected their HRQoL. Our results emphasize the need to closely monitor this vulnerable population., (Copyright © 2016 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Background: It is commonly assumed that a progressive sagging of the eyebrow occurs with the facial aging process. Only a few studies have analyzed this modification, and the findings are disparate and inconclusive. This study, based on reproducible and validated data, aimed to quantify the modifications of eyebrow position that may occur with aging. Also analyzed were the effects of these eyebrow position changes on the upper eyelid and on frontal muscle activity., Methods: The study included 95 Caucasian adults divided in three groups: 20 to 39, 40 to 59, and 60 to 79 years of age. Photographic portraits were made using a standardized photograph protocol. Eyebrow position was evaluated from the intercanthal line to four points along the upper margin of the eyebrow. The upper eyelid height, the lid sulcus height, and the upper iris coverage were measured. Severity of the forehead lines was analyzed using a validated scale. Reliability and validity of all measures were controlled beforehand., Results: No significant differences were found when comparing eyebrow height above the medial canthus, the pupil, the lateral sclero-corneal limbus, and the lateral canthus between groups (p > 0.5). In addition, results suggested an increase of dermatochalasis when eyebrow height decreased. Also demonstrated was a significant increase of frontal muscle activity with aging., Conclusions: In contrast to conventional descriptions, this study demonstrated the stability of the eyebrow position with aging. This results from a physiological state of muscular compensation and may help explain the questionable results of eyebrow surgery.

Objective: The objectives of this study are to assess the quality of life (QoL) of siblings of childhood leukemia survivors in comparison with population controls and to identify determinants of sibling's QoL., Methods: The nearest-aged siblings (8-17 years) of minor CLS participating in the French LEA cohort (Childhood and Adolescent Leukemia), at the Marseilles center, were included. Siblings' QoL was self-reported using the 'Vécu et Santé Perçue de l'Adolescent et l'enfant' questionnaire. Results were compared with those obtained for age-matched and sex-matched French controls subjects. Characteristics likely to be associated with siblings' QoL (sibling's and survivor's sociodemographic and health-related and cancer-related characteristics) were explored through multivariate analysis., Results: Fifty-one siblings participated (mean age 12.7 ± 2.8 years, mean follow-up duration from diagnosis to evaluation 8.8 ± 2.5 years). They reported a significantly higher perception of QoL compared with the general population regarding psychological domains, while reporting a lower perception regarding social domains. In multivariate analysis, older age at diagnosis for both siblings and survivors was risk factor for impaired psychological QoL. An elevated leukemia burden index was linked with lower scores in self-esteem dimension, whereas having at least one sequelae for the survivor was linked with better scores in psychological well-being dimension. Low or middle affluence and older sibling's age at diagnosis were risk factors for impaired social QoL. Maximal R(2) was 30%., Conclusions: Minor siblings of CLS reported a relatively good QoL, particularly in psychological domains. Given the low proportion of QoL variability explained, other contributing factors (e.g., family functioning) must be explored., (Copyright © 2014 John Wiley & Sons, Ltd.)