In high‐risk neuroblastoma (HR‐NB), the clinical significance of long‐term minimal residual disease (MRD) monitoring using quantitative reverse transcription‐polymerase chain reaction (qRT‐PCR) for ...neuroblastoma mRNAs has not been investigated. We report long‐term MRD follow‐ups of four patients with HR‐NB throughout the disease (diagnosis, remission, and relapse) and treatment course (chemotherapy, autologous and allogeneic stem cell transplantation, and donor lymphocyte and natural killer cell infusions). The results showed the stability of mRNA marker expression after different treatments and demonstrated their validity to predict relapse and assess therapeutic response. This opens up the possibility of investigating the utility of long‐term molecular monitoring of MRD in prospective multicenter studies.
Background
Apheresis is a major challenge in peripheral stem cell collection from low‐weight children with cancer. Comparisons between the new apheresis device Optia (TerumoBCT) and the earlier COBE ...Spectra (CaridianBCT) have been performed in adults but not in low‐weight children. The objective was to compare the performance of these two devices in small children.
Study Design and Methods
In this retrospective study, all patients were reviewed weighing less than 15 kg undergoing stem cell collection using the Optia device between April 2011 and April 2012. They were paired on weight in a 3:1 ratio with patients whose cells had been collected with the COBE Spectra since 2006.
Results
Six patients were treated with the Optia and were matched with 18 patients treated with the Spectra. No side effects occurred. Collection efficiency (CE) was similar between the two groups (50% vs. 47%), but CD34 cell blood clearance was lower with the Optia (0.4 mL/min/kg vs. 0.6 mL/min/kg, p < 0.01). Platelet (PLT) loss and hemoglobin (Hb) loss were significantly reduced with the Optia (respectively, 32% vs. 54%, p < 0.01; and 1.4 g/dL vs. 2.9 g/dL, p < 0.01). Apheresis duration was increased with the Optia (159 min vs. 134 min, p < 0.05). The cell product harvested with the Optia had a lower volume and lower hematocrit, but similar white blood cell and PLT content.
Conclusion
Compared with the Spectra, the Optia allows similar CE with a reduced PLT and Hb loss but with a longer duration.
Hematopoietic progenitor cells‐apheresis (HPC‐A) collection is now a routine procedure for autologous hematopoietic stem cell transplantation. Here we present our 25 years' experience of HPC‐A ...collection in children weighing 8 kg or less, with a focus on the evolution of our standard operating procedures, and the safety limits for these young patients, in the Pediatric Apheresis Unit of Clermont‐Ferrand University Hospital (France). Fifteen children weighing 8 kg or less underwent 26 HPC‐A collections over 25 years. Median CD34+ cell yield by leukapheresis was 4.4 106/kg. No procedure‐related complications were encountered during or after the collection. No patient had profound thrombocytopenia or anemia that needed post‐collection transfusions. Our experience in pediatric oncology patients who underwent HPC‐A collections shows that this procedure can be performed even in the smallest of children with no increase in toxicity provided all precautions are taken to ensure that the procedure is carried out under the ideal conditions.
The objective of this study was to evaluate muscular metabolic function in children with inactive juvenile idiopathic arthritis (JIA).
Fifteen children with inactive JIA and fifteen healthy controls ...were matched by sex, biological age, and Tanner stage. Participants completed a submaximal incremental exercise test to determine their fat and carbohydrate oxidation rates.
Between the two groups, heart rate values and carbohydrate oxidation rates were the same, regardless of the relative intensity of exercise. Lipid oxidation rates were lower in JIA patients, regardless of the percentage of VO
peak (
< 0.05). Respiratory exchange ratios beyond 50% of VO
peak were higher in patients with JIA (
< 0.05). Respective maximal fat oxidation rates (MFO) for controls and children with JIA were 218.7 ± 92.2 vs. 157.5 ± 65.9 mg ⋅ min
(
= 0.03) and 4.9 ± 1.9 vs. 3.4 ± 1.2 mg ⋅ min
⋅ kg
(
= 0.04). There was no difference between the two groups in heart rate, percentage of VO
peak, or power of exercise to achieve MFO. Controls reached their MFO at an exercise power significantly higher than did JIA subjects (42.8 ± 16.8 and 31.9 ± 9.8 W,
= 0.004).
Children with JIA show metabolic disturbance during exercise, even when the disease is considered inactive. This disturbance is seen in a lower lipid oxidation rate during submaximal exercise.
Leukemia is the most common cancer in pediatrics, with many late effects such as higher risk of dyslipidemia, insulin resistance, obesity, and metabolic syndrome. The objective of this work was to ...investigate substrate oxidation during submaximal exercise in survivors of childhood acute leukemia.
A total of 20 leukemia survivors and 20 healthy children were matched by sex, age, and Tanner stage. They all took a submaximal incremental exercise test to determine fat and carbohydrate oxidation rates.
Cardiorespiratory fitness was significantly lower in leukemia survivors, with lower relative VO
peaks (
< 0.001), lower heart rate values (
= 0.02), and lower exercise power (
= 0.012), whereas rest metabolism and body mass index did not differ between the two groups. During exercise, upward of heart rate relative to VO
peak was significantly higher (
< 0.001) in childhood leukemia survivors. We found lower carbohydrate and fat oxidation rates (
= 0.07) in leukemia survivors compared with healthy children, and also a significantly lower relative maximal fat oxidation rate (
= 0.014).
Despite impaired physical fitness and metabolic response to exercise, childhood leukemia survivors remained sensitive to physical activity interventions, and could readily adapt to submaximal exercise intensity.
Neuroblastoma (NB) is the most common type of extracranial solid tumor in children with a high prevalence in toddlers. For childhood cancer survivors, preservation of reproductive potential is an ...important factor for quality of life. The optimization of NB minimal residual disease (MRD) detection in testicular tissue is crucial to evaluate the risk of malignant cell reintroduction. The first step in the present study was to assess the accuracy of reverse transcription-quantitative polymerase chain reaction (RT-qPCR) to detect tyrosine hydroxylase (
), paired-like homeobox 2b (
) and doublecortin (
) mRNA expression in frozen/thawed testicular tissues of patients with non-obstructive azoospermia (NOA) contaminated (
model) with an increasing number of IMR-32 and SK-N-SH NB cells. Testicular tissues were frozen by slow or snap freezing. The second step was to determine the expression levels of these markers in testicular samples from 4 pre-pubertal males (2 with stage IV NB and 2 with non-NB malignancy). The yield of extracted RNA was similar in testicular samples frozen by slow or snap freezing. In the
model,
and
transcripts were detected in uncontaminated testicular tissues, whereas
mRNA was not detected. There was a strong positive association between the number of NB cells used for contamination and
transcript levels. For IMR-32 and SK-N-SH NB cell lines, specificity and sensitivity rates of detection were 100% for
following
contamination with 10 tumor cells. In testicular samples from pre-pubertal males with and without NB,
mRNA expression was not observed, but high expression levels of
and
mRNA were detected, which were similar to expression detected in the
model. Among the markers used in blood and bone marrow for NB MRD studies, the detection of
transcripts by RT-qPCR may provide an accurate assessment of NB cells in testicular tissues from males who require fertility preservation.
Hematopoietic progenitor cells‐apheresis (HPC‐A) collection is now a routine procedure for autologous hematopoietic stem cell transplantation. Here we present our 25 years' experience of HPC‐A ...collection in children weighing 8 kg or less, with a focus on the evolution of our standard operating procedures, and the safety limits for these young patients, in the Pediatric Apheresis Unit of Clermont‐Ferrand University Hospital (France). Fifteen children weighing 8 kg or less underwent 26 HPC‐A collections over 25 years. Median CD34+ cell yield by leukapheresis was 4.4 106/kg. No procedure‐related complications were encountered during or after the collection. No patient had profound thrombocytopenia or anemia that needed post‐collection transfusions. Our experience in pediatric oncology patients who underwent HPC‐A collections shows that this procedure can be performed even in the smallest of children with no increase in toxicity provided all precautions are taken to ensure that the procedure is carried out under the ideal conditions.
Les cancers des adolescents et jeunes adultes présentent des spécificités épidémiologiques particulières. L’amélioration de leur survie doit s’accompagner d’une prise en considération accrue des ...effets indésirables des traitements, parmi lesquels la possible diminution de la fertilité. L’objectif de l’étude était de décrire l’accès à la préservation de la fertilité de ces patients au CHU de Clermont-Ferrand sur une période de trois ans.
Au cours de cette étude descriptive rétrospective, différentes données sociodémographiques et cliniques ont été collectées.
Cent cinquante nouveaux cas de cancers ont été diagnostiqués chez les patients de 15–24 ans. Quarante-quatre pour cent ont bénéficié d’au moins une consultation au centre d’étude et de conservation des œufs et du sperme humains, 29 % chez les filles et 58 % chez les garçons (p<0,001). Le nombre de cas n’ayant pas abouti à une préservation de la fertilité était significativement plus élevé chez les filles que chez les garçons (p=0,005). La préservation de la fertilité a été réalisée majoritairement par cryoconservation de tissu ovarien chez les adolescentes, d’ovocytes chez les jeunes femmes et de sperme chez les garçons.
Nous avons pu observer des disparités dans l’accès à la préservation de la fertilité selon le sexe. Malgré l’existence de recommandations, des progrès restent à faire. La mise en place de plateformes clinicobiologiques devrait permettre une meilleure sensibilisation des patients et professionnels de santé, et ainsi de promouvoir l’accès aux techniques de préservation de la fertilité des jeunes patients atteints de cancers.
Cancers of adolescents and young adults have particular epidemiological specificities. The improvement in their survival should be accompanied by an increased consideration of the treatments’ side effects, among which the potential decrease in fertility. The objective of the study was to describe the access to fertility preservation of these patients at the University Hospital of Clermont-Ferrand over a period of 3 years.
During this retrospective descriptive study, various socio-demographic and clinical data were collected.
One hundred and fifty new cases of cancers were diagnosed in patients aged 15 to 24 years. Forty-four percent received at least one fertility consultation, 29 % for girls and 58 % for boys (P<0.001). The number of cases that did not result in fertility preservation was significantly higher for girls than boys (P=0.005). Fertility preservation was mainly achieved by cryopreservation of ovarian tissue in female adolescents, ovocytes in young women and sperm in boys.
We observed sex disparities in access to fertility preservation. Despite the existence of recommendations, progress remains to be made. The establishment of clinico-biological platforms should allow a better awareness of patients and professionals, and thus promote access to fertility preservation techniques for young patients with cancer.
Background/objectives
Survival rates in children diagnosed with malignant brain tumors exceed 70%. A higher risk of dyslipidemia, central obesity, and insulin resistance has been reported among these ...children. We investigated substrate utilization during submaximal exercise.
Design/methods
Ten brain tumor survivors and 10 healthy children were matched by sex, age, and Tanner stage. Participants completed a submaximal incremental exercise test to determine their fat and carbohydrate oxidation rates.
Results
The relative oxygen volume (VO2) peak was significantly higher in the control group than in the survivors of childhood brain tumors (43.3 ± 11.9 and 32.4 ± 10.2 mL/kg /min, P = .04). At the same relative exercise intensity, there was no difference in the carbohydrate or lipid oxidation rate between the two groups, or in the maximal fat oxidation (MFO) rate, or in the heart rate or percentage of VO2 peak to reach MFO. Healthy children achieved MFO at significantly higher muscular power than did brain tumor survivors (47.9 ± 20.8 and 21.8 ± 9.6 W, P = .003).
Conclusion
Because child brain tumor survivors are less physically fit than healthy children, and substrate utilization during submaximal exercise is not different, physical activity should be promoted for child brain tumor survivors.
This study aims to assess the functional outcome of children treated with elastic stable intramedullary nailing (ESIN) for displaced proximal humerus fractures using the short version of the ...disabilities of the arm, shoulder, and hand outcome questionnaire (Quick DASH). Fifty-eight consecutive children with displaced proximal humerus fractures were treated with ESIN. Fifty-two children (89.7%) were available for follow-up and responded to the questionnaire after hardware removal. Average age at time of injury was 11.1±2.8 years (range, 4–15.9). Among the 52 patients available for evaluation, 37 had a Quick DASH score of 0 (71.1%), seven a score of 2.3 (13.5%), four a score of 4.5 (7.7%), and four a score of 6.5 (7.7%). Shoulder and elbow ranges of motion were comparable with the noninjured side. No skin irritation or local infections were observed. There was no radiographic evidence of delayed union, refracture, hardware migration, or secondary displacement. Mean follow-up was 18.3±8.3 months (range, 6–39.5). Our study reports good functional outcomes in children with closed isolated fractures, both physeal and metaphyseal, of the proximal humerus treated with ESIN. The use of a standardized rating scale is recommended to homogeneously compare functional outcome and may facilitate the comparison of clinical outcome in different patient populations.
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