Ποιότητα ζωής σε εφήβους και νεαρούς ενήλικες που έχουν ιαθεί από καρκίνο στην παιδική ηλικία

Objective: The purpose of this descriptive, retrospective, comparative study was toassess the quality of life of adolescent and young adult survivors of childhood cancer,by addressing the physical, psychological, spiritual and social dimensions of theirfunctioning. At the same time, a comparison with the quality of life of other people oftheir age was attempted.Sample-Methods: The sample constituted of 45 adolescents 15 to 18 years old and58 young adults 19 to 30 years old, coming from the Oncology Department of acentral Children’s Hospital of Athens. The control group constituted from 57 HighSchool and 78 University students. The core hypothesis of the study was: “The qualityof life of adolescent and young adult survivors of childhood cancer is of the samelevel of other healthy people of their age”. The secondary hypotheses regarding to thedimensions of the quality of life were: a) survivors of childhood cancer present withgreater levels of anxiety, insecurity and uncertainty comparing το healthy controls, b)the alterations in the survivors’ body, because of cancer and aggressive therapy, havea negative influence on their body image, resulting to a lower self-esteem incomparison το other healthy people of their age, c) survivors have some problems intheir interpersonal, heterosexual relationships. For data collection, the followingquestionnaires and scales were used: a) Questionnaire for the Quality of Life, b) SF-36 Health Survey, c) Battle Culture-Free Self-Esteem Inventory (BCSEI), d) Liakou-Spielberger State-Trait Anxiety Inventory, e) Lazarus and Folkman Ways of Coping,f) Free Text with Personal Views about the Disease.Results: Young survivors of childhood cancer, in general terms, adapt quite well tothe new circumstances, according to the relevant results in the subscales “PhysicalFunctioning”, “GeneralHealth” and “Vitality” of SF-36. More precisely, the overallphysical functioning of young survivors was satisfying, even though in theQuestionnaire for the Quality of Life some mild problems in daily activity werenoticed. Thus, it was shown that in statistically significant difference, they got tiredmore easily than controls, while there was increased prevalence in other symptomslike insomnia, nausea or anorexia, which have the possibility to influence their dailyactivity. Moreover, increased risk for short-term and long-term side effects fromseveral systems was noticed, from mild neurological dysfunction to second cancer.More problems were identified in their psychological function. According tothe results in Battle Culture-Free Self-Esteem Inventory and Liakou-SpielbergerState-Trait Anxiety Inventory, they had increased anxiety levels and lower selfesteem,basically distorted body-image. In addition, there was statistically significantdifference between survivors and controls adolescents, in the “Social functioning”sub scale of SF-36. From their answers in the relevant questions in Questionnaire forthe Quality of Life, a certain difficulty in establishing sexual relationships wasnoticed. The results suggest that the most serious risk for the global quality of life ofyoung survivors of cancer hides in problems in psychosocial level.On the other hand, they have the remarkable capacity to perceive a positiveaspect in their cancer experience: they support they have emotionally matured, theyIVhave intensified their bond with their family, they have learnt to co-suffer with otherpeople in trouble, they have learnt to value the life generally. This prospective helpsthem keep their psychospiritual balance and allows them to perceive their quality oflife as good and comparable with that of other people of their age.Σκοπός: Σκοπός της παρούσας περιγραφικής, αναδρομικής, συγκριτικής μελέτηςήταν η εκτίμηση της ποιότητας ζωής εφήβων και νεαρών ενηλίκων που έχουν ιαθείαπό κακοήθεια στην παιδική ηλικία, σε συνάρτηση με τις επιμέρους παραμέτρους της(σωματική, ψυχολογική, πνευματική και κοινωνική). Ταυτόχρονα, επιχειρήθηκε και ησύγκριση με την ποιότητα ζωής των συνομηλίκων τους.Υλικό-Μέθοδος: Το δείγμα αποτελούσαν 45 έφηβοι ηλικίας 15 έως 18 ετών και 58νεαροί ενήλικες ηλικίας 19 έως 30 ετών και προερχόταν από την Ογκο λογική Κλινικήκεντρικού Παιδιατρικού Νοσοκομείου της πρωτεύουσας. Την ομάδα ελέγχουαποτελούσαν αντίστοιχα 57 μαθητές της Δευτεροβάθμιας και 78 σπουδαστές τηςΤριτοβάθμιας Εκπαίδευσης. Η βασική υπόθεση που καθοδήγησε τη μελέτη ήταν ηεξής: “ Η ποιότητα ζωής των εφήβων και νεαρών ενηλίκων που έχουν ιαθεί απόκαρκίνο στην παιδική ηλικία βρίσκεται στα ίδια επίπεδα με αυτή των υγιώνσυνομηλίκων τους”. Οι δευτερεύουσες υποθέσεις που αφορούσαν στις επιμέρουςπαραμέτρους της ποιότητας ζωής ήταν: α) τα άτομα αυτά βιώνουν, εν τούτοις,μακροπρόθεσμα αυξημένα επίπεδα άγχους, ανασφάλειας και αβεβαιότητας για τομέλλον, συγκριτικά με ομάδα υγιών μαρτύρων, β) οι μεταβολές που επέρχονται στοσώμα των ιαθέντων από τον καρκίνο και την επιθετική θεραπεία επηρεάζουναρνητικά την αντίληψη που έχουν για τη σωματική τους εικόνα, με αποτέλεσμα ναπαρουσιάζουν χαμηλότερη αυτοεκτίμηση, συγκριτικά με ομάδα ατόμων παρόμοιαςηλικίας, χωρίς πρόβλημα υγείας, γ) τα άτομα αυτά παρουσιάζουν περισσότεραπροβλήματα στις διαπροσωπικές, ετεροφυλικές σχέσεις, συγκριτικά με ομάδα υγιώνμαρτύρων.Για τη συλλογή των δεδομένων χρησιμοποιήθηκαν τα ακόλουθαερωτηματολόγια και κλίμακες: α) Ερωτηματολόγιο για την Ποιότητα Ζωής, β)Κλίμακα Ποιότητας Ζωής SF-36 Health Survey, γ) Κλίμακα Αντίληψης Εαυτού BattleCulture-Free Self-Esteem Inventory (BCSEI), δ) Κλάμακα Άγχους Λιάκου-Spielberger,ε) Ερωτηματολόγιο Αντιμετώπισης Στρεσογόνων Καταστάσεων Ways of Coping, στ)Ελεύθερο Κείμενο Προσωπικών Απόψεων Σχετικά με τη Νόσο.Αποτελέσματα: Τα νεαρά, ιαθέντα από καρκίνο άτομα προσαρμόζονται γενικά καλά,όπως προκύπτει από τα αντίστοιχα αποτελέσματα στις υποκλίμακες “ΣωματικήΛειτουργικότητα ”, “Γενική κατάσταση" και “Ζωτικότητα” της SF-36. Αναλυτικότερα,η συνολική σωματική λειτουργικότητα των νεαρών ιαθέντων κρίνεται ικανοποιητική11αν και διαφάνηκαν ήπιες διαταραχές σε επίπεδο καθημερινής δραστηριότητας στοΕρωτηματολόγιο για την Ποιότητα Ζωής. Έτσι, διαπιστώθηκε ότι, σε στατιστικάσημαντική διαφορά, κουράζονταν ευκολότερα από τους μάρτυρες, ενώ σημειώθηκεαυξημένη επίπτωση και σε άλλα συμπτώματα όπως αϋπνία, ναυτία ή ανορεξία, ταοποία συνολικά δύνανται να επηρεάσουν την καθημερινότητά τους.Επίσης,παρατηρήθηκε κίνδυνος για βραχυπρόθεσμες και απώτερες επιπλοκές απόδιάφορα συστήματα, από ήπιες νευρολογικές δυσλειτουργίες έως δεύτερη κακοήθεια.Περισσότερα προβλήματα διαφάνηκαν στην ψυχολογική τους λειτουργία.Σύμφωνα με τα αποτελέσματα στις κλίμακες Αντίληψης Εαυτού BCSEI και ΑγχουςΑιάκου-Spielberger, διαπιστώθηκαν αυξημένα επίπεδα άγχους και διαταραχές στηναντίληψη εαυτού, εντονότερες στην εικόνα σώματος. Επίσης, στατιστικά σημαντικήδιαφορά προέκυψε στην υποκλίμακα “Κοινωνική λειτουργικότητα ” της κλίμακας SF-36 ανάμεσα στους έφηβους ιαθέντες και μάρτυρες. Από τις απαντήσεις στιςαντίστοιχες ερωτήσεις του Ερωτηματολογίου για την Ποιότητα Ζωής, παρατηρήθηκεσχετική δυστοκία των ιαθέντων στη σύναψη ερωτικών σχέσεων. Τα αποτελέσματαυποδεικνύουν πως ο σοβαρότερος κίνδυνος για την ποιότητα ζωής τους ελοχεύει σεπροβλήματα στην ψυχοκοινωνική σφαίρα.Αξιοπρόσεκτη είναι, εν τούτοις, η ικανότητά τους να αντιλαμβάνονται μίαθετική όψη στην εμπειρία του καρκίνου: υποστηρίζουν πως ωρίμασανσυναισθηματικά, ισχυροποίησαν τους δεσμούς τους με την οικογένεια, έμαθαν νασυμπάσχουν με τους συνανθρώπους που πονούν, έμαθαν να εκτιμούν τη ζωήγενικότερα. Αυτή η θεώρηση τους βοηθά να διατηρούν την ψυχοπνευματική τουςισορροπία και τους επιτρέπει να αντιλαμβάνονται την ποιότητα ζωής τους ως καλήκαι συγκρίσιμη με των συνομηλίκων τους

Dose–Effect Relationship of Alkylating Agents on Testicular Function in Male Survivors of Childhood Lymphoma

<div><p>The purpose of our study was to assess the gonadal function in male survivors of childhood lymphoma. We studied 171 male survivors of childhood lymphoma (83 with B-cell non-Hodgkin lymphoma [B-NHL], 32 with T-cell non-Hodgkin lymphoma [T-NHL], 50 with Hodgkin lymphoma [HL], and 6 with anaplastic large-cell lymphoma [ALCL]), measuring follicle-stimulating hormone [FSH] and luteinizing hormone [LH] levels at a median age of 21.1 (17–30.4) years after a median delay of 9.3 (2–22.4) years from treatment. FSH levels were above normal range (≥10 IU/L) in 42.1% and LH levels ≥8 IU/L in only 8.9% of survivors. In multivariate analysis, only the following chemotherapeutic agents were associated with higher FSH or LH levels: cyclophosphamide (<i>P</i> < .0001, .04), lomustine (CCNU; <i>P</i> = .002, 0.04), and procarbazine (<i>P</i> < .0001, .07). No significant correlation was found between FSH or LH levels and age or pubertal status at diagnosis. Mean FSH level was significantly lower in NHL survivors treated more recently: 6 ± 5.1 IU/L in B-NHL survivors treated since 1986 versus 12.3 ± 5.4 IU/L for those treated before 1981 (<i>P</i> = .0001), and 6.8 ± 9.6 IU/L in T-NHL survivors treated since 1989 versus 9.4 ± 5.7 IU/L for those treated before 1989 (<i>P</i> = .035). In HL, mean FSH level was 12.4 ± 9.9 IU/L following procarbazine containing chemotherapy versus 3.4 ± 1.9 IU/L in the absence of procarbazine and increased significantly with the number of MOPP/OPPA (mechlorethamine, Oncovin [vincristine], procarbazine, and prednisone/Oncovin, procarbazine, and prednisone, and Adriamycin [doxorubicin]) courses received, from 6.8 ± 5.7 IU/L for 1–2 MOPP/OPPA to 12.6 ± 7.5 for 3–4 MOPP/OPPA and 19.6 ± 13.3 for more than 4 MOPP/OPPA (<i>P</i> for trend = .006). Testicular toxicity of alkylating agents on childhood lymphoma survivors is dose dependent and not correlated to diagnosis, age, or pubertal status at diagnosis.</p></div

Overexpression of the GR Riborepressor LncRNA GAS5 Results in Poor Treatment Response and Early Relapse in Childhood B-ALL

Simple Summary Although childhood acute lymphoblastic leukemia (chALL) management is considered as one of the success stories in modern clinical oncology, the increased incidence of relapse in high-risk patients and the severe toxicity/long-term health effects due to chemotherapy intensity highlight the need for further improvements in patients’ risk stratification and personalized management. Synthetic glucocorticoids (GCs) are the core agents in chALL chemotherapy, exerting their role through the nuclear glucocorticoid receptor (GR), while GAS5 lncRNA suppresses the GCs-GR axis through binding to GR’s DNA binding domain. The objective of the study was the evaluation of GAS5 prognostic utility in chALL. GAS5 overexpression was strongly associated with a higher risk for short-term relapse and poor treatment outcome, independently of patients’ clinicopathological data. Moreover, “GAS5-including” multivariate models resulted in superior risk stratification and clinical benefit for disease prognosis, supporting precision medicine decisions in chALL. Glucocorticoids (GCs) remain the cornerstone of childhood acute lymphoblastic leukemia (chALL) therapy, exerting their cytotoxic effects through binding and activating of the glucocorticoid receptor (GR). GAS5 lncRNA acts as a potent riborepressor of GR transcriptional activity, and thus targeting GAS5 in GC-treated chALL could provide further insights into GC resistance and support personalized treatment decisions. Herein, to study the clinical utility of GAS5 in chALL prognosis and chemotherapy response, GAS5 expression was quantified by RT-qPCR in bone marrow samples of chB-ALL patients at diagnosis (n = 164) and at end-of-induction (n = 109), treated with ALL-BFM protocol. Patients’ relapse and death were used as clinical end-points for survival analysis. Bootstrap analysis was performed for internal validation, and decision curve analysis assessed the clinical net benefit for chALL prognosis. Our findings demonstrated the elevated GAS5 levels in blasts of chALL patients compared to controls and the significantly higher risk for short-term relapse and poor treatment outcome of patients overexpressing GAS5, independently of their clinicopathological data. The unfavorable prognostic value of GAS5 overexpression was strongly validated in the high-risk/stem-cell transplantation subgroup. Finally, multivariate models incorporating GAS5 levels resulted in superior risk stratification and clinical benefit for chALL prognostication, supporting personalized prognosis and precision medicine decisions in chALL

Disseminated nontuberculous mycobacterial infection in a child with interferon-gamma receptor 1 deficiency

We describe the case of a 2-year-old boy with disseminated infection by a rapidly growing, poorly pathogenic mycobacterial species that belonged to the Mycobacterium fortuitum-Mycobacterium peregrinum complex. He had a severe course characterized by a poor response to treatment and recurrent lymph node abscess formation. Sequencing of the interferon-gamma receptor 1 gene (IFN gamma R1) revealed that he was homozygous for a novel null mutation, 453delT. Patients presenting with disseminated infections by rapidly growing environmental mycobacteria must be investigated for complete IFN gamma R1 deficiency. The spectrum of IFN gamma R1 genotypes associated with this immunological disorder is expanding

Immune response to influenza vaccination in children with cancer

The aim of this study was to evaluate the ability of influenza immunization to evoke a protective immune response among children with cancer. We evaluated 75 children with cancer who received influenza vaccination. Hemagglutination Inhibition Antibody titers were determined before and after vaccination. The protective rates after vaccination were 79% for H1N1, 75% for H3N2 and 59% for influenza B virus whereas the seroconversion rates were 54%, 44% and 43% respectively. The differences pre- and post-vaccination were significant regardless the method which was used: seroprotection changes, seroconversion and geometric mean titers analyses. Variables such as the pre-vaccination antibody titers, the time when the responses were measured after the vaccination, the age and the type of malignancy as well as the absolute lymphocyte count were found to be correlated with the immune response but the findings were different for each vaccine subunit. In conclusion, influenza vaccination provides protection in a remarkable proportion of pediatric cancer patients whereas this protection is more obvious against H1N1 and H3N2 compared to influenza B. The immune response after vaccination is significant and seems to be influenced by a variety of factors

Acinar cell carcinoma in childhood: A case report of a very rare tumor

Pancreatic tumors are reported rarely in childhood and represent an extremely rare entity in Pediatric Oncology. One of the least common types of pediatric pancreatic tumor is acinar cell carcinoma (ACC). We aim to present a rare case of ACC and the difficulties we faced during diagnosis and treatment

Predictive Factors for Gram-negative Versus Gram-positive Bloodstream Infections in Children With Cancer

Background: Identifying potential predictive factors for the type of bacteremia (Gram-negative vs. Gram-positive) in children with cancer would be crucial for the timely selection of the appropriate empiric antibiotic treatment. Materials and Methods: Demographic, clinical, and laboratory characteristics of children with cancer and a bacterial bloodstream infection (BSI) (February 1, 2011 to February 28, 2018) in a tertiary pediatric oncology department were retrospectively examined and were correlated with the type of isolated bacteria. Results: Among 224 monomicrobial bacterial BSI episodes, Gram-negative and Gram-positive bacteria were isolated in 110 and 114 episodes, respectively. Gram-negative bacteria were isolated significantly more frequently in girls (Gram-negative/Gram-positive ratio 1.7:1) versus boys (Gram-negative/Gram-positive ratio 0.72:1), P=0.002, in patients with previous BSI episodes (1.4:1) versus those without (0.8:1), P=0.042, and in children with hematologic malignancy (1.3:1) versus those who suffered from solid tumors (0.52:1), P=0.003. Gram-negative BSI episodes were more frequently correlated with a lower count of leukocytes, P=0.009, neutrophils, P=0.009 and platelets, P=0.002, but with significantly higher C-reactive protein (CRP) levels, P=0.049. Female sex, hematologic malignancy, and higher CRP levels remained independent risk factors for Gram-negative BSI in the multivariate analysis. Among neutropenic patients, boys with solid tumors and a recent central venous catheter placement appear to be at increased risk for Gram-positive BSI in the multivariate analysis. Conclusions: Although Gram-negative and Gram-positive BSIs are close to balance in children with cancer, Gram-negative bacteria are more likely to be isolated in girls, children with hematologic malignancies and those with higher CRP level at admission. In contrast, neutropenic boys with solid tumors and a recently placed central venous catheter may be at increased risk for Gram-positive BSI indicating probably the need for initially adding antibiotics targeting Gram-positive bacteria

Survival patterns of childhood neuroblastoma: an analysis of clinical data from Southern-Eastern European countries.

The prognosis of children with neuroblastoma (NBL) can be dismal with significant variations depending on the stage and biology of the tumor. We assessed the event-free (EFS) and overall (OS) survival using harmonized data from three Southern-Eastern European (SEE) countries. Data for 520 incident NBL cases (2009-2018) were collected from Greece, Slovenia and Russia. Kaplan-Meier curves were fitted, and EFS/OS were derived from Cox proportional models by study variables including the protocol-based risk-group (low/observation, intermediate, high). Over one-third of cases were coded in the high-risk group, of which 23 children (4.4%) received treatment with anti-ganglioside 2 (GD2) mAb. Survival rates were inferior in older (OS 5-year; 1.5-4.9 years: 61%; EFS 5-year; 1.5-4.9 years: 48%) compared to children younger than 1.5 years (OS 5-year; &lt;1.5 years: 91%; EFS 5-year; &lt;1.5 years: 78%). Predictors of poor OS included stage 4 (hazard ratio, HROS: 18.12, 95% confidence intervals, CI: 3.47-94.54), N-myc amplification (HROS: 2.16, 95% CI: 1.40-3.34), no surgical excision (HROS: 3.27, 95% CI: 1.91-5.61) and relapse/progression (HROS: 5.46, 95% CI: 3.23-9.24). Similar unfavorable EFS was found for the same subsets of patients. By contrast, treatment with anti-GD2 antibody in high-risk patients was associated with decreased risk of death or unfavorable events (HROS: 0.11, 95% CI: 0.02-0.79; HREFS: 0.19, 95% CI: 0.07-0.52). Our results confirm the outstanding prognosis of the early NBL stages, especially in children &lt;1.5 years, and the improved outcomes of the anti-GD2 treatment in high-risk patients. Ongoing high-quality clinical cancer registration is needed to ensure comparability of survival across Europe and refine our understanding of the NBL biology