1,721,067 research outputs found
Essential thrombocythaemia in children: is a treatment needed?
The myeloproliferative disorder, essential thrombocythaemia (ET), is extremely rare in children. In adults, thrombosis is the most common complication whereas a low number of children develop thrombosis and/or haemorrhages. Diagnosis of ET is often difficult, but identifying ET from other causes of thrombocytosis is essential, otherwise therapy may be ineffective as the wrong disease will be treated. Only anecdotal experiences have been published with regard to the treatment of paediatric ET. A watch-and-wait strategy seems appropriate in asymptomatic cases and low-dose aspirin should be used to reduce microvascular disturbances. Anagrelide or IFNs may be considered as first-line, and hydroxyurea as second-line therapy. Anagrelide may become the treatment of choice for ET in children if a lack of leukaemogenic potential is confirmed
Preparazione di anticorpi monoclonali contro una nuova linea di neuroblastoma infantile.
Essential thrombocythemia in children and adolescents
This paper reviews the features of pediatric essential thrombocythemia (ET). ET is a rare disease in children, challenging pediatric and adult hematologists alike. The current WHO classification acknowledges classical Philadelphia‐negative MPNs and defines diagnostic criteria, mainly encompassing adult cases. The presence of one of three driver mutations (JAK2V617F, CALR, and MPL mutations) represent the proof of clonality typical of ET. Pediatric ET cases are thus usually confronted by adult approaches. These can fit only some patients, because only 25–40% of cases present one of the driver mutations. The diagnosis of hereditary, familial thrombocytosis and the exclusion of reactive/secondary thrombocytosis must be part of the diagnostic process in children and can clarify most of the negative cases. Still, many children present a clinical, histological picture of ET, with a molecular triple wild‐type status. Moreover, prognosis seems more benign, at least within the first few decades of follow‐up. Thrombotic events are rare, and only minor hemorrhages are ordinarily observed. As per the management, the need to control symptoms must be balanced with the collateral effects of lifelong drug therapy. We conclude that these differences concert a compelling case for a very careful therapeutic approach and advocate for the importance of further cooperative studies
The Moderating Effect of Parenting on Adaptation of Children with Leukemia
Parents’ attitudes and practices may support the children’s reactions to treatments for
leukaemia and their general adjustment. This study has two aims: to explore parenting depending on
the child’s age and to develop and test a model on how family processes influence the psycho-social
development of children with leukaemia. Patients were 118 leukemic children and their parents
recruited at the Haematology–Oncologic Clinic of the Department of Paediatrics, University of Padua.
All parents were Caucasian with a mean age of 37.39 years (SD = 6.03). Children’s mean age was
5.89 years (SD = 4.21). After the signature of the informed consent, the parents were interviewed
using the EFI-C from which we derived Parenting dimension and three parental perceptions on the
child’s factors. One year later, the clinical psychologist interviewed again parents using the Vineland
Adaptive Behavior Scales (VABS). The analyses revealed the presence of a significant difference in
parenting by the child’s age: Infants required a higher and more intensive parenting. The child’s
coping with medical procedures at the second week after the diagnosis, controlled for parenting effect,
impacted upon the child’s adaptation one-year post diagnosis. Specific intervention programmes are
proposed in order to help children more at risk just after the diagnosis of developmental delays
Pediatric patients treated for leukemia back to school: A mixed-method analysis of narratives about daily life and illness experience
In the last few years, more children and adolescents healed from leukemia go back to their daily life, even if they can show some psycho-social difficulties. The study adopted semi-structured interviews and a mixed-method approach to examine the narratives of 75 children and adolescents about their return to school post 2-years treatment for leukemia. The aims are to collect their illness experiences, to understand how they feel about school and daily routines and to identify the best socio-demographic and illness predictors of a good re-adaptation to school and daily life. The results show that by increasing age and when the pediatric patient have received a hematopoietic stem cell transplantation, at the stop-therapy time, her/his perception about relationships at school and academic performance decrease, especially if his/her feelings about the disease and follow-up visits are negative
Psychological wellbeing in adolescents with leukaemia: A comparative study with typical development peers
There is still little research on psychological wellbeing, life satisfaction and reported problems in preadolescents and adolescents under therapy for leukaemia, and also little research comparing them with their healthy peers. The present study aimed to analyse the life satisfaction, hope, psychological wellbeing and reported problems’ intensity in 60 patients aged 8–18 during the first year of therapy, to identify those more at risk and to compare their reports with matched healthy peers. A battery of self-reported questionnaires was administered during hospitalisation or day hospital admissions post 6 months and post 12 months from the diagnosis. Younger patients (aged 8–13 years) were more at risk than older ones in their problems’ intensity and psychological symptoms; females and Acute Myeloid Leukaemia patients reported lower current life satisfaction perceptions; hope was associated with lower depression symptoms and mood problems. Healthy peers have a better perception of current life, but reported a lower hope score, more anxiety symptoms and more cognitive problems than patients. The first 6 months were more critical for patients’ psychological health. Basing on these empirical data, the inclusion of mental health care professionals or supportive psychotherapy into the treatment is recognized as extremely useful
Going Beyond Counting First Authors in Author Co-citation Analysis
The present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
The chimeric transcript NPM-ALK is a useful marker for the study of minimal residual disease in anaplastic large cell lymphomas
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