The Use of Psychotropic Medication in Pediatric Oncology for Acute Psychological and Psychiatric Problems: Balancing Risks and Benefits.

Severe acute behavioral and emotional problems represent one of the most serious treatment-related adverse effects for children and adolescents who have cancer. The critical and severe nature of these symptoms often makes necessary the use of psychotropic drugs. A working group composed of experts in multiple disciplines had the task of creating an agreement regarding a management plan for severe acute behavioral and emotional problems (SABEPs) in children and adolescents treated for cancer. To obtain global information on the use of psychotropic drugs in pediatric oncology, the working group first developed and mailed a 15-item questionnaire to many Italian pediatric oncology centers. Overall, an evident lack of knowledge and education regarding the use of psychotropic medications for the treatment of SABEPs was found. Thus, by referring to an adapted version of the Delphi method of consensus and standard methods for the elaboration of clinical questions (PICOs), the working group elaborated evidence-based recommendations for psychotropic drugs in the pediatric oncology setting. Furthermore, based on a thorough multivariate analysis of needs and difficulties, a comprehensive management flow was developed to optimize therapeutic interventions, which allows more accurate and efficient matching of the acute needs of patients while guiding treatment options.

Psychosocial assessment of families caring for a child with acute lymphoblastic leukemia, epilepsy or asthma: Psychosocial risk as network of interacting symptoms.

The purpose of this study is to assess psychosocial risk across several pediatric medical conditions and test the hypothesis that different severe or chronic pediatric illnesses are characterized by disease specific enhanced psychosocial risk and that risk is driven by disease specific connectivity and interdependencies among various domains of psychosocial function using the Psychosocial Assessment Tool (PAT). In a multicenter prospective cohort study of 195 patients, aged 5-12, 90 diagnosed with acute lymphoblastic leukemia (ALL), 42 with epilepsy and 63 with asthma, parents completed the PAT2.0 or the PAT2.0 generic version. Multivariate analysis was performed with disease as factor and age as covariate. Graph theory and network analysis was employed to study the connectivity and interdependencies among subscales of the PAT while data-driven cluster analysis was used to test whether common patterns of risk exist among the various diseases. Using a network modelling approach analysis, we observed unique patterns of interconnected domains of psychosocial factors. Each pathology was characterized by different interdependencies among the most central and most connected domains. Furthermore, data-driven cluster analysis resulted in two clusters: patients with ALL (89%) mostly belonged to cluster 1, while patients with epilepsy and asthma belonged primarily to cluster 2 (83% and 82% respectively). In sum, implementing a network approach improves our comprehension concerning the character of the problems central to the development of psychosocial difficulties. Therapy directed at problems related to the most central domain(s) constitutes the more rational one because such an approach will inevitably carry over to other domains that depend on the more central function.

Needs and expectations of adolescent in-patients: the experience of Gaslini Children's Hospital.

BACKGROUND: Adolescents affected by a severe disease who undergo high-risk treatment may experience stress, pain, extreme frustration, depression, and anger. In this large spectrum of emotions, several situations must be considered carefully. To improve coping and quality of services offered to adolescents in paediatric hospitals, we elaborated a semi-structured interview and a short questionnaire (Quality of life-adolescent-Istituto Giannina Gaslini, QoL-adol-IGG scale) to investigate the quality of life, the needs and expectations of adolescent in-patients. METHODS: The study sample includes 117 in-patients aged between 10 and 20 years coming from several Italian regions who were admitted to the G. Gaslini Research Children's Hospital for a period of 10 days or more, regardless of the disease. The QoL-adol-IGG scale was administered - after obtaining informed consent from the children and their parents - in one single encounter by trained psychologist. The distribution of the answers was evaluated after stratification by patient's age, gender, area of residence, and clinical diagnosis. Continuous data were compared using the Kruskal-Wallis, while the χ2-test was used for categorical data. RESULTS: Seventy-two percent had difficulty practicing normal daily activities during their stay in the hospital, not only because of the disease (40%), but also because of the poor organization within the hospital, the lack of proposals/activities and space for spare time, and the limited access to technologies. Adolescents ≥15 years were more frequently dissatisfied than youngsters concerning the access to Internet and other technologies, the possibility to make new friends and to take part in social activities. CONCLUSION: The results of this study, which evaluated the hospital's organization, but also psychological functioning in a representative sample of patients, may contribute to optimize internal procedures of clinical departments where adolescent in-patients are present, on the basis of their requests and taking into account their age.

Rituximab-based immunosuppression for autoimmune haemolytic anaemia in infants.

We report a case series of four infants with severe autoimmune haemolytic anaemia (AIHA) who responded to treatment with rituximab and cyclosporine after having failed first line therapy with high-dose steroid (prednisolone 4-8 mg/kg/d). Rituximab was started at 11-90 d from onset due to continued haemolysis; three infants also received cyclosporine A. Three of four infants reached complete response, defined as normal haemoglobin, reticulocytes and negative indices of haemolysis, at 7-21 months from diagnosis. In long-term follow-up two infants remained disease-free with normal immunology, one had undefined immunodeficiency and one had autoimmune lymphoproliferative syndrome.

Health emigration: a challenge in paediatric oncology.

For the past 10 years European states have experienced an increasing flow of emigrating families from developing countries seeking better medical care for their sick children. For Italian paediatricians this has become a new challenge, considering the cultural differences in customs regarding the illness. The onset of a life-threatening disease has a strong impact on the family. The natural bond between the patient and the family must be preserved and strengthened; parents should be encouraged to entertain their child during hospitalization. The inclusion of art therapy may facilitate communication, especially for children of a different language and culture. This approach can help medical staff to understand better both the child's and the parents' anxieties and feelings. This article discusses facets of the now well-known phenomenon of ;health emigration', that is, when a family searches abroad in the hope of finding the most advanced medical treatment possible for their sick child.

Small FVIII gene rearrangements in 18 hemophilia A patients: five novel mutations.

Hemophilia A (HA) is a disorder caused by mutations of the FVIII gene, which is located on the tip of the long arm of the X chromosome. In a cohort of 18 unrelated Italian patients affected with HA of varying severity, we performed mutational screening of the gene by denaturing high-performance liquid chromatography (DHPLC) and direct sequencing of abnormal peaks. We identified five novel mutations and 9 previously reported DNA alterations. Two of the 9 previously reported alterations were each common to 3 unrelated patients. Six different mutations were characterized as missense alterations, while 8 were non-missense mutations. Among the new gene alterations, one created a stop codon, one consisted of an out-of frame deletion, and one was a splice-site mutation. The last two were missense alterations. In an attempt to better understand the causative effect of the mutations and the clinical variability of the patients, we investigated the consequences of each missense mutation and visualized the effect of the amino acid change on structural FVIII models.