Quality of life in children and adolescents with type 1 diabetes and coeliac disease.

Health-related quality of life (HRQOL) is an important health outcome and a well-know indicator of the long-term consequences of chronic diseases that affect the quality of life (QOL). Aim of our study was to investigate general and HRQOL of children with type 1 diabetes (T1DM) and subjects with coeliac disease (CD) compared to healthy controls. We studied 101 outpatients: 35 children with T1DM (12.8 \ub1 2.85 years, duration of T1DM 60.5 \ub1 33.4 months), 32 subjects with CD (9.60 \ub1 2.61 year, duration of CD 52.0 \ub1 47.9 months), and 34 controls children matched for age and sex. All subjects were assed using the Paediatric Quality of Life Inventory (PedsQL) Generic Core Scales to measure HRQOL with 23 items included in 4 scales. T1DM patients showed a satisfactory metabolic control HbA1c (8.06 \ub1 0.75%). Twenty-one out of 32 CD subjects showed a strict dietetic control. We demonstrated that social functioning (fx), school (fx), psychosocial health (fx), and total scale were significantly different between groups; the major concern was related to emotional (fx). Our results demonstrate that children and adolescents with chronic disease, despite a good adherence to therapy, have impairment in psychosocial health (fx). Our data disagree with common opinion that children with CD have a better adaptation and functioning. These findings contribute significant information on the effects of pediatric chronic conditions on generic QOL from the perspectives of children. It is conceivable that an immediate multidisciplinary approach to patients with T1DM can be responsible for this differences

Doxorubicin effect on myocardial metabolism as a prerequisite for subsequent development of cardiac toxicity: A translational18F-FDG PET/CT observation

The present translational study aimed to verify whether serial 18FFDG PET/CT predicts doxorubicin cardiotoxicity. Methods: Fifteen athymic mice were treated intravenously with saline (n = 5) or with 5 or 7.5 mg of doxorubicin per kilogram (n = = each) and underwent dynamic small-animal PET beforehand and afterward to estimate left ventricular (LV) metabolic rate of glucose (MRGlu). Thereafter, we retrospectively identified 69 patients who had been successfully treated with a regimen of doxorubicin, bleomycin, vinblastine, and dacarbazine for Hodgkin disease (HD) and had undergone 4 consecutive18F-FDG PET/CT scans. Volumes of interest were drawn on LV myocardium to quantify mean SUV. All patients were subsequently interviewed by telephone (median follow-up, 30 mo); 36 of them agreed to undergo electrocardiography and transthoracic echocardiography. Results: In mice, LV MRGlu was 17.9 \uc2\ub1 4.4 nmol \ue2\u80\ua2 min21 \uc3\u97 g21 at baseline. Doxorubicin selectively and dose-dependently increased this value in the standard-dose (27.9 \uc2\ub1 9 nmol \uc3\u97 min21 \uc3\u97 g-1, P < 0.05 vs. controls) and high-dose subgroups (37.2 6 7.8 nmol \uc3\u97 min21 \uc3\u97 g-1, P < 0.01 vs. controls, P < 0.05 vs. standard-dose). In HD patients, LV SUV showed a progressive increase during doxorubicin treatment that persisted at follow-up. New-onset cardiac abnormalities appeared in 11 of 36 patients (31%). In these subjects, pretherapy LV SUV was markedly lower with respect to the remaining patients (1.53 \uc2\ub1 0.9 vs. 3.34 \uc2\ub1 2.54, respectively, P < 0.01). Multivariate analysis confirmed the predictive value of baseline LV SUV for subsequent cardiac abnormalities. Conclusion: Doxorubicin dosedependently increases LV MRGlu, particularly in the presence of low baseline18F-FDG uptake. These results imply that low myocardial18F-FDG uptake before the initiation of doxorubicin chemotherapy in HD patients may predict the development of chemotherapy-induced cardiotoxicity, suggesting that prospective clinical trials are warranted to test this hypothesis

Bone sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

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The rehabilitation of children and adolescents with severe or medically complicated obesity: an ISPED expert opinion document

Severe/medically complicated obesity in childhood, and particularly in adolescence, is a real disability that requires an intensive and continuous approach which should follow the procedures and schedule of rehabilitation medicine. Given the lack of a specific document focusing on children and adolescents, the Childhood Obesity Study Group set out to explore the available evidence for the treatment of severe or medically complicated obesity and to set standards tailored to the specific context of the Italian Health Service. Through a series of meetings and electronic communications, the writing committee (selected from members of the Study Group) selected the key issues, explored the literature and produced a draft document which was submitted to the other experts until the final synthesis was approved by the group. In brief, the following issues were involved: (1) definition and epidemiology; (2) identification of common goals designed to regain functional competence and limit the progression of metabolic and psychological complications; (3) a multi-professional team approach; (4) the care setting. This paper is an expert opinion document on the rehabilitation of severe and medically complicated obesity in children and adolescents produced by experts belonging to the Childhood Obesity Study Group of the Italian Society for Pediatric Endocrinology and Diabetology (ISPED)

The sacral chordoma margin

Objective Aim of the manuscript is to discuss how to improve margins in sacral chordoma. Background Chordoma is a rare neoplasm, arising in half cases from the sacrum, with reported local failure in >50% after surgery. Methods A multidisciplinary meeting of the “Chordoma Global Consensus Group” was held in Milan in 2017, focusing on challenges in defining and achieving optimal margins in chordoma with respect to surgery, definitive particle radiation therapy (RT) and medical therapies. This review aims to report on the outcome of the consensus meeting and to provide a summary of the most recent evidence in this field. Possible new ways forward, including on-going international clinical studies, are discussed. Results En-bloc tumor-sacrum resection is the cornerstone of treatment of primary sacral chordoma, aiming to achieve negative microscopic margins. Radical definitive particle therapy seems to offer a similar outcome compared to surgery, although confirmation in comparative trials is lacking; besides there is still a certain degree of technical variability across institutions, corresponding to different fields of treatment and different tumor coverage. To address some of these questions, a prospective, randomized international study comparing surgery versus definitive high-dose RT is ongoing. Available data do not support the routine use of any medical therapy as (neo)adjuvant/cytoreductive treatment. Conclusion Given the significant influence of margins status on local control in patients with primary localized sacral chordoma, the clear definition of adequate margins and a standard local approach across institutions for both surgery and particle RT is vital for improving the management of these patients