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Una ragazzina di 11 anni presenta un nodulo cutaneo del viso a rapida crescita. Sia il quadro clinico che l'aspetto istologico non permettono una diagnosi differenziale certa tra linfoma B maligno e pseudolinfoma. L'osservazione clinica protratta per 30 mesi dopo l'asportazione chirurgica non mette in evidenza una recidiva locale o a distanza parlando quindi a favore di una neoformazione benigna della cute. Un follow up meticoloso è indispensabile in caso di lesioni cutanee di incerta interpretazione. [ABSTRACT FROM AUTHOR]

The Indoor Environmental Quality (IEQ) combines thermal, visual, acoustic, and air-quality conditions in indoor environments and affects occupants' health, well-being, and comfort. Performing continuous monitoring to assess IEQ is increasingly proving to be important, also due to the large amount of time that people spend in closed spaces. In the present study, the design, development, and metrological characterization of a low-cost multi-sensor device is presented. The device is part of a wider system, hereafter referred to as PROMET&O (PROactive Monitoring for indoor EnvironmenTal quality & cOmfort), that also includes a questionnaire for the collection of occupants' feedback on comfort perception and a dashboard to show end users all monitored data. The PROMET&O multi-sensor monitors the quality conditions of indoor environments thanks to a set of low-cost sensors that measure air temperature, relative humidity, illuminance, sound pressure level, carbon monoxide, carbon dioxide, nitrogen dioxide, particulate matter, volatile organic compounds, and formaldehyde. The device architecture is described, and the design criteria related to measurement requirements are highlighted. Particular attention is paid to the calibration of the device to ensure the metrological traceability of the measurements. Calibration procedures, based on the comparison to reference standards and following commonly employed or ad hoc developed technical procedures, were defined and applied to the bare sensors of air temperature and relative humidity, carbon dioxide, illuminance, sound pressure level, particulate matter, and formaldehyde. The next calibration phase in the laboratory will be aimed at analyzing the mutual influences of the assembled multi-sensor hardware components and refining the calibration functions.

Cranial cruciate ligament (CrCL) rupture is one of the most common orthopaedic conditions in veterinary medicine. CrCL plays a fundamental role in the stability and biomechanics of the femoral-tibio-patellar joint, and its incorrect functionality severely impacts on the quality of life of patients. In dogs, the structural weakening of this joint due to the progressive degeneration of the ligament is the most accredited etiopathogenetic hypothesis in relation to the dog signalment (breed, sex and age) and the stifle joint conformation. In humans, this injury is often traumatic and generally occurs during sporting activities. CrCL rupture can be managed conservatively or surgically, and decisions regarding treatment are due to numerous factors: the patient's age and health, the degree of stifle instability, and cost. Physiotherapy protocols play an important role in rehabilitation, with similar goals in humans and dogs: pain management, physiological articular range of motion recovery, periarticular and core muscle strengthening, and proprioceptive deficit correction. Physiotherapy, even if often neglected in veterinary medicine, is mandatory for the recovery of the correct functionality of the injured limb and for the return to normal daily and sporting activities.

The purpose of the study was to examine safety and efficacy of non-activated Protein C (PC) supplementation in our cohort of septic pediatric hematological patients. We conducted a retrospective study of 22 septic patients receiving human plasma-derived PC concentrate from 2008 to 2015 at our Pediatric Oncology Center (Bari, Italy). The Surviving sepsis campaign definitions for sepsis, severe sepsis and septic shock were used to define the patients' septic status. For each patient, we calculated Lansky performance status scale (LPSS) and a risk score defined the Hematologic risk score (HRS) that we created in 2007. Patients were defined as High risk for severe sepsis/septic shock in case of HRS>3. HRS<3 identified low risk patients. Baseline serum PC levels, PC administration dosage and duration and days until a 20% improvement in LPSS. Observed baseline serum PC levels (bPC) blood concentrations ranged from 31 to 80%. Patients received PC supplementation in case of low age-related bPC levels or >10% PC concentration decrease within 12 hours from the first evaluation. All patients received 80 U/kg/day PC, intravenously, every twenty-four hours. No drug-related adverse event was observed. The observed sepsis-related mortality rate in our cohort was 9%. PC supplementation in our cohort appeared to be safe, and, probably due to prompt PC administration, we observed an overall mortality that was much lower than expected mortality in cancer severe septic patients.

Patients with ataxia-telangiectasia (A-T) have an increased risk of developing malignancies and are prone to severe early or late toxicity owing to chemotherapy. Leukemia and lymphoma account for about 85% of malignancies, but solid tumors have also been reported. We describe an unusual case of an 8-year-old child affected by A-T, who presented a primary hepatic B-cell non-Hodgkin lymphoma, treated with reduced doses of R-CHOP cycles plus rituximab. Three years later, the patient developed hepatoblastoma as a second malignancy. This case clearly emphasizes the need for intensive monitoring of A-T patients for early signs of malignancy and the opportunity to consider specific and modified regimens of chemotherapy.

Metastases in the bone marrow (BM) are grim prognostic factors in patients with neuroblastoma (NB). In spite of extensive analysis of primary tumor cells from high- and low-risk NB patients, a characterization of freshly isolated BM-infiltrating metastatic NB cells is still lacking. Our aim was to identify proteins specifically expressed by metastatic NB cells, that may be relevant for prognostic and therapeutic purposes. Sixty-six Italian children over 18 months of age, diagnosed with stage 4 NB, were included in the study. Metastatic NB cells were freshly isolated from patients' BM by positive immunomagnetic bead manipulation using anti-GD2 monoclonal antibody. Gene expression profiles were compared with those obtained from archived NB primary tumors from patients with 5 y-follow-up. After validation by RT-qPCR, expression/secretion of the proteins encoded by the up-regulated genes in the BM-infiltrating NB cells was evaluated by flow cytometry and ELISA. Compared to primary tumor cells, BM-infiltrating NB cells down-modulated the expression of CX3CL1, AGT, ATP1A2 mRNAs, whereas they up-regulated several genes commonly expressed by various lineages of BM resident cells. BM-infiltrating NB cells expressed indeed the proteins encoded by the top-ranked genes, S100A8 and A9 (calprotectin), CD177 and CD3, and secreted the CXCL7 chemokine. BM-infiltrating NB cells also expressed CD271 and HLA-G. We have identified proteins specifically expressed by BM-infiltrating NB cells. Among them, calprotectin, a potent inflammatory protein, and HLA-G, endowed with tolerogenic properties facilitating tumor escape from host immune response, may represent novel biomarkers and/or targets for therapeutic intervention in high-risk NB patients.

Purpose: To describe treatment, clinical course, and survival of a cohort of Italian patients with neuroblastoma., Patients and Methods: The study includes data from 2,216 children (age 0 to 14 years) diagnosed between 1979 and 2005. Overall survival (OS) was analyzed by clinical and biologic features at presentation and periods of diagnosis: 1979 to 1984, 1985 to 1991, 1992 to 1998, and 1999 to 2005. The relative risk of second malignant neoplasm (SMN) was assessed by the standardized incidence ratio (SIR), with the Italian population selected as referent., Results: Yearly patient accrual increased over time from 58 to 102. Patients age 0 to 17 months represented 45.6% of the total population, and their incidence increased over time from 36.5% to 48.5%. The incidence of stage 1 patients increased over time from 5.8% to 23.2%. A total of 898 patients (40.5%) developed disease progression or relapse, 19 patients developed SMN, and two patients developed myelodysplasia. The cumulative risk of SMN at 20 years was 7.1%, for an SIR of 8.4 (95% CI, 5.1 to 13.2). A total of 858 patients (39%) died (779 of disease, 71 of toxicity, six of SMN, and two of tumor-unrelated surgical complications). Ten-year OS was 55.3% (95% CI, 53.0% to 57.6%) and increased over time from 34.9% to 65.0%; it was significantly better for females and patients age 0 to 17 months at diagnosis, with extra-abdominal primary, and stage 1 and 2 disease. OS improved significantly over time in stage 1 and 3 patients. In patients with stage 4 disease, the improvement occurred between the first and second time cohorts (6.7% v 23.5%), but not afterward., Conclusion: The outcome of children with neuroblastoma has progressively improved. Long-term survivors bear a significant risk of SMN.

Background: Lymphoblastic lymphoma (LBL) is the second most frequent lymphoma subtype in childhood. It is commonly treated according to therapy strategies for lymphoblastic leukemia., Methods: The AIEOP LNH-92 protocol was a modified LSA2-L2 therapy used for both T- and B-cell precursor LBL and included Induction, Consolidation, and Maintenance treatment with a total duration of 11 and 24 months for stages I and II, stages III and IV disease, respectively., Results: Fifty-five eligible patients were enrolled, 40 males and 15 females, with a median age of 8 years. Complete remission was achieved in 93% of the cases. With a median follow-up of 9 years the event-free survival (EFS) was 69% and overall survival 72%. EFS of localized disease was 100%. The most frequent grades III and IV toxicity was hematologic and hepatic (elevated transaminases) toxicity. No toxic death nor second tumor were observed. Outcome was comparable to most concomitant international protocols for LBL, but inferior to recent trials that included reinduction treatment or a higher intensity therapy for high stage disease., Conclusions: AIEOP LNH92 protocol demonstrated similar efficacy compared to contemporary regimens, with limited toxicity. Nevertheless, an intensified treatment is warranted for high stage disease.

Febrile Neutropenia (FN) is a complication of chemotherapy in childhood cancer and at the same time secondary deficit of Protein C (PC) is often present during sepsis in children with cancer. In this study we have compared the clinical outcome of two different groups. At the onset of FN during chemotherapy the first group (patients with a secondary deficit of PC) received Protein C Concentrate (PCC) replacement while the other group without PC deficiency received only symptomatic therapies. We report that PC replacement could shorten duration of FN and improve the clinical outcome. The administration of PCC was safe and without any complications.

Here we report a case of administration of defibrotide in an 11 months old infant with hepatic veno-occlusive disease during chemotherapy for nephroblastoma. He presented with abdominal distension, a weight gain of 15%, ascites, hepatomegaly with right upper quadrant pain, thrombocytopenia and hypertransaminasemia. Despite therapy, his clinical conditions aggravated, and, therefore intravenous administration of defibrotide on a compassionate-use basis was started. The dosage was 15 mg/kg/day in 4 divided doses, which was increased gradually (in 3 days) to 40 mg/kg/day in 4 divided doses. Defibrotide proved safe and effective in resolving clinical symptoms and normalizing serological findings in the syndrome.

Background: Irinotecan (CPT-11) is a novel antineoplastic agent that takes effect by inhibiting topoisomerase I. The Italian Soft Tissue Sarcoma (STS) Committee performed a multiinstitutional Phase II study to evaluate its effect on STS., Methods: Over a 2-year period between 2002 and 2004, 32 heavily pretreated patients were administered 60-minute infusions of irinotecan at 20 mg/m2/day, for 5 days a week, for 2 consecutive weeks. The courses were repeated every 4 weeks for at least 2 courses, unless there were signs of toxicity or disease progression. Thirty patients, 13 with peripheral primitive neuroectodermal tumor (PNET), 12 with rhabdomyosarcoma (RMS), 3 with desmoplastic small round cell tumor (DSRCT), and 2 with other STS were evaluable for response., Results: A total of 79 cycles were delivered. The main regimen-related toxicity was diarrhea, occurring in 58% of cycles with 9 episodes graded as 3 or 4. Grade 3-4 neutropenia was recorded in 10% of cycles. The overall response rate was 23% (2 complete remissions +5 partial remissions of 30 patients), 38% for PNET and 16% for RMS. In addition, 4 minor responses were noted., Conclusions: As a single agent in the treatment of recurrent and refractory STS, irinotecan administered on a daily x5 x2 schedule revealed a noteworthy response rate in a population of heavily pretreated patients, especially in the subset of patients with PNET. Its hematologic toxicity profile warrants further investigation in association with other myelotoxic agents., (Copyright (c) 2005 American Cancer Society.)

Elevated plasma concentrations of endogenous thrombin generation markers and thrombotic events have been reported in children with leukemia. The aim of this study was to evaluate the effects of cancer and its treatment on thrombin generation (TAT levels) in children with acute lymphoblastic leukemia (ALL). The authors evaluated 32 children (23 M, 9 F) aged between 1 and 15 years (mean 6) affected by ALL (immunophenotypic subgroups: 16 common, 7 T, and 9 pre-B type). In all patients TAT levels at onset and after 5-6 doses of L-asparaginase were evaluated. TAT levels were higher in patients both at onset (13.04 +/- 10.90 ng/L) and after the 5-6 doses of L-asp (19.41 +/- 11.05 ng/L) with respect to controls (4 +/- 1 ng/L) (p < .001 and p < .001). TAT levels after 5-6 doses of L-asp were higher than those at onset (p < .001). Factorial ANOVA showed that at onset there was a significant effect of leukemia immunophenotypic subgroups upon TAT levels (p < .05) and no effect of inherited thrombotic risk factors. These results indicate that in children with ALL an important role is played by acquired thrombotic risk factors, among which the indirect cancer procoagulant activity has its importance.

In 1989 the prevalence of hepatitis B virus markers was studied by radioimmunoassay in a sample of 1,426 healthy children, 3 to 11 years old, attending kindergarten and the primary schools in a large urban area of the Apulia Region in Southern Italy, where the hepatitis B surface antigen (HBsAg) prevalence among pregnant women is 5.6%. The overall prevalence of any hepatitis B virus marker was 3.4%, increasing from 1.7% in 3- to 5-year-old children to 5% in 10- to 11-year-old children (P less than 0.002). Prevalence was not associated with the father's years of schooling (odds ratio, 1.98; confidence interval, 95% (0.9 to 4.6] or with the family size (odds ratio, 2.96; confidence interval, (0.7 to 11.8]. The overall HBsAg prevalence was 0.8, a rate that was lower than the 5.6% found in pregnant women. The finding of only 12 HBsAg-positive children of the 1,426 tested, despite 80 of them being born to HBsAg-positive carrier mothers (on the basis of the 5.6% HBsAg prevalence among pregnant women), is probably attributable to the low proportion (5%) of hepatitis B e antigen positivity among the HBsAg-positive carrier mothers in the study area. The observed low HBV infection rate in younger age groups, which confirms recent studies in other areas of Italy, appears to be the result of several factors: improved socioeconomic conditions; decreased family size; and increased use of disposable syringes in the last few years.

Selected hemostatic parameters of 23 children affected by beta-thalassemia major were studied and compared to an age- and sex-matched group. Plasma prekallikrein level was reduced in all patients, splenectomized or not. In splenectomized patients, platelet count and in vitro platelet aggregability were significantly increased and Protein C was slightly increased. The activated partial thromboplastin time was prolonged and the normotest reduced. Finally, a reduction in the plasma levels of fibrinogen and of vitamin K-dependent proteins, including the antithrombotic Protein C, was observed in nonsplenectomized patients. Our data indicate that the hemostatic system in patients with thalassemia major may be altered. The relationship between these laboratory changes and clinical manifestations remains to be established.