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1.
Psychiatr Danub ; 35(Suppl 3): 42-52, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-37994060

RESUMEN

BACKGROUND: Assessment of pain in people with intellectual disability (PWID) is a difficult clinical task. Poor knowledge and confidence in assessing pain in PWID result in underestimation and undertreatment. Available resources for healthcare personnel and caregivers on pain assessment in PWID are still very limited. The aim of the study was to measure the level of knowledge and confidence in assessing pain in PWID of health and education personnel at Istituto Serafico, before and after training. SUBJECTS AND METHODS: The Istituto Serafico is a neuro-rehabilitation center caring for people with complex disabilities. Nurses, rehabilitation therapists, social health workers (SHW) and educators were invited to participate in a 4-hours theoretical and practical training. Participants were assessed through a knowledge and confidence questionnaire on pain assessment in PWID, administered before and after the training. RESULTS: 123 participants attended both the theoretical and practical sessions. Median age was 43 years (range 23-67); 89 were females and 34 males. They were 10 (8%) nurses, 9 (7%) rehabilitation therapists, 77 (63%) SHW, 27 (22%) educators. Only 7 (6%) participants (5 nurses and 2 SHW) declared to have previously received formation on pain. Participants who felt "quite confident" in assessing pain increased from 28% to 73% after the training. The median score to the 24 knowledge questions raised from 15/24 correct answers (range 6-22, 62.5%) in the pre-test to 21/24 (range 11-24, 87.5%) in the post-tests (p=0.001) Conclusions: The study highlights the great need of education programs for health and educational personnel working with PWID on pain assessment and the potential to improve knowledge and confidence through theoretical and practical training. A greater awareness of pain causes, clinical manifestations and consequences of untreated pain, could improve patient care, quality of life and rehabilitation goals.


Asunto(s)
Discapacidad Intelectual , Abuso de Sustancias por Vía Intravenosa , Masculino , Femenino , Humanos , Adulto Joven , Adulto , Persona de Mediana Edad , Anciano , Dimensión del Dolor , Discapacidad Intelectual/diagnóstico , Discapacidad Intelectual/epidemiología , Calidad de Vida , Dolor/diagnóstico
2.
FASEB J ; 35(3): e21424, 2021 03.
Artículo en Inglés | MEDLINE | ID: mdl-33609323

RESUMEN

Gorham-Stout disease (GSD) is a very rare disease characterized by increased bone erosion with angiomatous proliferation. The mechanisms underlying this disorder have not been deeply investigated. Due to its rarity, no guidelines are currently available for treatment and management of GSD. We recently evaluated the cellular alterations of the bone remodeling in patients showing that osteoclast precursors displayed increased ability to differentiate into osteoclasts and that affected osteoclasts resorb bone more actively than control cells. Moreover, osteoblasts isolated from a patient showed a defective ability to form mineralized nodules. In this paper, we investigated the molecular pathways involved in the cellular defects of GSD bone cells. For this study, we recruited nine patients and performed miRNome analysis of bone cells. Between the 178 miRNAs robustly expressed in GSD osteoclasts, significant modulation of three miRNAs (miR-1246, miR-1-3p, and miR-137-3p) involved in the regulation of osteoclast formation and activity or in the angiomatous proliferation was found in patients' cells. Interestingly, miR-1246 was also up-regulated in serum exosomes from patients. Analysis of miRNAs from patient osteoblasts suggested alteration of miR-204a-5p, miR-615-3p and miR-378a-3p regulating osteoblast function and differentiation. The resulting miRNA pattern may help to understand better the mechanisms involved in GSD and to identify new potential therapeutic targets for this rare disease.


Asunto(s)
Huesos/citología , MicroARNs/genética , Osteoclastos/metabolismo , Osteocitos/metabolismo , Osteólisis Esencial/sangre , Adolescente , Huesos/metabolismo , Diferenciación Celular/genética , Niño , Exosomas/metabolismo , Femenino , Humanos , Masculino , MicroARNs/sangre , Osteólisis Esencial/fisiopatología
3.
BMC Infect Dis ; 17(1): 302, 2017 04 24.
Artículo en Inglés | MEDLINE | ID: mdl-28438138

RESUMEN

BACKGROUND: A number of biomarkers have been studied for the diagnosis of sepsis in paediatrics, but no gold standard has been identified. Procalcitonin (PCT) was demonstrated to be an accurate biomarker for the diagnosis of sepsis in adults and showed to be promising in paediatrics. Our study reviewed the diagnostic accuracy of PCT as an early biomarker of sepsis in neonates and children with suspected sepsis. METHODS: A comprehensive literature search was carried out in Medline/Pubmed, Embase, ISI Web of Science, CINAHL and Cochrane Library, for studies assessing PCT accuracy in the diagnosis of sepsis in children and neonates with suspected sepsis. Studies in which the presence of infection had been confirmed microbiologically or classified as "probable" by chart review were included. Studies comparing patients to healthy subjects were excluded. We analysed data on neonates and children separately. Our primary outcome was the diagnostic accuracy of PCT at the cut-off of 2-2.5 ng/ml, while as secondary outcomes we analysed PCT cut-offs <2 ng/ml and >2.5 ng/ml. Pooled sensitivities and specificities were calculated by a bivariate meta-analysis and heterogeneity was graphically evaluated. RESULTS: We included 17 studies, with a total of 1408 patients (1086 neonates and 322 children). Studies on neonates with early onset sepsis (EOS) and late onset sepsis (LOS) were grouped together. In the neonatal group, we calculated a sensitivity of 0.85, confidence interval (CI) (0.76; 0.90) and specificity of 0.54, CI (0.38; 0.70) at the PCT cut-off of 2.0-2.5 ng/ml. In the paediatric group it was not possible to undertake a pooled analysis at the PCT cut-off of 2.0-2.5 ng/ml, due to the paucity of the studies. CONCLUSIONS: PCT shows a moderate accuracy for the diagnosis of sepsis in neonates with suspected sepsis at the cut-off of 2.0-2.5 ng/ml. More studies with high methodological quality are warranted, particularly in neonates, studies considering EOS and LOS separately are needed to improve specificity. TRIAL REGISTRATION: PROSPERO Identifier: CRD42016033809 . Registered 30 Jan 2016.


Asunto(s)
Calcitonina/sangre , Sepsis/diagnóstico , Biomarcadores/sangre , Niño , Preescolar , Humanos , Lactante , Recién Nacido , Estudios Prospectivos , Reproducibilidad de los Resultados , Sensibilidad y Especificidad , Sepsis/microbiología
4.
BMC Cancer ; 15: 79, 2015 Feb 21.
Artículo en Inglés | MEDLINE | ID: mdl-25886486

RESUMEN

BACKGROUND: Local control is always considered in metastatic neuroblastoma (NBL). The aim of this study is to evaluate the impact of radical surgery on survival in children over 1 year of age. METHODS: Fifty-eight patients older than 1 year of age with metastatic NBL were treated with conventional plus high-dose chemotherapy with or without addition of local radiotherapy (RT, 21Gy). Surgery was classified as radical surgery (complete resection and gross total resection) or non-radical surgery. The Kaplan-Meier method and the Cox proportional hazard model were used to calculate the probability of progression free and overall survival (PFS and OS) and for multivariate analysis. RESULTS: The 5-year PFS and OS for patients with radical surgery were 26% (95% CI 14-40%) and 38% (95% CI 23-53%) respectively, while the PFS and OS for patients without radical surgery were 33% (95% CI 10-59%) and 31% (95% CI 10-55%) (respectively, P 0.85 and P 0.42). The 5-year PFS and OS for patients who received RT were 36% (95% CI 19-53%) and 46% (95% CI 26-64%) respectively, while the 5-year PFS and OS for patients who did not receive RT were 22% (95% CI 9-38%) and 27% (95% CI 13-42%) respectively (P 0.02 for PFS). Multivariate analysis confirmed the role of well-known prognostic factors, such as the presence of MYCN amplification, age and response before high-dose chemotherapy. CONCLUSIONS: Our data suggest that the degree of resection does not influence survival in metastatic NBL patients treated with high-dose chemotherapy; local RT contributes to local disease control.


Asunto(s)
Neuroblastoma/patología , Neuroblastoma/cirugía , Adolescente , Niño , Preescolar , Humanos , Lactante , Metástasis de la Neoplasia , Estudios Retrospectivos , Factores de Riesgo , Análisis de Supervivencia , Resultado del Tratamiento
5.
Cancer ; 119(8): 1586-92, 2013 Apr 15.
Artículo en Inglés | MEDLINE | ID: mdl-23310931

RESUMEN

BACKGROUND: The optimal management of bilateral Wilms tumor (BWT) is challenging, and their survival is lower than for unilateral tumors. This report discusses a large series of BWTs treated in Italy in the last 2 decades. METHODS: This analysis concerns patients with synchronous BWT registered at Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) centers between 1990 and 2011; details on their treatment and outcome are presented and discussed. RESULTS: Ninety BWTs were registered in the AIEOP Wilms tumor database. Preoperative chemotherapy was given for a median 12 weeks before definitive tumor resection was attempted. Forty-eight percent of the patients had preservation of bilateral renal parenchyma. The proportion of bilateral nephron-sparing surgeries was not higher in the 37 patients initially given doxorubicin/vincristine/actinomycin D (32%) than in the 43 children receiving vincristine/actinomycin D alone (58%). The 4-year disease-free survival rate was 66.5% ± 5% and overall survival was 80% ± 5% for the cohort as a whole. The 4-year disease-free survival (overall survival) for 18 children with diffuse anaplasia or postchemotherapy blastemal-type tumors was 51% ± 13% (62% ± 13%), as opposed to 72% ± 3% (88% ± 4%) for 68 children with a favorable histology (log-rank P = .04 [P = .007]). CONCLUSIONS: These results provide further evidence that the optimal duration and choice of drugs for preoperative chemotherapy remain an open question. Outcome remained significantly worse for BWT than for unilateral Wilms tumor. To enable the conservative treatment of as many affected kidneys as possible, only centers with experience in BWT should manage such cases.


Asunto(s)
Neoplasias Renales/terapia , Tumor de Wilms/terapia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Dactinomicina/administración & dosificación , Supervivencia sin Enfermedad , Doxorrubicina/administración & dosificación , Femenino , Humanos , Neoplasias Renales/tratamiento farmacológico , Neoplasias Renales/radioterapia , Neoplasias Renales/cirugía , Masculino , Estudios Prospectivos , Vincristina/administración & dosificación , Tumor de Wilms/tratamiento farmacológico , Tumor de Wilms/radioterapia , Tumor de Wilms/cirugía
6.
Pediatr Blood Cancer ; 60(1): 152-5, 2013 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-22997183

RESUMEN

Long-term tunneled central venous catheters (CVC) are employed in critically ill patients. Manufacturers do not provide patient-customized devices; therefore, trimming is required for pediatric use. Scanning Electron Microscopy (SEM) coupled with energy-dispersive X-ray spectroscopy and attenuated total reflection-Fourier transform infrared spectroscopy (ATR-FTIR) was used to assess changes induced by different trimming methods on single and double lumen Hickman-Broviac catheters. Increased roughness, exposure of inorganic macroaggreagates and increase in surface inorganic charges were generated by the trimming procedure, with the scalpel producing a smoother surface compared to scissors. Trimming produces changes on the CVC surface that may influence the rate of long-term complications.


Asunto(s)
Catéteres Venosos Centrales , Catéteres Venosos Centrales/efectos adversos , Niño , Humanos , Microscopía Electrónica de Rastreo , Espectroscopía Infrarroja por Transformada de Fourier
8.
Haematologica ; 97(1): 147-50, 2012 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-21993676

RESUMEN

A nationwide questionnaire-based survey was designed to evaluate the management and prophylaxis of febrile neutropenia in pediatric patients admitted to hematology-oncology and hematopoietic stem cell transplant units. Of the 34 participating centers, 40 and 63%, respectively, continue to prescribe antibacterial and antimycotic prophylaxis in low-risk subjects and 78 and 94% in transplant patients. Approximately half of the centers prescribe a combination antibiotic regimen as first-line therapy in low-risk patients and up to 81% in high-risk patients. When initial empirical therapy fails after seven days, 63% of the centers add empirical antimycotic therapy in low-and 81% in high-risk patients. Overall management varies significantly across centers. Preventive nursing procedures are in accordance with international guidelines. This survey is the first to focus on prescribing practices in children with cancer and could help to implement practice guidelines.


Asunto(s)
Control de Enfermedades Transmisibles , Neoplasias Hematológicas/complicaciones , Antiinfecciosos/efectos adversos , Antiinfecciosos/uso terapéutico , Profilaxis Antibiótica , Niño , Enfermedades Transmisibles/tratamiento farmacológico , Enfermedades Transmisibles/etiología , Enfermedades Transmisibles/enfermería , Neoplasias Hematológicas/enfermería , Neoplasias Hematológicas/terapia , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Humanos , Encuestas y Cuestionarios
9.
J Pediatr Hematol Oncol ; 33(5): e176-9, 2011 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-21552143

RESUMEN

A 14-year-old girl with metastatic renal cell carcinoma was treated with nephrectomy, interferon, and several lines of the targeted agents sorafenib, bevacizumab, sunitinib, and everolimus, either alone or in combination. Treatment was well tolerated, but the patient developed hypothyroidism and significant hypertension with bevacizumab and sunitinib. She responded to all agents and was given radiation treatment twice at the time of symptomatic disease progression; she died 33 months from diagnosis.


Asunto(s)
Bencenosulfonatos/uso terapéutico , Carcinoma de Células Renales/tratamiento farmacológico , Carcinoma de Células Renales/secundario , Neoplasias Renales/tratamiento farmacológico , Neoplasias Renales/patología , Inhibidores de Proteínas Quinasas/uso terapéutico , Piridinas/uso terapéutico , Adolescente , Anticuerpos Monoclonales/uso terapéutico , Anticuerpos Monoclonales Humanizados , Bevacizumab , Carcinoma de Células Renales/cirugía , Terapia Combinada , Everolimus , Resultado Fatal , Femenino , Humanos , Neoplasias Renales/cirugía , Nefrectomía , Niacinamida/análogos & derivados , Compuestos de Fenilurea , Sirolimus/análogos & derivados , Sirolimus/uso terapéutico , Sorafenib
10.
Front Cell Dev Biol ; 9: 706596, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34513837

RESUMEN

Gorham-Stout disease (GSD) is a very rare syndrome displaying excessive bone erosion and vascular lesion. Due to the rarity of the disease and to the limited studies, its etiopathogenesis is not entirely known. The involvement of immune system in the progressive osteolysis was recently suggested. Indeed, extensive reciprocal interactions between the immune and skeletal systems have been demonstrated. This study aimed to evaluate alterations of immune cells in GSD. An increase of CD8+ cells and reduction of CD4+ and CD4+CD25+CD127low cells was revealed in patients. Interestingly, patients' regulatory T cells maintain the ability to respond to extracellular stimuli and to regulate osteoclastogenesis; GSD cells proliferate under aCD3/CD28 signal reaching similar levels to those observed in control culture and exert their immunomodulatory activity on effector T cells. GSD Treg cells preserved their inhibitory effects on the osteoclastogenesis. These results suggest that stimulation of Treg cells could open the way for the identification and testing of new therapeutic approaches for patients affected by GSD.

11.
Pediatr Blood Cancer ; 54(1): 55-61, 2010 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-19821523

RESUMEN

AIM: Activity and toxiciy of gefitinib in combination with topotecan and cyclophosphamide (CPA) were evaluated in a case-series of relapsed neuroblastoma (NB) patients. The in vitro activity of the combination was also assessed. PROCEDURE: Gefitinib (250 mg/day), topotecan (0.8 mg/m(2)/day), and CPA (50 mg/m(2)/day) (GTC) were administered orally for 14 consecutive days out of 28 days. Antitumor activity of gefitinib as single agent and in combination with either topotecan or CPA was assessed in a panel of NB cell lines. RESULTS: Ninety-two courses were given in 10 patients. Grade 4 neutropenia was observed in 7/92 courses (8%) and grade 4 thrombocytopenia in 8/92 (9%). Two patients had a grade 2 liver toxicity, four a grade 1/2 skin toxicity, and two a grade 1/2 diarrhea. Dose reduction of topotecan and/or CPA was required in eight patients. After four courses, three patients were in partial response (PR) and four with a stable disease (SD), while three experienced a progressive disease (PD). Time to progression (TTP) was 9 months (range, 1-27). After a median follow-up of 16 months (range 5-54), seven patients are died of disease (DOD) and three alive with disease (AWD). All but one patient discontinued oral chemotherapy because of a PD, whilst one patient stopped chemotherapy after 27 months with a SD. In vitro, gefitinib was synergistic with topotecan and additive with CPA. CONCLUSION: The GTC combination was well tolerated and the TTP was encouraging. These promising results, also supported by in vitro evidence, should be further confirmed in a phase II study.


Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Recurrencia Local de Neoplasia/tratamiento farmacológico , Neuroblastoma/tratamiento farmacológico , Terapia Recuperativa , Administración Oral , Anciano , Anciano de 80 o más Años , Ciclofosfamida/administración & dosificación , Resistencia a Antineoplásicos , Receptores ErbB/genética , Receptores ErbB/metabolismo , Femenino , Gefitinib , Humanos , Masculino , Persona de Mediana Edad , Estadificación de Neoplasias , Neuroblastoma/genética , Neuroblastoma/metabolismo , Proyectos Piloto , Pronóstico , Quinazolinas/administración & dosificación , ARN Mensajero/genética , ARN Mensajero/metabolismo , Reacción en Cadena de la Polimerasa de Transcriptasa Inversa , Tasa de Supervivencia , Topotecan/administración & dosificación , Resultado del Tratamiento
12.
Bone ; 130: 115068, 2020 01.
Artículo en Inglés | MEDLINE | ID: mdl-31525474

RESUMEN

Gorham-Stout disease (GSD) is a rare disorder characterized by progressive osteolysis and angiomatous proliferation. Since the mechanisms leading to bone loss in GSD are not completely understood, we performed histological, serum, cellular and molecular analyses of 7 patients. Increased vessels, osteoclast number and osteocyte lacunar area were revealed in patients' bone biopsies. Biochemical analysis of sera showed high levels of ICTP, Sclerostin, VEGF-A and IL-6. In vitro experiments revealed increased osteoclast differentiation and activity, and impaired mineralization ability of osteoblasts. To evaluate the involvement of systemic factors in GSD, control cells were treated with patients' sera and displayed an increase of osteoclastogenesis, bone resorption activity and a reduction of osteoblast function. Interestingly, GSD sera stimulated the vessel formation by endothelial cells EA.hy926. These results suggest that bone cell autonomous alterations with the cooperation of systemic factors are involved in massive bone loss and angiomatous proliferation observed in GSD patients.


Asunto(s)
Osteólisis Esencial , Osteólisis , Huesos , Células Endoteliales , Humanos , Osteoclastos
13.
Eur J Cancer ; 127: 52-66, 2020 03.
Artículo en Inglés | MEDLINE | ID: mdl-31986450

RESUMEN

The third multistakeholder Paediatric Strategy Forum organised by ACCELERATE and the European Medicines Agency focused on immune checkpoint inhibitors for use in combination therapy in children and adolescents. As immune checkpoint inhibitors, both as monotherapy and in combinations have shown impressive success in some adult malignancies and early phase trials in children of single agent checkpoint inhibitors have now been completed, it seemed an appropriate time to consider opportunities for paediatric studies of checkpoint inhibitors used in combination. Among paediatric patients, early clinical studies of checkpoint inhibitors used as monotherapy have demonstrated a high rate of activity, including complete responses, in Hodgkin lymphoma and hypermutant paediatric tumours. Activity has been very limited, however, in more common malignancies of childhood and adolescence. Furthermore, apart from tumour mutational burden, no other predictive biomarker for monotherapy activity in paediatric tumours has been identified. Based on these observations, there is collective agreement that there is no scientific rationale for children to be enrolled in new monotherapy trials of additional checkpoint inhibitors with the same mechanism of action of agents already studied (e.g. anti-PD1, anti-PDL1 anti-CTLA-4) unless additional scientific knowledge supporting a different approach becomes available. This shared perspective, based on scientific evidence and supported by paediatric oncology cooperative groups, should inform companies on whether a paediatric development plan is justified. This could then be proposed to regulators through the available regulatory tools. Generally, an academic-industry consensus on the scientific merits of a proposal before submission of a paediatric investigational plan would be of great benefit to determine which studies have the highest probability of generating new insights. There is already a rationale for the evaluation of combinations of checkpoint inhibitors with other agents in paediatric Hodgkin lymphoma and hypermutated tumours in view of the activity shown as single agents. In paediatric tumours where no single agent activity has been observed in multiple clinical trials of anti-PD1, anti-PDL1 and anti-CTLA-4 agents as monotherapy, combinations of checkpoint inhibitors with other treatment modalities should be explored when a scientific rationale indicates that they could be efficacious in paediatric cancers and not because these combinations are being evaluated in adults. Immunotherapy in the form of engineered proteins (e.g. monoclonal antibodies and T cell engaging agents) and cellular products (e.g. CAR T cells) has great therapeutic potential for benefit in paediatric cancer. The major challenge for developing checkpoint inhibitors for paediatric cancers is the lack of neoantigens (based on mutations) and corresponding antigen-specific T cells. Progress critically depends on understanding the immune macroenvironment and microenvironment and the ability of the adaptive immune system to recognise paediatric cancers in the absence of high neoantigen burden. Future clinical studies of checkpoint inhibitors in children need to build upon strong biological hypotheses that take into account the distinctive immunobiology of childhood cancers in comparison to that of checkpoint inhibitor responsive adult cancers.


Asunto(s)
Antineoplásicos/uso terapéutico , Desarrollo de Medicamentos , Agencias Gubernamentales/organización & administración , Inmunoterapia/métodos , Evaluación de Necesidades , Neoplasias/tratamiento farmacológico , Planificación de Atención al Paciente/organización & administración , Antígeno B7-H1/antagonistas & inhibidores , Antígeno CTLA-4/antagonistas & inhibidores , Niño , Quimioterapia Combinada , Humanos , Neoplasias/patología , Pronóstico
14.
Pediatr Blood Cancer ; 51(1): 23-8, 2008 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-18293386

RESUMEN

BACKGROUND: We evaluated an intensified chemotherapy strategy in children with Wilms tumor who relapsed with high-risk features. PROCEDURES: From January 2001 to June 2006, we treated 20 consecutive children with reinduction chemotherapy (using ifosfamide/carboplatin/etoposide in 15/20 cases), with (n = 15) or without (n = 5) subsequent high-dose chemotherapy and hematopoietic stem cell support, surgery where feasible, and radiation therapy. The median time to relapse was 10 months after nephrectomy. All but two children initially received doxorubicin as first-line therapy. RESULTS: All patients were assessed for outcome: 13 are currently alive, 12 of them in remission a median 25 months since their relapse, one with progressing tumor. The treatment was unsuccessful in eight children: the disease progressed during reinduction in three, and relapsed in five. There was one toxic death. All transplanted patients engrafted to a neutrophil count >0.5 x 10(3)/microl after a median 11 days, and to an unsustained platelet count >25,000/microl after a median of 13 days. Three-year disease-free and overall survival rates were 56 +/- 12% and 55 +/- 13%, respectively. Neither recurrence within 12 months of nephrectomy nor extra-lung recurrence negatively affected outcome. A survival advantage was demonstrated in patients without disease evidence prior to transplant. CONCLUSION: A disease-free survival rate nearing 50% is a realistic target in children with high-risk recurrent Wilms tumor. The benefit of autologous hematopoietic stem cell transplantation for consolidation deserves to be investigated in a randomized, controlled study.


Asunto(s)
Antineoplásicos/uso terapéutico , Trasplante de Células Madre Hematopoyéticas/métodos , Terapia Recuperativa/métodos , Acondicionamiento Pretrasplante/métodos , Tumor de Wilms/terapia , Antineoplásicos/toxicidad , Niño , Preescolar , Femenino , Supervivencia de Injerto , Humanos , Lactante , Italia , Masculino , Nefrectomía , Inducción de Remisión , Tasa de Supervivencia , Trasplante Autólogo , Resultado del Tratamiento , Tumor de Wilms/mortalidad
15.
Pediatr Blood Cancer ; 51(4): 475-8, 2008 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-18561180

RESUMEN

BACKGROUND: One of the most important adverse prognostic factors for adult renal cell carcinoma (RCC) is the retroperitoneal lymph node involvement. The aim of this article is to study the prognostic significance of local lymph node involvement in pediatric RCC and the role of retroperitoneal lymph node dissection (RLND) at diagnosis. PROCEDURE: The series included 16 patients with RCC and lymph nodes involvement registered in the Italian Rare Tumors Pediatric Age (TREP) project, accounting for 26.2% of 61 pediatric RCC observed at AIEOP centers. RESULTS: A radical nephrectomy was performed in all cases: at diagnosis in 12 cases, after preoperative chemotherapy (CT) in 4 cases. As a part of the same procedure 9 patients underwent RLND, and 7 received a more limited lymph nodes resection. Five (31.2%) developed disease recurrence 2-34 months after diagnosis (median, 6 months) plus 1 developed progression; 6 patients died, 1 of them from secondary leukemia. Among the nine patients receiving RLND, eight are alive and disease free. This compares with only one patient surviving among the seven receiving a more limited lymph nodes resection. The estimated 25-year PFS and OS rates for all patients were 61.4% (95% CI 33.2-80.5) and 50.8% (95% CI 16.5-77.5), respectively. CONCLUSIONS: Lymph node involvement is an unfavorable prognostic factor in children with RCC. RLND appears to be a critical factor to improve the outcome. However, when compared to similar adult patients, the outcome in children appears to be better, suggesting that pediatric RCC, or the host, may be critical differences.


Asunto(s)
Carcinoma de Células Renales/patología , Adolescente , Carcinoma de Células Renales/cirugía , Carcinoma de Células Renales/terapia , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Lactante , Italia , Escisión del Ganglio Linfático , Metástasis Linfática/patología , Masculino , Nefrectomía , Pronóstico , Tasa de Supervivencia , Resultado del Tratamiento
16.
Ital J Pediatr ; 44(1): 3, 2018 Jan 04.
Artículo en Inglés | MEDLINE | ID: mdl-29301554

RESUMEN

BACKGROUND: Prevalence of allergy has steeply increased during the past few decades, particularly in high-income countries. The development of atopy could present different characteristics in internationally adopted children with regard to incidence, specific patterns of allergies and timing of occurrence. We aimed to investigate the occurrence of allergic diseases among adopted children in Italy. METHODS: We collected demographic information, preadoption immunization data, infectious diseases screening results, immunological status, and performed hematological and biochemical tests according to a standardized protocol in 108 adopted children. RESULTS: At initial visit (mean age was 5.7 ± 3.2 years), 48 children displayed elevated total serum IgE levels with a prevalence of 56.5% (95%CI: 0.45; 0.67). The prevalences of children screened positive for one or more food allergens and inhalants were 30.1% (95%CI: 19.9%; 42.0%) and 34.3% (95%CI: 23.3%; 46.6%) respectively, only 9 children exhibited abnormal absolute eosinophil counts, 23 (21.3%) had a parasitic infection and 60 (55.6%) had received at least one dose of vaccine. CONCLUSIONS: Children without medical records or with a past medical history suggestive of atopy should perform a thorough allergy evaluation at the time of adoption. Our study offers also a glimpse at the vaccination status and immune-allergic profiles of recent migrant children in Italy.


Asunto(s)
Niño Adoptado/estadística & datos numéricos , Susceptibilidad a Enfermedades/epidemiología , Susceptibilidad a Enfermedades/inmunología , Hipersensibilidad/diagnóstico , Tamizaje Masivo/métodos , Niño , Preescolar , Estudios Transversales , Emigración e Inmigración/estadística & datos numéricos , Femenino , Hospitales Pediátricos , Humanos , Hipersensibilidad/epidemiología , Lactante , Italia/epidemiología , Masculino , Prevalencia , Valores de Referencia , Medición de Riesgo
17.
Ital J Pediatr ; 42: 44, 2016 Apr 27.
Artículo en Inglés | MEDLINE | ID: mdl-27116911

RESUMEN

BACKGROUND: Differential diagnosis between sepsis and non-infectious inflammatory disorders demands improved biomarkers. Soluble Triggering Receptor Expression on Myeloid cells (sTREM-1) is an activating receptor whose role has been studied throughout the last decade. We performed a systematic review to evaluate the accuracy of plasma sTREM-1 levels in the diagnosis of sepsis in children with Systemic Inflammatory Response Syndrome (SIRS). METHODS: A literature search of PubMed, Cochrane Central Register of Controlled Trials, Cumulative Index to Nursing and Allied Health Literature (CINAHL) and ISI Web of Knowledge databases was performed using specific search terms. Studies were included if they assessed the diagnostic accuracy of plasma sTREM-1 for sepsis in paediatric patients with SIRS. Data on sensitivity, specificity, positive predictive value, negative predictive value, area under receiver operating characteristic curve were extracted. The methodological quality of each study was assessed using a checklist based on the Quality Assessment Tool for Diagnostic Accuracy Studies. RESULTS: Nine studies comprising 961 patients were included, four of which were in newborns, three in children and two in children with febrile neutropenia. Some data from single studies support a role of sTREM-1 as a diagnostic tool in pediatric sepsis, but cannot be considered conclusive, because a quantitative synthesis was not possible, due to heterogeneity in studies design. CONCLUSIONS: This systematic review suggests that available data are insufficient to support a role for sTREM in the diagnosis and follow-up of paediatric sepsis.


Asunto(s)
Glicoproteínas de Membrana/sangre , Células Mieloides , Receptores Inmunológicos/sangre , Síndrome de Respuesta Inflamatoria Sistémica/diagnóstico , Biomarcadores/sangre , Niño , Diagnóstico Diferencial , Humanos , Síndrome de Respuesta Inflamatoria Sistémica/sangre , Receptor Activador Expresado en Células Mieloides 1
18.
J Clin Oncol ; 21(3): 530-5, 2003 Feb 01.
Artículo en Inglés | MEDLINE | ID: mdl-12560445

RESUMEN

PURPOSE: To identify the prognostic factors, treatment, and outcome of children affected by renal cell carcinoma (RCC). PATIENTS AND METHODS: The series included 41 patients (18 males and 23 females) with a median age of 124 months observed at the 11 Italian Association for Pediatric Hematology and Oncology centers from January 1973 to January 2001. Clinical data, surgical notes, pathologic findings, and summaries of therapy were taken from the charts. RESULTS: Seven (17%) of the 41 patients had a papillary histology, and 34 (82.4%) had nonpapillary histology. Eighteen patients (43.9%) had stage I, one patient (2.4%) had stage II, two patients (4.8%) had stage IIIA, 10 patients (24.3%) had stage IIIB, and nine patients (21.9%) had stage IV disease. One patient had a bilateral involvement at diagnosis. Seven patients experienced disease recurrence. Lung and liver were the most common distant lesions and usually were fatal. In this study, the major factor influencing the prognosis was the stage. Event-free survival at 20 years was 53.5% for all patients. Overall survival at 20 years was 54.9% for all patients. CONCLUSION: RCC is a rare disease in children and adolescents. This neoplasm has a different clinical presentation in children compared with adults but the same outcome. In our experience, patients with localized disease could be cured by nephrectomy alone. Prospective studies in a larger number of patients are needed to confirm radiation therapy and biologic response modifiers as effective adjunct therapy in RCC stage III. The alternative therapy seems warranted in patients with advanced disease.


Asunto(s)
Carcinoma de Células Renales/patología , Neoplasias Renales/patología , Recurrencia Local de Neoplasia , Nefrectomía , Adolescente , Carcinoma de Células Renales/radioterapia , Carcinoma de Células Renales/cirugía , Niño , Preescolar , Supervivencia sin Enfermedad , Femenino , Estudios de Seguimiento , Humanos , Neoplasias Renales/radioterapia , Neoplasias Renales/cirugía , Masculino , Metástasis de la Neoplasia , Estadificación de Neoplasias , Pronóstico , Factores de Riesgo , Resultado del Tratamiento
19.
Invest Ophthalmol Vis Sci ; 44(6): 2399-403, 2003 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-12766036

RESUMEN

PURPOSE: Fatty acid synthase (FAS) performs the anabolic conversion of dietary carbohydrate or protein to fatty acids. Many common human cancers express high levels of FAS, and its differential expression between normal and neoplastic tissues has led to the consideration of FAS as a target for anticancer therapy. To investigate the potential of targeting FAS in the treatment of retinoblastoma, we first determined whether FAS was activated in this human tumor. Moreover, correlation of FAS expression with tumor aggressiveness was determined. METHODS: FAS reactivity was evaluated by immunohistochemistry in 66 retinoblastoma specimens from 65 patients. Degree of tumor differentiation, choroid invasion, optic nerve infiltration, mitotic rate, and necrosis extension were estimated. FAS expression was correlated with all these tumor characteristics by means of parametric and nonparametric statistical analyses. RESULTS: Eighty-two percent of tumors were FAS positive. Stronger FAS expression correlated with more advanced choroid (P < 0.001) and optic nerve (P = 0.016) invasion, high mitotic index (P < 0.001), and less differentiated histology (P = 0.047). Correlation with extension of necrosis was not statistically significant. Unaffected retina was negative. CONCLUSIONS: The data suggest that expression of FAS and fatty acid synthesis support an essential functional aspect of retinoblastoma cells, perhaps cell growth or survival. FAS activation may serve as a novel target for systemic and local antineoplastic therapy and, because it increases with tumor aggressiveness, its inhibition could represent an alternative treatment strategy in advanced and resistant retinoblastomas.


Asunto(s)
Ácido Graso Sintasas/metabolismo , Neoplasias de la Retina/enzimología , Retinoblastoma/enzimología , Niño , Preescolar , Femenino , Humanos , Técnicas para Inmunoenzimas , Lactante , Masculino , Índice Mitótico , Invasividad Neoplásica , Neoplasias de la Retina/patología , Retinoblastoma/patología , Estudios Retrospectivos
20.
Pediatr Infect Dis J ; 23(10): 963-5, 2004 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-15602201

RESUMEN

We used an antibiotic lock technique with vancomycin in combination with urokinase in 10 consecutive eligible children with Gram-positive catheter-related bacteremia persisting after appropriate intravenous antibiotics. Treatment was successful in sterilizing all colonized central venous catheters, avoiding device removal and delay of further chemotherapy. The antibiotic lock technique may represent a safe and effective therapeutic option in patients with selected, uncomplicated catheter-related bacteremias resistant to systemic antimicrobial therapy, particularly when maintaining a venous access is mandatory.


Asunto(s)
Antibacterianos/uso terapéutico , Catéteres de Permanencia/microbiología , Contaminación de Equipos/prevención & control , Activador de Plasminógeno de Tipo Uroquinasa/administración & dosificación , Vancomicina/uso terapéutico , Adolescente , Antibacterianos/administración & dosificación , Anticoagulantes/administración & dosificación , Anticoagulantes/uso terapéutico , Bacteriemia/tratamiento farmacológico , Bacteriemia/prevención & control , Cateterismo Venoso Central/efectos adversos , Catéteres de Permanencia/efectos adversos , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Neoplasias , Activador de Plasminógeno de Tipo Uroquinasa/uso terapéutico , Vancomicina/administración & dosificación
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