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1.
Artigo em Inglês | MEDLINE | ID: mdl-38248541

RESUMO

The onset of chronic diseases in childhood represents a stressful event for both young patients and their caregivers. In this context, coping strategies play a fundamental role in dealing with illness-related challenges. Although numerous studies have explored coping strategies employed by parents of children with chronic diseases, there remains a gap in the understanding of children's coping strategies and their correlation with their and their parents' anxiety. This study aims to investigate coping strategies and their interaction with anxiety in groups of young patients with cancer, type 1 diabetes (T1D), and their respective caregivers, in comparison to healthy children and caregivers. We recruited a total of 61 control children, 33 with cancer, and 56 with T1D, 7 to 15 years old, along with their mothers. Each participant completed a customized survey and standardized questionnaires. No significant differences emerged in coping strategies used by children among the different groups. However, when examining the association between coping strategy and anxiety, we found specific patterns of interaction between children's use of coping strategies and their and their mothers' anxiety levels. This study underscores the importance of an illness-specific approach to gain deeper insights into this topic and develop targeted interventions aimed at enhancing the psychological well-being of these vulnerable populations.


Assuntos
Diabetes Mellitus Tipo 1 , Neoplasias , Criança , Humanos , Adolescente , Cuidadores , Capacidades de Enfrentamento , Ansiedade , Doença Crônica
2.
Blood Transfus ; 22(1): 75-85, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37146300

RESUMO

BACKGROUND: In transfusion-dependent thalassemia patients who started regular transfusions in early childhood, we prospectively and longitudinally evaluated the efficacy on pancreatic iron of a combined deferiprone (DFP) + desferrioxamine (DFO) regimen versus either oral iron chelator as monotherapy over a follow-up of 18 months. MATERIALS AND METHODS: We selected patients consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia network who received a combined regimen of DFO+DFP (No.=28) or DFP (No.=61) or deferasirox (DFX) (No.=159) monotherapy between the two magnetic resonance imaging scans. Pancreatic iron overload was quantified by the T2* technique. RESULTS: At baseline no patient in the combined treatment group had a normal global pancreas T2* (≥26 ms). At follow-up the percentage of patients who maintained a normal pancreas T2* was comparable between the DFP and DFX groups (57.1 vs 70%; p=0.517).Among the patients with pancreatic iron overload at baseline, global pancreatic T2* values were significantly lower in the combined DFO+DFP group than in the DFP or DFX groups. Since changes in global pancreas T2* values were negatively correlated with baseline pancreas T2* values, the percent changes in global pancreas T2* values, normalized for the baseline values, were considered. The percent changes in global pancreas T2* values were significantly higher in the combined DFO+DFP group than in either the DFP (p=0.036) or DFX (p=0.030) groups. DISCUSSION: In transfusion-dependent patients who started regular transfusions in early childhood, combined DFP+DFO was significantly more effective in reducing pancreatic iron than was either DFP or DFX.


Assuntos
Sobrecarga de Ferro , Talassemia , Talassemia beta , Humanos , Pré-Escolar , Ferro/uso terapêutico , Deferasirox , Deferiprona/uso terapêutico , Desferroxamina/uso terapêutico , Quelantes de Ferro/uso terapêutico , Piridonas/uso terapêutico , Talassemia beta/diagnóstico por imagem , Talassemia beta/tratamento farmacológico , Benzoatos/uso terapêutico , Triazóis/uso terapêutico , Quimioterapia Combinada , Sobrecarga de Ferro/diagnóstico por imagem , Sobrecarga de Ferro/tratamento farmacológico , Sobrecarga de Ferro/etiologia , Imageamento por Ressonância Magnética , Espectroscopia de Ressonância Magnética , Pâncreas/diagnóstico por imagem
4.
Tomography ; 9(5): 1711-1722, 2023 09 11.
Artigo em Inglês | MEDLINE | ID: mdl-37736989

RESUMO

BACKGROUND: The E-MIOT (Extension-Myocardial Iron Overload in Thalassemia) project is an Italian Network assuring high-quality quantification of tissue iron overload by magnetic resonance imaging (MRI). We evaluated the impact of the COVID-19 pandemic on E-MIOT services. METHODS: The activity of the E-MIOT Network MRI centers in the year 2020 was compared with that of 2019. A survey evaluated whether the availability of MRI slots for patients with hemoglobinopathies was reduced and why. RESULTS: The total number of MRI scans was 656 in 2019 and 350 in 2020, with an overall decline of 46.4% (first MRI: 71.7%, follow-up MRI: 36.9%), a marked decline (86.9%) in the period March-June 2020, and a reduction in the gap between the two years in the period July-September. A new drop (41.4%) was recorded in the period October-December for two centers, due to the general reduction in the total amount of MRIs/day for sanitization procedures. In some centers, patients refused MRI scans for fear of getting COVID. Drops in the MRI services >80% were found for patients coming from a region without an active MRI site. CONCLUSIONS: The COVID-19 pandemic had a strong negative impact on MRI multi-organ iron quantification, with a worsening in the management of patients with hemoglobinopathies.


Assuntos
COVID-19 , Hemoglobinopatias , Sobrecarga de Ferro , Humanos , COVID-19/diagnóstico por imagem , Pandemias , Hemoglobinopatias/complicações , Hemoglobinopatias/diagnóstico por imagem , Sobrecarga de Ferro/diagnóstico por imagem , Imageamento por Ressonância Magnética
5.
Sleep Med ; 103: 69-77, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36764044

RESUMO

OBJECTIVES: Children with chronic illnesses and their parents are more at risk to develop sleep problems, which are linked to worse psychological and physical well-being. This study aimed to assess sleep patterns and their connections with psychological outcomes in children with type 1 diabetes (T1D) and cancer and their caregivers, compared to a control sample. In addition, we explored the associations between caregiver and child's sleep quality across the three groups. METHODS: We enrolled 56 children with T1D, 33 children with cancer, and 61 healthy children between 7 and 15, and their respective caregivers. Caregivers filled out an ad-hoc survey assessing their sleep disturbances, parenting stress, general well-being, anxiety, and their children's sleep patterns and psychological adjustments. RESULTS: Children with cancer showed lower sleep quality than the other groups. Moreover, worse psychological adjustment was associated with greater sleep disturbances in both clinical groups. As for caregivers, the cancer group reported the worst sleep quality and greater anxiety compared to the other samples. Greater anxiety was also linked to worse sleep quality. Furthermore, greater sleep problems in children were associated with poorer caregivers' sleep quality in the whole sample and the T1D group. CONCLUSIONS: A better understanding of sleep patterns and problems for chronically ill children and their parents is fundamental to provide adequate care for these vulnerable populations. Furthermore, an illness-specific approach may better inform and guide the practitioners in clinical practice.


Assuntos
Diabetes Mellitus Tipo 1 , Neoplasias , Distúrbios do Início e da Manutenção do Sono , Transtornos do Sono-Vigília , Humanos , Criança , Cuidadores/psicologia , Diabetes Mellitus Tipo 1/complicações , Pais , Qualidade do Sono , Transtornos do Sono-Vigília/epidemiologia , Estresse Psicológico
6.
Int J Cardiovasc Imaging ; 39(5): 991-999, 2023 May.
Artigo em Inglês | MEDLINE | ID: mdl-36637709

RESUMO

PURPOSE: The left ventricular global function index (LVGFI) is a comprehensive marker of cardiac performance, integrating LV morphology with global function. We explored the cross-sectional association of LVGFI with myocardial iron overload (MIO), LV ejection fraction (LVEF), myocardial fibrosis, and heart failure (HF) in ß-thalassemia major (TM) patients. METHODS: We considered 1352 adult TM patients (708 females, 32.79 ± 7.16years) enrolled in the Myocardial Iron Overload in Thalassemia Network and 112 healthy subjects (50 females, 32.09 ± 6.08years). LVGFI and LVEF were assessed by cine images and MIO by multislice multiecho T2* technique. Replacement myocardial fibrosis was detected by late gadolinium enhancement technique. RESULTS: LVGFI and LVEF were significantly lower in patients with significant MIO (global heart T2*<20ms) than in patients without MIO and in healthy subjects but were comparable between TM patients without MIO and healthy subjects. In TM, LVGFI was significantly associated with LVEF (R = 0.733; p < 0.0001). Global heart T2* values were significantly associated with both LVGFI and LVEF, but the correlation with LVGFI was significantly stronger (p = 0.0001). Male sex, diabetes mellitus, significant MIO, and replacement myocardial fibrosis were the strongest predictors of LVGFI. Eighty-six patients had a history of HF and showed significantly lower global heart T2* values, LVEF, and LVGFI than HF-free patients. A LVGFI ≤ 44.9% predicted the presence of HF. The LVGFI showed a diagnostic performance superior to that of LVEF (area under the curve: 0.67 vs. 0.62; p = 0.039). CONCLUSION: In TM patients the LVGFI correlates with MIO and provides incremental diagnostic value for HF detection compared with LVEF.


Assuntos
Cardiomiopatias , Insuficiência Cardíaca , Sobrecarga de Ferro , Talassemia beta , Adulto , Feminino , Humanos , Masculino , Talassemia beta/complicações , Talassemia beta/diagnóstico , Estudos Transversais , Meios de Contraste , Valor Preditivo dos Testes , Gadolínio , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/complicações , Sobrecarga de Ferro/diagnóstico por imagem , Sobrecarga de Ferro/etiologia , Função Ventricular Esquerda , Fibrose , Imageamento por Ressonância Magnética/métodos
8.
Artigo em Inglês | MEDLINE | ID: mdl-36361022

RESUMO

Due to the COVID-19 pandemic, many families had to manage new difficulties, especially those of chronically ill children. More and more research has focused on the negative effects of the pandemic on psychological wellbeing, while less is known about the resources. The present study aimed to explore the role of time spent with mothers in chronically ill children's populations during the COVID-19 pandemic. Moreover, it explored the differences in mothers' and children's psychosocial functioning in three clinical populations. Four groups were recruited and compared: 7-15 year old children with asthma (45), type 1 diabetes (52), and cancer (33), as well as their healthy counterparts (41), and their respective mothers. They were administered standardized questionnaires and ad hoc surveys assessing psychological wellbeing and worries. Children of the four groups scored significantly differently with respect to the concerns for contagion, internalizing symptoms, and prosocial behaviors; mothers had worries about the consequences of their children's contagion related to the chronic illness, as well as time with the child. The multiple linear regression model showed an association of being affected by cancer, suffering from type 1 diabetes, and spending less time with the child with an increase in children's internalizing problems. Time with mothers seemed to be a resource for psychological wellbeing during the pandemic. Clinical implications are discussed.


Assuntos
Asma , COVID-19 , Diabetes Mellitus Tipo 1 , Neoplasias , Criança , Feminino , Humanos , Adolescente , Mães/psicologia , Pandemias , Diabetes Mellitus Tipo 1/epidemiologia , COVID-19/epidemiologia , Doença Crônica , Asma/epidemiologia , Neoplasias/epidemiologia
9.
Children (Basel) ; 9(4)2022 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-35455525

RESUMO

Having cancer in childhood is a risk factor for separation anxiety symptoms, with negative effects on the disease and psychological wellbeing. The Covid-19-pandemic-related concerns could have a negative effect. The present study explores the interplay between separation anxiety symptoms and COVID-19-related worries in pediatric cancer patients and their mothers, compared to a group of healthy children and their mothers, during the pandemic. Thirty-three subjects with cancer, aged 7-15 years, and their mothers were compared to a control sample of 36 healthy children and caregivers. They were administered a pandemic-related psychological experience survey and standardized questionnaires assessing psychological wellbeing, anxiety, and separation anxiety symptoms. Children with cancer reported significantly higher prosocial behaviors, fear of being alone, and fear of abandonment. Their mothers had worse psychological wellbeing, higher COVID-19 concerns, anxiety, and separation anxiety symptoms. The multiple linear regression model showed that an increase in children's separation anxiety symptoms was associated with younger age, more recent diagnosis, more mother-child time, lower mothers' worry for children's contagion, and higher mothers' and children's anxiety. COVID-19-related worries of clinical children's mothers seem to be protective for children's psychological wellbeing. Early psychosocial support interventions for mothers are essential in health services for cancer.

10.
J Clin Med ; 10(23)2021 Nov 26.
Artigo em Inglês | MEDLINE | ID: mdl-34884261

RESUMO

In thalassemia major, pancreatic iron was demonstrated as a powerful predictor not only for the alterations of glucose metabolism but also for cardiac iron, fibrosis, and complications, supporting a profound link between pancreatic iron and heart disease. We determined for the first time the prevalence of pancreatic iron overload (IO) in thalassemia intermedia (TI) and systematically explored the link between pancreas T2* values and glucose metabolism and cardiac outcomes. We considered 221 beta-TI patients (53.2% females, 42.95 ± 13.74 years) consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia project. Magnetic Resonance Imaging was used to quantify IO (T2* technique) and biventricular function and to detect replacement myocardial fibrosis. The glucose metabolism was assessed by the oral glucose tolerance test (OGTT). Pancreatic IO was more frequent in regularly transfused (N = 145) than in nontransfused patients (67.6% vs. 31.6%; p < 0.0001). In the regular transfused group, splenectomy and hepatitis C virus infection were both associated with high pancreatic siderosis. Patients with normal glucose metabolism showed significantly higher global pancreas T2* values than patients with altered OGTT. A pancreas T2* < 17.9 ms predicted an abnormal OGTT. A normal pancreas T2* value showed a 100% negative predictive value for cardiac iron. Pancreas T2* values were not associated to biventricular function, replacement myocardial fibrosis, or cardiac complications. Our findings suggest that in the presence of pancreatic IO, it would be prudent to initiate or intensify iron chelation therapy to prospectively prevent both disturbances of glucose metabolism and cardiac iron accumulation.

11.
Neuropathol Appl Neurobiol ; 47(6): 878-881, 2021 10.
Artigo em Inglês | MEDLINE | ID: mdl-34048085

RESUMO

AIMS: Histiocytoses are a heterogeneous group of localized or disseminated diseases. Clinical presentation and patients' outcome vary greatly, ranging from mild to life-threatening disorders. Rare cases of systemic or localized histiocytosis harboring ALK rearrangement have been reported. METHODS: Two cases of CNS histiocytosis were thoroughly investigated by implementing multiple molecular tests, i.e. FISH, RT-qPCR, NGS analysis. RESULTS: In a 10-month old girl (patient #1), MRI showed two left hemispheric lesions and a right fronto-mesial lesion histologically consisting of a moderately cellular infiltrative proliferation, composed by CD68(PGM1)+/CD163+ spindle cells. ALK 5'/3'-imbalance and a KIF5B(exon 24)-ALK(exon 20) fusion were documented by RT-qPCR and NGS analysis, respectively. A subsequent CT scan showed multiple hepatic and pulmonary lesions. The patient was started on chemotherapy (vinblastine) associated to an ALK-inhibitor (Alectinib) with remarkable response. In a 11-year-old girl (patient #2), MRI showed a right frontal 1.5 cm lesion. Neuropathological examination revealed a histiocytic proliferation composed by medium sized CD68(PGM1)+/HLA-DR+ cells, showing moderate ALK1 positivity. ALK rearrangement and a KIF5B(exon 24)-ALK(exon 20) fusion were demonstrated also in this case. Subsequent CT, 18F-FDG-PET and MRI scans showed the presence of a single right femoral lesion, proved to be a fibrous cortical defect. CONCLUSIONS: In ALK-histiocytoses, CNS involvement may occur as part of a systemic disease or, rarely, as its only primary disease localization, which could remain otherwise asymptomatic. The diagnosis often relies on neuropathological examination of brain biopsy, which may pose a diagnostic challenge due to the variable histopathological features. An integrated histological and molecular approach in such cases is recommended.


Assuntos
Quinase do Linfoma Anaplásico/metabolismo , Sistema Nervoso Central/patologia , Histiocitose/tratamento farmacológico , Inibidores de Proteínas Quinases/uso terapêutico , Biópsia/métodos , Sistema Nervoso Central/efeitos dos fármacos , Criança , Feminino , Histiocitose/diagnóstico , Histiocitose/patologia , Humanos , Lactente , Receptores Proteína Tirosina Quinases/efeitos dos fármacos , Receptores Proteína Tirosina Quinases/metabolismo
12.
J Pediatr Hematol Oncol ; 41(5): 404-406, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-30113356

RESUMO

BACKGROUND: Paratesticular fibrous pseudotumor (PFP) is a rare benign tumor that can mimic malignant tumors; thus, radical orchiectomy was the standard treatment in the past. OBSERVATION: A 15-year-old boy came to our attention for a hard right inguinal swelling. An ultrasound showed a highly vascularized mass. The boy underwent a gonadal-sparing excision of the mass that was diagnosed to be a PFP. The patient recovered well, without recurrences at the follow-up. CONCLUSIONS: PFP is mostly asymptomatic, and its diagnosis is incidental. Only further 4 pediatric cases were reported in the literature. Our case confirms that gonadal-sparing surgery is an optimal treatment.


Assuntos
Leiomioma/cirurgia , Doenças Testiculares/cirurgia , Adolescente , Edema , Humanos , Leiomioma/diagnóstico por imagem , Leiomioma/patologia , Masculino , Doenças Testiculares/diagnóstico por imagem , Doenças Testiculares/patologia , Resultado do Tratamento , Ultrassonografia
13.
Pediatr Blood Cancer ; 66(3): e27519, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30362240

RESUMO

BACKGROUND: Solid pseudopapillary pancreatic tumors (SPPT) are an extremely rare entity in pediatric patients. Even if the role of radical surgical resection as primary treatment is well established, data about follow-up after pancreatic resection in children are scant. METHODS: A retrospective review of data from the Italian Pediatric Rare Tumor Registry (TREP) was performed. Short-term (<30 days) and long-term complications of different surgical resections, as well as long-term follow-up were evaluated. RESULTS: From January 2000 to present, 43 patients (male:female = 8:35) were enrolled. The median age at diagnosis was 13.2 years (range, 7-18). Nine children had an incidental diagnosis, whereas 26 complained of abdominal pain and 4 of palpable mass. Tumors arose either from the head of pancreas (n = 14) or from body/tail (n = 29): only one patient presented with metastatic disease. Resection was complete in all patients (cephalic duodenopancreatectomy vs distal resection). At follow-up (median, 8.4 years; range, 0-17 years), one recurrence occurred in a patient with intraoperative rupture. All patients are alive. Three pancreatic fistulas occurred in the body/tail group, whereas four complications occurred in the head group (one ileal ischemia, two stenosis of the pancreatic duct, and one chylous fistula). CONCLUSION: Surgery is the best therapeutic option for these tumors; hence, complete resection is mandatory. Extensive resections, including cephalic duodenopancreatectomy, are safe when performed in specialized centers. Long-term follow-up should be aimed to detect tumor recurrence and to evaluate residual pancreatic function.


Assuntos
Carcinoma Papilar/cirurgia , Recidiva Local de Neoplasia/cirurgia , Pancreatectomia/métodos , Neoplasias Pancreáticas/cirurgia , Complicações Pós-Operatórias , Adolescente , Carcinoma Papilar/patologia , Criança , Feminino , Seguimentos , Humanos , Itália , Masculino , Recidiva Local de Neoplasia/patologia , Neoplasias Pancreáticas/patologia , Sistema de Registros , Estudos Retrospectivos , Resultado do Tratamento
14.
Ther Adv Neurol Disord ; 11: 1756286418775375, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29977341

RESUMO

Medulloblastoma is the most common malignant pediatric brain tumor. Medulloblastoma should not be viewed as a single disease, but as a heterogeneous mixture of various subgroups with distinct characteristics. Based on genomic profiles, four distinct molecular subgroups are identified: Wingless (WNT), Sonic Hedgehog (SHH), Group 3 and Group 4. Each of these subgroups are associated with specific genetic aberrations, typical age of onset as well as survival prognosis. Magnetic resonance imaging (MRI) is performed for all patients with brain tumors, and has a key role in the diagnosis, surgical guidance and follow up of patients with medulloblastoma. Several studies indicate MRI as a promising tool for early detection of medulloblastoma subgroups. The early identification of the subgroup can influence the extent of surgical resection, radiotherapy and chemotherapy targeted treatments. In this article, we review the state of the art in MRI-facilitated medulloblastoma subgrouping, with a summary of the main MRI features in medulloblastoma and a brief discussion on molecular characterization of medulloblastoma subgroups. The main focus of the article is MRI features that correlate with medulloblastoma subtypes, as well as features suggestive of molecular subgroups. Finally, we briefly discuss the latest trends in MRI studies and latest developments in molecular characterization.

15.
J Pediatr Hematol Oncol ; 40(4): e207-e214, 2018 05.
Artigo em Inglês | MEDLINE | ID: mdl-29432309

RESUMO

Epidemiologic studies have suggested that parental occupations, pesticide use, environmental factors, and genetic polymorphism are involved in the etiology of childhood acute leukemia (CAL). In total, 116 cases of CAL and 162 controls were recruited and submitted to blood drawing to assess the presence of genetic polymorphisms. Parental occupations, pesticides exposure, and other potential determinants were investigated. Increased risk for CAL was associated with prenatal maternal use of insecticides/rodenticides (odds ratio [OR]=1.87; 95% confidence intervals [CI], 1.04-3.33), with subjects living <100 m from pesticide-treated fields (OR=3.21; 95% CI, 1.37-7.53) and with a paternal occupation as traffic warden/policeman (OR=4.02; 95% CI, 1.63-9.87). Associations were found between CAL and genetic polymorphism of CYP2D6*4 for homozygous alleles (mutant type/mutant type: OR=6.39; 95% CI, 1.17-34.66). In conclusion, despite the small sample size, maternal prenatal exposure to pesticides, paternal occupation as a traffic warden/police officer, and CYP2D6*4 polymorphism could play a role in the etiology of CAL.


Assuntos
Alelos , Citocromo P-450 CYP2D6/genética , Leucemia , Exposição Materna/efeitos adversos , Exposição Ocupacional/efeitos adversos , Praguicidas/efeitos adversos , Polimorfismo Genético , Doença Aguda , Criança , Pré-Escolar , Feminino , Humanos , Leucemia/enzimologia , Leucemia/epidemiologia , Leucemia/etiologia , Leucemia/genética , Masculino , Militares , Polícia
16.
Tumori ; 104(3): 232-237, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29192742

RESUMO

INTRODUCTION: Rhabdomyosarcoma is a soft tissue malignant musculoskeletal tumor frequent in children. Biliary duct localization is extremely rare, but it is the most common cause of malignant obstructive jaundice in pediatric patients. METHODS: This report describes a series of 10 patients under 18 years of age with biliary tract rhabdomyosarcoma who were enrolled, from 1979 to 2004, in 3 consecutive Italian pediatric cooperative protocols that had been drawn up by the Soft Tissue Sarcoma Committee of the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP). RESULTS: Considering initial and delayed surgery, tumor resection was achieved in 7 cases, 3 complete with free margins (2 liver transplants) and 4 with microscopic residual disease. Chemotherapy was given to all patients and radiotherapy to 3. At present, 5 patients survive in complete remission 90-200 months after diagnosis while 4 died of disease progression or relapse and 1 of liver transplant-related complications. CONCLUSIONS: Better outcomes in this series were associated with the feasibility of conservative surgery due to the favorable location of the tumor, in particular in the common bile duct. Chemotherapy and radiotherapy might obviate the need for demolitive surgery or liver transplant, which were linked to worse outcomes in our series.


Assuntos
Neoplasias do Sistema Biliar/patologia , Rabdomiossarcoma/patologia , Sarcoma/patologia , Antineoplásicos/uso terapêutico , Sistema Biliar/patologia , Neoplasias do Sistema Biliar/cirurgia , Criança , Pré-Escolar , Progressão da Doença , Feminino , Humanos , Lactente , Itália , Masculino , Recidiva Local de Neoplasia/mortalidade , Recidiva Local de Neoplasia/patologia , Recidiva Local de Neoplasia/terapia , Indução de Remissão/métodos , Rabdomiossarcoma/mortalidade , Rabdomiossarcoma/terapia , Sarcoma/mortalidade , Sarcoma/cirurgia , Resultado do Tratamento
17.
Pediatr Blood Cancer ; 64(9)2017 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-28233470

RESUMO

BACKGROUND: Pediatric nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) may rarely occur in visceral tissues, and little is known about their clinical history. The present study retrospectively analyzed a group of patients prospectively registered in Italian pediatric protocols conducted between 1979 and 2004. METHODS: Inclusion criteria for the study were as follows: a pathological diagnosis of "adult-type NRSTS," arising at visceral sites (lung-pleurae, liver, kidney, and mesentery-bowel); age under 18 years; no previous treatment except for primary surgery; available clinical data; and written consent. RESULTS: Thirty cases with visceral NRSTS were collected and analyzed. Sites of origin were as follows: mesentery-bowel in 12 cases, lung-pleurae in 11, liver in 5, and kidney in 2. According to the Intergroup Rhabdomyosarcoma Study (IRS) surgical grouping system, patients were classified as follows: nine IRS group I, three group II, 12 group III, and six group IV. Patients were treated with a multimodal approach including surgery, radiotherapy, and/or chemotherapy, according to their characteristics. For the series as a whole, the 5-year event-free and overall survival rates were 33.3% and 40.0%, respectively. The IRS group (reflecting the feasibility of initial complete resection) emerged as the main prognostic factor. Survival rates also correlated with tumor size and local invasiveness, histological subtype, and tumor sites (the worst outcome was seen for tumors arising in the lung and pleurae). CONCLUSIONS: This study confirmed that visceral NRSTS are aggressive tumors carrying a worse prognosis than pediatric NRSTS arising in soft tissues of the extremities. Local treatment remains the main challenge for these tumors.


Assuntos
Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/terapia , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Criança , Pré-Escolar , Terapia Combinada , Intervalo Livre de Doença , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Prognóstico , Vísceras/patologia
18.
Oncotarget ; 6(15): 13295-308, 2015 May 30.
Artigo em Inglês | MEDLINE | ID: mdl-25980492

RESUMO

To get insights on the metastatic process of human neuroblastoma (NB), the miRNA expression profile of bone marrow (BM)-infiltrating cells has been determined and compared to that of primary tumors.Twenty-two BM-infiltrating cells, 22 primary tumors, and 4 paired samples from patients with metastatic NB aged > 12 months were analyzed for the expression of 670 miRNAs by stem-loop RT-qPCR. The miRNAs whose expression was significantly different were subjected to selection criteria, and 20 selected miRNAs were tested in 10 additional BM-infiltrating cells and primary tumors. Among the miRNAs confirmed to be differentially expressed, miR-659-3p was further analyzed. Transfection of miR-659-3p mimic and inhibitor demonstrated the specific suppression and over-expression, respectively, of the miR-659-3p target gene CNOT1, a regulator of transcription of genes containing AU-rich element (ARE) sequence. Among the ARE-containing genes, miR-659-3p mimic and inhibitor specifically modified the expression of AKT3, BCL2, CYR61 and THSB2, belonging to the focal adhesion pathway. Most importantly, in BM-infiltrating cells CNOT1 expression was significantly higher, and that of AKT3, BCL2, THSB2 and CYR61 was significantly lower than in primary tumors. Thus, our study suggests a role of the focal adhesion pathway, regulated by miR-659-3p through CNOT1, in the human NB metastatic process.


Assuntos
Neoplasias da Medula Óssea/genética , Regulação da Expressão Gênica/fisiologia , MicroRNAs/genética , Neuroblastoma/genética , Neoplasias da Medula Óssea/secundário , Linhagem Celular Tumoral , Criança , Pré-Escolar , Feminino , Adesões Focais/fisiologia , Perfilação da Expressão Gênica , Humanos , Lactente , Masculino , Neuroblastoma/patologia , Reação em Cadeia da Polimerase em Tempo Real , Fatores de Transcrição/metabolismo , Transfecção
19.
Clin Biochem ; 47(9): 848-52, 2014 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-24769278

RESUMO

BACKGROUND: Urinary homovanillic and vanillylmandelic acid (HVA and VMA) are well known biomarkers for the management of neuroblastoma (NB). Very few and contradictory publications on their diagnostic performance are present in the literature. The aim of this study is to review the results of HVA/Cr and VMA/Cr obtained by the reference laboratory of the Italian Cooperative Group for NB within a 7-year period using HPLC-EC. PROCEDURE: Updated reference intervals based on age as a continuous variable were calculated by using a multivariate statistical analysis. The diagnostic performance of the two biomarkers has been established by calculating their specificity and sensitivity and by receiver operating characteristics (ROC) curves for different ages and stages of disease. RESULTS: Accurate age-related reference intervals were obtained from 648 HVA/Cr and 671 VMA/Cr results derived from patients in which the diagnosis of neuroendocrine tumors was excluded. Sensitivity, specificity and ROC curves were obtained from 169 HVA/Cr and 179 VMA/Cr results from confirmed NB patients. The best diagnostic performance was obtained in stage 4S tumors and in children <18months. CONCLUSIONS: This is the first report, to our knowledge, that analyzes in depth the diagnostic performance of HVA/Cr and VMA/Cr for NB in different stages and age subgroups. In addition, the present work provides cut-off points able to discriminate between NB patients and negative subjects suspected to have NB and could be of help in taking medical decisions.


Assuntos
Biomarcadores Tumorais/urina , Ácido Homovanílico/urina , Neuroblastoma/diagnóstico , Ácido Vanilmandélico/urina , Adolescente , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Itália , Masculino , Neuroblastoma/urina , Curva ROC , Valores de Referência , Adulto Jovem
20.
Riv Psichiatr ; 47(5): 400-6, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-23160050

RESUMO

AIM: The aim of the study is to evaluate the effectiveness of the Strength and Difficulties Questionnaire (SDQ) as a screening tool for emotional and behavioral problems in three different populations at risk. METHODS: The SDQ is a brief screening questionnaire that investigates the presence of emotional and behavioural problems in children and adolescents. We have analyzed 497 questionnaires completed by parents of children referred to Child Neuropsychiatry, Pediatric Endocrinology and Pediatric Oncology Units. RESULTS: Results indicate a higher presence of psychopathological disorders in Neuropsychiatry patients than Oncology and Endocrinology patients. Furthermore the Oncology patients have more emotional and behavioral problems than Endocrinology patients. DISCUSSION: The findings support the use of the SDQ questionnaire in the assessment of emotional and behavioral problems in these populations at risk. So the SDQ could be used by clinicians to detect early psychopathological disorder in children with different kind of chronic organic diseases.


Assuntos
Sintomas Afetivos/diagnóstico , Transtornos Mentais/diagnóstico , Inquéritos e Questionários , Adolescente , Criança , Pré-Escolar , Humanos , Itália , Pacientes Ambulatoriais
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