Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 5 de 5
Filtrar
Mais filtros










Base de dados
Intervalo de ano de publicação
1.
Pediatr Blood Cancer ; 67(10): e28496, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32710685

RESUMO

OBJECTIVE: This study was undertaken to describe the spectrum of lung computed-tomography (CT) findings in children with pulmonary Langerhans cell histiocytosis (PLCH) and to evaluate for this population the CT-scan nodule and cyst scores proposed by adult pulmonologists at diagnosis and during follow-up. METHODS: Among 175 children with PLCH identified in the French national population-based Langerhans cell histiocytosis cohort, 60 were retrospectively selected by the availability of CT for a central review by three pediatric radiologists. These 60 patients are representative of childhood PLCH for almost all clinical aspects, except a lower percentage of risk organ involvement (38% vs 54%; P = 0.05). RESULTS: The 60 children's chest CT scans (n = 218) were reviewed. At diagnosis, 63% of them had nodules, 53% had cysts, and 29% had both. The percentages of patients with nodules or cysts increased from diagnosis to peak disease activity, respectively, from 63% to 73% and from 53% to 66%. The costophrenic angle was involved in 71%. Patients with pneumothorax (25%) had a higher median cyst score. Alveolar consolidation was observed in 34%. Patients with low CT-scan nodule and cyst scores had no long-term pulmonary sequelae. CONCLUSIONS: Well-known characteristics of adult PLCH (nodules and cysts) were observed in children. The chest CT scores proposed by adult pulmonologists could easily be applied to childhood PLCH. Lesions in children, unlike those in adults, are frequently located near the costophrenic angles. Alveolar consolidation might be considered an atypical feature of childhood PLCH.


Assuntos
Cistos/diagnóstico , Histiocitose de Células de Langerhans/diagnóstico , Pneumopatias/diagnóstico , Radiografia Torácica/métodos , Tomografia Computadorizada por Raios X/métodos , Criança , Pré-Escolar , Cistos/diagnóstico por imagem , Feminino , Seguimentos , Histiocitose de Células de Langerhans/diagnóstico por imagem , Humanos , Lactente , Pneumopatias/diagnóstico por imagem , Masculino , Prognóstico , Estudos Retrospectivos
2.
Genes Chromosomes Cancer ; 58(9): 673-679, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-30887579

RESUMO

Adipocytic tumors are rare in children and are mostly benign. Less than 25 cases of pediatric well-differentiated liposarcoma (WDLPS), atypical lipomatous tumors (ALT), and dedifferentiated liposarcoma (DDLPS) have been reported. Among them, only three cases were genetically analyzed. We describe the genetic features of a rapidly growing adipose tumor that occurred in the thigh of a 7-year-old girl. Histologically, it was composed of mature adipocytic cells with a few atypia. Molecular analysis showed high-level amplification of the 12q13-21 region including MDM2 among 64 amplified genes. MDM2 amplification is a diagnostic hallmark of ALT/WDLPS/DDLPS. In adult cases, it is typically located in ring or giant marker chromosomes. In the present case, extra-copies of MDM2 were located on double minute chromosomes (dmin). This raised the hypothesis of dmin being precursors of adult's rings and giant markers and may provide indications for a better understanding of the mechanisms of adipose tumor oncogenesis.


Assuntos
Amplificação de Genes , Lipossarcoma/genética , Proteínas Proto-Oncogênicas c-mdm2/genética , Neoplasias de Tecidos Moles/genética , Adipócitos/metabolismo , Adipócitos/patologia , Criança , Cromossomos Humanos Par 12/genética , Feminino , Humanos , Lipossarcoma/patologia , Proteínas Proto-Oncogênicas c-mdm2/metabolismo , Neoplasias de Tecidos Moles/patologia
3.
Eur J Pediatr ; 176(12): 1613-1618, 2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28913555

RESUMO

We analyzed the characteristics of the population with congenital portosystemic shunt diagnosed during the antenatal period and the organization of their perinatal care. This multicentric retrospective study included all the patients with a prenatal diagnosis of congenital portosystemic shunt. Between 1999 and 2015, 12 patients were included. Prenatal diagnosis was done at a median 26.5 weeks of gestation (21-34). All the patients presented intrahepatic CPSS, three of them had associated congenital cardiopathy, and one a Bannayan-Zonana syndrome. Ten patients had simple outcome on conservative treatment, eight of them having a spontaneous closure of their portosystemic shunt within the first 2 years of life. One patient had surgical treatment which failed and he developed a focal nodular hyperplasia. Another patient had radiological interventional closure of his shunt which was complicated by a venal portal thrombosis. CONCLUSION: Outcome of intrahepatic portosystemic shunt diagnosed prenatally is good in the majority of cases. What is known: • Multiples studies exist on congenital porto systemic shunt but when the diagnosis is done after birth. • The evolution, management, and complication are well known. What is new: • There is very few studies with only patients diagnosed in antenatal and it is a large series of cases. • Outcome of intrahepatic portosystemic shunt diagnosed prenatally is good in the majority of cases.


Assuntos
Veia Porta/anormalidades , Ultrassonografia Pré-Natal , Malformações Vasculares/diagnóstico por imagem , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Veia Porta/diagnóstico por imagem , Prognóstico , Estudos Retrospectivos , Malformações Vasculares/terapia
4.
Eur J Radiol ; 93: 195-199, 2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28668415

RESUMO

OBJECTIVE: Allergic broncho-pulmonary aspergillosis (ABPA) is a severe and under-diagnosed complication of cystic fibrosis (CF). The aim of the study was to determine whether the mucus content of bronchoceles in cystic fibrosis complicated with ABPA reveals a higher density than the mucus content of non-ABPA cystic fibrosis. MATERIALS AND METHODS: We studied retrospectively 43 computed tomography scans (CT scans) of a pediatric population of cystic fibrosis patients. We measured the mucus attenuation in Hounsfield Units (HU) of all bronchoceles >5mm in diameter. RESULTS: We found bronchoceles >5mm in 13/43 patients. 5/13 patients had a positive diagnosis of ABPA. The median HU value of bronchoceles was higher in patients with than without ABPA [98 HU (26-135) vs 28 HU (10-36); P=0,02]. Moreover, all patients with a bronchocele density >36HU were ABPA positive. CONCLUSIONS: CF complicated with ABPA shows higher attenuation bronchoceles on CT scans of the chest. Systematic density measurements of bronchoceles could help to raise the difficult diagnosis of ABPA in patients suffering from cystic fibrosis. Larger series could confirm a threshold in HU which could become a new imaging criterion for the diagnosis of ABPA.


Assuntos
Aspergilose Broncopulmonar Alérgica/patologia , Fibrose Cística/patologia , Adolescente , Aspergilose Broncopulmonar Alérgica/complicações , Aspergilose Broncopulmonar Alérgica/diagnóstico por imagem , Bronquiectasia/complicações , Bronquiectasia/diagnóstico por imagem , Bronquiectasia/patologia , Criança , Pré-Escolar , Fibrose Cística/complicações , Fibrose Cística/diagnóstico por imagem , Feminino , Humanos , Masculino , Muco/diagnóstico por imagem , Variações Dependentes do Observador , Estudos Retrospectivos , Tomografia Computadorizada por Raios X/métodos , Adulto Jovem
5.
J Pediatr Hematol Oncol ; 31(10): 794-6, 2009 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-19770685

RESUMO

First described in HIV-infected patients who recently initiated highly active antiretroviral therapy, the immune reconstitution inflammatory syndrome (IRIS) is best characterized as a collection of inflammatory disorders triggered by rapid resolution of immunosuppression. Treatment of IRIS is a clinical challenge due to the variety of clinical presentations and the presence of multiple pathogens capable of causing the syndrome. Hepatosplenic candidiasis, an uncommon form of invasive Candida species infection, was recently suggested to belong to the spectrum of fungus-related IRIS. We report 2 cases of probable hepatosplenic candidiasis according to the guidelines of the European Organization for Research and Treatment of Cancer and the Mycosis Study Group, occurring in pediatric patients with acute leukemia during rapid neutrophil recovery after cytotoxic chemotherapy. In both cases, abdominal computed tomography scan revealed multiple hepatic micronodules, and liver biopsy showed nonspecific granulomatous lesions. Hepatosplenic candidiasis symptoms (fever, nausea and vomiting, abdominal pain) resolved within 2 days after adjunction of corticosteroid therapy to antifungal treatment. Inflammatory markers and related radiologic abnormalities decreased or disappeared within 1 month. Recovery of neutrophil count in a context of hepatosplenic candidiasis may result in a heightened inflammatory response. Corticosteroid therapy in this setting is associated with prompt resolution of the symptoms.


Assuntos
Corticosteroides/uso terapêutico , Candidíase/complicações , Síndrome Inflamatória da Reconstituição Imune/etiologia , Quimioterapia Adjuvante , Criança , Feminino , Humanos , Hepatopatias/microbiologia , Masculino , Indução de Remissão , Esplenopatias/microbiologia
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...