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1.
Neuroradiology ; 66(3): 317-323, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38183424

RESUMO

PURPOSE: After standard treatment for glioblastoma, perfusion MRI remains challenging for differentiating tumor progression from post-treatment changes. Our objectives were (1) to correlate rCBV values at diagnosis and at first tumor progression and (2) to analyze the relationship of rCBV values at tumor recurrence with enhancing volume, localization of tumor progression, and time elapsed since the end of radiotherapy in tumor recurrence. METHODS: Inclusion criteria were (1) age > 18 years, (2) histologically confirmed glioblastoma treated with STUPP regimen, and (3) tumor progression according to RANO criteria > 12 weeks after radiotherapy. Co-registration of segmented enhancing tumor VOIs with dynamic susceptibility contrast perfusion MRI was performed using Olea Sphere software. For tumor recurrence, we correlated rCBV values with enhancing tumor volume, with recurrence localization, and with time elapsed from the end of radiotherapy to progression. Analyses were performed with SPSS software. RESULTS: Sixty-four patients with glioblastoma were included in the study. Changes in rCBV values between diagnosis and first tumor progression were significant (p < 0.001), with a mean and median decreases of 32% and 46%, respectively. Mean rCBV values were also different (p < 0.01) when tumors progressed distally (radiation field rCBV values of 1.679 versus 3.409 distally). However, changes and, therefore, low rCBV values after radiotherapy in tumor recurrence were independent of time. CONCLUSION: Chemoradiation alters tumor perfusion and rCBV values may be decreased in the setting of tumor progression. Changes in rCBV values with respect to diagnosis, with low rCBV in tumor progression, are independent of time but related to the site of recurrence.


Assuntos
Neoplasias Encefálicas , Glioblastoma , Humanos , Adulto , Pessoa de Meia-Idade , Glioblastoma/diagnóstico por imagem , Glioblastoma/radioterapia , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/radioterapia , Recidiva Local de Neoplasia/diagnóstico por imagem , Meios de Contraste , Quimiorradioterapia , Imageamento por Ressonância Magnética/métodos
2.
Neurologia (Engl Ed) ; 38(8): 550-559, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37437655

RESUMO

INTRODUCTION: Cerebral amyloid angiopathy-related inflammation (CAA-ri) is an entity characterised by an inflammatory response to ß-amyloid deposition in the walls of cerebral microvessels. METHODS: We conducted a retrospective review of a series of patients with a diagnosis of CAA-ri according to histopathological study findings or clinical-radiological diagnostic criteria. RESULTS: The study included 7 patients (5 men) with a mean age of 79 years. Disease onset was acute or subacute in 6 patients. The most frequent symptoms were cognitive impairment (n = 6), behavioural alterations (n = 5), epileptic seizures (n = 5), focal neurological signs (n = 4), and headache (n = 2). Cerebrospinal fluid was abnormal in 3 patients (lymphocytic pleocytosis and high protein levels). The most frequent MRI findings were microbleeds (n = 7), subcortical white matter hyperintensities on T2-FLAIR sequences (n = 7), and leptomeningeal enhancement (n = 6). Lesions were bilateral in 3 patients and most frequently involved the parieto-occipital region (n = 5). Amyloid PET studies were performed in 2 patients, one of whom showed pathological findings. Two patients underwent brain biopsy, which confirmed diagnosis. All patients received immunosuppressive therapy. An initially favourable clinical-radiological response was observed in all cases, with 2 patients presenting radiological recurrence after treatment withdrawal, with a subsequent improvement after treatment was resumed. CONCLUSIONS: Early diagnosis of CAA-ri is essential: early treatment has been shown to improve prognosis and reduce the risk of recurrence. Although a histopathological study is needed to confirm diagnosis, clinical-radiological criteria enable diagnosis without biopsy.


Assuntos
Angiopatia Amiloide Cerebral , Masculino , Humanos , Idoso , Angiopatia Amiloide Cerebral/complicações , Angiopatia Amiloide Cerebral/diagnóstico por imagem , Inflamação/patologia , Imageamento por Ressonância Magnética , Radiografia , Estudos Retrospectivos
3.
Database (Oxford) ; 20222022 06 07.
Artigo em Inglês | MEDLINE | ID: mdl-35670729

RESUMO

There are >2500 different genetically determined developmental disorders (DD), which, as a group, show very high levels of both locus and allelic heterogeneity. This has led to the wide-spread use of evidence-based filtering of genome-wide sequence data as a diagnostic tool in DD. Determining whether the association of a filtered variant at a specific locus is a plausible explanation of the phenotype in the proband is crucial and commonly requires extensive manual literature review by both clinical scientists and clinicians. Access to a database of weighted clinical features extracted from rigorously curated literature would increase the efficiency of this process and facilitate the development of robust phenotypic similarity metrics. However, given the large and rapidly increasing volume of published information, conventional biocuration approaches are becoming impractical. Here, we present a scalable, automated method for the extraction of categorical phenotypic descriptors from the full-text literature. Papers identified through literature review were downloaded and parsed using the Cadmus custom retrieval package. Human Phenotype Ontology terms were extracted using MetaMap, with 76-84% precision and 65-73% recall. Mean terms per paper increased from 9 in title + abstract, to 68 using full text. We demonstrate that these literature-derived disease models plausibly reflect true disease expressivity more accurately than widely used manually curated models, through comparison with prospectively gathered data from the Deciphering Developmental Disorders study. The area under the curve for receiver operating characteristic (ROC) curves increased by 5-10% through the use of literature-derived models. This work shows that scalable automated literature curation increases performance and adds weight to the need for this strategy to be integrated into informatic variant analysis pipelines. Database URL: https://doi.org/10.1093/database/baac038.


Assuntos
Deficiências do Desenvolvimento , Publicações , Criança , Mineração de Dados/métodos , Bases de Dados Factuais , Deficiências do Desenvolvimento/genética , Humanos , Curva ROC
5.
Neurologia (Engl Ed) ; 2021 Mar 13.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-33726968

RESUMO

INTRODUCTION: Cerebral amyloid angiopathy-related inflammation (CAA-ri) is an entity characterised by an inflammatory response to ß-amyloid deposition in the walls of cerebral microvessels. METHODS: We conducted a retrospective review of a series of patients with a diagnosis of CAA-ri according to histopathological study findings or clinical-radiological diagnostic criteria. RESULTS: The study included 7 patients (5 men) with a mean age of 79 years. Disease onset was acute or subacute in 6 patients. The most frequent symptoms were cognitive impairment (n = 6), behavioural alterations (n = 5), epileptic seizures (n = 5), focal neurological signs (n = 4), and headache (n = 2). Cerebrospinal fluid was abnormal in 3 patients (lymphocytic pleocytosis and high protein levels). The most frequent MRI findings were microbleeds (n = 7), subcortical white matter hyperintensities on T2-FLAIR sequences (n = 7), and leptomeningeal enhancement (n = 6). Lesions were bilateral in 3 patients and most frequently involved the parieto-occipital region (n = 5). Amyloid PET studies were performed in 2 patients, one of whom showed pathological findings. Two patients underwent brain biopsy, which confirmed diagnosis. All patients received immunosuppressive therapy. An initially favourable clinical-radiological response was observed in all cases, with 2 patients presenting radiological recurrence after treatment withdrawal, with a subsequent improvement after treatment was resumed. CONCLUSIONS: Early diagnosis of CAA-ri is essential: early treatment has been shown to improve prognosis and reduce the risk of recurrence. Although a histopathological study is needed to confirm diagnosis, clinical-radiological criteria enable diagnosis without biopsy.

6.
Neuropathol Appl Neurobiol ; 47(2): 283-296, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-32896923

RESUMO

AIMS: Recessive variants in CAPN3 gene are the cause of the commonest form of autosomal recessive limb girdle muscle dystrophy. However, two distinct in-frame deletions in CAPN3 (NM_000070.3:c.643_663del21 and c.598_621del15) and more recently, Gly445Arg and Arg572Pro substitutions have been linked to autosomal dominant (AD) forms of calpainopathy. We report 21 affected individuals from seven unrelated families presenting with an autosomal dominant form of muscular dystrophy associated with five different heterozygous missense variants in CAPN. METHODS: We have used massively parallel gene sequencing (MPS) to determine the genetic basis of a dominant form of limb girdle muscular dystrophy in affected individuals from seven unrelated families. RESULTS: The c.700G> A, [p.(Gly234Arg)], c.1327T> C [p.(Ser443Pro], c.1333G> A [p.(Gly445Arg)], c.1661A> C [p.(Tyr554Ser)] and c.1706T> C [p.(Phe569Ser)] CAPN3 variants were identified. Affected individuals presented in young adulthood with progressive proximal and axial weakness, waddling walking and scapular winging or with isolated hyperCKaemia. Muscle imaging showed fatty replacement of paraspinal muscles, variable degrees of involvement of the gluteal muscles, and the posterior compartment of the thigh and minor changes at the mid-leg level. Muscle biopsies revealed mild myopathic changes. Western blot analysis revealed a clear reduction in calpain 3 in skeletal muscle relative to controls. Protein modelling of these variants on the predicted structure of calpain 3 revealed that all variants are located in proximity to the calmodulin-binding site and are predicted to interfere with proteolytic activation. CONCLUSIONS: We expand the genotypic spectrum of CAPN3-associated muscular dystrophy due to autosomal dominant missense variants.


Assuntos
Calpaína/genética , Predisposição Genética para Doença/genética , Proteínas Musculares/genética , Distrofia Muscular do Cíngulo dos Membros/genética , Adolescente , Adulto , Feminino , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Masculino , Pessoa de Meia-Idade , Mutação de Sentido Incorreto , Linhagem , Análise de Sequência de DNA , Adulto Jovem
8.
Clin Transl Oncol ; 21(10): 1413-1423, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30877636

RESUMO

BACKGROUND: Some phase 2 trials had reported encouraging progression-free survival with Bevacizumab in monotherapy or combined with chemotherapy in glioblastoma. However, phase 3 trials showed a significant improvement in progression free survival without a benefit in overall survival. To date, there are no predictive biomarker of response for Bevacizumab in glioblastoma. METHODS: We used Immunochemical analysis on tumor samples and pretreatment and post-treatment perfusion-MRI to try to identify possible predictive angiogenesis-related biomarkers of response and survival in patients with glioblastoma treated with bevacizumab in the first recurrence. We analyzed histological parameters: vascular proliferation, mitotic number and Ki-67 index; molecular factors: MGMT promoter methylation, EGFR amplification and EGFR variant III; immunohistochemical: MET, Midkine, HIF1, VEGFA, VEGF-R2, CD44, Olig2, microvascular area and microvascular density; and radiological: rCBV. RESULTS: In the statistical analysis, no significant correlation of any histological, molecular, microvascular or radiological parameters could be demonstrated with the response rate, PFS or OS with bevacizumab treatment. CONCLUSION: Unfortunately, in this histopathological, molecular, immunohistochemical and neuroradiological study we did not find any predictive biomarker of response or survival benefit for Bevacizumab in glioblastoma.


Assuntos
Inibidores da Angiogênese/uso terapêutico , Antineoplásicos Imunológicos/uso terapêutico , Bevacizumab/uso terapêutico , Neoplasias Encefálicas/tratamento farmacológico , Glioblastoma/tratamento farmacológico , Recidiva Local de Neoplasia/tratamento farmacológico , Adulto , Idoso , Biomarcadores Tumorais/análise , Neoplasias Encefálicas/irrigação sanguínea , Neoplasias Encefálicas/química , Neoplasias Encefálicas/diagnóstico por imagem , Circulação Cerebrovascular , Metilases de Modificação do DNA/metabolismo , Enzimas Reparadoras do DNA/metabolismo , Feminino , Amplificação de Genes , Genes erbB-1 , Glioblastoma/irrigação sanguínea , Glioblastoma/química , Glioblastoma/diagnóstico por imagem , Humanos , Imuno-Histoquímica , Antígeno Ki-67/análise , Masculino , Metilação , Microvasos/patologia , Pessoa de Meia-Idade , Índice Mitótico , Recidiva Local de Neoplasia/irrigação sanguínea , Recidiva Local de Neoplasia/química , Recidiva Local de Neoplasia/diagnóstico por imagem , Estudos Retrospectivos , Análise Serial de Tecidos , Proteínas Supressoras de Tumor/metabolismo
9.
AJNR Am J Neuroradiol ; 40(4): 634-640, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30923085

RESUMO

BACKGROUND AND PURPOSE: Multifocal glioblastomas (ie, glioblastomas with multiple foci, unconnected in postcontrast pretreatment T1-weighted images) represent a challenge in clinical practice due to their poor prognosis. We wished to obtain imaging biomarkers with prognostic value that have not been found previously. MATERIALS AND METHODS: A retrospective review of 1155 patients with glioblastomas from 10 local institutions during 2006-2017 provided 97 patients satisfying the inclusion criteria of the study and classified as having multifocal glioblastomas. Tumors were segmented and morphologic features were computed using different methodologies: 1) measured on the largest focus, 2) aggregating the different foci as a whole, and 3) recording the extreme value obtained for each focus. Kaplan-Meier, Cox proportional hazards, correlations, and Harrell concordance indices (c-indices) were used for the statistical analysis. RESULTS: Age (P < .001, hazard ratio = 2.11, c-index = 0.705), surgery (P < .001, hazard ratio = 2.04, c-index = 0.712), contrast-enhancing rim width (P < .001, hazard ratio = 2.15, c-index = 0.704), and surface regularity (P = .021, hazard ratio = 1.66, c-index = 0.639) measured on the largest focus were significant independent predictors of survival. Maximum contrast-enhancing rim width (P = .002, hazard ratio = 2.05, c-index = 0.668) and minimal surface regularity (P = .036, hazard ratio = 1.64, c-index = 0.600) were also significant. A multivariate model using age, surgery, and contrast-enhancing rim width measured on the largest foci classified multifocal glioblastomas into groups with different outcomes (P < .001, hazard ratio = 3.00, c-index = 0.853, median survival difference = 10.55 months). Moreover, quartiles with the highest and lowest individual prognostic scores based on the focus with the largest volume and surgery were identified as extreme groups in terms of survival (P < .001, hazard ratio = 18.67, c-index = 0.967). CONCLUSIONS: A prognostic model incorporating imaging findings on pretreatment postcontrast T1-weighted MRI classified patients with glioblastoma into different prognostic groups.


Assuntos
Neoplasias Encefálicas/classificação , Neoplasias Encefálicas/patologia , Glioblastoma/classificação , Glioblastoma/patologia , Adulto , Idoso , Neoplasias Encefálicas/diagnóstico por imagem , Feminino , Glioblastoma/diagnóstico por imagem , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Prognóstico , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Análise de Sobrevida
10.
Clin Transl Oncol ; 20(7): 937, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-29858734

RESUMO

The SEOM/GEINO clinical guidelines provide recommendations for radiological, and molecular diagnosis, treatment and follow-up of adult patients with anaplastic gliomas (AG). We followed the 2016 WHO classification which specifies the major diagnostic/prognostic and predictive value of IDH1/IDH2 missense mutations and 1p/19q codeletions in AG. The diagnosis of anaplastic oligoastrocytoma is discouraged. Surgery, radiotherapy and chemotherapy with PCV or TMZ are the first-line standard of care for AG with slight modifications according to molecular variables. A multidisciplinary team is highly recommended in the management of these tumors.

11.
Clin Transl Oncol ; 20(1): 108-109, 2018 01.
Artigo em Inglês | MEDLINE | ID: mdl-29209952

RESUMO

The original version of this article unfortunately contained a mistake. Figure 3 was incorrect.

12.
Clin Transl Oncol ; 20(1): 16-21, 2018 01.
Artigo em Inglês | MEDLINE | ID: mdl-29058264

RESUMO

The SEOM/GEINO clinical guidelines provide recommendations for radiological, and molecular diagnosis, treatment and follow-up of adult patients with anaplastic gliomas (AG). We followed the 2016 WHO classification which specifies the major diagnostic/prognostic and predictive value of IDH1/IDH2 missense mutations and 1p/19q codeletions in AG. The diagnosis of anaplastic oligoastrocytoma is discouraged. Surgery, radiotherapy and chemotherapy with PCV or TMZ are the first-line standard of care for AG with slight modifications according to molecular variables. A multidisciplinary team is highly recommended in the management of these tumors.


Assuntos
Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/terapia , Glioma/diagnóstico , Glioma/terapia , Humanos
13.
Clin Transl Oncol ; 20(1): 3-15, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-29124520

RESUMO

Diffuse infiltrating low-grade gliomas include oligodendrogliomas and astrocytomas, and account for about 5% of all primary brain tumors. Treatment strategies for these low-grade gliomas in adults have recently changed. The 2016 World Health Organization (WHO) classification has updated the definition of these tumors to include their molecular characterization, including the presence of isocitrate dehydrogenase (IDH) mutation and 1p/19p codeletion. In this new classification, the histologic subtype of grade II-mixed oligoastrocytoma has also been eliminated. The precise optimal management of patients with low-grade glioma after resection remains to be determined. The risk-benefit ratio of adjuvant treatment must be weighed for each individual.


Assuntos
Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/terapia , Glioma/diagnóstico , Glioma/terapia , Humanos
14.
Cir Pediatr ; 30(1): 57-63, 2017 Jan 25.
Artigo em Espanhol | MEDLINE | ID: mdl-28585792

RESUMO

INTRODUCTION: Up to date the classical therapeutic approach for intralobar sequestrations (IS) has been surgical excision. However, systemic arteries embolization is presented as an alternative, and even constitutes the first line of treatment in some centers. We summarize our experience in selected cases with the aim of preserving the maximum lung parenchyma. MATERIAL AND METHODS: Retrospective study of IS who underwent endovascular embolization at our institution between 2013 and 2014. RESULTS: Three patients of 12, 14 and 21 months old were treated. Two patients had unilateral IS, one in the left lower lobe (LLL) and the other in the right lower lobe (RLL); the third one had bilateral lesions (a CAM-S complex in the RLL and a IS in the LII). The embolization of the four lesions was performed via right femoral artery. The case with bilateral lesions underwent thoracoscopic lobectomy of the CAM-S 7 months after embolization, finding adherences to the diaphragm. None of the patients had immediate complications and were discharged within 48 hours after embolization. In successive ultrasound and plain radiographs controls, with a follow-up of 6, 18 and 30 months, no complications were found. CONCLUSIONS: Systemic vessels embolization is a treatment option for the treatment of IS that avoids surgery, preserves lung parenchyma and does not preclude surgical resection in case of treatment failure or presence of parenchymal lesions. Longer follow-up is needed to determine long-term effectiveness.


INTRODUCCION: El tratamiento de elección de los secuestros intralobares (SI) es la lobectomía pulmonar. La mayoría de complicaciones se asocian a la vascularización sistémica pulmonar anómala. La embolización de las arterias sistémicas se presenta como una alternativa terapéutica, que incluso constituye la primera línea de tratamiento en algunos centros. Resumimos nuestra experiencia al aplicar esta técnica en casos seleccionados. MATERIAL Y METODOS: Estudio retrospectivo de los casos con SI tratados en nuestro centro mediante embolización entre 2013 y 2014, centrado en las indicaciones, resultados, complicaciones y seguimiento. Siendo la lobectomía la primera línea de tratamiento la embolización se reservó para casos seleccionados: bilaterales, con el objetivo de preservar parénquima pulmonar o ante el rechazo familiar a la intervención quirúrgica. RESULTADOS: Se trataron tres pacientes de 12, 14 y 21 meses. Dos presentaban un SI unilateral, uno en lóbulo inferior izquierdo (LII) y otro en el lóbulo inferior derecho (LID); el tercero lesiones bilaterales (un SI en LID y un complejo MAQ-secuestro en LII). Se embolizaron las cuatro lesiones por punción de arteria femoral derecha. El caso con lesiones bilaterales fue intervenido a los 7 meses tras la embolización, realizándose una lobectomía toracoscópica del complejo MAQ-secuestro, hallando adherencias al diafragma. Ninguno presentó complicaciones inmediatas y fueron dados de alta a las 48 horas. En los controles realizados con ecografía y radiografías simples, con seguimientos de 31, 47 y 56 meses, no se han observado complicaciones clínicas ni radiológicas. CONCLUSIONES: La embolización de los vasos sistémicos es una opción en el tratamiento del SI que permite evitar una cirugía, conservar parénquima pulmonar y no impide la exéresis quirúrgica en caso de fallo del tratamiento o lesión parenquimatosa. Es preciso un mayor seguimiento para determinar la efectividad a largo plazo.


Assuntos
Sequestro Broncopulmonar/terapia , Embolização Terapêutica/métodos , Toracoscopia/métodos , Sequestro Broncopulmonar/fisiopatologia , Diafragma/patologia , Feminino , Seguimentos , Humanos , Lactente , Masculino , Estudos Retrospectivos , Resultado do Tratamento
16.
Clin Transl Oncol ; 19(1): 51-57, 2017 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-27026567

RESUMO

BACKGROUND AND PURPOSE: In glioblastoma, tumor progression appears to be triggered by expression of VEGF, a regulator of blood vessel permeability. Bevacizumab is a monoclonal antibody that inhibits angiogenesis by clearing circulating VEGF, resulting in a decline in the contrast-enhancing tumor, which does not always correlate with treatment response. Our objectives were: (1) to evaluate whether changes in DSC perfusion MRI-derived leakage could predict survival in recurrent glioblastoma, and (2) to estimate whether leakage at baseline was related to treatment outcome. MATERIALS AND METHODS: We retrospectively analyzed DSC perfusion MRI in 24 recurrent glioblastomas treated with bevacizumab as second line chemotherapy. Leakage at baseline and changes in maximum leakage between baseline and the first follow-up after treatment were selected for quantitative analysis. Survival univariate analysis was made constructing survival curves using Kaplan-Meier method and comparing subgroups by log rank probability test. RESULTS: Leakage reduction at 8 weeks after initiation of bevacizumab treatment had a significant influence on overall survival (OS) and progression-free survival (PFS). Median OS and PFS were 2.4 and 2.8 months longer for patients with leakage reduction at the first follow-up. Higher leakage at baseline was associated with leakage reduction after treatment. Odds ratio of treatment response was 9 for patients with maximum leakage at baseline >5. CONCLUSIONS: Leakage decrease may predict OS and PFS in recurrent glioblastomas treated with bevacizumab. Leakage reduction postulates as a potential biomarker for treatment response evaluation. Leakage at baseline seems to predict response to treatment, but was not independently associated with survival.


Assuntos
Inibidores da Angiogênese/uso terapêutico , Bevacizumab/uso terapêutico , Neoplasias Encefálicas/mortalidade , Meios de Contraste , Glioblastoma/mortalidade , Imageamento por Ressonância Magnética/métodos , Recidiva Local de Neoplasia/mortalidade , Adulto , Idoso , Neoplasias Encefálicas/tratamento farmacológico , Neoplasias Encefálicas/metabolismo , Neoplasias Encefálicas/patologia , Feminino , Seguimentos , Glioblastoma/tratamento farmacológico , Glioblastoma/metabolismo , Glioblastoma/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/tratamento farmacológico , Recidiva Local de Neoplasia/metabolismo , Recidiva Local de Neoplasia/patologia , Estadiamento de Neoplasias , Perfusão , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida
17.
Cir Pediatr ; 29(1): 19-24, 2016 Jan 25.
Artigo em Espanhol | MEDLINE | ID: mdl-27911066

RESUMO

INTRODUCTION: The liver is the second most frequently involved organ in abdominal trauma in children. The aim of this study was to review the characteristics of liver traumas (LT) in children, its treatment and complications. MATERIAL AND METHODS: Retrospective study of patients with LT treated between 2010-2014. We analyzed data regarding diagnosis, conservative management (CM), complications and treatment. RESULTS: Twenty-four patients of LT with a mean age of 9.8 years (SD: 3.4) were treated at our center. Liver injury degrees according to the Organ Injury Scale of American Association for Surgery of Trauma (AAST) were: grade I (4), grade II (6), grade III (8), grade IV (5) and grade V (1). CT angiography was performed in 23 patients. Four of the six patients with initial hemodynamic instability required blood transfusion; of those, two required angioembolization for active bleeding in extrahepatic locations and a third required damage control surgery. Three patients presented late-onset complications: 2 large size bilomas which underwent spontaneous resolution and a third patient with a right biloma and section of the left bile duct who required a multidisciplinary approach. Median hospital stay was 6 days (r 1-92). With a mean follow-up of 17.75 months (SD: 16.35) (r: 2-57) long-term survival was 96%. The patient who received surgery died due to a post-traumatic brain injury. CONCLUSION: CM seems to be the treatment of choice in LT regardless of injury degree. Surgery should be reserved for cases refractory to CM due to the associated high mortality.


INTRODUCCION: El hígado es el segundo órgano más afectado en traumatismos abdominales en edad pediátrica. El objetivo de este trabajo es revisar las características de los traumatismos hepáticos (TH), su tratamiento y complicaciones. MATERIAL Y METODOS: Estudio retrospectivo de pacientes con TH atendidos entre 2010 -2014. Analizamos datos referentes al diagnóstico, tratamiento conservador (TC), complicaciones y tratamiento de las mismas. RESULTADOS: Se trataron 24 casos de TH con una edad media de 9,8 años (DS: 3,4). Los grados de lesiones hepáticas según la Organ Injury Scale of American Association for Surgery of Trauma (AAST) fueron: grado I (4), grado II (6), grado III (8), grado IV (5) y grado V (1). Se realizó angio-TC en 23 pacientes. Cuatro de los 6 pacientes que presentaron inestabilidad hemodinámica inicial requirieron transfusión de hemoderivados; de éstos, 2 requirieron angioembolización por sangrado activo extrahepático y un tercero se intervino de forma urgente. Tres pacientes presentaron complicaciones tardías: 2 bilomas de gran tamaño que se resolvieron espontáneamente y un biloma derecho con sección de vía biliar izquierda que requirió un abordaje multidisciplinar. En los 3 casos se trataba de traumatismos de alto grado (2 grado IV, 1 grado V). La mediana de estancia hospitalaria fue de 6 días (r: 1-92). Con una media de seguimiento de 17,75 meses (DS: 16,35) (r: 2-57) la supervivencia global fue del 96%. El paciente que requirió cirugía falleció por el traumatismo craneoencefálico asociado. CONCLUSIONES: El TC parece ser de primera elección en los TH independientemente del grado aunque no está exento de complicaciones. La cirugía debe reservarse para los casos refractarios al TC dada la alta mortalidad que conlleva.


Assuntos
Tratamento Conservador , Fígado/lesões , Criança , Hemorragia/terapia , Humanos , Escala de Gravidade do Ferimento , Complicações Pós-Operatórias , Padrões de Referência , Estudos Retrospectivos , Ferimentos e Lesões/classificação , Ferimentos e Lesões/terapia
18.
Cir Pediatr ; 29(1): 25-30, 2016 Jan 25.
Artigo em Espanhol | MEDLINE | ID: mdl-27911067

RESUMO

INTRODUCTION: The management of active bleeding with haemodinamic lability in the paediatric trauma patient is difficult and generally leads to damage control surgery. Vascular Interventional Radiology (VIR) techniques are useful for the diagnosis as for the definitive treatment. AIM: The aim of our study was to describe our experience and evaluate effectiveness of VIR in the management of the paediatric trauma patient with active bleeding signs. METHODS: Retrospective analysis (2003-2014) of politraumatic patients who showed contrast blush on computed tomography and then treated by VIR techniques. RESULTS: In the reported study period 16 patients underwent VIR procedures. Medium age was 13 years (5-17). The most frequent lesion mechanism was traffic accident (8 out of 17) and 93,75% were blunt traumas. Findings on initial Computed Tomography were 12 contrast blushes and 2 absences of arterial flow. In 2 cases the contrast blush appeared 48 hours after the accident. Arteriography allowed us to localize the bleeding vessels in all the cases, performing selective or supraselective renal (7), pelvic (5), hepatic (3), splenic (1) and intercostal (1) embolization. One patient required an endoprothesis for renal revascularization. Two cases needed additional surgical procedures (2 nephrectomies) because of complete section of the renal artery (1) and disruption of the ureteropelvic junction (1). One case required hemofiltration in relation to rhabdomyolysis. CONCLUSION: In our experience VIR is a valuable diagnostic and therapeutic procedure for the management of paediatric trauma patients, with high effectiveness and a low complication rate.


INTRODUCCION: El tratamiento del sangrado activo en niños politraumatizados con labilidad hemodinámica es difícil y generalmente obliga a realizar una cirugía de control de daños. La aplicación de técnicas de Radiología Vascular Intervencionista (RVI) ayuda al diagnóstico y tratamiento definitivo. OBJETIVO: Describir nuestra experiencia y valorar la eficacia de la RVI en el tratamiento del paciente traumático pediátrico con signos de sangrado activo. MATERIAL Y METODO: Análisis retrospectivo (2003-2014) de los pacientes politraumatizados tratados mediante RVI en los cuales la AngioTC mostraba fuga de contraste o ausencia de captación. RESULTADOS: En el periodo de estudio se trataron 16 pacientes, con una media de edad de 13 años (5-17). El mecanismo lesional más frecuente fue el accidente de tráfico. El 93,75% fueron traumatismos cerrados. Los hallazgos de la angioTC inicial fueron 12 sangrados activos, 2 ausencias de flujo arterial. Dos sangrados se produjeron a las 48 horas del trauma. La arteriografía permitió localizar los vasos sangrantes, realizándose embolización selectiva o supraselectiva a nivel renal (7), pélvico (5), hepático (3), esplénico (1) e intercostal (1). Un paciente preciso revascularización renal con endoprótesis. Un paciente requirió nefrectomía urgente tras la angiografía por avulsión arterial completa y en 1 caso se realizó nefrectomía a las 48 horas por fuga de contraste y disrupción de la unión pieloureteral. En un caso se observó rabdomiólisis como complicación que requirió hemofiltración. CONCLUSION: La RVI constituye un procedimiento diagnóstico y terapéutico en el abordaje del paciente pediátrico traumático, con alta efectividad y escasas complicaciones, siendo una herramienta fundamental en un centro de trauma pediátrico.


Assuntos
Hemorragia/diagnóstico por imagem , Traumatismo Múltiplo/diagnóstico por imagem , Radiologia Intervencionista/métodos , Ferimentos não Penetrantes/diagnóstico por imagem , Adolescente , Angiografia , Criança , Pré-Escolar , Embolização Terapêutica/métodos , Humanos , Nefrectomia , Artéria Renal/lesões , Artéria Renal/cirurgia , Estudos Retrospectivos , Tomografia Computadorizada por Raios X
19.
Cir Pediatr ; 28(4): 165-171, 2015 Oct 10.
Artigo em Espanhol | MEDLINE | ID: mdl-27775292

RESUMO

INTRODUCTION: Paediatric LTI is associated with significant morbid-mortality. Although historically first line treatment was surgical, conservative management is making headway. The purpose of this study was to analyze the management and surgical indications of LTI treated at our institution. MATERIAL AND METHODS: Retrospective study of patients with LTI treated between 2007 and 2014. RESULTS: Six out of seven patients, with a median age of 4 years (2-15), had blunt traumas and one had an open trauma. Respiratory distress, pain and subcutaneous emphysema were presented in 71.4% of the patients. Five children were intubated (3 pre-hospital care and 2 after reaching the hospital), all of them underwent fibrobronchoscopy, determining the location, the extention, and assessing the possibility of extubation. Esophagoscopy was made in 2 cases. The remaining 2 cases were bounded to observation as they did not have respiratory symptoms or progressive emphysema, precluding bronchoscopy. Conservative management was followed in 71.4% of the patients, with a 100% success rate. Conservative management criteria were no progression of emphysema or respiratory distress, regardless the degree and location of the lesions. Two patients needed surgery: one tracheoesophageal fistula caused by a button battery, and the other was a tracheal open section associated with vascular injury. Both of them recovered uneventfully. CONCLUSIONS: If the respiratory symptoms or the emphysema do not progress, patients with LTI can be managed conservatively, regardless of size or location of the injury. Associated esophageal lesions and open injuries require surgical management. An early diagnosis is mandatory in order to minimize morbidity and mortality.


INTRODUCCION: Las lesiones laringotraqueales traumáticas pediátricas están asociadas a una importante morb-imortalidad. Aunque históricamente el tratamiento ha sido quirúrgico, en la actualidad el enfoque conservador ha adquirido una mayor importancia. Valoramos el tratamiento y las indicaciones quirúrgicas de los traumatismos laringotraqueales tratados en nuestro centro. MATERIAL Y METODOS: Análisis retrospectivo de pacientes con traumatismos laringotraqueales del 2007 al 2014. RESULTADOS: Siete pacientes, mediana de edad 4 años (r: 2-15), 6 traumatismos cerrados y 1 abierto. El 71,4% presentaban dificultad respiratoria, dolor y enfisema subcutáneo. Cinco fueron intubados (3 en prehospitalaria y 2 en hospital). En todos ellos se realizó fibrobroncoscopia determinando la localización, la extensión de la lesión y la posibilidad de extubación. En dos de ellos se asoció esofagoscopia. Dos casos no presentaban clínica respiratoria y el enfisema no progresó por lo que se tomó una actitud expectante y no precisaron broncoscopia. El 71,4% se trataron conservadoramente, siendo el criterio de tratamiento conservador la no progresión del enfisema ni empeoramiento del estado respiratorio, independientemente del grado y localización de la lesión, con una tasa de éxito del 100%. Dos pacientes se intervinieron: una fístula tráqueo-esofágica producida por una quemadura (pila de botón) que requirió resección traqueal; y una sección traqueal abierta asociada a lesión vascular, ambas con correcta evolución posterior. CONCLUSIONES: En caso de no progresión de la clínica respiratoria o del enfisema, e independientemente del grado y localización, las lesiones laringotraqueales pueden tratarse de manera conservadora con o sin intubación. Lesiones esofágicas asociadas y lesiones abiertas obligan a un abordaje quirúrgico. Un diagnóstico precoz minimiza la morbimortalidad.

20.
AJNR Am J Neuroradiol ; 35(6): 1096-102, 2014 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-24457819

RESUMO

BACKGROUND AND PURPOSE: Diffuse gliomas are classified as grades II-IV on the basis of histologic features, with prognosis determined mainly by clinical factors and histologic grade supported by molecular markers. Our aim was to evaluate, in patients with diffuse gliomas, the relationship of relative CBV and ADC values to overall survival. In addition, we also propose a prognostic model based on preoperative MR imaging findings that predicts survival independent of histopathology. MATERIALS AND METHODS: We conducted a retrospective analysis of the preoperative diffusion and perfusion MR imaging in 126 histologically confirmed diffuse gliomas. Median relative CBV and ADC values were selected for quantitative analysis. Survival univariate analysis was made by constructing survival curves by using the Kaplan-Meier method and comparing subgroups by log-rank probability tests. A Cox regression model was made for multivariate analysis. RESULTS: The study included 126 diffuse gliomas (median follow-up of 14.5 months). ADC and relative CBV values had a significant influence on overall survival. Median overall survival for patients with ADC < 0.799 × 10(-3) mm(2)/s was <1 year. Multivariate analysis revealed that patient age, relative CBV, and ADC values were associated with survival independent of pathology. The preoperative model provides greater ability to predict survival than that obtained by histologic grade alone. CONCLUSIONS: ADC values had a better correlation with overall survival than relative CBV values. A preoperative prognostic model based on patient age, relative CBV, and ADC values predicted overall survival of patients with diffuse gliomas independent of pathology. This preoperative model provides a more accurate predictor of survival than histologic grade alone.


Assuntos
Neoplasias Encefálicas/mortalidade , Neoplasias Encefálicas/patologia , Glioma/mortalidade , Glioma/patologia , Angiografia por Ressonância Magnética/estatística & dados numéricos , Modelos de Riscos Proporcionais , Neoplasias Encefálicas/cirurgia , Feminino , Glioma/cirurgia , Humanos , Incidência , Angiografia por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Modelagem Computacional Específica para o Paciente/estatística & dados numéricos , Cuidados Pré-Operatórios/métodos , Cuidados Pré-Operatórios/estatística & dados numéricos , Prognóstico , Reprodutibilidade dos Testes , Estudos Retrospectivos , Medição de Risco/métodos , Fatores de Risco , Sensibilidade e Especificidade , Espanha/epidemiologia , Análise de Sobrevida , Taxa de Sobrevida
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