MARIA CLAUDIA NOGUEIRA ZERBINI
Projetos de Pesquisa
Unidades Organizacionais
Departamento de Patologia, Faculdade de Medicina - Docente
LIM/14 - Laboratório de Investigação em Patologia Hepática, Hospital das Clínicas, Faculdade de Medicina
LIM/14 - Laboratório de Investigação em Patologia Hepática, Hospital das Clínicas, Faculdade de Medicina
3 resultados
Resultados de Busca
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- Prenatal detection and postnatal management of an intranasal glioma(2012) OKUMURA, Maria; FRANCISCO, Rossana Pulcineli Vieira; LUCATO, Leandro Tavares; ZERBINI, Maria Claudia Nogueira; ZUGAIB, MarceloNasal gliomas are rare benign congenital midline tumors composed of heterotopic neuroglial tissue. They have potential for intracranial extension through a bony defect in the skull base. Neuroimaging is essential for identifying nasal lesions and for determining their exact location and any possible intracranial extension. Computed tomography is often the initial imaging study obtained because it provides good visualization of the bony landmarks of the skull base; it is not, however, well suited for soft tissue imaging. Magnetic resonance imaging has better soft tissue resolution and may be the best initial study in patients seen early in life because the anterior skull base consists of an unossified cartilage and may falsely appear as if there is a bony dehiscence on computed tomography. A frontal craniotomy approach is recommended if intracranial extension is identified, followed by a transnasal endoscopic approach for intranasal glioma. A case is presented of a huge fetal facial mass that was shown by ultrasound that protruded through the left nostril at 33 weeks of gestation. Computed tomography of the neonate suggested a transethmoidal encephalocele. Magnetic resonance imaging showed a huge mass occupying the nasopharynx and the nasal cavity and protruding externally to the face but ruled out bony discontinuity in the skull base and, therefore, any intracranial connection. The infant underwent an endoscopic resection of the mass via oral and nasal routes and pathologic examination revealed intranasal glioma.
conferenceObject Confocal Microscopy Image Analysis of Pancreatic beta-Cells K-ATP Channel Proteins in Congenital Hyperinsulinism (CHI)(2012) LOVISOLO, S. M.; GARIPPO, A. L.; GUEDES, F.; ZERBINI, M. C.Background: CHI is a life-threatening disorder of glucose metabolism of neonates characterized by serum insulin levels unresponsive to blood glucose concentrations. Pancreatic ß-cells regulates insulin secretion through ATP sensitive potassium channels (KATP channel) formed by sulfonylurea receptor 1 (SUR1) and potassium inward rectifying 6.2 (Kir 6.2) proteins. The ß-cell K ATP channel dependent CHI is classically associated with loss-of-function mutations of these subunits genes. In a previous study [1], no mutations in the Kir6.2 gene and in the 33-37 exons hot spot region of the SUR1 gene were identified in these patients. The aim of this study is to investigate if these subunits were present in the ß-cells of CHI patients. Design: Pancreatic surgical paraffin tissue from 7 neonates (3F/4M, 4-13 mo) with CHI and 8 autopsy pancreatic control tissue (5F/3M, 4-11 mo) were included in the study. Confocal microscopy double-staining immunofluorescence (insulin/SUR1 and insulin/Kir6.2) was performed in order to detect each protein specifically in the ß-cells All sections were analyzed under a Zeiss 510 Meta confocal laser scanning microscope (63x). At least 10 different endocrine islets were captured to evaluate the expression of either Kir6.2 or SUR1 (green-488nm) and insulin (red-633nm). Co-expression of insulin/SUR1 and insulin/Kir6.2 was analysed visually (green x red→yellow) and through correlation Pearson’s coefficient(PC) that estimates the goodness of rate association of the two fluorochromes. PC was compared among cases and controls using a nonparametric method (Mann-Whitney). Results: Visual observation clearly revealed the presence of Kir6.2 and SUR1 in a granular cytoplasmic pattern in the ß-cells of cases and controls. Nevertheless, overlap of insulin/Kir6.2 and insulin/SUR1 seemed to be more constant and uniform in controls than in CHI cases, confirmed by the analysis through PC showing a statistically significant decrease in Kir6.2 (P= 0.0084) and SUR1 (P= 0.041) in the ß-cells of CHI patients. Conclusions: This is the first demonstration of K ATP channels subunits Kir6.2 and SUR1 in the pancreatic ß-cells of CHI patients using an in situ method. Our results show that both subunits were present in the ß-cells, but are under-expressed in CHI patients compared to controls, adding a new information to this rare condition with a complex pathogenesis.conferenceObject Confocal Microscopy Image Analysis of Pancreatic beta-Cells K-ATP Channel Proteins in Congenital Hyperinsulinism (CHI)(2012) LOVISOLO, S. M.; GARIPPO, A. L.; GUEDES, F.; ZERBINI, M. C.Background: CHI is a life-threatening disorder of glucose metabolism of neonates characterized by serum insulin levels unresponsive to blood glucose concentrations. Pancreatic ß-cells regulates insulin secretion through ATP sensitive potassium channels (K ATP channel) formed by sulfonylurea receptor 1 (SUR1) and potassium inward rectifying 6.2 (Kir 6.2) proteins. The ß-cell K ATP channel dependent CHI is classically associated with loss-of-function mutations of these subunits genes. In a previous study [1], no mutations in the Kir6.2 gene and in the 33-37 exons hot spot region of the SUR1 gene were identified in these patients. The aim of this study is to investigate if these subunits were present in the ß-cells of CHI patients. Design: Pancreatic surgical paraffin tissue from 7 neonates (3F/4M, 4-13 mo) with CHI and 8 autopsy pancreatic control tissue (5F/3M, 4-11 mo) were included in the study. Confocal microscopy double-staining immuno fluorescence (insulin/SUR1 and insulin/Kir6.2) was performed in order to detect each protein specifically in the ß-cells. All sections were analyzed under a Zeiss 510 Meta confocal laser scanning microscope (63x). At least 10 different endocrine islets were captured to evaluate the expression of either Kir6.2 or SUR1 (green-488nm) and insulin (red-633nm). Co-expression of insulin/SUR1 and insulin/Kir6.2 was analysed visually (green x red→yellow) and through correlation Pearson’s coefficient(PC) that estimates the goodness of rate association of the two fluorochromes. PC was compared among cases and controls using a nonparametric method (Mann-Whitney). Results: Visual observation clearly revealed the presence of Kir6.2 and SUR1 in a granular cytoplasmic pattern in the ß-cells of cases and controls. Nevertheless, overlap of insulin/Kir6.2 and insulin/SUR1 seemed to be more constant and uniformin controls than in CHI cases, confirmed by the analysis through PC showing a statistically significant decrease in Kir6.2 ( P = 0.0084) and SUR1 ( P = 0.041) in the ß-cells of CHI patients. Conclusions: This is the first demonstration of K ATP channels subunits Kir6.2 and SUR1 in the pancreatic ß-cells of CHI patients using an in situ method. Our results show that both subunits were present in the ß-cells, but are under-expressed in CHI patients compared to controls, adding a new information to this rare condition with a complex pathogenesis.