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Sample records for nodular heterotopia pnh

  1. Epilepsy Outcome in Periventricular Nodular Heterotopia

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    J Gordon Millichap

    2004-07-01

    Full Text Available The clinical, electroencephalographic, and neuroimaging features and course of seizures in 16 patients with periventricular (subependymal nodular heterotopia (PNH were investigated at the University of Bologna, Italy.

  2. Pseudotemporal Epilepsy with Periventricular Nodular Heterotopia

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    Aysun Ünal

    2006-04-01

    Full Text Available Scientific background: Periventricular nodular heterotopia (PNH that is one of the subgroups of neuronal migration disorders is associated with intractable epilepsy. However the etiopathogenesis of seizures in PNH patients are still unclear. Heterotopias are not involved in the epileptic network, alone. A complex relation between nodules, hippocampus and neocortex has been reported as the cause. Case: A 20 year-old male patient with intractable seizures is presented with ictal and interictal EEG findings compatible with right temporal lobe seizure. Although his first MRI was reported as hyperintensity in right hippocampus, repeated MRI examination according to epilepsy protocol revealed bilateral PNH, with no signal change in hippocampus. Conclusion: The aim of this case report is to discuss the importance of PNH in the differential diagnosis of intractable seizures, and to mention that neurologists and radiologists can easily miss these lesions, if the walls of ventricles are not checked carefully

  3. Periventricular Nodular Heterotopia and Epilepsy

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    J Gordon Millichap

    2006-02-01

    Full Text Available The clinical, MRI, and EEG findings in 54 patients (35 female, 19 male; aged 1 to 64 years with periventricular nodular heterotopia (PNH were analyzed in relation to epileptic outcome and genesis of epileptic discharges, in a study at the Neurological Institute and Epilepsy Surgery Center, Niguarda General Hospital, Milan, Italy.

  4. Bilateral periventricular nodular heterotopia – neonatal cranial ultrasound.

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    A R Horn

    2010-03-01

    Full Text Available A female neonate was delivered at 30 weeks’ gestation to a mother with suspected bilateral periventricular nodular heterotopia (PNH on computed tomography brain scan. Antenatal ultrasound (US performed at 28 weeks’ gestation showed mild lateral cerebral ventricle dilatation, but no other fetal anomalies. Clinical examination revealed mild dysmorphic features: facial and skull asymmetry, square-shaped ears, and long tapering fingers. Chromosomal analysis showed a 46XX karyotype.

  5. Pathophysiological analyses of periventricular nodular heterotopia using gyrencephalic mammals.

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    Matsumoto, Naoyuki; Hoshiba, Yoshio; Morita, Kazuya; Uda, Natsu; Hirota, Miwako; Minamikawa, Maki; Ebisu, Haruka; Shinmyo, Yohei; Kawasaki, Hiroshi

    2017-03-15

    Although periventricular nodular heterotopia (PNH) is often found in the cerebral cortex of people with thanatophoric dysplasia (TD), the pathophysiology of PNH in TD is largely unknown. This is mainly because of difficulties in obtaining brain samples of TD patients and a lack of appropriate animal models for analyzing the pathophysiology of PNH in TD. Here we investigate the pathophysiological mechanisms of PNH in the cerebral cortex of TD by utilizing a ferret TD model which we recently developed. To make TD ferrets, we electroporated fibroblast growth factor 8 (FGF8) into the cerebral cortex of ferrets. Our immunohistochemical analyses showed that PNH nodules in the cerebral cortex of TD ferrets were mostly composed of cortical neurons, including upper layer neurons and GABAergic neurons. We also found disorganizations of radial glial fibers and of the ventricular lining in the TD ferret cortex, indicating that PNH may result from defects in radial migration of cortical neurons along radial glial fibers during development. Our findings provide novel mechanistic insights into the pathogenesis of PNH in TD. © The Author 2017. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  6. Sporadic periventricular nodular heterotopia: Classification, phenotype and correlation with Filamin A mutations.

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    Liu, Wenyu; Yan, Bo; An, Dongmei; Xiao, Jiahe; Hu, Fayun; Zhou, Dong

    2017-07-01

    The purpose of this study was to better delineate the clinical spectrum of periventricular nodular heterotopia (PNH) in a large patient population after long term follow up. Specifically, this study aimed to relate PNH subtypes to clinical or epileptic outcomes, epileptic discharges and underlying Filamin A (FLNA) mutations by analyzing anatomical features. The study included 100 patients with radiologically confirmed nodular heterotopia. Patients' FLNA gene sequences and medical records were analyzed. Two-sided Chi-square test and Fisher's exact t-test were used to assess associations between the distribution of PNHs and specific clinical features. Based on imaging data, patients were subdivided into three groups: (a) classical (bilateral frontal and body, n=41 patients), (b) bilateral asymmetrical or posterior (n=16) and (c) unilateral heterotopia (n=43). Most patients with classical heterotopia were females (P=0.033) and were likely to have arachnoid cysts (P=0.025) and cardiac abnormalities (P=0.041), but were mostly seizure-free. Additionally, hippocampal abnormalities (P=0.022), neurological deficits (P=0.028) and cerebellar abnormalities (P=0.005) were more common in patients with bilateral asymmetrical heterotopia. Patients with unilateral heterotopia were prone to develop refractory epilepsy (P=0.041). FLNA mutations were identified in 8 patients. Each group's distinctive genetic mutations, epileptic discharge patterns and overall clinical outcomes confirm that the proposed classification system is reliable. These findings could not only be an indicator of a more severe morphological and clinical phenotype, but could also have clinical implications with respect to the epilepsy management and optimization of therapeutic options. Copyright © 2017 Elsevier B.V. All rights reserved.

  7. Prenatal diagnosis of periventricular nodular heterotopia in borderline ventriculomegaly using sonography and magnetic resonance imaging.

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    Sahinoglu, Zeki; Yapicier, Ozlem; Ozcan, Nahit

    2016-10-01

    Periventricular nodular heterotopia (PNH) is usually missed on prenatal sonographic examinations, even on targeted scans. Irregular ventricular walls on axial view and irregular square-shaped lateral ventricles on coronal view are suggestive of PNH in the early third trimester. To achieve an early prenatal diagnosis, it is important to keep in mind the possible coexistence of PNH with brain malformations such as ventriculomegaly, posterior fossa anomalies, or agenesis of corpus callosum. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 44:510-513, 2016. © 2016 Wiley Periodicals, Inc.

  8. Paternal inheritance of classic X-linked bilateral periventricular nodular heterotopia.

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    Kasper, Burkhard S; Kurzbuch, Katrin; Chang, Bernard S; Pauli, Elisabeth; Hamer, Hajo M; Winkler, Jürgen; Hehr, Ute

    2013-06-01

    Periventricular nodular heterotopia (PNH) is a developmental disorder of the central nervous system, characterized by heterotopic nodules of gray matter resulting from disturbed neuronal migration. The most common form of bilateral PNH is X-linked dominant inherited, caused by mutations in the Filamin A gene (FLNA) and associated with a wide variety of other clinical findings including congenital heart disease. The typical patient with FLNA-associated PNH is female and presents with difficult to treat seizures. In contrast, hemizygous FLNA loss of function mutations in males are reported to be perinatally lethal. In X-linked dominant traits like FLNA-associated PNH the causal mutation is commonly inherited from the mother. Here, we present an exceptional family with paternal transmission of classic bilateral FLNA-associated PNH from a mildly affected father with somatic and germline mosaicism for a c.5686G>A FLNA splice mutation to both daughters with strikingly variable clinical manifestation and PNH extent in cerebral MR imaging. Our observations emphasize the importance to consider in genetic counseling and risk assessment the rare genetic constellation of paternal transmission for families with X-linked dominant inherited FLNA-associated PNH. Copyright © 2013 Wiley Periodicals, Inc.

  9. Region-specific connectivity in patients with periventricular nodular heterotopia and epilepsy: A study combining diffusion tensor imaging and functional MRI.

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    Liu, Wenyu; An, Dongmei; Tong, Xin; Niu, Running; Gong, Qiyong; Zhou, Dong

    2017-10-01

    Periventricular nodular heterotopia (PNH) is an important cause of chronic epilepsy. The purpose of this study was to evaluate region-specific connectivity in PNH patients with epilepsy and assess correlation between connectivity strength and clinical factors including duration and prognosis. Diffusion tensor imaging (DTI) and resting state functional MRI (fMRI) were performed in 28 subjects (mean age 27.4years; range 9-56years). The structural connectivity of fiber bundles passing through the manually-selected segmented nodules and other brain regions were analyzed by tractography. Cortical lobes showing functional correlations to nodules were also determined. For all heterotopic gray matter nodules, including at least one in each subject, the most frequent segments to which nodular heterotopia showed structural (132/151) and functional (146/151) connectivity were discrete regions of the ipsilateral overlying cortex. Agreement between diffusion tensor tractography and functional connectivity analyses was conserved in 81% of all nodules (122/151). In patients with longer duration or refractory epilepsy, the connectivity was significantly stronger, particularly to the frontal and temporal lobes (P<0.05). Nodules in PNH were structurally and functionally connected to the cortex. The extent is stronger in patients with longstanding or intractable epilepsy. These findings suggest the region-specific interactions may help better evaluate prognosis and seek medical or surgical interventions of PNH-related epilepsy. Copyright © 2017 Elsevier B.V. All rights reserved.

  10. Periventricular nodular heterotopia and bilateral intraventricular xanthogranulomas in 22q11.2 deletion syndrome

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    Moogeh Baharnoori

    2017-09-01

    Full Text Available 22q11.2 deletion syndrome (22q11DS is the most common pathogenic copy number variant in humans. Neuropsychiatric phenotypes, including schizophrenia, are prominent. Imaging studies of individuals with this syndrome show a variety of abnormalities that may indicate abnormal neuronal migration. Here we present the neuroimaging and neuropathologic features of a 22q11DS patient with bilateral periventricular nodular heterotopias (PNH and intraventricular xanthogranulomas that were identified by post-mortem examination.

  11. Clinical features and long term outcome of epilepsy in periventricular nodular heterotopia. Simple compared with plus forms

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    d'Orsi, G; Tinuper, P; Bisulli, F; Zaniboni, A; Bernardi, B; Rubboli, G; Riva, R; Michelucci, R; Volpi, L; Tassinari, C; Baruzzi, A

    2004-01-01

    Objectives: Little is known about the long term outcome of patients with periventricular nodular heterotopia (PNH) and epilepsy, particularly the course of seizures. This study investigated the electroclinical and prognostic features of 16 patients with PNH. Methods: Of 120 patients with epilepsy and malformations of cortical development, 16 had PNH. Of these, eight patients had periventricular nodules only (simple PNH) and eight also presented with other cortical or cerebral malformations (subcortical heterotopia; polymicrogyria; focal dysplasia; schizencephaly; cortical infolding; agenesis of the corpus callosum; mega cisterna magna and cerebellar atrophy) (PNH plus). All patients underwent clinical, neurophysiological, and MRI investigation. The mean follow up was 17.3 years (2–40 years). Results: Two electroclinical patterns emerged: (1) The first pattern, associated with simple PNH, was characterised by normal intelligence and seizures, usually partial, which began during the second decade of life. The seizures never became frequent and tended to disappear or become very rare. The EEG showed focal abnormalities. (2) The second pattern, associated with PNH plus, was characterised by mental retardation and seizures that began during the first decade of life. The seizures were very frequent in most cases and sudden drops were observed in six patients. Seizures were medically refractory in four patients. The EEG showed focal and bisynchronous abnormalities. Conclusions: Two groups of PNH patients with different electroclinical and neuroradiological features can be identified after a long term follow up. The presence of other types of cortical or cerebral malformations, in addition to periventricular nodules, determines a poor prognosis. PMID:15146004

  12. Perilesional and contralateral white matter evolution and integrity in patients with periventricular nodular heterotopia and epilepsy: a longitudinal diffusion tensor imaging study.

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    Liu, W; Yan, B; An, D; Niu, R; Tang, Y; Tong, X; Gong, Q; Zhou, D

    2017-12-01

    This study aimed to assess the evolution of perinodular and contralateral white matter abnormalities in patients with periventricular nodular heterotopia (PNH) and epilepsy. Diffusion tensor imaging (DTI) (64 directions) and 3 T structural magnetic resonance imaging were performed in 29 PNH patients (mean age 27.3 years), and 16 patients underwent a second scan (average time between the two scans 1.1 years). Fractional anisotropy and mean diffusivity were measured within the perilesional and contralateral white matter. Longitudinal analysis showed that white matter located 10 mm from the focal nodule displayed characteristics intermediate to tissue 5 mm away, and normal-appearing white matter (NAWM) also established evolution profiles of perinodular white matter in different cortical lobes. Compared to 29 age- and sex-matched healthy controls, significant decreased fractional anisotropy and elevated mean diffusivity values were observed in regions 5 and 10 mm from nodules (P < 0.01), whilst DTI metrics of the remaining NAWM did not differ significantly from controls. Additionally, normal DTI metrics were shown in the contralateral region in patients with unilateral PNH. Periventricular nodular heterotopia is associated with microstructural abnormalities within the perilesional white matter and the extent decreases with increasing distance from the nodule. In the homologous contralateral region, white matter diffusion metrics were unchanged in unilateral PNH. These findings have clinical implications with respect to the medical and surgical interventions of PNH-related epilepsy. © 2017 EAN.

  13. Nodular Neuronal Heterotopia

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    J Gordon Millichap

    1999-01-01

    Brain tissue from 4 children with intractable epilepsy and subcortical or periventricular nodular heterotopia of different etiologies (megalencephaly, cortical dysplasia, polymicrogyria) was examined at the University of Oxford, Radcliffe Infirmary, UK.

  14. Like father, like son: periventricular nodular heterotopia and nonverbal learning disorder.

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    McCann, Marcia V; Pongonis, Stephen J; Golomb, Meredith R; Edwards-Brown, Mary; Christensen, Celanie K; Sokol, Deborah K

    2008-08-01

    Periventricular nodular heterotopia is a common malformation of cortical development in which the migration of developing neurons destined for the cerebral cortex is abbreviated. Bilateral periventricular nodular heterotopia is most commonly an X-linked disorder that involves mutations in the filamin A (FLNA) gene, but an autosomal recessive form and sporadic forms have been identified. To our knowledge, autosomal dominant transmission of isolated periventricular nodular heterotopia has not been reported. Periventricular nodular heterotopia has a heterogeneous phenotype, associated commonly with seizure disorder, and more recently with reading deficits and visual-spatial deficits in some patients. We present a father and son with bilateral periventricular nodular heterotopia and similar visual-spatial learning deficits, consistent with nonverbal learning disability.

  15. Periventricular nodular heterotopia in patients with filamin-1 gene mutations: neuroimaging findings

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    Poussaint, T.Y. [Dept. of Radiology, Children' s Hospital, Boston, MA (United States); Fox, J.W.; Walsh, C.A. [Program in Biological and Biomedical Sciences, Harvard Medical School, Boston, MA (United States); Dept. of Neurology, Beth Israel Deaconess Medical Center, Harvard Institutes of Medicine, Boston, MA (United States); Dobyns, W.B. [Department of Human Genetics, The University of Chicago, Chicago, IL (United States); Radtke, R. [Division of Neurology, Duke University Medical Center, Durham, NC (United States); Scheffer, I.E.; Berkovic, S.F. [Department of Neurology, University of Melbourne, Austin and Repatriation Medical Centre, Heidelberg (Australia); Barnes, P.D. [Department of Radiology, Children' s Hospital and Harvard Medical School, Boston, MA (United States); Huttenlocher, P.R. [Department of Pediatrics, University of Chicago, Chicago, Illinois (United States)

    2000-11-01

    Background. The filamin-1 (FLN-1) gene is responsible for periventricular nodular heterotopia (PNH), which is an X-linked dominant neuronal migration disorder. Objective. To review the clinical and imaging findings in a series of patients with documented filamin-1 mutations. Materials and methods. A retrospective review of the medical records and MR studies of a series of patients with PNH and confirmed FLN-1 mutations was done. There were 16 female patients (age range:.67-71 years; mean = 28.6) with filamin-1 gene mutations. Results. In six of the patients the same mutation was inherited in four generations in one pedigree. In a second pedigree, a distinct mutation was found in two patients in two generations. In a third pedigree, a third mutation was found in four patients in two generations. The remaining four patients had sporadic de novo mutations that were not present in the parents. Ten patients had seizures, and all patients had normal intelligence. In all 16 patients MR demonstrated bilateral near-continuous PNH. There were no consistent radiographic or clinical differences between patients carrying different mutations. Conclusion. Patients with confirmed FLN-1 gene mutations are usually female and have a distinctive MR pattern of PNH. Other female patients with this same MR pattern probably harbor FLN-1 mutations and risk transmission to their progeny. This information is important for genetic counseling. (orig.)

  16. Periventricular nodular heterotopia in patients with filamin-1 gene mutations: neuroimaging findings

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    Poussaint, T.Y.; Fox, J.W.; Walsh, C.A.; Dobyns, W.B.; Radtke, R.; Scheffer, I.E.; Berkovic, S.F.; Barnes, P.D.; Huttenlocher, P.R.

    2000-01-01

    Background. The filamin-1 (FLN-1) gene is responsible for periventricular nodular heterotopia (PNH), which is an X-linked dominant neuronal migration disorder. Objective. To review the clinical and imaging findings in a series of patients with documented filamin-1 mutations. Materials and methods. A retrospective review of the medical records and MR studies of a series of patients with PNH and confirmed FLN-1 mutations was done. There were 16 female patients (age range:.67-71 years; mean = 28.6) with filamin-1 gene mutations. Results. In six of the patients the same mutation was inherited in four generations in one pedigree. In a second pedigree, a distinct mutation was found in two patients in two generations. In a third pedigree, a third mutation was found in four patients in two generations. The remaining four patients had sporadic de novo mutations that were not present in the parents. Ten patients had seizures, and all patients had normal intelligence. In all 16 patients MR demonstrated bilateral near-continuous PNH. There were no consistent radiographic or clinical differences between patients carrying different mutations. Conclusion. Patients with confirmed FLN-1 gene mutations are usually female and have a distinctive MR pattern of PNH. Other female patients with this same MR pattern probably harbor FLN-1 mutations and risk transmission to their progeny. This information is important for genetic counseling. (orig.)

  17. Mutations in the HECT domain of NEDD4L lead to AKT-mTOR pathway deregulation and cause periventricular nodular heterotopia.

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    Broix, Loïc; Jagline, Hélène; Ivanova, Ekaterina; Schmucker, Stéphane; Drouot, Nathalie; Clayton-Smith, Jill; Pagnamenta, Alistair T; Metcalfe, Kay A; Isidor, Bertrand; Louvier, Ulrike Walther; Poduri, Annapurna; Taylor, Jenny C; Tilly, Peggy; Poirier, Karine; Saillour, Yoann; Lebrun, Nicolas; Stemmelen, Tristan; Rudolf, Gabrielle; Muraca, Giuseppe; Saintpierre, Benjamin; Elmorjani, Adrienne; Moïse, Martin; Weirauch, Nathalie Bednarek; Guerrini, Renzo; Boland, Anne; Olaso, Robert; Masson, Cecile; Tripathy, Ratna; Keays, David; Beldjord, Cherif; Nguyen, Laurent; Godin, Juliette; Kini, Usha; Nischké, Patrick; Deleuze, Jean-François; Bahi-Buisson, Nadia; Sumara, Izabela; Hinckelmann, Maria-Victoria; Chelly, Jamel

    2016-11-01

    Neurodevelopmental disorders with periventricular nodular heterotopia (PNH) are etiologically heterogeneous, and their genetic causes remain in many cases unknown. Here we show that missense mutations in NEDD4L mapping to the HECT domain of the encoded E3 ubiquitin ligase lead to PNH associated with toe syndactyly, cleft palate and neurodevelopmental delay. Cellular and expression data showed sensitivity of PNH-associated mutants to proteasome degradation. Moreover, an in utero electroporation approach showed that PNH-related mutants and excess wild-type NEDD4L affect neurogenesis, neuronal positioning and terminal translocation. Further investigations, including rapamycin-based experiments, found differential deregulation of pathways involved. Excess wild-type NEDD4L leads to disruption of Dab1 and mTORC1 pathways, while PNH-related mutations are associated with deregulation of mTORC1 and AKT activities. Altogether, these data provide insights into the critical role of NEDD4L in the regulation of mTOR pathways and their contributions in cortical development.

  18. Bilateral Periventricular Nodular Heterotopia

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    J Gordon Millichap

    1998-01-01

    Two unrelated boys, ages 8 and 5 years, with a syndrome of bilateral periventricular nodular heterotopia (BPNH), regional cortical dysplasia, mild mental retardation, and frontonasal malformation (FNM) are reported from the Instituto di Neuropsychiatria Infantile, University of Pisa, Italy, and the Institute of Human Genetics, University of Minnesota Medical School, Minneapolis, MN.

  19. Periventricular Nodular Heterotopia, Surgical Goal in Drug-Resistant Epilepsy

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    Fernando Velandia-Hurtado

    2014-09-01

    Full Text Available Introduction: Neuronal heterotopia is a migration disorder in which these cells do not complete their movement toward the cerebral cortex. Periventricular nodular heterotopia is the most frequently reported form, characterized by neuronal conglomerates adjacent to the lateral ventricles walls. About 90 % of patients with this condition suffer epilepsy at some point in their lives and the major proportion of them will be resistant to pharmacologic treatment. This makes an appropriate diagnostic approach necessary in order to determine which patients would benefit from surgical resection of the lesion, which in most cases offers a high rate of crisis control. Development: This article presents a review of the most important topics approached from the practice of periventricular nodular heterotopia pathophysiology, clinical features, diagnosis and therapy. It is aimed at exploring the role of this condition as a cause of intractable epilepsy. Conclusion: Pharmacologic treatment for resistant epilepsy will have a severe impact on patient’s quality of life. Periventricular nodular heterotopia is frequently associated to this condition, which must be successfully approached by the medical team attempting to an opportune diagnosis and defining which patients would benefit from surgical management. This positively impacts the quality of life of these patients and their caregivers.

  20. Movement disorder and epilepsy in subependymal nodular heterotopia

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    Anurag Lohmror

    2017-01-01

    Full Text Available Subependymal nodular heterotopia is a cortical development malformation that is commonly associated with refractory epilepsy. Patients with heterotopia show a wide spectrum of clinical manifestations, from being asymptomatic to presenting with intractable seizures and intellectual impairment. We report a case of drug-resistant epilepsy with normal intelligence, having bilateral subependymal heterotopic nodules in the brain, presenting to us with a movement disorder in the form of myoclonus of bilateral lower limbs which is an unusual manifestation of gray matter heterotopias. Although rare, gray matter heterotopias may present as movement disorder and should be considered in differential diagnosis while workup of movement disorders.

  1. A Case with Bilateral Periventricular Nodular Heterotopia Diagnosed as Depression

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    Melek Kandemir

    2010-06-01

    Full Text Available Periventricular nodular heterotopia is a form of neuronal migration abnormality. Periventricular nodular heterotopia can easily be recognized by cranial magnetic resonance imaging. The most common clinical appearance is epileptic seizures. In some cases, symptoms are accompanied with psychiatric complaints. In this article, we report a 33-year-old female with complaints of left-sided paresthesia induced by emotional stress. She had been followed at an outpatient psychiatry clinic for about 10 years with the diagnosis of somatization disorder. Her electroencephalography recordings -awake as well as during sleep- were found to be normal. The cranial magnetic resonance imaging showed bilateral periventricular nodular heterotopia. Her seizures were controlled with carbamazepine treatment. Partial epileptic seizures might also be observed, even though the cerebral heterotopic lesions are bilateral. When a history is obtained from a patient with somatoform complaints, it should be kept in mind that these symptoms might be seizures, and the patient should be questioned accordingly

  2. A Case with Bilateral Periventricular Nodular Heterotopia Diagnosed as Depression

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    Melek Kandemir

    2010-06-01

    Full Text Available Periventricular nodular heterotopia is a form of neuronal migration abnormality. Periventricular nodular heterotopia can easily be recognized by cranial magnetic resonance imaging. The most common clinical appearance is epileptic seizures. In some cases, symptoms are accompanied with psychiatric complaints. In this article, we report a 33-year-old female with complaints of left-sided paresthesia induced by emotional stress. She had been followed at an outpatient psychiatry clinic for about 10 years with the diagnosis of somatization disorder. Her electroencephalography recordings -awake as well as during sleep- were found to be normal. The cranial magnetic resonance imaging showed bilateral periventricular nodular heterotopia. Her seizures were controlled with carbamazepine treatment. Partial epileptic seizures might also be observed, even though the cerebral heterotopic lesions are bilateral. When a history is obtained from a patient with somatoform complaints, it should be kept in mind that these symptoms might be seizures, and the patient should be questioned accordingly.

  3. Integrity of the corpus callosum in patients with periventricular nodular heterotopia related epilepsy by FLNA mutation.

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    Liu, Wenyu; An, Dongmei; Niu, Running; Gong, Qiyong; Zhou, Dong

    2018-01-01

    To investigate the quantitative diffusion properties of the corpus callosum (CC) in a large group of patients with periventricular nodular heterotopia (PNH) related epilepsy and to further investigate the effect of Filamin A ( FLNA ) mutation on these properties. Patients with PNH (n = 34), subdivided into FLNA -mutated (n = 11) and FLNA -nonmutated patients (n = 23) and healthy controls (n = 34), underwent 3.0 T structural MRI and diffusion imaging scan (64 direction). Fractional anisotropy (FA) and mean diffusivity (MD) were measured in the three major subdivisions of the CC (genu, body and splenium). Correlations between DTI metric changes and clinical parameters were also evaluated. Furthermore, the effect of FLNA mutation on structural integrity of the corpus callosum was examined. Patients with PNH and epilepsy had significant reductions in FA for the genu and splenium of the CC, accompanied by increases in MD for the splenium, as compared to healthy controls. There were no correlations between clinical parameters of epilepsy and MD. The FA value in the splenium negatively correlated with epilepsy duration. Interestingly, FLNA -mutated patients showed significantly decreased FA for all three major subdivisions of the CC, and increased MD for the genu and splenium, as compared to HCs and FLNA -nonmutated patients. These findings support the conclusion that patients with epilepsy secondary to PNH present widespread microstructural changes found in the corpus callosum that extend beyond the macroscopic MRI-visible lesions. This study also indicates that FLNA may affect white matter integrity in this disorder.

  4. Stereo-EEG: Diagnostic and therapeutic tool for periventricular nodular heterotopia epilepsies.

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    Mirandola, Laura; Mai, Roberto F; Francione, Stefano; Pelliccia, Veronica; Gozzo, Francesca; Sartori, Ivana; Nobili, Lino; Cardinale, Francesco; Cossu, Massimo; Meletti, Stefano; Tassi, Laura

    2017-11-01

    Periventricular nodular heterotopias (PNHs) are malformations of cortical development related to neuronal migration disorders, frequently associated with drug-resistant epilepsy (DRE). Stereo-electroencephalography (SEEG) is considered a very effective step of the presurgical evaluation, providing the recognition of the epileptogenic zone (EZ). At the same time, via the intracerebral electrodes it is possible to perform radiofrequency thermocoagulation (SEEG-guided RF-TC) with the aim of ablating and/or disrupting the EZ. The purpose of this study was to evaluate both the relationships between PNH and the EZ, and the efficacy of SEEG-guided RF-TC. Twenty patients with DRE related to PNHs were studied. Inclusion criteria were the following: (1) patients with epilepsy and PNHs (unilateral or bilateral, single or multiple nodules) diagnosed on brain magnetic resonance imaging (MRI); (2) SEEG recordings available as part of the presurgical investigations, with at least one intracerebral electrode inside the heterotopia; (3) complete surgical workup with SEEG-guided RF-TC and/or with traditional neurosurgery, with a follow-up of at least 12 months. Complex and heterogenic epileptic networks were found in these patients. SEEG-guided RF-TC both into the nodules and/or the cortex was efficacious in the 76% of patients. Single or multiple, unilateral or bilateral PNHs are the most suitable for this procedure, whereas patients with PNHs associated with complex cortical malformations obtained excellent outcome only with traditional resective surgery. Each patient had a specific epileptogenic network, independent from the number, size, or location of nodules and from the cortical malformation associated with. SEEG-guided RF-TC appears as a new and very effective diagnostic and therapeutic approach for DRE related to PNHs. Wiley Periodicals, Inc. © 2017 International League Against Epilepsy.

  5. Periventricular nodular and subcortical neuronal heterotopia in adult epileptic patients Heterotopía neuronal nodular y subcortical en pacientes adultos con epilepsia

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    Damián E. Consalvo

    2006-04-01

    Full Text Available Developmental malformations are brain abnormalities that occur during embryogenesis. Neuronal migration disorders, including heterotopic lesions, constitute one type of such abnormalities. The aim of the study was to compare the epileptic clinical patterns of patients with periventricular nodular heterotopia (PNH (G1 with those affected by subcortical heterotopia (SCH (G2 looking for differences between both groups which, eventually, might suggest the type of the underlying malformation. The variables studied in both groups were: type of the heterotopia depicted on MRI studies, sex, age, age at seizure onset, annual seizure frequency, localization of the ictal symptomatogenic zone, characteristics of the EEG, other associated anomalies on the magnetic resonance images (MRI besides the heterotopia, and response to treatment. The only difference found between both groups was the type of heterotopia as shown by MRI studies. The other assessed variables did not significantly (p>0.05 differ between groups. No differences in the clinical features characterizing epilepsy could be found in patients with PNH or SCH, being the images the only tool able to differentiate them.Las malformaciones de la corteza cerebral son un grupo de entidades que se producen durante las etapas del desarrollo embrionario y cuya manifestación clínica puede ser la epilepsia. Estas malformaciones pueden ser diagnosticadas in vivo a través de las imágenes por resonancia magnética (IRM. Un subtipo particular de éstas lo constituyen los trastornos en la migración neuronal, dentro de los cuales se ubican las heterotopías (HT. El objetivo del estudio fue comparar enfermos portadores de HT periventriculares (G1 con aquellos portadores de HT subcorticales (G2. Se analizaron las variables sexo, edad y edad de inicio de la epilepsia (EI en años, antecedentes familiares (AF o prenatales (AP, frecuencia anual de crisis (FAC y características semiológicas de las crisis

  6. Bilateral Periventricular Nodular Heterotopia and Lissencephaly in an Infant with Unbalanced t(12;17)(q24.31; p13.3) Translocation

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    Grosso, Salvatore; Fichera, Marco; Galesi, Ornella; Luciano, Daniela; Pucci, Lucia; Giardini, Francesca; Berardi, Rosario; Balestri, Paolo

    2008-01-01

    Periventricular nodular heterotopia and Miller-Dieker syndrome are two different disorders of brain development. Miller-Dieker syndrome exhibits classical lissencephaly and is related to defects in the lissencephaly gene ("LIS1"). Periventricular nodular heterotopia is characterized by aggregates of grey matter adjacent to the lateral ventricle…

  7. Disrupted intrinsic and remote functional connectivity in heterotopia-related epilepsy.

    Science.gov (United States)

    Liu, W; Hu, X; An, D; Gong, Q; Zhou, D

    2018-01-01

    Several neuroimaging studies have examined neural interactions in patients with periventricular nodular heterotopia (PNH). However, features of the underlying functional network remain poorly understood. In this study, we examined alterations in the local (regional) and remote (interregional) cerebral networks in this disorder. Twenty-eight subjects all having suffered from PNH with epilepsy, as well as 28 age- and sex- matched healthy controls, were enrolled in this study. Amplitude of low-frequency fluctuation (ALFF) and seed-based functional connectivity (FC) were calculated to detect regional neural function and functional network integration, respectively. Compared with healthy controls, patients with PNH-related epilepsy showed decreased ALFF in the ventromedial prefrontal cortex (vmPFC) and precuneus areas. ALFF values in both areas were negative correlated with epilepsy duration (P < .05, Bonferroni-corrected). Furthermore, patients with PNH-related epilepsy had increased remote interregional FC mainly in bilateral prefrontal and parietal cortices, supramarginal gyrus, dorsal cingulate gyrus, and right insula; lower FC was found in posterior brain regions including bilateral parahippocampal gyrus and inferior temporal gyrus. Focal spontaneous hypofunction, as assessed by ALFF, correlates with epilepsy duration in patients with PNH-related epilepsy. Abnormalities existed both within the default-mode network and then across the whole brain, demonstrating that intrinsic brain dysfunction may be related to specific network interactions. Our findings provide novel understanding of the connectivity-based pathophysiological mechanisms of PNH. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  8. Familial bilateral periventricular nodular heterotopia mimics tuberous sclerosis.

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    Jardine, P E; Clarke, M A; Super, M

    1996-01-01

    A mother and daughter with an initial diagnosis of tuberous sclerosis are described. The daughter presented with partial seizures at the age of 8 months. Computed tomography showed uncalcified periventricular nodules which on magnetic resonance imaging were ovoid, almost contiguous, of grey matter density, and did not enhance with gadolinium. Brain imaging of her asymptomatic mother was similar. Absence of severe mental retardation, extracranial hamartomas, and depigmented patches distinguishes familial bilateral periventricular nodular heterotopia (FNH) from tuberous sclerosis. FNH is probably inherited as an X linked dominant with lethality in males. Images Figure 1 Figure 2 PMID:8787433

  9. Epileptogenic networks in nodular heterotopia: A stereoelectroencephalography study.

    Science.gov (United States)

    Pizzo, Francesca; Roehri, Nicolas; Catenoix, Hélène; Medina, Samuel; McGonigal, Aileen; Giusiano, Bernard; Carron, Romain; Scavarda, Didier; Ostrowsky, Karine; Lepine, Anne; Boulogne, Sébastien; Scholly, Julia; Hirsch, Edouard; Rheims, Sylvain; Bénar, Christian-George; Bartolomei, Fabrice

    2017-12-01

    Defining the roles of heterotopic and normotopic cortex in the epileptogenic networks in patients with nodular heterotopia is challenging. To elucidate this issue, we compared heterotopic and normotopic cortex using quantitative signal analysis on stereoelectroencephalography (SEEG) recordings. Clinically relevant biomarkers of epileptogenicity during ictal (epileptogenicity index; EI) and interictal recordings (high-frequency oscillation and spike) were evaluated in 19 patients undergoing SEEG. These biomarkers were then compared between heterotopic cortex and neocortical regions. Seizures were classified as normotopic, heterotopic, or normoheterotopic according to respective values of quantitative analysis (EI ≥0.3). A total of 1,246 contacts were analyzed: 259 in heterotopic tissue (heterotopic cortex), 873 in neocortex in the same lobe of the lesion (local neocortex), and 114 in neocortex distant from the lesion (distant neocortex). No significant difference in EI values, high-frequency oscillations, and spike rate was found comparing local neocortex and heterotopic cortex at a patient level, but local neocortex appears more epileptogenic (p < 0.001) than heterotopic cortex analyzing EI values at a seizure level. According to EI values, seizures were mostly normotopic (48.5%) or normoheterotopic (45.5%); only 6% were purely heterotopic. A good long-term treatment response was obtained in only two patients after thermocoagulation and surgical disconnection. This is the first quantitative SEEG study providing insight into the mechanisms generating seizures in nodular heterotopia. We demonstrate that both the heterotopic lesion and particularly the normotopic cortex are involved in the epileptogenic network. This could open new perspectives on multitarget treatments, other than resective surgery, aimed at modifying the epileptic network. Wiley Periodicals, Inc. © 2017 International League Against Epilepsy.

  10. Phenotypic and imaging features of FLNA-negative patients with bilateral periventricular nodular heterotopia and epilepsy.

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    Fallil, Zianka; Pardoe, Heath; Bachman, Robert; Cunningham, Benjamin; Parulkar, Isha; Shain, Catherine; Poduri, Annapurna; Knowlton, Robert; Kuzniecky, Ruben

    2015-10-01

    Periventricular nodular heterotopia (PVNH) is a malformation of cortical development due to impaired neuronal migration resulting in the formation of nodular masses of neurons and glial cells in close proximity to the ventricular walls. We report the clinical characteristics of the largest case series of FLNA-negative patients with seizures and bilateral periventricular heterotopia. Participants were recruited through the Epilepsy Phenome/Genome Project (EPGP), a multicenter collaborative effort to collect detailed phenotypic data and DNA on a large number of individuals with epilepsy, including a cohort with symptomatic epilepsy related to PVNH. Included subjects had epilepsy, and MRI confirmed bilateral PVNH. Magnetic resonance imaging studies were visually and quantitatively reviewed to investigate the topographic extent of PVNH, symmetry, and laterality. We analyzed data on 71 patients with bilateral PVNH. The incidence of febrile seizures was 16.6%. There was at least one other family member with epilepsy in 36.9% of this population. Developmental delay was present in 21.8%. Focal onset seizures were the most common type of seizure presentation (79.3%). High heterotopia burden was strongly associated with female gender and trigonal nodular localization. There was no evidence for differences in brain volume between PVNH subjects and controls. No relationship was observed between heterotopic volume and gender, developmental delay, location of PVNH, ventricular or cerebellar abnormalities, laterality of seizure onset, age at seizure onset, and duration of epilepsy. A direct correlation was observed between high heterotopia burden, female gender, and trigonal location in this large cohort of FLNA-negative bilateral PVNH patients with epilepsy. Quantitative MRI measurements indicated that this correlation is based on the diffuse nature of the heterotopic nodules rather than on the total volume of abnormal heterotopic tissue. Copyright © 2015 Elsevier Inc. All

  11. De Morsier syndrome associated with periventricular nodular heterotopia: case reporte Síndrome de De Morsier associada a heterotopia nodular periventricular: relato de caso

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    Mônica Jaques Spinosa

    2007-09-01

    Full Text Available INTRODUCTION: Septo-optic dysplasia (De Morsier syndrome is defined as the association between optic nerve hypoplasia, midline central nervous system malformations and pituitary dysfunction. CASE REPORT: Third child born to nonconsanguineous parents, female, adequate pre-natal medical care, cesarean term delivery due to breech presentation, Apgar score 3 at the first minute and 8 at 5 minutes, symptomatic hypoglycemia at 18 hours. Neurological follow-up identified a delay in acquisition of motor and language developmental milestones. Epileptic generalized seizures began at 12 months and were controlled with phenobarbital. EEG was normal. MRI revealed agenesis of the pituitary stalk, hypoplasia of the optic chiasm and periventricular nodular heterotopia. Ophthalmologic evaluation showed bilateral optic disk hypoplasia. Endocrine function laboratory tests revealed primary hypothyroidism and hyperprolactinemia. CONCLUSION: The relevance of this case report relies on its uniqueness, since periventricular heterotopia had not been described in association with septo-optic dysplasia until 2006.INTRODUÇÃO: Displasia septo-óptica (síndrome de De Morsier é definida como a associação entre hipoplasia do nervo óptico, malformações de linha média do sistema nervoso central e disfunção pituitária. RELATO DE CASO: Terceiro filho, pais não consangüíneos, sexo feminino, pré-natal adequado, parto cesário a termo por apresentação pélvica, Apgar 3 no primeiro minuto e 8 no quinto minuto, hipoglicemia sintomática com 18 horas de vida. Durante o acompanhamento neurológico identificou-se atraso na aquisição dos marcos de desenvolvimento motor e linguagem. Crises epilépticas generalizadas iniciaram com 12 meses de vida sendo controladas com fenobarbital. EEG era normal. Ressonância magnética revelou agenesia de haste pituitária, hipoplasia de quiasma óptico e heterotopia nodular periventricular. Avaliação oftalmológica demonstrou

  12. A novel Xp22.11 deletion causing a syndrome of craniosynostosis and periventricular nodular heterotopia

    NARCIS (Netherlands)

    Kogelenberg, M. van; Lerone, M.; Toni, T. De; Divizia, M.T.; Brouwer, A.P. de; Veltman, J.A.; Bokhoven, J.H.L.M. van; Robertson, S.P.

    2011-01-01

    We report on a follow-up evaluation of a male with a phenotype including craniosynostosis, periventricular nodular heterotopia, and neurodevelopmental delay. He was initially assigned a clinical diagnosis of Fontaine-Farriaux syndrome (FFS) as an infant although now, with improved delineation of

  13. Intracranial evaluation and laser ablation for epilepsy with periventricular nodular heterotopia.

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    Thompson, Stephen A; Kalamangalam, Giridhar P; Tandon, Nitin

    2016-10-01

    Surgical treatment of focal epilepsy in the presence of periventricular nodular heterotopia (PVNH) poses a challenge, as the relative roles of the nodular tissue and the overlying cortex in the generation of seizures can be complex and variable. Here, we review the literature on chronic invasive EEG recordings in humans with this substrate and present two illustrative cases from our practice. We found that while inter-ictal spiking from nodules is common, clinical seizures rarely arise solely from nodular tissue. More typically, ictal onset is simultaneous with overlying neocortex or mesial temporal structures. Surgical outcome is more favorable in cases with unilateral (as opposed to bilateral) PVNH, and when a substantial or complete ablation of PVNH is performed. In rare cases, nodular ablation alone may be sufficient, as may be completed by MRI-guided laser interstitial thermal therapy. The mechanism(s) by which PNVH interacts with overlying cortex are not fully understood, but we suggest that PVNH either orchestrates or amplifies local network epileptogenicity. At present, invasive recordings with penetrating depth electrodes are required prior to surgical therapy, as illustrated in our cases. Published by Elsevier Ltd.

  14. Periventricular Nodular Heterotopia: Detection of Abnormal Microanatomic Fiber Structures with Whole-Brain Diffusion MR Imaging Tractography.

    Science.gov (United States)

    Farquharson, Shawna; Tournier, J-Donald; Calamante, Fernando; Mandelstam, Simone; Burgess, Rosemary; Schneider, Michal E; Berkovic, Samuel F; Scheffer, Ingrid E; Jackson, Graeme D; Connelly, Alan

    2016-12-01

    Purpose To investigate whether it is possible in patients with periventricular nodular heterotopia (PVNH) to detect abnormal fiber projections that have only previously been reported in the histopathology literature. Materials and Methods Whole-brain diffusion-weighted (DW) imaging data from 14 patients with bilateral PVNH and 14 age- and sex-matched healthy control subjects were prospectively acquired by using 3.0-T magnetic resonance (MR) imaging between August 1, 2008, and December 5, 2012. All participants provided written informed consent. The DW imaging data were processed to generate whole-brain constrained spherical deconvolution (CSD)-based tractography data and super-resolution track-density imaging (TDI) maps. The tractography data were overlaid on coregistered three-dimensional T1-weighted images to visually assess regions of heterotopia. A panel of MR imaging researchers independently assessed each case and indicated numerically (no = 1, yes = 2) as to the presence of abnormal fiber tracks in nodular tissue. The Fleiss κ statistical measure was applied to assess the reader agreement. Results Abnormal fiber tracks emanating from one or more regions of heterotopia were reported by all four readers in all 14 patients with PVNH (Fleiss κ = 1). These abnormal structures were not visible on the tractography data from any of the control subjects and were not discernable on the conventional T1-weighted images of the patients with PVNH. Conclusion Whole-brain CSD-based fiber tractography and super-resolution TDI mapping reveals abnormal fiber projections in nodular tissue suggestive of abnormal organization of white matter (with abnormal fibers both within nodules and projecting to the surrounding white matter) in patients with bilateral PVNH. © RSNA, 2016.

  15. A novel missense mutation in the HECT domain of NEDD4L identified in a girl with periventricular nodular heterotopia, polymicrogyria and cleft palate.

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    Kato, Koji; Miya, Fuyuki; Hori, Ikumi; Ieda, Daisuke; Ohashi, Kei; Negishi, Yutaka; Hattori, Ayako; Okamoto, Nobuhiko; Kato, Mitsuhiro; Tsunoda, Tatsuhiko; Yamasaki, Mami; Kanemura, Yonehiro; Kosaki, Kenjiro; Saitoh, Shinji

    2017-09-01

    We identified a novel de novo heterozygous missense mutation in the NEDD4L gene (NM_015277: c.2617G>A; p.Glu873Lys) through whole-exome sequencing in a 3-year-old girl showing severe global developmental delay, infantile spasms, cleft palate, periventricular nodular heterotopia and polymicrogyria. Mutations in the HECT domain of NEDD4L have been reported in patients with a neurodevelopmental disorder along with similar brain malformations. All patients reported with NEDD4L HECT domain mutations showed periventricular nodular heterotopia, and most had seizures, cortex anomalies, cleft palate and syndactyly. The unique constellation of clinical features in patients with NEDD4L mutations might help clinically distinguish them from patients with other genetic mutations including FLNA, which is a well-known causative gene of periventricular nodular heterotopia. Although mutations in the HECT domain of NEDD4L that lead to AKT-mTOR pathway deregulation in forced expression system were reported, our western blot analysis did not show an increased level of AKT-mTOR activity in lymphoblastoid cell lines (LCLs) derived from the patient. In contrast to the forced overexpression system, AKT-mTOR pathway deregulation in LCLs derived from our patient seems to be subtle.

  16. Infantile spasms with periventricular nodular heterotopia, unbalanced chromosomal translocation 3p26.2 -10p15.1 and 6q22.31 duplication.

    Science.gov (United States)

    Jones, Kevin; Weiss, Shelly K; Minassian, Berge

    2016-07-01

    Patients presenting with infantile spasms, dysmorphic features, and periventricular nodular heterotopia may benefit from genetic copy number variation microarray, or whole-exome sequencing to identify candidate genes. This will allow personalized diagnosis and prognostication and the eventual understanding of single and combined gene functions in brain health and disease.

  17. Intracranial investigation of a patient with nodular heterotopia and hippocampal sclerosis: dealing with a dual pathology.

    Science.gov (United States)

    Ladino, Lady Diana; Dash, Chelsea; Wu, Adam; Tellez-Zenteno, Jose Francisco

    2017-06-01

    The pre-operative assessment and surgical management of patients with dual pathology is challenging. We describe a patient with drug-resistant focal epilepsy with hippocampal sclerosis and extensive periventricular nodular heterotopia in the same hemisphere. The semiology, scalp EEG, and imaging were divergent, but the presence of focal interictal and ictal epileptic discharges of the putative ictal onset zone resulted in successful localization of the epileptogenic zone. A less aggressive resection was performed based on intracranial EEG recording. The patient has been seizure-free for three years since resection. Electroclinical hypotheses and challenges in defining the epileptogenic network are discussed.

  18. Identification of a duplication of Xq28 associated with bilateral periventricular nodular heterotopia.

    Science.gov (United States)

    Fink, J M; Dobyns, W B; Guerrini, R; Hirsch, B A

    1997-01-01

    Bilateral periventricular nodular heterotopia (BPNH) is a malformation of neuronal migration and is characterized by nodules of heterotopic gray matter lining the lateral ventricles of the brain. The majority of BPNH patients are female and have epilepsy as a sole clinical manifestation of their disease. Familial BPNH has been mapped to Xq28 by linkage analysis. A multiple congenital anomaly-mental retardation syndrome (BPNH/MR) was recently delineated in three unrelated boys with BPNH, cerebellar hypoplasia, severe mental retardation, epilepsy, and syndactyly. High-resolution chromosome analysis revealed a subtle abnormality of Xq28 in one of the boys with BPNH/MR syndrome. FISH with cosmids and YACs from Xq28 further characterized this abnormality as a 2.25-3.25-Mb inverted duplication. No abnormality of Xq28 was detected by G-banding or FISH in the other two boys. These data support the linkage assignment of BPNH to band Xq28 and narrow the critical region to the distal 2.25-3.25 Mb of Xq28. Images Figure 1 Figure 3 Figure 4 Figure 5 PMID:9311743

  19. De novo and inherited private variants in MAP1B in periventricular nodular heterotopia.

    Science.gov (United States)

    Heinzen, Erin L; O'Neill, Adam C; Zhu, Xiaolin; Allen, Andrew S; Bahlo, Melanie; Chelly, Jamel; Dobyns, William B; Freytag, Saskia; Guerrini, Renzo; Leventer, Richard J; Poduri, Annapurna; Robertson, Stephen P; Walsh, Christopher A; Zhang, Mengqi

    2018-05-08

    Periventricular nodular heterotopia (PVNH) is a malformation of cortical development commonly associated with epilepsy. We exome sequenced 202 individuals with sporadic PVNH to identify novel genetic risk loci. We first performed a trio-based analysis and identified 219 de novo variants. Although no novel genes were implicated in this initial analysis, PVNH cases were found overall to have a significant excess of nonsynonymous de novo variants in intolerant genes (p = 3.27x10-7), suggesting a role for rare new alleles in genes yet to be associated with the condition. Using a gene-level collapsing analysis comparing cases and controls, we identified a genome-wide significant signal driven by four ultra-rare loss-of-function heterozygous variants in MAP1B, including one de novo variant. In at least one instance, the MAP1B variant was inherited from a parent with previously undiagnosed PVNH. The PVNH was frontally predominant and associated with perisylvian polymicrogyria. These results implicate MAP1B in PVNH. More broadly, our findings suggest that detrimental mutations likely arising in immediately preceding generations with incomplete penetrance may also be responsible for some apparently sporadic diseases.

  20. 47 patients with FLNA associated periventricular nodular heterotopia.

    Science.gov (United States)

    Lange, Max; Kasper, Burkhard; Bohring, Axel; Rutsch, Frank; Kluger, Gerhard; Hoffjan, Sabine; Spranger, Stephanie; Behnecke, Anne; Ferbert, Andreas; Hahn, Andreas; Oehl-Jaschkowitz, Barbara; Graul-Neumann, Luitgard; Diepold, Katharina; Schreyer, Isolde; Bernhard, Matthias K; Mueller, Franziska; Siebers-Renelt, Ulrike; Beleza-Meireles, Ana; Uyanik, Goekhan; Janssens, Sandra; Boltshauser, Eugen; Winkler, Juergen; Schuierer, Gerhard; Hehr, Ute

    2015-10-15

    Heterozygous loss of function mutations within the Filamin A gene in Xq28 are the most frequent cause of bilateral neuronal periventricular nodular heterotopia (PVNH). Most affected females are reported to initially present with difficult to treat seizures at variable age of onset. Psychomotor development and cognition may be normal or mildly to moderately impaired. Distinct associated extracerebral findings have been observed and may help to establish the diagnosis including patent ductus arteriosus Botalli, progressive dystrophic cardiac valve disease and aortic dissection, chronic obstructive lung disease or chronic constipation. Genotype-phenotype correlations could not yet be established. Sanger sequencing and MLPA was performed for a large cohort of 47 patients with Filamin A associated PVNH (age range 1 to 65 years). For 34 patients more detailed clinical information was available from a structured questionnaire and medical charts on family history, development, epileptologic findings, neurological examination, cognition and associated clinical findings. Available detailed cerebral MR imaging was assessed for 20 patients. Thirty-nine different FLNA mutations were observed, they are mainly truncating (37/39) and distributed throughout the entire coding region. No obvious correlation between the number and extend of PVNH and the severity of the individual clinical manifestation was observed. 10 of the mutation carriers so far are without seizures at a median age of 19.7 years. 22 of 24 patients with available educational data were able to attend regular school and obtain professional education according to age. We report the clinical and mutation spectrum as well as MR imaging for a large cohort of 47 patients with Filamin A associated PVNH including two adult males. Our data are reassuring in regard to psychomotor and cognitive development, which is within normal range for the majority of patients. However, a concerning median diagnostic latency of 17 to 20

  1. A de novo frameshift in HNRNPK causing a Kabuki-like syndrome with nodular heterotopia.

    Science.gov (United States)

    Lange, L; Pagnamenta, A T; Lise, S; Clasper, S; Stewart, H; Akha, E S; Quaghebeur, G; Knight, S J L; Keays, D A; Taylor, J C; Kini, U

    2016-09-01

    Kabuki syndrome is a heterogeneous condition characterized by distinctive facial features, intellectual disability, growth retardation, skeletal abnormalities and a range of organ malformations. Although at least two major causative genes have been identified, these do not explain all cases. Here we describe a patient with a complex Kabuki-like syndrome that included nodular heterotopia, in whom testing for several single-gene disorders had proved negative. Exome sequencing uncovered a de novo c.931_932insTT variant in HNRNPK (heterogeneous nuclear ribonucleoprotein K). Although this variant was identified in March 2012, its clinical relevance could only be confirmed following the August 2015 publication of two cases with HNRNPK mutations and an overlapping phenotype that included intellectual disability, distinctive facial dysmorphism and skeletal/connective tissue abnormalities. Whilst we had attempted (unsuccessfully) to identify additional cases through existing collaborators, the two published cases were 'matched' using GeneMatcher, a web-based tool for connecting researchers and clinicians working on identical genes. Our report therefore exemplifies the importance of such online tools in clinical genetics research and the benefits of periodically reviewing cases with variants of unproven significance. Our study also suggests that loss of function variants in HNRNPK should be considered as a molecular basis for patients with Kabuki-like syndrome. © 2016 The Authors. Clinical Genetics published by John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  2. PET MRI Coregistration in Intractable Epilepsy and Gray Matter Heterotopia.

    Science.gov (United States)

    Seniaray, Nikhil; Jain, Anuj

    2017-03-01

    A 25-year-old woman with intractable seizures underwent FDG PET/MRI for seizure focus localization. MRI demonstrated bilateral carpetlike nodular subependymal gray matter and asymmetrical focal dilatation in the right temporal horn. PET/MRI showed increased FDG within subependymal gray matter with significant hypometabolism in right anterior temporal lobe. EEG and ictal semiology confirmed the right temporal seizure origin. This case highlights the importance of identification of gray matter heterotopia on FDG PET/MRI.

  3. Role of a heterotrimeric G-protein, Gi2, in the corticogenesis: possible involvement in periventricular nodular heterotopia and intellectual disability.

    Science.gov (United States)

    Hamada, Nanako; Negishi, Yutaka; Mizuno, Makoto; Miya, Fuyuki; Hattori, Ayako; Okamoto, Nobuhiko; Kato, Mitsuhiro; Tsunoda, Tatsuhiko; Yamasaki, Mami; Kanemura, Yonehiro; Kosaki, Kenjiro; Tabata, Hidenori; Saitoh, Shinji; Nagata, Koh-Ichi

    2017-01-01

    We analyzed the role of a heterotrimeric G-protein, Gi2, in the development of the cerebral cortex. Acute knockdown of the α-subunit (Gαi2) with in utero electroporation caused delayed radial migration of excitatory neurons during corticogenesis, perhaps because of impaired morphology. The migration phenotype was rescued by an RNAi-resistant version of Gαi2. On the other hand, silencing of Gαi2 did not affect axon elongation, dendritic arbor formation or neurogenesis at ventricular zone in vivo. When behavior analyses were conducted with acute Gαi2-knockdown mice, they showed defects in social interaction, novelty recognition and active avoidance learning as well as increased anxiety. Subsequently, using whole-exome sequencing analysis, we identified a de novo heterozygous missense mutation (c.680C>T; p.Ala227Val) in the GNAI2 gene encoding Gαi2 in an individual with periventricular nodular heterotopia and intellectual disability. Collectively, the phenotypes in the knockdown experiments suggest a role of Gαi2 in the brain development, and impairment of its function might cause defects in neuronal functions which lead to neurodevelopmental disorders. © 2016 International Society for Neurochemistry.

  4. Eculizumab treatment: stochastic occurrence of C3 binding to individual PNH erythrocytes

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    Michela Sica

    2017-06-01

    Full Text Available Abstract Background C5 blockade by eculizumab prevents complement-mediated intravascular hemolysis in paroxysmal nocturnal hemoglobinuria (PNH. However, C3-bound PNH red blood cells (RBCs, arising in almost all treated patients, may undergo extravascular hemolysis reducing clinical benefits. Despite the uniform deficiency of CD55 and of CD59, there are always two distinct populations of PNH RBCs, with (C3+ and without (C3− C3 binding. Methods To investigate this paradox, the phenomenon has been modeled in vitro by incubating RBCs from eculizumab untreated PNH patients with compatible sera containing eculizumab, and by assessing the C3 binding after activation of complement alternative pathway. Results When RBCs from untreated patients were exposed in vitro to activated complement in the context of C5-blockade, there was the prompt appearance of a distinct C3+ PNH RBC population whose size increased with time and also with the rate of complement activation. Eventually, all PNH RBCs become C3+ to the same extent, without differences between old and young (reticulocytes PNH RBCs. Conclusions This study indicates that the distinct (C3+ and C3− PNH RBC populations are not intrinsically different; rather, they result from a stochastic all-or-nothing phenomenon linked to the time-dependent cumulative probability of each individual PNH red cell to be exposed to levels of complement activation able to trigger C3 binding. These findings may envision novel approaches to reduce C3 opsonization and the subsequent extravascular hemolysis in PNH patients on eculizumab.

  5. Integrity of the corpus callosum in patients with periventricular nodular heterotopia related epilepsy by FLNA mutation

    Directory of Open Access Journals (Sweden)

    Wenyu Liu

    2018-01-01

    Conclusions: These findings support the conclusion that patients with epilepsy secondary to PNH present widespread microstructural changes found in the corpus callosum that extend beyond the macroscopic MRI-visible lesions. This study also indicates that FLNA may affect white matter integrity in this disorder.

  6. Paroxysmal nocturnal hemoglobinuria (PNH)

    Science.gov (United States)

    ... help slow the breakdown of red blood cells. Blood transfusions may be needed. Supplemental iron and folic acid ... is no known way to prevent this disorder. Alternative Names PNH Images Blood cells References Brodsky RA. Proxymal nocturnal hemoglobinuria. In: ...

  7. PNH revisited: Clinical profile, laboratory diagnosis and follow-up

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    Gupta P

    2009-01-01

    Full Text Available Background: Paroxysmal nocturnal hemoglobinuria (PNH is characterized by intravascular hemolysis, marrow failure, nocturnal hemoglobinuria and thrombophila. This acquired disease caused by a deficiency of glycosylphosphatidylinositol (GPI anchored proteins on the hematopoietic cells is uncommon in the Indian population. Materials and Methods: Data of patients diagnosed with PNH in the past 1 year were collected. Clinical data (age, gender, various presenting symptoms, treatment information and follow-up data were collected from medical records. Results of relevant diagnostic tests were documented i.e., urine analysis, Ham′s test, sucrose lysis test and sephacryl gel card test (GCT for CD55 and CD59. Results: A total of 5 patients were diagnosed with PNH in the past 1 year. Presenting symptoms were hemolytic anemia (n=4 and bone marrow failure (n=1. A GCT detected CD59 deficiency in all erythrocytes in 4 patients and CD55 deficiency in 2 patients. A weak positive PNH test for CD59 was seen in 1 patient and a weak positive PNH test for CD55 was seen in 3 patients. All patients were negative by sucrose lysis test. Ham′s test was positive in two cases. Patients were treated with prednisolone and/or androgen and 1 patient with aplastic anemia was also given antithymocyte globulin. A total of 4 patients responded with a partial recovery of hematopoiesis and 1 patient showed no recovery. None of the patients received a bone marrow transplant. Conclusion: The study highlights the diagnostic methods and treatment protocols undertaken to evaluate the PNH clone in a developing country where advanced methods like flowcytometry immunophenotyping (FCMI and bone marrow transplants are not routinely available.

  8. A novel truncating mutation in FLNA causes periventricular nodular heterotopia, Ehlers-Danlos-like collagenopathy and macrothrombocytopenia.

    Science.gov (United States)

    Ieda, Daisuke; Hori, Ikumi; Nakamura, Yuji; Ohshita, Hironori; Negishi, Yutaka; Shinohara, Tsutomu; Hattori, Ayako; Kato, Takenori; Inukai, Sachiko; Kitamura, Katsumasa; Kawai, Tomoki; Ohara, Osamu; Kunishima, Shinji; Saitoh, Shinji

    2018-06-01

    Filamin A (FLNA) is located in Xq28, and encodes the actin binding protein, filamin A. A mutation in FLNA is the most common cause of periventricular nodular heterotopia (PVNH), but a clear phenotype-genotype correlation has not been established. Indeed, some patients with a FLNA mutation have recently been shown to additionally have Ehlers-Danlos-like collagenopathy or macrothrombocytopenia. In an attempt to establish a clearer correlation between clinical symptoms and genotype, we have investigated a phenotype that involves thrombocytopenia in a patient with a truncation of the FLNA gene. We present the case of a 4-year-old girl who, at birth, showed a ventral hernia. At 2 months of age, she was diagnosed with patent ductus arteriosus (PDA) and aortic valve regurgitation. At 11 months, she underwent ligation of the PDA. She was also diagnosed with diaphragmatic eventration by a preoperative test. At 19 months, motor developmental delay was noted, and brain MRI revealed bilateral PVNH with mega cisterna magna. Presently, there is no evidence of epilepsy, intellectual disability or motor developmental delay. She has chronic, mild thrombocytopenia, and a platelet count that transiently decreases after viral infection. Dilation of the ascending aorta is progressing gradually. Genetic testing revealed a de novo nonsense heterozygous mutation in FLNA (NM_001456.3: c.1621G > T; p.Glu541Ter). Immunofluorescence staining of a peripheral blood smear showed a lack of filamin A expression in 21.1% of her platelets. These filamin A-negative platelets were slightly larger than her normal platelets. Our data suggests immunofluorescence staining of peripheral blood smears is a convenient diagnostic approach to identify patients with a FLNA mutation, which will facilitate further investigation of the correlation between FLNA mutations and patient phenotype. Copyright © 2018 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  9. Subependymal heterotopia: a distinct neuronal migration disorder associated with epilepsy.

    Science.gov (United States)

    Raymond, A A; Fish, D R; Stevens, J M; Sisodiya, S M; Alsanjari, N; Shorvon, S D

    1994-01-01

    Subependymal heterotopia has recently been recognised as a cause of epilepsy, but the clinical and investigational features have not been fully described. The clinical, psychometric, imaging, and electroencephalographic features of 13 adult patients with subependymal heterotopia and epilepsy have been reviewed. Age at seizure onset ranged from 18 months to 20 years (median 13 years). There were significantly more female (12) than male (1) patients (p < 0.01). Diagnosis of subependymal heterotopia was made by MRI in 11 patients and CT in two. The heterotopic grey matter was nodular in 11 patients and diffuse in two; bilateral in eight and unilateral in five. There were significantly more patients with predominant right than left cerebral hemisphere involvement (p < 0.01). The most commonly involved site was the occipital horn of the lateral ventricles (10 of 13 patients). Eleven patients presented with partial epilepsy, 10 of whom also had secondarily generalised seizures. The clinical description of the seizures often suggested either an occipital (four patients) or temporal (five patients) onset. Two patients presented with absence attacks without clear focal features. Patients demonstrated normal early milestones (12 of 13 patients), including normal motor development (all patients) and average or above average intelligence (10 of 13 patients). An EEG examination showed normal background activity in all but two patients, one of whom had large intracranial haematomas. Epileptiform activity was usually widespread (10 of 13 patients) and in three patients, there was generalised 3-Hz spike and wave activity that had previously led to an erroneous diagnosis of concomitant primary generalised epilepsy. Onset of epilepsy in the second decade of life, normal developmental milestones and intelligence, and the finding of an overwhelming female preponderance differentiates subependymal heterotopia from other cortical dysgeneses. The female preponderance supports the

  10. A Dual Phenotype of Periventricular Nodular Heterotopia and Frontometaphyseal Dysplasia in One Patient Caused by a Single FLNA Mutation Leading to Two Functionally Different Aberrant Transcripts

    Science.gov (United States)

    Zenker, Martin; Rauch, Anita; Winterpacht, Andreas; Tagariello, Andreas; Kraus, Cornelia; Rupprecht, Thomas; Sticht, Heinrich; Reis, André

    2004-01-01

    Two disorders, periventricular nodular heterotopia (PVNH) and a group of skeletal dysplasias belonging to the oto-palato-digital (OPD) spectrum, are caused by FLNA mutations. They are considered mutually exclusive because of the different presumed effects of the respective FLNA gene mutations, leading to loss of function (PVNH) and gain of function (OPD), respectively. We describe here the first patient manifesting PVNH in combination with frontometaphyseal dysplasia, a skeletal dysplasia of the OPD-spectrum. A novel de novo mutation, 7315C→A in exon 45 of the FLNA gene, was identified. It leads to two aberrant transcripts, one full-length transcript with the point mutation causing a substitution of a highly conserved leucine residue (L2439M) and a second shortened transcript lacking 21 bp due to the creation of an ectopic splice donor site in exon 45. We propose that the dual phenotype is caused by two functionally different, aberrant filamin A proteins and therefore represents an exceptional model case of allelic gain-of-function and loss-of-function phenotypes due to a single mutational event. PMID:14988809

  11. HETEROTOPIA

    OpenAIRE

    Dinesh; Devinder; Jasmine

    2013-01-01

    ABSTRACT: Cortical dysplasias i.e. cortical developmental disorders are emerging as important and common causes of epilepsy with the advent of modern high - resolution MR scanning. Among these, heterotopias are the most common developmental disorders in patients with epilepsy. 1 Heterotopia is the name given to focal collections of ectopic neurons in the cerebral hemispheres, i.e. normal cells present in an abnormal location. It is believed to be the result of pr...

  12. An inter-laboratory comparison of PNH clone detection by high-sensitivity flow cytometry in a Russian cohort.

    Science.gov (United States)

    Sipol, Alexandra A; Babenko, Elena V; Borisov, Vyacheslav I; Naumova, Elena V; Boyakova, Elena V; Yakunin, Dimitry I; Glazanova, Tatyana V; Chubukina, Zhanna V; Pronkina, Natalya V; Popov, Alexander M; Saveliev, Leonid I; Lugovskaya, Svetlana A; Lisukov, Igor A; Kulagin, Alexander D; Illingworth, Andrea J

    2015-01-01

    Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal stem cell disorder characterized by partial or absolute deficiency of glycophosphatidyl-inositol (GPI) anchor-linked surface proteins on blood cells. A lack of precise diagnostic standards for flow cytometry has hampered useful comparisons of data between laboratories. We report data from the first study evaluating the reproducibility of high-sensitivity flow cytometry for PNH in Russia. PNH clone sizes were determined at diagnosis in PNH patients at a central laboratory and compared with follow-up measurements in six laboratories across the country. Analyses in each laboratory were performed according to recommendations from the International Clinical Cytometry Society (ICCS) and the more recent 'practical guidelines'. Follow-up measurements were compared with each other and with the values determined at diagnosis. PNH clone size measurements were determined in seven diagnosed PNH patients (five females, two males: mean age 37 years); five had a history of aplastic anemia and three (one with and two without aplastic anemia) had severe hemolytic PNH and elevated plasma lactate dehydrogenase. PNH clone sizes at diagnosis were low in patients with less severe clinical symptoms (0.41-9.7% of granulocytes) and high in patients with severe symptoms (58-99%). There were only minimal differences in the follow-up clone size measurement for each patient between the six laboratories, particularly in those with high values at diagnosis. The ICCS-recommended high-sensitivity flow cytometry protocol was effective for detecting major and minor PNH clones in Russian PNH patients, and showed high reproducibility between laboratories.

  13. Functional Connectivity and Genetic Profile of a “Double-Cortex”-Like Malformation

    Science.gov (United States)

    Sprugnoli, Giulia; Vatti, Giampaolo; Rossi, Simone; Cerase, Alfonso; Renieri, Alessandra; Mencarelli, Maria A.; Zara, Federico; Rossi, Alessandro; Santarnecchi, Emiliano

    2018-01-01

    Laminar heterotopia is a rare condition consisting in an extra layer of gray matter under properly migrated cortex; it configures an atypical presentation of periventricular nodular heterotopia (PNH) or a double cortex (DC) syndrome. We conducted an original functional MRI (fMRI) analysis in a drug-resistant epilepsy patient with “double-cortex”-like malformation to reveal her functional connectivity (FC) as well as a wide genetic analysis to identify possible genetic substrates. Heterotopias were segmented into region of interests (ROIs), whose voxel-wise FC was compared to that of (i) its normally migrated counterpart, (ii) its contralateral homologous, and (iii) those of 30 age-matched healthy controls. Extensive genetic analysis was conducted to screen cortical malformations-associated genes. Compared to healthy controls, both laminar heterotopias and the overlying cortex showed significant reduction of FC with the contralateral hemisphere. Two heterozygous variants of uncertain clinical significance were found, involving autosomal recessive disease-causing genes, FAT4 and COL18A1. This first FC analysis of a unique case of “double-cortex”-like malformation revealed a hemispheric connectivity segregation both in the laminar cortex as in the correctly migrated one, with a new pattern of genes’ mutations. Our study suggests the altered FC could have an electrophysiological and functional impact on large-scale brain networks, and the involvement of not yet identified genes in “double-cortex”-like malformation with a possible role of rare variants in recessive genes as pathogenic cofactors. PMID:29946244

  14. Identification of a novel synaptic protein, TMTC3, involved in periventricular nodular heterotopia with intellectual disability and epilepsy.

    Science.gov (United States)

    Farhan, Sali M K; Nixon, Kevin C J; Everest, Michelle; Edwards, Tara N; Long, Shirley; Segal, Dmitri; Knip, Maria J; Arts, Heleen H; Chakrabarti, Rana; Wang, Jian; Robinson, John F; Lee, Donald; Mirsattari, Seyed M; Rupar, C Anthony; Siu, Victoria M; Poulter, Michael O; Hegele, Robert A; Kramer, Jamie M

    2017-11-01

    Defects in neuronal migration cause brain malformations, which are associated with intellectual disability (ID) and epilepsy. Using exome sequencing, we identified compound heterozygous variants (p.Arg71His and p. Leu729ThrfsTer6) in TMTC3, encoding transmembrane and tetratricopeptide repeat containing 3, in four siblings with nocturnal seizures and ID. Three of the four siblings have periventricular nodular heterotopia (PVNH), a common brain malformation caused by failure of neurons to migrate from the ventricular zone to the cortex. Expression analysis using patient-derived cells confirmed reduced TMTC3 transcript levels and loss of the TMTC3 protein compared to parental and control cells. As TMTC3 function is currently unexplored in the brain, we gathered support for a neurobiological role for TMTC3 by generating flies with post-mitotic neuron-specific knockdown of the highly conserved Drosophila melanogaster TMTC3 ortholog, CG4050/tmtc3. Neuron-specific knockdown of tmtc3 in flies resulted in increased susceptibility to induced seizures. Importantly, this phenotype was rescued by neuron-specific expression of human TMTC3, suggesting a role for TMTC3 in seizure biology. In addition, we observed co-localization of TMTC3 in the rat brain with vesicular GABA transporter (VGAT), a presynaptic marker for inhibitory synapses. TMTC3 is localized at VGAT positive pre-synaptic terminals and boutons in the rat hypothalamus and piriform cortex, suggesting a role for TMTC3 in the regulation of GABAergic inhibitory synapses. TMTC3 did not co-localize with Vglut2, a presynaptic marker for excitatory neurons. Our data identified TMTC3 as a synaptic protein that is involved in PVNH with ID and epilepsy, in addition to its previously described association with cobblestone lissencephaly. © The Author 2017. Published by Oxford University Press.

  15. Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex evolution and liver transplant

    OpenAIRE

    Alencar,Railene Célia B.; Guimarães,Andréa M.; Brito Junior,Lacy C.

    2016-01-01

    ABSTRACT The paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disease, with thrombotic episodes and frequent pancytopenia. We report the case of a 32 year-old female PNH patient with bone marrow aplasia, which followed a complex course, diagnosed with aplastic anemia associated with PNH, evolving in three years with Budd-Chiari syndrome and liver transplantation. Post-transplant complications, hepatic arterial thrombosis, graft rejection, liver retransplantation and treatment of P...

  16. Church as heterotopia

    Directory of Open Access Journals (Sweden)

    Tanya van Wyk

    2014-09-01

    Full Text Available This article reflects on an ecclesiastical institution as a spatial panoptic structure which domesticates representational space as a hierarchy of power devoid of a sensitivity for the ‘human Other’ (Autrui. The notion of heterotopia is promoted to deconstruct spatiality and linearity (time as theological binary concepts. Being church as heterotopia does not deny the desire for the utopian dimension in religious thinking but holds on to utopian thinking amidst adversity and diversity. Therefore the concept of heterotopia is used to describe reconciliatory diversity, which is characteristic of an inclusive postmodern church which is a space where unity is not threatened by diversity, where the one is not afraid of the Other.

  17. Heterotopia als sociaal-ruimtelijke constructie

    NARCIS (Netherlands)

    Lengkeek, J.

    2003-01-01

    Uitleg van het begrip heterotopia, waar het gaat om het gebruik van de ruimte. Volgens de auteur wordt heterotopia als persoonlijke ruimte en als vrijetijdsruimte op tal van manieren tot stand gebracht; het is een georganiseerde wereld

  18. Heterotopia and structuralism

    OpenAIRE

    Arun Saldanha

    2008-01-01

    The concept of heterotopia was introduced and immediately abandoned by Michel Foucault in 1966 – 67, but it quickly diffused across human geography, urban theory, and cultural studies during the 1990s. Notwithstanding the deserved impact of Foucault’s overall work on these fields, there are some conceptual problems with the heterotopia concept. While the desire for a single term to probe spatial difference is understandable, the author takes issue with the kind of space envisioned in heteroto...

  19. Heterotopias of Homelessness: Citizenship on the Margins

    Science.gov (United States)

    Mendel, Maria

    2011-01-01

    The concept of heterotopia challenges political theory, which has often focused on utopic thinking. Foucault describes a heterotopia as a heterogenous space that juxtaposes in a single real place several spaces, several sites that are in themselves incompatible. Streets, squares and parks form heterotopias when their utopic purity as public space…

  20. Bilateral periventricular nodular heterotopia (BPNH) detected on fetal and maternal MRI, caused by a novel Filamin A mutation.

    Science.gov (United States)

    Stoecklein, S; Haberler, C; Gruber, G; Diogo, M; Ulm, B; Laccone, F A; Prayer, D

    2017-12-20

    We present the case of a 31-year-old, neurologically unremarkable woman who underwent fetal MRI for evaluation of suspected corpus callosum agenesis at 23+0 gestational weeks (GW). On fetal MRI, the corpus callosum appeared thin, but all portions could be clearly delineated (Fig. 1A). However, T2-weighted images revealed subependymal heterotopia and a megacisterna magna (Fig. 1B). This article is protected by copyright. All rights reserved.

  1. Gray matter heterotopias: MR and clinical features

    International Nuclear Information System (INIS)

    Moon, Tae Myung; Yoon, Jeong Hee; Chung, Chun Phil

    1995-01-01

    To evaluate types of gray matter heterotopias, associated brain anomalies, and its correlation with the patterns of seizure. We evaluated retrospectively 19 patients (male:female=10:9, mean age 21 years) with gray matter heterotopias on brain MRI. Using 1.0T superconducting MR unit, spin echo T1-, proton -density and T2-weighted images in axial, coronal and sagittal planes were obtained. Types of gray matter heterotopias were single subependymal in four patients, multiple subependymal in one, focal subcortical in eight, diffuse subcortical in two, mixed multiple subependymal and focal subcortical in four. Associated anomalies were seen in 11 patients: other neuronal migration anomalies in eight patients, corpus callosum agenesis in two, and combined holoprosencephaly and Dandy-Walker malformation in one. Fifteen patients had seizure. The patterns of seizure were not correlated with the types of heterotopias. In addition to subependymal, focal subcortical, and diffuse subcortical types, gray matter heterotopias included mixed variant of multiple subependymal and subcortical type. Schizencephaly was the most common form of accompanying anomalies, and patterns of seizure were not correlated with types of gray matter heterotopias, even though main clinical manifestation was seizure

  2. Foucault's Heterotopia and Children's Everyday Lives.

    Science.gov (United States)

    McNamee, Sara

    2000-01-01

    Discusses Foucault's notion of "heterotopia"--real places but which exist unto themselves, such as a floating ship. Considers data on children's use of computer and video games to apply "heterotopia" to children's everyday social lives. Argues that childhood is subject to increasing boundaries, and that children create…

  3. Glial heterotopia of the oral cavity

    Directory of Open Access Journals (Sweden)

    Radhames E. Lizardo

    2015-07-01

    Full Text Available We report an unusual case of a glial heterotopia arising from the oral cavity of an African neonate. The patient presented with an external pedunculated oral mass which was connected to the anterior hard palate by a firm, rubbery stalk of mucosal tissue. While the mass appeared painless, it interfered with the infant's feeding and was disturbing to the parents. After a computed tomography scan excluded an intracranial connection, the mass was excised at its base and sent for biopsy. Histopathology examination confirmed glial heterotopia. Glial heterotopias should be included in the differential diagnosis of congenital masses in the oral region.

  4. Generation of induced pluripotent stem cells as a potential source of hematopoietic stem cells for transplant in PNH patients.

    Science.gov (United States)

    Phondeechareon, Tanapol; Wattanapanitch, Methichit; U-Pratya, Yaowalak; Damkham, Chanapa; Klincumhom, Nuttha; Lorthongpanich, Chanchao; Kheolamai, Pakpoom; Laowtammathron, Chuti; Issaragrisil, Surapol

    2016-10-01

    Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia caused by lack of CD55 and CD59 on blood cell membrane leading to increased sensitivity of blood cells to complement. Hematopoietic stem cell transplantation (HSCT) is the only curative therapy for PNH, however, lack of HLA-matched donors and post-transplant complications are major concerns. Induced pluripotent stem cells (iPSCs) derived from patients are an attractive source for generating autologous HSCs to avoid adverse effects resulting from allogeneic HSCT. The disease involves only HSCs and their progeny; therefore, other tissues are not affected by the mutation and may be used to produce disease-free autologous HSCs. This study aimed to derive PNH patient-specific iPSCs from human dermal fibroblasts (HDFs), characterize and differentiate to hematopoietic cells using a feeder-free protocol. Analysis of CD55 and CD59 expression was performed before and after reprogramming, and hematopoietic differentiation. Patients' dermal fibroblasts expressed CD55 and CD59 at normal levels and the normal expression remained after reprogramming. The iPSCs derived from PNH patients had typical pluripotent properties and differentiation capacities with normal karyotype. After hematopoietic differentiation, the differentiated cells expressed early hematopoietic markers (CD34 and CD43) with normal CD59 expression. The iPSCs derived from HDFs of PNH patients have normal levels of CD55 and CD59 expression and hold promise as a potential source of HSCs for autologous transplantation to cure PNH patients.

  5. Nasal Glial Heterotopia with Cleft Palate.

    Science.gov (United States)

    Chandna, Sudhir; Mehta, Milind A; Kulkarni, Abhishek Kishore

    2018-01-01

    Congenital midline nasal masses are rare anomalies of which nasal glial heterotopia represents an even rarer subset. We report a case of a 25-day-old male child with nasal glial heterotopia along with cleft palate suggesting embryonic fusion anomaly which was treated with excision and primary closure for nasal mass followed by palatal repair at later date.

  6. The First Korean Case of Cutaneous Lung Tissue Heterotopia

    Science.gov (United States)

    Jeon, Ga Won; Han, Seong Woo; Jung, Ji Mi; Kang, Mi Seon

    2010-01-01

    Cutaneous lung tissue heterotopia is a very rare disorder where mature lung tissues develop in the skin. This is only the second known report of cutaneous lung tissue heterotopia, with the first by Singer et al. in 1998. A newborn infant had a hemangioma-like, freely movable mass connected to the anterior aspect of the sternal manubrium. Pathologic findings showed mature lung tissues with bronchi, bronchioles, and alveoli through the dermis and subcutis, and it was diagnosed as cutaneous lung tissue heterotopia. Cutaneous lung tissue heterotopia is hypervascular, so grossly it looks like a hemangioma. It can be differentiated from pulmonary sequestration, teratoma, bronchogenic cyst, and branchial cleft cyst by histology and the location of the mass. We describe the clinical, radiologic, and pathologic findings of a cutaneous lung tissue heterotopia, the first reported in Korea. PMID:20808688

  7. Nasal glial heterotopia with cleft palate

    Directory of Open Access Journals (Sweden)

    Sudhir Chandna

    2018-01-01

    Full Text Available Congenital midline nasal masses are rare anomalies of which nasal glial heterotopia represents an even rarer subset. We report a case of a 25-day-old male child with nasal glial heterotopia along with cleft palate suggesting embryonic fusion anomaly which was treated with excision and primary closure for nasal mass followed by palatal repair at later date.

  8. Glial heterotopia of maxilla: A clinical surprise

    Directory of Open Access Journals (Sweden)

    Santosh Kumar Mahalik

    2011-01-01

    Full Text Available Glial heterotopia is a rare congenital mass lesion which often presents as a clinical surprise. We report a case of extranasal glial heterotopia in a neonate with unusual features. The presentation, management strategy, etiopathogenesis and histopathology of the mass lesion has been reviewed.

  9. Cortical heterotopia in Aicardi's syndrome - CT findings

    International Nuclear Information System (INIS)

    Besenski, N.; Bosnjak, V.; Ligutic, I.; Marusic-Della Marina, B.

    1988-01-01

    The case of 5-month-old female infant with Aicardi's syndrome is presented. The main clinical features were severe developmental retardation and intractable epileptic seizures. Ophthalmoscopic examination revealed pathognomonic choriorethinopathy. Ultrasonic examination of the brain detected agenesis of the corpus callosum, whereas CT showed a coexisting malformation of the brain, i.e. cortical heterotopia of the gray matter. Agenesis of the corpus callosum is an entity well-recognized by sonography. However, ultrasonography is an insufficient modality for the visualization of cortical heterotopia which is common to all cases of Aicardi's syndrome. Therefore, in cases of suspected Aicardi's syndrome CT is recommended, as it enables the diagnosis of cortical heterotopia. (orig.)

  10. Incidental finding of cutaneous meningeal heterotopia in aplasia cutis congenita.

    Science.gov (United States)

    Kenyon, Katharine; Zedek, Daniel; Sayed, Christopher

    2016-07-01

    Aplasia cutis congenita and cutaneous meningeal heterotopia are both rare congenital conditions that most commonly occur on the scalp and may appear clinically and histologically similar. A subtype of aplasia cutis congenita, membranous aplasia cutis congenita, and cutaneous meningeal heterotopia are both proposed to result from neural tube closure errors. However, neither non-membranous nor membranous aplasia cutis congenita are known to occur together with cutaneous meningeal heterotopia in the same lesion. We report the incidental finding of cutaneous meningeal heterotopia within a lesion of aplasia cutis congenita. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  11. Glial heterotopia of the lip: A rare presentation.

    Science.gov (United States)

    Dadaci, Mehmet; Bayram, Fazli Cengiz; Ince, Bilsev; Bilgen, Fatma

    2016-01-01

    Glial heterotopia represents collections of normal glial tissue in an abnormal location distant to the central nervous system or spinal canal with no intracranial connectivity. Nasal gliomas are non-neoplastic midline tumours, with limited growth potential and no similarity to the central nervous system gliomas. The nose and the nasopharynx are the most common sites of location. Existence of glial heterotopia in the lip region is a rare developmental disorder. We report a case of large glial heterotopia in the upper lip region in a full-term female newborn which had intracranial extension with a fibrotic band. After the surgery, there was no recurrence in the follow-up period of 3 years. When glial heterotopia, which is a rare midline anomaly, is suspected, possible intracranial connection and properties of the mass should be evaluated by magnetic resonance imaging. By this way, lower complication rate and better aesthetic results can be achieved with early diagnosis and proper surgery.

  12. Glial heterotopia of the lip: A rare presentation

    Directory of Open Access Journals (Sweden)

    Mehmet Dadaci

    2016-01-01

    Full Text Available Glial heterotopia represents collections of normal glial tissue in an abnormal location distant to the central nervous system or spinal canal with no intracranial connectivity. Nasal gliomas are non-neoplastic midline tumours, with limited growth potential and no similarity to the central nervous system gliomas. The nose and the nasopharynx are the most common sites of location. Existence of glial heterotopia in the lip region is a rare developmental disorder. We report a case of large glial heterotopia in the upper lip region in a full-term female newborn which had intracranial extension with a fibrotic band. After the surgery, there was no recurrence in the follow-up period of 3 years. When glial heterotopia, which is a rare midline anomaly, is suspected, possible intracranial connection and properties of the mass should be evaluated by magnetic resonance imaging. By this way, lower complication rate and better aesthetic results can be achieved with early diagnosis and proper surgery.

  13. MR imaging of marrow heterotopia in the hemoglobinopathies

    International Nuclear Information System (INIS)

    Trakadas, S.; Papavasiliou, C.; Gouliamos, A.D.; Vlahos, L.

    1987-01-01

    The MR imaging findings of marrow heterotopia in the costovertebral angles in patients with various hemoglobinopathies are presented. There was good correlation between the MR imaging findings and the appearance of the masses on conventional radiography or CT. The masses were of low signal intensity, similar to the signal intensity of the adjacent marrow of the thoracic spine, and surrounded by a high-intensity rim attributed to a layer of fat surrounding the masses. The latter finding is thought to be characteristic of marrow heterotopia masses. MR imaging may also reveal potential intrusion of marrow heterotopia into the spinal canal, thus eliminating the need for myelography

  14. Glial Heterotopia of the orbit: A rare presentation

    Science.gov (United States)

    2011-01-01

    Background Glial heterotopias are rare, benign, congenital, midline, non-teratomatous extracranial glial tissue. They may masquerade as encephalocoele or dermoid cyst and mostly present in nose. Herein, we present an unusual case of glial heterotopia of the orbit with unilateral blindness. Case presentation A 6 year-old-boy presented with a progressive painless mass over the nose and medial aspect of the left eye noticed since birth. On examination, the globe was displaced laterally by a firm, regular, mobile, non-pulsatile and non-tender medial mass. The affected eye had profound loss of vision. Computed tomography scan showed a large hypodense mass in the extraconal space with no intracranial connectivity and bony erosion. The child underwent total surgical excision of the mass and histopathological examination confirmed glial heterotopia of the orbit. Conclusion Though the incidence of this condition is rare, the need of appropriate diagnosis and management of such mass to prevent the visual and cosmetic deterioration is warranted. To our knowledge this is the first reported case of Glial heterotopia of orbit causing unilateral blindness. PMID:22088230

  15. Glial Heterotopia of the orbit: A rare presentation

    Directory of Open Access Journals (Sweden)

    Sitaula Ranju

    2011-11-01

    Full Text Available Abstract Background Glial heterotopias are rare, benign, congenital, midline, non-teratomatous extracranial glial tissue. They may masquerade as encephalocoele or dermoid cyst and mostly present in nose. Herein, we present an unusual case of glial heterotopia of the orbit with unilateral blindness. Case presentation A 6 year-old-boy presented with a progressive painless mass over the nose and medial aspect of the left eye noticed since birth. On examination, the globe was displaced laterally by a firm, regular, mobile, non-pulsatile and non-tender medial mass. The affected eye had profound loss of vision. Computed tomography scan showed a large hypodense mass in the extraconal space with no intracranial connectivity and bony erosion. The child underwent total surgical excision of the mass and histopathological examination confirmed glial heterotopia of the orbit. Conclusion Though the incidence of this condition is rare, the need of appropriate diagnosis and management of such mass to prevent the visual and cosmetic deterioration is warranted. To our knowledge this is the first reported case of Glial heterotopia of orbit causing unilateral blindness.

  16. Presence of acute and chronic renal failure in patients with paroxysmal nocturnal hemoglobinuria: results of a retrospective analysis from the Spanish PNH Registry.

    Science.gov (United States)

    Villegas, Ana; Núñez, Ramiro; Gaya, Anna; Cuevas-Ruiz, María Victoria; Bosch, José Miguel; Carral, Anna; Arrizabalaga, Beatriz; Gómez-Roncero, María Isabel; Mora, Asunción; Bravo, Pilar; Lavilla, Esperanza; Monteserín, Carmen; Hernández, Belén; Martínez-Barranco, Pilar; Jarque, Isidro; Urquía, María Anunciación; García-Donas, Gloria; Brunet, Salut; González, Fernando Ataulfo; Urbano, Álvaro

    2017-10-01

    Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, life-threatening blood disease. With the advent of eculizumab treatment, renal function has substantially improved, although no data from real-world clinical practice are available. An observational, retrospective, multicenter study was conducted in Spain on clinical data obtained from outpatient visits of patients with PNH (Spanish PNH Registry) who had experienced acute (ARF) or chronic (CRF) renal failure. Of the 128 patients registered (April 2014), 60 were diagnosed with classic PNH. Twenty-seven (45.0%) patients with a mean age of 48.5 (±16.2) years had renal failure, ARF or CRF, and were included in this study. Near half of the patients (n = 13; 48.1%) presented with ARF alone, 33.3% (n = 9) had CRF with episodes of ARF, while 18.5% (n = 5) were diagnosed with CRF alone. For patients with diagnosis of PNH and renal failure (n = 27), the median time to the first ARF episode was 6.5 (CI 95%; 2.2, 14.9) years, whereas the median to the diagnosis of CRF was 14.5 (CI 95%; 3.8, 19.2) years after the diagnosis of PNH. Patients with ARF (n = 22) were treated with eculizumab and did not experience new episodes of ARF, except for one patient with sepsis. Of the patients with CRF, two received treatment without experiencing further episodes of ARF. Sixteen patients who completed treatment (11 with ARF and 5 with ARF + CRF) recovered from the episode of ARF or from CRF. Of the remaining patients treated with eculizumab, one patient improved from stages III to II, three patients stabilized without showing disease progression, and one patient progressed from stages III to IV. Treatment with eculizumab in PNH patients has beneficial effects on renal function, preventing ARF and progression to CRF.

  17. Making Payton's Rocket: Heterotopia and Lines of Flight

    Science.gov (United States)

    MacRae, Christina

    2011-01-01

    This article explores the potential of heterotopia as a way to prompt us to think differently about children's art-making. Foucault uses the term to describe a space of difference. As something that is not easily located within a system of representation, a heterotopia is not amenable to interpretation. It is this resistance to interpretation that…

  18. Disease: H01835 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available vances in molecular genetics and brain magnetic resonance imaging. NMD include lissencephaly (H00268), heterotopia...See also H00268 Lissencephaly, H00270 Periventricular nodular heterotopia, H00271 Polymicrogyria, H01160 Sch

  19. Gastric heterotopia in rectum: A literature review and its diagnostic pitfall.

    Science.gov (United States)

    Dinarvand, Peyman; Vareedayah, Ashley A; Phillips, Nancy J; Hachem, Christine; Lai, Jinping

    2017-01-01

    The term heterotopia, in pathology, refers to the presence of normal tissues at foreign sites. Gastric heterotopia has been reported anywhere in the gastrointestinal tract. However, the presence of gastric heterotopia in the rectum is very rare. We, here, report a rare case of a localized 2-cm area of cratered mucosa with heaped-up borders in the rectum of a 51-year-old, asymptomatic woman who underwent screening colonoscopy. Histologic examination of the biopsy from the lesional tissue in rectum demonstrated fragments of rectal mucosa co-mingling with oxyntic- and antral-type gastric mucosa. No intestinal metaplasia or Helicobacter pylori is identified. Patients with gastric heterotopia in rectum usually present with bleeding and/or abdominal pain. Definite treatment of choice is surgical or endoscopic resection, although the lesions also respond to histamine-2 receptor blockers. In this article, most recent literature about gastric heterotopia in rectum is reviewed, following a case presentation about it.

  20. Gastric heterotopia in rectum: A literature review and its diagnostic pitfall

    Directory of Open Access Journals (Sweden)

    Peyman Dinarvand

    2017-02-01

    Full Text Available Objectives: The term heterotopia, in pathology, refers to the presence of normal tissues at foreign sites. Gastric heterotopia has been reported anywhere in the gastrointestinal tract. However, the presence of gastric heterotopia in the rectum is very rare. Methods: We, here, report a rare case of a localized 2-cm area of cratered mucosa with heaped-up borders in the rectum of a 51-year-old, asymptomatic woman who underwent screening colonoscopy. Results: Histologic examination of the biopsy from the lesional tissue in rectum demonstrated fragments of rectal mucosa co-mingling with oxyntic- and antral-type gastric mucosa. No intestinal metaplasia or Helicobacter pylori is identified. Conclusion: Patients with gastric heterotopia in rectum usually present with bleeding and/or abdominal pain. Definite treatment of choice is surgical or endoscopic resection, although the lesions also respond to histamine-2 receptor blockers. In this article, most recent literature about gastric heterotopia in rectum is reviewed, following a case presentation about it.

  1. Tentang Ruang-Ruang yang Lain (atau Heterotopia

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    Michel Foucault

    2017-09-01

    Full Text Available Artikel ini merupakan terjemahan, yang kesekian kalinya ke dalam bahasa Inggris (lih. J. Miskowiec [1986], Jurnal Lotus [1986], & R. Hurley [1998], dari sebuah kuliah bertajuk “Des espaces autres” yang disampaikan Michel Foucault pada 14 Maret 1967 untuk Cercle d’études architecturales (Lingkar Studi Arsitektur. Dalam kuliah tersebut Foucault menguraikan konseptualisasinya tentang ruang-ruang yang lain, atau heterotopia. Foucault menyebutkan ciri-ciri heterotopia sebagai panduan untuk melakukan analisis relasi ruang dan kekuasan.

  2. Application of magnetic source imaging in localizing the epileptic foci in patients with grey matter heterotopia

    International Nuclear Information System (INIS)

    Sun Jilin; Wu Jie; Jia Xiuchuan; Li Sumin

    2011-01-01

    Objective: To evaluate the value of magnetic source imaging (MSI) in localizing the epileptic foci of patients with histologically proved grey matter heterotopia (GMH) and seizure. Methods: MSI examinations were performed on 8 patients with GMH and seizure. The location of the epileptic foci defined by MSI was compared with the results of the ECoG. After imaging examinations, all patients received operation with 13-48 months follow up to observe the effectiveness of the operation. Results: Among the 8 patients, 1 had hippocampal sclerosis, 2 had focal cortical dysplasia of type Ⅰ B and 1 had focal cortical dysplasia of type Ⅱ B. MRI showed normal findings in 2 cases, subcortical heterotopia in 4 cases, and nodular heterotopia in 2 cases with one having schizencephaly. The epileptic foci defined by MSI were at right temporal lobe in 2 cases, left frontal lobe in 2 cases, biparietal lobe in 1 case, left parietal lobe in 1 case, left temporal lobe in 1 case, and left frontal-parietal lobe in 1 case. The epileptic foci defined by MSI were completely overlaid with area of GMH in 4 cases, closely behind the area of GMH in case, and partly overlaid with area of CMH in 1 cases with size larger than that of the latter. One patient showed two epileptic foci with one located within the area of GMH and the other one 2 centimeters anterior to the area of GMH. One case's epileptic focus located 2 centimeters posteolateral to the area of GMH. The locations of the epileptic foci defined by MSI showed no difference with those defined by ECoG in all patients. According to Engel classification of treatment effect of epilepsy, 6 patients achieved Engle class Ⅰ ( seizure free after operation), and 2 patients Engel class Ⅳ (no changes in the frequency of occurrence of seizures before and after operation). Conclusion: MSI can noninvasively and precisely localize the epileptic foci before operation in patients with GMH and seizure. (authors)

  3. Marrow heterotopia in thalassemia

    Energy Technology Data Exchange (ETDEWEB)

    Papavasiliou, C.; Gouliamos, A.; Andreou, J.

    1986-05-01

    The subject of marrow heterotopia has been reviewed on the basis of 15 cases suffering from thalassemia. Other cases reported in the literature were also reviewed. Using conventional radiography, scintigraphy, computerized tomography and myelography, 17% of the cases admitted into the hospital with the diagnosis of Thalassemia, were found to have macroscopic masses of marrow heterotopia. The most common site of development of these masses was the costovertebral gutter, followed by the anterior end of the ribs and the extradural space of the spinal canal. In one case, masses were located in the maxillary antra. The clinical implications, the pathogenesis of the masses and the differential diagnosis from other tumour-like entities are discussed. Three patients presented with symptoms and signs of spinal cord compression. All three patients were treated satisfactorily with small doses of radiotherapy.

  4. Prenatal and postnatal evaluation of polymicrogyria with band heterotopia.

    Science.gov (United States)

    Nagaraj, Usha D; Hopkin, Robert; Schapiro, Mark; Kline-Fath, Beth

    2017-09-01

    The coexistence of band heterotopia and polymicrogyria is extremely rare though it has been reported in the presence of corpus callosum anomalies and megalencephaly. We present prenatal and postnatal MRI findings of a rare case of diffuse cortical malformation characterized by polymicrogyria and band heterotopia. Agenesis of the corpus callosum and megalencephaly were also noted. In addition, bilateral closed-lip schizencephaly was identified on postnatal MRI, which has not been previously reported with this combination of imaging findings. Polymicrogyria with band heterotopia can occur and can be diagnosed with fetal MRI. The coexistence of corpus callosum anomalies and megalencephaly comprises a rare phenotype that has been previously described, suggesting an underlying genetic abnormality.

  5. Cortical heterotopia in Aicardi's syndrome - CT findings

    Energy Technology Data Exchange (ETDEWEB)

    Besenski, N.; Bosnjak, V.; Ligutic, I.; Marusic-Della Marina, B.

    1988-07-01

    The case of 5-month-old female infant with Aicardi's syndrome is presented. The main clinical features were severe developmental retardation and intractable epileptic seizures. Ophthalmoscopic examination revealed pathognomonic choriorethinopathy. Ultrasonic examination of the brain detected agenesis of the corpus callosum, whereas CT showed a coexisting malformation of the brain, i.e. cortical heterotopia of the gray matter. Agenesis of the corpus callosum is an entity well-recognized by sonography. However, ultrasonography is an insufficient modality for the visualization of cortical heterotopia which is common to all cases of Aicardi's syndrome. Therefore, in cases of suspected Aicardi's syndrome CT is recommended, as it enables the diagnosis of cortical heterotopia.

  6. Prenatal and postnatal evaluation of polymicrogyria with band heterotopia

    Directory of Open Access Journals (Sweden)

    Usha D. Nagaraj, MD

    2017-09-01

    Full Text Available The coexistence of band heterotopia and polymicrogyria is extremely rare though it has been reported in the presence of corpus callosum anomalies and megalencephaly. We present prenatal and postnatal MRI findings of a rare case of diffuse cortical malformation characterized by polymicrogyria and band heterotopia. Agenesis of the corpus callosum and megalencephaly were also noted. In addition, bilateral closed-lip schizencephaly was identified on postnatal MRI, which has not been previously reported with this combination of imaging findings. Polymicrogyria with band heterotopia can occur and can be diagnosed with fetal MRI. The coexistence of corpus callosum anomalies and megalencephaly comprises a rare phenotype that has been previously described, suggesting an underlying genetic abnormality.

  7. Clinical and Morphological Aspects of Gray Matter Heterotopia Type Developmental Malformations

    International Nuclear Information System (INIS)

    Zając-Mnich, Monika; Kostkiewicz, Agnieszka; Guz, Wiesław; Dziurzyńska-Białek, Ewa; Solińska, Anna; Stopa, Joanna; Kucharska-Miąsik, Iwona

    2014-01-01

    Gray matter heterotopia (GMH) is a malformation of the central nervous system characterized by interruption of normal neuroblasts migration between the 7 th and 16 th week of fetal development. The aim of the study was the analysis of clinical symptoms, prevalence rate and the most common concurrent central nervous system (CNS) developmental disorders as well as assessment of characteristic morphological changes of gray matter heterotopia in children hospitalized in our institution between the year 2001 and 2012. We performed a retrospective analysis of patients’ data who were hospitalized in our institution between the year 2001 and 2012. We assessed clinical data and imaging exams in children diagnosed with gray matter heterotopia confirmed in MRI (magnetic resonance imaging). GMH occurred in 26 children hospitalized in our institution between the year 2001 and 2012. Among children with gray matter heterotopia most common clinical symptoms were: epilepsy, intellectual disability and hemiparesis. The commonest location of heterotopic gray matter were fronto-parietal areas of brain parenchyma, mostly subependymal region. Gray matter heterotopia occurred with other developmental disorders of the central nervous system rather than solely and in most cases it was bilateral. Schizencephaly and abnormalities of the corpus callosum were the most often developmental disorders accompanying GMH. 1. Subependymal gray matter heterotopia was more common than subcortical GMH. Subependymal GMH showed tendency to localize in the region of the bodies of the lateral ventricles. The least common was laminar GMH. 2. Gray matter heterotopia occurred more often with other developmental disorders of the central nervous system rather than solely. The most frequent concurrent disorders of the central nervous system were: schizencephaly, developmental abnormalities of the corpus callosum, arachnoid cyst, abnormalities of the septum pellucidum and the fornix. 3. GMH foci were more often

  8. Advanced structural multimodal imaging of a patient with subcortical band heterotopia.

    Science.gov (United States)

    Kini, Lohith G; Nasrallah, Ilya M; Coto, Carlos; Ferraro, Lindsay C; Davis, Kathryn A

    2016-12-01

    Subcortical band heterotopia (SBH) is a disorder of neuronal migration most commonly due to mutations of the Doublecortin (DCX) gene. A range of phenotypes is seen, with most patients having some degree of epilepsy and intellectual disability. Advanced diffusion and structural magnetic resonance imaging (MRI) sequences may be useful in identifying heterotopias and dysplasias of different sizes in drug-resistant epilepsy. We describe a patient with SBH and drug-resistant epilepsy and investigate neurite density, neurite dispersion, and diffusion parameters as compared to a healthy control through the use of multiple advanced MRI modalities. Neurite density and dispersion in heterotopia was found to be more similar to white matter than gray matter. Neurite density and dispersion maps obtained using diffusion imaging may be able to better characterize different subtypes of heterotopia.

  9. MR imaging and positron emission tomography of cortical heterotopia

    Energy Technology Data Exchange (ETDEWEB)

    Bairamian, D.; Di Chiro, G.; Theodore, W.H.; Holmes, M.D.; Dorwart, R.H.; Larson, S.M.

    1985-11-01

    Heterotopia of the gray matter is a developmental malformation in which ectopic cortex is found in the white matter of the brain. A case of a 33-year-old man with cortical heterotopia who had a lifelong history of seizures and psychomotor retardation is reported, including the results of cerebral CT, magnetic resonance imaging, and positron emission tomography using YF-2-deoxyglucose.

  10. MR imaging and positron emission tomography of cortical heterotopia

    International Nuclear Information System (INIS)

    Bairamian, D.; Di Chiro, G.; Theodore, W.H.; Holmes, M.D.; Dorwart, R.H.; Larson, S.M.

    1985-01-01

    Heterotopia of the gray matter is a developmental malformation in which ectopic cortex is found in the white matter of the brain. A case of a 33-year-old man with cortical heterotopia who had a lifelong history of seizures and psychomotor retardation is reported, including the results of cerebral CT, magnetic resonance imaging, and positron emission tomography using 18 F-2-deoxyglucose

  11. Evaluation of Subependymal Gray Matter Heterotopias on Fetal MRI.

    Science.gov (United States)

    Nagaraj, U D; Peiro, J L; Bierbrauer, K S; Kline-Fath, B M

    2016-04-01

    Subependymal grey matter heterotopias are seen in a high proportion of children with Chiari II malformation and are potentially clinically relevant. However, despite its growing use, there is little in the literature describing its detection on fetal MRI. Our aim was to evaluate the accuracy in diagnosing subependymal gray matter heterotopias in fetuses with spinal dysraphism on fetal MR imaging. This study is a retrospective analysis of 203 fetal MRIs performed at a single institution for spinal dysraphism during a 10-year period. Corresponding obstetric sonography, postnatal imaging, and clinical/operative reports were reviewed. Of the fetal MRIs reviewed, 95 fetuses were included in our analysis; 23.2% (22/95) were suspected of having subependymal gray matter heterotopias on fetal MR imaging prospectively. However, only 50% (11/22) of these cases were confirmed on postnatal brain MR imaging. On postnatal brain MR imaging, 28.4% (27/95) demonstrated imaging findings consistent with subependymal gray matter heterotopia. Only 40.7% (11/27) of these cases were prospectively diagnosed on fetal MR imaging. Fetal MR imaging is limited in its ability to identify subependymal gray matter heterotopias in fetuses with spinal dysraphism. It is believed that this limitation relates to a combination of factors, including artifacts from fetal motion, the very small size of fetal neuroanatomy, differences in imaging techniques, and, possibly, irregularity related to denudation of the ependyma/subependyma in the presence of spinal dysraphism and/or stretching of the germinal matrix in ventriculomegaly. © 2016 by American Journal of Neuroradiology.

  12. Subcortical laminar heterotopia and lissencephaly in two families: a single X linked dominant gene.

    Science.gov (United States)

    Pinard, J M; Motte, J; Chiron, C; Brian, R; Andermann, E; Dulac, O

    1994-01-01

    Neuronal migration disorders can now be recognised by MRI. This paper reports two families in which the mothers had subcortical laminar heterotopia and four of their children had either similar heterotopia (two girls) or severe pachygyria or lissencephaly (two boys). Laminar heterotopia was more evident on MRI T2 weighted images. The patients had mild to severe epilepsy and mental retardation depending on the extent of cortical abnormalities. In these families, subcortical laminar heterotopia, pachygyria, and lissencephaly seem to share the same X linked or autosomal dominant gene. No chromosomal abnormalities, especially of chromosome 17, could be identified. For appropriate genetic counselling of the family of a child with lissencephaly or subcortical laminar heterotopia, MRI should be performed in parents or siblings with mental retardation or epilepsy. Images PMID:8057113

  13. Megalencephaly, polymicrogyria and ribbon-like band heterotopia: A new cortical malformation.

    Science.gov (United States)

    Kobayashi, Yu; Magara, Shinichi; Okazaki, Kenichi; Komatsubara, Takao; Saitsu, Hirotomo; Matsumoto, Naomichi; Kato, Mitsuhiro; Tohyama, Jun

    2016-11-01

    Megalencephalic polymicrogyria syndromes include megalencephaly-capillary malformation and megalencephaly-polymicrogyria-polydactyly-hydrocephalus. Recent genetic studies have identified that genes in the PI3K-AKT pathway are involved in the pathogenesis of these disorders. Herein, we report a patient who presented with developmental delay, epilepsy and peculiar neuroimaging findings of megalencephaly, polymicrogyria, and symmetrical band heterotopia in the periventricular region. The heterotopias exhibited inhomogeneous signals with undulatory mixtures of gray and white matter, resembling ribbon-like heterotopia, with a predominance in the temporal to occipital regions. These neuroradiological findings were not consistent with those in known megalencephalic polymicrogyria syndromes. No genetic abnormality was identified through whole-exome sequencing. The neuroimaging findings of this patient may represent a novel cortical malformation involving megalencephaly with polymicrogyria and ribbon-like band heterotopia. Copyright © 2016 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  14. The marrow heterotopia in thalassemia

    International Nuclear Information System (INIS)

    Papavasiliou, C.; Gouliamos, A.; Andreou, J.

    1986-01-01

    The subject of marrow heterotopia has been reviewed on the basis of 15 cases suffering from thalassemia. Other cases reported in the literature were also reviewed. Using conventional radiography, scintigraphy, computerized tomography and myelography, 17% of the cases admitted into the hospital with the diagnosis of Thalassemia, were found to have macroscopic masses of marrow heterotopia. The most common site of development of these masses was the costovertebral gutter, followed by the anterior end of the ribs and the extradural space of the spinal canal. In one case, masses were located in the maxillary antra. The clinical implications, the pathogenesis of the masses and the differential diagnosis from other tumour-like entities are discussed. Three patients presented with symptoms and signs of spinal cord compression. All three patients were treated satisfactorily with small doses of radiotherapy. (orig.)

  15. Nasal heterotopia versus pilocytic astrocytoma: A narrow border.

    Science.gov (United States)

    Ellouze, N; Born, J; Hoyoux, C; Michotte, A; Retz, C; Tebache, M; Piette, C

    2015-08-01

    Failure of the anterior neuropore can lead to three main types of anomalies: nasal dermal sinus, encephalocele and nasal glioma or heterotopia. In this report, we describe a case of intracranial and extracranial glial heterotopia that probably resulted from a common failure of anterior neuropore development. We describe the prenatal radiological assessment based on ultrasound and MRI results, and consider their limitation for early fetal diagnosis. We also discuss the embryogenesis and the possible pathogenic mechanisms involved. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  16. Meckel's Diverticulum in Children-Parameters Predicting the Presence of Gastric Heterotopia.

    Science.gov (United States)

    Slívová, Ivana; Vávrová, Zuzana; Tomášková, Hana; Okantey, Okaikor; Penka, Igor; Ihnát, Peter

    2018-05-10

    The presence of gastric ectopic mucosa in Meckel's diverticulum is associated with a higher risk of development of complications. The aim of the present study was to investigate which demographic/clinical parameters predict the presence of gastric heterotopia in Meckel's diverticulum. This was a retrospective cohort study conducted in a single institution (University Hospital Ostrava, Czech republic). All children who underwent laparoscopic/open resection of Meckel's diverticulum within a 20-year study period were included in the study. In total, 88 pediatric patients underwent analysis. The mean age of the children was 4.6 ± 4.73 years; the male-female ratio was approximately 2:1. There were 50 (56.8%) patients with asymptomatic Meckel's diverticulum in our study group. Laparoscopic resection was performed in 24 (27.3%) patients; segmental bowel resection through laparotomy was performed in 13 (14.8%) patients. Gastric heterotopia was found in 39 (44.3%) patients; resection margins of all patients were clear of gastric heterotopia. No correlation was found between the presence of gastric heterotopia and the following parameters: age, gender, maternal age, prematurity, low birth weight, perinatal asphyxia, distance from Bauhin's valve and length of Meckel's diverticulum. The width of the diverticulum base was significantly higher in patients with gastric heterotopia (2.1 ± 0.57 vs. 1.2 ± 0.41 cm; p < 0.001). According to the study outcomes, the width of the diverticulum base seems to be a significant predictive factor associated with the presence of gastric heterotopia in Meckel's diverticulum. The laparoscopic/open resection of asymptomatic MD with a wide base should therefore be recommended.

  17. A Case of Nasal Glial Heterotopia in an Adult

    Directory of Open Access Journals (Sweden)

    Akira Hagiwara

    2014-01-01

    Full Text Available We report a rare case of nasal glial heterotopia in an adult. After the surgery, frontal lobe cerebral hemorrhage developed. A 58-year-old man had unilateral nasal obstruction that progressed for one year. He had been treated for hypertension, chronic heart failure, and cerebral infarction with aspirin and warfarin. A computed tomography scan showed that the tumor occupied the right nasal cavity and the sinuses with small defect in the cribriform plate. The tumor was removed totally with endoscopy. After the operation, the patient developed convulsions and frontal lobe cerebral hemorrhage. The hemorrhage site was located near a defect in the cribriform plate. Nasal glial heterotopia is a rare developmental abnormality, particularly rare in adult. Only few cases were reported. We could not find any report of adult nasal glial heterotopias that developed cerebral hemorrhage as a complication of the surgery.

  18. Heterotopia and capitalism in architecture. The ideological function of Heterotopias as own discourse of the architectural discipline in the age of biopolitical governance

    Directory of Open Access Journals (Sweden)

    Jorge León Casero

    2017-09-01

    Full Text Available Almost all the studies about Heterotopias undertaken in the field of architecture have focused on the identification or development of certain spaces as counter-sites, i.e., spaces able to put up resistance to the disciplinary techniques of an administrative power that normalises and controls individuals. By contrast, the present paper holds that, once the disciplinary conception of power has lead to biopolitics and completed the process of a real subsumption of society into capital, Heterotopias have begun to work unambiguously, optimising neoliberal relations of governance and production, losing the emancipatory power sometimes still ascribed to give way to their having an ideological and mystifying function for the power they purport to resist. Through the concept of Heterotopia, architects, urban planners and geographers continue to act as technicians in the service of capital, while nevertheless sincerely believing in the emancipatory power of their work.

  19. Linking social capital, cultural capital and heterotopia at the folk festival

    Directory of Open Access Journals (Sweden)

    Linda Wilks

    2016-06-01

    Full Text Available This paper investigates the role of folk festivals in transforming interconnections between people, space and culture. It interlinks three sets of theoretical ideas: social capital, cultural capital and heterotopia to suggest a new conceptual framework that will help to frame a deeper understanding of the nature of celebration. Qualitative data were collected at two long-established folk festivals, Sidmouth Folk Festival in southern England and the Feakle Traditional Music Festival in western Ireland, in order to investigate these potential links. Although Foucault did not fully develop the concept of heterotopia, his explanation that heterotopias are counter-sites, which, unlike utopias, are located in real, physical, space-time, has inspired others, including some festival researchers, to build on his ideas. This study concludes that the heterotopian concept of the festival as sacred space, with the stage as umbilicus, may be linked to the building of social capital; while it is suggested that both social capital and appropriate cultural capital are needed to gain full entry to the heterotopia.

  20. Cellular and Axonal Diversity in Molecular Layer Heterotopia of the Rat Cerebellar Vermis

    Directory of Open Access Journals (Sweden)

    Sarah E. Van Dine

    2013-01-01

    Full Text Available Molecular layer heterotopia of the cerebellar primary fissure are a characteristic of many rat strains and are hypothesized to result from defect of granule cells exiting the external granule cell layer during cerebellar development. However, the cellular and axonal constituents of these malformations remain poorly understood. In the present report, we use histochemistry and immunocytochemistry to identify neuronal, glial, and axonal classes in molecular layer heterotopia. In particular, we identify parvalbumin-expressing molecular layer interneurons in heterotopia as well as three glial cell types including Bergmann glia, Olig2-expressing oligodendrocytes, and Iba1-expressing microglia. In addition, we document the presence of myelinated, serotonergic, catecholaminergic, and cholinergic axons in heterotopia indicating possible spinal and brainstem afferent projections to heterotopic cells. These findings are relevant toward understanding the mechanisms of normal and abnormal cerebellar development.

  1. Subependymal Heterotopia Mimicking Mass in Conventional Magnetic Resonance Imaging: Demonstration With 3T Advanced Neuroimages.

    Science.gov (United States)

    Aktas, Filiz; Ogul, Hayri

    2017-10-01

    The authors reported a rare patient with large subependymal heterotopia mimicking cerebral neoplasia. A 22-year-old female was admitted with a history of right-sided paresthesia accompanied by progressive headache. Cerebral magnetic resonance (MR) imaging showed a large solid lesion in the left frontal lobe. Advanced MR images proved that the lesion was compatible with subependymal heterotopia. Large subependymal heterotopia may mimick cerebral neoplasia.

  2. The region's borders: Heterotopia and identity - Epistemological aspect

    Directory of Open Access Journals (Sweden)

    Milenković Pavle D.

    2002-01-01

    Full Text Available This paper has been developed on the margins of Foucault's idea of Heterotopia, on the basic distinction of Utopian and heterotopian. If pre-civil epoch is characterized experience of temporaries, which is overcome by going back (genealogy or leaving to the future (prediction superstition then its main feature is Utopian thinking, which means domination of the conception of Time over the conception of Space. On the contrary, the birth of Moderne introduces, gradually but surely overwhelming of Time over Space, where Utopia is being replaced by Heterotopia. It means, at the same time, abolishing of the eschatology of the pas (myths, genealogy and the future (ideal community, ideal order with an 'eschatology' of the present (here, now, which implies space variables, distribution. If Utopia is the place that is nowhere, then Heterotopia is the place that can be anywhere. Distribution means permanent change of borders, prevents institutionalization in the categories of the classical political theory, and questions categories of Tradition and Identity. In dealing with the problem of a region and regionalization, the paper starts from relation this historical transformation in human through and structuration of relations and the reality derived from these. The basic argument is that a region is paradoxical, if it lies on the local traditional, cultural, which are anachronistic values from the point of view of Heterotopia. At the same time if means that the borders of a region are of changeable, negotiable character, and that they don't have to match the previous administrative, political and cultural entities. The tendency of matching these borders means renewal of Utopia.

  3. Is It Barrett's Esophagus or Gastric Heterotopia

    Directory of Open Access Journals (Sweden)

    Zeynel Mungan

    2014-10-01

    Full Text Available Columnar epithelium in the distal part of the esophagus is generally related to Barrett's esophagus. Barrett's esophagus is a well-known premalignant lesion for adenocarcinoma of the esophagus. Therefore, its diagnosis and surveillance are important. Columnar epithelium in the esophagus other than Barrett's esophagus can be gastric heterotopia, which generally takes place in the upper part of the esophagus and is named inlet patch. The presence of gastric metaplasia in the distal part of the esophagus is rare and can cause misdiagnosis. Therefore, its differentiation from Barrett's esophagus is important. Here we present a case of gastric heterotopia located in the distal part of the esophagus that caused reflux-like symptoms and needed differentiation from Barrett's esophagus.

  4. NEURONS COMPRISING A HETEROTOPIA INDUCED BY DEVELOPMENTAL HYPOTHYROIDISM ARE BORN LATE IN GESTATION.

    Science.gov (United States)

    We previously described an abnormal cluster of neurons, a heterotopia, located in the corpus callosum in rat pups born to dams exposed to the goitrogen, propylthiouracil (PTU, Goodman et al., SfN 2004). In this study we determined 1) whether the formation of the heterotopia was u...

  5. Periventricular heterotopia in common microdeletion syndromes.

    NARCIS (Netherlands)

    Kogelenberg, M. van; Ghedia, S.; McGillivray, G.; Bruno, D.; Leventer, R.; Macdermot, K.; Nelson, J.; Nagarajan, L.; Veltman, J.A.; Brouwer, A.P.M. de; McKinlay Gardner, R.J.; Bokhoven, J.H.L.M. van; Kirk, E.P.; Robertson, S.P.

    2010-01-01

    Periventricular heterotopia (PH) is a brain malformation characterised by heterotopic nodules of neurons lining the walls of the cerebral ventricles. Mutations in FLNA account for 20-24% of instances but a majority have no identifiable genetic aetiology. Often the co-occurrence of PH with a

  6. Nodular Corrosion Characteristics of Zirconium Alloys

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Hyun Gil; Jeong, Y. H.; Park, S. Y.; Lee, D. J

    2003-01-15

    This study was reported the effect of the nodular corrosion on the nuclear reactor environmental along with metallurgical influence, also suggested experimental scheme related to evaluate nodular corrosion characteristics of Zr-1 Nb alloy. Remedial strategies against the nodular corrosion should firstly develop plan to assess the effect of the water quality condition (Oxygen, Hydrogen) as well as the boiling on the nodular corrosion, secondarily establish plan to control heat treatment process to keep a good resistance on nodular corrosion in Zr-1Nb alloy as former western reactor did.

  7. Divergence and inheritance of neocortical heterotopia in inbred and genetically-engineered mice.

    Science.gov (United States)

    Toia, Alyssa R; Cuoco, Joshua A; Esposito, Anthony W; Ahsan, Jawad; Joshi, Alok; Herron, Bruce J; Torres, German; Bolivar, Valerie J; Ramos, Raddy L

    2017-01-18

    Cortical function emerges from the intrinsic properties of neocortical neurons and their synaptic connections within and across lamina. Neurodevelopmental disorders affecting migration and lamination of the neocortex result in cognitive delay/disability and epilepsy. Molecular layer heterotopia (MLH), a dysplasia characterized by over-migration of neurons into layer I, are associated with cognitive deficits and neuronal hyperexcitability in humans and mice. The breadth of different inbred mouse strains that exhibit MLH and inheritance patterns of heterotopia remain unknown. A neuroanatomical survey of numerous different inbred mouse strains, 2 first filial generation (F1) hybrids, and one consomic strain (C57BL/6J-Chr 1 A/J /NaJ) revealed MLH only in C57BL/6 mice and the consomic strain. Heterotopia were observed in numerous genetically-engineered mouse lines on a congenic C57BL/6 background. These data indicate that heterotopia formation is a weakly penetrant trait requiring homozygosity of one or more C57BL/6 alleles outside of chromosome 1. These data are relevant toward understanding neocortical development and disorders affecting neocortical lamination. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  8. Nodular scleritis following alendronate therapy.

    Science.gov (United States)

    Tabbara, Khalid F

    2008-01-01

    To report a case of nodular scleritis following alendronate sodium. A 54 year-old male was treated with alendronate sodium 70 mg orally once per week. Eight weeks later, he experienced pain and redness of the right eye. Biomicroscopy was performed. Patient was found to have inferonasal nodular scleritis. Laboratory work-up was unrevealing. Patient was given 4 mg of subtenon's triamcinolone acetate in the area of the nodular scleritis and had complete resolution. Two weeks following rechallenge with alendronate sodium resulted in recurrence of his scleritis. Alendronate led to nodular scleritis and rechallenge caused recurrence of scleritis.

  9. Asymptomatic gastric heterotopia in the rectum with Helicobacter pylori infection.

    Science.gov (United States)

    Swatek, Jarosław; Wronecki, Lech; Ciechanek, Roman; Szumiło, Justyna

    2015-12-01

    Gastric heterotopia is very rare in the rectum - less than 50 cases have been reported so far. Only in six of them Helicobacter pylori has been observed in heterotopic mucosa. We report a case of a 58-year-old woman with asymptomatic gastric heterotopia in the rectum, incidentally revealed during colonoscopy as a small, sessile polyp. The presence of H. pylori was confirmed by immunohistochemistry. This finding supports the opinion that H. pylori may pass along the gastrointestinal tract in a viable form and that the fecal-oral route of transmission is possible.

  10. Subcortical laminar heterotopia in two sisters and their mother : MRI, clinical findings and pathogenesis

    NARCIS (Netherlands)

    van der Valk, PHM; Snoeck, [No Value; Meiners, LC; des Portes, [No Value; Chelly, J; Pinard, JM; Ippel, PF; van Nieuwenhuizen, O

    MR imaging, clinical data and underlying pathogenesis of subcortical laminar heterotopia (SCLH), also known as band heterotopia, in two sisters and their mother are presented. On MR imaging a different degree of SCLH was found in all three affected family-members. The inversion recovery sequence was

  11. A Doubled Heterotopia: Shifting Spatial and Visual Symbolism in the Jewish Museum Berlin's Development

    Science.gov (United States)

    Saindon, Brent Allen

    2012-01-01

    This essay considers the rhetoric of space in a rapidly transforming culture. Using Michel Foucault's concept of "heterotopias" to understand the rhetorical power of a building's disposition, it is argued that the Jewish Museum Berlin contains two heterotopias, one within the other. The first is Daniel Libeskind's original building…

  12. Jejunal Gastric Heterotopia causing Multiple Strictures and Perforation Peritonitis- A Case Report with Review of Literature.

    Science.gov (United States)

    Vani, M; Nambiar, Ajit; Geetha, K; Kundil, Byju

    2017-03-01

    Gastric heterotopias beyond the ligament of Treitz though rare, should be thought of in the differential diagnosis of polypoid lesions presenting with gastrointestinal bleed or obstructive symptoms especially in children and in the young. Here is a 24-year-old male with multifocal jejunal gastric heterotopias causing multiple strictures and perforation peritonitis. Patient presented with acute abdomen pain and an emergency laparotomy was performed revealing jejunum with multiple strictures and perforation, followed by jejunal resection. On gross examination polypoid mucosa was noted at the stricture sites which showed heterotopic gastric mucosa on microscopy. Jejunal gastric heterotopias are extremely rare with less than ten reported cases and those presenting with multiple strictures are even rarer. To our knowledge this is the second case of jejunal gastric heterotopia presenting with multiple strictures.

  13. Nerdfighters, "Paper Towns," and heterotopia

    Directory of Open Access Journals (Sweden)

    Lili Wilkinson

    2012-06-01

    Full Text Available Socially and politically controlled teenagers find emancipatory spaces in young adult (YA literature, spaces where institutions can be challenged and individuals can gain agency and empowerment. Drawing on Foucault's theory of heterotopia, I examine the literary spaces in John Green's YA novel Paper Towns and examine how Green's online social networking community Nerdfighters shares an ideological common ground with the novel.

  14. Different spaces : Exploring Facebook as heterotopia

    NARCIS (Netherlands)

    Rymarczuk, R.; Derksen, Maarten

    2014-01-01

    In this paper we explore the space of Facebook, and use Michel Foucault’s concept of heterotopia to describe it. We show that the heterotopic nature of Facebook explains not only much of its attraction, but even more the discomfort that many people, users as well as non–users, experience in it.

  15. Neuroglial Heterotopia and Nasopharyngeal Obstruction in a Neonate

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2008-12-01

    Full Text Available The clinical presentation, imaging, treatment, and pathology of a case of neuroglial heterotopia in the nasopharynx causing airway obstruction in a newborn are reported from Columbus Children’s Hospital, OH.

  16. Superficially located enlarged lymphoid follicles characterise nodular gastritis.

    Science.gov (United States)

    Okamura, Takuma; Sakai, Yasuhiro; Hoshino, Hitomi; Iwaya, Yugo; Tanaka, Eiji; Kobayashi, Motohiro

    2015-01-01

    Nodular gastritis is a form of chronic Helicobacter pylori gastritis affecting the gastric antrum and characterised endoscopically by the presence of small nodular lesions resembling gooseflesh. It is generally accepted that hyperplasia of lymphoid follicles histologically characterises nodular gastritis; however, quantitative analysis in support of this hypothesis has not been reported. Our goal was to determine whether nodular gastritis is characterised by lymphoid follicle hyperplasia.The number, size, and location of lymphoid follicles in nodular gastritis were determined and those properties compared to samples of atrophic gastritis. The percentages of high endothelial venule (HEV)-like vessels were also evaluated.The number of lymphoid follicles was comparable between nodular and atrophic gastritis; however, follicle size in nodular gastritis was significantly greater than that seen in atrophic gastritis. Moreover, lymphoid follicles in nodular gastritis were positioned more superficially than were those in atrophic gastritis. The percentage of MECA-79 HEV-like vessels was greater in areas with gooseflesh-like lesions in nodular versus atrophic gastritis.Superficially located hyperplastic lymphoid follicles characterise nodular gastritis, and these follicles correspond to gooseflesh-like nodular lesions observed endoscopically. These observations suggest that MECA-79 HEV-like vessels could play at least a partial role in the pathogenesis of nodular gastritis.

  17. Unilateral periventricular heterotopia and epilepsy in a girl with Ehlers–Danlos syndrome

    Directory of Open Access Journals (Sweden)

    Salvatore Savasta

    2015-01-01

    Conclusion: To our knowledge, this is the first report of unilateral periventricular heterotopia associated with Ehlers–Danlos syndrome. We first hypothesized a mosaicism as the cause of both, a unilateral localization of the heterotopias and a favorable long-term course with good response to anticonvulsant therapy; however, intriguingly, we could not demonstrate a mosaicism as the genetic condition in our patient and the neuroradiological findings and the favorable clinical outcome still remain unexplained.

  18. Double Cortex Syndrome (Subcortical Band Heterotopia): A Case Report.

    Science.gov (United States)

    Momen, Ali Akbar; Momen, Mehdi

    2015-01-01

    Objective Approximately 5-10% of preschool age children are considered developmentally disabled. Brain Magnetic Resonance Imaging (MRI) plays a key role in the diagnostic evaluation in these children. Many congenital or acquired brain anomalies are revealed with MRIs. Although the majority of these abnormalities are sporadic but patients with subcortical band heterotopia or double cortex syndrome have sex-linked inheritance. We are going to present the first case in Iran from Ahvaz city, which was presented with status epilepticus associated with developmental delay and finally diagnosed as double cortex syndrome, because band heterotopia cases especially for continuous or generalized form is rare. A 4.5-year-old developmentally delayed girl was admitted for generalized tonic clonic seizure attack of 1 hr, upward gaze, locked mouth, and urinary incontinence (status epilepticus) in the child neurology ward. She had a history of recurrent seizures that started as febrile seizures since she was 12 months of age and had frequent admissions for having recurrent seizure attacks. She was the only child of consanguineous parents with negative family history of any neurologic problems. She was a product of uneventful term pregnancy, vaginal delivery with a low Apgar score at birth who was admitted for six days in the neonatal ward for hypotonia and cyanosis. At 4.5 years of age, she had HC: 45cm (spike-wave discharges. A brain MRI showed corpus callosal dysplasia, generalized band heterotopia, and polymicrogyria. She was discharged home with oral valproate and regular outpatient follow-ups. In the diagnostic evaluation of developmentally delayed and epileptic children, a brain MRI is strongly recommended for accurate diagnosis of anomalies such as neuronal migration disorders (band heterotopia) and others, because appropriate therapeutic management, prognosis, prevention, and genetic counseling for prenatal diagnosis are dependent on definite diagnosis of the proband case.

  19. A Mass of Pancreatic and Gastric Heterotopia Causing a Small Bowel Obstruction in a 61-Year-Old Male.

    Science.gov (United States)

    Alfrejat, Majd; Khalil, Bassem; Jackobs, Jordan; Anderson, William; Eschbacher, Jennifer

    2017-01-01

    Heterotopic tissue is a congenital anomaly that has been previously reported. Gastric and pancreatic heterotopia are among the most studied ones. Herein, we describe a case of a combined pancreatic and gastric heterotopia that formed a mass and caused a small intestine obstruction in a 61-year-old male. We also did a brief literature review of cases with gastric and pancreatic heterotopia in adult patients.

  20. Carnivorous heterotopias

    DEFF Research Database (Denmark)

    Lapina, Linda; Leer, Jonatan

    2016-01-01

    . We argue that these spaces of consumption express nostalgia and longing for authenticity that are simultaneously articulated as progressive and emancipatory. Consequently, these sites represent middle-class masculine counter-spaces – masculine, carnivorous heterotopias where archaic, working class...... and decorations), appeasing a presumed masculine appetite and conveying ideas about masculine, carnivorous bonding/community and a masculine, heterosexual, middle-class gaze. This article examines two manifestations of these celebrations of meat and masculinity: the hotdog restaurant Foderbrættet (‘The Bird Table......’, opened in 2014 and elected as the 2014 Best New Restaurant in Copenhagen) and WarPigs, a Texas-inspired barbecue opened in 2015. We discuss negotiations of masculinity in these meatscapes that challenge contemporary ideals for (sustainable, moderate, wholesome) food consumption and gender performances...

  1. A Mass of Pancreatic and Gastric Heterotopia Causing a Small Bowel Obstruction in a 61-Year-Old Male

    Directory of Open Access Journals (Sweden)

    Majd Alfrejat

    2017-01-01

    Full Text Available Heterotopic tissue is a congenital anomaly that has been previously reported. Gastric and pancreatic heterotopia are among the most studied ones. Herein, we describe a case of a combined pancreatic and gastric heterotopia that formed a mass and caused a small intestine obstruction in a 61-year-old male. We also did a brief literature review of cases with gastric and pancreatic heterotopia in adult patients.

  2. [Subcortical laminar heterotopia 'double cortex syndrome'].

    Science.gov (United States)

    Teplyshova, A M; Gaskin, V V; Kustov, G V; Gudkova, A A; Luzin, R V; Trifonov, I S; Lebedeva, A V

    2017-01-01

    This article presents a clinical case of a 29-year-old patient with 'Double cortex syndrome' with epilepsy, intellectual and mental disorders. Subcortical band heterotopia is a rare disorder of neuronal migration. Such patients typically present with epilepsy and variable degrees of mental retardation and behavioral and intellectual disturbances. The main diagnostic method is magnetic resonance imaging (MRI).

  3. Unusual Case of Combined Gliomeningeal Heterotopia on the Nose of an Infant.

    Science.gov (United States)

    Schauer, Anna; Harvey, Nathan T; Vijayasekaran, Shyan; Wood, Benjamin A

    2017-10-24

    Nasal glial heterotopia ("nasal glioma") and cutaneous heterotopic meningeal nodules ("primary cutaneous meningioma") are rare congenital lesions characterized by the presence of heterotopic mature cerebral tissues. Nasal glial heterotopia occurs predominantly in the nasal area and typically does not contain meningothelial elements, whereas heterotopic meningeal nodules occur predominantly on the scalp and do not contain glial elements. In this article, we report an unusual case of cutaneous heterotopia on the nose of an infant composed of both glial and meningothelial elements. The glial component was characterized by irregular islands of predominantly astrocytic cells, on a fibrillary background. The meningothelial component was characterized by bland ovoid cells with focal intranuclear inclusions forming whorled arrangements, with associated psammomatous calcification. To our knowledge, this is the first time such a lesion has been documented. It has also provided us with an opportunity to review the literature regarding heterotopic deposits of both glial and meningothelial tissues.

  4. Malignant Nodular Hidradenoma of Face

    Directory of Open Access Journals (Sweden)

    Bansal N

    2014-10-01

    Full Text Available We report a case of malignant nodular hidradenoma in an old woman, who presented with a nodular swelling in the right side of nose near the medial canthus of the right eye. Wide excision of the nodular mass with a clear margin of healthy surrounding tissue was performed along with primary closure. Post operatively, adjuvant radiation therapy was given on a telecobalt machine due to the presence of high risk features. In general, malignant forms of hidradenomas are not usual and treatment strategies should be individualized.

  5. Is endoscopic nodular gastritis associated with premalignant lesions?

    Science.gov (United States)

    Niknam, R; Manafi, A; Maghbool, M; Kouhpayeh, A; Mahmoudi, L

    2015-06-01

    Nodularity on the gastric mucosa is occasionally seen in general practice. There is no consensus about the association of nodular gastritis and histological premalignant lesions. This study is designed to investigate the prevalence of histological premalignant lesions in dyspeptic patients with endoscopic nodular gastritis. Consecutive patients with endoscopic nodular gastritis were compared with an age- and sex-matched control group. Endoscopic nodular gastritis was defined as a miliary nodular appearance of the gastric mucosa on endoscopy. Biopsy samples of stomach tissue were examined for the presence of atrophic gastritis, intestinal metaplasia, and dysplasia. The presence of Helicobacter pylori infection was determined by histology. From 5366 evaluated patients, a total of 273 patients with endoscopic nodular gastritis and 1103 participants as control group were enrolled. H. pylori infection was detected in 87.5% of the patients with endoscopic nodular gastritis, whereas 73.8% of the control group were positive for H. pylori (p gastritis were significantly higher than in the control group. Prevalence of atrophic gastritis and complete intestinal metaplasia were also more frequent in patients with endoscopic nodular gastritis than in the control group. Dysplasia, incomplete intestinal metaplasia and H. pylori infection are significantly more frequent in patients with endoscopic nodular gastritis. Although further studies are needed before a clear conclusion can be reached, we suggest that endoscopic nodular gastritis might serve as a premalignant lesion and could be biopsied in all patients for the possibility of histological premalignancy, in addition to H. pylori infection.

  6. ICCS/ESCCA consensus guidelines to detect GPI-deficient cells in paroxysmal nocturnal hemoglobinuria (PNH) and related disorders part 4 - assay validation and quality assurance.

    Science.gov (United States)

    Oldaker, Teri; Whitby, Liam; Saber, Maryam; Holden, Jeannine; Wallace, Paul K; Litwin, Virginia

    2018-01-01

    Over the past six years, a diverse group of stakeholders have put forth recommendations regarding the analytical validation of flow cytometric methods and described in detail the differences between cell-based and traditional soluble analyte assay validations. This manuscript is based on these general recommendations as well as the published experience of experts in the area of PNH testing. The goal is to provide practical assay-specific guidelines for the validation of high-sensitivity flow cytometric PNH assays. Examples of the reports and validation data described herein are provided in Supporting Information. © 2017 International Clinical Cytometry Society. © 2017 International Clinical Cytometry Society.

  7. Sonographic Features of Nodular Hashimoto Thyroiditis.

    Science.gov (United States)

    Oppenheimer, Daniel Corey; Giampoli, Ellen; Montoya, Simone; Patel, Swapnil; Dogra, Vikram

    2016-09-01

    The aim of the study was to analyze the sonographic features of nodular Hashimoto thyroiditis (HT) in patients with diffuse background thyroiditis and normal background thyroid parenchyma. Eighty-six patients who had fine-needle aspiration biopsy of 100 thyroid nodules confirmed to be HT and a thyroid ultrasound within 1 year of the biopsy were included in the study. Retrospective analysis of several sonographic features of each nodule was then performed. The mean age of patients with nodular HT was 53 years, 84% of which were female. Nodular HT occurred in a background of diffuse thyroiditis in 85% and in a homogeneous normal background in 15%. Ninety-three percent of nodules were completely solid and 7% of nodules were cystic and solid. Although the sonographic appearance of nodular HT was variable, the most common appearance was a solid (93/100) and hypoechoic nodule (44/100) with a thin hypoechoic halo (42/100) without calcifications (96/100). On color Doppler, 17% of nodules showed peripheral hypervascularity, 14% of nodules were diffusely hypervascular, 34% were iso vascular, 32% were hypovascular, and 3% were avascular. The sonographic appearance of nodular HT was not significantly different in patients with diffuse background thyroiditis compared with those without background thyroiditis. The sonographic appearance of nodular HT is variable, but the most common appearance is a solid sharply circumscribed hypoechoic nodule with thin hypoechoic halo without calcification. There was no significant difference in the appearance of nodular HT in patients with diffuse background thyroiditis compared with patients with normal background thyroid parenchyma.

  8. A Further Education College as a Heterotopia

    Science.gov (United States)

    Blair, Erik

    2009-01-01

    Foucault suggests that there are "other spaces", heterotopias, which are located in the world we live in but are "outside of all places, even though it may be possible to indicate their location in reality". Foucault offers examples of this concept by suggesting that prisons, brothels and boats at sea are specific spaces that…

  9. Tracing Heterotopias: Writing Women Educators in Greece

    Science.gov (United States)

    Tamboukou, Maria

    2004-01-01

    Over the last 15 years, feminist theorists have sought to redefine female subjectivity. Amongst a wide range of critical notions of the female self, this paper focuses on what Foucault has defined as heterotopias, 'different places' which disrupt the dominance of the one single 'real' social place, offering shelter to subjects in crisis. I will…

  10. Neuropsychological Profile of Children with Subcortical Band Heterotopia

    Science.gov (United States)

    Spencer-Smith, Megan; Leventer, Richard; Jacobs, Rani; De Luca, Cinzia; Anderson, Vicki

    2009-01-01

    Aim: Subcortical band heterotopia (SBH) or "double cortex" is a malformation of cortical development resulting from impaired neuronal migration. So far, research has focused on the neurological, neuroimaging, and genetic correlates of SBH. More recently, clinical reports and small sample studies have documented neuropsychological dysfunction in…

  11. Giant polypoid gastric heterotopia of jejunum

    Directory of Open Access Journals (Sweden)

    Suresh Ramchandra Shenovi Mandrekar

    2016-01-01

    Full Text Available Heterotopic gastric tissue has been described in various parts of the gastrointestinal tract as an incidental finding. However, its presentation as a mass in the jejunum with obstructive manifestations is a rare event. We report here a rare case of giant polypoid gastric heterotopia in the jejunum that presented with intestinal obstruction in a 22-year-old female, along with a brief review of the literature.

  12. Glial heterotopia of the orbit: a rare cause of proptosis.

    Science.gov (United States)

    Bakhti, Souad; Terkmani, Fella; Tighilt, Nabila; Benmouma, Youcef; Boumehdi, Nazim; Djennas, Mohamed

    2016-11-01

    Glial heterotopia is defined as presence of normal glial tissue in an unusual location without connection with the brain. It is a very rare clinical entity occuring mostly in the head and neck region which is generally present at birth. Orbital location is very rare. We report a case of a 4-month-old girl presenting congenital proptosis with progressive increase. CT scan revealed an intraorbital mass without bony defect. The patient was operated, and resection was subtotal. Histologically, the tumor was composed of glial tissue with plexus choroid and pathologist concluded glial heterotopia. The child is under constant medical supervision because recurrences can be observed after incomplete resection; she had no new clinical signs at 18 months follow-up.

  13. ‘Double cortex’ sign on FDG-PET/CT in diffuse band heterotopia

    International Nuclear Information System (INIS)

    Tripathi, Madhavi; Tripathi, Manjari; Kumar, Ganesh; Malhotra, Arun; Bal, Chandra Sekhar

    2013-01-01

    F-18 Fluorodeoxyglucose (FDG) Positron emission tomography/Computed Tomography (PET/CT) has come to play an increasingly important role for the pre-surgical evaluation of drug resistant epilepsy and complements Magnetic Resonance Imaging (MRI) in the evaluation of grey matter heterotopias. This case illustrates the characteristic pattern of metabolic abnormality in diffuse band heterotopia (DBH) which is otherwise called double cortex syndrome. The presence of metabolic activity in the heterotopic inner cortical band and in the overlying true cortex gives rise to the ‘double cortex’ sign on FDG-PET, concurrent CT provides a good anato-metabolic coregistration

  14. ‘Double cortex’ sign on FDG-PET/CT in diffuse band heterotopia

    Science.gov (United States)

    Tripathi, Madhavi; Tripathi, Manjari; Kumar, Ganesh; Malhotra, Arun; Bal, Chandra Sekhar

    2013-01-01

    F-18 Fluorodeoxyglucose (FDG) Positron emission tomography/Computed Tomography (PET/CT) has come to play an increasingly important role for the pre-surgical evaluation of drug resistant epilepsy and complements Magnetic Resonance Imaging (MRI) in the evaluation of grey matter heterotopias. This case illustrates the characteristic pattern of metabolic abnormality in diffuse band heterotopia (DBH) which is otherwise called double cortex syndrome. The presence of metabolic activity in the heterotopic inner cortical band and in the overlying true cortex gives rise to the ‘double cortex’ sign on FDG-PET, concurrent CT provides a good anato-metabolic coregistration. PMID:24379541

  15. Intracardiac heterotopia--mesenchymal and endodermal.

    Science.gov (United States)

    Ariza, S; Rafel, E; Castillo, J A; Garcia-Canton, J A

    1978-01-01

    A case is reported of an intracardiac 'epithelial heterotopia' with a predominant mesenchymal component. This is thought to have resulted from the differentiation of aberrant primitive cell(s) displaced into the heart during its development. Though microscopically resembling a myxoma, this lesion is clearly distinguished by the presence of glandular structures. The myxoid component exhibited a startling invasiveness which resulted in occlusion of the superior vena cava, causing symptoms very early in life and death at the age of 6 months. Images PMID:637987

  16. Airport Heterotopia

    DEFF Research Database (Denmark)

    Christiansen, Steen Ledet

    Airports are areas of transit, places of in-between-ness where mobility is key; airports function not just as transfer points where people are moved from one place to another, but also as areas where mobile technology is critically important to make sure that travellers can stay connected....... The airport serves as a node in the network of flows that is air travel. Airports orchestrate social life into distinct movements and behaviors. The ontology of the airport is peculiarly split between a sense of placelessness, while at the same time being a place of material organization and social complexity....... It is a system of interconnected material worlds, and thus a heterotopia; a blending space of overlapping ontologies. Ursula Le Guin playfully examines this concept of overlapping ontologies in her short story collection Changing Planes, where airport travellers can shift realities due to the unpleasantries...

  17. Symptomatic pancreatic heterotopia treated by local excision.

    Science.gov (United States)

    De Friend, D J; Saa-Gandi, F W; Humphrey, C S; Foster, D N

    1991-01-01

    Non-ulcer dyspepsia is a continuing problem and in many cases a precise cause is never identified. We present five patients with an allegedly uncommon condition--pancreatic heterotopia. They were managed by local excision of the tumour and after a mean (range) follow up of 42 (9-80) months all remain free of the original symptoms. Images Figure 1 Figure 2 PMID:2013433

  18. RF-ablation in periventricular heterotopia-related epilepsy.

    Science.gov (United States)

    Cossu, Massimo; Mirandola, Laura; Tassi, Laura

    2018-05-01

    Drug-resistant focal epilepsy is a common occurrence in patients with gray matter nodular heterotopia (NH), and surgical treatment is often considered in these cases. NH-related epileptogenicity is sustained by complex networks, which may involve the nodules and extralesional cortex in various combinations. Therefore, invasive EEG is usually required to identify the structures involved in seizure generation. It has been reported that surgery may be effective in cases with unilateral lesions, whereas bilateral cases are not optimal candidates for surgical success. Furthermore, violation of cortical and subcortical structures for approaching deep-seated nodules may result in neurological deficits. For these reasons, selective stereotactic ablation with radiofrequency thermocoagulation (RFTC) has been proposed as an alternative option in these patients. In particular, RFTC may be performed by using the same recording intracerebral electrodes implanted for stereo-electro-encephalo-graphy (SEEG) monitoring, with the advantage of a reliable electro-clinical guide. Excellent results on seizures have been initially reported following coagulation of single, unilateral NH. Subsequent experience has indicated that, basing on the evidence of SEEG recording, promising results may be obtained also in more extended unilateral and bilateral cases. In more complex cases, coagulation of both the nodules and of the involved extralesional cortical structures is often required. In a recently reported series, 67% of patients experienced sustained seizure freedom after the procedure. However, post RFTC seizure outcome in complex cases (NH plus other malformations of cortical development) is not as good as in other patterns of NH. RFTC, especially if guided by SEEG evaluation, should be considered as a first-line treatment option in NH-related epilepsy. Satisfactory results may be obtained also in cases not amenable to traditional surgery. The procedure is safe and does not prevent

  19. Os Circos Contemporâneos como Heterotopias Organizacionais: Uma Etnografia Multissituada no Contexto Brasil-Canadá

    Directory of Open Access Journals (Sweden)

    Josiane Silva de Oliveira

    2017-03-01

    Full Text Available Neste artigo buscamos entender os circos contemporâneos desde a perspectiva das heterotopias organizacionais. Realizamos uma aproximação teórica entre Estudos Baseados em Práticas nos Estudos Organizacionais e o conceito de heterotopias desenvolvido por Michel Foucault. A partir de uma etnografia multissituada focada nos processos organizativos do circo contemporâneo no Brasil e no Canadá, identificamos conjuntos de práticas organizativas que alteraram as relações de forças no campo das artes nos contextos estudados, resultando na produção de outros espaços – ou heterotopias, de acordo com Michel Foucault – na sociedade que possibilitaram os circos se constituírem como organizações. Como contribuição teórica, apresentamos o conceito de heterotopias organizacionais para as análises de organizações que se constituem com base em multiplicidade socioespaciais. A contribuição metodológica do artigo é a apresentação da etnografia multissituada como estratégia de pesquisa de estudos dos processos organizacionais estabelecidos em diferentes contextos culturais e localidades.

  20. Disease: H01930 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available e syndrome with nodular heterotopia. ... JOURNAL ... Clin Genet 90:258-62 (2016) DOI:10.1111/cge.12773 ... ...Taylor JC, Kini U ... TITLE ... A de novo frameshift in HNRNPK causing a Kabuki-lik

  1. Aplasia cutis congenita, skull defect, brain heterotopia, and intestinal lymphangiectasia

    NARCIS (Netherlands)

    Bonioli, Eugenio; Hennekam, Raoul C.; Spena, Gianantonio; Morcaldi, Guido; Di Stefano, Antonio; Serra, Giovanni; Bellini, Carlo

    2005-01-01

    We describe a female infant with a previously unreported combination of manifestations characterized by aplasia cutis, skull defect, brain heterotopia, mild congenital lymphedema, and intestinal lymphangiectasia. The association of intestinal lymphangiectasia and aplasia cutis, and the association

  2. Radiology of nodular lesions of the lung parenchyma

    International Nuclear Information System (INIS)

    Meschan, I.; Pugatch, R.D.

    1987-01-01

    The authors have arbitrarily classified these nodular lesions as (1) single, (2) coarsely nodular throughout one or both lungs, and (3) finely granular and irregularly or homogeneously distributed throughout both lungs. They have further classified pulmonary and nodular lesions as being most likely single as against those that may be either single or multiple throughout the lung fields

  3. [Nodular gastritis and gastric cancer in young adult].

    Science.gov (United States)

    Kamada, Tomoari; Shiotani, Akiko; Haruma, Ken

    2012-10-01

    Nodular gastritis is a popular endoscopic gastritis in H. pylori-positive children and young adults. The endoscopic findings of nodular gastritis were mainly characterized by a unique, small granulated pattern in the antrum of the stomach. The cases of gastric cancer with nodular gastritis showed the same characteristics: all were diagnosed histologically as the diffuse-type and were located in the corpus with H. pylori infection. We recommended that endoscopists should carefully examine not only the antrum but also the corpus in patients with nodular gastritis, and H. pylori should be eradicated as soon as possible to prevent gastric cancer.

  4. Heterotopia on Screen- Blue Velvet (1986

    Directory of Open Access Journals (Sweden)

    Filimon Eliza Claudia

    2014-03-01

    Full Text Available Based on a framework consisting of postmodern theories of heterotopias, spatial pastiche, schizophrenic temporality and postmodern speed, this paper seeks to identify cinematic features in the works of the American director David Lynch, which exemplify time and space in postmodernism. Michel Foucault's theory of space will trigger the whole problematic of the time-space relation. This is followed by a discussion of Fredric Jameson's concepts of spatial pastiche and schizophrenic temporality and of the involute interaction between the two

  5. Syariah as Heterotopia: Responses from Muslim Women in Aceh, Indonesia

    Directory of Open Access Journals (Sweden)

    Reed Taylor

    2015-05-01

    Full Text Available In this paper, I argue that the implementation of syariah is best understood as a heterotopia by women in Aceh, Indonesia. The current debates over the role of syariah for women in Acehnese society focus on either a secular human rights critique of non-liberal norms that restrict the rights and freedom of women or a religiously prescribed defense of communal norms that protect women and society. Based on interviews, I identify three variants of how women conceive of and inhabit syariah in Aceh. Two of these variants are underrepresented in the current academic literature on syariah in Aceh. Two key distinctions are drawn between blueprint and iconoclastic utopian thought and state-centric and non-state-centric models of political legitimacy. Rethinking syariah as a “socio-spatial dialectic” allows for all three variants of syariah existing simultaneously as a heterotopia in Acehnese society.

  6. Pigmented Nodular Basal Cell Carcinomas in Differential Diagnosis with Nodular Melanomas: Confocal Microscopy as a Reliable Tool for In Vivo Histologic Diagnosis

    International Nuclear Information System (INIS)

    Casari, A.; Pellacani, G.; Seidenari, S.; Pepe, P.; Longo, C.; Cesinaro, A. M.; Beretti, F.

    2011-01-01

    Nodular basal cell carcinoma, especially when pigmented, can be in differential diagnosis with nodular melanomas, clinically and dermoscopically. Reflectance confocal microscopy is a relatively new imaging technique that permits to evaluate in vivo skin tumors with a nearly histological resolution. Here, we present four cases of challenging nodular lesions where confocal microscopy was able to clarify the diagnosis.

  7. Regeneration of 5-HT fibers in hippocampal heterotopia of methylazoxymethanol-induced micrencephalic rats after neonatal 5,7-DHT injection.

    Science.gov (United States)

    Nakamura, Arata; Kadowaki, Taro; Sakakibara, Shin-ichi; Yoshimoto, Kanji; Hirata, Koichi; Ueda, Shuichi

    2010-03-01

    In order to elucidate the regeneration properties of serotonergic fibers in the hippocampus of methylazoxymethanol acetate (MAM)-induced micrencephalic rats (MAM rats), we examined serotonergic regeneration in the hippocampus following neonatal intracisternal 5,7-dihydroxytryptamine (5,7-DHT) injection. Prenatal exposure to MAM resulted in the formation of hippocampal heterotopia in the dorsal hippocampus. Immunohistochemical and neurochemical analyses revealed hyperinnervation of serotonergic fibers in the hippocampus of MAM rats. After neonatal 5,7-DHT injection, most serotonergic fibers in the hippocampus of 2-week-old MAM rats had degenerated, while a small number of serotonergic fibers in the stratum lacunosum-moleculare (SLM) of the hippocampus and in the hilus adjacent to the granular cell layer of the dentate gyrus (DG) had not. Regenerating serotonergic fibers from the SLM first extended terminals into the hippocampal heterotopia, then fibers from the hilus reinnervated the DG and some fibers extended to the heterotopia. These findings suggest that the hippocampal heterotopia exerts trophic target effects for regenerating serotonergic fibers in the developmental period in micrencephalic rats.

  8. Gastric heterotopia in the rectum. A rare cause of ectopic gastric tissue.

    Science.gov (United States)

    Salem, George A; Fazili, Javid; Ali, Tauseef

    2017-03-01

    Gastric heterotopia refers to the discovery of normal gastric tissue at foreign, unexpected sites. It has been described anywhere in the alimentary tract, even in the mediastinum, scrotum, and spinal cord. It is not uncommonly seen in the oesophagus or small intestine. However, large bowel lesions are rare, with the most common location of colonic lesions is the rectum. Although it is a rare entity, it may be the source for significant problems such as rectal bleeding, abdominal pain, deep rectal pain, and malignancy. Here, we report an additional case of gastric heterotopia in the rectum of a 56year old gentleman, and review the literature. Copyright © 2017 Pan-Arab Association of Gastroenterology. Published by Elsevier B.V. All rights reserved.

  9. Nasal glial heterotopia or congenital hemangioma? A case report.

    Science.gov (United States)

    Lartizien, R; Durand, C; Blaise, S; Morand, B

    2017-10-01

    Nasal glial heterotopia (NGH) is a rare benign tumor of the median line. We describe the case of a child presenting a lateral nasal mass. The characteristics of the prenatal ultrasound and the postnatal clinical examination argued in favor of a congenital hemangioma (CH). The MRI performed at 6 weeks of life suggested glial heterotopia. This diagnosis was confirmed by the pathological analysis. Congenital hemangiomas and nasal glial heterotopies have similar clinical presentations. Prenatal ultrasound diagnosis between NGH and CH is difficult. Fetal MRI is not yet highly specific for these two lesions, but it can eliminate an intracerebral connection in cases of NGH. Postnatal exams are more specific. Flow on the Doppler exam is rapid for CH and slow for NGH. On MRI, these two lesions appear as a hypersignal on T2-weighted sequences, but less intense for NGH than for CH. Distinguishing between NGH and CH can be difficult. This does not have a direct incidence on treatment because it is surgical in both cases. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  10. A Heterotopology of the Academy: Mapping Assemblages as Possibilised Heterotopias

    Science.gov (United States)

    Charteris, Jennifer; Jones, Marguerite; Nye, Adele; Reyes, Vicente

    2017-01-01

    Heterotopias are counter-sites of enacted utopias through which reality is simultaneously represented, contested and inverted. They are physical or mental spaces where, although norms of behaviours are suspended, there are connections with a plethora of other spaces. This article constructs a collective biography as a heterotopology of the…

  11. Parapharyngeal neuroglial heterotopia appearing as high uptake on 18F-FDG PET: case report and literature review of radiographical findings.

    Science.gov (United States)

    Kameyama, Masayuki; Kawaguchi, Tomohiro; Niizuma, Hidetaka; Ogawa, Takenori; Watanabe, Kenichi; Hayashi, Toshiaki; Sato, Kanako; Kanamori, Masayuki; Watanabe, Mika; Katori, Yukio; Kure, Shigeo; Tominaga, Teiji

    2018-04-01

    Parapharyngeal neuroglial heterotopia is a rare entity, and the specific radiographical findings are unclear. We present a case of parapharyngeal neuroglial heterotopia examined with proton magnetic resonance spectroscopy ( 1 H-MRS) and 18 F-fluorodesoxyglucose positron emission tomography ( 18 F-FDG PET). Our neonate patient presented with neck mass and polyhydramnios during gestation. Computed tomography and magnetic resonance imaging demonstrated the morphological characteristics, but failed to establish the diagnosis. 1 H-MRS showed a non-malignant pattern, but 18 F-FDG PET demonstrated high glucose metabolism. Complete resection was achieved and the histopathological diagnosis was neuroglial heterotopia. Assessment of biological activity may be useful for both preoperative diagnosis and postoperative evaluation of residual lesions.

  12. Complete pancreatic heterotopia of gallbladder with hypertrophic duct simulating an adenomyoma

    Science.gov (United States)

    Pilloni, Luca; Cois, Alessandro; Uccheddu, Alessandro; Ambu, Rossano; Coni, Pierpaolo; Faa, Gavino

    2006-01-01

    The gallbladder is an unusual location of pancreatic heterotopia, defined as the presence of pancreatic tissue lacking anatomical and vascular continuity with the main body of the gland. A 28-year-old man presented with anorexia, nausea and pain in the right upper abdomen. On physical examination, the abdomen was tender to palpation and Murphy sign was positive. The patient underwent a cholecystecomy. This case, in our opinion, is very interesting since it permits to consider a controversial issue in the pathology of the gallbladder. The histological appearance of ductal structure in pancreatic heterotopia resembles the histological picture of both Aschoff-Rokitansky (AR) sinuses and adenomyomas. This finding suggests that these lesions are linked by a common histogenetic origin. We suggest that the finding of an adenomyoma in the gallbladder should prompt an extensive sampling of the organ in order to verify the coexistence of pancreatic rests. PMID:16586554

  13. Carcinoma thyroid in multi and uni nodular goiter

    International Nuclear Information System (INIS)

    Iqbal, M.; Mehmood, Z.; Rasul, S.; Inamullah, S.; Sagheer, H.; Bokhari, I.

    2010-01-01

    To determine the frequency and profile of carcinoma in multi-nodular goiter and solitary thyroid nodule. Study Design: Case series. Place and Duration of Study: Surgical Unit-I, Ward-3 of Jinnah Postgraduate Medical Centre, Karachi, from January 1999 to January 2009. Methodology: Cases with solitary thyroid nodules and multi-nodular goiter were included. Patients under 12 years of age, cystic benign lesion in solitary thyroid nodules or those multi-nodular goiters which were not causing pressure symptoms, cosmetic problems or sign of malignancy were excluded. In solitary thyroid nodule, hemithyroidectomy was done and if histopathology examination revealed carcinoma thyroid then completion thyroidectomy was done. In multi-nodular goiter sub-total thyroidectomy done. Results were described as frequency percentages and mean. Results: Out of 397 patients of multi-nodular goiter only one patient was found to be papillary carcinoma (0.25%). In 220 patients of solitary thyroid nodules, 93 patients were diagnosed as carcinoma of thyroid (42.27%). Others diagnosed in solitary thyroid nodule were thyroid adenoma, colloid goiter, thyroiditis and multi-nodular goiter. The frequency of papillary carcinoma in 65.95% occurring females of 12-30 years of age and being multifocal in 6.45%, follicular carcinoma in 23.40%, medullary carcinoma in 7.44%, anaplastic carcinoma in 2.12% and lymphoma in 1.01%. Female were predominantly involved and papillary carcinoma was common in 12-30 years of age (71.63%) and follicular was common in 30-40 years of age (68.18%). 6.45% of papillary carcinoma was found to be multifocal in nature. Conclusion: Frequency of carcinoma of thyroid is very high in solitary thyroid nodule (42.27%), but markedly low in multi nodular goiter. Papillary carcinoma is the most common variety, most of in younger female. (author)

  14. Movement disorder and neuronal migration disorder due to ARFGEF2 mutation

    NARCIS (Netherlands)

    M.C.Y. de Wit (Marie Claire); I.F.M. de Coo (René); D. Halley (Dicky); M. Leguin (Maarten); G.M.S. Mancini (Grazia)

    2009-01-01

    textabstractWe report a child with a severe choreadystonic movement disorder, bilateral periventricular nodular heterotopia (BPNH), and secondary microcephaly based on compound heterozygosity for two new ARFGEF2 mutations (c.2031_2038dup and c.3798_3802del), changing the limited knowledge about the

  15. What are the clinical implications of nodular gastritis? Clues from histopathology.

    Science.gov (United States)

    Sokmensuer, Cenk; Onal, Ibrahim Koral; Yeniova, Ozgur; Ersoy, Osman; Aydinli, Musa; Yonem, Ozlem; Harmanci, Ozgur; Onal, Eda Demir; Altinok, Gulcin; Batman, Figen; Bayraktar, Yusuf

    2009-10-01

    There is no widely accepted histopathological definition for nodular gastritis. In this study we aim to uncover the pathologic entity responsible for the nodular appearance and to find clues about the clinical implications of nodular gastritis. Antral biopsy specimens of 160 patients with nodular gastritis and 133 patients without nodular gastritis were examined by an experienced pathologist for dysplasia, foveolar hyperplasia, inflammatory activity, intraepithelial lymphocytosis, intestinal metaplasia, and lymphoid follicle/aggregate formation, and comparative analysis was performed between the two groups of patients. The presence of intraepithelial lymphocytosis was more frequent in patients with nodular gastritis (P < 0.05). There was no difference between the two groups regarding the other pathological features such as presence of dysplasia, inflammatory activity, intestinal metaplasia, lymphoid hyperplasia, and Helicobacter pylori (H. pylori) infection. Increase of intraepithelial lymphocytes may contribute to formation of macroscopical nodules in this peculiar type of gastritis. Nodular gastritis would not indicate a new therapeutic approach in addition to the current measures for Helicobacter pylori infection.

  16. Nodular fasciitis: MRI appearance and literature review

    International Nuclear Information System (INIS)

    Leung, L.Y.J.; Shu, S.J.; Chan, M.K.; Chan, C.H.S.; Chan, A.C.L.

    2002-01-01

    Objective: To describe the MRI features of nodular fasciitis and to review the clinical, MRI and histologic aspects of the tumor. Design and patients: Three patients with biopsy-proven nodular fasciitis were selected for a retrospective study. A literature review was also carried out. Results and conclusions: All the lesions appeared slightly hyperintense to skeletal muscle on T1-weighted images, and hyperintense on T2-weighted images with fat saturation [either frequency saturation or Short TI Inversion Recovery (STIR) sequences]. Two enhanced homogeneously after intravenous gadolinium, whereas the third showed heterogeneous enhancement with a nonenhancing area. Despite the difference in enhancing patterns, the histologic appearances of these lesions were similar. Our study shows that the MRI appearance of nodular fasciitis may not be related to the location of lesion. It is thought that the age of nodular fasciitis may reflect its gross morphology, and it is possible that the MRI and histologic appearances could correlate with the age of the lesion, but it would require a larger series to evaluate this concept. (orig.)

  17. Nodular type of muscular sarcoidosis : a case report

    International Nuclear Information System (INIS)

    Chae, Soo Hyun; Kim, Hong Soo; Park, Sook Hee; Kim, Sung Me

    1999-01-01

    Muscular involvement of sarcoidosis is rare and occurs in two forms:nodular and myopathic. In the nodular variety, lesions are long and extend along muscle fibers. Axial MR imaging reveals a star-shaped central structure of decreased signal intensity. Sagittal and coronal MR images show three stripes:an inner stripe of decreased signal intensity and outer stripes of increased signal intensity. Longitudinal sonography shows an echogenic inner stripe and hypoechoic outer stripes. We report a case of nodular- type muscular sarcoidosis in a 53-year-old man, describing the findings of MRI and ultrasonography

  18. Scleroderma with Nodular Scleroderma

    Directory of Open Access Journals (Sweden)

    Chutika Srisuttiyakorn

    2016-11-01

    Full Text Available Background: Nodular scleroderma is a rare variant of scleroderma which can occur in connection with systemic sclerosis or morphea. A biopsy from the lesion can demonstrate the scleroderma pattern, i.e., keloid pattern or mixed type. Treatment is challenging, and several treatments modalities have been reported with unsatisfactory results. Main Observations: We present a case of systemic sclerosis in a 50-year-old female who developed nodular scleroderma in the absence of deterioration of the scleroderma condition. Although no additional treatment was given, the lesions remained stable without progression. Conclusions: Although this condition is rare, it has been reported sporadically, and clinicians should be able to recognize this variant in cases of scleroderma presenting with firm nodules or plaques.

  19. Proliferation and cell death in an experimental model of brain tissue heterotopia in the lung Proliferação e morte celular na heterotopia encefálica experimental

    Directory of Open Access Journals (Sweden)

    Paulo Roberto Veiga Quemelo

    2010-08-01

    Full Text Available PURPOSE: To investigate the proliferation and neuronal death in brain tissue heterotopia in the lung in an experimental model during both fetal and neonatal periods. METHODS: Twenty four pregnant female Swiss mice were used to induce brain tissue heterotopia on the 15th gestational day. Briefly, the brain of one fetus of each dam was extracted, disaggregated and injected into the right hemithorax of siblings. Six of these fetuses with pulmonary brain tissue implantation (PBI were collected on the 18th gestational day (group E18 and six other on the 8th postnatal day (group P8. Immunohistochemical staining for PCNA and Bcl2 were used to assess proliferation and cell death. RESULTS: PCNA Labelling Index (LI in heterotopic brain tissue was greater in fetal than postnatal period (E18 > P8 (pOBJETIVO: Investigar a proliferação e morte neuronal na heterotopia encefálica pulmonar em modelo experimental durante o período fetal e neonatal. MÉTODOS: Foram utilizados 24 camundongos Swiss fêmeas prenhes para induzir a heterotopia encefálica no pulmão. O tecido encefálico de um feto de cada fêmea prenha foi removido, picotado e injetado no pulmão dos irmãos. Seis fetos com Implantação Encefálica Pulmonar (IEP foram coletados no 18º dia gestacional (grupo E18 e seis outros fetos no 8º dia pós-natal (grupo P8. Foi realizada a reação Imuno-histoquímica para PCNA e Bcl2 para analisar a proliferação e morte celular. RESULTADOS: O índice de marcação (IM para PCNA era maior no período fetal quando comparado com o período pós-natal (E8 > P18 (p<0,05 e a imunomarcação para o anticorpo Bcl2 não apresentou diferença. CONCLUSÃO: A proliferação celular foi mantida no tecido heterotópico encefálico, embora a apoptose também foi observada.

  20. Heterotopia and Its Role in the Lived Experiences of Resettlement

    Science.gov (United States)

    Rossetto, Marietta

    2006-01-01

    Place, as a metaphor, can be experienced in different ways, existing or created. If created, space can be Foucault's "placeless place", a utopia. A place that exists, however, can be a heterotopic space. A heterotopia is what we as individuals interpret it to be: it can be a space for reconstituting the self, rewriting the scripts of…

  1. [Nasal glial heterotopia: Clinical and morphological characteristics].

    Science.gov (United States)

    Bykova, V P; Bakhtin, A A; Polyakov, D P; Yunusov, A S; Daikhes, N A

    The paper describes a case of nasal glial heterotopia in a 10-month-old girl with a mixed (intranasal and subcutaneous) localization, which is accompanied by the divergence of the nasal bones. Histological examination supplemented by immunohistochemical reactions with antibodies to vimentin, S100 protein, neuron-specific enolase, as well as Ki-67 and smooth muscle actin confirmed the neural nature of the tumor. Fields of mature astrocytic glia including individual cells with neuronal differentiation were found among the fibrous and fibrovascular tissues. The paper provides a brief overview of the discussed pathology.

  2. Current status of studies on nodular corrosion

    International Nuclear Information System (INIS)

    Yasuda, Takayoshi; Kawasaki, Satoru; Echigoya, Hironori; Kinoshita, Yutaka; Kubota, Hiroyuki; Konishi, Takao; Yamanaka, Tuneyasu.

    1993-01-01

    The studies on nodular corrosion formed on the outer surface of BWR fuel cladding tubes were reviewed. Main factors affecting the corrosion behavior were material and environmental conditions and combined effect. The effects of such material conditions as fabrication process, alloy elements, texture and surface treatment and environmental factors as neutron irradiation, thermo-hydrodynamic, water chemistry, purity of the coolant and contact with foreign metals on the corrosion phenomena were surveyed. Out-of-reactor corrosion test methods and models for the corrosion mechanism were also reviewed. Suppression of the accumulated annealing temperature during tube reduction process improved the nodular corrosion resistance of Zircaloys. Improved resistance for the nodular corrosion was reported for the unirradiated Zircaloys with some additives. Detailed irradiation test under the BWR conditions is needed to confirm the trend. Concerning the environmental factors, boiling on the cladding surface due to heat flux reduces the nodular corrosion susceptibility, while oxidizing radical generated from dissolved oxygen accelerates the corrosion. Concerning corrosion mechanisms, importance of such phenomena as the depleted zone of alloying elements in zirconium matrix, reduction of H + to H 2 in oxide layer, electrochemical property of precipitates, crystallographic anisotropy of oxidation rates were revealed. (author) 59 refs

  3. Nodular Morphea

    OpenAIRE

    Kauer, Friederike; Simon, Jan C.; Sticherling, Michael

    2013-01-01

    Scleroderma may present as being strictly limited to the skin, as in morphea, or within a multiorgan disease, as in systemic sclerosis. Accordingly, cutaneous manifestations vary clinically. In nodular or keloidal scleroderma, patients develop lesions that are clinically indistinguishable from a keloid; however, the histopathological findings are more variable. We describe a 16-year-old girl with morpheic lesions for 3–4 years and additional development of keloidal nodules within these lesion...

  4. Long segment ileal duplication with extensive gastric heterotopia

    Directory of Open Access Journals (Sweden)

    Jacob Sunitha

    2009-07-01

    Full Text Available Duplications of the alimentary tract are rare congenital anomalies which can be found at all levels of the alimentary tract. Majority of the duplications present as spherical cysts and usually range from a few millimeters to less than ten centimeters in size. Duplications produce complications such as intestinal obstruction or hemorrhage. A two-month-old infant presented with recurrent episodes of bleeding per rectum. Laparotomy revealed a giant ileal duplicated bowel segment which exhibited extensive gastric heterotopia with focal ulceration.

  5. Nodular gastritis: an endoscopic indicator of Helicobacter pylori infection in children.

    Science.gov (United States)

    Mazigh Mrad, Sonia; Abidi, Kamel; Brini, Ines; Boukthir, Samir; Sammoud, Azza

    2012-11-01

    Helicobacter pylori (Hp) infection is a common and universally distributed bacterial infection. It is predominantly acquired in childhood. To assess the relationship between endoscopic nodular gastritis and Hp infection. A retrospective study was conducted in children who underwent upper gastrointestinal endoscopy for chronic abdominal pain. All children who had five gastric biopsies for histologic analysis, urease test and Hp culture were included in the study. The Sensitivity and sensibility of nodular gastritis were determined and different parameters were studied according the presence or not of nodular gastritis(age, gender, Hp status, gastritis score and lymphoid follicles). 49 children, mean age 6.9 ± 3 years (range 1-12 years) were eligible for the analysis. Hp Infection was diagnosed in 35 out of the 49 children (71.5 %). Nodular gastritis was recorded in 16 out of 49 children and in 14 infected children and 2 out of 14 non infected children (p=0.07), 40% sensitivity, 85.7% specificity, 87.5% positive predictive value, and 36,4% negative predictive value. The parameters associated significantly to the presence of nodular gastritis were older age: (p=0.04), Hp infection: (p=0.01), chronic gastritis: (p=0.05), active gastritis: (p=0.02), follicular gastritis: (p=0.005), higher gastritis score: (p=0.005). Completely normal gastric mucosal histology was never found in infected children with antral nodularity. Among all infected children, the gastritis score was significantly higher when there was a nodular gastritis and follicular gastritis was significantly associated to nodular gastritis. The endoscopic pattern of antral nodularity identifies children with Hp infection, and active chronic follicular gastritis.

  6. Diffusion Tensor Imaging of Heterotopia: Changes of Fractional Anisotropy during Radial Migration of Neurons

    Science.gov (United States)

    Kim, Jinna

    2010-01-01

    Purpose Diffusion tensor imaging provides better understanding of pathophysiology of congenital anomalies, involving central nervous system. This study was aimed to specify the pathogenetic mechanism of heterotopia, proved by diffusion tensor imaging, and establish new findings of heterotopia on fractional anisotropy maps. Materials and Methods Diffusion-weighted imaging data from 11 patients (M : F = 7 : 4, aged from 1 to 22 years, mean = 12.3 years) who visited the epilepsy clinic and received a routine seizure protocol MRI exam were retrospectively analyzed. Fractional anisotropy (FA) maps were generated from diffusion tensor imaging of 11 patients with heterotopia. Regions of interests (ROI) were placed in cerebral cortex, heterotopic gray matter and deep gray matter, including putamen. ANOVA analysis was performed for comparison of different gray matter tissues. Results Heterotopic gray matter showed signal intensities similar to normal gray matter on T1 and T2 weighted MRI. The measured FA of heterotopic gray matter was higher than that of cortical gray matter (0.236 ± 0.011 vs. 0.169 ± 0.015, p < 0.01, one way ANOVA), and slightly lower than that of deep gray matter (0.236 ± 0.011 vs. 0.259 ± 0.016, p < 0.01). Conclusion Increased FA of heterotopic gray matter suggests arrested neuron during radial migration and provides better understanding of neurodevelopment. PMID:20499428

  7. Nodular Lymphangitis (Sporotrichoid Lymphocutaneous Infections. Clues to Differential Diagnosis

    Directory of Open Access Journals (Sweden)

    Andrés Tirado-Sánchez

    2018-05-01

    Full Text Available Nodular lymphangitis, also known as sporotrichoid lymphocutaneous infections, is characterized by suppurative inflammatory nodules along the lymphatic vessels. This manifestation is classic of sporotrichosis, however, other infections such as nocardiosis, atypical mycobacteriosis, leishmaniasis, among others, can also express this clinical pattern. Sporotrichosis, which often occurs in gardeners, remains the most recognized cause of nodular lymphangitis. The histopathological studies, as well as the culture are diagnostic standards of lesions that do not respond to empirical treatment. In this article, we will review the main causes of nodular lymphangitis or lymphocutaneous sporotrichoid infections.

  8. Nodular Fasciitis of External Auditory Canal

    Directory of Open Access Journals (Sweden)

    Jihyun Ahn

    2016-09-01

    Full Text Available Nodular fasciitis is a pseudosarcomatous reactive process composed of fibroblasts and myofibroblasts, and it is most common in the upper extremities. Nodular fasciitis of the external auditory canal is rare. To the best of our knowledge, less than 20 cases have been reported to date. We present a case of nodular fasciitis arising in the cartilaginous part of the external auditory canal. A 19-year-old man complained of an auricular mass with pruritus. Computed tomography showed a 1.7 cm sized soft tissue mass in the right external auditory canal, and total excision was performed. Histologic examination revealed spindle or stellate cells proliferation in a fascicular and storiform pattern. Lymphoid cells and erythrocytes were intermixed with tumor cells. The stroma was myxoid to hyalinized with a few microcysts. The tumor cells were immunoreactive for smooth muscle actin, but not for desmin, caldesmon, CD34, S-100, anaplastic lymphoma kinase, and cytokeratin. The patient has been doing well during the 1 year follow-up period.

  9. [Pancreatic serous cystadenoma associated with pancreatic heterotopia].

    Science.gov (United States)

    Mohamed, Hedfi; Dorra, Belghachem; Hela, Bouhafa; Cherif, Abdelhedi; Azza, Sridi; Karim, Sassi; Khadija, Bellil; Adnen, Chouchene

    2016-01-01

    Pancreatic heterotopias (HP) are rare. They can occur at any age with a slight male predominance. These lesions are usually asymptomatic and they are often found incidentally during upper or lower GI endoscopy or during the anatomo-pathological examination of an organ which was resected for other reasons; they can be isolated or associated with a digestive pathology. We report, through observation, the association of HP with serous cystadenoma of the pancreas discovered during examinations to identify the etiology of isolated abdominal pain. The aim of this study is to analyse clinical and histological features of this rare pathology.

  10. IMAGING DIAGNOSIS-MAGNETIC RESONANCE IMAGING OF A NEURONAL HETEROTOPIA IN THE BRAIN OF A CAT.

    Science.gov (United States)

    DeJesus, Antonia; Turek, Bradley J; Galban, Evelyn; Suran, Jantra Ngosuwan

    2018-03-01

    A domestic shorthair kitten was presented for evaluation and further treatment of seizures. Magnetic resonance imaging of the brain revealed a large multilobulated mass in the third ventricle extending into the right lateral ventricle with secondary obstructive hydrocephalus. The mass was homogeneously isointense to gray matter on T2W, T2-FLAIR, T2 * W, T1W, and ADC images, and hyperintense on DW-EPI. There was no appreciable contrast enhancement. Seizures were managed medically and with subsequent ventriculoperitoneal shunt placement. Clinical status later deteriorated and the cat was euthanized. Histopathology confirmed that the mass was the result of neuronal heterotopia. To the authors' knowledge this is the first report of neuronal heterotopia in a cat. © 2016 American College of Veterinary Radiology.

  11. Subcortical Band Heterotopia (SBH) in Rat Offspring Following Maternal Hypothyroxinemia: Structural and Functional Characteristics

    Science.gov (United States)

    Thyroid hormones (TH) play crucial roles in brain maturation, neuronal migration, and neocortical lamination. Subcortical band heterotopia (SBH) represent a class of neuronal migration errors in humans that are often associated with childhood epilepsy. We have previously reported...

  12. Disturbances in the positioning, proliferation, and apoptosis of neural progenitors contribute to subcortical band heterotopia formation

    Science.gov (United States)

    Fitzgerald, MP; Covio, M; Lee, KS

    2011-01-01

    Cortical malformations are commonly associated with intractable epilepsy and other developmental disorders. Our studies utilize the tish rat, a spontaneously occurring genetic model of subcortical band heterotopia (SBH) associated with epilepsy, to evaluate the developmental events underlying SBH formation in the neocortex. Our results demonstrate that Pax6+ and Tbr2+ progenitors are mislocalized in tish+/− and tish−/− neocortex throughout neurogenesis. In addition, mislocalized tish−/− progenitors possess a longer cell cycle than wildtype or normally-positioned tish−/− progenitors, owing to a lengthened G2+M+G1 time. This mislocalization is not associated with adherens junction breakdown or loss of radial glial polarity in the ventricular zone, as assessed by immunohistochemistry against phalloidin (to identify F-actin), aPKC-λ, and Par3. However, vimentin immunohistochemistry indicates that the radial glial scaffold is disrupted in the region of the tish−/− heterotopia. Moreover, lineage tracing experiments using in utero electroporation in tish−/− neocortex demonstrate that mislocalized progenitors do not retain contact with the ventricular surface and that ventricular/subventricular zone progenitors produce neurons that migrate into both the heterotopia and cortical plate. Taken together, these findings define a series of developmental errors contributing to SBH formation that differs fundamentally from a primary error in neuronal migration. PMID:21145942

  13. Medulloblastoma with Excessive Nodularity: Radiographic Features and Pathologic Correlate

    Directory of Open Access Journals (Sweden)

    L. A. Yeh-Nayre

    2012-01-01

    Full Text Available Medulloblastoma with extensive nodularity is a rare subtype of the most common malignant childhood brain tumor and has been associated with more favorable prognosis. The authors report the case of a 10-month-old girl with a posterior fossa tumor of excessive nodularity with decreased diffusivity on diffusion-weighted magnetic resonance imaging sequences and robust grape-like postgadolinium contrast enhancing features. The unique neuroradiographic features were confirmed by histopathology and a diagnosis of medulloblastoma with extensive nodularity was made. This case highlights the importance of recognizing this unique medulloblastoma subtype preoperatively, as the more favorable outcome may preclude less aggressive medical management.

  14. A series of parapharyngeal glial heterotopia mimicking lymphatic malformation.

    Science.gov (United States)

    Haloob, Nora; Pepper, Christopher; Hartley, Benjamin

    2015-12-01

    Otolaryngologists will most frequently encounter extra-cranial glial tissue within the nasal cavity, where it is known as a 'nasal glioma', and may communicate with the dura. However, glial tissue can also present extra-nasally in the form of a neck mass with no intracranial connection. In these rare cases, they can present soon after birth as an enlarging neck mass, causing compressive symptoms with airway obstruction and feeding difficulties. In this way, it is often initially misdiagnosed as a more common lesion such as a lymphatic malformation, teratoma, branchial anomaly or vascular malformation. As with many congenital head and neck masses, offering the most the appropriate management relies heavily on radiological imaging and, where possible, histopathology from a diagnostic biopsy. Once the diagnosis of extra-nasal glial heterotopia has been confirmed, the gold standard management is complete surgical excision. We review three cases of extra-nasal glial heterotopia presenting to our institution over an eleven year period as a large neck mass, which mimicked other congenital neck lumps, and discuss them in the context of those in the literature. We highlight how their clinical and radiological features can easily be confused with lymphatic malformations, and the potential implications of misdiagnosis. Raising awareness of this diagnostic confusion will highlight the need for management of these cases within an appropriate paediatric multidisciplinary setting. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  15. Medulloblastoma with extensive nodularity: US, CT and MRI findings

    International Nuclear Information System (INIS)

    Bekiesinska-Figatowska, M.; Uliasz, M.; Roszkowski, M.

    2008-01-01

    Medulloblastoma accounts for up to 25% of all paediatric CNS tumours. According to WHO classification (2007) medulloblastoma with extensive nodularity (MBEN) is a separate rare entity associated with younger age and better prognosis. A 9-month-old girl was admitted and examined because of macrocephaly and disturbed psychomotor development. Transfontanel ultrasound revealed dilated ventricular system and hyperechoic mass in the posterior cranial fossa. Computed tomography showed hyperdense mass in the cerebellum. Magnetic resonance imaging revealed a mass with gyriform pattern and strong contrast enhancement after gadolinium administration. Differential diagnosis included dysplastic heterotopic cortex, Lhermitte-Duclos disease, atypical teratoid/rhabdoid tumour (AT/RT), and medulloblastoma (MB). The patient was operated on. Medulloblastoma with extensive nodularity (MBEN) was finally diagnosed. The authors present and discuss three other cases of this rare entity.Transfontanel sonografic examination is capable of detection of the posterior fossa tumour as a cause of hydrocephalus and macrocephaly. The mass in a child's posterior cranial fossa that is hyperdense on unenhanced CT and gyriform, nodular, and markedly enhancing on MRI may strongly suggest medulloblastoma with extensive nodularity (MBEN). (authors)

  16. Nodular smooth muscle metaplasia in multiple peritoneal endometriosis.

    Science.gov (United States)

    Kim, Hyun-Soo; Yoon, Gun; Ha, Sang Yun; Song, Sang Yong

    2015-01-01

    We report here an unusual presentation of peritoneal endometriosis with smooth muscle metaplasia as multiple protruding masses on the lateral pelvic wall. Smooth muscle metaplasia is a common finding in rectovaginal endometriosis, whereas in peritoneal endometriosis, smooth muscle metaplasia is uncommon and its nodular presentation on the pelvic wall is even rarer. To the best of our knowledge, this is the first case of nodular smooth muscle metaplasia occurring in peritoneal endometriosis. As observed in this case, when performing laparoscopic surgery in order to excise malignant tumors of intra-abdominal or pelvic organs, it can be difficult for surgeons to distinguish the metastatic tumors from benign nodular pelvic wall lesions, including endometriosis, based on the gross findings only. Therefore, an intraoperative frozen section biopsy of the pelvic wall nodules should be performed to evaluate the peritoneal involvement by malignant tumors. Moreover, this report implies that peritoneal endometriosis, as well as rectovaginal endometriosis, can clinically present as nodular lesions if obvious smooth muscle metaplasia is present. The pathological investigation of smooth muscle cells in peritoneal lesions can contribute not only to the precise diagnosis but also to the structure and function of smooth muscle cells and related cells involved in the histogenesis of peritoneal endometriosis.

  17. Nodular Fasciitis of the Breast Mimicking Breast Cancer

    Directory of Open Access Journals (Sweden)

    Shinya Yamamoto

    2014-01-01

    Full Text Available Nodular fasciitis is a benign proliferative lesion that is usually found in the soft tissue of the upper extremity and trunk in young to middle-aged persons. It has rarely been described in the breast. A 35-year-old woman had noticed a mass in her left breast. It was elastic-hard, 13 mm in size, and located mainly in the upper inner quadrant of the left breast. Mammography did not detect the mass. Ultrasonography revealed a hypoechoic lesion with an irregular margin. Neither fine-needle aspiration cytology nor core needle biopsy established a definitive diagnosis. Excisional biopsy was therefore performed. Histologically, the excised tumor tissue results were consistent with a diagnosis of nodular fasciitis of the breast. We report a case of nodular fasciitis of the breast, a rare histological type of breast tumor.

  18. Nodular Fasciitis of the Auricle: A Case Report

    Directory of Open Access Journals (Sweden)

    Mohammad Reza Majidi

    2013-03-01

    Full Text Available Introduction: Nodular fasciitis is described as a benign reactive proliferation of myofibroblasts. Due to its rapid-growing nature, a precise clinical diagnosis is difficult and the condition is frequently misdiagnosed as malignant lesions.   Case Report: In this study, we present the case of a young woman with an auricular nodular fasciitis as an example of one of the rarest sites of this tumor. The patient underwent an excision of the lesion under general anesthesia. The literature choices for treatment include complete excision, partial excision, or intralesional injection of steroids. Due to its associated local discomfort, and in order to exclude other differential diagnosis, we recommend a complete surgical excision.   Conclusion:  Auricular nodular fasciitis is a rare lesion. Due to its associated local discomfort, and in order to exclude other differential diagnosis, we recommend a complete surgical excision.

  19. STRUCTURE OF THE NODULAR GOITER IN CHILDREN

    Directory of Open Access Journals (Sweden)

    O. S. Rogova

    2015-01-01

    Full Text Available For today the question of early diagnosis of nosological variants, the method of treatment and prevention of nodular goiter in children is still not fully resolved. There is a few available literature data about study of this problem in children. Objective: to study morphofunctional structure of thyroid nodules in children.The article provides information consisting of the results of a retrospective analysis of case histories of 73 patients, operated with nodular goiter in Z.A. Bashlyaeva Children’sCityClinicalHospital(Moscow from 2003 to May 2015. The examination included an evaluation of the functional status of the pituitary-thyroid system: there were investigated serum thyroxine, triiodothyronine and thyroid stimulating hor-mone, also was performed thyroid scintigraphy 99mТc. In the postoperative period, there was carried out a final verification of the diagnosis according to the results of the histological conclusion. The histologi-cal type of nodal disease was defined by the WHO classification of 2004. It was established that in the structure of a single-node goiter in children prevail nodular colloid goiter with equal frequency in boys and girls, with the same frequency occurs thyroid adenoma, but more often in girls than in boys. The multinodular goiter equally often detected in girls and boys both nodular goiter colloidal with varying degree of proliferation and colloid in combination with follicular thyroid adenoma. "Hot" nodes in the multinodular goiter are more common than in the single-node goiter and more fre-quently in girls than in boys. The half of children with nodular pathology have "hot" nodes and one out of every three – decompensated form of functional autonomy. Decompensated form of functional autonomy of the thyroid gland in children with "hot" nodes detects regardless of the number of nodes. Papillary thyroid cancer occurs not only in patients with a single-node, but with a multinodular goiter. The results allow to

  20. Nodular smooth muscle metaplasia in multiple peritoneal endometriosis

    OpenAIRE

    Kim, Hyun-Soo; Yoon, Gun; Ha, Sang Yun; Song, Sang Yong

    2015-01-01

    We report here an unusual presentation of peritoneal endometriosis with smooth muscle metaplasia as multiple protruding masses on the lateral pelvic wall. Smooth muscle metaplasia is a common finding in rectovaginal endometriosis, whereas in peritoneal endometriosis, smooth muscle metaplasia is uncommon and its nodular presentation on the pelvic wall is even rarer. To the best of our knowledge, this is the first case of nodular smooth muscle metaplasia occurring in peritoneal endometriosis. A...

  1. The micro-mechanisms of failure of nodular cast iron

    Directory of Open Access Journals (Sweden)

    Alan Vaško

    2014-12-01

    Full Text Available The contribution deals with a comparison of the micro-mechanisms of failure of nodular cast irons at static, impact and fatigue stress. Several specimens of ferrite-pearlitic nodular cast irons with different content of ferrite in a matrix were used for metallographic analysis, mechanical tests and micro-fractographic analysis. Mechanical properties were found by static tensile test, impact bending test and fatigue tests. The micro-fractographic analysis was made with use of scanning electron microscope VEGA II LMU on fracture surfaces of the specimens fractured by these mechanical and fatigue tests. Fracture surfaces of analysed specimens are characteristic of mixed mode of fracture. Micro-mechanism of failure of nodular cast irons is dependent on the method of stress.

  2. Microstructural aspects of zircaloy nodular corrosion in steam

    International Nuclear Information System (INIS)

    Taylor, D.F.

    1999-01-01

    Zircaloy-2 becomes susceptible to nodular corrosion in high-temperature, high-pressure steam when the total solute concentration of the β-stabilizing alloying elements Fe, Ni and Cr in the α-zirconium matrix falls below a critical value C c that is characteristic of the test conditions. C c for typical commercial Zircaloy-2 in a 24hr/510 C/10.4MPa steam-test is the precipitate-free a-matrix concentration in equilibrium with solute-saturated β phase at about 840 C, the corresponding critical temperature T c .Thus, immunity to nodular corrosion is a metastable condition for α-Zircaloy that requires fast cooling from above T c to achieve adequate solute concentration throughout the matrix. Annealing Zircaloy at any temperature below T c for a sufficiently long time makes it susceptible to nodular corrosion. In the (α+χ) phase field, where χ collectively designates the Fe-, Cr-, and Ni-containing precipitate phases, lowering the solute concentration to less than C c by Ostwald ripening can require many hundreds of hours. Above about 825 C, the temperature of the (α+χ)/(α+β+χ) transus, solute-saturated β phase surrounds each precipitate and a strong inverse activity gradient promotes equilibration with the much lower solute concentration in the α matrix. Sensitization to nodular corrosion occurs most rapidly at about 835 C between the (α+χ)/(α+β+χ) transus and T c . Annealing Zircaloy at temperatures above T c for a sufficiently long time will raise the solute concentration above C c and, with rapid cooling, heal any degree of susceptibility. Annealing within the protective coarsening window between T c and about 850 C, the temperature of the (α+β+χ)/(α+β) transus, achieves rapid precipitate growth in a matrix immune to nodular corrosion

  3. A Rare Case of Medulloblastoma with Excessive Nodularity: Imagistic Features

    Directory of Open Access Journals (Sweden)

    Tascu A.

    2014-10-01

    Full Text Available Medulloblastoma is the most common malignant tumor of childhood. Neuroimaging can play a role in the diagnosis of medulloblastoma, however atypical features do exist [2]. We report the case of a 1 year and 10- month-old infant diagnosed with a medulloblastoma with what we term “excessive” nodularity based on neuroimaging features and confirmed by neuropathology. CT-scan (CT and magnetic resonance imaging (MRI examination of the brain revealed a very large posterior fossa tumor attached to tentorium. On T2-weighted and post-gadolinium sequences, the tumor shows an extensive nodular grape-like appearance. Initial the patient was underwent a ventriculo-peritoneal shunt. The second operative procedure was tumour resection. Histology examen revealed a diagnosis of medulloblastoma desmoplastic with extensive nodularity. The neuroradiographic features of this medulloblastoma with what we describe as “excessive” nodularity are important to recognize as these children may be cured with chemotherapy alone.

  4. Fatigue behaviour of synthetic nodular cast irons

    Directory of Open Access Journals (Sweden)

    A. Vaško

    2015-01-01

    Full Text Available The paper shows the influence of charge composition on microstructure, fatigue properties and failure micromechanisms of nodular cast irons. The additive of metallurgical silicon carbide (SiC in analysed specimens increases the content of ferrite in the matrix, decreases the size of graphite and increases the average count of graphitic nodules per unit of area. Consequently, the mechanical and fatigue properties of nodular cast iron are improved. The best fatigue properties (fatigue strength were reached in the melt which was created by 60 % of steel scrap and 40 % of pig iron in the basic charge with SiC additive.

  5. Nodular Scleroderma - Successful Treatment With Extracorporeal Photochemotherapy

    Directory of Open Access Journals (Sweden)

    Wollina U

    2001-01-01

    Full Text Available Nodular seleroderma is rare variant of circumscribed scleroderma (morphea. Treatment is often unsatisfactory. This report is on the use of extracorporeal photochemotherapy. A 12 year old girl and a 49 year old woman have been treated once a month on two consecutive days. A complete remission was achieved in one patient after 10 months and an almost complete remission in the young girl after 6 months. The treatment was well-tolerated and no severe side â€" effects occurred. In contrast to previous attempts in treating nodular scleroderma with different modalities, ECP seems to be an effective therapy.

  6. Nasopharyngeal glial heterotopia with delayed postoperative meningitis.

    Science.gov (United States)

    Maeda, Kenichi; Furuno, Kenji; Chong, Pin Fee; Morioka, Takato

    2017-06-22

    A male infant, who underwent radical resection of a large glial heterotopia at the nasopharynx at 8 days, developed delayed postoperative bacterial meningitis at 9 months. Neuroradiological examination clearly demonstrated that meningitis had occurred because of the intracranial and extracranial connections, which were scarcely seen in the perioperative period. A transsphenoidal extension of hypothalamic hamartoma is possible because the connection started from the right optic nerve, running through the transsphenoidal canal in the sphenoid bone and terminating at the recurrent mass in the nasopharyngeal region. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  7. Latinas, Heterotopia, and Home: Pedagogies of Gender and Sexuality in Quinceañera

    Science.gov (United States)

    Bondy, Jennifer M.

    2012-01-01

    In this article the author discusses Foucault's heterotopia analytic and applies it to a film analysis of Quinceañera. Engaging in feminist and critical media scholarship, the author outlines U.S. discourses on girlhood and their collusion with patriarchal and heteronormative discourses on Latino cultural nationalism in the regulation, control,…

  8. MRI Findings of Coexistence of Ectopic Neurohypophysis, Corpus Callosum Dysgenesis, and Periventricular Neuronal Heterotopia

    Directory of Open Access Journals (Sweden)

    Harun Arslan

    2014-01-01

    Full Text Available Ectopic neurohypophysis is a pituitary gland abnormality, which can accompany growth hormone deficiency associated with dwarfism. Here we present magnetic resonance imaging (MRI findings of a rare case of ectopic neurohypophysis, corpus callosum dysgenesis, and periventricular neuronal heterotopia coexisting, with a review of the literature.

  9. Duodenal nodularity in children: A clinical and pathologic study of 17 cases

    Directory of Open Access Journals (Sweden)

    Çaltepe Dinler Gönül

    2011-01-01

    Full Text Available Aims: Duodenal nodularity is an uncommon endoscopic appearance of numerous visible mucosal nodules in the proximal duodenum. In this retrospective study we aimed to determine the clinical significance and histopathologic features of duodenal nodularity in children. Materials and Methods: The medical records of the patients who were defined to have duodenal nodularity by endoscopy were reviewed. Statistical Analysis Used: The data were expressed as mean ± SD and percentages (%. Results: Seventeen patients with endoscopically defined duodenal nodularity were chosen. The mean age at diagnosis was 12.1 years (range: 6-17 years, 9 males. Abdominal pain (47% was the most common clinical symptom and antral nodularity (41% was the most common endoscopic finding in children with duodenal nodularity. Histopathologic evaluation of duodenal nodules revealed chronic inflammation in all patients, increased intercryptal and intraepithelial numbers of eosinophils in 70.5%, and villous atrophy in 47% of patients. Giardia infestation was demonstrated in 6 patients by histologic examination and/or Giardia lamblia-specific antigen positivity in stools. The clinical diagnoses of the patients have shown variations, such as celiac disease, giardiasis, secretory IgA deficiency, and Helicobacter pylori gastritis, and some of them were associated with the others. Conclusions: Although the endoscopic appearance is similar, clinical spectrum and pathologic features are not so similar and there are no specific histomorphologic findings for nodularity. The most demonstrative findings we observed in children were increased lymphocyte and/or eosinophil infiltration in the duodenal mucosa. We suggested that care should be taken in the evaluation of microbiological and immunologic etiologies causing this prominent inflammatory reaction.

  10. Pancreatic and Gastric Heterotopia with Associated Submucosal Lipoma Presenting as a 7-cm Obstructive Tumor of the Ileum: Resection with Double Balloon Enteroscopy.

    Science.gov (United States)

    Jiang, Kun; Stephen, F Otis; Jeong, Daniel; Pimiento, Jose M

    2015-01-01

    Pancreatic and gastric heterotopias are rare clinical entities which have been identified throughout the entire length of the gastrointestinal tract. Combined gastric and pancreatic heterotopias, although unusual, have been described in the duodenum and jejunum, and in other structures, including Meckel's diverticulum and the ampulla of Vater. We report a novel case of pancreatic and gastric heterotopia with an associated submucosal lipoma in a 38-year-old female with a recent history of rectal cancer and chronic crampy abdominal pain. On computed tomography, a 7-cm luminal polypoid mass extending into the distal ileum was discovered. The mass was successfully resected using retrograde double balloon enteroscopy. We believe this is the first report of all three histological entities co-existing in an obstructive ileal lesion in an adult. It highlights endoscopic resection trough double enteroscopy as a safe alternative to more invasive surgical approaches for this type of lesion.

  11. Gastric heterotopia of the anus: Report of two rare cases and review of the literature

    Directory of Open Access Journals (Sweden)

    Rifat Mannan Abul Ala

    2008-04-01

    Full Text Available Heterotopic gastric mucosa is an extremely rare occurrence in the anorectal region, with 41 reported cases till date. Of these, in only nine cases the heterotopic tissue has been found to be located within 2 cm of the dentate line. We report two cases of gastric heterotopia in the anus - one, in a 55-year-old man; and the other, in a 35-year-old woman. Sigmoidoscopy showed presence of a single sessile anal polyp in the first patient and hemorrhoid in the other. Pathologic examination of the biopsy specimens revealed fundic-type gastric mucosa in both the cases. Both the patients had complete resolution of symptoms after the excision. We present these cases to highlight the significance of recognizing this unusual histologic entity. To the best of our knowledge, the second case represents the first reported description of gastric heterotopia in association with hemorrhoid.

  12. Stimulation of the bilateral anterior nuclei of the thalamus in the treatment of refractory epilepsy: two cases of subcortical band heterotopia.

    Science.gov (United States)

    Franco, Ana; Pimentel, José; Campos, Alexandre Rainha; Morgado, Carlos; Pinelo, Sara; Ferreira, António Gonçalves; Bentes, Carla

    2016-12-01

    Subcortical band heterotopia is a neuronal migration disorder that may cause refractory epilepsy. In these patients, resective surgery has yielded inadequate results. Deep brain stimulation of the anterior nuclei of the thalamus has been used for the treatment of refractory epilepsy with good results. We describe the first two patients with subcortical band heterotopia who were submitted to deep brain stimulation of the anterior nuclei of the thalamus, with evaluation of seizure outcome after 12 and 18 months of follow-up. At these times, both showed a >50% decrease in seizure frequency and an increase in seizure freedom. Both patients had a depressive syndrome after surgery that responded fully to anti-depressive medication in one patient and partly in the other. In both, deep brain stimulation of the anterior nuclei of the thalamus was associated with good seizure outcome. This procedure can therefore be considered in the treatment of patients with subcortical band heterotopia and refractory epilepsy. Depression may be a transient adverse event of the surgery or stimulation, however, its aetiology is probably multifactorial.

  13. Ectopic gastric mucosa in the duodenal bulb

    International Nuclear Information System (INIS)

    Schnell, H.; Oehler, G.; Schulz, A.; Rau, W.S.; Giessen Univ.; Giessen Univ.

    1989-01-01

    The radiological and clinical findings of 12 patients with ectopic gastric mucosa in the duodenal bulb are presented. This is a defined disease with characteristic radiological features: multiple small nodular defects of the contrast medium of 1-3 mm diameter. Histology shows complete heterotopia. Pathogenesis and clinical significance are discussed with reference to the literature on this subject. (orig.) [de

  14. Nodular hidradenoma of the caruncle

    DEFF Research Database (Denmark)

    Christakopoulos, Christos; Prause, Jan Ulrik; Heegaard, Steffen

    2013-01-01

    . Immunohistochemical staining of the tumor cells demonstrated positive staining with antibodies against CK-CAM 5.2, BER-EP4, p63, and smooth muscle actin-positive cells in some areas. The tumor was diagnosed as a nodular hidradenoma of the caruncle, which has never been reported before and should be included...

  15. Pancreatic and Gastric Heterotopia with Associated Submucosal Lipoma Presenting as a 7-cm Obstructive Tumor of the Ileum: Resection with Double Balloon Enteroscopy

    Directory of Open Access Journals (Sweden)

    Kun Jiang

    2015-07-01

    Full Text Available Pancreatic and gastric heterotopias are rare clinical entities which have been identified throughout the entire length of the gastrointestinal tract. Combined gastric and pancreatic heterotopias, although unusual, have been described in the duodenum and jejunum, and in other structures, including Meckel's diverticulum and the ampulla of Vater. We report a novel case of pancreatic and gastric heterotopia with an associated submucosal lipoma in a 38-year-old female with a recent history of rectal cancer and chronic crampy abdominal pain. On computed tomography, a 7-cm luminal polypoid mass extending into the distal ileum was discovered. The mass was successfully resected using retrograde double balloon enteroscopy. We believe this is the first report of all three histological entities co-existing in an obstructive ileal lesion in an adult. It highlights endoscopic resection trough double enteroscopy as a safe alternative to more invasive surgical approaches for this type of lesion.

  16. Study on the reuse of nodular casting

    International Nuclear Information System (INIS)

    Bermont, V.M; Gomez, C.A; Lamas, J.F; Castillo, R.N

    2004-01-01

    Nodular cast pieces that have worn out are an attractive alternative to be reused as a cheap raw material for directly making other pieces. This materials recycling process often requires new and successive thermal treatments in order to be machined, to obtain the proper mechanical and microstructural properties. This work includes the results of the microstructural analysis by optic and Scanning Electron Microscopy and of the mechanical tests for traction and hardness of the test pieces submitted to different successive thermal treatments. The results show that by means of successive thermal treatments, followed by austemperizing, the appropriate mechanical and microstructural properties can be recovered permitting the nodular castings that were studied to be reliably reused (CW)

  17. Malignant nodular hidradenoma of scalp

    Directory of Open Access Journals (Sweden)

    Tanmoy Maiti

    2014-01-01

    Full Text Available Malignant nodular hidradenoma (MNH is a rare tumor of sweat gland known by many names in the literature. Scalp is a known and yet uncommon site of occurrence. We describe two patients with scalp MNH with brain parenchymal invasion. Both tumors recurred in spite of total excision and radiotherapy.

  18. What Is It Like to Be a Child? Childhood Subjectivity and Teacher Memories as Heterotopia

    Science.gov (United States)

    Chang-Kredl, Sandra; Wilkie, Gala

    2016-01-01

    Foucault's notion of heterotopia offers a novel way to understand teachers' conceptualizations of childhood, in juxtaposing adult memories of childhood with their present context of teaching children. Memory writing prompts were given to 41 early childhood teachers, and the resulting written narratives were analyzed as heterotopic spaces. The…

  19. Primary pigmented nodular adrenocortical disease

    Directory of Open Access Journals (Sweden)

    Marie T Manipadam

    2011-01-01

    Full Text Available Primary pigmented nodular adrenocortical disease (PPNAD is a rare cause of ACTH-independent Cushing′s syndrome and has characteristic gross and microscopic pathologic findings. We report a case of PPNAD in a 15-year-old boy, which was not associated with Carney′s complex. Bilateral adrenalectomy is the treatment of choice.

  20. Title: Cytoskeletal proteins in cortical development and diseasesubtitle: Actin associated proteins in periventricular heterotopia

    Directory of Open Access Journals (Sweden)

    Gewei eLian

    2015-04-01

    Full Text Available The actin cytoskeleton regulates many important cellular processes in the brain, including cell division and proliferation, migration, and cytokinesis and differentiation. These developmental processes can be regulated through actin dependent vesicle and organelle movement, cell signaling, and the establishment and maintenance of cell junctions and cell shape. Many of these processes are mediated by extensive and intimate interactions of actin with cellular membranes and proteins. Disruption in the actin cytoskeleton in the brain gives rise to periventricular heterotopia (PH, a malformation of cortical development, characterized by abnormal neurons clustered deep in the brain along the lateral ventricles. This disorder can give rise to seizures, dyslexia and psychiatric disturbances. Anatomically, PH is characterized by a smaller brain (impaired proliferation, heterotopia (impaired initial migration and disruption along the neuroependymal lining (impaired cell-cell adhesion. Genes causal for PH have also been implicated in actin-dependent processes. The current review provides mechanistic insight into actin cytoskeletal regulation of cortical development in the context of this malformation of cortical development.

  1. Periventricular heterotopia and white matter abnormalities in a girl with mosaic ring chromosome 6.

    Science.gov (United States)

    Nishigaki, Satsuki; Hamazaki, Takashi; Saito, Mika; Yamamoto, Toshiyuki; Seto, Toshiyuki; Shintaku, Haruo

    2015-01-01

    Ring chromosome 6 is a rare chromosome abnormality that arises typically de novo. The phenotypes can be highly variable, ranging from almost normal to severe malformations and neurological defects. We report a case of a 3-year-old girl with mosaic ring chromosome 6 who presented with being small for gestational age and intellectual disability, and whose brain MRI later revealed periventricular heterotopia and white matter abnormalities. Mosaicism was identified in peripheral blood cells examined by standard G-bands, mos 46,XX,r(6)(p25q27)[67]/45,XX,-6[25]/46,XX,dic r(6:6)(p25q27:p25q27)[6]/47,XX,r(6)(p25q27) × 2[2]. Using array-comparative genomic hybridization, we identified terminal deletion of 6q27 (1.5 Mb) and no deletion on 6p. To our knowledge, this is the first report of periventricular heterotopia and white matter abnormalities manifested in a patient with ring chromosome 6. These central nervous system malformations are further discussed in relation to molecular genetics.

  2. CT and MR evaluation of migrational disorders of the brain. Pt. 2. Schizencephaly, heterotopia and polymicrogyria

    Energy Technology Data Exchange (ETDEWEB)

    Byrd, S E; Osborn, R E; Bohan, T P; Naidich, T P

    1989-05-01

    The migrational disorders are a rare group of congenital malformations of the brain. They consist of the following entities - lissencephaly (agyria - pachygyria), pachygyria, schizencephaly, heterotopia and polymicrogyria. We studied 40 children with migrational disorders radiologically with CT and MR. This article (Part II) deals with our patients with schizencephaly, heterotopia and polymicrogyria. These patients presented clinically with a variety of symptoms. The most common were seizures, delayed development, failure to thrive and hydrocephalus. CT and MR both demonstrated the characteristic findings in all of our patients except the polymicrogyria group. The gray matter and cleft abnormalities seen in these disorders were demonstrated with CT and MR. However, MR provided better delineation of these disorders than CT. Because some forms of migrational disorders can be inherited, it is extremely important for the radiologist to understand the characteristic findings for correct diagnosis which is essential for parental counseling.

  3. Unilateral nodular adrenal hyperplasia: Case series

    African Journals Online (AJOL)

    A.F. Kotb

    2016-07-26

    Jul 26, 2016 ... Abstract. Introduction: Nodular adrenal hyperplasia is one of rare causes of adrenocortical hyperplasia. The disease usually presents bilaterally. Few publications discussed the possibility of unilateral disease, in association with hyperaldosteronism or Cushing syndrome. Case series: We are reporting 3 ...

  4. [A patient with muscular torticollis caused by nodular fasciitis in the sternocleidomastoid muscle (SCM)].

    Science.gov (United States)

    Hemmi, Shoji; Murakami, Tatufumi; Shirabe, Teruo; Sunada, Yoshihide

    2002-09-01

    Nodular fasciitis is a benign pseudosarcomatous proliferative lesion which is frequently misdiagnosed as malignant tumor clinically and microscopically. It usually occurs as a rapidly enlarging subcutaneous mass on the upper extremities, especially on the forearm. Here we report a patient showing muscular torticollis caused by nodular fasciitis in the sternocleidomastoid muscle (SCM). A 17-year-old woman was hospitalized because of rapidly progressive torticollis. The right SCM was markedly enlarged and firm on palpation. Muscle biopsy taken from the right SCM revealed massive proliferation of spindle shaped fibroblasts infiltrating into the endomysium. These findings coincided with the intramuscular nodular fasciitis. However, different from typical nodular fasciitis, no apparent nodule formation was found in this patient. Instead, diffuse proliferative lesion extended widely into the neck soft tissue. To our knowledge, this is the first report of muscular torticollis caused by nodular fasciitis involving the SCM.

  5. Sonographic Findings of Hashimoto's Thyroiditis and Associated Nodular Lesions

    International Nuclear Information System (INIS)

    Kang, Bong Joo; Park, Young Ha; Jung, So Lyung; Chung, Soo Kyo

    2007-01-01

    To evaluate the sonographic findings of Hashimoto's thyroiditis and associated nodular lesions. We retrospectively reviewed the sonographic findings of twenty patients who had surgically confirmed Hashimoto's thyroiditis between 1 March 2005, and 26 November 2005. In these patients, we reviewed the sonographic findings of the associated focal nodular lesion. Assessed were size, homogeneity, and echogenicity of the diseased thyroid gland and shape, echogenicity, margin, rim, microcal cification of the associated nodules. Without knowledge of the pathological diagnosis of the nodular lesions, based on the sonographic criteria, the nodules were classified as either malignant or benign. Hashimoto's thyroiditis demonstrates a variety of sonographic findings for size, homogeneity, and echogenicity. Among the nineteen nodules that were sonographically diagnosed and pathologically confirmed, nine papillary cancers, seven nodular hyperplasias, two Huthle cell adenomas, and one focal hyalinized fibrosing nodule were included. All of the nine papillary cancers showed more than one malignant finding such as marked hypoechogenicity, an irregular shape, a taller than wide shape, a spiculated margin, or microcalcifications that were classified as malignant nodulea, and all of the ten benign nodules showed no malignant findings. Circumscribed isoechoic, hyperechoic, or hypoechoic nodules without calcification were classified as bending nodules. Hashimoto's thyroiditis demonstrates various findings on a sonographic examination,and associated various benign and malignant lesions. Moreover, a sonographic examination is helpful to differentiate between malignant and benign lesions in Hashimoto's thyroiditis as in the normal thyroid

  6. Iodinated Contrast Media-Induced Thyroid Dysfunction in Euthyroid Nodular Goiter Patients.

    Science.gov (United States)

    Kornelius, Edy; Chiou, Jeng-Yuan; Yang, Yi-Sun; Lo, Shih-Chang; Peng, Chiung-Huei; Lai, Yung-Rung; Huang, Chien-Ning

    2016-08-01

    The risks of thyroid dysfunction after iodinated contrast media exposure in patients with euthyroid nodular goiter are largely unknown. This observational, retrospective cohort study included a random selection of one million people in Taiwan. All patients with iodinated contrast media exposure during this study period were selected. Patients with euthyroid nodular goiter were identified as cases, while patients without thyroid nodule were selected as controls. We followed these patients until the first event of thyroid dysfunction including hyperthyroidism or hypothyroidism after iodinated contrast media exposure. A total of 334 cases and 2672 matched controls were selected in this study. The mean age of cases and controls were 58.6 and 58.4 years old, and mean follow-up durations were 2.1 and 2 years respectively. After adjustment, patients with euthyroid nodular goiter had a higher risk of thyroid dysfunction (hazard ratio 5.43, [confidence interval (CI) 3.01-9.80]) compared with controls after iodinated contrast media exposure. In the subgroup analysis, the risks of hyperthyroidism and hypothyroidism in cases compared with controls were 5.77 [CI 2.64-12.62] and 4.95 [CI 2.15-11.40] respectively. Half of the euthyroid nodular goiter cases developed thyroid dysfunction within one year after iodinated contrast media exposure. Interestingly, all thyroid-related comorbidities and drug prescriptions did not increase the risk of thyroid dysfunction. Presence of euthyroid nodular goiter was associated with higher risk of thyroid dysfunction including hyperthyroidism and hypothyroidism after iodinated contrast media exposure.

  7. Grave's Eye disease developing following radioiodine treatment for toxic nodular goitre.

    Science.gov (United States)

    Tahrani, A A; Rangan, S; Moulik, P

    2007-07-01

    The development of Grave's ophthalmopathy (GO) following radioiodine (RI) treatment for Grave's thyrotoxicosis, though controversial, is well described. The development of ophthalmopathy following RI treatment for toxic nodular goitre is much less recognised. We report a 49 year-old female patient who developed thyrotoxicosis and GO after receiving RI treatment for toxic nodular goitre and we also review the relevant literature.

  8. Agenesis of the corpus callosum and gray matter heterotopia in three patients with constitutional mismatch repair deficiency syndrome.

    Science.gov (United States)

    Baas, Annette F; Gabbett, Michael; Rimac, Milan; Kansikas, Minttu; Raphael, Martine; Nievelstein, Rutger Aj; Nicholls, Wayne; Offerhaus, Johan; Bodmer, Danielle; Wernstedt, Annekatrin; Krabichler, Birgit; Strasser, Ulrich; Nyström, Minna; Zschocke, Johannes; Robertson, Stephen P; van Haelst, Mieke M; Wimmer, Katharina

    2013-01-01

    Constitutional mismatch repair deficiency (CMMR-D) syndrome is a rare inherited childhood cancer predisposition caused by biallelic germline mutations in one of the four mismatch repair (MMR)-genes, MLH1, MSH2, MSH6 or PMS2. Owing to a wide tumor spectrum, the lack of specific clinical features and the overlap with other cancer predisposing syndromes, diagnosis of CMMR-D is often delayed in pediatric cancer patients. Here, we report of three new CMMR-D patients all of whom developed more than one malignancy. The common finding in these three patients is agenesis of the corpus callosum (ACC). Gray matter heterotopia is present in two patients. One of the 57 previously reported CMMR-D patients with brain tumors (therefore all likely had cerebral imaging) also had ACC. With the present report the prevalence of cerebral malformations is at least 4/60 (6.6%). This number is well above the population birth prevalence of 0.09-0.36 live births with these cerebral malformations, suggesting that ACC and heterotopia are features of CMMR-D. Therefore, the presence of cerebral malformations in pediatric cancer patients should alert to the possible diagnosis of CMMR-D. ACC and gray matter heterotopia are the first congenital malformations described to occur at higher frequency in CMMR-D patients than in the general population. Further systematic evaluations of CMMR-D patients are needed to identify possible other malformations associated with this syndrome.

  9. Nodular Hepatic Tuberculosis Masquerading as a Seminoma Liver Metastasis.

    Science.gov (United States)

    Harbi, Houssem; Chaabouni, Amine; Kallel, Rim; Toumi, Nozha; Fendri, Sami; Krichene, Jihene; Boujelbene, Salah; Mzali, Rafik

    2018-04-01

    Isolated macro-nodular liver tuberculosis is a very rare condition. It may mimic primitive or secondary tumors of the liver. This could delay or mislead the therapeutic management. An immunocompetent 48-year-old man with a history of non-metastatic seminoma was treated with right orchidectomy followed by 20 Gy radiotherapy. The discovery, 8 months later, of a 2 cm nodule of the hepatic dome evoked a liver metastasis. Percutaneous biopsy was not feasible. Wedge resection was performed whereas medical treatment would have sufficed, as pathologic examination of the resected specimen showed a macro-nodular hepatic tuberculosis. The patient received anti-tuberculosis drugs for 9 months. The diagnosis of isolated macro-nodular liver tuberculosis is frequently misleading, particularly in immunocompetent and paucisymptomatic patients. Thus percutaneous biopsy is mandatory for diagnosis and also prior to any major surgeries. © 2018 The Author(s). This is an open-access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

  10. Inclusion as heterotopia: Spaces of encounter between people with and without intellectual disability

    Directory of Open Access Journals (Sweden)

    Herman Meininger

    2013-08-01

    Full Text Available The public space to which - in line with common definitions of inclusion - persons with intellectual disability are designated often turns out to be a space of discrimination. The aim of this study is to identify spaces of encounter. Michel Foucault's concept of heterotopia - 'other spaces' - and its subsequent interpretations are used as an analytical tool for evaluating efforts to include persons with intellectual disability in society. In Foucault's work 'space' refers to geographical places and to 'sites' that can be defined by sets of relations. Among these spaces are 'counter-sites' like large scale residential facilities for people with intellectual disability. Though policies of deinstitutionalisation aim at erasure of these places of exclusion, the results are often disappointing, largely because these policies ignore the analysis of power dynamics that install exclusionary processes and structures. Recent interpretations of the concept of heterotopia position the 'other places' in the middle of ordinary life as social spaces of encounter and dialogue between the 'normal' and the 'abnormal'. Characteristics and practical conditions of such spaces of encounter are explored. These interpretations open up a new conceptualisation of inclusion in terms of niches in which encounter and dialogue are cultivated and an alternative social ordering can be exercised.

  11. Giant hidradenocarcinoma: a report of malignant transformation from nodular hidradenoma.

    Science.gov (United States)

    Lim, S C; Lee, M J; Lee, M S; Kee, K H; Suh, C H

    1998-10-01

    A giant hidradenocarcinoma presented by a 75-year-old female is reported. The patient had a malignant transformation within a nodular hidradenoma involving the right postauricular area, which was treated by mass removal and a right radical neck dissection with a free-flap covering. Malignant hidradenocarcinoma is the least common adnexal tumor of uncertain origin. They are usually malignant from their inception, but some develop from a benign counterpart. To the authors' knowledge, only three cases have been reported previously. Two histologically distinct components were seen in this tumor: (i) typical nodular hidradenoma, which constituted a small part of the tumor; and (ii) carcinoma with areas of transition. The secretory cells of hidradenocarcinoma showed decapitation secretion on light and electron microscopic observations, which is evidence of apocrine differentiation. Histologically, this case was concluded as a hidradenocarcinoma arising from a long-standing nodular hidradenoma. A literature review is presented and the histological, immunohistochemical and ultrastructural features are described.

  12. Malignant transformation of nodular hyperplasia in the thyroid: a case report

    International Nuclear Information System (INIS)

    In, Hyun Sin; Kim, Dong Wook; Yoon, Hye Kyoung

    2007-01-01

    Thyroid carcinogenesis is traditionally thought to originate 'de novo'. However, it is debatable whether a malignant transformation can possibly arise from a benign thyroid nodule, as suggested for the malignant transformation of a thyroid adenoma. To the best of our knowledge, no studies have been performed addressing the malignant transformation of nodular hyperplasia in the thyroid gland. Here, we report a case of nodular hyperplasia with focally malignant degeneration

  13. Giant polypoid gastric heterotopia in the small intestine in a boy: A case report and literature review.

    Science.gov (United States)

    Cai, Jing; Yu, Haibo

    2017-01-01

    Heterotopic gastric mucosa has been described at various locations of the body; however, the polyp composed of heterotopic gastric mucosa in the small intestine is rare. A 15-year-old boy visited us for investigation of recurrent episodes of melena. Capsule endoscopy (CE) revealed a polypoid tumor in the ileum, with an active nearby hemorrhage. Contrast-enhanced computed tomography (CECT) showed a tumor in the right quadrant of the abdomen, with a diameter of about 18 × 14 mm. The patient was diagnosed with polypoid gastric heterotopia. We performed an operation to resect the lesion. The patient recovered smoothly after surgery and was discharged on postoperative day 7 and followed up for 3 months. He has not experienced gastrointestinal intestinal (GI) symptoms up to now. Giant polypoid gastric heterotopia in the small intestine is extremely rare, which can express as an occasional finding with or without symptoms. Surgical resection is the preferred therapy when symptoms appear.

  14. [Histopathological Study of the Relationship between Lymphoid Follicles and Different Endoscopic Types of Nodular Gastritis].

    Science.gov (United States)

    Nagata, Takuo; Ishitake, Hisahito; Shimamoto, Fumio; Tamura, Tadamasa; Matsumura, Kazunori; Sumii, Masaharu; Nakai, Shirou

    2014-11-01

    Nodular gastritis is characterized histologically by hyperplasia and enlargement of lymphoid follicles in the lamina propria. With the objective of elucidating the relationship between different endoscopic types of nodular gastritis and lymphoid follicles, distributions of lymphoid follicles in the lamina propria were investigated in young gastric cancer patients with nodular gastritis. For the study, whole-mucosal step sectioning of each resected stomach was performed, the densities of lymphoid follicles of all specimens were measured microscopically, and the horizontal and depth distributions were calculated. For assessment in the horizontal direction, density distribution diagrams of lymphoid follicles were created. For assessment in the depth direction, the different endoscopic types of nodular gastritis were compared in the five different analysis sites. In the assessment of the horizontal distribution, no characteristic distribution tendencies were observed in either the granular type group or the scattered type group; however, it was found that areas with relatively high densities of lymphoid follicles generally coincided with the areas where nodular gastritis was observed endoscopically. These results suggested that hyperplasia and aggregation of lymphoid follicles in the lamina propria are involved at the sites where nodular gastritis is observed endoscopically. In the assessment of the depth distribution, lymphoid follicles tended to be more unevenly distributed in the upper lamina propria in the granular type group than in the scattered type at the three different analysis sites where nodular gastritis was observed endoscopically. These results suggested the possibility of a granular type characteristic.

  15. Pediatric Glial Heterotopia in the Medial Canthus.

    Science.gov (United States)

    Kim, Soung Min; Amponsah, Emmanuel Kofi; Eo, Mi Young; Cho, Yun Ju; Lee, Suk Keun

    2017-11-01

    Glial heterotopias are rare, benign, congenital, midline, and nonteratomatous extracranial glial tissue. They may be confused as encephalocele or dermoid cysts and are mostly present in the nose.An 8-month-old African female child presented with a slow growing paranasal mass. The mass had been present at the left upper medial canthus since birth and had slowly and progressively enlarged. There was no communication between the mass and the cranial cavity during the operational procedure. The mass was immunohistochemically positive for S-100 protein as well as for glial fibrillary acidic protein, but negative for proliferating cell nuclear antigen. This suggested that the mass was composed of benign glial tissues with many astrocytes.The purpose of this report is to demonstrate the first patient with pediatric glial heterotopic tissue in the medial canthus and to report the clinical importance of its immunohistochemical findings.

  16. Genetic and non-iodine-related factors in the aetiology of nodular goitre.

    Science.gov (United States)

    Knudsen, Nils; Brix, Thomas Heiberg

    2014-08-01

    Genetic and a large number of environmental non-iodine-related factors play a role in the cause of nodular goitre. Most evidence for the influence of genetic and environmental factors in the cause of goitre is from cross-sectional, population-based studies. Only a few studies have included prospective data on risk factors for nodular goitre, although few prospective data are available on the effect of iodine and tobacco smoking on goitre development. Goitre is not one single phenotype. Many epidemiological studies do not distinguish diffuse from nodular goitre, as the investigated parameter is often thyroid volume or frequency with increased thyroid volume. Moreover, information on the presence and effect of gene-environment, gene-gene, and environment-environment effect modifications is limited. Thus, firm conclusions about the relative contributions and causality of the investigated risk factors should be made with caution. Smoking seems to be an established risk factor for nodular goitre, possibly with effect modification from iodine intake, as the risk associated with smoking is smaller or absent in areas with sufficient iodine intake. The use of oral contraceptives might have protective effects against goitre, and childbirth is an increased risk factor for goitre in areas with non-optimal iodine intake. Insulin resistance is a recently investigated risk factor, and the risk of goitre may be reversible with metformin treatment. Iodine remains the major environmental risk factor for nodular goitre. Copyright © 2014 Elsevier Ltd. All rights reserved.

  17. Nodular Epiescleritis Granulomatous Canine. Case Report

    Directory of Open Access Journals (Sweden)

    Camilo Guarín Patarroyo

    2011-12-01

    Full Text Available Granulomatous epiescleritis nodular disease in canines is a very unusual presentation that affects or external fibrous tunic of the eyeball and conjunctiva, which was an increase similar to a unilateral or bilateral tumor. Suspected immune-mediated disease due to lack of identification of an etiologic agent and the response to treatment with immunosuppressive drugs (Couto, 1992. The ideal therapy is the application of steroids via intralesional, topical or systemic, or other immunosuppressants such as cyclosporine and azathioprine; it is still advisable to apply antibiotic is the ideal combination of tetracycline and neomycin (Gilger & Whitley, 1999. The diagnostic method of episcleritis is made by histopathology, which is evident in changes similar to chronic granulomatous inflammation. Are claiming a racial bias in Alsatian, Shepherd Collie Shetland Shepherd, Coker Spaniel, Rottweiler and Labrador Retriever (Gough & Thomas, 2004. The following case is a report of a nodular epiescleritis affecting the cornea, sclera, and the corneoscleral limbus, which describes the diagnosis, signology and treatment.

  18. Visions and heterotopias: the Iconografy of the ruins in the definitio of contemporary urban landscapes

    Directory of Open Access Journals (Sweden)

    Gregorio Froio

    2014-05-01

    Full Text Available The purpose of this essay is to describe the thin red line that connects the multiple meanings of the term archeology with the visual representation of the contemporary city. The iconography of urban views especially from a reading decentralized and alienating: the archaelogical character of the post-metropolis becomes the kay scenes in the balance between urban utopias and heterotopias.

  19. Visions and heterotopias: the Iconografy of the ruins in the definitio of contemporary urban landscapes

    OpenAIRE

    Gregorio Froio

    2014-01-01

    The purpose of this essay is to describe the thin red line that connects the multiple meanings of the term archeology with the visual representation of the contemporary city. The iconography of urban views especially from a reading decentralized and alienating: the archaelogical character of the post-metropolis becomes the kay scenes in the balance between urban utopias and heterotopias.

  20. Fatal Lyme carditis and endodermal heterotopia of the atrioventricular node.

    Science.gov (United States)

    Cary, N. R.; Fox, B.; Wright, D. J.; Cutler, S. J.; Shapiro, L. M.; Grace, A. A.

    1990-01-01

    A fatal case of Lyme carditis occurring in a Suffolk farmworker is reported. Post-mortem examination of the heart showed pericarditis, focal myocarditis and prominent endocardial and interstitial fibrosis. The additional finding of endodermal heterotopia ('mesothelioma') of the atrioventricular node raises the possibility that this could also be related to Lyme infection and account for the relatively frequent occurrence of atrioventricular block in this condition. Lyme disease should always be considered in a case of atrioventricular block, particularly in a young patient from a rural area. The heart block tends to improve and therefore only temporary pacing may be required. Images Figure 1 Figure 2 Figure 3 PMID:2349186

  1. Nodular Hyperplasia Arising from the Lateral Aberrant Thyroid Tissue: A Case Report

    International Nuclear Information System (INIS)

    Jeong, Min Hye; Park, Jeong Seon; Lee, Young Jun

    2012-01-01

    The presence of aberrant thyroid tissue in the lateral neck is very rare. In addition, nodular hyperplasia in ectopic thyroid has rarely been reported. Due to the unusual location, the presence of lateral aberrant thyroid tissue could be misdiagnosed as a lymphadenopathy, neurogenic tumor, etc. We report on a case of nodular hyperplasia arising from the right lateral aberrant thyroid tissue.

  2. Hashimoto thyroiditis: Part 1, sonographic analysis of the nodular form of Hashimoto thyroiditis.

    Science.gov (United States)

    Anderson, Lauren; Middleton, William D; Teefey, Sharlene A; Reading, Carl C; Langer, Jill E; Desser, Terry; Szabunio, Margaret M; Hildebolt, Charles F; Mandel, Susan J; Cronan, John J

    2010-07-01

    The purpose of this article is to analyze the sonographic appearance of nodular Hashimoto thyroiditis. As part of an ongoing multiinstitutional study, patients who underwent ultrasound examination and fine-needle aspiration of one or more thyroid nodules were analyzed for multiple predetermined sonographic features. Patients completed a questionnaire, including information about thyroid function and thyroid medication. Patients (n = 61) with fine-needle aspiration cytologic results consistent with nodular Hashimoto thyroiditis (n = 64) were included in the study. The mean (+/- SD) diameter of nodular Hashimoto thyroiditis was 15 +/- 7.33 mm. Nodular Hashimoto thyroiditis occurred as a solitary nodule in 36% (23/64) of cases and in the setting of five or more nodules in 23% (15/64) of cases. Fifty-five percent (35/64) of the cases of nodular Hashimoto thyroiditis occurred within a sonographic background of diffuse Hashimoto thyroiditis, and 45% (29/64) of cases occurred within normal thyroid parenchyma. The sonographic appearance was extremely variable. It was most commonly solid (69% [42/61] of cases) and hypoechoic (47% [27/58] of cases). Twenty percent (13/64) of nodules had calcifications (seven with nonspecific bright reflectors, four with macrocalcifications, and three eggshell), and 5% (3/64) of nodules had colloid. Twenty-seven percent (17/64) of nodules had a hypoechoic halo. The margins were well defined in 60% (36/60) and ill defined in 40% (24/60) of nodules. On Doppler analysis, 35% (22/62) of nodules were hypervascular, 42% (26/62) were isovascular or hypovascular, and 23% (14/62) were avascular. The sonographic features and vascularity of nodular Hashimoto thyroiditis were extremely variable.

  3. Nodular fasciitis: A pseudomalignant clonal neoplasm characterized by USP gene rearrangements and spontaneous regression

    LENUS (Irish Health Repository)

    Hennebry, Jennifer

    2017-01-01

    Introduction: Nodular fasciitis (NF) is a rapidly growing, self-limited, myofibroblastic neoplasm that typically arises in subcutaneous tissues of young adults and regresses spontaneously. Nodular fasciitis mimics sarcoma on clinical, radiological, and histological grounds and is usually, diagnosed following excision.\\r\

  4. {sup 131}I treatment of nodular non-toxic goitre

    Energy Technology Data Exchange (ETDEWEB)

    Nygaard, B.; Faber, J.; Hegdeues, L.; Hansen, J.M. [Herlev Hospital (Denmark)

    1996-01-01

    The traditional treatment of a growing nodular non-toxic goitre has for many years been surgical resection or levothyroxine suppressive treatment. During recent years, several studies have reported promising results of {sup 131}I treatment in terms of thyroid size reduction. This review outlines the different treatment modalities on non-toxic nodular goitre with special emphasis on {sup 131}I treatment. By the term nodular goitre the authors include glands with solitary or multiple thyroid nodules with uptake on a scintiscan. At what point of the natural history of non-toxic multinodular goitre {sup 131}I therapy should be used is not clear. In principle, the best result is obtained in smaller goitres and it is possible that the best effect of {sup 131}I is seen if treatment is given to patients with diffuse goitre before these become nodular. However, then there is a potential risk to swing in the direction to where {sup 131}I is used in an indiscriminate way, since the prevalence of non-toxic multinodular goitre is much higher than that of hyperthyroidism. Although we have data on the long-term hazards of {sup 131}I treatment in hyperthyroidism in terms of risk of cancer, we have only follow-up periods of 5 to 10 years for non-toxic goitres in small groups of patients and no data regarding the long-term risk of high-dose {sup 131}I treatment (>600 MBq) for this condition. Ideally, long term randomized studies comparing the effect, side effect and cost-benefit of surgery as opposed to {sup 131}I treatment should be performed. Awaiting this, it is at present mandatory that each individual patient be given a choice of treatment after proper information. 44 refs.

  5. The many faces of intestinal tract gastric heterotopia; a series of four cases highlighting clinical and pathological heterogeneity.

    Science.gov (United States)

    Nasir, Aqsa; Amateau, Stuart K; Khan, Sabina; Simpson, Ross W; Snover, Dale C; Amin, Khalid

    2018-04-01

    Gastric heterotopia of the intestinal tract can have a diverse clinicopathologic presentation, resulting in a diagnostic dilemma. We present a series of four cases, two male and two female patients with age range of 31-82 years, found in the duodenum, jejunum, and transverse colon. The most common and rather unusual clinical presentation was iron deficiency anemia, seen in three cases, while one patient presented with abdominal pain. Endoscopically, two cases were visualized as pedunculated polyps and two as sessile/plaque-like lesions. Polypectomy was performed in three patients, and one patient underwent biopsy followed by resection. Two cases showed oxyntic-type epithelium, and two cases exhibited pyloric-type gastric epithelium. Three patients were relieved of their presenting symptoms after therapeutic procedures with no evidence of recurrence noted on follow-up. Follow-up was not available on one patient. This case series highlights a diverse clinicopathologic spectrum of gastric heterotopia. Accurate diagnosis is essential for proper management. Copyright © 2018. Published by Elsevier Inc.

  6. The body as the subject’s heterotopia

    Directory of Open Access Journals (Sweden)

    Cleudemar Alves Fernandes

    2016-10-01

    Full Text Available http://dx.doi.org/10.5007/1984-8420.2016v17n2p6 This study is embodied as an enunciation space, which reflects the subject’s position, through discourses materialized in it; thus, the body is considered as a support of a social-historical objectified subject. For this proposition, we are anchored in Michel Foucault’s thought, specifically in the notions of space, heterotopias, utopia, subjectivity; and we analyze a video presented in the talent show contest Se ela dança, eu danço, from the Sistema Brasileiro de Televisão – SBT, in January, 2011. In this video, the choreog­raphy proposed by the contester John Lennon da Silva results from a rereading of the classic ballet The Dying Swan. We focus on the transformation of the subjects’ position that composes the jury and on the objectification of the dancer subject before, during and after the choreography.

  7. Nodular granulomatous phlebitis: a phlebitic tuberculid.

    Science.gov (United States)

    McHugh, Angela; Siller, Gregory; Williamson, Richard; Faulkner, Catherine

    2008-11-01

    A 22-year-old woman presented with recurrent non-ulcerating skin nodules overlying the great saphenous vein on the anteromedial lower legs. Histology showed a granulomatous phlebitis, and polymerase chain reaction performed on lesional skin detected DNA specific for Mycobacterium tuberculosis. The lesions resolved with anti-tuberculous therapy. This case may be a further example of nodular granulomatous phlebitis, a phlebitic tuberculid.

  8. Nodular cast iron and casting monitoring

    Directory of Open Access Journals (Sweden)

    S. Pietrowski

    2008-10-01

    Full Text Available In this paper quality monitoring of nodular cast iron and casting made of it is presented. A control system of initial liquid cast iron to spheroidization, after spheroidization and inoculation with using of TDA method was shown. An application of an ultrasonic method to assessment of the graphite form and the metal matrix microstructure of castings was investigated.

  9. Genetic and non-iodine-related factors in the aetiology of nodular goitre

    DEFF Research Database (Denmark)

    Knudsen, Nils; Brix, Thomas Heiberg

    2014-01-01

    Genetic and a large number of environmental non-iodine-related factors play a role in the cause of nodular goitre. Most evidence for the influence of genetic and environmental factors in the cause of goitre is from cross-sectional, population-based studies. Only a few studies have included...... prospective data on risk factors for nodular goitre, although few prospective data are available on the effect of iodine and tobacco smoking on goitre development. Goitre is not one single phenotype. Many epidemiological studies do not distinguish diffuse from nodular goitre, as the investigated parameter...... is often thyroid volume or frequency with increased thyroid volume. Moreover, information on the presence and effect of gene-environment, gene-gene, and environment-environment effect modifications is limited. Thus, firm conclusions about the relative contributions and causality of the investigated risk...

  10. Nodular Gastritis and Pathologic Findings in Children and Young Adults with Helicobacter pylori Infection

    Science.gov (United States)

    Koh, Hong; Noh, Tae-Woong; Baek, Seoung-Yon

    2007-01-01

    Purpose The aim of this study was to investigate the pathologic characteristics of nodular gastritis in children and young adults infected with Helicobacter pylori (H. pylori). Materials and Methods A total of 328 patients were enrolled in this study, and the diagnosis of H. pylori infection was done with gastroduodenal endoscopy concomitant with a CLO™ test and pathologic analysis of the biopsy specimens. Diagnoses of normal, superficial gastritis, nodular gastritis, and peptic ulcer disease were made from the gastroduodenal endoscopic findings. The density of H. pylori organisms in the gastric mucosa was rated as normal, mild, moderate, or marked. The pathologic findings of nodular gastritis were based on the histopathologic findings of inflammation, immune activity, glandular atrophy and intestinal metaplasia. Each of these findings was scored as either normal (0), mild (1), moderate (2), or marked (3) according to the updated Sydney system and using visual analog scales. The gastritis score was the sum of the four histopathologic scores. Results In this study, nodular gastritis (50.6%) was most common, and mild density (51.5%) H. pylori infection was also common upon microscopic examination. Intestinal metaplasia occurred in 9 patients (2.7%). Conclusion Logistic regression revealed a significant increase in the incidence of nodular gastritis with gastritis score (p = 0.008), but not an association with sex, age, or H. pylori density. Gastritis score was the only significant factor influencing the occurrence of nodular gastritis. Intestinal metaplasia, which was originally thought to be a pre-malignant lesion, occurred in 2.7% of the patients with H. pylori infection. PMID:17461522

  11. Open questions in the management of nodular lymphocyte predominant hodgkin lymphoma.

    Science.gov (United States)

    Tyran, Marguerite; Gonzague, Laurence; Bouabdallah, Reda; Resbeut, Michel

    2014-01-01

    Localized Nodular Lymphocyte Predominant Hodgkin Lymphoma is a rare disease with an overall good prognosis but frequent late relapses. Due to it's rarity there is no standard therapeutic approach and pathological diagnosis may be hard. In this paper we discuss the technical aspects of the radiation therapy and histological issues. The new fields reductions proposed for classical Hodgkin lymphoma cannot be applied to early stages Nodular Lymphocyte Predominant Hodgkin lymphomas which are usually treated with radiation therapy without systemic chemotherapy.

  12. Association of Helicobacter pylori infection with nodular antritis and follicular gastritis

    Directory of Open Access Journals (Sweden)

    Tomašević Ratko

    2006-01-01

    Full Text Available Introduction. Helicobacter pylori (H. pylori infection is known to be the must common cause of chronic gastritis having some endoscopic and pathologic characteristies as determinated by the Sydney System for Gastritis Classification. The aim of our case report was to point out the relationship between an endoscopic finding of nodular antritis and the presence of H. pylori infection and active chronic gastritis. Case report. Our patient underwent upper gastrointestinal endoscopy for dyspeptic complaints and was diagnosed as having nodular antritis, but also underwent urease test and hystopathologic examination of antral mucosa, to determine the presence and density of H. pylori infection and the presence and severity of gastritis. After a course of anti H. pylori treatment, dyspepsia improved and new biopsy specimens obtained two months and six months afterwards revealed no pathological findings. Conclusion. The case report supported the association of H. pylori infection of lymphoid follicles with nodular gastric mucosis.

  13. Amiloidose pulmonar: relato de caso de achado radiológico da apresentação nodular em grande fumante Pulmonary amyloidosis: radiographic finding of nodular opacities in a heavy smoker

    Directory of Open Access Journals (Sweden)

    Jorge Montessi

    2007-06-01

    Full Text Available A amiloidose pulmonar é uma doença rara, caracterizada pelo depósito extracelular de proteínas fibrilares no pulmão. Amiloidose é um termo genérico para grupos heterogêneos de doenças, incluindo doença de Alzheimer e diabetes mellitus tipo II. Apresenta-se no aparelho respiratório sob as formas traqueobrônquica, nodular pulmonar e septal alveolar (parenquimatosa difusa. Relata-se o caso de uma mulher, tabagista (20 anos/maço, portadora de amiloidose nodular pulmonar, diagnosticada através de exames pré-operatórios à realização de colecistectomia videolaparoscópica.Pulmonary amyloidosis is a rare disease, characterized by extracellular deposition of fibrillary protein in the lungs. Amyloidosis is a generic term for a heterogeneous group of diseases, including Alzheimer's disease and type 2 diabetes mellitus. In the respiratory system, it appears in various forms: tracheobronchial; nodular pulmonary; and alveolar septal (diffuse parenchymal. We present the case of a woman who was a 20 pack-year smoker and had nodular pulmonary amyloidosis, as diagnosed through tests performed prior to laparoscopic cholecystectomy.

  14. Low Iodine in the Follicular Lumen Caused by Cytoplasm Mis-localization of Sodium Iodide Symporter may Induce Nodular Goiter.

    Science.gov (United States)

    Huang, Huibin; Shi, Yaxiong; Liang, Bo; Cai, Huiyao; Cai, Qingyan

    2017-10-01

    Iodine is a key ingredient in the synthesis of thyroid hormones and also a major factor in the regulation of thyroid function. A local reduction of iodine content in follicular lumen leads to overexpression of local thyroid-stimulating hormone receptor (TSHr), which in turn excessively stimulates the regional thyroid tissue, and result in the formation of nodular goiter. In this study, we investigated the relationship between iodine content and sodium iodide symporter (NIS) expression by using the clinical specimens from patients with nodular goiter and explored the pathogenesis triggered by iodine deficiency in nodular goiter. In total, 28 patients were clinically histopathologically confirmed to have nodular goiter and the corresponding adjacent normal thyroid specimens were harvested simultaneously. Western blot and immunohistochemistry were performed to assay NIS expression and localization in thyrocytes of both nodular goiter and adjacent normal thyroid tissues. NIS expression mediated by iodine in follicular lumen was confirmed by follicular model in vitro. Meanwhile, radioscan with iodine-131were conducted on both nodular goiter and adjacent normal thyroid. Our data showed that NIS expression in nodular goiter was significantly higher than that in adjacent normal tissues, which was associated with low iodine in the follicular lumen. Abnormal localization of NIS and lower amount of radioactive iodine-131 were also found in nodular goiter. Our data implied that low iodine in the follicular lumen caused by cytoplasm mis-localization of NIS may induce nodular goiter.

  15. Sonographic Findings of Hashimoto's Thyroiditis and Associated Nodular Lesions

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Bong Joo; Park, Young Ha; Jung, So Lyung; Chung, Soo Kyo [Catholic University of Korea College of Medicine, Seoul (Korea, Republic of)

    2007-12-15

    To evaluate the sonographic findings of Hashimoto's thyroiditis and associated nodular lesions. We retrospectively reviewed the sonographic findings of twenty patients who had surgically confirmed Hashimoto's thyroiditis between 1 March 2005, and 26 November 2005. In these patients, we reviewed the sonographic findings of the associated focal nodular lesion. Assessed were size, homogeneity, and echogenicity of the diseased thyroid gland and shape, echogenicity, margin, rim, microcal cification of the associated nodules. Without knowledge of the pathological diagnosis of the nodular lesions, based on the sonographic criteria, the nodules were classified as either malignant or benign. Hashimoto's thyroiditis demonstrates a variety of sonographic findings for size, homogeneity, and echogenicity. Among the nineteen nodules that were sonographically diagnosed and pathologically confirmed, nine papillary cancers, seven nodular hyperplasias, two Huthle cell adenomas, and one focal hyalinized fibrosing nodule were included. All of the nine papillary cancers showed more than one malignant finding such as marked hypoechogenicity, an irregular shape, a taller than wide shape, a spiculated margin, or microcalcifications that were classified as malignant nodulea, and all of the ten benign nodules showed no malignant findings. Circumscribed isoechoic, hyperechoic, or hypoechoic nodules without calcification were classified as bending nodules. Hashimoto's thyroiditis demonstrates various findings on a sonographic examination,and associated various benign and malignant lesions. Moreover, a sonographic examination is helpful to differentiate between malignant and benign lesions in Hashimoto's thyroiditis as in the normal thyroid

  16. Unilateral nodular adrenal hyperplasia: Case series | Kot | African ...

    African Journals Online (AJOL)

    Introduction: Nodular adrenal hyperplasia is one of rare causes of adrenocortical hyperplasia. The disease usually presents bilaterally. Few publications discussed the possibility of unilateral disease, in association with hyperaldosteronism or Cushing syndrome. Case series: We are reporting 3 cases of radiologically ...

  17. Focal nodular hyperplasia: imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Kehagias, D.; Moulopoulos, L.; Antoniou, A.; Hatziioannou, A.; Smyrniotis, V.; Trakadas, S.; Lahanis, S.; Vlahos, L. [Dept. of Radiology, University of Athens (Greece)

    2001-02-01

    Focal nodular hyperplasia is an uncommon benign hepatic tumor that continues to pose diagnostic dilemmas. Imaging techniques are of great value in diagnosis of this tumor. In this article we present the US, CT, MR imaging, scintigraphy, and angiography findings. The demonstration of a central vascular scar is very helpful. Although the radiologic features may be diagnostic, many atypical cases must be differentiated from other benign or malignant hepatic tumors. In these cases excisional biopsy and histopathologic examination are necessary to determine a definite diagnosis. (orig.)

  18. Focal nodular hyperplasia: imaging findings

    International Nuclear Information System (INIS)

    Kehagias, D.; Moulopoulos, L.; Antoniou, A.; Hatziioannou, A.; Smyrniotis, V.; Trakadas, S.; Lahanis, S.; Vlahos, L.

    2001-01-01

    Focal nodular hyperplasia is an uncommon benign hepatic tumor that continues to pose diagnostic dilemmas. Imaging techniques are of great value in diagnosis of this tumor. In this article we present the US, CT, MR imaging, scintigraphy, and angiography findings. The demonstration of a central vascular scar is very helpful. Although the radiologic features may be diagnostic, many atypical cases must be differentiated from other benign or malignant hepatic tumors. In these cases excisional biopsy and histopathologic examination are necessary to determine a definite diagnosis. (orig.)

  19. Nodular goiter after occupational accidental exposure to radiation

    Energy Technology Data Exchange (ETDEWEB)

    Pisarev, M.A. [Radiobiology, National Atomic Energy Commission, Buenos Aires (Argentina); Human Biochemistry, Uninversity of Buenos Aires, School of Medicine, Buenos Aires (Argentina); Schnitman, M. [Center of Endocrinology and Metabolism, French Hospital C.Milstein, Buenos Aires (Argentina)

    2012-07-01

    In the present paper we present the consequences of an accidental occupational radiation exposure at a local hospital in Buenos Aires. Control at a local radiology service showed the lack of correct shielding in the X-ray equipment. The physicians and technicians (14 persons) exposed to radiation during 12 months were examined. The survey shows that: a) In 11 out of 14 radiation-exposed patients nodular goiter developed and an additional patient had diffuse goiter which means a goiter incidence of 85.7%; b) In 5 of the nodular goiter patients an increase in the size or the appearance of new nodules was observed along the follow-up period. No cancer was detected by FNA; c) Hypothyroidism was observed in 3/14 patients, and an additional patient had an abnormal TRH-TSH test, suggesting subclinical hypothyroidism; and d) Increased circulating antithyroid antibodies were found in one of the hypothyroid patients

  20. Laparoscopic Partial Hepatectomy of Focal Nodular Hyperplasia

    Directory of Open Access Journals (Sweden)

    Mayu Sakata

    2012-11-01

    Full Text Available Focal nodular hyperplasia is a benign liver lesion incidentally discovered with increasing frequency because of the proliferation of imaging studies. Radiographic characterization can diagnose this pathologic lesion and nonoperative therapy is the standard of care. However, surgical resection may be required for diagnostic reasons or symptomatic patients. Depending on the anatomic location of the lesion, biopsy and/or resection can be performed laparoscopically. We herein report the case of a 26-year-old Japanese woman with a hepatic tumor who required a medical examination. Her medical history was negative for alcohol abuse, oral contraceptive administration and trauma. Clinical examination showed no significant symptoms. Ultrasonography, computed tomography and magnetic resonance imaging showed a mass located in the left lateral segment of the liver with a diameter of about 40 mm. It was difficult to diagnose the tumor definitively from these imaging studies, so we performed laparoscopic partial hepatectomy with successive firing of endoscopic staplers. The histopathological diagnosis was focal nodular hyperplasia. Surgical procedures and postoperative course were uneventful and the patient was discharged from the hospital on postoperative day 5.

  1. A comparison of nodular defect seed geometeries from different deposition techniques

    International Nuclear Information System (INIS)

    Stolz, C.J.; Tench, R.J.; Kozlowski, M.R.; Fornier, A.

    1995-01-01

    A focused ion-beam milling instrument commonly utilized in the semiconductor industry for failure analysis and IC repair, is capable of cross-sectioning nodular defects. Utilizing the instrument's scanning on beam, high-resolution imaging of the seeds that initiate nodular defect growth is possible. In an attempt to understand the origins of these seeds, HfO 2 /SiO 2 and Ta 2 O 5 /SiO 2 coatings were prepared by a variety of coating vendors and different deposition processes including e-beam, magnetron sputtering, and ion beam sputtering. By studying the shape, depth, and composition of the seed, inferences of its origin can be drawn. The boundaries between the nodule and thin film provide insight into the mechanical stability of the nodule. Significant differences in the seed composition, geometry of nodular growth and mechanical stability of the defects for sputtered versus e-beam coatings are reported. Differences in seed shape were also observed from different coating vendors using e-beam deposition of HfO 2 /SiO 2 coatings

  2. Mucosal surface nodularity on upper gastrointestinal series (UGIS) : prospective analysis of its primary cause and prevalence of gastric malignancy

    Energy Technology Data Exchange (ETDEWEB)

    Park, Soo Youn; Kim, Sun Mi; Kim, Ah Young; Kim, Tae Kyoung; Kim, Pyo Nyun; Ha, Hyun Kwon [Univ. of Ulsan College of Medicine, Seoul (Korea, Republic of)

    2001-10-01

    Mucosal surface nodularity was defined as present at UGIS when multiple nodular defects larger than 5 mm were scattered in the gastric mucosa in an area greater than 5 x 5 cm. The purpose of this study was to determine the primary causes of this radiographic finding and to assess the incidence of gastric malignancy in these patients. During a one-year period were prospectively collected among patients who underwent UGIS, data for 51 [aged 30-78 (mean, 51) years] above who met the criteria of mucosal surface nodularity. Whether or not this was present was decided by two radiologists who in reaching a consensus excluded the possibility of erosive gastritis, indicated by central barium collection in the nodular defects. The primary causes of mucosal nodularity and associated gastric pathologies were determined by the histopathological results obtained from the specimens after surgery (n=18) or endoscopic biopsy (n=33). Pathological examinations revealed that the primary causes of the mucosal nodularity in these 51 patients were intestinal metaplasia in 28 (54.9%), MALT lymphoma in seven (13.7%), early gastric cancer in six (11.8%), chronic gastritis in five (9.8%), low grade dysplasia in four (7.8%), and gastritis cystica profunda in one (2%). Gastric malignancy was present either in or outside the area of mucosal nodularity in 34 (66/7%) of the 51 (27 carcinomas and 7 MALT lymphomas). No different patterns of mucosal surface nodularity were noted between the groups of each disease entity. Mucosal surface nodularity is observed at UGIS in various gastric pathologies. Because of the high incidence of gastric malignancy in these patients, close follow-up or gastrofiberscopic biopsy is mandatory.

  3. Mucosal surface nodularity on upper gastrointestinal series (UGIS) : prospective analysis of its primary cause and prevalence of gastric malignancy

    International Nuclear Information System (INIS)

    Park, Soo Youn; Kim, Sun Mi; Kim, Ah Young; Kim, Tae Kyoung; Kim, Pyo Nyun; Ha, Hyun Kwon

    2001-01-01

    Mucosal surface nodularity was defined as present at UGIS when multiple nodular defects larger than 5 mm were scattered in the gastric mucosa in an area greater than 5 x 5 cm. The purpose of this study was to determine the primary causes of this radiographic finding and to assess the incidence of gastric malignancy in these patients. During a one-year period were prospectively collected among patients who underwent UGIS, data for 51 [aged 30-78 (mean, 51) years] above who met the criteria of mucosal surface nodularity. Whether or not this was present was decided by two radiologists who in reaching a consensus excluded the possibility of erosive gastritis, indicated by central barium collection in the nodular defects. The primary causes of mucosal nodularity and associated gastric pathologies were determined by the histopathological results obtained from the specimens after surgery (n=18) or endoscopic biopsy (n=33). Pathological examinations revealed that the primary causes of the mucosal nodularity in these 51 patients were intestinal metaplasia in 28 (54.9%), MALT lymphoma in seven (13.7%), early gastric cancer in six (11.8%), chronic gastritis in five (9.8%), low grade dysplasia in four (7.8%), and gastritis cystica profunda in one (2%). Gastric malignancy was present either in or outside the area of mucosal nodularity in 34 (66/7%) of the 51 (27 carcinomas and 7 MALT lymphomas). No different patterns of mucosal surface nodularity were noted between the groups of each disease entity. Mucosal surface nodularity is observed at UGIS in various gastric pathologies. Because of the high incidence of gastric malignancy in these patients, close follow-up or gastrofiberscopic biopsy is mandatory

  4. Pathophysiological analyses of leptomeningeal heterotopia using gyrencephalic mammals.

    Science.gov (United States)

    Matsumoto, Naoyuki; Kobayashi, Naoki; Uda, Natsu; Hirota, Miwako; Kawasaki, Hiroshi

    2018-03-15

    Leptomeningeal glioneuronal heterotopia (LGH) is a focal malformation of the cerebral cortex and frequently found in patients with thanatophoric dysplasia (TD). The pathophysiological mechanisms underlying LGH formation are still largely unclear because of difficulties in obtaining brain samples from human TD patients. Recently, we established a new animal model for analysing cortical malformations of human TD by utilizing our genetic manipulation technique for gyrencephalic carnivore ferrets. Here we investigated the pathophysiological mechanisms underlying the formation of LGH using our TD ferrets. We found that LGH was formed during corticogenesis in TD ferrets. Interestingly, we rarely found Ki-67-positive and phospho-histone H3-positive cells in LGH, suggesting that LGH formation does not involve cell proliferation. We uncovered that vimentin-positive radial glial fibers and doublecortin-positive migrating neurons were accumulated in LGH. This result may indicate that preferential cell migration into LGH underlies LGH formation. Our findings provide novel mechanistic insights into the pathogenesis of LGH in TD.

  5. Juvenile muscular atrophy of the distal upper extremities associated with x-linked periventricular heterotopia with features of Ehlers-Danlos syndrome.

    Science.gov (United States)

    Hommel, Alyson L; Jewett, Tamison; Mortenson, Megan; Caress, James B

    2016-10-01

    Juvenile muscular atrophy of the distal upper extremities (JMADUE) is a rare, sporadic disorder that affects adolescent males and is characterized by progressive but self-limited weakness of the distal upper extremities. The etiology is unknown, but cervical hyperflexion has been hypothesized. We report a case of an adolescent male who presented with typical JMADUE but also had joint hypermobility and multiple congenital anomalies, including periventricular heterotopias, suggesting a multisystem syndrome. Subsequent diagnostic testing confirmed a diagnosis of JMADUE, and sequencing of the filamin-A gene showed a novel, pathogenic mutation that confirmed an additional diagnosis of X-linked periventricular heterotopias with features of Ehlers-Danlos syndrome (XLPH-EDS). The concurrent diagnosis of these 2 rare conditions suggests a pathogenic connection. It is likely that the joint hypermobility from XLPH-EDS predisposed this patient to developing JMADUE. This supports the cervical hyperflexion theory of pathogenesis. This case also expands the phenotype associated with FLNA mutations. Muscle Nerve 54: 794-797, 2016. © 2016 Wiley Periodicals, Inc.

  6. Idiopathic nodular glomerulosclerosis (ING) in an African American (AA) man with hepatitis C.

    Science.gov (United States)

    Onteddu, Nirmal K; Duggirala, Jayasri; Reddy, Anand C

    2018-05-18

    Idiopathic nodular glomerulosclerosis (ING) in a non-diabetic patient is uncommon. Nodular glomerulosclerosis is hallmark sign of diabetic nephropathy. ING is a very rare clinicopathological disease associated with smoking, obesity and hypertension, chronic obstructive pulmonary disease and metabolic syndrome. A 68-year-old non-obese African American man with hypertension, smoking and history of hepatitis C presented to the clinic with progressive worsening of lower extremity oedema and declining renal function over few months. Renal biopsy demonstrated nodular glomerulosclerosis. In this case, ING is hypothesised to be associated with hepatitis C along with smoking and hypertension (HTN). We present this case to speculate the existence of yet unknown aetiologies of ING. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  7. Thyroid cancers in nodular goitres in Northwestern Nigeria

    African Journals Online (AJOL)

    BACKGROUND: Cancer is an occasional incidental finding in nodular goitres, but there has been no formal study on this disease in our centre which is located in an endemic goitrous region. AIMS AND OBJECTIVES: To determine the prevalence and pattern of malignant growths in multinodular goitres. METHODOLOGY: ...

  8. Periventricular heterotopia in a boy with interstitial deletion of chromosome 4p.

    Science.gov (United States)

    Gawlik-Kuklinska, Katarzyna; Wierzba, Jolanta; Wozniak, Agnieszka; Iliszko, Mariola; Debiec-Rychter, Maria; Dubaniewicz-Wybieralska, Miroslawa; Limon, Janusz

    2008-01-01

    We report on a 4-year-old boy with a proximal interstitial deletion in the short arm of chromosome 4p with the karyotype 46,XY,del(4)(p14p15.32),inv(9)(p13q13). For a precise delineation of the deleted region, an array-based comparative genomic hybridization (a-CGH) analysis was performed. The proband's phenotype and cytogenetic findings are compared with previously reported cases with proximal 4p deletion syndrome. The syndrome is associated with normal growth, varying degrees of mental retardation, characteristic facial appearance and minor dysmorphic features. Additionally, our patient developed a seizure disorder due to abnormal neuronal migration, i.e., periventricular heterotopia.

  9. Giant Solitary Nodular Trichoepithelioma: A Case Report and Review of Literature

    Directory of Open Access Journals (Sweden)

    Sunder Goyal

    2012-02-01

    Full Text Available A giant solitary nodular trichoepithelioma (GST is a rare trichogenic tumor, which may present as a pigmented lesion. A 45-year-old female was diagnosed as having a giant solitary nodular trichoepithelioma on her right forearm. About 11 cases have been reported in literature. Our case is the 2nd largest of all reported cases and, so far, GST of the forearm has not been reported in literature. The recognition of GST is important because of its close resemblance to basal cell carcinoma and other skin adnexal tumors, both clinically and histopathologically. [Arch Clin Exp Surg 2012; 1(1.000: 58-60

  10. Nodular hidradenocarcinoma with prominent squamous differentiation: case report and immunohistochemical study.

    Science.gov (United States)

    Park, H J; Kim, Y C; Cinn, Y W

    2000-09-01

    We report the case of a 24-year-old woman with nodular hidradenocarcinoma on the scalp. While histopathology of the tumor showed a circumscribed, lobulated intradermal mass with prominent squamous differentiation, the immunohistochemical study with antibodies to cytokeratins, CAM 5.2 and 19, epithelial membrane antigen, carcinoembryonic antigen, S-100 protein and p53 all demonstrated positivity. These findings confirmed that the tumor was of eccrine sweat gland origin and it was thought to be a nodular hidradenocarcinoma differentiating toward the eccrine duct and/or secretory portions. She was treated with a wide local excision and no recurrence was observed 18 months after excision.

  11. Cushing syndrome in a young woman due to primary pigmented nodular adrenal disease.

    Science.gov (United States)

    Hackman, Kathryn L; Davis, Anna L; Curnow, Paul A; Serpell, Jonathan W; McLean, Catriona A; Topliss, Duncan J

    2010-01-01

    To report a case of Cushing syndrome due to apparently sporadic primary pigmented nodular adrenal disease in a young woman. We describe the clinical, biochemical, radiologic, and histologic findings of Cushing syndrome due to the rare condition of primary pigmented nodular adrenal disease. A 30-year-old woman presented with a 2-year history of worsening itch without rash over her shoulders and arms and weight gain, particularly around the abdomen and face. Careful questioning did not elicit any history of exogenous glucocorticoid use (systemic or topical), including hydrocortisone. On examination, the patient had a slightly rounded and plethoric face, a small buffalo hump, central adiposity, and thin skin with a few small striae on her inner thighs. No features of the Carney complex were observed. Investigations showed hypercortisolism with suppressed corticotropin and normal adrenal imaging despite documentation of enlarged adrenal glands at removal. High-dose dexamethasone administration was followed by a decrease in urinary free cortisol excretion rather than a paradoxical rise as previously reported in primary pigmented nodular adrenal disease. No mutations were detected in the PRKAR1A gene. Primary pigmented nodular adrenal disease should be suspected in patients with corticotropin-independent Cushing syndrome who have normal adrenal imaging. The role of genetic testing in apparently sporadic cases is not established, but cumulative experience may be helpful in defining the frequency of PRKAR1A mutations.

  12. Characterization and growth mechanism of a peculiar nodular structure in shale: Comprehensive study over the Sitakund anticline, Bengal basin.

    Science.gov (United States)

    Gazi, M. Y.; Kabir, S. M. M.; Imam, M. B.

    2017-12-01

    Nodular shales commonly occur in comparatively older and silty shales near the axial (proximity to core) region of Sitakund Anticline (Study area), Sitapahar Anticline, Patharia Structure, Sylhet Anticline and Mirinja Anticline as observed. Stratigraphically, they are pronounced in the Surma group of Neogene succession. They are less abundant in limb portion. In many outcrop, they are found in the incompetent bed with the obliterated bedding bounded by well bedded competent beds. Their occurrence are sporadic rather than continuous along and across the strike of the bed. At some places huge number cluster of small and big nodular shales occur while in the other places, they occur as isolated mass in the highly disturbed or obliterated beds. The Surma group is the prime startigraphic unit in Bangladesh with major economic and academic importance. Yet there is a lack of comprehensive characterization of mudrocks of Surma group. This has prompted the present research to be undertaken. An initial field based study has been followed by detail textural, mineralogical, petrological and geochemical by using upscale laboratory techniques that include Thin Section Microscopy, Laser Particle Size Analyses, X-ray Diffraction (XRD), Scanning Electron Microscopy (SEM), and X-ray Florescence (XRF). From laser diffraction analysis, it is evident that nodular shales are silty in nature containing approximately 60% silt (Mainly quartz). XRD pattern shows that Nodular shale contains clay minerals, predominantly illite, Kaolinite, Chlorite and expandable mixed layer clay mineral. Detail geochemical analysis of some nodular shale samples shows that there are no significant variation from other samples in major and trace element concentration. Microcrack's within the quartz grains were observed in nodular shale. Projection of 15 nodular shale long axes in outcrop shows their orientation in NNW-SSE that is parallel to the fold axis. The study suggests a new name of conventionally called

  13. Clinical surgical and pathological characterization of nodular thyroid disease

    International Nuclear Information System (INIS)

    Gonzalez Mendoza, Dayanis Ceila; Rodriguez Valdes, Adriana; Bejerano Garcia, Ramiro Julio

    2012-01-01

    Nodular thyroid disease is a worrisome endocrine problem due to its association with cancer. To characterize patients hospitalized with this condition according to clinical surgical and pathological aspects, as well as to determine the effectiveness of the cytology by fine-needle aspiration biopsy for diagnosis

  14. Crystallization of nodular cast iron with carbides

    Directory of Open Access Journals (Sweden)

    S. Pietrowski

    2008-12-01

    Full Text Available In this paper a crystallization process of nodular cast iron with carbides having a different chemical composition have been presented. It have been found, that an increase of molybdenum above 0,30% causes the ledeburutic carbides crystallization after (γ+ graphite eutectic phase crystallization. When Mo content is lower, these carbides crystallize as a pre-eutectic phase. In this article causes of this effect have been given.

  15. Nodular calcified neurocysticercosis with signs of reactivation

    Energy Technology Data Exchange (ETDEWEB)

    Coeli, Gustavo Nunes Medina; Tiengo, Rodrigo Ribeiro; Silva, Guilherme Carlos da; Silva, Leandro Urquiza Marques Alves da, E-mail: gustavonmc@yahoo.com.br [Department of Radiology and Imaging Diagnosis, Hospital Escola de Itajuba, MG (Brazil); Silva, Afonso Carlos da [Medical Practice, Hospital Escola de Itajuba, MG (Brazil); Fernandes, Jose Otavio Meyer [Clinica Sul Mineira Tomosul and Clinica Magsul, Itajuba, MG (Brazil)

    2012-09-15

    Neurocysticercosis is a disease characterized by the involvement of the central nervous system by the intermediate larval stage of the parasite Taenia solium. The larva degeneration process and the inflammatory reaction of the body cause clinical symptoms. The authors report a case of clinical and radiological reactivation of nodular calcified neurocysticercosis in a patient who was asymptomatic for more than 20 years. Antiparasitic treatment showed a good response (author)

  16. Confluent focal nodular hyperplasia mimicking liver cancer: Value of liver-specific contrast-enhanced MRI for diagnosis

    Directory of Open Access Journals (Sweden)

    Yu-Chi Cheng

    2012-07-01

    Full Text Available Focal nodular hyperplasia is the second most common benign hepatic tumor. Unlike adenoma as well as the malignant neoplasms, focal nodular hyperplasia can often be managed successfully without surgery. Use of liver-specific contrast-enhanced magnetic resonance imaging allows clinicians to confirm the diagnosis noninvasively in some patients, allowing select patients to avoid surgery. We report a case of a patient who presented with the rare profile of multiple, confluent lesions that were diagnosed, using magnetic resonance imaging with gadolinium-dimeglumine, as focal nodular hyperplasia. This complicated case was managed successfully and noninvasively based on algorithm found in the recent literature that allows patients to avoid unnecessary surgery.

  17. The clinical application of percutaneous auto-biopsy of small lung nodular under CT-Guided

    International Nuclear Information System (INIS)

    Zhou Yuanmin; Ye Genxin; Zhang Chenghui; Wang Yu; Chen Wei

    2008-01-01

    Objective: To evaluated the clinical value of technology of petcutaneous auto-biopsy of small lung nodular under CT- Guide. Methods: 44 cases of small single lung nodular were underwent biopsy with 20G auto-biopsy needle under CT guidance. All cases underwent pathological diagnosis. Results: All 44 cases were punctured successfully. 41 cases were succeeded in first puncturation. The success ratio was 93.02%. Other 3 cases needed second puncturation. 39 of 44 cases pathological diagnosis were malignant. Only 1 case could not be diagnosed. 6 patients had lightly pneumatothorax after biopsy. 10 cases had mild pneumonorrhagia after biopsy. 2 of them had haemptysis. All cases had no complication such as infection, needle track implantation. Conclusion: The technology of CT Guidance auto-biopsy of small lung nodular is safe and effective; it has extreme diagnostic ratio and less complication. (authors)

  18. Thyroid Hormone-Dependent Formation of a Subcortical Band Heterotopia (SBH) in the Neonatal Brain is not Exacerbated Under Conditions of Low Dietary Iron

    Science.gov (United States)

    Thyroid hormones (TH) are critical for brain development. Modest TH insufficiency in pregnant rats induced by propylthiouracil (PTU) results in formation of a structural abnormality, a subcortical band heterotopia (SBH), in brains of offspring. PTU reduces TH by inhibiting the s...

  19. Focal nodular hyperplasia of the liver: an unusual association with diabetes mellitus in a child and review of literature

    Directory of Open Access Journals (Sweden)

    Trizzino Antonino

    2010-05-01

    Full Text Available Abstract Hepatic hemangioma, adenoma and focal nodular hyperplasia are the most frequent benign lesions of the liver, but they are all infrequent among pediatric population. The reports of focal nodular hyperplasia in children have recently increased in number, with many cases associated to drug intake, particularly to chemotherapy. We here describe, to our knowledge, the first case of focal nodular hyperplasia in association with diabetes mellitus in childhood.

  20. The Rare Benign Lesion That Mimics a Malignant Tumor in Breast Parenchyma: Nodular Fasciitis of the Breast

    Directory of Open Access Journals (Sweden)

    Hilal Erinanc

    2018-01-01

    Full Text Available We herein report the clinical and pathological findings of a rare case of nodular fasciitis in the breast parenchyma of a 48-year-old female. Because of potentially malignant findings on ultrasonography and during clinical examination, the patient underwent an excisional biopsy. Histologically, the lesion was composed of spindle to round shaped cells arranged in short bundles in a storiform pattern. Immunohistochemically, the cells were positive for vimentin and SMA and negative for desmin, S100, and CD34. Based on these morphological and immunohistochemical features, a diagnosis of nodular fasciitis was made. We emphasize that nodular fasciitis of the breast may show clinical features and imaging findings similar to those of breast cancer. The histopathologic diagnosis of nodular fasciitis can also be challenging. The purpose of this case report is to highlight the characteristics and the differential diagnosis of this rare neoplasm.

  1. Congenital nasal neuroglial heterotopia and encephaloceles: An update on current evaluation and management.

    Science.gov (United States)

    Adil, Eelam; Robson, Caroline; Perez-Atayde, Antonio; Heffernan, Colleen; Moritz, Ethan; Goumnerova, Liliana; Rahbar, Reza

    2016-09-01

    To describe our experience and current management approach for congenital nasal neuroglial heterotopia (NGH) and encephaloceles. Retrospective chart review at a tertiary pediatric hospital from 1970 to 2013. Thirty patients met inclusion criteria: 21 NGH and nine encephaloceles. Data including demographics, pathology, imaging modality, surgical approach, resection extent, outcomes, and complications were analyzed. Fourteen NGH patients (67%) presented with an internal nasal mass and nasal obstruction. Three patients (14%) presented with an external nasal mass and four (19%) had a mixed lesion. Median age at surgery was 0.51 years (interquartile range 1.32 years). Thirteen (62%) had an intranasal endoscopic approach. Median operative time was 1.6 hours (interquartile range 1.2 hours), and there were no major complications. Nine patients with encephalocele were identified: six (67%) presented with transethmoidal encephaloceles, two (22%) presented with nasoethmoidal encephaloceles, and one (11%) presented with a nasofrontal lesion. The median age at surgery was 1.25 years (interquartile range 1.4 years). All patients required a craniotomy for intracranial extension. Median operative time was 5 hours (interquartile range 1.9 hours), and eight patients (88%) had a total resection. Length of stay ranged from 3 to 14 days. Nasal neuroglial heterotopia and encephaloceles are very rare lesions that require multidisciplinary evaluation and management. At our institution, there has been a shift to magnetic resonance imaging alone for the evaluation of NGH to avoid radiation exposure. Endoscopic extracranial resection is feasible for most intranasal and mixed NGH without an increase in operative time, residual disease, or complications. 4. Laryngoscope, 126:2161-2167, 2016. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

  2. Adrenal cortical heterotopia in an inguinal hernia sac of an adult: A case report and literature review

    Directory of Open Access Journals (Sweden)

    Sarah S. Kassaby, MD

    2017-06-01

    Full Text Available Ectopic adrenal cortical tissue is not an infrequent incidental finding during abdominal and inguinal operations in infants; however, it is a rare finding in adults with only a few case reports described in the literature. We report a case of adrenal heterotopia as an incidental finding in a hernia sac from a 56 year-old man. We review the literature and discuss the importance of recognizing this rare finding.

  3. Nodular Lymphangitis in HIV-Infected Patients in Tanzania | Mapesi ...

    African Journals Online (AJOL)

    Early diagnosis, biopsy or culture of skin lesions and treatment are essential for improving outcomes. However, this is challenging in resource-limited settings. We present two HIV-infected patients with nodular lymphangitis treated with ketoconazole in the absence of itraconazole or amphotericin B with good initial response ...

  4. Intra-articular Nodular Fasciitis: An Unexpected Diagnosis for a Joint Lesion: A Case Report

    Directory of Open Access Journals (Sweden)

    MF Michelle Chan

    2014-07-01

    Full Text Available Pathological lesions in and around a joint can arise from underlying dermis, subcutis, deep muscle, bone or synovium. Clinical presentation can include joint pain, joint swelling, palpable masses and mechanical restriction. Whilst giant cell tumour of tendon sheath, pigmented villonodular synovitis, synovial chondromatosis, lipoma arborescens, juxta articular myxomas and inflammatory arthritis are the better-known conditions of the joint. Intra-articular nodular fasciitis, on the other hand, is less well recognized both clinically and radiologically. It is rarely seen in routine practice and is only described in case reports in the literature. Due to the non-specific clinical and radiological findings as well as the unfamiliarity with the entity, the diagnosis of intra-articular nodular fasciitis is usually clinched only after histological examination. We present a case of intra-articular nodular fasciitis arising in the knee joint which was not suspected clinically or radiologically.

  5. Nodular hidradenocarcinoma on the scalp of a young woman: case report and review of literature.

    Science.gov (United States)

    Ohta, Masayoshi; Hiramoto, Michiaki; Fujii, Miki; Togo, Takeshi

    2004-09-01

    Nodular hidradenocarcinoma was first reported as clear-cell eccrine carcinoma by Keasby and Hadley in 1954 (Cancer 1954;7:934-52) and rare malignant tumor. Several synonyms and related terms for nodular hidradenocarcinoma have appeared in the literature. They have potential for uncontrollable local recurrence, tend to metastasize, and often cause death. Most cases have been reported in the pathology literature with limited clinical information. We report a 27-year-old woman with nodular hidradenocarcinoma on the scalp. The management of rare cases is not well defined. In our case, she was only treated with a wide local excision, and no recurrence was observed 2 years after excision. Most authors have concluded that early wide surgical excision of the tumor is the treatment of choice. The efficiency of adjuvant therapy generally has not established.

  6. Nodular hidradenoma and hidradenocarcinoma. A 10-year review.

    Science.gov (United States)

    Hernández-Pérez, E; Cestoni-Parducci, R

    1985-01-01

    We studied eighty-nine nodular hidradenomas (NHs) and six clear cell hidradenocarcinomas (CCHs) reported in a 10-year period. NHs were more commonly seen in women (ratio of 1.7 to 1); the average age was 37.2 years, and they were located mainly on the head. One CCH had widely disseminated metastasis that led to death. Other CCHs underwent spontaneous regression.

  7. Violations of macro- and micronutrient support in patients with nodular thyroid pathology from regions injured after the Chornobyl accident

    Directory of Open Access Journals (Sweden)

    V.I. Kravchenko

    2017-09-01

    Full Text Available Background. The purpose of our study was to determine the state of macro- and micronutrient support of patients with nodular thyroid pathology from the regions injured after the Chornobyl accident. Materials and methods. 65 habitants of the Chernihiv area were examined: 40 — without thyroid pathology (15 men and 25 women aged 36.62 ± 0.60 years, and 25 (5 men and 20 women — with the diagnosed nodular goiter. Results. Research of urinary iodine excretion showed that a median in the control group was 95.6 µg/l, among patients with nodular goiter — 89.3 µg/l, indicating the presence of moderate iodine deficiency. Blood thyroglobulin level in patients of control group was 9.26 ± 0.99 ng/l, among patients with nodular goiter — 23.03 ± 4.70 ng/l. Thyroid-stimulating hormone level higher than 4.0 mIU/l was in 8.9 % of patients with nodular goiter and in 5.0 % of persons from the control group. The data of ultrasonic researches demonstrated that the average thyroid volume in patients of control group was 10.1 ± 0.3 cm3, among patients with nodular goiter — 18.6 ± 1.7 cm3. In the group of patients with nodular goiter, the decreased (p < 0.0001 level of macronutrients in blood plasma was detected: calcium was 67.26 ± 3.21 µg/l and magnesium — 14.88 ± 0.35 µg/l, as well as of microelements: zinc content was 0.72 ± 0.04 µg/l (p < 0.0001 and iron — 0.54 ± 0.06 µg/l (p < 0.05, in comparison with the results in the control group. Conclusions. The relative risk (χ2 criterion for the four-course table of connectivity with Fisher’s correction of nodular goiter development at subzero content of calcium was 2.24 (95% confidence interval (CI 1.58–2.61, the risk of nodular goiter development at subzero levels of magnesium — 2.56 (95% CI 1.77–3.03.

  8. Cistadenocarcinoma renal e dermatofibrose nodular em cães pastor alemão: 4 casos Renal cystadenocarcinoma and nodular dermatofibrosis in german shepherd dogs: 4 cases

    Directory of Open Access Journals (Sweden)

    Ingeborg Maria Langohr

    2002-08-01

    Full Text Available São descritos quatro casos da síndrome cistadenocarcinoma renal-dermatofibrose nodular em Pastor Alemão. Todos eram cães machos, com idades entre 6 e 10 anos. Os aspectos clínicos incluíam hematúria e retenção urinária, emagrecimento progressivo, anorexia, vômito, polidipsia e dificuldade respiratória. Em um animal, foram observados múltiplos nódulos recidivantes na pele. Três cães tiveram morte espontânea e um foi submetido à eutanásia. Os principais achados de necropsia incluíram nódulos multifocais ou coalescentes na pele e múltiplos nódulos, muitas vezes císticos, em ambos os rins. Na avaliação histológica, observaram-se dermatofibrose nodular na pele e cistos, hiperplasia, displasia e neoplasia de células epiteliais dos túbulos renais. As neoplasias foram classificadas como cistadenoma em um dos casos e como cistadenocarcinoma nos outros três. Metástases foram observadas em dois casos: no fígado, no baço e no pulmão em um cão e no linfonodo renal em outro.Renal cystadenocarcinoma-nodular dermatofibrosis syndrome of German shepherd are described in four 6 to 10-year-old male dogs. Clinical findings included hematuria and urinary retention, progressive weight loss, polydipsia, anorexia, vomiting, and respiratory distress. Several recurrent skin nodules were observed in one of the dogs. Three dogs died spontaneously and one was euthanatized. Main gross findings included multifocal to coalescent skin nodules and multiple nodules, mostly cystic, in both kidneys. Microscopically there was nodular dermatofibrosis in the skin and renal cysts, dysplastic, hyperplastic and neoplastic changes of the renal tubular epithelium. The neoplasms were classified as cystadenoma in one case and cystadenocarcinoma in the other three. There were metastases in the liver, spleen and lung of one dog and in the renal lymph node in another one.

  9. Nodular Purpura and Intracranial Bleeding due to Late Onset ...

    African Journals Online (AJOL)

    She developed nodular purpuric spots for about three days prior to the intracranial bleeding. She was unconscious and responding only to pain, and was severely pale. She was transfused with two units of whole blood and underwent a craniotomy for draining the hemorrhage. She was treated with Vitamin K supplements.

  10. Papulo-Nodular Reactions in Black Tattoos as Markers of Sarcoidosis: Study of 92 Tattoo Reactions from a Hospital Material.

    Science.gov (United States)

    Sepehri, Mitra; Hutton Carlsen, Katrina; Serup, Jørgen

    2016-01-01

    Sarcoidosis is, from historical data, suggested to be more prevalent among patients with tattoo reactions. We aimed to evaluate this association in a systematic study. This is a consecutive study of patients with tattoo complications, diagnosed in the "Tattoo Clinic" at Bispebjerg University Hospital in Copenhagen, Denmark, from 2008 to 2015, based on clinical assessment and histology. From the overall group of 494 tattoo complications in 406 patients, 92 reactions in 72 patients showed a papulo-nodular pattern studied for local and systemic sarcoidosis, since sarcoidosis is expected to be nodular. Of the 92 reactions with a papulo-nodular pattern, 27 (29%) reactions in 19 patients were diagnosed as cutaneous or systemic sarcoidosis, supported by histology; 65 (71%) were diagnosed as non-sarcoidosis due to histology and no clinical sarcoid manifestations. "Rush phenomenon" with concomitant reaction in many other black tattoos, triggered by a recent tattoo with a papulo-nodular reaction, was observed in 70% in the sarcoidosis group and 28% in the non-sarcoidosis group, indicating a predisposing factor which may be autoimmune and linked with sarcoidosis. Agglomerates of black pigment forming foreign bodies may in the predisposed individual trigger widespread reaction in the skin and internal organs. Black tattoos with papulo-nodular reactions should be seen as markers of sarcoidosis. Papulo-nodular reactions may, as triggers, induce widespread reactions in other black tattoos - a "rush phenomenon" - depending on individual predisposition. Sarcoidosis is estimated to be 500-fold increased in papulo-nodular reactions compared to the prevalence in the general population, and the association with black tattoos is strong. © 2017 S. Karger AG, Basel.

  11. Animal models of disease: feline hyperthyroidism: an animal model for toxic nodular goiter.

    Science.gov (United States)

    Peterson, Mark E

    2014-11-01

    Since first discovered just 35 years ago, the incidence of spontaneous feline hyperthyroidism has increased dramatically to the extent that it is now one of the most common disorders seen in middle-aged to senior domestic cats. Hyperthyroid cat goiters contain single or multiple autonomously (i.e. TSH-independent) functioning and growing thyroid nodules. Thus, hyperthyroidism in cats is clinically and histologically similar to toxic nodular goiter in humans. The disease in cats is mechanistically different from Graves' disease, because neither the hyperfunction nor growth of these nodules depends on extrathyroidal circulating stimulators. The basic lesion appears to be an excessive intrinsic growth capacity of some thyroid cells, but iodine deficiency, other nutritional goitrogens, or environmental disruptors may play a role in the disease pathogenesis. Clinical features of feline toxic nodular goiter include one or more palpable thyroid nodules, together with signs of hyperthyroidism (e.g. weight loss despite an increased appetite). Diagnosis of feline hyperthyroidism is confirmed by finding the increased serum concentrations of thyroxine and triiodothyronine, undetectable serum TSH concentrations, or increased thyroid uptake of radioiodine. Thyroid scintigraphy demonstrates a heterogeneous pattern of increased radionuclide uptake, most commonly into both thyroid lobes. Treatment options for toxic nodular goiter in cats are similar to that used in humans and include surgical thyroidectomy, radioiodine, and antithyroid drugs. Most authorities agree that ablative therapy with radioiodine is the treatment of choice for most cats with toxic nodular goiter, because the animals are older, and the disease will never go into remission. © 2014 Society for Endocrinology.

  12. Report of a case of Hodgkin's nodular sclerosis disease

    International Nuclear Information System (INIS)

    Canizares, Claudio; Araujo, Ivan; Flores, Marlon; Guerrero, Patricia; Sanchez, Mayra

    2004-01-01

    We report a case of Hodgkin's nodular sclerosis disease that presents moderate anemia and lymph node enlargement with a massive infiltrate to the bone marrow. It is rare for these patients to develop such an infiltrate, which give a bad prognosis. The treatment recommended in stage I is Radiotherapy and chemotherapy. (The author)

  13. Primary Pulmonary Mucosa-Associated Lymphoid Tissue Lymphoma with a Nodular Opacity: Report of a Case.

    Science.gov (United States)

    Yoshino, Naoyuki; Hirata, Tomomi; Takeuchi, Chie; Usuda, Jitsuo; Hosone, Masaru

    2017-01-01

    Herein, we describe our experience in treating a case of primary pulmonary mucosa-associated lymphoid tissue lymphoma detected as a nodular opacity. A 79-year-old man was referred to our hospital. Computed tomography showed a nodular opacity measuring 20 mm in diameter with regular margins in segment 5 of the right middle lobe of the lung. Although the bronchoscopic brush cytology result was class III, the patient was tentatively diagnosed with suspected mucosa-associated lymphoid tissue lymphoma. A thoracoscopic right middle lobectomy was performed. The pathological findings showed nodular proliferation of small to medium-sized, mature-appearing atypical lymphoid cells, lymphoepithelial lesions, and vague follicles suggesting follicular colonization in some areas. The patient was diagnosed with low-grade small B-cell lymphoma and mucosa-associated lymphoid tissue lymphoma. He has remained well to date, 23 months after surgery, without evidence of recurrence.

  14. Sentinel lymph node biopsy: is it possible to reduce false negative rates by excluding patients with nodular melanoma?

    LENUS (Irish Health Repository)

    Corrigan, M A

    2012-02-03

    OBJECTIVE: The aim of this study was to review the outcome of sentinel lymph node biopsy (SLNB) in patients with melanoma and to delineate whether patients with nodular melanoma are more likely to develop nodal recurrence despite negative SLNB. METHODS: Consecutive patients with cutaneous melanoma undergoing SLNB were identified from a departmental database between 1997 and 2005. Factors including demographic data, site, histological subtype, depth and outcome were examined. RESULTS: Of 131 patients, 103 were node negative and eligible for study. The median age was 53 (16-82) years with 46 patients being male (45%) and 57 female (55%). Primary melanoma sites included lower limb (49; 48%), upper limb (29; 28%), head (12; 11%), trunk (7; 7%) and back (6; 6%). The median Breslow thickness was 2mm. Superficial spreading accounted for 43% of melanoma with nodular accounting for 42%. Median follow-up was 40 (3-90) months. Of 20 relapses, seven recurred in the same nodal basin, three were satellite recurrences, one recurred with both satellite and nodal lesions simultaneously, and nine experienced haematogenous spread. Of the eight patients who developed recurrence in the same nodal basin, four were of nodular histological subtype (p=NS). All of the three patients with satellite lesions had nodular melanoma histologically (p=0.02). When nodal and satellite recurrences were combined, eight of 11 were histologically nodular (p=0.01). CONCLUSIONS: This study indicates that lymphatic recurrence occurs more often in SLNB negative patients with nodular melanoma. Further evaluation of the inclusion criteria for sentinel node biopsy is warranted.

  15. [Analysis of predictors of malignancy of nodular goiters: about 500 cases].

    Science.gov (United States)

    Bouaity, Brahim; Darouassi, Youssef; Chihani, Mehdi; Touati, Mohamed Mliha; Ammar, Haddou

    2016-01-01

    Thyroid nodules are very common and less than 10% of them are malignant. They pose a serious diagnostic and therapeutic problem with respect to their benign or malignant nature. The study of some clinical and paraclinical factors for presumed malignancy makes it possible to codify appropriate therapeutic strategy. The aim of this study was to investigate predictors of malignancy in nodular goiters and to compare our results with those reported in the literature. This retrospective study consisted of 500 cases of nodular goiter operated in the Department of Otorhinolaryngology (ear, nose and throat or ENT) and Head and Neck Surgery at Avicenne military hospital in Marrakech between 2006 and 2012. The percentage of cancers was 6,8%. The average age of our patients was 46 years, with a sex-ratio of 5 (F/H). The hard nature of the nodule was present in 94,4% of the cases of cancer by palpation; irregular boundaries were present in 64.70% of the cases of cancer. Three nodules were fixed and malignant. Cervical lymphadenopathy were observed in 8 patients, 7 of which had cancer. Ultrasound examination showed hypoechoic appearance in 61,8% of the malignant nodules, with smooth-edges in 88,24% of the cases. Intranodular vascularization was found in 35,3% of the cases of cancers, with microcalcifications in 55,9% of the cases. Perinodular hypoechoic halo was incomplete in 73,5% of the cases of cancer. Our patients were euthyroid in 84,6% of the cases. Predictors of malignancy in nodular goiters were present in our first clinical study: patients over the age of 60 years, hard consistency of nodule, fixity, irregular and poorly defined character by palpation, as well as presence of cervical adenopathy on examination; and echographic features: hypoechoic character, smooth-edges, presence of microcalcifications and visualization of intranodular vascularization with or without peri-nodular vascularization. Although some of these factors are highly predictive of malignancy, only

  16. Budd-Chiari syndrome and secondary nodular regenerative hyperplasia of the liver. Case report with special reference to diagnostic imaging

    International Nuclear Information System (INIS)

    Mutze, A.; Rueckert, R.; Rudolph, B.; Paris, S.; Podrabski, P.

    1993-01-01

    Nodular regenerative hyperplasia is a benign epithelial proliferation of the liver with unknown etiology. We observed a female patient with Budd-Chiari syndrome and secondary nodular regenerative hyperplasia of the liver over a period of five years. Patient history, diagnostic imaging (sonography, CT, MR imaging, angiography), and clinical course are demonstrated along with results of macroscopic and microscopic studies of explanted liver prior to liver transplantation. The patient presented with various predisposing factors in combination that favour the development of nodular regenerative hyperplasia. (orig.) [de

  17. Disruption of neural progenitors along the ventricular and subventricular zones in periventricular heterotopia

    Science.gov (United States)

    Ferland, Russell J.; Batiz, Luis Federico; Neal, Jason; Lian, Gewei; Bundock, Elizabeth; Lu, Jie; Hsiao, Yi-Chun; Diamond, Rachel; Mei, Davide; Banham, Alison H.; Brown, Philip J.; Vanderburg, Charles R.; Joseph, Jeffrey; Hecht, Jonathan L.; Folkerth, Rebecca; Guerrini, Renzo; Walsh, Christopher A.; Rodriguez, Esteban M.; Sheen, Volney L.

    2009-01-01

    Periventricular heterotopia (PH) is a disorder characterized by neuronal nodules, ectopically positioned along the lateral ventricles of the cerebral cortex. Mutations in either of two human genes, Filamin A (FLNA) or ADP-ribosylation factor guanine exchange factor 2 (ARFGEF2), cause PH (Fox et al. in ‘Mutations in filamin 1 prevent migration of cerebral cortical neurons in human periventricular heterotopia'. Neuron, 21, 1315–1325, 1998; Sheen et al. in ‘Mutations in ARFGEF2 implicate vesicle trafficking in neural progenitor proliferation and migration in the human cerebral cortex'. Nat. Genet., 36, 69–76, 2004). Recent studies have shown that mutations in mitogen-activated protein kinase kinase kinase-4 (Mekk4), an indirect interactor with FlnA, also lead to periventricular nodule formation in mice (Sarkisian et al. in ‘MEKK4 signaling regulates filamin expression and neuronal migration'. Neuron, 52, 789–801, 2006). Here we show that neurons in post-mortem human PH brains migrated appropriately into the cortex, that periventricular nodules were primarily composed of later-born neurons, and that the neuroependyma was disrupted in all PH cases. As studied in the mouse, loss of FlnA or Big2 function in neural precursors impaired neuronal migration from the germinal zone, disrupted cell adhesion and compromised neuroepithelial integrity. Finally, the hydrocephalus with hop gait (hyh) mouse, which harbors a mutation in Napa [encoding N-ethylmaleimide-sensitive factor attachment protein alpha (α-SNAP)], also develops a progressive denudation of the neuroepithelium, leading to periventicular nodule formation. Previous studies have shown that Arfgef2 and Napa direct vesicle trafficking and fusion, whereas FlnA associates dynamically with the Golgi membranes during budding and trafficking of transport vesicles. Our current findings suggest that PH formation arises from a final common pathway involving disruption of vesicle trafficking, leading to impaired cell

  18. Localized nodular synovitis of the infrapatellar fat pad

    Directory of Open Access Journals (Sweden)

    Jong-Hoon Park

    2013-01-01

    Full Text Available We report a case of localized nodular synovitis of the infrapatellar fat pad impinging on the patellofemoral joint causing limitation of extension. Arthroscopy involved use of a superolateral portal because location of lesion hindered access via a conventional anterior portal. The infrapatellar mass impinged in the patellofemoral joint upon knee extension and retracted upon flexion. Superior-superior triangulation allowed for complete excision of the mass.

  19. Nestin is expressed in HMB-45 negative melanoma cells in dermal parts of nodular melanoma.

    Science.gov (United States)

    Kanoh, Maho; Amoh, Yasuyuki; Tanabe, Kenichi; Maejima, Hideki; Takasu, Hiroshi; Katsuoka, Kensei

    2010-06-01

    Nestin, a marker of neural stem cells, is expressed in the stem cells of the mouse hair follicle. The nestin-expressing hair follicle stem cells can differentiate into neurons, glia, keratocytes, smooth muscle cells and melanocytes in vitro. These pluripotent nestin-expressing stem cells are keratin 15 (K15)-negative, suggesting that they are in a relatively undifferentiated state. Recent studies suggest that the epithelial stem cells are important in tumorigenesis, and nestin expression is thought to be important in tumorigenesis. In the present study, we examined the expression of the hair follicle and neural stem cell marker nestin, as well as S-100 and HMB-45, in melanoma. Nestin immunoreactivity was observed in the HMB-45-negative melanoma cells in all five cases of amelanotic nodular melanomas. Moreover, nestin immunoreactivity was observed in the dermal parts in seven of 10 cases of melanotic nodular melanomas. Especially, nestin immunoreactivity was observed in the HMB-45-negative melanoma cells in the dermal parts of all 10 cases of HMB-45-negative amelanotic and melanotic nodular melanomas. On the other hand, nestin expression was negative in 10 of 12 cases of superficial spreading melanoma. These results suggest that nestin is an important marker of HMB-45-negative melanoma cells in the dermal parts of patients with nodular melanoma.

  20. The effects of corrosion conditions and cold work on the nodular corrosion of zircaloy-4

    International Nuclear Information System (INIS)

    You, Gil Sung

    1992-02-01

    The nodular corrosion of Zircaloy-4 was investigated on the effects of corrosion conditions and cold work. Variation of steam pressures, heat-up environments and prefilms were considered and cold work effects were also studied. The corrosion rate of Zircaloy-4 was dependent on pressure between 1 and 100 atm and it followed the cubic law as W=16.85 x P 0.31 for plate specimens and W=12.69 x P 0.27 for tube specimens, where W is weight gain (mg/dm 2 ) and P is the steam pressure (atm). The environment variation in autoclave during heat-up period did not affect the early stage of nodular corrosion. The prefilm, which was formed at 500 .deg. C under 1 atm steam for 4 hours, restrained the formation of the initial small nodules. The oxide film formed under 1 atm steam showed no difference of electrical resistivity from the oxides formed under 100 atm steam pressure. Cold work specimens showed the higher resistivity against nodular corrosion than as-received specimens. The corrosion resistance arising from cold work seems to be due to the texture changes by the cold work. The results showed that cold work can affect the later stage of uniform corrosion and the early stage of nodular corrosion, namely, the nodule initiation stage

  1. O deserto e o espelho. Refletindo sobre a heterotopia com Michel Foucault e Nadine Gordimer

    Directory of Open Access Journals (Sweden)

    Anderson Bastos Martins

    2009-06-01

    Full Text Available Este ensaio representa uma tentativa de releitura do artigo “De outros espaços”, de Michel Foucault, seguindo uma análise da relaçáo estabelecida entre a protagonista do romance The pickup [O engate], de Nadine Gordimer, e o deserto, durante a viagem que ela faz à casa de campo de seu noivo. Apresentaremos um reenfoque do conceito foucaultiano de heterotopia através da elucidaçáo de sua noçáo de espelho e sua contribuiçáo potencial para refletir sobre a experiência espacial na contemporaneidade.

  2. Novel bandlike signal abnormality suggestive of heterotopia in patient with a KCNQ1 frameshift mutation.

    Science.gov (United States)

    Sabharwal, Priyanka; Devinsky, Orrin; M Shepherd, Timothy

    2017-12-01

    Malformations of cortical development are associated with epilepsy and cognitive dysfunction, and can occur in patients with SCN1A ion channel mutations. We report a novel and subtle bandlike subcortical heterotopia on integrated positron emission tomography-magnetic resonance imaging ( PET-MRI) in a patient with treatment-resistant epilepsy due to a de novo KCNQ1 frameshift mutation. Our case highlights the potential for other channel mutations to cause both epilepsy and cortical malformations. Further scrutiny of high contrast resolution MRI studies is warranted for patients with KCNQ1 and other epilepsy genes to further define their extended phenotype.

  3. Thyroid nodular disease: their usefulness of diagnostic methods

    International Nuclear Information System (INIS)

    Mendez Ayala, Irene Maria; Sanchez Luthard, Maria de los Angeles; Martins Schmitz; Gomez, Silvia

    2009-01-01

    The thyroid nodular disease is defined by the presence of nodules of thyroid of solid, liquid or mixed consistency, they are or non concrete. Solitary nodule thyroid, diffuse and multinodular goiter forms can be presented in. The thyroid nodule is a frequent clinical problem, the clinical prevalence in adult population is of 4%. Objective: to make a bibliographical revision to near the utility of the different methods available for the thyroid nodular pathology diagnosis and determining which of them turns out more specific to differentiate benignancy from malignancy in the found nodules. Materials and Methods: the bibliographical search was made in the data base Medline (Pubmed), in scientific magazines of the region and text books. Criteria of inclusion: bibliographical references pertaining to medical magazines and free full text with an antiquity nongreater to 8 years were included. Conclusion: the presence of thyroid nodules constitutes a reason for frequent consultation, the main diagnostic method at the moment is the fine needle aspiration cytology, due to its simple accomplishment, low cost, not requiring the hospitalization of the patient, and being fundamental in the decision making of the doctor. This diagnostic method is, in addition, the one that with greater certainty allows to differentiate benign from malignant nodules. (authors) [es

  4. The influence of various cooling rates during laser alloying on nodular iron surface layer

    Science.gov (United States)

    Paczkowska, Marta; Makuch, Natalia; Kulka, Michał

    2018-06-01

    The results of research referring to modification of the nodular iron surface layer by laser alloying with cobalt were presented. The aim of this study was to analyze the possibilities of cobalt implementation into the surface layer of nodular iron in various laser heat treatment conditions (by generating different cooling rates of melted surface layer). The modified surface layer of nodular iron was analyzed with OM, SEM, TEM, XRD, EDS and Vickers microhardness tester. The modified surface layer of nodular iron after laser alloying consisted of: the alloyed zone (melted with cobalt), the transition zone and the hardened zone from solid state. The alloyed zone was characterized by higher microstructure homogeneity - in contrast to the transition and the hardened zones. All the alloyed zones contained a dendritic microstructure. Dendrites consisted of martensite needles and retained austenite. Cementite was also detected. It was stated, that due to similar dimension of iron and cobalt atoms, their mutual replacement in the crystal lattice could occur. Thus, formation of phases based on α solution: Co-Fe (44-1433) could not be excluded. Although cobalt should be mostly diluted in solid solutions (because of its content in the alloyed zone), the other newly formed phases as Co (ε-hex.), FeC and cobalt carbides: Co3C, CoC0.25 could be present in the alloyed zones as a result of unique microstructure creation during laser treatment. Pearlite grains were observed in the zone, formed using lower power density of the laser beam and its longer exposition time. Simply, such conditions resulted in the cooling rate which was lower than critical cooling rate. The alloyed zones, produced at a higher cooling rate, were characterized by better microstructure homogeneity. Dendrites were finer in this case. This could result from a greater amount of crystal nuclei appearing at higher cooling rate. Simultaneously, the increased amount of γ-Fe and Fe3C precipitates was expected in

  5. INSULIN LIKE GROWTH FACTOR 1 POSSIBLE DEPENDENCE IN PATIENTS WITH METABOLIC SYNDROME OF NODULAR PATHOLOGY OF THE THYROID GLAND.

    Science.gov (United States)

    Rekvava, M; Dundua, T; Kobulia, M; Javashvili, L; Giorgadze, E

    2017-09-01

    Metabolic syndrome and nodular pathology of the thyroid gland is a widespread problem nowadays. Recently there has been a notable coincidence between metabolic syndrome and nodular pathology of thyroid gland. Hence, it is interesting to reveal the connection between these two diseases. It is possible that insulin-like growth factor system (IGF), namely IGF 1 is connecting link between metabolic syndrome and nodular pathology of thyroid gland, because IGF1 stimulates growth and proliferation of cells in the body. We have investigated18-82 years of age 71 patients. group 1 n27- subjects with thyroid nodular disease, and metabolic syndrome, group 2 n31- subjects with thyroid nodular disease and without metabolic syndrome. group 3 n13 - subjects with metabolic syndrome and no thyroid pathology. In all groups were assessed thyroid structural data, defined parameters of carbohydrate metabolism, thyroid function and blood concentration of IGF1. In patients with hyperinsulinemia IGF 1 was noted in normal or reduced concentration. In I group IGF1 was normal in 70,4% (n=19), decreased in 29,6% (n=8), In II group was normal in 77,4 % (n=24), decreased in 22,6% (n=7) and in III group was normal in 76,9% (n=10), decreased in 23,1% (n=3). Increase of IGF 1 in patients with thyroid nodular disease patients was not noted. Statistically significant connection between IGF1 and thyroid nodules was not revealed. For the further investigation of this connection we plan to measure IGF1 in the thyroid histological samples in the future studies.

  6. Heterotopia and cultural activism – the case of Hamburg’s Gängeviertel

    Directory of Open Access Journals (Sweden)

    Helten, Michael

    2015-09-01

    Full Text Available This paper investigates the Gängeviertel movement in Hamburg and the place which the activists have (reconstructed since its occupation in 2009 through the lens of Foucault’s (1986 concept of heterotopia. In the light of the recent debate about the role of cultural activism in the contemporary struggle about urban development, it explores the question which spatial practices and structures have evolved as oppositional to or in alignment with the neoliberal status quo of Hamburg’s spatial policy. Based on a qualitative case-study approach, the research was carried out in 2013. By using the analytical categories built environment, social practice and neoliberal normalisation, it illustrates that the Gängeviertel is characterised by practices that position it simultaneously both in- and outside of the neoliberal logic.

  7. Endoscopic Mucosectomy in a Child Presenting with Gastric Heterotopia of the Rectum

    Directory of Open Access Journals (Sweden)

    Joana Soares

    2017-09-01

    Full Text Available Gastric mucosal heterotopia has been described in all levels of the gastrointestinal tract. Its occurrence in the rectum is uncommon. We report the case of a 4-year-old boy referred to Pediatric Gastroenterology for intermittent rectal bleeding for the past 2 years. Total ileocolonoscopy revealed a flat, well-circumscribed lesion of 4 cm, with elevated margins, localized at 10 cm from the anal verge. Histologic examination showed typical gastric mucosa of the oxyntic type. Treatment with proton pump inhibitors was started without resolution of the symptoms and, therefore, an endoscopic mucosal resection was performed. Heterotopic gastric mucosa represents a rare cause of rectal bleeding in children and endoscopic evaluation is fundamental for diagnosis. Although not usually performed in pediatric ages, endoscopic mucosectomy allows complete resolution of the problem avoiding surgery.

  8. Endoscopic Mucosectomy in a Child Presenting with Gastric Heterotopia of the Rectum.

    Science.gov (United States)

    Soares, Joana; Ferreira, Carla; Marques, Margarida; Corujeira, Susana; Tavares, Marta; Lopes, Joanne; Carneiro, Fátima; Amil Dias, Jorge; Trindade, Eunice

    2017-11-01

    Gastric mucosal heterotopia has been described in all levels of the gastrointestinal tract. Its occurrence in the rectum is uncommon. We report the case of a 4-year-old boy referred to Pediatric Gastroenterology for intermittent rectal bleeding for the past 2 years. Total ileocolonoscopy revealed a flat, well-circumscribed lesion of 4 cm, with elevated margins, localized at 10 cm from the anal verge. Histologic examination showed typical gastric mucosa of the oxyntic type. Treatment with proton pump inhibitors was started without resolution of the symptoms and, therefore, an endoscopic mucosal resection was performed. Heterotopic gastric mucosa represents a rare cause of rectal bleeding in children and endoscopic evaluation is fundamental for diagnosis. Although not usually performed in pediatric ages, endoscopic mucosectomy allows complete resolution of the problem avoiding surgery.

  9. Cushing's Syndrome caused by pigmented adrenocortical micro nodular dysplasia - A familial case

    Energy Technology Data Exchange (ETDEWEB)

    Gomez-Segovia, I; Gallowitsch, H J; Kresnik, E; Kumnig, G; Mikosch, P; Lind, P [Dept.of Nuclear Medicine and Endocrinology, LKH Klagenfurt (Austria)

    2002-09-01

    Introduction: We present a Case of Cushing's syndrome (CS) in a 16 year old male adolescent. Adrenocortical micro nodular dysplasia is a rare cause of CS. It mostly develops in the first two decades of life. In pathogenesis a stimulatory effect of circulating Immunoglobulins on adrenal steroidogenesis has been postulated. Familial cases have been reported in relation to Carney's Syndrome. We report the clinical case at first diagnosis and preoperative follow up of 1 year prior to treatment. The leading symptoms were severe bilateral (fibrotic) gynaecomastia, weight gain and growth retardation, without hypertension,but osteoporosis, secondary hypogonadism and glucose intolerance. Laboratory findings and the results of functional tests were diagnostic for CS. In addition LDH (I-131 Isotopes), CK, Lipoproteins, GPT, Androstendion, Prolactin were elevated. MRI abdomen revealed a slight enlargement of the adrenals, and suspected a bilateral micro nodular dysplasia. Iodo-cholesterol-scan under dexamethason suppression showed a diffuse bilateral Iodo-cholesterol uptake confirming the autonomous production of cortisol bilateral in the adrenals.Whole body bone scan showed a diffuse reduced diphosphonate uptake in the skeleton and the growth plates. The bone mineral density was significantly reduced.Radiologically osteoporosis was overt. The rapid increase of free urinary cortisol excretion/24h within one year of observation led to a total bilateral adrenalectomy. Postoperative 5 year follow up examinations. Documentation of the outcome and recovery of clinical signs,symptoms and laboratory findings, discussion about the most appropriate long-term substitution therapy. Familial anamnesis:affected family member was the father (micro nodular bilateral adrenocortical dysplasia), the aunt (pararenal incidentaloma, histologically lipoma) and a cousin (micro nodular adrenocortical dysplasia). Sequential analysis of the menin gene from the patient was negative.The detection of

  10. Cushing's Syndrome caused by pigmented adrenocortical micro nodular dysplasia - A familial case

    International Nuclear Information System (INIS)

    Gomez-Segovia, I.; Gallowitsch, H.J.; Kresnik, E.; Kumnig, G.; Mikosch, P.; Lind, P.

    2002-01-01

    Introduction: We present a Case of Cushing's syndrome (CS) in a 16 year old male adolescent. Adrenocortical micro nodular dysplasia is a rare cause of CS. It mostly develops in the first two decades of life. In pathogenesis a stimulatory effect of circulating Immunoglobulins on adrenal steroidogenesis has been postulated. Familial cases have been reported in relation to Carney's Syndrome. We report the clinical case at first diagnosis and preoperative follow up of 1 year prior to treatment. The leading symptoms were severe bilateral (fibrotic) gynaecomastia, weight gain and growth retardation, without hypertension,but osteoporosis, secondary hypogonadism and glucose intolerance. Laboratory findings and the results of functional tests were diagnostic for CS. In addition LDH (I-131 Isotopes), CK, Lipoproteins, GPT, Androstendion, Prolactin were elevated. MRI abdomen revealed a slight enlargement of the adrenals, and suspected a bilateral micro nodular dysplasia. Iodo-cholesterol-scan under dexamethason suppression showed a diffuse bilateral Iodo-cholesterol uptake confirming the autonomous production of cortisol bilateral in the adrenals.Whole body bone scan showed a diffuse reduced diphosphonate uptake in the skeleton and the growth plates. The bone mineral density was significantly reduced.Radiologically osteoporosis was overt. The rapid increase of free urinary cortisol excretion/24h within one year of observation led to a total bilateral adrenalectomy. Postoperative 5 year follow up examinations. Documentation of the outcome and recovery of clinical signs,symptoms and laboratory findings, discussion about the most appropriate long-term substitution therapy. Familial anamnesis:affected family member was the father (micro nodular bilateral adrenocortical dysplasia), the aunt (pararenal incidentaloma, histologically lipoma) and a cousin (micro nodular adrenocortical dysplasia). Sequential analysis of the menin gene from the patient was negative.The detection of

  11. Mutation in filamin A causes periventricular heterotopia, developmental regression, and West syndrome in males.

    Science.gov (United States)

    Masruha, Marcelo R; Caboclo, Luis O S F; Carrete, Henrique; Cendes, Iscia L; Rodrigues, Murilo G; Garzon, Eliana; Yacubian, Elza M T; Sakamoto, Américo C; Sheen, Volney; Harney, Megan; Neal, Jason; Hill, R Sean; Bodell, Adria; Walsh, Christopher; Vilanova, Luiz C P

    2006-01-01

    Familial periventricular heterotopia (PH) represents a disorder of neuronal migration resulting in multiple gray-matter nodules along the lateral ventricular walls. Prior studies have shown that mutations in the filamin A (FLNA) gene can cause PH through an X-linked dominant pattern. Heterozygotic female patients usually remain asymptomatic until the second or third decade of life, when they may have predominantly focal seizures, whereas hemizygotic male fetuses typically die in utero. Recent studies have also reported mutations in FLNA in male patients with PH who are cognitively normal. We describe PH in three male siblings with PH due to FLNA, severe developmental regression, and West syndrome. The study includes the three affected brothers and their parents. Video-EEG recordings and magnetic resonance image (MRI) scanning were performed on all individuals. Mutations for FLNA were detected by using polymerase chain reaction (PCR) on genomic DNA followed by single-stranded conformational polymorphism (SSCP) analysis or sequencing. Two of the siblings are monozygotic twins, and all had West syndrome with hypsarrhythmia on EEG. MRI of the brain revealed periventricular nodules of cerebral gray-matter intensity, typical for PH. Mutational analyses demonstrated a cytosine-to-thymidine missense mutation (c. C1286T), resulting in a threonine-to-methionine amino acid substitution in exon 9 of the FLNA gene. The association between PH and West syndrome, to our knowledge, has not been previously reported. Males with PH have been known to harbor FLNA mutations, although uniformly, they either show early lethality or survive and have a normal intellect. The current studies show that FLNA mutations can cause periventricular heterotopia, developmental regression, and West syndrome in male patients, suggesting that this type of FLNA mutation may contribute to severe neurologic deficits.

  12. Subcortical heterotopia appearing as huge midline mass in the newborn brain.

    Science.gov (United States)

    Fukumura, Shinobu; Watanabe, Toshihide; Kimura, Sachiko; Ochi, Satoko; Yoshifuji, Kazuhisa; Tsutsumi, Hiroyuki

    2016-02-01

    We report the case of a 2-year-old boy who showed a huge midline mass in the brain at prenatal assessment. After birth, magnetic resonance imaging (MRI) revealed a conglomerate mass with an infolded microgyrus at the midline, which was suspected as a midline brain-in-brain malformation. MRI also showed incomplete cleavage of his frontal cortex and thalamus, consistent with lobar holoprosencephaly. The patient underwent an incisional biopsy of the mass on the second day of life. The mass consisted of normal central nervous tissue with gray and white matter, representing a heterotopic brain. The malformation was considered to be a subcortical heterotopia. With maturity, focal signal changes and decreased cerebral perfusion became clear on brain imaging, suggesting secondary glial degeneration. Coincident with these MRI abnormalities, the child developed psychomotor retardation and severe epilepsy focused on the side of the intracranial mass.

  13. Learning through Nomadic Interiors - Villa Rotonda through the lenses of Heterotopia

    DEFF Research Database (Denmark)

    Hvejsel, Marie Frier; Fisker, Anna Marie

    2015-01-01

    in a series of nomadic interiors allowing the students to travel in time and place; from Palladio to the 14th Architectural Biennale and from Vincenza to Venice. In the paper we use Foucaults concept of Heterotopia to analyze how a window in 1:10 from the south façade of Villa Rotonda now re-designed......The question of the critical role of our built heritage to the complex and interdisciplinary processes that govern the current development of the built environment define a continuous challenge in interior studies and education: Can a study of the fundamental interior elements of Villa Rotonda...... of human geography as elaborated by Foucault and apply it on a project developed and exhibited on the 14th International Architecture Biennale in Venice that focused on the current need to develop Urban Farming strategies. Here an interdisciplinary team of students analyzed fundamental interior elements...

  14. Rare cause of multiple nodular opacities at chest x-ray: pulmonary hydatid cyst

    International Nuclear Information System (INIS)

    Inan, K.; Hamcan, S.; Gumus, S.; Turhan, U.; Karaman, B.

    2012-01-01

    Full text: Introduction: Hydatid disease is incidentally common in our country. Objectives and tasks: In this study, unlike the classical radiological appearance of hydatid disease of the lung, MDCT appearance of multiple nodules were demonstrated. Materials and methods: The patient who comes our hospital's Pulmonary Clinic with shortness of breath and with membranes that come from his mouth, referred to our clinic for chest radiography and chest HRCT. Results: In the conventional chest x-ray, multiple nodular opacities in both lungs were common. HRCT was performed with 5 mm and 1 mm thick sections of our patient. In both hemithorax, multiple nodular lesions were found in various sizes and configurations, some of them opened to the bronchus which is the largest one is 2 cm in diameter. Nodule in the left hemithorax inferior lingular segment has calcified wall. Patient's Echinococcus granulosus test was evaluated positive for IgG. Conclusion: Hydatid disease is a parasitic infestation created by Echinococcus granulosus. Although seen most frequently in the liver, often seen in the lungs 10-30%.. 30 to 50% of cases are asymptomatic and incidentally diagnosed radiologically. Although we know that the classic radiologic findings of hydatid cyst, different radiographic views (eg nodular mass) should be considered in rare circumstances

  15. Nodular Scleritis Associated with Herpes Zoster Virus: An Infectious and Immune-Mediated Process

    Directory of Open Access Journals (Sweden)

    Mónica Loureiro

    2016-01-01

    Full Text Available Purpose. To describe a case of anterior nodular scleritis, preceded by an anterior hypertensive uveitis, which was primarily caused by varicella zoster virus (VZV. Case Report. A 54-year-old woman presented with anterior uveitis of the right eye presumably caused by herpetic viral disease and was successfully treated. Two months later, she developed a nodular scleritis and started oral nonsteroidal anti-inflammatory without effect. A complete laboratory workup revealed positivity for HLA-B27; the infectious workup was negative. Therapy was changed to oral prednisolone and an incomplete improvement occurred. Therefore, a diagnostic anterior paracentesis was performed and the polymerase chain reaction (PCR analysis revealed VZV. She was treated with valacyclovir and the oral prednisolone began to decrease; however, a marked worsening of the scleritis occurred with the reduction of the daily dose; subsequently, methotrexate was introduced allowing the suspension of the prednisolone and led to clinical resolution of the scleritis. Conclusion. This report of anterior nodular scleritis caused by VZV argues in favor of an underlying immune-mediated component, requiring immunosuppressive therapy for clinical resolution. The PCR analysis of the aqueous humor was revealed to be a valuable technique and should be considered in cases of scleritis with poor response to treatment.

  16. Diagnostic accuracy of nodular gastritis for H. pylori infection

    Directory of Open Access Journals (Sweden)

    Romero-Flores JL

    2016-12-01

    Full Text Available Juan L Romero-Flores,1 Justo A Fernandez-Rivero,1 Erika Marroquín-Fabian,1 Félix I Téllez-Ávila,2 Beatriz A Sánchez-Jiménez,1 Eva Juárez-Hernández,3 Misael Uribe,1 Norberto C Chávez-Tapia1,3 1Obesity and Digestive Diseases Unit, Medica Sur Clinic & Foundation, 2Department of Gastrointestinal Endoscopy, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, 3Translational Research Unit, Medica Sur Clinic & Foundation, Mexico City, Mexico Background: The term nodular is not included in the Sydney classification and there is no widely accepted histopathological definition. It has been proposed that the presence of antral nodularity could predict Helicobacter pylori (H. pylori infection. The aim of this study was to determine the diagnostic accuracy of nodular gastritis (NG for H. pylori infection after a rigorous standardization process, and to describe the associated histopathological characteristics. Materials and methods: Endoscopic images of patients submitted to endoscopy with biopsy sampling were included. Endoscopic images were distributed among six endoscopists. The analysis was performed sequentially in three rounds: the first round assessed the interobserver variability, the second evaluated the intraobserver variability, and the third calculated the interobserver variability after training. A correlation analysis between endoscopic and histopathological findings was performed. Results: A total of 917 studies were included. In the first analysis of interobserver variability, a poor kappa value (0.078 was obtained. The second evaluation yielded good intraobserver variability, with kappa values of 0.62–0.86. The evaluation of interobserver variability after training revealed an improvement in the kappa value of 0.42. A correlation was found between endoscopic images and histopathological reports. Conclusion: There was a strong correlation between NG and H. pylori, but only after rigorous evaluation. The use of

  17. Ultrasonography, angiography, computed tomography and magnetic resonance in nodular regenerative hyperplasia of the liver

    International Nuclear Information System (INIS)

    Patriarche, C.; Pelletier, G.; Attali, P.; Ladouch-Badre, A.; Fabre, M.; Roche, A.; Etienne, J.P.

    1988-01-01

    Ultrasonographic, computed tomographic, and angiographic abnormalities of nodular regenerative hyperplasia have been described in very few cases. We report here the case of a 50-year-old man with round, well-limited hypoechogenic lesions involving the two lobes of the liver, and hypervascular, poorly delineated angiographic lesions. Computed tomography and magnetic resonance of the liver were normal. Histological examination of large liver specimens provided by intraoperative biopsy allowed the diagnosis of nodular regenerative hyperplasia. Such a pseudo-tumoral ultrasonographic and angiographic pattern must be recognized in order to avoid diagnostic and therapeutic mistakes, especially since percutaneous liver biopsy usually fails to diagnose this disease. (author)

  18. Periorbital nodular fasciitis arising during pregnancy

    Directory of Open Access Journals (Sweden)

    Brandon N Phillips

    2014-01-01

    Full Text Available Nodular fasciitis (NF is a benign proliferation of fibroblasts and myofibroblasts that rarely occurs in the periorbital region. We report what we believe to be the first case of periorbital NF associated with pregnancy. A case of intravascular fasciitis, a NF variant, has been reported during pregnancy, but it was not located in the periorbital region. A weak presence of estrogen receptors has been reported in NF. This may make it more susceptible to the hormone-related changes during pregnancy and contribute to the development of the lesion by stimulating fibroblasts and smooth muscle cell types. Although rare, NF should be considered in the differential diagnosis of periorbital soft-tissue masses arising during pregnancy.

  19. HRCT-pathologic correlation of nodular bronchioloalveolar carcinoma

    International Nuclear Information System (INIS)

    Abe, Katsumi; Suzuki, Kenzo; Koike, Morio; Yokoyama, Yoshiaki; Asai, Sae; Ushimi, Naofumi; Kamata, Noriko

    1996-01-01

    Lung cancers, shown as nodules on conventional chest radiographs, are detected easily. However, nodular bronchioloalveolar carcinoma (BAC), usually located at the periphery of the lung, are faint and poorly defined on conventional chest radiographs, it is thus difficult to detect and make the diagnosis. On the other hand, these lesions are clearly depicted on high-resolution computed tomography (HRCT), therefore HRCT is effective in assessing these faint pulmonary nodules. We retrospectively investigated preoperative HRCT of thirteen patients (8 men and 5 women, 53-80 years old) with pathologically proven nodular BACs of 2.2 (±0.43) cm in mean diameter by two radiologists. All the lesions were located periphery of the lung. In 11 cases (85%), HRCT showed a zone of ground-glass attenuation surrounding the nodule, which correlated pathologically with papillary tumor growth without disrupting the overall lung architecture along the framework of normal alveoli containing air-spaces, alveolar spaces filled with fluid, blood, and infiltration of macrophages. In 10 cases (77%), bubblelike radiolucencies of focal areas of air attenuation peripherally or centrally were observed and correlated pathologically with intratumoral patent air-containing bronchi and cystic glandular spaces within papillary tumor growth. Notches were demonstrated in 12 cases (92%), air bronchogram in 11 cases (85%), convergence of vessels in 10 cases (77%), pleural tags in 9 cases (69%), and spiculations in 6 cases (46%). Calcification was not seen in any cases. A zone of ground-glass attenuation and bubblelike radiolucencies were observed frequently among the CT findings of BAC, and are considered to be characteristic enough to suggest BAC. (author)

  20. Investigations of Ferritic Nodular Cast Iron Containing About 5-6% Aluminium

    Directory of Open Access Journals (Sweden)

    Soiński M.S.

    2016-12-01

    Full Text Available The work presents results of investigations concerning the production of cast iron containing about 5-6% aluminium, with the ferritic matrix in the as-cast state and nodular or vermicular graphite precipitates. The examined cast iron came from six melts produced under the laboratory conditions. It contained aluminium in the amount of 5.15% to 6.02% (carbon in the amount of 2.41% to 2.87%, silicon in the amount of 4.50% to 5.30%, and manganese in the amount of 0.12% to 0.14%. After its treatment with cerium mixture and graphitization with ferrosilicon (75% Si, only nodular and vermicular graphite precipitates were achieved in the examined cast iron. Moreover, it is possible to achieve the alloy of pure ferritic matrix, even after the spheroidizing treatment, when both the aluminium and the silicon occur in cast iron in amounts of about 5.2÷5.3%.

  1. Pancreatic heterotopia in the gall bladder: a case report and literature review

    Directory of Open Access Journals (Sweden)

    Sansom Henrique Bromberg

    2009-03-01

    Full Text Available Pancreatic heterotopia in the gall bladder is a rare entity, with approximately 31 cases reported in English language literature until 2006; there are no Brazilian studies on the topic. As it is usually asymptomatic, the definitive diagnosis is made by the anatomopathological material excised due to common diseases of the organ, such as lithiasis, inflammatory processes, polyps and tumors. A case is reported of a 40-year-old female patient submitted to surgery with a diagnosis of lithiasic cholecystopathy. Ectopy was evidenced by the histological study, due to the presence of acinar and ductal components of the pancreas and gall bladder corpus. The authors used their own pathological classification for this ectopy, and recommended that this entity be included more frequently in the differential diagnosis of alithiasic cholecystopathy and intramural and exophytic lesions of the gall bladder.

  2. Projected reduction in healthcare costs in Belgium after optimization of iodine intake: impact on costs related to thyroid nodular disease.

    Science.gov (United States)

    Vandevijvere, Stefanie; Annemans, Lieven; Van Oyen, Herman; Tafforeau, Jean; Moreno-Reyes, Rodrigo

    2010-11-01

    Several surveys in the last 50 years have repeatedly indicated that Belgium is affected by mild iodine deficiency. Within the framework of the national food and health plan in Belgium, a selective, progressive, and monitored strategy was proposed in 2009 to optimize iodine intake. The objective of the present study was to perform a health economic evaluation of the consequences of inadequate iodine intake in Belgium, focusing on undisputed and measurable health outcomes such as thyroid nodular disease and its associated morbidity (hyperthyroidism). For the estimation of direct, indirect, medical, and nonmedical costs related to thyroid nodular diseases in Belgium, data from the Federal Public Service of Public Health, Food Chain Safety and Environment, the National Institute for Disease and Disability Insurance (RIZIV/INAMI), the Information Network about the prescription of reimbursable medicines (FARMANET), Intercontinental Marketing Services, and expert opinions were used. These costs translate into savings after implementation of the iodization program and are defined as costs due to thyroid nodular disease throughout the article. Costs related to the iodization program are referred to as program costs. Only figures dating from before the start of the intervention were exploited. Only adult and elderly people (≥18 years) were taken into account in this study because thyroid nodular diseases predominantly affect this age group. The yearly costs due to thyroid nodular diseases caused by mild iodine deficiency in the Belgian adult population are ∼€38 million. It is expected that the iodization program will result in additional costs of ∼€54,000 per year and decrease the prevalence of thyroid nodular diseases by 38% after a 4-5-year period. The net savings after establishment of the program are therefore estimated to be at least €14 million a year. Optimization of iodine intake in Belgium should be quite cost effective, if only considering its impact on

  3. [Morel-Lavallée syndrome and post-traumatic nodular fat necrosis: Two post-traumatic complications mimicking cellulitis].

    Science.gov (United States)

    Moulin, C; Barthélémy, I; Emering, C; D'Incan, M

    Dermal and subcutaneous inflammation following direct trauma is initially evocative of soft-tissue infection. However, two differential diagnoses must be considered: Morel-Lavallée syndrome and post-traumatic nodular fat necrosis. Case 1: a 51-year-old woman fell off her motorbike and had dermabrasions on her right and left tibial ridges that rapidly developed into dermo-hypodermitis of the entire limb. There was no improvement after 3 weeks of antibiotics. The patient was apyretic. She had a soft, non-inflammatory tumefaction on the inner aspect of her left knee. Ultrasound revealed subcutaneous collection in both legs. The surgeons confirmed a diagnosis of Morel-Lavallée syndrome and drained the two collections. Progress was good and the patient healed without major consequences. Case 2: following a fall on her stairs, a 40-year-old woman presented dermabrasions and haematomas on her left leg. Antibiotic therapy failed to prevent the progression of dermo-hypodermitis. The patient remained apyretic and there was no inflammatory syndrome. A CT scan showed thickening of a subcutaneous fat and fluid collection, resulting in diagnosis of post-traumatic nodular fat necrosis. Management was surgical and the outcome was good. These two cases show two post-traumatic cutaneous complications: Morel-Lavallée syndrome and post-traumatic nodular fat necrosis. Morel-Lavallée syndrome occurs after tangential trauma next to richly vascularized tissue. Post-traumatic nodular fat necrosis is defined as necrosis of adipocytes. In both cases, diagnosis is confirmed by imagery (Ultrasonography, tomography). Our two case reports show that inflammatory presentation of both Morel-Lavallée syndrome and post-traumatic nodular fat necrosis can lead to diagnostic and therapeutic errors while a surgical procedure is necessary since tissue necrosis can occur. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  4. Perianal nodular hidradenocarcinoma. Case report.

    Science.gov (United States)

    Sierra Montenegro, Ernesto; Sierra Luzuriaga, Gastón; Leone Stay, Gaetano; Salazar Menéndez, Vilma; Quiñonez Auria, Carlos

    2010-01-01

    Eccrine glands (sweat glands) appear in all sites of the skin and are more abundant in hands and feet. Nodular hidradenocarcinoma (NH) is a rare malignant and aggressive tumor of the eccrine glands. The objective of this study is to report a case of perianal hidradenocarcinoma. We present the case of a 75-year-old female with diabetes, hypertension, and hypothyroidism. Physical examination revealed a small perianal tumor that was palpated near the anal canal. Biopsy was done. Pathology report revealed perianal hidradenoma. Rectosigmoidoscopy was normal. Computed tomography showed 1-cm adenopathies in inguinal and right iliac regions. Extensive resection of the tumor was done. Definitive pathology report was malignant eccrine acrospiroma. The patient underwent adjuvant chemotherapy in the Oncology Service and died 1 month later. In patients with a first symptom of metastases in the inguinal region, suspicion must be directed to the anal canal.

  5. Role of ultrasound in the assessment of nodular thyroid disease

    International Nuclear Information System (INIS)

    Bastin, S.; Bolland, M. J.; Croxson, M. S.

    2009-01-01

    Full text: The role for ultrasound (US) in the assessment of nodular thyroid disease has increased in recent years. This expanded role has been highlighted in recent consensus guidelines on the management of nodular thyroid disease. In this review, we address the potential roles for US in assessing thyroid nodules and review these recent guidelines. In particular, we review the evidence that US characteristics of thyroid nodules can predict the risk of malignancy. A predominantly solid nodule, hypoechogenicity, microcalcification, macrocalcification, ill-defined margins, intranodular vascularity, and taller-than-wide shape have all been associated with increased risk of malignancy, but no single US characteristic is sufficiently sensitive or specific to exclude or diagnose malignancy by itself. However, the use of combinations of US characteristics to stratify nodules into high and low risk for malignancy appears a promising strategy. Unselected nodules without any suspicious US features have a low risk of malignancy (<2%), whereas malignancy rates are much higher in nodules with at least two suspicious features. Recent guidelines endorse this approach of using combinations of US features to guide nodule selection for fine needle aspiration.

  6. Primary pigmented nodular adrenocortical disease presenting with a unilateral adrenocortical nodule treated with bilateral laparoscopic adrenalectomy: a case report

    Directory of Open Access Journals (Sweden)

    Kaltsas Gregory

    2010-07-01

    Full Text Available Abstract Introduction Primary pigmented nodular adrenocortical disease is a rare cause of adrenocorticotropic hormone-independent Cushing's syndrome. We report an uncommon primary pigmented nodular adrenocortical disease case presenting with a unilateral adrenocortical nodule and provide a brief overview of the existing literature. Case presentation A 27-year-old Caucasian woman was admitted to our Department with adrenocorticotropic hormone-independent Cushing's syndrome. Its cause was initially considered a left adrenocortical adenoma based on computer tomography imaging. The patient underwent left laparoscopic adrenalectomy and histological examination revealed pigmented micronodular adrenal hyperplasia. Evaluation for the presence of Carney complex was negative. Six months later recurrence of hypercortisolism was documented and a right laparoscopic adrenalectomy was performed further establishing the diagnosis of primary pigmented nodular adrenocortical disease. After a nine-year follow-up there is no evidence of residual disease. Conclusions Even though primary pigmented nodular adrenocortical disease is a rare cause of Cushing's syndrome, it should be included in the differential diagnosis of adrenocorticotropic hormone-independent Cushing's syndrome, especially because adrenal imaging can be misleading mimicking other adrenocortical diseases. Bilateral laparoscopic adrenalectomy is the preferred treatment in these subjects.

  7. Lymphoid nodular hyperplasia in a patient with severe combined immunodeficiency disease; Hiperplasia nodular linfoide en un paciente con inmunodeficiencia combinada grave

    Energy Technology Data Exchange (ETDEWEB)

    Sanchez-Alegre, M. L.; Casanova, A.; Delgado, J.; Relanzon, S. [Hospital General Universitario Gregorio Maranon. Madrid (Spain)

    2001-07-01

    We describe a case of lymphoid nodular hyperplasia in a woman with severe combined immunodeficiency disease. the patient complained of constipation and episodes of abdominal pain, and examination revealed the presence of a large abdominal mass. The diagnosis was suspected on the basis of the initial radiological studies, but intestinal biopsy was necessary to rule out lymphomatous involvement. We point out the radiological features of this entity which, despite the fact that it may be a chance finding of no pathological significance, requires special attention, especially in immuno deficient individuals. (Author) 10 refs.

  8. The Importance of a Proper Selection Area to be Biopsied in Nodular Leukoplakia: a Case Report

    Directory of Open Access Journals (Sweden)

    Paulo Sérgio da Silva Santos

    2014-01-01

    Full Text Available Nodular leukoplakia is a non-homogeneous type of oral leukoplakia presenting a white surface with verrucous, nodular, ulcerated or erythematous features with a greater risk of malignant transformation when compared to the homogeneous type. Common sites of involvement include lip commissures, buccal mucosa and soft palate. It is often associated with epithelial dysplasia or carcinoma and requires detailed microscopic assessment and regular follow-up. The importance of a proper selection of the area to be biopsied and the close teamwork between a dentist and oral pathologist is the basis of providing an accurate final diagnosis.

  9. Long-term results in patients with low-grade nodular non-Hodgkin's lymphoma

    International Nuclear Information System (INIS)

    Aviles, A.; Diaz-Maqueo, J.C.; Sanchez, E.; Cortes, H.D.; Ayala, J.R.; Oncology Hospital, Mexico City; National Medical Center, Mexico City

    1991-01-01

    One hundred and eighteen patients with nodular non-Hodgkin's lymphoma were randomized to receive either chemotherapy alone or chemotherapy plus radiotherapy (total nodal or involved field irradiation). Although the complete remission rate was similar in the three programs (about 90%) the relapse-free survival rate (RFS) among patients with complete remission was significantly higher in the groups treated with chemotherapy plus radiotherapy than among those treated with chemotherapy alone. The 7-year RFS in the groups treated with total node irradiation and involved field irradiation was 71% and 66% respectively, compared to only 33% in the group treated by chemotherapy alone (p<0.01). The results suggest that combined chemoradiotherapy may achieve complete long-term remission and potential cure in more than 60% of patients with nodular low-grade non-Hodgkin's lymphoma. Toxicity was moderate in all three arms. Bulky disease and a high level of lactic dehydrogenase were associated with a poor prognosis. (orig.)

  10. [Characteristics of thyroid carcinoma in Grave's disease Hashimoto's thyroiditis and nodular goiter].

    Science.gov (United States)

    Filipović, A; Paunović, I

    2003-01-01

    The biology of thyroid cancer represents a spectrum of behavior ranging from well-differentiated lesions with an excellent prognosis to anaplastic carcinoma, which is almost fatal. For this reason, it is important that clinicians have methods at their disposal to asses the characteristics of patient's thyroid malignancy. In this work we discuss the behavior of differentiated thyroid cancer in associated diseases of thyroid as: Graves' disease, chronic lymphocytic thyroiditis--Hashimoto and nodular goiter. This is retrospectively reviewing of 50 patients treated for differentiated thyroid carcinoma at Department of surgery, Clinical Centre of Montenegro in Podgorica from 1998 until 2003. We evaluated occurrence, as well as the role of this diseases in patients with thyroid cancer. We found a more favorable course of thyroid cancer in the presence of chronic lymphocytic thyroiditis and nodular goiter, a contrary Graves' disease. In associated diseases of thyroid, a significantly greater proportion of patients with thyroid cancer, have modular goiter.

  11. A novel DCX missense mutation in a family with X-linked lissencephaly and subcortical band heterotopia syndrome inherited from a low-level somatic mosaic mother: Genetic and functional studies.

    Science.gov (United States)

    Tsai, Meng-Han; Kuo, Pei-Wen; Myers, Candace T; Li, Shih-Wen; Lin, Wei-Che; Fu, Ting-Ying; Chang, Hsin-Yun; Mefford, Heather C; Chang, Yao-Chung; Tsai, Jin-Wu

    2016-09-01

    To study the genetics and functional alteration of a family with X-linked lissencephaly and subcortical band heterotopia. Five affected patients (one male with lissencephaly, four female with subcortical band heterotopia) and their relatives were studied. Sanger sequencing of DCX gene, allele specific PCR and molecular inversion probe technique were performed. Mutant and wild type of the gene products, namely doublecortin, were expressed in cells followed by immunostaining to explore the localization of doublecortin and microtubules in cells. In vitro microtubule-binding protein spin-down assay was performed to quantify the binding ability of doublecortin to microtubules. We identified a novel DCX mutation c.785A > G, p.Asp262Gly that segregated with the affected members of the family. Allele specific PCR and molecular inversion probe technique demonstrated that the asymptomatic female carrier had an 8% mutant allele fraction in DNA derived from peripheral leukocytes. This mother had 7 children, 4 of whom were affected and all four affected siblings carried the mutation. Functional study showed that the mutant doublecortin protein had a significant reduction of its ability to bind microtubules. Low level mosaicism could be a cause of inherited risk from asymptomatic parents for DCX related lissencephaly-subcortical band heterotopia spectrum. This is particularly important in terms of genetic counselling for recurrent risk of future pregnancies. The reduced binding affinity of mutant doublecortin may contribute to developmental malformation of the cerebral cortex. Copyright © 2016 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

  12. The prevalence of thyroid cancers in surgically treated patients with nodular goiter in Şırnak city

    Directory of Open Access Journals (Sweden)

    Sevda Sert Bektaş

    2010-12-01

    Full Text Available Objectives: Iodine deficiency is still considered to be the major etiological factor for endemic goiter. The pathogenesis of the goiter in iodine deficient area caries different characteristics. The aim of this study investigate the prevalence of thyroid cancers and type of thyroid cancers in surgically treated patients with nodular goiter in Şırnak city where is iodine deficiency region.Materials and methods: Thyroid surgical materials which were sent to our department were screened retrospectively from the archives between the years 2009-2010. Thyroid resection was performed on 241 patients with nodular goiter in one year. We evaluated patients who received the diagnosis of thyroid carcinoma with histhopatological examination.Results: 222 of our patients (92.1% female and 19 (7.9% were male. The youngest patient 16 and the oldest patient was 80 years old and the average age is 40.9 ± 12.8. Histopathological examination of 197 (81.7% cases of nodular goiter, 31 (12.9% cases lymphocytic thyroiditis, 13 (5.4% patients had thyroid tumors. The three tumors on the 2 cases (0.8% benign, 11 (4.6% were malignant. As a type of cancer 1 (0.4% patients, follicular carcinoma-oncocytic variant, 10 (4.2% cases were papillary carcinoma.Conclusions: Iodine deficiency area in the province of Şırnak in patients with nodular goiter who underwent surgery for thyroid cancer rate of 4.6%, and most cancers is seen as a type of thyroid papillary carcinoma.

  13. Suppressive therapy for radiation-associated nodular thyroid disease

    International Nuclear Information System (INIS)

    Tamura, Kazuo; Shimaoka, Katsutaro; Tsukada, Yoshiaki; Razack, M.S.; Sciasicia, Michael.

    1981-01-01

    A thyroid screening program for individuals who had irradiation to the head and neck areas was started at Roswell Park Memorial Institute in February 1977 and by June 1979, 1,071 patients were seen in the clinic. Three hundred and ninety-six patients were found to have palpable abnormalities of the thyroid, and following pretreatment evaluation, suppressive therapy with triiodothyronine (T3) (50 μg/day) or DT (desiccated thyroid) (120 mg/day) was administered in a double-blind fashion. Two hundred fifty patients with nodular disease completed 6 mo of treatment and are analyzed in this paper. Pretreatment thyroid function tests showed that two patients had hypothyroidism with a high thyroid-stimulating hormone (TSH) and a low thyroxine level. A high incidence of thyroid autoantibodies was also noted and surgical findings confirmed a high incidence of chronic thyroiditis. Complete disappearance of the nodules was seen in 29% of the patients, and in addition, 38% of the patients were seen to have significant shrinkage of the nodules, indicating that radiation-associated thyroid nodules were as sensitive to the thyroactive agents as nonirradiated nodular thyroid disease. There was little difference in the response rate between T3 and DT. Both agents suppressed circulating TSH levels to an unmeasurable level in 76% of the patients. There was no correlation between scan findings and response rates. Thyroid carcinoma was found in 19% of the patients who underwent surgery; although all were well-differentiated carcinomas, two-thirds of the patients already had evidence of dissemination and/or invasion suggesting the aggressive nature of postirradiation thyroid carcinoma. (author)

  14. Fatigue strength of nodular cast iron with regard to heavy-wall applications

    Energy Technology Data Exchange (ETDEWEB)

    Bleicher, Christoph; Wagener, Rainer; Kaufmann, Heinz [Fraunhofer-Institut fuer Betriebsfestigkeit und Systemzuverlaessigkeit LBF, Darmstadt (Germany); Melz, Tobias [Fraunhofer-Institut fuer Betriebsfestigkeit und Systemzuverlaessigkeit LBF, Darmstadt (Germany); TU Darmstadt (Germany). Faculty of Mechanical Engineering

    2015-11-01

    For a proper estimation of the fatigue life of a heavy-walled cast component made of nodular cast iron, sufficient knowledge regarding the cyclic properties of the material is necessary. Based on the material parameters at hand for component design, different fatigue analysis procedures can be used. Elastic and elastic-plastic approaches can be adopted, with the latter being reserved only for local approaches. The present publication summarizes the cyclic material parameters gained during a research project by extensive material tests under stress and strain controlled cyclic loading at different load ratios for three nodular cast iron grades. In addition to an improved knowledge of the cyclic material behavior, the notch, the size effects and the mean stress sensitivity were of special concern during the investigations in order to provide an entire overview of the tested materials and thus input information for both stress and strain based design approaches. Tests were performed for specimens taken from large cast blocks of the nodular cast iron grades EN-GJS-400-18U-LT and EN-GJS-450-18, both with ferritic matrices, and EN-GJS-700-2 with a pearlitic matrix. For some of these materials, mean stress sensitivities above 0.5 were obtained during the investigations. These values are not covered by the common standards, which calculate lower values for the mean stress sensitivity. Cyclic material parameters for stress and strain controlled tests are given in this paper as well as values for the size effect, based on the concept of the highly stressed volume. The effect of different specimen sizes could be shown not only by stress but also by strain controlled tests.

  15. Heterotopia pancreática: análise clínico-patológica de 18 doentes

    Directory of Open Access Journals (Sweden)

    Sansom Henrique Bromberg

    Full Text Available OBJETIVO: Analisar as características clínico-patológicas do tecido pancreático heterotópico em órgãos digestivos abdominais. MÉTODOS: Realizamos estudo retrospectivo analisando 18 portadores de pâncreas heterotópico diagnosticados histologicamente. Seus dados clínicos e histopatológicos foram revistos. O tecido pancreático heterotópico foi classificado em três modelos histológicos: tipo I constituído por três componentes do pâncreas normal (ácinos, ductos e ilhotas, tipo II com dois componentes e tipo três com somente um componente. RESULTADOS: A média de idade foi de 52,7 anos, variando de 34 a 73 anos, com nove homens e nove mulheres. Sintomas foram observados em somente quatro doentes, sendo suas lesôes diagnosticadas por gastroscopia. Os 14 restantes eram assintomáticos e suas anomalias descobertas acidentalmente. A maioria das lesões situava-se no trato superior: sete (38,9% no estômago, seis (33,3% no duodeno e três (16,6% no jejuno. A heterotopia localizou-se preferencialmente na submucosa (83,3%, mas também foi observada na muscular própria e na sub-serosa. Em sete (38,9% espécimes todos os componentes pancreáticos foram constatados (tipo I, em oito (44,4% estavam presentes glândulas exócrinas e ductos excretores (tipo II e em três (16,7% somente o tecido exócrino foi observado (tipo III. CONCLUSÃO: A heterotopia pancreática é rara. Doentes com ectopia pancreática diagnosticadas pelo estudo patológico, assintomáticos ou com sintomas discretos devem permanecer em observação. As lesões detectadas acidentalmente durante procedimentos cirúrgicos necessitam ser removidas por procedimentos conservadores.

  16. Metastatic ocular melanoma to the liver exhibits infiltrative and nodular growth patterns

    DEFF Research Database (Denmark)

    Grossniklaus, Hans E; Zhang, Qing; You, Shuo

    2016-01-01

    We examined liver specimens from 15 patients with uveal melanoma (UM) who had died of their disseminated disease. We found 2 distinct growth patterns of UM metastasis: infiltrative (n = 12) and nodular (n = 3). In the infiltrative pattern, individual UM cells with a CD133+ cancer stem cell-like p...

  17. Nodular Epithelial Hyperplasia after Photorefractive Keratectomy Followed by Corneal Collagen Cross-Linking

    OpenAIRE

    Bogoni, Ayla; Salerno, Liberdade Cezaro; Ghanem, Vinícius Coral; Ghanem, Ramon Coral

    2013-01-01

    This study describes a case of nodular epithelial hyperplasia and stromal alterations in a patient with keratoconus who was submitted to topography-guided photorefractive keratectomy (PRK) followed by corneal collagen cross-linking. Debridement of the epithelial nodule was performed. After a 2-year followup, a new topography-guided PRK was indicated.

  18. Comportamiento del hierro nodular austemperizado en condiciones de corrosión y desgaste // Behavior of austempered ductile iron under wear and corrosion conditions

    Directory of Open Access Journals (Sweden)

    L. Goyos Pérez

    1999-07-01

    Full Text Available Los hierros nodulares en general y los austemperizados en particular han sido usados con cada vez mayor frecuencia debido asus relevantes propiedades mecánicas en comparación con su costo.En el presente trabajo se valora el comportamiento del hierro nodular ante el trabajo en condiciones de desgaste y corrosión,luego de ser sometido a diferentes tratamientos de austemperizado.Fueron usados un hierro nodular aleado con níquel y molibdeno y otro no aleado. Ambos hierros fueron sometidos a diferentestratamientos de austemperización con mantenimientos isotérmicos a temperaturas entre 250°C y 425°C por tiempos entre 15 y180 minutos.Las muestras tratadas fueron sometidas a ensayos de desgaste por fricción en condiciones no lubricadas determinando laspendientes de desgaste uniforme para cada caso. La resistencia a la corrosión fue determinada mediante el métodopotenciométrico usando como medio el jugo de caña sintético.A partir de los resultados obtenidos se valora la influencia de los diferentes tratamientos sobre las propiedades estudiadas y sedeterminan los más efectivos desde el punto de vista técnico económico.Palabras claves: Hierro nodular, corrosión, desgaste, austemperizado.____________________________________________________________________________AbstractNodular irons and particularly austempered ductile iron has been used more and more due to their excellent mechanicalproperties in comparison with their cost.Presently work deals on behavior of nodular iron working under wear and corrosion conditions, after being submitted todifferent austempered treatments.A nodular iron alloyed with nickel and molybdenum were used as well as a not alloyed one. Both irons were treated underdifferent austempered treatment combinations using isothermal maintenance to temperatures between 250°C and 425°C andspending times between 15 and 180 minutes.Samples were submitted to non-lubricated wear using a “pin on disk” method evaluating the

  19. Low-Dose Involved-Field Radiotherapy as Alternative Treatment of Nodular Lymphocyte Predominance Hodgkin's Lymphoma

    International Nuclear Information System (INIS)

    Haas, Rick L.M.; Girinsky, Theo; Aleman, Berthe; Henry-Amar, Michel; Boer, Jan-Paul de; Jong, Daphne de

    2009-01-01

    Purpose: Nodular lymphocyte predominance Hodgkin's lymphoma is a very rare disease, characterized by an indolent clinical course, with sometimes very late relapses occurring in a minority of all patients. Considerable discussion is ongoing on the treatment of primary and relapsed disease. Patients and Methods: A group of 9 patients were irradiated to a dose of 4 Gy on involved areas only. Results: After a median follow-up of 37 months (range, 6-66), the overall response rate was 89%. Six patients had complete remission (67%), two had partial remission (22%), and one had stable disease (11%). Of 8 patients, 5 developed local relapse 9-57 months after radiotherapy. No toxicity was noted. Conclusion: In nodular lymphocyte predominance Hodgkin's lymphoma, low-dose radiotherapy provided excellent response rates and lasting remissions without significant toxicity.

  20. Effects of alloying elements on nodular and uniform corrosion resistance of zirconium-based alloys

    International Nuclear Information System (INIS)

    Abe, Katsuhiro

    1992-01-01

    The effects of alloying and impurity elements (tin, iron, chromium, nickel, niobium, tantalum, oxygen, aluminum, carbon, nitrogen, silicon, and phosphorus) on the nodular and uniform corrosion resistance of zirconium-based alloys were studied. The improving effect of iron, nickel and niobium in nodular corrosion resistance were observed. The uniform corrosion resistance was also improved by nickel, niobium and tantalum. The effects of impurity elements, nitrogen, aluminum and phosphorus were negligibly small but increasing the silicon content seemed to improve slightly the uniform corrosion resistance. Hydrogen pick-up fraction were not changed by alloying and impurity elements except nickel. Nickel addition increased remarkably hydrogen pick-up fraction. Although the composition of secondary precipitates changed with contents of alloying elements, the correlation of composition of secondary precipitates to corrosion resistance was not observed. (author)

  1. Hiperplasia nodular focal do fígado: apresentação de um caso e revisão da literatura Focal nodular hyperplasia of the liver: a case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Marise Silva Teixeira

    2007-08-01

    Full Text Available Neste trabalho apresentamos um caso de hiperplasia nodular focal que foi diagnosticado aos seis anos de idade e que está sendo acompanhado até o momento presente. Para o diagnóstico foram imprescindíveis as técnicas de imagem, tendo importância de realce a cintilografia hepatoesplênica e a tomografia computadorizada. Apresentamos, também, revisão da literatura sobre o assunto.In this case report we discuss a focal nodular hyperplasia diagnosed in a female, six-year old patient, as well as her follow-up from the diagnosis to the present time. Imaging techniques, particularly hepatosplenic scintigraphy and computed tomography, are essential for the diagnosis. Also, a literature review is presented.

  2. Nodular focal fatty infiltration of the liver: CT appearance

    International Nuclear Information System (INIS)

    Baker, M.E.; Silverman, P.M.

    1985-01-01

    Focal fatty infiltration of the liver is a well recognized entity generally characterized by a nonspherical, low-density area without significant mass effect. CT usually distinguishes this from focal liver processes such as abscess or metastasis by its sharply marginated, geographic pattern and lack of mass effect on hepatic and portal veins. Recently, the authors formed a CT scan of the liver in one patient in whom fatty infiltration appeared nodular or rounded. The clinical presentation and radiographic and pathologic features form the basis of this report

  3. Anxiety and depression are more prevalent in patients with graves' disease than in patients with nodular goitre.

    Science.gov (United States)

    Bové, Kira Bang; Watt, Torquil; Vogel, Asmus; Hegedüs, Laszlo; Bjoerner, Jakob Bue; Groenvold, Mogens; Bonnema, Steen Joop; Rasmussen, Åse Krogh; Feldt-Rasmussen, Ulla

    2014-09-01

    Graves' disease has been associated with an increased psychiatric morbidity. It is unclarified whether this relates to Graves' disease or chronic disease per se. The aim of our study was to estimate the prevalence of anxiety and depression symptoms in patients with Graves' disease compared to patients with another chronic thyroid disease, nodular goitre, and to investigate determinants of anxiety and depression in Graves' disease. 157 cross-sectionally sampled patients with Graves' disease, 17 newly diagnosed, 140 treated, and 251 controls with nodular goitre completed the Hospital Anxiety and Depression Scale (HADS). The differences in the mean HADS scores between the groups were analysed using multiple linear regression, controlling for socio-demographic variables. HADS scores were also analysed dichotomized: a score >10 indicating probable 'anxiety'/probable 'depression'. Determinants of anxiety and depression symptoms in Graves' disease were examined using multiple linear regression. In Graves' disease levels of anxiety (p = 0.008) and depression (p = 0.014) were significantly higher than in controls. The prevalence of depression was 10% in Graves' disease versus 4% in nodular goitre (p = 0.038), anxiety was 18 versus 13% (p = 0.131). Symptoms of anxiety (p = 0.04) and depression (p = 0.01) increased with comorbidity. Anxiety symptoms increased with duration of Graves' disease (p = 0.04). Neither thyroid function nor autoantibody levels were associated with anxiety and depression symptoms. Anxiety and depression symptoms were more severe in Graves' disease than in nodular goitre. Symptoms were positively correlated to comorbidity and duration of Graves' disease but neither to thyroid function nor thyroid autoimmunity.

  4. A Case Report: Krukenberg Tumour with Nodular Skin Lesions

    Directory of Open Access Journals (Sweden)

    Özden Demir

    2017-03-01

    Full Text Available The Krukenberg tumor is a rare variety of metastatic cancer to the ovary. The stomach is the most common site for the primary tumor, followed by the breast, gall bladder, colon and pancreas. It is not always possible to distinguish ovarian metastasis from primary ovarian masses in patients in whom the malignancy center is unidentified. This case report firstly aims to present a patient who presented with common nodular skin lesions, migrating arthritis and severe clinical characteristics, and diagnosed with Krukenberg tumor with further examinations.

  5. Microsegregation in Nodular Cast Iron with Carbides

    Directory of Open Access Journals (Sweden)

    S. Pietrowski

    2012-12-01

    Full Text Available In this paper results of microsegregation in the newly developed nodular cast iron with carbides are presented. To investigate the pearlitic and bainitic cast iron with carbides obtained by Inmold method were chosen. The distribution of linear elements on the eutectic cell radius was examined. To investigate the microsegregation pearlitic and bainitic cast iron with carbides obtained by Inmold method were chosen.The linear distribution of elements on the eutectic cell radius was examined. Testing of the chemical composition of cast iron metal matrix components, including carbides were carried out. The change of graphitizing and anti-graphitizing element concentrations within eutectic cell was determined. It was found, that in cast iron containing Mo carbides crystallizing after austenite + graphite eutectic are Si enriched.

  6. Microsegregation in Nodular Cast Iron with Carbides

    Directory of Open Access Journals (Sweden)

    Pietrowski S.

    2012-12-01

    Full Text Available In this paper results of microsegregation in the newly developed nodular cast iron with carbides are presented. To investigate the pearlitic and bainitic cast iron with carbides obtained by Inmold method were chosen. The distribution of linear elements on the eutectic cell radius was examined. To investigate the microsegregation pearlitic and bainitic cast iron with carbides obtained by Inmold method were chosen. The linear distribution of elements on the eutectic cell radius was examined. Testing of the chemical composition of cast iron metal matrix components, including carbides were carried out. The change of graphitizing and anti-graphitizing element concentrations within eutectic cell was determined. It was found, that in cast iron containing Mo carbides crystallizing after austenite + graphite eutectic are Si enriched.

  7. Prognostic factors in nodular lymphomas: a multivariate analysis based on the Princess Margaret Hospital experience

    International Nuclear Information System (INIS)

    Gospodarowicz, M.K.; Bush, R.S.; Brown, T.C.; Chua, T.

    1984-01-01

    A total of 1,394 patients with non-Hodgkin's lymphoma were treated at the Princess Margaret Hospital between January 1, 1967 and December 31, 1978. Overall actuarial survival of 525 patients with nodular lymphomas was 40% at 12 years; survival of patients with localized (Stage I and III) nodular lymphomas treated with radical radiation therapy was 58%. Significant prognostic factors defined by multivariate analysis included patient's age, stage, histology, tumor bulk, and presence of B symptoms. By combining prognostic factors, distinct prognostic groups have been identified within the overall population. Patients with Stage I and II disease, small or medium bulk, less than 70 years of age achieved 92% 12 year actuarial survival and a 73% relapse-free rate in 12 years of follow-up. These patients represent groups highly curable with irradiation

  8. Gray Matter Heterotopia, Mental Retardation, Developmental Delay, Microcephaly, and Facial Dysmorphisms in a Boy with Ring Chromosome 6: A 10-Year Follow-Up and Literature Review.

    Science.gov (United States)

    Liu, Shu; Wang, Zhiqing; Wei, Sisi; Liang, Jinqun; Chen, Nuan; OuYang, Haimei; Zeng, Weihong; Chen, Liying; Xie, Xunjie; Jiang, Jianhui

    2018-04-14

    Ring chromosome 6, r(6), is an extremely rare cytogenetic abnormality with clinical heterogeneity which arises typically de novo. The phenotypes of r(6) can be highly variable, ranging from almost normal to severe malformations and neurological defects. Up to now, only 33 cases have been reported in the literature. In this 10-year follow-up study, we report a case presenting distinctive facial features, severe developmental delay, and gray matter heterotopia with r(6) and terminal deletions of 6p25.3 (115426-384174, 268 kb) and 6q26-27 (168697778-170732033, 2.03 Mb) encompassing 2 and 15 candidate genes, respectively, which were detected using G-banding karyotyping, FISH, and chromosomal microarray analysis. We also analyzed the available information on the clinical features of the reported r(6) cases in order to provide more valuable information on genotype-phenotype correlations. To the best of our knowledge, this is the first report of gray matter heterotopia manifested in a patient with r(6) in China, and the deletions of 6p and 6q in our case are the smallest with the precise size of euchromatic material loss currently known. © 2018 S. Karger AG, Basel.

  9. Regenerative nodular hyperplasia, portal vein thrombosis and primary myelofibrosis: an unusual triple association.

    Science.gov (United States)

    Sández Montagut, Víctor Manuel; Giráldez Gallego, Álvaro; Ontanilla Clavijo, Guilermo

    2018-03-01

    We report a case of a regenerative nodular hyperplasia with a portal vein cavernomatosis with a subsequent progression to symptomatic, occlusive thrombosis of the superior mesenteric vein. A thorough investigation resulted in a final diagnosis of primary myelofibrosis associated with the V617F mutation in the JAK2 gene.

  10. Synthesis of nanoparticles of vanadium carbide in the ferrite of nodular cast iron

    CERN Document Server

    Fras, E; Guzik, E; Lopez, H

    2005-01-01

    The synthesis method of nanoparticles of vanadium carbide in nodular cast iron is presented. After introduction of this method, the nanoparticles with 10-70 nm of diameter was obtained in the ferrite. The diffraction investigations confirmed that these particles are vanadium carbides of type V/sub 3/C/sub 4/.

  11. Vitamin K deficiency bleeding presenting as nodular purpura in infancy: A rare and life-threatening entity

    Directory of Open Access Journals (Sweden)

    Pratik Gahalaut

    2013-01-01

    Full Text Available Vitamin K deficiency bleeding (VKDB disorder is an uncommon entity, which occurs due to inadequate activity of vitamin K-dependant coagulation factors. An 8-months-old exclusively breast-fed male infant presented with multiple, purpuric and nodular non-collapsible swellings on trunk of 4 days duration. Investigations revealed raised activated partial thromboplastin time and prothrombintime. Fibrinogen level and platelet counts were normal. Late VKDB usually presents as intra-cranial or mucosal hemorrhages. [1] Though skin and mucosal bleeding may occur in 1/3 rd of infants with VKDB, ′nodular purpura′ is not the common presenting feature. Earlier recognition of VKDB and immediate investigation/treatment helps prevent the potentially fatal outcome of the disease. Very little is mentioned about this entity in dermatology literature.

  12. The role of radioiodine therapy in benign nodular goitre

    DEFF Research Database (Denmark)

    Bonnema, Steen Joop; Fast, Søren; Hegedüs, Laszlo

    2014-01-01

    For treatment of benign nodular goitre the choice usually stands between surgery and (131)I therapy. (131)I therapy, used for 30 years for this condition, leads to a goitre volume reduction of 35-50% within 1-2 years. However, this treatment has limited efficacy if the thyroid (131)I uptake is low...... for additional therapy due to insufficient goitre reduction, but the price is a higher rate of hypothyroidism. Another approach with rhTSH-stimulation is to reduce the administered (131)I activity by a factor that equals the increase in the thyroid (131)I uptake. Using this approach, radiation exposure...

  13. Carbides in Nodular Cast Iron with Cr and Mo

    Directory of Open Access Journals (Sweden)

    S. Pietrowski

    2007-07-01

    Full Text Available In these paper results of elements microsegregation in carbidic nodular cast iron have been presented. A cooling rate in the centre of the cross-section and on the surface of casting and change of moulding sand temperature during casting crystallization and its self-cooling have been investigated. TDA curves have been registered. The linear distribution of elements concentration in an eutectic grain, primary and secondary carbides have been made. It was found, that there are two kinds of carbides: Cr and Mo enriched. A probable composition of primary and secondary carbides have been presented.

  14. Cavitation Erosion of Nodular Cast Iron − Microstructural Effects

    Directory of Open Access Journals (Sweden)

    Orłowicz A.W.

    2017-12-01

    Full Text Available The paper deals with susceptibility of nodular cast iron with ferritic-pearlitic matrix on cavitation erosion. Cavitation tests were carried out with the use of a cavitation erosion vibratory apparatus employing a vibration exciter operated at frequency of 20 kHz. The study allowed to determine the sequence of subsequent stages in which microstructure of cast iron in superficial regions is subject to degradation. The first features to be damaged are graphite precipitates. The ferritic matrix of the alloy turned out to be definitely less resistant to cavitation erosion compared to the pearlitic matrix component.

  15. Digital Cities in the making: exploring perceptions of space, agency of actors and heterotopia

    Directory of Open Access Journals (Sweden)

    Asne Kvale Handlykken

    2011-12-01

    Full Text Available

    This paper is an attempt to explore how we imagine, sense and experience spaces in digital cities by a study of the hybrid relations between digital media, users' bodies, architecture and the city. Digital and physical spaces of the city are intertwined, the city and urban places and things become sentient, embedded with sensors and digital infrastructure, challenging traditional notions of space, and how we perceive and experience urban space.  Crucial issues to explore are how interactions and agency operating amongst actors in these spaces; between sentient non-human actors, places and people?  How are spaces of interaction embedded in the city, what characterizes these spaces, can they be explored as heterotopias (Foucault? These processes are a mutual shaping of society and technology, where the role of the imaginary, of mental representations and creation are being transformed.

  16. Utopia, heterotopia e nostalgia na performance dublada de música pop

    Directory of Open Access Journals (Sweden)

    Alan Mangabeira Mascarenhas

    2016-09-01

    Full Text Available Refletir fãs no cenário atual traz à tona uma visão antropológica de uma experiência estética que parece destacar a nostalgia dos consumidores da narrativa do ídolo. Neste trabalho, partimos da hipótese de que é a nostalgia um dos principais elos que mantém um fandom de música pop através da narrativa de seu ídolo no palco e fora dele. Propomos uma análise do show-fixo, que aqui chamamos de “monumento”, o “Piece of Me”, no qual a performer Britney Spears rememora momentos de sua carreira no teatro do Planet Hollywood em Las Vegas fazendo uso de dublagem em todo o show e artefatos corporais para simular sua aparência e voz de outrora numa cidade quase fantasma. Questionamos, então, como se dá a construção narrativa do espaço de pertencimento na música pop capaz de gerar nostalgia através dos conceitos de utopia e heterotopia (Foucault, a noção de “lar” (Boym.

  17. Fascitis nodular cervical en paciente gestante: revisión de la literatura y presentación de un caso Cervical nodular fasciitis in a pregnant woman: Review of the literature and presentation of a new case

    Directory of Open Access Journals (Sweden)

    Manuel Acosta-Feria

    2010-09-01

    Full Text Available La fascitis nodular es una lesión benigna rara, de crecimiento rápido, cuya patogenia es aún desconocida. Si bien a nivel de cabeza y cuello aparece en un 20% de los casos, su aparición en pacientes gestantes es extremadamente rara. Presentamos el segundo caso descrito en la literatura en el cual está presente dicha asociación. Mujer de 25 años y gestante de 13 semanas, con una tumoración retroauricular derecha de 3 cm de diámetro, 3 meses de evolución y rápido crecimiento. No presentaba parálisis facial ni dolor en la exploración inicial. Tras la exéresis quirúrgica de la lesión, el diagnóstico anatomopatológico definitivo de ésta, fue de fascitis nodular. Tras 2 años desde la intervención quirúrgica no se ha evidenciado recidiva de la tumoración, llevándose el embarazo a término sin presentar complicaciones ni para la madre ni para el feto.The nodular fasciitis is a rare benign lesion of rapid growth, whose pathogenesis is still unknown. While at the head and neck appeared in 20% of cases, its occurrence in pregnant patients is extremely rare. We report the second case described in the literature in which this association is present. Woman of 25 years and 13 weeks pregnant, who had a right auricular tumour 3 cm in diameter, 3 months of development and rapid growth. No facial paralysis or pain in the initial exploration. After surgical resection of the lesion, the final pathological diagnosis of the same was nodular fasciitis. After two years since the surgery, there was no evidence of recurrence of the tumour, taking ad términun pregnancy without complications or the mother or the fetus.

  18. Mutational spectrum of intraepithelial neoplasia in pancreatic heterotopia.

    Science.gov (United States)

    Ma, Changqing; Gocke, Christopher D; Hruban, Ralph H; Belchis, Deborah A

    2016-02-01

    Heterotopic pancreatic parenchyma recapitulates the normal pancreas in extrapancreatic locations and, on rare occasions, can even give rise to pancreatic adenocarcinoma. The genetic signatures of pancreatic adenocarcinoma and its precursor lesions are well characterized. We explored the genetic alterations in precursor lesions (intraductal papillary mucinous neoplasms [IPMN], pancreatic intraepithelial neoplasia [PanIN]) in patients with pancreatic heterotopias but without concomitant pancreatic ductal adenocarcinomas. This allowed us to determine whether the stereotypical dysplasia--infiltrating carcinoma sequence also occurs in these extrapancreatic foci. Seven cases of heterotopic pancreas with ductal precursor lesions were identified. These included 2 IPMNs with focal high-grade dysplasia and 5 PanINs with low- to moderate-grade dysplasia (PanIN grades 1-2). Neoplastic epithelium was microdissected and genomic DNA was extracted. Sequencing of commonly mutated hotspots (KRAS, TP53, CDKN2A, SMAD4, BRAF, and GNAS) in pancreatic ductal adenocarcinoma and its precursor lesions was performed. Both IPMNs were found to have KRAS codon 12 mutations. The identification of KRAS mutations suggests a genetic pathway shared with IPMN of the pancreas. No mutations were identified in our heterotopic PanINs. One of the possible mechanisms for the development of dysplasia in these lesions is field effect. At the time of these resections, there was no clinical or pathologic evidence of a prior or concomitant pancreatic lesion. However, a clinically undetectable lesion is theoretically possible. Therefore, although a field effect cannot be excluded, there was no evidence for it in this study. Copyright © 2015 Elsevier Inc. All rights reserved.

  19. Evaluation of peanut hulls as an alternative to bleaching clays

    Energy Technology Data Exchange (ETDEWEB)

    Hassanein, M. M.; El-Shami, S. M.; Taha, F. S.

    2011-07-01

    Peanut hulls (PNH) were carbonized at different temperatures, times, and evaluated at different concentrations as an alternative to bleaching clays. Evaluation of bleached crude soybean oil with PNH was based on their delta free fatty acids, reduction in peroxide value (PV), reduction in phospholipids (PL) and bleachability. The performance of several commercially used bleaching clays was evaluated, for comparison. Mixtures were formulated including: PNH and Tonsil -N (TN), PNH and Fuller's earth (FE) and PNH and O-passive (OP) and examined. The oxidative stability of oils was determined. Results for the investigated commercial bleaching clays revealed: TN > FE > F > TF > OP. Highest reduction in PV and PL, and highest bleachability were achieved for soybean oil bleached with 2% PNH carbonized at 500 degree centigrade for 30 min (PNH). Mixtures of PNH with the three chosen bleaching clays indicated that 1PNH : 2TN gave the highest bleachability. CSO was miscella bleached in hexane using PNH and resulted in an appreciable improvement in all oil characteristics, especially in bleachability. Oxidative stability of oils was in the following order: TN > control > FE > PNH with Induction period values of 23.1 > 6.43 > 5.73 > 2.85 h, respectively. (Author) 20 refs.

  20. A report of unusual diffuse nodular lesions in intestines of lambs with coccidiosis

    Directory of Open Access Journals (Sweden)

    a.r Taghavi Razavizadeh

    2016-11-01

    Full Text Available Ovine and caprine coccidiosis has always been regarded as one of the important diseases with high morbidity and economic losses such as weight loss, death and costs of treatment and control. In a sheep farm, 4 sick newborn lambs (out of 10 lambs with the age of 1 to 2 months died with clinical signs of weight loss, loss of appetite, yellowish diarrhea, fever, ruffled wool, contamination of tail and perineal area to diarrheic stool and abdominal pain at the touch of abdomen. At necropsy on one lamb, multiple and diffuse nodular hyperplastic lesions yellowish-white in color were seen in the mucosal surface of jejunum. Microscopically, in addition to hyperplasia of the small intestinal villi, various developmental stages of Eimeria were observed. Based on morphological characteristics and presence of numerous polyp like formations in small intestine, the involved protozoan species was determined as Eimeria bakuensis. The relatively high case fatality rate in this flock could be related to sever nodular lesions in the small intestines, age and immune status of the affected animals.

  1. Prevalence of malignancy in resected specimen of patients operated for benign nodular goitre

    International Nuclear Information System (INIS)

    Moosa, F.A.; Junaid, M.; Khan, F.W.; Afzal, Y.

    2006-01-01

    To determine the frequency of malignancy on histopathology amongst resected specimen of thyroid gland in patients, who had no evidence of malignancy pre-operatively on clinical grounds and investigations. A total of 190 patients who were operated for benign nodular thyroid disease during the study period; 100 cases had multinodular goitre and 90 solitary nodules. Biodata, clinical features, investigations, diagnosis, details of surgery, complications and histopathology reports of all the patients were reviewed and analyzed. Amongst the 190 patients the mean age was 33.42+-12.4 years (range 17-45 years), while the male: female ratio was 1:6.6. Seven (3.6%) cases were found to be malignant on histopathology, with a frequency of 3% (3/100) amongst multinodular cases and 4.4% (4/90) amongst solitary nodules. Prevalence of malignancy in multinodular goitre does not differ significantly from solitary nodules. Hence, multinodularity should no longer be considered as an indicator of benign disease. Both varieties of nodular goitres should be considered for surgery even if there is no suspicion of malignancy. (author)

  2. Ultrasound-based scores as predictors for nodular hyperplasia in patients with secondary hyperparathyroidism: a prospective validation study.

    Science.gov (United States)

    Gwiasda, Jill; Kaltenborn, Alexander; Müller, Jörg A; Serttas, Michaela; Scheumann, Georg W F; Schrem, Harald; Jäger, Mark D

    2017-03-01

    Former studies evaluated echostructural and vascular patterns in ultrasound of the parathyroid gland to identify nodular hyperplasia in patients with secondary hyperparathyroidism due to chronic kidney disease. This prospective study aims to externally validate suggested ultrasound classifications. Parathyroid glands of 27 patients with secondary hyperparathyroidism undergoing parathyroidectomy were prospectively analyzed. Ultrasound including Doppler imaging was performed 1 day prior to surgery. Ultrasound data were available for 70 parathyroid glands. Echostructural and vascular scores according to previous studies were applied calculating the area under the receiver operating characteristic curve (AUROC). Overall correctness, sensitivity, and specificity of the investigated scores were assessed with the Youden index method. The Doppler score introduced by Vulpio and colleagues based on characteristic blood flow patterns in parathyroid glands showed an AUROC of 0.749 for the prediction of nodular hyperplasia with an overall correctness of 72.8%. Other ultrasound classifications based on blood flow patterns, as well as echostructure of the parathyroid gland displayed AUROCs of secondary hyperparathyroidism was externally validated for the first time. Other ultrasound scores fail as prognostic models in this study population. Doppler sonography of the parathyroid gland has prognostic capability to identify nodular hyperplasia as surrogate marker for patients with secondary hyperparathyroidism indicating the need for ablative or surgical treatment when failing conservative therapy.

  3. Papulo-Nodular Reactions in Black Tattoos as Markers of Sarcoidosis

    DEFF Research Database (Denmark)

    Sepehri, Mitra; Hutton Carlsen, Katrina; Serup, Jørgen

    2016-01-01

    BACKGROUND/AIMS: Sarcoidosis is, from historical data, suggested to be more prevalent among patients with tattoo reactions. We aimed to evaluate this association in a systematic study. METHODS: This is a consecutive study of patients with tattoo complications, diagnosed in the "Tattoo Clinic......" at Bispebjerg University Hospital in Copenhagen, Denmark, from 2008 to 2015, based on clinical assessment and histology. From the overall group of 494 tattoo complications in 406 patients, 92 reactions in 72 patients showed a papulo-nodular pattern studied for local and systemic sarcoidosis, since sarcoidosis...

  4. Quality-of-Life Impairments Persist Six Months After Treatment of Graves' Hyperthyroidism and Toxic Nodular Goiter: A Prospective Cohort Study.

    Science.gov (United States)

    Cramon, Per; Winther, Kristian Hillert; Watt, Torquil; Bonnema, Steen Joop; Bjorner, Jakob Bue; Ekholm, Ola; Groenvold, Mogens; Hegedüs, Laszlo; Feldt-Rasmussen, Ulla; Rasmussen, Åse Krogh

    2016-08-01

    The treatment of hyperthyroidism is aimed at improving health-related quality of life (HRQoL) and reducing morbidity and mortality. However, few studies have used validated questionnaires to assess HRQoL prospectively in such patients. The purpose of this study was to assess the impact of hyperthyroidism and its treatment on HRQoL using validated disease-specific and generic questionnaires. This prospective cohort study enrolled 88 patients with Graves' hyperthyroidism and 68 with toxic nodular goiter from endocrine outpatient clinics at two Danish university hospitals. The patients were treated with antithyroid drugs, radioactive iodine, or surgery. Disease-specific and generic HRQoL were assessed using the thyroid-related patient-reported outcome (ThyPRO) and the Medical Outcomes Study 36-item Short Form (SF-36), respectively, evaluated at baseline and six-month follow-up. The scores were compared with those from two general population samples who completed ThyPRO (n = 739) and SF-36 (n = 6638). Baseline scores for patients with Graves' hyperthyroidism and toxic nodular goiter were significantly worse than those for the general population scores on all comparable ThyPRO scales and all SF-36 scales and component summaries. ThyPRO scores improved significantly with treatment on all scales in Graves' hyperthyroidism and four scales in toxic nodular goiter, while SF-36 scores improved on five scales and both component summaries in Graves' hyperthyroidism and only one scale in toxic nodular goiter. In Graves' hyperthyroidism, large treatment effects were observed on three ThyPRO scales (Hyperthyroid Symptoms, Tiredness, Overall HRQoL) and moderate effects on three scales (Anxiety, Emotional Susceptibility, Impaired Daily Life), while moderate effects were seen in two ThyPRO scales in toxic nodular goiter (Anxiety, Overall HRQoL). However, significant disease-specific and generic HRQoL deficits persisted on multiple domains across both patient groups. Graves

  5. Habitus, Heterotopia and Endocolonialism in Early Spanish Literary Fascism

    Directory of Open Access Journals (Sweden)

    Nil Santiáñez

    2009-06-01

    Full Text Available This article explores strategies of symbolic production of national space (e.g. technologies of tropological striation in early fascist works of Tomás Borrás, Luys Santa Marina, and Rafael Sánchez Mazas written à propos the Rif War (1919-27. Considered as perlocutionary speech-acts, these texts conceive Morocco as a heterotopia and embody a fascist habitus produced by a heterogeneous group of writers, intellectuals, politicians and military personnel—in particular the notorious Foreign Legion—posted in Morocco; they all shared the defense of an authoritarian concept of nation as a model for the political organization of Spain as well as an endocolonialist gaze and stance towards their own country. By means of its tropological conquest of Moroccan territory, Fascist writing devoted to the Rif War duplicated the empirical spatial production carried out in situ by the army and the civilian administration of the Spanish protectorate of Morocco. Making it intelligible as well as modifying it, such writing brought to the Peninsula an endocolonial project and an incipient fascist habitus. Its development in the 1930s (the theory of fascism, Falange Española, the Falangists’ direct action in the streets of Spain, the tactics and strategy followed by the High Command of Franco’s army during the civil war would culminate after 1939 in the empiric production of a new administrative, political and economic organization of Spain’s national territory.

  6. Pathophysiology of the nodular and micronodular small bowel fold

    International Nuclear Information System (INIS)

    Olmstead, W.W.; Ros, P.R.; Moser, R.P.; Shekitka, K.M.; Lichtenstein, J.E.; Buck, J.L.

    1987-01-01

    The normal small bowel fold is easily seen on conventional studies of the small intestine, but visualization of the small bowel villus is just at the resolution of current roentgenographic technique. When the villi are enlarged, they can be seen radiographically as an irregularity or micronodularity of the small bowel fold. The anatomy of the fold and the pathophysiology of diseases producing fold nodularity (tumor, inflammatory disease, NLH, mastocytosis) and micronodularity (lymphangiectasia, Waldenstrom macroglobulinemia, Whipple disease) are presented, with an emphasis on radiologic-pathologic correlation. The radiologist should suggest certain diseases or conditions based on the roentgenographic characteristics of the closely analyzed small bowel fold

  7. New data on aetiology of nodular gill disease in rainbow trout, Oncorhynchus mykiss

    Czech Academy of Sciences Publication Activity Database

    Dyková, Iva; Kostka, Martin; Wortberg, F.; Nardy, E.; Pecková, Hana

    2010-01-01

    Roč. 57, č. 3 (2010), s. 157-163 ISSN 0015-5683 R&D Projects: GA ČR GA524/09/0137; GA MŠk LC522 Institutional research plan: CEZ:AV0Z60220518 Keywords : nodular gill disease * aetiological study * amoebae * Naegleria sp. * fish diseases * aquaculture * Oncorhynchus mykiss Subject RIV: GJ - Animal Vermins ; Diseases, Veterinary Medicine Impact factor: 1.533, year: 2010

  8. Ehlers-Danlos syndrome: a cause of epilepsy and periventricular heterotopia.

    Science.gov (United States)

    Verrotti, Alberto; Monacelli, Debora; Castagnino, Miriam; Villa, Maria Pia; Parisi, Pasquale

    2014-11-01

    Ehlers-Danlos syndrome (EDS) comprises a variety of inherited connective tissue disorders that have been described in association with various neurological features. Until now the neurological symptoms have not been studied in detail; therefore, the aim of this review is to analyze the possible association between EDS, epilepsy and periventricular heterotopia (PH). We have carried out a critical review of all cases of epilepsy in EDS patients with and without PH. Epilepsy is a frequent neurological manifestation of EDS; generally, it is characterized by focal seizures with temporo-parieto-occipital auras and the most common EEG findings epileptiform discharges and slow intermittent rhythm with delta-theta waves. Epilepsy in EDS patients is usually responsive to common antiepileptic therapy; very few cases of drug resistant focal epilepsy requested surgical treatment, with favorable results in terms of outcome. Epilepsy is the most common presenting neurological manifestation associated with PH in EDS patients. Abnormal anatomic circuitries (including heterotopic nodules) could generate epilepsy in patients with PH. Among the principal neurological manifestations, epilepsy and PH have a considerable importance and can influence the long-term evolution of these patients. We hypothesize that PH may determine the epileptic manifestations in patients with EDS; much remains to be learnt about the relationships between nodules and the epileptic manifestations in EDS syndrome. Copyright © 2014 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

  9. Likelihood ratio-based differentiation of nodular Hashimoto thyroiditis and papillary thyroid carcinoma in patients with sonographically evident diffuse hashimoto thyroiditis: preliminary study.

    Science.gov (United States)

    Wang, Liang; Xia, Yu; Jiang, Yu-Xin; Dai, Qing; Li, Xiao-Yi

    2012-11-01

    To assess the efficacy of sonography for discriminating nodular Hashimoto thyroiditis from papillary thyroid carcinoma in patients with sonographically evident diffuse Hashimoto thyroiditis. This study included 20 patients with 24 surgically confirmed Hashimoto thyroiditis nodules and 40 patients with 40 papillary thyroid carcinoma nodules; all had sonographically evident diffuse Hashimoto thyroiditis. A retrospective review of the sonograms was performed, and significant benign and malignant sonographic features were selected by univariate and multivariate analyses. The combined likelihood ratio was calculated as the product of each feature's likelihood ratio for papillary thyroid carcinoma. We compared the abilities of the original sonographic features and combined likelihood ratios in diagnosing nodular Hashimoto thyroiditis and papillary thyroid carcinoma by their sensitivity, specificity, and Youden index. The diagnostic capabilities of the sonographic features varied greatly, with Youden indices ranging from 0.175 to 0.700. Compared with single features, combinations of features were unable to improve the Youden indices effectively because the sensitivity and specificity usually changed in opposite directions. For combined likelihood ratios, however, the sensitivity improved greatly without an obvious reduction in specificity, which resulted in the maximum Youden index (0.825). With a combined likelihood ratio greater than 7.00 as the diagnostic criterion for papillary thyroid carcinoma, sensitivity reached 82.5%, whereas specificity remained at 100.0%. With a combined likelihood ratio less than 1.00 for nodular Hashimoto thyroiditis, sensitivity and specificity were 90.0% and 92.5%, respectively. Several sonographic features of nodular Hashimoto thyroiditis and papillary thyroid carcinoma in a background of diffuse Hashimoto thyroiditis were significantly different. The combined likelihood ratio may be superior to original sonographic features for

  10. Postoperative recurrent nontoxic nodular (multinodular goiter

    Directory of Open Access Journals (Sweden)

    Tolstokorov A.S.

    2011-12-01

    Full Text Available The extensive use of modern methods of examination (ultrasound investigation with Doppler mapping, aspiration (needle biopsy, radiodiagnostics cannot resolve the problem of verification of nodular mass in case of multinodular goiter before operation. Operative intervention is the method of choice in this case, which gives rise to the problem of surgery extent. The present study is useful in improvement of remote results of surgical treatment of multinodular goiter through statistical estimation of recurrent goiter development. The study was retrospective and included 102 consecutively admitted and operated patients with recurrent goiter and 102 patients of control group with first diagnosed and operated goiter. Patients of both groups were in euthyroid state. Patients were admitted to the Clinic of Surgery and Oncology of Raising Skills Faculty of Saratov State Medical University n.a. V. I. Razumovsky from 2000 till 2004. Statistical analyses included calculation of average mean, standard deviation and mean error. Student's ratio was used as the test of validity of average mean. Chi-square test was used for assessment of qualitative characteristics.

  11. Nucleation and growth of intermetallic precipitates in Zircaloy-2 and zircaloy-4 and correlation to nodular corrosion behavior

    International Nuclear Information System (INIS)

    Maussner, G.; Steinberg, E.; Tenckhoff, E.

    1987-01-01

    One of the fundamental aspects in the history of the development of zirconium alloys for nuclear applications is the corrosion behavior under in-pile conditions. In boiling-water reactors (BWRs) and pressurized-water reactors (PWRs) the zirconium alloys Zircaloy-2 and Zircaloy-4 are the most commonly used materials, permitting attainment of a very high level of integrity and reliability. Nevertheless, efforts are required to optimize these well-established alloys with regard to their resistance to nodular corrosion, where improvements will give long-term advantages in fuel integrity and fuel economy. Phenomenological studies allow correlation of the nodular corrosion behavior with the morphological appearance of precipitated intermetallic phases in the microstructures of Zry-2 and Zry-4. To understand the fundamental processes of precipitation, particle nucleation-and-growth studies were made with Zry-2 and Zry-4 in different fabrication dimensions and with variations in β-quenching rates followed by isothermal and isochronical heat treatments. The microstructural characteristics of the precipitates were investigated by optical and transmission-electron microscopy. The macroscopic behavior was studied by electrical-resistivity measurements and hardness measurements. The nodular-corrosion susceptibility was determined by weight-gain and nodule distribution measurements after a 500 0 C laboratory-autoclave test

  12. Neuropsychiatric symptoms and diagnosis of grey matter heterotopia: A case-based reflection

    Directory of Open Access Journals (Sweden)

    Gian Lippi

    2017-03-01

    Full Text Available Neuropsychiatric symptoms can be related to less common underlying neuropsychiatric conditions – in this case report, the condition discussed is that of grey matter heterotopia (GMH. The patient presented with a history of prominent aggression, impulsivity and manipulative and attention-seeking behaviour. Episodes of depression and incidents of deliberate self-harm and suicide attempts had been reported. Neuropsychiatric symptoms included anxiety, a labile mood, delusional thinking and auditory hallucinations. Testing revealed some cognitive difficulties and severe impairment of frontal lobe functions. A magnetic resonance imaging (MRI scan of his brain revealed the presence of GMH, which had previously been misdiagnosed as tuberous sclerosis. An MRI scan of the brain is the special investigation of choice for the correct diagnosis of GMH. The pathognomonic finding is that of heterotopic grey matter abnormally located within areas of white matter. Defective foetal neuronal migration between the third and fifth month of pregnancy can lead to GMH, which can present later on in childhood or adolescence with epilepsy, intellectual impairment or reading difficulties. During the late teenage years or early adulthood, a wide variety of neuropsychiatric symptoms may be present, which can lead to diagnostic difficulties.

  13. Dynamic fracture toughness and evaluation of fracture in a ferritic nodular cast iron for casks

    International Nuclear Information System (INIS)

    Yasunaka, T.; Nakano, K.

    1993-01-01

    The effect of loading rate and temperature on fracture toughness of a ferritic nodular cast iron obtained from a thick-walled cylindrical casting has been investigated. Based upon this result, the cast iron is evaluated as a material for casks. (1) In the ductile fracture region, fracture toughness increases with increases in loading rate. (2) Ductile-brittle transition temperature is linearly related to the logarithm of stress intensity rate. (3) In the ductile fracture region, converted plain strain fracture toughness divided by yield stress can be adopted as a material constant which is independent of loading rate and temperature. From the result of a static fracture toughness test, the evaluation of fracture in high loading rate can be made. (4) In the ductile fracture region of the material investigated, the maximum allowable flaw depth exceeded the minimum detectable flaw size by a nondestructive inspection. Ferritic nodular cast iron can be used as a material for casks in the ductile fracture region at least. (J.P.N.)

  14. [Nocardia brasiliensis leg ulcer and nodular lymphangitis in France].

    Science.gov (United States)

    Hamm, M; Friedel, J; Siré, C; Semon, J

    2005-01-01

    Nocardia brasiliensis is a very rarely reported cause of chronic phagedenic ulcerations. We report the case of an elderly woman who developed such an infection after falling on her right leg on the road in the Bresse country (an essentially agricultural and bovine-cattle breading region) and developed a chronic phagedenic ulcer secondarily complicated by nodular lymphangitis of the thigh. A 75 year-old woman fell on her right leg on the side of the main road outside her hamlet in the Bresse country and secondarily developed a chronique phagedenic ulceration. We first considered her as suffering from pyoderma gangrenosum. A complete scanning only revealed an autoimmune thyroiditis and a rapidly healing gastric ulceration, and none of the treatments, either local or systemic, helped the skin condition to heal. After 3 weeks of application of a local corticoid ointment, the patient developed fever, general malaise, an exacerbation of her wound and an infiltration of the skin round her knee, together with nodular lymphangitic dissemination. A supplementary bacterial swab disclosed massive proliferation of a slow-growing Gram-positive bacillus, which proved to be Nocardia brasiliensis, together with a methicillino-sensitive Staphylococcus aureus. The treatment with sulfamethoxazole-trimetoprim gave a rash after 12 hours and was changed to amoxicillin and clavulanic acid, which rapidly proved to be permanently effective. The revelation of this particular slow-growing bacteria is difficult and requires bacterial swabs. Nocardia brasiliensis is relatively rare in primary skin ulcerations and we discuss the reasons why an elderly women should find this bacteria on the road outside her hamlet in the French countryside. This particular infectious condition requires general scanning, to make sure that the primary skin condition does not extend to other organs. We review the therapeutical options for patients who exhibit allergic reactions to the classically effective

  15. Compromiso nodular hepático secundario a mieloma múltiple Nodular hepatic lesions secondary to multiple myeloma

    Directory of Open Access Journals (Sweden)

    Fernando J. Vázquez

    2010-08-01

    Full Text Available El mieloma múltiple (MM es una enfermedad maligna caracterizada por una proliferación clonal de células plasmáticas atípicas en la médula ósea, que produce una inmunoglobulina (Ig con estructura homogénea en la sangre y/o en la orina (cadenas livianas. El compromiso del parénquima hepático con masa ocupante en los pacientes con MM es excepcional. Describimos 3 casos clínicos confirmados histológicamente. La ecografía y la tomografía computarizada tienen una sensibilidad intermedia y las imágenes no son patognomónicas, por lo tanto es necesaria la confirmación a través de la biopsia. Se desconoce la implicancia pronóstica de este hallazgo.Multiple myeloma is characterized by the neoplastic proliferation of a single clone of plasma cells producing a monoclonal immunoglobulin. Myelomatous nodular lesions of the liver are infrequent. We describe 3 cases with histological confirmation and we review the bibliography.

  16. Anxiety and depression are more prevalent in patients with graves' disease than in patients with nodular goitre

    DEFF Research Database (Denmark)

    Bové, Kira Bang; Watt, Torquil; Vogel, Asmus

    2014-01-01

    BACKGROUND AND OBJECTIVE: Graves' disease has been associated with an increased psychiatric morbidity. It is unclarified whether this relates to Graves' disease or chronic disease per se. The aim of our study was to estimate the prevalence of anxiety and depression symptoms in patients with Graves......' disease compared to patients with another chronic thyroid disease, nodular goitre, and to investigate determinants of anxiety and depression in Graves' disease.METHODS: 157 cross-sectionally sampled patients with Graves' disease, 17 newly diagnosed, 140 treated, and 251 controls with nodular goitre......'/probable 'depression'. Determinants of anxiety and depression symptoms in Graves' disease were examined using multiple linear regression.RESULTS: In Graves' disease levels of anxiety (p = 0.008) and depression (p = 0.014) were significantly higher than in controls. The prevalence of depression was 10% in Graves...

  17. Nodular hidradenocarcinoma over the parotid gland: a pathologic presentation.

    Science.gov (United States)

    Verret, D J; Kabbani, Wareef; DeFatta, Robert J

    2007-02-01

    Nodular hidradenocarcinoma (NHAC), an eccrine carcinoma, has been reported in the dermatology and pathology literature, but few references have been made in the otolaryngology literature even though the head and neck is a common site of occurrence. A case report of a 37-year-old Hispanic man with a right-sided neck mass diagnosed preoperatively as a parotid mass by imaging and fine-needle aspiration is presented. After presentation at our multidisciplinary tumor board, excision of the mass was undertaken. Final pathology revealed a NHAC, which is presented in our report. NHAC is an aggressive malignant tumor that is often misdiagnosed preoperatively and that must be treated with aggressive multimodality therapy for increased survival.

  18. Diffuse Muscular Pain, Skin Tightening, and Nodular Regenerative Hyperplasia Revealing Paraneoplastic Amyopathic Dermatomyositis due to Testicular Cancer

    Directory of Open Access Journals (Sweden)

    Sarah Norrenberg

    2012-01-01

    Full Text Available Paraneoplastic dermatomyositis (DM associated with testicular cancer is extremely rare. We report the case of a patient with skin tightening, polymyalgia, hypereosinophilia, and nodular regenerative hyperplasia revealing seminoma and associated paraneoplastic DM.

  19. Thyroid nodularity and chromosome aberrations among women in areas of high background radiation in China

    International Nuclear Information System (INIS)

    Wang, Z.Y.; Boice, J.D. Jr.; Wei, L.X.; Beebe, G.W.; Zha, Y.R.; Kaplan, M.M.; Tao, Z.F.; Maxon, H.R. III; Zhang, S.Z.; Schneider, A.B.

    1990-01-01

    Thyroid nodularity following continuous low-dose radiation exposure in China was determined in 1,001 women aged 50-65 years who resided in areas of high background radiation (330 mR/yr) their entire lives, and in 1,005 comparison subjects exposed to normal levels of radiation (114 mR/yr). Cumulative doses to the thyroid were estimated to be of the order of 14 cGy and 5 cGy, respectively. Personal interviews and physical examinations were conducted, and measurements were made of serum thyroid hormone levels, urinary iodine concentrations, and chromosome aberrations in circulating lymphocytes. For all nodular disease, the prevalences in the high background and control areas were 9.5% and 9.3%, respectively. For single nodules, the prevalences were 7.4% in the high background area and 6.6% in the control area (prevalence ratio = 1.13; 95% confidence interval = 0.82-1.55). There were no differences found in serum levels of thyroid hormones. Women in the high background region, however, had significantly lower concentrations of urinary iodine and significantly higher frequencies of stable and unstable chromosome aberrations. Increased intake of allium vegetables such as garlic and onions was associated with a decreased risk of nodular disease, which seems consistent with experimental studies suggesting that allium compounds can inhibit tumor growth and proliferation. The prevalence of mild diffuse goiter was higher in the high background radiation region, perhaps related to a low dietary intake of iodine. These data suggest that continuous exposure to low-level radiation throughout life is unlikely to appreciably increase the risk of thyroid cancer. However, such exposure may cause chromosomal damage

  20. Some approaches to treatment of patients with thyroid nodular diseases in the Semipalatinsk region of Kazakhstan.

    Science.gov (United States)

    Zhumadilov, Zhaxybay; Hoshi, Masaharu; Takeichi, Nobuo; Abisheva, Gulniaz; Taooka, Yasuyuki; Bhattarcharjee, Deborshi; Kamiya, Kenji

    2003-12-01

    It was determined by experts that in the region adjacent to the Semipalatinsk Nuclear Test Site thyroid nodular prevalence was significantly associated with radiation dose from nuclear weapons testing. The medical rehabilitation of patients is of special practical and scientific importance in this region. Some patients have contraindications for surgery and radioiodine or refuse them for various reasons. Percutaneous intranodular injection therapy by "Paoscle" (PIITP) was used for treatment of patients with benign thyroid nodular diseases. The study group included a total of 107 patients (mainly "pretoxic" and "compensated" nodules). Seventy-four patients received PIITP on an out-patient basis. Seventy-two of them were women and 2 men, mean age, 52.9 +/- 1.3 years; range 26 to 77 years. Thyroid ultrasound examination, fine-needle aspiration biopsy, thyroid function tests and cytopathology were performed in all patients to evaluate the effectiveness of treatment. The nodule volume reduction rate for patients with thyroid adenomas was 56 % on average and 60.2 % in the case of colloid nodules. The reduction rate of thyroid adenomas with necrotic and cystic degeneration was higher and reached 72 %. The suggested method is indicated for cases of benign nodular thyroid diseases (cysts, adenomas, adenomas with necrotic and cystic degeneration, colloid nodules, polynodular goiter). We did not reveal any complications or thyroid test abnormalities after the treatment. A tendency to normalization of the blood serum thyroglobulin level and antibodies to thyroglobulin was documented. Analysis of the treatment results revealed that this approach is effective, inexpensive, safe, well tolerated and can be used on an outpatient basis.

  1. Nodular Esophageal Xanthoma: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Ahmed Dirweesh

    2017-01-01

    Full Text Available Xanthomas are localized nonneoplastic lesions within tissues that may manifest as papules, plaques, or nodules. These lesions can be found anywhere along the gastrointestinal tract, commonly in the stomach and colon, and rarely in the small intestine and esophagus. Esophagogastroduodenoscopy (EGD with biopsy is the gold standard tool for diagnosis. Here, we report a rare case of a lower solitary nodular esophageal xanthoma in an elderly black female. Correspondingly, all cases of esophageal xanthomas reported in the English medical literature were reviewed and presented with the reported case.

  2. Lymphoid nodular hyperplasia in a patient with severe combined immunodeficiency disease

    International Nuclear Information System (INIS)

    Sanchez-Alegre, M. L.; Casanova, A.; Delgado, J.; Relanzon, S.

    2001-01-01

    We describe a case of lymphoid nodular hyperplasia in a woman with severe combined immunodeficiency disease. the patient complained of constipation and episodes of abdominal pain, and examination revealed the presence of a large abdominal mass. The diagnosis was suspected on the basis of the initial radiological studies, but intestinal biopsy was necessary to rule out lymphomatous involvement. We point out the radiological features of this entity which, despite the fact that it may be a chance finding of no pathological significance, requires special attention, especially in immuno deficient individuals. (Author) 10 refs

  3. A Novel Missense Mutation of Doublecortin: Mutation Analysis of Korean Patients with Subcortical Band Heterotopia

    Science.gov (United States)

    Kim, Myeong-Kyu; Park, Man-Seok; Kim, Byeong-Chae; Cho, Ki-Hyun; Kim, Young-Seon; Kim, Jin-Hee; Heo, Tag; Kim, Eun-Young

    2005-01-01

    The neuronal migration disorders, X-linked lissencephaly syndrome (XLIS) and subcortical band heterotopia (SBH), also called "double cortex", have been linked to missense, nonsense, aberrant splicing, deletion, and insertion mutations in doublecortin (DCX) in families and sporadic cases. Most DCX mutations identified to date are located in two evolutionarily conserved domains. We performed mutation analysis of DCX in two Korean patients with SBH. The SBH patients had mild to moderate developmental delays, drug-resistant generalized seizures, and diffuse thick SBH upon brain MRI. Sequence analysis of the DCX coding region in Patient 1 revealed a c.386 C>T change in exon 3. The sequence variation results in a serine to leucine amino acid change at position 129 (S129L), which has not been found in other family members of Patient 1 or in a large panel of 120 control X-chromosomes. We report here a novel c.386 C>T mutation of DCX that is responsible for SBH. PMID:16100463

  4. Edward Said’s Worldliness, Amateurism and Heterotopia: Negotiating the Interdisciplinarity of Literary Theory, Canonicity, and Paradigm

    Directory of Open Access Journals (Sweden)

    Ayman Abu-Shomar

    2016-06-01

    Full Text Available Literary criticism nowadays is essentially crossing the boundaries of disciplinarity and canonicity where literary theory has increasingly been shaped by overlapping concepts and branching out of theories as well as whipping out the limitations imposed by theory itself. The post-conditions of contemporaneity have imposed a view of reading and analysing the literary text that is dynamic, proliferated and in flux as well as resistant to monolithic critique and confined disciplines and professionalization. This outlook has increasingly made the notions of literary criticism, theoretical paradigms and canons not only artificial and irrelevant to our materialistic world, but, in many cases, ‘violent’ to those whose life concerns exist in the margins of these paradigmatic notions. In this essay, I argue that those of us who aspire to an interdisciplinary and a metacritical analyses would be well served by importing inspirations from Edward Said’s work, scholarship and life, particularly drawing on his ‘Worldliness’, ‘Amateurism’ and ‘Heteroglossia’ (or heterotopia as well as drawing examples from his negotiation with intellectual paradoxes and tensions informed by his positionality as a border crosser intellectual (or his exilic consciousness. Specifically, this article engages with Said as an author of a radically secular body of work marked by as comportment towards being, and as an example of an “amateur” critic who “speak[s] truth to power”. It argues that Said instates a critically-interrogative scholarship as antidote to essentialist, politicised, determinist and hegemonic literary canons (whether those of texts or theory which are paradigmatically informed by relations of power in academia. The paper argues that through the investment of his scholarship and personal life, Said rejects academic institutions and affiliations with their tendency towards doctrinaire assumptions of critical work. Further to this, Said

  5. Ethylene polymerization by PN3-type pincer chromium(III) complexes

    KAUST Repository

    Gong, Dirong; Liu, Wen; Chen, Tao; Chen, Zhongren; Huang, Kuo-Wei

    2014-01-01

    Chromium (III) complexes, Cr1, [2,6-(tBu2PNH) 2C5H4N]CrCl3; Cr2, [2,6-(Ph 2PNH)2C5H4N]CrCl3; Cr3, [2-(tBu2PNH)C5H4N]CrCl3 THF; Cr4, [6-(tBu2PNH)C5H4N-2- CH2NEt2]CrCl3; Cr5, [6-(tBu 2PNH)C5H4N-2-C3H2N 2]CrCl3; Cr6, [6-(tBu2PNH)C 5H4N-2-(3,5-Me2)C3H 2

  6. Cervical nodular fasciitis in a 10-year-old girl: a case report of a rare ...

    African Journals Online (AJOL)

    Thus, as clinical and morphologic characteristics are similar to those of malignant tumours, this entity is often misdiagnosed, with a few cases described in the literature. We present a case of nodular fasciitis involving the retropharynx and hypopharynx of a 10-year-old girl and discuss the importance of considering this rare ...

  7. Progression from an Immature Teratoma with Miliary Gliomatosis Peritonei to Growing Teratoma Syndrome with Nodular Gliomatosis Peritonei

    Directory of Open Access Journals (Sweden)

    Yuh-Lin Hsieh

    2009-04-01

    Full Text Available A 4½-year-old girl presented with an incompletely resected, huge, immature abdominal teratoma, elevated serum alpha-fetoprotein (AFP, and numerous miliary gliomatosis peritonei (GP. Two courses of chemotherapy resulted in normalization of her AFP level and marked tumor shrinkage. Further chemotherapy was interrupted by complications. During treatment for these complications, ascites increased and the tumor enlarged, but serum AFP remained within the normal range. Second-look surgery revealed that the tumor had changed histologically to a mature teratoma, and GP had enlarged to nodular size, causing massive ascites. The still incompletely resected, growing mature teratoma was reduced with inter-feron. Nodular GP and ascites slowly regressed with interferon use, and finally disappeared after several months. One residual mass thought to be GP was reduced by gamma-knife surgery 3 years later.

  8. The pathophysiology of the nodular and micronodular small bowel fold

    International Nuclear Information System (INIS)

    Olmsted, W.W.; Ros, P.R.; Moser, R.P.; Shekita, K.M.; Lichtenstein, J.E.

    1986-01-01

    The normal small bowel fold is easily seen on conventional studies of the small intestine, but visualization of the small bowel villus is at the limit of resolution of current roentgenographic technique. When the villi are enlarged, they appear radiographically as an irregularity or micronodularity of the small bowel fold. The anatomy of the fold and the pathophysiology of diseases producing fold nodularity (tumor,inflammatory disease, NLH, mastocytosis) and micronodularity (lymphangiectasia, Waldenstrom macroglobulinemia, Whipple disease) are presented, with an emphasis on radiologic-pathologic correlation. The radiologist should suggest certain diseases or conditions based on the roentgenographic characteristics of the closely analyzed small bowel fold

  9. Mobile heterotopia: movement, circulation and the function of the university

    Directory of Open Access Journals (Sweden)

    Bradley Rink

    Full Text Available This paper explores the function of the university through the lens of mobility as seen from a South African perspective. Understanding the role of the university as one that requires the movement and circulation of academic bodies in the form of students and staff, and bodies of academic knowledge in the form of teaching, research and academic content, I use a theoretical framework from the interdisciplinary field of mobilities in order to understand the role of movement in the university and to highlight what is ruptured and catalysed by frictions enacted through power geometry, austerity and disruption. Sighted from the perspective of the University of the Western Cape in South Africa, this paper poses a series of provocations that reveal the obligations of presence that comprise the production and transfer of knowledge in the twenty-first-century university. I discuss how disruption and austerity, amongst other embedded mobility limitations, impact on the multiple/intersecting universes of the university; how the austere and disrupted university influences our engagement at various scales from local to global; and, finally, how disruption and austerity act to fix academic bodies in place even as they may allow virtual mobility to replace the face-to-face engagement that is the hallmark of the academic project. This paper demonstrates the critical role of mobility in the institution of the university and concludes that the university is a form of Foucauldian heterotopia mobilising diverse academic bodies and bodies of knowledge.

  10. Malignant Transformation of Nodular Hidradenoma in the Lower Leg

    Directory of Open Access Journals (Sweden)

    Nhuan Ngo

    2018-05-01

    Full Text Available Nodular hidradenoma (NH is a benign adnexal tumor that arises from either eccrine or apocrine sweat glands. NH can originate from any cutaneous site, but the most common sites are the head and anterior surface of the trunk, with very rare cases in the extremities. Long-standing NH has been reported to undergo malignant transformation to malignant NH (MNH; however, its occurrence in the lower leg is extremely rare with only one other case reported to date. In this report, we present a rare case of MNH occurring in the lower leg which was resected with the intent to make a diagnosis. At the final follow-up after 11 months, no local recurrence or metastasis has been observed.

  11. Zircaloy nodular corrosion analysis by an image processing technique

    International Nuclear Information System (INIS)

    Kawashima, Junko; Sato, Kanemitsu; Kuwae, Ryosho; Higashinakagawa, Emiko

    1987-01-01

    An image processor has been fabricated to examine out-of-pile nodular corrosion for Zircaloy-2 tubings. The covering fraction, which is the percentage of the nodule occupying area on the Zircaloy surface, was measured with the processor. The covering fraction showed a strong correlation with the weight gain at any corrosion time of this experiment. The correlation observed can be explained by a model for the lenticular shape of the nodules. The image processor also gives unfolded pictures of the whole Zircaloy surface. By analyzing the picture, the location of the nodules generated was found to be determined in an early stage of corrosion. New nodules were not produced later, and the nodules only grew larger with time. (orig.)

  12. Standard test method for determining nodularity and nodule count in ductile iron using image analysis

    CERN Document Server

    American Society for Testing and Materials. Philadelphia

    2011-01-01

    1.1 This test method is used to determine the percent nodularity and the nodule count per unit area (that is, number of nodules per mm2) using a light microscopical image of graphite in nodular cast iron. Images generated by other devices, such as a scanning electron microscope, are not specifically addressed, but can be utilized if the system is calibrated in both x and y directions. 1.2 Measurement of secondary or temper carbon in other types of cast iron, for example, malleable cast iron or in graphitic tool steels, is not specifically included in this standard because of the different graphite shapes and sizes inherent to such grades 1.3 This standard deals only with the recommended test method and nothing in it should be construed as defining or establishing limits of acceptability or fitness for purpose of the material tested. 1.4 The values stated in SI units are to be regarded as standard. No other units of measurement are included in this standard. 1.5 This standard does not purport to address al...

  13. Clinical, pathological, and molecular data on desmoplastic/nodular medulloblastoma: case studies and a review of the literature.

    Science.gov (United States)

    Siegfried, Aurore; Bertozzi, Anne Isabelle; Bourdeaut, Franck; Sevely, Annick; Loukh, Najat; Grison, Camille; Miquel, Catherine; Lafon, Delphine; Sevenet, Nicolas; Pietsch, Torsten; Dufour, Christelle; Delisle, Marie-Bernadette

    2016-01-01

    The aim of this study was to better define the clinical and biopathological features of patients with desmoplastic/nodular medulloblastoma (DNMB) and to further characterize this subgroup. 17 children aged 3 years, and residual tumor may also have been an explanation for recurrence.

  14. Part 1: MRI features of focal nodular hyperplasia with an emphasis on hepatobiliary contrast agents

    International Nuclear Information System (INIS)

    Sutherland, Tom; Seale, Melanie; Yap, Yap

    2014-01-01

    Focal nodular hyperplasia (FNH) is the second most common benign liver tumour and typically do not require any treatment. An accurate non-invasive diagnosis is therefore vital to avoid unnecessary intervention and to reassure patients. This article discusses the demographics and pathology of FNH and reviews the appearance of FNH at MRI using liver-specific contrast agents.

  15. Differential Diagnosis of Nodular Goiter by Aspiration Cytology

    International Nuclear Information System (INIS)

    Koh, Suk Man; Lee, Houn Young; Han, Bong Heon; Kim, Sam Young; Ro, Heung Kyu

    1982-01-01

    113 patients with nodular goiter were studied cytologically by needle aspiration for differential diagnosis at the department of internal medicine, Chungnam National University Hospital since October 1980 till July 1981, and the final diagnosis taken from biopsies were compared with the cytologic method on the 44 cases who received operation. The results were obtained as follows: 1. Among the 113 cases of total patients, male were 15 cases (13.3%) and female were 98 cases (86.7%) and the sex ratio (M : F) was 1 : 6.5. The peak age incidence was in the third decade followed by forth and second decades. 2. The findings of cytological diagnosis in 113 cases showed benign adenoma in 69 cases (61.1%), subacute and chronic thyroiditis in 22 cases (19.5%), papillary carcinoma in 15 cases (13.3%) and follicular carcinoma in 7 cases (6.2%), respectively, and 48 cases (69.6%) of the adenomas and 2 cases (9.1%) of papillary carcinomas showed combined cystic degeneration of the nodules. 3. The diameter of the nodules by palpation revealed within 2-5 cm in 88 cases (77.9%) out of 113 cases, below 2 cm in 17 cases and over 5 cm in 8 cases and there were no significant relationship between the size of the nodule and disease entity. 4. The findings of thyroid scintigram using 131 I in 113 cases of nodular goiter showed 'cold nodule' in 111 cases (98.2%) and normal scan (radioactivity) in 2 cases (1.8%) which showed adenoma in cytology and there was no cases with h ot nodule'. 5. The thyroid functions of the 113 cases revealed as euthyroidism in 108 cases (95.6%), hypothyroidism in 2 cases (2.7%) in adenomas but there was no evidence that the nodules of the above 3 cases were the reason of hyperthyroidism. 6. In 44 operated cases, the histological diagnosis revealed 23 cases of adenoma out of 27 cases (85.2%) who were diagnosed as adenoma by cytology and 15 cases of malignancy out of 17 cases (88.2%), and the overall diagnostic accuracy of aspiration cytology was 86.4%.

  16. Multiparameter FLAER-based flow cytometry for screening of paroxysmal nocturnal hemoglobinuria enhances detection rates in patients with aplastic anemia.

    Science.gov (United States)

    Sachdeva, Man Updesh Singh; Varma, Neelam; Chandra, Dinesh; Bose, Parveen; Malhotra, Pankaj; Varma, Subhash

    2015-05-01

    Flow cytometry is the gold standard methodology for screening of paroxysmal nocturnal hemoglobinuria. In the last few years, proaerolysin conjugated with fluorescein (FLAER) has become an important component of antibody panel used for the detection of paroxysmal nocturnal hemoglobinuria (PNH) clone. This study aimed to compare PNH clone detection by flow cytometry in the pre-FLAER era versus the FLAER era. This was a retrospective analysis of 4 years and included 1004 individuals screened for PNH clone, either presenting as hemolytic anemia or as aplastic anemia. In the pre-FLAER time period, the RBCs and neutrophils were screened with antibodies against CD55 and CD59. With the introduction of FLAER, neutrophils were screened with FLAER/CD24/CD15 and monocytes with FLAER/CD14/CD33 combination. A comparative analysis was done for detection of PNH clone in aplastic anemia patients versus non-aplastic anemia patients, as well as between pre-FLAER and FLAER era. Out of a total of 1004 individuals, 59 (5.8%) were detected to have PNH clone positivity. The frequency of PNH clone detected in aplastic anemia and non-aplastic anemia groups was 12.02 and 3.36%, respectively. The detection rate of PNH clone increased from 4.5% (32/711) in the pre-FLAER era to 9.2% (27/293) with the introduction of FLAER. However, this increase could be attributed to increased detection of PNH clone in the aplastic anemia group, which showed a significant increase from 8.3 to 18.2% after use of FLAER. In the non-aplastic group, PNH clone was detected with similar frequencies before and after use of FLAER (3.2 versus 3.8%, respectively). Mean PNH clone size was lower in the aplastic anemia group when compared with the non-aplastic group. RBCs always showed a lower clone size than neutrophils. PNH clone on neutrophils and monocytes was however similar. Inclusion of FLAER increases the sensitivity of the test which is especially useful in picking up small PNH clones in patients of aplastic anemia.

  17. Esclerite nodular e poliangiite granulomatosa (Wegener mimetizando tuberculose

    Directory of Open Access Journals (Sweden)

    Cybelle Moreno Luize Franco

    2015-04-01

    Full Text Available Poliangiite granulomatosa é uma doença sistêmica que afeta qualquer órgão, com predileção pelo trato respiratório superior, pulmões e rins. Este artigo tem como objetivo relatar um caso atípico de uma paciente com esclerite nodular como manifestação inicial da poliangiite granulomatosa (Wegener, mimetizando um quadro de tuberculose. A paciente apresentou hiperemia ocular e baixa acuidade visual progressiva por 1,5 anos, seguido por dor ocular por dois meses. A paciente possuía nódulos subpleurais com densidade de partes moles, linfonodomegalia em janela aorto-pulmonar e espessamento pleural bilateral discreto, negativo para bacilos álcool-ácido resistentes (BAAR. O diagnóstico histológico revelou uma vasculite granulomatosa sugestiva de vasculite não infecciosa (poliangiite granulomatosa. Foi iniciada pulsoterapia com ciclofosfamida.

  18. Liver heterotopia in the head of a patient with osteosarcoma of the maxilla. A paracrine tumor-induced hepatogenesis?

    Science.gov (United States)

    Corsi, Alessandro; Riminucci, Mara

    2017-02-01

    Heterotopia of liver tissue is uncommon. It has been reported at various sites, more frequently near the orthotopic liver, including gallbladder, hepatic ligaments, omentum, and retroperitoneum, rarely within the diaphragm and the thoracic cavity, and never within the head. We report here a 22-year-old patient surgically treated for a maxillary osteosarcoma in which microscopic liver tissue islands were incidentally detected in the respiratory mucosa of the surgical margin. The islands comprised well-differentiated HepPar-1-positive hepatocytes and were surrounded by cytokeratin-7- and cytokeratin-19-positive bile duct-like structures. This case, which is unique in the medical literature, may suggest an inductive paracrine effect of the osteosarcoma cells by secretion of factors promoting hepatocyte specification of primitive endodermal progenitors and subsequent liver morphogenesis. Copyright © 2016 Elsevier Inc. All rights reserved.

  19. MRI-findings of nodular lesions in an enlarged spleen, associated with visceral Leishmaniasis

    Energy Technology Data Exchange (ETDEWEB)

    Raeymaeckers, Steven, E-mail: Steven.Raeymaeckers@vub.ac.be [ZNA Middelheim, Lindendreef 1, 2020 Antwerpen (Belgium); Docx, Martine; Demeyere, Nathan [ZNA Middelheim, Lindendreef 1, 2020 Antwerpen (Belgium)

    2012-10-15

    Highlights: ► We confirm a previous report that infection with Leishmania can manifest multiple nodular lesions of the spleen. ► We confirm these lesions to be readily detectable with present imaging modalities. ► We affirm the fact that these lesions are hypoechoic on ultrasound, though in our case no hypoechoic halo was observed. ► We found these lesions to be hypodense on CT in the delayed phase after intravenous contrast administration. ► In addition to these previous findings we found that the spleen showed an inhomogeneous intensity on MRI; upon closer examination whilst scrolling through the T2-weighted sequences we can note multiple ill-defined and heterogeneous hypointense nodules. -- Abstract: We present a case of a 15-month-old Moroccan girl with fever of unknown origin, hepatosplenomegaly and multiple hypoechoic nodular splenic lesions that appear hypodense on CT. T2-weighted MRI sequences show a markedly inhomogeneous intensity of the parenchyma, seemingly caused by multiple ill-defined and heterogeneous hypointense nodules. Laboratory tests confirmed a recent infection with Leishmania, a parasite endemic to (sub)tropic regions. During and after therapy these lesions gradually resolved. To our knowledge this is the second published case in which different imaging modalities were able to demonstrate organ lesions associated with Leishmania. It is also the first report of MRI-findings associated with these lesions.

  20. Mammotome biopsy under ultrasound control in the diagnostics and treatment of nodular breast lesions - own experience.

    Science.gov (United States)

    Kibil, Wojciech; Hodorowicz-Zaniewska, Diana; Kulig, Jan

    2012-05-01

    Mammotome biopsy is an effective, minimally invasive, novel technique used in the verification of breast lesions.The aim of the study was to assess the value of ultrasound-guided vacuum-assisted core needle biopsy (mammotome biopsy) in the diagnostics and treatment of nodular breast lesions, considering own data.Material and methods. Analysis comprised 1183 mammotome biopsies under ultrasound control performed in 1177 female patients during the period between 2000 and 2010, at the Regional Clinic for Early Diagnostics and Treatment of Breast Lesions, I Chair and Department of General Surgery, Jagiellonian University, Collegium Medicum.Results. The average patient age amounted to 41.7 years. The size of the investigated lesions ranged between 4 and 65 mm (mean - 12 mm). The histopathological examination result was as follows: fibrocystic lesions (n=285), adenofibroma (n=477), adenosis sclerosans (n=188), hyperplasia without atypy (n=58), phyllode tumor (n=2), papilloma (n=14), hamartoma (n=1), atypical hyperplasia (n=25), in situ ductal carcinoma (n=4), in situ lobular carcinoma (n=5), infiltrating ductal carcinoma (n=114), infiltrating lobular carcinoma (n=4), non-diagnostic result (n=6). The histopathological diagnosis was obtained in 99.5% of cases. Patients diagnosed with atypical hyperplasia or cancer were qualified for surgery, according to accepted standards. The presence of a hematoma was the most common complication after the biopsy, observed in 16.5% of patients.Conclusions. The obtained results confirmed the high value of ultrasound-guided biopsies in the diagnostics of nodular breast lesions. The method is safe, minimally invasive, with few complications, providing a good cosmetic effect. In case of benign lesions with a diameter of less than 15 mm the mammotome biopsy enables to completely excise the lesions, being an alternative to open surgical biopsies. The mammotome biopsy should become the method of choice considering the diagnostics of nodular

  1. Laser-damage susceptibility of nodular defects in dielectric mirror coatings: AFM measurements and electric-field modeling

    International Nuclear Information System (INIS)

    Kozlowski, M.R.; DeFord, J.F.; Staggs, M.C.

    1993-01-01

    Atomic force microscopy (AFM) and electromagnetic field modeling were used to study the influence of nodular coating defects on laser-induced damage of multilayer dielectric coatings. In studies of HfO 2 /SiO 2 mirrors with 1.06 μm illumination, AFM results showed that nodular defects with high dome heights (>0.6 μm) were most susceptible to laser damage. Crater defects, formed by nodules ejected from the coating prior to illumination, were not damaged when illuminated over the same range of fluences. A finite-difference time-domain electromagnetic modeling code was used to study the influence of 3-D nodule defects on the E-field distribution within the interference coating. The modeling results show that Enfield enhancements as large as a factor of 4 can be present at the defects. Crater defects, however, result in minimal enhancement of the E-fields within the coating. These modeling results are consistent with the AFM experimental data, indicating that E-field enhancement is a contributing mechanism in defect-dominated laser damage of optical coatings

  2. No hypothyroidism after I-131 therapy in pts with toxic nodular goiter, treated under combined thyreostatic, thyreomimetic medication

    International Nuclear Information System (INIS)

    Giubbini, R.; Panarotto, M.B.; Paghera, B.; Pagliaini, R.; Pajoro, U.; Pizzocaro, C.; Rossini, P.L.; Terzi, A.; Maira, G.

    2002-01-01

    Background. Treatment of toxic nodular goiter with 131-I is generally satisfactory and will cause a reversion of hyperthyroidism. To avoid the risk of thyrotoxic storm I-131 therapy is usually performed after pre-treatment with antithyroid drugs, which causes a TSH increase and functional recruitment of previously inhibited normal thyroid tissue. In this functional state both autonomous nodule(s) and normal tissue are irradiated after I-131 administration. This may be the reason of late hypothyroidism occurring in 15-25% of Pts. Aim of the study was the evaluation of a different pre-treatment modality with combined methimazole (10-20 mg) and Triiodo-thyronine (T3 - 60 μg) in order to treat pts in euthyroid state with suppressed TSH. Study protocol. After diagnosis of hyperthyroidism with autonomous functioning tissue the pts were put under thyreostatic medication. Two months later TSH was checked and if >0.5% U.I the T3 treatment was associated. After two more months, the TSH level was checked again and, if suppressed, the pt received I-131 therapy. Study population. 93 pts (74f, 19m - age 75±10) were consecutively enrolled. 24 pts had a toxic nodular goiter and 69 a multi nodular one, respectively; they were evaluated at diagnosis, at pre-treatment, two months after therapy and at late follow-up (3.1 ± 3.5 yrs). Methods: 557±225 MBq of I-131 (according to uptake determinations and gland weight) were administered. Methymazole was discontinued 3 days before treatment whereas T3 was maintained during I-131 therapy. Results: Euthyroidism was achieved after the first treatment in 71% of pts. At late follow-up TSH values over the normal range were observed in only 4 pts (4.3% - however all 4 pts had TSH level below 6 I.U.). Summaries of FT3 and FT4 values are presented. Conclusions: The treatment of toxic nodular goiter under combined thyreostatic-thyreomimetic treatment is a safe, well tolerated and effective procedure allowing a 71% success at the first treatment

  3. CELSR2, encoding a planar cell polarity protein, is a putative gene in Joubert syndrome with cortical heterotopia, microophthalmia, and growth hormone deficiency.

    Science.gov (United States)

    Vilboux, Thierry; Malicdan, May Christine V; Roney, Joseph C; Cullinane, Andrew R; Stephen, Joshi; Yildirimli, Deniz; Bryant, Joy; Fischer, Roxanne; Vemulapalli, Meghana; Mullikin, James C; Steinbach, Peter J; Gahl, William A; Gunay-Aygun, Meral

    2017-03-01

    Joubert syndrome is a ciliopathy characterized by a specific constellation of central nervous system malformations that result in the pathognomonic "molar tooth sign" on imaging. More than 27 genes are associated with Joubert syndrome, but some patients do not have mutations in any of these genes. Celsr1, Celsr2, and Celsr3 are the mammalian orthologues of the drosophila planar cell polarity protein, flamingo; they play important roles in neural development, including axon guidance, neuronal migration, and cilium polarity. Here, we report bi-allelic mutations in CELSR2 in a Joubert patient with cortical heterotopia, microophthalmia, and growth hormone deficiency. © 2017 Wiley Periodicals, Inc. © 2017 Wiley Periodicals, Inc.

  4. “Bugabug ang dagat” (Rough seas: Experiencing Foucault’s heterotopia in fish trading houses

    Directory of Open Access Journals (Sweden)

    Nelson N. Turgo

    2012-06-01

    Full Text Available Places in the contemporary world are subjected to the workings of differentiating logics, foremost of which is globalization and to the other end, the counter-logic of localization, which results in, amongst others, the instantiation of differing spaces. These spaces, oftentimes co-existing and overlapping, are a result of contrapuntal forces, enacting their own colonization of places by people of varying interests. This article explores the other uses of kumisyunan (fish trading houses by magririgaton (fish vendors from a small fishing community in Quezon province that “simultaneously represent, contest, and invert” the very purpose and nature of the places’ rationale: fish trading. Heterotopia will be deployed in this article to further the ends of how a particular place could be inhabited by a number of spaces or exhibit alternate spatial possibilities and display a plethora of spatial practices within one singular location at different times in a particular spatial and temporal context. The article hopes to contribute to the further understanding of how everyday life and place is lived and reproduced in the variegated geographies of globalization in a developing economy like the Philippines.

  5. Intratumoral steatosis in focal nodular hyperplasia coinciding with diffuse hepatic steatosis : CT and MRI findings with histologic correlation

    NARCIS (Netherlands)

    Mortele, KJ; Stubbe, J; Praet, M; Van Langenhove, P; De Bock, G; Kunnen, M

    2000-01-01

    Focal nodular hyperplasia (FNH) is a benign tumorlike condition that is thought to be a hyperplastic response to increased blood flow in an arterial malformation rather than a true neoplasm. Radiologically, FNH usually shows typical findings on unenhanced and enhanced computed tomography (CT) and

  6. Clinical features, diagnosis, treatment and molecular studies in paediatric Cushing's syndrome due to primary nodular adrenocortical hyperplasia

    DEFF Research Database (Denmark)

    Storr, Helen L; Mitchell, J H; Swords, F M

    2004-01-01

    0.2-0.8 years) from diagnosis. Hypercortisolaemia was treated preoperatively by metyrapone alone 0.50-0.75 g/day (n = 4), metyrapone 0.75-1.50 g/day + o'p'DDD/mitotane 1-2 g/day (n = 1), or ketoconazole (n = 1). Adrenal histology showed nodular cortical hyperplasia with shrinkage of intervening...

  7. Solitary nodular bronchioloalveolar carcinoma of the lung: prediction of histology at high-resolution CT

    International Nuclear Information System (INIS)

    Jang, Hyun Jung; Lee, Kyung Soo; Choo, In Wook; Kim, Seung Hoon; Lee, Won Jae; Byun, Hong Sik; Kim, Yoo Kyung; Shin, Myung Hee; Kim, Sang Jin

    1998-01-01

    The purpose of this study is to descdribe the characteristic high-resolution (HR) CT findings of solitary nodular bronchioloalveolar carcinoma (BAC) of the lung which are valuable for specific diagnosis of the disease. HRCT scans of 46 patients (31 with malignant and 15 with benign lesion) with a solitary pulmonary nodule seen on chest radiograph were distributed in random order and analyzed retrospectively. Two blinded observers jointly analyzed the marginal and internal characteristics of nodules as seen on HRCT, and decisions on the findings were reached by consensus. Stepwise discriminant analysis for characteristic findings of BAC was performed. The most frequent CT findings of BAC (n=3D15) were internal bubble lucency (14/15, 93%)(p=3D0.001), area of ground-glass opacity (12/15, 80%; average 58% of tumor volume)(p=3D0.0001), pleural tag(12/15, 80%; p=3D0.097), and lobulated and spiculated margin(8/15, 53%; p=3D0.459). Findings of ground-glass opacity (p=3D0.0001) and bubble lucency (p=3D0.0187) appeared to be discriminant in the diagnosis of BAC. Peripheral pulmonary nodules containing an area of ground-glass opacity associated with internal bubble-lucency are characteristic of BAC. Specific histologic diagnosis of solitary nodular BAC can be suggested by careful analysis of HRCT findings.=20

  8. Radiological Findings in a Case of Multiple Focal Nodular Hyperplasia Associated with Portal Vein Atresia and Portopulmonary Hypertension

    Energy Technology Data Exchange (ETDEWEB)

    Lee, In Joon; Jeong, Sook Hyang; Choi, Jin Woo; Park, Hee Sun; Lee, Kyoung Ho; Kim, Hae Ryoung [Seoul National University Bundang Hospital, Seoul (Korea, Republic of)

    2008-08-15

    We present here the radiological findings of a rare case of multiple focal nodular hyperplasia that was associated with portal vein atresia and portopulmonary hypertension in a young woman. This case illustrates and supports the pathophysiological hypotheses that were previously proposed for the coexistence of these three abnormalities

  9. Intravoxel incoherent motion diffusion-weighted magnetic resonance imaging of focal vertebral bone marrow lesions: initial experience of the differentiation of nodular hyperplastic hematopoietic bone marrow from malignant lesions

    Energy Technology Data Exchange (ETDEWEB)

    Park, Sunghoon; Kwack, Kyu-Sung; Kim, Jae Ho [Ajou University School of Medicine, Division of Musculoskeletal Radiology, Department of Radiology, Suwon, Gyeonggi-do (Korea, Republic of); Ajou University Medical Center, Musculoskeletal Imaging Laboratory, Suwon (Korea, Republic of); Chung, Nam-Su [Ajou University School of Medicine, Department of Orthopaedic Surgery, Suwon (Korea, Republic of); Hwang, Jinwoo [Philips Healthcare, Department of Clinical Science, Seoul (Korea, Republic of); Lee, Hyun Young [Ajou University Medical Center, Regional Clinical Trial Center, Suwon (Korea, Republic of); Yonsei University College of Medicine, Department of Biostatistics, Seoul (Korea, Republic of)

    2017-05-15

    To evaluate the ability of intravoxel incoherent motion (IVIM) diffusion-weighted magnetic resonance imaging (MRI) parameters to differentiate nodular hyperplastic hematopoietic bone marrow (HHBM) from malignant vertebral bone marrow lesions (VBMLs). A total of 33 patients with 58 VBMLs, including 9 nodular HHBM lesions, 39 bone metastases, and 10 myelomas, were retrospectively assessed. All diagnoses were confirmed either pathologically or via image assessment. IVIM diffusion-weighted MRI with 11 b values (from 0 to 800 s/mm{sup 2}) were obtained using a 3.0-T MR imager. The apparent diffusion coefficient (ADC), pure diffusion coefficient (D), perfusion fraction (f), and pseudodiffusion coefficient (D*) were calculated. ADC and IVIM parameters were compared using the Mann-Whitney U test. Receiver operating characteristic (ROC) curve analysis was performed to assess the diagnostic performances of ADC, D, f, and D* in terms of VBML characterization. The diagnostic performance of morphological MR sequences was also assessed for comparison. The ADC and D values of nodular HHBM were significantly lower than those of malignant VBML (both p values < 0.001), whereas the f value was significantly higher (p < 0.001). However, there were no significant differences in D* between the two groups (p = 0.688). On ROC analysis, the area under the curve (AUC) for D was 1.000, which was significantly larger than that for ADC (AUC = 0.902). Intravoxel incoherent motion diffusion-weighted MRI can be used to differentiate between nodular HHBM and malignant VBML. The D value was significantly lower for nodular HHBM, and afforded a better diagnostic performance than the ADC, f, and D* values in terms of such differentiation. (orig.)

  10. A comparison of microRNA expression profiles from splenic hemangiosarcoma, splenic nodular hyperplasia, and normal spleens of dogs.

    Science.gov (United States)

    Grimes, Janet A; Prasad, Nripesh; Levy, Shawn; Cattley, Russell; Lindley, Stephanie; Boothe, Harry W; Henderson, Ralph A; Smith, Bruce F

    2016-12-03

    Splenic masses are common in older dogs; yet diagnosis preceding splenectomy and histopathology remains elusive. MicroRNAs (miRNAs) are short, non-coding RNAs that play a role in post-transcriptional regulation, and differential expression of miRNAs between normal and tumor tissue has been used to diagnose neoplastic diseases. The objective of this study was to determine differential expression of miRNAs by use of RNA-sequencing in canine spleens that were histologically confirmed as hemangiosarcoma, nodular hyperplasia, or normal. Twenty-two miRNAs were found to be differentially expressed in hemangiosarcoma samples (4 between hemangiosarcoma and both nodular hyperplasia and normal spleen and 18 between hemangiosarcoma and normal spleen only). In particular, mir-26a, mir-126, mir-139, mir-140, mir-150, mir-203, mir-424, mir-503, mir-505, mir-542, mir-30e, mir-33b, mir-365, mir-758, mir-22, and mir-452 are of interest in the pathogenesis of hemangiosarcoma. Findings of this study confirm the hypothesis that miRNA expression profiles are different between canine splenic hemangiosarcoma, nodular hyperplasia, and normal spleens. A large portion of the differentially expressed miRNAs have roles in angiogenesis, with an additional group of miRNAs being dysregulated in vascular disease processes. Two other miRNAs have been implicated in cancer pathways such as PTEN and cell cycle checkpoints. The finding of multiple miRNAs with roles in angiogenesis and vascular disease is important, as hemangiosarcoma is a tumor of endothelial cells, which are driven by angiogenic stimuli. This study shows that miRNA dysregulation is a potential player in the pathogenesis of canine splenic hemangiosarcoma.

  11. Association of the M1V PRKAR1A mutation with primary pigmented nodular adrenocortical disease in two large families

    NARCIS (Netherlands)

    Pereira, Alberto M.; Hes, Frederik J.; Horvath, Anelia; Woortman, Sanne; Greene, Elizabeth; Bimpaki, Eirini; Alatsatianos, Anton; Boikos, Sosipatros; Smit, Johannes W.; Romijn, Johannes A.; Nesterova, Maria; Stratakis, Constantine A.

    2010-01-01

    Carney complex (CNC) is a familial multiple neoplasia syndrome frequently associated with primary pigmented nodular adrenocortical disease (PPNAD), a bilateral form of micronodular adrenal hyperplasia that leads to Cushing's syndrome (CS). Germline PRKAR1A mutations cause CNC and only rarely

  12. Periventricular Heterotopia: Shuttling of Proteins through Vesicles and Actin in Cortical Development and Disease

    Directory of Open Access Journals (Sweden)

    Volney L. Sheen

    2012-01-01

    Full Text Available During cortical development, proliferating neural progenitors exhibit polarized apical and basolateral membranes that are maintained by tightly controlled and membrane-specific vesicular trafficking pathways. Disruption of polarity through impaired delivery of proteins can alter cell fate decisions and consequent expansion of the progenitor pool, as well as impact the integrity of the neuroependymal lining. Loss of neuroependymal integrity disrupts radial glial scaffolding and alters initial neuronal migration from the ventricular zone. Vesicle trafficking is also required for maintenance of lipid and protein cycling within the leading and trailing edge of migratory neurons, as well as dendrites and synapses of mature neurons. Defects in this transport machinery disrupt neuronal identity, migration, and connectivity and give rise to a malformation of cortical development termed as periventricular heterotopia (PH. PH is characterized by a reduction in brain size, ectopic clusters of neurons localized along the lateral ventricle, and epilepsy and dyslexia. These anatomical anomalies correlate with developmental impairments in neural progenitor proliferation and specification, migration from loss of neuroependymal integrity and neuronal motility, and aberrant neuronal process extension. Genes causal for PH regulate vesicle-mediated endocytosis along an actin cytoskeletal network. This paper explores the role of these dynamic processes in cortical development and disease.

  13. Ultrasonic inspection of nodular cast iron

    International Nuclear Information System (INIS)

    Hersh, S.; Zhang, Yingda

    1990-01-01

    On the basis of experimental results collected from several nodular cast iron (NCI) specimens, Amdata, Inc., has developed a tentative procedure for performing ultrasonic testing (UT) preservice inspection of NCI casks and qualifying personnel and equipment. The authors anticipate that this procedure will be a component in a comprehensive program to certify that casks are free from critical flaws prior to their introduction into service, with testing being performed on a production line basis by UT inspection personnel. The tentative procedure was applied to inspection of NCI block SGR-483-001 manufactured by Siempelkamp Giesserei GmbH and Co. of West Germany. This block is 59 by 39.5 by 13.8 inches and weighs 5.2 tons. Several indications were detected with the I/98, in accordance with the tentative procedure, and they were analyzed using two-dimensional synthetic aperture technique (Line-SAFT). When compared with conventional sizing methods that may confound the effects of beam spread with flaw size, Line-SAFT significantly improved sizing accuracy. SAFT is an electronic simulation of a lens and has the property of reducing the effect of beam spread on the resultant indication sizes. Although a higher-precision 3-D SAFT option was also available, it would necessitate data transfer to a separate VAX computer and lengthy calculations. As an alternative, Line-SAFT, a faster but less precise 2-D simplification, was implemented on the I/98 data acquisition system

  14. Microstructure and erosion characteristic of nodular cast iron surface modified by tungsten inert gas

    International Nuclear Information System (INIS)

    Abboud, Jaafar Hadi

    2012-01-01

    Highlights: ► Local surface melting. ► Significant improvement in erosion resistance. ► The ductile behaviour was found. -- Abstract: The surface of nodular cast iron has been melted and rapidly solidified by Tungsten Inert Gas (TIG) process to produce a chilled structure of high hardness and better erosion resistance. Welding currents of magnitude 100, 150, and 200 A at a constant voltage of 72 have been used to melt the surface of nodular cast iron. Microstructural characterization, hardness measurements, and erosion wear tests have been performed on these modified surfaces as well as on the untreated material. Microstructural characterization has shown that surface melting resulted in complete or partial dissolution of the graphite nodules and resolidification of primary austenite dendrites, which undergo further decomposition into ferrite and cementite, and interdendritic of acicular eutectic; their microhardness measured across the melted depth ranged between 600 and 800 Hv. The scale of the dendrites and the interdendritic eutectic became coarser when a higher current is used. The results also indicated that remelting process by TIG improved erosion resistance by three to four times. Eroded surface observations of the as-received and TIG melted samples showed a ductile behavior with a maximum erosion rate at 30°. The fine microstructures obtained by the rapid cooling and the formation of a large amount of eutectic cementite instead of the graphite have contributed greatly to the plastic flow and consequently to the better erosion resistance of the TIG surface melted samples.

  15. Imiquimod 5% cream in the treatment of superficial and nodular basal cell carcinomas: study of 10 cases Tratamento tópico do carcinoma basocelular superficial e nodular pelo imiquimod creme a 5%: observação de 10 casos

    Directory of Open Access Journals (Sweden)

    Cyro Festa Neto

    2002-12-01

    Full Text Available BACKGROUND: Topical treatment with 5% imiquimod cream has been demonstrated to be effective in patients with basal cell carcinoma. OBJECTIVES: In the present study, efficacy and tolerability of this treatment was analyzed in 10 patients with 13 different types of superficial and nodular basal cell carcinomas. METHODS: Imiquimod cream was applied daily for a mean period of 23 days. RESULTS AND CONCLUSIONS: All patients responded favorably to the drug with healing of the lesions. No recurrence was observed during two to three months of follow up.FUNDAMENTOS: O uso tópico do imiquimod 5% em creme para o tratamento de carcinomas basocelulares tem-se mostrado eficaz. OBJETIVOS: Com base nesse fato o autor analisa a efetividade e tolerabilidade desse método em 10 doentes com 13 carcinomas basocelulares dos tipos superficial e nodular. MÉTODOS: As aplicações foram diárias, e o número médio de dias de tratamento foi 23. RESULTADOS E CONCLUSÕES: Todos os doentes responderam à medicação com desaparecimento das lesões e são seguidos a cada dois ou três meses, até o momento sem recidiva do quadro.

  16. Nodular Regenerative Hyperplasia and Portal Hypertension in a Patient with Coeliac Disease

    Directory of Open Access Journals (Sweden)

    Erwin Biecker

    2011-01-01

    Full Text Available Nodular regenerative hyperplasia (NRH of the liver is often associated with rheumatologic or lymphoproliferative disorders and a cause of portal hypertension in some patients. We report the case of a 71-year-old patient with celiac disease and unexplained portal hypertension. Biopsy of the liver revealed NRH as the underlying cause. The patient did not suffer from an autoimmune, rheumatologic or lymphoproliferative disease. A thrombophilic disorder that might cause NRH was ruled out. Celiac disease is often associated with mild elevation of liver enzymes and steatosis of the liver, but the association with NRH was described in only a few patients. We discuss the possible relationship of celiac disease and NRH.

  17. Early-stage focal nodular hyperplasia: US/CT/MR features correlated with histology

    International Nuclear Information System (INIS)

    Golfieri, R.; Giampalma, E.; Berardi, R.; Caputo, M.; Lalli, A.; Grazi, G.; Mazziotti, A.; Gozzetti, G.; D'Errico, A.; Grigioni, W.; Gavelli, G.

    1994-01-01

    Two cases of focal nodular hyperplasia (FNH), in which ultrasound (US), computed tomography (CT), and magnetic resonance imaging (MR) studies detected an atypical hemorrhagic pattern associated with an intrahepatic arterio venous malformation (AVM) around the growths, are presented. In both cases, histology demonstrated a very early regenerative stage and necrotic-hemorrhagic areas within the lesions. In these cases, the analysis of radiological findings, surgical specimens and histology seemed to confirm the pathogenetic hypothesis suggested by Wanless: in normal liver parenchyma, a ''blood steal'' phenomenon due to congenital or acquired intrahepatic AVM could cause ischemic damage, appearing as a hemorrhagic necrotic area, the extent of which depends on the degree of residual portal supply. (orig.)

  18. Graves' disease and toxic nodular goiter are both associated with increased mortality but differ with respect to the cause of death

    DEFF Research Database (Denmark)

    Brandt, Frans; Thvilum, Marianne; Pedersen, Dorthe Almind

    2013-01-01

    Background: Hyperthyroidism has been associated with increased all-cause mortality. Whether the underlying cause of hyperthyroidism influences this association is unclear. Our objectives were to explore whether mortality risk and cause of death differ between Graves' disease (GD) and toxic nodular...

  19. The Outlet Patch: Gastric Heterotopia of the Colo-rectum and Anus.

    Science.gov (United States)

    Mannan, Abul Ala Syed Rifat; Vieth, Michael; Khararjian, Armen; Khandakar, Binny; Lam-Himlin, Dora; Heydt, David; Bhaijee, Feriyl; Venbrux, Henry J; Byrnes, Kathleen; Voltaggio, Lysandra; Barker, Norman; Yuan, Songyang; Montgomery, Elizabeth

    2018-04-18

    Gastric heterotopia (GH) has been described throughout the gastrointestinal tract. However, the colorectal region is an extremely rare location for it. We describe the clinicopathologic features of GH of the colon, rectum, and anus. We identified 33 cases in 20 males and 13 females (median age 54 years; range, 4 months to 73 years). Sites included the rectum (N=26), anus (N=4), ileocecal junction (N=1), ascending colon (N=1) and descending colon (N=1). Presenting symptoms(N=27) included hematochezia (41%) and altered bowel habits (4%); 15 patients (55%) were asymptomatic. On colonoscopy (N=31), all appeared as solitary lesions, (median size 6.5 mm, range 2 mm - 55 mm), either as polyps (61%), raised erythematous patches (23%), an ulcer(10%), within a rectal diverticulum (3%), or a hemorrhoid (3%). Patients were managed by polypectomy. One with an associated carcinoma in the area of GH underwent resection. No morbidity related to GH itself was reported following excision. Histologically, heterotopic gastric mucosa was oxyntic type (85%), mixed oxyntic and non-oxyntic type (12%), and not specified (3%). In 5 patients a pyloric gland adenoma (PGA) arose from heterotopic gastric mucosa, two of which contained a focus of invasive adenocarcinoma. One case had associated surface foveolar type low-grade dysplasia. Another had associated adenocarcinoma arising from the heterotopic mucosa. One example harbored Helicobacter pylori organisms. We highlight the features of GH in the distal GIT - the 'outlet patch'. Association with PGA, surface dysplasia and adenocarcinoma suggests that lower tract GH can undergo neoplastic transformation. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  20. Unusual MRI findings in grey matter heteropia

    International Nuclear Information System (INIS)

    Soto Ares, G.; Hamon-Kerautret, M.; Leclerc, X.; Pruvo, J.P.; Houlette, C.; Godefroy, O.

    1998-01-01

    We report unusual MRI patterns in patients with grey matter heterotopia. Standard T1- and T2-weighted spin-echo and inversion-recovery sequences were used in 22 patients presenting with seizures or developmental delay. The images were reviewed for signal change surrounding white matter and for atypical size, morphology or topography. We found 10 cases of subependymal heterotopias 11 of focal subcortical heterotopia and of diffuse subcortical heterotopia. On clinical or MRI grounds, 8 cases were considered unusual: 2 of the subependymal type, 2 of focal subcortical heterotopia with white matter abnormalities, 2 of focal subcortical heterotopia with no clinicoradiological correlation 1 of extensive hemispheric subcortical heterotopia and 1 of diffuse subcortical heterotopia confined to the frontal lobe. The classical classification of heterotopia enables easy radiological diagnosis even in cases with unusual patterns. In some cases, heterogeneity and high signal in surrounding white matter can be found. Cortical dysplasia is the most frequent associated malformation. (orig.)

  1. Tracheobronchial puncture-site nodular reaction (TPNR following endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA: Systematic review of case reports

    Directory of Open Access Journals (Sweden)

    Karan Madan

    2017-01-01

    Full Text Available Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA is a minimally invasive and efficacious diagnostic modality for lung cancer staging and evaluation of undiagnosed mediastinal lymphadenopathy. Procedure-related complications are uncommon. We herein report an infrequently described phenomenon following EBUS-TBNA in which two patients developed nodular granulation tissue at the tracheobronchial puncture site. On systematic review, we found description of such phenomena by terminologies such as endobronchial inflammatory polyp, granuloma, and endobronchial mass. The endobronchial inflammatory polyp has been one of the most commonly used terminologies for these; but in most cases, the classical features of an inflammatory polyp are lacking. We propose the term, tracheobronchial puncture-site nodular reaction (TPNR with further classification into granulomatous and nongranulomatous subtypes, for standardized reporting of such reactions following transbronchial needle aspiration procedures. Knowledge of this entity and standardized nomenclature shall help in better characterization of the outcomes and risk factors for the occurrence of these reactions.

  2. Paroxysmal nocturnal haemoglobinuria at Oslo University Hospital 2000-2010.

    Science.gov (United States)

    Nissen-Meyer, Lise Sofie H; Tjønnfjord, Geir E; Golebiowska, Elzbieta; Kjeldsen-Kragh, Jens; Akkök, Çiğdem Akalın

    2015-06-16

    Paroxysmal nocturnal haemoglobinuria (PNH) is a rare haematological disease characterised by chronic haemolysis, pancytopenia and venous thrombosis. The condition is attributable to a lack of control of complement attack on erythrocytes, thrombocytes and leukocytes, and can be diagnosed by means of flow cytometry. In this quality assurance study, we have reviewed information from the medical records of all patients tested for PNH using flow cytometry at our laboratory over a ten-year period. In the period 2000-2010 a total of 28 patients were tested for PNH using flow cytometry at the Department of Immunology and Transfusion Medicine, Oslo University Hospital. We have reviewed the results of these examinations retrospectively together with information from medical records and transfusion data for the patients concerned. Flow cytometry identified 22 patients with PNH: four with classic disease and 18 with PNH secondary to another bone marrow disease. Five patients had atypical thrombosis. Seventeen patients received antithymocyte globulin or drug treatment; of these, six recovered from their bone marrow disease, while six died and five had a need for long-term transfusion. Five patients with life-threatening bone marrow disease underwent allogeneic stem cell transplantation, three of whom died. Six of 22 patients received eculizumab; the need for transfusion has been reduced or eliminated in three patients treated with eculizumab over a longer period. Flow cytometry identified PNH in a majority of patients from whom we obtained samples. Most patients had a PNH clone secondary to bone marrow failure. Atypical thrombosis should be borne in mind as an indication for the test. Treatment with eculizumab is relevant for selected patients with PNH.

  3. Graves Disease Induced by Radioiodine Therapy for Toxic Nodular Goiter: A Case Report

    Directory of Open Access Journals (Sweden)

    Yakup Yürekli

    2015-10-01

    Full Text Available Graves’ disease (GD may be observed as an infrequent adverse effect after radioiodine therapy (RAIT for toxic thyroid adenoma (TA and toxic multi nodular goiter (MNG. We present a case of a 55-year-old male with a toxic nodule who was treated with RAI. After therapy, the patient’s serum free triiodothyronine (fT3 and free thyroxine (fT4 levels gradually increased. Antithyroid peroxidase (TPOAb, antithyroglobulin (TgAb and TSH-receptor antibodies (TRAb were also positive. Thyroid scintigraphy revealed diffuse intense uptake after four months of RAIT. Radiation-induced GD should be considered in patients with aggravated hyperthyroidism 3-4 months after therapy.

  4. Ultrasonic features and radionuclide correlation in liver cell adenoma and focal nodular hyperlasia.

    Science.gov (United States)

    Sandler, M A; Petrocelli, R D; Marks, D S; Lopez, R

    1980-05-01

    Ultrasonic features of three cases of liver cell adenoma (LCA) and two cases of focal nodular hyperplasia (FNH) are presented. These tumors have similar sonographic appearances presenting either as solid masses or containing sonolucent areas due to hemorrhage or necrosis. Although these ultrasonic features in patients with an area of decreased activity on 99mTc-sulfur colloid (Tc-SC) liver scans are not specific for LCA or FNH, such findings in the appropriate clinical setting are suggestive of these lesions. The combination of a solid mass on ultrasonography and a normal Tc-SC radioisotope liver study may be relatively specific for uncomplicated FNH.

  5. Ultrasonic features and radionuclide correlation in liver cell adenoma and focal nodular hyperplasia

    International Nuclear Information System (INIS)

    Sandler, M.A.; Petrocelli, R.D.; Marks, D.S.; Lopez, R.

    1980-01-01

    Ultrasonic features of three cases of liver cell adenoma (LCA) and two cases of focal nodular hyperplasia (FNH) are presented. These tumors have similar sonographic appearances presenting either as solid masses or containing sonolucent areas due to hemorrhage or necrosis. Although these ultrasonic features in patients wth an area of decreased activity on /sup 99m/Tc-sulfur colloid (Tc-SC) liver scans are not specific for LCA or FNH, such findings in the appropriate clinical setting are suggestive of these lesions. The combination of a solid mass on ultrasonography and a normal Tc-SC radioisotope liver study may be relatively specific for uncomplicated FNH

  6. Nodular fasciitis of the face diagnosed by US-guided core needle biopsy: a case report

    International Nuclear Information System (INIS)

    Lee, Sang Kwon; Kwon, Sun Young

    2006-01-01

    We report here on a case of nodular fasciitis (NF) that was diagnosed by ultrasonography (US)-guided core needle biopsy in a 31-year-old man, and we include the US and computed tomographic (CT) findings and the histopathologic findings at US-guided core needle biopsy (CNB). We suggest that high-resolution US is useful for the detailed evaluation of NF in the superficial regions, such as the face, and US-guided CNB is useful for the definitive histologic diagnosis of NF without causing scarring

  7. Hybrid Single Photon Emission Computed Tomography/Computed Tomography Sulphur Colloid Scintigraphy in Focal Nodular Hyperplasia

    International Nuclear Information System (INIS)

    Bhoil, Amit; Gayana, Shankramurthy; Sood, Ashwani; Bhattacharya, Anish; Mittal, Bhagwant Rai

    2013-01-01

    It is important to differentiate focal nodular hyperplasia (FNH), a benign condition of liver most commonly affecting women, from other neoplasm such as hepatic adenoma and metastasis. The functional reticuloendothelial features of FNH can be demonstrated by scintigraphy. We present a case of breast cancer in whom fluorodeoxyglucose positron emission tomography/computerized tomography (CT) showed a homogenous hyperdense lesion in liver, which on Tc99m sulfur colloid single-photon emission computed tomography/CT was found to have increased focal tracer uptake suggestive of FNH

  8. Malignant nodular hidradenoma on the scalp: report of a case with fine needle aspiration cytology features and histologic correlation.

    Science.gov (United States)

    Garcia-Bonafe, María Magdalena; Campins, Maria M Company; Redecilla, Pere Huguet

    2009-01-01

    Malignant nodular hidradenoma (MNH) is a malignant adnexal tumor of the eccrine sweat glands. The histology is similar to that of benign nodular hidradenoma, but MNH shows an infiltrative and invasive pattern, necrosis and angiolymphatic invasion. A 60-year-old woman, diagnosed with rectal adenocarcinoma 6 months before, underwent fine needle aspiration (FNA) of a nodule on the scalp. The aspirate smears showed (1) necrotic debris; (2) cohesive cell clusters and tissue fragments; (3) tubular formations; (4) globoid or cylindrical clear cells; (5) cells showing dense cytoplasm and a basaloid appearance; (6) cell sheets with anaplasia and squamous differentiation; and (7) multinucleated giant cells. The histology showed a lobulated pattern with necrosis and cyst formation. The clear cells that were seen on cytology occupied the periphery of the lobules, whereas the anaplastic cells were located in the central portion. Squamous differentiation and tubular formations were observed. Rapid, accurate diagnosis of these tumors is important, particularly when they develop at sites where a metastatic process must be ruled out. Cytology of FNA material provides this capability.

  9. Identification by real-time PCR with SYBR Green of Leishmania spp. and Serratia marcescens in canine 'sterile' cutaneous nodular lesions.

    Science.gov (United States)

    Cornegliani, Luisa; Corona, Antonio; Vercelli, Antonella; Roccabianca, Paola

    2015-06-01

    Noninfectious, non-neoplastic, nodular to diffuse, so-called 'sterile' granulomatous/pyogranulomatous skin lesions (SGPSLs) are infrequently identified in dogs and may represent a diagnostic challenge. Their correct identification is based on history, histopathology and absence of intralesional foreign bodies and micro-organisms. The aim of this study was to investigate the presence of Leishmania spp., Mycobacterium spp., Serratia marcescens and Nocardia spp. by real-time PCR in canine nodular skin lesions histologically diagnosed as putatively sterile. Formalin-fixed skin biopsies were collected from 40 dogs. All samples were associated with an SGPSL diagnosis characterized by multifocal, nodular to diffuse, periadnexal and perifollicular pyogranulomas/granulomas. Neither micro-organisms nor foreign bodies were detected with haematoxylin and eosin staining, under polarized light. Further analyses included periodic acid Schiff, Ziehl-Neelsen, Fite Faraco, Giemsa and Gram histochemical stains; anti-Bacillus Calmette-Guérin (BCG) and Leishmania spp. immunohistochemistry; and real-time PCR analysis for Leishmania spp., Mycobacterium spp., S. marcescens and Nocardia spp. Special stains and BCG/immunohistochemistry were negative in all samples. Real-time PCR was positive for Leishmania spp. in four of 40 biopsies and for S. marcescens in two of 40 samples. Real-time PCR for Mycobacterium spp. and Nocardia spp. was negative. No correlation between real-time PCR positivity and a specific histological pattern was identified. Leishmania spp. have been previously identified as possible agents of certain SGPSLs, while the involvement of S. marcescens has not been investigated previously. According to our findings, Serratia spp. should be included in the list of agents possibly associated with a subgroup of granulomatous/pyogranulomatous skin lesions in dogs. © 2015 ESVD and ACVD.

  10. Pure ground glass nodular adenocarcinomas: Are preoperative positron emission tomography/computed tomography and brain magnetic resonance imaging useful or necessary?

    Science.gov (United States)

    Cho, Hyoun; Lee, Ho Yun; Kim, Jhingook; Kim, Hong Kwan; Choi, Joon Young; Um, Sang-Won; Lee, Kyung Soo

    2015-09-01

    The utility of (18)F-Fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) scanning and brain magnetic resonance imaging (MRI) as a staging workup for lung adenocarcinoma manifesting as pure ground glass opacity (GGO) is unknown. The purpose of this study was to determine the utility of these 2 tests for preoperative staging of pure GGO nodular lung adenocarcinoma. The study included 164 patients (male:female, 73:91; mean age, 62 years) with pure GGO nodular lung adenocarcinoma who underwent PET/CT (in 136 patients) and/or brain MRI (in 109 patients) before surgery. Pathologic N staging and dedicated standard imaging or follow-up imaging findings for M staging were used as reference standards. The median follow-up time was 47.9 months. On PET/CT scan, abnormal FDG uptake of lymph nodes was found in 2 of 136 patients (1.5%); both were negative on final pathology. Abnormal FDG uptake of the liver was detected in 1 patient, which was also confirmed to be negative by dedicated abdominal CT. The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of PET/CT in detecting metastases were not applicable, 98% (95% confidence interval [CI], 94%-100%), 0% (95% CI, 0%-71%), 100% (95% CI, 97%-100%), and 98% (95% CI, 94%-100%), respectively. No brain metastasis was found in preoperative brain MRI of 109 patients. Of 109 patients, 1 (0.9%) developed brain metastasis 30 months after surgical resection. PET/CT and brain MRI is not necessary in the staging of pure GGO nodular lung adenocarcinoma. Copyright © 2015 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

  11. Diffuse glomerular nodular lesions in diabetic pigs carrying a dominant-negative mutant hepatocyte nuclear factor 1-alpha, an inheritant diabetic gene in humans.

    Directory of Open Access Journals (Sweden)

    Satoshi Hara

    Full Text Available Glomerular nodular lesions, known as Kimmelstiel-Wilson nodules, are a pathological hallmark of progressive human diabetic nephropathy. We have induced severe diabetes in pigs carrying a dominant-negative mutant hepatocyte nuclear factor 1-alpha (HNF1α P291fsinsC, a maturity-onset diabetes of the young type-3 (MODY3 gene in humans. In this model, glomerular pathology revealed that formation of diffuse glomerular nodules commenced as young as 1 month of age and increased in size and incidence until the age of 10 months, the end of the study period. Immunohistochemistry showed that the nodules consisted of various collagen types (I, III, IV, V and VI with advanced glycation end-product (AGE and Nε-carboxymethyl-lysine (CML deposition, similar to those in human diabetic nodules, except for collagen type I. Transforming growth factor-beta (TGF-β was also expressed exclusively in the nodules. The ultrastructure of the nodules comprised predominant interstitial-type collagen deposition arising from the mesangial matrices. Curiously, these nodules were found predominantly in the deep cortex. However, diabetic pigs failed to show any of the features characteristic of human diabetic nephropathy; e.g., proteinuria, glomerular basement membrane thickening, exudative lesions, mesangiolysis, tubular atrophy, interstitial fibrosis, and vascular hyalinosis. The pigs showed only Armanni-Ebstein lesions, a characteristic tubular manifestation in human diabetes. RT-PCR analysis showed that glomeruli in wild-type pigs did not express endogenous HNF1α and HNF1β, indicating that mutant HNF1α did not directly contribute to glomerular nodular formation in diabetic pigs. In conclusion, pigs harboring the dominant-negative mutant human MODY3 gene showed reproducible and distinct glomerular nodules, possibly due to AGE- and CML-based collagen accumulation. Although the pathology differed in several respects from that of human glomerular nodular lesions, the

  12. Sonographic pattern of focal nodular hyperplasia of the liver

    International Nuclear Information System (INIS)

    Majewski, A.; Gratz, K.F.; Broelsch, C.; Gebel, M.

    1984-01-01

    Focal nodular hyperplasia of the liver (FNH) is a histologically defined benign hepatic tumour, possibly associated with the use of oral contraceptives. The present study deals with the question whether FNH can be distinguished from other lesions of the liver by ultrasonography alone or by a combination of realtime sonography and radiologic procedures. Therefore 18 cases of FNH, 16 females and 2 males, are presented. We retrospectively reviewed the diagnostic results of real-time sonography, hepatic scintigraphy, computed tomography with dynamic CT-densitometry, and angiography on these 18 patients. Ultrasonography enables the identification of the lesion in all of the 18 cases. FNH appears to show varying degrees of echogenicity (slight hypoechoic in 6 cases, isoechoic in 6 cases, and slight hyperechoic in 4 cases). In most of the patients the tumour had similar acoustic characteristics at the surrounding normal liver. By ultrasonography alone there is no possible differentation of FNH from other hepatic tumours. Both the echo pattern of FNH and the results of the ultrasonically guided cytopuncture are unspecific. (orig.)

  13. Perturabation of nodular operation under salt and water deficit stress in rhizobium common bean symbiosis

    International Nuclear Information System (INIS)

    Harzalli Jebara, Salwa

    2006-01-01

    This work aims at the search for markers of tolerance to the osmotic stress and nodular efficiency of symbiosis Haricot Rhizobium. Thus, after having fixed the best period of hydroponic culture, we showed that a severe salt treatment generated an inhibition of the parameters of growth and nodulation. These inhibitions are accompanied by an inhibition of the enzymatic activities: ascorbate peroxidase (APX) and catalase (CAT), but an activation of peroxidase (POX) and superoxide dismutase (SOD), suggesting that these two antioxydants can be biochemical markers of the tolerance to salinity. To check the validity of these markers and to see the participation of the vegetable genotype in the response to the stress, we compared the effect of two concentrations salt 25 and 50 mM NaCe at two contrasting genotypes BAT477 tolerant and sensitive COCOT. This study illustrates the role of the vegetable genotype in the tolerance and efficiency and emphasize a significant result that SOD and POX constitute biochemical markers of tolerance to salinity. In order to ensure itself of the validity of this assumption in the event of water deficit stress, a treatment of 50 mM mannitol is applied to 16 symbioses formed by four genotypes of bean BAT477, COCOT, Flamingo and BRB17 inoculated by four strains of rhizobium CIAT899, 12 to 3, 1 to 6 and 8 to 3. This study permits us to make a screening of these symbioses according to their efficiency and their tolerance based on parameters of growth, of fixing and extent of the antioxydant enzymatic activities. It gets clear that the response of enzymatic antioxydants is in relation to the intrinsic potentialities of the partners of symbioses and appears to act as of the first stages of recognition plants bacterium. It will be retained that activities POX and SOD are markers of nodular tolerance. The CAT is the enzyme most connected to each partner of symbiosis and the APX would play a rather functional role. The heterogeneity of found answer

  14. First case report of locally advanced malignant nodular hidradenoma of the scrotum.

    Science.gov (United States)

    Shah, Binay K; Qamruzzaman, Yusuf; Serban, Karina; Hire, Ervin; Ying, Shan-Ching

    2010-01-01

    Malignant nodular hidradenoma (MNH) is a malignant tumor of the eccrine glands, and most commonly involves the head, trunk, and extremities. To the best of our knowledge, MNH of the scrotum has not yet been described in the English literature. Despite the use of surgery, chemotherapy, radiotherapy, and hormonal therapy, optimal treatment of MNH is unclear. We describe the case of a 30-year-old African American man who was diagnosed with locally advanced MNH of the scrotum and treated with surgery. More than 2 years after surgery, the patient is without evidence of disease. This is the first case report of MNH of the scrotum. Surgery alone may be sufficient for the treatment of localized or locally advanced MNH. Copyright © 2010 S. Karger AG, Basel.

  15. Ethylene polymerization by PN3-type pincer chromium(III) complexes

    KAUST Repository

    Gong, Dirong

    2014-12-01

    Chromium (III) complexes, Cr1, [2,6-(tBu2PNH) 2C5H4N]CrCl3; Cr2, [2,6-(Ph 2PNH)2C5H4N]CrCl3; Cr3, [2-(tBu2PNH)C5H4N]CrCl3 THF; Cr4, [6-(tBu2PNH)C5H4N-2- CH2NEt2]CrCl3; Cr5, [6-(tBu 2PNH)C5H4N-2-C3H2N 2]CrCl3; Cr6, [6-(tBu2PNH)C 5H4N-2-(3,5-Me2)C3H 2N2]CrCl3; Cr7, [6-(tBu 2PNH)C5H4N-2-(3,5-iPr 2)C3H2N2]CrCl3; Cr8, [6-(tBu2PNH)C5H4N-2-(3,5-Ph 2)C3H2N2]CrCl3, bearing a family of neutral PN3-type pincer ligands have been prepared. The molecular structure of Cr2 was further elucidated by the X-ray crystallographic analysis, showing an octahedral geometry. Treatment of these complexes with MAO or alkylaluminum led to catalysts with moderate activities (about 105 g (PE)/Cr(mol) h) for ethylene polymerization, affording exclusively linear low molecular weight solid PE without any detectable oligomers. Among Cr1-Cr8, the highest activity was achieved for Cr1/MAO at room temperature with production of PE with highest molecular weight, indicating that replacement of both tBu groups in Cr1 with Ph groups, or one PtBu2 with the N (imine) arm, resulted in a lower catalytic activity and lower M w. © 2014 Elsevier B.V.

  16. Ccdc85C, a causative protein for hydrocephalus and subcortical heterotopia, is expressed in the systemic epithelia with proliferative activity in rats.

    Science.gov (United States)

    Tanaka, Natsuki; Izawa, Takeshi; Takenaka, Shigeo; Yamate, Jyoji; Kuwamura, Mitsuru

    2015-07-01

    Coiled-coil domain containing 85c (Ccdc85c) is a causative gene for spontaneous mutant mouse with non-obstructive hydrocephalus and subcortical heterotopia. Detailed functions of Ccdc85C protein have not been clarified. To reveal roles of Ccdc85C, we examined the distribution and expression pattern of Ccdc85C in the systemic developing organs in rats. Ccdc85C was expressed in various simple epithelia but not stratified epithelia. In the various epithelia, Ccdc85C was localized at cell-cell junctions and its expression was strong at apical junctions. Furthermore, intense expression was seen at developing period and gradually decreased with advancing development. Distribution of Ccdc85C coincides with that of proliferating epithelial cells. These results suggest that Ccdc85C plays an important role in the proliferative property of simple epithelia.

  17. Radioimmunoassay of iodine metabolism state in euthyroid and nodular goiter under conditions of moderately increased intake of fluorine with drinking water

    International Nuclear Information System (INIS)

    Sidora, V.D.; Mazurik, M.F.; Shlyakhta, A.I.; Kuznetsov, A.A.

    1987-01-01

    A reliable decrease of triiodotyronine in blood cerum in the region with moderately increased intake of fluorine with drinking water (1.2-3.1 mg/l) is detected. This results in increase of euthyroid goiter among population by 14-16 times, of nodular - by 6 times. In the studied area there is indirect dependence between function of thyroid gland and degree of its hyperplasia, i.e., the larger is the gland, the weaker is its ability to produce hormones. The practical significance of detected dependence lies in the fact, that in surgical removal of the nodes (malignant cases are excluded) not only morphological but functional criteria as well of the gland increase should be taken account of. Such approach to nodular goiter therapy promotes sufficient reduction of postoperative hypothyrosis. These peculiarities of the ratio between the thyroid gland hyperplasia degree and its function under conditions of studied area must be taken account of when selecting methods of conservative treatment of patients with euthyroid goiter

  18. Late follow up results after J - 131 therapy of toxic multi-nodular goiter

    International Nuclear Information System (INIS)

    Petrovski, Z.

    2015-01-01

    Full text of publication follows. Objective: the aim of this study was to analyze success of radioiodine therapy in patients with toxic multi-nodular goiter (TMG). Methods: The group of 43 patients (36 females / 7 males, aged 47 ± 11 yrs, range 27 - 75 yrs) with TMG were treated with radioiodine. 28 patients were treated with one dose, 12 patients with two doses and 7 patients with three and more doses according to Marinelli's formula. The administered activity of J -131 was established basing on radioiodine uptake and goiter size ( median 555 MBq, range: 370 - 1100 MBq). Patients were evaluated by clinical and thyroid examination of TSH, FT4, FT3 after 1 - 3 months. Thyroid scintigraphy was performed 3 months after radioiodine therapy. Prior to treatment with J -131 all patients were treated with antithyroid medications, who were suspended 4 - 7 days and restarted one week after J - 131 therapy. Results: in 76,8% (33/43) patients there was control of disease after the first J -131 dose and in 95,2% (40/43) patients after the second and more doses. At 20 years of follow up, there were 84,4% (36/43) patients euthyroid, 13,9% (6/43) patients hypothyroid and 4,6% (2/43) patients hyperthyroid. Reduction of gland weight were in 74,4% (32/43) patients. During 20 years of follow up no adverse side effects were observed after J - 131 therapy. Conclusion: radioiodine therapy is the right choice of treatment for toxic multi-nodular goiter and single dose of J -131 is successful in most of the cases. A single higher radioiodine dose diminishes the need for additional J -131 therapy, without increasing of developing hypothyroidism. (authors)

  19. Escleritis nodular posterior gigante compatible con sarcoidosis ocular simulando un melanoma de la coroides

    OpenAIRE

    Pérez-Campagne, E.; Guex-Crosier, Y.; Schalenbourg, A.; Uffer, S.; Zografos, L.

    2007-01-01

    Caso clínico: Un paciente de 30 años fue enviado a nuestro servicio oncológico de referencia con el diagnóstico de melanoma amelanótico de la coroides del ojo izquierdo. Se hicieron los siguientes exámenes: oftalmoscopía, angiografía fluoresceínica, angiografía con verde de indocianina, ecografía, resonancia magnética y biopsia. Discusión: El diagnóstico de escleritis nodular posterior gigante basado en la ecografía se confirmó mediante una biopsia. Se realizó un examen médico completo buscán...

  20. Organelle and cellular abnormalities associated with hippocampal heterotopia in neonatal doublecortin knockout mice.

    Directory of Open Access Journals (Sweden)

    Reham Khalaf-Nazzal

    Full Text Available Heterotopic or aberrantly positioned cortical neurons are associated with epilepsy and intellectual disability. Various mouse models exist with forms of heterotopia, but the composition and state of cells developing in heterotopic bands has been little studied. Dcx knockout (KO mice show hippocampal CA3 pyramidal cell lamination abnormalities, appearing from the age of E17.5, and mice suffer from spontaneous epilepsy. The Dcx KO CA3 region is organized in two distinct pyramidal cell layers, resembling a heterotopic situation, and exhibits hyperexcitability. Here, we characterized the abnormally organized cells in postnatal mouse brains. Electron microscopy confirmed that the Dcx KO CA3 layers at postnatal day (P 0 are distinct and separated by an intermediate layer devoid of neuronal somata. We found that organization and cytoplasm content of pyramidal neurons in each layer were altered compared to wild type (WT cells. Less regular nuclei and differences in mitochondria and Golgi apparatuses were identified. Each Dcx KO CA3 layer at P0 contained pyramidal neurons but also other closely apposed cells, displaying different morphologies. Quantitative PCR and immunodetections revealed increased numbers of oligodendrocyte precursor cells (OPCs and interneurons in close proximity to Dcx KO pyramidal cells. Immunohistochemistry experiments also showed that caspase-3 dependent cell death was increased in the CA1 and CA3 regions of Dcx KO hippocampi at P2. Thus, unsuspected ultrastructural abnormalities and cellular heterogeneity may lead to abnormal neuronal function and survival in this model, which together may contribute to the development of hyperexcitability.

  1. A Novel PRKAR1A Mutation Identified in a Patient with Isolated Primary Pigmented Nodular Adrenocortical Disease

    Directory of Open Access Journals (Sweden)

    Sira Korpaisarn

    2017-08-01

    Full Text Available Primary pigmented nodular adrenocortical disease (PPNAD is a rare cause of Cushing syndrome, especially the isolated form without Carney complex, associated with germline mutations in PRKAR1A, the protein kinase A regulatory subunit type 1 alpha gene. We report a 31-year-old female who presented with secondary amenorrhea, cushingoid appearance, and hypertension without Carney complex. Biochemical laboratory examinations confirmed the ACTH-independent adrenal Cushing syndrome with negative Liddle test. A small right adrenal adenoma of 0.8 cm was shown on computed tomography while magnetic resonance imaging revealed nodularity of both adrenal glands. The histological report confirmed PPNAD using laparoscopic right adrenalectomy, and subsequent left adrenalectomy was performed 6 months later. She had inherited heterozygosity of a novel germline mutation of the PRKAR1A gene (g.114213T>G or c.709-5T>G. This splice site mutation results in exon 8 skipping. Her father carrying the same mutation had no clinical features of either PPNAD or Carney complex. This novel PRKAR1A gene mutation, c.709-5T>G, is reported here for the first time manifesting as an incomplete clinical expression of the isolated form of PPNAD and being inherited with low penetrance unlike other inherited mutations of the Carney complex which have a penetrance of almost 100%.

  2. [The variation and clinical significance of paroxysmal nocturnal hemoglobinuria clone in patients with aplastic anemia before and after immunosuppressive therapy].

    Science.gov (United States)

    Sun, Ying-xin; Zhu, Ming-qing; He, Guang-sheng; Wang, Xiu-li; Fang, Bao-zhi; Lu, Cong; Liu, Zhen-zhen; Wu, Qian; Yang, Yong; Wu, De-pei; Sun, Ai-ning

    2013-07-01

    To evaluate the evolution of paroxysmal nocturnal hemoglobinuria (PNH) clone and its clinical significance before and after immunosuppressive therapy (IST) in patients with aplastic anemia (AA). A total of 186 patients diagnosed as AA were enrolled in this study. Among them, 55 patients were diagnosed as severe AA (SAA) and treated with cyclosporine (CsA) plus anti-thymocyte globulin (ATG), 131 were diagnosed as non SAA (NSAA) and treated with CsA alone. All patients were screened for PNH clone by flow cytometry before treatment and followed up for 18-76 months, with a median time of 22 months. Positive PNH clones were detected in 10 SAA (18.9%) patients, significantly more than that of NSAA group [9 patients (7.4%), t = 5.041, P = 0.025]. The proportions of PNH clones in SAA group at 6, 12, 24 and > 24 months were 13.38%, 14.88%, 20.00% and 18.85%, respectively, also significantly higher than those of NSAA patients (5.67%, 5.31%, 5.47% and 9.08%, all P values clone was positive or negative. PNH clone are detectable in AA patients either treated with ATG plus CsA or CsA alone, and more significant by ATG plus CsA. Whether PNH clone occurred before or after IST does not affect the therapeutic efficacy.

  3. The SINS trial: A randomised controlled trial of excisional surgery versus imiquimod 5% cream for nodular and superficial basal cell carcinoma

    Directory of Open Access Journals (Sweden)

    Armstrong Sarah J

    2010-04-01

    Full Text Available Abstract Background Basal cell carcinoma is the commonest human cancer. Despite increasing incidence it remains poorly researched. While not life threatening it can cause significant cosmetic disfigurement. Imiquimod, a cream which enhances the body's immune response, may help deal with the number of cases that occur in low-risk sites, especially when good cosmetic results and home use without surgery are needed. This study aims 1. To compare excisional surgery with imiquimod cream for nodular or superficial basal cell carcinoma in low risk sites, with respect to 3 year clinical clearance, cost-effectiveness and cosmetic results. 2. To ascertain if certain phenotypic features and gene polymorphisms predict tumour responsiveness to treatment. Methods/Design Five hundred participants with low risk nodular or superficial basal cell carcinoma will be recruited from hospitals to this multi-centre, randomised, parallel group, controlled phase III trial. Treatment in the imiquimod group is for 6 weeks for superficial basal cell carcinoma and 12 weeks for nodular basal cell carcinoma. Both treatment groups are followed up in clinic for 3 years. Primary outcome variable: the proportion of participants with clinical evidence of success (no recurrence at 3 years. The primary outcome will be compared between the two treatment groups. Secondary outcomes include: i clinical success at 1, 2 and 5 years, ii time to first recurrence, iii cosmetic appearance of lesion site after treatment, iv level of pain, and v cost-effectiveness. Safety and tolerability data will also be reported. Discussion This study protocol describes a pragmatic randomised controlled trial which it is hoped will address the above uncertainties. Three-year results will be available towards the end of 2010. Trial registration Meta-register: NCT00066872, Eudract No. 2004-004506-24, ISRCTN48755084.

  4. Botulinum as a Toxin for Treating Post-herpetic Neuralgia

    OpenAIRE

    DING, Xu-Dong; ZHONG, Jing; LIU, Yan-Ping; CHEN, Hua-Xian

    2017-01-01

    Background: We aimed to study the curative effects of botulinum A toxin (BTX-A) on the treatment of post-herpetic neuralgia (PNH). Methods: We enrolled 58 PNH patients and treated them with hypodermic injection of BTX-A in Xiangyang No.1 People?s Hospital, Hubei University of Medicine, Hubei, China. We measured and compared the Visual Analog Score (VAS), Neuropathy Pain Scale (NPS), Quality of Life Scale (SF-36) score, PNH seizure severity degree, seizure duration, frequency of attack and the...

  5. Nodular immunocyte-derived (AL) amyloidosis in the trachea of a dog.

    Science.gov (United States)

    Besancon, M Faulkner; Stacy, Brian A; Kyles, Andrew E; Moore, Peter F; Vernau, William; Smarick, Sean D; Rasor, Liberty A

    2004-04-15

    A 7-year-old castrated male Miniature Schnauzer was examined because of labored breathing and episodes of respiratory distress that progressed to collapse. On cervical radiographs, a focal soft tissue mass in the caudal cervical portion of the trachea was observed, and during tracheoscopy, a 1 x 1 cm, pedunculated, multinodular, pink, intraluminal mass extending from the dorsal tracheal membrane and obstructing approximately 80% of the tracheal lumen was seen. Tracheal resection and anastomosis was performed to remove the mass, and the dog recovered without complications. On histologic examination, the mass consisted of a large accumulation of homogeneous, faintly fibrillar eosinophilic material admixed with a predominantly plasma cell infiltrate; examination of sections stained with thioflavin T and Congo red stain confirmed that the eosinophilic material was amyloid. A diagnosis of nodular, immunocyte-derived (AL) amyloidosis was made. Seventeen months after surgery, the dog had a relapse of respiratory distress because of an extramedullary plasmacytoma involving the trachea.

  6. Elastosonography in the diagnosis of the nodular breast lesions: preliminary report

    International Nuclear Information System (INIS)

    Giuseppetti, Gian Marco; Di Cioccio, Barbara; Baldassarre, Silvia; Martegani, Alberto

    2005-01-01

    Purpose. To assess the diagnostic accuracy of elastography in characterising nodular breast lesions. Materials and methods. A total of 82 patients who received mammographic, ultrasonographic and elastographic evaluation in a single session at two Italian centres (Dept of Radiology, Az. Ospedali Riuniti, Ancona, and Ospedale Valduce, Corno, Italy) between January and August 2004 according to identical protocols exhibited 91 nodules that were subjected to cytological/histological examination. Lesions were classified and scored and the sensitivity of elastography calculated. Results. Overall sensitivity and specificity were 79% and 89%, respectively. However, sensitivity was 86% and 65% and specificity 100% and 62% for lesions 2 cm in diameter, respectively. Despite the small sample and the fact that it does not include the full range of cell types, tumour cell composition seemed to affect accuracy. Conclusions. Elastography is easy and rapid to perform. Larger studies are needed to establish semiological patterns; despite the small patient sample, these data provide an interesting contribution [it

  7. An Electron Microscopy Study of Graphite Growth in Nodular Cast Irons

    Science.gov (United States)

    Laffont, L.; Jday, R.; Lacaze, J.

    2018-04-01

    Growth of graphite during solidification and high-temperature solid-state transformation has been investigated in samples cut out from a thin-wall casting which solidified partly in the stable (iron-graphite) and partly in the metastable (iron-cementite) systems. Transmission electron microscopy has been used to characterize graphite nodules in as-cast state and in samples having been fully graphitized at various temperatures in the austenite field. Nodules in the as-cast material show a twofold structure characterized by an inner zone where graphite is disoriented and an outer zone where it is well crystallized. In heat-treated samples, graphite nodules consist of well-crystallized sectors radiating from the nucleus. These observations suggest that the disoriented zone appears because of mechanical deformation when the liquid contracts during its solidification in the metastable system. During heat-treatment, the graphite in this zone recrystallizes. In turn, it can be concluded that nodular graphite growth mechanism is the same during solidification and solid-state transformation.

  8. Development of an experimental model of brain tissue heterotopia in the lung

    Science.gov (United States)

    Quemelo, Paulo Roberto Veiga; Sbragia, Lourenço; Peres, Luiz Cesar

    2007-01-01

    Summary The presence of heterotopic brain tissue in the lung is a rare abnormality. The cases reported thus far are usually associated with neural tube defects (NTD). As there are no reports of experimental models of NTD that present this abnormality, the objective of the present study was to develop a surgical method of brain tissue heterotopia in the lung. We used 24 pregnant Swiss mice divided into two groups of 12 animals each, denoted 17GD and 18GD according to the gestational day (GD) when caesarean section was performed to collect the fetuses. Surgery was performed on the 15th GD, one fetus was removed by hysterectomy and its brain tissue was cut into small fragments and implanted in the lung of its litter mates. Thirty-four live fetuses were obtained from the 17GD group. Of these, eight (23.5%) were used as control (C), eight (23.5%) were sham operated (S) and 18 (52.9%) were used for pulmonary brain tissue implantation (PBI). Thirty live fetuses were obtained from the females of the 18GD group. Of these, eight (26.6%) were C, eight (26.6%) S and 14 (46.6%) were used for PBI. Histological examination of the fetal trunks showed implantation of GFAP-positive brain tissue in 85% of the fetuses of the 17GD group and in 100% of those of the 18GD group, with no significant difference between groups for any of the parameters analysed. The experimental model proved to be efficient and of relatively simple execution, showing complete integration of the brain tissue with pulmonary and pleural tissue and thus representing a model that will permit the study of different aspects of cell implantation and interaction. PMID:17877535

  9. Correlation between Focal Nodular Low Signal Changes in Hoffa’s Fat Pad Adjacent to Anterior Femoral Cartilage and Focal Cartilage Defect Underlying This Region and Its Possible Implication

    Directory of Open Access Journals (Sweden)

    Chermaine Deepa Antony

    2016-01-01

    Full Text Available Purpose. This study investigates the association between focal nodular mass with low signal in Hoffa’s fat pad adjacent to anterior femoral cartilage of the knee (FNMHF and focal cartilage abnormality in this region. Method. The magnetic resonance fast imaging employing steady-state acquisition sequence (MR FIESTA sagittal and axial images of the B1 and C1 region (described later of 148 patients were independently evaluated by two reviewers and categorized into four categories: normal, FNMHF with underlying focal cartilage abnormality, FNMHF with normal cartilage, and cartilage abnormality with no FNMHF. Results. There was a significant association (p=0.00 between FNMHF and immediate adjacent focal cartilage abnormality with high interobserver agreement. The absence of focal nodular lesions next to the anterior femoral cartilage has a very high negative predictive value for chondral injury (97.8%. Synovial biopsy of focal nodular lesion done during arthroscopy revealed some fibrocollagenous tissue and no inflammatory cells. Conclusion. We postulate that the FNMHF adjacent to the cartilage defects is a form of normal healing response to the cartilage damage. One patient with FHMHF and underlying cartilage abnormality was rescanned six months later. In this patient, the FNMHF disappeared and normal cartilage was observed in the adjacent region which may support this theory.

  10. Cytokine gene expression profile distinguishes CD4+/CD57+T cells of the nodular lymphocyte predominance type of Hodgkin's lymphoma from their tonsillar counterparts

    NARCIS (Netherlands)

    Atayar, Cigdem; Poppema, Sibrand; Visser, Lydia; van den Berg, Anke

    Little is known about the cytokine profile of nodular lymphocyte predominance Hodgkin's lymphoma (NLPHL) and the significance of the characteristic rosetting CD4(+)/CD57(+) T cells. We analysed the T lymphocyte populations isolated from lymph node suspensions from five patients with NLPHL, two with

  11. Variable uptake feature of focal nodular hyperplasia in Tc-99m phytate hepatic scintigraphy/single-photon emission computed tomography-A parametric analysis.

    Science.gov (United States)

    Hsu, Yu-Ling; Chen, Yu-Wen; Lin, Chia-Yang; Lai, Yun-Chang; Chen, Shinn-Cherng; Lin, Zu-Yau

    2015-12-01

    Tc-99m phytate hepatic scintigraphy remains the standard method for evaluating the functional features of Kupffer cells. In this study, we demonstrate the variable uptake feature of focal nodular hyperplasia (FNH) in Tc-99m phytate scintigraphy. We reviewed all patients who underwent Tc-99m phytate hepatic scintigraphy between 2008 and 2012 in Kaohsiung Medical University Hospital, Kaohsiung, Taiwan. Cases with FNH were diagnosed on the basis of pathology or at least one or more prior imaging with a periodic clinical follow-up. All patients received a standard protocol of dynamic flow study and planar and Tc-99m phytate single-photon emission computed tomography (E. CAM; Siemens). The correlation of variable nodular radioactivity with parameters such as tumor size and localization was analyzed. In total, 15 lesions of 14 patients in the clinic were diagnosed as FNH. The tumor size was approximately 2.9-7.4 cm (mean size 4.6 cm). Four lesions were larger than 5 cm. The major anatomic distribution was in the right hepatic lobe (10 lesions), particularly in the superior segments (7 lesions). Tc-99m phytate single-photon emission computed tomography imaging for determining the functional features of Kupffer cells included cool/cold (8 lesions), isoradioactive/warm (6 lesions), and hot (1 lesion) patterns of uptake. We did not observe any statistically significant correlation between variable nodular radioactivity and tumor size (p=0.68) or localization (p=0.04). Herein, we demonstrate the variable uptake feature of FNH in Tc-99m phytate scintigraphy. In small FNH tumors (< 5 cm), increased or equal uptake still provided specificity for the differential diagnosis of hepatic solid tumors. Copyright © 2015. Published by Elsevier Taiwan.

  12. Clinical roundtable monograph: Paroxysmal nocturnal hemoglobinuria: a case-based discussion.

    Science.gov (United States)

    Szer, Jeff; Hill, Anita; Weitz, Ilene Ceil

    2012-11-01

    Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired disorder characterized by chronic intravascular hemolysis as the primary clinical manifestation and morbidities that include anemia, thrombosis, renal impairment, pulmonary hypertension, and bone marrow failure. The prevalence of the PNH clone (from <1-100% PNH granulocytes) is approximately 16 per million, and careful monitoring is required. The average age of onset of the clinical disease is the early 30s, although it can present at all ages. PNH is caused by the acquisition of a somatic mutation of the gene phosphatidylinositol glycan anchor (PIG-A) in a multipotent hematopoietic stem cell (HSC), with clonal expansion of the mutated HSC. The mutation causes a deficiency in the synthesis of glycosylphosphatidylinositol (GPI). In cells derived from normal HSCs, the complement regulatory proteins CD55 and CD59 are anchored to the hematopoietic cell membrane surface via GPI, protecting the cells from complement-mediated lysis. However, in patients with PNH, these 2 proteins, along with numerous other GPI-linked proteins, are absent from the cell surface of red cells, granulocytes, monocytes, and platelets, resulting in complement-mediated intravascular hemolysis and other complications. Lysis of red blood cells is the most obvious manifestation, but as other cell lineages are also affected, this complement-mediated attack contributes to additional complications, such as thrombosis. Eculizumab, a humanized monoclonal antibody against the C5 complement protein, is the only effective drug therapy for PNH patients. The antibody prevents cleavage of the C5 protein by C5 convertase, in turn preventing generation of C5b-9 and release of C5a, thereby protecting from hemolysis of cells lacking the CD59 surface protein and other complications associated with complement activation. Drs. Ilene C. Weitz, Anita Hill, and Jeff Szer discuss 3 recent cases of patients with PNH.

  13. Outcomes of allogeneic stem cell transplantation in patients with paroxysmal nocturnal hemoglobinuria with or without aplastic anemia.

    Science.gov (United States)

    Lee, Sung-Eun; Park, Sung Soo; Jeon, Young-Woo; Yoon, Jae-Ho; Cho, Byung-Sik; Eom, Ki-Sung; Kim, Yoo-Jin; Lee, Seok; Min, Chang-Ki; Kim, Hee-Je; Cho, Seok-Goo; Kim, Dong-Wook; Min, Woo-Sung; Lee, Jong Wook

    2017-10-01

    The aim of this study was to evaluate the long-term outcomes of allogeneic stem cell transplantation (SCT) in patients with paroxysmal nocturnal hemoglobinuria (PNH) with or without aplastic anemia (AA). A total of 33 patients with PNH clones who underwent allogeneic SCT were analyzed. After a median follow-up of 57 months (range, 6.0-151.3), the 5-year estimated overall survival rate was 87.9±5.7%. Four patients died of transplant-related mortality (TRM). With the exception of one patient with early TRM, 32 patients were engrafted. Two patients who had developed delayed GF received a second transplant and recovered. The cumulative incidences of acute graft-vs-host disease (GVHD) (≥grade II) and chronic GVHD (≥moderate) were 27.3±7.9% and 18.7±7.0%, respectively. Twenty-one patients receiving SCT with reduced-intensity conditioning (RIC) had available follow-up data for PNH cell population for the first 6 months post-transplant. Analysis of these data revealed that the PNH clones disappeared within approximately 2 months. RIC regimen was sufficient to eradicate PNH clones with sustained donor-type engraftment after allogeneic SCT. Therefore, application of allogeneic SCT with RIC should be considered in patients with PNH, in accordance with the severity of the underlying bone marrow failure. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  14. [A case of focal epilepsy manifesting multiple psychiatric auras].

    Science.gov (United States)

    Ezura, Michinori; Kakisaka, Yosuke; Jin, Kazutaka; Kato, Kazuhiro; Iwasaki, Masaki; Fujikawa, Mayu; Aoki, Masashi; Nakasato, Nobukazu

    2015-01-01

    We present a case of epilepsy with multiple types of focal seizures that were misdiagnosed as psychiatric disorders. A 20-year-old female patient presented with a variety of episodes, including loss of consciousness, deja vu, fear, delusion of possession, violent movements, and generalized convulsions. Each of these symptoms appeared in a stereotypic manner. She was initially diagnosed with a psychiatric disorder and treated with psychoactive medications, which had no effect. Long-term video electroencephalography revealed that her episodes of violent movement with impaired consciousness and secondarily generalized seizure were epileptic events originating in the right hemisphere. High-field brain magnetic resonance imaging for detecting subtle lesions revealed bilateral lesions from periventricular nodular heterotopia. Her final diagnosis was right hemispheric focal epilepsy. Carbamazepine administration was started, which successfully controlled all seizures. The present case demonstrates the pitfall of diagnosing focal epilepsy when it presents with multiple types of psychiatric aura. Epilepsy should thus be included in differential diagnoses, considering the stereotypic nature of symptoms, to avoid misdiagnosis.

  15. Glioneuronal Heterotopia Presenting As a Cerebellopontine angle Tumor of the cranial Nerve VIII, Case Report.

    Science.gov (United States)

    Peris-Celda, M; Giannini, C; Diehn, F E; Eckel, L J; Neff, B A; Van Gompel, J J

    2018-04-03

    Vestibular schwannomas and meningiomas account for the great majority of lesions arising in the cerebellopontine angle (CPA). In this report, we present a case of glioneuronal heterotopia, also known as glioneuronal hamartoma, arising from the VIII cranial nerve, which is an extremely uncommon lesion. Important radiologic and surgical aspects are reviewed, which may help in early recognition and intraoperative decision making when these lesions are encountered. A healthy 29-year-old female presented with intermittent right facial numbness. Magnetic resonance imaging (MRI) showed an incidental minimally enhancing cerebellopontine angle lesion on the right VII-VIII cranial nerve complex. The patient declined serial observation and opted for operative intervention for resection. Intraoperatively, the lesion resembled neural tissue and was continuous with the VIII cranial nerve. Pathological analysis demonstrated mature glioneuronal tissue consistent with hamartomatous brain tissue. The patient maintained normal hearing and facial nerve function after surgery. Radiologic, surgical and pathological characteristics are described. Ectopic glioneuronal tissue of the VIII cranial nerve is a rare non-neoplastic lesion, and should be considered in the differential diagnosis of unusual appearing intracanalicular and cerebellopontine angle lesions. The congenital and benign nature of this entity makes observation a valid option for these cases, although they are so infrequent that they are often presumptively managed as vestibular schwannomas. Attempts to radically resect these lesions may result in higher rates of hearing loss or facial palsy due to their continuity with the cranial nerves. Copyright © 2018 Elsevier Inc. All rights reserved.

  16. Effect of the complement inhibitor eculizumab on thromboembolism in patients with paroxysmal nocturnal hemoglobinuria.

    NARCIS (Netherlands)

    Hillmen, P.; Muus, P.; Duhrsen, U.; Risitano, A.M.; Schubert, J.; Luzzatto, L.; Schrezenmeier, H.; Szer, J.; Brodsky, R.A.; Hill, A.; Socie, G.; Bessler, M.; Rollins, S.A.; Bell, L.; Rother, R.P.; Young, N.S.

    2007-01-01

    Hemolysis and hemoglobinemia contribute to serious clinical sequelae in hemolytic disorders. In paroxysmal nocturnal hemoglobinuria (PNH) patients, hemolysis can contribute to thromboembolism (TE), the most feared complication in PNH, and the leading cause of disease-related deaths. We evaluated

  17. Magnetic resonance imaging of hepatic adenoma and focal nodular hyperplasia. Report of eight cases

    International Nuclear Information System (INIS)

    Menu, Y.; Arrive, L.; Grenier, Ph.

    1988-01-01

    Hepatic Adenoma (HA) and Focal Nodular Hyperplasia (FNH) are rare benign tumors of the liver, occurring mostly in females between 25 and 40 years old. HA is clearly related to oral contraceptive intake. Detection of such tumors may be assessed by Ultrasonography (US). Computed Tomography (CT) or Angiography. These examinations usually allow differenciation with hepatic angiomas but it is much more difficult to rule out a well-differenciated primary liver cancer. MR proved to be a valuable technique for detection of liver tumors. Initial experience suggested that T1 and T2 relaxation times had little value for tissue characterization. The aim of this study is to show the presentation of HA and FNH and to evaluate the possibility for this method to characterize these lesions [fr

  18. Nodular goiter after occupational accidental exposure to radiation

    International Nuclear Information System (INIS)

    Pisarev, Mario A.; Schnitman, M.A.; Buenos Aires Univ.; Hospital Frances, Buenos Aires

    2008-01-01

    Full text: Purpose: During a control of the safety measures at the Radiology Service in a local hospital in Buenos Aires, a lack of an appropriate shielding in the X-ray equipment was detected. Methods and Materials: Therefore studies were performed in the group of physicians and technicians exposed and in an age and sex matched group of non-exposed physicians and technicians. Check-ups included thyroid sonography, circulating thyroid hormones, TSH and thyroid antibodies. The exposed group included 9 females and 5 males, while the non-exposed group comprised 8 females and 5 males. Results: No thyroid abnormalities were found in the non-exposed patients. On the contrary in the exposed cohort in 11 out of 14 patients nodular goiter was diagnosed, while an additional patient presented diffuse goiter. In 5 out of the 12 patients with goiter, nodule growth or the appearance of new nodules was observed along the follow-up. Four patients presented changes in thyroid function: two had elevated basal TSH with increased titters of anti TPO antibodies in one, while another showed an abnormal TRH-TSH test. A fourth patient had low total T 4 values. Other abnormalities included: sperm abnormalities in 2 out of the 5 males, dry-eye syndrome in other two, mammary nodules in one female and a basocellular epithelioma in another one. Conclusions: accidental irradiation leads to thyroid abnormalities, such as the appearance of nodules and alterations in thyroid function. (author)

  19. Producción de hierros nodulares ferríticos directamente de fundición partiendo de chatarra de acero de alto manganeso // Production of ferrítics nodular irons directly from foundry leaving of high manganese scrap steel

    Directory of Open Access Journals (Sweden)

    C. DeFranch

    1999-07-01

    Full Text Available La producción de hierros nodulares con matriz ferrítica es lograda normalmente con el uso de arrabios especiales con bajoscontenidos de elementos formadores de carburos, o mediante largos y costosos tratamientos térmicos. Estos arrabios tienenaltos precios, por lo que se hace necesario la búsqueda de variantes más económicas para la producción de este tipo dematerial. El objetivo de este trabajo es la obtención de dicho material usando como carga chatarra de acero, la cual tiene unprecio mucho mas bajo.La estructura de la matriz en el hierro nodular, depende de su composición química, su velocidad de enfriamiento, así como dela forma y cantidad de nódulos de grafito. Cuando lo que se desean son matrices ferríticas se deben mantener los elementosperlitizantes en contenidos lo más bajo posible para desfavorecer de esta forma la solidificación de acuerdo al diagramametaestable.El uso de chatarra plantea el problema de los niveles de manganeso presentes en la misma (generalmente entre 0.6 y 0.8%. Elmanganeso, que es un elemento perlitizante evita la obtención de altas elongaciones ya que la matriz nunca seríacompletamente ferrítica. Es por esto que si se desea usar chatarra de acero como material de carga sea necesario el estudio deformas de eliminarlo del baño.Para la eliminación del manganeso existen diversas referencias en la literatura a procesos desarrollados a base de cloro,oxígeno, sulfatos, óxido de hierro, y procesos con el uso de hornos al vacío.En este trabajo la eliminación del manganeso mediante el uso de óxido de hierro, Sulfato de sodio, y PVC. ha sido investigada,llegando a soluciones factibles para la producción de este tipo de hierro.El primero de los tres métodos ha demostrado ser el más factible ya que el segundo aumenta el contenido de azufre en el baño,requiriendo una desulfuración previa al tratamiento de esferoidización y el tercero de los métodos desprende una gran cantidadde gases da

  20. Clinical benefit of eculizumab in patients with no transfusion history in the International Paroxysmal Nocturnal Haemoglobinuria Registry

    NARCIS (Netherlands)

    Almeida, A.M.; Bedrosian, C.; Cole, A.; Muus, P.; Schrezenmeier, H.; Szer, J.; Rosse, W.F.

    2017-01-01

    BACKGROUND: Eculizumab reduces intravascular haemolysis and improves disease symptoms in patients with paroxysmal nocturnal haemoglobinuria (PNH). AIMS: To characterise, in a real-world setting, the effect of eculizumab in patients with haemolytic PNH (lactase dehydrogenase (LDH) >/= 1.5 upper limit

  1. Influencia de la relación "área nodular / área parenquimal" en la conversión de nódulo tiroideo autónomo compensado (NAC a descompensado (NAD

    Directory of Open Access Journals (Sweden)

    Raúl Sanchez -Vasquez

    2003-01-01

    Full Text Available Objetivo: Como la teoría de la inhibición de TSH por la secreción hormonal nodular, no explica satisfactoriamente la presencia de yodo a nivel parénquima (NAC, ni su ausencia en las gammagrafias en el estadio NAD, se estudió el posible papel de la relación "área nodular/área parenquimal" y su efecto sobre la presencia o ausencia de incorporación de yodo al parénquima en estos estadios gammagráficos. Material y métodos: Se ha estudiado 100 casos de nódulo autónomo divididos en dos grupos: 75 casos de nódulo autónomo compensado (NAC y 25 casos de nódulo autónomo descompensado (NAD, con una edad promedio de 48.8 años, a predominio del sexo femenino y una relación (M/H de 10:1. Resultados: No se encontró diferencia entre el área parénquimal de ambos grupos (p< 0.821, no significativa. Se encontró diferencia entre el área nodular de ambos grupos: (NAC: 4.62 cm², (NAD: 16.14 cm², asi como en el cociente (NAC: 0.13,(NAD: 0.49 de la relación "nodulo/parenquima" con p< 0.0001 altamente significativa. Conclusiones: El análisis de correlación dio resultados significativos en ambos grupos: p< 0.001 y el análisis de regresión mediante la F de Fisher, mostró una relación lineal significativa directa positiva en relación al área del nódulo con el parénquima así como el factor de incremento nodular respecto al parénquima, necesario para pasar de NAC a NAD. El análisis de estos resultados permite plantear la hipótesis que en la evolución de NAC a NAD, frente a áreas parenquimales semejantes, las áreas nodulares son menores en NAC y notablemente mayores en NAD y que la relación del área "nódulo/parénquima" juega un papel importante en la presencia o ausencia de incorporación de yodo radioactivo en el parénquima paranodular (NAC-NAD, fenómeno que guarda relación con el grado de saturación parenquimal de yoduro, que independientemente de la supresión de TSH, se origina por el lento metabolismo intratiroideo de

  2. Diagnostic imaging of focal nodular hyperplasis of the liver developing during nitrofurantoin therapy

    Energy Technology Data Exchange (ETDEWEB)

    Anttinen, H.; Ahonen, A.; Leinonen, A.; Kallioinen, M.; Heikkinen, E.S. (University of Oulu (Finland))

    1982-01-01

    An asymptomatic palpable liver tumor developed in a six-year-old girl seven months after commencement of prophylactic nitrofurantoin therapy for recurrent urinary tract infections. The tumor was examined by /sup 99/ sup (m) Tc colloid radionuclide scan, compound ultrasonography and angiography. Ultrasonography demonstrated a large, solid tumor (5x5x8 cm) in the right lobe of the liver which had an echogenic central core surrounded by an area giving low-amplitude echoes. Angiography disclosed that the tumor was well demarcated and hypervascular, containing large tortuous arteries. The uptake of radionuclide in the tumor was normal. The tumor was resected and the pathological findings were typical for focal nodular hyperplasia (FNH) of the liver. The combination of the findings of these three diagnostic imaging methods is probably specific for uncomplicated (FNH), a benign and innocuous tumor of the liver.

  3. Reactive Retinal Astrocytic Tumor (Focal Nodular Gliosis): Report of the Clinical Spectrum of 3 Cases.

    Science.gov (United States)

    Singh, Arun D; Soto, Hansell; Bellerive, Claudine; Biscotti, Charles V

    2017-09-01

    To report 3 cases providing insight into clinical progression of reactive retinal astrocytic tumor. The clinical, imaging, and when available, the cytologic features of 3 cases of reactive retinal astrocytic tumor (focal nodular gliosis) were reviewed. A 6-year-old female, a 49-year-old man, and a 39-year-old man each developed a white retinal mass associated with laser photocoagulation, lattice degeneration, and treatment of a presumed vascular tumor, respectively. All tumors were white, circumscribed retinal masses that tended to be associated with exudation and either initially or eventually minimal vascularity. Reactive retinal astrocytic tumor can be observed in response to a degenerative, inflammatory, or ischemic retinal insult. Such tumors may progress after therapeutic intervention.

  4. The unique entity of nodular lymphocyte-predominant Hodgkin lymphoma: current approaches to diagnosis and management.

    Science.gov (United States)

    Hawkes, Eliza A; Wotherspoon, Andrew; Cunningham, David

    2012-03-01

    Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare disease constituting only 3-8% of all Hodgkin lymphoma. It has a distinct histological and clinical presentation as well as significantly different natural history compared to the classical form of Hodgkin lymphoma. Presenting most often as early-stage disease, NLPHL is characterized by frequent relapses, but paradoxically an overall good prognosis. The approach to management should therefore reflect this pattern and focus on attaining prolonged remissions, with long-term follow-up paramount. Due to the rarity of the disease, few prospective data exist. Options for treatment include radiotherapy, chemotherapy or combined chemotherapy plus radiotherapy and targeted anti-CD20 antibody therapy, as well as observation in selected patients.

  5. [Nodular regenerative hyperplasia associated with common variable immunodeficiency and other comorbidities].

    Science.gov (United States)

    León, Rafael; Sánchez-Martínez, Rosario; Palazón, José M; Payá, Artemio; Ramos, José M; Pinargote, Héctor

    2016-03-18

    Currently, there are not many data on the evolution of nodular regenerative hyperplasia (NRH) associated or not with underlying diseases and in particular that associated with common variable inmunodeficiency (CVID). Twenty cases of NRH are presented, and the differences between the cases associated with CVID and those related to other diseases are analysed. Retrospective and descriptive study over a period of 14 years. Twelve out of the 20 patients were men; the median age was 51 years. CVID was the main illness associated with NRH. In patients with CVID and NRH, gastrointestinal haemorrhage was more common, all the patients had high gamma glutamyl transferase and alkaline phosphatase and none had altered albumin and bilirubin levels compared to the patients without CVID. On follow-up, 50% of patients with CVID (2/4) had died compared to 33.3% (5/15) without CVID. NRH in patients with CVID seems to have more biochemical data of anicteric cholestasis and portal hypertension and could be associated with lower survival. Copyright © 2015 Elsevier España, S.L.U. All rights reserved.

  6. Acute tubular necrosis in a patient with paroxysmal nocturnal hemoglobinuria

    Directory of Open Access Journals (Sweden)

    Eranga S Wijewickrama

    2013-01-01

    Full Text Available Acute renal failure (ARF is a well-recognized complication of paroxysmal nocturnal hemoglobinuria (PNH. The predominant mechanism is intravascular hemolysis resulting in massive hemoglobinuria ARF. We report a case of acute tubular necrosis (ATN developed in the absence of overwhelming evidence of intravascular hemolysis in a 21-year-old man with anemia, who was eventually diagnosed to have PNH. The patient presented with rapidly deteriorating renal functions in the background of iron deficiency anemia, which was attributed to reflux esophagitis. There was no clinical or laboratory evidence of intravascular hemolysis. Renal biopsy revealed ATN with deposition of hemosiderin in the proximal tubular epithelial cells. Diagnosis of PNH was confirmed with a positive Ham′s test and flow cytometry. Our case emphasizes the need to consider ATN as a possible cause for ARF in patients suspected to have PNH even in the absence of overwhelming evidence of intravascular hemolysis.

  7. 99mTc-MIBI radio-guided minimally invasive parathyroidectomy: experience with patients with normal thyroids and nodular goiters.

    Science.gov (United States)

    Casara, Dario; Rubello, Domenico; Cauzzo, Cristina; Pelizzo, Maria Rosa

    2002-01-01

    The surgical approach to primary hyperparathyroidism (HPT) is changing. In patients with a high probability to be affected by a solitary parathyroid adenoma (PA), a unilateral neck exploration (UNE) or a minimally invasive radio-guided surgery (MIRS) using the intraoperative gamma probe (IGP) technique have recently been proposed. We investigated the role of IGP in a group of 84 patients with primary HPT who were homogeneously evaluated before surgery by a single-day imaging protocol including 99mTcO4/MIBI subtraction scan and neck ultrasound (US) and then operated on by the same surgical team. Quick parathyroid hormone (QPTH) was intraoperatively measured in all cases to confirm successful parathyroidectomy. In 70 patients with scan/US evidence of a single enlarged parathyroid gland (EPG) and with a normal thyroid gland, MIRS was planned. In the other 14 patients, the IGP technique was utilized during a standard bilateral neck exploration (BNE) because of the presence of concomitant nodular goiter (11 cases) or multiglandular disease (MGD) (3 cases). The IGP technique consisted of the following: (1) in the operating room, a low 99mTc-MIBI dose (37 MBq) was injected intravenously during anesthesia induction; (2) subsequently, the patient's neck was scanned with the probe by the surgeon to localize the cutaneous projection of the EPG; (3) in patients who underwent MIRS, the EPG was detected intraoperatively with the probe and removed through a small, 2 to 2.5 cm skin incision; (4) radioactivity was measured on the EPG both in vivo and ex vivo, the thyroid, the background and the parathyroid bed after EPG removal. In patients with concomitant nodular goiter, the radioactivity was also measured on the thyroid nodules. Surgical and pathologic findings were consistent with a single PA in 78 patients, parathyroid carcinoma in 2, and MGD in 4. MIRS was successfully performed in 67 of the 70 patients (97.7%) in whom this approach was planned. It must be pointed out that

  8. Paroxysmal Nocturnal Hemoglobinuria (PNH)

    Science.gov (United States)

    ... on to develop myelodysplastic syndrome (MDS). Special Precautions Airplane Travel and High Altitudes The farther you move ... is. If you have anemia , flying in an airplane or visiting places at higher elevations than you’ ...

  9. Radioiodine therapy for combined disseminated and nodular thyroid autonomy. Results after using a correction term for the disseminated part

    International Nuclear Information System (INIS)

    Vogt, H.; Dorn, R.; Otto, I.; Sciuk, J.; Wengenmair, H.; Kopp, J.

    2006-01-01

    Aim: in combined focal and disseminated thyroid autonomy a variety of concepts in the treatment with radioiodine are used. The difference lies mainly in the calculation of the autonomous volume. This retrospective study shows a new method of calculating the autonomous volume. Patients and methods: in 398 patients with combined thyroid autonomy and good correlation of scintigraphically hot nodules and lesions defined by ultrasound the volume of the nodules is ascertained from scintigraphic and ultrasound parameters and the volume of the disseminated autonomous tissue is assessed with a weighting factor (VF). This factor is the ratio of impulse density in a ROI over the disseminated volume divided by the corresponding impulse density over the nodular volume of the thyroid scintigraphy. The sum of nodular volume and weighted perinodular volume gives the total autonomous volume. A standard radioiodine test gives the maximum iodine-131-uptake and effective half-life to calculate the activity to obtain a treatment dose of 400 Gy. Results: the rate of success with and without thyrostatic medication was 97% with an 18.6% rate of hypothyroidism observed from 4 months post therapy onwards. Conclusion: the use of the weighting factor VF in the treatment of combined autonomy leads to an excellent rate of success in patients with good correlation of functional imaging and ultrasound findings. (orig.)

  10. Study of factors that influence the outcome of 131I treatment in hyperthyroidism secondary to nodular goitre.

    Science.gov (United States)

    Tabuenca-Dopico, O; Boente-Varela, R; Lamas-Ferreiro, J L

    To assess the outcome after 131 I treatment in patients with multinodular (MNG) and nodular toxic goitre (NTG) according to the administered dose and other factors related to the patient, pathology, or previous treatments. A retrospective study was conducted on 108 patients (67 MNG and 41 NTG) treated in our department, with a follow-up period of at least 2 years. Development of hypothyroidism and treatment failure were evaluated along with their relationship with the administered dose and other factors such as age, sex, grade of hyperthyroidism, type of goitre, presence of autoimmunity, or previous antithyroid medication. More than one-third (36.9%) of MNG patients, and even higher proportion of NTG patients (51.2%) developed non-transient hypothyroidism, particularly in those receiving 740MBq (66.7%). No relationship was found with any other variable. The development of early hypothyroidism (before one year) was also not related to any variable. Treatment failure was not related to the dose, but in MNG there was a relationship with male gender, presence of autoimmunity, or previous antithyroid drugs use. The high rate of hypothyroidism obtained with high doses of 131 I in hyperthyroidism secondary to nodular goitre treatment suggests that lower doses might be sufficient to control the disease without an increase in treatment failures. Only patients with positive autoimmunity, in previous anti-thyroid medication, and perhaps male gender in MNG might be given higher doses, as the failure rate increases, but further studies are required. Copyright © 2017 Elsevier España, S.L.U. y SEMNIM. All rights reserved.

  11. Clinical impact of duodenal pancreatic heterotopia - Is there a need for surgical treatment?

    Science.gov (United States)

    Betzler, Alexander; Mees, Soeren T; Pump, Josefine; Schölch, Sebastian; Zimmermann, Carolin; Aust, Daniela E; Weitz, Jürgen; Welsch, Thilo; Distler, Marius

    2017-05-08

    Pancreatic heterotopia (PH) is defined as ectopic pancreatic tissue outside the normal pancreas and its vasculature and duct system. Most frequently, PH is detected incidentally by histopathological examination. The aim of the present study was to analyze a large single-center series of duodenal PH with respect to the clinical presentation. A prospective pancreatic database was retrospectively analyzed for cases of PH of the duodenum. All pancreatic and duodenal resections performed between January 2000 and October 2015 were included and screened for histopathologically proven duodenal PH. PH was classified according to Heinrich's classification (Type I acini, ducts, and islet cells; Type II acini and ducts; Type III only ducts). A total of 1274 pancreatic and duodenal resections were performed within the study period, and 67 cases of PH (5.3%) were identified. The respective patients were predominantly male (72%) and either underwent pancreatoduodenectomy (n = 60); a limited pancreas resection with partial duodenal resection (n = 4); distal pancreatectomy with partial duodenal resection (n = 1); total pancreatectomy (n = 1); or enucleation (n = 1). Whereas 65 patients (83.5%) were asymptomatic, 11 patients (18.4%) presented with symptoms related to PH (most frequently with abdominal pain [72%] and duodenal obstruction [55%]). Of those, seven patients (63.6%) had chronic pancreatitis in the heterotopic pancreas. The risk of malignant transformation into adenocarcinoma was 2.9%. PH is found in approximately 5% of pancreatic or duodenal resections and is generally asymptomatic. Chronic pancreatitis is not uncommon in heterotopic pancreatic tissue, and even there is a risk of malignant transformation. PH should be considered for the differential diagnosis of duodenal lesions and surgery should be considered, especially in symptomatic cases.

  12. Biphasic and SAPC Hydroformylation Catalyzed by Rh-phosphines Bound to Water-Soluble Polymers

    DEFF Research Database (Denmark)

    Malmstrøm, Torsten; Andersson, Carlaxel; Hjortkjær, Jes

    1999-01-01

    Coupling of the triphenylphosphine moiety to poly-acrylic acid and poly-ethyleneimine respectively afford the macromolecular ligands PAA-PNH and PEI-PNH. Reaction of the ligands with Rh(CO)2(acac) give water-soluble complexes that are active as catalysts in the hydroformylation ofdifferent olefins...

  13. Improvement of Renal Function by Long-Term Sustained Eculizumab Treatment in a Patient with Paroxysmal Nocturnal Hemoglobinuria

    Directory of Open Access Journals (Sweden)

    Haruhiko Ninomiya

    2015-01-01

    Full Text Available Chronic kidney disease (CKD is one of the major manifestations of paroxysmal nocturnal hemoglobinuria (PNH. CKD in PNH is induced mainly by intravascular hemolysis of PNH-affected red blood cells (RBC missing the glycosylphosphatidylinositol-anchored proteins with complement-regulatory activities, CD55 and CD59. CKD develops by heme absorption in the proximal tubules resulting in the interstitial deposition of iron in the kidneys. We administered eculizumab to a patient with PNH, who was one of 29 patients enrolled in the AEGIS clinical trial, an open-label study of eculizumab in Japan. The patient was complicated by stage 3 CKD with impaired estimated glomerular filtration rate (eGFR, at grade G3b, and had obvious proteinuria (2-3+, 1-2 g/day. In a two-year extension to the 12-week AEGIS study, eGFR improved significantly, and the eGFR has since been maintained at grade G2 without proteinuria by sustained eculizumab treatment (>6 years. Renal function improved and maintained by long-term sustained eculizumab treatment, presumably by clearance of iron from the kidney as well as inhibition of the production of anaphylatoxin C5a, even in advanced stages of CKD, is one of the benefits of eculizumab treatment in PNH.

  14. Primary pigmented nodular adrenocortical disease associated with Carney complex: case report and literature review

    Directory of Open Access Journals (Sweden)

    Fabrícia Torres Gonçalves

    Full Text Available CONTEXT: Carney complex (CNC, a familial multiple neoplasm syndrome with dominant autosomal transmission, is characterized by tumors of the heart, skin, endocrine and peripheral nervous system, and also cutaneous lentiginosis. This is a rare syndrome and its main endocrine manifestation, primary pigmented nodular adrenal disease (PPNAD, is an uncommon cause of adrenocorticotropic hormone-independent Cushing's syndrome. CASE REPORT: We report the case of a 20-year-old patient with a history of weight gain, hirsutism, acne, secondary amenorrhea and facial lentiginosis. Following the diagnosing of CNC and PPNAD, the patient underwent laparoscopic bilateral adrenalectomy, and she evolved with decreasing hypercortisolism. Screening was also performed for other tumors related to this syndrome. The diagnostic criteria, screening and follow-up for patients and affected family members are discussed.

  15. Correlation between intravoxel incoherent motion MR parameters and MR nodular grade of parotid glands in patients with Sjögren’s syndrome: A pilot study

    International Nuclear Information System (INIS)

    Chu, Chen; Zhou, Nan; Zhang, Huayong; Dou, Xin; Li, Ming; Liu, Song; Zhu, Yun; Chen, Weibo; Chan, Queenie; He, Jian; Sun, Lingyun; Zhou, Zhengyang

    2017-01-01

    Highlights: • Parotid glands at grade 0–3 had significant values from those in healthy glands. • Parotid D and f values were correlated with MR nodule grade significantly. • There were significant differences of D, f, and D * values among glands at grade 0–3. - Abstract: Purpose: To explore the correlation between intravoxel incoherent motion (IVIM) magnetic resonance (MR) parameters and MR nodular grade of parotid glands in patients with Sjögren’s syndrome (SS). Materials and methods: A total of 31 consecutive patients with SS and 28 gender- and age-matched healthy volunteers underwent bilateral parotid 3.0T MR examination including the IVIM sequence (9 b values, 0–800 s/mm 2 ). The apparent diffusion coefficient (ADC), diffusion coefficient D, pseudo-diffusion coefficient D * , and perfusion fraction f of bilateral parotid glands were obtained, and the nodular grade of each parotid gland was evaluated according to the MR morphological appearance. Results: Sixty-two parotid glands in 31 patients with SS consisted of 32, 14, 8, and 8 parotid glands at MR nodular grades 0, 1, 2, and 3, respectively. In parotid glands of grade 0, 1, 2, 3 and healthy volunteers, the ADC values were (1.13 ± 0.25, 1.11 ± 0.17, 1.05 ± 0.24, 0.89 ± 0.04 and 1.00 ± 0.21) × 10 −3 mm 2 /s, D values were (0.92 ± 0.13, 0.90 ± 0.19, 0.90 ± 0.03, 0.67 ± 0.03, 0.81 ± 0.03) × 10 −3 mm 2 /s, f values were 0.20 ± 0.04, 0.18 ± 0.02, 0.15 ± 0.01, 0.11 ± 0.01, 0.15 ± 0.06, and D * values were (53.89 ± 28.26, 41.78 ± 16.35, 51.24 ± 18.69, 31.83 ± 18.03, 36.83 ± 16.14) × 10 −3 mm 2 /s respectively. The ADC, D, f, and D * values of parotid glands in patients with SS at grade 0 were significantly higher than those in healthy volunteers (all P < 0.05). Significant differences were observed in the D and f values of parotid glands in patients with SS among different grades (P = 0.003, < 0.001, respectively). The IVIM parameters (D, f) of parotid glands at early

  16. Correlation between intravoxel incoherent motion MR parameters and MR nodular grade of parotid glands in patients with Sjögren’s syndrome: A pilot study

    Energy Technology Data Exchange (ETDEWEB)

    Chu, Chen, E-mail: chuchen19920905@163.com [Department of Radiology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, 210008 (China); Zhou, Nan, E-mail: snscorpion@163.com [Department of Radiology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, 210008 (China); Zhang, Huayong, E-mail: 13770560567@163.com [Department of Rheumatology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, 210008 (China); Dou, Xin, E-mail: douxin125@126.com [Department of Radiology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, 210008 (China); Li, Ming, E-mail: lm069393@163.com [Department of Radiology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, 210008 (China); Liu, Song, E-mail: liusongnj@126.com [Department of Radiology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, 210008 (China); Zhu, Yun, E-mail: qqt111@hotmail.com [Department of Rheumatology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, 210008 (China); Chen, Weibo, E-mail: Weibo.Chen@philips.com [Philips Healthcare, Shanghai, 200233 (China); Chan, Queenie, E-mail: queenie.chan@philips.com [Philips Healthcare, Hong Kong (China); He, Jian, E-mail: hjxueren@126.com [Department of Radiology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, 210008 (China); Sun, Lingyun, E-mail: lysun_nju@163.com [Department of Rheumatology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, 210008 (China); Zhou, Zhengyang, E-mail: zyzhou@nju.edu.cn [Department of Radiology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, 210008 (China)

    2017-01-15

    Highlights: • Parotid glands at grade 0–3 had significant values from those in healthy glands. • Parotid D and f values were correlated with MR nodule grade significantly. • There were significant differences of D, f, and D{sup *} values among glands at grade 0–3. - Abstract: Purpose: To explore the correlation between intravoxel incoherent motion (IVIM) magnetic resonance (MR) parameters and MR nodular grade of parotid glands in patients with Sjögren’s syndrome (SS). Materials and methods: A total of 31 consecutive patients with SS and 28 gender- and age-matched healthy volunteers underwent bilateral parotid 3.0T MR examination including the IVIM sequence (9 b values, 0–800 s/mm{sup 2}). The apparent diffusion coefficient (ADC), diffusion coefficient D, pseudo-diffusion coefficient D{sup *}, and perfusion fraction f of bilateral parotid glands were obtained, and the nodular grade of each parotid gland was evaluated according to the MR morphological appearance. Results: Sixty-two parotid glands in 31 patients with SS consisted of 32, 14, 8, and 8 parotid glands at MR nodular grades 0, 1, 2, and 3, respectively. In parotid glands of grade 0, 1, 2, 3 and healthy volunteers, the ADC values were (1.13 ± 0.25, 1.11 ± 0.17, 1.05 ± 0.24, 0.89 ± 0.04 and 1.00 ± 0.21) × 10{sup −3} mm{sup 2}/s, D values were (0.92 ± 0.13, 0.90 ± 0.19, 0.90 ± 0.03, 0.67 ± 0.03, 0.81 ± 0.03) × 10{sup −3} mm{sup 2}/s, f values were 0.20 ± 0.04, 0.18 ± 0.02, 0.15 ± 0.01, 0.11 ± 0.01, 0.15 ± 0.06, and D{sup *}values were (53.89 ± 28.26, 41.78 ± 16.35, 51.24 ± 18.69, 31.83 ± 18.03, 36.83 ± 16.14) × 10{sup −3} mm{sup 2}/s respectively. The ADC, D, f, and D{sup *} values of parotid glands in patients with SS at grade 0 were significantly higher than those in healthy volunteers (all P < 0.05). Significant differences were observed in the D and f values of parotid glands in patients with SS among different grades (P = 0.003, < 0.001, respectively). The IVIM

  17. Vesico-ureteral reflux in children with prenatally detected hydronephrosis: a systematic review

    NARCIS (Netherlands)

    van Eerde, A. M.; Meutgeert, M. H.; de Jong, T. P. V. M.; Giltay, J. C.

    2007-01-01

    To investigate the value of prenatally detected hydronephrosis (PNH) as a prognostic factor for vesico-ureteral reflux (VUR). The MEDLINE database was searched for articles on PNH and VUR published between 1980 and 2004. A total of 18 studies were identified and reviewed for various aspects. Results

  18. Ehlers-Danlos syndrome with lethal cardiac valvular dystrophy in males carrying a novel splice mutation in FLNA.

    Science.gov (United States)

    Ritelli, Marco; Morlino, Silvia; Giacopuzzi, Edoardo; Carini, Giulia; Cinquina, Valeria; Chiarelli, Nicola; Majore, Silvia; Colombi, Marina; Castori, Marco

    2017-01-01

    Filamin A is an X-linked, ubiquitous actin-binding protein whose mutations are associated to multiple disorders with limited genotype-phenotype correlations. While gain-of-function mutations cause various bone dysplasias, loss-of-function variants are the most common cause of periventricular nodular heterotopias with variable soft connective tissue involvement, as well as X-linked cardiac valvular dystrophy (XCVD). The term "Ehlers-Danlos syndrome (EDS) with periventricular heterotopias" has been used in females with neurological, cardiovascular, integument and joint manifestations, but this nosology is still a matter of debate. We report the clinical and molecular update of an Italian family with an X-linked recessive soft connective tissue disorder and which was described, in 1975, as the first example of EDS type V of the Berlin nosology. The cutaneous phenotype of the index patient was close to classical EDS and all males died for a lethal cardiac valvular dystrophy. Whole exome sequencing identified the novel c.1829-1G>C splice variation in FLNA in two affected cousins. The nucleotide change was predicted to abolish the canonical splice acceptor site of exon 13 and to activate a cryptic acceptor site 15 bp downstream, leading to in frame deletion of five amino acid residues (p.Phe611_Gly615del). The predicted in frame deletion clusters with all the mutations previously identified in XCVD and falls within the N-terminus rod 1 domain of filamin A. Our findings expand the male-specific phenotype of FLNA mutations that now includes classical-like EDS with lethal cardiac valvular dystrophy, and offer further insights for the genotype-phenotype correlations within this spectrum. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  19. Creep testing of nodular iron at ambient and elevated temperatures

    Energy Technology Data Exchange (ETDEWEB)

    Martinsson, Aasa; Andersson-Oestling, Henrik C.M.; Seitisleam, Facredin; Wu, Rui; Sandstroem, Rolf (Swerea KIMAB AB, Stockholm (Sweden))

    2010-12-15

    The creep strain at room temperature, 100 and 125 deg C has been investigated for the ferritic nodular cast iron insert intended for use as the load-bearing part of canisters for long term disposal of spent nuclear fuel. The microstructure consisted of ferrite, graphite nodules of different sizes, compacted graphite and pearlite. Creep tests have been performed for up to 41,000 h. The specimens were cut out from material taken from two genuine inserts, I30 and I55. After creep testing, the specimens from the 100 deg C tests were hardness tested and a metallographic examination was performed. Creep strains at all temperatures appear to be logarithmic, and accumulation of creep strain diminishes with time. The time dependence of the creep strain is consistent to the W-model for primary creep. During the loading plastic strains up to 1% appeared. The maximum recorded creep strain after the loading phase was 0.025%. This makes the creep strains technically insignificant. Acoustic emission recordings during the loading of the room temperature tests showed no sounds or other evidence of microcracking during the loading phase. There is no evidence that the hardness or the graphite microstructure changed during the creep tests

  20. Paroxysmal Nocturnal Hemoglobinuria Superimposed with Preeclampsia

    Directory of Open Access Journals (Sweden)

    Mann-Ling Chen

    2006-09-01

    Conclusion: The most frequent causes of PNH-related fetomaternal morbidity and mortality are hemolysis and thrombosis. The situation becomes even more complicated when PNH is superimposed with preeclampsia. Appropriate clinical surveillance, awareness of the potential risks of hemolysis and thrombosis, as well as evaluation of fetal wellbeing are essential.

  1. Investigations of Caspr2, an autoantigen of encephalitis and neuromyotonia

    Science.gov (United States)

    Lancaster, Eric; Huijbers, Maartje GM; Bar, Vered; Boronat, Anna; Wong, Andrew; Martinez-Hernandez, Eugenia; Wilson, Christina; Jacobs, Dina; Lai, Meizan; Walker, Russell W; Graus, Francesc; Bataller, Luis; Illa, Isabel; Markx, Sander; Strauss, Kevin A.; Peles, Elior; Scherer, Steven S; Dalmau, Josep

    2010-01-01

    Objective To report clinical and immunological investigations of contactin-associated protein-like 2 (Caspr2), an autoantigen of encephalitis and peripheral nerve hyperexcitability (PNH) previously attributed to voltage-gated potassium channels (VGKC). Methods Clinical analysis of patients with encephalitis, PNH, or both. Immunoprecipitation and mass spectrometry were used to identify the antigen and to develop an assay with Caspr2-expressing cells. Immunoabsorption with Caspr2 and comparative immunostaining of brain and peripheral nerve of wild-type and Caspr2-null mice were used to assess antibody specificity. Results Using Caspr2-expressing cells, antibodies were identified in 8 patients but not in 140 patients with several types of autoimmune or viral encephalitis, PNH, or mutations of the Caspr2-encoding gene. Patients’ antibodies reacted with brain and peripheral nerve in a pattern that co-localized with Caspr2. This reactivity was abrogated after immunoabsorption with Caspr2 and was absent in tissues from Caspr2-null mice. Of the 8 patients with Caspr2 antibodies, 7 had encephalopathy or seizures, 5 neuropathy or PNH, and 1 isolated PNH. Three patients had also myasthenia gravis, bulbar weakness, or symptoms that initially suggested motor neuron disease. None of the patients had active cancer; 7 responded to immunotherapy and were healthy or only mildly disabled at last follow-up (median 8 months, range 6–84). Interpretation Caspr2 is an autoantigen of encephalitis and PNH previously attributed to VGKC antibodies. The occurrence of other autoantibodies may result in a complex syndrome that at presentation could be mistaken for a motor neuron disorder. Recognition of this disorder is important because it responds to immunotherapy. PMID:21387375

  2. OXIDATIVE STRESS IN HUMAN THYROID GLAND UNDER IODINE DEFICIENCY NODULAR GOITER: FROM HARMLESSNESS TO HAZARD DEPENDING ON COPPER AND IODINE SUBCELLULAR DISTRIBUTION

    Directory of Open Access Journals (Sweden)

    H. Falfushynska

    2014-12-01

    Conclusions. Excess of copper unbound to metallothionein in goitrous-changed tissue and high level of inorganic iodine could be the reason for elevated DNA fragmentation and increased lysosomal membrane permeability and activation of antioxidant defense. The main criterions of goiter formation were represented by low level of organificated iodine and high level of DNA damage in thyroid gland. KEY WORDS: iodine deficiency nodular colloidal goiter, iodine, copper, metallothioneins, oxidative stress, cytotoxicity

  3. OBSERVATION OF FATIGUE CRACK PATHS IN NODULAR CAST IRON AND ADI MICROSTRUCTURES

    Directory of Open Access Journals (Sweden)

    Lukáš Bubenko

    2009-07-01

    Full Text Available When speaking about quality of construction materials, fatigue crack propagation resistance is one of the most important considered properties. That is essentially influenced by character of matrix. Here presented contribution deals with the fatigue crack propagation mode through the matrix of as-cast nodular cast iron (NCI and austempered ductile iron (ADI, whereas influence of microstructure has been considered and discussed. Experimental materials used in presented contribution were pearlitc-ferritic NCI and heat treated ADI 800. Pearlitic-ferritic NCI was used as the base for ADI production. Experiments were performed on mini round compact tension (RCT specimens using an Amsler vibrophore. Fatigue crack paths in both materials were investigated and compared. Light microscopy was used to analyze the microstructure, crack initiation and propagation within broken specimens. In both tested materials fatigue cracks always initiated at graphite-matrix interface, while graphite nodules remained generally unbroken, eventually only surface of nodules was damaged. Though, comparing two materials with different microstructures, the diversity of fatigue crack propagation modes at high deltaK and low deltaK was observed.

  4. Travels Through Heterotopia: The Textual Realms of Patrick Modiano's Rue des Boutiques Obscures and Mikhail Kuraev's Kapitan Dikshtein

    Directory of Open Access Journals (Sweden)

    Vitaly Chernetsky

    1998-06-01

    Full Text Available Within contemporary prose, one distinct mode or paradigm that can be discerned is constituted by the texts that daringly tackle the dark, suppressed, erased parts of our history and mentality; however, they approach this task not by way of self-righteous denunciatory investigations, but by provocatively problematizing the most established everyday facts, by depriving the reader of the possibility of even conceiving any firm ground of the stable construct of an origin or a self-identification—historically and culturally. Their irreverent and playful deconstruction of the all-pervasive national cultural mythologies has mounted a powerful challenge to ideological constructs big and small. This article considers two representative examples of texts of this kind, which the author proposes to call heterotopic: Patrick Modiano's Rue des Boutiques Obscures and Mikhail Kuraev's Kapitan Dikshtein . It offers an attempt at defining this paradigm through a reading of these two novels, drawing upon Michel Foucault's usage of the term "heterotopia" for the purpose of designating the "other" cultural spaces of our civilization, as well as on Deleuze and Guattari's concept of "minor literature" and the work of several other theorists. The texts of the kind exemplified by these two novels are considered as an instance of successful partaking in the project of cognitive mapping, which has been proposed by Fredric Jameson and others as the positive political edge of postmodern culture.

  5. Sclerosing Angiomatoid Nodular Transformation: Laparoscopic Splenectomy as Therapeutic and Diagnostic Approach at the Same Time.

    Science.gov (United States)

    Cipolla, Calogero; Florena, Ada Maria; Ferrara, Gabriella; Di Gregorio, Riccardo; Unti, Elettra; Giannone, Antonino G; Lazzaro, Luigi A; Graceffa, Giuseppa; Pantuso, Gianni

    2018-01-01

    Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare benign vascular lesion with unknown etiopathogenesis and with definite features of imaging, histopathology, and immunohistochemistry. It was first described by Martel et al. in 2004, and to date, only 151 cases have been reported. We report a case of SANT of the spleen detected in a 66-year-old Caucasian, without comorbidities, presented to our department with epigastric pain. We, also, presented a review of the literature. SANT is a benign incidentally vascular condition in the majority of cases. The wide age and gender distribution in our review is in accordance with that in previous studies in English literature. In our opinion, splenectomy is the choice treatment because it is at the same time diagnostic and therapeutic in a definitive way.

  6. Duodenal Bulb Mucosa with Hypertrophic Gastric Oxyntic Heterotopia in Patients with Zollinger Ellison Syndrome

    Science.gov (United States)

    Kohan, Emil; Oh, David; Wang, Hank; Hazany, Salar; Ohning, Gordon; Pisegna, Joseph R.

    2009-01-01

    Objectives. Zollinger-Ellison Syndrome (ZES) results in hypersecretion of gastric acid (via gastrinoma) leading to peptic ulcers, diarrhea, and abdominal pain. We describe the novel discovery of hypertrophic, heterotopic gastric mucosa in the proximal duodenal bulb in patients with ZES, which we hypothesize results in an increased incidence of postbulbar ulcers in patients with ZES (a mechanism previously unreported). We determined the incidence of the novel finding of duodenal gastric oxyntic hypertrophic heterotopia (GOH) in patients with ZES. Methods. Seven patients with ZES were enrolled. The diagnosis of ZES was established by hypergastrinemia, gastric acid hypersecretion, and a positive secretin test or based on biopsy specimens (evaluated via tissue staining). Basal acid output (BAO) and baseline gastrin secretion were determined by established methods. Endoscopic examinations with methylene blue staining and biopsy of the gastric and duodenal mucosa were conducted in all patients every 3–6 months for an average of 5 years. Results. The duodenal mucosa demonstrated hypertrophic GOH in 5 out of 7 patients with ZES and an intact stomach and duodenum. Biopsies from the bowel mucosa demonstrated patchy replacement of surface epithelium by gastric-type epithelium with hypertrophic oxyntic glands in the lamina propria in 5 patients. Two of the patients had no evidence of GOH in the duodenal bulb. Patients with GOH had an average serum gastrin level of 1245 pg/mL and BAO of 2.92 mEq/hr versus 724 pg/mL and 0.8 mEq/hr in patients without GOH. Conclusions. This study demonstrated the presence of duodenal mucosa with GOH in 5 out of 7 patients with ZES and an intact stomach and duodenum. The presence of hypertrophic and heterotopic gastric mucosa is proposed to result from increased gastrin levels and may contribute to the increased incidence of postbulbar ulcers in these patients. PMID:19587828

  7. Duodenal Bulb Mucosa with Hypertrophic Gastric Oxyntic Heterotopia in Patients with Zollinger Ellison Syndrome

    Directory of Open Access Journals (Sweden)

    Emil Kohan

    2009-01-01

    Full Text Available Objectives. Zollinger-Ellison Syndrome (ZES results in hypersecretion of gastric acid (via gastrinoma leading to peptic ulcers, diarrhea, and abdominal pain. We describe the novel discovery of hypertrophic, heterotopic gastric mucosa in the proximal duodenal bulb in patients with ZES, which we hypothesize results in an increased incidence of postbulbar ulcers in patients with ZES (a mechanism previously unreported. We determined the incidence of the novel finding of duodenal gastric oxyntic hypertrophic heterotopia (GOH in patients with ZES. Methods. Seven patients with ZES were enrolled. The diagnosis of ZES was established by hypergastrinemia, gastric acid hypersecretion, and a positive secretin test or based on biopsy specimens (evaluated via tissue staining. Basal acid output (BAO and baseline gastrin secretion were determined by established methods. Endoscopic examinations with methylene blue staining and biopsy of the gastric and duodenal mucosa were conducted in all patients every 3–6 months for an average of 5 years. Results. The duodenal mucosa demonstrated hypertrophic GOH in 5 out of 7 patients with ZES and an intact stomach and duodenum. Biopsies from the bowel mucosa demonstrated patchy replacement of surface epithelium by gastric-type epithelium with hypertrophic oxyntic glands in the lamina propria in 5 patients. Two of the patients had no evidence of GOH in the duodenal bulb. Patients with GOH had an average serum gastrin level of 1245 pg/mL and BAO of 2.92 mEq/hr versus 724 pg/mL and 0.8 mEq/hr in patients without GOH. Conclusions. This study demonstrated the presence of duodenal mucosa with GOH in 5 out of 7 patients with ZES and an intact stomach and duodenum. The presence of hypertrophic and heterotopic gastric mucosa is proposed to result from increased gastrin levels and may contribute to the increased incidence of postbulbar ulcers in these patients.

  8. MRI findings of multiple focal nodular hyperplasia of the liver

    International Nuclear Information System (INIS)

    Wang Xin; Yu Qingtai; Jing Yu; Wang Haiyi; Pan Jingjing; Duan Weidong; Wang Dianjun; Ye Huiyi

    2010-01-01

    Objective: To assess the diagnostic value of MRI on multiple focal nodular hyperplasia (FNH) of the liver. Methods: MR images of 9 cases with pathological-confirmed multiple FNH were retrospectively analyzed. MRI features of the lesions were correlated with pathological findings. Results: Multiple FNH was considered in all these 9 cases. Among them, the primary diagnosis was FNH in 5, hepatic adenoma in 3 and fibrolamellar hepatocellular carcinoma in 1 case. A total of 31 lesions were detected in the 9 cases. On T 2 WI, 19 lesions presented slightly high-signal intensity, and the other 12 presented iso-signal intensity. On T 1 WI, 12 lesions presented slightly low-signal intensity, 7 presented iso-signal intensity, and the other 12 presented high-signal intensity. On opposed-phase, the signal intensity of 1 lesion dropped unevenly. After bolus injection of contrast agent Gd-DTPA, in hepatic arterial phase 18 lesions showed mild to marked heterogeneous enhancement, 11 showed marked homogeneous enhancement, 1 showed moderate ring-like enhancement, and the last one did not have obvious enhancement. In portal venous and delayed phase, all the lesions turned to iso- or slightly high-signal intensity gradually. Sixteen of 31 lesions presented central scar, which demonstrated mild star-like enhancement in delayed phase. Conclusion: Multiple FNH presented certain MRI features, which contributed to the preoperative diagnosis. (authors)

  9. A Moessbauer study of hemoglobin in paroxysmal nocturnal hemoglobinuria

    International Nuclear Information System (INIS)

    Zamorano-Ulloa, R.; Yee-Madeira, H.; Flores-Llamas, H.; Perez-Ramirez, J.G.

    1991-01-01

    The 57 Fe Moessbauer spectra of concentrated hemoglobin (Hb) of normal subjects and six patients with Paroxysmal Nocturnal Hemoglobinuria (PNH) were studied at 300deg K and 77 K. PNH is a very rate autoimmune hematological disease. The possibility of structural alterations of Hb induced by, or as part of the altered PNH-red cell membrane was the objective of this study. The Moessbauer parameters of the Hb of the normal subjects, both at 300 K and at 77 K, are identical to values previously reported. The PNH-Hb spectra show clear differences. They are wider and more asymmetric. At 77 K, an extra doublet grows in with an isomer shift of 0.425 mm/sec. and a quadrupolar splitting of 1.951 mm/sec. The other two doublets have δ's and ΔQ's slightly, but significantly, different from the corresponding values for normal Hb. These results are rationalized in terms of a population of Hb molecules with structures varying very slightly in a narrow range. The spread in structures manifests itself in a wider and more asymmetric Moessbauer spectrum. (orig.)

  10. Disgenesia do corpo caloso e más-formações associadas: achados de tomografia computadorizada e ressonância magnética Dysgenesis of the corpus callosum and associated malformations: computed tomography and magnetic resonance imaging findings

    Directory of Open Access Journals (Sweden)

    Cristiano Montandon

    2003-10-01

    Full Text Available O termo disgenesia do corpo caloso refere-se a uma má-formação deste com origem na embriogênese do telencéfalo. O relato analisa os achados de tomografia computadorizada e ressonância magnética em 11 pacientes com disgenesia calosa e em um caso de corpo caloso normal com lipoma associado. Esta pode ser distinguida em três grupos: agenesia total (três casos, agenesia parcial (seis casos e hipoplasia (dois casos. Anomalias associadas foram observadas em nove casos, incluindo má-formação de Chiari tipo II (um caso, esquizencefalia (um caso, cisto inter-hemisférico (dois casos, heterotopia nodular (um caso, cisto de Dandy-Walker (um caso e lipoma do corpo caloso (quatro casos. Este artigo demonstra um espectro destes distúrbios, auxiliando na sua interpretação diagnóstica.Callosal dysgenesis is a malformation of the corpus callosum with origins in the embryogenesis of the telencephalon. We reviewed the computed tomography and magnetic resonance imaging findings of 11 patients with callosal dysgenesis and one patient with normal corpus callosum associated with a lipoma. The patients were divided into three distinct groups: total agenesis (three patients, partial agenesis (six patients and hypoplasia (two patients. Associated abnormalities were observed in nine patients, including Chiari II malformation (one patient, schizencephaly (one patient, interhemispheric cyst (two patients, Dandy-Walker cyst (one patient, nodular heterotopy (one patient and lipoma of the corpus callosum (four patients. This paper presents a review that may contribute to the diagnosis of these disorders.

  11. Familial isolated primary pigmented nodular adrenocortical disease associated with a novel low penetrance PRKAR1A gene splice site mutation

    DEFF Research Database (Denmark)

    Storr, Helen L; Metherell, Louise A; Dias, Renuka

    2010-01-01

    Primary pigmented nodular adrenocortical disease (PPNAD) is associated with inactivating germline protein kinase A regulatory subunit type 1-alpha (PRKAR1A) mutations and loss of heterozygosity at the 17q22-24 locus in approximately 50% patients. PRKAR1A mutations are observed in both isolated PP...... PPNAD (iPPNAD) and Carney complex (CNC). Most mutations result in a functionally null-allele and exhibit high penetrance. We genotyped members of an extended family for a novel PRKAR1A mutation and undertook detailed phenotyping for CNC in the affected individuals....

  12. [The imaging diagnosis of hepatic focal nodular hyperplasia].

    Science.gov (United States)

    Bazzocchi, M; Macorig, D; Cecconi, P; Gozzi, G

    1991-12-01

    Focal nodular hyperplasia (FNH) is a rare benign hepatocellular tumor occurring in noncirrhotic patients, mostly females, 20-50 years of age. It is usually asymptomatic. The authors took the lead from 5 cases of FNH studied over last year to analyze the different patterns exhibited by the condition on the various imaging techniques currently available. At scintigraphy with 99mTc DISIDA or with TcSC, FNH can be hyper, normal, or hypocaptating. On US scans, the lesion is often homogeneous and isoechoic, but it can also be hyper/hypoechoic. With Doppler US, high-flow signals can be observed. On unenhanced CT scans the lesion is solid, well-demarcated, isodense or slightly hyperdense; sometimes it shows a central hypodense area corresponding to fibrovascular scar. On postcontrast scans it appears hyper/isodense. At dynamic CT the lesion density, which is high during the arterial phase, decreases quickly in the parenchymal and the venous phases and reaches equal/inferior values to surrounding liver parenchyma. On liver angio-CT it is sometimes possible to visualize the bile ducts in the central scar. At angiography, FNH is hypervascular and homogeneous. On MR scans, in T1-weighted SE sequences, the condition is isointense or slightly hypointense, whereas on T2-weighted pulse sequences it is slightly hyperintense; the central scar is hypointense on T1, and hyperintense on T2, weighted scans. As we have no pathognomonic patterns but only orientative ones, a reliable differential diagnosis with hepatocellular adenoma (HA) and fibrolamellar hepatocellular carcinoma (FL-HCC) must be based on biopsy or cytology or, even better, histology. The differential diagnosis is nevertheless necessary because, while FNH does not usually require a surgical approach but only a radiological follow-up, both HA (due to possible bleeding and degeneration) and FL-HCC require surgery.

  13. Sclerosing Angiomatoid Nodular Transformation: Laparoscopic Splenectomy as Therapeutic and Diagnostic Approach at the Same Time

    Directory of Open Access Journals (Sweden)

    Calogero Cipolla

    2018-01-01

    Full Text Available Introduction. Sclerosing angiomatoid nodular transformation (SANT of the spleen is a rare benign vascular lesion with unknown etiopathogenesis and with definite features of imaging, histopathology, and immunohistochemistry. It was first described by Martel et al. in 2004, and to date, only 151 cases have been reported. Case Description. We report a case of SANT of the spleen detected in a 66-year-old Caucasian, without comorbidities, presented to our department with epigastric pain. We, also, presented a review of the literature. Conclusions. SANT is a benign incidentally vascular condition in the majority of cases. The wide age and gender distribution in our review is in accordance with that in previous studies in English literature. In our opinion, splenectomy is the choice treatment because it is at the same time diagnostic and therapeutic in a definitive way.

  14. Evaluation of Danazol, Cyclosporine, and Prednisolone as Single Agent or in Combination for Paroxysmal Nocturnal Hemoglobinuria

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    Kanjaksha Ghosh

    2013-12-01

    Full Text Available OBJECTIVE: The responses of 32 patients with paroxysmal nocturnal hemoglobinuria (PNH were assessed after the patients were put on various combinations of danazol, prednisolone, and cyclosporine. METHODS: Nineteen males and 13 females aged between 14 and 60 years with confirmed diagnosis of PNH were treated with danazol (4, danazol + cyclosporine (7, cyclosporine (1, and prednisolone + danazol (20. Response to these interventions was assessed regularly. Danazol was added to cyclosporine in patients with aplastic bone marrow after 3 months of cyclocporine use only unless the former therapy was successful. Four patients with aplastic marrow received only danazol because they had renal insufficiency at presentation. Patients were evaluated with regular complete blood count and routine liver and renal function tests. RESULTS: One patient responded to cyclosporine only. Thirteen of 32 patients (40% had complete response, 12/32 patients (37% had partial response leading to freedom from red cell transfusion, and 2/32 (7% had no response. Five patients (16% died due to thrombosis or hemorrhage within 3 months of therapy before their response to therapy could be assessed. The median period of review of the cases was 4 years and 6 months. CONCLUSION: Danazol is a useful addition to PNH therapy both in combination with cyclosporine for hypoplastic PNH and with prednisolone for other forms of PNH, and this therapy could be a good alternative where eculizumab and anti-lymphocyte globulin cannot be used for various reasons.

  15. Cerebellopontine angle medulloblastoma with extensive nodularity in a child: case report and review of the literature.

    Science.gov (United States)

    Noiphithak, Raywat; Yindeedej, Vich; Thamwongskul, Chatchai

    2017-05-01

    Cerebellar medulloblastomas (MBs) are one of the most common posterior fossa tumors in children but rarely occur in the cerebellopontine angle (CPA). Only 39 cases of CPA MBs were reported in the literature, and most of them were classic and desmoplastic MBs. A 22-month-old girl presented with progressive cerebellar ataxia. Magnetic resonance imaging showed a large tumor in the right CPA and obstructive hydrocephalus. Surgical resection was performed and achieved total tumor removal. Microscopic examination and immunohistochemical staining revealed the diagnosis of MB with extensive nodularity. The patient recovered from her symptoms during follow-up and was transferred for adjuvant chemotherapy. MB should be considered as a differential diagnosis of a lesion in the CPA. The treatment and outcome of CPA MBs are similar to cerebellar MBs.

  16. Cavernous angioma after chemotherapy for desmoplastic/nodular medulloblastoma associated with anhidrotic ectodermal dysplasia.

    Science.gov (United States)

    Yamasaki, Fumiyuki; Takayasu, Takeshi; Nosaka, Ryo; Kawaguchi, Hiroshi; Sugiyama, Kazuhiko; Kobayashi, Masao; Kurisu, Kaoru

    2016-02-01

    While cavernous angioma (CVA) after cranial irradiation has been documented, its development after high-dose chemotherapy with autologous peripheral blood stem cell transplantation (PBSCT) has not. We present a patient with desmoplastic/nodular medulloblastoma (DNMB) associated with anhidrotic ectodermal dysplasia (AED) who developed CVA 2 years after high-dose chemotherapy and PBSCT. A 1-year-old boy with ingravescent vomiting was admitted to our institute. He presented with a large head, a depressed nasal bridge, low-set ears, thick lips with peg-shaped teeth, hypohidrosis, sparse hair, thin atrophic skin, scaly dermatitis with frontal bossing, and a bulging anterior fontanel. Neuroradiological examination revealed multiple cerebellar masses with heterogeneous enhancement and speckled calcifications and severe obstructive hydrocephalus. The histological diagnosis of surgical specimens was DNMB, and he underwent postoperative multiple-drug chemotherapy with autologous PBSCT. The outcome was favorable and he did not undergo radiotherapy. After 2 years, intracranial hemorrhage was detected at his regular radiological check-up and he again underwent surgery. The histological diagnosis was CVA. To our knowledge, this is the first report of AED-associated DNMB and CVA.

  17. Surgical excision versus imiquimod 5% cream for nodular and superficial basal-cell carcinoma (SINS): a multicentre, non-inferiority, randomised controlled trial.

    Science.gov (United States)

    Bath-Hextall, Fiona; Ozolins, Mara; Armstrong, Sarah J; Colver, Graham B; Perkins, William; Miller, Paul S J; Williams, Hywel C

    2014-01-01

    Basal-cell carcinoma is the most common form of skin cancer and its incidence is increasing worldwide. We aimed to assess the effectiveness of imiquimod cream versus surgical excision in patients with low-risk basal-cell carcinoma. We did a multicentre, parallel-group, pragmatic, non-inferiority, randomised controlled trial at 12 centres in the UK, in which patients were recruited between June 19, 2003, and Feb 22, 2007, with 3 year follow-up from June 26, 2006, to May 26, 2010. Participants of any age were eligible if they had histologically confirmed primary nodular or superficial basal-cell carcinoma at low-risk sites. We excluded patients with morphoeic or recurrent basal-cell carcinoma and those with Gorlin syndrome. Participants were randomly assigned (1:1) via computer-generated blocked randomisation, stratified by centre and tumour type, to receive either imiquimod 5% cream once daily for 6 weeks (superficial) or 12 weeks (nodular), or surgical excision with a 4 mm margin. The randomisation sequence was concealed from study investigators. Because of the nature of the interventions, masking of participants was not possible and masking of outcome assessors was only partly possible. The trial statistician was masked to allocation until all analyses had been done. The primary outcome was the proportion of participants with clinical success, defined as absence of initial treatment failure or signs of recurrence at 3 years from start of treatment. We used a prespecified non-inferiority margin of a relative risk (RR) of 0.87. Analysis was by a modified intention-to-treat population and per protocol. This study is registered as an International Standard Randomised Controlled Trial (ISRCTN48755084), and with ClinicalTrials.gov, number NCT00066872. 501 participants were randomly assigned to the imiquimod group (n=254) or the surgical excision group (n=247). At year 3, 401 (80%) patients were included in the modified intention-to-treat group. At 3 years, 178 (84%) of

  18. Treatment of paroxysmal nocturnal hemoglobinuria

    Directory of Open Access Journals (Sweden)

    I. A. Lisukov

    2014-07-01

    Full Text Available Paroxysmal nocturnal hemoglobinuria (PNH is a rare, life‑threatening clonal hematological disorder caused by an acquired mutation in the phosphatidylinositol glucan (PIG-A gene. PNH is characterized by chronic intravascular hemolysis, marrow failure, thrombophilia and other severe clinical syndromes. Until recently, the treatment of PNH has been symptomatic with blood transfusions, anticoagulation and supplementation with folic acid or iron. The only potentially curative treatment is allogeneic stem cell transplantation, but this has severe complications with high mortality rates. A new targeted treatment strategy is the inhibition of the terminal complement cascade with anti‑C5 monoclonal antibody (eculizumab. Eculizumab has shown significant efficacy in controlling of intravascular hemolysis resulting in improving quality of life and survival.

  19. Treatment of paroxysmal nocturnal hemoglobinuria

    Directory of Open Access Journals (Sweden)

    I. A. Lisukov

    2012-01-01

    Full Text Available Paroxysmal nocturnal hemoglobinuria (PNH is a rare, life‑threatening clonal hematological disorder caused by an acquired mutation in the phosphatidylinositol glucan (PIG-A gene. PNH is characterized by chronic intravascular hemolysis, marrow failure, thrombophilia and other severe clinical syndromes. Until recently, the treatment of PNH has been symptomatic with blood transfusions, anticoagulation and supplementation with folic acid or iron. The only potentially curative treatment is allogeneic stem cell transplantation, but this has severe complications with high mortality rates. A new targeted treatment strategy is the inhibition of the terminal complement cascade with anti‑C5 monoclonal antibody (eculizumab. Eculizumab has shown significant efficacy in controlling of intravascular hemolysis resulting in improving quality of life and survival.

  20. Pathogenesis of nodular goiter and its implications for surgical management

    International Nuclear Information System (INIS)

    Teuscher, J.; Peter, H.J.; Gerber, H.; Berchtold, R.; Studer, H.

    1988-01-01

    Despite sufficient iodine supply, goiter continues to be of considerable surgical significance in formerly endemic countries. It now appears that iodine deficiency and increased thyrotropin stimulation are not the only causes of goiter. Xenotransplantation of human thyroid tissue onto nude mice allowed study of the regulation of growth and function in human goiter tissue. Grafts of human thyroid tissue growing in nude mice could be shown to react to endogenous mouse thyrotropic stimulation and suppression. 131 I autoradiographs of xenotransplanted goiter tissue showed as marked a heterogeneity as did the original goitrous tissue prior to transplantation. There was no firm correlation between the morphologic appearance of a follicle and its iodine metabolism. Scintigraphically cold and hot goiter tissue differed from each other quantitatively but not qualitatively; i.e., both hot and cold tissue were composed of metabolically active and nonactive follicles. Iodine organification was not completely suppressible by thyroxine treatment; this indicates autonomous functional activity. The distribution of proliferating tissue labeled by 3 H-thymidine did not parallel the distribution of functionally active tissue labelled by 131 I. Thyroxine treatment did not completely inhibit 3 H-thymidine incorporation, indicating autonomous growth. Thus, our pathogenetic concept of goiter formation is based on three mainstays: (1) goiter heterogeneity, (2) autonomy of growth and function, and (3) dissociation of growth and function in human goiter tissue. Thus, the surgeon dealing with goiter ought to remove all pathologically altered tissue, i.e., nodular tissue, irrespective of its appearance on scintiscans

  1. MSCT diagnosis and differential diagnosis of hepatic focal nodular hyperplasia

    International Nuclear Information System (INIS)

    Zhang Haitao; Xu Qinsha; Chen Yutang; Song Yupiao

    2011-01-01

    Objective: To evaluate the MSCT findings of focal nodular hyperplasia (FNH) of the liver, and to improve the diagnostic standard. Methods: The MSCT findings were analyzed retrospectively in 25 patients pathologically approved with FNH. All patients underwent 16 detector row spiral CT scanning with and without contrast enhancement. Results: 28 lesions were discovered by CT, with single lesion showed in 22 cases, 2 lesions showed in 3 cases. All lesions showed a solitary nodules or lobulated mass in the liver. On plain CT, FNH showed equal or slightly low density. In the arterial phase, 27 lesions were vigorously and homogeneously enhanced, except the central scars with CT value of 99∼149HU and the mean CT-number of 124 HU. 1 lesion ws enhanced inter-homogeneously. Tortuous and enlarged arteries were seen at the center or periphery in 8 of the 28 lesions. In the portal venous phase, 16 lesions remained slightly hyperdense, 8 lesions turned to isodense, and 1 lesion turned to slightly hypodense. The typical central scar was showed in 8 lesions and 2 lesions showed delayed enhancement. 21 cases were correctly diagnosed by MSCT, with the diagnostic accuracy of 84.0%. Conclusion: MSCT scanning can fully show the pathologic and the blood supplying characteristics of FNH. The typical FNH can be easily diagnosed by CT, while the atypical cases should be differentiated from hepatocellular carcinoma, hemangioma of liver, liver cell adenoma and fibrolamellar hepatocarcinoma. (authors)

  2. Canine sterile nodular panniculitis: a retrospective study of 39 dogs.

    Science.gov (United States)

    Contreary, Caitlin L; Outerbridge, Catherine A; Affolter, Verena K; Kass, Philip H; White, Stephen D

    2015-12-01

    Canine sterile nodular panniculitis (SNP) is an inflammatory disease of the panniculus that is typically managed with immunomodulatory or immunosuppressive treatments. It has been reported to be a cutaneous marker of an underlying systemic disease. To assess the presence or absence of concurrent systemic diseases associated with canine SNP and to document breed predispositions. Thirty nine dogs presented to a veterinary teaching hospital from 1990 to 2012 which met inclusion criteria. Inclusion in this retrospective study required a diagnosis of SNP via histopathological analysis and negative special stains for infectious organisms. Breed distributions of affected dogs were compared to all other dogs examined at this hospital during the study period. Correlations between the histological pattern of panniculitis and the histological presence of dermatitis, clinical presentation of lesions, dog breed and therapeutic outcomes were assessed. Australian shepherd dogs, Brittany spaniels, Dalmatians, Pomeranians and Chihuahuas were significantly over-represented, but correlations between inflammatory patterns of panniculitis and other histological and clinical factors were not identified. Based on the information available in medical records, 32 dogs (82.1%) had no concurrent systemic diseases identified. Four dogs had concurrent polyarthritis, which may be related to SNP through unknown mechanisms. This study identified several novel breed predilections for SNP; it failed to find any clear correlations with associated systemic diseases other than polyarthritis. The histological inflammatory pattern of SNP does not predict therapeutic outcome. © 2015 ESVD and ACVD.

  3. Oral Nodular Lesions in Patients with Sjögren's Syndrome: Unusual Oral Implications of a Systemic Disorder.

    Science.gov (United States)

    Pinheiro, Juliana Barchelli; Tirapelli, Camila; Silva, Claudia Helena Lovato da; Komesu, Marilena Chinali; Petean, Flávio Calil; Louzada Junior, Paulo; León, Jorge Esquiche; Motta, Ana Carolina Fragoso

    2017-01-01

    Sjögren's syndrome (SS) is a systemic chronic autoimmune disorder affecting the lacrimal and salivary glands. SS may manifest as primary SS (pSS) or secondary SS (sSS), the latter occurring in the context of another autoimmune disorder. In both cases, the dry eyes and mouth affect the patient's quality of life. Late complications may include blindness, dental tissue destruction, oral candidiasis and lymphoma. This paper reports two cases of SS, each of them presenting unusual oral nodular lesion diagnosed as relapsed MALT lymphoma and mucocele. The importance of the diagnosis, treatment and management of the oral lesions by a dentist during the care of SS patients is emphasized, as the oral manifestations of SS may compromise the patient's quality of life.

  4. Necrotizing Fasciitis in Paroxysmal Nocturnal Hemoglobinuria

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    Pusem Patir

    2015-01-01

    Full Text Available Paroxysmal nocturnal hemoglobinuria (PNH is a rare, progressive, and life-threatening hematopoietic stem cell disorder characterized by complement-mediated intravascular hemolysis and a prothrombotic state. Patients with PNH might have slightly increased risk of infections due to complement-associated defects subsequent to CD59 deficiency. Here, we report a rare case of a 65-year-old male patient with necrotic ulcers on both legs, where the recognition of pancytopenia and microthrombi led to the diagnosis of PNH based on FLAER (FLuorescent AERolysin flow cytometric analysis. He was subsequently started on eculizumab therapy, with starting and maintenance doses set as per drug labelling. Progression of the patient’s leg ulcers during follow-up, with fulminant tissue destruction, purulent discharge, and necrotic patches, led to a later diagnosis of necrotizing fasciitis due to Pseudomonas aeruginosa and Klebsiella pneumonia infection. Courses of broad-spectrum antibiotics, surgical debridement, and superficial skin grafting were applied with successful effect during ongoing eculizumab therapy. This case highlights the point that it is important to maintain treatment of underlying disorders such as PNH in the presence of life-threatening infections like NF.

  5. Cerebral stroke in a teenage girl with paroxysmal nocturnal hemoglobinuria

    Directory of Open Access Journals (Sweden)

    Francesco Gervasi

    2017-06-01

    Full Text Available We report a case of paroxysmal nocturnal hemoglobinuria (PNH in a 14 year-old girl presenting a cerebral arterial thrombosis. The initial diagnosis was carential anemia due to menarche following identification of slight macrocytic anemia, leucopenia and mild thrombocytopenia at routine blood analysis. The child was eventually referred to a children’s hospital after the onset of progressive fatigue, anorexia and paleness. Severe anemia (hemoglobin 6 g/dL with negative Coombs test, mild leucopenia (white blood cells 4.9×109/L and thrombocytopenia (platelets 97×109/L and high values of lactate dehydrogenase (2855 U/L were identified; a packed red cells transfusion was administered. Her condition worsened and she subsequently presented complete right hemiplegia, aphasia and coma; magnetic resonance imaging revealed a massive ischemic lesion. A diagnosis of PNH was eventually made following high sensitivity flow cytometry, which identified a PNH clone (CD66b negative equal to 93.7% of granulocytes. Fast recovery from neurologic and hematological problems occurred in response to anticoagulant therapy and intravenous therapy with eculizumab. We are convinced that PNH should be included in the differential diagnosis of children presenting with cytopenia.

  6. O Trabalho em Saúde e a Política de Humanização do Sistema Único de Saúde (SUS: o ponto de vista da atividade industriosa na análise do trabalho

    Directory of Open Access Journals (Sweden)

    Ana Rita Castro TRAJANO

    2012-12-01

    Full Text Available Objetivou-se discutir a perspectiva ergológica do trabalho em articulação com a Política Nacional de Humanização (PNH como referencial de análise do processo de trabalho em saúde. Partiu-se de abordagens do processo de trabalho no campo da Saúde Coletiva, em que se toma a PNH como parte deste, procurandose estabelecer interlocuções com a ergologia como postura ético-epistemológica, tanto em práticas de pesquisa como em processos de gestão do trabalho. Em seguida, discutiu-se a emergência da PNH, como política pública do SUS, criada pelo Ministério da Saúde em 2003, quando foi discutido o sentido de humanização como ‘conceito-experiência’. Como parte essencial deste artigo, procurouse abordar noções e conceitos elaborados pela ergologia, no esforço de construir referenciais de análise da atividade industriosa em saúde, contribuindo para as discussões atuais sobre a PNH, como ‘política pública e a produção do comum’.

  7. Efficacy of percutaneous microwave coagulation therapy for nodular hepatic tumor : a preliminary study

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Seon Hee; Bang, Sun Woo; Baek, Seung Il; Kim, Ho Kyun [Inje Univ. College of Medicine, Seoul (Korea, Republic of); Kim, Jeong Seok [Dongguk Univ. College of Medicine, Seoul (Korea, Republic of)

    2001-04-01

    Percutaneous microwave coagulation therapy (PMCT) uses a new energy source, microwave, in the treatment of solid neoplasms. We evaluated the efficacy of PMCT for nodular hepatic tumors in nine patients. Between December 1998 and June 1999, we performed PMCT in six patients with hepatocelluar carcinoma (HCC), two with one and three metastatic nodules each from colon cancer, and one with adenomatous hyperplasia. Four patients were female and five were male, and their age ranged between 44 and 71 (mean, 58.8) years. Under sonographic guidance a 14-gauge guiding needle was inserted percutaneously toward the lesion, and within it a needle electrode was precisely positioned. Microwave 2450MHz in frequency and with 60 or 80-watt emission was generated for 80{approx}90 seconds. We evaluated the ultrasound findings obtained during the procedure, pre-PMCT and follow-up CT images, changes in tumor marker(AFP or CA19-9) levels and the results of liver function tests, and complications arising during the six-month period following PMCT. Immediately after microwave emission, characteristic hyperechogenicity appeared on the real-time sonogram. Two patients with HCC underwent CT before PMCT, and typical enhancement during the arterial phase and washout during the portovenous phase was observed. In one patient, two metastatic lesions from colon cancer showed delayed enhancement on pre-PMCT CT. Initial follow-up CT, performed between I and 4 weeks after the PMCT procedure, showed that eight lesions-including two HCCs which were highly enhanced on CT before PMCT-showed no contrast enhancement, and three others showed delayed enhancement. Two of the eight lesions which showed no contrast enhancement at initial follow-up CT were markedly decreased in size (from 2.9 and 4.0 cm to 1.0 and 2.0 cm, respectively) at subsequent follow-up 3 months and 6 months later, respectively. One of the three lesions showing delayed enhancement had increased in size from 4.1 to 5.5 cm at subsequent

  8. Contrast-enhanced ultrasound of focal nodular hyperplasia: a matter of size

    Energy Technology Data Exchange (ETDEWEB)

    Bertin, Caroline [Hopital Beaujon APHP, Radiology Department, Clichy (France); Egels, Sophie; Huynh-Charlier, Isabelle [Hopital Pitie-Salpetriere APHP, Radiology Department, Paris (France); Wagner, Mathilde [Hopital Pitie-Salpetriere APHP, Radiology Department, Paris (France); Universite Paris Diderot, INSERM, UMR 1149, Laboratoire IPMA, Centre de Recherche sur l' Inflammation, Faculte de Medecine X Bichat, Paris (France); Vilgrain, Valerie [Hopital Beaujon APHP, Radiology Department, Clichy (France); Universite Paris Diderot, INSERM, UMR 1149, Laboratoire IPMA, Centre de Recherche sur l' Inflammation, Faculte de Medecine X Bichat, Paris (France); Lucidarme, Olivier [Hopital Pitie-Salpetriere APHP, Radiology Department, Paris (France); Sorbonne Universites, UPMC Univ Paris 06, CNRS UMR 7371, INSERM UMRS 1146, Laboratoire d' Imagerie Biomedicale, Paris (France); Groupe Hospitalier Pitie-Salpetriere - Charles Foix, Service de Radiologie Polyvalente et Oncologique, Paris (France)

    2014-10-15

    To assess the contrast-enhanced ultrasound (CEUS) frequencies of centrifugal enhancement, spoke-wheel sign and central scar in focal nodular hyperplasia (FNH) as a function of lesion size. Ninety-four FNHs were retrospectively reviewed to assess their largest diameter and enhancement pattern, including centrifugal enhancement from one central artery, spoke-wheel sign, diffuse or centripetal enhancement, central scar and late-phase washout. Mean FNH-lesion size was 3.7 ± 2.1 cm. Only 43.6 % of FNHs had centrifugal enhancement, with a spoke-wheel pattern (23.4 %) or without (20.2 %), while 56.4 % showed diffuse or centripetal enhancement. Centrifugal enhancement was observed in 73.9 % of FNHs ≤3.1 cm and 14.6 % of FNHs >3.1 cm (P < 10{sup -4}). Size and frequency of centrifugal enhancement were negatively correlated (r = -0.57, P < 10{sup -4}). The spoke-wheel pattern was also seen more frequently in smaller (37 %) than in larger FNHs (10.4 %) (P < 10{sup -3}). Late-phase washout was described in 5.3 % of FNHs and was not size-dependent. Lesions with a central scar were larger than those without, respectively, 5.7 ± 1.7 and 3.6 ± 2.0 cm (P = 0.012). Typical centrifugal enhancement yielding a confident FNH diagnosis is seen significantly more frequently when the lesion is ≤3.1 cm. (orig.)

  9. Limited value of delayed radiothallium image in the diagnosis of nodular goiter

    International Nuclear Information System (INIS)

    Misaki, Takashi; Yagi, Kanji; Dokoh, Shigeharu; Koh, Toshikiyo; Shimbo, Shin-ichiro

    1991-01-01

    To test whether delayed thallium image can improve the diagnostic accuracy of thyroid tumor, we have reviewed 35 patients with cold nodules of proven histopathology. Early and delayed images were taken 10-20 min and 3-4 hr postinjection, respectively. In this study, a thyroid nodule was interpreted as malignant if it had normal or increased uptake on early scan and more residual radioactivity than paranodular tissue on delayed scan. In the eighteen patients who had carcinoma, a disappointing 44% false negative rate was observed. No significant difference was seen between retention-positive and negative cases in tumor size or histological type of cancer. Nor could any demographic bias explain the low sensitivity. Of the six false negatives, macroscopic cystic degeneration was seen in two cases, but tumors in the other four were grossly solid. Considering the histological heterogeneity often seen within a thyroid tumor, the portion with increased retention of radiothallium may be too small to be detected in the false negative cases. Furthermore, 3 false positive readings were obtained in 17 patients with benign conditions. We conclude that the contribution of the delayed thallium image was quite limited in predicting or ruling out malignancy in nodular goiters. (author)

  10. The prevalence of gastric heterotopia of the proximal esophagus is underestimated, but preneoplasia is rare - correlation with Barrett's esophagus.

    Science.gov (United States)

    Peitz, Ulrich; Vieth, Michael; Evert, Matthias; Arand, Jovana; Roessner, Albert; Malfertheiner, Peter

    2017-07-12

    The previously reported prevalence of gastric heterotopia in the cervical esophagus, also termed inlet patch (IP), varies substantially, ranging from 0.18 to 14%. Regarding cases with adenocarcinoma within IP, some experts recommend to routinely obtain biopsies from IP for histopathology. Another concern is the reported relation to Barrett's esophagus. The objectives of the study were to prospectively determine the prevalence of IP and of preneoplasia within IP, and to investigate the association between IP and Barrett's esophagus. 372 consecutive patients undergoing esophagogastroduodenoscopy were carefully searched for the presence of IP. Biopsies for histopathology were targeted to the IP, columnar metaplasia of the lower esophagus, gastric corpus and antrum. Different definitions of Barrett's esophagus were tested for an association with IP. At least one IP was endoscopically identified in 53 patients (14.5%). Histopathology, performed in 46 patients, confirmed columnar epithelium in 87% of cases, which essentially presented corpus and/or cardia-type mucosa. Intestinal metaplasia was detected in two cases, but no neoplasia. A previously reported association of IP with Barrett's esophagus was weak, statistically significant only when short segments of cardia-type mucosa of the lower esophagus were included in the definition of Barrett's esophagus. The prevalence of IP seems to be underestimated, but preneoplasia within IP is rare, which does not support the recommendation to regularly obtain biopsies for histopathology. Biopsies should be targeted to any irregularities within the heterotopic mucosa. The correlation of IP with Barrett's esophagus hints to a partly common pathogenesis.

  11. Paroxysmal nocturnal hemoglobinuria: a case report of MR, CT findings

    International Nuclear Information System (INIS)

    Yang, Ik; Chung, Soo Young; Park, Hai Jung; Lee Yul; Chun, Rho Won; Noh, Jung Woo

    1995-01-01

    Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired disease involving multiple hematopoietic cell lines. Characteristics of PNH are intrinsic hemolytic anemia, iron deficiency anemia and venous thrombosis. We report a case of PNH with characterostoc MR and CT findings. The signal intensity of renal cortex was lower than that of medulla on both T1-and T2-weighted MR imaging. On T2 weighted MR images, the liver showed very low signal intensity but the signal intensity of the spleen was normal. On precontrast CT the attenuation of renal cortex was higher than that of renal medulla and the attenuation of liver was higher than that of the spleen. These findings of MR imaging and CT were the result from the deposition of hemosiderin in the cells of proximal convoluted tubules and transfusional hemosiderosis of liver

  12. Contrast-enhanced ultrasonographic spoke-wheel sign in hepatic focal nodular hyperplasia

    International Nuclear Information System (INIS)

    Yen, Y.-H.; Wang, J.-H.; Lu, S.-N.; Chen, T.-Y.; Changchien, C.-S.; Chen, C.-H.; Hung, C.-H.; Lee, C.-M.

    2006-01-01

    Background: To determine the utility of contrast-enhanced ultrasonography (CEUS) in assessing hepatic tumors with central feeding arteries found by color/power Doppler ultrasonograophy (CDUS/PDUS). Methods: We prospectively studied 37 hepatic tumors (34 patients), with a mean size of 2.9 cm and each having a central feeding artery, by CDUS/PDUS. The CEUS was performed with a galactose-based microbubble contrast agent. The detection of a spoke-wheel sign was interpreted as evidence of focal nodular hyperplasia (FNH). All patients underwent tumor biopsies or surgical resection. Results: CEUS showed a central feeding artery with a spoke-wheel sign in 36 tumors, including 34 FNHs and 2 hepatocellular carcinomas. The remaining tumor was demonstrated to be FNH despite the absence of a spoke-wheel sign as detected by CEUS. The sensitivity of the spoke-wheel sign or central scar for FNH was 97.1% (34/35), 40% (14/35), 28.6% (10/35), 50% (8/16) and 0% (0/15) for CEUS, CDUS/PDUS, dynamic computed tomography (CT) or magnetic resonance imaging (MRI), hepatic angiography and liver scintigraphy, respectively. The two hepatocellular carcinomas showed scirrhous changes histologically. Conclusions: CEUS is more sensitive than CDUS/PDUS, dynamic CT, MRI, hepatic angiography and liver scintigraphy in the detection of the spoke-wheel sign or central scar in FNH. Scirrhous hepatocellular carcinoma should be included in the differential diagnosis for liver tumors with spoke-wheel sign detected by CEUS

  13. Calorimetric analysis of heating and cooling process of nodular cast iron

    Directory of Open Access Journals (Sweden)

    Bińczyk F.

    2007-01-01

    Full Text Available The study presents the results of investigations of the thermal effects which take place during heating and cooling of samples of the nodular graphite cast iron taken from the stepped test casting of the wall thicknesses amounting to 5, 10, 15 and 20 mm. For investigations, a differential scanning calorimeter, type Multi HTC S60, was used. During heating, three endothermic effects related with pearlite decomposition, phase transformation α → γ, and carbon dissolution in austenite were observed on a DSC diagram. During cooling, two exothermic effects related with phase transformation γ→ α and pearlite formation were observed to consecutively take place on a DSC diagram. The values of the enthalpy of these processes differ and depend on the initial microstructure of the examined samples. The metallic matrix in 5 mm sample after the process of heating and cooling changes totally in favour of ferrite. The same effect, though less advanced in intensity, takes place in 10 mm sample, while in 15 and 20 mm samples the matrix constitution remains unchanged. The higher is the content of ferrite in samples, the stronger is the endothermic effect of the α → γ transformation and the weaker is the endothermic effect related with carbon dissolution in austenite. The total of the endothermic effects (heating is reduced, while that of the exothermic effects (cooling increases along with the increasing thickness of walls in a stepped test casting, from which samples for the investigations were taken.

  14. [Rapidly-growing nodular pseudoangiomatous stromal hyperplasia of the breast: case report].

    Science.gov (United States)

    Elıyatkin, Nuket; Karasu, Başak; Selek, Elif; Keçecı, Yavuz; Postaci, Hakan

    2011-01-01

    Pseudoangiomatous stromal hyperplasia is a benign proliferative lesion of the mammary stroma that rarely presents as a localized mass. Pseudoangiomatous stromal hyperplasia is characterized by a dense, collagenous proliferation of the mammary stroma, associated with capillary-like spaces. Pseudoangiomatous stromal hyperplasia can be mistaken with fibroadenoma on radiological examination or with low-grade angiosarcoma on histological examination. Its main importance is its distinction from angiosarcoma. The presented case was a 40-year-old woman who was admitted with a rapidly growing breast tumor. Physical examination revealed an elastic-firm, well-defined, mobile and painless mass in her right breast. Mammograms revealed a 6.7 x 3.7 cm, lobulated, well-circumscribed mass in her right breast but no calcification. Sonographic examination showed a well-defined and homogenous mass, not including any cyst. Based on these findings, a provisional diagnosis of fibroadenoma was made. Considering the rapid growth history of the mass, tumor excision was performed. The excised tumor was well demarcated and had a smooth external surface. Histological examination revealed the tumor to be composed of markedly increased fibrous stroma and scattered epithelial components (cystic dilatation of the ducts, blunt duct adenosis). The fibrous stroma contained numerous anastomosing slit-like spaces. Isolated spindle cells appeared intermittently at the margins of the spaces resembled endothelial cells. Immunohistochemical staining showed that the spindle cells were positive for CD34 and negative for Factor VIII-related antigen. The lesion was diagnosed as nodular pseudoangiomatous stromal hyperplasia.

  15. Contrast-enhanced ultrasonographic spoke-wheel sign in hepatic focal nodular hyperplasia

    Energy Technology Data Exchange (ETDEWEB)

    Yen, Y.-H. [Division of Hepato-Gastroenterology, Department of Internal Medicine, Kaohsiung Chang Gung Memorial Hospital, 123 Ta-Pei Road, Kaohsiung 833, Taiwan (China); Wang, J.-H. [Division of Hepato-Gastroenterology, Department of Internal Medicine, Kaohsiung Chang Gung Memorial Hospital, 123 Ta-Pei Road, Kaohsiung 833, Taiwan (China)]. E-mail: wajing@adm.cgmh.org.tw; Lu, S.-N. [Division of Hepato-Gastroenterology, Department of Internal Medicine, Kaohsiung Chang Gung Memorial Hospital, 123 Ta-Pei Road, Kaohsiung 833, Taiwan (China); Chen, T.-Y. [Department of Radiology, Kaohsiung Chang Gung Memorial Hospital, Kaohsiung, College of Medicine, Chang Gung University, Taoyuan, Taiwan (China); Changchien, C.-S. [Division of Hepato-Gastroenterology, Department of Internal Medicine, Kaohsiung Chang Gung Memorial Hospital, 123 Ta-Pei Road, Kaohsiung 833, Taiwan (China); Chen, C.-H. [Division of Hepato-Gastroenterology, Department of Internal Medicine, Kaohsiung Chang Gung Memorial Hospital, 123 Ta-Pei Road, Kaohsiung 833, Taiwan (China); Hung, C.-H. [Division of Hepato-Gastroenterology, Department of Internal Medicine, Kaohsiung Chang Gung Memorial Hospital, 123 Ta-Pei Road, Kaohsiung 833, Taiwan (China); Lee, C.-M. [Division of Hepato-Gastroenterology, Department of Internal Medicine, Kaohsiung Chang Gung Memorial Hospital, 123 Ta-Pei Road, Kaohsiung 833, Taiwan (China)

    2006-12-15

    Background: To determine the utility of contrast-enhanced ultrasonography (CEUS) in assessing hepatic tumors with central feeding arteries found by color/power Doppler ultrasonograophy (CDUS/PDUS). Methods: We prospectively studied 37 hepatic tumors (34 patients), with a mean size of 2.9 cm and each having a central feeding artery, by CDUS/PDUS. The CEUS was performed with a galactose-based microbubble contrast agent. The detection of a spoke-wheel sign was interpreted as evidence of focal nodular hyperplasia (FNH). All patients underwent tumor biopsies or surgical resection. Results: CEUS showed a central feeding artery with a spoke-wheel sign in 36 tumors, including 34 FNHs and 2 hepatocellular carcinomas. The remaining tumor was demonstrated to be FNH despite the absence of a spoke-wheel sign as detected by CEUS. The sensitivity of the spoke-wheel sign or central scar for FNH was 97.1% (34/35), 40% (14/35), 28.6% (10/35), 50% (8/16) and 0% (0/15) for CEUS, CDUS/PDUS, dynamic computed tomography (CT) or magnetic resonance imaging (MRI), hepatic angiography and liver scintigraphy, respectively. The two hepatocellular carcinomas showed scirrhous changes histologically. Conclusions: CEUS is more sensitive than CDUS/PDUS, dynamic CT, MRI, hepatic angiography and liver scintigraphy in the detection of the spoke-wheel sign or central scar in FNH. Scirrhous hepatocellular carcinoma should be included in the differential diagnosis for liver tumors with spoke-wheel sign detected by CEUS.

  16. GATE based Monte Carlo simulation of planar scintigraphy to estimate the nodular dose in radioiodine therapy for autonomous thyroid adenoma

    International Nuclear Information System (INIS)

    Hammes, Jochen; Schmidt, Matthias; Schicha, Harald; Eschner, Wolfgang; Pietrzyk, Uwe; Wuppertal Univ.

    2011-01-01

    The recommended target dose in radioiodine therapy of solitary hyperfunctioning thyroid nodules is 300-400 Gy and therefore higher than in other radiotherapies. This is due to the fact that an unknown, yet significant portion of the activity is stored in extranodular areas but is neglected in the calculatory dosimetry. We investigate the feasibility of determining the ratio of nodular and extranodular activity concentrations (uptakes) from post-therapeutically acquired planar scintigrams with Monte Carlo simulations in GATE. The geometry of a gamma camera with a high energy collimator was emulated in GATE (Version 5). A geometrical thyroid-neck phantom (GP) and the ICRP reference voxel phantoms 'Adult Female' (AF, 16 ml thyroid) and 'Adult Male' (AM, 19 ml thyroid) were used as source regions. Nodules of 1 ml and 3 ml volume were placed in the phantoms. For each phantom and each nodule 200 scintigraphic acquisitions were simulated. Uptake ratios of nodule and rest of thyroid ranging from 1 to 20 could be created by summation. Quantitative image analysis was performed by investigating the number of simulated counts in regions of interest (ROIs). ROIs were created by perpendicular projection of the phantom onto the camera plane to avoid a user dependant bias. The ratio of count densities in ROIs over the nodule and over the contralateral lobe, which should be least affected by nodular activity, was taken to be the best available measure for the uptake ratios. However, the predefined uptake ratios are underestimated by these count density ratios: For an uptake ratio of 20 the count ratios range from 4.5 (AF, 1 ml nodule) to 15.3 (AM, 3 ml nodule). Furthermore, the contralateral ROI is more strongly affected by nodular activity than expected: For an uptake ratio of 20 between nodule and rest of thyroid up to 29% of total counts in the ROI over the contralateral lobe are caused by decays in the nodule (AF 3 ml). In the case of the 1 ml nodules this effect is smaller: 9

  17. Perineal nodular indurations ("accessory testicles") in cyclists. Fine needle aspiration cytologic and pathologic findings in two cases.

    Science.gov (United States)

    Vuong, P N; Camuzard, P; Schoonaert, M F

    1988-01-01

    The cytologic and histologic findings from two cases of perineal nodular indurations observed in two cyclists are reported. These lesions, also referred to as "accessory testicles" or "third testicle" or "ischial hygromas" of cyclists, consist of a localized aseptic area of necrosis with pseudocyst formation involving connective tissue in the superficial fascia of the perineum. These histologic findings, which were seen in the subsequent surgical specimens in these two cases, were reflected in the fine needle aspiration findings. The aspirates contained few cellular elements, mainly a few vacuolated histiocytes, against a background of fibrinous material. These indurations, which develop as a result of repeated, chronic microtrauma to the perineum impressed by the vibration of the saddle of the bicycle, constitute an authentic handicap for the professional cyclist and are a contraindication to cycling for amateur cyclists.

  18. A simple evaluation of numbers of asbestos bodies in bronchoalveolar lavage fluid under light microscopy. Analysis of 35 pulmonary nodular lesions

    International Nuclear Information System (INIS)

    Kawahara, Kunimitsu; Kawasumi, Hiromi; Nagano, Teruaki; Sasada, Shinji; Okamoto, Norio

    2008-01-01

    More than 1 asbestos body (AB) per ml of bronchoalveolar lavage fluid (BALF) under light microscopy was defined as AB positive (ABP) and suggests an occupational asbestos exposure. We microscopically evaluated the AB number per one ml of BALF, which we defined as the AB concentration (ABC), using bronchoalveolar lavage (BAL) cytocentrifuge slides obtained from 35 patients having pulmonary nodular lesions (20 carcinoma and 15 nonneoplastic disease) and examined the correlation between ABC and clinicopathological data including findings on Helical computed tomography scan (HCTS) and occupational history of asbestos exposure (OHAE). BAL was performed by the standard technique without removing mucous with a gauze filter. AB was microscopically defined as a structure consisting of a core of transparent asbestos surrounded by an iron-protein coat. Twenty of 35 patients were ABP (ABP rate; 57%) and ABC ranged from 0 to 207.98/ml (mean ABC; 11.33/ml). Mean ABC was significantly higher in patients with OHAE (15.04/ml) compared to that in patients without OHAE (3.23/ml). Twenty-two of 35 patients (63%) lacked abnormality on HCTS and among these, 12 patients (55%) were ABP. In 20 pulmonary carcinoma patients, the ABP rate was 85% and ABC ranged from 0 to 31.1/ml (Mean ABC; 2.99/ml). The ABP rate of pulmonary carcinoma patients was 40% (8 patients) and among these, 5 patients (63%) did not show any abnormality on HCTS. In conclusion, our method was simple and useful and should be applied to patients with pulmonary nodular lesions and OHAE, even if there are no abnormalities on HCTS. (author)

  19. Variations in the size of focal nodular hyperplasia on magnetic resonance imaging.

    Science.gov (United States)

    Ramírez-Fuentes, C; Martí-Bonmatí, L; Torregrosa, A; Del Val, A; Martínez, C

    2013-01-01

    To evaluate the changes in the size of focal nodular hyperplasia (FNH) during long-term magnetic resonance imaging (MRI) follow-up. We reviewed 44 FNHs in 30 patients studied with MRI with at least two MRI studies at least 12 months apart. We measured the largest diameter of the lesion (inmm) in contrast-enhanced axial images and calculated the percentage of variation as the difference between the maximum diameter in the follow-up and the maximum diameter in the initial study. We defined significant variation in size as variation greater than 20%. We also analyzed predisposing hormonal factors. The mean interval between the two imaging studies was 35±2 months (range: 12-94). Most lesions (80%) remained stable during follow-up. Only 9 of the 44 lesions (20%) showed a significant variation in diameter: 7 (16%) decreased in size and 2 (4%) increased, with variations that reached the double of the initial size. The change in size was not related to pregnancy, menopause, or the use of birth control pills or corticoids. Changes in the size of FNHs during follow-up are relatively common and should not lead to a change in the diagnosis. These variations in size seem to be independent of hormonal factors that are considered to predispose. Copyright © 2011 SERAM. Published by Elsevier Espana. All rights reserved.

  20. Nodular fasciitis: correlation of MRI findings and histopathology

    International Nuclear Information System (INIS)

    Wang, X.L.; De Schepper, A.M.A.; Vanhoenacker, F.; Gielen, J.; De Raeve, H.; Aparisi, F.; Rausin, L.; Somville, J.

    2002-01-01

    Objective: To compare the histopathology of nodular fasciitis (NF) with the magnetic resonance imaging (MRI) findings in order to evaluate the basis of the MR signal characteristics. Design and patients: Ten patients with NF, nine females and 1 male, with an age ranging from 13 to 58 years (mean 26.8 years) were studied. MRI findings, available in all 10 patients, were compared with the histopathology in nine patients, and an area-to-area comparative study of the whole specimen section histopathology and MRI was performed in two patients. Results: On the basis of an excisional biopsy or resection specimen, the nine lesions were classified into myxoid (n=4), cellular (n=3) and fibrous (n=2) subtypes. Four myxoid lesions with a subcutaneous location showed a homogeneous SI comparable with muscle on T1-weighted images, high SI on T2-weighted images, and had homogeneous enhancement. One cellular lesion presented with homogeneous, slightly higher SI than muscle on T1-weighted images and inhomogeneous, high SI on T2-weighted images. Alcian blue stain of the whole specimen section revealed the lesion had two parts corresponding to different enhancement patterns on MRI. The blue-stained myxoid part showed markedly diffuse enhancement, while the non-stained cystic space had only peripheral enhancement. Two other cellular lesions had the same appearance on both T1- and T2-weighted images and showed inhomogeneous, diffuse enhancement. One fibrous subtype lesion presented with inhomogeneous, overall slightly higher SI than muscle on T1-weighted images, lower SI at the periphery and high SI in the center on STIR images and only peripheral enhancement. Microscopy and CD-31 staining of the lesion showed more extracellular matrix, with poor vascularity in the center and more collagenous matrix with higher vascularity at the periphery. Conclusion: Although similar findings were found in some lesions, the large histologic variability of NF hampers the definition of a prototype of NF