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Sample records for dutch paraganglioma family

  1. SDHAF2 mutations in familial and sporadic paraganglioma and phaeochromocytoma.

    Science.gov (United States)

    Bayley, Jean-Pierre; Kunst, Henricus P M; Cascon, Alberto; Sampietro, Maria Lourdes; Gaal, José; Korpershoek, Esther; Hinojar-Gutierrez, Adolfo; Timmers, Henri J L M; Hoefsloot, Lies H; Hermsen, Mario A; Suárez, Carlos; Hussain, A Karim; Vriends, Annette H J T; Hes, Frederik J; Jansen, Jeroen C; Tops, Carli M; Corssmit, Eleonora P; de Knijff, Peter; Lenders, Jacques W M; Cremers, Cor W R J; Devilee, Peter; Dinjens, Winand N M; de Krijger, Ronald R; Robledo, Mercedes

    2010-04-01

    Paragangliomas and phaeochromocytomas are neuroendocrine tumours associated frequently with germline mutations of SDHD, SDHC, and SDHB. Previous studies have shown the imprinted SDHAF2 gene to be mutated in a large Dutch kindred with paragangliomas. We aimed to identify SDHAF2 mutation carriers, assess the clinical genetic significance of SDHAF2, and describe the associated clinical phenotype. We undertook a multicentre study in Spain and The Netherlands in 443 apparently sporadic patients with paragangliomas and phaeochromocytomas who did not have mutations in SDHD, SDHC, or SDHB. We analysed DNA of 315 patients for germline mutations of SDHAF2; a subset (n=200) was investigated for gross gene deletions. DNA from a group of 128 tumours was studied for somatic mutations. We also examined a Spanish family with head and neck paragangliomas with a young age of onset for the presence of SDHAF2 mutations, undertook haplotype analysis in this kindred, and assessed their clinical phenotype. We did not identify any germline or somatic mutations of SDHAF2, and no gross gene deletions were noted in the subset of apparently sporadic patients analysed. Investigation of the Spanish family identified a pathogenic germline DNA mutation of SDHAF2, 232G-->A (Gly78Arg), identical to the Dutch kindred. SDHAF2 mutations do not have an important role in phaeochromocytoma and are rare in head and neck paraganglioma. Identification of a second family with the Gly78Arg mutation suggests that this is a crucial residue for the function of SDHAF2. We conclude that SDHAF2 mutation analysis is justified in very young patients with isolated head and neck paraganglioma without mutations in SDHD, SDHC, or SDHB, and in individuals with familial antecedents who are negative for mutations in all other risk genes. Dutch Cancer Society, European Union 6th Framework Program, Fondo Investigaciones Sanitarias, Fundación Mutua Madrileña, and Red Temática de Investigación Cooperativa en Cáncer. 2010

  2. SDHAF2 mutations in familial and sporadic paraganglioma and phaeochromocytoma.

    NARCIS (Netherlands)

    Bayley, J.P.M.; Kunst, H.P.M.; Cascon, A.; Sampietro, M.L.; Gaal, J.; Korpershoek, E.; Hinojar-Gutierrez, A.; Timmers, H.J.L.M.; Hoefsloot, L.H.; Hermsen, M.A.; Suarez, C.; Hussain, A.K.; Vriends, A.H.; Hes, F.J.; Jansen, J.C.; Tops, C.M.; Corssmit, E.P.; Knijff, P. de; Lenders, J.W.M.; Cremers, C.W.R.J.; Devilee, P.; Dinjens, W.N.; Krijger, R.R. de; Robledo, M.

    2010-01-01

    BACKGROUND: Paragangliomas and phaeochromocytomas are neuroendocrine tumours associated frequently with germline mutations of SDHD, SDHC, and SDHB. Previous studies have shown the imprinted SDHAF2 gene to be mutated in a large Dutch kindred with paragangliomas. We aimed to identify SDHAF2 mutation

  3. The first Dutch SDHB founder deletion in paraganglioma – pheochromocytoma patients

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    Devilee Peter

    2009-04-01

    Full Text Available Abstract Background Germline mutations of the tumor suppressor genes SDHB, SDHC and SDHD play a major role in hereditary paraganglioma and pheochromocytoma. These three genes encode subunits of succinate dehydrogenase (SDH, the mitochondrial tricarboxylic acid cycle enzyme and complex II component of the electron transport chain. The majority of variants of the SDH genes are missense and nonsense mutations. To date few large deletions of the SDH genes have been described. Methods We carried out gene deletion scanning using MLPA in 126 patients negative for point mutations in the SDH genes. We then proceeded to the molecular characterization of deletions, mapping breakpoints in each patient and used haplotype analysis to determine whether the deletions are due to a mutation hotspot or if a common haplotype indicated a single founder mutation. Results A novel deletion of exon 3 of the SDHB gene was identified in nine apparently unrelated Dutch patients. An identical 7905 bp deletion, c.201-4429_287-933del, was found in all patients, resulting in a frameshift and a predicted truncated protein, p.Cys68HisfsX21. Haplotype analysis demonstrated a common haplotype at the SDHB locus. Index patients presented with pheochromocytoma, extra-adrenal PGL and HN-PGL. A lack of family history was seen in seven of the nine cases. Conclusion The identical exon 3 deletions and common haplotype in nine patients indicates that this mutation is the first Dutch SDHB founder mutation. The predominantly non-familial presentation of these patients strongly suggests reduced penetrance. In this small series HN-PGL occurs as frequently as pheochromocytoma and extra-adrenal PGL.

  4. Paragangliomas and paraganglioma syndromes

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    Boedeker, Carsten Christof

    2012-01-01

    Paragangliomas are rare tumors of neural crest origin. They are benign in the majority of cases and are characterized by a strong vascularisation. In the head and neck region they most commonly occur as carotid body tumors. Jugulotympanic and especially vagal paragangliomas are seen less frequently. Complete surgical resection represents the only curative treatment option even though resection of locally advanced tumors regularly results in lesions of the lower cranial nerves and major vessels. Appoximately 30% of all head and neck paragangliomas (HNPs) are hereditary and associated with different tumor syndromes. The paraganglioma syndromes 1, 3 and 4 (PGL 1, 3 and 4) make up the majority of those familial cases. PGL 1 is associated with mutations of the succinate dehydrogenase subunit D (SDHD) gene, PGL 3 is caused by SDHC and PGL 4 by SDHB gene mutations. Multiple HNPs and the occurance of HNPs together with pheochromocytomas are seen in SDHD as well as SDHB mutation carriers. In patients with SDHB mutations the risk for the development of malignant paraganglial tumors is significantly higher compared to SDHC and SDHD patients as well as patients with sporadic tumors. SDHC mutation carriers almost exclusively present with benign HNP that are unifocal in the majority of cases. The role of transmission is autosomal dominant for all three symptoms. Interestingly, there is a “parent-of-origin-dependent-inheritance” in subjects with SDHD gene mutations. This means that the disease phenotype may only become present if the mutation is inherited through the paternal line. We recommend screening for mutations of the genes SDHB, SDHC and SDHD in patients with HNPs. Certain clinical parameters can help to set up the order in which the three genes should be tested. PMID:22558053

  5. Mutation analysis of SDHB and SDHC: novel germline mutations in sporadic head and neck paraganglioma and familial paraganglioma and/or pheochromocytoma

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    Wong Nora

    2006-01-01

    Full Text Available Abstract Background Germline mutations of the SDHD, SDHB and SDHC genes, encoding three of the four subunits of succinate dehydrogenase, are a major cause of hereditary paraganglioma and pheochromocytoma, and demonstrate that these genes are classic tumor suppressors. Succinate dehydrogenase is a heterotetrameric protein complex and a component of both the Krebs cycle and the mitochondrial respiratory chain (succinate:ubiquinone oxidoreductase or complex II. Methods Using conformation sensitive gel electrophoresis (CSGE and direct DNA sequencing to analyse genomic DNA from peripheral blood lymphocytes, here we describe the mutation analysis of the SDHB and SDHC genes in 37 patients with sporadic (i.e. no known family history head and neck paraganglioma and five pheochromocytoma and/or paraganglioma families. Results Two sporadic patients were found to have a SDHB splice site mutation in intron 4, c.423+1G>A, which produces a mis-spliced transcript with a 54 nucleotide deletion, resulting in an 18 amino acid in-frame deletion. A third patient was found to carry the c.214C>T (p.Arg72Cys missense mutation in exon 4 of SDHC, which is situated in a highly conserved protein motif that constitutes the quinone-binding site of the succinate: ubiquinone oxidoreductase (SQR complex in E. coli. Together with our previous results, we found 27 germline mutations of SDH genes in 95 cases (28% of sporadic head and neck paraganglioma. In addition all index patients of five families showing hereditary pheochromocytoma-paraganglioma were found to carry germline mutations of SDHB: four of which were novel, c.343C>T (p.Arg115X, c.141G>A (p.Trp47X, c.281G>A (p.Arg94Lys, and c.653G>C (p.Trp218Ser, and one reported previously, c.136C>T, p.Arg46X. Conclusion In conclusion, these data indicate that germline mutations of SDHB and SDHC play a minor role in sporadic head and neck paraganglioma and further underline the importance of germline SDHB mutations in cases of

  6. Paraganglioma Anatomy

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    ... e.g. -historical Searches are case-insensitive Paraganglioma Anatomy Add to My Pictures View /Download : Small: 648x576 ... View Download Large: 2700x2400 View Download Title: Paraganglioma Anatomy Description: Paraganglioma of the head and neck; drawing ...

  7. Chromaffin paraganglioma

    International Nuclear Information System (INIS)

    Ocampo, Jose; Trejos, Javier; Morales, Mariano; Encalada, Jose; Canizares, Manuela

    2006-01-01

    We present the case of a 37 year old male patient with a left sub maxillary region tumoral mass, submitted to exploratory cervicotomy; the pathologic diagnosis, was Limpho-hemangioma and lymphatic node with reactive follicular hyperplasia. The patient had a tumoral recidive, with a big ganglial tumor that occupied the left side of triangle of the neck, anterior and posterior, with larynx and trachea displacement; after tracheostomy and surgical biopsy, the pathologic diagnosis was chromaffin paraganglioma (carotid glomus quemodectoma). Paragangliomas are uncommon tumors, these are located at abdomen, chest, head and neck; computer tomography and nuclear magnetic resonance determine their tumoral extension. Surgery and radiotherapy are specific treatment. (The author)

  8. Pheochromocytoma and paraganglioma

    International Nuclear Information System (INIS)

    Reckova, M.

    2013-01-01

    Pheochromocytoma and paraganglioma are rare tumors that originate from the cells of neural crest. Despite a wide variety of clinical features, the main remains the hypertension. Most of pheochromocytoma (FEO) and paraganglioma (PGL) represent sporadic tumors but about 20-30% of these tumors are familial. Besides standard imagining techniques, functional diagnostics plays important role, as well. The definitive treatment is surgical therapy, however in inoperable cases, radionuclide therapeutic methods are used. Systemic chemotherapy has only limited efficacy and, currently, targeted therapeutic approaches are studied. External-beam radiotherapy is used in palliative settings. The most frequent causes of morbidity and mortality are cardiovascular events, such as sudden death, heart attack, heart failure and cerebrovascular events. Early diagnosis is very important not just for prevention of disease dissemination, but for the prevention of possible complications, as well. (author)

  9. Exploring the social origins of Dutch mothers' ideal family lives

    NARCIS (Netherlands)

    Ruitenberg, J.; de Beer, P.

    2014-01-01

    This paper examines how Dutch mothers’ ideal family lives (traditional, adaptive or egalitarian) have been formed in interaction with other people, an assumption that is central to socialization theories. With nominal regression analysis of data from a representative survey in 2010 among 935 Dutch

  10. Pheochromocytomas and secreting paragangliomas

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    Gimenez-Roqueplo Anne-Paule

    2006-12-01

    Full Text Available Abstract Catecholamine-producing tumors may arise in the adrenal medulla (pheochromocytomas or in extraadrenal chromaffin cells (secreting paragangliomas. Their prevalence is about 0.1% in patients with hypertension and 4% in patients with a fortuitously discovered adrenal mass. An increase in the production of catecholamines causes symptoms (mainly headaches, palpitations and excess sweating and signs (mainly hypertension, weight loss and diabetes reflecting the effects of epinephrine and norepinephrine on α- and β-adrenergic receptors. Catecholamine-producing tumors mimic paroxysmal conditions with hypertension and/or cardiac rhythm disorders, including panic attacks, in which sympathetic activation linked to anxiety reproduces the same signs and symptoms. These tumors may be sporadic or part of any of several genetic diseases: familial pheochromocytoma-paraganglioma syndromes, multiple endocrine neoplasia type 2, neurofibromatosis 1 and von Hippel-Lindau disease. Familial cases are diagnosed earlier and are more frequently bilateral and recurring than sporadic cases. The most specific and sensitive diagnostic test for the tumor is the determination of plasma or urinary metanephrines. The tumor can be located by computed tomography, magnetic resonance imaging and metaiodobenzylguanidine scintigraphy. Treatment requires resection of the tumor, generally by laparoscopic surgery. About 10% of tumors are malignant either at first operation or during follow-up, malignancy being diagnosed by the presence of lymph node, visceral or bone metastases. Recurrences and malignancy are more frequent in cases with large or extraadrenal tumors. Patients, especially those with familial or extraadrenal tumors, should be followed-up indefinitely.

  11. A cryptic BAP1 splice mutation in a family with uveal and cutaneous melanoma, and paraganglioma

    DEFF Research Database (Denmark)

    Wadt, K.; Choi, J.; Chung, J.Y.

    2012-01-01

    Inactivating germ line BRCA1-associated protein-1 (BAP1) mutations have recently been reported in families with uveal or cutaneous malignant melanoma (UMM, CMM), mesothelioma, and meningioma. Although apparently predisposing to a wide range of tumors, the exact tumor spectrum associated with germ...

  12. Paraganglioma de cuerpo carotídeo: reporte de un caso clínico con correlación familiar Carotid body paraganglioma: clinical case report with family correlation

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    José Martín Toranzo Fernández

    2011-06-01

    Full Text Available Los paragangliomas de cabeza y cuello son neoplasias infrecuentes que se originan de un tejido paraganglionar del grupo braquiomérco que está ligado al desarrollo de los arcos branquiales. No son cromafines, ya que no secretan catecolaminas¹. De estos paragangliomas, el que se presenta con más frecuencia es el del cuerpo carotídeo, que se origina a partir de los paraganglios localizados en la adventicia de la bifurcación del cuerpo carotídeo².The head and neck paragangliomas are infrequent neoplases that originate from a paraganglionar tissue of the brachiomeric group that are linked to the development of the branchial arches; they are not cromafins since they do not secrete catecholamines¹. The most frequent one appears in the carotid body that originates from the paraganglions located in the adventitia of the carotid body's bifurcation².

  13. Evaluation of management of desmoid tumours associated with familial adenomatous polyposis in Dutch patients

    NARCIS (Netherlands)

    Nieuwenhuis, M. H.; Mathus-Vliegen, E. M.; Baeten, C. G.; Nagengast, F. M.; van der Bijl, J.; van Dalsen, A. D.; Kleibeuker, J. H.; Dekker, E.; Langers, A. M.; Vecht, J.; Peters, F. T.; van Dam, R.; van Gemert, W. G.; Stuifbergen, W. N.; Schouten, W. R.; Gelderblom, H.; Vasen, H. F. A.

    2011-01-01

    BACKGROUND: The optimal treatment of desmoid tumours is controversial. We evaluated desmoid management in Dutch familial adenomatous polyposis (FAP) patients. METHODS: Seventy-eight FAP patients with desmoids were identified from the Dutch Polyposis Registry. Data on desmoid morphology, management,

  14. Disentangling an Invisible Trade : state interventions in Dutch and Dutch-Curaçaoan single-mother families

    NARCIS (Netherlands)

    Verhallen, T.L.

    2015-01-01

    The doctoral thesis sheds light on the interactional and institutional processes through which child welfare and child protection practices are delivered to fifteen Dutch and fifteen Dutch-Curaçaoan single-mother families with multiple problems in the Netherlands in order to reveal structures of

  15. Family Stress in Dutch Families with Motor Impaired Toddlers: A Survey in a Dutch Rehabilitation Centre

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    Tibosch, Marijke

    2008-01-01

    The study investigated the relationship between family stress and child characteristics in families with motor impaired toddlers. Families of 20 children between 2 1/2 and 5 years old with motor impairments, who visit a therapeutic toddler class in a rehabilitation centre, participated. The study was carried out in the Netherlands. Family stress…

  16. Parental Work Demands and Parent-Child, Family, and Couple Leisure in Dutch Families: What Gives?

    Science.gov (United States)

    Roeters, Anne; Treas, Judith K.

    2011-01-01

    This study uses data on 898 Dutch couples with minor children to examine whether parental work demands are related differently to one-on-one parent-child, family, and couple leisure activities. The authors presume that the impact of working hours and work arrangements is smaller on activities that are prioritized highly and that are easier and…

  17. Characterization and metabolic synthetic lethal testing in a new model of SDH-loss familial pheochromocytoma and paraganglioma.

    Science.gov (United States)

    Smestad, John; Hamidi, Oksana; Wang, Lin; Holte, Molly Nelson; Khazal, Fatimah Al; Erber, Luke; Chen, Yue; Maher, L James

    2018-01-19

    Succinate dehydrogenase (SDH)-loss pheochromocytoma and paraganglioma (PPGL) are tumors driven by metabolic derangement. SDH loss leads to accumulation of intracellular succinate, which competitively inhibits dioxygenase enzymes, causing activation of pseudohypoxic signaling and hypermethylation of histones and DNA. The mechanisms by which these alterations lead to tumorigenesis are unclear, however. In an effort to fundamentally understand how SDH loss reprograms cell biology, we developed an immortalized mouse embryonic fibroblast cell line with conditional disruption of Sdhc and characterize the kinetics of Sdhc gene rearrangement, SDHC protein loss, succinate accumulation, and the resultant hypoproliferative phenotype. We further perform global transcriptomic, epigenomic, and proteomic characterization of changes resulting from SDHC loss, identifying specific perturbations at each biological level. We compare the observed patterns of epigenomic derangement to another previously-described immortalized mouse chromaffin cell model of SDHB loss, and compare both models to human SDH-loss tumors. Finally, we perform analysis of SDHC synthetic lethality with lactate dehydrogenase A (LDHA) and pyruvate carboxylase (PCX), which are important for regeneration of NAD+ and aspartate biosynthesis, respectively. Our data show that SDH-loss cells are selectively vulnerable to LDH genetic knock-down or chemical inhibition, suggesting that LDH inhibition may be an effective therapeutic strategy for SDH-loss PPGL.

  18. Family group conferencing in Dutch child welfare : Which families are most likely to organize a family group conference?

    NARCIS (Netherlands)

    Dijkstra, S.; Creemers, H.E.; Asscher, J.J.; Deković, M.; Stams, G.J.J.M.

    2017-01-01

    Aim of the present study was to identify which families involved in child welfare are willing to organize a Family Group conference (FGc; phase 1) and which are most likely to complete a conference (phase 2). Data were used of a Dutch randomized controlled trial (N =229). First, the proportion of

  19. Family group conferencing in Dutch child welfare : Which families are most likely to organize a family group conference?

    NARCIS (Netherlands)

    Dijkstra, Sharon; Creemers, Hanneke E.; Asscher, Jessica J.; Dekovic, Maja; Stams, Geert Jan J. M.

    2017-01-01

    Aim of the present study was to identify which families involved in child welfare are willing to organize a Family Group conference (FGc; phase 1) and which are most likely to complete a conference (phase 2). Data were used of a Dutch randomized controlled trial (N = 229). First, the proportion of

  20. Giant paraganglioma in

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    Alka Gupta

    2017-07-01

    Full Text Available Paraganglioma is a rare neuroendocrine catecholamine producing tumour in childhood which arises outside the adrenal medulla. We present a 12 year old girl with giant paraganglioma with severe hypertension and end organ damage. Diagnosis was confirmed with 24 h urinary Vanillymandelic Acid (VMA and CT scan. Preoperative blood pressure was controlled with intravenous nitroprusside, and oral prazosin, amlodepine, labetalol and metoprolol. General anaesthesia with epidural analgesia was given. Intra operative blood pressure rise was managed with infusion of nitriglycerine (NTG, esmolol, nitroprusside and propofol.

  1. Imaging and management of head and neck paragangliomas

    Energy Technology Data Exchange (ETDEWEB)

    Berg, Rene van den [Leiden University Medical Center, Department of Radiology, Leiden (Netherlands)

    2005-07-01

    Paragangliomas of the head and neck are highly vascular lesions originating from paraganglionic tissue located at the carotid bifurcation (carotid body tumors), along the vagus nerve (vagal paragangliomas), and in the jugular fossa and tympanic cavity (jugulotympanic paragangliomas). Diagnostic imaging can be considered in two clinical situations: (1) patients who present with clinical symptoms suggestive of a paraganglioma, and (2) individuals from families with hereditary paragangliomas. It is not only necessary to detect and characterize the lesion, but also to study the presence of multiplicity. For these purposes, MR imaging, and especially 3D TOF MRA, is the modality of choice. CT scanning is especially useful to show destruction of the temporal bone. Angiography in combination with embolization will mainly be used prior to surgical resection, but can also be used for diagnostic purposes when the diagnosis is not yet clear. Many parameters play a role in the decision to treat of which multifocality and impairment of cranial nerves are the most important. The primary therapeutic option for paragangliomas is complete excision of tumor with preservation of vital neurovascular structures. Resection however, should be balanced against a more conservative ''wait and scan'' policy or palliative treatments such as radiotherapy. (orig.)

  2. Simultaneous adrenal pheochromocytoma and carotid body paraganglioma in a woman

    Energy Technology Data Exchange (ETDEWEB)

    Han, Eun Ji; Lee, Sang Hoon; Song, In Uk; Chung, Yong An; Maeng, Lee So [The Catholic Univ. of Korea, Incheon (Korea, Republic of)

    2012-03-15

    Simultaneous occurrence of carotid body tumor and pheochromocytoma is rare. Most pheochromocytomas have grown on adrenal medulla, but some of the pheochromocytoma patients have multifocal paragangliomas arising from extraaderenal tissues. Pheochromocytomas and paragangliomas occur as sporadic tumors or they can be associated with several hereditary syndromes such as (1) multiple endocrine neoplasia type 2 (MEN 2), (2) Von Hippel Lindau disease (VHL) and (3) neurofibromatosis type 1 as an unusual genetic cause of pheochromocytomas. Genetic testing is recommended for patients with an apparently sporadic pheochromocytoma under the age of 20 years with a family history or features suggestive of hereditary pheochromocytoma or for patients with sympathetic paragangliomas. For individuals who do not meet these criteria, genetic testing is optional. Discovery of pheochromocytoma or paraganglioma in a patient should lead to a careful search to rule out multifocal lesions and/or hereditary syndromes. The diagnosis of pheochromocytoma and paraganglioma is made by biochemical testing, and imaging is done to localize the tumor for surgical planning. F 18 FDG PET has proved to be an effective tool in the localization of pheochromocytomas and paragangliomas.

  3. Changing Places: A Cross-Language Perspective on Frequency and Family Size in Dutch and Hebrew

    Science.gov (United States)

    Moscoso del Prado Martin, Fermin; Deutsch, Avital; Frost, Ram; Schreuder, Robert; De Jong, Nivja H.; Baayen, R. Harald

    2005-01-01

    This study uses the morphological family size effect as a tool for exploring the degree of isomorphism in the networks of morphologically related words in the Hebrew and Dutch mental lexicon. Hebrew and Dutch are genetically unrelated, and they structure their morphologically complex words in very different ways. Two visual lexical decision…

  4. Malignant vagal paraganglioma

    DEFF Research Database (Denmark)

    Carlsen, Camilla S; Godballe, Christian; Krogdahl, Annelise S

    2003-01-01

    Approximately 20 cases of malignant vagal paragangliomas (MVP)have been reported in English literature. Malignancy is based on the presence of metastases. A careful preoperative evaluation is necessary to detect multicentricity and/or significant production of catecholamines. A new case of MVP...... treated with embolization and surgery is presented and the literature discussed. It is concluded, that preoperative embolization followed by radical surgical resection is a rational treatment of patients with unilateral MVP....

  5. Paraganglioma of Prostatic Origin

    Directory of Open Access Journals (Sweden)

    B. Padilla-Fernández

    2012-01-01

    Full Text Available Introduction Paragangliomas are usually benign tumors arising from chromaffin cells located outside the adrenal gland. Prostatic paraganglioma is an unusual entity in adult patients, with only 10 cases reported in the medical literature. Case Report A 34-year-old male with a history of chronic prostatitis consulted for perineal pain. On digital rectal examination the prostate was enlarged and firm, without nodules. The PSA level was 0.8 ng/mL and the catecholamines in the urine were elevated. On ultrasound a retrovesical 9 cm mass of undetermined origin measuring was present. A PET-CT scan showed a pelvic lesion measuring 9 cm with moderate increase in glucidic metabolism localized in the area of the prostate. A biopsy of the prostate revealed a neuroendocrine tumor, possibly a prostatic paraganglioma. A body scintigraphy with MIBG I-123 ruled out the presence of metastases or multifocal tumor. A radical prostatectomy with excision of the pelvic mass was performed under adrenergic blockade. One year after surgery the patient is asymptomatic and disease free. Discussion/Conclusions Prostatic paraganglioma is a rare, usually benign tumor, which should be considered in the differential diagnosis of prostate tumors in young males. Its diagnosis is based on the determination of catecholamine in blood and 24-hour urine and in imaging studies principally scintigraphy with MIBG I-123. Diagnostic confirmation is by histopathological study. The treatment consists of radical resection under adrenergic blockade and volume expansion. Given the limited number of cases reported, it is difficult to establish prognostic factors. Malignancy is defined by clinical criteria, and requires life long follow-up.

  6. A legacy of tinnitus: multiple head and neck paragangliomas

    Directory of Open Access Journals (Sweden)

    Jeremy J. Turner

    2009-12-01

    Full Text Available We describe the case of a patient who presented with a right-sided glomus jugulare tumor and bilateral glomus vagale tumors. These proved to be nonmalignant paragangliomas on histopathological analysis. Genetic analysis revealed a germline heterozygous missense mutation (Pro81Leu in the succinate dehydrogenase subunit D (SDHD gene. We discuss the clinical presentations of the familial paraganglioma syndrome type 1, which is caused by mutations in SDHD, and the implications for the clinical diagnosis and care of such patients.

  7. Stages of Pheochromocytoma and Paraganglioma

    Science.gov (United States)

    ... foods high in tyramine (such as red wine, chocolate, and cheese). Tests that examine the blood and ... cause signs or symptoms are treated with drug therapy. Drug therapy begins when pheochromocytoma or paraganglioma is ...

  8. Living arrangements of migrant and Dutch young adults: the family influence disentangled

    NARCIS (Netherlands)

    de Valk, H.A.G.; Billari, F.

    2007-01-01

    The purpose of this study was to examine the impact of the family of origin on whether migrant and Dutch young adults live in the parental home. Using a sample of 1,678 young adults aged between 15 and 30 years from 847 families with five different ethnic backgrounds, we identified patterns of

  9. Genomewide scan identifies susceptibility locus for dyslexia on Xq27 in an extended Dutch family.

    NARCIS (Netherlands)

    Kovel, C.G.F. de; Hol, F.A.; Heister, J.G.A.M.; Willemen, J.J.H.T.; Sandkuijl, L.A.; Franke, B.; Padberg, G.W.A.M.

    2004-01-01

    CONTEXT: Dyslexia is a common disorder with a strong genetic component, but despite significant research effort, the aetiology is still largely unknown. OBJECTIVE: To identify loci contributing to dyslexia risk. METHODS: This was a genomewide linkage analysis in a single large family. Dutch families

  10. Family Business and Corporate Social Responsibility in a Sample of Dutch Firms

    NARCIS (Netherlands)

    prof. Uhlaner, L.M.; van Goor-Balk, H.J.M.; Masurel, E.

    2004-01-01

    This paper explores corporate social responsibility in family businesses. In particular, the research investigates family businesses in relation to a wide variety of constituent or stakeholder groups. It reports the preliminary results of focused interviews with 42 small and medium-sized Dutch

  11. Family business and corporate social responsibility in a samle of Dutch firms

    NARCIS (Netherlands)

    prof. Uhlaner, L.M.; van Goor-Balk, H.J.M.; Masurel, E.

    2004-01-01

    This paper explores corporate social responsibility in family businesses. In particular, the research investigates family businesses in relation to a wide variety of constituent or stakeholder groups. It reports the preliminary results of focused interviews with 42 small and medium-sized Dutch

  12. Adaptive Strategies, Gender Ideology, and Work-Family Balance among Dutch Dual Earners

    Science.gov (United States)

    Wierda-Boer, Hilde H.; Gerris, Jan R. M.; Vermulst, Ad A.

    2008-01-01

    Using questionnaire data on 149 Dutch dual-earner couples with young children participating in the European Famwork study, we examine how adaptive strategies and gender ideology relate to parents' perceived success in balancing work and family. Path analysis indicates that some adaptive strategies may harm individuals' work-family balance,…

  13. Family communication as strategy in diabetes prevention: an observational study in families with Dutch and Surinamese South-Asian ancestry.

    Science.gov (United States)

    van Esch, Suzanne C M; Cornel, Martina C; Geelhoed-Duijvestijn, Petronella H L M; Snoek, Frank J

    2012-04-01

    To explore the possibility of utilizing family communication as a diabetes prevention strategy, specifically targeting high-risk families with South-Asian ancestry in The Netherlands. In a cross-sectional study, type 2 diabetes patients from Dutch (n=311) and Surinamese South-Asian (n=157) origin filled in a questionnaire assessing socio-demographic characteristics, beliefs and concerns about familial diabetes risk, primary prevention, and diabetes-related family communication. Discussing diabetes is regarded acceptable in most families. Especially Surinamese South-Asian patients (68%) seemed motivated to convey risk messages to their relatives; they reported a higher risk perception and expressed more concern than Dutch patients. While 40% in both groups thought relatives are able to prevent developing diabetes, 46% in Dutch and 33% in Surinamese South-Asian patients were unsure. Promoting family communication appears a feasible strategy in diabetes prevention in high-risk (Surinamese South-Asian) families. Health care providers should address patients' concern and emphasize opportunities for prevention. Findings favor training of clinicians in utilizing a family approach as prevention strategy. Patients (particularly Surinamese South-Asians) are in need of professional help in the process of family risk disclosure. (Online) Educational tools should be made available at which patients can refer their relatives. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

  14. Multipoint linkage analysis and homogeneity tests in 15 Dutch X-linked retinitis pigmentosa families

    NARCIS (Netherlands)

    Bergen, A. A.; van den Born, L. I.; Schuurman, E. J.; Pinckers, A. J.; van Ommen, G. J.; Bleekers-Wagemakers, E. M.; Sandkuijl, L. A.

    1995-01-01

    Linkage analysis and homogeneity tests were carried out in 15 Dutch families segregating X-linked retinitis pigmentosa (X L R P). The study included segregation data for eight polymorphic DNA markers from the short arm of the human X chromosome. The results of both multipoint linkage analysis in

  15. Management of children's urinary tract infections in Dutch family practice: a cohort study.

    NARCIS (Netherlands)

    Harmsen, M.; Wensing, M.J.P.; Braspenning, J.C.C.; Wolters, R.J.; Wouden, J.C. van der; Grol, R.P.T.M.

    2007-01-01

    BACKGROUND: Optimal clinical management of childhood urinary tract infections (UTI) potentiates long-term positive health effects. Insight into the quality of care in Dutch family practices for UTIs was limited, particularly regarding observation periods of more than a year. Our aim was to describe

  16. Management of children's urinary tract infections in Dutch family practice: A cohort study

    NARCIS (Netherlands)

    M. Harmsen (Mirjam); M.E. Wensing (Michel); J.C.C. Braspenning (Jozé); R.J. Wolters (René); J.C. van der Wouden (Hans); R.P.T.M. Grol (Richard)

    2007-01-01

    textabstractBackground. Optimal clinical management of childhood urinary tract infections (UTI) potentiates long-term positive health effects. Insight into the quality of care in Dutch family practices for UTIs was limited, particularly regarding observation periods of more than a year. Our aim was

  17. Dutch Children at Family Risk of Dyslexia: Precursors, Reading Development, and Parental Effects

    Science.gov (United States)

    van Bergen, Elsje; de Jong, Peter F.; Regtvoort, Anne; Oort, Frans; van Otterloo, Sandra; van der Leij, Aryan

    2011-01-01

    The study concerns reading development and its precursors in a transparent orthography. Dutch children differing in family risk for dyslexia were followed from kindergarten through fifth grade. In fifth grade, at-risk dyslexic (n = 22), at-risk non-dyslexic (n = 45), and control children (n = 12) were distinguished. In kindergarten, the at-risk…

  18. Familial clustering of major depression and anxiety disorders in Australian and Dutch twins and siblings

    NARCIS (Netherlands)

    Middeldorp, C.M.; Birley, A.J.; Cath, D.C.; Gillespie, N.A.; Willemsen, G.; Statham, D.J.; Geus, de J.C.N.; Andrews, J.G.; Dyck, van R.; Beem, A.L.; Sullivan, P.F.; Martin, N.G.; Boomsma, D.I.

    2005-01-01

    The aim of this study was to investigate familial influences and their dependence on sex for panic disorder and/or agoraphobia, social phobia, generalized anxiety disorder and major depression. Data from Australian (N = 2287) and Dutch (N = 1185) twins and siblings who were selected for a linkage

  19. A Dutch kindred with familial amyloidotic polyneuropathy associated with the transthyretin Cys 114 mutant

    NARCIS (Netherlands)

    Haagsma, EB; Post, JG; DeJager, AEJ; Nikkels, PGJ; Hamel, BCJ; Hazenberg, BPC

    A Dutch kindred with transthyretin Cys 114 related familial amyloidotic polyneuropathy is reported. The finding of early involvement of the heart is of great concern and of special relevance to the optimal timing for liver transplantation. To date, the variant had only been reported to be present in

  20. Secondary traumatic stress and secondary posttraumatic growth in a sample of Dutch police family liaison officers

    NARCIS (Netherlands)

    Kunst, Maarten; Saan, M.C.; Bollen, Lidewij; Kuijpers, Karlijn

    2017-01-01

    This study investigated secondary traumatic stress (STS) and secondary posttraumatic growth (SPG) in a sample of Dutch police family liaison officers (N = 224). Our study had two aims: (a) to identify potential risk and protective factors for STS and (b) to investigate the association between STS

  1. Intervention in Lower-class Surinam-Dutch Families : Effects on Mothers and Infants

    NARCIS (Netherlands)

    Riksen-Walraven, J Marianne; Meij, Johannes Th; Hubbard, Frans O.; Zevalkink, D.J.

    1996-01-01

    Thirty-seven Surinam-Dutch lower-class families with a one-year-old child participated in "Instapje", a parent-focused home-based intervention programme. The intervention was devised to improve quality of parental support to the child on four behavioural dimensions: supportive presence, respect for

  2. Pathways into adulthood. A comparative study on family life transitions among migrant and Dutch youth

    NARCIS (Netherlands)

    Valk, H.A.G. de

    2006-01-01

    The ethnic composition of the Dutch population has changed considerably in the past decades. Nowadays a substantial proportion of youth in the Netherlands has a migrant background. This study focuses on how these young adults make the transition to adulthood in the family domain. What preferences

  3. The impact of home literacy and family factors on screen media use among Dutch Preteens

    NARCIS (Netherlands)

    Duursma, Elisabeth; Meijer, Anna; Bot, de Kees

    This study examined preteens' screen media use and potential differences in media use by child and family demographics among 1464 Dutch preteens. The results demonstrated that watching TV is still a very popular activity among children. However, other electronic media are also popular within this

  4. Bilateral hereditary micro-epiphyseal dysplasia : clinical and genetic analysis of a Dutch family

    NARCIS (Netherlands)

    Mostert, Adrianus Klazinus

    2003-01-01

    This thesis is based upon a study of a Dutch family with a unique skeletal dysplasia first described by Elsbach in 1959. Because of the presence of microepiphyses, he called this disorder bilateral hereditary micro-epiphyseal dysplasia (BHMED) and distinguished it from more common multiple

  5. The link between family orientation, strategy, and innovation in Dutch SMEs : A longitudinal study

    NARCIS (Netherlands)

    Uhlaner, L.M.; Kemp, R.; Tan, S.

    2006-01-01

    This study aimed at identifying some of the factors determining innovation performance in the SME. Based on a sample of 388 Dutch SME's, hypotheses were tested in particular regarding the main effects of strategy, and the direct and indirect efects of family orientation on firm performance. The

  6. Audiometric Characteristics of a Dutch DFNA10 Family With Mid-Frequency Hearing Impairment

    NARCIS (Netherlands)

    Beelen, E. van; Oonk, A.M.M.; Leijendeckers, J.M.; Hoefsloot, E.H.; Pennings, R.J.E.; Feenstra, I.; Dieker, H.J.; Huygen, P.L.M.; Snik, A.F.M.; Kremer, H.; Kunst, H.P.M.

    2016-01-01

    OBJECTIVES: Mutations in EYA4 can cause nonsyndromic autosomal dominant sensorineural hearing impairment (DFNA10) or a syndromic variant with hearing impairment and dilated cardiomyopathy. A mutation in EYA4 was found in a Dutch family, causing DFNA10. This study is focused on characterizing the

  7. Demonstration of S-100 protein in sustentacular cells of phaeochromocytomas and paragangliomas

    DEFF Research Database (Denmark)

    Schroder, H D; Johannsen, L

    1986-01-01

    to the sustentacular cells of normal paraganglia and adrenal medulla were found in all paragangliomas and in the benign and aggressively growing phaeochromocytomas. In the two malignant tumours no positive reaction was demonstrated. In one tumour the sustentacular cells were shown to contain glial fibrillary acidic......Eighteen phaeochromocytomas, including both sporadic and familial cases, four cervical paragangliomas, two jugular paragangliomas, and one abdominal paraganglioma were examined immunohistochemically for the presence of S-100 protein. Positive staining in cells morphologically similar...... protein further supporting their Schwann cell relationship. The number of S-100 positive cells varied considerably. They demonstrated a spindle celled or elongated configuration with long slender processes. The nature of the sustentacular cell proliferation, neoplastic versus reactive, is discussed....

  8. Contemporary management of jugular paragangliomas.

    Science.gov (United States)

    Wanna, George B; Sweeney, Alex D; Haynes, David S; Carlson, Matthew L

    2015-04-01

    Jugular paragangliomas are generally benign slow-growing tumors that can cause pulsatile tinnitus, hearing loss, and cranial nerves neuropathy. Progressive growth can also lead to intracranial extension. Historically, the treatment of choice for these lesions has been gross total resection. However, over the last 15 years, many groups have adopted less invasive management strategies including stereotactic radiation therapy, subtotal resection, and primary observation in order to reduce treatment-associated morbidity. The focus of this article is to review the modern management of jugular paraganglioma, highlighting the evolving treatment paradigm at the Otology Group of Vanderbilt. Copyright © 2015 Elsevier Inc. All rights reserved.

  9. L1 and L2 reading skills in Dutch adolescents with a familial risk of dyslexia

    Directory of Open Access Journals (Sweden)

    Ellie R.H. van Setten

    2017-10-01

    Full Text Available Background The present study investigated differences in reading and spelling outcomes in Dutch and English as a second language (ESL in adolescents with a high familial risk of dyslexia, of whom some have developed dyslexia (HRDys while others have not (HRnonDys, in comparison to a low familial risk control group without dyslexia (LRnonDys. This allowed us to investigate the persistence of dyslexia in the first language (L1 and the effect of dyslexia on the second language (L2, which has, in this case, a lower orthographic transparency. Furthermore, the inclusion of the HRnonDys group allowed us to investigate the continuity of the familial risk of dyslexia, as previous studies observed that the HRnonDys group often scores in between the HRDys and LRnonDys group, and whether these readers without reading deficits in Dutch, have more reading difficulties in ESL. Methods The data of three groups of adolescents were analyzed; 27 LRnonDys, 25 HRdys 25 HRnonDys. The mean age was 14;1 years; months, and 37 were male. All were native speakers of Dutch, attended regular secondary education (grade 7–10, and were non-native speakers of English. Using MANOVA the groups were compared on Dutch and English word reading fluency (WRF, spelling and vocabulary, Dutch pseudoword and loanword reading fluency, phonological awareness (PA, rapid automatized naming (RAN, and verbal short term and working memory. A repeated measures ANOVA was used to compare English and Dutch WRF, spelling and vocabulary directly within the three groups. Results The analyses revealed that the HRDys group had a deficit in both reading and spelling in Dutch and ESL. They also performed poorer than the LRnonDys group on all other measures. Effect sizes were especially large for pseudoword reading and the reaction times during the PA task. The HRnonDys group scored generally poorer than the LRnonDys group but this difference was only significant for Dutch pseudoword reading, PA reaction

  10. Reciprocity revisited: Give and take in Dutch and immigrant families

    NARCIS (Netherlands)

    Komter, A.; Schans, J.M.D.

    2008-01-01

    Classical theory suggests that "generalized reciprocity," giving without clear expectations of returns, is characteristic for exchange within the family. Modern theory assumes differences between Western, "individualistic" cultures, and non-Western, more "collectivistic" cultures, presumably leading

  11. Dutch family physicians' awareness of cognitive impairment among the elderly

    NARCIS (Netherlands)

    van den Dungen, Pim; Moll van Charante, Eric P.; van de Ven, Peter M.; Foppes, Gerbrand; van Campen, Jos P. C. M.; van Marwijk, Harm W. J.; van der Horst, Henriëtte E.; van Hout, Hein P. J.

    2015-01-01

    Dementia is often not formally diagnosed in primary care. To what extent this is due to family physicians' (FPs) watchful waiting, reluctance to diagnose or to their unawareness of the presence of cognitive impairment is unclear. The objective of this study was to assess FPs' awareness of cognitive

  12. Dutch family physicians' awareness of cognitive impairment among the elderly

    NARCIS (Netherlands)

    van den Dungen, P.; van Charante, E.P.M.; van de Ven, P.M.; Foppes, G.; van Campen, J.P.C.M.; van Marwijk, H.W.J.; van der Horst, H.E.; van Hout, H.P.J.

    2015-01-01

    Background: Dementia is often not formally diagnosed in primary care. To what extent this is due to family physicians' (FPs) watchful waiting, reluctance to diagnose or to their unawareness of the presence of cognitive impairment is unclear. The objective of this study was to assess FPs' awareness

  13. Reciprocity Revisited : Give and Take in Dutch and Immigrant Families

    NARCIS (Netherlands)

    Komter, Aafke; Schans, Djamila

    2008-01-01

    The idea that reciprocity is the basic principle underlying forms of social organization, among which the family, is as old as classical anthropology and sociology. The essence of the principle is that giving prompts receiving, thereby creating forms of ongoing exchange and durable cooperation.

  14. Clinical value of somatostatin receptor imaging in patients with suspected head and neck paragangliomas

    Energy Technology Data Exchange (ETDEWEB)

    Schmidt, Matthias; Dietlein, Markus; Weber, Kerstin; Moka, Detlef; Schicha, Harald [Klinik und Poliklinik fuer Nuklearmedizin, Universitaet zu Koeln, Joseph-Stelzmann-Strasse 9, 50924 Koeln (Germany); Fischer, Eva; Michel, Olaf; Stennert, Eberhard [Klinik und Poliklinik fuer Hals-, Nasen- und Ohrenheilkunde, Universitaet zu Koeln, Koeln (Germany)

    2002-12-01

    Paragangliomas or glomus tumours of the head and neck region are rare somatostatin receptor-expressing neuroendocrine tumours. Precise preoperative diagnosis is of special importance in order to adequately weigh the potential benefit of the operation against the inherent risks of the procedure. In this study, the clinical value of somatostatin receptor imaging was assessed in 19 patients who underwent somatostatin receptor scintigraphy because of known or suspected paraganglioma of the head and neck region. The results were compared with the results of computed tomography and/or magnetic resonance imaging, histology and clinical follow-up. [{sup 111}In-DTPA-D-Phe{sup 1}]-octreotide scintigraphy was performed 4-6 and 24 h after i.v. injection of 140-220 MBq {sup 111}In-octreotide. Whole-body and planar images as well as single-photon emission tomography images were acquired and lesions were graded according to qualitative tracer uptake. Somatostatin receptor imaging was positive in nine patients, identifying paragangliomas for the first time in three patients and recurrent disease in six patients. In one patient, a second, previously unknown paraganglioma site was identified. Negative results were obtained in ten patients. These patients included one suffering from chronic hyperplastic otitis externa, one with granuloma tissue and an organised haematoma, one with an acoustic neuroma, one with an asymmetric internal carotid artery, two with ectasia of the bulbus venae jugularis and one with a jugular vein thrombosis. In two patients with a strong family history of paraganglioma, individual involvement could be excluded. In only one patient did somatostatin receptor imaging and magnetic resonance imaging yield false negative results in respect of recurrent paraganglioma tissue. It is concluded that somatostatin receptor scintigraphy provides important information in patients with suspected paragangliomas of the head and neck region and has a strong impact on further

  15. Parental and family-related influences on dental caries in children of Dutch, Moroccan and Turkish origin

    NARCIS (Netherlands)

    Duijster, D.; Jong-Lenters, M. de; Ruiter, C. de; Thijssen, J.; Loveren, C. van; Verrips, E.

    2015-01-01

    Objectives The aim of this cross-sectional study was to investigate the relationship between parental and family-related factors and childhood dental caries in a sample of 5- to 6-year-old children of Dutch, Moroccan and Turkish origin. Furthermore, the relationship of parental and family-related

  16. Treatment of Head and Neck Paragangliomas With External Beam Radiation Therapy

    Energy Technology Data Exchange (ETDEWEB)

    Dupin, Charles, E-mail: c.dupin@bordeaux.unicancer.fr [Department of Radiotherapy, Comprehensive Cancer Center, Institut Bergonié, Bordeaux (France); Lang, Philippe [Department of Radiotherapy, Pitié Salpétrière, Paris (France); Dessard-Diana, Bernadette [Department of Radiotherapy, Hopital Européen Georges Pompidou, Paris (France); Simon, Jean-Marc; Cuenca, Xavier; Mazeron, Jean-Jacques; Feuvret, Loïc [Department of Radiotherapy, Pitié Salpétrière, Paris (France)

    2014-06-01

    Purpose: To retrospectively assess the outcomes of radiation therapy in patients with head and neck paragangliomas. Methods and Materials: From 1990 to 2009, 66 patients with 81 head and neck paragangliomas were treated by conventional external beam radiation therapy in 25 fractions at a median dose of 45 Gy (range, 41.4-68 Gy). One case was malignant. The median gross target volume and planning target volume were 30 cm{sup 3} (range, 0.9-243 cm{sup 3}) and 116 cm{sup 3} (range, 24-731 cm{sup 3}), respectively. Median age was 57.4 years (range, 15-84 years). Eleven patients had multicentric lesions, and 8 had family histories of paraganglioma. Paragangliomas were located in the temporal bone, the carotid body, and the glomus vagal in 51, 18, and 10 patients, respectively. Forty-six patients had exclusive radiation therapy, and 20 had salvage radiation therapy. The median follow-up was 4.1 years (range, 0.1-21.2 years). Results: One patient had a recurrence of temporal bone paraganglioma 8 years after treatment. The actuarial local control rates were 100% at 5 years and 98.7% at 10 years. Patients with multifocal tumors and family histories were significantly younger (42 years vs 58 years [P=.002] and 37 years vs 58 years [P=.0003], respectively). The association between family predisposition and multifocality was significant (P<.001). Two patients had cause-specific death within the 6 months after irradiation. During radiation therapy, 9 patients required hospitalization for weight loss, nausea, mucositis, or ophthalmic zoster. Two late vascular complications occurred (middle cerebral artery and carotid stenosis), and 2 late radiation-related meningiomas appeared 15 and 18 years after treatment. Conclusion: Conventional external beam radiation therapy is an effective and safe treatment option that achieves excellent local control; it should be considered as a first-line treatment of choice for head and neck paragangliomas.

  17. Treatment of Head and Neck Paragangliomas With External Beam Radiation Therapy

    International Nuclear Information System (INIS)

    Dupin, Charles; Lang, Philippe; Dessard-Diana, Bernadette; Simon, Jean-Marc; Cuenca, Xavier; Mazeron, Jean-Jacques; Feuvret, Loïc

    2014-01-01

    Purpose: To retrospectively assess the outcomes of radiation therapy in patients with head and neck paragangliomas. Methods and Materials: From 1990 to 2009, 66 patients with 81 head and neck paragangliomas were treated by conventional external beam radiation therapy in 25 fractions at a median dose of 45 Gy (range, 41.4-68 Gy). One case was malignant. The median gross target volume and planning target volume were 30 cm 3 (range, 0.9-243 cm 3 ) and 116 cm 3 (range, 24-731 cm 3 ), respectively. Median age was 57.4 years (range, 15-84 years). Eleven patients had multicentric lesions, and 8 had family histories of paraganglioma. Paragangliomas were located in the temporal bone, the carotid body, and the glomus vagal in 51, 18, and 10 patients, respectively. Forty-six patients had exclusive radiation therapy, and 20 had salvage radiation therapy. The median follow-up was 4.1 years (range, 0.1-21.2 years). Results: One patient had a recurrence of temporal bone paraganglioma 8 years after treatment. The actuarial local control rates were 100% at 5 years and 98.7% at 10 years. Patients with multifocal tumors and family histories were significantly younger (42 years vs 58 years [P=.002] and 37 years vs 58 years [P=.0003], respectively). The association between family predisposition and multifocality was significant (P<.001). Two patients had cause-specific death within the 6 months after irradiation. During radiation therapy, 9 patients required hospitalization for weight loss, nausea, mucositis, or ophthalmic zoster. Two late vascular complications occurred (middle cerebral artery and carotid stenosis), and 2 late radiation-related meningiomas appeared 15 and 18 years after treatment. Conclusion: Conventional external beam radiation therapy is an effective and safe treatment option that achieves excellent local control; it should be considered as a first-line treatment of choice for head and neck paragangliomas

  18. Audiometric Characteristics of a Dutch DFNA10 Family With Mid-Frequency Hearing Impairment.

    Science.gov (United States)

    van Beelen, Eline; Oonk, Anne M M; Leijendeckers, Joop M; Hoefsloot, Elisabeth H; Pennings, Ronald J E; Feenstra, Ilse; Dieker, Hendrik-Jan; Huygen, Patrick L M; Snik, Ad F M; Kremer, Hannie; Kunst, Henricus P M

    2016-01-01

    Mutations in EYA4 can cause nonsyndromic autosomal dominant sensorineural hearing impairment (DFNA10) or a syndromic variant with hearing impairment and dilated cardiomyopathy. A mutation in EYA4 was found in a Dutch family, causing DFNA10. This study is focused on characterizing the hearing impairment in this family. Whole exome sequencing was performed in the proband. In addition, peripheral blood samples were collected from 23 family members, and segregation analyses were performed. All participants underwent otorhinolaryngological examinations and pure-tone audiometry, and 12 participants underwent speech audiometry. In addition, an extended set of audiometric measurements was performed in five family members to evaluate the functional status of the cochlea. Vestibular testing was performed in three family members. Two individuals underwent echocardiography to evaluate the nonsyndromic phenotype. The authors present a Dutch family with a truncating mutation in EYA4 causing a mid-frequency hearing impairment. This mutation (c.464del) leads to a frameshift and a premature stop codon (p.Pro155fsX). This mutation is the most N-terminal mutation in EYA4 found to date. In addition, a missense mutation, predicted to be deleterious, was found in EYA4 in two family members. Echocardiography in two family members revealed no signs of dilated cardiomyopathy. Results of caloric and velocity step tests in three family members showed no abnormalities. Hearing impairment was found to be symmetric and progressive, beginning as a mid-frequency hearing impairment in childhood and developing into a high-frequency, moderate hearing impairment later in life. Furthermore, an extended set of audiometric measurements was performed in five family members. The results were comparable to those obtained in patients with other sensory types of hearing impairments, such as patients with Usher syndrome type IIA and presbyacusis, and not to those obtained in patients with (cochlear

  19. Functional paraganglioma extra-adrenal

    International Nuclear Information System (INIS)

    Arroyo-Martinez, Laura; Alvarez-Pertuz, Humberto; Acuna-Calvo, Jorge; Montoya-Calles, Juan Diego

    2006-01-01

    Functioning paragangliomas are rare tumours that produce catecholamines.They originate from extra-adrenal chromaffin cells. They are frequently malignant and are associated with high incidence of persistent or recurrent disease after their primary treatment. They are known as glomus, chemodectomas, chromaffin paragangliomas and glomerulocytomas. The location is diverse and reflects the paragangliomar distribution in the body from the base of the skull to the pelvic floor. The paragangliomas are found where there are nodes of the autonomous system, however, approximately 90% of these tumours appear in the adrenal glands (and they constitute the pheochromocytomas) and the remaining 10% is a location extra adrenal, but it has been said that its impact can be underestimated, ranging from 18% to 22% in adults and children up to 30%. The extra-adrenal are originated more frequently in the abdomen (85%), other in the chest (12%) and more rarely in the head and neck (3%). Imaging studies and measurement of non-physiological production of catecholamines may aid in the diagnosis of this entity. Surgery is the treatment of choice. It is presented the case of a primigravidas patient aged 32 with HTAIE requiring caesarean section, who had a postpartum torpid and despite to multiple antihypertensive treatments their pathology was difficult to deal, with ophthalmic complications. Some time later, the patient is studied by hyperhidrosis, laboratory tests and images are requested and it is documented incidentally, a left retroperitoneal tumour, the studies are expanded and reach the correct diagnosis. The tumour required surgical resection. The patient had a satisfactory postoperative period and she discharged with control in the external consultation. (author) [es

  20. Treatment Option Overview (Pheochromocytoma and Paraganglioma)

    Science.gov (United States)

    ... foods high in tyramine (such as red wine, chocolate, and cheese). Tests that examine the blood and ... cause signs or symptoms are treated with drug therapy. Drug therapy begins when pheochromocytoma or paraganglioma is ...

  1. General Information about Pheochromocytoma and Paraganglioma

    Science.gov (United States)

    ... foods high in tyramine (such as red wine, chocolate, and cheese). Tests that examine the blood and ... cause signs or symptoms are treated with drug therapy. Drug therapy begins when pheochromocytoma or paraganglioma is ...

  2. Treatment Options for Pheochromocytoma and Paraganglioma

    Science.gov (United States)

    ... foods high in tyramine (such as red wine, chocolate, and cheese). Tests that examine the blood and ... cause signs or symptoms are treated with drug therapy. Drug therapy begins when pheochromocytoma or paraganglioma is ...

  3. Pheochromocytomas and paragangliomas in humans and dogs

    NARCIS (Netherlands)

    Galac, S.; Korpershoek, E

    2017-01-01

    Pheochromocytomas (PCCs) and paragangliomas (PGLs) are described in several species. In humans and dogs they have many similarities: the excessive catecholamine release in hormonally active PCC causes similar clinical signs, the frequency of metastasis is similar, and they are histopathologically

  4. Mosaicism in HIF2A-related polycythemia-paraganglioma syndrome.

    Science.gov (United States)

    Buffet, Alexandre; Smati, Sarra; Mansuy, Ludovic; Ménara, Mélanie; Lebras, Maëlle; Heymann, Marie-Françoise; Simian, Christophe; Favier, Judith; Murat, Arnaud; Cariou, Bertrand; Gimenez-Roqueplo, Anne-Paule

    2014-02-01

    HIF2A germline mutations were known to cause congenital polycythemia. Recently, HIF2A somatic mutations were found in several patients with polycythemia and paraganglioma, pheochromocytoma, or somatostatinoma, suggesting the occurrence of a de novo postzygotic HIF2A mutation that has not been demonstrated clearly. Patient 1 is a woman suffering from polycythemia diagnosed at the age of 16 years. She was operated on for a pheochromocytoma at 45 years and for two abdominal paragangliomas at 59 years. She was also diagnosed with somatostatinoma. Patient 2 is a young boy who suffered from polycythemia since infancy. He underwent surgery for a nonfunctional adrenal paraganglioma at the age of 9 years. We sequenced by Sanger and next-generation sequencing the HIF2A gene in DNA extracted from tumors, leukocytes, and buccal cells. In patient 1, we identified a somatic HIF2A mutation (c.1586T>C; p.Leu529Pro) in DNA extracted from both paragangliomas. The mutation was detected as a somatic mosaic in DNA extracted from somatostatinoma and was absent from germline DNA. In patient 2, we found an HIF2A heterozygous mutation (c.1625T>C; p.Leu542Pro) in the paraganglioma, but the mutation was also present as a mosaic in leukocyte DNA and in DNA extracted from buccal cells (3.3 and 8.96% of sequencing reads, respectively). Both mutations disrupt the hydroxylation domain of the HIF2α protein. Our study shows that HIF2A-related tumors are caused by postzygotic mutations occurring in early developmental stages. Potential germline mosaicism should be considered during the familial genetic counseling when an individual has been diagnosed with HIF2A-related polycythemia-paraganglioma syndrome.

  5. Suprasellar paraganglioma: a case report and review of the literature

    Energy Technology Data Exchange (ETDEWEB)

    Naggara, O.; Oppenheim, C.; Meder, J.F. [Centre Hospitalier Sainte-Anne, Department of Neuroradiology, Paris (France); Varlet, P. [Centre Hospitalier Sainte-Anne, Department of Anatomopathology, Paris (France); Page, P. [Centre Hospitalier Sainte-Anne, Department of Neurosurgery, Paris (France)

    2005-10-01

    Paragangliomas arising in the suprasellar region are extremely rare. We report a case of suprasellar paraganglioma in a 47-year-old man who presented with amnesia and impaired visual acuity without any endocrine dysfunction. Magnetic resonance imaging (MRI) showed a large enhancing tumour in the suprasellar area. Following subtotal surgical excision, the diagnosis of paraganglioma was confirmed by pathology. In this case report we describe the MRI pattern of suprasellar paraganglioma and review the literature of this uncommon lesion. (orig.)

  6. Familial occurrence of epilepsy in children with newly diagnosed multiple seizures : Dutch study of epilepsy in childhood

    NARCIS (Netherlands)

    Callenbach, PMC; Geerts, AT; Arts, WFM; van Donselaar, CA; Peters, A.C. Boudewyn; Stroink, H; Brouwer, OF

    Purpose: To study the familial occurrence of epilepsy in children with newly diagnosed multiple unprovoked seizures. Methods: Between August 1988 and September 1992, 462 children with two or more unprovoked seizures were included in the prospective Dutch Study of Epilepsy in Childhood. Seizures and

  7. A novel TECTA mutation in a Dutch DFNA8/12 family confirms genotype-phenotype correlation.

    NARCIS (Netherlands)

    Plantinga, R.F.; Brouwer, A.P.M. de; Huygen, P.L.M.; Kunst, H.P.M.; Kremer, H.; Cremers, C.W.R.J.

    2006-01-01

    A novel TECTA mutation, p.R1890C, was found in a Dutch family with nonsyndromic autosomal dominant sensorineural hearing impairment. In early life, presumably congenital, hearing impairment occurred in the midfrequency range, amounting to about 40 dB at 1 kHz. Speech recognition was good with all

  8. Pheochromocytoma-paraganglioma: Biochemical and genetic diagnosis.

    Science.gov (United States)

    Cano Megías, Marta; Rodriguez Puyol, Diego; Fernández Rodríguez, Loreto; Sención Martinez, Gloria Lisette; Martínez Miguel, Patricia

    Pheochromocytomas and paragangliomas are tumours derived from neural crest cells, which can be diagnosed by biochemical measurement of metanephrine and methoxytyramine. Advances in genetic research have identified many genes involved in the pathogenesis of these tumours, suggesting that up to 35-45% may have an underlying germline mutation. These genes have a singular transcriptional signature and can be grouped into 2 clusters (or groups): cluster 1 (VHL and SHDx), involved in angiogenesis and hypoxia pathways; and cluster 2 (MEN2 and NF1), linked to the kinase signalling pathway. In turn, these genes are associated with a characteristic biochemical phenotype (noradrenergic and adrenergic), and clinical features (location, biological behaviour, age of presentation, etc.) in a large number of cases. Early diagnosis of these tumours, accompanied by a correct genetic diagnosis, should eventually become a priority to enable better treatment, early detection of complications, proper screening of family members and related tumours, as well as an improvement in the overall prognosis of these patients. Copyright © 2016 Sociedad Española de Nefrología. Published by Elsevier España, S.L.U. All rights reserved.

  9. [Head and neck paragangliomas. Embryological origin and anatomical characteristics: topographic distribution and vascularization pattern].

    Science.gov (United States)

    Carretero González, José; Blanco Pérez, Pedro; Vázquez Osorio, María Teresa; Benito González, Fernando; Sañudo Tejedo, José Ramón

    2009-02-01

    Paragangliomas are tumors that arise in the extraadrenal paraganglia and result from migration of neural crest cells during embryonic development. Based on their anatomical distribution, innervation and microscopic structure, these tumors can be classified into interrelated families: branchiomeric paraganglia (related to the branchial clefts and arches), intravagal, aortic-sympathetic and visceral-autonomic. Head and neck paragangliomas belong mainly to the first two of these families. The present article is divided into two parts. The first part reviews the embryological origin of these tumors. Special emphasis is placed on the process of neurulation or neural tube formation, neurosegmentation (with a summary of the mechanisms involved in the initial segmentation of the neural tube and of the hindbrain and spinal medulla), and the development of the sensory placodes and secondary inductions in the cranial region. Subsequently, the neural crest is analyzed, with special attention paid to the cranial neural crest. The embryonogenesis of paragangliomas is also described. The second part describes the topographical distribution of head and neck paragangliomas according to their localization: jugulotympanic, orbit, intercarotid, subclavian and laryngeal. The embryonogenesis and most important anatomical characteristics are described for each type.

  10. Genomewide scan identifies susceptibility locus for dyslexia on Xq27 in an extended Dutch family.

    Science.gov (United States)

    de Kovel, C G F; Hol, F A; Heister, J G A M; Willemen, J J H T; Sandkuijl, L A; Franke, B; Padberg, G W

    2004-09-01

    Dyslexia is a common disorder with a strong genetic component, but despite significant research effort, the aetiology is still largely unknown. To identify loci contributing to dyslexia risk. This was a genomewide linkage analysis in a single large family. Dutch families with at least two first degree relatives suffering from dyslexia participated in the study. Participants were recruited through an advertisement campaign in papers and magazines. The main outcome measure was linkage between genetic markers and dyslexia phenotype. Using parametric linkage analysis, we found strong evidence for a locus influencing dyslexia on Xq27.3 (multipoint lod = 3.68). Recombinations in two family members flanked an 8 cM region, comprising 11 currently confirmed genes. All four males carrying the risk haplotype had very low scores on the reading tests. The presentation in females was more variable, but 8/9 females carrying the risk haplotype were diagnosed dyslexic by our composite score, so we considered the putative risk allele to be dominant with reduced penetrance. Linkage was not found in an additional collection of affected sibling pairs. A locus influencing dyslexia risk is probably located between markers DXS1227 and DXS8091 on the X chromosome, closely situated to a locus indicated by a published genome scan of English sibling pairs. Although the locus may not be a common cause for dyslexia, the relatively small and gene poor region offers hope to identify the responsible gene.

  11. A familial hemorrhagic diathesis in a Dutch family: An inherited deficiency of ??2-antiplasmin

    NARCIS (Netherlands)

    Kluft, C.; Vellenga, E.; Brommer, E.J.P.; Wijngaards, G.

    1982-01-01

    This study concerns a case of congenital homozygous deficiency in ??2-antiplasmin associated with a severe hemorrhagic diathesis. Heterozygous family members also show a mild bleeding tendency. The propositus is a 17-yr-old male born of white parents and showing a severe hemorrhagic diathesis

  12. Civic Competence of Dutch Children in Female Same-Sex Parent Families: A Comparison With Children of Opposite-Sex Parents

    Science.gov (United States)

    Bos, Henny; Gartrell, Nanette; Roeleveld, Jaap; Ledoux, Guuske

    2016-01-01

    This study examined whether Dutch children reared in families headed by female same-sex parents differ in civic competence from Dutch children reared by opposite-sex parents. The participants, drawn from a national sample, included 32 children (11-13 years old) parented by female same-sex couples who were matched on demographic characteristics…

  13. Review of Pediatric Pheochromocytoma and Paraganglioma

    Directory of Open Access Journals (Sweden)

    Reshma Bholah

    2017-07-01

    Full Text Available Pheochromocytoma (PCC and paraganglioma (PGL are rare chromaffin cell tumors which secrete catecholamines and form part of the family of neuroendocrine tumors. Although a rare cause of secondary hypertension in pediatrics, the presentation of hypertension in these patients is characteristic, and treatment is definitive. The gold standard for diagnosis is via measurement of plasma free metanephrines, with imaging studies performed for localization, identification of metastatic lesions and for surgical resection. Preoperative therapy with alpha-blocking agents, beta blockers, and potentially tyrosine hydroxylase inhibitors aid in a safe pre-, intra- and postoperative course. PCC and PGL are inherited in as much as 80% of pediatric cases, and all patients with mutations should be followed closely given the risk of recurrence and malignancy. While the presentation of chromaffin cell tumors has been well described with multiple endocrine neoplasia, NF1, and Von Hippel–Lindau syndromes, the identification of new gene mutations leading to chromaffin cell tumors at a young age is changing the landscape of how clinicians approach such cases. The paraganglioma–pheochromocytoma syndromes (SDHx comprise familial gene mutations, of which the SDHB gene mutation carries a high rate of malignancy. Since the inheritance rate of such tumors is higher than previously described, genetic screening is recommended in all patients, and lifelong follow-up for recurrent tumors is a must. A multidisciplinary team approach allows for optimal health-care delivery in such children. This review serves to provide an overview of pediatric PCC and PGL, including updates on the preferred methods of imaging, guidelines on gene testing as well as management of hypertension in such patients.

  14. Paragangliomas of the head and neck: clinical, morphological and immunohistochemical aspects

    Directory of Open Access Journals (Sweden)

    Pedro de Alcântara de Andrade Filho

    2001-05-01

    Full Text Available CONTEXT: Protein marker positivity can assist in the definition of the therapeutic approach towards head and neck paragangliomas. The establishment of the therapeutic approach should incorporate the results of such an investigation. OBJECTIVE: To establish criteria for benignancy and malignancy of vagal and jugular-tympanic paragangliomas, via the study of the relationships of sex, age, tumor size, duration of complaints, site, family history, presence of metastases, treatment, histological architecture and cell type with the immunohistochemical reactions to S100 protein, chromogranin and AgKi67. DESIGN: A retrospective study of histological and clinical records. SETTING: The Heliópolis and Oswaldo Cruz tertiary general hospitals, São Paulo. SAMPLE: 8 cases of head and neck paragangliomas. MAIN MEASUREMENTS: Determination of degree of positivity to paragangliomas via immunohistochemical reactions. RESULTS: 1. The protein markers for the principal cells (AgKi67 and chromogranin were sensitive in 100% of the tumors when used together. 2. S100 protein was well identified in the cytoplasm and nucleus of sustentacular cells and underwent reduction in the neoplasias. CONCLUSIONS: Chromogranin was proven to be a generic marker for neuroendocrine tumors; S100 protein was positive in all 8 cases and the AgKi67 had low positivity in all cases.

  15. Nonfunctional Cystic Hepatic Paraganglioma Mimicking Hydatid Cyst

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    Preeti Balkisanji Agrawal

    2017-11-01

    Full Text Available An extra-adrenal pheochromocytoma is also known as a paraganglioma. We are reporting the case of a 68-year-old female patient with an extremely rare primary nonfunctioning hepatic paraganglioma without any clinical signs and symptoms. A CECT scan of whole abdomen was done which showed a huge well defined peripherally enhancing fluid density cystic lesion measuring 14 cm × 14 cm × 12 cm with internal enhancing septations and few foci of calcification involving V to VIII segments of right lobe of liver which was compressing and displacing portal vein, its right branch, common bile duct, gall bladder. Mass was also compressing and displacing right kidney inferiorly. Laparotomy was done and sample of cyst wall sent for histopathology. After immunohistochemistry a diagnosis of paraganglioma was confirmed.

  16. Descolamento de retina seroso em paraganglioma: relato de caso Serous retinal detachment in paraganglioma: case report

    Directory of Open Access Journals (Sweden)

    Oscar Villas Boas

    2008-02-01

    Full Text Available Os autores descrevem um caso de uma paciente gestante com hipertensão arterial resistente ao tratamento e descolamento seroso bilateral de retina. Confirmou-se, pelo exame anátomo-patológico, ser um paraganglioma.The authors describe a case of a pregnant patient with arterial hypertension that resists to the treatment and retinal bilateral serous detachment. It was confirmed to be a paraganglioma by anatomicopathological examination.

  17. Rare vertebral metastasis in a case of Hereditary Paraganglioma

    Directory of Open Access Journals (Sweden)

    da Silva Manuel Eduardo

    2012-09-01

    Full Text Available Abstract Paragangliomas are rare tumours with a prevalence of 1/10000 to 1/30000. Tumors arising from the paraganglia are characteristically of low malignant potential. Vertebral metastases are exceedingly rare, and only isolated case reports have described them. The authors present the clinical course of a 47 years-old female patient with a familial paraganglioma [PGL] with vertebral metastastization, who underwent an intralesional tumor excision and corpectomy. Genetic screening demonstrated a new germinal frameshift mutation of the SDHB exon 6 [c.587-591DelC]. After surgery there was normalization of the analytical parameters and imagiologic screening. One year later she presented a new image in the the pedicle of T11 on the contralateral side of the surgical incision. She performed 2 treatments with MIBG and 1 cicle of radiotherapy that made the new lesion regress. Currently the patient does not present any clinical or analytical evidence of new metastasis. This case outlines the clinical course of a patient with a PGL syndrome for whom a rare vertebral metastasis was diagnosed. It highlights the importance of identifying patients with germline SDHB mutations, as these patients are at a high risk of developing malignant disease.

  18. Osteological evidence of short-limbed dwarfism in a nineteenth century Dutch family: Achondroplasia or hypochondroplasia.

    Science.gov (United States)

    Waters-Rist, Andrea L; Hoogland, Menno L P

    2013-12-01

    An opportunity to explore osteological features of a form of disproportionate dwarfism is presented by a recent archaeological discovery. Excavation of a predominately nineteenth century Dutch cemetery from the rural, agricultural village of Middenbeemster revealed an older adult female with skeletal changes consistent with achondroplasia. The most marked features are a rhizomelic pattern of shortened and thickened upper and lower limbs, frontal bossing and a moderately depressed nasal bridge, small lumbar neural canals with short pedicles, bowing of the femora and tibiae, and short stature (130.0±5cm). However, some common features of achondroplasia like cranial base reduction and shortened fingers and toes are absent. The alternative diagnosis of a more mild form of short-limbed dwarfism, hypochondroplasia, is explored and aided by archival identification of the individual and her offspring. Five offspring, including three perinates, a 10-year-old daughter, and a 21-year-old son, are analysed for evidence of an inherited skeletal dysplasia. The unique addition of family history to the paleopathological diagnostic process supports a differential outcome of hypochondroplasia. This combination of osteological and archival data creates a unique opportunity to track the inheritance and manifestation of a rare disease in a past population. Copyright © 2013 Elsevier Inc. All rights reserved.

  19. Comprehensive Molecular Characterization of Pheochromocytoma and Paraganglioma

    NARCIS (Netherlands)

    Fishbein, Lauren; Leshchiner, Ignaty; Walter, Vonn; Danilova, Ludmila; Robertson, A. Gordon; Johnson, Amy R.; Lichtenberg, Tara M.; Murray, Bradley A.; Ghayee, Hans K.; Else, Tobias; Ling, Shiyun; Jefferys, Stuart R.; de Cubas, Aguirre A.; Wenz, Brandon; Korpershoek, Esther; Amelio, Antonio L.; Makowski, Liza; Rathmell, W. Kimryn; Gimenez-Roqueplo, Anne Paule; Giordano, Thomas J.; Asa, Sylvia L.; Tischler, Arthur S.; Akbani, Rehan; Ally, Adrian; Amar, Laurence; Amelio, Antonio L.; Arachchi, Harindra; Asa, Sylvia L.; Auchus, Richard J.; Auman, J. Todd; Baertsch, Robert; Balasundaram, Miruna; Balu, Saianand; Bartsch, Detlef K.; Baudin, Eric; Bauer, Thomas; Beaver, Allison; Benz, Christopher; Beroukhim, Rameen; Beuschlein, Felix; Bodenheimer, Tom; Boice, Lori; Bowen, Jay; Bowlby, Reanne; Brooks, Denise; Carlsen, Rebecca; Carter, Suzie; Cassol, Clarissa A.; Cherniack, Andrew D.; Chin, Lynda; Cho, Juok; Chuah, Eric; Chudamani, Sudha; Cope, Leslie; Crain, Daniel; Curley, Erin; Danilova, Ludmila; de Cubas, Aguirre A.; de Krijger, Ronald R.; Demchok, John A.; Deutschbein, Timo; Dhalla, Noreen; Dimmock, David; Dinjens, Winand N M; Else, Tobias; Eng, Charis; Eschbacher, Jennifer; Fassnacht, Martin; Felau, Ina; Feldman, Michael; Ferguson, Martin L.; Fiddes, Ian; Fishbein, Lauren; Frazer, Scott; Gabriel, Stacey B.; Gardner, Johanna; Gastier-Foster, Julie M.; Gehlenborg, Nils; Gerken, Mark; Getz, Gad; Geurts, Jennifer; Ghayee, Hans K.; Gimenez-Roqueplo, Anne Paule; Giordano, Thomas J.; Goldman, Mary; Graim, Kiley; Gupta, Manaswi; Haan, David; Hahner, Stefanie; Hantel, Constanze; Haussler, David; Hayes, D. Neil; Heiman, David I.; Hoadley, Katherine A.; Holt, Robert A.; Hoyle, Alan P.; Huang, Mei; Hunt, Bryan; Hutter, Carolyn M.; Jefferys, Stuart R.; Johnson, Amy R.; Jones, Steven J M; Jones, Corbin D.; Kasaian, Katayoon; Kebebew, Electron; Kim, Jaegil; Kimes, Patrick; Knijnenburg, Theo; Korpershoek, Esther; Lander, Eric; Lawrence, Michael S.; Lechan, Ronald; Lee, Darlene; Leraas, Kristen M.; Lerario, Antonio; Leshchiner, Ignaty; Lichtenberg, Tara M.; Lin, Pei; Ling, Shiyun; Liu, Jia; LiVolsi, Virginia A.; Lolla, Laxmi; Lotan, Yair; Lu, Yiling; Ma, Yussanne; Maison, Nicole; Makowski, Liza; Mallery, David; Mannelli, Massimo; Marquard, Jessica; Marra, Marco A.; Matthew, Thomas; Mayo, Michael; Méatchi, Tchao; Meng, Shaowu; Merino, Maria J.; Mete, Ozgur; Meyerson, Matthew; Mieczkowski, Piotr A.; Mills, Gordon B.; Moore, Richard A.; Morozova, Olena; Morris, Scott; Mose, Lisle E.; Mungall, Andrew J.; Murray, Bradley A.; Naresh, Rashi; Nathanson, Katherine L.; Newton, Yulia; Ng, Sam; Ni, Ying; Noble, Michael S.; Nwariaku, Fiemu; Pacak, Karel; Parker, Joel S.; Paul, Evan; Penny, Robert; Perou, Charles M.; Perou, Amy H.; Pihl, Todd; Powers, James; Rabaglia, Jennifer; Radenbaugh, Amie; Ramirez, Nilsa C.; Rao, Arjun; Rathmell, W. Kimryn; Riester, Anna; Roach, Jeffrey; Robertson, A. Gordon; Sadeghi, Sara; Saksena, Gordon; Salama, Sofie; Saller, Charles; Sandusky, George; Sbiera, Silviu; Schein, Jacqueline E.; Schumacher, Steven E.; Shelton, Candace; Shelton, Troy; Sheth, Margi; Shi, Yan; Shih, Juliann; Shmulevich, Ilya; Simons, Janae V.; Sipahimalani, Payal; Skelly, Tara; Sofia, Heidi J.; Sokolov, Artem; Soloway, Matthew G.; Sougnez, Carrie; Stuart, Josh; Sun, Charlie; Swatloski, Teresa; Tam, Angela; Tan, Donghui; Tarnuzzer, Roy; Tarvin, Katherine; Thiessen, Nina; Thorne, Leigh B.; Timmers, Henri J.; Tischler, Arthur S.; Tse, Kane; Uzunangelov, Vlado; van Berkel, Anouk; Veluvolu, Umadevi; Vicha, Ales; Voet, Doug; Waldmann, Jens; Walter, Vonn; Wan, Yunhu; Wang, Zhining; Wang, Tracy S.; Weaver, Joellen; Weinstein, John N.; Weismann, Dirk; Wenz, Brandon; Wilkerson, Matthew D.; Wise, Lisa; Wong, Tina; Wong, Christopher; Wu, Ye; Yang, Liming; Zelinka, Tomas; Zenklusen, Jean C.; Zhang, Jiashan (Julia); Zhang, Wei; Zhu, Jingchun; Zinzindohoué, Franck; Zmuda, Erik; Pacak, Karel; Nathanson, Katherine L.; Wilkerson, Matthew D.

    2017-01-01

    We report a comprehensive molecular characterization of pheochromocytomas and paragangliomas (PCCs/PGLs), a rare tumor type. Multi-platform integration revealed that PCCs/PGLs are driven by diverse alterations affecting multiple genes and pathways. Pathogenic germline mutations occurred in eight

  20. Retroperitoneal extraadrenal paraganglioma report of two cases.

    Science.gov (United States)

    Becheanu, G; Laky, D

    1997-01-01

    Two cases with retroperitoneal tumours affecting young women who cannot undergo surgery were investigated by biopsy and needle sampling. One of these cases presented a mainly alveolar histologic pattern and was easily diagnosed by immunohistochemical means, as well as a chromaffin paraganglioma. We discussed different diagnoses and approaches in the literature.

  1. Breast Metastasis from Malignant Paraganglioma: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Nam, Sang Yu; Han, Boo Kyung [Dept. of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2013-06-15

    We report a case of metastatic breast cancer from paraganglioma and describe the radiologic findings in a 32-year-old woman who had a history of excision for carotid body paraganglioma. Breast metastasis from malignant paraganglioma showed a well-defined mass with hypoechogenicity, posterior acoustic enhancement and iNcreased vascularity on ultrasonography, and strong enhancements on contrast-enhanced MRI and diffusion restriction on diffusion weighted image.

  2. Familial clustering and genetic risk for dementia in a genetically isolated Dutch population.

    NARCIS (Netherlands)

    K. Sleegers (Kristel); F. Forey; J. Theuns (Jessie); Y.S. Aulchenko (Yurii); S. Rademakers (Suzanne); M. Cruts (Marc); W.A. van Gool (Willem); P. Heutink (Peter); B.A. Oostra (Ben); J.C. van Swieten (John); C.M. van Duijn (Cornelia); C. van Broeckhoven (Christine)

    2004-01-01

    textabstractDespite advances in elucidating the genetic epidemiology of Alzheimer's disease and frontotemporal dementia, the aetiology for most patients with dementia remains unclear. We examined the genetic epidemiology of dementia in a recent genetically isolated Dutch population founded around

  3. Familial clustering and genetic risk for dementia in a genetically isolated Dutch population

    NARCIS (Netherlands)

    Sleegers, K.; Roks, G.; Theuns, J.; Aulchenko, Y. S.; Rademakers, R.; Cruts, M.; van Gool, W. A.; van Broeckhoven, C.; Heutink, P.; Oostra, B. A.; van Swieten, J. C.; van Duijn, C. M.

    2004-01-01

    Despite advances in elucidating the genetic epidemiology of Alzheimer's disease and frontotemporal dementia, the aetiology for most patients with dementia remains unclear. We examined the genetic epidemiology of dementia in a recent genetically isolated Dutch population founded around 1750. The

  4. Cervical Paraganglioma Mimicking Thyroid Nodule: A Rare Clinical Case

    Directory of Open Access Journals (Sweden)

    Berna İmge Aydoğan

    2016-01-01

    Full Text Available Objective. Paraganglioma is a rare neuroendocrine tumor. When it is located in the neck, it is commonly misdiagnosed as other thyroid neoplasms. Case Report. We report a case of cervical paraganglioma in a 55-year-old female. Patient was admitted to our clinic with goiter and neck pain. Thyroid ultrasonography revealed a 20 mm solitary, heterogeneous nodule located in the upper pole of left thyroid lobe. Fine needle aspiration cytology was nondiagnostic. She underwent left lobectomy and histopathology showed paraganglioma. Discussion. Cervical paragangliomas should be considered in the differential diagnosis of thyroid nodules.

  5. Prenatal famine, birthweight, reproductive performance and age at menopause: the Dutch hunger winter families study.

    Science.gov (United States)

    Yarde, F; Broekmans, F J M; van der Pal-de Bruin, K M; Schönbeck, Y; te Velde, E R; Stein, A D; Lumey, L H

    2013-12-01

    Is there an association between acute prenatal famine exposure or birthweight and subsequent reproductive performance and age at menopause? No association was found between intrauterine famine exposure and reproductive performance, but survival analysis showed that women exposed in utero were 24% more likely to experience menopause at any age. Associations between prenatal famine and subsequent reproductive performance have been examined previously with inconsistent results. Evidence for the effects of famine exposure on age at natural menopause is limited to one study of post-natal exposure. This cohort study included men and women born around the time of the Dutch famine of 1944-1945. The study participants (n = 1070) underwent standardized interviews on reproductive parameters at a mean age of 59 years. The participants were grouped as men and women with prenatal famine exposure (n = 407), their same-sex siblings (family controls, n = 319) or other men and women born before or after the famine period (time controls, n = 344). Associations of famine exposure with reproductive performance and menopause were analysed using logistic regression and survival analysis with competing risk, after controlling for family clustering. Gestational famine exposure was not associated with nulliparity, age at birth of first child, difficulties conceiving or pregnancy outcome (all P> 0.05) in men or women. At any given age, women were more likely to experience menopause after gestational exposure to famine (hazard ratio 1.24; 95% CI 1.03, 1.51). The association was not attenuated with an additional control for a woman's birthweight. In this study, there was no association between birthweight and age at menopause after adjustment for gestational famine exposure. Age at menopause was self-reported and assessed retrospectively. The study power to examine associations with specific gestational periods of famine exposure and reproductive function was limited. Our findings support

  6. Paraganglioma pré-aórtico gigante Giant preaortic paraganglioma

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    Sergio Renato Pais Costa

    2008-12-01

    Full Text Available INTRODUÇÃO: O paraganglioma é um tumor neuroendócrino raro que ocorre mais frequentemente em adultos jovens. Geralmente produz catecolaminas acarretando síndrome adrenérgica. No entanto, muito raramente, quando não-funcionante seus sintomas são mais frequentemente associados à massa abdominal ou mesmo dor. Nessas circunstâncias costumam representar diagnóstico difícil sendo confundidos com os sarcomas de retroperitônio. RELATO DO CASO: Paraganglioma pré-aórtico gigante não-funcionante em paciente com níveis baixos de catecolaminas (sérico e urinário. O diagnóstico pré-operatório foi dado por tomografia computadorizada, onde foi observada massa sólida, hipervascular com calcificações e área cística central. O paciente foi submetido à ressecção cirúrgica da massa com boa evolução pós-operatória. Um ano após a operação, o doente encontra-se vivo sem recidiva tumoral. CONCLUSÃO: Em que pese a raridade o paraganglioma pré-aortico não-funcionante deve ser lembrado com diagnóstico diferencial com os sarcomas de retroperitônio. Seu tratamento é cirúrgico e apresenta bom prognóstico.RACIONAL: Paraganglioma is a rare neuroendocrine tumor which often diagnosed in the young adult. Generally, paraganglioma produces catecholamines causing adrenergic syndrome. However, more rarely when tumor is nonfunctioning, their symptoms are more associated with an abdominal mass or even pain. In these circumstances, paraganglioma present a difficult diagnosis confounding with retroperitoneal sarcomas. CASE REPORT: The authors present a case of nonfunctioning giant preaortic paraganglioma. This patient had low levels of catecholamines (both seric and urinary. The preoperative diagnosis was done by means computed tomography. This lesion presented as well-vascularized tumor with calcifications and necrotic central area. The patient underwent a surgical resection with good postoperative outcome. To date, one year after surgical

  7. Paragangliomas of the head and neck region: A single center experience

    Directory of Open Access Journals (Sweden)

    Kumudachalam Pindicura

    2017-01-01

    Conclusion: Paragangliomas of the head and neck are uncommon lesions. The most common site in the head and neck region was the jugulotympanic region. Most cases of jugulotympanic paraganglioma presented in the fifth decade and later. Laryngeal paraganglioma presented clinically earlier in the second decade. Jugulotympanic and laryngeal paraganglioma showed dominance of tumor vasculature histologically. There was a rare case of laryngeal paraganglioma with lateral neck extension in this study.

  8. Unusual Cause of Acute Abdomen—Ruptured Retroperitoneal Paraganglioma

    OpenAIRE

    Kwok-Kay Yau; Wing-Tai Siu; Michael Ka-Wah Li

    2008-01-01

    Ruptured retroperitoneal paraganglioma is a rare cause of acute abdomen. Its clinical presentation and laparoscopic features have seldom been reported in the literature. Herein, we report a case of ruptured retroperitoneal paraganglioma that presented as acute abdomen, and its subsequent management.

  9. Unusual Cause of Acute Abdomen—Ruptured Retroperitoneal Paraganglioma

    Directory of Open Access Journals (Sweden)

    Kwok-Kay Yau

    2008-01-01

    Full Text Available Ruptured retroperitoneal paraganglioma is a rare cause of acute abdomen. Its clinical presentation and laparoscopic features have seldom been reported in the literature. Herein, we report a case of ruptured retroperitoneal paraganglioma that presented as acute abdomen, and its subsequent management.

  10. Happy Spouses, Happy Parents? Family Relationships among Finnish and Dutch Dual Earners

    Science.gov (United States)

    Malinen, Kaisa; Kinnunen, Ulla; Tolvanen, Asko; Ronka, Anna; Wierda-Boer, Hilde; Gerris, Jan

    2010-01-01

    In this study links between spousal and parent-child relationships among Finnish (n = 157 couples) and Dutch (n = 276 couples) dual earners with young children were examined using paired questionnaire data. Variable-oriented analyses (structural equation modeling with a multigroup procedure) supported the spillover hypothesis, as higher levels of…

  11. Paraganglioma de mediastino com metástases pulmonares Pulmonary metastasis of mediastinal paraganglioma

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    Manoel Ximenes Netto

    2005-02-01

    Full Text Available Descrevemos uma paciente de 27 anos que se apresentou com paraganglioma de mediastino anterior e médio e nódulos pulmonares bilaterais. O tratamento consistiu na ressecção das lesões pulmonares através de toracotomia anterior bilateral transesternal e retirada do paraganglioma com auxílio de circulação extracorpórea. Como tratamento neoadjuvante foram usadas radioterapia e quimioterapia. A evolução pós-operatória foi satisfatória, e catorze meses depois a paciente encontrava-se assintomática.Herein, we describe the case of a 27-year-old female presenting with paraganglioma of the anterior and middle mediastinum and bilateral pulmonary nodules. Treatment consisted of pulmonary resection by anterior bilateral thoracotomy and transverse sternotomy, in which the paraganglioma was excised with the aid of extracorporeal circulation. As neoadjuvant treatments, radiotherapy and chemotherapy were applied. Postoperative evolution was uneventful, and the patient was classified as asymptomatic after 14 months.

  12. Jugular paraganglioma treatment at the UMC Maribor

    Directory of Open Access Journals (Sweden)

    Janez Rebol

    2017-11-01

    Full Text Available Background: Jugular paragangliomas are rare, almost exclusively benign and slowly growing tumors. Arising from the cells of paraganglia in jugular bulb, they infiltrate the temporal bone and later grow intracranially. Because of insidious onset, their diagnosis is relatively late. Patients at presentation usually complain of pulsatile tinnitus and hearing loss, lasting for years. Also common are palsies of the cranial nerves in the area of tumor growth – i.e., facial, glossopharyngeal, vagal, accessory and hypoglossal nerves. Surgical resection is the standard treatment, though technically demanding because of difficult approach to the lateral cranial base and vital structures in the area. The team for treating such patients comprises an ENT specialist, interventional radiologist and a neurosurgeon. The purpose of this article is to review clinical experiences with the treatment of jugular paragangliomas at our Department of ENT and Maxillofacial Surgery of the University Medical Centre Maribor. Methods: We reviewed the documentation of ten patients treated for jugular paraganglioma in the last 15 years, and presented the clinical data in a table. Results: Nine patients were treated by preoperative embolization and surgical resection. Surgery was contraindicated in one patient with highly dominant venous drainage on the side of the tumor and aplastic transverse sinus on the opposite side. She was treated by primary radiotherapy. One patient with intracranial tumor growth was treated by subtotal resection and adjuvant radiotherapy. At presentation, lower cranial nerve palsies were present in 60 % of our patients. With the other 40 % we managed to preserve the nerve function postoperatively. Facial nerve function practically normalized in all patients with anterior transposition of the nerve. Our patients were provided with postoperative rehabilitation and corrective procedures such as vocal cord medialization and BAHA hearing aid implantation

  13. Temporal bone paragangliomas: 15 years experience

    Directory of Open Access Journals (Sweden)

    Mehmet Düzlü

    Full Text Available Abstract Introduction Temporal bone paragangliomas (TBPs are benign tumors arising from neural crest cells located along the jugular bulbus and the tympanic plexus. In general surgical excision, radiotherapy and wait-and-scan protocols are the main management modalities for TBPs. Objective In this paper we aim to present our clinical experience with TBPs and to review literature data. Methods The patients who were operated for tympanomastoid paraganglioma (TMP or tympanojugular paraganglioma (TJP in our clinic in the last 15 years were enrolled in the study. A detailed patient's charts review was performed retrospectively. Results There were 18 (52.9% cases with TMPs and 16 (47.1% cases with TJPs, a total of 34 patients operated for TBPs in this time period. The mean age was 50.3 ± 11.7 (range 25-71 years. The most common presenting symptoms were tinnitus and hearing loss for both TMPs and TJPs. Gross total tumor resection was achieved in 17 (94.4% and 10 (62.5% cases for TMPs and TJPs, respectively. Five patients (31.2% with TJP experienced facial palsy following the operation. For all the patients the mean follow-up period was 25.8 months (range 4-108 months. Conclusion In conclusion, based on our findings and literature review, total surgical excision alone or with preoperative embolization is the main treatment modality for TBPs. However radiotherapy, observation protocol and subtotal resection must be considered in cases of preoperative functioning cranial nerves, large tumors and advanced age.

  14. Temporal bone paragangliomas: 15 years experience.

    Science.gov (United States)

    Düzlü, Mehmet; Tutar, Hakan; Karamert, Recep; Karaloğlu, Furkan; Şahin, Muammer Melih; Göcek, Mehmet; Uğur, Mehmet Birol; Göksu, Nebil

    2016-12-08

    Temporal bone paragangliomas (TBPs) are benign tumors arising from neural crest cells located along the jugular bulbus and the tympanic plexus. In general surgical excision, radiotherapy and wait-and-scan protocols are the main management modalities for TBPs. In this paper we aim to present our clinical experience with TBPs and to review literature data. The patients who were operated for tympanomastoid paraganglioma (TMP) or tympanojugular paraganglioma (TJP) in our clinic in the last 15 years were enrolled in the study. A detailed patient's charts review was performed retrospectively. There were 18 (52.9%) cases with TMPs and 16 (47.1%) cases with TJPs, a total of 34 patients operated for TBPs in this time period. The mean age was 50.3± 11.7 (range 25-71 years). The most common presenting symptoms were tinnitus and hearing loss for both TMPs and TJPs. Gross total tumor resection was achieved in 17 (94.4%) and 10 (62.5%) cases for TMPs and TJPs, respectively. Five patients (31.2%) with TJP experienced facial palsy following the operation. For all the patients the mean follow-up period was 25.8 months (range 4-108 months). In conclusion, based on our findings and literature review, total surgical excision alone or with preoperative embolization is the main treatment modality for TBPs. However radiotherapy, observation protocol and subtotal resection must be considered in cases of preoperative functioning cranial nerves, large tumors and advanced age. Copyright © 2016 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  15. Paraganglioma of the urinary bladder with pelvic metastasis

    Directory of Open Access Journals (Sweden)

    Jiun-Hung Geng

    2014-09-01

    Full Text Available A 52-year-old male, diagnosed with paraganglioma of the urinary bladder, underwent transurethral resection of the bladder tumor 10 years ago. He was lost to follow-up after the operation but was recently admitted to our hospital for the treatment of nasopharyngeal cancer. However, refractory hypertension with palpitation was noted and a computed tomography scan revealed a round, well-defined mass at the right pelvic region. Retroperitoneal tumor excision surgery was performed and a subsequent pathological analysis revealed paraganglioma. The diagnosis of paraganglioma of the urinary bladder with pelvic metastasis was confirmed and his blood pressure returned to normal level without medication after the operation.

  16. Anterior mediastinal paraganglioma: A case for preoperative embolization

    Directory of Open Access Journals (Sweden)

    Shakir Murtaza

    2012-07-01

    Full Text Available Abstract Background Paraganglioma is a rare but highly vascular tumor of the anterior mediastinum. Surgical resection is a challenge owing to the close proximity to vital structures including the heart, trachea and great vessels. Preoperative embolization has been reported once to facilitate surgical treatment. Case presentation We report a case of anterior mediastinal paraganglioma that was embolized preoperatively, and was resected without the need for cardiopulmonary bypass and without major bleeding complications. Conclusion We make a case to further the role of preoperative embolization in the treatment of mediastinal paragangliomas.

  17. Identification of familial colorectal cancer and hereditary colorectal cancer syndromes through the Dutch population-screening program : Results ofa pilot study

    NARCIS (Netherlands)

    van Erp, Sanne J H; Leicher, Laura W; Hennink, Simone D; Ghorbanoghli, Zeinab; Breg, Simone A C; Morreau, Hans; Nielsen, Maartje; Hardwick, James C H; Roukema, J.A.; Langers, Alexandra M J; Cappel, Wouter H de Vos Tot Nederveen; Vasen, Hans F A

    2016-01-01

    OBJECTIVES: In 2014, a population-screening program using immuno-faecal occult blood testing (I-FOBT) has started in the Netherlands. The aims of this study were to evaluate the proportion of individuals in the Dutch screening program with a positive I-FOBT that fulfill the criteria for familial

  18. Screening for Psychosocial Risk in Dutch Families of a Child With Cancer: Reliability, Validity, and Usability of the Psychosocial Assessment Tool

    NARCIS (Netherlands)

    Sint Nicolaas, Simone M.; Schepers, Sasja A.; Hoogerbrugge, Peter M.; Caron, Huib N.; Kaspers, Gertjan J. L.; van den Heuvel-Eibrink, Marry M.; Grootenhuis, Martha A.; Verhaak, Chris M.

    2016-01-01

    The Psychosocial Assessment Tool (PAT) was developed to screen for psychosocial risk in families of a child diagnosed with cancer. The current study is the first describing the cross-cultural adaptation, reliability, validity, and usability of the PAT in an European country (Dutch translation).   A

  19. CONFIRMATION OF X-LINKED INHERITANCE AND PROVISIONAL MAPPING OF THE KERATOSIS FOLLICULARIS SPINULOSA DECALVANS GENE ON XP IN A LARGE DUTCH FAMILY

    NARCIS (Netherlands)

    Oosterwijk, JC; NELEN, M; VANZANDVOORT, PM; VANOSCH, LDM; ORANJE, AP; WITTEBOLPOST, D; VANOOST, BA

    In a large Dutch family with keratosis follicularis spinulosa decalvans (KFSD, MIM 308800), DNA linkage analysis was performed in order to locate the gene. Pedigree analysis and lod score calculation confirmed X-linked inheritance and revealed significant linkage to DNA markers on Xp. A maximum lod

  20. Linkage analysis in a Dutch family with X-linked recessive congenital stationary night blindness (XL-CSNB).

    Science.gov (United States)

    Berger, W; van Duijnhoven, G; Pinckers, A; Smits, A; Ropers, H H; Cremers, F

    1995-01-01

    Linkage analysis has been performed in a large Dutch pedigree with X-linked recessive congenital stationary night blindness (CSNB) by utilizing 16 DNA markers from the proximal short arm of the human X chromosome (Xp21.1-11.2). Thirteen polymorphic markers are at least partially informative and have enabled pairwise and multipoint linkage analysis. For three loci, i.e. DXS228, the monoamine oxidase B gene and the Norrie disease gene (NDG), multipoint linkage studies have yielded maximum lod scores of > 3.0 at a recombination fraction of zero. Analysis of recombination events has enabled us to rule out the possibility that the underlying defect in this family is allelic to RP3; the gene defect could also be excluded from the proximal part of the region known to carry RP2. Linkage data are consistent with a possible involvement of the NDG but mutations in the open reading frame of this gene have not been found.

  1. Nasal paraganglioma: A case report and literature review

    Directory of Open Access Journals (Sweden)

    Granato, Lídio

    2013-01-01

    Full Text Available Introduction: Paragangliomas are neuroendocrine tumors that most commonly originate in the adrenal gland, a type that is called pheochromocytoma; however, 5-10% of paragangliomas are extra-adrenal and may arise in any area between the neck and pelvic region along the sympathetic nervous system. Those located in the head and neck comprise 3% of extra-adrenal tumors, with the majority originating in the tympanic-jugular region and carotid body. Objective: To present a rare case of nasal paraganglioma and review the literature. Case report: The patient was submitted to medial subtotal maxillectomy, and her clinical findings, diagnostic data, and treatment outcome were recorded. Conclusion: Paragangliomas are considered benign tumors, but they occasionally display a malignant character. The most important finding in this case was the need for total resection of the tumor to avoid recurrence.

  2. EANM 2012 guidelines for radionuclide imaging of phaeochromocytoma and paraganglioma

    NARCIS (Netherlands)

    Taieb, D.; Timmers, H.J.L.M.; Hindie, E.; Guillet, B.A.; Neumann, H.P.; Walz, M.K.; Opocher, G.; de Herder, W.W.; Boedeker, C.C.; de Krijger, R.R.; Chiti, A.; Al-Nahhas, A.; Pacak, K.; Rubello, D.

    2012-01-01

    PURPOSE: Radionuclide imaging of phaeochromocytomas (PCCs) and paragangliomas (PGLs) involves various functional imaging techniques and approaches for accurate diagnosis, staging and tumour characterization. The purpose of the present guidelines is to assist nuclear medicine practitioners in

  3. Nuclear molecular imaging of paragangliomas; Imagerie moleculaire nucleaire des paragangliomes

    Energy Technology Data Exchange (ETDEWEB)

    Taieb, D.; Tessonnier, L.; Mundler, O. [Service central de biophysique et de medecine nucleaire, CHU de la Timone, 13 - Marseille (France)

    2010-08-15

    Paragangliomas (PGL) are relatively rare neural crest tumors originating in the adrenal medulla (usually called pheochromocytoma), chemoreceptors (i.e., carotid and aortic bodies) or autonomic ganglia. These tumors are highly vascular, usually benign and slow-growing. PGL may occur as sporadic or familial entities, the latter mostly in association with germline mutations of the succinate dehydrogenase (SDH) B, SDHC, SDHD, SDH5, von Hippel-Lindau (VHL), ret proto-oncogene (RET), neurofibromatosis 1 (NF1) (von Recklinghausen's disease), prolyl hydroxylase domain protein 2 (PHD2) genes and TMEM127. Molecular nuclear imaging has a central role in characterization of PGL and include: somatostatin receptor imaging ({sup 111}In, {sup 68}Ga), MIBG scintigraphy ({sup 131}I, {sup 123}I), {sup 18}F-dihydroxy-phenylalanine ({sup 18}F-DOPA) positron emission tomography (PET), and {sup 18}F-deoxyglucose ({sup 18}F-FDG) PET. The choice of the tracer is not yet fully established but the work-up of familial forms often require the combination of multiple approaches. (authors)

  4. Hb Nouakchott [α114(GH2)Pro→Leu; HBA1: c.344C>T], A Second and Third Case Described in Two Unrelated Dutch Families

    NARCIS (Netherlands)

    Pondman, Kirsten M.; Brinkman, Jacoline W.; van der Straaten, Hanneke M.; Stroobants, An K.; Harteveld, Cornelis L.

    2018-01-01

    We report two families, members of which are carriers of a hemoglobin (Hb) variant previously described as Hb Nouakchott [α114(GH2)Pro→Leu; HBA1: c.344C>T; p.Pro115Leu]. In the first family of Dutch origin, the proband, a 32-year-old male and his 65-year-old father, were both carriers of Hb

  5. [Pheochromocytoma and paraganglioma in childhood: a report of 2 cases report].

    Science.gov (United States)

    Mosquera Gorostidi, A; Justo Ranera, A; Zakirian Denis, S E; González Temprano, N; Sagaseta de Ilúrdoz Uranga, M; Molina Garicano, J

    2015-01-01

    Pheochromocytomas and paragangliomas are rare neuroendocrine tumors in children and most of them are sporadic. However, they represent the most common endocrine tumor in childhood, and hereditary tumor syndromes are most relevant in these age. Advances in genetic, biochemistry and imaging techniques have revised the management of these tumors; thus A biochemical study should be always initiated once the clinical diagnosis is suspected, followed by imaging and molecular studies, particularly in the context of known familial disease. The diagnostic and therapeutic features are reviewed after the presentation of two clinical cases, where the second one is a patient with type 1 Neurofibromatosis. Copyright © 2014. Published by Elsevier Espana.

  6. Hb Nouakchott [α114(GH2)Pro→Leu; HBA1: c.344C>T], A Second and Third Case Described in Two Unrelated Dutch Families.

    Science.gov (United States)

    Pondman, Kirsten M; Brinkman, Jacoline W; van der Straaten, Hanneke M; Stroobants, An K; Harteveld, Cornelis L

    2018-01-01

    We report two families, members of which are carriers of a hemoglobin (Hb) variant previously described as Hb Nouakchott [α114(GH2)Pro→Leu; HBA1: c.344C>T; p.Pro115Leu]. In the first family of Dutch origin, the proband, a 32-year-old male and his 65-year-old father, were both carriers of Hb Nouakchott. Of the second family we tested, only the proband, a 56-year-old Dutch female was a Hb Nouakchott carrier. Hematological analyses of these cases showed the anomaly behaves as a silent Hb variant without clinical consequences. The Hb variant remained unnoticed using high performance liquid chromatography (HPLC), while an additional peak was detected by capillary electrophoresis (CE). These independent findings of Hb Nouakchott indicate that this Hb variant might not be very rare, but simply remains under diagnosed depending on the Hb separation technique used.

  7. Multiple paragangliomas of head and neck associated with hepatic paraganglioma: a case report

    International Nuclear Information System (INIS)

    Xiao, Zebin; She, Dejun; Cao, Dairong

    2015-01-01

    Paragangliomas (PGs) are neuroendocrine tumors derived embryonically from the neural crest cells of the autonomic nervous system. Approximately 3 % of all paragangliomas occur in the head and neck area. Head and neck paragangliomas (HNPGs) are rare and highly vascularized tumors, the majority of which are benign. Multiple HNPGs with hepatic paraganglioma are exceedingly rare. We report a 59-year-old male patient with a 40-year history of an enlarged mass at the right side of the neck and two months of epigastric discomfort. Neck physical examination revealed a 6 × 6 cm, ovoid, firm mass on the right side of the neck. A pre-contrast computed tomography (CT) scan of the head and neck revealed bilateral heterogeneous soft tissue masses at the bifurcation of the carotid artery with indistinct border, the size of which was 2.4 cm × 2.6 cm on the left and 5.4 cm × 4.3 cm on the right. The lesions were intensely and heterogeneously enhanced with the internal and external carotid arteries surrounded and pushed anteriorly after contrast administration. Magnetic resonance imaging (MRI) showed a hyperintense signal on T2 weighted images compared to the surrounding muscle tissue and an intense contrast enhancement on T1 weighted images. Digital subtraction angiography (DSA) exhibited a highly vascularized masses that occupied and deformed both sides of the carotid bifurcation. As for the hepatic mass, non-contrasted CT imaging of the upper abdomen showed a 6.1 cm × 5.5 cm × 5.8 cm low density mass in the liver with indistinct border. On late arterial phase, the mass showed slight enhancement with an enlarged hepatic artery pushed around the lesion. MR imaging of the lesion in the liver demonstrated low signal intensity on T1 weighted images but heterogeneous high signal intensity on T2 weighted images. On diffusion weighted images, the mass showed high signal intensity whereas low signal intensity was seen on the image of apparent diffusion coefficient (ADC). Moreover

  8. Biases in the effects of family background characteristics on voting preference: The Dutch case

    NARCIS (Netherlands)

    Vries, J. de; Graaf, N.D. de; Eisinga, R.N.

    2009-01-01

    This article examines whether intergenerational transmission of party preference is biased by retrospective and other-report measurement of family background characteristics [In our model the dependent variable is voting preference, while one of our explanatory variables is father's party

  9. Speciality preferences in Dutch medical students influenced by their anticipation on family responsibilities.

    Science.gov (United States)

    Alers, Margret; Pepping, Tess; Bor, Hans; Verdonk, Petra; Hamberg, Katarina; Lagro-Janssen, Antoine

    2014-12-01

    Physician gender is associated with differences in the male-to-female ratio between specialities and with preferred working hours. We explored how graduating students' sex or full-time or part-time preference influences their speciality choice, taking work-life issues into account. Graduating medical students at Radboud University Medical Centre, the Netherlands participated in a survey (2008-2012) on career considerations. Logistic regression tested the influence of sex or working hour preference on speciality choice and whether work-life issues mediate. Of the responding students (N = 1,050, response rate 83, 73.3 % women), men preferred full-time work, whereas women equally opted for part time. More men chose surgery, more women family medicine. A full-time preference was associated with a preference for surgery, internal medicine and neurology, a part-time preference with psychiatry and family medicine. Both male and female students anticipated that foremost the career of women will be negatively influenced by family life. A full-time preference was associated with an expectation of equality in career opportunities or with a less ambitious partner whose career would affect family life. This increased the likelihood of a choice for surgery and reduced the preference for family medicine among female students. Gender specifically plays an important role in female graduates' speciality choice making, through considerations on career prospects and family responsibilities.

  10. Pheochromocytoma and Paraganglioma: Current Functional and Future Molecular Imaging

    International Nuclear Information System (INIS)

    Blanchet, Elise M.; Martucci, Victoria; Pacak, Karel

    2012-01-01

    Paragangliomas are neural crest-derived tumors, arising either from chromaffin sympathetic tissue (in adrenal, abdominal, intra-pelvic, or thoracic paraganglia) or from parasympathetic tissue (in head and neck paraganglia). They have a specific cellular metabolism, with the ability to synthesize, store, and secrete catecholamines (although most head and neck paragangliomas do not secrete any catecholamines). This disease is rare and also very heterogeneous, with various presentations (e.g., in regards to localization, multifocality, potential to metastasize, biochemical phenotype, and genetic background). With growing knowledge, notably about the pathophysiology and genetic background, guidelines are evolving rapidly. In this context, functional imaging is a challenge for the management of paragangliomas. Nuclear imaging has been used for exploring paragangliomas for the last three decades, with MIBG historically as the first-line exam. Tracers used in paragangliomas can be grouped in three different categories. Agents that specifically target catecholamine synthesis, storage, and secretion pathways include: 123 and 131I-metaiodobenzylguanidine (123/131I-MIBG), 18F-fluorodopamine (18F-FDA), and 18F-fluorodihydroxyphenylalanine (18F-FDOPA). Agents that bind somatostatin receptors include 111In-pentetreotide and 68Ga-labeled somatostatin analog peptides (68Ga-DOTA-TOC, 68Ga-DOTA-NOC, 68Ga-DOTA-TATE). The non-specific agent most commonly used in paragangliomas is 18F-fluorodeoxyglucose (18F-FDG). This review will first describe conventional scintigraphic exams that are used for imaging paragangliomas. In the second part we will emphasize the interest in new PET approaches (specific and non-specific), considering the growing knowledge about genetic background and pathophysiology, with the aim of understanding how tumors behave, and optimally adjusting imaging technique for each tumor type.

  11. Skrining Sistematik terhadap Hiperkolesterolemia Familial pada Anak Berdasarkan Kriteria MedPed, Simon Brome Register Register dan Dutch Lipid Clinic

    Directory of Open Access Journals (Sweden)

    Titis Prawitasari

    2016-11-01

    Kesimpulan. Kriteria Dutch Lipid Clinicdapat lebih banyak mendeteksi kemungkinan anak yang mengalami HF berdasarkan riwayat orangtua mengalami penyakit jantung koroner dini dan hiperkolesterolemia dibandingkan kriteria lainnya.

  12. Desmoid tumors in a dutch cohort of patients with familial adenomatous polyposis

    NARCIS (Netherlands)

    Nieuwenhuis, Marry H.; de Vos Tot Nederveen Cappel, Wouter; Botma, Akke; Nagengast, Fokko M.; Kleibeuker, Jan H.; Mathus-Vliegen, Elisabeth M. H.; Dekker, Evelien; Dees, Jan; Wijnen, Juul; Vasen, Hans F. A.

    2008-01-01

    BACKGROUND & AIMS: Desmoid tumors are a severe extracolonic manifestation in familial adenomatous polyposis (FAP). Identification of risk factors might be helpful in the management of FAP patients with such tumors. The aim of this study was to assess potential risk factors for the development of

  13. Dutch home-based pre-reading intervention with children at familial risk of dyslexia

    NARCIS (Netherlands)

    van Otterloo, S.G.; van der Leij, A.

    2009-01-01

    Children (5 and 6 years old, n = 30) at familial risk of dyslexia received a home-based intervention that focused on phoneme awareness and letter knowledge in the year prior to formal reading instruction. The children were compared to a no-training at-risk control group (n = 27), which was selected

  14. Dutch Home-Based Pre-Reading Intervention with Children at Familial Risk of Dyslexia

    Science.gov (United States)

    van Otterloo, Sandra G.; van der Leij, Aryan

    2009-01-01

    Children (5 and 6 years old, n = 30) at familial risk of dyslexia received a home-based intervention that focused on phoneme awareness and letter knowledge in the year prior to formal reading instruction. The children were compared to a no-training at-risk control group (n = 27), which was selected a year earlier. After training, we found a small…

  15. Heritability of Polycystic Ovary Syndrome in a Dutch Twin-family study

    NARCIS (Netherlands)

    Vink, J.M.; Sadrzadeh, S.; Lambalk, C.B.; Boomsma, D.I.

    2006-01-01

    Background: Polycystic ovary syndrome (PCOS) is one of the most common endocrine disorders among women of reproductive age. There is evidence for a genetic component in PCOS based on familial clustering of cases. Objective: In the present study, the heritability of PCOS was estimated.

  16. Biases in the effects of family background characteristics on voting preference : The Dutch case

    NARCIS (Netherlands)

    de Vries, J.; de Graaf, Nan Dirk; Eisinga, R.

    This article examines whether intergenerational transmission of party preference is biased by retrospective and other-report measurement of family background characteristics [In our model the dependent variable is voting preference, while one of our explanatory variables is father’s party

  17. Pathways into adulthood: a comparative study on family life transitions among migrant and Dutch youth

    NARCIS (Netherlands)

    de Valk, H.A.G.

    2006-01-01

    This study focuses on intergenerational transmission in the parental family. Socialization has received a great deal of attention in social sciences. Initially most research and theory took modern Western society as its premise and focus. More recently, the cultural context has been included by

  18. Desmoid tumors in a dutch cohort of patients with familial adenomatous polyposis

    NARCIS (Netherlands)

    Nieuwenhuis, M.H.; Vos to Nederveen Cappel, de W.; Botma, A.; Nagengast, F.M.; Kleibeuker, J.H.; Mathus-Vliegen, E.M.; Dekker, E.; Dees, J.; Wijnen, J.; Vasen, H.F.

    2008-01-01

    Background & Aims: Desmoid tumors are a severe extracolonic manifestation in familial adenomatous polyposis (FAP). Identification of risk factors might be helpful in the management of FAP patients with such tumors. The aim of this study was to assess potential risk factors for the development of

  19. Obstructing Gangliocytic Paraganglioma in the Third Portion of the Duodenum

    Directory of Open Access Journals (Sweden)

    Carlos M. Nuño-Guzmán

    2012-07-01

    Full Text Available Gangliocytic paragangliomas are infrequent tumors almost exclusively found in the second portion of the duodenum. An unusual case of a gangliocytic paraganglioma in the third portion of the duodenum with obstructive symptoms is herein reported. A 16-year-old male patient presented with epigastric pain, postprandial plenitude and reflux. A barium swallow failed to demonstrate abnormalities. Endoscopy showed a pedunculated submucosal tumor, originating at the third duodenal portion and causing partial obstruction. Biopsy was not performed due to the risk of bleeding. CT scan demonstrated a polypoid lesion. Through a transmesocolic approach and an anterior duodenotomy, resection of the tumor was performed. No lymph node or other organ affection was found. Histologic examination revealed a gangliocytic paraganglioma. Immunohistochemical examination was performed. Gangliocytic paragangliomas originating in the third or fourth portion of the duodenum, as in the present case, are extremely rare. Characteristic histologic features including epithelioid cells, spindle-shaped cells and ganglion-like cells were met. The majority of cases manifest with a similar benign behavior. Local resection of the tumor is recommended for these cases. An infrequent case of a gangliocytic paraganglioma located in the third portion of the duodenum, with a less common clinical presentation, is herein reported.

  20. Paragangliomas timpânicos: relato de casos Tympanic paragangliomas: case reports

    Directory of Open Access Journals (Sweden)

    Maria Eugênia L. R. B de V. Neto

    2005-02-01

    Full Text Available Os tumores glômicos, também chamados de paragangliomas, são formados por células não cromafins. O tumor é tipicamente vascular formado por vasos capilares e pré-capilares, interposto por células epiteliais. Para a sua abordagem inicial, deve-se ressaltar que os sintomas mais comumentes encontrados são o zumbido pulsátil e hipoacusia. A investigação através de imagem (tomografia computadorizada e ressonância magnética se faz necessária. Apresentaremos neste trabalho 5 pacientes portadores de paragangliomas timpânicos atendidos no Hospital (de 1995 a 2001. O sexo predominante foi o feminino, a idade variou de 48 a 60 anos, com média de 50 anos. A queixa predominante foi o zumbido pulsátil e a hipoacusia. A conduta foi cirúrgica em todos os casos.Glomus tumors, also called paragangliomas, originate from nonchromaffin cells. The tumor is typically vascular and grows from capillary and pre-capillary vessels in-between epithelial cells. It is worth mentioning that the most common symptoms are pulsating tinnitus and hearing loss. Imaging studies (CT and MRI are necessary for diagnosis. This paper shows five patients seen at the Hospital between 1995 and 2001 presenting glomus tympanicum. Women were most commonly affected, and the age ranged from 48 to 60 years (mean age of 50 years. The most common complaints were pulsating tinnitus and hearing loss. All patients were treated surgically.

  1. Pitfalls in genetic analysis of pheochromocytomas/paragangliomas-case report.

    Science.gov (United States)

    Canu, Letizia; Rapizzi, Elena; Zampetti, Benedetta; Fucci, Rossella; Nesi, Gabriella; Richter, Susan; Qin, Nan; Giachè, Valentino; Bergamini, Carlo; Parenti, Gabriele; Valeri, Andrea; Ercolino, Tonino; Eisenhofer, Graeme; Mannelli, Massimo

    2014-07-01

    About 35% of patients with pheochromocytoma/paraganglioma carry a germline mutation in one of the 10 main susceptibility genes. The recent introduction of next-generation sequencing will allow the analysis of all these genes in one run. When positive, the analysis is generally unequivocal due to the association between a germline mutation and a concordant clinical presentation or positive family history. When genetic analysis reveals a novel mutation with no clinical correlates, particularly in the presence of a missense variant, the question arises whether the mutation is pathogenic or a rare polymorphism. We report the case of a 35-year-old patient operated for a pheochromocytoma who turned out to be a carrier of a novel SDHD (succinate dehydrogenase subunit D) missense mutation. With no positive family history or clinical correlates, we decided to perform additional analyses to test the clinical significance of the mutation. We performed in silico analysis, tissue loss of heterozygosity analysis, immunohistochemistry, Western blot analysis, SDH enzymatic assay, and measurement of the succinate/fumarate concentration ratio in the tumor tissue by tandem mass spectrometry. Although the in silico analysis gave contradictory results according to the different methods, all the other tests demonstrated that the SDH complex was conserved and normally active. We therefore came to the conclusion that the variant was a nonpathogenic polymorphism. Advancements in technology facilitate genetic analysis of patients with pheochromocytoma but also offer new challenges to the clinician who, in some cases, needs clinical correlates and/or functional tests to give significance to the results of the genetic assay.

  2. Retroperitoneal extra-adrenal paraganglioma: a rare but important diagnosis.

    LENUS (Irish Health Repository)

    Ahmad, S

    2012-01-31

    BACKGROUND: Extra-adrenal paragangliomas of the retroperitonium are infrequently diagnosed. Their malignant behaviour cannot be predicted on initial clinical and histological assessment. These tumours have higher propensity for subsequent metastasis compared with pargangliomas at other sites. AIM: We aim to describe a case report of an incidental finding of left retroperitoneal paraganglioma in a young man who presented with right flank pain. We also aim to emphasize the importance of diagnosis and the malignant potential of these tumours. METHOD: Patient\\'s clinical notes, operative findings, imaging studies and laboratory investigations including histology results were reviewed. A literature search was done to look into the incidence, presentation, follow-up plan and treatment options for these tumours. CONCLUSION: Surgical resection is the only available curative option for extra-adrenal paragangliomas. Metastasis is observed years after surgery, hence long-term follow-up is required.

  3. Dutch home-based pre-reading intervention with children at familial risk of dyslexia

    OpenAIRE

    van Otterloo, Sandra G.; van der Leij, Aryan

    2009-01-01

    Children (5 and 6 years old, n = 30) at familial risk of dyslexia received a home-based intervention that focused on phoneme awareness and letter knowledge in the year prior to formal reading instruction. The children were compared to a no-training at-risk control group (n = 27), which was selected a year earlier. After training, we found a small effect on a composite score of phoneme awareness (d = 0.29) and a large effect on receptive letter knowledge (d  = 0.88). In first grade, however, t...

  4. A novel lamin A/C mutation in a Dutch family with premature atherosclerosis.

    Science.gov (United States)

    Weterings, A A W; van Rijsingen, I A W; Plomp, A S; Zwinderman, A H; Lekanne Deprez, R H; Mannens, M M; van den Bergh Weerman, M A; van der Wal, A C; Pinto-Sietsma, S J

    2013-07-01

    We report a novel lamin A/C (LMNA) mutation, p.Glu223Lys, in a family with extensive atherosclerosis, diabetes mellitus and steatosis hepatis. Sequence analysis of LMNA (using Alamut version 2.2), co-segregation analysis, electron microscopy, extensive phenotypic evaluation of the mutation carriers and literature comparison were used to determine the loss of function of this mutation. The father of three siblings died at the age of 45 years. The three siblings and the brother and sister of the father were referred to the cardiovascular genetics department, because of the premature atherosclerosis and dysmorphic characteristics observed in the father at autopsy. The novel LMNA mutation, p.Glu223Lys, was identified in the proband and his two sons. Clinical evaluation revealed atherosclerosis, insulin resistance and hypertension in the proband and dyslipidemia and hepatic steatosis in all the patients with the mutation. Based on the facts that in silico analysis predicts a possibly pathogenic mutation, the mutation co-segregates with the disease, only fibroblasts from mutation carriers show nuclear blebbing and a similar phenotype was reported to be due to missense mutations in LMNA we conclude that we deal with a pathogenic mutation. We conclude that the phenotype is similar to Dunnigan-type familial partial lipodystrophy. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

  5. Paraganglioma of the thyroid gland: A case report

    Directory of Open Access Journals (Sweden)

    Filipović Aleksandar

    2014-01-01

    Full Text Available Introduction. Thyroid paraganglioma is a very rare malignant neuroendocrine tumor. Immunohistochemical features of thyroid paraganglioma are helpful for the diagnosis. Case report. A 69-year-old female came to hospital with the presence of a growing thyroid nodule of the left lobe. Ultrasonic neck examination showed 5 cm hypoechoic nodule in the left thyroid lobe. Thyroid scintigraphy showed a big cold nodule in the left lobe. Computed tomography (CT scan showed left lobe thyroid tumor with tracheal deviation on the right site. Extended total thyroidectomy was done. Intraoperative consultation with the pathologist confirmed thyroid cancer. The pathologist diagnosed thyroid paraganglioma on the base of immuohistochemical investigation. This thyroid paraganglioma was positive for neuron-specific enolase, chomogranin A, synaptophysin, and S-100 protein highlighted the sustentacular cells. Tumor cells were nega-tive for thyroglobulin, epithelial membrane antigen, cytokeratin, calcitonin, and carcinoembryonic. After the surgery the patient was treated with chemotherapy, peptide receptor radionuclide therapy, and permanent TSH suppressive therapy. The patient was followed with measurements of thyroid hormone and serum neuron-specific enolase, chromogranin A level, every 6 months. Gastroscopy, colonoscopy, chest and abdomen CT scan as well as further tests (chest x-ray, ultrasound of the neck, and whole body octreotide scintigraphy were done. No primary neuroendocrine tumor in digestive sistem or in the chest was found. After more than 3 years the patient has no evidence of the recurrent disease. Conclusion. Radical resection of thyroid paraganglioma, followed by chemotherapy and peptide receptor radionuclide therapy, should be considered the treatment of choice in patients with thyroid gland paraganglioma.

  6. Dutch home-based pre-reading intervention with children at familial risk of dyslexia.

    Science.gov (United States)

    van Otterloo, Sandra G; van der Leij, Aryan

    2009-12-01

    Children (5 and 6 years old, n = 30) at familial risk of dyslexia received a home-based intervention that focused on phoneme awareness and letter knowledge in the year prior to formal reading instruction. The children were compared to a no-training at-risk control group (n = 27), which was selected a year earlier. After training, we found a small effect on a composite score of phoneme awareness (d = 0.29) and a large effect on receptive letter knowledge (d = 0.88). In first grade, however, this did not result in beneficial effects for the experimental group in word reading and spelling. Results are compared to three former intervention studies in The Netherlands and comparable studies from Denmark and Australia.

  7. Familial Aggregation between the 14th and 21st Century and Type 2 Diabetes Risk in an Isolated Dutch Population.

    Directory of Open Access Journals (Sweden)

    Kees L de Visser

    Full Text Available The development of type 2 diabetes results from an interaction of hereditary factors and environmental factors. This study aimed to investigate the contribution of interrelatedness to the risk of developing type 2 diabetes in an isolated Dutch population.A genealogical database from inhabitants living on the former island Urk between the 14th and 21st century was constructed. In a case-control study, effects of interrelatedness and the risk of type 2 diabetes were estimated with Kinship Coefficients (KCs. Relative risks in first, second, and third degree relatives and spouses of inhabitants with type 2 diabetes were compared to matched controls.Patients with type 2 diabetes were more interrelated, expressed by a higher KC compared to controls (7.2 vs. 5.2, p=0.001. First, second and third degree relatives had an increased risk of developing type 2 diabetes. Second degree relatives had a similar risk,1.7 (1.5-2.0 as third degree relatives,1.8 (1.5-2.2. Spouses of patients with diabetes had a 3.4 (2.7-4.4 higher risk of developing type 2 diabetes.Interrelatedness was higher among inhabitants with type 2 diabetes compared to controls. This differences extended beyond the nuclear family, thereby supporting the hypothesis that interrelatedness contributed to the development of type 2 diabetes on Urk. However, the size of this effect was small and the patterns of risk in first, second and third degree relatives suggested that factors other than interrelatedness were the main contributors to the development of type 2 diabetes on Urk.

  8. Paraganglioma of the larynx. A case report and clinical review.

    Science.gov (United States)

    Shipton, E A; van der Linde, J C

    1984-02-04

    An 8-year-old Black boy recently underwent surgery for the excision of a non-chromaffin laryngeal paraganglioma. The peri-operative management of this patient is presented. The specific problems involved and their importance to the anaesthesiologist and surgeon alike are discussed.

  9. Clinical application of preoperative endovascular management for jugular paraganglioma

    International Nuclear Information System (INIS)

    Yu Juming; Fan Guoping; Zhong Weixing; Zhang Yongping; Peng Haiteng; Cheng Yongde

    2009-01-01

    Objective: To investigate the clinical value of preoperative angiography and embolization managements for jugular paraganglioma. Methods: Fourteen patients with jugular paraganglioma were carefully evaluated with CT, MRI and clinical ENT exams. Bilateral carotid and affected-side vertebral angiography together with embolization of the feeding arteries and tumor nidi were performed in all 14 patients before surgery. Internal carotid artery balloon occlusive test was employed to check the function of Willis' circle in 7 patients. The tumors were excised within 48 hours after embolization. Results: Preoperative angiographic and embolization procedures of jugular paraganglioma were successfully accomplished in all patients. The mean blood loss during the surgery was obviously less than usual. Of seven cases who passed the internal carotid artery balloon occlusive test,carotid artery ligation was adopted in 3. No new symptoms and signs of nervous system developed after the surgery and during the follow-up period. Conclusion: The angiography and embolization of feeding-arteries and tumor nidi, and the preoperative balloon occlusive test of carotid artery performed before the surgery of jugular paraganglioma are safe and reliable, which can be regarded as a routine preoperative preparation. (authors)

  10. Functional extra-adrenal paraganglioma of the retroperitoneum ...

    African Journals Online (AJOL)

    Functional extra-adrenal paraganglioma of the retroperitoneum giving thoracolumbar spine metastases after a five-year disease-free follow-up: a rare malignant condition with challenging management. Stylianos Kapetanakis, Danai Chourmouzi, Grigorios Gkasdaris, Vasileios Katsaridis, Eleftherios Eleftheriadis, Panagiotis ...

  11. SOLITARY PARAGANGLIOMA OF THE HYPOGLOSSAL NERVE: CASE REPORT.

    LENUS (Irish Health Repository)

    2012-02-01

    SOLITARY PARAGANGLIOMA OF THE HYPOGLOSSAL NERVE:: Case Report BACKGROUND AND IMPORTANCE:: We report the case history of solitary hypoglossal paraganglioma in a 64-year-old woman. The surgical difficulties encountered in the removal of this challenging tumour are discussed with literature review. CLINICAL PRESENTATION:: A 64-year-old woman presented with a short history of dysphonia, occasional dysphagia, tinnitus, altered taste, and unilateral left sided tongue wasting. On examination there was left lower motor hypoglossal paralysis. Imaging showed a discrete enhancing lobulated mass, measuring 2cm x 2cm, in the region of the hypoglossal nerve extending into the hypoglossal canal suggestive of hypoglossal paraganglioma. A left dorsolateral sub occipital craniotomy was carried out in the sitting position. The hypoglossal nerve appeared to be enlarged and the jugular foramen was normal. Complete surgical debulking of the tumour was not attempted due to its vascular nature. The nerve was decompressed and neuropathology confirmed a low grade paraganglioma arising from the hypoglossal nerve. The patient is scheduled to receive stereotactic radiation for further management. CONCLUSION:: When a case of solitary hypoglossal paraganglioma is encountered in clinical practice, the aim of management should be mainly focussed on achieving a diagnosis and preserving the hypoglossal nerve function. If there is evidence of vascularity in the lesion noted in the MRI scan, a pre-operative angiogram should be performed with a view for embolisation.We decompressed the hypoglossal canal and achieved a good improvement in the patient\\'s symptoms. We recommend stereotactic radiosurgery for remnant and small hypoglossal tumours and regular follow up with MRI scans.

  12. SOLITARY PARAGANGLIOMA OF THE HYPOGLOSSAL NERVE: CASE REPORT.

    LENUS (Irish Health Repository)

    Raza, Kazim

    2011-01-25

    SOLITARY PARAGANGLIOMA OF THE HYPOGLOSSAL NERVE:: Case Report BACKGROUND AND IMPORTANCE:: We report the case history of solitary hypoglossal paraganglioma in a 64-year-old woman. The surgical difficulties encountered in the removal of this challenging tumour are discussed with literature review. CLINICAL PRESENTATION:: A 64-year-old woman presented with a short history of dysphonia, occasional dysphagia, tinnitus, altered taste, and unilateral left sided tongue wasting. On examination there was left lower motor hypoglossal paralysis. Imaging showed a discrete enhancing lobulated mass, measuring 2cm x 2cm, in the region of the hypoglossal nerve extending into the hypoglossal canal suggestive of hypoglossal paraganglioma. A left dorsolateral sub occipital craniotomy was carried out in the sitting position. The hypoglossal nerve appeared to be enlarged and the jugular foramen was normal. Complete surgical debulking of the tumour was not attempted due to its vascular nature. The nerve was decompressed and neuropathology confirmed a low grade paraganglioma arising from the hypoglossal nerve. The patient is scheduled to receive stereotactic radiation for further management. CONCLUSION:: When a case of solitary hypoglossal paraganglioma is encountered in clinical practice, the aim of management should be mainly focussed on achieving a diagnosis and preserving the hypoglossal nerve function. If there is evidence of vascularity in the lesion noted in the MRI scan, a pre-operative angiogram should be performed with a view for embolisation.We decompressed the hypoglossal canal and achieved a good improvement in the patient\\'s symptoms. We recommend stereotactic radiosurgery for remnant and small hypoglossal tumours and regular follow up with MRI scans.

  13. Magnetic resonance imaging findings of a nonfunctional mediastinal paraganglioma with an unusual presentation

    International Nuclear Information System (INIS)

    Sahin-Akyar, G.; Erden, I.; Yagci, C.; Akyar, S.; Erekul, S.

    1997-01-01

    A case of histologically proven mediastinal paraganglioma presenting with metastatic spread to supraclavicular lymph nodes is presented. Mediastinal paragangliomas are extremely rare tumors and their CT and MR imaging features have not been well documented in the radiologic literature. The T 1-weighted and T 2-weighted spin-echo MR findings of a locally invasive mediastinal paraganglioma with metastasis to the supraclavicular lymph nodes are described and the literature is briefly reviewed. (orig.). With 4 figs

  14. Unravelling fears of genetic discrimination: an exploratory study of Dutch HCM families in an era of genetic non-discrimination acts.

    Science.gov (United States)

    Geelen, Els; Horstman, Klasien; Marcelis, Carlo L M; Doevendans, Pieter A; Van Hoyweghen, Ine

    2012-10-01

    Since the 1990s, many countries in Europe and the United States have enacted genetic non-discrimination legislation to prevent people from deferring genetic tests for fear that insurers or employers would discriminate against them based on that information. Although evidence for genetic discrimination exists, little is known about the origins and backgrounds of fears of discrimination and how it affects decisions for uptake of genetic testing. The aim of this article is to gain a better understanding of these fears and its possible impact on the uptake of testing by studying the case of hypertrophic cardiomyopathy (HCM). In a qualitative study, we followed six Dutch extended families involved in genetic testing for HCM for three-and-a-half years. Semi-structured interviews were conducted with 57 members of these families. Based on the narratives of the families, we suggest that fears of discrimination have to be situated in the broader social and life-course context of family and kin. We describe the processes in which families developed meaningful interpretations of genetic discrimination and how these interpretations affected family members' decisions to undergo genetic testing. Our findings show that fears of genetic discrimination do not so much stem from the opportunity of genetic testing but much more from earlier experiences of discrimination of diseased family members. These results help identify the possible limitations of genetic non-discrimination regulations and provide direction to clinicians supporting their clients as they confront issues of genetic testing and genetic discrimination.

  15. Walk-in homes for people living with cancer and their family members: A new Dutch communication and support approach

    NARCIS (Netherlands)

    A. Visser; A. van den Brom; R. den Hollander

    2015-01-01

    Dutch walk-in homes are a meeting place for people suffering from cancer and for their loved ones. A study was performed in order to assess to what extent the offered complementary activities and the psycho-social support in these houses are, as well as whether or not these homes are present,

  16. Walk-in homes for people living with cancer and their family members: A new Dutch communication and support approach

    OpenAIRE

    Visser, A.; Brom, van den, A.; Hollander, den, R.

    2015-01-01

    Dutch walk-in homes are a meeting place for people suffering from cancer and for their loved ones. A study was performed in order to assess to what extent the offered complementary activities and the psycho-social support in these houses are, as well as whether or not these homes are present, comparable to initiatives in other countries.

  17. Familial Aggregation between the 14th and 21st Century and Type 2 Diabetes Risk in an Isolated Dutch Population

    NARCIS (Netherlands)

    de Visser, Kees L.; Landman, Gijs W. D.; Meyboom-de Jong, Betty; de Visser, Wim; te Meerman, Gerard J.; Bilo, Henk J. G.

    2015-01-01

    Introduction The development of type 2 diabetes results from an interaction of hereditary factors and environmental factors. This study aimed to investigate the contribution of interrelatedness to the risk of developing type 2 diabetes in an isolated Dutch population. Materials and Methods A

  18. Malignant paraganglioma with vertebral metastasis: case report Paraganglioma maligno com metástase vertebral: relato de caso

    Directory of Open Access Journals (Sweden)

    Bruno Lázaro

    2003-06-01

    Full Text Available A paraganglioma is a rare tumor, composed of chromaffin cells, groups of cells associated to the autonomous system. When the tumor occurs in the adrenal gland, it is called pheochromocitoma. The malignant paraganglioma is a very rare presentation; it is diagnosed by local recurrence after total resection of the primary mass, or findings of distant metastases. We present a case report of a 29-year-old woman with cervico-brachial pain. In 1995 she underwent a carotid body tumor resection. Magnetic resonance imaging (MRI, plain X-rays and computerized tomography scan revealed multiple lesions in C5, T5 and T12. She underwent a surgical procedure to correct the cervical lesion. The histological and immunohistochemical assays revealed a malignant paraganglioma. She received adjuvant radiotherapy, showing clinical improvement after treatment, presenting no symptoms after one year. The therapeutic approach is based on the total resection of the tumor. The treatment of distant metastases can be made with adjuvant measures such as conventional radiotherapy, I¹³¹-MIBG, or chemotherapy, especially in malignant pheochromocitomas.O paraganglioma é tumor raro, composto de células cromafins, associado ao sistema nervoso autônomo. Quando localizado na glândula supra-renal, o tumor é chamado feocromocitoma. Descreve-se um caso de paciente do sexo feminino, 29 anos, que se apresentou com cervicobraquialgia e que havia sido operada em 1995 para exérese de tumor glômico da carótida cervical. RM, RX e TC revelaram múltiplas lesões acometendo o corpo vertebral de C5, T5 e T12. Foi submetida à ressecção cirúrgica radical da lesão cervical, com substituição do corpo vertebral por prótese de titânio. A histopatologia e o estudo imunohistoquímico da lesão confirmaram o diagnóstico de paraganglioma maligno. As outras lesões foram tratadas com radioterapia. Um ano após os procedimentos, a paciente apresenta-se assintomática. O tratamento destas

  19. Retroperitoneal Paraganglioma – a rare cause of arterial hypertension

    Directory of Open Access Journals (Sweden)

    Isabel Silva

    2017-03-01

    Full Text Available Paragangliomas are rare neuroendocrine tumours, most commonly found in adults. These differ from pheochromocytomas in that their location is extra-adrenal, and they are responsible for about 1% of arterial hypertension aetiologies. We report the case of a 30-year-old female whose past medical history was unremarkable. However, her arterial hypertension led to further examination in search of secondary aetiologies, in which a retroperitoneal mass and an increase in levels of catecholamines were detected; findings that led to the final diagnosis of paraganglioma. A multidisciplinary team, whose approach was to use pharmacological alpha-adrenergic blocking agents and a surgical resection of the lesion, treated the patient. The patient is clinically well but will continue to be monitored as an outpatient, and genetic testing is being encouraged.

  20. Successful Removal of Giant Intrapericardial Paraganglioma via Posterolateral Thoracotomy

    Directory of Open Access Journals (Sweden)

    Yoko Yamamoto

    2014-01-01

    Full Text Available Intrapericardial paraganglioma remains a surgical challenge because of its hypervascular nature and firm adhesion to adjacent mediastinal structures. Here, we describe a 63-year-old female with a giant nonfunctioning intrapericardial paraganglioma tightly adhered to the left atrium. Marginal but complete resection of the tumor was achieved via right posterolateral thoracotomy. At the time of dissection between the tumor and the left atrial wall, we encountered massive hemorrhage leading to cardiac arrest. We were able to repair the wall laceration with minimal time under an optimal operative field, which avoids air embolism. She was discharged without complications and is currently in good health with no recurrence or metastasis for 15 months. Based on our experience, cardiopulmonary bypass should be considered, if surgeons are able to secure suitable sites for arterial and venous cannulations while right posterolateral thoracotomy is employed.

  1. Solitary paraganglioma of the hypoglossal nerve: case report.

    LENUS (Irish Health Repository)

    Raza, Kazim

    2011-04-01

    BACKGROUND AND IMPORTANCE: We report the case history of solitary hypoglossal paraganglioma in a 64-year-old woman. The surgical difficulties encountered in the removal of this challenging tumor are discussed and as a literature review provided. CLINICAL PRESENTATION: A 64-year-old woman presented with a short history of dysphonia, occasional dysphagia, tinnitus, altered taste, and unilateral left-sided tongue wasting. On examination, there was left lower motor hypoglossal paralysis. Imaging showed a discrete enhancing lobulated mass, measuring 2 × 2 cm, in the region of the hypoglossal nerve extending into the hypoglossal canal suggestive of hypoglossal paraganglioma. A left dorsolateral suboccipital craniotomy was performed with the patient in the sitting position. The hypoglossal nerve appeared to be enlarged, and the jugular foramen was normal. Complete surgical debulking of the tumor was not attempted because of its vascular nature. The nerve was decompressed, and neuropathology confirmed a low-grade paraganglioma arising from the hypoglossal nerve. The patient was scheduled to receive stereotactic radiation for further management. CONCLUSION: When a case of solitary hypoglossal paraganglioma is encountered in clinical practice, the aim of management should be mainly focused on achieving a diagnosis and preserving the hypoglossal nerve function. If there is evidence of vascularity in the lesion noted on magnetic resonance imaging, a preoperative angiogram should be obtained with a view for embolization. We decompressed the hypoglossal canal and achieved good improvement in the patient\\'s symptoms. We recommend stereotactic radiosurgery for remnant and small hypoglossal tumors and regular follow-up with magnetic resonance imaging scans.

  2. Solitary paraganglioma of the hypoglossal nerve: case report.

    LENUS (Irish Health Repository)

    Raza, Kazim

    2012-02-01

    BACKGROUND AND IMPORTANCE: We report the case history of solitary hypoglossal paraganglioma in a 64-year-old woman. The surgical difficulties encountered in the removal of this challenging tumor are discussed and as a literature review provided. CLINICAL PRESENTATION: A 64-year-old woman presented with a short history of dysphonia, occasional dysphagia, tinnitus, altered taste, and unilateral left-sided tongue wasting. On examination, there was left lower motor hypoglossal paralysis. Imaging showed a discrete enhancing lobulated mass, measuring 2 x 2 cm, in the region of the hypoglossal nerve extending into the hypoglossal canal suggestive of hypoglossal paraganglioma. A left dorsolateral suboccipital craniotomy was performed with the patient in the sitting position. The hypoglossal nerve appeared to be enlarged, and the jugular foramen was normal. Complete surgical debulking of the tumor was not attempted because of its vascular nature. The nerve was decompressed, and neuropathology confirmed a low-grade paraganglioma arising from the hypoglossal nerve. The patient was scheduled to receive stereotactic radiation for further management. CONCLUSION: When a case of solitary hypoglossal paraganglioma is encountered in clinical practice, the aim of management should be mainly focused on achieving a diagnosis and preserving the hypoglossal nerve function. If there is evidence of vascularity in the lesion noted on magnetic resonance imaging, a preoperative angiogram should be obtained with a view for embolization. We decompressed the hypoglossal canal and achieved good improvement in the patient\\'s symptoms. We recommend stereotactic radiosurgery for remnant and small hypoglossal tumors and regular follow-up with magnetic resonance imaging scans.

  3. Hypertension secondary to a periprostatic paraganglioma: case report and review of the literature

    NARCIS (Netherlands)

    Kers, Jesper; Choudhry, Zaheeb A.; Roeleveld, Ton A.; Houdijk, Alexander P. J.

    2013-01-01

    Around 10 per cent of catecholamine-secreting tumours can be found outside the adrenal medulla (paraganglioma). We report a case of a functional sporadic paraganglioma that was localized lateral to the prostate without causing lower urinary tract symptoms. A 76-year old male with an extensive

  4. Paraganglioma with intracranial metastasis: a case report and review of the literature.

    Science.gov (United States)

    Cai, Peihao; Mahta, Ali; Kim, Ryan Y; Kesari, Santosh

    2012-10-01

    Paragangliomas are rare neuroendocrine tumors of neural crest origin. They are mostly benign, however; malignant tumors with aggressive behavior and distant metastasis can also occur. Intracranial involvement is extremely rare and has been sporadically reported in the literature. Here we report a case who presented with progressive neurologic deficits due to multiple intracranial lesions found to be metastasis from an occult retroperitoneal malignant paraganglioma.

  5. Jugular and vagal paragangliomas: Systematic study of management with surgery and radiotherapy

    NARCIS (Netherlands)

    Suarez, C.; Rodrigo, J.P.; Bodeker, C.C.; Llorente, J.L.; Silver, C.E.; Jansen, J.C.; Takes, R.P.; Strojan, P.; Pellitteri, P.K.; Rinaldo, A.; Mendenhall, W.M.; Ferlito, A.

    2013-01-01

    BACKGROUND: The definitive treatment for head and neck paraganglioma (PG) is surgical excision. Unfortunately, surgery, particularly of vagal paraganglioma (VPG; "glomus vagale") and foramen jugulare ("glomus jugulare") tumors, may be complicated by injuries to the lower cranial nerves, a high price

  6. Melanotic paraganglioma arising in the temporal horn following Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Yoo, Jeong Hyun; Rivera, Andreana; Naeini, Ramin M.; Yedururi, Sireesha; Megahead, Hatem; Bayindir, Petek; Fuller, Gregory N.; Suh, Jeong Soo; Adesina, Adekunle M.; Hunter, Jill V.

    2008-01-01

    Intracerebral paragangliomas are rare because of the lack of paraganglial cells in the cerebral tissue. We report a rare case of melanotic paraganglioma arising from the temporal horn of the lateral ventricle in a patient with prior Langerhans cell histiocytosis (LCH) treated with chemotherapy and radiation. (orig.)

  7. Imaging findings of a primary paraganglioma of the liver: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Ji, Seung Woo; Kang, Ung Rae; Park, Jae Bok [Daegu Catholic University Medical Center, Catholic University of Daegu College of Medicine, Daegu (Korea, Republic of)

    2016-11-15

    Primary hepatic paraganglioma is an extremely rare type of tumor originating from extra-adrenal chromaffin cells. We report a case of primary intrahepatic paraganglioma in a 52-year-old man, with pathologic confirmation through right hepatectomy. An imaging study indicated a predominately hemorrhagic septated cystic mass and peripheral marked enhancement of the solid portions, which showed persistent enhancement.

  8. Long-term results of irradiation for paraganglioma

    International Nuclear Information System (INIS)

    Krych, Aaron J.; Foote, Robert L.; Brown, Paul D.; Garces, Yolanda I.; Link, Michael J.

    2006-01-01

    Purpose: The management of paragangliomas is controversial. Observation, surgery, external-beam radiotherapy (EBRT), and stereotactic radiosurgery (SRS) may, alone or in combination, be appropriate, depending on the size and extent of the tumor, previous treatment, and patient age, general health, and neurologic condition. Few data exist regarding long-term tumor control and late effects after EBRT or SRS. Methods and Materials: We performed a retrospective review of all patients treated with EBRT or SRS for paraganglioma at our institution between 1967 and 1994. The endpoints of the study were tumor control and late complications. Results: The 33 patients in this study had a median follow-up of 13 years (range, 4 months to 36 years). The 10-year tumor control rate was 92% (95% confidence interval, 75-98%). At the last follow-up visit, no patient had developed a radiation-induced malignancy. Conclusion: External-beam RT and SRS are safe and effective for enlarging and/or symptomatic paragangliomas. The risk of developing a delayed radiation-induced malignancy after EBRT or SRS is low. This risk must be weighed against the significant immediate and permanent risk of cranial nerve deficits if the tumor is untreated or is surgically resected. This risk must also be weighed against the immediate but low risk of surgical mortality

  9. Large gangliocytic paraganglioma of the duodenum: A rare entity.

    Science.gov (United States)

    Hernández, Alejandra Gordillo; Lanuza, Eduardo Dominguez-Adame; Matias, Auxiliadora Cano; Huertas, Rosario Perez; Rodriguez, Katherine Maria Gallardo; Perez, Purificacion Gallinato; Mompean, Fernando Oliva

    2015-08-27

    Gangliocytic paragangliomas are rare tumors that almost exclusively occur within the second portion of the duodenum. Although these tumors generally have a benign clinical course, they have the potential to recur or metastasize to regional lymph nodes. The case report presented here describes a 57-year-old female patient with melena, progressive asthenia, anemia, and a mass in the second-third portion of the duodenum that was treated by local excision. The patient was diagnosed with a friable bleeding tumor. The histologic analysis showed that the tumor was a 4 cm gangliocytic paraganglioma without a malignant cell pattern. In the absence of local invasion or distant metastasis, endoscopic resection represents a feasible, curative therapy. Although endoscopic polypectomy is currently considered the treatment of choice, it is not recommended if the size of the tumor is > 3 cm and/or there is active or recent bleeding. Patients diagnosed with a gangliocytic paraganglioma should be closely followed-up for possible local recurrence.

  10. Unrecognized paraganglioma of the urinary bladder as a cause for basilar-type migraine.

    Science.gov (United States)

    Pichler, Renate; Heidegger, Isabel; Klinglmair, Gerald; Kroiss, Alexander; Uprimny, Christian; Gasser, Rudolf Wolfgang; Schäfer, Georg; Steiner, Hannes

    2014-01-01

    Extra-adrenal paraganglioma with isolated localization in the urinary bladder is a rare neuroendocrine tumor. Although the typical symptoms like headache, nausea, weight loss, flushing, heart palpitation or paroxysmal hypertension during micturition are well established, we present an unusual case of bladder paraganglioma, 'misdiagnosed' with basilar-type migraine due to headache for the past 8 years. As urologists linked the presence of a tumor (by CT) and symptoms connected with micturition, no cystoscopy and no transurethral resection of the bladder was performed prior to detailed diagnostic workup. After diagnosis of an extra-adrenal paraganglioma, the patient was scheduled for open partial cystectomy. In consideration of the fact that bladder paraganglioma is an infrequent genitourinary cancer, this case report clearly points out the importance of an exact anamnesis and clinical examination to minimize the probability of misdiagnosis with possible fatal consequences in any case with clinical suspicion of bladder paraganglioma. Copyright © 2013 S. Karger AG, Basel.

  11. Successful chemotherapy of hepatic metastases in a case of succinate dehydrogenase subunit B-related paraganglioma.

    Science.gov (United States)

    He, J; Makey, D; Fojo, T; Adams, K T; Havekes, B; Eisenhofer, G; Sullivan, P; Lai, E W; Pacak, K

    2009-10-01

    Compared to other familial pheochromocytoma/paragangliomas (PHEO/PGLs), the succinate dehydrogenase subunit B (SDHB)-related PHEO/PGLs often present with aggressive and rapidly growing metastatic lesions. Currently, there is no proven effective treatment for malignant PHEO/PGLs. Here, we present a 35-year-old white man with primary malignant abdominal extra-adrenal 11 cm paraganglioma underwent surgical successful resection. But 6 months later, he developed extensive bone, liver, and lymph nodes metastasis, which were demonstrated by computed tomography scan and the (18)F-fluorodeoxyglucose positron emission tomography. However, his (123)I-metaiodobenzylguanidine scintigraphy was negative; therefore, the cyclophosphamide, vincristine, and dacarbazine (CVD) combination chemotherapy was initiated. The combination chemotherapy was very effective showing 80% overall reduction in the liver lesions and 75% overall reduction in the retroperitoneal mass and adenopathy, and normalization of plasma catecholamine and metanephrine levels. However, plasma levels of dopamine (DA) and methoxytyramine (MTY) were only partially affected and remained consistently elevated throughout the remaining period of follow-up evaluation. Genetic testing revealed an SDHB gene mutation. Here, we present an SDHB-related PHEO/PGL patient with extensive tumor burden, numerous organ lesions, and rapidly growing tumors, which responded extremely well to CVD therapy. We conclude patients with SDHB-related PHEO/PGLs can be particularly sensitive to CVD chemotherapy and may have an excellent outcome if this therapy is used and continued on periodic basis. The data in this patient also illustrate the importance of measuring plasma levels of DA and MTY to provide a more complete and accurate assessment of the biochemical response to therapy than provided by measurements restricted to other catecholamines and O-methylated metabolites.

  12. Devascularization of Head and Neck Paragangliomas by Direct Percutaneous Embolization

    International Nuclear Information System (INIS)

    Ozyer, Umut; Harman, Ali; Yildirim, Erkan; Aytekin, Cuneyt; Akay, Tankut Hakki; Boyvat, Fatih

    2010-01-01

    Preoperative transarterial embolization of head and neck paragangliomas using particulate agents has proven beneficial for decreasing intraoperative blood loss. However, the procedure is often incomplete owing to extensive vascular structure and arteriovenous shunts. We report our experience with embolization of these lesions by means of direct puncture and intratumoral injection of n-butyl cyanoacrylate (NBCA) or Onyx. Ten patients aged 32-82 years who were referred for preoperative embolization of seven carotid body tumors and three jugular paragangliomas were retrospectively analyzed. Intratumoral injections were primarily performed in four cases with multiple small-caliber arterial feeders and adjunctive to transarterial embolization in six cases with incomplete devascularization. Punctures were performed under ultrasound and injections were performed under roadmap fluoroscopic guidance. Detailed angiographies were performed before and after embolization procedures. Control angiograms showed complete or near-complete devascularization in all tumors. Three tumors with multiple small-caliber arterial feeders were treated with primary NBCA injections. One tumor necessitated transarterial embolization after primary injection of Onyx. Six tumors showed regional vascularization from the vasa vasorum or small-caliber branches of the external carotid artery following the transarterial approach. These regions were embolized with NBCA injections. No technical or clinical complications related to embolization procedures occurred. All except one of the tumors were surgically removed following embolization. In conclusion, preoperative devascularization with percutaneous direct injection of NBCA or Onyx is feasible, safe, and effective in head and neck paragangliomas with multiple small-caliber arterial feeders and in cases of incomplete devascularization following transarterial embolization.

  13. Paraganglioma of the Cauda Equina Presenting with Erectile and Sphincter Dysfunction

    Directory of Open Access Journals (Sweden)

    Wiesław Marcol

    2009-06-01

    Full Text Available Paragangliomas of the cauda equina are rare neuroepithelial tumors, usually manifesting clinically as sciatica. Here, we report a case of cauda equina paraganglioma with an unusual course in a 43-year-old man. His main complaints were erectile and sphincter dysfunction. The low back pain was initially ascribed to accidental injury. Magnetic resonance imaging revealed intradural tumor at the L2/L3 level. The patient underwent gross tumor resection, and the diagnosis of paraganglioma was based on neuropathologic examination. The symptoms completely resolved after tumor resection.

  14. Extra-adrenal malignant paragangliomas presenting as mesenteric and pararectal masses: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Sun Hye [Dept. of Radiology, Korea University Anam Hospital, Korea University College of Medicine, Seoul (Korea, Republic of); Lee, Jong Mee; Kim, Baek Hui; Kim, Kyeong Ah; Park, Cheol Min [Korea University Guro Hospital, Korea University College of Medicine, Seoul (Korea, Republic of)

    2017-07-15

    Extra-adrenal paraganglioma is a rare tumor arising from the neural crest cells. Most tumors that develop in the abdomen arise from paraganglia along the paravertebral and para-aortic areas, in particular the organ of Zuckerkandl, which is close to the origin of the inferior mesenteric artery. However, extra-adrenal paraganglioma also occurs in relatively rare places such as the urinary bladder, gallbladder, hepatoduodenal ligament, and gastrointestinal tract. Here, we report imaging findings of extra-adrenal paragangliomas presenting as mesenteric and pararectal masses with lymph node metastasis.

  15. Carotid body paraganglioma metastatic to bone: report of two cases

    International Nuclear Information System (INIS)

    Kawai, A.; Healey, J.H.; Wilson, S.C.; Huvos, A.G.; Yeh, S.D.J.

    1998-01-01

    Two patients with carotid body paraganglioma developed bone metastases 3 and 6 years respectively after surgical excision of the primary tumors. Plain radiographs showed ill-defined metastatic lesions. Scintigram using radiolabeled metaiodobenzylguanidine, an analogue of noradrenaline that is taken up by neurosecretary granules, showed an abnormal accumulation in the corresponding metastatic lesion. Histologically, nests of epithelioid cells with clear cytoplasm and pyknotic nuclei and abundant collagen fibers were observed within destroyed trabeculae. Treatment including external radiation and surgery provided pain relief and early local disease control. (orig.)

  16. Family orientation, strategy and organizational learning as predictors of knowledge management in Dutch SMEs : A test of lagged effects

    NARCIS (Netherlands)

    Uhlaner, L.M.; Tan, S.; Meijaard, J.

    2007-01-01

    Past research suggests a negative effect of family orientation on innovation performance. However, many past studies have certain limitations that this study is designed to overcome. In particular,this study estimates lagged effects of family orientation on innovation performance while controlling

  17. Addressing risk factors for child abuse among high risk pregnant women: design of a randomised controlled trial of the nurse family partnership in Dutch preventive health care

    Directory of Open Access Journals (Sweden)

    Mejdoubi Jamila

    2011-10-01

    Full Text Available Abstract Background Low socio-economic status combined with other risk factors affects a person's physical and psychosocial health from childhood to adulthood. The societal impact of these problems is huge, and the consequences carry on into the next generation(s. Although several studies show these consequences, only a few actually intervene on these issues. In the United States, the Nurse Family Partnership focuses on high risk pregnant women and their children. The main goal of this program is primary prevention of child abuse. The Netherlands is the first country outside the United States allowed to translate and culturally adapt the Nurse Family Partnership into VoorZorg. The aim of the present study is to assess whether VoorZorg is as effective in the Netherland as in the United States. Methods The study consists of three partly overlapping phases. Phase 1 was the translation and cultural adaptation of Nurse Family Partnership and the design of a two-stage selection procedure. Phase 2 was a pilot study to examine the conditions for implementation. Phase 3 is the randomized controlled trial of VoorZorg compared to the care as usual. Primary outcome measures were smoking cessation during pregnancy and after birth, birth outcomes, child development, child abuse and domestic violence. The secondary outcome measure was the number of risk factors present. Discussion This study shows that the Nurse Family Partnership was successfully translated and culturally adapted into the Dutch health care system and that this program fulfills the needs of high-risk pregnant women. We hypothesize that this program will be effective in addressing risk factors that operate during pregnancy and childhood and compromise fetal and child development. Trial registration Current Controlled Trials ISRCTN16131117

  18. Is the c.3G>C mutation in the succinate dehydrogenase subunit D (SDHD) gene due to a founder effect in Chinese head and neck paraganglioma patients?

    Science.gov (United States)

    Zha, Yang; Chen, Xing-ming; Lam, Ching-wan; Lee, Soo-chin; Tong, Sui-fan; Gao, Zhi-qiang

    2011-08-01

    Three Chinese patients with head and neck paragangliomas have been reported to carry the c.3G>C mutation in the succinate dehydrogenase subunit D (SDHD) gene. In addition, in our hospital, two further patients were identified who have the same mutation. It is unclear whether the c.3G>C mutation in Chinese patients is a recurrent mutation or if it is due to a founder effect. We conducted haplotype analysis on these patients to answer this question. Individual case-control study. Germ-line mutations were confirmed in the patients and their families examined in this study using direct sequencing. We also constructed and analyzed haplotypes in four Chinese families. Genotype frequencies were compared to the control group. Three of four families shared the same haplotype, which rarely occurred in the control group. The last family shared a very short area on the physical map with the other three families. There is a founder effect in Chinese head and neck paraganglioma patients carrying the SDHD c.3G>C mutation. Copyright © 2011 The American Laryngological, Rhinological, and Otological Society, Inc.

  19. Comparing quality of dying and death perceived by family members and nurses for patients dying in US and Dutch ICUs

    DEFF Research Database (Denmark)

    Gerritsen, Rik T; Koopmans, Matty; Hofhuis, José G M

    2017-01-01

    with 8 higher in the US and 2 higher in NL. CONCLUSION: The QODD was rated similarly by family members in the US and the Netherlands but varied when assessed by nurses. These differences may be due to organizational or cultural differences between the two countries or to expectations of respondents.......BACKGROUND: The Quality of Dying and Death (QODD) questionnaire is used as a self-reported measure to allow families and clinicians to assess patients' quality of dying and death. We evaluated end-of-life (EOL) experiences as measured by the QODD completed by families and nurses in the United...... States (US) and the Netherlands (NL) to explore similarities and differences in these experiences and identify opportunities for improving EOL care. METHODS: Questionnaire data were gathered from family members of patients dying in the ICU and nurses caring for these patients. In NL, data were gathered...

  20. Results of a systematic literature review of treatment modalities for jugulotympanic paraganglioma, stratified per Fisch class

    NARCIS (Netherlands)

    Jansen, T.T.G.; Timmers, H.J.L.M.; Marres, H.A.M.; Kaanders, J.H.A.M.; Kunst, H.P.M.

    2018-01-01

    OBJECTIVE: Key for successful jugulotympanic paraganglioma management is a personalised approach aiming for the best practice for each individual patient. To this end, a systematic review is performed, evaluating the local control and complication rates for the different treatment modalities

  1. Multiple Recurrent Paraganglioma in a Pediatric Patient with Germline SDH-B Mutation

    Directory of Open Access Journals (Sweden)

    Aidan McGowan

    2017-07-01

    Full Text Available Magnetic Resonance Imaging (MRI and fluoro-2-deoxy-d-glucose positron emission tomography (FDG-PET are recognized approaches for locating paragangliomas. Recently, gallium-68 DOTA-octreotate (DOTATATE scans have shown promise detecting neuroendocrine tumors missed by FDG-PET and MRI. 13-year-old male with SDH-B mutation presented with symptoms of paraganglioma and elevated catecholamines. MRI did not demonstrate the T2 hyper intense signal typical of paraganglioma and pheochromocytoma; FDG-PET scan did not reveal increased foci of uptake. DOTATATE scan revealed a signal consistent only with residual adrenal tissue. Resection of the right adrenal bed revealed paraganglioma. Following surgery, no further symptoms were reported and biochemical tests normalized.

  2. The diagnosis of bilateral primary renal paragangliomas in a cat

    Directory of Open Access Journals (Sweden)

    Ryan B. Friedlein

    2017-01-01

    Full Text Available A 9-year-old sterilised female domestic short-hair cat was referred with a history of vomiting and anorexia of 3 months’ duration. Biochemistry, full-blood counts, thoracic radiographs, feline pancreatic-specific lipase, abdominal ultrasonography and feline immunodeficiency virus/feline leukaemia virus (FIV/FeLV SNAP tests had been performed. Mild hypochloraemia and moderate hypokalaemia were evident on initial presentation. Abdominal ultrasonography initially revealed unilateral renal nodules on the left side. These were subjected to fine-needle aspiration and cytological evaluation. A neuroendocrine tumour was suspected, and biopsies via midline coeliotomy were taken to confirm the diagnosis. Initial histopathology diagnosed primary renal carcinomas or neuroendocrine neoplasia; however, the definitive diagnosis became renal paragangliomas after immunohistochemistry and transmission electron microscopy were performed. The cat was regularly monitored with serum biochemistry parameters, blood pressure determinations, thoracic radiographs and subsequent abdominal ultrasonography. Biochemistry, radiography and blood pressures remained normal over a 24-week follow-up period, while subsequent ultrasonography revealed tumour progression in both number and size in both kidneys. Primary neuroendocrine tumours of the kidney are frequently incorrectly diagnosed as other renal tumours such as renal cell carcinoma, mesonephric tumours or undifferentiated carcinomas. This case report highlights the importance of additional testing, including immunohistochemistry and transmission electron microscopy, to obtain a definitive diagnosis of paragangliomas.

  3. Update on Modern Management of Pheochromocytoma and Paraganglioma.

    Science.gov (United States)

    Lenders, Jacques W M; Eisenhofer, Graeme

    2017-06-01

    Despite all technical progress in modern diagnostic methods and treatment modalities of pheochromocytoma/paraganglioma, early consideration of the presence of these tumors remains the pivotal link towards the best possible outcome for patients. A timely diagnosis and proper treatment can prevent the wide variety of potentially catastrophic cardiovascular complications. Modern biochemical testing should include tests that offer the best available diagnostic performance, measurements of metanephrines and 3-methoxytyramine in plasma or urine. To minimize false-positive test results particular attention should be paid to pre-analytical sampling conditions. In addition to anatomical imaging by computed tomography (CT) or magnetic resonance imaging, new promising functional imaging modalities of photon emission tomography/CT using with somatostatin analogues such as ⁶⁸Ga-DOTATATE (⁶⁸Ga-labeled DOTA(0)-Tyr(3)-octreotide) will probably replace ¹²³I-MIBG (iodine-123-metaiodobenzylguanidine) in the near future. As nearly half of all pheochromocytoma patients harbor a mutation in one of the 14 tumor susceptibility genes, genetic testing and counseling should at least be considered in all patients with a proven tumor. Post-surgical annual follow-up of patients by measurements of plasma or urinary metanephrines should last for at least 10 years for timely detection of recurrent or metastatic disease. Patients with a high risk for recurrence or metastatic disease (paraganglioma, young age, multiple or large tumors, genetic background) should be followed up lifelong. Copyright © 2017 Korean Endocrine Society.

  4. Update on Modern Management of Pheochromocytoma and Paraganglioma

    Directory of Open Access Journals (Sweden)

    Jacques W. M. Lenders

    2017-06-01

    Full Text Available Despite all technical progress in modern diagnostic methods and treatment modalities of pheochromocytoma/paraganglioma, early consideration of the presence of these tumors remains the pivotal link towards the best possible outcome for patients. A timely diagnosis and proper treatment can prevent the wide variety of potentially catastrophic cardiovascular complications. Modern biochemical testing should include tests that offer the best available diagnostic performance, measurements of metanephrines and 3-methoxytyramine in plasma or urine. To minimize false-positive test results particular attention should be paid to pre-analytical sampling conditions. In addition to anatomical imaging by computed tomography (CT or magnetic resonance imaging, new promising functional imaging modalities of photon emission tomography/CT using with somatostatin analogues such as 68Ga-DOTATATE (68Ga-labeled DOTA(0-Tyr(3-octreotide will probably replace 123I-MIBG (iodine-123-metaiodobenzylguanidine in the near future. As nearly half of all pheochromocytoma patients harbor a mutation in one of the 14 tumor susceptibility genes, genetic testing and counseling should at least be considered in all patients with a proven tumor. Post-surgical annual follow-up of patients by measurements of plasma or urinary metanephrines should last for at least 10 years for timely detection of recurrent or metastatic disease. Patients with a high risk for recurrence or metastatic disease (paraganglioma, young age, multiple or large tumors, genetic background should be followed up lifelong.

  5. Mothers and fathers of young Dutch adolescents with Down syndrome: Health related quality of life and family functioning.

    Science.gov (United States)

    Marchal, Jan Pieter; Maurice-Stam, Heleen; van Trotsenburg, A S Paul; Grootenhuis, Martha A

    2016-12-01

    Like any child, children with Down syndrome (DS) affect the lives of their families. Most studies focus on the adaptation of parents and families of young children with DS, while relatively few studies include the perspective of fathers. To determine 1) whether mothers and fathers of 11 to 13-year-olds with DS differ from reference parents in health related quality of life (HRQoL) and family functioning, and 2) whether HRQoL in parents of children with DS changes over time, from when the child was 6-8 years old to when the child was 11-13 years old. 80 mothers and 44 fathers completed HRQoL and family functioning questionnaires. 58 parents (53 mothers) had completed the HRQoL-questionnaire in a previous study. Mothers differed from reference mothers in one HRQoL-domain (Sexuality), while fathers' HRQoL did not significantly differ from reference fathers. Both mothers and fathers scored in the (sub)clinical range more frequently than reference parents in Total family functioning, and in the domains Partner relation and Social network. Furthermore, fathers scored in the (sub)clinical range more frequently than reference parents in Responsiveness and Organization. HRQoL showed no significant change over time. Our findings indicate frequent family functioning problems but few HRQoL problems in parents and families of children with DS. In offering care, a family based approach with special attention for partner relation and social functioning is needed. Copyright © 2016 Elsevier Ltd. All rights reserved.

  6. Paraganglioma of the vagina: a case report and review of the literature

    OpenAIRE

    Cai, Tao; Huang,Yu; Li,Yucong; Jiang,Qingming; Wang,Dong

    2014-01-01

    Tao Cai,1 Yucong Li,2 Qingming Jiang,3 Dong Wang,2 Yu Huang2 1Department of Dermatology, The First Affiliated Hospital of Chongqing Medical University, 2Department of Gynecologic Oncology, 3Department of Pathology, Chongqing Cancer Research Institute, Chongqing, People’s Republic of China Abstract: Vaginal paraganglioma is a rare and unusual tumor occurring in the vaginal wall. It is a solitary primary paraganglioma, especially in atypical sites. Herein, we report an unusual case o...

  7. Dutch Dwellings

    NARCIS (Netherlands)

    Vries, de B.; Zee, van der A.; Carp, J.C.; Soddu, C.

    2004-01-01

    Applying Generative Design (GD) for dwelling is not very common but it opens up the possibility to study whether GD systems can reproduce existing design typologies. Dutch dwellings as an exemplification of a design typology are analysed using the SAR methodology. Building regulations are used as

  8. Successful treatment of paraganglioma with sorafenib: a case report and brief review of the literature

    Directory of Open Access Journals (Sweden)

    Lin Y

    2013-11-01

    Full Text Available Yun Lin,1,* Qun Li,1,* Wei Huang,1 Xinyan Jia,1 Hong Jiang,1 Yong Gao,1 Qi Li1,21Department of Oncology, Shanghai East Hospital, 2Department of Oncology, Shanghai First People's Hospital, Shanghai, People's Republic of China*These authors contributed equally to this work, and are joint first authorsIntroduction: To date, no effective systemic therapies have been made available for paraganglioma. However, multiple mutations in susceptibility genes have been identified that are potential targets for sorafenib, an oral multitargeted tyrosine-kinase inhibitor.Case presentation: We report the case of a 69-year-old Chinese man with mediastinal paraganglioma that had metastasized to the bone. The paraganglioma responded to sorafenib, a novel multi-tyrosine kinase inhibitor that targets angiogenesis, the Raf-kinase pathway, the platelet-derived growth factor Ret, and c-Kit. The patient was diagnosed as having paraganglioma after biopsy of the mediastinal mass. We first treated the patient with radiotherapy. Then he tolerated an etoposide-and-cisplatin chemotherapy regimen. Subsequently, he received 6 months of maintenance treatment with sorafenib (400 mg twice daily. A dramatic reduction in tumor volume was observed. At present, the patient has achieved a partial response, and his clinical status remains unchanged.Conclusion: We suggest that sorafenib should be further investigated in the management of patients with paraganglioma.Keywords: sorafenib, paraganglioma, molecular-targeted drug

  9. A Dutch family with autosomal recessively inherited lower motor neuron predominant motor neuron disease due to optineurin mutations

    NARCIS (Netherlands)

    Beeldman, Emma; van der Kooi, Anneke J.; de Visser, Marianne; van Maarle, Merel C.; van Ruissen, Fred; Baas, Frank

    2015-01-01

    Approximately 10% of motor neuron disease (MND) patients report a familial predisposition for MND. Autosomal recessively inherited MND is less common and is most often caused by mutations in the superoxide dismutase 1 (SOD1) gene. In 2010, autosomal recessively inherited mutations in the optineurin

  10. Germline variant in MSX1 identified in a Dutch family with clustering of Barrett’s esophagus and esophageal adenocarcinoma

    NARCIS (Netherlands)

    A.M.J. van Nistelrooij (Annemarie); R. van Marion (Ronald); W.F.J. van IJcken (Wilfred); A. de Klein (Annelies); A. Wagner (Anja); K. Biermann (Katharina); M.C.W. Spaander (Manon); J.J.B. van Lanschot (Jan); W.N.M. Dinjens (Winand); B.P.L. Wijnhoven (Bas)

    2017-01-01

    textabstractThe vast majority of esophageal adenocarcinoma cases are sporadic and caused by somatic mutations. However, over the last decades several families have been identified with clustering of Barrett’s esophagus and esophageal adenocarcinoma. This observation suggests that one or more

  11. Acute type A aortic dissection in a patient with paraganglioma.

    Science.gov (United States)

    Dos Santos Borrego, Andreia; Carrilho Ferreira, Pedro; Pinto, Fausto J

    2017-10-01

    Acute aortic dissection is the most common acute aortic syndrome. It is more prevalent in males and in the elderly, and has a high mortality. Hypertension is the main risk factor. Diagnosis is based on clinical features, laboratory tests and imaging exams. Treatment is usually surgical, although in some cases an endovascular approach is an alternative. Paraganglioma is an uncommon neuroendocrine tumor. Most produce catecholamines, and so usually manifest with hypertensive crisis, palpitations, headache and sweating. This tumor is diagnosed by measurement of plasma or urinary catecholamines and by computed tomography, magnetic resonance imaging and 123 I-metaiodobenzylguanidine (MIBG) scintigraphy. Surgery is the only potentially curative treatment. Copyright © 2017 Sociedade Portuguesa de Cardiologia. All rights reserved.

  12. Malignant pheochromocytomas and paragangliomas - the importance of a multidisciplinary approach

    DEFF Research Database (Denmark)

    Andersen, Kim Francis; Altaf, Rahim; Krarup-Hansen, Anders

    2011-01-01

    -secreted with catecholamines, may indicate tumour mass and malignancy and can be used to monitor response and relapse. The secretory and non-secretory tumours can be visualised with functional (specific and non-specific) imaging as SPECT and PET using ¹²³I-MIBG, somatostatin analogues, ¹8F-DOPA, and ¹8F-FDG. These modalities...... imaging with somatostatin analogues generally has high sensitivity in malignant disease. There are no curative therapeutic options for malignant, metastatic pheochromocytomas/paragangliomas, wherefore consolidation of quality of life is essential. Adjuvant radionuclide treatment with beta......-emitting isotopes coupled to MIBG or somatostatin analogues have shown response in approximately 30%. Chemotherapy is restricted to patients not accessible for surgery and resistant to radionuclide therapy. Novel targeted therapies, which mainly through a cytostatic effect interfere with specific targeted molecules...

  13. Osseous Metastase of Occult Paraganglioma: A Diagnostic Medical Error

    Directory of Open Access Journals (Sweden)

    Ghasemi TA

    2013-10-01

    Full Text Available Introduction: Diagnostic errors have a natural complexity. Medical diagnoses make up a large proportion of all medical errors and cause much suffering and harm. Compared to other types of error, diagnostic errors receive little attention-a major factor in continuity of unacceptable rates of diagnostic error. Case: A 55-year-old woman presented to the emergency department (ED complaining of bone pain which has been started a month ago and increased gradually in the upper right thigh. Following the emergency evaluation she was sent home with pain medication. On the second visit, a femur neck fracture was seen in the x-ray. She underwent hemiarthroplasty and was discharged. Over several weeks she was reevaluated by many Physicians, because of her worsening pain .In the third visit after the surgery, her x-ray showed bone destruction and following bone biopsy, malignant paraganglioma was diagnosed. Discussion and solution: In all cases in which patient comes to us with skeletal pain, getting a comprehensive history and a full physical examination are prior to lab tests and x-rays. Bone metastasis which can develop severe pain and pathological fractures, is common in patients with malignant paraganglioma. Effective steps for diagnostic error prevention are: Considering the diagnostic error in the normal range of quality assurance surveillance and review, identifying the elements leading to diagnostic errors and getting feedback on the diagnoses Physicians make, in order to improve their skills. Conclusion: It is an every health system priority to identify, analyze, and prevent diagnostic errors in order to improve patient safety

  14. Familial amyloidotic polyneuropathy with severe renal involvement in association with transthyretin Gly47Glu in Dutch, British and American-Finnish families

    NARCIS (Netherlands)

    Haagsma, EB; Hawkins, PN; Benson, MD; Lachmann, HJ; Bybee, A; Hazenberg, BPC

    Familial amyloidotic polyneuropathy (FAP) is an autosomal dominant disorder associated with more than 80 different transthyretin (TTR) mutations. The clinical features of FAP are broad and variable, but knowledge of the pattern and natural history of disease associated with particular mutations

  15. Perioperative management of paraganglioma and catecholamine-induced cardiomyopathy in child- a case report and review of the literature.

    Science.gov (United States)

    Jia, Xixi; Guo, Xiangyang; Zheng, Qing

    2017-10-17

    Paragangliomas are catecholamine-secreting tumors of the paraganglia. Perioperative mortality of children with paraganglioma is high, but preoperative therapy and anesthetic management of paraganglioma resection are controversial in children. The literatures on catecholamine-induced cardiomyopathy are limited to several case reports,with few reports of studies on children. Here we report the anesthetic management of a child with paraganglioma and catecholamine-induced cardiomyopathy, and the possible perioperative anesthesia problems of the paraganglioma resection are discussed. Preoperative and intraoperative anesthetic management of Pheochromocytomas children should follow the same principles as for adults, The most important aspects are the control of blood pressure liability and maintenance of adequate blood volume. Pheochromocytomas patient may have cardiomoyopathy due to myocardial toxicity of excessive circulating catecholamines level. The perioperative management of catecholamine-induced cardiomyopathy should include lowering sympathetic activation by means of α-and β-adrenergic receptor blocker and diuretics administration in case of volume overload.

  16. Does fear of childbirth or family history affect whether pregnant Dutch women prefer a home- or hospital birth?

    Science.gov (United States)

    Sluijs, Anne-Marie; Cleiren, Marc P H D; Scherjon, Sicco A; Wijma, Klaas

    2015-12-01

    It is a generally accepted idea that women who give birth at home are less fearful of giving birth than women who give birth in a hospital. We explored fear of childbirth (FOC) in relation to preferred and actual place of birth. Since the Netherlands has a long history of home birthing, we also examined how the place where a pregnant woman׳s mother or sisters gave birth related to the preferred place of birth. A prospective cohort study. Five midwifery practises in the region Leiden/Haarlem, the Netherlands. 104 low risk nulliparous and parous women. Questionnaires were completed in gestation week 30 (T1) and six weeks post partum (T2). No significant differences were found in antepartum FOC between those who preferred a home or a hospital birth. Women with a strong preference for either home or hospital had lower FOC (mean W-DEQ=60.3) than those with a weak preference (mean W-DEQ=71.0), t (102)=-2.60, p=0.01. The place of birth of close family members predicted a higher chance (OR 3.8) of the same place being preferred by the pregnant woman. Pre- to postpartum FOC increased in women preferring home- but having hospital birth. The idea that FOC is related to the choice of place of birth was not true for this low risk cohort. Women in both preference groups (home and hospital) made their decisions based on negative and positive motivations. Mentally adjusting to a different environment than that preferred, apart from the medical complications, can cause more FOC post partum. The decreasing number of home births in the Netherlands will probably be a self-reinforcing effect, so in future, pregnant women will be less likely to feel supported by their family or society to give birth at home. Special attention should be given to the psychological condition of women who were referred to a place of birth and caregiver they did not prefer, by means of evaluation of the delivery and being alert to anxiety or other stress symptoms after childbirth. These women have higher

  17. A patient with a painless neck tumour revealed as a carotid paraganglioma: a case report.

    Science.gov (United States)

    Peric, Barbara; Marinsek, Ziva Pohar; Skrbinc, Breda; Music, Maja; Zagar, Ivana; Hocevar, Marko

    2014-08-20

    Carotid paragangliomas are usually slowly enlarging and painless lateral neck masses. These mostly benign lesions are recognized due to their typical location, vessel displacement and specific blood supply, features that are usually seen on different imaging modalities. Surgery for carotid paraganglioma can be associated with immediate cerebrovascular complications or delayed neurological impairment.We are reporting the case of a 36-year-old man who presented with a painless mass on the right side of his neck 11 months after being treated for testicular cancer. After a fine-needle aspiration biopsy, he was diagnosed with a testicular cancer lymph node metastasis. Neck US and fluorine [F-18]-fluorodeoxy-D-glucose (FDG) PET-CT showed no signs of hypervascularity or vessel displacement. The patient underwent a level II to V functional neck dissection. During the procedure, suspicion of a carotid paraganglioma was raised and the tumour was carefully dissected from the walls of the carotid arteries with minimal blood loss and no cranial nerve dysfunction.The histology report revealed carotid paraganglioma with no metastasis in the rest of the lymph nodes. The patient's history of testicular germ cell tumour led to a functional neck dissection during which a previously unrecognized carotid paraganglioma was removed.Surgery for carotid PG can be associated with complications that have major impact on quality of life. A thorough assessment of the patient and neck mass must therefore be performed preoperatively in order to perform the surgical procedure under optimal conditions.

  18. Family-centredness of professionals who support people with profound intellectual and multiple disabilities: validation of the Dutch 'Measure of Processes of Care for Service Providers' (MPOC-SP-PIMD).

    Science.gov (United States)

    Jansen, Suzanne L G; van der Putten, Annette A J; Post, Wendy J; Vlaskamp, Carla

    2014-07-01

    A Dutch version of the 'Measure of Processes of Care for Service Providers' (MPOC-SP) was developed to determine the extent to which professionals apply the principles of family-centred care in the rehabilitation of children with physical disabilities. However, no data were available on the reliability and construct validity of this instrument when it comes to supporting people with profound intellectual and multiple disabilities (PIMD). This study aimed to validate an adapted version of the Dutch MPOC-SP for assessing the family-centred behaviours of professionals who support this group (MPOC-SP-PIMD). A total of 105 professionals took part in the study. A Mokken scale analysis was conducted to determine whether the instrument satisfied the assumptions of both monotone homogeneity and double monotonicity. Loevinger's scalability coefficient (H) was used for the scalability of the entire scale and of each item separately. Rho was calculated as a measure of the internal consistency of the scales. The analyses resulted in two scales: a nine-item scale interpreted as 'Showing Interpersonal Sensitivity', with H=.39 and rho=.76, and a seven-item scale interpreted as 'Treating People Respectfully', with H=.49 and rho=.78. A validated version of the MPOC-SP-PIMD, suitable for supporting people with PIMD, consists of a subset of two scales from the original Dutch MPOC-SP. This instrument can be used to compare the family-centredness of professionals with parent's expectations and views. This information can be used in practice to match the support to the needs of the parents and family of the child with PIMD. Copyright © 2014 Elsevier Ltd. All rights reserved.

  19. Paraganglioma of the vagina: a case report and review of the literature

    Science.gov (United States)

    Cai, Tao; Li, Yucong; Jiang, Qingming; Wang, Dong; Huang, Yu

    2014-01-01

    Vaginal paraganglioma is a rare and unusual tumor occurring in the vaginal wall. It is a solitary primary paraganglioma, especially in atypical sites. Herein, we report an unusual case of a 17-year-old woman who had not experienced vomiting, or hypertension. She was found to have an immobile solid mass in the right side of her vaginal wall. Positron emission tomography/computed tomography scans revealed a well-defined solid ovoid mass adjacent to the bladder and pelvic floor. Tumor markers were within the normal range. A transient blood pressure increase occurred during the biopsy. After oral administration of antihypertensive drugs, surgery was performed to completely remove the mass. Histopathological examination indicated that it was a paraganglioma of the vagina. Repeat computed tomography examination did not reveal any local recurrence or distant metastasis during the 12-month follow-up period. PMID:24959083

  20. Going Dutch

    Energy Technology Data Exchange (ETDEWEB)

    Hoorn, C.

    2002-09-07

    The proposal by the Dutch to extract natural gas from the 40 billion cubic metres beneath the Wadden Sea has been cited as a serious cause for concern by environmentalists. The new right wing government is less concerned than was the recently ousted left wing government and stands to gain directly through increased revenue. The Wadden Sea is a vast area of tidal channels, mudflats, shifting sandbanks, salt marshes, wet meadows and dunes, and provides a safe haven for numerous protected plants and animals. It is also a sanctuary for migrating birds and a feeding ground for herring, sole and plaice. Subsidence is cited by the environmentalists as a major potential problem and pollution is said to be always a risk.

  1. Going Dutch

    International Nuclear Information System (INIS)

    Hoorn, C.

    2002-01-01

    The proposal by the Dutch to extract natural gas from the 40 billion cubic metres beneath the Wadden Sea has been cited as a serious cause for concern by environmentalists. The new right wing government is less concerned than was the recently ousted left wing government and stands to gain directly through increased revenue. The Wadden Sea is a vast area of tidal channels, mudflats, shifting sandbanks, salt marshes, wet meadows and dunes, and provides a safe haven for numerous protected plants and animals. It is also a sanctuary for migrating birds and a feeding ground for herring, sole and plaice. Subsidence is cited by the environmentalists as a major potential problem and pollution is said to be always a risk

  2. Paraganglioma with unusual presentation in parotid gland: A diagnostic dilemma in fine needle aspiration

    Directory of Open Access Journals (Sweden)

    Anagh A Vora

    2012-01-01

    Full Text Available Paragangliomas (PGLs are uncommon tumors. Although PGLs are known to occur in the head and neck region, especially the carotid body, middle ear, and larynx, involvement of the parotid glands has not been reported. In this article, we report the fine needle aspiration features of tumor in an unusual location, presenting as a parotid gland mass, submitted to pathology for initial diagnosis. The clinical presentation, cytomorphology, and the immunohistochemical features for the diagnosis are described. To our knowledge, this is the first case of paraganglioma of the parotid gland reported in the literature.

  3. Familial Investigations of Childhood Cancer Predisposition

    Science.gov (United States)

    2018-01-03

    Acute Leukemia; Adenomatous Polyposis; Adrenocortical Carcinoma; AML; BAP1 Tumor Predisposition Syndrome; Carney Complex; Choroid Plexus Carcinoma; Constitutional Mismatch Repair Deficiency Syndrome; Diamond-Blackfan Anemia; DICER1 Syndrome; Dyskeratosis Congenita; Emberger Syndrome; Familial Acute Myeloid Leukemia; Familial Adenomatous Polyposis; Fanconi Anemia; Familial Cancer; Familial Wilms Tumor; Familial Neuroblastoma; GIST; Hereditary Breast and Ovarian Cancer; Hereditary Paraganglioma-Pheochromocytoma Syndrome; Hodgkin Lymphoma; Juvenile Polyposis; Li-Fraumeni Syndrome; Lynch Syndrome; MDS; Melanoma Syndrome; Multiple Endocrine Neoplasia Type 1; Multiple Endocrine Neoplasia Type 2; Neuroblastoma; Neurofibromatosis Type 1; Neurofibromatosis Type II; Nevoid Basal Cell Carcinoma Syndrome; Non Hodgkin Lymphoma; Noonan Syndrome and Other Rasopathy; Overgrowth Syndromes; Pancreatic Cancer; Peutz-Jeghers Syndrome; Pheochromocytoma/Paraganglioma; PTEN Hamartoma Tumor Syndrome; Retinoblastoma; Rhabdoid Tumor Predisposition Syndrome; Rhabdomyosarcoma; Rothmund-Thomson Syndrome; Tuberous Sclerosis; Von Hippel-Lindau Disease

  4. Precision medicine in pheochromocytoma and paraganglioma: current and future concepts.

    Science.gov (United States)

    Björklund, P; Pacak, K; Crona, J

    2016-12-01

    Pheochromocytoma and paraganglioma (PPGL) are rare diseases but are also amongst the most characterized tumour types. Hence, patients with PPGL have greatly benefited from precision medicine for more than two decades. According to current molecular biology and genetics-based taxonomy, PPGL can be divided into three different clusters characterized by: Krebs cycle reprogramming with oncometabolite accumulation or depletion (group 1a); activation of the (pseudo)hypoxia signalling pathway with increased tumour cell proliferation, invasiveness and migration (group 1b); and aberrant kinase signalling causing a pro-mitogenic and anti-apoptotic state (group 2). Categorization into these clusters is highly dependent on mutation subtypes. At least 12 different syndromes with distinct genetic causes, phenotypes and outcomes have been described. Genetic screening tests have a documented benefit, as different PPGL syndromes require specific approaches for optimal diagnosis and localization of various syndrome-related tumours. Genotype-tailored treatment options, follow-up and preventive care are being investigated. Future new developments in precision medicine for PPGL will mainly focus on further identification of driver mechanisms behind both disease initiation and malignant progression. Identification of novel druggable targets and prospective validation of treatment options are eagerly awaited. To achieve these goals, we predict that collaborative large-scale studies will be needed: Pheochromocytoma may provide an example for developing precision medicine in orphan diseases that could ultimately aid in similar efforts for other rare conditions. © 2016 The Association for the Publication of the Journal of Internal Medicine.

  5. Pheochromocytoma/Paraganglioma: A Poster Child for Cancer Metabolism.

    Science.gov (United States)

    Tevosian, Sergei G; Ghayee, Hans K

    2018-05-01

    Pheochromocytomas (PCCs) are tumors that are derived from the chromaffin cells of the adrenal medulla. Extra-adrenal PCCs called paragangliomas (PGLs) are derived from the sympathetic and parasympathetic chain ganglia. PCCs secrete catecholamines, which cause hypertension and have adverse cardiovascular consequences as a result of catecholamine excess. PGLs may or may not produce catecholamines depending on their genetic type and anatomical location. The most worrisome aspect of these tumors is their ability to become aggressive and metastasize; there are no known cures for metastasized PGLs. Original articles and reviews indexed in PubMed were identified by querying with specific PCC/PGL- and Krebs cycle pathway-related terms. Additional references were selected through the in-depth analysis of the relevant publications. We primarily discuss Krebs cycle mutations that can be instrumental in helping investigators identify key biological pathways and molecules that may serve as biomarkers of or treatment targets for PCC/PGL. The mainstay of treatment of patients with PCC/PGLs is surgical. However, the tide may be turning with the discovery of new genes associated with PCC/PGLs that may shed light on oncometabolites used by these tumors.

  6. Phaeochromocytoma and functioning paraganglioma in childhood and adolescence: Role of iodine 131 metaiodobenzylguanidine

    International Nuclear Information System (INIS)

    Khafagi, F.A.; Shapiro, B.; Fischer, M.; Sisson, J.C.; Beierwaltes, W.H.; Hutchinson, R.

    1991-01-01

    Phaeochromocytomas and functioning paragangliomas are rare tumours in childhood and adolescence. We review our experience of 43 cases (24 men, 19 women) who were first diagnosed at the age of ≤ 18 years. All patients were evaluated at some point in their illness, with iodine 131 metaiodobenzylguanidine ( 131 I-mIBG) scintigraphy. Eight patients (19%) had bilateral adrenal tumours, 12 (28%) had solitary extraadrenal tumours, and 8 (19%) had multiple tumours. In 10 patients (23%), the tumours were associated with a familial neurocristopathic syndrome. Thirteen of 24 (54%) unifocal tumours which were initially considered to be benign ultimately proved to be multi-focal and/or malignant. The final prevalence of malignancy was 60% - 26 patients, of whom only 15 (57%) had obviously malignant tumours at the time of diagnosis. Primary tumour size ≥ 5 cm was more commonly associated with a malignant course in adrenal but not extra-adrenal tumours. No other clinical, biochemical or morphological characteristic was significantly associated with malignancy. Although the high prevalence of malignancy in this series at least partly reflects referral bias, the need for lifelong follow-up of these patients is underscored. 131 I-mIBG scintigraphy was positive in 36 patients (84%), with a somewhat lower false-negative rate (12%) than X-ray computed tomography (20%). Eight patients with malignant tumours received therapeutic doses of 131 I-mIBG, with partial tumour responses in 3. Thus, 131 I-mIBG is an efficacious, non-invasive, localising agent and may be considered as a palliative therapeutic agent when alternatives have failed. (orig.)

  7. Predictors of malignancy in patients with pheochromocytomas/paragangliomas: Asian Indian experience

    Science.gov (United States)

    Sarathi, Vijaya; Kasaliwal, Rajeev; Pandit, Reshma; Goroshi, Manjunath; Malhotra, Gaurav; Dalvi, Abhay; Bakshi, Ganesh; Bhansali, Anil; Rajput, Rajesh; Shivane, Vyankatesh; Lila, Anurag; Bandgar, Tushar; Shah, Nalini S

    2016-01-01

    Background and aims Malignant transformation of pheochromocytomas/paragangliomas (PCC/PGL) is a rare occurrence, and predictive factors for the same are not well understood. This study aims to identify the predictors of malignancy in patients with PCC/PGL. Materials and methods We performed a retrospective analysis of 142 patients with either PCC or PGL registered at our institute between 2000 and 2015. Records were evaluated for clinical parameters like age, gender, familial/syndromic presentation, symptomatic presentation, biochemistry, size, number and location of tumours and presence of metastases and mode of its diagnosis. Results Twenty patients were found to have metastases; 13 had metastases at diagnosis and seven during follow-up. Metastases were detected by radiology (CT-neck to pelvis) in 11/20 patients (5/13 synchronous and 6/7 metachronous), 131I-metaiodobenzylguanidine in five (2/12 synchronous and 3/6 metachronous) patients and 18F-flurodeoxyglucose PET/CT in 15 (12/12 synchronous and 3/3 metachronous) patients. Malignant tumours were significantly larger than benign tumours (8.3 ± 4.1 cm, range: 3–22 cm vs 5.7 ± 2.3 cm, range: 2–14 cm, P = 0.0001) and less frequently metanephrine secreting. On linear regression analysis, tumour size and lack of metanephrine secretion were the independent predictors of malignancy. Conclusions Patients with primary tumour size >5.7 cm and lack of metanephrine secretory status should be evaluated for possible malignancy not only at diagnosis but also in the postoperative period. As compared to CT and 131I-MIBG scan, 18F-flurodeoxyglucose PET/CT analyses are better (sensitivity: 100%) for the diagnosis of metastases in our study. PMID:27852633

  8. Predictors of malignancy in patients with pheochromocytomas/paragangliomas: Asian Indian experience

    Directory of Open Access Journals (Sweden)

    Kranti Khadilkar

    2016-12-01

    Full Text Available Background and aims: Malignant transformation of pheochromocytomas/paragangliomas (PCC/PGL is a rare occurrence, and predictive factors for the same are not well understood. This study aims to identify the predictors of malignancy in patients with PCC/PGL. Materials and methods: We performed a retrospective analysis of 142 patients with either PCC or PGL registered at our institute between 2000 and 2015. Records were evaluated for clinical parameters like age, gender, familial/syndromic presentation, symptomatic presentation, biochemistry, size, number and location of tumours and presence of metastases and mode of its diagnosis. Results: Twenty patients were found to have metastases; 13 had metastases at diagnosis and seven during follow-up. Metastases were detected by radiology (CT-neck to pelvis in 11/20 patients (5/13 synchronous and 6/7 metachronous, 131I-metaiodobenzylguanidine in five (2/12 synchronous and 3/6 metachronous patients and 18F-flurodeoxyglucose PET/CT in 15 (12/12 synchronous and 3/3 metachronous patients. Malignant tumours were significantly larger than benign tumours (8.3 ± 4.1 cm, range: 3–22 cm vs 5.7 ± 2.3 cm, range: 2–14 cm, P = 0.0001 and less frequently metanephrine secreting. On linear regression analysis, tumour size and lack of metanephrine secretion were the independent predictors of malignancy. Conclusions: Patients with primary tumour size >5.7 cm and lack of metanephrine secretory status should be evaluated for possible malignancy not only at diagnosis but also in the postoperative period. As compared to CT and 131I-MIBG scan, 18F-flurodeoxyglucose PET/CT analyses are better (sensitivity: 100% for the diagnosis of metastases in our study.

  9. The Hidden Cost of Untreated Paragangliomas of the Head and Neck: Systemic Reactive (AA Amyloidosis

    Directory of Open Access Journals (Sweden)

    Erkan Dervisoglu

    2015-01-01

    Full Text Available We report a case of a 51-year-old man who was diagnosed with systemic reactive (AA amyloidosis in association with untreated glomus jugulare and glomus caroticum tumors. He refused radiotherapy and renal replacement therapy. Paragangliomas, although rare, should be considered one of the tumors that can result in AA amyloidosis.

  10. Paraganglioma of the vagina: a case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Cai T

    2014-06-01

    Full Text Available Tao Cai,1 Yucong Li,2 Qingming Jiang,3 Dong Wang,2 Yu Huang2 1Department of Dermatology, The First Affiliated Hospital of Chongqing Medical University, 2Department of Gynecologic Oncology, 3Department of Pathology, Chongqing Cancer Research Institute, Chongqing, People’s Republic of China Abstract: Vaginal paraganglioma is a rare and unusual tumor occurring in the vaginal wall. It is a solitary primary paraganglioma, especially in atypical sites. Herein, we report an unusual case of a 17-year-old woman who had not experienced vomiting, or hypertension. She was found to have an immobile solid mass in the right side of her vaginal wall. Positron emission tomography/computed tomography scans revealed a well-defined solid ovoid mass adjacent to the bladder and pelvic floor. Tumor markers were within the normal range. A transient blood pressure increase occurred during the biopsy. After oral administration of antihypertensive drugs, surgery was performed to completely remove the mass. Histopathological examination indicated that it was a paraganglioma of the vagina. Repeat computed tomography examination did not reveal any local recurrence or distant metastasis during the 12-month follow-up period. Keywords: vaginal paraganglioma, neuroendocrine tumor, clinical features, treatment

  11. The mTORC1 Complex Is Significantly Overactivated in SDHX-Mutated Paragangliomas

    NARCIS (Netherlands)

    Oudijk, Lindsey; Papathomas, Thomas; de Krijger, Ronald; Korpershoek, Esther; Gimenez-Roqueplo, Anne Paule; Favier, Judith; Canu, Letizia; Mannelli, Massimo; Rapa, Ida; Currás-Freixes, Maria; Robledo, Mercedes; Smid, Marcel; Papotti, Mauro; Volante, Marco

    2017-01-01

    AIM: We aimed at exploring the activation pattern of the mTOR pathway in sporadic and hereditary pheochromocytomas (PCCs) and paragangliomas (PGLs). METHODS: A total of 178 PCCs and 44 PGLs, already characterized for the presence of germline mutations in VHL, RET, NF1, MAX, SDHA, SDHB, SDHC, and

  12. PheoSeq : A Targeted Next-Generation Sequencing Assay for Pheochromocytoma and Paraganglioma Diagnostics

    NARCIS (Netherlands)

    Currás-Freixes, Maria; Piñeiro-Yañez, Elena; Montero-Conde, Cristina; Apellániz-Ruiz, María; Calsina, Bruna; Mancikova, Veronika; Remacha, Laura; Richter, Susan; Ercolino, Tonino; Rogowski-Lehmann, Natalie; Deutschbein, Timo; Calatayud, María; Guadalix, Sonsoles; Álvarez-Escolá, Cristina; Lamas, Cristina; Aller, Javier; Sastre-Marcos, Julia; Lázaro, Conxi; Galofré, Juan C.; Patiño-García, Ana; Meoro-Avilés, Amparo; Balmaña-Gelpi, Judith; De Miguel-Novoa, Paz; Balbín, Milagros; Matías-Guiu, Xavier; Letón, Rocío; Inglada-Pérez, Lucía; Torres-Pérez, Rafael; Roldán-Romero, Juan M.; Rodríguez-Antona, Cristina; Fliedner, Stephanie M J; Opocher, Giuseppe; Pacak, Karel; Korpershoek, Esther; de Krijger, Ronald R.; Vroonen, Laurent; Mannelli, Massimo; Fassnacht, Martin; Beuschlein, Felix; Eisenhofer, Graeme; Cascón, Alberto; Al-Shahrour, Fátima; Robledo, Mercedes

    2017-01-01

    Genetic diagnosis is recommended for all pheochromocytoma and paraganglioma (PPGL) cases, as driver mutations are identified in approximately 80% of the cases. As the list of related genes expands, genetic diagnosis becomes more time-consuming, and targeted next-generation sequencing (NGS) has

  13. 'Burnout' among Dutch midwives.

    NARCIS (Netherlands)

    Bakker, R.H.C.; Groenewegen, P.P.; Jabaaij, L.; Meijer, W.; Sixma, H.; Veer, A. de

    1996-01-01

    OBJECTIVE: to determine the effect of workload on 'burnout' having considered work capacity. DESIGN: cross-sectional study. SETTING: Dutch community midwives in independent practice. PARTICIPANTS: 200 Dutch community midwives. MEASUREMENTS: three-week diary recordings, a questionnaire on practice

  14. Novel insights into the polycythemia-paraganglioma-somatostatinoma syndrome.

    Science.gov (United States)

    Därr, Roland; Nambuba, Joan; Del Rivero, Jaydira; Janssen, Ingo; Merino, Maria; Todorovic, Milena; Balint, Bela; Jochmanova, Ivana; Prchal, Josef T; Lechan, Ronald M; Tischler, Arthur S; Popovic, Vera; Miljic, Dragana; Adams, Karen T; Prall, F Ryan; Ling, Alexander; Golomb, Meredith R; Ferguson, Michael; Nilubol, Naris; Chen, Clara C; Chew, Emily; Taïeb, David; Stratakis, Constantine A; Fojo, Tito; Yang, Chunzhang; Kebebew, Electron; Zhuang, Zhengping; Pacak, Karel

    2016-12-01

    Worldwide, the syndromes of paraganglioma (PGL), somatostatinoma (SOM) and early childhood polycythemia are described in only a few patients with somatic mutations in the hypoxia-inducible factor 2 alpha (HIF2A). This study provides detailed information about the clinical aspects and course of 7 patients with this syndrome and brings into perspective these experiences with the pertinent literature. Six females and one male presented at a median age of 28 years (range 11-46). Two were found to have HIF2A somatic mosaicism. No relatives were affected. All patients were diagnosed with polycythemia before age 8 and before PGL/SOM developed. PGLs were found at a median age of 17 years (range 8-38) and SOMs at 29 years (range 22-38). PGLs were multiple, recurrent and metastatic in 100, 100 and 29% of all cases, and SOMs in 40, 40 and 60%, respectively. All PGLs were primarily norepinephrine-producing. All patients had abnormal ophthalmologic findings and those with SOMs had gallbladder disease. Computed tomography (CT) and magnetic resonance imaging revealed cystic lesions at multiple sites and hemangiomas in 4 patients (57%), previously thought to be pathognomonic for von Hippel-Lindau disease. The most accurate radiopharmaceutical to detect PGL appeared to be [ 18 F]-fluorodihydroxyphenylalanine ([ 18 F]-FDOPA). Therefore, [ 18 F]-FDOPA PET/CT, not [ 68 Ga]-(DOTA)-[Tyr3]-octreotate ([ 68 Ga]-DOTATATE) PET/CT is recommended for tumor localization and aftercare in this syndrome. The long-term prognosis of the syndrome is unknown. However, to date no deaths occurred after 6 years follow-up. Physicians should be aware of this unique syndrome and its diagnostic and therapeutic challenges. © 2016 Society for Endocrinology.

  15. Effects of PPARα inhibition in head and neck paraganglioma cells.

    Directory of Open Access Journals (Sweden)

    Rosalba Florio

    Full Text Available Head and neck paragangliomas (HNPGLs are rare tumors that may cause important morbidity, because of their tendency to infiltrate the skull base. At present, surgery is the only therapeutic option, but radical removal may be difficult or impossible. Thus, effective targets and molecules for HNPGL treatment need to be identified. However, the lack of cellular models for this rare tumor hampers this task. PPARα receptor activation was reported in several tumors and this receptor appears to be a promising therapeutic target in different malignancies. Considering that the role of PPARα in HNPGLs was never studied before, we analyzed the potential of modulating PPARα in a unique model of HNPGL cells. We observed an intense immunoreactivity for PPARα in HNPGL tumors, suggesting that this receptor has an important role in HNPGL. A pronounced nuclear expression of PPARα was also confirmed in HNPGL-derived cells. The specific PPARα agonist WY14643 had no effect on HNPGL cell viability, whereas the specific PPARα antagonist GW6471 reduced HNPGL cell viability and growth by inducing cell cycle arrest and caspase-dependent apoptosis. GW6471 treatment was associated with a marked decrease of CDK4, cyclin D3 and cyclin B1 protein expression, along with an increased expression of p21 in HNPGL cells. Moreover, GW6471 drastically impaired clonogenic activity of HNPGL cells, with a less marked effect on cell migration. Notably, the effects of GW6471 on HNPGL cells were associated with the inhibition of the PI3K/GSK3β/β-catenin signaling pathway. In conclusion, the PPARα antagonist GW6471 reduces HNPGL cell viability, interfering with cell cycle and inducing apoptosis. The mechanisms affecting HNPGL cell viability involve repression of the PI3K/GSK3β/β-catenin pathway. Therefore, PPARα could represent a novel therapeutic target for HNPGL.

  16. Krebs cycle metabolite profiling for identification and stratification of pheochromocytomas/paragangliomas due to succinate dehydrogenase deficiency

    NARCIS (Netherlands)

    Richter, S; Peitzsch, M.; Rapizzi, E.; Lenders, J.W.M.; Qin, N.; Cubas, A.A. de; Schiavi, F.; Rao, J.U.; Beuschlein, F.; Quinkler, M.; Timmers, H.J.L.M.; Opocher, G.; Mannelli, M.; Pacak, K.; Robledo, M.; Eisenhofer, G.

    2014-01-01

    CONTEXT: Mutations of succinate dehydrogenase A/B/C/D genes (SDHx) increase susceptibility to development of pheochromocytomas and paragangliomas (PPGLs), with particularly high rates of malignancy associated with SDHB mutations. OBJECTIVE: We assessed whether altered succinate dehydrogenase

  17. Individual, family and offence characteristics of high risk childhood offenders: comparing non-offending, one-time offending and re-offending Dutch-Moroccan migrant children in the Netherlands

    Directory of Open Access Journals (Sweden)

    Stevens Gonneke WJM

    2011-10-01

    Full Text Available Abstract Background Childhood offenders are at an increased risk for developing mental health, social and educational problems later in life. An early onset of offending is a strong predictor for future persistent offending. Childhood offenders from ethnic minority groups are a vulnerable at-risk group. However, up until now, no studies have focused on them. Aims To investigate which risk factors are associated with (re-offending of childhood offenders from an ethnic minority. Method Dutch-Moroccan boys, who were registered by the police in the year 2006-2007, and their parents as well as a control group (n = 40 were interviewed regarding their individual and family characteristics. Two years later a follow-up analysis of police data was conducted to identify one-time offenders (n = 65 and re-offenders (n = 35. Results All groups, including the controls, showed substantial problems. Single parenthood (OR 6.0 and financial problems (OR 3.9 distinguished one-time offenders from controls. Reading problems (OR 3.8, having an older brother (OR 5.5 and a parent having Dutch friends (OR 4.3 distinguished re-offenders from one-time offenders. First offence characteristics were not predictive for re-offending. The control group reported high levels of emotional problems (33.3%. Parents reported not needing help for their children but half of the re-offender's families were known to the Child Welfare Agency, mostly in a juridical framework. Conclusion The Moroccan subgroup of childhood offenders has substantial problems that might hamper healthy development. Interventions should focus on reaching these families tailored to their needs and expectations using a multi-system approach.

  18. Literature survey on epidemiology and pathology of gangliocytic paraganglioma

    International Nuclear Information System (INIS)

    Okubo, Yoichiro; Sasai, Daisuke; Ishiwatari, Takao; Tsuchiya, Masaru; Hiruta, Nobuyuki; Wakayama, Megumi; Nemoto, Tetsuo; Kitahara, Kanako; Nakayama, Haruo; Shibuya, Kazutoshi; Yokose, Tomoyuki; Yamada, Manabu; Shimodaira, Kayoko

    2011-01-01

    Although gangliocytic paraganglioma (GP) has generally been regarded as a neuroendocrine tumor, its origin remains unclear. We therefore aimed to investigate the details of this disease by carefully analyzing and extracting common features of the disease as presented in selected publications. We searched for English and Japanese cases of GP using the PubMed and IgakuChuoZasshi databases on August 2010. We then extracted and sampled raw data from the selected publications and performed appropriate statistical analyses. Additionally, we evaluated the expression of hormone receptors based on our previously reported case. 192 patients with GP were retrieved from the databases. Patient ages ranged from 15 y to 84 y (mean: 52.3 y). The gender ratio was 114:76 (male to female, 2 not reported). Maximum diameter of the tumors ranged from 5.5 mm to 100 mm (mean: 25.0 mm). The duodenum (90.1%, 173/192) was found to be the most common site of the disease. In 173 patients with duodenal GP, gastrointestinal bleeding (45.1%, 78/173) was found to be the most common symptom of the disease, followed by abdominal pain (42.8%, 74/173), and anemia (14.5%, 25/173). Rate of lymph node metastasis was 6.9% (12/173). Our statistical analysis indicated that significant differences were found for gender between GP within the submucosal layer and exceeding the submucosal layer. Furthermore, our immunohistochemical evaluation showed that both epithelioid and pancreatic islet cells showed positive reactivity for progesterone receptors. Our literature survey revealed that there were many more cases of GP exceeding the submucosal layer than were expected. Meanwhile, our statistical analyses and immunohistochemical evaluation supported the following two hypotheses. First, vertical growth of GP might be affected by progesterone exposure. Second, the origin of GP might be pancreatic islet cells. However, it is strongly suspected that our data have been affected by publication bias and to confirm

  19. Heterogeneous genetic background of the association of pheochromocytoma/paraganglioma and pituitary adenoma: results from a large patient cohort.

    Science.gov (United States)

    Dénes, Judit; Swords, Francesca; Rattenberry, Eleanor; Stals, Karen; Owens, Martina; Cranston, Treena; Xekouki, Paraskevi; Moran, Linda; Kumar, Ajith; Wassif, Christopher; Fersht, Naomi; Baldeweg, Stephanie E; Morris, Damian; Lightman, Stafford; Agha, Amar; Rees, Aled; Grieve, Joan; Powell, Michael; Boguszewski, Cesar Luiz; Dutta, Pinaki; Thakker, Rajesh V; Srirangalingam, Umasuthan; Thompson, Chris J; Druce, Maralyn; Higham, Claire; Davis, Julian; Eeles, Rosalind; Stevenson, Mark; O'Sullivan, Brendan; Taniere, Phillipe; Skordilis, Kassiani; Gabrovska, Plamena; Barlier, Anne; Webb, Susan M; Aulinas, Anna; Drake, William M; Bevan, John S; Preda, Cristina; Dalantaeva, Nadezhda; Ribeiro-Oliveira, Antônio; Garcia, Isabel Tena; Yordanova, Galina; Iotova, Violeta; Evanson, Jane; Grossman, Ashley B; Trouillas, Jacqueline; Ellard, Sian; Stratakis, Constantine A; Maher, Eamonn R; Roncaroli, Federico; Korbonits, Márta

    2015-03-01

    Pituitary adenomas and pheochromocytomas/paragangliomas (pheo/PGL) can occur in the same patient or in the same family. Coexistence of the two diseases could be due to either a common pathogenic mechanism or a coincidence. The objective of the investigation was to study the possible coexistence of pituitary adenoma and pheo/PGL. Thirty-nine cases of sporadic or familial pheo/PGL and pituitary adenomas were investigated. Known pheo/PGL genes (SDHA-D, SDHAF2, RET, VHL, TMEM127, MAX, FH) and pituitary adenoma genes (MEN1, AIP, CDKN1B) were sequenced using next generation or Sanger sequencing. Loss of heterozygosity study and pathological studies were performed on the available tumor samples. The study was conducted at university hospitals. Thirty-nine patients with sporadic of familial pituitary adenoma and pheo/PGL participated in the study. Outcomes included genetic screening and clinical characteristics. Eleven germline mutations (five SDHB, one SDHC, one SDHD, two VHL, and two MEN1) and four variants of unknown significance (two SDHA, one SDHB, and one SDHAF2) were identified in the studied genes in our patient cohort. Tumor tissue analysis identified LOH at the SDHB locus in three pituitary adenomas and loss of heterozygosity at the MEN1 locus in two pheochromocytomas. All the pituitary adenomas of patients affected by SDHX alterations have a unique histological feature not previously described in this context. Mutations in the genes known to cause pheo/PGL can rarely be associated with pituitary adenomas, whereas mutation in a gene predisposing to pituitary adenomas (MEN1) can be associated with pheo/PGL. Our findings suggest that genetic testing should be considered in all patients or families with the constellation of pheo/PGL and a pituitary adenoma.

  20. Heterogeneous Genetic Background of the Association of Pheochromocytoma/Paraganglioma and Pituitary Adenoma: Results From a Large Patient Cohort

    Science.gov (United States)

    Dénes, Judit; Swords, Francesca; Rattenberry, Eleanor; Stals, Karen; Owens, Martina; Cranston, Treena; Xekouki, Paraskevi; Moran, Linda; Kumar, Ajith; Wassif, Christopher; Fersht, Naomi; Baldeweg, Stephanie E.; Morris, Damian; Lightman, Stafford; Agha, Amar; Rees, Aled; Grieve, Joan; Powell, Michael; Boguszewski, Cesar Luiz; Dutta, Pinaki; Thakker, Rajesh V.; Srirangalingam, Umasuthan; Thompson, Chris J.; Druce, Maralyn; Higham, Claire; Davis, Julian; Eeles, Rosalind; Stevenson, Mark; O'Sullivan, Brendan; Taniere, Phillipe; Skordilis, Kassiani; Gabrovska, Plamena; Barlier, Anne; Webb, Susan M.; Aulinas, Anna; Drake, William M.; Bevan, John S.; Preda, Cristina; Dalantaeva, Nadezhda; Ribeiro-Oliveira, Antônio; Garcia, Isabel Tena; Yordanova, Galina; Iotova, Violeta; Evanson, Jane; Grossman, Ashley B.; Trouillas, Jacqueline; Ellard, Sian; Stratakis, Constantine A.; Maher, Eamonn R.; Roncaroli, Federico

    2015-01-01

    Context: Pituitary adenomas and pheochromocytomas/paragangliomas (pheo/PGL) can occur in the same patient or in the same family. Coexistence of the two diseases could be due to either a common pathogenic mechanism or a coincidence. Objective: The objective of the investigation was to study the possible coexistence of pituitary adenoma and pheo/PGL. Design: Thirty-nine cases of sporadic or familial pheo/PGL and pituitary adenomas were investigated. Known pheo/PGL genes (SDHA-D, SDHAF2, RET, VHL, TMEM127, MAX, FH) and pituitary adenoma genes (MEN1, AIP, CDKN1B) were sequenced using next generation or Sanger sequencing. Loss of heterozygosity study and pathological studies were performed on the available tumor samples. Setting: The study was conducted at university hospitals. Patients: Thirty-nine patients with sporadic of familial pituitary adenoma and pheo/PGL participated in the study. Outcome: Outcomes included genetic screening and clinical characteristics. Results: Eleven germline mutations (five SDHB, one SDHC, one SDHD, two VHL, and two MEN1) and four variants of unknown significance (two SDHA, one SDHB, and one SDHAF2) were identified in the studied genes in our patient cohort. Tumor tissue analysis identified LOH at the SDHB locus in three pituitary adenomas and loss of heterozygosity at the MEN1 locus in two pheochromocytomas. All the pituitary adenomas of patients affected by SDHX alterations have a unique histological feature not previously described in this context. Conclusions: Mutations in the genes known to cause pheo/PGL can rarely be associated with pituitary adenomas, whereas mutation in a gene predisposing to pituitary adenomas (MEN1) can be associated with pheo/PGL. Our findings suggest that genetic testing should be considered in all patients or families with the constellation of pheo/PGL and a pituitary adenoma. PMID:25494863

  1. Posterior Retroperitoneoscopic Resection of Extra-adrenal Paraganglioma Located in the Aorto-caval Space.

    Science.gov (United States)

    Kang, Sang-Wook; Kandil, Emad; Kim, Min Jhi; Kim, Kwang Soon; Lee, Cho Rok; Jeong, Jong Ju; Nam, Kee-Hyun; Chung, Woong Youn; Park, Cheong Soo

    2018-04-01

    The posterior retroperitoneoscopic adrenalec tomy has several advantages compared with the transperitoneal approach such as a shorter and more direct route to the target organ, no breach of the intraperitoneal space, and no required retraction of the adjacent organs. It also is a safe procedure with a short learning curve.1 - 5 This report presents a challenging case of an extra-adrenal paraganglioma located in the aorto-caval space and managed using the retroperitoneal approach. A 39-year-old man was placed in the prone jackknife position, and three incisions were made in the right posterior abdominal wall for placement of the laparoscopic ports. The retroperitoneal space was entered with diathermy and blunt finger dissection, and retropneumoperitoneum was achieved with carbon dioxide insufflation pressure up to 18 mmHg. After identification of the right kidney and vessels, the tumor was meticulously dissected and excised with an energy device. The specimen was removed using a laparoscopic specimen retrieval bag, and the port sites were closed in layers. The operative time was 130 min, and the total blood loss was 30 ml. The tumor was diagnosed as a moderately differentiated extra-adrenal paraganglioma. The Von Hippel-Lindau gene mutation was detected using next-generation sequencing. The posterior retroperitoneoscopic approach is a safe, feasible, and effective method for excising an extra-adrenal paraganglioma even in the aorto-caval space. The authors suggest that this procedure is a useful surgical option for treatment of an aorto-caval paraganglioma for selected patients and by experienced surgeons.

  2. Subtotal resection for management of large jugular paragangliomas with functional lower cranial nerves.

    Science.gov (United States)

    Wanna, George B; Sweeney, Alex D; Carlson, Matthew L; Latuska, Richard F; Rivas, Alejandro; Bennett, Marc L; Netterville, James L; Haynes, David S

    2014-12-01

    To evaluate tumor control following subtotal resection of advanced jugular paragangliomas in patients with functional lower cranial nerves and to investigate the utility of salvage radiotherapy for residual progressive disease. Case series with planned chart review. Tertiary academic referral center. Patients who presented with advanced jugular paragangliomas and functional lower cranial nerves were analyzed. Primary outcome measures included extent of resection, long-term tumor control, need for additional treatment, and postoperative lower cranial nerve function. Twelve patients (mean age, 46.2 years; 7 women, 58.3%) who met inclusion criteria were evaluated between 1999 and 2013. The mean postoperative residual tumor volume was 27.7% (range, 3.5%-75.0%) of the preoperative volume. When the residual tumor volume was less than 20% of the preoperative volume, no tumor growth occurred over an average of 44.6 months of follow-up (P cranial neuropathy as a result of surgery. Subtotal resection of jugular paragangliomas with preservation of the lower cranial nerves is a viable management strategy. If more than 80% of the preoperative tumor volume is resected, the residual tumor seems less likely to grow. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2014.

  3. Functioning Mediastinal Paraganglioma Associated with a Germline Mutation of von Hippel-Lindau Gene

    Directory of Open Access Journals (Sweden)

    Thibault Bahougne

    2018-05-01

    Full Text Available We report the case of a 21-year old woman presenting with high blood pressure and raised normetanephrine levels. Indium-111-pentetreotide single photon-emission computed tomography with computed tomography (SPECT/CT and 2-deoxy-2-[fluorine-18]fluoro-d-glucose (FDG positron emission tomography/computed tomography (PET/CT imaging showing isolated tracer-uptake by a 2 cm tumor close to the costovertebral angle of the third thoracic vertebra. Thoracic surgery led to normalization of normetanephrine levels. Histological findings were consistent with the presence of a paraganglioma. Mutations in SDHA, SDHB, SDHC, SDHD, RET, SDHAF2, TMEM127, MAX, NF1, FH, MDH2, and EPAS1 were absent, but a heterozygous missense mutation, c.311G > T, was found in exon 1 of the von Hippel-Lindau gene, VHL, resulting in a glycine to valine substitution in the VHL protein at position 104, p.Gly104Val. This same mutation was found in both the mother and the 17-year old sister in whom a small retinal hemangioblastoma was also found. We diagnose an unusual functional mediastinal paraganglioma in this young patient with a germline VHL gene mutation, a mutation previously described as inducing polycythemia and/or pheochromocytoma but not paraganglioma or retinal hemangioblastoma.

  4. Aspirations and occupational achievements of Dutch fathers and mothers

    NARCIS (Netherlands)

    van der Horst, Mariska; van der Lippe, Tanja; Kluwer, Esther

    2014-01-01

    Purpose - To investigate how work and family aspirations relate to occupational achievements and gender differences herein. Design/methodology/approach - Using data from 2009 we examined the relationship between career and childrearing aspirations and occupational achievements of Dutch parents.

  5. Investment decision making in Dutch greenhouse horticulture

    NARCIS (Netherlands)

    Oude Lansink, A.G.J.M.; Verstegen, J.A.A.M.; Hengel, van den J.J.

    2001-01-01

    Panel data from Dutch horticultural firms over the period 1986-1998 were used to analyse the effects of different factors on investment decisions. The factors analysed relate to the firm operator and his family, the firm structure and the economic environment. Results show that firm-operator and

  6. Dutch solar vs conservation: an unbalanced match?

    NARCIS (Netherlands)

    Hensen, J.L.M.; Hoen, P.J.J.

    1987-01-01

    A real scale experiment with energy efficient singe-family housing has been carried out. This paper outlines the experiment and the main results. It concentrates on the results related to the balance of conservation vs solar measures and the factors which influence this balance. Under current Dutch

  7. The Dutch nuclear programme

    International Nuclear Information System (INIS)

    1986-01-01

    Engineering Services Ltd. has, in fulfillment of its contractual commitment to the Dutch Industrial Council for Energy and Environment Technology (IREM), investigated the broad capability of the Dutch industry in playing a major role in the supply of goods and services for the construction of nuclear plants in the Netherlands. The findings are summarized in this document. 5 tabs

  8. Biotechnology : A Dutch perspective

    NARCIS (Netherlands)

    Van Apeldoorn, J.H.F.

    1981-01-01

    Biotechnology: a Dutch Perspective assesses the future potential of biotechnology in the Netherlands. It has been published in English because it is felt that the Dutch case could be of relevance to other industrialised nations. Although the report is aimed primarily at policy planners and decision

  9. Dutch elm disease

    Science.gov (United States)

    James W. Walters

    1992-01-01

    Since its discovery in the United States in 1930, Dutch elm disease has killed thousands of native elms. The three native elms, American, slippery, and rock, have little or no resistance to Dutch elm disease, but individual trees within each species vary in susceptibility to the disease. The most important of these, American elm, is scattered in upland stands but is...

  10. Long Distance Endovascular Growth of Jugulotympanic Paraganglioma Evident in 68Ga-DOTATATE PET but Concealed on CT.

    Science.gov (United States)

    Avramovic, Nemanja; Weckesser, Matthias; Velasco, Aglaé; Stenner, Markus; Noto, Benjamin

    2017-02-01

    A 60-year-old woman was referred to contrast-enhanced CT for evaluation of jugular vein thrombosis incidentally detected by ultrasound. Contrast-enhanced CT showed an enhanced tumor of the right skull base highly suspicious of jugulotympanic paraganglioma. However, the jugular veins showed a nearly symmetric contrast enhancement without clear evidence of thrombosis. Consecutive Ga-DOTATATE PET/CT depicted high tumor uptake, which comprised the entire internal jugular vein. Endovascular growth of paraganglioma might be missed on contrast-enhanced CT because of high vascularization of the lesion. Ga-DOTATATE PET is suited for accurate determination of tumor extent.

  11. Sdhd and SDHD/H19 knockout mice do not develop paraganglioma or pheochromocytoma.

    Directory of Open Access Journals (Sweden)

    Jean-Pierre Bayley

    Full Text Available BACKGROUND: Mitochondrial succinate dehydrogenase (SDH is a component of both the tricarboxylic acid cycle and the electron transport chain. Mutations of SDHD, the first protein of intermediary metabolism shown to be involved in tumorigenesis, lead to the human tumors paraganglioma (PGL and pheochromocytoma (PC. SDHD is remarkable in showing an 'imprinted' tumor suppressor phenotype. Mutations of SDHD show a very high penetrance in man and we postulated that knockout of Sdhd would lead to the development of PGL/PC, probably in aged mice. METHODOLOGY/PRINCIPAL FINDINGS: We generated a conventional knockout of Sdhd in the mouse, removing the entire third exon. We also crossed this mouse with a knockout of H19, a postulated imprinted modifier gene of Sdhd tumorigenesis, to evaluate if loss of these genes together would lead to the initiation or enhancement of tumor development. Homozygous knockout of Sdhd results in embryonic lethality. No paraganglioma or other tumor development was seen in Sdhd KO mice followed for their entire lifespan, in sharp contrast to the highly penetrant phenotype in humans. Heterozygous Sdhd KO mice did not show hyperplasia of paraganglioma-related tissues such as the carotid body or of the adrenal medulla, or any genotype-related pathology, with similar body and organ weights to wildtype mice. A cohort of Sdhd/H19 KO mice developed several cases of profound cardiac hypertrophy, but showed no evidence of PGL/PC. CONCLUSIONS: Knockout of Sdhd in the mouse does not result in a disease phenotype. H19 may not be an initiator of PGL/PC tumorigenesis.

  12. Polycythemia and paraganglioma with a novel somatic HIF2A mutation in a male.

    Science.gov (United States)

    Toyoda, Hidemi; Hirayama, Jyunya; Sugimoto, Yuka; Uchida, Keiichi; Ohishi, Kohshi; Hirayama, Masahiro; Komada, Yoshihiro

    2014-06-01

    Recently, a new syndrome of paraganglioma, somatostatinoma, and polycythemia has been discovered (known as Pacak-Zhuang syndrome). This new syndrome, with somatic HIF2A gain-of-function mutations, has never been reported in male patients. We describe a male patient with Pacak-Zhuang syndrome who carries a newly discovered HIF2A mutation. Congenital polycythemias have diverse etiologies, including germline mutations in the oxygen-sensing pathway. These include von Hippel-Lindau (Chuvash polycythemia), prolyl hydroxylase domain-containing protein-2, and hypoxia-inducible factor-2α (HIF-2α). Somatic gain-of-function mutations in the gene encoding HIF-2α were reported in patients with paraganglioma and polycythemia and have been found exclusively in female patients. Through sequencing of the HIF2A using DNA from paraganglioma in 15-year-old male patient, we identified a novel mutation of HIF2A: a heterozygous C to A substitution at base 1589 in exon 12 of HIF2A. The mutation was not found in germline DNA from leukocytes. The C1589A mutations resulted in substitution of alanine 530 in the HIF-2α protein with glutamic acid. This mutation is undoubtedly associated with increased HIF-2α activity and increased protein half-life, because it affects the vicinity of the prolyl hydroxylase target residue, proline 531. To our knowledge, this is the first report describing Pacak-Zhuang syndrome with somatic gain-of-function mutation in HIF2A in a male patient. Congenital polycythemia of unknown origin should raise suspicion for the novel disorder Pacak-Zhuang syndrome, even in male patients. Copyright © 2014 by the American Academy of Pediatrics.

  13. First report of bilateral pheochromocytoma in the clinical spectrum of HIF2A-related polycythemia-paraganglioma syndrome.

    Science.gov (United States)

    Taïeb, David; Yang, Chunzhang; Delenne, Blandine; Zhuang, Zhengping; Barlier, Anne; Sebag, Fréderic; Pacak, Karel

    2013-05-01

    Molecular genetic research has so far resulted in the identification of 10 well-characterized susceptibility genes for hereditary pheochromocytoma (PHEO) or paraganglioma (PGL). Recently, a new syndrome characterized by multiple PGLs and somatostatinomas associated with congenital polycythemia due to somatic mutations in HIF2A has been reported. The aim of the study was to define the genetic defect in a new case of bilateral PHEO and multiple PGLs associated with congenital polycythemia. A female patient presented with neonatal polycythemia (treated by phlebotomies, 1 session approximately every 4 mo), mildly enlarged cerebral ventricles, and bilateral PHEO and multiple PGLs. There was no family history of any neuroendocrine tumor or polycythemia. Surgical removal of the tumors only temporarily normalized plasma erythropoietin (Epo) levels and discontinued phlebotomies. No germline mutations were initially detected in the SDHB, SDHC, SDHD, VHL, and PHD2 genes, known to be associated with polycythemia. The PHEOs presented with a typical noradrenergic biochemical phenotype. A heterozygous missense mutation (c.1589C>T) was identified in exon 12 of HIF2A, resulting in an alanine 530 substitution in the HIF-2α protein with valine (A530V). This somatic mutation was detected in the tissue from 1 PHEO and 1 PGL, with no HIF2A germline mutation found. This mutation led to stabilization of HIF-2α and hence a gain-of-function phenotype, as in previously published studies. This case represents the first association of a somatic HIF2A gain-of-function mutation with PHEO and congenital polycythemia, and it alerts physicians to perform proper genetic screening in patients presenting with multiple norepinephrine-producing PHEOs and polycythemia. This report also extends the previous findings of a new syndrome of only multiple PGLs, somatostatinomas, and polycythemia to multiple PHEOs.

  14. MOOC Introduction to Dutch

    NARCIS (Netherlands)

    den Hollander, Franciscus; Piersma, Kristien

    Interview met Margriet Hidding, Birgit Lijmbach en Jeroen van Engen, medewerkers van het Talencentrum en de makers van de Massive Open Online Course (MOOC) Introduction to Dutch over het grote succes van deze cursus

  15. Effect of family-style meals on energy intake and risk of malnutrition in dutch nursing home residents: A randomized controlled trial

    NARCIS (Netherlands)

    Nijs, K.A.N.D.; Graaf, de C.; Siebelink, E.; Blauw, Y.H.; Vanneste, V.; Kok, F.J.; Staveren, van W.A.

    2006-01-01

    Background. Social facilitation and meal ambiance have beneficial effects on food intake in healthy adults. Extrapolation to the nursing home setting may lead to less malnutrition among the residents. Therefore, we investigate the effect of family-style meals on energy intake and the risk of

  16. Gangliocytic paraganglioma of duodenum metastatic to lymph nodes and liver and extending into the retropancreatic space

    DEFF Research Database (Denmark)

    Amin, S M; Albrechtsen, N Wewer; Forster, J

    2013-01-01

    Gangliocytic paraganglioma (GP) is a rare benign neuroendocrine tumour found most often in the duodenum. To our knowledge, only a dozen cases of possibly malignant duodenal GP with local lymph node metastasis and only one case with liver metastasis have previously been published. Herein, we report...... an unusual case of GP of the duodenum spreading to the retropancreatic space and metastatic to the liver and lymph nodes. Additionally, the present tumour secreted pancreatic polypeptide (PP) which was detected in the serum during the follow-up period. We suggest that serum PP could be a valuable marker...

  17. The Dutch Economy 2009

    International Nuclear Information System (INIS)

    2010-09-01

    In the series 'The Dutch Economy' the Dutch Statistical Office describes and analyzes annual developments in enterprises, households and governments, and with respect to employment and the environment. One of the subjects is 'Economy and Environment' with the sub-topics 'Resources and Energy', 'Emissions' and 'Environmental Taxes'. Furthermore, in articles on specific themes current economic issues are discussed. One of those themes has the title 'Share of renewable energy in the Netherlands is still small'. [nl

  18. Anti-cancer potential of MAPK pathway inhibition in paragangliomas-effect of different statins on mouse pheochromocytoma cells

    NARCIS (Netherlands)

    Fliedner, S.M.; Engel, T.G.P.; Lendvai, N.K.; Shankavaram, U.; Nolting, S.; Wesley, R.; Elkahloun, A.G.; Ungefroren, H.; Oldoerp, A.; Lampert, G.; Lehnert, H.; Timmers, H.J.; Pacak, K.

    2014-01-01

    To date, malignant pheochromocytomas and paragangliomas (PHEOs/PGLs) cannot be effectively cured and thus novel treatment strategies are urgently needed. Lovastatin has been shown to effectively induce apoptosis in mouse PHEO cells (MPC) and the more aggressive mouse tumor tissue-derived cells

  19. Risk of malignant paraganglioma in SDHB-mutation and SDHD-mutation carriers: a systematic review and meta-analysis

    NARCIS (Netherlands)

    van Hulsteijn, L.T.; Dekkers, O.M.; Hes, F.J.; Smit, J.W.A.; Corssmit, E.P.

    2012-01-01

    The main objective of this study was to perform a systematic review and meta-analysis on the risk of developing malignant paraganglioma (PGL) in SDHB-mutation and SDHD-mutation carriers. PubMed, EMBASE, Web of Science, COCHRANE and Academic Search Premier (2000-August 2011) and references of key

  20. Plasma chromogranin A levels are increased in a small portion of patients with hereditary head and neck paragangliomas

    NARCIS (Netherlands)

    van Duinen, Nicolette; Kema, Ido P.; Romijn, Johannes A.; Corssmit, Eleonora P. M.

    2011-01-01

    The majority of patients with head and neck paragangliomas (HNPGL) have biochemically silent tumours. Chromogranin A (CgA) is a tumour marker for neuroendocrine tumours. To assess the role of CgA as a tumour marker in patients with hereditary HNPGL. We included 95 consecutive patients with

  1. Plasma chromogranin A levels are increased in a small portion of patients with hereditary head and neck paragangliomas

    NARCIS (Netherlands)

    van Duinen, Nicolette; Kema, Ido P.; Romijn, Johannes A.; Corssmit, Eleonora P. M.

    P>Context The majority of patients with head and neck paragangliomas (HNPGL) have biochemically silent tumours. Chromogranin A (CgA) is a tumour marker for neuroendocrine tumours. Objective To assess the role of CgA as a tumour marker in patients with hereditary HNPGL. Patients and Methods We

  2. In-111-octreotide is superior to I-123-metaiodobenzylguanidine for scintigraphic detection of head and neck paragangliomas

    NARCIS (Netherlands)

    Koopmans, Klaas Pieter; Jager, Pieter L.; Kema, Ido P.; Kersten, Michiel N.; Albers, Frans; Dullaart, Robin P. F.

    In this study, we evaluated the diagnostic yield of somatostatin receptor scintigraphy (SRS), 1-metaiodobenzylguanidine (MIBG) scintigraphy, and morphologic imaging (CT or MRI) in patients with head and neck paragangliomas. Methods: In a university hospital setting, patients considered to have head

  3. A rare case of spinal cord compression due to cervical spine metastases from paraganglioma of the jugular foramen-how should it be treated?

    Science.gov (United States)

    Kapetanakis, Stylianos; Chourmouzi, Danai; Gkasdaris, Grigorios; Katsaridis, Vasileios; Eleftheriadis, Eleftherios; Givissis, Panagiotis

    2018-02-01

    Paragangliomas are benign neoplasms that arise from the autonomic nervous system and the associated paraganglia. Although benign, they have been shown to possess metastatic potential. Involvement of the spine is rare. Even rarer is considered the involvement of the cervical spine. We report a case of a patient with a history of an extra-adrenal non-functional paraganglioma of the jugular foramen which was initially treated with intra-arterial embolization. After a 3-year disease-free follow-up, the patient was presented with symptoms of spinal cord compression due to spinal metastases in C2 and C3 vertebrae. The patient was then treated with surgical decompression and external beam radiation. Therapeutic management with additional treatment options is now under discussion by a multidisciplinary team. Paraganglioma of the jugular foramen with spinal metastasis is an uncommon presentation where increased physician awareness and long-term follow-up are mandatory for all patients with history of paraganglioma.

  4. Head and neck paragangliomas: A two-decade institutional experience and algorithm for management.

    Science.gov (United States)

    Smith, Joshua D; Harvey, Rachel N; Darr, Owen A; Prince, Mark E; Bradford, Carol R; Wolf, Gregory T; Else, Tobias; Basura, Gregory J

    2017-12-01

    Paragangliomas of the head and neck and cranial base are typically benign, slow-growing tumors arising within the jugular foramen, middle ear, carotid bifurcation, or vagus nerve proper. The objective of this study was to provide a comprehensive characterization of our institutional experience with clinical management of these tumors and posit an algorithm for diagnostic evaluation and treatment. This was a retrospective cohort study of patients undergoing treatment for paragangliomas of the head and neck and cranial base at our institution from 2000-2017. Data on tumor location, catecholamine levels, and specific imaging modalities employed in diagnostic work-up, pre-treatment cranial nerve palsy, treatment modality, utilization of preoperative angiographic embolization, complications of treatment, tumor control and recurrence, and hereditary status (ie, succinate dehydrogenase mutations) were collected and summarized. The mean (SD) age of our cohort was 51.8 (±16.1) years with 123 (63.4%) female patients and 71 (36.6%) male patients. Catecholamine-secreting lesions were found in nine (4.6%) patients. Fifty-one patients underwent genetic testing, with mutations identified in 43 (20 SDHD , 13 SDHB, 7 SDHD , 1 SDHA, SDHAF2, and NF1 ). Observation with serial imaging, surgical extirpation, radiation, and stereotactic radiosurgery were variably employed as treatment approaches across anatomic subsites. An algorithmic approach to clinical management of these tumors, derived from our longitudinal institutional experience and current empiric evidence, may assist otolaryngologists, radiation oncologists, and geneticists in the care of these complex neoplasms. 4.

  5. From Bad to Worse: Paraganglioma Diagnosis during Induction of Labor for Coexisting Preeclampsia

    Directory of Open Access Journals (Sweden)

    Sasima Dusitkasem

    2017-01-01

    Full Text Available Pheochromocytomas and extra-adrenal paragangliomas are catecholamine-secreting tumors that rarely occur in pregnancy. The diagnosis of these tumors in pregnancy can be challenging given that many of the signs and symptoms are commonly attributed to preeclampsia or other more common diagnoses. Early diagnosis and appropriate management are essential in optimizing maternal and fetal outcomes. We report a rare case of a catecholamine-secreting tumor in which diagnosis occurring at the time labor was being induced for concomitant preeclampsia with severe features. Her initial presentation in hypertensive crisis with other symptoms led to diagnostic workup for secondary causes of hypertension and led to eventual diagnosis of paraganglioma. Obtaining this diagnosis prior to delivery was essential, as this led to prompt multidisciplinary care, changed the course of her clinical management, and ultimately enabled good maternal and fetal outcomes. This case highlights the importance of maintaining a high index of suspicion for secondary causes of hypertension and in obstetric patients and providing timely multidisciplinary care.

  6. Paragangliomas: Presentation and management by radiotherapy at the Prince of Wales Hospital

    International Nuclear Information System (INIS)

    Smee, Robert I.; Jayasekara, Jayana; Williams, Janet R.; Hanna, Claire

    2015-01-01

    Paragangliomas are commonly treated with surgery, while radiotherapy is reserved for those that are inoperable or have relapsed. However, this retrospective study aims to determine whether radiotherapy is a viable initial treatment for paragangliomas. Of 73 tumours researched, 44 were diagnosed and treated from January 1967 to December 2012 at the Radiation Oncology Department at the Prince of Wales Hospital and thus were eligible for analysis. Median follow-up time was 3.5 years with a range of 1 to 40 years. Thirty-four tumours were treated with radiotherapy only, and 10 tumours were treated with both surgical resection and radiotherapy. Local control and cause-specific survival were the primary end points measured. Five-year local control rate for the population of 44 lesions was 89%; it was 100% in the group treated by radiotherapy alone, but only 50% in the group treated by surgery followed by radiotherapy, with radiation used for salvage. The difference in control rates between these two subset groups was found to be statistically significant (P < 0.001). Cause-specific survival rates for this eligible population at 5 and 10 years were 98% and 90%, respectively. After initial radiotherapy, 4 patients had improved cranial nerve function, there was clinical improvement in tinnitus, and one new cranial nerve deficit developed where a high dose was used. Radiotherapy has high local control rates and few complications. The local control and complication rates compare favourably to surgery.

  7. Dutch School in Geneva

    CERN Multimedia

    2011-01-01

    The Dutch School in Geneva organizes Dutch education for children in the primary and secondary school. For the school year 2011-2012 the Dutch School in Geneva is looking for qualified part time teachers Dutch for the primary and secondary school. If you are interested, please send your application and curriculum vitae in Dutch by email to the NTC coordinator, Mrs. Anne Saeys (anne.saeys@free.fr). More info : www.ntcgeneve.info De vereniging Nederlandse Taal en Cultuur De Taalfontein, kortweg NTC Genève, is een oudervereniging, die tot doel heeft de Nederlandse taal en de Nederlandse en Vlaamse cultuur in de regio Genève te stimuleren. Dit geschiedt d.m.v. het organiseren van Nederlandse les voor leerlingen zowel in het basisonderwijs als het voortgezet onderwijs. Voor het volgende schooljaar zoekt NTC parttime docenten (M/V) vanaf 2 uur per week, zowel voor het Primair Onderwijs als voor het Voortgezet Onderwijs, met name voor de IB-opleiding. Voor het Primair Onderwijs dienen ge&...

  8. Paternalizing mothers: feminist repertoires in contemporary Dutch civilizing offensives

    NARCIS (Netherlands)

    van den Berg, M.; Duyvendak, J.W.

    2012-01-01

    The stress in Dutch policy texts and policy practices on the emancipation of migrant women from their family and spouses goes hand in hand with a focus on precisely women’s role within the family: that of the mother. In this paper, we ask the question how this is possible. We aim to shed light on

  9. Dutch workers and time pressure : household and workplace characteristics

    NARCIS (Netherlands)

    Lippe, Tanja van der

    2007-01-01

    Balancing work with family life has become one of the most important issues for families nowadays. In this article I study the varying degrees of success of governance structures in households and firms in dealing with competing time claims. Using Dutch data from firms, employees and their spouses

  10. DutchParl: A corpus of parliamentary documents in Dutch

    NARCIS (Netherlands)

    Marx, M.; Schuth, A.

    2010-01-01

    A corpus called DutchParl is created which aims to contain all digitally available parliamentary documents written in the Dutch language. The first version of DutchParl contains documents from the parliaments of The Netherlands, Flanders and Belgium. The corpus is divided along three dimensions: per

  11. Paraganglioma of the head and neck region: treatment outcome and toxicity

    International Nuclear Information System (INIS)

    Ramakrishna, Naren; Bhaddy, Nasir; Holliday, Michael; Lee, D.-J.

    1997-01-01

    Purpose: To evaluate the long term results of treating paraganglioma (glomus tumor) of the head and neck region, in terms of local control and toxicities. Materials and Methods: We retrospectively reviewed the records of 38 patients with a diagnosis of a paraganglioma of the head and neck region who had received their therapy at our hospital from 1972 to 1994. Nine patients received definitive radiotherapy (XRT), 7 had surgical resection plus postoperative XRT and 22 patients had surgical resection only. There were 11 males and 27 females. The median age was 50 years with a range of 15 to 78. XRT, either definitive or postoperative, consisted of 1.8 Gy per fraction to a total of 45 to 50.4 cGy. In terms of surgery for the postoperative XRT group, resection of the glomus tumor in the neck or temporal bone was carried out in 6, and in one patient additional suboccipital craniotomy (2-staged) for removal of the intracranial portion of the tumor was performed. For the surgery alone group, 18 patients had resection of the glomus tumor in the neck or temporal bone, and 4 patients required additional two-staged suboccipital craniotomy. Results: In the XRT group, 14 patients had glomus jugulare and 2 patients had glomus typanicum tumor. With a median follow-up of 5 years (range: 2 to 21), one of the 16 patients developed local recurrence. In the surgery only group (15 glomus jugulare, 4 glomus typanicum and 3 glomus vegalis), 5 of the 22 patients developed local recurrence, with a median follow-up of 6 years (range: 2 to 15). The difference in the local control rates was not statistically significant (Fisher's test, p= 0.18). XRT was well tolerated. One patient developed facial nerve paralysis 5 years following XRT. Whereas in the surgery only group, the long term sequela was mainly cranial nerve dysfunction which affected the facial nerve in 2, the facial nerve plus the lingual nerve in one and facial plus cranial nerves VI, IX and XII in another (p=0.28). Conclusion

  12. Revisiting the Dutch hypothesis

    NARCIS (Netherlands)

    Postma, Dirkje S.; Weiss, Scott T.; van den Berge, Maarten; Kerstjens, Huib A. M.; Koppelman, Gerard H.

    The Dutch hypothesis was first articulated in 1961, when many novel and advanced scientific techniques were not available, such as genomics techniques for pinpointing genes, gene expression, lipid and protein profiles, and the microbiome. In addition, computed tomographic scans and advanced analysis

  13. Dutch Building Decree

    NARCIS (Netherlands)

    Scholten, N.P.M.

    2000-01-01

    On the 1 October 1992 a change in the Dutch building legislation took effect: the revised Housing Act, the Building Decree and the technical documents related to this legislation came into force. This publication contains an English translation of the Building Decree. In order to give an idea of the

  14. Survival of Dutch heathlands

    NARCIS (Netherlands)

    Diemont, W.H.

    1996-01-01

    OBJECTIVES OF THE THESIS

    Heathlands in The Netherlands are vanishing due to the invasion of trees. The transition from heathland to woodland in Dutch heathlands may either proceed directly or is preceded by the development of an intermediate grass heath. These changes are due to natural

  15. Dutch surgery in Japan

    NARCIS (Netherlands)

    van Gulik, Thomas M.; Nimura, Yuji

    2005-01-01

    An isolation policy was adopted in feudal Japan from 1639 to 1853 owing to the fear of foreign influence. During those 200 years of isolation, all foreigners were withheld from the country with the exception of the Dutch, who were permitted to establish a trading post on a small island in the Bay of

  16. Dutch Risk Assessment tools

    NARCIS (Netherlands)

    Venema, A.

    2015-01-01

    The ‘Risico- Inventarisatie- en Evaluatie-instrumenten’ is the name for the Dutch risk assessment (RA) tools. A RA tool can be used to perform a risk assessment including an evaluation of the identified risks. These tools were among the first online risk assessment tools developed in Europe. The

  17. 'A Dutch Miracle'

    NARCIS (Netherlands)

    Visser, Jelle; Hemerijck, Anton

    1997-01-01

    The Dutch polder model recently dubbed a 'success story' by Bill Clinton and Jacques Delors - plays a prominent role in current discussions about possibilities for a new 'capitalism with a social face', and appeals to experts all over the world. Just ten years ago the Swedish sociologist Goran

  18. The Dutch Grey Market

    NARCIS (Netherlands)

    Renneboog, L.D.R.; Spaenjers, C.

    2008-01-01

    When-issued trading concerns transactions in securities that have not yet been issued. This type of trade often takes place in a so-called ‘grey market’, in which all contracts are conditional on the issuance of the security. In this paper, we investigate the Dutch grey market for when-issued shares

  19. The Dutch Grey Market

    NARCIS (Netherlands)

    Renneboog, L.D.R.; Spaenjers, C.

    2008-01-01

    When-issued trading concerns transactions in securities that have not yet been issued. This type of trade often takes place in a so-called 'grey market', in which all contracts are conditional on the issuance of the security. In this paper, we investigate the Dutch grey market for when-issued shares

  20. Dutch Ships and Sailors

    NARCIS (Netherlands)

    de Boer, Victor; Hoekstra, F.G.; Leinenga, Jurjen; van Rossum, Matthias

    2014-01-01

    Dutch Ships and Sailors provides an infrastructure for maritime historical datasets, linking correlating data through semantic web technology. It brings together datasets related to recruitment and shipping in the East-India trade (mainly 18th century) and in the shipping of the northern provinces

  1. Dutch medical oath

    NARCIS (Netherlands)

    Westerveld, H. E.; Briet, J. W.; Houwaart, E. S.; Legemaate, J.; Meerman, Th J. A. M.; Breetvelt, E. J.; van der Wall, E.

    2005-01-01

    In the first part of this article, the booklet Dutch Medical Oath is reviewed. The content of the new oath is discussed as are the reasons for revision of the previous version of the oath. This is followed by a short history of the oath. In the second part of the article the oath is compared with

  2. Dutch house price fundamentals

    NARCIS (Netherlands)

    Haffner, M.E.A.; de Vries, P.

    2009-01-01

    This paper discusses house price developments in the Netherlands, specifically focussing on the question whether current house prices in the Dutch owner-occupied market are likely to decrease. We analyse three aspects of the question based on a literature review: (1) whether there is a house price

  3. Regression and local control rates after radiotherapy for jugulotympanic paragangliomas: Systematic review and meta-analysis

    International Nuclear Information System (INIS)

    Hulsteijn, Leonie T. van; Corssmit, Eleonora P.M.; Coremans, Ida E.M.; Smit, Johannes W.A.; Jansen, Jeroen C.; Dekkers, Olaf M.

    2013-01-01

    The primary treatment goal of radiotherapy for paragangliomas of the head and neck region (HNPGLs) is local control of the tumor, i.e. stabilization of tumor volume. Interestingly, regression of tumor volume has also been reported. Up to the present, no meta-analysis has been performed giving an overview of regression rates after radiotherapy in HNPGLs. The main objective was to perform a systematic review and meta-analysis to assess regression of tumor volume in HNPGL-patients after radiotherapy. A second outcome was local tumor control. Design of the study is systematic review and meta-analysis. PubMed, EMBASE, Web of Science, COCHRANE and Academic Search Premier and references of key articles were searched in March 2012 to identify potentially relevant studies. Considering the indolent course of HNPGLs, only studies with ⩾12 months follow-up were eligible. Main outcomes were the pooled proportions of regression and local control after radiotherapy as initial, combined (i.e. directly post-operatively or post-embolization) or salvage treatment (i.e. after initial treatment has failed) for HNPGLs. A meta-analysis was performed with an exact likelihood approach using a logistic regression with a random effect at the study level. Pooled proportions with 95% confidence intervals (CI) were reported. Fifteen studies were included, concerning a total of 283 jugulotympanic HNPGLs in 276 patients. Pooled regression proportions for initial, combined and salvage treatment were respectively 21%, 33% and 52% in radiosurgery studies and 4%, 0% and 64% in external beam radiotherapy studies. Pooled local control proportions for radiotherapy as initial, combined and salvage treatment ranged from 79% to 100%. Radiotherapy for jugulotympanic paragangliomas results in excellent local tumor control and therefore is a valuable treatment for these types of tumors. The effects of radiotherapy on regression of tumor volume remain ambiguous, although the data suggest that regression can

  4. Paragangliomas: presentation and management by radiotherapy at the Prince of Wales Hospital.

    Science.gov (United States)

    Smee, Robert I; Jayasekara, Jayana; Williams, Janet R; Hanna, Claire

    2015-04-01

    Paragangliomas are commonly treated with surgery, while radiotherapy is reserved for those that are inoperable or have relapsed. However, this retrospective study aims to determine whether radiotherapy is a viable initial treatment for paragangliomas. Of 73 tumours researched, 44 were diagnosed and treated from January 1967 to December 2012 at the Radiation Oncology Department at the Prince of Wales Hospital and thus were eligible for analysis. Median follow-up time was 3.5 years with a range of 1 to 40 years. Thirty-four tumours were treated with radiotherapy only, and 10 tumours were treated with both surgical resection and radiotherapy. Local control and cause-specific survival were the primary end points measured. Five-year local control rate for the population of 44 lesions was 89%; it was 100% in the group treated by radiotherapy alone, but only 50% in the group treated by surgery followed by radiotherapy, with radiation used for salvage. The difference in control rates between these two subset groups was found to be statistically significant (P < 0.001). Cause-specific survival rates for this eligible population at 5 and 10 years were 98% and 90%, respectively. After initial radiotherapy, 4 patients had improved cranial nerve function, there was clinical improvement in tinnitus, and one new cranial nerve deficit developed where a high dose was used. Radiotherapy has high local control rates and few complications. The local control and complication rates compare favourably to surgery. © 2014 The Royal Australian and New Zealand College of Radiologists.

  5. Duodenal gangliocytic paraganglioma with lymph node metastasis and an 8-year follow-up: a case report.

    Science.gov (United States)

    Barret, Maximilien; Rahmi, Gabriel; Duong van Huyen, Jean-Paul; Landi, Bruno; Cellier, Christophe; Berger, Anne

    2012-01-01

    Gangliocytic paraganglioma (GP) is a rare tumor, usually located in the second part of the duodenum. On pathological examination, GP is characterized by the association of the histological features of paragangliomas, ganglioneuromas, and carcinoid tumors. Classical clinical presentations are upper gastrointestinal bleeding and abdominal pain. Preoperative diagnosis is difficult because of the submucosal site of the tumor, with usually negative mucosal biopsies. Endoscopic ultrasound helps establish the diagnosis and allows lymph node staging, which will guide the choice of the treatment. If GPs usually follow a benign course, metastatic spread to regional lymph nodes treated by surgical resection alone has been reported. We report a case of GP with lymph node metastases treated by duodenopancreatectomy with long-term disease-free survival, suggesting that surgical resection is a reasonable approach for metastatic GPs.

  6. A significant discrepancy of uptake between I-131 MIBG and F-18 FDG in a patient with malignant paraganglioma

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jong Su; Kim, Hyun Keun; Choi, Kyu Young; Park, Hyung Ki; Kim, Eun Sil; Kim, Yun Kwon; Kim, So Yon [National Police Hospital, Seoul (Korea, Republic of)

    2007-06-15

    A 38-year-old man who was diagnosed with malignant paraganglioma underwent computed tomography (CT) and I-131 metaiodobenzylguanidine (MIBG) scan. CT showed extensive lymph node enlargement in right iliac area and retroperitoneum with severe hydronephrosis and mass on posterior bladder wall. However, I-131 MIBG scan didn't showed abnormal uptake. He also underwent F-18 fluorodeoxyglucose (FDG) positron emission tomography/CT for localizing accurate tumor site. F-18 FDG PET/CT showed multiple metastases of left supraclavicular, hilar, mediastinal para-aortic, inguinal, right iliac lymph nodes, lung, vertebrae, and pelvis. There are a few reports showing that the F-18 FDG PET/CT is helpful for staging and localizing tumor site of patients who are diagnosed with negative on the MIBG scans. Thus, we report a case with paraganglioma which showed negative I-131 MIBG scan, but revealed multiple intense hypermetabolic foci in F-18 FDG PET/CT.

  7. Validity of the Parental Burnout Inventory Among Dutch Employees.

    Science.gov (United States)

    Van Bakel, Hedwig J A; Van Engen, Marloes L; Peters, Pascale

    2018-01-01

    The purpose of this study was to validate the Parental Burnout Inventory (PBI) in a Dutch sample of working parents. The Dutch version of the PBI and questionnaires about work were administered to 627 working parents, with at least one child living at home. We investigated whether the tri-dimensional structure of the PBI held in a sample of male and female employed parents. Furthermore, we examined the relationships between PBI and the constructs work-related burnout, depressive mood, parenting stress and work-family conflict, which we assessed with widely used and validated instruments, i.e., emotional exhaustion [a subscale of the Dutch version of Maslach's Burnout Inventory], a Dutch Parental Stress Questionnaire and Work-Family Conflict. The results support the validity of a tri-dimensional parental burnout syndrome, including exhaustion, distancing and inefficacy. Low to moderate correlations between parents' burnout symptoms and professional exhaustion, parenting stress, depressive complaints and work-family conflict experiences were found, suggesting that the concept of PBI differs significantly from the concepts of job burnout, depression and stress, respectively. The current study confirms that some parents are extremely exhausted by their parental role. However, the number of Dutch employees reporting extreme parental burnout is rather low.

  8. Validity of the Parental Burnout Inventory Among Dutch Employees

    Directory of Open Access Journals (Sweden)

    Hedwig J. A. Van Bakel

    2018-05-01

    Full Text Available The purpose of this study was to validate the Parental Burnout Inventory (PBI in a Dutch sample of working parents. The Dutch version of the PBI and questionnaires about work were administered to 627 working parents, with at least one child living at home. We investigated whether the tri-dimensional structure of the PBI held in a sample of male and female employed parents. Furthermore, we examined the relationships between PBI and the constructs work-related burnout, depressive mood, parenting stress and work-family conflict, which we assessed with widely used and validated instruments, i.e., emotional exhaustion [a subscale of the Dutch version of Maslach’s Burnout Inventory], a Dutch Parental Stress Questionnaire and Work-Family Conflict. The results support the validity of a tri-dimensional parental burnout syndrome, including exhaustion, distancing and inefficacy. Low to moderate correlations between parents’ burnout symptoms and professional exhaustion, parenting stress, depressive complaints and work-family conflict experiences were found, suggesting that the concept of PBI differs significantly from the concepts of job burnout, depression and stress, respectively. The current study confirms that some parents are extremely exhausted by their parental role. However, the number of Dutch employees reporting extreme parental burnout is rather low.

  9. Novel use of F-DOPA PET/CT imaging in a child with paraganglioma/pheochromocytoma syndrome

    International Nuclear Information System (INIS)

    Levine, Daniel S.; Nadel, Helen R.; Metzger, Daniel L.; Oviedo, Angelica; Adam, Michael J.; Skarsgard, Erik

    2011-01-01

    We report the use of F-DOPA PET/CT imaging in the evaluation of a teenager with marked hypertension and right pararenal, left adrenal and left para-aortic mass lesions. The use of the modality for this clinical application has not been described previously within the pediatric imaging literature. The value of this technique relative to conventional imaging modalities is discussed and warrants consideration of its use, if available, for evaluating children with suspected paragangliomas/pheochromocytomas. (orig.)

  10. Novel use of F-DOPA PET/CT imaging in a child with paraganglioma/pheochromocytoma syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Levine, Daniel S.; Nadel, Helen R. [University of British Columbia, Department of Radiology and Nuclear Medicine, British Columbia Children' s Hospital, Vancouver (Canada); Metzger, Daniel L. [University of British Columbia, Department of Pediatrics, Division of Endocrinology, British Columbia Children' s Hospital, Vancouver (Canada); Oviedo, Angelica [University of British Columbia, Department of Pathology, British Columbia Children' s Hospital, Vancouver (Canada); Adam, Michael J. [University of British Columbia, Department of PET Chemistry, Tri-University Meson Facility (TRIUMF), Vancouver (Canada); Skarsgard, Erik [University of British Columbia, Department of Surgery, British Columbia Children' s Hospital, Vancouver (Canada)

    2011-10-15

    We report the use of F-DOPA PET/CT imaging in the evaluation of a teenager with marked hypertension and right pararenal, left adrenal and left para-aortic mass lesions. The use of the modality for this clinical application has not been described previously within the pediatric imaging literature. The value of this technique relative to conventional imaging modalities is discussed and warrants consideration of its use, if available, for evaluating children with suspected paragangliomas/pheochromocytomas. (orig.)

  11. Malignant paraganglioma of the prostate: case report, depiction by meta-iodobenzylguanidine scintigraphy and review of the literature

    International Nuclear Information System (INIS)

    Shapiro, B.; Gonzalez, E.; Weissman, A.; Ann Arbor, Univ. of Michigan; McHugh, T.; Markel, S.F.

    1997-01-01

    Objective: to describe the 123-I-MIBG scintigraphic, CT, MRI, operative and pathological findings in a case of malignant prostatic paraganglioma and to review the literature on this very rare tumor. Experimental design: clinical imaging and pathological correlation of data in a referred patient. Setting: regional referral center and tertiary referral academic medical center. Patient: 17 year old man presenting with painless hematuria and a large prostatic mass. Interventions and measures: renal ultrasound, transrectal ultrasound, ultrasound guided prostatic biopsy, pelvic CT and MRI, planar and SPECT 123-I-MIBG scintigraphy, and surgical exploration. Results: the patient had a significant hydronephrosis of the left kidney and marked enlargement (120 ml) of the prostate gland by ultrasound. Ultrasound guided biopsies of the prostate and a left pelvic lymph node revealed a neuroendocrine tumor staining positive for chromogranin. CT and MRI revealed a large tumor of the prostate invading the seminal vesicles, bladder and rectum with extensive pelvic lymph node spread. The primary tumor and one of the nodes were shown to be 123-I-MIBG avid confirming the neuroendocrine nature of the tumor. The lesion was unresponsive to chemotherapy and unresectable at surgical exploration. Conclusions: today there have only been 5 reports of prostatic paragangliomas. To our knowledge this is the first to have been studied by MIBG scintigraphy and like most paragangliomas it was MIBG-avid

  12. Dutch ministerial visit

    CERN Multimedia

    2007-01-01

    Dutch Minister of Education, Culture and Science R. Plasterk (third from left) in the ATLAS cavern with NIKHEF Director F. Linde, CERN Chief Scientific Officer J. Engelen, Ambassador J. van Eenennaam, ATLAS Collaboration Spokesperson P. Jenni, Mission Representative G. Vrielink and ATLAS Magnet Project Leader H. ten Kate.Minister of Education, Culture and Science from the Kingdom of the Netherlands, Ronald Plasterk, visited CERN on 25th October. With Jos Engelen, CERN Scientific Director, as his guide he visited Point 1 of the LHC tunnel and ATLAS, where Nikhef (the national institute for subatomic physics, a Dutch government and university collaboration) constructed all 96 of the largest muon drift chambers in the barrel as well as parts of the magnet system, the inner detector, the DAQ and triggering. Overall the Netherlands contribute 4.5% to the annual CERN budget and the minister’s visit celebrated the contributions of the 79 ...

  13. Dutch taboo norms.

    Science.gov (United States)

    Roest, Sander A; Visser, Tessa A; Zeelenberg, René

    2018-04-01

    This article provides norms for general taboo, personal taboo, insult, valence, and arousal for 672 Dutch words, including 202 taboo words. Norms were collected using a 7-point Likert scale and based on ratings by psychology students from the Erasmus University Rotterdam in The Netherlands. The sample consisted of 87 psychology students (58 females, 29 males). We obtained high reliability based on split-half analyses. Our norms show high correlations with arousal and valence ratings collected by another Dutch word-norms study (Moors et al.,, Behavior Research Methods, 45, 169-177, 2013). Our results show that the previously found quadratic relation (i.e., U-shaped pattern) between valence and arousal also holds when only taboo words are considered. Additionally, words rated high on taboo tended to be rated low on valence, but some words related to sex rated high on both taboo and valence. Words that rated high on taboo rated high on insult, again with the exception of words related to sex many of which rated low on insult. Finally, words rated high on taboo and insult rated high on arousal. The Dutch Taboo Norms (DTN) database is a useful tool for researchers interested in the effects of taboo words on cognitive processing. The data associated with this paper can be accessed via the Open Science Framework ( https://osf.io/vk782/ ).

  14. Dutch euthanasia revisited.

    Science.gov (United States)

    Fenigsen, R

    1997-01-01

    The results of a follow-up study of euthanasia by the Dutch government, five years after the first study, were published on November 26, 1996. This article provides a detailed review of the two reports comparing and contrasting the statistics cited therein. The author notes that the "rules of careful conduct" proposed by the courts and by the Royal Dutch Society of Medicine were frequently disregarded. Special topics included for the first time in the second study were the notification and non-prosecution procedure, euthanasia of newborns and infants, and assisted suicide in psychiatric practice. The authors of the follow-up report state that it would be desirable to reduce the number of "terminations of life without patients' request," but this must be the common responsibility of the doctor and the patient. They suggest that the person who does not wish to have his life terminated should declare this clearly, in advance, verbally and in writing, preferably in the form of a living will. Involuntary euthanasia was rampant in 1990 and equally rampant in 1995. The author concludes that Dutch doctors who practice euthanasia are not on the slippery slope. From the very beginning, they have been at the bottom.

  15. Will Dutch Become Flemish? Autonomous Developments in Belgian Dutch

    Science.gov (United States)

    Van de Velde, Hans; Kissine, Mikhail; Tops, Evie; van der Harst, Sander; van Hout, Roeland

    2010-01-01

    In this paper a series of studies of standard Dutch pronunciation in Belgium and the Netherlands is presented. The research is based on two speech corpora: a diachronic corpus of radio speech (1935-1995) and a synchronic corpus of Belgian and Netherlandic standard Dutch from different regions at the turn of the millennium. It is shown that two…

  16. Dutch self-employment between 1980 and 1997

    NARCIS (Netherlands)

    Blumberg, B.F.; Graaf, P.M. de; Arum, R.; Müller, W.

    2004-01-01

    Data from two retrospective life course surveys, the Family Survey Dutch Population 1992 and 1998, are used to investigate the determinants of entry into and exit out of self-employment in the Netherlands. First, explanations for trends in self-employment in the Netherlands are offered, and the

  17. Entrepreneurial innovation in chains and networks in Dutch greenhouse horticulture

    NARCIS (Netherlands)

    Pannekoek, L.; Kooten, van O.; Kemp, R.G.M.; Omta, S.W.F.

    2005-01-01

    This paper focuses on the critical success factors in entrepreneurial innovation, and the role of cooperation with chain partners, knowledge institutions and colleague firms in the Dutch glasshouse industry. This industry, typified by a large number of family-owned entrepreneurial firms, is

  18. Malignant extra-adrenal pancreatic paraganglioma: case report and literature review

    International Nuclear Information System (INIS)

    Al-Jiffry, Bilal O; AlNemary, Yasir; Khayat, Samah H; Haiba, Moutaz; Hatem, Mohammed

    2013-01-01

    Pancreatic paragangliomas are rare tumors, with only 16 reported cases to date. One of these cases demonstrates metastasis to lymph node, while another case was functional, however, none of these cases showed malignant and large, pancreatic paraganglioma with marked invasion. Also another unique feature was the age of our patient compared to the average reported ages in published literature (42–85 years). A 19-year-old woman presented with a one-year history of intermittent abdominal pain. Physical examination showed a palpable mass in the right upper abdomen, but initial laboratory results were within normal ranges; tumor markers (CEA, AFP, and CA19-9) were negative. An abdominal and pelvic computed tomography (CT) scan showed a well-defined retroperitoneal para-aortic mass. The CT scan revealed that the surrounding lymph nodes were not enlarged, but the liver showed evidence of parenchymal infiltration. Intraoperatively, a large, firm tumor originating from the head of pancreas was found pushing on the caudate hepatic lobe and the inferior vena cava (IVC). The tumor was resected through a pancreaticoduodenectomy, involving segment VI of the liver and a small segment of the IVC. The blood pressure spiked (>220 mm Hg) when the tumor was manipulated during the operation. The final pathology report showed a 9-cm tumor with lymphovascular invasions; immunohistochemistry was positive for synaptophysin and chromogranin. All resection margins were negative and 1/15 lymph nodes was positive for metastasis. Post-operative recovery was unremarkable. One month after discharge, the patient was re-admitted with abdominal pain and found to have an abdominal collection at the resection site, which was drained under CT guidance. She received a therapeutic dose of I 131 -metaiodobenzylguanidine (MIBG). Follow-ups showed the absence of recurrence, and she has remained disease free. This patient was an extraordinary example of a rare tumor. Even more remarkable was that the tumor

  19. Dutch Colonial Nostalgia Across Decolonisation

    NARCIS (Netherlands)

    Bijl, P.

    2013-01-01

    This article argues that nostalgia for colonialism in the Netherlands, the so called tempo doeloe culture, is not a specifically postcolonial phenomenon caused by the collapse of the Dutch empire in Asia. In fact, nostalgia for the Dutch East Indies can be traced back to the nineteenth century, when

  20. Income smoothing by Dutch hospitals

    NARCIS (Netherlands)

    Boterenbrood, D.R.

    2014-01-01

    Research indicates that hospitals manage their earnings. However, these findings might be influenced by methodological issues. In this study, I exploit specific features of Dutch hospitals to study income smoothing while limiting these methodological issues. The managers of Dutch hospitals have the

  1. The Dutch surgical colorectal audit

    NARCIS (Netherlands)

    van Leersum, N. J.; Snijders, H. S.; Henneman, D.; Kolfschoten, N. E.; Gooiker, G. A.; ten Berge, M. G.; Eddes, E. H.; Wouters, M. W. J. M.; Tollenaar, R. A. E. M.; Bemelman, W. A.; van Dam, R. M.; Elferink, M. A.; Karsten, Th M.; van Krieken, J. H. J. M.; Lemmens, V. E. P. P.; Rutten, H. J. T.; Manusama, E. R.; van de Velde, C. J. H.; Meijerink, W. J. H. J.; Wiggers, Th; van der Harst, E.; Dekker, J. W. T.; Boerma, D.

    2013-01-01

    In 2009, the nationwide Dutch Surgical Colorectal Audit (DSCA) was initiated by the Association of Surgeons of the Netherlands (ASN) to monitor, evaluate and improve colorectal cancer care. The DSCA is currently widely used as a blueprint for the initiation of other audits, coordinated by the Dutch

  2. Dutch radiodiagnostics viewed internationally

    International Nuclear Information System (INIS)

    Valois, J.C. de

    1990-01-01

    Dutch radiodiagnostics viewed internationally. - A quantitative description of diagnostic radiology is given in terms of radiological density (the number of radiological examinations per 1000 inhibitants), consumptions of roentgen film and contrast media. The data concerning examinations were recorded by a yearly inquiry system addressing all Dutch radiologists. The consumption of film and contrast media were derived from the data banks of the industries. In comparing these data with the data for Western Europe, Japan and the United States it is remarkable that diagnostic radiology scores lowest in regard to density, film consumption and use of contrast media. Only in the use of 35 mm cinefilm (coronary angiography) is The Netherlands number 2 on the list preceded by the United States. As a consequence radiation exposure of the population caused by diagnostic radiology is low in The Netherlands. Although the technical condition of the equipment is good due to regular and preventive service the life-span of the radiological equipment is gradually increasing beyond the limits of the normal economic depreciation. Growing arrears are found in the application of new technology: ultrasound, computer tomography and magnetic resonance imaging. The substitution of high osmolar contrast media by low osmolar media is also laggin gbehind. (author). 10 refs.; 1 fig.; 4 tab

  3. Dutch offshore skills assessed

    Energy Technology Data Exchange (ETDEWEB)

    Schaap, P

    1988-11-01

    The position is described of the different Dutch industries involved in the oil and gas exploitation business in the North Sea. Mentioned are, in the first place, the activities of towing services Smit Tak, and Wijsmuller. A well known drilling contractor is Neddrill. Drilling rigs are often designed in cooperation with consulting engineers firms Protech International, Heerema Engineering, Global Engineering, KTI, Fluor, and Tebodin. Drilling rigs constructors Grootint, Mercon Steel Structures, Heerema Havenbedrijf, HCG, Boele, and IHC have a sound name in the offshore construction scene. Offshore contractors like Heerema, Smit Tak, Volker Stevin and Allseas Engineering should be mentioned too. Special attention is given to the operation of saving drilling rigs in the Ekofisk field. Years of exploitation of oil and gas from this field resulted in subsidence of the sea floor, which endangered the drilling rigs. Hydraudyne designed the hydraulic lifter necessary for this saving operation. Dutch firms in this field have an estimated yearly turnover of Dfl 3 x 10/sup 9/. 6 figs.

  4. Results of a systematic literature review of treatment modalities for jugulotympanic paraganglioma, stratified per Fisch class.

    Science.gov (United States)

    Jansen, T T G; Timmers, H J L M; Marres, H A M; Kaanders, J H A M; Kunst, H P M

    2018-04-01

    Key for successful jugulotympanic paraganglioma management is a personalised approach aiming for the best practice for each individual patient. To this end, a systematic review is performed, evaluating the local control and complication rates for the different treatment modalities stratified by the broadly accepted Fisch classification. A systematic literature review according to the PRISMA statement was performed. A detailed overview of individual treatment outcomes per Fisch class is provided. Local control, cranial nerve damage, complications, function recovery. Eighteen studies were selected, resembling 83 patients treated with radiotherapy and 299 with surgery. Excellent local control was found post-surgery for class A and B tumours, and risk of cranial nerve damage was class C1-4 tumours, local control was 80%-95% post-surgery (84% post-radiotherapy), and cranial nerve damage was found in 71%-76% (none post-radiotherapy; P class. For class C1-4De/Di tumours, local control was 38%-86% (98% post-radiotherapy; P class. For class A and B tumours, surgery is a suitable treatment option. For class C and D tumours, radiotherapy results in lower complication rates and similar or better local control rates when compared to the surgical group. © 2017 John Wiley & Sons Ltd.

  5. Radiotherapy as adjunct to surgery for malignant carotid body paragangliomas presenting with lymph node metastases

    International Nuclear Information System (INIS)

    Mayer, R.; Poschauko, J.; Fruhwirth, J.; Beham, A.; Groell, R.

    2000-01-01

    Between 1985 and 1994, 3 female patients (51 to 65 years of age) were referred for postoperative radiotherapy after complete (2) or incomplete (1) surgical excision of a malignant carotid paraganglioma (Shamblin III). Preoperative angiographic embolization of the tumor-supplying arteries was performed in all cases. In 2 patients resection of the internal carotid artery and reconstruction by saphenous vein graft was necessary. Continuous course radiotherapy of the tumor bed (50 to 56 Gy/2 Gy) and regional lymph nodes (50 Gy) using photon beams was delivered in 2 patients. The third patient having had incomplete resection cancelled radiotherapy after 4 Gy. Results: Within an observation time of 110 and 119 months no evidence of recurrence was obtained in both patients irradiated postoperatively. The third patient died of progressive disease. Twelve months after the withdrawn irradiation she presented with a tumor progression into the brain and an ulcerated mass on the right side of the neck and was irradiated consecutively for palliation. In none of the patients severe acute or late radiation-induced complications were observed. (orig.) [de

  6. Molecular and therapeutic advances in the diagnosis and management of malignant pheochromocytomas and paragangliomas.

    LENUS (Irish Health Repository)

    Lowery, Aoife J

    2013-01-01

    Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare catecholamine-secreting tumors derived from chromaffin cells originating in the neural crest. These tumors represent a significant diagnostic and therapeutic challenge because the diagnosis of malignancy is frequently made in retrospect by the development of metastatic or recurrent disease. Complete surgical resection offers the only potential for cure; however, recurrence can occur even after apparently successful resection of the primary tumor. The prognosis for malignant disease is poor because traditional treatment modalities have been limited. The last decade has witnessed exciting discoveries in the study of PCCs and PGLs; advances in molecular genetics have uncovered hereditary and germline mutations of at least 10 genes that contribute to the development of these tumors, and increasing knowledge of genotype-phenotype interactions has facilitated more accurate determination of malignant potential. Elucidating the molecular mechanisms responsible for malignant transformation in these tumors has opened avenues of investigation into targeted therapeutics that show promising results. There have also been significant advances in functional and radiological imaging and in the surgical approach to adrenalectomy, which remains the mainstay of treatment for PCC. In this review, we discuss the currently available diagnostic and therapeutic options for patients with malignant PCCs and PGLs and detail the molecular rationale and clinical evidence for novel and emerging diagnostic and therapeutic strategies.

  7. Metabologenomics of Phaeochromocytoma and Paraganglioma: An Integrated Approach for Personalised Biochemical and Genetic Testing.

    Science.gov (United States)

    Eisenhofer, Graeme; Klink, Barbara; Richter, Susan; Lenders, Jacques Wm; Robledo, Mercedes

    2017-04-01

    The tremendous advances over the past two decades in both clinical genetics and biochemical testing of chromaffin cell tumours have led to new considerations about how these aspects of laboratory medicine can be integrated to improve diagnosis and management of affected patients. With germline mutations in 15 genes now identified to be responsible for over a third of all cases of phaeochromocytomas and paragangliomas, these tumours are recognised to have one of the richest hereditary backgrounds among all neoplasms. Depending on the mutation, tumours show distinct differences in metabolic pathways that relate to or even directly impact clinical presentation. At the same time, there has been improved understanding about how catecholamines are synthesised, stored, secreted and metabolised by chromaffin cell tumours. Although the tumours may not always secrete catecholamines it has become clear that almost all continuously produce and metabolise catecholamines. This has not only fuelled changes in laboratory medicine, but has also assisted in recognition of genotype-biochemical phenotype relationships important for diagnostics and clinical care. In particular, differences in catecholamine and energy pathway metabolomes can guide genetic testing, assist with test interpretation and provide predictions about the nature, behaviour and imaging characteristics of the tumours. Conversely, results of genetic testing are important for guiding how routine biochemical testing should be employed and interpreted in surveillance programmes for at-risk patients. In these ways there are emerging needs for modern laboratory medicine to seamlessly integrate biochemical and genetic testing into the diagnosis and management of patients with chromaffin cell tumours.

  8. Additional value of hybrid SPECT/CT systems in neuroendocrine tumors, adrenal tumors, pheochromocytomas and paragangliomas.

    Science.gov (United States)

    Wong, K K; Chondrogiannis, S; Fuster, D; Ruiz, C; Marzola, M C; Giammarile, F; Colletti, P M; Rubello, D

    The aim of this review was to evaluate the potential advantages of SPECT/CT hybrid imaging in the management of neuroendocrine tumors, adrenal tumors, pheochromocytomas and paragangliomas. From the collected data, the superiority of fused images was observed as providing both functional/molecular and morphological imaging compared to planar imaging. This provided an improvement in diagnostic imaging, with significant advantages as regards: (1) precise locating of the lesions; (2) an improvement in characterization of the findings, resulting higher specificity, improved sensitivity, and overall greater accuracy, (3) additional anatomical information derived from the CT component; (4) CT-based attenuation correction and potential for volumetric dosimetry calculations, and (5) improvement on the impact on patient management (e.g. in better defining treatment plans, in shortening surgical operating times). It can be concluded that SPECT/CT hybrid imaging provides the nuclear medicine physician with a powerful imaging modality in comparison to planar imaging, providing essential information about the location of lesions, and high quality homogeneous images. Copyright © 2016 Elsevier España, S.L.U. y SEMNIM. All rights reserved.

  9. Paraganglioma of the urinary bladder: a clinicopathologic spectrum of a series of 14 cases emphasizing diagnostic dilemmas.

    Science.gov (United States)

    Menon, Santosh; Goyal, Pankaj; Suryawanshi, Pallavi; Tongaonkar, Hemant; Joshi, Amit; Bakshi, Ganesh; Desai, Sangeeta

    2014-01-01

    Paraganglioma (PG) of the urinary bladder is a rare neuroendocrine neoplasm, accounting for bladder tumours. Distinction from urothelial carcinoma is imperative as management and prognosis vary markedly. In this report, we describe our experience with the histopathology of paragangliomas of the urinary bladder with emphasis on the histologic features that have led to their being misdiagnosed as conventional urothelial cancer and, most importantly, those that will help pathologists recognize this rare tumor of the bladder. All cases of PG of urinary bladder diagnosed at our institute from 2002-2012 were retrieved and diagnosis confirmed in accordance with WHO classification. Clinical and treatment details were obtained from hospital medical records. Fourteen cases of PG of urinary bladder including 5 consult cases were analysed. These included 11 transurethral resections ± partial cystectomies, 2 partial cystectomies and 1 radical cystectomy. Two out of the 5 consult cases had been submitted with a diagnosis of urothelial carcinoma and 1 with that of a rhabdomyosarcoma. Age ranged from 15-84 years (median, 43 years) with a male to female ratio of 1:2.5. Presenting symptoms were haematuria, dysuria and flank pain; only 1 case had antecedent hypertension. Histologically, typical 'zellballen' (72%), diffuse (21%) and ribbon-like (7%) growth patterns amidst a richly vascularised stroma were seen. Muscularis propria invasion and necrosis was present in 72% and 21%, respectively. Substantial cautery artifacts led to misdiagnosis in the 3 erroneous cases. Tumour cells were positive for chromogranin, synaptophysin; sustentacular cells were S-100 positive. Follow up was available in 6 patients; median follow-up was 29 months (8-120 months). One patient developed distant metastasis in cervical lymph node 10 years after diagnosis; remaining were alive without evidence of disease. Paraganglioma of the urinary bladder is a rare tumor and may be misdiagnosed as urothelial cancer

  10. Remembering Dutch-Moluccan radicalism

    DEFF Research Database (Denmark)

    Marselis, Randi Lorenz

    2016-01-01

    This article examines memory politics in relation to radical actions of young Dutch-Moluccans, more specifically a train hijacking in 1977 at the village of De Punt in the Netherlands. The article examines how these historical events were remembered in the drama-documentary television film, De Punt...... of the web debate examines how viewers reacted to this interpretation. The web debate functioned as a participatory forum, where collective and national memories and postcolonial history were intensely discussed, and the debate made room for some degree of reconciliation between viewers of Dutch......-Moluccan and of Dutch majority background...

  11. Glomus Tumor of the Larynx: A Rare Synchronous Paraganglioma in a Patient with Bilateral Carotid Body Tumor Detected on 68Ga-DOTANOC PET/CT.

    Science.gov (United States)

    Tripathy, Sarthak; Mukherjee, Anirban; Singh, Chirom A; Jat, Bhinyaram; Bal, Chandrasekhar; Shamim, Shamim Ahmed

    2017-01-01

    Paragangliomas are neoplasms arising from extra-adrenal chromaffin tissue. They frequently cause symptoms by overproduction of catecholamines with known predilection to multicentricity. We describe the case of a patient with bilateral carotid body tumor who underwent a baseline 68 Gallium labeled [1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acid]-1-NaI3-Octreotide.

  12. Solidarity in Dutch families - Family ties under strain?

    NARCIS (Netherlands)

    Komter, A.E.; Vollebergh, W.A.M.

    2002-01-01

    The nature and extent of interpersonal exchange of care and help may indicate solidarity and cohesion in modern society. This article focuses on intergenerational solidarity. On the basis of empirical data from an earlier research project on giving in the Netherlands, the authors examine the nature

  13. Digital subtraction angiography in evaluation of vascular supply of head and neck paragangliomas

    International Nuclear Information System (INIS)

    Juszkat, R.; Szyfter, P.; Zarzecka, A.

    2008-01-01

    Paragangliomas (PGs) of the head and neck are relatively rare and represent 0.6% of all head and neck tumors and 0.03% of all tumors. There are four groups of head and neck PGs: carotid body tumors, vagal PGs, jugular PGs, and tympanic PGs. The resection of head and neck PGs carries an inherent risk of injury to cranial nerves and vascular structures which may lead to excessive bleeding. To plan the surgical strategy for PGs, detailed information about the vascular supply of the tumor is required. Between January 1998 and April 2007, 41 tumors of the head and neck were identified in 37 patients (20 females, 17 males, mean age: 38.4 years). Single tumors were observed in 33 patients, two head and neck PGs were identified in 3 patients, and 1 patient presented 3 PGs, one of which was located laterally to the aortic arch. There were 21 PGs located at the carotid bifurcation, 10 in the jugular foramen, 6 in the tympanic cavity, and 4 along the course of the vagus nerve. In all the cases of PGs located in the head and neck, the vascular supply came from branches of the external carotid artery. Vascular supply from the internal carotid and the vertebral arteries was not seen in any of the patients. The most common vascular supply in the cases of carotid body tumors and jugular PGs was the pharyngeal ascending artery. In the cases of vagal PGs it was the pharyngeal ascending artery and the posterior auricular artery and in the case of tympanic PGs the posterior auricular artery. DSA is an important tool in the diagnosis of head and neck PGs. The evaluation of its vascularization is essential in planning further treatment, both endovascular and surgical. (author)

  14. CT AND MRI FEATURES OF CAROTID BODY PARAGANGLIOMAS IN 16 DOGS.

    Science.gov (United States)

    Mai, Wilfried; Seiler, Gabriela S; Lindl-Bylicki, Britany J; Zwingenberger, Allison L

    2015-01-01

    Carotid body tumors (paragangliomas) arise from chemoreceptors located at the carotid bifurcation. In imaging studies, this neoplasm may be confused with other neck neoplasms such as thyroid carcinoma. The purpose of this retrospective, cross-sectional study was to describe computed tomographic (CT) and magnetic resonance imaging (MRI) characteristics of confirmed carotid body tumors in a multi-institutional sample of dogs. A total of 16 dogs met inclusion criteria (14 examined using CT and two with MRI). The most common reason for imaging was a palpable cervical mass or respiratory signs (i.e., dyspnea or increased respiratory noises). The most commonly affected breed was Boston terrier (n = 5). Dogs were predominantly male castrated (n = 10) and the median age was 9 years [range 3-14.5]. Most tumors appeared as a large mass centered at the carotid bifurcation, with poor margination in six dogs and discrete margins in ten dogs. Masses were iso- to hypoattenuating to adjacent muscles in CT images and hyperintense to muscles in T1- and T2-weighted MRI. For both CT and MRI, masses typically showed strong and heterogeneous contrast enhancement. There was invasion into the adjacent structures in 9/16 dogs. In six of these nine dogs, the basilar portion of the skull was affected. The external carotid artery was entrapped in seven dogs. There was invasion into the internal jugular vein in three dogs, and into the external jugular, maxillary, and linguo-facial veins in one dog. Imaging characteristics helped explain some clinical presentations such as breathing difficulties, Horner's syndrome, head tilt, or facial nerve paralysis. © 2015 American College of Veterinary Radiology.

  15. Lower cranial nerves function after surgical treatment of Fisch Class C and D tympanojugular paragangliomas.

    Science.gov (United States)

    Bacciu, Andrea; Medina, Marimar; Ait Mimoune, Hassen; D'Orazio, Flavia; Pasanisi, Enrico; Peretti, Giorgio; Sanna, Mario

    2015-02-01

    The aim of this study was to report the postoperative lower cranial nerves (LCNs) function in patients undergoing surgery for tympanojugular paraganglioma (TJP) and to evaluate risk factors for postoperative LCN dysfunction. A retrospective case review of 122 patients having Fisch class C or D TJP, surgically treated from 1988 to 2012, was performed. The follow-up of the series ranged from 12 to 156 months (mean, 39.4 ± 32.6 months). The infratemporal type A approach was the most common surgical procedure. Gross total tumor removal was achieved in 86% of cases. Seventy-two percent of the 54 patients with preoperative LCN deficit had intracranial tumor extension. Intraoperatively, LCNs had to be sacrificed in 63 cases (51.6%) due to tumor infiltration. Sixty-six patients (54.09%) developed a new deficit of one or more of the LCNs. Of those patients who developed new LCN deficits, 23 of them had intradural extension. Postoperative follow-up of at least 1 year showed that the LCN most commonly affected was the CN IX (50%). Logistic regression analysis showed that intracranial transdural tumor extension was correlated with the higher risk of LCN sacrifice (p < 0.05). Despite the advances in skull base surgery, new postoperative LCN deficits still represent a challenge. The morbidity associated with resection of the LCNs is dependent on the tumor's size and intradural tumor extension. Though no recovery of LCN deficits may be expected, on long-term follow-up, patients usually compensate well for their LCNs loss.

  16. Immunohistochemical profile of neurotrophins and MIB-1 in jugulotympanic paragangliomas: prognostic value and review of the literature.

    Science.gov (United States)

    Artico, M; De Vincentiis, M; Ionta, B; Bianchi, E; Bosco, S; Onteleone, M; Fumagalli, L; Magliulo, G

    2012-01-01

    Jugulo-tympanic paragangliomas are the most common primary neoplasm of the middle ear, but little is still known about the histological features differentiating the benign and malignant forms. We investigated, with an immunohistochemical procedure, the expression of neurotrophins with their receptors, in fifteen samples of paragangliomas, and MIB-1 in order to consider them as prognostic factors of malignancy. We observed a general positivity for NGF - TrKA - NT4 - TrKC in the cytoplasm, and a strong expression for BDNF in the extracellular space. MIB-1 was moderate in the nucleus of neoplastic cells, weak in the cytoplasm and totally absent in the extracellular space. The comparison between the clinical recurrences and the rate of cytoplasmatic neurotrophins showed strong immunoreactivity in recurrent patients. It should be emphasized that 2 of the 3 recurrences had a wider distribution of the neutrophins, leading to hypothesize the involvement of these substances in the cell proliferation of glomus tumors. Malignant forms of these rare glomus tumors cannot be clearly identified using MIB-1 as a prognostic marker, although we can affirm that neurotrophins and their receptors can be considered as a panel of potential diagnostic markers to monitor the development of such malignancies. Although the small number of patients does not allow definitive conclusions to be made, our findings showed a possible trend towards significance which requires a more powerful study to evaluate this further.

  17. Germ-line PHD1 and PHD2 mutations detected in patients with pheochromocytoma/paraganglioma-polycythemia.

    Science.gov (United States)

    Yang, Chunzhang; Zhuang, Zhengping; Fliedner, Stephanie M J; Shankavaram, Uma; Sun, Michael G; Bullova, Petra; Zhu, Roland; Elkahloun, Abdel G; Kourlas, Peter J; Merino, Maria; Kebebew, Electron; Pacak, Karel

    2015-01-01

    We have investigated genetic/pathogenetic factors associated with a new clinical entity in patients presenting with pheochromocytoma/paraganglioma (PHEO/PGL) and polycythemia. Two patients without hypoxia-inducible factor 2α (HIF2A) mutations, who presented with similar clinical manifestations, were analyzed for other gene mutations, including prolyl hydroxylase (PHD) mutations. We have found for the first time a germ-line mutation in PHD1 in one patient and a novel germ-line PHD2 mutation in a second patient. Both mutants exhibited reduced protein stability with substantial quantitative protein loss and thus compromised catalytic activities. Due to the unique association of patients' polycythemia with borderline or mildly elevated erythropoietin (EPO) levels, we also performed an in vitro sensitivity assay of erythroid progenitors to EPO and for EPO receptor (EPOR) expression. The results show inappropriate hypersensitivity of erythroid progenitors to EPO in these patients, indicating increased EPOR expression/activity. In addition, the present study indicates that HIF dysregulation due to PHD mutations plays an important role in the pathogenesis of these tumors and associated polycythemia. The PHD1 mutation appears to be a new member contributing to the genetic landscape of this novel clinical entity. Our results support the existence of a specific PHD1- and PHD2-associated PHEO/PGL-polycythemia disorder. • A novel germ-l i n e PHD1 mutation causing heochromocytoma/paraganglioma and polycythemia. • Increased EPOR activity and inappropriate hypersensitivity of erythroid progenitors to EPO.

  18. Radiological and pathological findings of a metastatic composite paraganglioma with neuroblastoma in a man: a case report

    Directory of Open Access Journals (Sweden)

    Koch Sonja

    2010-11-01

    Full Text Available Abstract Introduction Composite tumors of the adrenal medulla or paraganglia are extremely rare and present a diagnostic dilemma. These tumors consist of a neuroendocrine component mixed with a neural component. We describe the imaging characteristics together with the corresponding pathological findings of a composite tumor. Apart from any component-specific imaging findings, the hallmark of this entity is the presence of histologically distinguishable components. Case presentation A 61-year-old Caucasian man was referred to our hospital due to a suspect lesion found on chest computed tomography carried out for unclear thoracic pain. An abdominal computed tomography scan and ultrasound examination detected a retroperitoneal tumor comprising two different tumor components. Twenty-four-hour urine revealed high levels of normetanephrine, characteristic of a neuroendocrine tumor. An octreoscan prior to surgical procedures revealed multiple osseous and intra-hepatic metastases. The final histopathological workup revealed a composite paraganglioma with neuroblastoma. Our patient died ten months after the initial diagnosis from tumor-associated complications. Conclusions Composite paragangliomas with neuroblastoma are rare tumors of the retroperitoneum. Such tumors should be considered in the differential diagnosis of retroperitoneal masses.

  19. 77 FR 19967 - Safety Zone, Port of Dutch Harbor; Dutch Harbor, AK

    Science.gov (United States)

    2012-04-03

    ...-AA00 Safety Zone, Port of Dutch Harbor; Dutch Harbor, AK AGENCY: Coast Guard, DHS. ACTION: Notice of proposed rulemaking. SUMMARY: The Coast Guard proposes temporary safety zones in the Port of Dutch Harbor... Dutch Harbor, Alaska, and the adjacent territorial sea due to additional vessel traffic associated with...

  20. Teaching the Dutch how to pronounce English

    NARCIS (Netherlands)

    Frans Hermans; Peter Sloep

    2015-01-01

    The Dutch overestimate their English speaking skills. Their pronunciation is not always convincing and certain pronunciation mistakes are easily recognised as being typical for Dutch speakers of English. Although intelligibility cannot exist without adequate pronunciation, teaching English

  1. Teaching the Dutch how to pronounce English

    NARCIS (Netherlands)

    Hermans, Frans; Sloep, Peter

    2018-01-01

    The Dutch overestimate their English speaking skills. Their pronunciation is not always convincing, and certain pronunciation mistakes are easily recognised as being typical for Dutch speakers of English. Although intelligibility cannot exist without adequate pronunciation, teaching English

  2. Phase II trial of pazopanib in advanced/progressive malignant pheochromocytoma and paraganglioma.

    Science.gov (United States)

    Jasim, Sina; Suman, Vera J; Jimenez, Camilo; Harris, Pamela; Sideras, Kostandinos; Burton, Jill K; Worden, Francis Paul; Auchus, Richard J; Bible, Keith C

    2017-08-01

    Pheochromocytomas and paragangliomas (Pheo/PGL) are rare, vascular, sometimes malignant endocrine tumors. Case reports indicate the activity of vascular endothelium growth factor receptor-targeted kinase inhibitors in these cancers. To assess the antitumor activity and tolerability of pazopanib in progressive malignant Pheo/PGL. This multicenter Phase II trial (MC107C) enrolled individuals  ≥18 years old with disease progression ≤ 6 months prior to registration, Eastern Cooperative Oncology Group PS 0-2, and measurable disease (response evaluation criteria in solid tumors 1.0). Pazopanib was administered in 28-day cycles, with the regimen ultimately being as follows: cycle 1: 400 mg daily on days 1-14, cycle 2: 800 mg daily on days 1-14, and then cycle 2 + : 800 mg daily on all days. The study was halted due to poor accrual. Seven patients were enrolled (05/2011-11/2014). One patient withdrew consent prior to treatment, leaving six evaluable patients. Treatment was discontinued, due to the following reasons: disease progression (4); withdrawal (1); and grade 4 (Takotsubo) cardiomyopathy (1). The median number of cycles administered was 4 (range: 2-29, total: 49). Four patients had >1 dose reduction due to the following reasons: fatigue (1), abnormal liver tests (2), hypertension and (Takotsubo) cardiomyopathy (1), and headaches (1). Common severe (Common Terminology Criteria for Adverse Events v3.0 grades 3-5) toxicities were as follows: hypertension (3/6), (Takotsubo) cardiomyopathy (2/6), diarrhea (1/6), fatigue (1/6), headache (1/6), and hematuria (1/6). One confirmed partial response was observed in PGL (17%, duration 2.4 years); median progression-free survival and overall survival were 6.5 and 14.8 months, respectively. Pazopanib has activity in Pheo/PGL requiring more study; optimal alpha- and beta-blockade are imperative pre-therapy in patients with secretory tumors, as risk of hypertension and cardiomyopathy are potentially life

  3. Functional Imaging Signature of Patients Presenting with Polycythemia/Paraganglioma Syndromes.

    Science.gov (United States)

    Janssen, Ingo; Chen, Clara C; Zhuang, Zhenping; Millo, Corina M; Wolf, Katherine I; Ling, Alexander; Lin, Frank I; Adams, Karen T; Herscovitch, Peter; Feelders, Richard A; Fojo, Antonio T; Taieb, David; Kebebew, Electron; Pacak, Karel

    2017-08-01

    Pheochromocytoma/paraganglioma (PPGL) syndromes associated with polycythemia have previously been described in association with mutations in the von Hippel-Lindau gene. Recently, mutations in the prolyl hydroxylase gene ( PHD ) 1 and 2 and in the hypoxia-inducible factor 2 α ( HIF2A ) were also found to be associated with multiple and recurrent PPGL. Such patients also presented with PPGL and polycythemia, and later on, some presented with duodenal somatostatinoma. In additional patients presenting with PPGL and polycythemia, no further mutations have been discovered. Because the functional imaging signature of patients with PPGL-polycythemia syndromes is still unknown, and because these tumors (in most patients) are multiple, recurrent, and metastatic, the goal of our study was to assess the optimal imaging approach using 4 different PET radiopharmaceuticals and CT/MRI in these patients. Methods: Fourteen patients (10 women, 4 men) with confirmed PPGL and polycythemia prospectively underwent 68 Ga-DOTATATE (13 patients), 18 F-FDG (13 patients), 18 F-fluorodihydroxyphenylalanine ( 18 F-FDOPA) (14 patients), 18 F-fluorodopamine ( 18 F-FDA) (11 patients), and CT/MRI (14 patients). Detection rates of PPGL lesions were compared between all imaging studies and stratified between the underlying mutations. Results: 18 F-FDOPA and 18 F-FDA PET/CT showed similar combined lesion-based detection rates of 98.7% (95% confidence interval [CI], 92.7%-99.8%) and 98.3% (95% CI, 90.9%-99.7%), respectively. The detection rates for 68 Ga-DOTATATE (35.3%; 95% CI, 25.0%-47.2%), 18 F-FDG (42.3; 95% CI, 29.9%-55.8%), and CT/MRI (60.3%; 95% CI, 48.8%-70.7%) were significantly lower ( P < 0.01), irrespective of the mutation status. Conclusion: 18 F-FDOPA and 18 F-FDA are superior to 18 F-FDG, 68 Ga-DOTATATE, and CT/MRI and should be the radiopharmaceuticals of choice in this rare group of patients. © 2017 by the Society of Nuclear Medicine and Molecular Imaging.

  4. The role of women on Dutch farms

    NARCIS (Netherlands)

    Meulen, van der H.A.B.; Terluin, I.J.; Matser, I.A.

    2015-01-01

    In this paper an analysis is made of the contribution of women to labour input and management on Dutch farms. We used a written survey among the participants of the Dutch Farm Accountancy Data Network (FADN), in-depth interviews and a group discussion with farm women. Over half of the women on Dutch

  5. Third Dutch Process Security Control Event

    NARCIS (Netherlands)

    Luiijf, H.A.M.

    2009-01-01

    On June 4th, 2009, the third Dutch Process Control Security Event took place in Amsterdam. The event, organised by the Dutch National Infrastructure against Cybercrime (NICC), attracted both Dutch process control experts and members of the European SCADA and Control Systems Information Exchange

  6. Syntactic Atlas of the Dutch Dialects

    NARCIS (Netherlands)

    Barbiers, Sjef; Bennis, Hans; Vogelaer, De Gunther; Devos, Magda; Ham, van der Margreet

    2005-01-01

    Available in a Dutch and English Edition, the Syntactic Atlas of the Dutch Dialects (SAND) provides a detailed overview of the surprisingly rich syntactic variation found in 267 dialects of Dutch at the beginning of the 21th century. 200 full color maps show the geographic distribution of more than

  7. Interpreting The Chinese Wall knowing Dutch and ‘Dutchness'

    NARCIS (Netherlands)

    Forceville, C.

    2015-01-01

    Many film scholars equate "film" with "moving images." But, along with sound and music, language is often no less important for a film’s interpretation. This paper discusses how comprehension of the original Dutch affords subtle aspects of meaning unavailable to viewers who have to rely exclusively

  8. Repeated Radionuclide therapy in metastatic paraganglioma leading to the highest reported cumulative activity of 131I-MIBG

    International Nuclear Information System (INIS)

    Ezziddin, Samer; Sabet, Amir; Ko, Yon-Dschun; Xun, Sunny; Matthies, Alexander; Biersack, Hans-Jürgen

    2012-01-01

    131 I-MIBG therapy for neuroendocrine tumours may be dose limited. The common range of applied cumulative activities is 10-40 GBq. We report the uneventful cumulative administration of 111 GBq (= 3 Ci) 131 I-MIBG in a patient with metastatic paraganglioma. Ten courses of 131 I-MIBG therapy were given within six years, accomplishing symptomatic, hormonal and tumour responses with no serious adverse effects. Chemotherapy with cisplatin/vinblastine/dacarbazine was the final treatment modality with temporary control of disease, but eventually the patient died of progression. The observed cumulative activity of 131 I-MIBG represents the highest value reported to our knowledge, and even though 12.6 GBq of 90 Y-DOTATOC were added intermediately, no associated relevant bone marrow, hepatic or other toxicity were observed. In an individual attempt to palliate metastatic disease high cumulative activity alone should not preclude the patient from repeat treatment

  9. Sustainable innovations in Dutch SMEs

    International Nuclear Information System (INIS)

    Bertens, C.; Snoei, J.

    2011-11-01

    The 'Dutch Monitor Determinanten Bedrijfsprestaties in het MKB' (Determinants Company Performance Monitoring SMEs) has consulted almost 3.500 persons in SMEs on a number of questions, including on innovations. 40% of these persons perceive market opportunities for sustainable products, whereas only 25% of these businesses actually tries to capitalize on these opportunities. [nl

  10. The dutch surgical colorectal audit

    NARCIS (Netherlands)

    Leersum, N.J. van; Snijders, H.S.; Henneman, D.; Kolfschoten, N.E.; Gooiker, G.A.; Berge, M.G. Ten; Eddes, E.H.; Wouters, M.W.; Tollenaar, R.A.E.M.; Bemelman, W.A.; Dam, R.M. van; Elferink, M.A.; Karsten, T.M.; Krieken, J.H. van; Lemmens, V.E.; Rutten, H.J.; Manusama, E.R.; Velde, C.J. van de; Meijerink, W.J.H.J.; Wiggers, T.; Harst, E. van der; Dekker, J.W.T.; Boerma, D.

    2013-01-01

    INTRODUCTION: In 2009, the nationwide Dutch Surgical Colorectal Audit (DSCA) was initiated by the Association of Surgeons of the Netherlands (ASN) to monitor, evaluate and improve colorectal cancer care. The DSCA is currently widely used as a blueprint for the initiation of other audits, coordinated

  11. The Aggregate Dutch Historical Censuses

    NARCIS (Netherlands)

    Ashkpour, Ashkan; Meroño-Peñuela, Albert; Mandemakers, Kees

    2015-01-01

    Historical censuses have an enormous potential for research. In order to fully use this potential, harmonization of these censuses is essential. During the last decades, enormous efforts have been undertaken in digitizing the published aggregated outcomes of the Dutch historical censuses

  12. The Aggregate Dutch Historical Censuses

    NARCIS (Netherlands)

    A. Ashkpour (Ashkan); A. Meronõ-Peñuela (Albert); C.A. Mandemakers (Kees)

    2015-01-01

    textabstractHistorical censuses have an enormous potential for research. In order to fully use this potential, harmonization of these censuses is essential. During the last decades, enormous efforts have been undertaken in digitizing the published aggregated outcomes of the Dutch historical censuses

  13. Dutch museum marks Einstein anniversary

    Science.gov (United States)

    van Calmthout, Matijn

    2016-01-01

    A new painting of Albert Einstein's field equation from his 1915 general theory of relativity was unveiled in a ceremony in November 2015 by the Dutch physicist Robbert Dijkgraaf, who is director of the Princeton Institute for Advanced Study in the US.

  14. Drug Policy: the "Dutch Model"

    NARCIS (Netherlands)

    van Ooijen-Houben, M.M.J.; Kleemans, E.R.

    2015-01-01

    Dutch drug policy, once considered pragmatic and lenient and rooted in a generally tolerant attitude toward drug use, has slowly but surely shifted from a primarily public health focus to an increasing focus on law enforcement. The "coffee shop" policy and the policy toward MDMA/ecstasy are

  15. Political radicalism among Dutch farmers

    NARCIS (Netherlands)

    Nooij, A.T.J.

    1969-01-01

    Although income parity has been achieved in Dutch agriculture, dissatisfaction with income is prevalent among farmers for reasons which are quite understandable. Low income is the most important factor that brings about the decrease in the number of farmers. A real opposition against government

  16. Dutch Architecture with Landscape Methods

    NARCIS (Netherlands)

    Abrami, F.; Bijster, J.; Bitarafan, N.; Cao, Z.; Cui, Y.; Liu, Y.; Ruan, L.; Maas, M.; Mayr, R.; Rozenmuller, M.; Toriano, L.; Yoshitake, M.; Jauslin, D.

    2009-01-01

    14 Project Documentations and Analysis of Dutch Architecture with Landscape Methods. MVRDV Villa VPRO, Powerhouse Company Villa 1, Herman Herzberger Coda Museum, NL Architects Basket Ba, SeARCH Posbank Pavillion, Wiel Arets Hedge House, OMA Kunsthal and Educatorium, Maaskant Johnson Wax, Diller &

  17. The Dutch Banking Chipcard Game

    NARCIS (Netherlands)

    H.J. de Vries (Henk); G.W.J. Hendrikse (George)

    2001-01-01

    textabstractThe banks in the Dutch chipcard market initially agreed on one chipcard system. One system is attractive for companies as well as consumers. Companies, banks and retailers, prevent costs of duplication, while consumers enjoy the benefits of a widespread acceptance of one card and do not

  18. The Dutch Interbank Computer Network

    NARCIS (Netherlands)

    Michiels, E.F.

    1988-01-01

    At the end of 1980, a strategic decision was made by the Dutch banks and savings banks to commence the development of a Data Communications Infrastructure (DCI), to be used for a number of forthcoming interbank applications. It was agreed that this new data communications infrastructure should be

  19. Indo-Dutch Business & Culture

    NARCIS (Netherlands)

    Pieter van Nispen tot Pannerden; Dr. P. Ester

    2013-01-01

    As outlook of commercial cooperation and business alliances between India and the Netherlands remains promising and continues to grow, Indian and Dutch entrepreneurs who consider doing business in each other’s country, will need to understand one another. Peter Ester & Pieter van Nispen report on

  20. Dutch-Chinese virtual cooperation

    NARCIS (Netherlands)

    Wei, Y.; de Boer, S.J.; Stegwee, R.A.; Chen, Z.

    2003-01-01

    Technology advances in ICT have permitted an increased flow of information across borders, and increasingly companies are moving to so-called virtual alliances. The business relationships between Dutch and Chinese companies are becoming closer by ways of outsourcing, new market development and so

  1. Preoperative Embolization Reduces the Risk of Cathecolamines Release at the Time of Surgical Excision of Large Pelvic Extra-Adrenal Sympathetic Paraganglioma

    Directory of Open Access Journals (Sweden)

    Nicola Di Daniele

    2012-01-01

    Full Text Available A 30-year-old woman with severe hypertension was admitted to the hospital with a history of headache, palpitations, and diaphoresis following sexual intercourse. Twenty-four hour urinary excretion of free catecholamines and metabolites was markedly increased as was serum chromogranin A. Computed tomography scan revealed a large mass in the left adnex site and magnetic resonance imaging confirmed the computer tomography finding, suggesting the presence of extra-adrenal sympathetic paraganglioma. I-metaiodobenzyl guanidine scintigram revealed an increased uptake in the same area. Transcatheter arterial embolization of the mass resulted in marked decreases in blood pressure and urinary excretion of free catecholamines and metabolites. Surgical excision of the mass was then accomplished without complication. Preoperative embolization is a useful and safe procedure which may reduce the risk of catecholamines release at the time of surgical excision in large pelvic extra-adrenal sympathetic paraganglioma.

  2. PET/CT comparing 68Ga-DOTATATE and other radiopharmaceuticals and in comparison with CT/MRI for the localization of sporadic metastatic pheochromocytoma and paraganglioma

    International Nuclear Information System (INIS)

    Janssen, Ingo; Chen, Clara C.; Millo, Corina M.; Herscovitch, Peter; Ling, Alexander; Taieb, David; Lin, Frank I.; Adams, Karen T.; Wolf, Katherine I.; Pacak, Karel; Fojo, Antonio T.; Buchmann, Inga; Kebebew, Electron

    2016-01-01

    Pheochromocytomas/paragangliomas (PPGLs) and their metastases are tumors that predominantly express somatostatin receptor 2 (SSR2). 68 Ga-DOTA(0)-Tyr(3)-octreotate ( 68 Ga-DOTATATE) is a PET radiopharmaceutical with both high and selective affinity for SSRs. The purpose of this study was to evaluate the utility of 68 Ga-DOTATATE in comparison with other specific and nonspecific radiopharmaceuticals recommended in the current guidelines for the localization of metastatic sporadic PPGL by PET/CT. This prospective study included 22 patients (15 men, 7 women; aged 50.0 ± 13.9 years) with confirmed metastatic PPGL, a negative family history for PPGL, and negative genetic testing, who underwent 68 Ga-DOTATATE, 18 F-fluoro-2-deoxy-D-glucose ( 18 F-FDG) PET/CT, and CT/MRI. Only 12 patients underwent an additional 18 F-fluorodihydroxyphenylalanine ( 18 F-FDOPA) PET/CT scan and only 11 patients underwent an additional 18 F-fluorodopamine ( 18 F-FDA) PET/CT scan. The rates of detection of metastatic lesions were compared among all the imaging studies. A composite of all functional and anatomical imaging studies served as the imaging comparator. 68 Ga-DOTATATE PET/CT showed a lesion-based detection rate of 97.6 % (95 % confidence interval, CI, 95.8 - 98.7 %). 18 F-FDG PET/CT, 18 F-FDOPA PET/CT, 18 F-FDA PET/CT, and CT/MRI showed detection rates of 49.2 % (CI 44.5 - 53.6 %; p < 0.01), 74.8 % (CI 69.0 - 79.9 %; p < 0.01), 77.7 % (CI 71.5 - 82.8 %; p < 0.01), and 81.6 % (CI 77.8 - 84.8 %; p < 0.01), respectively. The results of this study demonstrate the superiority of 68 Ga-DOTATATE PET/CT in the localization of sporadic metastatic PPGLs compared to all other functional and anatomical imaging modalities, and suggest modification of future guidelines towards this new imaging modality. (orig.)

  3. Cytocidal activities of topoisomerase 1 inhibitors and 5-azacytidine against pheochromocytoma/paraganglioma cells in primary human tumor cultures and mouse cell lines.

    Directory of Open Access Journals (Sweden)

    James F Powers

    Full Text Available There is currently no effective treatment for metastatic pheochromocytomas and paragangliomas. A deficiency in current chemotherapy regimens is that the metastases usually grow very slowly. Drugs that target dividing tumor cells have therefore had limited success. To improve treatment, new strategies and valid experimental models are required for pre-clinical testing. However, development of models has itself been hampered by the absence of human pheochromocytoma/paraganglioma cell lines for cultures or xenografts. Topoisomerase 1 (TOP1 inhibitors are drugs that interfere with mechanisms that maintain DNA integrity during transcription in both quiescent and dividing cells. We used primary cultures of representative human tumors to establish the cytotoxicity of camptothecin, a prototypical TOP1 inhibitor, against non-dividing pheochromocytoma/paraganglioma cells, and then employed a mouse pheochromocytoma model (MPC to show that efficacy of low concentrations of camptothecin and other TOP1 inhibitors is increased by intermittent coadministration of sub-toxic concentrations of 5-azacytidine, a DNA methylation inhibitor that modulates transcription. We then tested the same drugs against a clonal MPC derivative that expresses CMV reporter-driven luciferase and GFP, intended for in vivo drug testing. Unexpectedly, luciferase expression, bioluminescence and GFP expression were paradoxically increased by both camptothecin and SN38, the active metabolite of irinotecan, thereby masking cell death. Expression of chromogranin A, a marker for neuroendocrine secretory granules, was not increased, indicating that the drug effects on levels of luciferase and GFP are specific to the GFP-luciferase construct rather than generalized cellular responses. Our findings provide proof of principle for use of TOP1 inhibitors against pheochromocytoma/paraganglioma and suggest novel strategies for enhancing efficacy and reducing toxicity by optimizing the combination and

  4. MANAGEMENT OF ENDOCRINE DISEASE: Recurrence or new tumors after complete resection of pheochromocytomas and paragangliomas: a systematic review and meta-analysis.

    Science.gov (United States)

    Amar, Laurence; Lussey-Lepoutre, Charlotte; Lenders, Jacques W M; Djadi-Prat, Juliette; Plouin, Pierre-Francois; Steichen, Olivier

    2016-10-01

    To systematically review the incidence and factors associated with recurrences or new tumors after apparent complete resection of pheochromocytoma or thoraco-abdomino-pelvic paraganglioma. A systematic review and meta-analysis of published literature was performed. Pubmed and Embase from 1980 to 2012 were searched for studies published in English on patients with non-metastatic pheochromocytoma or thoraco-abdomino-pelvic paraganglioma, complete tumor resection, postoperative follow-up exceeding 1 month, and recurrence or new tumor documented by pathology, hormonal dosages, or imaging tests. Incidence rates of new events after curative surgery were calculated for each study that had sufficient information and pooled using random-effect meta-analysis. In total, 38 studies were selected from 3518 references, of which 36 reported retrospective cohorts from the USA, Europe, and Asia. Patient follow-up was neither standardized nor exhaustive in the included studies. A clear description of patient retrieval methods was available for nine studies and the follow-up protocol and patient flow for four studies. Only two studies used multivariable methods to assess potential predictors of postoperative events.The overall rate of recurrent disease from 34 studies was 0.98 events/100 person-years (95% confidence interval 0.71, 1.25). Syndromic diseases and paragangliomas were consistently associated with a higher risk of a new event in individual studies and in meta-regression analysis. The risk of recurrent disease after complete resection of pheochromocytoma may be lower than that previously estimated, corresponding to five events for 100 patients followed up for 5 years after complete resection. Risk stratification is required to tailor the follow-up protocol after complete resection of a pheochromocytoma or paraganglioma. Large multicenter studies are needed to this end. © 2016 European Society of Endocrinology.

  5. Comparison of the 68Ga-DOTATATA PET/CT, FDG PET/CT, and MIBG SPECT/CT in the Evaluation of Suspected Primary Pheochromocytomas and Paragangliomas.

    Science.gov (United States)

    Jing, Hongli; Li, Fang; Wang, Ling; Wang, Zhenghua; Li, Wei; Huo, Li; Zhang, Jingjing

    2017-07-01

    Anatomical imaging modalities including CT and MRI are the mainstay of evaluation of primary pheochromocytoma or paraganglioma. However, nuclear medicine imaging is frequently necessary to determine the nature of the lesions. The purpose of this investigation is to assess which commonly used nuclear medicine modality might have a better diagnostic value in this clinical setting. Eight patients who had been suspected of having either primary pheochromocytoma or primary paraganglioma and 1 patient with known pheochromocytoma were included in the analysis. Among the 8 patients without known diagnosis, 7 had been suggested by anatomical imaging modalities, whereas one of them presented with initial negative anatomical imaging interpretation. All of 9 patients underwent Ga-DOTATATA PET/CT, FDG PET/CT, and MIBG SPECT/CT for further evaluation. The imaging findings were compared with postsurgical pathology and follow-up. Both Ga-DOTATATA PET/CT and MIBG SPECT/CT accurately identified 9 primary tumors, whereas FDG PET/CT showed increased activity in 8 of 9 primary tumors. Both Ga-DOTATATA and FDG PET/CT are able to detect associated extra-adrenal lesions not shown on MIBG study in patients with multiple endocrine neoplasia syndrome. Ga-DOTATATA PET/CT could be the nuclear medicine imaging choice to evaluate suspected primary pheochromocytoma or paraganglioma, especially in the situation of multiple endocrine neoplasia syndrome.

  6. The challenges of treating paraganglioma patients with 177Lu-DOTATATE PRRT: Catecholamine crises, tumor lysis syndrome and the need for modification of treatment protocols

    International Nuclear Information System (INIS)

    Makis, William; Mccann, Karey; Mcewan, Alexander J. B.

    2015-01-01

    A high percentage of paragangliomas express somatostatin receptors that can be utilized for targeted radioisotope therapy. The aim of this study was to describe and discuss the challenges of treating these tumors with 177 Lu-[DOTA0,Tyr3]octreotate (DOTATATE) radioisotope therapy using established protocols. Three paraganglioma patients were treated with 4–5 cycles of 177 Lu-DOTATATE and were evaluated for side effects and response to therapy. Two of the three patients developed severe adverse reactions following their first 177 Lu-DOTATATE treatment. One patient developed a catecholamine crisis and tumor lysis syndrome within hours of treatment, requiring intensive care unit (ICU) support, and another developed a catecholamine crisis 3 days after treatment, requiring hospitalization. The treatment protocols at our institution were subsequently modified by increasing the radioisotope infusion time from 15 to 30 min, as recommended in the literature, to 2–4 h and by reducing the administered dose of 177 Lu-DOTATATE. Subsequent 177 Lu-DOTATATE treatments utilizing the modified protocols were well tolerated, and response to therapy was achieved in all three patients, resulting in significantly improved quality of life. 177 Lu-DOTATATE is an exciting new therapeutic option in the management of paragangliomas; however, current treatment protocols described in the literature may need to be modified by lengthening the infusion time and/or lowering the initial treatment dose to prevent or reduce the severity of adverse reactions

  7. The challenges of treating paraganglioma patients with {sup 177}Lu-DOTATATE PRRT: Catecholamine crises, tumor lysis syndrome and the need for modification of treatment protocols

    Energy Technology Data Exchange (ETDEWEB)

    Makis, William; Mccann, Karey; Mcewan, Alexander J. B. [Dept. of Diagnostic Imaging, Cross Cancer Institute, Alberta (China)

    2015-09-15

    A high percentage of paragangliomas express somatostatin receptors that can be utilized for targeted radioisotope therapy. The aim of this study was to describe and discuss the challenges of treating these tumors with {sup 177}Lu-[DOTA0,Tyr3]octreotate (DOTATATE) radioisotope therapy using established protocols. Three paraganglioma patients were treated with 4–5 cycles of {sup 177}Lu-DOTATATE and were evaluated for side effects and response to therapy. Two of the three patients developed severe adverse reactions following their first {sup 177}Lu-DOTATATE treatment. One patient developed a catecholamine crisis and tumor lysis syndrome within hours of treatment, requiring intensive care unit (ICU) support, and another developed a catecholamine crisis 3 days after treatment, requiring hospitalization. The treatment protocols at our institution were subsequently modified by increasing the radioisotope infusion time from 15 to 30 min, as recommended in the literature, to 2–4 h and by reducing the administered dose of {sup 177}Lu-DOTATATE. Subsequent {sup 177}Lu-DOTATATE treatments utilizing the modified protocols were well tolerated, and response to therapy was achieved in all three patients, resulting in significantly improved quality of life. {sup 177}Lu-DOTATATE is an exciting new therapeutic option in the management of paragangliomas; however, current treatment protocols described in the literature may need to be modified by lengthening the infusion time and/or lowering the initial treatment dose to prevent or reduce the severity of adverse reactions.

  8. Functional extra-adrenal paraganglioma of the retroperitoneum giving thoracolumbar spine metastases after a five-year disease-free follow-up: a rare malignant condition with challenging management.

    Science.gov (United States)

    Kapetanakis, Stylianos; Chourmouzi, Danai; Gkasdaris, Grigorios; Katsaridis, Vasileios; Eleftheriadis, Eleftherios; Givissis, Panagiotis

    2017-01-01

    Paragangliomas are benign neoplasms that arise from the autonomic nervous system and the associated paraganglia. Although benign, they have been shown to possess metastatic potential. Extra-adrenal retroperitoneal paraganglioma with vertebral metastasis is considered very uncommon. Here, we present a case of a functional extra-adrenal paraganglioma of the retroperitoneum giving metastasis to T4 vertebra after five years of follow-up in a 48-year-old man who had been initially treated with complete resection of the primary tumor. The condition of the patient improved significantly after radiosurgery and somatostatin analogs treatment, until lumbar spine lesions appeared six months later. Our case demonstrates that retroperitoneal paraganglioma is a rare condition which should be considered in the differential diagnosis of a retroperitoneal mass combined with vertebral lesions. Additionally, increased physician awareness and long-term follow-up is mandatory for all patients with history of retroperitoneal paraganglioma since metastases may occur after long latent intervals from the initial diagnosis.

  9. Mutation analysis of the RET proto-oncogene in Dutch families with MEN 2A, MEN 2B and FMTC : Two novel mutations and one de novo mutation for MEN 2A

    NARCIS (Netherlands)

    Landsvater, RM; Jansen, RPM; Hofstra, RMW; Buys, CHCM; Lips, CJM; vanAmstel, HKP

    Hereditary C-cell carcinoma is encountered in multiple endocrine neoplasia type 2A (MEN 2A), MEN 2B, and familial medullary thyroid carcinoma (FMTC). Mutations of the RET proto-oncogene are associated with all three diseases. To obtain an insight into the molecular heterogeneity of MEN 2 syndromes

  10. Design methodology of Dutch banknotes

    Science.gov (United States)

    de Heij, Hans A. M.

    2000-04-01

    Since the introduction of a design methodology for Dutch banknotes, the quality of Dutch paper currency has improved in more than one way. The methodology is question provides for (i) a design policy, which helps fix clear objectives; (ii) design management, to ensure a smooth cooperation between the graphic designer, printer, papermaker an central bank, (iii) a program of requirements, a banknote development guideline for all parties involved. This systematic approach enables an objective selection of design proposals, including security features. Furthermore, the project manager obtains regular feedback from the public by conducting market surveys. Each new design of a Netherlands Guilder banknote issued by the Nederlandsche Bank of the past 50 years has been an improvement on its predecessor in terms of value recognition, security and durability.

  11. Do Dutch Musea Compete Or Cooperate?

    OpenAIRE

    Thomas De Graaff; Jaap Boter; Jan Rouwendal

    2006-01-01

    This paper looks into the effect of distance on market shares of Dutch museums. To this end, we assume a generic distance decay function for all museums. In addition, we allow for spatial dependence between museums to account for local competition or synergy effects. Using a unique transaction database with the visiting behavior of 80,821 museum cardholders to 108 Dutch museums, we are able to calculate market shares of each museum in all 484 Dutch municipalities. To account for possible meas...

  12. The Dutch National Research Agenda in Perspective

    OpenAIRE

    2017-01-01

    The Dutch National Research Agenda is a set of national priorities that are set by scientists working in conjunction with corporations, civil society organisations, and interested citizens. The agenda consolidates the questions that scientific research will be focused on in the coming year. This book covers the current status of the Dutch National Research Agenda and considers what changes and adjustments may need to be made to the process in order to keep Dutch national research at the top o...

  13. Beyond the Dutch Miracle? Challenges to and Responses of the Dutch Welfare System

    NARCIS (Netherlands)

    van Gerven-Haanpää, Minna Marja-Leena; Schubert, Klaus; de Villota, Paloma; Kuhlmann, Johanna

    2016-01-01

    This chapter explores how the Dutch welfare system has evolved in the last decade(s). It shows that the economic crisis and the process of demographic change have put the Dutch welfare system to the test. The surmounting pressures from 2007 onwards have revealed structural problems of the Dutch

  14. Comparison between Dutch and German buildings

    Energy Technology Data Exchange (ETDEWEB)

    Lony, R.J.M.; Molenaar, D.J.; Rietkerk, J.; Schuiling, D.J.B.W.; Zeiler, W. [TU/e, Univ. of Technology Eindhoven (Netherlands); Brunk, M. [RWTH Aachen (Germany)

    2006-07-01

    German buildings are often seen as an example to Dutch architects and Dutch building services consultants. Goal of this article is to examine and to understand differences between the Dutch and German top office buildings. Objective is to examine to which extent these buildings were designed intelligently. An Intelligent Building is one that provides a productive cost effective environment through the optimisation of six basic elements; site, skin, systems, structures, services, space plan and staff and the interrelationship between them. Based on these six aspects the comparison is made between Dutch and German buildings. (orig.)

  15. The role of women on Dutch farms

    OpenAIRE

    Meulen, van der, H.A.B.; Terluin, I.J.; Matser, I.A.

    2015-01-01

    In this paper an analysis is made of the contribution of women to labour input and management on Dutch farms. We used a written survey among the participants of the Dutch Farm Accountancy Data Network (FADN), in-depth interviews and a group discussion with farm women. Over half of the women on Dutch farms spend more than ten hours per week on agricultural activitieson the farm. More than 40% of women on Dutch farms have paid work off farm. The majority of the respondents’ farms is legally org...

  16. ACCURACY OF PLASMA FREE METANEPHRINES IN THE DIAGNOSIS OF PHEOCHROMOCYTOMA AND PARAGANGLIOMA: A SYSTEMATIC REVIEW AND META-ANALYSIS.

    Science.gov (United States)

    Chen, Yan; Xiao, Huangmeng; Zhou, Xieda; Huang, Xiaoyu; Li, Yanbing; Xiao, Haipeng; Cao, Xiaopei

    2017-10-01

    Various studies have validated plasma free metanephrines (MNs) as biomarkers for pheochromocytoma and paraganglioma (PPGL). This meta-analysis aimed to estimate the overall diagnostic accuracy of this biochemical test for PPGL. We searched the PubMed, the Cochrane Library, Web of Science, Embase, Scopus, OvidSP, and ProQuest Dissertations & Theses databases from January 1, 1995 to December 2, 2016 and selected studies written in English that assessed plasma free MNs in the diagnosis of PPGL. Quality Assessment of Diagnostic Accuracy Studies 2 (QUADAS-2) was used to evaluate the quality of the included studies. We calculated pooled sensitivities, specificities, positive and negative likelihood ratios, diagnostic odds ratios (DORs) and areas under curve (AUCs) with their 95% confidence intervals (95% CIs). Heterogeneity was assessed by I 2 . To identify the source of heterogeneity, we evaluated the threshold effect and performed a meta-regression. Deeks' funnel plot was selected for investigating any potential publication bias. Although the combination of metanephrine (MN) and normetanephrine (NMN) carried lower specificity (0.94, 95% CI 0.90-0.97) than NMN (0.97, 95% CI 0.92-0.99), NMN was generally more accurate than individual tests, with the highest AUC (0.99, 95% CI 0.97-0.99), DOR (443.35, 95% CI 216.9-906.23), and pooled sensitivity (0.97, 95% CI 0.94-0.98) values. Threshold effect and meta-regression analyses showed that different cut-offs, blood sampling positions, study types and test methods contributed to heterogeneity. This meta-analysis suggested an effective value for combined plasma free MNs for the diagnosis of PPGL, but testing for MNs requires more standardization using tightly regulated studies. AUC = area under curve; CI = confidence interval; DOR = diagnostic odds ratio; EIA = enzyme immunoassay; LC-ECD = liquid chromatography-electrochemical detection; LC-MS/MS = liquid chromatography-tandem mass spectrometry; MN = metanephrine; NMN

  17. [Burnout in Dutch medical students: prevalence and causes].

    Science.gov (United States)

    Conijn, Maartjie; Boersma, Henri J M V; van Rhenen, Willem

    2015-01-01

    To investigate the prevalence and causes of burnout in Dutch medical students. Questionnaire survey. All 14,570 student members of the KNMG (Royal Dutch Medical Association) were invited to fill in a digital survey. Burnout was determined with the Utrecht Burnout Scale (UBOS). Triggering and protective factors for burnout were also investigated . 2,739 medical students (18.8%) completed the survey and 14.5 per cent of all respondents met the burnout criteria. 17.8 per cent of the hospital interns who responded and 11.6 per cent of the preclinical students who responded met these criteria. Work-home interference and high levels of emotional pressure had the strongest link to burn-out, while a sufficient amount of support from family, friends and peers reduced the risk of burnout in both undergraduates and hospital interns. Our exploratory research suggests that the prevalence of burnout is high, particularly among the hospital interns who responded. The most important contributory factors are high levels of emotional pressure and work-home interference. The low percentage of respondents makes it difficult to make any statement about the prevalence and causes of burnout among all Dutch medical students.

  18. Dyslexia and early intervention: what did we learn from the Dutch Dyslexia Programme?

    NARCIS (Netherlands)

    van der Leij, A.

    2013-01-01

    Part of the Dutch Dyslexia Programme has been dedicated to early intervention. The question of whether the genetically affected learning mechanism of children who are at familial risk (FR) of developing dyslexia could be influenced by training phoneme awareness and letter-sound associations in the

  19. Precursors of developmental dyslexia: an overview of the longitudinal Dutch Dyslexia Programme study

    NARCIS (Netherlands)

    van der Leij, A.; van Bergen, E.; van Zuijen, T.; de Jong, P.; Maurits, N.; Maassen, B.

    2013-01-01

    Converging evidence suggests that developmental dyslexia is a neurobiological disorder, characterized by deficits in the auditory, visual, and linguistic domains. In the longitudinal project of the Dutch Dyslexia Programme, 180 children with a familial risk of dyslexia (FR) and a comparison group of

  20. Essays on economics of language and family economics

    NARCIS (Netherlands)

    Yao, Yuxin

    2017-01-01

    The thesis consists of four chapters in economics of language and family economics. Chapter 2 studies how language problems in Dutch affect labor market performance on immigrants. Chapter 3 investigates the effect of dialect-speaking on native Dutch residents. Chapter 4 studies the effects of

  1. Effects of Morphological Family Size for Young Readers

    Science.gov (United States)

    Perdijk, Kors; Schreuder, Robert; Baayen, R. Harald; Verhoeven, Ludo

    2012-01-01

    Dutch children, from the second and fourth grade of primary school, were each given a visual lexical decision test on 210 Dutch monomorphemic words. After removing words not recognized by a majority of the younger group, (lexical) decisions were analysed by mixed-model regression methods to see whether morphological Family Size influenced decision…

  2. [Dutch parliament legitimizes harmful quackery].

    Science.gov (United States)

    van Dam, Frits S A M; Renckens, Cees N M

    2010-01-01

    The Dutch parliament has recently accepted a tax law in which certain groups of alternative therapists can be exempt from VAT. To be eligible for this VAT exemption, the disciplines to which the therapists belong have to meet certain training requirements. In this article it is contended, in agreement with the Royal College of Physicians in the UK, that statutory regulation is inappropriate for disciplines whose therapies are neither of proved benefit nor appropriately tested. It legitimizes harmful therapies. This is illustrated by two serious accidents, previously described in this journal, caused by a chiropractor and a craniosacral therapist.

  3. Seed bank characteristics of Dutch plant communities

    NARCIS (Netherlands)

    Bekker, RM; Schaminee, JHJ; Bakker, JP; Thompson, K

    With the recent appearances of a new and well-documented classification of the Dutch plant communities (Schaminee et al 1995a,b; 1996) and a database on the seed longevity of plant species of North West Europe (Thompson ct al. 1997a) it was possible to investigate patterns of seed longevity in Dutch

  4. Rewarding peak avoidance: the Dutch 'Spitsmijden' projects

    NARCIS (Netherlands)

    Knockaert, J.; Bakens, J.; Ettema, D.F.; Verhoef, E.

    2011-01-01

    The Dutch road network is becoming increasingly congested. In late 2006, a group of companies, universities and government institutions established the Spitsmijden project. ‘Spitsmijden’ is the Dutch term for ‘avoiding the peak’. This joint initiative aimed to identify and assess a short-term

  5. Lexical preferences in Dutch verbal cluster ordering

    NARCIS (Netherlands)

    Bloem, J.; Bellamy, K.; Karvovskaya, E.; Kohlberger, M.; Saad, G.

    2016-01-01

    This study discusses lexical preferences as a factor affecting the word order variation in Dutch verbal clusters. There are two grammatical word orders for Dutch two-verb clusters, with no clear meaning difference. Using the method of collostructional analysis, I find significant associations

  6. Losses on Dutch residential mortgage insurances

    NARCIS (Netherlands)

    Francke, M.K.; Schilder, F.P.W.

    2014-01-01

    Purpose - This paper aims to study the data on losses on mortgage insurance in the Dutch housing market to find the key drivers of the probability of loss. In 2013, 25 per cent of all Dutch homeowners were "under water": selling the property will not cover the outstanding mortgage debt. The

  7. Fourth Dutch Process Security Control Event

    NARCIS (Netherlands)

    Luiijf, H.A.M.; Zielstra, A.

    2010-01-01

    On December 1st, 2009, the fourth Dutch Process Control Security Event took place in Baarn, The Netherlands. The security event with the title ‘Manage IT!’ was organised by the Dutch National Infrastructure against Cybercrime (NICC). Mid of November, a group of over thirty people participated in the

  8. Dutch Corporate Finance, 1602-1850

    NARCIS (Netherlands)

    A. de Jong (Abe); J. Jonker (Joost); A. Roëll (Ailsa)

    2013-01-01

    textabstractEarly Modern Dutch corporate finance had two notable features, a remarkable ease of raising large amounts of capital and a flexible legal framework. Having pioneered new corporate forms with two intercontinental trading companies, Dutch business adopted such forms on a wider scale only

  9. Sustainable Entrepreneurship in the Dutch Construction Industry

    NARCIS (Netherlands)

    Klein Woolthuis, R.J.A.

    2010-01-01

    This article discusses the strategies that sustainable entrepreneurs use to interact with their environment in the Dutch construction industry. The Dutch construction industry is under great pressure to move towards sustainability, and entrepreneurs are believed to be able to play a large role in

  10. Sustainable entrepreneurship in the Dutch construction industry

    NARCIS (Netherlands)

    Klein Woolthuis, R.J.A.

    2010-01-01

    This article discusses the strategies that sustainable entrepreneurs use to interact with their environment in the Dutch construction industry. The Dutch construction industry is under great pressure to move towards sustainability, and entrepreneurs are believed to be able to play a large role in

  11. The Dutch private company: successfully relaunched?

    NARCIS (Netherlands)

    van der Elst, C.F.; Vermeulen, E.P.M.; De Cordt, Y.; Navez, E.J.

    2014-01-01

    This Chapter in a comparative book on private limited liability companies starts with an illustration of the former success of the Dutch limited liability company (BV). Next it addresses the competitive European legal environment within which the Dutch BV has to operate. The study shows how the

  12. Management of the Dutch development cooperation

    NARCIS (Netherlands)

    Janssen, Lodevicus Johannes Henricus

    2009-01-01

    There is reason for concern about the Dutch development cooperation. A survey of some aid evaluations of the Dutch Ministry of Foreign Affairs (MFA) showed that the aid results are limited and that the goals of the programs are not achieved. Management concerns using an organisation's resources to

  13. Accuracy of recommended sampling and assay methods for the determination of plasma-free and urinary fractionated metanephrines in the diagnosis of pheochromocytoma and paraganglioma: a systematic review.

    Science.gov (United States)

    Därr, Roland; Kuhn, Matthias; Bode, Christoph; Bornstein, Stefan R; Pacak, Karel; Lenders, Jacques W M; Eisenhofer, Graeme

    2017-06-01

    To determine the accuracy of biochemical tests for the diagnosis of pheochromocytoma and paraganglioma. A search of the PubMed database was conducted for English-language articles published between October 1958 and December 2016 on the biochemical diagnosis of pheochromocytoma and paraganglioma using immunoassay methods or high-performance liquid chromatography with coulometric/electrochemical or tandem mass spectrometric detection for measurement of fractionated metanephrines in 24-h urine collections or plasma-free metanephrines obtained under seated or supine blood sampling conditions. Application of the Standards for Reporting of Diagnostic Studies Accuracy Group criteria yielded 23 suitable articles. Summary receiver operating characteristic analysis revealed sensitivities/specificities of 94/93% and 91/93% for measurement of plasma-free metanephrines and urinary fractionated metanephrines using high-performance liquid chromatography or immunoassay methods, respectively. Partial areas under the curve were 0.947 vs. 0.911. Irrespective of the analytical method, sensitivity was significantly higher for supine compared with seated sampling, 95 vs. 89% (p sampling compared with 24-h urine, 95 vs. 90% (p sampling, seated sampling, and urine. Test accuracy increased linearly from 90 to 93% for 24-h urine at prevalence rates of 0.0-1.0, decreased linearly from 94 to 89% for seated sampling and was constant at 95% for supine conditions. Current tests for the biochemical diagnosis of pheochromocytoma and paraganglioma show excellent diagnostic accuracy. Supine sampling conditions and measurement of plasma-free metanephrines using high-performance liquid chromatography with coulometric/electrochemical or tandem mass spectrometric detection provides the highest accuracy at all prevalence rates.

  14. The SDH mutation database: an online resource for succinate dehydrogenase sequence variants involved in pheochromocytoma, paraganglioma and mitochondrial complex II deficiency

    Directory of Open Access Journals (Sweden)

    Devilee Peter

    2005-11-01

    Full Text Available Abstract Background The SDHA, SDHB, SDHC and SDHD genes encode the subunits of succinate dehydrogenase (succinate: ubiquinone oxidoreductase, a component of both the Krebs cycle and the mitochondrial respiratory chain. SDHA, a flavoprotein and SDHB, an iron-sulfur protein together constitute the catalytic domain, while SDHC and SDHD encode membrane anchors that allow the complex to participate in the respiratory chain as complex II. Germline mutations of SDHD and SDHB are a major cause of the hereditary forms of the tumors paraganglioma and pheochromocytoma. The largest subunit, SDHA, is mutated in patients with Leigh syndrome and late-onset optic atrophy, but has not as yet been identified as a factor in hereditary cancer. Description The SDH mutation database is based on the recently described Leiden Open (source Variation Database (LOVD system. The variants currently described in the database were extracted from the published literature and in some cases annotated to conform to current mutation nomenclature. Researchers can also directly submit new sequence variants online. Since the identification of SDHD, SDHC, and SDHB as classic tumor suppressor genes in 2000 and 2001, studies from research groups around the world have identified a total of 120 variants. Here we introduce all reported paraganglioma and pheochromocytoma related sequence variations in these genes, in addition to all reported mutations of SDHA. The database is now accessible online. Conclusion The SDH mutation database offers a valuable tool and resource for clinicians involved in the treatment of patients with paraganglioma-pheochromocytoma, clinical geneticists needing an overview of current knowledge, and geneticists and other researchers needing a solid foundation for further exploration of both these tumor syndromes and SDHA-related phenotypes.

  15. Effects of primary and secondary morphological family size in monolingual and bilingual word processing

    NARCIS (Netherlands)

    Mulder, K.; Dijkstra, A.F.J.; Schreuder, R.; Baayen, Harald

    2014-01-01

    This study investigated primary and secondary morphological family size effects in monolingual and bilingual processing, combining experimentation with computational modeling. Family size effects were investigated in an English lexical decision task for Dutch-English bilinguals and English

  16. Morphological Family Size Effects in Young First and Second Language Learners: Evidence of Cross-Language Semantic Activation in Visual Word Recognition

    Science.gov (United States)

    de Zeeuw, Marlies; Verhoeven, Ludo; Schreuder, Robert

    2012-01-01

    This study examined to what extent young second language (L2) learners showed morphological family size effects in L2 word recognition and whether the effects were grade-level related. Turkish-Dutch bilingual children (L2) and Dutch (first language, L1) children from second, fourth, and sixth grade performed a Dutch lexical decision task on words…

  17. The Implementation of the AIFMD in Dutch Tax Law

    NARCIS (Netherlands)

    Vermeulen, H.; Elink Schuurman, J.H.

    2014-01-01

    In this article, the authors explain the amendments to Dutch tax law as a result of the recent implementation of the Alternative Investment Fund Managers Directive. Changes were made to the Dutch Corporate Income Tax Act, the Dutch Dividend Withholding Tax Act and the Dutch General Tax Act. Given

  18. Comparison of 18F-fluoro-L-DOPA, 18F-fluoro-deoxyglucose, and 18F-fluorodopamine PET and 123I-MIBG scintigraphy in the localization of pheochromocytoma and paraganglioma.

    NARCIS (Netherlands)

    Timmers, H.J.L.M.; Chen, C.C.; Carrasquillo, J.A.; Whatley, M.; Ling, A.; Havekes, B.; Eisenhofer, G.; Martiniova, L.; Adams, K.T.; Pacak, K.

    2009-01-01

    CONTEXT: Besides (123)I-metaiodobenzylguanidine (MIBG), positron emission tomography (PET) agents are available for the localization of paraganglioma (PGL), including (18)F-3,4-dihydroxyphenylalanine (DOPA), (18)F-fluoro-2-deoxy-D-glucose ((18)F-FDG), and (18)F-fluorodopamine ((18)F-FDA). OBJECTIVE:

  19. High specificity of spot urinary free metanephrines in diagnosis and prognosis of pheochromocytomas and paragangliomas by HPLC with electrochemical detection.

    Science.gov (United States)

    Zuo, Ming; Zhen, Qianna; Zhang, Xiaoqing; Zou, Wenbi; Yang, Xiangchun; Tian, Gang; Shi, Zhenghu; Li, Qifu; Ding, Min

    2018-03-01

    The metanephrines (MNs) in plasma and urine were proposed as biomarkers for the diagnosis of pheochromocytomas and paragangliomas (PPGLs). However, plasma free MNs and 24h urinary fractionated MNs were not satisfactory enough in specificity for the diagnosis of PPGLs. Moreover, the collection of 24h urine was inconvenient. This work examined the diagnostic and prognostic efficiency of free MNs in spot urine for PPGLs. We measured free MNs concentration in spot urine and plasma of 28 PPGLs patients and 155 control subjects by HPLC with electrochemical detection. Postoperative free MNs levels in spot urine and plasma of 14 PPGLs patients were also determined. Creatinine (Cr) concentration was used for the correction of urine volume. The specificity of spot urinary free MNs/Cr in the diagnosis of PPGLs was significantly higher than that of plasma free MNs [normetanephrine (NMN), 98.7% (95.4%-99.8%) vs 93.0% (87.4%-96.6%); metanephrine (MN), 93.6% (88.5%-96.9%) vs 84.5% (77.5%-90.0%)]. Meanwhile, the positive likelihood ratios for spot urinary free NMN/Cr and MN/Cr were 69.21 and 13.29, compared with 12.68 and 5.30 for plasma free NMN and MN, respectively. For the PPGLs patients underwent surgery, the plasma free MNs level appeared an abnormal elevation and yielded false-positive results for some patients. Our findings were validated in an independent cohort, resulting in the specificity of 100% for both urinary free NMN/Cr and MN/Cr, and 97.3% and 83.8% for plasma free NMN and MN, respectively. Spot urinary free MNs/Cr, superior to plasma free MNs, presented a promising biomarker for the diagnosis and prognosis of PPGLs. Copyright © 2017 Elsevier B.V. All rights reserved.

  20. Metabolome Profiling by HRMAS NMR Spectroscopy of Pheochromocytomas and Paragangliomas Detects SDH Deficiency: Clinical and Pathophysiological Implications

    Directory of Open Access Journals (Sweden)

    Alessio Imperiale

    2015-01-01

    Full Text Available Succinate dehydrogenase gene (SDHx mutations increase susceptibility to develop pheochromocytomas/paragangliomas (PHEOs/PGLs. In the present study, we evaluate the performance and clinical applications of 1H high-resolution magic angle spinning (HRMAS nuclear magnetic resonance (NMR spectroscopy–based global metabolomic profiling in a large series of PHEOs/PGLs of different genetic backgrounds. Eighty-seven PHEOs/PGLs (48 sporadic/23 SDHx/7 von Hippel-Lindau/5 REarranged during Transfection/3 neurofibromatosis type 1/1 hypoxia-inducible factor 2α, one SDHD variant of unknown significance, and two Carney triad (CTr–related tumors were analyzed by HRMAS-NMR spectroscopy. Compared to sporadic, SDHx-related PHEOs/PGLs exhibit a specific metabolic signature characterized by increased levels of succinate (P < .0001, methionine (P = .002, glutamine (P = .002, and myoinositol (P < .0007 and decreased levels of glutamate (P < .0007, regardless of their location and catecholamine levels. Uniquely, ATP/ascorbate/glutathione was found to be associated with the secretory phenotype of PHEOs/PGLs, regardless of their genotype (P < .0007. The use of succinate as a single screening test retained excellent accuracy in distinguishing SDHx versus non–SDHx-related tumors (sensitivity/specificity: 100/100%. Moreover, the quantification of succinate could be considered a diagnostic alternative for assessing SDHx-related mutations of unknown pathogenicity. We were also able, for the first time, to uncover an SDH-like pattern in the two CTr-related PGLs. The present study demonstrates that HRMAS-NMR provides important information for SDHx-related PHEO/PGL characterization. Besides the high succinate–low glutamate hallmark, SDHx tumors also exhibit high values of methionine, a finding consistent with the hypermethylation pattern of these tumors. We also found important levels of glutamine, suggesting that glutamine metabolism might be involved in the

  1. Meta[{sup 131}I]iodobenzylguanidine therapy for patients with metastatic and unresectable pheochromocytoma and paraganglioma

    Energy Technology Data Exchange (ETDEWEB)

    Goldsby, Robert E. [Division of Pediatric Oncology, Department of Pediatrics, University of California, San Francisco, CA 94143-0106 (United States); Fitzgerald, Paul A. [Division of Endocrinology, Department of Medicine, University of California, San Francisco, CA 94143-1222 (United States)], E-mail: paul.fitzgerald@ucsf.edu

    2008-08-15

    Introduction: Pheochromocytomas (PHEOs) and paragangliomas (PGLs) are tumors that can exhibit a malignant behavior. Targeted radiotherapy with {sup 131}I-metaiodobenzylguanidine ({sup 131}I-MIBG) has proven useful in patients with unresectable, metastatic and/or relapsed disease. Methods: We review the literature and our experience at UCSF to highlight important characteristics of PHEO/PGL and the use of {sup 131}I-MIBG in the treatment of this disease. Results: These tumors are rare, with a diagnosed incidence of only two to four cases per million annually; 40% are discovered at autopsy. Clinical manifestations are caused by excess secretion of catecholamines, although some PGLs are nonsecretory. Approximately 25% of patients with PHEO/PGLs have an underlying genetic predisposition. The risk of a germline mutation is higher in children. Diagnostic evaluation should include serial determinations of fractionated metanephrines and serum chromogranin A. Staging requires both {sup 123}I-MIBG and full-body magnetic resonance imaging or {sup 18}FDG-PET scanning. The primary treatment for PHEO/PGL is resection. Patients may be candidates for treatment with {sup 131}I-MIBG if they have unresectable or metastatic tumors that are avid for MIBG. Such patients usually respond to this targeted radioisotope therapy and many achieve a durable remission. Myelosuppression is a dose-related side effect that can be treated with transfusions or autologous hematopoietic stem cells. Late side effects can include infertility, myelodysplasia and second cancers. Conclusions: Treatment with {sup 131}I-MIBG can be considered for patients if surgery is not feasible. There are significant risks associated with this treatment, but the majority of patients will respond. Treatment with {sup 131}I-MIBG should be done at institutions with experience in delivering targeted radiotherapeutics.

  2. Krebs cycle metabolite profiling for identification and stratification of pheochromocytomas/paragangliomas due to succinate dehydrogenase deficiency.

    Science.gov (United States)

    Richter, Susan; Peitzsch, Mirko; Rapizzi, Elena; Lenders, Jacques W; Qin, Nan; de Cubas, Aguirre A; Schiavi, Francesca; Rao, Jyotsna U; Beuschlein, Felix; Quinkler, Marcus; Timmers, Henri J; Opocher, Giuseppe; Mannelli, Massimo; Pacak, Karel; Robledo, Mercedes; Eisenhofer, Graeme

    2014-10-01

    Mutations of succinate dehydrogenase A/B/C/D genes (SDHx) increase susceptibility to development of pheochromocytomas and paragangliomas (PPGLs), with particularly high rates of malignancy associated with SDHB mutations. We assessed whether altered succinate dehydrogenase product-precursor relationships, manifested by differences in tumor ratios of succinate to fumarate or other metabolites, might aid in identifying and stratifying patients with SDHx mutations. PPGL tumor specimens from 233 patients, including 45 with SDHx mutations, were provided from eight tertiary referral centers for mass spectrometric analyses of Krebs cycle metabolites. Diagnostic performance of the succinate:fumarate ratio for identification of pathogenic SDHx mutations. SDH-deficient PPGLs were characterized by 25-fold higher succinate and 80% lower fumarate, cis-aconitate, and isocitrate tissue levels than PPGLs without SDHx mutations. Receiver-operating characteristic curves for use of ratios of succinate to fumarate or to cis-aconitate and isocitrate to identify SDHx mutations indicated areas under curves of 0.94 to 0.96; an optimal cut-off of 97.7 for the succinate:fumarate ratio provided a diagnostic sensitivity of 93% at a specificity of 97% to identify SDHX-mutated PPGLs. Succinate:fumarate ratios were higher in both SDHB-mutated and metastatic tumors than in those due to SDHD/C mutations or without metastases. Mass spectrometric-based measurements of ratios of succinate:fumarate and other metabolites in PPGLs offer a useful method to identify patients for testing of SDHx mutations, with additional utility to quantitatively assess functionality of mutations and metabolic factors responsible for malignant risk.

  3. Feocromocitoma-paraganglioma: del diagnóstico bioquímico al genético

    Directory of Open Access Journals (Sweden)

    Marta Cano Megías

    2016-09-01

    Full Text Available Los feocromocitomas y paragangliomas son tumores derivados de células de la cresta neural, que pueden ser diagnosticados mediante la determinación bioquímica de metanefrinas y metoxitiramina. Los avances en la investigación genética han permitido identificar múltiples genes implicados en la fisiopatogenia de estos tumores, de forma que hasta el 35-45% podrían tener una mutación germinal subyacente. Estos genes tienen una firma biológica de transcripción característica y se pueden agrupar en 2 grandes grupos (o clusters, el grupo 1 (VHL y SHDx, con implicación de la vía de la angiogénesis e hipoxia; y el grupo 2 (MEN2 y NF1, implicados en la vía de señalización de la cinasa. A su vez estos genes se asocian a un fenotipo bioquímico (adrenérgicos y noradrenérgicos, y presentación clínica (localización, comportamiento biológico, edad de presentación… característicos en un número elevado de casos. Un diagnóstico precoz de estos tumores, acompañado de un correcto diagnóstico genético, debe ser una prioridad que permita un mejor tratamiento, la detección precoz de complicaciones, un correcto screening de familiares y de otros tumores relacionados, así como una mejoría en el pronóstico global de estos pacientes.

  4. Improved Dutch Roll Approximation for Hypersonic Vehicle

    Directory of Open Access Journals (Sweden)

    Liang-Liang Yin

    2014-06-01

    Full Text Available An improved dutch roll approximation for hypersonic vehicle is presented. From the new approximations, the dutch roll frequency is shown to be a function of the stability axis yaw stability and the dutch roll damping is mainly effected by the roll damping ratio. In additional, an important parameter called roll-to-yaw ratio is obtained to describe the dutch roll mode. Solution shows that large-roll-to-yaw ratio is the generate character of hypersonic vehicle, which results the large error for the practical approximation. Predictions from the literal approximations derived in this paper are compared with actual numerical values for s example hypersonic vehicle, results show the approximations work well and the error is below 10 %.

  5. Clinical practice: neonatal resuscitation. A Dutch consensus

    NARCIS (Netherlands)

    van den Dungen, F.A.M.; van Veenendaal, M.B.; Mulder, A.L.M.

    2010-01-01

    The updated Dutch guidelines on Neonatal Resuscitation assimilate the latest evidence in neonatal resuscitation. Important changes with regard to the 2004 guidelines and controversial issues concerning neonatal resuscitation are reviewed, and recommendations for daily practice are provided and

  6. Subjective Oral Health in Dutch Adults

    Directory of Open Access Journals (Sweden)

    Gijsbert H.W. Verrips

    2013-05-01

    Full Text Available Aim: To determine whether the subjective oral health (SOH of the Dutch adult population was associated with clinical and demographic variables. Methods: A clinical examination was conducted in a sample of 1,018 people from the Dutch city of ‘s-Hertogenbosch. SOH was measured using the Dutch translation of the short form of the Oral Health Impact Profile (OHIP-NL14. Results: The average score on the OHIP-NL14 was 2.8 ± 5.9 and 51% of the respondents had a score of 0. Dental status was the most important predictor of SOH. Conclusions:  The SOH in the Dutch adult population was much better than in groups of adults in Australia, the United Kingdom and New Zealand. Nevertheless, there were important variations in SOH related to dental and socio-economic status.

  7. Ethnic identity, externalizing problem behaviour and the mediating role of self-esteem among Dutch, Turkish-Dutch and Moroccan-Dutch adolescents

    NARCIS (Netherlands)

    Wissink, I.B.; Deković, M.; Yağmur, S.; Stams, G.J.; de Haan, M.

    2008-01-01

    The present study examined whether self-esteem mediates the relationship between two aspects of ethnic identity (i.e. ethnic identity exploration and ethnic identity commitment-affirmation) and externalizing problem behaviour in Dutch, Turkish-Dutch and Moroccan-Dutch adolescents living in the

  8. Ethnic Identity, Externalizing Problem Behaviour and the Mediating Role of Self-Esteem among Dutch, Turkish-Dutch and Moroccan-Dutch Adolescents

    Science.gov (United States)

    Wissink, Inge B.; Dekovic, Maja; Yagmur, Sengul; Stams, Geert Jan; de Haan, Mariette

    2008-01-01

    The present study examined whether self-esteem mediates the relationship between two aspects of ethnic identity (i.e. ethnic identity exploration and ethnic identity commitment-affirmation) and externalizing problem behaviour in Dutch, Turkish-Dutch and Moroccan-Dutch adolescents living in the Netherlands. A total number of 345 adolescents (115…

  9. Dutch voices: exploring the role of oral history in Dutch secondary history teaching

    NARCIS (Netherlands)

    Huijgen, Tim; Holthuis, Paul; Trškan, Danijela

    2016-01-01

    Oral history may enhance students’ historical content knowledge, historical reasoning competencies, and motivation to learn history. However, little is known regarding the role of oral history in Dutch history education. This study therefore explores the role of oral history in Dutch history

  10. Perceived antecedents of marital satisfaction among Turkish, Turkish-Dutch, and Dutch couples

    NARCIS (Netherlands)

    Celenk, O.; van de Vijver, F.J.R.

    2013-01-01

    We studied mainstream couples in The Netherlands and Turkey as well as Turkish-Dutch immigrant couples to address cultural factors associated with marital satisfaction. A total of 13 Turkish (mainstream couples living in Turkey), 19 Turkish-Dutch (Turkish immigrant couples living in The

  11. Marxism and the 'Dutch miracle': the Dutch Republic and the transition-debate

    NARCIS (Netherlands)

    Brandon, P.

    2011-01-01

    The Dutch Republic holds a marginal position in the debate on the transition from feudalism to capitalism, despite its significance in the early stage of the development of global capitalism. While the positions of those Marxists who did consider the Dutch case range from seeing it as the first

  12. ZNF408 is mutated in familial exudative vitreoretinopathy and is crucial for the development of zebrafish retinal vasculature

    NARCIS (Netherlands)

    Collin, R.W.J.; Nikopoulos, K.; Dona, M.A.; Gilissen, C.F.H.A.; Hoischen, A.; Boonstra, F.N.; Poulter, J.A.; Kondo, H.; Berger, W.; Toomes, C.; Tahira, T.; Mohn, L.R.; Blokland, E.A.W.; Hetterschijt, L.; Ali, M.; Groothuismink, J.M.; Duijkers, L.E.M.; Inglehearn, C.F.; Sollfrank, L.; Strom, T.M.; Uchio, E.; Nouhuys, C.E. van; Kremer, H.; Veltman, J.A.; Wijk, E. van; Cremers, F.P.M.

    2013-01-01

    Familial exudative vitreoretinopathy (FEVR) is a genetically heterogeneous disorder characterized by abnormal vascularization of the peripheral retina, which can result in retinal detachment and severe visual impairment. In a large Dutch FEVR family, we performed linkage analysis, exome sequencing,

  13. Radioactivity in Dutch consumer products

    CERN Document Server

    Janssen, M P M

    2002-01-01

    This study took place within the framework of a general update of the average radiation dose for the Dutch population. It focuses on consumer products in which radionuclides have been intentionally incorporated and on radiation-emitting devices that can be supplied to members of the public without special surveillance. Eleven consumer products were studied in more detail. The radiation from these products determined 90% of the total collective dose due to consumer products in the Netherlands in 1988. Individual and collective doses are presented here for each product. The total collective dose has decreased from 130 personSv in 1988 to 4.6 personSv at present. This reduction was attributed to: a decrease in the number of radioactive products (gas mantles), lower estimates of the number of radioactive products present in the Netherlands thanks to new information (camera lenses, smoke detectors containing Ra-226), replacement of radioactive by non-radioactive products (gas mantles, dental protheses), and a lowe...

  14. Opportunities for Dutch Roadmap Biorefineries

    International Nuclear Information System (INIS)

    Annevelink, E.; Broeze, J.; Van Ree, R.

    2009-09-01

    This Dutch Roadmap Biorefinery forms the framework and knowledge basis for Research, Development and Demonstration (RD and D) activities, covering both technical and non-technical issues, necessary to develop biorefinery-based value chains to such an extend that large-scale market implementation as part of the future Bio-based Economy will become a reality. The Roadmap describes the broad landscape of biorefinery options in The Netherlands. The descriptions of possible initiatives within the so called Moonshots (general biorefinery strategies containing more specific biorefinery-based value chains that will become fully operational at industrial scale in the short and midterm to facilitate the transition to a Bio-based Economy in the longer-term) deliberately do not contain the names of parties that might be involved. However, many of the current initiatives have been described in another document, the 'Status Report Biorefinery 2007'. Also the exact economics of possible initiatives have not been specified yet. These will become clearer when proposals will be submitted by consortia of the stakeholders involved.

  15. MOLECULAR-GENETIC REEVALUATION OF THE DUTCH HYPEREKPLEXIA FAMILY

    NARCIS (Netherlands)

    de Koning-Tijssen, Marina; SHIANG, R; VANDEUTEKOM, J; BOERMAN, RH; WASMUTH, JJ; SANDKUIJL, LA; FRANTS, RR; PADBERG, GW

    Objectives: To confirm linkage of the locus of the major form of hyperekplexia to markers on chromosome 5q, to screen for a point mutation in the gene encoding the oil subunit of the glycine receptor, and to investigate whether the putative ''minor'' form of hyperekplexia, consisting of an excessive

  16. Diagnostic performance of {sup 18}F-dihydroxyphenylalanine positron emission tomography in patients with paraganglioma: a meta-analysis

    Energy Technology Data Exchange (ETDEWEB)

    Treglia, Giorgio; Cocciolillo, Fabrizio; Castaldi, Paola; Rufini, Vittoria; Giordano, Alessandro [Catholic University of the Sacred Heart, Institute of Nuclear Medicine, Rome (Italy); De Waure, Chiara; Di Nardo, Francesco; Gualano, Maria Rosaria [Catholic University of the Sacred Heart, Institute of Hygiene, Rome (Italy)

    2012-07-15

    The aim of this study was to systematically review and conduct a meta-analysis of published data about the diagnostic performance of {sup 18}F-dihydroxyphenylalanine (DOPA) positron emission tomography (PET) in patients with paraganglioma (PG). A comprehensive computer literature search of studies published through 30 June 2011 regarding {sup 18}F-DOPA PET or PET/computed tomography (PET/CT) in patients with PG was performed in PubMed/MEDLINE, Embase and Scopus databases. Pooled sensitivity and specificity of {sup 18}F-DOPA PET or PET/CT in patients with PG on a per patient- and on a per lesion-based analysis were calculated. The area under the receiver-operating characteristic (ROC) curve was calculated to measure the accuracy of {sup 18}F-DOPA PET or PET/CT in patients with PG. Furthermore, a sub-analysis taking into account the different genetic mutations in PG patients was also performed. Eleven studies comprising 275 patients with suspected PG were included in this meta-analysis. The pooled sensitivity of {sup 18}F-DOPA PET and PET/CT in detecting PG was 91% [95% confidence interval (CI) 87-94%] on a per patient-based analysis and 79% (95% CI 76-81%) on a per lesion-based analysis. The pooled specificity of {sup 18}F-DOPA PET and PET/CT in detecting PG was 95% (95% CI 86-99%) on a per patient-based analysis and 95% (95% CI 84-99%) on a per lesion-based analysis. The area under the ROC curve was 0.95 on a per patient- and 0.94 on a per lesion-based analysis. Heterogeneity between the studies about sensitivity of {sup 18}F-DOPA PET or PET/CT was found. A significant increase in sensitivity of {sup 18}F-DOPA PET or PET/CT was observed when a sub-analysis excluding patients with succinate dehydrogenase subunit B (SDHB) gene mutations was performed. In patients with suspected PG {sup 18}F-DOPA PET or PET/CT demonstrated high sensitivity and specificity. {sup 18}F-DOPA PET or PET/CT are accurate methods in this setting. Nevertheless, possible sources of false

  17. 18F-fluorodihydroxyphenylalanine PET/CT in pheochromocytoma and paraganglioma: relation to genotype and amino acid transport system L

    International Nuclear Information System (INIS)

    Feral, Chloe C.; Tissot, Floriane S.; Tosello, Lionel; Fakhry, Nicolas; Sebag, Frederic; Pacak, Karel; Taieb, David

    2017-01-01

    F-FDOPA is a highly sensitive and specific radiopharmaceutical for pheochromocytoma and paraganglioma (PPGL) imaging. However, 18 F-FDOPA might be falsely negative in these tumors, especially those related to mutations in succinate dehydrogenase genes (SDHx). The aim of the present study was to evaluate the relationship between expression of L-DOPA transporters and 18 F-FDOPA PET imaging results in PPGL. From 2007 to 2015, 175 patients with non-metastatic PPGL were evaluated by 18 F-FDOPA PET/CT for initial diagnosis/staging and follow-up. 18 F-FDOPA PET/CT was considered as falsely negative for at least one lesion in 10/126 (8%) patients (two sporadic, six SDHD, two SDHB PPGLs). The mRNA and protein expression levels of CD98hc and LATs were evaluated in samples with different genetic backgrounds and imaging phenotypes. The qRT-PCR and immunohistochemical analyses were performed in 14 and 16 tumor samples, respectively. The SDHx mutated samples exhibited a significant decrease in mRNA expression of LAT3 when compared to sporadic PPGLs (P = 0.042). There was also a statistical trend toward decreased CD98hc (P = 0.147) and LAT4 (P = 0.012) levels in SDHx vs sporadic PPGLs. No difference was observed for LAT1/LAT2 mRNA levels. LAT1 protein was expressed in 15 out of 16 (93.75%) SDHx tumors, regardless of the 18 F-FDOPA positivity. LAT1 and CD98hc were co-expressed in 6/8 18 F-FDOPA-negative PPGLs. In contrast, in one case with absence of LAT1/CD98hc, 18 F-FDOPA uptake was positive and attributed to LAT4 expression. We conclude that down-regulation of LAT1/CD98hc cannot explain the imaging phenotype of SDHx-related PPGLs. A reduced activity of LAT1 remains the primary hypothesis possibly due to a modification of intracellular amino acid content which may reduce 18 F-FDOPA uptake. (orig.)

  18. Diagnostic performance of 18F-dihydroxyphenylalanine positron emission tomography in patients with paraganglioma: a meta-analysis

    International Nuclear Information System (INIS)

    Treglia, Giorgio; Cocciolillo, Fabrizio; Castaldi, Paola; Rufini, Vittoria; Giordano, Alessandro; De Waure, Chiara; Di Nardo, Francesco; Gualano, Maria Rosaria

    2012-01-01

    The aim of this study was to systematically review and conduct a meta-analysis of published data about the diagnostic performance of 18 F-dihydroxyphenylalanine (DOPA) positron emission tomography (PET) in patients with paraganglioma (PG). A comprehensive computer literature search of studies published through 30 June 2011 regarding 18 F-DOPA PET or PET/computed tomography (PET/CT) in patients with PG was performed in PubMed/MEDLINE, Embase and Scopus databases. Pooled sensitivity and specificity of 18 F-DOPA PET or PET/CT in patients with PG on a per patient- and on a per lesion-based analysis were calculated. The area under the receiver-operating characteristic (ROC) curve was calculated to measure the accuracy of 18 F-DOPA PET or PET/CT in patients with PG. Furthermore, a sub-analysis taking into account the different genetic mutations in PG patients was also performed. Eleven studies comprising 275 patients with suspected PG were included in this meta-analysis. The pooled sensitivity of 18 F-DOPA PET and PET/CT in detecting PG was 91% [95% confidence interval (CI) 87-94%] on a per patient-based analysis and 79% (95% CI 76-81%) on a per lesion-based analysis. The pooled specificity of 18 F-DOPA PET and PET/CT in detecting PG was 95% (95% CI 86-99%) on a per patient-based analysis and 95% (95% CI 84-99%) on a per lesion-based analysis. The area under the ROC curve was 0.95 on a per patient- and 0.94 on a per lesion-based analysis. Heterogeneity between the studies about sensitivity of 18 F-DOPA PET or PET/CT was found. A significant increase in sensitivity of 18 F-DOPA PET or PET/CT was observed when a sub-analysis excluding patients with succinate dehydrogenase subunit B (SDHB) gene mutations was performed. In patients with suspected PG 18 F-DOPA PET or PET/CT demonstrated high sensitivity and specificity. 18 F-DOPA PET or PET/CT are accurate methods in this setting. Nevertheless, possible sources of false-negative results should be kept in mind. Furthermore

  19. Peptide receptor radionuclide therapy with 90Y/177Lu-labelled peptides for inoperable head and neck paragangliomas (glomus tumours)

    International Nuclear Information System (INIS)

    Puranik, Ameya D.; Kulkarni, Harshad R.; Singh, Aviral; Baum, Richard P.

    2015-01-01

    Head and neck paragangliomas (HNPGLs) are rare tumours arising from autonomic nervous system ganglia. Although surgery offers the best chance of complete cure, there is associated morbidity due to the crucial location of these tumours. Radiotherapy arrests tumour growth and provides symptomatic improvement, but has long-term consequences. These tumours express somatostatin receptors (SSTR) and hence peptide receptor radionuclide therapy (PRRT) is now a treatment option. We assessed the molecular, morphological and clinical responses of inoperable HNPGLs to PRRT. Nine patients with inoperable HNPGL assessed between June 2006 and June 2014 were included. Four patients had a solitary lesion, four had multifocal involvement and one had distant metastases (bone and lungs). The patients were treated with PRRT using 90 Y/ 177 Lu-labelled peptides after positive confirmation of SSTR expression on 68 Ga-DOTATOC PET/CT. All patients received two to four courses of PRRT. Subsequent serial imaging with 68 Ga-DOTATOC PET/CT was carried out every 6 months to assess response to treatment. Clinical (symptomatic) response was also assessed. Based on molecular response (EORTC) criteria, four of the nine patients showed a partial molecular response to treatment seen as significant decreases in SUV max , accompanied by a reduction in tumour size. Five patients showed stable disease on both molecular and morphological criteria. Six out of nine patients were symptomatic at presentation with manifestations of cranial nerve involvement, bone destruction at the primary site and metastatic bone pain. Molecular responses were correlated with symptomatic improvement in four out of these six patients; while two patients showed small reductions in tumour size and SUV max . The three asymptomatic patients showed no new lesions or symptomatic worsening. PRRT was effective in all patients, with no disease worsening seen, either in the form of neurological symptoms or distant spread. Though these

  20. Diagnostic performance of 18F-dihydroxyphenylalanine positron emission tomography in patients with paraganglioma: a meta-analysis.

    Science.gov (United States)

    Treglia, Giorgio; Cocciolillo, Fabrizio; de Waure, Chiara; Di Nardo, Francesco; Gualano, Maria Rosaria; Castaldi, Paola; Rufini, Vittoria; Giordano, Alessandro

    2012-07-01

    The aim of this study was to systematically review and conduct a meta-analysis of published data about the diagnostic performance of (18)F-dihydroxyphenylalanine (DOPA) positron emission tomography (PET) in patients with paraganglioma (PG). A comprehensive computer literature search of studies published through 30 June 2011 regarding (18)F-DOPA PET or PET/computed tomography (PET/CT) in patients with PG was performed in PubMed/MEDLINE, Embase and Scopus databases. Pooled sensitivity and specificity of (18)F-DOPA PET or PET/CT in patients with PG on a per patient- and on a per lesion-based analysis were calculated. The area under the receiver-operating characteristic (ROC) curve was calculated to measure the accuracy of (18)F-DOPA PET or PET/CT in patients with PG. Furthermore, a sub-analysis taking into account the different genetic mutations in PG patients was also performed. Eleven studies comprising 275 patients with suspected PG were included in this meta-analysis. The pooled sensitivity of (18)F-DOPA PET and PET/CT in detecting PG was 91% [95% confidence interval (CI) 87-94%] on a per patient-based analysis and 79% (95% CI 76-81%) on a per lesion-based analysis. The pooled specificity of (18)F-DOPA PET and PET/CT in detecting PG was 95% (95% CI 86-99%) on a per patient-based analysis and 95% (95% CI 84-99%) on a per lesion-based analysis. The area under the ROC curve was 0.95 on a per patient- and 0.94 on a per lesion-based analysis. Heterogeneity between the studies about sensitivity of (18)F-DOPA PET or PET/CT was found. A significant increase in sensitivity of (18)F-DOPA PET or PET/CT was observed when a sub-analysis excluding patients with succinate dehydrogenase subunit B (SDHB) gene mutations was performed. In patients with suspected PG (18)F-DOPA PET or PET/CT demonstrated high sensitivity and specificity. (18)F-DOPA PET or PET/CT are accurate methods in this setting. Nevertheless, possible sources of false-negative results should be kept in mind

  1. Warburg effect's manifestation in aggressive pheochromocytomas and paragangliomas: insights from a mouse cell model applied to human tumor tissue.

    Directory of Open Access Journals (Sweden)

    Stephanie M J Fliedner

    Full Text Available A glycolytic profile unifies a group of pheochromocytomas and paragangliomas (PHEOs/PGLs with distinct underlying gene defects, including von Hippel-Lindau (VHL and succinate dehydrogenase B (SDHB mutations. Nevertheless, their tumor aggressiveness is distinct: PHEOs/PGLs metastasize rarely in VHL-, but frequently in SDHB-patients. To date, the molecular mechanisms causing the more aggressive phenotype in SDHB-PHEOs/PGLs remain largely unknown. Recently, however, an excellent model to study aggressive PHEOs (mouse tumor tissue (MTT cells has been developed from mouse PHEO cells (MPC. We employed this model for a proteomics based approach to identify changes characteristic for tumor aggressiveness, which we then explored in a homogeneous set of human SDHB- and VHL-PHEOs/PGLs. The increase of glucose transporter 1 in VHL, and of hexokinase 2 in VHL and SDHB, confirmed their glycolytic profile. In agreement with the cell model and in support of decoupling of glycolysis, the Krebs cycle and oxidative phosphorylation (OXPHOS, SDHB tumors showed increased lactate dehydrogenase levels. In SDHB-PGLs OXPHOS complex activity was increased at complex III and, as expected, decreased at complex II. Moreover, protein and mRNA expression of all tested OXPHOS-related genes were higher in SDHB- than in VHL-derived tumors. Although there was no direct evidence for increased reactive oxygen species production, elevated superoxide dismutase 2 expression may reflect elevated oxidative stress in SDHB-derived PHEOs/PGLs. For the first time, we show that despite dysfunction in complex II and evidence for a glycolytic phenotype, the Warburg effect does not seem to fully apply to SDHB-PHEOs/PGLs with respect to decreased OXPHOS. In addition, we present evidence for increased LDHA and SOD2 expression in SDHB-PHEOs/PGLs, proteins that have been proposed as promising therapeutic targets in other cancers. This study provides new insight into pathogenic mechanisms in

  2. Minimally Invasive Surgery (MIS) in Children and Adolescents with Pheochromocytomas and Retroperitoneal Paragangliomas: Experiences in 42 Patients.

    Science.gov (United States)

    Walz, Martin K; Iova, Laura D; Deimel, Judith; Neumann, Hartmut P H; Bausch, Birke; Zschiedrich, Stefan; Groeben, Harald; Alesina, Pier F

    2018-04-01

    Pheochromocytomas (PH) and paragangliomas (PGL) are rare tumours in children accounting for about 1% of the paediatric hypertension. While minimally invasive surgical techniques are well established in adult patients with PH, the experience in children is extremely limited. To the best of our knowledge, we herewith present the largest series of young patients operated on chromaffin tumours by minimally invasive access. In the setting of a prospective study (1/2001-12/2016), 42 consecutive children and adolescents (33 m, 9 f) were operated on. Thirty-seven patients (88%) suffered from inherited diseases. Twenty-six patients had PH, 11 presented retroperitoneal PGL, and five patients suffered from both. Altogether, 70 tumours (mean size 2.7 cm) were removed (45 PH, 25 PGL). All operations were performed by a minimally invasive access (retroperitoneoscopic, laparoscopic, extraperitoneal). Partial adrenalectomy was the preferred procedure for PH (31 out of 39 patients). Twenty patients received α-receptor blockade preoperatively. One patient died after induction of anaesthesia due to cardiac arrest. All other complications were minor. Conversion to open surgery was necessary in two cases with PGL. Median operating time for unilateral PH was 55 min, in bilateral cases 125, 143 min in PGs, and 180 min in combined cases. Median blood loss was 20 ml (range 0-1000). Blood transfusion was necessary in two cases. Intraoperative, systolic peak pressure was 170 ± 39 mmHg with α-receptor blockade and 191 ± 33 mmHg without α-receptor blockade (p = 0.41). The median post-operative hospital stay was 3 days. After a mean follow-up of 8.5 years, two patients presented ipsilateral recurrence (after partial adrenalectomy). All patients with bilateral PH (n = 13) are steroid independent post-operatively. PH and PGL in children and adolescents should preferably be removed by minimally invasive surgery. Partial adrenalectomy provides long-term steroid independence

  3. Dutch Druggists in Distress : Franchisees Facing the Complex Decision of How to React to Their Franchisor's Strategic Plans

    NARCIS (Netherlands)

    Croonen, E.P.M.; Brand, M.J.

    This case focuses on the decisions confronting Marc van der Bilt and his family. At the age of 59, Marc had been a franchisee of the Dutch DA drugstore chain for 23 years and had always planned to remain one until his retirement. In the spring of 2003, however, a change of management at the DA

  4. Correlates of self-reported adult attachment styles in a Dutch sample of married men and women

    NARCIS (Netherlands)

    Gerlsma, C.; Buunk, Abraham (Bram); Mutsaers, W.C M

    The relationships between attachment style, recollections of the family of origin, working model of self and others and current relational satisfaction were examined in a Dutch sample of married subjects from the general community. Measures were adapted from those developed by Hazan & Shaver (1987).

  5. DNA barcoding of Dutch birds

    Directory of Open Access Journals (Sweden)

    Mansour Aliabadian

    2013-12-01

    Full Text Available The mitochondrial cytochrome c-oxidase subunit I (COI can serve as a fast and accurate marker for the identification of animal species, and has been applied in a number of studies on birds. We here sequenced the COI gene for 387 individuals of 147 species of birds from the Netherlands, with 83 species being represented by >2 sequences. The Netherlands occupies a small geographic area and 95% of all samples were collected within a 50 km radius from one another. The intraspecific divergences averaged 0.29% among this assemblage, but most values were lower; the interspecific divergences averaged 9.54%. In all, 95% of species were represented by a unique barcode, with 6 species of gulls and skua (Larus and Stercorariusat least one shared barcode. This is best explained by these species representing recent radiations with ongoing hybridization. In contrast, one species, the Lesser Whitethroat Sylvia curruca showed deep divergences, averaging 5.76% and up to 8.68% between individuals. These possibly represent two distinct taxa, S. curruca and S. blythi, both clearly separated in a haplotype network analysis. Our study adds to a growing body of DNA barcodes that have become available for birds, and shows that a DNA barcoding approach enables to identify known Dutch bird species with a very high resolution. In addition some species were flagged up for further detailed taxonomic investigation, illustrating that even in ornithologically well-known areas such as the Netherlands, more is to be learned about the birds that are present.

  6. Perceived antecedents of marital satisfaction among Turkish, Turkish-Dutch, and Dutch couples.

    Science.gov (United States)

    Celenk, Ozgur; van de Vijver, Fons J R

    2013-01-01

    We studied mainstream couples in The Netherlands and Turkey as well as Turkish-Dutch immigrant couples to address cultural factors associated with marital satisfaction. A total of 13 Turkish (mainstream couples living in Turkey), 19 Turkish-Dutch (Turkish immigrant couples living in The Netherlands), and 17 Dutch (mainstream couples living in The Netherlands) married dyads (total of 98 individuals) were independently interviewed about positive and negative characteristics of marriages, determinants of general marital satisfaction and dissatisfaction, spousal communication, marital conflict, and marital roles. Multivariate tests revealed ethnic group differences on all marriage-related domains except the conflict resolution strategies. However, univariate analyses showed differences in few themes within domains; main differences were assessed between the Turkish/Turkish-Dutch (who put more emphasis on children and economical aspects) and Dutch couples (who put more emphasis on behavior, and personality of the spouse, reciprocity, emotional sharing, and psychological roles). Turkish-Dutch couples were more similar to Turkish than to Dutch couples. Results were discussed in light of the socioeconomic development and cultural value theories, which are believed to provide a useful framework for understanding the role of culture in marital satisfaction.

  7. Tolerance at arm's length: the Dutch experience.

    Science.gov (United States)

    Schuijer, J

    1990-01-01

    With respect to pedophilia and the age of consent, the Netherlands warrants special attention. Although pedophilia is not as widely accepted in the Netherlands as sometimes is supposed, developments in the judicial practice showed a growing reservedness. These developments are a spin-off of related developments in Dutch society. The tolerance in the Dutch society has roots that go far back in history and is also a consequence of the way this society is structured. The social changes of the sixties and seventies resulted in a "tolerance at arm's length" for pedophiles, which proved to be deceptive when the Dutch government proposed to lower the age of consent in 1985. It resulted in a vehement public outcry. The prevailing sex laws have been the prime target of protagonists of pedophile emancipation. Around 1960, organized as a group, they started to undertake several activities. In the course of their existence, they came to redefine the issue of pedophilia as one of youth emancipation.

  8. Thai migrant women in the Netherlands : cross-cultural marriages and families

    NARCIS (Netherlands)

    Suksomboon, Panitee

    2009-01-01

    This thesis has focused on the cross-cultural marriage of Thai migrant women in the Netherlands and the ups-and-downs of their everyday life of creating and maintaining their relationships with their Dutch husband, Dutch in-laws, Thai relatives and friends in the Netherlands as well as family and

  9. The Strange Death of Dutch Tolerance: the Timing and Nature of the Pessimist Turn in the Dutch Migration Debate

    NARCIS (Netherlands)

    Lucassen, Leo; Lucassen, Jan

    2015-01-01

    In this article we explain why the Dutch nativist turn from the 1990s onwards was nourished both by the political left and the right and what the role was of specific Dutch developments in secularization and libertarian attitudes

  10. Prepayment Behavior of Dutch Mortgagors : An Empirical Analysis

    NARCIS (Netherlands)

    Charlier, E.; van Bussel, A.

    2001-01-01

    The booming Dutch mortgage market and the development of a promising secondary mortgage market in the Netherlands stress the need for an accurate mortgage prepayment model that incorporates typical Dutch market and contract characteristics.One of those typical Dutch features prescribes that each

  11. Home Bias and Dutch Pension Funds’ Investment behaviour

    NARCIS (Netherlands)

    Verschoor, W.F.C.; Rubbaniy, G.; Lelyveld van, I.P.P.

    2014-01-01

    Using a panel data set of more than 600 Dutch pension funds (PFs) between 1992 and 2006, we investigate asset allocation behavior of Dutch PFs across multiple asset classes. We find that domestic investments, also known as home bias, in portfolio choices of Dutch institutional investors have fallen.

  12. Eumedion in the Dutch Corporate Governance and Sustainability Landscape

    NARCIS (Netherlands)

    Hermes, Niels; Hooghiemstra, Reggy; van Veen, Kees

    2016-01-01

    Eumedion is a Dutch foundation representing the interests of Dutch and foreign institutional investors with investments in Dutch listed companies. In particular, it represents the interests of these participants in the field of corporate governance and sustainability. The foundation was established

  13. A memory-based shallow parser for spoken Dutch

    NARCIS (Netherlands)

    Canisius, S.V.M.; van den Bosch, A.; Decadt, B.; Hoste, V.; De Pauw, G.

    2004-01-01

    We describe the development of a Dutch memory-based shallow parser. The availability of large treebanks for Dutch, such as the one provided by the Spoken Dutch Corpus, allows memory-based learners to be trained on examples of shallow parsing taken from the treebank, and act as a shallow parser after

  14. European Society of Endocrinology Clinical Practice Guideline for long-term follow-up of patients operated on for a phaeochromocytoma or a paraganglioma.

    Science.gov (United States)

    Plouin, P F; Amar, L; Dekkers, O M; Fassnacht, M; Gimenez-Roqueplo, A P; Lenders, J W M; Lussey-Lepoutre, C; Steichen, O

    2016-05-01

    Phaeochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumours. Standard treatment is surgical resection. Following complete resection of the primary tumour, patients with PPGL are at risk of developing new tumoural events. The present guideline aims to propose standardised clinical care of long-term follow-up in patients operated on for a PPGL. The guideline has been developed by The European Society of Endocrinology and based on the Grading of Recommendations Assessment, Development and Evaluation (GRADE) principles. We performed a systematic review of the literature and analysed the European Network for the Study of Adrenal Tumours (ENS@T) database. The risk of new events persisted in the long term and was higher for patients with genetic or syndromic diseases. Follow-up in the published cohorts and in the ENS@T database was neither standardised nor exhaustive, resulting in a risk of follow-up bias and in low statistical power beyond 10 years after complete surgery. To inform patients and care providers in this context of low-quality evidence, the Guideline Working Group therefore prepared recommendations on the basis of expert consensus. Key recommendations are the following: we recommend that all patients with PPGL be considered for genetic testing; we recommend assaying plasma or urinary metanephrines every year to screen for local or metastatic recurrences or new tumours; and we suggest follow-up for at least 10 years in all patients operated on for a PPGL. High-risk patients (young patients and those with a genetic disease, a large tumour and/or a paraganglioma) should be offered lifelong annual follow-up. © 2016 European Society of Endocrinology.

  15. A Somatic HIF2α Mutation-Induced Multiple and Recurrent Pheochromocytoma/Paraganglioma with Polycythemia: Clinical Study with Literature Review.

    Science.gov (United States)

    Liu, Qiuli; Wang, Yan; Tong, Dali; Liu, Gaolei; Yuan, Wenqiang; Zhang, Jun; Ye, Jin; Zhang, Yao; Yuan, Gang; Feng, Qingxing; Zhang, Dianzheng; Jiang, Jun

    2017-03-01

    A syndrome known as pheochromocytomas (PCC)/paragangliomas (PGL) and polycythemia resulted from gain-of-function mutation of hypoxia-inducible factor 2α (HIF2α) has been reported recently. However, clinical features of this syndrome vary from patient to patient. In our study, we described the clinical features of the patient within 15-year follow-up with a literature review. The patient presented with "red face" since childhood and was diagnosed with polycythemia and pheochromocytoma in 2000, and then, tumor was removed at his age of 27 (year 2000). However, 13 years later (2013), he was diagnosed with multiple paragangliomas. Moreover, 2 years later (2015), another two paragangaliomas were also confirmed. Genetic analysis of hereditary PCC/PGL-related genes was conducted. A somatic heterozygous missense mutation of HIF2α (c.1589C>T) was identified at exon 12, which is responsible for the elevated levels of HIF2α and erythropoietin (EPO) and subsequent development of paragangaliomas. However, this mutation was only found in the tumors from three different areas, not in the blood. So far, 13 cases of PCC/PGL with polycythemia have been reported. Among them, somatic mutations of HIF2α at exon 12 are responsible for 12 cases, and only 1 case was caused by germline mutation of HIF2α at exon 9. The HIF2α mutation-induced polycythemia with PCC/PGL is a rare syndrome with no treatment for cure. Comprehensive therapies for this disease include removal of the tumors and intermittent phlebotomies; administration of medications to control blood pressure and to prevent complications or death resulted from high concentration of red blood cell (RBC). Genetic test is strongly recommended for patients with early onset of polycythemia and multiple/recurrent PCC/PGL.

  16. Liquidity in the Dutch wholesale electricity market

    International Nuclear Information System (INIS)

    Newbery, D.; Von der Fehr, N.H.; Van Damme, E.

    2003-05-01

    Industry concerns over perceived reductions in the liquidity of the Dutch wholesale electricity market led the DTe to ask the Market Surveillance Committee (MSC) to examine recent developments. This report starts with a generic examination of wholesale power markets and liquidity and its measurement. An overview of the Dutch wholesale electricity market and its constituent segments follows together with a summary of events and opinions connected to liquidity that have been reported in the trade press. Sources of information on market liquidity are then reviewed. Participation in the market is analysed before examining each market segment and this analysis and the earlier sections are then drawn together in conclusions and recommendations

  17. Royal Dutch Petroleum Company annual report 1992

    International Nuclear Information System (INIS)

    Anon.

    1993-01-01

    The Royal Dutch Petroleum Company has no operations of its own and virtually the whole of its income derives from its 60% interest in the companies known collectively as the Royal Dutch/Shell Group of Companies; the other 40% is owned by the Shell Transport and Trading Company, p.l.c. The company is engaged in the oil, natural gas, chemicals, coal and metals businesses throughout the world. The annual report summarises the year's results and analyses earnings in each industry segment. Financial statements for the year ended 31 December 1992 are presented. The Group companies' estimated net quantities of crude oil, natural gas and coal are given

  18. The Dutch gas policy: 'Dutch disease' or imaginary disease. CERI Studies Nr 11

    International Nuclear Information System (INIS)

    Crowley, John

    1998-04-01

    The Netherlands are the first European gas producer and exporter. The author proposes an analysis of the gas policy of this country. He first recalls some elements of context: peculiarities of energy as a domain of public policy, and characteristics of the Dutch political system. He recalls the historical development of the Dutch gas industry from the 1960's: political economics of natural gas and of competing fuels, development of the Groningen site as the keystone of the Dutch gas policy, and regulation of the industrial sector. In the next part, the author comments the emergence of energy as a topic of public debate from 1995, and how the Dutch gas policy has been put into question again: actors and interaction dynamics, stakes, present policy and perspectives

  19. Invalidating stagnation theory for family owned businesses : comparing family-to-family and third party ownership transfers

    NARCIS (Netherlands)

    Alija Ibrahimovic; Lex van Teeffelen; Roger Heaver

    2015-01-01

    Miller, Le Breton-Miller and Scholnick (2008) summarize and discuss two major perspectives constructed from the literature on family owned businesses (FOBs): stewardship and stagnation theory. In this paper the stagnation theory is being put to the test on Dutch small/medium enterprises (SMEs).

  20. Question Answering for Dutch : Simple does it

    NARCIS (Netherlands)

    Hoekstra, A.H.; Hiemstra, Djoerd; van der Vet, P.E.; Huibers, Theo W.C.; Schobbens, Pierre-Yves; Vanhoof, Wim; Schwanen, Gabriel

    2006-01-01

    When people pose questions in natural language to search for information on the web, the role of question answering (QA) systems becomes important. In this paper the QAsystem simpleQA, capable of answering Dutch questions on which the answer is a person or a location, is described. The system's

  1. The Dutch Open Telescope: History, Status, Prospects

    NARCIS (Netherlands)

    Rutten, R.J.

    1999-01-01

    After many years of persistent telescope design and telescope construction, R.H. Hammerschlag has installed his Dutch Open Telescope (DOT) on La Palma. I brie y review its history and design. The future of optical solar physics at Utrecht hinges on a recently-funded three- year DOT science

  2. Proxy magnetometry with the Dutch Open Telescope

    NARCIS (Netherlands)

    Rutten, R.J.; Hammerschlag, R.H.; Sütterlin, P.; Bettonvil, F.C.M.

    1999-01-01

    Superb movies from the Dutch Open Telescope (DOT) on La Palma have proven the validity of the open concept of this innovative telescope for high-resolution imaging of the solar atmosphere. A five- camera speckle-burst registration system is being installed that should permit consistent and

  3. The Dutch vocational education and training system

    NARCIS (Netherlands)

    Verhagen, A.M.C.

    2014-01-01

    The Dutch educational system is highly stratified from secondary education onwards3, and this also applies to MBO. Each MBO course can be followed in two different learning pathways, called the vocationally educating learning pathway (beroepsopleidende leerweg: BOL) and the vocationally guiding

  4. Cancer incidence in Dutch Balkan veterans

    NARCIS (Netherlands)

    Bogers, R.P.; Leeuwen, F.E. van; Grievink, L.; Schouten, L.J.; Kiemeney, L.A.L.M.; Schram-Bijkerk, D.

    2013-01-01

    Suspicion has been raised about an increased cancer risk among Balkan veterans because of alleged exposure to depleted uranium. The authors conducted a historical cohort study to examine cancer incidence among Dutch Balkan veterans. Male military personnel (n=18,175, median follow-up 11 years) of

  5. Bruxism and associated factors among Dutch adolescents

    NARCIS (Netherlands)

    van Selms, M.K.A.; Visscher, C.M.; Naeije, M.; Lobbezoo, F.

    2013-01-01

    Objectives To assess the prevalence rates of self-reported sleep bruxism and awake bruxism and their associations with several demographical, exogenous, and psychosocial factors among Dutch adolescents. Methods In a cross-sectional questionnaire survey, 4285 questionnaires were completed, with an

  6. Delinquent Behavior of Dutch Rural Adolescents

    NARCIS (Netherlands)

    Weenink, D.

    2011-01-01

    This article compares Dutch rural and non-rural adolescents’ delinquent behavior and examines two social correlates of rural delinquency: communal social control and traditional rural culture. The analyses are based on cross-sectional data, containing 3,797 participants aged 13–18 (48.7% females).

  7. Changing concerns of beginning Dutch university teachers

    NARCIS (Netherlands)

    van den Bos, Paul

    2017-01-01

    Purpose: The purpose of this paper is to address the concerns of beginning university teachers about learning to teach in the context of an induction programme. Design/methodology/approach: The author asked 162 beginning teachers at Dutch universities of applied sciences to express their concerns

  8. Dutch diabetes prevalence estimates (DUDE-1)

    NARCIS (Netherlands)

    Kleefstra, Nanne; Landman, Gijsw. D.; Van Hateren, Kornelis J. J.; Meulepas, Marianne; Romeijnders, Arnold; Rutten, Guy E. H.; Klomp, Maarten; Houweling, Sebastiaan T.; Bilo, Henk J. G.

    Background: Recent decades have seen a constant upward projection in the prevalence of diabetes. Attempts to estimate diabetes prevalence rates based on relatively small population samples quite often result in underestimation. The aim of the present study was to investigate whether the Dutch

  9. Ethical motivation of Dutch egg consumers

    NARCIS (Netherlands)

    Burrell, A.M.; Vrieze, G.

    2003-01-01

    This paper analyses a representative sample of 356 Dutch egg consumers in order to measure the extent to which ethical principles concerning the welfare of hens in different rearing systems are translated into egg purchasing intentions, and the extent to which declared intentions are expressed in

  10. Dutch diabetes prevalence estimates (DUDE-1)

    NARCIS (Netherlands)

    Kleefstra, Nanne; Landman, Gijsw. D.; Van Hateren, Kornelis J. J.; Meulepas, Marianne; Romeijnders, Arnold; Rutten, Guy E. H.; Klomp, Maarten; Houweling, Sebastiaan T.; Bilo, Henk J. G.

    2016-01-01

    Background: Recent decades have seen a constant upward projection in the prevalence of diabetes. Attempts to estimate diabetes prevalence rates based on relatively small population samples quite often result in underestimation. The aim of the present study was to investigate whether the Dutch

  11. Economic assessment of Dutch agricultural research

    NARCIS (Netherlands)

    Poppe, K.J.

    2008-01-01

    To cope with globalisation and other changes in the industry, in the last 20 years the Dutch Ministry of Agriculture has reorganised its system for education, extension and research considerably. It moved away from the concept of a linear process of innovation to that of an agro-innovation system.

  12. First Dutch Process Control Security Event

    NARCIS (Netherlands)

    Luiijf, H.A.M.

    2008-01-01

    On May 21st , 2008, the Dutch National Infrastructure against Cyber Crime (NICC) organised their first Process Control Security Event. Mrs. Annemarie Zielstra, the NICC programme manager, opened the event. She welcomed the over 100 representatives of key industry sectors. “Earlier studies in the

  13. 200 years of Dutch transport policy

    NARCIS (Netherlands)

    Annema, J.A.; De Wee, G.P.

    2009-01-01

    In this paper eight stories are told about Dutch passenger transport history. The first story is from 1814 about building the first national paved road network suitable for stagecoaches; the last is from 1997 and is about the decision to construct a high speed rail link from Amsterdam to the

  14. Dutch Journalism in the Digital Age

    NARCIS (Netherlands)

    M.J. Kemman (Max); M. Kleppe (Martijn); B. Nieman; H.J.G. Beunders (Henri)

    2013-01-01

    textabstractWith an ever-growing supply of online sources, information to produce news stories seems to be one mouse click away. But in what way do Dutch journalists actually use computer-aided research tools? This article provides an inventory of the ways journalists use digital (re)sources and

  15. Performance management in Dutch central government

    NARCIS (Netherlands)

    Mol, Nico P.; de Kruijf, J.A.M.

    2004-01-01

    This article investigates how and to what extent performance indicators in Dutch central government are actually embedded in performance management. In a case study encompassing 12 government organizations, the relevance of the indicators presented is analysed in three stages: (1) with respect to

  16. Governmental filtering of websites: The Dutch case

    NARCIS (Netherlands)

    Stol, W.PH.; Kaspersen, H.W.K.; Kerstens, J.; Leukfeldt, E.R.; Lodder, A.R.

    2009-01-01

    Following the example of Norway and other European Countries, such as Sweden and Denmark, in April 2007 the Dutch government started filtering and blocking web pages with child pornographic content. In this paper we present a research into the technological, legal and practical possibilities of this

  17. Ageing, funded pensions and the Dutch economy

    NARCIS (Netherlands)

    Bovenberg, A.L.; Knaap, T.; Fenge, R.; de Menil, G.; Pestieau, P.

    2008-01-01

    This chapter presents a discussion that seeks to identify through which different channels aging and pensions affect the Dutch economy, how these effects propagate, what the feedback effects are, and how the income distribution across and within generations is affected. It also seeks to quantify

  18. Introducing gaming simulation in the Dutch railways

    NARCIS (Netherlands)

    Meijer, S.

    2012-01-01

    Innovation in the Dutch railways in the domain of capacity management and traffic control is increasingly difficult to implement because of the large interconnectedness of all processes and separation into different institutions and organizations. Meanwhile there is a push for quality improvements

  19. Collectivism versus individualism in Dutch employment relations

    NARCIS (Netherlands)

    de Leede, Jan; Looise, Jan C.; van Riemsdijk, Maarten

    2004-01-01

    From a very centralistic and collectivistic tradition after World War Two, Dutch employment relations now show a trend towards radical decentralisation and individualisation. What might be the consequences of this trend for labour relations? Do developments still fit within a movement towards

  20. Consumer price sensitivity in Dutch health insurance

    NARCIS (Netherlands)

    M. van Dijk (Machiel); M. Pomp (Marc); R.C.H.M. Douven (Rudy); T. Laske-Aldershof (Trea); F.T. Schut (Erik); W. de Boer (Willem); A. Boo (Anne)

    2008-01-01

    textabstractAim: To estimate the price sensitivity of consumer choice of health insurance firm. Method: Using paneldata of the flows of insured betweenpairs of Dutch sickness funds during the period 1993-2002, we estimate the sensitivity of these flows to differences in insurance premium. Results:

  1. Processing subject-object ambiguities in Dutch

    NARCIS (Netherlands)

    Kaan, Edith

    1997-01-01

    Various clause types in Dutch and German are at least temporarily ambiguous with respect to the order of subject and object. A number of previous studies regarding the processing of such subject-object ambiguities have reported a preference for a subject-object interpretation. This order preference

  2. Becoming Digital: A Dutch Heritage Perspective

    NARCIS (Netherlands)

    Navarrete, T.

    2014-01-01

    Digitization of collections in Dutch museums can be traced back to 1969. Since then, significant resources have been allocated to digital activities. Results from the digitization trajectory analysis show that museums present a similar rate of adoption (and dissemination) for computers, the

  3. Infrastructure protection in the Dutch financial sector

    NARCIS (Netherlands)

    van Oers, M.H.M.; Strous, L.; Berndsen, R.J.; Butts, J.; Shenoi, S.

    2012-01-01

    This paper presents a case study of critical infrastructure protection in the Dutch financial sector. The organizational structures are examined to discern the roles and functions that facilitate public-private cooperation. An assessment of the organizational structures is provided along with a

  4. Determining authority of Dutch case law

    NARCIS (Netherlands)

    Winkels, R.; de Ruyter, J.; Kroese, H.

    2011-01-01

    In this paper we present the results of two studies to see whether the analysis of the network of citations between cases can be used as an indication of the relevance and authority in the Dutch legal system. Fowler e.a. have shown such results for the US common law system, but given the different

  5. Balance in competition in Dutch soccer

    NARCIS (Netherlands)

    Koning, Ruud H.

    2000-01-01

    In this paper, we estimate an ordered probit model for soccer results in The Netherlands. The result of a game is assumed to be determined by home advantage and quality differences of the opposing teams. The parameters of the model are used to assess whether competitive balance in Dutch professional

  6. Competition in the Dutch consumer credit market

    NARCIS (Netherlands)

    Toolsema-Veldman, Linda

    2002-01-01

    This paper considers the degree of competitiveness of the Dutch consumer credit market. We use the well-known Bresnahan-Lau method that estimates a structural model consisting of a demand relation and a supply relation, based on aggregate data. The level of competition is derived from the estimated

  7. Competition in the Dutch consumer credit market

    NARCIS (Netherlands)

    Toolsema-Veldman, Linda

    2001-01-01

    This paper considers the degree of competitiveness of the Dutch consumer credit market. We use the well-known I Bresnahan-Lau method that estimates it structural model consisting of a demand relation and a supply relation, based on aggregate data. The level of compelition is derived from the

  8. The Dutch economy and the environment.

    NARCIS (Netherlands)

    Ierland, van E.C.

    1986-01-01

    On initiative of the Dutch Parliament a scenario study took place on economics, energy and the environment, to analyse the need of building additional nuclear power plants in the Netherlands in the coming decades. Characteristic for the study is the application of empirical models that describe the

  9. The Dutch Experience with Weighted Student Funding

    Science.gov (United States)

    Fiske, Edward B.; Ladd, Helen F.

    2010-01-01

    Weighted student funding (WSF) is used in several U.S. cities as a method for providing more funds to schools with high concentrations of disadvantaged students. The practice has been used successfully in the Netherlands since 1985. Several factors make the success of the Dutch system unlikely to transfer to the United States, including the Dutch…

  10. Dutch elm disease control: performance and costs

    Science.gov (United States)

    William N., Jr. Cannon; David P. Worley

    1980-01-01

    Municipal programs to suppress Dutch elm disease have had highly variable results. Performance as measured by tree mortality was unrelated to control strategies. Costs for control programs were 37 to 76 percent less than costs without control programs in the 15-year time-span of the study. Only those municipalities that conducted a high-performance program could be...

  11. Quality systems in Dutch health care institutions.

    NARCIS (Netherlands)

    Casparie, A.F.; Sluijs, E.M.; Wagner, C.; Bakker, D.H. de

    1997-01-01

    The implementation of quality systems in Dutch health care was supervised by a national committee during 1990-1995. To monitor the progress of implementation a large survey was conducted in the beginning of 1995. The survey enclosed all subsectors in health care. A postal questionnaire-derived

  12. Institutional developments in the Dutch waste market

    NARCIS (Netherlands)

    Gradus, R.H.J.M.; Dijkgraaf, E.

    2008-01-01

    We analyse the Dutch waste-collection market for 1998-2005. We show that municipalities with unit-based pricing (UBP) or waste collection by private companies have lower costs. However, this cost advantage decreases over time as competition decreases, because of the introduction of the

  13. Direct speech constructions in aphasic Dutch narratives

    NARCIS (Netherlands)

    Groenewold, Rimke; Bastiaanse, Roelien; Huiskes, Mike

    2013-01-01

    Background: Previous studies have shown that individuals with aphasia are usually able to produce direct reported speech constructions. So far these studies have mainly been conducted in English. The results show that direct speech is beneficial for aphasic speakers for various reasons. In Dutch the

  14. Employment protection in Dutch collective labour agreements

    NARCIS (Netherlands)

    Schils, T.

    2007-01-01

    The Dutch system of employment protection is often perceived as too strict for workers on permanent contracts, because of the large procedural inconveniences, difficulty of dismissal and high severance pay. It is suggested that the system of employment protection lowers labour market flexibility by

  15. Performance Persistence of Dutch Pension Funds

    NARCIS (Netherlands)

    Huang, Xiaohong; Mahieu, R.

    2012-01-01

    This paper studies the investment performance of pension funds with a focus on their ability in implementing their intended investment strategy. We use a sample of Dutch industry-wide pension funds, which are obliged by law to report their investment performance according to the so-called z-score.

  16. Performance Persistence of Dutch Pension Funds

    NARCIS (Netherlands)

    Huang, Xiaohong; Mahieu, Ronald

    2010-01-01

    This paper studies the investment performance of pension funds with a focus on their ability in implementing the investment strategy. We use a sample of Dutch industry-wide pension funds, which are obliged by law to report their investment performance according to the so-called z-score. The z-score

  17. The Essential Guide to Dutch Music

    NARCIS (Netherlands)

    Klis, van der Jolande

    2000-01-01

    Of all the composers produced by the Netherlands, only two have achieved world renown: Jan Pieterszoon Sweelinck in the early seventeenth century and Louis Andriessen today. Nonetheless, so much more Dutch music is worthy of attention. For the first time ever, four centuries of composition in the

  18. Crisis, Inequality and Consumption - a Dutch Perspective

    NARCIS (Netherlands)

    Wahlen, S.

    2016-01-01

    Inequalities have been exacerbating in the Netherlands since the economic crisis hit ground in 2008, with poverty increasing substantially. The amount of the Dutch population living under the poverty line increased from 7,4 % in 2010 to 10,3 % in 2013. Different types of household are affected:

  19. Creating sport consumers in Dutch sport policy

    NARCIS (Netherlands)

    van der Roest, Jan Willem; Vermeulen, Jeroen; van Bottenburg, Maarten; LS Sportontw. & Managing Social Issues; UU LEG Research USG Public Matters Managing Social Issues; LS Management van Cultuur en Zingeving

    2014-01-01

    This article deals with the tension between the association logic and the market logic that appears in the domain of voluntary sport clubs (VSCs). We present a qualitative analysis of sport policy texts of fifteen Dutch national sport organizations (NSOs) and the national umbrella organization to

  20. Rostering at a Dutch Security Firm

    NARCIS (Netherlands)

    R. Freling (Richard); N. Piersma (Nanda); A.P.M. Wagelmans (Albert); A. van de Wetering

    2001-01-01

    textabstractThe roster planning process at the Dutch security firm NVD was traditionally carried out by hand. A few years NVD was traditionally carried out by hand. A few years ago, because of changing labor laws in the Netherlands, this became practically impossible. We developed a decision support

  1. Rostering at a Dutch Security Firm

    NARCIS (Netherlands)

    R. Freling (Richard); N. Piersma (Nanda); A.P.M. Wagelmans (Albert); A. van de Wetering

    2001-01-01

    textabstractThe roster planning process at the Dutch security firm NVD was traditionally carried out by hand. A few years ago, because of changing labor laws in The Netherlands, this became practically impossible. We developed a decision support system which has four main modules. The first one

  2. Regional differences in Dutch maternal mortality.

    NARCIS (Netherlands)

    Graaf, J. de; Schutte, J.; Poeran, J.; Roosmalen, J. van; Bonsel, G.; Steegers, E.

    2012-01-01

    Please cite this paper as: de Graaf J, Schutte J, Poeran J, van Roosmalen J, Bonsel G, Steegers E. Regional differences in Dutch maternal mortality. BJOG 2012;119:582-588. Objective To study regional differences in maternal mortality in the Netherlands. Design Confidential inquiry into the causes of

  3. Topics in Corpus-Based Dutch Syntax

    NARCIS (Netherlands)

    Beek, Leonoor Johanneke van der

    2005-01-01

    In this dissertation, corpus data is applied in various kinds of linguistic analyses. The data serves as a source of examples and counterexamples in a theoretical linguistic analysis of the Dutch cleft construction, as the source of quantitative data in a probabilistic account of the dative

  4. Competition in the Dutch Health Care Sector

    NARCIS (Netherlands)

    F.T. Schut (Erik)

    1995-01-01

    textabstractFor more than two decades, Dutch health policy has been marked by a search for a suitable market order in health care. Suitable in the sense of maintaining universal access, containing the growth of health care expenditure and improving the technical and allocative efficiency of

  5. Crossing the chasm in Dutch greenhouse horticulture

    NARCIS (Netherlands)

    Buurma, J.S.; Smit, P.X.

    2016-01-01

    Dutch greenhouse horticulture has an innovation and development programme called 'Kas als Energiebron' (Greenhouse as Energy Producer). The objective of this programme is reducing the carbon footprint and improving the energy efficiency of greenhouse horticulture, and developing a climate neutral

  6. Workshops in Dutch sustainable building design practice

    NARCIS (Netherlands)

    Zeiler, W.; Quanjel, E.M.C.J.; Borsboom, W.A.

    2009-01-01

    Within the present context of the Dutch Sustainable Building Practice it is hard for the different involved building design disciplines to give a good answer to sustainability. Especially this is the case for the application solar energy either in passive or active form. As traditional methods did

  7. Flexible benefit plans in Dutch organisations

    NARCIS (Netherlands)

    Hillebrink, C.

    2006-01-01

    Flexible benefit plans give employees a greater say over the composition of their benefits than traditional Dutch benefit plans. These arrangements developed in a time of further individualisation, increasing flexibility in the workplace, and a tight labour market in the Netherlands. By giving

  8. The financing behavior of Dutch firms

    NARCIS (Netherlands)

    Chen, Linda H.; Jiang, George J.

    2001-01-01

    This paper investigates the financing behaviour of Dutch firms by testing whether a firm’s financing decisions are determined by certain factors identified in various theories. Since a firm’s financing decision is reflected in the changes of its leverage, our research focuses on the relationship

  9. Partial word order freezing in Dutch

    NARCIS (Netherlands)

    Bouma, G.J.; Hendriks, P.

    2012-01-01

    Dutch allows for variation as to whether the first position in the sentence is occupied by the subject or by some other constituent, such as the direct object. In particular situations, however, this commonly observed variation in word order is ‘frozen’ and only the subject appears in first

  10. Strategy disclosure in Dutch annual reports

    NARCIS (Netherlands)

    Santema, S.C.; Rijt, van de J.

    2001-01-01

    Much attention is being paid to the financial part of the annual reports of companies. Not much research has been done into the quality of the Report of the Executive Board in annual reports (the narrative part). During the past 2 years Dutch firms were reviewed on the way the Executive Board dealt

  11. Performance of Dutch children on the Bayley III: a comparison study of US and Dutch norms.

    Science.gov (United States)

    Steenis, Leonie J P; Verhoeven, Marjolein; Hessen, Dave J; van Baar, Anneloes L

    2015-01-01

    The Bayley Scales of Infant and Toddler Development-third edition (Bayley-III) are frequently used to assess early child development worldwide. However, the original standardization only included US children, and it is still unclear whether or not these norms are adequate for use in other populations. Recently, norms for the Dutch version of the Bayley-III (The Bayley-III-NL) were made. Scores based on Dutch and US norms were compared to study the need for population-specific norms. Scaled scores based on Dutch and US norms were compared for 1912 children between 14 days and 42 months 14 days. Next, the proportions of children scoring Dutch norms fluctuated around values based on US norms on all subtests. The extent of the deviations differed across ages and subtests. Differences in means were significant across all five subtests (p Dutch norms resulted in over-referral regarding gross motor skills, and under-referral regarding cognitive, receptive communication, expressive communication, and fine motor skills. The Dutch norms differ from the US norms for all subtests and these differences are clinically relevant. Population specific norms are needed to identify children with low scores for referral and intervention, and to facilitate international comparisons of population data.

  12. Dutch hi-tech companies exhibit at CERN

    CERN Multimedia

    Roberto Cantoni

    2010-01-01

    Twenty-seven Dutch companies will present the state of the art of their technological developments at the industrial exhibition Holland @ CERN from 8 to 11 November. The exhibition is designed to help strengthen the ties between fundamental science and Dutch industry.   The exhibition, supported by the Dutch Ministry of Economic Affairs and organised by the Netherlands National Institute for Subatomic Physics (Nikhef), in cooperation with the Foundation for Fundamental Research on Matter (FOM), the FOM Institute for Plasma Physics Rijnhuizen, and Dutch Scientific, an association of manufacturers of scientific equipment, will be held in the Main Building from 8 to11 November. “The last Holland @ CERN exhibition took place fifteen years ago”, says Robert Klöpping from Nikhef, Dutch Industrial Liaison Officer for CERN and Purchasing Advisor for Grenoble ESRF. “This kind of event is very important for Dutch industry as it allows us to show what Dutch companies c...

  13. Staging and Functional Characterization of Pheochromocytoma and Paraganglioma by 18F-Fluorodeoxyglucose (18F-FDG) Positron Emission Tomography

    Science.gov (United States)

    Timmers, Henri J. L. M.; Chen, Clara C.; Carrasquillo, Jorge A.; Whatley, Millie; Ling, Alexander; Eisenhofer, Graeme; King, Kathryn S.; Rao, Jyotsna U.; Wesley, Robert A.; Adams, Karen T.

    2012-01-01

    Background Pheochromocytomas and paragangliomas (PPGLs) are rare tumors of the adrenal medulla and extra-adrenal sympathetic chromaffin tissues; their anatomical and functional imaging are critical to guiding treatment decisions. This study aimed to compare the sensitivity and specificity of 18F-fluorodeoxyglucose positron emission tomography with computed tomography (18F-FDG PET/CT) for tumor localization and staging of PPGLs with that of conventional imaging by [123I]-metaiodobenzylguanidine single photon emission CT (123I-MIBG SPECT), CT, and magnetic resonance imaging (MRI). Methods A total of 216 patients (106 men, 110 women, aged 45.2 ± 14.9 years) with suspected PPGL underwent CT or MRI, 18F-FDG PET/CT, and 123I-MIBG SPECT/CT. Sensitivity and specificity were measured as endpoints and compared by the McNemar test, using two-sided P values only. Results Sixty (28%) of patients had nonmetastatic PPGL, 95 (44%) had metastatic PPGL, and 61 (28%) were PPGL negative. For nonmetastatic tumors, the sensitivity of 18F-FDG was similar to that of 123I-MIBG but less than that of CT/MRI (sensitivity of 18F-FDG = 76.8%; of 123I-MIBG = 75.0%; of CT/MRI = 95.7%; 18F-FDG vs 123I-MIBG: difference = 1.8%, 95% confidence interval [CI] = −14.8% to 14.8%, P = .210; 18F-FDG vs CT/MRI: difference = 18.9%, 95% CI = 9.4% to 28.3%, P < .001). The specificity was 90.2% for 18F-FDG, 91.8% for 123I-MIBG, and 90.2% for CT/MRI. 18F-FDG uptake was higher in succinate dehydrogenase complex– and von Hippel–Lindau syndrome–related tumors than in multiple endocrine neoplasia type 2 (MEN2) related tumors. For metastases, sensitivity was greater for 18F-FDG and CT/MRI than for 123I-MIBG (sensitivity of 18F-FDG = 82.5%; of 123I-MIBG = 50.0%; of CT/MRI = 74.4%; 18F-FDG vs 123I-MIBG: difference = 32.5%, 95% CI = 22.3% to 42.5%, P < .001; CT/MRI vs 123I-MIBG: difference = 24.4%, 95% CI = 11.3% to 31.6%, P < .001). For bone metastases, 18F-FDG was more sensitive than CT/MRI (sensitivity of 18

  14. Child sexual abuse and family outcomes

    NARCIS (Netherlands)

    de Jong, Rinke; Bijleveld, C.C.J.H.

    2015-01-01

    This study aimed to examine the association between different characteristics of sexual abuse and adverse family outcomes in later life. Through archived court files, a large sample of Dutch men and women who have been sexually abused as a child could be identified. Outcome variables were assessed

  15. Clinical and genetic aspects of Marfan syndrome and familial thoracic aortic aneurysms and dissections

    NARCIS (Netherlands)

    Hilhorst-Hofstee, Yvonne

    2013-01-01

    This thesis concerns the clinical and genetic aspects of familial thoracic aortic aneurysms and dissections, in particular in Marfan syndrome. It includes the Dutch multidisciplinary guidelines for diagnosis and management of Marfan syndrome. These guidelines contain practical directions for

  16. Primary prevention of cardiovascular diseases: a cost study in family practices.

    NARCIS (Netherlands)

    Bekker-Grob, E.W. de; Dulmen, S. van; Berg, M. van den; Verheij, R.A.; Slobbe, L.C.J.

    2011-01-01

    BACKGROUND: Considering the scarcity of health care resources and the high costs associated with cardiovascular diseases, we investigated the spending on cardiovascular primary preventive activities and the prescribing behaviour of primary preventive cardiovascular medication (PPCM) in Dutch family

  17. The second Dutch national survey on radon in dwellings. Set-up of the project

    International Nuclear Information System (INIS)

    Hiemstra, Y.; Stoop, P.; Lembrechts, J.

    1997-03-01

    In 1994 the Dutch Ministry of Housing, Spatial Planning and the Environment (VROM) commissioned RIVM to carry out a national survey on radon in dwellings, the set-up of which is described in this report. The goals of the survey were: (a) to determine the average and the range of the radon (222Rn) concentration in Dutch dwellings built since the previous survey in 1984, and (b) to quantify the relative contribution of radon from the building materials and from the soil to the indoor radon level in dwellings built in the course of the last decade. These data will be used for the selection of possible countermeasures and as a reference for evaluating the effectiveness of final regulations on radon. Two separate random samples from the Dutch housing stock were taken to answer these questions. A sample of about 1000 dwellings built between 1985 and 1993 in 52 municipalities was taken to answer the first question. The composition of the sample differs from the Dutch housing stock for a number of characteristics, the most obvious of which is the over-representation of single-family and private homes. A sample of about 450 single-family dwellings built in 14 municipalities on different soil types was taken to answer the second question. Two groups of municipalities were distinguished because of geographic differences in building practices: those where town houses predominate and those with mainly detached and semi-detached houses. Information on radon and its sources was collected through placement of alpha track detectors, installation of so-called PFT sources and passive samplers for air infiltration measurement, collection of soil samples and completion of a questionnaire on building and ventilation characteristics. The results of the study are dealt with in RIVM report no. 610058006. 8 figs., 9 tabs., 31 refs. 4 appendices

  18. Pheochromocytoma and Paraganglioma

    Science.gov (United States)

    ... Español NICHD Theme Browse AZTopics Browse A-Z Adrenal Gland Disorders Autism Spectrum Disorder (ASD) Down Syndrome Endometriosis Learning ... Funding Opportunities & Notices Health A to Z List Adrenal Gland Disorders About NICHD Research Information Find a Study More ...

  19. Reforming Dutch substance abuse treatment services.

    Science.gov (United States)

    Schippers, Gerard M; Schramade, Mark; Walburg, Jan A

    2002-01-01

    The Dutch substance abuse treatment system is in the middle of a major reorganization. The goal is to improve outcomes by redesigning all major primary treatment processes and by implementing a system of regular monitoring and feedback of clinical outcome data. The new program includes implementing standardized psychosocial behavior-oriented treatment modalities and a stepped-care patient placement algorithm in a core-shell organizational model. This article outlines the new program and presents its objectives, developmental stages, and current status.

  20. Investing in Climate Change. Dutch Banks Compared

    International Nuclear Information System (INIS)

    Buurgaard Nielsen, J.; Pols, D.; Van Gelder, J.W.; Denie, S.; Scheire, C.

    2007-06-01

    This report is the first comparison of the climate change performance of Dutch banks that analyses the actual investments of these banks. It reviews a substantial share of each bank's portfolio, according to three criteria: how much they invest in the main cause of climate change, fossil fuels; how much they invest in the main alternative to fossil fuels, renewable energy; the bank's plans and policies to limit their contribution to climate change

  1. Dutch industry prepares for the future

    International Nuclear Information System (INIS)

    Anon.

    1978-01-01

    In a review of the Dutch nuclear industry descriptions are given of the contributions of the following: Rotterdam Dockyard Company (RDM), manufacturers of nuclear pressure vessels; Rheine-Schelde-Verolme and Comprimo BV who co-operate in the field of nuclear engineering and turnkey power plants; Neeratom, which leads the industry in the SNR fast reactor project; and Royal Schelde an engineering company with many activities in the nuclear engineering field, and particularly in welding technology. (UK)

  2. Price competition among Dutch sickness funds

    OpenAIRE

    Varkevisser, Marco; Geest, Stéphanie

    2003-01-01

    textabstractIn general, competition enhances efficiency. On the market for health insurance free market competition, however, has unwanted side-effects. The existence of asymmetrical information can lead to adverse selection and cream skimming. Adequate risk-adjustment removes the incentives for cream skimming and balances the negative consequences of adverse selection. In an attempt to enhance efficiency, the Dutch government in 1992 introduced price competition between social health insurer...

  3. Flexible benefit plans in Dutch organisations

    OpenAIRE

    Hillebrink, C.

    2006-01-01

    Flexible benefit plans give employees a greater say over the composition of their benefits than traditional Dutch benefit plans. These arrangements developed in a time of further individualisation, increasing flexibility in the workplace, and a tight labour market in the Netherlands. By giving employees a choice in the way they are paid, employers hoped to become more attractive employers, and lend a helping hand to employees who were combining work and care. In this study, flexible benefit p...

  4. Dutch chemical producers pledge emissions cuts

    International Nuclear Information System (INIS)

    Chynoweth, E.; Schoenmakers, J.

    1993-01-01

    Dutch chemical producers have negotiated a long-term agreement with government ministries to reduce emissions of a wide range of chemicals. Industry association Vereniging van de Nederlandse Chemische Industrie (VNCI; Leidschendam) says implementing the commitment will cost companies Dfl 10 billion ($5.4 billion) between 1993 and 2000. VNCI technical director Wim Quik welcomes the accord, which he describes as a management contract, saying, Rather than have legislation, there is a certain adjustment available. Peter Santen, managing director of midsized chemicals player Cindu Chemicals (Uithoorn, the Netherlands) voices some concern about the details of the accord, but adds, we are flexible in trying to agree with the contents of the covenant [it] is better than having new rules from law. The Dutch government, traditionally eager for consensus, has struck a number of such deals with Dutch industries - including packaging, metal, and tire - to reduce emissions and set up environmental management programs. The effort is based on the government's National Environmental Policy Plans - NMP and NMP Plus. Targets for emissions reduction by the chemical industry were provided by a government-funded environmental research institute

  5. Performance of Dutch children on the Bayley III: a comparison study of US and Dutch norms.

    Directory of Open Access Journals (Sweden)

    Leonie J P Steenis

    Full Text Available The Bayley Scales of Infant and Toddler Development-third edition (Bayley-III are frequently used to assess early child development worldwide. However, the original standardization only included US children, and it is still unclear whether or not these norms are adequate for use in other populations. Recently, norms for the Dutch version of the Bayley-III (The Bayley-III-NL were made. Scores based on Dutch and US norms were compared to study the need for population-specific norms.Scaled scores based on Dutch and US norms were compared for 1912 children between 14 days and 42 months 14 days. Next, the proportions of children scoring < 1-SD and < -2 SD based on the two norms were compared, to identify over- or under-referral for developmental delay resulting from non-population-based norms.Scaled scores based on Dutch norms fluctuated around values based on US norms on all subtests. The extent of the deviations differed across ages and subtests. Differences in means were significant across all five subtests (p < .01 with small to large effect sizes (ηp2 ranging from .03 to .26. Using the US instead of Dutch norms resulted in over-referral regarding gross motor skills, and under-referral regarding cognitive, receptive communication, expressive communication, and fine motor skills.The Dutch norms differ from the US norms for all subtests and these differences are clinically relevant. Population specific norms are needed to identify children with low scores for referral and intervention, and to facilitate international comparisons of population data.

  6. Tradeable emission permits in Dutch environmental policy. A utopia?

    International Nuclear Information System (INIS)

    Schuurman, S.J.

    1997-01-01

    Because of the lack of experience with permits in the Netherlands, and in view of the similarities between various other tradeable permit systems, the functioning of Dutch systems of tradeable fish, milk and manure quotas is discussed. Evaluation of these systems is based on criteria of effectiveness, target-group efficiency and government efficiency. These systems of tradeable permits appear to constitute a successful addition to the Dutch policy of direct regulation. Considering this, and the favorable American experience with the Emissions Trading Program, tradeable emission permits deserve a chance to be implemented in Dutch environmental policy. The question remains, however, whether the Dutch government is ready for such a step. 28 refs

  7. Outcomes of a coaching program for families with multiple problems in the Netherlands : A prospective study

    NARCIS (Netherlands)

    Tausendfreund, Tim; Knot-Dickscheit, Jana; Post, Wendy J.; Knorth, Erik J.; Grietens, Hans

    2014-01-01

    Families who face a multitude of severe and persistent problems in a number of different areas of life are commonly referred to as multi-problem families in Dutch child welfare. Although evidence suggests that short-term crisis interventions can have positive effects in these families, they have up

  8. Social relations model analyses of perceived self-control and trust in families

    NARCIS (Netherlands)

    Büyükcan Tetik, A.; Finkenauer, C.; Siersema, M.; Vander Heyden, K.; Krabbendam, L.

    2015-01-01

    How do people know which family member is trustworthy? In this study, the authors tested the hypothesis that people use their perception of a family member's self-control as an indicator of his or her trustworthiness. Eighty-four Dutch families consisting of 2 parents and 2 children completed

  9. Balancing work and family obligations in Pakistan and the Netherlands : A comparative study

    NARCIS (Netherlands)

    Syed, S.; Arain, G.A.; Schalk, Rene; Freese, C.

    2015-01-01

    A study of 486 banking employees in Pakistan and the Netherlands investigated the effect of work-family conflict on psychological contract breach of work-family balance obligations. The results showed that 127 Dutch respondents were more likely to experience work interference with family than

  10. Dyslexia and early intervention: what did we learn from the Dutch Dyslexia Programme?

    Science.gov (United States)

    van der Leij, Aryan

    2013-11-01

    Part of the Dutch Dyslexia Programme has been dedicated to early intervention. The question of whether the genetically affected learning mechanism of children who are at familial risk (FR) of developing dyslexia could be influenced by training phoneme awareness and letter-sound associations in the prereading phase was investigated. The rationale was that intervention studies reveal insights about the weaknesses of the learning mechanisms of FR children. In addition, the studies aimed to gather practical insights to be used in the development of a system of early diagnosis and prevention. Focused on the last period of kindergarten before formal reading instruction starts in Grade 1, intervention methods with comparable samples and designs but differences in delivery mode (use of computer or manual), tutor (semi-professional or parent), location (at school or at home), and additional practices (serial rapid naming or simple word reading) have been executed to test the hypothesis that the incidence and degree of dyslexia can be reduced. The present position paper summarizes the Dutch Dyslexia Programme findings and relates them to findings of other studies. It is discussed that the Dutch studies provide evidence on why prevention of dyslexia is hard to accomplish. It is argued that effective intervention should not only start early but also be adapted to the individual and often long-lasting educational needs of children at risk of reading failure. Copyright © 2013 John Wiley & Sons, Ltd.

  11. Work, family, and happiness : essays on interdependencies within families, life events, and time allocation decisions

    OpenAIRE

    Pouwels, B.

    2011-01-01

    In this thesis we investigate how today’s work and family life influence people’s happiness – or the lack thereof. We contribute to the research agenda by focusing on three underexplored issues in the literature, namely i) interdependencies within families, ii) life events, and iii) time allocation decisions. Using data of the Dutch Time Competition Survey 2003 and the German Socio-Economic Panel 1984 – 2005 (GSOEP), this thesis shows that the happiness of partners in marital relationships is...

  12. Confirmation of dyslexia susceptibility loci on chromosomes 1p and 2p, but not 6p in a Dutch sib-pair collection.

    NARCIS (Netherlands)

    Kovel, C.G.F. de; Franke, B.; Hol, F.A.; Lebrec, J.J.; Maassen, B.A.M.; Brunner, H.G.; Padberg, G.W.A.M.; Platko, J.; Pauls, D.

    2008-01-01

    In this study, we attempted to confirm genetic linkage to developmental dyslexia and reading-related quantitative traits of loci that have been shown to be associated with dyslexia in previous studies. In our sample of 108 Dutch nuclear families, the categorical trait showed strongest linkage to

  13. Structural and diffusion effects in the Dutch fertility transition, 1870-1940

    Directory of Open Access Journals (Sweden)

    Hilde Bras

    2014-01-01

    Full Text Available Background: Ever since the Princeton European Fertility Project, structural and diffusion effects on fertility behavior have been juxtaposed. However, we still hardly know what the relative effects were of shifting socio-economic conditions and shifts in sociability in explaining the historical fertility decline. Objective: To what extent and how did structural and diffusion effects play a role in the adoption of fertility control in the Dutch historical fertility transition? Methods: A national data set was used with more than 3,000 maternity histories of married Dutch women aged 15-50, whose reproductive careers took place between 1870 and 1940. Apart from husbands' occupations, characteristics of the set of couples' marriage witnesses were included to measure their social networks. Cox regression analyses of age at last birth and negative binomial regressions of net family size were conducted. Results: Results indicate that unskilled laborers and farm laborers were laggards in the practice of fertility control during the Dutch fertility transition. Besides SES differentials, differences in couples' social networks were important in explaining fertility behavior. Those who had networks consisting of lateral kin, age peers, and people of urban background stopped childbearing earlier and had smaller families than other couples did. Particularly the presence of lateral kin of the bride and of female witnesses was strongly associated with smaller family size. Conclusions: The evidence lends support for so-called "blended diffusion models" and suggests that the fertility transition must be understood as much from the viewpoint of changed cost-benefit calculations related to structural changes, as from shifting patterns of sociability associated with the decline of patriarchy and the increasing lateralization and age homophily of people's social networks.

  14. Verb inflection in Monolingual Dutch and Sequential Bilingual Turkish-Dutch Children with and without SLI

    Science.gov (United States)

    Blom, Elma; De Jong, Jan; Orgassa, Antje; Baker, Anne; Weerman, Fred

    2013-01-01

    Both children with specific language impairment (SLI) and children who acquire a second language (L2) make errors with verb inflection. This overlap between SLI and L2 raises the question if verb inflection can discriminate between L2 children with and without SLI. In this study we addressed this question for Dutch. The secondary goal of the study…

  15. Sustainable Entrepreneurship in the Dutch Construction Industry

    Directory of Open Access Journals (Sweden)

    Rosalinde J.A. Klein Woolthuis

    2010-02-01

    Full Text Available This article discusses the strategies that sustainable entrepreneurs use to interact with their environment in the Dutch construction industry. The Dutch construction industry is under great pressure to move towards sustainability, and entrepreneurs are believed to be able to play a large role in this transition by introducing new products and new business practices. But how can entrepreneurs prosper in an environment that is not geared up towards such a change? And which strategies do they use to bend conditions in their favor? In this article we make use of the Market and System Failure Framework to analyze the pressures that entrepreneurs are confronted with when introducing sustainable innovations. We recognize that these pressures can be seen as bottlenecks, but that market and system failures can also create entrepreneurial opportunities. We interviewed 16 entrepreneurs in the Dutch construction industry to determine the influences they experienced from their environment and the strategies they use to deal with these. We conclude that we can distinguish between system building and following entrepreneurs, where the former aims to build a new system to challenge the old one, whereas the latter rather makes use of existing structures to build a business. We find that both strategies can be successful and that overall, the entrepreneurs confirm the belief that sustainability on people, planet and transparency aspects, can contribute very well to the long term profitability of the businesses the entrepreneurs are running. These in-depth insights into the influences from the context on the one hand, and the entrepreneurs’ strategic reactions on the other hand, contribute to our understanding of the interactions between entrepreneurs and the system context. This can help us to more effectively stimulate and support innovating entrepreneurs to contribute to the transition towards sustainability.

  16. A Dutch Nationwide Bariatric Quality Registry: DATO.

    Science.gov (United States)

    Poelemeijer, Youri Q M; Liem, Ronald S L; Nienhuijs, Simon W

    2017-12-22

    In the Netherlands, the number of bariatric procedures increased exponentially in the 90s. To ensure and improve the quality of bariatric surgery, the nationwide Dutch Audit for Treatment of Obesity (DATO) was established in 2014. The audit was coordinated by the Dutch Institute for Clinical Auditing (DICA). This article provides a review of the aforementioned process in establishing a nationwide registry in the Netherlands. In collaboration with the DATO's scientific committee and other stakeholders, an annual list of several external quality indicators was formulated. This list consists of volume, process, and outcome indicators. In addition to the annual external indicators, the database permits individual hospitals to analyze their own data. The dashboard provides several standardized reports and detailed quality indicators, which are updated on a weekly base. Since the start, all 18 Dutch bariatric centers participated in the nationwide audit. A total of 21,941 cases were registered between 2015 and 2016. By 2016, the required variables were registered in 94.3% of all cases. A severe complicated course was seen in 2.87%, and mortality in 0.05% in 2016. The first-year follow-up shows a > 20% TWL in 86.1% of the registered cases. The DATO has become rapidly a mature registry. The well-organized structure of the national audit institution DICA and governmental funding were essential. However, most important were the bariatric teams themselves. The authors believe reporting the results from the registry has already contributed to more knowledge and acceptance by other health care providers.

  17. The end of the Dutch car tax?

    International Nuclear Information System (INIS)

    Besseling, P.J.; Lebouille, R.A.J.

    2009-01-01

    In the framework of the project 'Paying differently for mobility' an option that is considered is to switch the Dutch Tax on Motor cars and Vehicles (BPM) to a higher tariff for the future road pricing system. Due to some special characteristics of the BPM, this will be a highly complex financial operation. In this article, we explore three variants for their effect on the vehicle market and on the budget. We also explain the advantages and disadvantages for several groups of car owners. [nl

  18. Major changes ahead for Dutch energy sector

    International Nuclear Information System (INIS)

    Kort, C.J.M.

    1996-01-01

    The Dutch energy distribution companies are again facing important adaptations to current market forces. The operational activities of the conventional energy distribution companies are split into monopoly tasks and commercial activities. By separation of functions and by further technological developments and market trends quite different types of companies will emerge, such as grid operators, energy-telecommunications-environment concerns and energy sellers. Some companies will focus on grid operation, while others will clearly opt for more commercial enterprising. Just like other private companies energy companies will develop new activities in other markets based on their own corporate strategies. Grid operation may then become only a mirror element or be abandoned altogether. 5 figs

  19. Seasonal variation in the Dutch bovine raw milk composition

    NARCIS (Netherlands)

    Heck, J.M.L.; Valenberg, van H.J.F.; Dijkstra, J.; Hooijdonk, van A.C.M.

    2009-01-01

    In this study, we determined the detailed composition of and seasonal variation in Dutch dairy milk. Raw milk samples representative of the complete Dutch milk supply were collected weekly from February 2005 until February 2006. Large seasonal variation exists in the concentrations of the main

  20. Learning to read Dutch as a transparent orthography

    NARCIS (Netherlands)

    Verhoeven, L.T.W.

    2017-01-01

    The present longitudinal study investigated the growth of word reading fluency in Dutch as a transparent orthography as a function of meaning (words vs pseudowords) and orthographic complexity throughout the elementary school years. For a representative sample of 511 Dutch children, decoding

  1. Ethnic differences in disability risk between Dutch and Turkish scaffolders

    NARCIS (Netherlands)

    Elders, L.A.M.; Burdorf, A.; Öry, F.G.

    2004-01-01

    The number of native Dutch and Turkish workers receiving a permanent disability pension in the Netherlands is still rising. To assess ethnic differences in disability risk between Dutch and Turkish scaffolders, a retrospective study was conducted within a large scaffolding company. Medical files for

  2. Ethnic differences in disability risk between Dutch and Turkish scaffolders

    NARCIS (Netherlands)

    A. Burdorf (Alex); F.G. Öry; L.A.M. Elders (Leo)

    2004-01-01

    textabstractThe number of native Dutch and Turkish workers receiving a permanent disability pension in the Netherlands is still rising. To assess ethnic differences in disability risk between Dutch and Turkish scaffolders, a retrospective study was conducted within a large

  3. CESAM: The CCSO annual model of the Dutch economy

    NARCIS (Netherlands)

    Jongbloed, Benjamin W.A.; Kuipers, S.K.; Kuper, G.H.; Sterken, E.

    1990-01-01

    This paper presents CESAM, a macroeconometric model of the Dutch economy based on annual data. CESAM can be characterized as a Keynesian expenditure model including a neoclassical production model and a post-Keynesian financial model. This characterization holds for most of the Dutch

  4. Socialized choices: Labour market behaviour of Dutch mothers

    NARCIS (Netherlands)

    Ruitenberg, J.F.

    2014-01-01

    Dutch mothers display diverse labour market behaviour, though typically they work part-time, making modest use of childcare. It is generally assumed that Dutch women are free to make their own choices regarding employment. This narrative of 'choice' finds fertile ground in an era of general

  5. The South African War, Dutch Observers and Military Memory

    African Journals Online (AJOL)

    In 1900, the Dutch Ministry of War sent four military observers to South Africa, in an attempt to come to terms with the latest developments in the military field. Once in South Africa, the promising young Dutch officers selected for the mission remained focused strongly on decisive battles Jominian style, which they considered ...

  6. Management of Tracheostomy: A Survey of Dutch Intensive Care Units

    NARCIS (Netherlands)

    Veelo, Denise P.; Schultz, Marcus J.; Phoa, Kai Y. N.; Dongelmans, Dave A.; Binnekade, Jan M.; Spronk, Peter E.

    2008-01-01

    OBJECTIVE: To determine tracheostomy-management practices in Dutch intensive care units (ICUs) and post-ICU step-down facilities. METHODS: We surveyed the physician medical directors of all Dutch nonpediatric ICUs that have : 5 beds suitable for mechanical ventilation. The survey asked for

  7. The impact of microgeneration upon the Dutch balancing market

    NARCIS (Netherlands)

    Van der Veen, R.A.C.; De Vries, L.J.

    2009-01-01

    The share of microgeneration (power generation at the level of households and small businesses) in the Dutch electricity system continues to grow. Over time, this development may pose a threat to the reliability and efficiency of the Dutch electricity balancing market. We investigated possible

  8. Dutch Agribusiness in Russia: a review of enterpreneurs' perspectives

    NARCIS (Netherlands)

    Belt, J.C.; Berkum, van S.; Roza, P.

    2007-01-01

    Russia¿s economy is booming; that¿s no news. Certainly not for Dutch agribusiness, looking at the steady increase of Dutch exports and investments in Russia. Not only our bigger agro-industries are involved, also medium size companies have found their way to access a market of 143 million consumers.

  9. The future of the Dutch egg processing industry

    NARCIS (Netherlands)

    Tacken, G.M.L.; Cotteleer, G.; Horne, van P.L.M.

    2003-01-01

    This research examines the competitiveness of the Dutch egg products industry, as well as the consequences of tightening up the laws concerning layer poultry on the competitive position of the Dutch egg production industry. A tightening of the laws has, in fact, the effect of raising the production

  10. Sex differences among recipients of benzodiazepines in Dutch general practice.

    NARCIS (Netherlands)

    Waals, F.W. van der; Mohrs, J.; Foets, M.

    1993-01-01

    Objective: To analyse sex differences among recipients of benzodiazepines in Dutch general practice. Design-Study of consultations and associated interventions as recorded in the Dutch national survey of general practice. Setting: Practices of 45 general practitioners monitored during 1 April to 30

  11. Risks of radicalization among Turkish-Dutch young adults?

    NARCIS (Netherlands)

    R.H.J.M. Staring (Richard)

    2014-01-01

    markdownabstract__Abstract__ Young adults heading for Syria to join the jihad and the safety risks associated with their return are currently a topic of debate in the Netherlands and in many other European countries. Around 120 Dutch citizens have gone abroad to join the jihad and the Dutch

  12. Dutch City Network feeds the Innovation of Urban Agriculture

    NARCIS (Netherlands)

    Jansma, J.E.; Veen, E.J.; Kop, van de P.J.; Eijk, van O.N.M.

    2015-01-01

    Since 2010, the Dutch City Network on Urban Agriculture (Stedennetwerk in Dutch), has linked up civil servants of fourteen cities in order to see opportunities, share knowledge and solve issues on urban agriculture in their cities. Though it started as an internally focused network for civil

  13. Lion and dragon: four centuries of Dutch-Vietnamese relations

    NARCIS (Netherlands)

    Kleinen, J.; van der Zwan, B.; Moors, H.; van Zeeland, T.

    2008-01-01

    Dutch-Vietnamese relations go back as far as the beginning of the seventeenth century. For a long time, relations between the Dutch lion and the Vietnamese dragon have been fragile and even violent. Although the relations were not continuously bad, they remained distant rather than warm. Today

  14. Ethnic inequalities in patient safety in Dutch hospital care

    NARCIS (Netherlands)

    van Rosse, F.

    2015-01-01

    This thesis shows the first results of Dutch studies on the relation between ethnicity and patient safety. We used mixed methods to identify patient safety outcomes and patient safety risks in a cohort study in 4 urban hospitals among 763 Dutch patients and 576 ethnic minority patients. In a record

  15. Distributional vowel training may not be effective for Dutch adults

    NARCIS (Netherlands)

    Wanrooij, K.; De Vos, J.F.; Boersma, P.

    2015-01-01

    Distributional vowel training for adults has been reported as "effective" for Spanish and Bulgarian learners of Dutch vowels, in studies using a behavioural task. A recent study did not yield a similar clear learning effect for Dutch learners of the English vowel contrast /æ/~/ε/, as measured with

  16. Cost efficiency of waste management in Dutch municipalities

    NARCIS (Netherlands)

    de Groot, Hans; van Heezik, A.; Hollanders, D.; Felsö, F.

    2011-01-01

    This paper analyses the cost efficiency of waste management of Dutch municipalities. For the first time stochastic frontier analysis is applied to Dutch data, employing recent multi-year data (2005-2008). The preliminary findings confirm earlier results on the importance for cost efficiency of

  17. Collusion in the Dutch construction industry: an industrial organization perspective

    NARCIS (Netherlands)

    Doree, Andries G.

    2004-01-01

    Several investigations by parliament, cabinet, justice and antitrust authorities have shown a widespread use of cartels and structural bid rigging within the Dutch construction industry. The reputation of the Dutch construction industry has been dented with both the general public and clients. As a

  18. Development and evaluation of the Dutch Healthy Diet index 2015.

    NARCIS (Netherlands)

    Looman, Moniek; Feskens, Edith Jm; de Rijk, Mariëlle; Meijboom, Saskia; Biesbroek, Sander; Temme, Elisabeth Hm; de Vries, Jeanne; Geelen, Anouk

    To update the Dutch Healthy Diet index, a measure of diet quality, to reflect adherence to the Dutch dietary guidelines 2015 and to evaluate against participants' characteristics and nutrient intakes with the score based on 24 h recall (24 hR) data and FFQ data.

  19. Euthanasia in the Broader Framework of Dutch Penal Policies

    NARCIS (Netherlands)

    Groenhuijsen, M.S.; van Laanen, F.; Groenhuijsen, M.S.; van Laanen, F.

    2006-01-01

    The authors have regarded euthanasia in the broader framework of Dutch penal policies. They present euthanasia as a typical example of the pragmatic - rather than dogmatic - way the Dutch try to tackle difficult moral problems in connection with the criminal justice system. Definitions, statutory

  20. A Neural Network Based Dutch Part of Speech Tagger

    NARCIS (Netherlands)

    Boschman, E.; op den Akker, Hendrikus J.A.; Nijholt, A.; Nijholt, Antinus; Pantic, Maja; Pantic, M.; Poel, M.; Poel, Mannes; Hondorp, G.H.W.

    2008-01-01

    In this paper a Neural Network is designed for Part-of-Speech Tagging of Dutch text. Our approach uses the Corpus Gesproken Nederlands (CGN) consisting of almost 9 million transcribed words of spoken Dutch, divided into 15 different categories. The outcome of the design is a Neural Network with an

  1. ON THE RELATIONSHIP BETWEEN DUTCH AND GERMAN INTEREST-RATES

    NARCIS (Netherlands)

    DEHAAN, J; PILAT, DJ; ZELHORST, D

    1991-01-01

    In this paper the relationship between Dutch and German short-term and long-term interest rates is examined. Using cointegration techniques, it is found that the covered interest parity hypothesis holds for short-term interest rates. This evidence supports the recent shift of emphasis of Dutch

  2. Patient participation in collective healthcare decision making: the Dutch model

    NARCIS (Netherlands)

    van de Bovenkamp, H.; Trappenburg, M.J.; Grit, K.

    2010-01-01

    Objective To study whether the Dutch participation model is a good model of participation. Background Patient participation is on the agenda, both on the individual and the collective level. In this study, we focus on the latter by looking at the Dutch model in which patient organizations are

  3. Patient participation in collective healthcare decision making: the Dutch model

    NARCIS (Netherlands)

    van de Bovenkamp, H.M.; Trappenburg, M.J.; Grit, K.J.

    2010-01-01

    Objective  To study whether the Dutch participation model is a good model of participation. Background  Patient participation is on the agenda, both on the individual and the collective level. In this study, we focus on the latter by looking at the Dutch model in which patient organizations are

  4. Patient participation in collective healthcare decision making: the Dutch model

    NARCIS (Netherlands)

    Bovenkamp, H. van de; Trappenburg, M.J.; Grit, K. J.

    2009-01-01

    Objective To study whether the Dutch participation model is a good model of participation. Background Patient participation is on the agenda, both on the individual and the collective level. In this study, we focus on the latter by looking at the Dutch model in which patient organizations are

  5. Mainstream and alternative sources of finance in Dutch agriculture

    NARCIS (Netherlands)

    Meulen, van der H.A.B.; Asseldonk, van M.A.P.M.

    2017-01-01

    In this paper mainstream and alternative sources of finance in
    Dutch agriculture is analysed. Dutch farmers make use of
    different sources of finance whereby bank loans continue to
    serve as the major source of debt financing. The average bank
    loan was approximately 740,000 euro per

  6. A century of Dutch copyright law: auteurswet 1912-2012

    NARCIS (Netherlands)

    Hugenholtz, B.; Quaedvlieg, A.; Visser, D.

    2012-01-01

    On September 23, 1912, the Dutch Copyright Act - Auteurswet - was enacted. A century after its enactment the Dutch law is one of the world's oldest 'living' acts of the author's rights tradition. While the Act has seen many small and large amendments since its adoption in 1912, it has never been

  7. The Kenyan meat sector Opportunities for Dutch agribusiness

    NARCIS (Netherlands)

    Bergevoet, R.H.M.; Engelen, A.

    2014-01-01

    This study identifies opportunities for the Dutch agribusiness sector (industry as well as research and education) in Kenyan meat value chains at all levels. It can serve as a background document for Dutch agro-food business companies as well as other stakeholders. At the moment there is increased

  8. The acquisition of the Dutch discourse particle wel

    NARCIS (Netherlands)

    Hogeweg, L.; Gerrevink, R.

    2015-01-01

    Dutch is known for its large inventory of discourse particles. Discourse particles are very frequent and highly important in conversation. Yet, not much research has been conducted on how children acquire them. In this paper we focus on the acquisition of the Dutch discourse particle wel. In earlier

  9. Cultural Hybridity Reconsidered : Religious Visual Culture and the Dutch Republic

    NARCIS (Netherlands)

    Stronks, E.

    2012-01-01

    This article engages with the overriding tendency to see cultural hybridity as a progressive force in the Dutch Republic, focusing on the case of Dutch religious literature. It is a puzzling fact that in the literary realm, processes of cultural hybridity were put on hold between 1560 and 1680. In

  10. Questions of legal responsibility for Srebrenica before the Dutch courts

    NARCIS (Netherlands)

    Spijkers, Otto

    This contribution provides an overview of the litigation in the Dutch civil and criminal courts concerning the Srebrenica massacre. The author maps out the Dutch courts' divergent approaches to immunity of United Nations peacekeepers, state responsibility and individual criminal responsibility for

  11. Arousing Discontent : Dutch Pornographic Plays, 1670-1800

    NARCIS (Netherlands)

    Leemans, I.B.

    2012-01-01

    n the second half of the seventeenth century, the Dutch Republic became the "sex shop" of Europe, printing large varieties of erotic works and distributing them through various reader groups. The production of pornographic works also inspired Dutch authors, who developed new forms of erotic prose

  12. National Identification of Dutch Youth: An Exploratory Study

    Science.gov (United States)

    Oppenheimer, Louis

    2011-01-01

    246 Dutch participants aged 8, 10, 12, 14, and 16 years were presented with the Strength of Identification Scale (SoIS; Barrett, 2007) and the National Identity scale based on Cultural and Historical achievements (NICH; derived from the NATID, Keillor & Hult, 1999). The study aimed to examine the extent and nature of Dutch children and…

  13. The Dutch Are Missing in the American Curriculum

    Science.gov (United States)

    Claunch, Ann

    2009-01-01

    The Dutch are missing in any U.S. history textbook, in the content standards, and in the nationally endorsed curriculum. Outside of New York State history classes, there is almost no mention of the Dutch influence in early 17th-century America. Fleeting references to the Netherlands as a staging area for the Pilgrims' famous "Mayflower"…

  14. Dutch gas amendment throws open market to generators

    International Nuclear Information System (INIS)

    Anon.

    1997-01-01

    An analysis of the text of an amendment to European Union legislation on gas power production rules from the Dutch government is set out. Its pro-liberalisation stance has profound implications for the French, Belgian and Austrian gas power producers. Reactions to the Dutch proposals are noted and data on gas markets in various Member States is presented in table form. (UK)

  15. Quantifying fishers' and citizens' support for Dutch flatfish management policy

    NARCIS (Netherlands)

    Groeneveld, R.A.

    2011-01-01

    Fisheries policy is most effective when supported by fishers and the general public. Dutch citizens' and fishers' support for a selection of policy alternatives to enhance the sustainability of the Dutch North Sea cutter fleet is estimated, and the same groups' support for policy alternatives is

  16. Validity of the Parental Burnout Inventory among Dutch employees

    NARCIS (Netherlands)

    van Bakel, H.J.A.; van Engen, M.L.; Peters, P.

    2018-01-01

    The purpose of this study was to validate the Parental Burnout Inventory (PBI) in a Dutch sample of working parents. The Dutch version of the PBI and questionnaires about work were administered to 627 working parents, with at least one child living at home. We investigated whether the

  17. Regulating the Dutch pharmaceutical market: improving efficiency or controlling costs?

    NARCIS (Netherlands)

    Wolf, de P.; Brouwer, W.B.F.; Rutten, F.H.

    2005-01-01

    In this paper, we describe the Dutch pharmaceutical market, which is heavily regulated by the government. Through the regulation of prices and promoting prudent use, the Dutch government tries to bring down the cost of pharmaceuticals, which increases every year at a higher rate than total health

  18. EFL Academic writing. What should Dutch business communication students learn?

    NARCIS (Netherlands)

    Meurs, Frank van; Hendriks, B.C.; Planken, B.C.; Barasa, S.N.; Groot, E.B. de; Nederstigt, U.; Arnhem, M. van; Smakman, D.

    2016-01-01

    Many Dutch university students are expected to read and write academic research papers in English. In this article, we discuss a number of areas of EFL academic writing that are relevant for first-year Dutch business communication students. These students need to become familiar with quantitative

  19. A comparison to West Germanic languages and Dutch dialects

    African Journals Online (AJOL)

    Kate H

    From an applied perspective (i.e. human language technology), we aim to .... Frisian group)?. 3. 2. If so, are there Dutch dialects which are closer to Afrikaans than Standard Dutch is? If this is so, which one is closest and would therefore be better suited for our ...... divergence: Conceptual and methodological considerations.

  20. Decreasing the overall environmental impact of the Dutch diet

    NARCIS (Netherlands)

    Kramer, Gerard F.H.; Tyszler, Marcelo; Veer, van 't Pieter; Blonk, Hans

    2017-01-01

    Objective: To find diets optimised on nutrition and environmental impact close to the current Dutch diet and to identify the most effective and acceptable options for mitigating environmental impact. Design: Linear programming was used to optimise diets of Dutch men and women aged 9–69 years,