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Sample records for large dutch paraganglioma

  1. 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 ...

  2. 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.

  3. 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

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

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    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.

  5. 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

  6. 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.

  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. 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

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

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    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.)

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

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    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.

  11. 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.

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

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    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.

  13. 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

  14. 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.

  15. 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)

  16. 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....

  17. Paraganglioma of Prostatic Origin

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    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.

  18. Upscaling Large Scale Deep Renovation in the Dutch Residential Sector : A Case Study

    NARCIS (Netherlands)

    van Oorschot, John; Hofman, Erwin; Halman, Johannes I.M.

    2016-01-01

    The 2014 carbon emission of the Dutch economy was estimated at 194.4 billion kg. Dutch households are responsible for a particular large part of the total emission, about 37.1 billion kg or 19% of the total emission in 2014. While society becomes aware of the negative side effects of carbon emission

  19. Compound Decomposition in Dutch Large Vocabulary Speech Recognition

    NARCIS (Netherlands)

    Ordelman, Roeland J.F.; van Hessen, Adrianus J.; de Jong, Franciska M.G.

    2003-01-01

    This paper addresses compound splitting for Dutch in the context of broadcast news transcription. Language models were created using original text versions and text versions that were decomposed using a data-driven compound splitting algorithm. Language model performances were compared in terms of

  20. 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 ...

  1. Temporal bone paragangliomas: 15 years experience

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    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.

  2. 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.

  3. 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.

  4. Cohort Profile of The GOALS Study: A Large-scale Research of Physical Activity in Dutch Students

    NARCIS (Netherlands)

    De Groot, Renate; Van Dijk, Martin; Kirschner, Paul A.

    2016-01-01

    The GOALS study (Grootschalig Onderzoek naar Activiteiten van Limburgse Scholieren [Large-scale Research of Activities in Dutch Students]) was set up to investigate possible associations between different forms of physical activity and inactivity with cognitive performance, academic achievement and

  5. 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

  6. 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 ...

  7. 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 ...

  8. 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 ...

  9. 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

  10. 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.

  11. Automatic Trip Detection with the Dutch Mobile Mobility Panel: Towards Reliable Multiple-Week Trip Registration for Large Samples

    NARCIS (Netherlands)

    Thomas, Tom; Geurs, Karst T.; Koolwaaij, Johan; Bijlsma, Marcel E.

    2018-01-01

    This paper examines the accuracy of trip and mode choice detection of the last wave of the Dutch Mobile Mobility Panel, a large-scale three-year, smartphone-based travel survey. Departure and arrival times, origins, destinations, modes, and travel purposes were recorded during a four week period in

  12. Cohort Profile of the Goals Study: A Large-Scale Research of Physical Activity in Dutch Students

    Science.gov (United States)

    de Groot, Renate H. M.; van Dijk, Martin L.; Kirschner, Paul A.

    2015-01-01

    The GOALS study (Grootschalig Onderzoek naar Activiteiten van Limburgse Scholieren [Large-scale Research of Activities in Dutch Students]) was set up to investigate possible associations between different forms of physical activity and inactivity with cognitive performance, academic achievement and mental well-being. It was conducted at a…

  13. Nonfunctional Cystic Hepatic Paraganglioma Mimicking Hydatid Cyst

    Directory of Open Access Journals (Sweden)

    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.

  14. 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.

  15. 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

  16. 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.

  17. 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.

  18. 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.

  19. 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.

  20. 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.

  1. Annoyance from environmental tobacco smoke and support for no-smoking policies at eight large Dutch workplaces

    OpenAIRE

    Willemsen, M. C.; de Vries, H.; Genders, R.

    1996-01-01

    OBJECTIVE: To collect descriptive data on annoyance from environmental tobacco smoke (ETS), attitudes toward smoking at the workplace, and support for a no-smoking policy. DESIGN: Survey of eight large companies in four sectors of Dutch industry. SUBJECTS: A representative sample of smoking and non-smoking employees (n = 1480). RESULTS: Thirty- five per cent of non-smoking employees felt it was "annoying" to "very annoying" when their colleagues smoked during worktime, and 78% thought a...

  2. Large-scale offshore wind energy. Cost analysis and integration in the Dutch electricity market

    International Nuclear Information System (INIS)

    De Noord, M.

    1999-02-01

    The results of analysis of the construction and integration costs of large-scale offshore wind energy (OWE) farms in 2010 are presented. The integration of these farms (1 and 5 GW) in the Dutch electricity distribution system have been regarded against the background of a liberalised electricity market. A first step is taken for the determination of costs involved in solving integration problems. Three different types of foundations are examined: the mono-pile, the jacket and a new type of foundation: the concrete caisson pile: all single-turbine-single-support structures. For real offshore applications (>10 km offshore, at sea-depths >20 m), the concrete caisson pile is regarded as the most suitable. The price/power ratios of wind turbines are analysed. It is assumed that in 2010 turbines in the power range of 3-5 MW are available. The main calculations have been conducted for a 3 MW turbine. The main choice in electrical infrastructure is for AC or DC. Calculations show that at distances of 30 km offshore and more, the use of HVDC will result in higher initial costs but lower operating costs. The share of operating and maintenance (O ampersand M) costs in the kWh cost price is approximately 3.3%. To be able to compare the two farms, a base case is derived with a construction time of 10 years for both. The energy yield is calculated for a wind regime offshore of 9.0 m/s annual mean wind speed. Per 3 MW turbine this results in an annual energy production of approximately 12 GWh. The total farm efficiency amounts to 82%, resulting in a total farm capacity factor of 38%. With a required internal rate of return of 15%, the kWh cost price amounts to 0.24 DFl and 0.21 DFl for the 1 GW and 5 GW farms respectively in the base case. The required internal rate of return has a large effect on the kWh cost price, followed by costs of subsystems. O ampersand M costs have little effect on the cost price. Parameter studies show that a small cost reduction of 5% is possible when

  3. Paraganglioma pré-aórtico gigante Giant preaortic paraganglioma

    Directory of Open Access Journals (Sweden)

    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

  4. 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.

  5. 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.

  6. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  8. 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

  9. 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

  10. 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.

  11. 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.

  12. Pooled Resequencing of 122 Ulcerative Colitis Genes in a Large Dutch Cohort Suggests Population-Specific Associations of Rare Variants in MUC2.

    Science.gov (United States)

    Visschedijk, Marijn C; Alberts, Rudi; Mucha, Soren; Deelen, Patrick; de Jong, Dirk J; Pierik, Marieke; Spekhorst, Lieke M; Imhann, Floris; van der Meulen-de Jong, Andrea E; van der Woude, C Janneke; van Bodegraven, Adriaan A; Oldenburg, Bas; Löwenberg, Mark; Dijkstra, Gerard; Ellinghaus, David; Schreiber, Stefan; Wijmenga, Cisca; Rivas, Manuel A; Franke, Andre; van Diemen, Cleo C; Weersma, Rinse K

    2016-01-01

    Genome-wide association studies have revealed several common genetic risk variants for ulcerative colitis (UC). However, little is known about the contribution of rare, large effect genetic variants to UC susceptibility. In this study, we performed a deep targeted re-sequencing of 122 genes in Dutch UC patients in order to investigate the contribution of rare variants to the genetic susceptibility to UC. The selection of genes consists of 111 established human UC susceptibility genes and 11 genes that lead to spontaneous colitis when knocked-out in mice. In addition, we sequenced the promoter regions of 45 genes where known variants exert cis-eQTL-effects. Targeted pooled re-sequencing was performed on DNA of 790 Dutch UC cases. The Genome of the Netherlands project provided sequence data of 500 healthy controls. After quality control and prioritization based on allele frequency and pathogenicity probability, follow-up genotyping of 171 rare variants was performed on 1021 Dutch UC cases and 1166 Dutch controls. Single-variant association and gene-based analyses identified an association of rare variants in the MUC2 gene with UC. The associated variants in the Dutch population could not be replicated in a German replication cohort (1026 UC cases, 3532 controls). In conclusion, this study has identified a putative role for MUC2 on UC susceptibility in the Dutch population and suggests a population-specific contribution of rare variants to UC.

  13. 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.

  14. 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

  15. Correlates of sedentary time in different age groups: results from a large cross sectional Dutch survey.

    Science.gov (United States)

    Bernaards, Claire M; Hildebrandt, Vincent H; Hendriksen, Ingrid J M

    2016-10-26

    Evidence shows that prolonged sitting is associated with an increased risk of mortality, independent of physical activity (PA). The aim of the study was to identify correlates of sedentary time (ST) in different age groups and day types (i.e. school-/work day versus non-school-/non-work day). The study sample consisted of 1895 Dutch children (4-11 years), 1131 adolescents (12-17 years), 8003 adults (18-64 years) and 1569 elderly (65 years and older) who enrolled in the Dutch continuous national survey 'Injuries and Physical Activity in the Netherlands' between 2006 and 2011. Respondents estimated the number of sitting hours during a regular school-/workday and a regular non-school/non-work day. Multiple linear regression analyses on cross-sectional data were used to identify correlates of ST. Significant positive associations with ST were observed for: higher age (4-to-17-year-olds and elderly), male gender (adults), overweight (children), higher education (adults ≥ 30 years), urban environment (adults), chronic disease (adults ≥ 30 years), sedentary work (adults), not meeting the moderate to vigorous PA (MVPA) guideline (children and adults ≥ 30 years) and not meeting the vigorous PA (VPA) guideline (4-to-17-year-olds). Correlates of ST that significantly differed between day types were working hours and meeting the VPA guideline. More working hours were associated with more ST on school-/work days. In children and adolescents, meeting the VPA guideline was associated with less ST on non-school/non-working days only. This study provides new insights in the correlates of ST in different age groups and thus possibilities for interventions in these groups. Correlates of ST appear to differ between age groups and to a lesser degree between day types. This implies that interventions to reduce ST should be age specific. Longitudinal studies are needed to draw conclusions on causality of the relationship between identified correlates and ST.

  16. Correlates of sedentary time in different age groups: results from a large cross sectional Dutch survey

    Directory of Open Access Journals (Sweden)

    Claire M. Bernaards

    2016-10-01

    Full Text Available Abstract Background Evidence shows that prolonged sitting is associated with an increased risk of mortality, independent of physical activity (PA. The aim of the study was to identify correlates of sedentary time (ST in different age groups and day types (i.e. school-/work day versus non-school-/non-work day. Methods The study sample consisted of 1895 Dutch children (4–11 years, 1131 adolescents (12–17 years, 8003 adults (18–64 years and 1569 elderly (65 years and older who enrolled in the Dutch continuous national survey ‘Injuries and Physical Activity in the Netherlands’ between 2006 and 2011. Respondents estimated the number of sitting hours during a regular school-/workday and a regular non-school/non-work day. Multiple linear regression analyses on cross-sectional data were used to identify correlates of ST. Results Significant positive associations with ST were observed for: higher age (4-to-17-year-olds and elderly, male gender (adults, overweight (children, higher education (adults ≥ 30 years, urban environment (adults, chronic disease (adults ≥ 30 years, sedentary work (adults, not meeting the moderate to vigorous PA (MVPA guideline (children and adults ≥ 30 years and not meeting the vigorous PA (VPA guideline (4-to-17-year-olds. Correlates of ST that significantly differed between day types were working hours and meeting the VPA guideline. More working hours were associated with more ST on school-/work days. In children and adolescents, meeting the VPA guideline was associated with less ST on non-school/non-working days only. Conclusions This study provides new insights in the correlates of ST in different age groups and thus possibilities for interventions in these groups. Correlates of ST appear to differ between age groups and to a lesser degree between day types. This implies that interventions to reduce ST should be age specific. Longitudinal studies are needed to draw conclusions on causality of

  17. 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.)

  18. 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.

  19. 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

  20. 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

  1. CYP2D6 and CYP2C19 activity in a large population of Dutch healthy volunteers : indications for oral contraceptive-related gender differences

    NARCIS (Netherlands)

    Tamminga, WJ; Wemer, J; Oosterhuis, B; Wieling, J; Wilffert, B; de Leij, LFMH; de Zeeuw, RA; Jonkman, JHG

    Objective: We examined a large database containing results on CYP2D6 and CYP2C19 activity of 4301 Dutch volunteers phenotyped in the context of various clinical pharmacology studies. Methods: The subjects were given 22 mg dextromethorphan, 100 mg mephenytoin and 200 mg caffeine. For CYP2D6, the

  2. 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.

  3. 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.

  4. 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.

  5. 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.

  6. 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.

  7. 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.

  8. 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

  9. 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.

  10. 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.

  11. 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)

  12. 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 ...

  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. 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.

  15. 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.

  16. 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

  17. Restoration of wet dune slacks on the Dutch Wadden Sea islands : Recolonization after large-scale sod cutting

    NARCIS (Netherlands)

    Grootjans, AP; Everts, H; Bruin, K; Fresco, L; Grootjans, Ab P.

    The effects of sod cutting were studied in a dune area on the Dutch Wadden Sea Island of Texel. Sod cutting was carried out in a range of different dune slacks in order to restore dune slack vegetation with many endangered Red List species. Sod cutting removed approximately 96% of the soil seed

  18. Restoration of Wet Dune Slacks on the Dutch Wadden Sea Islands: Recolonization After Large-Scale Sod Cutting

    NARCIS (Netherlands)

    Grootjans, A.P.; Everts, H.; Bruin, K.; Fresco, L.

    2011-01-01

    The effects of sod cutting were studied in a dune area on the Dutch Wadden Sea Island of Texel. Sod cutting was carried out in a range of different dune slacks in order to restore dune slack vegetation with many endangered Red List species. Sod cutting removed approximately 96% of the soil seed

  19. Validation of a disease-specific quality-of-life questionnaire in a large sample of Dutch achalasia patients

    NARCIS (Netherlands)

    Frankhuisen, R.; Heijkoop, R.; van Herwaarden, M. A.; de Vries, D. R.; Smout, A. J. P. M.; Baron, A.; Samsom, M.

    2008-01-01

    SUMMARY: The aim of this study was to validate a translated version of an achalasia-specific quality-of-life questionnaire (achalasia-DSQoL) by examining its psychometric properties in a Dutch cohort of achalasia patients. The achalasia-DSQoL was administered to 171 treated achalasia patients

  20. 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

  1. 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.

  2. 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.

  3. 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.

  4. 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.

  5. 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

  6. 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.

  7. 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

  8. 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.)

  9. 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.

  10. 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

  11. 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.

  12. 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.

  13. 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.

  14. 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.

  15. 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.

  16. 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.)

  17. 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....

  18. 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

  19. 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.

  20. 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.

  1. 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.

  2. The effects of language bias and cultural bias estimated using the method of correlated vectors on a large database of IQ comparisons between native Dutch and ethnic minority immigrants from non-Western countries

    NARCIS (Netherlands)

    te Nijenhuis, J.; Willigers, D.; Dragt, J.; van der Flier, H.

    2015-01-01

    Cultural factors and language bias are often used to explain the large differences in mean intelligence test scores between ethnic groups, for instance majority Dutch compared to ethnic minorities. A competing explanation comes from Spearman's hypothesis, which states that these group differences in

  3. 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...

  4. Evaluation of a large scale implementation of disease management programmes in various Dutch regions: a study protocol.

    Science.gov (United States)

    Lemmens, Karin M M; Rutten-Van Mölken, Maureen P M H; Cramm, Jane M; Huijsman, Robbert; Bal, Roland A; Nieboer, Anna P

    2011-01-10

    Disease management programmes (DMPs) have been developed to improve effectiveness and economic efficiency within chronic care delivery by combining patient-related, professional-directed, and organisational interventions. The benefits of DMPs within different settings, patient groups, and versions remain unclear. In this article we propose a protocol to evaluate a range of current DMPs by capturing them in a single conceptual framework, employing comparable structure, process, and outcome measures, and combining qualitative and quantitative research methods. To assess DMP effectiveness a practical clinical trial will be conducted. Twenty-two disease management experiments will be studied in various Dutch regions consisting of a variety of collaborations between organisations and/or professionals. Patient cohorts include those with cardiovascular diseases, chronic obstructive pulmonary disease, diabetes, stroke, depression, psychotic diseases, and eating disorders. Our methodological approach combines qualitative and quantitative research methods to enable a comprehensive evaluation of complex programmes. Process indicators will be collected from health care providers' data registries and measured via physician and staff questionnaires. Patient questionnaires include health care experiences, health care utilisation, and quality of life. Qualitative data will be gathered by means of interviews and document analysis for an in depth description of project interventions and the contexts in which DMPs are embedded, and an ethnographic process evaluation in five DMPs. Such a design will provide insight into ongoing DMPs and demonstrate which elements of the intervention are potentially (cost)-effective for which patient populations. It will also enable sound comparison of the results of the different programmes. The study will lead to a better understanding of (1) the mechanisms of disease management, (2) the feasibility, and cost-effectiveness of a disease management

  5. Evaluation of a large scale implementation of disease management programmes in various Dutch regions: a study protocol

    Science.gov (United States)

    2011-01-01

    Background Disease management programmes (DMPs) have been developed to improve effectiveness and economic efficiency within chronic care delivery by combining patient-related, professional-directed, and organisational interventions. The benefits of DMPs within different settings, patient groups, and versions remain unclear. In this article we propose a protocol to evaluate a range of current DMPs by capturing them in a single conceptual framework, employing comparable structure, process, and outcome measures, and combining qualitative and quantitative research methods. Methods To assess DMP effectiveness a practical clinical trial will be conducted. Twenty-two disease management experiments will be studied in various Dutch regions consisting of a variety of collaborations between organisations and/or professionals. Patient cohorts include those with cardiovascular diseases, chronic obstructive pulmonary disease, diabetes, stroke, depression, psychotic diseases, and eating disorders. Our methodological approach combines qualitative and quantitative research methods to enable a comprehensive evaluation of complex programmes. Process indicators will be collected from health care providers' data registries and measured via physician and staff questionnaires. Patient questionnaires include health care experiences, health care utilisation, and quality of life. Qualitative data will be gathered by means of interviews and document analysis for an in depth description of project interventions and the contexts in which DMPs are embedded, and an ethnographic process evaluation in five DMPs. Such a design will provide insight into ongoing DMPs and demonstrate which elements of the intervention are potentially (cost)-effective for which patient populations. It will also enable sound comparison of the results of the different programmes. Discussion The study will lead to a better understanding of (1) the mechanisms of disease management, (2) the feasibility, and cost

  6. Evaluation of a large scale implementation of disease management programmes in various Dutch regions: a study protocol

    Directory of Open Access Journals (Sweden)

    Bal Roland A

    2011-01-01

    Full Text Available Abstract Background Disease management programmes (DMPs have been developed to improve effectiveness and economic efficiency within chronic care delivery by combining patient-related, professional-directed, and organisational interventions. The benefits of DMPs within different settings, patient groups, and versions remain unclear. In this article we propose a protocol to evaluate a range of current DMPs by capturing them in a single conceptual framework, employing comparable structure, process, and outcome measures, and combining qualitative and quantitative research methods. Methods To assess DMP effectiveness a practical clinical trial will be conducted. Twenty-two disease management experiments will be studied in various Dutch regions consisting of a variety of collaborations between organisations and/or professionals. Patient cohorts include those with cardiovascular diseases, chronic obstructive pulmonary disease, diabetes, stroke, depression, psychotic diseases, and eating disorders. Our methodological approach combines qualitative and quantitative research methods to enable a comprehensive evaluation of complex programmes. Process indicators will be collected from health care providers' data registries and measured via physician and staff questionnaires. Patient questionnaires include health care experiences, health care utilisation, and quality of life. Qualitative data will be gathered by means of interviews and document analysis for an in depth description of project interventions and the contexts in which DMPs are embedded, and an ethnographic process evaluation in five DMPs. Such a design will provide insight into ongoing DMPs and demonstrate which elements of the intervention are potentially (cost-effective for which patient populations. It will also enable sound comparison of the results of the different programmes. Discussion The study will lead to a better understanding of (1 the mechanisms of disease management, (2 the

  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. Vegetable, fruit and nitrate intake in relation to the risk of Barrett's oesophagus in a large Dutch cohort

    NARCIS (Netherlands)

    Keszei, A.P.; Schouten, L.J.; Driessen, A.L.C.; Huysentruyt, C.J.R.; Keulemans, Y.C.A.; Goldbohm, R.A.; Brandt, P.A. van den

    2014-01-01

    There are few epidemiological data on the dietary risk factors of Barrett's oesophagus, a precursor of oesophageal adenocarcinoma. The present study investigated the association between vegetable, fruit and nitrate intake and Barrett's oesophagus risk in a large prospective cohort. The Netherlands

  10. Large scale implementation of clinical medication reviews in Dutch community pharmacies: Drug-related problems and interventions

    NARCIS (Netherlands)

    Kempen, Thomas G. H.; Van De Steeg-Van Gompel, Caroline H. P. A.; Hoogland, Petra; Liu, Yuqian; Bouvy, Marcel L.

    2014-01-01

    Background: Research on the benefits of clinical medication reviews (CMRs) performed by pharmacists has been conducted mostly in controlled settings and has been widely published. Less is known of the effects after large scale implementation in community pharmacies. An online CMR tool enabled the

  11. Transformations of large technical systems : a multi-level analysis of the Dutch highway system (1950-2000)

    NARCIS (Netherlands)

    Geels, F.W.

    2007-01-01

    The transformation of existing systems is an underexposed topic in large technical systems (LTS) research. Most LTS research has focused on the emergence and stabilization of systems, ending with momentum. But how is momentum overcome, and how do transformations come about? This article presents a

  12. 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

  13. 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.

  14. 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...

  15. 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

  16. Final Results from Mexnext-I. Analysis of detailed aerodynamic measurements on a 4.5 m diameter rotor placed in the large German Dutch Wind Tunnel DNW

    Energy Technology Data Exchange (ETDEWEB)

    Schepers, J.G.; Boorsma, K. [Energy research Center of the Netherlands ECN, Petten (Netherlands); Munduate, X. [National Renewable Energy Center, CENER, Pamplona (Spain)

    2013-02-15

    The paper presents the final results from the first phase of IEA Task 29 'Mexnext'. Mexnext was a joint project in which 20 parties from 11 different countries cooperated. The main aim of Mexnext was to analyse the wind tunnel measurements which have been taken in the EU project 'MEXICO'. In the MEXICO project 10 institutes from 6 countries cooperated in doing experiments on an instrumented, three-bladed wind turbine of 4.5 m diameter placed in the 9.5 by 9.5 m{sup 2} open section of the Large Low-speed Facility (LLF) of the test facility DNW (German-Dutch Wind Tunnels). Pressure distributions on the blades were obtained from 148 Kulite pressure sensors, distributed over 5 sections at 25, 35, 60, 82 and 92% radial position respectively. Blade loads were monitored through two strain-gauge bridges at each blade root. Most interesting however are the extensive PIV flow field measurements, which have been taken simultaneously with the pressure and load measurements. As a result of the international collaboration within this task a very thorough analysis of the data could be carried out and a large number of codes were validated not only in terms of loads but also in terms of underlying flow field. The paper will present several results from Mexnext-I, i.e. validation results and conclusion on modelling deficiencies and directions for model improvement. The future plans of the Mexnext consortium are also briefly discussed. Amongst these are Mexnext-II, a project in which also aerodynamic measurements other than MEXICO are included, and 'New MEXICO' in which additional measurement on the MEXICO model are performed.

  17. 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.

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

    Directory of Open Access Journals (Sweden)

    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

  19. 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.

  20. Fetal Environment Is a Major Determinant of the Neonatal Blood Thyroxine Level: Results of a Large Dutch Twin Study.

    Science.gov (United States)

    Zwaveling-Soonawala, Nitash; van Beijsterveldt, Catharina E M; Mesfum, Ertirea T; Wiedijk, Brenda; Oomen, Petra; Finken, Martijn J J; Boomsma, Dorret I; van Trotsenburg, A S Paul

    2015-06-01

    The interindividual variability in thyroid hormone function parameters is much larger than the intraindividual variability, suggesting an individual set point for these parameters. There is evidence to suggest that environmental factors are more important than genetic factors in the determination of this individual set point. This study aimed to quantify the effect of genetic factors and (fetal) environment on the early postnatal blood T4 concentration. This was a classical twin study comparing the resemblance of neonatal screening blood T4 concentrations in 1264 mono- and 2566 dizygotic twin pairs retrieved from the population-based Netherlands Twin Register. Maximum-likelihood estimates of variance explained by genetic and environmental influences were obtained by structural equation modeling in data from full-term and preterm twin pairs. In full-term infants, genetic factors explained 40%/31% of the variance in standardized T4 scores in boys/girls, and shared environment, 27%/22%. The remaining variance of 33%/47% was due to environmental factors not shared by twins. For preterm infants, genetic factors explained 34%/0% of the variance in boys/girls, shared environment 31%/57%, and unique environment 35%/43%. In very preterm twins, no significant contribution of genetic factors was observed. Environment explains a large proportion of the resemblance of the postnatal blood T4 concentration in twin pairs. Because we analyzed neonatal screening results, the fetal environment is the most likely candidate for these environmental influences. Genetic influences on the T4 set point diminished with declining gestational age, especially in girls. This may be due to major environmental influences such as immaturity and nonthyroidal illness in very preterm infants.

  1. Increased Risk of Biochemical and Clinical Failure for Prostate Patients with a Large Rectum at Radiotherapy Planning: Results from the Dutch Trial of 68 GY Versus 78 Gy

    International Nuclear Information System (INIS)

    Heemsbergen, Wilma D.; Hoogeman, Mischa S.; Witte, Marnix G.; Peeters, Stephanie T.H.; Incrocci, Luca; Lebesque, Joos V.

    2007-01-01

    Purpose: To investigate whether a large rectum filling visible on the planning CT scan was associated with a decrease in freedom from any failure (FFF) and freedom from clinical failure (FFCF) for prostate cancer patients. Methods and Materials: Patients from the Dutch trial (78 Gy vs. 68 Gy) with available acute toxicity data were analyzed (n = 549). A 10-mm margin was applied for the first 68 Gy and 0-5 mm for the 10-Gy boost. The dose in the seminal vesicles (SVs) was prescribed within four treatment groups according to the estimated risk of SV involvement. Two potential risk factors (RFs) for a geometric miss were defined: (1) an anorectal volume ≥ 90 cm 3 and ≥ 25% of treatment-time diarrhea (RF1); and (2) the mean cross-sectional area of the anorectum (RF2). We tested whether these were significant predictors for FFF and FFCF within each treatment group. Results: Significant results were observed only for patients with a risk of SV involvement > 25% (dose of 68-78 Gy to the SVs, n = 349). We found a decrease in FFF (p = 0.001) and FFCF (p = 0.01) for the 87 patients with RF1 (for RF2, p = 0.02 and p = 0.01, respectively). The estimated decrease in the FFCF rate at 5 years was 15%. Conclusion: Tumor control was significantly decreased in patients with a risk of SV involvement > 25% and at risk of geometric miss. Current image guidance techniques offer several solutions to geometrically optimize the treatment. Additional research is needed to evaluate whether geometric misses can be prevented using these techniques

  2. 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

  3. 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

  4. 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

  5. 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

  6. 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.

  7. 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

  8. 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.

  9. 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.

  10. 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

  11. 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

  12. 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.

  13. 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.

  14. 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)

  15. [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.

  16. 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.

  17. 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.

  18. 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

  19. 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

  20. 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

  1. Contrast-enhanced spectral mammography in recalls from the Dutch breast cancer screening program : validation of results in a large multireader, multicase study

    OpenAIRE

    Lalji, U C; Houben, I P L; Prevos, R; Gommers, S; van Goethem, M; Vanwetswinkel, S; Pijnappel, R; Steeman, R; Frotscher, C; Mok, W; Nelemans, P; Smidt, M L; Beets-Tan, R G; Wildberger, J E; Lobbes, M B I

    2016-01-01

    OBJECTIVES: Contrast-enhanced spectral mammography (CESM) is a promising problem-solving tool in women referred from a breast cancer screening program. We aimed to study the validity of preliminary results of CESM using a larger panel of radiologists with different levels of CESM experience. METHODS: All women referred from the Dutch breast cancer screening program were eligible for CESM. 199 consecutive cases were viewed by ten radiologists. Four had extensive CESM experience, three had no C...

  2. '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

  3. 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.

  4. 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.

  5. 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.

  6. 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

  7. 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.

  8. 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

  9. 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 %.

  10. 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.

  11. 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.

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

    Directory of Open Access Journals (Sweden)

    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. 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

  14. 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

  15. 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...

  16. Contrast-enhanced spectral mammography in recalls from the Dutch breast cancer screening program: validation of results in a large multireader, multicase study.

    Science.gov (United States)

    Lalji, U C; Houben, I P L; Prevos, R; Gommers, S; van Goethem, M; Vanwetswinkel, S; Pijnappel, R; Steeman, R; Frotscher, C; Mok, W; Nelemans, P; Smidt, M L; Beets-Tan, R G; Wildberger, J E; Lobbes, M B I

    2016-12-01

    Contrast-enhanced spectral mammography (CESM) is a promising problem-solving tool in women referred from a breast cancer screening program. We aimed to study the validity of preliminary results of CESM using a larger panel of radiologists with different levels of CESM experience. All women referred from the Dutch breast cancer screening program were eligible for CESM. 199 consecutive cases were viewed by ten radiologists. Four had extensive CESM experience, three had no CESM experience but were experienced breast radiologists, and three were residents. All readers provided a BI-RADS score for the low-energy CESM images first, after which the score could be adjusted when viewing the entire CESM exam. BI-RADS 1-3 were considered benign and BI-RADS 4-5 malignant. With this cutoff, we calculated sensitivity, specificity and area under the ROC curve. CESM increased diagnostic accuracy in all readers. The performance for all readers using CESM was: sensitivity 96.9 % (+3.9 %), specificity 69.7 % (+33.8 %) and area under the ROC curve 0.833 (+0.188). CESM is superior to conventional mammography, with excellent problem-solving capabilities in women referred from the breast cancer screening program. Previous results were confirmed even in a larger panel of readers with varying CESM experience. • CESM is consistently superior to conventional mammography • CESM increases diagnostic accuracy regardless of a reader's experience • CESM is an excellent problem-solving tool in recalls from screening programs.

  17. 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.

  18. 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.

  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. 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

  1. 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

  2. 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

  3. 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

  4. 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

  5. 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.

  6. 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.

  7. 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

  8. 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...

  9. [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.

  10. 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

  11. 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

  12. 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

  13. 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

  14. 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

  15. 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

  16. Large-Scale Assessment of Change in Student Achievement: Dutch Primary School Students' Results on Written Division in 1997 and 2004 as an Example

    Science.gov (United States)

    van den Heuvel-Panhuizen, Marja; Robitzsch, Alexander; Treffers, Adri; Koller, Olaf

    2009-01-01

    This article discusses large-scale assessment of change in student achievement and takes the study by Hickendorff, Heiser, Van Putten, and Verhelst (2009) as an example. This study compared the achievement of students in the Netherlands in 1997 and 2004 on written division problems. Based on this comparison, they claim that there is a performance…

  17. 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.

  18. 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.

  19. 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.

  20. 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.

  1. 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

  2. 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

  3. 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

  4. 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

  5. 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&...

  6. 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

  7. Bio-based economy in the Netherlands. Macro-economic outline of a large-scale introduction of green resources in the Dutch energy supply

    International Nuclear Information System (INIS)

    Van der Hoeven, D.

    2009-03-01

    The Bio-based Raw Materials Platform (PGG), part of the Energy Transition in The Netherlands, commissioned the Agricultural Economics Research Institute (LEI) and the Copernicus Institute of Utrecht University to conduct research on the macro-economic impact of large scale deployment of biomass for energy and materials in the Netherlands. Two model approaches were applied based on a consistent set of scenario assumptions: a bottom-up study including technoeconomic projections of fossil and bio-based conversion technologies and a topdown study including macro-economic modelling of (global) trade of biomass and fossil resources. The results of the top-down and bottom-up modelling work are reported separately. This is the public version of studies [nl

  8. Final Results from Mexnext-I: Analysis of detailed aerodynamic measurements on a 4.5 m diameter rotor placed in the large German Dutch Wind Tunnel DNW

    International Nuclear Information System (INIS)

    Schepers, J G; Boorsma, K; Munduate, X

    2014-01-01

    The paper presents the final results from the first phase of IEA Task 29 'Mexnext'. Mexnext was a joint project in which 20 parties from 11 different countries cooperated. The main aim of Mexnext was to analyse the wind tunnel measurements which have been taken in the EU project 'MEXICO'. In the MEXICO project 10 institutes from 6 countries cooperated in doing experiments on an instrumented, 3 bladed wind turbine of 4.5 m diameter placed in the 9.5 by 9.5 m 2 open section of the Large Low-speed Facility (LLF) of DNW in the Netherlands. Pressure distributions on the blades were obtained from 148 Kulite pressure sensors, distributed over 5 sections at 25, 35, 60, 82 and 92 % radial position respectively. Blade loads were monitored through two strain-gauge bridges at each blade root. Most interesting however are the extensive PIV flow field measurements, which have been taken simultaneously with the pressure and load measurements. As a result of the international collaboration within this task a very thorough analysis of the data could be carried out and a large number of codes were validated not only in terms of loads but also in terms of underlying flow field. The paper will present several results from Mexnext-I, i.e. validation results and conclusion on modelling deficiencies and directions for model improvement. The future plans of the Mexnext consortium are also briefly discussed. Amongst these are Mexnext-II, a project in which also aerodynamic measurements other than MEXICO are included, and 'New MEXICO' in which additional measurement on the MEXICO model are performed

  9. 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

  10. 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

  11. 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

  12. 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

  13. 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

  14. 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

  15. '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

  16. 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

  17. 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

  18. 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

  19. 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

  20. 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

  1. 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

  2. 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.

  3. 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.

  4. 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.

  5. 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.

  6. 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.)

  7. 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.)

  8. 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 ...

  9. 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/ ).

  10. 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.

  11. Afrikaans and Dutch as closely-related languages: A comparison to West Germanic languages and Dutch dialects

    Directory of Open Access Journals (Sweden)

    Wilbert Heeringa

    2015-02-01

    Full Text Available Following Den Besten’s (2009 desiderata for historical linguistics of Afrikaans, this article aims to contribute some modern evidence to the debate regarding the founding dialects of Afrikaans. From an applied perspective (i.e. human language technology, we aim to determine which West Germanic language(s and/or dialect(s would be best suited for the purposes of recycling speech resources for the benefit of developing speech technologies for Afrikaans. Being recognised as a West Germanic language, Afrikaans is first compared to Standard Dutch, Standard Frisian and Standard German. Pronunciation distances are measured by means of Levenshtein distances. Afrikaans is found to be closest to Standard Dutch. Secondly, Afrikaans is compared to 361 Dutch dialectal varieties in the Netherlands and North-Belgium, using material from the Reeks Nederlandse Dialectatlassen, a series of dialect atlases compiled by Blancquaert and Pée in the period 1925-1982 which cover the Dutch dialect area. Afrikaans is found to be closest to the South-Holland dialectal variety of Zoetermeer; this largely agrees with the findings of Kloeke (1950. No speech resources are available for Zoetermeer, but such resources are available for Standard Dutch. Although the dialect of Zoetermeer is significantly closer to Afrikaans than Standard Dutch is, Standard Dutch speech resources might be a good substitute.

  12. Competition in the Dutch mortgage market: notes on concentration, entry, funding, and margins

    NARCIS (Netherlands)

    Treur, L.; Boonstra, W.W.

    2014-01-01

    This article describes the institutions that shape the Dutch mortgage market, as well as how they affect mortgage funding, and, ultimately, mortgage interest rates. The Dutch housing market is characterized by a very large social housing sector, as well as a relatively large share of owner-occupied

  13. 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

  14. 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…

  15. 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.

  16. 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

  17. 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

  18. 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

  19. 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

  20. 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.

  1. 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.

  2. 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

  3. 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.

  4. 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.

  5. 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.

  6. 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

  7. Strategic and structural responses to international dynamics in the open Dutch economy, 1963-2003

    NARCIS (Netherlands)

    de Jong, Abe; Sluyterman, Keetie; Westerhuis, Gerarda

    2011-01-01

    This paper investigates the strategies, structures and performance of large Dutch firms in the period 1963-2003, and compares the results with those of other European companies. Did Dutch companies develop corporate strategies and structures comparable to other European companies in response to the

  8. Social housing in the Netherlands : The development of the Dutch social housing model

    NARCIS (Netherlands)

    Hoekstra, J.S.C.M.

    2013-01-01

    The Dutch model of social rental housing is often seen as a good practice for other European coun-tries. This is due to the fact that the Dutch social rental sector has a large size, offers dwellings of a relatively good quality and functions without receiving substantial subsidies. However, current

  9. Creating a Dutch testbed to evaluate the retrieval from textual databases

    NARCIS (Netherlands)

    Hiemstra, Djoerd; van Leeuwen, David A.

    This paper describes the first large-scale evaluation of information retrieval systems using Dutch documents and queries. We describe in detail the characteristics of the Dutch test data, which is part of the official CLEF multilingual texttual database, and give an overview of the experimental

  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. 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.

  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. 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.)

  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. 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

  17. 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.

  18. 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.

  19. 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.

  20. 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.

  1. Plasma methoxytyramine: a novel biomarker of metastatic pheochromocytoma and paraganglioma in relation to established risk factors of tumour size, location and SDHB mutation status.

    Science.gov (United States)

    Eisenhofer, Graeme; Lenders, Jacques W M; Siegert, Gabriele; Bornstein, Stefan R; Friberg, Peter; Milosevic, Dragana; Mannelli, Massimo; Linehan, W Marston; Adams, Karen; Timmers, Henri J; Pacak, Karel

    2012-07-01

    There are currently no reliable biomarkers for malignant pheochromocytomas and paragangliomas (PPGLs). This study examined whether measurements of catecholamines and their metabolites might offer utility for this purpose. Subjects included 365 patients with PPGLs, including 105 with metastases, and a reference population of 846 without the tumour. Eighteen catecholamine-related analytes were examined in relation to tumour location, size and mutations of succinate dehydrogenase subunit B (SDHB). Receiver-operating characteristic curves indicated that plasma methoxytyramine, the O-methylated metabolite of dopamine, provided the most accurate biomarker for discriminating patients with and without metastases. Plasma methoxytyramine was 4.7-fold higher in patients with than without metastases, a difference independent of tumour burden and the associated 1.6- to 1.8-fold higher concentrations of norepinephrine and normetanephrine. Increased plasma methoxytyramine was associated with SDHB mutations and extra-adrenal disease, but was also present in patients with metastases without SDHB mutations or those with metastases secondary to adrenal tumours. High risk of malignancy associated with SDHB mutations reflected large size and extra-adrenal locations of tumours, both independent predictors of metastatic disease. A plasma methoxytyramine above 0.2nmol/L or a tumour diameter above 5cm indicated increased likelihood of metastatic spread, particularly when associated with an extra-adrenal location. Plasma methoxytyramine is a novel biomarker for metastatic PPGLs that together with SDHB mutation status, tumour size and location provide useful information to assess the likelihood of malignancy and manage affected patients. Copyright © 2011 Elsevier Ltd. All rights reserved.

  2. Plasma methoxytyramine: A novel biomarker of metastatic pheochromocytoma and paraganglioma in relation to established risk factors of tumor size, location and SDHB mutation status

    Science.gov (United States)

    Eisenhofer, Graeme; Lenders, Jacques W.M.; Siegert, Gabriele; Bornstein, Stefan R.; Friberg, Peter; Milosevic, Dragana; Mannelli, Massimo; Linehan, W. Marston; Adams, Karen; Timmers, Henri J.; Pacak, Karel

    2012-01-01

    Summary Background There are currently no reliable biomarkers for malignant pheochromocytomas and paragangliomas (PPGLs). This study examined whether measurements of catecholamines and their metabolites might offer utility for this purpose. Methods Subjects included 365 patients with PPGLs, including 105 with metastases, and a reference population of 846 without the tumor. Eighteen catecholamine-related analytes were examined in relation to tumor location, size and mutations of succinate dehydrogenase subunit B (SDHB). Results Receiver-operating characteristic curves indicated that plasma methoxytyramine, the O-methylated metabolite of dopamine, provided the most accurate biomarker for discriminating patients with and without metastases. Plasma methoxytyramine was 4.7-fold higher in patients with than without metastases, a difference independent of tumor burden and the associated 1.6- to 1.8-fold higher concentrations of norepinephrine and normetanephrine. Increased plasma methoxytyramine was associated with SDHB mutations and extra-adrenal disease, but was also present in patients without SDHB mutations and metastases or those with metastases secondary to adrenal tumors. High risk of malignancy associated with SDHB mutations reflected large size and extra-adrenal locations of tumors, both independent predictors of metastatic disease. A plasma methoxytyramine above 0.2 nmol/L or a tumor diameter above 5 cm indicated increased likelihood of metastatic spread, particularly when associated with an extra-adrenal location. Interpretation Plasma methoxytyramine is a novel biomarker for metastatic PPGLs that together with SDHB mutation status, tumor size and location provide useful information to assess the likelihood of malignancy and manage affected patients. PMID:22036874

  3. Time for EU Matters: The Europeanization of Dutch Central Government

    NARCIS (Netherlands)

    Mastenbroek, E.; Princen, S.B.M.

    2010-01-01

    To what extent does the European Union (EU) affect national governments? This article seeks to answer this question by assessing the Europeanization of Dutch central government. Using data from a large-scale survey among civil servants, we assess to what extent the EU affects the structure and

  4. Energy productivity growth in the Dutch Greenhouse Industry

    NARCIS (Netherlands)

    Oude Lansink, A.G.J.M.; Ondersteijn, C.J.M.

    2006-01-01

    Profitability of Dutch greenhouse firms is largely dependent on energy costs, and policy makers focus on reducing the use of energy by these firms. This article uses Russell measures of TE to develop indicators of energy productivity growth. Results show that energy productivity grew by 2.8%

  5. Innovative quality management practices in the Dutch construction industry

    NARCIS (Netherlands)

    Bossink, B.A.G.

    2002-01-01

    Quality management practices do support the management of strategically important innoυation processes. This conclusion is based on empirical research in the Dutch construction industry. A large-scale innovative construction project is intensively studied during a three-year period. The quality

  6. Two Profiles of the Dutch High Performing Employee

    Science.gov (United States)

    de Waal, A. A.; Oudshoorn, Michella

    2015-01-01

    Purpose: The purpose of this study is to explore the profile of an ideal employee, to be more precise the behavioral characteristics of the Dutch high-performing employee (HPE). Organizational performance depends for a large part on the commitment of employees. Employees provide their knowledge, skills, experiences and creativity to the…

  7. How Dutch employees experience freedom of learning for work

    NARCIS (Netherlands)

    van Dellen, Theo; Heidekamp, Ina

    2015-01-01

    This article focuses on the perceived freedom of Dutch employees to embark on workplace learning in terms of whether they feel it is“voluntary” or “compulsory”. The paper is based on the findings of a large international explorative survey carried out by the Workplace Learning (WPL) Research Network

  8. How Dutch Employees Experience Freedom of Learning for Work

    Science.gov (United States)

    van Dellen, Theo; Heidekamp, Ina

    2015-01-01

    This article focuses on the perceived freedom of Dutch employees to embark on workplace learning in terms of whether they feel it is "voluntary" or "compulsory". The paper is based on the findings of a large international explorative survey carried out by the Workplace Learning (WPL) Research Network (RN2) of the Asia-Europe…

  9. 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

  10. 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...

  11. The Dutch Pancreas Biobank within the Parelsnoer Institute : A nationwide Biobank of pancreatic and periampullary diseases

    NARCIS (Netherlands)

    Strijker, Marin; Gerritsen, Arja; Van Hilst, Jony; Bijlsma, Maarten F.; Bonsing, Bert A.; Brosens, Lodewijk A.; Bruno, Marco J.; Van Dam, Ronald M.; Dijk, Frederike; Van Eijck, Casper H.; Farina Sarasqueta, Arantza; Fockens, Paul; Gerhards, Michael F.; Groot Koerkamp, Bas; Van Der Harst, Erwin; De Hingh, Ignace H.; Van Hooft, Jeanin E.; Huysentruyt, Clément J.; Kazemier, Geert; Klaase, Joost M.; Van Laarhoven, Cornelis J.; Van Laarhoven, Hanneke W.; Liem, Mike S.; De Meijer, Vincent E.; Van Rijssen, L. Bengt; Van Santvoort, Hjalmar C.; Suker, Mustafa; Verhagen, Judith H.; Verheij, Joanne; Verspaget, Hein W.; Wennink, Roos A.; Wilmink, Johanna W.; Molenaar, I. Quintus; Boermeester, Marja A.; Busch, Olivier R.; Besselink, Marc G.

    2018-01-01

    Objectives Large biobanks with uniform collection of biomaterials and associated clinical data are essential for translational research. The Netherlands has traditionally been well organized in multicenter clinical research on pancreatic diseases, including the nationwide multidisciplinary Dutch

  12. The Dutch Pancreas Biobank Within the Parelsnoer Institute : A Nationwide Biobank of Pancreatic and Periampullary Diseases

    NARCIS (Netherlands)

    Strijker, Marin; Gerritsen, Arja; van Hilst, Jony; Bijlsma, Maarten F; Bonsing, Bert A; Brosens, Lodewijk A; Bruno, Marco J; van Dam, Ronald M; Dijk, Frederike; van Eijck, Casper H; Farina Sarasqueta, Arantza; Fockens, Paul; Gerhards, Michael F; Groot Koerkamp, Bas; van der Harst, Erwin; de Hingh, Ignace H; van Hooft, Jeanin E; Huysentruyt, Clément J; Kazemier, Geert; Klaase, Joost M; van Laarhoven, Cornelis J; van Laarhoven, Hanneke W; Liem, Mike S; de Meijer, Vincent E; van Rijssen, L Bengt; van Santvoort, Hjalmar C; Suker, Mustafa; Verhagen, Judith H; Verheij, Joanne; Verspaget, Hein W; Wennink, Roos A; Wilmink, Johanna W; Molenaar, I Quintus; Boermeester, Marja A; Busch, Olivier R; Besselink, Marc G

    OBJECTIVES: Large biobanks with uniform collection of biomaterials and associated clinical data are essential for translational research. The Netherlands has traditionally been well organized in multicenter clinical research on pancreatic diseases, including the nationwide multidisciplinary Dutch

  13. o. Setting. On Reconstructing Variation in Cape Dutch (1710-1840 ...

    African Journals Online (AJOL)

    European languages in an extraterritorial ... extraterritorial varieties of language that developed from contact of diverse metropolitan dialects (and ..... The Netherlandic perfect auxiliary system remains largely intact in mesolectal Cape Dutch, although.

  14. 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

  15. 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

  16. 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

  17. 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.

  18. 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

  19. 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

  20. 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

  1. 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.

  2. 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

  3. The impact of microgeneration upon the Dutch balancing market

    International Nuclear Information System (INIS)

    Van der Veen, Reinier A.C.; De Vries, Laurens 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 changes to the design of the Dutch balancing market that can maintain or even improve upon its current operational performance level. The first step of the research was an analysis of the existing Dutch balancing market. It consists of three main instruments: programme responsibility, the single buyer market for regulating and reserve power (RRP), and imbalance settlement. The balancing market currently functions satisfactorily. Subsequently, the effects of large-scale development of microgeneration in the Netherlands were evaluated with a qualitative scenario analysis. Four microgeneration scenarios and two methods for allocating the household electricity consumption and generation were considered. The four scenarios concerned large-scale penetration of PV, heat-led micro CHP, electricity-led micro CHP operated by the household consumer, and electricity-led micro CHP operated by the supply company. The last scenario was found to have the strongest positive net effect. Finally, six design options were identified for improving the Dutch balancing market design in case the share of microgeneration would increase substantially. Of these six options, adjusting the profile methodology and the regulation of smart meters are no-regret options that can be implemented immediately. The attractiveness of the other options depends upon the microgeneration portfolio that emerges, the manageability of large metering data flows, and the nature of the technical effects of large-scale microgeneration penetration.

  4. 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

  5. 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

  6. 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

  7. 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

  8. 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

  9. 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.

  10. 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

  11. 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

  12. 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 &

  13. 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

  14. 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

  15. 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

  16. 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

  17. Incidence of repeat testing and diagnoses of Chlamydia trachomatis and Neisseria gonorrhoea in swingers, homosexual and heterosexual men and women at two large Dutch STI clinics, 2006-2013.

    Science.gov (United States)

    Dukers-Muijrers, Nicole H T M; van Rooijen, Martijn S; Hogewoning, Arjan; van Liere, Genevieve A F S; Steenbakkers, Mieke; Hoebe, Christian J P A

    2017-09-01

    Swingers, that is, heterosexuals who as a couple have sex with others, including group sex and bisexual behaviour, are an older-aged risk group for STIs. Here, we report on their repeat testing (reattendance) and STI yield compared with other heterosexuals and men who have sex with men (MSM, homosexual men) at two Dutch STI clinics. Swingers are routinely (since 2006, South Limburg, registration-completeness: 99%) or partially (since 2010, Amsterdam, registration-completeness: 20%) included in the clinic patient registries. Data (retrospective cohort) are analysed to assess incidence (per 100 person-years (PY)) of reattendance and STI ( Chlamydia trachomatis (CT) and/or Neisseria gonorrhoeae (NG)) and associated factors calculating HRs. In South Limburg 7714 and in Amsterdam 2070 swinger consultations were identified. Since 2010, swingers' incidence of reattendance was 48-57/100 PY. Incidence was lower in MSM (30-39/100 PY, HR 0.56; 95% CI 0.51 to 0.61, South Limburg; HR 0.88; 95% CI 0.80 to 0.96, Amsterdam), heterosexual men (8-14/100 PY, HR 0.16; 95% CI 0.15 to 0.17, South Limburg; HR 0.33; 95% CI 0.30 to 0.36, Amsterdam) and women (13-20/100 PY, HR 0.56; 95% CI 0.51 to 0.61, South Limburg; HR 0.46; 95% CI 0.42 to 0.51, Amsterdam). Swingers' STI incidence at reattendance was 11-12/100 PY. Incidence was similar in heterosexual men (14-15/100 PY; HR 1.19; 95% CI 0.90 to 1.57, South Limburg; HR 1.20; 95% CI 0.91 to 1.59, Amsterdam) and women (12-14/100 PY; HR 1.14; 95% CI 0.88 to 1.49, South Limburg; HR 0.98; 95% CI 0.74 to 1.29, Amsterdam) and higher in MSM (18-22/100 PY; HR 1.59; 95% CI 1.19 to 2.12, South Limburg; HR 1.80; 95% CI 1.36 to 2.37, Amsterdam). Risk factors for STI incidence were partner-notified (contact-tracing), symptoms and previous STI. Swingers' positivity at any clinic attendance was 3-4% for NG (ie, higher than other heterosexuals) and 6-8% for CT (ie, lower than heterosexuals overall but higher than older heterosexuals

  18. Dutch virtual integration of healthcare information.

    Science.gov (United States)

    de Graaf, J C; Vlug, A E; van Boven, G J

    2007-01-01

    As information technology creates opportunities for cooperation which crosses the boundaries between healthcare institutions, it will become an integral part of the Dutch healthcare system. Along with many involved organizations in healthcare the National IT Institute for Healthcare in the Netherlands (NICTIZ) is working on the realization of a national IT infrastructure for healthcare and a national electronic patient record (EPR). An underlying national architecture is designed to enable the Dutch EPR virtually, not in a national database, nor on a patient's smartcard. The required secure infrastructure provides generic functions for healthcare applications: patient identification, authentication and authorization of healthcare professionals. The first national applications in the EPR program using a national index of where patient data is stored, are the electronic medication record and the electronic record for after hours GP services. The rollout of the electronic medication record and electronic record for after hours GP services has been started in 2007. To guarantee progress of electronic data exchange in healthcare in the Netherlands we have primarily opted for two healthcare applications: the electronic medication record and the electronic record for after hours GP services. The use of a national switch-point containing the registry of where to find what information, guarantees that the professional receives the most recent information and omits large databases to contain downloaded data. Proper authorization, authentication as well as tracing by the national switchpoint also ensures a secure environment for the communication of delicate information.

  19. 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

  20. Discord amongst the wind turbine buyers with regard to Dutch wind turbine manufacturers

    International Nuclear Information System (INIS)

    Janse, F.

    1992-01-01

    In the Netherlands two medium-large manufacturers of wind turbines exist: NedWind (Hollandia Kloos) and Windmaster Nederland (Begemann Groep). Also Lagerwey, which constructs wind turbines for the private market (farmers, cooperatives), is important for the Dutch market and successfull in Germany. There is a tendency amongst the Dutch energy utilities, which cooperate in the foundation WindPlan, to buy wind turbines from foreign manufacturers. Some energy utilities want to take advantage of the most attractive offers, while others want to cooperate in building up a Dutch wind turbine industry. 5 ills

  1. 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.

  2. 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

  3. 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.

  4. 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.

  5. Seasonal variation in the Dutch bovine raw milk composition.

    Science.gov (United States)

    Heck, J M L; van Valenberg, H J F; Dijkstra, J; van Hooijdonk, A C M

    2009-10-01

    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 components and milk fatty acid composition. Milk lactose concentration was rather constant throughout the season. Milk true protein content was somewhat more responsive to season, with the lowest content in June (3.21 g/100 g) and the highest content in December (3.38 g/100 g). Milk fat concentration increased from a minimum of 4.10 g/100 g in June to a maximum of 4.57 g/100 g in January. The largest (up to 2-fold) seasonal changes in the fatty acid composition were found for trans fatty acids, including conjugated linoleic acid. Milk protein composition was rather constant throughout the season. Milk unsaturation indices, which were used as an indication of desaturase activity, were lowest in spring and highest in autumn. Compared with a previous investigation of Dutch dairy milk in 1992, the fatty acid composition of Dutch raw milk has changed considerably, in particular with a higher content of saturated fatty acids in 2005 milk.

  6. ATLAS Experiment Brochure - Dutch

    CERN Multimedia

    AUTHOR|(CDS)2081027

    2018-01-01

    ATLAS is one of the four major experiments at the Large Hadron Collider at CERN. It is a general-purpose particle physics experiment run by an international collaboration, and is designed to exploit the full discovery potential and the huge range of physics opportunities that the LHC provides.

  7. Quality and safety of products containing Ephedra Herba on the Dutch market

    OpenAIRE

    Lake OA; Slijkhuis C; Maas WF; Vliet MEA van; Kaste D de; Verdonk-Kleinjan W; Keuringsdienst van Waren, regio Zuid; LGO

    2001-01-01

    We performed analytical studies on dietary supplements and smart products containing Ephedra herba on the Dutch market. Such products are labelled 'from natural, herbal sources' and do not fall under Dutch Medicines Act. Most of the samples tested from 1993 to 1999 contained unacceptably large amounts of ephedrine (EP) alkaloids (the active substances of Ephedra herba) in relation to the safety criteria in the literature. Some samples also contained an effect-enhancing substance (e.g. coffein...

  8. The Dutch Pancreas Biobank Within the Parelsnoer Institute: A Nationwide Biobank of Pancreatic and Periampullary Diseases.

    Science.gov (United States)

    Strijker, Marin; Gerritsen, Arja; van Hilst, Jony; Bijlsma, Maarten F; Bonsing, Bert A; Brosens, Lodewijk A; Bruno, Marco J; van Dam, Ronald M; Dijk, Frederike; van Eijck, Casper H; Farina Sarasqueta, Arantza; Fockens, Paul; Gerhards, Michael F; Groot Koerkamp, Bas; van der Harst, Erwin; de Hingh, Ignace H; van Hooft, Jeanin E; Huysentruyt, Clément J; Kazemier, Geert; Klaase, Joost M; van Laarhoven, Cornelis J; van Laarhoven, Hanneke W; Liem, Mike S; de Meijer, Vincent E; van Rijssen, L Bengt; van Santvoort, Hjalmar C; Suker, Mustafa; Verhagen, Judith H; Verheij, Joanne; Verspaget, Hein W; Wennink, Roos A; Wilmink, Johanna W; Molenaar, I Quintus; Boermeester, Marja A; Busch, Olivier R; Besselink, Marc G

    2018-04-01

    Large biobanks with uniform collection of biomaterials and associated clinical data are essential for translational research. The Netherlands has traditionally been well organized in multicenter clinical research on pancreatic diseases, including the nationwide multidisciplinary Dutch Pancreatic Cancer Group and Dutch Pancreatitis Study Group. To enable high-quality translational research on pancreatic and periampullary diseases, these groups established the Dutch Pancreas Biobank. The Dutch Pancreas Biobank is part of the Parelsnoer Institute and involves all 8 Dutch university medical centers and 5 nonacademic hospitals. Adult patients undergoing pancreatic surgery (all indications) are eligible for inclusion. Preoperative blood samples, tumor tissue from resected specimens, pancreatic cyst fluid, and follow-up blood samples are collected. Clinical parameters are collected in conjunction with the mandatory Dutch Pancreatic Cancer Audit. Between January 2015 and May 2017, 488 patients were included in the first 5 participating centers: 4 university medical centers and 1 nonacademic hospital. Over 2500 samples were collected: 1308 preoperative blood samples, 864 tissue samples, and 366 follow-up blood samples. Prospective collection of biomaterials and associated clinical data has started in the Dutch Pancreas Biobank. Subsequent translational research will aim to improve treatment decisions based on disease characteristics.

  9. Landfill gas in the Dutch perspective

    International Nuclear Information System (INIS)

    Scheepers, M.J.J.

    1991-01-01

    Until 1986 landfill gas had a considerable value because of the relative high energy prices. It appeared also that landfill gas was formed in large quantities. However after the collapse of the energy prices in 1986 many new landfill gas projects were delayed or stopped. Recently, the gas emissions on landfills have attracted attention again, but now because of various environmental aspects. With respect to landfill management a well controlled gas extraction seems to be necessary. Utilisation of the gas is still favourable for economic reasons and because of energy savings. The Dutch policy for the next ten years will be reduction of the amount of waste by prevention and recycling. The organic fraction of the municipal solid waste (refuse from vegetables, fruit and garden), obtained by separation in households, will be composted. The other part will be burnt in incinerators. Only the remaining inert refuse will be deposited on landfills. (author)

  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. A Dutch Fanconi Anemia FANCC Founder Mutation in Canadian Manitoba Mennonites

    Directory of Open Access Journals (Sweden)

    Yne de Vries

    2012-01-01

    Full Text Available Fanconi anemia (FA is a recessive DNA instability disorder associated with developmental abnormalities, bone marrow failure, and a predisposition to cancer. Based on their sensitivity to DNA cross-linking agents, FA cells have been assigned to 15 complementation groups, and the associated genes have been identified. Founder mutations have been found in different FA genes in several populations. The majority of Dutch FA patients belongs to complementation group FA-C. Here, we report 15 patients of Dutch ancestry and a large Canadian Manitoba Mennonite kindred carrying the FANCC c.67delG mutation. Genealogical investigation into the ancestors of the Dutch patients shows that these ancestors lived in four distinct areas in The Netherlands. We also show that the Dutch and Manitoba Mennonite FANCC c.67delG patients share the same haplotype surrounding this mutation, indicating a common founder.

  18. Bruxism and associated factors among Dutch adolescents.

    Science.gov (United States)

    van Selms, Maurits K A; Visscher, Corine M; Naeije, Machiel; Lobbezoo, Frank

    2013-08-01

    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. In a cross-sectional questionnaire survey, 4285 questionnaires were completed, with an about equal gender distribution and with ages ranging from 10 to 22 years. In the group of 4235 12- to 18-year-old adolescents, sleep bruxism had a reported prevalence of 14.8% and awake bruxism of 8.7%. Logistic regression analyses revealed that sleep bruxism was associated with female gender [OR = 1.49 (95% CI = 1.23-1.81)], pain or tense feeling in the jaws upon awakening in the morning [OR = 1.47 (95% CI = 1.17-1.86)], clicking joint sounds [OR = 1.31 (95% CI = 1.03-1.65)], stress [OR = 1.25 (95% CI = 1.00-1.55)], and depressive mood [OR = 1.35 (95% CI = 1.10-1.65)]. Awake bruxism was associated with orofacial pain [OR = 1.49 (95% CI = 1.16-1.91)], clicking joint sounds [OR = 1.50 (95% CI = 1.13-1.98)], scraping joint sounds [OR = 2.03 (95% CI = 1.21-3.37)], stress [OR = 1.36 (95% CI = 1.03-1.78)], depressive mood [OR = 1.82 (95% CI = 1.42-2.35)], and smoking [OR = 1.42 (95% CI = 1.06-1.89)]. Sleep bruxism and awake bruxism are common conditions among Dutch adolescents, with self-reported prevalence rates that are slightly higher than those derived from most large-scale studies on adults. Several predictor variables were found to be exclusively associated with either form of bruxism, corroborating the common suggestion that both circadian manifestations are, at least in part, different entities. © 2012 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  19. [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.

  20. 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

  1. 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

  2. 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

  3. 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

  4. 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

  5. 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

  6. 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

  7. 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.

  8. 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.

  9. 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.

  10. 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

  11. New rate structure of the Dutch natural gas trading company Gasunie

    International Nuclear Information System (INIS)

    Koevoet, H.

    1999-01-01

    Starting January 1, 2000, many of Gasunie's large-scale consumers will have to deal with a new rate structure: the CDS (Dutch abbreviation for Commodity Services System). Unlike in the old zone system, the consumption profile is of great importance in the CDS. Those who must heat large spaces have a particular interest in sizing up the consequences of the CDS

  12. 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

  13. Dutch food bank recipients have poorer dietary intakes than the general and low-socioeconomic status Dutch adult population.

    Science.gov (United States)

    Neter, J E; Dijkstra, S C; Dekkers, A L M; Ocké, M C; Visser, M; Brouwer, I A

    2017-10-03

    Food-assistance program users are a specific group of nutritional concern, as they are often food insufficient and have poorer diet quality compared to non-food-assistance program users. The aim of our study was to assess dietary intake of Dutch food bank recipients (n = 167) and to compare this with dietary intake of a representative sample of the general population (Dutch National Food Consumption Survey (DNFCS-all): n = 1933), including a low-socioeconomic status (SES) sample (DNFCS-low SES: n = 312), using data from the DNFCS 2007-2010. In this cross-sectional study, 12 food banks throughout The Netherlands participated. Food bank recipients' characteristics were assessed with a self-administered questionnaire. Dietary intake data were collected through three 24-h recalls. Habitual dietary intake (mean, percentiles, and 95% CI) was estimated for all samples. Differences between samples were determined by comparing the 95% CIs. Mean age of the study population (62.9% female) was 48.6 years (SD:10.1). Mean energy intake was 1986 (95% CI 1830-2089) kcal. The majority of the Dutch food bank recipients had lower intakes than dietary reference intakes for dietary fiber, fruit, vegetables, and fish (range 86.6-99.3%), and a higher intake for saturated fat [88.1% (95% CI 84.1-98.9)]. Furthermore, mean intakes of energy, fiber, fruit, and vegetables were significantly lower in Dutch food bank recipients than in the DNFCS-all and the DNFCS-low-SES [e.g., daily mean fruit intake (g) food bank recipients 62.8 (95% CI 45.5-76.5), DNFCS-all 105.8 (95% CI 105.4-117.9), and DNFCS-low-SES 85.1 (95% CI 78.7-100.2)]. Fish intake was significantly lower compared with the DNFCS-all, but not compared with the DNFCS-low-SES. Dutch food bank recipients, who largely rely on the content of food parcels, are not able to meet the nutritional guidelines for a healthy diet, and their dietary intake is poorer than the general as well as the low-SES sample of the Dutch adult population

  14. 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:

  15. Growing up with Frisian and Dutch: The role of language input in the early development of Frisian and Dutch among preschool children in Friesland

    NARCIS (Netherlands)

    Dijkstra, J.E.

    2013-01-01

    Bilingual acquisition largely depends on the input that children receive in each language. The more input in a language, the more proficient a child becomes in that language. The current project studied the role of language input among bilingual Frisian-Dutch preschool children (age 2.5-4 years) in

  16. Evaluating congestion management in the Dutch electricity transmission grid

    International Nuclear Information System (INIS)

    Blijswijk, Martti J. van; Vries, Laurens J. de

    2012-01-01

    Due to the increase in electricity generation capacity in the Netherlands and a new connection policy, transmission system operator (TSO) TenneT expects a significant increase in congestion in the Dutch transmission grid. To manage this, the Dutch government implemented redispatching, a method which is argued in the literature to potentially impose large congestion costs upon the TSO. A quantitative model of the Dutch electricity system was developed in order to evaluate this method. The outcomes were compared to the performance of three alternative congestion management methods. Regardless of the method, congestion costs were found to be substantially lower than in previous studies. Because combined-cycle gas turbines are the marginal generation technology in almost all cases, the costs of up and down regulation do not differ much. Consequently, the redispatching costs for the TSO are expected to be relatively low, and the opportunities for abuse of market power appear to be limited. While all the evaluated methods are effective and economically efficient, they have significantly different welfare effects. Market splitting creates significantly larger welfare effects than the different varieties of redispatching. - Highlights: ► Congestion management was recently introduced in the Netherlands. ► We quantitatively evaluate the effects of its application. ► We compare this to other congestion management methods. ► Given the specific situation that the marginal cost curve of production is flat, congestion costs are expected to be low.

  17. 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.

  18. 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.

  19. 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.

  20. 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.

  1. 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.

  2. 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.

  3. 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

  4. 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.

  5. Nationwide prospective audit of pancreatic surgery: design, accuracy, and outcomes of the Dutch Pancreatic Cancer Audit.

    Science.gov (United States)

    van Rijssen, L Bengt; Koerkamp, Bas G; Zwart, Maurice J; Bonsing, Bert A; Bosscha, Koop; van Dam, Ronald M; van Eijck, Casper H; Gerhards, Michael F; van der Harst, Erwin; de Hingh, Ignace H; de Jong, Koert P; Kazemier, Geert; Klaase, Joost; van Laarhoven, Cornelis J; Molenaar, I Quintus; Patijn, Gijs A; Rupert, Coen G; van Santvoort, Hjalmar C; Scheepers, Joris J; van der Schelling, George P; Busch, Olivier R; Besselink, Marc G

    2017-10-01

    Auditing is an important tool to identify practice variation and 'best practices'. The Dutch Pancreatic Cancer Audit is mandatory in all 18 Dutch centers for pancreatic surgery. Performance indicators and case-mix factors were identified by a PubMed search for randomized controlled trials (RCT's) and large series in pancreatic surgery. In addition, data dictionaries of two national audits, three institutional databases, and the Dutch national cancer registry were evaluated. Morbidity, mortality, and length of stay were analyzed of all pancreatic resections registered during the first two audit years. Case ascertainment was cross-checked with the Dutch healthcare inspectorate and key-variables validated in all centers. Sixteen RCT's and three large series were found. Sixteen indicators and 20 case-mix factors were included in the audit. During 2014-2015, 1785 pancreatic resections were registered including 1345 pancreatoduodenectomies. Overall in-hospital mortality was 3.6%. Following pancreatoduodenectomy, mortality was 4.1%, Clavien-Dindo grade ≥ III morbidity was 29.9%, median (IQR) length of stay 12 (9-18) days, and readmission rate 16.0%. In total 97.2% of >40,000 variables validated were consistent with the medical charts. The Dutch Pancreatic Cancer Audit, with high quality data, reports good outcomes of pancreatic surgery on a national level. Copyright © 2017 International Hepato-Pancreato-Biliary Association Inc. Published by Elsevier Ltd. All rights reserved.

  6. Persistent Identifiers for Dutch cultural heritage institutions

    Science.gov (United States)

    Ras, Marcel; Kruithof, Gijsbert

    2016-04-01

    Over the past years, more and more collections belonging to archives, libraries, media, museums, and knowledge institutes are being digitised and made available online. These are exciting times for ALM institutions. They are realising that, in the information society, their collections are goldmines. Unfortunately most heritage institutions in the Netherlands do not yet meet the basic preconditions for long-term availability of their collections. The digital objects often have no long lasting fixed reference yet. URL's and web addresses change. Some digital objects that were referenced in Europeana and other portals can no longer be found. References in scientific articles have a very short life span, which is damaging for scholarly research. In 2015, the Dutch Digital Heritage Network (NDE) has started a two-year work program to co-ordinate existing initiatives in order to improve the (long-term) accessibility of the Dutch digital heritage for a wide range of users, anytime, anyplace. The Digital Heritage Network is a partnership established on the initiative of the Ministry of Education, Culture and Science. The members of the NDE are large, national institutions that strive to professionally preserve and manage digital data, e.g. the National Library, The Netherlands Institute for Sound and Vision, the Netherlands Cultural Heritage Agency, the Royal Netherlands Academy of Arts and Sciences, the National Archive of the Netherlands and the DEN Foundation, and a growing number of associations and individuals both within and outside the heritage sector. By means of three work programmes the goals of the Network should be accomplished and improve the visibility, the usability and the sustainability of digital heritage. Each programme contains of a set of projects. Within the sustainability program a project on creating a model for persistent identifiers is taking place. The main goals of the project are (1) raise awareness among cultural heritage institutions on the

  7. Economic Impact of the Dutch Gas Hub Strategy on the Netherlands

    International Nuclear Information System (INIS)

    Harris, D.; Bazelon, C.; Humphreys, B.; Dickson, P.

    2010-09-01

    Since the discovery of the Groningen gas field in 1959, the Netherlands has been a key player in the European gas market. The Netherlands has built up a large onshore and offshore Exploration and Production sector, it has a highly developed gas transmission and distribution network, and is a major exporter of gas to other EU Member States. The Netherlands has considerable expertise in all parts of the gas supply chain, and is a world centre for Research and development in natural gas supply and use. More recently construction has begun on terminals to allow the import of Liquid Natural Gas to the Netherlands. However, Dutch gas reserves are now in decline, and the Netherlands will become a net importer of gas around 2025. The Dutch government wishes to capitalise on the existing industry and skills and sustain the Netherlands' place in the European gas industry beyond the life of the existing gas fields. In November 2009 the Minister of Economic Affairs published a paper describing the strategy of turning the Netherlands into a 'Gas Hub' or 'gas roundabout' for north-west Europe. The Dutch government intends that the gas hub would capitalise on the existing skills and industry, increase competition and security of supply in the Dutch gas market, create employment and make a significant contribution to the Dutch economy. The Dutch Ministry of Economic Affairs, Agriculture and Innovation has commissioned The Brattle Group to perform an analysis of the economic impact of the gas hub concept. The study has several aims including: To analyse the current contribution of the Dutch gas sector to the economy; To assess the Strengths, Weaknesses, Opportunities and Threats associated with the gas hub strategy; To quantify the benefits of the gas hub strategy to the Dutch economy. Note that the aim of the study is to define in more detail what a successful Dutch gas hub strategy would look like, to assess the strengths and weakness of the Dutch gas sector in achieving a

  8. Palliative sedation largely in accordance with Dutch national guideline

    NARCIS (Netherlands)

    Swart, S. J.; Rietjens, J. A.; Brinkkemper, T.; Zuylen, L. van; Burg-Verhage, W. A. van; Zuurmond, W. W.; Ribbe, M. W.; Blanker, M. H.; Perez, R. S.; Heide, A. van der

    2011-01-01

    OBJECTIVE: To evaluate the practice of continuous palliative sedation after the introduction of a national guideline. DESIGN: Investigation by questionnaire. METHOD: In 2008, 1580 physicians were asked to fill out a questionnaire regarding the last patient for whom they had prescribed continuous

  9. On a large specimen of Orthragoriscus on the Dutch Coast

    NARCIS (Netherlands)

    Lidth de Jeude, van Th.W.

    1890-01-01

    On the 13th of Dec. 1889 a great Sun-fish was washed ashore at Ameland. Through kind intervention of the mayor of the island, D. W. J. baron van Heeckeren , the gigantic fish was sent up to the Leyden Museum, where it arrived a few days afterwards in a satisfactory state. As far as I know of, it is

  10. 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

  11. 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…

  12. 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

  13. 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

  14. 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

  15. 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...

  16. Ideology, politics, and personality: shaping forces in Dutch psychology of religion, 1907-1957.

    Science.gov (United States)

    Belzen, Jacob A

    2009-08-01

    Although the academic establishment of the psychology of religion in the Netherlands has been stronger than in any other Western country, the start of these developments has been remarkably late (in 1957), especially when taking into account that Dutch academic life: (1) before World War II modeled itself after Germany (where psychology of religion flourished); and (2) was to a considerable extent included in the system of pillarization, which characterized Dutch society at large. The general factors that can be distinguished as having played an important role in the shaping of the situation for psychology of religion in the Netherlands had different impacts in the several universities under consideration.

  17. A Metrical Theory of Stress and Destressing in English and Dutch

    NARCIS (Netherlands)

    Kager, R.W.J.

    1989-01-01

    The topic of this study is word stress, more specifically the relation between rules of stress and destressing within the framework of metrical phonology. Our claims will be largely based on in-depth analyses of two word stress systems: those of English and Dutch. We intend to offer a

  18. The impact of farm size on sustainability of dutch dairy farms

    NARCIS (Netherlands)

    Meulen, van der H.A.B.; Dolman, M.A.; Jager, J.H.; Venema, G.S.

    2014-01-01

    Sustainable milk production systems require economically viable, environmentally sound and socially acceptable practices. This study compared the economic, environmental and societal impact of large-scale farms with other dairy farms in the Dutch Farm Accountancy Data Network (FADN). Moreover the

  19. Systematic screening for Chlamydia trachomatis : Estimating cost-effectiveness using dynamic modeling and Dutch data

    NARCIS (Netherlands)

    de Vries, R.; Van Bergen, J.E.A.M.; de Jong-van den Berg, Lolkje; Postma, Maarten

    2006-01-01

    To estimate the cost-effectiveness of a systematic one-off Chlamydia trachomatis (CT) screening program including partner treatment for Dutch young adults. Data on infection prevalence, participation rates, and sexual behavior were obtained from a large pilot study conducted in The Netherlands.

  20. Systematic screening for Chlamydia trachomatis: estimating cost-effectiveness using dynamic modeling and Dutch data

    NARCIS (Netherlands)

    de Vries, Robin; van Bergen, Jan E. A. M.; de Jong-van den Berg, Lolkje T. W.; Postma, Maarten J.

    2006-01-01

    To estimate the cost-effectiveness of a systematic one-off Chlamydia trachomatis (CT) screening program including partner treatment for Dutch young adults. Data on infection prevalence, participation rates, and sexual behavior were obtained from a large pilot study conducted in The Netherlands.

  1. Distinctly variable mudscapes: Distribution gradients of intertidal macrofauna across the Dutch Wadden Sea

    NARCIS (Netherlands)

    Compton, T.J.; Holthuijsen, S.; Koolhaas, A.; Dekinga, A.; ten Horn, J.; Smith, J.; Galama, Y.; Brugge, M.; van der Wal, D.; van der Meer, J.; van der Veer, H.W.; Piersma, T.

    2013-01-01

    The Wadden Sea is a shallow coastal region, with a large area of sedimentary tidal flats that extends from The Netherlands to Denmark and has been declared a site of international importance in the Dutch and German parts (Ramsar status and UNESCO World Heritage Site). Benthic macrofauna are central

  2. Managing innovation in regional supply networks : a Dutch case of "knowledge industry clustering"

    NARCIS (Netherlands)

    Rutten, R.P.J.H.; Batenburg, R.S.

    2003-01-01

    This paper presents a case study on the collaboration between Océ, a large Dutch manufacturer of copiers and printers, and several of its lead suppliers within the so-called "knowledge industry clustering" (KIC) project. In 1993 Océ initiated this project to enable innovation by creating

  3. The whereabouts of Migrants : A comparison of Dutch migrant registration systems

    NARCIS (Netherlands)

    van Faassen, M.

    2017-01-01

    The whereabouts of migrants: a comparison of Dutch migrant registration systems. Today, one way of visualising the current refugee and migrant crisis on the outer borders of Europe is by showing a bottleneck in the processing of migrant flows: large groups of people waiting endlessly for their

  4. Observations of a narrow zone of high suspended particulate matter (SPM) concentrations along the Dutch coast

    NARCIS (Netherlands)

    van der Hout, C.M.; Gerkema, T.; Nauw, J.J.; Ridderinkhof, H.

    2015-01-01

    The objective of the study described in this paper is to localize the transport path of suspended particulate matter (SPM) in the Dutch coastal zone in the southern North Sea. It is known that a large mass of SPM is transported northward from the Strait of Dover, which is however mostly hidden from

  5. Parent-reported feeding and feeding problems in a sample of Dutch toddlers

    NARCIS (Netherlands)

    Moor, J.M.H. de; Didden, H.C.M.; Korzilius, H.P.L.M.

    2007-01-01

    Little is known about the feeding behaviors and problems with feeding in toddlers. In the present questionnaire study, data were collected on the feeding behaviors and feeding problems in a relatively large (n = 422) sample of Dutch healthy toddlers (i.e. 18-36 months old) who lived at home with

  6. Modelling climate change effects on a dutch coastal groundwater system using airborne electromagnetic measurements

    NARCIS (Netherlands)

    Faneca S̀anchez, M.; Gunnink, J.L.; Baaren, E.S. van; Oude Essink, G.H.P.; Siemon, B.; Auken, E.; Elderhorst, W.; Louw, P.G.B. de

    2012-01-01

    The forecast of climate change effects on the groundwater system in coastal areas is of key importance for policy makers. The Dutch water system has been deeply studied because of its complex system of low-lying areas, dunes, land won to the sea and dikes, but nowadays large efforts are still being

  7. Associations between dietary factors and markers of NAFLD in a general Dutch adult population

    NARCIS (Netherlands)

    Rietman, A.; Sluik, D.; Feskens, E.J.M.; Kok, F.J.; Mensink, M.R.

    2018-01-01

    Background/Objectives: The objective of this sudy was to assess the relationship between dietary intake and fatty liver as scored by the validated Fatty Liver Index (FLI) in a large cross-sectional study among a general Dutch adult population. Diet is known to affect liver fat accumulation in

  8. Dutch social housing sector reforms : Exploring the effects on low income households

    NARCIS (Netherlands)

    De Groen, A.; Pruyt, E.; Boumeester, H.J.F.M.

    2012-01-01

    Social rental housing ought to function as safety net for the lower income groups in the housing system. However, the Dutch housing system has a relatively large social housing stock in relation to other housing systems in Europe – larger than would be required for a safety net for lower income

  9. How certain are Dutch households about future income? An emprical analysis

    NARCIS (Netherlands)

    Das, J.W.M.; Donkers, A.C.D.

    1996-01-01

    The precautionary saving literature shows that income uncertainty increases savings and wealth. To estimate the magnitude of this effect, we need a measure of income uncertainty. This paper empirically analyzes subjective income uncertainty in the Netherlands. Data come from a large Dutch household

  10. A retrospective analysis of noise-induced hearing loss in the Dutch construction industry

    NARCIS (Netherlands)

    Leensen, M. C. J.; Van Duivenbooden, J. C.; Dreschler, W. A.

    2011-01-01

    Purpose Noise exposure is an important and highly prevalent occupational hazard in the construction industry. This study examines hearing threshold levels of a large population of Dutch construction workers and compares their hearing thresholds to those predicted by ISO-1999. Methods In this

  11. Putting advertising and marketing communications strategy into practice: Case of dutch companies

    NARCIS (Netherlands)

    A. Mosmans (Andy); W.J.M.I. Verbeke (Willem)

    1992-01-01

    textabstractWillem Verbeke and Andy Mosmans have undertaken a large survey of Dutch companies focusing on how effectively advertising and marketing communications compaigns are implemented. Their interesting results show, among other things, the extensive involvement of top management in advertising

  12. Educational Differences in Adolescents' Sexual Health : A Pervasive Phenomenon in a National Dutch Sample

    NARCIS (Netherlands)

    De Graaf, Hanneke; Vanwesenbeeck, Wilhelmina; Meijer, Suzanne

    2015-01-01

    Educational level is strongly associated with age of first intercourse and risk of unintended pregnancies. This study examined these associations in a large representative sample of Dutch adolescents and also included associations of educational level with other sexual health aspects. Adolescents

  13. The effects of recent changes in breeding preferences on maintaining traditional Dutch chicken genomic diversity

    NARCIS (Netherlands)

    Bortoluzzi, Chiara; Crooijmans, Richard P.M.A.; Bosse, Mirte; Hiemstra, Sipke Joost; Groenen, Martien A.M.; Megens, Hendrik Jan

    2018-01-01

    Traditional Dutch chicken breeds are marginalised breeds of ornamental and cultural-historical importance. In the last decades, miniaturising of existing breeds (so called neo-bantam) has become popular and resulted in alternatives to original large breeds. However, while backcrossing is increasing

  14. 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

  15. 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.)

  16. 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

  17. 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

  18. 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

  19. 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

  20. 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.

  1. Dutch Minister of Science Visits ESO Facilities in Chile

    Science.gov (United States)

    2005-05-01

    Mrs. Maria van der Hoeven, the Dutch Minister of Education, Culture and Science, who travelled to the Republic of Chile, arrived at the ESO Paranal Observatory on Friday afternoon, May 13, 2005. The Minister was accompanied, among others, by the Dutch Ambassador to Chile, Mr. Hinkinus Nijenhuis, and Mr. Cornelis van Bochove, the Dutch Director of Science. The distinguished visitors were able to acquaint themselves with one of the foremost European research facilities, the ESO Very Large Telescope (VLT), during an overnight stay at this remote site, and later, with the next major world facility in sub-millimetre and millimetre astronomy, the Atacama Large Millimeter Array (ALMA). At Paranal, the guests were welcomed by the ESO Director General, Dr. Catherine Cesarsky; the ESO Council President, Prof. Piet van der Kruit; the ESO Representative in Chile, Prof. Felix Mirabel; the Director of the La Silla Paranal Observatory, Dr. Jason Spyromilio; by one of the Dutch members of the ESO Council, Prof. Tim de Zeeuw; by the renowned astrophysicist from Leiden, Prof. Ewine van Dishoek, as well as by ESO staff members. The visitors were shown the various high-tech installations at the observatory, including many of the large, front-line VLT astronomical instruments that have been built in collaboration between ESO and European research institutes. Explanations were given by ESO astronomers and engineers and the Minister gained a good impression of the wide range of exciting research programmes that are carried out with the VLT. Having enjoyed the spectacular sunset over the Pacific Ocean from the Paranal deck, the Minister visited the VLT Control Room from where the four 8.2-m Unit Telescopes and the VLT Interferometer (VLTI) are operated. Here, the Minister was invited to follow an observing sequence at the console of the Kueyen (UT2) and Melipal (UT3) telescopes. "I was very impressed, not just by the technology and the science, but most of all by all the people involved

  2. 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.

  3. 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.

  4. Assessing the impact of cancer among Dutch non-Hodgkin lymphoma survivors compared with their American counterparts: a cross-national study.

    Science.gov (United States)

    Oerlemans, Simone; Smith, Sophia K; Crespi, Catherine M; Zimmerman, Sheryl; van de Poll-Franse, Lonneke V; Ganz, Patricia A

    2013-06-01

    To understand cultural differences in the impact of cancer (IOC) by (i) performing an independent psychometric evaluation of the Dutch version of the Impact of Cancer Scale version 2 (IOCv2) in a non-Hodgkin lymphoma (NHL) sample and (ii) examining differences between Dutch and American NHL survivors in perceived IOC and identifying associations with socio-demographic and clinical characteristics. Data collected from 491 Dutch and 738 American NHL survivors were used in this study. IOCv2 responses were obtained from all survivors; the Dutch survivors also completed the European Organization for Research and Treatment of Cancer Quality of Life Core questionnaire, which measures quality of life. Exploratory factor analysis of the Dutch version yielded a factor solution similar to the American structure but with some subscales merging into single factors. Internal consistency was good; Cronbach's alpha was 0.88 for the Positive and 0.94 for the Negative summary scales. Large differences were observed between survivors, whereby Dutch survivors reported fewer Positive (Δ -0.4, p Dutch sample, providing evidence that IOCv2 scales measure common and important survivor concerns across two different Western nations. Higher positive impacts for US survivors might be explained by more personal control and availability of supportive services. Future research should focus on determinants of the IOC in both Dutch and American survivors to gain better understanding of the factors that might improve it and suggest how health care may be modified toward that end. Copyright © 2012 John Wiley & Sons, Ltd.

  5. 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

  6. 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

  7. 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.

  8. 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

  9. 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.

  10. 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

  11. 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

  12. 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

  13. 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

  14. 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

  15. 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

  16. 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

  17. 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

  18. 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

  19. 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).

  20. 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

  1. 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

  2. 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

  3. 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

  4. 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.

  5. 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

  6. 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

  7. 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

  8. 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

  9. 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

  10. 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

  11. 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

  12. 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

  13. 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

  14. 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:

  15. 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

  16. 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

  17. 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

  18. 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

  19. 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

  20. 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

  1. 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

  2. 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

  3. 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…

  4. 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...

  5. 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

  6. 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

  7. 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.

  8. 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

  9. 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

  10. 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:

  11. 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

  12. 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

  13. 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

  14. 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

  15. 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

  16. 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

  17. 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

  18. 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

  19. 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

  20. 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

  1. 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

  2. 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

  3. 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.

  4. 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...

  5. 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

  6. 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

  7. Why are Dutch rheumatologists reluctant to use the COBRA treatment strategy in early rheumatoid arthritis?

    Science.gov (United States)

    van Tuyl, Lilian H D; Plass, Anne Marie C; Lems, Willem F; Voskuyl, Alexandre E; Dijkmans, Ben A C; Boers, Maarten

    2007-01-01

    Background The Combinatietherapie Bij Reumatoide Artritis (COBRA) trial has proved that combination therapy with prednisolone, methotrexate and sulphasalazine is superior to sulphasalazine monotherapy in suppressing disease activity and radiological progression of early rheumatoid arthritis (RA). In addition, 5 years of follow‐up proved that COBRA therapy results in sustained reduction of the rate of radiological progression. Despite this evidence, Dutch rheumatologists seem reluctant to prescribe COBRA therapy. Objective To explore the reasons for the reluctance in Dutch rheumatologists to prescribe COBRA therapy. Methods A short structured questionnaire based on social–psychological theories of behaviour was sent to all Dutch rheumatologists (n = 230). Results The response rate was 50%. COBRA therapy was perceived as both effective and safe, but complex to administer. Furthermore, rheumatologists expressed their concern about the large number of pills that had to be taken, the side effects of high‐dose prednisolone and the low dose of methotrexate. Although the average attitude towards the COBRA therapy was slightly positive (above the neutral point), the majority of responding rheumatologists had a negative intention (below the neutral point) to prescribe COBRA therapy in the near future. Conclusion The reluctance of Dutch rheumatologists to prescribe effective COBRA therapy may be due to perceptions of complexity of the treatment schedule and negative patient‐related consequences of the therapy. PMID:17392349

  8. 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 ...

  9. 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.

  10. 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

  11. 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)

  12. 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...

  13. 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...

  14. 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

  15. The Dutch-Flemish PROMIS Physical Function item bank exhibited strong psychometric properties in patients with chronic pain.

    Science.gov (United States)

    Crins, Martine H P; Terwee, Caroline B; Klausch, Thomas; Smits, Niels; de Vet, Henrica C W; Westhovens, Rene; Cella, David; Cook, Karon F; Revicki, Dennis A; van Leeuwen, Jaap; Boers, Maarten; Dekker, Joost; Roorda, Leo D

    2017-07-01

    The objective of this study was to assess the psychometric properties of the Dutch-Flemish Patient-Reported Outcomes Measurement Information System (PROMIS) Physical Function item bank in Dutch patients with chronic pain. A bank of 121 items was administered to 1,247 Dutch patients with chronic pain. Unidimensionality was assessed by fitting a one-factor confirmatory factor analysis and evaluating resulting fit statistics. Items were calibrated with the graded response model and its fit was evaluated. Cross-cultural validity was assessed by testing items for differential item functioning (DIF) based on language (Dutch vs. English). Construct validity was evaluated by calculation correlations between scores on the Dutch-Flemish PROMIS Physical Function measure and scores on generic and disease-specific measures. Results supported the Dutch-Flemish PROMIS Physical Function item bank's unidimensionality (Comparative Fit Index = 0.976, Tucker Lewis Index = 0.976) and model fit. Item thresholds targeted a wide range of physical function construct (threshold-parameters range: -4.2 to 5.6). Cross-cultural validity was good as four items only showed DIF for language and their impact on item scores was minimal. Physical Function scores were strongly associated with scores on all other measures (all correlations ≤ -0.60 as expected). The Dutch-Flemish PROMIS Physical Function item bank exhibited good psychometric properties. Development of a computer adaptive test based on the large bank is warranted. Copyright © 2017 Elsevier Inc. All rights reserved.

  16. PBDE and HBCDD levels in blood from Dutch mothers and infants. Analysis of a Dutch Groningen infant cohort

    Energy Technology Data Exchange (ETDEWEB)

    Weiss, J.; Linderholm, L.; Athanassiadis, I.; Bergman, Aa. [Dept. of Environmental Chemistry, Stockholm Univ. (Sweden); Meijer, L.; Sauer, P. [Dept. of Paediatrics, State Univ. Groningen (Netherlands)

    2004-09-15

    Brominated flame retardants (BFRs) are chemicals with a large industrial production and ubiquitous global use. The three major classes of BFRs produced worldwide are; tetrabromobisphenol A (TBBPA), three commercial mixtures of polybrominated diphenyl ethers (PBDEs), Deca-, Octa- and PentaBDE and hexabromocyclododecane (HBCDD). Several BFRs are, due to their lipophilicity and bioaccumulation properties, both expected and in reality found in wildlife and humans. The aim of this study was to establish a clean up method for HBCDD analysis in human serum, as well as to investigate levels of PBDEs and HBCDD in serum from mothers and infants from a Dutch cohort. A total of 90 human serum samples were analyzed. To the best of our knowledge, HBCDD has not been reported previously in humans, and PBDEs have not been reported in human serum from The Netherlands.

  17. Dutch elm disease: an overview of the biology and management regimens

    Science.gov (United States)

    R. Jay. Stipes

    2017-01-01

    Much of the information on the Dutch elm disease (DED) topic was generated by a large group of dedicated scientists, in several different agencies, primarily in the United States and Europe, over the last century. My work on the fungicidal management is but a modest contribution to the whole. It goes without saying that much more work needs to be done to open up new...

  18. The Dutch Social Interaction Anxiety Scale and the Social Phobia Scale: Reliability, Validity, and Clinical Utility

    OpenAIRE

    de Beurs, Edwin; Tielen, Deirdre; Wollmann, Lisa

    2014-01-01

    The social interaction anxiety scale (SIAS) and the social phobia scale (SPS) assess anxiety in social interactions and fear of scrutiny by others. This study examines the psychometric properties of the Dutch versions of the SIAS and SPS using data from a large group of patients with social phobia and a community-based sample. Confirmatory factor analysis revealed that the SIAS is unidimensional, whereas the SPS is comprised of three subscales. The internal consistency of the scales and subsc...

  19. Sustainability and the cultural factor. Results from the Dutch GOES Mass Public Module

    Energy Technology Data Exchange (ETDEWEB)

    Ester, P.; Vinken, H. [Institute for Globalization and Sustainable Development GLOBUS, Tilburg University, Tilburg (Netherlands)

    2000-07-01

    GOES stands for the GlObal Environmental Survey. The Dutch government declared sustainable development as the cornerstone of its environmental policy. This strategic policy shift presupposes a concerned citizenry willing to accept the way this might affect private lifestyles. The Dutch GOES MASS PUBLIC MODULE - part of a large-scale international study on citizens' environmental perceptions, values, and behaviors - shows the Dutch are concerned about environmental problems. Though they are most worried about classic environmental problems (e.g. air pollution), they realize that more long-term issues (e.g. global warming) are pressing too. They define themselves as being well-informed, feel the individual can make a difference, display a high self-efficacy, and prioritize ecological concerns over economic gains. They, however, do not accept policies that limit personal choice. The Dutch favor soft policies, voluntary lifestyle change, and softer ways of taking action (e.g. checkbook participation). Water and energy conservation no longer seem on the household agenda. Explaining policy preferences, protective action, and individual environmentally-friendly behavior shows environmental attitudes hardly make a difference. Basic values (e.g. post materialism), supporting the environment over economic growth, and a higher education do have an impact as regards protective action. This is less so for policy preferences or environmentally-friendly behavior. The Dutch GOES MASS PUBLIC MODULE points at the imperative necessity to bridge the grand environmental narratives of policy makers, particularly when they frame ecological problems as global, abstract, and long-term issues, and the here-and-now narratives of the well-informed and concerned citizens in their role of responsible consumers.

  20. Can Dutch co-generation survive threats of the liberalisation of the energy markets

    International Nuclear Information System (INIS)

    Battjes, J.J.; Rijkers, F.A.M.

    2000-07-01

    The paper presents an analysis of the effects of liberalisation of the Dutch energy markets on the future development of combined heat and power generation (co-generation) in the Netherlands. First, it reviews the historical growth in co-generation in the Netherlands and the supportive policy measures that have contributed to this growth. Second, the liberalisation process of the Dutch electricity market and the Dutch gas market is described. Subsequently, we discuss the impacts of these new market structures on co-generation by using two scenarios for the Dutch energy markets. Our assessment of the impacts is mainly focused on the cost-effectiveness of co-generation projects. We determine the key aspects that influence the cost-effectiveness of a co-generation project and analyse some of the calculations for different small-scale and large-scale co-generation projects. Based on the results, we conclude that investments in new co-generation plants are unlikely in the short term and the existing plants can barely produce with a positive cash flow. As many parties have an interest in reducing the negative effects of a liberalised energy market on co-generation, approaches are sought to improve the cost-effectiveness of co-generation in the Netherlands. We describe several optional supportive measures for co-generation mainly resulting from the determination of the barriers for co-generation. Moreover, Dutch authorities have already responded to these barriers by preparing policy measures such as investment subsidies and exemption from the energy tax. 2 refs

  1. 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

  2. Identification document for large by-catch species in the Mauritanian Exclusive Economic Zone

    NARCIS (Netherlands)

    Hofstede, ter R.

    2003-01-01

    The coastal waters of Mauritania (Northwest Africa) contain large numbers of pelagic fish such as sardinella, sardine, mackerel and horse mackerel. Freezer-trawlers from the European Union (EU), mainly of Dutch origin, have exploited these resources since 1996. In 1998 the Dutch ship owners

  3. Enacting Fit in Work Organization and Occupational Structure Design : The Case of Intermediary Occupations in a Dutch Hospital

    NARCIS (Netherlands)

    van Offenbeek, M.A.G.; Sorge, A.; Knip, Marrig

    2009-01-01

    We report and interpret findings from an investigation of work redesign and the introduction of a new occupational position, nurse practitioner (NP), in four different subunits in a large Dutch university hospital. Full development of the new position and its contribution to performance crucially

  4. Nutritional Supplement Use by Dutch Elite and Sub-Elite Athletes: Does Receiving Dietary Counselling Make a Difference?

    NARCIS (Netherlands)

    Wardenaar, F.C.; Ceelen, I.J.M.; Dijk, van J.W.; Hangelbroek, R.W.J.; Roy, van L.; Pouw, van der B.; Vries, de J.H.M.; Mensink, M.R.; Witkamp, R.F.

    2017-01-01

    The use of nutritional supplements is highly prevalent among athletes. In this cross-sectional study we assessed the prevalence of nutritional supplement use by a large group of Dutch competitive athletes in relation to dietary counselling. A total of 778 athletes (407 males and 371 females)

  5. Making Materiality Matter : A sociological analysis of price formation on the Dutch fiction book market, 1980-2009

    NARCIS (Netherlands)

    Franssen, T.; Velthuis, O.

    2016-01-01

    This article analyzes determinants of prices in the Dutch fiction book market between 1980 and 2009. It does so on the basis of interviews with editors of large publishing houses and regression analysis of a dataset that contains prices and their determinants of over 80 000 fiction books. We show

  6. Major adverse cardiac and cerebrovascular events after the ross procedure: A report from the german-dutch ross registry

    NARCIS (Netherlands)

    H. Sievers (Hans Hinrich); U. Stierle (Ulrich); E.I. Charitos (Efstratios); T. Hanke; M. Misfeld (Martin); J.F.M. Bechtel (Matthias); A. Gorski (Armin); U.F. Franke (Ulrich); B. Graf (Bernhard); D.R. Robinson (Derek); A.J.J.C. Bogers (Ad); A. Dodge-Khatami (Ali); J.O. Boehm (Juergen); J.G. Rein (Joachim); C.A. Botha (Cornelius); R. Lange (Rüdiger); J. Hoerer (Juergen); A. Moritz (Anton); T. Wahlers (Thorsten); M. Breuer (Martin); K. Ferrari-Kuehne (Katharina); R. Hetzer (Roland); M. Huebler (Michael); G. Ziemer (Gerhard); J.J.M. Takkenberg (Hanneke); W. Hemmer

    2010-01-01

    textabstractBackground-: The purpose of the study is to report major cardiac and cerebrovascular events after the Ross procedure in the large adult and pediatric population of the German-Dutch Ross registry. These data could provide an additional basis for discussions among physicians and a source

  7. Animal-Assisted Interventions in Dutch Nursing Homes: A Survey.

    Science.gov (United States)

    Schuurmans, Lonneke; Enders-Slegers, Marie-Jose; Verheggen, Theo; Schols, Jos

    2016-07-01

    sophisticated robot seal Paro was used in 7 nursing homes. A large percentage (80%) of nursing homes that worked with animals did not have AAI-specific health protocols or animal welfare and safety protocols underlying the animal activities or specific selection criteria for the selection of suitable animals. Most of the participating Dutch nursing homes offer AAI in recreational programs (animal-assisted activities) for psychogeriatric clients (using visiting animals, especially dogs). Most nursing homes do not have specific AAI protocols for animal welfare, hygiene, and safety during animal activities, nor do they employ specific selection criteria for participating animals and their handlers. Copyright © 2016 AMDA – The Society for Post-Acute and Long-Term Care Medicine. Published by Elsevier Inc. All rights reserved.

  8. A Swiss Village in the Dutch Tropics: The Limitations of Empire-Centred Approaches to the Early Modern Atlantic World

    Directory of Open Access Journals (Sweden)

    Karwan Fatah-Black

    2013-03-01

    Full Text Available This article considers what the migration circuits to and from Suriname can tell us about Dutch early modern colonisation in the Atlantic world. Did the Dutch have an Atlantic empire that can be studied by treating it as an integrated space, as suggested by New Imperial Historians, or did colonisation rely on circuits outside Dutch control, stretching beyond its imperial space? An empire-centred approach has dominated the study of Suriname’s history and has largely glossed over the routes taken by European migrants to and from the colony. When the empirecentred perspective is transcended it becomes possible to see that colonists arrived in Suriname from a range of different places around the Atlantic and the European hinterland. The article takes an Atlantic or global perspective to demonstrate the choices available to colonists and the networks through which they moved.

  9. Validation of a Dutch risk score predicting poor outcome in adults with bacterial meningitis in Vietnam and Malawi.

    Directory of Open Access Journals (Sweden)

    Ewout S Schut

    Full Text Available We have previously developed and validated a prognostic model to predict the risk for unfavorable outcome in Dutch adults with bacterial meningitis. The aim of the current study was to validate this model in adults with bacterial meningitis from two developing countries, Vietnam and Malawi. Demographic and clinical characteristics of Vietnamese (n = 426, Malawian patients (n = 465 differed substantially from those of Dutch patients (n = 696. The Dutch model underestimated the risk of poor outcome in both Malawi and Vietnam. The discrimination of the original model (c-statistic [c] 0.84; 95% confidence interval 0.81 to 0.86 fell considerably when re-estimated in the Vietnam cohort (c = 0.70 or in the Malawian cohort (c = 0.68. Our validation study shows that new prognostic models have to be developed for these countries in a sufficiently large series of unselected patients.

  10. Large impact of small particulates; Grote invloed van klein fijnstof

    Energy Technology Data Exchange (ETDEWEB)

    Hiemstra-Holtkamp, I.V.L. [Astma Fonds, Leusden (Netherlands)

    2006-06-15

    PM2.5 particulates are responsible for a large part of thousands of people that die of air pollution. The Asthma Fund in the Netherlands wants the Dutch government to take specific action and measures for those small particulates. [Dutch] PM2.5 is verantwoordelijk voor een groot deel van de 1000-en mensen die elk jaar sterven door luchtverontreiniging. Het Astma Fonds wil dat de overheid aparte maatregelen neemt voor PM2.5.

  11. Assessing peak aerobic capacity in Dutch law enforcement officers

    Directory of Open Access Journals (Sweden)

    Harriet Wittink

    2015-06-01

    Full Text Available Objectives: To cross-validate the existing peak rate of oxygen consumption (VO2peak prediction equations in Dutch law enforcement officers and to determine whether these prediction equations can be used to predict VO2peak for groups and in a single individual. A further objective was to report normative absolute and relative VO2peak values of a sample of law enforcement officers in the Netherlands. Material and Methods: The peak rate of oxygen consumption (ml×kg–1×min–1 was measured using a maximal incremental bicycle test in 1530 subjects, including 1068 male and 461 female police officers. Validity of the prediction equations for groups was assessed by comparing predicted VO2peak with measured VO2peak using paired t-tests. For individual differences limits of agreement (LoA were calculated. Equations were considered valid for individuals when the difference between measured and predicted VO2peak did not exceed ±1 metabolic equivalent (MET in 95% of individuals. Results: None of the equations met the validity criterion of 95% of individuals having ±1 MET difference or less than the measured value. Limits of agreement (LoAs were large in all predictions. At the individual level, none of the equations were valid predictors of VO2peak (ml×kg–1×min–1. Normative values for Dutch law enforcement officers were presented. Conclusions: Substantial differences between measured and predicted VO2peak (ml×kg–1×min–1 were found. Most tested equations were invalid predictors of VO2peak at group level and all were invalid at individual levels.

  12. 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…

  13. 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.

  14. 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

  15. 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

  16. Actual heating energy savings in thermally renovated Dutch dwellings

    International Nuclear Information System (INIS)

    Majcen, Daša; Itard, Laure; Visscher, Henk

    2016-01-01

    The register of the Dutch social housing stock was analysed, containing 300.000 dwellings, renovated between 2010 and 2013. The main objective was twofold: to evaluate the performance gap in these dwellings before and after the renovation and to establish what renovation measures achieve the highest reduction of consumption, particularly in practice (actual savings). The results showed large performance gaps in dwellings with low R and high U values, local heating systems, changes from a non-condensing into a condensing boiler and upgrades to a natural ventilation system. Regarding the actual effectiveness of renovation measures, replacement of old gas boilers with more efficient ones yields the highest energy reduction, followed by deep improvements of windows. Installing mechanical ventilation yields a small reduction compared to other measures, but still much larger than theoretically expected. The paper shows once more that the calculation method currently in use cannot be considered accurate if compared to actual consumption. The study demonstrated that unrealistic theoretical efficiencies of heating systems and insulation values are causing a part of the performance gap. Nowadays, large datasets of buildings thermal performance and actual consumption offer an opportunity to improve these misconceptions. - Highlights: • Performance gap is lower in more efficient buildings. • Replacements of gas boilers – the most energy reduction among renovation measures. • Replacing the ventilation system yields a much larger reduction than expected. • How well are the standard values of the calculation methods defined? • Provide large public building performance databases including actual use data.

  17. 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

  18. 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

  19. 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

  20. 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 ...

  1. 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

  2. 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

  3. 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

  4. 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.

  5. 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

  6. 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

  7. 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

  8. 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

  9. 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

  10. 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

  11. 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

  12. 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

  13. 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

  14. 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.

  15. 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

  16. 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

  17. 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

  18. 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

  19. 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

  20. 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

  1. 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

  2. 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

  3. 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

  4. 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

  5. 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…

  6. 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"…

  7. 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)

  8. 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

  9. 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

  10. 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

  11. 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

  12. 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.

  13. 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,

  14. Labour Market Segmentation: An Investigation into the Dutch Hospitality Industry

    NARCIS (Netherlands)

    A. Bispo

    2007-01-01

    textabstractAlmost 42,000 establishments, over 310,000 employees and approximately 13 billion Euro annual turnover (including VAT). This, in a nutshell, captures the Dutch hospitality industry in 2005. The aim of this study is to examine the functioning of the labour market in the Dutch hospitality

  15. Evaluation of the Dutch subsurface geoportal: What lies beneath?

    NARCIS (Netherlands)

    Lance, K.T.; Georgiadou, Y.; Bregt, A.K.

    2011-01-01

    This paper focuses on a geoportal from a “what lies beneath” perspective. It analyses processes of budgeting, planning, monitoring, performance measurement, and reporting of the national initiative titled Digital Information of the Dutch Subsurface (known by its Dutch acronym, DINO). The study is

  16. A sustainable marketing strategy for Dutch tourists to South Africa ...

    African Journals Online (AJOL)

    A sustainable marketing strategy for Dutch tourists to South Africa. ... The Dutch market is regarded as a priority market because of cultural and historical links, but it is also a market that has not been ... EMAIL FULL TEXT EMAIL FULL TEXT

  17. Physical activity among Dutch workers-differences between occupations

    NARCIS (Netherlands)

    Proper, K.I.; Hildebrandt, V.H.

    2006-01-01

    Objective.: This study describes the total physical activity and the contribution of work to total physical activity among Dutch workers. The study was conducted in the Netherlands in the years 2000-2002. Methods.: 2,417 respondents of a cross-sectional survey representing the Dutch adult working

  18. Knowledge Externalities, Agglomeration Economies, and Employment Growth in Dutch Cities

    NARCIS (Netherlands)

    van Soest, D.P.; Gerking, S.D.; van Oort, F.G.

    2002-01-01

    This paper extends the work of Glaeser et al.(1992) by looking at effects of agglomeration economies on employment growth in Dutch city-industries and in very small (postal) zip code-industries in the Dutch province of South-Holland. At both levels of geographic detail, findings are broadly

  19. Acquisition of Morpho-Phonology: the Dutch voicing alternation

    NARCIS (Netherlands)

    Kerkhoff, A.O.

    2007-01-01

    This dissertation investigates how and when the Dutch voicing alternation is acquired. In Dutch, final neutralisation of the voice contrast (or ‘final devoicing’) leads to alternations in singular - plural pairs such as bed ~ bedden ‘bed(s)’. In such pairs, the singular always ends in a voiceless

  20. Pupping habitat ofd grey seals in the Dutch Wadden Sea

    NARCIS (Netherlands)

    Brasseur, S.M.J.M.; Groot, de A.V.; Aarts, G.M.; Dijkman, E.M.; Kirkwood, R.J.

    2015-01-01

    Atlantic grey seals (Halichoerus grypus grypus) started recolonising Dutch coastal haul-outs in the 1950s, after practically 500 years of rarity in the Dutch coastal zone which was caused mainly by human hunting. The first pup-birth was recorded in 1985 at the Wadden Sea sandbank of Engelschhoek.