157 research outputs found
Renal insufficiency, a frequent complication with age in oral-facial-digital syndrome type I.
No full textpeer reviewedSaal S, Faivre L, Aral B, Gigot N, Toutain A, Van Maldergem L, Destree A, Maystadt I, Cosyns J-P, Jouk P-S, Loeys B, Chauveau D, Bieth E, Layet V, Mathieu M, Lespinasse J, Teebi A, Franco B, Gautier E, Binquet C, Masurel-Paulet A, Mousson C, Gouyon J-B, Huet F, Thauvin-Robinet C. Renal insufficiency, a frequent complication with age in oral-facial-digital syndrome type I. The oral-facial-digital syndrome type I (OFD I) is characterized by multiple congenital malformations of the face, oral cavity and digits. A polycystic kidney disease (PKD) is found in about one-third of patients but long-term outcome and complications are not well described in the international literature. Renal findings have been retrospectively collected in a cohort of 34 females all carrying a pathogenic mutation in the OFD1 gene with ages ranging from 1 to 65 years. Twelve patients presented with PKD - 11/16 (69%) if only adults were considered -with a median age at diagnosis of 29 years [IQR (interquartile range) = (23.5-38)]. Among them, 10 also presented with renal impairment and 6 were grafted (median age = 38 years [IQR = (25-48)]. One grafted patient under immunosuppressive treatment died from a tumor originated from a native kidney. The probability to develop renal failure was estimated to be more than 50% after the age of 36 years. Besides, neither genotype-phenotype correlation nor clinical predictive association with renal failure could be evidenced. These data reveal an unsuspected high incidence rate of the renal impairment outcome in OFD I syndrome. A systematic ultrasound (US) and renal function follow-up is therefore highly recommended for all OFD I patients
Some modern Belgian writers. A critical study.
No full textPrefatory note, by Edmund Gosse.--The renascence of Belgian letters.--Maurice Maeterlinck.--Verhaeren--Rodenbach.--Camille Lemonnier.--Georges Eckhoud.--Max Eiskamp and Charles van Lerberghe.--The Destree brothers.--Courouble.Mode of access: Internet
Parution : P. Destrée & P. Murray (éds.) A Companion to Ancient Aesthetics
No full textVient de paraître : A Companion to Ancient Aesthetics Pierre Destrée, Penelope Murray ISBN: 978-1-4443-3764-8 552 pages July 2015, Wiley-Blackwell Source : Wiley: A Companion to Ancient Aesthetics - Pierre Destree, Penelope Murra
Adult phenotype of KCNQ2 encephalopathy
No full textBackground: Pathogenic KCNQ2 variants are a frequent cause of developmental and epileptic encephalopathy. Methods: We recruited 13 adults (between 18 years and 45 years of age) with KCNQ2 encephalopathy and reviewed their clinical, EEG, neuroimaging and treatment history. Results: While most patients had daily seizures at seizure onset, seizure frequency declined or remitted during childhood and adulthood. The most common seizure type was tonic seizures (early) infancy, and tonic-clonic and focal impaired awareness seizures later in life. Ten individuals (77%) were seizure-free at last follow-up. In 38% of the individuals, earlier periods of seizure freedom lasting a minimum of 2 years followed by seizure recurrence had occurred. Of the 10 seizure-free patients, 4 were receiving a single antiseizure medication (ASM, carbamazepine, lamotrigine or levetiracetam), and 2 had stopped taking ASM. Intellectual disability (ID) ranged from mild to profound, with the majority (54%) of individuals in the severe category. At last contact, six individuals (46%) remained unable to walk independently, six (46%) had limb spasticity and four (31%) tetraparesis/tetraplegia. Six (46%) remained non-verbal, 10 (77%) had autistic features/autism, 4 (31%) exhibited aggressive behaviour and 4 (31%) destructive behaviour with self-injury. Four patients had visual problems, thought to be related to prematurity in one. Sleep problems were seen in six (46%) individuals. Conclusion: Seizure frequency declines over the years and most patients are seizure-free in adulthood. Longer seizure-free periods followed by seizure recurrence are common during childhood and adolescence. Most adult patients have severe ID. Motor, language and behavioural problems are an issue of continuous concern
Een cleen tractaetkin van de waerde des helich sacrame[n]ts, en[de] preparatie oft beredinghe tot die[n], om dat waerdeghelick te ontfanghene /
No full textBib. Belgica (1964-1970 éd.) ; A 15Cockx-Indestege, E. Belgica typographica ; 7770Machiels, J. Catalogus van de boeken gedrukt vóór 1600 ; A 311Europeana-GoogleBooksVander Haeghen, Ferdinand François Ernes
“Letter to the King” By Jules Destry: from Separatism to Unitarism in Belgium
No full textContradictions between the regions of Belgium - Wallonia and Flanders - have a long history associated with uneven political, economic and cultural development of the territories, which in 1830 became parts of an independent state, the Kingdom of Belgium. The "Letter to the King", written by eminent Belgian politician and writer, socialist Jules Destree (1863 - 1936 gg.), is one of the most interesting sources on the history of Belgium of this period, It became a kind of manifesto of balance between the ideas of separatism and unitarianism. This article includes excerpts from the "Letter to the King", which were given for the first time in the author's Russian translation and conducted historical criticism of the source. The study is based on a set of scientific methods and approaches, including the principle of scientific objectivity and systematic approach, used in historical research. The main methods are problematic and historical-comparative analysis, classification and comparison of political and historical concepts. An external source of criticism included information about the place and the time of the creation of document, the biography of the author studies; internal critic source recreates the idea of the letter and the background of historical events. Prominent Belgian politician and writer, socialist Jules Destree (1863 - 1936), highly appreciated the personal qualities of King Albert I, appealed to his experience and political vision, describing all the problems Walloons faced in their opposition to the Flemish. Destree in his letter opposed the unequal development of the regions of Belgium and the protectionist policies of the central government in respect to the detriment of Flanders Wallonia. He summarized and formulated the factors that, in his opinion, interfere with the full development of his country. Destree pays great attention to linguistic differences, which were at the heart of conflicts between Walloons and Flemings, and are still shaken by Belgium. He reflects on the fact that the central government should be doing to reduce the national tensions that unites two people, and that the shares, which should be a system of education that future generations were able to overcome the historical alienation of such geographically close and ideologically distant peoples. Although Jules Destree firmly defends separatism idea for Wallonia as the only solution to the problem, however, by the end of his letter, reasoning it takes a more balanced position. The idea of a unitary state was not denied completely, but Destree emphasizes the importance of a balanced central government policy in relation to the regions in order to reduce internal tensions between the two nations. The ideas embodied in his "Letter to the King", formed the basis of the principles of peaceful co-existence of further autonomous regions as part of the union, which was manifested in the future during the First World War, when the Walloons and Flemings alongside confronted a common enemy
‘We work for the Devil’ : oil extraction, kinship and the fantasy of time on the offshore frontier
No full textFunding: H2020 European Research Council Energy Ethics (715146).In the offshore oil industry of Takoradi, Ghana, white expatriate workers describe oil extraction as both ‘the work of the Devil’ and a ‘labour of love’. While companies strive to produce the offshore as a timeless and spaceless fantasy of ‘frictionless profit’, workers emphasize oil work as a sacrificial economy where risk, loss and distance are traded in the pursuit of an ideal of family life. In this article, I argue that the operational structures and labour regime of the offshore (characterized by a rotation pattern, continuous production, distant locations, a segregated workforce, and mobile installations) create not only a model of capital accumulation, but a mode of being and making kin. I describe oil workers’ aspirations to a ‘good family life’ and parental care, pitting time against distance, and the interpersonal ruins that remain when they fray. In probing how oil workers make petro-capitalism affectively workable, by exploring the entangled processes of extractive and reproductive labour, this article contributes to recent scholarship on the role of kinship in sustaining global capitalism.Peer reviewe
Medicinael bouc. Inwendichen en de wtwendich, voir alle crancheydt des lichaems, die een mensce ouerco[m]men moghen [...] /
No full textBewerking door Symon Andriessen van J. Schöner's Ein nützlichs Büchlein viler bewerter Artzney en L. Burres' Ein new Wund Artzney BüchleinDrukkersmerk op titelblad en achteraanHerkomst: vignet Bibliotheek SnellaertBib. Belgica (1964-1970 éd.) ; S 120Cockx-Indestege, E. Belgica typographica ; 9063Diegerick, A. Bib. yproise ; p. 27-28Machiels, J. Catalogus van de boeken gedrukt vóór 1600 ; S 201Europeana-GoogleBook
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