Ratings of age of acquisition of 299 words across 25 languages: Is there a cross-linguistic order of words?

We present a new set of subjective age-of-acquisition (AoA) ratings for 299 words (158 nouns, 141 verbs) in 25 languages from five language families (Afro-Asiatic: Semitic languages; Altaic: one Turkic language: Indo-European: Baltic, Celtic, Germanic, Hellenic, Slavic, and Romance languages; Niger-Congo: one Bantu language; Uralic: Finnic and Ugric languages). Adult native speakers reported the age at which they had learned each word. We present a comparison of the AoA ratings across all languages by contrasting them in pairs. This comparison shows a consistency in the orders of ratings across the 25 languages. The data were then analyzed (1) to ascertain how the demographic characteristics of the participants influenced AoA estimations and (2) to assess differences caused by the exact form of the target question (when did you learn vs. when do children learn this word); (3) to compare the ratings obtained in our study to those of previous studies; and (4) to assess the validity of our study by comparison with quasi-objective AoA norms derived from the MacArthur–Bates Communicative Development Inventories (MB-CDI). All 299 words were judged as being acquired early (mostly before the age of 6 years). AoA ratings were associated with the raters’ social or language status, but not with the raters’ age or education. Parents reported words as being learned earlier, and bilinguals reported learning them later. Estimations of the age at which children learn the words revealed significantly lower ratings of AoA. Finally, comparisons with previous AoA and MB-CDI norms support the validity of the present estimations. Our AoA ratings are available for research or other purposes

Seniority-based entitlements : extent, policy debates and research

Aquesta publicació s'elabora a partir de les contribucions de cadascú dels membres nacionals que integren la Network of Eufound Correspondent. Pel cas d'Espanya la contribució ha estat realitzada per l'Oscar MolinaSeniority systems - schemes that allot improving employment rights or benefits to employees as their length of employment increases - have not been widely studied. This report provides the first comprehensive study comparing the design and spread of seniority-based entitlements (SBEs) in Europe and mapping related policy debates. It is primarily based on contributions from the Network of Eurofound Correspondents, covering the 28 EU Member States and Norway, but also presents aggregate seniority-earnings curves for the EU based on data from the Structure of Earnings Survey. The aim of the report is to take stock of the currently existing different types of SBEs in the private and public sectors. It concludes that despite an obvious trend to remove them from regulations or reform them, a substantial amount of such entitlements is here to stay. Paradoxically, countries which have regulations on seniority pay in place tend to have flatter aggregate seniority-earnings curves than countries without such regulations

The European internet-based patient and research database for primary immunodeficiencies: update 2011.

In order to build a common data pool and estimate the disease burden of primary immunodeficiencies (PID) in Europe, the European Society for Immunodeficiencies (ESID) has developed an internet-based database for clinical and research data on patients with PID. This database is a platform for epidemiological analyses as well as the development of new diagnostic and therapeutic strategies and the identification of novel disease-associated genes. Since its start in 2004, 13,708 patients from 41 countries have been documented in the ESID database. Common variable immunodeficiency (CVID) represents the most common entity with 2880 patients or 21% of all entries, followed by selective immunoglobulin A (sIgA) deficiency (1424 patients, 10·4%). The total documented prevalence of PID is highest in France, with five patients per 100,000 inhabitants. The highest documented prevalence for a single disease is 1·3 per 100,000 inhabitants for sIgA deficiency in Hungary. The highest reported incidence of PID per 100,000 live births was 16·2 for the period 1999-2002 in France. The highest reported incidence rate for a single disease was 6·7 for sIgA deficiency in Spain for the period 1999-2002. The genetic cause was known in 36·2% of all registered patients. Consanguinity was reported in 8·8%, and 18·5% of patients were reported to be familial cases; 27·9% of patients were diagnosed after the age of 16. We did not observe a significant decrease in the diagnostic delay for most diseases between 1987 and 2010. The most frequently reported long-term medication is immunoglobulin replacement

Initial presenting manifestations in 16,486 patients with inborn errors of immunity include infections and noninfectious manifestations

none313siBackground: Inborn errors of immunity (IEI) are rare diseases, which makes diagnosis a challenge. A better description of the initial presenting manifestations should improve awareness and avoid diagnostic delay. Although increased infection susceptibility is a well-known initial IEI manifestation, less is known about the frequency of other presenting manifestations. Objective: We sought to analyze age-related initial presenting manifestations of IEI including different IEI disease cohorts. Methods: We analyzed data on 16,486 patients of the European Society for Immunodeficiencies Registry. Patients with autoinflammatory diseases were excluded because of the limited number registered. Results: Overall, 68% of patients initially presented with infections only, 9% with immune dysregulation only, and 9% with a combination of both. Syndromic features were the presenting feature in 12%, 4% had laboratory abnormalities only, 1.5% were diagnosed because of family history only, and 0.8% presented with malignancy. Two-third of patients with IEI presented before the age of 6 years, but a quarter of patients developed initial symptoms only as adults. Immune dysregulation was most frequently recognized as an initial IEI manifestation between age 6 and 25 years, with male predominance until age 10 years, shifting to female predominance after age 40 years. Infections were most prevalent as a first manifestation in patients presenting after age 30 years. Conclusions: An exclusive focus on infection-centered warning signs would have missed around 25% of patients with IEI who initially present with other manifestations.noneThalhammer J.; Kindle G.; Nieters A.; Rusch S.; Seppanen M.R.J.; Fischer A.; Grimbacher B.; Edgar D.; Buckland M.; Mahlaoui N.; Ehl S.; Boztug K.; Brunner J.; Demel U.F.; Forster-Waldl E.; Gasteiger L.M.; Goschl L.; Kojic M.; Schroll A.; Seidel M.G.; Wintergerst U.; Wisgrill L.; Sharapova S.O.; Goffard J.-C.; Kerre T.; Meyts I.; Roosens F.; Smet J.; Haerynck F.; Eric Z.P.; Milenova V.; Gagro A.; Richter D.; Chovancova Z.; Hlavackova E.; Litzman J.; Milota T.; Sediva A.; Elaziz D.A.; Alkady R.S.; El Sayed El Hawary R.; Eldash A.S.; Galal N.; Lotfy S.; Meshaal S.S.; Reda S.M.; Sobh A.; Elmarsafy A.; Brosselin P.; Courteille V.; De Vergnes N.; Kracker S.; Pergent M.; Randrianomenjanahary P.; Ahrenstorf G.; Albert M.H.; Ankermann T.; Atschekzei F.; Baumann U.; Becker B.C.; Behrends U.; Belohradsky B.H.; Biegner A.-K.; Binder N.; Bode S.F.N.; Boesecke C.; Boetticher B.; Borte M.; Borte S.; Classen C.F.; Dirks J.; Duckers G.; El-Helou S.; Ernst D.; Fasshauer M.; Fecker G.; Felgentreff K.; Foell D.; Ghosh S.; Girschick H.J.; Goldacker S.; Graf N.; Graf D.; Greil J.; Hanitsch L.G.; Hauck F.; Heeg M.; Heine S.I.; Henes J.C.; Hoenig M.; Holzer U.; Holzinger D.; Horneff G.; Hundsdoerfer P.; Jablonka A.; Jakoby D.; Joean O.; Kaiser-Labusch P.; Klemann C.; Kobbe R.; Korholz J.; Kramm C.M.; Kruger R.; Landwehr-Kenzel S.; Lehmberg K.; Liese J.G.; Lippert C.F.; Maccari M.E.; Masjosthusmann K.; Meinhardt A.; Metzler M.; Morbach H.; Muller I.; Naumann-Bartsch N.; Neubert J.; Niehues T.; Peter H.-H.; Rieber N.; Ritterbusch H.; Rockstroh J.K.; Roesler J.; Schauer U.; Scheible R.; Schmalzing M.; Schmidt R.E.; Schneider D.T.; Schreiber S.; Schuetz C.; Schulz A.; Schulze-Koops H.; Schulze-Sturm U.; Schuster V.; Schwaneck E.C.; Schwarz K.; Schwarze-Zander C.; Sirin M.; Skapenko A.; Sogkas G.; Sparber-Sauer M.; Speckmann C.; Steinmann S.; Stiehler S.; Tenbrock K.; von Bernuth H.; Warnatz K.; Wasmuth J.-C.; Weiss M.; Witte T.; Wittke K.; Wittkowski H.; Zeuner R.A.; Farmaki E.; Hatzistilianou M.N.; Kakkas I.; Kanariou M.G.; Kapousouzi A.; Liatsis E.; Maggina P.; Papadopoulou-Alataki E.; Raptaki M.; Speletas M.; Tantou S.; Goda V.; Krivan G.; Marodi L.; Abolhassani H.; Aghamohammadi A.; Rezaei N.; Feighery C.; Leahy T.R.; Ryan P.; Batzir N.A.; Garty B.Z.; Tamary H.; Aiuti A.; Amodio D.; Azzari C.; Barzaghi F.; Baselli L.A.; Cancrini C.; Carrabba M.; Cazzaniga M.; Cesaro S.; Chinello M.; Danieli M.G.; Dellepiane R.M.; Fabio G.; Gambineri E.; Lodi L.; Lougaris V.; Marasco C.; Martire B.; Marzollo A.; Milito C.; Moschese V.; Pignata C.; Plebani A.; Porta F.; Quinti I.; Ricci S.; Soresina A.; Tommasini A.; Vacca A.; Vanessa C.; Blaziene A.; Sitkauskiene B.; Gowin E.; Heropolitanska-Pliszka E.; Pietrucha B.; Szaflarska A.; Wiesik-Szewczyk E.; Wolska-Kusnierz B.; Esteves I.; Faria E.; Marques L.H.; Neves J.F.; Silva S.L.; Teixeira C.; Pereira da Silva S.; Capilna B.R.; Guseva M.N.; Shcherbina A.; Bobcakova A.; Ciznar P.; Gabzdilova J.; Jesenak M.; Kapustova L.; Orosova J.; Petrovicova O.; Raffac S.; Kopac P.; Allende L.M.; Antoli A.; Blanch G.R.; Carbone J.; Dieli-Crimi R.; Garcia-Prat M.; Gil-Herrera J.; Gonzalez-Granado L.I.; Agullo P.L.; Olbrich P.; Parra-Martinez A.; Paz-Artal E.; Pleguezuelo D.E.; Rodriguez N.S.; Sanchez-Ramon S.; Santos-Perez J.L.; Solanich X.; Soler-Palacin P.; Gonzalez-Amores M.; Ekwall O.; Fasth A.; Bitzenhofer-Gruber M.; Candotti F.; Dimitriou F.; Heininger U.; Holbro A.; Jandus P.; Kolios A.G.A.; Marschall K.; Schmid J.P.; Posfay-Barbe K.M.; Prader S.; Reichenbach J.; Steiner U.C.; Truck J.; Bredius R.G.; de Kruijf- Bazen S.; de Vries E.; Henriet S.S.V.; Kuijpers T.W.; Potjewijd J.; Rutgers A.; Stol K.; van Aerde K.J.; Van den Berg J.M.; van de Ven A.A.J.M.; Montfrans J.; Aydemir S.; Baris S.; Dogu F.; Ikinciogullari A.; Karakoc-Aydiner E.; Kilic S.S.; Kiykim A.; Kokcu Karadag S.I.; Kutukculer N.; Ocak S.; UNAL E.; Boyarchuk O.; Hilfanova A.; Kostyuchenko L.V.; Alachkar H.; Arkwright P.D.; Baxendale H.E.; Bernatoniene J.; Coulter T.I.; Garcez T.; Goddard S.; Gompels M.M.; Grigoriadou S.; Herriot R.; Herwadkar A.; Huissoon A.; Ibberson L.; Nademi Z.; Noorani S.; Parvin S.; Steele C.L.; Thomas M.; Waruiru C.; Yong P.F.K.; Bourne H.Thalhammer, J.; Kindle, G.; Nieters, A.; Rusch, S.; Seppanen, M. R. J.; Fischer, A.; Grimbacher, B.; Edgar, D.; Buckland, M.; Mahlaoui, N.; Ehl, S.; Boztug, K.; Brunner, J.; Demel, U. F.; Forster-Waldl, E.; Gasteiger, L. M.; Goschl, L.; Kojic, M.; Schroll, A.; Seidel, M. G.; Wintergerst, U.; Wisgrill, L.; Sharapova, S. O.; Goffard, J. -C.; Kerre, T.; Meyts, I.; Roosens, F.; Smet, J.; Haerynck, F.; Eric, Z. P.; Milenova, V.; Gagro, A.; Richter, D.; Chovancova, Z.; Hlavackova, E.; Litzman, J.; Milota, T.; Sediva, A.; Elaziz, D. A.; Alkady, R. S.; El Sayed El Hawary, R.; Eldash, A. S.; Galal, N.; Lotfy, S.; Meshaal, S. S.; Reda, S. M.; Sobh, A.; Elmarsafy, A.; Brosselin, P.; Courteille, V.; De Vergnes, N.; Kracker, S.; Pergent, M.; Randrianomenjanahary, P.; Ahrenstorf, G.; Albert, M. H.; Ankermann, T.; Atschekzei, F.; Baumann, U.; Becker, B. C.; Behrends, U.; Belohradsky, B. H.; Biegner, A. -K.; Binder, N.; Bode, S. F. N.; Boesecke, C.; Boetticher, B.; Borte, M.; Borte, S.; Classen, C. F.; Dirks, J.; Duckers, G.; El-Helou, S.; Ernst, D.; Fasshauer, M.; Fecker, G.; Felgentreff, K.; Foell, D.; Ghosh, S.; Girschick, H. J.; Goldacker, S.; Graf, N.; Graf, D.; Greil, J.; Hanitsch, L. G.; Hauck, F.; Heeg, M.; Heine, S. I.; Henes, J. C.; Hoenig, M.; Holzer, U.; Holzinger, D.; Horneff, G.; Hundsdoerfer, P.; Jablonka, A.; Jakoby, D.; Joean, O.; Kaiser-Labusch, P.; Klemann, C.; Kobbe, R.; Korholz, J.; Kramm, C. M.; Kruger, R.; Landwehr-Kenzel, S.; Lehmberg, K.; Liese, J. G.; Lippert, C. F.; Maccari, M. E.; Masjosthusmann, K.; Meinhardt, A.; Metzler, M.; Morbach, H.; Muller, I.; Naumann-Bartsch, N.; Neubert, J.; Niehues, T.; Peter, H. -H.; Rieber, N.; Ritterbusch, H.; Rockstroh, J. K.; Roesler, J.; Schauer, U.; Scheible, R.; Schmalzing, M.; Schmidt, R. E.; Schneider, D. T.; Schreiber, S.; Schuetz, C.; Schulz, A.; Schulze-Koops, H.; Schulze-Sturm, U.; Schuster, V.; Schwaneck, E. C.; Schwarz, K.; Schwarze-Zander, C.; Sirin, M.; Skapenko, A.; Sogkas, G.; Sparber-Sauer, M.; Speckmann, C.; Steinmann, S.; Stiehler, S.; Tenbrock, K.; von Bernuth, H.; Warnatz, K.; Wasmuth, J. -C.; Weiss, M.; Witte, T.; Wittke, K.; Wittkowski, H.; Zeuner, R. A.; Farmaki, E.; Hatzistilianou, M. N.; Kakkas, I.; Kanariou, M. G.; Kapousouzi, A.; Liatsis, E.; Maggina, P.; Papadopoulou-Alataki, E.; Raptaki, M.; Speletas, M.; Tantou, S.; Goda, V.; Krivan, G.; Marodi, L.; Abolhassani, H.; Aghamohammadi, A.; Rezaei, N.; Feighery, C.; Leahy, T. R.; Ryan, P.; Batzir, N. A.; Garty, B. Z.; Tamary, H.; Aiuti, A.; Amodio, D.; Azzari, C.; Barzaghi, F.; Baselli, L. A.; Cancrini, C.; Carrabba, M.; Cazzaniga, M.; Cesaro, S.; Chinello, M.; Danieli, M. G.; Dellepiane, R. M.; Fabio, G.; Gambineri, E.; Lodi, L.; Lougaris, V.; Marasco, C.; Martire, B.; Marzollo, A.; Milito, C.; Moschese, V.; Pignata, C.; Plebani, A.; Porta, F.; Quinti, I.; Ricci, S.; Soresina, A.; Tommasini, A.; Vacca, A.; Vanessa, C.; Blaziene, A.; Sitkauskiene, B.; Gowin, E.; Heropolitanska-Pliszka, E.; Pietrucha, B.; Szaflarska, A.; Wiesik-Szewczyk, E.; Wolska-Kusnierz, B.; Esteves, I.; Faria, E.; Marques, L. H.; Neves, J. F.; Silva, S. L.; Teixeira, C.; Pereira da Silva, S.; Capilna, B. R.; Guseva, M. N.; Shcherbina, A.; Bobcakova, A.; Ciznar, P.; Gabzdilova, J.; Jesenak, M.; Kapustova, L.; Orosova, J.; Petrovicova, O.; Raffac, S.; Kopac, P.; Allende, L. M.; Antoli, A.; Blanch, G. R.; Carbone, J.; Dieli-Crimi, R.; Garcia-Prat, M.; Gil-Herrera, J.; Gonzalez-Granado, L. I.; Agullo, P. L.; Olbrich, P.; Parra-Martinez, A.; Paz-Artal, E.; Pleguezuelo, D. E.; Rodriguez, N. S.; Sanchez-Ramon, S.; Santos-Perez, J. L.; Solanich, X.; Soler-Palacin, P.; Gonzalez-Amores, M.; Ekwall, O.; Fasth, A.; Bitzenhofer-Gruber, M.; Candotti, F.; Dimitriou, F.; Heininger, U.; Holbro, A.; Jandus, P.; Kolios, A. G. A.; Marschall, K.; Schmid, J. P.; Posfay-Barbe, K. M.; Prader, S.; Reichenbach, J.; Steiner, U. C.; Truck, J.; Bredius, R. G.; de Kruijf- Bazen, S.; de Vries, E.; Henriet, S. S. V.; Kuijpers, T. W.; Potjewijd, J.; Rutgers, A.; Stol, K.; van Aerde, K. J.; Van den Berg, J. M.; van de Ven, A. A. J. M.; Montfrans, J.; Aydemir, S.; Baris, S.; Dogu, F.; Ikinciogullari, A.; Karakoc-Aydiner, E.; Kilic, S. S.; Kiykim, A.; Kokcu Karadag, S. I.; Kutukculer, N.; Ocak, S.; Unal, E.; Boyarchuk, O.; Hilfanova, A.; Kostyuchenko, L. V.; Alachkar, H.; Arkwright, P. D.; Baxendale, H. E.; Bernatoniene, J.; Coulter, T. I.; Garcez, T.; Goddard, S.; Gompels, M. M.; Grigoriadou, S.; Herriot, R.; Herwadkar, A.; Huissoon, A.; Ibberson, L.; Nademi, Z.; Noorani, S.; Parvin, S.; Steele, C. L.; Thomas, M.; Waruiru, C.; Yong, P. F. K.; Bourne, H