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Background New treatments are currently offering new opportunities and challenges in clinical management and research in the migraine field. There is the need of homogenous criteria to identify candidates for treatment escalation as well as of reliable criteria to identify refractoriness to treatment. To overcome those issues, the European Headache Federation (EHF) issued a Consensus document to propose criteria to approach difficult-to-treat migraine patients in a standardized way. The Consensus proposed well-defined criteria for resistant migraine (i.e., patients who do not respond to some treatment but who have residual therapeutic opportunities) and refractory migraine (i.e., patients who still have debilitating migraine despite maximal treatment efforts). The aim of this study was to better understand the perceived impact of resistant and refractory migraine and the attitude of physicians involved in migraine care toward those conditions. Methods We conducted a web-questionnaire-based cross-sectional international study involving physicians with interest in headache care. Results There were 277 questionnaires available for analysis. A relevant proportion of participants reported that patients with resistant and refractory migraine were frequently seen in their clinical practice (49.5% for resistant and 28.9% for refractory migraine); percentages were higher when considering only those working in specialized headache centers (75% and 46% respectively). However, many physicians reported low or moderate confidence in managing resistant (8.1% and 43.3%, respectively) and refractory (20.7% and 48.4%, respectively) migraine patients; confidence in treating resistant and refractory migraine patients was different according to the level of care and to the number of patients visited per week. Patients with resistant and refractory migraine were infrequently referred to more specialized centers (12% and 19%, respectively); also in this case, figures were different according to the level of care. Conclusions This report highlights the clinical relevance of difficult-to-treat migraine and the presence of unmet needs in this field. There is the need of more evidence regarding the management of those patients and clear guidance referring to the organization of care and available opportunities.

BACKGROUND: Several models have been proposed to predict kidney graft failure in adult recipients but none in younger recipients. Our objective was to propose a dynamic prediction model for graft failure in young kidney transplant recipients. METHODS: We included 793 kidney transplant recipients waitlisted before the age of 18 years who received a first kidney transplantation before the age of 21 years in France in 2002-13 and survived >90 days with a functioning graft. We used a Cox model including baseline predictors only (sex, age at transplant, primary kidney disease, dialysis duration, donor type and age, human leucocyte antigen matching, cytomegalovirus serostatus, cold ischaemia time and delayed graft function) and two joint models also accounting for post-transplant estimated glomerular filtration rate (eGFR) trajectory. Predictive performances were evaluated using a cross-validated area under the curve (AUC) and R2 curves. RESULTS: When predicting the risk of graft failure from any time within the first 7 years after paediatric kidney transplantation, the predictions for the following 3 or 5 years were accurate and much better with the joint models than with the Cox model (AUC ranged from 0.83 to 0.91 for the joint models versus 0.56 to 0.64 for the Cox model). CONCLUSION: Accounting for post-transplant eGFR trajectory strongly increased the accuracy of graft failure prediction in young kidney transplant recipients.

ispartof: Physical Review C vol:102 issue:1 status: published

Background: The γ softness of 136Nd makes it possible to study the shape changes induced by two-proton or two-neutron excitation. Purpose: We measure the lifetimes of two-quasiparticle states of the bands based on the 10+ states at 3296 and 3279 keV to investigate the shape change induced by the alignment of two protons or two neutrons in the h11/2 orbital. Methods: The recoil-distance Doppler shift method was used for the study of 136Nd studies, which was formed by the fusion reaction 120Sn(20Ne, 4n)136Nd, at Ebeam = 85 MeV. Calculations were performed within the microscopic-macroscopic approach, based on the deformed Woods-Saxon single-particle potential and the Yukawa-plus-exponential macroscopic energy. Results: The lifetime of the 10+ state at 3279 keV of 136Nd was measured to be T 10+1/2 = 1.63(9) ns. The lifetimes of the 2+ state at 374 keV and of the 12+ state at 3686 keV of the ground band were also measured to be T 2+1/2 = 26.5(14) ps and T 12+1/2 = 22.5(14) ps. Conclusions: The measured lifetime of 10+ the state at 3279 keV together with other observables confirm the structure change in 136Nd. A rather small reduced hindrance of the electromagnetic decay of the 10+ state at 3279 keV would be consistent with its K-mixed character.

© 2019 authors. Published by the American Physical Society. Published by the American Physical Society under the terms of the Creative Commons Attribution 4.0 International license. Further distribution of this work must maintain attribution to the author(s) and the published article's title, journal citation, and DOI. Excited states in Sn133 were investigated through the β decay of In133 at the ISOLDE facility. The ISOLDE Resonance Ionization Laser Ion Source (RILIS) provided isomer-selective ionization for In133, allowing us to study separately, and in detail, the β-decay branch of In133Jπ=(9/2+) ground state and its Jπ=(1/2-) isomer. Thanks to the large spin difference of the two β-decaying states of In133, it is possible to investigate separately the lower and higher spin states in the daughter, Sn133, and thus to probe independently different single-particle and single-hole levels. We report here new γ transitions observed in the decay of In133, including those assigned to the deexcitation of the neutron-unbound states. ispartof: Physical Review C vol:99 issue:2 status: published

Excited states in Sn133 were investigated through the β decay of In133 at the ISOLDE facility. The ISOLDE Resonance Ionization Laser Ion Source (RILIS) provided isomer-selective ionization for In133, allowing us to study separately, and in detail, the β-decay branch of In133Jπ=(9/2+) ground state and its Jπ=(1/2-) isomer. Thanks to the large spin difference of the two β-decaying states of In133, it is possible to investigate separately the lower and higher spin states in the daughter, Sn133, and thus to probe independently different single-particle and single-hole levels. We report here new γ transitions observed in the decay of In133, including those assigned to the deexcitation of the neutron-unbound states.

Background: Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenic cause of renal failure. For several decades, ADPKD was regarded as an adult-onset disease. In the past decade, it has become more widely appreciated that the disease course begins in childhood. However, evidence-based guidelines on how to manage and approach children diagnosed with or at risk of ADPKD are lacking. Also, scoring systems to stratify patients into risk categories have been established only for adults. Overall, there are insufficient data on the clinical course during childhood. We therefore initiated the global ADPedKD project to establish a large international pediatric ADPKD cohort for deep characterization. Methods: Global ADPedKD is an international multicenter observational study focusing on childhood-diagnosed ADPKD. This collaborative project is based on interoperable Web-based databases, comprising 7 regional and independent but uniformly organized chapters, namely Africa, Asia, Australia, Europe, North America, South America, and the United Kingdom. In the database, a detailed basic data questionnaire, including genetics, is used in combination with data entry from follow-up visits, to provide both retrospective and prospective longitudinal data on clinical, radiologic, and laboratory findings, as well as therapeutic interventions. Discussion: The global ADPedKD initiative aims to characterize in detail the most extensive international pediatric ADPKD cohort reported to date, providing evidence for the development of unified diagnostic, follow-up, and treatment recommendations regarding modifiable disease factors. Moreover, this registry will serve as a platform for the development of clinical and/or biochemical markers predicting the risk of early and progressive disease. C1 [De Rechter, Stephanie; Mekahli, Djalila] Univ Hosp Leuven, Dept Pediat Nephrol, Herestr 49, B-3000 Leuven, Belgium. [De Rechter, Stephanie; Mekahli, Djalila] Katholieke Univ Leuven, Dept Dev & Regenerat, PKD Res Grp, Leuven, Belgium. [Bockenhauer, Detlef] UCL Ctr Nephrol, London, England. [Bockenhauer, Detlef] Great Ormond St Hosp NHS Fdn Trust, London, England. [Guay-Woodford, Lisa M.] Childrens Natl Hlth Syst, Ctr Translat Sci, Washington, DC USA. [Liu, Isaac] Natl Univ Hlth Syst, Khoo Teck Puat Natl Univ, Childrens Med Inst, Singapore, Singapore. [Mallett, Andrew J.] Royal Brisbane & Womens Hosp, Kidney Hlth Serv, Brisbane, Qld, Australia. [Mallett, Andrew J.] Royal Brisbane & Womens Hosp, Conjoint Renal Res Lab, Brisbane, Qld, Australia. [Mallett, Andrew J.] Univ Queensland, Fac Med, Brisbane, Qld, Australia. [Mallett, Andrew J.] Univ Queensland, Inst Mol Biosci, Brisbane, Qld, Australia. [Mallett, Andrew J.] KidGen Collaborat & Australian Genom Hlth Allianc, Melbourne, Vic, Australia. [Soliman, Neveen A.] Cairo Univ, Ctr Pediat Nephrol & Transplantat, Kasr Al Ainy Sch Med, Dept Pediat, Cairo, Egypt. [Sylvestre, Lucimary C.] Hosp Pequeno Principe, Curitiba, Parana, Brazil. [Schaefer, Franz] Heidelberg Univ, Ctr Pediat & Adolescent Med, Div Pediat Nephrol, Med Ctr, Heidelberg, Germany. [Liebau, Max C.] Univ Hosp Cologne, Dept Pediat, Cologne, Germany. [Liebau, Max C.] Univ Hosp Cologne, Ctr Mol Med, Cologne, Germany. [Adamczyk, P.; Bjanid, O.; Brylka, A.; Morawiec-Knysak, A.; Szczepanska, M.] Dept Pediat, Zabrze, Poland. [Akinci, N.] Sariyer SISLI Hamidiye Etfal Res & Educ Hosp, Istanbul, Turkey. [Alpay, H.; Cicek, N.; Gokce, I] Marmara Univ, Sch Med, Div Pediat Nephrol, Istanbul, Turkey. [Ardelean, C.; Chirita, A.; Gafencu, M.; Stroescu, R.] Timisoara Children Hosp, Timisoara, Romania. [Ayasreh, N.; Furlano, M.; Torra, R.] Fundacio Puigvert, Barcelona, Spain. [Aydin, Z.; Bayrakci, U. S.] Ankara Univ Hlth Sci, Child Hlth & Dis, Ankara, Turkey. [Bael, A.; Docx, M.; Segers, N.] Koningin Paola Kinderziekenhuis Antwerpen, Antwerp, Belgium. [Baudouin, V; Cambier, A.; Couderc, A.; Dossier, C.; Kwon, V] Hop Robert Debre, AP HP, Paris, France. [Bensman, A.; Biebuyck, A.; Boyer, O.; Charbit, M.; Heidet, L.; Krid, S.; Krug, P.; Salomon, R.] Pediat Nephrol Necker Hosp, Paris, France. [Bialkevich, H.; Kazyra, I] 2nd City Childrens Clin Hosp, Natl Ctr Pediat Nephrol & RRT, Minsk, BELARUS. [Caliskan, S.; Ozcan, S.; Saygili, S. K.] Istanbul Cerrahpasa Fac Med, Istanbul, Turkey. [Camelio, A.; Nobili, F.; Vieux, R.] CHU Besancon, Besancon, France. [Carbone, V; Diomeda, F.; Torres, D.] Pediat Nephrol Unit Bari, Bari, Italy. [Chiodini, B.] HUDERF, Brussels, Belgium. [Collard, L.] CHR La Citadelle, Liege, Belgium. [Conceicao, M.; Teixeira, A.] Ctr Hosp Porto, Ctr Materno Infantil Norte, Porto, Portugal. [Constantinescu, I; Lungu, A. C.; Marin, A.; Negru, I; Stroescu, R.] Fundeni Clin Inst, Bucharest, Romania. [Crapella, B.; Giani, M.; Mastrangelo, A.; Montini, G.] Fdn IRCCS Ca Granda, Pediat Nephrol Dialysis & Transplant Unit, Milan, Italy. [Cvetkovic, M.; Gojkovic, I] Univ Childrens Hosp, Belgrade, Serbia. [Dima, B.] Clin Europe Hop St Elisabeth, Brussels, Belgium. [Dolan, N.] Our Ladys Childrens Hosp, Dublin, Ireland. [Drozdz, D.; Miklaszewska, M.; Zachwieja, K.] Jagiellonian Univ, Med Coll Cracow, Pediat Nephrol & Hypertens, Krakow, Poland. [Drube, J.; Pape, L.] Hannover Med Sch, Hannover, Germany. [Dunand, O.; Leroy, V] Pediat Nephrol Unit St Denis, St Denis, Reunion, France. [Dusan, P.; Spasojevic, B.; Stabouli, S.] Aristotle Univ Thessaloniki, Dept Pediat, Thessaloniki, Greece. [Eid, L. A.] Dubai Hosp, Pediat Nephrol Dept, Dubai, U Arab Emirates. [Emma, F.; Massella, L.] Bambino Gesu Pediat Hosp, Rome, Italy. [Espino Hernandez, M.] Hosp Infantil 12 Octubre Madrid, Madrid, Spain. [Fila, M.; Hemery, F.; Morin, D.] CHU Arnaud Villeneuve, Montpellier, France. [Ghuysen, Ms] CHU Liege, Liege, Belgium. [Giordano, M.] Pediat Nephrol Unit, Bari, Italy. [Girisgen, I; Yuksel, S.] Pamukkale Univ, Med Fac, Dept Pediat Nephrol, Denizli, Turkey. [Godefroid, N.; Ranguelov, N.] Clin Univ St Luc, Brussels, Belgium. [Godron-Dubrasquet, A.; Harambat, J.; Ilanas, B.] Bordeaux Univ Childrens Hosp, Bordeaux, France. [Gonzalez, E.] Childrens Univ Hosp, Geneva, Switzerland. [Groothoff, J. W.] Emma Childrens Hosp, Amsterdam, Netherlands. [Guarino, S.; La Manna, A.; Marzuillo, P.] Univ Campania Luigi Vanvitelli, Caserta, Italy. [Guffens, A.] CHC Clin Esperence, Montegnee, Belgium. [Hansen, P.] CHU Tivoli, La Louviere, Belgium. [Haumann, S.] Univ Klinikum Koln, Cologne, Germany. [He, G.] Foshan Women & Children Hosp, Foshan, Peoples R China. [Helmy, R.] Cairo Univ, Kasr Al Ainy Sch Med, Cairo, Egypt. [Hooman, N.; Otoukesh, H.] Iran Univ Med Sci, Aliasghar Clin Res Dev Unit, Tehran, Iran. [Janssens, P.] Univ Hosp Brussels, Brussels, Belgium. [Karamaria, S.; Prytula, A.; Raes, A.; Snauwaert, E.; Vande Walle, J.] UZ Gent, Ghent, Belgium. [Koenig, J.] Univ Hosp Muenster, Munster, Germany. [Litwin, M.; Obrycki, L.] Childrens Mem Hlth Inst, Warsaw, Poland. [Lombet, J.] CHR Citadelle, Liege, Belgium. [Longo, G.; Murer, L.] Hosp Univ Padova, Pediat Nephrol Dialysis & Transplant Unit, Padua, Italy. [Mallawaarachchi, A.] Garvan Inst, Darlinghurst, NSW, Australia. [Mallawaarachchi, A.] Royal Prince Alfred Hosp, Camperdown, NSW, Australia. [Mallawaarachchi, A.; McCarthy, H.; Quinlan, C.] KidGen, Sydney, NSW, Australia. [McCarthy, H.] Childrens Hosp Westmead, Westmead, NSW, Australia. [McCarthy, H.] Sydney Childrens Hosp, Sydney, NSW, Australia. [Papizh, S.; Prikhodina, L.] Pirogov Russian Nat Res Med Uni, Res & Clin Inst Pediat, Moscow, Russia. 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Y.] Istanbul Univ, Fac Med, Pediat Nephrol Dept, Istanbul, Turkey.

International audience; Excited states in $^{133}Sn$ were investigated through the $\beta$ decay of $^{133}In$ at the ISOLDE facility. The ISOLDE Resonance Ionization Laser Ion Source (RILIS) provided isomer-selective ionization for $^{133}In$, allowing us to study separately, and in detail, the β-decay branch of $^{133}In$ J$^{\pi}$=(9/2$^+$) ground state and its J$^{\pi}$=(1/2$^−$) isomer. Thanks to the large spin difference of the two β-decaying states of $^{133}In$, it is possible to investigate separately the lower and higher spin states in the daughter, $^{133}Sn$, and thus to probe independently different single-particle and single-hole levels. We report here new $\gamma$ transitions observed in the decay of $^{133}In$, including those assigned to the deexcitation of the neutron-unbound states.

International audience; Background: The γ softness of Nd136 makes it possible to study the shape changes induced by two-proton or two-neutron excitation. Purpose: We measure the lifetimes of two-quasiparticle states of the bands based on the 10+ states at 3296 and 3279 keV to investigate the shape change induced by the alignment of two protons or two neutrons in the h11/2 orbital. Methods: The recoil-distance Doppler shift method was used for the study of Nd136 studies, which was formed by the fusion reaction Sn120(Ne20,4n)Nd136, at Ebeam=85 MeV. Calculations were performed within the microscopic-macroscopic approach, based on the deformed Woods-Saxon single-particle potential and the Yukawa-plus-exponential macroscopic energy. Results: The lifetime of the 10+ state at 3279 keV of Nd136 was measured to be T1/210+=1.63(9) ns. The lifetimes of the 2+ state at 374 keV and of the 12+ state at 3686 keV of the ground band were also measured to be T1/22+=26.5(14) ps and T1/212+=22.5(14) ps. Conclusions: The measured lifetime of 10+ the state at 3279 keV together with other observables confirm the structure change in Nd136. A rather small reduced hindrance of the electromagnetic decay of the 10+ state at 3279 keV would be consistent with its K-mixed character.

10 pags., 8 figs., 1 tab.-- Open Access funded by Creative Commons Atribution Licence 4.0, Excited states in Sn133 were investigated through the ß decay of In133 at the ISOLDE facility. The ISOLDE Resonance Ionization Laser Ion Source (RILIS) provided isomer-selective ionization for In133, allowing us to study separately, and in detail, the ß-decay branch of In133J¿=(9/2+) ground state and its J¿=(1/2-) isomer. Thanks to the large spin difference of the two ß-decaying states of In133, it is possible to investigate separately the lower and higher spin states in the daughter, Sn133, and thus to probe independently different single-particle and single-hole levels. We report here new ¿ transitions observed in the decay of In133, including those assigned to the deexcitation of the neutron-unbound states., We acknowledge the support of the ISOLDE Collaboration and technical teams. This work was supported in part by the Polish National Science Center under Contract No. UMO-2015/18/E/ST2/00217 and under Contract No. UMO-2015/18/M/ST2/00523, by the Spanish MINECO via FPA2015-65035-P project, by the Portuguese FCT via CERN/FIS-NUC/0004/2015 and CERN-FIS-PAR-0005-2017 projects. The research leading to these results has received funding from the European Union’s Horizon 2020 research and innovation programme under Grant Agreement No. 654002.

BACKGROUND: Primary distal renal tubular acidosis (dRTA) is a rare disorder, and we aimed to gather data on treatment and long-term outcome. METHODS: We contacted paediatric and adult nephrologists through European professional organizations. Responding clinicians entered demographic, biochemical, genetic and clinical data in an online form. RESULTS: Adequate data were collected on 340 patients (29 countries, female 52%). Mutation testing had been performed on 206 patients (61%); pathogenic mutations were identified in 170 patients (83%). The median (range) presentation age was 0.5 (0-54) years and age at last follow-up was 11.0 (0-70.0) years. Adult height was slightly below average with a mean (SD score) of -0.57 (±1.16). There was an increased prevalence of chronic kidney disease (CKD) Stage ≥2 in children (35%) and adults (82%). Nephrocalcinosis was reported in 88%. Nephrolithiasis was more common with SLC4A1 mutations (42% versus 21%). Thirty-six percent had hearing loss, particularly in ATP6V1B1 (88%). The median (interquartile range) prescribed dose of alkali (mEq/kg/day) was 1.9 (1.2-3.3). Adequate metabolic control (normal plasma bicarbonate and normocalciuria) was achieved in 158 patients (51%), more commonly in countries with higher gross domestic product (67% versus 23%), and was associated with higher height and estimated glomerular filtration rate. CONCLUSION: Long-term follow-up from this large dRTA cohort shows an overall favourable outcome with normal adult height for most and no patient with CKD Stage 5. However, 82% of adult patients have CKD Stages 2-4. Importance of adequate metabolic control was highlighted by better growth and renal function but was achieved in only half of patients.

Water accumulation in the interstitium (edema) and the peritoneum (ascites) of nephrotic patients is classically thought to stem from the prevailing low plasma albumin concentration and the decreased transcapillary oncotic pressure gradient. However several clinical and experimental observations suggest that it might also stem from changes in capillary permeability. We addressed this hypothesis by studying the peritoneum permeability of rats with puromycin aminonucleoside induced nephrotic syndrome. The peritoneum of puromycin aminonucleoside rats displayed an increase in the water filtration coefficient of paracellular and transcellular pathways and a decrease in the reflection coefficient to proteins. It also displayed oxidative stress and subsequent activation of NF ?B. Scavenging of reactive oxygen species and inhibition of NF ?B prevented the changes in the water permeability and reflection coefficient to proteins and reduced the volume of ascites by over 50. Changes in water permeability were associated with the overexpression of the water channel aquaporin 1 which was prevented by reactive oxygen species scavenging and inhibition of NF ?B. In conclusion nephrotic syndrome is associated with an increased filtration coefficient of the peritoneum and a decreased reflection coefficient to proteins. These changes which account for over half of ascite volume are triggered by oxidative stress and subsequent activation of NF ?B.