Resolution of unilateral sensorineural hearing loss in a pediatric patient with a severe phenotype of Muckle-Wells syndrome treated with Anakinra: a case report and review of the l... by Cinzia Marchica, Faisal Zawawi, Dania Basodan, Rosie Scuccimarri, Sam J. Daniel

“…Abstract Background Muckle-Wells syndrome (MWS) is a rare auto-inflammatory disease characterized by the presence of recurrent urticaria, deafness and amyloidosis. Progressive sensorineural hearing loss (SNHL) is reported to occur in up to 85% of patients occurring in the second and third decades and as early as the first decade in patients with a more severe phenotype, thus potentially having a significant impact on a child’s development. …”
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Clinical report of Mediterranean fever in a child in Ukraine – Don’t miss it! by К.Y. Pashenko, Т.V. Frolova, Y.V. Кarpushenko, G.R. Muratov, О.М. Savvo, Т.О. Khalturina, О.М. Ashcheulov

“…Its long term without appropriate treatment the disease can lead to a violation of the child's physical development and result in the development of amyloidosis. The aim is to focus the attention of the medical community of Ukraine and inform European medical specialists on the FMF cases due to migration changes in Ukraine. …”
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Ocular Signs and Ocular Comorbidities in Monoclonal Gammopathy: Analysis of 80 Subjects by Kitti Kormányos, Klaudia Kovács, Orsolya Németh, Gábor Tóth, Gábor László Sándor, Anita Csorba, Cecília Nóra Czakó, Achim Langenbucher, Zoltán Zsolt Nagy, Gergely Varga, László Gopcsa, Gábor Mikala, Nóra Szentmáry

“…The hematological diagnosis was MGUS in 9 (11.25%), multiple myeloma in 61 (76.25%), smoldering myeloma in 6 (7.50%), and amyloidosis or Waldenström macroglobulinemia in 2 cases (2.50%–2.50%). …”
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The relation of MEFV gene variants to clinical phenotype and selected laboratory markers in Egyptian patients with familial Mediterranean fever by Hala T. El-Bassyouni, Ghada Nour Eldeen, Mona F. Sokkar, Mohamed B. Taher, Mohammed M. Sayed-Ahmed, Iman I. Salama, Hala M. Raslan

“…Met694Val variant could be a possible risk factor for developing amyloidosis. Investigating the whole MEFV gene is recommended to fully understand the molecular background of FMF cases and properly establish a good correlation with the variable phenotypes.…”
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Deeper response predicts better outcomes in high-risk-smoldering-myeloma: results of the I-PRISM phase II clinical trial by Omar Nadeem, Michelle P. Aranha, Robert Redd, Michael Timonian, Sophie Magidson, Elizabeth D. Lightbody, Jean-Baptiste Alberge, Luca Bertamini, Ankit K. Dutta, Habib El-Khoury, Mark Bustoros, Jacob P. Laubach, Giada Bianchi, Elizabeth O’Donnell, Ting Wu, Junko Tsuji, Kenneth C. Anderson, Gad Getz, Lorenzo Trippa, Paul G. Richardson, Romanos Sklavenitis-Pistofidis, Irene M. Ghobrial

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The ABC transporter A7 modulates neuroinflammation via NLRP3 inflammasome in Alzheimer’s disease mice by Irene Santos-García, Pablo Bascuñana, Mirjam Brackhan, María Villa, Ivan Eiriz, Thomas Brüning, Jens Pahnke

“…Methods We developed the first humanized, Cre-inducible ABCA7 flx knock-in mouse model, crossbred it with the APPPS1-21 β-amyloidosis model, and generated constitutive ABCA7ko and microglia Cx3cr1-specific conditional ABCA7ko AD mice. …”
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APOE Christchurch enhances a disease-associated microglial response to plaque but suppresses response to tau pathology by Kristine M. Tran, Nellie E. Kwang, Claire A. Butler, Angela Gomez-Arboledas, Shimako Kawauchi, Cassandra Mar, Donna Chao, Rocio A. Barahona, Celia Da Cunha, Kate I. Tsourmas, Zechuan Shi, Shuling Wang, Sherilyn Collins, Amber Walker, Kai-Xuan Shi, Joshua A. Alcantara, Jonathan Neumann, Duc M. Duong, Nicholas T. Seyfried, Andrea J. Tenner, Frank M. LaFerla, Lindsay A. Hohsfield, Vivek Swarup, Grant R. MacGregor, Kim N. Green

“…Methods We introduced the R136S variant into mouse Apoe (ApoeCh) and investigated its effect on the development of AD-related pathology using the 5xFAD model of amyloidosis and the PS19 model of tauopathy. We used immunohistochemical and biochemical analysis along with single-cell spatial omics and bulk proteomics to explore the impact of the ApoeCh variant on AD pathological development and the brain’s response to plaques and tau. …”
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Clinical impact of [18F]FDG-PET/CT in ARI0002h treatment, a CAR-T against BCMA for relapsed/refractory multiple myeloma by Inés Zugasti, Marta Tormo-Ratera, Aina Oliver-Caldés, Juan Carlos Soler-Perromat, Verónica González-Calle, David F. Moreno, Valentín Cabañas, Nieves López-Muñoz, Álvaro Bartolomé-Solanas, Marta Español-Rego, Juan Luis Reguera-Ortega, Laura Rosiñol, Lucía López-Corral, Natalia Tovar, Luis Gerardo Rodríguez-Lobato, Rosa Maria Alvarez Perez, Sara Varea, Eulàlia Olesti, Adolfo Gomez-Grande, Laura Frutos, Pilar Tamayo, Manel Juan, José M. Moraleda, Álvaro Urbano-Ispizua, E. Azucena González-Navarro, Joaquín Martínez-López, Maria-Victoria Mateos, Xavier Tomás, Xavier Setoain, Carlos Fernández de Larrea

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