Results 61 to 70 of about 14,924 (223)

Phenotype‐specific immune profiles and outcomes in childhood autoimmune neutropenia: A 20‐year cohort study

British Journal of Haematology, EarlyView.
Summary Childhood autoimmune neutropenia (AIN) encompasses heterogeneous entities; phenotype‐specific immunological profiles and their relationship to infection outcomes remain incompletely defined. To characterise clinical, immunological and long‐term outcomes across distinct phenotypes of childhood AIN.
Ioanna Saougou, Chrysoula Kosmeri, Nikolaos Chaliasos, Irene Mavroudi, Charalampos Pontikoglou, Alexandros Makis   +5 more
wiley   +1 more source

A Phase 2 Trial of Frexalimab, a CD40L Antagonist, in Adolescents and Adults With Recent‐Onset Type 1 Diabetes (FABULINUS): Rationale and Study Design

Diabetes, Obesity and Metabolism, EarlyView.
ABSTRACT Introduction Type 1 diabetes (T1D) is a chronic autoimmune disease, characterised by progressive destruction of the insulin‐producing pancreatic β‐cells. Preserving remaining β‐cells at the time of diagnosis may improve long‐term outcomes. Frexalimab is a humanised monoclonal antibody specific for CD40L undergoing evaluation for treatment of ...
Andriy Cherkas, Baerbel Rotthaeuser, Niels Porksen, Pascale Labard, My‐Liên Nguyên‐Pascal, Mathieu Coudert, Baptiste Berthou, Marie Baccara‐Dinet, Gabor Boka, Mark Peakman, Felix Reschke, Michael J. Haller   +11 more
wiley   +1 more source

Clinical and genetic features of UNC13D deficiency with hypogammaglobulinemia

Frontiers in Immunology
BackgroundUNC13D deficiency is the most common form of familial hemophagocytic lymphohistiocytosis (FHL) in Asia. Hypogammaglobulinemia is a rare phenotype observed in both patients with FHL3 and sporadic hemophagocytic lymphohistiocytosis (HLH).
Linyan Xiong, Linyan Xiong, Linyan Xiong, Linyan Xiong, Qin Zhao, Qin Zhao, Qin Zhao, Qian Zhao, Qian Zhao, Qian Zhao, Zhiyong Zhang, Zhiyong Zhang, Zhiyong Zhang, Zhiyong Zhang, Yunfei An, Yunfei An, Yunfei An, Yunfei An, Xuemei Tang, Xuemei Tang, Xuemei Tang, Xuemei Tang, Hirokazu Kanegane, Xi Yang, Xi Yang, Xi Yang, Xi Yang, Xiaodong Zhao, Xiaodong Zhao, Xiaodong Zhao, Xiaodong Zhao   +30 more
doaj   +1 more source

Leukocytic function in hypogammaglobulinemia [PDF]

Journal of Clinical Investigation, 1970
The phagocytic, bactericidal, and metabolic capabilities of circulating blood leukocytes from three adults (two males, one female) with hypogammaglobulinemia and recurrent pneumonia, chronic sinusitis, and intestinal giardiasis were studied. These functions were found to be normal when leukocytes from the patients were incubated in media containing ...
I D, Mickenberg, R K, Root, S M, Wolff
openaire   +2 more sources

Regional Standardization of CLL Management: Results of a Delphi Consensus Process

European Journal of Haematology, EarlyView.
ABSTRACT The therapeutic landscape of chronic lymphocytic leukemia (CLL) has been profoundly transformed by the introduction of Bruton tyrosine kinase and BCL‐2 inhibitors. Despite improved survival outcomes, treatment selection remains complex, particularly in older patients with comorbidities, frailty, and increased infectious vulnerability.
Enrica Antonia Martino, Annalisa Chiarenza, Salvatrice Mancuso, Marco Rossi, Caterina Stelitano, Ignazio Abbene, Sergio Cabibbo, Daniele Caracciolo, Emilia Cotzia, Amalia Figuera, Antonino Greco, Vanessa Innao, Luciano Levato, Carla Marino, Giuseppe Mineo, Laura Nocilli, Giuseppa Penna, Marika Porrazzo, Giorgio Tona, Ernesto Vigna, Fortunato Morabito, Daniela D'Angela, Massimo Gentile   +22 more
wiley   +1 more source

Retrospective Study of Hypogammaglobulinemia A [PDF]

, 1990
In this study, we investigated 202 patients with hypogammaglobulinemia A, in whom blood IgA levels were 50 mg/dl or less, out of a total 29,302 patients examined between January 1978 and July 1988. The incidence of hypogammaglobulinemia was 0.689% of the
Obana, Yasuro, Ohba, Yasuhiro, Maruta, Mayumi, 大場, 康寛, 秋山, 利行, 竹中, 清悟, Takenaka, Kiyonori, Akiyama, Toshiyuki, 丸田, 真由美, 古田, 格, 尾鼻, 康朗, Furuta, Itaru   +11 more
core  

Hypogammaglobulinemia in sub-Saharan Africa: a case report and review of the literature

, 2015
Patients with hypogammaglobulinemia are susceptible to recurrent bacterial, viral, fungal, and parasitic infections. The most common clinical manifestation includes recurrent severe infections caused by encapsulated bacteria, in which antibody ...
Opoka, Robert, Hsu, J, Opoka, R, Lund, T C, Lund, TC   +4 more
core   +1 more source

Mitigating Teclistamab Toxicity: Prophylactic Tocilizumab and Timing of Immunoglobulin Replacement Therapy in a Nationwide Cohort

American Journal of Hematology, EarlyView.
Astrid Hundebøll Torpe, Jonathan Thorsen, Gustav Mathiasen, Katrine Fladeland Iversen, Sarah Farmer, Thomas Lund, Charlotte Toftmann Hansen, Lene Kongsgaard Nielsen, Nicolai Grønne Dahlager Jørgensen, Maja Ølholm Vase, Elena Manuela Teodorescu, Morten Salomo, Christina Wennerström, Tobias S. Slørdahl, Agoston Gyula Szabo   +14 more
wiley   +1 more source

NEMO‐NDAS: Case Report and Review of the Literature

Pediatric Dermatology, EarlyView.
ABSTRACT NEMO‐deleted exon 5 autoinflammatory syndrome (NEMO‐NDAS) is the result of a gain‐of‐function IKBKG pathogenic variant leading to dysregulated NF‐κB signaling and systemic inflammation. We present a case of NEMO‐NDAS in a 2‐year‐old female presenting with recurrent fevers, subcutaneous nodules, lymphadenopathy, and splenomegaly.
Angela Yang, Kristie Mar, Kimberly A. Morishita, Kevin Meesters, Antonio Torrelo, Joseph M. Lam   +5 more
wiley   +1 more source

Predictors of hypogammaglobulinemia in ANCA-associated vasculitis after a rituximab-based induction : a multicentre study

, 2022
OBJECTIVES: Rituximab has become the cornerstone of induction treatment in ANCA-associated vasculitis (AAV). B cell depletion may increase the risk of hypogammaglobulinemia, potentially leading to severe infections.
Tesar, Vladimir, Cid, Maria C, Jayne, David, Sophie Ohlsson, Bruchfeld, Annette, Sabiu, Gianmarco, Gallieni, Maurizio, Mescia, Federica,, Vaglio, Augusto, Cozzolino, Mario, Lorraine Harper, Ohlsson, Sophie, Rona Smith, Cassia, Matthias Arnaldo, Vladimir Tesar, Cassia, Matthias Arnaldo,, Federico Alberici, Maurizio Gallieni, David Jayne, Bruchfeld, Annette,, Sinico, Renato Alberto,, Manuel Alfredo Podestà, Hruskova, Zdenka, Mario Cozzolino, Annette Bruchfeld, Lund University., Francesco Scolari, Zdenka Hruskova, Tedesco, Martina, Gianmarco Sabiu, Mescia, Federica, Ricchiuto, Anna,, Podestà, Manuel Alfredo, Podestà, Manuel Alfredo,, Holle, Julia,, Scolari, Francesco, Smith, Rona,, Gunnarsson, Iva, Baslund, Bo, Federica Mescia, Matthias Arnaldo Cassia, Julia Holle, Bo Baslund, Augusto Vaglio, Gunnarsson, Iva,, Anna Ricchiuto, Smith, Rona, Ricchiuto, Anna, Sinico, Renato Alberto, Holle, Julia, Iva Gunnarsson, Martina Tedesco, Renato Alberto Sinico, Alberici, Federico, Cid, Maria C., Ohlsson, Sophie,, Maria C Cid, Harper, Lorraine, Tedesco, Martina,   +58 more
core   +1 more source