Results 151 to 160 of about 21,130 (289)

The IL‐10/IL‐6 Ratio and the Risk Score: Two Cytokines‐Based Predictors for Malignancy‐Associated Hemophagocytic Lymphohistiocytosis in Adults (M‐HLHa)

American Journal of Hematology, EarlyView.
ABSTRACT The predictive value of cytokines (CK) for malignancy‐associated adult hemophagocytic lymphohistiocytosis (M‐HLHa) remains uncertain. We evaluated a cytokine‐based Risk Score (RS) and the IL‐10/IL‐6 Ratio to predict M‐HLHa. Adult patients (n = 112) from the French HLH cohort (NCT02113917) with complete data for nine key HLH related CK measured
Coralie Bloch, Stephanie Chhun, Nanthara Sritharan, Marine Gil, Raphael Lhote, Marouane Boubaya, Olivier Lambotte, David Launay, Claire Larroche, Estibaliz Lazaro, Francois Liffermann, Marc Michel, Jean‐Marie Michot, Pierre Morel, Louis Terriou, Geoffrey Urbansk, Jean‐Francois Viallard, Morgane Cheminant, Felipe Suarez, Yves Lepelletier, Geneviève de Saint Basile, Olivier Hermine, the French HLH Study Group, Coralie Bloch, Jean Philippe Jais, Francois Liffermann, Pierre Morel, Morgane Cheminant, Felipe Suarez, Olivier Hermine, Fabrice Bonnet, Pascal Godemer, Damaj Gandhi, Olivier Fain, Olivier Lambotte, David Launay, Louis Terriou, Claire Larroche, Estibaliz Lazaro, Jean‐Francois Viallard, Francois Liffermann, Olivier Lortholary, Marc Michel, Jean‐Marie Michot, Pierre Morel, Frederic Pene, Antoinette Perlat, Geoffrey Urbanski   +47 more
wiley   +1 more source

Primary Immunodeficiencies in Children Initially Admitted with Gastrointestinal/Liver Manifestations. [PDF]

Pediatr Gastroenterol Hepatol Nutr, 2023
Cakir M, Yakici N, Sag E, Kaya G, Bahadir A, Cebi AH, Orhan F.   +6 more
europepmc   +1 more source

The 9th International RASopathies Symposium

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT The RASopathies are a group of congenital disorders with overlapping clinical manifestations that are caused by pathogenic germline or early somatic variants that result in the hyperactivation of the RAS/mitogen‐activated protein kinase (MAPK) signaling pathway.
Pau Castel, Lisa Schoyer, Beth Stronach, Raya Bogdanova, Anton M. Bennett, Jaishri Blakeley, Miriam Bornhorst, Tammy Bowers, Saskia M. Brachmann, Emma Burkitt‐Wright, Kathryn Chatfield, Alessandro De Luca, Khalil El‐Chammas, Abdul Elkadri, John E. Fortunato, Bruce D. Gelb, Anne Goriely, Karen Gripp, Kassidy Grover, Lindsay Homan, Kenneth A. Kern, Maija Kiuru, Charles (Chuck) Lawson, Yong‐Seok Lee, Frank McCormick, Gina Ney, Cristina Nuevo‐Tapioles, Sara Pardej, Elizabeth I. Pierpont, Julia Plank, Nancy Ratner, Katherine A. Rauen, J. Elliott Robinson, Les Rogers, Sarah E. Sheppard, Keir Shiels, David Stevenson, Dagmar Tiemens, Matthew Traylor, K. Nicole Weaver, Marielle Yohe, Tamar Green   +41 more
wiley   +1 more source

Assessment of autoantibodies in paediatric population with primary immunodeficiencies: a pilot study. [PDF]

BMC Immunol, 2023
Pieniawska-Śmiech K, Lewandowicz-Uszyńska A, Zemelka-Wiacek M, Jutel M.   +3 more
europepmc   +1 more source

Atypical Clinical Course of Griscelli Syndrome Type 2 With Primarily Neurologic Presentation and Adult‐Onset in a 46‐Year‐Old Male

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Griscelli Syndrome Type 2 (GS2) is a rare autosomal recessive disorder caused by pathogenic mutations in the RAB27A gene. Typically, it is characterized by cutaneous hypopigmentation, immunodeficiency, with or without neurological abnormalities secondary to hemophagocytic lymphohistiocytosis (HLH). Without treatment, GS2 often results in fatal
Dzhoy Papingi, Michael Kutsche, Helena Lichtenfeld, Fanny Kortüm, Angela Abicht, Laura Herrmann, Theresia Herget   +6 more
wiley   +1 more source

Immunity to Cryptosporidium: Lessons from Acquired and Primary Immunodeficiencies. [PDF]

J Immunol, 2022
Cohn IS, Henrickson SE, Striepen B, Hunter CA.   +3 more
europepmc   +1 more source

Effect of Budesonide Nasal Irrigation in Patients With Chronic Rhinosinusitis With Nasal Polyps Without Prior Sinus Surgery: A Randomized, Double‐Blind, Placebo‐Controlled Study

International Forum of Allergy &Rhinology, EarlyView.
ABSTRACT Background The indication for nasal irrigation with corticosteroids after sinus surgery in patients with Chronic Rhinosinusitis with Nasal Polyps (CRSwNP) is well established, as surgery facilitates distribution throughout the sinonasal cavity.
Juliana Sant'Ana, Isabela Pontes, Caio Floriano, Renato Rios, Marlos Cortez, Marcel Miyake   +5 more
wiley   +1 more source

SARS-CoV-2 Infection and Response to COVID-19 Vaccination in Patients With Primary Immunodeficiencies. [PDF]

J Infect Dis, 2023
Paris R.
europepmc   +1 more source

Extent of Sinus Surgery Is Associated With Disease Control in Biologic Treated Type 2 Dominant CRS

International Forum of Allergy &Rhinology, EarlyView.
ABSTRACT Background A greater benefit of biologics is observed after surgery in Type 2 chronic rhinosinusitis with nasal polyps (CRSwNP). However, the extent of surgery remains undefined in many studies. This study evaluated the extent of surgery on disease control in patients receiving biologics for refractory Type 2 dominant CRSwNP.
Nicholas J. Campion, Peta‐Lee Sacks, Lu Hui Png, Ruoxin Hua, Rhea Darbari Kaul, Raquel Alvarado, Christine Choy, Raymond Sacks, Peter Earls, Raewyn G. Campbell, Larry Kalish, Richard J. Harvey   +11 more
wiley   +1 more source

Newborn screening as a fully integrated system to stimulate equity in neonatal screening in Europe

The Lancet Regional Health. Europe, 2022
Maurizio Scarpa, James R. Bonham, Carlo Dionisi-Vici, Johan Prevot, Martine Pergent, Isabelle Meyts, Nizar Mahlaoui, Peter C.J.I. Schielen   +7 more
doaj   +1 more source