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Your search keyword '"Neehus, Anna-Lena"' showing total 8 results
Start Over Author "Neehus, Anna-Lena" Journal journal of clinical immunology
8 results on '"Neehus, Anna-Lena"'

1. Recombinant IFN-γ1b Treatment in a Patient with Inherited IFN-γ Deficiency

Author

Rosain, Jérémie, primary, Kiykim, Ayca, additional, Michev, Alexandre, additional, Kendir-Demirkol, Yasemin, additional, Rinchai, Darawan, additional, Peel, Jessica N., additional, Li, Hailun, additional, Ocak, Suheyla, additional, Ozdemir, Pinar Gokmirza, additional, Le Voyer, Tom, additional, Philippot, Quentin, additional, Khan, Taushif, additional, Neehus, Anna-Lena, additional, Migaud, Mélanie, additional, Soudée, Camille, additional, Boisson-Dupuis, Stéphanie, additional, Marr, Nico, additional, Borghesi, Alessandro, additional, Casanova, Jean-Laurent, additional, and Bustamante, Jacinta, additional

Published
2024
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2. Fulminant Viral Hepatitis in Two Siblings with Inherited IL-10RB Deficiency

Author

Korol, Cecilia B., primary, Belkaya, Serkan, additional, Alsohime, Fahad, additional, Lorenzo, Lazaro, additional, Boisson-Dupuis, Stéphanie, additional, Brancale, Joseph, additional, Neehus, Anna-Lena, additional, Vilarinho, Silvia, additional, Zobaida, Alsum, additional, Halwani, Rabih, additional, Al-Muhsen, Saleh, additional, Casanova, Jean-Laurent, additional, and Jouanguy, Emmanuelle, additional

Published
2022
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3. A New Patient with p40phox Deficiency and Chronic Immune Thrombocytopenia.

Author

Neehus, Anna-Lena, Fusaro, Mathieu, NCF4 consortium, Bodemer, Christine, Roelens, Marie, Gervais, Adrian, Casanova, Jean-Laurent, Lévy, Romain, and Bustamante, Jacinta

Subjects
*IDIOPATHIC thrombocytopenic purpura, *IMMUNODEFICIENCY, *HERPES zoster
Abstract

Patients with classic CGD frequently suffer from recurrent, invasive bacterial and fungal infections, whereas patients with autosomal recessive (AR) p40 SP I phox i sp deficiency have a higher frequency of hyperinflammation and peripheral infections, resembling a milder, variant, atypical form of CGD [[1]-[3]]. The patient presented with herpes zoster at the age of 8 years, which has not been reported previously in patients with p40 SP I phox i sp deficiency and very rarely in patients with other genetic etiologies conferring CGD. Patients with classic CGD have been reported to have a low frequency of memory B cells, whereas this patient with atypical CGD displayed no changes in B-cell phenotype during follow-up. [Extracted from the article]

Published
2023
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5. Pulmonary Alveolar Proteinosis and Multiple Infectious Diseases in a Child with Autosomal Recessive Complete IRF8 Deficiency

Author

Rosain, Jérémie, primary, Bernasconi, Andrea, additional, Prieto, Emma, additional, Caputi, Lucia, additional, Le Voyer, Tom, additional, Buda, Guadalupe, additional, Marti, Marcelo, additional, Bohlen, Jonathan, additional, Neehus, Anna-Lena, additional, Castaños, Claudio, additional, Gallagher, Rosario, additional, Dorgham, Karim, additional, Oleastro, Matias, additional, Perez, Laura, additional, Danielian, Silvia, additional, Dipierri, Jose Edgardo, additional, Casanova, Jean-Laurent, additional, Bustamante, Jacinta, additional, and Villa, Mariana, additional

Published
2022
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6. Fulminant Viral Hepatitis in Two Siblings with Inherited IL-10RB Deficiency.

Author

Korol, Cecilia B., Belkaya, Serkan, Alsohime, Fahad, Lorenzo, Lazaro, Boisson-Dupuis, Stéphanie, Brancale, Joseph, Neehus, Anna-Lena, Vilarinho, Silvia, Zobaida, Alsum, Halwani, Rabih, Al-Muhsen, Saleh, Casanova, Jean-Laurent, and Jouanguy, Emmanuelle

Subjects
VIRAL hepatitis, INFLAMMATORY bowel diseases, HEPATITIS A virus, HEPATITIS viruses, SIBLINGS
Abstract

Fulminant viral hepatitis (FVH) caused by hepatitis A virus (HAV) is a life-threatening disease that typically strikes otherwise healthy individuals. The only known genetic etiology of FVH is inherited IL-18BP deficiency, which unleashes IL-18-dependent lymphocyte cytotoxicity and IFN-γ production. We studied two siblings who died from a combination of early-onset inflammatory bowel disease (EOIBD) and FVH due to HAV. The sibling tested was homozygous for the W100G variant of IL10RB previously described in an unrelated patient with EOIBD. We show here that the out-of-frame IL10RB variants seen in other EOIBD patients disrupt cellular responses to IL-10, IL-22, IL-26, and IFN-λs in overexpression conditions and in homozygous cells. By contrast, the impact of in-frame disease-causing variants varies between cases. When overexpressed, the W100G variant impairs cellular responses to IL-10, but not to IL-22, IL-26, or IFN-λ1, whereas cells homozygous for W100G do not respond to IL-10, IL-22, IL-26, or IFN-λ1. As IL-10 is a potent antagonist of IFN-γ in phagocytes, these findings suggest that the molecular basis of FVH in patients with IL-18BP or IL-10RB deficiency may involve excessive IFN-γ activity during HAV infections of the liver. Inherited IL-10RB deficiency, and possibly inherited IL-10 and IL-10RA deficiencies, confer a predisposition to FVH, and patients with these deficiencies should be vaccinated against HAV and other liver-tropic viruses. [ABSTRACT FROM AUTHOR]

Published
2023
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7. Inherited GATA2 Deficiency Is Dominant by Haploinsufficiency and Displays Incomplete Clinical Penetrance

Author

Oleaga-Quintas, Carmen, primary, de Oliveira-Júnior, Edgar Borges, additional, Rosain, Jérémie, additional, Rapaport, Franck, additional, Deswarte, Caroline, additional, Guérin, Antoine, additional, Sajjath, Sairaj Munavar, additional, Zhou, Yu Jerry, additional, Marot, Stéphane, additional, Lozano, Claire, additional, Branco, Lidia, additional, Fernández-Hidalgo, Nuria, additional, Lew, Dukhee Betty, additional, Brunel, Anne-Sophie, additional, Thomas, Caroline, additional, Launay, Elise, additional, Arias, Andrés Augusto, additional, Cuffel, Alexis, additional, Monjo, Vanesa Cunill, additional, Neehus, Anna-Lena, additional, Marques, Laura, additional, Roynard, Manon, additional, Moncada-Vélez, Marcela, additional, Gerçeker, Bengü, additional, Colobran, Roger, additional, Vigué, Marie-Gabrielle, additional, Lopez-Herrera, Gabriela, additional, Berron-Ruiz, Laura, additional, Méndez, Nora Hilda Segura, additional, O’Farrill Romanillos, Patricia, additional, Le Voyer, Tom, additional, Puel, Anne, additional, Bellanné-Chantelot, Christine, additional, Ramirez, Kacy A., additional, Lorenzo-Diaz, Lazaro, additional, Alejo, Noé Ramirez, additional, de Diego, Rebeca Pérez, additional, Condino-Neto, Antonio, additional, Mellouli, Fethi, additional, Rodriguez-Gallego, Carlos, additional, Witte, Torsten, additional, Restrepo, José Franco, additional, Jobim, Mariana, additional, Boisson-Dupuis, Stéphanie, additional, Jeziorski, Eric, additional, Fieschi, Claire, additional, Vogt, Guillaume, additional, Donadieu, Jean, additional, Pasquet, Marlène, additional, Vasconcelos, Julia, additional, Ardeniz, Fatma Omur, additional, Martínez-Gallo, Mónica, additional, Campos, Regis A., additional, Jobim, Luiz Fernando, additional, Martínez-Barricarte, Rubén, additional, Liu, Kang, additional, Cobat, Aurélie, additional, Abel, Laurent, additional, Casanova, Jean-Laurent, additional, and Bustamante, Jacinta, additional

Published
2021
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