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Lineage-Specific Chimerism and Outcome After Hematopoietic Stem Cell Transplantation for DOCK8 Deficiency
We report on nine patients, four of whom with mixed chimerism, with a median follow-up of 78  months after transplantation. Overall, we report successful transplantation with improvement of susceptibility to infections and allergies, and resolution of eczema in all patients. Immunological outcome in patients with mixed chimerism suggests a selective advantage for wild-type donor T-cells bu t lower donor B-cell chimerism possibly results in a tendency to hypogammaglobulinemia. No increased infectious and allergic complications were associated with mixed chimerism. Aware of the relatively small cohort size, we could not dem...
Source: Journal of Clinical Immunology - June 2, 2021 Category: Allergy & Immunology Source Type: research

M247 early detection of a rare disorder: wiskott-aldrich syndrome
Wiskott-Aldrich Syndrome is a X-linked primary immunodeficiency characterized by microthrombocytopenia, eczema, and recurrent infections. It is important to maintain a high suspicion for WAS in a patient who presents with microthrombocytopenia, as Hematopoietic Stem Cell Transplantation (HSCT) is the only curative treatment and early diagnosis is key.
Source: Annals of Allergy, Asthma and Immunology - November 1, 2020 Category: Allergy & Immunology Authors: C. Basta, J. Krajewski, T. Weinberger Source Type: research

Qingpeng Ointment Ameliorates Inflammatory Responses and Dysregulation of Itch-Related Molecules for Its Antipruritic Effects in Experimental Allergic Contact Dermatitis
Conclusion Via a HPLC system and a BATMAN-TCM platform, for the first time we performed detailed HPLC fingerprint analysis and network pharmacology studies of QP ointment. By in-depth animal and molecular analysis, we identified a number of chronic dermatitis-associated Th1/2 cytokines, itch mediators, and cellular signaling pathways in the skin, DRG and serum for QP to treat the inflammation and chronic itch associated with ACD. Our data clearly suggested, upon stimulation, cytokines and itch-related mediators are released from the skin and neuronal tissues, and QP could effectively work on both the skin and sensory neur...
Source: Frontiers in Pharmacology - April 8, 2019 Category: Drugs & Pharmacology Source Type: research

Hematopoietic stem cell transplantation as treatment for patients with DOCK8 deficiency
ConclusionHSCT is curative in most DOCK8 deficient patients, confirming this approach as the treatment of choice. HSCT using a reduced toxicity regimen may offer the best chance for survival.
Source: The Journal of Allergy and Clinical Immunology: In Practice - November 3, 2018 Category: Allergy & Immunology Source Type: research

Hematopoietic stem cell transplantation corrects WIP deficiency
Wiskott-Aldrich syndrome (WAS) is an X-linked primary immunodeficiency disease characterized by immune deficiency, thrombocytopenia, and eczema. The gene responsible for X-linked WAS encodes Wiskott-Aldrich syndrome protein (WASP).1 WASP is expressed in hematopoietic cells and has multiple functions in the immune system. WASP-interacting protein (WIP) forms a constitutive complex with WASP. A  major function of WIP is to stabilize WASP and prevent its degradation.2 A novel primary immunodeficiency had been reported in a single patient with a nonsense mutation in WIPF1, the gene that encodes WIP, producing a protein that ...
Source: Journal of Allergy and Clinical Immunology - October 11, 2016 Category: Allergy & Immunology Authors: Hamoud Al-Mousa, Abbas Hawwari, Abdulaziz Al-Ghonaium, Bandar Al-Saud, Hasan Al-Dhekri, Saleh Al-Muhsen, Sahar Elshorbagi, Majed Dasouki, Lina El-Baik, Amal Alseraihy, Mouhab Ayas, Rand Arnaout Tags: Letter to the Editor Source Type: research

XXIV World Allergy Congress 2015
Table of Contents A1 Pirfenidone inhibits TGF-b1-induced extracellular matrix production in nasal polyp-derived fibroblasts Jae-Min Shin, Heung-Man Lee, Il-Ho Park A2 The efficacy of a 2-week course of oral steroid in the treatment of chronic spontaneous urticaria refractory to antihistamines Hyun-Sun Yoon, Gyeong Yul Park A3 The altered distribution of follicular t helper cells may predict a more pronounced clinical course of primary sjögren’s syndrome Margit Zeher A4 Betamethasone suppresses Th2 cell development induced by langerhans cell like dendr...
Source: World Allergy Organization Journal - April 18, 2016 Category: Allergy & Immunology Source Type: research

B-cell reconstitution after lentiviral vector–mediated gene therapy in patients with Wiskott-Aldrich syndrome
Wiskott-Aldrich syndrome (WAS) is a severe X-linked immunodeficiency characterized by microthrombocytopenia, eczema, recurrent infections, and susceptibility to autoimmunity and lymphomas. Hematopoietic stem cell transplantation is the treatment of choice; however, administration of WAS gene–corrected autologous hematopoietic stem cells has been demonstrated as a feasible alternative therapeutic approach.
Source: Journal of Allergy and Clinical Immunology - March 17, 2015 Category: Allergy & Immunology Authors: Maria Carmina Castiello, Samantha Scaramuzza, Francesca Pala, Francesca Ferrua, Paolo Uva, Immacolata Brigida, Lucia Sereni, Mirjam van der Burg, Giorgio Ottaviano, Michael H. Albert, Maria Grazia Roncarolo, Luigi Naldini, Alessandro Aiuti, Anna Villa, Ma Source Type: research

Haploidentical T-cell alpha beta receptor and CD19–depleted stem cell transplant for Wiskott-Aldrich syndrome
Hematopoietic stem cell transplant (HSCT) is curative for patients with Wiskott-Aldrich syndrome (WAS), a severe X-linked disorder characterized by microthrombocytopenia, eczema, and immunodeficiency. Nontransplanted patients often develop autoimmune and inflammatorycomplications, and there is an increased risk of hematological malignancies. Median life expectancy without HSCT is 15 years.1 HSCT using a HLA-identical sibling is highly successful, with above 90% 5-year survival. Results of matched unrelated donor HSCT for treatment of WAS have improved over time, with recent studies showing above 80% survival.
Source: Journal of Allergy and Clinical Immunology - June 27, 2014 Category: Allergy & Immunology Authors: Gaurav Kharya, Zohreh Nademi, T. Ronan Leahy, Janice Dunn, Dawn Barge, Ansgar Schulz, Andrew Cant, Andrew Gennery, Mary Slatter Tags: Letter to the Editor Source Type: research

Cutaneous manifestations in patients with Wiskott-Aldrich syndrome submitted to haematopoietic stem cell transplantation
Conclusions Cutaneous manifestations in patients with WAS are frequent, especially those similar to the eczema found in AD.
Source: Archives of Disease in Childhood - March 11, 2013 Category: Pediatrics Authors: Loyola Presa, J. G., de Carvalho, V. O., Morrisey, L. R., Bonfim, C. M., Abagge, K. T., Vasselai, A., Marinoni, L. P. Tags: Immunology (including allergy), Dermatology, Artificial and donated transplantation Original article Source Type: research

Combined DOCK8 and CLEC7A mutations causing immunodeficiency in 3 brothers with diarrhea, eczema, and infections
We report 4 brothers from a consanguineous Italian family with an immunodeficiency syndrome characterized by severe eczema, milk and egg allergies, recurrent infections, intractable diarrhea, failure to thrive, and, in 2 of the 3 surviving brothers, lymphoma. One of the brothers (patient II-2) died at age 12 months of cardiovascular failure caused by intractable diarrhea, and the other 3 were evaluated as part of this study ( and , A). The proband (patient II-1) presented shortly after birth with severe eczema and intractable diarrhea without gastrointestinal infection. In the first year of life, he had multiple allergies ...
Source: Journal of Allergy and Clinical Immunology - February 1, 2013 Category: Allergy & Immunology Authors: Darrell L. Dinwiddie, Stephen F. Kingsmore, Sonia Caracciolo, Giuseppe Rossi, Daniele Moratto, Cinzia Mazza, Cristiano Sabelli, Rosa Bacchetta, Laura Passerini, Chiara Magri, Callum J. Bell, Neil A. Miller, Shannon L. Hateley, Carol J. Saunders, Lu Zhang, Tags: Letters to the Editor Source Type: research