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Infections
Published in Evelyne Jacqz-Aigrain, Imti Choonara, Paediatric Clinical Pharmacology, 2021
Evelyne Jacqz-Aigrain, Imti Choonara
Indications for the use of immunoglobulins have decreased following improved immunisation programmes. There are, however, occasions when immunoglobulins are recommended and these include the following: The prevention of measles in immunocompromised children following exposure to measles.Contact with hepatitis A in immunocompromised children.Replacement therapy for patients with agammaglobulinaemia or hypogammaglobulinaemia. Specific immunoglobulins are indicated in the following situations: For infants born to mothers who have become infected with hepatitis B during the pregnancy or who are high risk carriers.Varicella-zoster immunoglobulin following contact with chickenpox in an immunocompromised child or a neonate.
Measles and its neurological complications
Published in Avindra Nath, Joseph R. Berger, Clinical Neurovirology, 2020
Benedikt Weissbrich, Jürgen Schneider-Schaulies
Although antibody titers normally rise in the exanthematous stage of the illness, they are not thought to be the major factor in promoting recovery. MV-infected cells are lysed inefficiently by the classic pathway of the complement activation, although more so by the alternative pathway. Furthermore, patients with agammaglobulinemia handle MV infection normally and recover. However, those with T cell deficiencies usually do not develop the rash and can be severely ill. Evidently, measles infection in the immunocompetent host triggers an efficient virus-specific immune response that leads to the clearance of the virus from the peripheral blood and the establishment of usually lifelong immunity against reinfection. Paradoxically, at the same time a general suppression of responsiveness to other pathogens is established which is the major reason for the high morbidity and mortality rate associated worldwide with acute measles.
Inflammation and immunology
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
Christopher Bellamy, Stephen J. Jenkins, Henry J. McSorley, David A. Dorward, Timothy J. Kendall
These are rare, often life-threatening diseases that nevertheless have contributed greatly to our understanding of the immune system. X-linked agammaglobulinaemia is the most common of these disorders and is caused by a failure of cell signalling and maturation of B cells, with failure of the light chain gene rearrangement which normally allows the formation of Ig molecules. Circulating B cells are markedly reduced or absent and there is a failure to make antibody. Once maternal antibody has declined the children become susceptible to recurrent episodes of bacterial infection.
Selective inhibition of thromboinflammation in COVID-19 by Btk inhibitors
Published in Platelets, 2020
Wolfgang Siess, Philipp Von Hundelshausen, Reinhard Lorenz
Despite the attractive profile of Btk-inhibitor effects on platelets and myeloid cells that could counteract detrimental derangements in severe COVID-19, any future clinical study of Btk-inhibitors in COVID-19 must take into account that Btk plays a central role in B-cell function, proliferation, and replication. The obvious evidence is the clinical picture of X-linked agammaglobulinemia (XLA) caused by genetic Btk-deficiency. Taking into account the dysfunctional immunity associated with chronic lymphocytic leukemia (CLL) and other B-cell dyscrasias, in a small case series of CLL-patients on ibrutinib SARS-Cov2 infection ran an unexpected mild course [28]. Apparently, antiviral antibody generation by B-cells of COVID-19 patients was not critically compromised by CLL-doses of ibrutinib or other mechanisms of immunity were sufficient in these patients. Ibrutinib had previously been shown to partially reconstitute humoral immunity and reduce infections in CLL patients [29].
Global systematic review of primary immunodeficiency registries
Published in Expert Review of Clinical Immunology, 2020
Hassan Abolhassani, Gholamreza Azizi, Laleh Sharifi, Reza Yazdani, Monireh Mohsenzadegan, Samaneh Delavari, Mahsa Sohani, Paniz Shirmast, Zahra Chavoshzadeh, Seyed Alireza Mahdaviani, Arash Kalantari, Marzieh Tavakol, Farahzad Jabbari-Azad, Hamid Ahanchian, Tooba Momen, Roya Sherkat, Mahnaz Sadeghi-Shabestari, Soheila Aleyasin, Hossein Esmaeilzadeh, Waleed Al-Herz, Ahmed Aziz Bousfiha, Antonio Condino-Neto, Mikko Seppänen, Kathleen E. Sullivan, Lennart Hammarström, Vicki Modell, Fred Modell, Jessica Quinn, Jordan S. Orange, Asghar Aghamohammadi
The PID registry next to emerge in Africa was from Morocco; Bousfiha et al. reported 424 PID patients registered by 2014. The reported prevalence of PID was 0.81 in 100000 inhabitants. The most prevalent disorders were reported as follows: well-defined immunodeficiency syndromes (27.4%), antibody deficiencies (22.7%) and combined immunodeficiencies (20.6%). Furthermore, agammaglobulinemia accounted for the majority of antibody deficiencies patients (54.7%) and MHC class II deficiency was reported in 41.2% of combined immunodeficiencies. Parental consanguinity was identified in 43.2% of cases and 19.1% of patients had a positive family history [56]. Three recent additions to the Maghreb registry have released their preliminary reports on the PID patients in Algeria, Libya and Sudan with 600, 106 and 72 patients, respectively [49].
Disturbed Transcription of TLRs’ Negative Regulators and Cytokines Secretion among TLR4- and 9-Activated PBMCs of Agammaglobulinemic Patients
Published in Immunological Investigations, 2019
Roozbeh Sanaei, Nima Rezaei, Asghar Aghamohammadi, Ali-Akbar Delbandi, Parsova Tavasolian, Nader Tajik
There have been efforts to clarify TLRs partaking in the pathogenesis of antibody deficiencies. Inconsistent observations in this context (Gonzalez-Serrano et al., 2012; Hasan et al., 2008; Horwood et al., 2003, 2006; Lougaris et al., 2014; Mangla et al., 2004; Marron et al., 2012; Schmidt et al., 2006; Teocchi et al., 2015; Uckun and Qazi, 2010) made us to investigate a novel approach about TLRs in probable XLA patients. Adjustment of TLR responses through a regulatory network may be of interest as well as the fact that regulatory molecules could simultaneously play fundamental roles (Vidya et al., 2018) in bringing abovementioned difficulties in patients. Therefore, to achieve these two purposes, transcription of TLRs’ negative regulators (including SOCS1, TNFAIP3, IRAK-M, and RNF216) and TLRs’ ability to induce these elements besides the production of inflammatory cytokines was investigated. These would suggest new insights into their part in the pathogenesis of agammaglobulinemia and relevant complications in affected individuals.