Clinical Communications
Liver disturbances in activated phosphoinositide 3-kinase δ syndrome

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  • Nodular regenerative hyperplasia in X-linked agammaglobulinemia: An underestimated and severe complication

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    While the pathophysiology of NRH remains elusive, its increased frequency in patients with PID/IEIs provides hints about mechanisms involved or redundant. The conditions in which NRH is encountered suggest that the integrity of several pathways is relevant to prevent its evolution, including the nicotinamide adenine dinucleotide phosphate–oxidase pathway (chronic granulomatous disease),38,39,41 the PI3K pathway (activated phosphoinositide 3–kinase δ syndrome),42 specific antibody responses (CVID),12 DNA repair mechanisms (ataxia telangiectasia),40 or BTK pathway (XLA). On the other hand, whereas infectious agents, particularly viruses, have been only sporadically found and rarely associated with NRH pathophysiology,10,19,20,24 this hypothesis remains valid in immunocompromised patients.

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    Skin testing (Table LVIII) is usually useful in identifying patients with an IgE-mediated hypersensitivity reaction, and desensitization (Table LIX) should be considered the method of choice to reexpose those patients to tocilizumab (Figure 6).470,474,502,503 Omalizumab is a humanized mAb that binds free IgE antibodies.504 It is administered subcutaneously and is used in the treatment of moderate to severe allergic asthma and chronic idiopathic urticaria.504

  • PI3K pathway defects leading to immunodeficiency and immune dysregulation

    2019, Journal of Allergy and Clinical Immunology
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    For instance, lymphoproliferative disease has been proposed to contribute to the development of bronchiectasis.61 In another example, the high prevalence of NRH found in a small sample of patients with APDS who were submitted to liver biopsy was surprising, and this finding could influence the choice of conditioning regimen for HSCT.42 The molecular characterization of these defects had a critical effect on the management of patients with APDS because targeted therapies were introduced.

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This work was supported by the intramural research programs of the National Institute of Diabetes and Digestive and Kidney Diseases, National Institute of Allergy and Infectious Diseases, and National Cancer Institute, National Institutes of Health.

Conflicts of interest: The authors declare that they have no relevant conflicts of interest.

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