Rituximab-induced hypogammaglobulinemia in childhood nephrotic syndrome: a systematic review and meta-analysis.

A patient with WHIM syndrome presenting relatively low TREC, KREC, and housekeeping gene levels by newborn screening -a case report and the literature review.

Novel indications for hematopoietic stem cell transplantation in inborn errors of immunity.

First 2-year experience of nationwide newborn screening for severe forms of T and B cell immunodeficiency: 2.3 million newborns analyzed using TREC and KREC in Russia.

Good's Syndrome Mirrors a Combined Immunodeficiency with Anti-Cytokine Antibodies in the Total Absence of B Cells.

Translational immunothrombosis in autoimmune Heparin-Induced thrombocytopenia: targeting the FcγRIIa-Syk-BTK and complement pathways.

Immuno-deficient features of thymoma-associated myasthenia gravis patients with hypogammaglobulinemia: A condition comparable to Good's syndrome.

COVID-19 Outcomes and Risk Factors for Hospitalization in Adult Patients With Primary Immunodeficiency.

Immunoglobulin disorders in pediatric chronic rhinosinusitis.

A pharmacovigilance study of Bruton's tyrosine kinase inhibitors: a multidimensional analysis based on FAERS and VigiBase.

Good's Syndrome-A Rare Cause of Acquired Adult Immunodeficiency: A Case Report.

Case Report: A simple case of drug-induced secondary antibody deficiency or a rare primary immune deficiency?

Sclerosing Cholangitis and Multiple Liver Abscesses in a Patient with Thymoma, Myasthenia Gravis, and Immune Deficiency (Good's Syndrome).

Sustained remission with PD-1 and BTK inhibitors maintenance after chimeric antigen receptor T-cell therapy in CNS lymphoma.

Five Years of Combined Newborn Screening Quantifying TREC and KREC in Switzerland.

BTK Inhibitor Synergizes With CD19-Targeted Chimeric Antigen Receptor-T Cells in Patients With Relapsed or Refractory B-Cell Lymphoma: An Open-Label Pragmatic Clinical Trial.

Novel SYK Variant Causes Enhanced SYK Autophosphorylation and PI3K Activation in an Antibody-Deficient Patient.

Early-onset neutropenia and mixed phenotype in ADA2 deficiency: diagnostic and therapeutic challenges.

[Primary immunodeficiency with hypogammaglobulinemia and minimum midline defect].

[Ormond syndrome: a rare diagnosis in a patient with hypogammaglobulinemia secondary to rituximab].

Thrombocytopenia in patients with inborn errors of immunity.

The prevalence of allergic manifestations in inborn errors of immunity: a retrospective cohort study.

Early-onset vasculitis: a toddler with ADA2 deficiency.

Membranous nephropathy preceding Bing-Neel syndrome: successful treatment with tirabrutinib.

Is it time for the A/I (allergist/immunologist) to embrace AI (artificial intelligence) in diagnosis and treatment of the inborn errors of immunity?

Case Report: A novel CXCR4 variant (p.S341Y) in a family with a pathogenic NFKB1 variant and variable clinical manifestations.

Interstitial lung disease associated with immunodeficiency with hypogammaglobulinemia: Five case reports.

Pediatric and Adult Inborn Errors of Immunity and COVID-19: A Comparative Study.

Impairment of Collagen-Induced Thrombus Formation in Microfluidic Assay Correlates with Bleeding Complications Better Than Cytofluorometric Parameters.

Double Trouble: A Case of ARDS as a Consequence of Influenza and Plasma Donation Causing Severe Neutropenia and Hypogammaglobulinemia.

Shashi-Pena syndrome with late-onset specific hypogammaglobinaemia and autoimmune cytopenia.

FNIP1 Deficiency: Pathophysiology and Clinical Manifestations of a Rare Syndromic Primary Immunodeficiency.

Functional rescue of a fatal ERAD mutation via alternative splicing.

Paracentral acute middle maculopathy as a major clinical manifestation of adenosine deaminase-2 deficiency.

Adenosine and Adenosine Deaminase Contrary Manifestations in Immunity.

Uncovering the mechanism of Buyang Huanwu Decoction in regulating mitochondrial dysfunction to alleviate atherosclerosis: BTK, PREPL, and P2RX7 proteins play key roles.

Report of Consensus Panel 2 from the 12th International Workshop on the management of Bing-Neel syndrome in patients with Waldenstrom's Macroglobulinemia.

Clinicopathologic Features and the Spectrum of Myelokathexis in Warts, Hypogammaglobulinemia, Infections, Myelokathexis Syndrome.

Heterozygous deletion of 10q24.31-q24.33- a new syndrome associated with multiple congenital anomalies: case report and literature review.

Enhanced T-cell immunity and lower humoral responses following 5-dose SARS-CoV-2 vaccination in patients with inborn errors of immunity compared with healthy controls.

Gene therapy for inborn errors of immunity: Current clinical progress.

Remibrutinib in Chronic Spontaneous Urticaria.

Bruton's tyrosine kinase - A new target for immune mediated inflammatory diseases?

Zanubrutinib for the treatment of Bing-Neel syndrome.

A randomized, placebo-controlled trial of the BTK inhibitor zanubrutinib in hospitalized patients with COVID-19 respiratory distress: immune biomarker and clinical findings.

Infectious outcomes of a standardized subcutaneous immunoglobulin dose reduction strategy in primary immune deficiencies amid global shortage.

Advancing Newborn Screening in Washington State: A Novel Multiplexed LC-MS/MS Proteomic Assay for Wilson Disease and Inborn Errors of Immunity.

Nutritional status in pediatric patients with predominant antibody deficiency.

Infections, autoimmunity and immunodeficiencies are the leading etiologies of non-cystic fibrosis bronchiectasis in adults from the southwest of Colombia.

Functional validation of a novel STAT3 'variant of unknown significance' identifies a new case of STAT3 GOF syndrome and reveals broad immune cell defects.

Mutations disrupting the kinase domain of IKKα lead to immunodeficiency and immune dysregulation in humans.

Isolated loss of vaccine immunity in the protein losings syndrome in a patient with a reverse one and a half ventricle palliation ("failing Fontan-like physiology").

Differential regulatory effects of the N-terminal region in SYK-fusion kinases reveal unique activation-inducible nuclear translocation of ITK-SYK.

Sequelae of B-Cell Depleting Therapy: An Immunologist's Perspective.

A second look at secondary hypogammaglobulinemia.

Immunodeficiencies in Foals.

Persistent COVID-19 improved with immunoglobulin replacement therapy in Good's syndrome.

Nephrotic Syndrome and Recurrent Infection.

Reversible posterior leukoencephalopathy syndrome (RPLS) in a patient with chronic lymphocytic leukemia (CLL) treated with Acalabrutinib, a Bruton's tyrosine kinase (BTK) inhibitor: a case report.

A novel mutation in FNIP1 associated with a syndromic immunodeficiency and cardiomyopathy.

Comprehensive newborn screening for severe combined immunodeficiency, X-linked agammaglobulinemia, and spinal muscular atrophy: the Chinese experience.

Recovery from rituximab-associated persistent hypogammaglobulinaemia in children with nephrotic syndrome.

Rare primary vasculitis: update on multiple complex diseases and the new kids on the block.

A single center experience on PI3K/AKT/MTOR signaling defects: Towards pathogenicity assessment for four novel defects.

Biallelic PI4KA Mutations Disrupt B-Cell Metabolism and Cause B-Cell Lymphopenia and Hypogammaglobulinemia.

Pure Red Cell Aplasia in a Patient with Thymic Hyperplasia, Hypogammaglobulinemia and Adult T-cell Leukemia/Lymphoma.

Clinical spectrum of and outcomes for Indian children with deficiency of adenosine deaminase 2 (DADA2): a multicentric study.

Good syndrome combined with multiple microbial pulmonary infections: case report and review of the literature.

Targeting Bruton's tyrosine kinase (BTK) as a signaling pathway in immune-mediated diseases: from molecular mechanisms to leading treatments.

Three-year cardiovascular and non-cardiovascular adverse events in patients with chronic lymphocytic leukemia or small cell lymphocytic lymphoma treated with Bruton tyrosine kinase inhibitors acalabrutinib or ibrutinib: a real-world analysis.

Deficiency of Adenosine Deaminase 2.

A Novel Variant of the PIK3R1 Gene Mutation Associated With SHORT Syndrome and Agammaglobulinemia.

Omenn Syndrome can Occur during Enzyme Therapy for Adenosine Deaminase Deficiency.

Transient hypogammaglobulinemia of infancy and unclassified syndromic immunodeficiencies are highly common in oesophageal atresia patients.

A Clinical Neurological Approach to the Child With Adenosine Deaminase Deficiency.

Analysis of LRBA pathogenic variants and the association with functional protein domains and clinical presentation.

Clinical efficacy of SARS-CoV-2 Omicron-neutralizing antibodies in immunoglobulin preparations for the treatment of agammaglobulinemia in patients with primary antibody deficiency.

Antibody Response Before and After the Booster Dose of Inactivated Corona Vaccine in Antibody Deficient Patients.

Hypogammaglobulinaemia during rituximab treatment in multiple sclerosis: A Swedish cohort study.

A Nationwide Study of the Delayed Diagnosis and the Clinical Manifestations of Predominantly Antibody Deficiencies and CTLA4-Mediated Immune Dysregulation Syndrome in Greece.

Variable Syndromic Immunodeficiency in Patients with Biallelic PRIM1 Mutations.

Severe enterovirus infections in patients with immune-mediated inflammatory diseases receiving anti-CD20 monoclonal antibodies.

Case report: BTK inhibitors is effective in type II mixed cryoglobulinemia with wild-type MyD88.

Clinical and Immunologic Features of a Patient With Homozygous FNIP1 Variant.

Viral infections and inborn errors of immunity.

[COVID-19 in patients with Good syndrome: report of 4 cases and literature review].

Immunogenicity of COVID-19 booster vaccination in IEI patients and their one year clinical follow-up after start of the COVID-19 vaccination program.

Rituximab-induced hypogammaglobulinemia in nephrotic syndrome: what is the true burden?

Hypogammaglobulinemia in 2 children with Zhu-Tokita-Takenouchi-Kim syndrome.

Newborn Screening for Severe T and B Cell Lymphopenia Using TREC/KREC Detection: A Large-Scale Pilot Study of 202,908 Newborns.

Association between serum IgG concentrations and the incidence of infections in patients with chronic lymphocytic leukemia and secondary immunodeficiency under treatment with Privigen.

Inhibitors of Bruton's tyrosine kinase as emerging therapeutic strategy in autoimmune diseases.

CXCR4: from B-cell development to B cell-mediated diseases.

Safety and efficacy of biologic immunosuppressive treatment in juvenile idiopathic arthritis associated with inborn errors of immunity.

CXCR4 WHIM syndrome is a cancer predisposition condition for virus-induced malignancies.

[Incidence of hypogammaglobulinaemia in children with steroid-dependent/frequently relapsing nephrotic syndrome treated with rituximab and its association with severe infections].

Gene regulation in inborn errors of immunity: Implications for gene therapy design and efficacy.

X-Linked Lymphoproliferative Syndrome: A Spectrum of Clinical and Immunological Profile and Novel Pathogenic Variants from Chandigarh, India.

Good syndrome and cytomegalovirus retinitis: A literature review.

Unraveling the Natural History of Good's Syndrome: A Progressive Adult Combined Immunodeficiency.

In-depth blood immune profiling of Good syndrome patients.

Immune compromise in patients with Down syndrome. A case series.

Enhanced CD19 activity in B cells contributes to immunodeficiency in mice deficient in the ICF syndrome gene Zbtb24.

Hypogammaglobulinemia and immune dysregulation-not just 2 sides of a coin.

Cutaneous vasculitis in autoinflammatory diseases.

IRAK-4 inhibition: emavusertib for the treatment of lymphoid and myeloid malignancies.

Biallelic Cys141Tyr variant of SEL1L is associated with neurodevelopmental disorders, agammaglobulinemia, and premature death.

[Management of adverse effects in CAR T-cell therapy].

Antibody Deficiency in Patients with Biallelic KARS1 Mutations.

A novel mutation in DNMT3B gene causing ICF1 syndrome in an infant with refractory thrombocytopenia.

Vasculitis associated with adenosine deaminase 2 deficiency: at the crossroads between Behçet's disease and autoinflammation. A viewpoint.

Who's your data? Primary immune deficiency differential diagnosis prediction via machine learning and data mining of the USIDNET registry.

A new CECR1 mutation associated with severe hematological involvement in ADA2 deficiency.

Recurrent flare-associated urticaria in adenosine deaminase type 2 deficiency.

[Early detection of WHIM symdrome. A case report].

A Narrative Review of the Neurological Manifestations of Human Adenosine Deaminase 2 Deficiency.

Monitoring, prophylaxis, and treatment of infections in patients with MM receiving bispecific antibody therapy: consensus recommendations from an expert panel.

Safety and efficacy of elapegademase in patients with adenosine deaminase deficiency: A multicenter, open-label, single-arm, phase 3, and postmarketing clinical study.

Inborn Errors of Immunity-the Sri Lankan Experience 2010-2022.

Kawasaki Disease and Inborn Errors of Immunity: Exploring the Link and Implications.

Hemophagocytic inflammatory syndrome in ADA-SCID: report of two cases and literature review.

Recommendations for Management of Secondary Antibody Deficiency in Multiple Myeloma.

Deficient anterior pituitary with common variable immune deficiency (DAVID syndrome): a new case and literature reports.

Brainstem Infarction in Immunodeficiency Identified as Adenosine Deaminase 2 Deficiency: Case Report.

[Good syndrome: a rare, unusual immunodeficiency condition].

A novel variant in ALG1 gene associated with congenital disorder of glycosylation: A case report and short literature review.

Persistent and fatal severe acute respiratory syndrome coronavirus 2 infection in a patient with severe hypogammaglobulinemia: a case report.

Impaired Response to Polysaccharide Vaccine in Selective IgE Deficiency.

Quality of Life Evaluation in Saudi Arabian Pediatric Patients with Primary Immunodeficiency Diseases Receiving 20% Subcutaneous IgG Infusions at Home.

Severe CD8+ T Lymphopenia in WHIM Syndrome Caused by Selective Sequestration in Primary Immune Organs.

COVID-19 relapse associated with SARS-CoV-2 evasion from CD4+ T-cell recognition in an agammaglobulinemia patient.

Association Between Bruton's Tyrosine Kinase Gene Overexpression and Risk of Lymphoma in Primary Sjögren's Syndrome.

SARS-CoV-2 spike antibody concentration in gamma globulin products from high-prevalence COVID-19 countries are transmitted to X-linked agammaglobulinemia patients.

[Hypogammaglobulinemia and immunodeficiency in multiple myeloma and chronic lymphoid leukemia].

Rituximab-associated hypogammaglobulinemia in children with idiopathic nephrotic syndrome: results of an ESPN survey.

Autoimmunity and immunodeficiency associated with monoallelic LIG4 mutations via haploinsufficiency.

Co-Occurring X-Linked Agammaglobulinemia and X-Linked Chronic Granulomatous Disease: Two Isolated Pathogenic Variants in One Patient.

Association between hypogammaglobulinaemia and severe infections during induction therapy in ANCA-associated vasculitis: from J-CANVAS study.

Haematopoietic Stem Cell Transplantation (HSCT) for Primary Immune System Disorders in Children: A Single Centre Experience.

COVID-19 disease frequency, risk factors, and re-infection rates in patients with autoimmune rheumatic disease receiving rituximab.

TNF-Blockade for Primary Stroke Prevention in Adenosine Deaminase 2 Deficiency: A Case Series.

Safety and Efficacy of BCMA CAR-T Cell Therapy in Older Patients With Multiple Myeloma.

Bruton's tyrosine kinase inhibition attenuates disease progression by reducing renal immune cell invasion in mice with hemolytic-uremic syndrome.

Serum Protein Electrophoretic in Children.

Human genetic and immunological determinants of SARS-CoV-2 and Epstein-Barr virus diseases in childhood: Insightful contrasts.

Bruton's Tyrosine Kinase Inhibitors (BTKIs): Review of Preclinical Studies and Evaluation of Clinical Trials.

GOOD SYNDROME: CYTOMEGALOVIRUS RETINITIS CASE CHALLENGE.

[Management of hypogammaglobulinemia].

Ischaemic cerebral small vessel disease caused by adenosine deaminase 2 deficiency syndrome.

National experience with adenosine deaminase deficiency related SCID in Polish children.

The Importance of Endoscopy with Biopsy: Real-World Evidence of Gastrointestinal Involvement in Primary Immunodeficiency in Two Main Northern Italian Centres.

A novel frameshift variant in the ADA2 gene of a patient with a neurological phenotype: a case report.

Chronic graft vs. host disease and hypogammaglobulinemia predict a lower immunological response to the BNT162b2 mRNA COVID-19 vaccine after allogeneic hematopoietic stem cell transplantation.

SARS-COV-2 infections in inborn errors of immunity: A single center study.

Use of Rituximab in Childhood Idiopathic Nephrotic Syndrome.

[Common variable immune deficiency complicated by amyloidosis: a case report].

Comparison of pulmonary lesions using lung ultrasound and high-resolution computed tomography in adult patients with primary humoral immunodeficiencies.

Long-term follow up of families with pathogenic NFKB1 variants reveals incomplete penetrance and frequent inflammatory sequelae.

Ibrutinib: Pediatric First Approval.

COVID-19 in unvaccinated patients with inborn errors of immunity-polish experience.

Immunogenicity and Safety of the Spikevax® (Moderna) mRNA SARS-CoV-2 Vaccine in Patients with Primary Humoral Immunodeficiency.

Reactogenicity and immunogenicity of the second COVID-19 vaccination in patients with inborn errors of immunity or mannan-binding lectin deficiency.

Newborn screening for severe combined immunodeficiency: The results of the first pilot TREC and KREC study in Ukraine with involving of 10,350 neonates.

Case Report: Susceptibility to viral infections and secondary hemophagocytic lymphohistiocytosis responsive to intravenous immunoglobulin as primary manifestations of adenosine deaminase 2 deficiency.

Real-world results with IgPro20 for hypo- or agammaglobulinemia in Japan.

Break down the barriers of auto-inflammation: How to deal with a monogenic auto-inflammatory disease and immuno-haematological features in 2022?

Long-term prognosis of rituximab-induced hypogammaglobulinemia in children with complicated steroid-dependent nephrotic syndrome: impact of multiple rituximab courses.

A Rare Autoinflammatory Disorder in a Pediatric Patient with Favorable Response to Etanercept: Sideroblastic Anemia with B Cell Immunodeficiency, Periodic Fevers, and Developmental Delay Syndrome.

Genotype-phenotype correlations in WHIM syndrome: a systematic characterization of CXCR4WHIM variants.

Various phenotypes of LRBA gene with compound heterozygous variation: A case series report of pediatric cytopenia patients.

Sequential rituximab therapy sustains remission of nephrotic syndrome but carries high risk of adverse effects.

Bruton tyrosine kinase (BTK) may be a potential therapeutic target for interstitial cystitis/bladder pain syndrome.

Identification and analysis of deletion breakpoints in four Mohr-Tranebjærg syndrome (MTS) patients.

Immunodeficiency syndromes differentially impact the functional profile of SARS-CoV-2-specific T cells elicited by mRNA vaccination.

Disease Progression of WHIM Syndrome in an International Cohort of 66 Pediatric and Adult Patients.

Biallelic PAX5 mutations cause hypogammaglobulinemia, sensorimotor deficits, and autism spectrum disorder.

Hypogammaglobulinemia is associated with reduced antibody response after anti-SARS-CoV-2 vaccination in MS patients treated with antiCD20 therapies.

[Antibodydeficiencies Epidemiology, Clinical manifestation, Diagnostics and Therapy].

Clinical and Hematologic Effects of Endotoxin in Warts, Hypogammaglobulinemia, Infections, and Myelokathexis Syndrome Model Mice.

Targeted Gene Sanger Sequencing Should Remain the First-Tier Genetic Test for Children Suspected to Have the Five Common X-Linked Inborn Errors of Immunity.

Chronic Enteroviral Meningoencephalitis in a Patient with Good's Syndrome Treated with Pocapavir.

Algerian Registry for Inborn Errors of Immunity in Children: Report of 887 Children (1985-2021).

Common Variable Immunodeficiency and Neurodevelopmental Delay Due to a 13Mb Deletion on Chromosome 4 Including the NFKB1 Gene: A Case Report.

Risk Factors of Pneumonia in Primary Antibody Deficiency Patients Receiving Immunoglobulin Therapy: Data from the US Immunodeficiency Network (USIDNET).

The association between hypogammaglobulinemia severity and infection risk in rituximab-treated patients with childhood-onset idiopathic nephrotic syndrome.

Hypogammaglobulinaemia and B cell lymphopaenia in Barth syndrome.

Utility of targeted next generation sequencing for inborn errors of immunity at a tertiary care centre in North India.

T Cell Abnormalities in X-Linked Agammaglobulinaemia: an Updated Review.

Diagnosis of immune thrombocytopenia, including secondary forms, and selection of second-line treatment.

Long-term follow-up and future direction on the management of chronic lymphocytic leukemia/small lymphocytic leukemia.

Deficiency of Human Adenosine Deaminase Type 2 - A Diagnostic Conundrum for the Hematologist.

Variants Disrupting CD40L Transmembrane Domain and Atypical X-Linked Hyper-IgM Syndrome: A Case Report With Leishmaniasis and Review of the Literature.

Decreased BAFF Receptor Expression and Unaltered B Cell Receptor Signaling in Circulating B Cells from Primary Sjögren's Syndrome Patients at Diagnosis.

Fatal SARS in X-Linked Lymphoproliferative Disease Type 1: A Case Report.

Lipopolysaccharide Responsive Beige-like Anchor Protein Deficiency in a Patient with Autoimmune Lymphoproliferative Syndrome-like Disease Phenotype: A Case Report and Literature Review.

A Sherlock Approach to a Kindred With a Variable Immunohematologic Phenotype.

Immunogenicity of the mRNA-1273 COVID-19 vaccine in adult patients with inborn errors of immunity.

Specific T-cell responses for guiding treatment with convalescent plasma in severe COVID-19 and humoral immunodeficiency: a case report.

Keratoconjunctivitis as a Single Entity in X-linked Agammaglobulinemia?