Keck School Faculty

Joseph August Church, MD
Joseph August Church, MD
Professor of Clinical Pediatrics
Pediatrics
CHL Mail Stop 75 Off Campus Los Angeles

Dominant activating RAC2 mutation with lymphopenia, immunodeficiency and cytoskeletal defects Blood. 2019 Feb 05. . View in PubMed

Newborn Screening for Severe Combined Immunodeficiency and T-cell Lymphopenia in California, 2010-2017 Pediatrics. 2019 Jan 25. . View in PubMed

White matter microstructure among perinatally HIV-infected youth: a diffusion tensor imaging study J Neurovirol. 2019 Jan 04. . View in PubMed

Chronic rhinosinusitis with severe nasal polyposis in hyper-IgE syndrome Ann Allergy Asthma Immunol. 2018 Aug 03. . View in PubMed

Activating de novo mutations in NFE2L2 encoding NRF2 cause a multisystem disorder Nat Commun. 2017 10 10; 8(1):818. . View in PubMed

Pharmacokinetics, Safety, and Tolerability of Subcutaneous Immune Globulin Injection (Human), 10 % Caprylate/Chromatography Purified (GAMUNEX®-C) in Pediatric Patients with Primary Immunodeficiency Disease J Clin Immunol. 2016 Aug; 36(6):600-9. . View in PubMed

Long-Term Tolerability, Safety, and Efficacy of Recombinant Human Hyaluronidase-Facilitated Subcutaneous Infusion of Human Immunoglobulin for Primary Immunodeficiency J Clin Immunol. 2016 Aug; 36(6):571-82. . View in PubMed

Supranormal thymic output up to 2 decades after HIV-1 infection AIDS. 2016 Mar 13; 30(5):701-11. . View in PubMed

Pharmacokinetics, Safety, and Tolerability of Subcutaneous Immune Globulin Injection (Human), 10 % Caprylate/Chromatography Purified (GAMUNEX®-C) in Pediatric Patients with Primary Immunodeficiency Disease J Clin Immunol. 2016 08; 36(6):600-9. . View in PubMed

Long-Term Tolerability, Safety, and Efficacy of Recombinant Human Hyaluronidase-Facilitated Subcutaneous Infusion of Human Immunoglobulin for Primary Immunodeficiency J Clin Immunol. 2016 08; 36(6):571-82. . View in PubMed

The Effect of Maternal Peripartum Anti-TNFa Use on Infant Immune Response Dig Dis Sci. 2016 06; 61(6):1622-7. . View in PubMed

Pilot Assessment of Brain Metabolism in Perinatally HIV-Infected Youths Using Accelerated 5D Echo Planar J-Resolved Spectroscopic Imaging PLoS One. 2016; 11(9):e0162810. . View in PubMed

Single amino acid charge switch defines clinically distinct proline-serine-threonine phosphatase-interacting protein 1 (PSTPIP1)-associated inflammatory diseases J Allergy Clin Immunol. 2015 Nov; 136(5):1337-45. . View in PubMed

Regions of homozygosity identified by oligonucleotide SNP arrays: evaluating the incidence and clinical utility Eur J Hum Genet. 2015 May; 23(5):663-71. . View in PubMed

A randomized, open-label study of the safety and efficacy of switching stavudine or zidovudine to tenofovir disoproxil fumarate in HIV-1-infected children with virologic suppression Pediatr Infect Dis J. 2015 Apr; 34(4):376-82. . View in PubMed

Clinical characterization of int22h1/int22h2-mediated Xq28 duplication/deletion: new cases and literature review BMC Med Genet. 2015 Mar 14; 16:12. . View in PubMed

Nijmegen breakage syndrome detected by newborn screening for T cell receptor excision circles (TRECs) J Clin Immunol. 2015 Feb; 35(2):227-33. . View in PubMed

Antibody levels to Bordetella pertussis and Neisseria meningitidis in immunodeficient patients receiving immunoglobulin replacement therapy J Clin Immunol. 2015 Feb; 35(2):213-7. . View in PubMed

Cell Death by Pyroptosis Drives CD4 T-Cell Depletion in HIV-1 Infection Pediatrics. 2014 Nov; 134 Suppl 3:S184. . View in PubMed

Vectored Immunoprophylaxis Protects Humanized Mice From Mucosal HIV Transmission Pediatrics. 2014 Nov; 134 Suppl 3:S184-5. . View in PubMed

Absence of Detectible HIV-1 Viremia After Treatment Cessation in an Infant Pediatrics. 2014 Nov; 134 Suppl 3:S183-4. . View in PubMed

Synchronous HIV/AIDS-related Epstein-Barr Virus-associated smooth muscle tumors in a 20-year-old female Pediatr Infect Dis J. 2014 Oct; 33(10):1055-6. . View in PubMed

Newborn screening for severe combined immunodeficiency in 11 screening programs in the United States JAMA. 2014 Aug 20; 312(7):729-38. . View in PubMed

Major histocompatibility complex class II deficiency complicated by Mycobacterium avium complex in a boy of mixed ethnicity J Clin Immunol. 2014 Aug; 34(6):677-80. . View in PubMed

Signaling lymphocytic activation molecule (SLAM)/SLAM-associated protein pathway regulates human B-cell tolerance J Allergy Clin Immunol. 2014 Apr; 133(4):1149-61. . View in PubMed

Regional brain gray and white matter changes in perinatally HIV-infected adolescents Neuroimage Clin. 2014; 4:29-34. . View in PubMed

Exome sequencing reveals RAG1 mutations in a child with autoimmunity and sterile chronic multifocal osteomyelitis evolving into disseminated granulomatous disease J Clin Immunol. 2013 Nov; 33(8):1289-92. . View in PubMed

Newborn screening for severe combined immunodeficiency and T-cell lymphopenia in California: results of the first 2 years J Allergy Clin Immunol. 2013 Jul; 132(1):140-50. . View in PubMed

Newborn screening for severe combined immunodeficiency does not identify bare lymphocyte syndrome J Allergy Clin Immunol. 2013 Jun; 131(6):1693-5. . View in PubMed

Subcutaneous Panniculitis-like T-cell lymphoma in two pediatric patients: an HIV-positive adolescent and a 4-month-old infant Fetal Pediatr Pathol. 2013 Jun; 32(3):175-83. . View in PubMed

Newborn screening for SCID identifies patients with ataxia telangiectasia J Clin Immunol. 2013 Apr; 33(3):540-9. . View in PubMed

Acidomonas methanolica-associated necrotizing lymphadenitis in a patient with chronic granulomatous disease J Clin Immunol. 2012 Dec; 32(6):1193-6. . View in PubMed

Hepatic veno-occlusive disease with immunodeficiency (VODI): first reported case in the US. and identification of a unique mutation in Sp110. Clin Immunol. 2012 Nov; 145(2):102-7. . View in PubMed

Clinical, molecular, and cellular immunologic findings in patients with SP110-associated veno-occlusive disease with immunodeficiency syndromeJ Allergy Clin Immunol. 2012 Sep; 130(3):735-742. e6. . View in PubMed

Safety, efficacy and pharmacokinetics of a new 10% liquid intravenous immunoglobulin (IVIG) in patients with primary immunodeficiency J Clin Immunol. 2012 Aug; 32(4):663-9. . View in PubMed

Functional analysis of HIV type 1 Nef gene variants from adolescent and adult survivors of perinatal infection AIDS Res Hum Retroviruses. 2012 May; 28(5):486-92. . View in PubMed

Association of microbial IgE sensitizations with asthma in young children with atopic dermatitis Ann Allergy Asthma Immunol. 2012 Mar; 108(3):212-3. . View in PubMed

Pharmacokinetics of subcutaneous IgPro20 in patients with primary immunodeficiency Clin Pharmacokinet. 2011 Jun; 50(6):405-14. . View in PubMed

Hemophagocytic lymphohistiocytosis in children with chronic granulomatous disease Pediatr Blood Cancer. 2011 Mar; 56(3):460-2. . View in PubMed

A child with x-linked agammaglobulinemia and enthesitis-related arthritis Int J Rheumatol. 2011; 2011:175973. . View in PubMed

Hypohidrotic ectodermal dysplasia and immunodeficiency with coincident NEMO and EDA mutations Front Immunol. 2011; 2:61. . View in PubMed

Short communication: enhanced CD8+ T cell apoptosis in HIV-infected adolescents with virologic failure on protease inhibitor-based therapy AIDS Res Hum Retroviruses. 2010 Jun; 26(6):681-4. . View in PubMed

Skin-deep clues to a complex disease Ann Allergy Asthma Immunol. 2010 Jan; 104(1):93-4. . View in PubMed

Pharmacokinetics of a new 10% intravenous immunoglobulin in patients receiving replacement therapy for primary immunodeficiency Eur J Pharm Sci. 2009 Jun 28; 37(3-4):272-8. . View in PubMed

Safety and efficacy of Privigen, a novel 10% liquid immunoglobulin preparation for intravenous use, in patients with primary immunodeficiencies J Clin Immunol. 2009 Jan; 29(1):137-44. . View in PubMed

Association of staphylococcal superantigen-specific immunoglobulin e with mild and moderate atopic dermatitis J Pediatr. 2008 Dec; 153(6):803-6. . View in PubMed

Down-regulation of atopic dermatitis-associated serum chemokines by wet-wrap treatment: a pilot study Ann Allergy Asthma Immunol. 2008 Mar; 100(3):286-7. . View in PubMed

Safety and efficacy of enfuvirtide for 48 weeks as part of an optimized antiretroviral regimen in pediatric human immunodeficiency virus 1-infected patients Pediatr Infect Dis J. 2007 Sep; 26(9):799-805. . View in PubMed

Leukocyte adhesion deficiency in a female patient without delayed umbilical cord separation J Paediatr Child Health. 2007 May; 43(5):406-8. . View in PubMed

Nonlinear pharmacokinetics of high-dose recombinant fusion protein CD4-IgG2 (PRO 542) observed in HIV-1-infected children J Allergy Clin Immunol. 2007 Mar; 119(3):747-50. . View in PubMed

Pectin anaphylaxis and possible association with cashew allergy Ann Allergy Asthma Immunol. 2006 Dec; 97(6):759-60. . View in PubMed

Case report: evidence for transplacental transfer of maternally administered infliximab to the newborn Clin Gastroenterol Hepatol. 2006 Oct; 4(10):1255-8. . View in PubMed

Denial of insurance authorization at a subspecialty office as a cause of missed opportunities for influenza vaccination in children with asthma Arch Pediatr Adolesc Med. 2006 Sep; 160(9):987-8. . View in PubMed

Susceptibility of pediatric HIV-1 isolates to recombinant CD4-IgG2 (PRO 542) and humanized mAb to the chemokine receptor CCR5 (PRO 140) J Allergy Clin Immunol. 2006 Aug; 118(2):518-21. . View in PubMed

Mixed-up nuts: identification of peanuts and tree nuts by children Ann Allergy Asthma Immunol. 2006 Jul; 97(1):73-7. . View in PubMed

Efficacy, safety and tolerability of a new 10% liquid intravenous immune globulin [IGIV 10%] in patients with primary immunodeficiency J Clin Immunol. 2006 Jul; 26(4):388-95. . View in PubMed

Safety and immunogenicity of live varicella virus vaccine in children with human immunodeficiency virus type 1 Pediatr Infect Dis J. 2006 Apr; 25(4):368-70. . View in PubMed

Thymic volume, T-cell populations, and parameters of thymopoiesis in adolescent and adult survivors of HIV infection acquired in infancy AIDS. 2006 Mar 21; 20(5):667-74. . View in PubMed

Trichosporon pullulans as a complication of chronic granulomatous disease in a patient undergoing immunosuppressive therapy for inflammatory bowel disease Pediatr Infect Dis J. 2006 Jan; 25(1):87-9. . View in PubMed

A pediatric genetic disorder diagnosed in adulthood PLoS Med. 2006 Jan; 3(1):e15. . View in PubMed

Genetic and stochastic influences on the interaction of human immunodeficiency virus type 1 and cytotoxic T lymphocytes in identical twins J Virol. 2005 Dec; 79(24):15368-75. . View in PubMed

Bone measures in HIV-1 infected children and adolescents: disparity between quantitative computed tomography and dual-energy X-ray absorptiometry measurements Osteoporos Int. 2005 Nov; 16(11):1393-6. . View in PubMed

Selective survival of peripheral blood lymphocytes in children with HIV-1 following delivery of an anti-HIV gene to bone marrow CD34(+) cells Mol Ther. 2005 Jul; 12(1):77-86. . View in PubMed

Variable presentation of disseminated nontuberculous mycobacterial infections in a family with an interferon-gamma receptor mutation Clin Infect Dis. 2004 Sep 15; 39(6):868-70. . View in PubMed

Long term tolerability and safety of enfuvirtide for human immunodeficiency virus 1-infected children Pediatr Infect Dis J. 2004 Aug; 23(8):713-8. . View in PubMed

Successful anti-CD20 monoclonal antibody treatment of severe autoimmune hemolytic anemia due to warm reactive IgM autoantibody in a child with common variable immunodeficiency Am J Hematol. 2004 Jun; 76(2):152-5. . View in PubMed

Oxandrolone treatment of childhood hereditary angioedema Ann Allergy Asthma Immunol. 2004 Mar; 92(3):377-8. . View in PubMed

Rapid intravenous desensitization to antithymocyte globulin in a patient with aplastic anemia Transplantation. 2004 Jan 27; 77(2):321-3. . View in PubMed

Population pharmacokinetics of enfuvirtide in pediatric patients with human immunodeficiency virus: searching for exposure-response relationships Clin Pharmacol Ther. 2003 Dec; 74(6):569-80. . View in PubMed

Progressive outer retinal necrosis caused by varicella-zoster virus in children with acquired immunodeficiency syndrome Pediatr Infect Dis J. 2003 Apr; 22(4):384-6. . View in PubMed

Assessment of thymic activity in human immunodeficiency virus-negative and -positive adolescents by real-time PCR quantitation of T-cell receptor rearrangement excision circles Clin Diagn Lab Immunol. 2003 Mar; 10(2):323-8. . View in PubMed

Reversible leukoencephalopathy in a patient with nucleoside analogue-associated mitochondrial DNA depletion and metabolic disease AIDS. 2002 Nov 22; 16(17):2366-7. . View in PubMed

Safety and antiretroviral activity of chronic subcutaneous administration of T-20 in human immunodeficiency virus 1-infected children Pediatr Infect Dis J. 2002 Jul; 21(7):653-9. . View in PubMed

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