Keck School Faculty

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

Defining clinical subgroups and genotype-phenotype correlations in NBAS-associated disease across 110 patients Genet Med. 2019 Nov 25. . View in PubMed

Human SNORA31 variations impair cortical neuron-intrinsic immunity to HSV-1 and underlie herpes simplex encephalitis Nat Med. 2019 12; 25(12):1873-1884. . View in PubMed

Lentiviral Gene Therapy Combined with Low-Dose Busulfan in Infants with SCID-X1 N Engl J Med. 2019 04 18; 380(16):1525-1534. . View in PubMed

Dominant activating RAC2 mutation with lymphopenia, immunodeficiency, and cytoskeletal defects Blood. 2019 05 02; 133(18):1977-1988. . View in PubMed

Chronic rhinosinusitis with severe nasal polyposis in hyper-IgE syndrome Ann Allergy Asthma Immunol. 2018 12; 121(6):738-739. . View in PubMed

Genetic influences on hormonal markers of chronic hypothalamic-pituitary-adrenal function in human hair Psychol Med. 2017 Jan 19; 1-13. . 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

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

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

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

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

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 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

Neuropsychological function and cerebral metabolites in HIV-infected youth J Neuroimmune Pharmacol. 2012 Dec; 7(4):981-90. . 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

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

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

Late-onset of IgE sensitization to microbial allergens in young children with atopic dermatitis Br J Dermatol. 2010 Jan; 162(1):159-61. . 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

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

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

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

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

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

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

Mitochondrial DNA depletion, near-fatal metabolic acidosis, and liver failure in an HIV-infected child treated with combination antiretroviral therapy J Pediatr. 2001 May; 138(5):748-51. . View in PubMed

Determination of dosing guidelines for stavudine (2',3'-didehydro-3'-deoxythymidine) in children with human immunodeficiency virus infection Antimicrob Agents Chemother. 2001 Mar; 45(3):758-63. . View in PubMed

A randomized, double-blind study of triple nucleoside therapy of abacavir, lamivudine, and zidovudine versus lamivudine and zidovudine in previously treated human immunodeficiency virus type 1-infected childrenThe CNAA3006 Study Team. Pediatrics. 2001 Jan; 107(1):E4. . View in PubMed

Failure of intravenous immunoglobulin to prevent varicella-zoster infection Pediatr Infect Dis J. 2000 Dec; 19(12):1219-20. . View in PubMed

Neonatal urticaria as a symptom of a multisystem inflammatory disease J Allergy Clin Immunol. 2000 Nov; 106(5):986-7. . View in PubMed

Gene therapy for pediatric AIDS Ann N Y Acad Sci. 2000 Nov; 918:318-29. . View in PubMed

HIV disease in childrenThe many ways it differs from the disease in adults. Postgrad Med. 2000 Apr; 107(4):163-6169-71175-6 passim. . View in PubMed

Development of an instrument to assess nutritional risk factors for children infected with human immunodeficiency virus J Am Diet Assoc. 2000 Mar; 100(3):323-9. . View in PubMed

Evaluation of the effects of oxandrolone on malnourished HIV-positive pediatric patients Pediatrics. 1999 Dec; 104(6):e73. . View in PubMed

Effects of high-dose intravenous immunoglobulin on virus load in HIV-infected children J Acquir Immune Defic Syndr. 1999 Nov 01; 22(3):309-11. . View in PubMed

A clinical trial of retroviral-mediated transfer of a rev-responsive element decoy gene into CD34(+) cells from the bone marrow of human immunodeficiency virus-1-infected children Blood. 1999 Jul 01; 94(1):368-71. . View in PubMed

Maggots are useful in treating infected or necrotic wounds BMJ. 1999 Mar 20; 318(7186):807-8. . View in PubMed

Ethical issues of placebo-controlled trials J Pediatr. 1999 Feb; 134(2):251-2. . View in PubMed

Effects of noradrenaline on intracellular pH in acutely dissociated adult rat hippocampal CA1 neurones J Physiol. 1998 Oct 15; 512 ( Pt 2):487-505. . View in PubMed

Adenovirus viremia in human immunodeficiency virus-infected children Pediatr Infect Dis J. 1997 Apr; 16(4):413-5. . View in PubMed

Immunologic evaluation of the child with recurrent otitis media Ear Nose Throat J. 1997 Jan; 76(1):31-442. . View in PubMed

Lamivudine in children with human immunodeficiency virus infection: a phase I/II studyThe National Cancer Institute Pediatric Branch-Human Immunodeficiency Virus Working Group. J Infect Dis. 1996 Jul; 174(1):16-25. . View in PubMed

Acquired protein S deficiency in children infected with human immunodeficiency virus Pediatr Infect Dis J. 1996 Feb; 15(2):106-11. . View in PubMed

Frequent recurrence and persistence of varicella-zoster virus infections in children infected with human immunodeficiency virus type 1 J Pediatr. 1996 Jan; 128(1):52-7. . View in PubMed

A phase I/II evaluation of stavudine (d4T) in children with human immunodeficiency virus infection Pediatrics. 1995 Aug; 96(2 Pt 1):247-52. . View in PubMed

A rapid and sensitive chemiluminescence assay for evaluation of functional opsonic activity of Haemophilus influenzae type b-specific antibodies Clin Diagn Lab Immunol. 1995 May; 2(3):286-90. . View in PubMed

A comparative study of transfusion-acquired human immunodeficiency virus-infected children with and without disseminated Mycobacterium avium complex Pediatr Infect Dis J. 1994 Jun; 13(6):484-8. . View in PubMed

Immunologic and virologic effects of glucocorticoids on human immunodeficiency virus infection in children: a preliminary study Pediatr Infect Dis J. 1994 Mar; 13(3):212-6. . View in PubMed

Mycobacterium avium complex in HIV-infected children Ann N Y Acad Sci. 1993 Oct 29; 693:288-90. . View in PubMed

Evaluation of families wherein a single male manifests a phenotype of X-linked lymphoproliferative disease (XLP) Am J Med Genet. 1993 Sep 15; 47(4):458-63. . View in PubMed

Correction of antigen-specific T-lymphocyte function by recombinant cytokines in children infected with human immunodeficiency virus type 1 J Pediatr. 1992 Oct; 121(4):565-8. . View in PubMed

Comparison of cytobrush with Cervex-Brush for endocervical cytologic sampling J Adolesc Health. 1992 Sep; 13(6):520-3. . View in PubMed

Chronic arthritis in a 3-year-old boy Ann Allergy. 1992 Mar; 68(3):215-8. . View in PubMed

Human immunodeficiency virus infection in children who received transfusions in Mexico West J Med. 1991 Nov; 155(5):547. . View in PubMed

An inherited defect of neutrophil motility and microfilamentous cytoskeleton associated with abnormalities in 47-Kd and 89-Kd proteins Blood. 1991 Sep 01; 78(5):1338-46. . View in PubMed

CD4 T-lymphocyte counts and Pneumocystis carinii pneumonia in pediatric HIV infection JAMA. 1991 Apr 03; 265(13):1698-703. . View in PubMed

Complications of central venous catheters in pediatric patients with acquired immunodeficiency syndrome Pediatr Infect Dis J. 1991 Jan; 10(1):11-4. . View in PubMed

Lymphocyte phenotype does not predict immune function in pediatric patients infected with human immunodeficiency virus type 1 J Pediatr. 1989 Dec; 115(6):944-8. . View in PubMed

Comfort of male adolescents during general and genital examination J Pediatr. 1989 Sep; 115(3):494-7. . View in PubMed

Immune functions in children treated with biosynthetic growth hormone J Pediatr. 1989 Sep; 115(3):420-3. . View in PubMed

Lack of functional asplenia in HIV infection AIDS Res Hum Retroviruses. 1989 Jun; 5(3):257-8. . View in PubMed

Lymphomatoid granulomatosis in a 13-month-old infant J Rheumatol. 1989 Feb; 16(2):235-8. . View in PubMed

Evaluation of a new, shorter method of administration of adrenergic aerosols in the treatment of asthma Ann Allergy. 1988 Aug; 61(2):147-50. . View in PubMed

Cardiac arrest associated with halothane anesthesia in a patient receiving theophylline Ann Allergy. 1988 Aug; 61(2):83-4. . View in PubMed

Human immunodeficiency virus-associated Kaposi's sarcoma in a pediatric renal transplant recipient Nephron. 1987; 47(1):62-5. . View in PubMed

New scarlet letter(s), pediatric AIDS Pediatrics. 1986 Mar; 77(3):423-7. . View in PubMed

Lymphoid interstitial pneumonia and rheumatoid arthritis J Pediatr. 1985 Sep; 107(3):485. . View in PubMed

Abnormal T-cell subsets and mitogen responses in hemophiliacs exposed to factor concentrate Am J Dis Child. 1984 Jul; 138(7):645-8. . View in PubMed

Induction of tolerance to factor VIII in a child with a high-titer inhibitor: in vitro and in vivo observations J Pediatr. 1984 Jan; 104(1):70-5. . View in PubMed

Administration of metered-dose inhalers: comparison of open- and closed-mouth techniques in childhood asthmatics Ann Allergy. 1983 Dec; 51(6):571-3. . View in PubMed

Antimuscle antibody in infantile botulism West J Med. 1983 Feb; 138(2):266-7. . View in PubMed

Isoetharine-isoproterenol: a comparison of effects in childhood status asthmaticus Ann Allergy. 1982 Apr; 48(4):230-2. . View in PubMed

An allergy-clinical immunology training program for pediatric residents Ann Allergy. 1982 Apr; 48(4):227-9. . View in PubMed

Los Angeles air pollution and asthma in children Ann Allergy. 1981 Nov; 47(5 Pt 1):348-54. . View in PubMed

Lymphoid interstitial pneumonitis and hypogammaglobulinemia in children Am Rev Respir Dis. 1981 Oct; 124(4):491-6. . View in PubMed

A self-help program for childhood asthma in a residential treatment center Clin Pediatr (Phila). 1981 Jul; 20(7):453-7. . View in PubMed

Neutrophil and monocyte chemotaxis in acutely infected patients with cystic fibrosis Ann Allergy. 1980 Oct; 45(4):217-9. . View in PubMed

Abnormal absorption of a sustained-release theophylline preparation Ann Allergy. 1980 Aug; 45(2):95-6. . View in PubMed

Eosinophilia in the hospitalized neonate Ann Allergy. 1980 Jun; 44(6):349-52. . View in PubMed

Immunological mechanisms in the maintenance of pregnancy Ann Allergy. 1980 Mar; 44(3):166-73. . View in PubMed

Inhibition of polymorphonuclear leukocyte chemotaxis by streptokinase-streptodornase Ann Allergy. 1979 Dec; 43(6):333-6. . View in PubMed

Agammaglobulinemia and poliomyelitis-like illness Ann Allergy. 1979 Feb; 42(2):86-7. . View in PubMed

Cow's milk allergy in a premature infant with hypereosinophilia and hyperimmunoglobulinemia E Ann Allergy. 1978 Nov; 41(5):307-10. . View in PubMed

Hyposmia associated with atopy Ann Allergy. 1978 Feb; 40(2):105-9. . View in PubMed

Air leak syndromes as complications of respiratory disease in infancy and childhood Ann Allergy. 1977 Dec; 39(6):393-6. . View in PubMed

Improvement of idiopathic nephrotic syndrome following allergy therapy Ann Allergy. 1977 Nov; 39(5):332-3. . View in PubMed

A quantitative fluorochromatic assay for alloantibodies Transplantation. 1968 Dec; 6(9):1010-4. . View in PubMed

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