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Hans D. Ochs, MD

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Hans D. Ochs, MD

Immunology

On staff since February 1974

Academic Title: Professor, Department of Pediatrics

Research Center: Center for Immunity and Immunotherapies

"I decided to enter the medical profession because the Hippocratic Oath rendered it unique, apolitical and not money-driven; at the same time, medicine promised to be a field where research has a purpose, teaching has an impact and providing care for patients is considered a privilege and a vocation. I am content and happy I did."

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Overview

Board Certification(s)
Allergy and Immunology
Pediatrics
Residency
Pediatrics, University of Washington School of Medicine, Seattle
Fellowship
Allergy and Immunology, University of Washington School of Medicine, Seattle
Research Description

Dr. Ochs' research focuses on the molecular basis of Primary Immune Deficiency Diseases with special interest in the genes that have been linked to the Wiskott Aldrich Syndrome, Hyper IgM syndromes, X-linked Agammaglobulinemia, IPEX syndrome, autosomal recessive Hyper IgE syndrome. To improve the long-term outcome of these disorders, he has actively participated in clinical trials to develop immunoglobulin replacement, hematopoietic stem cell transplantation, and gene therapy.

Dr. Ochs' clinical interests focus on the use of intravenous and subcutaneous immunoglobulin and the in vivo analysis of antibody production using Bacteriophage FX174. He and his collaborators contributed to the identification of several genes associated with PIDD located on the X chromosome, including CD40L, Wiskott-Aldrich Syndrome protein, BTK, and FOXP3.

Recently, he focused on the gene Uracil-DNA Glycosulase, causing a rare form of autosomal recessive hyper IgM syndrome and on STAT3, the gene causing autosomal dominant Hyper IgE Syndrome if mutated. In 1995, he moved the immunodeficiency clinic from the University to Children's Hospital, providing diagnostic evaluations and clinical care for both pediatric and adult patients with PIDD. He initiated and maintained a successful cooperation with the bone marrow transplant unit since the early 70s and participated in the design of protocols related to stem cell transplantation for patients with SCID, Wiskott-Aldrich Syndrome, GGD, and Hyper IgM Syndrome.

He is a PI for the NIH contract supporting the United States Immune Deficiency Network (USIDNet) and member of the U.S. Summer School Faculty devoted to teaching fellows interested in primary immunodeficiency diseases. As co-editor of the textbooks Primary Immunodeficiency Diseases, A Molecular and Genetic Approach, 2nd Edition and for Immunologic Disorders in Infants and Children, 3rd Edition.

The Ochs/Torgerson Lab focuses on:

1) The molecular basis of primary immunodeficiency disorders
2) Autoimmunity and Immune dysregulation
3) Regulatory T cells and mutations of FOXP3
4) Eventual consequences of heterozygous hypermorphic STAT3 mutations and their relationship to autosomal dominant Hyper IgE syndrome.

Research Focus Area

Immunology

Awards and Honors

Award NameAward DescriptionAwarded ByAward Date
 Clinical Immunology Society President’s AwardClinical Immunology SocietyJan. 1, 2009

Publications

Reduced expression of FOXP3 and regulatory T-cell function in severe forms of early-onset autoimmune enteropathy.
Gastroenterology , 2010 Sep: 770-8
A custom 148 gene-based resequencing chip and the SNP explorer software: new tools to study antibody deficiency.
Human mutation , 2010 Sep: 1080-8
Diagnostic approach to the hyper-IgE syndromes: immunologic and clinical key findings to differentiate hyper-IgE syndromes from atopic dermatitis.
The Journal of allergy and clinical immunology , 2010 Sep: 611-7.e1
Stable hematopoietic cell engraftment after low-intensity nonmyeloablative conditioning in patients with immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome.
The Journal of allergy and clinical immunology , 2010 Jul 17
Astrovirus encephalitis in boy with X-linked agammaglobulinemia.
Emerging infectious diseases , 2010 Jun: 918-25
X-linked thrombocytopenia (XLT) due to WAS mutations: clinical characteristics, long-term outcome, and treatment options.
Blood , 2010 Apr 22: 3231-8
Potentiation of TLR9 responses for human naïve B-cell growth through RP105 signaling.
Clinical immunology (Orlando, Fla.) , 2010 Apr: 125-36
Rapid molecular analysis of the STAT3 gene in Job syndrome of hyper-IgE and recurrent infectious diseases.
The Journal of molecular diagnostics : JMD , 2010 Mar: 213-9
Subcutaneous immunoglobulin replacement therapy in the treatment of patients with primary immunodeficiency disease.
Therapeutics and clinical risk management , 2010 Feb 2: 1-10
Improving cellular therapy for primary immune deficiency diseases: recognition, diagnosis, and management.
The Journal of allergy and clinical immunology , 2009 Dec: 1152-60.e12
Primary immunodeficiencies: 2009 update.
The Journal of allergy and clinical immunology , 2009 Dec: 1161-78
Anti-CD3 antibodies modulate anti-factor VIII immune responses in hemophilia A mice after factor VIII plasmid-mediated gene therapy.
Blood , 2009 Nov 12: 4373-82
CD4+FOXP3+ regulatory T cells confer long-term regulation of factor VIII-specific immune responses in plasmid-mediated gene therapy-treated hemophilia mice.
Blood , 2009 Nov 5: 4034-44
FOXP3 forkhead domain mutation and regulatory T cells in the IPEX syndrome.
The New England journal of medicine , 2009 Oct 22: 1710-3
Comèl-Netherton syndrome defined as primary immunodeficiency.
The Journal of allergy and clinical immunology , 2009 Sep: 536-43
FOXP3 inhibits activation-induced NFAT2 expression in T cells thereby limiting effector cytokine expression.
Journal of immunology (Baltimore, Md. : 1950) , 2009 Jul 15: 907-15
Wiskott-Aldrich syndrome protein is required for homeostasis and function of invariant NKT cells.
Journal of immunology (Baltimore, Md. : 1950) , 2009 Jun 15: 7370-80
TH17 cells and regulatory T cells in primary immunodeficiency diseases.
The Journal of allergy and clinical immunology , 2009 May: 977-83; quiz 984-5
Immune responses in adult female volunteers during the bed-rest model of spaceflight: antibodies and cytokines.
The Journal of allergy and clinical immunology , 2009 Apr: 900-5
FBP17 Mediates a Common Molecular Step in the Formation of Podosomes and Phagocytic Cups in Macrophages.
The Journal of biological chemistry , 2009 Mar 27: 8548-56
Relevance of biallelic versus monoallelic TNFRSF13B mutations in distinguishing disease-causing from risk-increasing TNFRSF13B variants in antibody deficiency syndromes.
Blood , 2009 Feb 26: 1967-76
Immunologic reconstitution during PEG-ADA therapy in an unusual mosaic ADA deficient patient.
Clinical immunology (Orlando, Fla.) , 2009 Feb: 162-74
Interleukin-21 stimulates B-cell immunoglobulin E synthesis in human beings concomitantly with activation-induced cytidine deaminase expression and differentiation into plasma cells.
Human immunology , 2009 Jan: 35-40
Mutations of the Wiskott-Aldrich Syndrome Protein affect protein expression and dictate the clinical phenotypes.
Immunologic research , 2009: 84-8
Wiskott-Aldrich syndrome: diagnosis, clinical and laboratory manifestations, and treatment.
Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation , 2009 Jan: 84-90
WASP confers selective advantage for specific hematopoietic cell populations and serves a unique role in marginal zone B-cell homeostasis and function.
Blood , 2008 Nov 15: 4139-47
Effect of therapeutic integrin (CD11a) blockade with efalizumab on immune responses to model antigens in humans: results of a randomized, single blind study.
The Journal of investigative dermatology , 2008 Nov: 2615-24
Transient blockade of the inducible costimulator pathway generates long-term tolerance to factor VIII after nonviral gene transfer into hemophilia A mice.
Blood , 2008 Sep 1: 1662-72
Familial hemophagocytic lymphohistiocytosis in two brothers with X-linked agammaglobulinemia.
Pediatric blood & cancer , 2008 Aug: 293-5
Restricted immunoglobulin constant heavy G chain genes in primary immunodeficiencies.
Clinical immunology (Orlando, Fla.) , 2008 Aug: 190-8
Novel signal transducer and activator of transcription 3 (STAT3) mutations, reduced T(H)17 cell numbers, and variably defective STAT3 phosphorylation in hyper-IgE syndrome.
The Journal of allergy and clinical immunology , 2008 Jul: 181-7
Patients with abnormal IgM levels: assessment, clinical interpretation, and treatment.
Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology , 2008 May: 509-11
Wiskott-Aldrich syndrome.
Current opinion in hematology , 2008 Jan: 30-6
Developmental changes of FOXP3-expressing CD4+CD25+ regulatory T cells and their impairment in patients with FOXP3 gene mutations.
Clinical immunology (Orlando, Fla.) , 2007 Dec: 237-46
Regulatory T cells in primary immunodeficiency diseases.
Current opinion in allergy and clinical immunology , 2007 Dec: 515-21
Contiguous X-chromosome deletion syndrome encompassing the BTK, TIMM8A, TAF7L, and DRP2 genes.
Journal of clinical immunology , 2007 Nov: 640-6
STAT3 mutation in the original patient with Job's syndrome.
The New England journal of medicine , 2007 Oct 18: 1667-8
Immune dysregulation, polyendocrinopathy, enteropathy, X-linked: forkhead box protein 3 mutations and lack of regulatory T cells.
The Journal of allergy and clinical immunology , 2007 Oct: 744-50; quiz 751-2
Images in immunodeficiency.
The Journal of allergy and clinical immunology , 2007 Oct: 982-4
Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee.
The Journal of allergy and clinical immunology , 2007 Oct: 776-94
Severe food allergy as a variant of IPEX syndrome caused by a deletion in a noncoding region of the FOXP3 gene.
Gastroenterology , 2007 May: 1705-17
A potential screening tool for IPEX syndrome.
Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society , 2007 Mar-Apr: 98-105
WIP is a chaperone for Wiskott-Aldrich syndrome protein (WASP).
Proceedings of the National Academy of Sciences of the United States of America , 2007 Jan 16: 926-31
Immune dysregulation, polyendocrinopathy, enteropathy, X-linked inheritance: model for autoaggression.
Advances in experimental medicine and biology , 2007: 27-36
IPEX, FOXP3 and regulatory T-cells: a model for autoimmunity.
Immunologic research , 2007: 112-21
Human tyrosine kinase 2 deficiency reveals its requisite roles in multiple cytokine signals involved in innate and acquired immunity.
Immunity , 2006 Nov: 745-55
Analysis of FOXP3 reveals multiple domains required for its function as a transcriptional repressor.
Journal of immunology (Baltimore, Md. : 1950) , 2006 Sep 1: 3133-42
Immunomodulation of transgene responses following naked DNA transfer of human factor VIII into hemophilia A mice.
Blood , 2006 Jul 1: 19-27
X-linked agammaglobulinemia: report on a United States registry of 201 patients.
Medicine , 2006 Jul: 193-202
Wiskott-Aldrich syndrome protein is involved in alphaIIb beta3-mediated cell adhesion.
EMBO reports , 2006 May: 506-11
Safety and efficacy of self-administered subcutaneous immunoglobulin in patients with primary immunodeficiency diseases.
Journal of clinical immunology , 2006 May: 265-73
Single-cell analysis of normal and FOXP3-mutant human T cells: FOXP3 expression without regulatory T cell development.
Proceedings of the National Academy of Sciences of the United States of America , 2006 Apr 25: 6659-64
The Wiskott-Aldrich syndrome.
The Journal of allergy and clinical immunology , 2006 Apr: 725-38; quiz 739
X-linked agammaglobulinemia in northern Thailand.
Asian Pacific journal of allergy and immunology / launched by the Allergy and Immunology Society of Thailand , 2006 Mar: 57-63
Health-related quality of life and treatment satisfaction in North American patients with primary immunedeficiency diseases receiving subcutaneous IgG self-infusions at home.
Journal of clinical immunology , 2006 Jan: 65-72
WASP and the phenotypic range associated with deficiency.
Current opinion in allergy and clinical immunology , 2005 Dec: 485-90
Successful use of the new immune-suppressor sirolimus in IPEX (immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome).
The Journal of pediatrics , 2005 Aug: 256-9
Gains of glycosylation comprise an unexpectedly large group of pathogenic mutations.
Nature genetics , 2005 Jul: 692-700
Structure and function of the Wiskott-Aldrich syndrome protein.
Current opinion in hematology , 2005 Jul: 284-91
WAVE/Scars in platelets.
Blood , 2005 Apr 15: 3141-8
Molecular analysis of a large cohort of patients with the hyper immunoglobulin M (IgM) syndrome.
Blood , 2005 Mar 1: 1881-90
The Wiskott-Aldrich syndrome protein regulates nuclear translocation of NFAT2 and NF-kappa B (RelA) independently of its role in filamentous actin polymerization and actin cytoskeletal rearrangement.
Journal of immunology (Baltimore, Md. : 1950) , 2005 Mar 1: 2602-11
FOXP3 acts as a rheostat of the immune response.
Immunological reviews , 2005 Feb: 156-64
Rituximab inhibits the in vivo primary and secondary antibody response to a neoantigen, bacteriophage phiX174.
American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons , 2005 Jan: 50-7
Mutations of the Wiskott-Aldrich Syndrome Protein (WASP): hotspots, effect on transcription, and translation and phenotype/genotype correlation.
Blood , 2004 Dec 15: 4010-9
The hyper IgM syndrome--an evolving story.
Pediatric research , 2004 Oct: 519-25
X-linked immunodeficiencies.
Current allergy and asthma reports , 2004 Sep: 339-48
Naked DNA transfer of Factor VIII induced transgene-specific, species-independent immune response in hemophilia A mice.
Molecular therapy : the journal of the American Society of Gene Therapy , 2004 Jul: 117-26
Octagam 5%, an intravenous IgG product, is efficacious and well tolerated in subjects with primary immunodeficiency diseases.
Journal of clinical immunology , 2004 May: 309-14
Dermatologic and immunologic findings in the immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome.
Archives of dermatology , 2004 Apr: 466-72
Primary immunodeficiency: looking backwards, looking forwards.
The Journal of allergy and clinical immunology , 2004 Apr: 607-9
Clinical course of patients with WASP gene mutations.
Blood , 2004 Jan 15: 456-64
WASP (Wiskott-Aldrich syndrome protein) gene mutations and phenotype.
Current opinion in allergy and clinical immunology , 2003 Dec: 427-36
CD4+ T-cell-directed antibody responses are maintained in patients with psoriasis receiving alefacept: results of a randomized study.
Journal of the American Academy of Dermatology , 2003 Nov: 816-25
Inducible CO-stimulator molecule, a candidate gene for defective isotype switching, is normal in patients with hyper-IgM syndrome of unknown molecular diagnosis.
The Journal of allergy and clinical immunology , 2003 Nov: 958-64
Human uracil-DNA glycosylase deficiency associated with profoundly impaired immunoglobulin class-switch recombination.
Nature immunology , 2003 Oct: 1023-8
Wiskott-Aldrich Syndrome: a model for defective actin reorganization, cell trafficking and synapse formation.
Current opinion in immunology , 2003 Oct: 585-91
Functional analysis of human memory B-cell subpopulations: IgD+CD27+ B cells are crucial in secondary immune response by producing high affinity IgM.
Clinical immunology (Orlando, Fla.) , 2003 Aug: 128-37
Immune dysregulation, polyendocrinopathy, enteropathy, and X-linked inheritance (IPEX), a syndrome of systemic autoimmunity caused by mutations of FOXP3, a critical regulator of T-cell homeostasis.
Current opinion in rheumatology , 2003 Jul: 430-5
Novel Artemis gene mutations of radiosensitive severe combined immunodeficiency in Japanese families.
Human genetics , 2003 Apr: 348-52
[X-linked hyper-IGM syndrome associated to sclerosing cholangitis and gallbladder neoplasm: clinical case]
Revista medica de Chile , 2003 Mar: 303-8
Immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome: a model of immune dysregulation.
Current opinion in allergy and clinical immunology , 2002 Dec: 481-7
Unimpaired activation of c-Jun NH2-terminal kinase (JNK) 1 upon CD40 stimulation in B cells of patients with X-linked agammaglobulinemia.
Journal of clinical immunology , 2002 Jul: 244-51
Immune reconstitution in ADA-SCID after PBL gene therapy and discontinuation of enzyme replacement.
Nature medicine , 2002 May: 423-5
The Wiskott-Aldrich syndrome.
The Israel Medical Association journal : IMAJ , 2002 May: 379-84
Absence of memory B cells in patients with common variable immunodeficiency.
Clinical immunology (Orlando, Fla.) , 2002 Apr: 34-42
Missense mutations of the WASP gene cause intermittent X-linked thrombocytopenia.
Blood , 2002 Mar 15: 2268-9
Progressive neurodegeneration in patients with primary immunodeficiency disease on IVIG treatment.
Clinical immunology (Orlando, Fla.) , 2002 Jan: 19-24
Survival of wild polio by a patient with XLA.
Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology , 2002 Jan: 59-60

Primary Office

Seattle Children's Research Institute
C9S - 7 - Immunology
1900 - 9th Ave
Seattle, WA 98101
206-987-7450

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