Michael S Hershfield Selected Research

Severe combined immunodeficiency due to adenosine deaminase deficiency

1/2022	Outcomes following treatment for ADA-deficient severe combined immunodeficiency: a report from the PIDTC.
10/2021	Long-term outcomes after gene therapy for adenosine deaminase severe combined immune deficiency.
10/2021	Normal IgH Repertoire Diversity in an Infant with ADA Deficiency After Gene Therapy.
1/2020	Morbidity in an adenosine deaminase-deficient patient during 27 years of enzyme replacement therapy.
1/2019	Consensus approach for the management of severe combined immune deficiency caused by adenosine deaminase deficiency.
12/2015	Effects of enzyme replacement therapy on immune function in ADA deficiency patient.
10/2015	Diagnosis, Treatment and Long-Term Follow Up of Patients with ADA Deficiency: a Single-Center Experience.
5/2015	Outcomes in two Japanese adenosine deaminase-deficiency patients treated by stem cell gene therapy with no cytoreductive conditioning.
12/2013	Elevated IgE and atopy in patients treated for early-onset ADA-SCID.
2/2013	Successful bone marrow transplantation with reduced intensity conditioning in a patient with delayed-onset adenosine deaminase deficiency.

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Michael S Hershfield Research Topics

Disease

22	Severe combined immunodeficiency due to adenosine deaminase deficiency 01/2022 - 02/2002
13	Severe Combined Immunodeficiency (Bare Lymphocyte Syndrome) 01/2022 - 01/2005
10	Gout 01/2022 - 05/2001
3	Hypersensitivity (Allergy) 01/2022 - 03/2007
3	Purine Nucleoside Phosphorylase Deficiency 03/2010 - 11/2004
2	Gouty Arthritis 11/2019 - 01/2019
2	deficiency of adenosine deaminase 2 01/2018 - 11/2017
2	Schmid-Fraccaro syndrome 01/2018 - 03/2014
2	Infections 12/2015 - 12/2005
2	Hyperuricemia 03/2014 - 01/2006
2	Lymphopenia (Lymphocytopenia) 09/2011 - 11/2004
1	Sarcoma (Soft Tissue Sarcoma) 10/2021
1	Familial Mediterranean Fever (Periodic Disease) 01/2021
1	autosomal dominant familial Periodic fever 01/2021
1	Fever (Fevers) 01/2021
1	Lymphadenitis (Adenitis) 01/2021
1	Pharyngitis (Sore Throat) 01/2021
1	Aphthous Stomatitis (Aphthous Ulcer) 01/2021
1	Mevalonate Kinase Deficiency 01/2021
1	Cryopyrin-Associated Periodic Syndromes 01/2021
1	Juvenile Arthritis (Juvenile Idiopathic Arthritis) 01/2020
1	Macrophage Activation Syndrome 01/2020
1	Deficiency Diseases (Deficiency Disease) 10/2017
1	Vasculitis (Vasculitides) 10/2016
1	Pure Red-Cell Aplasia 10/2016
1	GATA2 Deficiency 01/2016
1	Type 2 Diabetes Mellitus (MODY) 01/2016
1	Neutropenia 09/2011
1	Knee Osteoarthritis 02/2011
1	Osteoarthritis 02/2011
1	Failure to Thrive 03/2010
1	Neoplasms (Cancer) 03/2010
1	Reinfection 03/2010
1	Autoimmune Diseases (Autoimmune Disease) 03/2010
1	Nervous System Diseases (Neurological Disorders) 03/2010
1	Metabolic Diseases (Metabolic Disease) 04/2007
1	Pancytopenia 01/2007
1	Headache (Headaches) 12/2005
1	Lymphoma (Lymphomas) 12/2005
1	Inflammation (Inflammations) 01/2005
1	Prostatic Neoplasms (Prostate Cancer) 05/2004
1	Diabetes Insipidus 05/2001

Drug/Important Bio-Agent (IBA)

26	Adenosine DeaminaseIBA 01/2022 - 02/2002
9	Uric Acid (Urate)IBA 11/2019 - 05/2001
7	Urate Oxidase (Uricase)IBA 01/2022 - 05/2001
5	Pegloticase FDA Link 03/2014 - 05/2001
4	EnzymesIBA 01/2022 - 03/2007
4	Polyethylene Glycols (Polyethylene Glycol)FDA LinkGeneric 01/2016 - 07/2005
3	Adenosine (Adenocard)FDA LinkGeneric 12/2015 - 01/2005
2	AntibodiesIBA 01/2022 - 03/2007
2	InflammasomesIBA 01/2021 - 02/2011
2	Biomarkers (Surrogate Marker)IBA 01/2020 - 12/2005
2	Ethylene Glycol (Monoethylene Glycol)IBA 01/2006 - 05/2001
1	Pharmaceutical PreparationsIBA 01/2022
1	Busulfan (Busulfex)FDA Link 10/2021
1	Deficiency of interleukin-1 receptor antagonistIBA 01/2021
1	Blood Glucose (Blood Sugar)IBA 01/2016
1	POEGMAIBA 01/2016
1	Nucleotide AptamersIBA 01/2016
1	RB 006IBA 01/2016
1	Exenatide (Byetta)FDA Link 01/2016
1	Peptides (Polypeptides)IBA 01/2016
1	Immunoglobulin E (IgE)IBA 12/2013
1	Anti-Bacterial Agents (Antibiotics)IBA 09/2011
1	Interleukin-18 (Interleukin 18)IBA 02/2011
1	F2-Isoprostanes (F2 Isoprostanes)IBA 08/2010
1	AntioxidantsIBA 08/2010
1	Arachidonic Acid (Vitamin F)IBA 08/2010
1	LipidsIBA 08/2010
1	Proteins (Proteins, Gene)FDA Link 08/2010
1	Purine-Nucleoside PhosphorylaseIBA 03/2010
1	purineIBA 10/2009
1	triphosphoric acid (triphosphate)IBA 10/2007
1	2'-deoxyadenosineIBA 10/2007
1	Febuxostat FDA Link 06/2007
1	Immunoglobulin G (IgG)IBA 03/2007
1	pegademase bovine (pegADA)FDA Link 12/2005
1	Dihydrotachysterol (AT 10)IBA 12/2005
1	CytokinesIBA 07/2005
1	beta-apocarotenoid-14',13'-dioxygenase (ADO)IBA 01/2005
1	Purinergic P1 Receptors (Adenosine Receptor)IBA 01/2005
1	butyl phosphorotrithioate (DEF)IBA 11/2004
1	A-factor (Streptomyces)IBA 05/2004
1	PlasminogenIBA 05/2004

Therapy/Procedure

7	Hematopoietic Stem Cell Transplantation 10/2021 - 04/2007
7	Therapeutics 11/2012 - 05/2001
6	Enzyme Replacement Therapy 10/2021 - 02/2002
2	Subcutaneous Injections 01/2016 - 01/2006
2	Transplantation 10/2015 - 11/2004
2	Bone Marrow Transplantation (Transplantation, Bone Marrow) 02/2013 - 06/2008
2	Stem Cell Transplantation 10/2009 - 12/2005
1	Investigational Therapies (Experimental Therapy) 09/2011
1	Drug Therapy (Chemotherapy) 09/2011