Priya S Kishnani Selected Research

glucose tetrasaccharide

3/2021	Diurnal variability of glucose tetrasaccharide (Glc4) excretion in patients with glycogen storage disease type III.
1/2021	Quantitative whole-body magnetic resonance imaging in children with Pompe disease: Clinical tools to evaluate severity of muscle disease.
1/2015	Baseline Urinary Glucose Tetrasaccharide Concentrations in Patients with Infantile- and Late-Onset Pompe Disease Identified by Newborn Screening.
2/2012	Assessing disease severity in Pompe disease: the roles of a urinary glucose tetrasaccharide biomarker and imaging techniques.
7/2009	Long-term monitoring of patients with infantile-onset Pompe disease on enzyme replacement therapy using a urinary glucose tetrasaccharide biomarker.
8/2005	Glucose tetrasaccharide as a biomarker for monitoring the therapeutic response to enzyme replacement therapy for Pompe disease.

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Priya S Kishnani Research Topics

Disease

126	Glycogen Storage Disease Type II (Pompe's Disease) 05/2022 - 08/2005
20	Down Syndrome (Down's Syndrome) 01/2022 - 01/2003
13	Glycogen Storage Disease (Glycogenosis) 03/2021 - 07/2010
12	Disease Progression 01/2022 - 12/2011
11	Cardiomyopathies (Cardiomyopathy) 09/2022 - 05/2007
11	Gaucher Disease (Gaucher's Disease) 06/2021 - 03/2013
11	Hypophosphatasia 01/2021 - 01/2016
10	Muscular Diseases (Myopathy) 09/2022 - 05/2006
10	Glycogen Storage Disease Type III (Cori's Disease) 12/2021 - 03/2007
9	Muscle Weakness 12/2021 - 12/2006
8	Fibrosis (Cirrhosis) 06/2022 - 03/2007
6	Glycogen Storage Disease Type Ix 01/2021 - 12/2011
6	Glycogen Storage Disease IIIA 09/2020 - 11/2012
5	Liver Cirrhosis (Hepatic Cirrhosis) 06/2022 - 06/2014
5	Hepatomegaly 06/2022 - 01/2017
5	Liver Diseases (Liver Disease) 06/2022 - 03/2007
5	Hypoglycemia (Reactive Hypoglycemia) 01/2019 - 04/2010
4	Metabolic Bone Diseases (Osteopenia) 01/2021 - 10/2016
4	Pain (Aches) 11/2020 - 01/2017
4	Infections 10/2020 - 09/2009
4	Neoplasms (Cancer) 01/2020 - 08/2006
4	Hepatorenal form of glycogen storage disease 11/2018 - 11/2007
4	Lysosomal Storage Diseases (Lysosomal Storage Disease) 02/2016 - 12/2006
3	Alzheimer Disease (Alzheimer's Disease) 04/2021 - 12/2006
3	Glycogen Storage Disease Type I 02/2021 - 04/2010
3	Liver Cell Adenoma (Hepatocellular Adenoma) 02/2021 - 11/2007
3	Body Weight (Weight, Body) 01/2021 - 04/2010
3	Anemia 01/2020 - 09/2012
3	Glycogen Storage Disease IB 01/2019 - 09/2012
3	Hyperlipidemias (Hyperlipidemia) 01/2019 - 04/2010
3	Glycogen Storage Disease Type IV (Andersen's Disease) 01/2017 - 01/2016
3	Cardiac Arrhythmias (Arrythmia) 01/2015 - 08/2007
3	Vacuolar myopathy 11/2014 - 01/2010
2	Anaphylaxis (Anaphylactic Shock) 12/2021 - 04/2010
2	Respiratory Insufficiency (Respiratory Failure) 01/2021 - 01/2017
2	Congenital Abnormalities (Deformity) 01/2021 - 02/2019
2	Metabolic Diseases (Metabolic Disease) 01/2021 - 11/2020
2	Spasm (Spasms) 01/2020 - 01/2018
2	Cardiomegaly (Heart Hypertrophy) 01/2020 - 10/2008
2	Tremor (Tremors) 01/2020 - 01/2018
2	Cognitive Dysfunction 01/2019 - 12/2010
2	Splenomegaly 01/2019 - 03/2018
2	Thrombocytopenia (Thrombopenia) 09/2017 - 03/2013

Drug/Important Bio-Agent (IBA)

65	AcidsIBA 05/2022 - 08/2003
52	human GAA protein (Myozyme)FDA Link 09/2022 - 08/2005
49	GlycogenIBA 05/2022 - 12/2006
36	GlucosidasesIBA 05/2022 - 12/2010
36	EnzymesIBA 01/2022 - 01/2003
29	alpha-Glucosidases (Acid Maltase)IBA 01/2021 - 05/2006
12	Biomarkers (Surrogate Marker)IBA 01/2022 - 08/2005
10	N- (4'- fluorobutyrophenone)- 4- (4- chlorophenyl)pyridinium (HPP(+))IBA 01/2021 - 01/2016
8	AntibodiesIBA 07/2019 - 01/2009
7	Alkaline PhosphataseIBA 01/2021 - 12/2016
6	Pharmaceutical PreparationsIBA 12/2021 - 04/2010
6	glucose tetrasaccharideIBA 03/2021 - 08/2005
6	Methotrexate (Mexate)FDA LinkGeneric 01/2021 - 08/2006
6	asfotase alfaIBA 01/2021 - 01/2017
6	Donepezil (Aricept)FDA LinkGeneric 12/2010 - 01/2003
5	Liver GlycogenIBA 06/2022 - 11/2012
5	Intravenous Immunoglobulins (IVIG)FDA Link 01/2021 - 04/2012
5	Rituximab (Mabthera)FDA Link 01/2021 - 04/2012
5	Glycogen Debranching Enzyme System (Glycogen Debranching Enzyme)IBA 09/2020 - 03/2007
5	Hemoglobins (Hemoglobin)IBA 01/2020 - 09/2012
5	Proteins (Proteins, Gene)FDA Link 01/2019 - 05/2011
4	Glucose-6-Phosphatase (Glucose 6 Phosphatase)IBA 10/2019 - 04/2008
4	Glucose (Dextrose)FDA LinkGeneric 01/2019 - 04/2008
4	eliglustatIBA 01/2019 - 02/2016
3	DextrinsIBA 03/2021 - 03/2007
3	IsoenzymesIBA 01/2021 - 05/2006
3	Albuterol (Salbutamol)FDA LinkGeneric 01/2020 - 02/2012
3	Transaminases (Aminotransferases)IBA 12/2019 - 01/2017
3	Immunoglobulin G (IgG)IBA 07/2019 - 02/2010
3	Phosphorylase Kinase (Phosphorylase Kinase, Glycogen)IBA 01/2019 - 03/2014
3	ClenbuterolIBA 01/2018 - 02/2012
3	imiglucerase FDA Link 09/2017 - 03/2013
3	Cholinergic Agents (Cholinergics)IBA 01/2016 - 12/2006
3	Rivastigmine (Exelon)FDA LinkGeneric 01/2016 - 12/2006
2	Glucosylceramides (Glucocerebrosides)IBA 06/2021 - 02/2016
2	sphingosyl beta-glucosideIBA 06/2021 - 02/2016
2	GadoliniumIBA 02/2021 - 12/2010
2	PyridoxalIBA 01/2021 - 01/2019
2	diphosphoric acid (pyrophosphoric acid)IBA 01/2021 - 01/2019
2	Propranolol (Inderal)FDA LinkGeneric 01/2020 - 02/2016
2	Creatine Kinase (Creatine Phosphokinase)IBA 01/2020 - 02/2016
2	Vitamin B 6IBA 01/2019 - 12/2016
2	Carnitine (L-Carnitine)FDA LinkGeneric 01/2018 - 08/2003
2	Sirolimus (Rapamycin)FDA Link 12/2017 - 06/2014
2	DNA (Deoxyribonucleic Acid)IBA 07/2017 - 01/2016
2	1,4-alpha-Glucan Branching Enzyme (Glycogen Branching Enzyme)IBA 01/2017 - 01/2016

Therapy/Procedure

86	Enzyme Replacement Therapy 09/2022 - 08/2005
34	Therapeutics 01/2022 - 05/2006
6	Mechanical Ventilators (Ventilator) 07/2019 - 05/2006
4	Breathing Exercises 11/2020 - 01/2014
4	Enzyme Therapy 01/2017 - 11/2007
3	Physical Therapy Modalities (Physical Therapy Technique) 01/2021 - 02/2012
3	Immunomodulation 10/2020 - 04/2012
3	Liver Transplantation 01/2019 - 11/2007
2	Diet Therapy (Therapy, Diet) 12/2021 - 11/2007
2	Immunotherapy 01/2021 - 01/2019
2	Intravenous Injections 09/2020 - 03/2019