Page last updated: 2024-08-23

1-deoxynojirimycin and Enlarged Spleen

1-deoxynojirimycin has been researched along with Enlarged Spleen in 13 studies

Research

Studies (13)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's5 (38.46)29.6817
2010's8 (61.54)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Hughes, DA; Mehta, A; Thomas, AS1
Bembi, B; Cox, TM; Deegan, P; Deroma, L; Hollak, C; Shemesh, E; Weinreb, NJ1
Degtyareva, AV; Mikhailova, SV; Puchkova, AA; Tumanova, EL; Zakharova, EY1
Chérin, P; Giraldo, P; Mehta, A; Pastores, GM1
Sedel, F1
Lo, SM; McNamara, J; Mistry, PK; Seashore, MR1
Czartoryska, B; Groener, JE; Jurkiewicz, E; Kamiński, ML; Tylki-Szymańska, A; Ługowska, A1
McKiernan, P1
Klünemann, HH; Santosh, PJ; Sedel, F1
Stirnemann, J1
Germain, DP; Mistry, P1
Chippington, S; McHugh, K; Vellodi, A1
Mistry, PK1

Reviews

5 review(s) available for 1-deoxynojirimycin and Enlarged Spleen

ArticleYear
Gaucher disease: haematological presentations and complications.
    British journal of haematology, 2014, Volume: 165, Issue:4

    Topics: 1-Deoxynojirimycin; Anemia; Combined Modality Therapy; Disease Management; Enzyme Replacement Therapy; Gaucher Disease; Genetic Predisposition to Disease; Glucosylceramidase; Glycosphingolipids; Hemorrhagic Disorders; Humans; Inflammation; Lewy Body Disease; Lysosomes; Macrophage Activation; Multiple Myeloma; Parkinson Disease; Splenectomy; Splenomegaly; Thrombocytopenia; Unfolded Protein Response

2014
Enzyme replacement and substrate reduction therapy for Gaucher disease.
    The Cochrane database of systematic reviews, 2015, Mar-27, Issue:3

    Topics: 1-Deoxynojirimycin; Enzyme Inhibitors; Enzyme Replacement Therapy; Gaucher Disease; Glucosylceramidase; Hemoglobin A; Hepatomegaly; Humans; Platelet Count; Randomized Controlled Trials as Topic; Splenomegaly; Substrate Specificity

2015
Goal-oriented therapy with miglustat in Gaucher disease.
    Current medical research and opinion, 2009, Volume: 25, Issue:1

    Topics: 1-Deoxynojirimycin; Adult; Aged; Bone and Bones; Female; Gaucher Disease; Hepatomegaly; Humans; Lung; Male; Middle Aged; Splenomegaly

2009
[Clinical diagnosis of the adult form of Niemann-Pick type C disease].
    Archives de pediatrie : organe officiel de la Societe francaise de pediatrie, 2010, Volume: 17 Suppl 2

    Topics: 1-Deoxynojirimycin; Adult; Cerebellar Ataxia; Cognition Disorders; Deglutition Disorders; Dysarthria; Enzyme Inhibitors; Epilepsy; Humans; Mental Disorders; Movement Disorders; Niemann-Pick Disease, Type C; Splenomegaly; Supranuclear Palsy, Progressive

2010
Treatable metabolic psychoses that go undetected: what Niemann-Pick type C can teach us.
    International journal of psychiatry in clinical practice, 2012, Volume: 16, Issue:3

    Topics: 1-Deoxynojirimycin; Age of Onset; Ataxia; Diagnosis, Differential; Disease Progression; Enzyme Inhibitors; Genetic Testing; Humans; Magnetic Resonance Spectroscopy; Niemann-Pick Disease, Type C; Ophthalmoplegia; Prevalence; Psychotic Disorders; Splenomegaly; Treatment Outcome

2012

Trials

1 trial(s) available for 1-deoxynojirimycin and Enlarged Spleen

ArticleYear
[Clinical study of the French cohort of Gaucher disease patients].
    La Revue de medecine interne, 2006, Volume: 27 Suppl 1

    Topics: 1-Deoxynojirimycin; Adolescent; Adult; Aged; Aged, 80 and over; Asthenia; Biomarkers; Child; Child, Preschool; Cohort Studies; Enzyme Inhibitors; Follow-Up Studies; Forecasting; France; Gaucher Disease; Glucosylceramidase; Humans; Infant; Infant, Newborn; Middle Aged; Recombinant Proteins; Splenectomy; Splenomegaly; Time Factors; Treatment Outcome

2006

Other Studies

7 other study(ies) available for 1-deoxynojirimycin and Enlarged Spleen

ArticleYear
Visceral symptoms as a key diagnostic sign for the early infantile form of Niemann-Pick disease type C in a Russian patient: a case report.
    Journal of medical case reports, 2016, Jun-01, Volume: 10, Issue:1

    Topics: 1-Deoxynojirimycin; Child, Preschool; Cholestasis, Intrahepatic; Diagnostic Errors; Galactosemias; Genetic Testing; Glycoside Hydrolase Inhibitors; Hepatomegaly; Humans; Infant; Infant, Newborn; Jaundice, Neonatal; Liver; Male; Niemann-Pick Disease, Type C; Russia; Splenomegaly

2016
Misdiagnosis of Niemann-Pick disease type C as Gaucher disease.
    Journal of inherited metabolic disease, 2010, Volume: 33 Suppl 3

    Topics: 1-Deoxynojirimycin; Acid Phosphatase; Biomarkers; Carrier Proteins; Cells, Cultured; Cholesterol Esters; Diagnostic Errors; DNA Mutational Analysis; Enzyme Inhibitors; Enzyme Replacement Therapy; Esterification; Female; Gaucher Disease; Genetic Predisposition to Disease; Glucosylceramidase; Glucosyltransferases; Hepatomegaly; Heterozygote; Hexosaminidases; Humans; Infant; Intracellular Signaling Peptides and Proteins; Isoenzymes; Membrane Glycoproteins; Mutation; Niemann-Pick C1 Protein; Niemann-Pick Disease, Type C; Phenotype; Predictive Value of Tests; Splenomegaly; Tartrate-Resistant Acid Phosphatase; Unnecessary Procedures

2010
Gaucher disease due to saposin C deficiency, previously described as non-neuronopathic form--no positive effects after 2-years of miglustat therapy.
    Molecular genetics and metabolism, 2011, Volume: 104, Issue:4

    Topics: 1-Deoxynojirimycin; Adult; Diagnostic Errors; Enzyme Inhibitors; Female; Gaucher Disease; Hepatomegaly; Humans; Male; Saposins; Splenomegaly; Treatment Failure

2011
Metabolic liver disease.
    Clinics and research in hepatology and gastroenterology, 2012, Volume: 36, Issue:3

    Topics: 1-Deoxynojirimycin; Cyclohexanones; Diet; Enzyme Inhibitors; Enzyme Replacement Therapy; Hepatocytes; Hepatomegaly; Humans; Hydrops Fetalis; Infant; Infant, Newborn; Jaundice, Neonatal; Liver Diseases; Liver Transplantation; Metabolism, Inborn Errors; Nitrobenzoates; Splenomegaly

2012
Therapeutic goals in Gaucher disease.
    La Revue de medecine interne, 2006, Volume: 27 Suppl 1

    Topics: 1-Deoxynojirimycin; Adolescent; Adult; Age Factors; Child; Evidence-Based Medicine; Gaucher Disease; Glucosylceramidase; Glycoside Hydrolase Inhibitors; Humans; Injections, Intravenous; Phenotype; Quality of Life; Recombinant Proteins; Splenectomy; Splenomegaly; Time Factors

2006
Splenic nodules in paediatric Gaucher disease treated by enzyme replacement therapy.
    Pediatric radiology, 2008, Volume: 38, Issue:6

    Topics: 1-Deoxynojirimycin; Child; Child, Preschool; Cohort Studies; Enzyme Inhibitors; Female; Follow-Up Studies; Gaucher Disease; Glucosylceramidase; Humans; Infant; Male; Retrospective Studies; Spleen; Splenic Diseases; Splenomegaly; Ultrasonography

2008
Treatment of Gaucher's disease with OGT 918.
    Lancet (London, England), 2000, Aug-19, Volume: 356, Issue:9230

    Topics: 1-Deoxynojirimycin; Enzyme Inhibitors; Gaucher Disease; Glucosylceramidase; Glucosylceramides; Glycoside Hydrolase Inhibitors; Hepatomegaly; Hexosaminidases; Humans; Splenomegaly; Time

2000