Niemann-Pick Disease, Type B
"Niemann-Pick Disease, Type B" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus,
MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure,
which enables searching at various levels of specificity.
An allelic disorder of TYPE A NIEMANN-PICK DISEASE, a late-onset form. It is also caused by mutation in SPHINGOMYELIN PHOSPHODIESTERASE but clinical signs involve only visceral organs (non-neuropathic type).
MeSH Number(s)
C10.228.140.163.100.435.825.700.750
C15.604.250.410.625.750
C16.320.565.189.435.825.700.750
C16.320.565.398.641.803.730.750
C16.320.565.595.554.825.700.750
C18.452.132.100.435.825.700.750
C18.452.584.687.803.730.750
C18.452.648.189.435.825.700.750
C18.452.648.398.641.803.730.750
C18.452.648.595.554.825.700.750
Concept/Terms
Niemann-Pick Disease, Type B- Niemann-Pick Disease, Type B
- Niemann Pick Disease, Type B
- Niemann-Pick Disease, Visceral
- Niemann Pick Disease, Visceral
- Type B Niemann-Pick Disease
- Type B Niemann Pick Disease
- Niemann-Pick Disease, Non-Neuronopathic Type
- Niemann Pick Disease, Non Neuronopathic Type
- Niemann-Pick's Disease Type B
- Niemann Pick's Disease Type B
Niemann-Pick Disease, Type E- Niemann-Pick Disease, Type E
- Niemann Pick Disease, Type E
- Niemann-Pick's Disease Type E
- Niemann Pick's Disease Type E
- Niemann-Pick Disease, Adult Non-Neuronopathic
- Niemann Pick Disease, Adult Non Neuronopathic
Below are MeSH descriptors whose meaning is more general than "Niemann-Pick Disease, Type B".
- Diseases [C]
- Nervous System Diseases [C10]
- Central Nervous System Diseases [C10.228]
- Brain Diseases [C10.228.140]
- Brain Diseases, Metabolic [C10.228.140.163]
- Brain Diseases, Metabolic, Inborn [C10.228.140.163.100]
- Lysosomal Storage Diseases, Nervous System [C10.228.140.163.100.435]
- Sphingolipidoses [C10.228.140.163.100.435.825]
- Niemann-Pick Diseases [C10.228.140.163.100.435.825.700]
- Niemann-Pick Disease, Type B [C10.228.140.163.100.435.825.700.750]
- Hemic and Lymphatic Diseases [C15]
- Lymphatic Diseases [C15.604]
- Histiocytosis [C15.604.250]
- Histiocytosis, Non-Langerhans-Cell [C15.604.250.410]
- Niemann-Pick Diseases [C15.604.250.410.625]
- Niemann-Pick Disease, Type B [C15.604.250.410.625.750]
- Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]
- Genetic Diseases, Inborn [C16.320]
- Metabolism, Inborn Errors [C16.320.565]
- Brain Diseases, Metabolic, Inborn [C16.320.565.189]
- Lysosomal Storage Diseases, Nervous System [C16.320.565.189.435]
- Sphingolipidoses [C16.320.565.189.435.825]
- Niemann-Pick Diseases [C16.320.565.189.435.825.700]
- Niemann-Pick Disease, Type B [C16.320.565.189.435.825.700.750]
- Lipid Metabolism, Inborn Errors [C16.320.565.398]
- Lipidoses [C16.320.565.398.641]
- Sphingolipidoses [C16.320.565.398.641.803]
- Niemann-Pick Diseases [C16.320.565.398.641.803.730]
- Niemann-Pick Disease, Type B [C16.320.565.398.641.803.730.750]
- Lysosomal Storage Diseases [C16.320.565.595]
- Lysosomal Storage Diseases, Nervous System [C16.320.565.595.554]
- Sphingolipidoses [C16.320.565.595.554.825]
- Niemann-Pick Diseases [C16.320.565.595.554.825.700]
- Niemann-Pick Disease, Type B [C16.320.565.595.554.825.700.750]
- Nutritional and Metabolic Diseases [C18]
- Metabolic Diseases [C18.452]
- Brain Diseases, Metabolic [C18.452.132]
- Brain Diseases, Metabolic, Inborn [C18.452.132.100]
- Lysosomal Storage Diseases, Nervous System [C18.452.132.100.435]
- Sphingolipidoses [C18.452.132.100.435.825]
- Niemann-Pick Diseases [C18.452.132.100.435.825.700]
- Niemann-Pick Disease, Type B [C18.452.132.100.435.825.700.750]
- Lipid Metabolism Disorders [C18.452.584]
- Lipidoses [C18.452.584.687]
- Sphingolipidoses [C18.452.584.687.803]
- Niemann-Pick Diseases [C18.452.584.687.803.730]
- Niemann-Pick Disease, Type B [C18.452.584.687.803.730.750]
- Metabolism, Inborn Errors [C18.452.648]
- Brain Diseases, Metabolic, Inborn [C18.452.648.189]
- Lysosomal Storage Diseases, Nervous System [C18.452.648.189.435]
- Sphingolipidoses [C18.452.648.189.435.825]
- Niemann-Pick Diseases [C18.452.648.189.435.825.700]
- Niemann-Pick Disease, Type B [C18.452.648.189.435.825.700.750]
- Lipid Metabolism, Inborn Errors [C18.452.648.398]
- Lipidoses [C18.452.648.398.641]
- Sphingolipidoses [C18.452.648.398.641.803]
- Niemann-Pick Diseases [C18.452.648.398.641.803.730]
- Niemann-Pick Disease, Type B [C18.452.648.398.641.803.730.750]
- Lysosomal Storage Diseases [C18.452.648.595]
- Lysosomal Storage Diseases, Nervous System [C18.452.648.595.554]
- Sphingolipidoses [C18.452.648.595.554.825]
- Niemann-Pick Diseases [C18.452.648.595.554.825.700]
- Niemann-Pick Disease, Type B [C18.452.648.595.554.825.700.750]
Below are MeSH descriptors whose meaning is more specific than "Niemann-Pick Disease, Type B".
This graph shows the total number of publications written about "Niemann-Pick Disease, Type B" by people in Harvard Catalyst Profiles by year, and whether "Niemann-Pick Disease, Type B" was a major or minor topic of these publication.
To see the data from this visualization as text,
click here.
| Year | Major Topic | Minor Topic | Total |
|---|
| 2008 | 1 | 0 | 1 |
| 2012 | 1 | 0 | 1 |
| 2013 | 1 | 0 | 1 |
| 2014 | 1 | 0 | 1 |
| 2015 | 1 | 0 | 1 |
| 2016 | 2 | 0 | 2 |
Below are the most recent publications written about "Niemann-Pick Disease, Type B" by people in Profiles.
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Clearance of Hepatic Sphingomyelin by Olipudase Alfa Is Associated With Improvement in Lipid Profiles in Acid Sphingomyelinase Deficiency. Am J Surg Pathol. 2016 09; 40(9):1232-42.
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Cause of death in patients with chronic visceral and chronic neurovisceral acid sphingomyelinase deficiency (Niemann-Pick disease type B and B variant): Literature review and report of new cases. Mol Genet Metab. 2016 07; 118(3):206-213.
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SMPD1 Mutation Update: Database and Comprehensive Analysis of Published and Novel Variants. Hum Mutat. 2016 Feb; 37(2):139-47.
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Novel first-dose adverse drug reactions during a phase I trial of olipudase alfa (recombinant human acid sphingomyelinase) in adults with Niemann-Pick disease type B (acid sphingomyelinase deficiency). Genet Med. 2016 Jan; 18(1):34-40.
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A novel missense SMPD1 gene mutation, T460P, and clinical findings in a patient with Niemann-Pick disease type B presenting to a lipid disorders clinic. Ann Clin Biochem. 2014 Sep; 51(Pt 5):615-8.
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Lyso-sphingomyelin is elevated in dried blood spots of Niemann-Pick B patients. Mol Genet Metab. 2014 Feb; 111(2):209-11.
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Liver and skin histopathology in adults with acid sphingomyelinase deficiency (Niemann-Pick disease type B). Am J Surg Pathol. 2012 Aug; 36(8):1234-46.
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A prospective, cross-sectional survey study of the natural history of Niemann-Pick disease type B. Pediatrics. 2008 Aug; 122(2):e341-9.