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Type B Niemann-Pick Disease

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).
Also Known As:
Niemann-Pick Disease, Type B; Niemann-Pick Disease, Adult Non-Neuronopathic; Niemann-Pick Disease, Non-Neuronopathic Type; Niemann-Pick Disease, Type E; Niemann-Pick Disease, Visceral; Niemann-Pick's Disease Type B; Niemann-Pick's Disease Type E; Niemann Pick Disease, Adult Non Neuronopathic; Niemann Pick Disease, Non Neuronopathic Type; Niemann Pick Disease, Type B; Niemann Pick Disease, Type E; Niemann Pick Disease, Visceral; Niemann Pick's Disease Type B; Niemann Pick's Disease Type E; Type B Niemann Pick Disease
Networked: 52 relevant articles (1 outcomes, 3 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Mucolipidoses (Sialidosis)
2. GM2 Gangliosidoses (GM2 Gangliosidosis)
3. Mucopolysaccharidoses
4. Mannosidase Deficiency Diseases (Mannosidosis)
5. Metachromatic Leukodystrophy (Sulfatide Lipidosis)

Experts

1. McGovern, Margaret M: 7 articles (02/2014 - 12/2002)
2. Schuchman, Edward H: 6 articles (01/2013 - 12/2002)
3. Wasserstein, Melissa P: 6 articles (08/2012 - 12/2002)
4. Desnick, Robert J: 5 articles (08/2008 - 12/2002)
5. Genest, Jacques: 4 articles (03/2013 - 06/2005)
6. Lee, Ching Yin: 4 articles (03/2013 - 06/2005)
7. Cox, Gerald F: 3 articles (02/2014 - 08/2008)
8. Ruel, Isabelle: 2 articles (03/2013 - 12/2007)
9. Denis, Maxime: 2 articles (03/2013 - 03/2006)
10. Groener, J E: 2 articles (11/2012 - 01/2005)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Type B Niemann-Pick Disease:
1. CholesterolIBA
2. PhosphotyrosineIBA
3. Sphingomyelin Phosphodiesterase (Sphingomyelinase)IBA
4. CeroidIBA
01/01/1990 - "The data suggest that mucopolysaccharidosis (MPS) type IVA (Morquio disease), multiple sulphatase deficiency, Niemann-Pick disease type B, GM2 gangliosidosis type '0' (Sandhoff disease), and ceroid lipofuscinosis (Jansky-Bielschowsky and Batten-Spielmeyer-Vogt syndromes) are encountered frequently in Saudi Arabia, as compared to other storage diseases. "
01/01/1979 - "The diseases were Tay-Sachs disease, ceroid-lipofuscinosis (Jansky-Bielschowsky type), Niemann-Pick disease (type B), highly suspected adrenoleukodystrophy, I-cell disease, mucolipidosis of the beta-galactosidase deficient type, Hurler disease, Hunter disease and Morquio disease. "
10/01/2007 - "Lysosomal diseases were classified into 3 groups: (1) leukodystrophies (metachromatic leukodystrophy, Krabbe's disease and Salla's disease); (2) Neurodegenerative or psychiatric-like diseases (GM1 and GM2 gangliosidoses, Niemann Pick type C disease, sialidosis type I, ceroid-lipofuscinosis, mucopolysaccharidosis type III); (3) multisystemic diseases (Gaucher's disease, Fabry's disease, alpha and B mannosidosis, Niemann Pick disease type B, fucosidosis, Schindler/Kanzaki disease, and mucopolysaccharidosis type I and II. We propose a diagnostic approach guided by clinical examination, brain MRI, electrodiagnostic studies and abdominal echography."
08/01/2006 - "Autopsy findings of hepatosplenomegaly, lymphadenopathy and ceroid-rich foam cells raised the suspicion of the visceral form of acid sphingomyelinase deficiency (Niemann-Pick disease type B; NPB) or a much rarer disorder, variant adult visceral form of Niemann-Pick disease type C (NPC). "
5. Kanzaki diseaseIBA
6. neodymium pyrocatechin disulfonate (NPD)IBA
7. Sphingomyelin lipidosisIBA
8. 4-nitrophenethyl bromide (NPB)IBA
9. HDL CholesterolIBA
10. Lipid AIBA

Therapies and Procedures

1. Enzyme Replacement Therapy
2. Transplants (Transplant)
3. Hematopoietic Stem Cell Transplantation
4. Bone Marrow Transplantation (Transplantation, Bone Marrow)
5. Coronary Artery Bypass (Coronary Artery Bypass Surgery)