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Type A Niemann-Pick Disease Summary

Description: The classic infantile form of Niemann-Pick Disease, caused by mutation in SPHINGOMYELIN PHOSPHODIESTERASE. It is characterized by accumulation of SPHINGOMYELINS in the cells of the MONONUCLEAR PHAGOCYTE SYSTEM and other cell throughout the body leading to cell death. Clinical signs include JAUNDICE, hepatosplenomegaly, and severe brain damage.

Also Known As: Niemann-Pick Disease, Type A; Classical Niemann-Pick Disease; Niemann-Pick Disease, Acute Neuronopathic Form; Niemann-Pick Disease, Acute Neurovisceral Form; Niemann-Pick Disease, Neuronopathic Type Show All >>

Networked: 61 relevant articles (0 outcomes, 5 trials/studies) for this Disease

Key Drugs and Agents for Type A Niemann-Pick Disease

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. Sphingomyelin Phosphodiesterase (Sphingomyelinase) : 2 studies in 40 results : IBA
  2. NF-kappa B (NF-kB) : 1 study in 1 result : IBA
  3. Sphingomyelin lipidosis : 5 results : IBA
  4. neodymium pyrocatechin disulfonate (NPD) : 5 results : IBA
  5. DNA (Deoxyribonucleic Acid) : 3 results : IBA
  6. Sphingolipids : 2 results : IBA
  7. LDL Receptors (LDL Receptor) : 2 results : IBA
  8. chitotriosidase : 2 results : IBA
  9. Phospholipases A2 (Phospholipase A2) : 1 result : IBA
  10. Transcription Factor AP-1 (Transcription Factor AP 1) : 1 result : IBA
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Diseases Related to Type A Niemann-Pick Disease

  1. Type C Niemann-Pick Disease (Niemann Pick Disease, Type C)
  2. Lysosomal Storage Diseases (Lysosomal Storage Disease)
  3. Type B Niemann-Pick Disease
  4. Type A Niemann-Pick Disease
  5. Niemann-Pick Diseases (Niemann Pick Disease)
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Key Therapies for Type A Niemann-Pick Disease

Efficacy Chart >>
  1. Transplantation (Transplant Recipients) : 1 study in 2 results
  2. Induced Abortion (Induced Abortions) : 1 study in 1 result
  3. Cord Blood Stem Cell Transplantation : 1 result
  4. Hematopoietic Stem Cell Transplantation : 1 result
  5. Organ Transplantation : 1 result
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