Sea-Blue Histiocyte Syndrome [syndrom histiocytů barvy mořské modři]

topical
4
Terms

Histiocyte Syndrome, Sea Blue
Histiocyte Syndromes, Sea Blue
Histiocytosis, Sea-Blue
Sea Blue Histiocytosis
Sea-Blue Histiocyte Disease
Sea-Blue Histiocytosis

Persistent link   https://www.medvik.cz/link/D012618
Definition

A congenital disease caused by an inborn error involving APOLIPOPROTEINS E leading to abnormal LIPID METABOLISM and the accumulation of GLYCOSPHINGOLIPIDS, particularly SPHINGOMYELINS in the HISTIOCYTES. This disorder is characterized by SPLENOMEGALY and the sea-blue histiocytes in the spleen and bone marrow after May Grunwald staining.

DUI
D012618 MeSH Browser
CUI
M0019573
Previous indexing
Histiocytes (1966-1983)
History note
1984
Public note
1984

C Diseases
C10.228.140 Brain Diseases 1 178
C10.228.140.163 Brain Diseases, Metabolic 84
C10.228.140.163.100.435.825 Sphingolipidoses 12
C10.228.140.163.100.435.825.200 Fabry Disease 187
C10.228.140.163.100.435.825.250 Farber Lipogranulomatosis 5
C10.228.140.163.100.435.825.300 Gangliosidoses 11
C10.228.140.163.100.435.825.400 Gaucher Disease 83
C10.228.140.163.100.435.825.590 Leukodystrophy, Globoid Cell 16
C10.228.140.163.100.435.825.700 Niemann-Pick Diseases 36
C10.228.140.163.100.435.825.775 Sea-Blue Histiocyte Syndrome 4
C10.228.140.163.100.435.825.850 Sulfatidosis 1
C15.604.250 Histiocytosis 67
C15.604.250.410.224 Erdheim-Chester Disease 39
C15.604.250.410.450 Histiocytosis, Sinus 21
C15.604.250.410.625 Niemann-Pick Diseases 36
C15.604.250.410.800 Sea-Blue Histiocyte Syndrome 4
C15.604.250.410.900 Xanthogranuloma, Juvenile 25
C16.320.565.189.435.825 Sphingolipidoses 12
C16.320.565.189.435.825.200 Fabry Disease 187
C16.320.565.189.435.825.250 Farber Lipogranulomatosis 5
C16.320.565.189.435.825.300 Gangliosidoses 11
C16.320.565.189.435.825.400 Gaucher Disease 83
C16.320.565.189.435.825.590 Leukodystrophy, Globoid Cell 16
C16.320.565.189.435.825.700 Niemann-Pick Diseases 36
C16.320.565.189.435.825.775 Sea-Blue Histiocyte Syndrome 4
C16.320.565.189.435.825.850 Sulfatidosis 1
C16.320.565.398.641 Lipidoses 35
C16.320.565.398.641.803 Sphingolipidoses 12
C16.320.565.398.641.803.300 Fabry Disease 187
C16.320.565.398.641.803.325 Farber Lipogranulomatosis 5
C16.320.565.398.641.803.350 Gangliosidoses 11
C16.320.565.398.641.803.441 Gaucher Disease 83
C16.320.565.398.641.803.585 Leukodystrophy, Globoid Cell 16
C16.320.565.398.641.803.730 Niemann-Pick Diseases 36
C16.320.565.398.641.803.850 Sea-Blue Histiocyte Syndrome 4
C16.320.565.398.641.803.925 Sulfatidosis 1
C16.320.565.595 Lysosomal Storage Diseases 72
C16.320.565.595.554.825 Sphingolipidoses 12
C16.320.565.595.554.825.200 Fabry Disease 187
C16.320.565.595.554.825.250 Farber Lipogranulomatosis 5
C16.320.565.595.554.825.300 Gangliosidoses 11
C16.320.565.595.554.825.400 Gaucher Disease 83
C16.320.565.595.554.825.590 Leukodystrophy, Globoid Cell 16
C16.320.565.595.554.825.700 Niemann-Pick Diseases 36
C16.320.565.595.554.825.775 Sea-Blue Histiocyte Syndrome 4
C16.320.565.595.554.825.850 Sulfatidosis 1
C18.452 Metabolic Diseases 1 196
C18.452.132.100.435.825 Sphingolipidoses 12
C18.452.132.100.435.825.200 Fabry Disease 187
C18.452.132.100.435.825.250 Farber Lipogranulomatosis 5
C18.452.132.100.435.825.300 Gangliosidoses 11
C18.452.132.100.435.825.400 Gaucher Disease 83
C18.452.132.100.435.825.590 Leukodystrophy, Globoid Cell 16
C18.452.132.100.435.825.700 Niemann-Pick Diseases 36
C18.452.132.100.435.825.775 Sea-Blue Histiocyte Syndrome 4
C18.452.132.100.435.825.850 Sulfatidosis 1
C18.452.584.563.641 Lipidoses 35
C18.452.584.563.641.803 Sphingolipidoses 12
C18.452.584.563.641.803.300 Fabry Disease 187
C18.452.584.563.641.803.325 Farber Lipogranulomatosis 5
C18.452.584.563.641.803.350 Gangliosidoses 11
C18.452.584.563.641.803.441 Gaucher Disease 83
C18.452.584.563.641.803.585 Leukodystrophy, Globoid Cell 16
C18.452.584.563.641.803.730 Niemann-Pick Diseases 36
C18.452.584.563.641.803.850 Sea-Blue Histiocyte Syndrome 4
C18.452.584.563.641.803.925 Sulfatidosis 1
C18.452.648.189.435.825 Sphingolipidoses 12
C18.452.648.189.435.825.200 Fabry Disease 187
C18.452.648.189.435.825.250 Farber Lipogranulomatosis 5
C18.452.648.189.435.825.300 Gangliosidoses 11
C18.452.648.189.435.825.400 Gaucher Disease 83
C18.452.648.189.435.825.590 Leukodystrophy, Globoid Cell 16
C18.452.648.189.435.825.700 Niemann-Pick Diseases 36
C18.452.648.189.435.825.775 Sea-Blue Histiocyte Syndrome 4
C18.452.648.189.435.825.850 Sulfatidosis 1
C18.452.648.398.641 Lipidoses 35
C18.452.648.398.641.803 Sphingolipidoses 12
C18.452.648.398.641.803.300 Fabry Disease 187
C18.452.648.398.641.803.325 Farber Lipogranulomatosis 5
C18.452.648.398.641.803.350 Gangliosidoses 11
C18.452.648.398.641.803.441 Gaucher Disease 83
C18.452.648.398.641.803.585 Leukodystrophy, Globoid Cell 16
C18.452.648.398.641.803.730 Niemann-Pick Diseases 36
C18.452.648.398.641.803.850 Sea-Blue Histiocyte Syndrome 4
C18.452.648.398.641.803.925 Sulfatidosis 1
C18.452.648.595 Lysosomal Storage Diseases 72
C18.452.648.595.554.825 Sphingolipidoses 12
C18.452.648.595.554.825.200 Fabry Disease 187
C18.452.648.595.554.825.250 Farber Lipogranulomatosis 5
C18.452.648.595.554.825.300 Gangliosidoses 11
C18.452.648.595.554.825.400 Gaucher Disease 83
C18.452.648.595.554.825.590 Leukodystrophy, Globoid Cell 16
C18.452.648.595.554.825.700 Niemann-Pick Diseases 36
C18.452.648.595.554.825.775 Sea-Blue Histiocyte Syndrome 4
C18.452.648.595.554.825.850 Sulfatidosis 1