1 / 1 DeCS    
Descriptor English:   Hyperlipoproteinemia Type I 
Descriptor Spanish:   Hiperlipoproteinemia Tipo I 
Descriptor Portuguese:   Hiperlipoproteinemia Tipo I 
Synonyms English:   Anapolipoproteinemia, C-II
Anapolipoproteinemias, C-II
Apolipoprotein C II Deficiency
Apolipoprotein C-II Deficiencies
Apolipoprotein C-II Deficiency
Burger Grutz Syndrome
Burger-Grutz Syndrome
Burger-Grutz Syndromes
C-II Anapolipoproteinemia
C-II Anapolipoproteinemias
Chylomicronemia, Familial
Chylomicronemias, Familial
Deficiencies, Apolipoprotein C-II
Deficiencies, Familial LPL
Deficiencies, LIPD
Deficiencies, Lipase D
Deficiencies, Lipoprotein Lipase
Deficiency, Apolipoprotein C-II
Deficiency, Familial LPL
Deficiency, LIPD
Deficiency, Lipase D
Deficiency, Lipoprotein Lipase
Essential Familial Hyperlipemia
Essential Familial Hyperlipemias
Familial Chylomicronemia
Familial Chylomicronemias
Familial Fat Induced Hypertriglyceridemia
Familial Fat-Induced Hypertriglyceridemia
Familial Fat-Induced Hypertriglyceridemias
Familial Hyperchylomicronemia
Familial Hyperchylomicronemias
Familial Hyperlipemia, Essential
Familial Hyperlipemias, Essential
Familial Hyperlipoproteinemia Type 1
Familial LPL Deficiencies
Familial LPL Deficiency
Familial Lipoprotein Lipase Deficiency
Fat-Induced Hypertriglyceridemia, Familial
Fat-Induced Hypertriglyceridemias, Familial
Hyperchylomicronemia, Familial
Hyperchylomicronemias, Familial
Hyperlipemia, Essential Familial
Hyperlipemia, Idiopathic, Burger-Grutz Type
Hyperlipemias, Essential Familial
Hyperlipoproteinemia Type Ia
Hyperlipoproteinemia Type Ias
Hyperlipoproteinemia Type Ib
Hyperlipoproteinemia Type Ibs
Hyperlipoproteinemia Type Is
Hyperlipoproteinemia, Type I
Hyperlipoproteinemia, Type Ia
Hyperlipoproteinemia, Type Ib
Hyperlipoproteinemias, Type I
Hyperlipoproteinemias, Type Ia
Hyperlipoproteinemias, Type Ib
Hypertriglyceridemia, Familial Fat-Induced
Hypertriglyceridemias, Familial Fat-Induced
LIPD Deficiencies
LIPD Deficiency
LPL Deficiencies, Familial
LPL Deficiency, Familial
Lipase D Deficiencies
Lipase D Deficiency
Lipase Deficiencies, Lipoprotein
Lipoprotein Lipase Deficiencies
Lipoprotein Lipase Deficiency
Lipoprotein Lipase Deficiency, Familial
Syndrome, Burger-Grutz
Syndromes, Burger-Grutz
Type I Hyperlipoproteinemia
Type I Hyperlipoproteinemias
Type Ia Hyperlipoproteinemia
Type Ia Hyperlipoproteinemias
Type Ib Hyperlipoproteinemia
Type Ib Hyperlipoproteinemias  
Tree Number:   C16.320.565.398.465
C18.452.584.500.500.644.237
C18.452.648.398.465
Definition English:   An inherited condition due to a deficiency of either LIPOPROTEIN LIPASE or APOLIPOPROTEIN C-II (a lipase-activating protein). The lack of lipase activities results in inability to remove CHYLOMICRONS and TRIGLYCERIDES from the blood which has a creamy top layer after standing. 
See Related English:   Apolipoprotein C-II
Lipoprotein Lipase
 
History Note English:   2007 (1980) 
Allowable Qualifiers English:  
BL blood CF cerebrospinal fluid
CI chemically induced CL classification
CO complications DI diagnosis
DG diagnostic imaging DH diet therapy
DT drug therapy EC economics
EM embryology EN enzymology
EP epidemiology EH ethnology
ET etiology GE genetics
HI history IM immunology
ME metabolism MI microbiology
MO mortality NU nursing
PS parasitology PA pathology
PP physiopathology PC prevention & control
PX psychology RT radiotherapy
RH rehabilitation SU surgery
TH therapy UR urine
VE veterinary VI virology
Record Number:   8246 
Unique Identifier:   D008072 


   DISEASES [C]
      Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]
         Genetic Diseases, Inborn [C16.320]
            Metabolism, Inborn Errors [C16.320.565]
               Amino Acid Metabolism, Inborn Errors [C16.320.565.100] +
               Amino Acid Transport Disorders, Inborn [C16.320.565.151] +
               Amyloidosis, Familial [C16.320.565.176] +
               Brain Diseases, Metabolic, Inborn [C16.320.565.189] +
               Carbohydrate Metabolism, Inborn Errors [C16.320.565.202] +
               Cytochrome-c Oxidase Deficiency [C16.320.565.240]
               Hyperbilirubinemia, Hereditary [C16.320.565.300] +
               Lipid Metabolism, Inborn Errors [C16.320.565.398]
                  Barth Syndrome [C16.320.565.398.224]
                  Hyperlipidemia, Familial Combined [C16.320.565.398.450]
                  Hyperlipoproteinemia Type I [C16.320.565.398.465] 
                  Hyperlipoproteinemia Type II [C16.320.565.398.481]
                  Hyperlipoproteinemia Type III [C16.320.565.398.483]
                  Hyperlipoproteinemia Type IV [C16.320.565.398.487]
                  Hyperlipoproteinemia Type V [C16.320.565.398.493]
                  Hypolipoproteinemias [C16.320.565.398.500] +
                  Lipidoses [C16.320.565.398.641] +
                  Lipodystrophy, Congenital Generalized [C16.320.565.398.745]
                  Smith-Lemli-Opitz Syndrome [C16.320.565.398.850]
                  Xanthomatosis, Cerebrotendinous [C16.320.565.398.925]
               Lysosomal Storage Diseases [C16.320.565.595] +
               Metal Metabolism, Inborn Errors [C16.320.565.618] +
               Peroxisomal Disorders [C16.320.565.663] +
               Progeria [C16.320.565.753]
               Purine-Pyrimidine Metabolism, Inborn Errors [C16.320.565.798] +
               Renal Tubular Transport, Inborn Errors [C16.320.565.893]
               Steroid Metabolism, Inborn Errors [C16.320.565.925] +


   DISEASES [C]
      Nutritional and Metabolic Diseases [C18]
         Metabolic Diseases [C18.452]
            Lipid Metabolism Disorders [C18.452.584]
               Dyslipidemias [C18.452.584.500]
                  Hyperlipidemias [C18.452.584.500.500]
                     Hypercholesterolemia [C18.452.584.500.500.396]
                     Hyperlipidemia, Familial Combined [C18.452.584.500.500.438]
                     Hyperlipoproteinemias [C18.452.584.500.500.644]
                        Hyperlipoproteinemia Type I [C18.452.584.500.500.644.237] 
                        Hyperlipoproteinemia Type II [C18.452.584.500.500.644.475]
                        Hyperlipoproteinemia Type III [C18.452.584.500.500.644.485]
                        Hyperlipoproteinemia Type IV [C18.452.584.500.500.644.490]
                        Hyperlipoproteinemia Type V [C18.452.584.500.500.644.495]
                     Hypertriglyceridemia [C18.452.584.500.500.851] +


   DISEASES [C]
      Nutritional and Metabolic Diseases [C18]
         Metabolic Diseases [C18.452]
            Metabolism, Inborn Errors [C18.452.648]
               Amino Acid Metabolism, Inborn Errors [C18.452.648.100] +
               Amino Acid Transport Disorders, Inborn [C18.452.648.151] +
               Amyloidosis, Familial [C18.452.648.176] +
               Brain Diseases, Metabolic, Inborn [C18.452.648.189] +
               Carbohydrate Metabolism, Inborn Errors [C18.452.648.202] +
               Hyperbilirubinemia, Hereditary [C18.452.648.300] +
               Lipid Metabolism, Inborn Errors [C18.452.648.398]
                  Barth Syndrome [C18.452.648.398.224]
                  Hyperlipidemia, Familial Combined [C18.452.648.398.450]
                  Hyperlipoproteinemia Type I [C18.452.648.398.465] 
                  Hyperlipoproteinemia Type II [C18.452.648.398.481]
                  Hyperlipoproteinemia Type III [C18.452.648.398.483]
                  Hyperlipoproteinemia Type IV [C18.452.648.398.487]
                  Hyperlipoproteinemia Type V [C18.452.648.398.493]
                  Hypolipoproteinemias [C18.452.648.398.500] +
                  Lipidoses [C18.452.648.398.641] +
                  Lipodystrophy, Congenital Generalized [C18.452.648.398.745]
                  Smith-Lemli-Opitz Syndrome [C18.452.648.398.850]
                  Xanthomatosis, Cerebrotendinous [C18.452.648.398.925]
               Lysosomal Storage Diseases [C18.452.648.595] +
               Metal Metabolism, Inborn Errors [C18.452.648.618] +
               Peroxisomal Disorders [C18.452.648.663] +
               Progeria [C18.452.648.753]
               Purine-Pyrimidine Metabolism, Inborn Errors [C18.452.648.798] +
               Renal Tubular Transport, Inborn Errors [C18.452.648.893]
               Steroid Metabolism, Inborn Errors [C18.452.648.925] +