Search on: FRUCTOSE INTOLERANCES 
Descriptors Found: 1
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Descriptor English:   Fructose Intolerance 
Descriptor Spanish:   Intolerancia a la Fructosa 
Descriptor Portuguese:   Intolerância à Frutose 
Synonyms English:   ALDOB Deficiencies
ALDOB Deficiency
Aldolase B Deficiencies
Aldolase B Deficiency
Aldolase Deficiencies, Fructose-1,6-Biphosphate
Aldolase Deficiencies, Fructose-1-Phosphate
Aldolase Deficiency, Fructose-1,6-Biphosphate
Aldolase Deficiency, Fructose-1-Phosphate
Deficiencies, ALDOB
Deficiencies, Aldolase B
Deficiencies, Fructose-1,6-Biphosphate Aldolase
Deficiencies, Fructose-1-Phosphate Aldolase
Deficiency, ALDOB
Deficiency, Aldolase B
Deficiency, Fructose-1,6-Biphosphate Aldolase
Deficiency, Fructose-1-Phosphate Aldolase
Fructose 1 Phosphate Aldolase Deficiency
Fructose 1,6 Biphosphate Aldolase Deficiency
Fructose 1,6 Bisphosphate Aldolase B Deficiency
Fructose Aldolase B Deficiency
Fructose Intolerance, Hereditary
Fructose Intolerances
Fructose Intolerances, Hereditary
Fructose-1,6-Biphosphate Aldolase Deficiencies
Fructose-1,6-Biphosphate Aldolase Deficiency
Fructose-1,6-Bisphosphate Aldolase B Deficiency
Fructose-1-Phosphate Aldolase Deficiencies
Fructose-1-Phosphate Aldolase Deficiency
Fructosemia
Fructosemias
Hereditary Fructose Intolerance
Hereditary Fructose Intolerances
Intolerance, Fructose
Intolerances, Fructose  
Tree Number:   C16.320.565.202.251.271
C18.452.648.202.251.271
Definition English:   An autosomal recessive fructose metabolism disorder due to deficient fructose-1-phosphate aldolase (EC 2.1.2.13) activity, resulting in accumulation of fructose-1-phosphate. The accumulated fructose-1-phosphate inhibits glycogenolysis and gluconeogenesis, causing severe hypoglycemia following ingestion of fructose. Prolonged fructose ingestion in infants leads ultimately to hepatic failure and death. Patients develop a strong distaste for sweet food, and avoid a chronic course of the disease by remaining on a fructose- and sucrose-free diet. 
Indexing Annotation English:   an inborn error of fructose metab; do not use /congen & do not coord with INFANT, NEWBORN, DISEASES
See Related English:   Fructose-Bisphosphate Aldolase
 
History Note English:   1991(1975); use FRUCTOSE METABOLISM, INBORN ERRORS 1989-1990; use CARBOHYDRATE METABOLISM, INBORN ERRORS 1964-1988 
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:   24002 
Unique Identifier:   D005633 

Occurrence in VHL: 		 

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