DiseaseID 26561

遗传性果糖不耐受

Fructose Intolerance, Hereditary

Relationship Network

Interactive first-hop connections across herbs, ingredients, formulas, targets, diseases, symptoms, syndromes, evidence, and monographs.

Click a node to open it in a new tab

Disease: 1Symptom: 12Target: 16Links: 36

Arranging relationship network...

Record Fields

Scalar fields from the final disease record.

Disease Id: 26561
Core Entity Id: 119129
Source Entity Count: 1
Preferred Name: Fructose Intolerance, Hereditary
Name Cn: 遗传性果糖不耐受
Name Pinyin: Yi Chuan Xing Guo Tang Bu Nai Shou
Name En: Fructose Intolerance, Hereditary
Name Latin
Bilingual Status: complete
Disease Type
Umls Disease Type
Disgenet Type
Mesh Class
Do Class
Hpo Class
Mesh Class Name
Hpo Class Name
Do Class Name
Disease Definition: NCI2016_02D:A genetic disorder characterized by the absence of the enzyme aldolase-B from the liver. This enzyme is essential for the metabolism of fructose. Signs and symptoms from fructose ingestion are evident in infancy and include vomiting, abdominal pain and hypoglycemia. Long term complications include hepatic and renal failure.|MSH2017_2016_08_12: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.|CSP2006: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; 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.
Version: v1,v2
Suppressed: No

Names

Preferred names, aliases, and source labels retained in the final schema.

Name

Fructose Intolerance, Hereditary

Role

preferred

Cross References

Trusted external identifiers retained for this final record.

Umls

C0016751

Icd10

E74.12

Sym Map

SMDE04071

Etcm Disease

Fructose Intolerance, Hereditary

Itcmdb Generated

ITX-DISEASE-71519F0D41F4

Attributes

Merged source attributes and domain-specific metadata.

Version

v1,v2

Suppress

Page Title

Disease Fructose Intolerance, Hereditary Details page

Basic Information

Disease Name

Fructose Intolerance, Hereditary

Global Category

Genetic diseases;Metabolic diseases;Rare diseases

Anatomical Category

Blood diseases;Gastrointestinal Diseases;Liver diseases;Nephrological diseases

Disease Definition

NCI2016_02D:A genetic disorder characterized by the absence of the enzyme aldolase-B from the liver. This enzyme is essential for the metabolism of fructose. Signs and symptoms from fructose ingestion are evident in infancy and include vomiting, abdominal pain and hypoglycemia. Long term complications include hepatic and renal failure.|MSH2017_2016_08_12: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.|CSP2006: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; 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.