DiseaseID 14792

家族性高胰岛素性低血糖症6型

disease

SNOMEDCT_US_2016_09_01:A frequent form of diazoxide-sensitive diffuse hyperinsulinism characterized by an excessive uncontrolled insulin secretion (inappropriate for the level of glycemia), asymptomatic hyperammonemia an

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Disease: 1Symptom: 2Target: 20Links: 26
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Record Fields

Scalar fields from the final disease record.

Disease Id
14792
Core Entity Id
72632
Source Entity Count
1
Preferred Name
Hyperinsulinemic Hypoglycemia, Familial, 6
Name Cn
家族性高胰岛素性低血糖症6型
Name Pinyin
Jia Zu Xing Gao Yi Dao Su Xing Di Xue Tang Zheng 6 Xing
Name En
Hyperinsulinemic Hypoglycemia, Familial, 6
Name Latin
Bilingual Status
complete
Disease Type
disease
Umls Disease Type
Disease or Syndrome
Disgenet Type
disease
Mesh Class
Nutritional and Metabolic Diseases
Do Class
genetic disease; disease of metabolism
Hpo Class
Mesh Class Name
Nutritional and Metabolic Diseases
Hpo Class Name
Do Class Name
disease of metabolism; genetic disease
Disease Definition
SNOMEDCT_US_2016_09_01:A frequent form of diazoxide-sensitive diffuse hyperinsulinism characterized by an excessive uncontrolled insulin secretion (inappropriate for the level of glycemia), asymptomatic hyperammonemia and recurrent episodes of profound hypoglycemia induced by fasting and protein rich meals, requiring rapid and intensive treatment to prevent neurological sequelae. Epilepsy and cognitive deficit that are unrelated to hypoglycemia may also occur.|SNOMEDCT_US_2016_09_01:A frequent form of diazoxide-sensitive diffuse hyperinsulinism characterised by an excessive uncontrolled insulin secretion (inappropriate for the level of glycaemia), asymptomatic hyperammonaemia and recurrent episodes of profound hypoglycaemia induced by fasting and protein rich meals, requiring rapid and intensive treatment to prevent neurological sequelae. Epilepsy and cognitive deficit that are unrelated to hypoglycaemia may also occur.
Version
v1,v2
Suppressed
No

Names

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

Name
Hyperinsulinemic Hypoglycemia, Familial, 6
Role
preferred
Name
Familial Hyperinsulinemic Hypoglycemia 6
Role
alias
Name
HHF6
Role
alias
Name
HYPERINSULINISM-HYPERAMMONEMIA SYNDROME
Role
alias

Cross References

Trusted external identifiers retained for this final record.

Herb
HBDIS016395
Omim
606762
Umls
C1847555
Sym Map
SMDE00974
Do Class
DOID:0014667DOID:630
Dis Ge Net
C1847555
Umls Sty
T047
Me Sh Class
C18
Etcm Disease
Hyperinsulinemic Hypoglycemia, Familial, 6
Tcmbank Disease
27754
Itcmdb Generated
ITX-DISEASE-FA277B6F15C9

Attributes

Merged source attributes and domain-specific metadata.

Version
v1,v2
Suppress
0
Page Title
Disease Hyperinsulinemic Hypoglycemia, Familial, 6 Details page
Do Class Name
disease of metabolism; genetic disease
Disease Type
disease
Do Disease Class
genetic disease; disease of metabolism
Umls Disease Type
Disease or Syndrome
Basic Information
Disease Name
Hyperinsulinemic Hypoglycemia, Familial, 6
Global Category
Fetal diseases;Genetic diseases;Metabolic diseases;Rare diseases
Anatomical Category
Blood diseases;Endocrine diseases;Neuronal diseases
Disease Definition
SNOMEDCT_US_2016_09_01:A frequent form of diazoxide-sensitive diffuse hyperinsulinism characterized by an excessive uncontrolled insulin secretion (inappropriate for the level of glycemia), asymptomatic hyperammonemia and recurrent episodes of profound hypoglycemia induced by fasting and protein rich meals, requiring rapid and intensive treatment to prevent neurological sequelae. Epilepsy and cognitive deficit that are unrelated to hypoglycemia may also occur.|SNOMEDCT_US_2016_09_01:A frequent form of diazoxide-sensitive diffuse hyperinsulinism characterised by an excessive uncontrolled insulin secretion (inappropriate for the level of glycaemia), asymptomatic hyperammonaemia and recurrent episodes of profound hypoglycaemia induced by fasting and protein rich meals, requiring rapid and intensive treatment to prevent neurological sequelae. Epilepsy and cognitive deficit that are unrelated to hypoglycaemia may also occur.
Me Sh Disease Class
Nutritional and Metabolic Diseases
Dis Ge Net Disease Type
disease
Disease Class Name Me Sh
Nutritional and Metabolic Diseases
Umls Semantic Type Name
Disease or Syndrome