|
Hyperglycemia
|
0.200 |
Biomarker
|
disease |
BEFREE |
This report describes GCK-MODY in a Chinese family and stresses that in managing this condition it is important to avoid unnecessary drug treatment and excessive anxiety about mild hyperglycemia.
|
31571622 |
2020 |
|
Hyperglycemia
|
0.200 |
Biomarker
|
disease |
BEFREE |
At diagnosis of pediatric diabetes, absence of all islet autoantibodies and modest hyperglycemia (HbA<sub>1c</sub> <7.5% [58 mmol/mol]) should result in testing for GCK, HNF1A, and HNF4A MODY.
|
31704690 |
2020 |
|
Hyperglycemia
|
0.200 |
Biomarker
|
disease |
BEFREE |
However, recent analyses have also demonstrated ABCC8 gene mutations in patients with monogenic diabetes (maturity onset diabetes of the young, MODY), with milder clinical phenotypes and later onset of hyperglycemia.
|
30734462 |
2019 |
|
Hyperglycemia
|
0.200 |
Biomarker
|
disease |
BEFREE |
GCK-MODY is characterized by mild hyperglycemia.
|
30535721 |
2019 |
|
Hyperglycemia
|
0.200 |
Biomarker
|
disease |
BEFREE |
This case raises the question as to whether hyperglycaemia in GCK-MODY may increase the risk of fetal caudal regression syndrome as reported in women with pre-existing diabetes mellitus.
|
30362177 |
2019 |
|
Hyperglycemia
|
0.200 |
Biomarker
|
disease |
BEFREE |
We assessed the contribution of hyperglycemia by comparing insulin sensitivity in control and GCK-MODY and the contribution of hyperinsulinemia by comparing GCK-MODY and type 1 diabetes.
|
31092478 |
2019 |
|
Hyperglycemia
|
0.200 |
Biomarker
|
disease |
BEFREE |
Eleven children with asymptomatic hyperglycemia and clinically suspected GCK-MODY were identified from the database of children with diabetes in the biggest children's hospital in South China.
|
29510678 |
2018 |
|
Hyperglycemia
|
0.200 |
Biomarker
|
disease |
BEFREE |
The findings leading to the diagnosis were impaired fasting glucose (IFG) (15/37), symptoms of hyperglycemia (4/37), and a GCK-MODY family history (18/37).
|
28663157 |
2017 |
|
Hyperglycemia
|
0.200 |
CausalMutation
|
disease |
CLINVAR |
Characteristics of maturity onset diabetes of the young in a large diabetes center.
|
26059258 |
2016 |
|
Hyperglycemia
|
0.200 |
Biomarker
|
disease |
BEFREE |
GCK-MODY leads to mildly elevated blood glucose typically not requiring therapy.
|
27106716 |
2016 |
|
Hyperglycemia
|
0.200 |
Biomarker
|
disease |
BEFREE |
People with GCK-MODY have a defect in glucose sensing; hence, glucose homeostasis is maintained at a higher set point resulting in mild, asymptomatic fasting hyperglycemia (5.4-8.3 mmol/L, HbA1c range 5.8-7.6% [40-60 mmol/mol]), which is present from birth and shows slight deterioration with age.
|
26106223 |
2015 |
|
Hyperglycemia
|
0.200 |
GeneticVariation
|
disease |
BEFREE |
However, mutations in the HNF1A and HNF4A cause a progressive pancreatic β-cell dysfunction and hyperglycemia that can result in microvascular complications.
|
25581748 |
2015 |
|
Hyperglycemia
|
0.200 |
CausalMutation
|
disease |
CLINVAR |
Clinical heterogeneity of abnormal glucose homeostasis associated with the HNF4A R311H mutation.
|
24947580 |
2014 |
|
Hyperglycemia
|
0.200 |
Biomarker
|
disease |
BEFREE |
MODY generally occurs in non-obese patients with negative autoantibodies presenting with mild to moderate hyperglycemia.
|
25372588 |
2014 |
|
Hyperglycemia
|
0.200 |
Biomarker
|
disease |
BEFREE |
Out of these 78 people with GCK-MODY and 40 additional family members with hyperglycaemia whose genetic status was unknown, only one had diabetic nephropathy.
|
24804978 |
2014 |
|
Hyperglycemia
|
0.200 |
GeneticVariation
|
disease |
BEFREE |
Family history for mild hyperglycaemia and GADA fluctuation alerted us to a possible MODY diagnosis which was confirmed by detection of GCK mutation c.626C>T; p.T209M.
|
23352578 |
2013 |
|
Hyperglycemia
|
0.200 |
GeneticVariation
|
disease |
CLINVAR |
Mutations in the genes encoding the transcription factors hepatocyte nuclear factor 1 alpha and 4 alpha in maturity-onset diabetes of the young and hyperinsulinemic hypoglycemia.
|
23348805 |
2013 |
|
Hyperglycemia
|
0.200 |
CausalMutation
|
disease |
CLINVAR |
Mutations in the genes encoding the transcription factors hepatocyte nuclear factor 1 alpha and 4 alpha in maturity-onset diabetes of the young and hyperinsulinemic hypoglycemia.
|
23348805 |
2013 |
|
Hyperglycemia
|
0.200 |
CausalMutation
|
disease |
CLINVAR |
Multidomain integration in the structure of the HNF-4α nuclear receptor complex.
|
23485969 |
2013 |
|
Hyperglycemia
|
0.200 |
Biomarker
|
disease |
BEFREE |
In contrast, mutations in the genes encoding the transcription factors HNF1A and HNF4A cause a progressive insulin secretory defect and hyperglycaemia that can lead to vascular complications.
|
23878349 |
2013 |
|
Hyperglycemia
|
0.200 |
GeneticVariation
|
disease |
BEFREE |
MODY1 and MODY3 mutations may interact by chance to give a more severe form of diabetes (younger age at presentation and early need of insulin therapy to control hyperglycemia).
|
20705777 |
2010 |
|
Hyperglycemia
|
0.200 |
CausalMutation
|
disease |
CLINVAR |
Macrosomia and hyperinsulinaemic hypoglycaemia in patients with heterozygous mutations in the HNF4A gene.
|
17407387 |
2007 |
|
Hyperglycemia
|
0.200 |
GeneticVariation
|
disease |
BEFREE |
We genetically analyzed four families of young children with fasting hyperglycemia with family histories of diabetes for mutations in the genes for hepatocyte nuclear factor 4 alpha (HNF4alpha), glucokinase (GCK), and hepatocyte nuclear factor 1 alpha (HNF1alpha), the genes responsible for MODY1, MODY2, and MODY3, respectively.
|
16444761 |
2006 |
|
Hyperglycemia
|
0.200 |
CausalMutation
|
disease |
CLINVAR |
Mutations in the genes encoding the transcription factors hepatocyte nuclear factor 1 alpha (HNF1A) and 4 alpha (HNF4A) in maturity-onset diabetes of the young.
|
16917892 |
2006 |
|
Hyperglycemia
|
0.200 |
CausalMutation
|
disease |
CLINVAR |
Molecular genetics and phenotypic characteristics of MODY caused by hepatocyte nuclear factor 4alpha mutations in a large European collection.
|
15830177 |
2005 |