PedAM
Pediatric Disease Annotations & Medicines
| Disease | maturity-onset diabetes of the young |
| Phenotype | |maturity-onset diabetes of the young |
| Sentences | 137 |
| PubMedID- 21515849 | Stable fasting hyperglycemia is observed in individuals with gck maturity-onset diabetes of the young (mody), a monogenic form of diabetes caused by mutations in the gck gene, and is maintained over the course of a lifetime (27), with many of these individuals showing no symptoms, suggesting that stable mild fasting hyperglycemia may not in itself be detrimental. |
| PubMedID- 23610061 | Significantly, mutations in pdx1 (5), pax6 (6), and neurod1 (7) also cause maturity-onset diabetes of the young in humans. |
| PubMedID- 23264425 | They are referred to as maturity-onset diabetes of the young (mody) and are characterized by impaired insulin secretion with minimal or no defects in insulin action. |
| PubMedID- 22577560 | The hepatocyte nuclear factor-1α (hnf1α), which haploinsufficiency causes the maturity-onset diabetes of the young type 3 (mody3), also appears to modulate shp expression via the fxr pathway. |
| PubMedID- 22723580 | Candidates were excluded if they had diabetes secondary to a specific disease (maturity-onset diabetes of the young, pancreatitis, or pancreatectomy), drug or alcohol addiction, recent vascular event (myocardial infarction, coronary angioplasty, or stroke within 6 months), internal malignancy, portal hypertension, inability to cooperate in long-term follow-up, poor understanding of the operation, or unrealistic expectations of outcomes or mental impairment (as judged by investigators during the first clinic visit). |
| PubMedID- 26110391 | In human, heterozygous mutations in nr2a1 are associated with maturity-onset diabetes of the young type 1 (mody1), an autosomal dominant genetic disorder that is characterized by early onset type 2 diabetes. |
| PubMedID- 23776849 | [37] heterozygous mutations in the hnf4a gene causes maturity-onset diabetes of the young type 1 (mody1), which is characterized by progressive β-cell dysfunction and failure of glucose induced insulin secretion. |
| PubMedID- 22299039 | Rare mutations in hnf1b have been associated with maturity-onset diabetes of the young subtype 5 (mody5), renal cysts, pancreatic atrophy, and uterine abnormalities caused by incomplete mullerian duct fusion and mullerian duct aplasia [15], [16]. |
| PubMedID- 22291974 | In contrast, homozygous inactivating mutations cause complete gk deficiency and permanent neonatal diabetes mellitus (pndm), whereas heterozygous inactivating mutations cause familial, mild fasting hyperglycaemia also known as maturity-onset diabetes of the young type 2 (mody2; [10] [11]). |
| PubMedID- 24586696 | Heterozygous inactivating gck mutations cause maturity-onset diabetes of the young (mody), homozygous or compound heterozygous inactivating mutations cause permanent neonatal diabetes mellitus (pndm), and heterozygous activating mutations cause congenital hyperinsulinism (chi) [10]. |
| PubMedID- 21805196 | Karlsson et al., looking for biomarkers for maturity-onset diabetes of the young (mody), found that complement c5 and c8 are both elevated in diabetes, regardless of aetiology [117], a possible mechanism for these abnormalities being that complement activation can be driven by glycated immunoglobulins [118]. |
| PubMedID- 20863361 | Bmi: body mass index; gada: glutamic acid decarboxylase autoantibodies; iaa: insulin autoantibodies; ia-2a: insulinoma-associated antigen-2 autoantibodies; ica: islet cell antibodies; k-atp channel: atp-sensitive potassium channel; monogenic diabetes: maturity-onset diabetes of the young; ru: relative units. |
| PubMedID- 24244580 | maturity-onset diabetes of the young type 2 (mody2) is a monogenic form of diabetes with autosomic dominant transmission caused by heterozygous, inactivating mutation in the glucokinase gene (gck). |
| PubMedID- 23110382 | Lada (latent autoimmune diabetes in adults), mody (maturity-onset diabetes of the young), pre-diabetes, diabetes in remission. |
| PubMedID- 21429197 | Exclusion criteria were: suspected non-type 1 diabetes (type 2 diabetes, maturity-onset diabetes of the young (mody) or secondary diabetes), decline of enrolment into the study by patients or parents, and patients initially treated outside of the centres for more than 5 days. |
| PubMedID- 23166422 | Among young-onset, non-insulin-dependent diabetes, distinguishing type 2 diabetes from maturity-onset diabetes of the young (mody) on the basis of family history can be problematic. |
| PubMedID- 26442106 | Tested against kegg database, enriched pathways unique to the tumors included abc transporters, lysine degradation, mody (maturity-onset diabetes of the young), pantothenate and coa biosynthesis, cell cycle, and cell adhesion as well as olfactory receptors (table 3). |
| PubMedID- 20056949 | Subjects who tested positive for anti-gad antibodies and those with mitochondrial disease (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) or maturity-onset diabetes of the young (mody) were not included. |
| PubMedID- 23281592 | Likewise, maturity-onset diabetes of the young type (mody syndrome) is linked to kinetic alterations and regulation of glucokinase activity [46,47] and in our ranking glucokinase receptor is the top ranked gene for mody syndrome. |
| PubMedID- 25894234 | Moreover, in maturity-onset diabetes of the young (mody) patients with low insulin responses, there are delayed and decreased insulin and c-peptide secretory responses to glucose due to beta cell dysfunction [31]. |
| PubMedID- 24023612 | The sequence variations in or near the blk gene have been shown to cosegregate with maturity-onset diabetes of the young (mody) in familial studies. |
| PubMedID- 23274891 | We hypothesized that loss-of-function hnf1a mutations causal for maturity-onset diabetes of the young (mody) would display altered fucosylation of n-linked glycans on plasma proteins and that glycan biomarkers could improve the efficiency of a diagnosis of hnf1a-mody. |
| PubMedID- 24303504 | The following were excluded: (1) patients with diabetes undergoing thiazolidinedione therapy; (2) patients with hypertension undergoing angiotensin-converting enzyme inhibitors therapy; (3) patients with diagnosed diabetic nephropathy; (4) patients with acute or chronic inflammatory disease; and (5) patients with type 1 diabetes, maturity-onset diabetes of the young, or mitochondrial diabetes. |
| PubMedID- 20571754 | However, it is worth noting that not all glucose-raising loci appear to influence type 2 diabetes risk [11], possibly because some loci may cause modest elevations in glucose concentrations that do not worsen over time, as observed in maturity-onset diabetes of the young [17]. |
| PubMedID- 20360843 | Mutations in human hnf4α are associated with maturity-onset diabetes of the young, an autosomal dominant genetic condition associated with early onset diabetes [8]. |
| PubMedID- 21636800 | This group may also include individuals with single-gene mutations affecting β-cell function, historically referred to as maturity-onset diabetes of the young (mody). |
| PubMedID- 23419424 | Patients with normal c-peptide levels, those who were considered to have maturity-onset diabetes of the young (mody) based on the family history and clinical findings, those with t2dm, and those with a chronic disease (such as thalassemia, cystic fibrosis, drug-induced types) were excluded from the study. |
| PubMedID- 23139355 | maturity-onset diabetes of the young (mody) is a group of monogenic forms of diabetes (1) that exhibit specific loss-of-function mutations with characteristic phenotypes. |
| PubMedID- 24606082 | maturity-onset diabetes of the young (mody) accounts for approximately 5% of all diabetes cases diagnosed before the age of 45 years (1). |
| PubMedID- 19933992 | Serum 1,5 anhydroglucitol (1,5ag) levels were reported to differentiate maturity-onset diabetes of the young due to hnf1a mutations (hnf1a-mody) from type 2 diabetes, but this requires further validation. |
| PubMedID- PMC4429118 | maturity-onset diabetes of the young type 5 encompasses a wide clinical spectrum. |
| PubMedID- 23282078 | We reported that psmd9 may rarely cause t2d by unique mutations [12] and that is linked to t2d [13], maturity-onset diabetes of the young 3 (mody3) [14], t2d-microvascular [15-18] and t2d-macrovascular complications [19], hypercholesterolemia [20], hypertension [21], carpal tunnel syndrome [22], and depression [23]. |
| PubMedID- 26430540 | These adenomas occur almost always in females, are multiple in half the cases, and can be associated with familial hepatic adenomatosis and maturity-onset diabetes of the young (mody). |
| PubMedID- 22611063 | We identified three probands with a phenotype consistent with maturity-onset diabetes of the young (mody) subtype gck-mody, in whom two potential pathogenic mutations were identified: [r43h/g68d], [e248 k/i225m], or [g261r/d217n]. |
| PubMedID- 23274887 | Heterozygous loss-of-function pdx1 mutations are linked to common human type 2 diabetes and cause heritable maturity-onset diabetes of the young type 4 (13,14). |
| PubMedID- 20628089 | However, any unidentified maturity-onset diabetes of the young lineages could falsely increase risk estimates. |
| PubMedID- 22808921 | maturity-onset diabetes of the young (mody), is a monogenic form of diabetes characterized by autosomal dominant mode of inheritance including a three-generation family history of diabetes, age at diagnosis of diabetes of 25 years or less in at least one family member and reduced glucose-stimulated insulin secretion [1]. |