# Diagnosis and Treatment of MODY: An Updated Mini Review
## 3 Significance of MODY Diagnosis  
Although MODY accounts for only 1-5% of all diabetes cases, it has significant implications [28]. The diagnosis of MODY is crucial for patients and their families; therefore, it is important to characterize each MODY subtype and distinguish these from other types  
\begin{table}
\begin{tabular}{c c c c c} \hline \hline
**Type** & **Gene Name** & **Locus** & **Clinical Features** & **Reference** \\ \hline \hline \multirow{4}{*}{1} & \multirow{4}{*}{_HNF4A_} & \multirow{4}{*}{20q13.12} & \multicolumn{3}{c}{Mild - increased fasting and} \\  & & & postprandial plasma glucose, & \\  & & & sensitivity to sulfyniurea derivatives, & \\  & & & low levels of apolipoprotein and & \\  & & & triglycerides, neonatal macrosmia, & \\  & & & neonatal hypoglycemic events & \\  & & & Mild fasting hyperspecmia, impaired & \\
2 & _GCK_ & 7p13 & fasting glucose and impaired glucose & \\  & & & tolerance, HbA1c is usually 7.3–7.5\% & \\  & & & Diminished renal threshold for & \\
3 & _HNF1A_ & 12q24.31 & glycosuria, sensitivity to sulfyniurea derivatives, transient neonatal & \\  & & & hyperinsulinemic hypoglycemia & \\
4 & _IPF/PDXI_ & 13q12.2 & Pancreatic agens, permanent & \\  & & & neonatal diabetes in homozygote & \\  & & & Characterized by renal disease and & \\
5 & _HNF1B_ & 17q12 & urogeninal tract abnormalities in & \\  & & & females, exocrine pancreatic & \\  & & & dysfunction, hyperuricemia & \\  & & & Characterized by obesity and insulin & \\
6 & _NEUROD1_ & 2q13.3 & resistance, neonatal diabetes, child or & \\  & & & adult-onset diabetes neurological & \\  & & & abnormalities & \\
7 & _KLF11_ & 2p25.1 & Pancreatic malignancy & [26] \\  & & & Associated with both endocrine and & \\
8 & _CEL_ & 9q34.13 & exocrine pancreatic dysfunction, & \\  & & & lipomatosis and fibrosis & \\
9 & _PAX4_ & 7q32.1 & Important for transcription for beta & \\  & & & cell development & \\
10 & _INS_ & 11p15.5 & Associated with neonatal diabetes & \\
11 & _BLK_ & 8p23.1 & Contributes to control of beta & \\  & & & signaling & \\
12 & _ABCC8_ & 11p15.1 & Associated with renal diabetes & \\
13 & _KCN11_ & 11p15.1 & Associated with renal diabetes & \\  & & & Associated with renal diabetes & \\
14 & _APPL1_ & 3p14.3 & Associated with Wolfram or & \\  & & & DIDMOAD syndrome & \\ \hline \hline \end{tabular}
\end{table}
Table 1: Summary of MODY subtypes, gene names, locus, and their clinical features.  
of diabetes. MODY patients are often misdiagnosed with either T1 DM or T2 DM, resulting in patients receiving inappropriate treatment [26]. This could be due to overlapping clinical features, which are more common in diabetes, the high cost of genetic testing, and clinicians' lack of awareness [29]. Accurate diagnosis would enable optimal therapeutic management and treatment strategies that differ significantly from those used for T1 DM or T2 DM [7]. Patients who had been receiving T1 DM treatment can switch to oral agents (i.e., sulfonylurea), which will improve their quality of life and glycemic control [30]. For example, _HNF1A_-MODY (MODY 3) and _HNF4A_-MODY (MODY 1) patients are best managed with oral sulfonylurea and can avoid the unnecessary insulin therapy that is generally prescribed before MODY diagnosis [31]. MODY diagnosis is key to providing accurate counselling regarding the predicted clinical outcome, genetic counselling, and identification of affected family members [32].