MODY 1: Difference between revisions

MODY 1
Synonyms	HNF4A-MODY, Hepatocyte nuclear factor 4 alpha maturity onset diabetes of the young
Pronounce	N/A
Specialty	N/A
Symptoms	Hyperglycemia, polyuria, polydipsia, weight loss
Complications	Diabetic complications, cardiovascular disease
Onset	Typically in adolescence or early adulthood
Duration	Chronic
Types	N/A
Causes	Mutations in the HNF4A gene
Risks	Family history of diabetes, genetic predisposition
Diagnosis	Genetic testing, blood glucose tests
Differential diagnosis	Type 1 diabetes, Type 2 diabetes, other forms of MODY
Prevention	N/A
Treatment	Dietary management, oral hypoglycemic agents, insulin therapy
Medication	Sulfonylureas, insulin
Prognosis	Generally good with proper management
Frequency	Rare, accounts for a small percentage of all diabetes mellitus cases
Deaths	N/A

Latest revision as of 23:47, 8 April 2025

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD medical weight loss NYC and sleep center NYC

MODY 1 (Maturity Onset Diabetes of the Young type 1) is a form of monogenic diabetes that is characterized by an autosomal dominant inheritance pattern. It is one of the several types of MODY, which are caused by mutations in a single gene that affect insulin production or secretion.

Genetic Basis[edit]

MODY 1 is caused by mutations in the HNF4A gene, which encodes the hepatocyte nuclear factor 4 alpha. This gene plays a crucial role in the regulation of insulin secretion and glucose metabolism. The mutation leads to impaired insulin secretion, resulting in hyperglycemia.

Clinical Features[edit]

Patients with MODY 1 typically present with mild to moderate hyperglycemia at a young age, often before the age of 25. Unlike type 1 diabetes, MODY 1 does not usually require insulin therapy initially, and patients may be managed with oral hypoglycemic agents.

Diagnosis[edit]

The diagnosis of MODY 1 is confirmed through genetic testing, which identifies mutations in the HNF4A gene. Family history is also an important factor, as MODY 1 follows an autosomal dominant pattern, meaning that a single copy of the mutated gene can cause the disorder.

Management[edit]

Management of MODY 1 involves lifestyle modifications, such as diet and exercise, and the use of oral hypoglycemic agents like sulfonylureas. In some cases, insulin therapy may be required as the disease progresses.

@@ Line 1: / Line 1: @@
-'''MODY 1''' is a form of [[Maturity onset diabetes of the young]] (MODY), a group of inherited forms of [[diabetes mellitus]]. MODY 1 is caused by mutations in the [[HNF4A]] gene.
+{{SI}}
+{{Infobox medical condition
-==Overview==
+| name            = MODY 1
-MODY 1 is a rare form of diabetes that is different from both [[Type 1 diabetes]] and [[Type 2 diabetes]]. It is caused by a mutation in a single gene and is inherited in an autosomal dominant manner. This means that a person only needs to inherit one copy of the mutated gene from one parent to develop the disease.
+| image           = [[File:Autosomal_dominant_-_en.svg|200px]]
+| caption         = MODY 1 is inherited in an [[autosomal dominant]] pattern.
-==Symptoms==
+| synonyms        = [[HNF4A-MODY]], Hepatocyte nuclear factor 4 alpha maturity onset diabetes of the young
-The symptoms of MODY 1 are similar to those of other forms of diabetes. They include frequent urination, excessive thirst, fatigue, blurred vision, and weight loss. However, because MODY 1 is a form of monogenic diabetes, it often presents at a younger age than type 2 diabetes and may be misdiagnosed as type 1 diabetes.
+| field           = [[Endocrinology]]
+| symptoms        = [[Hyperglycemia]], [[polyuria]], [[polydipsia]], [[weight loss]]
-==Diagnosis==
+| complications   = [[Diabetic complications]], [[cardiovascular disease]]
-Diagnosis of MODY 1 involves genetic testing to identify the presence of a mutation in the HNF4A gene. This is usually done through a blood test. It is important to correctly diagnose MODY 1 as it can have implications for treatment and for other family members who may also be at risk.
+| onset           = Typically in [[adolescence]] or early [[adulthood]]
+| duration        = [[Chronic condition|Chronic]]
-==Treatment==
+| causes          = Mutations in the [[HNF4A]] gene
-Treatment for MODY 1 typically involves lifestyle changes such as diet and exercise, and may also include medication. Unlike type 1 diabetes, people with MODY 1 do not always require insulin therapy.
+| risks           = Family history of [[diabetes]], [[genetic predisposition]]
+| diagnosis       = [[Genetic testing]], [[blood glucose]] tests
-==See also==
+| differential    = [[Type 1 diabetes]], [[Type 2 diabetes]], other forms of [[MODY]]
-* [[Maturity onset diabetes of the young]]
+| treatment       = [[Dietary management]], [[oral hypoglycemic agents]], [[insulin therapy]]
-* [[Diabetes mellitus]]
+| medication      = [[Sulfonylureas]], [[insulin]]
-* [[HNF4A]]
+| prognosis       = Generally good with proper management
+| frequency       = Rare, accounts for a small percentage of all [[diabetes mellitus]] cases
+}}
+'''MODY 1''' (Maturity Onset Diabetes of the Young type 1) is a form of [[monogenic diabetes]] that is characterized by an autosomal dominant inheritance pattern. It is one of the several types of MODY, which are caused by mutations in a single gene that affect insulin production or secretion.
+=== Genetic Basis ===
+MODY 1 is caused by mutations in the [[HNF4A]] gene, which encodes the [[hepatocyte nuclear factor 4 alpha]]. This gene plays a crucial role in the regulation of [[insulin]] secretion and [[glucose]] metabolism. The mutation leads to impaired insulin secretion, resulting in [[hyperglycemia]].
+=== Clinical Features ===
+Patients with MODY 1 typically present with mild to moderate [[hyperglycemia]] at a young age, often before the age of 25. Unlike [[type 1 diabetes]], MODY 1 does not usually require [[insulin therapy]] initially, and patients may be managed with [[oral hypoglycemic agents]].
+=== Diagnosis ===
+The diagnosis of MODY 1 is confirmed through genetic testing, which identifies mutations in the HNF4A gene. Family history is also an important factor, as MODY 1 follows an autosomal dominant pattern, meaning that a single copy of the mutated gene can cause the disorder.
+=== Management ===
+Management of MODY 1 involves lifestyle modifications, such as [[diet]] and [[exercise]], and the use of oral hypoglycemic agents like [[sulfonylureas]]. In some cases, insulin therapy may be required as the disease progresses.
+== See also ==
+* [[MODY 2]]
+* [[MODY 3]]
+* [[Type 1 diabetes]]
+* [[Type 2 diabetes]]
+* [[Genetic testing]]
 [[Category:Diabetes]]
 [[Category:Genetic disorders]]
-[[Category:Endocrine disorders]]
-{{stub}}