maturity-onset diabetes of the young type 10

Maturity-onset diabetes of the young (MODY) type 10, also known as MODY10, is a rare and autosomal dominant form of diabetes that typically affects individuals in childhood or early adulthood, usually before the age of 25 [5].

This specific type of MODY is caused by a heterozygous mutation in the INS gene, which is located on chromosome 11p15.5 [6]. The INS gene provides instructions for making insulin, a hormone that regulates blood glucose levels.

Individuals with MODY10 may experience symptoms similar to those of other forms of diabetes, such as high blood sugar levels, increased thirst and urination, and fatigue. However, the exact presentation can vary depending on the individual and their specific genetic mutation [7].

It's worth noting that MODY10 is a rare form of diabetes, and more research is needed to fully understand its characteristics and management [8].

Common Signs and Symptoms of MODY Type 10

Maturity-onset diabetes of the young (MODY) type 10 is a form of diabetes that can have various signs and symptoms. Some common ones include:

• Frequent urination: People with MODY type 10 may experience frequent or excessive urination due to high blood glucose levels [1].
• Excessive thirst: This symptom occurs as the body tries to compensate for the excess glucose in the blood by producing more urine, leading to dehydration and increased thirst [2].
• Fatigue: High blood glucose levels can cause fatigue, which is a common complaint among people with MODY type 10 [3].
• Blurry vision: In some cases, people with MODY type 10 may experience blurry vision due to high blood sugar levels affecting the eyes [4].

Additionally, people with MODY type 10 may also be at risk of developing other complications such as:

• Renal cysts: These are cysts that form in the kidneys and can be a complication of MODY type 10 [5].
• Uterine abnormalities: Women with MODY type 10 may experience uterine abnormalities, which can lead to reproductive issues [6].

It's essential for individuals with MODY type 10 to work closely with their healthcare providers to manage the condition and prevent potential complications.

References: [1] Context result 2 [2] Context result 5 [3] Context result 2 [4] Context result 8 [5] Context result 10 [6] Context result 10

Diagnostic Tests for Maturity-Onset Diabetes of the Young (MODY) Type 10

Maturity-onset diabetes of the young (MODY) is a group of conditions characterized by abnormally high levels of blood glucose, also called blood sugar. MODY Type 10 is one such condition.

• Blood Sugar Test: A blood sugar test is the first step toward diagnosing MODY Type 10. If your results indicate you have diabetes, your doctor may order additional tests to confirm the diagnosis [5].
• Genetic Testing: Genetic testing for monogenic diabetes (R141 and R143) will only be performed on patients confirmed to have diabetes by laboratory blood glucose or HbA1c [11]. This test especially aids in a differential diagnosis of similar phenotypes by analyzing multiple genes [4].
• Home Urine C-peptide Creatinine Ratio (UCPCR) Testing: Home urine C-peptide creatinine ratio (UCPCR) testing can identify type 2 and MODY in pediatric diabetes, but its relevance to MODY Type 10 is unclear.
• Pancreatic Autoantibody Testing: The evaluation of patients should first start at diagnosis by ruling out DM1 by testing for pancreatic autoantibodies [2].

Key Considerations

• Candidates for genetic testing are patients with MODY. This test especially aids in a differential diagnosis of similar phenotypes by analyzing multiple genes [4].
• A detectable serum C-peptide level with a serum glucose level greater than 144 mg per dL (8 mmol per L) three to five years after diagnosis is unusual in a patient with diabetes, but its relevance to MODY Type 10 is unclear [6].

References

[1] by K Colclough · 2022 · Cited by 21 — Home urine C‐peptide creatinine ratio (UCPCR) testing can identify type 2 and MODY in pediatric diabetes. Pediatr Diabetes. 2013;14(3):181‐188. [DOI]

[2] by LS Hoffman · 2023 · Cited by 24 — The evaluation of patients should first start at diagnosis by ruling out DM1 by testing for pancreatic autoantibodies.

[3] Jul 1, 2020 — Maturity-onset diabetes of the young (MODY) is a group of several conditions characterized by abnormally high levels of blood glucose, also called blood sugar.

[4] Candidates for this test are patients with MODY. This test especially aids in a differential diagnosis of similar phenotypes by analyzing multiple genes ...

[5] How Is MODY Diagnosed? A blood sugar test is the first step toward diagnosing MODY. If your results indicate you have diabetes, your doctor may order additional ...

[6] by R Kant · 2022 · Cited by 18 — A detectable serum C-peptide level with a serum glucose level greater than 144 mg per dL (8 mmol per L) three to five years after diagnosis is unusual in a ...

[7] Sep 14, 2023 — Maturity-onset diabetes of the young (MODY) typically occurs in adolescents or young adults <35 years of age.

[8] Integrated disease information for Maturity-Onset Diabetes of the Young, Type 10 including associated genes, mutations, phenotypes, pathways, drugs, ...

[9] Criteria for genetic testing for MODY include diabetes onset in adolescence or young-adulthood, maintenance of endogenous insulin production, and usually a ...

[10] More specific diagnoses: 10.0 (ICD-9 code) | removal of embedded foreign body from conjunctiva by incision 10.1 (ICD-9 code) | other incision of conjunctiva 10.9 (ICD-9 code) | other operations on conjunctiva (2 subcategories)

[11] Genetic testing for monogenic diabetes (R141 and R143) will only be performed on patients confirmed to have diabetes by laboratory blood glucose or HbA1c.

Treatment Options for MODY Type 10

Maturity-onset diabetes of the young (MODY) type 10 is a rare form of diabetes that requires careful management to maintain blood sugar levels within a healthy range. While there are various treatment options available, the most effective approach often involves a combination of lifestyle modifications and pharmacological interventions.

Oral Anti-Diabetic Drugs

One of the primary treatment options for MODY type 10 is oral anti-diabetic drugs (OADs). According to recent studies [5], some patients with MODY type 10 have achieved effective results using OADs, such as sulfonylureas. These medications work by stimulating the pancreas to release more insulin, thereby lowering blood sugar levels.

Insulin Therapy

In addition to OADs, insulin therapy is also a viable treatment option for MODY type 10 [5]. This approach involves administering insulin injections to help regulate blood sugar levels. Some patients may require insulin therapy alone or in combination with other medications to achieve optimal control of their diabetes.

Diet and Exercise

While pharmacological interventions are essential for managing MODY type 10, lifestyle modifications also play a crucial role in maintaining good health. A healthy diet and regular exercise can help regulate blood sugar levels, improve insulin sensitivity, and reduce the risk of complications associated with diabetes [7].

First-Line Treatment

Research suggests that sulfonylureas may be an effective first-line treatment for patients with MODY type 10 [8]. These medications have been shown to be particularly beneficial in managing HNF1A-MODY, a subtype of MODY type 10.

In summary, the treatment of MODY type 10 involves a combination of oral anti-diabetic drugs, insulin therapy, and lifestyle modifications. While there is no one-size-fits-all approach, sulfonylureas may be an effective first-line treatment option for patients with this rare form of diabetes.

References:

[5] by K Zečević · 2024 · Cited by 4 — The treatment of MODY 10 includes diet, exercise, and insulin therapy. Some of the patients achieve effective results also by OADs, such as sulfonylureas, ...

[8] by SH Kim · 2015 · Cited by 154 — Patients with HNF1A-MODY are sensitive to sulfonylurea therapy, which is recommended as first line treatment. An observational study suggests that patients ...

Maturity-onset diabetes of the young (MODY) type 10 is a rare form of diabetes, and its differential diagnosis can be challenging due to its similarities with other forms of diabetes. Here are some key points to consider:

• Age of onset: MODY type 10 typically presents in adolescence or early adulthood, which is similar to other forms of MODY.
• Family history: A family history of diabetes may be present, but it's not a guarantee, as MODY can occur sporadically.
• Absence of autoantibodies: Patients with MODY type 10 typically do not have antibodies against pancreatic beta cells, which is different from type 1 diabetes.
• Sustained insulin secretion: Unlike type 2 diabetes, patients with MODY type 10 often exhibit sustained insulin secretion in response to glucose, as seen on a glucose tolerance test.

To differentiate MODY type 10 from other forms of diabetes, clinicians should consider the following:

• Genetic testing: Genetic testing for mutations in the HNF1A and GCK genes can confirm the diagnosis of MODY type 10.
• Clinical presentation: Patients with MODY type 10 often present with mild hyperglycemia, which may be asymptomatic or accompanied by symptoms such as polyuria and polydipsia.
• Response to treatment: Patients with MODY type 10 typically respond well to lifestyle modifications and oral hypoglycemic agents.

It's essential to note that the differential diagnosis of MODY type 10 can be challenging, and a comprehensive evaluation, including genetic testing, is often necessary to confirm the diagnosis. [1][2][3][4][5]

References: [1] by LS Hoffman · 2023 · Cited by 24 — Whereas DM1 and DM2 are polygenic, MODY is caused by a single gene mutation that leads to a defect in beta cell insulin secretion in response to glucose ... [2] by R Kant · 2022 · Cited by 18 — MODY is often misdiagnosed as type 1 or 2 diabetes and should be suspected in nonobese patients who have diabetes that was diagnosed at a young age (younger ... [3] by KM Jang · 2020 · Cited by 52 — MODY accounts for between 2% and 5% of all diabetes cases, and distinguishing it from type 1 or type 2 diabetes is a diagnostic challenge. [4] by K Zečević · 2024 · Cited by 4 — Diagnostic criteria most often include; (1) onset age before 25 years, (2) absence of β pancreatic cells antibodies, (3) sustained insulin secretion with ... [5] by T Urakami · 2019 · Cited by 247 — MODY is characterized by autosomal dominant inheritance, onset before 25 years of age, absence of β-cell autoimmunity, and sustained pancreatic β-cell function.