ICD-10: N04.6

Nephrotic syndrome with dense deposit disease is a specific subtype of nephrotic syndrome characterized by the presence of dense deposits in the glomerular basement membrane. This condition is associated with significant proteinuria, hypoalbuminemia, and edema, which are hallmark features of nephrotic syndrome.

Clinical Description

Definition

Nephrotic syndrome is a kidney disorder that results in the excretion of excessive protein in the urine, leading to a range of systemic effects. Dense deposit disease (DDD), also known as membranoproliferative glomerulonephritis type II, is a rare form of kidney disease that primarily affects the glomeruli, the filtering units of the kidneys. It is characterized by the presence of dense deposits in the glomerular basement membrane, which can lead to inflammation and damage to the kidney tissue.

Symptoms

Patients with nephrotic syndrome with dense deposit disease typically present with:
- Proteinuria: High levels of protein in the urine, often exceeding 3.5 grams per day.
- Edema: Swelling, particularly in the legs, ankles, and around the eyes, due to fluid retention.
- Hypoalbuminemia: Low levels of albumin in the blood, which can lead to further edema and other complications.
- Hyperlipidemia: Elevated levels of lipids in the blood, which is a common response to low albumin levels.

Pathophysiology

The pathophysiology of dense deposit disease involves the deposition of complement component C3 and other proteins in the glomerular basement membrane. This leads to complement-mediated damage and inflammation, resulting in the characteristic symptoms of nephrotic syndrome. The exact cause of DDD is not fully understood, but it is believed to involve dysregulation of the complement system, which plays a crucial role in immune response and inflammation.

Diagnosis

Diagnosis of nephrotic syndrome with dense deposit disease typically involves:
- Urinalysis: To detect proteinuria and assess urine sediment.
- Blood tests: To evaluate levels of albumin, cholesterol, and kidney function markers such as creatinine.
- Kidney biopsy: This is often necessary to confirm the diagnosis and assess the extent of glomerular damage. Histological examination reveals the characteristic dense deposits in the glomerular basement membrane.

Treatment

Management of nephrotic syndrome with dense deposit disease focuses on controlling symptoms and preventing complications:
- Corticosteroids: These may be used to reduce inflammation and proteinuria.
- Immunosuppressive agents: In cases resistant to steroids, medications such as cyclophosphamide or mycophenolate mofetil may be considered.
- Diuretics: To manage edema and fluid overload.
- ACE inhibitors or ARBs: These can help reduce proteinuria and protect kidney function.
- Dietary modifications: A low-sodium diet may be recommended to help manage edema.

Prognosis

The prognosis for patients with nephrotic syndrome with dense deposit disease can vary. Some patients may experience remission, while others may progress to chronic kidney disease or end-stage renal disease. Regular monitoring and management are essential to optimize outcomes and address any complications that arise.

In summary, ICD-10 code N04.6 specifically refers to nephrotic syndrome with dense deposit disease, highlighting the need for careful diagnosis and management of this complex condition. Understanding the clinical features, diagnostic criteria, and treatment options is crucial for healthcare providers in delivering effective care to affected patients.

Nephrotic syndrome with dense deposit disease (ICD-10 code N04.6) is a specific form of nephrotic syndrome characterized by the presence of dense deposits in the glomerular basement membrane. This condition is associated with a range of clinical presentations, signs, symptoms, and patient characteristics that are important for diagnosis and management.

Clinical Presentation

Overview of Nephrotic Syndrome

Nephrotic syndrome is a kidney disorder that results in significant proteinuria, hypoalbuminemia, and edema. It can be caused by various underlying conditions, including primary kidney diseases and systemic diseases. Dense deposit disease is a specific type of membranoproliferative glomerulonephritis (MPGN) that leads to nephrotic syndrome due to the accumulation of complement component C3 in the kidney[1][2].

Signs and Symptoms

Patients with nephrotic syndrome with dense deposit disease typically present with the following signs and symptoms:

• Edema: Swelling, particularly in the legs, ankles, and around the eyes, is a hallmark of nephrotic syndrome. This occurs due to fluid retention resulting from low serum albumin levels[3].
• Proteinuria: Patients often exhibit significant protein loss in urine, which can be detected through urine dipstick tests or 24-hour urine collection. The proteinuria is usually greater than 3.5 grams per day[4].
• Hypoalbuminemia: Low levels of albumin in the blood are common, contributing to edema and other complications[5].
• Hyperlipidemia: Increased levels of lipids in the blood may occur as a compensatory response to low albumin levels[6].
• Fatigue and Weakness: General malaise and decreased energy levels are frequently reported by patients due to the systemic effects of the syndrome[7].

Additional Clinical Features

• Hypertension: Some patients may develop high blood pressure, which can complicate the clinical picture[8].
• Increased Risk of Infections: Due to the loss of immunoglobulins in urine, patients may be more susceptible to infections[9].
• Thromboembolic Events: There is an increased risk of thrombosis due to changes in coagulation factors associated with nephrotic syndrome[10].

Patient Characteristics

Demographics

• Age: Dense deposit disease can occur in both children and adults, but it is more commonly diagnosed in children and young adults[11].
• Gender: There is a slight male predominance in cases of dense deposit disease, although it can affect individuals of any gender[12].

Underlying Conditions

• Associated Conditions: Dense deposit disease is often associated with complement dysregulation, and patients may have underlying conditions such as systemic lupus erythematosus (SLE) or infections that can trigger or exacerbate the syndrome[13][14].
• Family History: A family history of kidney disease may be present in some patients, suggesting a genetic predisposition[15].

Conclusion

Nephrotic syndrome with dense deposit disease (ICD-10 code N04.6) presents with a distinct set of clinical features, including significant proteinuria, edema, hypoalbuminemia, and hyperlipidemia. Understanding these signs and symptoms, along with patient demographics and potential underlying conditions, is crucial for accurate diagnosis and effective management. Early recognition and treatment can help mitigate complications and improve patient outcomes.

References

1. Nephrotic Syndrome | 5-Minute Clinical Consult.
2. Nephrotic Syndrome | Diseases & Conditions.
3. Nephrotic syndrome - Symptoms & causes - Mayo Clinic.
4. Nephrotic Syndrome Clinical Presentation - Medscape.
5. ICD-10-CM Diagnosis Code N04.6 - ICD List.
6. Premature mortality due to nephrotic syndrome and the trend.
7. 2025 ICD-10-CM Codes N04*: Nephrotic syndrome.
8. 2025 ICD-10-CM Diagnosis Code N04.9: Nephrotic syndrome with ...
9. ICD-10 Coordination and Maintenance Committee Meeting.
10. 14: Diseases of the Genitourinary System (N00-N99).
11. 2025 ICD-10-CM Diagnosis Code N04.0 - The Web's Free 2023 ICD-10-CM/PCS.
12. 14: Diseases of the Genitourinary System (N00 - N99).
13. ICD-10 International statistical classification of diseases and ...
14. Nephrotic syndrome - Symptoms & causes - Mayo Clinic.
15. Nephrotic Syndrome | 5-Minute Clinical Consult.

Nephrotic syndrome with dense deposit disease, classified under ICD-10 code N04.6, is a specific type of nephrotic syndrome characterized by the presence of dense deposits in the glomerular basement membrane. This condition is often associated with various underlying causes and can be referred to by several alternative names and related terms. Below is a detailed overview of these terms.

Alternative Names for Nephrotic Syndrome with Dense Deposit Disease

1. Dense Deposit Disease (DDD): This is the most common alternative name for N04.6. It emphasizes the histological finding of dense deposits in the kidney's glomeruli.

2. Membranoproliferative Glomerulonephritis (MPGN): While not synonymous, dense deposit disease is often classified under the broader category of MPGN, which includes various forms of glomerulonephritis characterized by the proliferation of glomerular cells and thickening of the glomerular membrane.

3. C3 Glomerulopathy: This term is used to describe a group of diseases, including dense deposit disease, that are characterized by abnormalities in the complement component C3. It highlights the role of the complement system in the pathogenesis of the disease.

4. Atypical Hemolytic Uremic Syndrome (aHUS): In some cases, dense deposit disease can be associated with aHUS, a condition that involves complement dysregulation and can lead to kidney damage.

Related Terms and Concepts

1. Nephrotic Syndrome: This is a general term for a group of symptoms that include proteinuria, hypoalbuminemia, and edema. N04.6 is a specific subtype of nephrotic syndrome.

2. Glomerular Disease: This term encompasses a variety of kidney diseases affecting the glomeruli, including nephrotic syndrome with dense deposit disease.

3. Complement-Mediated Kidney Disease: This term refers to kidney diseases that involve the complement system, which plays a crucial role in the immune response and can contribute to the pathogenesis of dense deposit disease.

4. Kidney Biopsy Findings: The diagnosis of dense deposit disease is often confirmed through kidney biopsy, which reveals characteristic dense deposits in the glomeruli.

5. Chronic Kidney Disease (CKD): Patients with nephrotic syndrome with dense deposit disease may progress to CKD if not managed appropriately.

Conclusion

Understanding the alternative names and related terms for ICD-10 code N04.6 is essential for healthcare professionals involved in the diagnosis and treatment of kidney diseases. Recognizing these terms can facilitate better communication among clinicians and improve patient care by ensuring accurate diagnosis and appropriate management strategies. If you have further questions or need more specific information, feel free to ask!

Nephrotic syndrome with dense deposit disease, classified under ICD-10 code N04.6, is a specific type of nephrotic syndrome characterized by the presence of dense deposits in the glomerular basement membrane. The diagnosis of this condition involves a combination of clinical evaluation, laboratory tests, and imaging studies. Below are the key criteria used for diagnosis:

Clinical Criteria

1. Symptoms of Nephrotic Syndrome: The primary clinical features include:
   - Proteinuria: Typically, a 24-hour urine collection shows protein excretion greater than 3.5 grams.
   - Hypoalbuminemia: Serum albumin levels are usually less than 3.0 g/dL.
   - Edema: Patients often present with significant swelling, particularly in the legs, abdomen, and around the eyes.
   - Hyperlipidemia: Elevated levels of lipids in the blood are common.

2. History and Physical Examination: A thorough medical history and physical examination are essential to rule out other causes of nephrotic syndrome and to assess the severity of symptoms.

Laboratory Tests

1. Urinalysis: A urinalysis will typically reveal:
   - Heavy proteinuria: The presence of protein in the urine is a hallmark of nephrotic syndrome.
   - Lipiduria: Fatty casts or free fat droplets may be observed.

2. Serum Tests: Important serum tests include:
   - Serum albumin: Low levels indicate hypoalbuminemia.
   - Lipid profile: Elevated cholesterol and triglycerides are often noted.

3. Complement Levels: In cases of dense deposit disease, complement levels (C3 and C4) may be assessed, as abnormalities can indicate the underlying pathology.

Imaging Studies

1. Renal Ultrasound: This imaging study can help assess kidney size and rule out structural abnormalities or obstructions.

Kidney Biopsy

A definitive diagnosis of dense deposit disease often requires a kidney biopsy, which allows for:
- Histological Examination: The biopsy will show characteristic findings, including:
- Dense deposits in the glomerular basement membrane.
- Proliferation of mesangial cells and other changes consistent with membranoproliferative glomerulonephritis.

Differential Diagnosis

It is crucial to differentiate dense deposit disease from other forms of nephrotic syndrome, such as minimal change disease or focal segmental glomerulosclerosis, as treatment and prognosis may differ significantly.

Conclusion

The diagnosis of nephrotic syndrome with dense deposit disease (ICD-10 code N04.6) is based on a combination of clinical symptoms, laboratory findings, imaging studies, and often a kidney biopsy. Accurate diagnosis is essential for appropriate management and treatment of the condition, which may include corticosteroids, immunosuppressive agents, and supportive care to manage symptoms and complications.

Nephrotic syndrome with dense deposit disease (DDD), classified under ICD-10 code N04.6, is a complex condition characterized by significant proteinuria, hypoalbuminemia, and edema, often associated with specific pathological changes in the kidney. The treatment of this condition requires a multifaceted approach tailored to the underlying causes and the severity of the symptoms.

Overview of Dense Deposit Disease

Dense deposit disease is a rare form of nephrotic syndrome that is often linked to abnormalities in the complement system, particularly involving the alternative pathway. It is characterized by the presence of dense deposits in the glomeruli, which can lead to significant kidney damage and dysfunction. Patients typically present with symptoms such as swelling, fatigue, and foamy urine due to high levels of protein.

Standard Treatment Approaches

1. Corticosteroids

Corticosteroids are often the first line of treatment for nephrotic syndrome, including DDD. They help reduce inflammation and proteinuria. The typical regimen may involve high-dose steroids initially, followed by a tapering schedule based on the patient's response. However, the effectiveness of steroids in DDD can vary, and some patients may not respond adequately[2].

2. Immunosuppressive Agents

For patients who do not respond to corticosteroids or have frequent relapses, additional immunosuppressive therapies may be considered. Common agents include:

• Mycophenolate mofetil (MMF): This medication is often used in pediatric and adult patients with DDD, particularly when steroid-sparing strategies are needed. It works by inhibiting lymphocyte proliferation and reducing immune-mediated damage to the kidneys[4].
• Cyclophosphamide: This is another option that may be used in cases of steroid-resistant nephrotic syndrome, although it is less commonly used due to its side effects.
• Calcineurin inhibitors (e.g., cyclosporine, tacrolimus): These agents can also be effective in managing nephrotic syndrome and may be used in conjunction with other therapies[2].

3. Supportive Care

Supportive care is crucial in managing symptoms and preventing complications associated with nephrotic syndrome:

• Diuretics: To manage edema, diuretics such as furosemide may be prescribed to help reduce fluid retention.
• Antihypertensive medications: If hypertension is present, medications such as ACE inhibitors or angiotensin receptor blockers (ARBs) can be beneficial, as they also help reduce proteinuria[1].
• Dietary modifications: A low-sodium diet may be recommended to help control edema, and protein intake may be adjusted based on the patient's kidney function and nutritional needs.

4. Management of Complications

Patients with DDD are at risk for various complications, including infections, thromboembolic events, and chronic kidney disease. Therefore, regular monitoring and preventive measures are essential:

• Vaccinations: Patients should be up to date on vaccinations to prevent infections, particularly pneumococcal and influenza vaccines.
• Monitoring renal function: Regular follow-up with kidney function tests is necessary to assess the progression of the disease and adjust treatment accordingly.

Conclusion

The management of nephrotic syndrome with dense deposit disease (ICD-10 code N04.6) involves a combination of corticosteroids, immunosuppressive agents, supportive care, and careful monitoring for complications. Given the complexity of the disease and the variability in patient response to treatment, a personalized approach is essential. Collaboration with a nephrologist is crucial for optimizing treatment strategies and improving patient outcomes. Regular follow-up and adjustments to the treatment plan based on the patient's response and side effects are vital for effective management.