ICD-10: N04.4

Nephrotic syndrome with diffuse endocapillary proliferative glomerulonephritis

Additional Information

Clinical Information

Nephrotic syndrome with diffuse endocapillary proliferative glomerulonephritis, classified under ICD-10 code N04.4, is a specific type of nephrotic syndrome characterized by a combination of nephrotic symptoms and the histological findings of diffuse endocapillary proliferation in the glomeruli. This condition can arise from various underlying causes, including infections, autoimmune diseases, and certain medications. Below is a detailed overview of its clinical presentation, signs, symptoms, and patient characteristics.

Clinical Presentation

Overview of Nephrotic Syndrome

Nephrotic syndrome is a clinical condition defined by a triad of symptoms:
1. Proteinuria: Excessive protein in the urine, typically greater than 3.5 grams per day.
2. Hypoalbuminemia: Low levels of albumin in the blood, often leading to edema.
3. Edema: Swelling, particularly in the legs, ankles, and around the eyes, due to fluid retention.

Specific Features of N04.4

In the case of nephrotic syndrome with diffuse endocapillary proliferative glomerulonephritis, the clinical presentation may include:

  • Acute or Subacute Onset: Symptoms may develop rapidly, often over days to weeks.
  • Hematuria: Presence of blood in the urine, which may be microscopic or grossly visible.
  • Hypertension: Elevated blood pressure is common due to fluid overload and renal impairment.
  • Decreased Renal Function: Patients may exhibit signs of acute kidney injury, with elevated serum creatinine levels.

Signs and Symptoms

Common Symptoms

  • Swelling (Edema): Particularly noticeable in the face, abdomen, and lower extremities.
  • Foamy Urine: Due to high protein content, urine may appear foamy.
  • Fatigue: Generalized weakness and fatigue due to fluid overload and anemia.
  • Weight Gain: Rapid weight gain due to fluid retention.

Physical Examination Findings

  • Pitting Edema: Swelling that leaves an indentation when pressed.
  • Hypertension: Blood pressure readings may be elevated.
  • Signs of Fluid Overload: Such as jugular venous distension or pulmonary crackles on auscultation.

Patient Characteristics

Demographics

  • Age: Nephrotic syndrome can occur at any age but is more common in children and young adults. In adults, it may be associated with underlying conditions such as diabetes or systemic lupus erythematosus.
  • Gender: There may be a slight male predominance in certain types of glomerulonephritis.

Risk Factors

  • Underlying Conditions: Conditions such as infections (e.g., hepatitis B, HIV), autoimmune diseases (e.g., lupus), and certain malignancies can predispose individuals to develop this form of nephrotic syndrome.
  • Medications: Some drugs, including nonsteroidal anti-inflammatory drugs (NSAIDs) and certain antibiotics, may trigger glomerulonephritis.

Laboratory Findings

  • Urinalysis: Shows significant proteinuria and may reveal red blood cells and casts.
  • Blood Tests: Typically show low serum albumin levels, elevated cholesterol, and possible renal impairment indicated by increased creatinine levels.

Conclusion

Nephrotic syndrome with diffuse endocapillary proliferative glomerulonephritis (ICD-10 code N04.4) presents with a distinct set of clinical features, including significant proteinuria, edema, and potential renal impairment. Understanding the signs, symptoms, and patient characteristics associated with this condition is crucial for timely diagnosis and management. Early intervention can help mitigate complications and improve patient outcomes. If you suspect nephrotic syndrome in a patient, a thorough clinical evaluation and appropriate laboratory testing are essential for confirming the diagnosis and determining the underlying cause.

Approximate Synonyms

Nephrotic syndrome with diffuse endocapillary proliferative glomerulonephritis, classified under ICD-10 code N04.4, is a specific condition characterized by a combination of nephrotic syndrome and a particular type of kidney inflammation. Understanding alternative names and related terms can enhance clarity in medical documentation and communication. Below are some alternative names and related terms associated with this condition.

Alternative Names

  1. Diffuse Endocapillary Proliferative Glomerulonephritis: This term refers specifically to the type of glomerulonephritis involved, highlighting the diffuse nature of the capillary proliferation within the glomeruli.

  2. Nephrotic Syndrome with Glomerulonephritis: A broader term that encompasses nephrotic syndrome occurring alongside various forms of glomerulonephritis, including the diffuse endocapillary type.

  3. Membranoproliferative Glomerulonephritis (MPGN): While not identical, MPGN can sometimes be used interchangeably in discussions about nephrotic syndrome, particularly when the proliferative aspect is emphasized.

  4. Proliferative Glomerulonephritis: A general term that describes any glomerulonephritis characterized by an increase in the number of cells within the glomeruli, which can include endocapillary proliferation.

  1. Nephrotic Syndrome: A condition defined by a set of symptoms including proteinuria, hypoalbuminemia, edema, and hyperlipidemia, which is a key component of N04.4.

  2. Glomerulonephritis: A general term for inflammation of the glomeruli, which can lead to nephrotic syndrome and encompasses various subtypes, including the one specified by N04.4.

  3. Kidney Disease: A broader category that includes all forms of kidney dysfunction, including nephrotic syndrome and glomerulonephritis.

  4. Chronic Kidney Disease (CKD): While N04.4 may not directly indicate CKD, nephrotic syndrome can lead to chronic kidney issues if not managed properly.

  5. Proteinuria: The presence of excess protein in the urine, a hallmark of nephrotic syndrome, which is relevant in the context of N04.4.

  6. Hypoalbuminemia: A condition characterized by low levels of albumin in the blood, often seen in patients with nephrotic syndrome.

  7. Edema: Swelling caused by excess fluid trapped in the body's tissues, commonly associated with nephrotic syndrome.

Understanding these alternative names and related terms can facilitate better communication among healthcare providers and improve patient education regarding nephrotic syndrome with diffuse endocapillary proliferative glomerulonephritis. This knowledge is crucial for accurate diagnosis, treatment planning, and coding in medical records.

Diagnostic Criteria

Nephrotic syndrome with diffuse endocapillary proliferative glomerulonephritis, classified under ICD-10 code N04.4, is a specific diagnosis that requires careful evaluation based on clinical criteria and laboratory findings. Below is a detailed overview of the criteria used for diagnosing this condition.

Understanding Nephrotic Syndrome

Nephrotic syndrome is characterized by a group of symptoms that indicate kidney damage, primarily involving:

  • Proteinuria: Excessive protein in the urine, typically greater than 3.5 grams per day.
  • Hypoalbuminemia: Low levels of albumin in the blood, often leading to edema.
  • Edema: Swelling, particularly in the legs, ankles, and around the eyes.
  • Hyperlipidemia: Elevated levels of lipids in the blood.

Specific Criteria for N04.4 Diagnosis

1. Clinical Presentation

Patients typically present with the following symptoms:

  • Swelling: Noticeable swelling in various parts of the body due to fluid retention.
  • Fatigue: General tiredness and weakness.
  • Foamy Urine: Due to high protein content, urine may appear foamy.

2. Laboratory Tests

Diagnosis of nephrotic syndrome with diffuse endocapillary proliferative glomerulonephritis involves several laboratory tests:

  • Urinalysis:
  • Proteinuria: A 24-hour urine collection showing more than 3.5 grams of protein is indicative of nephrotic syndrome.
  • Microscopic Examination: May reveal casts, red blood cells, or white blood cells, depending on the underlying cause.

  • Blood Tests:

  • Serum Albumin: Levels typically below 3.0 g/dL indicate hypoalbuminemia.
  • Lipid Profile: Elevated cholesterol and triglycerides are common in nephrotic syndrome.

3. Kidney Biopsy

A definitive diagnosis often requires a kidney biopsy, which can reveal:

  • Diffuse Endocapillary Proliferation: This histological finding is characterized by an increase in the number of cells within the capillary loops of the glomeruli, often associated with inflammatory processes.
  • Other Findings: Depending on the underlying cause, additional findings may include immune complex deposition or other specific patterns indicative of glomerulonephritis.

4. Exclusion of Other Conditions

It is crucial to rule out other causes of nephrotic syndrome, such as:

  • Diabetic Nephropathy
  • Minimal Change Disease
  • Focal Segmental Glomerulosclerosis
  • Secondary Causes: Such as infections, medications, or systemic diseases (e.g., lupus).

5. Clinical Guidelines

The diagnosis should align with established clinical guidelines, such as those from the Kidney Disease: Improving Global Outcomes (KDIGO) or the American College of Physicians, which provide comprehensive criteria for evaluating and managing nephrotic syndrome.

Conclusion

Diagnosing nephrotic syndrome with diffuse endocapillary proliferative glomerulonephritis (ICD-10 code N04.4) involves a combination of clinical assessment, laboratory tests, and often a kidney biopsy to confirm the histological findings. Proper diagnosis is essential for determining the appropriate treatment and management strategies for affected patients. If you have further questions or need additional information, feel free to ask!

Description

Nephrotic syndrome with diffuse endocapillary proliferative glomerulonephritis, classified under ICD-10 code N04.4, represents a specific type of kidney disorder characterized by a combination of nephrotic syndrome symptoms and a particular histological pattern observed in glomerular disease. Below is a detailed clinical description and relevant information regarding this condition.

Clinical Description

Nephrotic Syndrome Overview

Nephrotic syndrome is a clinical condition defined by a triad of symptoms:
- Proteinuria: Excessive protein in the urine, typically greater than 3.5 grams per day.
- Hypoalbuminemia: Low levels of albumin in the blood, leading to decreased oncotic pressure.
- Edema: Swelling due to fluid retention, often noticeable in the legs, abdomen, and around the eyes.

These symptoms arise from damage to the glomeruli, the filtering units of the kidneys, which leads to increased permeability and loss of proteins from the bloodstream.

Diffuse Endocapillary Proliferative Glomerulonephritis

Diffuse endocapillary proliferative glomerulonephritis is a specific type of glomerular inflammation characterized by:
- Proliferation of glomerular cells: This includes mesangial and endothelial cells, which can lead to thickening of the capillary walls.
- Endocapillary hypercellularity: An increase in the number of cells within the capillary loops, often seen in response to inflammatory stimuli.
- Immune complex deposition: This condition may be associated with various underlying causes, including infections, autoimmune diseases, or systemic conditions.

Etiology and Pathophysiology

The etiology of nephrotic syndrome with diffuse endocapillary proliferative glomerulonephritis can vary widely. Common causes include:
- Infections: Such as post-streptococcal glomerulonephritis.
- Autoimmune diseases: Including systemic lupus erythematosus (SLE).
- Vasculitis: Conditions that cause inflammation of blood vessels can also lead to this type of glomerulonephritis.

The pathophysiological mechanism involves immune-mediated injury to the glomeruli, leading to the clinical manifestations of nephrotic syndrome. The inflammatory process can result in significant kidney damage if not addressed promptly.

Diagnosis

Diagnosis typically involves:
- Urinalysis: To detect proteinuria and assess urine sediment.
- Blood tests: To evaluate serum albumin levels, kidney function (creatinine), and possible underlying causes (e.g., autoimmune markers).
- Kidney biopsy: This is often necessary to confirm the diagnosis and assess the specific type of glomerulonephritis, including the presence of endocapillary proliferation.

Treatment

Management of nephrotic syndrome with diffuse endocapillary proliferative glomerulonephritis focuses on:
- Corticosteroids: To reduce inflammation and immune response.
- Immunosuppressive agents: Such as cyclophosphamide or rituximab, especially in cases resistant to steroids.
- Supportive care: Including diuretics for edema, dietary modifications, and management of hypertension and hyperlipidemia.

Prognosis

The prognosis for patients with nephrotic syndrome and diffuse endocapillary proliferative glomerulonephritis varies based on the underlying cause, response to treatment, and the extent of kidney damage at the time of diagnosis. Early intervention is crucial for improving outcomes and preventing progression to chronic kidney disease.

In summary, ICD-10 code N04.4 encapsulates a complex interplay of nephrotic syndrome and specific glomerular pathology, necessitating a comprehensive approach to diagnosis and management to optimize patient outcomes.

Treatment Guidelines

Nephrotic syndrome with diffuse endocapillary proliferative glomerulonephritis, classified under ICD-10 code N04.4, is a complex condition characterized by significant proteinuria, hypoalbuminemia, and edema, often resulting from various underlying causes, including infections, autoimmune diseases, and certain medications. The management of this condition typically involves a multifaceted approach aimed at addressing both the symptoms and the underlying causes.

Standard Treatment Approaches

1. Pharmacological Interventions

Corticosteroids

Corticosteroids are often the first line of treatment for nephrotic syndrome, particularly when the condition is associated with glomerulonephritis. Prednisone is commonly prescribed to reduce inflammation and proteinuria. The dosage and duration depend on the severity of the disease and the patient's response to treatment[2].

Immunosuppressive Agents

In cases where corticosteroids are ineffective or if the patient experiences frequent relapses, additional immunosuppressive medications may be introduced. These can include:
- Cyclophosphamide: Often used in cases of severe nephrotic syndrome.
- Calcineurin inhibitors (e.g., cyclosporine, tacrolimus): These are effective in reducing proteinuria and preserving kidney function.
- Mycophenolate mofetil: Another option for patients who do not respond to steroids[1][2].

2. Management of Edema and Hypertension

Diuretics

To manage edema, diuretics such as furosemide (Lasix) are frequently prescribed. These medications help to reduce fluid overload and alleviate symptoms of swelling[1].

Antihypertensive Medications

Patients with nephrotic syndrome often experience hypertension. Angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs) are beneficial not only for controlling blood pressure but also for reducing proteinuria[1][2].

3. Dietary Modifications

Low-Sodium Diet

A low-sodium diet is recommended to help manage edema and hypertension. Reducing salt intake can significantly decrease fluid retention and improve overall symptoms[1].

Protein Intake

While patients with nephrotic syndrome may need to limit protein intake to reduce the burden on the kidneys, it is essential to ensure adequate protein consumption to prevent malnutrition, especially in cases of significant protein loss[2].

4. Monitoring and Supportive Care

Regular Monitoring

Frequent follow-up appointments are crucial for monitoring kidney function, protein levels, and the effectiveness of treatment. This may include regular urinalysis and blood tests to assess kidney function and electrolyte balance[1].

Patient Education

Educating patients about their condition, treatment options, and lifestyle modifications is vital for effective management. Patients should be informed about the signs of complications, such as infections or worsening kidney function, and the importance of adhering to prescribed therapies[2].

5. Addressing Underlying Causes

If the nephrotic syndrome is secondary to an underlying condition (e.g., lupus, diabetes), treating that condition is essential. This may involve specific therapies tailored to the underlying disease, such as antimalarials for lupus or strict glycemic control in diabetic patients[1][2].

Conclusion

The management of nephrotic syndrome with diffuse endocapillary proliferative glomerulonephritis (ICD-10 code N04.4) requires a comprehensive approach that includes pharmacological treatment, dietary modifications, and regular monitoring. By addressing both the symptoms and the underlying causes, healthcare providers can help improve patient outcomes and quality of life. Ongoing research and clinical trials continue to explore new therapeutic options, which may further enhance treatment strategies in the future.

Related Information

Clinical Information

  • Proteinuria >3.5g/day
  • Hypoalbuminemia and edema
  • Acute or subacute onset
  • Hematuria and hypertension
  • Decreased renal function
  • Swelling, particularly in legs and face
  • Foamy urine due to high protein content
  • Fatigue and weight gain due to fluid overload
  • Pitting edema and signs of fluid overload
  • Elevated blood pressure readings
  • Significant proteinuria on urinalysis
  • Low serum albumin levels on blood tests

Approximate Synonyms

  • Diffuse Endocapillary Proliferative Glomerulonephritis
  • Nephrotic Syndrome with Glomerulonephritis
  • Membranoproliferative Glomerulonephritis (MPGN)
  • Proliferative Glomerulonephritis
  • Kidney Disease
  • Chronic Kidney Disease (CKD)

Diagnostic Criteria

  • Proteinuria > 3.5g per day
  • Hypoalbuminemia < 3.0 g/dL
  • Edema and swelling in various parts of body
  • Hyperlipidemia with elevated cholesterol and triglycerides
  • Diffuse endocapillary proliferation on kidney biopsy
  • Exclusion of other nephrotic syndrome causes

Description

  • Nephrotic syndrome symptoms
  • Diffuse endocapillary proliferative glomerulonephritis
  • Proteinuria and hypoalbuminemia
  • Edema and fluid retention
  • Glomerular inflammation and damage
  • Immune complex deposition
  • Infections, autoimmune diseases, and vasculitis as causes

Treatment Guidelines

  • Corticosteroids first line treatment
  • Prednisone commonly prescribed
  • Immunosuppressive agents used if steroids ineffective
  • Cyclophosphamide used for severe cases
  • Calcineurin inhibitors reduce proteinuria and kidney damage
  • Mycophenolate mofetil for non-responsive patients
  • Furosemide diuretics manage edema
  • ACE inhibitors or ARBs control hypertension
  • Low-sodium diet helps manage edema and hypertension
  • Adequate protein intake essential to prevent malnutrition
  • Regular monitoring of kidney function and protein levels

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It is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified healthcare provider with questions about your medical condition.