ICD-10: N04.7

Nephrotic syndrome with diffuse crescentic glomerulonephritis, classified under ICD-10 code N04.7, is a specific condition characterized by a combination of nephrotic syndrome and the presence of crescentic glomerulonephritis. The diagnosis of this condition involves several criteria, which can be categorized into clinical, laboratory, and histopathological findings.

Clinical Criteria

1. Symptoms of Nephrotic Syndrome: The primary clinical features include:
   - Proteinuria: Typically greater than 3.5 grams per day, indicating significant protein loss in urine.
   - Edema: Swelling, particularly in the legs, ankles, and around the eyes, due to fluid retention.
   - Hypoalbuminemia: Low levels of albumin in the blood, often below 3 g/dL, resulting from protein loss.
   - Hyperlipidemia: Elevated levels of lipids in the blood, which can occur as a compensatory response to low albumin levels.

2. Acute Kidney Injury: Patients may present with signs of acute kidney injury, which can manifest as elevated serum creatinine levels and reduced urine output.

Laboratory Criteria

1. Urinalysis: A urinalysis will typically show:
   - Heavy Proteinuria: The presence of protein in the urine, often assessed using a urine dipstick or 24-hour urine collection.
   - Hematuria: The presence of blood in the urine may also be noted, although it is not always present.

2. Serum Tests: Blood tests are essential to evaluate:
   - Serum Creatinine: To assess kidney function.
   - Serum Albumin: To confirm hypoalbuminemia.
   - Lipid Profile: To check for hyperlipidemia.

Histopathological Criteria

1. Kidney Biopsy: A definitive diagnosis often requires a kidney biopsy, which may reveal:
   - Crescentic Glomerulonephritis: The presence of crescents in the glomeruli, indicating severe glomerular injury.
   - Glomerular Damage: Other forms of glomerular damage may also be observed, depending on the underlying cause of the crescentic glomerulonephritis.

2. Immunofluorescence and Electron Microscopy: These techniques may be used to identify specific patterns of immune complex deposition or other abnormalities that can help determine the underlying etiology of the nephrotic syndrome.

Conclusion

The diagnosis of nephrotic syndrome with diffuse crescentic glomerulonephritis (ICD-10 code N04.7) is multifaceted, requiring a combination of clinical symptoms, laboratory findings, and histopathological evidence. Early and accurate diagnosis is crucial for effective management and treatment of the underlying causes, which may include autoimmune diseases, infections, or other systemic conditions. If you have further questions or need more specific information, feel free to ask!

Nephrotic syndrome with diffuse crescentic glomerulonephritis, classified under ICD-10 code N04.7, represents a specific and serious form of kidney disease characterized by a combination of nephrotic syndrome and crescentic glomerulonephritis. This condition is significant due to its implications for kidney function and overall health.

Clinical Description

Nephrotic Syndrome

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 edema.
- Edema: Swelling, particularly in the legs, ankles, and around the eyes, due to fluid retention.

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 into the urine[1][2].

Crescentic Glomerulonephritis

Crescentic glomerulonephritis is a severe form of glomerulonephritis characterized by the presence of crescent-shaped cellular formations in the glomeruli. This condition often indicates rapid progression to kidney failure and is associated with significant inflammation and damage to the kidney tissue. The crescents are formed by the proliferation of epithelial cells and inflammatory cells in response to severe injury to the glomeruli[3][4].

Combined Condition

When nephrotic syndrome occurs alongside diffuse crescentic glomerulonephritis, it suggests a particularly aggressive form of kidney disease. The presence of crescents indicates that the glomerular injury is acute and severe, often leading to a rapid decline in kidney function. Patients may experience symptoms such as:
- Severe edema
- Hypertension
- Hematuria (blood in urine)
- Oliguria (reduced urine output)

Diagnosis and Management

Diagnosis typically involves a combination of clinical evaluation, laboratory tests (including urinalysis and serum albumin levels), and imaging studies. A kidney biopsy may be necessary to confirm the diagnosis and assess the extent of glomerular damage[5][6].

Management of nephrotic syndrome with diffuse crescentic glomerulonephritis often requires:
- Immunosuppressive therapy: To reduce inflammation and prevent further damage to the kidneys.
- Corticosteroids: Commonly used to manage inflammation.
- Supportive care: Including diuretics for edema, antihypertensive medications, and dietary modifications.

In severe cases, renal replacement therapy (dialysis or kidney transplantation) may be necessary if kidney function deteriorates significantly[7][8].

Conclusion

ICD-10 code N04.7 encapsulates a complex and serious condition that necessitates prompt diagnosis and aggressive management to prevent irreversible kidney damage. Understanding the interplay between nephrotic syndrome and crescentic glomerulonephritis is crucial for healthcare providers in delivering effective care and improving patient outcomes.

References

1. ICD-10-CM Code for Nephrotic syndrome N04.
2. Nephrotic Syndrome | 5-Minute Clinical Consult.
3. ICD-10 Codes to Report Kidney-related Illnesses.
4. Nephrotic syndrome (N04) - ICD List.
5. 2025 ICD-10-CM Diagnosis Code N04.7 - The Web's Free 2023 ICD-10-CM/PCS.
6. End Stage Renal Disease Medical Evidence Report.
7. Chronic Renal Disease Program, Enrollment Application.
8. ITAP TT1 MC - Chronic Renal Failure – June 2016.

Nephrotic syndrome with diffuse crescentic glomerulonephritis, classified under ICD-10 code N04.7, represents a specific and severe form of kidney disease characterized by a combination of nephrotic syndrome features and crescentic glomerulonephritis. Understanding the clinical presentation, signs, symptoms, and patient characteristics associated with this condition is crucial for effective diagnosis and management.

Clinical Presentation

Overview of Nephrotic Syndrome

Nephrotic syndrome is primarily defined by a triad of clinical features:
- 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, due to fluid retention.

Crescentic Glomerulonephritis

Crescentic glomerulonephritis is characterized by the presence of crescent-shaped structures in the glomeruli, indicating severe inflammation and damage. This condition can lead to rapid progression to kidney failure if not treated promptly.

Signs and Symptoms

Common Symptoms

Patients with nephrotic syndrome with diffuse crescentic glomerulonephritis may present with:
- Severe Edema: Notable swelling in various body parts, particularly the face and lower extremities.
- Foamy Urine: Due to high protein content, urine may appear foamy.
- Fatigue: Generalized weakness and fatigue are common due to fluid imbalance and anemia.
- Hypertension: Elevated blood pressure may occur as a result of fluid overload and kidney dysfunction.

Additional Symptoms

• Hematuria: Blood in the urine may be present, indicating glomerular damage.
• Oliguria: Reduced urine output can occur as kidney function declines.
• Nausea and Vomiting: These symptoms may arise from metabolic disturbances or fluid overload.

Patient Characteristics

Demographics

• Age: Nephrotic syndrome can occur at any age but is more common in children and young adults. Crescentic glomerulonephritis is often seen in adults.
• Gender: There may be a slight male predominance in certain types of glomerulonephritis.

Risk Factors

• Underlying Conditions: Conditions such as systemic lupus erythematosus, vasculitis, or infections can predispose individuals to develop crescentic glomerulonephritis.
• Genetic Factors: A family history of kidney disease may increase risk.

Laboratory Findings

• Urinalysis: Significant proteinuria and possible hematuria.
• Blood Tests: Low serum albumin levels, elevated creatinine, and possible electrolyte imbalances.
• Imaging: Renal ultrasound may show kidney enlargement or other abnormalities.

Conclusion

Nephrotic syndrome with diffuse crescentic glomerulonephritis is a complex condition that requires careful clinical evaluation and management. Recognizing the signs and symptoms, along with understanding patient characteristics, is essential for timely diagnosis and intervention. Early treatment can significantly impact the prognosis and help prevent progression to end-stage renal disease. Regular monitoring and a multidisciplinary approach involving nephrologists, dietitians, and primary care providers are crucial for optimal patient outcomes.

ICD-10 code N04.7 refers specifically to "Nephrotic syndrome with diffuse crescentic glomerulonephritis." This condition is characterized by the presence of nephrotic syndrome alongside a specific type of kidney inflammation known as crescentic glomerulonephritis. Below are alternative names and related terms that can be associated with this diagnosis.

Alternative Names

1. Crescentic Glomerulonephritis: This term highlights the primary pathological feature of the condition, which is the formation of crescents in the glomeruli, indicating severe inflammation.

2. Rapidly Progressive Glomerulonephritis (RPGN): This is a broader term that encompasses various types of glomerulonephritis that lead to rapid loss of kidney function, including those with crescent formation.

3. Nephrotic Syndrome with Crescentic Glomerulonephritis: A more descriptive term that specifies the combination of nephrotic syndrome and the crescentic form of glomerulonephritis.

4. Diffuse Crescentic Glomerulonephritis: This term focuses on the diffuse nature of the crescentic changes observed in the glomeruli.

Related Terms

1. Nephrotic Syndrome: A condition characterized by significant proteinuria, hypoalbuminemia, and edema, which can occur due to various underlying causes, including glomerulonephritis.

2. Glomerulonephritis: A general term for inflammation of the glomeruli, which can be caused by a variety of factors, including infections, autoimmune diseases, and systemic conditions.

3. Immunological Disorders: Conditions such as systemic lupus erythematosus (SLE) or vasculitis that can lead to crescentic glomerulonephritis.

4. Kidney Failure: A potential outcome of untreated or severe nephrotic syndrome with crescentic glomerulonephritis, where the kidneys lose their ability to function effectively.

5. Proteinuria: The presence of excess protein in the urine, a hallmark of nephrotic syndrome.

6. Hematuria: The presence of blood in the urine, which may accompany glomerulonephritis.

Understanding these alternative names and related terms can help in the clinical context, as they provide insight into the nature of the condition and its implications for patient management and treatment. If you need further details or specific information regarding treatment options or prognosis, feel free to ask!

Nephrotic syndrome with diffuse crescentic glomerulonephritis, classified under ICD-10 code N04.7, represents a serious kidney condition characterized by significant proteinuria, edema, and the presence of crescent-shaped lesions in the glomeruli. This condition often requires a multifaceted treatment approach aimed at managing symptoms, addressing the underlying cause, and preventing complications. Below, we explore the standard treatment strategies for this condition.

Overview of Nephrotic Syndrome with Diffuse Crescentic Glomerulonephritis

Nephrotic syndrome is a collection of symptoms that indicate kidney damage, primarily involving excessive protein loss in urine, low blood protein levels, high cholesterol, and swelling. When associated with diffuse crescentic glomerulonephritis, it indicates a more severe form of kidney injury, often linked to autoimmune diseases or infections. The presence of crescents in the glomeruli signifies a rapid progression of kidney damage, necessitating prompt and aggressive treatment.

Standard Treatment Approaches

1. Corticosteroids

Corticosteroids, such as prednisone, are often the first line of treatment for nephrotic syndrome. They help reduce inflammation and suppress the immune response, which can be particularly beneficial in cases where the syndrome is secondary to autoimmune conditions. The dosage and duration of corticosteroid therapy depend on the severity of the disease and the patient's response to treatment[2].

2. Immunosuppressive Agents

In cases where corticosteroids alone are insufficient, or if the patient has a severe form of the disease, additional immunosuppressive medications may be prescribed. Common agents include:

• Cyclophosphamide: Often used in conjunction with corticosteroids for its ability to further suppress the immune system.
• Mycophenolate mofetil: An alternative that may be used for patients who cannot tolerate cyclophosphamide.
• Rituximab: A monoclonal antibody that targets CD20 on B cells, used in some cases of refractory nephrotic syndrome[3].

3. Angiotensin-Converting Enzyme (ACE) Inhibitors

ACE inhibitors, such as lisinopril, are frequently prescribed to manage hypertension and reduce proteinuria. They work by relaxing blood vessels, which can help decrease the pressure in the kidneys and reduce the amount of protein lost in urine[4].

4. Diuretics

Diuretics are often used to manage edema associated with nephrotic syndrome. They help the body eliminate excess fluid, thereby reducing swelling. Common diuretics include furosemide and spironolactone, which can be used based on the patient's specific needs[5].

5. Dietary Modifications

Dietary changes can play a supportive role in managing nephrotic syndrome. A low-sodium diet is often recommended to help control edema, while a diet rich in protein may be necessary to counteract protein loss, although this should be tailored to the individual’s kidney function and overall health status[6].

6. Management of Complications

Patients with nephrotic syndrome are at risk for various complications, including infections, thromboembolism, and chronic kidney disease. Prophylactic measures, such as vaccinations and anticoagulation therapy, may be necessary depending on the patient's risk factors and clinical status[7].

Conclusion

The management of nephrotic syndrome with diffuse crescentic glomerulonephritis requires a comprehensive approach that includes corticosteroids, immunosuppressive agents, ACE inhibitors, diuretics, and dietary modifications. Close monitoring and adjustment of treatment based on the patient's response and any emerging complications are crucial for optimizing outcomes. Collaboration among nephrologists, dietitians, and primary care providers is essential to ensure a holistic approach to care for patients with this complex condition.