ICD-10: N04

Nephrotic syndrome

Clinical Information

Includes

  • congenital nephrotic syndrome
  • lipoid nephrosis

Additional Information

Description

Nephrotic syndrome is a significant clinical condition characterized by a group of symptoms that indicate kidney dysfunction, particularly affecting the glomeruli, which are the filtering units of the kidneys. The ICD-10 code N04 specifically pertains to nephrotic syndrome, and it is essential to understand its clinical description, causes, symptoms, and classifications.

Clinical Description of Nephrotic Syndrome (ICD-10 Code N04)

Nephrotic syndrome is defined by a triad of clinical features:

  1. Proteinuria: This is the presence of excess protein in the urine, typically greater than 3.5 grams per day. It results from damage to the glomerular filtration barrier, allowing proteins, particularly albumin, to leak into the urine.

  2. Hypoalbuminemia: This refers to low levels of albumin in the blood, which occurs due to the loss of protein through urine. Albumin is crucial for maintaining oncotic pressure, and its deficiency can lead to fluid accumulation in tissues.

  3. Edema: Patients often experience swelling, particularly in the legs, ankles, and around the eyes, due to fluid retention. This is exacerbated by the low oncotic pressure caused by hypoalbuminemia.

  4. Hyperlipidemia: Increased levels of lipids in the blood are common, as the liver compensates for low protein levels by producing more lipoproteins.

Causes of Nephrotic Syndrome

Nephrotic syndrome can be classified into primary and secondary forms:

  • Primary Nephrotic Syndrome: This includes conditions that originate in the kidneys, such as:
  • Minimal Change Disease: The most common cause in children, characterized by a lack of visible changes in kidney tissue under a microscope.
  • Focal Segmental Glomerulosclerosis (FSGS): A condition that causes scarring in some parts of the kidney's filtering units.
  • Membranous Nephropathy: Involves thickening of the glomerular membrane.

  • Secondary Nephrotic Syndrome: This occurs due to systemic diseases or conditions, such as:

  • Diabetes Mellitus: Leading to diabetic nephropathy.
  • Lupus Nephritis: Associated with systemic lupus erythematosus.
  • Infections: Such as HIV or hepatitis B and C.
  • Medications: Certain drugs can induce nephrotic syndrome.

Symptoms and Diagnosis

Patients with nephrotic syndrome may present with:

  • Swelling (edema) in various body parts.
  • Foamy urine due to high protein content.
  • Weight gain due to fluid retention.
  • Fatigue and general malaise.

Diagnosis typically involves:

  • Urinalysis: To detect proteinuria.
  • Blood tests: To assess albumin levels and kidney function.
  • Kidney biopsy: Sometimes necessary to determine the underlying cause.

Classification of Nephrotic Syndrome

The ICD-10 code N04 is further divided into specific categories based on the underlying causes and morphological changes:

  • N04.0: Nephrotic syndrome with minimal change disease.
  • N04.1: Nephrotic syndrome with focal segmental glomerulosclerosis.
  • N04.2: Nephrotic syndrome with membranous nephropathy.
  • N04.8: Nephrotic syndrome with other morphologic changes.
  • N04.9: Nephrotic syndrome, unspecified.

Treatment and Management

Management of nephrotic syndrome focuses on addressing the underlying cause, controlling symptoms, and preventing complications. Treatment options may include:

  • Corticosteroids: Often the first line of treatment for primary nephrotic syndrome.
  • Immunosuppressive agents: For steroid-resistant cases or secondary nephrotic syndrome.
  • Diuretics: To manage edema.
  • ACE inhibitors or ARBs: To reduce proteinuria and protect kidney function.
  • Dietary modifications: Such as low-sodium diets to manage fluid retention.

Conclusion

Nephrotic syndrome, classified under ICD-10 code N04, is a complex condition that requires careful diagnosis and management. Understanding its clinical features, causes, and treatment options is crucial for healthcare providers to effectively support patients suffering from this condition. Early intervention can significantly improve outcomes and quality of life for those affected.

Clinical Information

Nephrotic syndrome is a clinical condition characterized by a group of symptoms that indicate significant kidney dysfunction, particularly affecting the glomeruli. The ICD-10 code N04 encompasses various forms of nephrotic syndrome, which can arise from different underlying causes. Understanding the clinical presentation, signs, symptoms, and patient characteristics associated with this syndrome is crucial for diagnosis and management.

Clinical Presentation

Nephrotic syndrome typically presents with a classic triad of symptoms:

  1. Proteinuria: This is the hallmark of nephrotic syndrome, where there is an excessive amount of protein in the urine, often exceeding 3.5 grams per day. This condition is usually detected through urine tests that show a high protein-to-creatinine ratio or through 24-hour urine collection.

  2. Hypoalbuminemia: Due to the loss of protein in the urine, patients often exhibit low levels of albumin in the blood. This can lead to a decrease in oncotic pressure, contributing to fluid retention.

  3. Edema: Patients commonly experience swelling, particularly in the legs, ankles, and around the eyes (periorbital edema). This is a direct consequence of fluid retention due to low serum albumin levels.

Signs and Symptoms

In addition to the classic triad, nephrotic syndrome may present with various other signs and symptoms:

  • Weight Gain: Due to fluid retention, patients may notice a rapid increase in weight.
  • Fatigue: Generalized fatigue and malaise are common as the body struggles with the effects of protein loss and fluid imbalance.
  • Hypertension: Some patients may develop high blood pressure as a compensatory mechanism due to fluid overload.
  • Hyperlipidemia: Increased levels of lipids in the blood are often observed, as the liver compensates for low protein levels by increasing lipid production.
  • Increased Susceptibility to Infections: The loss of immunoglobulins in the urine can lead to a weakened immune response, making patients more prone to infections.

Patient Characteristics

Nephrotic syndrome can affect individuals of all ages, but certain characteristics may be more prevalent in specific populations:

  • Age: In children, nephrotic syndrome is often idiopathic and may be associated with minimal change disease, while in adults, it can be secondary to conditions such as diabetes mellitus or systemic lupus erythematosus.
  • Gender: There is a slight male predominance in childhood cases, whereas adult cases show a more equal distribution between genders.
  • Underlying Conditions: Patients with a history of diabetes, hypertension, or autoimmune diseases are at a higher risk of developing nephrotic syndrome. Additionally, certain genetic factors may predispose individuals to hereditary forms of the syndrome.

Conclusion

Nephrotic syndrome, classified under ICD-10 code N04, presents with a distinct set of clinical features, including significant proteinuria, hypoalbuminemia, and edema. Recognizing these signs and symptoms is essential for timely diagnosis and management. The syndrome can affect individuals across various age groups, with specific characteristics and underlying conditions influencing its presentation. Early intervention and appropriate treatment can help manage symptoms and prevent complications associated with nephrotic syndrome.

Approximate Synonyms

Nephrotic syndrome, classified under ICD-10 code N04, is a kidney disorder characterized by a group of symptoms that include proteinuria, low blood protein levels, high cholesterol, and swelling. Understanding the alternative names and related terms for this condition can enhance clarity in medical documentation and communication. Below are some of the key alternative names and related terms associated with ICD-10 code N04.

Alternative Names for Nephrotic Syndrome

  1. Nephrosis: This term is often used interchangeably with nephrotic syndrome, although it can refer to a broader category of kidney diseases that do not involve inflammation.

  2. Minimal Change Disease: This is a common cause of nephrotic syndrome, particularly in children, where the kidneys appear normal under a microscope but have significant functional impairment.

  3. Focal Segmental Glomerulosclerosis (FSGS): This condition is another specific type of nephrotic syndrome characterized by scarring in some parts of the kidney's filtering units.

  4. Membranous Nephropathy: This is a specific type of nephrotic syndrome that involves thickening of the membranes within the kidney's filtering units.

  5. Lipoid Nephrosis: This term is sometimes used to describe nephrotic syndrome, particularly in pediatric cases, where there is a significant presence of lipids in the urine.

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

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

  3. Edema: Swelling caused by excess fluid trapped in the body's tissues, frequently observed in nephrotic syndrome patients.

  4. Hyperlipidemia: Elevated levels of lipids in the blood, which often occur as a compensatory response to low protein levels in nephrotic syndrome.

  5. Glomerular Filtration Rate (GFR): A test used to assess kidney function, which may be affected in patients with nephrotic syndrome.

  6. Secondary Nephrotic Syndrome: This term refers to nephrotic syndrome that arises as a result of another underlying condition, such as diabetes or lupus.

Conclusion

Understanding the alternative names and related terms for nephrotic syndrome (ICD-10 code N04) is crucial for healthcare professionals in accurately diagnosing and documenting this condition. The terminology can vary based on the underlying cause and specific characteristics of the syndrome, which can aid in effective communication and treatment planning. If you have further questions or need more detailed information about nephrotic syndrome, feel free to ask!

Diagnostic Criteria

Nephrotic syndrome is a clinical condition characterized by a specific set of symptoms and laboratory findings. The ICD-10 code N04 is used to classify nephrotic syndrome, which can be further specified based on its underlying causes and characteristics. Here’s a detailed overview of the criteria used for diagnosing nephrotic syndrome, particularly in relation to the ICD-10 code N04.

Diagnostic Criteria for Nephrotic Syndrome

1. Clinical Symptoms

The diagnosis of nephrotic syndrome typically involves the presence of the following clinical symptoms:

  • Proteinuria: This is the hallmark of nephrotic syndrome, defined as the excretion of more than 3.5 grams of protein in a 24-hour urine collection. It can also be assessed through a urine dipstick test showing 3+ or 4+ protein[4][10].

  • Edema: Patients often present with significant swelling, particularly in the legs, ankles, and around the eyes. This occurs due to fluid retention resulting from low serum albumin levels[4][9].

  • Hypoalbuminemia: A serum albumin level of less than 3.0 g/dL is typically observed, indicating a loss of protein from the bloodstream[4][10].

  • Hyperlipidemia: Elevated levels of lipids in the blood are common, often manifesting as increased cholesterol and triglycerides[4][9].

2. Laboratory Findings

In addition to clinical symptoms, specific laboratory tests are crucial for confirming the diagnosis:

  • Urinalysis: A 24-hour urine collection is performed to quantify protein loss. A urine protein-to-creatinine ratio can also be used as a more convenient alternative[4][10].

  • Serum Tests: Blood tests are conducted to measure serum albumin levels, lipid profiles, and kidney function (e.g., serum creatinine) to assess the overall health of the kidneys and the extent of nephron damage[4][9].

3. Exclusion of Other Conditions

It is essential to rule out other potential causes of proteinuria and edema, such as:

  • Diabetic nephropathy
  • Lupus nephritis
  • Hypertensive nephrosclerosis
  • Acute kidney injury

This often involves additional tests, including imaging studies or kidney biopsy, to determine the underlying etiology of the nephrotic syndrome[4][10].

4. Classification of Nephrotic Syndrome

The ICD-10 code N04 encompasses various types of nephrotic syndrome, which can be classified based on their underlying causes:

  • Primary Nephrotic Syndrome: This includes idiopathic forms such as Minimal Change Disease and Focal Segmental Glomerulosclerosis (FSGS).

  • Secondary Nephrotic Syndrome: This can result from systemic diseases like diabetes mellitus, systemic lupus erythematosus, or infections[4][9].

5. ICD-10 Specific Codes

The ICD-10 classification for nephrotic syndrome includes several specific codes, such as:

  • N04.0: Nephrotic syndrome with diffuse glomerular lesions
  • N04.1: Nephrotic syndrome with focal segmental glomerulosclerosis
  • N04.2: Nephrotic syndrome with minimal change disease
  • N04.9: Nephrotic syndrome, unspecified[1][12].

Conclusion

The diagnosis of nephrotic syndrome (ICD-10 code N04) is based on a combination of clinical symptoms, laboratory findings, and the exclusion of other conditions. The presence of significant proteinuria, edema, hypoalbuminemia, and hyperlipidemia are critical indicators. Understanding these criteria is essential for healthcare providers to accurately diagnose and manage patients with nephrotic syndrome, ensuring appropriate treatment and monitoring of potential complications.

Treatment Guidelines

Nephrotic syndrome, classified under ICD-10 code N04, is a kidney disorder characterized by a group of symptoms including proteinuria (excess protein in urine), hypoalbuminemia (low levels of albumin in the blood), edema (swelling), and hyperlipidemia (high levels of lipids in the blood) [1]. The management of nephrotic syndrome varies based on its underlying cause, age of the patient, and severity of symptoms. Here’s a detailed overview of standard treatment approaches for nephrotic syndrome.

Initial Assessment and Diagnosis

Before initiating treatment, a thorough assessment is essential. This includes:

  • Clinical Evaluation: Assessing symptoms such as swelling, fatigue, and changes in urine output.
  • Laboratory Tests: Urinalysis to check for protein levels, blood tests to measure albumin and lipid levels, and kidney function tests.
  • Imaging Studies: Ultrasound may be used to evaluate kidney structure and rule out other conditions.

Treatment Approaches

1. Medications

Corticosteroids

Corticosteroids, such as prednisone, are often the first line of treatment, especially in cases of idiopathic nephrotic syndrome. They help reduce inflammation and proteinuria by suppressing the immune response [2].

  • Dosage: Typically, high doses are given initially, followed by a tapering schedule based on response.
  • Duration: Treatment usually lasts for several weeks to months, depending on the patient's response.

Immunosuppressants

For patients who are steroid-resistant or have frequent relapses, immunosuppressive agents may be used. These include:

  • Mycophenolate mofetil: Often used in pediatric patients and those with steroid-resistant nephrotic syndrome [3].
  • Cyclophosphamide: Another option, particularly in adults, though it has more side effects.
  • Calcineurin Inhibitors: Such as cyclosporine or tacrolimus, which can be effective in reducing proteinuria.

2. Supportive Care

Diuretics

Diuretics are commonly prescribed to manage edema. They help the body eliminate excess fluid, thus reducing swelling [4].

  • Types: Loop diuretics (e.g., furosemide) are frequently used due to their effectiveness.

Dietary Modifications

Dietary changes can also play a crucial role in managing nephrotic syndrome:

  • Low-Sodium Diet: Reducing salt intake helps control edema.
  • Protein Intake: While protein intake may need to be adjusted based on kidney function, a moderate protein diet is often recommended to prevent malnutrition while managing proteinuria.

3. Management of Complications

Hyperlipidemia

Patients with nephrotic syndrome often experience elevated lipid levels. Statins or other lipid-lowering agents may be prescribed to manage this condition [5].

Infection Prevention

Due to the loss of immunoglobulins in urine, patients are at increased risk for infections. Vaccinations and prophylactic antibiotics may be considered, especially in children [6].

4. Monitoring and Follow-Up

Regular follow-up is essential to monitor kidney function, protein levels, and overall health. Adjustments to treatment may be necessary based on the patient's response and any side effects experienced.

Conclusion

The management of nephrotic syndrome (ICD-10 code N04) involves a multifaceted approach that includes corticosteroids, immunosuppressants, supportive care, and monitoring for complications. The specific treatment plan should be tailored to the individual patient, considering the underlying cause of the syndrome and their overall health status. Ongoing research continues to refine these treatment strategies, aiming for better outcomes and quality of life for patients affected by this condition.

References

  1. ICD-10 Codes to Report Kidney-related Illnesses.
  2. Nephrotic Syndrome | 5-Minute Clinical Consult.
  3. Use of mycophenolate mofetil in patients with pediatric and adult nephrotic syndrome.
  4. Clinical course & management of childhood nephrotic syndrome.
  5. Management of hyperlipidemia in nephrotic syndrome.
  6. Infection prevention strategies in nephrotic syndrome patients.

Related Information

Description

  • Proteinuria present in urine
  • Hypoalbuminemia causing low blood albumin
  • Edema leading to swelling in legs
  • Hyperlipidemia with increased blood lipids
  • Primary nephrotic syndrome classified into conditions
  • Secondary nephrotic syndrome caused by systemic diseases
  • Diabetes mellitus a common cause of secondary nephrotic syndrome
  • Lupus nephritis associated with systemic lupus erythematosus
  • Infections like HIV or hepatitis B/C can induce nephrotic syndrome
  • Medications can also lead to nephrotic syndrome

Clinical Information

  • Significant kidney dysfunction
  • Proteinuria exceeding 3.5 grams per day
  • Hypoalbuminemia leading to fluid retention
  • Edema particularly in legs, ankles and around eyes
  • Weight gain due to fluid retention
  • Fatigue and malaise due to protein loss and fluid imbalance
  • Hypertension as a compensatory mechanism for fluid overload
  • Hyperlipidemia due to increased lipid production by liver
  • Increased susceptibility to infections due to immunoglobulin loss

Approximate Synonyms

  • Nephrosis
  • Minimal Change Disease
  • Focal Segmental Glomerulosclerosis (FSGS)
  • Membranous Nephropathy
  • Lipoid Nephrosis
  • Proteinuria
  • Hypoalbuminemia
  • Edema
  • Hyperlipidemia

Diagnostic Criteria

  • Proteinuria >3.5g/24hr
  • Significant edema
  • Hypoalbuminemia <3.0 g/dL
  • Hyperlipidemia
  • Urinalysis with protein quantification
  • Serum tests for albumin, lipids, and kidney function
  • Exclusion of other conditions like diabetic nephropathy

Treatment Guidelines

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