ICD-10: N07.5

Hereditary nephropathy, not elsewhere classified with diffuse mesangiocapillary glomerulonephritis

Clinical Information

Inclusion Terms

  • Hereditary nephropathy, not elsewhere classified with membranoproliferative glomerulonephritis, types 1 and 3, or NOS

Additional Information

Description

ICD-10 code N07.5 refers to "Hereditary nephropathy, not elsewhere classified, with diffuse mesangiocapillary glomerulonephritis." This classification is part of the broader category of hereditary nephropathies, which are genetic disorders affecting kidney function and structure.

Clinical Description

Definition

Hereditary nephropathy encompasses a range of kidney diseases that are inherited and can lead to chronic kidney disease (CKD) or end-stage renal disease (ESRD). The specific condition denoted by N07.5 involves diffuse mesangiocapillary glomerulonephritis, a type of kidney inflammation characterized by changes in the glomeruli, the filtering units of the kidneys.

Pathophysiology

Diffuse mesangiocapillary glomerulonephritis is marked by the proliferation of mesangial cells and an increase in the mesangial matrix, leading to thickening of the capillary walls. This condition can result from various underlying genetic mutations or hereditary syndromes, which may affect the kidney's ability to filter blood effectively. The inflammation and structural changes can lead to proteinuria (excess protein in urine), hematuria (blood in urine), and ultimately, renal impairment.

Symptoms

Patients with hereditary nephropathy and diffuse mesangiocapillary glomerulonephritis may present with a variety of symptoms, including:
- Edema: Swelling due to fluid retention, often seen in the legs and around the eyes.
- Hypertension: Elevated blood pressure, which can be a consequence of kidney dysfunction.
- Urinary Changes: Such as foamy urine (indicative of proteinuria) or blood in the urine.
- Fatigue: Resulting from anemia or the overall impact of kidney disease.

Diagnosis

Diagnosis typically involves a combination of clinical evaluation, laboratory tests, and imaging studies. Key diagnostic tools include:
- Urinalysis: To detect proteinuria and hematuria.
- Blood Tests: To assess kidney function (e.g., serum creatinine, blood urea nitrogen).
- Kidney Biopsy: Often necessary to confirm the diagnosis and assess the extent of glomerular damage.

Genetic Considerations

Given that N07.5 is classified as a hereditary condition, genetic counseling and testing may be recommended for affected individuals and their families. Identifying specific genetic mutations can help in understanding the prognosis and potential treatment options.

Treatment and Management

Management of hereditary nephropathy with diffuse mesangiocapillary glomerulonephritis focuses on controlling symptoms and slowing disease progression. Treatment strategies may include:
- Medications: Such as angiotensin-converting enzyme (ACE) inhibitors to manage hypertension and reduce proteinuria.
- Dietary Modifications: Low-protein diets may be recommended to reduce the workload on the kidneys.
- Monitoring: Regular follow-up with nephrologists to monitor kidney function and adjust treatment as necessary.
- Renal Replacement Therapy: In advanced cases, dialysis or kidney transplantation may be required.

Conclusion

ICD-10 code N07.5 captures a specific hereditary nephropathy characterized by diffuse mesangiocapillary glomerulonephritis, highlighting the importance of genetic factors in kidney disease. Early diagnosis and comprehensive management are crucial for improving patient outcomes and quality of life. Understanding the hereditary nature of this condition can also guide family planning and genetic counseling for affected individuals.

Clinical Information

The ICD-10 code N07.5 refers to "Hereditary nephropathy, not elsewhere classified, with diffuse mesangiocapillary glomerulonephritis." This condition is characterized by a specific set of clinical presentations, signs, symptoms, and patient characteristics that are important for diagnosis and management. Below is a detailed overview of these aspects.

Clinical Presentation

Hereditary nephropathy with diffuse mesangiocapillary glomerulonephritis typically presents in patients with a family history of kidney disease. The onset can vary, but it often occurs in childhood or early adulthood. Patients may present with a range of symptoms related to kidney dysfunction, which can include:

  • Edema: Swelling in the legs, ankles, or around the eyes due to fluid retention.
  • Hypertension: Elevated blood pressure, which is common in patients with kidney disease.
  • Hematuria: Presence of blood in the urine, which may be visible or detected through urinalysis.
  • Proteinuria: Excess protein in the urine, often leading to foamy urine.
  • Reduced kidney function: Indicated by elevated serum creatinine levels and decreased glomerular filtration rate (GFR).

Signs and Symptoms

The signs and symptoms associated with N07.5 can be categorized as follows:

Common Symptoms

  • Fatigue: Generalized tiredness due to anemia or metabolic disturbances.
  • Nausea and vomiting: Resulting from uremia or electrolyte imbalances.
  • Anorexia: Loss of appetite, which can contribute to weight loss.
  • Pruritus: Itching, often due to the accumulation of waste products in the blood.

Physical Examination Findings

  • Pallor: Indicative of anemia, which is common in chronic kidney disease.
  • Elevated blood pressure: Often noted during routine examinations.
  • Signs of fluid overload: Such as pulmonary edema or ascites in advanced cases.

Patient Characteristics

Patients with hereditary nephropathy and diffuse mesangiocapillary glomerulonephritis often share certain characteristics:

  • Family History: A significant number of patients have a family history of kidney disease, suggesting a genetic predisposition.
  • Age of Onset: Symptoms may manifest in childhood or early adulthood, although late-onset cases can occur.
  • Ethnic Background: Certain populations may have a higher prevalence of hereditary nephropathies, which can influence the clinical presentation.
  • Comorbid Conditions: Patients may have associated conditions such as hypertension or diabetes, which can complicate the clinical picture.

Conclusion

In summary, ICD-10 code N07.5 encompasses hereditary nephropathy characterized by diffuse mesangiocapillary glomerulonephritis, presenting with a variety of symptoms and signs indicative of kidney dysfunction. Recognizing the clinical presentation, associated symptoms, and patient characteristics is crucial for timely diagnosis and management. Early intervention can help mitigate the progression of kidney disease and improve patient outcomes. If you have further questions or need additional information, feel free to ask!

Approximate Synonyms

ICD-10 code N07.5 refers specifically to "Hereditary nephropathy, not elsewhere classified, with diffuse mesangiocapillary glomerulonephritis." This classification is part of a broader system used for coding various health conditions, particularly those related to kidney diseases. Below are alternative names and related terms associated with this specific ICD-10 code.

Alternative Names

  1. Hereditary Nephropathy: This term broadly encompasses genetic kidney diseases, including those that may not be classified elsewhere.
  2. Diffuse Mesangiocapillary Glomerulonephritis: This is a specific type of glomerulonephritis characterized by changes in the mesangial and capillary structures of the kidney, which can be hereditary in nature.
  3. Familial Mesangiocapillary Glomerulonephritis: This term emphasizes the hereditary aspect of the condition, indicating that it can run in families.
  4. Genetic Glomerulonephritis: A broader term that includes various types of glomerulonephritis with a genetic basis, including those classified under N07.5.
  1. Glomerulonephritis: A general term for inflammation of the glomeruli in the kidneys, which can be caused by various factors, including hereditary conditions.
  2. Nephropathy: A general term for kidney disease, which can include various types of kidney damage, including hereditary forms.
  3. Mesangiocapillary Glomerulonephritis: A specific type of glomerulonephritis that involves both mesangial and capillary changes, often associated with hereditary conditions.
  4. Chronic Kidney Disease (CKD): While not specific to hereditary nephropathy, many hereditary kidney diseases can lead to CKD over time.
  5. Kidney Failure: A potential outcome of untreated or severe hereditary nephropathy, leading to the need for dialysis or transplantation.

Conclusion

Understanding the alternative names and related terms for ICD-10 code N07.5 is crucial for healthcare professionals involved in diagnosis, treatment, and coding of kidney-related conditions. These terms help in accurately identifying and communicating about the specific hereditary nephropathy and its implications for patient care. If you need further details or specific information about treatment options or management strategies for this condition, feel free to ask!

Diagnostic Criteria

To diagnose hereditary nephropathy classified under ICD-10 code N07.5, specifically with diffuse mesangiocapillary glomerulonephritis, healthcare providers typically follow a set of clinical criteria and diagnostic procedures. Here’s a detailed overview of the criteria and considerations involved in this diagnosis.

Understanding Hereditary Nephropathy

Hereditary nephropathy refers to kidney diseases that are genetically inherited. The specific subtype mentioned, diffuse mesangiocapillary glomerulonephritis, is characterized by changes in the glomeruli, the filtering units of the kidney, which can lead to significant renal impairment.

Diagnostic Criteria

Clinical Evaluation

  1. Family History: A thorough family history is essential, as hereditary nephropathies often have a genetic basis. A pattern of kidney disease in family members can support the diagnosis.

  2. Symptoms: Patients may present with symptoms such as:
    - Hematuria (blood in urine)
    - Proteinuria (excess protein in urine)
    - Edema (swelling due to fluid retention)
    - Hypertension (high blood pressure)

Laboratory Tests

  1. Urinalysis: This test can reveal the presence of blood and protein in the urine, which are indicative of glomerular damage.

  2. Serum Creatinine and Blood Urea Nitrogen (BUN): Elevated levels of these substances can indicate impaired kidney function.

  3. Genetic Testing: If hereditary nephropathy is suspected, genetic testing may be performed to identify specific mutations associated with the condition.

Imaging Studies

  1. Ultrasound: Renal ultrasound can help assess kidney size and structure, although it may not provide definitive evidence of mesangiocapillary glomerulonephritis.

Kidney Biopsy

A kidney biopsy is often the definitive diagnostic tool. It allows for:
- Histological Examination: Microscopic examination of kidney tissue can reveal characteristic changes associated with diffuse mesangiocapillary glomerulonephritis, such as:
- Mesangial proliferation
- Capillary wall thickening
- Immune complex deposition

Exclusion of Other Conditions

It is crucial to rule out other causes of glomerulonephritis, such as:
- Secondary causes (e.g., infections, systemic diseases)
- Other hereditary conditions (e.g., Alport syndrome)

Conclusion

The diagnosis of hereditary nephropathy with diffuse mesangiocapillary glomerulonephritis (ICD-10 code N07.5) involves a comprehensive approach that includes clinical evaluation, laboratory tests, imaging studies, and often a kidney biopsy. Genetic testing may also play a critical role in confirming the hereditary nature of the condition. By following these criteria, healthcare providers can accurately diagnose and manage this complex renal disorder, ensuring appropriate treatment and monitoring for affected individuals.

Treatment Guidelines

Hereditary nephropathy, classified under ICD-10 code N07.5, refers to a group of kidney disorders that are genetically inherited and can lead to various complications, including diffuse mesangiocapillary glomerulonephritis (MCGN). This condition is characterized by the proliferation of mesangial cells and the thickening of capillary walls, which can significantly impair kidney function. Understanding the standard treatment approaches for this condition is crucial for managing symptoms and preventing progression to end-stage renal disease.

Overview of Hereditary Nephropathy and MCGN

Hereditary nephropathies encompass a range of genetic disorders affecting kidney function, often leading to chronic kidney disease (CKD). Diffuse mesangiocapillary glomerulonephritis is a specific type of glomerulonephritis that can be associated with hereditary conditions, such as Alport syndrome or other genetic syndromes. The clinical presentation may include hematuria (blood in urine), proteinuria (excess protein in urine), and hypertension, which can progress to renal failure if not managed appropriately.

Standard Treatment Approaches

1. Medical Management

  • Blood Pressure Control: Managing hypertension is critical in patients with MCGN. Angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs) are commonly prescribed to help control blood pressure and reduce proteinuria, which can slow the progression of kidney disease[1].

  • Immunosuppressive Therapy: In cases where there is significant inflammation or immune-mediated damage, corticosteroids or other immunosuppressive agents may be indicated. This approach is particularly relevant if the MCGN is associated with an autoimmune process[2].

  • Management of Proteinuria: Alongside blood pressure control, strategies to reduce proteinuria are essential. This may include dietary modifications, such as a low-protein diet, and the use of medications like ACE inhibitors[3].

2. Supportive Care

  • Dietary Modifications: Patients may benefit from dietary adjustments, including a low-sodium diet to help manage blood pressure and a controlled protein intake to reduce the workload on the kidneys[4].

  • Fluid Management: Monitoring and managing fluid intake is important, especially in patients with edema or those at risk of fluid overload due to kidney dysfunction[5].

3. Renal Replacement Therapy

  • Dialysis: In cases where kidney function deteriorates significantly, dialysis may be required. This can be either hemodialysis or peritoneal dialysis, depending on the patient's condition and preferences[6].

  • Kidney Transplantation: For eligible patients with end-stage renal disease, kidney transplantation may be the best long-term solution. Genetic counseling is often recommended for patients and their families to understand the hereditary nature of the condition and the implications for potential donors[7].

4. Genetic Counseling

Given the hereditary nature of nephropathies, genetic counseling is an important aspect of care. It helps patients and their families understand the risks of transmission, implications for family planning, and the potential for screening other family members for kidney disease[8].

Conclusion

The management of hereditary nephropathy with diffuse mesangiocapillary glomerulonephritis involves a multifaceted approach that includes medical management, supportive care, and potential renal replacement therapy. Early intervention and regular monitoring are crucial to improving outcomes and quality of life for affected individuals. As research continues to evolve, new therapies and management strategies may emerge, highlighting the importance of ongoing medical care and genetic counseling for patients and their families.


References

  1. [1] Blood Pressure Control in Chronic Kidney Disease.
  2. [2] Immunosuppressive Therapy in Glomerulonephritis.
  3. [3] Management of Proteinuria in Kidney Disease.
  4. [4] Dietary Modifications for Kidney Health.
  5. [5] Fluid Management in Renal Disease.
  6. [6] Dialysis Options for End-Stage Renal Disease.
  7. [7] Kidney Transplantation and Genetic Considerations.
  8. [8] Importance of Genetic Counseling in Hereditary Conditions.

Related Information

Description

Clinical Information

  • Hereditary nephropathy with diffuse mesangiocapillary glomerulonephritis
  • Family history of kidney disease
  • Onset in childhood or early adulthood
  • Edema due to fluid retention
  • Hypertension common in patients
  • Hematuria and proteinuria present
  • Reduced kidney function indicated by elevated creatinine
  • Fatigue due to anemia or metabolic disturbances
  • Nausea and vomiting from uremia or electrolyte imbalances
  • Anorexia leading to weight loss
  • Pruritus from waste product accumulation
  • Pallor indicative of anemia
  • Elevated blood pressure often noted
  • Signs of fluid overload in advanced cases

Approximate Synonyms

  • Hereditary Nephropathy
  • Diffuse Mesangiocapillary Glomerulonephritis
  • Familial Mesangiocapillary Glomerulonephritis
  • Genetic Glomerulonephritis
  • Glomerulonephritis
  • Nephropathy
  • Mesangiocapillary Glomerulonephritis

Diagnostic Criteria

Treatment Guidelines

  • Blood Pressure Control
  • Angiotensin-Converting Enzyme (ACE) Inhibitors
  • Angiotensin Receptor Blockers (ARBs)
  • Immunosuppressive Therapy
  • Corticosteroids
  • Dietary Modifications
  • Low-Protein Diet
  • Fluid Management
  • Renal Replacement Therapy
  • Dialysis
  • Kidney Transplantation

Coding Guidelines

Excludes 1

  • Hereditary nephropathy, not elsewhere classified with C3 glomerulopathy (N07.A)
  • Hereditary nephropathy, not elsewhere classified with C3 glomerulonephritis (N07.A)

Medical Disclaimer: The information provided on this website is for general informational and educational purposes only.

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.