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Nephrosis

Disease Details

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Description: Nephrosis, also known as nephrotic syndrome, is a kidney disorder characterized by excessive protein loss in the urine, leading to swelling and increased risk of infection and blood clots.
Type: Nephrosis, specifically referring to diseases characterized by nephrotic syndrome, can be caused by various genetic and non-genetic factors. For genetic forms, one example is Congenital Nephrotic Syndrome. The type of genetic transmission for this condition can vary, but a common mode is autosomal recessive inheritance. This means a child must inherit two copies of the mutated gene, one from each parent, to be affected by the disease.
Signs And Symptoms: Signs and symptoms of nephrosis, also known as nephrotic syndrome, may include:
- Severe swelling (edema), particularly around the eyes and in the ankles and feet
- Foamy urine, which may result from excess protein in the urine
- Weight gain due to fluid retention
- Fatigue
- Loss of appetite

Nan generally refers to "not a number" or a null value and does not apply in the context of nephrosis.
Prognosis: The prognosis for nephrosis, specifically nephrotic syndrome, can vary widely based on the underlying cause, response to treatment, and the presence of any complications. Generally, children with minimal change disease, the most common cause, often respond well to corticosteroids and have a good prognosis. Adults may have a more variable prognosis, depending on the underlying cause such as focal segmental glomerulosclerosis or membranous nephropathy. Early diagnosis and treatment are crucial for better outcomes, and ongoing management may be necessary to monitor and address potential complications such as infections, blood clots, and progression to chronic kidney disease.
Onset: Nephrosis, also known as nephrotic syndrome, often has a gradual onset. Symptoms may not be immediately noticeable and can develop over time. Common signs include swelling (edema), especially around the eyes, feet, and ankles, as well as weight gain due to fluid retention, foamy urine due to excess protein, and fatigue. Pediatric cases may present more abruptly than in adults.

"Nan" is not a recognized term in the context of nephrosis. If you meant a specific aspect such as "nanoscale" implications in nephrosis or another topic, please provide more details for a precise answer.
Prevalence: Nephrosis, also known as nephrotic syndrome, affects approximately 2-7 per 100,000 children annually. The prevalence in adults is less well-defined but is considered to be rare.
Epidemiology: Nephrosis, commonly referred to as nephrotic syndrome, is a kidney disorder characterized by significant proteinuria, hypoalbuminemia, hyperlipidemia, and edema. It can affect people of all ages but has distinct patterns in different populations:

**Epidemiology:**
- **Prevalence:** Nephrotic syndrome is relatively rare, with an annual incidence estimated at 2-7 cases per 100,000 children and 3 cases per 100,000 adults.
- **Age:** It is more commonly diagnosed in children, particularly between the ages of 2 and 6. In adults, it frequently appears between the ages of 40 and 60.
- **Gender:** In children, boys are more commonly affected than girls (approximately 2:1 ratio). In adults, the incidence tends to be more balanced between genders.
- **Ethnicity:** Some studies suggest that certain ethnic groups, including African Americans, Hispanics, and Asians, may have higher susceptibility to certain forms of nephrotic syndrome, such as focal segmental glomerulosclerosis (FSGS).

Nephrotic syndrome can result from primary kidney disease (primary nephrotic syndrome) or secondary causes such as diabetes, infections, or autoimmune diseases (secondary nephrotic syndrome). The epidemiology can vary significantly based on the underlying cause.
Intractability: Nephrosis, also known as nephrotic syndrome, is not inherently intractable but can be challenging to manage. With proper treatment, including medications, dietary changes, and monitoring, many patients can achieve disease control and remission. However, some cases may be more resistant to treatment based on the underlying cause and patient-specific factors.
Disease Severity: Nephrosis, also known as nephrotic syndrome, can vary in severity. In its mild form, it may be managed with medication and lifestyle changes. However, severe nephrosis can lead to significant complications such as infections, blood clots, and chronic kidney disease, potentially requiring more intensive treatments like dialysis or a kidney transplant.
Healthcare Professionals: Disease Ontology ID - DOID:2527
Pathophysiology: Nephrosis, also known as nephrotic syndrome, is characterized by significant proteinuria, hypoalbuminemia, hyperlipidemia, and edema. The pathophysiology involves damage to the glomeruli, leading to increased permeability of the glomerular basement membrane. This permeability allows proteins, especially albumin, to leak into the urine (proteinuria). Loss of albumin decreases the oncotic pressure in the blood, causing fluid to shift into the interstitial spaces, leading to edema. The liver increases lipid production in response to hypoalbuminemia, resulting in hyperlipidemia.
Carrier Status: Nephrosis, or nephrotic syndrome, does not have a carrier status as it is not a single genetic disease that is inherited in a straightforward manner like recessive or dominant gene disorders. Rather, it is a condition characterized by a set of symptoms including proteinuria, hypoalbuminemia, and edema, which can result from a variety of underlying causes, including diseases, infections, or genetic disorders. The term "nan" (not a number) therefore does not apply in this context.
Mechanism: Nephrosis, also known as nephrotic syndrome, is primarily characterized by excessive protein loss in the urine due to damage to the glomeruli of the kidneys. The mechanisms and molecular pathways involved are complex and can vary depending on the underlying cause, but they generally include:

**Mechanism:**
1. **Glomerular Injury:** Damage to the glomerular filtration barrier, which consists of endothelial cells, the glomerular basement membrane (GBM), and podocytes (specialized epithelial cells), leads to increased permeability.
2. **Proteinuria:** Increased permeability allows proteins, especially albumin, to pass from the blood into the urine, resulting in proteinuria.
3. **Hypoalbuminemia:** The loss of albumin exceeds the liver's ability to synthesize new protein, leading to low levels of albumin in the blood (hypoalbuminemia).
4. **Edema:** Reduced plasma oncotic pressure due to low serum albumin levels leads to fluid shifting from the vascular space to the interstitial space, causing generalized edema.
5. **Hyperlipidemia:** Altered lipid metabolism, often a compensatory response to hypoalbuminemia, results in elevated levels of cholesterol and triglycerides in the blood.

**Molecular Mechanisms:**
1. **Podocyte Injury:** Damage or dysfunction of podocytes, which play a critical role in maintaining the filtration barrier, is central to nephrosis. This can be due to genetic mutations, toxins, or immune-mediated processes.
2. **Cytokines and Growth Factors:** Increased levels of cytokines like tumor necrosis factor-alpha (TNF-α) and transforming growth factor-beta (TGF-β) contribute to inflammation and fibrosis.
3. **Immune Responses:** In certain types of nephrotic syndrome (e.g., minimal change disease, membranous nephropathy), immune complex deposition or T-cell mediated injury results in glomerular damage.
4. **Protein Alterations:** Mutations or alterations in structural proteins of the glomerular basement membrane or slit diaphragm proteins (e.g., nephrin, podocin) can lead to filtration barrier dysfunction.
5. **Reactive Oxygen Species (ROS):** Elevated ROS levels can cause oxidative stress and contribute to cellular injury in the glomeruli.
6. **Endothelial Dysfunction:** Injury to endothelial cells can disrupt the GBM and contribute to increased protein permeability.

Understanding these mechanisms is essential for developing targeted therapies to treat or mitigate the effects of nephrosis.
Treatment: Nephrosis, also known as nephrotic syndrome, is primarily treated by addressing the underlying cause and managing symptoms. Treatment options may include:

1. **Medications:**
- **Corticosteroids:** Reduce inflammation and proteinuria.
- **Diuretics:** Help reduce swelling (edema).
- **ACE inhibitors/ARBs:** Lower blood pressure and reduce protein loss in urine.
- **Statins:** Manage high cholesterol levels.
- **Immunosuppressants:** Used if nephrosis is due to an autoimmune condition.

2. **Dietary Changes:**
- **Low-sodium Diet:** Helps control blood pressure and reduce edema.
- **Low-fat Diet:** Manages cholesterol levels.
- **Adequate Protein Intake:** To prevent muscle wasting while not overloading the kidneys.

3. **Lifestyle Modifications:**
- **Regular Exercise:** Helps maintain overall health.
- **Smoking Cessation:** Reduces cardiovascular risk.

4. **Monitoring and Support:**
- Regular check-ups with a nephrologist.
- Monitoring kidney function, blood pressure, and urine protein levels.
- Supportive treatments for complications like blood clots or infections.

In severe cases, kidney dialysis or transplant may be necessary if kidney function continues to decline.
Compassionate Use Treatment: Treatment options for nephrosis (nephrotic syndrome) can include several off-label or experimental approaches, although their use depends greatly on individual patient circumstances and the specific underlying cause of the condition. Here are some of these approaches:

1. **Rituximab**: This monoclonal antibody is sometimes used off-label in cases of steroid-resistant nephrotic syndrome, particularly in conditions like minimal change disease or focal segmental glomerulosclerosis (FSGS).

2. **Adalimumab and Infliximab**: These are anti-TNF biologics that have been used experimentally in some cases of steroid-resistant nephrotic syndrome, particularly in FSGS.

3. **ACTH (Adrenocorticotropic Hormone)**: This hormone has been used as a treatment for some cases of nephrotic syndrome, particularly in adults, showing promise in certain resistant cases.

4. **Abatacept**: This immunomodulatory drug has been used experimentally in treating certain forms of nephrotic syndrome, especially in patients with recurrent FSGS post-transplant.

5. **Stem Cell Therapy**: While still very much in the experimental stages, stem cell treatments are being investigated as potential therapies for nephrotic syndrome.

6. **Sparsentan**: This is an investigational drug that acts as both an angiotensin receptor blocker and an endothelin receptor antagonist. It is being studied for use in treating FSGS and other forms of primary nephrotic syndrome.

It’s important for patients to consult with their nephrologist to understand the best course of treatment for their specific condition, including the potential risks and benefits of experimental or off-label treatments.
Lifestyle Recommendations: For nephrosis, also known as nephrotic syndrome, lifestyle recommendations include:

1. **Dietary Changes:**
- **Low Sodium:** Reduce salt intake to help control blood pressure and reduce fluid retention.
- **Low Fat:** Opt for a diet low in saturated fats to manage cholesterol levels.
- **Adequate Protein:** Monitor protein intake; too much can strain the kidneys, but too little can lead to malnutrition. Consult a dietitian for personalized advice.

2. **Fluid Management:**
- Monitor fluid intake if you have swelling and fluid retention. Follow your doctor's recommendations on the appropriate amount.

3. **Weight Management:**
- Maintain a healthy weight to reduce the burden on your kidneys and overall cardiovascular health.

4. **Exercise:**
- Engage in regular, moderate physical activity to maintain overall health and well-being. Avoid strenuous activities that can cause strain.

5. **Smoking Cessation:**
- Quit smoking to improve overall kidney health and reduce the risk of cardiovascular complications.

6. **Blood Pressure Control:**
- Regularly monitor and manage your blood pressure through lifestyle changes and medication if prescribed.

7. **Regular Medical Follow-up:**
- Keep regular appointments with your healthcare provider to monitor kidney function and manage any associated conditions.

8. **Medication Adherence:**
- Strictly follow prescribed medications to control symptoms and prevent complications.

These lifestyle changes can help manage symptoms and improve quality of life for those with nephrosis. Always consult with healthcare professionals for personalized advice.
Medication: Nephrosis, also known as nephrotic syndrome, is treated with medications that target the underlying cause and symptoms. Common medications include:

1. Corticosteroids: Reduce inflammation and immune system activity.
2. Immunosuppressants: Lower immune system activity to prevent kidney damage (e.g., cyclophosphamide, cyclosporine).
3. ACE inhibitors or ARBs: Control blood pressure and reduce protein loss in urine (e.g., lisinopril, losartan).
4. Diuretics: Reduce fluid retention and swelling (e.g., furosemide).
5. Statins: Lower cholesterol levels (e.g., atorvastatin).

Patient treatment plans should be personalized based on individual health status and underlying causes.
Repurposable Drugs: Repurposable drugs for nephrosis include:

1. **Statins** (e.g., atorvastatin) - Traditionally used for lowering cholesterol but may have protective effects on kidney function.
2. **Angiotensin-converting enzyme (ACE) inhibitors** (e.g., enalapril) - Primarily for hypertension, they can reduce proteinuria and slow the progression of kidney disease.
3. **Angiotensin II receptor blockers (ARBs)** (e.g., losartan) - Also used for hypertension, they help in reducing proteinuria and protecting kidney function.
4. **Spironolactone** - A diuretic that may help reduce proteinuria and edema associated with nephrosis.
5. **Dexamethasone** - A corticosteroid that can be used to manage inflammation and proteinuria.

These drugs are utilized based on their secondary benefits in managing the symptoms and progression of nephrosis. It’s important that any off-label use be supervised by a healthcare professional.
Metabolites: Nephrosis, also known as nephrotic syndrome, is characterized by abnormal excretion of protein in urine due to kidney damage. Metabolites detected in nephrotic syndrome can include:

1. **Albumin**: A significant presence of albumin in urine (albuminuria) is a hallmark of nephrotic syndrome.
2. **Cholesterol and Triglycerides**: Elevated levels are often found in the blood due to hepatic overproduction in response to hypoalbuminemia.
3. **Urea and Creatinine**: These may be affected due to compromised kidney function, although they are more typically indicators of nephritic syndrome.
4. **Lipids**: Increased urinary excretion of lipids can occur.

The mencion "nan" (not a number) could refer to a computational result or data point that is non-applicable, indicating either missing or undefined data in the context of the disease’s database analysis or clinical assessments. However, it is not a standard term associated with nephrotic syndrome pathology.
Nutraceuticals: Nutraceuticals are substances that can be used to aid in the management of nephrosis (nephrotic syndrome). Here are some common ones:

1. **Omega-3 Fatty Acids:** They have anti-inflammatory properties and may help reduce proteinuria, a hallmark of nephrosis.

2. **Coenzyme Q10 (CoQ10):** This antioxidant can support kidney function and reduce oxidative stress.

3. **Curcumin:** Found in turmeric, it has anti-inflammatory and antioxidant properties that may benefit kidney health.

4. **Resveratrol:** An antioxidant found in grapes and berries, resveratrol may provide renal protection.

Consult with a healthcare professional before pursuing nutraceuticals for nephrosis to ensure safety and appropriateness.
Peptides: Nephrosis, also known as nephrotic syndrome, is a kidney disorder characterized by excessive protein loss in urine, leading to low levels of protein in the blood.

- **Peptides**: In the context of nephrosis, peptides like albumin and other plasma proteins are lost through the urine. Restoring or managing these protein levels is crucial for treatment.

- **Nanotechnology (Nan)**: Nanotechnology offers promising diagnostic and therapeutic approaches, such as nanoparticle-based drug delivery systems to target kidney tissues and improve treatment efficacy with minimal side effects.