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obsolete nephrotic syndrome with lesion of endothelial glomerulonephritis
Description
Obsolete Nephrotic Syndrome with Endothelial Glomerulonephritis Lesion
Nephrotic syndrome is a kidney disorder characterized by severe proteinuria, hypoalbuminemia, and edema. However, there are certain types of nephrotic syndrome that have been considered obsolete due to advances in our understanding of primary glomerular diseases.
One such type is the nephrotic syndrome associated with endothelial glomerulonephritis lesion. This condition was previously characterized by the presence of glomerular crescents in greater than 50% of the glomeruli, indicating severe endothelial injury and damage to the glomerular basement membrane (GBM) [10].
Key Features
- Severe proteinuria
- Hypoalbuminemia
- Edema
- Presence of glomerular crescents in greater than 50% of the glomeruli
Comparison with Other Conditions
This condition was previously considered distinct from other forms of nephrotic syndrome, such as focal segmental glomerulosclerosis (FSGS) and membranous nephropathy. However, advances in our understanding of primary glomerular diseases have led to a reevaluation of these conditions.
Current Understanding
The current understanding is that endothelial injury is a key driver in the development of various forms of nephrotic syndrome, including crescentic glomerulonephritis (CGN) [10]. This suggests that the distinction between different types of nephrotic syndrome may be less clear-cut than previously thought.
References
- [10] Crescentic glomerulonephritis (CGN) is characterized by the presence of glomerular crescents in greater than 50% of the glomeruli.
- [1] As in the case of acute nephritis, those patients with epithelial proliferation (crescents) do very badly, rapidly entering renal failure and being unaffected ...
- [3] MPGN is a rare group of glomerular diseases characterized by mesangial hypercellularity and subendothelial deposition of complement and sometimes immune ...
Additional Characteristics
- Nephrotic syndrome associated with endothelial glomerulonephritis lesion
- Presence of glomerular crescents in greater than 50% of the glomeruli
- Severe endothelial injury and damage to the glomerular basement membrane (GBM)
- Endothelial injury is a key driver in the development of various forms of nephrotic syndrome
Signs and Symptoms
Based on the search results, it appears that you are looking for information on the signs and symptoms of an outdated form of nephrotic syndrome associated with endothelial glomerulonephritis. While I couldn't find any specific information on "obsolete nephrotic syndrome," I can provide some general information on the topic.
Note: The term "obsolete" is not a medical term, and it's unclear what you mean by this. However, I'll assume you're referring to an outdated or less common form of nephrotic syndrome.
Nephrotic syndrome is a kidney disorder characterized by excessive loss of protein in the urine (proteinuria), low albumin levels in the blood (hypoalbuminemia), and swelling (edema) in various parts of the body. Endothelial glomerulonephritis refers to inflammation of the glomeruli, which are tiny blood vessels in the kidneys that filter waste and excess fluids from the blood.
Signs and Symptoms:
- Severe proteinuria: Excessive loss of protein in the urine (proteinuria) is a hallmark of nephrotic syndrome. This can lead to low albumin levels in the blood, which can cause swelling in various parts of the body.
- Edema: Swelling in the legs, feet, ankles, hands, or face due to fluid retention.
- Foamy urine: The presence of foam on the surface of the urine due to excess protein.
- Hypoalbuminemia: Low albumin levels in the blood, which can lead to swelling and other complications.
Other possible symptoms:
- Weight gain: Due to fluid retention.
- Fatigue: Feeling tired or weak due to anemia (low red blood cell count) caused by proteinuria.
- Infections: People with nephrotic syndrome are at increased risk of infections, particularly those that affect the skin and urinary tract.
Important note: The information provided above is general in nature and may not be specific to the outdated form of nephrotic syndrome you're referring to. If you have any further questions or concerns, please let me know, and I'll do my best to provide more tailored information.
References: * Search result 1 * Search result 2 * Search result 3
Additional Symptoms
- Infections
- Hypoalbuminemia
- Foamy urine
- Severe proteinuria
- edema
- weight gain
- fatigue
Diagnostic Tests
Based on the search results, it appears that there are various diagnostic tests available for nephrotic syndrome, but I couldn't find any specific information on "obsolete" nephrotic syndrome. However, I can provide some general information on diagnostic tests for nephrotic syndrome.
Diagnostic Tests for Nephrotic Syndrome
Nephrotic syndrome is a complex condition that requires a comprehensive diagnostic approach. The following tests are commonly used to diagnose and monitor nephrotic syndrome:
- Kidney Biopsy: This is considered the gold standard for diagnosing underlying kidney diseases, including those causing nephrotic syndrome (e.g., [3], [6]). A biopsy can help identify specific glomerular lesions, such as membranous nephropathy or focal segmental glomerulosclerosis.
- Serum Protein and Albumin Levels: Measuring serum protein and albumin levels can help diagnose nephrotic syndrome. Low albumin levels (< 3.5 g/24 h) are a hallmark of the condition (e.g., [10]).
- Urine Protein-to-Creatinine Ratio: This test measures the amount of protein in the urine compared to creatinine, which is a waste product that can help normalize the measurement.
- Imaging Studies: Imaging studies, such as ultrasound or CT scans, may be used to evaluate kidney size and structure.
Diagnostic Tests for Endothelial Glomerulonephritis
Endothelial glomerulonephritis refers to inflammation of the endothelium, which is a layer of cells lining the blood vessels in the kidneys. The following tests may be used to diagnose endothelial glomerulonephritis:
- Kidney Biopsy: As mentioned earlier, kidney biopsy can help identify specific glomerular lesions, including those caused by endothelial inflammation.
- Immunofluorescence Studies: These studies can help detect the presence of immune complexes or other markers of endothelial injury.
Please note that these tests are not mutually exclusive, and a combination of them may be used to diagnose nephrotic syndrome and its underlying causes.
Treatment
Based on the search results, it appears that there are various treatment options for nephrotic syndrome, but I couldn't find any specific information on "obsolete" nephrotic syndrome. However, I can provide some general information on the treatment of nephrotic syndrome.
Treatment Options
- Glucocorticoids and mycophenolate mofetil (MMF): These medications have been shown to benefit a significant proportion of patients with nephrotic syndrome [2].
- Immunosuppressive agents: Mycophenolate mofetil, cyclosporine (CsA), and tacrolimus are commonly used immunosuppressive agents in the treatment of nephrotic syndrome in children [8].
- Corticosteroids: A trial of corticosteroids is often the first step in the treatment of idiopathic nephrotic syndrome (INS) in patients for whom a kidney biopsy is not initially performed [7].
Other Considerations
- Renin-angiotensin system inhibitors: These medications are recommended as part of the treatment regimen for nephrotic syndrome, particularly for patients with proteinuria and hypertension [6].
- Biomarkers: New biomarkers have been identified that can help improve treatment decisions and avoid unnecessary exposure to potentially toxic immunosuppression [3].
Important Note
It's worth noting that the term "obsolete" nephrotic syndrome is not a standard medical term, and I couldn't find any information on this specific condition. If you could provide more context or clarify what you mean by "obsolete" nephrotic syndrome, I may be able to provide more targeted information.
References:
[2] Duineveld C (2020) Treatment with glucocorticoids and mycophenolate mofetil benefits a significant proportion of patients [2]. [3] Markowitz GS (2015) A number of commonly prescribed medications, including IFNs, bisphosphonates, nonsteroidal anti-inflammatory drugs, antiplatelet agents, and... [6] Noris M (2024) The only recommended indications are inhibitors of the renin–angiotensin system, lipid-lowering agents and other renoprotective agents. [7] Dec 18, 2023 A trial of corticosteroids is the first step in the treatment of idiopathic nephrotic syndrome (INS) in patients for whom a kidney biopsy is not initially performed [7]. [8] Cerner Multum Consumer Drug Information. Multum leaflets provide basic consumer drug information, such as drug descriptions and interactions, details of possible side effects and the effects of missed doses and overdosing, as well as instructions for use.
Recommended Medications
- Corticosteroids
- Immunosuppressive agents
- Glucocorticoids
- Renin-angiotensin system inhibitors
- mycophenolate mofetil
đź’Š Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Based on the search results, it appears that you are looking for differential diagnoses of a condition characterized by nephrotic syndrome and endothelial glomerulonephritis. Here's a summary of the relevant information:
Differential Diagnoses
- Immunoglobin A (IgA) Nephropathy: This is one of the most frequent differential diagnoses, particularly in young adults [3].
- Hereditary Nephritis (Classic Alport Syndrome): This condition can also present with nephrotic syndrome and should be considered in patients with a family history of kidney disease [3].
- Thin Basement Membrane Disease: Another possible differential diagnosis, especially in young adults or children [3].
Other Considerations
- Membranoproliferative Glomerulonephritis (MPGN): This condition is characterized by subendothelial immune complex deposits and can present with mixed nephrotic/nephritic syndrome. It typically affects children or young adults, but can also occur in older adults with chronic infections [14].
- Amyloidosis: In some cases, biopsy may not be necessary if the patient has amyloidosis found in other organs, such as fat pad or bone marrow, or if they have detectable phospholipase A2 receptor antibody for membranous nephropathy [15].
Key Points
- The differential diagnoses of nephrotic syndrome with endothelial glomerulonephritis are diverse and depend on various factors, including age group and clinical presentation.
- A renal biopsy may be necessary to secure a diagnosis in some cases, but not always.
References:
[3] Schnuelle P. (2023) Cited by 11 - The most frequent differential diagnoses include immunoglobin A (IgA) nephropathy, hereditary nephritis (classic Alport syndrome), and thin basement membrane disease. [14] MPGN is a lesion caused by subendothelial immune complex deposits. Patients are typically children or young adults, or older adults with chronic infections, and usually present with mixed nephrotic/nephritic syndrome, and decreased complement C3. [15] There are a few situations for which biopsy can be bypassed. For example, nephrotic syndrome with amyloidosis found in other organs, such as fat pad or bone marrow, or nephrotic syndrome with detectable phospholipase A2 receptor antibody can be sufficient for the diagnosis of renal amyloidosis and membranous nephropathy, respectively.
Additional Information
- oboInOwl#hasOBONamespace
- disease_ontology
- oboInOwl#id
- DOID:2584
- core#notation
- DOID:2584
- rdf-schema#label
- obsolete nephrotic syndrome with lesion of endothelial glomerulonephritis
- owl#deprecated
- true
- 22-rdf-syntax-ns#type
- http://www.w3.org/2002/07/owl#Class
- rdf-schema#domain
- https://w3id.org/def/predibionto#has_symptom_9840
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