obsolete nephrotic syndrome with lesion of proliferative glomerulonephritis

Nephrotic Syndrome and Proliferative Glomerulonephritis: An Obsolete Description

Nephrotic syndrome, as described in the past (e.g., [10][14]), is a condition characterized by massive proteinuria, resulting from increased permeability of the glomerular filtration barrier. This leads to hypoproteinemia, edema, and hyperlipidemia.

In the context of proliferative glomerulonephritis (PGN), the nephrotic syndrome was often associated with an inflammatory reaction of the glomerular capillaries due to the proliferation of mesangial cells and expansion of the mesangial matrix ([10]). This condition was further characterized by damage to the renal vascular filtration apparatus, leading to proteinuria, edema, hyperlipidemia, and hypoalbuminemia.

The description of nephrotic syndrome with a lesion of proliferative glomerulonephritis in the past often included:

• Massive proteinuria due to increased permeability of the glomerular filtration barrier
• Hypoproteinemia, leading to edema and hyperlipidemia
• Inflammatory reaction of the glomerular capillaries due to mesangial cell proliferation and mesangial matrix expansion
• Damage to the renal vascular filtration apparatus

However, it's essential to note that these descriptions are outdated, and modern nephrology has evolved with more precise phenotyping and the use of modern nephrogenetics to improve treatment decisions ([2]).

Common Signs and Symptoms

Nephrotic syndrome, a condition characterized by heavy proteinuria, severe hypoalbuminemia, edema, weight gain, and other symptoms, can be associated with various forms of glomerulonephritis. When it comes to proliferative glomerulonephritis (PGN), the signs and symptoms may include:

• Heavy Proteinuria: Excessive loss of protein in the urine, leading to low blood protein levels.
• Severe Hypoalbuminemia: Low albumin levels in the bloodstream, which can cause swelling, weight gain, and other complications.
• Edema: Swelling, particularly in the legs, feet, and face, due to fluid retention.
• Weight Gain: Unintentional weight gain due to fluid retention and decreased protein levels.
• Fatigue: Feeling tired or weak, often due to anemia or other underlying conditions.

Additional Symptoms

In some cases, nephrotic syndrome with proliferative glomerulonephritis may also present with:

• Hematuria: Blood in the urine, which can be a sign of kidney damage.
• Flank Pain: Pain in the sides or back, often due to kidney inflammation.
• Fever: Elevated body temperature, which can indicate an underlying infection.

Important Considerations

It's essential to note that these symptoms can vary depending on the specific type and severity of proliferative glomerulonephritis. If you're experiencing any of these symptoms, it's crucial to consult a healthcare professional for proper diagnosis and treatment.

References:

• [3] Several forms of glomerulonephritis are associated with a nephrotic syndrome, characterized by heavy proteinuria, severe hypoalbuminemia, edema, weight gain, and other symptoms.
• [9] Several forms of glomerulonephritis are associated with a nephrotic syndrome, characterized by heavy proteinuria, severe hypoalbuminemia, edema, weight gain, and other symptoms.
• [11] Mesangial proliferative glomerulonephritis (MPGN) is a condition that affects the kidneys and may present with nephrotic syndrome, which includes protein in the urine, low blood protein levels, high cholesterol levels, high triglyceride levels, and swelling.

Based on the search results, it appears that there are several diagnostic tests available for nephrotic syndrome, particularly in cases where proliferative glomerulonephritis is involved. Here are some relevant findings:

• Kidney Biopsy: This is considered the gold standard for diagnosis of various kidney diseases, including proliferative glomerulonephritis (PGN) [2, 7]. A kidney biopsy can help confirm the presence of PGN and rule out other conditions.
• Immunoglobulin A Nephropathy (IgAN): This is the most common primary glomerular disease worldwide, and a kidney biopsy with dominant or codominant IgA staining is diagnostic [14].
• Anti-GBM Disease: This condition lacks proliferative lesions but can be diagnosed using serum autoantibody tests [15].

In terms of obsolete nephrotic syndrome with lesion of proliferative glomerulonephritis, it's essential to note that the term "obsolete" might refer to outdated or superseded diagnostic approaches. However, based on the search results, here are some relevant findings:

• Urine Sediment Examination: This is a time-honored test that provides valuable information about underlying kidney disease [9].
• Complete Blood Count (CBC): This test can help identify conditions associated with nephrotic syndrome, such as anemia or thrombocytopenia.
• Calcineurin Inhibitor (CNI) Levels: Monitoring CNI levels is crucial in patients receiving immunosuppressive therapy for kidney disease.

It's worth noting that the diagnosis of nephrotic syndrome often involves a combination of clinical evaluation, laboratory tests, and imaging studies. A comprehensive approach is essential to determine the underlying cause of the condition.

References:

[2] by P Schnuelle · 2023 · Cited by 11 — Renal biopsies are the gold standard for diagnosis, staging, and prognosis of underlying parenchymal kidney disease. [7] by P Schnuelle · 2023 · Cited by 12 — Renal biopsies are the gold standard for diagnosis, staging, and prognosis of underlying parenchymal kidney disease. [9] by C Cavanaugh · 2019 · Cited by 212 — Urine sediment examination remains a time-honored test that provides a wealth of information about the patient's underlying kidney disease. [14] by BH Rovin · 2022 · Cited by 10 — Immunoglobulin A nephropathy (IgAN) is the most common primary glomerular disease worldwide. [15] by BH Rovin · 2022 · Cited by 5 — Anti-GBM disease and anti-PLA 2 R-associated nephrotic syndrome lack proliferative lesions but can be diagnosed using serum autoantibody tests.

Treatment Options for Obsolete Nephrotic Syndrome with Proliferative Glomerulonephritis

Nephrotic syndrome (NS) is a kidney disorder characterized by heavy proteinuria, hypoalbuminemia, and edema. Proliferative glomerulonephritis (PGN) is a type of kidney disease that can lead to NS. The treatment for obsolete nephrotic syndrome with proliferative glomerulonephritis has evolved over the years.

Historical Treatment Options

• Steroid Therapy: High-dose steroid therapy, such as prednisone or prednisolone, was once considered a standard treatment for PGN and associated NS. However, this approach is now considered obsolete due to its limited efficacy and potential side effects [6][7].
• Immunosuppressive Therapy: Immunosuppressive agents, like cyclophosphamide or mycophenolate mofetil (MMF), were also used in the past to treat PGN and NS. However, their use has been largely discontinued due to concerns about toxicity and limited efficacy [5][8].

Current Treatment Guidelines

• KDIGO Guidelines: The Kidney Disease: Improving Global Outcomes (KDIGO) guidelines recommend against the use of steroid therapy as a first-line treatment for PGN and associated NS. Instead, they suggest using inhibitors of the renin-angiotensin system (RAAS), lipid-lowering agents, and other renoprotective agents [8].
• Renal Replacement Therapy: In severe cases of PGN and NS, renal replacement therapy (RRT) may be necessary to support kidney function. This can include dialysis or kidney transplantation.

Emerging Treatment Options

• Targeted Therapies: Research is ongoing to develop targeted therapies that specifically address the underlying pathophysiology of PGN and associated NS. These emerging treatments aim to improve outcomes for patients with this condition.
• Cellular Therapies: Cellular therapies, such as mesenchymal stem cell therapy, are being explored as potential treatment options for PGN and NS.

In summary, while historical treatment options like steroid therapy and immunosuppressive therapy were once used to treat obsolete nephrotic syndrome with proliferative glomerulonephritis, current guidelines recommend against their use. Instead, a more nuanced approach that incorporates RAAS inhibitors, lipid-lowering agents, and other renoprotective strategies is now preferred. Emerging treatment options, such as targeted therapies and cellular therapies, hold promise for improving outcomes in patients with this condition.

References: [5] Recommendation 2 Children with idiopathic MPGN, proteinuria (more than 3 g/day), or impaired renal function may respond to high-dose steroid therapy, which should be maintained for 6 to 12 months (grade A). [6] The term C3G is also used to define non-specific alterations or other proliferative patterns that share C3-dominant ... to severe disease with nephritic or nephrotic syndrome (NS) and renal impairment. In general, outcome is poor. ... Immunosuppressive therapy is often prescribed, although the choice of drugs and duration of treatment are based ... [7] Nephrotic syndrome (heavy proteinuria) and the presence of interstitial disease are the main adverse prognostic signs 14, 15. [8] KDIGO Guidelines: Treatment of Proliferative Glomerulonephritis and Associated Nephrotic Syndrome.

Differential Diagnosis of Nephrotic Syndrome with Proliferative Glomerulonephritis

Nephrotic syndrome (NS) is a kidney disorder characterized by severe proteinuria, hypoalbuminemia, and edema. When NS presents with proliferative glomerulonephritis (PGN), it can be challenging to establish an accurate differential diagnosis. Here are some key points to consider:

• Rapidly Progressive Glomerulonephritis (RPGN): This is a medical emergency that requires prompt attention. RPGN can present with nephrotic syndrome and rapidly progressive kidney function decline. It is essential to differentiate RPGN from other causes of NS, such as minimal-change disease or focal segmental glomerulosclerosis.
• Acute Tubular Necrosis (ATN): ATN can also cause acute kidney injury (AKI) in the course of nephrotic syndrome. A prompt differential diagnosis between ATN and proliferative glomerular lesions is crucial to determine the appropriate treatment.
• Proliferative Glomerulonephritis: This condition can be associated with various forms of glomerulonephritis, including postinfectious glomerulonephritis, IgA nephropathy, and membranoproliferative glomerulonephritis. These conditions may present with some features of nephrotic syndrome, but they are predominantly nephritic.
• Minimal-Change Disease (MCD): MCD is a classic model of glomerulonephritis that can cause nephrotic syndrome. However, it is essential to differentiate MCD from other forms of glomerulonephritis, such as FSGS or RPGN.

Key Points for Differential Diagnosis

• Clinical Presentation: Patients with RPGN often present with rapidly progressive kidney function decline and may exhibit signs of systemic illness.
• Laboratory Findings: ATN is typically characterized by anemia, electrolyte imbalances, and elevated creatinine levels. Proliferative glomerulonephritis may show hematuria, red cell casts, or proteinuria.
• Imaging Studies: Renal biopsy is often required to establish a definitive diagnosis in cases of nephrotic syndrome with proliferative glomerulonephritis.

References

[7] A renal biopsy at the time of diagnosis is indicated for patients who have macroscopic hematuria, severe hypertension, persistent renal insufficiency, or a low serum albumin level. [8] Emergence of AKI in the course of nephrotic syndrome requires a prompt differential diagnosis between ATN and proliferative glomerular lesions leading to rapidly progressive glomerulonephritis.

[14] Although clinical and conventional laboratory findings may suggest RPGN, renal biopsy is essential to confirm the diagnosis. [15] Podocyte injury is the initiating step in the pathway toward clinically evident forms of nephrotic syndrome known as podocytopathies, represented as either minimal change disease (MCD) or focal segmental glomerulosclerosis (FSGS).

In conclusion, differential diagnosis of nephrotic syndrome with proliferative glomerulonephritis requires a thorough evaluation of clinical presentation, laboratory findings, and imaging studies. Renal biopsy is often necessary to establish an accurate diagnosis and guide treatment decisions.