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Questions and Answers
What is a common marker of poor prognosis in adolescents presenting with MPGN?
Which type of MPGN is characterized by granular immune complex deposition in the mesangium capillary loops?
What characterizes Type II MPGN concerning C3 nephritic factor?
Which of the following findings is typically associated with Type III MPGN?
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Which condition is NOT commonly associated with secondary MPGN?
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What usually characterizes the serum levels of C3 in Type II MPGN?
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What is the usual immunofluorescent staining pattern in Type I MPGN?
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In adolescents with MPGN, what condition is unusual despite most being hypertensive at presentation?
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What percentage of pediatric patients with idiopathic membranous nephropathy are typically nephrotic at presentation?
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Which condition can be included in the differential diagnosis of glomerulonephritis in adolescents?
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What is a common urinary finding in children with Alport syndrome?
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Which ocular defect is most commonly associated with Alport syndrome?
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How is Alport syndrome often inherited in males from affected families?
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Which pathological finding is diagnostic for Alport syndrome?
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What is a risk factor for individuals with Alport syndrome regarding progression to ESRD?
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Which of the following symptoms might NOT be present in all individuals diagnosed with Alport syndrome?
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What is a common presenting symptom of glomerulonephritis?
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Which finding is highly sensitive for glomerular disease when greater than 30% is observed?
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What is the typical protein-to-creatinine ratio indicating nephrotic range?
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Which of the following is NOT typically associated with glomerulonephritis?
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What type of renal condition may present with normal physical examination findings?
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Which test is indicated to determine the precise diagnosis of glomerular disease?
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Which of the following conditions typically shows dysmorphic red blood cells in the urine?
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What condition is mainly characterized by significant depression of serum C3 concentration?
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Study Notes
Renal Function and Glomerulonephritis (GN)
- Renal function can be normal or impaired, influenced by acute conditions or chronic glomerular injury.
- Patients may exhibit normal physical examination and blood pressure, but can also present with oliguria, hypertension, and edema.
- Some GN types may show associated symptoms like vasculitic rash, arthritis, or pulmonary hemorrhage.
Hematuria and Urinalysis Findings
- Hematuria can be either macroscopic (visible) or microscopic.
- Urinary sediment typically reveals dysmorphic red blood cells (RBCs) and often RBC casts.
- Dysmorphic RBCs are more effectively identified with phase contrast microscopy.
- Over 30% of dysmorphic RBCs is a sensitive marker for glomerular disease.
Proteinuria Assessment
- Proteinuria can range from normal (less than 4 mg/m²/h) to nephrotic range (greater than 40 mg/m²/h).
- A random urine protein-to-creatinine ratio is comparable to a 24-hour collection, with normal < 0.2 and nephrotic range > 2.0.
Diagnosis and Referral
- A renal biopsy is essential for accurate diagnosis and assessment except in uncomplicated cases like poststreptococcal acute glomerulonephritis (PSAGN).
- Referral to a nephrologist is advised if GN is suspected beyond mild or typical cases of PSAGN.
Important Blood Tests
- Baseline tests include complete blood count, creatinine levels, complement (C3 and C4), and streptococcal serology.
- Significant decline in serum C3 is commonly seen in PSAGN, membranoproliferative glomerulonephritis (MPGN), systemic lupus erythematosus (SLE), and chronic bacteremia-related nephritis.
Membranous Nephropathy in Pediatrics
- Most children with idiopathic membranous nephropathy present with nephrotic syndrome; only 15% were non-nephrotic in a study.
- Non-nephrotic cases may display microscopic hematuria, warranting consideration of membranous GN in adolescents.
Alport Syndrome
- Alport syndrome is a hereditary glomerular basement membrane defect leading to hematuria and potential progression to end-stage renal disease (ESRD).
- Common initial presentation includes heavy microscopic hematuria, which may progress to proteinuria.
- Associated symptoms include bilateral sensorineural deafness and ocular defects, with specific eye features like anterior lenticonus.
- Inheritance patterns include X-linked dominant, with males usually more affected and progressing to ESRD in adulthood or adolescence.
Pathological Findings and Classification of MPGN
- Type I MPGN exhibits granular immune complex deposition; electron microscopy shows sub-endothelial immune deposits containing IgG, IgM, and complement components.
- Type II MPGN is characterized by presence of C3 nephritic factor (C3Nef) activating the alternative complement pathway, leading to consumption of C3.
- Type III MPGN shows electron-dense deposits on both sides of the basement membrane and is associated with mutations linked to chromosome 1q31–32.
- Secondary MPGN can be seen in conditions like chronic bacteremia and hepatitis infections.
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Description
This quiz explores the characteristics and presentation of membranous nephropathy in pediatric patients, particularly focusing on the occurrence of nephrotic syndrome. It also discusses the significance of considering this condition in differential diagnoses of glomerulonephritis in adolescents.
