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Questions and Answers
A 26-year-old male presents with recurrent leg and abdominal swelling, proteinuria, but not haematuria. His creatinine is normal. Which pathophysiological mechanism most likely explains the normal creatinine levels despite significant proteinuria?
A 26-year-old male presents with recurrent leg and abdominal swelling, proteinuria, but not haematuria. His creatinine is normal. Which pathophysiological mechanism most likely explains the normal creatinine levels despite significant proteinuria?
- Reduced creatinine production occurs secondary to liver dysfunction from hypovolemia.
- Increased proximal tubular reabsorption of creatinine compensates for glomerular loss.
- Elevated creatinine excretion is masked by increased muscle mass.
- Hyperfiltration elevates creatinine clearance, maintaining serum creatinine within normal limits. (correct)
A 26-year-old male with nephrotic syndrome, relapsing frequently even with increased steroid use, is being evaluated. Given the history, which immunological process is least likely to be directly involved in the pathogenesis of his condition?
A 26-year-old male with nephrotic syndrome, relapsing frequently even with increased steroid use, is being evaluated. Given the history, which immunological process is least likely to be directly involved in the pathogenesis of his condition?
- Antibody-mediated injury to podocytes resulting in disruption of the filtration barrier.
- Activation of the complement cascade causing glomerular inflammation.
- Deposition of immune complexes in the glomerular basement membrane. (correct)
- T-cell dysfunction leading to podocyte damage and proteinuria.
A 26-year-old male being treated for nephrotic syndrome develops hyperlipidemia. Which of the following best explains the primary mechanism leading to hypercholesterolemia in nephrotic syndrome?
A 26-year-old male being treated for nephrotic syndrome develops hyperlipidemia. Which of the following best explains the primary mechanism leading to hypercholesterolemia in nephrotic syndrome?
- Increased hepatic synthesis of lipoproteins in response to decreased plasma oncotic pressure. (correct)
- Decreased hepatic LDL receptor expression.
- Increased catabolism of LDL particles.
- Reduced activity of lipoprotein lipase (LPL).
A 26-year-old male with nephrotic syndrome is treated with an ACE inhibitor. What is the primary rationale for using this medication in the context of nephrotic syndrome?
A 26-year-old male with nephrotic syndrome is treated with an ACE inhibitor. What is the primary rationale for using this medication in the context of nephrotic syndrome?
A 26-year-old male with nephrotic syndrome is being considered for a renal biopsy. Which of the following clinical findings would most strongly suggest the need for a renal biopsy to determine the underlying cause?
A 26-year-old male with nephrotic syndrome is being considered for a renal biopsy. Which of the following clinical findings would most strongly suggest the need for a renal biopsy to determine the underlying cause?
A 26-year-old male with nephrotic syndrome had a renal biopsy revealing focal and segmental glomerulosclerosis (FSGS). Which of the following is the most accurate statement regarding the potential long-term outcomes?
A 26-year-old male with nephrotic syndrome had a renal biopsy revealing focal and segmental glomerulosclerosis (FSGS). Which of the following is the most accurate statement regarding the potential long-term outcomes?
A 47-year-old male presents with headaches, hypertension, and pedal edema. He also reports malaise of 2 months' duration, along with a history of hypertension for the past four years and a single episode of frank haematuria. Urinalysis shows RBC +++ and 1+ protein. Considering this information, what is the most critical next step in evaluating the haematuria?
A 47-year-old male presents with headaches, hypertension, and pedal edema. He also reports malaise of 2 months' duration, along with a history of hypertension for the past four years and a single episode of frank haematuria. Urinalysis shows RBC +++ and 1+ protein. Considering this information, what is the most critical next step in evaluating the haematuria?
A 47-year-old male with hypertension and haematuria is suspected of having a glomerular cause for his haematuria. Which of the following conditions is least likely to present primarily with isolated glomerular haematuria?
A 47-year-old male with hypertension and haematuria is suspected of having a glomerular cause for his haematuria. Which of the following conditions is least likely to present primarily with isolated glomerular haematuria?
A 47-year-old male diagnosed with IgA nephropathy is being assessed using the Oxford scoring system. Presence of which of the following features indicates the poorest prognosis?
A 47-year-old male diagnosed with IgA nephropathy is being assessed using the Oxford scoring system. Presence of which of the following features indicates the poorest prognosis?
A 47-year-old male with IgA nephropathy presents with worsening renal function. A renal biopsy shows mesangial IgA deposits. Which cellular component of the glomerulus is most directly involved in producing the pathological changes seen in IgA nephropathy?
A 47-year-old male with IgA nephropathy presents with worsening renal function. A renal biopsy shows mesangial IgA deposits. Which cellular component of the glomerulus is most directly involved in producing the pathological changes seen in IgA nephropathy?
A 26-year-old male presents with nephrotic syndrome. Further investigations reveal heavy proteinuria, hypoalbuminemia, edema, and hypercholesterolemia. Which of the following mechanisms most directly contributes to the development of edema in this patient?
A 26-year-old male presents with nephrotic syndrome. Further investigations reveal heavy proteinuria, hypoalbuminemia, edema, and hypercholesterolemia. Which of the following mechanisms most directly contributes to the development of edema in this patient?
A 26-year-old male with nephrotic syndrome, secondary to Focal Segmental Glomerulosclerosis (FSGS), is treated with high-dose corticosteroids. Which of the following is the least likely complication associated with long-term corticosteroid therapy?
A 26-year-old male with nephrotic syndrome, secondary to Focal Segmental Glomerulosclerosis (FSGS), is treated with high-dose corticosteroids. Which of the following is the least likely complication associated with long-term corticosteroid therapy?
A 47-year-old male is diagnosed with IgA nephropathy following a renal biopsy. What is the primary mechanism by which upper respiratory tract infections (URTIs) exacerbate IgA nephropathy?
A 47-year-old male is diagnosed with IgA nephropathy following a renal biopsy. What is the primary mechanism by which upper respiratory tract infections (URTIs) exacerbate IgA nephropathy?
A 47-year-old male with IgA nephropathy is noted to have hypertension and microscopic hematuria. Which of the following is the most appropriate initial therapeutic intervention to reduce proteinuria and preserve renal function?
A 47-year-old male with IgA nephropathy is noted to have hypertension and microscopic hematuria. Which of the following is the most appropriate initial therapeutic intervention to reduce proteinuria and preserve renal function?
A 26-year-old male with nephrotic syndrome is found to have echogenic kidneys upon ultrasound. What does this finding suggest regarding the underlying condition?
A 26-year-old male with nephrotic syndrome is found to have echogenic kidneys upon ultrasound. What does this finding suggest regarding the underlying condition?
A 47-year-old male is diagnosed with IgA nephropathy. He asks about the likelihood of recurrence if he needs a kidney transplant in the future. What is the most accurate statement?
A 47-year-old male is diagnosed with IgA nephropathy. He asks about the likelihood of recurrence if he needs a kidney transplant in the future. What is the most accurate statement?
A 26-year-old male presents with nephrotic syndrome. Which initial investigation is most crucial to determine the degree of proteinuria and guide treatment?
A 26-year-old male presents with nephrotic syndrome. Which initial investigation is most crucial to determine the degree of proteinuria and guide treatment?
A 26-year-old male with nephrotic syndrome is prescribed spironolactone. What is the primary mechanism by which spironolactone helps to reduce edema in this patient?
A 26-year-old male with nephrotic syndrome is prescribed spironolactone. What is the primary mechanism by which spironolactone helps to reduce edema in this patient?
A 26-year-old male presents with a first episode of nephrotic syndrome. What is the most likely differential diagnosis?
A 26-year-old male presents with a first episode of nephrotic syndrome. What is the most likely differential diagnosis?
A 26-year-old male diagnosed with nephrotic syndrome secondary to minimal change disease initially responds well to corticosteroid therapy, but then relapses shortly after tapering the dose. What does this pattern suggest?
A 26-year-old male diagnosed with nephrotic syndrome secondary to minimal change disease initially responds well to corticosteroid therapy, but then relapses shortly after tapering the dose. What does this pattern suggest?
A 47-year-old male with IgA nephropathy is prescribed an ACE inhibitor. Which of the following assessment parameters is most crucial to monitor shortly after initiating ACE inhibitor therapy?
A 47-year-old male with IgA nephropathy is prescribed an ACE inhibitor. Which of the following assessment parameters is most crucial to monitor shortly after initiating ACE inhibitor therapy?
A 26-year-old male with nephrotic syndrome has a normal creatinine level. What is the best explanation?
A 26-year-old male with nephrotic syndrome has a normal creatinine level. What is the best explanation?
A 26-year-old male presenting with nephrotic syndrome undergoes an auto-antibody screen as part of his diagnostic workup. A negative result on this screen primarily helps to rule out which category of underlying causes for his condition?
A 26-year-old male presenting with nephrotic syndrome undergoes an auto-antibody screen as part of his diagnostic workup. A negative result on this screen primarily helps to rule out which category of underlying causes for his condition?
A 26-year-old male with nephrotic syndrome has a total urinary protein level of 6.87 g/24hr. Which of the following values represents the minimum total urinary protein level required to meet the diagnostic criteria for nephrotic syndrome?
A 26-year-old male with nephrotic syndrome has a total urinary protein level of 6.87 g/24hr. Which of the following values represents the minimum total urinary protein level required to meet the diagnostic criteria for nephrotic syndrome?
A 26-year-old male presents with nephrotic syndrome secondary to Focal Segmental Glomerulosclerosis (FSGS). A renal biopsy is performed. Which electron microscopy finding is most consistent with this diagnosis?
A 26-year-old male presents with nephrotic syndrome secondary to Focal Segmental Glomerulosclerosis (FSGS). A renal biopsy is performed. Which electron microscopy finding is most consistent with this diagnosis?
A 26-year-old male with nephrotic syndrome is treated with furosemide. Which of the following potential electrolyte imbalances is most directly associated with the mechanism of action of furosemide?
A 26-year-old male with nephrotic syndrome is treated with furosemide. Which of the following potential electrolyte imbalances is most directly associated with the mechanism of action of furosemide?
A 26-year-old male with nephrotic syndrome is diagnosed with primary focal segmental glomerulosclerosis (FSGS). Considering the natural history of primary FSGS, which of the following is the most likely long-term outcome for this patient?
A 26-year-old male with nephrotic syndrome is diagnosed with primary focal segmental glomerulosclerosis (FSGS). Considering the natural history of primary FSGS, which of the following is the most likely long-term outcome for this patient?
A 47 year old male presents with hypertension and haematuria. The lab results show a creatinine clearance of 91ml/min. What does this suggest?
A 47 year old male presents with hypertension and haematuria. The lab results show a creatinine clearance of 91ml/min. What does this suggest?
Which of the following is LEAST likely to be associated with glomerular haematuria?
Which of the following is LEAST likely to be associated with glomerular haematuria?
A 26-year-old male with nephrotic syndrome has a Cr. Clearance of 185 ml/min. What could be a possible explanation for this result?
A 26-year-old male with nephrotic syndrome has a Cr. Clearance of 185 ml/min. What could be a possible explanation for this result?
A 47-year-old male is diagnosed with IgA nephropathy. He asks what part of his kidney is affected in this condition. Which one of the following is correct?
A 47-year-old male is diagnosed with IgA nephropathy. He asks what part of his kidney is affected in this condition. Which one of the following is correct?
A 26-year-old male presents with nephrotic syndrome. He is on prednisolone, frusemide and spironolactone. What is one of the treatment goals?
A 26-year-old male presents with nephrotic syndrome. He is on prednisolone, frusemide and spironolactone. What is one of the treatment goals?
A 47-year-old male presents with IgA nephropathy. A biopsy result shows increased mesangial cellularity and segmental sclerosis. According to the Oxford scoring system, what classification is given?
A 47-year-old male presents with IgA nephropathy. A biopsy result shows increased mesangial cellularity and segmental sclerosis. According to the Oxford scoring system, what classification is given?
You are investigating a 26-year-old male for recurrent proteinuria. Which result is most likely to suggest the presence of glomerular haematuria?
You are investigating a 26-year-old male for recurrent proteinuria. Which result is most likely to suggest the presence of glomerular haematuria?
A 47-year-old male with IgA nephropathy had a renal biopsy performed. An immunofluorescence stain was done on the sample. What is the expected finding for this test?
A 47-year-old male with IgA nephropathy had a renal biopsy performed. An immunofluorescence stain was done on the sample. What is the expected finding for this test?
A 47-year-old male with IgA nephropathy has worsening hypertension and progressive decline in renal function despite treatment with an ACE inhibitor and dietary modifications. What is the most appropriate next step in management?
A 47-year-old male with IgA nephropathy has worsening hypertension and progressive decline in renal function despite treatment with an ACE inhibitor and dietary modifications. What is the most appropriate next step in management?
Which of the following best illustrates the concept of differential diagnosis?
Which of the following best illustrates the concept of differential diagnosis?
Flashcards
Case Presentation 1: Key symptoms
Case Presentation 1: Key symptoms
Recurrent swelling of legs and abdomen, proteinuria on dipstick urinalysis, no haematuria.
Key findings of Nephrotic Syndrome
Key findings of Nephrotic Syndrome
Massive proteinuria, Hypoalbuminaemia, Oedema including ascites, Hypercholesterolaemia.
Approach to Proteinuria
Approach to Proteinuria
Assess degree (nephrotic range or low level), Additional nephritic features, Other system involvement, Additional nephrotic features (alb, lipids).
Differential diagnosis of Nephrotic Syndrome
Differential diagnosis of Nephrotic Syndrome
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Primary focal and segmental glomerulosclerosis
Primary focal and segmental glomerulosclerosis
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Case Presentation 2: Key Symptoms
Case Presentation 2: Key Symptoms
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Case 2: Patient History
Case 2: Patient History
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Case 2: Creatinine Clearance
Case 2: Creatinine Clearance
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Approach to Haematuria
Approach to Haematuria
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Glomerular causes of haematuria
Glomerular causes of haematuria
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Dx: IgA Glomerulonephritis
Dx: IgA Glomerulonephritis
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IgA Glomerulonephritis Oxford scoring system
IgA Glomerulonephritis Oxford scoring system
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Study Notes
Case Presentation 1: B.S.
- B.S. is a 26-year-old male.
- He experiences recurrent swelling of the legs and abdomen.
- He shows proteinuria on dipstick urinalysis.
- He does not have haematuria
History of Presenting Complaint (HPC)
- Symptoms first appeared at 15 years old.
- Diagnosed with Nephrotic Syndrome, which responded well to steroids.
- A biopsy was not performed.
- He had recurrent courses of steroids with good response.
- Symptoms have been increasing in frequency for the last 2 years.
- Increasing use of steroids is required.
- Early relapse occurs when weaning off steroids.
- He has never been hypertensive.
- Home dipstick tests show recurrent proteinuria, but no haematuria.
- Medications include prednisolone, frusemide, and spironolactone.
Relevant Histories
- Past, family and social histories are not relevant.
Examination Findings
- Oedema is present.
- BP is 110/60.
- Cardiovascular, respiratory, and central nervous systems show "no apparent distress" (NAD).
- Ascites and abdominal striae are present.
- Urine shows 4+ protein and negative for red blood cells.
Investigations
- Full blood count (FBC), liver function tests (LFT), and urea/electrolytes (U/E) are normal.
- Albumin is 27, while cholesterol is raised.
- Auto-antibody screen is negative.
- HIV, Hep B, and Hep C tests are negative.
- Ultrasound of kidneys shows echogenic cortex with normal size
- Creatinine clearance is 185 ml/min, which is elevated, and the reason is unclear.
- Total Urinary Protein (TUP) is 6.87 g/24hr.
Summary of Findings
- Massive proteinuria
- Hypoalbuminaemia
- Oedema, including ascites
- Hypercholesterolaemia
- Low to normal serum creatinine
- Raised creatinine filtration rate
- Low blood pressure
- No blood in urine
- consistent with NEPHROTIC SYNDROME
Treatment
- Steroids are being reduced.
- ACE inhibitors are used.
- Lipid-lowering statins are prescribed.
Approach to Proteinuria
- Proteinuria degree is assessed to determine if is in the nephrotic range or low level.
- Additional nephritic features, such as BP, RBC, or acute renal failure (ARF) are checked
- Involvement of other systems are looked into
- Additional nephrotic features like albumin or lipids are assessed
- Possible aetiological factors or associations, such as chronic infections, neoplasms, or drugs, are considered.
Clinical Diagnosis
- Clinical diagnosis is Nephrotic Syndrome.
- There are no nephritic features present.
- This implies a limited differential diagnosis.
- A renal biopsy is performed.
Differential Diagnosis of Nephrotic Syndrome
- Minimal change disease
- Primary focal and segmental glomerulosclerosis
- Membranous glomerulonephritis
- Amyloidosis
- Diabetic Nephropathy
- Other rare causes
Pathology
- Segmental Sclerosis is observed.
- Sclerosed and normal glomeruli are present.
- Atrophic tubules and mild fibrosis are seen.
- Global foot process effacement is present.
Diagnosis
- Primary focal and segmental glomerulosclerosis is diagnosed.
- It is steroid responsive and steroid dependant.
- It will result in chronic renal failure.
- It may recur in transplant.
Case Presentation 2: N.M.
- N.M. is a 47-year-old male.
- Chief complaints are headaches and hypertension.
- Admitted to a peripheral hospital for severe hypertension, headache, and pedal oedema 6/52 prior to referral.
- He has experienced malaise for 2 months prior.
- He is on several anti-hypertensive drugs.
- He has a history of hypertension for 4 years.
- He had one episode of frank haematuria in 2011, persistent microscopic haematuria and 1+ proteinuria.
- Symptoms worsen with URTI.
- His sister has hypertension.
- His social history is not relevant.
Examination
- BP is 140/90.
- Pedal oedema is present.
- Cardiovascular, respiratory, abdominal and central nervous systems are normal.
- Urine shows +++ red blood cells and 1+ protein, with no organisms.
Investigations
- MSU is negative for bacteria.
- FBC, LFT, U/E are normal; serum creatinine is 94.
- Auto-antibody screen is negative.
- Creatinine clearance is 91ml/min, which is decreased.
- TUP is 1.5 g/24hr urine collection.
- Ultrasound of kidneys is normal.
- A renal biopsy is performed.
Approach to Haematuria
- Consider the urinary tract from kidney to urethra for causes.
- Glomerular causes are associated with dysmorphic RBCs in urinalysis.
- Glomerular causes may be associated with systemic diseases such as Alport’s Syndrome, IgA GN with HSP, and Vasculitis with pulmonary renal syndrome.
IgA Glomerulonephritis
- Immunofluorescence for IgA shows mesangium positivity.
- Sclerosed glomeruli and enlarged glomerulus with increased mesangial cellularity is observed.
- Small fibro-cellular crescent bottom glomerulus grades the disease.
- Trichrome staining is highlighted.
- Interstitial fibrosis indicates the stage of disease.
- Mesangial deposits are seen in IgA GN EM.
- Segmental Sclerosis is observed.
Diagnosis of IgA Glomerulonephritis
- It is the most common glomerulonephritis in the world that is biopsied.
- It can be mild, moderately aggressive, or very aggressive.
IgA Oxford Scoring System (Classification)
- M: Mesangial hypercellularity
- E: Endocapillary inflammation
- S: Segmental sclerosis
- T: Tubulointerstitial fibrosis
- C: Crescents
- Endocapillary inflammation, segmental sclerosis, advanced intertubulointerstitial fibrosis, and crescents suggest a poorer prognosis.
Learning Outcomes
- List the features of nephrotic syndrome.
- List the common and differential diagnoses of nephrotic syndrome.
- List the investigations to establish a diagnosis of nephrotic syndrome.
- Describe the pathogenesis of nephrotic syndrome.
- List the causes of haematuria.
- List glomerular causes of haematuria.
- Describe the pathology of IgA glomerulonephritis.
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