Podcast
Questions and Answers
In a patient presenting with macroscopic hematuria without associated pain, which of the following potential etiologies should be prioritized in the initial diagnostic workup?
In a patient presenting with macroscopic hematuria without associated pain, which of the following potential etiologies should be prioritized in the initial diagnostic workup?
- Benign prostatic hyperplasia
- Acute urinary tract infection
- Ureteral calculi
- Urothelial carcinoma of the bladder (correct)
A microscopic finding of red cell casts in urine sediment strongly suggests what location of hemorrhage?
A microscopic finding of red cell casts in urine sediment strongly suggests what location of hemorrhage?
- Ureter
- Renal vasculature excluding glomeruli
- Lower urinary tract
- Glomerular (correct)
A 62-year-old male presents with hematuria, lower back pain, and a palpable abdominal mass. Imaging reveals a large staghorn calculus. Which of the following best describes the calculus and its complications?
A 62-year-old male presents with hematuria, lower back pain, and a palpable abdominal mass. Imaging reveals a large staghorn calculus. Which of the following best describes the calculus and its complications?
- Composed of calcium oxalate, associated with high dietary oxalate intake, rarely obstructs
- Composed of uric acid, typically causing intermittent hematuria
- Composed of struvite, associated with recurrent UTIs, and may cause obstruction (correct)
- Composed of cystine, associated with cystinuria, typically causing flank pain only
What systemic condition would be most likely to present with hematuria due to its direct impact on the integrity of the glomerular filtration barrier?
What systemic condition would be most likely to present with hematuria due to its direct impact on the integrity of the glomerular filtration barrier?
Which of the following etiological classifications of IgA glomerulonephritis best describes the condition's primary pathophysiology regarding IgA deposition?
Which of the following etiological classifications of IgA glomerulonephritis best describes the condition's primary pathophysiology regarding IgA deposition?
If a renal biopsy of a patient with IgA glomerulonephritis reveals a significant increase in mesangial cellularity and endocapillary inflammation, according to the Oxford classification, what would these features primarily influence regarding patient management?
If a renal biopsy of a patient with IgA glomerulonephritis reveals a significant increase in mesangial cellularity and endocapillary inflammation, according to the Oxford classification, what would these features primarily influence regarding patient management?
A 25-year-old male with Alport's syndrome is considering fatherhood. Given the inheritance pattern, what is the most accurate genetic counseling information you can provide?
A 25-year-old male with Alport's syndrome is considering fatherhood. Given the inheritance pattern, what is the most accurate genetic counseling information you can provide?
Which of the following electron microscopy findings is most consistent with Alport's Syndrome?
Which of the following electron microscopy findings is most consistent with Alport's Syndrome?
In ANCA-associated vasculitis, which pathogenic mechanism directly leads to glomerular injury and subsequent hematuria?
In ANCA-associated vasculitis, which pathogenic mechanism directly leads to glomerular injury and subsequent hematuria?
An individual with ANCA-associated vasculitis presents with both hemoptysis and hematuria. Which diagnosis is most likely?
An individual with ANCA-associated vasculitis presents with both hemoptysis and hematuria. Which diagnosis is most likely?
What is the primary target antigen in Goodpasture syndrome?
What is the primary target antigen in Goodpasture syndrome?
A patient with hematuria and pulmonary hemorrhage is suspected of having Goodpasture syndrome. Which of the following laboratory findings would best support this diagnosis?
A patient with hematuria and pulmonary hemorrhage is suspected of having Goodpasture syndrome. Which of the following laboratory findings would best support this diagnosis?
A patient presents with nephrotic syndrome, including significant proteinuria, edema, and hyperlipidemia. This clinical presentation is most directly caused by damage to what?
A patient presents with nephrotic syndrome, including significant proteinuria, edema, and hyperlipidemia. This clinical presentation is most directly caused by damage to what?
Which of the following clinical findings in a patient with nephrotic syndrome would most strongly suggest the presence of an underlying glomerular inflammation?
Which of the following clinical findings in a patient with nephrotic syndrome would most strongly suggest the presence of an underlying glomerular inflammation?
A 6-year-old child presents with nephrotic syndrome following an upper respiratory infection. Initial investigations, including light microscopy, show normal glomeruli. What is the most likely diagnosis?
A 6-year-old child presents with nephrotic syndrome following an upper respiratory infection. Initial investigations, including light microscopy, show normal glomeruli. What is the most likely diagnosis?
What specific ultrastructural finding confirms the diagnosis for minimal change disease?
What specific ultrastructural finding confirms the diagnosis for minimal change disease?
A kidney biopsy from a patient with nephrotic syndrome shows focal and segmental glomerulosclerosis (FSGS). Which clinical feature is most critical in determining the subtype and guiding subsequent treatment?
A kidney biopsy from a patient with nephrotic syndrome shows focal and segmental glomerulosclerosis (FSGS). Which clinical feature is most critical in determining the subtype and guiding subsequent treatment?
A patient diagnosed with steroid-resistant FSGS is found to have a mutation affecting nephrin. Which mechanism is most likely responsible for the patient's condition?
A patient diagnosed with steroid-resistant FSGS is found to have a mutation affecting nephrin. Which mechanism is most likely responsible for the patient's condition?
What is the primary antigen targeted in most cases of membranous glomerulonephritis?
What is the primary antigen targeted in most cases of membranous glomerulonephritis?
A patient with nephrotic syndrome has a renal biopsy that shows thickened capillary loops with 'spikes' on silver stain. Immunofluorescence reveals granular IgG deposits. What is the likely diagnosis?
A patient with nephrotic syndrome has a renal biopsy that shows thickened capillary loops with 'spikes' on silver stain. Immunofluorescence reveals granular IgG deposits. What is the likely diagnosis?
Where do fibrils primarily deposit in the kidney in cases associated with amyloidosis?
Where do fibrils primarily deposit in the kidney in cases associated with amyloidosis?
Which underlying condition is most commonly associated with amyloidosis causing nephrotic syndrome?
Which underlying condition is most commonly associated with amyloidosis causing nephrotic syndrome?
What microscopic finding on a kidney biopsy would most strongly suggest a diagnosis of amyloidosis?
What microscopic finding on a kidney biopsy would most strongly suggest a diagnosis of amyloidosis?
A patient with IgA nephropathy develops rapidly progressive glomerulonephritis (RPGN). What additional pathological finding would you expect to see?
A patient with IgA nephropathy develops rapidly progressive glomerulonephritis (RPGN). What additional pathological finding would you expect to see?
The presence of crescents in a kidney biopsy from a patient is indicative of?
The presence of crescents in a kidney biopsy from a patient is indicative of?
Segmental sclerosis observed in a glomerulus indicates which pathological condition?
Segmental sclerosis observed in a glomerulus indicates which pathological condition?
In a patient with pulmonary-renal syndrome, which of the following antibodies are most specific to Goodpasture syndrome?
In a patient with pulmonary-renal syndrome, which of the following antibodies are most specific to Goodpasture syndrome?
Which of the following conditions commonly presents with a combination of lung hemorrhage and glomerulonephritis due to antibody-mediated injury?
Which of the following conditions commonly presents with a combination of lung hemorrhage and glomerulonephritis due to antibody-mediated injury?
Anti-GBM disease results in the activation of PMN (polymorphonuclear neutrophils) leading to glomerular injury and subsequent hematuria. What is the process that contributes to activation of PMNs?
Anti-GBM disease results in the activation of PMN (polymorphonuclear neutrophils) leading to glomerular injury and subsequent hematuria. What is the process that contributes to activation of PMNs?
In minimal change disease, why is the kidney biopsy often normal when viewed under light microscopy?
In minimal change disease, why is the kidney biopsy often normal when viewed under light microscopy?
In which of the following glomerular diseases are 'spikes' observed on silver staining due to new basement membrane formation between subepithelial deposits?
In which of the following glomerular diseases are 'spikes' observed on silver staining due to new basement membrane formation between subepithelial deposits?
A patient presents with nephrotic syndrome and is diagnosed with FSGS. Which of the following best describes the long-term prognosis for patients with focal segmental glomerulosclerosis (FSGS)?
A patient presents with nephrotic syndrome and is diagnosed with FSGS. Which of the following best describes the long-term prognosis for patients with focal segmental glomerulosclerosis (FSGS)?
IgA nephropathy is characterized by the deposition of IgA in the mesangium. What diagnostic method is best suited to visualize these deposits?
IgA nephropathy is characterized by the deposition of IgA in the mesangium. What diagnostic method is best suited to visualize these deposits?
In a patient with Alport's syndrome, which of the following findings is most likely to be present in addition to hematuria?
In a patient with Alport's syndrome, which of the following findings is most likely to be present in addition to hematuria?
In the context of glomerular diseases causing hematuria, what role do neutrophils play in ANCA-associated vasculitis?
In the context of glomerular diseases causing hematuria, what role do neutrophils play in ANCA-associated vasculitis?
How does Goodpasture syndrome typically affect the kidneys and lungs?
How does Goodpasture syndrome typically affect the kidneys and lungs?
Damage to the visceral epithelial cells (podocytes) of the glomerulus leads directly to which of the following?
Damage to the visceral epithelial cells (podocytes) of the glomerulus leads directly to which of the following?
A child presents with proteinuria and edema, but the glomerular architecture appears normal on light microscopy. What is the most likely underlying diagnosis?
A child presents with proteinuria and edema, but the glomerular architecture appears normal on light microscopy. What is the most likely underlying diagnosis?
Focal segmental glomerulosclerosis (FSGS) is characterized by sclerosis affecting some glomeruli and parts of each glomerulus, but not all of it. What best characterizes this condition?
Focal segmental glomerulosclerosis (FSGS) is characterized by sclerosis affecting some glomeruli and parts of each glomerulus, but not all of it. What best characterizes this condition?
When a patient with proteinuria is diagnosed with Membranous Glomerulonephritis, which receptor is the primary antigen located on Podocytes?
When a patient with proteinuria is diagnosed with Membranous Glomerulonephritis, which receptor is the primary antigen located on Podocytes?
Which immune complex deposition disease presents with severe Nephrotic Syndrome, deposition of fibrils in the mesangium/capillary loops, multiple types, and is commonly due to multiple myeloma/plasma cell dyscrasia?
Which immune complex deposition disease presents with severe Nephrotic Syndrome, deposition of fibrils in the mesangium/capillary loops, multiple types, and is commonly due to multiple myeloma/plasma cell dyscrasia?
Urothelial carcinoma is a cause of Hematuria associated with the Ureteric, Bladder, and Prostate but NOT Urethral. Which type of carcinoma is specifically likely to be associated with the Ureteric, Bladder and Prostate?
Urothelial carcinoma is a cause of Hematuria associated with the Ureteric, Bladder, and Prostate but NOT Urethral. Which type of carcinoma is specifically likely to be associated with the Ureteric, Bladder and Prostate?
Which cause of Haematuria can be categorized as 'Latrogenic'?
Which cause of Haematuria can be categorized as 'Latrogenic'?
The following are causes of haematuria associated with which part of the body; UTI, STD's, Urethral Caruncle?
The following are causes of haematuria associated with which part of the body; UTI, STD's, Urethral Caruncle?
In a patient presenting with both haematuria and proteinuria, which of the following glomerular pathologies is LEAST likely to be associated with a painless presentation?
In a patient presenting with both haematuria and proteinuria, which of the following glomerular pathologies is LEAST likely to be associated with a painless presentation?
A 30-year-old male presents with microscopic haematuria detected during a routine medical examination. His renal function is normal, and he has no other symptoms. Given the context alone, which of the following conditions is most likely, needing further investigation?
A 30-year-old male presents with microscopic haematuria detected during a routine medical examination. His renal function is normal, and he has no other symptoms. Given the context alone, which of the following conditions is most likely, needing further investigation?
Which of the following conditions is most likely to cause haematuria due to the direct impact on the integrity of the glomerular filtration barrier without significant inflammation?
Which of the following conditions is most likely to cause haematuria due to the direct impact on the integrity of the glomerular filtration barrier without significant inflammation?
What underlying genetic defect is most likely in a 10-year-old boy presenting with haematuria, sensorineural deafness, and progressive visual impairment?
What underlying genetic defect is most likely in a 10-year-old boy presenting with haematuria, sensorineural deafness, and progressive visual impairment?
Which of the following pathological processes is most directly responsible for the glomerular injury observed in ANCA-associated vasculitis?
Which of the following pathological processes is most directly responsible for the glomerular injury observed in ANCA-associated vasculitis?
A patient with ANCA-associated vasculitis presents with haematuria and rapidly progressive glomerulonephritis (RPGN). What is the most critical next step in managing this patient?
A patient with ANCA-associated vasculitis presents with haematuria and rapidly progressive glomerulonephritis (RPGN). What is the most critical next step in managing this patient?
In Goodpasture syndrome, the disease targets a specific component of the glomerular basement membrane (GBM). What is the antigen?
In Goodpasture syndrome, the disease targets a specific component of the glomerular basement membrane (GBM). What is the antigen?
A patient with Goodpasture syndrome presents with haematuria and pulmonary haemorrhage. Which treatment approach would be the most effective in directly addressing the underlying pathological mechanism?
A patient with Goodpasture syndrome presents with haematuria and pulmonary haemorrhage. Which treatment approach would be the most effective in directly addressing the underlying pathological mechanism?
A 5-year-old child presents with nephrotic syndrome characterized by edema, proteinuria, and hypoalbuminemia, but without haematuria or hypertension. A kidney biopsy is performed. What is the most likely diagnosis?
A 5-year-old child presents with nephrotic syndrome characterized by edema, proteinuria, and hypoalbuminemia, but without haematuria or hypertension. A kidney biopsy is performed. What is the most likely diagnosis?
A patient with minimal change disease is treated with corticosteroids and shows significant improvement in their proteinuria. Which of the following mechanisms is most likely responsible for the therapeutic effect of corticosteroids in this disease?
A patient with minimal change disease is treated with corticosteroids and shows significant improvement in their proteinuria. Which of the following mechanisms is most likely responsible for the therapeutic effect of corticosteroids in this disease?
A patient with nephrotic syndrome has a kidney biopsy that shows focal and segmental glomerulosclerosis (FSGS). Which of the following clinical findings would suggest a primary rather than a secondary cause of FSGS?
A patient with nephrotic syndrome has a kidney biopsy that shows focal and segmental glomerulosclerosis (FSGS). Which of the following clinical findings would suggest a primary rather than a secondary cause of FSGS?
A child with steroid-resistant FSGS is found to have a mutation affecting podocin. Which of the following mechanisms is most likely responsible for the patient's condition?
A child with steroid-resistant FSGS is found to have a mutation affecting podocin. Which of the following mechanisms is most likely responsible for the patient's condition?
What is the most commonly implicated antigen targeted by autoantibodies in primary (idiopathic) membranous glomerulonephritis (MGN)?
What is the most commonly implicated antigen targeted by autoantibodies in primary (idiopathic) membranous glomerulonephritis (MGN)?
A 55-year-old patient presents with nephrotic syndrome. A kidney biopsy reveals thickened glomerular basement membranes with subepithelial deposits and 'spikes' on silver stain. Immunofluorescence shows granular IgG and C3 deposits. What is the next diagnostic step to evaluate whether this MGN may be secondary to other causes?
A 55-year-old patient presents with nephrotic syndrome. A kidney biopsy reveals thickened glomerular basement membranes with subepithelial deposits and 'spikes' on silver stain. Immunofluorescence shows granular IgG and C3 deposits. What is the next diagnostic step to evaluate whether this MGN may be secondary to other causes?
In patients with amyloidosis-related nephrotic syndrome, where are the amyloid fibrils primarily deposited within the kidney?
In patients with amyloidosis-related nephrotic syndrome, where are the amyloid fibrils primarily deposited within the kidney?
What is the most common underlying systemic condition associated with amyloidosis that leads to nephrotic syndrome?
What is the most common underlying systemic condition associated with amyloidosis that leads to nephrotic syndrome?
A patient presents with nephrotic syndrome, and a kidney biopsy shows evidence of amyloid deposition. Which of the following staining techniques is most reliable for confirming the presence and type of amyloid?
A patient presents with nephrotic syndrome, and a kidney biopsy shows evidence of amyloid deposition. Which of the following staining techniques is most reliable for confirming the presence and type of amyloid?
A patient with IgA nephropathy develops rapidly progressive glomerulonephritis (RPGN). Which of the following pathological findings would be most indicative of this progression on a kidney biopsy?
A patient with IgA nephropathy develops rapidly progressive glomerulonephritis (RPGN). Which of the following pathological findings would be most indicative of this progression on a kidney biopsy?
In a patient with pulmonary-renal syndrome, which of the following autoantibodies is most specifically associated with Goodpasture syndrome alone?
In a patient with pulmonary-renal syndrome, which of the following autoantibodies is most specifically associated with Goodpasture syndrome alone?
Which process directly contributes to glomerular injury in Anti-GBM disease ultimately leading to haematuria?
Which process directly contributes to glomerular injury in Anti-GBM disease ultimately leading to haematuria?
Why are light microscopy results typically normal in cases of minimal change disease?
Why are light microscopy results typically normal in cases of minimal change disease?
Which immunological complexes result in new basement membrane formation between subepithelial deposits, observed as 'spikes' on silver staining?
Which immunological complexes result in new basement membrane formation between subepithelial deposits, observed as 'spikes' on silver staining?
A patient is diagnosed with FSGS. Is the following statement true or false? 'Patients with FSGS typically have a good long-term prognosis, especially if they are steroid-sensitive'.
A patient is diagnosed with FSGS. Is the following statement true or false? 'Patients with FSGS typically have a good long-term prognosis, especially if they are steroid-sensitive'.
In IgA nephropathy, which diagnostic method is best suited to visualize the IgA in the mesangium?
In IgA nephropathy, which diagnostic method is best suited to visualize the IgA in the mesangium?
A young male patient with Alport's syndrome is undergoing genetic counseling. What associated finding is important?
A young male patient with Alport's syndrome is undergoing genetic counseling. What associated finding is important?
In the setting of glomerular diseases causing haematuria, what is the primary role of neutrophils in ANCA-associated vasculitis?
In the setting of glomerular diseases causing haematuria, what is the primary role of neutrophils in ANCA-associated vasculitis?
Damage to the visceral epithelial cells (podocytes) of the glomerulus directly leads to what?
Damage to the visceral epithelial cells (podocytes) of the glomerulus directly leads to what?
A person with proteinuria and edema has normal glomerular architecture on light microscopy. What is the most likely diagnosis?
A person with proteinuria and edema has normal glomerular architecture on light microscopy. What is the most likely diagnosis?
Focal segmental glomerulosclerosis (FSGS) is characterised by sclerosis affecting some glomeruli and parts of each glomerulus, but not all of it. What best characterizes FSGS?
Focal segmental glomerulosclerosis (FSGS) is characterised by sclerosis affecting some glomeruli and parts of each glomerulus, but not all of it. What best characterizes FSGS?
Which immune complex deposition disease presents with severe Nephrotic Syndrome, deposition of fibrils in the mesangium/capillary loops. What type of amyloid is most frequently found in these cases?
Which immune complex deposition disease presents with severe Nephrotic Syndrome, deposition of fibrils in the mesangium/capillary loops. What type of amyloid is most frequently found in these cases?
Haematuria can be categorised as 'Latrogenic', which of the examples below is the most relevant?
Haematuria can be categorised as 'Latrogenic', which of the examples below is the most relevant?
The following can contribute to Haematuria: UTI, STD's, Urethral Caruncle. Where would you typically find these injuries?
The following can contribute to Haematuria: UTI, STD's, Urethral Caruncle. Where would you typically find these injuries?
Flashcards
Haematuria
Haematuria
Blood in the urine, can be microscopic or macroscopic (gross).
Haematuria Classification
Haematuria Classification
A classification method that divides based on the presence or absence of pain and whether it is treated medically or surgically.
Causes of Haematuria
Causes of Haematuria
Includes systemic conditions, kidney, ureter, bladder and urethra disorders.
IgA Glomerulonephritis
IgA Glomerulonephritis
Signup and view all the flashcards
Alport's Syndrome
Alport's Syndrome
Signup and view all the flashcards
ANCA Vasculitis
ANCA Vasculitis
Signup and view all the flashcards
Goodpasture Syndrome
Goodpasture Syndrome
Signup and view all the flashcards
ANCA and Anti GBM
ANCA and Anti GBM
Signup and view all the flashcards
Causes of Haematuria - Ureteric
Causes of Haematuria - Ureteric
Signup and view all the flashcards
Causes of Haematuria - Bladder
Causes of Haematuria - Bladder
Signup and view all the flashcards
Causes of Haematuria - Urethral
Causes of Haematuria - Urethral
Signup and view all the flashcards
Causes of Haematuria - Prostatic
Causes of Haematuria - Prostatic
Signup and view all the flashcards
Nephrotic Syndrome
Nephrotic Syndrome
Signup and view all the flashcards
Main Causes of Nephrotic Syndrome
Main Causes of Nephrotic Syndrome
Signup and view all the flashcards
Minimal Change Disease
Minimal Change Disease
Signup and view all the flashcards
Pathology of Minimal Change Disease
Pathology of Minimal Change Disease
Signup and view all the flashcards
Focal Segmental Glomerulosclerosis (FSGS)
Focal Segmental Glomerulosclerosis (FSGS)
Signup and view all the flashcards
Membranous Glomerulonephritis
Membranous Glomerulonephritis
Signup and view all the flashcards
Amyloid and the Kidney
Amyloid and the Kidney
Signup and view all the flashcards
Study Notes
- Renal Pathology 2 covers glomerular causes of hematuria and nephrotic syndrome.
Clinical Presentation of Haematuria
- Blood present in the urine is a sign of haematuria.
- Microscopic haematuria presents as trace amounts of blood.
- Macroscopic haematuria presents as gross or frank blood.
- In microscopic hematuria, the urine may appear normal.
- Red cell casts in urine indicate glomerular bleeding.
- Blood in the tubule is another sign of hematuria.
Classification and Causes of Haematuria
- Haematuria can be classified as painless or painful, and medical or surgical.
- Systemic causes of haematuria can include clotting disorders, low platelets, and warfarin use.
- Other causes can include kidney, ureter, bladder, and urethra related issues.
- In the kidneys, glomerulonephritis, IgA nephropathy, acute post-infectious glomerulonephritis, thin membranes, Alport's syndrome, vasculitis, and neoplasms can cause haematuria.
- Ureteral obstruction can also cause haematuria.
IgA Glomerulonephritis
- Direct immunofluorescence for IgA shows IgA deposits in the glomeruli.
- Histological findings in IgA glomerulonephritis include sclerosed glomeruli, enlarged glomeruli with increased mesangial cellularity and segmental sclerosing lesions.
- Electron microscopy reveals mesangial deposits.
Oxford Classification for IgA
- The Oxford system is used for IgA classification and prognosis using the acronym "MEST-C"
- M stands for Mesangial cellularity increase.
- E stands for Endocapillary inflammation.
- S stands for Segmental sclerosis.
- T stands for Tubulo-Interstitial Fibrosis.
- C stands for Crescents.
Thin Membrane Nephropathy and Alport's Syndrome
- Thin membrane nephropathy is also known as benign familial haematuria or March haematuria
- It typically has a benign course with thin membranes
- Females may be carriers of the Alport's gene.
- Alport's Syndrome is associated with nerve deafness and eye abnormalities.
- It is X-linked in 85% of cases.
- Males typically develop full disease, with 90% developing end-stage kidney disease by age 40.
- Females can be carriers with thin membranes.
- Genetic counseling is available for milder forms of the disease
Vasculitis
- ANCA vasculitis is a systemic disease
- It is characterized by anti-neutrophil cytoplasmic autoantibodies
- Neutrophils are activated, with molecules protruding through the PMN cell wall.
- Antibodies bind to myeloperoxidase or proteinase 3, causing blood vessel wall inflammation.
ANCA & PMN Activation
- Activated PMNs can create "holes" in the basement membrane.
- ANCA can present as cytoplasmic or perinuclear patterns.
Goodpasture Syndrome
- Goodpasture syndrome is an anti-glomerular basement membrane disease involving autoantibodies against the alpha3 chain of collagen type 4.
- It affects the kidneys and lungs.
- It also results in PMN activation, similar to ANCA disease.
- Linear IgG positivity is observed in anti-GBM disease.
- Rupture of capillary loops can also occur.
ANCA and Anti-GBM
- ANCA and Anti-GBM diseases involve crossover immunological responses and molecular mimicry.
- Neutrophil activating processes, such as infection or allergy, may cause them.
- Antibodies target neutrophil proteins (ANCA) or collagen proteins (Anti-GBM).
Additional Causes of Haematuria
- Ureteric causes include urothelial carcinoma, urinary tract infections (UTIs), and calculi.
- Bladder-related causes include: urothelial carcinoma, UTIs, calculi, and iatrogenic causes such as catheter use.
- Urethral causes include UTIs, STDs, and urethral caruncles.
- Prostatic causes include BPH, prostate cancer, and prostatitis.
Clinical Presentation of Nephrotic Syndrome
- Nephrotic syndrome is different from nephritis, due to nephrosis vs nephritis.
- Absence of inflammation leads to absence of raised blood pressure and haematuria.
- Pathogenesis involves damage to visceral epithelial cells.
Normal Kidney Ultra Structure
- Normal filter membrane ultra structure includes parietal/visceral epithetlial cells, BM (basement membrane), Bowman's space, capillary etc
Nephrotic Syndrome: Clinical Features
- Key clinical features include:
- Proteinuria (≥ 3.5g/24hrs)
- Oedema
- Hypoalbuminaemia
- Hypercholesterolaemia
- Thrombophilia
Main Causes of Nephrotic Syndrome
- Primary causes of nephrotic syndrome
- Minimal change disease
- Focal and segmental glomerulosclerosis
- Membranous glomerulonephritis
- Amyloidosis
- Membranoproliferative glomerulonephritis(MPGN) can present with either nephrotic or acute nephritic syndrome
Nephrotic Syndrome with Normal Creatinine and Raised Creatinine: Signficance
- Clinical features predict the site of the pathology.
- If the patient presents with nephrotic syndrome with normal creatinine, it may indicate glomerular damage without inflammation.
- Raised creatinine along with blood in the urine typically indicates glomerular inflammation.
Minimal Change Disease
- Minimal change disease affects individuals of all ages, especially children and the elderly
- It leads to acute onset of florid nephrotic syndrome with albuminuria often exceeding 20g/24 hours.
- Minimal change disease is self-limiting and responds well to steroids, leading to full recovery.
- It results from direct injury to foot processes by a short term agent that is currently unknown.
Pathology of Minimal Change Disease
- On light microscopy (LM), the glomeruli appear normal.
- Immunofluorescence microscopy (F.M.) shows no deposits
- Electron microscopy shows diffuse flattening and simplification of foot processes.
Focal and Segmental Glomerulosclerosis (FSGS)
- FSGS always presents with nephrotic range proteinuria
- There are 2 main types of FSGS
- Steroid responsive/dependent
- Steroid unresponsive
- Lesions are described as diffuse, global, focal, and segmental.
FSGS: Steroid Responsive/Dependent
- a circulating factor can cause foot process injury
- Inevitably leads to end-stage kidney disease over several years or decades
- Likely to come back in transplants due to the presence of the circulating factor even after the transplant.
FSGS: Steroid Unresponsive
- The condition occurs due to an abnormality/absence of foot process protein.
- The most profound form is due to nephrin abnormality or Finnish type congenital NS.
- There have since been dozens of abnormalities found, for which cytogenetic tests are now available.
- FSGS does not come back in transplants.
Foot process
- The foot process has many components, an absence of abnormality of which can cause FSGS usually resulting in chronic renal failure.
Membranous Glomerulonephritis
- Can be described as an immune complex deposition disease
- It is also a cross over immunological disease
- The antigen is Phospholipase A2 receptor, PLA2, which is abundant in foot processes of epithelial cells
- The antibody that is seen is produced as a reaction to different diseases and/or drugs
- Responds well to theraphy with a 3 years recovery time
Membranous Glomerulonephritis findings
- Capillary loops in MGN are thickened
- "Spikes” are new membrane between deposits
- An abundance of IgG in MGN
- Deposits on epithelial side of BM in MGN, with Note no inflammation
Amyloid and the Kidney
- The kidney is often involved.
- Fibrils deposit in mesangium and capillary loops, as well as arterioles and interstitium.
- Presents with severe nephrotic syndrome (NS) and some renal function impairment.
- All types of amyloid can cause kidney damage
- The most commonly seen is due to multiple myeloma or plasma cell dyscrasia.
- Amyloid deposits have a waxy, starch like appearance.
Studying That Suits You
Use AI to generate personalized quizzes and flashcards to suit your learning preferences.