GLOMERULAR DISEASES Flashcards
What substance holds water intravascularly and intracellularly, preventing edema?
Albumin
What do dysmorphic red blood cells look like under a microscope?
Mickey Mouse
What does frothy or bubbly urine indicate?
Proteinuria
What is the threshold for detecting proteinuria on an ordinary urinalysis?
300 mg/day
What is one of the earliest signs of diabetic nephropathy?
Microalbuminuria
What type of casts are specific for glomerulonephritis?
Red blood cell casts
What type of casts are indicative of chronic kidney disease?
Waxy, broad casts
What is the gold standard for diagnosing glomerular diseases?
Renal biopsy
What stain is used to detect amyloid deposits in a kidney biopsy?
Congo red
How much proteinuria defines nephrotic syndrome?
> 3 g/day
What are the primary characteristics of nephrotic syndrome?
Proteinuria, hypoalbuminemia, edema, hyperlipidemia
What type of red blood cells are specific for nephritic syndrome?
Dysmorphic RBCs
What is a hallmark of nephritic syndrome seen in urine microscopy?
Red cell casts
What histologic feature in glomerular diseases indicates rapidly progressive or severe kidney disease?
Crescents
What type of glomerular injury is associated with nephrotic syndrome?
Podocyte injury
What type of glomerular injury is associated with nephritic syndrome?
Endothelial or mesangial cell injury
Which immune deposits are more nephrogenic: in-situ or circulating immune complexes?
In-situ immune deposits
What are the three final pathways of renal damage in glomerular diseases?
Interstitial nephritis, renal fibrosis, tubular atrophy
How do acute and chronic tubulointerstitial nephritis differ?
Acute is reversible (PMNs), chronic is irreversible (fibrosis, atrophy)
What is the purpose of the PAS stain in renal biopsies?
To stain carbohydrate moieties in the glomerular tuft and tubules
What does the presence of microalbuminuria signify in diabetic nephropathy?
An early sign of kidney damage below the detection threshold of dipsticks
What is the primary microscopic feature of Minimal Change Disease (MCD)?
Podocyte effacement seen on electron microscopy.
What percentage of nephrotic syndrome in children is caused by Minimal Change Disease (MCD)?
70-90% of nephrotic syndrome in childhood.
What first-line treatment is used for Minimal Change Disease (MCD) in children?
Prednisone 60 mg/m²/day for 4 to 6 weeks.
What are the common secondary associations of Minimal Change Disease (MCD)?
Hodgkin’s lymphoma, NSAID use, and allergies (e.g., food, dust).
What is the hallmark clinical feature of Focal Segmental Glomerulosclerosis (FSGS)?
Proteinuria, which may be nephrotic or non-nephrotic.
What is the collapsing variant of FSGS characterized by?
Segmental collapse of capillaries, hypertrophy of epithelial cells, and protein resorption droplets in podocytes.
What is a poor prognostic factor for FSGS?
Nephrotic-range proteinuria (>3 g/day).
What biopsy finding is diagnostic of Membranous Nephropathy (MN)?
Subepithelial immune complex deposits seen on electron microscopy.
What is the most common cause of chronic renal failure in diabetic patients?
Diabetic Nephropathy.
What are Kimmelstiel-Wilson nodules, and in which condition are they found?
Eosinophilic PAS-positive nodules, found in Diabetic Nephropathy.
What is the defining electron microscopy feature of Fibrillary Glomerulonephritis?
Randomly arranged nonbranching fibrils ~20 nm in diameter.
What stain is diagnostic of amyloidosis, and what characteristic does it show?
Congo red stain shows apple-green birefringence under polarized light.
What is the main treatment goal for AL Amyloidosis?
Decrease the production of monoclonal light chains.
What organ is most commonly affected by Amyloidosis?
Kidneys (in ~50% of cases).
What are the risk factors for Diabetic Nephropathy?
Hyperglycemia, hypertension, dyslipidemia, smoking, and family history of diabetes.
What is the first-line conservative therapy for Membranous Nephropathy (MN)?
RAAS blockade.
What is the median survival for patients with AA Amyloidosis?
Approximately 2 years.
What are common infections associated with Minimal Change Disease (MCD)?
Viral and parasitic infections.
Which variant of FSGS has a good prognosis and marked nephrosis?
Glomerular Tip Lesion Variant.
How is microalbuminuria defined in Diabetic Nephropathy?
30-300 mg/24 hours or <30 mg/day.
What is the pathognomonic sign of Diabetic Nephropathy on light microscopy?
Kimmelstiel-Wilson nodules.
What are the features of nephrotic syndrome?
Proteinuria, hypoalbuminemia, hyperlipidemia, and edema.
What percentage of nephrotic syndrome cases in adults is caused by Membranous Nephropathy?
Approximately 20%.
Which drugs are used in steroid-resistant cases of Minimal Change Disease (MCD)?
Cyclosporine or Cyclophosphamide.
What is the typical demographic for AL Amyloidosis?
Patients over 50 years old, with men affected twice as often as women.
What is the classic presentation of Poststreptococcal Glomerulonephritis (PSGN)?
Tea-colored urine, hematuria, pyuria, RBC casts, edema, hypertension, and oliguric renal failure.
What are the diagnostic markers for PSGN in the first week of symptoms?
Decreased CH50 and C3, normal C4; positive rheumatoid factor, cryoglobulins, circulating immune complexes, and ANCA-MPO; increased ASO titer, anti-DNAse, and antihyaluronidase antibodies.
What is the typical prognosis for PSGN?
Good, with most children experiencing resolution of hematuria and proteinuria within 3-6 weeks. Persistent symptoms may indicate misdiagnosis or chronic PSGN.
What is the pathognomonic gross appearance in Subacute Bacterial Endocarditis (SBE)-associated glomerulonephritis?
Subcapsular hemorrhages with a ‘flea-bitten’ appearance.
What are the most common comorbidities in SBE-associated glomerulonephritis?
Valvular heart disease, IV drug use, Hepatitis C, and Diabetes Mellitus.
What is the most common renal manifestation of Lupus Nephritis?
Proteinuria, often accompanied by hematuria, hypertension, and active urinary sediment with RBC casts.
List the DOPAMINE RASH criteria for diagnosing systemic lupus erythematosus (SLE).
Discoid rash, Oral ulcers, Photosensitivity, Arthritis, Malar rash, Immunologic disorder, Neurologic disorder, Renal disorder, Antinuclear antibody, Serositis, Hematologic disorder.
What are the main classifications of Lupus Nephritis based on biopsy findings?
Class I: Minimal mesangial, Class II: Mesangial proliferative, Class III: Focal proliferative, Class IV: Diffuse proliferative, Class V: Membranous, Class VI: Advanced sclerosing.
What are the two most common clinical presentations of IgA Nephropathy?
Recurrent episodes of macroscopic hematuria during or after an upper respiratory tract infection and persistent asymptomatic microscopic hematuria.
What are the poor prognostic factors in Henoch-Schönlein Purpura (HSP)-associated nephritis?
Older age, acute nephritic presentation, severe nephrotic syndrome, and biopsy findings of glomerular fibrinoid necrosis or crescents.
What is the triad of Wegener’s Granulomatosis (Granulomatosis with Polyangiitis)?
Vasculitis, granulomatous inflammation of the upper and lower respiratory tracts, and glomerulonephritis.
What characterizes Anti-GBM Disease (Goodpasture’s Syndrome)?
Circulating antibodies targeting type IV collagen in the glomerular basement membrane, leading to pulmonary hemorrhage and glomerulonephritis.
What are the treatment goals for severe Class IV Lupus Nephritis?
Aggressive immunosuppression with corticosteroids and cyclophosphamide or mycophenolate mofetil, followed by maintenance therapy.
What are the risk factors for loss of renal function in IgA Nephropathy?
Hypertension, proteinuria, absence of macroscopic hematuria episodes, older age at onset, and extensive glomerulosclerosis or interstitial fibrosis.
What are the classic tetrad of symptoms in Henoch-Schönlein Purpura (HSP)?
Dermal involvement (purpura), gastrointestinal disease, joint involvement, and glomerulonephritis.
What is MPGN Type I commonly associated with?
Persistent Hepatitis C infections, autoimmune diseases (e.g., lupus), cryoglobulinemia, or neoplastic disease.
What age group is most commonly affected by MPGN Type I?
Children aged 8-16 years.
What gender is affected equally by MPGN Type I?
Male = Female.
What characterizes MPGN Type I on renal biopsy?
Mesangial proliferation with lobular segmentation, mesangial interposition between capillary basement membrane and endothelial cells, producing a double contour or ‘tram-tracking.’
What are the clinical features of MPGN Type I?
Nephrotic range proteinuria, hematuria, pyuria, hypertension, and nephritic picture with RPGN.
What is the prognosis of MPGN Type I 10 years after diagnosis?
50% of patients develop ESRD.
What is the treatment for MPGN Type I?
RAAS inhibitors, glucocorticoids, anticoagulation, and treatment of underlying conditions like hepatitis or neoplasia.
What are poor prognostic factors in MPGN Type I?
Hypertension, nephrotic syndrome, and renal insufficiency.
What is a key feature of MPGN Type II?
Low serum C3 with dense thickening of the GBM containing ribbons of dense deposits.
What age group is most commonly affected by MPGN Type II?
Children and young adults.
What are common clinical features of MPGN Type II?
Hematuria, proteinuria, hypertension (mild or severe), and kidney dysfunction.
What is a poor prognostic factor for Dense Deposit Disease in MPGN Type II?
The prognosis is worse in adults than in children.
What is the main treatment for MPGN Type II?
Inhibition of angiotensin II (e.g., RAAS inhibitors).
What is the inheritance pattern of Alport’s Syndrome?
Usually X-linked.
What are the clinical features of Alport’s Syndrome?
Persistent microscopic hematuria, episodic gross hematuria, mild proteinuria, chronic glomerulosclerosis, sensorineural deafness, and ocular abnormalities.
What is the treatment strategy for Alport’s Syndrome?
Proteinuria reduction, aggressive control of hypertension (e.g., ACEI), and renal replacement therapy.
What are the clinical features of Thin Basement Membrane Nephropathy?
Persistent or recurrent hematuria, typically not associated with proteinuria, hypertension, or loss of renal function.
What are the characteristic features of Nail-Patella Syndrome?
Tetrad of anomalies: nail dysplasia, patellar aplasia, elbow and iliac horn abnormalities.
What are common features of Hypertensive Nephropathy?
Systemic hypertension causing arteriosclerosis, chronic nephrosclerosis, and interstitial fibrosis.
What is the treatment for Hypertensive Nephrosclerosis?
RAAS inhibitors, combination therapy (e.g., ACEI + HCTZ), and strict blood pressure control (<130/80 in CKD or DM).
What is the main pathology in Cholesterol Emboli?
Irregular emboli trapped in microcirculation causing ischemic damage and inflammation.
What is the characteristic renal lesion in Sickle Cell Disease?
FSGS, interstitial nephritis, renal infarction, and hyposthenuria.
What are the two major conditions causing Thrombotic Microangiopathies?
TTP and HUS.
What is the recommended treatment for TTP and HUS?
Plasmapheresis.
What is the defining renal feature of Antiphospholipid Antibody Syndrome (APAS)?
Proteinuria, active urinary sediment, hypertension, and progressive renal dysfunction.
What is the mainstay treatment for APAS with recurrent thrombosis?
Heparin followed by warfarin (target INR >3).
What is the renal pathology associated with HIV?
HIV-associated nephropathy (HIVAN) with FSGS (collapsing type).
What is the treatment for HIV-associated nephropathy?
ACEI and HIV medications to reduce proteinuria.
What is the most common glomerular lesion in Hepatitis B?
Membranous glomerulonephritis (MGN) in children and MPGN in adults.
What is the typical renal manifestation of Syphilis?
Nephrotic syndrome due to MGN with subepithelial immune deposits.
What is the characteristic renal lesion in Leprosy?
FSGS, mesangioproliferative GN, or renal amyloidosis.
What is the renal pathology caused by P. malariae malaria?
Severe mesangioproliferative GN with subendothelial and mesangial deposits.