RENAL PATHOLOGY Flashcards
1
Q
What is the most common congenital renal anomaly?
A
Horseshoe kidney, where the kidneys are conjoined at the lower pole.
2
Q
Where is a horseshoe kidney typically located?
A
In the lower abdomen, caught on the root of the inferior mesenteric artery.
3
Q
What is renal agenesis?
A
Absence of kidney formation, which can be unilateral or bilateral.
4
Q
What is the outcome of bilateral renal agenesis?
A
Oligohydramnios, Potter sequence (lung hypoplasia, flat face, limb defects), and incompatibility with life.
5
Q
What characterizes dysplastic kidney?
A
Non-inherited congenital malformation with cysts and abnormal tissue such as cartilage.
6
Q
How is polycystic kidney disease (PKD) inherited?
A
It can be autosomal recessive or autosomal dominant.
7
Q
What are features of autosomal recessive PKD?
A
Presents in infants with renal failure, hypertension, Potter sequence, congenital hepatic fibrosis, and hepatic cysts.
8
Q
What are features of autosomal dominant PKD?
A
Presents in young adults with hypertension, hematuria, renal failure, and associations with berry aneurysms, hepatic cysts, and mitral valve prolapse.
9
Q
What is medullary cystic kidney disease?
A
An autosomal dominant defect causing cysts in the medullary collecting ducts, fibrosis, shrunken kidneys, and renal failure.
10
Q
What are common risk factors for urinary tract infections (UTIs)?
A
Sexual intercourse, urinary stasis, and catheters.
11
Q
What are the symptoms of cystitis?
A
Dysuria, urinary frequency, urgency, and suprapubic pain without systemic signs.
12
Q
What are the common causes of cystitis?
A
E. coli (80%), Staphylococcus saprophyticus, Klebsiella pneumoniae, Proteus mirabilis, and Enterococcus faecalis.
13
Q
What does sterile pyuria suggest?
A
Urethritis caused by Chlamydia trachomatis or Neisseria gonorrhoeae.
14
Q
What are the symptoms of pyelonephritis?
A
Fever, flank pain, WBC casts, leukocytosis, and symptoms of cystitis.
15
Q
What pathogens commonly cause pyelonephritis?
A
E. coli (90%), Klebsiella species, and Enterococcus faecalis.
16
Q
What causes chronic pyelonephritis?
A
Recurrent acute pyelonephritis due to vesicoureteral reflux or obstruction.
17
Q
What is characteristic of vesicoureteral reflux in chronic pyelonephritis?
A
Cortical scarring and blunted calyces at the upper and lower poles.
18
Q
What is thyroidization of the kidney?
A
Atrophic tubules containing eosinophilic proteinaceous material resembling thyroid follicles.
19
Q
What are waxy casts
A
and when are they seen?
20
Q
What is the gold standard for diagnosing cystitis?
A
Urine culture with >100,000 colony-forming units.
21
Q
What laboratory findings indicate cystitis?
A
Cloudy urine, >10 WBCs/high-power field, positive leukocyte esterase, and nitrites on dipstick.
22
Q
What symptom is associated with Proteus mirabilis in cystitis?
A
Alkaline urine with an ammonia scent.
23
Q
How does renal agenesis affect the remaining kidney in unilateral cases?
A
Leads to hypertrophy and increased risk of renal failure later in life.
24
Q
What is the origin of the urinary system?
A
The urinary system develops from the intermediate mesoderm.
25
What are the three stages of kidney development?
Pronephros, mesonephros, and metanephros.
26
Which stage of kidney development is functional in the fetus?
The metanephros stage.
27
What is the pronephros?
The earliest stage of kidney development, which is nonfunctional and regresses by the 4th week of gestation.
28
What is the mesonephros?
A temporary kidney structure that functions during the early fetal period and contributes to the formation of the Wolffian duct.
29
What does the metanephros form?
The permanent kidney, beginning development at 5 weeks of gestation.
30
What is the ureteric bud?
An outgrowth from the mesonephric duct that induces the formation of the metanephros.
31
What structures does the ureteric bud give rise to?
The ureter, renal pelvis, calyces, and collecting ducts.
32
What is the metanephric blastema?
A mass of mesenchymal tissue that forms the glomeruli, proximal tubules, and nephron loops.
33
What is renal agenesis?
A congenital absence of one or both kidneys due to failure of the ureteric bud to develop.
34
What causes Potter sequence?
Bilateral renal agenesis leading to oligohydramnios, lung hypoplasia, and facial and limb deformities.
35
What is a horseshoe kidney?
A congenital anomaly where the kidneys are fused at the lower poles and trapped by the inferior mesenteric artery.
36
What is the significance of the cloaca in development?
The cloaca is a common cavity that divides into the rectum and urogenital sinus during development.
37
What does the urogenital sinus form?
The bladder, urethra, and part of the reproductive tract.
38
What is the origin of the trigone of the bladder?
The trigone develops from the mesonephric ducts.
39
What are congenital anomalies of the ureter?
Duplicated ureter, ectopic ureter, and ureterocele.
40
What is a duplicated ureter?
A condition where two ureters drain a single kidney due to abnormal ureteric bud development.
41
What is an ectopic ureter?
A ureter that does not insert into the bladder normally, often draining into the urethra or vagina.
42
What is a urachal remnant?
A persistent remnant of the allantois that can form a urachal cyst, sinus, or fistula.
43
What is exstrophy of the bladder?
A congenital defect where the bladder is exposed on the surface of the body due to failure of the anterior abdominal wall to close.
44
What is the role of intermediate mesoderm in kidney development?
It forms the nephric structures, gonads, and parts of the adrenal glands.
45
What is the time frame for nephron formation?
Nephron formation begins at 9 weeks of gestation and is completed by 36 weeks.
46
What is the clinical significance of congenital renal anomalies?
They can lead to chronic kidney disease, hypertension, and urinary tract infections.
47
What are the main types of inflammatory renal diseases?
Glomerulonephritis, pyelonephritis, and tubulointerstitial nephritis.
48
What is glomerulonephritis?
Inflammation of the glomeruli, often due to immune complex deposition.
49
What are the clinical presentations of glomerulonephritis?
Hematuria, proteinuria, edema, and hypertension.
50
What is nephrotic syndrome?
A condition characterized by heavy proteinuria, hypoalbuminemia, hyperlipidemia, and edema.
51
What is nephritic syndrome?
A condition marked by hematuria, hypertension, and mild proteinuria.
52
What causes post-streptococcal glomerulonephritis (PSGN)?
Immune complex deposition following a streptococcal infection.
53
What is the characteristic histology of PSGN?
Subepithelial humps on electron microscopy and hypercellular glomeruli on light microscopy.
54
What is IgA nephropathy (Berger disease)?
IgA deposition in the mesangium causing hematuria and glomerular inflammation.
55
What is the main feature of rapidly progressive glomerulonephritis (RPGN)?
Crescents in Bowman's space composed of fibrin and macrophages.
56
What are the three types of RPGN?
Type I: Anti-GBM disease, Type II: Immune complex-mediated, Type III: Pauci-immune (ANCA-associated).
57
What is Goodpasture syndrome?
An autoimmune disorder with anti-GBM antibodies causing glomerulonephritis and lung hemorrhage.
58
What is pyelonephritis?
Inflammation of the renal pelvis and parenchyma, typically due to bacterial infection.
59
What are the clinical features of acute pyelonephritis?
Flank pain, fever, dysuria, WBC casts, and leukocytosis.
60
What are risk factors for pyelonephritis?
Vesicoureteral reflux, urinary obstruction, and catheter use.
61
What is chronic pyelonephritis?
Chronic inflammation leading to interstitial fibrosis and tubular atrophy.
62
What are the hallmarks of chronic pyelonephritis?
Cortical scarring, blunted calyces, and thyroidization of the kidney.
63
What is tubulointerstitial nephritis?
Inflammation of the renal interstitium and tubules, often due to drugs or infections.
64
What drugs commonly cause tubulointerstitial nephritis?
NSAIDs, antibiotics (e.g., penicillin), and diuretics.
65
What are the clinical features of tubulointerstitial nephritis?
Fever, rash, eosinophilia, and hematuria after drug exposure.
66
What is the histological hallmark of tubulointerstitial nephritis?
Interstitial inflammation with eosinophils.
67
What is granulomatous interstitial nephritis?
A rare form of tubulointerstitial nephritis associated with sarcoidosis or tuberculosis.
68
What complications can arise from inflammatory renal diseases?
Chronic kidney disease, hypertension, and recurrent infections.
69
What are the diagnostic tools for inflammatory renal diseases?
Urinalysis, serum markers (e.g., creatinine), imaging, and renal biopsy.
70
What is the treatment for post-streptococcal glomerulonephritis?
Supportive care, including fluid management and antihypertensives.
71
How is pyelonephritis treated?
Antibiotics targeting common pathogens like E. coli.
72
What is glomerulonephritis?
Inflammation of the glomeruli caused by immune or non-immune mechanisms.
73
What are the clinical syndromes associated with glomerulonephritis?
Nephritic syndrome, nephrotic syndrome, and rapidly progressive glomerulonephritis (RPGN).
74
What is nephritic syndrome?
A condition characterized by hematuria, hypertension, proteinuria, and oliguria.
75
What is nephrotic syndrome?
A condition marked by proteinuria >3.5 g/day, hypoalbuminemia, hyperlipidemia, and edema.
76
What causes post-streptococcal glomerulonephritis (PSGN)?
Immune complex deposition following Group A streptococcal infection.
77
What are the histological features of PSGN?
Subepithelial humps on electron microscopy and hypercellular glomeruli on light microscopy.
78
What is IgA nephropathy (Berger disease)?
IgA deposition in the mesangium leading to episodic hematuria.
79
What systemic condition is associated with IgA nephropathy?
Henoch-Schönlein purpura, a systemic vasculitis.
80
What is the hallmark feature of rapidly progressive glomerulonephritis (RPGN)?
Crescent formation in Bowman's space.
81
What are the three types of RPGN?
Type I: Anti-GBM disease, Type II: Immune complex-mediated, Type III: Pauci-immune (ANCA-associated).
82
What is Goodpasture syndrome?
An autoimmune disorder with anti-GBM antibodies affecting kidneys and lungs.
83
What is membranous nephropathy?
A nephrotic syndrome caused by subepithelial immune complex deposits leading to thickened glomerular capillary walls.
84
What is minimal change disease?
A nephrotic syndrome with podocyte effacement on electron microscopy, common in children.
85
What is focal segmental glomerulosclerosis (FSGS)?
Segmental sclerosis and hyalinosis of glomeruli, often causing nephrotic syndrome.
86
What is membranoproliferative glomerulonephritis (MPGN)?
A glomerular disease with mesangial proliferation and immune complex deposition.
87
What are the types of MPGN?
Type I: Subendothelial deposits, Type II: Dense deposit disease (intramembranous deposits).
88
What is Alport syndrome?
A genetic disorder caused by mutations in type IV collagen, leading to hematuria, hearing loss, and ocular abnormalities.
89
What is thin basement membrane disease?
A hereditary condition causing isolated hematuria with thin glomerular basement membranes.
90
What is lupus nephritis?
Renal involvement in systemic lupus erythematosus (SLE), classified into six classes.
91
What is the most severe form of lupus nephritis?
Class IV: Diffuse proliferative glomerulonephritis.
92
What is ANCA-associated glomerulonephritis?
Pauci-immune glomerulonephritis associated with anti-neutrophil cytoplasmic antibodies.
93
What is the treatment for Goodpasture syndrome?
Plasmapheresis and immunosuppression.
94
What causes diabetic nephropathy?
Non-enzymatic glycosylation of glomerular basement membranes leading to proteinuria and glomerulosclerosis.
95
What are Kimmelstiel-Wilson nodules?
Eosinophilic nodular glomerulosclerosis seen in diabetic nephropathy.
96
What are the complications of nephrotic syndrome?
Hypercoagulability, infections, and chronic kidney disease.
97
What diagnostic tools are used for glomerulonephritis?
Urinalysis, renal biopsy, and serological tests (e.g., ANCA, anti-GBM, complement levels).
98
What is the hallmark finding in minimal change disease under light microscopy?
Normal glomeruli despite heavy proteinuria.
99
What are the primary glomerular diseases?
Diseases affecting the glomeruli directly, such as minimal change disease, FSGS, and membranous nephropathy.
100
What are secondary glomerular diseases?
Diseases where glomerular damage occurs due to systemic conditions like diabetes, lupus, or infections.
101
What triggers IgA nephropathy episodes?
Infections like respiratory or gastrointestinal infections.
102
What type of hypersensitivity reaction is post-streptococcal glomerulonephritis?
Type III hypersensitivity reaction due to immune complex deposition.
103
What is the typical presentation of lupus nephritis?
Proteinuria, hematuria, hypertension, and nephritic or nephrotic syndrome.
104
What are the classes of lupus nephritis?
Class I: Minimal mesangial, Class II: Mesangial proliferative, Class III: Focal proliferative, Class IV: Diffuse proliferative, Class V: Membranous, Class VI: Advanced sclerosing.
105
What type of deposits are seen in membranous nephropathy?
Subepithelial immune complex deposits.
106
What is the most common cause of nephrotic syndrome in adults?
Focal segmental glomerulosclerosis (FSGS).
107
What conditions are associated with focal segmental glomerulosclerosis?
HIV, heroin use, and sickle cell disease.
108
What is the characteristic histological finding in membranoproliferative glomerulonephritis?
Tram-track appearance due to mesangial interposition.
109
What antibodies are associated with Type III RPGN?
Anti-neutrophil cytoplasmic antibodies (ANCAs), including c-ANCA and p-ANCA.
110
What is the treatment for rapidly progressive glomerulonephritis (RPGN)?
Immunosuppression with corticosteroids and cyclophosphamide.
111
What genetic mutation causes Alport syndrome?
Mutations in the COL4A3, COL4A4, or COL4A5 genes affecting type IV collagen.
112
What are the clinical features of Alport syndrome?
Hematuria, sensorineural hearing loss, and ocular abnormalities like lenticonus.
113
What is the key finding in diabetic nephropathy on light microscopy?
Mesangial expansion and nodular glomerulosclerosis (Kimmelstiel-Wilson nodules).
114
What is crescent formation in glomerulonephritis?
Accumulation of fibrin and macrophages in Bowman's space, seen in RPGN.
115
What are common triggers for membranous nephropathy?
Hepatitis B or C, autoimmune diseases, and drugs like NSAIDs and gold.
116
What is dense deposit disease (Type II MPGN)?
A condition with dense intramembranous deposits caused by complement dysregulation.
117
What laboratory finding is common in ANCA-associated vasculitis?
Elevated c-ANCA or p-ANCA levels.
118
What is the treatment for IgA nephropathy?
ACE inhibitors, steroids, and fish oil for proteinuria and inflammation.
119
What infections are associated with membranoproliferative glomerulonephritis?
Hepatitis B and C infections.
120
What is fibrillary glomerulonephritis?
A rare glomerular disease with fibrillary deposits that stain negative for Congo red.
121
What is cryoglobulinemic glomerulonephritis?
A glomerular disease caused by cryoglobulin deposition, often associated with hepatitis C.
122
What are the complications of RPGN if untreated?
End-stage renal disease and dialysis dependency.
123
What is the significance of low complement levels in glomerulonephritis?
Seen in immune complex-mediated diseases like PSGN, lupus nephritis, and cryoglobulinemia.
124
What is the primary diagnostic test for glomerular diseases?
Renal biopsy for histology, immunofluorescence, and electron microscopy.
125
What percentage of people are born with significant urinary system malformations?
About 10%.
126
What accounts for 20% of chronic kidney disease in children?
Renal dysplasias and hypoplasias.
127
What is bilateral renal agenesis associated with?
Stillbirth, hypoplastic lungs, and limb defects; incompatible with life.
128
What happens in unilateral renal agenesis?
The solitary kidney undergoes compensatory hypertrophy and can sustain life.
129
What is renal hypoplasia?
A kidney with fewer lobes and pyramids, often causing early renal failure if bilateral.
130
What is multicystic dysplasia?
A developmental anomaly with cysts, ducts, and abnormal mesenchyme in the kidney.
131
Where are ectopic kidneys located?
Above the pelvic brim or within the pelvis.
132
What is a horseshoe kidney?
A congenital anomaly where the kidneys are fused, usually at the lower poles.
133
What causes cystic kidney diseases?
Defects in the cilia-centrosome complex of tubular epithelial cells.
134
What are the features of simple renal cysts?
1-5 cm in diameter, clear fluid, confined to the renal cortex.
135
What is acquired cystic kidney disease?
Multiple cysts in end-stage renal disease patients on long-term dialysis.
136
What increases in acquired cystic kidney disease?
Risk of renal neoplasms, over 100 times higher than the general population.
137
What is autosomal dominant polycystic kidney disease (ADPKD)?
A genetic disorder with multiple cysts in both kidneys, often causing renal failure.
138
What gene is mutated in 85-90% of ADPKD cases?
PKD1 on chromosome 16.
139
What does polycystin-1 do?
Functions in the primary cilium of tubular cells, related to mechanosensing and calcium influx.
140
What is the difference between PKD1 and PKD2 mutations in ADPKD?
PKD1 mutations lead to faster progression, while PKD2 mutations are slower.
141
What is the morphology of ADPKD kidneys?
Enlarged kidneys filled with fluid-filled cysts, replacing normal parenchyma.
142
What is autosomal recessive polycystic kidney disease (ARPKD)?
A childhood disorder with small cysts and PKHD1 gene mutations.
143
What protein is affected in ARPKD?
Fibrocystin, found in cilia of tubular epithelial cells.
144
What are common complications of ARPKD?
Liver fibrosis and early renal failure.
145
What is minimal-change disease?
A nephrotic syndrome with podocyte effacement and excellent steroid response.
146
What is focal segmental glomerulosclerosis (FSGS)?
Sclerosis of some glomeruli, often causing nephrotic syndrome.
147
What is the cause of acute postinfectious glomerulonephritis?
Immune complexes after infections like streptococcus, staphylococcus, or viral illnesses.
148
What is the hallmark of acute pyelonephritis?
Liquefactive necrosis and abscess formation in the renal parenchyma.
149
What predisposes to papillary necrosis in pyelonephritis?
Diabetes, urinary tract obstruction, and sickle cell anemia.
150
What are the two forms of chronic pyelonephritis?
Chronic obstructive pyelonephritis and reflux nephropathy.
151
What is vesicoureteral reflux?
Abnormal urine backflow from the bladder into the ureters and kidneys.
152
What is the pathogenesis of ascending UTIs?
Bacteria colonize the urethra, bladder, and ascend via the ureters to infect the kidney.
153
What is the morphology of chronic pyelonephritis?
Scarring, calyceal deformity, and interstitial fibrosis with tubular atrophy.
154
What characterizes nephrotic syndrome?
Proteinuria (>3.5 g/day), hypoalbuminemia, edema, hyperlipidemia, and hypercoagulability.
155
What causes the hypercoagulable state in nephrotic syndrome?
Loss of antithrombin III in the urine.
156
What is minimal change disease (MCD)?
The most common cause of nephrotic syndrome in children, characterized by effacement of podocyte foot processes.
157
What is the typical response of MCD to steroids?
Excellent response due to T-cell-mediated cytokine damage.
158
What is the most common cause of nephrotic syndrome in adults?
Membranous nephropathy.
159
What are the histological features of membranous nephropathy?
Thickened glomerular basement membrane and subepithelial immune complex deposits ('spike and dome' appearance).
160
What is focal segmental glomerulosclerosis (FSGS)?
Segmental sclerosis of some glomeruli, often associated with HIV and sickle cell disease.
161
What is the hallmark of diabetic nephropathy?
Kimmelstiel-Wilson nodules in the mesangium.
162
What slows the progression of diabetic nephropathy?
ACE inhibitors, which reduce glomerular hyperfiltration.
163
What is the characteristic histology in systemic amyloidosis?
Amyloid deposits in the mesangium showing apple-green birefringence with Congo red staining.
164
What are key features of nephritic syndrome?
Hematuria, oliguria, azotemia, hypertension, and RBC casts.
165
What is poststreptococcal glomerulonephritis (PSGN)?
A nephritic syndrome following a group A streptococcal infection, marked by subepithelial humps on EM.
166
What is the key histology of rapidly progressive glomerulonephritis (RPGN)?
Crescents in Bowman's space made of fibrin and macrophages.
167
What distinguishes Goodpasture syndrome in RPGN?
Linear IF pattern due to anti-GBM antibodies affecting kidneys and lungs.
168
What causes IgA nephropathy (Berger disease)?
IgA immune complex deposition in the mesangium, often after mucosal infections.
169
What is Alport syndrome?
An inherited defect in type IV collagen causing hematuria, hearing loss, and ocular disturbances.
170
What are the subtypes of membranoproliferative glomerulonephritis (MPGN)?
Type I (subendothelial deposits) and Type II (dense deposit disease).
171
What is the role of C3 nephritic factor in MPGN?
Stabilizes C3 convertase, leading to complement overactivation and low C3 levels.
172
What is the primary cause of nephritic syndrome in SLE?
Diffuse proliferative glomerulonephritis (subendothelial immune complex deposits).
173
What infections can cause postinfectious glomerulonephritis?
Group A streptococcus, pneumococcus, staphylococcus, and certain viral infections.
174
What are renal papillary adenomas?
Small benign adenomas (<0.5 cm) arising from renal tubular epithelium, commonly found in the renal cortex.
175
What is the clinical importance of angiomyolipomas?
They may spontaneously hemorrhage and are associated with tuberous sclerosis.
176
What is a distinguishing feature of oncocytomas?
A central stellate scar seen on imaging.
177
What is the most significant risk factor for renal cell carcinoma?
Tobacco use.
178
What are the three major types of renal cell carcinoma?
Clear cell carcinoma, papillary carcinoma, and chromophobe carcinoma.
179
What is the genetic mutation commonly found in clear cell carcinoma?
Loss of VHL gene sequences on chromosome 3.
180
What are the cytogenetic features of papillary carcinoma?
Trisomies 7 and 17 and loss of Y in male sporadic cases.
181
What is the prognosis of chromophobe carcinoma compared to other renal cancers?
It has an excellent prognosis compared to clear cell and papillary carcinoma.
182
What is Xp11 translocation carcinoma?
A subtype of renal cell carcinoma defined by TFE3 gene translocations, often occurring in young patients.
183
What is the origin of collecting duct carcinoma?
Arises from collecting duct cells in the renal medulla.
184
What is a common morphological feature of clear cell carcinoma?
Yellow appearance due to lipid accumulations in tumor cells.
185
What characteristic behavior is associated with renal cell carcinoma?
Tendency to invade the renal vein and metastasize widely before causing local symptoms.
186
What is the most common solid renal cancer in children?
Wilms tumor.
187
What are urothelial carcinomas of the renal pelvis?
Tumors arising from the urothelium, ranging from benign papillomas to invasive carcinomas.
188
What syndromes are associated with hereditary renal cell carcinoma?
Von Hippel-Lindau (VHL) syndrome and hereditary papillary carcinoma.
189
What is the primary mechanism of immune-mediated glomerulonephritis?
Immune complex deposition leading to complement activation and neutrophil recruitment.
190
What distinguishes nephritic syndrome from nephrotic syndrome?
Nephritic syndrome features inflammation and hematuria, while nephrotic syndrome involves severe proteinuria and edema.
191
What is the main pathological feature of diffuse proliferative glomerulonephritis?
Diffuse hypercellularity and subendothelial immune complex deposits.
192
What systemic condition is associated with membranous nephropathy?
Systemic lupus erythematosus (SLE).
193
What triggers IgA nephropathy episodes?
Mucosal infections, such as respiratory or gastrointestinal infections.
194
What is the characteristic finding in Alport syndrome on electron microscopy?
Basket-weave appearance of the glomerular basement membrane.
195
How is post-streptococcal glomerulonephritis confirmed?
Presence of anti-streptolysin O (ASO) titers or anti-DNAse B antibodies.
196
What is the most common cause of end-stage renal disease in diabetic patients?
Diabetic nephropathy.
197
What role do ANCA antibodies play in glomerulonephritis?
They are associated with pauci-immune glomerulonephritis and vasculitis.
198
What distinguishes Type I from Type II membranoproliferative glomerulonephritis?
Type I involves subendothelial deposits, while Type II (dense deposit disease) involves intramembranous deposits.
199
What causes the 'tram-track' appearance in membranoproliferative glomerulonephritis?
Mesangial cell proliferation and splitting of the glomerular basement membrane.
200
What is the significance of 'humps' in post-streptococcal glomerulonephritis?
They represent subepithelial immune complex deposits.
201
What are the major complications of nephrotic syndrome?
Hypercoagulability, infections, and progression to chronic kidney disease.
202
What laboratory findings indicate RPGN?
Crescents on biopsy, hematuria, proteinuria, and rapidly declining renal function.
203
What distinguishes Churg-Strauss syndrome from other ANCA-associated vasculitides?
The presence of eosinophilia and asthma.
204
What is the first-line treatment for crescentic glomerulonephritis?
High-dose corticosteroids and immunosuppressants like cyclophosphamide.
205
What distinguishes primary from secondary glomerulonephritis?
Primary affects the glomeruli directly, while secondary results from systemic conditions like lupus or diabetes.
206
What is the characteristic urine finding in nephritic syndrome?
RBC casts and dysmorphic red blood cells.
207
What is the pathophysiological mechanism in Goodpasture syndrome?
Anti-GBM antibodies targeting type IV collagen in glomerular and alveolar basement membranes.
208
What is the role of complement levels in glomerulonephritis diagnosis?
Low complement levels are seen in immune complex-mediated diseases like PSGN and lupus nephritis.
209
What is glomerulonephritis?
Inflammation of the glomeruli, often due to immune-mediated damage.
210
What are the two broad categories of glomerulonephritis?
Nephritic syndrome and nephrotic syndrome.
211
What are the clinical features of nephritic syndrome?
Hematuria, hypertension, oliguria, and proteinuria (<3.5 g/day).
212
What are the clinical features of nephrotic syndrome?
Proteinuria (>3.5 g/day), hypoalbuminemia, edema, and hyperlipidemia.
213
What is post-streptococcal glomerulonephritis (PSGN)?
A nephritic syndrome caused by immune complex deposition after Group A Streptococcal infection.
214
What is the characteristic finding in PSGN on electron microscopy?
Subepithelial humps of immune complexes.
215
What is rapidly progressive glomerulonephritis (RPGN)?
A severe glomerulonephritis with crescent formation in Bowman's space, leading to rapid renal failure.
216
What are the three immunofluorescence patterns in RPGN?
Linear (anti-GBM disease), granular (immune complex deposition), and negative (pauci-immune).
217
What is Goodpasture syndrome?
An anti-GBM disease causing hematuria and hemoptysis due to antibodies against collagen in the basement membrane.
218
What is the most common cause of nephritic syndrome in SLE?
Diffuse proliferative glomerulonephritis.
219
What is IgA nephropathy (Berger disease)?
IgA immune complex deposition in the mesangium, causing episodic hematuria.
220
What is Alport syndrome?
An inherited defect in type IV collagen causing hematuria, hearing loss, and ocular abnormalities.
221
What is membranous nephropathy?
A nephrotic syndrome with subepithelial immune complex deposits, leading to a 'spike and dome' appearance.
222
What is minimal change disease?
A nephrotic syndrome common in children, characterized by podocyte effacement on electron microscopy.
223
What is focal segmental glomerulosclerosis (FSGS)?
Segmental glomerular scarring often associated with HIV, heroin use, or sickle cell disease.
224
What is membranoproliferative glomerulonephritis (MPGN)?
A glomerular disease with a 'tram-track' appearance due to mesangial proliferation and immune deposits.
225
What are the two types of MPGN based on deposit location?
Type I (subendothelial deposits) and Type II (dense deposit disease).
226
What is the hallmark feature of diabetic nephropathy?
Kimmelstiel-Wilson nodules and hyaline arteriolosclerosis.
227
What is the treatment for nephrotic syndrome due to minimal change disease?
Corticosteroids, with excellent response in most cases.
228
What is the role of ACE inhibitors in diabetic nephropathy?
They reduce glomerular hyperfiltration and slow disease progression.
229
What is systemic amyloidosis in the context of nephrotic syndrome?
Amyloid deposits in the mesangium leading to nephrotic syndrome, showing apple-green birefringence on Congo red staining.
230
What infections are associated with membranoproliferative glomerulonephritis?
Hepatitis B and C.
231
What is pauci-immune glomerulonephritis?
A type of RPGN associated with ANCA-related vasculitis, showing no immune complex deposition on IF.
232
What are the complications of untreated RPGN?
Chronic kidney disease and end-stage renal failure.
233
What is the pathophysiology of crescent formation in RPGN?
Fibrin and macrophages accumulate in Bowman's space, causing rapid glomerular damage.
