Renal- revision Flashcards

1
Q

Alport syndrome is an X-linked dominant disease caused by mutations in genes encoding ———-?

A

a5 type IV collagen

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2
Q

Morphology
(Electron Microscopy):
* Irregular foci of thickening and thinning and splitting of GBM–> “Basket-weave” appearance

Features of?

A

Alport Syndrome

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3
Q

Alport Syndrome

EM: Irregular thickening and thinning and splitting of the GBM –> ————— appearance

A

“Basket weave”

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4
Q

CF of Alprot syndrome

A

1) Eye disorders (e.g. Lens dislocation, Posterior
Cataracts, Corneal dystrophy
)
2) Slowly progressing Proteinuria
3) Gross or microscopic Haematuria
4) Nerve Deafness

* Can’t see, Can’t pee, Can’t hear a bee

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5
Q

menomic : Can’t see, Can’t pee, Can’t hear
what CM do these stand for+ which disorder is this?

A

1) Gross or microscopic Haematuria
2) Slowly progressing Proteinuria
3) Nerve Deafness
4) Eye disorders (e.g. Lens dislocation, Posterior
Cataracts, Corneal dystrophy)

Alport Syndrome

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6
Q

causes of Rapidly progressive Glomerulonephritis

A

Goodpasture syn., post-infectious GN, SLE

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7
Q

What distinctive microscopic feature identifies Rapidly progressiVe Glomerulonephritis

A

“Crescents”

aka Rapidly progressive (Crescentic) GN

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8
Q

CM of Rapidly progressive GN

A

1) Rapid and progressive loss of renal function
2) Severe Oliguria
3) Signs of Nephritic Syndrome

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9
Q

what stain is used to demeonstrate Renal BM?

A

jones’ Methenamine Silver stain (JMS)

* Same as PAS

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10
Q

Microscopic features:
- Areas of necrosis with rupture of capillary loops
- “Wire loop” lesion of Lupus Nephritis
- Epithelial Crescent
- IF: Fibrinogen Ab

Feautres of?

A

Rapidly progressive (Crescentic) GM

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11
Q

Macroscopic Features:
* Enlarged and pale kidneys
* Petechial, cortical located haemorrhages

Microscopic Findings:
* Formation of “Crescents”, by the proliferation parietal cells and the infiltration of monocytic inflammatory cells
* IF: Strong staining of linear IgG and C3 deposits, along the GBM
* EM: Focal disruptions of the GBM
* Treatment: Plasmapheresis

Features of?

A

Anti-glomerular BM Antibody-mediated Crescentic GN

*type I class of Rapidly progressive GN

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12
Q

On IF of Anti-Glomerular BM Antibody-mediated Crescentic GN , Strong staining of linear ——- and ———– deposits, along the GBM are found

A

Strong staining of linear IgG
and C3 deposits, along the GBM

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13
Q

Immune Complex-Mediated progressive GN (Type II)

Microscopic Findings:
* Segmental necrosis and GBM breaks –> ”———–“ and
* Diffuse proliferation and leukocyte exudation (Post Infectious Glomerulonephritis, SLE)
* Mesangial proliferation (IgA Nephropathy, HenochSchönlein Purpura)
* IF: Characteristic granular (“—————”) pattern of immune complex disease

A

*Segmental necrosis and GBM breaks –> “Crescents”
*IF: (“lumpy bumpy”) pattern of immune complex disease

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14
Q

Microscopic Findings:
* Segmental necrosis and GBM breaks -> “Crescents” and
* Diffuse proliferation and leukocyte exudation
* Mesangial proliferation
* Immunofluorescence: Characteristic granular (“lumpy bumpy”)

Features of?

A

Immune Complex-Mediated progressive GN (Type II)

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15
Q

Most common type of Lupus Nephritis

A

Diffuse Lupus Nephritis (Class IV)

* (35-60% of patients) and most
serious form

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16
Q

Micro features of Diffuse Lupus Nephritis (Class ):
* Extensive —————– immune complexes –>
Circumferential thickening of the capillary wall
(“—————”)
* Involvement of >–%of glomeruli

A

(Class IV)
* Extensive sub-endothelial immune complexes -> Circumferential thickening of the capillary wall
(“wire loops”)
* Involvement of >50% of glomeruli

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17
Q

CF of Diffuse Lupus Nephritis (Class IV)

A

1) Haematuria,
2) moderate to severe Proteinuria,
3) Hypertension and
4) Renal Insufficiency

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18
Q

Microscopic Findings:
* Segmental necrosis and GBM breaks –> “Crescents”
* Lack of anti-GBM Abs or Immune Complexes (IF & EM)

features of?

A

Pauci-immune Crescentic GN

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19
Q

Lab findins of Pauci-immune GN?

A

ANCA in serum

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20
Q

———-: Disorder in which chronic tubulo- interstitial inflammation and renal scarring are associated with pathologic involvement of the pelvi-calyceal system

A

Chronic Pyelonephritis

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21
Q

Macroscopic Features:
* Irregular scarring; Coarse, discrete, cortico-medullary scars
* Dilated, blunted, or deformed calyces
* Flattened papillae

Microscopic Findings:
* Uneven interstitial fibrosis
* Mixed inflammatory cell infiltrates
* Dilatation of tubules, with atrophy of the lining
epithelium
* Presence of intraluminal colloid (PAS[+]) casts –> Resemblance to Thyroid tissue [“Thyroidization”]
* Hyaline Arteriolosclerosis (Hypertension)
* Focal Segmental Glomerulosclerosis

features of?

A

Chronic Pyleonephritis

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22
Q

* Chronic Pyelonephritis

Macroscopic Features:
* —————; Coarse, discrete, cortico-medullary scars
* Dilated, blunted, or deformed ——-
* Flattened ————–

Microscopic Findings:
* Uneven interstitial fibrosis
* Mixed inflammatory cell infiltrates
* Dilatation of tubules, with atrophy of the lining
epithelium
* Presence of ——————- –> Resemblance to Thyroid tissue [”————–”]
* Hyaline Arteriolosclerosis (Hypertension)
* Focal Segmental Glomerulosclerosis

A

Macroscopic Features:
* Irregular scarring; Coarse, discrete, cortico-medullary scars
* Dilated, blunted, or deformed calyces
* Flattened papillae

Microscopic Findings:
* Uneven interstitial fibrosis
* Mixed inflammatory cell infiltrates
* Dilatation of tubules, with atrophy of the lining
epithelium
* Presence of intraluminal colloid (PAS[+]) casts –> Resemblance to Thyroid tissue [“Thyroidization”]
* Hyaline Arteriolosclerosis (Hypertension)
* Focal Segmental Glomerulosclerosis

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23
Q

CF of Chronic Obstructive Pyelonephritis

A
  • Insidious Onset or
  • Manifest as Acute Recurrent Pyelonephritis, with:
  • Back pain
  • Fever
  • Frequent Pyuria (puss in pee)
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24
Q

causes of MALIGNANT HYPERTENSION?

A

Appearance either de novo or with a sudden onset in patients with pre-existing mild hypertension

* de novo –> ‘from the beginning’

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25
Q

Macroscopic features:
- Multiple small, pin-point petechial haemorrhages (on the kidneys)
- Flea-bitten Kidney

Microscopic features:
- Hyperplastic Arteriolosclerosis (Onion skin lesions)
- Fibrinoid Necrosis of Afferent Arteriole

A

Malignant HTN

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26
Q

Epi of Minimal-Change disease

* Type of Nephrotic Syndrome

A

Most common cause of Nephrotic
Syndrome in children (1-7 years)

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27
Q

CF of Minimal change disease

* Type of Nephrotic syndrome

A

1) Insidious (slow) onset of Nephrotic Syndrome
2) Albumin selective proteinuria

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28
Q

CF of Membranous Nephropathy

A
  • Insidious onset of Nephrotic Syndrome (most cases)
  • Non-selective proteinuria
  • Haematuria and mild hypertension (15-35% of cases)
29
Q

Progression and Prognosis of Membranous Nephropathy

A
  • Persistent proteinuria (>60% of cases)
  • Progressive disease leading to Renal Failure, after 2 to 20 years (40% of cases)
  • Partial or complete remission (disappearnace) of proteinuria (10-30% of cases)
30
Q

Microscopic Findings (Electron & Fluorescence Microscy):
* Discrete sub-endothelial electron-dense deposits (EM)
* Irregular granular deposition of C3 (IF)
* Presence of IgG and early complement components (IF)

Features of ?

A

MPGN- Type I

31
Q

**

Microscopic Findings:

  • LM:Glomeruli enlarged and Hypercellular
    (in a diffuse pattern : Leukocytic infiltration [neutrophils and monocytes, Proliferation of endothelial and mesangial cells, Crescent formation (severe cases)]
  • EM: Subepithelial IC “humps”
  • IF: Scattered granular appearance “lumpy hump” due to IgG, IgM, and C3 deposits within GBM walls and mesangium

Features of?

A

Acute Post-streptococal Glomerulonephritis

32
Q

CF of Acute Post-Infectious Streptococcal GN

A

Abrupt onset of:
* Malaise
* Slight Fever
* Nausea
* Oliguria and Haematuria (smoky or cola-coloured urine)
* HTN
* Peripheral and periorbital oedema
* Mild Proteinuria

33
Q

**

MPGN-1 vs Dense Deposit disease

A
34
Q

Microscopic features:
Light microscopy:
* “Double-contour” or “Tram-track” appearance of the glomerular capillary wall.
* “Splitting” of GBM
IF: IgG and early complement components.
EM: Discrete sub-endothelial electron-dense deposits.

features of?

A

Membrano-Proliferative GN type 1

35
Q

Cause Post-Streptococcal GN

* type of Nephritic Syndrome

A

~ 2-4 weeks after infection w/ certain “Nephritogeninc” strains of group Α,β-heam. Streptoc.
(infection of pharynx or skin)

* most common in children

36
Q

Lab findings of ACUTE POST-INFECTIOUS
(POST-STREPTOCOCCAL) GN

A
  • +ve strep titers (Elevated anti-Streptolysin O Ab-titers)
  • ↓ complement levels (C3) due to consumption (during the active phase)
37
Q

Clinical Manifestations of Nephritic vs Nephrotic Syndrome.

A

Note : there is proteinuria and oedema in Nephritic however it’s mild

38
Q

patho of MALIGNANT HYPERTENSION:
* Long-standing hypertension –> Injury to the arteriolar walls –> i. ——-,———-,———–> Fibrinoid necrosis of vessels —> (————) -> Increased narrowing of the arteriolar lumen –> Marked —— changes of the kidneys
* Renal ischaemia –> Further, Renin secretion (“———-”) -> Elevated ——— levels –> Salt retention -> Aggravation of blood pressure

A
  • Long-standing hypertension –> Injury to the arteriolar walls –> i. increased permeabilty, endothelial injury and platelet deposition–> Fibrinoid necrosis of vessels —> (Hyperplastic Arteriolosclerosis) -> Increased narrowing of the arteriolar lumen –> Marked ischaemic changes of the kidneys
  • Renal ischaemia –> Further, Renin secretion (“vicious cycle”) -> Elevated Aldosterone levels –> Salt retention -> Aggravation of blood pressure
39
Q

Light microscopy
* Normal appearance of the glomeruli
* Protein droplets and lipids within the PCT -proximal convoluted tubules

Electron microscopy (Masson’s trichrome dye)
* Flattening of podocytes’ cytoplasm -> effacement (d.t cytokine damage)
* Epithelial cell vacuolisation
* Microvillus formation
* Focal detachments

Features of?

A

Minimal change disease

40
Q

Macroscopic Features:
* Involvement of some of the glomeruli (focal); initially only the juxta-medullary glomeruli -> With disease progress, all cortical levels

Microscopic Findings:
* segmental glomerular lesions
* Increased mesangial matrix
* Obliterated capillary lumina
* Deposition of hyaline (hyalinosis)
* Foamy (lipid-laden) macrophages
* IF: Non-specific trapping of IgM and complement (C1,C3)
* EM: Effacement of podocytes’ foot processes; similar to Minimal Change Disease

Features of?

A

FOCAL SEGMENTAL GLOMERULOSCLEROSIS (FSGS)

* ass. w/ HIV

41
Q

Patho of Membranous Nephropathy:

diffuse thickening of the —– and —- caused by subepithelial immune deposits -> Activation of the Complement –> ———— –> Damage of the mesangial cells and ——- –> Loss of slit filter integrity -> ——-

A

diffuse thickening of the glomerular capillary wall and GBM caused by subepithelial immune deposits -> Activation of the Complement –> C5b-C9 Membrane Attack Complex (MAC) –> Damage of the mesangial cells and podocytes –> Loss of slit filter integrity -> Proteinuria

42
Q

**

Microscopic Findings:
Light microscopy: Uniform, diffuse thickening of the glomerular capillary wall and BM
IF: Granular deposits of IgG and complement (C5b-C9 -MAC) along the GBM

Electron microscopy:
* Characteristic “spike and dome” appearnace of sub-epithelial deposits
* Irregular dense deposits of immune complexes , deposition between the BM and the overlying epithelial cells
* Effacement of podocytes’ foot processes

A

MEMBRANOUS NEPHROPATHY

43
Q

CM of Prune Belly Syndrome

A

Classical triad:
1. Urinary Tract anomalies
2. Deficient abdominal musculature (wrinkled/Prune-like Appearnace of the abdomin wall)
3. Bilateral Cryptorchidism

other CF:
1) Club foot (Talipes Equinovarus
2) Pulmonary hypoplasia
3) Imperforate Anus
4) Arthrogryposis (joint stiffness at birth)

44
Q

CF of Autosomal Dominant (Adult) Polycystic kideny disease

A

1) Flank pain
2) Heavy dragging sensation
3) Palpation of an Abdominal Mass
4) Intermittent Gross Haematuria
5) Berry Aneurysms, with high incidence of Subarachnoid Haemorrhage (10-30% of patients)

45
Q

Macroscopic Features:
* Enlarged organs, with a smooth external surface
* Cut surface: Numerous small Cysts in the Cortex and
Medulla –> Sponge-like appearance

Microscopic Findings:
* Dilated, elongated cystic spaces, vertical to the cortical surface
* Uniform lining of cuboidal cells
* Cysts in the Liver

Features of?

A

AR (Childhood) Polycystic Kidney disease

46
Q

Macroscopic features:
* Staghorn Calculi in the renal pelvis
* marked hydronephrosis

Microscopic features:
* mix of calcium oxalate w/ calcium phosphate stones (in the calcyces)

features of?

A

Renal stones - Uroluthiasis

Staghorn calculi : large renal stones in the renal pelvis

47
Q

Macroscopic Features:
* Tan colour
* Central Stellate Scar

Microscopic Findings:
* Numerous mitochondria, within neoplastic cells –> Finely granular eosinophilic cytoplasm
* Hypocellular Hyalinised Stroma
* small, round nuceli

features of?

A

Oncocytoma

48
Q

Macroscopic Features:
* Usually, solitary and large, sphaerical masses (3-15cm)
* Cut surface: Yellow to orange to gray-white
* Dilated renal Calcyes
* Central tumour Necrosis and Haemorrhage
* Invasion of Calyces and Pelvis, as well as of the Renal Vein, Inferior Vena Cava and even the Right Heart-side

Microscopic Findings:
* Vacuolated, Clear cells (due to intra-cytoplasmic glycogen and lipid) - classic sign
* Small, round nuclei
Solid cytological picture:
* Cells with pink granular cytoplasm (as tubular epithelium)

features of?

A

Renal Clear cell carcinoma

49
Q

Epi of CHromophobe Renal Carcinomas

A

5% of all Renal Cell Carcinoma

50
Q

Macroscopic Features:
* Tan-brown colour

Microscopic findings:
* Clear, flocculent (Fine reticular) cytoplasm
* Prominent distinct, thick cell membranes
* Peri-nuclear “halos” of clear cytoplasm

features of?

A

Chromophobe Renal Carcinomas

51
Q

CF of Chromophobe Renal carcinomas

A

Classic Triad:
1) Haematuria (>50% of cases)
2) Dull Flank Pain
3) Palpable Abdominal Mass

52
Q

Macroscopic Features:
* Most common, large, solitary, well-circumscribed mass
Cut surface:
* Soft, homogeneous Tumour, with tan to gray colour
* Foci of Cystic degeneration, Haemorrhage and Necrosis

Microscopic Findings:
* Classic triphasic combination of blastemal, stromal and epithelial cell types
- Blastemal component: Sheets of small blue cells
- Stromal component: Fibrocytic or Myxoid cells
- Epithelial component: Abortive Tubules or Glomeruli

features of?

A

WILMS tumour

53
Q

Macroscopic Features:
* Very large Kidneys
* Cut surface: Mass of Cysts (up to 3-4 cm), with no
intervening parenchyma
* Content: Clear, turbid or haemorrhagic fluid

Microscopic Findings:
* Variable lining epithelia of the cystic spaces
* Occasionally, presence of glomerular tufts within the cysts

features of?

A

AD (Adult) Polycystic Kidney disease

54
Q

Adults w/ AD Polycystic Kidney disease have a high incidence of developing?

A

Subarachnoid
Haemorrhage (10-30% of patients)

55
Q

complications of AD (Adult) Polycystic kideny disease

A
  • Hypertension (75% of cases)
  • Urinary infection
56
Q

Cf of AR (Childhood) polycystic kideny disease

A
  • Death of young infants, from Hepatic or Renal Failure
  • Patients, who survive infancy -> Development of Liver
    Cirrhosis
57
Q

Macroscopic Features:
* Marked Kidney enlargement
* Prominent distention of the Pelvi-Calyceal System
* Compression and Atrophy of the Renal Parenchyma
* Obliteration of the Papillae & Flattening of the Pyramids

Microscopic Findings:
* Tubular dilation
* Atrophy and Fibrosis of the tubular lining
* Atrophy and Loss of Glomeruli
* Coagulative Necrosis of the Renal Papillae
* Superimposed Pyelonephritis

features of?

A

Hydronephrosis

58
Q

CF of Hydronephrosis in cases of Complete, Bilateral Obstruction

A

Anuria

59
Q

CF of Hydronephrosis in cases of Incomplete, Bilateral Obstruction

A

Polyuria

60
Q

Complications of Oncocytoma

A

Spontaneous Haemorrhage

61
Q

location of Renal Cell Carcinoma

A

renal Cortex

62
Q

Epi of Renal Cell Carcinoma

A

80-85% of all primary Renal Malignant Tumours
M>F

63
Q

Macroscopic Features:
* Multifocal and bilateral occurrence; Early-stage Tumours
* Cut surface: Weak orange-yellow colour; Necrosis,
Haemorrhage and Cystic degeneration

Microscopic Findings:
* Formation of Papillae with fibrovascular cores
* Foamy macrophages

features of?

A

Papillary Renal cell carcioma

64
Q

WILMS tumour is aka?

A

Nephroblastoma

65
Q

WILMS tumour causes

  • Patients with the ———- Syndrome (1/3 of cases -> Wilms Tumour); Loss of genetic material of WT1 gene
  • Patients with ———- Syndrome (90% -> Wilms Tumour); Dominant negative inactivating mutation in WT1 gene
A
  • Patients with the WAGR Syndrome (1/3 of cases -> Wilms Tumour); Loss of genetic material of WT1 gene
  • Patients with Denys-Drash Syndrome (90% -> Wilms Tumour); Dominant negative inactivating mutation in WT1 gene
66
Q

CF of WAGR Syndrome

A

1) Wilms Tumour
2) Aniridia (partial or complete absence of the iris)
3) Genito-Urinary anomalies
4) Mental Retardation

67
Q

CF of Denys-Drash Syndrome

A

1) Wilms Tumour
2) Intersex disorders (Gonadal dysgenesis)
3) Congenital Nephropathy

68
Q

CF of WILMS tumour

A
  • Commonly: Palpable abdominal mass, extending across the midline and down into the pelvis
  • Less often: Fever, Abdominal Pain, and Haematuria