Renal- revision Flashcards by Elyaa Saleh

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

a5 type IV collagen

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

Features of?

Alport Syndrome

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Alport Syndrome

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

“Basket weave”

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CF of Alprot syndrome

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

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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causes of Rapidly progressive Glomerulonephritis

Goodpasture syn., post-infectious GN, SLE

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What distinctive microscopic feature identifies Rapidly progressiVe Glomerulonephritis

“Crescents”

aka Rapidly progressive (Crescentic) GN

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CM of Rapidly progressive GN

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

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what stain is used to demeonstrate Renal BM?

jones’ Methenamine Silver stain (JMS)

* Same as PAS

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

Feautres of?

Rapidly progressive (Crescentic) GM

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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?

Anti-glomerular BM Antibody-mediated Crescentic GN

*type I class of Rapidly progressive GN

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

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

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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

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

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

Features of?

Immune Complex-Mediated progressive GN (Type II)

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Most common type of Lupus Nephritis

Diffuse Lupus Nephritis (Class IV)

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

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

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

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CF of Diffuse Lupus Nephritis (Class IV)

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

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

features of?

Pauci-immune Crescentic GN

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Lab findins of Pauci-immune GN?

ANCA in serum

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

Chronic Pyelonephritis

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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?

Chronic Pyleonephritis

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* 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

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

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CF of Chronic Obstructive Pyelonephritis

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

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causes of MALIGNANT HYPERTENSION?

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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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**

Malignant HTN

Epi of Minimal-Change disease | * Type of Nephrotic Syndrome

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

CF of Minimal change disease | * Type of Nephrotic syndrome

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

CF of Membranous Nephropathy

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

Progression and Prognosis of Membranous Nephropathy

* **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)

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 ?

MPGN- Type I

# ** 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?

Acute Post-streptococal Glomerulonephritis

CF of Acute Post-Infectious Streptococcal GN

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

# ** MPGN-1 vs Dense Deposit disease

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?

Membrano-Proliferative GN type 1

Cause Post-Streptococcal GN | * type of Nephritic Syndrome

~ 2-4 weeks after infection w/ certain **"Nephritogeninc"** strains of **group Α,β-heam. Streptoc.** (infection of pharynx or skin) | * most common in children

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

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

Clinical Manifestations of Nephritic vs Nephrotic Syndrome.

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

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

* 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

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?

Minimal change disease

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?

FOCAL SEGMENTAL GLOMERULOSCLEROSIS (FSGS) | * ass. w/ HIV

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 -> **-------**

**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**

# ** 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**

MEMBRANOUS NEPHROPATHY

CM of Prune Belly Syndrome

**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)

CF of Autosomal Dominant (Adult) Polycystic kideny disease

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)

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?

AR (Childhood) Polycystic Kidney disease

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?

Renal stones - Uroluthiasis | Staghorn calculi : large renal stones in the renal pelvis

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?

Oncocytoma

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?

Renal Clear cell carcinoma

Epi of CHromophobe Renal Carcinomas

5% of all Renal Cell Carcinoma

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?

Chromophobe Renal Carcinomas

CF of Chromophobe Renal carcinomas

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

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?

WILMS tumour

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?

AD (Adult) Polycystic Kidney disease

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

Subarachnoid Haemorrhage (10-30% of patients)

complications of AD (Adult) Polycystic kideny disease

* **Hypertension** (75% of cases) * Urinary infection

Cf of AR (Childhood) polycystic kideny disease

* Death of young infants, from **Hepatic or Renal Failure** * Patients, who survive infancy -> Development of **Liver Cirrhosis**

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?

Hydronephrosis

CF of Hydronephrosis in cases of **Complete**, Bilateral Obstruction

**Anuria**

CF of Hydronephrosis in cases of **Incomplete**, Bilateral Obstruction

**Polyuria**

Complications of Oncocytoma

Spontaneous Haemorrhage

location of Renal Cell Carcinoma

renal Cortex

Epi of Renal Cell Carcinoma

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

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?

Papillary Renal cell carcioma

WILMS tumour is aka?

Nephroblastoma

# 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**

* 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**

CF of **WAGR** Syndrome

1) **W**ilms Tumour 2) **A**niridia (partial or complete absence of the iris) 3) **G**enito-Urinary anomalies 4) Mental **R**etardation

CF of Denys-Drash Syndrome

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

CF of WILMS tumour

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