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Pathology -- Nephrology > Congenital and Vascular Diseases > Flashcards

Congenital and Vascular Diseases Flashcards

1
Q

Important congenital anomalis to know…

A

Renal agenesis
Renal hypoplasia
Ectopic Kidneys
Horseshoe Kidney

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2
Q

What happens in renal agenesis?

A 
Bilateral Agenesis (Potter's syndrome) incompatible with life
Unilateral is uncommon. Contralateral side will hypertrophy with progressive sclerosis.
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3
Q

What happens in renal hypoplasia?

A

True is very rare, usually due to scarring

Bilat. can cause renal failure in childhood

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4
Q

What happens with ectopic kidneys?

A

Kidneys develop at lower levels of the urinary tract, usually right above or within the pelvis. Can predispose to obstruction/infection

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5
Q

What happens with horseshoe kidney?

A

Fusion of upper (10%) or lower (90%) poles of the kidneys, typically anterior to great vessels.
Pretty common 1:500

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6
Q

Why are cystic diseases of the kidney important?

A

Common, can be diagnostically confused with tumors, can cause chronic renal failure

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7
Q

Pathogenic features of cystic diseases?

A

Compression of the parenchyma –> chronic dynfunction

Associated with dynfxn of other organs (esp. liver)

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8
Q

Describe Cystic Renal dysplasia

A
  1. Persistence of kidney abnormal structures such as cartilage/undifferentiated mesenchyme
  2. Associated with ureteropelvic obstruction, ureteral agenesis
  3. Large, irregular, cystic kidneys. Lined with flattened epithelium.
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9
Q

Important details about autosomal dominant adult polycystic kidney disease

A

Hereditary disorder of cell-cell matrix
Presents in 40s, 50s
Cysts expand and destroy parenchyma –> renal failure

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10
Q

Mutations associated with dominant PKD

A

PKD1 and PKD2

Codes for polycystin, which localizes in cilia for tubular epithelium

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11
Q

How does dominant PKD present?

A

Abdominal mass, Proteinuria, polyuria, hypertension
Slow progression to azotemia
Asymptomatic cysts in liver, intracranial berry aneurysms w/ hemorrhage, MVP

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12
Q

What is autosomal recessive PKD?

A

Pediatric Renal Failure
Kidneys enlarged, but smooth exterior
Cylindrical dilation of all tubules
Cysts come from collecting tubules, cuboidal lines

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13
Q

What important non-kidney effects are associated with autosomal recessive PKD?

A

Patients who survive infancy develop hepatic fibrosis

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14
Q

What is medullary cystic disease?

A

Medullary sponge kidney. Multiple cystic lesion of the CD in the medulla. Leads to calcification, infection, calculi.
Childhood cysts associated with tubular atrophy + interstitial fibrosis.

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15
Q

Important association to make with Cystic disease secondary to dialysis?

A

Renal Cell Carcinoma

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16
Q

Cystic disease secondary to dialysis cysts typically contain

A

Clear fluid

Calcium oxalate crystals

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17
Q

What to know about simple cysts?

A

Common postmortem finding, not clinically significant

Differentiate from tumors

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18
Q

What is nephrosclerosis

A

A term which applies to sclerotic changes to small arteries/arterioles seondary to HTN and diabetes. Overall, leads to ischemia of the parenchyma

19
Q

Pathogenesis of benign nephrosclerosis?

A

Medial and Intimal Thickening
Hyaline deposition of proteins which extravasate through injured endothelium
Thickening of the BM

20
Q

Notabe morphology in benign nephrosclerosis

A

Gross – Fine granularity, Narrowed Cortex
Microscopic – Thickening/Hyalinization of vessel wall
Patchy ischemic atrophy from tubular ischemia/fibrosis and glomerular deposits

21
Q

Malignant nephrosclerosis is a frequent cause of death in….

A

Scleroderma patients via uremia

22
Q

Pathogenesis of Malignant nephrosclerosis

A

Vascular damage in kidneys, fibrinoid necrosis, hyperplasia of smooth muscle, narrowed lumen.
Activation of RAA –> pos. feedback

23
Q

Morphology of Malignant nephrosclerosis

A

Gross - pinpoint petechial hemorrhages

Micro – Fibrinoid necrosis, hyperplstic arteriolitis (onion skin), necrotizing glomeruitis (fills with PMN)

24
Q

Non-renal Clinical features of Malignant nephrosclerosis?

A

Diastolic over 130
Papilledema, retinopathy (scomatoma), encephalopathy, CV abnormalities
Hypertensive crisis

25
Q

Renal Clinical features of Malignant nephrosclerosis?

A

Proteinuria, hematuria, Later –> Renal Failure

26
Q

What care about Malignant nephrosclerosis??

A

Mortality (90% mortality untreated in 1 year)

27
Q

General features of Renal artery stenosis?

A

Rare cause of HTN, Curable with surgery

28
Q

Pathogenesis of Renal artery stenosis?

A

Constriction of artery promotes RAA via JG
Vasoconstriction of kidney
Sodium retention, endothelin, loss of NO

29
Q

Origins of Renal artery stenosis

A

70% – atheromatous plaque

Fibromuscular dysplasia of renal artery

30
Q

What happens in fibromuscular dysplasia of the renal artery

A

Fibrous or fibromuscular thickening of intima, media, or adventitia (usually medial)

31
Q

Final result of Renal artery stenosis?

A

Reduced kidney size, diffuse atrophy

Non-effected kidney ends up with arteriolosclerosis from HTN

32
Q

General features of thrombotic microangiopathies

A

Thrombosis in capillaries/arterioles systemically
Microangiopathic hemolytic anemia
Thrombocytopenia
Renal failure secondary to thrombi

33
Q

Two types of thrombotic microangiopathies with considerable overlap mentioned

A

Hemolytic Uremic Syndrome

Thrombotic Thrombocytopenic Purpura

34
Q

Pathogenesis of thrombotic microangiopathies

A

Endothelial injury and activation with subsequent intravascular thrombosis
Platelet aggregation and release of PFs
Thrombosis of vasculature, resulting in distal ischemia

35
Q

Important details for presentation of Classic hemolytic Uremic Syndrome?

A

E Colo O157:H7 Verocytotoxin
GI Upset/Bleeding. Severe oliguria, hematouria, microangiopathic hemolytic anemia.
Sometimes neuro changes, HTN

36
Q

How does Classic hemolytic Uremic Syndrome happen?

A

Verocytotoxin damages endothelial cells —> thrombosis/vasoconstriction.
Kidneys get widespread renal cortical necrosis

37
Q

Infections associated with Adult hemolytic Uremic Syndrome/Thrombotic thrombocytopenic purpura

A

Typhoid Fever, E Coli septicemia, viral infections, shigellosis

38
Q

Causes of Adult hemolytic Uremic Syndrome/Thrombotic thrombocytopenic purpura

A

Infections, Antiphospholipid Syndrome, Pregnancy complications, vascular renal disease, Chemotherapeutic drugs/Immunosuppressives

39
Q

Important details for Idiopathic HUS/Classic TPP

A

Fever, Neurologic Sx, hemolytic anemia,
Thrombi in glomerular capillaries
Women under 40

40
Q

Cause of most renal infarcts

A

Embolism

41
Q

Why is sickle cell disease associated with renal infarction?

A

Medulla is low oxygen. This will cause cells to start sickling.

42
Q

Important details for renal cortical necrosis

A

Rare cause of acute renal failure
Acute decreases in perfusion due to vasospasm, microvascular disease, or intravascular coagulation
Sudden Anuria, rapidly progressing anemia

43
Q

Causes of Renal cortical necrosis

A

Pregnancy/Neonatal Distress

Rxn to contrast media, hyperacute transplant rejection, snake bites, HUS

44
Q

Pathology of Renal cortical necrosis

A

Limited to cortex, patchy
Acute necrosis of small arterioles and capillaries
hemorrhages in glom.

Pathology -- Nephrology (12 decks)

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