Renal Vasculature and Related Conditions

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Questions and Answers

Which of the following best describes the primary function of the renal vasculature?

  • Producing red blood cells and storing them for release when needed.
  • Filtering blood to remove waste products and maintain electrolyte balance. (correct)
  • Regulating systemic blood pressure by secreting hormones into the bloodstream.
  • Synthesizing vitamin D and regulating calcium absorption in the body.

A patient presents with renovascular hypertension due to renal artery stenosis. Which physiological response is LEAST likely to contribute to the elevated blood pressure?

  • Activation of the sympathetic nervous system causing vasoconstriction.
  • Increased aldosterone secretion promoting sodium retention.
  • Increased renin secretion leading to angiotensin II formation.
  • Reduced sodium and water reabsorption in the distal tubule and collecting duct. (correct)

A researcher is studying the effects of chronic kidney disease on vascular calcification. Which of the following mechanisms is most likely to contribute to this process?

  • Enhanced production of nitric oxide (NO) promoting vasodilation.
  • Decreased phosphate excretion leading to hyperphosphatemia. (correct)
  • Increased levels of matrix Gla protein (MGP) inhibiting calcium deposition.
  • Upregulation of Klotho expression preventing vascular smooth muscle cell transformation.

A patient with a history of poorly controlled diabetes mellitus is diagnosed with nephrosclerosis. Which pathological change is most likely to be observed in the renal vasculature?

<p>Hyaline thickening of the arteriolar walls. (C)</p> Signup and view all the answers

A patient is suspected of having renal artery stenosis. Which non-invasive imaging modality is most appropriate for initial screening?

<p>Duplex Doppler ultrasound to assess renal blood flow. (D)</p> Signup and view all the answers

In cases of renal artery stenosis, what percentage of occurrences originate from the aorta?

<p>50% (A)</p> Signup and view all the answers

Which patient demographic is the most affected by renal artery stenosis?

<p>Older men (A)</p> Signup and view all the answers

Medial fibroplasia, a variant of fibromuscular dysplasia, is characterized by which of the following?

<p>Typically bilateral presentation and rarely causing complete arterial blockage. (C)</p> Signup and view all the answers

What is a typical vascular characteristic of fibromuscular dysplasia?

<p>Alternating constrictions and aneurysmal dilatations (D)</p> Signup and view all the answers

What is the likely outcome of a sudden complete occlusion of the renal artery?

<p>Renal Infarction (D)</p> Signup and view all the answers

A patient is diagnosed with long-standing unilateral renal artery stenosis. What is a likely outcome if the stenosis is corrected?

<p>Hypertension may not be relieved. (B)</p> Signup and view all the answers

What are the primary characteristics defining thrombotic microangiopathies such as Hemolytic Uremic Syndrome (HUS) and Thrombotic Thrombocytopenic Purpura (TTP)?

<p>Microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. (D)</p> Signup and view all the answers

The classic form of Hemolytic Uremic Syndrome (HUS) is typically associated with what condition and patient population?

<p>Young children with prodromal diarrhea. (D)</p> Signup and view all the answers

A patient with long-standing hypertension develops sudden onset severe headache, blurred vision, and progressive kidney failure. Blood pressure is 220/130 mmHg. Which of the following renal vascular changes is MOST likely to be seen in this patient?

<p>Fibrinoid necrosis of arterioles (C)</p> Signup and view all the answers

Reduced kidney size, granular capsular surfaces, and thin cortices are MOST characteristic of which condition?

<p>Benign hypertension (A)</p> Signup and view all the answers

Atherosclerosis affecting the main renal arteries primarily contributes to ischemic renal disease by which mechanism?

<p>Activation of the renin-angiotensin-aldosterone system (A)</p> Signup and view all the answers

Why does sudden occlusion of renal arteries usually result in infarction?

<p>The arteries of the kidneys are considered end-organ type, with no effective collateral circulation. (B)</p> Signup and view all the answers

Which of the following is the MOST common cause of secondary hypertension related to renal vascular disease?

<p>Atherosclerosis of the renal artery (D)</p> Signup and view all the answers

What is the primary function of the metalloprotease ADAMTS-13 in the context of Thrombotic Thrombocytopenic Purpura (TTP)?

<p>Breaking down large Von Willebrand factor (VWF) multimers. (D)</p> Signup and view all the answers

The activation of the renin-angiotensin-aldosterone system (RAAS) in ischemic renal disease is a direct consequence of which of the following?

<p>Reduced blood flow to the kidneys due to arterial narrowing (D)</p> Signup and view all the answers

A patient presents with neurologic symptoms such as seizures and aphasia, and is suspected of having a thrombotic microangiopathy. Severe renal damage is noted to be uncommon. Which condition is most likely?

<p>Thrombotic Thrombocytopenic Purpura (TTP). (B)</p> Signup and view all the answers

A patient with benign hypertension is likely to exhibit which vascular change in the kidneys?

<p>Hyaline arteriolosclerosis (D)</p> Signup and view all the answers

Which treatment is considered the most important in managing both Thrombotic Thrombocytopenic Purpura (TTP) and Hemolytic Uremic Syndrome (HUS)?

<p>Plasma exchange. (C)</p> Signup and view all the answers

An elderly patient with known atherosclerosis undergoes a cardiovascular procedure and, weeks later, develops increased serum BUN and creatinine. Which condition is most likely?

<p>Cholesterol Emboli. (C)</p> Signup and view all the answers

A 55-year-old male with a history of poorly controlled hypertension presents with acute kidney injury. Renal biopsy reveals 'onion-skin lesions' in the interlobular arteries. Which of the following conditions is MOST likely associated with these findings?

<p>Malignant hypertension (D)</p> Signup and view all the answers

A patient has a significant reduction in glomerular filtration rate due to hemodynamically significant renovascular occlusive disease. This condition directly leads to:

<p>Ischemic renal disease (D)</p> Signup and view all the answers

What is a common cause of renal cortical necrosis?

<p>Severe circulatory failure often related to obstetric complications. (D)</p> Signup and view all the answers

Which of the following best describes the sequence of events in renal ischemia caused by renal artery stenosis?

<p>Renal artery stenosis → activation of renin-angiotensin-aldosterone system → increased blood pressure (C)</p> Signup and view all the answers

A patient with a history of left-sided heart issues presents with sudden flank pain and is diagnosed with a renal infarct. The severity of the patient's condition most directly depends on:

<p>The size of the affected vessel and the amount of infarcted renal tissue. (D)</p> Signup and view all the answers

A patient is diagnosed with atypical Hemolytic Uremic Syndrome (HUS). Genetic testing reveals a mutation. Which protein is most likely affected by this mutation?

<p>Complement regulatory protein factor H. (D)</p> Signup and view all the answers

Which of the following conditions is LEAST likely to be directly associated with the sporadic form of non-diarrhea-associated Hemolytic Uremic Syndrome (HUS)?

<p>Infection with <em>E. coli</em> O157:H7. (B)</p> Signup and view all the answers

Flashcards

Nephrology

Branch of medicine focused on the kidneys.

Renal Vascular Disease

Illnesses affecting the kidney's blood vessels.

Kidney Function

The kidneys' main job: filtering waste and extra fluid from the blood.

Renovascular Hypertension

High blood pressure specifically in the kidneys.

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Renal Artery Stenosis

Narrowing of the arteries carrying blood to the kidneys.

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Ischemic Renal Disease

Reduced glomerular filtration rate due to renovascular occlusive disease.

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Large Renal Artery Diseases

Atherosclerosis, thrombosis, and thromboembolism.

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Small Renal Artery Diseases

Hypertension and thromboembolism.

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Renal Arteries

No effective collateral circulation.

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Renal Ischemia & Hypertension

Stenosis activates renin-angiotensin-aldosterone system, increasing blood pressure.

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Essential Hypertension

Divided into mild to moderate (benign) and severe (malignant) forms.

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Benign Hypertension Risks

Major risk factor for cerebrovascular accidents and coronary artery disease.

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Malignant Hypertension

Defined by clinical conditions like papilledema, heart failure, stroke, encephalopathy and renal insufficiency.

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Kidneys in Benign Hypertension

Kidneys are normal or moderately reduced in size with granular surface.

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Kidneys in Malignant Hypertension

Surface petechial hemorrhages occur.

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Less Common Causes of RAS

Thromboembolic disease, main renal artery thrombosis, vasculitis, and fibromuscular dysplasia.

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Fibromuscular Dysplasia

A condition mainly in young women causing artery narrowing and aneurysms.

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Consequences of Renal Artery Occlusion

Infarction or ischemic nephropathy, depending on vessel size, closure rate and collateral circulation.

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Ischemic Nephropathy

Kidney tissue damage due to insufficient blood supply from gradual artery narrowing.

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Treatment for Renal Artery Stenosis

Angioplasty or bypass graft, if detected early.

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Thrombotic Microangiopathies

Syndromes with microangiopathic hemolytic anemia, thrombocytopenia, and renal failure, linked to endothelial injury.

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Hemolytic Uremic Syndrome

A type of thrombotic microangiopathy mainly in young children, often after diarrhea from verotoxin-producing E. coli.

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Hemolytic Uremic Syndrome (HUS)

A complication often linked to E. coli strains like O157:H7 and O104:H4.

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Thrombotic Thrombocytopenic Purpura (TTP)

Most commonly occurs in women, characterized by neurological symptoms like seizures. Renal damage is uncommon.

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TTP Cause

Caused by abnormalities in ADAMTS-13, leading to excess Von Willebrand factor multimers and spontaneous platelet activation.

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TMA Treatment & Outcome

Plasma exchange is vital for both TTP and HUS. HUS often resolves spontaneously; TTP has ~80% survival with treatment.

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Cholesterol Emboli Risk Factors

Kidneys are commonly affected due to emboli from aortic atherosclerosis, often after procedures like angiography.

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Cholesterol Emboli Effects

Emboli in small renal arteries/arterioles, leading to increased creatinine and potentially severe renal failure.

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Renal Cortical Necrosis Causes

Rare renal failure due to severe circulatory failure, often linked to obstetric issues like placental abruption.

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Renal Cortical Necrosis Outcomes

Renal failure, resembling renal infarction histologically; survivors often have residual renal insufficiency.

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Study Notes

Vascular Diseases of the Kidney

  • Ischemic renal disease shows a significant reduction in the glomerular filtration rate.
  • This is due to significant renovascular occlusive disease affecting the entire functioning renal parenchyma.
  • Larger arteries may experience diseases like atherosclerosis, thrombosis, and thromboembolism.
  • Hypertension and thromboembolism affect smaller arteries and arterioles more.
  • Renal arteries are considered end-organ type, lacking effective collateral circulation.
  • Sudden arterial occlusion often results in infarction.
  • Renal ischemia from main renal artery stenosis or intrarenal artery narrowing activates the renin-angiotensin-aldosterone system.
  • This activation leads to an increase in blood pressure.

Essential Hypertension

  • Essential hypertension is the most common cause of systemic hypertension
  • It is divided into mild to moderate (benign) and severe (malignant) forms

Essential Hypertension Risks

  • Benign form increases the risk of cerebrovascular accidents, coronary artery disease, myocardial infarction, and renal disease.
  • Malignant form typically presents with pressure exceeding 190/120.
  • It's defined by associated clinical conditions such as papilledema, congestive heart failure, stroke, encephalopathy, and renal insufficiency.

Benign Hypertension Indicators

  • Kidneys are normal or moderately reduced in size and weight;
  • Kidneys develop a granular capsule, the cortex thins;
  • Ischemic nephropathy changes may be present.
  • Arteries have intimal fibroplasia and reduplication of the internal elastic. lamina.
  • Homogeneous eosinophilic material replaces the media of arterioles, characterizing hyaline arteriolosclerosis.

Malignant Hypertension Indicators

  • Cortical surface petechial hemorrhages are typically present.
  • Ischemic nephropathy changes are possible
  • Expect arterial intimal fibroplasia
  • Look for fibrinoid necrosis of arterioles with or without necrotizing arteriolitis.
  • Interlobular and larger arterioles may express hyperplastic arteriolitis (onion-skin lesion).

Renal Artery Stenosis Facts

  • Renal Artery Stenosis is a significant cause of secondary hypertension.
  • Atherosclerosis is the most common cause of large renal artery occlusion, predominately in older men affecting mostly the proximal renal arteries.
  • Bilateral disease is present in up to 60% of renal artery stenosis cases.
  • In 50% of cases, renal Artery Stenosis originates from the aorta.
  • Less common causes of occlusion: thromboembolic disease, thrombosis of main renal arteries, vasculitis, and fibromuscular dysplasia.

Fibromuscular Dysplasia

  • Fibromuscular Dysplasia occurs more commonly in young women.
  • It causes usually concentric narrowing of the artery.
  • Alternating constrictions and aneurysmal dilatations may be present.
  • Medial fibroplasia is the most common variant.
  • It is most often bilateral and seldom causes total occlusion.

Clinical Course

  • Consequences of occlusion depends on size and of vessels involved
  • Rate of closure,
  • The presence or absence of collateral circulation and/or occlusion being unilateral or bilateral.
  • Gradual narrowing results in ischemic nephropathy.
  • Ischemic disease tends to progress fairly rapidly, with approximately half of patients showing progression within two years.
  • Sudden occlusion commonly results in infarction

Renal Artery Stenosis Treatment

  • Early detection may allow reversal with angioplasty or bypass graft.
  • In long-standing unilateral stenosis, correction may not relieve hypertension.

Thrombotic Microangiopathies

  • Hemolytic uremic syndrome (HUS) and Thrombotic thrombocytopenic purpura (TTP) are closely related.
  • Both share virtually identical renal pathology, making them difficult to distinguish.
  • Characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal failure.
  • Disease is related to endothelial Injury and subsequent activation of the coagulation system.

Hemolytic Uremic Syndrome Forms

  • Classic form occurs mainly in young children and is associated with prodromal diarrhea.
  • Classic form cases associate with infection from verotoxin-producing strains of E. coli (especially 0157:H7, recently 0104.H4).
  • Non-diarrhea-associated disease classifies as sporadic or familial.
  • Pregnancy, SLE, HIV, and drugs can be associated with the sporadic form.
  • The familial form is linked to mutations in complement regulatory protein factor H.

Thrombotic Thrombocytopenic Purpura

  • Disease occurs most commonly in women during the fourth and fifth decades.
  • Neurologic symptoms (seizures, aphasia, altered consciousness) present in most patients.
  • Severe renal damage is uncommon.
  • Disease has been linked to abnormalities of metalloprotease ADAMTS-13, which breaks down large Von Willebrand factor multimers.
  • ADAMTS-13 absence results in excess VWF multimers, which are prothrombotic and promote platelet activation.

Thrombotic Microangiopathies Course

  • Plasma exchange is the single most important treatment in TTP and HUS.
  • Renal damage resolves spontaneously in over 90% of classic HUS cases.
  • The survival rate for patients with TTP is up to 80%.

Cholesterol Emboli

  • Kidneys are the most common organs involved by cholesterol embolization.
  • Occur usually occur in older arteriosclerosis patients of the thoracic or upper abdominal aorta.
  • Onset occurs one to several weeks after angiography, cardiovascular surgery, or another precipitating event.
  • Emboli impacts smaller arteries and arterioles.
  • Renal manifestations include increased serum BUN and creatinine.
  • Renal involvement's severity is variable; if progression occurs, it can result in severe renal failure that needs dialysis.

Renal Cortical Necrosis

  • Uncommon cause of renal failure occurring in severe circulatory failure.
  • The condition is frequently bilateral.
  • Is most common from obstetric complications such as placental abruption or eclampsia.
  • Acute renal failure is the common clinical finding.
  • The histologic appearance resembles those of renal infarction.
  • A significant number of patients survive with hemodialysis but are left with considerable residual renal insufficiency.

Renal Infarction Causes

  • Typically related to embolism, usually from the left side of the heart.
  • Many renal infarcts are clinically silent.
  • The severity of disease depends on vessel size and the amount of infarcted renal tissue.

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