Medical Terminology Quiz
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Questions and Answers

What is the early symptom that indicates a progression towards nephrosclerosis?

  • Weight gain
  • Fatigue
  • Visual disturbances (correct)
  • Chest pain

    • What drastic change does hypertension cause in the small renal arteries?

  • Dilation and increased blood flow
  • Calcification and rigidity
  • Thickening and narrowing (correct)
  • Increased elasticity and length

    • Which of the following are symptoms associated with nephrosclerosis?

  • Abdominal pain, diarrhea, and hypotension
  • Nausea, vomiting, and visual disturbances (correct)
  • Headache, fever, and chills
  • Swelling, fatigue, and hypertension

    • What medical emergency requires urgent treatment in the context of nephrosclerosis?

    <p>Azotemia as a result of renal ischemia</p> Signup and view all the answers

    What percentage of patients with nephrosclerosis may survive at least 5 years?

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

    What is a likely cause of death in patients with nephrosclerosis?

    <p>Uremia</p> Signup and view all the answers

    What is the consequence of chronic ischemia in nephrosclerosis?

    <p>Damage to renal parenchyma</p> Signup and view all the answers

    Which treatment approach is necessary for the management of nephrosclerosis?

    <p>Antihypertensive agents</p> Signup and view all the answers

    What is the primary consequence of prolonged severe obstruction in the kidney?

    <p>Expansion of the renal pelvis and calyces</p> Signup and view all the answers

    Which microscopic changes occur in the kidney during prolonged obstruction?

    <p>Tubular dilation and atrophy</p> Signup and view all the answers

    What is the most likely clinical manifestation of bilateral complete kidney obstruction?

    <p>Anuria</p> Signup and view all the answers

    What is the term used to describe the compression of the kidney parenchyma due to obstruction?

    <p>Renal atrophy</p> Signup and view all the answers

    What is the specific microscopic change observed in the glomeruli during prolonged obstruction?

    <p>Glomerular atrophy</p> Signup and view all the answers

    What is the primary pathological process underlying the microscopic changes in the kidney during obstruction?

    <p>Ischemia due to reduced blood flow</p> Signup and view all the answers

    What is the relationship between the severity of obstruction and the severity of kidney damage?

    <p>More severe obstruction leads to more kidney damage</p> Signup and view all the answers

    What is the primary factor determining the frequency of anuria in patients with kidney obstruction?

    <p>The severity of the obstruction</p> Signup and view all the answers

    What is the typical course of TTP therapy?

    <p>A rapidly improving health condition with high survival rates</p> Signup and view all the answers

    What is the primary enzyme deficiency that causes TTP?

    <p>ADAMTS13</p> Signup and view all the answers

    What is the primary mechanism of TTP therapy?

    <p>Removing the pathogenic autoantibody</p> Signup and view all the answers

    What is the approximate survival rate of patients with TTP?

    <p>10%</p> Signup and view all the answers

    What is the name of the condition characterized by a deficiency of ADAMTS13?

    <p>Thrombotic Thrombocytopenic Purpura</p> Signup and view all the answers

    What is the primary organ affected in TTP?

    <p>Brain</p> Signup and view all the answers

    What is the primary symptom of TTP?

    <p>Low platelet count</p> Signup and view all the answers

    What is the secondary mechanism of TTP therapy?

    <p>Replacing the missing ADAMTS13 enzyme</p> Signup and view all the answers

    What is the primary feature observed in hydronephrosis?

    <p>Marked dilation of the pelvis and calyces</p> Signup and view all the answers

    Which of the following best describes renal cell carcinoma?

    <p>A malignant tumor of the tubular epithelium</p> Signup and view all the answers

    Renal cell carcinoma is associated with which of the following pathological processes?

    <p>Adaptive responses to hypoxia</p> Signup and view all the answers

    What is a significant characteristic of renal cell carcinoma regarding its metastasis?

    <p>It has a strong propensity to metastasize through blood vessels</p> Signup and view all the answers

    Which of the following is the most common histologic form of renal cell carcinoma?

    <p>Clear cell carcinoma</p> Signup and view all the answers

    What is the relationship between urine acidity and renal dysfunction?

    <p>Elevated urine acidity is associated with renal dysfunction</p> Signup and view all the answers

    What percentage of individuals with renal cell carcinoma also have urinary tract stones?

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

    Which factor is NOT typically associated with renal cell carcinoma?

    <p>Urinary tract infections</p> Signup and view all the answers

    What is a potential consequence of arterioles showing "onion-skin" appearance, as described in the provided text?

    <p>Decreased blood flow to the kidneys</p> Signup and view all the answers

    Based on the text, what is the relationship between arterioles and renal cell carcinoma?

    <p>The &quot;onion-skin&quot; appearance of arterioles is a risk factor for renal cell carcinoma.</p> Signup and view all the answers

    What is the primary focus of the text regarding renal vascular diseases?

    <p>The relationship between renal vascular diseases and kidney function</p> Signup and view all the answers

    What is the term used in the text to describe the narrowing of arterioles and smaller arteries?

    <p>Proliferation</p> Signup and view all the answers

    What is the primary cause of papilledema in the context of renal vascular diseases?

    <p>Leakage of fluid from injured retinal vessels</p> Signup and view all the answers

    What is the approximate percentage of patients observed over 10 years who developed renal cell carcinoma, based on the text?

    <p>7%</p> Signup and view all the answers

    Based on the text, what is the main symptom associated with increased intracranial pressure caused by renal vascular diseases?

    <p>Encephalopathy</p> Signup and view all the answers

    What is the primary focus of the text regarding renal vascular diseases?

    <p>The impact of renal vascular diseases on the kidneys and related systems</p> Signup and view all the answers

    The presence of dilated cysts in the kidney indicates benign nephrosclerosis.

    <p>False</p> Signup and view all the answers

    Fibrinoid necrosis of the afferent arteriole is a chronic lesion associated with malignant hypertension.

    <p>False</p> Signup and view all the answers

    Hyperplastic arteriolosclerosis is commonly observed in cases of long-standing hypertension.

    <p>True</p> Signup and view all the answers

    The external surface of a kidney affected by benign nephrosclerosis is typically smooth and shiny.

    <p>False</p> Signup and view all the answers

    Marked thickening of arteriole walls is a sign of renal damage due to vascular narrowing.

    <p>True</p> Signup and view all the answers

    Collagen fibers stain red when subjected to trichrome staining in kidney biopsies.

    <p>False</p> Signup and view all the answers

    Cortical atrophy in the kidney can result from benign nephrosclerosis.

    <p>True</p> Signup and view all the answers

    Benign nephrosclerosis and malignant hypertension share similar microscopic features.

    <p>False</p> Signup and view all the answers

    A deficiency in the ADAMTS13 protein can be acquired or inherited.

    <p>True</p> Signup and view all the answers

    The primary cause of Shiga toxin–mediated HUS is an infection with Streptococcus dysenteriae.

    <p>False</p> Signup and view all the answers

    The most common cause of complement-mediated HUS is due to genetic abnormalities, leading to complement dysregulation.

    <p>True</p> Signup and view all the answers

    Microangiopathic hemolytic anemia, a hallmark of thrombotic microangiopathies, is primarily caused by the fragmentation of red blood cells as they pass through narrowed blood vessels.

    <p>True</p> Signup and view all the answers

    The pathogenesis of TTP involves the disruption of the ADAMTS13 protein, which plays a crucial role in the cleavage of von Willebrand factor. This leads to the formation of abnormally large von Willebrand factor multimers, which can lead to platelet aggregation and microthrombi formation.

    <p>True</p> Signup and view all the answers

    The incidence of thrombotic thrombocytopenic purpura (TTP) is higher in individuals with a genetic deficiency in ADAMTS13 compared to those with an acquired deficiency.

    <p>False</p> Signup and view all the answers

    Patients with HUS commonly present with a rapid onset of symptoms, including hemolytic anemia, thrombocytopenia, and kidney failure.

    <p>True</p> Signup and view all the answers

    Acquired HUS is more common than inherited HUS due to the higher prevalence of genetic abnormalities affecting the complement system.

    <p>False</p> Signup and view all the answers

    Activation and aggregation of platelets in injured vessels release growth factors.

    <p>True</p> Signup and view all the answers

    Congestion of peritubular capillaries is frequently observed in acute tubular injury.

    <p>True</p> Signup and view all the answers

    Microangiopathic changes are present in approximately 20% of patients with malignant hypertension.

    <p>False</p> Signup and view all the answers

    Necrotic tubular epithelial cells are often observed in the lumen of tubules in acute tubular injury.

    <p>True</p> Signup and view all the answers

    Malignant hypertension is not a possible cause of acute tubular injury.

    <p>False</p> Signup and view all the answers

    Injury to tubular epithelial cells can lead to significant bleeding.

    <p>True</p> Signup and view all the answers

    Acute tubular injury is always reversible.

    <p>False</p> Signup and view all the answers

    Microangiopathic changes are a rare feature of acute tubular injury.

    <p>False</p> Signup and view all the answers

    Urinary stones generally have no connection to the risk of bacterial infections.

    <p>False</p> Signup and view all the answers

    Clear cell type of renal cell carcinoma is commonly associated with gross hemorrhage.

    <p>True</p> Signup and view all the answers

    Bathroom habits play a significant role in the diagnosis of kidney stones.

    <p>False</p> Signup and view all the answers

    The presence of necrosis and hemorrhage in renal cell carcinoma usually suggests that the tumor is benign.

    <p>False</p> Signup and view all the answers

    Radiological techniques are typically inadequate for diagnosing renal conditions.

    <p>False</p> Signup and view all the answers

    In renal vein invasion, cancer dissemination has a low tendency.

    <p>False</p> Signup and view all the answers

    Tumor cells in the clear cell type of renal carcinoma contain high levels of lipids and glycogen.

    <p>True</p> Signup and view all the answers

    Renal stones do not affect the morphology of the kidney tissue.

    <p>False</p> Signup and view all the answers

    Renal cell carcinoma is commonly associated with acquired mutations that mimic adaptive responses to hyperthermia.

    <p>False</p> Signup and view all the answers

    The most common histologic form of renal cell carcinoma is clear cell carcinoma.

    <p>True</p> Signup and view all the answers

    About 20% of individuals with renal cell carcinoma also have urinary tract stones.

    <p>False</p> Signup and view all the answers

    Pathogenesis of renal cell carcinoma involves the compression of the kidney parenchyma due to obstruction.

    <p>False</p> Signup and view all the answers

    Hydronephrosis is characterized by marked dilation of the cortex and thinning of renal medulla.

    <p>False</p> Signup and view all the answers

    Urine acidity is associated with improved renal function.

    <p>False</p> Signup and view all the answers

    Renal cell carcinoma has a striking propensity to metastasize through lymphatic vessels.

    <p>False</p> Signup and view all the answers

    The primary enzyme deficiency that causes renal cell carcinoma is ADAMTS13.

    <p>False</p> Signup and view all the answers

    What is the pathophysiological consequence of hypertension on small renal arteries, leading to a significant impact on kidney function?

    <p>Nephrosclerosis, which is characterized by the narrowing of arterioles and smaller arteries.</p> Signup and view all the answers

    What is the term used to describe the type of necrosis that occurs in the kidneys due to hypertension, and what is its characteristic feature?

    <p>Fibrinoid necrosis, characterized by the appearance of fibrin deposits.</p> Signup and view all the answers

    What is the primary mechanism by which chronic hypertension leads to end-stage renal disease?

    <p>Chronic ischemia, which causes nephrosclerosis and eventually leads to kidney failure.</p> Signup and view all the answers

    What is the characteristic histopathological feature of malignant hypertension in the kidneys?

    <p>Fibrinoid necrosis of the afferent arteriole.</p> Signup and view all the answers

    What is the term used to describe the narrowing of arterioles and smaller arteries in the kidneys, leading to ischemia and nephrosclerosis?

    <p>Arteriolosclerosis.</p> Signup and view all the answers

    What is the primary consequence of chronic ischemia in the kidneys, leading to nephrosclerosis?

    <p>Fibronoid necrosis and eventual kidney failure.</p> Signup and view all the answers

    What is the relationship between arterioles and arteriolosclerosis in the context of hypertension?

    <p>Arterioles are affected by arteriolosclerosis, leading to narrowing and ischemia.</p> Signup and view all the answers

    What is the clinical significance of papilledema in the context of renal vascular diseases?

    <p>It is a sign of increased intracranial pressure.</p> Signup and view all the answers

    What is the underlying cause of the 'lea-ben' appearance observed in medullary cystic disease?

    <p>rupture of small blood vessels leading to numerous medullary cysts</p> Signup and view all the answers

    What is the primary renal condition associated with thrombotic microangiopathies (TMAs) that involves severe hypertension?

    <p>Hemolytic-uremic syndrome (HUS)</p> Signup and view all the answers

    How does chronic ischemia contribute to the progression of nephrosclerosis?

    <p>Chronic ischemia leads to nephrosclerosis by causing widespread cortical atrophy and glomerular damage.</p> Signup and view all the answers

    What is the primary mechanism underlying the development of end-stage renal disease in patients with severe hypertension?

    <p>Severe hypertension leads to nephrosclerosis, which eventually progresses to end-stage renal disease.</p> Signup and view all the answers

    In the context of thrombotic microangiopathies, what condition is characterized by systemic sclerosis and hypertension?

    <p>Thrombotic thrombocytopenic purpura (TTP)</p> Signup and view all the answers

    What is the characteristic microscopic feature observed in the renal cortex during benign nephrosclerosis?

    <p>marked thickening of arteriole walls and cortical atrophy</p> Signup and view all the answers

    What contributes to increased blood pressure in the renin-angiotensin system during thrombotic microangiopathies?

    <p>Narrowing of small blood vessels.</p> Signup and view all the answers

    How does the injury to the endothelial surface contribute to the development of nephrosclerosis?

    <p>Endothelial injury leads to the release of vasoactive substances, which cause vasoconstriction and further damage to the kidney.</p> Signup and view all the answers

    What is a less severe form of vascular narrowing that differs from malignant hypertension but still results in renal damage?

    <p>Benign nephrosclerosis.</p> Signup and view all the answers

    What is the primary pathogenic factor underlying the development of thrombotic thrombocytopenic purpura (TTP)?

    <p>a deficiency of the ADAMTS13 enzyme</p> Signup and view all the answers

    Identify the condition characterized by both hypertension and vascular narrowing resulting in thrombocytopenia.

    <p>Thrombotic thrombocytopenic purpura (TTP).</p> Signup and view all the answers

    How does the compression of the kidney parenchyma contribute to the development of nephrosclerosis?

    <p>Compression of the kidney parenchyma leads to chronic ischemia, which causes further damage to the kidney.</p> Signup and view all the answers

    What pathological process is indicated by hyperplastic arteriolosclerosis seen in chronic hypertension?

    <p>Thickening of the walls of arterioles.</p> Signup and view all the answers

    What is the primary consequence of prolonged severe obstruction in the kidney?

    <p>Prolonged severe obstruction leads to cortical atrophy and nephrosclerosis.</p> Signup and view all the answers

    What is the role of increased renin release in the pathogenesis of thrombotic microangiopathies?

    <p>It elevates blood pressure and exacerbates renal ischemia.</p> Signup and view all the answers

    In the context of thrombotic microangiopathies, what systemic implications arise from renal tissue ischemia?

    <p>Activation of the coagulation cascade and systemic inflammation.</p> Signup and view all the answers

    What is the name of the disease characterized by an increased risk of developing renal cell carcinoma, and what is the approximate percentage increase in this risk?

    <p>IgG4-related disease, 30-fold</p> Signup and view all the answers

    What is the unusual type of hypertension that can be generated in the affected kidney parenchyma, and what is its effect on the renal pelvis and ureters?

    <p>Unusual type of hypertension is hydronephrosis, which can cause compression</p> Signup and view all the answers

    What is the presenting feature of more than 50% of cases of renal cell carcinoma, and what is the mechanism of its transmission?

    <p>Hematuria, transmitted through the collecting ducts</p> Signup and view all the answers

    What is the rare complication of IgG4-related disease, and how does it affect the renal vasculature?

    <p>Renal vasculitis, leading to arterial insufficiency</p> Signup and view all the answers

    What is the term used to describe the compression of the kidney parenchyma due to obstruction, and what is its effect on the renal function?

    <p>Hydronephrosis, impaired renal function</p> Signup and view all the answers

    What is the primary microscopic change observed in the glomeruli during prolonged obstruction, and what is its effect on the renal function?

    <p>Fibrosis, leading to impaired renal function</p> Signup and view all the answers

    What is the primary factor determining the frequency of anuria in patients with kidney obstruction, and how does it affect the renal function?

    <p>Severity of obstruction, leading to impaired renal function</p> Signup and view all the answers

    What is the term used to describe the condition characterized by a deficiency of ADAMTS13, and what is its effect on the blood clotting system?

    <p>Thrombotic thrombocytopenic purpura (TTP), leading to abnormal blood clotting</p> Signup and view all the answers

    What are the two primary microscopic changes observed in the kidney parenchyma during prolonged obstruction, and what is the underlying pathological process responsible for these changes?

    <p>The two primary microscopic changes observed are tubular dilation and atrophy, followed by interstitial fibrosis. The underlying pathological process is chronic ischemia due to the reduced blood flow caused by the obstruction.</p> Signup and view all the answers

    Based on the provided text, explain why anuria is a rare outcome of bilateral complete kidney obstruction.

    <p>Anuria, complete absence of urine production, is rare in bilateral complete obstruction because even with complete blockage, some degree of urine production is usually maintained by the kidneys. The text states that anuria only results when the obstruction is both bilateral and complete, a rare scenario.</p> Signup and view all the answers

    Describe the specific morphological changes that occur in the kidney due to chronic hypertension and their impact on the organ's function.

    <p>Chronic hypertension leads to hyaline arteriolosclerosis, characterized by thickening of the arteriolar walls due to deposition of hyaline material. This narrowing of the arterioles reduces blood flow to the nephrons, causing ischemia and eventually leading to atrophy of the kidney parenchyma and impaired renal function.</p> Signup and view all the answers

    Explain the role of ADAMTS13 in the pathogenesis of thrombotic thrombocytopenic purpura (TTP), and why its deficiency leads to the disease.

    <p>ADAMTS13 is an enzyme responsible for cleaving large von Willebrand factor (VWF) multimers into smaller units. Deficiency in ADAMTS13 leads to an accumulation of large VWF multimers, which can bind platelets and cause widespread microvascular thrombosis, leading to the clinical manifestations of TTP.</p> Signup and view all the answers

    Describe the key characteristics of renal cell carcinoma, including its histologic features, typical metastasis pattern, and any associated factors.

    <p>Renal cell carcinoma is characterized by clear cell histology, often with a tendency to metastasize to the lungs, liver, bones, and brain. It is associated with smoking, obesity, and exposure to certain chemicals, and can present with hematuria, flank pain, and a palpable abdominal mass.</p> Signup and view all the answers

    How does the text explain the development of papilledema in the context of renal vascular diseases, and what is the primary clinical consequence of this phenomenon?

    <p>The text describes papilledema as a consequence of increased intracranial pressure due to hypertensive encephalopathy caused by renal vascular diseases. This condition leads to headache, blurred vision, and potential blindness, highlighting the serious implications of uncontrolled hypertension in the context of renal disease.</p> Signup and view all the answers

    Based on the text, explain the relationship between chronic ischemia and the microscopic changes observed in the kidney during prolonged obstruction.

    <p>The text states that chronic ischemia, resulting from prolonged obstruction, leads to atrophy of the kidney parenchyma, characterized by tubular dilation and atrophy followed by interstitial fibrosis. This underscores the impact of reduced blood flow on kidney structure and function, highlighting the importance of timely intervention in cases of obstruction.</p> Signup and view all the answers

    Explain how the text describes the progression of nephrosclerosis in relation to hypertension, including the microscopic changes that occur in the arterioles.

    <p>The text describes nephrosclerosis as a consequence of chronic hypertension. It explains that the small renal arteries undergo hyaline arteriolosclerosis, resulting in thickening of the arteriolar walls due to deposition of hyaline material. This narrowing of the arterioles reduces blood flow to the nephrons, causing ischemia and eventually leading to atrophy of the kidney parenchyma. The text emphasizes the relationship between hypertension, arteriolosclerosis, ischemia, and the decline in renal function.</p> Signup and view all the answers

    The most common type of kidney stones (about 80%) consists of ______ oxalate.

    <p>calcium</p> Signup and view all the answers

    In severe cases, ______ or ______ may be admixed with calcium phosphate.

    <p>whey</p> Signup and view all the answers

    Most patients with these stones may produce ischemic necrosis of the ______ cortex.

    <p>renal</p> Signup and view all the answers

    The ______ type of stone excretes excess calcium in the urine.

    <p>narrow</p> Signup and view all the answers

    Narrowing caused by thrombi in small vessels creates sheer forces that may be ______ to unexplained, excessive absorption of calcium.

    <p>secondary</p> Signup and view all the answers

    Only a minority of patients with calcium stones have ______ in peripheral blood smears.

    <p>schistocytes</p> Signup and view all the answers

    Less common than calcium stones are ______ stones, which typically arise in the setting of.

    <p>magnesium-containing</p> Signup and view all the answers

    The clinical features of nephrosclerosis are primarily a consequence of ______ of the kidney.

    <p>ischemia</p> Signup and view all the answers

    Tumors are usually ______, but there is also a rare familial form.

    <p>sporadic</p> Signup and view all the answers

    There is neither hyperuricemia nor increased urine ______, but instead an increased urine frequency.

    <p>urate</p> Signup and view all the answers

    This association has led to the discovery that the most frequent ______ mutation in both the familial and sporadic forms is loss or inactivation of the VHL gene.

    <p>driver</p> Signup and view all the answers

    The VHL gene regulates the ______ of several genes, including cystine.

    <p>transcription</p> Signup and view all the answers

    Cystine stones are almost invariably associated with ______ urine (pH < 5.5).

    <p>acidic</p> Signup and view all the answers

    This association has led to the discovery that the most frequent driver mutation in both the familial and sporadic forms is loss or inactivation of the ______ gene.

    <p>VHL</p> Signup and view all the answers

    The VHL gene regulates the transcription of several genes, including ______.

    <p>cystine</p> Signup and view all the answers

    Tumors are usually sporadic, but there is also a rare ______ form.

    <p>familial</p> Signup and view all the answers

    In _____, the dominant symptoms are more often related to involvement of organs other than the kidney, such as the brain.

    <p>TTP</p> Signup and view all the answers

    TTP typically follows a rapid __________ course, with survival rates of approximately 10%.

    <p>fatal</p> Signup and view all the answers

    Thrombotic thrombocytopenic purpura (TTP) is another form of _______ microangiopathic anemia.

    <p>systemic</p> Signup and view all the answers

    TTP is caused by a deficiency of the __________ enzyme and/or the presence of an inhibitor of the enzyme.

    <p>ADAMTS13</p> Signup and view all the answers

    Treatment of TTP involves _________ exchange or infusion of a solvent detergent-treated plasma.

    <p>plasma</p> Signup and view all the answers

    The primary goal of therapy in TTP is to __________ the missing ADAMTS13 enzyme and remove the pathogenic autoantibody.

    <p>replace</p> Signup and view all the answers

    The __________ factor is a protease that cleaves von Willebrand factor, reducing its activity and preventing platelet adhesion.

    <p>ADAMTS13</p> Signup and view all the answers

    TTP is characterized by the formation of __________ thrombi in small blood vessels, leading to thrombocytopenia and microangiopathic hemolytic anemia.

    <p>platelet-rich</p> Signup and view all the answers

    Malignant ______, defined as a rapid increase in blood pressure to greater than 200/120 mm Hg, occurs in less than 5% of individuals.

    <p>hypertension</p> Signup and view all the answers

    Small arterioles and arterioles in the kidney suffer severe consequences of malignant ______.

    <p>hypertension</p> Signup and view all the answers

    Endothelial injury and leakage of plasma proteins into the vessel wall produce ______ necrosis.

    <p>fibrinoid</p> Signup and view all the answers

    The appearance of fibrinoid necrosis is named because it has the appearance of ______.

    <p>fibrin</p> Signup and view all the answers

    Acute renal ______ is often caused by malignant hypertension.

    <p>failure</p> Signup and view all the answers

    A rapid increase in blood pressure can lead to major ______ consequences in the kidney.

    <p>consequences</p> Signup and view all the answers

    Malignant hypertension is characterized by the presence of small renal ______.

    <p>arterioles</p> Signup and view all the answers

    Malignant hypertension occurs due to an increase in blood pressure affecting the renal ______.

    <p>arteries</p> Signup and view all the answers

    Severe _______________ paroxysms of flank pain radiating towards the groin (renal colic) can occur,

    <p>paroxysms</p> Signup and view all the answers

    Morphology. Grossly, the clear cell type of renal cell _______________ is often associated with gross hemorrhage.

    <p>carcinoma</p> Signup and view all the answers

    Because they often obstruct the low urinary tract, stones of a certain _______________ may predispose to bacteremia and infections.

    <p>types</p> Signup and view all the answers

    The diagnosis is readily made radiologically. Microscopically, the cells contain abundant _______________ and glycogen, accounting for the clear appearance of the cells after tissue processing.

    <p>lipid</p> Signup and view all the answers

    Renal cell carcinoma is often associated with gross _______________ and may exhibit areas of necrosis and hemorrhage, and may excrete urine.

    <p>hematuria</p> Signup and view all the answers

    Microscopically, the cells of renal cell carcinoma are characterized by a clear _______________ and typically have a abundant cytoplasm.

    <p>cytoplasm</p> Signup and view all the answers

    The most common histologic form of renal cell carcinoma is the clear cell _______________ type.

    <p>subtype</p> Signup and view all the answers

    Renal cell carcinoma has a _______________ propensity to invade renal veins.

    <p>marked</p> Signup and view all the answers

    Match the following kidney conditions with their associated characteristics:

    <p>Benign nephrosclerosis = Cortical atrophy and finely granular surface Malignant hypertension = Fibrinoid necrosis of afferent arteriole Hyperplastic arteriolosclerosis = Onion-skin appearance of arterial walls Tubular atrophy = Resulting from vascular narrowing and interstitial fibrosis</p> Signup and view all the answers

    Match the type of staining with the component it visualizes in kidney biopsies:

    <p>Trichrome stain = Collagen fibers are stained blue PAS stain = Stains for carbohydrates in the kidney Hematoxylin-eosin stain = Commonly used for general tissue structure Immunofluorescence = Visualizes specific proteins in tissues</p> Signup and view all the answers

    Match the following pathological features with their kidney conditions:

    <p>Marked thickening of walls = Associated with vascular narrowing Dilated cysts = Indicates potential renal dysfunction Granular surface = Charateristic of benign nephrosclerosis Arteriolar hyaline deposition = Observed in hypertensive conditions</p> Signup and view all the answers

    Match the following descriptions with the correct renal pathology:

    <p>Benign nephrosclerosis = External surface shows granular appearance Malignant hypertension = Typically acute lesion with severe consequences Hyperplastic arteriolosclerosis = More often seen in long-standing hypertension Chronic ischemia = Can lead to irreversible kidney damage</p> Signup and view all the answers

    Match the changes observed in the kidney under specific conditions:

    <p>Dilated cysts = Marked enlargement of kidney Cortical atrophy = Result of benign nephrosclerosis Fibrinoid necrosis = Indicator of malignant hypertension Onion-skin lesion = Related to hyperplastic arteriolosclerosis</p> Signup and view all the answers

    Match the following microscopic findings to their kidney condition:

    <p>Hyaline deposition = Characteristic of benignant nephrosclerosis Onion-skin appearance = Seen in chronic hypertension cases Tubular atrophy = Related to interstitial fibrosis Fibrinoid necrosis = Acute manifestation in malignant hypertension</p> Signup and view all the answers

    Match the fibers with their corresponding staining methods:

    <p>Collagen fibers = Stained blue in trichrome stain Elastic fibers = Typically stained with Verhoeff's method Muscle fibers = Stained pink with eosin Glomerular basement membrane = Visualized using PAS stain</p> Signup and view all the answers

    Match the following terms to their descriptions:

    <p>Cortical atrophy = Resulting from chronic ischemic injury Fibrinoid necrosis = Damage seen in acute renal pathology Onion-skin lesion = Clinically indicative of severe hypertension Hyaline deposition = Affects smaller arteries in hypertensive patients</p> Signup and view all the answers

    Match the following thrombotic microangiopathies with their etiology:

    <p>Shiga toxin–mediated HUS = Acquired from Shiga toxin-producing E. coli Complement-mediated HUS (Inherited) = Genetic abnormalities causing complement dysregulation TTP (Acquired) = ADAMTS13 deficiency due to autoantibodies Complement-mediated HUS (Acquired) = Autoantibodies causing complement dysregulation</p> Signup and view all the answers

    Match the following types of HUS with their characteristics:

    <p>Shiga toxin–mediated HUS = Commonly caused by E. coli infection Complement-mediated HUS (Inherited) = Relatively rare genetic condition TTP (Inherited) = Caused by ADAMTS13 deficiency TTP (Acquired) = ADAMTS13 deficiency due to autoimmune response</p> Signup and view all the answers

    Match the following forms of HUS with their associated organisms:

    <p>Shiga toxin–mediated HUS = Shiga toxin-producing E. coli Complement-mediated HUS (Acquired) = Autoantibodies leading to dysregulation TTP = Deficiency of ADAMTS13 enzyme</p> Signup and view all the answers

    Match the following thrombotic microangiopathy diseases with their pathogenesis:

    <p>Shiga toxin–mediated HUS = E. coli infection leading to toxin release Complement-mediated HUS (Inherited) = Genetic defects in complement regulation TTP (Acquired) = Immunological attack on ADAMTS13 TTP (Inherited) = Inborn error causing enzyme deficiency</p> Signup and view all the answers

    Match the following definitions with their corresponding terms:

    <p>ADAMTS13 = A protease cleaving von Willebrand factor HUS = Hemolytic-uremic syndrome TTP = Thrombotic thrombocytopenic purpura Complement dysregulation = Anterior to complement-mediated HUS</p> Signup and view all the answers

    Match the following types of HUS with their inherited or acquired classification:

    <p>Shiga toxin–mediated HUS = Acquired Complement-mediated HUS (Inherited) = Inherited TTP (Inherited) = Inherited TTP (Acquired) = Acquired</p> Signup and view all the answers

    Match the following descriptions with their respective forms of HUS:

    <p>Shiga toxin–mediated HUS = Common in children with diarrhea Complement-mediated HUS (Acquired) = Associated with antibodies TTP = Characterized by thrombocytopenia and organ failure Complement-mediated HUS (Inherited) = Genetically predisposed cases</p> Signup and view all the answers

    Match the following clinical features with their respective thrombotic microangiopathy:

    <p>Shiga toxin–mediated HUS = May lead to acute renal failure Complement-mediated HUS (Inherited) = Symptoms arise from inherited defects TTP (Acquired) = Triggered by immune response TTP (Inherited) = Due to genetic deficiency of protease</p> Signup and view all the answers

    Match the following medical conditions with their corresponding descriptions based on the provided text:

    <p>Nephrosclerosis = Characterized by arteriolar sclerosis, with thickening of the walls and narrowing of the lumen, leading to decreased blood flow to the kidney. Renal cell carcinoma = A type of cancer arising from the epithelial cells lining the renal tubules, often presenting with hematuria and a palpable mass. Hydronephrosis = A condition characterized by dilation of the renal pelvis and calyces due to obstruction of the urinary outflow tract. Thrombotic Thrombocytopenic Purpura (TTP) = A rare, life-threatening condition caused by a deficiency of the ADAMTS13 protein, leading to the formation of small blood clots in the microcirculation.</p> Signup and view all the answers

    Match the following microscopic features with their corresponding conditions based on the provided text:

    <p>Fibrinoid necrosis of the afferent arteriole = A characteristic feature of malignant hypertension. Onion-skin appearance of arterioles = A hallmark of hyperplastic arteriolosclerosis, often seen in patients with long-standing hypertension. Dilated cysts in the kidney = Typically associated with benign nephrosclerosis. Papillary architecture with complex branching = A characteristic feature of renal cell carcinoma.</p> Signup and view all the answers

    Match the following terms with their corresponding definitions based on the provided text:

    <p>Angiogenesis = The formation of new blood vessels, a process often regulated by vascular endothelial growth factor (VEGF). Arteriolosclerosis = A thickening and narrowing of the walls of small arteries and arterioles, often associated with hypertension. Hematuria = The presence of blood in the urine, a common symptom of renal cell carcinoma. Metastasis = The spread of cancer cells from the primary tumor to distant sites in the body.</p> Signup and view all the answers

    Match the following statements with their corresponding conditions based on the provided text:

    <p>The external surface of the kidney is smooth and shiny. = Characteristic of benign nephrosclerosis. A palpable mass in the kidney may be present. = A common finding in renal cell carcinoma. The patient may present with symptoms of headache and visual disturbances. = May occur due to increased intracranial pressure caused by renal vascular diseases. The patient may experience fatigue, shortness of breath, and edema. = Common symptoms associated with chronic kidney disease due to nephrosclerosis.</p> Signup and view all the answers

    Match the following genetic alterations with their corresponding effects on renal cell carcinoma based on the provided text:

    <p>Mutations in the MET oncogene = Can activate signaling pathways leading to increased cell growth and survival. Mutations in the VHL gene = Can lead to the overproduction of vascular endothelial growth factor (VEGF), promoting angiogenesis. Mutations in the TP53 gene = Can contribute to the development of renal cell carcinoma. Mutations in the PTEN gene = Can lead to the deregulation of cell growth and proliferation.</p> Signup and view all the answers

    Match the following clinical presentations with their corresponding conditions based on the provided text:

    <p>Sudden onset of hematuria, oliguria, and hypertension. = Suggestive of thrombotic thrombocytopenic purpura (TTP). Gradual onset of proteinuria, hypertension, and edema. = Consistent with chronic kidney disease due to nephrosclerosis. Painless hematuria, a palpable mass, and fatigue. = Classic symptoms of renal cell carcinoma. Severe headache, visual disturbances, and altered mental status. = May indicate increased intracranial pressure caused by renal vascular diseases.</p> Signup and view all the answers

    Match the following treatment modalities with their corresponding conditions based on the provided text:

    <p>Plasmapheresis = Used in the treatment of thrombotic thrombocytopenic purpura (TTP). Antihypertensive medications = Essential for managing hypertension in patients with nephrosclerosis. Surgery and chemotherapy = Often employed in the treatment of renal cell carcinoma. Dialysis and kidney transplantation = May be necessary for patients with end-stage renal disease due to nephrosclerosis.</p> Signup and view all the answers

    Match the following microscopic features with their corresponding conditions based on the provided text:

    <p>Cortical atrophy = A consequence of chronic ischemia and damage to the kidney parenchyma in nephrosclerosis. Thickening of the glomerular basement membrane = A feature of diabetic nephropathy. Infiltration of inflammatory cells = A hallmark of acute pyelonephritis. Papillary necrosis = A potential complication of long-standing urinary tract obstruction.</p> Signup and view all the answers

    Match the following terms related to kidney disease with their corresponding descriptions:

    <p>Hydronephrosis = Expansion of the renal pelvis and calyces due to urine obstruction. Nephrosclerosis = Hardening of the renal arteries, leading to reduced blood flow to the kidneys. Renal cell carcinoma = A type of kidney cancer that originates from the lining of the renal tubules. Paroxysms = Sudden attacks or episodes of a condition, such as pain or hypertension.</p> Signup and view all the answers

    Match the following microscopic findings with their corresponding conditions:

    <p>Onion-skin appearance of arterioles = Hyperplastic arteriolosclerosis, a feature of long-standing hypertension. Fibrinoid necrosis of the afferent arteriole = Malignant hypertension, a severe form of high blood pressure. Dilated cysts in the kidney = Benign nephrosclerosis, a common age-related kidney change. Clear cell appearance of tumor cells = Renal cell carcinoma, a type of kidney cancer.</p> Signup and view all the answers

    Match the following clinical manifestations with their corresponding causes:

    <p>Severe pain in the flank = Kidney stones, which can obstruct the urinary tract. Increased intracranial pressure = Renal vascular diseases, which can lead to papilledema. Anemia = Chronic kidney disease, which can impair the production of erythropoietin. Urinary tract infections = Stones in the urinary tract, which can create a breeding ground for bacteria.</p> Signup and view all the answers

    Match the following pathological processes with their potential consequences:

    <p>Obstruction of the urinary tract = Hydronephrosis, expansion of the renal pelvis and calyces. Hypertension = Nephrosclerosis, hardening of the renal arteries. Tumor growth in the kidney = Renal cell carcinoma, a type of kidney cancer. Deficiency of ADAMTS13 = Thrombotic thrombocytopenic purpura (TTP), a rare but serious blood disorder.</p> Signup and view all the answers

    Match the following terms with their corresponding definitions:

    <p>Necrosis = Death of cells or tissues. Hemorrhage = Bleeding. Glycogen = A complex carbohydrate stored in the liver and muscles. Invade = To spread or penetrate into surrounding tissues.</p> Signup and view all the answers

    Match the following histological features with their corresponding conditions:

    <p>Clear cell appearance = Renal cell carcinoma, a type of kidney cancer. Fibrinoid necrosis = Malignant hypertension, a severe form of high blood pressure. Onion-skin appearance = Hyperplastic arteriolosclerosis, a feature of long-standing hypertension. Dilated cysts = Benign nephrosclerosis, a common age-related kidney change.</p> Signup and view all the answers

    Match the following risk factors with their corresponding diseases:

    <p>Hypertension = Nephrosclerosis, hardening of the renal arteries. Smoking = Renal cell carcinoma, a type of kidney cancer. Obesity = Kidney stones, which can obstruct the urinary tract. Family history = Thrombotic thrombocytopenic purpura (TTP), a rare but serious blood disorder.</p> Signup and view all the answers

    Match the following treatments with their corresponding conditions:

    <p>Plasma exchange = Thrombotic thrombocytopenic purpura (TTP), a rare but serious blood disorder. Surgery = Kidney stones, which can obstruct the urinary tract. Antihypertensive medications = Nephrosclerosis, hardening of the renal arteries. Chemotherapy and radiation therapy = Renal cell carcinoma, a type of kidney cancer.</p> Signup and view all the answers

    Match the following terms related to TTP with their descriptions:

    <p>TTP = A thrombotic emergency with a high mortality rate ADAMTS13 = An enzyme that cleaves UL-vWF multimers Plasma exchange = A treatment approach that removes the pathogenic autoantibody Microangiopathic hemolytic anemia = A condition characterized by the destruction of red blood cells</p> Signup and view all the answers

    Match the following terms related to TTP with their consequences:

    <p>Thrombocytopenia = Decreased platelet count Microangiopathic hemolytic anemia = Destruction of red blood cells Neurological symptoms = Damage to the brain Renal dysfunction = Damage to the kidneys</p> Signup and view all the answers

    Match the following terms related to TTP therapy with their mechanisms:

    <p>Plasma exchange = Removes the pathogenic autoantibody and repaces the missing ADAMTS13 enzyme Plasma infusion = Replaces the missing ADAMTS13 enzyme Rituximab = Suppresses the production of the pathogenic autoantibody Corticosteroids = Suppresses the immune system</p> Signup and view all the answers

    Match the following terms related to TTP with their associations:

    <p>TTP = Thrombotic thrombocytopenic purpura HUS = Hemolytic uremic syndrome ADAMTS13 = A disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 UL-vWF = Unusually large von Willebrand factor</p> Signup and view all the answers

    Match the following terms related to TTP with their characteristics:

    <p>TTP = A life-threatening thrombotic emergency HUS = A thrombotic microangiopathy ADAMTS13 = A metalloprotease enzyme UL-vWF = A multimeric protein</p> Signup and view all the answers

    Match the following terms related to TTP with their primary sites of involvement:

    <p>TTP = Brain and kidneys HUS = Kidneys ADAMTS13 = Liver UL-vWF = Endothelial cells</p> Signup and view all the answers

    Match the following terms related to TTP with their frequencies:

    <p>TTP = Affects approximately 1 in 1,000,000 individuals HUS = Affects approximately 1 in 100,000 individuals ADAMTS13 deficiency = Occurs in approximately 10% of TTP cases UL-vWF = Is present in approximately 50% of TTP cases</p> Signup and view all the answers

    Match the following terms related to TTP with their outcomes:

    <p>TTP = Has a mortality rate of approximately 90% without treatment HUS = Has a mortality rate of approximately 10% with treatment ADAMTS13 replacement = Improves outcomes in approximately 80% of TTP cases Plasma exchange = Reduces mortality in approximately 90% of TTP cases</p> Signup and view all the answers

    Study Notes

    Renal Carcinoma and Vascular Diseases

    • Increased risk of renal cell carcinoma (RCC) with a 30-year history, occurring in about 7% of cases.
    • Larger renal vessels exhibit greater proliferation of intimal cells, leading to structural changes.
    • Arterioles and small arteries become severely narrowed, contributing to ischemic conditions.

    Clinical Features of Vascular Diseases

    • Malignant hypertension can impact kidney function and may lead to conditions like papilledema and encephalopathy.
    • Symptoms include headache, nausea, vomiting, and visual disturbances due to increased intracranial pressure.
    • Nephrosclerosis is characterized by chronic ischemia and progressive narrowing of renal arteries.

    Pathogenesis and Clinical Implications

    • Thrombotic thrombocytopenic purpura (TTP) caused by deficiency of the ADAMTS13 protease results in a rapid clinical decline.
    • Early intervention significantly improves survival rates, with typical survival rates estimated around 10% without treatment.
    • Management may involve plasma exchange to remove pathogenic autoantibodies.

    Morphological Changes in Kidney Diseases

    • Renal morphology in conditions like hydronephrosis shows dilation of the pelvis and calyces, compressing renal parenchyma.
    • Chronic obstruction can result in severe and prolonged obstruction effects, leading to renal damage.
    • Renal parenchyma may undergo atrophy and fibrosis as part of the disease process.

    Renal Cell Carcinoma

    • RCC is a malignant tumor of tubular epithelium linked with hypoxia-mimicking mutations that increase metastatic potential.
    • Common portal of metastasis is through the blood vessels, leading to systemic spread.
    • About 50% of individuals with RCC may also have renal calculi (stones), which can complicate clinical management.

    Pathophysiology of Kidney Injury

    • Platelets activate and aggregate at sites of injured vessels, releasing growth factors that stimulate cell proliferation in the adjacent intima, potentially leading to lumen narrowing.
    • Severe vessel damage can result in small hemorrhages, indicating notable clinical and morphologic changes in the kidneys.

    Acute Tubular Injury

    • Characterized by necrotic tubular epithelial cells and cellular debris in tubular lumens.
    • Congestion of peritubular capillaries is a prominent feature in this condition.

    Malignant Hypertension

    • Fibrinoid necrosis of afferent arterioles is an acute lesion typically seen in malignant hypertension.
    • Hyperplastic arteriolosclerosis presents as onion-skin lesions, more common in cases of long-standing hypertension.
    • Patients often show symptoms including sudden onset of renal issues, bleeding problems, and microangiopathic hemolytic anemia.

    Primary Thrombotic Microangiopathies

    • Shiga toxin–mediated Hemolytic-Uremic Syndrome (HUS): Acquired condition linked to infections like E. coli.
    • Complement-mediated HUS: May arise from genetic abnormalities or autoantibodies.
    • Thrombotic Thrombocytopenic Purpura (TTP): Associated with ADAMTS13 deficiency; can be inherited or acquired.

    Renal Cell Carcinoma

    • A malignant tumor of tubular epithelium linked to acquired mutations that emulate responses to hypoxia.
    • Has a high propensity for metastasizing via blood vessels.
    • Renal cell carcinoma often presents with painless hematuria and potential flank pain.

    Morphology of Renal Conditions

    • Benign nephrosclerosis features a granular external surface, cortical atrophy, and hyaline deposition in arterioles.
    • Malignant tumors present with areas of necrosis and hemorrhage, often causing urinary obstruction.
    • Clear cell renal carcinoma exhibits clear cytoplasm due to glycogen and lipid accumulation.

    Hydronephrosis

    • Identified by dilation of the renal pelvis and calyces, resulting in thinning of renal parenchyma.
    • Often associated with obstructions or tumors affecting urinary flow.

    Malignant Hypertension

    • Defined by a rapid increase in blood pressure exceeding 200/120 mm Hg, found in less than 5% of individuals.
    • Often results in acute renal failure due to damage to small arterioles in the kidneys.

    Pathogenesis

    • Major consequences include endohelial injury and plasma protein leakage into vessel walls, resulting in fibrinoid necrosis, characterized by a fibrin-like appearance.
    • Severe hypertension can lead to hemolytic anemia and is associated with primary forms of Hemolytic Uremic Syndrome (HUS).
    • Injury contributes to secondary forms of thrombotic microangiopathies (TMAs), leading to narrowing of small vessels.

    Morphological Changes

    • Widespread cortical atrophy and necrosis can occur, with evidence of hemorrhages within the renal tissue.
    • Cysts may develop in the renal medulla and appear as “leafy” patterns due to ruptured blood vessels.

    Clinical Features

    • Chronic kidney damage results in atrophy and often fibrosis of renal tissue.
    • Symptoms can include oliguria due to severe obstruction, though complete loss of function is rare.
    • Patients may have an increased risk of renal cell carcinoma, particularly in benign nephrosclerosis cases.

    Associated Conditions

    • Unusual high blood pressure in the renal pelvis can cause hydronephrosis and associated complications.
    • Typically presents with hematuria or palpable masses, which can cause flank pain or indicate vascular insufficiency.
    • Imaging may show related changes in the kidneys and collecting ducts, suggesting underlying vascular disturbances.

    Thrombotic Microangiopathies

    • Characterized by different pathogenic mechanisms related to malignant hypertension, leading to conditions like HUS and Thrombotic Thrombocytopenic Purpura (TTP).
    • These disorders induce significant injury and vascular narrowing, compounding renal and systemic effects.

    Overall Renal Impact

    • Chronic conditions lead to progressive deterioration of renal function, with significant morbidity associated with the sequelae of long-standing high blood pressure.
    • Complications arise from both renal and systemic vasculature due to the sustained high pressure, underscoring the importance of early intervention and management of hypertension.

    Malignant Hypertension

    • Defined as a rapid increase in blood pressure exceeding 200/120 mm Hg.
    • Affects less than 5% of individuals with hypertension.
    • Often leads to acute renal failure due to damage in small arteries and arterioles in the kidneys.

    Pathogenesis

    • Results in endothelial injury and plasma protein leakage into the bloodstream.
    • Causes fibrinoid necrosis characterized by a fibrin-like appearance.
    • Thrombotic thrombocytopenic purpura (TTP) displays distinct symptoms from C3 glomerulonephritis.
    • Can affect organs beyond the kidneys, including the brain.

    TTP and Thrombotic Microangiopathy (TMA)

    • Caused by deficiency of the enzyme ADAMTS13 or pathogenic autoantibodies.
    • Typical treatment involves plasma exchange to reduce antibody levels and restore enzyme activity.
    • TMA primarily leads to thrombotic microangiopathy in glomerular capillaries.

    Kidney Stones

    • About 80% of stones consist of calcium oxalate.
    • Severe cases may involve mixed calcium phosphate stones.
    • Asymmetric necrosis may be induced by excessive calcium absorption.
    • Stones can cause severe flank pain known as renal colic.

    Clinical Features

    • Tumors associated with renal conditions are usually sporadic but also rare familial hyperuricemia syndromes.
    • Low urine pH (<5.5) related to acidic urine can contribute to stone formation.
    • Cystine stones are often linked to genetic mutations affecting renal transport of amino acids.

    Morphology

    • Clear cell renal carcinoma typically presents with gross hemorrhage and necrosis.
    • Kidney stones can obstruct urinary pathways, leading to bacterial infections.
    • Diagnosis is often made through radiological imaging, with evidence of lipids and glycogen in affected cells indicative of clear cell carcinoma.

    Miscellaneous

    • The association between von Hippel-Lindau disease and renal tumors highlights genetic predispositions.
    • Obstruction from stones increases the risk of renal infections and invasion by surrounding tissues.

    Kidney Pathology

    • Enlarged kidneys can exhibit numerous dilated cysts, indicating potential pathology.
    • Benign nephrosclerosis presents with a finely granular external surface due to scarring and cortical atrophy when sectioned.
    • Tubular atrophy may occur from vascular narrowing, and interstitial fibrosis is observable upon biopsy.
    • Trichrome stain is used to visualize collagen, which appears blue, aiding in the identification of fibrosis.

    Hypertension Effects on Kidneys

    • Malignant hypertension can cause fibrinoid necrosis in afferent arterioles, characterizing an acute pathological state.
    • Hyperplastic arteriolosclerosis, often seen with long-standing hypertension, shows typical onion-skin lesions, requiring PAS stain for visualization.

    Thrombotic Microangiopathies (TMA)

    • Thrombotic Thrombocytopenic Purpura (TTP) arises from a deficiency in the ADAMTS13 enzyme, crucial for von Willebrand factor cleavage.
    • TTP can lead to a rapid fulminant course with survival rates around 10%, but timely treatment significantly reduces mortality risks.
    • Treatment may involve plasma exchange to remove pathogenic autoantibodies or replace missing enzymes.

    Forms of Hemolytic Uremic Syndrome (HUS)

    • Shiga toxin-mediated HUS is acquired from Shiga toxin-producing E. coli and Shigella dysenteriae serotype 1.
    • Complement-mediated HUS can be either inherited, due to genetic abnormalities, or acquired through autoantibodies inducing complement dysregulation.

    Pathogenesis and Clinical Features

    • In the context of kidney injury, patients may exhibit signs of hematuria, bleeding problems, and acute renal failure.
    • Malignant processes such as renal cell carcinoma can lead to the development of stones, especially in the renal pelvis and calyces, contributing to further complications.

    Kidney Stones Morphology

    • Kidney stones are typically small, ranging from 2 to 3 mm, and can obstruct urine flow, causing significant renal damage.
    • Clear cell renal carcinoma often shows areas of necrosis and hemorrhage, with a propensity to invade renal veins and can be diagnosed radiographically due to their distinct morphology.
    • Microscopic examination often reveals abundant lipids and glycogen in tumor cells, contributing to their clear appearance.

    Genetic Considerations in TMA

    • TMA pathologies showcase a variety of genetic predispositions, emphasizing the need for genetic diagnoses in affected individuals.
    • The identification of specific gene mutations or deficiencies plays a crucial role in determining treatment strategy and understanding disease progression.

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