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Questions and Answers
Which of the following is NOT a cause of thrombotic microangiopathy?
Which of the following is NOT a cause of thrombotic microangiopathy?
One of the components of the triad for Hemolytic Uremic Syndrome is hypertension.
One of the components of the triad for Hemolytic Uremic Syndrome is hypertension.
False
What is the common age of onset for typical Hemolytic Uremic Syndrome?
What is the common age of onset for typical Hemolytic Uremic Syndrome?
Childhood
Malignant Hypertension includes accelerated HTN and ______.
Malignant Hypertension includes accelerated HTN and ______.
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Match the following symptoms with their related conditions:
Match the following symptoms with their related conditions:
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Which of the following is considered the first-line treatment for renal artery stenosis?
Which of the following is considered the first-line treatment for renal artery stenosis?
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Bilateral renal artery stenosis has a good prognosis.
Bilateral renal artery stenosis has a good prognosis.
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What is the primary radiological screening test for renal artery stenosis?
What is the primary radiological screening test for renal artery stenosis?
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In renal artery stenosis, the contraindicated treatment that may decrease GFR is _____ inhibitors.
In renal artery stenosis, the contraindicated treatment that may decrease GFR is _____ inhibitors.
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Match the following treatments with their effects or indications:
Match the following treatments with their effects or indications:
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What condition is necessary to rule out before diagnosing chronic kidney disease (CKD)?
What condition is necessary to rule out before diagnosing chronic kidney disease (CKD)?
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Acute kidney injury (AKI) can be caused by acute tubular nephrosis (ATN) and acute interstitial nephrosis (AIN).
Acute kidney injury (AKI) can be caused by acute tubular nephrosis (ATN) and acute interstitial nephrosis (AIN).
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What is the most common cause of rapidly progressive renal failure (RPRF)?
What is the most common cause of rapidly progressive renal failure (RPRF)?
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Renal artery stenosis (RAS) is also known as _______.
Renal artery stenosis (RAS) is also known as _______.
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Match the renal conditions with their characteristics:
Match the renal conditions with their characteristics:
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What is a common cause of renal artery stenosis in a young male population?
What is a common cause of renal artery stenosis in a young male population?
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Increased aldosterone is part of the pathophysiology of unilateral renal artery stenosis.
Increased aldosterone is part of the pathophysiology of unilateral renal artery stenosis.
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What happens to renal blood flow in unilateral renal artery stenosis?
What happens to renal blood flow in unilateral renal artery stenosis?
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The primary renal artery condition affecting the Indian/Asian population is __________.
The primary renal artery condition affecting the Indian/Asian population is __________.
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Match the conditions with their corresponding characteristics:
Match the conditions with their corresponding characteristics:
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What is a primary indicator of renal artery stenosis seen during an ultrasound?
What is a primary indicator of renal artery stenosis seen during an ultrasound?
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The presence of chronic kidney disease (CKD) can result in the need for hemodialysis or kidney transplant.
The presence of chronic kidney disease (CKD) can result in the need for hemodialysis or kidney transplant.
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Name two parameters measured in a 24-hour urine analysis.
Name two parameters measured in a 24-hour urine analysis.
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Cortico-medullary differentiation (CMD) is considered lost when there is a loss of differentiation in kidneys larger than _____ cm.
Cortico-medullary differentiation (CMD) is considered lost when there is a loss of differentiation in kidneys larger than _____ cm.
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Match the following clinical findings with their descriptions:
Match the following clinical findings with their descriptions:
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Which crystal type is represented by a rhomboid shape?
Which crystal type is represented by a rhomboid shape?
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Calcium oxalate monohydrate crystals have a dumbbell shape.
Calcium oxalate monohydrate crystals have a dumbbell shape.
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What is the chemical composition of struvite crystals?
What is the chemical composition of struvite crystals?
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The shape of calcium phosphate crystals in alkaline urine is a ___.
The shape of calcium phosphate crystals in alkaline urine is a ___.
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Match the following types of crystals with their corresponding shapes:
Match the following types of crystals with their corresponding shapes:
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Which of the following is a screening test for renal artery stenosis?
Which of the following is a screening test for renal artery stenosis?
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Fibromuscular dysplasia is characterized by atherosclerosis in the renal arteries.
Fibromuscular dysplasia is characterized by atherosclerosis in the renal arteries.
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What is the characteristic waveform pattern associated with renal artery stenosis that is observed in Color Doppler Ultrasound?
What is the characteristic waveform pattern associated with renal artery stenosis that is observed in Color Doppler Ultrasound?
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A peak systolic velocity greater than _____ cm/s may suggest renal artery stenosis.
A peak systolic velocity greater than _____ cm/s may suggest renal artery stenosis.
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Match the following findings with their suggested implications regarding renal artery stenosis:
Match the following findings with their suggested implications regarding renal artery stenosis:
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Which type of cast is predominantly associated with glomerular hematuria?
Which type of cast is predominantly associated with glomerular hematuria?
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WBC casts are indicative of chronic kidney disease.
WBC casts are indicative of chronic kidney disease.
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What type of cast is characterized by muddy brown cells and primarily linked to Acute Tubular Necrosis?
What type of cast is characterized by muddy brown cells and primarily linked to Acute Tubular Necrosis?
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A __________ cast contains fat droplets and is often associated with nephrotic syndrome.
A __________ cast contains fat droplets and is often associated with nephrotic syndrome.
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Match the following casts with their associated clinical conditions:
Match the following casts with their associated clinical conditions:
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What is a common feature of microangiopathic hemolytic anemia (MAHA)?
What is a common feature of microangiopathic hemolytic anemia (MAHA)?
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Thrombocytopenia can result from the formation of platelet plugs in hemolytic uremic syndrome.
Thrombocytopenia can result from the formation of platelet plugs in hemolytic uremic syndrome.
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Name one toxin associated with typical hemolytic uremic syndrome.
Name one toxin associated with typical hemolytic uremic syndrome.
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A common blood investigation finding in hemolytic uremic syndrome is elevated ______ levels.
A common blood investigation finding in hemolytic uremic syndrome is elevated ______ levels.
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Match the following terms with their corresponding descriptions:
Match the following terms with their corresponding descriptions:
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Which of the following is NOT a cause of atypical hemolytic uremic syndrome?
Which of the following is NOT a cause of atypical hemolytic uremic syndrome?
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Low levels of C3 are a characteristic of atypical hemolytic uremic syndrome.
Low levels of C3 are a characteristic of atypical hemolytic uremic syndrome.
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What kind of blood smear finding would indicate mechanical hemolysis in a patient suspected to have hemolytic uremic syndrome?
What kind of blood smear finding would indicate mechanical hemolysis in a patient suspected to have hemolytic uremic syndrome?
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Which of the following casts is associated with normal conditions?
Which of the following casts is associated with normal conditions?
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Granular casts are typically present in kidney disease.
Granular casts are typically present in kidney disease.
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What condition is suggested by the presence of Maltese cross appearance in a urine sample?
What condition is suggested by the presence of Maltese cross appearance in a urine sample?
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The formation of casts in urine primarily occurs in the ______ convoluted tubule.
The formation of casts in urine primarily occurs in the ______ convoluted tubule.
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Match the following cast types with their clinical associations:
Match the following cast types with their clinical associations:
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Study Notes
Thrombotic Microangiopathy
- Thrombotic microangiopathy (TMA) is a condition that affects smaller vessels of the kidney.
- This can be caused by several conditions including Hemolytic uremic syndrome (HUS), HELLP syndrome, Catastrophic APS, and Diffuse Scleroderma.
- TMA can also be a rare cause of rapidly progressive renal failure (RPRF).
- Hemolytic Uremic Syndrome (HUS) is characterized by a triad of thrombocytopenia, renal failure, and microangiopathic hemolytic anemia (MAHA).
- TTP (Thrombotic Thrombocytopenic Purpura) is a distinct disease characterized by thrombocytopenia, fever, GI symptoms, neurological symptoms (brain), and MAHA.
- Typical HUS (D⁺) is commonly seen in childhood and has a faster onset, while the atypical form (D⁻) is more common in adults and has a slower onset.
- Atypical HUS is often caused by genetic factors affecting the complement pathway.
Basic Approach to Kidney Disease & Renal Artery Stenosis
- Rule out hydroureteronephrosis before diagnosing CKD.
- Kidney disease can present with different timeframes, from hours to days for cases like acute kidney injury or postrenal causes, to days to weeks for chronic kidney disease or thrombotic microangiopathy.
- Hypertensive crisis of vascular origin can be due to renal artery stenosis (RAS), which is large vessel kidney disease.
- The most common cause of rapidly progressive renal failure (RPRF) is rapidly progressive glomerulonephritis (RPGN), which is diagnosed with a biopsy.
- The initial investigation for kidney disease includes ultrasound, renal function tests, and 24-hour urine analysis.
- Ultrasound is a critical tool to assess kidney size and cortico-medullary differentiation (CMD).
Renal Artery Stenosis
- Renal artery stenosis (RAS), also known as renovascular hypertension (RVH), or renal artery occlusion disease (RAOD), involves narrowing of the renal artery.
- RAS can progress to CKD or ischemic nephropathy.
- The most common cause of RAS in young people is Fibromuscular dysplasia, while Atherosclerosis is more common in older individuals, particularly in Indian and Asian populations.
- Polyarteritis nodosa is a common cause of RAS in young males leading to microaneurysm formation in the renal artery.
- Unilateral RAS is explained by the Goldblatt model (
Two kidney one clip model
), which causes asymmetric kidney size due to reduced blood flow to the affected kidney. - Unilateral RAS leads to decreased renal blood flow, increased renal interstitial hydrostatic pressure, and activation of the Renin-Angiotensin system.
- The increased Renin-Angiotensin system leads to increased aldosterone and angiotensin II, causing vasoconstriction and resulting in hypertension.
- The pressure natriuresis theory explains how the kidneys regulate sodium and water balance.
- Diagnosis of RAS involves USG to detect asymmetric kidney and Doppler ultrasound as a screening tool.
- Confirmatory investigations include CT Renal angio or MR angiography.
- Treatment for RAS involves ACE inhibitors or ARBs (DOC) and percutaneous transluminal renal angioplasty (PTRA) if the kidney size is >8cm.
- Bilateral RAS has a poor prognosis, leading to decreased renal blood flow, increased Renin-angiotensin system activity, and loss of pressure natriuresis.
- Treatment for bilateral RAS focuses on managing blood pressure with diuretics as first-line therapy. ACE inhibitors and ARBs are contraindicated due to their effect on GFR.
Pathophysiology of a Disease (Hemolytic Uremic Syndrome)
- This document describes the pathophysiology and investigation of a disease likely to be Hemolytic Uremic Syndrome (HUS).
- Endothelial damage leads to the release of von Willebrand factor (vWF), which forms platelet plugs and traps platelets, causing thrombocytopenia.
- Circulating platelet plugs damage erythrocytes, leading to the formation of schistocytes, a characteristic sign of microangiopathic hemolytic anemia (MAHA).
Causes of Hemolytic Uremic Syndrome (HUS)
- Typical HUS (childhood) is usually caused by toxins like Shiga-like toxin from EHEC, particularly E.coli O157:H7. Shiga toxin from Shigella dysentery and neuraminidase toxin from Streptococcus pneumonia can also cause HUS.
- Atypical HUS (adults) is largely attributed to genetic factors, specifically the alternate complement pathway activation, leading to low C3 and normal C4 levels.
- HUS can be drug-induced with various medications, including mitomycin, gemcitabine, bevacizumab, calcineurin inhibitors, ticlopidine, clopidogrel, and cisplatin.
- Some immune-mediated responses can also trigger HUS, including quinine, gemcitabine, quetiapine, and cotrimoxazole.
Investigation of Hemolytic Uremic Syndrome (HUS)
- Peripheral blood smear is used to look for schistocytes.
- Blood investigations include elevated LDH, negative Coombs test, elevated indirect bilirubin, decreased haptoglobin levels, and normal PT/APTT.
- HUS is largely divided into typical (childhood), atypical (adult), and sporadic forms.
- The complement pathways involved differ between typical and atypical HUS: the alternative pathway is involved in atypical HUS, while the classical pathway is involved in systemic lupus erythematosus.
Renal Artery Stenosis - Diagnosis and Treatment
- This document explores the diagnosis and treatment of renal artery stenosis.
- Pre-op and post-op images highlight the changes in blood flow before and after angioplasty.
Diagnostic Workup for Renal Artery Stenosis
- Color Doppler Ultrasound (CDU) is used as a screening test to look for renal artery stenosis, identifying parvus tardus pattern and abnormal Resistivity Index (RI).
- Additional investigations include peak systolic velocity, renal artery resistance, and the change in RI.
- CT/MR renal angiography and conventional angiography are considered more conclusive and invasive investigations.
Fibromuscular Dysplasia
- Fibromuscular dysplasia is a non-atherosclerotic and non-inflammatory arteriopathy that commonly affects young females.
- It involves affecting the tunica media of the renal artery.
- It often affects both renal arteries, as well as cerebral vessels.
- The "string of beads" feature in angiograms is a characteristic sign of fibromuscular dysplasia.
- Treatment with percutaneous transluminal renal angioplasty (PTRA) yields a good prognosis
Crystals in Urine
- The document describes various types of crystals in urine, highlighting their shapes and chemical compositions.
- The crystals include uric acid, calcium oxalate (dihydrate and monohydrate), calcium phosphate, struvite, cholesterol, Amoxicillin, and hexagonal cystine.
Casts In Nephrology
- The document explains different types of casts found in urine samples, linking them to potential clinical associations and diseases.
- Cast types include pigment fine granular, RBC, WBC, tubular epithelial (muddy brown), broad, waxy, fatty, eosinophil, and fractured casts.
- Each cast type is associated with specific clinical conditions. For example, a pigment fine granular cast may indicate pigment nephropathy, hemolysis, or rhabdomyolysis.
Microscopic Examination
- RBCs are smaller than WBCs and epithelial cells.
- Oval fat bodies have a Maltese cross appearance under polarized light.
- Hyaline casts are often seen in normal urine but can also indicate kidney disease.
- Coarse and fine granular casts indicate kidney disease.
Polarizing Microscope
- The Maltese cross appearance under polarized light in blood indicates Babesiosis, while in kidney, it suggests Fabry's disease or ADPKD.
Casts in Urine
- Casts are formed in the distal convoluted tubule (DCT).
Urine Analysis
- Urine analysis is an important part of the diagnostic process, with findings like cell size, cell morphology, and presence of casts contributing to the diagnosis.
- The document includes a section on urine analysis, highlighting key aspects like date and time, active space, phase contrast microscopy, cell morphology, and the presence of oval fat bodies.
Conclusion
- The text provides valuable information on various kidney diseases focusing on their pathophysiology, diagnosis, and treatment.
- This information is crucial for healthcare professionals in understanding and managing these conditions. It focuses on the unique features of each disease and the role of various investigations in their diagnosis.
- The document also highlights the importance of microscopic examination of urine samples, including the identification and interpretation of casts and crystals, which are essential tools for diagnosing and monitoring kidney diseases.
- Additionally, the document emphasizes the role of imaging techniques such as ultrasound, CT scans, and angiography in assessing kidney function and blood flow, aiding in the diagnosis and treatment of renal artery stenosis.
- The information provided in the text serves as a comprehensive resource for understanding the complex interplay of various factors in kidney disease and how they can be effectively managed and treated.
- The document emphasizes the importance of a multi-disciplinary approach to managing kidney diseases, involving pathologists, nephrologists, radiologists, and other specialists.
- The use of various medical imaging techniques and laboratory investigations is essential for accurate diagnosis and personalized treatment strategies for patients with kidney diseases.
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Test your knowledge on renal diseases, including Hemolytic Uremic Syndrome and renal artery stenosis. This quiz encompasses causes, symptoms, treatments, and diagnostic procedures related to kidney health. Perfect for students and healthcare professionals alike!