Kidney Diseases and Treatments

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Questions and Answers

What is a common complication associated with adult polycystic kidney disease?

Liver fibrosis

Salt wasting

Chronic renal failure (correct)

Bilateral renal agenesis

Which of the following features differentiates childhood polycystic kidney disease from adult polycystic kidney disease?

Enlarged, cystic kidneys at birth (correct)

Cystic degeneration in end-stage kidney disease

Berry aneurysms

Large multicystic kidneys

What type of kidney stones are characterized by being coffin-shaped and are associated with urinary tract infections?

Uric acid stones

Calcium oxalate stones

Cystine stones

Struvite stones (correct)

Which treatment is not typically used for calcium stones associated with hypocitraturia?

High-protein diet Signup and view all the answers

What distinguishes medullary sponge kidney from other cystic kidney diseases?

Medullary cysts visible on urography Signup and view all the answers

Which cystic kidney disease is primarily associated with a risk of renal failure starting in childhood?

Familial juvenile nephronophthisis Signup and view all the answers

What is the typical clinical feature that occurs with simple kidney cysts?

Microscopic hematuria Signup and view all the answers

Which of the following is NOT a typical outcome of acquired renal cystic disease?

Berry aneurysms Signup and view all the answers

What is a common clinical manifestation in patients with renovascular hypertension?

Decreased circadian blood pressure variation Signup and view all the answers

Which of the following is a defining feature of fibromuscular dysplasia?

String of beads appearance on imaging Signup and view all the answers

What typically triggers secondary aldosteronism in renovascular hypertension?

Renin release from ischemic renal tissue Signup and view all the answers

When is surgical intervention indicated in a patient with atherosclerotic renovascular disease?

In progressive disease with significant comorbidities Signup and view all the answers

What symptom can be associated with renal vein thrombosis?

Testicular swelling Signup and view all the answers

Which type of kidney disease is Denys-Drash syndrome most commonly associated with?

Nephroblastoma Signup and view all the answers

What clinical feature is least likely associated with renal infarction?

Increased cardiac output Signup and view all the answers

Which condition is characterized by impairment of blood flow to the kidney leading to transient renovascular hypertension?

Renal vein thrombosis Signup and view all the answers

Which clinical sign indicates potential target organ damage due to renovascular hypertension?

Renal injury with hypokalemia Signup and view all the answers

What is the primary initial management approach for a patient with stable kidney function but suspected renovascular disease?

Optimizing medical therapy Signup and view all the answers

Which benign neoplasm of the kidney arises from perivascular epithelioid cells?

Angiomyolipoma Signup and view all the answers

What is a characteristic feature of clear cell renal cell carcinoma?

High intracellular lipid concentration Signup and view all the answers

What genetic syndrome is associated with an increased risk of various tumors and cysts in the kidneys?

Von Hippel-Lindau Syndrome Signup and view all the answers

Which type of renal cell carcinoma is associated with hypodiploidy and has an excellent prognosis?

Chromophobe RCC Signup and view all the answers

Which hereditary syndrome is characterized by a deletion of the WT1 gene and associated with Wilms tumor?

WAGR syndrome Signup and view all the answers

What type of renal cell carcinoma is represented by tumors with 'finger-like' projections?

Papillary RCC Signup and view all the answers

Which malignant neoplasm of the kidney is associated with fumarate hydratase gene mutations?

Hereditary Leiomyomatosis & Renal Cancer Syndrome Signup and view all the answers

What is a common characteristic of oncocytomas in the kidney?

Mahogany-brown tumor appearance Signup and view all the answers

Which neoplasm has a prognosis that varies depending on tumor stage?

Clear cell renal cell carcinoma Signup and view all the answers

Which type of renal cancer is known for having an aggressive form and poor prognosis?

Type 2 papillary RCC Signup and view all the answers

Study Notes

MRA

Endovascular stent treatment for atherosclerosis is indicated when there is bilateral disease, high-grade stenosis, progressive disease, and working kidney function.
If any of the previous conditions are absent, blood pressure and creatinine levels should be monitored, and re-imaging should be performed to assess for restenosis.
The goal of treatment is to stabilize kidney function and achieve excellent blood pressure control.

Cystic Diseases of the Kidney

Adult polycystic kidney disease is an autosomal dominant condition characterized by large multicystic kidneys, liver cysts, and berry aneurysms.
Adult polycystic kidney disease commonly presents with hematuria, flank pain, urinary tract infection, renal stones, and hypertension.
Chronic renal failure typically begins between the ages of 40 and 60.
Childhood polycystic kidney disease is an autosomal recessive condition characterized by enlarged, cystic kidneys at birth.
Childhood polycystic kidney disease can lead to liver fibrosis and variable outcomes, including death in infancy or childhood.
Medullary sponge kidney is characterized by medullary cysts on urography and is typically benign, presenting with hematuria, UTI, and recurrent renal stones.
Familial juvenile nephronophthisis is characterized by corticomedullary cysts, shrunken kidneys, and progressive renal failure beginning in childhood.
Multicystic renal dysplasia is characterized by irregular kidneys with cysts of variable size and can lead to renal failure if bilateral. Unilateral multicystic renal dysplasia is surgically curable.
Acquired renal cystic disease develops in end-stage kidney disease and is characterized by cystic degeneration.
Simple cysts are single or multiple cysts in normal-sized kidneys and can be microscopic or present as hematuria.
A.ARPKD (microcysts)
B.ADPKD (macrocysts)
C.Nephronophthisis
D.Distortion Renal calyces (Bardet-Biedl Syndrome)
E.Glomerulocystic kidney disease
F.Cystic Dysplastic Kidneys
G.Multicystic dysplastic kidneys
H.Medullary Sponge kidney

Urolithiasis

Calcium stones are the most common type of kidney stone, accounting for 80% of all cases.
Calcium oxalate stones are more common than calcium phosphate stones.
Calcium stones can be caused by ethylene glycol ingestion, vitamin C overuse, and hypocitraturia.
Treatment for calcium stones includes thiazides, citrate, and a low-sodium diet.
Struvite stones account for 15% of kidney stones and are caused by infection with urease-producing organisms.
Struvite stones are radiopaque and appear coffin-shaped on urine crystal analysis

Neoplasms of the Kidney

Benign Neoplasms

Angiomyolipoma is a benign tumor composed of vessels, muscle, and fat tissue and is associated with tuberous sclerosis.
Angiomyolipoma can cause hematuria and retroperitoneal bleeding if greater than 4 cm.
Oncocytoma is a benign tumor that originates from intercalated tubular cells of the collecting duct.
Oncocytoma is characterized by mitochondria-rich eosinophilic cells.
Oncocytoma can transform into renal cell carcinoma (RCC).

Malignant Neoplasms

Von Hippel-Lindau Syndrome is characterized by an increase in tumors and cysts throughout the kidneys.
Von Hippel-Lindau Syndrome is associated with mutations in the VHL gene on chromosome 3.
Hereditary Leomyiomatosis and Renal Cancer Syndrome is characterized by aggressive papillary carcinoma with metastatic potential.
Hereditary Leomyiomatosis and Renal Cancer Syndrome is associated with FH gene mutations, which encode fumarate hydratase.
Hereditary Papillary Carcinoma is a malignant tumor characterized by multiple bilateral tumors with papillary histology.
Hereditary Papillary Carcinoma is associated with mutations in the MET protooncogene.
Birt-Hogg-Dube Syndrome is associated with mutations in the BHD gene, which encodes folliculin.
Birt-Hogg-Dube Syndrome is characterized by skin, lung, and kidney tumors.

RCC Classification

Clear cell renal cell carcinoma (CCRCC) is the most common type of RCC, accounting for 70% of cases.
CCRCC is characterized by clear cells and polygonal cells arranged in cords or tubules.
CCRCC has a variable prognosis depending on the tumor stage.
Papillary renal cell carcinoma is the second most common type of RCC, accounting for 10-15% of cases.
Papillary renal cell carcinoma is classified into two types, with type 1 having a better prognosis than type 2.
Chromophobe renal cell carcinoma is characterized by large polygonal cells with a prominent cell membrane and eosinophilic cytoplasm.
Chromophobe renal cell carcinoma has an excellent prognosis.
Oncocytic renal cell carcinoma originates from oncocytomas and is similar to chromophobic RCC without a perinuclear halo.
Collecting duct carcinoma is a rare type of RCC that is associated with sickle cell disease.
Collecting duct carcinoma has a poor prognosis.

Nephroblastoma (Wilms Tumor)

Nephroblastoma is the most common malignant kidney tumor in children.
Nephroblastoma is associated with specific syndromes:
- WAGR syndrome: Deletion of the 11p13 band leads to the deletion of the WT1 gene and other genes, such as PAX6.

Vascular Diseases of the Kidney

Renovascular Diseases

Reduction or loss of renal arterial or venous blood flow is associated with several clinical syndromes.
Activation of the renin-angiotensin system in response to reduced renal perfusion pressure raises systemic arterial pressure to restore renal perfusion pressure.
Renovascular hypertension (RVH) is a syndrome of elevated blood pressure caused by any condition that leads to reduced perfusion of the kidneys.

Clinical Features of Renovascular Hypertension

Increased activation of the renin-angiotensin system.
Early-onset or late-onset hypertension.
Elevated sympathetic nervous system activation.
Abnormal circadian rhythm characterized by a lack of nocturnal blood pressure decline.
Secondary aldosteronism and hypokalemia.
Accelerated target organ damage, including microvascular disease, left ventricular hypertrophy, and renal injury.
Hyponatremic hypertensive syndrome, unstable cardiac syndromes, and nephrotic range proteinuria.

Clinical Presentations of Renovascular Disease

Renovacular hypertension
Ischemic renal disease
Unstable cardiac syndromes
Renal infarction
Atheroembolic renal disease
Renal vein thrombosis
Transplant renovascular disease

Fibromuscular Dysplasia

Fibromuscular dysplasia is a non-inflammatory, non-atherosclerotic arteriopathy caused by smooth muscle cell proliferation and fibrosis.
Fibromuscular dysplasia is the most common cause of RVH in children and young adults.
Fibromuscular dysplasia typically affects the middle to distal renal artery or its branches.
Most common variant: Medial Fibroplasia
Medial Fibroplasia is characterized by alternating thin and thick collagen deposits resulting in “string of beads” appearance on imaging.
Renal infarction is an impairment of blood flow to the kidney
Renal infarction is characterized by flank, loin, or abdominal pain with vomiting, microhematuria, and proteinuria.

Renal Vein Thrombosis

Renal vein thrombosis is characterized by loin, testicular, or flank pain, testicular swelling, fever, leukocytosis, oliguric AKI, nausea, vomiting, hematuria, and proteinuria.
Renal vein thrombosis is associated with renal edema and swelling.
Renal vein thrombosis can lead to an acute pulmonary embolus.

Evaluation and Management of Atherosclerotic Renovascular Disease

Clinical syndrome suggestive of renovascular disease.
Stable kidney function and blood pressure control?
- Yes: Optimize medical therapy: RAAS blockade with ACEI/ARB, statin therapy, smoking cessation, aspirin, follow-up imaging to monitor progression.
- No:
  - Progressive disease, total renal mass, comorbid risk, life expectancy, working kidney function?
    - Yes: Surgery for complex disease, failed stent, or aortic disease.
    - No:: Noninvasive imaging, such as duplex ultrasound or CTA.

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Description

This quiz covers essential aspects of atherosclerosis treatment related to kidney function, focusing on the indications for endovascular stent treatment. Additionally, it explores cystic diseases of the kidney, including adult and childhood polycystic kidney disease, their symptoms, and progression. Test your knowledge on these crucial nephrology topics.