Renal- Polycystic Kidney Disease

A collection of vocabulary flashcards based on critical terminology and concepts related to the urinary system, urinary tract infections, and associated conditions.

Which statement regarding the inheritance pattern of Autosomal Dominant Polycystic Kidney Disease (ADPKD) is correct?

1. It requires both parents to carry the gene.

2. A child of an affected parent has a 50\% chance of inheriting the disease.

3. It only affects male offspring.

4. It skip generations frequently.

Answer: 2

Rationale: ADPKD is an autosomal dominant disorder, meaning only one copy of the mutated gene (from one parent) is needed to pass on the condition. Each child of an affected parent has a 50\% risk of inheritance.

In the pathophysiology of PKD, where do the fluid-filled cysts primarily originate?

1. The renal pelvis
2. The ureters
3. The nephrons
4. The bladder wall

Answer: 3

Rationale: In PKD, cysts develop within the nephron segments (the functional units of the kidney). As they grow, they detach from the nephron and continue to enlarge via fluid secretion.

What is the primary mechanism by which PKD leads to renal failure? (Select all that apply)

A. Compression of healthy tissue by expanding cysts
B. Localized ischemia due to blood vessel compression
C. Autoimmune destruction of the glomerulus
D. Replacement of functional parenchyma with non-functional cysts
E. Retrograde urine flow causing hydronephrosis

Answer: A, B, D

Rationale: As cysts enlarge, they compress adjacent healthy renal tissue and blood vessels, leading to ischemia and tissue death. Eventually, the functional kidney mass is replaced by cysts, resulting in renal failure. It is not an autoimmune process.

Which gene mutation is responsible for the more severe and rapidly progressing form of ADPKD?

1. PKD1
2. PKD2
3. PKD3
4. HLA-B27

Answer: 1

Rationale: Mutations in the PKD1 gene (located on chromosome 16) account for approximately 85\% of cases and typically lead to earlier onset and more rapid progression to end-stage renal disease (ESRD) compared to PKD2 mutations.

Autosomal Recessive Polycystic Kidney Disease (ARPKD) is most commonly diagnosed during which life stage?

1. Late adulthood
2. Puberty
3. Infancy or childhood
4. Elderly years

Answer: 3

Rationale: Unlike the 'adult' dominant form, ARPKD is rare and usually manifests in utero or during early childhood, often presenting with severe pulmonary hypoplasia and renal failure at birth.

What is often the first clinical manifestation of PKD?

1. Hypotension
2. Hypertension
3. Jaundice
4. Edema

Answer: 2

Rationale: Hypertension is typically the earliest sign of PKD, occurring in 60\% to 70\% of patients before any significant decline in glomerular filtration rate (GFR). It results from activation of the renin-angiotensin system due to cyst-induced ischemia.

A patient with PKD reports a sudden, sharp increase in flank pain. What does the nurse suspect? (Select all that apply)

A. Cyst rupture
B. Infection within a cyst
C. Renal calculi (kidney stones)
D. Development of diabetes
E. Hemorrhage into a cyst

Answer: A, B, C, E

Rationale: Sharp or sudden pain is usually associated with acute events like cyst rupture, infection, stone passage (which occurs more frequently in PKD due to urinary stasis), or bleeding into a cyst.

Why do patients with PKD often experience increased abdominal girth?

1. Ascites from liver failure
2. Massive enlargement of the kidneys by thousands of cysts
3. Excessive subcutaneous fat deposition
4. Fluid retention in the abdominal wall

Answer: 2

Rationale: In PKD, the kidneys can grow to be the size of footballs and weigh over 10 kilograms each. This massive organ enlargement causes the abdomen to distend and increases girth.

Which urinary finding is common when a PKD cyst ruptures or bleeds?

1. Glycosuria
2. Hematuria
3. Ketonuria
4. Pale, straw-colored urine

Answer: 2

Rationale: Hematuria (blood in the urine) is a classic finding occurring in nearly half of PKD patients. It usually indicates a cyst has ruptured into the renal pelvis.

A patient with PKD complains of constant 'dull' aching in the lower back. The nurse explains this is likely due to:

1. Heavy lifting at work
2. Stretching of the renal capsule by enlarging cysts
3. Referred pain from the gallbladder
4. Spinal stenosis

Answer: 2

Rationale: The steady, dull, aching pain common in PKD is generally caused by the physical pressure and stretching of the renal capsule or displacement of other abdominal organs by the massive kidneys.

Which gastrointestinal symptom is common in advanced PKD due to physical pressure?

1. Hyperactive bowel sounds
2. Constipation
3. Diarrhea
4. Increased appetite

Answer: 2

Rationale: As the kidneys enlarge, they take up significant space in the abdomen and can compress the colon, frequently leading to chronic constipation.

What is an early urinary sign of PKD indicating a loss of concentrating ability?

1. Anuria
2. Nocturia
3. Oliguria
4. Dysuria

Answer: 2

Rationale: One of the earliest signs of renal tubular damage in PKD is the inability to concentrate urine, leading to nocturia (frequent urination at night) and polyuria.

Which extra-renal complication of PKD is most life-threatening?

1. Liver cysts
2. Pancreatic cysts
3. Cerebral (Berry) aneurysms
4. Diverticulosis

Answer: 3

Rationale: Approximately 5\% to 10\% of patients with ADPKD have intracranial berry aneurysms. If these rupture, they cause a subarachnoid hemorrhage, which can be fatal.

Which clinical manifestations are associated with Polycystic Kidney Disease? (Select all that apply)

A. Flank pain
B. Palpable abdominal masses
C. Hypotension
D. Kidney stones
E. Urinary tract infections (UTIs)

Answer: A, B, D, E

Rationale: Hypertension, not hypotension, is expected. Masses, pain, stones (due to stasis), and infections (due to cyst involvement) are all standard clinical manifestations.

Extra-renal manifestations of ADPKD include which of the following? (Select all that apply)

A. Polycystic liver disease
B. Mitral valve prolapse
C. Colon diverticula
D. Optic neuritis
E. Seminal vesicle cysts

Answer: A, B, C, E

Rationale: PKD is a systemic disorder. Liver cysts are the most common extra-renal finding (70\%+). Cardiac valve issues, colonic diverticula, and cysts in the reproductive tract are also frequently seen.

What is the diagnostic test of choice for initial screening and confirmation of PKD?

1. Renal Biopsy
2. Ultrasonography
3. MRI with contrast
4. IV Pylogram

Answer: 2

Rationale: Renal ultrasound is the gold standard for screening and diagnosis because it is non-invasive, cost-effective, and highly sensitive at detecting cysts.

In what scenario might a clinician order a CT or MRI for a PKD patient instead of ultrasound?

1. To check the patient's blood type
2. When cysts are too small to be seen on ultrasound or to measure Total Kidney Volume (TKV)
3. To rule out diabetes
4. As a routine monthly screening

Answer: 2

Rationale: CT and MRI provide higher resolution and are used to detect tiny cysts or to precisely calculate Total Kidney Volume (TKV), which is a biomarker for predicting disease progression.

Which lab finding is most likely to be elevated in the late stages of PKD progression?

1. Serum Albumin
2. Hemoglobin
3. Serum Creatinine
4. Red Blood Cell Count

Answer: 3

Rationale: As functional nephrons are lost and GFR declines, serum creatinine and BUN levels rise, indicating the onset of Chronic Kidney Disease (CKD) and potential progression to ESRD.

Urinalysis findings in a patient with PKD and a secondary infection might include: (Select all that apply)

A. Nitrites
B. Proteinuria
C. Gross hematuria
D. Pyuria (WBCs in urine)
E. Large amounts of glucose

Answer: A, B, C, D

Rationale: Proteinuria occurs from glomerular damage; hematuria from cyst rupture; nitrites and pyuria indicate a concurrent urinary tract infection. Glucose is not a typical finding for PKD.

Which lifestyle modification is critical for a patient with PKD to slow disease progression?

1. High-protein diet
2. Effective blood pressure control
3. Sedentary lifestyle
4. Decreased oral fluid intake

Answer: 2

Rationale: Controlling hypertension (typically with ACE inhibitors or ARBs) is the single most important intervention to protect the kidneys from further cyst-induced damage.

What is the recommended daily fluid intake for a patient with PKD to help suppress cyst growth and prevent stones?

1. 500 mL to 1 L
2. 1 L to 1.5 L
3. 2 to 3 Liters
4. Fluids should be restricted strictly

Answer: 3

Rationale: High fluid intake suppresses vasopressin, which is thought to drive cyst growth. It also helps prevent renal calculi and UTIs.

Which class of medication is specifically approved to slow the growth of cysts in ADPKD by targeting the vasopressin receptor?

1. Loop diuretics
2. Vasopressin V2-receptor antagonists (Tolvaptan)
3. Beta-blockers
4. Calcium channel blockers

Answer: 2

Rationale: Tolvaptan (Jynarque) is a specific treatment for ADPKD that slows the increase in kidney volume and the decline in kidney function by blocking vasopressin stimulation of cyst growth.

What is a major nursing priority for a patient taking Tolvaptan (Jynarque)?

1. Monitoring for hyperglycemia
2. Assessing for signs of liver toxicity
3. Checking for hearing loss
4. Administering a high-sodium diet

Answer: 2

Rationale: Tolvaptan carries a risk of serious liver injury. Patients must have frequent liver function tests (LFTs) and report symptoms such as jaundice or dark urine.

Why are NSAIDs (like Ibuprofen) contraindicated for pain management in PKD?

1. They increase cyst growth
2. They are nephrotoxic and can worsen renal failure
3. They cause berry aneurysms
4. They lead to liver cysts

Answer: 2

Rationale: NSAIDs interfere with renal blood flow and are nephrotoxic. Acetaminophen is usually the preferred analgesic for patients with PKD.

Which surgical intervention may be used if a single large cyst is causing debilitating pain or infection?

1. Nephrectomy
2. Percutaneous cyst aspiration and sclerotherapy
3. Kidney transplant
4. Lithotripsy

Answer: 2

Rationale: For isolated cysts causing severe localized pain, percutaneous aspiration followed by sclerotherapy (using an agent to scar the cyst closed) may be performed.

When educating a patient with PKD about physical activity, the nurse should advise:

1. Engaging in tackle football for stress relief
2. Avoiding all exercise
3. Avoiding contact sports and heavy lifting
4. Training for a marathon immediately

Answer: 3

Rationale: Due to the high risk of cyst rupture and subsequent hemorrhage or infection, patients with enlarged kidneys should avoid heavy contact sports or activities that risk abdominal trauma.

A PKD patient asks why they must limit sodium intake. The nurse's best response is:

1. 'Sodium causes cysts to burst.'
2. 'Sodium creates kidney stones.'
3. 'Restricting sodium helps control your blood pressure.'
4. 'Sodium makes your kidneys turn blue.'

Answer: 3

Rationale: Sodium restriction is necessary because excessive sodium intake contributes to hypertension and fluid retention, both of which accelerate renal damage.

A nurse is teaching a patient with PKD about preventing constipation. Which should be included? (Select all that apply)

A. Increase dietary fiber intake
B. Maintain adequate hydration
C. Use a daily stimulant laxative
D. Establish a regular bowel routine
E. Decreased activity levels

Answer: A, B, D

Rationale: High fiber, fluids, and routine help manage the constipation caused by the physical pressure of the massive kidneys. Daily stimulant laxatives are not recommended for long-term use.

Which sign should a patient with PKD report to their healthcare provider immediately?

1. Frequent urination at night
2. Burning or pain during urination
3. Mild back ache
4. Stable blood pressure

Answer: 2

Rationale: Burning or pain (dysuria) indicates a urinary tract infection, which can quickly spread to the cysts (cystitis) or kidney tissue, causing rapid renal decline.

Dietary education for a patient with advancing renal failure (CKD stage 4) from PKD includes: (Select all that apply)

A. Phosphorus restriction
B. Potassium restriction (if levels are high)
C. Unlimited protein intake
D. Sodium restriction
E. High fat intake

Answer: A, B, D

Rationale: As kidney function declines, the kidneys can no longer excrete sodium, phosphorus, and potassium effectively. Protein may also be moderately restricted to reduce the workload on the kidneys.

What is the ultimate treatment for PKD once the kidneys progress to End-Stage Renal Disease (ESRD)?

1. Continued Tolvaptan therapy
2. Dialysis or Kidney Transplantation
3. High-dose antibiotics
4. Complete bed rest

Answer: 2

Rationale: There is no cure for PKD. Once GFR falls below 15\text{ mL/min}, dialysis or a kidney transplant becomes necessary.

Which valve abnormality is most frequently associated with ADPKD?

1. Aortic stenosis
2. Mitral valve prolapse
3. Tricuspid atresia
4. Pulmonary valve stenosis

Answer: 2

Rationale: Mitral valve prolapse is the most common cardiac manifestation in ADPKD patients, affecting approximately 25\% of cases.

A patient with PKD asks why they have foul-smelling, cloudy urine. The nurse explains this is likely:

1. A normal occurrence in PKD
2. Sign of a ruptured cyst
3. Sign of a urinary tract infection
4. Due to high protein diet

Answer: 3

Rationale: Cloudiness and foul odor are classic signs of infection. Infections in PKD must be treated aggressively as they can be difficult to clear once they involve the fluid inside cysts.

A patient with PKD is frustrated that their blood pressure medication was changed to an ACE inhibitor. The nurse explains:

1. 'It's cheaper than other meds.'
2. 'It works specifically to protect kidney function and reduce cyst pressure.'
3. 'It's only because your heart is failing.'
4. 'It makes your kidneys work harder.'

Answer: 2

Rationale: ACE inhibitors and ARBs are preferred for PKD because they specifically target the renin-angiotensin-aldosterone system (RAAS), which is overactive in PKD, thereby protecting the nephrons.

Which finding on a KUB (Kidney, Ureter, Bladder) X-ray would be suggestive of PKD?

1. Kidney size larger than the lumbar vertebrae span
2. Cysts appearing as dark circles
3. Kidney displacement and enlargement
4. Stones only

Answer: 3

Rationale: While KUB shows the massive size and displacement of kidneys, it cannot visualize fluid-filled cysts well. Ultrasound remains the superior tool for cyst detection.

The nurse teaches a PKD patient that 'Total Kidney Volume' is used by doctors to:

1. Decide which kidney to remove
2. Predict how fast the disease is progressing
3. Calculate the number of nephrons left
4. Check for liver involvement

Answer: 2

Rationale: Total Kidney Volume (TKV) is an important prognostic tool. A higher TKV or a rapid increase in TKV indicates a faster decline toward renal failure.

Which statement by the patient indicates a need for further education regarding PKD?

1. 'I need to monitor my blood pressure every day.'
2. 'My kids don't need to worry because I am the only one with it.'
3. 'I should drink lots of water to help my kidneys.'
4. 'I will call my doctor if I see blood in my pee.'

Answer: 2

Rationale: Because ADPKD is autosomal dominant, there is a 50\% chance the patient's children will inherit the gene. Screening and counseling for offspring are highly recommended.

What is the pathophysiological cause of 'berry' aneurysms in PKD patients?

1. High blood sugar
2. Weakness in the arterial wall of the brain's circle of Willis
3. Local infection in the brain
4. Trauma from falls

Answer: 2

Rationale: ADPKD is a generalized connective tissue disorder. A weakness in the muscular layer of the arterial walls predisposes patients to aneurysms, particularly in the brain.

A patient with PKD has a GFR of 30. Which dietary item should be most strictly monitored?

1. Simple sugars
2. Phosphorus (e.g., dairy, sodas)
3. Vitamin C
4. Fiber

Answer: 2

Rationale: As kidney function declines (Stage 3 CKD), the kidneys cannot excrete phosphorus, leading to bone disease. Dairy and processed foods high in phosphorus must be limited.

In a patient with PKD, if the urine appears reddish-brown or 'cola' colored, what does this suggest?

1. Dehydration
2. Old blood from a ruptured cyst
3. High intake of carrots
4. Presence of bilirubin

Answer: 2

Rationale: Red or brownish urine indicates hematuria. In PKD, this is often 'cola-colored' when blood has sat in the cyst or bladder for a period after rupture.

Which physical assessment finding corresponds with 'advanced' PKD progression?

1. Flat abdomen
2. Hypofrontality
3. Palpable, knobby kidneys on abdominal palpation
4. Decreased heart rate

Answer: 3

Rationale: As cysts grow, the kidneys become easily palpable and are often described as feeling 'knobby' or irregular due to the thousands of protruding cysts.

A patient with PKD is being evaluated for a kidney transplant. The patient asks if the cysts will come back in the new kidney. The nurse responds:

1. 'Yes, PKD always attacks new kidneys.'
2. 'No, the new kidney does not have the genetic mutation for PKD.'
3. 'Only if you eat too much protein.'
4. 'Maybe, it's a 50\% chance.'

Answer: 2

Rationale: PKD does not recur in the transplanted kidney because the donor organ does not contain the genetic defect that causes cyst formation.

How does Tolvaptan (Jynarque) affect urine output?

1. It significantly decreases urine output.
2. It has no effect on urine output.
3. It causes significant aquaresis (large volume of dilute urine).
4. It causes hematuria.

Answer: 3

Rationale: Tolvaptan is an aquaretic. It blocks vasopressin, leading to the excretion of large amounts of electrolyte-free water. Patients must stay hydrated to prevent dehydration.

Which of the following describes the 'Polydipsia-Polyuria' cycle in PKD patients on Tolvaptan?

1. Too much salt causes thirst and urination
2. Excessive water loss in urine causes intense thirst
3. Kidney failure causes low thirst
4. Cysts cause the patient to hate water

Answer: 2

Rationale: Tolvaptan causes massive water loss (polyuria), which leads to intense thirst (polydipsia) to compensate. This is an expected side effect of the medication.

Why is genetic counseling recommended for patients with PKD? (Select all that apply)

A. To discuss the risk for future children
B. To identify if siblings should be screened
C. To find a cure for the patient
D. To help the patient understand the inheritance pattern
E. To plan for potential financial impacts

Answer: A, B, D

Rationale: Genetic counseling focuses on inheritance risks for the family and educating on the genetic nature of the disease. It does not provide a cure for the patient.

What is the goal of 'supportive care' in PKD?

1. To remove all cysts surgically
2. To manage symptoms and prevent complications
3. To reverse the genetic mutation
4. To stop urine production

Answer: 2

Rationale: Supportive care aims to manage pain, control blood pressure, treat UTIs early, and maintain hydration to slow progression, as the underlying genetic disease cannot be reversed.

A patient with PKD has a sudden fever, chills, and flank pain. The nurse should prioritize:

1. Getting a heating pad for the back
2. Obtaining a urine culture and sensitivity
3. Giving the patient a high-protein snack
4. Encouraging a nap

Answer: 2

Rationale: Fever and chills with flank pain suggest an infection (UTI or cyst infection). Identifying the causative organism via culture is the priority for effective antibiotic treatment.

Which of the following is an associated risk with renal cyst aspiration?

1. Diabetes
2. Infection or recurrence of the cyst
3. Brain aneurysm
4. Weight gain

Answer: 2

Rationale: While aspiration can provide temporary relief, the risks include introducing infection into the kidney or the cyst simply refilling with fluid shortly after the procedure.

Patients with PKD have an increased risk for which type of abdominal protrusion?

1. Hiatal hernia
2. Inguinal and umbilical hernias
3. Gastric ulcers
4. Scoliosis

Answer: 2

Rationale: Increased intra-abdominal pressure from massive kidneys and potential generalized defects in the connective tissue make PKD patients more prone to hernias.

Which finding necessitates immediate cessation of Tolvaptan?

1. Thirst
2. Clear urine
3. Jaundice or significantly elevated liver enzymes
4. Increased urination at night

Answer: 3

Rationale: Jaundice and high liver enzymes indicate drug-induced liver injury, the most