21 terms

Polycystic kidney disease

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Overview
Growth of multiple, bilateral, grapelike clusters of fluid-filled cysts in the kidneys
May progress slowly even after symptoms of renal insufficiency appear
Adult form, which has an insidious onset but usually becomes obvious between ages 30 and 50, has autosomal dominant inheritance
Usually fatal within 4 years of uremic symptom onset, unless dialysis begins
Carries a widely varying prognosis in adults
Also known as PKD and autosomal dominant polycystic kidney disease (ADPKD)
Overview-Pathophysiology
Cysts enlarge the kidneys, leading to thickening of the basement membranes in the tubules, macrophage infiltration, neovascularization, fibrosis, and cystic dilation of the renal tubules, and ultimately resulting in the compression and eventual replacement of functioning renal tissue. (See Polycystic kidney.)
Renal deterioration results.
The condition progresses relentlessly to fatal uremia.
Overview-Causes
Autosomal dominant form inherited as an autosomal dominant trait (ADPKD), affecting the short arm of chromosome 16 and the long arm of chromosome 4
Acquired (more than one-half of patients receiving dialysis for more than 3 years)
Overview-Incidence
The disease affects both sexes equally, with a slight increase in severity of the disease in men.
Autosomal dominant form accounts for 6% to 10% of all cases of end-stage renal disease in the United States.
The usual age of onset for the autosomal form typically ranges from age 20 to 40.
Overview-Complications
Cyst rupture
Hepatic failure
Cerebral aneurysm, subarachnoid hemorrhage
Diverticula
Mitral valve prolapse
Metabolic abnormalities
Renal calculi
Renal failure
Respiratory failure
Heart failure
Recurrent hematuria
Life-threatening retroperitoneal bleeding
Assessment-History
Family history
Polyuria
Urinary tract infections
Headaches
Pain in back or flank area
Gross hematuria
Abdominal pain, usually worsened on exertion and eased by lying down
Assessment-Physical Findings
Hypertension
Microscopic or gross hematuria
Increased abdominal girth
Signs of an enlarging kidney mass
Grossly enlarged kidneys (in advanced stages)
Uremic fetor
Pallor
Diagnostic Test Results-Laboratory
Urinalysis may show hematuria, bacteriuria, or proteinuria.
Creatinine clearance test results may show renal insufficiency or failure.
Hematocrit may be elevated.
Serum albumin levels may be decreased.
Serum electrolyte levels may reveal hyponatremia, hyperkalemia, hyperphosphatemia, or hypocalcemia.
Diagnostic Test Results-Imaging
Excretory or retrograde urography reveals enlarged kidneys, with pelvic elongation, flattening of the calyces, and indentations caused by cysts.
Magnetic resonance imaging (preferred test) shows multiple areas of cystic damage.
Treatment-General
Monitoring of renal function
Dialysis; renal replacement therapy
Transcutaneous electrical nerve stimulation (TENS) and hypnotherapy for chronic pain
Treatment-Diet
Low-sodium
Fluid management based on degree of renal dysfunction
Treatment-Activity
As tolerated in early stages
Avoidance of contact sports with advanced disease
Exercise to manage hypertension
Treatment-Medications
Analgesics, such as opioids, transcutaneous opioid patches, or perinephric injection of local anesthetics, for pain relief
Antibiotics, such as ciprofloxacin hydrochloride, levofloxacin, or trimethoprim-sulfamethoxazole for urinary tract infection
Antihypertensive agents, such as angiotensin-converting enzyme (ACE) inhibitors (captopril, enalapril, or lisinopril) or angiotensin receptor blockers (losartan potassium, irbesartan, or candesartan cilexetil), for hypertension
Diuretics, such as furosemide, to treat hypertension and renal calculi
Electrolyte replacements, such as calcium carbonate, and phosphate binders to reduce phosphorus load in renal failure.
Treatment-Surgery
Kidney transplantation
Surgical drainage for cystic abscess or retroperitoneal bleeding
Nursing Considerations-Nursing Diagnoses
Acute pain
Chronic pain
Deficient fluid volume
Fatigue
Impaired urinary elimination
Ineffective coping
Risk for ineffective renal perfusion
Risk for infection
Risk for injury
Nursing Considerations-Expected Outcomes
report feelings of increased comfort
identify measures to address chronic pain
maintain fluid balance
verbalize the importance of balancing activity with adequate rest periods
demonstrate skill in managing the urinary elimination problem
demonstrate adaptive coping behaviors
exhibit adequate renal function
remain free from signs and symptoms of infection
avoid or minimize complications.
Nursing Considerations-Nursing Interventions
Give prescribed drugs, including ACE inhibitors to control hypertension; if giving diuretics, obtain specimens for serum electrolyte levels, especially potassium, which may be decreased.
Provide comfort measures, including opioid analgesics; assist the patient with relaxation techniques and the use of TENS.
Provide fluids and foods based on the patient's condition, encourage increased fluids if the patient has a urinary tract infection, and restrict fluids if the patient has renal failure.
Provide supportive care to minimize symptoms.
Obtain specimens for urinalysis and culture and sensitivity as ordered to evaluate for hematuria, proteinuria, and infection; obtain specimens for laboratory tests, such as electrolyte levels, as ordered.
Individualize patient care, as appropriate
Allow the patient to verbalize his feelings and concerns, especially related to possible progression of the disease and renal failure; provide support and guidance.
Prepare the patient for dialysis or renal replacement therapy as indicated.
Encourage the parents of a child with the infantile form to obtain genetic counseling.
Prepare the patient and his family for possible renal transplant or surgery.
Nursing Considerations-Monitoring
Renal function
Urinary elimination, including urine color and characteristics
Fluid balance and hydration
Electrolyte balance
Vital signs, especially blood pressure
Pain level and relief
Laboratory test results (urine and blood specimens)
Access site for dialysis if appropriate
Effectiveness of therapy and dialysis
Nursing Considerations-Associated Nursing Procedures
Blood pressure assessment
Clean-catch (midstream) urine collection, female
Clean-catch (midstream) urine collection, male
Continuous ambulatory peritoneal dialysis (CAPD)
Continuous renal replacement therapy (CRRT)
Health history interview and physical assessment
Hemodialysis, arteriovenous access
Hemodialysis, double-lumen catheter
Intake and output assessment
Nutritional screening
Oral drug administration
Postoperative care
Preoperative care
Preparing a patient for urologic surgery, OR
Pulse assessment
Venipuncture
Weight measurement
Patient Teaching-General
disorder, underlying cause, diagnosis, and treatment, including medication therapy and possible dialysis or transplantation
prescribed drug therapy, including the drug name(s), dosage(s), frequency of administration, and duration of therapy
possible adverse effects of therapy, such as hypokalemia with diuretic therapy and GI upset with ACE inhibitors
follow-up with the practitioner for severe or recurring headaches
signs and symptoms of urinary tract infection and renal calculi and the need to notify the practitioner immediately if any occur
signs and symptoms of electrolyte imbalances and the need to notify the practitioner if any occur
importance of blood pressure control and adhering to medication therapy
use of analgesics and possible sedative effects if opioid agents are used and how to use opioid patches if prescribed
how to use TENS or hypnotherapy for chronic pain control
that disorder is slowly progressive, and that dialysis or transplantation may be needed
dialysis procedure, technique, and frequency, if indicated, and care of the access site.
ongoing need for follow-up and laboratory testing to evaluate disease progress, including ultrasonography every 1 to 2 years.
Patient Teaching-Discharge Planning
Refer the patient and his family to community and social services for support.