Polycystic Kidney Disease

Polycystic Kidney Disease
Definition
1. Hereditary disease characterized by cyst
formation and massive kidney enlargement
2. Adult form of disorder is autosomal dominant
polycystic kidney disease and accounts for 10% of
persons in End Stage Renal Disease (ESRD)
Pathophysiology
1. Renal cysts are fluid-filled sacs affecting
nephrons; cysts fill, enlarge, multiply thus
compressing and obstructing kidney tissue; renal
parenchyma atrophies, becomes fibrotic
2. Cysts occur elsewhere in body including liver,
spleen

Manifestations
1. Disease is slowly progressive; symptoms develop in age
30 40s
2. Common manifestations include
a. Flank pain
b. Microscopic or gross hematuria
c. Proteinuria
d. Polyuria and nocturia (impaired ability to concentrate
urine)
e. UTI and renal calculi are common
f. Hypertension from disrupted renal vessels
g. Kidneys become palpable, enlarged, knobby
h. Symptoms of renal insufficiency and chronic renal
failure by age of 50 60

Collaborative Care: Determine extent of
polycystic kidney disease
Diagnostic tests
1. Renal ultrasonography: primary choice for
diagnostic; assesses kidney size, identifies
and locates renal masses: cysts, tumors,
calculi
2. Intravenous pyelography (IVP): evaluate
structure and excretory function of kidneys,
ureters, bladder
3. CT scan of kidneys: detects and
differentiates renal masses

Management
1. Mainly supportive: prevent further renal
damage from UTI, nephrotoxic substances,
obstruction, hypertension
2. Fluid intake of 2000 2500 mL to prevent
UTI, calculi
3. Control of hypertension with ACE
inhibitors and other antihypertensive agents
4. Eventually require dialysis or
transplantation (typically good candidates)

Nursing Care and Nursing Diagnoses
1. Risk for Ineffective Coping: address
genetic counseling and screening for
family members
2. Excess Fluid Volume
3. Anticipatory Grieving
4. Knowledge Deficit: of measures to
preserve kidney function
Clients with Renal Failure

Definition
1. Condition in which kidneys are unable to remove
accumulated metabolites from blood; leads to altered
fluid, electrolyte and acid-base balance
2. May be due to kidney (primary disorder) or resulting
from another disease in another organ or systemic
(secondary disorder)
3. Classified as acute (abrupt onset and may be
reversible) or chronic (develops slowly and insidiously
with few symptoms until kidneys are severely damaged
and unable to meet bodys excretory needs)
4. Common and costly disease with people with End
Stage Renal Disease requiring dialysis or transplant to
live
5. 5 year survival rate for clients on dialysis is 31.3%

Acute Renal Failure (ARF)
1. Definition
a. Rapid decline in renal function with azotemia fluid
and electrolyte imbalances
b. High mortality rate but is related to clients being
seriously ill and aged
Risk Factors
a. Major surgery or trauma
b. Infection
c. Hemorrhage
d. Severe heart failure, liver disease
e. Lower urinary tract obstruction
f. Use of nephrotoxic contrast media and medications

Pathophysiology involved with cause categories
a. Prerenal
1. 55 60% cases of ARF
2. Cause: Conditions that affect renal blood flow and
perfusion
A .Decrease vascular volume
B .Decrease cardiac output
C .Decrease vascular resistance
b. Intrarenal
1. 35-40% cases of ARF
2. Cause: Acute damage to renal parenchyma and nephron
a. Acute glomerulonephritis
b. Vascular disorders including vasculitis, malignant
hypertension, arterial or venous occlusion

c. Acute Tubular Necrosis (ATN): Destruction of tubular
epithelial cell with abrupt decline in renal function from
1. Prolonged ischemia (>2 hours) as with surgery,
severe hypovolemia, sepsis, trauma, burns
2. Nephrotoxins
a. Aminoglycoside antibiotics
b. Radiologic contrast media
c. Other potential drugs: NSAIDs, heavy metals,
ethylene glycol (antifreeze)
3. Nephrotoxins have increased risk with clients with
preexisting renal insufficiency or state of dehydration
4. Rhabdomyolysis: excess myoglobin from skeletal
muscle injury clogs renal tubules (muscle trauma, drug
overdose, infection)
5. Hemolysis: red blood cell destruction

Postrenal
1. <5% cases of ARF
2. Cause: Obstructive; prevents urine
excretion
a. Benign prostatic hypertrophy
b. Renal or urinary tract calculi or tumors

Course and Manifestations of ARF in 3 phases
a. Initiation Phase
1. Lasts hours to day
2. Begins with initiating event ends when maintenance
phase begins
3. Good prognosis if treated at this phase
4. Few manifestations; identified when maintenance phase
begins
b. Maintenance Phase
1. Characterized by significant fall in GFR and tubular
necrosis
2. Oliguric or non-oliguric but kidneys not eliminating
wastes, water, electrolytes, acids: azotemia, fluid retention,
electrolyte imbalances (hyperkalemia, hypocalcemia,
hyperphosphatemia), acidosis (impaired hydrogen ion
elimination)
3. Anemia after several days due to suppressed
erythropoetin secretion; impaired immune function

4. Salt and water retention leading to hypertension
and risk for heart failure and pulmonary edema
5. Hyperkalemia: cardiac dysrhythmias and EKG
changes, muscle weakness, nausea, diarrhea
6. Confusion, disorientation, agitation or lethargy,
hyperreflexia, possible seizures, coma
7. Vomiting, decreased or absent bowel sounds
c. Recovery Phase
1. Progressive tubule cell repair and regeneration;
return of GFR to pre-ARF levels
2. Diuresis occurs as kidney recover but BUN,
Creatinine, potassium and phosphate remain high
3. Renal function improves rapidly first 5 25 days
but improvement may continue for up to a year

Collaborative Care
a. Prevention of ARF is goal for all clients, especially those
at high-risk
1. Preserve kidney perfusion by adequate vascular
volume, cardiac output and blood pressure
2. Limiting use of nephrotoxic medications or using
minimal effective dose, maintaining hydration,
monitoring renal function tests
b. Treatment goals
1. Identify and correct underlying cause
2. Prevent additional renal damage
3. Restore urine output and kidney function
4. Compensate for impaired renal function: maintain
fluid and electrolyte balance

Diagnostic tests to identify ARF
a. Urinalysis
1. Fixed specific gravity 1.010 (low)
2. Proteinuria, if glomerular damage
3. Presence of red blood cells (glomerular dysfunction),
white blood cells (inflammation), renal tubule epithelial cells
(ATN)
4. Cell casts (protein and cellular debris molded in shape
of tubular lumen); brown color may indicate hemoglobinuria
or myoglobinuria
b. Serum BUN and creatinine
1. Creatinine rises rapidly (24 48 hours) and peaks in 5
10 days; rise is slower if output maintained
2. Halt in rise of BUN and Creatinine signals onset of
recovery

Serum Electrolytes
1. Monitored to determine whether to initiate dialysis
2. Moderate rise in potassium
3. Hyponatremia related to water excess
d. CBC showed moderate anemia and low hematocrit (Iron
and folate may be low and add to anemia)
e. Renal ultrasound: used to identify any obstruction,
identify acute from chronic renal failure
f. CT scan: identify obstruction and kidney size
g. IVP, retrograde pyelography, or antegrade pyelography
1. Assess renal structure and function
2. Retrograde and antegrade testing less toxicity from
contrast media
h. Renal biopsy: determine cause, differentiate acute from
chronic

Medications
a. Intravenous fluids and blood volume expanders
to restore renal perfusion
b. Low dose Dopamine (Intropin) intravenous
infusion to increase renal blood flow and improve
cardiac output
c. Diuretic: Furosemide (Lasix) or osmotic diuretic
such as mannitol along with intravenous fluids;
washes out nephrons; prevents oliguria reducing
azotemia and electrolyte imbalance
d. Antihypertensive medications including ACE
inhibitors to limit renal injury

Medications to prevent possible complications
1. Prevention of gastrointestinal bleeding (at risk due
to stress, impaired platelet function)
a. Antacids
b. H2 receptor antagonists
c. Proton-pump inhibitors
2. Hyperkalemia: serum K > 6.5 mEq/L puts client at
risk for cardiac arrest
a. Calcium chlorides
b. Bicarbonate
c. Insulin and glucose

d.Sodium polystyrene sulfonate (Kayexalete)
1. Removes potassium from body primarily
in large intestine
2. If given orally, is combined with sorbitol
3. May be given as retention enema with tap
water enema to follow after 30 60 minutes
3. Hyperphosphatemia
a. Aluminum hydroxide (AlternaGEL,
Amphojel, Nephrox)
b. Binds with phosphates in GI tract and is
eliminated from bowel

Fluid Management
a. Once vascular volume and renal
perfusion restored, fluids are restricted
b. Often intake is calculated by adding
output from previous 24 hours and 500
ml for insensible losses
c. Fluid balance monitored by daily
weights and serum Na level

Dietary Management
a. Renal insufficiency and underlying disease
creates increased rate of catabolism (breakdown of
body proteins) and decreased rate of anabolism
(tissue repair)
b. Client needs adequate nutrition and calories to
prevent catabolism but protein intake needs to be
limited to minimize azotemia
c. Protein limited to 0.6g/kg body weight per day;
protein should be of high biologic value (contains
essential amino acids)
d. Carbohydrate intake is increased for adequate
calories and protein-sparing effect

Dialysis
a. Dialysis is the diffusion of solute
molecules across semipermeable
membrane from area of higher solute
concentration to lower concentration
b. Dialysis used to remove excess fluid,
waste products from client with renal
failure; can rapidly remove nephrotoxins
from blood
A hemodialysis system
Continuous arteriovenous hemofiltration

(CAVH)
An arteriovenous fistula
An arteriovenous shunt in the forearm
Options for long-term vascular access for hemodialysis
Peritoneal dialysis
Manual peritoneal dialysis via an implanted abdominal catheter

(Tenckhoff catheter)

Hemodialysis: Dialysis process
a. In this type of dialysis, blood is taken from client
via vascular access and pumped into a dialyzer;
blood is separated from the dialysate (dialysis
solution) by semipermeable membrane
b. Processes of diffusion and ultrafiltration remove
waste products, electrolytes, excess water
c. Glucose, electrolytes, water can pass through,
but larger molecules (protein, red blood cells) are
blocked
d. Substances can be added to dialysate to diffuse
into the blood of the client
e. Client with ARF may undergo hemodialysis daily
initially, then 3 4 times/week according to client
condition; 3 4 hours at a time

Complications associated with hemodialysis
a. Hypotension, most common, related to
changes in osmolality, rapid removal from
vascular department, vasodilation
b. Bleeding related to platelet function and
use of heparin during dialysis
c. Infection, local or systemic;
Staphylococcus aureus septicemia
associated with infected vascular access
site; higher rates of hepatitis B and C,
cytomegalovirus, HIV in hemodialysis clients

13.
Continuous Renal Replacement Therapy (CRRT)
a. Technique used, which allows more gradual fluid
and solute removal than hemodialysis; used for
clients with ARF unable to tolerate hemodialysis
b. Done over period of 12 hours or more
14.
Vascular Access for Hemodialysis
a. Acute or temporary access is gained inserting
double lumen catheter into subclavian, jugular, or
femerol vein
b. Blood is drawn from proximal portion of catheter
and returned to circulation through distal end of
catheter

Arteriovenous (AV) fistula created for longer term access for
dialysis
1. Surgical anastomosis of artery and vein in non-dominant
arm, usually radial artery and cephalic vein
2. Usually cannot use fistula for hemodialysis access for a
month while it matures
3. Nurse or client can assess functional fistula for
complications
a. Thrombosis (clotted off): check for palpable thrill,
audible bruit
b. Infection: check for redness, drainage
4. Venipunctures and blood pressures should not be done in
arm with the AV fistula
5. AV fistulas are commonly used for vascular access for
dialysis clients with chronic renal failure

Peritoneal dialysis process involves:
a. Peritoneal membrane of client is used as dialyzing
surface
b. Warmed sterile dialysate instilled into peritoneal
cavity through a catheter that has been inserted into
peritoneal cavity
c. Metabolic waster products and excessive
electrolytes diffuse into dialysate while it remains in
abdomen
d. Water diffusion is controlled by glucose in the
dialysate which acts as an osmotic agent
e. Fluid is drained off by gravity into sterile bag at
set intervals, thus removing waste products and
excess fluid

Disadvantages of peritoneal dialysis
a. Dialysis is more gradual and may be slow for
ARF
b. Risk of peritonitis
c. Contraindicated for clients with abdominal
surgery, peritonitis, significant lung disease
Health Promotion: Prevention of ARF
a. Maintenance of fluid volume and cardiac output
b. Reduce risk of exposure to nephrotoxins
c. Report output < 30 ml per hour in clients at risk
d. Report dehydration, monitor renal function tests
in clients receiving nephrotoxic medications

Nursing Diagnoses for clients in ARF
a. Excess Fluid Volume
b. Imbalanced Nutrition: Less than body
requirements
c. Deficient Knowledge
Home care: Client who is recovering from
ARF will need teaching for prescribed diet
and fluid intake, avoidance of nephrotoxins,
prevention of infection, continue under
medical supervision

Client with Chronic Renal Failure (CRF)
Definition
a. Progressive renal tissue destruction and loss of
function
b. May progress over many years without being
recognized until kidneys are unable to excrete
metabolic wastes and regulate fluid and electrolytes:
End-stage Renal Disease (ESRD)
c. Incidence is increasing especially in older adults;
higher in African Americans, Native Americans
d. Conditions causing chronic renal failure diffuse
bilateral disease of kidneys with progressive
destruction and scarring; diabetes is leading cause of
ESRD; then hypertension

Pathophysiology and Manifestations of Stages
a. Decreased Renal Reserve: Early Stage
1. Unaffected nephrons compensate for lost
nephrons
2. GFR is about 50% of normal
3. Client is asymptomatic
4. BUN and serum creatinine are normal
b. Renal Insufficiency
1. GRF falls to 20 50% of normal
2. Azotemia and some manifestations
3. Insult to kidneys could precipitate onset renal
failure (infection, dehydration, exposure to
nephrotoxins, urinary tract obstructions)

c. Renal failure
1. GRF < 20% of normal
2. BUN and serum creatinine rise sharply
3. Oliguria, manifestations of uremia
d.End-stage renal disease (ESRD)
1. GRF < 5 % of normal
2. Renal replacement therapy necessary to
sustain life

ESRD: Uremia (urine in blood)
a. Early manifestations
1. Nausea, apathy, weakness, fatigue
2. Progresses to frequent vomiting, increasing
weakness, lethargy, confusion
b. Fluid and electrolyte effects
1. Urine less concentrated with proteinuria and
hematuria
2. Sodium and water retention
3. Hyperkalemia (Muscle weakness, paresthesia, EKG
changes)
4. Hyperphosphatemia, hypocalcemia, hypermagesemia
5. Metabolic acidosis

Cardiovascular effects
1. Systemic hypertension
2. Edema and heart failure; pulmonary edema
3. Pericarditis: metabolic toxins irritate pericardial
sac; less often now with dialysis
4. Cardiac tamponade: fluid in pericardial sac
Hematologic effects
1. Anemia contributing to fatigue, weakness,
depression, impaired cognition, impaired cardiac
function
2. Impaired platelet function
Immune system effects
1. WBC declines
2. Humoral and cell-mediated immunity impaired
3. Fever suppressed

Gastrointestinal effects
1. Anorexia, nausea, vomiting, hiccups
2. GI ulcerations, increased risk for GI bleeding
3. Uremic fetor: urinelike breath odor
Neurologic effects
1. Changes in mentation, poor concentration
2. Fatigue, insomnia
3. Psychotic symptoms, seizures, coma
4. Peripheral neuropathy: restless leg syndrome,
sensations of crawling, prickling
5. Muscle weakness, decreased deep tendon
reflexes, gait disturbances

Musculoskeletal effects
1. Renal osteodystrophy (renal rickets) characterized by
osteomalacia (bone softening) and osteoporosis
2. Bone tenderness and pain
Endocrine and metabolic effects
1. Elevated uric acid levels; risk for gout
2. Resistance to insulin, glucose intolerance
3. High triglyceride and < HDL levels resulting in accelerated
atherosclerotic process
4. Menstrual irregularities; reduced testosterone levels
Dermatologic effects
1. Yellowish hue to skin
2. Dry skin with poor turgor
3. Pruritis due to metabolic wastes deposited in skin
4. Uremic frost crystallized deposits of urea on skin

Collaborative Care
a. Eliminate factors that further
decrease renal function
b. Maintenance of nutritional status
with minimal toxic waste products
c. Identify and treat complications of
CRF
d. Preparation for dialysis or renal
transplantation

Diagnostic Tests: Identify CRF and monitor renal function by
following levels of metabolic wastes and electrolytes
a. Urinalysis: fixed specific gravity at 1.010; excess protein,
blood cells, cellular casts
b. Urine culture: identify infection
c. BUN and serum creatinine: evaluate kidney function
1. BUN levels
a. Mild azotemia: 20 50 mg/dL
b. Severe renal impairment: > 100 mg/dL
c. Uremic symptoms: > 200mg/dL
2. Creatinine levels >4 mg/dL indicate serious renal impairment
d. Creatinine Clearance: evaluates GFR and renal function
1. Decreased renal reserve: 32.5 130 mL/min
2. Renal insufficiency: 10 30 mL/min
3. ESRD: 5 10 mL/min

e. Serum electrolytes: monitored
throughout course of CRF
f. CBC: moderately severe anemia with
hematocrit 20 30%; low hemoglobin;
reduced RBCs and platelets
g. Renal ultrasonography: CRF: decreased
kidney size
h. Kidney biopsy: diagnose underlying
disease process; differentiate acute from
chronic

Medications
a. General effects of CRF on medication effects
1.
Increased half-life and plasma levels of meds excreted by
kidneys
2.
Decreased drug absorption if phosphate-binding agents
administered concurrently
3.
Low plasma protein levels can lead to toxicity when proteinbound drugs are given
4.
Avoid nephrotoxic meds or give with extreme caution
b. Diuretics (furosemide, other loop diuretics)
1.
Reduce edema
2.
Reduce blood pressure
3.
Lower potassium
c. Antihypertensive medications: ACE inhibitors preferred
d. Sodium bicarbonate or calcium carbonate correct mild acidosis
e. Oral phosphorus binding agents (calcium carbonate, calcium acetate)
to lower phosphate levels and normalize calcium levels
f. Aluminum hydroxide for acute treatment of hyperphosphatemia

g. Vitamin D supplements to improve calcium absorption
h. To treat dangerously high potassium levels
1. Intravenous bicarbonate, insulin, glucose
2. Sodium polystyrene sulfonate (Kayexalate)
i. Folic acid, iron supplements to combat anemia
j. Multiple vitamin supplement
Dietary and Fluid Management
a. Early in course of CRF: diet modifications to slow kidney
failure, uremic symptoms, and complications
b. Restrict proteins (40 gm/day) of high biologic value
c. Increase carbohydrate intake (35kcal/kg/day)
d. Limit fluid to 1 2 L per day; limit sodium to 2 g/day
e. Restrict potassium (60 -70 mEq/day); no salt substitutes
f. Restrict phosphorus foods (meat, eggs, dairy products)

Renal Replacement Therapies: considered when medications
and dietary modifications are no longer effective
a. Hemodialysis: establish vascular access (create AV
fistula) months ahead
b. Peritoneal dialysis: can be initiated when indicated;
training client and/or family involved
c. Transplantation: tissue typing and identification of
living related potential donors including health assessment
of donor
Dialysis
a. Considerations
1. Dialysis manages ESRD, but does not cure it
2. Hemodialysis or peritoneal dialysis is constant factor
of life
3. Depending on individual client situation and total
health, client may prefer death to dialysis

Hemodialysis for ESRD
1. Treatments are 3 times per week for 9 12 hours
2. Specific dialysis orders according to body size,
residual renal function (based on that days current
lab test results), dietary intake, concurrent illnesses
3. Complications during treatment are hypotension
and muscle cramps; dialysis disequilibrium
syndrome
4. Long term complications are infection and
vascular access problems
5. Cardiovascular disease is leading cause of death
for hemodialysis clients; higher death rate than
clients on peritoneal dialysis or transplanted

Peritoneal Dialysis for ESRD
1. Continuous ambulatory peritoneal dialysis (CAPD) most
common
2. 2 liters of dialysate instilled into peritoneal cavity and
catheter sealed; empty and replace every 4 6 hours
3. Continuous cyclic peritoneal dialysis (CCPD) uses
delivery device during nighttime hours and continuous dwell
during day
Advantages over hemodialysis
a. Eliminates vascular access and heparinization
b. Avoids rapid fluctuation in extracellular fluid
c. Diet intake is more liberal with fluids and nutrients
d. Regular insulin can be added to dialysate to manage
hyperglycemia for diabetics
e. Client more able to self-manage

Disadvantages of peritoneal dialysis
a. Less effective metabolite elimination
b. Risk for infection (peritonitis:
dialysate returns cloudy; should be
straw colored)
c. Serum triglyceride levels increase
d. Altered body image with peritoneal
catheter

Kidney Transplant
a. Background
1. Treatment of choice for ESRD
2. Primarily limited by availability of kidneys
3. Many persons on waiting list for kidney
4. Improves survival and quality of life for ESRD client
b. Organ Donors
1. Majority are from cadavers
2. Transplants from living donors increasing
3. Close match between blood and tissue type desired; HLA
are compared; 6 in common is perfect match
4. Living donors must be in good physical health;
nephrectomy is major surgery and remaining kidney must be
healthy
Placement of a transplanted kidney.
Placement of a transplanted kidney in the right iliac fossa

Cadaver donors
1. Cadaver kidney from persons who
a. Meets criteria for brain death
b. Are aged < 65 years old
c. Are free of systemic disease, malignancy, or
infection including HIV, hepatitis B, C
2. Kidney removed and preserved by hypothermia
a. Transplant in 24 48 hours
b. Use technique: continuous hypothermic
pulsatile perfusion, and transplant up to 3 days
3. Donor kidney placed in lower abdominal cavity, renal
artery, vein, and ureter are anastomosed

Immunosuppressive therapy
1. Necessary to block immune response that would
reject transplanted organ
2. Medications include
a. Glucocorticoids: prednisone and
methylprednisolone used for maintenance and
treatment of acute rejection episodes
b. Azathioprine: inhibits cellular and humoral
immunity; metabolized by liver
c. Mycophenolate mofetil: more potent and minimal
bone marrow suppression
d. Cyclosporine: affects cellular immunity; is
hepatotoxic and nephrotoxic
1.
2.
Rejection
Can occur at any time
Acute rejection
a.
Occurs within months of transplant
b. Cellular immune response with T cells
c.
Few manifestations
1. Rise in serum creatinine
2. Possibly oliguria
d.
Treatment
1. Methylprednisolone
2. OKT3 monoclonal antibody
3.
Chronic rejection
a.
Develops months to years post transplant
b. Major cause of graft loss
c.
Involves both humoral and cellular immune response
d. Manifestations (same as renal failure)
1. Progressive azotemia
2. Proteinuria
3. Hypertension

Complications of kidney transplant
1. Hypertension
2. Glomerular lesions with manifestations of
nephrosis
3. Increased risk for myocardial infarction and
stroke
Complications associated with long-term
immunosuppression
1. Infection: bacterial, viral, fungal in blood, lung,
CNS
2. Tumors: carcinoma in situ in cervix, lymphomas,
skin cancers
3. Steroid use leads to bone problems, peptic ulcer
disease, cataract formation

Health Promotion
a. Ensure all clients with impaired renal function
are well hydrated, especially while receiving
nephrotoxic drugs
b. Encourage clients with ESRD to explore
transplant options
Nursing Diagnoses
a. Impaired Tissue Perfusion: renal
b. Imbalanced Nutrition: Less than body
requirements
c. Risk for Infection
d. Disturbed Body Image

Home Care
a. CRF and ESRD are long-term processes
requiring client management
b.Extensive teaching required
1. Monitoring health status
2. Compliance with fluid and dietary
restriction and medications
3. Care involved with hemodialysis,
peritoneal dialysis, or living with transplant

Polycystic Kidney Disease

Enviado por

Dados do documento

Título original

Direitos autorais

Formatos disponíveis

Compartilhar este documento

Compartilhar ou incorporar documento

Opções de compartilhamento

Você considera este documento útil?

Este conteúdo é inapropriado?

Direitos autorais:

Formatos disponíveis

Polycystic Kidney Disease

Enviado por

Direitos autorais:

Formatos disponíveis

Polycystic Kidney Disease