Nephrotic Disorders

Question 1

Hemolytic-Uremic syndrome

Answer 1

• combo of hemolytic anemia and thrombocytopenia that leads to acute renal failure
• occurs most often in kids aged 6M-5Y
• watery diarrhea progresses to bloody diarrhea, then to hemolytic anemia and thrombocytopenia

Question 2

HUS etiology

Answer 2

• bacterial toxins, chemicals, viruses
• diarrhea (if last for few weeks, think HUS)
• idiopathic
• inherited
• drug related
• malignancies

Question 3

HUS patho

Answer 3

• epithelial wall; microthrombi in the glomerular arterioles lead to occlusion of the glomerular capillary loops and glumerulosclerosis, resulting in renal failure
• RBCs and platelets are damaged as they move thru partially occluded BVs
• hemolytic anemia occurs as spleen tries to remove
• damaged platelets=thrombocytopenia

Question 4

Where does infection come from in HUS?

Answer 4

• most from verotoxin-producing strain of E. Coli
• may also be streptococcus pneumonia or Shigella dysenteries
• undercooked ground beef accounts for most cases; also consuming animal fevers, unpasteurized dairy and fruit products, unwashed fresh veg

Question 5

HUS s/s

Answer 5

• V/D
• marked pallor
• oliguria or anuria
• edema
• fatigue
• inc BP
• ab pain or tenderness
• neuro chx—irritability, altered LOC, sx, posturing, coma

Question 6

HUS lab findings

Answer 6

Urinalysis
- positive for blood, protein, pus, Casts
Serum
- elevated BUN and creatinine (indicated renal failure)
- mod to severe anemia
- mild to severe thrombocytopenia
- leukocytosis with left shift (inc WBCs and infection with more immature neutrophils)
- hyponatremia
- hyperkalemia
- Hyperphosphatemia

Question 7

HUS complications

Answer 7

• chronic renal failure
• sz and coma
• pancreatitis
• intussuscpetion
• rectal prolapse
• cardiomyopathy
• congestive heart failure
• acute resp distress sx

Question 8

HUS therapeutic management

Answer 8

• maintain fluid balance
• correct HTN, acidosis, electrolyte abnormalities
• replenish circ RBCs
• provide dialysis if needed

Question 9

HUS NC

Answer 9

• contact precautions
• close attn to fluid volume status
• may be on dialysis again
• family support—not dx quickly; can be hard to deal with the unknown
• encourage adequate nutrition w/i dietary restrictions (fluid restriction)
• monitor for bleeding
• teach preventative strategies—cook meat to 155, wash fresh produce, don’t swim with diaper, hand wash after petting animals

Question 10

Nephrotic syndrome patho

Answer 10

Result of inc glomerular basement membrane permeability which allows abnormal loss of protein in urine
- Massive protein lost in urine which causes low protein in blood so swelling occurs bc fluid leaks into tissue since there is not enough protein to attract it into the blood (hypovolemia)
- low proteins trigger body to increase hepatic synthesis of proteins and lipids (Hyperlipidemia)
- hypovolemia leads to vasoconstriction due to renin release from dec renal blood flow due to hypovolemia

Question 11

Types of nephrotic syndrome

Answer 11

Idiopathic aka minimal change
- most cases
- onset ages 1-8Y
Secondary
- from glomerulonephritis, systemic lupus erthematosus, Henoch-Schonlein purpura, DM
Congenital
- rare, autosomal recessive
- death in first year w/o transplant

Question 12

Nephrosis CM

Answer 12

Massive proteinuria, hypoproteinemia, Hyperlipidemia, edema (sudden wt gain), pleural effusion, Dec urine output, diarrhea, anorexia

Question 13

Nephrosis dx

Answer 13

• urinalysis
• serum—hematocrit and hemoglobin normal, BUN, creatinine
• renal biopsy—gives info about glomerular status and type of nephrotic syndrome (performed based on urinalysis and serum results after abx are tried and failed)

Question 14

Nephrosis goals of therapy

Answer 14

• Dec protein excretion
• Dec tissue fluid retention
• prevent infx and other complications like anemia, infx, poor growth, peritonitis, thrombosis, renal failure

Question 15

Nephrosis management

Answer 15

• activity: bed rest during edema than unrestricted during remission
• diet: during edema, NO ADDED SALT and high protein, may give albumin; regular during remission
• drugs: corticosteroids, immunosuppressant therapy, loop diuretics, salt poor, albumin (helps with edema and low BP)

Question 16

Nephrosis NC

Answer 16

• fluid volume excess r/t accumulation of fluid in tissues and third spaces
• make sure give meds safely
• potential intravascular fluid volume deficit r/t protein and fluid loss, sodium changes
• potential for infx r/t Dec resistance, steroids, fluid overload
• watch skin b/d from edema and lowered defenses
• no live immunizations
• altered nutrition bc loss of appetite and protein, give small, frequent snacks
• body image disturbances
• fatigued—activity tolerance
• knowledge deficit—watch for infx, etc

Question 17

Acute glomerulonephritis

Answer 17

• condition in which immune processes injure the glomeruli
• can range from minimal to severe
• antibodies get trapped, causing inflammation and changing renal tissue and leading to leaky kidneys from damaged glomeruli
• acute post-streptococcal glomerulonephritis (APSG) most common

Question 18

Glomerulonephritis patho

Answer 18

• damage to kidneys from trapped antibody complexes in the glomeruli, which causes inflammation and damaged renal tissue
• leads to leaky kidneys and proteins leak from bloodstream into urine through damaged kidneys
• causes excessive protein loss

Question 19

APSG CM

Answer 19

• fever (if infx is active—recent strep infx)
• lethargy fatigue, malaise, weak
• HA
• anorexia or vom
• facial edema
• urine—often brown, cloudy, tea colored, slightly dec
• edema—wt gain (can last couple weeks)
• pallor
• flank or ab pain
• HTN and s/s circ overload
• normal serum electrolytes

Question 20

APSG CM

Answer 20

• urinalysis—gross heat Uria, mild proteinuria, inc specific gravity
• negative urine culture
• serum—normal electrolytes (watch K), elevated ASO titer, BUN, creatinine and sed. Rate (ESR), RBC may be low

Question 21

APSG therapeutic management

Answer 21

• activity—bed rest during acute phase (lasts 1-2W); often self-limit
• diet—no added salt, low protein (if BUN elevated)
• control HTN
• abx if evidence of current strep infx (fever)
• iso from other kids that have infx bc vulnerable
• kids show they are improving when have inc in urine output
• can recover at home if BP and urine output are ok
• often recover spontaneously

Question 22

APSG NC

Answer 22

• Fluid volume excess—intravascular, r/t Dec glomerular filtration rate
• DW, accurate I&O, monitor hematuria, BP, electrolytes, diuretics, cardiopulmonary congestion
• infection prevention—keep from other kids
• watch for renal failure, sz precautions, encephalopathy, neuro evaluation
• teach parents on s/s, urine monitoring, neuro monitor

Question 23

Difference btwn glomerulnephrits and nephrotic syndrome

Answer 23

• G involved lodged antibodies; nephrotic sx not a disease, just sx collection
• nephrotic sx get steroids, G doesn’t

Question 24

Acute renal failure (ARF)

Answer 24

• sudden, often reversible decline in renal rxn that results in accum of metabolic toxins as well as fluid and electrolyte imbalance

Question 25

ARF etiologies

Answer 25

- pre-renal—poor renal perfusion from dehydration, surgical shock, burns - intrinsic—diseases, nephrotic drugs (ex: vancomycin—need to keep hydrated and monitor peak and trough) - post-renal—obstruction (tumor, obstructive uropthy)

Question 26

ARF patho

Answer 26

- Sudden dec in glomerular filtration rate—> elevated BUN—>significant reduction in renal blood flow - reversible ARF: low output phase—>high output phase—> gradual return to normal - kids improve by peeing more

Question 27

ARF CM

Answer 27

- n/v/d - oliguria=UO<1cc/kg/hr - edema - HTN - drowsy, lethargy - circ congestion - cardiac arrhythmias

Question 28

ARF complications

Answer 28

Hyponatremia, hyperkalemia (most immediate threat to life), HTN (freq and serious), anemia, sz, cardiac failure

Question 29

ARF management

Answer 29

- prevention - treat underlying cause - manage fluid and electrolyte disturbances - Dec BP - provide supportive therapy - drugs—lasix or mannitol, albumin

Question 30

ARF NC

Answer 30

- get peak and trough on drugs we know can lead to ARF like vancomycin - stay extra hydrated - seizure precautions - monitor VS and I&O - regulate fluid intake - nutrition—encourage snacking, may need low protein, K, Na diets - monitor for complications - expect significant diuresis when urine is restored

Question 31

Chronic renal failure

Answer 31

Dec in renal rxn that occurs over time in response to irreversible damage to the nephrons causing metabolic, biochemical, and clinical disturbances—slow gradual process - kidneys work until about 50% fxnal capacity is restored - causes: nephrotic sx, prolonged UT obstruction, polycystic kidney disease

Question 32

CRF patho

Answer 32

- glomerular death or hypertrophy—accumulation of various biochemical substances in blood - retention of waste products, water sodium - hyperkalemia - metabolic acidosis - hypocalcemia and Hyperphosphatemia - anemia (shorter life of RBCs) - growth probs from altered electrolytes and nutrition probs—osteodystrophy (bone b/d)

Question 33

CRF CM

Answer 33

- early signs are nonspecific and subtle - loss of normal energy - Dec appetite - HTN - HA, muscle cramp, nausea - wt loss or growth retardation - urine output inc or Dec - facial edema - dental defects - sallow skin - bone or joint pain - untreated uremic sx that can lead to confusion, lethargy, seizure, etc

Question 34

CRF treatment

Answer 34

- supportive therapy—restrict fluid, give meds, fix electrolytes - dialysis—can support for long time (try peritoneal dialysis first at home/at night, hemodialysis next—more intensive (ex/W) but less risk of infx) - transplant—need one eventually; gives better QoL, living related donor best then cadaver donor

Question 35

CRF diet

Answer 35

- Dec excretory demands on kidney - minimize metabolic bone disease - minimize F&E disturbances - maintain sufficient cals for growth - need adequate protein (but eat foods high in bio value) - vitamins—water soluble and vit D - need extra calcium and calcium carbonate - need aluminum hydroxide get—binds to phosphorous to flush out - restrict phosphorous in diet

Question 36

CRF NC

Answer 36

- watch for metabolic acidosis - encourage feeding - good dental care - may need erythropoietin for anemia - infection prevention - will be immunosuppressed - rejection possible (inc BP, Dec output) - F&E imbalance—diet edu and good nutrition - body image disturbance (may be small) - activity unrestricted - fleet enema CI bc can inc phosphorous! - fam support