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62 Cards in this Set

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Idiopathic Nephrotic Syndrome
-90% are idiopathic
Minimal change nephrotic syndrome – 85% (Greater than 95% of these respond to steroids)
Mesangial proliferation – 3%
Focal segmental glomerulosclerosis – 10
Idiopathic Nephrotic Syndrome
Clinical findings:
Male:female ratio – 2:1
MC between ages 2-6 yrs
20%-30% of adolescents have MCNS
Initial episode and subsequent relapses occur after minor infections and sometimes after insect bites, bee stings, or poison ivy
Mild edema, initially peri-orbital and LE’s
Gradually becomes generalized with subsequent ascites, pleural effusion, and genital edema
Anorexia, irritability, abdominal pain, and diarrhea also occur
Idiopathic Nephrotic Syndrome
diagnosis
3-4 plus proteinuria
Microscopic hematuria in 20% of children
Protein/creatinine ratio greater than 2.0
Serum creatinine usually nl
Serum albumin level usually < 2.5 g/dL. Serum cholesterol and triglycerides elevated. C3 and C4 levels nl
Renal biopsy usually not necessary but considered if child is less than one yr of age or greater than 8 yrs
Idiopathic Nephrotic Syndrome
TX
-Oral diuretics
-Hospitalize for severe edema
Sodium restriction
Restrict fluids for hyponatremia
Chlorothiazide (10 mg/kg/dose IV every 12 hrs, followed by furosemide 30 min later (1-2 mg/kg/dose IV every 12 hrs)
25% human albumin (0.5g/kg/dose) every 6-12 hrs over 1-2 hrs, followed by furosemide (1-2 mg/kg/dose IV)
-For presumed MCNS
Prednisone (be sure PPD test is negative) at 60 mg/m2/day divided into 2-3 doses for 6 wks; 80% respond by 2 wks
After initial 6 wk course, taper to 40 mg/m2 in the am every other day, then slowly taper and discontinue over the next 2-3 mo; if proteinuria still present (2 plus or greater) after 8 wks of tx, patient is steroid resistant, and biopsy is indicated
Relapse rates 30-40% with the 6 wk course of tx; treat with the 60 mg/m2/day dose until remission, then to alternate day dosing and taper over 1-3 mo
Idiopathic Nephrotic Syndrome
Complications:
Spontaneous bacterial peritonitis most frequent infection
Sepsis
Pneumonia, cellulitis
UTI’s
Strep pneumoniae most common pathogen with peritonitis, but may also see E. coli
At risk for thromboembolic events
Idiopathic Nephrotic Syndrome
vaccinations
All should get polyvalent pneumococcal vaccine
Varicella vaccine when varicella titer is negative
Should get influenza vaccine once yearly
Idiopathic Nephrotic Syndrome
Prognosis
Good in children who respond rapidly to steroids
TBW in
fetus
after birth
1st year
Puberty
Total body water (TBW) varies with age

Fetus – very high
After birth – 75% water by weight
1st year – TBW decreases to 60% (stable into puberty)
Puberty – females have increased fat stores, TBW decreases to 50%, males remain about 60%
Extracellular Fluid (ECF)
At birth
1yr to Puberty
At birth ECF>ICF
1 yr to puberty
-ECF 20-25%
-ICF 30-40%
ECF Cation difference maintained by
cell permeability
ICF Cation difference maintained by
ion pumps
Control of water balance depends on
Osmolality
Control of volume status depends on
sodium balance
Plasma Osmolality
normal value
285-295 osm/kg
Plasma osmolality controlled by :
Modification of water intake and excretion
Increased osm >>> thirst
Increased osm >>> increased ADH >>> decreased water loss
Sodium found mostly in
ECF
Na balance controlled by
kidney
Kidney regulates Na by
altering the percentage of sodium resorbed along the nephron
RA system
Decreased volume >> renin cleavage of
Decreased volume >> renin cleavage of angiotensinogen to angiotensin 1
Angiotensin 1 changed to angiotensin 2 by ACE
Angiotensin 2 leads to increased sodium resorption and increased aldosterone
Atrial Natriuretic Peptide
Produced by the cardiac atrium in response to increased volume
Leads to increased glomerular filtration rate and decreased sodium resorption
Maintenance Fluids
For children who cannot be fed
Not for dehydration or ongoing losses
Children are very tolerant to variation in fluid intake
Recommendations for maintenance therapy are guidelines, not absolutes

Composed of water, sodium, chloride, potassium, glucose
Maintenance Fluids
What it does?
Replace electrolytes in urine and stool
Replace fluid lost in urine, stool, skin, lungs
Typically provides about 20% of needed calories
Maintenance Fluids
what it doesn't do:
Supply adequate calories, fat, protein, vitamins, minerals
Kids lose 0.5-1% of weight daily on maintenance therapy
Make up a deficit in any of these areas
Sensible
Water loss
Urine 60%
Stool 5%
Emesis ?
Insensible Water loss
Skin and lungs 35%
Electrolyte Needs
Sodium
2-3 mEq/kg/day
Potassium
1-2 mEq/kg/day
Glucose
Always 5% in maintenance fluid
Fluid Needs
Water need per 24 hours
First 10 kilos = 100cc/kg
Second 10 kilos (11-20 kg) = 1000cc + 50cc/kg
Above 20 kilos = 1500cc + 20cc/kg

or
4
2
1
Fluid Content
Normal saline
144 mEq sodium per liter
Maintenance therapy
Patient weighs 15 kilos
His maintenance fluid would be…
1000 cc/24 hours for 1st ten kilos
Then 50 cc/24 hours next 5 kilos

1000 + 250=1250 / 24 = 52 cc/hour
The Dehydrated Child
normal or near normal exam
mild
infant: 5%
child: 3%
The Dehydrated Child
increased heart rate
decreased urine output
moderate
infant: 10%
child: 6%
The Dehydrated Child
sleepy, very ill appearing
severe:
infant: 15%
child: 9%
Lab Evaluation
BUN
Creatinine
Urine Specific Gravity
Serum sodium
Serum bicarbonate
Water deficit calculation
= % dehydration x weight in kg
Na deficit calculation
water deficit x 80 mEq/liter
K deficit calculation
water deficit x 30 mEq/liter
Which Deficiet calculation is the most useful?
water
Approach to Dehydration
Goals
Maintain adequate tissue perfusion
Replenish losses
Maintain steady state
Rehydrate children in 2 phases
Resuscitation phase
Rehydration phase
Resuscitation Phase
May not be necessary in mild cases
Always accomplished with isotonic fluid
20 ml/kg over 20 minutes of normal saline or lactated ringers
Assess the patient for clinical improvement when completed
Repeat until general clinical improvement occurs
Rehydration Phase
Next 24+ hours
Give maintenance fluid + remainder of deficit
Add total amount of water and electrolytes together (subtract boluses) then choose the most appropriate fluid
Give ½ over the first 8 hours
Give the last half over next 16 hours
Remember to make allowances for ongoing losses
Usually done with D5 ½ normal saline with 20mEq Potassium Chloride per liter
Isonatremic dehydration
Sodium and water lost at similar rate
ECF >ICF
Hyponatremic dehydration
Caused by diarrhea or ingestion of hypotonic liquid
Hypernatremic dehydration
Caused by decreased intake
Fluid moves from ICF to ECF to protect IVV
Lethargy and irritability
Over-rapid correction can lead to cerebral edema, herniation, death
Oral Rehydration
Pedialyte, WHO rehydration solution
Glucose 20% + electrolytes + water
Mild dehydration
Give 50 ml/kg over 4 hours
Severe dehydration
100 ml/kg over 4 hours
Give 10 ml/kg for each stool

May be given via naso-gastric tube
Parenteral Nutrition
Partial parenteral nutrition (PPN)
Total parenteral nutrition (TPN)
Necessary when enteral feeds are inadequate to meet nutritional needs
Less physiologic
More expensive
More complications
Indications for Parenteral Nutrition
Short term
Severe illness

Long term
Short gut syndrome
Venous access for Parenteral Nutrition
-Peripheral IV
Frequent failures
Phlebitis
Must be 12.5% dextrose or less

-Central venous line
PICC most common, also broviac or femoral line
Allows much higher levels of glucose infusion
Parenteral Feed Composition
Glucose
Amino acids
Electrolytes
Minerals
Vitamins
Essential fatty acids
Iron
Trace elements

Fats may be divided into a separate solution or combined as one
High doses/rates of glucose infusion must be tapered up and down at start and end to prevent severe glucose changes
Parenteral Feeding Complications
Central venous line
Insertion difficult in children
Correct placement
Thrombosis
Catheter related sepsis (coag negative staph)
Cholestatic liver disease
Can lead to cirrhosis and liver failure over time
Hyponatremia
Na < 132
Pseudohyponatremia
Lab artifact secondary to high proteins and lipids
Hyperosmolality
After infusion of medication – i.e. mannitol
Hypovolemic hyponatremia
Body water content normal, body sodium low
Renal – sodium in urine
Nonrenal – no sodium in urine
Euvolemic hyponatremia
Body water increased, body sodium low
SIADH or diluted formula
Hypervolemic hyponatremia
Body water increased, body sodium increased
Most commonly seen in renal failure
As sodium decreases, ECF becomes ______ and water moves ICF, can lead to CNS dysfuction
relatively less osmolar
Hyponatremia Therapy
Most common complication
is seizures
Hyponatremia Therapy
Complications
Over-rapid correction can lead to central pontine myelinolysis
After Na >120, prefer rate of increase 1 mEq every 2 hours
Hypernatremia due to
Excessive sodium intake
Water deficit
Water and sodium deficit
clinical findings with Hypernatremia
Doughy skin
Fever
CNS problems
Brain hemorrhage as water moves out of cells
Hypernatremia Therapy
Boluses of normal saline until patient clinially improved
Water defecit is severe, but so is sodium defecit
Once clinically stable
Goal is to lower serum Na by 1 meq every 2 hours
Frequent sodium checks

These are the most severely dehydrated patients and the most complex to care for
Mild edema, initially peri-orbital and LE’s
Gradually becomes generalized with subsequent ascites, pleural effusion, and genital edema
Anorexia, irritability, abdominal pain, and diarrhea also occur
Idiopathic Nephrotic Syndrome
Doughy skin
Fever
CNS problems
Brain hemorrhage as water moves out of cells
Hypernatremia