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62 Cards in this Set
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Idiopathic Nephrotic Syndrome
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-90% are idiopathic
Minimal change nephrotic syndrome – 85% (Greater than 95% of these respond to steroids) Mesangial proliferation – 3% Focal segmental glomerulosclerosis – 10 |
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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 |
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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 |
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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 |
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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 |
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Idiopathic Nephrotic Syndrome
vaccinations |
All should get polyvalent pneumococcal vaccine
Varicella vaccine when varicella titer is negative Should get influenza vaccine once yearly |
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Idiopathic Nephrotic Syndrome
Prognosis |
Good in children who respond rapidly to steroids
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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% |
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Extracellular Fluid (ECF)
At birth 1yr to Puberty |
At birth ECF>ICF
1 yr to puberty -ECF 20-25% -ICF 30-40% |
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ECF Cation difference maintained by
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cell permeability
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ICF Cation difference maintained by
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ion pumps
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Control of water balance depends on
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Osmolality
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Control of volume status depends on
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sodium balance
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Plasma Osmolality
normal value |
285-295 osm/kg
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Plasma osmolality controlled by :
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Modification of water intake and excretion
Increased osm >>> thirst Increased osm >>> increased ADH >>> decreased water loss |
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Sodium found mostly in
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ECF
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Na balance controlled by
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kidney
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Kidney regulates Na by
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altering the percentage of sodium resorbed along the nephron
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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 |
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Atrial Natriuretic Peptide
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Produced by the cardiac atrium in response to increased volume
Leads to increased glomerular filtration rate and decreased sodium resorption |
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Maintenance Fluids
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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 |
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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 |
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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 |
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Sensible
Water loss |
Urine 60%
Stool 5% Emesis ? |
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Insensible Water loss
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Skin and lungs 35%
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Electrolyte Needs
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Sodium
2-3 mEq/kg/day Potassium 1-2 mEq/kg/day Glucose Always 5% in maintenance fluid |
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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 |
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Fluid Content
Normal saline |
144 mEq sodium per liter
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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 |
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The Dehydrated Child
normal or near normal exam |
mild
infant: 5% child: 3% |
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The Dehydrated Child
increased heart rate decreased urine output |
moderate
infant: 10% child: 6% |
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The Dehydrated Child
sleepy, very ill appearing |
severe:
infant: 15% child: 9% |
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Lab Evaluation
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BUN
Creatinine Urine Specific Gravity Serum sodium Serum bicarbonate |
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Water deficit calculation
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= % dehydration x weight in kg
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Na deficit calculation
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water deficit x 80 mEq/liter
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K deficit calculation
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water deficit x 30 mEq/liter
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Which Deficiet calculation is the most useful?
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water
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Approach to Dehydration
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Goals
Maintain adequate tissue perfusion Replenish losses Maintain steady state |
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Rehydrate children in 2 phases
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Resuscitation phase
Rehydration phase |
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Resuscitation Phase
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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 |
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Rehydration Phase
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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 |
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Isonatremic dehydration
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Sodium and water lost at similar rate
ECF >ICF |
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Hyponatremic dehydration
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Caused by diarrhea or ingestion of hypotonic liquid
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Hypernatremic dehydration
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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 |
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Oral Rehydration
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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 |
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Parenteral Nutrition
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Partial parenteral nutrition (PPN)
Total parenteral nutrition (TPN) Necessary when enteral feeds are inadequate to meet nutritional needs Less physiologic More expensive More complications |
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Indications for Parenteral Nutrition
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Short term
Severe illness Long term Short gut syndrome |
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Venous access for Parenteral Nutrition
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-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 |
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Parenteral Feed Composition
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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 |
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Parenteral Feeding Complications
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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 |
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Hyponatremia
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Na < 132
Pseudohyponatremia Lab artifact secondary to high proteins and lipids Hyperosmolality After infusion of medication – i.e. mannitol |
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Hypovolemic hyponatremia
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Body water content normal, body sodium low
Renal – sodium in urine Nonrenal – no sodium in urine |
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Euvolemic hyponatremia
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Body water increased, body sodium low
SIADH or diluted formula |
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Hypervolemic hyponatremia
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Body water increased, body sodium increased
Most commonly seen in renal failure |
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As sodium decreases, ECF becomes ______ and water moves ICF, can lead to CNS dysfuction
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relatively less osmolar
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Hyponatremia Therapy
Most common complication |
is seizures
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Hyponatremia Therapy
Complications |
Over-rapid correction can lead to central pontine myelinolysis
After Na >120, prefer rate of increase 1 mEq every 2 hours |
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Hypernatremia due to
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Excessive sodium intake
Water deficit Water and sodium deficit |
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clinical findings with Hypernatremia
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Doughy skin
Fever CNS problems Brain hemorrhage as water moves out of cells |
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Hypernatremia Therapy
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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 |
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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
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Doughy skin
Fever CNS problems Brain hemorrhage as water moves out of cells |
Hypernatremia
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