Nephrology

Question 1

HYPERTENSION

Select patients

Answer 1

HYPERTENSION
Select patients <3 yo should have BP measured in medical settings or if predisposing condition. These patients are

• Past medical history
  
  • Prematurity, esp born <32 weeks
  • Very low birth weight
  • SGA
  • NICU stay
  • Hx of umbilical artery catheterization
  • Recurrent UTI
  • Malignancy
  • Bone marrow transplant
  • Solid organ transplant
• Current medical conditions
  
  • Congenital heart disease
  • Genitourinary abnormalities
  • Renal disease: renal or urologic malformations
  • Hematuria
  • Proteinuria
  • Increased intracranial pressure
  • Systemic illness associated with HTN: Any child w systemic/congenital illness known to result in HTN (ie tuberous sclerosis)
• Medications- Taking medications known to raise BP (ie chronic steroid therapy)
• Family Hx- Congenital kidney disease

Question 2

Diagnosis of HTN if child has BP readings >__th % on __ different visits.

Answer 2

Diagnosis of HTN if child has BP readings >95th % on 3 different visits.

Question 3

Age 1-12

• > ___% = Elevated blood pressure (Prehypertension)
  • Recheck BP at a 2nd visit in __ months and discuss lifestyle changes.
• > __% = Stage I HTN.
  • Recheck BP measurement in __ weeks and discuss lifestyle changes.
• > __% + ___mmHg = Stage II HTN
  • If asymptomatic, recheck measurement in __ week.
• > 95% + __mmHg (urgent HTN) OR Symptomatic (end organ damage; emergent HTN)→ emergency, go to ED right away

Answer 3

Age 1-12

• > 90% = Elevated blood pressure (Prehypertension)
  • Recheck BP at a 2nd visit in 6 months and discuss lifestyle changes.
• > 95% = Stage I HTN.
  • Recheck BP measurement in 1-2 weeks and discuss lifestyle changes.
• > 95% + 12mmHg = Stage II HTN
  • If asymptomatic, recheck measurement in 1 week.
• > 95% + 30mmHg (urgent HTN) OR Symptomatic (end organ damage; emergent HTN)→ emergency, go to ED right away

Question 4

Age >13

• <120/80 = Normal
• Between = Elevated blood pressure
• > 130/80 = Stage I HTN
• > 140/90 = Stage II HTN

Answer 4

Age >13

• <120/80 = Normal
• Between = Elevated blood pressure
• > 130/80 = Stage I HTN
• > 140/90 = Stage II HTN

Question 5

Serum osmolality = ____________

• Normal serum osmolality is ___ +/- ___
• If glucose and BUN are normal, use 2 x [Na+] to see if patient’s osmolality is ~280
• Used to differentiated euvolemia, hypovolemia, and hypervolemic hyponatremia

Answer 5

Serum osmolality = 2x [serum Na] + glucose/18 + BUN/2.8

• Normal serum osmolality is 285 +/- 10 mOsm/kg
• If glucose and BUN are normal, use 2 x [Na+] to see if patient’s osmolality is ~280
• Used to differentiated euvolemia, hypovolemia, and hypervolemic hyponatremia

Question 6

Hyponatremia

• Causes
  
  • Net depletion of sodium or dilution of sodium
  • Improper formula preparation
  • Children with diarrhea, vomiting, and burns have sodium losses through the GI or skin and present with hyponatremia when the fluid replacement occurs with excess free water (eg water, juice, ginger ale, or tea). The ECF sodium is decreased with hyponatremic dehydration, and the ICF sodium is also decreased with fluids shifting from the ECF to ICF.
• Pt:
  
  • Symptoms include CNS (headache, confusion, delirium, seizure), musculoskeletal (fatigue, muscle cramps), and GI (nausea, vomiting) findings.
• Classify:
  
  • Hyperosmolar (Pseudohyponatremia): Glucose and mannitol shift water out of cells, diluting plasma Na OR due to elevated triglycerides
    
    • Common presents in setting of hyperglycemia in DKA
    • Tx: Treat underlying disease process
  • Isoosmolar
    
    • Artifactual decrease in serum Na associated with older lab instrument that miscalculate sodium in settings of high protein or lipid
    • Tx: Artifact, no treatment
  • Hypoosmolar
    
    • Hypovolemic hyponatremia
      
      • Diuretics
      • GI losses (emesis, diarrhea)
      • 3rd spacing of fluid
      • Sodium-losing nephropathies
      • Adrenal insufficiency (Addison disease)
      • Tx: Use normal saline, watch for rapid correction
    • Hypervolemia hyponatremia
      
      • Causes: Edema-forming states: heart failure, cirrhosis, and nephrotic syndrome
      • Tx: Restrict fluids and Na +/- loop diuretics
    • Euvolemic hyponatremia
      
      • ________
        
        • Dx: Urine inappropriately concentrated/high (>250 mOsm/kg) in the setting of a low serum osmolality.
        • Rule out ____ or ___ deficiency before making diagnosis as both can have low serum osmolalities and high urine osmolalities.
      • Tx: ______. Refractory cases can be treated with ADH receptor antagonist (conivaptan or tolvaptan)
• Treatment
  
  • Symptomatic hyponatremia:
    
    • 0.9 NS to increase serum Na at a rate not to exceed 0.5 mEq/L/hr
  • Severe symptoms (Lethargy, confusion, coma, seizures) and not hypovolemia
    
    • 3% (hypertonic) saline. 100mL bolus to raise serum Na by 2-3 mEq/L
  • __________:
    
    • Path: If sodium concentration is raised too rapidly, cells then shrink as water rushes out of cells into the bloodstream, where the osmolality has risen
    • This rare effect is more likely to occur in patient with chronic, severe hyponatremia (Na<115 mEq/L for >2 days) whose sodium is corrected rapidly (>____mEq/L over 24 hours)

Answer 6

Hyponatremia

• Causes
  
  • Net depletion of sodium or dilution of sodium
  • Improper formula preparation
  • Children with diarrhea, vomiting, and burns have sodium losses through the GI or skin and present with hyponatremia when the fluid replacement occurs with excess free water (eg water, juice, ginger ale, or tea). The ECF sodium is decreased with hyponatremic dehydration, and the ICF sodium is also decreased with fluids shifting from the ECF to ICF.
• Pt:
  
  • Symptoms include CNS (headache, confusion, delirium, seizure), musculoskeletal (fatigue, muscle cramps), and GI (nausea, vomiting) findings.
• Classify:
  
  • Hyperosmolar (Pseudohyponatremia): Glucose and mannitol shift water out of cells, diluting plasma Na OR due to elevated triglycerides
    
    • Common presents in setting of hyperglycemia in DKA
    • Tx: Treat underlying disease process
  • Isoosmolar
    
    • Artifactual decrease in serum Na associated with older lab instrument that miscalculate sodium in settings of high protein or lipid
    • Tx: Artifact, no treatment
  • Hypoosmolar
    
    • Hypovolemic hyponatremia
      
      • Diuretics
      • GI losses (emesis, diarrhea)
      • 3rd spacing of fluid
      • Sodium-losing nephropathies
      • Adrenal insufficiency (Addison disease)
      • Tx: Use normal saline, watch for rapid correction
    • Hypervolemia hyponatremia
      
      • Causes: Edema-forming states: heart failure, cirrhosis, and nephrotic syndrome
      • Tx: Restrict fluids and Na +/- loop diuretics
    • Euvolemic hyponatremia
      
      • SIADH (increased reabsorption of water in kidneys), but normal sodium excretion.
        
        • Dx: Urine inappropriately concentrated/high (>250 mOsm/kg) in the setting of a low serum osmolality.
        • Rule out hypothyroidism or glucocorticoid deficiency before making diagnosis of SIADH as both can have low serum osmolalities and high urine osmolalities (although more commonly seen in SIADH).
      • Tx: Fluid restriction. Refractory cases can be treated with ADH receptor antagonist (conivaptan or tolvaptan)
• Treatment
  
  • Symptomatic hyponatremia:
    
    • 0.9 NS to increase serum Na at a rate not to exceed 0.5 mEq/L/hr
  • Severe symptoms (Lethargy, confusion, coma, seizures) and not hypovolemia
    
    • 3% (hypertonic) saline. 100mL bolus to raise serum Na by 2-3 mEq/L
  • Osmotic demyelination syndrome (Central pontine myelinolysis):
    
    • Path: If sodium concentration is raised too rapidly, cells then shrink as water rushes out of cells into the bloodstream, where the osmolality has risen
    • This rare effect is more likely to occur in patient with chronic, severe hyponatremia (Na<115 mEq/L for >2 days) whose sodium is corrected rapidly (>10 mEq/L over 24 hours)

Question 7

Hypernatremia
- Always hyperosmolar. 1st step is to determine volume status

• Hypovolemic Hypernatremia
  
  • Low volume implies both water and total body Na loss, with the water deficit exceeding the Na deficit
  • Causes: Sodium intoxication (iatrogenic, administration of baking soda), water deficit (diabetes insipidus, inadequate intake, increased insensible loss), or both water and sodium deficits (GI, cutaneous or renal losses)
  • Tx: 20ml/kg NS boluses to correct volume deficit, and then hypotonic fluids / additional free water
• Hypervolemic hypernatremia
  
  • Causes: Mineralocorticoid excess (primary hyperaldosteronism).
  • Tx: Loop diuretics and free water
• Euvolemic Hypernatremia
  
  • Most often seen in pts w ______ with reduced access to water who have not yet developed frank volume depletion.
    
    • Typically, pts with DI who have normal access to water have normal or borderline-high serum Na levels bc they are constantly drinking water (These pts are hyperosmolar and therefore always thirsty).
  • Dx: The water restriction test not only diagnoses DI but also differentiates between central and nephrogenic types.
    
    • In a healthy person, when plasma osmolality increases to 295mOsm/kg, ADH (vasopressin) is high and urine is maximally concentrated.
    • In central DI, the ADH stays low and the urine remains dilute, even with water restriction.
    • In nephrogenic DI, the ADH is appropriately high, but the urine remains dilute.
  • Tx:
    
    • Resistant central DI: _____
    • Nephrogenic DI: ___ or ____
• Avoid rapid decrease in serum osmolality. Rapid decrease in serum osmolality and sodium is associated with increased risk for cerebral edema bc of fluid shift from the extracellular fluid (lower osmolality after correction) to the brain cells (intracellular). If hypernatremia is corrected too quickly, water will move into these cells causing cerebral edema and seizures.
  Remember: SIADH typically presents as hyponatremia with normal volume. DI commonly presents as polyuria/polydipsia, with or without hypernatremia and also with normal volume.

Answer 7

Hypernatremia
- Always hyperosmolar. 1st step is to determine volume status

• Hypovolemic Hypernatremia
  
  • Low volume implies both water and total body Na loss, with the water deficit exceeding the Na deficit
  • Causes: Sodium intoxication (iatrogenic, administration of baking soda), water deficit (diabetes insipidus, inadequate intake, increased insensible loss), or both water and sodium deficits (GI, cutaneous or renal losses)
  • Tx: 20ml/kg NS boluses to correct volume deficit, and then hypotonic fluids / additional free water
• Hypervolemic hypernatremia
  
  • Causes: Mineralocorticoid excess (primary hyperaldosteronism).
  • Tx: Loop diuretics and free water
• Euvolemic Hypernatremia
  
  • Most often seen in pts w diabetes insipidus with reduced access to water who have not yet developed frank volume depletion.
    
    • Typically, pts with DI who have normal access to water have normal or borderline-high serum Na levels bc they are constantly drinking water (These pts are hyperosmolar and therefore always thirsty).
  • Dx: The water restriction test not only diagnoses DI but also differentiates between central and nephrogenic types.
    
    • In a healthy person, when plasma osmolality increases to 295mOsm/kg, ADH (vasopressin) is high and urine is maximally concentrated.
    • In central DI, the ADH stays low and the urine remains dilute, even with water restriction.
    • In nephrogenic DI, the ADH is appropriately high, but the urine remains dilute.
  • Tx:
    
    • Resistant central DI: Oral or intranasal desmopressin.
    • Nephrogenic DI: Thiazide diuretics or amiloride
• Avoid rapid decrease in serum osmolality. Rapid decrease in serum osmolality and sodium is associated with increased risk for cerebral edema bc of fluid shift from the extracellular fluid (lower osmolality after correction) to the brain cells (intracellular). If hypernatremia is corrected too quickly, water will move into these cells causing cerebral edema and seizures.
  Remember: SIADH typically presents as hyponatremia with normal volume. DI commonly presents as polyuria/polydipsia, with or without hypernatremia and also with normal volume.

Question 8

Hyperkalemia

• Causes:
  
  • Pseudohyperkalemia (falsely elevated serum potassium) is the most frequent cause of elevated potassium concentrations in the pediatric setting.
  • Transcellular shifts
    
    • _____ and _____ decrease K+ uptake → hyperkalemia (and hyperkalemia causes acidosis)
  • Decreased excretion
    
    • Renal failure
    • Type ___ RTA
    • TMP/SMX
    • Inhibited ____ release or action increases serum K
      
      • Potassium sparing diuretics (_____ blocks aldosterone receptor; _____ blocks channel for aldosterone to act)
  • Gordon Syndrome (pseudohypoaldosteronism)- Rare genetic disorder associated with abnormalities in the renal tubules, leading to HTN.
• Serum potassium concentrations >7.0 mEq/L (mmol/L) are considered severe hyperkalemia and require urgent treatment.
• Management: 1. Recheck K, 2. Get EKG (Sequence of progressive hyperkalemia: 1) ____ wave and short QT interval (K 5.5-6.5) 2) Progressive lengthening of PR and QRS intervals (K 6.5-7.5) 3) Loss of P wave and QRS widening into sine wave (K >8) 4) _______ or cardiac standstill
  
  • If wide QRS or absence of P waves, stabilize cardiac membranes and prevent fatal arrhythmias, as hyperkalemia leads to depolarization of cardiac membrane
    
    • IV _____
  • Shift K into cells
    
    • _____
    • _____
    • ____
  • Remove K from body
    
    • ____
    • _____
    • ___
• If K>6.5 without symptoms or EKG changes, treat with insulin + glucose, beta-agonists, sodium bicarbonate (if acidosis), and SPS enema.
• If K <6.5, tx with loop diuretics and dietary restriction.

Answer 8

Hyperkalemia

• Causes:
  
  • Pseudohyperkalemia (falsely elevated serum potassium) is the most frequent cause of elevated potassium concentrations in the pediatric setting.
  • Transcellular shifts
    
    • Acidosis and alpha agonists decrease K+ uptake → hyperkalemia (and hyperkalemia causes acidosis)
  • Decreased excretion
    
    • Renal failure
    • Type 4 RTA
    • TMP/SMX
    • Inhibited aldosterone release or aldosterone action increases serum K
      
      • Potassium sparing diuretics (spironolactone blocks aldosterone receptor; amiloride blocks channel for aldosterone to act)
  • Gordon Syndrome (pseudohypoaldosteronism)- Rare genetic disorder associated with abnormalities in the renal tubules, leading to HTN.
• Serum potassium concentrations >7.0 mEq/L (mmol/L) are considered severe hyperkalemia and require urgent treatment.
• Management: 1. Recheck K, 2. Get EKG (Sequence of progressive hyperkalemia: 1) peaked T wave and short QT interval (K 5.5-6.5) 2) Progressive lengthening of PR and QRS intervals (K 6.5-7.5) 3) Loss of P wave and QRS widening into sine wave (K >8) 4) Ventricular fibrillation or cardiac standstill
  
  • If wide QRS or absence of P waves, stabilize cardiac membranes and prevent fatal arrhythmias, as hyperkalemia leads to depolarization of cardiac membrane
    
    • IV calcium gluconate
  • Shift K into cells
    
    • Insulin with glucose infusion
    • Sodium bicarbonate
    • Albuterol nebulization
  • Remove K from body
    
    • Loop diuretics
    • Resin binders
    • Dialysis
• If K>6.5 without symptoms or EKG changes, treat with insulin + glucose, beta-agonists, sodium bicarbonate (if acidosis), and SPS enema.
• If K <6.5, tx with loop diuretics and dietary restriction.

Question 9

Hypokalemia

• Causes: 4 general causes: 1) inadequate intake, 2) GI losses (diarrhea has high concentration of potassium leading to hypokalemia in pts with gastroenteritis; emesis has less K but contributes to hypokalemia by way of renal losses from volume depletion), 3) non-GI losses (ie renal), and 4) transcellular shift
  
  • Increased ____ lowers serum K: Hypokalemia without obvious cause in a patient with HTN and metabolic alkalosis must cause consider hyperaldosteronism!
  • Type __ RTA for unclear reasons
  • Type __ RTA due to cotransport problems
  • ___magnesemia
  • Medications:
    
    • Loop and thiazide diuretics
    • ____ cause renal K wasting (only occurs with the sodium salt and not the potassium salt, ie PCN VK)
  • ____, ___, and ____ increase K+ uptake → hypokalemia (and hypokalemia causes alkalosis)
  • Pseudohyperkalemia: An artificial cause of hypokalemia can occur in pts with very elevated WBC counts
• Pt: Flattened T waves followed by ST depression and the emergence of U waves, decreased deep tendon reflexes, rhabdomyolysis, muscle weakness or cramping that may progress to paralysis, cardiac arrhythmias
• Management: 1) Recheck K 2) Check magnesium and replete (magnesium deficiency causes renal potassium wasting)
  
  • Potassium chloride is preferred over potassium phosphate, potassium citrate, or potassium bicarbonate
  • Asymptomatic hypokalemia patients with serum K 3-3.5 mEq/L (mmol/L) can be treated with increased intake of dietary K/
  • In patients with no contraindication to oral intake, PO enteral administration is the preferred route for K replacement.

Answer 9

Hypokalemia

• Causes: 4 general causes: 1) inadequate intake, 2) GI losses (diarrhea has high concentration of potassium leading to hypokalemia in pts with gastroenteritis; emesis has less K but contributes to hypokalemia by way of renal losses from volume depletion), 3) non-GI losses (ie renal), and 4) transcellular shift
  
  • Increased aldosterone lowers serum K: Hypokalemia without obvious cause in a patient with HTN and metabolic alkalosis must cause consider hyperaldosteronism!
  • Type 1 RTA for unclear reasons
  • Type 2 RTA due to cotransport problems
  • Hypomagnesemia
  • Medications:
    
    • Loop and thiazide diuretics
    • Penicillins cause renal K wasting (only occurs with the sodium salt of penicillin and not the potassium salt, ie PCN VK)
  • Alkalosis, beta-agonists, and insulin increase K+ uptake → hypokalemia (and hypokalemia causes alkalosis)
  • Pseudohyperkalemia: An artificial cause of hypokalemia can occur in pts with very elevated WBC counts
• Pt: Flattened T waves followed by ST depression and the emergence of U waves, decreased deep tendon reflexes, rhabdomyolysis, muscle weakness or cramping that may progress to paralysis, cardiac arrhythmias
• Management: 1) Recheck K 2) Check magnesium and replete (magnesium deficiency causes renal potassium wasting)
  
  • Potassium chloride is preferred over potassium phosphate, potassium citrate, or potassium bicarbonate
  • Asymptomatic hypokalemia patients with serum K 3-3.5 mEq/L (mmol/L) can be treated with increased intake of dietary K/
  • In patients with no contraindication to oral intake, PO enteral administration is the preferred route for K replacement.

Question 10

Hypokalemia and nonanion gap metabolic acidosis without HTN

• K+ loss usually from diarrhea or RTA
• Urine anion gap differentiate causes: negative UAG in diarrhea, positive UAG in RTA

Answer 10

Hypokalemia and nonanion gap metabolic acidosis without HTN

• K+ loss usually from diarrhea or RTA
• Urine anion gap differentiate causes: negative UAG in diarrhea, positive UAG in RTA

Question 11

Hypokalemia and metabolic alkalosis with HTN

• Diuretics (most common) causing contraction alkalosis. Urine sodium is increased in spite of volume contraction.
• Hyperaldosteronism (primary or secondary)
• Cushing syndrome
• Liddle syndrome
• Adrenal hydroxylase deficiencies

Answer 11

Hypokalemia and metabolic alkalosis with HTN

• Diuretics (most common) causing contraction alkalosis. Urine sodium is increased in spite of volume contraction.
• Hyperaldosteronism (primary or secondary)
• Cushing syndrome
• Liddle syndrome
• Adrenal hydroxylase deficiencies

Question 12

Hypokalemia and metabolic alkalosis without HTN

• Bartter syndrome
• Gitelman syndrome

Answer 12

Hypokalemia and metabolic alkalosis without HTN

• Bartter syndrome
• Gitelman syndrome

Question 13

• Genetic syndromes:
  - Liddle syndrome (Pseudoaldosteronism):
  - ____Inheritance? condition
  - Path: Mutation that causes the epithelial sodium channel in the ____ tubule to become constitutively active
  - Pt: ___ blood pressure , ___kalemia, ___natremia, and metabolic ____ (high urinary chloride) with ___ aldosterone
  - Tx: ______

Answer 13

• Genetic syndromes:
  - Liddle syndrome (Pseudoaldosteronism):
  - AD condition
  - Path: Mutation that causes the epithelial sodium channel in the distal tubule to become constitutively active
  - Pt: HTN, hypokalemia, hypernatremia, and metabolic alkalosis (high urinary chloride) with low aldosterone
  - Tx: Amiloride or triamterene
  - Bartter’s and Gittleman’s are due to abnormal solute transport in the thick ascending limb. Increased aldosterone and renin.

Question 14

• Bartter’s:
  - _____Inheritance?
  - Path: Primary defect in NaCl reabsorption in ascending limb of loop of Henle. Looks like taking a ____ diuretic
  - Pt:
  - Polyhydramnios, premature delivery
  - Metabolic ___, ___kalemia, ___natremia, ___chloremia, polyuria (12-50ml/kg/day) or polydipsia, ____ blood pressure
  - Associated ____. ___calciuria (may present with stones/nephrocalcinosis)

Answer 14

• Bartter’s:
  - AR
  - Path: Primary defect in NaCl reabsorption in ascending limb of loop of Henle. Looks like taking a loop diuretic
  - Pt:
  - Polyhydramnios, premature delivery
  - Metabolic alkalosis, hypokalemia, hyponatremia, hypochloremia, polyuria (12-50ml/kg/day) or polydipsia, normal blood pressure WITHOUT HTN
  - Associated deafness. Hypercalciuria (may present with stones/nephrocalcinosis)

Question 15

• Gitleman’s
  - ____ Inheritance?
  - Path: Defect in NaCl cotransporter in early distal tubule. Looks like taking _____ diuretic except that pt has severe magnesium wasting that is not understood
  - Pt:
  - Milder than Bartter’s and presents later in life
  - ___kalemia, metabolic ___, ____ blood pressure
  - ____magnesemia, ____calciuria (urine) (in contrast to Bartter syndrome, children with Gittleman have reduced ____ and ____ (more common)

Answer 15

• Gitleman’s
  - AR
  - Path: Defect in NaCl cotransporter in early distal tubule. Looks like taking thiazide diuretic except that pt has severe magnesium wasting that is not understood
  - Pt:
  - Milder than Bartter’s and presents later in life
  - Hypokalemia, metabolic alkalosis, WITHOUT HTN
  - Hypomagnesemia, hypocalciuria (urine) (in contrast to Bartter syndrome, children with Gittleman have reduced urinary calcium and hypomagnesemia (more common)

Question 16

• Bartter’s and Gittleman’s are due to abnormal solute transport in the thick ascending limb. Increased ____ and ____.

Answer 16

• Bartter’s and Gittleman’s are due to abnormal solute transport in the thick ascending limb. Increased aldosterone and renin.

Question 17

CALCIUM

• Calcium is regulated by parathyroid hormone and vitamin D metabolites
• Measure ionized calcium directly to get most reliable assessment of calcium status
• Calcium levels should be corrected for the ____ level, or an ionized calcium should be measured.
  
  • Corrected Ca = _____
  • Correct calcium: for each 1 g/dL decrease in albumin, increase the measured serum calcium by 0.8 mg/dL. If albumin decreases, the measured serum calcium decreases.

Answer 17

CALCIUM

• Calcium is regulated by parathyroid hormone and vitamin D metabolites
• Measure ionized calcium directly to get most reliable assessment of calcium status
• Calcium levels should be corrected for the albumin level, or an ionized calcium should be measured.
  
  • Corrected Ca = Ca + [0.8x(4-Alb)]
  • Correct calcium: for each 1 g/dL decrease in albumin, increase the measured serum calcium by 0.8 mg/dL. If albumin decreases, the measured serum calcium decreases.

Question 18

Hypercalcemia

• Causes
  
  • Primary hyperparathyroidism (most common etiology)
  • Prolonged immobilization - excess calcium release from bone suppresses PTH level
  • Thiazide diuretics
  • Hypercalcemia of malignancy
  • Familial hypercalcemia hypocalciuria
    
    • _____ Inheritance???
    • Parathyroid glands are normal. Mildly ___ serum calcium, ____ urine calcium, inappropriately ____ PTH for calcium level (as should be suppressed in hypercalcemia)
    • Tx: _____.
  • Hypervitaminosis
  • Multiple myeloma
  • Granulomatous disease: sarcoidosis and tuberculosis
  • Hypophosphatemia
  • Williams syndrome
  • Subcutaneous fat necrosis (self-limited panniculitis)
  • Other: leukemia, thyrotoxicosis
  • Hypervitaminosis A
  • Milk alkali (taking too many antacids)
• Pt: ____, ____ (fatigue, weight loss, dehydration, decreased appetite), ___ (abdominal pain, constipation, vomiting), ____ (bone pain), ______ (depression, anxiety, confusion, and impairment of cognitive functioning).
• Labs: PTH level is useful to classify hypercalcemia as PTH dependent or non-PTH dependent.
• Tx:
  
  • Most pts with significant hypercalcemia are volume depleted and need NS replacement fluid.
  • ____ and _____ are now standard of care for persistent hypercalcemia.

Answer 18

Hypercalcemia

• Causes
  
  • Primary hyperparathyroidism (most common etiology)
  • Prolonged immobilization - excess calcium release from bone suppresses PTH level
  • Thiazide diuretics
  • Hypercalcemia of malignancy
  • Familial hypercalcemia hypocalciuria
    
    • AD
    • Parathyroid glands are normal. Mildly elevated serum calcium, low urine calcium, inappropriately normal PTH for calcium level (as should be suppressed in hypercalcemia)
    • Tx: None.
  • Hypervitaminosis
  • Multiple myeloma
  • Granulomatous disease: sarcoidosis and tuberculosis
  • Hypophosphatemia
  • Williams syndrome
  • Subcutaneous fat necrosis (self-limited panniculitis)
  • Other: leukemia, thyrotoxicosis
  • Hypervitaminosis A
  • Milk alkali (taking too many antacids)
• Pt: Stones (kidney), moans (fatigue, weight loss, dehydration, decreased appetite), groans (abdominal pain, constipation, vomiting), bones (bone pain), psych overtones (depression, anxiety, confusion, and impairment of cognitive functioning).
• Labs: PTH level is useful to classify hypercalcemia as PTH dependent or non-PTH dependent.
• Tx:
  
  • Most pts with significant hypercalcemia are volume depleted and need NS replacement fluid.
  • Calcitonin and bisphosphates are now standard of care for persistent hypercalcemia.

Question 19

Hypocalcemia

• Neonatal hypocalcemia
  
  • Early onset
    
    • Most common cause is _____
  • Late onset
    
    • Often due to vitamin D deficiency with hypomagnesemia and can be treated with limited therapy
• Causes:
  
  • Vitamin D deficiency
  • Chronic kidney disease results in secondary hyperparathyroidism
  • Severe pancreatitis
  • Rhabdomyolysis
  • _____magnesemia (always check magnesium level!)
    
    • Magnesium deficiency, which can result from malabsorption syndromes, and cystic fibrosis. Hypomagnesemia is thought to cause hypocalcemia by not permitting release of PTH. This is why you always want to check magnesium when a pt’s calcium is low bc you never fix calcium until you fix the magnesium.
  • Hungry bone syndrome following parathyroidectomy
  • Hypoparathyroidism
  • Pseudohypoparathyroidism
  • Citrate (anticoagulant in whole blood) chelates calcium in serum
  • Poisoning with inorganic phosphate
  • Early in the course of tx of ALL, hypocalcemia can occur due to the destruction of lymphoblasts.
  • Kenny-Caffey syndrome is a condition with medullary stenosis of the long bones, short stature, delayed bone age, and eye abnormalities. It causes episodic hypocalcemia.
• Pt: Jitteriness, muscle twitching, seizures, tetany, perioral tingling, muscle twitching
  
  • Positive ___ or ____ signs may be present.
• In more severe cases of _____, an ECG shows prolongation of the QT interval, which can lead to a fatal arrhythmia ____.
• Tx: 1) Check and correct with ___ 2) Check ionized calcium and check ___
  
  • Mild (paresthesias): ___
  • Severe (seizures, arrhythmias):___

Answer 19

Hypocalcemia

• Neonatal hypocalcemia
  
  • Early onset
    
    • Most common cause is prematurity
  • Late onset
    
    • Often due to vitamin D deficiency with hypomagnesemia and can be treated with limited therapy
• Causes:
  
  • Vitamin D deficiency
  • Chronic kidney disease results in secondary hyperparathyroidism
  • Severe pancreatitis
  • Rhabdomyolysis
  • Hypermagnesemia (always check magnesium level!)
    
    • Magnesium deficiency, which can result from malabsorption syndromes, and cystic fibrosis. Hypomagnesemia is thought to cause hypocalcemia by not permitting release of PTH. This is why you always want to check magnesium when a pt’s calcium is low bc you never fix calcium until you fix the magnesium.
  • Hungry bone syndrome following parathyroidectomy
  • Hypoparathyroidism
  • Pseudohypoparathyroidism
  • Citrate (anticoagulant in whole blood) chelates calcium in serum
  • Poisoning with inorganic phosphate
  • Early in the course of tx of ALL, hypocalcemia can occur due to the destruction of lymphoblasts.
  • Kenny-Caffey syndrome is a condition with medullary stenosis of the long bones, short stature, delayed bone age, and eye abnormalities. It causes episodic hypocalcemia.
• Pt: Jitteriness, muscle twitching, seizures, tetany, perioral tingling, muscle twitching
  
  • Positive Chvostek or Trousseau signs may be present.
• In more severe cases of hypocalcemia, an ECG shows prolongation of the QT interval, which can lead to a fatal arrhythmia torsades de pointes.
• Tx: 1) Check and correct with albumin 2) Check ionized calcium and check magnesium
  
  • Mild (paresthesias): Oral calcium
  • Severe (seizures, arrhythmias): IV calcium gluconate while monitoring heart rates

Question 20

• Diarrhea results in metabolic ___ with a ___ bicarb.

Answer 20

• Diarrhea results in metabolic acidosis with a low bicarb.

Question 21

• Vomiting causes metabolic ____ due to loss of hydrochloric acid; would have ___ bicarb.

Answer 21

• Vomiting causes metabolic alkalosis due to loss of hydrochloric acid; would have elevated bicarb.

Question 22

• Cystic fibrosis is at high risk for _natremic, _chloremic metabolic ___ when dehydrated.

Answer 22

• Cystic fibrosis is at high risk for hyponatremic, hypochloremic metabolic alkalosis when dehydrated.

Question 23

• Alkalemia causes ______calcemia - even though serum calcium level is unchanged (= ionized + bound).

Answer 23

• Alkalemia causes an increase in the fraction of bound calcium, and the resulting decrease in ionized calcium produces these symptoms of hypocalcemia - even though serum calcium level is unchanged (= ionized + bound).

Question 24

• 1) High OG and high AG
  
  • *Ethylene glycol (antifreeze). Toxic metabolite is glycolic acid
    
    • Ethylene glycol (____ in urine. Metabolizes to glycolic acid and then oxalic acid)
    • Tx: ____ and dialysis
  • *Methanol (paint thinner, de-icing solutions/windshield wiper fluids, moonshine). Toxic metabolite is formic acid
    
    • Visual symptoms. Metabolizes to formic acid, which is directly toxic to ___
    • Tx: ____ and dialysis
  • *Propylene glycol (rarely, low toxicity). Toxic metabolite is lactic acid
    
    • Used as solvent for IV lorazepam, is approved food sweetener. Can cause severe lactic acidosis

Answer 24

• 1) High OG and high AG
  
  • *Ethylene glycol (antifreeze). Toxic metabolite is glycolic acid
    
    • Ethylene glycol (Calcium oxalate crystals in urine. Metabolizes to glycolic acid and then oxalic acid)
    • Tx: Fomepizole and dialysis
  • *Methanol (paint thinner, de-icing solutions/windshield wiper fluids, moonshine). Toxic metabolite is formic acid
    
    • Visual symptoms. Metabolizes to formic acid, which is directly toxic to optic nerve
    • Tx: Fomepizole and dialysis
  • *Propylene glycol (rarely, low toxicity). Toxic metabolite is lactic acid
    
    • Used as solvent for IV lorazepam, is approved food sweetener. Can cause severe lactic acidosis

Question 25

High anion gap metabolic acidosis (HAGMA) - Anion gap acidosis occurs when an acid is added to the blood: CO2 is combined with H+, leading to low CO2, but Cl is ____ - MUDPILES - M - U - D - P, P, P - I, I, i, I - L - E - S - Patients with metabolic acidosis often develop a compensatory respiratory alkalosis via tachypnea to normalize acid-base status.

Answer 25

High anion gap metabolic acidosis (HAGMA) - Anion gap acidosis occurs when an acid is added to the blood: CO2 is combined with H+, leading to low CO2, but Cl is normal - MUDPILES - Methanol (directly toxic to optic nerve, causes vision loss, coma) - Uremia - DKA - Propylene glycol (food sweetener), Propofol, Paraldehyde - Isopropyl alcohol, Iron, isoniazid, Infection - Lactic acidosis (ie from post seizure) - Ethylene glycol (antifreeze, damages kidneys, calcium oxalate crystals in urine, see hypocalcemia) - Salicylates - Patients with metabolic acidosis often develop a compensatory respiratory alkalosis via tachypnea to normalize acid-base status.

Question 26

Normal anion gap metabolic acidosis (NAGMA) - Occurs when HCO3- is lost. - __chloremic acidosis that shows increase in Cl with decrease in HCO3. Cl is increased to maintain electrical neutrality - Low bicarb with a ____ chloride - Causes: HARDUP - H - A - R - D - U - P - K - NAGMA + Hyperkalemia: Think Type ___ RTA - NAGMA + Hypokalemia: GI loss or RTA __ or __ - UAG - NAGMA + positive UAG: RTA - NAGMA + negative UAG: GI losses

Answer 26

Normal anion gap metabolic acidosis (NAGMA) - Occurs when HCO3- is lost. - Hyperchloremic acidosis that shows increase in Cl with decrease in HCO3. Cl is increased to maintain electrical neutrality - Low bicarb with a high chloride - Causes: HARDUP - Hyperalimentation - TPN can increase the generation of NH4+ as a breakdown product of amino acids - Acetazolamide - Renal tubular acidosis, Renal failure - inability to excrete H+ - Diarrhea - Urinary diversion - Pancreatic fistula - K - NAGMA + Hyperkalemia: Think Type 4 RTA (hypoaldosteronism) - NAGMA + Hypokalemia: GI loss or RTA 1 or 2 - UAG - NAGMA + positive UAG: RTA - NAGMA + negative UAG: GI losses

Question 27

Urine anion gap (UAG) = ________ - Estimate of unmeasured ions in the urine, the most important one being ____ - Normal value is close to 0 - Differentiates between GI loss of HCO3 (diarrhea) and RTA - Normal/positive UAG suggests low urinary NH4 (RTA) - Negative UAG (NeGUTive) suggests high renal excretion of H in form of NH4 (diarrhea with HCO3- loss)

Answer 27

Urine anion gap (UAG) = Na + K - Cl - Estimate of unmeasured ions in the urine, the most important one being ammonium (NH4+) - Normal value is close to 0 - Differentiates between GI loss of HCO3 (diarrhea) and RTA - Normal/positive UAG suggests low urinary NH4 (RTA) - Negative UAG (NeGUTive) suggests high renal excretion of H in form of NH4 (diarrhea with HCO3- loss)

Question 28

Metabolic Alkalosis - ____chloremia is usually associated with metabolic alkalosis resulting from chloride loss associated with GI or renal losses - Chloride responsive - urinary Cl- <10 mEq/L (NaCl reabsorbed to maintain intravascular volume) - Vomiting - Diuretics - Posthypercapnia - Cystic fibrosis - Treatment: Restoration of volume with IV fluids (NaCl or KCl). Potassium correction is integral - Chloride resistant - Urinary Cl- >10 mEq/L - No HTN - Bartter syndrome (hypokalemia, hyponatremia) - Gitelman syndrome (hypomagnesemia, hypocalciuria) - HTN - Cushing syndrome - Primary hyperaldosteronism - Liddle syndrome - 11-beta-OH deficiency - Exogenous steroids

Answer 28

Metabolic Alkalosis - Hypochloremia is usually associated with metabolic alkalosis resulting from chloride loss associated with GI or renal losses - Chloride responsive - urinary Cl- <10 mEq/L (NaCl reabsorbed to maintain intravascular volume) - Vomiting - Diuretics - Posthypercapnia - Cystic fibrosis - Treatment: Restoration of volume with IV fluids (NaCl or KCl). Potassium correction is integral - Chloride resistant - Urinary Cl- >10 mEq/L - No HTN - Bartter syndrome (hypokalemia, hyponatremia) - Gitelman syndrome (hypomagnesemia, hypocalciuria) - HTN - Cushing syndrome - Primary hyperaldosteronism - Liddle syndrome - 11-beta-OH deficiency - Exogenous steroids

Question 29

If there is metabolic acidosis, check the Winter’s formula to see if there is also a respiratory acidosis/alkalosis present. If there is high anion gap metabolic acidosis, check the AG vs HCO3 to see if there is a metabolic alkalosis component; also consider checking Osmolal gap if intoxicated. If there is a non-anion gap metabolic acidosis, check the urine anion gap.

Answer 29

If there is metabolic acidosis, check the Winter’s formula to see if there is also a respiratory acidosis/alkalosis present. If there is high anion gap metabolic acidosis, check the AG vs HCO3 to see if there is a metabolic alkalosis component; also consider checking Osmolal gap if intoxicated. If there is a non-anion gap metabolic acidosis, check the urine anion gap.

Question 30

- The primary treatment for all forms of RTA is ______ replacement - Think of the type number as indicating the serum K level: Type __ = low, Type __ = low-normal, Type __ = high

Answer 30

- The primary treatment for all forms of RTA is bicarbonate replacement - Think of the type number as indicating the serum K level: Type 1 = low, Type 2 = low-normal, Type 4 = high

Question 31

Type I Distal: Impaired distal ___ secretion into urine. _____ K - Path: Defect of distal tubule H+ secretion by cortical collecting duct - Causes: - Meds (amphotericin B, toluene (glue sniffing), lithium) - Autoimmune (sjogren's, SLE, rheumatoid arthritis) - Chronic active hepatitis - Genetic disorders - Pt: - Urinary pH >___. ___kalemia - Commonly causes ___ and ___, likely caused by decreased citrate excretion and hypercalcemia (citrate is a natural neutralizer of calcium crystallization)

Answer 31

Type I Distal: Impaired distal hydrogen secretion into urine. Low K - Path: Defect of distal tubule H+ secretion by cortical collecting duct - Causes: - Meds (amphotericin B, toluene (glue sniffing), lithium) - Autoimmune (sjogren's, SLE, rheumatoid arthritis) - Chronic active hepatitis - Genetic disorders - Pt: - Urinary pH >5.5. Hypokalemia - Commonly causes nephrocalcinosis and renal stones, likely caused by decreased citrate excretion and hypercalcemia (citrate is a natural neutralizer of calcium crystallization)

Question 32

Type 2 Proximal tubule: Poor ____ resorption. ___ K - Path: Mechanism similar to _____ - decreasing bicarbonate reabsorption in proximal tubule - Causes: - Idiopathic - ____ syndrome - Associated with: Rickets - Cystinosis - Drugs: Acetazolamide, 6-mercaptopurine, expired tetracycline - Heavy metal poisonings (lead, copper, mercury, cadmium) - Multiple myeloma - Disorders of protein, carbohydrate, or amino acid metabolism - Pt: - Urine pH

Answer 32

Type 2 Proximal tubule: Poor bicarb resorption. Low/normal K - Path: Mechanism similar to acetazolamide - decreasing bicarbonate reabsorption in proximal tubule - Causes: - Idiopathic - Fanconi syndrome - Associated with: Rickets - Cystinosis - Drugs: Acetazolamide, 6-mercaptopurine, expired tetracycline - Heavy metal poisonings (lead, copper, mercury, cadmium) - Multiple myeloma - Disorders of protein, carbohydrate, or amino acid metabolism - Pt: - Urine pH <5.5. Hypokalemia (decreased)

Question 33

Type 4 Distal: ____ resistance (or secretion problem) (defect in Na/K exchange). ___ K - Path: Similar to ____ or hypo___ - Causes: Most commonly in pts with chronic renal failure ass w renal parenchymal injury and scarring - Obstructive uropathy (most common) - Interstitial renal disease (most common) - Type 1 pseudohypoaldosteronism - Diabetic nephropathy - CAH / 21-hydroxylase deficiency - Renal transplant - Pt: Urine pH _____, ____kalemia

Answer 33

Type 4 Distal: Aldosterone resistance (or aldosterone secretion problem) (defect in Na/K exchange). High K - Path: Similar to spironolactone or hypoaldosteronism - Causes: Most commonly in pts with chronic renal failure ass w renal parenchymal injury and scarring - Obstructive uropathy (most common) - Interstitial renal disease (most common) - Type 1 pseudohypoaldosteronism - Diabetic nephropathy - CAH / 21-hydroxylase deficiency - Renal transplant - Pt: Urine pH <5.5, hyperkalemia (elevated)

Question 34

GFR - GFR can be estimated from creatinine clearance (using a 24-hour urine collection). - GFR = CrCl = (UCr x V) / PCr - Creatinine is produced by muscle tissue - the more muscle tissue there is, the more creatinine there is to be cleared from the blood - Estimate GFR (Schwartz formula) = 0.413 x (height in cm/plasma creatinine in mg/dL) - ____ is a promising and perhaps more reliable marker to estimate GFR.

Answer 34

GFR - GFR can be estimated from creatinine clearance (using a 24-hour urine collection). - GFR = CrCl = (UCr x V) / PCr - Creatinine is produced by muscle tissue - the more muscle tissue there is, the more creatinine there is to be cleared from the blood - Estimate GFR (Schwartz formula) = 0.413 x (height in cm/plasma creatinine in mg/dL) - Cystatin C is a promising and perhaps more reliable marker to estimate GFR.

Question 35

An elevated BUN:Cr (LESS/GREATER THAN ___:1) can indicate prerenal azotemia FENa is typically LESS/GREATER THAN ___ in early AGN and prerenal azotemia; LESS/GREATER THAN __ in other causes of AKI

Answer 35

An elevated BUN:Cr (>20:1) can indicate prerenal azotemia FENa is typically <1 in early AGN and prerenal azotemia; >1 in other causes of AKI

Question 36

``` PROTEINURIA Urine Dipstick - Positive protein (Detects albumin. Does not detect amino acids or kappa/lambda) - Trace = __ mg - 1+ = __-__mg - 2+ = ___-__mg. Equivalent to Pr:Cr 0.1-0.3. Need to quantify (below) - 3+ = __-___mg - 4+ = 1000mg ``` - Normal urine albumin secretion is less than ____

Answer 36

``` PROTEINURIA Urine Dipstick - Positive protein (Detects albumin. Does not detect amino acids or kappa/lambda) - Trace = 30 mg - 1+ = 30-100mg - 2+ = 100-300mg. Equivalent to Pr:Cr 0.1-0.3. Need to quantify (below) - 3+ = 300-1000mg - 4+ = 1000mg ``` - Normal urine albumin secretion is <30mg/day.

Question 37

Microalbuminuria is defined as urine albumin secretion ___-300mg/day and is an important RF for end organ damage in pts with DM or HTN. - Microalbuminuria is one the earliest signs of _____. - Indication of early glomerular injury, including diabetic nephropathy - Detected on Random urine Albumin:Creatinine - __-__ mg/g suggests incipient diabetic nephropathy - >___ mg/g indicates overt diabetic nephropathy - For patients with Type I DM, screen yearly for albuminuria, beginning 3-5 years after initial diagnosis. For those with Type 2 DM, screen yearly after initial diagnosis.

Answer 37

Microalbuminuria is defined as urine albumin secretion 30-300mg/day and is an important RF for end organ damage in pts with DM or HTN. - Microalbuminuria is one the earliest signs of diabetic nephropathy. - Indication of early glomerular injury, including diabetic nephropathy - Detected on Random urine Albumin:Creatinine - 30-300 mg/g suggests incipient diabetic nephropathy - >300 mg/g indicates overt diabetic nephropathy - For patients with Type I DM, screen yearly for albuminuria, beginning 3-5 years after initial diagnosis. For those with Type 2 DM, screen yearly after initial diagnosis.

Question 38

Protein:Creatinine ratio - Normal Pr:Cr = less than ____ - Proteinuria = ___ mg protein/mg creatinine on single spot urine collection - Nephrotic range proteinuria = spot urine (preferably 1st morning sample) Pr-to-Cr ratio >___

Answer 38

Protein:Creatinine ratio - Normal Pr:Cr = <0.2 - Proteinuria = >0.2 mg protein/mg creatinine on single spot urine collection - Nephrotic range proteinuria = spot urine (preferably 1st morning sample) Pr-to-Cr ratio >2

Question 39

24 hour urine is most accurate - Normal 24-hour urine protein excretion is ___ mg/m2/hour - Proteinuria = >__ mg/m2/day - Nephrotic range = >___ mg/m2/day

Answer 39

24 hour urine is most accurate - Normal 24-hour urine protein excretion is 4 mg/m2/hour - Proteinuria = >100 mg/m2/day - Nephrotic range = >1000 mg/m2/day

Question 40

An random urine pr:cr ratio >__ or excretion rate >__ mg/m2/hr or >__g/m2/day indicates nephrotic range proteinuria

Answer 40

An random urine pr:cr ratio >2 or excretion rate >40 mg/m2/hr or >1g/m2/day indicates nephrotic range proteinuria

Question 41

Causes of false-positive urine albumin on dipstick: - Elevated Pr:Cr may be due to - Increased specific gravity/concentrated urine - Decreased specific gravity can decrease ratio - Alkaline urine pH > 8 - Nitrates, leuk esterase, bacteria present - Transient reasons due to fever, viral illness, increased activity Transient proteinuria - During febrile illness, after strenuous exercise, stress, cold exposure, seizures, epinephrine use, stress, abdominal surgery, and in patients with heart failure.

Answer 41

Causes of false-positive urine albumin on dipstick: - Elevated Pr:Cr may be due to - Increased specific gravity/concentrated urine - Decreased specific gravity can decrease ratio - Alkaline urine pH > 8 - Nitrates, leuk esterase, bacteria present - Transient reasons due to fever, viral illness, increased activity Transient proteinuria - During febrile illness, after strenuous exercise, stress, cold exposure, seizures, epinephrine use, stress, abdominal surgery, and in patients with heart failure.

Question 42

Orthostatic/postural proteinuria - This is the most common cause of proteinuria in adolescents - On average, these pts excrete <1g protein in 24 hours in the upright position. - Diagnosis: - Confirm by comparing urine protein-to-creatinine ratio in urine samples collected in both supine and standing positions. - A _____ specimen for proteinuria is an easy way to differentiate orthostatic and fixed/persistent proteinuria - Tx: - Yearly follow-up to monitor for fixed proteinuria, which would require additional evaluation. - It has excellent prognosis

Answer 42

Orthostatic/postural proteinuria - This is the most common cause of proteinuria in adolescents - On average, these pts excrete <1g protein in 24 hours in the upright position. - Diagnosis: - Confirm by comparing urine protein-to-creatinine ratio in urine samples collected in both supine and standing positions. - A 1st morning urine specimen for proteinuria is an easy way to differentiate orthostatic and fixed/persistent proteinuria - Tx: - Yearly follow-up to monitor for fixed proteinuria, which would require additional evaluation. - It has excellent prognosis

Question 43

NEPHROTIC SYNDROMES - Tetrad: - ____ - ____ - ____ - ____ - Complications: 2 leading life-threatening associated conditions are ______ and _____ - Infection due to immunoglobulin deficiency - Nearly 50% of infection are due to Strep pneumoniae - Add PPSV23 - Thrombotic disease - Edema and anasarca (massive generalized edema) - Renal insufficiency - Hypovolemia - Hyperlipidemia - Oval fat bodies / Maltese crosses under polarized light and waxy/hyaline casts are typical - If HTN or increased Cr, consider ______ - Nephrotic range proteinuria = spot urine (preferably 1st morning sample) Pr-to-Cr ratio >2 - Treatment of idiopathic NS - Initial episode is treated with oral ____ - The most important determinant of renal prognosis in idiopathic NS is ____

Answer 43

NEPHROTIC SYNDROMES - Tetrad: - Heavy proteinuria (>1000mg/m2/day or >40mg/m2/hr or random urine protein:creatinine >2) - Edema (facial puffiness or generalized anasarca) - Hypoalbuminemia (serum albumin <3mg/dL) - Hyperlipidemia (elevated cholesterol and LDL cholesterol) - Complications: 2 leading life-threatening associated conditions are infection and hypercoagulability - Infection due to immunoglobulin deficiency - Nearly 50% of infection are due to Strep pneumoniae - Add PPSV23 - Thrombotic disease - Edema and anasarca (massive generalized edema) - Renal insufficiency - Hypovolemia - Hyperlipidemia - Oval fat bodies / Maltese crosses under polarized light and waxy/hyaline casts are typical - If HTN or increased Cr, consider FSGS - Nephrotic range proteinuria = spot urine (preferably 1st morning sample) Pr-to-Cr ratio >2 - Treatment of idiopathic NS - Initial episode is treated with oral steroids - The most important determinant of renal prognosis in idiopathic NS is steroid responsiveness.

Question 44

Minimal Change Disease Nephrotic Syndrome - Most common cause of nephrotic syndrome in childhood - Pt: - Early-morning eyelid swelling is common, and allergic reactions are frequently misdiagnosed. - Ascites is also typical, and “my clothes don’t fit right” or “my shoes are too tight” are classic complaints. - Labs: - Proteinuria is universal. Oval fat bodies (which look like Maltese crosses under polarized light) and waxy or hyaline casts are typical. - Dx: Most clinically - Typically, clinical presentation is treated empirically without a renal biopsy. Renal biopsy is not indicated except in those <1yo or in the presence of microscopic hematuria, hypocomplementemia, resistance to steroids within 6-8 weeks of treatment, frequent relapses, or renal failure not due to volume contraction. - Management: - Management of edema: - Restrict ____. - If severe cases, may consider diuretics/Lasix. - IV albumin is not indicated except in children with pleural effusions and ascites causing significant discomfort and/or respiratory difficulties. - Inoculate with the ____ vaccines and avoid NSAIDs. - Treat empirically with steroids without renal biopsy. - Initial episode of NS is tx with oral ______ (prednisone 2mg/kg/day (or 60mg/m2/day; max 80mg) daily for 4-6 weeks, followed by 40mg/m2/dose every other day for 2-5 months, with gradual tapering). - Consider renal biopsy if remission does not occur in 8-10 weeks - Relapse - 1st relapse is treated with ______ 2mg/kg/day or 60 mg/m2/day (max 80mg) for 6 weeks, which will help decrease change of further relapses. Consider biopsy if remission does not occur in 8-10 weeks - Consider steroid-sparing agent to avoid the complications ass with long-term steroid therapy: Cyclosporine-tacrolimus, mycophenolate mofetil, cyclophosphamide, chlorambucil - Prognosis: Survival rates are 90-95%, but deaths still occur mainly bc of infections (rarely due to thrombotic complications); therefore, tx bacterial infections aggressively with antibiotics.

Answer 44

Minimal Change Disease Nephrotic Syndrome - Most common cause of nephrotic syndrome in childhood - Pt: - Early-morning eyelid swelling is common, and allergic reactions are frequently misdiagnosed. - Ascites is also typical, and “my clothes don’t fit right” or “my shoes are too tight” are classic complaints. - Labs: - Proteinuria is universal. Oval fat bodies (which look like Maltese crosses under polarized light) and waxy or hyaline casts are typical. - Dx: Most clinically - Typically, clinical presentation is treated empirically without a renal biopsy. Renal biopsy is not indicated except in those <1yo or in the presence of microscopic hematuria, hypocomplementemia, resistance to steroids within 6-8 weeks of treatment, frequent relapses, or renal failure not due to volume contraction. - Management: - Management of edema: - Restrict sodium. - If severe cases, may consider diuretics/Lasix. - IV albumin is not indicated except in children with pleural effusions and ascites causing significant discomfort and/or respiratory difficulties. - Inoculate with the PCV23 vaccines and avoid NSAIDs. - Treat empirically with steroids without renal biopsy. - Initial episode of NS is tx with oral steroids (prednisone 2mg/kg/day (or 60mg/m2/day; max 80mg) daily for 4-6 weeks, followed by 40mg/m2/dose every other day for 2-5 months, with gradual tapering). - Consider renal biopsy if remission does not occur in 8-10 weeks - Relapse - 1st relapse is treated with high dose steroids 2mg/kg/day or 60 mg/m2/day (max 80mg) for 6 weeks, which will help decrease change of further relapses. Consider biopsy if remission does not occur in 8-10 weeks - Consider steroid-sparing agent to avoid the complications ass with long-term steroid therapy: Cyclosporine-tacrolimus, mycophenolate mofetil, cyclophosphamide, chlorambucil - Prognosis: Survival rates are 90-95%, but deaths still occur mainly bc of infections (rarely due to thrombotic complications); therefore, tx bacterial infections aggressively with antibiotics.

Question 45

Focal Segmental Glomerulosclerosis (FSGS) - Most common glomerular cause of ESRD in childhood (overall, congenital anomalies are the #1 cause of ESRD in childhood). 2nd most common cause of NS in childhood. - Pt: - Most children with FSGS present with steroid-resistant nephrotic syndrome. - _____ in conjunction with nephrotic syndrome in an adolescent is more likely to be FSGS than MCN - Tx: Often difficult. - Typically, high-dose IV _______ combined with another immunosuppressive agent is used to tx FSGS presenting with significant symptoms. - Tx includes ACE inhibitors, angiotensin 2 blockers, and addressing the underlying causes.

Answer 45

Focal Segmental Glomerulosclerosis (FSGS) - Most common glomerular cause of ESRD in childhood (overall, congenital anomalies are the #1 cause of ESRD in childhood). 2nd most common cause of NS in childhood. - Pt: - Most children with FSGS present with steroid-resistant nephrotic syndrome. - HTN in conjunction with nephrotic syndrome in an adolescent is more likely to be FSGS than MCN - Tx: Often difficult. - Typically, high-dose IV methylprednisolone combined with another immunosuppressive agent is used to tx FSGS presenting with significant symptoms. - Tx includes ACE inhibitors, angiotensin 2 blockers, and addressing the underlying causes.

Question 46

Membranous Nephropathy - Characterized by immune deposits (IgG and C3) in the basement membrane or subepithelial area of glomerular capillary wall - Rare cause of NS in children or adolescents - Tx: - Tx those with severe disease or persistent nephrotic syndrome with corticosteroids along with cyclosporine or cyclophosphamide.

Answer 46

Membranous Nephropathy - Characterized by immune deposits (IgG and C3) in the basement membrane or subepithelial area of glomerular capillary wall - Rare cause of NS in children or adolescents - Tx: - Tx those with severe disease or persistent nephrotic syndrome with corticosteroids along with cyclosporine or cyclophosphamide.

Question 47

Positive blood on dipstick - Consider hemoglobulin, hemoglobulinuria, or myoglobinuria - Blood (Dipstick really measures Heme) - Dipstick + with RBCs = ____ - Dipstick + without RBCs = ___ or ____ - Confirm with a urine _____ test, which precipitates hemoglobin but not myoglobin.

Answer 47

Positive blood on dipstick - Consider hemoglobulin, hemoglobulinuria, or myoglobinuria - Blood (Dipstick really measures Heme) - Dipstick + with RBCs = hematuria - Dipstick + without RBCs = hemoglobinuria or myoglobinuria - Confirm with a urine ammonium sulfate test, which precipitates hemoglobin but not myoglobin.

Question 48

Microscopic Hematuria = positive urine dipstick for heme AND >__ rbc/hpf

Answer 48

Microscopic Hematuria = positive urine dipstick for heme AND >5 rbc/hpf

Question 49

Persistent asymptomatic hematuria in an otherwise healthy child can be attributed to the following causes: - ____ - _____ - _____ - _____

Answer 49

Persistent asymptomatic hematuria in an otherwise healthy child can be attributed to the following causes: - Idiopathic hypercalciuria (diagnosed with spot urine Ca:Cr (mg:mg) >0.2 in a child >2-3yo) - IgA nephropathy - Thin basement membrane nephropathy (benign familial) - Early Alport syndrome (check family hx for early hearing loss or renal failure - esp males in family)

Question 50

Glomerulonephritis - Pt - Hematuria +/- RBC casts - Variable proteinuria - Edema - Hypertension (often) - Triad of ___, ___, and ___ - Key tool is serum complement level - Systemic: SLE - C3 AND C4 will be low in ____. - Isolated renal disease: Post-infectious strep, MPGN - C3 will likely be low in ___ and ___. - C3 levels return to normal by 12 weeks after presentation in ____ - ____: C3 levels are PERISTENTLY low - If you were having PMS you won’t “complement people”: Post-infectious strep, MPGN, SLE - The level of total complement activity (____ is decreased in patients with hypocomplementemic GN and normal in normocomplementemic GN

Answer 50

Glomerulonephritis - Pt - Hematuria +/- RBC casts - Variable proteinuria - Edema - Hypertension (often) - Triad of coca-cola urine, HTN, and azotemia - Key tool is serum complement level - Systemic: SLE - C3 AND C4 will be low in lupus. - Isolated renal disease: Post-infectious strep, MPGN - C3 will likely be low in post-infectious GN and MPGN. - C3 levels return to normal by 12 weeks after presentation in PIGN - MPGN: C3 levels are PERSISTENTLY low - If you were having PMS you won’t “complement people”: Post-infectious strep, MPGN, SLE - The level of total complement activity (CH50) is decreased in patients with hypocomplementemic GN and normal in normocomplementemic GN

Question 51

Clue to help differentiate the cause of hematuria is the latency bw the disease and presentation of GN: ___ days for PIGN due to GABHS skin infection ___ days for PIGN due to GABHS pharyngitis ___ days for IgA nephropathy

Answer 51

Clue to help differentiate the cause of hematuria is the latency bw the disease and presentation of GN: 21 days for PIGN due to GABHS skin infection 10 days for PIGN due to GABHS pharyngitis 0-5 days for IgA nephropathy

Question 52

Acute Postinfectious Glomerulonephritis - Most common primary acute glomerulopathy in children - Pt: - Classic nephritis (gross hematuria, edema, hypertension, renal insufficiency). - Edema and gross hematuria are the most common presenting symptoms/signs. - Latency between pharyngitis and APSGN is 1-2 weeks. - Latency between skin infection and APSGN is 3-6 weeks. - Labs - LOW serum C3 level remains low for ____vweeks and eventually normalize by 6-8 weeks. NORMAL C4 level - Tests are not sensitive or specific enough - ____ is best antibody titer, best specificity and sensitivity - ____ (but many healthy children with strep in throats and can have elevated ASO titers even without acute infection) - Streptozyme test - No indication for renal biopsy - Tx: - Resolves spontaneously. - Supportive care - HTN and edema, resulting from sodium and fluid retention, are best treated using a potent diuretic such as furosemide. - Penicillin, cephalosporin or macrolide to eradicate nephritogenic strains of strep - Antibiotic therapy does not prevent ____ but does prevent ______ and the spread of nephritogenic strain to others

Answer 52

Acute Postinfectious Glomerulonephritis - Most common primary acute glomerulopathy in children - Pt: - Classic nephritis (gross hematuria, edema, hypertension, renal insufficiency). - Edema and gross hematuria are the most common presenting symptoms/signs. - Latency between pharyngitis and APSGN is 1-2 weeks. - Latency between skin infection and APSGN is 3-6 weeks. - Labs - LOW serum C3 level remains low for 2-12 weeks and eventually normalize by 6-8 weeks. NORMAL C4 level - Tests are not sensitive or specific enough - DNase B is best antibody titer, best specificity and sensitivity - ASO (streptolysin O) enzyme (but many healthy children with strep in throats and can have elevated ASO titers even without acute infection) - Streptozyme test - No indication for renal biopsy - Tx: - Resolves spontaneously. - Supportive care - HTN and edema, resulting from sodium and fluid retention, are best treated using a potent diuretic such as furosemide. - Penicillin, cephalosporin or macrolide to eradicate nephritogenic strains of strep - Antibiotic therapy does not prevent acute glomerulonephritis but does prevent rheumatic fever and the spread of nephritogenic strain to others

Question 53

Membranoproliferative Glomerulonephritis (MPGN) (aka mesangiocapillary GN) - Pt: - 20-30%: Acute nephritis - 20-40%: Incidental findings of proteinuria and hematuria - 30-50%: Nephrotic syndrome - Lab: C3 levels are PERSISTENTLY low (whereas C3 levels return to normal by 12 weeks after presentation in PIGN) (+/- low C4) - Tx: No definite guidelines. Treatment is determined by the amount of renal dysfunction. - Many children have normal renal function for years without specific therapy. - Some do better if treated with a prolonged course 3-10 years of steroids.

Answer 53

Membranoproliferative Glomerulonephritis (MPGN) (aka mesangiocapillary GN) - Pt: - 20-30%: Acute nephritis - 20-40%: Incidental findings of proteinuria and hematuria - 30-50%: Nephrotic syndrome - Lab: C3 levels are PERSISTENTLY low (whereas C3 levels return to normal by 12 weeks after presentation in PIGN) (+/- low C4) - Tx: No definite guidelines. Treatment is determined by the amount of renal dysfunction. - Many children have normal renal function for years without specific therapy. - Some do better if treated with a prolonged course 3-10 years of steroids.

Question 54

Lupus Nephritis. See “SLE” - Immune complex-mediated tissue injury to the kidney - Labs: - Decreased C3 AND C4 - “Active” urine sediment - RBCs and RBC casts - ______ is most specific for active lupus nephritis - Tx: - Management of milder forms of lupus nephritis is dictated more by the systemic disease symptoms/signs. - In diffuse proliferative lupus nephritis, a combination of prednisone and cyclophosphamide or mycophenolate mofetil

Answer 54

Lupus Nephritis. See “SLE” - Immune complex-mediated tissue injury to the kidney - Labs: - Decreased C3 AND C4 - “Active” urine sediment - RBCs and RBC casts - Anti-dsDNA is most specific for active lupus nephritis - Tx: - Management of milder forms of lupus nephritis is dictated more by the systemic disease symptoms/signs. - In diffuse proliferative lupus nephritis, a combination of prednisone and cyclophosphamide or mycophenolate mofetil

Question 55

``` IgA Nephropathy (Berger Disease) - Most common cause of gross hematuria. Also the most common primary chronic glomerulopathy worldwide. ``` - Pt: - A) RECURRENT episodes painless gross hematuria, usually during URIs (concomitant or within 1-2 days of infection). - Hematuria occurs _____ with infection. - B) Incidental finding of microhematuria and mild proteinuria. - C3 levels are normal. Serum IgA are not helpful in diagnosing IgA nephropathy. - Tx: Controversial - Benign for many patients, resolves on own. - Some at risk for ESRD - 4 considerations: - Give ACE inhibitors to HTNive patients - Treatment with w-3 fatty acids (fish oils) may slow progression to renal insufficiency - Steroids may be of benefit and typically used empirically - Intensive immunosuppressive is helpful for those with cescentic disease

Answer 55

``` IgA Nephropathy (Berger Disease) - Most common cause of gross hematuria. Also the most common primary chronic glomerulopathy worldwide. ``` - Pt: - A) RECURRENT episodes painless gross hematuria, usually during URIs (concomitant or within 1-2 days of infection). - Hematuria occurs CONCOMITANT with infection. - B) Incidental finding of microhematuria and mild proteinuria. - C3 levels are normal. Serum IgA are not helpful in diagnosing IgA nephropathy. - Tx: Controversial - Benign for many patients, resolves on own. - Some at risk for ESRD - 4 considerations: - Give ACE inhibitors to HTNive patients - Treatment with w-3 fatty acids (fish oils) may slow progression to renal insufficiency - Steroids may be of benefit and typically used empirically - Intensive immunosuppressive is helpful for those with cescentic disease

Question 56

Henoch-Schonlein Purpura Nephritis - Henoch-Schonlein Purpura Nephritis - Pt - 4 classic features - Purpuric rash (esp over buttocks, abdomen, and lower extremities). - Abdominal pain - Arthralgias - Glomerulonephritis with IgA deposition - ARENA: ___, ___, ___, ___, ___ - Work up - The serum IgA level is elevated in approx half of all pts with HSP but is not helpful in diagnosis. - Prognosis: - Typically a self-limiting illness. - Tx: - Management depends on the severity of presentation, which is based on the number of crescents seen on biopsy. - Initial treatment is supportive care - Treat children who have a large percentage of crescents on biopsy with steroids and/or cyclophosphamide or azathioprine - Oral steroids are often used for relief of rash or GI symptoms

Answer 56

Henoch-Schonlein Purpura Nephritis - Henoch-Schonlein Purpura Nephritis - Pt - 4 classic features - Purpuric rash (esp over buttocks, abdomen, and lower extremities). - Abdominal pain - Arthralgias - Glomerulonephritis with IgA deposition - ARENA: Abdominal pain, rash, edema, nephritis, arthritis - Work up - The serum IgA level is elevated in approx half of all pts with HSP but is not helpful in diagnosis. - Prognosis: - Typically a self-limiting illness. - Tx: - Management depends on the severity of presentation, which is based on the number of crescents seen on biopsy. - Initial treatment is supportive care - Treat children who have a large percentage of crescents on biopsy with steroids and/or cyclophosphamide or azathioprine - Oral steroids are often used for relief of rash or GI symptoms

Question 57

``` Rapidly Progressive (Crescentic) Glomerulonephritis - Acute presentation of a number of aggressive glomerular disorders, with the common abnormality being the presence of crescents in the majority of glomeruli. ``` - Path - 1) Anti-GBM disease (5-7%) - 2) Immune complex nephritis (80-85%) - 3) Pauci-immune disease (13-15%) - Pt: Gross hematuria, edema, anemia, HTN - Dx: - ANCA allows earlier noninvasive diagnosis of pauci-immune disease - Tx: - Aim at underlying cause - Prognosis is poor; almost all disorders progress to ESRD unless aggressive tx instituted - Immunosuppressive therapy with high dose IV steroids is cornerstone if RPGN is diagnosed early

Answer 57

``` Rapidly Progressive (Crescentic) Glomerulonephritis - Acute presentation of a number of aggressive glomerular disorders, with the common abnormality being the presence of crescents in the majority of glomeruli. ``` - Path - 1) Anti-GBM disease (5-7%) - 2) Immune complex nephritis (80-85%) - 3) Pauci-immune disease (13-15%) - Pt: Gross hematuria, edema, anemia, HTN - Dx: - ANCA allows earlier noninvasive diagnosis of pauci-immune disease - Tx: - Aim at underlying cause - Prognosis is poor; almost all disorders progress to ESRD unless aggressive tx instituted - Immunosuppressive therapy with high dose IV steroids is cornerstone if RPGN is diagnosed early

Question 58

Antiglomerular Basement Membrane (Anti-GBM) Disease - Pt: - A) Isolated renal disease - B) _____ syndrome if have pulmonary hemorrhage - Acute nephritis and progress to renal failure within weeks. Hemoptysis is often the presenting pulmonary symptom. - Dx: An anti-GBM antibody is found in plasma 90% of time. Renal biopsy confirms diagnosis with crescentic nephritis and linear deposits of IgG and C3 in the GBM. - Tx: - Plasmapheresis is the main therapy especially if pulmonary disease is a component. Most patients are also treated with corticosteroids and immunosuppressive therapy. - In most pts, anti-GBM antibody production is short-lived, lasting 8-14 weeks, but it quickly causes irreversible renal injury.

Answer 58

Antiglomerular Basement Membrane (Anti-GBM) Disease - Pt: - A) Isolated renal disease - B) Goodpasture syndrome if have pulmonary hemorrhage - Acute nephritis and progress to renal failure within weeks. Hemoptysis is often the presenting pulmonary symptom. - Dx: An anti-GBM antibody is found in plasma 90% of time. Renal biopsy confirms diagnosis with crescentic nephritis and linear deposits of IgG and C3 in the GBM. - Tx: - Plasmapheresis is the main therapy especially if pulmonary disease is a component. Most patients are also treated with corticosteroids and immunosuppressive therapy. - In most pts, anti-GBM antibody production is short-lived, lasting 8-14 weeks, but it quickly causes irreversible renal injury.

Question 59

ANCA-Positive Focal Necrotizing Glomerulonephritis - 1) Eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome) - Pt: Allergic asthma, followed by vasculitis (characterized by eosinophilic infiltrates and peripheral blood eosinophilia) - 2) Granulomatosis with polyangiitis (____ granulomatosis) - Rare in children. - Path: Lung disease due to necrotizing granulomas that form pulmonary nodules and cavitary lesions. Granulomas also form in the nasal passages, sinuses, and ears. - Pt: - Flu-like illness and systemic symptoms of myalgia, fever, anorexia, and weight loss - Lung disease is typical and can vary from mild disease to life-threatening pulmonary hemorrhage - The nephritis is generally severe, with hematuria, proteinuria, and renal insufficiency. The renal disease, though, is NOT granulomatous in nature. - Dx: The ___-ANCA test is sensitive and specific for GPA. - Tx: ____ and _____. TMP/SMX for PCP prophylaxis - 3) Microscopic polyangiitis - Elevated serum titers of antimyeloperoxidase ANCA antibodies (perinuclear pattern) - Pt: - Similar to granulomatosis with polyangiitis but do NOT have granulomatous disease of the lungs - C-ANCA (related to PR3) and P-ANCA (related to myeloperoxidase) are both commonly seen - Tx and prognosis are similar to GPA

Answer 59

ANCA-Positive Focal Necrotizing Glomerulonephritis - 1) Eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome) - Pt: Allergic asthma, followed by vasculitis (characterized by eosinophilic infiltrates and peripheral blood eosinophilia) - 2) Granulomatosis with polyangiitis (Wegener granulomatosis) - Rare in children. - Path: Lung disease due to necrotizing granulomas that form pulmonary nodules and cavitary lesions. Granulomas also form in the nasal passages, sinuses, and ears. - Pt: - Flu-like illness and systemic symptoms of myalgia, fever, anorexia, and weight loss - Lung disease is typical and can vary from mild disease to life-threatening pulmonary hemorrhage - The nephritis is generally severe, with hematuria, proteinuria, and renal insufficiency. The renal disease, though, is NOT granulomatous in nature. - Dx: The c-ANCA test is sensitive and specific for GPA. - Tx: Cyclophosphamide and high-dose steroids. TMP/SMX for PCP prophylaxis - 3) Microscopic polyangiitis - Elevated serum titers of antimyeloperoxidase ANCA antibodies (perinuclear pattern) - Pt: - Similar to granulomatosis with polyangiitis but do NOT have granulomatous disease of the lungs - C-ANCA (related to PR3) and P-ANCA (related to myeloperoxidase) are both commonly seen - Tx and prognosis are similar to GPA

Question 60

Alport syndrome (Hereditary nephritis) - Inherited disorder of basement membrane collagen characterized by involvement of the ____ (always), ___ (often), and ___ (occasionally) - ____Inheritance?? is most common form of inheritance. Followed by AR - Path: Gene defects of the alpha chain of collagen Type ___ of basement membranes - Pt: - ____ is the earliest manifestation and universal symptom in patients with AS. - Extrarenal manifestations - Bilateral _____ develops over the 1st decade - Ocular defects - ___ is pathognomonic of AS. - Consider diagnosis in any child with kidney problems (ie hematuria), ocular problems, and hearing problems or a family hx of early hearing loss or ESRD. - Tx: - No specific therapy, though early ______ delays progression of renal failure in some cases.

Answer 60

Alport syndrome (Hereditary nephritis) - Inherited disorder of basement membrane collagen characterized by involvement of the kidneys (always), ears (often), and eyes (occasionally) - XLD is most common form of inheritance. Followed by AR - Path: Gene defects of the alpha chain of collagen Type 4 of basement membranes - Pt: - Hematuria is the earliest manifestation and universal symptom in patients with AS. - Extrarenal manifestations - Bilateral sensorineural hearing loss develops over the 1st decade - Ocular defects - Anterior lenticonus is pathognomonic of AS. - Consider diagnosis in any child with kidney problems (ie hematuria), ocular problems, and hearing problems or a family hx of early hearing loss or ESRD. - Tx: - No specific therapy, though early ACE inhibitor delays progression of renal failure in some cases.

Question 61

Thin Basement Membrane Nephropathy - ____Inheritance? condition in which multiple family members have hematuria (with or wo RBC casts) but no family hx of kidney failure. - Because disease is confined to kidney and has no long-term sequelae, it differs from Alport syndrome. However, like pts with Alport syndrome, many have an abnormality in ___ and ___ genes that encode for Type __ Collagen. - Tx: _____

Answer 61

Thin Basement Membrane Nephropathy - AD condition in which multiple family members have hematuria (with or wo RBC casts) but no family hx of kidney failure. - Because disease is confined to kidney and has no long-term sequelae, it differs from Alport syndrome. However, like pts with Alport syndrome, many have an abnormality in COL4A3 and COL4A4 genes that encode for Type 4 Collagen. - Tx: None required, prognosis is good. However, long-term surveillance as some patients go on to develop proteinuria and progressive renal failure over time.

Question 62

Nail-Patella Syndrome - ___inheritance? - Gene defect is in the LMX1B on chromosome _ - Pt: - Hypoplasia or absence of patellae - Dystrophic nails - Dysplasia of elbows - ___ disease

Answer 62

Nail-Patella Syndrome - Autosomal dominant - Gene defect is in the LMX1B on chromosome 9 - Pt: - Hypoplasia or absence of patellae - Dystrophic nails - Dysplasia of elbows - Renal disease

Question 63

Multicystic Dysplastic Kidney (MCDC) - Pt: - Usually asymptomatic. - Usually unilateral. No kidney function. - Dx: - Most detected _____ - Classic findings on renal US include multiple ____ cysts with intervening dysplastic renal tissue. - Differs from ADPKD bc MCDK is_____. ADPKD starts slowly and takes decades to develop many cysts - Tx: In most cases, the affected kidney will _______, so _____ management is appropriate. - There is a small risk for malignant transformation in the MCDK; thus, observation with _____ is important to confirm involution and is preferred over nephrectomy. - Serial US of the unaffected kidney to assure proper growth (compensatory hypertrophy)

Answer 63

Multicystic Dysplastic Kidney (MCDC) - Pt: - Usually asymptomatic. - Usually unilateral. No kidney function. - Dx: - Most detected antenatally - Classic findings on renal US include multiple noncommunicating cysts with intervening dysplastic renal tissue. - Differs from ADPKD bc MCDK is present at birth. ADPKD starts slowly and takes decades to develop many cysts - Tx: In most cases, the affected kidney will undergo spontaneous involution, so conservative management is appropriate. - There is a small risk for malignant transformation in the MCDK; thus, observation with serial US is important to confirm involution and is preferred over nephrectomy. - Serial US of the unaffected kidney to assure proper growth (compensatory hypertrophy)

Question 64

Autosomal Recessive Polycystic Kidney Disease (ARPKD) - Age at presentation is relatively evenly divided into ⅓ each before 1 year, between 1-20 years, and after age 20 years - Pt: - _____ occurs because of intrauterine renal failure. “_____ facies”: low-set ears, flat nose, retracted chin, limb abnormalities. - Results in bilateral palpable kidney enlargement and transforms collecting ducts into fusiform cysts - A finding of palpable kidney is the most common feature of ARPKD, followed by HTN and recurrent UTIs. - Complications: ESRD, anemia, growth failure, osteodystrophy, infection is typical, over time dilation of intrahepatic bile ducts (Caroli disease) - _____ are the most commonly seen extrarenal manifestations of ARPKD. - _____ is universal - US shows ____ kidneys bilaterally with hyperechogenicity (“salt and pepper”) and decreased corticomedullary differentiation Later, rounded cysts may appear. - Tx: - Control HTN, but this can be difficult. - It is vital to be vigilant in looking for UTIs. - Portal HTN due to hepatic fibrosis is a major complication that must be dealt with - Eventually renal and/or liver transplant is required.

Answer 64

Autosomal Recessive Polycystic Kidney Disease (ARPKD) - Age at presentation is relatively evenly divided into ⅓ each before 1 year, between 1-20 years, and after age 20 years - Pt: - Oligohydramnios occurs because of intrauterine renal failure. “Potter facies”: low-set ears, flat nose, retracted chin, limb abnormalities. - Results in bilateral palpable kidney enlargement and transforms collecting ducts into fusiform cysts - A finding of palpable kidney is the most common feature of ARPKD, followed by HTN and recurrent UTIs. - Complications: ESRD, anemia, growth failure, osteodystrophy, infection is typical, over time dilation of intrahepatic bile ducts (Caroli disease) - Abnormalities of the liver are the most commonly seen extrarenal manifestations of ARPKD. - Congenital hepatic fibrosis is universal - US shows enlarged kidneys bilaterally with hyperechogenicity (“salt and pepper”) and decreased corticomedullary differentiation Later, rounded cysts may appear. - Tx: - Control HTN, but this can be difficult. - It is vital to be vigilant in looking for UTIs. - Portal HTN due to hepatic fibrosis is a major complication that must be dealt with - Eventually renal and/or liver transplant is required.

Question 65

Autosomal Dominant Polycystic Kidney Disease - Most common disease of AD inheritance in US - Pt: - Usually ____. - Microscopic hematuria, proteinuria, HTN, family hx of kidney disease - The most common extrarenal manifestation seen in children with ADPKD is _____. Berry aneurysms can be fatal if ruptured and cause intracranial bleeding. - Dx: Positive family history and renal ultrasonography that shows bilateral cysts in enlarged kidneys - Renal macrocysts are visible on renal US - Identification of a child with multiple kidney cysts requires a complete family hx and an evaluation of both parents. - For child who has parents with the disease, a single cyst on child’s US is highly predictive of ADPKD

Answer 65

Autosomal Dominant Polycystic Kidney Disease - Most common disease of AD inheritance in US - Pt: - Usually asymptomatic. - Microscopic hematuria, proteinuria, HTN, family hx of kidney disease - The most common extrarenal manifestation seen in children with ADPKD is cerebral aneurysm. Berry aneurysms can be fatal if ruptured and cause intracranial bleeding. - Dx: Positive family history and renal ultrasonography that shows bilateral cysts in enlarged kidneys - Renal macrocysts are visible on renal US - Identification of a child with multiple kidney cysts requires a complete family hx and an evaluation of both parents. - For child who has parents with the disease, a single cyst on child’s US is highly predictive of ADPKD

Question 66

Juvenile Nephronophthisis Type 1 (NPHP1) - Ciliopathy (problem of cilia of kidney in interstitial compartment). Interstitial disease. - Pt: - Polyuria, polydipsia, anemia, FTT, enuresis, nocturia. - Dx: Renal US shows poor differentiation between cortical and medullary areas of the kidney. After 9 years of age, cysts are common at the corticomedullary border. - Slightly small kidneys with corticomedullary cyst on RU by 8-10yo

Answer 66

Juvenile Nephronophthisis Type 1 (NPHP1) - Ciliopathy (problem of cilia of kidney in interstitial compartment). Interstitial disease. - Pt: - Polyuria, polydipsia, anemia, FTT, enuresis, nocturia. - Dx: Renal US shows poor differentiation between cortical and medullary areas of the kidney. After 9 years of age, cysts are common at the corticomedullary border. - Slightly small kidneys with corticomedullary cyst on RU by 8-10yo

Question 67

Medullary Sponge Kidney - Congenital structural abnormally of the kidney in medulla - Rare congenital cystic disorder with ectasia of cortical ducts within the inner medulla of the kidney, giving rise to sponge-like appearance - Pt: Frequent UTIs, hematuria, and renal stones - RUS with increased echogenicity. XR show calcifications in these areas. - Tx: - Prognosis is good - Only antibiotics for UTIs and therapy for stone formation

Answer 67

Medullary Sponge Kidney - Congenital structural abnormally of the kidney in medulla - Rare congenital cystic disorder with ectasia of cortical ducts within the inner medulla of the kidney, giving rise to sponge-like appearance - Pt: Frequent UTIs, hematuria, and renal stones - RUS with increased echogenicity. XR show calcifications in these areas. - Tx: - Prognosis is good - Only antibiotics for UTIs and therapy for stone formation

Question 68

Bardet-Biedl Syndrome - Path: ____Inheritance??? syndrome - Pt - Obesity, retinitis pigmentosa, hypogenitalism, polydactyly, intellectual disability, cystic dysplasia of the kidney

Answer 68

Bardet-Biedl Syndrome - Path: AR syndrome - Pt - Obesity, retinitis pigmentosa, hypogenitalism, polydactyly, intellectual disability, cystic dysplasia of the kidney

Question 69

ACUTE KIDNEY INJURY - Neonatal serum creatinine concentration (usually <1.0mg/dL) is reflective of maternal serum creatinine concentration. Gradually declines to gradually declines to neonatal levels (0.2-0.4mg/dL) in the first 1-2 weeks after birth.

Answer 69

ACUTE KIDNEY INJURY - Neonatal serum creatinine concentration (usually <1.0mg/dL) is reflective of maternal serum creatinine concentration. Gradually declines to gradually declines to neonatal levels (0.2-0.4mg/dL) in the first 1-2 weeks after birth.

Question 70

Neonatal AKI - _______ is the most common cause of noncatheter-associated thrombosis in the newborn - Flank mass, thrombocytopenia, and hematuria are the classic features ass with renal vein thrombosis - US of kidney is imaging modality of choice to diagnose. In early stages of thrombosis, the kidneys appear swollen and echogenic. The subsequent loss of corticomedullary differentiation followed by scarring and decreased renal size occurs gradually.

Answer 70

Neonatal AKI - Renal vein thrombosis is the most common cause of noncatheter-associated thrombosis in the newborn - Flank mass, thrombocytopenia, and hematuria are the classic features ass with renal vein thrombosis - US of kidney is imaging modality of choice to diagnose. In early stages of thrombosis, the kidneys appear swollen and echogenic. The subsequent loss of corticomedullary differentiation followed by scarring and decreased renal size occurs gradually.

Question 71

AKI - Def: Acute decrease in GFR (kidney function) resulting in an increase in serum Cr - Treatment points: - Furosemide does not help preserve renal function in presence of established AKI, esp if there is no increased UOP after its first use - Always treat the hyperkalemia - In prerenal AKI, these urinary indices reflect the renal tubular reabsorption of filtered sodium as well as water in response to decreased renal perfusion. - BUN-to-creatinine ratio __20:1 - Urine specific gravity __1.020 - Urine sodium __10 mEq/L - Fractional excretion of sodium (FENa) is ___1% - Urine to plasma osmolality is >1.5 - In renal AKI - BUN-to-creatinine ratio is 10:1 to 15:1 - The urine is dilute with specific gravity __1.010 - Urine sodium level __40 mEq/L - FENa __2% - Urine-plasma osmolality is <1.5

Answer 71

AKI - Def: Acute decrease in GFR (kidney function) resulting in an increase in serum Cr - Treatment points: - Furosemide does not help preserve renal function in presence of established AKI, esp if there is no increased UOP after its first use - Always treat the hyperkalemia - In prerenal AKI, these urinary indices reflect the renal tubular reabsorption of filtered sodium as well as water in response to decreased renal perfusion. - BUN-to-creatinine ratio >20:1 - Urine specific gravity >1.020 - Urine sodium <10 mEq/L - Fractional excretion of sodium (FENa) is <1% - Urine to plasma osmolality is >1.5 - In renal AKI - BUN-to-creatinine ratio is 10:1 to 15:1 - The urine is dilute with specific gravity <1.010 - Urine sodium level >40 mEq/L - FENa >2% - Urine-plasma osmolality is <1.5

Question 72

- Prerenal: - Etiologies - Volume depletion: Dehydration, blood loss, hemorrhage, diuretics, burns, shock - Decreased intravascular/expanded total body volume: HF, cirrhosis/hepatorenal syndrome, nephrotic syndrome, renal artery stenosis, vascular problems (large blood vessels, renal artery emboli, thrombosis, or stenosis) - Pts with severe proteinuria (or hypoalbuminemia of any cause such as cirrhosis) are usually volume depleted and therefore very susceptible to developing prerenal failure. - Peripheral vasodilation: Sepsis, anaphylaxis, vasodilator drugs, IL-2 - Medications: Diuretics (most common), ACE inhibitors, NSAIDs, cyclosporine, tacrolimus - NSAIDs are particularly likely to cause prerenal azotemia in pts whose renal function is compromised. - Lab: - High BUN:Cr ratio - Elevated urine osmolality (UOsm >500 mOsm/kg) - Low urine sodium (UNa <20mEq/L), indicates normal tubular function and attempt by kidney to conserve volume - Concentrated urine - FeNa <1% (FeNa only used in AKI and not when on diuretics) - Urine sediment: Typically normal, can show granular casts or hyaline casts - Tx: If due to dehydration, tx with aggressive volume expansion with isotonic fluids (NS or LR) rapidly: a 20ml/kg bolus over 20-30 mins - Ppx: Do not prescribe ibuprofen to febrile patients who are dehydrated or likely to develop dehydration as they are at higher risk!

Answer 72

- Prerenal: - Etiologies - Volume depletion: Dehydration, blood loss, hemorrhage, diuretics, burns, shock - Decreased intravascular/expanded total body volume: HF, cirrhosis/hepatorenal syndrome, nephrotic syndrome, renal artery stenosis, vascular problems (large blood vessels, renal artery emboli, thrombosis, or stenosis) - Pts with severe proteinuria (or hypoalbuminemia of any cause such as cirrhosis) are usually volume depleted and therefore very susceptible to developing prerenal failure. - Peripheral vasodilation: Sepsis, anaphylaxis, vasodilator drugs, IL-2 - Medications: Diuretics (most common), ACE inhibitors, NSAIDs, cyclosporine, tacrolimus - NSAIDs are particularly likely to cause prerenal azotemia in pts whose renal function is compromised. - Lab: - High BUN:Cr ratio - Elevated urine osmolality (UOsm >500 mOsm/kg) - Low urine sodium (UNa <20mEq/L), indicates normal tubular function and attempt by kidney to conserve volume - Concentrated urine - FeNa <1% (FeNa only used in AKI and not when on diuretics) - Urine sediment: Typically normal, can show granular casts or hyaline casts - Tx: If due to dehydration, tx with aggressive volume expansion with isotonic fluids (NS or LR) rapidly: a 20ml/kg bolus over 20-30 mins - Ppx: Do not prescribe ibuprofen to febrile patients who are dehydrated or likely to develop dehydration as they are at higher risk!

Question 73

- Intrinsic renal (Renal AKI) - 1. Acute tubular necrosis (Most common cause of AKI) - Hallmark of disease is tubular dysfunction. - Path: - Most commonly due to renal hypoperfusion, usually caused by surgery or shock - Extrinsic or intrinsic toxins: - Myoglobinuria (rhabdomyolysis): - Muscle injury can lead to rhabdomyolysis, which can lead to ATN. The myoglobin released due to rhabdomyolysis can cause ATN if urine is acidic AND the patient is volume depleted. - Labs: - Rhabdomyolysis-associated Hypocalcemia due to 1) decreased production of 1,25-OH2-vit D due to renal injury and 2) hyperphosphatemia due to renal injury and tissue breakdown - Dx: Very elevated serum CK - If CK is unknown, consider rhabdomyolysis by finding high K, high Phos, very high uric acid, and low calcium, along with a disproportionate increase in Cr compared to the BUN. - Classic when urine dips heme-positive but without RBCs on microscopy. - Tx: Medical emergency! If left untreated, shock and/or arrhythmia from hyperkalemia can result - 1) Aggressive intravascular volume replacement with NS - 2) Alkalinization of urine. - The pt is at risk for hypercalcemia during recovery. For this reason, do not treat hypocalcemia unless severe or patient is symptomatic. DO treat arrhythmias by administering calcium to stabilize cardiac membrane. - Heavy metals - Contrast dye - Drugs (amphotericin B, aminoglycoside, cisplatin, cyclosporine, IV contrast, vancomycin) - Amphotericin B is esp likely to cause ATN. It has a direct nephrotoxic effect and can cause a Type 1 RTA. - Aminoglycosides cause proximal tubule damage, resulting in a nonoliguric ATN! Aminoglycosides also cause ______________. Know: ATN is commonly delayed 7-10 days after the start of therapy, and early therapeutic levels are not a guarantee of safety. - Cisplatin also causes a magnesiuria, resulting in _______. (This is its most-asked about side effect) - Cyclosporine causes renal toxicity. - IV contrast maternal can cause AKI.' - Tx: Treat precipitating cause and any hyperkalemia. Match fluid input with output (urinary plus insensible losses) and encourage eating - which decreases catabolism! - Urine sediment: Hallmark is large, _______ casts with red/brown urine, which is nonspecific but very sensitive - Note that oliguria is not require for the diagnosis of ATN

Answer 73

- Intrinsic renal (Renal AKI) - 1. Acute tubular necrosis (Most common cause of AKI) - Hallmark of disease is tubular dysfunction. - Path: - Most commonly due to renal hypoperfusion, usually caused by surgery or shock - Extrinsic or intrinsic toxins: - Myoglobinuria (rhabdomyolysis): - Muscle injury can lead to rhabdomyolysis, which can lead to ATN. The myoglobin released due to rhabdomyolysis can cause ATN if urine is acidic AND the patient is volume depleted. - Labs: - Rhabdomyolysis-associated Hypocalcemia due to 1) decreased production of 1,25-OH2-vit D due to renal injury and 2) hyperphosphatemia due to renal injury and tissue breakdown - Dx: Very elevated serum CK - If CK is unknown, consider rhabdomyolysis by finding high K, high Phos, very high uric acid, and low calcium, along with a disproportionate increase in Cr compared to the BUN. - Classic when urine dips heme-positive but without RBCs on microscopy. - Tx: Medical emergency! If left untreated, shock and/or arrhythmia from hyperkalemia can result - 1) Aggressive intravascular volume replacement with NS - 2) Alkalinization of urine. - The pt is at risk for hypercalcemia during recovery. For this reason, do not treat hypocalcemia unless severe or patient is symptomatic. DO treat arrhythmias by administering calcium to stabilize cardiac membrane. - Heavy metals - Contrast dye - Drugs (amphotericin B, aminoglycoside, cisplatin, cyclosporine, IV contrast, vancomycin) - Amphotericin B is esp likely to cause ATN. It has a direct nephrotoxic effect and can cause a Type 1 RTA. - Aminoglycosides cause proximal tubule damage, resulting in a nonoliguric ATN! Aminoglycosides also cause hypomagnesemia. Know: ATN is commonly delayed 7-10 days after the start of therapy, and early therapeutic levels are not a guarantee of safety. - Cisplatin also causes a magnesiuria, resulting in hypomagnesemia. (This is its most-asked about side effect) - Cyclosporine causes renal toxicity. - IV contrast maternal can cause AKI.' - Tx: Treat precipitating cause and any hyperkalemia. Match fluid input with output (urinary plus insensible losses) and encourage eating - which decreases catabolism! - Urine sediment: Hallmark is large, muddy-brown granular casts with red/brown urine, which is nonspecific but very sensitive - Note that oliguria is not require for the diagnosis of ATN

Question 74

- 2. Glomerulopathies - Urine sediment: Nephritic (hematuria w RBC casts, sometimes pyuria with WBC casts) and nephrotic (oval fat bodies, proteinuria) - 3. Acute/Allergic interstitial nephritis - 4. Vascular injury as in HUS (thrombotic microangiopathy) - 5. Intratubular obstruction:

Answer 74

- 2. Glomerulopathies - Urine sediment: Nephritic (hematuria w RBC casts, sometimes pyuria with WBC casts) and nephrotic (oval fat bodies, proteinuria) - 3. Acute/Allergic interstitial nephritis - 4. Vascular injury as in HUS (thrombotic microangiopathy) - 5. Intratubular obstruction:

Question 75

- 3) Postrenal (obstruction to the flow of the urine) - Path: Bladder outlet obstruction. Can be caused by bilateral ureteral obstruction - Labs: - BUN:Cr is elevated because urea diffuses back into the system. - Urine sediment: Blood is common. WBC casts if due to papillary necrosis - You can see papillary necrosis in pyogenic kidneys with postrenal obstruction or sterile pyuria with WBC casts in the urine sediment. - Tx: Catheterization

Answer 75

- 3) Postrenal (obstruction to the flow of the urine) - Path: Bladder outlet obstruction. Can be caused by bilateral ureteral obstruction - Labs: - BUN:Cr is elevated because urea diffuses back into the system. - Urine sediment: Blood is common. WBC casts if due to papillary necrosis - You can see papillary necrosis in pyogenic kidneys with postrenal obstruction or sterile pyuria with WBC casts in the urine sediment. - Tx: Catheterization

Question 76

Acute Interstitial Nephritis - Idiosyncratic drug-induced (hypersensitivity) reaction - Antibiotics (beta-lactams, penicillin derivatives, cephalosporins, TMP/SMX, rifampin, fluoroquinolones), NSAIDs, cimetidine, thiazides, phenytoine, allopurinol, carbamazepine - Pt: - Antibiotics cause a classic triad of ___, ___, and ___. It is an idiosyncratic response to the antibiotic and is not related to the amount of antibiotic or the antibiotic’s duration of use! - NSAID-induced AIN is different in that the NSAIDs are typically ingested for months before symptoms occur. The rash, fever, and eosinophilia may not occur. - Contrary to all other types of AIN, with NSAID-induced AIN, there is often nephrotic-range proteinuria with minimal glomerular changes. - Note: NSAID-induced AIN (an intrinsic AKI) has a different mechanism than NSAID-induced prerenal AKI. The latter causes vasoconstriction due to prostaglandin blockage; not that dehydrated pts are at higher risk to develop this complication. So remember not to prescribe ibuprofen to febrile pts who are dehydrated or likely to develop dehydration. - Urine sediment: - ______, RBCs, WBCs, and WBC casts. - Differs from GN in that it does not have significant albuminuria, RBC casts, or oval fat bodies. - Though more specific than eosinophilia, eosinophiluria is not a very sensitive finding. - Slight proteinuria (<1-1.5g/day); proteinuria is marked by excessive _______ that is excreted from tubules - Tx - Discontinue offending agent. - If AIN persists for 3-7 days after discontinuing the drug, initiate glucocorticoid therapy for 1-2 weeks (unless offending drug is an NSAID, then do not use glucocorticoid tx)

Answer 76

Acute Interstitial Nephritis - Idiosyncratic drug-induced (hypersensitivity) reaction - Antibiotics (beta-lactams, penicillin derivatives, cephalosporins, TMP/SMX, rifampin, fluoroquinolones), NSAIDs, cimetidine, thiazides, phenytoine, allopurinol, carbamazepine - Pt: - Antibiotics cause a classic triad of fever, rash, and eosinophilia. It is an idiosyncratic response to the antibiotic and is not related to the amount of antibiotic or the antibiotic’s duration of use! - NSAID-induced AIN is different in that the NSAIDs are typically ingested for months before symptoms occur. The rash, fever, and eosinophilia may not occur. - Contrary to all other types of AIN, with NSAID-induced AIN, there is often nephrotic-range proteinuria with minimal glomerular changes. - Note: NSAID-induced AIN (an intrinsic AKI) has a different mechanism than NSAID-induced prerenal AKI. The latter causes vasoconstriction due to prostaglandin blockage; not that dehydrated pts are at higher risk to develop this complication. So remember not to prescribe ibuprofen to febrile pts who are dehydrated or likely to develop dehydration. - Urine sediment: - Eosinophils, RBCs, WBCs, and WBC casts. - Differs from GN in that it does not have significant albuminuria, RBC casts, or oval fat bodies. - Though more specific than eosinophilia, eosinophiluria is not a very sensitive finding. - Slight proteinuria (<1-1.5g/day); proteinuria is marked by excessive beta-2 microglobulin that is excreted from tubules - Tx - Discontinue offending agent. - If AIN persists for 3-7 days after discontinuing the drug, initiate glucocorticoid therapy for 1-2 weeks (unless offending drug is an NSAID, then do not use glucocorticoid tx)

Question 77

Chronic Interstitial Nephritis - Consider in patient with history of frequent pain who presents with ____ and an elevated ___ - Remember, if a pt with renal dysfunction and heavy proteinuria, think of GN causing nephrotic syndrome or of NSAID-induced nephritis. - If a pt presents with light proteinuria and active sediment, then consider GN causing nephritis (esp IgA nephropathy) and consider tubulointerstitial disease (eg AIN). - Any time patient is spilling glucose into urine and yet has normal serum glucose, think chronic tubulointerstitial disease (also think of type 2 RTA and pregnancy!).

Answer 77

Chronic Interstitial Nephritis - Consider in patient with history of frequent pain who presents with proteinuria and an elevated creatinine - Remember, if a pt with renal dysfunction and heavy proteinuria, think of GN causing nephrotic syndrome or of NSAID-induced nephritis. - If a pt presents with light proteinuria and active sediment, then consider GN causing nephritis (esp IgA nephropathy) and consider tubulointerstitial disease (eg AIN). - Any time patient is spilling glucose into urine and yet has normal serum glucose, think chronic tubulointerstitial disease (also think of type 2 RTA and pregnancy!).

Question 78

Chronic Kidney Disease - Path: - Renal dysplasia with Congenital anomalies of the kidney and urinary tract (CAKUT) and cystic kidney diseases (nonglomerular CKD) account for nearly 60% of pediatric CKD. - Pt - In early stages of CKD (GFR >60), patients are often asymptomatic. - Primary daytime and nocturnal enuresis in the setting of poor linear growth and proteinuria is concerning for chronic kidney disease. - Dependent edema, nausea/vomiting, fatigue, hematuria, and/or proteinuria - Complications - HTN - Anemia - Poor growth - Bone-calcium metabolic derangements nerve damage - Cognitive delays in young children - Tubulointerstitial injury associated with CAKUT leads to reduced urinary concentration (acquired nephrogenic DI) and these patients usually present with polyuria - Nutritional issues; in particular, short stature/growth restriction - Evaluation - Urinalysis (look for protein) - CBC (look for anemia) - Blood chemistry (look at Cr (high indicates loss of filtering function) - Albumin (low if being lost through kidneys) - Electrolytes (___kalemia and metabolic ____ due to poor renal excretion) - Calcium, phosphate, PTH, and vitamin D levels (__calcemia, ___phosphatemia, ___parathyroidism, and vitamin D ____) - Renal ultrasound (structural abnormalities), kidney biopsy (glomerular and tubulointerstitial diseases) - Other - Increasing proteinuria, albuminuria, and/or new-onset or worsening HTN is indicative of progressive CKD despite a stable GFR - Serum Cr and GFR are not good indicators of the loss of functioning nephrons bc of the compensatory increased function in remaining nephrons - Risk for CKD progression and deteriorating glomerular filtration rate is greatest during growth spurts of infancy and puberty - Management: - Tx reversible causes of renal dysfunction - For nonreversible causes, treatment is aimed at stopping/slowing the progression of CKD and treating complications - Strict control of blood pressure - Decreasing proteinuria or albuminuria with ACEI/ARB helps to slow progression of CKD, lower BP, and protect kidney. - Other supportive management - Volume overload - sodium restriction and loop diuretics - Hyperkalemia - prevent with low potassium diet - Metabolic acidosis - treat with sodium bicarbonate or citrate - Anemia - Iron supplementation - Renal osteodystrophy - Dietary _______ restriction, oral phosphate binders (calcium carbonate), active vitamin D analogs (calcitriol) to control hyperparathyroidism, vitamin D - Dyslipidemia - treat with diet and exercise. Add statin drug if LDL is consistently elevated - Nutrition: - Patients with CKD often need increased protein - Dietary restriction of phosphorus - Growth hormone is beneficial for children with ESRD in obtaining improved final adult height.

Answer 78

Chronic Kidney Disease - Path: - Renal dysplasia with Congenital anomalies of the kidney and urinary tract (CAKUT) and cystic kidney diseases (nonglomerular CKD) account for nearly 60% of pediatric CKD. - Pt - In early stages of CKD (GFR >60), patients are often asymptomatic. - Primary daytime and nocturnal enuresis in the setting of poor linear growth and proteinuria is concerning for chronic kidney disease. - Dependent edema, nausea/vomiting, fatigue, hematuria, and/or proteinuria - Complications - HTN - Anemia - Poor growth - Bone-calcium metabolic derangements nerve damage - Cognitive delays in young children - Tubulointerstitial injury associated with CAKUT leads to reduced urinary concentration (acquired nephrogenic DI) and these patients usually present with polyuria - Nutritional issues; in particular, short stature/growth restriction - Evaluation - Urinalysis (look for protein) - CBC (look for anemia) - Blood chemistry (look at Cr (high indicates loss of filtering function) - Albumin (low if being lost through kidneys) - Electrolytes (hyperkalemia and metabolic acidosis due to poor renal excretion) - Calcium, phosphate, PTH, and vitamin D levels (hypocalcemia, hyperphosphatemia, hyperparathyroidism, and vitamin D deficiency) - Renal ultrasound (structural abnormalities), kidney biopsy (glomerular and tubulointerstitial diseases) - Other - Increasing proteinuria, albuminuria, and/or new-onset or worsening HTN is indicative of progressive CKD despite a stable GFR - Serum Cr and GFR are not good indicators of the loss of functioning nephrons bc of the compensatory increased function in remaining nephrons - Risk for CKD progression and deteriorating glomerular filtration rate is greatest during growth spurts of infancy and puberty - Management: - Tx reversible causes of renal dysfunction - For nonreversible causes, treatment is aimed at stopping/slowing the progression of CKD and treating complications - Strict control of blood pressure - Decreasing proteinuria or albuminuria with ACEI/ARB helps to slow progression of CKD, lower BP, and protect kidney. - Other supportive management - Volume overload - sodium restriction and loop diuretics - Hyperkalemia - prevent with low potassium diet - Metabolic acidosis - treat with sodium bicarbonate or citrate - Anemia - Iron supplementation - Renal osteodystrophy - Dietary phosphorus restriction, oral phosphate binders (calcium carbonate), active vitamin D analogs (calcitriol) to control hyperparathyroidism, vitamin D - Dyslipidemia - treat with diet and exercise. Add statin drug if LDL is consistently elevated - Nutrition: - Patients with CKD often need increased protein - Dietary restriction of phosphorus - Growth hormone is beneficial for children with ESRD in obtaining improved final adult height.

Question 79

- Dialysis - Start when patient with CKD has advancing uremia (any uremic symptoms in a patient with a CrCl <10-15 ml/min/1.73 m2). - Uremic symptoms can initially present as difficulty with concentration and sleep, metallic taste, anorexia, and hiccups, and then progresses to nausea and vomiting and eventually to seizures, coma, and death. It often feels like a bad case of the flu during the initial phase. - Renal replacement therapy (RRT) should be initiated once the GFR declines to <30 (onset of stage 4). Options for RRT include hemodialysis, peritoneal dialysis, and renal transplant. - Continuous peritoneal dialysis - most common modality used in infants/young children - Main complication of PD: - Peritonitis - Other complications: High protein loss - Hemodialysis - The most common cause of death in dialysis pts is cardiovascular disease, followed by infection - Only transplant can reverse the late manifestations of small-vessel calcification and motor neuropathy. Like dialysis, improves platelet dysfunction, renal osteodystrophy, and sensory and cognitive dysfunction. - If there is renal function deterioration in the 1st week after transplant - 1) Check cyclosporin or tacrolimus levels - 2) Do a renal US to rule out outlet obstruction or vascular events - 3) If levels are normal and renal US is negative then do a renal biopsy to assess for tissue rejection - Common infection after transplant: pneumonia, sepsis, CMV reactivation - Drugs - Cyclosporin - Side effects include: Tremors, hirsutism, nephro/hepato/CNS toxicity, HTN, _____ like phenytoin (know this!) - Tacrolimus - Azathioprine: Does affect the bone marrow. Most significant side effect is ____. - Mycophenolate mofetil (CellCept): Side effect of ___ - Sirolimus (aka rapamycin): Hyper____ (lipid monitoring is required), proteinuria, thrombocytopenia, delayed wound healing

Answer 79

- Dialysis - Start when patient with CKD has advancing uremia (any uremic symptoms in a patient with a CrCl <10-15 ml/min/1.73 m2). - Uremic symptoms can initially present as difficulty with concentration and sleep, metallic taste, anorexia, and hiccups, and then progresses to nausea and vomiting and eventually to seizures, coma, and death. It often feels like a bad case of the flu during the initial phase. - Renal replacement therapy (RRT) should be initiated once the GFR declines to <30 (onset of stage 4). Options for RRT include hemodialysis, peritoneal dialysis, and renal transplant. - Continuous peritoneal dialysis - most common modality used in infants/young children - Main complication of PD: - Peritonitis - Other complications: High protein loss - Hemodialysis - The most common cause of death in dialysis pts is cardiovascular disease, followed by infection - Only transplant can reverse the late manifestations of small-vessel calcification and motor neuropathy. Like dialysis, improves platelet dysfunction, renal osteodystrophy, and sensory and cognitive dysfunction. - If there is renal function deterioration in the 1st week after transplant - 1) Check cyclosporin or tacrolimus levels - 2) Do a renal US to rule out outlet obstruction or vascular events - 3) If levels are normal and renal US is negative then do a renal biopsy to assess for tissue rejection - Common infection after transplant: pneumonia, sepsis, CMV reactivation - Drugs - Cyclosporin - Side effects include: Tremors, hirsutism, nephro/hepato/CNS toxicity, HTN, hypertrophies the gum like phenytoin (know this!) - Tacrolimus - Azathioprine: Does affect the bone marrow. Most significant side effect is Leukopenia. - Mycophenolate mofetil (CellCept): Side effect of GI - Sirolimus (aka rapamycin): Hyperlipidemia (lipid monitoring is required), proteinuria, thrombocytopenia, delayed wound healing

Question 80

Hemolytic Uremic Syndrome - Complicated sequence of events that begins with endothelial and glomerular injury and eventually results in microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury - Path: Thrombotic microangiopathy following Shiga toxin E coli (STEC) diarrhea illness - Pt: - Triad: ___, ____, _____ - Diarrhea (often bloody) and abdominal pain with initial colitis; HUS develops on average 6 days (2-14 days) after onset of diarrhea. - Labs and evaluation - CBC with smear to reveal diagnosis - Peripheral smear reveals - Echinocytes (burr cells) are indicative of microangiopathy - MAHA: _______, other fragmented RBCs, and decreased or absent platelets - Stool cultures (MacConkey agar base with sorbitol) or stool assays (ELISA) for Shiga toxins to look for E coli O157:H7. - Management - _____ with close monitoring of volume status and careful fluid administration. - The use of ___ agents is discouraged and ____ are contraindicated - Many patients (nearly 50%) require dialysis. - Dialysis is indicated when BUN >80mg/dL, volume overload not responsive to diuresis, hyperkalemia or acidosis not responsive to medication correction, or kidney failure

Answer 80

Hemolytic Uremic Syndrome - Complicated sequence of events that begins with endothelial and glomerular injury and eventually results in microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. - Path: Thrombotic microangiopathy following Shiga toxin E coli (STEC) diarrhea illness - Pt: - Triad: Microangiopathic hemolytic anemia, thrombocytopenia, acute kidney injury - Diarrhea (often bloody) and abdominal pain with initial colitis; HUS develops on average 6 days (2-14 days) after onset of diarrhea. - Labs and evaluation - CBC with smear to reveal diagnosis - Peripheral smear reveals - Echinocytes (burr cells) are indicative of microangiopathy - MAHA: Helmet-shaped schistocytes, other fragmented RBCs, and decreased or absent platelets - Stool cultures (MacConkey agar base with sorbitol) or stool assays (ELISA) for Shiga toxins to look for E coli O157:H7. - Management - Supportive care with close monitoring of volume status and careful fluid administration. - The use of antimotility agents is discouraged and antibiotics are contraindicated - Many patients (nearly 50%) require dialysis. - Dialysis is indicated when BUN >80mg/dL, volume overload not responsive to diuresis, hyperkalemia or acidosis not responsive to medication correction, or kidney failure

Question 81

Kidney Stones - RF: - ______ is the most commonly identified RF - Increased urinary excretion of citrate (most important), magnesium, and pyrophosphate is associated with a decreased risk of renal stone formation, and a low level of these inhibitors is associated with an increased risk for nephrolithiasis in children and adults. - _____ is the major inhibitor of calcium stones. - Workup following initial stone passage: - Chemical analysis of the stone, serum calcium level (to rule out hypercalcemia), mg, phos, electrolytes (to rule out type 1 RTA), urinalysis with culture/sensitivity, and renal imaging (spiral CT and ultrasound). - Checking 24-hour urine excretion of cystine, oxalate, calcium, Na, uric acid, citrate, and creatinine is important to find and treat specific underlying metabolic abnormalities (the most common being hypercalciuria). - Diagnosis - Renal US is the initial test, but it may not detect small stones or ureteral stones. - _____ (most sensitive) is the most sensitive test for detection of nephrolithiasis. - Tx - less than ____mm: _____ - ___mm-__cm: ____ - __cm: Resective nephrostomy and ureterostomy - If there are signs of acute urethral obstruction with a concurrent kidney infection, the pt must be hospitalized bc of risk of sepsis and papillary necrosis

Answer 81

Kidney Stones - RF: - Hypercalciuria is the most commonly identified RF - Increased urinary excretion of citrate (most important), magnesium, and pyrophosphate is associated with a decreased risk of renal stone formation, and a low level of these inhibitors is associated with an increased risk for nephrolithiasis in children and adults. - Citrate is the major inhibitor of calcium stones. - Workup following initial stone passage: - Chemical analysis of the stone, serum calcium level (to rule out hypercalcemia), mg, phos, electrolytes (to rule out type 1 RTA), urinalysis with culture/sensitivity, and renal imaging (spiral CT and ultrasound). - Checking 24-hour urine excretion of cystine, oxalate, calcium, Na, uric acid, citrate, and creatinine is important to find and treat specific underlying metabolic abnormalities (the most common being hypercalciuria). - Diagnosis - Renal US is the initial test, but it may not detect small stones or ureteral stones. - Noncontrast helical/spiral CT (most sensitive) is the most sensitive test for detection of nephrolithiasis. - Tx - <5mm (Or 8mm): Pain control (NSAIDs or opioids) and aggressive fluids - 8mm-2cm: Extracorporeal shock wave lithotripsy - >2cm: Resective nephrostomy and ureterostomy - If there are signs of acute urethral obstruction with a concurrent kidney infection, the pt must be hospitalized bc of risk of sepsis and papillary necrosis

Question 82

Calcium oxalate stone (most common) - Occurs in ___ pH environment. - Calcium stone inducers: - Hypercalciuria - Hyperoxaluria - Hyperuricosuria - Hypocitraturia - Medullary sponge disease - Tx: - Increase fluid intake - Decrease dietary ___ and ____ - Decrease ____ in diet - ____ to inhibit calcium stone formation/increase pH - Consider ___ to decrease urine calcium when hypercalciuria is significant - Note: Do not decrease ____ intake; this only increases oxaluria!

Answer 82

Calcium oxalate stone (most common) - Occurs in low pH environment. - Calcium stone inducers: - Hypercalciuria - Hyperoxaluria - Hyperuricosuria - Hypocitraturia - Medullary sponge disease - Tx: - Increase fluid intake - Decrease dietary protein and sodium - Decrease oxalate in diet - Potassium citrate to inhibit calcium stone formation/increase pH - Consider HCTZ to decrease urine calcium when hypercalciuria is significant - Note: Do not decrease calcium intake; this only increases oxaluria!

Question 83

Calcium phosphorus stone - Occurs in ____ pH environment - RF: More common with Type ___ distal RTA (alkaline urine promotes CaP precipitation and the associated metabolic acidosis predisposes stone formation bc it buffers calcium out of the bones), patients with primary hyperparathyroidism, patients on acetazolamide, topiramate, ketogenic diet - Tx: - Do not decrease ____ intake; this only increases oxaluria! - Decrease dietary ___ and ___ - Consider ___ to decrease urine calcium - Do not give citrate as it increases pH

Answer 83

Calcium phosphorus stone - Occurs in high pH environment - RF: More common with Type I distal RTA (alkaline urine promotes CaP precipitation and the associated metabolic acidosis predisposes stone formation bc it buffers calcium out of the bones), patients with primary hyperparathyroidism, patients on acetazolamide, topiramate, ketogenic diet - Tx: - Do not decrease calcium intake; this only increases oxaluria! - Decrease dietary protein and sodium - Consider HCTZ to decrease urine calcium - Do not give citrate as it increases pH

Question 84

Struvite Stones - Composed of magnesium ammonium phosphate and calcium carbonate - Occurs in ____ pH environment/alkaline - Cause ____ calculi - Think infection when you see these - RF: Infection with ammonium producing organisms (via urea) - P___, P___, y___, s___ (PPYS) - Treatment - Removal of the stones and/or calculi - ______ of the urine (This is the only other stone, besides calcium phosphate, made more likely by alkaline urine) - Antibiotics

Answer 84

Struvite Stones - Composed of magnesium ammonium phosphate and calcium carbonate - Occurs in high pH environment/alkaline - Cause staghorn calculi - Think infection when you see these - RF: Infection with ammonium producing organisms (via urea) - Proteus, Pseudomonas, yeast, staph (PPYS) - Treatment - Removal of the stones and/or calculi - Acidification of the urine (This is the only other stone, besides calcium phosphate, made more likely by alkaline urine) - Antibiotics

Question 85

Cystine Stones - Path: Due to ____. Patients who are homozygous for cystinuria, an AR disease, excrete large amounts of cysteine. - Look for_________ in the urine. - Tx: - Increasing fluids and alkalinizing the urine - to keep urine cystine concentration normal

Answer 85

Cystine Stones - Path: Due to cystinuria. Patients who are homozygous for cystinuria, an AR disease, excrete large amounts of cysteine. - Look for clear hexagonal crystals in the urine. - Tx: - Increasing fluids and alkalinizing the urine - to keep urine cystine concentration normal

Question 86

Uric acid - Path: Hyperuricemia. Usually seen in patients who chronically excrete acidic urine. - Idiopathic - Tumor breakdown - Myeloproliferative syndromes, chemotherapy, and Lesch-Nyhan syndrome - Tx: ______ +/- urinary alkalinization.

Answer 86

Uric acid - Path: Hyperuricemia. Usually seen in patients who chronically excrete acidic urine. - Idiopathic - Tumor breakdown - Myeloproliferative syndromes, chemotherapy, and Lesch-Nyhan syndrome - Tx: Allopurinol +/- urinary alkalinization.

Question 87

Posterior Urethral Valves - Most common cause of urinary obstruction in male infants. Most common cause of obstructive uropathy that leads to renal failure in childhood. - Pt: - Usually diagnosed prenatally on prenatal US. Most are discovered in utero with bilateral hydroureteronephrosis - If presents later in childhood, typically presents with recurrent UTIs, diurnal enuresis in pts >5yo or secondary diurnal enuresis, pain with voiding, voiding dysfunction, or an abnormal urinary stream. - Dx: - Postnatal US of Bilateral ______, dilated bladder, thickened bladder wall, and a dilated posterior urethra in male pts on are highly suggestive of PUV. - After birth, diagnosis of PUV can be confirmed using a ______ - Management - All infants with PUV require _____ - You must look for signs of sepsis, electrolyte abnormalities, acidemia, and fluid imbalance and treat aggressively. - After the infant’s is stabilized, perform primary_____ if possible, which is curative.

Answer 87

Posterior Urethral Valves - Most common cause of urinary obstruction in male infants. Most common cause of obstructive uropathy that leads to renal failure in childhood. - Pt: - Usually diagnosed prenatally on prenatal US. Most are discovered in utero with bilateral hydroureteronephrosis - If presents later in childhood, typically presents with recurrent UTIs, diurnal enuresis in pts >5yo or secondary diurnal enuresis, pain with voiding, voiding dysfunction, or an abnormal urinary stream. - Dx: - Postnatal US of Bilateral hydronephrosis, dilated bladder, thickened bladder wall, and a dilated posterior urethra in male pts on are highly suggestive of PUV. - After birth, diagnosis of PUV can be confirmed using a voiding cystourethrogram (VCUG) - Management - All infants with PUV require immediate urology consult and bladder drainage. - You must look for signs of sepsis, electrolyte abnormalities, acidemia, and fluid imbalance and treat aggressively. - After the infant’s is stabilized, perform primary transurethral valve ablation if possible, which is curative.

Question 88

Ureteropelvic Junction Obstruction - Most common cause of hydronephrosis / obstructive uropathy in infancy and children - Roughly 60% of are cases are left-sided while only 10% are bilateral - Pt: - Pain, hematuria, HTN, UTIs. After drinking liquids, pain occurs due to dilatation of the kidney pelvis. In infants, you sometimes feel a palpable mass. - Dx: - Most commonly diagnosed upon postnatal evaluation of antenatal hydronephrosis detected on maternal US screening. - RUS finding of _____ without _____ favor a UPJ obstruction. - Work-up: ______ study - In children with UPJ obstruction, a renal scan such as 99mTc mercaptoacetyltriglycine (____) with furosemide is commonly performed. The diuretic renography provides an assessment on differential function of each kidney and also suggests the degree of obstruction - Tx: - Aimed at preservation of renal parenchyma and function - In infants, many recommend waiting until 4-6 months of age, then reassessing whether the obstruction has resolved or improved. - If conservative management has not resulted in resolution of the obstruction by 4-5 years of age, consider performing pyeloplasty. - Early pyeloplasty is indicated if the postnatal evaluation shows significant decrease in function on the affected side or a massive dilatation of the renal pelvis.

Answer 88

Ureteropelvic Junction Obstruction - Most common cause of hydronephrosis / obstructive uropathy in infancy and children - Roughly 60% of are cases are left-sided while only 10% are bilateral - Pt: - Pain, hematuria, HTN, UTIs. After drinking liquids, pain occurs due to dilatation of the kidney pelvis. In infants, you sometimes feel a palpable mass. - Dx: - Most commonly diagnosed upon postnatal evaluation of antenatal hydronephrosis detected on maternal US screening. - RUS finding of hydronephrosis without ureteral dilation favor a UPJ obstruction. - Work-up: Nuclear study - In children with UPJ obstruction, a renal scan such as 99mTc mercaptoacetyltriglycine (MAG-3) with furosemide is commonly performed. The diuretic renography provides an assessment on differential function of each kidney and also suggests the degree of obstruction - Tx: - Aimed at preservation of renal parenchyma and function - In infants, many recommend waiting until 4-6 months of age, then reassessing whether the obstruction has resolved or improved. - If conservative management has not resulted in resolution of the obstruction by 4-5 years of age, consider performing pyeloplasty. - Early pyeloplasty is indicated if the postnatal evaluation shows significant decrease in function on the affected side or a massive dilatation of the renal pelvis.

Question 89

Urethral Prolapse - Pt: - Vaginal bleeding or spotting on underwear. - Exam with everted, hemorrhagic, _____-shaped periurethral mass. The prolapsed tissue, which easily bleeds, is distinct from the vagina. - Management: Topical _____ cream, applied twice daily following a sitz bath. This often leads to resolution within 2-4 weeks. Surgery is required only if there is associated evidence of necrosis.

Answer 89

Urethral Prolapse - Pt: - Vaginal bleeding or spotting on underwear. - Exam with everted, hemorrhagic, Donut-shaped periurethral mass. The prolapsed tissue, which easily bleeds, is distinct from the vagina. - Management: Topical estrogen cream, applied twice daily following a sitz bath. This often leads to resolution within 2-4 weeks. Surgery is required only if there is associated evidence of necrosis.

Question 90

Urethrorrhagia - Occurs only in boys. - Pt: - Syndrome of hematuria at the end of urinating, evidenced by spotting of blood in the underwear. - Tx: Self-limited and requires no specific therapy

Answer 90

Urethrorrhagia - Occurs only in boys. - Pt: - Syndrome of hematuria at the end of urinating, evidenced by spotting of blood in the underwear. - Tx: Self-limited and requires no specific therapy

Question 91

Prune Belly Syndrome / Eagle-Barrett Syndrome - Pt: - Characterized by triad of ____, ____, and ______. - Most common presentation: Anatomical genitourinary anomalies but normal renal function - Management: Individualized - If renal function deteriorates, temporary urinary diversion with a vesicostomy or cutaneous pyelostomy is sometimes required. It is important to do orchiopexy early with abdominoplasty.

Answer 91

Prune Belly Syndrome / Eagle-Barrett Syndrome - Pt: - Characterized by triad of anterior abdominal wall musculature, cryptorchidism, and urinary tract anomalies (dilation of the prostatic urethra, bladder, ureters). - Most common presentation: Anatomical genitourinary anomalies but normal renal function - Management: Individualized - If renal function deteriorates, temporary urinary diversion with a vesicostomy or cutaneous pyelostomy is sometimes required. It is important to do orchiopexy early with abdominoplasty.

Question 92

Vesicoureteral reflux - Retrograde passage of urine from bladder to the kidneys. - Pt: - More frequently suspected antenatally by fetal US reporting increased diameter of the renal pelvis / hydronephrosis - Dx: - Diagnosis must be considered in any pt with recurrent UTIs and/or when renal US reports hydronephrosis or hydroureter. - The gold standard to diagnose VUR is the ______, which requires bladder catheterization and fluoroscopy. - Management - ____ prophylaxis - most clinicians still choose to use ppx abx in infants/young children with known VUR - Studies show a decreased risk of UTIs in pts given ppx, but no study has shown that ppx prevents renal scarring. - When VCUG is positive for Grade 2 (in some circumstances) or higher of vesicoureteral reflux (VUR), should continue on prophylactic antibiotics. ____ and ___ are the prophylactic antibiotics of choice - Prognosis - High-end VUR (Grades 4 and 5) increases the risk for development of renal scarring during UTIs.

Answer 92

Vesicoureteral reflux - Retrograde passage of urine from bladder to the kidneys. - Pt: - More frequently suspected antenatally by fetal US reporting increased diameter of the renal pelvis / hydronephrosis - Dx: - Diagnosis must be considered in any pt with recurrent UTIs and/or when renal US reports hydronephrosis or hydroureter. - The gold standard to diagnose VUR is the VCUG, which requires bladder catheterization and fluoroscopy. - Management - Antibiotic prophylaxis - most clinicians still choose to use ppx abx in infants/young children with known VUR - Studies show a decreased risk of UTIs in pts given ppx, but no study has shown that ppx prevents renal scarring. - When VCUG is positive for Grade 2 (in some circumstances) or higher of vesicoureteral reflux (VUR), should continue on prophylactic antibiotics. Bactrim and nitrofurantoin are the prophylactic antibiotics of choice - Prognosis - High-end VUR (Grades 4 and 5) increases the risk for development of renal scarring during UTIs.

Question 93

____ and ____ are most common causes of congenital hydronephrosis

Answer 93

UPJ obstruction and VUR are most common causes of congenital hydronephrosis

Question 94

Loop diuretics - Furosemide and other loop diuretics decrease Na and cl absorption in the medullary and cortical thick ascending limb. - With high-dose IV furosemide, ototoxicity, and deafness can occur.

Answer 94

Loop diuretics - Furosemide and other loop diuretics decrease Na and cl absorption in the medullary and cortical thick ascending limb. - With high-dose IV furosemide, ototoxicity, and deafness can occur.

Question 95

Thiazides - Unlike loop diuretics, thiazides decrease Ca excretion, leading to hypercalcemia - Are NOT effective at a low GFR (ie CrCl <30ml/min/1.73m2).

Answer 95

Thiazides - Unlike loop diuretics, thiazides decrease Ca excretion, leading to hypercalcemia - Are NOT effective at a low GFR (ie CrCl <30ml/min/1.73m2).

Question 96

Spironolactone | - Aldosterone antagonist, so it is K sparing and hence can cause acidosis

Answer 96

Spironolactone | - Aldosterone antagonist, so it is K sparing and hence can cause acidosis

Question 97

Nephrotoxic medications - Antibiotics - Cisplatin - Aminoglycosides (gentamicin, tobramycin) - Antifungal agents (amphotericin B, foscarnet) - Vancomycin- increased risk with higher trough levels - Antiviral agents (eg acyclovir, cidofovir, indinavir, foscarnet, tenofovir) have been associated with tubular injury and renal dysfunction - Pentamidine - (Sulfonylurea is not considered to be nephrotoxic) Chemotherapy - Ifosfamide is more commonly associated with nephrotoxicity. - Cisplatin - Pilamycin - 5-Fluorouracil - Cytarabine - 6-Thioguanine - Methotrexate Calcineurin inhibitors - Cyclosporine - Tacrolimus Misc - NSAIDs - Radiocontrast agents are exogenous toxins to the kidney. - IVIG - Oral phosphate bowel preparations

Answer 97

Nephrotoxic medications - Antibiotics - Cisplatin - Aminoglycosides (gentamicin, tobramycin) - Antifungal agents (amphotericin B, foscarnet) - Vancomycin- increased risk with higher trough levels - Antiviral agents (eg acyclovir, cidofovir, indinavir, foscarnet, tenofovir) have been associated with tubular injury and renal dysfunction - Pentamidine - (Sulfonylurea is not considered to be nephrotoxic) Chemotherapy - Ifosfamide is more commonly associated with nephrotoxicity. - Cisplatin - Pilamycin - 5-Fluorouracil - Cytarabine - 6-Thioguanine - Methotrexate Calcineurin inhibitors - Cyclosporine - Tacrolimus Misc - NSAIDs - Radiocontrast agents are exogenous toxins to the kidney. - IVIG - Oral phosphate bowel preparations

Question 98

Winter’s formula epCO2 = ______

Answer 98

Winter’s formula epCO2 = (1.5 x [HCO3-]) + 8 +/- 2

Question 99

Normal urine sodium is ___

Answer 99

Urine sodium is 20. - Urine sodium <20 suggests extra renal etiology (insensible loss of sodium from burns or sweat or GI tract). Urine sodium >20 suggests a renal etiology (diuretic therapy, adrenal insufficiency, post-obstructive uropathy, other nephropathies, late-phase acute tubular necrosis, cerebral salt wasting)