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84 Cards in this Set
- Front
- Back
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Impaired reabsorption of cystine, argnine, lysine, and ornithine results in what?
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Cystinuria
Recurrent cystine stones |
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What is the hereditary pattern of cystinuria?
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AR
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How is cystinuria treated?
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Alkalinazation of urine
Lots of fluid Abx |
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What causes renal glycosuria?
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impaired rebasorption of glucose in presence of normal serum glucose
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What are symptoms of vitamin D-resistant rickets?
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abnormal growth
rickets bowed bones |
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What is the hereditary pattern of vitamin D-resistant rickets?
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X-linked
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What is vitamin D-resistant rickets?
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Impaired reabsorption of phosphate in presence of normal serum PTH & Ca2+
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What is the treatment for vitamin D-resistant rickets?
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Vitamin D, phosphate, growth hormone
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Fanconi syndrome results in what?
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Many defects-->more global syndrome
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What are clinical features of diabetes insipidus?
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Prefer water to nutrition
Failure to grow Frequent fevers |
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What does Fanconi syndrome consist of?
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Loss of: bicarb, glucose, phosphate, amino acids, sodium
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How is a diagnosis of Fanconi syndrome made?
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RTA (normal anion gap metabolic acidosis)
Dip urine for glucose (measure serum glucose) FEphos=(U/Pphos)/(U/Pcr) should be <10-15%-->more=Fanconi |
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If a patient comes in with deposits in the corneas, what do you suspect?
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Cystinosis
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What is type 1 RTA?
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Distal RTA
Inability to excrete daily acid load |
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What causes type 1 RTA?
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Defects in medullary proton pumps
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How does type 1 RTA present?
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Failure to grow
Potassium can be low or normal Nephrocalcinosis Progressive RF Progressive acidemia |
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Why does nephrocalcinosis develop in type 1 RTA?
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Hypercalciuria: acidosis causing release from bone
Hypercitraturia: reabsorbed in PT |
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What is the treatment for type 1 RTA?
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HCO3- 1-3mEq/day
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What is type 2 RTA?
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Proximal
Impaired absorption of bicarbonate in PT |
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What type of RTA can occur transiently secondary to carbonic anhydrase inhibitors?
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type 2 (proximal) RTA
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What is the most common RTA?
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Type 4 RTA
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What is type 4 RTA?
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Impaired ability to secrete H+ & K+ in CCD
Inability to reabsorb Na+ |
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What are the possible etiologies of type 4 RTA?
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Often as part of global CCD dysfunction
Mineralocorticoid def Drugs: ACEi, cyclosporine A DM tubulo-interstitial nephropathy |
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How do you treat type 4 RTA?
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Bicarb 1-3mEq/day
K restriction Mineralocorticoid repalcement |
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How do you differentiate the different RTAs?
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Positive dip urine glucose-->type 2
Urine pH by pH meter>5.5-->type 1 Hyperkalemia-->type 4 |
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A non-anion gap metabolic acidosis and a positive dip urine glucose indicate what?
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Type 2 RTA
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A non-anion gap metabolic acidosis and urine pH>5.5 indicate what?
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Type 1 RTA
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A non-anion gap metabolic acidosis and high serum K indicate what?
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Type 4 RTA
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What causes X-linked nephrogenic DI?
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ADH receptor mutations (AVPR2)
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What causes autosomal recessive DI?
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water channel mutations (AQP-2)
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What is hyporeninemia hypoaldosteronism?
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type 4 RTA
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What is Bartter's syndrome?
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TAL defect
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What is the etiology of Bartter's syndrome?
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Defect in Na-K-2Cl transporter, Cl transporter, or Cl transporter Beta-subunit
Loss of NaCl & cations |
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How Bartter's syndrome present?
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Failure to grow, nephrocalcinosis, UTIs
Hypokalemic metabolic acidosis |
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What should you think in a patient that has hypokalemic metabolic acidosis?
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Bartters
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How do you treat Bartters?
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NaCl
KCl Indomethacin |
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Where is the highest incidence of kidney stones?
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Southeast US
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What are possible etiologies of stone formation?
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Excessive solute
Decreased solvent (urine) pH condition favor solute formation Presence of factor which favors formation of insoluble salts Lack of urolithiasis inhibitors |
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Urate crystals favor the formation of what type of stone?
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Calcium oxalate stones
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Alkaline urine favors the formation of what kinds of stones?
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Struvite
Calcium phosphate |
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Acid urine favors the formation of what kinds of stones?
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Uric acid
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What stones are radio-opaque?
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Calcium phosphate
Calcium oxalate Struvite Cystine |
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What stones are radio-lucent?
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Uric acid
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What stones are more common in women?
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Struvite
Cystine |
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What are risk factors for stone formation?
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Low urine volume
Hypercalciuria Hyperuricosuria Hypocitraturia Hyperoxaluria High Na intake High protein intake Anatomic factors |
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What are causes of hypercalciuria with hypercalcemia?
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Hyperparathyroidism
Granulomatous disease Hyperthyroidism Malignancies |
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What kind of hypercalciuria would type 1 RATE produce?
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Hypercalciuria w/o hypercalcemia
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What is the most common cause of calcium stone disease?
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Idiopathic hypercalciuria w/o hypercalcemia
AD trait Excessive absorption of Ca by GI |
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What are the causes of hyperoxaluria?
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Enteric oxaluria
Dietary hyperoxaluria Primary hyperoxaluria |
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What is primary hyperoxaluria?
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AR disorder causing massive urinary excretion of oxalate
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How does high Na intake result in stone formation?
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High Na intake-->increased urinary Ca excretion
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How does high protein intake result in stone formation?
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Increases hypercalciuria
Decreases urinary citrate Increases urinary uric acid levels |
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What are anatomic factors favoring stone formation?
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Horseshoe kidney
Medullary sponge kidney Polycystic kidney |
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Which kidney stone patients get evaluated?
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Those w/active stone disease & recurrent stone formers
1st stone formers get limited w/u |
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What imaging is done for nephrolithiasis?
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KUB film
IV pyelogram US CT MRI |
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What is the best imaging for nephrolithiasis?
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CT stone study
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What is a disadvantage of KUB film?
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Cannot detect radiolucent stones
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How can hypercalciuria be modulated to prevent stone formation
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Increase urine volume
HCTZ Low salt diet Normal Ca diet Normal protein diet |
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How can hyperoxaluria be modulated to prevent stone formation?
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Increase urine volume
Low oxalate diet Avoid ascorbic acid Calcium supplements |
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How can hypocitraturia be modulated to prevent stone formation?
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Increase urine volume
Potassium citrate supplement NOrmal protein diet Bicarb for metabolic acidosis |
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How can hyperuricosuria be modulated to prevent stone formation?
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Decrease dietary purines
Alkalinize urine Potassium citrate allopurinol |
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How is uric acid nephrolithiasis treated?
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Acetazolamide at bedtime to alkalinize urine
Decrease dietary purines Potassium citrate Allopurinol |
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How is struvite stone formation treated?
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Catheter drainage may be needed
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What do uric acid stones look like?
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oblong with pointy ends
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What do struvite stones look like?
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Coffins
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What do calcium oxalate dihydrate stones look like?
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square with diamondy shape
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What do cystine crystals look like?
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hexagon
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What do calcium oxalate monohydrate stones look like?
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Dumbbells
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What is the anatomy of reflux?
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Ureteric orifices displaced laterally in bladder and submucosal segments are short
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What is the genetic pattern of reflux?
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Autosomal dominant
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What is the most accurate imaging for reflux?
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Voiding cystourethrography (VCUG)
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What is the most accurate imaging for reflux nephropathy?
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DMSA radionuclide scan
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What are the complications of reflux?
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Infection
HTN CRF Urinary calculi Heavy proteinuria: FSGS |
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What is the most common cause of HTN in childhood?
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Reflux
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What is the most common cause of secondary ureteric reflux in children?
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Posterior urethral valves
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What is the most important determinant of development of renal damage in reflux nephropathy?
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Reflex nephropathy
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How is VUR managed?
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Surgery in first year of life
Otherwise focus on sterility of urine |
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What is the most common cause of obstruction in children?
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Posterior urethral valves
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What is the most common cause of obstruction in 20-30 year olds?
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renal calculi
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What is the most common cause of obstruction in the elderly?
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Prostate & pelvic malignancies
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What is a diuretic isotopic renogram and what can it diagnose?
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Obstruction.
Uptake of isotope remains steady & diuretic administration does not change it in case of obstruction. |
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What are complications of UTO?
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ARF
CRF Abdominal or flank pain Tubular dysfunction Recurrent UTI Calculi HTN Post-obstructive diuresis |
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What causes post-obstructive diuresis?
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Build-up of waste products during ARF
IV saline administration |
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How is post-obstructive diuresis treated?
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Monitor urine output & weight
Appropriate fluid & electrolyte replacement Use half iso-osmolar fluids Avoid over-zealous fluid replacement |
