Renal Tubular Acidosis Flashcards
Type 1 Distal RTA
Impaired ability to secrete acid (acidify urine), results in:
- alkaline urine (< 5.5)
- metabolic acidosis
- hyperchloremia (loss of HCO3)
- hypokalemia (unable to secrete H+)
- hypercalciuria
Which type of RTA results in an ability to acidify urine?
Type 1 distal RTA
What is the metabolic abnormality in distal type 1 RTA?
hyperchloremic metabolic acidosis
- hypokalemia
- hypercalciuria
What is Fanconi syndrome?
global proximal tubule dysfunction
In Fanconi syndrome, what is lost in the urine?
Glucose Amino acids LMW protein Phosphate Bicarbonate
MSK manifestation of Fanconi syndrome
FTT
short stature
hypophosphatemic rickets
Genetic causes of Fanconi syndrome
Cystinosis Lowe syndrome Tyrosinemia Galactosemia WIlson's disease Dent's disease
Acquired causes of fanconi syndrome
Medications (Cisplatin, ifosfamide, valproate)
Clinical features of Fanconi syndrome
Rickets FTT short stature dental enamel defects intellectual impairment ocular anomalies - glaucoma, cataracts
What is cystinosis?
Accumulation of cystine crystals in major organs (kidney, liver, eye, brain)
What type of RTA do people with cystinosis get?
Type II, proximal (Fanconi syndrome)
What is type 4 RTA?
Hypoaldosteronism (or pseudohypoaldosteronism)
What can cause pseudohypoaldosteronism?
obstructive uropathy, UTI / pyelo
What are the important electrolyte abnormalities in pseudohypoaldosteronism?
Hyperkalemia
Hyponatremia
