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20 Cards in this Set
- Front
- Back
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Name all of the anatomy of the nephron in order to the collecting duct
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Glomerulus, PCT, TDL, TAL, DCT, connecting tubule, cortical collecting duct, and medullary collecting duct
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What are the 5 types of Proximal Tubule Disorders?
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AA, glucose, phosphate, HCO3 transport defects, and diffuse injury
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This is most the most type of AA defect? Name 2 others?
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Cystinuria. Iminoglycinuria, and Hartnup disease.
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What is the problem in Cystinuria? What is the cause of infections and renal failure in this disease?
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Impaired absorption of cystine, arginine, lysine, & ornithine. Cystine stones.
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What is the genetic inheritance of Cystinuria? How do you treat it?
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Autosomal Recessive. Inc. cystine solubility.
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How do you treat Cystinuria via inc. cystine solubility? (3)
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Alkanize the urine, give lots of fluids, penacillamine, and tiopronin.
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This autosomal recessive disorder cause a glucose transport defect?
What is the primary problem? |
Renal glycosuria. Malabsorption of glucose.
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This is seen in the disorder of phosphate transport? What is the inheritance?
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Vitamin-D resistant rickets, Pseudohypoparathyroidism. X-linked.
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What is the clinical presentation in Pseudohypoparathyroidism? (PHPT) How do you treat it?
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Growth failure, rickets. Give Vitamin D and phosphate.
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Bicarbone tranport defect causes what disorder? What drug can also cause this disorder?
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Proximal RTA, Type 2. Diamox (carbonic anhydrase inhibitor)
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What are the 2 ways in which the kidney maintains acid/base homeostasis?
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Reclaims filtered bicard (in PCT), and excretes acid/regenerates bicarb (in collecting duct)
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In what 2 situations should one suspect RTA?
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1) metabolic acidosis
2) non-gap acidosis |
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What are the differential dx's of normal anion gap acidosis?
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Diarrhea, diarrhea, diarrhea, and RTA
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What is the eqn for the urinary anion gap (UAG)? What does a value of zero mean? negative?
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Una + Uk - Ucl.
Normal value or RTA. Diarrhea! |
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So basically, if I have a normal anion gap acidosis, and I need to know if the stool or urine is loosing my bicarb, what do I look for?
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A very negative UAG!!!!
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What cation and anion inc. during acidosis to large proportions?
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NH4+ and Cl-
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What is Fanconi Syndrome?
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It is global proximal injury that includes all the the PCT disorders.
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What are some inherited etiologies of Fanconi syndrome? What drugs may induce it?
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Cystinosis, galactosemia, Wilson's disease, hereditary fructose intolerance, tyrosinemia, and Lowe syndrome. Gentamicin, tetracycline, and heavy metals.
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What is the most common pediatric tubulopathy? What is its inheritance pattern? clinical fx's?
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cystinosis. Autosomal recessive. Failure to grow, dehydration, rickets, hypothyroidism.
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What are the tx's for Cystinosis?
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HCO3, PO4 replacement, thiazides, NSAIDS, vasotec to reduce GFR, cysteamine, and diet.
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