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Proximal Tubule Transport & Disorders


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

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