Horseho Kidney
Seen commonly in Turners syndrome
- Can cause later uinary tract obstructions and nincreased risk fo stones
- Also increased risk for wilms tumor
Multicystic dysplastic kidney
- Improper communication between the metanephric mesoderm and ureteric bud (Mesonephros) results in impaired formation filled with CT and cysts
- Usually unilateral and other side hypertrophies to take over
Fanconi’s
Impaired secretion of anything in PCT can cause problems with renal function
- Wilsons disease is a common cause
- Fanconi’s anemia is related to DS break repair
Barter
- Defect in K,Cl,Na cotransporter in ascending limb
- Acts like loop diuretic
- Leads to hypokalemia, hypercalciuria, alkalosis
Gieltmans
-Defect in DCT Na/Cl transporter acts like a thiazide diuretic
-Milder hypokalemia with no hypercalciurua
Mild metabolic acidosis
Liddle
OVeractivity of ENaC channels in CD leads to spironolactone insensitive hypokalemia, HTN (Hypernatremia)
RTA 1
Defect in acid secretion by alpha intercalated cells
- Amphoteracin B, analgesic nephropathy
- Causes an increased urine pH and can lead to formation of calcium phosphate stones
RTA 2
Defective HCO3 secretion in PCT leads to metabolic acidoss
-There will be no decrease in H secretion in alpha intercalated cells, so urine will remain acidic
RTA-4
Hypoaldosteronism
Leads to hyperkalemia that impairs ammoniagenesis in the PCT leading to decrease buffering capacity
-urine will still be acidic