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03

Proximal convoluted tubule

The workhorse of the nephron: it takes back about two thirds of everything the glomerulus filtered — salt, water, essentially all of the filtered glucose, most of the bicarbonate — before the fluid ever reaches the loop.

How Proximal convoluted tubule fits together: 4 things it normally does, the 5 ways it fails, and the 4 drugs that act on those failures. Arrows run from each normal function to the failure it explains, and from each failure to the drug that answers it.What it doesWhat goes wrongWhat we giveBulk Na+ reabsorptionGlucose via SGLT2HCO3- reclamationBrush border transportOsmotic diuresisFanconi syndromeType 2 (proximal) RTAAcute tubular necrosisHyperuricaemia, goutSGLT2 inhibitorsAcetazolamideProximal tubule toxinsUricosurics
Every arrow is a link in the content itself, not a decoration: each failure points back to the normal function it breaks, and each drug to the failure it answers. Hover a box to light its whole chain, or click to jump to it.Swipe the diagram to see all of it.

What it normally does

  • It reabsorbs in bulk, not in fine detail: roughly 65% of the filtered sodium and water. The whole operation is powered by one pump on the blood side of the cell that pushes sodium out (Na+/K+-ATPase), creating a low intracellular sodium that everything else rides on.

    almost every uptake pathway on the brush border depends on that sodium gradient, directly (sodium-coupled carriers) or indirectly (the H+ gradient NHE3 sets up) — knock out the cell's energy supply and they fail together — and a drug that blocks reabsorption here loses much of its effect because the loop and distal tubule downstream simply take the sodium back.

  • All of the filtered glucose is normally reabsorbed here, about 90% of it by a sodium-glucose cotransporter in the first segment (SGLT2, low affinity but high capacity) and the rest by a higher-affinity, lower-capacity one further along in S3 (SGLT1). The system has a ceiling — it saturates once blood glucose passes roughly 10 to 11 mmol/L.

    glucose appearing in the urine means either the blood level has overwhelmed a normal transporter, or the transporter itself is broken or blocked.

  • This is where filtered bicarbonate is reclaimed — around 80% of it. The cell secretes H+ into the lumen (sodium-hydrogen exchanger, NHE3); that H+ joins filtered bicarbonate to make CO2 and water under brush border carbonic anhydrase; the CO2 diffuses into the cell, where carbonic anhydrase runs the reaction backwards and the regenerated bicarbonate leaves across the basolateral membrane into the blood.

    bicarbonate is not absorbed as a molecule, it is dismantled and rebuilt — so blocking the enzyme in the middle of that loop dumps bicarbonate into the urine and acidifies the blood.

  • The same brush border reclaims amino acids, phosphate, urate and small filtered proteins, and the cells are packed with mitochondria to pay for it. The straight segment running into the outer medulla (S3) sits in the region of the kidney with the lowest oxygen supply, alongside the medullary thick ascending limb, while still running a high ATP demand.

    one cell, many jobs, one power source: poison or starve these cells and you lose glucose, amino acids, phosphate and bicarbonate simultaneously — and the S3 segment is among the first parts of the kidney to be injured when perfusion drops.

What goes wrong

  • When blood glucose climbs past about 10 to 11 mmol/L, the filtered load exceeds what SGLT2 and SGLT1 can carry. The excess glucose stays in the tubular fluid and holds water with it osmotically, so the patient loses litres of urine plus the salt and potassium dissolved in it. This is why uncontrolled type 2 diabetes presents as thirst and passing urine all night, rather than as a number on a meter.

    Filtered load beats transport maximum (threshold about 10 to 11 mmol/L) → glucose stays in the lumen → osmotic diuresis → polyuria, thirst, volume depletion.

    You would find: Polyuria, nocturia and thirst, with glucose on the urine dipstick and a raised blood glucose. In a hyperglycaemic, dehydrated patient this same osmotic loss is what creates the huge fluid deficit. Type 2 diabetes and its complications fall disproportionately on Aboriginal and Torres Strait Islander communities, where rates of kidney failure are several times the national figure.

  • Fanconi syndrome (generalised proximal tubule failure)← from “The same brush border reclaims amino acids, ph

    Anything that cripples the energy supply of these cells — a drug, light chains from myeloma, copper in Wilson disease, cystine in children with cystinosis — drops ATP, the sodium pump slows, and the whole brush border fails at once. Glucose, amino acids, phosphate, bicarbonate, urate and small proteins all leak into the urine together. The phosphate loss is the one that shows up in bone, and it is compounded because the proximal tubule is also where 1-alpha-hydroxylase converts 25-hydroxyvitamin D to calcitriol: the patient loses phosphate and loses the hormone that would help them absorb more.

    Everything leaks at once: glycosuria with normal blood glucose, aminoaciduria, hypophosphataemia, type 2 RTA, low urate. Adults — think drugs or myeloma. Children — think cystinosis.

    You would find: Glucose on the dipstick with a normal blood glucose — once an SGLT2 inhibitor has been excluded from the medication list, that pairing points hard at the proximal tubule. Add low phosphate, low potassium, low bicarbonate, low urate, and bone pain or difficulty rising from a chair (osteomalacia in adults, rickets in children). Dipstick protein is often negative, because the proteins being lost are small and the stick detects albumin.

  • The bicarbonate reclamation machinery is blocked or broken, so the tubule can only hold bicarbonate up to a lowered threshold. Bicarbonate spills into the urine until plasma bicarbonate falls to that new set point — commonly quoted as around 12 to 18 mmol/L — and then the leak stops. The bicarbonate carries sodium with it to the distal nephron, where the extra sodium delivery (helped by the volume depletion and secondary aldosterone that go with it) drives distal sodium reabsorption through ENaC, leaving the lumen more negative and driving potassium secretion — so the patient becomes hypokalaemic as well as acidotic.

    Leaky reclamation, not failed acidification. Normal gap acidosis, hypokalaemia, plasma bicarbonate plateaus low, urine can still reach pH <5.5. Usually part of Fanconi syndrome rather than isolated.

    You would find: A normal anion gap metabolic acidosis with a low potassium. Early on, and whenever plasma bicarbonate is above the tubule's threshold, the urine is alkaline; once plasma bicarbonate has dropped below that threshold the urine can acidify normally (pH under 5.5) — that is what separates it from distal (type 1) RTA, where the urine cannot be acidified below about pH 5.5 even under an acid load. Give bicarbonate and the potassium falls further.

  • The S3 segment sits in the outer medulla with a high ATP demand and a marginal oxygen supply. Sepsis, haemorrhage, major surgery or sustained hypotension drop perfusion below what those cells survive; nephrotoxins (aminoglycosides, contrast, myoglobin from rhabdomyolysis) do the same by direct injury. Dead cells slough into the lumen, obstruct the tubule, and stop reabsorbing sodium — so the kidney fails and the urine sodium goes up rather than down.

    Commonest cause of acute kidney injury arising in hospital. Ischaemic or toxic, S3 segment first. Muddy brown casts, FENa >2%, isosthenuric urine. No drug has been shown to reverse established ATN — the management is restoring perfusion, removing the toxin and supporting the patient while the epithelium regenerates.

    You would find: Creatinine climbing over days after a septic or hypotensive episode, urine output falling, muddy brown granular casts on microscopy, and a fractional excretion of sodium above 2% — the opposite of the avid sodium retention of pre-renal failure (FENa under 1%). The FENa is only interpretable if the patient is not on a diuretic.

  • About 90% of filtered urate is taken back in the proximal tubule, largely by an exchanger that takes urate up from the lumen in exchange for an intracellular organic anion (URAT1). Anything that increases proximal reabsorption raises blood urate: volume depletion and diuretics, because more sodium and water reabsorbed here means more urate reabsorbed with it, and lactate or ketoacids from alcohol or starvation, which supply extra counter-anion and so pull more urate back into the cell. Once plasma urate stays high, monosodium urate crystallises in cooler peripheral joints.

    Urate is largely a proximal tubule handling problem rather than an overproduction problem in most patients. Volume depletion and diuretics → more proximal reabsorption → higher urate → gout. The usual urate-lowering target in Australian practice is under 0.36 mmol/L (lower again with tophi).

    You would find: A hot, exquisitely tender first metatarsophalangeal joint coming on overnight, often after alcohol, a diuretic or dehydration. Serum urate is usually raised — plasma is saturated somewhere around 0.40 to 0.42 mmol/L — though it can read normal or low during the attack itself, so a normal level does not exclude gout; joint aspiration showing negatively birefringent needle-shaped crystals is the definitive test. Gout is more common, earlier and more severe in Aboriginal and Torres Strait Islander people and in Māori and Pacific Islander communities in Australia, and is under-treated in all of them.

What we give, and how it works

Open a drug to see what it binds, what that does to the cell, and what you then see in the patient.

Glucose in the urine is not one disease, it is three. A normal transporter overwhelmed by a high blood glucose (diabetes), a broken transporter with a normal blood glucose (Fanconi syndrome, or inherited familial renal glycosuria), or a normal transporter deliberately blocked (an SGLT2 inhibitor). Read the dipstick next to the blood glucose and the medication list — that one pairing separates all three.

Now test whether it stuck

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