⌘K
Volume 04

A study guide to nephrology.

For the internal medicine resident who'd rather trend the creatinine than wing it, keeping your knowledge well-filtered and your confidence from going into retention. Last revised 2026.

12 topics

Nephrology

Acid-Base · Electrolytes · Hypertension · Glomerular · AKI · CKD

01

Acid-Base Disorders

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Definitions & Compensation

Acidosis: pH <7.37, metabolic HCO3 <22 mEq/L, respiratory PCO2 >44.

  • Met acidosis compensation: PCO2 = 1.5 × HCO3 + 8 ± 2
  • Acute resp acidosis: 1 ↑ HCO3 per 10 ↑ PCO2
  • Chronic resp acidosis: 3.5 ↑ HCO3 per 10 ↑ PCO2

Alkalosis: pH >7.44, metabolic HCO3 >26, respiratory PCO2 <36.

  • Met alkalosis: 0.7 ↑ PCO2 per 1 ↑ HCO3
  • Acute resp alkalosis: 2 ↓ HCO3 per 10 ↓ PCO2
  • Chronic resp alkalosis: 4–5 ↓ HCO3 per 10 ↓ PCO2
Gaps

Anion gap = Na − Cl − HCO3 · normal 8–10 ± 2. <4 › MM, hypoalbuminemia. Low albumin › corrected AG = AG + 2.5 × (40 − albumin).

Delta-delta = ΔAG (12 − AG) : HCO3 (24 − HCO3): >2 › concomitant metabolic alkalosis (vomiting, diuretics). 1–2 › pure HAGMA. <1 › concurrent NAGMA.

Osmolar gap = calculated osm (2×Na + glucose + BUN + ethanol) − serum osm. Normal <10. High › EtOH or toxic alcohol.

  • HAGMA: ethylene glycol, methanol, lactate, salicylate toxicity, AKI, DKA, oxoproline/pyroglutamic acid (chronic paracetamol use, pregnancy, veganism, malnutrition), CKD, alcoholic ketoacidosis
    • Type A lactic acidosis: tissue hypoperfusion › multisystem organ dysfunction
    • Type B lactic acidosis: propofol, metformin, NRTI, hematologic malignancy, thiamine deficiency, propylene glycol toxicity
    • D-lactic acidosis: short bowel syndrome
    • Alcoholic ketoacidosis › β-hydroxybutyrate › thiamine then D5
    • Salicylate toxicity: resp alkalosis + HAGMA
  • High osmolar gap + HAGMA: methanol, ethylene glycol, propylene glycol, paraldehyde, DKA, lactic acidosis, severe CKD
    • Ethylene glycol › fomepizole, IVF, NaHCO3, pyridoxine, thiamine, HD (severe)
  • High osmolar gap (no metabolic acidosis): ethanol, isopropyl alcohol, mannitol, sorbitol, pseudohyponatremia, early toxic alcohol, hyperlipidemia, hyperproteinemia (MM)
NAGMA — Urine Anion Gap

UAG = Urine(Na) + Urine(K) − Urine(Cl). Urine(Cl) = Urine(NH3).

  • UAG <0 › GI HCO3 loss (diarrhea), pancreatic fistula, NJ tube
  • UAG >0 › RTA I/IV
RTA Type I (Distal)

NAGMA, hypokalemia, urine pH >5.5, serum HCO3 ≅ 10 › NaHCO3, K citrate.

CTD (Sjögren, RA, SLE), hypercalciuria (nephrocalcinosis), amphotericin B, urinary obstruction, ifosfamide.

RTA Type II (Proximal)

NAGMA, hypokalemia, urine pH <5.5, low serum PO4, serum HCO3 ≅ 16–18 › NaHCO3, thiazide diuretic, potassium-sparing diuretic.

Fanconi (glycosuria, phosphaturia, hypouricemia, aminoaciduria), low vit D, MM, acetazolamide, topiramate, tenofovir, tubular proteinuria.

RTA Type IV

Hyporeninemic hypoaldosteronism. NAGMA, hyperkalemia, urine pH <5.5 › hyperkalemia correction.

MRA, RASi, heparin, calcineurin inhibitors, AI, DM, urinary obstruction.

Metabolic Alkalosis

Normotension/hypotension:

  • Urine Cl >20 › Bartter, Gitelman, diuretics
  • Urine Cl <20 › vomiting, NG suction › IVF, K replacement

HTN:

  • Low renin + low aldosterone › Cushing, Liddle, licorice, fludrocortisone, glucocorticoids
  • Low renin + high aldosterone › Conn
  • High renin + high aldosterone › reninoma
Bartter Syndrome

Ascending loop, autosomal recessive › hypokalemic metabolic alkalosis, ↓Cl, ↓Ca, ↓Mg, hypercalciuria, ↓/normal BP, ↑renin, ↑aldosterone › K/Mg replacement, K-sparing diuretics, RASi, NSAIDs.

Aminoglycosides › acquired Bartter.

Gitelman Syndrome

Distal tubule Na/Cl co-transporter, autosomal recessive › hypokalemic metabolic alkalosis, ↓Cl, ↓Mg, hypocalciuria, ↓/normal BP, ↑renin, ↑aldosterone › K/Mg replacement, K-sparing diuretics, RASi, NSAIDs.

Liddle Syndrome

Autosomal dominant, collecting duct › hypokalemic metabolic alkalosis, HTN, ↓renin, ↓aldosterone › amiloride.

02

Sodium Disorders

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Hyponatremia

  • Isotonic (Sosm 275–295) › pseudohyponatremia: HyperTg, paraproteinemia (MM), lipoprotein X (obstructive jaundice)
  • Hypertonic (Sosm >295): hyperglycemia, mannitol, IVIG, BUN, alcohols, sorbitol, glycine
    • Corrected Na = Na + Glu/4
Hypotonic — Hypovolemic

Hypotension, tachycardia. GI losses, renal Na losses, AI › UNa <20, BUN:Cr >20, Uosm >300 › IVF.

Hypotonic — Hypervolemic

Edema, ascites. Cirrhosis, CHF, hypoalbuminemia, AKI/CKD › UNa <20 / >20 (AKI/CKD), Uosm >300 › diuretics.

Hypotonic — Euvolemic (SIADH)

SIADH, hypothyroidism, AI › UNa >20, Uosm >100.

SIADH causes: TCAs, SSRIs, carbamazepine, MDMA, opioids, phenothiazines, thiazides, pneumonia, SCLC, intracranial pathology › UNa >40, Uosm >300 › fluid restriction › demeclocycline, tolvaptan.

Hypotonic — Euvolemic (Low Solute)

Beer potomania, psychogenic polydipsia, D5W › UNa <20, Uosm <100 › fluid restriction.

Management
  • Symptomatic/acute (<48h) › 100 mL 3% hypertonic saline › ↑ Na 2–4
  • Target Na ↑ 4–6/24h
  • Over-correction › osmotic demyelination syndrome › DDAVP + D5W

Hypernatremia

  • Hypotonic fluid loss (GI, kidney, skin), inadequate fluid intake, DI, impaired ADH secretion (sarcoidosis)
  • Water deficit = (Na − 140)/140 × TBW
    • TBW = 0.5 × weight (women) or 0.6 × weight (men)
    • Correct over 48–72h › hypotonic fluids
  • Volume depletion › NS › hypotonic fluids, target ↓ Na 0.5/h
    • Rapid correction › cerebral edema
03

Potassium Disorders

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Hyperkalemia

  • Causes: increased intake, blood transfusion, CKD, AKI, ACEi/ARBs, NSAIDs, MRAs, BBs, pentamidine, cotrimoxazole, cyclosporine, AI, RTA type IV, TLS, rhabdomyolysis, burns, metabolic acidosis, insulin deficiency, hyperosmolality (glucose, mannitol), familial hyperkalemic periodic paralysis, hemolysis, heparin
  • Pseudohyperkalemia: phlebotomy, hematological malignancy
  • ECG: peaked T, short QT, prolonged PR, flat P, widened QRS, sine wave
Management
  • Stop offending medications, dietary K restriction
  • ECG changes › IV calcium gluconate
  • Dextrose + insulin, salbutamol
  • Acidosis › NaHCO3
  • K binders: patiromer, sodium zirconium cyclosilicate
  • Hypovolemia › IVF; hypervolemia › furosemide
  • Life-threatening › HD

Hypokalemia

  • Causes: vomiting, diarrhea, diuretics, decreased intake, hypomagnesemia, high insulin, hypokalemic periodic paralysis (inherited, thyrotoxicosis), metabolic alkalosis, alcohol withdrawal, hypothermia, amphetamines, antipsychotics, RTA type I/II, transcellular shifts, Conn, Bartter, Gitelman
  • ECG: prolonged PR, flat TW/TWI, U waves, STD
  • Urine K:Cr <13 › extrarenal causes
  • Complications: ileus, muscle cramps, rhabdomyolysis, hypomagnesemia, respiratory muscle weakness, arrhythmia
  • Severe › IV KCl
  • Correct hypomagnesemia and metabolic alkalosis
04

Phosphate & Magnesium

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Hypophosphatemia

  • Causes: refeeding syndrome, insulin for severe hyperglycemia, post-parathyroidectomy hungry bone syndrome, respiratory alkalosis, chronic diarrhea, chronic alcohol use disorder, hyperparathyroidism, vitamin D deficiency
  • Severe › HF, muscle weakness, rhabdomyolysis, hemolytic anemia, metabolic encephalopathy, delirium, seizures, coma, respiratory failure
  • Urine P >100 mg/d or FEPO4 >5% › renal cause
  • Oral phosphorus replacement
  • Symptomatic or P <2 mg/dL (0.65 mmol/L) › parenteral phosphate

Hypomagnesemia

  • Causes: diarrhea, steatorrhea, intestinal bypass, pancreatitis, loop/thiazide diuretics, alcohol induced, cisplatin, aminoglycosides, amphotericin B, cyclosporine, calcineurin inhibitors, post-parathyroidectomy hungry bone syndrome
  • Clinical: neuromuscular irritability, hypocalcemia, hypokalemia, cardiac arrhythmias, seizures
  • 24h urine Mg high (>2%) › renal cause
  • Mild/moderate › PO slow-release magnesium
  • Severe › IV magnesium sulfate
  • Target Mg >1 mg/dL
Hypermagnesemia

IV NS + furosemide.

Severe (>2.48): diminished tendon reflexes, flaccid paralysis, bradycardia, hypotension › IV NS + IV calcium gluconate.

05

Hypertension

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  • Definition: ABPM ≥130/80
  • Secondary contributors: NSAIDs, contraceptives, antidepressants, decongestants, stimulants, caffeine, ethanol
  • Lifestyle: exercise, weight loss, EtOH limitation, smoking cessation, Na <2 g/d, stress reduction
  • Workup: CBC, RFT, HbA1c, lipids, TSH, URM, urine ACR, ECG (LVH, IHD)
  • Treatment indications: BP ≥140/90 or ≥130/80 + high CVD risk (CVD, HF, DM, CKD, 10-year ASCVD ≥20%, age ≥75y)
Treatment by Stage
  • Stage 1 (BP 120–130/80–90) › lifestyle › ACEi/ARB or CCB or thiazide › combination
    • Stage 1 + ASCVD ≥10% › medication
  • DM/CKD › ACEi/ARB; eGFR <20–30 › loop/thiazide diuretic
  • Stage 2 (BP ≥140/90) › combination
  • Pregnancy › nifedipine, labetalol
  • White coat HTN › lifestyle modification
Secondary HTN Workup

US renal artery doppler (atherosclerosis, FMD), urine metanephrines/catecholamines (pheochromocytoma), plasma renin:aldosterone (hyperaldosteronism), 24h urine cortisol (Cushing), IGF-1 (acromegaly), TFT (hypothyroidism), PTH (hyperparathyroidism).

FMD › CTA/MRA/renal artery doppler.

Workup indications: early onset, absent FH, hypokalemia, kidney disease, resistant HTN, HTN emergency.

Renal artery stenosis: ↑Cr ≥30% ACEi/ARB, asymmetrical kidneys › RASi › angioplasty.

  • Target BP: <130/80
    • Acute thrombolysis post-CVA › BP <180/105
    • Acute post-CVA › BP <220/120
    • CKD › SBP <120
    • Age ≥60y › SBP <150, shared decision making
Hypertensive Urgency & Emergency

HTN urgency (BP >180/120, no end-organ damage) › ↓BP ≤25% first 1h › BP <160/100 next 6h › normal over 24–48h.

HTN emergency: BP >180/120 + end-organ damage (pulmonary edema, MI, CVA, AKI, aortic dissection). Same general targets unless specified below:

  • ICH › SBP <140 within 1h
  • Aortic dissection › IV BB (esmolol, labetalol) then nitroglycerin › SBP <120 within 1h
  • Acute pulmonary edema › IV nitroglycerin, nitroprusside, clevidipine
  • ACS › IV esmolol, labetalol, nitroglycerin, nicardipine
  • AKI › IV clevidipine, nicardipine
  • Eclampsia/pre-eclampsia › IV hydralazine, labetalol, nicardipine, nifedipine
  • Cocaine › IV phentolamine + benzodiazepines
06

Nephritic Syndrome

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Features

Hematuria, proteinuria <3.5 g/d, HTN.

URM › RBC casts, acanthocytes.

  • RPGN: oliguria, rising creatinine, hematuria, erythrocyte casts, proteinuria › biopsy (extensive glomerular crescents)
Anti-GBM

AKI, pulmonary hemorrhage › GC + cyclophosphamide + PLEX.

Biopsy › proliferative GN, linear Ig deposition.

ANCA-Associated

Constitutional symptoms, arthralgias, palpable purpura, sinusitis, asthma, pulmonary hemorrhage, AKI, mononeuritis multiplex › induction GC + rituximab/cyclophosphamide › maintenance rituximab/azathioprine.

Drug-induced: hydralazine, minocycline, antithyroid drugs.

Biopsy › necrotizing and crescentic pauci-immune GN.

GPA › cANCA (PR3) › ↑risk relapse. MPA › pANCA (MPO). RPGN adults.

IRGN

1–4 weeks post-infection (S aureus, strep), AMH, proliferative GN (red/brown urine, proteinuria, edema, HTN, AKI) › ↓C3, +ive ASOT, anti-DNase › treat underlying infection.

Biopsy › proliferative GN, C3, IgA, subepithelial hump-like deposits.

Proteinuria › ACEi/ARB. Edema › diuretics.

Lupus Nephritis

↓C3/C4, ANA, anti-dsDNA.

Class I/II (minimal/mesangial) › RASi.

Class III/IV (focal/diffuse) › steroids + MMF/cyclophosphamide › MMF/azathioprine.

Class V (membranous) › RASi; proteinuria >3.5 g/d › steroids + MMF/CTX/CNI/rituximab/AZA.

Class VI (advanced sclerosing) › RRT.

IgA Nephropathy

AMH, post-URTI › kidney biopsy.

Associations: celiac disease, autoimmune disease, hepatitis, HIV, cirrhosis.

Biopsy › IgA deposits.

HSP: arthritis, purpura, AKI, GI symptoms.

Proteinuria >0.5 g/d › ACEi/ARB + SGLT2i › PO low-dose steroids. RPGN › CTX.

MPGN

Immune-complex: HCV, HIV, cryoglobulinemia, infections, monoclonal gammopathy, bacterial infections, malaria, schistosomiasis, SLE, Sjögren, RA › ↓C4 › treat underlying cause.

Complement: C3 GN, dense deposit disease › MMF + steroids or eculizumab.

07

Nephrotic Syndrome

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Features & General Management

Proteinuria >3.5 g/d, hypoalbuminemia, edema, HLD, ↑risk thrombosis.

URM › oval fat bodies (Maltese cross).

  • Edema › Na restriction, loop diuretics (furosemide › metolazone)
  • DLP › statins
  • Proteinuria › RASi
  • Warfarin indications: Alb <25 + 1 of (BMI >35, inherited thrombophilia, NYHA 3–4, prolonged immobilization, proteinuria >10 g/d, recent ortho/abdo surgery)
Minimal Change Disease

Primary, Hodgkin lymphoma, leukemia, NSAIDs, COX2i, lithium, infections, TB › biopsy (foot process effacement) › GC.

FSGS

Primary, HIV, parvovirus, COVID-19, EBV, heroin, pamidronate, anabolic steroids, IFN, lithium, SCD, obesity, single kidney, reflux nephropathy › biopsy › GC or CNI.

Membranous Nephropathy

Primary (PLA2R, THSD7A), SLE (class V), solid tumors, lymphoma, HBV, HCV, malaria, syphilis, HIV, NSAIDs, anti-TNF, gold, penicillamine, sarcoidosis › biopsy › RASi + statin + diuretics.

↑Risk thrombosis.

Persistent proteinuria >3.5 g/d or anti-PLA2R >6–12 months › GC + CTX or RTX or CNI.

Diabetic Nephropathy

Longstanding DM + micro/macrovascular disease › RASi, GLP1RA.

Persistent albuminuria › finerenone. eGFR ≥20 › SGLT2i.

08

Acute Kidney Injury

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Pre-Renal

Hypovolemia (dehydration, hemorrhage, vomiting, rhabdomyolysis, TLS), CRS, HRS, sepsis, anaphylaxis, NSAIDs, contrast, RASi.

Urea:Cr >20, Uosm >500, Urine Na <20, FeNa <1%.

Renal (Intrinsic)

AIN, ATN (nephrotoxic drugs, ischemia, sepsis), GN, autoimmune, vasculitis, thrombosis, SCD, abdominal compartment syndrome, rhabdomyolysis, MAHA, cholesterol emboli.

Post-Renal

Obstructive uropathy › US or CT KUB.

Bladder › catheterization. Ureter/kidney › nephrostomy.

  • ATN: Urea:Cr 10–15, Uosm 300, Urine Na >40, FeNa >1%
    • URM › muddy brown/granular casts, tubular epithelial cells
  • CIN: 24–48h post contrast
    • Prevention › avoid nephrotoxic drugs, IVF
  • CRS › diuretics, RASi, SGLT2i
  • Post-transplant: acute rejection, cyclosporine, tacrolimus, infection (bacterial, BK virus), renal artery/vein thrombosis, hydronephrosis, recurrence, PTLD (EBV)
AIN

AKI, fever, rash, hematuria, proteinuria, eosinophilia › remove offending agent + biopsy.

Causes: drugs (NSAIDs, COX-2i, penicillins, cephalosporins, sulfonamides, rifampin, cipro, septra, allopurinol, PPIs, diuretics, immunotherapy), DRESS, infections, SLE, sarcoidosis, Sjögren, IgG4 disease.

URM › pyuria, WBC casts, eosinophils.

TINU: infection/NSAIDs/abx › young females, anterior uveitis, fever, weight loss, arthralgias, myalgias, headache, ↑CRP, anemia, eosinophilia, ↑LFTs, +ive ANCA/ANA/RF › PO prednisone.

Acute Renal Infarction

Flank pain, hematuria, ↑LDH › CTA/US doppler.

Cholesterol Emboli

Urine eosinophils, ↑ESR, eosinophilia, ↓C3/C4, livedo reticularis, blue toes, ulcerations › CTA.

Fundoscopy › Hollenhorst plaques.

Rhabdomyolysis

Myalgia, weakness, cola-colored urine, ↑CK, AKI, hyperkalemia, hyperphosphatemia, hypocalcemia, hyperuricemia, metabolic acidosis › IVF.

URM › RBC.

Causes: trauma, crush injury, compartment syndrome, severe exertion, statins, illicit drugs, NMS, malignant hyperthermia, metabolic/mitochondrial disorders, autoimmune, DM/PM, immune-mediated necrotizing myopathy, myositis secondary to infections.

Dialysis Indications

Refractory hyperkalemia/acidosis/hypervolemia, symptomatic uremia (altered mentation, asterixis, pericarditis, vomiting).

  • Abdominal compartment syndrome › ↑intravesicular pressure › surgical decompression
  • TLS: hyperkalemia, hyperphosphatemia, hypocalcemia, hyperuricemia › rasburicase
09

Nephrolithiasis

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  • Colicky flank pain, groin radiation, hematuria › ketorolac, CT KUB
  • Increase fluid intake, UO >2.5 L/d
  • 5–10 mm › tamsulosin
  • Urology indications: pyelonephritis, urosepsis, anuria, AKI, size >10 mm, bilateral obstruction, solitary kidney
Calcium Oxalate

Ethylene glycol, hyperparathyroidism, fat malabsorption, excess vitamin D/C, ileal disease › Na restriction, reduce meat intake, oxalate restriction, thiazides.

Radio-opaque.

Calcium Phosphate

RTA I, hyperparathyroidism, carbonic anhydrase inhibitors › Na restriction, reduce meat intake, thiazides, treat hyperparathyroidism.

Radio-opaque, urine pH >6.

Urate

Hemolysis, ADPKD, gout, hyperuricemia, metabolic acidosis, diarrhea › K citrate/NaHCO3, allopurinol, acetazolamide.

Radiolucent, urine pH <5.

Struvite

Magnesium ammonium phosphate. UTI (Proteus, Klebsiella, Pseudomonas, Ureaplasma, Staph) › staghorn calculi › treat UTI, urological removal.

Radio-opaque, urine pH >7.

Cystine

Autosomal recessive › K citrate/NaHCO3, acetazolamide.

Radio-opaque, urine pH <6.5.

10

Chronic Kidney Disease

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Screening & Diagnosis

Screening indications: DM, HTN, CVD, previous AKI, FH.

Diagnosis (≥3 months): urine ACR >30 mg/g, urine sediment abnormalities, persistent hematuria, electrolyte abnormalities due to tubular disorders, structural abnormalities, renal transplant, eGFR <60.

  • Lifestyle: exercise, smoking cessation, weight loss
    • G3–5 › protein 0.8 g/kg/d
  • BP: target <120–130/80 › ACEi/ARB
    • eGFR <30 › loop diuretics
  • Statin indications: age ≥50y, CAD, DM, ischemic CVA, ASCVD risk >10%
    • CI dialysis
  • Avoid contrast and NSAIDs
Electrolyte & Acid-Base Management
  • Na/K/P restriction
  • Hyperkalemia › K-restricted diet › K binders (sodium zirconium, patiromer)
  • Hyperphosphatemia › low PO4 diet › phosphate binders (sevelamer, lanthanum)
  • HCO3 <18 › PO NaHCO3
Proteinuria & Disease-Modifying Therapy
  • Target proteinuria <0.5–1 g/d
  • Albuminuria >30 mg/g › RASi
  • eGFR ≥20 › SGLT2i
  • DM + retinopathy › CKD-DM
    • eGFR >25 + ACR ≥30 › finerenone
    • eGFR >25 + ACR >100 › GLP1RA
Anemia & Bone Disease
  • Target Hb 10–11 › EPO (SE: CVA, CAD, HTN)
    • Ferritin ≤500 or TS ≤30 › IV iron
  • CKD-MBD › bisphosphonates (eGFR >30), denosumab
    • Secondary hyperparathyroidism › osteitis fibrosa cystica
    • Vitamin D deficiency › osteomalacia › vitamin D
    • Target PTH 2–9× ULN on dialysis › calcitriol (not in hyperphosphatemia/hypercalcemia), cinacalcet › parathyroidectomy
    • Hypocalcemia › calcium carbonate + calcitriol
  • Hyperuricemia + gout › allopurinol
    • Acute gout › colchicine or steroids
Dialysis & Transplant

Dialysis indications: severe acidosis, refractory hyperkalemia, volume overload, symptomatic uremia, refractory to medical management › renal transplant (eGFR 15–20).

eGFR <30 › refer for RRT education.

Uremia › fatigue, nausea, anorexia, insomnia, irritability, difficulty concentrating, confusion, pruritus, pleuritis, pericarditis.

Uremic pruritus › emollients › difelikefalin.

Renal transplant CI: systemic malignancy, chronic infection, severe CVD, neuropsychiatric disorders.

Post-transplant risks: SCC, Kaposi sarcoma, PTLD (EBV), DM › skin cancer screening.

Immunosuppressant SE: cyclosporine (HTN, ↓eGFR, DLP, hirsutism), tacrolimus CYP450 (DM, HTN, ↓eGFR), MMF (leukopenia, anemia), AZA (leukopenia), mTORi (proteinuria, DLP, diabetes, anemia, leukopenia).

PD-associated peritonitis: leukocytes >100 + PMN >50%.

  • Acquired cystic kidney disease › ↑RCC
    • RCC › gross hematuria, flank pain, polycythemia
  • CVD leading cause of death
  • Calciphylaxis RF: secondary hyperparathyroidism, long-term dialysis, obesity, DM, warfarin
  • Moderate/severe pain › tramadol, hydromorphone
11

Genetic Kidney Disease

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ADPKD

Autosomal dominant. HTN, abdominal/flank/back pain, hematuria, proteinuria, urate stones, Ca oxalate stones, UTI, thirst, polyuria, polydipsia, nocturia, erythrocytosis, hepatic cysts, diverticula, cerebral aneurysms, pancreatic cysts, MVP.

Genes: ADPKD1/ADPKD2.

Diagnosis: US › age 15–39y ≥2 cysts, age 40–59y ≥3 cysts, age ≥60y ≥4 cysts. No FH › MRI ≥10 cysts.

Management: Na restriction <2 g, fluid intake 2–3 L/d, target BP <120/80 › RASi.

eGFR ≥25 + rapid progression risk › tolvaptan.

Personal/family h/o ICH › MRA.

Tuberous Sclerosis

Ash-leaf spots, renal AMLs, seizures, developmental delay, intellectual disability, autism.

RCC screening › MRI every 1–3y.

Alport Syndrome

X-linked. Sensorineural hearing loss, lenticonus, proteinuria, HTN, CKD, microscopic hematuria › genetic testing › RASi.

TBMN

Microscopic/macroscopic hematuria, FH, no CKD.

12

Kidney Manifestations of Deposition Diseases

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MM / MGRS

Light chains, hypercalcemia, hyperviscosity syndrome › AKI, nephrotic syndrome, Fanconi syndrome.

Amyloidosis

Nephrotic syndrome, Congo red positive, apple-green birefringence.

Cryoglobulinemia

HCV › nephritic syndrome, RPGN, low C4, elevated RF.

Fibrillary Glomerulopathy

Nephrotic syndrome, biopsy › DNAJB9.