Quick look at Renal Diseases Flashcards
Minimal Change Disease
Nephrotic syndrome (mostly kids)
Foot process effacement on EM–> Albuminuria
Steroids
FSGS
Nephrotic syndrome
Black adults
IgM and C3 IF
Steroids, not all respond
Membranous Glomerulopathy
Nephrotic syndrome (white adults most common nephrosis)
RVT and PE!
silver stain spikes
Antiphospholipase A2 receptor = antigen
Diabetic Glomerulosclerosis
Nephrotic Syndrome (most common kidney dz cause)
Diffuse, KW nodules, membrane thickening
BP reduction w/ ACE I
Amyloidosis
Nephrotic Syndrome
Rheum arthritis, gamma light chain stain w/ CONGO RED
2 cause: AL amyloid (primary) AA amyloid (SAA)
treat inflamed state, symptom tx
Nephrotic syndrome
U/A protein >3.5 g/day
Oval fat bodies/maltese cross
Low albumin, edema, hyperlipidemia
ATN
Tubulointerstitial disease
Ischemic or nephrotoxic kidney failure
muddy brown casts, high FeNa
Don’t biopsy
AIN
Tubulointerstitial disease
Mostly drug induced (beta lactams, NSAIDS, PPI)
Rash/hematuria, eosinophils
Acute pyelonephritis
Tubulointerstitial, unilateral; no biopsy
CVA T, fever, pyruia
Hematogenous vs ascending (more common)
85% gram neg (e coli)
Chronic pyelonephritis
Tubulointerstitial dz
Slow onset RF, scarring and inflammation, U scars
Usually from VUR (reflux/obstruction)
Relieve reflux
Papillary necrosis
Tubulointerstitial dz
Analgesis/NSAID abuse, flank pain
SODA: Sickle cell, obstructive pyelo, DM, analgesic abuse,
Post Strep (or infectious) GN
Nephritic syndrome (Glomerulonephritis)
Mostly kids, 10-14 day onset. Sub epithelial.
LOW COMPLEMENT, high ASLO titer
IF shows IgG/C3, humps/bumps
Nephritic Syndrome
Hematuria (RBC Casts or deformed/crenated, acanthocytes) Low GFR Oliguria Edema HTN Variable proteinuria
IgA Nephropathy
Nephritic syndrome (GN) IgA and C3 deposits in mesangium Due to defective glycosylation of hinge region of IgA Normal complement; biopsy Treat w/ ACE I, steroids
Henoch Schonlein Purpura
Systemic version of IgA nephropathy
Abd pain, LE purpura, arthralgias
Lupus Nephritis
Nephritic Syndrome (GN)
“Full house staining” of IgA, IgG, IgM, C3, C1Q
LOW COMPLEMENT
ANA and anti dsDNA positive
Only treat Class III/IV focal and diff w/ steroids, most resolve, rituximab
Granulomatosis w/ polyangiitis
Nephritic Syndrome (GN) Aka "ANCA" and "Wegener's"; test for ANCA RPGN, crescentic! Pauci-immune Cavitary lesion in lungs, post URI Rituximab, steroids, plasmapheresis
Angi-GBM aka Goodpasture’s
Nephritic Syndrome (GN) RPGN, rare Onset s/p URI or viral infx Linear IgG on IF Steroids, plasmapheresis
Shiga Toxin HUS
TMA (Renal Vascular Disease)
D+, Mostly kids (hamburgers, petting zoo)
Gastroenteritis from E Coli
Acute TMA (fibrin deposition, RBC congestion)
Supportive care (dialysis, transfusion)
TMA
Vascular = Thrombotic microangiopathy Anemia (elevated LDH, low haptoglobin) Schistocytes Systemic organ damage from ischemia Thrombocytopenia Caused from endothelial injury and clot formation FIBRIN on imaging
Atypical HUS
TMA (Renal Vascular Disease)
ESRD at young age, D-
Genetic or auto-immune against complement regulating Factor H, I or MCP
LOW COMPLEMENT, high recurrence in transplant
tx: eculizumab
TTP
TMA (Renal Vascular Disease) not always renal involvement
Low platelets, bruising, anemia, normal PT/aPTT
Neurologic symptoms (AMS)
Caused from genetic or acquired ADAMTS13 deficiency
Tx: Daily plasmapheresis, rituximab
APS (Anti phospholipid Ab syndrome)
TMA (Renal Vascular Disease)
Recurrent clots in multiple organs (e.g., placenta–> miscarriages)
Arthralgia/fever/malaise, SLE or lupus like
ANA pos, false VDRL, RBC casts
Fibrin thrombi
Renal Atheroembolic Disease
Renal Vascular Disease
Worsening HTN, usually following invasive arterial procedures (CABG)
Acute renal failure
Cholesterol emboli due to loosened plaque (needle shaped inclusions on biopsy)
Pre-Renal Azotemia
Volume depletion, poor perfusion 2/2 sepsis or antihypertensives, heart failure.
Low FeNa (kidneys working still) increased BUN:Cr
Concentrated urine
Intrarenal causes (ATN specifically)
2/2 ischemia or nephrotoxins Muddy brown casts Due to nephrotoxic drugs or pigments (Hgb, Mgb) Can see hyperK, oliguria, low GFR high FeNa, Normal BUN:Cr
Post-renal failure
Obstructive from stones, tumors, BPH
Anuria (
ADPKD
Cystic Kidney Disease
Hetero AD mutation in PDK1 or PDK2, “2nd hit” causes disease (polycystins in cilia)
Adult onset HTN (high RAAS) & gross hematuria
Massively enlarged kidneys, bilateral
Extrarenal: aneurysm, hepatic cysts (80%)
Cardiac PFO or MV prolapse
tx: Nephrectomy
MCKD
Cystic Kidney Disease
AD mutation in MUC1 or UMOD
Altered proteins trapped in ER –> damage and fibrosis
Adult onset, polyuria, renal insufficiency
Biopsy: chronic tubular interstitial nephritis, BM disruption
Hyperuricemia and gout (due to low uric acid excretion)
NPHP
Cystic Kidney Disease
AR Pleiotropic mutations in NPHP1-15
Most frequent genetic cause of ESRD in kids
Retinitis pigmentosa, cerebellar vermis aplasia, liver fibrosis
BLINDNESS
AR PKD
Cystic Kidney Disease
AR mutation in PKHD1 (fibrocystin in biliary/renal cilia)
very rare, echo genetic in utero
Neonates w/ Potter’s sequence, survivors have HTN, RF and liver disease.
Potters: Lung hypoplasia, IUGR, characteristic face
Liver fibrosis, portal HTN
Bartter’s syndrome
Hypotension. Genetic disease resembling chronic furosemide use
- chronic volume depletion, high renin/aldo
- hypokalemia, met alkalosis
- mutations in either: NKCC, ROMK (these ones won’t have hypokalemia), CLC-Kb
Gittleman’s syndrome
Hypotentsion. Genetic disease resembling thiazide abuse. Mutation in NaCl transporter in DCT, mild volume depletion, hypocalciuria, hypomagnesiuria
2 subepithelial diseases
Membranous
Post-infectious
3 Mesangial diseases
IgA, amyloidosis, lupus nephritis (LN can be pretty much anywhere)
4 diseases w/ low complement
LN, MPGN, Cryo, post-infx
Diabetes insipitus
ADH not made –> dilute urine
Nephrogenic Diabetes insipitus
ADH not sensed at kidney –> dilute urine.
If you give ADH and problem doesn’t correct, it’s this one
Glucocorticoid remedial aldosteronism (GRA)
AD mutation of chimeric aldo synthase w/ regulatory elements of 11beta-hydroxylase gene –> stimulation of aldo by ACTH instead of AT II –> loss off feedback
- low renin, high aldo. Treat w/ corticosteroids
Apparent mineralcorticoid excess syndrome (AME)
Enzyme that converts cortisol to cortisone doesn’t work –> elevated cortisol, stimulates aldo receptor –> elevated Aldo
Mimicked by licorice!
Adrenal adenoma
Cushings –> cortisol secreting tumor –> same effect as AME
Also Conn syndrome (hyperaldo)
Congenital bilateral adrenal hyperplasia
Presents like Conn syndrome (hyperaldo) but congenital AR mutation. Treat w/ mineral corticoid antag.
Conn’s syndrome
Primary hyperaldo from acquired somatic KCNJ5 mutation in K+ channel
Fibromuscular dysplasia
Multiple stenoses in renal arteries; young women usually, stenotic kidney w/ “bead” appearance
Pheochromocytoma
Adrenal medulla tumor secreting Epi and norepi + metabolites, SNS overdrive. . Will see increased urine catecholamine metabolites (metanephrine); remove surgically
Liddle’s syndrome
Constitutively active ENaC in CD –> Na reabs independent of aldo stimulus
Use amiloride to block enac channel
Renal agenesis
Unilateral – decreased urine, in utero w/ potter sequence and resp insufficiency at birth
Bilateral – perinatal lethal due to lung hypoplasia
Primary VUR
intravesical ureter shortening–> reflux into kidney.
M>F, renal hypoplasia from scarring, can be prenatal (Hydronephrosis) or postnatal (post febrile illness or UTI)
Secondary VUR
Abnormal increase in bladder pressure 2/2 post urethral valves, neurogenic bladder, or functional bladder obstruction.
Renal-Coloboma Syndrome
AD Pax2 mutation –> renal hypoplasia renal sx, holes of optic nerve w/ visual impairment
Wilms’ Tumor
Mutations in WT genes, embryonic cancer of developing kidney in children.
Aka nephroblastoma
Nephrophthisis (NPH)
AR mutations in ciliary function and planar cell polarity, abnormal renal tubules, fibrosis –> ESRD, blindness, liver probs
Ascent abnormalities
Failure of unilateral ascent –> unilateral pelvic kidney (below IMA)
Bloth stuck –> lower pole fusion, horseshoe kidney
Ureteropelvic junction obstruction (UPJ)
Often congenital aperistaltic segment or aberrant impinging crossing vessels
Ca Oxalate stones
70%, dietery oxalate absorbed in colon and bound by Ca2+. Urinalysis: folded envelope appearance
Weird tidbit: low calcium diet actually increases stone risk.
Ca2+ phosphate stones
10-15%, risk: alkaline urine, hypercalciuria. Coffin lid appearance
Uric acid stones
10-15%. Risk factors: acidic urine, gout, metabolic syndrome, malignancy. Diamond shape.
Struvite stones
10-15%. Urease producing bacteria! UTI/chronic UTI. Staghorn stones
Proteus, klebsiella, haemophilus
Cystine Stones
Less than 1%
Genetic defect plus acidic urine. Microhexagonal crystals
Medication caused stones
Stone inhibitors in urine
Mg2+, citrate, tartrate, etc.
Stone likely to pass if what size?
CDK-MBD
CKD caused low bit D and phosphate retention. Elevated serum P –> FGF23–> decreased P reabsorption and D production. Complications are calcification of arteries, stomach, anywhere alkaline cells exist. Hyperparathyroidism, ectopic calcification, fractures.
RTA I
Inability to secrete H+ in distal nephron
RTAII
Proximal tubular acidosis: Capacity to reabsorb bicarb is impaired.
RTA IV
Impaired ammoniagenesis, usually have hyperkalemia (hyporenin-hypoaldo)
