renal Flashcards

Question

normal size of kidneys

Answer 1

120 ml/min/1.73m | 20% of CO

Answer 2

Predict creatinine generation age, gender, race, sex (n.b. Extreme muscle mass e.g. cachexia/body builder = misleading

Answer 3

Chemical waste product from muscle metabolism Secreted as well as filtered! Therefore Cr clearance is > GFR Therefore inhibitors of secretion will make Cr rise and function look worse e.g. trimethoprim

Answer 4

Aldosterone (adrenal) -> decreased excretion Angiotensin II -> decreased excretion ANP - released by heart in response to high pressure -> increases excretion N.b. diuretics are not nephrotoxic but hypovolaemia is!

Answer 5

RAAS - renin-angiotensin- aldosterone system responds to low BP angiotensinogen released from liver -> travels to kidney which spots low bp at juxtaglomerular apparatus in afferent arteriole and releases renin -> converts the angiotensinogen to angiotensin I -> this gets converted to angiotensin II by ACE on surface of pulm and renal epithelium angiotensin II = 1. increase sympth activity 2. tubular Na Cl reabsorption, and k excretion, h2o retention 3. adrenal gland cortex to secrete aldosterone -> 2. 4. arteriolar vasoconstriction, increase in blood pressure 5. pituitary gland post lobe -> ADH secretion -> collecting duct: H20 absorption

Answer 6

indications HTN, heart failure, post MI dose 1.25/2.5mg PO ON SE 1. electrolytes - hyperkalaemia (by decrease aldosterone), reduce GFR 2. post hypo 3. dry cough - bradykinin 4. fatigue CI Hypersensitivity. Pregnancy. Monitor closely if hepatic/renal impairment

Answer 7

angiotensin 2 receptor blockers mech Modulation of RAAS, similar to ACEI but no dry cough indication HTN, heart failure, diabetic nephropathy dose Losartan - usually 50mg PO OD, elderly =- 25mg PO OD SE Renal impairment Postural hypotension Hyperkalaemia CI Avoid in pregnancy, beware use with other RAAS drugs esp. Aliskiren (direct renin inhibitor) and other drugs causing hyperkalaemia

Answer 8

70% proximal tubule 25% loop of henle 5% DCT 2% collecting duct

Answer 9

Potassium freely filtered at proximal tubule and loop of henle Distal secretion determines renal excretion (Na, aldosterone driven) Hypokalaemia meds: - Loop diuretics, thiazide diuretics Hyperkalaemia meds: - Spironolactone, amiloride, ACEI, ARB

Answer 10

stimulates RBC production Renal cortex acts as an O2 sensor; blood flow and oxygen requirement matched. (GFR) Therefore at low GFR anaemia may occur (GFR < 30)

Answer 11

@ proximal tubule calcitriol increases Ca and PO4 absorption from gut and suppresses PTH *This process is inhibited by FGF-23 which is increased in CKD

Answer 12

loop of henle + collecting tubule these are the pyramids

Answer 13

Move up concentration or electrochemical gradient. Energy dependent

Answer 14

Move down concentration or electrochemical gradient.

Answer 15

70% most essential stuff absorbed here ``` Nutrients - Glucose (why diabetics pee a loy) - Amino acids Ions - Na (As NA2HCO3 or NaCl), K, Cl Small plasma proteins Some urea and uric acid ```

Answer 16

3 out sodium 2 in pot maintains sodium conc gradient requires energy

Answer 17

thin descending limb water thick ascending limb - concentrates or dilutes fluid by countercurrent multiplication - 25% sodium - key transporter = NKCC2 (Na, k, Cl cotransporter - energy dependent) - diuretic = furose - effect = hypotens, hyponat, kal (dig tox), hypochlor, mag (indirect), calc (indirect), alkalosis (loss of hydrogen ions - indirect), inhibit uric acid excretion -> lead to gout barters syndrome - same effect as loop diuretic , early childhood, low serum mag, urine calc high, conc capacity severely impaired, AR, met alk

Answer 18

sodium 5% @ early DCT, 3% @ late DCT and CD determining how much sodium is excreted transporters Sodium/potassium ATPase drives reabsorption of calcium and chloride hormones Sodium reabsorption is regulated by hormones which stimulate or inhibit sodium resorption as required Aldosterone and ADH

Answer 19

function 1. Reabsorbs Na through coupled secretion of H+ or K+ ions into tubular fluid via aldosterone 2. Acidifies urine (acid-base balance) through secretion of hydrogen or ammonium (LATE) 3. Electrolyte homeostasis - Ca reabsorption via vitamin D dependent process 4. Part of juxtaglomerular apparatus (renin for haemodynamic regulation, macula densa senses intra-cellular Cl) channel Passive Na/Cl co-transporter prevents reapsorption of sodium and water hormones 1. PTH - Ca reabsorbed and phosphate excreted 2. *Aldosterone - More Na absorbed (ENaC), more K excreted (ROMK) - mainly at CD 3. ANP - causes DCT to excrete Na diuretic thiazide - Hypotension, hyponatraemia, hypokalaemia (increased delivery of sodium to distal tubule where it may be exchanged for potassium) - Over time may activate RAAS (normally prescribed with ACEI) -> hypokalaemia = cardiac arrhythmias May increase plasma concentration of glucose Gitelmans syndrome - same effect as thiazide - late childhood - serum mag always decreased, urine calc normal/low, conc cap may be impaired - AR - met alk

Answer 20

``` function Concentration of urine and acid/base balance ``` ``` cell types Principal cells (respond to aldosterone and vasopressin affecting sodium/potassium) + alpha intercalated cells + beta intercalated cells (secrete H+) ``` channels ENaC ROMK both @ principle cells hormones aldosterone and ADH/vasopressin mech Aldosterone (steroid/binds nucleus) increases expression of ENaC in apical membrane and N+/K+ antiporters Vasopressin binds V2 channels inserts aquaporin 2 channel to apical membrane allowing free movement of water Alpha intercalated cells (damage can result in distal renal tubular acidosis - classical) - secrete acid to urine Beta intercalated cells - secrete bicarbonate to urine drugs 1. aldosterone antagonists - spironolactone, competitive binding of aldosterone receptor, causes hypotension, hyponat, hyperkal, gynaecomastia 2. potassium sparing diuretic - amiloride (may use with furose to counteract pot loss - co-amilofruse) - @DCT inhibs absorb sodium and water @ ENaC - hypotension, hyponat, hyperkal liddles syndrome = opposite effect to pot sparing diuretics, acts at ENaC, causes HTN, hypokalaemia, AD, met alk

Answer 21

calc similar to sodium phosphate - glucose mag - ascending limb of loop of henle

Answer 22

what is it Numerous disorders in which excretion of acid or reabsorption of bicarbonate is absorbed disproportionately to GFR results in Hyperchloraemic metabolic acidosis + hypobicarbonataemia + decreased arterial pH + normal anion gap May be hypokalaemic or hyperkalaemic depending on defect fanconi syndrome Generalised dysfunction of renal proximal tubule -> urinary loss of bicarb, glucose, aa, phosphate, peptides, organic acids. Leads to salt wasting and volume depletion ``` types 1 = CLASSIC DISTAL inability to excrete H+ in distal tubules min urine pH >5.5 low serum pot, plasma bicarb <10 comp = stones/nephrocalcinosis ``` ``` 2= INHERITED ISOLATED PROXIMAL inability to reabsorb bicarbonate min urine PH <5.5 serum pot low-normal plasma bicarb <12-20 comp = osteomalacia ``` 3 = MIXED extremely rare caused by carbonic anhydrase II deficiency results in hypokalaemia ``` 4 = HYPERKALAEMIC DISTAL hyperkalaemia inhibits production of ammonia and decreases urine buffering capacity min urine ph <5.5 serum pot high plasma bicarb >17 ``` FANCONI'S SYNDROME myeloma proteins and various drugs cause proximal tubule injury and proximal RTA, or AD inherited bicarb <18 ``` RFs childhood urinary tract obstruction DM stones adrenal insufficency ``` pres growth retardation/FTT muscle weakness - fanconi hypoglycaemia after fructose rickets - Fanconi + T2 proximal have persistent phosphate loss distal RTA with deafness - inherited AR - H+ATPase kussmaul breathing if severe ``` ix ABG low serum bicarb high serum chloride variable pot low ph serum anion gap normal 12-18 serum aldosterone ``` MGMT classic distal T1 = sodium or pot alkali (1mmol/kg) eg shohl's solution +/- pot supplements proximal T2 and fanconi - same as above + thiazide diuretic (volume contraction will increase proximal absorption but will cause loss of potassium) hyperkalaemia + mineralcorticoid deficiency - fludrocortisone + dietary restriction of pot If resistant to mineralocorticoid give loop diuretic + potassium restriction + increased salt diet comp 1. Volume depletion - loss of sodium etc at proximal tubule dysfunction 2. Nephrocalcinosis - classic distal, increased loads of filtered calcium because of release of calc phos and calc carb in bone buffering of acidosis 3. Osteoporosis - bone buffering of acidosis leads to demineralisation 4. Growth retardation - acidosis associated with muscle catabolism 5. Renal rickets - Fanconi, can’t reabsorb phosphate

Answer 23

the negative electrolytes that cant be measured - protein, phosphate, citrate, sulfate so difference between HCO3- + Cl- and Na+ levels = anion gap

Answer 24

Glomerulonephritis Pyelonephritis Diabetes PKD

Answer 25

``` def Infection/inflammatory disease of renal parenchyma, calyces and pelvis that may be acute, recurrent or chronic ``` causes Enteric bacteria (e.g. Escherichia coli, UPEC uropathogenic with P pili) ascending from lower urinary tract or spread hematogenously to kidney Gram -ve: E. coli (60%), proteus (15%), klebsiella (15%) @Diabetes = klebsiella or candida @HIV, malignancy, transplant = candida who women <35 complicated pyelonephritis = men, preg women, structural abnormalities, immunosuppression, obstruction, catheterisation RF Age - Infants and older Anatomical abnormality - VUR, PKD, horseshoe, double ureter Foreign body - Stone, catheter Impaired renal function Immunocompromised - DM, sickle cell, transplant, malignancy, radio/chemo, alcohol, HIV, steroids Instrumentation - Cystoscopy Male sex Obstruction - BPH, stone, foreign body, bladder neck obstruction, posterior ureteral valve, neurogenic bladder Pregnant patho 1. Uncomplicated - bacterial ascent 2. Men - prostatitis and BPH cause urethral blockage -> bacteriuria -> pyelonephritis 3. Obstruction (calculi, tumour, BPH, neurogenic bladder) -> treatment failure and eventual abscess formation (re-infection) ``` pres TRIAD !!!! 1. loin pain 2. fever - or not if on steroids/antiinflam 3. renal tenderness/costovertebral angel + n/v, rigors ``` ix Urine dip: blood, protein, nitrites, leukocyte esterase Urinalysis (microscopy): WBC ≥ 10/HPF, RBC ≥ 5/HPF Gram stain: G -ve rods (e.coli, klebsiella, proteus) Urine culture: ≥ 100’000 CFU/ml FBC: leukocytosis ESR/CRP raised Blood culture (systemic infection SEPSIS) IMAGING - renal USS: gross abnormal, hydronephrosis, stones abscess contrast CT: altered perfusion, structural abnormality DMSA - renal scarring ``` mgmt mild/mod - ciprofloxacin or cefixime severe or comp or preg: admit IV ceftriaxone OR cipro OR gent (not preg) IV fluids, para catheter if needed ``` ``` comp renal failure abscess formation parenchymal renal scarring reucrrent UTI ```

Answer 26

85% ``` def Renal malignancy arising from renal parenchyma/cortex ``` types 80% clear cell/adeno renal carcinoma (renal cortical parenchyma) -> due to cholesterol and glycogen 15% papillary tumor (types 1 and 2) pres asymp + dx incidentally triad = haematuria, flank pain, abdo mass b symps left sided varicocele by invasion of left renal vein lower limb oedema RF Smoking, obesity, HTN, age, renal transplant and dialysis (15%), cystic dis +ve family history x4 risk: - Von Hippel Lindau (AD): 30% develop RCC -> born with germline loss of one VHL tumour suppressor gene (Ch3) then lose the other (2nd hit hypothesis) -> tend to be bilateral and multifocal ``` class small renal mass <4cm = more likely to be benign ``` stage T1 - confined to kidney and ≤ 7cm (a ≤ 4cm, b ≤ 7cm) T2 - confined to kidney and > 7cm T3 - to major veins or adrenals (e.g. vena cava) T4 - beyond gerota fascia LNs - para-aortic and hilar spread Direct - renal vein Lymphatic - paraaortic then mediastinal Haem - bone, liver, lung (cannonball mets + colono) ix BP - increased from renin secretion Percutaneous renal biopsy *FBC - polycythaemia (EPO) LDH - raised is poor prognosis (x 1.5) Corrected calcium - >2.5 mmol/l poor prognosis LFT - raised AST/ALT = metastatic disease (cholestasis in absence of liver mets = stauffers syndrome) *Cr - elevated with reduced clearance Urinalysis - haematuria and/or proteinuria *Abdominal/pelvis USS - cyst, mass, mets *CT abdo/pelvis - lymphadenopathy, mass, bone mets inc contralateral kidney MRI - for local invasion etc CXR - cannonball metastasis, bone scan, MRI brain/spine ``` ddx benign cyst - bosniak classification on imaging simple = benign complex = cancer ureteric/bladder cancer ``` mgmt NOTE - Contain high levels of multiple drug resistance protein P-glycoprotein therefore chemoresistant S1/2 - small mass - surgical resection, local ablation, T1 = partial T2= lap nephrectomy S3 = radical nephrectomy S4 = targeted molecular therapy: tyrosine kinase inhibitor = 1st line eg SUNITINIB +/- local radiation + bisphos for bone mets ``` comp Paraneoplastic syndrome (30% of patients) - anaemia Hypercalcaemia (PNS) Erythrocytosis (PNS) SIADH (PNS) ```

Answer 27

Wilm’s tumour -> nephroblastoma Childhood tumour of primitive renal tubules and mesenchymal cells Abdominal mass and haematuria

Answer 28

U+E+Cr: - *Elevated creatinine, *high serum potassium (or on *VBG), *metabolic acidosis Urine dip, MC + S - Infection -> leukocytes/nitrates - Glomerular disease -> blood/protein FBC - Anaemia (CKD/blood loss), leukocytosis (infx), thrombocytopenia (HUS, TTP) Imaging *priority if anuric - Renal USS -> obstruction, cysts, mass *ECG For *hyperkalaemia - increased PR, widened QRS, peaked T, sine wave Ratio serum urea:creatinine + other tests of cause - 20:1 -> pre-renal cause

Answer 29

stop nephrotoxic drugs - MED REVIEW ``` ABCDE Catherise for accurate urine output urgent ABG for k+ and ECG Urgent USS KUB for obstruction urine dip for GN/infx if uraemic, severe metabolic acidosis, severe hyperkalaemia -> dialysis ```

Answer 30

``` def Proteinuria or haematuria (evidence of kidney damage) and/or reduction in GFR to <60ml/min/1.73m2 for more than 3 months ``` ``` causes most common = DM, HTN (+AI disorder, smoking, obesity) PKD glomerular nephrotic/nephritic sydromes obstructive uropathy ``` stage Stage 1: kidney damage with normal or increased GFR (> 90) Stage 2: kidney damage with mild decrease GFR (60-89) CKD below *Stage 3a: kidney damage with mod decrease GFR (45-59) *Stage 3b: kidney damage with mod decrease GFR (30-44) *Stage 4: kidney damage with severe decrease GFR (15-29) *Stage 5: kidney failure (ESRD) with GFR < 15 -> Expected oliguric This requires RRT: haemodialysis, peritoneal dialysis, transplantation ``` pres mainly asymp HTN diabetic >50 fatigue - lack of EPO oedema - salt/water retention nausea - toxic waste/uraemia arthralgia - AI disease large prostate pruritis - uraemia anorexia - uraemia infection related glomerular disease ``` ix Serum creatinine elevated Urinalysis: haematuria or proteinuria Urine microalbumin: microalbuminuria Renal USS: small kidney, obstruction/hydronephrosis, large kidney (infiltration myeloma, amyloidosis) eGFR < 60 Blood sugar FBC - anaemia, normochromic normocytic Osteodystrophy: hypocalcaemia, hyperphosphataemia and hyperparathyroidism, high alkaline phosphatase Antibodies: autoantibodies, antibodies to streptococcal antigens of hep B/C antibodies mgmt primary prev - DM - HbA1c <7%, HTN BP <140/90, smoking cessation, BMI <27 secondary - + salt/protein restriction identify and treat cause treat anaemia - EPO alpha +/- ferrous sulfate renal bone disease - first line = reduce dietary phosphate. then calcium carbonate +/- calcitriol met acid? sodium bicarb oedema - furose educate stage 5 or uraemic - RRT proteinuria - ACEi ``` comp high risk CVD chief cause of death = HF anaemia renal osteodystrophy protein malnutrition met acidosis hyperkalaemia pulm oedema ```

Answer 31

1. Insult leads to renal injury (DM, HTN, glomerular) - loss of nephrons 2. Increase in intra-glomerular pressure with glomerular hypertrophy (adaptation to nephron loss) to maintain constant GFR 3. Increased glomerular permeability to macro-molecules (TGF-beta, protein, fatty acids) leads to mesangial matrix toxicity 4. Mesangial cell expansion, inflammation, fibrosis and glomerular scarring 5. Renal injury leads to increased angiotensin 2 production -> TGF-beta is upregulated -> collagen synthesis and glomerular scarring 6. Structural alterations and accompanying changes lead to progressive scarring and loss of kidney function 7. All result in tubulointerstitial fibrosis

Answer 32

aka the uraemic syndrome ``` symp Uraemic tinge (grey/yellow) Nausea/vomiting Itch *Uraemic encephalopathy *Pericarditis *Bleeding metallic taste delirium/seizures pericardical friction rub ``` mgmt RRT

Answer 33

when severe AKI ESRD - stage 5 CKD indications 1. Uraemia (pericarditis, gastritis, encephalopathy) 2. Pulmonary oedema (fluid retention) unresponsive med Rx 3. Severe hyperkalaemia (>6.5) unresponsive to med Rx 4. Severe hypo/hypernatramia 5. Severe metabolic acidosis (<7.0) unresponsive to bicarb 6. Severe renal failure (urea > 30) life expectancy Each year on dialysis increases risk of death by 6% Main cause of death is cardiovascular Will give 4-8 years

Answer 34

how Blood passed over semi-permeable membrane against dialysis fluid (opposite direction) Blood meets less concentrated solution and small solutes may diffuse along concentration gradient Blood drawn from AV fistula, heparin constantly infused Access requires AV fistula (connect artery to vein to bypass capillaries), CVC (VC via IJV), or synthetic graft regimen Hospital 3x per week (4 hours), home 5x per week (2-3 hours) 40% of RRT comp Access: *infection, thrombosis, aneurysm, endocarditis, stenosis *Hypotension (common), cardiac arrhythmia, air emboli Nausea, vomiting, headache, cramps *Anaphylaxis to sterilising agent *Disequilibration syndrome: restless, tremor, fits, coma good for live alone frail elderly or unsuitable for PD eg surg

Answer 35

how Dialysate infused into peritoneal cavity, uses blood in peritoneal capillaries Waste collected below (gravity) Ultrafiltration controlled by altering osmolality to draw water out of blood (e.g. glucose) access Catheter to peritoneum under LA or GA regimen May be continuous ambulatory peritoneal dialysis (CAPD) = 4 exchanges of 20 mins, or may be overnight with one or two in day 10% of RRT CI Intra-abdominal adhesions, stoma, obesity, intestinal disease comp Infection: peritonitis Catheter problems: infection, blockage, leak Constipation, fluid retention, hyperglycaemia + wt gain Hernia good for young people independant lack of access to health service

Answer 36

advantage Survival, QoL, economic, enable pregnancy, reversal of anaemia and renal bone disease ``` types Cadaveric (90%), living donor (10%) ``` place Iliac fossa ``` match 1. ABO group and crossmatch 2. HLA (tissue) match 3. Antibody screening 30% of living donors are antibody incompatible = paired/pooled programme ``` induction/main At time of transplant with basiliximab + IV methylprednisolone Long term immunosuppression: prednisolone, calcineurin inhibitors (tacrolimus/ciclosporin), anti-metabolites (azathioprine, mycophenolate mofetil) CI Cancer, active infection, uncontrolled IHD, AIDS with opportunistic infx risk Immediate operative: local infx, pain, DVT Infections due to immunosuppression (viral HSV for 4 weeks then CMV, bact, fungal) Urinary tract obstruction Drug toxicity: bone marrow suppression *Cancer (skin, lymphoma) *CV disease (main cause of death), hypertension, dyslipidaemia Rejection: - Hyperacute (mins), rare due to crossmatch - Accelerated (days), T cell mediated crisis -> fever, swollen kidney, increased Cr -> IV steroids - Acute cellular (weeks), 25% in <3 weeks -> fluid retention, rising BP, rising Cr, high dose IV steroids - Chronic (>6m), gradual rise in Cr and proteinuria, resistant HTN -> graft biopsy shows vascular changes, fibrosis and atrophy (non-responsive to immunosuppression) Disease recurrence

Answer 37

``` def Glomerular injury by a group of diseases characterised by changes in the glomerular capillaries and glomerular basement membrane. Changes are most likely immune mediated ``` causes Leukocyte infiltration, antibody deposition, complement activation Commonly idiopathic Infection - GABH streptococcus (pyogenes), resp/GI infection, hep B/C Systemic inflammatory conditions - SLE, RA, Goodpasture’s, Wegener’s, HSP, HUS, scleroderma Drugs - Penicillamine, gold, NSAIDs, ciclosporin, mitomycin Metabolic - DM, HTN Malignancy, hereditary, deposition - lung/colorectal, fabry's/alport syndrome, amyloidosis pres RFs + haematuria + oedema + HTN + oliguria Anorexia/nauseas -> vasculitic Weight loss -> systemic Fever -> infectious aetiology Skin rash -> vasculitic Arthralgia -> vasculitic Abdo pain -> HSP and post-streptococcal glomerulonephritis Haemoptysis -> in anti-glomerular basement membrane disease and Wegener’s ix FBC - anaemia -> systemic U + E + Cr + LFT -> ?hepatitis, advanced disease, albumin Urinalysis -> haematuria, proteinuria, RBC casts GFR -> normal or reduced Lipid profile + glucose 24 hour urine collection ESR - vasculitis Complement - low in immune complex RF - RA ANCA - anti GBM disease Anti GBM antibody - anti-GBM disease or Goodpasture’s Antistreptolysin O antibody/anti DNase - post strep Anti DS DNA/ANA - SLE Hep B/C/HIV serology Electrophoresis - raised gamma globulin in lymphoma and amyloidosis *Renal biopsy and light or electron + immunofluorescence microscopy mgmt mild - treat cause + supportive E.g. antibiotics, antivirals, withdraw drug, *limit salt + fluid + ABX if post strep -> phenoxymethylpenicillin (or IM benpen 1 dose) mod-severe - ORAL Proteinuria reducing meds (ACEI/ARB), + ABX + furosemide + prednisolone (1 mg/kg/day) with immunosuppressant e.g. cyclophosphamide/az/mm (nephrotic) rapidly progressing -IV 1. anti-GBM - plasma exchange (remove aB) + IV methylprednisolone + IV cyclophos 2. Immune complex - IV methylpred or oral pred ± ABX 3. Lupus nephritis - IV methylprednisolone + cyclophosphamide

Answer 38

NEPHROTIC = NON-PROLIF 1. Deposition disease (amyloidosis + light chain dep) 2. Minimal change disease - abnormal podocytes, pred + support 3. Focal and segmental GN - steroids ineffective, 50% RF 4. Membranous nephropathy 5. Membranoproliferative GN - thickened glomerular basement membrane, 1/3 chronic, 1/3 remission 1/3 progress to RF Proteinuria (>3.5g/24 hours) Hypoalbuminaemia (<30g/L) Peripheral oedema Hyperlipidaemia NEPHRITIC = PROLIFERATIVE IgA nephropathy (1ary) - most common, 24-48 hours post URTI/GI infection Postinfectious GN (1ary) - weeks after URTI, strep pyogenes, supportive Rapidly progressive GN - Vasculitis - wegeners, microscopic polyangitis - Anti-GBM GN (1ary) - good pastures Oliguria/AKI (renal dysfunction) HTN Haematuria: active urinary sediment (red cells and casts)

Answer 39

patho 1. immune mediated 2. cellular immune response -> infiltration of glom by LC and MP and crescent formation in absence of antibody deposition (min change, focal glomerulosclerosis) 3. humoral immune response -> immune deposit formation and complement activation in glom. Antibodies deposited when react with intrinsic autoantigens (AGBM disease) or with extrinsic antigens trapped in glom (post-infective). Injury due to activation and release of inflammatory mediators (complement, cytokines, growth factors) that lead to structural and functional characteristics of disease 4. non-immune - e.g. hyperglycaemia (diabetic nephropathy), high intraglomerular pressure (systemic HTN)

Answer 40

causes PRIMARY children = minimal change disease younger adult = focal segmental glomerulosclerosis adult = membranous nephropathy *antiphospholipase A2 antibody diabetics - diabetic nephropathy SECONDARY (more freq in females): diabetes, amyloidosis, SLE, drugs (see above), infection ddx CCF (increased JVP, pulmonary oedema, oedema) Liver disease (decreased albumin) comp Susceptibility to infection - increased urinary loss of IgG (streptococcal), or secondary to steroids Hypercoagubility and thromboembolism - renal vein thrombosis (lupus or urinary loss of antithrombin, altered protein C and S) Hypercholesterolaemia - increased hepatic lipoprotein synthesis and loss of lipid regulating proteins Hypervolaemia - severe decrease in GFR resulting in oedema AKI - more likely with acute GN HTN - impaired GFR and increased reabsorption of salt and water

Answer 41

An acute kidney injury with rapid deterioration in function CAUSES Most common at adults - Buerger’s disease: 1. Macroscopic haematuria 24/48 hours post GI/URTI 2. IgA deposition in mesangial matrix Membranoproliferative: 1. Primary (immune mediated) or secondary (SLE) 2. Thickening of glomerular basement membrane and mesangium Post infection 1. Weeks after URTI -> strep pyogenes -> resolves Rapidly progressive glomerulonephritis = CRESCENTIC - fibrin, epithelial and inflammatory cell matrix compress the glomerulus 1. Goodpasture’s syndrome - AI, antiGBM 2. Vasculitic: Wegener’s - cANCA, Microscopic polyangiitis - pANCA

Answer 42

``` def inherited cystic disease of the kidneys ``` forms 2 forms, ADPKD (most common) and ARPKD characteristics Renal cysts, extrarenal cysts, intracranial aneurysms, aortic root dilation and aneurysms, mitral valve prolapse, abdominal wall hernias causes 2 genes for ADPKD - PKD1 @85% (Ch 16) encodes polycystin 1 - PKD2 @15% (Ch 4) encodes polycystin 2 ``` patho Cysts (from tubular portion of nephron and CD) -> compression of normal renal architecture and intrarenal vasculature, increased renal size, interstitial fibrosis and tubular atrophy ``` ``` pres fhx of PKD/ESRD or stroke flank/abdo discomfort lumbar pain haematuria HTN DUFS - infection, UTI palp kidneys headaches - intracranial aneurysm hepatomeg - liver cystic locations - liver (most common extra-renal manifestation), panc, seminal vesicles, brain ``` ix Renal USS -> Ravine’s criteria (PPV = 100%) <30 years - 2 total 30-60 - 2 in each 60 - 4 or more cysts in each No fam Hx = 10 in each Genetic testing - PKD1 or PKD2 mutation CT abdo pelvis: <30 = 2 or more unilateral or bilateral cysts, 30-60 = 2 cysts in each kidney, 60+ = 4 cysts in each kidney No family history requires more than 10 cysts in each kidney Relevant further testing: - Urinalysis (protein, bacteria), ECG - LVH + echo - aortic root dilation, MR angiography - screen for aneurysm ``` mgmt symptomatic mgmt IC haem - neurosurg + nimodipine HTN - ACEi or ARB <130/80 UTI - cipro *Target fluid secretion - Calcium mimetics - CFTR inhibitors - Metformin *Target cell proliferation - Somatostatin ``` comp HTN, increased CV morbidity, CKD, ruptured intracranial aneurysm -> SAH, ESRD

Answer 43

Chromosome 6: fibrocystin -> renal collecting ducts and bile ducts Patients often present in the neonatal period with enlarged echogenic kidneys, renal cysts congenital hepatic fibrosis. This has a high mortality and many require ventilation If survive infancy, probability of surviving till 15 is low (50-80%)