Renal Pathology

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Renal Pathology
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  1. Potter's Syndrome
    • Oligohydramnios leads to compression of the fetus
    • 1. Limb deformities
    • 2. Facial deformities (flat face with low set ears)
    • 3. Lung hypoplasia (cause of death)

    "Babies who can Pee in utero develop Potter's"

    • Causes:
    • -ARPKD
    • -posterior urethral valves
    • -bilateral renal agenesis
  2. Horseshoe Kidney
    -most common renal congenital abnormality

    • Pathophysiology:
    • -inferior poles of both kidneys fuse
    • -as they ascend from the pelvis during fetal development the get trapped under the IMA and remain low in the abdomen
    • -kidney functions normally

    Associated with Turner Syndrome
  3. Multicystic Dysplastic Kidney
    • Pathophysiology:
    • -due to abnormal interaction between ureteric bud and metanephric mesenchyme
    • -leads to a nonfunctional kidney consisting of cysts and connective tissue

    If unilateral (most common) generally asymptomatic with compensatory hypertrophy of contralateral kidney

    -often diagnosed prenatally via U/S
  4. Acid Base Physiology
    • Metabolic Acidosis
    • Metabolic Alkalosis
    • Respiratory Acidosis
    • Respiratory Alkalosis

    • Henderson-Hasselbalch Equation:
    • pH = 6.1 + log [HCO3-]/(0.03 PCO2)

    • Winters Formula:
    • -predicts the respiratory compensation for a simple metabolic acidosis
    • PCO2 = 1.5 (HCO3-) + 8 ± 2

    • *HINT: last two digits of pH = PCO2
    • -if measured PCO2 differs significantly from predicted PCO2 then a mixed acid-base disorder is likely present
  5. Acidosis/Alkalosis Workup
    • 1. Check ABG pH
    • 2. Check PCO2 (determine if it's metabolic and respiratory)
    • 3. Estimate PCO2 and HCO3- compensation to determine if it's simple or mixed

    **in metabolic acidosis check serum anion gap
  6. Respiratory Acidosis
    • pH < 7.4
    • PCO2 > 40 mmHg

    • Caused by Hypoventilation:
    • -airway obstruction
    • -acute lung disease
    • -chronic lung disease
    • -opioids, sedatives
    • -weakening of respiratory muscles
  7. Metabolic Acidosis
    • pH < 7.4
    • PCO2 < 40 mmHg (compensatory hyperventilation)

    • Check serum anion gap
    • Anion gap = Na+ - (Cl- + HCO3-)
    • Normal = 8-12

    • Elevated Anion Gap
    • "MUDPILES"
    • Methanol (formic acid)
    • Uremia
    • DKA
    • Propylene glycol
    • Iron tablets or INH
    • Lactic acidosis
    • Ethylene glycol
    • Salicylates (late)

    • Normal Anion Gap (8-12)
    • "HARD-ASS"
    • Hyperalimentation
    • Addison's Disease
    • Renal Tubular Acidosis
    • Diarrhea
    • Acetazolamide
    • Spironolactone
    • Saline Infusion
  8. Respiratory Alkalosis
    • pH > 7.4
    • PCO2 < 40 mmHg

    • Causes:
    • -Hyperventilation (early high altitude exposure)
    • -Salicylates (early)
  9. Metabolic Alkalosis
    • pH > 7.4
    • PCO2 > 40 mmHg (compensation)

    • Causes:
    • -Loop diuretics
    • -Vomiting
    • -Antacid use
    • -Hyperaldosteronism
  10. Renal Tubular Acidosis
    • Type 1 ("distal")
    • -defect in collecting tubule's ability to excrete H+
    • -untx patients have urine pH > 5.5 (can't acidify urine)
    • -hypokalemia
    • -increased risk for calcium phosphate stones (↑ urine pH and bone resorption)

    • Type 2 ("proximal")
    • -defect in proximal tubule HCO3- reabsorption
    • -may be seen with Fanconi's Syndrome
    • -untx patients have urine pH < 5.5
    • -hypokalemia
    • -increased risk for hypophosphatemic rickets

    • Type 4 ("hyperkalemic")
    • -hypoaldosteronism or lack of collecting tubule response to aldosterone → hyperkalemia
    • -hyperkalemia impairs ammoniagenesis in the proximal tubule
    • -↓buffering capacity and ↓pH of urine
  11. Casts in Urine
    -casts indicate that hematuria/pyuria is of renal (vs bladder) origin

    • RBC Casts
    • -glomerulonephritis
    • -ischemia
    • -malignant HTN
    • *Bladder cancer/kidney stones → hematuria WITHOUT casts

    • WBC Casts
    • -tubulointerstitial inflammation
    • -acute pyelonephritis
    • -transplant rejection
    • *Acute cystitis → pyuria WITHOUT casts

    • Fatty Casts ("oval fat bodies")
    • -Nephrotic syndrome

    • Granular ("muddy brown") Casts
    • -acute tubular necrosis

    • Waxy Casts
    • -advanced renal disease/chronic renal failure

    • Hyaline Casts
    • -non-specific
    • -can be a normal finding
  12. Nomenclature of Glomerular Disorders
    -Focal
    -Diffuse
    -Proliferative
    -Membranous
    -Primary
    -Secondary
    • Focal
    • -<50% of glomeruli involved
    • -EG: FSGS

    • Diffuse
    • ->50% glomeruli involved
    • -EG: diffuse proliferative glomerulonephritis

    • Proliferative
    • -hypercellular glomeruli
    • -EG: mesangial proliferative

    • Membranous
    • -thickening of GBM
    • -EG: membranous nephropathy

    • Primary Glomerular Disease
    • -involves only glomeruli, primary disease of kidney
    • -EG: minimal change disease

    • Secondary Glomerular Disease
    • -involves glomeruli and other organs (systemic disease that has an impact on kidney)
    • -EG: SLE, diabetic nephropathy
  13. Nephritic Syndromes
    "NephrItic syndrome = an Inflammatory process"

    • Clinical Presentation:
    • -hematuria
    • -RBC casts in urine
    • -azotemia
    • -oliguria
    • -HTN (salt retention)
    • -proteinuria (<3.5 g/day)

    • Types:
    • -Acute poststreptococcal glomerulonephritis
    • -Rapidly progressive glomerulonephritis
    • -Berger's IgA glomerulonephropathy
    • -Alport Syndrome

    • Both Nephritic and Nephrotic
    • -Diffuse proliferative glomerulonephritis
    • -Membranoproliferative glomerulonephritis
  14. Nephrotic Syndrome
    "NephrOtic syndrome presents with massive prOteinuria"

    • Clinical Presentation:
    • -proteinuria (>3.5 g/d → frothy urine)
    • -hyperlipidemia
    • -fatty casts
    • -hypoalbuminemia → edema
    • -thromboembolism (hypercoagulable due to loss of ATIII)
    • -increased risk of infection (loss of Igs)

    • Types:
    • -Focal segmental glomerulosclerosis
    • -Membranous nephropathy
    • -Minimal change disease
    • -Amyloidosis
    • -Diabetic glomerulonephropathy

    • Both Nephritic and Nephrotic
    • -Diffuse proliferative glomerulonephritis
    • -Membranoproliferative glomerulonephritis
  15. Focal Segmental Glomerulosclerosis
    -most common cause of nephrotic syndrome in adults (esp Hispanics and African Americans)

    • Associated with:
    • -HIV infection
    • -heroin abuse
    • -massive obesity
    • -interferon tx
    • -CKD due to congenital absence or surgical removal

    • LM:
    • -segmental sclerosis and hyalinosis

    • EM:
    • -effacement of foot processes similar to minimal change disease

    • IF:
    • -negative

    • Prognosis:
    • -most progress to chronic renal failure
  16. Membranous Nephropathy
    -second most common cause of primary nephrotic syndrome in adults (most common in Caucasian adults)

    • Causes:
    • -idiopathic
    • -drugs (NSAIDs, penicillamine)
    • -infection (hepatitis B or C)
    • -SLE
    • -solid tumors

    • LM:
    • -diffuse capillary and GBM thickening

    • EM:
    • -"spike and dome" appearance
    • -subepithelial deposits

    • IF:
    • -granular pattern

    **SLE's nephrotic presentation (usually nephritic)
  17. Minimal Change Disease
    "Lipoid Nephrosis"

    • Epidemiology:
    • -Most common cause of nephrotic syndrome in children

    • Pathophysiology:
    • -Usually idiopathic, may be associated with Hodgkin Lymphoma (massive cytokine production)
    • -damaged mediated by cytokines from T cells
    • -may be triggered by a recent infection or immune stimulus

    • LM:
    • -normal glomeruli

    • EM:
    • -foot process effacement → selective loss of albumin (not globulins) caused by GBM polyanion loss

    • IF:
    • -negative

    • Prognosis and Treatment:
    • -excellent response to steroids!
  18. Amyloidosis
    • Pathophysiology:
    • -amyloid deposits in the mesangium
    • -kidney most commonly involved organ in systemic amyloidosis

    • LM:
    • -congo red stain shows apple green birefringence under polarized light

    *associated with chronic conditions (MM, TB, RA)
  19. Membranoproliferative glomerulonephritis (MPGN)
    • Thick GBM
    • Type I:
    • -associated with HBV and HCV
    • -subendothelial immune complex deposits
    • -"tram track" appearance (due to GBM splitting caused by mesangial ingrowth)

    • Type II:
    • -intramembranous IC deposits
    • -"dense deposits
    • -associated with C3 nephrotic factor (stabilizes C3 convertase → overactive C3)

    **can also present as nephritic syndrome
  20. Diabetic Glomerulonephropathy
    • Pathophysiology:
    • -nonenzymatic glycosylation (NEG) of GBM
    • -leads to ↑ permeability and thickening
    • -NEG of efferent arteriole more than afferent → increased GFR → mesangial expansion
    • -hyperfiltration leads to microalbuminuria
    • -eventually progressed to nephrotic syndrome

    • LM:
    • -mesangial expansion
    • -GBM thickening
    • -eosinophilic nodular sclerosis (Kimmelstiel-Wilson Lesions)

    • Tx:
    • -ACEIs slow progression of hyperfiltration induced damage
  21. Acute Poststreptococcal Glomerulonephritis
    • Epidemiology:
    • -most frequently seen in children
    • -arises after group A β-hemolytic strep infection of skin or pharynx (nephritogenic strains with M protein virulence factor)
    • -may occur with non-strep organisms as well

    • Clinical Presentation:
    • -2-3 weeks after infection
    • -hematuria (cola colored urine)
    • -oliguria
    • -HTN
    • -periorbital edema

    • LM:
    • -glomeruli enlarged and hypercellular
    • -neutrophils present
    • -"lumpy-bumpy" appearance

    • EM:
    • -subepithelial immune complex humps

    • IF:
    • -granular appearance due to IgG, IgM and C3 deposition along GBM and mesangium

    • Treatment:
    • -supportive
    • -often resolves spontaneously
  22. Rapidly Progressive Glomerulonephritis (RPGN)
    "Crescentic"

    -nephritic syndrome that progresses to renal failure in weeks to months

    • LM:
    • -crescents made of fibrin and plasma proteins with glomerular parietal cells, monocytes and macrophages

    • 1. Linear
    • -anti-basement membrane antibody
    • -Goodpasture syndrome (Ab against glomerular and alveolar BM, type II hypersensitivity)
    • -presents as hematura/hemoptysis
    • -classically = young adult males

    • 2. Granular
    • -most commonly PSGN
    • -most common type of renal disease in SLE
    • -usually subendothelial

    • 3. Pauci-immune
    • -negative IF
    • -Wegener Granulomatosis (associated with C-ANCA)
    • -Microscopic polyangiitis (P-ANCA)
    • -Churg-Strauss Syndrome (P-ANCA, granulomatous inflammation, eosinophilia, asthma)

    • Prognosis:
    • -poor
    • -rapidly deteriorating renal function
  23. Diffuse Proliferative Glomerulonephritis
    • Etiology:
    • -due to SLE or MPGN
    • -most common cause of cell death in SLE
    • -SLE and MPGN can present as both nephrotic and nephritic syndromes

    • LM:
    • -"wire looping" of capillaries

    • EM:
    • -subendothelial and sometimes intramembranous IgG-based immune complexes, often with C3 deposition

    • IF:
    • -granular
  24. Berger's Disease (IgA Nephropathy)
    • Epidemiology:
    • -most common nephropathy worldwide
    • -related to Henoch-Schonlein purpura

    • Clinical Presentation:
    • -presents during childhood as episodic gross microscopic hematuria with RBC casts
    • -often presents/flares with URI or acute gastroenteritis

    • LM:
    • -mesangial proliferation

    • EM:
    • -mesangial Immune complex deposits

    • IF:
    • -IgA-based deposits in mesangium
  25. Alport Syndrome
    • Pathophysiology:
    • -inherited defect in type IV collagen
    • -most commonly X-linked
    • -results in thinning and splitting of the GBM

    • Clinical Presentation:
    • -isolated hematuria
    • -sensory hearing loss
    • -ocular disturbances (less common)
  26. Nephrolithiasis
    Kidney Stones

    • -can lead to severe complications (hydronephrosis and pyelonephritis)
    • -tx and prevent by encouraging fluid intake

    • Types:
    • 1. Calcium
    • 2. Ammonium Magnesium Phosphate (struvite)
    • 3. Uric acid
    • 4. Cystine
  27. Calcium Kidney Stones
    -calcium oxalate, calcium phosphate or both

    • Frequency:
    • -80%
    • -most common type
    • -usually seen in adults

    • Precipitates at:
    • -↑pH (calcium phosphate)
    • -↓pH (calcium oxalate)

    • Xray:
    • -radiopaque

    • Caused by:
    • -most commonly idiopathic hypercalciuria (with normocalcemia)
    • -conditions that cause hypercalcemia (cancer, elevated PTH)
    • -oxalate crystals can result from ethylene glycol or vitamin C abuse

    • Treatments:
    • -thiazide diuretics
    • -citrates
  28. Ammonium Magnesium Phosphate (Struvite) Kidney Stones
    • Frequency:
    • -15%
    • -second most common type

    • Precipitates at:
    • -↑pH (alkaline urine)

    • Xray:
    • -radiopaque

    • Caused by:
    • -infection with urease positive bugs (Proteus mirabilis, Staph, Klebsiella)
    • -hydrolyze ammonia to make urine more alkaline

    • *Can form staghorn caliculi
    • *form nidus for UTIs

    • Treatment:
    • -surgical removal (size)
    • -eradication of pathogen (prevent recurrence)
  29. Uric Acid Stones
    • Frequency:
    • -5%
    • -third most common stone

    • Precipitates at:
    • -↓pH

    • Xray:
    • -radiolUcent
    • -BUT visible on CT and U/S

    • Caused by:
    • -hyperuricemia (gout!)
    • -often seen in diseases with increased cell turnover (leukemia)
    • -Risk Factors: hot, arid climates, low urine volume

    • Treatment:
    • -hydration and alkalinization of urine (postassium bicarbonate)
    • -allopurinol for gout
  30. Cystine Kidney Stones
    • Frequency:
    • -1%
    • -most commonly seen in children

    • Precipitates at:
    • -↓pH

    • Xray:
    • -radiopaque

    • Caused by:
    • -most often secondary to cystinuria (genetic defect of tubules → decreased reabsorption of cystine)
    • -hexagonal crystals

    • Treatment:
    • -hydration and alkalinization of urine
  31. Hydronephrosis
    • Back up of urine into the kidney
    • -dilation of renal pelvis and calyces proximal to obstruction
    • -may result in parenchymal thinning in chronic severe cases

    • Causes:
    • -urinary tract obstruction
    • -vesicoureteral reflex
  32. Renal Cell Carcinoma
    • Epidemiology:
    • -most common renal malignancy
    • -most common in men 50-70 years
    • -increased incidence with smoking and obesity

    • Clinical Presentation (Triad):
    • -fever
    • -weight loss
    • -paraneoplastic syndrome (secondary polycythemia)
    • -palpable mass
    • -flank pain

    • -invades renal vein then IVC (hematogenous spread)
    • -can prevent with a L sided varicocoele (L spermatic vein drains into L renal vein)

    -Paraneoplastic syndromes (EPO, ACTH, PTHrP)

    -silenct cancer (b/c in retroperitoneum), often presents as metastatic neoplasm

    • Pathophysiology:
    • -associated with deletion of VHL (on chromosome 3)
    • -VHL increases expression of IGF-1 and HIF
    • -can be sporadic or hereditary (part of VHL disease)

    • Pathology:
    • -originates from proximal tubule cells
    • -polygonal clear cells filled with accumulated lipids and carbohydrates

    • Treatment:
    • -resection if localized disease
    • -resistant to conventional chemo and radiation
  33. Wilm's Tumor
    "nephroblastoma"

    • Epidemiology:
    • -most common renal malignancy of early childhood
    • -ages 2-4

    • Presentation:
    • -huge, palpable flank mass
    • -hematuria
    • -HTN

    • Pathophysiology:
    • -deletion of TSG WT1 on chromosome 11
    • -may be part of WAGR syndrome or Beckwith-Wiedemann syndrome
    • -WAGR: Wilms Tumor, Aniridia, Genital abnormalities, Retardation
    • -Beckwith-Wiedermann Syndrome: Wilms tumor, neonatal hypoglycemia, muscular hemihypertrophy, organomegaly (tongue)

    • Pathology:
    • -composed of blastema (immature kidney mesenchyme), primitive glomeruli/tubules, stromal cells
  34. Transitional Cell Carcinoma
    • Epidemiology:
    • -most common tumor of urinary tract system (can occur in renal calyces, renal pelvis, ureters and bladder)
    • -Associated with problems in your Pee SAC:
    •      -Phenacetin
    •      -Smoking
    •      -Aniline dyes
    •      -Cyclophosphamide

    • Presentation:
    • -painless hematuria
    • -NO CASTS
    • -generally seen in older adults

    • Pathophysiology:
    • -Flat: high grade then invades (p53 mutations)
    • -Papillary: low grade → high grade then invades

    • Prognosis:
    • -often multifocal and recur ("field defect")
  35. Acute Pyelonephritis
    • Infection of kidney
    • -usually due to ascending infection
    • -affects cortex with relative sparing of glomeruli/vessels
    • -increased risk with vesicoureteral reflex

    • Symptoms:
    • -fever
    • -CVA tenderness
    • -nausea
    • -vomiting
    • -white cell casts in urine

    • Most common pathogens:
    • -E coli (90%)
    • -Klebsiella
    • -Enterococcus faecalis

    • Pathology:
    • -neutrophilic invasion in interstitium
  36. Chronic Pyelonephritis
    • Pathophysiology:
    • -typically requires predisposition to infection (vesicoureteral reflux or chronically obstructing kidney stones)
    • -interestitial fibrosis with atrophy of tubules due to multiple bouts of acute pyelonephritis

    • Pathology:
    • -coarse, asymmetric corticomedullary scarring
    • -blunted calyces
    • -lymphocytic invasion and fibrosis
    • -thyroidization: tubules contain eosinophilic casts
  37. Drug-Induced Interstitial Nephritis (Tubulointerstitial Nephritis)
    Acute interstitial renal inflammation

    • Clinical Presentation:
    • -pyuria (usually eosinophils)
    • -azotemia
    • -fever
    • -rash
    • -hematuria
    • -CVA tenderness
    • *can be asymptomatic

    • Pathophysiology:
    • -occurs after administration of drugs that act as haptens → hypersensitivity
    • -Drugs: diuretics, penicillin derivatives, sulfonamides, rifampin, NSAIDs
    • -usually 1-2 weeks after administration
    • -can occur months after starting NSAIDs

    • Prognosis:
    • -usually resolves with cessation of the drug
    • -may progress to renal papillary necrosis
  38. Diffuse Cortical Necrosis
    Acute generalized cortical infarction of both kidneys

    Likely due to a combination of vasospasm and DIC

    Associated with obstetric catastrophes (eg: abruptio placentae) and septic shock
  39. Acute Tubular Necrosis
    • Epidemiology:
    • -most common cause of intrinsic renal failure

    • Etiology:
    • 1. Ischemic
    • -often caused by prerenal azotemia
    • 2. Nephrotoxic
    • -aminoglycosides (most common)
    • -heavy metals
    • -myoglobinuria
    • -ethylene glycol
    • -radiocontrast dye
    • -urate (tumor lysis syndrome)

    • Clinical Presentation:
    • -oliguria
    • -granular "muddy brown" casts
    • -elevated BUN and Cr
    • -Hyperkalemia
    • -Metabolic acidosis

    • Labs:
    • -BUN:Cr < 15 (decreased reabsorption of BUN)
    • -FeNa > 2% (decrease Na+ reabsorption)
    • -Uosm < 500 (inability to concentrate urine)

    • Pathophysiology:
    • -necrotic cells plug tubules
    • -obstruction decreases GFR

    • Stages:
    • 1. Inciting Event
    • 2. Maintenance phase
    • -oliguric
    • -lasts 1-3 weeks
    • -risk of hyperkalemia
    • -death most often occurs here
    • 3. Recovery phase
    • -polyuric
    • -BUN and Cr fall toward normal
    • -risk of hypokalemia

    • Treatment:
    • -reversible
    • -usually requires supportive dialysis (electrolyte imbalances)
  40. Renal Papillary Necrosis
    Sloughing off of renal papillae

    • Symptoms:
    • -gross hematuria and proteinuria

    • Causes:
    • -recent infection
    • -immune stimulus

    • Associated with:
    • -diabetes mellitus
    • -acute pyelonephritis
    • -chronic phenacetin use (acetaminophen is a phenacetin deriviative)
    • -SCA and trait
  41. Acute Kidney Injury
    • -acute renal failure
    • -acute, severe decrease in renal function with ↑BUN and Cr over several days

    *in the normal nephron BUN is reabsorbed (for countercurrent multiplication) but Cr is not

    • Three Causes:
    • 1. Prerenal azotemia
    • 2. Intrinsic renal failure
    • 3. Postrenal azotemia

  42. Prerenal Azotemia
    • Causes:
    • -hypovolemia

    • Pathophysiology:
    • -↓RBF (hypotension) → ↓GFR
    • -Na+, H2O and urea retained by kidney in an attempt to conserve volume so BUN/Cr ratio increases

    • Labs:
    • -concentrated urine (Uosm > 500)
    • -Sodium retention (low UNa)
    • -low FeNa
    • -elevated serum BUN/Cr
  43. Intrinsic Renal Failure
    • Causes:
    • -generally due to ATN or ischemia/toxins
    • -loss commonly due to glomerulonephritis (RPGN)

    • Pathophysiology:
    • -patchy necrosis
    • -debris obstructs tubules
    • -backflow across tubule
    • -leads to decreased GFR

    • Clinical Features:
    • -epithelial/granular casts

    • Labs:
    • -unable to concentrate urine (Uosm < 350)
    • -no sodium retention (UNa > 40)
    • -FeNa >2%
    • -decreased BUN/Cr ratio (BUN reabsorption is impaired)
  44. Postrenal Azotemia
    • Causes:
    • -outflow obstruction
    • -stones
    • -BPH
    • -neoplasia
    • -congenital abnormalities

    • Pathophysiology:
    • -decreased outflow causes decreased GFR
    • -develops only with bilateral obstruction
    • -early: increased tubular pressure forces BUN into blood (BUN/Cr > 15)
    • -late: tubular damage decreases BUN absorption (BUN/Cr < 15)

    • Labs:
    • -unable to concentrate urine (Uosm < 350)
    • -unable to reabsorb Na (UNa > 40)
    • -FeNa > 2%
  45. Consequences of Renal Failure
    Inability to make urine and excrete nitrogenous wastes

    • 2 Forms:
    • -acute (ATN)
    • -chronic (HTN, DM)

    • Na+/H2O retention
    • -CHF
    • -Pulmonary Edema
    • -Hypertension

    Hyperkalemia

    Metabolic acidosis

    • Uremia
    • -increased BUN and Cr
    • -Nausea
    • -Anorexia
    • -Pericarditis
    • -Asterixis
    • -Encephalopathy
    • -Platelet dysfunction

    • Anemia
    • -failure to produced Epo

    Renal Osteodystrophy

    • Dyslipidemia
    • -especially increased TGs

    • Growth retardation/developmental delay
    • -children
  46. Renal Osteodystrophy
    • Pathophysiology:
    • -failure of Vitamin D hydroxylation
    • -hypocalcemia and hyperphosphatemia leads to secondary hyperparathyroidism
    • -hyperphosphatemia also independently lowers serum Ca2+ by causing tissue calcification
    • -UCSD SOM: phosphate binds calcium in serum → hypocalcemia

    Causes subperiosteal thinning of bones
  47. Renal Cysts
    • 1. ADPKD
    • 2. ARPKD
    • 3. Medullary cystic disease
  48. ADPKD
    Adult polycystic kidney disease

    • Pathophysiology:
    • -Autosomal Dominant mutation in PKD1 or PKD2
    • -results in multiple large, bilateral cysts that ultimately destroy the kidney parenchyma

    • Clinical Presentation:
    • -young adults
    • -hypertension (due to increased renin)
    • -hematuria
    • -worsening renal failure
    • -flank pain
    • -urinary infection

    • Associated with:
    • -berry aneurysm (common cause of death)
    • -mitral valve prolapse (common cause of death)
    • -benign hepatic cysts
  49. ARPKD
    Infantile Polycystic Kidney Disease

    • Pathophysiology:
    • -Autosomal Recessive
    • -significant renal failure in utero can lead to Potter Syndrome

    • Clinical Presentation:
    • -infantile presentation in parenchyma
    • -worsening renal failure
    • -hypertension
    • -Potter's Sequence
    • -Portal HTN

    • Associated with:
    • -congenital hepatic fibrosis and hepatic cysts
  50. Medullary Cystic Disease
    • Pathophysiology:
    • -inherited disease
    • -tubulointerstitial fibrosis

    • Clinical Presentation:
    • -progressive renal insufficiency
    • -inability to concentrate urine
    • -medullary cysts usually not visualized
    • -shrunken kidneys on U/S

    Poor Prognosis

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