cystic congenital disease TI disease

Question 1

Acute interstitial nephritis

Answer 1

inflammation of renal tubules and interstitium

Causes: hypersensitivity RXN to drugs– PCN derivatives, NSAIDs, Sulfonamides, rifampin
Infections, autoimmune diseases– sle sjorgens

Question 2

Kidney biopsy

Answer 2

Major indications: nephrotic syndrome (Proteinuria (3g/Dl), edema, low albumin
Nephritic syndrome (HTN, glomerular hematuria, azotemia)
Unexplained acute kidney injury

Pathology:

Question 3

morphology

Answer 3

inflammation and edema of interstitium with involvement of tubules (tubulitis) sparing glomeruli and vessels, lymphoctes, plasma cells, eosinophils, may see granulomas

Question 4

Acute pylonephritis

Answer 4

Acute inflammation of the kidney due to a bacterial infection (urinary route, hematogenous route)

Typical Urinary tract pathogens, usually gram negative bacilli

Predisposing conditions: urinary obstruction (congenital or acquired), urinary tract intrumemtation, vesicouretral reflux, pregnancy, diabetes

Question 5

Multiple myeloma

Answer 5

Renal failure develops in 25% of patients
Chronic renal failure results from: direct tubular toxicity of light chain, tubular obstruction by casts, interstitial inflammation

Question 6

Multiple myeloma cast nephropathy

Answer 6

Due to excessive production and urinary excretion of light chains
Present as AKI

Factors that favor intratubular precipitation and cast formaton (hyper calcemia, volume depletion, nephrotoxins)

Presenting features (older pts, renal insufficiency, proteinuria, history of bone pain, fractures, anemia, hypercalcemia, monoclonal light chains in blood or urine

Renal biopsy helpful

FRACTURED CASTS

Question 7

Myeloma cast nephropathy

Answer 7

treatment: acutely hydration and urinary alkalinization to prevent tubular obstruction by casts, chemotherapy or Stem cell transplantation

Question 8

Cystic disease of the kidney

Answer 8

Heterogenous (hereditary, developmental, acquired disorders)

Simple cysts: generally innocuous, 1-5 cm in diameter translucent, filled with cleat fluid, cysts are usually confined to the cortex but massive cysts (10 cm) can occur, Smooth contours avascular

Acquired cystic kidney disease: occurs in End stage renal disease (ESRD), multiple cysts present in cortex medulla, risk of renal neoplasms is 100x greater than general population

Question 9

Autosomal dominant polycystic kidney disease

Answer 9

Multiple expanding cysts affecting both kidneys, cysts destroy renal parenchyma

90%- PKD 1, more severe, chromosome 16 , polycystin 1

10% PKD- chromosome 4, encodes polycystin 2, less severe progression in kidney function

Question 10

Extrarenal manifestations of ADPKD

Answer 10

Saccular aneurysms of the circle willis
10-30% of patients, High incidence of subarachnoid hemorrhage (thunderclap headache
Screening MRI
Polycystic liver disease

Question 11

Nephronophthisis

Answer 11

Rare, nephronophtisis (progressive loss of nephrons,

Question 12

Tuberous sclerosis

Answer 12

Tumor suppressor gene syndrome, hamartomas

Kidney, brain, heart lung and skin

Question 13

Von Hippel lindau

Answer 13

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