(4.1) Renal Pathology II/III (Putthoff) Flashcards
What is this a hallmark of?
Membranoproliferative Glomerulonephritis (MPGN)
What are the major clinical characteristics of membranoproliferative glomerulonephritis (MPGN) type 1?
- Children/Young adults
- Mild hypertension
- 50% of MPGN 1 pts. develop chronic renal failure over 10 year time span
What are the major clinical manifestations of membranoproliferative glomerulonephritis (MPGN) type I SECONDARY?
- Almost exclusively in ADULTS
- Is a reflection of renal glomerular disease in pateints with (Hep C, SLE, Malignancies)
- 50% will experience chronic renal failure in 10 years
What is the other name for “membranoproliferative glomerulonephritis II”?
Dense deposit disease
What are the clinical manifestations of membranoproliferative glomerulonephritis II?
Primarily occurs in children
Less common than MPGN 1
Hematuria is a more dominant clinical finding
Severe renal disease and poorer prognosis than MGPN Type 1
If you have LOW complement levels and a glomerulonephritis syndrome… what do you most likely have?
MPGN II
What is this pattern of immunofluorescence a hallmark of?
IgA Nephropathy
What type of disease is berger?
Renal IgA nephropathy – no systemic disease
What type of disease is Henoch - Schonlein purpura (HSP)?
IgA nephropathy associated with systemic disease
What are the clinical manifestations of Henoch-Schonlein purpura (HSP)?
Skin (purpuric) manifestations
IgA nephropathy
What is the population associated with IgA nephropathy?
Caucasians
Asians
Older children and young adults
Over time with chronic glomerulonephritis, trichrome stains will reveal…
Sclerosing/obliteration of glomeruli
What are the (6) forms of primary glomerular diseases that progress to chronic glomerulonephritis?
90% = Crescentic GN
50-80% = Focal segmental glomerulosclerosis
50% = Membranoproliferative GN
30-80% = Membranous nephropathy
30-50% = IgAN
90% = Crescentic GN
NOW ON SECOND POWERPOINT SET
What are the (2) forms of hereditary nephritis?
Alport syndrome
Thin basement membrane disease
What is the appearance of alport syndrome on EM?
Irregular thickening basement membrane, lamination of the lamina densa
“Moth-earten” “frayed” appearance
What are the clinical manifestations of thin basement membrane disease (benign familial hematuria)?
- Hematuria due to thinned glomerular basement membranes
- Pts have asymptomatic microscopic hematuria
- Renal function is unremarkable
- Anomaly is due to gene mutations in alpha3 or alpha4 chains in type IV collagen
- MOST PTs are HETEROZYGOUS
Summarize the mechanism of
Type I DM
Type II DM
Type I DM = Autoimmune disease
Type II DM = Peripheral resistance to insulin
What are the two major processes that play key pathophysiologic roles as diabetic glomerular lesions develop?
Metabolic defect linked to hyperglycemia
Hemodynamic effects associated with glomerular hypertrophy
What are histologic indications of diabetic nephropathy?
Thickened tubular basement membranes
What is this image an example of?
End Stage Diabetic Nephrosclerosis
What are the histologic hallmarks of diffuse proliferative lupus nephritis?
Marked increase in cellularity
Glomerular size is greatly enlarged
What is the worst type of lupus?
Diffuse lupus nephritis (class IV)
What are two common reasons for acute tubular injury?
Ischemia
Toxic injury
What are two UNCOMMON etiologies of acute tubular injury?
Acute tubulointerstitial nephritis
Urniary obstruction
Define:
Urinary tract infection
Infection anywhere along urinary tract from renal cortex to urethra
Define:
Cystitis
Inflammation of the urinary bladder mucosa
Define:
Acute pyelonephritis
Acute bacterial infection of kidney
Define:
Chronic pyelonephritis
Recurrent or continuous long-term chronic infection of the kidney with resultant damage to pelvis/calyceal system and parenchyma that results in anatomical distortion
What are the most likely gram-negative bacteria that cause UTIs?
E. coli
Proteus
Klebsiella
Enterobacter
What does hydronephrosis mean?
Dilated ureter
What is the etiology of benign neprhosclerosis?
Increasing age
Hypertension
DM
What is the pathogenesis of benign neprhosclerosis?
Medial and intimal thickening in response to hemodynamic changes
Hyaline protein depositions in the arteriolar walls
Malignant hypertension is…
A Clinical syndrome and Medical EMERGENCY
What are the pathologic effects of malignant hypertension?
Ischemic kidneys
Elevated RENIN
Self-perpetuation cycle of damge and hypertension
What is the gross appearance of a kidney that has undergone malignant hypertension?
“Flea-bitten” appearance of renal hemorrhages
What is the definition of malignant hypertension?
Systolic >180
Diastolic >120
What are the clinical manifestations of malignant hypertension?
Papilledema
Retinal hemorrhages
Encephalopathy
Cardiovascular abnormalities
Renal failure
What is this?
Atherosclerosis
What is this?
Fibromuscular dysplasia
What are the classic features of thrombotic thrombocytopenic purpura (TTP)?
Fever
Neurologic symptoms
Microangiopathic hemolytic anemia
Thrombocytopenia
Renal failure (50% of patients)
Usually adults <40; more common in females
What is this?
Diffuse cortical necrosis
SLIDE DECK 3 NOW
What are examples of congenital disorders of the kidney?
Agenesis
Hypoplasia
Ectopic kidney
Horseshoe kidney
Renal cysts are:
Common/Uncommon?
COMMON!!!!
More than 50% of those over 50 have cysts in the renal parenchyma
Memorize this table
I know it sucks but just do it
Anotha one
Name the disorder:
“This hereditary disorder is characterized by multiple expanding cysts of both kidneys that ultimately destroy the renal parenchyma and result in renal failure”
Autosomal dominant (adult) polycystic kidney disease (ADPKD)
What is the gross appearance of ADPKD (autosomal dominant polycystic kidney disease)?
ENLARGED kidneys
Tons of cysts
85% of ADPKD patient’s have a defective _______ gene
PKD1
15% of ADPKD patient’s have a defective _______ gene
PKD2
Autosomal dominant polycystic kidney disease (ADPKD)
40% have _______
Hepatic cysts
Autosomal recessive polycystic kidney disease (ARPKD)
Population?
Children
Autosomal recessive polycystic kidney disease (ARPKD)
Gross appearance?
Autosomal recessive polycystic kidney disease (ARPKD)
What gene? What chromosome?
PKHD 1
Chromosome 6
Autosomal recessive polycystic kidney disease (ARPKD)
What are the most common clinical subtypes?
Perinatal
Neonatal
Another table to memorize
It feels like pier one imports with all these tables
Multicystic renal dysplasia
Most cases have and absent…
URETER
What does nephrolithiasis mean?
Kidney stones
