Lecture 8- Renal Disease 1 Flashcards
… is defined as elevated BUN and creatinine levels, due to decreased GFR
Azotemia
… is defined as azotemia (elevated BUN and creatinine) plus other symptoms (gastroenteritis, peripheral neuropathy, dermatitis, acidosis, pericarditis, hyperkalemia)
Uremia
3 major clinical renal syndromes
acute nephritic syndrome
nephrotic syndrome
acute renal failure
Most significant symptom of acute nephritic syndrome
hematuria
Most significant symptom of nephrotic syndrome
severe proteinuria
Most significant symptom of acute renal failure
oliguria/anuria
2 congenital cystic renal diseases
autosomal dominant (adult) polycystic kidney disease
autosomal recessive (childhood) polycystic kidney disease
AD adult polycystic kidney disease is seen in 1 out of every … people and is characterized by…
500-1000
multiple expanding cysts in both kidneys
What is the deective gene in AD adult polycystic kidney disease? what does this gene encode for?
PKD1 located on chromosome 16
polycystin-1
what are the extrarenal symptoms in AD adult polycystic kidney disease?
1/3 of patients have cysts in liver
saccular “berry” anueryms in the circle of willis
How are the kidneys described in AD adult polycystic kidney disease?
very large (up to 4 kg) with numerous cytss that arise in every part of the tubular system
Clinical presentation of AD adult polycystic kidney disease
flank pain around 4th decade, hematuria, HTN, uti, renal failure
AR childhood polycystic kidney disease happens in 1 out of … live births
20,000
AR childhood polycytic kidney disease is due to mutations in … gene
PKHD1
Defective protein on AR childhood polycystic kidney disease?
fibrocystin
when does renal failure occur in AR p.c. kidney disease
shortly after birth to several years
AR p.c. kidney disease have numerous … that arise from the collecting tubules
uniform size cysts
What is the extrarenal pathology of AR p.c. kidney disease?
liver cysts and progressive liver fibrosis
what is the most obvious clinical sign of nephrotic syndrome
severe edema (because of the heavy proteinuria)
4 different types of nephrotic syndrome
minimal change disease
focal segmental glomerulosclerosis
membranous neuropathy
nodular glomerulosclerosis (DM)
Which nephrotic syndrome is most common in children (2/3 of all cases)
minimal change disease
What can you see in electron microscopy of minimal change disease?
epithelial foot process effacement
What can you see in light microscopy of minimal change disease?
nothing- normal appearing glomeruli
What can you see in immunofluroesence in minimal change disease?
nothing- no immune complexes
Treatment for minimal change disease?
corticosteroid - good response
Common cause of nephrotic syndrome in adults?
focal and segmental glomerulosclerosis
is focal segmental glomerulosclerosis primary (idiopathic), secondary, or genetic ?
can be any of the 3
What can you see in light microscopy of focal segmental glomerulosclerosis?
focal (some gloreuli) and segmental (part of involved glomerulus) sclerosis with obliteration of capillary loops
What can you see in EM and IF of focal segmental glomerulosclerosis?
no immune complex deposits in idiopathic form
What is the response to treatment in focal segmental glomerulosclerosis?
poor response to corticosteroid treatment
What percent of patients progress to renal failure after 10 years with focal segmental glomerulosclerosis?
50%
Which kind of nephrotic syndrome if most commone in adults age 30-50 but may be seen in children?
membranous neuropathy
Membranous neuropathy may be primary (disease limited to the ….) or secondary to …,…,… or …
idney
infection, malignancy, SLE, or drugs
Pathology of membranous neuropathy:
LM:
IF:
EM:
LM: nearly normal
IF: immune complex deposits
EM: depostis in subepithelial side of the GBM
WHat is the response to treatment in membranous neuropathy?
poor response to corticosteroid tx
Pathology of glomerular disease in diabetes mellitus:
LM:
IF:
EM:
LM: nodular glomerulosclerosis (know this)
IF: no immune complex deposits
EM: thick GBM
3 other changes you see in glomerular disease in diabetics
hyaline arteriolosclerosis
atherosclerosis
nephrosclerosis
renal failure is … to MI as cause of death in diabetics
2nd
what disease do you see a kimmelstiel-wilson lesion?
glomerulo disease in diabetes
Nephritic syndrome is charactarized by
- Hematuria (know this)
- oliguria and azotemia
- HTN
2 major forms of nephritic syndrome
acute postinfectious (poststreptococcal) glomerulonephritis
IgA neuropathy
acute postinfectious glomerulonephritis is most common in … about 1-4 weeks after a bout of ….
children
streptococcal pharyngitis
Pathology of acute postinfect glom.nephr.
LM:
IF:
EM:
LM: proliferation of endothelial and mesangial cells, inflamm cells; may develop into cellular crests
IF: immune complex deposition, granular
EM: immune complexes in GBM + or - mesangium
… is one of the most common causes of renal disease
IgA neuropathy
… affects children and young adults, heamaturia is noted 1-2 days post upper respiratory tract infection; resolves then recurs
IgA neuropathy
When renal disease is associated with pupuric skin rash, GI pain, arthritis then its called
Henoch-Schonlein pupura
Pathology of IgA neuropathy
LM:
IF and EM:
LM: variable mesangial proliferation
IF and EM: immune (IgA) complexes within the mesangium
What is the characteristic finding of rapidly progressive glomerulonephritis?
crescentic glomerulonephritis due to proliferation of epithelial cells with infiltration of histiocytes
… is an acute clinical syndrome but not a specific form of glomerulonephritis
crescentic or rapidly progressing glomerulonephritis
