Renal Path, IL Flashcards
Name the different section of the nephron
Glomerulus Proximal tubule Descending (thin) loop of Henle Ascending (thick) loop of Henle Distal convoluted tubule Connecting tubule Collecting duct
If patient develops fibrinous pericarditis from elevated BUN, does this patient have azotemia or uremia?
Uremia (development of signs and sx from circulating nitrogenous compounds)
The end result of all chronic renal parenchymal disease
CKD
Requires chronic dialysis
ESRD
is often reversible
AKI
focal and segmental: defn
focal some of the glomeruli affected
Segmental: within an individual glomerulus, different parts affected
The presence of inflammatory cells in the glomerulus
light microscopy
effacement of podocytes
EM
Deposition of antibodies on the BM
IF
Sclerosis of the glomerulus
light microscopy
Reduplication of BM
EM
Clinical significance of fetal lobulation in adulthood
none
Name two genes involved in the development of ADPKD, chromosomal location, protein encoded
PKD1 - chr 16 - Polycystin-1
PKD2 - chr 4 - Polycystin-2
More severe of ADPKD
PKD1 - age of onset of ESRD: 53
PKD2 is 69 yo
What tissue becomes cystic in ADPKD
tubules of the nephron
What may cause pain in ptns with ADPKD
Expanding cysts
Passing blood clots
Name three extrarenal lesions that may be seen in ptns with ADPKD
Hepatic cysts
Berry aneurysm
Cardiac valve anomalies
Name the gene involved in the development of ARPKD, location, protein
age group affected
PKHD1
Chr 6
fibrocystin
Pediatric
What tissue becomes cystic in ADPKD?
Collecting ducts
What does the surface of the kidney look like in ADPKD? ARPKD?
AD: cystic
AR: smooth
What extrarenal organ is almost always affected in ARPKD?
The liver - hepatic cysts are almost always present.
Also may cause Periportal fibrosis which leads to liver compromise and cirrhosis in children who survive past the neonatal period
Name the four subcategories of ARPKD
most common?
Perinatal - MC
Neonatal - MC
Infantile
Juvenile
Is nephronophthisis AD or AR?
AR
Three variants of nephronophthisis
most common?
Sporadic, nonfamilial
Familial juvenile - MC
Renal-retinal dysplasia
three genes involved in juvenile variant of neprhonophthisis
NPH1
NPH2
NPH3
Nephronophthisis
Where does the cyst occur?
Corticomedullary junction
What changes are seen in the cortex?
Nephronophthisis
Tubular atrophy
Thickening of BM of proximal and distal tubules
Interstitial fibrosis
Name extrarenal tissues that may be affected by Nephronophthisis
Extraocular muscles - motor abnormalities
Retina - dystrophy
Cerebellum
Liver - fibrosis
Which two renal cystic dz causes liver fibrosis?
who does it affect?
Nephronophthisis
ARPKD (periportal fibrosis)
Children
Name one possible serious complication of acquired (dialysis-assoc) cystic renal disease
Renal cell carcinoma
What is the most common cause of genetic renal disease in children and young adults?
Nephronophthisis
What helps distinguish a lesion on CT scan as a cyst rather than a second primary cancer or a metastasis?
Smooth edges
Avascular
Fluid signal rather than solid signal
Premature infant - autopsy shows kidneys enlarged bilaterally with smooth surfaces and elongated cysts
Additional findings of hepatic cysts and hepatic fibrosis (ARPKD)
61 yo with diabetes mellitus on dialysis for 12 yrs
Multiple 1-2 cm cortical and medullary cysts within small kidneys bilaterally
(acquired/Dialysis-associated cysts)
Multiple cystic dilations of medullary collecting ducts
Medullary sponge kidney
Normal left kidney but enlarged right kidney with variably-sized cysts
Multicystic renal dysplasia
Markedly enlarged kidneys with multiple cysts along with a polycystic liver
ADPKD, ARPKD
Bilaterally enlarged cystic kidneys along with congenital hepatic fibrosis
ARPKD
