renal pathology Flashcards
agenesis
born without kidneys
hypoplasia
small, distorted kidneys
horshoe kidney
malformation
ectopic kidneys
not in location
PKD that has an adult life presentation:
- inheritance
- presents in adulthood as
- what do we see
- what usually occurs at midlife
- inheritance: autosomal dominant
- presents in adulthood as mass, pain, hematuria and HTN
- we see enlarged kidneys with large cysts and berry aneurisms
- ESRDoccurs at midlife
pathophysiological mechanism in PKD- AD (2)
- cell proliferation, alt. in cell planar polarity, remodeling of the ECM, cell cilia malfunction
- transepithelial fluid secretion leading to the outpouching
PKD that presents at birth
- inheritance
- what do we see
- additional organs
- teenage
- autosomal recessive 2. enlarged kidneys with small cysts and pulmonary hypoplasia 3. hepatic fibrosis, systemic HTN and cysts in other organs 4. ESRD at teenage
alport syndrome and thin basement membrane disease disorders of the
collagen IV molecule
alport syndrome
a. inheritance
b. mutation that results in
c. triad
a. X-linked b. mutation in collagen resulting in shorter molecule and abnormal GBM resulting in membrane breaks - triad: visual disturbances, hearing loss, renal failure
thin basement membrane disease
a. more common in
b. inheritance
c. mutation
d. GBM weird
e. disease is
a. more common in asian population b. Autosomal dominant c. mutation in collagen 4 with altered molecule d. thin GB< with breakage nad microhematuria e. benign disease
ESRD other complications
- diabetes
- HTN
- primary glomerular disorders
- cystic disorders
horshoe kidney
dysplastic kidney
sporadic cysts
benign renal cysts
PKD AD
acquired cystic disease- dialysis associated
CT abdomen advanced PKD
berry aneurysm
PKD AR
PKD AR gross findings
identify
identify
identify
identify
normal GBM
abnormally thin GBM which is indicative thin basement membrane lesion
abnormally split and laminated GBM see the nl and thin fenestration indicative of alport
what is this
ESRD
