Renal Pathology III Flashcards
(69 cards)
Can someone live with unilateral renal agenesis?
What change occurs in the sole kidney?
Rarely, some patients may develop…
It can be compatible with life if no other abnormality exists.
The sole kidney will hypertrophy.
Some patients may develop progressive glomerular sclerosis in the sole kidney.
Can someone live with bilateral renal agenesis?
No, it is not compatible with life.
Renal hypoplasia typically occurs only…
Unilaterally. It is very rare.
Ectopic kidney(s) can develop…
What location is most common?
Associated ureteral abnormalities may be associated with…
Anywhere along the path of the ureter, not at the usualy T10-L2 retroperitoneal location.
Most common location is just above the pelvic brim or within the pelvis.
Infection and obstruction.
Horseshoe kidney usually affects which pole?
Upper poles - 90%
Lower poles - 10%
ADPKD
Genetics:
Pathological features:
Clinical features:
Typical outcome:
Kidney size:
Genetics: AD
Pathological features: large multicystic kidneys, liver cysts and aneurysms.
Clinical features: Hematuria, flank pain, UTI, stones, HTN.
Typical outcome: CRF beginning at 40-60 y/o.
Kidney size: Large
Adult-onset Nephrolithiasis
Genetics:
Pathological features:
Clinical features:
Typical outcome:
Kidney size:
Genetics: AD
Pathological features: Corticomedullary cysts, shrunken kidneys.
Clinical features: Salt wasting, polyuria.
Typical outcome: Progressive RF onset in adulthood.
Kidney size: Small
Familial Juvenile Nephrolithiasis
Genetics:
Pathological features:
Clinical features:
Typical outcome:
Kidney size:
Genetics: AR
Pathological features: Corticomedullary cysts, shrunken kidneys.
Clinical features: Salt wasting, polyuria, growth retardation.
Typical outcome: Progressive RF onset in childhood.
Kidney size: Small
Childhood Polycystic Kidney Disease
Genetics:
Pathological features:
Clinical features:
Typical outcome:
Kidney size:
Genetics: AR
Pathological features: Enlarged cystic kidneys at birth.
Clinical features: Hepatic fibrosis.
Typical outcome: Variable, but death in infancy or childhood.
Kidney size: Large
What occurs to the kidney in ADPKD?
Multiple expanding cysts destroys the renal parenchyma and leads to RF.
The cysts eventually replace all of the functional cortex, but some scattered remnants of nephrons can be seen.
ADPKD is always…
BL
What population is most likely to develop ADPKD?
Manifestation of ADPKD requires…
Northern Europeans
Requires mutation of both alleles of either PKD gene
85% of ADPKD have what defective gene? On what chromosome?
What does this gene code for?
PKD1 gene, on chr. 16p13.3
Polycystin-1, an integral membrane glycoprotein. It is believed to have a role in cell-cell or cell-matrix interactions.
15% of ADPKD have what defective gene? On what chromosome?
What does this gene code for?
PKD2 gene, on chr. 4q13-p23
Polycystin-2, a Ca++ cation channel
Which genetic profile has a better prognosis in ADPKD?
Patients with PKD2 mutations
Presentation of ADPKD occurs at what age?
What are the symptoms?
Which patients may have a more aggressive course?
4-6th decades.
Renal insufficiency (HTN, azotemia), abdominal pain, possible hematuria.
AAs (sickle-cell correlation), males, patients with concomitant HTN.
Extra-renal manifestations of ADPKD include… (4)
40% have hepatic cysts
4-10% die from subarachnoid hemorrhage due to berry aneurysms
25% have MV prolapse
82% have diverticular disease of the colon
Gross appearance of kidneys in ARPKD
Slightly enlarged with many small linear/radial-arrayed cysts derived from dilated collecting ducts.
Several major clinical subtypes exist in ARPKD, but 2 are of main concern:
What are the main features of both subtypes?
Perinatal: >90% of CDs are cystic; minimal hepatic fibrosis. Babies live for only a few hours, and death is due to hypoplastic lungs, usually.
Neonatal: about 60% of CDs are cystic; mild hepatic fibrosis. Babies live for a few months, but then die fron renal failure.
2 major Medullary Cystic Diseases
Medullary sponge kidney: benign findings on imaging.
Nephronophthisi cystic disease (medullary cystic disease): cysts localized to corticomedullary junction and medulla.
Which disorders are Acquired Renal Cystic diseases?
Simple cysts
Acquired renal cystic disease
Unilateral multicystic renal dysplasia
Acquired renal cystic disease
Morphology:
Clinical complications:
Typical outcome:
Morphology: cystic degenration in end-stage disease.
Clinical complications: Hemorrhage, erthrocytosis, neoplasia.
Typical outcome: Dialysis dependent.
Unilateral multicystic renal dysplasia
Morphology:
Clinical complications:
Typical outcome:
Morphology: Multiple large cysts and cartilage.
Clinical complications: Abdominal mass
Typical outcome: Normal life expectancy
Where do simple cysts usually occur? Filled with?
On the renal cortical surface and are clear fluid-filled
