Reading...
Play button
Play button
Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
52 Cards in this Set
- Front
- Back
|
What are the features of bilateral renal agenesis?
|
- Potter facies (flat nose, low ears, recessed chin)
- Talipes equinovarus - Pulmonary hyperplasia - Incompatible with life |
|
|
What is found on ultrasound in order to detect bilateral renal agenesis?
|
Oligohydraminos
|
|
|
What happens to the remaining kidney in unilateral renal agenesis?
|
Hypertropy
|
|
|
What is a consequence of unilateral renal agenesis
|
May lead to progressive glomerular sclerosis
|
|
|
What is the defect characterized by failure of normal kidney weight, decreased number of calyces and lobes?
|
Hypoplasia (usually unilateral)
|
|
|
Where do the kidneys usually fuse in "Horseshoe Kidney"?
|
Lower poles
|
|
|
What congenital malformation of the kidney has increased predisposition to renal calculi?
|
Horseshoe kidney
|
|
|
What is the most common abnormal location for a kidney to be found?
|
Pelivc kidney
|
|
|
What can a kidney in an abnormal location predispose to?
|
pyelonephritis
|
|
|
What is the pattern of inheritance in infantile childhood polycystic kidney disease?
|
Autosomal recessive
|
|
|
What is the orientation of cysts in autosomal recessive polycystic kidney disease?
|
Radial fashion with long axis at right angle to capsule.
|
|
|
In childhood polycystic kidney disease what is the prognosis?
|
Bilateral renal enlargement with cysts in cortex/medulla and fibrosis
- progressive/fatal renal failure |
|
|
What is the inheritance of adult polycystic kidney disease?
|
Autosomal dominant
|
|
|
In APKD what is the defect?
|
Mutation of PKD1 gene on chromosome 16
- codes polycystin 1 |
|
|
What proportion of nephrons are affected in APKD?
|
10% nephrons, gradually expand to compress rest of kidney
|
|
|
What are the clinical features of adult polycystic kidney disease?
|
- asymptomatic with normal renal function until middle age
- renal insufficiency, hematuria, hypertension - abdominal masses, flank pain |
|
|
What is found inside cysts of adult polycystic kidney disease?
|
- serous, turbid or hemorrhagic fluid
|
|
|
What are the extrarenal manifestations of APKD?
|
- liver cysts
- berry aneurysms - mitral valve prolapse - colonic diverticula |
|
|
What are the features of nephritic syndromes?
|
- hematuria (RBC casts)
- hypertension - azotemia - oliguria - proteinuria <3.5g/day |
|
|
What are the features of nephrotic syndromes?
|
- severe proteinuria >3.5g/day
- hypoalbuminemia - edema - hyperlipidemia - lipiduria |
|
|
Name 5 nephritic syndromes.
|
Acute poststreptococcal GN
Goodpasture's syndrome Rapidly Progressive GN IgA Nephropathy Membranoproliferative GN |
|
|
What population gets poststreptococcal GN most often?
|
Children
|
|
|
When is poststreptococcal GN most common?
|
2-4 weeks after throat/skin infection
|
|
|
What is the most common pathogen of acute proliferative GN?
|
B hemolytic, group A streptococci
|
|
|
What is the classic presentation of postinfectious GN?
|
Child with fever, malaise, periorbital edema, HTN, smoky urine, oliguria
- 2 weeks after throat infection |
|
|
What lab values are commonly seen in poststreptococcal GN?
|
- elevated ASO titer
- low serum complement |
|
|
What is seen on light microscopy in poststreptococcal GN?
|
- hypercellular glomeruli with neutrophils
- red cell casts in renal tubules |
|
|
What disease is characterized by the following IF = granular deposits of IgG, IgM and C3
|
Poststreptococcal glomerulonephritis
|
|
|
What is seen on EM in postinfectious GN?
|
subepithelial immune deposits (humps)
|
|
|
What perecentage of postinfectious leads to recovery, for children?
|
95%
|
|
|
What percentage of adults recover from poststreptococcal GN?
|
60%
|
|
|
What disease is characterized by antibodies against BM resulting in damage of lungs/kidney?
|
Goodpasture's disease (priamrily nephritic)
|
|
|
In what group is Goodpasture's disease most common?
|
- males, 20-40 years old
|
|
|
What is a common presentation of Goodpasture's?
|
Pulmonary hemorrhage and recurrent hemoptysis (lungs involved first)
|
|
|
What is seen on LM in Goodpasture's?
|
- hypercellularity
- crescents - fibrin |
|
|
What is seen on EM in Goodpasture's?
|
NO deposits
glomerular membrane disruption |
|
|
What is the pattern of IF in Goodpasture's?
|
Smooth and linear pattern of IgG and C3
|
|
|
What is the prognosis of Goodpasture's disease?
|
Poor prognosis
- rapid progressive renal failure common |
|
|
What is the other name for rapidly progressive glomerulonephritis (RPGN)?
|
Crescentic glomerulonephritis
- follows Goodpasture's, sometimes poststreptococcal GN - associated with Wegner's and microscopic polyarteritis |
|
|
What are the components of crescents in RPGN?
|
- fibrin
- parietal epithelial cells - monocytes - macrophages |
|
|
What is seen on light microscopy in RPGN?
|
- crescent formation in BC
- hypercellular glomeruli |
|
|
What is seen in IF of RPGN?
|
- variable (linear or granular)
|
|
|
What is seen on EM of RPGN?
|
- variable, may/may not have deposits
- glomerular basement membrane disruption common |
|
|
What is another name for IgA nephropathy?
|
Berger disease
|
|
|
What is the most common cause of glomerulonephritis in the world?
|
IgA nephropathy
|
|
|
What population is commonly affected by IgA nephropathy?
|
Children and young adults
|
|
|
What are the clinical features of Berger disease?
|
Primarily nephritic
- recurrent hematuria - often follows respiratory infection |
|
|
What are two diseases associated with IgA nephropathy?
|
Celiac sprue
Henoch-Schonlein purpura |
|
|
What are features of Henoch Schonlein purpura?
|
- follows URI
- IgA nephropathy - abdominal pain - GI bleeding - arthralgia - palpable purpura on legs/buttock |
|
|
What is seen on LM in Berger disease?
|
- variable
- mesangial proliferation |
|
|
What is seen on IF in IgA nephropathy?
|
mesangial deposits of IgA and C3
|
|
|
What disease is characterized by mesangial immune complex deposits on EM?
|
Berger disease
|
