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