Kidney and Urinary Tract Flashcards
What vessel does the horseshoe kidney get caught on?
IMA
How does the body compensate for unilateral renal agenesis? What does this do in later life?
hypertrophy of existing kidney
increases risk of renal failure
What are the two results of bilateral renal agenesis?
oligohydramnios
lung hypoplasia
Is PKD inherited or non-inherited?
inherited
Does PKD affect the kidneys unilaterally or bilaterally?
bilaterally
What is the mode of inheritance for juvenile PKD?
autosomal recessive
What are the components of the Potter Sequence?
lung hypoplasia
face deformities
extremity deformities
How does juvenile PKD present?
HTN and decreasing renal function
What GI tract disease can juvenile PKD present with? What two conditions can this lead to?
congenital hepatic fibrosis
portal HTN and hepatic cysts
What are two ways adult PKD often present?
HTN
hematuria
What two genes are defective during PKD?
APKD1
APKD2
What are three extra-renal presentations of adult PKD?
berry aneurysm
mitral valve prolapse
hepatic cysts
What is the mode of inheritance of Medullary Cystic Kidney disease?
autosomal dominant
What is the effect on the kidneys during Medullary Cystic Kidney Disease?
shrink
What laboratory marker for acute renal failure?
azotemia
BUN and creatinine
What gets reabsorbed more during acute renal failure?
BUN
What is the minimum ratio of BUN:Creatinine during prerenal azotemia?
at least > 15:1 BUN:creatinine
What causes Prerenal Azotemia?
decreased blood flow
What causes Postrenal azotemia?
obstruction
Does kidney function remain intact during Prerenal Azotemia?
yes
Does kidney function remain intact during Postrenal Azotemia?
yes
What is the definition of Acute Tubular Necrosis?
injury and necrosis of tubular epithelial cells
Why does Acute Tubular Necrosis decrease GFR?
necrotic cells plug tubule
What is observed in the urine during acute tubular necrosis? Why?
brown casts
necrotic cells plug tubule and assume shape of nephron
What happens to BUN during Acute Tubular Necrosis? Why? What does this do to the ratio?
decreased reabsorption of BUN
dysfunctional tubular epithelial cells
What happens to sodium during Acute Tubular Necrosis? What does this do to the FENa?
decreased reabsorption of sodium
FENa = >2%
What two portions of the nephron are most severely effected by ischemia?
PCT
MEDULLARY segment of TAL
What pathology usually preceedes the ischemia of Acute Tubular Necrosis?
prerenal azotemia
What portion of the nephron is most susceptible to toxic agents?
PCT
What type of antibiotic most often causes acute tubular necrosis?
aminoglycosides
What type of metal most often causes acute tubular necrosis?
lead
What type of endogenous toxin most often causes acute tubular necrosis?
myoglobinuria
What type of crystal forms in the urine because of ethylene glycol?
oxalate
What condition can produce urate that damages the kidney?
tumor lysis syndrome
What two methods are employed at the initiation of chemotherapy to prevent Acute Tubular Necrosis?
allopurinol and copious hydration
What are the four clinical features of Acute Tubular Necrosis often seen regardless of disease etiology?
oliguria with casts
hyperkalemia
increased BUN/creatinine
acidosis
Does Acute Tubular Necrosis cause metabolic acidosis or metabolic alkalosis?
metabolic acidosis
Is Acute Tubular Necrosis reversible or irreversible?
reversible
What type of reaction is Acute Interstitial Nephritis?
drug induced hypersensitivity
What two structures does Acute Interstitial Nephritis involve?
tubules and interstitium
What three drugs are known to cause Acute Interstitial Nephritis?
NSAIDs, penicillin and diuretics
What are the three presenting symptoms of Acute Interstitial Nephritis?
oliguria
fever
rash
What is the timeline for the development of Acute Interstitial Nephritis after insult?
days to weeks
What cell type is almost pathogonomic for Acute Inerstitial Nephritis if found in the urine?
Eosinophils
What can Acute Interstitial Nephritis progress to?
Renal Papillary Necrosis
What are the four causes of Renal Papillary Necrosis?
chronic Analgesic use
Diabetes mellitus
Sickle cell
Severe acute pyelonephritis
What is diagnostic criteria for nephrotic syndrome in terms or urine loss?
> 3.5 g/day
Why does nephrotic syndrome carry a risk for infection?
hypogammaglobulinemia
Why does nephrotic syndrome carry a risk for a hypercoaguable state?
loss of antithrombin III
How does the liver react to nephrotic syndrome regarding lipids and cholesterol?
hyperlipidemia
hypercholesterolemia
What disease does Minimal Change Disease cause? In what patient group?
nephrotic syndrome
children
What disease is Minimal Change Disease associated with? What is the rationale for this?
Hodgkins Lymphoma
cytokines cause effacement of podocytes
What happens to the foot processes during minimal change disease?
effacement
Why is Minimal Change Disease different than other types of nephrotic syndrome?
MCD only causes loss of albumin
Does Minimal Change Disease possess immmunofluoresence? If so, what color?
no
How is Minimal Change Disease treated?
steroids
What disease is the most common cause of nephrotic syndrome in Hispanics?
FSGS
What disease is the most common cause of nephrotic syndrome in African Americans?
FSGS
What are the three most common causes of FSGS?
HIV
sickle cell disease
heroin
What does the Focal of FSGS mean?
only some glomeruli are affected
What does the segmental of FSGS mean?
only certain parts of glomeruli are affected
What happens to the foot processes during FSGS?
effacement
Does FSGS possess immmunofluoresence? If so, what color?
no
What is the difference between FSGS and MCD regarding treatment?
FSGS doesn’t respond to steroids
What does FSGS progress to?
chronic renal failure
What is the most common cause of nephrotic syndrome in Caucasian Adults?
Membranous Nephropathy
Regarding Membranous Nephropathy, what is observable upon microscopy?
thickening of glomerular basement membrane
Regarding Membranous Nephropathy, what drives the disease process?
immune complex deposition
Does Membranous Nephropathy respond to steroids?
no
What does Membranous Nephropathy often progress to?
chronic renal disease
What specific kidney pathology do HBV and HCV cause?
Membranous nephropathy
What specific kidney pathology do solid tumors produce?
membranous nephropathy
What specific nephritic syndrome does SLE produce? Nephrotic?
nephritic = DPGN
nephrotic = membranous nephropathy
What two drugs are known to cause Membranous Nephropathy?
NSAIDs and penicillamine
What process causes Membranoproliferative Glomerulonephritis?
immune complex deposition
Is Type I Membranoproliferative Glomerulonephritis subendothelial or intramembranous?
subendothelial
What are two strong associations of Type I Membranoproliferative Glomerulonephritis?
HBV and HCV
Is Type II Membranoproliferative Glomerulonephritis subendothelial or intramembranous?
intramembranous
What is the cause of Type II Membranoproliferative Glomerulonephritis associated with?
C3 convertase
What arteriole does DM effect more?
efferent
Why does DM cause microalbuminuria?
increased pressure of efferent arteriole pushes protein into urine
Diabetes Mellitus leads to sclerosis of what strucutre of the glomerulus? Leading to the formation of what structure?
sclerosis of the Mesangium
Kimmelstiel-Wilson nodules
What organ is most commonly effected during Systemic Amyloidosis?
kidney
Where does Amyloid deposit in the kidney during Systemic Amyloidosis? Nephritic or nephrotic?
mesangium
nephrotic syndrome
What stain is used for Systemic Amyloidosis?
congo red
Does Systemic Amyloidosis fluoresce? If so, what color?
apple green
What are the two main disease processes of nephritic syndrome?
glomerular inflammation and bleeding
How much protein per day is found in the urine during Nephritic Syndrome?
less than 3.5 grams
What two symptoms of nephritic syndrome would be evident during a physical?
periorbital edema
HTN
What is found in the urine of patients with nephritic syndrome?
RBC casts
What deposits in the kidney during Nephritic Syndrome?
immune complex
What does immune complex deposition in the kidney attract during Nephritic Syndrome? What cell is activated?
C5a
neutrophils
What group of bacteria are known to cause post-streptococcal glomerulonephritis?
Group A β-hemolytic
What specific protein of the Group A β-hemolytic strep mediates the damage?
M-protein
How long after infection does post-streptococcal glomerulonephritis often present?
2-3 weeks
What are four common presenting symptoms for a patient with post-streptococcal glomerulonephritis?
hematuria
oliguria
HTN
periorbital edema
What mediates the damage during post-streptococcal glomerulonephritis? Fluoresce?
immune complex deposition
yes
What is Rapidly Progressing Glomerulonephritis?
nephritic syndrome that progresses to renal failure in weeks to months
In what disease do crescents appear on the glomeruli?
rapidly progressing glomerulonephritis
What are the crescents of Rapidly Progressing Glomerulonephritis composed of? Where are these found?
macrophages and fibrin
Bowmans capsule
Where is IgA found during Berger disease?
mesangium
What type of infection usually preceedes the renal symptoms of Berger Disease?
mucosal
Does Berger Disease fluoresce?
yes
What type of collagen is defective during Alport Syndrome?
type IV
What are the three common presenting symptoms of Alport Syndrome?
hematuria
sensory hearing loss
visual disturbances
What is the mode of inheritance for Alport Syndrome?
X-linked
What enzyme is present in the urine of a patient with Cystitis?
leukocyte esterase
What metabolite is present in the urine of a patient with Cystitis?
nitrites
How many bacteria has to be present in to confirm a Dx of cystitis?
greater than 100,000
What bacteria is the most common cause of cystitis?
E. coli
What bacteria gives urine an alkalinity with a ammonia scent?
Proteus mirabilis
Other than E. coli, what bacteria is known to infect young, sexually active women?
S. saprophyticus
Other than E.coli/Proteus mirabilis/S. saprophyticus, what other two bacteria is known to cause cystitis?
Klebsiella pneumoniae
Enterococcus faecalis
If a sterile cystitis is detected, what two bacteria are the most likely cause? Where does this suggest the infection is located?
Neisseria gonorrhoeae and Chlamydia trachomatis
urethritis
What type of cast is preset in the urine with a patient with pyelonephritis?
WBC casts
What are the three most common bacterial cause of pyelonephritis?
E. coli
Enterococcus faecalis
Klebsiella
What are the two causes of Chronic Pyelonephritis?
vesicoureteral reflux
obstruction
What type of scarring does the vesicoureteral reflex lead to?
cortical scarring
What disease produces ‘Thyroidization’ of the kidney?
Chronic Pyelonephritis
Why does Chronic Pyelonephritis produce ‘Thyroidization’ of the kidney?
eosinophilic protein material
What heart condition can uremia lead to?
fibrinous pericarditis
What part of the coagulation cascade can uremia interfere with?
platelet function
Does chronic renal failure result in hyperkalemia or hypokalemia?
hyperkalemia
Does Chronic Renal Failure result in metabolic acidosis or alkalosis?
acidosis
Does chronic renal failure cause hypocalcemia or hypercalcemia? Why?
hypo
last step of vitamin D synthesis occurs in healthy kidney
Which Vitamin D hydroxylation rxn takes place in the kidney?
1-alpha-hydroxylation
Other than vitamin D synthesis, why can chronic renal failure produce hypocalcemia?
excessive phosphate can sequester calcium
ESRD increases the liklihood of what kidney cancer?
renal cell carcinoma
What is the most common benign tumor of the kidney?
Angiomyolipoma
What type of growth is an angiomyolipoma?
Hamartoma
Presence of what other diseases increases the liklihood of an angiomyolipoma being present?
Tuberous Sclerosis
What is the composition of the two most common kidneys stones in adults?
calcium oxalate
calcium phosphate
Does Crohns or UC produce kidney stones?
Crohns
What is the treatment of a calcium kidney stone?
Hydrochlorothiazide
Urease positive organisms produce what type of kidney stone?
Ammonium magnesium phosphate
Which two bugs are known to produce Ammonium Magnesium Phosphate stones?
Proteus vulgaris
Klebsiella
What is the only type of kidney stone that is radiolucent?
uric acid
What type of climates can predispose an individual to developing a uric acid stone?
hot and arid
WIll uric acid stone precipitate at a high or low pH?
low
What drug is given to alkalize the urine during uric acid nephrolithiasis?
potassium bicarbonate
In what age group are cystine kidney stones most often seen?
children
What is the most common sign of Renal Cell Carcinoma?
hematuria
Renal Cell Carcinoma commonly involves the loss of what tumor suppressor gene?
VHL
What chromosome is VHL located on? Arm?
3p
Which two cytokines are increased when VHL is mutated?
IGF-1 and HIF
What type of protein is VHL?
E3 ubiquitin ligase
What three proteins does HIF usually sequester?
VEGF and PDGF and EPO
What patient population do sporadic renal cell carcinomas often arise?
older, males
What is the major risk factor for the development of sporadic renal cell carcinoma? Where in the kidney?
smoking
upper pole
Are sporadic renal cell carcinomas unilateral or bilateral?
bilateral
Where in the kidney do renal cell carcinomas often arise?
upper pole
Are hereditary RCCs often unilateral or bilateral?
bilateral
In what population do hereditary RCCs often arise?
young adults
What is the mode of inheritance for VHL Disease?
autosomal dominant
What two cancers most commonly arise due to VHL Disease?
hemangioblastoma of cerebellum
renal cell carcinoma
What type of spread does renal cell carcinoma prefer?
hematogenous
What two locations does renal cell carcinoma prefer to spread via a hematogenous route?
lung and bone
What lymph node does RCC like to spread to?
retroperitoneal
What type of cell is a Wilms tumor composed of?
blastema
What are the two most common presenting symptoms in a Wilms tumor?
hematuria and HTN
What is the W of WAGR Syndrome?
Wilms Tumor
What is the A of WAGR Syndrome?
Aniridia
What is the G of WAGR Syndrome?
genital abnormalities
What is the R of WAGR Syndrome?
mental/motor Retardation
What gene is often deleted during WAGR Syndrome? What is the specific chromosomal location of this gene?
WT1
11p13
What are the three symptoms of Denys-Drash Syndrome?
Wilms tumor
progressive renal failure
male pseudo-hermaphroditism
What defective gene is Denys Drash associated with?
WT1
What gene is defective in Beckwith-Wiedemann?
WT2
What are the symptoms of Beckwith-Wiedemann Syndrome?
wilms tumor
neonatal hypoglycemia
muscular hemihypertrophy
organomegaly
What organ is particularly large during Beckwith-Wiedemann Syndrome?
tongue
What is the main risk factor for the development of a transitional cell carcinoma?
smoking
What type of cancer does naphthylamine cause?
transitional cell carcinoma
What type of cancer do azo dyes cause?
transitional cell carcinoma
What drug can cause transitional cell carcinoma?
cyclophosphamide
What is the most common presentation of a transitonal/urothelial tumor? What are the two types?
painless hematuria
flat and papillary
What type of transitional cell epithelium is associated with a p53 mutation?
flat
What are the three risk factors for the development of squamous cell carcinoma of the bladder?
chronic cystitis
Schistosoma haemotobia
chronic nephrolithiasis
From what structure does adenocarcinoma in the bladder arise? Where in the bladder?
from a urachal remnant
at the dome
What invades the parenchyma during Dysplastic Kidney?
cartilage
When does dysplatic kidney form?
in utero
Where in the kidney are the cysts during Medullary Cystic Kidney Disease located?
medullary collecting ducts
What are the main cause of Prerenal Azotemia?
decreased blood flow to kidney
What does Acute Tubular Necrosis do to urine osmolality?
> 500 mOsm
What type of kidney pathology would be created by radiocontrast dye?
acute tubular necrosis
Which nephrotic syndrome is associated with normal glomeruli on H and E stain?
Minimal change disease
Why does MCD not fluoresce?
no immune complex deposition
What process drives MCD?
cytokine release
If MCD doesnt respond to steroids, what disease will it likely progress to?
FSGS
What four causes may Membranous Nephropathy be associated with?
HBV/HCV
SLE
solid tumors
Does membranous nephropathy fluoresce?
yes
What type of immunofluorescence pattern does Membranous Nephropathy possess?
granular
Where in the nephron do immune complexes deposit during membranous nephropathy?
sub-epithelial
Does Membranoproliferative Glomerulonephritis fluoresce?
yes
What cell proliferates during Membranoproliferative Glomerulonephritis? What sort of appearance does this take on?
Mesangial
‘tram-track’
Which type of Membranoproliferative Glomerulonephritis has more Tram Tracks?
Type I
What antibody is present during Type II Membranoproliferative Glomerulonephritis?
C3 Nephritic Factor
What is the function of C3 Nephritic Factor?
stabilizes C3 convertase
Does Membranoproliferative Glomerulonephritis produce nephritic syndrome, nephrotic syndrome or both?
both
What part of the glomerulus undergoes NES first?
vascular basement membrane
What type of renal disease is PSGN?
nephritic
What is the treatment for PSGN?
supportive
How often do children with PSGN progress to chronic renal failure?
1%
How often do adults with PSGN progress to chronic renal failure? What is this disease called?
25%
rapidly progressive glomerulonephritis
What are the three types of Immunofluorescence in the glomerulus?
Linear
Granular
Negative IF (pauci-immune)
What disease is linear glomerular fluorescence indicative of? What specific renal disease?
Goodpasture Syndrome
RPGN
What is an auto-antibody formed against during Goodpasture Sydrome?
type four collagen
In what two organs does Goodpasture Syndrome manifest itself?
against collagen in kidneys and lungs
How would a patient with Goodpastures syndrome present regarding their lungs?
Hemoptysis
How would a patient with Goodpastures syndrome present regarding their kidneys?
Hematuria
What patient population is clasically effected by Goodpastures Syndrome?
young, adult males
What causes granular immunofluorescence?
immune complex deposition
What is the most common kidney disease caused by SLE?
diffuse proliferative glomerulonephritis
SLE with nephrotic syndrome is due to what disease?
membranous nephropathy
What are the two main differences in presentation between Wegeners Granulomatosis and Goodpasture Syndrome?
Wegeners involves nasopharynx
c-ANCA
When does IgA Nephropathy most commonly present? How?
childhood
hematuria with casts
Would IgA Nephropathy fluoresce?
yes
What is the most common mode of inheritance for Alport Syndrome?
X-linked
What type of scarring pattern is characteristic of VUR?
scarring at upper and lower poles
What shape of caliculi is associated with Proteus or Klebsiella?
staghorn
Scarring at the upper and lower pole during Chronic Pyelonephritis is indicative of what disease happening?
vesicouretal reflex
What can produce a staghorn caliculi in a child?
cystinuria
How are cystine kidney stones treated?
hydration and alkalinization of urine
What is often the first metabolite to increase during chronic renal failure?
uremia
What specific cells of the kidney produce EPO?
renal peritubular interstitial cells
What are our common cytokines does RCC often produce that can cause a paraneoplastic syndrome?
EPO/renin/PTHrP/ACTH
A transitional cell carcinoma develops by which two pathways?
flat and papillary
Which type of renal cell carcinoma develops as a high grade tumor?
flat
Other than a urachal remnant, what are the other two methods by which an adenocarcinoma can develop in the bladder?
cystitis glandularis
bladder exstrophy
What is cystitis glandularis?
columnar metaplasia of bladder in response to chronic inflammation
What develops during Dysplastic Kidney?
cysts
Is dysplastic kidney usually unilateral or bilateral?
unilateral
Where in the kidney does PKD produce cysts?
cortex and medulla
Does Medullary Cystic Kidney Disease produce an enlarged or shrunken kidney?
shrunken
What are the two types of Acute Tubular Necrosis?
ischemic and nephrotoxic
What molecule may be present in the cells of the proximal tubule cells during minimal change disease?
lipid
Does PSGN fluoresce?
yes
Does ESRD produce shrunken or enlarged kidneys?
shrunken
What develops in the kidney during dialysis? What does this increase the chance of?
cysts
RCC
What type of kidney stone does Crohns often present with? Why?
oxalate
Crohns increases the reabsorption of oxalate crystals
According to Sattar, who is most likely to present with a bladder squamous cell carcinoma caused by Schistosoma haemotobium?
Egyptian male
What causes the Potter sequence to appear?
oligohydramnios
Long-term use of which two drugs can cause Renal Papillary Necrosis?
aspirin and phenacetin
What type of kidney disease is Focal Segmental Glomerularsclerosis?
nephrotic
What is the appearance of Membranous Nephopathy on EM?
spike and dome
Does strep cause a nephritic or nephrotic syndrome?
nephritic
What is the age and sex of the classic patient that presents with Goodpasture Syndrome?
young adult males
What chromosome is WT1 located on?
11p13
Phenacetin is associated with what type of cancer?
transitional cell carcinoma
What chromosome is WT2 located?
11p15.5
Where in the bladder does cystitis glandularis often arise?
trigone
Which three disease would have negative fluorescence yet still cause Rapidly Progressing Glomerulonephritis?
Wegener
Microscopic Polyangiitis
Churg Strauss
Is dysplastic kidney inherited or non-inherited?
non-inherited
Are there low or high levels of C3 during Type Two MPGN?
low
Is nephritic or nephrotic hypercellular?
nephritic
Where are the deposits during PSGN?
subepithelial
What disease can PSGN progress to in adults?
RPGN
What two things happen to the GBM during Alport Disease?
thin and split
What cells of the kidney produce Epo?
renal peritubular interstitial cells
What three hormones can RCC produce?
Epo
renin
PTHrp
ACTH
What is the most common variant of RCC?
clear cell
Where chromosome is WT2 located on?
11p15.5
Is BUN reabsorbed?
yes
Is creatinine reabsorbed?
no
If minimal chage disease doesnt respond to steroids, what disease may it progress to?
FSGS
How long after mucosal infection does Berger Disease present?
few days
What is osteomalacia?
cant mineralize osteoid made by osteoblasts
What is the color of RCC on gross appearance?
yellow
