Renal/Male Flashcards
normal histology of glands of prostate?
inner layer of luminal cells and outer layer of basal cells
19 year old male presents with dysuria, fever and chills. On DRE, prostate feels tender and boggy. Diagnosis? What will you see in prostatic secretions? Pathogenesis? What if the patient was 55?
WBCs and a +bacterial culture
in young adults: Chlamydia or Neisseria
in older men: E. coli or Pseudomonas
Male presents with dysuria and pelvic/low back pain. On exam of prostatic secretions you notice WBCs but cultures are negative. Diagnosis?
chronic prostatitis
BPH is very common condition. Do people usually show symptoms? What is the increased risk of carcinoma?
No, only 10% are symptomatic
0% risk of cancer, exception for hyperplasia
What enzyme is targeted in treatment of BPH? Why?
5-alpha reductase
converts testosterone to DHT in stromal cells which acts on androgen receptors of both stromal and epithelial cells -> hyperplasia
60 year old man presents with difficulty urinating, and frequently has to wake up to use the bathroom. Diagnosis? Two important complications?
BPH
increased risk of bladder infection
hydronephrosis
What are the effects of treating BPH with testosterone? Explain
testosterone itself does not aggravate BPH, it is thought that estrogen sensitizes the androgen receptors so they respond even with the natural decline in testosterone with age
On examination of the prostate you see inspissated secretions in the lumen of the glands. What is the name of this histology and what condition do you see it in?
Corpora amylacea of BPH
Are you more likely to see symptoms with BPH or adenocarcinoma of the prostate? why?
BPH, occurs periurethral leading to compression of the urethra and symptoms. Adenocarcinoma is usually found on the outside of the gland, and must become very large to cause symptoms
what is the advantage of treating BPH with transurethral resection as opposed to drugs?
Can sometimes find incidental signs of cancer on tissue exam after TURP
63 year old man presents complaining of difficulty maintaing a stream of urine and frequency. Microscopic hematuria is present and PSA levels are slightly elevated. Diagnosis? The resulting hypertrophy of the bladder wall can lead to an increased risk of what?
BPH
bladder diverticula
Prostate cancer is more common in what race? Is least common in what race? what kind of diet increases risk?
Blacks at highest risk
Asians at lowest risk
high fat diet
66 year old male has a family history with HPC1 and RNASEL genes. What is he at risk for?
prostatic adenocarcinoma
67 year old male presents with back pain, fatigue, weight loss, and dysuria. DRE reveals an irregular, nodular, firm prostate. Diagnosis? What is unique about grading?
Prostatic adenocarcinoma
Gleason grading system based on architecture NOT atypia like most cancers
What is important to do when taking a biopsy of the prostate?
Take multiple biopsies from many different locations
On a routine screening PSA test, you note a decreased % of free-PSA. Does this have a good or bad prognosis? Why?
bad, suggests cancer which makes bound-PSA
On biopsy of the prostate you notice glands are lined by a single layer of cuboidal epithelium that are missing outer basal layer of epithelial cells. Diagnosis? what is another important histological feature?
prostatic adenocarcinoma
back to black glands
You measure a PSAd to be >0.155. What is PSAd? What does this finding tell you?
PSAd = PSA density: ratio of the serum PSA to the volume of the prostate
PSAd > 0.125 is assoc with 80% chance of finding cancer
You notice one of your elderly male patient’s PSA levels have increased by .55ng/mL since last year. Should you be concerned?
No, but an increase in .75ng/mL/yr indicates prostate cancer
what is the difference between a Gleason score of 4/10 and 8/10? Which is better prognosis? What are you looking at to give a score?
the lower the score, the better the prognosis
you are looking at ARCHITECTURE not atypia!!
On biopsy of prostate you see small, invasive glands in a back to back arrangement. Diagnosis? what is the expected description of these cells nucleoli?
prostatic adenocarcinoma
darkly staining, prominent nucleoli
Reinke crystals are formed in what kind of cells?
Leydig cells in the testicular interstitium
1 year old infant presents with a nonpalpable testicle. Diagnosis? Which side is more common? Risk factor? How common?
Cryptorchidism
usually right sided
prematurity
1% of male infants
A male with an undescended testicle has surgery at the age of 6 to fix this condition. What is he at an increased risk for?
After the age of 5: sterility and seminoma
A male with an undescended testicle has surgery at the age of 18 months to fix this condition. What is he at an increased risk for?
Nothing, before age 2, no complications
A male with an undescended testicle has surgery at the age of 3 to fix this condition. What is he at an increased risk for?
After the age of 2: Infertility
an 18 month old male is noted to have obesity, short fingers, and mental retardation. Name of this condition? what is he at increased risk for?
Prader-willi syndrome
cryptorchidism
On examination of a testicle, you note a thickened basement membrane of the tubules, hyalinization, leydig cell hyperplasia, and tubular atrophy. What is the most likely cause of this histology? What age would the patient be?
Cryptorchidism
adolescent, hyalinization is not seen until this age
orchiopexy is what?
a surgery to correct cryptorchidism
What stimulates leydig cells to produce what?
LH stimulates them to produce testosterone
28 year old male presents with inflammation of his testes. What are the 2 most likely causative agents? At what age do these agents change and what do they change to?
Gonorrhea and Chlamydia
35
>35: E. coli and pseudomonas
19 year old male presents with inflammation of his testes. What is the name of this condition? would he complain about loss of libido? why or why not
Orchitis
no, leydig cells are not affected, testosterone levels would be normal, not affecting libido
Patient presents with testicles that have a “bag of blood” appearance. Diagnosis? Pathogenesis? Usual cause?
testicular torsion
spermatic cord twists, thin-walled veins are obstructed -> hemorrhagic infarction (blood can get in, but can’t get out)
Due to congenital failure of testes to attach to inner lining of the tunica vaginalis
18 year old male presents with sudden testicular pain and absent cremasteric reflex. Diagnosis?
testicular torsion
scrotal “bag of worms” - diagnosis? What is it? which side is more common? What is an important association? why?
Varicocele: dilated spermatic vein due to impaired drainage
left side more common, drains into left renal vein (right testicular vein drains directly into IVC)
assoc with renal cell carcinoma - likes to invade renal vein, blocks drainage of left testicular vein
incomplete closure of the processus vaginalis in infants can lead to what pathology?
hydrocele
blockage of lymph drainage of the testes in adults can lead to what? Can it be transilluminated?
hydrocele
yes, cysts can be, tumors cannot
testicular tumors are more common in what race?
whites
when should you biopsy a suspicious testicular mass? why?
Never! its usually cancer and you dont want to seed it
most common testicular tumor in children? 15-30? 30-50? >60?
children: teratoma and yolk sac
15-30: mixed germ cell tumor
30-50: seminoma
>60: lymphoma
2 types of testicular tumors? which is more common?
germ cell (95%) sex cord-stroma
Male presents with gynecomastia, unilateral mass in scrotum that cannot be transilluminated, and heaviness in the scrotum. Should you be concerned?
Yes, most solid masses in testicles are malignant
What are the 2 subdivisions of germ cell tumors of the testes?
seminoma
non-seminoma
Give the 5 divisions of non-seminoma germ cell tumors of the testes. Which is most common?
embryonal carcinoma yolk sac (endodermal sinus) tumor choriocarcinoma teratoma mixed germ cell tumor (most common)
This testicular tumor has an excellent prognosis, metastasizes late, and is very responsive to radiotherapy?
seminoma
This testicular tumor is comprised of large cells with clear cytoplasm and central nuclei. Form a homogenous mass.
seminoma
This testicular tumor has immature, primitive cells that can produce glands. It is aggressive with early hematogenous spread - diagnosis? how to treat? complication of treatment?
embryonal carcinoma
chemotherapy - can result in differentiation to another type of mature germ cell tumor (teratoma)
Isochromosome 12p
OCT3/4
NANOG
c-KIT amplification
Seminoma
how do seminomas spread? non-seminomas?
how does treatment vary for these?
seminomas: lymph - radiotherapy
non-seminomas: blood and lymph - chemotherapy
which testicular tumor is associated with gonadal dysgenesis? what disease could cause this?
seminoma
Kleinfelter’s
testicular tumor with sheets of cells resembling fried eggs with lymphocytic infiltrate and fibrovascular septae - diagnosis? name 2 other conditions with identical pathology
seminoma
dysgerminoma in ovary
medullary breast carcinoma
This testicular tumor is associated with high levels of AFP. The cells have cytoplasmic pink inclusions with eosinophilc hyaline globules - diagnosis? what is characteristic on histo?
Yolk sac (endodermal sinus) tumor Schiller-Duval bodies
Schiller-Duval bodies are common in what testicular cancer? What do they look like?
yolk sac (endodermal sinus) tumor look like glomeruli from the kidney
19 year old male presents with signs of hyperthyroidism and gynecomastia. You notice a mass in his testicles that has small, painless nodules. Chest x-ray shows cannon-ball nodules in the lungs. Diagnosis? what is elevated in serum? explain his symptoms. prognosis?
Choriocarcinoma
elevated beta-hCG
alpha subunit of hCG acts similar to FSH, LH, and TSH
prognosis is poor
histo of a testicular tumor shows embryonic epithelial tissue and immature neuronal tissue. Diagnosis? how does the prognosis differ from that of a female?
teratoma
malignant in males after age 12, benign in females
if your testicular mass is hemorrhagic with signs of necrosis, what differential can you rule out?
seminoma
you find Reinke crystals on histo of a testes specimen. Diagnosis? should you be concerned?
Leydig cell tumor (sex cord-stromal tumor)
no, usually benign
pre-adolescent male presents with precocious puberty. you notice a non-transilluminated mass in his testicle. diagnosis? prognosis? how would this present differently in an adult?
leydig cell tumor (sex cord-stromal tumor)
good, usually benign
it produces androgens so it would lead to gynecomastia
65 year old male presents with a bilateral mass in his testicles that cannot be transilluminated. Diagnosis (specifically)?
diffuse large B-cell lymphoma
congenital malformation of the penis involving an abnormal positioning of the genital tubercle. Opening of the urethra on the dorsal surface. Diagnosis? how common?
epispadias, much less common than hypospadias
you notice bladder exstrophy of a newborn, what else would you expect to find?
epispadias
congenital malformation of the penis involving a failure of the urethral folds to close. Opening of the urethra on the ventral surface of the penis. Diagnosis? how common?
hypospadias, more common than epispadias
at what age do teratomas become malignant in males?
12, benign beforehand
male presents with balanitis. what is this? what is a predisposing condition?
non-specific infection of the glans (swollen and red)
uncircumcised males more at risk
phimosis - what is it? who is at risk? what causes it? increased risk of what (2)?
prepuce (foreskin) is too small to allow retraction of foreskin over penis. Uncircumcised males at risk, due to congenital anomaly or infection leading to scarring
inc risk of infection or cancer
paraphimosis - what is it? what does it cause? increased risk of what (2)?
happens when a phimotic prepuce is forcibly retracted over the glans leading to constriction, swelling, and pain
inc risk of UTI or necrosis of penis if severe enough and untreated
male presents with shiny, red plaque over glans of his penis - diagnosis? what is it? prognosis? what causes it?
Erythroplasia of Queyrat
in situ carcinoma of glans
can progress to squamous cell carcinoma in immunocompromised
high risk HPV 16, 18, 31, 33
male presents with solitary gray lesion on the shaft of his penis - diagnosis? what is it? prognosis? what causes it?
Bowen disease
in situ carcinoma
can progress to squamous cell carcinoma
high risk HPV 16, 18, 31, 33
male presents with multiple reddish plaques over the shaft of his penis - diagnosis? what is it? prognosis? what causes it?
Bowenoid disease
in situ carcinoma
does NOT progress to squamous cell carcinoma
high risk HPV 16, 18, 31, 33
raisin nuclei and koilocytic change are seen along with raised, nodules on surface of penis - diagnosis? etiology?
condyloma acuminatum
low risk HPV 6, 11
name 3 risk factors for penile squamous cell carcinoma
being uncircumcised
smoking
high risk HPV 16,18
you notice lymphatic spread to the paraaortic lymph nodes - diagnosis?
seminoma testis
you notice lymphatic spread to the inguinal lymph nodes - diagnosis?
penile squamous cell carcinoma
chimney sweepers and people who work around coal tar are more at risk for what type of cancer?
scrotal cancer
evagination of bladder wall - name of condition? two causes?
diverticula
congenital or acquired (BPH)
urine stasis increases the risk of what 2 benign conditions? give two causes
infection and bladder stones (calculi)
diverticula and BPH
what is the only painful testicular cancer?
embryonal carcinoma (non-seminoma)
you notice urine dribbling from the umbilicus of a new born - name of condition? what is he at increased risk for?
urachus
adenocarcinoma of bladder
what is cystitis? is it more common in males or females? why?
inflammation of the bladder
females, shorter urethras are more prone to infection
how can you tell the difference between cystitis and pyelonephritis on presentation?
cystitis does not have systemic symptoms like night sweats or fever
cystoscopic examination reveals edema hemorrhage, and ulceration - what area are you looking at? what should your next step be?
bladder
chronic cystitis looks like cancer on scope, so need to biopsy for diagnosis
on bladder tissue biopsy you seen urothelial papilloma - why do you need to be careful about this diagnosis?
urothelial papilloma are benign, but VERY rare, there is usually a low grade carcinoma there
on bladder biopsy you see intestinal metaplasia - diagnosis? important association?
adenocarcinoma of the bladder
urachus
person has been swimming in the nile and presents with urinary frequency and suprapubic pain - diagnosis? etiology?
squamous cell carcinoma of the bladder
schistosomiasis: ova irritate bladder wall, leading to metaplasia, dysplasia, and carcinoma
most common type of bladder cancer?
urothelial carcinoma - 90%
most important risk factor for bladder cancer?
smoking!!!!
someone who smokes and has been taking aspiring multiple times a day for many years has an increased risk of what?
urothelial carcinoma
patient presents with dysuria, urgency, flank pain, and painless hematuria - diagnosis?
painless hematuria is commonly associated with urothelial carcinoma
azo dyes increase your chance of what?
urothelial carcinoma
what are the two growth types of urothelial carcinoma? which has the worst prognosis and where does it metastasize to?
papillary/exophytic
flat/invasive - poorly differentiated, poor prognosis, metastasizes to regional nodes, liver, lung, bone
where can you get urothelial carcinoma?
urothelial epithelium extends from urethra to calyces of kidney
name 3 associations with urothelial carcinoma
bilateral hydroureter
hydronephrosis
stone formation
name three substances the kidney produces
erythropoietin
renin (JG cells)
prostaglandins
normal GFR?
90mL/min
endogenous substance used to predict renal clearance? why is it not perfect?
creatinine
-freely filtered, not reabsorbed, but is secreted by tubules so can increase GFR by up to 15%
why is serum creatinine a poor marker of renal function?
its levels are influenced by muscle mass
what is azotemia? what increases it?
increased BUN
urea and creatinine
hallmark of nephritic syndrome? 2nd finding?
hematuria
HTN
hallmark of nephrotic syndrome? 2nd finding?
proteinuria >3.5g
hypoalbuminemia/edema
oliguria and azotemia indicate what pathology of the kidney?
acute renal failure
polyuria, nocturia, and electrolyte disorders indicate what pathology of the kidney?
tubular defects
what is the hallmark of a pre-renal disease leading to renal failure?
inadequate blood supply
pruritis and uremia indicate what?
chronic renal failure
heavy proteinuria (glomerular or tubulointerstitial)?
glomerular
WBCs (glomerular or tubulointerstitial)?
tubulointerstitial
oval fat bodies (glomerular or tubulointerstitial)?
glomerular
RBC casts (glomerular or tubulointerstitial)?
glomerular
poluria and electrolyte imbalance (glomerular or tubulointerstitial)?
tubulointerstitial
RBC casts (nephritic or nephrotic)?
nephritic
heavy proteinuria (nephritic or nephrotic)?
nephrotic
white blood cells (nephritic or nephrotic)?
nephritic
fatty casts (nephritic or nephrotic)?
nephrotic
immune complexes activate which complement pathway? which molecule stabilizes the converstase?
alternative
properdin stabilizes C3bBb
what is the difference between diffuse and focal in relation to the kidney?
diffuse involves all glomeruli, focal is just some of them
what is the difference between global and segmental in relation to the kidney?
global means the entire glomerulus, segmental is just part of a glomerulus
you have a young child with nephrotic syndrome, what should you not do during diagnosis?
don’t biopsy!
why is hyperlipidemia associated with nephrotic syndrome?
albumin is being lost in the urine, so the liver tries to make up for this loss by increasing synthesis of proteins, including lipoproteins
name the only two nephrotic diseases with immunoglobulin deposition
membranous nephropathy membranoproliferative glomerulonephritis (can also be nephritic)
Remember! if its nephrotic and has membrane in the name, it has immune complex deposition!
name the 4 nephrotic disease that don’t involve immune complex deposition
minimal change
Focal segmental glomerulosclerosis (FSGS)
diabetic nephropathy
amyloidosis
associated with hypercoagulable state (nephritic or nephrotic)? why?
nephrotic
loss of antithrombin III (breaks up thrombin and other clotting factors)
most common nephrotic syndrome in kids
minimal change disease
tram track appearance on H&E
membranoproliferative glomerulonephritis
most common cause of nephrotic syndrome in Caucasian adults
membranous nephropathy
most common cause of nephrotic syndrome in Hispanics and African Americans
focal segmental glomerulosclerosis (FSGS)
this nephrotic syndrome can be associated with lymphoma or renal cell carcinoma
Minimal change disease
A mother brings her 7 year old child in to see you because his shoes have not been fitting properly. On examination, you find low serum albumin, proteinuria, and no casts. Diagnosis? What is the cause of the damage? How to treat?
Minimal change disease
T cell cytokine mediated damage
treat with steroids (they are immune suppressant and counteract the cytokines)
normal H&E, effacement of foot processes on EM, negative IF - 2 differentials, how to tell apart?
minimal change disease
FSGS
minimal change responds to steroids, FSGS does not
“spike and dome” appearance on EM
membranous nephropathy
pathogenesis of this kidney disease involves circulating glomerular permeability factors
minimal change disease
prognosis of minimal change disease?
good, no tendency to progress to chronic renal disease or end stage renal disease, but relapses are common
HIV, heroin use, and sickle cell are associated with what kidney pathology?
focal segmental glomerulosclerosis (FSGS)
what is anasarca?
generalized edema
kidney disease caused by a mutation of the protein podocin
focal segmental glomerulosclerosis (FSGS)
African American with HIV presents with hypertension, massive proteinuria and microscopic hematuria. LM of a kidney biopsy looks normal. Diagnosis? Prognosis? why is biopsy normal?
focal segmental glomerulosclerosis (FSGS)
progresses to end stage renal disease in 5-20 years
biopsy can be normal because the disease is focal (not all glomeruli are involved)
name 3 indicators of a poor prognosis for membranous nephropathy
male
>50 years old
>10 gm proteinuria
this kidney disease is associated with hepatitis B/C, SLE, and chronic NSAID use
membranous nephropathy
kidney disease with subepithelial deposits on EM, thick basement membrane on H&E, and granular deposition on IF - diagnosis? prognosis? what are the deposits on IF composed of?
membranous nephropathy
poor response to steroids, progresses to chronic renal failure
IgG C3
kidney disease related to mercury and gold
membranous nephropathy
glomerular disease with thickening of the basement membrane, mesangial cell proliferation, and infiltration of inflammatory cells
membranoproliferative glomerulonephritis
kidney disease associated with hep B/C, subendothelial immune complexes of IgG, and tram track appearance on H&E - diagnosis? what are the deposits made of?
membranoproliferative glomerulonephritis
type I
IgG
kidney disease with deposition in the glomerular basement membrane, low levels of C3 and associated with C3 nephritic factor -diagnosis? what are the deposits made of?
membranoproliferative glomerulonephritis
type II
C3
kidney disease with tram tracks on H&E, and subendothelial deposits, mesangial deposits, and subepithelial deposits - diagnosis? what are the deposits made of?
membranoproliferative glomerulonephritis
type III
IgG and C3
Which type of membranoproliferative glomerulonephritis involves complement activation via the classical pathway? alternate? what is normal age of onset? prognosis?
classical: type I
alternate: type II
age: <30
prognosis: most progress to ESRD within 10-15 years
what is the leading cause of end stage renal disease in USA?
diabetic nephropathy
a 55 year old man just received a kidney transplant from a donor who was just discovered to have diabetes mellitus and had diabetic changes in his kidney - should the recipient be concerned?
no, diabetic changes can reverse themselves when placed in a healthy patient
in diabetic nephropathy, which arteriole is more affected by the hyalinization? why does this matter?
efferent -> inc GFR -> hyperfiltration and microalbuminia
name the 2 early lesions in diabetic nephropathy
expansion of the mesangial matrix and thickening of the glomerular basement membrane
Kimmelstiel-Wilson nodules are unique to which kidney pathology? what are they made of?
diabetic nephropathy
lipids and fibrin NOT collagen
you notice deposits in the mesangium of the glomerulus that stains with congo red - diagnosis? how common is it? does it involve immune or inflammatory response?
amyloidosis
kidney is most common of systemic amyloidosis
neither immune or inflammatory reactions are provoked
which amyloid is associated with primary amyloidosis? prognosis?
AL
aggressive, poor prognosis
which amyloid is associated with secondary amyloidosis? prognosis?
AA
better prognosis than primary
where does AA amyloid come from?
its an apolipoprotein produced by the liver as an acute phase reactant in response to long standing infection or inflammation
LM shows nodular, amorphous hyaline material in mesangium and capillary loops that narrows or closes capillary lumens - EM shows subendothelial and mesangial fibers - diagnosis?
amyloidosis in kidney
patient presents with purpura and easy bruising. Also proteinuria, edema, arrhythmias, malabsorption symptoms and orthostatic hypotension - diagnosis? prognosis?
amyloidosis in kidney
very poor prognosis, most die from end-organ failure from amyloid deposition
inflamed glomeruli, hematuria and salt retention are characteristic of what kind of kidney disorder?
nephritic
list the nephritic disorders with normal complement levels
IgA nephropathy/Henoch-Schonlein purpura
Alport’s syndrome (hereditary nephritis)
SLE (class I, II, V)
benign hematuria
list the nephritic disorders with low complement levels
post-streptococcal glomerulonephritis membranoproliferative glomerulonephritis SLE (class III, IV) bacterial endocarditis cryoglobulinemia
list the nephritic disorder with variable complement levels
rapidly progressive glomerulonephritis
most common type of glomerulonephritis? what race is it rare in?
IgA nephropathy/Berger disease
blacks
Pt presents with episodic hematuria with RBC casts, proteinuria of <3.5g/day, normal C3/C4, and has a history of viral respiratory illness. - Diagnosis? Prognosis? Common age group?
IgA nephropathy/Berger disease
usually prolonged benign course, 20% progress to ESRD (end stage renal disease)
15-30
18 year old male presents with abdominal pain, pain in the joints, vasculitis and palpable purpura. Diagnosis? what changes will you see in his kidney?
Henoch-Schonlein Purpura (assoc with IgA nephropathy)
IgA deposition in the mesangium
on biopsy of a kidney you see on LM segmental areas of increased mesangium and hypercellularity. On IF you see large patches of deposition. On EM you see mesangial and subendothelial deposits. Diagnosis?
IgA nephropathy/Berger disease
characteristic subepithelial “humps” on EM
post-streptococcal glomerulonephritis
8 year old child who had a skin rash a month ago presents with sudden onset proteinuria >2g/day with low complement levels and coca cola colored urine. LM shows proliferation of mesangium, endothelium, and epithelium. - Diagnosis? prognosis? What do characteristically expect to find in their serum?
post-streptococcal glomerulonephritis
good prognosis, only 1% of kids progress to renal failure, 25% of adults develop rapidly progressive glomerulonephritis. 10-40 years later can develop renal insufficiency
serum: anti-ASO Abs or anti-DNAase B
IF shows granular deposits of IgG and C3 in mesangium and capillary walls. EM shows large deposits in subepithelium. Diagnosis?
post-streptococcal glomerulonephritis
crescents in Bowman’s space - diagnosis? what are the crescents made of? what is the prognosis of the presence of crescents?
Rapidly Progressive Glomerulonephritis
fibrin and macrophages - not collagen!!
crescents are a very aggressive finding
A pt. with Rapidly Progressive Glomerulonephritis shows a linear pattern on IF. Diagnosis? what is an important symptom for diagnosis? why is it a linear pattern?
Goodpasture’s syndrome
hemoptysis (LUNG involvement!)
linear due to anti-GBM Abs (anti-glomerular basement membrane)
A pt. with Rapidly Progressive Glomerulonephritis shows a granular pattern on IF. Give 3 possible diagnoses. Which is most common? why is it granular?
post-streptococcal glomerulonephritis (most common)
SLE
IgA nephropathy
granular due to immune complex deposition
A pt. with Rapidly Progressive Glomerulonephritis shows a non staining pattern on IF. Give 2 possible diagnoses. How do you differentiate? why don’t they stain?
Wegener’s (look for sinusitis!) c-ANCA
Churg-Strauss - p-ANCA
no staining due to no immune complexes, they are vasculitis diseases
What is the outcome of Rapidly Progressive Glomerulonephritis? which types are most treatable? why?
75% die or are placed on dialysis within 2 years of diagnosis
better prognosis with those involving a treatable underlying disorder like SLE or one that resolves spontaneously like post-strep
List of lupus symptoms - just go read them
need 4 or more for diagnosis
joints are most commonly affected: 90% cases, followed by rashes in 70% of cases
malar rash photosensitivity oral ulcers arthritis pericarditis renal disease neurological disorder (+) IF antinuclear antibody test
anti-GBM Abs are seen in what disorder? What specifically is the Ab targeting?
Goodpasture’s syndrome
type IV collagen of GBM
Pauci immune describes what kind of staining pattern?
minimal to no staining on IF
you see a granular pattern on IF that tests positive for all immune complexes - diagnosis?
SLE
to make the differential between class III and class IV lupus nephritis, what must you see on LM?
class III has < 50% of glomeruli involved, and class IV has >50% of glomeruli involved
list the 6 classes of Lupus nephritis
which is most common? most severe?
class I: minimal mesangial class II: mesangial proliferative class III: focal segmental proliferative class IV: diffuse proliferative class V: membranous class VI: advanced sclerosing
class IV is most common and most severe
*see page 38 in class notes
List the 7 tubular and interstitial diseases
acute tubular necrosis (ATN) acute interstitial nephritis (tubulointerstitial) acute pyelonephritis chronic pyelonephritis papillary necrosis obstructive nephropathy renal stones
pt presents with dislocated lens, sensorineural hearing loss and hematuria - diagnosis? what is unique about this disease?
alport’s syndrome
only congenital/hereditary nephritic syndrome
80% X-linked
“basket weave” appearance on EM
Alport’s syndrome
a Mormon shows a mutation in the COL4A4 and COL4A5 genes - diagnosis? what do you see on EM?
Alport’s syndrome
thinning and splitting of the GBM
patient presents with elevated BUN, metabolic acidosis, anemia, and hypotension. Brown, granular casts are seen in urine - Diagnosis? Give 2 etiologies and examples
Acute tubular necrosis
ischemic (decreased blood supply)
nephrotoxic (due to aminoglycosides or heavy metals like lead)
aminoglycosides are antibiotics
describe the 3 phases of Acute tubular necrosis (ATN)
initiation: 36hrs - very low GFR, rapid inc in serum BUN
maintenance: days-3weeks - oliguria, fluid overload, metabolic acidosis
recovery phase: increasing GFR, function is restored
pt presents with oliguria, muddy brown casts, epithelial cell casts, no pyuria and mild proteinuria - diagnosis?
Acute tubular necrosis
pt presents with hyponatremia, metabolic acidosis, but no proteinuria or hematuria. Pt also has fever and rash. Eosinophils are seen in urine - diagnosis? cause? treatment? prognosis?
Acute interstitial nephritis
usually due to a drug-induced hypersensitivity like NSAIDs or penicillins
stop drug use
resolves with cessation of drug, but can progress to renal papillary necrosis
Pt with sickle cell who has a long term history of aspirin use presents with gross hematuria and flank pain. Gross appearance of kidney shows necrosis of apical 2/3rds of the pyramids - diagnosis? give the 4 causes
Renal papillary necrosis
chronic analgesic abuse
diabetes mellitus
sickle cell trait
severe acute pyelonephritis
Pt presents with fever, flank pain, WBC casts and leukocytosis - diagnosis? most common cause? more common in who? why?
acute pyelonephritis
ascending infection with E. coli
women, short urethra
biopsy of kidney shows interstitial fibrosis and atrophy of tubules. you see progressive cortical scarring and blunted calyces - diagnosis? most common cause? second cause?
chronic pyelonephritis reflux nephropathy (vesicoureteral reflux) most common obstruction second cause: ex. kidney stones, BPH
on microscopic exam of kidney biopsy you see eosinophilic proteinaceous material resembling colloid inside atrophic tubules - waxy casts are also seen in urine - diagnosis?
chronic pyelonephritis
Pt presents with colicky pain, hematuria and unilateral flank tenderness. Kidney shows dilated renal pelvis and calyces. Diagnosis? prognosis? time frame for related damage?
nephrolithiasis - kidney stone
stone usually passed in hours, surgery may be required after that - resolves fully upon removal of stone
if left 2-3 weeks -> irreversible damage and renal failure
composition of most common renal stone? list 3 other compositions
75% of stones are calcium oxalate or calcium phosphate
10-15% are struvite (Mg/ammonium phosphate)
5% uric acid
2% cystine
3 methods to prevent kidney stones
increase fluid intake 2L/day
low sodium diet (decreases urinary calcium excretion)
alkalinization of urine to help inc solubility of uric acid
which type of kidney stone is radiolucent?
uric acid
I can’t see U on x-ray
these casts can be seen in normal people after exercise or fever due to dehydration
hyaline casts
these muddy brown, cigar shaped granular casts are seen in what diseases?
acute tubular necrosis and chronic renal disease (most cases)
these casts are seen with longstanding renal disease
waxy casts
these casts are made of solidified tamm horsfall proteins
hyaline casts
these casts are made of albumin or Ig light chains
granular casts
these aggregates found in urine are in the shape of maltese crosses
oval fat bodies
NOT a cast! - below the level of the kidney
the presence of RBCs and WBCs in urine tell you about about the problem?
that it is lower than the kidney… if it were a kidney problem, they would be in casts
on kidney biopsy you see medial and intimal thickening, hyaline arteriolosclerosis and notice that the surface of the kidney is finely granular - diagnosis? who is most at risk (2: 1 disease 1 race) cause?
benign hypertensive nephrosclerosis
elderly diabetic pts and blacks (8x)
long standing hypertension
flea bitten kidney with onion skinning of the arterioles - diagnosis? cause? course of disease? prognosis? who is at risk?
malignant hypertensive nephrosclerosis HTN with diastolic > 130 happens very quickly, hours or days >50% mortality in 3 months young black males
you find flame-shaped retinal hemorrhages and AV nicking on fundoscopy - diagnosis?
malignant hypertension
a 6 year old child eats a hamburger and 1 week later develops symptoms including purpuric rash, proteinuria, headache, and fever - diagnosis? cause?
hemolytic uremic syndrome (HUS)
usually E. coli O157:H7
a patient with HIV develops purpuric rash, headache, confusion, seizures and acute renal failure. Schistocytes are seen on peripheral blood smear - diagnosis? prognosis?
Thrombotic thrombocytopenic purpura (TTP)
most recover but 25% progress to chronic renal failure
how do you differentiate the two disorders that involve fibrin thrombi in glomeruli and small vessels, resulting in acute renal failure?
HUS and TTP
TTP has more pronounced CNS involvement
25 year old female with fibromuscular dysplasia presents with suden onset HTN and intermittent pulmonary edema - ultrasound shows asymmetrical kidney size - diagnosis? describe the changes you would see in each kidney and why
renal artery stenosis
shrunken kidney (with stenosis) has no arteriolosclerosis due to low blood flow, but this lack of blood flow causes release of renin which leads to HTN - ischemic changes include crowded glomeruli, atrophic tubules, and interstitial fibrosis
HTN leads to hypertensive, hyaline arteriolosclerosis in the unaffected kidney
associated with berry aneurysms and liver involvement
autosomal dominant polycystic kidney disease (AD for aDult)
34 year old pt presents with bilateral enlargement of kidneys involving cortex and medulla and HTN. Kidneys are palpable - diagnosis? common cause of death?
autosomal dominant polycystic kidney disease
cerebral hemorrhage from rupture of associated berry aneurysm
this disease involves a mutation in the APKD1 or APKD2 genes - diagnosis? what chromosomes are each of these genes found on and what do they code for?
autosomal dominant polycystic kidney disease
APKD1: ch16 - polycysteine II
APKD2: ch4 - polycysteine I
baby is born with HTN, hypoplastic lungs, flat face, and limb malformations - what is the name of this condition? what disease is it associated with? outcome? why is the baby’s face flat?
Potter sequence
Childhood (autosomal recessive) polycystic kidney disease
death in infancy or childhood
oligohydramnios
this disease involves a mutation in the PKHD1 gene - what disease? what chromosome? what does it code for?
Childhood (autosomal recessive) polycystic kidney disease
chromosome 6, codes for fibrocystin
an infant’s kidneys have a smooth outer surface, but a sponge-like appearance in the cortex and medulla in a radial/spoke arrangement - diagnosis? what part of the kidney is involved? associated finding?
Childhood (autosomal recessive) polycystic kidney disease
collecting ducts (Children - Collecting ducts - Cs!)
hepatic fibrosis and cysts
what can cause potter sequence in utero? what is characteristic of these fetuses?
bilateral renal agenesis -> oligohydramnios and lung hypoplasia
flat face, low set ears
patients with autosomal dominant polycystic kidney disease have an increased risk fo what?
cancer
list the 3 malignant tumors of the kidney
renal cell carcinoma (90%)
urothelial carcinoma
Wilm’s tumor in kids
painless hematuria is associated with what?
renal tumor
this renal tumor has sheets of cells and shows a central scar - name? malignancy?
renal oncocytoma
benign
this tumor presents as a white spot on the outside of the kidney, no clear cells - name? malignancy?
renal adenoma
benign
a 45 year old presents with flank pain, hematuria, and retroperitoneal hemorrhage. You find a mass in his kidney thats positive for CD117, HMB45, and actin. the tumor has a triphasic appearance. - name? malignancy?
angiomyolipoma (blood vessels, muscle, fat)
benign
cancer of the renal tubular epithelial cells - diagnosis? more common in who?
renal cell carcinoma
males 2:1
Male presents with hematuria, flank pain, and fever. He noticed a swelling in his left testicle that can be transilluminated. Should you be concerned?
Yes, left-sided varicocele can indicate renal cell carcinoma that has invaded the left renal vein
on biopsy of a kidney mass you find lots of cells with clear cytoplasm - diagnosis? why is it clear?
renal cell carcinoma - clear cell type
filled with lipid, NOT mucin
paraneoplastic syndromes of renal cell carcinoma - just read
polycythemia (erythropoietin) HTN (renin) hypercalcemia (PTH) cushings syndrome (ACTH) leukemoid reaction amyloidosis
should you use urine cytology for diagnosis of renal cell carcinoma? why or why not?
no, its not a good marker, cells get degraded during passage along UT - good for bladder cancer though
you find a single, unilateral tumor in the upper pole of a kidney in a 65 year old male - diagnosis? what is the biggest risk factor? how does it spread?
renal cell carcinoma (sporadic)
smoking
hematogenously (carcinoma exception!)
30 year old presents with bilateral masses in their kidneys, you discover there has been inactivation of the VHL gene - name of this condition? disease? what chromosome is this gene on?increased risk for what associated factor?
Von Hippel-Lindau disease
Renal cell carcinoma
chromosome 3
hemangioblastoma of the cerebellum
4 year old presents with large unilateral flank mass, hematuria, and HTN - you discover a triphasic tumor - diagnosis? what gene and chromosome is involved? what 3 cell types are involved?
wilm’s tumor
WT1 mutation on chromosome 11
blastema (immature mesenchyme), stroma, epithelium
a 3 year old presents with aniridia and mental and motor retardation - what is the name of this syndrome? what disease does it predispose him to?
WAGR syndrome
Wilms tumor
W:wilms tumor
A: aniridia
G: genital abnormalities
R: retardation (mental and motor)
a child is born with 1 sided muscle hypertrophy, hypoglycemia and a protruding tongue - name of syndrome? what does it predispose him to? what gene is involved? prognosis?
Beckwith-Wiedemann syndrome
Wilms tumor
WT2
90% 5 year survival
baby presents with flank mass, how do you differentiate between the two differentials?
wilm’s tumor or adrenal neuroblastoma
wilm’s tumor has normal VMA levels
cadmium increases your risk of what?
renal cell carcinoma
