Clinical Flashcards
what is kidney agenesis?
a congenital absence of one or both kidneys
what is kidney hypoplasia?
a congenital condition causing small kidneys with normal development and function
(reduced capacity)
what is a horseshoe kidney?
kidneys congenitally fused at either pole- usually lower
do simple cysts usually cause a functional disturbance?
no
what are the 2 main types of polycystic kidney disease?
- autosomal recessive PKD (prev known as infantile)
- autosomal dominant PKD (prev known as adult)
what is the most common subtype of ARPKD?
perinatal group
what does the perinatal group of ARPKD cause?
terminal renal failure
what happens to the medullary collecting ducts in ARPKD?
cystic dilation
what liver condition is ARPKD associated with?
congenital hepatic fibrosis
what is the most common inherited kidney disease?
autosomal dominant polycystic kidney disease
compare ADPKD 1 and ADPKD2 in terms of chromosomes affected? (give percentages)
ADPKD 1: defect on chromosome 16 (90%)
ADPKD 2: defect on chromosome 4 (10%)
what is the aetiology of ARPKD?
genetic
what is the aetiology of ADPKD?
genetic
when does ADPKD present?
usually in middle adult life
what does ADPKD present with?
abdominal mass
haematuria
chronic renal failure
hypertension
where do cysts arise in ARPKD?
medullary collecting tubules
where do cysts arise in ADPKD?
any part of the nephron
which other organs can be affected with cysts in ADPKD?
liver
pancreas
lung
what functionally happens to the liver, pancreas, lung when affected in ADPKD?
no funcional effect
what aneurysm is ADPKD associated and what can this lead to?
berry aneurysm in circle of Willis
can lead to subarachnoid haemorrhage
compare ARPKD and ADPKD in terms of the gross shape of the kidney?
ARPKD- enlargment but shape is still there
ADPKD- massive enlargement, shape is distorted
why can haematuria occur in ADPKD?
cysts can be filled with blood
compare the causes of intracerebral haemorrhage and subarachnoid haemorrhage in ADPKD?
intracerebral- due to hypertension caused by chronic renal faiulre
subarachnoid- due to berry aneurysm in circle of Willis
what is the most common benign renal tumour?
fibroma
what part of the kidney does a fibroma originate from?
medulla
what is an adenoma? (of anywhere)
a benign tumour of the epithelium
where do renal adenomas originate from?
usually capillary walls in the cortex
what type of cells do renal angiomyolipomas contain?
fat, muscle, blood vessels
what type of cells does a juxtaglomerular cell tumour arise from?
juxtaglomerular cells
what renal tumour is tuberous sclerosis associated?
renal angiomyolipomas
why can juxtaglomerular cell tumours cause secondary hypertension?
they overproduce renin
even though renal angiomyolipomas are benign, why may they cause kidney dysfunction?
because they can be large and multiple
what is the most common intra-abdominal tumour in children?
nephroblastoma (Wilm’s tumour)
what cells does a nephroblastoma (Wilms tumour) arise from?
primitive renal tissue
where do urothelial carcinomas tend to arise?
renal pelvis and calyces
where do renal cell carcinomas arise from?
renal tubular epithelium
what are renal cell carcinomas also known as?
clear cell carcinoma
hypernephroma
grawitz tumour
what is the commonest primary renal tumour in adults?
renal cell carcinoma
what age group do renal cell carcinomas tend to present in?
55-60 years old
who is more likely to get a renal cell carcinoma- M or F?
males
what does a renal cell carcinoma present with?
abdominal mass
haematuria
flank pain
systemic features of malignancy
what are the paraneoplastic manifestations of renal cell carcinoma?
erythropoietic stimulating substance: polycythaemia and increased haemaglobin
hormone similar to parathyroid: hypercalcaemia
what specific finding is a poor prognosis of a renal cell carcinoma?
renal vein extension
compare blood and lymph spread in renal cell carcinoma?
blood spread is first
lymph spread is later
which is the most common subtype of renal cell carcinoma?
clear cell type
what system is used to histologically grade renal cell carcinomas?
Fuhrman grading system
what is the most common type of bladder cancer?
transitional cell carcinoma
where can transitional cell carcinomas arise?
renal calyces right down to urethra
what industries have occupational risk of transitional cell carcinoma?
dye industry
rubber industry
hydrocarbon industry
what is the biggest risk factor of transitional cell carcinoma?
smoking
what chronic parasitic infestation is a risk factor for transitional cell carcinoma?
schistosomiasis
what is the commonest symptom of transitional cell carcinoma?
haematuria
where do 75% of transitional cell carcinomas occur?
trigone region
what does a pTa grade transitional cell carcinoma mean?
superficial and non invasive carcinomal
what does a pT1 grade transitional cell carcinoma mean?
stromal invasion
what does a pT2 grade transitional cell carcinoma mean?
detrusor muscle invasion
which lymph nodes do transitional cell carcinomas tend to spread to?
obturator nodes in pelvis
why can transitional cell carcinomas in the badder lead to hydroureter and hydronephrosis?
obstruction causing back pressure of urine
what are the 3 risk factors of an adenocarcinoma in the urinary tract?
- congenital bladder extroversion
- urachal remnants
- long standing cystitis cystica
what are urachal remnants?
when the urachus- which connects bladder to umbilical cord- doesnt become fully obliterated
what is cystitis cystica?
a benign proliferation of the bladder as a response to chronic irritation
what are the 2 risk factors of a squamous cell carcinoma in the urinary tract?
- calculi
- long term schistosomiasis
why can calculi lead to a squamous cell carcinoma?
cause irritation which leads to metaplasia then dysplasia
what is the most common malignant bladder tumour in children?
embryonal rhabdomyosarcoma
what is urinary incontinence?
complaint of any involuntary leakage of urine
what is stress urinary incontinence?
involuntary leakage of urine on effort or exertion (ie sneezing/coughing)
what is urgency urinary incontinence?
involuntary leakage of urine accompanied by urgency
what is urgency?
complaint of a sudden compelling desire to pass urine which is difficult to defer
what is overactive bladder syndrome/urge syndrome/urgency-frequency syndrome?
urgency +/- urge incontinence, usually with frequency, and nocturia
what is detrusor overactivity incontinence?
involuntary leakage of urine due to an involuntary detrusor contaction
what is mixed urinary incontinence?
involuntary leakage of urine associated with urgency and also exertion/effort
what is the name of this collection of symptoms- slow stream, splitting of urinary stream, spraying of urinary stream, hesitancy, straining?
voiding symptoms
what is a frequency volume chart?
a chart which records volumes voided and times of each micturation for at least 24 hours
what are the 4 types of urinary incontinence? (urethral route)
overflow incontinence
stress incontinence
urge incontinence
mixed incontinence
what are the 2 main causes of extraurethral route of urine?
ectopic ureter
fistula
in storage phase, compare intravesical and urethral pressure?
intravesicle pressure is less than urethral pressure
in voiding phase, compare intravesical and urethral pressure?
intravesicle pressure is more than urethral pressure
what is urodynamic testing?
determines pressures within the micturation system
in a normal situation, what happens to the intravesicle pressure on coughing?
increases
in a normal situation what happens to the abdominal pressure on coughing?
increases
in a normal situation what happens to the detrusor pressure on coughing?
no change
what is the underlying cause of overflow incontinence?
bladder outflow obstruction causing chronic retention
in overflow incontinence, is there an urgency to urinate?
no, you don’t realise you have done it
compare the frequency of urination in a normal patient to someone with urge syndrome?
frequency is increased
compare the volume of urine voided in a normal patient to someone with urge syndrome?
small voided volumes
in a patient with urge syndrome due to detrusor overactivity, what happens to the detrusor pressure on coughing?
increases
what is the main cause of urge syndrome?
detrusor overactivity
what are the causes of detrusor overactivity?
- something in the wall of the bladder causing irritation (stone, tumour)
- loss of central inhibition of micturation reflex (paraplegia)
- idiopathic
compare loss of central inhibition of micturation reflex (paraplegia) to destruction of S2,3 centre in terms of cause of urge incontinence?
loss of central inhibition (paraplegia)- overacitivty of detrusor
destruction of S2-3 centre- loss of detrusor muscle function
what is idiopathic detrusor overacitivity?
urge syndrome caused by detrusor overacitivty with no undelying cause
how do you diagnose urge incontinence/syndrome?
urodynamic testing
what causes stress incontinence?
damage to pelvic floor or urethral function
what is the most common underlying cause of damage to pelvic floor/urethral function in stress incontinence?
childbirth
how do you diagnose stress incontinence?
urodynamic testing
is there urgency in stress incontinence?
no
unless mixed incontinence
in a patient with stress incontinence, what happens to the detrusor pressure on coughing?
nothing (detrusor is working normally)
what happens to the volume of the urine leak in stress incontinence as the bladder becomes fuller?
volume increases
why might people with stress incontinence go to the toilet frequently even though there is no sense of urgency?
a learned habit, a technique to prevent the bladder volume getting large therefore preventing leaking volumes being large
what is the most likely cause of a painless palpable mass arising from the pelvis which is dull to percus and unable to get below it in a female who has amenorrhoea?
pregnancy
what is the most likely cause of a painless palpable mass arising from the pelvis which is dull to percus and cannot get below it in a middle aged male?
bladder
how do you treat overflow urinary incontinence?
catheterise and teach patient to intermittently self catheterise (rehabilitates the bladder)
what is the dietary treatment of urge urinary incontinence?
avoid caffeine
what is the pharmacological treatment of urge urinary incontinence?
antimuscarinics (oxybutynin, tolterodine)
beta 3 adrenergic (mirabegron)
what invasive surgery can be done for the treatment of urge urinary incontinence?
bladder pacemaker
enterocystoplasty (makes bladder larger)
what is the lifestyle treatment of stress incontinence?
weight loss
stop smoking
what is the physio treatment of stress incontinence?
pelvic floor exercises
what is the surgical treatment of stress incontinence?
colposuspension
tape procedures
what is the cause of an ectopic ureter?
congenital
what is the main cause of a vesico-vaginal fistula in developing countries?
prolonged obstructed labour
describe the appearance of bowen’s disease of the penis?
dry crusty appearance
describe the appearance of erythroplasia of queyrat?
red velvety appearance
what type of ‘carcinoma-in-situ’ are bowen’s disease and erythroplasia of queyrat when sited on the penis?
squamous carcinoma-in-situ
which has a bigger risk of squmaous carcinoma of the penis- circumscised or uncircumcised?
uncircumcised
where in the penis does squamous carcinoma tend to occur?
glans or prepuce
what are the risk factors of squamous carcinoma of the penis?
poor hygiene
HPV
phimosis
what occupational risk can predispose to SCC of scrotum?
chimney sweeps
what is benign nodular hyperplasia of the prostate?
irregular proliferation of both glandular and stromal prostatic tissue within the prostate
what is the aetiology of benign nodular hyperplasia?
hormonal imbalance
alteration of androgen:oestrogen ratio
what is prostatism?
a group of symptoms caused by prostate disease
what are the main consequences of benign nodular hyperplasia of the prostate?
bladder hypertrophy
diverticulum
hydroureter/hydronephrosis
infection
what is the management of benign nodular hyperplasia?
usually drugs: alpha blockers, 5 alpha reductase inhibitors
surgery: transurethral resection
what is the peak incidence of prostate carcinoma?
60 - 80 years old
where in the prostate are carcinomas most likely to occur?
peripheral ducts and glands
usually posterior lobe
why are symptoms of prostatism a sign of advanced prostate cancer?
peri-urethral zone involved at a later stage
why are prostatic cancer bone mets distinct?
osteosclerotic instead of osteolytic
what protein is usually increased in prostatic carcinomas?
prostate specific antigen (PSA)
how do you take a biopsy of the prostate?
transurethal resection
multiple needle core biopsies under US (trans rectal)
what might you feel on a PR exam of a prostate carcinoma?
craggy, hard, irregular mass
what is the drug management of a prostate carcinoma?
hormone therapy:
anti-androgens
LHRH agonists
oestrogens
what is the management of prostatic carcinoma bone mets?
radiotherapy
what is the surgical management of a prostate carcinoma?
radical prostatectomy
what type of prostate carcinoma is a radical prostatectomy reserved for?
organ-confined disease
what is a major risk factor for a testicular tumour?
maldescent
what is the usual presenting complaint of a testicular tumour?
testicular enlargement
describe the pain felt with testicular enlargement in a testicular tumour?
painless
why can gynaecomastia be a feature of testicular tumour?
hormonal secretion
what is the main type of testicular tumour?
germ cell tumours
what are the types of germ cell testicular tumours?
seminoma
teratoma
mixed
what are the types of stromal testicular tumour?
sertoli cell
leydig cell
which specific stromal testicular cell tumour is known to cause gynaecomastia?
leydig cell tumour
what is the most common type of germ cell testicular tumour?
seminoma
what is the peak age of incidence of a seminoma?
30-50 years old
which lymph nodes does a seminoma usually spread to?
para-aortic lymph nodes
why is there such a high cure rate for seminomas, even with mets?
very radiosensitive
what is the peak age of incidence of a teratoma?
20-30 years
what cells does a teratoma arise from?
all 3 cell lines: endoderm, mesoderm, ectoderm
what are the 4 types of teratoma?
differentiated teratoma (DT)
malignant teratoma intermediate (MTI)
malignant teratoma undifferentiated (MTU)
malignant tertoma trophoblastic (MTT)
which of the teratomas is benign?
differentiated teratoma (DT)
which of the teratomas is entirely malignant?
malignant teratoma undifferentiated (MTU)
which of the teratomas contains trophoblastic (placental) tissue?
malignant teratoma trophoblastic (MTT)
which of the teratoma contains a mixture of differentiated and undifferentiated tissue?
malignant teratoma intermediate (MTI)
what hormone can malignant teratoma trophoblastic tumours secrete?
human chorionic gonadatrophin (bHCG)
what is a mixed seminoma teratoma tumour?
a type of germ cell tumour of the testes with seminoma and any variant of teratoma
what hormone can seminomas secrete?
placental alkaline phospatase (PLAP)
what part of the embryo secretes alpha fetoprotein? (AFP)
yolk sac
what tumours can secrete alpha fetoprotein? (AFP)
germ line tumours (testicular or ovarian)
hepatocellular carcinomas
liver mets
compare glomerulonephritis and pyelonephritis in terms of what causes it?
glomerulonephtiris- immunologcal basis
pylonephritis- infectious agent
why can glomerulonephritis occur several weeks after an infection despite it being non-infective?
immunological mechanism (ie antibody production)
what are the 2 main types of glomerulonephritis?
diffuse
focal
which is more common- diffuse or focal glomerulonephritis?
diffuse
what is the main type of infectious agent which causes pyelonephritis
bacterial infection
what parts of the kidneys are involed in pyelonephritis?
renal pelvis, calyces
spread into the tubules and interstitium
what is the most common organism of pyelonephritis?
E. Coli
what are the 2 subtypes of pyelonephritis?
acute
chronic
is pyelonephritis more common in F or M?
females
what are the 2 ways of infection spread causing pyelonephritis? -which is more common?
haematogenous (rare) ascending infection (common)
what is cystitis?
infection/inflammation of the bladder
why is pyelonephritis more common in females?
they have a shorter, wider urethra
why can pregnancy be a risk factor for pyelopnephritis?
ureteric dilatation with urine stasis because of:
- hormonal effects
- anatomial effects
what hormonal effects in pregnancy causes ureteric dilation with urine stasis? (a risk factor for pyelonephritis)
relaxation of smooth muscle in ureters
what anatomical effects in pregnancy cause ureteric dilation with urine stasis? (a risk factor for pyelonephritis)
obstruction from pregnant uterus
what is a major risk factor for pyelonephritis due to urine stasis?
urinary tract obstruction
what type of reflux can be a risk factor for pyelonephritis?
vesico-ureteric reflux
how can vesico-ureteric reflux be congenital?
ureters enter bladder perpendicular instead of oblique
what condition is a risk factor for pyelonephritis due to sugar content of urine?
diabetes
why do patients with chronic pyelonephritis urinate large volumes?
kidney damage so isn’t able to concentrate urine as effectively
what infection of the kidney does ‘sterile pyuria’ indicate?
TB
how does TB spread to the kidneys?
haemotengous spread (usually from lung primary)
what is dysuria?
painful passing of urine
what is the principle techniquie for diagnosing TB?
PCR
what is the type of inflammation/necrosis that occurs with TB?
caseating granulomatous inflammation
when can cystitis become necrotising?
if associated with outlet obstruction
what can form within the benign hyperplasia of ureteritis or cystitis cystica?
fluid filled cysts
is urethral obstruction more common in F or M?
M
why is urethral obstruction more common in males?
they have a longer, tortuous urethra
what is the main cause of bladder outlet obstruction in a newborn male?
posterior urethral valves (in utero development abnormality)
what are the 2 main causes of hydronephrosis?
urinary tract obstruction
prolonged vesico-ureteric reflux
would a neurogenic disturbance (ie in a paraplegic patient) cause unilateral or bilateral hydronephrosis?
bilateral
would a urethral obsturcion cause unilateral or bilateral hydronephrosis?
bilateral
would a calculi or neoplasm in a ureter cause unilateral or bilateral hydronephrosis?
unilateal
what happens to urine production if there is a sudden and complete obstuction?
urine production quickly ceases
what happens to urine production if there is gradual and partial obstruction?
urine production remains the same
compare sudden and complete obstruction to gradual and partial obstruction in terms of hydronephrosis?
sudden: little dilation
gradual: dilation
what is the term for secondary infection of a hydronephrotic kidney?
pyonephrosis
how do you determine whether there is haematuria?
urine dipstick test
what does macroscopic haematuria mean?
visible haematuria
wht does microscopic haematuria mean?
non-visible haematuria
what is a common contaminate of urine in a women of child-bearing age, causing it to become red?
menstruation
why might there be myglobin within the urine? (causing it to become red)
rhabdomyolisis mcArdle disease (metabolic disorder) bywaters/crush syndrome
what drugs cause red urine?
doxyrubicine chloroquine rifampicin nitrofurantoin senna containing laxatives
what toxins can cause red urine?
lead
mercury
what colour urine might increased urobilinogen in the urine cause?
brown coloured urine
what causes pneumaturia?
any connection between bowel and bladder
what causes faecaluria?
any connection between bowel and bladder
on CT urogram, what is indicated if there is a defect of bladder filling?
there is an obstruction within the bladder
what is a urethrocystoscopy?
an endoscopic picture of the bladder by placing an endoscope through the urethra
what is post-obstructive diuresis?
dramatic increase in urine output (200ml/hr) after release of urinary tract obstruction (must be bilateral- ie both kidneys affected)
what are the 2 factors necessary for post-obstructive diuresis?
accumulation of total body water, sodium and urea (eg oedema, CCF, hypertension, uraemia)
OR
impairement of tubular re-absorption
compare physiological post-obstructive diuresis to pathological post-obstructed diuresis?
physiological- self limiting, stops after return to euvolaemic state
pathological- inappropriate diuresis beyond euvolaemic state
usually post-obstructive diuresis is self limiting, how long does this take?
24-48 hours
in severe cases of post-obstructive diuresis beyond euvolaemic state, what management is needed?
IV fluid
sodium replacement
what is the treatment for ureteric colic?
NSAIDs +/- opiate,
+ alpha blocker (tamsulosin) for small stones expected to pass
wait for 1 month to see if surgical intervention is necessary
what is the likelihood of spontaneous passage of renal stones if they are less than 4mm?
80%
what is the likelihood of spontaneous passage of renal stones if they are between 4-6mm?
50-60%
what is the likelihood of spontaneous passage of renal stones if they are above 6mm?
20%
what are the indications for urgent intervention of a renal stone?
pain unrelieved
pyrexia
persistent nausea and vomiting
high-grade obstruction
what is the intervention for renal stones in the absence of infection?
ureteric stent
stone fragmentation
what is the intervention for renal stones with infected hydronephrosis?
percutaneous nephrostomy
what age is torsion of the spermatic cord most common?
puberty
torsion of spermatic cord is usually spontaneous but may occur with what?
trauma
athletic activities
describe the pain with torsion of spermatic cord?
sudden onset severe pain
may have prev episodes of self limiting pain
referral of pain to lower abdomen
on examination of torsion of spermatic cord what signs do you see?
testis high in scrotum
transverse lie
absence of cremasteric reflex
what is the management of torsion of spermatic cord?
prompt surgical exploration
why must you fix the contralateral side in testicular torsion?
to prevent it occuring to the other testis
-due to bell clapper deformity
what is the most common cause of testicular torsion?
bell clapper deformity
how is torsion of appendage differentiated from torsion of spermatic cord?
testis should be mobile
cremasteric reflex present
blue dot sign
what is generally seen in the history of epididymitis?
UTI
urethritis
catheterisation/instrumentation
is the cremasteric reflex present in epididymitis?
yes
what is a general indicator of epididymitis over testicular torsion?
pyrexia
what will you see on doppler US of epididymitis?
swollen epididymis, increased bloodflow
what is the management of confirmed torsion of appendage?
will resolve spontaneously
what is the management of epididymitis?
analgesia + scrotal support
bed rest
ofloxacin 400mg 14 days
instead of pain, what may be felt in idiopathic scrotal oedema?
pruiritis
what is paraphimosis?
painful swelling of foreskin distal to a phimotic ring
what often causes paraphimosis within hospital?
forgetting to replace foreskin in natural position after catheterisation or cystoscopy
what is priapism?
prolonged (often painful) erection >4hours
is priapism associated with sexual arousal?
no
what are the 5 causes of priapism?
- intracorporeal injection for erectile dysfunction
- trauma
- haematological conditions (eg sickle cell)
- neurological conditions
- idiopathic
what are the classifications of priapism?
ischaemic (low flow)
non-ischamic (high flow)
what is seen in the aspirate of blood from corpus cavernosum in low flow (ischaemic) priapism?
dark blood, low O2, high CO2
what is seen in the aspirate of blood from corpus cavernosum in high flow (non-ischaemic) priapism?
normal arterial blood flow
what is seen in duplex US in low flow (ischaemic) priapism?
minimal or absent flow
what is seen in duplex US in high flow (non-ischaemic) priapism?
normal to high flow
what is the management of ischaemic priapism?
aspiration +/- irrigation with saline
injections of alpha-agonist
surgical shunt
(only if early presentation)
what is the management of a non-ischaemic priapism?
observe, may resolve spontaneously
if not: selective arterioal embolisation with non-permanent materials
what is fornier’s gangrene?
necrotising fasciitis occuring around the male genitalia
what are the 4 main risk factors of fornier’s gangrene?
diabetes
local trauma
periurethral extravasation
perianal infection
how does fornier’s gangrene start?
as a cellulitis: swollen, red, tender, pain, fever
what investigations can confirm gas in the tissues in fornier’s gangrene?
US or X-ray
what is the management of fornier’s gangrene?
antibiotics
surgical debridement
what types of pathogens cause emphysematous pyelonephritis?
gas forming uropathogens
usually E coli
what risk factors predisposed to emphysematous pyelonephritis?
diabetics
ureteric obstruction
what investigations can confirm gas in the tissues in emphysematous pyelonephritis?
CT KUB
what does a perirenal abscess usually result from?
a rupture of an acute cortical abscess
or from haematogenous seeding from other sites of infection
how do you investigate a perirenal abscess?
CT
what is the management of a perinephric abscess?
antibiotics
percutaneous or surgical drainage
describe renal trauma type 1?
non-expanding haematoma, subcapsular, no parenchymal laceration
describe renal trauma type 2?
laceration less than 1cm parencymal depth
no urinary extravasation
describe renal trauma type 3?
laceration greater than 1cm
no collecting system rupture or extravasation
describe renal trauma type 4?
laceration through cortex, medulla and collecting system
arterial/venous injury with contained haemorrhage
describe renal trauma type 5?
shattered kidney
avulsion of hilum
devascularised kidney
what are the indications for imaging the kidneys after trauma in an adult?
frank haematuria
non visible haematuria + shock/penetrating injury
what are the indications for imaging the kidneys after trauma in a child?
frank or non-visible haematuria
what is the investigation for imaging the kidneys after trauma?
CT contrast
what fracture is bladder injury most commonly associated with?
pelvic fracture
what are the 6 main signs of bladder injury?
suprapubic/abdo pain inability to void suprapubic tenderness lower abdo bruising guarding diminished bowel sounds
what is the imaging investigation of choice for possible bladder trauma?
CT cystography
on examination of a urethral injury what 5 main signs are seen?
blood at external urethra meatus inability to urinate palpably full bladder 'high riding' prostate butterfly perineal haematoma
what is the imaging investigation of choice for possible urethra trauma?
retrograde urethrogram
what fractures are urethral injuries often associated with?
fracture of pubic rami
when do penile fractures typically occur?
during sex
-buckling injury when penis slips out of vagina and strikes pubis
what sound is heard on penile fracture?
cracking or popping
what are the symptoms of penile fracture?
pain
rapid detumescence
discolouration
swelling
what is the management of a penile fracture?
prompt exploration and repair
‘degloving of penis’ to expose all 3 compartments
what is the investiation of choice for testicular trauma?
ultrasound
what is the management of a urethral injury?
supraupubic catheter
delayed reconstruction after at least 3 months
what is the management of bladder injury with no indications for immediate repair?
antibiotics
repeat cystogram in 14 days
what is the verumontanum of the prostate?
where the ejaculatory ducts drain to each side of the prostatic urethra
what is the transitional zone of the prostate?
the area which surrounds the urethra, proximal to the verumontanum
what is the central zone of the prostate?
cone shaped region which surrounds the ejaculatory ducts
what is the peripheral zone of the prostate?
posteriolateral prostate
which zone of the prostate gives rise to benign prostate hyperplasia?
transitional zone
which zone of the prostate gives rise to the majority of carcinomas?
peripheral zone
what is the anterior part of the prostate made up?
fibromuscular stroma
what is the most common malignancy affecting men in the UK?
prostate cancer
what type of cancers are the majority of prostate cancers?
multifocal adenocarcinomas
what scoring system grades prostate cancers?
gleason’s scoring
what hormones is the growth of prostate cancer cells under the influence of?
testosterone
dihydrotestosterone
what happens if prostate cells are deprived of androgenic stimulation?
undergo apoptosis
what can initially happen on treatment of prostate carcinoma with LHRH agonists?
initial androgen surge
what can be given to prevent initial androgen surge with LHRH?
anti-androgens
what is the function of LHRH in prostate carcinoma?
cause suppression of pituitary LH and FSH secretion and therfore testosterone production
compare steroidal and non-steroidal anti-androgens in terms of libido and sexual interest?
steroidal (eg cyprosterone): loss of libido and sexual interest
non-steroiral: no loss of libido or sexual interest
what are the 2 types of transitional cell carcinoma? and give percentages
papillary 80%
non papillary 20%
compare papillary and nonpapillary transitional cell carcinomas in terms of percentage that are considered to be malignant?
papillary: 50%
non-papillary: all considered to be malignant
what are the 2 subtypes of papillary transitional carcinoma?
papilloma
invasive papillary carcinoma
what are the 2 subtypes of nonpapillary transitional carcinoma?
flat non invasive carcinoma
flat invasive carcinoma
what are the most common benign asymptomatic renal lesions?
benign renal cysts
what imaging is best for looking at a renal cyst?
ultrasound
what is the main consequence of angiomyolipomas?
haemorrhage
may have wunderlich’s syndrome- massive retroperitoneal bleeding
what imaging is best for looking at a suspected angiomyolipoma?
CT
what benign renal tumour can appear to be a carcinoma?
oncoytoma
what feature of oncocytoma is very characteristic?
stellate scar
what type of carcinoma is a renal cell carcinoma?
adenocarcinoma
what part of the kidney is affected by renal cell carcinoma?
proximal convoluted tubule
if renal cell carcinoma is multifocal or bilateral then what syndrome should you suspect?
von hippel-lindau syndrome
what is the best imaging for diagnosing a renal cell carcinoma?
triple phase contrast CT
what is the main form of renal cell carcinoma treatment?
radical or partial nephrectomy
what are the 4 main premalignant conditions of penile cancer?
Bowen’s disease
Erythroplasia of Queyrat
Balanitis Xerotica Obliterans
Leukoplakia
what do you see in balanitis xerotica obliterans of the penis?
white patches, fissuring, bleeding, scarring of prepuce and glans
what is the treatment of balantis xerotica obliterans?
circumcision
may need glans resurfacing
what is the main difference in location of bowen’s disease and erythroplasia of queyrat of the penis? (both are squamous cell carcinoma in situ)
erythroplasia of queyrat is on glans, prepuce or shaft
bowen’s is on the other parts of genitalia
what is the treatment of bowen’s disease or srythroplasia of queyrat of the penis?
circumcision
topical 5-fluorouracil
what is the surgical treatment of a penis carcinoma?
total/partial penectomy
reconstruction
what is the best imaging technique for suspected testicular tumour?
ultrasound
what hormone is 100% elevated in a malignant teratoma trophobastic?
bHCG
what is the main underling cause for glomerulonephritis?
immune-complex deposition
why can renal artery stenosis worsen pre-existing hypertension?
RAS kicks in
what type of necrosis is found in the kidneys due to malignant hypertension?
fibrinoid necrosis
compare seminomas and teratomas in terms of radio/chemotherapy?
seminomas- radiosensitive
teratomas- chemosensitive
why can a patient become anaemia due to kidney failure?
loss of production of erythropoietin
what must you do to a patient who has a pericardial rub in the presence of uraemia?
immediate dialysis
why might vomiting cause acute kidney injury?
due to dehydration
what does ACEI/ARBs have a protective function against?
proteinuria
-help preserve kidney function
what does ACEI/ARB have a negative effect on?
dehydration
ie in vomiting
who is at risk of contrast nephropathy?
patients who are dehydrated
patients who already have renal impairment
in what condition are you most likely to feel palpable kidneys?
ADPKD
why must you always correct hypotension in kidney disease?
kidneys need a certain perfusion to work
below what blood pressure should you aim to get a patient who has kidney disease?
less 130/80
what is the diastolic pressure above in accelerated hypertension?
above 120mmHg
what can be seen on fundoscopy of accelerated hypertension?
papilloedema
what is leukonychia found in?
profound hypoalbuminaemia
what immunoglobulin is involved in Henoch-Schonlein Purpura?
IgA
what is the classic distribution of Henoch-Schonlein Purpura?
extensor surfaces of legs and buttocks
what is rhabdomyolysis and why can it cause kidney injury?
muscle breakdown
myoglobin is a product and cannot be processed properly by the kidney
what is the CK like in rhabdomyolysis?
very high
how many grams of protein in the urine classes as asymptomatic low grade proteinuria?
up to 1g
how many grams of protein in the urine classes as heavy proteinuria?
1-3g per day
how many grams of protein in the urine classes as within nephrotic range?
> 3g per day
what are the 2 main ways to quantify urine protein?
24 hour urinary collection
urine protein/creatinine ratio
under microscopy of urine, compare what isomorphic and dysmorphic red blood cells indicate?
isomorphic- coming from lower down renal tract
dysmorphic- coming frohigher up the renal tract
what biochemistry abnormality does tented T waves on ECG suggest?
hyperkalaemia
what does hyperkalaemia eventually lead to in terms of patients heart rate/pulse?
cardiac arrest
what stage of kidney disease does GFR >90 with symptoms indicate?
stage 1
kidney damage with normal GFR
what stage of kidney disease does GFR from 60-89 with symptoms indicate?
stage 2
kidney damage with mildly reduced GFR
what stage of kidney disease does GFR from 30-59 indicate?
stage 3
moderately reduced GFR
what stage of kidney disease does GFR from 15- 29 indicate?
stage 4
severely reduced GFR
what stage of kidney disease does GFR of less than 15 indicate?
stage 5
kidney failure
what are the main features of nephrotic syndrome?
proteinuria >3g per day
hypoalbuminuria
oedema
(hypercholesterolaemia)
why might it be hard to determine if someone has nephrotic syndrome?
often have normal renal function
oedema of what site is a classical sign of nephrotic syndrome?
periorbital oedema
compare nephrotic syndrome and nephritic syndrome in terms of pulmonary oedema?
no pulmonary oedema in nephrotic syndrome
pulmonary oedema in nephritic syndrome
why do you not tend to get pulmonary oedema in nephrotic syndrome even though oncotic pressure is low?
becasue the capillary pressure within the lungs is still very low
which are you more likely to get with acute kidney injury- nephrotic or nephritic syndrome?
nephritis syndrome
what are the 5 main symptoms/signs of nephritic syndrome?
oliguria hypertension oedema proteinuria haematuria
what is chronic kidney disease?
reduced GFR over a length of time
what is eGFR calculated using?
serum creatinine
when muscle mass is low, is eGFR under or over estimated?
over estimated
when muscle mass is high is eGFR under or over estimated?
under estimated
stage 1 and stage 2 of CKD are dependent on evidence of kidney damage, what does this mean?
proteinuria, haematuria (in absence of lower urinary tract cause), or abnormal imaging
what does CKD do to cardiovascular disease?
increases risk
what does proteinuria do to the likelyhood of CKD to progress stages?
increases risk of progression
why can reflux nephropathy cause CKD?
due to recurrent UTI causing scarring
what is the most common cause of CKD?
diabetes
what might happen initially to gfr when putting a patient with CKD on an ACEI/ARB?
initial fall
what do ACEI/ARB do to proteinuria?
reduces proteinuria
what does smoking do to the rate of progression of CKD?
increases rate progression
at what stage of CKD are statins recommended?
stage 4
how do you correct iron deficiency anaemia in CKD?
IV iron
if patient on CKD is anaemic but iron has been replaced, what hmight be indicated?
erythropoietin injections
why can bone disease occur in CKD?
vitamin D cant be hydroxylated properly
leading to reduced calcium absorption
leading to secondary hyperparathyroidism which takes calcium out of bones to maintain serum calcium
what happens to the levels of serum phosphate in CKD and what does this do to the levels of PTH?
serum phosphate increases
PTH increases
what eventually happens to CKD patients in secondary hyperparathyroidism?
tertiary hyperparathyroidism
what happens to the blood vessels and heart valves in a patient with CKD who has tertiary hyperparathyroidism?
vascular and valvular calficiation
how do you treat bone disease in CKD?
alfacalcidol (hydroxylated vit D)
phosphate binders
at what gfr should dialysis education be started?
20ml/min (earlier if progressing fast)
what is the best form of access for haemodialysis?
arteriovenous fistula
how long does it take for an arteriovenous fistula to mature for haemodialysis?
6 weeks
at what gfr should you refer a patient to vascular surgeons for the creating of an arteriovenous fistula?
15ml/min
how long after creation of a catheter for peritoneal dialysis can it be used?
1-2 weeks
when do patients get put on the cadaveric transplantation list for a kidney?
within roughly 6 months of dialysis
what is acute kidney injury?
an abrupt reduction in kidney function defined as an absolute increase in serum creatinine by 26.4micromoles per litre (or 50% increase over baseline)
how should you split the causes of AKI?
pre renal
renal
post renal
what does pre-renal causes of AKI mean?
anything that reduces kidney perfusion
what does post-renal causes of AKI mean?
obstruction of renal outflow
what does renal causes of AKI mean?
intrinsic causes
what are the 3 subgroups of pre-renal AKI?
hypovolaemia
hypotension
renal hypoperfusion
why might haemorrhages, diarrhoea, vomitting or burns cause AKI?
causes hypovolaemia which is a pre-renal cause of AKI
why might cardiogenic, septic or anaphylactic shock cause AKI?
causes hypotension which is pre-renal cause of AKI
why might NSAIDs or ACEI/ARBs cause AKI?
reduces renal perfusion which is a pre-renal cause of AKI
what is hepatorenal syndrome?
kidney failure as a result of liver failure
why does hepatorenal syndrome cause AKI?
reduces renal perfusion which is a pre-renal cause of AKI
how do you calculate urine output depending on weight?
0.5ml/kg/hr
what defines oliguria?
less than 0.5mls/kg/hr of urine output
what does untreated pre-renal AKI lead to?
acute tubular necrosis
what is the commonest form of AKI?
acute tubular necrosis due to decreased renal perfusion (from a combination of factors)
what is the main aim of pre-renal AKI treatment?
reverse factors that have caused it (ie eupportive treatment)
to reverse hypotension causing AKI, what do you do?
fluid challenge with saline, if over 1000mls has been given with no improvement seek help
what are the 4 subgroups of renal AKI?
vascular disease
glomerular disease (glomerulonephritis)
interstitial injury
tubular injury
what is the main cause of vascular disease causing renal AKI?
vasculitis (eg ANCA associated)
what are the 3 main causes of interstitial nephritis causing renal AKI?
drugs
infection eg TB
systemic causes eg sarcoidosis
what types of drugs cause interstitial nephritis?
PPI
NSAIDs
antibiotics
what type of renal AKI does rhabdomyolysis cause?
tubular injury
what kind of renal AKI does contrast cause?
tubular injury
what does renal vascular bruits indicate?
renal artery stenosis
why can compartment syndrome lead to AKI?
causes rhabdomyolysis (a renal cause of AKI)
what initial tests alow you to look for myeloma?
protein electrophoresis and bence-jones protein
what 3 things must you ensure before performing a renal biopsy?
normal clotting
normotensive
no hydronephrosis
what imaging technique do you usually use to assist with renal biopsy?
ultrasound
what are the 4 indications for immediate dialysis?
- hyperkalaemia over 7 (or 6.5 unresponsive to medical therapy)
- fluid overload
- severe acidosis (pH below 7.15)
- uraemia (urea over 40) with pericardial effusion
what are the 2 ways of treating post renal AKI to relieve obstruction?
catheter
nephrostomy
what is the normal range for serum potassium?
3.5-5
what range of potassium indicates hyperkalaemia?
over 5.5
what range of potassium indicates life threatening hyperkalaemia?
over 6.5
what happens to the T wave, P wave and QRS complex in hyperkalaemia? (on ECG)
T wave becomes peaked
loss p of wave
widening of QRS complexes
what 4 drugs are indicated in acute life threatening hyperkalaemia?
calcium gluconate
insulin
dextrose
nebulised salbutamol
what is the function of calcium gluconate in hyperkalaemia?
protects myocardium
what is the function of insulin, dextrose and salbutamol in hyperkalaemia?
moves K back into cells
what is the function of calcium resonium?
prevents K absorption from the GI tract
what drug do you give a patient who is acidotic?
sodium bicarbonate
what are the 3 main nephrotoxic drugs?
NSAIDs
ACEI/ARB
antibiotics (eg gentamicin)
what are the 4 main risk factors for AKI development?
age, diabetes, CKD, co-morbidities
what is the most common cause of end stage renal disease?
diabetes
what is the second most common cause of end stage renal disease?
chronic glomerulonephritis
what is glomerulonephritis?
immune-mediated disease of the kidneys afecting the glomeruli
does damage to endothelial or mesangial cells lead to a proliferative or non-proliferative glomerulonephritis?
proliferative
does damage to podocytes lead to a proliferative or non-proliferative glomerulonephritis?
non-proliferative
what abnormality is found in urine in proliferative glomerulonephritis?
red blood cells
some protein
what abnormality is found in urine in non-proliferative glomerulonephritis?
protein
what does microalbuminuria mean?
30-300mg of albuminuria per day
is nephritic syndrome indicative of a proliferative or non-proliferative glomerulonephritis?
proliferative glomerulonephritis
is nephrotic syndrome indicative of a proliferative or non-proliferative glomerulonephritis?
non-proliferative
compare nephritic and nephrotic syndrome in terms of renal function?
nephritic- renal failure
nephrotic- normal renal function usually
why might nephrotic syndrome cause you to become mildly immunosuppressed?
lots of antibodies leave in the urine
why is there a prothrombotic state in nephrotic syndrome?
liver increases production of prothrombotic factors (exacerbated by volume depletion)
which vein should you be particularly concerned about thrombosis in within nephrotic syndrome?
renal vein thrombosis
compare focal and diffuse glomerulonephritis?
focal- less than 50% of glomeruli affected
diffuse- more than 50% of glomeruli affected
compare global and segmental glomerulonephritis?
global- all of glomerulus affected
segmental- parts of glomerulus affected
what drugs should be used for hypertension in the treatment of GN and why?
ACEI/ARB
also control proteinuria
what indicates complete nephrotic syndrome remission?
proteinuria less than 300mg per day
what indicates partial nephrotic syndrome remission?
proteinuria less than 3g per day
what is the most common cause of nephrotic syndrome in children?
minimal change glomerulonephritis
what do you see on light microscopy, electron microscopy and immunofluorescence (renal biopsy) of minimal change glomerulonephritis?
LM- normal
IF- normal
EM- foot process fusion
can minimal change nephropathy progress to renal failure?
no
what is the main treatment for minimal change glomerulonephritis?
oral steroids
is minimal change glomerulonephritis proliferative ir non-proliferative?
non proliferative
what is the most common cause of nephrotic syndrome in adults?
focal segmental glomerulosclerosis
can focal segmental glomerulosclerosis progress to renal failure?
yes
is focal segmental glomerulosclerosis proliferative or non-proliferative?
non proliferative
what is the 2nd most common cause of nephrotic syndrome in adults?
membranous nephropathy
what is seen on renal biopsy of membranous nephropathy?
immune complex deposition in the basement membrane
can membranous glomerulonephritis progress to renal failure?
yes
is membranous glomerulonephritis proliferative or non-proliferative?
non proliferative
what is the most common type of glomerulonephritis?
IgA nephropathy
can IgA nephropathy progress to renal failure?
yes
is IgA nephropathy proliferative or non-proliferative?
proliferative
what vasculitis is IgA nephropathy associated with?
Henoch-Schonlein Purpura
what glomerulonephritis is associated with glomerular crescents?
rapidly progressive glomerulonephritis
what are the 2 subgroups of rapid progressive glomerulonephritis?
anca positive
anca negative
what type of glomerulonephritis is SLE associated with?
rapidly progressive glomerulonephritis
is rapidly progressive glomerulonephritis proliferative or non proliferative?
proliferative
what type of glomerulonephritis is goodpastures disease associated with?
rapidly progressive glomerulonephritis
compare post-strep glomerulonephritis and IgA nephropathy in terms of how long after URTI it occurs?
post strep- 2-3 weeks
IgA nephropathy- 2-3 days
is post strep glomerulonephritis proliferative or non-proliferative?
proliferative
what does CKD do to the risk of cardiovascular disease?
increases it
what kind of diet must a patient who is on dialysis have?
fluid restricted (1l per day)
low salt
low potassium
low phosphate (take phosphate binders with meals)
what is the gold standard dialysis access?
fistula
compare a fistula to other dialysis access in terms of infection risk?
fistula access has a reduced infection risk
what can happen if haemodialysis isn’t done by a gradual build up?
disequilibrium syndrome
-cerebral oedema and seizures
what type of diabetic patients can get a kidney pancreas dual transplant?
type 1 diabetic patients
which MHC class are HLA A, HLA B and HLA DR?
class 1: HLA A, HLA B class II, HLA DR
what antibiotics are given for pneumocystits jirovecii?
co-trimoxazole
what type of skin cancer is much increases post transplant?
SCC
what cancercan does post-transplant EBV infection cause?
lymphoma
what are the 3 phases of rejection?
hyperacute (minutes)
acute
chronic
what is hyperacute rejection caused by?
preformed antibodies
how is hyperacute rejection treated?
unsalvageable
what is acute rejection caused by?
T or B cell mediated response
what are the 3 phases of transplant immunosuppression?
induction
consolidation
maintenance
what drug must azathioprine never be given with?
allopurinol
what are the types of donor kidney?
- deceased brain dead
- deceased cardiac death
- live donor
what is diabetic nephropathy defined by?
albuminuria (greater than 300mg in 24 hours) on 2 occasions 3-6 months apart
what are the haemodynamic changes that diabetes does to the afferent arteriolar? and how?
vasodilation of afferent arteriole by vasoactive mediaprs
why does diabetes initially cause a raised GFR?
vasodilated afferent arteriole increases the blood flow and so increases the filtration pressure
how does diabetes cause renal hypertrophy?
plasma glucose stimulates several growth factos within the kidneys
what are kimmelstein wilson lesions?
nodular lesions of diabetic glomerulosclerosis
why does proteinuria occur in diabetes?
glomerular basement membrane thickens and the podocytes become impaired (bigger spaces between them)
why may haematuria require renal biopsy in the context of diabetic nephropathy?
haematuria is not a feature of diabetic nephropathy so need to look for another cause
how do you prevent/treat diabetic nephropathy?
- good glycaemic control
- good blood pressure control
- lipid sontrol
what drugs are used as anti-hypertensive therapy to prevent/treat diabetic nephropathy?
ACE I/ARB
what drugs are used to maintain good lipid control to prevent/treat diabetic nephropathy?
statin
how do ACE I/ARBs help to reduce progression of proteinuria in diabetics?
dilates the efferent arteriole so reduces the filtration pressure
(this pressure was initially increased because of filation of the afferent arteriole due to diabetes)
what drug should you offer a diabetic patient with persistent microalbuminuria who is normotensive?
ACE I/ARB
what drugs should you offer a diabetic patient with persistent microalbuminruria and hypertension?
ACE I/ARB
plus diuretic or another antihypertensive
what is renovascular hypertension?
hypertension secondary to renal artery stenosis (renovascular disease)
why does renal artery stenosis cause hypertension?
reduces renal perfusion activates RAS system
what are the 2 main types of renovascular disease?
fibromuscular dysplasia
atherosclerotis renovascular disease
what is ischaemic nephropathy?
reduced renal blood flow (ie renal artery stenosis) beyond the level of autoregulatory compensation so gfr is reduced
what does ischaemic nephropathy lead to?
renal atrophy and progressive CKD
what is the treatment for renal artery stenosis?
blood pressure control
reduce cardiovascular risk factors
angioplasy
stenting
what is multiple myeloma?
a cancer of plasma cells which accumulates in the bone marrow
why can you get normocytic anaemia in multiple myeloma?
plasma cell accumulation in bone marrow and so intereferes with production of red blood cells
what paraprotein does myeloma tend to produce?
Bence Jones protein
why are the proteins secreted in myeloma not detected on urine dipstick?
urine dipstick only tests for albumi so doesnt detect abnormal paraproteins
what type of lesions are found on skeletal survey of multiple myeloma?
lytic lesions
what are the 4 renal manifestations of myeloma?
hypercalcaemia (leading to AKI)
monoclonal immunoglobulin deposition disease
cast nephropathy
amyloidosis
what are the 2 types of amyloidosis?
primary
secondary
what type of conditions does secondary amyloidosis occur in?
chronic inflammatory conditions
what is seen histologically in amyloidosis?
positive congo red staining showing apple-green birifringence under polarised light
why can GPA, eGPA, MPA have pulmonary haemorrhage?
as a consequence of alveolar capillary involvement
what is seen on renal biopsy of GPA, MPA and eGPA?
segmental necrotising glomerulonephritis
what are the 6 classes of lupus nephritis?
1: minimal mesangial
2: mesangial proliferative
3: focal proliferative
4: diffuse proliferative
5. membranous
6: advanced sclerosing
how do you calculate the therapeutic index?
Lethal Dose 50/Effective Dose 50
what are the 2 phases of drug metabolism?
phase 1: oxidation, reduction and hydrolysis
phase 2: conjugation (makes drug water soluble)
do adverse drug reactions tend to happen in phase 1 or phase 2 of metabolism?
phase 1
what are type A drug reactions?
dose dependent and predictable
what are type B drug reactions?
idiosyncratic
-dose independent and unpredictable
what are type C drug reactions?
chronic effects
What are type D drug reactions?
delayed effects
What are type E drug reactions?
end of treatment efects
what are type F drug reactions?
failure of therapy
what are the most common type of drug reactions?
type A
compare ADPKD type 1 to ADPKD type 2 in terms of progression to end stage kidney failure?
ADPKD 1 develops ESKF at an earlier stage
what is the most common extral renal feature of ADPKD?
hepatic cysts
what is the management of ADPKD before renal failure?
hypertension control
hydration
proteinuria control
control cyst haemorrhage/infection
what drug treatment can be used to reduce cyst volume and progression in ADPKD?
tolvaptan
what is the management of ADPKD after renal failure?
dialysis
transplant
what type of inheritance is Alport’s syndrome?
X linked
what type of collagen is affected in Alports syndrome?
type IV collagen
what does alports syndrome cause?
hereditary nephritis
what is the characteristic feature of alport’s syndrome?
haematuria
what confers bad prognosis in alport’s syndrome?
proteinuria
what are the 3 main extra renal manifestations of alports syndrome?
sensorineural deafness
ocular lens defect (anterior displacement)
dysphagia
what is the treatment of alports syndrome?
BP control
proteinuria control
dialysis
transplantation
what is seen on renal biopsy of alports disease?
variable thickness glomerular BM
what is the inheritance of anderson fabrys disease?
X linked
what causes anderson fabrys disease?
inborn error of metabolism
deficiency of a-galactosidase A
what is seen on the skin of patients with anderson fabrys disease?
angiokeratomas
what is the treatment of anderson fabrys disease?
fabryzyme (enzyme replacement)
management of complications
what is the inheritance of medullary cystic kidney?
autosomal dominant
where are cysts fund in medullary cystic kidney?
corticomedullary junction or medulla
what is the gross appearance of medullary cystic kidney?
normal or small kidneys
what is the treatment of medullary cystic kidney disease?
renal transplant
what hapens to the collecting ducts in medullary sponge kidney?
become dilated
how do you diagnose medullary sponge kidney?
excretion urography
what is seen in the cysts within medullary sponge disease?
calculi
what is the inheritance of medullary sponge kidney?
sporadic
