path kidney and the collecting system - formatted Flashcards
- 14.APRIL02 CT guided biopsy of an atypical non-functional kidney, shows “ replacement of the normal architecture by numerous foamy macrophages intermingled with plasma cell, lymphocytes, polymorphonuclear leukocytes and occasional giant cells. The latter are not of the Langerhan’s type,” This suggests:
- Renal Tuberculosis
- Renal cell carcinoma
- Xanthogranulomatous pyelonephritis
- Oncocytoma
- Post obstructive atrophy
- Xanthogranulomatous pyelonephritis
• Xanthogranulomatous pyelonephritis is an unusual and relatively rare form of chronic pyelonephritis characterized by accumulation of foamy macrophages intermingled with plasma cells, lymphocytes, polymorphonuclear leukocytes, and occasional giant cells.
• Often associated with Proteus infections and obstruction, the lesions sometimes produce large, yellowish orange nodules that may be confused with renal cell carcinoma
- 13.APRIL02 A 50-year-old diabetic woman has papillary necrosis on IVP. Which of the following pathological features would most favour analgesic nephropathy over diabetic nephropathy?
- Duration of diabetes of less than 15 years
- The absence of calcification
- Less than 5 papillae affected
- Lesions of differing ages
- Associated accelerated atherosclerosis
- Lesions of differing ages
• Kidneys either normal or slightly size with irregular outline due to cortical atrophy overlying necrotic papillae
• Papillae in various stages of necrosis with calcification, fragmentation, sloughing (cf diabetic papillary necrosis where all papillae are at same stage of necrosis)
wji - table in robbins:
diabetes takes 10yrs calcification rare, 80% get infected, affects several papillae all at same stage
analgesic takes >5yrs, 25% infection, frequently calcifies and affects almost all papillae with different stages of necrosis seen
obstruction calcifies, sickle cell doesnt
diabetes, analgesia more common in women, obstruction in men, sickle cell equal
sickle cell affects a few papillae
- 14.02.65 The features of analgesic nephropathy do not include ?
- Renal artery stenosis
- Renal calculi
- Increased urinary Tamm-Horsfall protein
- Nephritis
- TCC
- Renal artery stenosis
- 14.02.65 The features of analgesic nephropathy do not include ? Rob 530
- Renal artery stenosis
- Renal calculi (Nephrocalcinosis & stones 20 acquired renal tubular acidosis)
- Increased urinary Tamm-Horsfall protein (Ischaemic ATN)
- Nephritis (Papillary necrosis occurs first 20 tubulointerstitial nephritis)
- TCC
• Tamm-Horsfall mucoprotein is the basic matrix of all renal casts
o it originates from tubular epithelial cells and is always present in the urine.
o When the casts contain only mucoproteins, they are called hyaline casts and may not have any pathologic significance.
o Hyaline casts may be seen in the urine after exercise or heat exposure, but may also be observed in pyelonephritis or chronic renal disease
• Tamm-Horsfall protein = urinary glycoprotein
o Seen in multiple myeloma
o Ischaemic ATN
• Papillae in various stages of necrosis with calcification, fragmentation, sloughing (cf diabetic papillary necrosis where all papillae are at same stage of necrosis)
- 14.03.60 40 year old patient has staghorn calculus not present previous year, LEAST LIKELY cause is ?
- Hyperuricaemia
- Crohns Disease
- Hypercalcuria
- Hyperoxaluria
- Recurrent urinary tract infections
- Hyperuricaemia
• staghorn calculi
o The majority of staghorn calculi are infection stones.
o They may start as matrix calculi composed of a glycoprotein cast within the collecting system, upon which triple phosphate infection stones are deposited
o Most staghorn stones are composed of struvite
o Other stone materials less often encountered that can also assume a staghorn configuration include cystine, calcium oxalate monohydrate, and uric acid
• hyperuricemia
o uric acid stones are present in 22% of patients with gout and 42% of those with secondary hyperuricemia
o causes urine to be acidic(c.f. Infection by urea-splitting organisms – Proteus and Staph convert urea to ammonia alkaline urine with precipitation of magnesium ammonium phosphate salts )
• crohns frequently causes oxalate stones, which may cause staghorn type calculi
- 14.03.61 Which is NOT a type of renal cell cancer ?
- Clear
- Papillary
- Collecting duct
- Chromophobe
- Small cell
- Small cell
- 14.02.66 Features of Clear cell renal cell Ca include ? Rob p539
- commonest at lower pole
- tan/white tumour macroscopically
- tan/white tumour macroscopically (yellow-gray-white in colour)
In general RCC is more common in the renal poles, but can occur anywhere, most commonly the poles of which the upper pole is most common.
Clear cell - most common - characterized by abundant clear cytoplasm. Most commonly sporadic, but is the type seen in VHL (sporadic also has mutations in VHL tumor suppressor gene).
Robbins pg953 RY
- 14.03.62 Which is TRUE? Rob p541
- Intestinal cystitis/ Hunner Ulcer is associated with SLE
- Malokplakia is metaplasia secondary to chronic infection
- 5% of bladder tumours are adenocarcinoma
- 5 year survival for grade I tumours is 75%
- Sessile bladder tumour – 1/3 of low grade, 80% of high grade TCC
- Intestinal cystitis/ Hunner Ulcer is associated with SLE
*LW: agree it can be, unknown etiology, but considerd auto immune origin as associated with SLE. - 14.03.62 Which is TRUE? Rob p541
- Intestinal cystitis/ Hunner Ulcer is associated with SLE: TRUE
- Malakoplakia is metaplasia secondary to chronic infection: FALSE, not metaplasia, but related to chronic infection
**SCS: Dahnert. uncommon chronic inflammatory response to gram neg infection. E coli. Submucosal histiocytic granuloma containing Foamy macrophages. - 5% of bladder tumours are adenocarcinoma: FALSE; rare as in less than 5% (rare )
- 5 year survival for grade I tumours is 75%: FALSE (99%)
- Sessile bladder tumour – 1/3 of low grade, 80% of high grade TCC: FALSE (low grade tumours are almost always papillary)
• Malakoplakia is caused by defects in phagocytic or degradative functions of histiocytes in response to gram negative coliforms; laminated mineralized concretions known as Michaelis-Gutmann bodies are typically present forming an inflammatory plaque but no metaplasia) (leukoplakia = keratinizing squamous metaplasia / dysplasia) Cystitis Glandularis and Cystitis Cystica = intestinal metaplasia).
- 14.02.46 Features of Bladder tumours do NOT include:
- 70% Solitary
- low grade lesions tend to be flat
- Transitional cell in 95%
- low grade lesions tend to be flat (low grade tumours are almost always papillary)
**LJS - disagree with 70% solitary - Rob says often multiple (field defect)
Precursor lesions can be papillary (ranging from papilloma to high grade papillary urothelial carcinoma) or CIS (flat). Terminology around precursor lesions appears to have changed btw editions of Rob - ?old question
- 14.02.46 Features of Bladder tumours do NOT include:
- 70% solitary (70% confined to bladder at diagnosis lateral or posterior bladder walls)
- low grade lesions tend to be flat (low grade tumours are almost always papillary)
- Transitional cell in 95% (90% of TCC’s)
- 15.02.69 Sclerosing retroperitonitis NOT due to:
- Crohns
- Lymphoma
- Migraine medication
- Sclerosing conditions elsewhere
*LW:
Incomplete recall with correct answer not included.
Robbins quotes following causes:
Drugs - ergot derivatives (MIGRAINE Med), B adrenergic blockers.
Adjacent inflamm conditions: vasculitis, diverticulitis, Crohn disease.
malignant disease - lymphoma, urinary tract carcinomas.
70% idiopathic.
Not sure about Crohn’s (not in retroperitoneum) unless ascending or descending bowel/duodenum involved. - not listed in radiopedia nor statdx
Causes of retroperitoneal fibrosis
- haemorrhage
- infection
- inflammation (pancreatitis, duodenitis, pyelonephritis)- IgG4
- trauma
- tumour (primary, met or lymphoma)
- toxin (antihypertensives, )
- radiation
List morphological appearance of bladder cancer
• The gross patterns of urothelial cell tumors vary from purely papillary to nodular or flat to mixed papillary and nodular.
• The tumors may also be invasive or noninvasive.
• The papillary lesions appear as red elevated excrescences varying in size from less than 1 cm in diameter to large masses up to 5 cm in diameter.
• Multicentric origins may produce separate tumors.
• As noted, the histologic changes encompass a spectrum from benign papilloma to highly aggressive anaplastic cancers.
• Overall, about half of bladder cancers are high-grade lesions.
• Most arise from the lateral or posterior walls at the bladder base.
• Papillomas and low-grade lesions are almost always papillary.
• Higher grades may be flat or papillary.
• Many high-grade III lesions may be fungating, necrotic, sometimes ulcerative tumors that have unmistakably invaded deeply .
• With the higher grade neoplasms, in areas of the bladder devoid of tumor, there may frequently be areas of mucosal hyperplasia, dysplasia, or carcinoma in situ.
• In most analyses, less than 10% of low-grade cancers invade, but as many as 80% of high-grade transitional cell carcinomas are invasive.
• Aggressive tumors may extend only into the bladder wall, but the more advanced stages invade the adjacent prostate, seminal vesicles, ureters, and retroperitoneum, and some produce fistulous communications to the vagina or rectum.
• About 40% of these deeply invasive tumors metastasize to regional lymph nodes.
• Hematogenous dissemination, principally to the liver, lungs, and bone marrow, generally occurs late, and only with highly anaplastic tumors
- Sep03.37 Focal renal infarct, least likely?
- Atherosclerosis
- Mitral valve regurgitation
- PAN
- HUS
- Endocarditis
*LW:
Preferred answer is Mitral Regurgiation.
4. HUS (Acute cortical necrosis not focal renal infarct)
*LW: Mitral valve regurgitation by itself would not cause renal infarction, while HUS will cause microthrombi within renal vasculature and acute cortical necrosis, thus is more likely than isolated MR. Hence preferred answer is MR.
**SCS: MR can lead to AF, and thus emboli. A bit indirect. But I still would go with MR as BEST answer.
- Sep03.37 Focal renal infarct, least likely: ** check this **
- Atherosclerosis (thrombosis in advanced atherosclerosis)
- Mitral valve regurgitation
- PAN (acute vasculitis of polyarteritis nodosa)
- HUS (Acute cortical necrosis not focal renal infarct)
- Endocarditis (vegetative endocarditis)
• Most due to embolism
• Major source of emboli = mural thrombi from L atrium and vent. from AMI
• Less common sources
o vegetative endocarditis
o thrombosis in aortic aneurysms and aortic atherosclerosis
• Rare causes
o thrombosis in advanced atherosclerosis
o acute vasculitis of polyarteritis nodosa
• On gross examination, the kidneys are swollen and pale; many fleabite hemorrhages are on the surface.
• In HUS and TTP, the platelet and fibrin microthrombi within the renal microvasculature are accompanied by thrombocytopenia and a microangiopathic hemolytic anemia.
• Vasculitis is usually absent.
- Sep03.56 CRF secondary to Goodpastures on haemodialysis presents with collapse with haematuria. What is the most likely cause ?
- Renal cysts
- Stones
- Obstruction/ hydronephrosis
*LW: Robins states dialysis associated renal cystic disease can bleed causing haematuria. Hence renal cysts is my preferred option. Above instances can cause renal failure and haematuria, but acute presentation with hypotension favours a cyst rupture.
• The renal manifestations of all forms include hematuria with red cell casts in the urine, moderate proteinuria occasionally reaching the nephrotic range, and variable hypertension and edema.
• In Goodpasture syndrome, the course may be dominated by recurrent hemoptysis or even life-threatening pulmonary hemorrhage.
• Serum analyses for anti-GBM, antinuclear antibodies, and ANCA are helpful in the diagnosis of specific subtypes.
• Although milder forms of glomerular injury may subside, the renal involvement is usually progressive during a matter of weeks, culminating in severe oliguria.
• Recovery of renal function may follow early intensive plasmapheresis (plasma exchange) combined with steroids and cytotoxic agents in Goodpasture syndrome.
• This therapy appears to reverse both pulmonary hemorrhage and renal failure. Other forms of RPGN also respond well to steroids and cytotoxic agents. Despite therapy, patients may eventually require chronic dialysis or transplantation.
• Most cases begin clinically with respiratory symptoms, principally hemoptysis, and radiographic evidence of focal pulmonary consolidations.
• Soon, manifestations of glomerulonephritis appear, leading to rapidly progressive renal failure.
• The common cause of death is uremia (but not sudden collapse???)
• Most cases occur in the teens or twenties, and there is a preponderance among men.
• The kidneys reveal the characteristic findings of focal proliferative glomerulonephritis in the early cases, or crescentic glomerulonephritis in patients with rapidly progressive glomerulonephritis.
• Linear deposits of immunoglobulins and complement are also seen by immunofluorescence studies along the glomerular basement membranes, similar to those in the alveolar septa
- Sep03.60 Magnesium ammonium phosphate stones
- UTI – urea transformed into ammonia
- Nidus
- UTI – urea transformed into ammonia
• Magnesium ammonium phosphate stones are formed largely after infections by urea-splitting bacteria (e.g., Proteus and some staphylococci), which convert urea to ammonia.
o The resultant alkaline urine causes the precipitation of magnesium ammonium phosphate salts.
o These form some of the largest stones, as the amounts of urea excreted normally are huge.
o Indeed, so-called staghorn calculi are almost always associated with infection.
• Magnesium ammonium phosphate hexahydrate (struvite) calculi are associated with an alkaline urine and usually are accompanied by, and are the result of, a urea-splitting bacillary urinary tract infection, usually with Proteus mirabilis.
• Pure struvite stones are rare and of relatively low radiopacity but often are laminated with more dense calcium salts, usually calcium phosphate, less often calcium carbonate, resulting in a magnesium-ammonium-calcium phosphate or triple-phosphate stone.
• These laminations suggest that recurrent infections and frequent changes in urinary pH have occurred in the chemical milieu in which the calculus formed.
• Triple-phosphate stones usually form a cast of the renal pelvis and, by direct extension, one or more infundibula and calyces.
• Characteristically, the peripheral components of an infection-related staghorn calculus are directly connected extensions of the renal pelvis component
• Struvite and apatite, occurring either separately or admixed as a triple-phosphate stone, account for 5% to 30% of upper tract calculi.
• In combination, they are the most common constituents of a staghorn calculus.
- Sep03.66 Fatty lesion next to effaced kidney. Liposarcoma? Which of the following is most relevant?
- Tuberous sclerosis
- Previous radiotherapy
- Previous radiotherapy
• AML - tuberous sclerosis
• Liposarcomas are one of the most common sarcomas of adulthood and appear in the forties to sixties
o they are uncommon in children.
• The cause of most soft tissue tumors is unknown.
• There are documented associations, however, between radiation therapy and rare instances in which chemical burns, heat burns, or trauma were associated with subsequent development of a sarcoma
• They usually arise in the deep soft tissues of the proximal extremities and retroperitoneum and are notorious for developing into large tumors.
• Histologically, liposarcomas can be divided into the following variants.
o well-differentiated
o myxoid
o round cell
o pleomorphic
• The cells in well-differentiated liposarcomas are readily recognized as lipocytes.
• In the other variants, most of the tumor cells are not obviously adipogenic, but some cells indicative of fatty differentiation are almost always present.
• They are known as lipoblasts, which mimic fetal fat cells
• They contain round cytoplasmic vacuoles of lipid that scallop the nucleus.
• The myxoid and round cell variant of liposarcoma has a t(12;16) chromosomal abnormality in most cases.
• well-differentiated variant is relatively indolent,
• the myxoid type is intermediate in its malignant behavior
• round cell and pleomorphic variants usually are aggressive and frequently metastasize.
• All types of liposarcoma recur locally and often repeatedly unless adequately excised.
• 2/3rd’s of retroperitoneal liposarcomas are low-grade, well differentiated neoplasms.
• Most well-differentiated tumors contain mature adipose tissue and only occasional atypical cells.
• These neoplasms are often referred to as well-differentiated, lipogenic, or lipoma-like liposarcomas.
• Sclerosing liposarcomas are less commonly encountered well-differentiated tumors.
• Sclerosing liposarcomas contain an extensive fibrous tissue stroma.
• In one recent series, the sclerosing components of several well-differentiated liposarcomas (that also contained lipogenic areas) were of similar attenuation or signal intensity to muscle on computed tomography (CT) and magnetic resonance (MR) imaging examinations and did not contain any recognizable fat.
• Renal manifestations of TS
o Renal failure in severe cases (5%)
o Hypertension
o Angiomyolipomas – multiple, large
5% have fat elements that can be detected only by microscopy
AML associated with tuberous sclerosis in 20%
AML in 80% of patients with tuberous sclerosis (80% TS have AML, 20% AML have TS)
o Cysts
o Renal cell carcinoma (3%), bilateral in 40%
- Sep03.82 5 year survival for RCC with extension into the renal vein
- 90%
- 70%
- 30%
- 10%
*LW: Renal vein extension is Stage 3 disease, with radiopedia stating stage 3 disease = 30% 5YS.
Need to check in Robbins
ADB–> robbins
“the average 5 year survival rate of persons with renal cell carcinoma is 70% and as high as 100% in the absence of distant metastasis. With renal vein invasion or extension into the perinephric fat, the figure is reduced to approximately 60%.”
seems high compared to elsewhere
- 20%•
• 5 year survival in patients with renal vein invasion or extension into the perinephric fat is 15-20% may be bad recall
• Tumour stage + histologic grade are the most important prognosticators
• Papillary carcinomas have better prognosis
• Overall 45% 5YS
• 70% 5YS without metastases
• 15-20% 5YS if perinephric fat or renal vein invasion
- Sep03.83 Wilms tumour, age at presentation
- 2-5 years
• Wilms tumour is usually diagnosed between the ages of 2 and 5 years.
- Sep03.86 Incidence of polycystic kidneys
- 1:1000
- 1:10000
- 1:10
- none of the above
- 1:1000• APCKD : 1 in 400-1000
- PATH2004 Which of the following are all recognised bladder carcinoma morphological patterns:
- Papillary, ulcerative, diffuse, cystic, infiltrative
- Papillary, tubular, sessile, excavative
- Papillary, tubular, cystic, flat
- Flat, excavative, polypoid
- Flat, papillary-invasive, papilloma-papillary
- Flat, papillary-invasive, papilloma-papillary
- PATH2004 Focal renal infarct, least likely:
- Aortic atherosclerosis
- Mitral valve regurgitation
- Post AMI
- PAN
- Adult Haemolytic Uremic Syndrome
- Candida endocarditis
- MR
*LW: Mitral valve regurgitation by itself would not cause renal infarction, while HUS will cause microthrombi within renal vasculature, thus is more likely than isolated MR. Hence preferred answer is MR.
- PATH2004 Regarding renal cell carcinoma. Which of the following is the LEAST CORRECT:
- Strong association with Von-Hippel-Lindau syndrome
- Clear cell carcinoma is the most common (70-80%)
- Increased risk of renal cell carcinoma in chronic renal failure
- Most frequent presentation is flank pain or discomfort
- Hereditary tumours are more frequently bilateral and multifocal
- Most frequent presentation is flank pain or discomfort
• The three classic diagnostic features of costovertebral pain, palpable mass, and hematuria unfortunately appear in only 10% of cases. The most reliable of the three is hematuria, but it is usually intermittent and may be microscopic; thus, the tumor may remain silent until it attains a large size.
Wji: pathoma- 60% have haematuria
- PATH2004 Which of the following is not derived from renal tubules?
- Papillary cell carcinoma
- clear cell carcinoma
- oncocytoma
- renal tubular adenoma
- angiomyolipoma
- angiomyolipoma
- PATH2004 Regarding prostatic carcinoma. Which of the following is the least correct:
- 70-80% of males between the ages of 70-80 have foci
- Histologically sheets or cords of cells in dense fibrous sroma
- 70% arise in the peripheral zone
- Local invasion into the seminal vesicles, bladder and ureter
- Carcinoma of the prostate is less common in Asia compared with Australia and New Zealand
- Histologically sheets or cords of cells in dense fibrous sroma - FALSE
• Histologically, most lesions are adenocarcinomas that produce well-defined, readily demonstrable gland patterns.
- PATH2004 Following NOT associated with increased risk of testicular malignancy
- Cryptorchidism
- Testicular feminisation
- Testicular microlithiasis
- Klinefelters syndrome
- TB of the testis
Testicular microlithiasis
- PATH2004 scrotal US – correct
- varicocele usu right sided
- hydroceles not associated with ca
- cause of hydrocele may be unknown
- cryptorchidism may be associated with inguinal hernia
*LW:
Variocele usually left sided,
hydroceles not associated with cancer,
cause of hydrocele may be unknown, and cryptorchidism may be associated with inguinal hernia. Maybe incorrect recall, should read which is incorrect?
- cause of hydrocele may be unknown this is true as well (SG)
- cryptorchidism may be associated with inguinal hernia
- PATH2004 aFP increased in
- yolk sac tumours
YES
• AFP synthesized by the fetal gut, liver cells, and yolk sac
• One year after birth, the serum levels of AFP fall to less than 16 ng/ml,
• HCG is normally synthesized and secreted by the placental syncytiotrophoblast.
• The beta subunit of HCG has unique sequences not shared with other human glycoprotein hormones, and therefore the detection of HCG in the serum is based on a radioimmunoassay using antibodies to its beta chain.
• Yolk sac tumors produce AFP exclusively, and choriocarcinomas elaborate only HCG.
• Either or both of these markers are elevated in more than 80% of patients with NSGCT at the time of diagnosis.
- In epididymitis, which is most likely to have orchitis without epididymitis :
- Syphilis
- TB
- Pseudomonas
- E. Coli
- Gonococcus
*AJL has changed the question around.
Answer is now syphilis.
WILD - From personal experience can say this is true, causes isolated orchitis, quite painful.
- In epididymitis, which is most likely to have orchitis without epididymitis :
- Syphilis (testis first if acquired)
- TB (Begins in epididymis and spreads to testis)
- Pseudomanas and this one
- E. Coli and this one
- Gonococcus and this one
Robbins p973
• It is classically taught that, of the three major specific inflammatory states, gonorrhea and tuberculosis almost invariably arise in the epididymis, whereas syphilis affects first the testis.
• In sexually active men younger than age 35 years, the sexually transmitted pathogens Chlamydia trachomatis and Neisseria gonorrhoeae are the most frequent culprits.
• In men older than age 35, the common urinary tract pathogens, such as Escherichia coli and Pseudomonas, are responsible for most infections.
Mumps is stated infection which primarily affects testes; Primary orchitis is rare and caused by mumps (usually bilateral).
syphilis Saj
- 40 yo has staghorn calculus not present previous year, least likely cause is:
- Hyperuricemia
- Crohns disease
- Hyper calciuria
- Hyperoxaluria
- Recurrent urinary tract infections
- Hyperuricemia !!!!
- Regarding testicular tumours
- Choriocarcinoma are large haemorrhagic masses
- Spermatocystic seminoma occurs in 15-40 year olds
- Seminoma is most common homogeneous testicular mass
- Mixed tumours are rare in adults (<5%)
- Teratoma is the second commonest adult tumour
- Seminoma is most common homogenous testicular mass
- Regarding testicular tumours
- Choriocarcinoma are large haemorrhagic masses (Usually small lesions - < 5cm)
- Spermatologic seminoma occurs in 15-40 year olds (> 65 years)
- Seminoma is most common homogenous testicular mass
- Mixed tumours are rare in adults (<5%) (60%)
- teratoma second commonest adult tumour (Adults: Pure forms rare (2 – 3%), mixed more common (45%) ; Children: Pure forms common (2nd to yolk sac tumours))
- Nodular hyperplasia of prostate which is not true
- 20% in men 40 years old
- Occurs in periurethral and transitional zones
- Demonstrates regions of infarction and metaplasia
- Only 50% are symptomatic
- Has 2-3 x increased risk of carcinoma
- has 2-3X time have increase risk of carcinoma
- Nodular hyperplasia of prostate which is not true
- micro seen in 20% in men <40 years old (20% of 40yo men)
- occurs in periurethral and transitional zones (Almost exclusively in periurethral and transitional zones of gland extending from bladder neck to verumontanum)
- demonstate regions of infarction and metaplasia (Frequently, foci of squamous metaplasia and small areas of infarction)
- only 50% are symptatic
- has 2-3X time have increase risk of carcinoma
- 18.03.82 DEFINITION of Bowen disease ? define it
- A persistent progressive non-elevated red scaly or crusted plaque which is due to an intradermal carcinoma and is potentially malignant
SCS: dermnet. Intra epidermal SCC
RF: sun exposure, arsenic, radiation, HPV, immune suppression.
- HUS - ? most likely
- Following viral infection
- Kidneys may appear normal acutely
**LJS - typically follows bacterial infection (E.coli > shigella). Kidneys echogenic and enlarged due to renal impairment.
*LW; ongoing theme of approval. correct answer not recalled.
Previous work
1. Following viral infection
Hemolytic uremic syndrome (HUS) is a multisystem thrombotic microangiopathic disease characterized by the triad of renal failure, hemolytic anemia and thrombocytopenia. It is the most common cause of renal failure in infancy and childhood requiring dialysis. There are two forms of this syndrome:typical or D+ HUS: corresponds to >90% of all HUS, occurs in childhood and is caused by Shiga toxin-producing Escherichia coliatypical or D- HUS: can occur after infections, use of certain drugs, following other pathologies (e.g. malignancy), or, rarely, due to defective cobalamin metabolism 3This article is based on the features related to the typical HUS. HUS affects ~ 2:100,000 people worldwide 3. Clinical presentationIt is most commonly seen in young children.Usually, there is an interval of 2-12 days between the ingestion of contaminated food and the first day of diarrhea and abdominal pain. After 1-3 days the diarrhea becomes bloody (~90%). Fever is usually absent. PathologyHUS usually occurs (>90%) following a gastrointestinal infection with Shiga toxin-producing E. coli (STEC). Some cases are associated with medical conditions such as collagen vascular conditions like SLE, underlying malignancy, or medications such as cyclosporine, OCP, or 5 FU. Injury to the endothelium of the capillaries results in mechanical destruction of the RBCs. Kidneys can be the only organ involved; however involvement of the other organ like liver, pancreas, heart, intestine, muscles also possible
- Patient with previous Goodpastures, on dialysis. Gross hematuria and hypotensive. ?cause
- Prostate vol 25cc
- Renal cysts
- Renal calculi
- Amyloid
*LW: Favour renal cysts with rupture secondary to dialysis induced renal cystic disease, causing acute haematuira and hypotension.
**LJS - agree
Goodpasture syndrome, also referred as antiglomerular basement membrane (anti-GBM) antibody disease, is an autoimmune disease characterised by damage to the alveolar and renal glomerular basement membranes by a cytotoxic antibody. It is a type of pulmonary-renal syndrome.Goodpasture syndrome is defined by:- pulmonary haemorrhage- glomerulonephritis- circulating antiglomerular basement membrane antibodiesTreatment and prognosisThe overall prognosis is poor, although the use of immunosuppressive drugs and plasmapheresis has improved survival.Differential diagnosisOften radiograph and CT findings are indistinguishable from pulmonary oedema. Other causes of pulmonary haemorrhage should also be considered.
- Hypertensive patient most likely findings
- 2cm adrenal Ca
- 10cm adenoma
- Bilateral benign tumours
- Bilateral hyperplasia
- Adrenal atrophy secondary to sustained HTN
- Bilateral benign tumours (Adenomas are common, phaeos r bilat in 10%)
**LJS - or bilateral hyperplasia causing hyperaldosteronism and secondary HTN. 9th edition Rob has bilateral hyperplasia as most common cause Conn’s syndrome and most important cause of secondary HTN. This seems more likely than getting bilateral pheo?
*AJL agree
- Vascular 5cm renal mass found on investigation for RAS which was normal. ?Most likely
- Incidental RCC
- RCC as cause for hypertension
- Oncocytoma
- Need to check for urinary VMA before biopsy
- Incidental RCC - RCC 80-90% of all primary renal tumours
**LJS - could also be renin secreting RCC. RCC has common paraneoplastic syndromes incl renin secretion
*LW:
Although agree with LJS regarding could be paraneoplastic syndrome with renin secretion, renin is just one of the multiple ectopic hormones that can be produced, and para neoplastic syndromes are not always associated with RCC.
Conversely, HTN is a risk factor for developing RCC.
RCC is also becoming increasingly diagnosed an an incidental finding.
Chicken and egg argument follows.
Ideally further info required…
–> if relatively acute onset of HTN, then I would favour RCC causing HTN with renin production.
–> if non acute HTN, then i favour incidental RCC.
WJI: i would chose 2. Reason is 25% RCC have paraneoplastic syndrome (not all renin/HTN but still prob >1% chance) whereas prevalence of RCC if you just screen asymptomatic adults would be below 1% (2-3% all cancers are RCC, 1/3 people are not walking around with cancer). Also if they are having RAS workup they have refractory or atypical HTN and we know RAS isnt the cause.
- 20year old with 6cm testicular tumour and raised bHCG. ?Most likely
- Choriocarcinoma
- Spermatocytic seminoma
- Yolk Sac tumour
- Mixed teratoma
- Seminoma
*LW: Seminoma are common, and can produce hCG, and be large masses, hence my favoured answer
**AJL: Robbins says Chorio is ‘rarely above 5cm’ therefore Seminoma is the best answer (agree with LW above) (tricky tricky examiners!)
- Choriocarcinoma - are usually small, so less likely.
- Spermatocytic seminoma - flase, elderly
- Yolk Sac tumour - pure form rare in adults
- Mixed teratoma -
- Seminoma - Robbins states approx 15% of seminomas have syncytiotrophoblastas and hence can have elevated bHCG.
Previous answer:
I prefer choriocarcinoma given- appropriate age- bHCG increase but they prefer
5. Seminoma (50% of testicular tumourss, correct age group, 5-10% have elevated B HCG);
- Elderley patient with retroperitoneal soft tissue mass – Which is most likely
- Rhabdomyosarcoma
- Should consider fibrosarcoma
- More likely to be malignant if retroperitoneal
- Very unlikely to be synovial sarcoma as it is extraarticular
- If has clear distinct margins it is unlikely to be a malignant tumour
- LW:
RadPrimer states 80% of retroperitoneal masses / tumours are malignant. Hence my preferred answer is 3.
Fibrosarcoma are usually in limbs.
**LJS - agree. Retroperitoneal fibrosarcoma is rare
Can get fibrosarcoma in various systems as malignant tumour of fibroblast origin (bone, breast, soft tissue, ovary etc). Still exists in 2015 WHO soft tissue tumour classification. Liposarcoma is most common retroperitoneal primary
Newborn with multiple small renal cysts. Mother, father and sister to be imaged. Which is most likely? (? Unilateral, ? bilateral)
a. Mother, father and sister have normal imaging
b. M & F normal, sister may be abnormal
c. Sibling may have it
4. Both parents may have it
b. M & F normal, sister may be abnormal(Depending on age of sister)
Wji: i think multiple small cysts is more likely arpkd than renal cystic dysplasia.
Assuming this b is probably the answer they are after but if we are saying both parents are normal then there is 1/4 chance of affected sister so it is “most likely” ie 75% chance sister is also normal.