Renal Patient Case Questions

Question 1

An 8 year old male is brought to your clinic by his mother. She has stated that he has had a fever for the past couple of days, and hasn’t been able to get up and around. He has had significant N/V. You find out that he has not been using the bathroom as much as he used too, and when he has, has noticed a red twinge to the urine. On PE, you notice a slight peri-orbital edema and a distressed child.

He has elevated BUN levels, and on IF you notice what kind of staining?

Filled with?

on EM you would see?

Answer 1

Granular Deposits

IgG, C3, IgM

SUBepithelial Humps

Question 2

An 8 year old male is brought to your clinic by his mother. She has stated that he has had a fever for the past couple of days, and hasn’t been able to get up and around. He has had significant N/V. You find out that he has not been using the bathroom as much as he used too, and when he has, has noticed a red twinge to the urine. On PE, you notice a slight peri-orbital edema and a distressed child.

What Ag would be causing this?

And where do these Ag’s plant in the kidney?

Answer 2

SpeB (Streptococcal Pyogenic Exotoxin B)

Along the GBM, and Mesangium (forming those SUBepithelial humps)

Question 3

An 8 year old male is brought to your clinic by his mother. She has stated that he has had a fever for the past couple of days, and hasn’t been able to get up and around. He has had significant N/V. You find out that he has not been using the bathroom as much as he used too, and when he has, has noticed a red twinge to the urine. On PE, you notice a slight peri-orbital edema and a distressed child.

What would be expected upon Histological Section?

How would this be spread throughout the kidney?

Answer 3

Hypercellular Glomeruli, with ENALRGED mesangial and endothelial cells, and Red Cell Casts

It would be at all Lobules of all Glomeruli (Global and Diffuse)

Question 4

An 8 year old male is brought to your clinic by his mother. She has stated that he has had a fever for the past couple of days, and hasn’t been able to get up and around. He has had significant N/V. You find out that he has not been using the bathroom as much as he used too, and when he has, has noticed a red twinge to the urine. On PE, you notice a slight peri-orbital edema and a distressed child.

What clinical manifestation is he presenting with?

What disease caused the glomeruli disease?

What is his Glomeruli Disease?

Answer 4

Nephritic Syndrome

Strep

Acute Proliferative GN

Question 5

An 8 year old male is brought to your clinic by his mother. She has stated that he has had a fever for the past couple of days, and hasn’t been able to get up and around. He has had significant N/V. You find out that he has not been using the bathroom as much as he used too, and when he has, has noticed a red twinge to the urine. On PE, you notice a slight peri-orbital edema and a distressed child.

How long will it take for him to recover?

If he doesnt recover what 2 things could he progress too?

If this patient had been a 25 year old what would his prognosis have been?

Answer 5

6-8 weeks

Can progress to RPGN (type II) or Chronic GN (both less than 1%)

Longer Recovery time, and more chances of progressing to RPGN and Chronic GN

Question 6

An 8 year old male is brought to your clinic by his mother. She has stated that he has had a fever for the past couple of days, and hasn’t been able to get up and around. He has had significant N/V. You find out that he has not been using the bathroom as much as he used too, and when he has, has noticed a red twinge to the urine. On PE, you notice a slight peri-orbital edema and a distressed child.

Upon further testing you find that child had a staph infection that caused his illness – What sort of immunoglobulins would be found now?

Answer 6

IgA deposits (staph) – (IgG is for Strep)

Question 7

An 8 year old male is brought to your clinic by his mother. She has stated that he has had a fever for the past couple of days, and hasn’t been able to get up and around. He has had significant N/V. You find out that he has not been using the bathroom as much as he used too, and when he has, has noticed a red twinge to the urine. On PE, you notice a slight peri-orbital edema and a distressed child.

What is the best sort of treatment/management option you can offer this little guy?

Answer 7

Tx: w/ Fluid and Electrolyte Management

Question 8

You have a 23 year old male who presents to your office with hematuria. He notes that he has delt with this for some time now. His BP is 150/92 and is taking Lisinopril and HCTZ. He notes that he has also been short of breath for a while now. When you take a urine sample you note numerous red cell casts in his urine and mild proteinuria (<3gm).

How would his IF appear?

Answer 8

Linear with IgG and C3; and Fibrin CRESCENTS

Question 9

You have a 23 year old male who presents to your office with hematuria. He notes that he has delt with this for some time now. His BP is 150/92 and is taking Lisinopril and HCTZ. He notes that he has also been short of breath for a while now. When you take a urine sample you note numerous red cell casts in his urine and mild proteinuria (<3gm).

What would we Diagnosis this man with?

What makes up the antigen?

What HLA do we associate this with?

Answer 9

Goodpasture Syndrome

The Goodpasture Ag –> is an ALPHA 3 chain within the noncollagenous regions of collagen Type IV

HLA-DRB1

Question 10

You have a 23 year old male who presents to your office with hematuria. He notes that he has delt with this for some time now. His BP is 150/92 and is taking Lisinopril and HCTZ. He notes that he has also been short of breath for a while now. When you take a urine sample you note numerous red cell casts in his urine and mild proteinuria (<3gm).

How would we treat this patient most effectively?

Answer 10

Plasmapheresis

Question 11

You have a 23 year old male who presents to your office with hematuria. He notes that he has delt with this for some time now. His BP is 150/92 and is taking Lisinopril and HCTZ. He notes that he has also been short of breath for a while now. When you take a urine sample you note numerous red cell casts in his urine and mild proteinuria (<3gm).

What type of disease process does this man have?

Answer 11

he has RPGN (type I – Anti GBM ab)

Question 12

You have a 32 year old woman who shows up to your clinic with hematuria – you diagnose her with a Rapidly Progressive Glomerulonephritis. You see granular pattern to staining and cell proliferation and crescent formation. You note many immune complexes.

Which form of RPGN does she have?

How do we treat this form?

What disease(s) could be causing this RPGN?

Answer 12

She has Type 2 (immune complex deposition)

we treat her underlying disease

Could be a Post Strep GN, or Lupus Nephritis, IgA Nephropathy, of even HS Purpura

Question 13

You have a 32 year old woman who shows up to your clinic with hematuria – you diagnose her with a Rapidly Progressive Glomerulonephritis. You see granular pattern to staining and cell proliferation and crescent formation. You note many immune complexes.

If she were noted to not have had immune complexes, and she was noted to have had some dyspnea and upper respiratory complaints – what would we look for?

What would the possible diagnosis be now? (list all)

Answer 13

ANCA’s

Wegner’s (most likely), Microscopic Poly, etc

Question 14

You have a 32 year old woman who shows up to your clinic with hematuria – you diagnose her with a Rapidly Progressive Glomerulonephritis. You see granular pattern to staining and cell proliferation and crescent formation. You note many immune complexes.

How would you characterize her clinical symptoms?

Answer 14

Nephritic Syndrome

Question 15

A 45 year old male presents to your clinic with edema and complains of fatigue. He says that he noticed a slight red color to his urine: and upon further work up you notice that he has massive protein in his urine (3.5 gm).

You suspect this to be idiopathic, what would the autoantigen be?

If you suspected a systemic disease what would need to look for?

Answer 15

Phopholipase A2 Receptor

Malignant Tumors, SLE, Bacterial Infections, H Thyroiditis, Drugs that cause it

Question 16

A 45 year old male presents to your clinic with edema and complains of fatigue. He says that he noticed a slight red color to his urine: and upon further work up you notice that he has massive protein in his urine (3.5 gm).

What sort of clinical manifestation is showing?

Will this patient respond to corticosteroids?

Answer 16

Nephrotic Syndrome

NO, she will continue to have sclerosis of the glomerulus

Question 17

A 45 year old male presents to your clinic with edema and complains of fatigue. He says that he noticed a slight red color to his urine: and upon further work up you notice that he has massive protein in his urine (3.5 gm).

What would you expect to see upon Light Microscopy?

Answer 17

Uniform, Diffuse thickening of the capillary wall -- Silver Staining Spikes of matrix that project from the BM toward the urinary spaces

Question 18

A 45 year old male presents to your clinic with edema and complains of fatigue. He says that he noticed a slight red color to his urine: and upon further work up you notice that he has massive protein in his urine (3.5 gm).

What would you expect to see upon IF?

What would you see on EM?

Answer 18

A Granular “Lumpy Bumpy” IgG deposits

Epimembranous Deposits

Question 19

A 45 year old male presents to your clinic with edema and complains of fatigue. He says that he noticed a slight red color to his urine: and upon further work up you notice that he has massive protein in his urine (3.5 gm).

What is the antigen causing complex formation?

What allele is associated with it?

Answer 19

PLA2R Ag

HLADQ1

Question 20

A 45 year old male presents to your clinic with edema and complains of fatigue. He says that he noticed a slight red color to his urine: and upon further work up you notice that he has massive protein in his urine (3.5 gm).

What would the diagnosis be?

What physiologically is happening in this patient?

Answer 20

Membranous Nephropathy (Glomerulopathy)

He is having excessive complement activation (MAC) and IgG4

Question 21

A 45 year old male presents to your clinic with edema and complains of fatigue. He says that he noticed a slight red color to his urine: and upon further work up you notice that he has massive protein in his urine (3.5 gm).

The patient later has ESRD, and undergoes a transplant – What is the outcome?

Answer 21

He has a high chance of of Reccurence of symptoms.

Question 22

A 45 year old male presents to your clinic with edema and complains of fatigue. He says that he noticed a slight red color to his urine: and upon further work up you notice that he has massive protein in his urine (3.5 gm).

Because of his loss of protein what vital sign would you expect to be elevated?

Answer 22

You would expect him to have slightly elevated BP (maybe has mild HTN)

Question 23

You have an adorable little 4 year old girl who arrives at your clinic. Her mother says she recently got some of her immunizations and now has some fluid build up. She is noted to have some eczema. Urine tests confirm your diagnoses.

What is the quickest way to help this patient, and get a diagnosis?

Answer 23

Give her some Corticosteroids to see if she improves. (Minimal Change Dz)

Question 24

You have an adorable little 4 year old girl who arrives at your clinic. Her mother says she recently got some of her immunizations and now has some fluid build up. She is noted to have some eczema. Urine tests confirm your diagnoses.

What would be expected from the Urine Tests?

Answer 24

SELECTIVE Proteinuria (Albumin)

Question 25

You have an adorable little 4 year old girl who arrives at your clinic. Her mother says she recently got some of her immunizations and now has some fluid build up. She is noted to have some eczema. Urine tests confirm your diagnoses.

On Light Microscopy you would most likely see?

On IF you would see?

On EM what would be the most striking appearance?

Answer 25

A very normal appearnce, but with Lipids in the tubules.

Nothing

Loss of foot processes, and no deposits (confirms your IF)

Question 26

You have an adorable little 4 year old girl who arrives at your clinic. Her mother says she recently got some of her immunizations and now has some fluid build up. She is noted to have some eczema. Urine tests confirm your diagnoses.

What other clinical pearl of info would have helped you notice the disease process?

What does this ADORABLE little girl have?

Answer 26

If she were noted to have had a recent RESPIRATORY INFECTION (immunization was given)

MINIMAL CHANGE DZ

Question 27

You have an adorable little 4 year old girl who arrives at your clinic. Her mother says she recently got some of her immunizations and now has some fluid build up. She is noted to have some eczema. Urine tests confirm your diagnoses.

What is this Disease also assocaited with in Adults, Neoplasm wise?

Answer 27

Hodgkins Lymphoma

Question 28

You have a Hispanic 42 year of woman who comes into your clinic complaining of hematuria. You notice that she is on Atorvastatin, that she takes for her HTN. She says that she was recently taking corticosteroids given to her by an ER doc – but it hasnt helped. Upon analysis of her urine you find that she has protein in the urine (1.5 gm), and you calculate her GFR (which is low).

How do you think her proteinuria would be categorized?

Answer 28

NONSELECTIVE, and nonnephrotic (below 3.5gm)

(recall MCD has selective proteinuria)

Question 29

You have a Hispanic 42 year of woman who comes into your clinic complaining of hematuria. You notice that she is on Atorvastatin, that she takes for her HTN. She says that she was recently taking corticosteroids given to her by an ER doc – but it hasnt helped. Upon analysis of her urine you find that she has protein in the urine (1.5 gm), and you calculate her GFR (which is low).

You categorize her symptoms as Nephrotic like, with Non-Nephrotic like Proteinuria – what systemic diseases do you consider knowing she has a nephrotic syndrome dz?

Answer 29

SLE, Diabetes, Hep C, HIV Neph

(also Nephrotic Syndrome is associated with Renal Vein Thrombosis)

Question 30

You have a Hispanic 42 year of woman who comes into your clinic complaining of hematuria. You notice that she is on Atorvastatin, that she takes for her HTN. She says that she was recently taking corticosteroids given to her by an ER doc – but it hasnt helped. Upon analysis of her urine you find that she has protein in the urine (1.5 gm), and you calculate her GFR (which is low).

What would you expect her prognosis to be?

Answer 30

Progression to CKD, and a 50% chance of going to ESRD (within 10 years)

Question 31

You have a Hispanic 42 year of woman who comes into your clinic complaining of hematuria. You notice that she is on Atorvastatin, that she takes for her HTN. She says that she was recently taking corticosteroids given to her by an ER doc – but it hasnt helped. Upon analysis of her urine you find that she has protein in the urine (1.5 gm), and you calculate her GFR (which is low).

If you were to find out that she had HIV, what would be Diagnostic?

Answer 31

A Large Number of Tubuloreticular Inclusions in Endothelial Cells

(Collapsing Variant of FSGS)

Question 32

You have a Hispanic 42 year of woman who comes into your clinic complaining of hematuria. You notice that she is on Atorvastatin, that she takes for her HTN. She says that she was recently taking corticosteroids given to her by an ER doc – but it hasnt helped. Upon analysis of her urine you find that she has protein in the urine (1.5 gm), and you calculate her GFR (which is low).

Upon LM what would you expect to see?

Answer 32

Focal and Segmental sclerosis and hyalinosis

Question 33

You have a Hispanic 42 year of woman who comes into your clinic complaining of hematuria. You notice that she is on Atorvastatin, that she takes for her HTN. She says that she was recently taking corticosteroids given to her by an ER doc – but it hasnt helped. Upon analysis of her urine you find that she has protein in the urine (1.5 gm), and you calculate her GFR (which is low).

You are thinking she has a FSGS – how would you describe the cause of her nephrotic syndrome?

Answer 33

She has a disorder of her podocytes –> causing problems with her slit diaphragm –> causing protein loss –> causing nephrotic syndrome

Question 34

You have a Hispanic 42 year of woman who comes into your clinic complaining of hematuria. You notice that she is on Atorvastatin, that she takes for her HTN. She says that she was recently taking corticosteroids given to her by an ER doc – but it hasnt helped. Upon analysis of her urine you find that she has protein in the urine (1.5 gm), and you calculate her GFR (which is low).

You consider the 4 Mutations that are assocaited with FSGS and want to quickly associate the main ones with each?

Answer 34

NPHS1 – Congenital Nephrotic Syndrom (Finnish Type)

NPHS2 – Childhood Steroid Resistant Nephrotic Syndrome

Alpha Actin 4 –> AD FSGS (Childhood onset)

TRPC6 – Adult Onset FSGS

Question 35

You have a Hispanic 42 year of woman who comes into your clinic complaining of hematuria. You notice that she is on Atorvastatin, that she takes for her HTN. She says that she was recently taking corticosteroids given to her by an ER doc – but it hasnt helped. Upon analysis of her urine you find that she has protein in the urine (1.5 gm), and you calculate her GFR (which is low).

Because I need to milk every question I can out of this stem, she tells you that she is in fact half African American . . . Her mother is from Nigeria, and her father is from Mexico . . . .(racist) . . . What gene on what chromsome is she now at risk for, and explain how it matters?

Answer 35

APOL1 (on Chrom 22)

This gene helps with resistance to Malaria –> but increases the risk of FSGS

Question 36

You have a Hispanic 42 year of woman who comes into your clinic complaining of hematuria. You notice that she is on Atorvastatin, that she takes for her HTN. She says that she was recently taking corticosteroids given to her by an ER doc – but it hasnt helped. Upon analysis of her urine you find that she has protein in the urine (1.5 gm), and you calculate her GFR (which is low).

Upon IF you would see?

On EM you would see?

Answer 36

Focal Deposists of IgM and C3

Fusion Foot Processes and Sclerosis

Question 37

You have a 24 year old man come into your clinic. Upon examination you note that the patient is presenting with gross hematuria and signifcant proteinuria (3.8 gm). He has a BP of 156/90 and has mild edema, oliguria, and renal insufficiency. His Tx was not helpful with Immunosuppresive/Steroids/ or Antiplatelet drugs

You notice a “Tram-Track” apperance to his LM with Leukocyte Infiltration, what would be expected upon EM?

What would be expected upon IF?

Answer 37

Subendothelial Deposists

IgG, C3, C1q, C4 –> Granular Deposits in the GB membrane

Question 38

You have a 24 year old man come into your clinic. Upon examination you note that the patient is presenting with gross hematuria and signifcant proteinuria (3.8 gm). He has a BP of 156/90 and has mild edema, oliguria, and renal insufficiency. His Tx was not helpful with Immunosuppresive/Steroids/ or Antiplatelet drugs

You notice a “Tram-Track” apperance to his LM with Leukocyte Infiltration, where else would you see patterns of proliferation?

Answer 38

Mesangial Regions, GBM Thickening, and Splitting

Question 39

You have a 24 year old man come into your clinic. Upon examination you note that the patient is presenting with gross hematuria and signifcant proteinuria (3.8 gm). He has a BP of 156/90 and has mild edema, oliguria, and renal insufficiency. His Tx was not helpful with Immunosuppresive/Steroids/ or Antiplatelet drugs

What clinical syndrome(s) is he presenting with?

What disease is he having?

What would the expected prognosis be?

Answer 39

Mixed Nephritic and Nephrotic Syndrome

Primary MPGN (Type 1)

Chronic Renal Failure within 10 years

Question 40

You have a 24 year old man come into your clinic. Upon examination you note that the patient is presenting with gross hematuria and signifcant proteinuria (3.8 gm). He has a BP of 156/90 and has mild edema, oliguria, and renal insufficiency. His Tx was not helpful with Immunosuppresive/Steroids/ or Antiplatelet drugs

If this patient were to as well have Antigenemia and was reported to have had an Autoimmune disease – what would be expected to change in our diagnosis?

Would this change your patients prognosis?

Answer 40

You would start to think this is a Immune Complex Desposition of Type 1 (Secondary MPGN)

(This could be associated with Hep C with Crygloulinemia, SLE Endocarditis, Malignancies)

NO! She would still be expected to have Chronic Renal Failure within 10 years

Question 41

You have a 10 year old girl that comes into your clinic. Upon evaulation she has hematuria and proteinuria. Her serum levels have low levels of some complement, and low levels of Factor B and Properdin. Her kidney function is impacted.

What specific complement proteins are affected?

What is happening in this patient?

Answer 41

She will have low C3, with NORMAL C1 and C4

She is having EXCESSIVE activation of the alternative complement pathway.

Question 42

You have a 10 year old girl that comes into your clinic. Upon evaulation she has hematuria and proteinuria. Her serum levels have low levels of some complement, and low levels of Factor B and Properdin. Her kidney function is impacted.

What would be expected upon LM?

What would be seen upon EM?

Answer 42

Mesangial Proliferation, GBM Thickening, Splitting,

Intramembranous Dense Deposits, Ribbon like, homogenous (permeation of the lamina densa)

Question 43

You have a 10 year old girl that comes into your clinic. Upon evaulation she has hematuria and proteinuria. Her serum levels have low levels of some complement, and low levels of Factor B and Properdin. Her kidney function is impacted.

She undergoes a kidney transplant – what is her outcome?

Answer 43

She needs to be watched, as Reccurrence is big with this disease!

Question 44

You have a 10 year old girl that comes into your clinic. Upon evaulation she has hematuria and proteinuria. Her serum levels have low levels of some complement, and low levels of Factor B and Properdin. Her kidney function is impacted.

What is her clinical manifestations?

What is her disease?

Answer 44

Hematuria (Nephritic Like), Chronic Renal Failure

DENSE DEPOSIT DISEASE (MPGN Type II)

Question 45

You have 25 year old Asian Male who complaims of gross hematuria. Upon your thorough history taking, you find that he recently had a Respiratory Infection and a UTI. In his family history he notes that his father, whom he wasnt very close with, had similar problems (at least thats what his mother, Angie, told him). He explains that his hematuria is sporadic and that is lasts for 2-3 days before going away for a couple of months at a time before returning.

If you were able to interview his father, what other problems could his family history have – or he (the 23 year old) may have that would help you?

Answer 45

A Gluten Enteropathy (Celiac Dz), Liver Disease

Question 46

You have 25 year old Asian Male who complaims of gross hematuria. Upon your thorough history taking, you find that he recently had a Respiratory Infection and a UTI. In his family history he notes that his father, whom he wasnt very close with, had similar problems (at least thats what his mother, Angie, told him). He explains that his hematuria is sporadic and that is lasts for 2-3 days before going away for a couple of months at a time before returning.

You want to know how this is affecting his KIdneys, how would you describe it?

Answer 46

It is affecting them Focally, and Proliferatively.

Question 47

You have 25 year old Asian Male who complaims of gross hematuria. Upon your thorough history taking, you find that he recently had a Respiratory Infection and a UTI. In his family history he notes that his father, whom he wasnt very close with, had similar problems (at least thats what his mother, Angie, told him). He explains that his hematuria is sporadic and that is lasts for 2-3 days before going away for a couple of months at a time before returning.

What is the clinical manifestations of his disease?

If he were having no other systemic problems, what would be the possible diagnosis?

Answer 47

Recurrent Hematuria and Mild Proteinura

Berger Dz (IgA Nephropathy)

Question 48

You have 25 year old Asian Male who complaims of gross hematuria. Upon your thorough history taking, you find that he recently had a Respiratory Infection and a UTI. In his family history he notes that his father, whom he wasnt very close with, had similar problems (at least thats what his mother, Angie, told him). He explains that his hematuria is sporadic and that is lasts for 2-3 days before going away for a couple of months at a time before returning.

What other surgery would have clued you in to him having this disease?

Answer 48

A Kidney Transplant (reciepent)

Question 49

You have 25 year old Asian Male who complaims of gross hematuria. Upon your thorough history taking, you find that he recently had a Respiratory Infection and a UTI. In his family history he notes that his father, whom he wasnt very close with, had similar problems (at least thats what his mother, Angie, told him). He explains that his hematuria is sporadic and that is lasts for 2-3 days before going away for a couple of months at a time before returning.

What would be expected upon LM?

Upon IF?

Upon EM?

Answer 49

Focal Mesangial Proliferation, and widening

Mesangial Proliferation of IgA, IgM and C3

Mesangial Deposits

Question 50

You have 25 year old Asian Male who complaims of gross hematuria. Upon your thorough history taking, you find that he recently had a Respiratory Infection and a UTI. In his family history he notes that his father, whom he wasnt very close with, had similar problems (at least thats what his mother, Angie, told him). He explains that his hematuria is sporadic and that is lasts for 2-3 days before going away for a couple of months at a time before returning.

lets say this 25 year old had actually brought in his 3 year old infant, and the symptoms had more or less been the same – what would your new diagnosis be?

For his infant, would it be systemic or non-systemic?

What symptoms would the 3 year old have most likely, with the ones listed?

Answer 50

HS Purpura

Systemic

Abdominal Pain, Arthralgias, and Subepidural purpuric skin manifestations

Question 51

You have a couple of patients you are trying to quickly identify as the most likely to progress to Chronic Glomerulonephritis (CG).

Which order would the diseases be in?

Answer 51

1) Crescentic GN (RPGN)
2) FSGS
3) MPGN
4) Membranous Nephropathy & IgAN
5) Post Strep GN (least likely)

Question 52

You have a couple of patients you are trying to quickly identify as the most likely to progress to Chronic Glomerulonephritis (CG).

What stain would you use for CG?

What are the progressive symptoms of CG?

What could potentially cause death in these patients?

Answer 52

Massone Trichome Staine

Decreased GFR, Decresed Loss of Protein, Loss of Apetitie, Anemia, Vomtiing, Weakness, HTN, Cerebral or Cardiovascular Dz’s

Renal Insuffienciy/UREMIA

Question 53

You have a 10 year old boy with onset of hematuria. He is noted to have a family history of CRF, Lens Dislocation, and Corneal Dystrophy. Upon examination he has proteinuria, with an irregular thickening of the BM upon EM, and lamination of the lamina densa. . . one pathologist even walks in a says it looks “Moth Eaten” (Thanks Jerry . . . .we get it you vape)

What is the disease?

How is it transmitted

Answer 53

Alport Syndrome

Genetically (X-Linked) –> look for family history

Question 54

You have a 10 year old boy with onset of hematuria. He is noted to have a family history of CRF, Lens Dislocation, and Corneal Dystrophy. Upon examination he has proteinuria, with an irregular thickening of the BM upon EM, and lamination of the lamina densa. . . one pathologist even walks in a says it looks “Moth Eaten” (Thanks Jerry . . . .we get it you vape)

What is the prognosis for this little boy?

Answer 54

He will progress to renal failure by about age (20-50)

Question 55

You have a 18 year old female that has a family history of rare benign disease. She is afraid that she had mild to moderate proteinuria. She thinks she has noticed some hematuria.

What is the problem underlying her disease?

What is her disease she is talking about?

Answer 55

Type IV Collagen of the Alpha 3 and 4 chains

Thin Basement Membrane Lesion (Benign Familial Hematuria)

Question 56

You have a 18 year old female that has a family history of rare benign disease. She is afraid that she had mild to moderate proteinuria. She thinks she has noticed some hematuria.

You do a genetic test, and find out that she is a heterozygote for the disease. If she had been a homozygote how would that have changed your prognosis?

Answer 56

Homozygotes – have a clinical symptomology closer to Alport Syndrome (more severe)

Question 57

You have a 54 year old African American diabetic man who comes into your clinic complaining of LE edema, and unexpected weight gain. You record his BP at 165/100, and he tells you he is also taking BP medication. He is overweight, and his physical activity is low to none.

What 3 renal system lesions do you excpect to find with this Diabetic patient?

Answer 57

Glomerular Lesions, Vascular Lesions (arteriolosclerosis), Pyelonephritis with Necrotizing Papillitis

Question 58

You have a 54 year old African American diabetic man who comes into your clinic complaining of LE edema, and unexpected weight gain. You record his BP at 165/100, and he tells you he is also taking BP medication. He is overweight, and his physical activity is low to none.

He asks you about the timeline of his disease, and wants to know what diabetes affects in the beginning vs the later stages?

What sort of things do you need to worry about in the future with this patient?

Answer 58

Starts: Microvasculature

Ends: Macrovasculture

Worry about: MI, Renal Vasc Insuff, Cerebrovascular incidents, Retinopathy, Neuropathy, Bacterial Infections

Question 59

You have a 54 year old African American diabetic man who comes into your clinic complaining of LE edema, and unexpected weight gain. You record his BP at 165/100, and he tells you he is also taking BP medication. He is overweight, and his physical activity is low to none.

What would be expected upon LM?

What would be expected upon EM?

Answer 59

LM: Diffusely, thickened tubular GBM

EM: Injured Podocytes and a thickened GBM, also Diffuse Mesangial Sclerosis (an increase in the mesangial matrix)

Question 60

You have a 54 year old African American diabetic man who comes into your clinic complaining of LE edema, and unexpected weight gain. You record his BP at 165/100, and he tells you he is also taking BP medication. He is overweight, and his physical activity is low to none.

You realize that his Diabetic Glomerulosclerosis has the 3 main glomerular lesions –?

Upon biopsy how would these 3 things best be visculaized?

Answer 60

1) Diffuse Capillary BM thickening
2) Diffuse Mesangial Sclerosis
3) Nodular Glomerulosclerosis

PAS Stain

Question 61

You have a 54 year old African American diabetic man who comes into your clinic complaining of LE edema, and unexpected weight gain. You record his BP at 165/100, and he tells you he is also taking BP medication. He is overweight, and his physical activity is low to none.

This poor guy 10 years down the road progresses to what?

Answer 61

End Stage Diabetic Nephrosclerosis

(From severe HTN –> causing thinning of the renal cortex, and irregular cortical depressions, and a diffuse granular, pitted surface to the kideny.)

Question 62

A 35 year old African American Female comes into your office with a known history of SLE, upon inspection you find Subendothelial Immune Deposists, and upon EM you see “wire loops” to the renal glomerular capillary, which is also present in the mesangium. You notice her glomerulus has marked increase in cellularity, and a decrease in urinary space. Upon IF you see anti-IgG ab.

What is her best diagnosis?

How would we treat this?

Answer 62

(Class IV) Focal and Diffuse Proliferative Lupus Nephritis

Tx the underlying disease. (Lupus)

Question 63

A 35 year old African American Female comes into your office with a known history of SLE, upon inspection you find Subendothelial Immune Deposists, and upon EM you see “wire loops” to the renal glomerular capillary, which is also present in the mesangium. You notice her glomerulus has marked increase in cellularity, and a decrease in urinary space. Upon IF you see anti-IgG ab.

If she instead had immune complex deposition in the mesangium, with no structural changes how would this affect your diagnosis?

Answer 63

Class I – Minimal Mesangial Lupus Nephritis

(least common form)

Question 64

You have a 40 year old male who had a routine abdominal surgery the previous day, and is now not urinating. You monitor him for the next day and notice that he is still not urinating and he is becoming hyperkalemic, and has elevated BUN.

What stage of Acute Tubular Injury is he in?

What is this scernario describing?

What is his injury a common cause for?

Answer 64

He is in the Maintenance Phase of ATI

Oliguric Crisis

Acute Kidney Injury

Question 65

You have a 40 year old male who had a routine abdominal surgery the previous day, and is now not urinating. You monitor him for the next day and notice that he is still not urinating and he is becoming hyperkalemic, and has elevated BUN.

You continue to monitor him, and now he is experiencing an increase in urination (and was measured to be 3L a day)

What is this patient susceptible too now?

Answer 65

He is becoming Hypokalemic, and has an increased risk of Infection.

Question 66

You have a 40 year old male who had a routine abdominal surgery the previous day, and is now not urinating. You monitor him for the next day and notice that he is still not urinating and he is becoming hyperkalemic, and has elevated BUN.

What are the 2 main critical events that usually cause ATI?

Answer 66

Tubular Injury, and Persistent Disturbances in Blood Flow.

Question 67

You have a 40 year old male who had a routine abdominal surgery the previous day, and is now not urinating. You monitor him for the next day and notice that he is still not urinating and he is becoming hyperkalemic, and has elevated BUN.

The attending notes that he has had significant ischemia to portions of his tubules – and wants to know how they were affected?

How does this differ from if he had taken a toxic load of asprin?

What is this patient at risk for because of the ischemia?

Answer 67

He will have patchy Necrosis of the PCT, PST, and HL

If he had toxic injury you would see continous necrosis at those locations with swelling and vacuolization.

Necrotic Tubules cells sloughing off and causing obstructed flow.

Question 68

A 21 year old patient comes into your clinic for a regular screening, and upon a urine test you notice an elevated BUN and Creatinine. You note there is no blood in the urine and no protein in the urine. You also notice that he had rapidly unconcentrated urine.

What is the disease process?

What usually causes this in a patient?

Answer 68

Acute Tubulointersitial Nephritis

Infections, Toxins, Metabolic Dz, Chronic Obstruction, Bence Jones Protein Neoplasms, Immune Rxn, Vascular Dz’s

Question 69

A 21 year old patient comes into your clinic for a regular screening, and upon a urine test you notice an elevated BUN and Creatinine. You note there is no blood in the urine and no protein in the urine. You also notice that he had rapidly unconcentrated urine.

If this 21 year old had been noted to have Polycystic Kidney Dz or Diabetes how would this affect your diagnosis?

Answer 69

He would be said to have Secondary Tubulointerstitial Nephritis (since its coming from a systemic source)

Question 70

A 21 year old patient comes into your clinic for a regular screening, and upon a urine test you notice an elevated BUN and Creatinine. You note there is no blood in the urine and no protein in the urine. You also notice that he had rapidly unconcentrated urine.

How would you seperate the acute vs chronic forms histologically?

Answer 70

Acute: Interstitial Edema, Eosinophils, Neutrophils (INSIDIOUS onset)

Chronic: Fibrosis and Tubular Atrophy

Question 71

A 38 year old female has recently had pain while urinating, and frequent urination. She also has a slight fever, and is noted to be tired. You note that she has a UTI, but are worried it might be more.

What are you worried about and why?

What bacteria would normally cause this?

Where else would you ask her if she has had pain recently?

Answer 71

Acute Pyelonephritis, due to an ascending infection

Ecoli, Proteus, Klebsiella, Enterobacter

At the Costovertebral Angle

Question 72

A 38 year old female has recently had pain while urinating, and frequent urination. She also has a slight fever, and is noted to be tired. You note that she has a UTI, but are worried it might be more.

What would be expected to be found in the urine?

What anatomic defect could have made this woman more prone to this disease state?

Answer 72

Pus (Pyuria)

Vesicoureteral Jxn (reflux) –> leads to pyelonephritis

Question 73

A 38 year old female has recently had pain while urinating, and frequent urination. She also has a slight fever, and is noted to be tired. You note that she has a UTI, but are worried it might be more.

Lets say that she didnt have the UTI, but instead had a bacterial infection of Staph Aureus – how does this change your diagnosis?

Answer 73

You would expect a Hematogenous Spread of bacterial –> potentially sepsis infection

Question 74

A 38 year old female has recently had pain while urinating, and frequent urination. She also has a slight fever, and is noted to be tired. You note that she has a UTI, but are worried it might be more.

If this infection were to continue and become chronic what anatomy would it affect?

Answer 74

It would damage the Renal Pelvis, Calyx, and Parenchyma –> causing Chronic Pyelonephritis

Question 75

A 38 year old female has recently had pain while urinating, and frequent urination. She also has a slight fever, and is noted to be tired. You note that she has a UTI, but are worried it might be more.

You note Pyonephrosis, and Perinephric abscesses . . .but you know you are forgetting one other thing that you need to worry about with her case. . . ?

Answer 75

Papillary Necrosis

Question 76

A 38 year old female has recently had pain while urinating, and frequent urination. She also has a slight fever, and is noted to be tired. You note that she has a UTI, but are worried it might be more.

If she were to be Diabetic how would that affect the Papillae

If she were noted to be taking Analgesics how would that affect the papillae?

Answer 76

Diabetes –> a Grayish Necrosis to only the papillae

Analgesic Nephropathy –> causes a Red-Brown (mahogony) necrotic papillae

Both of these once again cause sloughing off, and can obstruct urine flow.

Question 77

A 38 year old female has recently had pain while urinating, and frequent urination. She also has a slight fever, and is noted to be tired. You note that she has a UTI, but are worried it might be more.

She is later noted to have Chronic Pyelonephritis due to this disease process, what other disease affects the Calyx of the Kidney?

She is likely to progress to what?

Answer 77

Analgesic Nephropathy

(Chronic Pyelonephritis and Analgesic Nephropathy are the only two)

ESRD

Question 78

A 38 year old female has recently had pain while urinating, and frequent urination. She also has a slight fever, and is noted to be tired. You note that she has a UTI, but are worried it might be more.

She is now noted to have Xanthagranulmatous Pyelonephritis . . . . what species causes this and how would describe it?

Answer 78

Proteus Sps

Foul Smelling urine!

Question 79

A 67 year old African American female is noted to have HTN, and diabetes. You note she has medial and intimal thickening,

She is at risk for Renal Failure and . . .

If her HTN was noted to have been severe HTN (180/100 etc) what would you classify it as?

Answer 79

Benign Nephrosclerosis

Malignant Nephrosclerosis

Question 80

A 67 year old African American female is noted to have HTN, and diabetes. You note she has medial and intimal thickening,

If she was noted to have Malignant HTN – what would you expect to see pathogenesis wise?

Answer 80

Endothelial Irreversible Injury

Focal Hemorrhage (due to focal vascular cell death)

Thrombosis due to platelet deposition

FIBRINOID Necrosis

Question 81

A 67 year old African American female is noted to have HTN, and diabetes. You note she has medial and intimal thickening,

Whar are some systemic problems that you need to worry about?

Answer 81

Renal failure

Papilledema

Retinal Hemorrhages

Encephalopathy

Cardiovascular Abnorm.

(Increased Intracrainial Pressure!)

Question 82

An 8 year old boy comes into the hospital with influenza and diarrheal symptoms. He has been coughing up blood, and having severe oliguria, and hematuria. His vital signs show that he has elevated BP. He is also noted to have elevated TNF in his blood.

What toxin is causing this?

What physiologically is happneing?

What disease process is occuring?

Answer 82

Shiga Like Toxin (E Coli)

Endothelial Apoptosis

Microangiopathy (Hemolytic Uremic Syndrome)

Question 83

An 8 year old boy comes into the hospital with influenza and diarrheal symptoms. He has been coughing up blood, and having severe oliguria, and hematuria. His vital signs show that he has elevated BP. He is also noted to have elevated TNF in his blood.

HUS can causes what?

What two organisms usually cause it?

A typical presentations (Adult) are due to what?

Answer 83

Thrombocytopenia, Anemia, AKI

E Coli, Shigella

Inherited Mutations of Proteins –> and activation of C3 convertase

Question 84

You have a 39 year old female, she is complaining of fever, anemia, and thrombocytopenia and neurological problems, and renal failure.

What gene is the problem, and what associated protein?

What is happening physiologically?

Answer 84

ADAMTS13

vWF

activating platelets

Question 85

a 69 year old (niceee) came into your clinic complaining of flank pain and hematuria. You note that the patient has a history of long standing HTN and has had arrhythmias (currently taking Amiodarone), as well as some other kidney trouble. He also notes that he has a N/V, oliguria and bouts of anuria.

What do we need to be on the look out for in this patient?

Answer 85

Acute Renal Failure, since he has had bad renal fxn for many years

Question 86

a 69 year old (niceee) came into your clinic complaining of flank pain and hematuria. You note that the patient has a history of long standing HTN and has had arrhythmias (currently taking Amiodarone), as well as some other kidney trouble. He also notes that he has a N/V, oliguria and bouts of anuria.

If he was noted to have just recently had an aortography what do we need to think?

Answer 86

Pedal pump . . . . . . (okay not this)

Embolization of Atheromatous Plaques

- We also need to worry about a Ischemic Kidney infarction due to that emboli (Wedge shaped)

Question 87

a 34 year old Nigerian male comes into your clinic with hematuria, and hyposthenuria. You notice he has papillary necrosis and cortical scarring. He is noted to have proteinuria (1.2 mg)

What is the most likely diagnosis?

Answer 87

Sickle Cell Nephropathy

Question 88

You are a pathologist, and your patient comes into your office, Dead. They do not have a BP, and their pulse is zero. You begin your autopsy and find a rather common defect to their kidneys?

What would be the most common defect?

A new guy comes in, and you find that his kidney(s) are located at the jxn of the pelvic brim/pelvis – what causes this?

Answer 88

Horseshoe Kidney (fusion of the lower poles)

Ectopic Kidney

Question 89

You are a pathologist, and your patient comes into your office, Dead. They do not have a BP, and their pulse is zero. You begin your autopsy of a fetus who perished in-utero.

If the fetus was said to have died of problems with her lungs – what would expect the problem to be?

Answer 89

Renal Agenesis (Bilateral) or even hypoplasia.

causing lung growth to retard.

Question 90

A 28 year old Swedish man comes into your clinic. He has flank pain and hematuria. His BP is noted to be high, and he says that he regularly takes hypertension medication for it. He says that he has a tendancy for kidney stones, but has normally not sought out medical care – as he lives a typical swede life. You decide to biopsy his kidney and find multiple cystic lesions throughout. You diagnose him with Adult Polycystic Kidney Dz

What is the inhertiance?

What gene is most likely associated and what does it code for?

What does this mean for his outcome?

Answer 90

AD

PKD1 –> Polycystin 1

He will progress to ESRD/CKD anywhere from 40-70

Question 91

A 28 year old Swedish man comes into your clinic. He has flank pain and hematuria. His BP is noted to be high, and he says that he regularly takes hypertension medication for it. He says that he has a tendancy for kidney stones, but has normally not sought out medical care – as he lives a typical swede life. You decide to biopsy his kidney and find multiple cystic lesions throughout. You diagnose him with Adult Polycystic Kidney Dz.

Will this disease affect both or only one kidney?

Where else should we check for in this patient?

What is inside the cysts of the kidney?

Answer 91

BILATERAL Kidneys (Diffuse over whole kidney)

Liver Cysts, Diverticulae of the Colon, Aneurysms

Clear Serous Fluid

Question 92

A 28 year old Swedish man comes into your clinic. He has flank pain and hematuria. His BP is noted to be high, and he says that he regularly takes hypertension medication for it. He says that he has a tendancy for kidney stones, but has normally not sought out medical care – as he lives a typical swede life. You decide to biopsy his kidney and find multiple cystic lesions throughout. You diagnose him with Adult Polycystic Kidney Dz

What other symptoms/problems will this patient have (now or in the future)?

Answer 92

Kidney Stones

Intracranial Berry Anueysms

MVP

Chronic Renal Failure – with azotemia

Question 93

A 28 year old Swedish man comes into your clinic. He has flank pain and hematuria. His BP is noted to be high, and he says that he regularly takes hypertension medication for it. He says that he has a tendancy for kidney stones, but has normally not sought out medical care – as he lives a typical swede life. You decide to biopsy his kidney and find multiple cystic lesions throughout. You diagnose him with Adult Polycystic Kidney Dz

If he were given a genetic test and told that he had the better prognosis of the two types of ADKD – what would the gene and product that it codes for be?

What do both of the genes (for the 2 types) code for, and their action?

Answer 93

PKD2 Gene

Polycystin 2

They both code for polycystin proteins that affect tubular cilia and calcium flux –> this causes problems with growth and differentiation –> causing cell proliferation and fluid accumulation –> cyst formation and fibrosis!

Question 94

A 28 year old Swedish man comes into your clinic. He has flank pain and hematuria. His BP is noted to be high, and he says that he regularly takes hypertension medication for it. He says that he has a tendancy for kidney stones, but has normally not sought out medical care – as he lives a typical swede life. You decide to biopsy his kidney and find multiple cystic lesions throughout. You diagnose him with Adult Polycystic Kidney Dz

If this patient had instead been an African American male, how would this have changed the prognosis?

Answer 94

It is less common in AA, but when it is present MUCH MORE Aggresive.

Question 95

A 1 week old baby is brought to you (lucky duck). You notice enlarged cystic kidneys (that were noted to be that way at birth by the OB/GYN) and you notice hepatic fibrosis (with your eyes of osteopathy). The baby has Portal HTN, and mild splenomegaly. You realize, at once, that this baby has Childhood Polycystic Kidney Dz.

What is the inheritance?

What is the specific group of this disease, the baby will fit into?

What is its prognosis, and why?

Answer 95

AR

Neonatal period

Poor –> may live a few months, but will probably die of renal failure

(This is because about 60% of their collecting ducts are cystic, and they have mild hepatic fibrosis)

Question 96

A 1 week old baby is brought to you (lucky duck). You notice enlarged cystic kidneys (that were noted to be that way at birth by the OB/GYN) and you notice hepatic fibrosis (with your eyes of osteopathy). The baby has Portal HTN, and mild splenomegaly. You realize, at once, that this baby has Childhood Polycystic Kidney Dz.

If this had been a 4 year old baby that was brought to you, how would that have affected your prognosis?

Answer 96

Better –> they would have been in the infantile range and would have only about 20% of their collecting ducts be cystic – but have hepatic fibrosis and failure.

They might live into early childhood, but would die around that time.

Question 97

A 1 week old baby is brought to you (lucky duck). You notice enlarged cystic kidneys (that were noted to be that way at birth by the OB/GYN) and you notice hepatic fibrosis (with your eyes of osteopathy). The baby has Portal HTN, and mild splenomegaly. You realize, at once, that this baby has Childhood Polycystic Kidney Dz.

If this had been a 9 year old, what would be expected for you to see upon examination?

Prognosis?

Answer 97

Esophageal Varices, due to heavy hepatic fibrosis causing portal HTN.

Most die in adolescence.

Question 98

A 1 week old baby is brought to you (lucky duck). You notice enlarged cystic kidneys (that were noted to be that way at birth by the OB/GYN) and you notice hepatic fibrosis (with your eyes of osteopathy). The baby has Portal HTN, and mild splenomegaly. You realize, at once, that this baby has Childhood Polycystic Kidney Dz.

What Gene and protein are associated with this?

If you were to visulize the kidney, how would the cysts be arranged in the kidney?

Answer 98

PKHD1 Gene –> Fibrocystin Protein

Cysts would be perpendicular to the Renal Capsule (in the collecting ducts)

Question 99

You have a 25 year old female, who complains of Hematuria, UTI, and recurrent renal stones. You note that her kidney fxn is suprisingly normal, but she does have multiple cystic dilations of the collecting duct.

Where would these cystic dilations most likely be found on the kidney?

Is this a serious condition?

Is this an inherited condition?

Answer 99

In the Medulla

No, its a Benign Medullary Sponge Kidney

No Inheritance (so it can be unilateral or bilateral)

Question 100

a 3 year old infant is brought to your clinic by her mother. She has noticed that little Debbie Ann-Reigxh (the X is silent, because her father left her, and the no-good-for-nothing man doesnt deserve the X in this child). She has noticed the baby has had excessive thirst, and lots of urination. She also is worried because she doesnt think that her baby is growing in comparison to her sisters baby (whom is much uglier – her words not mine). Upon inspection you notice that the baby is having excessive salt wasting, and has corticomedullary cysts.

You would expect the kidneys to be what?

What is this Disease?

What is the inheritance?

Answer 100

Shrunken

Familial Juvenile Nephronopthisis

AR

(Nephronophthisis is characterized by fibrosis and the formation of cysts at the cortico-medullary junction)

Question 101

A 27 year old man comes to your clinic with excessive urination and salt wasting. He noticed that he has been peeing a lot more than usual later, and he says he has been excessively thirsty (in more ways than one. . . . ;- ) ) nonetheless, you immediately realize that he has . . .

What is the Inheritance?

How would his kidneys look?

Outcome?

Answer 101

Adult Onset Medullary Cystic Disease (Nephronopthisis)

AD

He would have shrunken kidneys, with corticomedullary cysts

Progress to Chronic Renal Failure

Question 102

A 40 year old female named Susan, comes into your clinic (because she wanted to speak to the manager) and decides to question you on why she has been noticing microscopic hematuria (you wonder how she noticed this, but realize now is not the time). She also did her own biopsy of her kidney and found cysts. She wants to know how long she has to live – and wants her meal comp’d at the local diner. . . .

How would you characterize her disease proccess (besides being overdramatic).

What finding would tell you that maybe she is prone to RCC?

Answer 102

Benign, very good prognosis.

If she had undergone Dialysis. – RCC is common in these patients about 10 years afterwards.

Question 103

A 62 year old male has acquired renal cystic disease, he has been having hemorrhage and erythrocytosis, and has been dependent on dialysis lately.

How would you characterize the morphology of his disease process?

Answer 103

Cystic Degeneration in End-Stage Kidney

Question 104

Oh god, Susan is back now – and she has brought her 2 year old baby boy, because she found an abdominal mass. She says that the doctor when he was born, told her that he had unilateral ureter agenesis, and that he would die in 20 days . . . you realize that she is full of shit on that last part, and decide to tell her that her baby boy probably has. . .

She asks if he had this bilaterally if it would be worse?

Answer 104

Unilateral Multicystic Renal Dysplasia

-Irregular kidneys with cysts of various size.

YES! Bilateral could cause renal failure.

Question 105

A grumpy 30 year old man comes into your clinic, because he is in excrutiating pain. He has intense pain, and can’t pee. He drinks lots of sugary drinks, and is found to have hypercalciuria. He is most likely having what?

Answer 105

Nephrolithiasis (Kidney Stone)

Question 106

A patient of indiscriminate gender, and indiscriminate age shows up to your clinic and is found to have a kidney neoplasm that is less than 1.0 cm in diameter. On further examination you see gross cortical, discrete, yellow gray, small lesions and Psammoma Bodies. These lesions are made up of complex, branching, papillomatous structures.

What is the diagnosis?

How is it defined?

Benign or Malignant?

Answer 106

Renal Papillary Adenoma

By its size! (<1.0 cm)

Benign

Question 107

You evaluate a middle aged female patient who has a tumor that is mahogany-brown and well encapsulated. You see large eosinophilic cells, and small round benign appearing nuclei – with really large nucleoli. She tells you she is getting a second opinion, as the doctor before her diagnosed this as RCC.

Do you agree?

The tumors would have what shape of scar?

These tumors arise from what?

What would be seen on EM?

Answer 107

No, This is Renal Oncocytoma (it is commonly confused for RCC)

Central Stellate Scar

Type A Intercalated Cells of the Renal Cortical Collecting Duct

Lots of Mitochondria in the cells!

Question 108

Dr. Puttoff is talking to you about a patient he had, where a middle aged female who had tuberous sclerosis complex, an AD disorder, had a specific cancer where she have massive retroperitoneal and intra-abdominal hemorrhage.

What Cancer is he talking about? Benign or Malignant?

What gene is lost in these neoplasms?

What effects would the Tuberous Sclerosis complex have on the patient?

Answer 108

Renal Angiomyolipoma // Benign

TSC1/TSC2 (tumor suppressor gene)

Causing her to have: Tumors of the Brain, Skin, Kidney, Heart, Lungs and Eyes!

Question 109

Dr. Puttoff is talking to you about a patient he had, where a middle aged female who had tuberous sclerosis complex, an AD disorder, had a specific cancer where she have massive retroperitoneal and intra-abdominal hemorrhage.

What is a neurologic manifestation that this patient probably had, that could help clue you in to them having tuberous sclerosis and associating it with Angiomyolipoma?

Answer 109

If they had Mental Retardation or Epilepsy (due to the Brain lesions of Tuberous Sclerosis)

Question 110

You see an 78 year old male who is mixed. He is noted to have been a long time smoker, has mild HTN, is obese. He used to be a home construction worker in the 50’s-60’s. He is complaining of costovertebral pain. You also note some of his hormone levels are a little off. You also note that his VHL Tumor suppressor gene is affected.

Is this a Benign or Malignant cancer?

What specific type of cancer is highest on your Dx?

What is his prognosis?

Answer 110

Malignant

Clear Cell Carcinoma

an Average Prognosis

(The cytoplasm is clear – b/c of the glycogen and lipid accumulation)

Question 111

You see an 78 year old male who is mixed. He is noted to have been a long time smoker, has mild HTN, is obese. He used to be a home construction worker in the 50’s-60’s. He is complaining of costovertebral pain. You also note some of his hormone levels are a little off. You also note that his VHL Tumor suppressor gene is affected.

If instead of his VHL gene being affected, it affected the MET proto-oncogene – would this change your Dx?

Does this change your prognosis?

Answer 111

Yes, It would be potentially Papillary Carcinoma?

No – Same Average Prognosis

Question 112

You see an 78 year old male who is mixed. He is noted to have been a long time smoker, has mild HTN, is obese. He used to be a home construction worker in the 50’s-60’s. He is complaining of costovertebral pain. You also note some of his hormone levels are a little off.

Instead he now has Branching Tubules lined by highly typical cuboidal cells – what is his Dx?

What other diseases have a similar prognosis?

Answer 112

Collecting Duct Carcinoma

(WORST PROGNOSIS)

Xp11 Translocation Carcinoma

Medullary Carcinoma

Sarcomatoid RCC

Question 113

You see an 78 year old male who is mixed. He is noted to have been a long time smoker, has mild HTN, is obese. He used to be a home construction worker in the 50’s-60’s. He is complaining of costovertebral pain. You also note some of his hormone levels are a little off. You also note that his VHL Tumor suppressor gene is affected.

He exhibits 1/3 of the triad of symptoms for RCC –> What are the other two?

Answer 113

Hematuria

Costovertebral Pain

Palapable Flank Mass

Question 114

You see an 78 year old male who is mixed. He is noted to have been a long time smoker, has mild HTN, is obese. He used to be a home construction worker in the 50’s-60’s. He is complaining of costovertebral pain. You also note some of his hormone levels are a little off. You also note that his VHL Tumor suppressor gene is affected.

What is the route of spread? and what structure could be affected?

Answer 114

Hematogenous

Renal Vein is invaded

Question 115

You have 89 year old patient that has bladder tumors, and complaining of hematuria, hydronephrosis, and flank pain. She was diagnosed with Uroethial (Transitional Cell) Carcinoma (of the Kidney).

What other associations do we make with this cancer?

Answer 115

Analgesic Nephropathy

Balkan Nephropathy

Question 116

You see a 5 year old asian boy, that has a large abdominal mass, that is slightly tender to palpation. He has unusally high BP, and hematuria. His parents say that when we has born he had undescended testes.

What is the problem here?

What is this problem assocaited with?

Answer 116

WIlms Tumor

Associated with WAGR syndrome

Question 117

You see a 5 year old asian boy, that has a large abdominal mass, that is slightly tender to palpation. He has unusally high BP, and hematuria. His parents say that when we has born he had undescended testes.

If he had instead had Gonadal Tumors –> what would we associate it with?

If he had macroglossia, and Hemihypertrophy?

Answer 117

Denys-Drash Syndrome

Beckwith-Wiedemann Syndrome

Both have associations with WIlms Tumors (Syndromic)

Question 118

You see a 5 year old asian boy, that has a large abdominal mass, that is slightly tender to palpation. He has unusally high BP, and hematuria. His parents say that when we has born he had undescended testes.

What is the precursor lesion to this problem?

What would make this boys prognosis MUCH worse?

Answer 118

Nephrogenic Rests

Diffuse Anaplasia

Question 119

You have a 2 year old baby girl that comes to you with an abnormal organization of smooth muscle bundles bilaterally in her urinary tract. She was told that she had UPJ Obstruction, and this caused her to have what other condition?

What other types of conditions cause UPJ Obstruction?

Answer 119

Hydronephrosis

Retroperitoneal Tumors and Fibrosis (Extrinisic things)

Question 120

A 55 year old male complains of not being able to pee. He says that he has been taking Propanolol, Amlodipine for some heart stuff. He also says that he has mild Chron Disease. On further inspection you notice that his ureters are narrowed – and the process seems to have involved his pancreas.

What is the cause of his ureteral narrowing and obstruction?

What would you see microscopically?

Answer 120

IgG4-Related Disease

Germinal Centers, Plasma Cells and Eosinophils and lymphocytic infiltrate.

Question 121

A 55 year old male complains of not being able to pee. He says that he has been taking Propanolol, Amlodipine for some heart stuff. He also says that he has mild Chron Disease. On further inspection you notice that his ureters are narrowed – and the process seems to have involved his pancreas.

What is the idiopathic version of this disease process called?

Answer 121

Ormond Disease (most common)

Question 122

Sharon, the abnormally nosy woman has shown up to your clinic again – after berating a 18 year old for being “suspicious”. She now states that her new baby has many congenital defects of the lower urinary tract – and wants you to explain what the most common one is (or else your yelp review will be getting slammed).

What would you explain is the most serious and most common congenital anomalie of the Urinary Bladder?

Answer 122

Vesicoureteral Reflux

Question 123

Sharon, the abnormally nosy woman has shown up to your clinic again – after berating a 18 year old for being “suspicious”. She now states that her new baby has many congenital defects of the lower urinary tract – and wants you to explain (or else your yelp review will be getting slammed).

She now states her baby is peeing out his belly button, and she thinks he needs to take some vitamins – because clearly B4 is deficient. You sigh, and disagree and tell her . . .

Answer 123

Your baby has a Patent Urachus

(He doesn’t, shes a terrible parent)

Question 124

Sharon, the abnormally nosy woman has shown up to your clinic again – after berating a 18 year old for being “suspicious”. She now states that her new baby has many congenital defects of the lower urinary tract – and wants you to explain what the most common one is (or else your yelp review will be getting slammed).

Lastly she decides to tell you that her little guy had his bladder on the outside of his body, and wants to know if she should be worried – as no one seemed alarmed on her “Parents-Against-Everything-Good-For-Your-Child” Website.

Answer 124

He needs surgery (first of all), and he is at risk for infections, that can cause Pyelonephritis (Due to infections traveling up)

He is also at risk for Adenocarcinoma of the bladder

Question 125

You have a 32 year old female that complains of frequent urination, and lower abdominal pain, and pain while urinating. She has a lsight fever (99.0) and you notice her urine is slightly cloudy. She thinks she has a UTI, but isnt 100% sure. You notice that her white blood cell count is high, and that culture(s) show up positive for a Gram Neg Rod, she also has pus in the urine.

Where is her infection?

What are the most common organisms that cause this?

Answer 125

the Bladder (Cystitis)

E-Coli (most common), Proteus, Klebsiella, Enterobacter, Staph Sapro

Question 126

You have a 32 year old female that complains of frequent urination, and lower abdominal pain, and pain while urinating. She has a lsight fever (99.0) and you notice her urine is slightly cloudy. She thinks she has a UTI, but isnt 100% sure. You notice that her white blood cell count is high, and that culture(s) show up positive for a Gram Neg Rod, she also has pus in the urine.

What do we need to monitor her for?

If she had been noted to be Immunocomprimised, what sort of organisms would have risen to our differential?

If she were to progress to a chronic form of this disease, what would you expect to see on culture?

Answer 126

Pyelonephritis (due to traveling infection)

Mycobacteria, Fungi, Viruses, Protozoa

T-Cells, B-Cells, and NK Cells

Question 127

A 20 year old female has been taking cyclophosphamide (a Anti-Tumor Drug) given to her by another physician at your clinic. She has been feeling unwell, and has come back to your clinic to see what is going on. She is feverish, but her white cell count is normal. You suspect a virus. She also has pain in her abdomen (Suprapubic region). You diagnose her with Hemorrhagic Cystitis.

How would you characterize the spread of the hemorrhages?

Answer 127

Focal and Diffuse Hemorrhagic areas

Question 128

You have a 35 year old female that has severe suprapubic pain, urinary frequency, and urinary urgency. She states that she got worried after noticing red urine, and she hasn’t been going as often. Her lab cultures are negative, but you do find mast cells and lymphocytes on microscopy. You find specific ulcers that tell you what the disease is.

What ulcers did you find?

What is the disease?

Answer 128

Hunner Ulcers

Intersitial Cystitis (Chronic Pelvic Pain Syndrome)

Question 129

You have a 50 year old female that is immunocomprimised come into your clinic. You note her problems, and find that she has many yellow, raised mucoal plaques on her bladder. On Microscopy you see foamy macrophages, and lymphocytes that form granulomas.

What infection usually causes this?

What disease is this?

Answer 129

Proteus or Ecoli

Malakoplakia

Question 130

Youre attending is quizzing you on the urinary bladder neoplams. She wants you to quickly list off the Benign and Malignant Urinary Bladder neoplasms.

Benign?

Malignant?

Answer 130

Benign: Lipomas, Fibromas, Neurofibroma

Malignant: Rhabdomyoscarcoma, Leiomyosarcoma, Lymphoma

Question 131

You have a 4 year old female infant that presents to your clinic. You notice a botryoid cancer of her bladder. You find clusters of tumor cells beneath the epithelium and giving off a Nevoid appearance.

What does this child have?

Answer 131

Malignant Mesenchymal tumor – Embryonal Rhadomyosarcoma

(Sarcoma Botrypoides)

Question 132

You have a 60 year old male post chemotherapy. His physician referred him to you because he noticed he now had a new malignant mesenchymal tumor that is seen rarely in adults – and wanted a second opinion.

What tumor is he talking about?

Answer 132

Leiomyosarcoma

Question 133

You have a 65 year old female that has chronic cystitis, and you diagnose her with a primary malignant lymphoma. What type of lymphoma is usually associated, and what is it’s prognosis?

Answer 133

Non-Hodgkin Lymphoma (Diffuse Large B-Cell)

Good prognosis

Question 134

You diangose a patient with a Urothelial (Transitional Cell) Tumor, you note that they have thickened epithelium that is covering papillary projections. What variant is this?

Answer 134

PUNLMP (Papillary Urothelial Neoplasia of Low Malignant Potential)

(NON-Malignant)

Question 135

This patient you just diagnose with a bladder tumor has one of the most common patterns of bladder tumors.

What is the most common pattern, and what are the others?

Answer 135

Papilloma-Papillary Carcinoma (Most Common)

Invasive Papillary Carcinoma

Flat noninvasive carcinoma (CIS)

Flat Invasive Carcinoma

Question 136

This patient you just diagnose with a bladder tumor has one of the most common patterns of bladder tumors.

Who is at the highest risk for Urothelial Carcinoma?

What are the risk factors?

Answer 136

White Males at highest risk

Industrial, Urban people, smokers, Cyclophosphamide, Phenacetin

Question 137

You have a 54 year old woman who is diagnosed with uroethlial neoplasia. You note that she has a papillary lesion – what would you expect the patient to complain of?

What if she had a flat lesion?

Answer 137

Papillary – Hematuria is likely

Flat – Discomfort is likely

Question 138

You have a 70 year old male who presents with painless hematuria, and some minor dysuria. You notice the person has a cancer, and has multiple tumors. You notice that they have cytologic atypia – with hyperchromatic huge nuclei and a high N/C ratio.

What is the diagnoses?

What is most critical to staging in this type of cancer?

Answer 138

Urothelial Carcinoma – Papillary Carcinoma High Grade

Muscle Invasion (Depth)

Question 139

A 40 year old male comes into the ER with Squamous Cell Carcinoma of the male genital tract. He says that it has been there for a bout year, there is metastases to distant lymph nodes, but no other dissemination.

What part of the GU tract is commonly affected by this cancer?

What would anatomic/surgical distinguishing factor is associated with this?

Answer 139

Glans or Shaft of the Penis

Uncircumcised males

Question 140

A 1 year old boy is brought to your clinic with bilateral cryptorchidism. His parents are worried that he will never be able to have children, and request you fix their son.

What other anatomic problems could you expect to see on this boy?

What is the boy at higher risk for unless you perform an orchiopexy?

Answer 140

Hypospadias/Episadias

Testicular Cancer

Question 141

You have a 22 year old male that comes into your clinic complainging of pain in his genitals. He has epididymal abscesses, and suppurative orchitis.

What does he most likely have?

Answer 141

Gonorrhea

Question 142

A 21 year old male comes into your clinic with a swollen testicle, he has no other symptoms but is very worried.

What broad type of tumors do testicular cancers fall into?

What is the most likely tumor (by chance)?

what would you see on histology?

Answer 142

Germ Cell Tumors (95%) and Sex Cord-Stromal Tumors

(Germ Cell are further divided into Seminomas and Nonseminomatous)

Seminoma

an ill-defined granulomatous rxn

Question 143

A 21 year old male comes into your clinic with a swollen testicle, he has no other symptoms but is very worried.

You continue to exam him, and find out that he has a tumor arising from the cells that make his testosterone – what tumor is it now?

Answer 143

Leydig Cell Tumor

Question 144

A 21 year old male comes into your clinic with a swollen testicle, he has no other symptoms but is very worried.

You find that his histologic pattern is “Pure” meaning that it has only one element. What Tumors are on your differential?

If he had found to have a mixed histologic pattern, what tumors make the differential?

Answer 144

Pure: Seminoma, Embryonal Carcinoma, Yolk Sac Tumors, Choriocarcinoma, and Teratoma

Mixed: Emryonal Carcinoma, Teratoma, Yolk Sac Tumors

Question 145

A 21 year old male comes into your clinic with a swollen testicle, he has no other symptoms but is very worried.

If he is diagnosed with a non-seminamtous tumor what is the chance of spread?

Answer 145

Higher, due to spread by lymphatics and blood vessels – where as seminomatous rarely spread to paraaortic nodes.

Question 146

You have a 24 year old male that comes into the clinic with testicular complaints. You notice that his HCG is elevated.

What is the most likely tumor causing this?

If he had instead has an elevated AFP what would the tumor be?

Answer 146

Choriocarcinomas, or seminomas containing synctiotrophoblasts.

Yolk Sac Tumor

Question 147

You have a 65 year old male that comes into the ER with a testicular tumor. You realise that he has a Non-Germ Cell Tumor –

what is the most common testicular tumor in men older than 60?

Answer 147

Non-Hodgkin Lymphoma

Question 148

You have a a 55 year old man that complains of fever, chills, and dysuria after being catheterized. You do a urine culture and find gram negative rods. You do a rectal exam, and find a boggy and tender prostate.

What bacteria is most common?

What is the diagnoses?

Answer 148

E. Coli

Acute Bacterial Prostatitis

Question 149

You have a 53 year old man that has low back pain, dysruia, and perineal and suprapubic discomfort. he doesnt havea history of UTI, and there is negative bacterial cultures.

What is the most likely diagnoses?

If he had had UTIs and a bacterial culture – what would his diagnoses be?

Answer 149

Chronic Abacterial Prostatitis (most common)

Chronic Bacterial Prostatitis

Question 150

A 76 year old male comes into your clinic with complaints of hesitancy, urgency, and nocturia. He also states that his urinary stream is weak. You realize that something is affecting his inner periurethral zone of the prostate – and is compressing the urethra.

What is the diagnoses?

Answer 150

BPH

Question 151

You have a 72 year old African American male, that he has PCA3 noncoding RNA that is overexpressed, and deletions that activate the PI3K/AKT signaling pathway. You note that upon rectal exam he has a palpable peripheral gland.

What does he potentially have?

Answer 151

Prostate Cancer (Adenocarcinoma of the Prostate)