Polycystic Kidney Disorder

Question 1

1.

A 25-year-old male underwent bilateral nephrectomy.

Q1. On the light of the picture given, what’s the diagnosis?

Answer 1

PKD

Question 2

Q2. What’s the mode of inheritance of polycystic kidney disease & what
other organs could be affected as well?

Answer 2

AD
Liver / Pancreas/Ovary / Brain / Intestine / Spleen / Testis

Question 3

Q3. What complication of pathology is not seen in this picture?

Answer 3

Pt caould have HTN / Renal Failure / Infection

Question 4

Q4. What complications of pathology can lead to pain?

Answer 4

Due to stretch on the capsule by the wt of the cyct and also dragging of the pedicle/ also due to infection and hge of the cyst.

Question 5

Q5, What’re the causcs of renal failurc in PCKD?

Answer 5

Substitution of most of kidney parenchyma by cysts which is not functioning and also due to damging of alveoli by fibrosis

Question 6

Q6. What’s the type of fluid contained within the cysts?

Answer 6

Serous fluid contain water and NACL

Question 7

Q7. What’re pathological types of transplant rejections?

Answer 7

Hyperacute / Acute / Chronic

Question 8

Q8. What’re the type of matching required before transplant?

Answer 8

ABO computability, HLA type A , B , DR

Question 9

Q. What’s HLA ag, types?

Answer 9

Human leukocytes ag
Types; A , B, DR

Question 10

Q10. How to prevent transplant rejcction in this situation?
Q9. Can a non-perfectly matched kidney be transplanted?

Answer 10

Yes
..
By giving immunosuppressants

Question 11

Seven days After transplant, patient developed swelling at the opcrative site with declining renal function.

Q11. What do you think is the cause is? What are cellular types of rejection? What’s the immunological response behind it?

Answer 11

Acute rejection
cells are B lymphocytes / T lymphocytes (CD 4 – CD8)
Type 2 hypersensitivity (Ab mediated)

Question 12

Four months later he came with CBD sones, fever & sepsis.
Q12. What’s the diagnosis?

Q13. What’s the management needed?

Answer 12

Ascending cholangitis
..
Sepsis 6 protocol
Give O2/Fluid/Iv Abx
Take Culture / Lactate / UOP monitor
then ERCP to decompresses and evacuate the stone

Question 13

Q13. ERCP done , but he developed sepsis. What’re the possible risk factors of bacterial infection in this patient?

Answer 13

The previous stasis due to obstruction
the instrumentation
the pt is immunosuppressed as he got organ transplant

Question 14

Q14. What’s the cause of CBD stones in this patient?

Answer 14

Direct relation btw PKD and CBD disease
Infection / Dehydration / missed stones / Immunosuppressant status
some drugs as cyclosporine

Question 15

Q15. Cholecystectomy done & GB send for histopathological examination. What to look for?

Answer 15

Look for inflammatory changes to check for inflammatory cells
look for neoplastic changes to confirm the neoplasm, the original site and also differentiation of the neoplasm

Question 16

Patient comes 6 years after transplant on immunosuppression with declined renal function. Biopsy shows lymphocytic infiltration with blast cells.
Q16. What’s the diagnosis?

Answer 16

Post-transplant lymphoproliferative disorder PTLD

Question 17

Q17. Why is it not rejection?

Answer 17

As there’s lymphocytic infiltration with blast cells

Question 18

Q18. How to treat Post transplant lymphoproliferative disorder?

Answer 18

By immunosuppressant drugs (change type or later the dose) and also anti-CD20 (retoximab)

Question 19

Q19. What’re the causative factor for “PTLD” Post transplant lymphoproliferative disorder? What are the cellular features of PTLD?

Answer 19

Opportunistic infection as CMV and EBV
cellular features;
1-early lesions:◾️reactive lymphoplasmocytic hyperplasia◾️Infectious mononucleosis like lesions
2- Polymorphic PTLD
3- Monomorphic PTLD
4-Classical hodgkin lymphoma and hodgkin lymphoma like PTLD