Mock5 PCK
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Stem 1
A 34-year-old male underwent with asthma and hypertension, referred with backpain, CT of abdomen showed bilateral enlarged Kidney of 25cmOn the light of the picture given, what's the diagnosis?
Enlarged kidney, diffusely enlarged with Multiple cysts variable size, adult Polycystic Kidney Disease (APKD)
Other organs in the abdomen causing cyst formation?
Liver, pancreas and spleen
Other pathology associated with PCKD?
Berry aneurysm associated with risk of SAH.
Adult Polycystic kidney disease is normally associated with deterioration in creatinine and urea levels, can you explain why this occurs in terms of the kidney tissue?
The cystic changes in kidney tubules will progress will lead to loss of function and obstruction of other renal tubules
There is associated fibrosis and some glomerular loss, all of this will deteriorate normal Renal function causing Renal failureList 3 complications of adult polycystic kidney disease?
Hemorrhage
Renal Cell carcinoma
InfectionFollowing bilateral nephrectomy, the patient is maintained by hemodialysis and placed on the transplant list, what determine if a potential kidney donor is suitable?
Blood group antigens
HLA antigensCan a non-perfectly matched kidney be transplanted?
Yes, with enhanced induction and maintained of Immunotherapy.
After transplant, the patient developed fever, tiredness with declining renal function what's your pathology?
Kidney rejection
What are the 2 types of rejection generally recognized from this data on immunity basis?
Cellular rejection
Humoral antibody rejectionHis rejection was treated and maintained on immunotherapy. After 6 years, general malaise is developed renal biopsy shows diffuse infiltration of kidney parenchyma with high grade lymphocyte cells, with B-cell CD20 with scattered mitosis.
What does this suggest?Lymphoma
Post-transplant lymphoproliferative disorderWhy is this unlikely to be a cellular rejection process?
The histology sounds to be lymphoma because of B cells
Cellular rejection will typically show infiltration of T cells CD3What is the likely driver of PTLD?
EBV genome expression in the context of immunosuppression.
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Stem 2
34-year-old man with a previous history of asthma and HTN is referred with back pain. He undergoes a CT TAP which shows bilateral enlarged kidneys (measuring about 21 cm)
Stem 3
A 25-year-old male underwent bilateral nephrectomy.In light of the picture given, what's the diagnosis?
Polycystic Kidney Disease (APKD)
What's the mode of inheritance of polycystic kidney disease?
Autosomal dominant condition due to mutations in 2 genes: PKD1, PKD2
What other organs could be affected as well?
Liver
Ovaries
Pancreas
Spleen
IntestinesOther pathology associated with PCKD?
Brain aneurysm
Mitral valve prolapse
Liver cysts
Pancreatic cystWhat complication of pathology is not seen in this picture?
Infection
Hypertension
Renal failure
PainWhat complications of pathology can lead to pain?
Weight of the organ dragging upon its pedicle or stretching of renal capsule by cysts
Infection
HaemorrhageWhat are the causes of renal failure in PCKD?
Due to replacement of normal kidney tissue by cysts with infection leading to scaring
What's the type of fluid contained within the cysts?
Trans-epithelial secretion
What are pathological types of transplant rejections?
Hyperacute
Accelerated acute
Acute
ChronicWhat are the types of matching required before transplant?
ABO and HLA human leucocytic antigen (HLA-A, HLA-B, HLA-DR).
Can a non-perfectly matched kidney be transplanted?
Yes
How to prevent transplant rejection?
Use immunosuppression
Seven days after transplant, patient developed swelling at the operative site with declining renal function.
What do you think is the cause is?Acute rejection
InfectionFour months later he came with CBD stones, fever & sepsis.
What's the diagnosis?Ascending cholangitis
What is the management needed?
I will manage this patient by ABCDE approach, but most important management is to relieve the obstruction by ERCP.
ERCP done, but he developed sepsis. What are the possible causes of bacterial infection in this patient?
Because of the obstruction
If the patient developed fever, jaundice and sepsis. What is the diagnosis?
Ascending cholangitis
Risk factors for ascending cholangitis in this patient?
Immunosuppression.
Taking steroids
Obstruction by stone in CBD
IatrogenicWhat's the cause of CBD stones in this patient?
Secondary hyperparathyroidism
Cholecystectomy done & GB send for histopathological examination. What to look for?
Macroscopic for any ulcerations or gangrene
Microscopic for any malignant or inflammatory cellsPatient comes 6 years after transplant on immunosuppression with declined renal function. Biopsy shows lymphocytic infiltration with blast cells.
What's the diagnosis?LPD (lymphoproliferative disease)
Why is it not rejection?
The presence of blast cells its characteristic for PTLD
How to treat Post transplant lymphoproliferative disorder?
Decrease immunosuppressive therapy dose
Change the type of immunosuppressive therapyWhat are the causative factors for PTLD (Post transplant lymphoproliferative disorder)?
Opportunistic viral infection by CMV and EBV due to immunosuppression.
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