Renal cyst Flashcards
What is a cyst?
It is a sac filled with fluid
What are the characteristics of all cystic diseases?
1) All cysts are lined with cuboidal epithelium
2) Some cysts enlarge the kidney, while others do not affect the size of the kidney, and some shrink the kidney
3) Knowing the site of the cyst (Medullary or Cortical) is important as it can help us with the diagnosis
4) The age of the patient when renal failure occurred is very important as some diseases come in the 20s and 30s while other 40s/50s
What are the conditions that are associated with cysts?
- Asymptomatic
- Patients on dialysis
- Carcinoma
- Neonatal death
- Renal failure (at which age?)
What are the cortical and medullary cystic diseases?
1) Infantile polycystic kidney disease
2) Adult polycystic kidney disease (4-5th decade in patients with renal failure)
3) Renal dysplasia (multicystic renal dysplasia)
4) Acquired cystic disease
5) Medullary sponge disease
6) Adult-onset medullary cystic disease (nephronophthisis) (1-2nd decade in patients with renal failure)
What is infantile polycystic kidney disease?
- Autosomal recessive disease
- Causes stillbirth (death of the fetus after 20 weeks) or early death
- It is due to PKHD1 Mutation (remember that boss baby had a “PK HD”hahaha)
- It massively enlarges the kidneys via the small cysts in the cortex and the medulla, the cysts grow perpendicularly to the renal capsule
- The cyst is lined by cuboidal cells
- Extrarenal manifestations the congenital hepatic fibrosis in patients who survive infancy
What type of mutation if the infantile polycystic kidney disease?
Autosomal recessive
What is the cause of infantile polycystic kidney disease?
Mutations in PKHD1
Which parts of the kidney is involved in polycystic kidney disease?
The cortex and the medulla
What are the extrarenal manifestations of infertile polycystic kidney disease?
Congenital hepatic fibrosis in patients who survive infancy
What is the adult polycystic kidney disease?
- Autosomal dominant
- Usually occurs in the 4-5th decade with renal failure
- It is caused by two mutations (PKD1 & PKD2)
- Early few cysts in the cortex and medulla, which massively enlarges the kidney
- The cysts are lined by cuboidal epithelium, and there are signs of inflammation and damage (intervening kidney tissue: interstitial fibrosis, lymphocytic infiltration, tubular atrophy)
- Extrahepatic manifestations: Intracranial berry aneurysms, pancreatic and hepatic cysts, mitral valve prolapse
What type of mutation if the adult polycystic kidney disease?
Autosomal dominant
What is the cause of adult polycystic kidney disease?
- PKD1 Mutation
- PKD2 Mutation
In which part of the kidney does the adult polycystic kidney disease?
In the cortex and medulla
What are the extrahepatic manifestations of the adult polycystic kidney disease?
- Intracranial berry aneurysm
- Pancreatic & hepatic cysts
- Mitral valve prolapse
What is renal dysplasia (multicystic renal dysplasia)?
- Dysplasia means the disorganized proliferation of the cells acquiring some malignant features
- The most common pediatric cystic renal disease
- It can occur unilaterally or bilaterally
- It enlarges the kidney
- Microscopically (Blue tube CCs):
- Disorganized kidney parenchyma
- Cysts are lined by cuboidal epithelium
- Cartilage is present
- Immature tubules, which are surrounded by blastemal cella (undifferentiated cells)
- MNEMONIC for the microscopic photos (Blue “blastema cells” tube “immature tubules” C “cysts” C “cartilage”)
What is the most common pediatric cystic renal disease?
Renal Dysplasia (multicystic renal dysplasia)
In which part of the kidney does the renal dysplasia (multicystic renal dysplasia) occur?
Cystic, it enlarges the kidney
What is the microscopic photo of renal dysplasia (multicystic renal dysplasia)?
Microscopically (Blue tube CCs):
- Disorganized kidney parenchyma
- Cysts are lined by cuboidal epithelium
- Cartilage is present
- Immature tubules, which are surrounded by blastemal cella (undifferentiated cells)
- MNEMONIC for the microscopic photos (Blue “blastema cells” tube “immature tubules” C “cysts” C “cartilage”)
What is the acquired cystic disease?
- Occurs in patients with hemodynamics or with chronic renal failure
- Asymptomatic
- Cortical cysts, and might contain calcium oxalate crystals
- The crystals are lined by atypical, hyperplastic epithelium which can undergo malignant transformation
- It might become malignant
Acquired cystic disease occurs in which individuals?
1) Patients on hemodialysis
2) Patients with chronic renal failure
In which part of the kidney do the cysts of the acquired cystic disease occur?
Cortical, and the cysts might contain calcium oxalate crystals
The cysts in the acquired cystic disease is lined by which type of cells?
It is the only one lined with Atypical hyperplastic epithelium
What is the medullary sponge disease?
- Occurs in children or adolescents
- Kidneys are normal in size
- Small cysts are found at the medullary pyramids and papillae
- The cysts are lined by cuboidal epithelium
What is the size of the kidney in the medullary sponge disease?
They are normal in size and it is the only cyst that occurs in the medulla and keep the size of the kidney normal
