28 Questions
What is a common pathogenic factor in the renal pathology of malignant hypertension and thrombotic microangiopathies?
Endothelial injury
In cases of malignant hypertension, what percentage of cases may present with microangiopathic hemolytic anemia?
30%
What is the primary cause of hypertension secondary to renal artery stenosis?
Increased renin production
Which experiments demonstrated that constriction of one renal artery in dogs leads to hypertension?
Goldblatt's experiments
What effect does the reduction of angiotensin II activity have on individuals with renovascular hypertension?
Reduction of blood pressure
Which factor contributes to the maintenance of renovascular hypertension after the renin-angiotensin system has initiated it?
Sodium retention
Where is the PKD1 gene located?
Chromosome 16p13.3
What is the function of polycystin-1?
Regulating cell-cell and cell-matrix interactions
What percentage of cases do mutations in PKD1 account for?
85%
At what age does the likelihood of developing renal failure reach more than 70% for individuals with mutations in PKD1?
70 years
Which type of cells express polycystin-1 predominantly?
Tubular epithelial cells
Why has the genetic diagnosis of Polycystic Kidney Disease been complicated?
Because of the variety of mutations in PKD1 and PKD2
What is one of the reasons why renal cysts are considered important?
They are common and often pose diagnostic challenges.
What is a major consequence of adult polycystic kidney disease?
It causes the destruction of the renal parenchyma.
How common is autosomal dominant polycystic kidney disease among live births?
1 in 400 to 1000
In autosomal dominant polycystic kidney disease, how many alleles of the involved genes need to be nonfunctional for the disease to develop?
Both alleles need to be functional.
Why are unilateral cases of adult polycystic kidney disease rare?
Because it is typically bilateral.
What is the inheritance pattern of autosomal dominant polycystic kidney disease?
(Autosomal) Dominant
What is the role of the VHL gene in clear cell cancers?
Acts as a tumor suppressor gene
What happens to HIF-1 levels when VHL is inactive?
They remain high
Which transcription factor is targeted by the VHL protein for degradation?
HIF-1
What is the consequence of high HIF-1 levels under normoxic conditions?
Increased expression of growth-promoting genes
What is the key pathogenic abnormality in most cases of atypical HUS?
Activation of complement due to inherited mutations
What is the histologic appearance of diffuse cortical necrosis?
Acute ischemic infarction
What toxin is responsible for producing platelet activation and thrombosis in typical HUS?
Shiga-like toxin
What type of necrosis is predominantly seen in massive ischemic necrosis affecting the renal cortex?
Coagulative necrosis
Which factor permits the formation of abnormally large multimers of vWF in TTP?
ADAMTS13 deficiency
What are the key characteristics of thrombotic microangiopathy?
Deposition of thrombi, red cell hemolysis, thrombocytopenia
Test your knowledge about renal lesions associated with malignant hypertension, including the overlap with thrombotic microangiopathies and microangiopathic hemolytic anemia. Learn about the connection between severe hypertension and primary forms of hemolytic uremic syndrome.
Make Your Own Quizzes and Flashcards
Convert your notes into interactive study material.
Get started for free
