"Genetic testing is costly; therefore, the diagnosis is commonly made by a combination of family history and cyst detection. Advances in technology have enabled the identification of smaller cysts, and this has led to suggestions to modify the criteria, e.g. two or more cysts in each kidney in the US in an at-risk individual aged 30-40 years17. High-resolution T2 FSE is highly sensitive for subcentimetre cysts and more objective. Ten or more cysts in the 16-49 year age group were found to have a sensitivity and positive predictive value of 100% in at-risk individuals; a limit of five cysts can be used to identify suitable related renal donors 17."
"Ultimately, ESRF requiring transplant or dialysis eventually develops in many patients (45% by the age of 60). Patients with PKD1 mutations are more likely to progress to ESRF and often do so at an earlier age 1. Patients with a larger height-adjusted total kidney volume are at greater risk of ESRF and may benefit from vasopressin receptor antagonists, which slow cyst expansion16."
"T1: low signal"
"T2: high signal"
"presence of enhancement of a solid component or septa should raise the possibility of a renal cell carcinoma (RCC) (NB infected cysts may peripherally enhance, as do islands of trapped renal tissue). Subtraction MR may be helpful."
"Genetic testing is costly; therefore, the diagnosis is commonly made by a combination of family history and cyst detection. Advances in technology have enabled the identification of smaller cysts, and this has led to suggestions to modify the criteria, e.g. two or more cysts in each kidney in the US in an at-risk individual aged 30-40 years17. High-resolution T2 FSE is highly sensitive for subcentimetre cysts and more objective. Ten or more cysts in the 16-49 year age group were found to have a sensitivity and positive predictive value of 100% in at-risk individuals; a limit of five cysts can be used to identify suitable related renal donors 17."
"Genetic testing is costly; therefore, the diagnosis is commonly made by a combination of family history and cyst detection. Advances in technology have enabled the identification of smaller cysts, and this has led to suggestions to modify the criteria, e.g. two or more cysts in each kidney in the US in an at-risk individual aged 30-40 years17. High-resolution T2 FSE is highly sensitive for subcentimetre cysts and more objective. Ten or more cysts in the 16-49 year age group were found to have a sensitivity and positive predictive value of 100% in at-risk individuals; a limit of five cysts can be used to identify suitable related renal donors 17."
"less severe, ESRD at an average age of 74 years 16"
"consider using ACR group II ultra-low-risk contrast media to avoid nephrogenic systemic fibrosis"
"The risk of end-stage renal disease varies: PKD1 is associated with earlier ESKD (median ~50s), whereas PKD2 typically presents later (median ~70s) 23."
"contiguous gene syndrome: large deletions of chromosome 16 with overlapping features of APKD and TSC"
"contiguous gene syndrome: large deletions of chromosome 16 with overlapping features of APKD and TSC"
"PKD1"
"PKD2"
"GANAB (rare)"
"chromosome 11q13"
"simple cysts should not have any solid-enhancing components"
"The risk of end-stage renal disease varies: PKD1 is associated with earlier ESKD (median ~50s), whereas PKD2 typically presents later (median ~70s) 23."
Expected headings
"Associations"
"Complications"
"Genetic testing is costly; therefore, the diagnosis is commonly made by a combination of family history and cyst detection. Advances in technology have enabled the identification of smaller cysts, and this has led to suggestions to modify the criteria, e.g. two or more cysts in each kidney in the US in an at-risk individual aged 30-40 years17. High-resolution T2 FSE is highly sensitive for subcentimetre cysts and more objective. Ten or more cysts in the 16-49 year age group were found to have a sensitivity and positive predictive value of 100% in at-risk individuals; a limit of five cysts can be used to identify suitable related renal donors 17."
"Genetic testing is costly; therefore, the diagnosis is commonly made by a combination of family history and cyst detection. Advances in technology have enabled the identification of smaller cysts, and this has led to suggestions to modify the criteria, e.g. two or more cysts in each kidney in the US in an at-risk individual aged 30-40 years17. High-resolution T2 FSE is highly sensitive for subcentimetre cysts and more objective. Ten or more cysts in the 16-49 year age group were found to have a sensitivity and positive predictive value of 100% in at-risk individuals; a limit of five cysts can be used to identify suitable related renal donors 17."
"Decline in eGFR renal function often occurs later in the course of the disease; height-adjusted total kidney volume and the Mayo Imaging Classification provide strong prognostic information for risk stratification and treatment decisions 22."
"Plain films have no role in the surveillance of patients with established ADPKD. The diagnosis may be suspected when the renal outlines are enlarged, multilobulated or difficult to discern, with associated displacement of loops of bowel."
"Cysts may be detected in the liver, spleen, pancreas and seminal vesicles."
"A complex cystic mass with an enhancing solid component may indicate renal cell carcinoma (RCC). The Bosniak classification of renal cysts is of limited value in ADPKD. Subtraction CT, MRI or DECT are sensitive to enhancement."
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