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Fill in the Blanks: Understanding ARPKD

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Fill in the Blanks: Understanding ARPKD

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Test your knowledge on ARPKD with this engaging fill-in-the-blanks game!

Created by

pptzee
pptzee
India

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Fill in the Blanks: Understanding ARPKD
 

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Fill in the Blanks: Understanding ARPKDOnline version

Test your knowledge on ARPKD with this engaging fill-in-the-blanks game!

by pptzee
1

ADPKD collecting tubules PKHD1 renal recessive delivery autosomal infantile kidneys ARPKD glomeruli chromosome nephrons cysts

1 . is distinct from , occurring less frequently ( 1 : 20 , 000 births ) . 2 . It's an disorder , often without a family history . 3 . The disease is caused by mutations in the gene on 6 ( 6p21 ) . 4 . It invariably affects both . 5 . Presentation can be perinatal , neonatal , , or juvenile , with severe cases leading to early childhood death . 6 . Macroscopically , kidneys are bilaterally enlarged with a smooth surface and normal shape . 7 . Cut surfaces show small , fusiform or cylindrical radiating from the medulla to the cortex , giving a sponge - like appearance . 8 . Normal parenchyma is not grossly visible . 9 . The pelvis , calyces , and ureters are typically normal . 10 . Histologically , the total number of is normal . 11 . Cysts arise from dilated , lined by cuboidal to low columnar epithelium . 12 . Many are also cystically dilated . 13 . Clinical manifestations vary with age , with severe cases causing complications . 14 . Renal failure can manifest early in infancy . 15 . Almost all ARPKD cases have associated liver cysts or portal bile ductule proliferation , leading to congenital hepatic fibrosis , portal hypertension , and splenomegaly in older children .

 
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