renal: cystic disease

Cystic Diseases of the kidney:

Match the following:

A.      Cystic renal dysplasia

B.      ADPCKD

C.      ARPCKD

D.      Medullary sponge kidney

E.       Nephronophthisis

F.       Acquired cystic disease

G.     Simple cyst

        I.            Most common genetic cause of end stage renal disease in kids/young adults ____

      II.             Caused by abnormal gene on chromosome 16 –____

    III.            Caused by a mutation in PKHD1 gene (which encodes fibrocystin)- ___

    IV.            Cysts in the medulla are typically concentrated at the corticomedullary junction - ___

      V.            Failure in kidney development despite normally formed precursors- ___

    VI.            Likely caused by obstruction to drainage -___

  VII.            Cysts contain clear fluid and often calcium oxalate crystals –___

VIII.            Cysts are translucent and lined by single layer of cuboidal epithelium – these are a common postmortem finding -___

    IX.            Multiple cystic dilations of the collecting ducts in the medulla-___

      X.            Silent until adulthood when they classily present as: hematuria, HTN, abdominal mass - __

    XI.            Affected children present first with polyuria/polydypsia with terminal renal failure following in 5-10 years ___

  XII.            One of the variants of this disorder is associated with ocular lesions – ___

XIII.            Cortical and medullary cysts seen in patients following prolonged dialysis ___

XIV.            This disease affects the kidneys/liver in inverse proportions ___

  XV.            bilateral and symmetric enlargement but otherwise looks normal externally- but upon cutting the specimen there are multiple radially arranged cysts involving only the collecting ducts ___

XVI.            initially involves distal tubules with BM disruption which is followed by chronic and progressive tubular atrophy (medulla + cortex) à interstitial fibrosis à renal insufficiency ___

XVII.            Kidneys are massively enlarged with multiple variably sized cysts which may affect any area of the nephron ___

XVIII.            Histologically shows: arrested development with simple ducts and often cartilage ___

XIX.            Juvenile form is associated with NPH1,2,3 - ____

  XX.            20% of patients with this disease develop cerebral artery aneurysms ___