PMC3619039_01
Chest X-rays of patient 1 (A) and patient 2 (C) revealed dextracardia. (B) Abdominal CT scan of patient 1 shows situs inversus of all the abdominal organs and bilateral kidney cysts. Note small numbers of cysts, small kidneys, and absence of cysts in liver. (D) Abdominal CT scan of patient 2 revealed situs inversus totalis and multiple bilateral renal and hepatic cysts
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A chest X-ray revealed dextracardia and cardiomegaly (Figure 1A). Abdominal CT scan showed situs inversus totalis of all the abdominal organs and bilateral kidney cysts, but the number and sizes of the cysts were small (Figure 1B). A chest X-ray (Figure 1C) and an abdominal CT scan (Figure 1D) revealed situs inversus totalis and bilateral multiple renal and hepatic cysts.
Macroscopic images of autopsy findings of patient 2. Bilateral kidneys are enlarged and filled with numerous cysts (A - left kidney, B - right kidney). Many cysts exist in left-right reversed liver too (C). There are no cysts in the pancreas, and the pancreatic tail is atrophic due to marked fat infiltration (D).
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Figures 2 and 3 shows the macroscopic and microscopic findings of the postmortem autopsy of patient 2.
Microscopic images of autopsy findings of patient 2. (A) Kidneys are filled with cysts of various sizes, among which atrophic and dilated tubules and calcified veins ( ) persist. (B) Only a few glomeruli remain with no signs of glomerulonephritis.. Figure 3C-E. Microscopic images of autopsy findings of patient 2. (C) Liver has variously sized cysts consisting of a layer of biliary epithelium. Among the cysts there are many biliary micro hamartomas (arrow) and a subtle fibrous interstitium. (D) Bronchiectasis is present in the lungs. (E) Pancreas has mild fibrotic change and atrophy of lobules.
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Figures 2 and 3 shows the macroscopic and microscopic findings of the postmortem autopsy of patient 2.
Cilia staining of patient 2 (A) and ESRD patient due to noncystic kidney disease (B) and ESRD patient receiving HD due to ADPKD (C). In patient 2 and the ADPKD patient, decreased and shortened primary cilia were observed compared with the non-cystic kidney disease patient.
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Figure 4 shows cilia staining of patient 2 kidney (Figure 4A) along with that of an ESRD patient due to noncystic kidney disease (Figure 4B) and ESRD patients receiving hemodialysis due to ADPKD (Figure 4C).