scholarly journals Type IV choledochal cyst with polycystic kidney disease: a case report

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Yuxin He ◽  
Zhuwen Yu ◽  
Weichang Chen

Abstract Background Choledochal cysts are divided into 5 types. Physicians believe that Caroli disease (which refers to type V biliary cysts) is a special type of biliary cyst caused by a mutation in the PKHD1 gene and is associated with autosomal recessive polycystic kidney disease (ARPKD). There is currently no clear association between other types of choledochal cysts and polycystic kidney disease. Case presentation We report a 65-year-old male patient with jaundice, decreased appetite, and itchy skin. His biochemistry test results indicated obstructive jaundice disease. Cross-sectional imaging showed a type IVA choledochal cyst accompanied by autosomal dominant polycystic kidney disease (ADPKD). Due to economic difficulties, the patient achieved percutaneous transhepatic cholangial drainage (PTCD) instead of surgery. Conclusion To our knowledge, this is the second case report of the coexistence of type IVA choledochal cysts and ADPKD. We conclude that it is vital to be aware that the above condition is a possibility. This case report will aid earlier diagnosis and management and possibly prevent further damage to liver and kidney function.

2019 ◽  
Vol 44 (6) ◽  
pp. 1416-1422 ◽  
Author(s):  
Magda Fliszkiewicz ◽  
Mariusz Niemczyk ◽  
Andrzej Kulesza ◽  
Anna Łabuś ◽  
Leszek Pączek

Introduction: Autosomal dominant polycystic kidney disease (ADPKD) is the most prevalent monogenic renal disease with a prevalence of 1:1,000 births and it is the 4th most common cause of dialysis-dependent end-stage renal disease (ESDR). Recent reports suggest an association between APDKD and metabolic derangements, particularly impaired glucose metabolism. Methods: In this cross-sectional study we analyzed data obtained from case records of 189 patients with ADPKD, including kidney transplant recipients, managed in an outpatient department. Results: The mean BMI was 25.4 ± 3.9; 25.25 before and 27.7 after transplan­tation. A fasting glucose level above 100 mg/dL (5.6 mmol/L) was observed in 60 patients (29%) – 27% without transplantation and 41% kidney transplant recipients. Diabetes mellitus was diagnosed in 17 patients (8.9%), including 3 (2.3%) without a history of transplantation and 14 (24.1%) after kidney transplantation (p < 0.01). We observed dyslipidemia in 30% and hyperuricemia in 53% of patients. Conclusion: Demonstrated metabolic abnormalities should be considered in maintenance of ADPKD patients, including kidney transplant recipients.


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