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Autosomal dominant polycystic kidney disease with ectopic unilateral multicystic kidney: a case report
Yaw Amoah1, Mathew Yamoah Kyei2,3, James Edward Mensah2,3
1Urology Unit, Department of Surgery, Korle-Bu Teaching Hospital, Accra, Ghana. amoah2015@yahoo.com.
Insights
Autosomal dominant polycystic kidney disease (ADPKD) is a common genetic disorder. This case highlights a rare instance of ADPKD with an ectopic pelvic kidney, potentially accelerating kidney function decline.
Area of Science:
- Nephrology
- Medical Genetics
- Urology
Background:
- Autosomal dominant polycystic kidney disease (ADPKD) is the most prevalent hereditary kidney disorder and a leading cause of end-stage renal disease.
- ADPKD affects 1:400-1:1000 individuals, contributing to 10% of dialysis patients.
- Typically, ADPKD involves bilateral, similarly affected kidneys with numerous cysts.
Observation:
- A 46-year-old male presented with flank pain, hematuria, hypertension, and impaired renal function.
- Imaging revealed enlarged kidneys with multiple cysts; the right kidney was ectopically located in the pelvis.
- Diagnosis: ADPKD with a right pelvic ectopic multicystic kidney.
Findings:
- Ectopic kidneys in ADPKD are rare, with only a few cases documented.
- The presence of an ectopic, particularly dysplastic, kidney may correlate with accelerated kidney function deterioration.
- This case adds to the limited literature on ADPKD with ectopic renal anomalies.
Implications:
- Understanding the impact of ectopic kidneys on ADPKD progression is crucial for patient management.
- Further research is needed to elucidate the mechanisms behind accelerated decline in such cases.
- This case underscores the importance of comprehensive imaging in diagnosing and managing ADPKD variations.
Background:
Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disorder and the fourth cause of death of end-stage renal disease. The disease has a prevalence of 1:400-1:1000 accounting for 10% of patients on dialysis. In most ADPKD patients, bilateral kidneys are similarly affected, with numerous fluid-filled cysts arising from different nephron segments. Only a few cases of ADPKD with ectopic unilateral multicystic kidney have been reported. It has been observed that the deterioration of their kidney function seemed to be quicker than their age- and sex-matched controls and siblings especially when the ectopic kidney is dysplastic.
Case Presentation:
We report a case of a 46-year-old Ghanaian male patient who presented with left flank pain and hematuria with high BP and deranged renal function. Abdominal ultrasonography showed both kidneys to be larger than normal and had multiple cysts of varying sizes with the right kidney located in the right iliac fossa. Follow up Abdominopelvic computer tomographic scan (CT-Scan) without contrast showed enlarged kidneys with the renal parenchyma replaced by innumerable cyst of varying sizes. The right kidney was ectopically located in the right aspect of the pelvis. A diagnosis of ADPKD with right pelvic ectopic multicystic kidney was made. He was put on antihypertensives, analgesia for the left flank pain and to have follow up at the urology and nephrology departments.
Conclusion:
In most ADPKD patients, bilateral kidneys are similarly affected. Only a few cases of ADPKD with ectopic unilateral multicystic kidney have been reported. It has been observed that the deterioration of their kidney function seemed to be quicker than their age- and sex-matched controls and siblings especially when the ectopic kidney is dysplastic.
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