Volume 1 No. 1, 2016
+ Volume 1, 2016
Issue 1
Insight Nephrology>>  Volume 1 Issue 1, 2016
Giant Symptomatic Polycystic Kidneys
Federico Conti , Stefano Di Bella and Andrea Gori
Abstract: Background: Autosomal Dominant Polycystic Kidney Disease (ADPKD) is one of the most common inherited kidney diseases and is the 4th commonest reason for dialysis. ADPKD is usually diagnosed in the 4th decade of life and is caused by mutations in genes PKD1 (80%) and PKD2 (20%). In the late stages ADPKD is characterised by fibrosis and inflammatory damage, which leads to complications and rapid progressive deterioration of the kidney. Methodology: Through an educational radiological imaging, a clinical case of a patient with giant polycystic kidneys who experienced an infectious complication prompting kidney removal was described. Results: Fifty nine years old woman with end-stage chronic kidney disease due to ADPKD was admitted presenting with left flank pain, fever, tachycardia, haematuria and dyspnea. Blood examination showed leukocytosis (46700 cells μL–1), elevation of inflammatory indexes and raised serum creatinine (8.9 mg dL–1). An abdominal CT scan showed kidneys 3 time bigger than normal (~30 cm each) and super-infected cysts. Severe sepsis with bacteremia from extended spectrum β-lactamase producing E. coli was diagnosed. The patient was managed with percutaneous drainage of the larger abscess (removing 1.2 L of pus) and intravenous meropenem. A bilateral nephrectomy was planned in order to prevent further infections and to reduce dyspnea due to diaphragm compression. Conclusion: Patients with ADPKD usually suffer from complications such as recurrent infections of the urinary tract, cyst haemorrhage and symptoms related to mass and nephrolithiasis. Although, treatments which prevent progression to the end-stage renal disease are not available, early detection and treatment of hypertension can prevent kidney damage. Total kidney volume may predict the future need for nephrectomy but may be particularly warranted if complications arise e.g., abdominal symptoms, recurrent infections, haemorrhage and cystic lesions suspected for neoplasm.
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Federico Conti, Stefano Di Bella and Andrea Gori , 2016. Giant Symptomatic Polycystic Kidneys. Insight Nephrology, 1: 1-2
DOI: 10.5567/NEPHROLOGY-IK.2016.1.2
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