Liver cyst infection in kidney transplant patient with autosomal dominant polycystic kidney disease: Interest of PET/CT in diagnosis and treatment
Published on: 14th December, 2018
OCLC Number/Unique Identifier: 7964794073
Liver cyst infection in patients with autosomal dominant polycystic kidney disease (ADPKD) is a rare but life-threatening complication. Diagnosis and treatment remain challenging. We report the case of a 64-year-old kidney transplant patient presenting with fever and abdominal pain. The diagnosis of liver cyst infection was made by positron emission tomography scan (PET/CT). Moreover, the course of our patient illustrates the interest of subsequent PET/CT during follow-up as our patient failed on antibiotherapy and required liver cyst drainage.
Potter Syndrome: A case study
Published on: 31st August, 2017
OCLC Number/Unique Identifier: 7286353788
Potter syndrome (PS) is a term used to describe a typical physical appearance, which is the result of dramatically decreased amniotic fluid volume secondary to renal diseases such as bilateral renal agenesis (BRA). Other causes are abstraction of the urinary tract, autosomal recessive polycystic kidney disease (ARPKD), autosomal dominant polycystic kidney disease (ADPKD) and renal hypoplasia. In 1946, Dr Edith Potter characterized this prenatal renal failure/renal agenesis and the resulting physical characteristics of the fetus/infant that result from oligohydramnios as well as the complete absence of amniotic fluid (anhydramnios). Oligohydramnios and anhydramnios can also be due to the result of leakage of amniotic fluid from rupturing of the amniotic membranes. The case reported below, concerns of stillborn boy with potter syndrome.
Kidney Biopsy in Autosomal Dominant Polycystic Kidney Disease
Published on: 19th December, 2023
Proteinuria is an easily quantified biomarker of kidney disease and often a sign of glomerular pathology. Significant proteinuria is uncommon in cystic kidney diseases and should be further evaluated to exclude the presence of another simultaneous kidney disease. While renal biopsy is a valuable part of the diagnostic evaluation of proteinuria, careful consideration of risks and benefits is necessary before proceeding in a patient with bilateral renal cysts. We report the case of a man with Polycystic Kidney Disease (PKD) who was found to have nephrotic-range proteinuria. An ultrasound-guided kidney biopsy revealed evidence of Focal Segmental Glomerulosclerosis (FSGS), which was attributed to hyperfiltration-related injury in the context of extensive kidney cysts. Genetic testing did not reveal a cause of FSGS and showed a variant of uncertain significance in PKD1. We use this case to highlight three important issues that are applicable to patients with PKD: the role of diagnostic evaluation for proteinuria in cystic kidney disease, the feasibility of kidney biopsy despite the presence of bilateral renal cysts, and the roles and limitations of genetic testing in cystic kidney disease and FSGS.
A Case of Good Efficacy of Tolvaptan in a Patient with Markedly Enlarged Polycystic Kidney
Published on: 20th August, 2024
A 61-year-old patient with autosomal dominant polycystic kidney disease (Irazabal class 1E) in whom renal function had decreased and kidney size had increased over the past 3 years (change in serum creatinine, 1.3 mg/dL to 1.5 mg/dL; change in total kidney volume, 5632 cm3 to 7301 cm3) was treated with tolvaptan 60 mg/day. After 8 years of tolvaptan treatment, serum creatinine remained at 1.51 mg/dL, and total kidney volume was at 6812 cm3. Adequate salt intake and good weight control may have resulted in good outcomes, even in patients with treatment-resistant giant cystic kidney disease.
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