Renal Fanconi syndrome (RFS) is caused by generalized proximal tubular dysfunction and can be divided into hereditary and acquired form. Adult-onset RFS is usually associated with drug toxicity or systemic disorders, and modern molecular genetics may explain the etiology of previous idiopathic cases of RFS. Here, we report the case of a 52-year-old woman with RFS whose etiology could not be identified. She presented with features of phosphaturia, renal glucosuria, aminoaciduria, tubular proteinuria, and proximal renal tubular acidosis. Her family history was unremarkable, and previous medications were nonspecific. Her bone mineral density was compatible with osteoporosis, serum intact parathyroid hormone level was mildly elevated, and 25(OH) vitamin D level was insufficient. Her blood urea nitrogen and serum creatinine levels were 8.4 and 1.19 mg/dL, respectively (estimated glomerular filtration rate, 53 mL/min/1.73 m2). Percutaneous renal biopsy was performed but revealed no specific renal pathology, including mitochondrial morphology. No mutation was detected in EHHADH gene. We propose the possibility of involvement of other genes or molecules in this case of adult RFS.

• EHHADH gene
• Idiopathic Fanconi syndrome
• Osteoporosis
• Phosphaturia
• Renal tubular acidosis

A 22-year-old male patient was diagnosed with autosomal dominant polycystic kidney disease (ADPKD). He received conservative treatment with an angiotensin-converting enzyme inhibitor. Two years later, oral therapy, consisting of 60 mg tolvaptan per day, was initiated. Compared with height-adjusted total kidney volume, the rate of kidney growth reduced significantly from 7.33% to 0.66% annually, since commencement of the tolvaptan therapy. The liver enzyme profile and serum sodium level and osmolality were constantly within normal ranges. In Korea, this is the first reported case of a patient with ADPKD who received tolvaptan treatment for more than 1 year. This case demonstrates that long-term tolvaptan treatment appears to be safe, well tolerated, and effective for ADPKD.

• Autosomal dominant polycystic kidney disease
• Tolvaptan
• V2-antagonist
• Long-term outcome

Peritoneal dialysis (PD)-related peritonitis is a major cause of injury and technique failure in patients undergoing PD. Aeromonas hydrophila is ubiquitous in the environment, and is a Gram-negative rod associated with infections in fish and amphibians in most cases; however, it can also cause opportunistic infections in immunocompromised patients. We report a case of A. hydrophila peritonitis in a 56-year-old male on automated PD. Peritonitis may have been caused by contamination of the Set Plus, a component of the automated peritoneal dialysis device. Although Set Plus is disposable, the patient reused the product by cleansing with tap water. He was successfully treated with intraperitoneally-administered ceftazidime and has been well without recurrence for more than 2 years.

• Aeromonas hydrophila
• Peritoneal dialysis
• Peritonitis