Cystinosis is an autosomal recessive disease caused by a mutation in CTNS, which encodes the lysosomal transporter of cystine. Without this, cystine gradually accumulates in cells causing progressive damage. The commonest kind is “nephropathic cystinosis” which is the…
Last year, Matt wrote a great post detailing the preliminary results of a randomized, controlled trial that had been presented at the ASN in November. It was reported that the anti-inflammatory modulator, Bardoxolone, significantly increased eGFR, in a…
We were recently consulted on a patient with a history of repeated admissions with pneumonia who had developed AKI following treatment with Tobramycin. It got me thinking about the mechanisms of aminoglycoside toxicity. The traditional teaching is that…
A young patient was recently transferred to our service for evaluation of acute renal failure that had developed many years following a liver transplant for PSC. She had presented to another institution with nausea, vomiting, fevers and a…
We were recently consulted on a patient with a history of multiple myeloma who had developed severe hypercalcemia and acute renal failure. The calcium on admission was 15mg/dl (albumin 2.6) and the patient was treated aggressively with IV…
Imipenem is a beta-lactam antibiotic that has a wide spectrum of activity against gram negative and gram positive organisms and is commonly used in an ICU setting. It is always given in combination with Cilastatin and in most…
Should patients with nephrotic syndrome (NS) be anticoagulated? Is there any level of proteinuria or albumin at which we should be especially concerned? The rates of venous thromboembolism (VTE) have ranged from 8-44% in observational studies and it…
Cutting back your salt intake is an effective way to reduce systolic and diastolic blood pressure and decrease your risk of dying from cardiovascular disease. The current guidelines suggest that we should reduce our dietary sodium intake to…
As mentioned previously by Nate, there is some evidence that lowering urine osmolarity below that of the serum can reduce the rate of growth of cysts in PCKD and thus preserve renal function. The rationale for this is…
Spare a thought today for Ronald Lee Herrick who died on Monday at age 79. In 1954, his identical twin brother, Richard, had end-stage kidney disease with little prospect of long-term survival. Dr Joseph Murray, a pioneering surgeon…
