American journal of kidney diseases : the official journal of the National Kidney Foundation
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N-Terminal pro-B-type natriuretic peptide (NT-proBNP) level predicts underlying heart disease in the general population. However, because NT-proBNP clearance may depend on renal function, the significance of an elevated level in patients with chronic kidney disease (CKD) without cardiac symptoms is uncertain. We sought to determine whether there is an association between NT-proBNP level and coronary artery disease (CAD) and left ventricular hypertrophy (LVH) in this population. ⋯ NT-proBNP level elevation in asymptomatic patients with CKD reflects underlying ischemic heart disease and hypertrophy independent of renal function in a population with anticipated high cardiac morbidity.
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Fabry disease is a lysosomal storage disease resulting from deficient alpha-galactosidase A (alpha-Gal A) activity. End-stage renal disease generally occurs around the fourth decade of age, and dialysis therapy is a life-saving procedure. For patients with Fabry disease undergoing dialysis, death usually occurs from cardiac or cerebrovascular complications. Recently, enzyme replacement therapy was introduced for treatment of the disease. ⋯ Our observation indicates that in dialysis patients, enzyme replacement therapy is safe and effective, improving global quality of life and possibly ameliorating the progression of typical Fabry cardiomyopathy.
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Case Reports
Recurrent high anion gap metabolic acidosis secondary to 5-oxoproline (pyroglutamic acid).
High anion gap metabolic acidosis in adults is a severe metabolic disorder for which the primary organic acid usually is apparent by clinical history and standard laboratory testing. We report a case of recurrent high anion gap metabolic acidosis in a 48-year-old man who initially presented with anorexia and malaise. Physical examination was unrevealing. ⋯ Recently, there have been several reports of apparent acquired 5-oxoprolinuria and high anion gap metabolic acidosis in adults in association with acetaminophen use. Acetaminophen may, in susceptible individuals, disrupt regulation of the gamma-glutamyl cycle and result in excessive 5-oxoproline production. Suspicion for 5-oxoproline-associated high anion gap metabolic acidosis should be entertained when the cause of high anion gap metabolic acidosis remains poorly defined, the anion gap cannot be explained reasonably by measured organic acids, and there is concomitant acetaminophen use.
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Gabapentin is an antiepileptic medication that also has been used for restless legs syndrome. The mechanism of action is unknown. ⋯ Two patients on short daily hemodialysis therapy developed neuromuscular symptoms and an elevation in creatine kinase levels after starting gabapentin therapy. To our knowledge, this is the first case report of an increase in creatine kinase level after the administration of gabapentin.
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Cardiac surgery for carcinoid heart disease may be associated with acute renal failure (ARF) and multiorgan dysfunction postoperatively. This study was performed to determine the incidence, risk factors, and prognosis of ARF after cardiac surgery for carcinoid heart disease. ⋯ ARF developed in a relatively high proportion of this cohort, but traditional preoperative risks failed to predict post-cardiac surgery ARF. Variables closely related to perioperative hemodynamic compromise were associated with ARF. ARF portends a particularly poor prognosis in this cohort that is explained largely by multiorgan failure syndrome.