Randy L. Luciano

Extra-renal manifestations of autosomal dominant polycystic kidney disease (ADPKD): considerations for routine screening and management

Autosomal-dominant polycystic kidney disease (ADPKD) is a systemic disease, marked by progressive increase of bilateral renal cysts, resulting in chronic kidney disease (CKD) and often leading to end-stage renal disease (ESRD). Apart from renal cysts, patients often have extra-renal disease, involving the liver, heart and vasculature. Other less common but equally important extra-renal manifestations of ADPKD include diverticular disease, hernias, male infertility and pain. Extra-renal disease burden is often asymptomatic, but may result in increased morbidity and mortality. If the disease burden is significant, screening may prove beneficial. We review the rationale for current screening recommendations and propose some guidelines for screening and management of ADPKD patients.

Bile Acid Nephropathy in a Bodybuilder Abusing an Anabolic Androgenic Steroid

Bile acid nephropathy, also known as cholemic nephrosis or nephropathy, is an entity that can be seen in patients with severe cholestatic liver disease. It typically is associated with acute kidney injury (AKI) with various forms of hepatic disease. Most often, patients with severe obstructive jaundice develop this lesion, which is thought to occur due to direct bile acid injury to tubular cells, as well as obstructing bile acid casts. Patients with end-stage liver disease also can develop AKI, in which case a more heterogeneous lesion occurs that includes hepatorenal syndrome and acute tubular injury/necrosis. In this circumstance, acute tubular injury develops from a combination of hemodynamic changes with some contribution from direct bile acid-related tubular toxicity and obstructive bile casts. We present a case of AKI due to bile acid nephropathy in a bodybuilder who developed severe cholestatic liver disease in the setting of anabolic androgenic steroid use.

Kidney Involvement in Leukemia and Lymphoma

Leukemia and lymphoma are hematologic malignancies that can affect any age group. Disease can be aggressive or indolent, often with multiorgan system involvement. Kidney involvement in leukemia and lymphoma can be quite extensive. Acute kidney injury (AKI) is quite prevalent in these patients, with prerenal and acute tubular necrosis being the most common etiologies. However other prerenal, intrinsic, and obstructive etiologies are possible. AKI can be a direct effect of the malignancy, a complication of the malignancy, or the consequence or side effect of chemotherapy. Nephrotic syndrome and glomerulonephritis, often presenting without overt kidney failure, have also been seen in all forms of leukemia and lymphoma. Lastly, the direct effects of the malignancy and complications from the tumor often result in numerous electrolyte disturbances and acid-base disorders, with life-threatening consequences if left untreated.

Review of select causes of drug-induced AKI

Drug-induced acute kidney injury (AKI) is an important problem that is frequently encountered by clinicians [1]. Both prescribed and over-the-counter agents have the potential to injure all renal c...

Warfarin-related nephropathy in a patient with mild IgA nephropathy on dabigatran and aspirin

Dabigatran is a direct thrombin inhibitor used as an alternative to warfarin for long-term anticoagulation. We describe a patient who developed acute kidney injury (AKI) in the setting of warfarin conversion to dabigatran, and a renal biopsy demonstrating acute tubular injury. Although the patient had undiagnosed IgA nephropathy that may have predisposed him to bleeding, AKI was due to heme-associated tubular injury. We propose that severe hematuria in patients with underlying glomerular pathology treated with either dabigatran or warfarin may lead to toxic tubular injury through the accumulation of heme-proteins.

Crystalline-induced kidney disease: a case for urine microscopy

Urine microscopy is an integral part of the clinical evaluation of patients presenting with kidney disease [1, 2]. Along with history and physical examination, directed serum tests, dipstick urinalysis, and at times, renal imaging, urine sediment examination provides an informative view into the kidney. Identification and quantification of the cells, casts and crystals present in the spun urine sediment allow the clinician to synthesize all of the data and construct a rational diagnosis. This is particularly true for patients with crystalline-induced kidney disease [3]. We present illustrative cases of five different and clinically important causes of crystalline-induced kidney injury demonstrating the diagnostic utility of urine microscopy in clinching the diagnosis and facilitating therapy of the underlying process.

Comparison of amyloid deposition in human kidney biopsies as predictor of poor patient outcome

BackgroundAmyloidosis leads to deposition of abnormal protein with beta-pleated sheet structure in specific compartments of the affected organs. The histological localization of these amyloid deposits determines the overall survival of the patient.MethodsIn this study we have assessed the histological localization and severity of amyloid deposition in 35 patients with biopsy-proven renal amyloidosis and have compared those to clinical parameters, histo-pathological injury criteria and respective patient outcome. Comparisons were statistically analyzed using thus comparison between the different study groups, which was done using Student t-test and analysis of variance.ResultsWe find that the glomerulus is by far the most commonly and most severely affected renal compartment and patients with severe glomerular amyloidosis advance faster towards end stage renal disease (ESRD) and death, compared to those patients without glomerular amyloid deposits. Patients with severe glomerular amyloidosis showed higher serum creatinine and urine protein levels, while patients with severe vascular amyloidosis showed higher levels of interstitial inflammatory infiltrate.ConclusionIn kidneys affected by amyloidosis, the amyloid proteins are predominantly deposited along vessels, especially the small vessels including glomerular capillary loops. The severity of glomerular amyloid deposition enhances the risk of developing ESRD and increases the risk for premature death.

Acute Kidney Injury From Cherry Concentrate in a Patient With CKD

Nutraceuticals are supplements and medical foods that offer numerous health benefits. However, these substances may have adverse effects on multiple organ systems, leading to significant morbidity. I present a patient with chronic kidney disease who experienced hemodynamically mediated acute kidney injury and hyperkalemia after daily consumption of cherry concentrate. The method of injury was most likely cyclooxygenase inhibition by the compounds in cherries that mimic the mechanism of action of nonsteroidal anti-inflammatory medications. Ceasing cherry concentrate consumption led to improvements in both the patients hyperkalemia and kidney injury. Physicians should be aware of the potentially harmful side effects of cherry concentrate and approach the use of cherry extract or concentrate with caution in patients with underlying kidney disease.

Light chain crystalline kidney disease: diagnostic urine microscopy as the "liquid kidney biopsy".

Multiple myeloma (MM) is a plasma cell disorder, which often causes parenchymal kidney disease. Light chain (LC) cast nephropathy represents the most common renal lesion. In some instances, LC crystals precipitate within renal tubular lumens and deposit within proximal tubular cell cytoplasms. Importantly, urine microscopy in such patients can provide insight into the underlying LC-related lesion. Here we present two patients with MM complicated by acute kidney injury (AKI) where LC crystalline casts were observed on urinary sediment analysis. Kidney biopsy revealed acute tubular injury with LC crystal casts within both tubular lumens and renal tubular epithelial cell cytoplasms. These findings suggest that the urinary sediment may be a non-invasive way to diagnose LC crystalline-induced AKI in patients with MM.

Babesiosis-Induced Acute Kidney Injury With Prominent Urinary Macrophages

Babesia is an obligate intracellular erythrocyte parasite that can infect humans. Severe symptomatic disease from massive hemolysis and multiorgan system failure, including acute kidney injury (AKI), occurs. Acute tubular injury from a combination of volume depletion and heme pigment toxicity from profound hemolysis is the most common cause of AKI. We present a case of severe babesiosis complicated by dialysis-requiring AKI with the unique finding of large macrophages containing engulfed erythrocyte fragments in urine sediment. This urinary finding raised the possibility of another diagnosis distinct from acute tubular injury. Subsequent kidney biopsy demonstrated infection-associated acute interstitial nephritis.