Case Reports in Nephrology The latest articles from Hindawi Publishing Corporation © 2016 , Hindawi Publishing Corporation . All rights reserved. Drug-Induced Acute Interstitial Nephritis with Nifedipine Wed, 03 Feb 2016 12:11:54 +0000 Background. Acute interstitial nephritis (AIN) is a frequent cause of Acute Kidney Injury (AKI). Drug hypersensitivity is the most common etiology and the list of drugs that can induce AIN is not exhaustive yet. Case Report. Here, we describe the case of a 43-year-old man who was treated with nifedipine (Adalate®) for Raynaud’s syndrome. After nifedipine introduction, serum creatininemia progressively increased from 91 to 188 μmol/L in a few months and AKI was diagnosed. Laboratory work-up results indicated the presence of tubular proteinuria and nonspecific inflammatory syndrome. Histological analysis found granulomatous interstitial nephropathy without necrosis in 20% of the kidney biopsy without immunofluorescent deposit. Nifedipine was stopped and corticosteroid treatment was started with a rapid but incomplete reduction of serum creatininemia level to 106 μmol/L. Conclusion. This is the first case of AIN caused by nifedipine. Léonard Golbin, Thibault Dolley-Hitze, Nolwenn Lorcy, Nathalie Rioux-Leclercq, and Cécile Vigneau Copyright © 2016 Léonard Golbin et al. All rights reserved. Successful Pregnancy Using the NxStage Home Hemodialysis System Tue, 02 Feb 2016 07:09:02 +0000 Pregnancy in the setting of the uremic milieu of renal disease has a lower success rate than in the normal population and is a rare event. While intensified renal replacement therapy (RRT) during pregnancy can lead to improved outcomes, most studies have focused on nocturnal hemodialysis as the main RRT in pregnancy. Although thousands of patients use the home NxStage System One short daily hemodialysis (SDHD) machine in the United States, pregnancy outcomes with this therapy are unknown. The NxStage System One uses low-volume dialysate and hence small and middle molecule clearance may differ compared to conventional therapies and affect pregnancy outcomes. We report a case of a successful conception and pregnancy using the home NxStage system. The NxStage system may provide an alternative to the more routinely used NHD or standard SDHD therapies for women of childbearing age. Yasmin Brahmbhatt, Arinze Ikeme, Navjyot Bhogal, and Vincenzo Berghella Copyright © 2016 Yasmin Brahmbhatt et al. All rights reserved. Ganciclovir-Resistant Cytomegalovirus Infection in a Kidney Transplant Recipient Successfully Treated with Foscarnet and Everolimus Sun, 31 Jan 2016 16:44:17 +0000 Cytomegalovirus (CMV) infection remains a major cause of morbidity, graft failure, and death in kidney transplant recipients. We describe a case of a 53-year-old CMV-seronegative man who underwent renal transplant from a CMV-positive donor and who developed ganciclovir- (GCV-) resistant CMV infection. Foscarnet was started while immunosuppressive therapy was modified with the introduction of everolimus minimizing tacrolimus dosage. Only two weeks after the start of this treatment regimen was the patient’s viral load negative. At two-year follow-up the patient has no clinical or laboratory signs of CMV infection and a good and stable renal function or graft survival. In our case, administration of an mTOR inhibitor combined with foscarnet led to rapid and persistent viral clearance without compromising short- and medium-term graft function. This combination therapy supports the need for the kidney transplant community to individualize a target therapy for each type of GCV-resistant CMV infection. Viola Menghi, Giorgia Comai, Olga Baraldi, Giovanni Liviano D’Arcangelo, Tiziana Lazzarotto, and Gaetano La Manna Copyright © 2016 Viola Menghi et al. All rights reserved. Podocyturia: A Clue for the Rational Use of Amiloride in Alport Renal Disease Thu, 28 Jan 2016 12:51:42 +0000 No specific or efficient treatment exists for Alport syndrome, an X-linked hereditary disease caused by mutations in collagen type IV, a crucial component of the glomerular basement membrane. Kidney failure is usually a major complication of the disease, and patients require renal replacement therapy early in life. Microhematuria and subsequently proteinuria are hallmarks of kidney involvement, which are due to primary basement membrane alterations that mainly cause endothelial thrombosis and podocyte contraction and ulterior irreversible detachment. Commonly drug-based approaches include angiotensin-converting enzyme inhibitors and angiotensin receptor blockers, which are employed to reduce proteinuria and thus retard kidney disease progression and cardiovascular morbidity and mortality. However, as any hereditary disease, it is expressed as early as in the intrauterine life, and usually an index case is helpful to detect family-related cases. As no specific treatment exists, pathophysiologically based approaches are useful. The present case illustrates the reduction rate of urinary podocyte loss and proteinuria after amiloride administration and suggests the molecular pathways involved in Alport renal disease. Finally, podocyturia rather than proteinuria should be considered as an earlier biomarker of kidney involvement and disease progression in Alport disease. H. Trimarchi, R. Canzonieri, A. Muryan, A. Schiel, A. Araoz, M. Paulero, J. Andrews, T. Rengel, M. Forrester, F. Lombi, V. Pomeranz, R. Iriarte, and E. Zotta Copyright © 2016 H. Trimarchi et al. All rights reserved. Granulomatous Interstitial Nephritis Presenting as Hypercalcemia and Nephrolithiasis Tue, 19 Jan 2016 14:15:37 +0000 We report a case of acute kidney injury as the initial manifestation of sarcoidosis. A 55-year-old male was sent from his primary care physician’s office with incidental lab findings significant for hypercalcemia and acute kidney injury with past medical history significant for nephrolithiasis. Initial treatment with intravenous hydration did not improve his condition. The renal biopsy subsequently revealed granulomatous interstitial nephritis (GIN). Treatment with the appropriate dose of glucocorticoids improved both the hypercalcemia and renal function. Our case demonstrates that renal limited GIN due to sarcoidosis, although a rare entity, can cause severe acute kidney injury and progressive renal failure unless promptly diagnosed and treated. Saika Sharmeen, Esra Kalkan, Chunhui Yi, and Steven D. Smith Copyright © 2016 Saika Sharmeen et al. All rights reserved. Necrotizing ANCA-Positive Glomerulonephritis Secondary to Culture-Negative Endocarditis Sun, 27 Dec 2015 10:15:01 +0000 Infective endocarditis (IE) and small-vessel vasculitis may have similar clinical features, including glomerulonephritis. Furthermore the association between IE and ANCA positivity is well documented, making differential diagnosis between IE- and ANCA-associated vasculitis particularly difficult, especially in case of culture-negative IE. We report on one patient with glomerulonephritis secondary to culture-negative IE caused by Bartonella henselae which illustrates this diagnostic difficulty. Sophie Van Haare Heijmeijer, Dunja Wilmes, Selda Aydin, Caroline Clerckx, and Laura Labriola Copyright © 2015 Sophie Van Haare Heijmeijer et al. All rights reserved. A Rare Case of Central Pontine Myelinolysis in Overcorrection of Hyponatremia with Total Parenteral Nutrition in Pregnancy Tue, 22 Dec 2015 13:26:34 +0000 A 42-year-old high risk pregnant female presented with hyponatremia from multiple causes and was treated with total parenteral nutrition. She developed acute hypernatremia due to the stage of pregnancy and other comorbidities. All the mechanisms of hyponatremia and hypernatremia were summarized here in our case report. This case has picture (graph) representation of parameters that led to changes in serum sodium and radiological findings of central pontine myelinolysis on MRI. In conclusion we present a complicated case serum sodium changes during pregnancy and pathophysiological effects on serum sodium changes during pregnancy. Kalyana C. Janga, Tazleem Khan, Ciril Khorolsky, Sheldon Greenberg, and Priscilla Persaud Copyright © 2015 Kalyana C. Janga et al. All rights reserved. Three-Dimensional Imaging of a Central Venous Dialysis Catheter Related Infected Thrombus Tue, 24 Nov 2015 08:32:03 +0000 Three-dimensional (3D) echocardiography is becoming widely available and with novel applications. We report an interesting case of a 68-year-old lady with a central venous thrombosis coincident with both a dialysis catheter infection and a recent pacemaker insertion. Two-dimensional transesophageal echocardiography was unable to delineate whether the thrombosis was involved with the pacemaker wire or due to the tunneled catheter infection. The use of 3D echocardiography was able to produce distinct images aiding diagnosis. This circumvented the need for invasive investigations and inappropriate, high-risk removal of the pacing wire. This case highlights the emerging application of 3D echocardiography in routine nephrology practice. Diana Yuan Yng Chiu, Darren Green, Philip A. Kalra, and Nik Abidin Copyright © 2015 Diana Yuan Yng Chiu et al. All rights reserved. Long-Term Therapeutic Plasma Exchange to Prevent End-Stage Kidney Disease in Adult Severe Resistant Henoch-Schonlein Purpura Nephritis Tue, 03 Nov 2015 11:43:58 +0000 A 27-year-old man presented with a palpable purpuric skin rash and joint and abdominal pain in April 2010. He had acute kidney injury and his creatinine quickly deteriorated to 687 μmol/L, with associated nephrotic range proteinuria. Kidney biopsy showed crescentic Henoch-Schonlein nephritis. He was treated with intravenous cyclophosphamide and prednisolone despite which his renal function deteriorated; he required haemodialysis for a short duration and seven sessions of therapeutic plasma exchange (TPE). Renal function improved, but after discharge from hospital he suffered 2 further relapses, each with AKI, in 4 months. Cyclophosphamide was not effective and therefore Rituximab was introduced. He initially had a partial response but his renal function deteriorated despite continued therapy. TPE was the only treatment that prevented rapid renal functional deterioration. A novel long-term treatment strategy involving regular TPE every one to two weeks was initiated. This helped to slow his progression to end-stage kidney disease over a 3-year period and to prolong the need for renal replacement therapy over this time. Patrick Hamilton, Olumide Ogundare, Ammar Raza, Arvind Ponnusamy, Julie Gorton, Hana Alachkar, Jamil Choudhury, Jonathan Barratt, and Philip A. Kalra Copyright © 2015 Patrick Hamilton et al. All rights reserved. An Unexpected Cause of Severe Hypokalemia Thu, 15 Oct 2015 08:15:12 +0000 We describe an unusual case of severe hypokalemia with electrocardiographic changes, due to licorice consumption, in a 15-year-old female student with no previous medical history. Prompt replacement of potassium and cessation of licorice ingestion resulted in a favourable outcome. We also discuss the pathophysiology and diagnosis, emphasizing the importance of a detailed anamnesis to rule out an often forgotten cause of hypokalemia as the licorice poisoning. Fernando Caravaca-Fontan, Olga Martinez-Saez, Maria Delgado-Yague, Estefania Yerovi, and Fernando Liaño Copyright © 2015 Fernando Caravaca-Fontan et al. All rights reserved. Rhabdomyolysis and Acute Kidney Injury Requiring Dialysis as a Result of Concomitant Use of Atypical Neuroleptics and Synthetic Cannabinoids Tue, 13 Oct 2015 09:26:24 +0000 The use of synthetic cannabinoids (SCBs) is associated with many severe adverse effects that are not observed with marijuana use. We report a unique case of a patient who developed rhabdomyolysis and acute kidney injury (AKI) requiring dialysis after use of SCBs combined with quetiapine. Causes for the different adverse effects profile between SCBs and marijuana are not defined yet. Cases reported in literature with SCBs use have been associated with reversible AKI characterized by acute tubular necrosis and interstitial nephritis. Recent studies have showed the involvement of cytochromes P450s (CYPs) in biotransformation of SCBs. The use of quetiapine which is a substrate of the CYP3A4 and is excreted (73%) as urine metabolites may worsen the side effect profiles of both quetiapine and K2. SCBs use should be included in the differential diagnosis of AKI and serum Creatinine Phosphokinase (CPK) level should be monitored. Further research is needed to identify the mechanism of SCBs nephrotoxicity. Aiyu Zhao, Maybel Tan, Aung Maung, Moro Salifu, and Mary Mallappallil Copyright © 2015 Aiyu Zhao et al. All rights reserved. Pauci-Immune Necrotizing and Crescentic Glomerulonephritis with Membranous Lupus Nephritis, Fifteen Years after Initial Diagnosis of Secondary Membranous Nephropathy Thu, 08 Oct 2015 12:30:58 +0000 Renal involvement in systemic lupus erythematosus (SLE) is usually immune complex mediated and may have multiple different presentations. Pauci-immune necrotizing and crescentic glomerulonephritis (NCGN) refers to extensive glomerular inflammation with few or no immune deposits that may result in rapid decline in renal function. We report a case of a 79-year-old Hispanic male with a history of secondary membranous nephropathy (diagnosed by renal biopsy 15 years previously) who was admitted with acute kidney injury and active urinary sediment. P-ANCA titers and anti-myeloperoxidase antibodies were positive. The renal biopsy was diagnostic for NCGN superimposed on a secondary membranous nephropathy. A previous diagnosis of SLE based on American College of Rheumatology criteria was discovered via Veteran’s Administration records review after the completion of treatment for pauci-immune NCGN. ANCAs are detected in 20–31% of patients with SLE. There may be an association between SLE and ANCA seropositivity. In patients with lupus nephritis and biopsy findings of necrotizing and crescentic glomerulonephritis, without significant immune complex deposition, ANCA testing should be performed. In patients with secondary membranous nephropathy SLE should be excluded. Ryan Burkhart, Nina Shah, Michael Abel, James D. Oliver III, and Matthew Lewin Copyright © 2015 Ryan Burkhart et al. All rights reserved. A Rare Cause of Reversible Renal Hemosiderosis Wed, 30 Sep 2015 08:01:49 +0000 Kidney failure secondary to renal hemosiderosis has been reported in diseases with intravascular hemolysis, like paroxysmal nocturnal hemoglobinuria, and valvular heart diseases. We present here a case of hemosiderin induced acute tubular necrosis secondary to intravascular hemolysis from Clostridium difficile infection with possible role of supratherapeutic INR. We discuss the pathophysiology, causes, and prognosis of acute tubular injury from hemosiderosis. Rima Abou Arkoub, Don Wang, and Deborah Zimmerman Copyright © 2015 Rima Abou Arkoub et al. All rights reserved. Sildenafil Induced Acute Interstitial Nephritis Sun, 27 Sep 2015 09:38:40 +0000 Acute interstitial nephritis (AIN) is characterized by inflammation of the renal interstitium and usually occurs in a temporal relationship with the medication. We present a case of an Asian male who had nephrotic range proteinuria and presented with acute kidney injury. The patient reported an acute change in physical appearance and symptomatology after the ingestion of a single dose of sildenafil. Renal biopsy was notable for minimal change disease (MCD) with acute and chronic interstitial nephritis. Renal replacement and glucocorticoid therapy were initiated. Renal recovery within six weeks permitted discontinuation of dialysis. AIN superimposed on MCD is a known association of NSAID induced nephropathy. The temporal association and the absence of any new drugs suggest that the AIN was most likely due to the sildenafil. NSAIDs are less likely to have caused the AIN given their remote use. The ease of steroid responsiveness would also suggest another cause as NSAID induced AIN is often steroid resistant. The MCD was most likely idiopathic given the lack of temporal association with a secondary cause. As the number of sildenafil prescriptions increases, more cases of AIN may be identified and physician awareness for this potential drug disease association is necessary. Ryan Burkhart, Nina Shah, and Matthew Lewin Copyright © 2015 Ryan Burkhart et al. All rights reserved. Hyperchloremic Metabolic Acidosis due to Cholestyramine: A Case Report and Literature Review Thu, 03 Sep 2015 09:36:05 +0000 Cholestyramine is a bile acid sequestrant that has been used in the treatment of hypercholesterolemia, pruritus due to elevated bile acid levels, and diarrhea due to bile acid malabsorption. This medication can rarely cause hyperchloremic nonanion gap metabolic acidosis, a complication featured in this report of an adult male with concomitant acute kidney injury. This case emphasizes the caution that must be taken in prescribing cholestyramine to patients who may also be volume depleted, in renal failure, or taking spironolactone. Fareed B. Kamar and Rory F. McQuillan Copyright © 2015 Fareed B. Kamar and Rory F. McQuillan. All rights reserved. Biopsy Induced Arteriovenous Fistula and Venous Stenosis in a Renal Transplant Tue, 25 Aug 2015 10:02:25 +0000 Renal transplant vein stenosis is a rare cause of allograft dysfunction. Percutaneous stenting appears to be safe and effective treatment for this condition. A 56-year-old Caucasian female with end stage renal disease received a deceased donor renal transplant. After transplant, her serum creatinine improved to a nadir of 1.2 mg/dL. During the third posttransplant month, her serum creatinine increased to 2.2 mg/dL. Renal transplant biopsy showed BK nephropathy. Mycophenolate was discontinued. Over the next 2 months, her serum creatinine crept up to 6.2 mg/dL. BK viremia improved from 36464 copies/mL to 15398 copies/mL. A renal transplant ultrasound showed lower pole arteriovenous fistula and abnormal waveforms in the renal vein. Carbon dioxide (CO2) angiography demonstrated severe stenosis of the transplant renal vein. Successful coil occlusion of fistula was performed along with angioplasty and deployment of stent in the renal transplant vein. Serum creatinine improved to 1.5 mg/dL after. Sridhar R. Allam, Balamurugan Sankarapandian, Imran A. Memon, Patrick C. Nef, Tom S. Livingston, and George Rofaiel Copyright © 2015 Sridhar R. Allam et al. All rights reserved. Common Iliac Artery Thrombosis following Pelvic Surgery Resulting in Kidney Allograft Failure Successfully Treated by Percutaneous Transluminal Angioplasty with Balloon-Expandable Covered Stent Tue, 18 Aug 2015 09:53:34 +0000 We report the case of a 66-year-old woman who developed acute kidney allograft failure due to thrombotic occlusion of the common iliac artery after hysterectomy requiring emergent allograft rescue. She underwent percutaneous transluminal angioplasty with endovascular balloon expandable covered stent graft placement in the right common iliac artery. Although there are a handful of case reports of acute limb ischemia secondary to acute common iliac artery thrombosis, this is the first case reported in the literature resulting in successful kidney allograft rescue following pelvic surgery. Maheswara S. Golla, Subasit Acharjee, Bertrand L. Jaber, and Lawrence A. Garcia Copyright © 2015 Maheswara S. Golla et al. All rights reserved. Purple Urine Bag Syndrome in Two Elderly Men with Urinary Tract Infection Mon, 17 Aug 2015 08:34:46 +0000 Purple urine bag syndrome is a rare condition in which purple discoloration of urine inside its collection bag occurs. We describe two illustrative cases. The first patient is an 81-year-old man who was hospitalized for a newly diagnosed lymphoma with acute obstructive renal failure for which a nephrostomy procedure was performed. During the hospitalization, a sudden purple discoloration of the suprapubic catheter urine was noted, while the nephrostomy urine had a normal color. Urine culture from the suprapubic catheter was positive for Pseudomonas aeruginosa and Enterococcus faecalis; urine from the nephrostomy was sterile. The second case is an 80-year-old man who was admitted for heart failure with cardiorenal dilemma and who was started on intermittent hemodialysis. There was a sudden purple discoloration of the urine in the collection bag from his indwelling catheter. He was diagnosed with an E. coli urinary infection and treated with amoxicillin and removal of the indwelling catheter. These two cases illustrate the typical characteristics of purple urine bag syndrome. Jan Van Keer, Daan Detroyer, and Bert Bammens Copyright © 2015 Jan Van Keer et al. All rights reserved. Minimal Change Disease as a Secondary and Reversible Event of a Renal Transplant Case with Systemic Lupus Erythematosus Thu, 13 Aug 2015 14:06:18 +0000 Secondary causes of minimal change disease (MCD) account for a minority of cases compared to its primary or idiopathic form and provide ground for consideration of common mechanisms of pathogenesis. In this paper we report a case of a 27-year-old Latina woman, a renal transplant recipient with systemic lupus erythematosus (SLE), who developed nephrotic range proteinuria 6 months after transplantation. The patient had recurrent acute renal failure and multiple biopsies were consistent with MCD. However, she lacked any other features of the typical nephrotic syndrome. An angiogram revealed a right external iliac vein stenosis in the region of renal vein anastomosis, which when restored resulted in normalization of creatinine and relief from proteinuria. We report a rare case of MCD developing secondary to iliac vein stenosis in a renal transplant recipient with SLE. Additionally we suggest that, in the event of biopsy-proven MCD presenting as an atypical nephrotic syndrome, alternative or secondary, potentially reversible, causes should be considered and explored. Elena Gkrouzman, Kyriakos A. Kirou, Surya V. Seshan, and James M. Chevalier Copyright © 2015 Elena Gkrouzman et al. All rights reserved. Plotting of Ethylene Glycol Blood Concentrations Using Linear Regression before and during Hemodialysis in a Case of Intoxication and Pharmacokinetic Review Thu, 06 Aug 2015 09:32:49 +0000 Introduction. As blood concentration measurement of commonly abused alcohol is readily available, the equation was proposed in previous publication to predict the change of their concentration. The change of ethylene glycol (EG) concentrations was studied in a case of intoxication to estimate required time for hemodialysis (HD) using linear regression. Case Report. A 55-year-old female with past medical history of seizure disorder, bipolar disorder, and chronic pain was admitted due to severe agitation. The patient was noted to have metabolic acidosis with elevated anion gap and acute kidney injury, which prompted blood concentration measurement of commonly abused alcohol. Her initial EG concentration was 26.45 mmol/L. Fomepizole therapy was initiated, soon followed by HD to enhance clearance. Discussion. Plotting of natural logarithm of EG concentrations over time showed that EG elimination follows first-order kinetics and predicts the change of its concentration well. Pharmacokinetic review revealed minimal elimination of EG by alcohol dehydrogenase (ADH) which could be related to genetic predisposition for ADH activity and home medications as well as presence of propylene glycol. Pharmacokinetics of EG is relatively well studied with published parameters. Consideration and application of pharmacokinetics could assist in management of EG intoxication including HD planning. Youngho Kim Copyright © 2015 Youngho Kim. All rights reserved. Dabigatran-Related Nephropathy in a Patient with Undiagnosed IgA Nephropathy Wed, 05 Aug 2015 16:42:14 +0000 Dabigatran is a direct thrombin inhibitor used as an alternative to warfarin for long term anticoagulation. Warfarin-related nephropathy is an increasingly recognized entity, but recent evidence suggests that dabigatran can cause a WRN-like syndrome. We describe a case of a biopsy-proven anticoagulant nephropathy related to dabigatran in a patient with IgA nephropathy and propose that, despite the base glomerular disease, acute kidney injury was due to tubular obstruction by red blood cells and heme-associated tubular injury, and through a mechanism involving inhibition of anticoagulation cascade and barrier abnormalities caused by molecular mechanisms. Rachele Escoli, Paulo Santos, Sequeira Andrade, and Fernanda Carvalho Copyright © 2015 Rachele Escoli et al. All rights reserved. Unexpected Abscess Localization of the Anterior Abdominal Wall in an ADPKD Patient Undergoing Hemodialysis Sun, 02 Aug 2015 13:35:49 +0000 Autosomal Dominant Polycystic Kidney Disease (ADPKD) is one of the most common monogenic disorders and the leading inheritable cause of end-stage renal disease worldwide. Cystic and noncystic extrarenal manifestations are correlated with variable clinical presentations so that an inherited disorder is now considered a systemic disease. Kidney and liver cystic infections are the most common infectious complications in ADPKD patients. Furthermore, it is well known that ADPKD is commonly associated with colonic diverticular disease which recently has been reported to be linked to increased risk of infection on hemodialysis patients. Herein, we present a case of anterior abdominal wall abscess caused by Enterococcus faecalis in a patient with ADPKD undergoing hemodialysis. Although the precise pathway of infection remains uncertain, the previous medical history as well as the clinical course of our patient led us to hypothesize an alternative route of infection from the gastrointestinal tract through an aberrant intestinal barrier into the bloodstream and eventually to an atypical location. Nikos Sabanis, Eleni Paschou, Eleni Gavriilaki, Maria Mourounoglou, and Sotirios Vasileiou Copyright © 2015 Nikos Sabanis et al. All rights reserved. Crossed Renal Ectopia without Fusion: An Uncommon Cause of Abdominal Mass Tue, 28 Jul 2015 09:39:32 +0000 Crossed renal ectopia is a rare congenital anomaly usually associated with fused kidneys (90%). Most cases are asymptomatic and remain undiagnosed. We report an unusual case of nonfused crossed renal ectopia. The 11-year-old adolescent female patient was admitted with abdominal pain, anorexia, weight loss, and periumbilical mass. Although the initial clinical suspicion was a tumoral lesion, abdominal ultrasound and magnetic resonance examination revealed crossed renal ectopia without fusion. The renal ectopy was incidentally diagnosed, as described in 20 to 30% of cases. In this case, the associated nonspecific symptoms were a coincidence. Ana Ratola, Maria Miguel Almiro, Rita Lacerda Vidal, Nuno Neves, Adelaide Bicho, and Sofia Figueiredo Copyright © 2015 Ana Ratola et al. All rights reserved. Levamisole/Cocaine Induced Systemic Vasculitis and Immune Complex Glomerulonephritis Tue, 28 Jul 2015 08:52:25 +0000 Levamisole is an antihelminthic and immunomodulator medication that was banned by the USFDA in 1998. It has been increasingly used to adulterate cocaine due to its psychotropic effects and morphological properties. Adverse reactions including cutaneous vasculitis, thrombocytopenia, and agranulocytosis have been well described. Despite systemic vasculitis in this setting, renal involvement is uncommon. We report here a case of ANCA positive systemic vasculitis with biopsy proven immune complex mediated glomerulonephritis likely secondary to levamisole/cocaine. A 40-year-old Caucasian male with no past medical history presented with 3-week history of fatigue, skin rash, joint pains, painful oral lesions, oliguria, hematuria, worsening dyspnea on exertion, and progressive lower extremity edema. He had a history of regular tobacco and cocaine use. Lab testing revealed severe anemia, marked azotemia, deranged electrolytes, and 4.7 gm proteinuria. Rheumatologic testing revealed hypocomplementemia, borderline ANA, myeloperoxidase antibody, and positive atypical p-ANCA. Infectious and other autoimmune workup was negative. Kidney biopsy was consistent with immune mediated glomerulonephritis and showed mesangial proliferation and immune complex deposition consisting of IgG, IgM, and complement. High dose corticosteroids and discontinuing cocaine use resulted in marked improvement in rash, mucocutaneous lesions, and arthritis. There was no renal recovery and he remained hemodialysis dependent. Lohit Garg, Sagar Gupta, Abhishek Swami, and Ping Zhang Copyright © 2015 Lohit Garg et al. All rights reserved. Pulmonary Limited MPO-ANCA Microscopic Polyangiitis and Idiopathic Lung Fibrosis in a Patient with a Diagnosis of IgA Nephropathy Wed, 22 Jul 2015 12:24:33 +0000 We present a case of a male patient with chronic renal insufficiency, due to crescentic glomerulonephritis with IgA deposits, who successively developed (idiopathic) thrombocytopenic purpura (ITP) and MPO-ANCA microscopic polyangiitis (MPA) with pulmonary fibrosis. The patient presented with cough, weight loss, and dyspnea on exertion. CT imaging and pulmonary function tests were compatible with interstitial pneumonitis with pulmonary fibrosis. Laboratory results showed high MPO-ANCA titers; the urinary sediment was bland. The patient was treated successfully with cyclophosphamide and methyl-prednisolone. This unique case illustrates the diagnostic and therapeutic challenges of an unusual presentation of microscopic polyangiitis presenting first as isolated kidney disease with recurrence in the form of pneumonitis without renal involvement, in association with renal IgA deposits and ITP as coexisting autoimmune conditions. Alwin Tilanus, Patricia Van der Niepen, Caroline Geers, and Karl Martin Wissing Copyright © 2015 Alwin Tilanus et al. All rights reserved. Homozygosity for the E526V Mutation in Fibrinogen A Alpha-Chain Amyloidosis: The First Report Tue, 23 Jun 2015 09:44:34 +0000 Systemic hereditary amyloidoses are autosomal dominant diseases associated with mutations in genes encoding ten different proteins. The clinical phenotype has implications on therapeutic approach, but it is commonly variable and largely dependent on the type of mutation. Except for rare cases involving gelsolin or transthyretin, patients are heterozygous for the amyloidogenic variants. Here we describe the first patient identified worldwide as homozygous for a nephropathic amyloidosis, involving the fibrinogen variant associated with the fibrinogen alpha-chain E526V (p.Glu545Val) mutation. In 1989, a 44-year-old woman presented with hypertension, hepatosplenomegaly, nephrotic syndrome, and renal failure. She started hemodialysis in 1990 and 6 years later underwent isolated kidney transplantation from a deceased donor. Graft function and clinical status were unremarkable for 16 years, despite progressively increased left ventricular mass on echocardiography. In 2012, 4 months before death, she deteriorated rapidly with severe heart failure, precipitated by Clostridium difficile colitis and urosepsis. Affected family members developed nephropathy, on average, nearly three decades later, which may be explained by the gene dosage effects on the phenotype of E526V (p.Glu545Val) fibrinogen A alpha-chain amyloidosis. Isabel Tavares, Luísa Lobato, Carlos Matos, Josefina Santos, Paul Moreira, Maria João Saraiva, and António Castro Henriques Copyright © 2015 Isabel Tavares et al. All rights reserved. Solitary Crossed Renal Ectopia: Concurrence of Posterior Urethral Valve and Hypospadias Thu, 18 Jun 2015 11:49:28 +0000 Solitary crossed renal ectopia (SCRE) represents an exceedingly rare congenital disorder. Although skeletal and genitourinary abnormalities most commonly accompany this condition, vesicoureteral reflux (VUR) has been described in only a few cases. Here, we present two unique cases of SCRE complicated by high-grade VUR concomitant with posterior urethral valve in one case and hypospadias in the other one. We also provide a brief review of the literature on this subject. Amin Bagheri, Reza Khorramirouz, Sorena Keihani, Mehdi Fareghi, and Abdol-Mohammad Kajbafzadeh Copyright © 2015 Amin Bagheri et al. All rights reserved. Nonsecretory Multiple Myeloma and AL Amyloidosis Presenting with Nephrotic Range Proteinuria Thu, 21 May 2015 16:03:24 +0000 Nonsecretory multiple myeloma (NSMM) is the absence of a detectable monoclonal protein in serum and urine of a multiple myeloma (MM) patient and immunoglobulin light chain (AL) amyloidosis is a significantly rare complication. A case of NSMM with AL amyloidosis and nephrotic range proteinuria is presented. Sharing clinical, therapeutic, and prognostic characteristics with MM, real challenge may be during initial diagnosis of NSMM and assessment of treatment response. In elderly patients with unexplained renal dysfunction, MM should be in the differential diagnosis and the absence of a monoclonal protein should not rule out MM but should remind us of the possibility of NSMM. Ozlem Beyler Kilic, Ali Kemal Oguz, Ihsan Ergun, Dilek Ertoy Baydar, and Meltem Ayli Copyright © 2015 Ozlem Beyler Kilic et al. All rights reserved. Copious Podocyturia without Proteinuria and with Normal Renal Function in a Young Adult with Fabry Disease Tue, 12 May 2015 06:23:12 +0000 The time for starting a patient with Fabry disease on enzyme replacement therapy is still a matter of debate, particularly when no overt classical clinical signs or symptoms are present. With respect to Fabry nephropathy, a dual problem coexists: the reluctance of many nephrologists to start enzyme replacement infusion until signs of renal disease appear as the appearance of proteinuria or an elevation in serum creatinine and the lack of validated biomarkers of early renal damage. In this regard, proteinuria is nowadays considered as an early and appropriate marker of kidney disease and of cardiovascular morbidity and mortality. However, in this report we demonstrate that podocyturia antedates the classical appearance of proteinuria and could be considered as an even earlier biomarker of kidney damage. Podocyturia may be a novel indication for the initiation of therapy in Fabry disease. H. Trimarchi, R. Canzonieri, A. Muryan, A. Schiel, A. Araoz, M. Forrester, A. Karl, F. Lombi, J. Andrews, V. Pomeranz, T. Rengel, and E. Zotta Copyright © 2015 H. Trimarchi et al. All rights reserved. The Rarest of the Rare: Crossed Fused Renal Ectopia of the Superior Ectopia Type Wed, 29 Apr 2015 13:54:39 +0000 Crossed fused renal ectopia is a rare congenital anomaly of the urinary system where one kidney crosses over to opposite side and the parenchyma of the two kidneys fuse. Herein, we present an atypical CFRE case whose renal anatomy does not exactly match any of the already defined CFRE types. Both of the kidneys are ectopic with the crossed ectopic right kidney lying superiorly and fused to the upper pole of the left kidney. Renal arteries were originating from the common iliac arteries. A focal 90% stenosis was observed on the right main renal artery. The patient is borderline hypertensive. Leyla Akdogan, Ali Kemal Oguz, Tarkan Ergun, and Ihsan Ergun Copyright © 2015 Leyla Akdogan et al. All rights reserved.