Case Reports in Nephrology The latest articles from Hindawi Publishing Corporation © 2015 , Hindawi Publishing Corporation . 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. A Case of Newly Diagnosed Klippel Trenaunay Weber Syndrome Presenting with Nephrotic Syndrome Mon, 27 Apr 2015 08:14:50 +0000 Klippel Trenaunay Weber syndrome (KTWS) is a rare disease characterized by hemihypertrophy, variceal enlargement of the veins, and arteriovenous (AV) malformations. Renal involvement in KTWS is not known except in rare case reports. Herein, we present a case of KTWS with nephrotic syndrome. A 52-year-old male was admitted due to dyspnea and swelling of the body for the last three months. The pathological physical findings were diffuse edema, decreased lung sounds at the right basal site, increased diameter and decreased length of the left leg compared with the right one, diffuse variceal enlargements, and a few hemangiomatous lesions on the left leg. The pathological laboratory findings were hypoalbuminemia, hyperlipidemia, increased creatinine level (1.23 mg/dL), and proteinuria (7.6 g/day). Radiographic pathological findings were cystic lesions in the liver, spleen, and kidneys, splenomegaly, AV malformation on the left posterolateral thigh, and hypertrophy of the soft tissues of the proximal left leg. He was diagnosed to have KTWS with these findings. Renal biopsy was performed to determine the cause of nephrotic syndrome. The pathologic examination was consistent with focal segmental sclerosis (FSGS). He was started on oral methylprednisolone at the dosage of 1 mg/kg and began to be followedup in the nephrology outpatient clinic. Egemen Cebeci, Secil Demir, Meltem Gursu, Abdullah Sumnu, Mehmet Yamak, Barıs Doner, Serhat Karadag, Sami Uzun, Ahmet Behlul, Oktay Ozkan, and Savas Ozturk Copyright © 2015 Egemen Cebeci et al. All rights reserved. Donor-Derived Myeloid Sarcoma in Two Kidney Transplant Recipients from a Single Donor Tue, 21 Apr 2015 13:00:52 +0000 We report the rare occurrence of donor-derived myeloid sarcoma in two kidney transplant patients who received organs from a single deceased donor. There was no evidence of preexisting hematologic malignancy in the donor at the time of organ recovery. Both recipients developed leukemic involvement that appeared to be limited to the transplanted organ. Fluorescence in situ hybridization (FISH) and molecular genotyping analyses confirmed that the malignant cells were of donor origin in each patient. Allograft nephrectomy and immediate withdrawal of immunosuppression were performed in both cases; systemic chemotherapy was subsequently administered to one patient. Both recipients were in remission at least one year following the diagnosis of donor-derived myeloid sarcoma. These cases suggest that restoration of the immune system after withdrawal of immunosuppressive therapy and allograft nephrectomy may be sufficient to control HLA-mismatched donor-derived myeloid sarcoma without systemic involvement. Amudha Palanisamy, Paul Persad, Patrick P. Koty, Laurie L. Douglas, Robert J. Stratta, Jeffrey Rogers, Amber M. Reeves-Daniel, Giuseppe Orlando, Alan C. Farney, Michael W. Beaty, Mark J. Pettenati, Samy S. Iskandar, David D. Grier, Scott A. Kaczmorski, William H. Doares, Michael D. Gautreaux, Barry I. Freedman, and Bayard L. Powell Copyright © 2015 Amudha Palanisamy et al. All rights reserved. Two Brothers with Bardet-Biedl Syndrome Presenting with Chronic Renal Failure Wed, 15 Apr 2015 12:57:12 +0000 Bardet-Biedl Syndrome (BBS) is a rarely seen autosomal recessive transfer disease characterised by retinal dystrophy, obesity, extremity deformities, mental retardation, and renal and genital system anomalies. BBS shows heterogenic transfer. To date, 18 genes (BBS1–18) and 7 BBS proteins have been defined as related to BBS. All of the defined BBS genes have been shown to be related to the biogenesis or function of cilia. Renal failure accompanying the syndrome, especially in the advanced stages, is the most common cause of mortality. Therefore, as one of the major diagnostic criteria, renal damage is of great importance in early diagnosis. This paper presents the cases of two brothers with BBS who presented with chronic renal failure. Cem Sahin, Bulent Huddam, Gulhan Akbaba, Hasan Tunca, Emine Koca, and Mustafa Levent Copyright © 2015 Cem Sahin et al. All rights reserved. Neonatal Urinary Ascites: A Report of Three Cases Tue, 14 Apr 2015 14:33:12 +0000 Urinary ascites in neonates is not a common condition. Three cases of urinary ascites are presented and each of them has a different aetiology. Neonates with urinary ascites usually present as clinical emergency, requiring resuscitation, ventilator support, and subsequent drainage of urine. The ultimate management depends on the site of extravasation and the underlying cause. Adaobi Solarin, Priya Gajjar, and Peter Nourse Copyright © 2015 Adaobi Solarin et al. All rights reserved. A Novel Technique for Laparoscopic Salvage of CAPD Catheter Malfunction and Migration: The Santosh-PGI Hanging Loop Technique Mon, 30 Mar 2015 09:47:25 +0000 CAPD catheter malfunction is a common problem. Obstruction due to wrapping by appendices epiploicae of sigmoid colon has been rarely reported in literature. We report a case of CAPD catheter malfunction caused by catheter tip migration and obstruction by appendices epiploicae that was successfully managed by laparoscopic hanging loop technique. This case report highlights the ease with which epiplopexy can be performed and catheter tip migration can be prevented by this innovative laparoscopic procedure. Santosh Kumar, Shivanshu Singh, Aditya Prakash Sharma, and Manish Rathi Copyright © 2015 Santosh Kumar et al. All rights reserved. Severe Rhabdomyolysis Associated with Simvastatin and Role of Ciprofloxacin and Amlodipine Coadministration Thu, 26 Mar 2015 12:45:43 +0000 Simvastatin is among the most commonly used prescription medications for cholesterol reduction and the most common statin-related adverse drug reaction is skeletal muscle toxicity. Multiple factors have been shown to influence simvastatin-induced myopathy. In addition to age, gender, ethnicity, genetic predisposition, and dose, drug-drug interactions play a major role. This is particularly true for drugs that are extensively metabolized by cytochrome P450 (CYP)3A4. We describe a particularly severe case of rhabdomyolysis after the introduction of ciprofloxacin, a weak CYP3A4 inhibitor, in a patient who previously tolerated the simvastatin-amlodipine combination. Nicolas De Schryver, Xavier Wittebole, Peter Van den Bergh, Vincent Haufroid, Eric Goffin, and Philippe Hantson Copyright © 2015 Nicolas De Schryver et al. All rights reserved. Palliative Care for a Mentally Incompetent End Stage Renal Failure Patient: Why Is It Important? Mon, 09 Mar 2015 12:37:49 +0000 People with intellectual disabilities are among the most disadvantaged groups in society. Here we report a mentally incompetent end stage renal failure (ESRF) patient with frequent emergency visits who made a significant improvement in symptoms control and reduction in casualty visits after introduction of renal palliative care service. Multidisciplinary approach would be useful in this case. Kwok-Ying Chan, Terence Yip, Mau-Kwong Sham, Benjamin Hon-Wai Cheng, Cho-Wing Li, Yim-Chi Wong, and Vikki Wai-Kee Lau Copyright © 2015 Kwok-Ying Chan et al. All rights reserved. The Hidden Cost of Untreated Paragangliomas of the Head and Neck: Systemic Reactive (AA) Amyloidosis Mon, 09 Mar 2015 09:35:54 +0000 We report a case of a 51-year-old man who was diagnosed with systemic reactive (AA) amyloidosis in association with untreated glomus jugulare and glomus caroticum tumors. He refused radiotherapy and renal replacement therapy. Paragangliomas, although rare, should be considered one of the tumors that can result in AA amyloidosis. Erkan Dervisoglu, Murat Ozturk, Mehmet Tuncay, Gulhatun Kilic Dervisoglu, Yesim Gurbuz, and Serhan Derin Copyright © 2015 Erkan Dervisoglu et al. All rights reserved. The Impact of Intensified Hemodialysis on Pruritus in an End Stage Renal Disease Patient with Biliary Ductopenia Wed, 04 Mar 2015 13:37:29 +0000 We report a unique observation characterized by the coexistence of idiopathic adulthood ductopenia (IAD), a rare cholestatic disease, and end stage renal failure treated by conventional hemodialysis in a patient awaiting double renal and liver transplantation. As pruritus gradually worsened, we hypothesized that intensified dialysis could alleviate the symptoms. Conventional hemodialysis following 3 hours/3 times a week regimen was initiated in December 2013. Due to increasing pruritus not responding to standard medical therapy, intensified hemodialysis following 2.5 hours/5 times a week regimen was started in May 2014. During two weeks, a temporary decrease in bilirubin levels was observed. No major changes on other liver function tests and inflammatory markers occurred. Nevertheless, a persistent improvement on pruritus and general wellbeing was obtained during the four weeks’ study period. The pathogenesis of itch encompasses multiple factors, and, in our case, both uremic and cholestatic pruritus are involved, although the latter is likely to account for a greater proportion. By improving itch intensity, through better clearance of uremic and cholestatic toxins which we detail further, intensive dialysis appears to be an acceptable short-term method for patients with hepatic cholestasis and moderate pruritus not responding to conventional therapy. Additional studies are needed to assess and differentiate precisely factors contributing to pruritus of both origins. Sandra Chomicki and Omar Dahmani Copyright © 2015 Sandra Chomicki and Omar Dahmani. All rights reserved. Retracted: Anti-GBM of Pregnancy: Acute Renal Failure Resolved after Spontaneous Abortion, Plasma Exchange, Hemodialysis, and Steroids Thu, 26 Feb 2015 08:13:50 +0000 Case Reports in Nephrology Copyright © 2015 Case Reports in Nephrology. All rights reserved. A Case of Nephrotic Syndrome, Showing Evidence of Response to Saquinavir Sat, 31 Jan 2015 16:09:43 +0000 The treatment of primary nephrotic syndrome such as minimal change nephropathy, membranous nephropathy, and focal segmental glomerulosclerosis nephropathy remains challenging. Whilst most cases of idiopathic nephrotic syndrome respond to steroid therapy and experience a limited number of relapses prior to complete remission, some cases suffer from frequent relapses and become steroid dependent or are primarily steroid resistant. Treatment options are limited to immunosuppressive drugs with significant side effect profiles. New modalities targeting novel pathways in the pathogenesis of nephrotic syndrome are actively sought. Here we report the case of a patient with steroid dependent focal segmental glomerulosclerosis (FSGS) nephrotic syndrome with a favourable response to a novel proteasome inhibitor saquinavir. Giles Walters, Faisal A. Choudhury, and Budhima Nanayakkara Copyright © 2015 Giles Walters et al. All rights reserved.