Long-Term Effects of High-Dose Tolvaptan for Autosomal Dominant Polycystic Kidney Disease Patients
We report here 2 Japanese ADPKD patients (males, 36 and 29 years old) treated with tolvaptan (120 mg/day) for 9 years, during which time determinations of estimated glomerular filtration rate (eGFR) and total kidney volume (TKV) were performed. In these 2 patients, eGFR prior to therapy was 57.3 and 76.3 mL/min/1.73 m2, respectively, and 30.2 and 43.5 mL/min/1.73 m2, respectively, after 9 years of tolvaptan treatment, for a relatively constant annual decline of –3.01 and –3.64 mL/min/1.73 m2, respectively. As compared to the predicted (calculated) eGFR without tolvaptan treatment, eGFR actually measured was higher by 1...
Source: Case Reports in Nephrology and Dialysis - February 12, 2020 Category: Urology & Nephrology Source Type: research

A Case of Frequently Relapsing Minimal-Change Nephrotic Syndrome with Steroid-Induced Psychiatric Syndrome Treated by Low-Dose, Short-Term Steroid Therapy in Combination with Cyclosporine
Adults with minimal-change nephrotic syndrome (MCNS) generally receive oral prednisolone (PSL) at an initial dosage of 1.0 mg/kg/day for a minimum of 4 weeks, with 80% of patients achieving clinical remission. However, relapses are frequent, necessitating repeated treatment with high-dose PSL. Long-term treatment with high-dose steroids increases the risk of steroid toxicities, such as diabetes mellitus, gastric complications, infections, osteoporosis, and steroid-induced psychiatric syndrome (SIPS), which may compromise the patient ’s quality of life. Strategies are therefore needed to reduce the dosage and duration of ...
Source: Case Reports in Nephrology and Dialysis - January 10, 2020 Category: Urology & Nephrology Source Type: research

Daratumumab for Treatment of Antibody-Mediated Rejection after ABO-Incompatible Kidney Transplantation
We report the effectiveness of daratumumab, a human IgG κ monoclonal antibody targeting CD38 on plasma cells, for therapy-refractory antibody-mediated rejection (AMR) due to blood group antibodies in a 59-year-old man who received a living ABO-incompatible kidney transplantation. Standard treatment options for AMR due to blood group antibodies including immunoadsorption, lymphocyte depletion with anti-human T-lymphocyte globulins, intravenous methylprednisolone pulses and eculizumab limited tissue injury, however failed to sufficiently suppress blood group antibody production. After administration of daratumumab as a resc...
Source: Case Reports in Nephrology and Dialysis - November 13, 2019 Category: Urology & Nephrology Source Type: research

PLA2R-Negative Membranous Nephropathy Presenting as a Prodrome of IgG4-Related Autoimmune Pancreatitis
Membranous nephropathy (MN) with tubulointerstitial nephritis (TIN) is an established manifestation of immunoglobulin G4 (IgG4)-related disease (IgG4-RD). A pathological feature aiming to distinguish between primary and secondary MN is the presence or absence of glomerular staining for phospholipase A2 receptor (PLA2R), respectively. Isolated MN without TIN has been rarely reported in the context of IgG4-RD. This case report describes a patient with a history of MN successfully treated with steroids and cyclophosphamide, who, 3 years later, presented with unexplained exacerbation of diabetes mellitus due to IgG4-related au...
Source: Case Reports in Nephrology and Dialysis - June 26, 2019 Category: Urology & Nephrology Source Type: research

Remission of Membranous Nephropathy after Treatment of Localised Prostate Cancer
Membranous nephropathy is a cause of the nephrotic syndrome in adults; it can be a primary or secondary process. Secondary causes include solid organ and lymphoid malignancies. Prostate cancer has been reported as the second most common causative malignancy. Remission of membranous nephropathy following treatment of metastatic prostate cancer is well established. In this case, we describe a patient with localised prostate cancer who developed severe nephrotic syndrome (urine protein creatinine ratio 1,616 mg/mmol and serum albumin 17 g/L) secondary to membranous nephropathy. The prostate cancer was deemed of low risk and s...
Source: Case Reports in Nephrology and Dialysis - June 6, 2019 Category: Urology & Nephrology Source Type: research

Severe Complications from an Unexpectedly High Serum Mycophenolic Acid Concentration in a Patient with Renal Failure Secondary to Lupus Nephritis: A Case Report
We report a case of a patient with lupus nephritis in whom severe complications were possibly caused by MMF. The patient was a 17-year-old girl who received a diagnosis of lupus nephritis at the age of 14 years and had been taking steroid and immunosuppressive agents since then. One week after starting MMF 1 g/day instead of mizoribine owing to symptom relapse and serologic data deterioration, she presented with seizure, accompanied by leukopenia, thrombocytopenia, and renal failure. We discontinued MMF because she had extremely high serum mycophenolate acid concentration (88 µg/mL). A few weeks later, she recovered witho...
Source: Case Reports in Nephrology and Dialysis - June 4, 2019 Category: Urology & Nephrology Source Type: research

Postinfectious Glomerulonephritis with Crescents in an Elderly Diabetic Patient after Acute Gastroenteritis: Case Report
Postinfectious glomerulonephritis (PIGN) is primarily a disease of childhood. It occurs after upper respiratory tract infection or skin infections. Streptococcus is the most common causative agent, but in the elderly, staphylococcus is the main culprit. In adults, PIGN is more common in immunocompromised patients, particularly diabetics and alcoholics. Here, we report the case of an elderly diabetic male who presented with severe acute kidney injury with active urinary sediment after acute gastroenteritis. Additional analyses revealed a very low serum C3 level and a normal serum C4 level. Renal biopsy showed diffuse prolif...
Source: Case Reports in Nephrology and Dialysis - May 23, 2019 Category: Urology & Nephrology Source Type: research

IgG4-Related Kidney Disease: A Curious Case of Interstitial Nephritis with Hypocomplementemia
IgG4-related kidney disease has been relatively newly recognized over the last two decades as a combination of an autoimmune and allergic disorder, with elevated serum IgG4 level and hypocomplementemia among its characteristic features. Here we report the case of a man with interstitial nephritis presenting with acute kidney injury and hypocomplementemia but normal serum IgG4 level and provide a literature review of IgG4-related kidney disease. This case highlights the importance of IgG4-related kidney disease as an important differential diagnosis in any patient presenting with a clinical syndrome mimicking acute intersti...
Source: Case Reports in Nephrology and Dialysis - May 16, 2019 Category: Urology & Nephrology Source Type: research

Single Pass Albumin Dialysis and Plasma Exchange for Copper Toxicity in Acute Wilson Disease
Conclusion: We conclude that SPAD, with or without PLEX, is effective in reducing serum copper levels as a bridge to liver transplantation in WD. PLEX may be more efficient at removing copper but is associated with a rebound increase in copper levels between sessions.Case Rep Nephrol Dial 2019;9:55 –63 (Source: Case Reports in Nephrology and Dialysis)
Source: Case Reports in Nephrology and Dialysis - May 16, 2019 Category: Urology & Nephrology Source Type: research

Significance of Crescentic Glomeruli in Acute Kidney Injury with Rheumatoid Arthritis
Crescentic glomerulonephritis (GN) without immune reactants or deposits (referred to as pauci-immune) is typically characterized by the presence of anti-neutrophilic cytoplasmic antibodies (ANCA). While ANCA-negative patients might be expected to have a more benign course, they often have poor renal outcomes, especially without treatment with steroids and immune-modulating therapy. Pauci-immune crescentic GN can also co-exist with other autoimmune conditions, including rheumatoid arthritis (RA). Here, we describe an ANCA-negative patient with RA who developed dialysis-requiring acute kidney injury (AKI) with findings consi...
Source: Case Reports in Nephrology and Dialysis - May 5, 2019 Category: Urology & Nephrology Source Type: research

Severe, Symptomatic Hypocalcemia due to Denosumab Administration: Treatment and Clinical Course
We present 2 patients who developed severe, symptomatic hypocalcemia after administration of denosumab. These patients provide an opportunity to describe the clinical course and treatment, including the need to consider a continuous calcium infusion, of severe, symptomatic hypocalcemia caused by denosumab.Case Rep Nephrol Dial 2019;9:33 –41 (Source: Case Reports in Nephrology and Dialysis)
Source: Case Reports in Nephrology and Dialysis - April 24, 2019 Category: Urology & Nephrology Source Type: research

Rapidly Progressive Glomerulonephritis with Delayed Appearance of Anti-Glomerular Basement Membrane Antibody Successfully Treated with Multiple Courses of Steroid Pulse Therapy
Patients with anti-glomerular basement membrane (GBM) antibody glomerulonephritis typically exhibit rapidly progressive glomerulonephritis (RPGN). The renal outcome as well as the prognosis of this disease is worse than other forms of RPGN such as those from microscopic polyangiitis. Therefore, early therapeutic intervention is essential to improve its prognosis. One month before referral to our hospital, a 54-year-old female attended another hospital because of macrohematuria. At that time, she had proteinuria and macrohematuria with normal renal function, was negative for anti-GBM antibodies, and was diagnosed with chron...
Source: Case Reports in Nephrology and Dialysis - April 16, 2019 Category: Urology & Nephrology Source Type: research

MPGN Type 3 Associated with Pemphigus Herpetiformis Mimicking PGNMID and Dermatitis Herpetiformis
A 45-year-old man suffering from dermal blistering disease with proteinuria and hematuria underwent renal biopsy. The renal biopsy specimen suggested proliferative glomerulonephritis with monoclonal IgG deposits under routine light, immunofluorescence and electron microscopy. The staining for IgG subclasses (IgG1 and IgG2) and κ/λ light chain indicated secondary immune complex type MPGN type 3. The patient had been diagnosed as having dermatitis herpetiformis (DH), a phenotype of gluten hypersensitivity prior to the appearance of the renal abnormality. Although common autoantibodies might be related to the pathogenesis o...
Source: Case Reports in Nephrology and Dialysis - March 21, 2019 Category: Urology & Nephrology Source Type: research

Monotypic IgG1-kappa Atypical Anti-Glomerular Basement Membrane Nephritis: A Case Report
Anti-glomerular basement membrane (anti-GBM) glomerulonephritis is a rare disease caused by autoantibodies against the glomerular basement membrane. Atypical anti-GBM nephritis is clinically less aggressive and characterized by the absence of circulating autoantibodies to the basement membrane. A previously healthy 53-year-old white woman presented with a rising creatinine over a short observation period. Renal biopsy, urinary sediment, and laboratory testing confirmed the diagnosis of atypical anti-GBM disease. She received plasmapheresis, steroids, and cyclophosphamide. She developed hemorrhagic cystitis early in the tre...
Source: Case Reports in Nephrology and Dialysis - March 7, 2019 Category: Urology & Nephrology Source Type: research

Progressive Renal Dysfunction due to IgG4-Related Kidney Disease Refractory to Steroid Therapy: A Case Report
Recently, as the number of case reports of IgG4-related kidney disease (IgG4-RKD) has increased, the histopathological features and clinical approach have been clarified. IgG4-RKD generally has a benign prognosis due to the efficacy of steroid therapy and rarely requires dialysis. Herein, we report a case of IgG4-RKD that presented with a subacute onset, advanced to end-stage kidney disease, and finally required maintenance hemodialysis despite steroid therapy. A 75-year-old man was admitted to our hospital for further evaluation of subacute renal failure. Diffuse enlargement of the kidney on computed tomography and increa...
Source: Case Reports in Nephrology and Dialysis - February 8, 2019 Category: Urology & Nephrology Source Type: research