Novel Variant of < b > < i > AVPR2 < /i > < /b > Giving Rise to X-Linked Congenital Nephrogenic Diabetes Insipidus in a 7-Month-Old Danish Boy
We present a Danish male suffering from typical symptoms and diagnosed with CNDI at the age of 7 months. Gene sequencing of this proband and his mother revealed a novel variant in the gene encoding the antidiuretic hormone receptor (AVPR2). The variant is a deletion of nucleotide c.151 in exon 2 ofAVPR2 (GenBank NM_000054.4:c.151del). This 1bp deletion is predicted to cause a frameshift that results in tryptophan replacing valine at position 51 inAVPR2 and a premature stop codon three codons downstream (p.Val51Trpfs*3) likely resulting in faulty expression of the receptor. Identification of disease-causing variants such as...
Source: Case Reports in Nephrology and Dialysis - October 14, 2020 Category: Urology & Nephrology Source Type: research

Neuroblastoma Amplified Sequence Gene Mutations Inducing Acute Kidney and Liver Injury in an Adolescent Female
Acute liver injury (ALI) in children is a life-threatening event, and a definitive etiology can be identified in approximately 50% of cases. Neuroblastoma amplified sequence (NBAS) gene mutations have been associated with a broad phenotypic spectrum of this disease, ranging from recurrent episodes of fever-induced liver injuries to multiorgan involvement, including frequent infections as well as skeletal and immunological abnormalities. Here, we describe an adolescent female with a confirmed compound heterozygous NBAS gene mutation who presented with an episode of ALI complicated by severe acute kidney injury (AKI). The ki...
Source: Case Reports in Nephrology and Dialysis - October 13, 2020 Category: Urology & Nephrology Source Type: research

A Case Demonstrating the Pathological Relationship between Granulomatous Vasculitis and Glomerular Lesion in Renal Sarcoidosis
We experienced a rare case of tubulointerstitial angiocentric granulomatous vasculitis with focal segmental glomerulosclerosis (FSGS) and associated sarcoidosis. Our patient was an 18-year-old man who presented with exertional cough and dyspnea. He also had overt proteinuria (3.0 g/24 h), normal renal function (eGFR 95 mL/min/1.73 m2), heart failure, and hypertension. He had no previous episode of hypertension. These manifestations immediately improved after the administration of antihypertensive therapy that contained an angiotensin-converting enzyme inhibitor, calcium antagonists, beta antagonists, and diuretics. However...
Source: Case Reports in Nephrology and Dialysis - October 12, 2020 Category: Urology & Nephrology Source Type: research

Limited Treatment Options in Primary Hyperoxaluria with Renal Failure
This report details a unique presentation of a rare disease where kidney biopsy was instrumental.Case Rep Nephrol Dial 2020;10:104 –108 (Source: Case Reports in Nephrology and Dialysis)
Source: Case Reports in Nephrology and Dialysis - October 5, 2020 Category: Urology & Nephrology Source Type: research

Multiple Myeloma-Associated Light Chain Amyloidosis and a Proposed Approach to Monoclonal Immunoglobulin-Associated Renal Disease
Many challenges remain in diagnosing monoclonal immunoglobulin-associated renal disease, despite widespread application of immunofluorescence (IF) and immunohistochemistry. Here, we report a newly diagnosed case of multiple myeloma with clinical suspicion of renal amyloidosis, which had negative IF staining for kappa and lambda light chains in the glomeruli. Although laser microdissection and mass spectrometry-based proteomic analysis have emerged as important tools for amyloid typing in the literature, such facilities are still not widely available in Asia. We propose that a clinicopathological algorithm for the evaluatio...
Source: Case Reports in Nephrology and Dialysis - September 11, 2020 Category: Urology & Nephrology Source Type: research

Critical Coronavirus Disease 2019 in a Hemodialysis Patient: A Proposed Clinical Management Strategy
We report our experience in treating the first case of COVID-19 in a HD patient in Singapore who had a severe clinical course including acute respiratory distress syndrome and propose a clinical management strategy. We propose a clinical workflow in managing such patients based on available evidence from literature review. We also highlight the importance of early recognition and intervention for disease control, dialysis support in an acute hospital isolation facility, deisolation protocol, and discharge planning due to prolonged viral shedding. The case highlights important points specific to a HD patient with a COVID-19...
Source: Case Reports in Nephrology and Dialysis - July 30, 2020 Category: Urology & Nephrology Source Type: research

A Case of Myeloma Kidney with Perinuclear Anti-Neutrophil Cytoplasmic Antibody and Anti-Myeloperoxidase Positivity: The Importance of Determining the True Cause of Renal Impairment
We present the case of a 60-year-old man who presented with clinical features suggestive of AAV in the context of blood tests which demonstrated AKI and positive perinuclear ANCA (p-ANCA) and anti-myeloperoxidase (anti-MPO) titres. Further investigations demonstrated an underlying diagnosis of MM. A renal biopsy was ultimately required to determine the cause of AKI, a cast nephropathy. This case is the first to our knowledge which demonstrates a rare situation in which myeloma kidney is associated with positive p-ANCA and anti-MPO titres, without any evidence of a crescentic GN. It highlights the importance of following up...
Source: Case Reports in Nephrology and Dialysis - July 29, 2020 Category: Urology & Nephrology Source Type: research

A Family with Gitelman Syndrome with Asymptomatic Phenotypes while Carrying Reported SLC12A3 Mutations
Gitelman syndrome (GS) is an autosomal recessive disorder characterized by alkalosis, hypokalemia, and hypomagnesemia. Although hundreds of genetic variants associated with GS have been reported, many of them are categorized as of uncertain significance in ClinVar. Here, we describe a pediatric GS patient from a three-generation family whose mother and maternal grandmother were asymptomatic. The proband was a 16-year-old Japanese girl with muscle weakness and continuous hypokalemic metabolic alkalosis. The patient, her mother, and her maternal grandmother were compound heterozygous for, and each expressing a different comb...
Source: Case Reports in Nephrology and Dialysis - July 13, 2020 Category: Urology & Nephrology Source Type: research

Peritoneal Dialysis Zoonotic Bacterial Peritonitis with < b > < i > Staphylococcus pseudintermedius < /i > < /b >
We present the first reported case of peritoneal dialysis-associated peritonitis caused byStaphylococcuspseudintermedius, an organism that had been misclassified asS.aureus in the past.S.pseudintermedius is well recognized in the veterinary literature and noted as flora in the mouth, nares, and anus of domesticated animals. It has been associated with soft tissue infections in pets and is now being reported in increased frequency as the causative agent in various human infections. It also has a different antibiotic sensitivity profile. The patient had close contact with her pet dog and was successfully treated with intrave...
Source: Case Reports in Nephrology and Dialysis - June 12, 2020 Category: Urology & Nephrology Source Type: research

Giant Brachial Aneurysm after Arteriovenous Fistula Ligation: A Review of the Different Surgical Approaches
We describe our experience and our surgical approach. A 45-year-old man presented to our attention for a large pulsatile formation on the volatile face of the left forearm, which he reported to have enlarged in the last year. He had a history of chronic renal impairment in 2000, then AVF for dialysis was realized, and he was finally addressed to kidney transplantation in 2004. In 2011 the AVF was ligated. We observed absence of radial pulse and direct flow on the ulnar artery; a large pulsatile formation was evident along the course of the left brachial artery, associated with forearm venous dilatation. Doppler ultrasound ...
Source: Case Reports in Nephrology and Dialysis - May 27, 2020 Category: Urology & Nephrology Source Type: research

Cat Rearing: A Potential Risk of Fulminant Sepsis Caused by < b > < i > Capnocytophaga canimorsus < /i > < /b > in a Hemodialysis Patient
Capnocytophaga canimorsus is a commensal organism colonized in oral flora of dogs and cats and causes severe sepsis through bite wound in immunocompromised patients. To date, hemodialysis has not been reported as a risk ofC. canimorsus infection. A 75-year-old woman with end-stage renal disease secondary to hypertension suddenly developed septic shock. She reared 6 cats in her home, but no bite or scratch wound was found on her body. She was empirically treated with piperacillin-tazobactam and temporally received continuous hemodiafiltration. On the fifth day after sampling, blood culture revealedC. canimorsus as the cause...
Source: Case Reports in Nephrology and Dialysis - May 13, 2020 Category: Urology & Nephrology Source Type: research

Acute Kidney Injury Associated with Minimal Change Nephrotic Syndrome in an Elderly Patient Successfully Treated with both Fluid Management and Specific Therapy Based on Kidney Biopsy Findings
Oliguric acute kidney injury (AKI) with minimal change nephrotic syndrome (MCNS) has long been recognized. Several mechanisms such as hypovolemia due to hypoalbuminemia and the nephrosarca hypothesis have been proposed. However, the precise mechanism by which MCNS causes AKI has not been fully elucidated. Herein, we describe an elderly patient with AKI caused by MCNS who fully recovered after aggressive volume withdrawal by hemodialysis and administration of a glucocorticoid. A 75-year-old woman presented with diarrhea and oliguria, and laboratory examination revealed nephrotic syndrome (NS) and severe azotemia. Fluid admi...
Source: Case Reports in Nephrology and Dialysis - April 22, 2020 Category: Urology & Nephrology Source Type: research

Catheter Access Management for Acute Peritoneal Dialysis
Insertion of a peritoneal dialysis (PD) catheter is frequently done by interventional nephrologists, but these procedures are typically only performed for adults. Almost all invasive procedures in children are performed by pediatric surgeons. If a pediatric surgeon is unavailable, the initiation of PD in acute situations may be delayed, thus increasing the risk of complications and chronic kidney disease. For these patients, the main obstacle to initiating renal replacement therapy is access, even when involving central vein catheter (CVC) or peritoneal access. Here we report the case of a 10-kg baby affected by hemolytic ...
Source: Case Reports in Nephrology and Dialysis - April 14, 2020 Category: Urology & Nephrology Source Type: research

A Case of Acute Kidney Injury in a Patient with Renal Hypouricemia without Intense Exercise
Exercise-induced acute kidney injury (EIAKI) frequently develops in patients with renal hypouricemia (RHUC). However, several cases of RHUC with acute kidney injury (AKI) but without intense exercise have been reported. We encountered a 15-year-old male with RHUC who experienced AKI. He reported no episodes of intense exercise and displayed no other representative risk factors of EIAKI, although a vasopressor had been administered for orthostatic dysregulation before AKI onset. His kidney dysfunction improved with discontinuation of the vasopressor and conservative treatment. Thus, AKI can develop in patients with RHUC in ...
Source: Case Reports in Nephrology and Dialysis - March 30, 2020 Category: Urology & Nephrology Source Type: research

Effective Management of Peritoneal Dialysis-Associated Hydrothorax in a Child: A Case Report
Peritoneal dialysis (PD) confers many advantages, including a better quality of life for children with end-stage renal disease; however, the procedure is associated with several complications, including pleuroperitoneal leaks. Here, we report an unusual case of hydrothorax caused by long-term PD in a child, which was further complicated by pneumonia. A 9-year-old boy who had received CAPD for 22 months presented with dyspnea, swelling, and increased body weight. Chest tube drainage yielded 500 mL of transudative fluid. Computed tomography peritoneography revealed increased outflow from the peritoneum to the pleural cavity....
Source: Case Reports in Nephrology and Dialysis - February 12, 2020 Category: Urology & Nephrology Source Type: research

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 hig...
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...
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...
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 ...
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 pat...
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

Lumbar Artery Bleed as a Complication of Percutaneous Renal Biopsy and a Proposed Workflow for Massive Bleeding
Injuries to extrarenal arteries caused by percutaneous biopsy needles are very rare but highly lethal due to delay in recognition. Here we report the case of an inadvertent lumbar artery puncture after native renal biopsy and provide a literature review and a proposed workflow for management of massive bleed after renal biopsy. This case highlights evidence-based management considerations regarding massive bleed after renal biopsy, including the first-line imaging modality and the need to consider extrarenal site bleed. While angiographic embolization is an effective method of control of haemorrhage, surgical exploration i...
Source: Case Reports in Nephrology and Dialysis - December 13, 2018 Category: Urology & Nephrology Source Type: research

A Case of Rapid Progressive Kidney Dysfunction with Severely Calcified Stenotic Aorta
We present a case of coral reef aorta with severe abdominal aortic stenosis in a 67-year-old man. The patient presented with hypertension, claudication, and rapid progression of renal dysfunction over several months. Angiography revealed a severely stenotic suprarenal abdominal aorta resulting in renal ischemia and dysfunction. In addition, his right kidney was completely atrophied. After open surgical repair of the stenotic aorta including renal artery reconstruction, renal function did not improve. There was stenotic anastomosis to the renal artery. After endovascular therapy to the stenotic anastomosis, renal function d...
Source: Case Reports in Nephrology and Dialysis - November 27, 2018 Category: Urology & Nephrology Source Type: research

A Case Report of Cronkhite-Canada Syndrome Complicated by Membranous Nephropathy
Cronkhite-Canada syndrome (CCS) is a very rare disorder with less than 500 reported cases. It is characterized by extensive gastrointestinal polyposis and ectodermal anomalies including alopecia, cutaneous hyperpigmentation, and onychodystrophy. Only 3 cases of associated kidney disease (membranous nephropathy [MN]) have been reported. A 71-year-old male with CCS was referred for further evaluation of proteinuria. The patient initially presented with abdominal discomfort, weight loss, dysgeusia, skin hyperpigmentation, alopecia, and dystrophic nails. Endoscopic evaluation showed widespread gastrointestinal nodular inflamma...
Source: Case Reports in Nephrology and Dialysis - November 27, 2018 Category: Urology & Nephrology Source Type: research

A Case of Self-Limiting Crescentic Immunoglobulin A Glomerulonephritis Associated with Sternoclavicular Arthritis
We describe a rare case of crescentic IgA GN with sternoclavicular (SC) arthritis in a 75-year-old woman. Despite active IgA GN with cellular crescents, the patient achieved remission of IgA GN without glucocorticoid therapy a fter remission of SC arthritis was achieved. Considering the patient’s clinical course, this case suggested a relationship between IgA GN and SC arthritis.Case Rep Nephrol Dial 2018;8:246 –252 (Source: Case Reports in Nephrology and Dialysis)
Source: Case Reports in Nephrology and Dialysis - November 21, 2018 Category: Urology & Nephrology Source Type: research

Peritoneal Dialysis Exit Site Pyoderma Gangrenosum: A Case Report
Conclusions: Lesions at the PD catheter exit site are usually treated for infections. However, for ulcers that are painful, rapidly expanding, nonhealing, and unresponsive to antibiotics, PG should be considered as a differential diagnosis. This is the first reported case of PG occurring at the exit site of a PD catheter.Case Rep Nephrol Dial 2018;8:239 –245 (Source: Case Reports in Nephrology and Dialysis)
Source: Case Reports in Nephrology and Dialysis - November 14, 2018 Category: Urology & Nephrology Source Type: research

Erratum
Case Rep Nephrol Dial 2018;8:238 (Source: Case Reports in Nephrology and Dialysis)
Source: Case Reports in Nephrology and Dialysis - November 7, 2018 Category: Urology & Nephrology Source Type: research

Efficacy of Semiannual Single Fixed Low-Dose Rituximab Therapy in Steroid-Dependent Minimal Change Nephrotic Syndrome: A Case Series
The frequency of using rituximab to treat refractory nephrotic syndrome has recently been increasing, and the conventional dose of rituximab used to treat it, 375 mg/m2 body surface area once weekly for 4 weeks, has been modelled on the chemotherapy regimen for B-cell non-Hodgkin ’s lymphoma. The dose and intervals of rituximab in refractory nephrotic syndrome remain controversial. Clear lymphoma cell hyperplasia is seen in lymphoma patients, but not in nephrotic syndrome patients. Since we thought that it might be possible to reduce the dose of rituximab if only used for the purpose of depleting CD20-positive B cell...
Source: Case Reports in Nephrology and Dialysis - October 24, 2018 Category: Urology & Nephrology Source Type: research

Three Severe Cases of Viral Infections with Post-Kidney Transplantation Successfully Confirmed by Polymerase Chain Reaction and Flow Cytometry
Viral infections in patients with post-kidney transplantation are often difficult to diagnose as well as treat. We herein report three cases with severe viral infections after kidney transplantation. All their causative pathogens could be detected promptly by polymerase chain reaction and flow cytometry during the early stages of infection. These examinations would also be of great use to monitor therapeutic responses and disease activity. It is indeed true that no specific treatment is available for most of the viral infections, but we should be aware that some infections, such as Epstein-Barr virus infection, can be trea...
Source: Case Reports in Nephrology and Dialysis - October 5, 2018 Category: Urology & Nephrology Source Type: research

Bile Cast Nephropathy: A Pathologic Finding with Manifold Causes Displayed in an Adult with Alcoholic Steatohepatitis and in a Child with Wilson ’s Disease
We present 2 diverse cases of BCN. The first involves an adult with hepatorenal syndrome secondary to alcoholic steatohepatitis and early cirrhosis. Second, we describe the first reported case of BCN in a child with fulminant hepatic failure due to Wilson’s disease. Our cases expand the spectrum of causative diseases, and they provide further evidence that BCN is a distinct pathologic entity w hich may be found in both adult and pediatric patients with a variety of severe liver diseases.Case Rep Nephrol Dial 2018;8:207 –215 (Source: Case Reports in Nephrology and Dialysis)
Source: Case Reports in Nephrology and Dialysis - October 5, 2018 Category: Urology & Nephrology Source Type: research

Bioptically Proven “Anticoagulation-Related Nephropathy“ Induced by Dual Antiplatelet Therapy
We present a case of an 82-year-old man who developed biopsy-proven ARN after the administration of dual antiplatelet therapy with no previous anticoagulation treatment and normal coagulation tests.Case Rep Nephrol Dial 2018;8:216 –222 (Source: Case Reports in Nephrology and Dialysis)
Source: Case Reports in Nephrology and Dialysis - October 5, 2018 Category: Urology & Nephrology Source Type: research

Primary Hyperoxaluria Type 1 with Thrombophilia in Pregnancy: A Case Report
Conclusions: The diagnosis of PH should be considered in patients with severe, recurrent calcium oxalate nephrolithiasis. Early treatment with pyridoxine reduces urinary oxalate excretion and can delay progression to end-stage renal disease (ESRD). After ESRD, intensive dialysis is needed to prevent systemic oxalate accumulation and deposition. Combined liver and kidney transplantation is curative. In our patient, we anticipate that liver transplantation will cure both the hyperoxaluria and the hypercoagulable state.Case Rep Nephrol Dial 2018;8:223 –229 (Source: Case Reports in Nephrology and Dialysis)
Source: Case Reports in Nephrology and Dialysis - October 5, 2018 Category: Urology & Nephrology Source Type: research

Asymptomatic Lupus Cystitis with Bilateral Hydronephrosis
We report a 22-year-old female patient who presented at Cipto Mangunkusumo Teaching Hospital in Indonesia, with profuse and prolonged vaginal bleeding without any other accompanying symptoms. She had a history of polyarthralgias, fever, bleeding gums, anemia, and thrombocytopenia 3 months earlier. Abdominal ultrasound examination revealed bilateral hydronephrosis and a thickened bladder wall; the other organs were normal. Laboratory examination confirmed the diagnosis of SLE complicated by lupus nephritis and lupus cystitis. The patient responded well to the treatment with methylprednisolone. The vaginal bleeding stopped w...
Source: Case Reports in Nephrology and Dialysis - September 18, 2018 Category: Urology & Nephrology Source Type: research

Minimal Change Nephrotic Syndrome Relapse after 52 Years of Remission: A Case Report
Minimal change nephrotic syndrome (MCNS) is the most common cause of nephrotic syndrome in children and can also present in adults. Corticosteroids generally induce remission of MCNS, and relapses are common after reduction or discontinuation of corticosteroids. We experienced a rare case of steroid-sensitive MCNS where the patient relapsed after 52 years of remission. The patient was a 61-year-old Japanese male who visited our clinic for an edema of the lower extremities which had already persisted for a few days. Laboratory testing showed massive urinary protein and low serum total protein and albumin levels. Therefore, ...
Source: Case Reports in Nephrology and Dialysis - September 2, 2018 Category: Urology & Nephrology Source Type: research

Fibrillary Glomerulonephritis with Crescentic and Necrotizing Glomerulonephritis and Concurrent Thrombotic Microangiopathy
We present a 77-year-old Caucasian woman who presented with nephrotic-range proteinuria, microhematuria, renal impairment, and extremely elevated blood pressure. She had a long history of well-controlled type 2 diabetes. Renal biopsy revealed fibrillary deposits in the mesangium and glomerular basement membrane consistent with fibrillary glomerulopathy (FGN), with crescentic changes and thrombotic microangiopathy (TMA). We could not identify any radiological, clinical, or laboratory evidence of autoimmune disorders, lymphoproliferative disorders, and malignancy. It was decided not to offer her any immunosuppressive therapy...
Source: Case Reports in Nephrology and Dialysis - September 2, 2018 Category: Urology & Nephrology Source Type: research

A Case of Chronic Calcium Oxalate Nephropathy due to Short Bowel Syndrome and Cholecystectomy
Conclusion: Case reports of chronic oxalate neuropathy are rare in the literature, and its underlying mechanism has not been understood. Our patient had a history of small bowel resection and cholecystectomy. We considered that her short bowel syndrome had influenced the development of calcium oxalate nephropathy.Case Rep Nephrol Dial 2018;8:147 –154 (Source: Case Reports in Nephrology and Dialysis)
Source: Case Reports in Nephrology and Dialysis - August 10, 2018 Category: Urology & Nephrology Source Type: research

Disseminated Strongyloidiasis in Association with Nephrotic Syndrome
We present a case of disseminated strongyloidiasis in a patient with minimal change nephrotic syndrome treated with high-dose corticosteroids. The remission of nephrotic syndrome after treatment of strongyloidiasis suggests a possible causal relationship between Strongyloides and nephrotic syndrome.Case Rep Nephrol Dial 2018;8:155 –160 (Source: Case Reports in Nephrology and Dialysis)
Source: Case Reports in Nephrology and Dialysis - August 10, 2018 Category: Urology & Nephrology Source Type: research

Case Report: Patient with Hepatitis C, p-ANCA, and Cryoglobulin Antibodies Presenting with Necrotizing Crescentic p-ANCA Glomerulonephritis
We present a case of HCV-associated glomerular disease with the surprising biopsy finding of necrotizing and crescentic p-ANCA GN, with a background, low-grade mesangial immune complex GN. Thus, p-ANCA disease should also be considered in HCV-infected patients, in addition to the more typical lesions of MPGN or cryoglobulinemic GN.Case Rep Nephrol Dial 2018;8:161 –170 (Source: Case Reports in Nephrology and Dialysis)
Source: Case Reports in Nephrology and Dialysis - August 10, 2018 Category: Urology & Nephrology Source Type: research

Acute Kidney Injury after Pembrolizumab-Induced Adrenalitis and Adrenal Insufficiency
We report a 70-year-old Caucasian male who underwent treatment with pembrolizumab for adenocarcinoma of the lung. He developed acute adrenal insufficiency and concomitant severe hypotension upon presentation. He did not require renal replacement therapy, rather his severe acute kidney injury resolved with hydration, normalization of blood pressures with vasopressors, and treatment with high-dose corticosteroids. His urinary indices (fractional excretion of urea, FEUrea) and clinical course were highly suspicious for acute tubular necrosis that resolved quickly after treating his underlying adrenalitis. The urinary sediment...
Source: Case Reports in Nephrology and Dialysis - August 10, 2018 Category: Urology & Nephrology Source Type: research

Acute Kidney Failure as a Single Complication of Varicella Virus Infection in an Adult Patient
Conclusion: VZV infection occurs in a range of clinical scenarios, sometimes presenting only with mild symptoms, but in some other setting it can result in severe AKF even in healthy kidneys. Acute VZV infection can lead to isolated, clinically significant kidney failure. The administration of continuous renal replacement therapy and adjusted doses of acyclovir has a favorable effect on the course of the infection, with complete recovery of kidney function.Case Rep Nephrol Dial 2018;8:130 –137 (Source: Case Reports in Nephrology and Dialysis)
Source: Case Reports in Nephrology and Dialysis - August 3, 2018 Category: Urology & Nephrology Source Type: research

A Rare Case of Severe Metabolic Alkalosis with Unusual Hyperproteinemia Treated with Continuous Renal Replacement Therapy and Regional Citrate Anticoagulation
A 23-year-old woman was referred to the tertiary centre with acute kidney injury and severe metabolic alkalosis following an accidental ethylene glycol poisoning. The patient had been treated with continuous haemodiafiltration and regional citrate anticoagulation, and a tracheostomy was performed due to pneumonia. Besides severe metabolic alkalosis and hypernatremia, the laboratory tests revealed total protein of 108 g/L on admission to the tertiary centre. The haemodiafiltration with regional citrate anticoagulation continued with parallel correction of the alkalosis and normalisation of the total plasma protein. The trac...
Source: Case Reports in Nephrology and Dialysis - August 3, 2018 Category: Urology & Nephrology Source Type: research