Two cases of nivolumab plus ipilimumab therapy for dialysis patients with advanced bone metastasis from renal cell carcinoma
AbstractNivolumab and ipilimumab are immune checkpoint inhibitors. Combination therapy with these two drugs has been shown to improve the outcome of advanced renal cell carcinoma. However, data about the safety and the efficacy of combination therapy with these two drugs in hemodialysis patients are small. A 59-year-old male hemodialysis patient presented with bone metastasis from renal cell carcinoma, which was located at the right femur. He received nivolumab plus ipilimumab therapy. At 7 months after treatment, he was diagnosed with diabetes as an immune-related adverse event. He was managed with insulin therapy. At 11 ...
Source: CEN Case Reports - November 19, 2022 Category: Urology & Nephrology Source Type: research

A case of diffuse idiopathic hyperostosis of bone (DISH) in a patient on hemodialysis
AbstractDiffuse idiopathic skeletal hyperostosis (DISH) is a noninflammatory progressive disease resulting in ossification of the anterior longitudinal ligament of the spine and tendons. Herein, we describe a case of DISH in a patient on long-term hemodialysis. The patient was a 79-year-old man undergoing hemodialysis for chronic kidney disease due to diabetic nephropathy. He presented to the emergency department complaining of back pain after a slip and fall. Radiographs revealed bamboo spine-like findings, extending from the cervical to the lumbar spine. Computed tomography and magnetic resonance imaging revealed a compr...
Source: CEN Case Reports - November 19, 2022 Category: Urology & Nephrology Source Type: research

Anti-glomerular basement membrane glomerulonephritis concurrent with membranous nephropathy and acute tubular interstitial nephritis in a lung cancer patient treated with pembrolizumab
We report the case of a 65-year-old man with lung adenocarcinoma who was treated with pembrolizumab (a monoclonal antibody targeting programmed cell death protein-1 [PD-1]). Pembrolizumab was discontinued after seven cycles due to the development of destructive thyroiditis. Within three  months of discontinuing the pembrolizumab treatment, the patient developed rapid progressive glomerulonephritis (RPGN), liver dysfunction, and dysgeusia. The patient underwent renal biopsy and was diagnosed with crescentic glomerulonephritis due to anti-glomerular basement membrane (GBM) antibodie s complicated with membranous nephropathy...
Source: CEN Case Reports - November 19, 2022 Category: Urology & Nephrology Source Type: research

Allograft adenovirus nephritis accompanied by Crohn ’s disease in a kidney transplant recipient: a novel case report
We report a novel case wherein a kidney transplant recipient developed severe acute allograft injury and hemorrhagic cystitis at 4.5  years after KT due to adenovirus nephritis after treatment with infliximab for Crohn's disease. The diagnosis was made based on adenovirus immunohistochemistry staining and urine polymerase chain reaction tests. The patient was successfully treated by reducing IMs and administration of immunoglobu lin even though allograft function was eventually partially recovered. When new immunosuppressive agents, particularly biologic agents, are initiated for other diseases in addition to maintenance ...
Source: CEN Case Reports - November 18, 2022 Category: Urology & Nephrology Source Type: research

Valacyclovir-associated acute kidney injury and encephalopathy in an elderly woman with normal kidney function: a case report
AbstractA 72-year-old Japanese woman was treated by 3000  mg/day of valacyclovir for the herpes zoster in her left back. She had been treated as hypertension with no renal insufficiency. In two days, she visited an emergency room of a regional stroke care center with dysarthria, dexterity disorder and gait disturbance. Neither head CT nor MRI found intra cranial lesions, then, laboratory tests revealed that her serum creatinine level was 4.63 mg/dL. She was transferred and admitted to our hospital on the following day and received hemodialysis under the diagnosis of AKI due to acyclovir accompanied with encephalopathy. A...
Source: CEN Case Reports - November 18, 2022 Category: Urology & Nephrology Source Type: research

Rare perinephric myxoid pseudotumor of fat causing ureteropelvic junction obstruction: a case report
We describe a rare presentation of extrinsic perinephric myxoid pseudotumor of fat causing UPJ obstruction. In elderly patients with no history of malignancy, UPJ obstruction can occur because of atypical masses. (Source: CEN Case Reports)
Source: CEN Case Reports - November 15, 2022 Category: Urology & Nephrology Source Type: research

A case of chyluria with nephrotic-range proteinuria caused by lymphatic malformation, leading to a diagnosis of Klippel –Trenaunay syndrome
AbstractKlippel –Trenaunay syndrome (KTS) is a rare syndrome, which is clinically diagnosed by the presence of unilateral limb hypertrophy with vascular malformation including cutaneous capillaries, veins and lymphatic vessels. Most cases typically exhibit cutaneous manifestations such as port-wine stains and lim b hypertrophy from infancy, but cases with mild manifestations may remain undiagnosed. We here report a case of KTS who was diagnosed by chance chyluria. A 15-year-old girl who exhibited hematochyluria with nephrotic-range proteinuria was referred to our hospital. She had been diagnosed as idiopathi c scoliosis ...
Source: CEN Case Reports - November 11, 2022 Category: Urology & Nephrology Source Type: research

Tubulointerstitial nephritis with IgA kappa-positive plasma cells in a patient with primary Sj ögren’s syndrome and monoclonal gammopathy
AbstractThis is a case report of a 69-year-old Japanese man who has been undergoing treatment for primary Sj ögren’s syndrome (pSS) since he was 62 years. A renal biopsy, which revealed diffuse and severe mononuclear cell infiltration in the tubulointerstitium, was performed because of progressive renal dysfunction. Immunostaining demonstrated most of the infiltrating cells to be IgA, kappa, CD38, and CD138 positive. Immunofixation blood test revealed IgA kappa-type M protein; however, bone marrow abnormalities or lymph node enlargements on examination or imaging, respectively, were not observed. Tubulointerstitial nep...
Source: CEN Case Reports - November 11, 2022 Category: Urology & Nephrology Source Type: research

Methanol toxicity in a pediatric patient treated with fomepizole and hemodialysis
We present the case of a 16-year-old male with a past medical history of depression who intentionally ingested windshield wiper fluid containing methanol. Based on the patient ’s osmolal gap, he was estimated to have a serum methanol level of 374 mg/dL; a send-out laboratory measurement later revealed a serum methanol level of 436 mg/dL. Therapy included two hemodialysis treatments as well as fomepizole and supportive care. The patient recovered remarkably with no lon g-term sequelae. This case demonstrates the effectiveness of swift recognition and treatment of methanol ingestion. Optimization of methods of measuring ...
Source: CEN Case Reports - October 28, 2022 Category: Urology & Nephrology Source Type: research

A case of nephrogenic diabetes insipidus likely caused by anti-neutrophil cytoplastic antibody-associated vasculitis
AbstractAnti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a relatively rare form of autoimmune disease. Diabetes insipidus (DI) is characterized by diluted polyuria and thirstiness, and is clinically categorized into central and nephrogenic DI depending on damaged organs. In most previously reported cases, ANCA-related disorders have been implicated in central DI, which is attributed to impaired secretion of arginine vasopressin (AVP) from the posterior pituitary. However, no previous case of AAV-related nephrogenic DI has been reported in the English literature. Herein, we report a case of nephrog...
Source: CEN Case Reports - October 18, 2022 Category: Urology & Nephrology Source Type: research

A case of Potter sequence with WT1 mutation
We report an unusual case of Potter sequence due to fetal nephropathy and kidney failure with aWT1 mutation. The neonate was born at 37  weeks of gestation, and had no distinctive facial appearance or anomalies of the extremities. The external genitalia were ambiguous. Presence of a penile-like structure or hypertrophic clitoris was noted, and the urethra opened at the base of the penis or clitoris. On ultrasonographic examination, the kidney sizes were small. No kidney cysts were noted, but the kidney parenchymal luminosity was increased. Although the neonate received mechanical ventilation because of severe retractive b...
Source: CEN Case Reports - October 13, 2022 Category: Urology & Nephrology Source Type: research

Comparison of renal histopathology in three patients with gross hematuria after SARS-CoV-2 vaccination
We present three cases of IgA nephropathy with gross hematuria following severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) messenger RNA (mRNA) vaccination. Case 1 was a 60-year-old woman who has previously experienced transient proteinuria. Case 2 was a 22-year-old woman with no history of urinary abnormality. Finally, case 3 was a 66-year-old woman who has had microscopic hematuria since she was in her 50s. They were all diagnosed as IgA nephropathy with little histological active lesion. Their renal function and proteinuria improved without the use of corticosteroids. There were differences in the findings of...
Source: CEN Case Reports - October 11, 2022 Category: Urology & Nephrology Source Type: research

Monitoring of anti-drug antibodies and disease-specific biomarkers in three patients from a Japanese Fabry family treated with enzyme replacement therapy
AbstractWe monitored anti-drug antibodies and disease-specific biomarkers in three patients with a nonsense mutation from a Japanese Fabry family treated with enzyme replacement therapy (ERT). In two male patients from the family, neutralizing anti-drug antibodies were induced at an early stage of ERT, the antibody titer peak being found at an earlier stage of ERT in the patient treated with 1.0  mg/kg agalsidase beta than in that treated with 0.2 mg/kg agalsidase alfa. Then, the antibody titers decreased with continuation of ERT. The formation of neutralizing anti-drug antibodies adversely affected the plasma globotriao...
Source: CEN Case Reports - October 7, 2022 Category: Urology & Nephrology Source Type: research

Marked reduction of proteinuria after removal of a growth hormone-producing pituitary adenoma in a patient with focal segmental glomerulosclerosis: a case report and literature review
AbstractFocal segmental glomerulosclerosis is a rare complication of acromegaly. A 74-year-old man was found to have acromegaly features such as enlargement of the forehead, nose, and hands. Laboratory tests showed a urine protein/creatinine ratio of 3.16  g/gCr and serum creatinine of 1.34 mg/dL. The levels of growth hormone and insulin-like growth factor I were markedly elevated, and the growth hormone level was not suppressed after 75 g oral glucose loading. Magnetic resonance imaging revealed a pituitary tumor with a diameter of 1.2 cm. Renal biopsy confirmed the diagnosis of focal segmental glomerulosclerosis. Tra...
Source: CEN Case Reports - October 6, 2022 Category: Urology & Nephrology Source Type: research

A case report of invasive infantile primary hyperoxaluria type 1 and literature review
We report a case of a three-month-old female Chinese infant who was diagnosed with PH1 by renal biopsy and genetic studies. She carried two heterozygous mutations in the alanine-glyoxylate and serine pyruvate aminotransferase (AGXT) gene, one of which has never been previously reported. The patient had multiple organ failures caused by kidney failure, which was improved by extracorporeal membrane oxygenation and continuous renal replacement therapy. However, her primary disease responded poorly to conservative treatment. Fortunately, after waiting for four months, the patient underwent a successful combined liver-kidney tr...
Source: CEN Case Reports - October 4, 2022 Category: Urology & Nephrology Source Type: research