A case of idiopathic nodular glomerulosclerosis successfully treated by intensive blockade of the renin –angiotensin–aldosterone system
AbstractIdiopathic nodular glomerulosclerosis has a poor renal prognosis and is characterized by diffuse nodular glomerulosclerotic lesions in the absence of diabetic mellitus. Here, we report the case of a 69-year-old woman with no smoking history who developed renal dysfunction and proteinuria in the absence of overt diabetes or obesity. A biopsy specimen showed nodular mesangial sclerosis with arteriolar hyalinosis and severe large-vessel arteriosclerosis, leading to a diagnosis of idiopathic nodular glomerulosclerosis. Addition of esaxerenone to her existing renin –angiotensin–aldosterone inhibitor therapy led to a...
Source: CEN Case Reports - December 27, 2022 Category: Urology & Nephrology Source Type: research

Microbiome of infected cysts, feces and saliva in patients with autosomal dominant polycystic kidney disease
We report a 66-year-old woman and a 56-year-old man with ADPKD who had severe hepatic cyst infection. We analyzed the microbiome of infected cyst content, feces, and saliva in these two patients. The microbiome of patient 1 showed various bacteria in an infected cyst, whereas that of patient 2 showed only one bacterium. In both patients, the composition of the microbiome of the cyst content was quite different from those of feces and saliva, and the main bacteria in the infected cyst content represented a small proportion of those in feces and saliva.Lactobacilliwere not almost detected in the infected cyst content though ...
Source: CEN Case Reports - December 27, 2022 Category: Urology & Nephrology Source Type: research

Asymptomatic pulmonary thromboembolism due to hemodialisys catheter thrombosis: case series and literature review
AbstractRight atrial thrombus is commonly associated to catheters. Catheter-related right atrial thrombus (CRAT) in hemodialysis patients frequently presents as pulmonary embolism. Although CRAT is sometimes asymptomatic, even in these cases it is associated with worse prognosis. The management strategy for CRAT is not well established, however, along with catheter removal, anticoagulation, thrombolysis, and surgical thrombectomy may be performed. Suspicion of asymptomatic pulmonary embolism associated to CRAT is important in order to perform proper treatment. The authors of this article report two cases of asymptomatic pu...
Source: CEN Case Reports - December 27, 2022 Category: Urology & Nephrology Source Type: research

Central diabetes insipidus with anti-rabphilin-3A antibody positivity causing hypovolemic shock after resection of tumorous lesions in the pelvic cavity
AbstractA 36-year-old female was pointed out to have liver enzyme elevation by routine health checkup. Subsequent contrast-enhanced CT scan identified gigantic uterine fibroids and retroperitoneal tumor. She was referred to the gynecologist at JA Toride Medical Center and planned to undergo a uterus enucleation and biopsy of the retroperitoneal tumor. The surgery was conducted without any troubles. After the surgery, the patient presented polyuria with urine volume 10 –20 L a day and developed hypovolemic shock. Laboratory test revealed hypotonic urine and hypernatremia. Arginine vasopressin (AVP) loading test suggested ...
Source: CEN Case Reports - December 27, 2022 Category: Urology & Nephrology Source Type: research

Short-term effects of roxadustat on serum copper and iron changes in a peritoneal dialysis patient
AbstractDysregulation in total body copper causes severe complications and excess copper can be toxic.Divalent metal transporter 1,duodenal cytochrome B, and copper transporter ATPase7A are included in the many intestinal genes transactivated by HlF- α. On July X, 2022 an 80-year-old female patient on peritoneal dialysis was prescribed roxadustat 100 mg, because darbepoetin was unable to increase hemoglobin level effectively. On the same day, icodextrin 1 L was initiated to mitigate edema. Laboratory data showed hemoglobin 9.1 g/dL, transferr in saturation 77%, copper 123 μg/dL, and iron 170 μg/dL before changing to...
Source: CEN Case Reports - December 15, 2022 Category: Urology & Nephrology Source Type: research

Hilar lymphadenopathy, development of tubulointerstitial nephritis, and dense deposit disease following Pfizer-BioNTech COVID-19 vaccination
AbstractDespite the reports on glomerulonephritis associated with COVID-19 mRNA vaccines, no study has reported about the dense deposit disease (DDD). Here, we present a case of hilar lymphadenopathy after the COVID-19 mRNA vaccination, following which the patient developed tubulointerstitial nephritis (TIN) and DDD. A 74-year-old man received his second dose of mRNA vaccine, and on the next day, he developed fever, urticaria, and dyspnea. On further examination, he had pleural effusion and right hilar lymphadenopathies, which were improved with conservative therapy. After 48  days of the second vaccination, he developed ...
Source: CEN Case Reports - December 13, 2022 Category: Urology & Nephrology Source Type: research

An 8-month-old boy with infantile nephrotic syndrome caused by semaphorin 3B-associated membranous nephropathy
We present a case of nephrotic syndrome caused by semaphorin 3B-associated membranous nephropathy. The patient was an 8-month-old male infant who presented with severe proteinuria and hypertension. He was treated with prednisolone (PSL) for nephrotic syndrome; however, remission was not achieved within 4  weeks. He was diagnosed with steroid-resistant nephrotic syndrome and underwent kidney biopsy. Pathological examination revealed membranous nephropathy with IgG deposits on both the glomerular basement membrane (GBM) and the tubular basement membrane (TBM). He was treated with cyclosporine (CsA) i n addition to PSL and a...
Source: CEN Case Reports - December 12, 2022 Category: Urology & Nephrology Source Type: research

IgA nephropathy diagnosed as a result of acute exacerbation due to G-CSF administration
We report a case of immunoglobulin A (IgA) nephropathy that was aggravated by G-CSF. A 56-year-old Japanese man with no relevant medical history was admitted to our hospital as a donor of peripheral blood stem cells (PBSCs) for transplantation. To mobilize PBSCs, he received subcutaneous G-CSF (lenograstim), 500  μg for 4 days. Three days after the first dose of lenograstim, gross hematuria appeared, and after administration on the fourth day, renal dysfunction and nephrotic-range proteinuria were observed. Renal biopsy and light microscopic study revealed mild mesangial proliferation with expansion in a ssociation with...
Source: CEN Case Reports - December 12, 2022 Category: Urology & Nephrology Source Type: research

Nephrotic-range proteinuria and membranoproliferative glomerulonephritis-like pattern caused by interferon- β1b in a patient with multiple sclerosis
AbstractInterferon-beta (IFN- β) subtypes are widely used as immunomodulatory agents for relapsing–remitting multiple sclerosis (MS). Although generally well tolerated, a growing number of reports have recently shown association of long-term IFN-β therapy with several types of glomerulonephritis. Here, we present the case of a 42-year-old woman with MS who developed nephrotic-range proteinuria after taking IFN-β1b for nine years. Initially, due to the presence of histological features consistent with immunoglobulin A (IgA) nephropathy (granular IgA deposits in mesangial lesions), a tonsillectomy plus steroid pulse the...
Source: CEN Case Reports - December 12, 2022 Category: Urology & Nephrology Source Type: research

A rare case of nephrotic syndrome and Tangier disease
We report a patient presenting with nephrotic syndrome, leading to the unmasking of Tangier disease. A 34-year-old man presented with ankle oedema, nephrotic-range proteinuria and hypoalbuminaemia. Kidney biopsy demonstrated membranous nephropathy with features of immunoperoxidase staining, suggesting a secondary aetiology. Acute serology was negative. Imaging showed lymphadenopathy with splenomegaly suggestive of lymphoproliferative disorder. Bone marrow biopsy revealed foamy macrophages with widespread lipid deposition. Genomic sequencing revealed a pathological homozygous variant for ATP-binding cassette subfamily A mem...
Source: CEN Case Reports - December 10, 2022 Category: Urology & Nephrology Source Type: research

Anti-glomerular basement membrane disease developing 3  years after the development of Sweet syndrome and 1 year after the development of anti-neutrophil cytoplasmic antibody-associated vasculitis: a case report
AbstractA 73-year-old Japanese woman, with a history of Sweet syndrome diagnosed 3  years earlier and anti-myeloperoxidase (MPO) antibody anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis diagnosed 1 year earlier, presented with an episode of rapidly progressive glomerulonephritis (RPGN) with anti-glomerular basement membrane (GBM) disease. At the time of diagnos is of the ANCA-associated vasculitis 1 year earlier, serological testing yielded a negative result for anti-GBM antibody. However, at the present visit, serology for anti-MPO antibody was negative, while that for anti-GBM antibody was positive. T...
Source: CEN Case Reports - December 1, 2022 Category: Urology & Nephrology Source Type: research

A case of severe acute hyponatremia after colonoscopy with polyethylene glycol plus ascorbic acid bowel preparation
We present the case of an 86-year-old man who developed severe acute hyponatremia presenting with tremor and impaired consciousness after colonoscopy, which is life-threatening. The severe hyponatremia in our case was not caused by free water loads from drinking large amounts of water during bowel preparation or hypovolemia due to bowel preparation-induced nausea, vomiting, and diarrhea, but might have been due to non-osmotic stimuli of antidiuretic hormone (ADH) release (i.e., pre-existing nausea, stress, anxiety, pain, stress, or the colonoscopy itself). Our study indicates that it is important to choose safer bowel prep...
Source: CEN Case Reports - November 29, 2022 Category: Urology & Nephrology Source Type: research

A novel combination of OHVIRA syndrome and likely causal variant in UMOD gene
We report a patient who was diagnosed to have unilateral renal agenesis in early childhood, and then presented after menarche with features of OHVIRA syndrome. The contralateral kidney was relatively small and echogenic, and serum creatin ine and uric acid were raised. A likely causal variant of theUMOD gene was detected on whole exome sequencing. Genetic studies in more patients with OHVIRA syndrome may elucidate further, whether the association withUMOD gene is causal in nature. (Source: CEN Case Reports)
Source: CEN Case Reports - November 22, 2022 Category: Urology & Nephrology Source Type: research

Utility of renal biopsy in differentiating idiopathic multicentric Castleman disease from IgG4-related disease
We report the case of a woman in her 80  s who was diagnosed with iMCD based on renal pathological findings. Five years ago, hypergammaglobulinemia was detected, and her renal function declined. Renal biopsy revealed plasma cells infiltrating the stroma. Immunostaining revealed numerous IgG4-positive plasma cells. The serum IgG4 level wa s high, and she was initially diagnosed with IgG4-related disease (IgG4-RD) and treated with steroids. However, the therapeutic effect was poor. On re-examination, computed tomography revealed lymphadenopathy around the aorta and spleen. Renal histopathology showed numerous IL-6-positive ...
Source: CEN Case Reports - November 21, 2022 Category: Urology & Nephrology Source Type: research

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