Neural epidermal growth factor-like 1 protein (NELL1)-associated membranous nephropathy with heterogeneous underlying diseases: a case report
We present a case of a 78-year-old woman with long-standing RA who developed persistent proteinuria and was diagnosed with MN. Evaluation of the underlying cause revealed chronic active HCV infection and past HBV infection. The underlying cause was less likely to be drug-related; however, there was no evidence of malignancy. The patient was diagnosed with HCV-associated MN. At 4 years after the diagnosis of MN, the patient died of breast cancer with multiple metastases. Subsequent immunohistological analysis revealed that she had NELL1-MN, and her breast cancer tissue stained positive for NELL1. Our case illustrates the di...
Source: CEN Case Reports - October 28, 2023 Category: Urology & Nephrology Source Type: research
Familial focal segmental glomerulosclerosis with Alport-like glomerular basement changes caused by paired box protein 2 gene variant
AbstractPaired box protein 2 (PAX2) gene variant causes renal coloboma syndrome (MIM#120330). Further, they are associated with focal segmental glomerulosclerosis and characterized by basement membrane changes similar to Alport syndrome.Herein, we report an 8-year-old boy who presented with proteinuria and decreased renal function. His paternal uncle has focal segmental glomerulosclerosis and renal failure, and his paternal grandmother has renal failure and is receiving peritoneal dialysis. Further, his father has stage 2 chronic kidney disease. At 3 years of age, his serum creatinine-estimated glomerular filtration rate...
Source: CEN Case Reports - October 28, 2023 Category: Urology & Nephrology Source Type: research
Complications and prognosis of patients diagnosed with autosomal recessive polycystic kidney disease in neonatal period
AbstractThere are no clinical guidelines for performing nephrectomy in patients with autosomal recessive polycystic kidney disease (ARPKD). Few reports have described the clinical course of ARPKD diagnosed in the neonatal period in detail. Here, we report seven patients diagnosed with ARPKD and treated at our center during the neonatal period. Two died within 48 h of life due to pulmonary hypoplasia. Of the remaining five patients, three had anuria and required for kidney replacement therapy (KRT) within one week after birth, whereas two with a milder phenotype survived without KRT. All three patients who received KRT un...
Source: CEN Case Reports - October 25, 2023 Category: Urology & Nephrology Source Type: research
Pauci-immune crescentic glomerulonephritis caused to dilemma in a patient with suspected systemic lupus erythematosus: a case report
We present a young male who was admitted to the emergency with syncope. The brain imaging revealed small infarct areas and signs of cerebral vasculitis. Also, he had elevated inflammatory markers, moderate proteinuria, and preserved kidney function. Anti-nuclear antibodies and anti-dsDNA were positive. Pauci-immune crescentic glomerulonephritis (PICGN) was observed in a kidney biopsy, however, ANCA was negative. SLE diagnosis was established by neurological manifestation, specific antibodies, proteinuria, and kidney biopsy findings. We administered a combination induction regimen, including pulse steroid and parenteral cyc...
Source: CEN Case Reports - October 14, 2023 Category: Urology & Nephrology Source Type: research
Mixed cryoglobulinaemia vasculitis secondary to marginal zone lymphoma in a patient with end-stage renal failure on haemodialysis
We report a case of a 63-year-old male who had a series of presentations with rash, visual changes, abdominal pain, weight loss, fevers and digital ischaemia. This is on a background of a congenital single kidney with end-stage renal failure secondary to diabetes and hypertension, receiving haemodialysis for nearly 5 years. He initially experienced a leukocytoclastic vasculitis rash confirmed on skin biopsy, followed by multiple hospital presentations for undifferentiated abdominal pain and fever of unknown source. Jejunal biopsy revealed intestinal vasculitis. His peripheral blood flow cytometry and bone marr ow biopsy ...
Source: CEN Case Reports - October 6, 2023 Category: Urology & Nephrology Source Type: research
A case of emphysematous pyelonephritis in an older man with poorly controlled type 2 diabetes mellitus
We present the case of a 75-year-old man who was diagnosed early with EPN and received multidisciplinary treatment. He had poorly controlled type 2 diabetes mellitus (DM) and chronic kidney disease (CKD), and was treated with oral hypoglycemic drugs, including a sodium –glucose co-transporter-2 inhibitor. He experienced the onset of back pain in the midsection of his back, tenderness in the costovertebral angle, and a high fever (> 39 °C), accompanied by tachycardia, hypotension, and tachypnea. The patient was diagnosed with pyelonephritis and septic shock. Immediate measures encompassing empirical antibiotic the...
Source: CEN Case Reports - September 22, 2023 Category: Urology & Nephrology Source Type: research
Nephrotic syndrome induced by aortic regurgitation with Takayasu arteritis: an autopsy case with long-term clinical follow-up
AbstractTakayasu arteritis is a rare, chronic, and large-vessel vasculitis involving the aorta and its branches in a complex autoimmune reaction. Takayasu arteritis sometimes complicates aortic regurgitation and chronic kidney disease, but rarely accompanies nephrotic syndrome. We had a patient with Takayasu arteritis and concomitant aortic regurgitation. She had nephrotic syndrome that was refractory to immunosuppressive therapy but was promptly improved after surgical aortic valve replacement. In her kidney biopsy, glomeruli had mild mesangial proliferative changes without immune complex deposition. Her proteinuria remai...
Source: CEN Case Reports - September 22, 2023 Category: Urology & Nephrology Source Type: research
List of referees
(Source: CEN Case Reports)
Source: CEN Case Reports - September 22, 2023 Category: Urology & Nephrology Source Type: research
Importance of education on steroid cover: a case of hyponatremia after dental extraction with local anesthesia in a patient with idiopathic hypopituitarism
(Source: CEN Case Reports)
Source: CEN Case Reports - September 22, 2023 Category: Urology & Nephrology Source Type: research
Herpes zoster in a patient with first onset of childhood nephrotic syndrome following the second SARS-CoV-2 vaccination
(Source: CEN Case Reports)
Source: CEN Case Reports - September 11, 2023 Category: Urology & Nephrology Source Type: research
Concurrent emphysematous cystitis and emphysematous pyelonephritis in a patient with extrarenal pelvis
We report co-occurrence of emphysematous cystitis and emphysematous pyelonephritis (EC/EPN) in a 64-year-old female with poor-controlled diabetes mellitus (DM) that presented with flank pain, fever, and hematuria that turned out to have a bilateral extrarenal pelvis. On examination, she was feverish, and the costovertebral angle was tender. By considering herhemoglobin A1C, her DM was out of control. Inflammatory markers elevated. Renal function tests were impaired. Urine culture was positive for extended-spectrum beta-lactamaseEscherichia coli. Computed tomography scans (CT) confirmed the presence of air in the bladder an...
Source: CEN Case Reports - September 7, 2023 Category: Urology & Nephrology Source Type: research
A successful treatment for chronic active Epstein-Barr virus disease with Nephrotic Syndrome
In this report, we show that dose reduction of immunosuppressive agents wi thout chemotherapy or HSCT is an effective option for the treatment of CAEBV disease in patients receiving immunosuppressive agents. (Source: CEN Case Reports)
Source: CEN Case Reports - September 5, 2023 Category: Urology & Nephrology Source Type: research
Author's reply to “Immune complex-mediated glomerulonephritis with ANCA positivity: what should nephrologists consider?”
(Source: CEN Case Reports)
Source: CEN Case Reports - September 1, 2023 Category: Urology & Nephrology Source Type: research
A case of carcinoid syndrome probably exacerbated by hemodialysis in which prochlorperazine maleate was effective
We report the case of a patient with liver metastasis of neuroendocrine carcinoma who suffered from acute abdominal pain and diarrhea triggered by hemodialysis. Various differential diagnoses were considered, but we concluded these symptoms to be probably caused by exacerbation of carcinoid syndrome, as the serum 5HIAA level was markedly elevated, and a drug with anti-serotonin activity was effective. Prochlorperazine maleate, which has anti-serotonin activity, was effective for these symptoms, and the patient was able to continue maintenance hemodialysis, which contributed to his quality of life and prognosis. We speculat...
Source: CEN Case Reports - August 22, 2023 Category: Urology & Nephrology Source Type: research
Proliferative glomerulonephritis with monoclonal immunoglobulin deposits and atypical pathological findings treated with corticosteroid and rituximab
AbstractA 16-year-old girl with fever that appeared after taking the second COVID-19 vaccine presented to the clinic with a serum creatinine of 0.89 mg/dL and C-reactive protein of 6.9 mg/dL. She had proteinuria and microscopic hematuria, with slowly worsening kidney function. Her kidney biopsy showed fibrocellular crescents in seven of nine glomeruli that were observed under light microscopy. Another glomerulus showed endocapillary hypercell ularity and mesangial cell proliferation. Electron-dense deposits were significant in the mesangial area, with monoclonal IgG1-κ and C3 deposition by immunofluorescence. The patie...
Source: CEN Case Reports - August 7, 2023 Category: Urology & Nephrology Source Type: research
