Development of IgA vasculitis with severe glomerulonephritis after COVID-19 vaccination: a case report and literature review
We present the case of a 67-year-old Japanese woman who developed IgAV with purpura on her extremities and trunk in the evening of the day that she received the second dose of the Pfizer-BioNTech COVID-19 vaccine. She subsequently presented with acute kidney injury and nephrotic syndrome, and a kidney biopsy performed 14 days after the second vaccination showed diffuse mesangial and endocapillary glomerulonephritis with necrotizing crescent formation, accompanied by IgA deposition. One steroid pulse plus four administrations of a monthly intravenous cyclophosphamide injection were applied, followed by oral azathio prine ...
Source: CEN Case Reports - March 11, 2022 Category: Urology & Nephrology Source Type: research
A rare case of atypical ANCA-associated vasculitis without crescents overlapping with invasive pulmonary aspergillosis, successfully treated to remission with intravenous immunoglobulin therapy
We describe a 74-year-old man who was diagnosed with AAV following the aggravation of chronic pulmonary aspergillosis that required an aggressive antifungal agent. The laboratory data on admission demonstrated severe kidney failure requiring hemodialysis. Due to the active infection, we chose intravenous immunoglobulin (IVIg) as a low-risk initial treatment, which remarkably improved renal dysfunction (serum creatinine; 16.7 mg/dL–3.7 mg/dL) and systemic inflammation. Renal biopsy that was performed after renal recovery revealed atypical ANCA-associated nephritis without cellular crescents but with massive arteritis w...
Source: CEN Case Reports - March 10, 2022 Category: Urology & Nephrology Source Type: research
A case of COVID-19 reinfection in a hemodialysis patient: the role of antibody in SARS-CoV-2 infection
In conclusion, we experienced a case of COVID-19 reinfection in a hemodialysis patient 81 days after the first episode and showed the kinetics and role of antibodies in SARS-CoV-2 infection. Further studies are needed to understand SARS-CoV-2 reinfection risk in hemodialysis patients and its clinical significance. (Source: CEN Case Reports)
Source: CEN Case Reports - March 9, 2022 Category: Urology & Nephrology Source Type: research
A case of juvenile-onset fibrillary glomerulonephritis diagnosed by mass spectrometry and immunohistochemistry of DNAJB9
AbstractFibrillary glomerulonephritis (FGN) is a rare glomerular disease. FGN is characterized by the deposition of randomly arranged, nonbranching microfibrils in the mesangium and glomerular basement membrane. The discovery of DNAJ homolog subfamily B member 9 (DNAJB9) in 2017 was a breakthrough, and DNAJB9 has been proven to be extremely useful for the definitive diagnosis of FGN. While FGN often occurs in middle-aged individuals, this case was diagnosed at a relatively young age of 17. We performed renal biopsy, and light microscopic study revealed mesangial proliferation with expansion and subepithelial deposits. Elec...
Source: CEN Case Reports - February 23, 2022 Category: Urology & Nephrology Source Type: research
Clinical exome sequencing uncovers an unsuspected diagnosis of Bartter syndrome type 2 in a child with incidentally detected nephrocalcinosis
We describe an 11-year-old girl with incidentally detected medullary nephrocalcinosis on renal ultrasonography. She was clinically suspected to have primary hyperoxaluria based on high urine oxalate. However, clinical exome sequencing revealed a pathogenic missense variant in the KCNJ1 gene leading to the molecular diagnosis of Bartter syndrome type 2. Both parents were heterozygous carriers of the same variant. Subsequent investigations did reveal a mild Bartter syndrome phenotype with mild metabolic alkalosis, high urine chloride and high renin and aldosterone. Our case illustrates phenotypic heterogeneity of Bartter syn...
Source: CEN Case Reports - February 23, 2022 Category: Urology & Nephrology Source Type: research
A case of a preschool child with a successful kidney transplant following the long-term administration of antibiotics to treat peritoneal dialysis-related ESI/peritonitis by Mycobacterium abscessus
AbstractA preschool child with refractory peritoneal dialysis-related exit-site infection (ESI)/peritonitis caused byMycobacterium abscessus (M. abscessus) received multidrug antibacterial therapy for 6 months and then successfully underwent living-donor kidney transplantation. The patient was a 2.7-year-old boy and the primary disease was bilateral hypo/dysplastic kidneys. Peritoneal dialysis (PD) was initiated at the age of 4 months. Purulent drainage from the PD catheter exit site was observe d, and pus and PD effluent cultures were negative. Since living kidney transplantation was scheduled for 2 months later, the ...
Source: CEN Case Reports - February 18, 2022 Category: Urology & Nephrology Source Type: research
Identification of causative gene mutation in an Iranian family with coloboma and nephropathy using whole exome sequencing
AbstractPathogenic variants inFAT1 gene have recently been described in association with coloboma, nephropathy, and facial dismorphism. Here we describe a 5-year-old Iranian boy with iris coloboma and nephropathy, born to an Iranian family. Extracted genomic DNA from blood sample was used to perform whole exome sequencing in the patient. The mutational screening revealed a homozygote Fat1 gene mutation c.5320A > G (p.17747Val), not previously reported in homozygote state in Iran. Our findings establishFAT1 as a gene with pleiotropic effects in human, emphasizing it as one of the causative genes in syndromic neph...
Source: CEN Case Reports - February 18, 2022 Category: Urology & Nephrology Source Type: research
Bilateral renal subcapsular hematoma caused by polyarteritis nodosa: a case report
In conclusion, this is a rare case of polyarteritis nodosa manifesting as spontaneous bilateral subcapsular renal hemorrhage with deteriorated renal function, which was successful ly treated with immunosuppressive therapy. (Source: CEN Case Reports)
Source: CEN Case Reports - February 16, 2022 Category: Urology & Nephrology Source Type: research
A case of pathologically confirmed streptococcal infection-related IgA vasculitis with associated glomerulonephritis and leukocytoclastic cutaneous vasculitis
We report the case of an 80 year-old woman who developed bilateral lower extremity purpura and renal impairment with proteinuria a few days after a transient fever (day 0). High levels of both anti-streptolysin-O antibody (ASO) and anti-streptokinase antibody (ASK), as well as low levels of coagulation factor XIII in serum w ere noted. Skin biopsy was performed and showed a leukocytoclastic vasculitis with deposition of IgA and C3 in the cutaneous small vessels, indicating IgA vasculitis in the skin. After initiation of oral prednisolone, the skin lesions showed significant improvement. However, renal function and prote ...
Source: CEN Case Reports - February 14, 2022 Category: Urology & Nephrology Source Type: research
A case of acute focal bacterial nephritis with acute kidney injury presenting as acute abdomen
In conclusion, clinicians should be aware of AFBN and be cautious to avoid the unnecessary invasive interventions. (Source: CEN Case Reports)
Source: CEN Case Reports - February 6, 2022 Category: Urology & Nephrology Source Type: research
Proliferative glomerulonephritis with monoclonal immunoglobulin deposits triggered by COVID-19: a case report
We present a case of dialysis-dependent AKI developing soon after symptomatic COVID-19 which, on kidney biopsy, was found to be due to PGNMID with IgG3 kappa deposits. As is typical of PGNMID, a search for evidence of extra-renal monoclonal immunoglobulin or clonal lymphocyte population was negative. However, the patient had a favorable response to anti-plasma cell therapy and was ultimately able to stop hemodialysis. Though monoclonal gammopathy of renal significance (MGRS) is usually not associated with infection, other cases of post-viral MGRS, including PGNMID, have been previously reported. PGNMID has recently been li...
Source: CEN Case Reports - February 4, 2022 Category: Urology & Nephrology Source Type: research
An adolescent girl diagnosed with IgA nephropathy following the first dose of the COVID-19 vaccine
AbstractThere has been growing interest in reported cases of IgA nephropathy (IgAN) flare-up following administration of the coronavirus disease 2019 (COVID-19) vaccine. Our patient is a previously healthy 17-year-old girl who presented with a 10-year history of microscopic hematuria. Because there were no abnormal findings in blood examination or ultrasonography, we followed her up twice per year as asymptomatic hematuria. Although she never developed gross hematuria when she had upper respiratory infections or received an influenza vaccine, she presented with gross hematuria and proteinuria within a few days after receiv...
Source: CEN Case Reports - February 3, 2022 Category: Urology & Nephrology Source Type: research
Concomitant pleuritis and pericarditis developing during glucocorticoid therapy: a case report on granulomatosis with polyangiitis
AbstractGranulomatosis with polyangiitis is an anti-neutrophil cytoplasmic antibody-associated vasculitis that manifests in various ways by affecting the small-sized vessels in multiple organs. Acute pleuritis and pericarditis are both rare among the different manifestations of granulomatosis with polyangiitis. The symptoms in each of the organs are often apparent at the time of diagnosis and tend to diminish with treatment. Organ damage and progression of the disease during treatment are uncommon. We encountered a patient with granulomatosis with polyangiitis who, after starting intravenous methylprednisolone pulse therap...
Source: CEN Case Reports - February 2, 2022 Category: Urology & Nephrology Source Type: research
Elevation of serum fibroblast growth factor 23 level in a pediatric patient with lupus nephritis
AbstractFibroblast growth factor 23 (FGF-23), a hormone mainly secreted by osteocytes and osteoblasts, regulates phosphate and vitamin D levels. However, the in vivo significance of FGF-23 is not fully elucidated. This case report describes a 12-year-old girl with systemic lupus erythematosus (SLE), lupus nephritis, and an elevated serum FGF-23 level. The patient was treated with active vitamin D and oral sodium phosphate medications to manage low serum phosphate levels (2.2 mg/dL). Magnetic resonance imaging (MRI) revealed a high-intensity area in the left femur, but somatostatin receptor scintigraphy images did not ind...
Source: CEN Case Reports - February 1, 2022 Category: Urology & Nephrology Source Type: research
Tubulointerstitial nephritis in antineutrophil cytoplasmic antibody-associated vasculitis with monoclonal gammopathy
AbstractIsolated tubulointerstitial nephritis (TIN) without glomerular crescent formation is a rare manifestation of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Some patients with monoclonal gammopathy of undetermined significance present with renal complications due to serum monoclonal protein. Here, we present a case of TIN presumably attributable to AAV with monoclonal gammopathy. Laboratory data revealed acute kidney injury, elevated C-reactive protein (CRP) and ANCA titers, and elevated tubular injury markers. Renal biopsy revealed TIN with no apparent glomerular lesion. The findings of per...
Source: CEN Case Reports - February 1, 2022 Category: Urology & Nephrology Source Type: research
