Thrombotic microangiopathy in a patient with systemic lupus erythematosus and anti-factor H autoantibodies
We present a case of a 24-year-old man, previously diagnosed with SLE, class IV lupus nephritis, and antiphospholipid antibody syndrome, who was admitted with acute kidney injury, severe pancytopenia, and other features consistent with lupus flare. A clinical TMA diagnosis was made and the patient was treated with plasmapheresis, rituximab and immunoglobulin endovenous (EV) infusions. Hemodialysis was initiated during hospitalization and, despite the hematological recovery, the patient remained dialysis dependent. The complementary study revealed high levels of anti-factor H (fH) autoantibodies with no pathogenic mutations...
Source: CEN Case Reports - February 1, 2022 Category: Urology & Nephrology Source Type: research

A case of early recurrent immunoglobulin A nephropathy and T-cell-mediated rejection in a transplant patient with Wiskott –Aldrich syndrome
AbstractWiskott –Aldrich syndrome (WAS) is an X-chromosome recessive immunodeficiency disease characterized by the triad of thrombocytopenia, eczema, and susceptibility to infection owing to WAS protein gene abnormalities. Kidney transplantation is rarely offered to WAS patients with end-stage renal disease becau se of concerns that thrombocytopenia and immune disorders may affect the clinical outcome. Here, we report the case of a 20-year-old kidney transplant patient who developed end-stage renal disease owing to immunoglobulin (Ig)A nephropathy caused by WAS. Despite recurrent IgA nephropathy and T-cell-m ediated reje...
Source: CEN Case Reports - February 1, 2022 Category: Urology & Nephrology Source Type: research

A complicated case of Serratia marcescens catheter-related bloodstream infection misdiagnosed as hypersensitivity reactions to bicarbonate dialysate containing acetate
We present the case of a 79-year-old patient who had persistent post-dialysis fever after long-term tunneled central venous catheterization with acetate-containing bicarbonate dialysate. Drug-induced lymphocyte stimulation test (DLST) was positive for acetate dialysate, and he was suspected of having acetate dialysate-induced hypersensitivity reaction. However, switching to acetate-free dialysate did not attenuate the fever. SinceSerratia marcescens had been isolated twice from the blood, catheter-related bloodstream infection (CRBSI) was suspected. The culture of the catheter tip confirmed CRBSI caused byS. marcescens. El...
Source: CEN Case Reports - February 1, 2022 Category: Urology & Nephrology Source Type: research

Two episodes of acute dyspnea that were induced by COVID-19 in a peritoneal dialysis patient
AbstractDialysis patients have an increased risk of coronavirus disease 2019 (COVID-19)-related mortality. Acute heart failure is a frequent, lethal complication of COVID-19, and it is a risk factor for mortality in hemodialysis patients. Therefore, it is crucial to rapidly distinguish heart failure from COVID-19 pneumonia. Here, we report a case of two episodes of acute dyspnea that were induced by COVID-19 in a peritoneal dialysis (PD) patient. The first episode of acute dyspnea was an exacerbation of heart failure caused by COVID-19 when the patient had a volume overload status due to a peritoneal dialysis catheter malf...
Source: CEN Case Reports - February 1, 2022 Category: Urology & Nephrology Source Type: research

A case of Dent disease type 2 with large deletion of OCRL diagnosed after close examination of a school urinary test
AbstractA 7-year-old boy visited our hospital for a detailed examination of proteinuria identified in a school urinary test. He had short stature, misaligned teeth, and mild intellectual disability. A urinary examination identified mild proteinuria and extremely high levels of beta-2 microglobulin. On blood examination, his protein, albumin, and creatinine levels were found to be normal; however, his lactate dehydrogenase and creatinine phosphokinase levels were slightly elevated. Upon histological examination, no abnormalities in glomeruli or tubules were found. Considering these results, we diagnosed our patient with Den...
Source: CEN Case Reports - January 31, 2022 Category: Urology & Nephrology Source Type: research

Efficacy of vesicostomy for refractory metabolic acidosis in persistent cloaca
This report suggests vesicostomy as an effective treatment for refractory hyperchloremic metabolic acidosis associated with high-confluence type persistent cloaca. (Source: CEN Case Reports)
Source: CEN Case Reports - January 31, 2022 Category: Urology & Nephrology Source Type: research

New-onset kidney biopsy-proven IgA vasculitis after receiving mRNA-1273 COVID-19 vaccine: case report
AbstractAs mRNA COVID-19 vaccines have become widely available, cases of new-onset glomerular disease after receiving COVID-19 vaccination have been reported. Here, we present a case of kidney biopsy-proven new-onset IgA vasculitis after receiving the mRNA-1273 (Moderna) COVID-19 vaccination. A 47-year-old man with a 10-year medical history of hypertension and hyperuricemia visited our hospital 19  days after receiving an initial mRNA-1273 COVID-19 vaccine injection for purpuric eruption on the legs and dorsal regions of the feet. Although the eruptions spontaneously improved within 5 days, they developed again at 15 da...
Source: CEN Case Reports - January 25, 2022 Category: Urology & Nephrology Source Type: research

Effect of loaded exercise for renal osteodystrophy
AbstractWe performed bone histomorphometric analysis of biopsy specimens from two patients with hyper- and hypoparathyroidism and a history of long-term hemodialysis (HD) because of diabetes. Case 1, a 53-year-old man with hyperparathyroidism, had been on HD for 22  years, and Case 2, a 54-year-old woman with hypoparathyroidism, for 20 years. Intact parathyroid hormone levels were 1070 and 3 pg/mL, respectively. Case 1 had mixed renal osteodystrophy (fibrous tissue volume to total volume [Fb.V/TV], 5.21%; osteoid volume to bone volume [OV/BV], 19.8%), and C ase 2 had adynamic renal osteodystrophy (Fb.V/TV, 0%; OV/BV, 0....
Source: CEN Case Reports - January 21, 2022 Category: Urology & Nephrology Source Type: research

Atypical manifestations of infantile-onset nephropathic cystinosis: a diagnostic challenge
AbstractA 7-month-old male infant was referred to us for evaluation of hypercalcemia and failure to thrive. He was the second-born child to third-degree consanguineous parents with a birth weight of 3.5  kg. The index child was severely underweight. Initial laboratory investigations showed hypercalcemia (13.6 mg/dL), hypophosphatemia, hyponatremia, hypokalemia and hypochloremia. The initial serum bicarbonate level was 20.9 mEq/L. The urine calcium: creatinine ratio (0.05) was normal. He was note d to have polyuria (6 mL/kg/hr) and required intravenous fluids to maintain intravascular volume and manage hypercalcemia, al...
Source: CEN Case Reports - January 20, 2022 Category: Urology & Nephrology Source Type: research

A case of proliferative glomerulonephritis with monoclonal IgG3 κ deposits accompanied by glomerular capillary microaneurysms
AbstractGlomerular capillary aneurysms are distinctly rare and specific glomerular lesions characterized by aneurysmal dilatation of the glomerular capillaries. This formation is associated with glomerular capillary injuries with focal mesangiolysis. Here, we report a case of proliferative glomerulonephritis with monoclonal immunoglobulin G deposits (PGNMID) presenting with multiple glomerular capillary microaneurysms. A 53-year-old woman presented with persistent proteinuria and microhematuria. She had no underlying diseases, such as hematopoietic or lymphoproliferative disorders. A renal biopsy showed diffuse membranopro...
Source: CEN Case Reports - January 13, 2022 Category: Urology & Nephrology Source Type: research

Involvement of neutrophil extracellular traps in the pathogenesis of glomerulonephritis in a case of systemic lupus erythematosus and antineutrophil cytoplasmic antibody-associated vasculitis overlap syndrome
In this study, we detected renal deposition of NETs in a patient with SLE/AAV overlap syndrome. In addition, a significantly increased level of NET-inducing activity was observed in the patient ’s serum, which improved with treatment. On the other hand, a markedly lower level of NET degradation was observed in the patient’s serum as compared to healthy subjects’ sera, without any posttreatment changes. These findings suggest that NETs may play a role in the pathogenesis of renal inju ry associated with SLE/AAV overlap syndrome. (Source: CEN Case Reports)
Source: CEN Case Reports - January 13, 2022 Category: Urology & Nephrology Source Type: research

Safety of casirivimab/imdevimab administration in a SARS-CoV-2 positive maintenance dialysis patient in Japan
AbstractControlling excessive cytokine secretion is a crucial therapeutic strategy for managing coronavirus disease 2019 (COVID-19). Patients on dialysis are at a high risk of severe disease, given abnormal immune responses that can lead to prolonged inflammation. Moreover, patients undergoing dialysis have limited treatment options, as neither remdesivir nor baricitinib is available. The novel neutralizing monoclonal antibody cocktail REGEN-COV (formerly known as REGN-COV2; casirivimab/imdevimab), recently approved in Japan, is a promising drug for preventing severe diseases. However, there are few reports regarding its u...
Source: CEN Case Reports - January 9, 2022 Category: Urology & Nephrology Source Type: research

Influenza-induced thrombotic microangiopathy in a patient with cancer on proteasome inhibitor: a diagnostic dilemma
We present such a diagnos tic dilemma in our patient who was admitted with influenza and was found to have TMA on renal biopsy, while on proteasome inhibitor (PI) therapy. With this case, we would like to highlight the importance of understanding the true cause of TMA to avoid unwarranted long-term discontinuation of life sa ving anti-cancer drugs after TMA resolution. (Source: CEN Case Reports)
Source: CEN Case Reports - January 7, 2022 Category: Urology & Nephrology Source Type: research

Abrupt worsening of occult IgA nephropathy after the first dose of SARS-CoV-2 vaccination
AbstractHere, we report a case of abrupt onset of gross hematuria and nephrotic range proteinuria after the first dose of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination, which led to a diagnosis of immunoglobulin A nephropathy (IgAN). A Japanese woman in their forties with a significant medical history of occult blood by urine dipstick test (over the past 3  years) presented with fever, chills, shivering, marked thrombocytopenia, and gross hematuria 9 days after the first dose of the BNT162b2 mRNA vaccine (Pfizer) against SARS-CoV-2 infection. Although thrombotic microangiopathy (TMA) was first ...
Source: CEN Case Reports - January 6, 2022 Category: Urology & Nephrology Source Type: research

A case of membranous nephropathy secondary to asymptomatic Graves ’ disease
AbstractAlthough cases of secondary membranous nephropathy associated with autoimmune thyroid disease (AITD) have been reported, most of them, if not all, present with symptomatic thyroid disease. Here we report an asymptomatic case of AITD complicated with secondary membranous nephropathy. A 16-year-old girl was referred to our institute because of proteinuria found by an annual medical checkup. Urinalysis showed a urinary protein creatinine ratio (UPCR) of 3.0  g/gCre. Blood examination revealed that she had Graves’ disease, although she did not have any symptoms of hyperthyroidism such as weight loss, anxiety, tremor...
Source: CEN Case Reports - January 6, 2022 Category: Urology & Nephrology Source Type: research