A 72-year-old woman presented with nephrotic proteinuria and moderate haematuria. Renal pathology was compatible with immunotactoid glomerulopathy (ITG), for which there is no consensus for appropriate therapy. We, therefore, postponed immunosuppressive therapy. After 4 years, the patient’s renal function started to decline and renal pathology was re-evaluated, revealing a pathological change from mesangial proliferative glomerulonephritis to endocapillary proliferative glomerulonephritis. Treatment with oral prednisolone (30 mg/day) was initiated. Within 5 weeks, complete remission of proteinuria was obtained (proteinuria 6.02 g/gCr to 0.12 g/gCr), and the patient’s renal function stabilised. Generally, responsiveness to immunosuppressive therapy is poor in patients with ITG, and the present case represented a very rare clinical course. Some previous cases have indicated susceptibility to the therapy, regardless of the severity of renal damage. As a possible distinct entity that determines susceptibility to immunosuppressive therapy, we suggest the presence of a latent lymphoproliferative disease with no significant haematological symptoms.
Heparin is commonly used to prevent clotting; however, severe hypersensitivity reactions during vascular access (VA) placement are rarely but occasionally reported. A 49-year-old man experienced a heparin-induced hypersensitivity reaction during VA placement. Severe side effects may occur even while placing the VA; therefore, we reconsidered the routine use of heparin, as the side effects are unpredictable. Physicians should be aware of the risk of heparin-induced hypersensitivity and be ready to effectively manage it during VA placement.
Objective: Peritoneal function during peritoneal dialysis (PD) declines over time due to peritoneal inflammation; however, the immunological mechanism has not been fully clarified. Here, we examined changes in each cellular fraction in the peritoneal dialysis effluent by flow cytometry and their relationship to peritoneal damage. Patients and Methods: We enrolled 23 patients who began PD between 2006 and 2017 and had available datasets of the peritoneal equilibration test and flow cytometric analysis for at least three consecutive visits, with an interval of six months from six months after introducing PD. The levels and changes in each cellular fraction, dialysate/plasma (D/P) creatinine ratio, and the forward scatter (FSC) ratio of mesothelial cells to lymphocytes were compared using a simple linear regression analysis. Results: Among the examined variables, only the fraction of CD8 + T CM cells during the first observation was significantly correlated with the change rate in the D/P creatinine ratio (β=1.47, P=0.001, adjusted R 2 = 0.379). The CD8 + naïve T and CD8 + T CM cell fractions were negatively correlated with the change rate of the D/P creatinine ratio (naïve T cells: β=−0.058, P=0.022, adjusted R 2 = 0.188; T CM cells: β=−0.096, P=0.046, adjusted R 2 =0.137). In addition, the change rates of the D/P creatinine ratio tended to be higher, though not significantly (one way ANOVA; P=0.080), in accordance with the increase in the change rate of the CD8 + effector memory T cells (T EM ).
Conclusion:The CD8 + naïve T and T CM cells may transition into T EM cells by repeated exposure to the dialysate over time. The T EM cells residing in the peritoneum may play a significant role in the progression of peritoneal damage.
Objective: Intersectin 2 (ITSN2) is reported to cause hereditary nephrotic syndrome, but the number of cases remains quite small. We observed a case of progressive renal dysfunction and family history for end-stage kidney disease with a known single heterozygous ITSN2 variant. This study aimed to reveal the novel pathological significance of altered ITSN2 expression via a detailed examination. Patient and Methods: A 52-year-old Japanese woman with mild proteinuria and hematuria visited our center. The patient did not opt for a detailed examination but was instead followed up with conservative treatment consisting of low-dose angiotensin receptor blockers. Serum Cr worsened from 1.15 to 1.79 mg/dL after 7 years when precise diagnosis was performed by renal biopsy and genetic testing. Results: Kidney biopsy showed a thin basement membrane (TBM) and global glomerulosclerosis in 37.5% (6 out of 16) glomeruli examined. Comprehensive gene panel testing of 121 genes revealed a known ITSN2 variant, assumed to be involved in pathogenesis. No variants in the Alport syndrome genes, which are typically responsible for TBM, were detected. Conclusion: A possible novel phenotype of the heterozygous ITSN2 variant was identified as a cause of hereditary renal failure. Further investigation of similar cases is required for a better understanding.
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