Myeloperoxidase-antineutrophil cytoplasmic antibody-associated vasculitis with headache and kidney involvement at presentation and with arthralgia at relapse: A case report.

BACKGROUND: Patients with proteinase 3-antineutrophil cytoplasmic antibody associated vasculitis (AAV) experience different manifestations at the initial onset and relapse. However, such cases of different initial and relapse manifestations have not been reported in myeloperoxidase (MPO)-AAV patients. CASE SUMMARY: A 52-year-old woman was admitted to our hospital because of headache. Laboratory findings indicated nephrotic range proteinuria and microscopic hematuria, serum creatinine of 243 µmol/L, anti-MPO antibody titer of > 400 RU/mL, and positive perinuclearantineutrophil cytoplasmic antibody. Renal biopsy showed pauci-immune crescentic glomerulonephritis. The cerebrospinal fluid examination and brain magnetic resonance imaging did not show any abnormality. Therefore, MPO-AAV was diagnosed. Corticosteroids, plasmapheresis, and cyclophosphamide as induction therapy and mycophenolate mofetil (MMF) as maintenance therapy were administered. The patient's headache disappeared; serum creatinine returned to normal; complete remission of microscopic hematuria and proteinuria was observed. Anti-MPO antibody titer reached normal limits after immunosuppressive treatment. Twenty-five months after stopping the immunosuppressive treatment, the patient relapsed with arthralgia, without neurological or renal involvement. The patient's arthralgia improved after treatment with prednisone and MMF. CONCLUSION: We have reported a rare case of MPO-AAV who initially presented with headache and kidney involvement. However, relapse presented with only arthralgia, which was completely different from the initial manifestations. This case suggests that AAV relapse should be highly suspected in MPO-AAV patients after remission, when clinical manifestations at relapse are different from those at onset. Prednisone and MMF may provide a good choice for refractory arthralgia during relapse in MPO-AAV patients.

Mortality and associated risk factors between young and elderly maintenance haemodialysis patients: a multicentre retrospective cohort study in China.

OBJECTIVES: Mortality and associated risk factors in young and elderly haemodialysis patients with end-stage kidney disease (ESKD) have not been well examined in China. Therefore, we aimed to assess the all-cause mortality and risk factors associated with all-cause mortality between young and elderly haemodialysis patients in China. DESIGN: A population-based multicentre retrospective cohort study. SETTING: Using the Dialysis Initiation based on Fuzzy mathematics Equation study data, patients with ESKD undergoing maintenance haemodialysis from 24 centres in China from 1 January 2008 to 30 September 2015. PARTICIPANTS: 1601 enrolled patients with ESKD were categorised into young group (18-44 years old) and elderly (≥60 years old) group. OUTCOME MEASURES: The primary outcome was all-cause mortality. We estimated overall survival using a log-rank test. Cox proportional hazard regression analysis was implemented to identify risk factors and HR associated all-cause mortality. RESULTS: During a mean follow-up of 48.17±25.59 months, of the 1601 subjects, 319 (19.92%) patients death, including 64 (9.97%) in young group and 255 (26.59%) in elderly group, respectively. The cumulative survival in elderly group was lower than young group (Log Rank tests=63.31, p<0.001). Multivariate Cox proportional hazards analysis showed the cardiovascular disease (HR, 2.393; 95% CI 1.532 to 3.735; p<0.001), cerebrovascular disease (HR, 2.542; 95% CI 1.364 to 4.739; p=0.003) and serum albumin<3.5 g/dL (HR, 1.725; 95% CI 1.091 to 2.726; p=0.020) at the haemodialysis initiation were associated with increased risk of all-cause mortality in elderly groups; however, the cardiovascular disease only was associated with increased risk of all-cause mortality in young groups. CONCLUSIONS: The all-cause mortality of elderly haemodialysis patients were higher than young haemodialysis patients in China. Identified risk factors associated all-cause mortality may inform development of age-appropriate treatment, intervention strategies and improve survival prognosis of this unique population.

Coexistence of Fabry Disease and Membranous Nephropathy.

A 21-year-old man with no family history or characteristic symptoms of Fabry disease presented with proteinuria. Histological and immunofluorescent analysis of kidney tissue collected revealed stage 1 membranous nephropathy. Electron microscopy of the same tissue revealed a large number of myeloid bodies (zebra bodies) in the glomerular epithelial cytoplasm and a mild irregular thickening of basement membrane. A diagnosis of Fabry disease was supported by the low α-galactosidase A activity detected in the patient's plasma, and confirmed by the detection of a pathogenic homozygous mutation in the α-galactosidase A gene. Therefore, the final diagnosis was of coexistent Fabry disease and stage 1 membranous nephropathy. This is the first case study reporting the coexistence of Fabry disease and membranous nephropathy. Our results emphasize the importance of electron microscopy in Fabry disease diagnosis.

Chrysin suppressed inflammatory responses and the inducible nitric oxide synthase pathway after spinal cord injury in rats.

Chrysin (CH), a natural plant flavonoid, has shown a variety of beneficial effects. Our present study was conducted to evaluate the therapeutic potential of CH three days after spinal cord injury (SCI) in rats and to probe the underlying neuroprotective mechanisms. SCI was induced using the modified weight-drop method in Wistar rats. Then, they were treated with saline or CH by doses of 30 and 100 mg/kg for 26 days. Neuronal function was assessed with the Basso Beattle Bresnahan locomotor rating scale (BBB). The water content of spinal cord was determined after traumatic SCI. The NF-κB p65 unit, TNF-α, IL-1ß and IL-6 in serums, as well as the apoptotic marker, caspase-3, of spinal cord tissues were measured using commercial kits. The protein level and activity of inducible nitric oxide synthase (iNOS) were detected by western blot and a commercial kit, respectively. NO (nitric oxide) production was evaluated by the determination of nitrite concentration. The rats with SCI showed marked reductions in BBB scores, coupled with increases in the water content of spinal cord, the NF-κB p65 unit, TNF-α, IL-1ß, IL-6, iNOS, NO production and caspase-3. However, a CH supplement dramatically promoted the recovery of neuronal function and suppressed the inflammatory factors, as well as the iNOS pathway in rats with SCI. Our findings disclose that CH improved neural function after SCI in rats, which might be linked with suppressing inflammation and the iNOS pathway.