BackgroundUterine fibroids are the most common benign tumor in women, and surgical intervention is still the main fibroid treatment. Patient demands have encouraged development of less-invasive methods such as high-intensity focused ultrasound (HIFU). This study aimed to evaluate the safety and effectiveness of magnetic resonance-guided high-intensity focused ultrasound therapy using a volumetric ablation technique in the treatment of symptomatic uterine fibroids in China.MethodsOne hundred and seven patients were enrolled and treated with magnetic resonance-guided high-intensity focused ultrasound in this study. Clinical efficacy was based on the proportion of patients with fibroid shrinkage (10 % volume reduction or more compared to baseline) at 6 months post treatment as measured with magnetic resonance imaging. The quality of life and symptom outcome was assessed using the uterine fibroid symptom and quality of life questionnaire with symptom severity scoring. Safety was primarily assessed by evaluating the reported adverse events.ResultsNinety nine of the 107 treated patients had fibroid shrinkage at 6 months post treatment. Resulting in an overall 93 % (95 % confidence interval 86–97 %) treatment success rate, p value <0.001; the symptom severity scoring and health-related quality of life at 6 months was statistically different from the screening symptom severity scoring at 0.05 level. Of 366 adverse events reported, there were no study procedure-related or device-related serious adverse events were in the study.ConclusionsThis study demonstrated that the volumetric magnetic resonance-guided high-intensity focused ultrasound device is safe and technically effective and can be utilized in clinically efficient treatments of symptomatic uterine fibroids.Trial registration NCT01588899

Thrombotic microangiopathy (TMA) can sometimes manifest only histologically. Our aim was to retrospectively compare biopsy-proven adult TMA patients showing only histological (h-TMA) or both histological and clinical (c-TMA) TMA in 2006–2017. All native kidney biopsies with TMA were included. Biopsies were re-evaluated by light and electron microscopy, and immunofluorescence. Clinical characteristics, laboratory variables, and treatments were recorded from the electronic medical database. Patients were categorized into h-TMA and c-TMA and these groups were compared. In total, 30 biopsy-proven cases among 7943 kidney biopsies were identified and, of these, 15 had h-TMA and 15 c-TMA. Mean follow-up was 6.3 y, and 73.3% had secondary hemolytic uremic syndrome (HUS) and the rest were atypical HUS. Patient characteristics, treatments, and kidney, and patient survival in the groups were similar. Statistically significant differences were found in histological variables. Vascular myxoid swelling and vascular onion-skinning were almost exclusively detected in c-TMA and, thus, vascular occlusive changes indicate clinically apparent rather than merely histological TMA. In addition, regardless of clinical presentation, kidney and patient survival times were similar in the patient groups highlighting the importance of a kidney biopsy in the case of any kidney-related symptoms.

Membranoproliferative glomerulonephritis (MPGN) is subdivided into immune-complex-mediated glomerulonephritis (IC-MPGN) and C3 glomerulopathy (C3G). Classically, MPGN has a membranoproliferative-type pattern, but other morphologies have also been described depending on the time course and phase of the disease. Our aim was to explore whether the two diseases are truly different, or merely represent the same disease process. All 60 eligible adult MPGN patients diagnosed between 2006 and 2017 in the Helsinki University Hospital district, Finland, were reviewed retrospectively and asked for a follow-up outpatient visit for extensive laboratory analyses. Thirty-seven (62%) had IC-MPGN and 23 (38%) C3G (including one patient with dense deposit disease, DDD). EGFR was below normal (≤60 mL/min/1.73 m2) in 67% of the entire study population, 58% had nephrotic range proteinuria, and a significant proportion had paraproteins in their serum or urine. A classical MPGN-type pattern was seen in only 34% of the whole study population and histological features were similarly distributed. Treatments at baseline or during follow-up did not differ between the groups, nor were there significant differences observed in complement activity or component levels at the follow-up visit. The risk of end-stage kidney disease and survival probability were similar in the groups. IC-MPGN and C3G have surprisingly similar characteristics, kidney and overall survival, which suggests that the current subdivision of MPGN does not add substantial clinical value to the assessment of renal prognosis. The high proportion of paraproteins in patient sera or in urine suggests their involvement in disease development.

Maintenance patients had mean age 54 years with 56% male. 49% were studied from incident and 51% from time of a relapse. Physicians defined maintenance start with mean of 5.7 months from induction start on basis of fixed time point 40%, start of new drug for maintenance 26%, reaching full remission 26% and no specific criteria 8%. Treatment at start of maintenance as GCs 69%, Azathioprine 34%, Rituximab 21%, Mycophenolate 17%, Cyclophosphamide 16% and methotrexate 8%. After 36 months (Table 2) of maintenance, 81% were alive and in remission but 10% had major relapse requiring re-induction therapy and left follow up, 4% died (in 2/3 of cases at time of relapse). AEs and infection were common and many patients required long term GCs Conclusions: Response to remission induction therapy in AAV patients is still variable with many patients not achieving a full response over the first 12 months of therapy. Infections and treatment-related adverse events are common, especially in the first 3 months. Maintenance period is variably defined but typically around 6 months when many patients are still not fully in remission and the majority are still receiving GCs. Relapse, major and minor, are still common and patients are still faced with treatment AE burden. Unmet need in AAV remains high and there is a need for targeted therapy to improve clinical outcomes.