Relationship between glomerular number in fresh kidney biopsy samples and light microscopy samples.

BACKGROUND: On-site evaluation of fresh kidney biopsy (FKB) samples at the time of biopsy is useful to verify that adequate specimens are acquired. However, some cases present poor correlation between glomerular number in FKB samples and light microscopy (LM) samples. We examined the usefulness of such on-site evaluation. METHODS: We conducted a retrospective cross-sectional observational study (n = 129) to assess the correlation between glomerular number in FKB samples and LM samples and the associated factors hindering the evaluation. RESULTS: There was a significant positive correlation between glomerular number in FKB samples and LM samples. The median ratio of glomerular number (LM samples/FKB samples) was 0.74. According to this ratio, cases were divided into three groups: reasonable estimation (65 cases), underestimation (32 cases), and overestimation (32 cases). Comparing the reasonable and underestimation groups, significant differences were detected in the extent of interstitial fibrosis and tubular atrophy (IFTA) and interstitial inflammation. Logistic regression analysis demonstrated that IFTA and interstitial inflammation were significantly associated with the underestimation. Moreover, the cortex length of FKB samples correlated with glomerular number in LM samples regardless of tubulointerstitial lesions. CONCLUSIONS: Glomerular number determined during on-site evaluation can be a reference for the actual number of glomeruli in LM samples. Since tubulointerstitial lesions make it difficult to recognize glomeruli in FKB samples, the possibility of underestimation for cases with possibly severe tubulointerstitial lesions should be considered. In such cases, evaluation of cortex length of FKB samples may substitute for evaluating glomeruli on-site.

Efficacy of plasma exchange for antineutrophil cytoplasmic antibody-associated systemic vasculitis: a systematic review and meta-analysis.

OBJECTIVE: To assess through systematic review and meta-analysis whether plasma exchange (PE) is associated with prognosis in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients. METHODS: A systematic search of PubMed, MEDLINE, Embase, and CENTRAL databases from inception to 17 June 2020 was conducted. Ongoing or unpublished trials were also searched in ClinicalTrials.gov and the World Health Organization trials portal. Randomised controlled trials (RCTs) comparing PE vs. non-PE in AAV patients (microscopic polyangiitis [MPA], granulomatosis with polyangiitis [GPA], or eosinophilic granulomatosis with polyangiitis [EGPA]) were included. The combined risk ratio (RR) was calculated by the random-effects model using the Mantel-Haenszel method. Heterogeneity was measured using the I2 statistic. Primary outcomes were mortality, clinical remission (CR), and adverse events (AEs). RESULTS: Four RCTs comparing PE vs. no PE (N = 827) and 1 RCT comparing PE vs. pulse steroid treatment (N = 137) were included. All participants were MPA or GPA patients (no EGPA patients). PE was not associated with main primary outcomes compared with no PE (mortality RR 0.93 [95% confidence interval {CI} 0.70-1.24], I2 = 0%; CR RR 1.02 [95% CI 0.91-1.15], I2 = 0%; and AE RR 1.10 [95% CI 0.73-1.68], I2 = 37%) or pulse steroid (mortality RR 0.99 [95% CI 0.71-1.37]; CR [the Birmingham Vasculitis Activity score] mean difference - 0.53 [95% CI - 1.40-0.34]; and AE RR 1.05 [95% CI 0.74-1.48]). Focusing on the early treatment phases, PE was associated with a reduction in end-stage renal disease incidence compared with both no PE (PE 1/43 vs. no PE 10/41; RR 0.14 [0.03-0.77] at 3 months) and pulse steroid (PE 11/70 vs. pulse steroid 23/67; RR 0.46 [0.24-0.86] at 3 months). CONCLUSION: We carried out a systematic review and meta-analysis targeting all AAV patients, including MPA, GPA, and EGPA. In AAV patients, performing PE was not associated with the risk of mortality, CR, and AE. No RCT exists evaluating the efficacy of PE for EGPA; hence, this is required in the future. The results may affect the development of guidelines for AAV and may indicate the direction of future clinical research on AAV. TRIAL REGISTRATION: UMIN R000045239 , PROSPERO CRD42020182566 .

Thrombotic Thrombocytopenic Purpura Treated with Rituximab Associated with Primary Sjögren's Syndrome and Primary Hypothyroidism.

A 47-year-old man was admitted to our hospital because of thrombocytopenia and consciousness disturbance. As his laboratory data showed undetectable activity of a disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13 (ADAMTS13) and the presence of ADAMTS13 inhibitor, he was diagnosed with acquired thrombotic thrombocytopenic purpura (TTP). Asymptomatic primary Sjögren's syndrome (SS) and primary hypothyroidism were incidentally diagnosed on screening. After initial plasma exchange therapy and pulse corticosteroid therapy, the patient received rituximab therapy for refractory TTP with "inhibitor boosting" and recovered. TTP secondary to primary SS is rare but can trigger refractory TTP. Treatment with rituximab, which is considered "inhibitor boosting," should be considered when re-exacerbation occurs.

Verification of serum albumin elevating effect of cell-free and concentrated ascites reinfusion therapy for ascites patients: a retrospective controlled cohort study.

Cell-free and concentrated ascites reinfusion therapy (CART) is frequently used to treat refractory ascites in Japan. However, its efficacy remains unclear. This controlled cohort study verified the serum albumin elevating effect of CART by comparisons with simple paracentesis. Ascites patients receiving CART (N = 88) or paracentesis (N = 108) at our hospital were assessed for the primary outcome of change in serum albumin level within 3 days before and after treatment. A significantly larger volume of ascites was drained in the CART group. The change in serum albumin level was +0.08 ± 0.25 g/dL in the CART group and -0.10 ± 0.30 g/dL in the paracentesis group (P < 0.001). The CART - paracentesis difference was +0.26 g/dL (95%CI +0.18 to +0.33, P < 0.001) after adjusting for potential confounders by multivariate analysis. The adjusted difference increased with drainage volume. In the CART group, serum total protein, dietary intake, and urine volume were significantly increased, while hemoglobin and body weight was significantly decreased, versus paracentesis. More frequent adverse events, particularly fever, were recorded for CART, although the period until re-drainage was significantly longer. This study is the first demonstrating that CART can significantly increase serum albumin level as compared with simple paracentesis. CART represents a useful strategy to manage patients requiring ascites drainage.

Influence of arteriovenous fistula on daily living behaviors involving the upper limbs in hemodialysis patients: a cross-sectional questionnaire study.

BACKGROUND: Arteriovenous fistulae can restrict daily living behaviors involving the upper limbs in hemodialysis patients, but no studies have investigated the detailed effects of an arteriovenous fistula on routine life activities. Accordingly, many medical caregivers are unable to explain the effects of an arteriovenous fistula on daily life, particularly during non-dialysis periods, because they cannot observe them directly. METHODS: Thirty outpatients undergoing hemodialysis at 2 facilities scored the difficulty due to an arteriovenous fistula in performing 48 living behaviors during non-dialysis and 10 behaviors during dialysis into 5 grades in a comprehensive questionnaire survey. These behaviors were selected based on an open-answer pre-questionnaire administered to the 30 patients beforehand. The scores were also compared between dominant arm and non-dominant arm arteriovenous fistula groups. RESULTS: During non-dialysis, the difficulty scores of behaviors restricted out of concern for arteriovenous fistula obstruction (wear a wristwatch, hang a bag on the arm, carry a baby or a dog in the arms, wear a short-sleeved shirt, etc.) increased. The difficulties of "wear a wristwatch" and "hang a bag on the arm" were significantly higher in the non-dominant arm arteriovenous fistula group (both P < 0.05). In contrast, scores related to motor function (write, eat or drink, scratch an itch, etc.) increased remarkably during dialysis because of connection of the arteriovenous fistula to the dialysis machine. The difficulties of "write" and "eat or drink" were significantly higher in the dominant arm arteriovenous fistula group (both P < 0.05). CONCLUSIONS: Several key daily living behaviors restricted by an arteriovenous fistula were identified in this questionnaire survey. These results will be useful for pre-operative explanation of arteriovenous fistula surgery and arm selection in end-stage renal disease patients.

Protein Concentration of Refractory Ascites in Cancer Patients is Reflected by the Presence and Severity of Peritoneal and Liver Metastasis.

Ascites total protein concentration (A-TP) affects the performance of cell-free and concentrated ascites reinfusion therapy (CART). As the factors determining A-TP remain unclear, we examined peritoneal and liver metastasis. Among 98 patients who received CART, 68 with cancer, ascites from no other apparent cause, and complete CT and A-TP data were recruited. Sixty-six patients (97%) with peritoneal and/or liver metastasis on CT were divided into the peritoneal metastasis group (PM group), peritoneal and liver metastasis group (PM + LM group), and liver metastasis group (LM group). A-TP was highest in the PM group (3.9 g/dL [3.4-4.4]), lowest in the LM group (1.0 g/dL [0.9-2.0]), and broadly dispersed in the PM + LM group (3.3 g/dL [2.0-3.8]). All differences were statistically significant. The percentage of metastasis volume occupying the liver was negatively and significantly related to A-TP in the PM + LM group. Taken together, the presence and severity of peritoneal and liver metastasis may influence A-TP.

Herpes Simplex Encephalitis Complicated by Cerebral Hemorrhage during Acyclovir Therapy.

Herpes simplex encephalitis (HSE) can be complicated by adverse events in the acute phase. We herein present the case of a 71-year-old woman with HSE complicated by cerebral hemorrhage. She presented with acute deterioration of consciousness and fever and was diagnosed with HSE based on the detection of herpes simplex virus-1 in the cerebrospinal fluid by a polymerase chain reaction. The cerebral hemorrhage developed during acyclovir therapy; however, its diagnosis was delayed for 2 days. After the conservative treatment of the cerebral hemorrhage, the patient made a near-complete recovery. Cerebral hemorrhage should be considered as an acute-phase complication of HSE.