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Language：English   Publishing type：Research paper (scientific journal)   Publisher：The Japanese Society of Internal Medicine  

<p>Myoglobin is a well-known cause of acute kidney injury (AKI) due to rhabdomyolysis. However, whether or not removing serum myoglobin by on-line hemodiafiltration (OHDF) improves the kidney function remains unclear. We herein report a patient with a history of methamphetamine abuse who developed AKI due to rhabdomyolysis. A urinalysis and blood collection results obtained before and after OHDF demonstrated that OHDF improved the kidney function by removing a large amount of serum myoglobin rather than via urinary excretion. In conclusion, OHDF may prevent AKI progression effectively when the urine volume is insufficient. </p>

DOI： 10.2169/internalmedicine.1107-22

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Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)  

Fibrillary glomerulonephritis (FGN), a rare disease is pathologically characterized by glomerular fibril accumulation ranging from 12 to 24 nm in diameter with negative Congo red staining. Recently, the identification of DnaJ homolog subfamily B member 9 (DNAJB9) as a highly sensitive and specific marker for FGN has revolutionized diagnosis of this disease. However, few recent studies have reported DNAJB9-negative glomerulonephritis with fibrillar deposits. As such, it remains unclear whether DNAJB9-negative cases can be considered equivalent to FGN. Here, we report the case of a 70-year-old woman who developed renal impairment and nephrotic-range proteinuria. Renal biopsy and pathological examination revealed focal glomerulonephritis with fibrocellular crescents. Immunofluorescence microscopy showed IgA-dominant deposition of polytypic IgG in the glomerulus. Electron microscopy revealed hump-like subepithelial electron dense deposits with fibrils of 15-25 nm in diameter. These findings were consistent with FGN; thus, Congo red and direct fast scarlet (DFS) staining, and immunohistochemistry for DNAJB9 were performed. In addition to negative Congo red/DFS/DNAJB9 staining, laser microdissection (LMD) and liquid chromatography-tandem mass spectrometry (LC-MS/MS) resulted negative for DNAJB9, which is a highly sensitive and specific marker for FGN. The patient's renal function further declined, prompting administration of rituximab weekly for 2 weeks, similar to the treatment for FGN. This is a unique case of IgA-dominant glomerulonephritis with DNAJB9-negative fibrillar polytypic immunoglobulin deposits in the subepithelium, unlike previous DNAJB9-negative cases. Thus, DNAJB9-negative cases diagnosed based on accurate electron microscopic evaluation must be gathered, and LMD and LC-MS/MS must be used to analyze the organized fibrillar deposits to reveal the disease entity.

DOI： 10.1007/s13730-022-00759-2

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Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：The Japanese Society of Internal Medicine  

<p>Fabry disease is an X-linked lysosomal storage disorder due to mutations in the alpha-galactosidase A gene, which leads to the accumulation of globotriaosylceramide in various organs. In Fabry disease with end-stage renal disease (ESRD), cerebrovascular events are lethal, even with enzyme replacement therapy (ERT). However, the utility of biomarkers to evaluate the ERT response is unclear. We herein report a case of recurrent cerebrovascular complications under ERT in a Fabry disease patient, progressing to ESRD on peritoneal dialysis. Further studies are warranted, but Fabry disease patients with ESRD receiving ERT might need careful long-term follow-up in cases with cerebrovascular manifestations. </p>

DOI： 10.2169/internalmedicine.0185-22

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Authorship：Lead author,　Corresponding author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：Frontiers Media SA  

Background

When facing unprecedented emergencies such as the coronavirus disease 2019 (COVID-19) pandemic, a predictive artificial intelligence (AI) model with real-time customized designs can be helpful for clinical decision-making support in constantly changing environments. We created models and compared the performance of AI in collaboration with a clinician and that of AI alone to predict the need for supplemental oxygen based on local, non-image data of patients with COVID-19.

Materials and methods

We enrolled 30 patients with COVID-19 who were aged &amp;gt;60 years on admission and not treated with oxygen therapy between December 1, 2020 and January 4, 2021 in this 50-bed, single-center retrospective cohort study. The outcome was requirement for oxygen after admission.

Results

The model performance to predict the need for oxygen by AI in collaboration with a clinician was better than that by AI alone. Sodium chloride difference &amp;gt;33.5 emerged as a novel indicator to predict the need for oxygen in patients with COVID-19. To prevent severe COVID-19 in older patients, dehydration compensation may be considered in pre-hospitalization care.

Conclusion

In clinical practice, our approach enables the building of a better predictive model with prompt clinician feedback even in new scenarios. These can be applied not only to current and future pandemic situations but also to other diseases within the healthcare system.

DOI： 10.3389/fmed.2022.1042067

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Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)  

INTRODUCTION: In dialysis patients, cardiovascular disease (CVD) and infectious disease contribute to poor clinical outcomes. We investigated if a higher monocyte/lymphocyte ratio (MLR) is associated with an increased risk of CVD events and infectious disease hospitalizations in incident dialysis patients. METHODS: In an ongoing observational prospective cohort study, 132 Japanese dialysis patients (age 58.7 ± 11.7 years; 70% men) starting dialysis therapy were enrolled and followed up for a median of 48.7 months. Laboratory biomarkers, including white blood cell count and its differential count, were determined at baseline. Event-free time and relative risks (RRs) were calculated using the Kaplan-Meier curves and Cox models, respectively. RESULTS: When divided into 2 groups according to median MLR (0.35 [range, 0.27-0.46]), the periods without CVD events were significantly shorter in the high MLR group than in the low MLR group (log-rank test = 5.60, p = 0.018). The RR of CVD events, after adjusting for age, sex, and diabetes, was 2.43 (1.22-4.84) in the high MLR group compared to the low MLR group. The periods without infections requiring hospitalization were also shorter (log-rank test = 4.16, p = 0.041). The RR of infections requiring hospitalization was 1.98 (1.02-3.83) after the same adjustments. The number of CVD events was higher in the high MLR group (18.6 events per 100 person-years at risk [pyr]) than the low MLR group (11.1 events per 100 pyr). The duration of infectious disease hospitalization was longer in the high MLR group (6.3 days per pyr) than in the low MLR group (2.8 days per pyr). CONCLUSION: A higher MLR is associated with increased risks of both CVD events and infectious disease hospitalization in dialysis patients.

DOI： 10.1159/000519289

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Presentation type：Oral presentation (invited, special)  

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