Intermediate cystinosis: a case report of 10-year treatment with cysteamine.

Background: Cystinosis is a lysosomal storage disorder characterized by an autosomal recessive phenotype. Intermediate cystinosis, which progresses slowly and causes renal failure, accounts for approximately 5% of all cystinosis cases. Patients with intermediate cystinosis may not exhibit the typical symptoms of cystinosis, such as Fanconi syndrome and ocular symptoms.

Identification and characterization of a novel α-haemolytic streptococci, Streptococcus parapneumoniae sp. nov., which caused bacteremia with pyelonephritis.

Objectives: We report a case of bacteremia with pyelonephritis in an adult male with an underlying disease caused by α-hemolytic streptococci. α-Hemolytic streptococci were isolated from blood, but it was challenging to identify its species. This study aimed to characterize the causative bacterium SP4011 and to elucidate its species.

Blood perfusion with polymyxin B immobilized columns in patients with COVID-19 requiring oxygen therapy.

Extracorporeal blood purification with polymyxin B immobilized fiber column direct hemoperfusion (PMX-DHP), is reported to be effective in treating COVID-19 pneumonitis with oxygen demand. This multicenter prospective study evaluated the efficacy and safety of PMX-DHP in oxygen-requiring patients with COVID-19 admitted between September 28, 2020, and March 31, 2022. The primary endpoint was the percentage of clinical improvement 15 days after treatment.

Urinary liver-type fatty acid-binding protein level as a prognostic indicator of acute kidney injury secondary to severe falciparum malaria.

Acute kidney injury (AKI) secondary to severe falciparum malaria possesses a high mortality rate; however, a prognostic marker of renal dysfunction has not yet been identified. Thus, we reported a case of a patient with AKI secondary to falciparum malaria who underwent hemodialysis and a renal biopsy due to prolonged renal dysfunction. The male patient, in his 50 s, presented to our hospital with vomiting, diarrhea, fever, and decreased level of consciousness.

A case of rapidly progressive glomerulonephritis with double-positive anti-GBM antibody and MPO-ANCA after SARS-CoV-2 vaccination and relapse during 1 year follow-up.

Although mRNA vaccines for COVID-19 are highly beneficial and are recommended for patients with kidney disease, adverse reactions in some patients after vaccination have been problematic. Various vasculitis and renal disorders have been reported after vaccination; however, a causal relationship has not yet been identified. In this report, we describe a case of rapidly progressive glomerulonephritis that developed after SARS-CoV-2 vaccination, in which both anti-glomerular basement membrane (anti-GBM) and myeloperoxidase antineutrophil cytoplasmic antibodies (MPO-ANCA) were present.

Usefulness of gallium-67 scintigraphy for evaluating the histopathological activity in interstitial nephritis.

Background: Interstitial nephritis is a common cause of renal failure. Gallium-67 scintigraphy is reportedly useful for diagnosing interstitial nephritis; however, its ability to assess disease activity remains unknown. We aimed to analyze the relationship between the renal uptake of gallium-67 and the disease activity in interstitial nephritis.

Adjuvant diagnosis of tuberculosis in hemodialysis patients using fourth generation interferon γ releasing assay.

Introduction: Dialysis patients have a 10-25 times higher risk of reactivation of tuberculosis (TB). In this study, we investigated the diagnostic ability of QuantiFERON (QFT)-plus for TB in hemodialysis patients. QFT-plus, an interferon gamma release assay, is characterized by its use of CD4 and CD8 T cell signals.

Safety of casirivimab/imdevimab administration in a SARS-CoV-2 positive maintenance dialysis patient in Japan.

Controlling excessive cytokine secretion is a crucial therapeutic strategy for managing coronavirus disease 2019 (COVID-19). Patients on dialysis are at a high risk of severe disease, given abnormal immune responses that can lead to prolonged inflammation. Moreover, patients undergoing dialysis have limited treatment options, as neither remdesivir nor baricitinib is available.

Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits without Conspicuous Mesangial Proliferation, Complicated with Squamous Cell Lung Carcinoma.

We performed a renal biopsy for nephrotic syndrome in a patient with squamous cell lung carcinoma, which can worsen the prognosis. Chemoradiation therapy was effective for the cancer and proteinuria; we thus inferred that the nephrotic syndrome had been closely associated with the carcinoma. A pathological analysis of the kidney showed monoclonality for λ chain, satisfying the diagnostic criteria of proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID); however, conspicuous mesangial proliferation was not observed.

IL-6-producing Renal Cell Carcinoma Causing Renal and Endocrine Paraneoplastic Syndromes.

An 83-year-old man with stable chronic kidney disease (CKD) exhibited a sudden increase in urinary N-acetyl-β-D-glucosaminidase and protein excretion, suggesting aggravated kidney damage. Simultaneously, he lost diabetic control, requiring up to 54 units of insulin daily. A detailed examination revealed the presence of renal cell carcinoma, which was surgically resected and confirmed to be interleukin-6-positive by immunohistochemistry.

Correction: Effectiveness of plasma lyso-Gb3 as a biomarker for selecting high-risk patients with Fabry disease from multispecialty clinics for genetic analysis.

The HTML version of this Article contained errors in Supplementary Figure S2 "Flowchart of the lyso-Gb3 screening and gene analysis in female patients", which have been detailed in the associated Correction.

Atypical cause of intractable diarrhea in a hemodialysis patient, masked by Clostridium difficile-associated diarrhea and ischemic colitis: a case report.

Background: Patients with end-stage kidney disease (ESKD) most commonly complain of gastrointestinal symptoms, such as diarrhea. Diarrhea negatively affects patient quality of life and has miscellaneous etiologies, such as Clostridium difficile-associated diarrhea (CDAD) and ischemic colitis. However, it is sometimes extremely difficult to determine the true etiology given the comorbidities and complications the patients have.

Correction: Effectiveness of plasma lyso-Gb3 as a biomarker for selecting high-risk patients with Fabry disease from multispecialty clinics for genetic analysis.

The PDF and HTML versions of the article have been updated to include the Creative Commons Attribution 4.0 International License information.

Effectiveness of plasma lyso-Gb3 as a biomarker for selecting high-risk patients with Fabry disease from multispecialty clinics for genetic analysis.

Purpose: Plasma globotriaosylsphingosine (lyso-Gb3) is a promising secondary screening biomarker for Fabry disease. Here, we examined its applicability as a primary screening biomarker for classic and late-onset Fabry disease in males and females.

Methods: Between 1 July 2014 and 31 December 2015, we screened 2,359 patients (1,324 males) referred from 168 Japanese specialty clinics (cardiology, nephrology, neurology, and pediatrics), based on clinical symptoms suggestive of Fabry disease.

Evaluating the Efficacy of Double-Filtration Plasmapheresis in Treating Five Patients With Drug-Resistant Pemphigus.

Double-filtration plasmapheresis is an effective and safe treatment for pemphigus. We retrospectively evaluated the decrease in autoantibody titer and pemphigus disease area index following double-filtration plasmapheresis in five patients with moderate to severe pemphigus, who were physically and/or serologically unresponsive to 1.0 mg/kg per day of prednisolone and other supportive drugs and ointments.

Vascular adhesion protein-1 enhances neutrophil infiltration by generation of hydrogen peroxide in renal ischemia/reperfusion injury.

Vascular adhesion protein-1 (VAP-1) is a unique molecule since it acts as an adhesion molecule as well as an ectoenzyme catalyzing oxidative deamination of primary amines and generates hydrogen peroxide in the extracellular space. While VAP-1 is implicated in various inflammatory diseases, its role in acute kidney injury is less characterized. Here we studied VAP-1 expression in the kidney and the effect of its inhibition in a rat model of renal ischemia/reperfusion injury.

Disseminated Cryptococcosis with Rapidly Growing Lung Nodules in an End-stage Renal Disease Patient.

A 73-year-old man with type 2 diabetes mellitus and end-stage renal disease was diagnosed with acute myocardial infarction. He required continuous dialysis after percutaneous coronary intervention. Subsequently, multiple nodules were discovered in both lungs for the first time, and Cryptococcus neoformans was isolated from the patient's sputum, blood, bilateral pleural fluid, and cerebrospinal fluid cultures, resulting in a diagnosis of disseminated cryptococcosis.

A novel case of renal pathergy reaction in a Behçet's disease patient complicated by IgA vasculitis.

Background: A pathergy reaction is defined as a hyperreactivity of the skin in response to minimal trauma, which is important in the diagnosis of Behçet's disease (BD). However, a pathergy reaction may not be restricted to the skin, and little is known about whether an invasive medical procedure can induce the reaction. Here we present a pathergy reaction induced by renal biopsy, an invasive procedure.

Endothelial cell injury in acute and chronic glomerular lesions in patients with IgA nephropathy.

Endothelial cell injury may contribute to the progression of various glomerular diseases. In the present study, we examined glomerular capillary injury in acute and chronic glomerular lesions in patients with Immunoglobulin A nephropathy (IgAN). We selected renal biopsy samples of IgAN (n = 200), and glomerular capillary injury in the acute and chronic glomerular lesions was assessed using immunohistochemistry for CD34 and electron microscopy.

Successful Detection of Renal Involvement in Sjögren's Syndrome Secondary to Systemic Lupus Erythematosus by Renal Biopsy.

An 80-year-old man presented with a mildly decreased renal function and increased anti-double-stranded-DNA (anti-dsDNA) antibody levels, and met the diagnostic criteria of the American College of Rheumatology for systemic lupus erythematosus (SLE). However, the incremental increase in creatinine levels and the mild proteinuria were inconsistent with lupus nephritis. We performed a renal biopsy, which revealed interstitial nephritis and minor glomerular abnormalities.