Publications by authors named "Shigekazu Kurihara"

Emphysematous polycystic renal infection (EPRI) has a poor prognosis with conservative management, and early surgical nephrectomy has been recommended. However, percutaneous cyst drainage may be a possible treatment option. We experienced 6 patients with autosomal dominant polycystic kidney disease (ADPKD) presenting with EPRI.

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We herein report a 69-year-old man with acute kidney injury who required dialysis after receiving a combination of three drug-induced lymphocyte stimulation test-positive drugs. A kidney biopsy showed tubulointerstitial nephritis with severe eosinophilic infiltration and numerous granuloma formations. Acute eosinophilic granulomatous tubulointerstitial nephritis was diagnosed.

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A kidney biopsy was performed in a 64-year-old woman with type 2 diabetes mellitus and less than 1 g of proteinuria who rapidly progressed to end-stage renal failure after approximately 2 years of treatment with two dipeptidyl peptidase 4 (DPP-4) inhibitors for type 2 diabetes mellitus. The biopsy revealed not only a coincidental diagnosis of renal cell carcinoma, which was not evident on pre-biopsy computed tomography, but also severe thrombotic microangiopathy (TMA)-like glomerular endothelial cell damage in the noncancerous areas. These results suggest that DPP4 inhibitors may have been involved in two kidney diseases.

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  • After a relapse two years later, he was switched to baricitinib, achieving remission again before RA recurred and he discovered a tumor in his kidney at age 72.
  • Following surgical removal of the tumor, which was identified as renal cell carcinoma (RCC), the patient reported joint pain relief but ultimately died from systemic metastases about 10 weeks later, with the RA and RCC linked as a paraneoplastic syndrome.
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  • A 53-year-old woman with atypical hemolytic uremic syndrome (aHUS) caused by a complement factor H (CFH) mutation underwent multiple kidney biopsies as part of her treatment plan.
  • The initial biopsy showed significant kidney damage, including blood vessel problems and swelling, while subsequent biopsies indicated improvement after treatment with the anti-C5 monoclonal antibody eculizumab.
  • This case highlights the effectiveness of eculizumab in treating aHUS and provides valuable insights into the disease's kidney pathology over time, marking it as the first report to document these changes before and after treatment.
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Introduction: This study aimed to analyze the clinical course of TAFRO syndrome in patients through extended follow-up, focusing on recurrent cases and long-term remission.

Methods: This was a retrospective case series study. We assessed the clinical course of patients diagnosed with TAFRO syndrome between January 2012 and September 2022 at Toranomon Hospital or Toranomon Hospital Kajigaya, excluding those patients who died during the initial hospitalization.

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Objective: To evaluate the efficacy and safety of first-line biological disease-modifying antirheumatic drugs (bDMARDs) in patients with rheumatoid arthritis (RA) with chronic kidney disease (CKD), including those undergoing haemodialysis (HD).

Methods: This retrospective cohort study included 425 patients with RA prescribed their first bDMARDs at two hospitals from 2004 to 2021. Patients were categorised by kidney function and bDMARD modality (TNFα inhibitors (TNFαis), interleukin-6 inhibitors (IL-6is), cytotoxic T-lymphocyte antigen-4 immunoglobulin (CTLA4-Ig)).

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We investigated the pathogenesis of a perihilar variant of focal segmental glomerulosclerosis detected by kidney biopsy in a 16-year-old male. The disease was refractory to steroid therapy, and at the second kidney biopsy, abnormal mitochondrial proliferation was newly observed in the podocytes. The patient also developed late-onset hearing loss and had a family history of diabetes, and genetic testing confirmed the mitochondrial DNA mutation 3243A>G (48%).

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A 76-year-old woman was admitted with progressive renal function decline. A kidney biopsy was performed because of myeloperoxidase anti-neutrophil cytoplasmic antibody (ANCA; 333 IU/mL), proteinuria (1.21 g/d), and urinary erythrocyte sediment (10-19/high-power field).

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  • 90% of the 49 enrolled patients carried pathogenic or suspected pathogenic variants in known polycystic disease genes.
  • The research highlighted differences in organ volumes between patients with autosomal dominant polycystic kidney disease (ADPKD) and those with isolated PLD, indicating that some patients can experience severe liver disease without significant kidney involvement.
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  • A 28-year-old woman with untreated high blood pressure for 5 years was hospitalized due to breathing issues, coughing up blood, and eye problems.
  • Imaging revealed lung plaques and she experienced kidney damage, low platelets, and anemia.
  • After 8 weeks of antihypertensive treatment, her kidney function improved, leading to a diagnosis of malignant nephrosclerosis linked to a hypertensive crisis.
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Introduction: Data on longitudinal trajectory of kidney function decline and fluctuation in albuminuria leading to end-stage kidney disease (ESKD) is sparse in patients with type 2 diabetes.

Methods: Using data from an observational study of patients with type 2 diabetes and biopsy-confirmed diabetic kidney disease (DKD), generalized additive mixed models (GAMMs) were performed to quantify patterns of longitudinal trajectory of estimated glomerular filtration rate (eGFR) decline to ESKD associated with repeated measures of urine albumin-to-creatinine ratio (ACR).

Results: Over a median follow-up period of 3.

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We present a 51-year-old male patient with a history of Child-Pugh Grade B alcoholic liver cirrhosis (ALC) who developed renal impairment (serum creatinine of 2.00 mg/dL) and nephrotic syndrome (a urinary protein level of 4.35 g/gCr).

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  • Acute lymphoblastic leukemia (ALL) is a fast-growing blood cancer with potential for extramedullary relapse (EMR), most commonly in the central nervous system, but can also occur in other organs like the kidneys.
  • A 49-year-old man with Philadelphia chromosome-negative ALL developed a perirenal mass after a second umbilical cord blood transplant, which was diagnosed as EMR through a successful percutaneous biopsy performed while the patient was sitting.
  • This case highlights a novel biopsy technique for renal lesions and emphasizes the importance of collaboration between hematologists and nephrologists in identifying EMR in patients with ALL.
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A 49-year-old Japanese woman was admitted to our hospital with weight loss of 15 kg, nephrotic-range proteinuria (4.5 g/g.Cre), and hematuria over a 6-month period.

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  • * New treatments for renal anemia, specifically HIF-PHIs like roxadustat and daprodustat, have been shown to cause excess serum copper levels in some patients, which normalized upon switching to darbepoetin alfa.
  • * This suggests that HIF-PHIs might affect copper levels by influencing iron metabolism, highlighting the need to investigate the connection between HIF-PHIs and serum copper excess to prevent potential health issues.
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Perioperative nutritional therapy requires the consideration of metabolic changes, and it is desirable to reduce stress aiming at early metabolic normalization. Glutathione (GSH) is a tripeptide composed of glutamic acid, cysteine, and glycine. It is one of the strongest antioxidants in the body and important for adjusting immune function.

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Background: Chemotherapy is frequently used in cancer treatment; however, it may cause adverse events, which must be managed. Reactive oxygen species (ROS) have been reported to be involved in the induction of intestinal mucositis and diarrhea, which are common side effects of treatment with fluoropyrimidine 5-fluorouracil (5-FU). Our previous studies have shown that oral administration of cystine and theanine (CT) increases glutathione (GSH) production in vivo.

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Although both cinacalcet and etelcalcetide are calcimimetics that directly inhibit parathyroid hormone (PTH) secretion by activating the calcium (Ca)-sensing receptor (CaSR), their binding sites are different. We report a first case of a hemodialysis (HD) patient with secondary hyperparathyroidism (SHPT), in whom cinacalcet, but not etelcalcetide, could reduce serum intact PTH (i-PTH) levels. A HD patient received total parathyroidectomy (PTx) with auto-transplantation 16 years earlier.

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Mixed connective tissue disease (MCTD) causes multiple organ dysfunctions, such as joint swelling, pulmonary fibrosis and hypertension, and serositis, but hepatopancreatic complications are rare. Here, we report a case of young man who exhibited acute severe liver dysfunction. He also had impaired cardiac function: both ventriculi were hypokinetic, but pulmonary hypertension and pericarditis were not observed.

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Background: Renal tubular acidosis and tubulointerstitial nephritis constitute the primary renal complications associated with Sjögren's syndrome (SjS), and glomerulonephritis and nephrotic syndrome are rare.

Case Presentation: A 79-year-old Japanese woman presented with bilateral leg edema and weight gain and was diagnosed with nephrotic syndrome. In addition, she reported a 5-year history of dryness of mouth and was diagnosed with SjS.

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Background And Aim: Oral administration of cystine and theanine (CT) may modulate antioxidant glutathione (GSH) metabolism, thereby improving outcomes after gut ischemia reperfusion.

Methods: Experiment 1: Institute of Cancer Research mice (n = 35) were assigned to a Vehicle (n = 11), a CT140 (n = 14), or a CT280 (n = 10) group. The CT140 and 280 groups were given CT at respective dosages of 140 and 280 mg/kg (cystine: theanine = 5: 2) once daily via gavage for 5 days.

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