A 37-year-old woman with chronic kidney disease stage (CKD) G4 with membranoproliferative glomerulonephritis was hospitalized for nephrotic syndrome and hypertension due to superimposed preeclampsia at 27 weeks into her third pregnancy. Proteinuria did not worsen significantly after pulse steroid therapy. Delivery was induced at 30 weeks' gestation due to the maternal renal function and fetal growth. No obvious fetal complications other than preterm delivery were observed. In this case, we successfully managed a high-risk patient with membranoproliferative glomerulonephritis complicated by advanced CKD, nephrotic syndrome, and hypertension, which are independent risk factors for pregnancy complications.

Gastroduodenal stenting (GDS) is a less invasive alternative to gastrojejunostomy for the management of malignant gastric outlet obstruction (mGOO). GDS is a minimally invasive treatment with good technical and clinical success, and severe complications that require surgical intervention are rare. Stent fracture is an uncommon complication associated with GDS; however, migration of the fractured distal segment can result in small bowel obstruction. Adverse effects of stent fractures in patients with mGOO have rarely been reported. We herein report two surgical cases of small bowel obstruction caused by the migration of fractured metal stent in patients with mGOO.

Pneumonia caused by Legionella longbeachae, transmitted through contaminated soil, is very rare in Japan. A 70-year-old man with severe respiratory failure was admitted to our hospital and underwent multidisciplinary procedures. Although a urinary antigen test was negative for Legionella, he was clinically diagnosed with legionellosis and administered levofloxacin. His condition subsequently improved. Thereafter, sputum culture detected L. longbeachae. Because the DNA of L. longbeachae was detected in the gardening soil, it is suspected source of infection. Therefore, it is important to suspect legionellosis based on clinical information, even if a urine antigen test is negative.

A 29-year-old woman who had been diagnosed with acute myeloid leukemia presented with persistent grade-4 febrile neutropenia (FN) after initial chemotherapy with idarubicin and cytarabine. Despite intensive treatment, FN persisted. Subsequently, her nose became reddish and swollen, obstructing the nasal cavities. Computed tomography revealed swelling of the nostrils and an irregular tracheal surface. Debridement of the nasal lesion and a bronchoscopic biopsy of the tracheal lesion were also performed. A histopathological examination revealed pseudocarcinomatous hyperplasia (PCH) of the nose and necrotizing tracheitis. Both nasal PCH and necrotizing tracheitis ameliorated when the patient recovered from leukocytopenia.

Upadacitinib, a recently approved Janus kinase (JAK) inhibitor specific for JAK1, may be a promising candidate in patients with ulcerative colitis (UC) who present no response or intolerance to first-line JAK inhibitors. We assessed the therapeutic impact of upadacitinib on six UC patients who demonstrated an inadequate response or intolerance to tofacitinib or filgotinib. After 2 months of treatment, 5 patients (83.3%) achieved clinical remission, and all patients experienced decreased levels of CRP. One patient had coronavirus disease 2019 pneumonia and showed a mild increase in transaminase levels. This case series highlights the potential utility of a rotation strategy among JAK inhibitors.

A 79-year-old woman presented with difficulty walking and disturbance of consciousness. Magnetic resonance imaging revealed diffuse white matter lesions and abnormal signals along the surface of the brain and sulci. A brain biopsy revealed granulomatous vasculitis with eosinophil infiltration. There was no peripheral blood eosinophilia or evidence of angiitis in other organs, and primary angiitis of the central nervous system (PACNS) with pathological findings of eosinophilic granulomatosis with polyangiitis (EGPA) was diagnosed. Steroids and other immunosuppressant therapies showed only limited effects. PACNS with pathological findings of EGPA is extremely rare, and a prompt brain biopsy is necessary for a diagnosis.

Objective Testing for the Janus activating kinase 2 (JAK2) V617F mutation is important for diagnosing and treating myeloproliferative neoplasms (MPNs). Recently, urine cell-free DNA (ucfDNA) was reported to be useful for detecting tumor-specific gene mutations in several solid tumors. However, its utility in detecting such mutations in hematological malignancies has not yet been assessed. In this study, we assessed whether or not the JAK2 V617F mutation could be detected in ucfDNA and whether or not its positivity rate in ucfDNA was associated with the JAK2 V617F allele ratio of peripheral blood cells in patients with MPN.

Methods The JAK2 V617F allele ratio of genomic DNA from peripheral blood cells was determined using quantitative polymerase chain reaction (qPCR) or droplet digital PCR (ddPCR). ucfDNA was subjected to ddPCR. The correlation between the JAK2 V617F mutation positivity rates of blood-derived DNA and those of ucfDNA was assessed.

Materials Twelve patients with polycythemia vera and 12 patients with essential thrombocythemia were enrolled. Ethylenediaminetetraacetic acid-treated peripheral blood (100 mL) and 15-30 mL of fresh urine were used.

Results The JAK2 V617F mutation was detected in the ucfDNA from all 20 JAK2 V617F mutation-positive patients. In addition, the JAK2 V617F mutation positivity rate of ucfDNA was correlated with the JAK2 V617Fｓ allele ratio of blood-derived DNA, including in both estimated glomerular filtration rate (eGFR) groups (patients with an eGFR ≥50 or <50 mL/min/1.73 m²).

Conclusion Our results indicate that ucfDNA is a valuable tool for diagnosing and monitoring MPN. Given these findings, other disease-specific gene mutations in hematological malignancies may also be detectable in ucfDNA.

Spontaneous abdominal wall hematoma is a relatively uncommon condition triggered by various factors, including anticoagulation therapy and trauma. However, reports of unprovoked cases without anticoagulants that recur shortly after treatment are limited. We herein report an elderly woman who had been prescribed corticosteroids and experienced early recurrence of hematoma following treatment, with no discernible triggers. This case highlights the possibility that patients with underlying predisposing factors may experience early hematoma recurrence at the same site, even in the absence of apparent triggers. Clinicians should monitor these patients to promptly identify and address potential recurrences.

Among nontuberculous mycobacterial pulmonary diseases (NTM-PDs), Mycobacterium abscessus species pulmonary disease (MABS-PD) is one of the most severe and intractable infections. We herein report a 45-year-old woman with advanced lymphangioleiomyomatosis (LAM) who developed MABS-PD while undergoing sirolimus therapy. MABS-PD was immediately controlled using antibiotic therapy, although the patient's lung transplant registration was significantly delayed. To our knowledge, this is the first case report on the development of NTM-PD in a patient with LAM before lung transplantation. This case suggests that the early diagnosis and optimal treatment of NTM-PD are crucial in patients with advanced LAM.

A 52-year-old man who had been using a proton pump inhibitor (PPI) and a potassium-competitive acid blocker (P-CAB) for 14 years underwent esophagogastroduodenoscopy and was found to have three neuroendocrine tumors (NETs) in the gastric body. Following detailed examinations, parietal cell dysfunction was excluded, and the NETs did not meet the criteria for the Rindi classification types I-III. The lesions were ultimately considered to be associated with the long-term use of the PPI and P-CAB. We performed endoscopic submucosal dissection of the lesions, with no recurrence or new lesions noted after discontinuation of the PPI and P-CAB.

A 65-year-old woman with a history of peritoneal dialysis undergoing hemodialysis at our hospital presented with a fever after experiencing gastroenteritis symptoms. She had an implanted peritoneal dialysis catheter for draining chylous ascites. After commencing empirical treatment with meropenem, peritoneal effluent samples revealed an increased white blood cell count, and peritonitis was diagnosed. Enterococcus gallinarum was detected in blood and effluent cultures. Meropenem was changed to vancomycin based on susceptibility testing but subsequently restarted and thereafter changed to ampicillin following exacerbation of peritonitis. Finally, catheter removal led to complete recovery. E. gallinarum is vancomycin-resistant and a rare cause of peritonitis.

A 73-year-old man was admitted with Cheyne-Stokes respiration and progressive disturbance of consciousness over the course of a month. Cranial magnetic resonance imaging revealed signs suggestive of angioedema in the posterior limb of the internal capsule, external capsule, and subcortical white matter. Acute lead encephalopathy was diagnosed based on abnormally high plasma lead levels. After methylprednisolone pulse therapy followed by chelation therapy, the patient fully recovered. In this case, the angioedema with a distinctive magnetic resonance imaging appearance was attributed to the cytotoxic effects of lead on the nervous system, which responded well to methylprednisolone pulse therapy.

Objective Edoxaban is an anticoagulant used for venous thromboembolism (VTE) treatment and requires pretreatment with parenteral anticoagulants. However, pretreatment is not always performed in the clinical setting. In this study, we investigated the safety and effectiveness of edoxaban treatment in patients with VTE with or without pretreatment.

Methods We retrospectively enrolled 364 patients who received edoxaban for VTE treatment between September 2014 and March 2020 and investigated patient demographics, VTE recurrence, and major bleeding as clinical outcomes in patients with or without pretreatment. Furthermore, the factors contributing to pretreatment decisions were assessed.

Results Patients without pretreatment (n=208) had more active cancer cases and fewer pulmonary embolism complications than those with pretreatment (n=156). Lower levels of hemoglobin and higher levels of white blood cell counts, C-reactive protein, and D-dimer at the diagnosis were found in patients who received pretreatment than in those without pretreatment. No symptomatic VTE recurrence was observed. After propensity score matching, the cumulative incidence of major bleeding was not significantly higher in patients with pretreatment than in those without it (log-rank test, p=0.136). The incidence of deteriorated VTE on imaging did not significantly differ between patients with and without pretreatment, even after propensity matching (log-rank test, p=0.414).

Conclusion In a real-world clinical setting, where physicians determined the use of parenteral anticoagulant lead-in according to their experience, patient demographics, and VTE characteristics, no significant differences were found regarding safety and effectiveness in edoxaban-treated VTE patients with or without pretreatment with parenteral anticoagulants.

Nonbacterial thrombotic endocarditis (NBTE) is a condition that results in the development of vegetation on cardiac valves that are devoid of inflammation and bacteria. We herein report a 60-year-old man who transferred to our hospital because of a systemic embolism and heart failure. A mass in the right atrium and vegetation on the mitral valve were observed. He was first diagnosed with infectious endocarditis according to the Duke criteria. During treatment, however, the patient was diagnosed with antiphospholipid syndrome and cancer. After four weeks of antibacterial therapy, the patient underwent open chest surgery, and the postoperative histological diagnosis was NBTE.

In recent years, lesbian, gay, bisexual, and transgender (LGBT) populations have been gaining acceptance in society. However, very few cases of malignancy in the LGBT population have been reported thus far. We herein report a transgender woman receiving estrogen supplementation who developed primary mediastinal large B-cell lymphoma (PMBCL) and was treated with dose-adjusted EPOCH-rituximab (DA-EPOCH-R) therapy. The patient achieved complete remission after the sixth course of DA-EPOCH-R therapy. To help this LGBT patient continue receiving chemotherapy smoothly on admission, adjusting the hospital environment, such as the allocation of rooms, was essential.

Congenital hemoglobin disorders typically present as hemolytic anemia, but there are also numerous reports of thrombotic complications in these diseases, suggesting an associated hypercoagulative state. In hemoglobin Köln, the most common type of unstable hemoglobinopathy worldwide, there have not been many reports of such thrombotic phenomena. We herein describe the case of a patient with hemoglobin Köln demonstrating acute cerebral infarction. His father, who also had hemolytic anemia, presumably hemoglobin Köln, had a history of cerebral infarction. This case suggests that hemoglobin Köln, among other congenital hemoglobin disorders, may be a precipitating factor of thrombotic events.

A 72-year-old man presented with bilateral ground-glass opacities in the lower lung fields on chest radiography. Computed chest tomography showed ground-glass opacities and micronodules in both lower lungs. A video-assisted thoracoscopic biopsy of the right lower lung showed homogeneous thickening of the alveolar septa with fibrosis and inflammatory cell infiltration consistent with fibrotic non-specific interstitial pneumonia (fNSIP). Cicatricial organizing pneumonia and intraluminal pulmonary ossification containing bone marrow that was considered to represent dendriform pulmonary ossification. Idiopathic fNSIP was diagnosed. The patient remains stable under antifibrotic treatment.

Prothrombin time (PT) is a key parameter for assessing the severity of liver disease. We present the case of a 37-year-old woman with severe acute liver injury due to autoimmune hepatitis. Although prednisolone drastically improved her hepatocyte function, her PT did not recover to the reference range. A review of her medical records revealed that the patient had normal transaminase levels and prolonged PT 2 years previously. Further examinations of her coagulopathy revealed that she had low factor VII activity, suggesting a diagnosis of factor VII deficiency. Our experience suggests that altered coagulopathy should be considered in cases of liver injury with an extraordinary PT.

Metastatic colorectal neuroendocrine carcinoma (NEC) is often treated using a chemotherapy protocol for small-cell lung cancer; however, the prognosis is extremely poor. A 55-year-old woman with BRAF V600E-mutated transverse colon NEC and liver metastases underwent colectomy followed by FOLFOXIRI plus bevacizumab. Consequently, the liver metastases markedly shrank. Owing to later worsening of the liver metastases, she received encorafenib and binimetinib plus cetuximab. Despite discontinuing binimetinib due to myalgia, she had a long-term response with a progression-free survival of 14 months and an overall survival of more than 27 months. A chemotherapy protocol for BRAF-mutated metastatic colorectal cancer may be a treatment option for BRAF V600E-mutated colorectal NEC.

We herein report two cases of Guillain-Barré syndrome (GBS) mimicking lumbar spinal stenosis (LSS). Both cases were initially diagnosed as LSS based on prominent segmental weakness in the L5 and S1 myotomes and coexisting LSS on magnetic resonance imaging. However, neurological and electrophysiological examinations revealed abnormalities that extended to the upper extremities, although slight, prompting us to suspect GBS. Subsequently, serum antiganglioside antibodies and remarkable responsiveness to intravenous immunoglobulin therapy confirmed GBS. We suspect that the focal blood-nerve barrier disruption due to preexisting LSS might have contributed to the segmental weakness in this atypical GBS case.

This report describes a case of shock symptoms in a 72-year-old woman with epilepsy who had been in a state of polypharmacy, taking multiple antipsychotic drugs. After receiving a normal dose of periciazine, she exhibited impaired consciousness, hypothermia, and hypotension and was admitted to hospital. Despite poor response to vasopressors, conservative treatment led to gradual improvement. Subsequent pharmacokinetic analysis showed non-toxic blood concentrations of periciazine, suggesting that even small doses of phenothiazines could result in toxic symptoms. This case highlights the importance of monitoring for adverse reactions when prescribing multiple antipsychotic drugs, particularly in older polypharmacy patients.

A 51-year-old Thai woman diagnosed with β-thalassemia underwent regular blood transfusion and iron-chelating therapy. However, after voluntarily discontinuing treatment, the patient developed progressive dyspnea and was diagnosed with pulmonary hypertension following right heart catheterization. Despite resuming blood transfusions, her condition did not improve. Because the patient had a history of multiple organ failure, curative treatment for β-thalassemia was not feasible, and macitentan was administered. Despite experiencing hypotension as an adverse event, her condition remained stable during macitentan treatment. Thus, macitentan may be well tolerated in patients with pulmonary hypertension caused by β-thalassemia with multiple organ dysfunction.

Cold agglutinin disease is a subtype of autoimmune hemolytic anemia that occurs via the activation of specific anti-red blood cell antibodies (agglutinins) at low temperatures. Autoimmune hemolytic anemia has been reported to cause interstitial pneumonia; however, the underlying mechanism remains unclear. We herein report a 46-year-old man diagnosed with cold agglutinin disease complicated by pulmonary thrombosis and organizing pneumonia. Treatment with prednisolone improved the course of cold agglutinin disease and organizing pneumonia in a similar manner. To our knowledge, this is the first report of cold agglutinin associated with organizing pneumonia, suggesting a potential link between the two.

We herein report a case of diffuse large B-cell lymphoma (DLBCL) involving multiple renal and bone infiltrations presenting with giant cell arteritis-like (GCA)-like manifestations. One month prior, the present patient had left-sided temporal headache, jaw claudication, and renal failure. The patient was diagnosed with DLBCL based on a renal biopsy. After rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) plus intrathecal methotrexate/cytarabine/prednisone and rituximab, high-dose methotrexate, and cytarabine (R-MA) chemotherapy, the patient's clinical manifestations improved, and complete remission was achieved. DLBCL rarely but occasionally presents with GCA-like manifestations or multiple renal and bone infiltrations, highlighting the need for prompt and aggressive combination chemotherapy.

Vanishing bile duct syndrome (VBDS) is characterized by bile duct degeneration and necrosis, which result in bile duct loss and bile stasis. A 70-year-old man had malaise after receiving celecoxib. Laboratory tests revealed elevated hepatobiliary enzymes. His condition worsened without response to medical treatment, and he was transferred to our hospital. A liver biopsy revealed severe bile duct injury and mild cholestasis. He was diagnosed with celecoxib-induced VBDS and underwent bilirubin adsorption therapy. However, his condition continued to deteriorate, and he died. An autopsy showed that liver regeneration was poor, and bile duct loss was exacerbated. The pathological autopsy findings were consistent with VBDS.

Mesenchymal-epithelial transition (MET) exon 14-skipping mutation (METex14) is rare in pulmonary invasive mucinous adenocarcinomas (IMAs), and the clinical impact of MET-tyrosine kinase inhibitors (TKIs) remains unknown. We herein report a 75-year-old woman with IMA harboring METex14 who was treated with the MET-TKI tepotinib. The lung tumor regressed over six months; however, the patient ultimately died of exacerbated interstitial lung disease (ILD), possibly associated with tepotinib. An autopsy revealed diffuse alveolar damage in preexisting chronic fibrosis. We discuss how to pre-evaluate ILD deterioration risks and monitor TKI-induced lung toxicity during treatment.

We herein report a 76-year-old woman with situs inversus and dextrocardia who underwent pacemaker implantation for sick sinus syndrome. Situs inversus with dextrocardia, which is frequently associated with cardiovascular malformation, is a rare congenital malformation wherein the thoracic and abdominal viscera are inverted compared with their normal positions. This renders the implantation of cardiac devices an arduous task. We therefore decided to gather preoperative anatomical information on patients with situs inversus and dextrocardia. We used three-dimensional computed tomography to collect preoperative information in order to facilitate the safe implantation of cardiac devices.

Citrin deficiency (CD) is a hereditary disorder caused by SLC25A13 mutations that manifests as neonatal intrahepatic cholestasis caused by CD (NICCD), failure to thrive and dyslipidemia caused by CD (FTTDCD), and adult-onset type 2 citrullinemia (CTLN2). Citrin, an aspartate-glutamate carrier primarily expressed in the liver, is a component of the malate-aspartate shuttle, which is essential for glycolysis. Citrin-deficient hepatocytes have primary defects in glycolysis and de novo lipogenesis and exhibit secondarily downregulated PPARα, leading to impaired β-oxidation. They are unable to utilize glucose and free fatty acids as energy sources, resulting in energy deficiencies. Medium-chain triglyceride (MCT) supplements are effective for treating CD by providing energy to hepatocytes, increasing lipogenesis, and activating the malate-citrate shuttle. However, patients with CD often exhibit growth impairment and irreversible brain and/or liver damage. To improve the quality of life and prevent irreversible damage, MCT supplementation with a diet containing minimal carbohydrates is recommended promptly after the diagnosis.

A 65-year-old man with type 2 diabetes who was being treated with metformin developed lactic acidosis following excessive alcohol consumption. While an impaired renal function is a major risk factor for metformin-associated lactic acidosis (MALA), the patient's basal renal function was normal. Alcohol misuse reduces lactate clearance by utilizing nicotinamide adenine dinucleotides for ethanol oxidation, thereby promoting vulnerability to MALA. Nevertheless, as MALA in individuals with a normal renal function is extremely rare, the clinical picture of alcohol-induced MALA is unclear. We delineate the clinical picture and discuss the pathogenesis of alcohol-induced MALA based on our experience and previous case reports.

A 53-year-old man presented with abdominal symptoms and a fever for 6 months and ascites and lower body edema for 2 months before visiting our clinic. Heart failure, renal failure, inferior vena cava or portal vein obstruction, cirrhosis, and malignancy were suspected, but none were present. We also suspected protein-leakage gastroenteropathy based on the elevated alpha-1 antitrypsin clearance (224 mL/day). Based on the double-balloon endoscopy findings, we diagnosed the patient with primary intestinal lymphangiectasia. Since the patient's ascites were not satisfactorily controlled medically, a Denver peritoneovenous shunt was placed. As a result, the ascites volume was successfully controlled over an extended period.

Background Myocardial crypts are congenital abnormalities associated with hypertrophic cardiomyopathy (HCM) and other conditions. This study assessed the prevalence of myocardial crypts in Japanese patients.

Methods and Results Myocardial crypts were evaluated in a consecutive series of 300 patients (13-92 years old) who underwent computed tomography angiography (CTA) because of clinical suspicion of ischemic heart disease. We found a myocardial crypt incidence of 9.7% (29 patients) in our study population, with multiple crypts observed in 2.3% (7 patients). Among these, myocardial crypts were found in 2 out of 8 (25%) patients with hypertrophic cardiomyopathy (HCM), 1 of which was apical-type HCM. In patients with a single crypt (22 patients), the most common location of the crypt was at the left ventricular apex (16/22 patients, 72.7%), followed by the inferior wall (5/22 patients, 22.7%) and the interventricular septum (1/22 patients, 4.6%).

Conclusion The incidence of myocardial crypts observed in our study aligns with that reported in previous studies, although the most common location among the Japanese population was the left ventricular apex.

Non-tuberculosis mycobacterial (NTM) pulmonary disease (NTM-PD) is quite common, and newly identified species are being reported increasingly frequently thanks to advances in identification technologies. A 56-year-old woman had mild sputum production showed bronchiectasis with multiple small nodules, consistent with NTM-PD, on chest computed tomography. Mycobacterial species were isolated from the specimens; however, conventional methods could not identify the species. We conducted whole-genome sequencing and identified the NTM isolates as Mycobacterium kiyosense, a species newly registered in 2023 from Japan. She was diagnosed with NTM-PD caused by M. kiyosense and received watchful waiting.

Rectal prolapse is typically treated surgically, and internal therapy has not been reported. We encountered a case of rectal prolapse that improved with an over-the-scope clip system (OTSC). An 81-year-old woman complaining of anorectal pain underwent colonoscopy, and rectal prolapse was observed prior to colonoscopy. Unfortunately, rectal perforation occurred while attempting endoscopic reversal. The OTSC system was used to close the rectal perforation and subsequently improved her rectal prolapse, probably because the rectal wall was anchored to the retroperitoneum. This is the first report to show that rectal prolapse can be endoscopically improved and that an OTSC system might be a viable alternative method for managing inoperable rectal prolapse.

Traditionally, the treatment of chronic constipation has focused on lifestyle modification, dietary guidance and therapy, and osmotic and stimulant laxatives. Recently, several drugs with new mechanisms of action have been introduced as treatments for chronic constipation. In Japan, polyethylene glycol and lactulose can now be administered under insurance coverage. The number of treatment options for constipation has increased dramatically. First, lifestyle modifications and dietary therapies must be implemented. If constipation does not improve sufficiently, specialized functional tests are performed to diagnose physiological subgroups. If functional tests are not available, patients are classified as having the "decreased frequency of defecation" type or the "difficult defecation" type based on the patient's symptoms, with treatment applied according to each type. Medical therapy includes osmotic laxatives, secretagogues, bile acid transporter inhibitors, probiotics, prokinetics, and Kampo medicines. The temporary use of stimulant laxatives, suppositories, enemas, and digital evacuation is also recommended. The usefulness of biofeedback is yet to be determined.

Notably, certain nutrients are effective in preventing migraine. Nonetheless, zinc replacement therapy for migraine treatment has yet to be explored. We herein report four patients with migraine who were refractory to prophylactic therapy and whose headache frequency and severity improved with zinc supplementation. Zinc administration may be an option for treating patients with prophylaxis-refractory migraine. Further investigation is required to determine the efficacy of zinc replacement therapy as a treatment option for migraine.

We herein report a 79-year-old woman with subacute progressive ataxic sensory neuropathy. The patient's symptoms began with numbness in the lower extremities, which rapidly deteriorated, resulting in gait disturbance and abnormal sensations in the extremities, reaching a peak over a period of approximately two months. Nerve conduction studies revealed pure axonal-type sensory polyneuropathy. The expeditious progression of the disease initially prompted suspicion of Guillain-Barré syndrome or paraneoplastic syndrome. Nevertheless, after comprehensive evaluations, the conclusive diagnosis was confirmed as ataxic sensory neuropathy with Sjögren's syndrome. Intensive immunotherapy was administered; however, it was ineffective in halting disease progression. Consequently, this case underscores the significance of an early comprehensive diagnosis and prompt immunotherapy for ataxic sensory neuropathy associated with Sjögren's.

Objective Of the highly accurate tests for current Helicobacter pylori infection, the urea breath test (UBT) and stool antigen test (SAT) are noninvasive and do not require endoscopy. We conducted a prospective study to evaluate the accuracy of the newly developed SAT in a medical checkup setting.

Methods The accuracy of the proposed SAT was examined by determining H. pylori infection status based on a history of eradication therapy, endoscopic H. pylori infection diagnosis, and blood tests (serum H. pylori antibody, serum PG II) in individuals undergoing esophagogastroduodenoscopy (EGD) during a health checkup.

Results The new SAT showed 97.3% (108/111) sensitivity for those "currently infected," as well as 99.3% (530/534), 98.0% (402/410), and 98.7% (932/944) specificity for those "never infected," those "previously infected," and those "never/previously infected", respectively.

Conclusion The newly developed H. pylori SAT may be useful for diagnosing H. pylori infection. Patients should be suspected of being infected even after H. pylori eradication if they have a high cutoff index in this test.

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. She had received two doses of the COVID-19 vaccine one year before the onset of the disease, after which the estimated glomerular filtration rate increased. Laboratory tests and other tests led to a diagnosis of hyperthyroidism, and a kidney biopsy showed thrombotic microangiopathy-like glomerular microangiopathy comprising mainly glomerular endothelial cell damage. Thiamazole (30 mg) was started for the hyperthyroidism. Three months later, the thyroid function normalized, and two months later, the proteinuria and hematuria disappeared, suggesting that COVID-19 vaccination and these events were related.

Objective We retrospectively compared the dose, cost, and safety of vadadustat and daprodustat for the treatment of renal anemia in patients with chronic kidney diseases who were not undergoing dialysis.

Methods The primary outcome of this study was the change in dose and cost from the initiation of vadadustat and daprodustat treatment. The secondary outcome was the drug safety.

Patients We treated 30 patients each with the hypoxia-inducible factor prolyl-hydroxylase inhibitors (HIF-PHIs) daprodustat and vadadustat. The hemoglobin (Hb) concentration was targeted at 11-13 g/dL, and transferrin saturation was maintained at ≥20%, as per the 2018 Japanese guidelines for the diagnosis and treatment of chronic kidney disease.

Results Hb levels increased from 10.7 to 11.5 g/dL after the first month of daprodustat administration, whereas those for vadadustat patients remained relatively stable, going from 10.7 to 10.6 g/dL. After six months, the Hb level reached 12.1 g/dL and 11.3 g/dL for daprodustat and vadadustat, respectively. The dosage of vadadustat was significantly increased by 46% and 70% after 3 and 12 months, respectively, compared with the initial doses, whereas that of daprodustat did not change substantially. The average cost of vadadustat also increased in the first 3 months and remained over 500 yen/day after 3 months, while that of daprodustat showed little change from the initial cost of 360 yen/day.

Conclusion These results suggest that heterogeneity exists in the drug potency and dosage required for treatment between daprodustat and vadadustat. Serious adverse events (death, cardiovascular disease, end stage renal disease (ESRD), and malignancy) occurred in more than 20% of participants with both HIF-PHIs. Further studies are required to confirm the safety of HIF-PHIs.