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.

Objectives Fabry disease is characterized by the systemic accumulation of globotriaosylceramide (Gb3) and globotriaosylsphingosine (Lyso-Gb3), which are widely used as biomarkers of the disease. However, few reports have described the relationship of Lyso-Gb3 analogs and Gb3 isoforms with the disease. The present study determined the profiles of Lyso-Gb3 analogs and Gb3 isoforms accumulated in body fluids from various phenotypic Fabry patients to elucidate the basis of the disease.

Methods Plasma Lyso-Gb3 and related analogs were measured in 15 classic Fabry men, 6 later-onset Fabry men, 11 Fabry women, and 36 controls, while urinary Gb3 isoforms were measured in 5 classic Fabry men, 5 later-onset Fabry men, 17 Fabry women, and 11 controls, using liquid chromatography-tandem mass spectrometry (LC-MS/MS). Furthermore, these values were monitored for a classic Fabry man, in whom neutralizing anti-drug antibodies had developed following enzyme replacement therapy (ERT).

Results The levels of plasma Lyso-Gb3 analogs/urinary Gb3 isoforms were higher in Fabry patients than in controls, especially in classic Fabry men. However, minor differences in the ratio of each Lyso-Gb3 analog and Gb3 isoform with respect to the total Lyso-Gb3 analogs and Gb3 isoforms, respectively, were observed among individual classic Fabry men. Their time courses were well associated with the development and attenuation of anti-drug antibodies in a patient with classic Fabry disease during ERT.

Conclusion Quantification of Lyso-Gb3 analogs and Gb3 isoforms provides us with more detailed information about the substrates that accumulated in the body fluids of Fabry patients than does quantification of Lyso-Gb3 and Gb3 alone, so this approach may be useful for elucidating the basis of Fabry disease.

Objective While an association between a reduced kidney function and hyperuricemia has been reported, its association with hypouricemia is not well understood. The present study therefore investigated this association.

Methods Using a large Japanese health examination dataset, we performed a multivariable logistic regression analysis to assess the association between serum uric acid (SUA) levels and a reduced kidney function. The covariates included the age, body mass index, alcohol intake, and the presence of hypertension, dyslipidemia, or diabetes.

Patients This study included 227,672 patients (104,854 men; 46.1%), and the analyses were performed separately for men and women. The patients were classified into 5 groups: hypouricemia (SUA ≤2.0 mg/dL) (1st) and four other (2nd-5th) groups with SUA levels of ≤2.0, 2.1-5.1, 5.2-5.9, 6.0-6.8, ≥6.9 mg/dL in men and ≤2.0, 2.1-3.7, 3.8-4.4, 4.5-5.1, ≥5.2 mg/dL in women, respectively.

Results The characteristics of the study population were as follows: men, age 55.9±14.9 years old, SUA 5.9±1.3 mg/dL, estimated glomerular filtration rate (eGFR) 80.0±17.2 mL/min/1.73 m², and a reduced kidney function (eGFR <60.0 mL/min/1.73 m²) 9.4%; women, age 57.3±15.0 years old, SUA 4.5±1.1 mg/dL, eGFR 81.2±18.0 mL/min/1.73 m², and a reduced kidney function 9.4%. Compared with the 2nd group, the other 4 groups groups had a significantly higher prevalence of a reduced kidney function [odds ratio (OR), 2.58; 95% confidence interval (CI), 1.64-4.06 in men; OR, 1.66; 95% CI, 1.16-2.39 in women].

Conclusion The prevalence of a reduced kidney function was high in both men and women in the hypouricemia and high-SUA groups. SUA levels and the prevalence of a reduced kidney function showed a J-shaped association.

Objective Chronic myeloid leukemia (CML) is a malignant hematological disorder, and allogeneic stem cell transplantation (allo-SCT) was its only curative treatment until the introduction of tyrosine kinase inhibitors (TKIs). Allo-SCT is still considered for CML patients who are resistant to TKIs and in an advanced phase. Currently, second- and third-generation (2/3G TKIs are typically incorporated into the first-line treatment of CML. However, the impact of 2/3G TKIs on subsequent allo-SCT remains unclear. We therefore evaluated the effect of 2/3G TKIs on allo-SCT.

Methods We retrospectively evaluated the effect of pretransplant therapy with TKIs on the outcome of allo-SCT for CML using clinical data at our institution.

Patients Thirty-two CML patients who received their first allo-SCT procedure at our institute from 2001 to 2020 were included. We divided the patients into three subgroups based on TKI treatment before allo-SCT. Patients receiving no TKIs, only imatinib (IM), and 2/3G TKIs were classified into the Non-TKI, IM, and 2/3G TKI groups, respectively.

Results In a univariate analysis, the pretransplant use of 2/3G TKIs was significantly associated with a higher 5-year overall survival (91.7%) and relapse-free survival (75.0%) than the use of IM (37.5% and 12.5%) in patients presenting with or progressing to the advanced phase. In addition, pretransplant use of 2/3G TKIs did not increase the incidence of graft-versus-host disease (GVHD).

Conclusion We demonstrated that the pretransplant use of 2/3G TKIs was safe and improved the outcome of CML patients who presented with or progressed to the advanced phase without increasing the frequency of GVHD.

We herein report a rare case of idiopathic portal hypertension (IPH)-like disease that developed after allogeneic hematopoietic stem cell transplantation (allo-HSCT). A 53-year-old woman who underwent allo-HSCT for acute myeloid leukemia showed portal hypertension with radiological and histopathological findings consistent with IPH, distinct from veno-occlusive disease (VOD) and graft-versus-host disease (GVHD) of the liver. This case highlights the importance of considering IPH-like disease as a potential cause of portal hypertension after allo-HSCT. Awareness of this complication can aid in the early diagnosis and appropriate management of patients post allo-HSCT.

Cornelia de Lange syndrome (CdLS) is a congenital disorder occasionally associated with congenital portosystemic shunt (CPSS). We herein report a patient with CdLS and CPSS who developed hepatocellular adenomas (HCAs). The patient presented to our hospital for the further investigation of newly diagnosed liver tumors. Imaging findings and pathological examination results indicated that the liver tumors were inflammatory HCAs that subsequently shrank following transcatheter arterial embolization (TAE). Patients with CdLS and CPSS are at risk of developing HCAs, and TAE may be an effective management strategy for HCA in these patients.

In patients with wild-type transthyretin cardiac amyloidosis (ATTRwt-CA), the uptake of the tracer on technetium-99m-labeled pyrophosphate (^99mTc-PYP) scintigraphy, which indicates amyloid transthyretin (ATTR) per se, is often observed in skeletal muscles, such as the abdominal oblique and gluteal muscles. Among extracardiac biopsies for confirming ATTR deposition in ATTRwt-CA, a ^99mTc-PYP imaging-based computed tomography (CT)-guided core needle biopsy of the internal oblique muscle has relatively high sensitivity. In some patients, the ^99mTc-PYP uptake is more pronounced in the gluteal muscles than in oblique muscles. We herein report two cases of ATTRwt-CA in which a CT-guided biopsy of the gluteus medius muscle with ^99mTc-PYP uptake confirmed the presence of ATTR deposits.

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.

Osmotic demyelination syndrome (ODS) occurs in patients with diabetes and hyponatremia. We herein report a case of ODS with chorea detected on serial magnetic resonance imaging (MRI), despite no prompt hyponatremia correction. A 74-year-old man with cirrhosis and uncontrolled type 2 diabetes developed an altered mental status and chorea during treatment for diabetic ketoacidosis (DKA). Despite no rapid sodium correction and normal initial brain MRI findings, serial MRI revealed ODS-related abnormalities. Clinicians should consider ODS in patients with DKA and a hyperosmolar hyperglycemic state displaying unconsciousness and neurological manifestations, including chorea, even without substantial changes in serum sodium levels. An MRI re-examination can help capture missing ODS complications.

A 42-year-old Japanese woman with end-stage renal failure due to hypertension presented with a systolic blood pressure of 160-200 mmHg despite treatment with 4 different antihypertensive agents. The plasma aldosterone concentration (PAC) and plasma renin activity (PRA) were elevated. Adrenal vein sampling suggested bilateral excessive aldosterone secretion, whereas adrenocortical scintigraphy showed right-dominant accumulation. Open bilateral nephrectomy and right adrenalectomy improved the systolic blood pressure, PAC, and PRA. A pathological examination revealed zona glomerulosa hyperplasia but not microaldosteronoma. This report shows that bilateral nephrectomy, not unilateral adrenalectomy, is a potentially effective treatment option for resistant hypertension with an elevated renin-angiotensin-aldosterone system in hemodialysis patients.

A 47-year-old man was admitted to our hospital with acute kidney injury, severe hypertension, heart failure, thrombocytopenia, and elevated lactate dehydrogenase. Renal biopsy revealed fibrin thrombi within the glomerular capillaries and moderate fibrotic intimal thickening in the interlobular arteries. The histological diagnosis was thrombotic microangiopathy (TMA). Regarding cardiac involvement, we found marked stenosis in the left anterior descending artery on coronary angiography and cardiomyopathy on myocardial biopsy. Blood concentrations of amphetamine and methamphetamine were high (14.1 ng/mL and 333 ng/mL, respectively). It is important to consider methamphetamine as a cause of renal TMA and multi-organ dysfunction.

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder that causes abnormal blood vessel formation and bleeding. We herein report a 61-year-old woman with aggravated HHT symptoms after hemodialysis initiation. She was diagnosed with HHT based on her recurrent bleeding, abnormal blood vessel formation, and family history. Despite bleeding complications, the patient required anticoagulants and antiplatelet agents to treat cardiovascular complications. Eventually, the patient died of extensive cerebral hemorrhaging. Our experience suggests that special attention should be paid to bleeding complications in high-risk patients.

A 69-year-old woman was referred to our hospital because of an acute kidney injury with macroscopic hematuria. She had been taking dabigatran for atrial flutter for six years. Based on the typical histological findings of her kidney biopsy and her history of dabigatran use with prolonged activated partial thromboplastin time, she was diagnosed with dabigatran-related nephropathy complicated by tubulointerstitial nephritis with IgA nephropathy. After prednisolone therapy, the renal function improved. Direct-acting oral anticoagulants, including dabigatran, may cause anticoagulant-related nephropathy similar to warfarin, even in patients with a normal renal function. Tubulointerstitial nephritis may coexist with dabigatran-related nephropathy, and prednisolone therapy should be considered in such cases. IgA nephropathy has been reported as a background disease, and caution should be exercised when encountering it.

A 75-year-old woman presented with nausea and vomiting. Magnetic resonance imaging (MRI) revealed that she had a pituitary mass. A biopsy revealed lymphocytic hypophysitis (LYH). Symptoms were improved by hormone replacement therapy. Although she was asymptomatic, follow-up MRI revealed an increase in the size of the mass. Intravenous methylprednisolone (IVMP) reduced the size of the mass; however, right ophthalmalgia and oculomotor nerve palsy developed. MRI showed that the pituitary mass had enlarged to the right oculomotor nerve in the cavernous sinus and to the right internal carotid artery (ICA), causing stenosis of the ICA. After IVMP administration, the symptoms dramatically improved, but ICA stenosis persisted.

A 53-year-old woman was admitted to our hospital because of increasingly frequent transient speech disturbance and left upper limb weakness. Brain magnetic resonance imaging and angiography revealed multiple intracranial stenoses, including the proximal right middle cerebral artery (MCA), without evidence of infarction. The diagnosis of primary angiitis of the central nervous system was established based on circumferential vascular wall thickening with contrast enhancement observed in the right MCA. Following the administration of dual antiplatelet therapy and intravenous methylprednisolone pulse therapy, the patient experienced complete cessation of symptoms, and the stenosis gradually improved without infarction. The risk of infarct development in crescendo transient ischemic attacks may differ between inflammatory vascular stenosis and atherosclerosis.

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 syndrome.

We herein report a case of diffuse large B-cell lymphoma (DLBCL) involving multiple renal and bone infiltrations presenting with giant cell arteritis (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 plus intrathecal methotrexate/cytarabine/prednisone and rituximab, high-dose methotrexate, and cytarabine 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.

Clostridium paraputrificum bacteremia is very rare, and its clinical importance is poorly understood. An 86-year-old man was receiving lascufloxacin therapy for acute pharyngolaryngitis before presenting to our emergency department with a recurrent fever. Two sets of blood cultures on admission revealed C. paraputrificum. A stool culture showed a reduced presence of intestinal commensal bacteria. After admission, the patient's fever resolved without antibiotics. Colonoscopy revealed a rectal tumor. Rectal tumor and microbial substitutions caused by antibiotics may have led to bacteremia. When treating C. paraputrificum bacteremia, physicians should be mindful of coexisting gastrointestinal disorders and a history of antibiotic administration.

Allergic bronchopulmonary aspergillosis (ABPA) and chronic pulmonary aspergillosis (CPA) are diseases caused by Aspergillus infection, and CPA can develop from ABPA in some cases. We herein report a patient with CPA overlapping with ABPA. Serum cytokine levels were evaluated at 4 time points: the ABPA diagnosis, CPA diagnosis, 6 months after the start of voriconazole (VRCZ), and 12 months after re-administration of VRCZ. Interleukin (IL)-13 levels decreased upon glucocorticoid treatment, whereas IL-25 and IL-33 levels decreased rapidly with the initiation of antifungals. Early antifungal therapy may be important to control disease progression and prevent CPA overlap.