Objective The etiology of liver cancer is changing to metabolic dysfunction-associated steatotic liver disease, and ultrasound elastography is useful for identifying high-risk patients. However, these regional differences remain unclear. We aimed to investigate regional differences in the prevalence of liver cancer and the use of ultrasound elastography in Japan using the National Database of Health Insurance Claims (NDB).

Methods We used NDB open data from 2020. The standardized claims data ratio (SCR) was used to evaluate regional differences. We investigated medical receipt information for liver cancer, diabetes-related diseases, and elastography in 47 prefectures.

Results The highest SCR for liver cancer was observed in Kyushu [median 134, IQR (109-139)], followed by Chugoku [113, (110-132)], Kinki [109, (89-126)], and Shikoku [107, (100-121)], highlighting a significant concentration in Western Japan (The mean SCR; 114.9 in Western Japan, 88.5 in Eastern Japan: p<0.0001). A higher SCR has also been observed in the treatment of liver cancer, including hepatic resection and radiofrequency ablation, in Western Japan. Similarly, in Western Japan, higher SCRs have also been observed for diabetes-related diseases, such as glucose intolerance, ischemic cardiovascular disease, angina pectoris, and ischemic cerebrovascular disease. However, the SCR for transient elastography and shear wave elastography is low in many prefectures throughout Japan.

Conclusion The morbidity of liver cancer and diabetes-related diseases is higher in Western Japan. However, ultrasound elastography is not widely used in Japan. There is an urgent need to promote awareness of ultrasound elastography to screen patients at high risk for liver cancer, particularly in Western Japan.

Objective The Glasgow Coma Scale (GCS) is widely used to assess the levels of consciousness. This study examined whether or not the initial GCS score could be a marker of the length of hospital stay (LOS) in patients with thyroid storm.

Methods We retrospectively analyzed 29 patients with thyroid storm in our hospital between January 2010 and December 2023. Thyroid storm was diagnosed based on the latest diagnostic criteria in Japan. Since 30 days is the upper limit of LOS in the Diagnosis Procedure Combination [DPC]-based reimbursement system, the study population was divided into patients with permissible LOS (<30 days) and those with undesirable LOS (≥30 days). As a supplementary analysis, we examined the correlation of the GCS score and other clinical parameters with LOS using a Spearman's rank correlation analysis.

Results The GCS score was significantly higher (14.1±2.7 vs. 9.9±4.9 points; p<0.001), and the Sequential Organ Failure Assessment (SOFA) score significantly lower (1.68±2.16 vs. 7.50±7.91 points; p=0.009) in patients with permissible LOS than in those with undesirable LOS, while other clinical parameters were not significantly different between the groups. The area under the receiver operating characteristic curve of the GCS for the permissible vs. undesirable LOS was 0.866. The baseline parameters that were significantly correlated with LOS were the GCS (ρ=-0.665; p<0.001) and SOFA score (ρ=0.670; p<0.001). The subsequent rank partial correlation analysis showed that GCS was still inversely correlated with LOS, independent of SOFA score (ρ=-0.390; p=0.040).

Conclusion GCS, which can be evaluated more easily and quickly than the SOFA score, is a useful marker of LOS in patients with thyroid storm.

Objective Community-acquired pneumonia is an acute infectious disease with potentialy life-threatening consequences. Because invasive mechanical ventilation (IMV) requires the attention of many medical staff, early risk prediction at the time of admission is expected to lead to a predictable course of patient care and the appropriate allocation of medical resources. There are a limited number of reports on predictive factors for IMV, such as SMART-COP. Therefore, further studies are required.

Methods We retrospectively reviewed cases of patients with community-acquired pneumonia other than coronavirus disease 2019 admitted to our institution from 2002 to 2019. We performed competing risks analysis with the need for IMV from the day after admission as the outcome and used multivariable analysis to identify predictive factors of IMV from admission characteristics.

Results Among 2,227 patients (mean age 67.3 years, 69.0% male), 39 patients required IMV on or after the day following admission. A multivariable analysis showed that predictive factors of IMV were respiratory rate >30 breaths/min [subdistribution hazard ratio (SHR), 5.53; 95% confidence interval (CI), 2.09 to 14.67; p=0.001], PaO₂/FiO₂ ratio <250 (SHR, 8.02; 95% CI, 2.78 to 23.13; p<0.001), and Legionella pneumonia (SHR, 4.87; 95% CI, 1.56 to 15.13; p=0.006).

Conclusion This study revealed that among other factors including mainly vital signs, specific infection by a microorganism itself (Legionella in this study) was a predictive factor for the need of IMV.

Objective The prevalence rates of bronchial asthma (BA) and chronic obstructive pulmonary disease (COPD) are 3-11% and 8-16%, respectively, in the general Japanese adult population. Few reports on patients' perceptions of BA, cough variant asthma (CVA), COPD, and asthma and COPD overlap (ACO) are available in Japan, and we aimed to investigate the agreement between the perception and diagnosis of BA and COPD-related diseases.

Methods The subjects were 229 datasets matched to the sex and age distribution of the Japanese population of 1,000 adult patients who underwent respiratory function tests and screening for each disease at Nagasaki University Hospital between July 2014 and July 2017. The patients' self-perceptions of diagnosed BA, CVA, COPD, emphysema, chronic bronchitis, and ACO were determined.

Results In total, 229 datasets were included in this study. The prevalence of BA was 10.0% (23 cases, including 2 CVA and 6 ACO cases) and 11.8% (27 cases, including 6 ACO cases). The prevalence of ACO was 2.6% (28.6% of BA and 22.2% of COPD), and that of CVA was 0.9% (8.7% of BA). The perception of COPD-related diseases had a much lower sensitivity than that of asthma-related diseases (0.481 vs. 0.995, p<0.0001). Cohen's kappa for asthma-related disease was 0.976, and that for COPD-related disease was 0.621.

Conclusion Self-perception of asthma-related disease was adequately high, whereas that of COPD-related disease was low. The prevalence rates of BA, COPD, ACO, and CVA in our study were 10.0%, 11.8%, 2.6%, and 0.9%, respectively. An increase in the perception of COPD may help improve community healthcare for respiratory diseases.

Objective Spastic paraplegia 7 (SPG7) is an autosomal recessive neurodegenerative disorder caused by biallelic pathogenic variants in SPG7. It is predominantly characterized by adult-onset slowly progressive spastic paraparesis. While SPG7 presenting with ataxia with or without spasticity is relatively common in Europe and North America, it is considered rare in Japan. This study aimed to identify SPG7 patients among those with undiagnosed ataxia within the Japanese population.

Methods We retrospectively selected 351 patients with undiagnosed ataxia, excluding those with secondary and common spinocerebellar ataxia. Whole-exome sequence analysis was conducted, and homozygosity of the identified variants was confirmed using droplet digital polymerase chain reaction (ddPCR).

Results Among the 351 patients, 2 were diagnosed with SPG7, and homozygosity was confirmed by ddPCR. Both patients carried homozygous pathogenic variants in SPG7: c.1948G>A, p.Asp650Asn, and c.1192C>T, p.Arg398Ter (NM_003119.4). Clinically, both patients presented with progressive ataxia. In addition, Patient 1 exhibited partial ophthalmoplegia and spastic paraparesis, whereas Patient 2 demonstrated cerebellar ataxia without spasticity.

Conclusion The rarity of SPG7 in Japan may be attributed to variation in the minor allele frequency of the c.1529C>T, p.Ala510Val variant, which is more prevalent in Europe and North America than in other areas.

Objective Data on the first-line treatment options for patients with Pneumocystis pneumonia (PCP) without human immunodeficiency virus (HIV) infection are limited. Therefore, we evaluated the outcome of pentamidine compared to trimethoprim-sulfamethoxazole (TMP-SMX) in non-HIV patients with PCP.

Methods We used data from the Japanese Diagnosis Procedure Combination Inpatient Database. We included non-HIV PCP patients who initially received TMP-SMX or pentamidine between July 2010 and March 2022. We categorized eligible patients into TMP-SMX and pentamidine groups and performed a propensity score overlap weighting analysis to compare in-hospital mortality between the groups.

Results Among 5,870 eligible patients, 5,456 and 414 received TMP-SMX and pentamidine, respectively. Pentamidine treatment was associated with a higher in-hospital mortality than TMP-SMX treatment in the propensity score overlap weighting analysis (23.6% vs. 40.1%; risk difference, 16.5%; 95% confidence interval, 10.8-22.2%; p<0.001).

Conclusions Based on these findings, pentamidine may not be as effective as TMP-SMX for treating PCP in non-HIV patients.

Objective Although the management of coronavirus disease 2019 (COVID-19) has improved, chemoprevention remains a challenge. We recently identified that ursodeoxycholic acid (UDCA) is associated with subclinical infection with severe acute respiratory syndrome coronavirus, implying a reduction in the severity of COVID-19. We analyzed a large medical database to assess the utility of UDCA in the reduction of COVID-19 severity.

Methods This retrospective observational study was conducted using a large-scale healthcare administrative claims database. We extracted data on patients who were diagnosed with either chronic hepatitis B or C. Among them, patients ≥50 years of age diagnosed with COVID-19 before December 2022 were analyzed. Patients were divided into two groups: those with or without a prescription of UDCA. The primary outcome was the in-hospital mortality rate. A propensity score-matching analysis was performed using logistic regression.

Results A total of 6,413 patients diagnosed with COVID-19 (UDCA group, n =579; non-UDCA group, n =5,834) were analyzed. The median age was 73.0 (interquartile range, 64.0-81.0) years, and 57.8% of the patients were men. The UDCA group had significantly more complications with liver cirrhosis, hepatocellular carcinoma, type 2 diabetes, and hypertension. The UDCA group had a higher in-hospital mortality rate than the non-UDCA group, even after propensity score matching (7.4% vs. 4.3%, p=0.03), whereas there was no difference in the risks of hospitalization, oxygen therapy, or ventilation.

Conclusion Although the observed increase in mortality among UDCA users could have been due to unmeasured confounding factors, UDCA did not reduce the severity of COVID-19 in viral hepatitis patients.

A 32-year-old man presented with cirrhosis. At 8 years of age, he underwent resection of a craniopharyngioma, which resulted in panhypopituitarism. He underwent self-interrupted hormone replacement therapy at 20 years of age. Computed tomography revealed severe fatty liver and cirrhosis. An endocrinological evaluation revealed panhypopituitarism. Further assessment revealed a diagnosis of hepatopulmonary syndrome. Home oxygen therapy and hormone replacement therapy were initiated. Despite these efforts, poorly controlled hypothalamic obesity led to liver failure, and the patient is currently awaiting liver transplantation. Liver cirrhosis associated with long-term panhypopituitarism may have a poor prognosis even with hormone replacement therapy.

Cancer cachexia, which is characterized by weight loss and muscle weakness, is common in patients with pancreatic cancer. Anamorelin, a ghrelin receptor agonist, has shown potential for the management of cachexia in various cancers. We herein report a 64-year-old man with unresectable pancreatic cancer who experienced significant weight gain with anamorelin, allowing for continued chemotherapy and an improved quality of life. Despite disease progression, his response suggests the potential utility of anamorelin as a supportive therapy. Cachexia's complex metabolic changes make treatment difficult; however, anamorelin's appetite and weight gain effects highlight its possible role in managing cachexia in pancreatic cancer. Therefore, further research is required in this regard.

Although sarcoid-like reactions are uncommon in colorectal cancer, they can coexist with other cancers. We herein report a 50-year-old woman with sigmoid adenocarcinoma and osseous sarcoid-like reactions that resembled metastases. Positron emission tomography-computed tomography revealed an uptake in the iliac bone and para-aortic lymph nodes. The depth of invasion indicates non-metastatic lesions. Bone and lymph node biopsies revealed epithelioid non-caseating granulomas with bone trabeculae, resulting in a final diagnosis of pathological T2N2aM0, Stage IIIB. When the expected depth of invasion differs from that of typical bone metastases, colorectal cancer-associated bone lesions should be evaluated for sarcoid-like reactions.

A 67-year-old woman underwent a screening colonoscopy, which revealed a 7-mm rectal subepithelial lesion. Endoscopic submucosal dissection (ESD) confirmed a grade 1 rectal neuroendocrine neoplasm (NEN), and no further treatment was administered. One year after ESD, she was diagnosed with lung cancer, which recurred 4 years later and required chemotherapy. Nine years after ESD, multiple liver metastases from the rectal NEN were found, which were well controlled with radiofrequency ablation. The patient died 13 years after ESD due to the progression of lung cancer. This case highlights the need for long-term follow-up in patients with small rectal NENs treated with endoscopic resection.

A 70-year-old man with a history of left-sided renal donation surgery 11 days earlier developed rupture of a pancreaticoduodenal artery (PDA) aneurysm caused by median arcuate ligament syndrome (MALS). The patient also had a congenital anomaly and left-sided inferior vena cava (IVC). Surgical hemostasis was performed; however, the patient developed a massive pulmonary embolism on day 4 of hospitalization. Chest contrast-enhanced computed tomography revealed compression of the IVC by the abdominal aorta and a hematoma resulting from aneurysm rupture, which was considered the source of deep vein thrombi. Although PDA aneurysms related to MALS and left-sided IVC are rare conditions, PDA aneurysm rupture is life-threatening, and left-sided IVC presents a potential risk for deep vein thrombosis. However, comprehensive management strategies for these conditions have not yet been established.

A 71-year-old Japanese woman was hospitalized for heart failure with Loeffler endocarditis, bronchial asthma, fever, and mononeuritis multiplex after receiving the fifth dose of the coronavirus disease 2019 (COVID-19) vaccine. Laboratory tests revealed eosinophilia and increased antineutrophil cytoplasmic antibody levels. The patient's condition improved after anticoagulant therapy.

Loeffler endocarditis with antineutrophil cytoplasmic antibody-associated vasculitis may be associated with the COVID-19 vaccination.

We herein report a 56-year-old man with severe hypocalcemia during ruxolitinib therapy for myelofibrosis transitioning from Janus kinase 2 mutation-positive polycythemia vera. Blood transfusions were administered every one to two weeks for ruxolitinib-induced anemia. Blood tests revealed hypocalcemia with low tartrate-resistant acid phosphatase-5b, 25-hydroxyvitamin D (25 (OH) D), and 1,25-dihydroxyvitamin D₃ (1,25 (OH) ₂ D₃) levels within the lower reference range. Intact-parathyroid hormone levels were relatively low compared to calcium levels. Severe hypocalcemia with ruxolitinib is rare and may be caused by a combination of factors, impaired vitamin D activation due to liver or renal insufficiency, accumulation of calcium-chelating agents from blood transfusions, and inadequate compensatory response to PTH.

Pseudohypoparathyroidism (PHP) is a rare disorder characterized by resistance to parathyroid hormone (PTH). Albright's hereditary osteodystrophy (AHO) is a characteristic physical condition associated with PHP. We herein report a 33-year-old Japanese man diagnosed with PHP type 1b who presented with hypocalcemia, preserved PTH secretion, decreased urinary cyclic adenosine monophosphate and phosphate excretion (Ellsworth-Howard test), deletion of exons 5-7 in STX16, hypomethylation of A/B-differentially methylated region (DMR) and AS1-DMR in the guanine nucleotide binding protein alpha stimulating gene. The patient did not have AHO or other comorbidities but showed digital clubbing during childhood. Although digital clubbing is rarely associated with PHP, it may represent a phenotype of this disorder.

A 16-year-old girl presented with a high fever that had persisted for more than 4 weeks. Computed tomography revealed a 4-cm mass in the left adrenal gland. Clinically, there were no obvious symptoms of adrenal hormone excess; however, serum interleukin-6 (IL-6) and C-reactive protein levels were significantly elevated. After laparoscopic left adrenalectomy, the fever subsided, and her IL-6 level normalized. The tumor was pathologically diagnosed as adrenocortical carcinoma (ACC), with a Weiss score of 5/9. The tumor cells were immunoreactive for IL-6. To our knowledge, this is the first case report of symptomatic IL-6-producing ACC that initially presented with a persistent fever.

A 75-year-old man with a fever, shoulder pain, and lower leg edema was diagnosed with polymyalgia rheumatica and started on glucocorticoid therapy. Eighteen months later, he was admitted with rapidly progressive renal failure. Glucocorticoid therapy had been discontinued one month prior to admission. Serum anti-glomerular basement membrane antibodies were elevated, and a kidney biopsy revealed fibrinoid necrosis of the medium-sized renal arteries, tubulointerstitial nephritis, and collapsed glomeruli. An immunofluorescence study showed mild immunoglobulin G linear deposition. Polyarteritis nodosa was diagnosed based on the presence of fibrinoid necrosis in the medium-sized renal arteries. Glucocorticoid pulse therapy and plasmapheresis were initiated, but the patient died of alveolar hemorrhaging. This was a rare case of Goodpasture's syndrome with polyarteritis nodosa.

A 78-year-old woman experienced systemic edema and was diagnosed with nephrotic syndrome and Hashimoto's thyroiditis (HT). A renal biopsy revealed minimal change disease (MCD) and tubulointerstitial nephritis, which resulted in the diagnosis of primary Sjögren's syndrome (PSS). PSS and HT can be complicated with MCD; however, there are no published case reports of MCD presenting with both conditions simultaneously. We aimed to inform nephrologists and rheumatologists about this rare condition through a literature review of renal outcomes in patients with MCD associated with PSS and HT.

Fabry disease is a rare X-linked lysosomal storage disorder. Enzyme replacement therapies (ERTs), such as agalsidase α and β, are available treatment options. While infusion-related reactions (IRRs) are known to occur at the initiation of ERT owing to immune responses, there is limited information on IRRs during long-term ERT. We report the case of a female patient with Fabry disease who developed unexpected hypotension after six years of stable treatment with agalsidase α, leading to a switch to agalsidase β. Continuous monitoring may be essential to identify potential IRRs in female patients with Fabry disease receiving long-term ERT.

Lysinuric protein intolerance (LPI) is an autosomal recessive disease and pulmonary alveolar proteinosis is a respiratory complication. A Japanese man in his 30s, diagnosed with LPI in infancy, was diagnosed with interstitial lung disease (ILD) similar to fibrotic nonspecific interstitial pneumonia (f-NSIP) based on the findings of a transbronchial lung cryobiopsy. The pulmonary function deteriorated, and nintedanib was administered. Two years after initiation of nintedanib therapy, the patient was hospitalized for an acute exacerbation of interstitial pneumonia. Corticosteroid pulse therapy was administered twice, but the patient died approximately one month after emergency hospitalization. This is the first report of a patient with LPI diagnosed with ILD similar to f-NSIP using a cryobiopsy and who was treated with nintedanib.

A 52-year-old Japanese man with a history of childhood asthma presented at our emergency department with progressive dyspnea. Despite subcutaneous adrenaline injections, salbutamol nebulization, and intravenous methylprednisolone, the carbon dioxide partial pressure (pCO₂) increased to 110 mmHg. The patient was intubated, and mechanical ventilation was initiated because of severe respiratory failure. Severe bronchospasm frequently occurs despite appropriate treatment. Therefore, we decided to administer biologics. After the administration of a single dose of benralizumab, his respiratory condition improved, with normalization of pCO₂, tidal volume, and airway resistance. We successfully extubated the patient two days after the administration of benralizumab.

Currently, immune checkpoint inhibitors (ICIs) are the standard treatment for malignant pleural mesothelioma (MPM). The characteristic responses to ICI treatment include pseudoprogression (PP) and hyperprogressive disease (HPD), which require attention. Reports on PP of MPM are rare. We herein report a 59-year-old woman with MPM whose pleural lesions rapidly increased in size after initiating nivolumab treatment. This was initially judged as HPD but was subsequently diagnosed as PP owing to a marked reduction in tumor size. This case highlights the importance and difficulty of differentiating between PP and true progression, including HPD, during treatment of MPM with ICIs.

Phlegmonous gastritis (PG) is a rare, life-threatening bacterial infection characterized by thickening of the gastric wall. We report a case of PG in a 17-year-old male following allogeneic hematopoietic stem cell transplantation for severe aplastic anemia. Seven months after transplantation, the patient presented with severe abdominal pain, fever, and significant gastric wall thickening on CT. Initial antibiotic therapy was initiated immediately. The patient developed multiple organ failure; however, his condition rapidly improved with intensive care. After two weeks, the patient improved and was successfully discharged. This is the first report of PG after transplantation, highlighting the need for an early diagnosis and treatment.

This report describes the case of a 29-year-old patient with chronic myeloid leukemia in the blast phase who underwent hematopoietic stem cell transplantation (HSCT) after living-donor liver transplantation. Donor selection, conditioning regimens, and graft-versus-host disease (GVHD) prophylaxis are discussed. The patient received a non-myeloablative conditioning regimen and ABO blood group-matched unrelated human leukocyte antigen fully-matched donors. Immunosuppressants tacrolimus and mycophenolate mofetil were administered to prevent GVHD. Maintenance therapy with ponatinib effectively maintained remission. This case highlights the complexities of managing HSCT after solid organ transplantation and suggests strategies for future cases.

A 51-year-old woman with persistent proliferation of natural killer (NK) cells in her peripheral blood was diagnosed with NK-large granular lymphocytic leukemia (NK-LGLL). During follow-up, computed tomography revealed multiple infiltrative pulmonary lesions. A flow cytometric analysis of bronchoalveolar lavage fluid showed infiltration of NK cells, resulting in a diagnosis of pulmonary infiltration by NK-LGLL. A histological examination of thoracoscopic lung biopsy specimens supported this diagnosis. She was started on 1 mg/kg prednisolone, which resulted in a durable reduction in these lesions. This is the first report of a NK-LGLL patient with pulmonary infiltration who received medication. Furthermore, treatment with prednisolone alone was successful.

The patient was a 36-year-old woman in the 34th week of pregnancy who presented with headache. Magnetic resonance imaging (MRI) revealed bilateral internal carotid artery (ICA) stenosis and inflammatory changes in the sphenoid sinus. No cerebral infarction was detected on diffusion-weighted magnetic MRI. She was diagnosed with ICA stenosis induced by sphenoid sinusitis and underwent surgical treatment with intravenous antibiotics and corticosteroids. Her headache improved, and MRI showed remarkable improvement in bilateral ICA stenosis and no cerebral infarction. Severe sinusitis can also cause ICA stenoses. An early diagnosis and appropriate treatment of causative sinusitis can improve ICA stenosis and prevent stroke.

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a new spectrum of autoimmune inflammatory nervous system disorders associated with infection, neoplasm, or drug use. We present the first pediatric case of GFAP astrocytopathy caused by an influenza A infection. The patient manifested meningoencephalitis, central respiratory failure, quadriplegia, elimination disorders, and syndrome of inappropriate antidiuretic hormone secretion. Methylprednisolone pulse therapy led to a good prognosis. In cases of progressive atypical complications of influenza infection, immunotherapy-reactive GFAP astrocytopathy should be considered as a differential diagnosis for prompt treatment.

Nicorandil is commonly used to treat angina pectoris. However, the adverse neurological effects of nicorandil remain unclear. Herein, we report the case of a patient who presented with isolated abducens nerve palsy associated with the administration of nicorandil. A 74-year-old man presented to our hospital with abducens nerve palsy and headaches. The patient had started nicorandil treatment one week prior. No abnormalities were observed in the laboratory tests or neuroradiological findings. The patient recovered immediately after the discontinuation of nicorandil treatment. In cases of cranial nerve palsy after the initiation of nicorandil treatment, consideration of the involvement of nicorandil is necessary.