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 (pCO) 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 pCO, tidal volume, and airway resistance. We successfully extubated the patient two days after the administration of benralizumab.

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.

We herein report a 56-year-old man with severe hypocalcemia during ruxolitinib therapy for myelofibrosis transitioning from JAK2 mutation-positive polycythemia vera. Blood transfusions were administered every one to two weeks for ruxolitinib-induced anemia. Blood tests revealed hypocalcemia with low TRACP-5b, 25-hydroxyvitamin D (25 (OH) D), and 1,25-dihydroxyvitamin D (1,25 (OH) D) levels within the lower reference range. Intact-PTH 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.

Aortic intimal sarcoma is a rare disease with no established treatment and a poor prognosis. A 70-year-old man who underwent surgery for a mass shadow extending from the ascending aorta to the left common carotid artery on contrast-enhanced computed tomography was diagnosed with intimal sarcoma and underwent postoperative radiotherapy (66 Gy/33 Fr). Three brain metastases were identified after 1.5 months. No recurrence or enlargement was noted for four months following gamma knife radiosurgery and chemotherapy. To our knowledge, this is the first report of gamma knife radiosurgery delaying the progression of aortic intimal sarcoma.

A 17-year-old Japanese boy was admitted to our hospital with intermittent claudication. He belonged to a weightlifting team at a high school. He had occasionally dropped lightweight lifting weights on his right foot. Arteriography of the right anterior and posterior tibial arteries revealed a 4-cm total occlusion in the right dorsalis pedis artery (DPA). Intravascular ultrasonography revealed intimal thickening and thrombus formation He was diagnosed with thrombus occlusion of the right DPA due to intimal artery damage caused by lightweight weightlifting. DPA thrombosis may occur in young athletes who do not have atherosclerosis or vasculitis.

Whether or not pancreaticobiliary maljunction (PBM) is a risk factor for pancreatic cancer (PC) is unclear. We present a case of metachronous PC with PBM diagnosed after cholecystectomy for gallbladder cancer, in which follow-up imaging was possible until PC onset. A 63-year-old man who had been diagnosed with gallbladder cancer and had undergone cholecystectomy 5 years earlier developed pancreatitis. Pancreatography revealed PBM, and pancreatic duct brush cytology revealed adenocarcinoma. On reviewing the follow-up images, pancreatic morphological abnormalities, which had not been observed before cholecystectomy, were found to have gradually progressed, suggesting that biliopancreatic reflux led to pancreatic carcinogenesis.

Asthma is characterized by chronic airway inflammation as its primary pathological condition, which leads to various respiratory symptoms due to airway narrowing, with type 2 inflammation playing a central role. Asthma treatment, primarily centered on inhaled corticosteroids, aims to suppress type 2 inflammation and improve airway narrowing. However, severe asthma that cannot be controlled with high-dose inhaled corticosteroids or other asthma medications remains a clinical issue. The availability of multiple biological agents has recently improved the management of severe asthma. In addition, the concept of clinical remission has emerged as a treatment goal, further clarifying the objectives of asthma management. However, despite these advancements, the treatment of severe asthma driven primarily by non-type 2 inflammation remains a major challenge, and new biologics are currently being developed to address this issue.

Background Patients with hip fractures tend to have a poor prognosis. Although guideline-compliant practices are known to improve patient outcomes, there is a lack of evidence regarding the use of intervention to improve guideline adherence in hip fracture patients. The objective of our study was to evaluate guideline adherence by internists providing care to patients with hip fractures, using a protocol developed based on various guidelines. Method Protocol-driven care for hip fracture patients by internists began in April 2018 at our hospital. After its initiation, orthopedic surgeons performed the surgery, and the internists provided all other medical care. A controlled interrupted time-series analysis was used to evaluate the effects of protocol-driven care on guideline adherence to compare our hospital with other hospitals, using data extracted from a nationwide Japanese inpatient database covering the period April 2014 to March 2023. Results A total of 221,620 inpatients from 373 hospitals were included in the study. The initiation of protocol-driven care was associated with the guideline-recommended prescriptions: osteoporosis medication (Incidence rate ratio (IRR): 8.09; 95% CI 4.02-17.74), acetaminophen (IRR: 2.11; 95% CI 1.55-2.90), non-steroidal anti-inflammatory drugs (IRR: 0.16; 95% CI 0.11-0.24), and opioids (IRR: 5.96; 95% CI 3.14-12.15). However, there was no effect on the proportion of benzodiazepine prescriptions, surgery within 48 hours, deep venous thrombosis prophylaxis, or other perioperative outcomes, including medical fees. Conclusions The initiation of protocol-driven care by internists resulted in improved adherence to osteoporosis medication prescriptions and postoperative analgesic use compared with orthopedic care. This approach can be used as an effective method of care for elderly patients undergoing surgery.

We herein report the first case of dyspnea with hemidiaphragm elevation in a 68-year-old woman with active giant cell arteritis (GCA), including successful treatment. Contrast-enhanced computed tomography showed a reduced density of the left ophthalmic artery and the left superficial temporal artery with increased soft tissue compared to the other side, indicating that the GCA had flared up and suggesting that the hemidiaphragm elevation might be caused by vasculitis-associated ischemia of the right phrenic nerve. Hemidiaphragm paralysis due to vasculitis-associated ischemia in patients with GCA needs to be distinguished from local infection, tumors, and hepatomegaly, which are the major causes of hemidiaphragm elevation.

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.

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.

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. Patients and Methods We retrospectively reviewed cases of patients with community-acquired pneumonia other than COVID-19 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], PaO/FiO 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.

Background 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.

We herein report a case of Systemic Capillary Leak Syndrome (SCLS) attributed to coronavirus disease (COVID-19) that emerged in 2019. A 56-year-old woman presented with a COVID-19 infection 7 days prior to the visit with upper respiratory symptoms, fatigue, and decreased appetite. Secondary SCLS due to COVID-19 was diagnosed, veno-arterial extracorporeal membrane oxygenation (VA-ECMO) was initiated as mechanical support, and intravenous immunoglobulin was administered, marking the transition to the recovery phase with the initiation of fluid resuscitation. This case is noteworthy for successfully employing VA-ECMO in treating secondary SCLS due to COVID-19, mimicking the course of fulminant myocarditis.

A 65-year-old man with hepatocellular carcinoma and lung metastasis was treated with a combination of atezolizumab and bevacizumab. Subsequently, the patient developed fever and new liver lesions, which were considered to indicate a pseudoprogression. Despite this, the treatment was continued. Three months later, CT scans demonstrated that the new lesions had disappeared and both the primary tumor and metastases had shrunk. A partial response was achieved and maintained for two years. A histological examination revealed a predominance of CD8-positive lymphocytes and PD-L1-positive tumor-associated macrophages, which may predict a positive response to this therapy. Pseudoprogression may therefore be a favorable prognostic factor in hepatocellular carcinoma.

A 69-year-old man with systemic sclerosis and interstitial pneumonia presented with an abnormal shadow in the right upper lung lobe. A thoracoscopic needle biopsy was performed on the right upper lobe lesion, and Mycobacterium malmoense was identified by 16S rRNA and rpoB gene sequencing. Surgical treatment was performed to obtain a radical cure, and lung squamous cell carcinoma and M. malmoense infection were detected in the resected specimen. We herein report the first case of the successful treatment of a patient with pulmonary M. malmoense infection and concomitant lung squamous cell carcinoma.

A 51-year-old man presented to the emergency department with rapidly progressive dyspnea that developed while climbing Mount Fuji. He had climbed Mount Fuji twice without experiencing similar symptoms. On arrival, his oxygen saturation was 91% on 10 L/min of oxygen with a non-rebreather mask. Chest imaging revealed scattered bilateral infiltrating shadows. He was diagnosed with high-altitude pulmonary edema (HAPE) and treated with high-dose corticosteroids, calcium-channel blockers, antibiotics, and oxygen via a high-flow nasal cannula. The patient responded to treatment and was discharged on day 7. Climbers should be aware of the risk of HAPE when climbing Mount Fuji.

Objective Switching from one anti-calcitonin gene-related peptide monoclonal antibody (CGRP mAb) to another can be beneficial for treating patients with migraine who do not respond well to the first CGRP mAb. However, detailed and long-term follow-up reports of both efficacy and safety remain insufficient. We conducted a case-series analysis of patients with migraine who switched from galcanezumab to erenumab, both belonging to the class of CGRP mAbs. Methods We conducted a single-center retrospective real-world study. Patients with migraine who first received galcanezumab for ≥3 months and then switched to erenumab at Keio University Hospital were enrolled to investigate changes in monthly migraine days (MMD), response rate, and adverse effects (e.g., injection-site reactions). Additionally, we performed a narrative review of the literature on switching CGRP mAbs. Results Among the nine patients enrolled, the 50% response rate for MMD was 33% at 3 months after switching. Two patients (22%) initially responded at the 3-month assessment, but later reverted to baseline MMD levels. Switching from galcanezumab to erenumab increased the frequency of constipation, which was typically managed using laxatives. Participants who experienced injection-site reactions tended to exhibit similar reactions regardless of the type of CGRP mAb used. Five patients (56%) demonstrated an improvement in satisfaction after erenumab initiation at least once. A literature review revealed that the characteristics of the cohorts varied among studies. Conclusions Switching from galcanezumab to erenumab was effective in some patients, while it was associated with some tolerable side effects, and it improved patient satisfaction in approximately half of the patients, despite interindividual diversity in responses and fluctuating responses after switching, which warrants further investigation.

We encountered a family with hereditary renal failure, renal medullary cysts, pancreatic hypoplasia, hypomagnesemia, liver enzyme abnormalities, and diabetes mellitus (DM). We identified a novel heterozygous variant of HNF1B (NM_000458.4:c.791dup, p.L264Ffs*30) using whole-exome sequencing of genomic DNA samples from this family. This variant is located in the DNA-binding domain of the HNF1B protein and produces a truncated protein with a de novo sequence, suggesting that this variant changes HNF1B binding to genomic DNA or causes nonsense-mediated mRNA decay. Based on the phenotypes and identified gene variants, this family suffers from autosomal dominant tubulointerstitial kidney disease caused by this HNF1B variant.

A 69-year-old woman with left-sided breast cancer developed elevated creatine kinase levels and muscle weakness in her extremities after treatment with pembrolizumab. The patient was diagnosed with immune checkpoint inhibitor (ICI)-related myositis. Although the patient had no symptoms of dysphagia, we evaluated her swallowing function because esophageal dysfunction is a known complication of idiopathic inflammatory myopathy. A videofluoroscopic swallowing study detected barium residues in the lower esophagus. Furthermore, high-resolution manometry showed impaired upper esophageal sphincter opening and absence of esophageal peristalsis, which improved partially after immunotherapy. These findings suggest that esophageal dysfunction may be an unrecognized complication of ICI-related myositis.

Methotrexate-associated lymphoproliferative disorder (MTX-LPD) constitutes a subset of lymphoid proliferations and lymphomas that are associated with immune deficiency and dysregulation. The clinical management of MTX-LPDs is contingent on their histopathological subtypes. Polatuzumab vedotin is a novel therapeutic approach that is particularly beneficial for selecting patients with previously untreated diffuse large B-cell lymphoma (DLBCL); however, DLBCL-type MTX-LPD is still treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) because of the exclusion of MTX-LPD from clinical trials. We recently encountered a case of DLBCL-type MTX-LPD with parathyroid hormone-related protein-C (PTHrP)-mediated hypercalcemia that was managed with polatuzumab vedotin, rituximab, cyclophosphamide, doxorubicin, and prednisone (Pola-R-CHP). We herein report our experience to encourage hematologists to explore the safe and effective use of Pola-R-CHP under such conditions.