Objective 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 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.
Results 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.
Objective To survey the epidemiology of respiratory pathogens during the coronavirus disease 2019 (COVID-19) pandemic using multiplex polymerase chain reaction (PCR).
Methods Specimens were assayed using multiplex nested PCR.
Materials Specimens were obtained from outpatients who presented with symptoms of upper respiratory tract infection and asymptomatic outpatients who had contact with patients with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection at Tohoku Medical and Pharmaceutical University Hospital in Sendai, Japan, from November 1, 2020, to May 31, 2023. The analysis included multiple specimens collected from the same patients at different time-points. Data were collected from the electronic records after testing.
Results This study included 8,335 patients (4,311 men) with a median age of 59 years old, and 11,741 total specimens were collected. At least 1 positive SARS-CoV-2 result was obtained for 1,710 (14.6%) specimens. Furthermore, 15 pathogens were identified in the positive specimens, and rhinovirus/enterovirus was detected more frequently than other viruses. We identified a larger number of SARS-CoV-2-positive specimens in patients ≥10 years old. In contrast, in patients 0-9 years old, we identified a larger number of specimens positive for rhinovirus/enterovirus than for other viruses.
Conclusion In this study, we examined the epidemiology of circulating respiratory pathogens during the COVID-19 pandemic era.
Objective This longitudinal study aimed to clarify the changes in the medical treatment behavior of Japanese patients with chronic diseases during the early phase of the coronavirus disease 2019 (COVID-19) pandemic and examine the factors associated with disease worsening.
Methods Subjects with chronic diseases were selected from a panel survey that started at the beginning of the COVID-19 pandemic consists of 2,400 participants recruited via the Internet. Medical treatment behaviors (decrease in medical visit frequency, inability to take regular medications, and utilization of telephone/online medical care), psychological distress, and sociodemographic factors were evaluated at baseline (May 2020) and at the follow-up survey (February 2021). A worsening of chronic diseases was defined as those who answered "yes" to the question, "Has-the-condition-of-the-chronic-disease-worsened?". The factors related to the worsening of chronic diseases at follow-up were examined.
Results A total of 514 participants (mean age 61.6±12.9 years) were analyzed. The percentage of participants who reported decreasing medical visit frequency was 34% at the baseline and 16.5% at follow-up, and those who reported a worsening of chronic diseases was 5.1% and 5.1%, respectively. A worsening of chronic diseases at follow-up was significantly associated with a younger age, a decreased frequency of medical visits, unemployment, a history of smoking, and psychological distress.
Conclusions A decreased frequency of medical visits was observed among one-third of the participants with chronic disease in the early stage of the pandemic, and it reduced by half at follow-up. In the early stages of an emerging infectious disease pandemic, decreased regular hospital/clinic visits can lead to a worsening of chronic diseases. Those who had psychological distress, unemployment, and a history of smoking were vulnerable to a worsening chronic disease.
A 38-year-old Japanese man was diagnosed with extranodal marginal zone lymphoma of the mucosa-associated lymphoid tissue in the stomach (gastric MALT lymphoma). Fluorescence in situ hybridization analysis revealed the absence of t (11;18) (q21;q21) translocation but the presence of extra copies of MALT1, indicating tetrasomy 18. Helicobacter pylori eradication led to complete remission (CR). However, the gastric MALT lymphoma relapsed after 11 years old. This case underscores the need for long-term observation (>10 years) of patients with gastric MALT lymphoma. Further investigation is warranted to elucidate the correlation between trisomy/tetrasomy 18 and the recurrence propensity.
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.
A 57-year-old woman with a liver injury was referred to our hospital. She had a history of heavy alcohol consumption and had developed severe alcoholic hepatitis. Blood cultures revealed bacteremia caused by Bacillus cereus. The patient was treated with short-term steroid therapy for liver injury and vancomycin administration for B. cereus sepsis, which led to recovery. The findings in the present case suggest the need for empirical therapy with vancomycin in patients with severe alcoholic hepatitis and suspected B. cereus infection.
An 80-year-old man with jaundice and fatigue was referred to our hospital. A laboratory examination revealed increased levels of hepatobiliary enzymes, and CA19-9 levels increased to 29,512 U/mL. Based on the findings of imaging examination and laboratory data, the patient was diagnosed with acute cholecystitis and choledocholithiasis. The possibility of malignancy could not be ruled out because of the high levels of CA19-9. Antibiotic administration was commenced, and the common bile duct stone was endoscopically removed. One month after treatment, the CA19-9 level decreased to within the normal range. One year after treatment, imaging examinations did not reveal any malignancy.
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.
We herein report a 37-year-old man who experienced recurrence of metastatic cardiac rhabdomyosarcoma along with intractable ventricular tachycardia (VT) 7 years after resection of rhabdomyosarcoma in his right elbow. At 36 years old, he developed VT unresponsive to radiofrequency catheter ablation (RFCA). Initially, the cardiac tumor was not detected, but it gradually grew in size at the RFCA site. A surgical biopsy confirmed the diagnosis of metastatic cardiac rhabdomyosarcoma. Despite radiation therapy, cardiac tumor progression and VT instability could not be prevented. Ultimately, the patient died 27 months after the initial documentation of VT.
A gas exchange analysis with the cardiopulmonary exercise test is effective in discriminating non-cardiogenic components of limited exercise tolerance and is important for use in combination with the diastolic stress test. An 80-year-old woman with progressive exertional dyspnoea, hypertension, and untreated bronchial asthma was diagnosed with heart failure with a preserved ejection fraction by invasive testing. Diuretics were initiated, which resulted in partial symptom improvement. A subsequent non-invasive test revealed a reduced breathing reserve, suggesting exertional dyspnoea complications linked to lung disease. Bronchodilators were administered, which further improved the symptoms.
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.
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.
The most common cause of acute kidney injury (AKI) in multiple myeloma is light-chain cast nephropathy (LCCN), which consists of a light chain and Tamm-Horsfall protein (THP). We herein report a 46-year-old woman with hypercalcemia and AKI. A renal biopsy showed crystalline casts, which were consistent with lambda light chains but not THP. Hydration therapy and treatment to lower her serum calcium concentration were initiated immediately. She subsequently received bortezomib-based anti-myeloma therapy and recovered successfully. This was a rare case of LCCN, suggesting that hypercalcemia may play a role in the development of crystalline LCCN.
Renal medullary angiitis is characterized by interstitial hemorrhaging in the medulla with neutrophil infiltration. An 81-year-old man presented with a fever, kidney dysfunction, and purpura of the legs, which was diagnosed as leukocytoclastic vasculitis. Proteinase 3 antineutrophil cytoplasmic antibodies were weakly positive. A kidney biopsy showed severe tubulointerstitial hemorrhaging with neutrophilic infiltration in the perivascular areas surrounding the vasa recta in the medulla without crescent formation in the glomeruli. An immunofluorescence analysis was negative, and electron microscopy revealed no immune-dense deposits, ruling out immunoglobulin A vasculitis. Intravenous methylprednisolone for three days and plasma exchange followed by oral prednisolone improved his general condition.
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 coronavirus disease 2019 (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.
The patient was a 64-year-old man who had been receiving methotrexate (MTX) for rheumatoid arthritis for 8 years. Computed tomography (CT) obtained one month prior to admission revealed numerous enlarged lymph nodes. Lower leg edema appeared two weeks prior to admission. Severe proteinuria was confirmed, and the patient was admitted. A renal biopsy revealed minimal changes in glomeruli. CT on day 14 revealed shrinking lymph nodes, and the patient was diagnosed with Hodgkin lymphoma by a neck lymph node biopsy. This is the first report of secondary minimal change nephrotic syndrome caused by an MTX-associated lymphoproliferative disorder.
A 34-year-old Japanese man presented with blurred vision, headache, nausea, anemia, thrombocytopenia, and severe renal dysfunction. Thrombotic microangiopathy was initially suspected to have been caused by malignant hypertension. Antihypertensive medications did not improve his thrombocytopenia or renal dysfunction, and other diseases causing thrombotic microangiopathy were ruled out. Therefore, the patient was diagnosed with atypical hemolytic uremic syndrome. A renal biopsy revealed an overlap of thrombotic microangiopathy and C3 glomerulopathy. Genetic testing revealed c.848A>G (p.Asp283Gly), a missense heterozygous variant in the gene encoding complement factor I. Overlapping atypical hemolytic uremic syndrome and C3 glomerulopathy with complement factor I mutation is very rare, especially in Japan.
Early detection and appropriate management of treatment-related interstitial lung disease (ILD) are important in cancer treatment. We established an algorithm for quantifying fine crackles using machine learning and reported that the fine crackle quantitative value (FCQV) calculated by this algorithm was more sensitive than chest radiography for detecting interstitial changes. Using this algorithm, we periodically analyzed respiratory sounds in two patients with lung cancer who developed treatment-related ILDs and found that the FCQV was elevated before the diagnosis of ILD. These cases may indicate the usefulness of the FCQV in the early diagnosis of treatment-related ILDs.
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 pre-existing chronic fibrosis. We discuss how to pre-evaluate ILD deterioration risks and monitor TKI-induced lung toxicity during treatment.
A 71-year-old woman was treated with osimertinib for stage IV adenocarcinoma with epidermal growth factor receptor (EGFR) mutations. Treatment led to improvements in the primary tumor, multiple lung metastases, and multiple bone metastases. However, nine months later, she presented with marked liver dysfunction and jaundice. Chest and abdominal computed tomography did not show abnormal findings in the liver parenchyma or biliary system. However, blood tests were positive for hepatitis B surface antigen and hepatitis B virus DNA, suggesting hepatitis B virus reactivation. The patient died of liver failure despite treatment with steroids and antiviral drugs.
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.
Neuronal ceroid lipofuscinosis type 2 (CLN2) is an autosomal recessive lysosomal disease caused by decreased activity of the enzyme tripeptidyl peptidase 1 (TPP1) due to pathogenic variants in the TPP1 gene. Cerliponase alfa, a recombinant proenzyme form of TPP1, has shown efficacy in preventing motor and language function decline in early-stage CLN2. However, the safety and effects of this therapy in advanced-stage CLN2 are unclear. We herein report a case of intraventricular cerliponase alfa treatment for over a year in a patient with advanced-stage CLN2. The results suggest the safety and potential efficacy of treatment at an advanced stage of CLN2.
We herein report a 12-year-old boy who presented with a fever, erythematous rash on the cheeks, back pain, and dysphagia. Blood tests revealed increased creatine kinase levels, and muscle ultrasonography (MUS) revealed characteristic fascial thickening in the lumbar paraspinal muscles, where myalgia was prominent. Sarcoplasmic expression of myxovirus-resistant protein A on a muscle biopsy and the presence of anti-nuclear matrix protein 2 (NXP2) antibodies confirmed the diagnosis of dermatomyositis. Prednisolone and intravenous immunoglobulin therapy improved the clinical and laboratory parameters as well as fascial thickening. MUS is useful for evaluating fasciitis associated with anti-NXP2 autoantibodies and monitoring therapeutic efficacy.
Although the frequency of community-acquired infections caused by Klebsiella pneumoniae subsp. ozaenae (K. ozaenae) is low, they are often detected in sputum specimens. In addition, lung abscesses, necrotizing pneumonia, and urinary tract infections caused by K. ozaenae have also been reported. We herein report the first detection of K. ozaenae as an etiological agent of bacterial meningitis in Japan. Cases of K. ozaenae meningitis complicated by diabetes mellitus and sinusitis have been reported elsewhere. When Klebsiella pneumoniae is detected in such cases, it is important to use other detection methods in addition to mass spectrometry for correct identification.
Human brucellosis, one of the most common zoonoses worldwide, is rare in Japan. Brucella canis is the specific pathogen of human brucellosis carried by dogs. According to an epidemiological study of B. canis infection in Japan, B. canis is the specific pathogen of human brucellosis in dogs. We herein report a rare case of meningoencephalomyelitis caused by B. canis in a 68-year-old Japanese man. Neurobrucellosis was diagnosed based on a serum tube agglutination test and abnormal cerebrospinal fluid findings. The patient was started on targeted treatment with a combination of doxycycline and streptomycin. Although extremely rare, neurobrucellosis should be considered in patients with a fever of unknown origin and unexplained neurological symptoms.
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.