Objective This follow-up survey report describes medication adherence and patient preferences, beliefs, and expectations of maintenance hemodialysis treatment in Japan.

Methods This patient-reported questionnaire-based survey was conducted in six regions in Japan from September 2016 to November 2016.

Patients The questionnaire was provided to 700 patients (50-79 years old) on maintenance hemodialysis for >3 years who were members of the Japan Association of Kidney Disease Patients. Patients were randomly selected by a stratified sampling method based on patient distribution observed from the Japanese Society for Dialysis Therapy Renal Data Registry.

Results A total of 524 (74.9%) complete patient questionnaires were evaluated; the mean (SD) age was 66.6 (7.2) years (men, 63.4%) with a dialysis vintage of 16.9 (9.1) years. Adherence was high for all types of medications: between 76.7% for phosphate binders and 95.7% for antidiabetic medications. The most common reason for a missed dose was forgetting to take medication [52.5% (117/223)]. Patient preference for oral medication was as low as 0.9% (1/110), 9% (31/345), and 2.9% (2/69) for patients who felt mental burden, felt no mental burden, and neither, respectively, with their current treatment regimen. In addition, 37.8% (198/524) of patients responded that the elimination of 1 medication (1 tablet) would reduce their mental burden.

Conclusion The results of this survey show that overall medication adherence is high in Japanese patients on maintenance hemodialysis. While many patients perceive an absence of mental burden, they still prefer to avoid oral medication when possible.

Objective Rapidly progressive interstitial lung disease (RP-ILD) with anti-melanoma differentiation-associated protein 5 (MDA5) antibody potentially presents with a fatal clinical course and requires early intensive treatment. Recently, perilobular opacity was reported to pathologically correspond to the acute phase of diffuse alveolar damage in RP-ILD with anti-MDA5 antibody. We aimed to investigate whether or not perilobular opacity was a common radiological finding in RP-ILD patients with anti-MDA5 antibody.

Methods We conducted a retrospective review of the medical records of eight consecutive patients with RP-ILD with anti-MDA5 antibody. The clinical features and radiological findings of follow-up computed tomography (CT) during the course of their disease were evaluated.

Results Among eight RP-ILD patients with anti-MDA-5 antibody, six showed perilobular opacity in the lower lobes, and the remaining two had only consolidation on high-resolution CT. Of note, the perilobular opacity in all six patients thickened and progressed to consolidation with a loss of lung volume in a short period. Despite intensive treatment, 6 patients (75%) died within 100 days after the first visit. Notably, the two patients with consolidation presented with a very rapid clinical course and died in 13 days each. In the two survivors, the perilobular opacity and consolidation recovered with improvement in the loss of lung volume.

Conclusion Rapidly progressive perilobular opacity that thickens and progresses to consolidation is characteristic of RP-ILD with anti-MDA5 antibody. Chest physicians should immediately check the status of anti-MDA-5 antibody in order to initiate early aggressive therapy in RP-ILD patients with rapidly progressive perilobular opacity.

Objective TAFRO syndrome is rare, and its underlying mechanisms currently remain unknown. Furthermore, standard therapeutic strategies have yet to be established. One of the hallmarks of TAFRO is pathological hypercytokinemia, which involves vascular endothelial growth factor (VEGF). A correlation has been reported between elevated VEGF and TSH levels in patients with hypothyroidism. Although hypothyroidism is a common endocrine abnormality, its clinical significance in TAFRO syndrome remains unclear.

Methods and Patients We investigated six patients diagnosed with TAFRO syndrome and examined their thyroid function in detail to obtain a deeper understanding of its relationship with cytokines and the manifestations of thyroid abnormalities as well as their clinical significance in TAFRO syndrome.

Results Five patients had subclinical hypothyroidism, while one had clinical hypothyroidism. Plasma VEGF levels were elevated in all patients, with a mean level of 256 pg/mL. Treatment with thyroxine supplements and immunotherapy or chemotherapy improved the symptoms of TAFRO syndrome without recurrence as well as increased the VEGF levels in three patients.

Conclusion The present results suggest that subclinical hypothyroidism may be a potential factor in the pathogenesis and symptomatology of TAFRO syndrome with VEGF elevation.

Objective The importance of antimicrobial stewardship is increasingly highlighted in this age of antimicrobial resistance. A better comprehension of adverse drug events (ADEs) can promote the appropriate use of antibiotics. We aimed to quantify the incidence of ADEs associated with broad-spectrum systemic antibiotics in a hospital setting.

Methods We conducted a six-month prospective, observational study at Osaka University Hospital to describe the incidence of ADEs in patients hospitalized in general wards undergoing treatment with broad-spectrum antibiotics [carbapenems, piperacillin/tazobactam (PIPC/TAZ), and anti-methicillin-resistant Staphylococcus aureus agents]. The occurrence of ADE was defined as any cardiac, gastrointestinal, hepatobiliary, renal, neurologic, hematologic, dermatologic, or musculoskeletal manifestation after 48 hours or more of systemic antibiotic therapy.

Results The 3 most frequently prescribed antibiotics were PIPC/TAZ (242 cases), meropenem (181 cases), and vancomycin (92 cases). Of 689 patients, 118 (17.1%) experienced ADEs, including gastrointestinal (6.4%), hepatobiliary (4.2%), dermatologic (2.5%), and renal (2.3%) manifestations. Patients treated with PIPC/TAZ, meropenem, doripenem, vancomycin, daptomycin, and teicoplanin developed ADEs at rates of 20.7%, 16.0%, 15.4%, 19.6%, 11.8%, and 10.9%, respectively.

Conclusion Our study provides a quantitative value for the incidence of ADEs associated with broad-spectrum antibiotics in clinical practice. To optimize patient safety, clinicians need to be aware of the risks associated with antibiotic administration.

Objective In the management of patients with suspected acute drug poisoning, a screening test using the patient's urine is usually performed. The Triage DOA^® and INSTANT-VIEW M-1^® kits are two commonly used point-of-care screening kits in Japan. However, the relationship between the results of these screening kits and the blood concentration of the poisoning drug is not clear. In this study, we evaluated which kit is more useful for acute drug poisoning screening based on a comparison of their results with the results of a serum drug analysis.

Methods This prospective cross-sectional study investigated all patients with acute drug poisoning admitted to a general hospital in Tokyo, Japan, over a nine-month period. The Triage DOA^® and INSTANT-VIEW M-1^® screening kits were used, and a qualitative serum analysis was conducted simultaneously in all cases. We compared the kits for use in screening patients with acute drug poisoning and evaluated the utility of the kits.

Results For the 117 patients enrolled in this study, the 2 kits showed different sensitivities to benzodiazepines (Triage^®, 78.6%; INSTANT-VIEW^®, 90.5%). Both kits showed high sensitivity to barbiturates (Triage^®, 87.0%; INSTANT-VIEW^®, 91.3%) but low sensitivity to tricyclic antidepressants (Triage^®, 25.0%; INSTANT-VIEW^®, 45.8%).

Conclusion Because the sensitivity varies depending on the kind of drug, it is difficult to discuss the superiority of these kits. However, this study compared the results of two types of urinary drug screening kits with the results of qualitative analysis of drugs in serum as a gold standard, providing important reference data.

Synchronous early gastric cancer/neuroendocrine tumor (NET) associated with autoimmune gastritis is rare, and its endoscopic and pathological features remain poorly described. Screening esophagogastroduodenoscopy performed on a 71-year-old man revealed a whitish, superficial elevated lesion and a submucosal tumor with redness that appeared slightly centrally depressed. Endoscopic submucosal dissection (ESD) allowed these lesions to be resected with negative margins, and they were diagnosed as tubular adenocarcinoma, well-differentiated type (tub1), pT1a (M) and NET G1, pT1b (SM). To our knowledge, this is the first report describing the endoscopic and pathological findings of synchronous early gastric cancer/NET that was amenable to complete resection with ESD.

An 83-year-old man with a history of carbon ion radiotherapy for hepatocellular carcinoma nine years ago presented to a primary care hospital with a fever and abdominal pain. He underwent computed tomography, which revealed the rupture of a hepatic pseudoaneurysm close to the fiducial marker for carbon ion radiotherapy and bleeding into the bile duct. He was successfully treated with transcatheter arterial embolization. Thereafter, re-rupture occurred from a site proximal to the first rupture, and this was treated similarly. It is necessary to be alert for not only tumor recurrence but also pseudoaneurysm occurrence after carbon ion radiotherapy.

A 26-year-old woman complained of upper abdominal pain. Computed tomography (CT) showed acute pancreatitis, a left adrenal tumor and solitary right pulmonary metastasis. She underwent left adrenalectomy; the adrenal tumor was diagnosed as adrenocortical carcinoma (ACC). When preparing to resect the pulmonary metastasis, she suffered a second acute pancreatic attack. Magnetic resonance cholangiopancreatography (MRCP) showed that the proximal main pancreatic duct (MPD) was dilated, and the distal MPD was diminished; however, no pancreatic tumor was observed on CT or MRCP. Endoscopic ultrasonography revealed a solitary pancreatic mass, which was diagnosed as pancreatic metastasis from ACC by endoscopic ultrasonography-guided fine-needle aspiration.

Crizotinib has been approved for patients with advanced lung adenocarcinoma harboring rearrangements of the c-ROS-1 (ROS1) and anaplastic lymphoma kinase (ALK) genes. We report a patient with ROS1-rearranged lung adenocarcinoma who developed a crizotinib-induced mixed/cholestatic type of liver injury. The patient discontinued crizotinib after 34 days due to liver toxicity. Twenty-four days later, when transaminases and C reactive protein (CRP) were normalized, crizotinib was resumed using an oral desensitization method. The patient was successfully treated for manageable recurrence of liver injury and has been able to continue the treatment.

We experienced two cases of hepatitis C virus (HCV) eradication failure in patients with a history of non-responsiveness to previous treatments with direct-acting antiviral agents (DAAs) who were subsequently treated with the combination of glecaprevir and pibrentasvir (GLE/PIB). Direct sequencing at commencement of GLE/PIB therapy showed non-structural protein (NS) 5A-P32 deletion in the first patient and NS5A-R30E/Q54H/A92K in the second patient (both genotype 1b). The common point was that L31/Y93 was double wild-type, and the IL28B polymorphism was non-TT type. Even when L31/Y93 is double wild-type, other NS5A mutations may affect the DAA re-treatment outcome. We analyzed the transition of amino acid mutations at NS5A by ultra-deep sequencing.

A 44-year-old Japanese woman was admitted to our hospital with fatigue and an altered liver function. She had been receiving atorvastatin treatment for 10 months. Although no jaundice was seen, the patient's serum alkaline phosphatase and γ-glutamyl transpeptidase levels were markedly elevated. Based on the results of a drug-induced lymphocyte-stimulation test, her liver disease was diagnosed as atorvastatin-induced hepatic injury. Subsequently, anti-mitochondrial antibodies (AMAs) were detected in her serum; however, a liver biopsy specimen did not show the characteristic features of primary biliary cholangitis. We herein report the detection of AMAs accompanied by drug-induced hepatic injury caused by atorvastatin.

Thyroglobulin (TG) gene mutations cause thyroid dyshormonogenesis, which is typically associated with a congenital goiter. We herein report the case of a 64-year-old man with congenital primary hypothyroidism who had a normal-sized thyroid gland on levothyroxine replacement. He had short stature (−3.1 standard deviations) and mild intellectual impairment. Thyroid autoantibodies were all negative, and the serum TG levels were undetectable. Eventually, he was found to have the novel homozygous nonsense mutation p.K1374* in the TG gene. The possibility of TG mutation should be considered for patients with congenital primary hypothyroidism and a very low serum TG level, regardless of the thyroid size.

Mycobacterium abscessus subspecies abscessus is major subspecies in the M. abscessus complex and is usually refractory to standard antibiotherapy. Genetic tracing of erm (41) T28 is a mechanism for monitoring macrolide resistance. We treated a patient with a pulmonary infection caused by M. abscessus subsp. abscessus with the erm (41) T28 polymorphism, which was susceptible to clarithromycin, and his clinical treatment course was good. The identification of the M. abscessus complex genotype is important, but clinical confirmation of clarithromycin susceptibility is also needed to plan individual treatment strategies.

We herein report a 45-year-old woman with lung adenocarcinoma stage IV (cT4N3M1a). She was treated with pemetrexed (PEM) monotherapy following four cycles of first-line treatment with carboplatin, paclitaxel, and veliparib. After three cycles of PEM treatment, she presented with dyspnea, and chest computed tomography showed diffuse ground-glass attenuation (GGA), suggesting hypersensitivity pneumonia (HP). Bronchoalveolar lavage revealed a marked increase in lymphocytes (90.5%), and a transbronchial lung biopsy confirmed lymphocytic alveolitis with granuloma. Because her symptoms and diffuse GGA were spontaneously resolved with PEM discontinuation alone, PEM-induced interstitial lung disease was diagnosed. Chest physicians should be aware that PEM can induce HP-type interstitial lung disease.

Dyskeratosis congenita (DC) is occasionally complicated in patients with familial interstitial pneumonia (FIP). However, there have been no reports of FIP patients with DC that develop acute exacerbation (AE). We herein report a FIP patient with DC that showed AE of FIP after influenza virus B infection. Although DC is a rare disease in clinical practice, physicians should keep in mind that FIP combined with DC has the potential to cause AE.

A 69-year-old Japanese woman presented with mild muscle weakness of the neck and symmetrical proximal parts of the upper and lower limbs. Laboratory tests, needle electromyography, and a muscle biopsy revealed inflammatory myopathy with an apparent clinical classification of polymyositis and positive findings for anti-PM/Scl-75 antibody. This antibody is rare among Japanese populations, and most Japanese patients with the antibody are not classified with the inflammatory myopathy seen in polymyositis. The muscle biopsy also showed marked necrotic and regenerative fibers. We need to collectively investigate patients with the potential to develop this disease, and to identify any unique characteristics for Asian populations, including Japanese.

We report the clinical features of a patient with hereditary transthyretin (ATTR) amyloidosis associated with a novel mutation (Y114S, p.Y134S). A 65-year-old Japanese man was admitted to our hospital after a 3-year history of progressive dyspnea on exertion. Five years previously, he presented dysesthesia in both hands caused by carpal tunnel syndrome. A genetic analysis revealed a base pair substitution of adenine to cytosine in the second codon of exon 4, residue 114, in the TTR gene (c.401A>C). The clinical characteristics were progressive cardiomyopathy with a poor vital prognosis, late onset, sporadic case, bilateral carpal tunnel syndrome, hypothyroidism, and small fiber neuropathy.

Temporal muscle abscess in children usually occurs from acute otitis media, and rapid progression and concomitant infectious disease often make it easy to diagnose. We report a rare case of a nine-month-old infant who showed right temporal mass with no evidence of infection. Computed tomography showed an osteolytic round mass, and magnetic resonance imaging revealed heterogenous enhancement with a high apparent diffusion coefficient. Malignant tumor was first suspected, but an open biopsy revealed the swelling to be temporal muscle abscess. It should be noted that temporal abscess may mimic the features of a malignant tumor, and multiple examinations should be performed for an accurate diagnosis.

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system caused by reactivation of the JC virus under an immunosuppressed state. This condition carries a high risk of cryptococcal meningitis. We herein report a 65-year-old woman who simultaneously developed PML and cryptococcal meningitis and presented with bilateral sixth nerve palsy. She had been treated with methotrexate and infliximab for rheumatoid arthritis. Her symptoms improved with antifungal drug treatment and discontinuation of immunosuppression therapy. Although concurrent PML and cryptococcal meningitis is rare, it should be considered in immunosuppressed patients.

We herein report the successful management of a condition mimicking acquired laryngomalacia using conservative methods in an elderly man with a progressive neurological disorder. The patient developed stridor and was transferred to the intensive-care unit. Flexible laryngoscopy revealed a collapsed epiglottis during inspiration, as seen in acquired laryngomalacia, with mucinous material firmly adhered to the epiglottis. The stridor resolved after the removal of this material. Pathology revealed keratinized material, suggesting a collection of sputum or epithelial tissue. Thus, flexible laryngoscopy can differentiate the cause of airway obstruction and avoid unnecessary endotracheal intubation in patients with neurological disorders.

A 24-year-old Japanese man exhibited slowly progressive gait disturbance from childhood to young adulthood. Physical and physiological examinations showed the involvement of both upper and lower motor neurons, fulfilling the diagnostic criteria for amyotrophic lateral sclerosis (ALS). Mild cognitive impairment and subclinical sensory involvement were also observed. A genetic analysis revealed novel compound heterozygous mutations, c.767C>T (p.Thr256Ile) and c.800A>G (p.Asp267Gly), in the vaccinia-related kinase 1 gene (VRK1). This is the first report of a Japanese patient with a motor neuron disease phenotype caused by VRK1 mutations. This diagnosis should be considered in atypical cases of juvenile-onset and slowly progressive types of motor neuron disease.

A middle-aged man presented with a fever, arthralgia, gastrointestinal symptoms, headache, and rash. After two weeks, the patient suddenly complained of severe abdominal pain, and computed tomography revealed aneurysms in the hepatic and splenic arteries, which increased in size progressively. Given the elevated levels of inflammatory markers and orchitis, polyarteritis nodosa (PN) was initially suspected. Catheter embolization for the ruptured hepatic aneurysm and splenectomy for the large splenic ones were performed, and the pathological finding was consistent with segmental arterial mediolysis (SAM). Changes in inflammatory marker levels and aneurysmal size are also informative to differentiate SAM from PN.

Along with the increase in consumption of raw animal meat, the prevalence of food poisoning is increasing. A 67-year-old Japanese man had eaten raw venison 4 hours prior to the beginning of vomiting. Many white cysts were discovered in the venison, with numerous bradyzoites being detected after the cysts were punctured. The presence of the Sarcocystis spp. 18S rRNA gene was detected by polymerase chain reaction, and Sarcocystis truncata was isolated from the venison. Sarcocystis truncata has not previously been identified in sika deer (Cervus nippon) in Japan. This is the first report of possible Sarcocystis truncata-induced food poisoning following consumption of venison.

A 64-year-old Japanese woman developed fatigue, dyspnea, and wheezing in July. Although she had been undergoing treatment for chronic obstructive pulmonary disease for six days, she was transferred to our hospital with delirium and diarrhea. On admission, she had an eschar of 3 mm in diameter on her anterior chest. Polymerase chain reaction of her blood and eschar specimens led to the diagnosis of severe fever with thrombocytopenia syndrome. Chest computed tomography showed ground-glass opacities, suggesting noncardiogenic pulmonary edema or viral pneumonia. This is the first case report of severe fever with thrombocytopenia syndrome beginning with dyspnea and wheezing.

A 37-year-old woman presented to our hospital with mild abdominal pain experienced for 2 months and hepatic nodules in segments 3 and 8. Peripheral blood eosinophilia was observed, and toxocariasis was serologically diagnosed. Seventeen days after the first imaging evaluation, a new lesion was found in segment 9 of the right lung, which was contiguous through the diaphragm to the hepatic nodule in segment 8. After treatment with albendazole, the liver and lung nodules disappeared. We suspect that larvae had directly invaded the lung from the liver, through the diaphragm.

Accidental hypothermia is defined as a core body temperature <35°C. Even with the use of multiple active rewarming methods, it has a high mortality rate. No standard treatment strategy for moderate or severe hypothermia in the absence of cardiac arrest has yet been established. We herein report three patients with severe or moderate accidental hypothermia who were treated by hemodialysis in the acute phase. This case report with a literature review describes the usefulness of hemodialysis for the treatment of moderate and severe accidental hypothermia without cardiac arrest.