Autoimmune hepatitis (AIH) is thought to be associated with various genetic and immunological abnormalities. Concerning the pathogenesis of AIH, increasing attention has been paid to genome-wide association studies, toll-like receptors and Treg/Th17 balance. For Japanese patients with AIH, novel diagnostic guidelines have been proposed in view of the differential clinical features between Japanese and Caucasian patients. However, the diagnosis of some patients in acute hepatitis phase is not easy. Histologically, centrilobular necrosis without portal inflammation is particularly characteristic in the acute hepatitis phase. Some patients become resistant to steroid therapy and have a very poor prognosis once they progress to acute hepatic failure. Therefore, additional revision of the current diagnostic criteria, including severity grading, will be needed in the future.

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Objective Upgrading to biventricular (BiV) pacing benefits heart failure patients with right ventricular (RV) apical pacing. However, the impact of switching from RV apical pacing to BiV pacing on the left ventricular (LV) function accompanied by changes in the QRS duration remains unknown. We aimed to investigate the effects of BiV pacing in heart failure patients under RV apical pacing.
Methods In 37 patients with heart failure (LV ejection fraction: 22±9%), the maximum rate of LV pressure rise (LV dP/dt_max) and time constant of LV relaxation (tau) were determined in order to assess LV contractility and diastolic relaxation, respectively, under RV apical pacing and BiV pacing. Switching from RV pacing to BiV pacing, the QRS duration was shortened from 209±42 to 162±28 ms (p<0.001) and the LV dP/dt_max values were increased in all patients (+18.4±11.3%, p<0.001), whereas the LV tau values varied (-1.5±13.0%, p=0.723). Shortening of the QRS duration correlated with the increase in LV dP/dt_max (r=-0.689, p<0.001); however, it was not closely associated with the changes in LV tau.
Conclusion Switching from RV apical pacing to BiV pacing improves the LV contractile function in proportion to the degree of QRS shortening. BiV pacing is recommended in patients with systolic heart failure and a prolonged RV-paced QRS duration.

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Objective The objective of this study was to assess whether the distribution of pneumococcal capsular types has been changed, while also providing basic data on changes in the distribution after the introduction of Pneumococcal conjugated vaccine (PCV)13 in adult medical practice.
Methods We analyzed 431 Streptococcus pneumoniae strains (200 in 2006 and 231 in 2012) that had been isolated from respiratory infection specimens from adult patients. Capsular typing was performed by the Quellung reaction and multiplex polymerase chain reaction.
Results A comparison of the 2006 and 2012 strains revealed that the number and proportion of strains by serotype increased from 30 (15%) to 46 (20%) for serotype 3, from 4 (2%) to 14 (6%) for serotype 6A, and from 4 (2%) to 13 (6%) for serotype 6C, whereas the number and proportion of strains by serotype decreased from 8 (4%) to 0 (0%) for serotype 4 and from 24 (12%) to 17 (7%) for serotype 6B. From 2006 to 2012, the coverage rate significantly decreased from 39 to 28.1% for PCV7 (p=0.017).
Conclusion Our study showed a decrease in the vaccine coverage of PCV7. However, PCV13 covered serotypes 3 and 6A, which are prevalent, as well as penicillin-resistant S. pneumoniae strains. At present, PCV13 in adult clinical practice seems to be highly significant. However, there is a possibility that the distribution has changed, and careful screening should be continued in the future.

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A 66-year-old Japanese man consulted our institution due to paroxysmal and repetitive bouts of fever and abdominal pain that had persisted for more than one week. Capsule and double-balloon endoscopy (DBE) showed petal-shaped mucosal redness with white hemming in the jejunum and ileum, and histopathology of the biopsy specimens revealed villous atrophy and cryptitis with extensive severe neutrophil infiltration. A genetic examination disclosed compound heterozygous MEFV mutations (E84K, P369S), and familial Mediterranean fever was diagnosed. Treatment with colchicine and infliximab was very effective in inducing the complete disappearance of symptoms and normalization of the endoscopic findings. To the best of our knowledge, this is the first report to describe the findings of small intestinal endoscopic images obtained using capsule and DBE.

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A man in his thirties visited our hospital for an evaluation of a 12×10-mm pancreatic solid tumor that was accidentally detected on computed tomography performed for follow-up of familial adenomatous polyposis (FAP). We diagnosed the patient with a solid pseudopapillary neoplasm (SPN) based on endoscopic ultrasound-guided fine-needle aspiration, and he underwent pancreaticoduodenectomy. Small SPN tumors appear as solid tumors, without typical features of SPN, making the definitive diagnosis more difficult. The genetic background of FAP patients can predispose them to SPN, and imaging of the pancreas should be performed at prescribed intervals in FAP patients.

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Primary squamous cell carcinoma (SCC) of the pancreas is extremely rare. We herein report two such cases. Aside from various morphological characteristics, the clinical presentation of pancreatic SCC is the same as that of adenocarcinoma. The treatment is controversial, and the prognosis is poor.

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An 83-year-old man admitted for left hand pain due to a large necrotic ulcer presented with many sites of erythema on his trunk. Computed tomography revealed multiple poorly marginated masses in the liver; however, no malignant cells were detected on a biopsy of several skin lesions. He died on the 47th hospital day, and autopsy was subsequently performed, showing multiple nodules in the liver. The histological findings revealed clusters of malignant melanoma cells that had diffusely infiltrated the liver parenchyma. No tumor cells were detected in the skin lesions or lymph nodes. Immunohistochemically, the patient was diagnosed to have metastasis from malignant melanoma of unknown origin.

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Myxoma induces the onset of paraneoplastic syndromes by excreting various humoral mediators and is therefore known to present with diverse symptoms. A 40-year-old woman was admitted to our hospital for the treatment of an esophageal ulcer, the cause of which could not be identified on various examinations. Notably, a left atrial tumor was incidentally found on chest enhanced computed tomography. The esophageal ulcer, which was intractable to conventional therapy, improved with the administration of 5-aminosalicylate, a drug known to inhibit IL-1β. This inhibitory action effectively suppressed the development of myxoma-induced paraneoplastic syndrome.

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A 20-year-old woman was admitted to our hospital with an elevated serum creatinine level of 1.61 mg/dL and a normal C-reactive protein level of less than 0.1 mg/dL. Her myeloperoxidase anti-neutrophil cytoplasmic antibody (ANCA) titer was slightly increased at 9.2 U/mL; a kidney biopsy revealed that 23 of 32 glomeruli had crescents. The expression of pentraxin 3 was detected in her kidney and her plasma pentraxin 3 level was elevated at 63.53 ng/mL. Plasma pentraxin 3 levels may be an activity marker for ANCA-associated glomerulonephritis, particularly when serum C-reactive protein levels are within the normal limits.

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The onset of hyperammonemia due to the flow of ammonia-rich portal vein blood through a portal-systemic shunt causes a type of encephalopathy known as chronic portal-systemic shunt encephalopathy (CPSE). We herein report two cases of CPSE that presented with opposite changes in the blood ammonia concentrations during hemodialysis. It is curious that the encephalopathy was ameliorated by hemodialysis in case 1, but not case 2. Therefore, it is necessary to recognize CPSE and assess the blood ammonia concentrations in dialysis patients who develop a disturbance of consciousness, even if the serum transaminase level is normal.

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A 64-year-old man presented with diplopia, muscle weakness, a pulmonary nodule and mediastinal widening on a chest radiograph. He was diagnosed with clinical stage IIIA (T2aN2M0) lung cancer. His neurological symptoms worsened following the initiation of thoracic radiation therapy (60 Gy) and chemotherapy. A diagnosis of myasthenia gravis (MG) was confirmed with a repetitive nerve stimulation test that showed a waning pattern, and a positive edrophonium test, although neither anti-acetylcholine receptor antibodies nor anti-muscle-specific tyrosine kinase antibodies were detected. The ptosis and limb muscle weakness improved with prednisolone and acetylcholinesterase inhibitor treatment, and a partial response of the lung cancer to chemoradiotherapy was obtained. However, the ptosis and limb muscle weakness worsened again following a recurrence of the lung cancer. The herein described case, in which lung cancer and MG occurred and recurred simultaneously, suggests that MG can develop as a paraneoplastic syndrome of lung cancer.

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A 71-year-old man diagnosed with lung cancer in the right lower lobe with invasion to the middle lobe underwent right lower and middle lobectomy with mediastinal lymph node dissection. The cancer was pathologically diagnosed as stage IIB (pT3N0M0) with combined squamous cell carcinoma and an atypical carcinoid tumour. To the best of our knowledge, this is the first report of a combined atypical carcinoid tumour and non-small cell lung cancer. This case further expands the histological spectrum of combined neuroendocrine tumours.

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Lung metastasis showing radiographic findings of air-space consolidation is considered to be rare. This report describes the case of a man with progressive left lower lobe air-space consolidation with a history of hepatocellular carcinoma. The pulmonary lesion was initially suspected to be infection and later clinically diagnosed as primary adenocarcinoma of the lung. Although the patient was treated with systemic chemotherapy, the disease progressed very rapidly. A postmortem examination revealed that the alveolar spaces were filled with neoplastic cells subsequently proven to be metastases of combined hepatocellular and cholangiocarcinoma.

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A 68-year-old man was referred to our hospital due to a high fever and pancytopenia. Neither tumors nor infectious lesions were detected. Hemophagocytosis was observed on the bone marrow (BM) smear, although without abnormal cells. Prednisolone therapy was ineffective for the patient's high fever. Later on, we obtained the results of a BM biopsy indicating the presence of infiltration of atypical Reed-Sternberg cells, leading to a diagnosis of HIV-negative primary bone marrow Hodgkin lymphoma (PBMHL). However, the patient died of multiple organ failure before receiving chemotherapy. As the clinical course of PBMHL is rapid, physicians must keep in mind its possibility in similar cases.

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A 47-year-old woman with pancytopenia, excessive systemic lymphadenopathy and splenomegaly was referred to our hospital. The peripheral blood (PB) smear findings indicated neutropenia with lymphoid cells exhibiting hairy projections, while the histological findings of the cervical lymph node (LN) suggested hairy cell leukemia (HCL). In addition, the BRAF V600E mutation was detected, and the immunoglobulin gene rearrangement patterns were identical in both the cervical LN and PB specimens. Based on these findings, we diagnosed the patient with systemic lymphadenopathy due to HCL. This is the first report of a BRAF mutation detected in both the PB and LN at the onset of HCL.

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Intravascular large B-cell lymphoma (IVLBCL) is a rare extranodal lymphoma characterized by the presence of tumor cells within blood vessels, and it is considered to be a subtype of diffuse large B-cell lymphoma. We report a case of IVLBCL presenting as progressive hypoxemia. In this case, a definitive diagnosis could not be achieved by repeated transbronchial lung biopsy, a bone marrow biopsy, and a random skin biopsy, and the ultimate diagnosis was made on the basis of a pulmonary microvascular cytology (PMC) examination. Therefore, PMC is considered to be a useful strategy for the diagnosis of IVLBCL, particularly in this critically ill patient suffering from hypoxemia.

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Idiopathic granulomatous hypophysitis (GH) is a rare inflammatory disease of the pituitary gland. A 48-year-old woman was admitted to the hospital with an irregular menstrual cycle. MRI showed pituitary cystic lesion with sellar enlargement. The patient underwent endoscopic endonasal transsphenoidal surgical excision. A histopathological assessment revealed non-necrotizing granulomatous lesions and a diagnosis of GH was made. The rarity of GH and cystic form of the disease make it difficult to confirm the diagnosis until surgery. We herein report this rare case and provide a discussion of the MRI findings and relevant literature of GH.

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A 37-year-old woman gradually developed a gait disturbance due to sensory loss in the left lower extremity three years after being diagnosed with clinically isolated syndrome. Brain magnetic resonance imaging (MRI) demonstrated an incomplete ring-enhanced lesion with perifocal edema in the subcortex of the right parietal lobe. ¹¹C-methionine positron emission tomography (MET-PET) showed an insignificant uptake in the lesion. The patient was noninvasively diagnosed with tumefactive multiple sclerosis and treated with corticosteroids, and her neurological symptoms and MRI findings improved with treatment. The combination of MRI findings and insignificant uptake on MET-PET is useful for noninvasively differentiating tumefactive demyelinating lesions from brain tumors.

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Post-radiation lower motor neuron syndrome (PRLMNS) is a rare neurological complication of radiation therapy and its pathogenesis is unclear. We herein report a patient with PRLMNS who developed leg weakness 17 years after craniospinal radiation as a treatment for suprasellar germinoma. The electrophysiological evaluation, via a novel magnetic stimulation method, indicated a prolonged cauda equina conduction time, suggesting focal demyelination of the nerve roots in the cauda equina. The distribution of the denervated muscles detected by magnetic resonance imaging was consistent with patchy motor nerve root lesions. These results support the hypothesis that PRLMNS originates from ischemic radiculopathy in the cauda equina.

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Patients with rheumatoid arthritis (RA) treated with methotrexate (MTX) sometimes develop lymphoproliferative disease (LPD). MTX-associated LPD can affect nodal or extranodal sites, including the gastrointestinal tract, skin, lungs, kidneys and soft tissues, at almost equal frequency. However, it is very rare for MTX-associated LPD to manifest as multiple nodules in the lungs. We herein report the case of a RA patient who developed MTX-associated LPD with multiple pulmonary nodules during a 5-year course of MTX therapy.

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A 71-year-old man was admitted to our department due to arthralgia and renal dysfunction. A physical examination disclosed swelling of the right shoulder and left wrist joints. Laboratory tests showed elevated serum IgG4 and creatinine levels, and magnetic resonance imaging of the wrist revealed bone erosion and synovitis. In addition, fluorodeoxyglucose positron emission tomography showed uptake in the submandibular glands, pancreas, kidneys, and affected joints and a renal biopsy revealed tubulointerstitial nephritis with the infiltration of IgG4+ plasma cells. The patient was subsequently diagnosed with IgG4-related disease (IgG4-RD) and successfully treated with corticosteroid therapy. This case suggests that erosive arthritis may occur in patients with IgG4-RD.

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Cerebral toxoplasmosis is a rare disease predominantly found in immunocompromised hosts. However, cerebral toxoplasmosis has not been frequently described in association with the use of immunosuppressive medications. We herein report a case of cerebral toxoplasmosis in a 76-year-old Caucasian woman on methotrexate and infliximab for rheumatoid arthritis. The patient presented with right facial droop, slurred speech and difficulty walking. In addition to receiving methotrexate and infliximab and owning a cat, she had no other obvious risk factors. Imaging studies were not conclusive; however, brain biopsy confirmed the diagnosis. Serology was positive for anti-toxoplasma immunoglobulin G. Cerebral toxoplasmosis should be included in the differential diagnosis of patients under immunosuppressive medication who present with neurological manifestations.

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A 43-year-old man was referred to our hospital for an acute-onset fever and left flank pain. He had been previously diagnosed with lymphangioma, and abdominal computed tomography showed pararenal cysts with fat stranding around the left kidney, of which infection was subsequently confirmed on magnetic resonance imaging. Gram-negative spiral bacilli were isolated from two sets of blood cultures, and Helicobacter cinaedi was identified using 16S rRNA sequencing. The patient was successfully treated with ceftriaxone therapy without recurrence. A multilocus sequence typing analysis indicated the current H. cinaedi strain differed from previous strains isolated in Japan.

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A 47-year-old man with a fever was highly suspected of having influenza A infection since his wife and son who lived with him had been diagnosed with influenza A. Although repeated rapid tests with a nasopharyngeal swab showed negative findings, the patient developed bilateral pneumonia and reverse transcription polymerase chain reaction (PCR) for A (H1N1) pdm09 virus in the bronchoalveolar lavage fluid was positive. We therefore diagnosed him with primary influenza pneumonia and initiated treatment with peramivir plus corticosteroids, which rapidly improved his condition. During the influenza season, sample collection from the lower airway and PCR should be considered for the definitive diagnosis of primary influenza viral pneumonia.

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Although the polymerase chain reaction is effective for the diagnosis of extrapulmonary tuberculosis (EPTB), it is typically unavailable in resource-limited situations. In contrast, the loop-mediated isothermal amplification (LAMP) assay is a relatively cost-effective and accessible method. Additionally, when combined with the procedure for ultra-rapid extraction (PURE) kit, which enables simple DNA extraction, LAMP can detect Mycobacterium tuberculosis in sputum within 1.5 hours using a simple procedure. In this study, we investigated the utility of the PURE-LAMP technique to diagnose three cases of EPTB and showed that it may potentially be a valuable tool for the diagnosis of EPTB.

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Calcified pelvic masses are frequently detected on plain radiographs in the field of emergency radiology, particularly after trauma. While many of these findings are benign, a subset may be life-threatening if not accurately identified. The differential diagnosis depends on the location of the tumor and the patient's gender and history of trauma. Diagnostic possibilities include aneurysms, musculoskeletal and female pelvic malignancies and more benign entities, such as heterotopic ossification or phleboliths. Considering the possibility of these lesions will help to accurately identify relevant findings on radiographs and effectively select the appropriate treatment plan for patients presenting to the emergency room with pain.

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A 51-year-old man with nasopharyngeal carcinoma underwent chemoradiotherapy with cisplatin and 5-fluorouracil, followed by a left cervical lymphadenectomy. Distant metastatic disease was excluded using fluoro-deoxyglucose positron emission tomography. Seven months later, bone marrow metastasis and disseminated intravascular coagulation were diagnosed. The patient received weekly paclitaxel therapy and maintained a good performance status for seven months. During the treatment period, the patient developed no severe organ toxicity except for neutropenia. Weekly paclitaxel may therefore be considered as the treatment of choice in patients with advanced or recurrent nasopharyngeal carcinoma with bone marrow metastasis.

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