Objective Little information is available on the relationship between the clinical course of ulcerative colitis (UC) and the outcomes of pregnancy and delivery in pregnant Japanese women. The aim of this retrospective study was to determine the factors that influence pregnancy and childbirth in middle-aged UC patients.

Methods We studied 53 pregnancies in 45 pregnant women with UC who delivered at our department. They included 41 pregnancies that started while in UC remission and 12 pregnancies that started in the UC active phase. The following factors were evaluated: 1) the clinical course of UC; 2) the frequency and details of abnormal pregnancy/abnormal delivery; and 3) the course of pregnancy/delivery. We compared the clinical features, course of UC, and details of treatment between women with a normal pregnancy/delivery and those with an abnormal delivery.

Results A comparison of the remission and acute groups showed lower clinical activity indices (CAIs) during pregnancy in the remission group and significantly higher rates of recurrence/exacerbation in the active group (75%) than in the remission group (7.3%). The respective CAIs in the first, second, and third trimesters were 3 and 6, 3 and 5, and 3 and 4, in the remission and active groups, respectively. Live infants were delivered in 51 (96%) pregnancies, with 7 (17%) abnormal pregnancies in the remission group and 4 (33.3%) in the active group (p>0.05). Abnormal delivery occurred in 16 of 53 (30.1%) pregnancies, and the rate was higher in the remission group than in the active group (p>0.05). In both groups, the most common abnormal event during pregnancy was delivery of low-birth-weight infants. Delivery was normal in 37 cases and abnormal in 16 cases. A multivariate analysis showed that a shorter UC disease duration (odds ratio=1.16) and higher CAI in the first trimester (odds ratio=1.49) were associated with an increased risk of abnormal pregnancy.

Conclusion Our findings demonstrated that the clinical course of UC, as evaluated by the CAI, during pregnancy influenced the outcome of pregnancy and delivery.

Objective We investigated the possible factors for predicting the future progression to hepatocellular carcinoma (HCC) from hypovascular nodules detected in the hepatobiliary phase of gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid-enhanced magnetic resonance imaging (Gd-EOB-DTPA-MRI).

Methods A total of 91 hypovascular nodules detected by Gd-EOB-DTPA-MRI in 28 patients without any past history of treatment for HCC were retrospectively examined. The nodules were categorized into those with and without HCC progression, then comparisons were made to identify any factors possibly related to a progression to HCC in each case. In addition, we performed a receiver operating characteristics (ROC) analysis to determine the cut-off value for the initial nodule size for predicting HCC progression within 12 months.

Results The observation period of the 28 patients was 1,172.6±95.6 (mean±standard error) days. The number of hypovascular nodules that changed to hypervascular ones was 15 (16.5%), and the cumulative incidence of hypervascular transformation was 7.1% at 12 months and 12.7% at 24 months. Of all 91 hypovascular nodules, 33 in 18 patients were diagnosed as HCC based on hypervascular transformation and/or size enlargement, while the remaining 58 did not progress to HCC. There was no significant difference regarding the background characteristics between the HCC progressed and non-progressed groups according to a multivariate analysis, or between the patients who had nodules that progressed to HCC and those with nodules that did not progress to HCC. Regarding HCC progression at 12 months, the area under the ROC (AUROC) had a level of 0.745 and showed that an initial nodule cut-off size of 9.5 mm (sensitivity, 57.9%; specificity, 87.3%) was predictive.

Conclusion In patients without a past HCC treatment history, it is difficult to determine whether hypovascular nodules have a high risk of progression to HCC based on background factors alone.

Objective Docosahexaenoic acid (DHA) and eicosapentaenoic acid (EPA) have been reported to have beneficial effects in patients with IgA nephropathy (IgAN). Although DHA and EPA have different mechanisms of action, no study to date has assessed their individual actions in patients with IgAN. This study therefore analyzed the effects administering DHA in addition to EPA for the treatment of IgAN.

Methods Twenty-one IgAN patients who were being treated with EPA (1,800 mg/day) were switched to EPA (1,860 mg/day) and DHA (1,500 mg/day). The changes in their clinical parameters from 6 months before to 6 months after switching treatment were analyzed.

Results The triglyceride levels did not change during treatment with EPA alone, but tended to decrease-although not to a statistically significant extent-after the switch. The patients' low-density-lipoprotein cholesterol, blood pressure, proteinuria, and hematuria levels were similar before and after switching. The estimated glomerular filtration rate (eGFR) tended to decrease during EPA therapy, but became stable after switching and the median %⊿eGFR changed from -7.354% during EPA therapy to +1.26% during the 6 months after switching to EPA and DHA therapy (p=0.00132), and renal the function remained stable for another 6 months. Moreover, the median %⊿eGFR during the 6 months after switching was significantly higher in comparison to IgAN patients who were treated with EPA alone as a control (-3.26%, p=0.0361). No clinical parameters were independently associated with a stable renal function without switching to DHA/EPA.

Conclusion The addition of DHA to EPA stabilized the renal function of IgAN patients, and it seemed that there were pleiotropic effects beyond the improvement of the clinical parameters.

Objective To retrospectively investigate the clinical manifestations of systemic amyloid light-chain (AL) amyloidosis in Japanese patients and the treatment strategy for the condition.

Methods We conducted a survey of Japanese AL amyloidosis patients, who were treated between January 1, 2012, and December 31, 2014.

Results A total of 741 AL amyloidosis patients were included in this study (436 men and 305 women; median age: 65 years old, range: 31-93). The most frequently affected organ was the kidneys (n=542), followed by the heart (n=252), gastrointestinal (GI) tract (n=164), autonomic nervous system (n=131), liver (n=71), and peripheral nervous system (n=71). Diagnostic findings were most commonly detected in the GI tract (upper GI tract: 350 cases, lower GI tract: 167 cases), followed by the bone marrow and kidneys. An abdominal fat-pad biopsy was only conducted in 128 patients. Autologous stem cell transplants (ASCTs) and bortezomib were used to treat 126 and 276 patients, respectively.

Conclusion The clinical features of Japanese patients with systemic AL amyloidosis are similar to those reported previously for cases in the US and Europe. Regarding treatment, a significant number of ASCTs were performed in Japan as well as in Western countries. Surprisingly, a marked number of patients received bortezomib as a treatment for AL amyloidosis.

Objective We explored the association between the total small vessel disease (SVD) score obtained with magnetic resonance imaging and risk factors and outcomes in the Japanese population.

Methods The presence of SVD features, including lacunes, cerebral microbleeds, white matter changes, and basal ganglia perivascular spaces on MRI, was summed to obtain a "total SVD score" (range 0-4). Ordinal and multinomial logistic regression analyses were performed to investigate the association of higher total SVD scores with vascular risk factors, the Mini-Mental State Examination (MMSE) score, and cerebral atrophy.

Results We included 1,451 neurologically healthy adults (mean age, 57.1 years; 47% male). A multivariate ordinal logistic regression analysis showed that the total SVD score was associated with aging, hypertension, blood pressure (BP), diabetes mellitus, MMSE score, and deep cerebral atrophy, but the equal slopes assumption between scores did not hold. A multivariate multinomial logistic regression analysis (total SVD score 0=reference) showed that aging, hypertension, and BP were positively associated with scores of 1, 2, or ≥3. These effects, presented as odds ratios (ORs), increased as the score increased and were strongest with a score of ≥3 [aging (per 10-year increment), OR 4.00, 95% confidence interval (CI) 2.47-6.46; hypertension, OR 5.68, 95% CI 2.52-12.80; systolic BP (per standard deviation increase), OR 1.96, 95% CI 1.41-2.74, respectively]. Diabetes mellitus and deep cerebral atrophy tended to be associated with the SVD scores. The MMSE score showed no consistent associations.

Conclusion The total SVD score may be a promising tool for indexing SVD, even in the Japanese population.

Objective Cases of Japanese spotted fever (JSF) are sometimes complicated by disseminated intravascular coagulation (DIC) with an abnormal liver function, resulting in unfavorable outcomes. The aim of the present study was to clarify the correlation between liver function test results and DIC scores.

Methods Twenty patients diagnosed with JSF between April 2010 and April 2014 were enrolled. Age, gender, disturbance of consciousness, body temperature, pulse rate, presence of diffuse erythema, eschar and swelling of lymph nodes, laboratory test results at the time of initial presentation such as blood cell count, C-reactive protein, liver function, renal function and blood coagulation and fibrinolysis, maximum Japanese Association for Acute Medicine (JAAM) DIC score during the course of JSF, treatment and the prognosis were retrospectively reviewed.

Results The median age of the patients (8 men, 12 women) was 68.3 years. There were significant differences in the alkaline phosphatase (ALP) and rothrombin time international normalized ratio (PT-INR) between the DIC and non-DIC groups using Mann-Whitney's U test. A multiple logistic regression analysis showed that the ALP and blood urea nitrogen (BUN) levels at the time of initial presentation were independent predictors of the occurrence of DIC.

Conclusion We should pay special attention to JSF patients showing high levels of ALP at the initial presentation, since such patients may have a higher likelihood of developing DIC over the course of JSF and unfavorable outcomes than those with lower levels.

A reddish depressed lesion was found in the corpus of the stomach of a 56-year-old man. Gastric biopsy showed no findings of mucosa-associated lymphoid tissue lymphoma, including lympho-epithelial lesions. A urea breath test, stool antigen test and serum IgG antibody to Helicobacter pylori test were negative. Magnifying endoscopy using narrow-band-imaging showed no malignant structures. Gastric biopsy specimens were subjected to immunohistochemistry and a polymerase chain reaction, which identified Helicobacter suis infection. Triple therapy with esomeprazole, metronidazole, and amoxicillin was administered for 10 days. Three months later, endoscopy showed the significant improvement of the lesion. H. suis infection should be considered in chronic gastritis patients without H. pylori infection.

A 46-year-old man visited the emergency department of our hospital with a 3-day history of anal pain, hemorrhaging, and a slight fever. He had previously been diagnosed with protein C deficiency and was prescribed dabigatran, a direct oral anticoagulant. Contrast-enhanced computed tomography showed severe rectal wall thickening with partial defect of enhancement. In addition, sigmoidoscopy revealed a dusky purplish swollen anorectal mucosa just above the dentate line. He was diagnosed with acute anorectal thrombophlebitis, and anticoagulant therapy with heparin was initiated. To our knowledge, this is the first case report of acute anorectal thrombophlebitis caused by protein C deficiency.

We herein report a 69-year-old man who underwent right nephrectomy 1 year previously to remove renal cell carcinoma (RCC). On our examinations, contrast-enhanced computed tomography revealed a tumor with intensive early enhancement near the cystic duct of the gallbladder. Endoscopic ultrasonography showed a low echoic mass in the cystic duct. We diagnosed the patient's condition as cystic duct metastasis from RCC and performed open cholecystectomy. Histopathology indicated a metastatic tumor of clear cell RCC in the cystic duct wall. In patients with a medical history of RCC, hypervascular lesions suggest the possibility of metastasis. Therefore, detailed imaging examinations should be performed.

A 60-year-old Japanese man with autosomal dominant polycystic kidney disease (ADPKD) on maintenance hemodialysis underwent colonoscopy and endoscopic mucosal resection (EMR). He was hospitalized after 4 days of fever that began the day following colonoscopy. We detected Klebsiella pneumoniae in a blood culture and a ring-shaped integration in the liver cyst by gallium scintigraphy. He recovered with antibiotics and percutaneous drainage. The patient was believed to have contracted the liver cyst infection via an injured colonic mucosa and portal vein. Thus, if a patient exhibits fever after a colon EMR, for patients with ADPKD, then a liver cyst infection should also be considered in the differential diagnosis.

A 49-year-old woman who was asymptomatic was found to have a small liver tumor on abdominal ultrasonography (US) at her annual health checkup. US revealed a hypoechoic, solid, mass measuring 17-mm in size in segment 6. The tumor markers associated with liver malignancy were negative. An infectious disease screen was negative for hepatitis B surface antigen, but positive for antibody to hepatitis B core antigen. Imaging studies using computed tomography (CT), magnetic resonance imaging (MRI), and CT angiography suggested a malignant liver tumor, such as hepatocellular carcinoma. Partial hepatic resection of the posterior segment was performed. The pathological diagnosis was pseudolymphoma of the liver.

We herein report a 55-year-old woman who presented with erythema and bilateral hilar lymphadenopathy 4 months prior to the detection of pancreatic lesions on an ultrasound. A skin biopsy showed evidence of sarcoidosis. The largest lesion in the tail of the pancreas was hypoechoic on endoscopic ultrasonography (EUS). The lesion was initially iso-enhanced on contrast enhanced-EUS (CE-EUS) but subsequently became hypoenhanced. The lesion revealed heterogeneous components of both soft and hard tissue on EUS elastography. She was ultimately diagnosed with pancreatic sarcoidosis based on the presence of noncaseating granulomas seen on pancreatic tissue retrieved through an EUS-guided fine needle aspiration biopsy.

A 50-year-old woman with a large right breast mass was emergently hospitalized for generalized weakness and fatigue. A histological examination of tumor biopsy specimens revealed a phyllodes tumor (PT). She suddenly lost consciousness due to severe hypoglycemia. Non-islet cell tumor hypoglycemia (NICTH) due to the PT was suspected. The tumor was emergently resected. A histological examination revealed a borderline PT. The patient recovered from the hypoglycemic episode. High-molecular-weight insulin-like growth factor II was detected in serum that had been collected preoperatively and in the tumor tissue, but not in serum that had been collected postoperatively. We herein present a case of a borderline PT with NICTH.

Allergic bronchopulmonary mycosis (ABPM) is a pulmonary hypersensitivity disease mainly caused by Aspergillus fumigatus. The mainstay treatment for ABPM is systemic corticosteroid therapy. A 25-year-old man presented with pulmonary infiltrates. His peripheral eosinophil, total serum IgE, and serum Aspergillus-specific IgE levels were elevated. The patient tested positive in a skin test for Aspergillus. However, sputum cultures revealed a Curvularia lunata infection. We therefore diagnosed ABPM possibly caused by C. lunata, which is rare in Japan. The clinical state of the patient improved under observation. Identification of the causative fungus is an important aspect of the ABPM diagnosis.

A 66-year-old man had been treated with prednisolone for eosinophilic pneumonia for 8 years. His slowly progressing cough and dyspnea were accompanied by elevated levels of fibrotic serological markers and an increased reticular shadow on chest computed tomography images. The patient had recently tested positive for anti-EJ antibodies, a type of anti-aminoacyl-tRNA synthetase antibody; therefore, we diagnosed him with an exacerbation of interstitial pneumonia due to anti-synthetase syndrome (ASS). He was treated with tacrolimus and an increased prednisolone dosage. We herein present the first reported case of eosinophilic pneumonia preceding anti-EJ antibody-positive ASS.

A 53-year-old man was admitted to the hospital with a diagnosis of cellulitis and osteomyelitis. Twenty-four days after the initiation of daptomycin and sulbactam/ampicillin, he developed a fever and pulmonary infiltration. Bronchoalveolar lavage revealed a high number of eosinophils, while an intracutaneous test revealed positivity for daptomycin. The patient improved after discontinuing antimicrobial therapy. The plasma daptomycin minimum concentration (C_min) was elevated (27.4 μg/mL), but plasma protein binding of daptomycin was low (87.8%). Although the pathophysiology of eosinophilic pneumonia remains unclear, antigenic stimulation due to daptomycin accumulation in the alveoli may have caused continuous immune activation.

Tracheal stenosis caused by malignancy is a life-threatening complication. We performed stent therapy in two patients using the AERO™ stent, launched in late 2016 in Japan. One patient presented with stenosis of the trachea due to adenoid cystic carcinoma and the other with stenosis of the trachea due to esophageal cancer. Both patients showed improved symptoms, and no complications were identified. This is the first report of a favorable outcome with the use of this hybrid stent in Japan, and the findings suggest that insertion of the AERO hybrid stent is an effective way to improve patients' quality of life.

We herein report the case of myasthenic crisis occurring in a 51-year-old man. He had experienced ptosis, increased body weight with edema, and fatigue with dyspnea. He presented at our emergency department with disturbed consciousness. He was originally diagnosed with myxedema coma, and he required artificial respiration. Because his weakness persisted and he was positive for anti-acetylcholine receptor antibodies and anti-muscle-specific tyrosine kinase antibodies, we diagnosed myasthenic crisis after various examinations. His clinical response to treatment was good and he was discharged in an ambulatory status 3 months after admission. This case demonstrates that myasthenic crisis may occur in association with myxedema.

We herein report the case of a 53-year-old man with cerebellar ataxia with anti-glutamic acid decarboxylase antibody (GAD-Ab) who mimicked Miller Fisher syndrome (MFS). He developed ophthalmoplegia, diplopia, and gait ataxia for one week. The serum and cerebrospinal fluid GAD-Ab titers were greatly increased, and the GAD-Ab index suggesting intrathecal antibody synthesis was elevated, while GQ1b-Ab was negative. After steroid pulse therapy and following prednisolone, his symptoms dramatically improved over the course of 11 months with the simultaneous decline of GAD-Ab titers. This case indicates that cerebellar ataxia with GAD-Ab can present with acute neurological findings mimicking MFS, and that steroid therapy has an excellent therapeutic effect.

Sepsis caused by a Capnocytophaga canis infection has only been rarely reported. A 67-year-old female with a past medical history of splenectomy was admitted to our hospital with fever and general malaise. She had been bitten by a cat. She showed disseminated intravascular coagulation and multi-organ failure because of severe sepsis. On blood culture, characteristic gram-negative fusiform rods were detected; therefore, a Capnocytophaga species infection was suspected. A nucleotide sequence analysis revealed the species to be C. canis, which was newly identified in 2016. C. canis may have low virulence in humans; however, C. canis with oxidase activity may cause severe zoonotic infection.