This study aimed to evaluate the efficacy and safety of Chinese herbal medicine auxiliary therapy (CHMAT) in treating childhood cough variant asthma (CVA). A systematic literature review was conducted on RCTs that compared CHMAT, i.e., Chinese herbal medicine (CHM) plus pharmacotherapy (PT), versus the same PT alone in the treatment of CVA. All included trials were assessed for quality and risk bias and analyzed according to the criteria of the Cochrane Handbook based on the Review Manager 5.3 software program. Twenty studies were identified and the CHMAT group had a positive effect on the total effective rate and a lower recurrence rate compared with the control group. CHMAT may have positive effects on CVA, leading to better improvement in disorders of cough and asthma and less adverse effects. However, the methodology and reporting quality of current studies are generally low. Further studies should include larger sample sizes with a strict design to confirm these findings.

Objective The objectives of this study were to investigate the mRNA and protein expression of biomarkers related to absorption in the small intestinal mucosa of humans and determine the relationships between small intestinal diseases and nutrition.

Methods The study subjects consisted of patients scheduled to undergo double-balloon endoscopy (DBE) or total colonoscopy for suspected gastrointestinal disorder in a clinical practice. Biopsies were taken from apparently normal mucosa in the visible areas of 6 parts of the intestines from the duodenum to the colon. The mRNA expression of specific biomarkers (SGLT1, SGLT5, GIP, GLP, LAT1, LAT2, and NPC1L1) in the mucosa was compared among three patient groups: Inflammation, Tumor, and Control.

Results Sixty-six patients participated in this study. Both routes of DBE were performed in 20 patients, in whom biopsy samples were obtained from the mucosa for all sections. There were no remarkable differences in the mRNA expression levels among the 3 groups. However, SGLT1, GIP, GLP, and NPC1L1 exhibited specific distribution patterns. The expression levels of GIP and NPC1L1 were highest in the upper jejunum, but were extremely low in the terminal ileum and colon. A comparison of the mRNA expression profile in each intestinal section revealed that the SGLT1 mRNA expression in the Tumor group and the GIP mRNA expression in the Inflammation group were significantly higher than the corresponding levels in the Control group in the upper jejunum.

Conclusion The gastrointestinal mucosa of patients with small bowel diseases can maintain proper nutrient absorption, except in the upper jejunum.

Objective Some patients with malignant gastric outlet obstruction require combined biliary and gastroduodenal stenting (double stenting). However, biliary stent dysfunction can often disturb biliary route maintenance, thus making the optimal biliary stenting for these patients unclear. The present study was designed to assess the factors associated with the long-term maintenance of biliary drainage routes.

Methods The clinical features and long-term outcomes were assessed in patients who underwent double stenting.

Patients The outcomes were reviewed in 43 consecutive patients who successfully underwent endoscopic double stenting with metallic stents.

Results An univariate analysis of all patients with biliary stent dysfunction showed the separate type of double stenting (two stents placed in a non-crossed position) to be the only predictive factor related to successful biliary re-intervention for stent dysfunction (odds ratio 73.67, p=0.001). A comparison of the clinical features in patients who underwent separate and cross (two stents placed in a crossed position) stenting showed the functional success rate to be higher for the separate (93.3%) than for the cross (61.5%) stent type, with the median times to biliary stent dysfunction differing significantly (330 vs. 298 days, respectively; p=0.048). The success rates of re-intervention in patients with separate and cross type stents were 88.9% and 0.0%, respectively (p=0.001), and the initial biliary route maintenance rates were 96.7% and 53.8%, respectively (p=0.002).

Conclusion The separate type of double stenting may enhance successful biliary re-intervention for stent dysfunction and also maintain the initial drainage route longer. The biliary drainage outcomes should therefore be considered when choosing the type of double stenting.

Objective We retrospectively investigated the relationship between the tumor response and serial changes in α-fetoprotein (AFP) and des-γ-carboxy prothrombin (DCP) during hepatic arterial infusion of a cisplatin powder formulation (CDDP powder) in patients with advanced hepatocellular carcinoma (HCC).

Methods Seventy-six advanced HCC patients were analyzed. All HCC patients received high-concentration cisplatin (1.43 mg/mL) via the haptic artery at a dose of 65 mg/m². AFP and DCP were measured at baseline and four to eight weeks after treatment, and the antitumor responses were evaluated according to the response evaluation criteria in solid tumours (RECIST) criteria after one or two courses of treatment. The patients were classified into two groups, a decreased group and a non-decreased group, according to the change in the serum levels of AFP and DCP at four to eight weeks compared to baseline.

Results The response to treatment of the decreased group (n=16) and non-decreased group (n=60) was complete response/partial response/stable disease/progressive disease (CR/PR/SD/PD) in 4/4/5/3 and 1/11/8/40 patients, respectively. The response rate and disease control rate of the decreased group were significantly higher than those of the non-decreased group (p=0.016 and p<0.001, respectively). The median survival time (MST) of the decreased/non-decreased groups were 25.9/10.6 months, respectively. The cumulative survival rates for the decreased group were significantly higher than those of the non-decreased group (p=0.042). In the multivariate analysis, vascular invasion and the decreased group were significant factors that affected the therapeutic efficacy.

Conclusion A decrease in the levels of AFP and DCP after the first treatment with CDDP powder is a good predictor for the antitumor effect and the prognosis.

Objective Recent studies suggest that presepsin (soluble CD14-subtype) is a useful diagnostic and prognostic marker for sepsis, with secretion by activated macrophages potentially dependent on phagocytosis of microorganisms. As "hemophagocytosis" is one of the major characteristics in patients with hemophagocytic syndrome (HPS), we hypothesized that presepsin may reflect the phagocytic activity and be a useful prognostic marker for HPS. Therefore, we aimed to assess the prognostic potential of presepsin in secondary HPS in adult patients with hematological malignancies.

Methods Between April 2006 and August 2014, we retrospectively examined consecutive patients with HPS whose blood samples were available at our institution and compared the prognostic value of the following in HPS, singly and in combination: plasma presepsin, serum soluble interleukin (IL)-2 receptor (sIL-2R), ferritin, tumor necrosis factor-α (TNF-α), interferon-γ (IFN-γ), IL-6 and IL-10.

Results A total of 14 patients were enrolled. The median age of the patients was 46.5 years (range, 22-65). In univariable Cox models, there were no significant variables associated with the prognosis. However, in 12 evaluable patients, only the combination of higher median values of presepsin (>1,935 pg/mL) and sIL-2R (>4,585 U/mL) at the onset of HPS was significantly associated with the 90-day mortality (hazard ratio 14.5; 95% CI, 1.47-143.36; p=0.02).

Conclusion These results suggest that a composite model of plasma presepsin and serum sIL-2R levels at the onset of HPS might be a novel predictor of the prognosis of patients with hematological malignancies and secondary HPS.

Objective It is difficult to compare directly the practical effects of lifestyle modifications and antihypertensive medications on reducing cardiovascular disease (CVD). The purpose of this study was to compare the hypothetical potential of lifestyle modifications with that of antihypertensive medications in reducing CVD in an aging society using a success rate-oriented simulation.

Methods We constructed a simulation model for virtual Japanese subpopulations according to sex and age at 10-year intervals from 40 years of age as an example of an aging society. The fractional incidence rate of CVD was calculated as the product of the incidence rate at each systolic blood pressure (SBP) level and the proportion of the SBP frequency distribution in the fractional subpopulations of each SBP. The total incidence rate was calculated by the definite integral of the fractional incidence rate at each SBP level in the sex- and age-specific subpopulations.

Results If we consider the effects of lifestyle modifications on metabolic factors and transfer them onto SBP, the reductions in the total incidence rate of CVD were competitive between lifestyle modifications and antihypertensive medications in realistic scenarios. In middle-aged women, the preventive effects of both approaches were limited due to a low incidence rate. In middle-aged men and extremely elderly subjects whose adherence to antihypertensive medications is predicted to be low, lifestyle modifications could be an alternative choice.

Conclusion The success rate-oriented simulation suggests that the effectiveness of lifestyle modifications or antihypertensive medications in preventing cardiovascular events largely depends on the baseline incidence rate and sex- and age-specific behavioral factors.

Intimal sarcoma is a rare disease with a poor prognosis. We herein report the case of a 71-year-old man with intimal sarcoma of the pulmonary artery treated with pazopanib. The tumor showed regression after 1 month of treatment. Hand-foot syndrome led to cessation of pazopanib, which triggered a disease flare. Pazopanib should be considered in patients with intimal sarcoma of the pulmonary artery that is unresectable or recurrent after surgery or cytotoxic chemotherapy. We must be careful about drug cessation, as it can lead to a disease flare.

Platypnea-orthodeoxia syndrome (POS) is a rare condition characterized by interatrial right-to-left shunting that is exacerbated in the upright position. We herein report a 78-year-old woman with POS that remained undiagnosed for 2 years, despite repetitive transthoracic echocardiography (TTE). POS was ultimately diagnosed using contrast transesophageal echocardiography (TEE), which revealed a marked increase in right-to-left shunting in the sitting position, associated with simultaneous desaturation. Therefore, we propose that POS should be considered according to the clinical symptoms, regardless of the repetitive TTE results, and contrast TEE should be performed in both the supine and sitting positions to exclude a diagnosis of POS.

Hyperthyroidism and thyroid storm affect cardiac circulation in some conditions. Several factors including trauma can induce thyroid storms. We herein describe the case of a 57-year-old woman who experienced a thyroid storm and exacerbation of acute heart failure on thyroid echography. She initially demonstrated a good clinical course after medical rate control for atrial fibrillation; however, thyroid echography for evaluating hyperthyroidism led to a thyroid storm and she collapsed. A multidisciplinary approach stabilized her thyroid hormone levels and hemodynamics. Thus, the medical staff should be prepared for a deterioration in the patient's condition during thyroid echography in heart failure patients with hyperthyroidism.

A 46-year-old man was admitted to our hospital for near syncope and palpitations. An electrocardiogram showed a common type of atrial flutter (AFL) with 1:1 atrioventricular conduction. Transthoracic echocardiography revealed a massive right atrial (RA) thrombus with a huge RA and right ventricle. The patient was diagnosed with arrhythmogenic right ventricular cardiomyopathy. It was difficult to control the heart rate with beta-blockers during AFL, which resulted in the deterioration of right-sided heart failure. The effect of anticoagulation therapy for the RA thrombus was also limited. Restoration to sinus rhythm by catheter ablation effectively improved the right-sided heart failure, and the massive RA thrombus eventually disappeared.

In 1981, a 48-year old man was diagnosed with insulin autoimmune syndrome. In 2005, he experienced a substantial increase in his monoclonal insulin antibody levels; in 2006 and 2007, serum monoclonal gammopathy and an 11% marrow plasmacyte ratio were confirmed. In 2012, asymptomatic multiple myeloma was diagnosed based on an increased γ-globulin fraction and serum M-protein (IgG) levels. The insulin antibody binding rate was 75.4% in 2005 and 78.8% in 2012. In 2012, he was hospitalized for ileus and died. Autopsy identified multiple myeloma and no endocrinological tumors in the pancreas.

Behçet's disease (BD) is a multisystem disorder of unknown etiology. Renal involvement in Behçet's disease is not frequent. Pauciimmune necrotizing and crescentic glomerulonephritis is associated with antineutrophil cytoplasmic antibodies (ANCA) which are believed to activate neutrophils and cause vasculitis, especially if there is a concurrent synergistic inflammatory process. In this case report, we describe a patient diagnosed with BD who also developed pauciimmune necrotizing and crescentic glomerulonephritis. We also compared this case with similar previous cases reported in the literature. The aim of this study is to add information regarding ANCA, pauciimmune glomerulonephritis and Behçet's disease.

An 86-year-old woman developed acute kidney injury after colonoscopy. A renal biopsy showed diffuse tubular injury with minimal calcium phosphate deposits (CPDs), which were thought to be caused by an oral sodium phosphate bowel purgative before colonoscopy. According to these findings, she was diagnosed with acute phosphate nephropathy (APhN). In contrast to previous reports of diffuse tubular injury associated with tubular CPDs in APhN, this case demonstrated diffuse tubular injury despite a limited distribution of CPDs, suggesting that calcium phosphate can cause tubular injury without deposition. This case thus supports the hypothesis that urinary calcium phosphate crystals may cause tubular injury via other mechanisms, including inflammatory cytokines.

We herein report two separate cases in which a tuberculous abscess of the chest wall invaded the liver by penetrating through the diaphragm. After confirming the presence of tuberculous lesions in the chest wall and liver, both patients received preoperative anti-tuberculosis (TB) medications for two weeks; after which, the lesions were surgically removed. Following surgery, both patients fully recovered and were asymptomatic, but continued to receive routine postoperative care involving anti-TB medications. Neither patient showed recurrence of TB during a 15-month follow-up period.

We herein present the first case of rapidly progressive interstitial lung disease (RP-ILD) with anti-melanoma differentiation-associated protein 5 (MDA5) antibody evaluated by surgical lung biopsy (SLB). High-resolution CT scan revealed perilobular opacities, which rapidly became thicker and formed consolidation, resulting in remarkable loss of lung volume. Specimens taken from SLB revealed membranous organization with alveolar occlusion, dilation of alveolar ducts, and sacs with collapsed alveoli, which are typical features of diffuse alveolar damage (DAD). Rapidly progressive perilobular opacities may be characteristic of RP-ILD with anti-MDA5 antibody and DAD.

Primary lung tumor tissue exhibiting numerous multinucleated osteoclast-like giant cells (OGCs) is an extremely rare histological type. In this study, we describe the case of an 82-year-old woman. Chest CT demonstrated an extensive crazy-paving appearance in both of the lung fields and a solid round mass in the right lower lobe. A pathological examination of the tumor revealed sarcomatoid carcinoma containing OGCs combined with diffused, invasive lepidic predominant adenocarcinoma. We herein present an extremely rare lung cancer, invasive lepidic predominant adenocarcinoma extensively involving the lung with areas of sarcomatoid carcinoma containing OGCs.

We herein report a rare case of Burkitt lymphoma (BL) preceded by autoimmune hemolytic anemia (AIHA) caused by autoantibodies against D antigen. After a partial response to AIHA with prednisolone (PSL) treatment for 7 months, the patient developed BL with a t(8;22)(q24;q11.2) chromosomal translocation. Intensive immunochemotherapy, including rituximab, led to a complete response (CR) of BL; however, anti-D antibody remained detectable in the plasma and antibody-dissociated solution from erythrocytes, thus continuous therapy with PSL was necessary even after achievement of the CR. BL with AIHA is extremely rare, with only one previously reported case in the literature.

Primary bone lymphoma (PBL) is a rare disorder. We herein present a case of other iatrogenic immunodeficiency-associated lymphoproliferative disorder (OIIA-LPD) presenting as PBL. A 63-year-old woman was diagnosed with rheumatoid arthritis and had been treated with methotrexate for seven years. Two months before admission, she suffered from pain in the limbs. Magnetic resonance imaging revealed multiple irregular lesions in the bones of the limbs, which showed an uptake of ¹⁸F-FDG on positron emission tomography. A biopsy of the right radius revealed diffuse large B-cell lymphoma, leading to the diagnosis of OIIA-LPD. She received rituximab-containing regimens resulting in a complete response.

We herein describe a case of myelodysplastic syndrome with chronic graft-versus-host disease (cGVHD)-related polymyositis. On approximately day 1,570 post HLA-identical sibling bone marrow transplant, the patient presented with a fever, myalgia and liver dysfunction. A muscle biopsy revealed destruction of the muscle fibers and infiltration of CD20⁺ B cells and CD4⁺ and CD8⁺ T cells, and a liver biopsy confirmed the findings of cGVHD. An analysis of plasma cytokine profiles indicated elevation of not only T-helper (Th)1 and Th2, but also Th17 cytokines. Increases in these cytokines in addition to the invasion of inflammatory cells might be associated with the pathophysiology of cGVHD involving the muscle and liver.

Several reports have suggested an increased risk of malignant lymphoma in patients with rheumatoid arthritis treated with methotrexate (MTX). We herein describe the case of a 71-year-old woman with rheumatoid arthritis who developed MYC/BCL2 double-hit lymphoma associated with MTX therapy. She developed a fever and lymphadenopathies over a 2-week period and had elevated levels of soluble IL-2 receptor. Inguinal lymph node and bone marrow biopsies showed diffuse large B cell lymphoma. Fluorescent in situ hybridization revealed MYC and BCL2 gene rearrangements in her lymphoma cells. Accordingly, a diagnosis of MYC/BCL2 double-hit lymphoma was made. This is the first reported case of a double-hit lymphoma associated with MTX therapy.

A 49-year-old man with malignant hypertension, acute kidney injury and mental deterioration was referred to our hospital. We initially observed microangiopathic hemolytic anemia, thrombocytopenia and kidney damage, indicating he had thrombotic microangiopathy (TMA). We considered TMA was caused by malignant hypertension and therefore did not start plasma therapy. The French TMA reference center reported that platelet counts and serum creatine levels have high values for predicting severe ADAMTS13 deficiency. The patient fully recovered from his illness after treatment with antihypertensive drugs and intermittent hemodialysis. This case might thus be useful to understand the proper differential diagnosis and treatment of TMA.

Neuronal intranuclear inclusion disease (NIID) is an uncommon progressive neurodegenerative disorder. Adult-onset NIID can result in prominent dementia. We herein describe the case of a 74-year-old man who presented with dementia, cerebellar ataxia, neuropathy, and autonomic dysfunction. Diffusion-weighted imaging showed hyperintensity of the corticomedullary junction. Fluid-attenuated inversion recovery images showed frontal-dominant white matter hyperintensity. NIID was diagnosed from the presence of intranuclear inclusions in a skin biopsy sample. Neuropsychological testing revealed memory loss and frontal cognitive dysfunction, especially in relation to language and executive functions. We were therefore able to confirm the association of NIID with cognitive dysfunction.

Sjögren's syndrome (SS) may be complicated by neurological manifestations. We herein report three women (age range 26-60 years old) who all presented with limbic encephalitis (LE) as the predominant clinical feature 3 months to 15 years after the diagnosis of SS. The 26-year-old patient also developed acute motor axonal neuropathy one week after autoimmune encephalitis. All three patients showed contrast-enhanced MRI lesions and inflammatory cerebrospinal fluid findings, while not displaying any anti-neuronal antibodies and showing a remarkable response to immunotherapy. SS is often overlooked when the symptoms are mild. Therefore, in LE cases with no identifiable cause, serological screening for rheumatologic disorders is recommended.

A 78-year-old woman diagnosed with cyclic neutropenia 5 years previously had been treated with recombinant granulocyte-colony stimulating factor (G-CSF). She developed fever, tenderness and distension of temporal arteries after the treatment with G-CSF. Magnetic resonance imaging and ultrasonography revealed wall thickening of the temporal arteries. She was therefore diagnosed with giant cell arteritis (GCA). Small vessel vasculitis has been reported as a complication of G-CSF. However, the development of large vessel vasculitis after G-CSF treatment is quite rare. To our knowledge, the present case is the first report of GCA suspected to be associated with coexisting cyclic neutropenia and G-CSF treatment.

A 61-year-old Japanese woman with anemia, submandibular gland swelling, and enlarged lymph nodes was diagnosed with IgG4-related disease (IgG4-RD) by lymph node biopsy. Bone marrow involvement of IgG4-RD was detected by 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) and immunohistochemically proven by bone marrow biopsy in this patient. Anemia progressed gradually, and prednisolone treatment was initiated. Anemia and submandibular gland swelling improved soon after prednisolone treatment was initiated. We report a rare case of IgG4-RD involving the bone marrow. FDG-PET/CT and bone marrow biopsy were useful for diagnosis in this case.

We herein report the first domestic case of bacteremia caused by an intrinsic strain of colistin-resistant Acinetobacter. The Acinetobacter species was detected in the hemocultures in a febrile patient. The patient was a 65-year-old-man who was admitted to our hospital for laparotomic gastrostomy. The patient's antimicrobial susceptibility patterns were atypical; they were colistin resistant but not multiple drug resistant. A sequence analysis of rpoB identified the bacterium as an Acinetobacter genomic species 13BJ/14TU, which had only been previously reported in South Korea. He had never traveled to South Korea but frequently had contact with the South Korean community. We therefore demonstrated that infection with this species could occur in domestic cases.

Solitary small (<5 cm) amoebic liver abscesses in the right lobe are generally treated using medication alone, while large abscesses are typically treated via a combination of medication and drainage. However, the therapeutic indications for multiple medium (5-10 cm) amoebic liver abscesses remain unclear. We herein report the findings of a 53-year-old woman who was receiving lenalidomide for multiple myeloma and subsequently developed multiple amoebic abscesses. Metronidazole alone was unsuccessful, although metronidazole and repeated percutaneous catheter drainage of the right lobe, left lobe, and thorax proved to ultimately be successful. Therefore, the successful use of medication alone may be associated with the total combined abscess volume.