Objective Gastroesophageal reflux disease (GERD) is strongly associated with sleep disturbances; however, the detailed differences in the characteristics of sleep disturbances between GERD and non-GERD patients are unknown. The aim of the present study was to analyze the clinical characteristics as well as health-related quality of life in GERD and non-GERD patients with sleep disturbances. Methods Three hundred and fifty patients, including 124 patients with GERD and 226 patients without GERD, completed a self-administered questionnaire that evaluated clinical information. The Pittsburg Sleep Quality Index (PSQI), Hospital Anxiety and Depression Scale (HADS), Insomnia Severity Index (ISI), Epworth Sleepiness Scale (ESS) and 8-item Short-Form Health Survey (SF-8) were also used. Sleep disturbance was considered to be present if the PSQI was >5.5. Results The prevalence of sleep disturbances was significantly higher in the GERD patients (66/124, 53.9%) than in the non-GERD patients (89/226, 39.3%). Depression and anxiety were significantly more common in the subjects with sleep disturbances than in those without sleep disturbances, although there were no differences between the GERD and non-GERD patients. Among the subjects with sleep disturbances, daytime sleepiness was more common in the GERD patients than in the non-GERD patients. The subjects with sleep disturbances had a poorer health-related quality of life. The physical components of quality of life were impaired, particularly in the GERD patients with sleep disturbances. Conclusion GERD patients with sleep disturbances commonly experience daytime sleepiness and an impaired health-related quality of life, especially in terms of physical components.

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Objective The recurrence rate associated with antithyroid drug (ATD) treatment for Graves' disease (GD) is high compared with that for radioiodine therapy or surgery. It is important to identify patients in whom remission is unlikely, so that they are not given treatment that is destined to fail. The objective of this study was thus to evaluate factors influencing the prognosis of GD patients treated with ATDs. Patients One hundred and sixty-one patients were divided into two groups: 100 patients who could not discontinue ATDs for eight years or more (refractory group) and 61 patients who achieved remission within eight years after starting ATD treatment (nonrefractory group). The groups were compared in terms of age, thyroid function and thyroid-related autoantibodies at diagnosis, and the durations to the recovery of thyroid function and thyroid-related autoantibodies. Results The baseline levels of free triiodothyronine (T₃), free thyroxine (T₄), thyroid-stimulating antibodies (TSAbs) and thyroid-stimulating hormone (TSH) receptor antibodies (TRAbs) were high, and the age at diagnosis and the baseline level of thyroglobulin autoantibodies (TgAbs) were low in the refractory group compared with those in the nonrefractory group. The durations to the recovery of TSH, free T₄, TRAb and TSAb levels were longer in the refractory group than in the nonrefractory group. No significant difference was observed with regard to thyroid peroxidase autoantibodies. Conclusion We compared the clinical features of these two groups in order to identify factors influencing the prognosis of GD patients treated with ATDs. A low baseline level of TgAbs is associated with the refractoriness of GD to ATD treatment.

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Methods To investigate the safety and clinical significance of the method described in this study, we focused on 16 peritoneal dialysis patients with peritoneal dialysis (PD) catheter malposition and blockage in whom nonsurgical reposition was ineffective, who received a local incision about 5 cm below hypogastrium PD catheter insertions under local anesthesia. Tissues were separated layer by layer, 1-cm incisions were performed on the peritoneum vertically and conventionally, and then the PD catheters were pulled. Adherent mesentery was separated and the PD catheters were freed and removed sufficiently. PD catheters were introduced into the Dow cavity using large introducing forceps, were loop-ligated and fixed using 3# silk thread, and then the ligation line was sutured to the peritoneum. The tissues were managed layer by layer and the skin was sutured. All patients were followed up for half a year. Results Sixteen cases of refractory PD catheter malposition and blockage were managed successfully, with an operative incision of 3 cm and an operation time of 40±13 minutes. The localized anesthesia was well tolerated, and there were five cases in which lidocaine at 5 mg was added during the operation; postoperative pain was slight and only three patients used analgesics at night. All patients were treated with coagulation hemostasis, and there was no transfusion. No malposition, leakage or blockage was found at follow-up at more than six months. Conclusion It is safe, simple, inexpensive and associated with fewer complications to correct refractory PD catheter malposition and blockage by loop ligature and fixation through a minilaparotomy of inserted hypogastrium PD catheters promptly.

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Objective Since the term "combined pulmonary fibrosis and emphysema" (CPFE) was first proposed, the co-existence of pulmonary fibrosis and pulmonary emphysema (PE) has drawn considerable attention. However, conflicting results on the clinical characteristics of patients with both pulmonary fibrosis and PE have been published because of the lack of an exact definition of CPFE. The goal of this study was thus to clarify the clinical characteristics and phenotypes of idiopathic interstitial pneumonia (IIP) with PE. Methods We retrospectively analyzed IIP patients who had been admitted to our hospital. Their chest high-resolution computed tomography images were classified into two groups according to the presence of PE. We then performed a cluster analysis to identify the phenotypes of IIP patients with PE. Results Forty-four (53.7%) out of 82 patients had at least mild emphysema in their bilateral lungs. The cluster analysis separated the IIP patients with PE into three clusters. The overall survival rate of one cluster that consisted of mainly idiopathic pulmonary fibrosis (IPF) patients was significantly worse than those of the other clusters. Conclusion Three different phenotypes can be identified in IIP patients with PE, and IPF with PE is a distinct clinical phenotype with a poor prognosis.

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Objective In hematological malignancy patients, the complication of acute respiratory failure often reaches a degree of severity that necessitates mechanical ventilation. The objective of the present study was to investigate the therapeutic outcomes of mechanical ventilation in hematological malignancy patients with respiratory failure and to analyze the factors that are associated with successful treatment in order to identify the issues that should be addressed in the future. Methods The present study was a retrospective analysis of 71 hematological malignancy patients with non-cardiogenic acute respiratory failure who were treated with mechanical ventilation at Nippon Medical School Hospital between 2003 and 2014. Results Twenty-six patients (36.6%) were treated with mechanical ventilation in an intensive care unit (ICU). Non-invasive positive pressure ventilation (NPPV) was applied in 29 cases (40.8%). The rate of successful mechanical ventilation treatment with NPPV alone was 13.8%. The rate of endotracheal extubation was 17.7%. A univariate analysis revealed that the following factors were associated with the successful extubation of patients who received invasive mechanical ventilation: respiratory management in an ICU (p=0.012); remission of the hematological disease (p=0.011); female gender (p=0.048); low levels of accompanying non-respiratory organ failure (p=0.041); and the non-use of extracorporeal circulation (p=0.005). A subsequent multivariate analysis revealed that respiratory management in an ICU was the only variable associated with successful extubation (p=0.030). Conclusion The outcomes of hematological malignancy patients who receive mechanical ventilation treatment for respiratory failure are very poor. Respiratory management in an ICU environment may be useful in improving the therapeutic outcomes of such patients.

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Objective It is well known that grapefruit juice (GFJ) elevates the blood tacrolimus (TAC) concentration. We investigated the efficacy and safety of GFJ intake with TAC in cases of connective tissue diseases in which the TAC blood concentration was insufficiently high for clinical improvement, even when 3 mg/day or more of TAC was administered. Methods Seven patients took 200 mL of GFJ every day. The trough levels of the TAC blood concentration were measured before and after GFJ intake and the clinical courses were monitored thereafter. Results First, we surveyed the blood TAC trough levels of 30 recent patients who took 3 mg/day of TAC, and found that 21 patients (70%) did not achieve the minimum target TAC concentration (>5 ng/mL). Seven patients took GFJ due to a lack of efficacy and a relatively low TAC blood concentration. GFJ increased the TAC level from 4.3±2.4 ng/mL to 13.8±6.9 ng/mL (average increase: 3.3-fold). GFJ was also effective in achieving a clinical improvement in most cases without causing any severe adverse events, and it helped to decrease the dosages of glucocorticoid and TAC. In some cases, the blood TAC concentration fluctuated for no apparent reason. Conclusion GFJ intake was effective for the elevation of TAC concentration by approximately three fold and clinical improvement, but special care is required for monitoring its influence on concomitantly used drugs as well as TAC concentration. The addition of GFJ to TAC treatment could be an efficacious treatment option, when the plasma TAC concentration does not reach the minimal target concentration.

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Objective Although several studies have been conducted worldwide on factors that might improve residents' knowledge, the relationship between the hospital volume and the internal medicine residents' knowledge has not been fully understood. We conducted a cross-sectional study to compare the relationships of the hospital volume and hospital resources with the residents' knowledge assessed by the In-training Examination. Methods We conducted a retrospective survey and a clinical knowledge evaluation of postgraduate year 1 and 2 (PGY-1 and -2) resident physicians in Japan by using the General Medicine In-training Examination (GM-ITE) in 2014. We compared the ITE score and the hospital volume. Results A total of 2,015 participants (70.6% men; age, 27.3±2.9 years old) from 208 hospitals were retrospectively analyzed. Generalized estimating equations were used, and the results revealed that an increasing number of hospitalizations, decreasing staff number, decreasing age and PGY-2 were significantly associated with higher GM-ITE scores. Conclusion The hospital volume, such as the number of hospitalizations, is thus considered to have a positive impact on the GM-ITE scores.

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This report describes two cases with obstructive jaundice caused by poorly differentiated gastric adenocarcinoma. Computed tomography scans showed circumferential stenosis in the hilar bile ducts. Endoscopic retrograde cholangiopancreatography showed dilatation of the bilateral hepatic ducts and stenosis of the common hepatic ducts from the bifurcation of the bilateral hepatic ducts. The first diagnoses were hilar cholangiocarcinoma and biliary drainage decreased serum bilirubin; however, both patients died of cancer within a short period of time. Autopsies revealed lymphatic vessel invasion and possible subepithelial invasion by gastric adenocarcinoma into the hilar bile ducts. A differential diagnosis should thus be required in suspected cases of hilar cholangiocarcinoma.

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A small number of cases of small bowel obstruction caused by foods without the formation of phytobezoars have been reported. Repeated small bowel obstruction due to the ingestion of the same food is extremely rare. We present the case of 63-year-old woman who developed small bowel obstruction twice due to the ingestion of chestnuts without the formation of phytobezoars. This is the first reported case of repeated small bowel obstruction caused by chestnut ingestion. Careful interviews are necessary to determine the meal history of elderly patients and psychiatric patients.

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We herein report a case of Cronkhite-Canada syndrome (CCS) complicated with triple primary cancers. The patient was diagnosed with CCS at 65 years of age. At 76 years of age, one of his colon polyps was diagnosed as adenocarcinoma. At 81 years of age, gastric carcinoma was detected. Weight loss and fatigue appeared one month before he visited our hospital. An examination revealed dilatation of the intrahepatic bile duct. Cholangiocarcinoma was diagnosed as a result of bile duct cytology. Patients with CCS should be monitored carefully for carcinoma of systemic organs as well as the gastrointestinal tract.

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We herein report a case of a 56-year-old man with IgG4-related sclerosing cholangitis (IgG4-SC) with no biliary stricture, but with a severely thickened bile duct wall. Contrast-enhanced computed tomography showed diffuse swelling of the pancreas and thickening of the common bile duct (CBD) wall with delayed enhancement. Obvious diffuse wall thickening of the CBD was observed on endoscopic ultrasonography. However, endoscopic retrograde cholangiography showed no biliary stricture in the CBD that had thickened. Although IgG4-SC has been classified by a stenotic lesion on cholangiography, we should be aware of some IgG4-SC cases showing only bile duct wall thickness without any biliary stricture.

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Squamous cell carcinoma of the extrahepatic bile duct is quite rare. A 77-year-old woman with jaundice and general fatigue was referred to our hospital. Multiphase contrast-enhanced computed tomography visualized a 17-mm solid mass in the junction of the cystic and common bile ducts. The patient underwent pylorus-preserving pancreaticoduodenectomy. The pathological findings demonstrated keratin-positive poorly differentiated squamous cell carcinoma of the extrahepatic bile duct (T3N0M0, stage IIIA). Although adjuvant chemotherapy with gemcitabine was administered, the patient exhibited local recurrence at the site of anastomosis of biliojejunostomy 20 months after resection and died 32 months after resection.

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Systemic light chain (AL) amyloidosis is caused by abnormal plasma cell clones producing amyloidogenic light chains. The standard treatment is therefore chemotherapy targeting these clones, however, some patients are ineligible due to liver dysfunction. For these patients, preceding liver transplantation (LT) and following chemotherapy is a possible treatment option. We herein report a 58-year-old man with advanced hepatic AL amyloidosis who was successfully treated using this strategy. Previously reported cases treated with LT for this condition were reviewed, however, the outcomes were not favorable. We additionally investigated potential prognostic factors of this treatment approach to improve the outcome of these patients.

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A 75-year-old Japanese man with type 2 diabetes mellitus suffered from unresectable pancreatic head cancer and was admitted to our institution due to acute cholangitis. A partially covered metal stent was placed at that time. 11 months later, he was readmitted for acute cholangitis. Upper endoscopy revealed complete stent distal migration and a small hole on the oral side of the ampulla. While attempting cannulation into the hole, an upstream biliary tract was revealed. Accordingly, we diagnosed the patient to have a choledochoduodenal fistula. After metal stent removal and balloon dilation, we placed two 7 Fr plastic stents, which successfully relieved the patient's cholangitis.

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Adefovir dipivoxil (ADV) is effective for hepatitis B virus (HBV) infection; however, ADV may provoke renal injury resulting in osteomalacia, and this side effect is seldom recognized until bone fractures emerge. We herein present a 66-year-old woman with HBV infection who received ADV for 6 years. Although she exhibited no sign of bone fractures, her urinary β-2 microglobulin (β2MG) level increased to 83,837 μg/L and scintigraphy revealed minimal fractures of the third rib. ADV was subsequently reduced and her urinary β2MG rapidly fell to 3,637 μg/L. Conversely, her urinary N-acetyl-β-D-glucosaminidase, and serum phosphate, alkaline phosphatase levels did not respond.

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A 60-year-old man was admitted due to the onset of right coronary artery (RCA) aneurysms. Coronary angiography showed two RCA aneurysms and focal stenosis with limitations in the blood flow. Balloon angioplasty was performed. However, the follow-up coronary angiography showed restenosis, an enlarged proximal aneurysm and a newly formed aneurysm. The serum immunoglobulin G4 level was elevated to 1,350 mg/dL and fluorodeoxyglucose positron emission tomography showed increased uptake in the ascending aorta, so the patient was diagnosed with immunoglobulin G4-related vascular disease. The prevention of further enlargement of the aneurysms and an improvement in the RCA flow were achieved with steroid therapy. Steroid therapy may therefore be effective for immunoglobulin G4-related vascular disease.

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Protein-losing enteropathy (PLE) is a rare and life-threatening complication that occurs after the Fontan procedure. We herein report the case of an 11-year-old Japanese boy who developed PLE six times after undergoing the Fontan procedure. High-dose spironolactone therapy has been effective for 2 years. His high level of serum aldosterone decreased to a nearly normal range and spironolactone may have a diuretic and anti-inflammatory potential.

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A 63-year-old man was transferred to our hospital because of an exacerbation of asthma. His symptoms deteriorated even after increasing the dose of inhaled corticosteroids/long-acting beta2-adrenoceptor agonists (ICS/LABA). He had no chest pain and an electrocardiogram revealed ST elevation. A coronary angiogram revealed a reduced left ventricular function with an apical ballooning pattern without coronary stenosis. He was diagnosed with Takotsubo cardiomyopathy. Catecholamine elevation due to acute asthma and an overdose of ICS/LABA may be triggers of this disease. We should remember that Takotsubo cardiomyopathy is a complication of asthma and that catecholamine plays an important role in its onset, although it is essential for asthma treatment.

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An 80-year-old woman was referred to our hospital due to the presence of a mass that was identified on a chest X-ray. A further investigation demonstrated advanced adenoid cystic carcinoma of the lungs. Anti-cancer chemotherapy with docetaxel was carried out and the lesion remained as stable disease. Subsequently, pleural effusion was detected, and an investigation of the pleural effusion revealed the existence of malignant cells with an epidermal growth factor (EGFR) mutation. Gefitinib was administered and the pleural effusion resolved. This is the first case of a positive EGFR mutation of adenoid cystic carcinoma of the lung with a favorable response to an EGFR-tyrosine kinase inhibitor.

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Ataxia telangiectasia mutated (ATM) is a tumor suppressor gene, and its somatic inactivation plays a role in the pathogenesis of lymphoid malignancies. However, the role of ATM in patients with myeloid malignancies is still unknown. We herein report a case of acute megakaryoblastic leukemia (AMKL) with ATM gene deletion. An 84-year-old Japanese woman presenting with a pale face and pancytopenia was admitted to our institution and diagnosed to have AMKL with ATM gene deletion. She was treated with intravenous azacitidine. The azacitidine treatment was effective for approximately 1 year. Somatic inactivation of the ATM gene may therefore be involved in the pathogenesis of AMKL.

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A 53-year-old man was admitted for sub-acute progressive dementia and Gerstmann syndrome. MRI demonstrated lesions in the white matter involving the left parietal lobe, accompanied by speckled or faint linear peripheral enhancement. Brain biopsy revealed JC virus infection in oligodendrocytes and balanced infiltration of CD8+ and CD4+ T lymphocytes. We diagnosed progressive multifocal leukoencephalopathy (PML) with controlled inflammation. The finding of CD4/CD8 T cells in the infected PML brain suggested therapeutically valuable immune system involvement, which we decided to preserve by withholding corticosteroids. We treated the patient with risperidone, cytarabine and mefloquine to suppress virus replication, but not with the corticosteroid that is conventionally used in inflammatory PML cases. The patient was discharged three months after admission, and one year later, his score on the Mini-Mental State Examination had recovered to 26/30, from 5/30 on admission.

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Trousseau's syndrome is characterized by a cerebral or systemic thromboembolism caused by coagulation abnormalities in malignancy. We herein report a case of multiple ischemic strokes as the initial manifestation of anaplastic thyroid carcinoma (ATC). An 86-year-old man was admitted to our hospital due to a sudden-onset weakness of the left limbs. Brain magnetic resonance imaging revealed multiple ischemic lesions in the right middle cerebral artery territory and a mass in the left frontal lobe. Computed tomography revealed a thyroid mass and multiple lung tumors. A diagnosis of ATC was confirmed by a thyroid biopsy. Our case indicates that ATC should be considered as a cause of Trousseau's syndrome.

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Sensory neuronopathy is a well-established presentation in paraneoplastic neurological syndromes that is mostly associated with small cell lung cancer and anti-Hu antibodies. Motor neuronopathy, on the other hand, is an extremely rare observation in this syndrome. A 56-year-old man presented with asymmetric brachial diparesis and sensory ataxia. Electrophysiological studies revealed sensory ganglionopathy and progressive anterior horn degeneration in cervical segments. Small cell lung carcinoma with associated anti-Hu antibodies was later diagnosed. The patient did not improve despite the administration of steroids and chemotherapy. Paraneoplastic syndromes may exceptionally present with a bilateral arm weakness. Cases accompanied by sensory ganglionopathy should therefore be promptly investigated for any underlying malignancy.

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Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease that favors the cerebrum and typically occurs in immunosuppressed patients. We herein report the case of a 66-year-old man with PML, idiopathic CD4⁺ T lymphocytopenia (ICL), and chronic renal failure. Cranial magnetic resonance imaging (MRI) showed a crescent-shaped lesion in the left cerebellum, brainstem, and middle cerebellar peduncle. Although the patient did not present with HIV infection, collagen diseases, or tumors, JC virus DNA was detected in the cerebrospinal fluid. Clinicians should consider PML and ICL in the differential diagnosis if the patient develops progressive ataxia and a crescent-shaped cerebellar lesion on MRI.

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Crossed cerebellar diaschisis (CCD) is an interesting phenomenon which classically refers to the depressed blood flow and metabolism affecting one cerebellar hemisphere after a contralateral hemispheric infarction. However, CCD can also be caused by a prolonged seizure. We herein report a case of CCD due to status epilepticus in a patient who showed unique magnetic resonance imaging findings.

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A 62-year-old man presented with heliotrope rash, Gottron's sign, and mild muscle weakness. Both of his lung fields showed interstitial changes that worsened rapidly. He was diagnosed with clinical amyopathic dermatomyositis with rapidly progressive interstitial lung disease. The patient died of respiratory failure, despite the administration of immunosuppressive therapy. Autopsy revealed diffuse alveolar damage. An antibody analysis, which was performed postmortem, detected the presence of anti-melanoma differentiation-associated gene (MDA)-5 antibodies. Clinicians should note the clinical, radiologic, and serologic findings to predict anti-MDA-5 antibody-associated rapidly progressive interstitial lung disease.

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Intravascular large B-cell lymphoma (IVLBCL) is a rare and clinically aggressive lymphoma with an unfavorable prognosis. We report the case of a 50-year-old woman who was diagnosed with IVLBCL during treatment with methotrexate (MTX) and biologic agents for rheumatoid arthritis. The symptoms showed partial improvement only after the cessation of both treatments. She subsequently received chemotherapy and achieved a complete remission and has remained free of recurrence for 2 years without any further treatment. We herein describe a rare case of IVLBCL which presented with the features of an MTX-associated lymphoproliferative disorder.

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Primary autoimmune neutropenia (P-AIN) is an extremely rare disease. The most effective treatment for primary P-AIN is a granulocyte colony-stimulating factor; however, no curative treatment has been reported. We herein report a case of an adult P-AIN patient with a relatively mild medical history (irrespective of the severe neutropenia) who showed a sustained hematological response over seventeen months after the initiation of treatment with subcutaneous Alemtuzumab.

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