Objective It is well known that people with Down's syndrome (DS) frequently complicate with congenital heart diseases (CHDs). Patients with heart diseases often have sleep-disordered breathing as a co-morbidity (SDB) which worsens the heart diseases. However, the relationship between SDB and CHDs in DS people has not yet been fully elucidated. The aim of this study was to establish the association between SDB and CHDs in DS people using data from a large nationwide questionnaire survey in Japan.
Methods We conducted a cross-sectional questionnaire survey of a randomly selected sample of 2,000 DS people and their caregivers throughout Japan to examine the associations between observed signs of SDB and CHDs in DS people. The questionnaire included the presence of SDB symptoms (snoring, apnea, arousal, nocturia, and napping) and CHDs (the presence and types of CHDs).
Results Of the 1,222 replies received from the caregivers, 650 reported complications of some type of CHDs. The observed apnea tended to be higher among DS people with CHDs than those without CHDs (OR=1.28, 95% CI=0.97-1.70, p=0.09). DS people with tetralogy of Fallot reported significantly more frequent apnea than those without CHDs (OR=3.10, 95% CI=1.36-7.05, p<0.01).
Conclusion SDB prevailed among DS people with severe CHDs, such as tetralogy of Fallot. Careful attention to the signs of SDB in such patients may lead to earlier clinical intervention removing the vicious cycle between SDB and CHDs.

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Objective Prediabetes is a condition between a normal metabolic state and diabetes mellitus, which includes impaired glucose tolerance (IGT), impaired fasting glucose (IFG), or both. Prediabetes is related with undesirable cardiovascular outcomes. Epicardial fat thickness (EFT) has been correlated with cardiovascular risk factors. We herein aimed to assess EFT in prediabetic patients.
Methods We evaluated 64 patients with prediabetes and 30 age- and gender-matched healthy subjects. Demographic and anthropometric characteristics [age, sex, height, weight, body mass index (BMI), and waist circumference (WC)] and laboratory findings [fasting plasma glucose (FPG), postprandial plasma glucose (PPG), hemoglobin A1c (HbA1c), low density lipoprotein (LDL), high density lipoprotein (HDL), and triglycerides (TG)] were assessed. Transthoracic echocardiography was used to evaluate EFT.
Results There were no significant differences between the case and control groups in terms of age, gender, height, HDL, WC, systolic and diastolic blood pressure (SBP/DBP), or TG (p>0.05). Body weight, BMI, FPG, LDL, and, in particular, EFT were found to be significantly higher in the case group than control group (p<0.05). When compared with the control group, the median EFT was significantly higher in all prediabetic subgroups (IGT or IFG, p<0.001). However, no statistically significant difference was found between each case subgroup (p=0.795). When groups were adjusted according to age, sex, WC, and BMI with covariance test, the EFT remained increased in the prediabetes subgroups compared to the controls (p<0.001).
Conclusion We found the EFT levels to be increased in all subgroups of prediabetic patients regardless of FPG and HbA1c. Furthermore, EFT directly correlated with the patients' BMI and WC.

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Objective Acute respiratory worsening is defined as the unexpected rapid deterioration of idiopathic pulmonary fibrosis (IPF), and idiopathic acute respiratory worsening is known as an acute exacerbation of IPF. Torque teno virus (TTV) is a circular single-stranded DNA virus whose pathological significance remains unclear. The aim of the present study was to investigate the prevalence and titer of TTV DNA in IPF patients with acute respiratory worsening.
Methods The serum TTV DNA titer was measured using real-time PCR in nine IPF patients (two treated with steroids and immunosuppressants; seven treated without steroids or immunosuppressants) who developed acute worsening, including five patients with acute exacerbation. The serum TTV DNA titer was also measured in eight stable IPF cases and four IPF cases of lung cancer. In addition, in order to examine time course changes in the TTV DNA titer, the titer was measured more than once, with an interval of four weeks or longer, in eight patients.
Results Among the nine IPF patients with acute worsening, the TTV DNA titer was above 1×10⁶ copies/mL in two subjects without acute exacerbation who had been continuously treated with steroids and immunosuppressants. Meanwhile, the mean TTV DNA titer was 2.4±2.6 (×10⁴ copies/mL) in the five patients with acute exacerbation and 3.1±3.4 (×10⁴ copies/mL) in the eight patients with stable IPF. Moreover, the TTV DNA titers were increased in all three IPF patients who started treatment with steroids and immunosuppressants.
Conclusion Our results suggest that it is unlikely that TTV is directly involved in the onset of acute exacerbation of IPF and that the serum TTV DNA titer potentially reflects the immunosuppressive state of the host due to treatment.

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Objective Bronchoscopy using radial-endobronchial ultrasound (R-EBUS) and virtual bronchoscopic navigation (VBN) is a promising method for diagnosing peripheral pulmonary lesions. We previously performed a randomized comparative trial (RCT) (i.e., VBN combined with EBUS RCT) involving patients with 30-mm or smaller peripheral pulmonary lesions and found that the addition of VBN to R-EBUS improved the diagnostic yield. In the present study, we performed a retrospective subanalysis in order to identify patients for whom VBN is useful.
Methods The per-protocol population (194 cases) of the VBN combined with EBUS RCT was divided into subgroups based on the lesion size, lung lobe containing the lesion, lesion location, presence or absence of involved bronchi (bronchus sign) on thin-section CT and whether the lesion was detected on posterior-anterior (P-A) radiographs. The difference in the diagnostic yield between the VBN-assisted (VBNA) and non-VBN-assisted (NVBNA) groups was investigated.
Results Within the bronchus sign-positive subgroup, the diagnostic yield in the VBNA and NVBNA groups was 94.4% (68/72) and 77.8% (56/72), respectively, showing a significantly higher yield in the VBNA group (p=0.004; odds ratio: 4.9). The yield was particularly high for lesions smaller than 20 mm (94.6% vs. 70.7%; p=0.006), lesions located in the peripheral third of the lung field (95.1% vs. 71.4%; p=0.005) and lesions invisible on P-A radiographs (90.0% vs. 41.7%; p=0.026).
Conclusion VBN improves the diagnostic yield when combined with R-EBUS to assess lesions exhibiting involved bronchi on CT images.

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Objective Cerebral microbleeds (MBs) have been previously associated with cognitive dysfunction, including Alzheimer's disease. In the present study, we aimed to clarify the relationship between cerebral lobar MBs and the regional cerebral blood flow (CBF).
Methods We investigated the data obtained from 122 patients in our memory clinic who were examined by both MRI and ^99mTc-ethyl cysteinate dimer (ECD)-single photon emission computed tomography (SPECT). Patient brain scans were superimposed and brain regions containing both decreased CBF and MBs were visually identified. For each patient eight brain regions were evaluated, comprising the right and left frontal, temporal, parietal, and occipital lobes.
Results Cerebral MBs were detected in 36 of the 122 (29.5%) patients. Of these 36 patients, 23 had detectable lobar MBs, which were primarily distributed in the occipital lobe in 19 of the 46 (41.3%) regions with lobar MBs. The frequency of MBs accompanied by a decreased CBF in the parietal and occipital lobes was significantly higher than that observed in the frontal lobe (73.3% vs. 27.3%, p<0.05, and 73.7% vs. 27.3%, p<0.05, respectively). Additionally, a decreased CBF was observed significantly more frequently in the brain regions with 5 or more MBs compared to the regions with one microbleed (83.3 vs. 25.0%, p<0.0005). Among the 17 patients with observable MBs accompanied by a decreased CBF, none were initially diagnosed with either subjective complaints or mild cognitive impairment.
Conclusion We determined that the cerebral lobar MBs located in the parietal and occipital lobes, and the lobar regions with a large number of MBs, were significantly more likely to be accompanied by a decreased CBF.

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Objective The aim of this study was to analyze the efficacy of adalimumab (ADA) in patients with rheumatoid arthritis treated with or without methotrexate (MTX) and determine impact of the MTX dose.
Methods Pearson's product-moment correlation coefficient was used to assess the correlations between the improvement in the Disease Activity Score (DAS) 28- erythrocyte sedimentation rate (ESR) score and the MTX dose in patients receiving treatment with MTX at a dose of <8 mg/week, 8 mg/week and >8 mg/week.
Patients ADA therapy was initiated in 68 rheumatoid arthritis patients between July 2008 and June 2013. The mean MTX dose was 9.6 ± 2.6 mg/week, and the patients were followed for 24 weeks.
Results The mean DAS28-ESR scores at baseline and week 24 were 4.6 ± 1.3 and 2.7 ± 1.2 in the 60 patients treated with MTX and 4.5 ± 1.0 and 4.2 ± 1.5 in the eight patients treated without MTX, respectively. Clinical remission was achieved in 48% and 25% of the patients, respectively, by week 24. Moreover, 90.0% of the patients taking MTX continued to receive ADA until week 24, while 50.0% of the patients not taking MTX continued to receive ADA until week 24. Among the 35 patients receiving MTX at a dose of >8 mg/week, the DAS28-ESR scores decreased rapidly from 4.4 ± 1.2 at baseline to 3.2 ± 1.1 at week 4 and further decreased to 2.4 ± 1.0 at week 24. Meanwhile, clinical remission was achieved in 57% of the patients receiving MTX at a dose of >8 mg/week and 36% of those receiving MTX at a dose of ≤8 mg/week. A significant correlation was noted between the improvement in the DAS-ESR score and the MTX dose.
Conclusion In this study population, enhanced clinical efficacy of ADA was achieved in combination with the administration of a sufficient dose of MTX, determined to be >8 mg/week.

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We herein report a case regarding a 90-year-old woman with a history of recurrent episodes of urinary tract infections presenting with fever. Urinalysis revealed bacteria and white blood cells. Computed tomography showed dilated and fecally loaded rectum and colon with signs of obstructive uropathy. The patient was treated for urinary tract infection and constipation. Her bowel habits were controlled with lubiprostone, and she was discharged in good medical condition. This case highlights the importance of considering fecal impaction as a cause of urinary tract obstruction or infection.

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There are no previous reports of esophageal perforation due to endoscopic submucosal dissection developing into pyothorax. We herein describe a case of esophageal healing following perforation in a 60-year-old woman undergoing esophageal endoscopic submucosal dissection. Post-procedural computed tomography revealed pyothorax in the right thoracic cavity, compressing the right lung. The pyothorax did not improve despite treatment with thoracic drainage because the esophageal lumen was connected to the right thoracic cavity. In order to close the site of esophageal perforation, we inserted a covered self-expandable metal stent. The affected site subsequently healed without complications, allowing the drainage tube and stent to be removed.

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An anomalous origin of the left coronary artery from the right sinus of Valsalva (RSV) is rare. We herein report the case of an 80-year-old woman who presented to the emergency department with chest pain. Emergent coronary angiography was performed following a diagnosis of non-ST segment elevation myocardial infarction. A right coronary angiogram showed that the common trunk originating from the RSV branched into the left anterior descending and right coronary arteries. Although the initial angiogram failed to show the left circumflex artery (LCx), considered to be the culprit vessel, computed tomographic angiography demonstrated that the LCx was located immediately below the common trunk and exhibited a retroaortic course. We successfully treated the patient and obtained optimal angiography results.

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Coronary artery ectasia (CAE) is defined as a coronary artery dilatation with a diameter ≥1.5 times greater than that of a normal adjacent artery. All 3 coronary vessels can be affected by CAE, but the incidence of multivessel CAE among patients undergoing coronary angiography is quite low. We herein report an extremely rare case of acute myocardial infarction due to massive thrombi in the giant right coronary artery with multivessel CAE. Thrombus aspiration during percutaneous coronary intervention may be limited in giant coronary artery cases, and systemic thrombolysis may be effective in patients with massive thrombi in the giant coronary artery.

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An 81-year-old woman who had undergone dual chamber pacemaker implantation for sick sinus syndrome was referred to our hospital with drug-refractory common atrioventricular (AV) nodal reentrant tachycardia. Ventricular pacing (Vp) following premature atrial contraction (PAC) with a long AV interval induced ventriculoatrial (VA) conduction, which allowed the tachycardia to be initiated. The sensed AV interval was shortened to 80 ms, allowing Vp during the refractory period of VA conduction. Postventricular atrial refractory period was shortened to 180 ms to sense PACs with short coupling interval. After reprogramming, the suppression of the tachycardia by blocking VA conduction following Vp was confirmed.

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Platypnea-orthodeoxia syndrome (POS) is a rare syndrome of severe hypoxemia upon assuming an upright position. It is classically described as shunting from the right atrium to the left atrium usually via a patent foramen ovale (PFO). Alterations in the intrathoracic anatomy after liver resection and regeneration may trigger this condition in patients with clinically silent PFO -a previously unreported cause of POS.

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A 54-year-old woman was referred to our hospital for symptomatic sinus bradyarrhythmia with a sinus pause of 8 seconds. She was diagnosed with dextrocardia during childhood and discovered to have heterotaxy syndrome when she had an appendectomy during her teenager years. Chest and abdominal examinations by computed tomography showed multiple spleens located on the right side and abnormal drainages of the superior and inferior vena cava. Left isomerism was diagnosed by bilaterally bilobed lungs. Because of a patent bilateral superior vena cava, pacemaker leads were implanted using the right cephalic vein approach. Her fainting symptoms disappeared after pacemaker implantation.

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A 67-year-old woman was referred to our hospital due to a refractory lower extremity ulcer. Occlusion of the bilateral superficial femoral arteries and a difference (>50 mmHg) in blood pressure between the bilateral upper limbs were noted. In addition to occlusion of the left subclavian artery and stenosis at the ostium of the right coronary artery, these findings led to a diagnosis of Takayasu arteritis. Furthermore, a biopsy of the ulcerated skin lesion localized on the fibular surface showed a non-caseating cutaneous granulomatous lesion resulting in the diagnosis of cutaneous sarcoidosis. The simultaneous occurrence of cutaneous sarcoidosis and Takayasu arteritis, albeit rare, should not be overlooked.

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A 71-year-old woman was admitted with nephrotic syndrome. Light and electron microscopic analyses of renal biopsy tissue showed typical diffuse membranous features. In contrast, granular deposition of immunoglobulin A (IgA), but not IgG, IgM, C3 or C1q, was observed along the capillary walls on immunofluorescence. The patient was pathologically diagnosed with diffuse membranous nephropathy with solitary IgA deposition. Secondary membranous nephropathy was suspected; however, no underlying cause was found. The clinical and pathological findings, except for those of immunofluorescence, were all compatible with a diagnosis of primary membranous nephropathy. This is the first reported case of membranous nephropathy associated with solitary IgA deposition.

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A 65-year-old man was admitted to our hospital with edema and renal dysfunction. He had received a diagnosis of psoriatic arthritis at 50 years of age. As a renal biopsy showed IgA nephropathy (IgAN), bilateral tonsillectomy was performed, and one course of steroid pulse therapy with an oral steroid and mizoribine were subsequently administered. The patient's proteinuria gradually reduced in association with an improvement in the renal function. In addition, the rash and arthralgia were ameliorated. In this case, adding treatment for chronic epipharyngitis accelerated the curative effects, and focal infection therapy consisting of immunosuppressive drugs was effective for both IgAN and psoriatic arthritis.

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A 73-year-old man with a known history of asthma presented with dyspnea, worsening wheezing and a productive cough complicated by chronic sinusitis. Chest computed tomography showed bronchial wall thickening with centrilobular nodules and ground-glass opacity in the right lower lobe. Features meeting the diagnostic criteria for diffuse panbronchiolitis (DPB) were identified, and lobectomy confirmed the presence of lung cancer. Over the subsequent four years, the patient's symptoms worsened. We reevaluated a lung lobe specimen, which showed hypereosinophilic obliterative bronchiolitis (HOB). A transbronchial lung biopsy also indicated bronchitis with eosinophilic infiltration. Our initial diagnosis of DPB was subsequently changed to HOB after four years. We herein describe this case of HOB, which was initially diagnosed as DPB primarily based on high-resolution computed tomography, with a focus on the histopathology and long-term clinical course. This is the first report to document the long-term clinical course of HOB.

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Synovial sarcoma (SS) commonly arises in the para-articular soft tissue; however, very few cases of intravascular SS have so far been reported. We herein describe a case of pulmonary artery SS with massive pleural effusion. A biopsy of the pleural lesions showed uniform short spindle cell proliferation, while the SYT-SSX fusion gene, which is preceded by chromosomal translocation t(X;18)(p11;q11), was detected using reverse transcription-polymerase chain reaction. Treatment with ifosfamide chemotherapy and palliative radiation therapy was effective in reducing the growth of the tumor in the pulmonary artery and pleural lesions, indicating that this regimen may be useful for the treatment of unresectable SS in the pulmonary artery.

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A 40-year old woman presented with pyrexia, productive cough, and bilateral precordial pain. Positron emission tomography (PET)-computed tomography (CT) showed high, diffuse F-18 deoxyglucose accumulation in the tracheal, peribronchial, and bilateral costal cartilage. We diagnosed her with relapsing polychondritis (RP) based on McAdam's criteria. Airway lesions are a major prognostic indicator of RP, and so chronological assessment and control is essential. In this patient, PET-CT accurately reflected both the location and severity of the inflammation and helped to guide treatment decision-making and facilitated early detection of recurrence. However, its high cost is prohibitive to frequent use, making it necessary to comprehensively evaluate serum C-reactive protein levels, bronchoscopy, spirometry, and 3D-CT.

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Adalimumab is commonly used to treat autoimmune diseases with few reported hematological adverse reactions. We herein describe the case of an 85-year-old Japanese man with plaque psoriasis who developed autoimmune hemolytic anemia (AIHA) after 3 years of adalimumab treatment. The patient suddenly developed hematuria and dyspnea on exertion while receiving adalimumab treatment. Laboratory data showed low hemoglobin levels and slightly increased reticulocyte counts, while direct and indirect antiglobulin tests were positive. The patient was diagnosed with AIHA which resolved after replacing the adalimumab treatment with prednisolone therapy. The findings from this case indicate that AIHA may be caused by long-term adalimumab treatment.

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We herein report a case of Human T-lymphotropic virus type-I (HTLV-I)-associated myelopathy with bulbar palsy-type amyotrophic lateral sclerosis-like symptoms. A 52-year-old woman developed dyslalia at approximately 40 years of age, which slowly progressed. She presented with muscular atrophy and increased tendon reflexes of the extremities as well as bulbar palsy, from which motor neuron disease was suspected. Cerebrospinal fluid (CSF) testing revealed no abnormalities except for an elevated neopterin concentration at 143.17 pmol/mL (normal ≤30 pmol/mL). Her serum and CSF anti-HTLV-I antibody titers were also high. Intravenous infusions of methylprednisolone decreased the CSF neopterin concentration to 50.33 pmol/mL. Subsequent oral prednisolone therapy was effective in alleviating the symptoms.

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We herein report a patient with demyelinating inferior alveolar nerve hypertrophy, which was initially suspected to have a nerve tumor. A 39-year-old woman with childhood-onset polyneuropathy presented with tooth pain and visited a dental clinic. An X-ray examination of the mandible revealed enlargement of the mandibular canal, and a nerve tumor was suspected. CT scan and MRI showed hypertrophy of the inferior alveolar nerve along its entire length. We diagnosed the patient with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), which was supported by the spontaneous recovery reported in her childhood, the results from a nerve conduction study and MRI data. CIDP should be considered in the differential diagnosis of mandibular canal enlargement.

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Hemodialysis-related portal-systemic encephalopathy (HRPSE) is characterized by the presence of portosystemic encephalopathy without liver dysfunction, usually caused by changes in the systemic venous flow related to hemodialysis. We herein describe the case of a 75-year-old woman who developed hepatic encephalopathy five years after the initiation of hemodialysis. Abdominal contrast-enhanced computed tomography (CT) and three-dimensional CT angiography revealed a portosystemic venous shunt, and the patient was diagnosed with portosystemic encephalopathy. Occlusion therapy ameliorated her disturbance of consciousness. HRPSE should be recognized as a treatable neuropsychiatric disorder.

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Type II and type III cryoglobulinemic vasculitis (CV) are characterized by a deranged immune function due to concomitant chronic infections or rheumatic disorders. Conversely, type I CV is caused by plasma cell dyscrasia. Bortezomib is a proteasome inhibitor that is largely employed as a first-line treatment for multiple myeloma. The use of bortezomib in cases of monoclonal gammopathy of undetermined significance (MGUS)-related refractory type I CV has been reported in only four patients. In the current report, we discuss the efficacy of bortezomib treatment in a patient with type I CV, with a focus on the suitability and early application of this drug.

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We herein report a fatal case of Legionella pneumophila pneumonia in a tocilizumab-treated rheumatoid arthritis patient who was in a state of shock on admission but remained afebrile even during severe pneumonia. Legionella antigen was detected in the urine and neutrophil CD64 expression was highly elevated. Despite undergoing intensive treatment, the patient developed sepsis and died 12 days after admission. An autopsy indicated that while the Legionella infection had almost been controlled, a subarachnoid hemorrhage was the ultimate cause of death.

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We herein report a case of renal sarcoidosis presenting as acute kidney injury (AKI) during treatment with etanercept for rheumatoid arthritis. Blood tests showed a high level of angiotensin-converting enzyme and a renal biopsy demonstrated non-caseating granulomatous tubulointerstitial nephritis. The administration of high-dose steroid therapy (1 mg/kg) and discontinuation of etanercept resulted in an improvement in the patient's renal function. Although renal sarcoidosis induced by anti-tumor necrosis factor (TNF) therapy is an extremely rare manifestation, this case suggests that renal sarcoidosis should be considered in the differential diagnosis of AKI in patients receiving anti-TNF therapy, as providing an early diagnosis and treatment is important for preventing irreversible renal impairment.

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We herein report a rare form of sparganosis in a 29-year-old man presenting with pericardial effusion and lung lesions. The diagnosis was confirmed by the patient's history of eating inadequately cooked snake, significant elevated eosinophils in the peripheral blood and pericardial effusion, and marked positive reactions against Sparganum mansoni antigen in the serum. After two consecutive doses of praziquantel treatment, the patient's symptoms and laboratory and imaging findings were improved. Both specific antibody detection and follow-up of the patient's eosinophils, serum antibody, and imaging changes are important for sparganosis diagnosis, particularly in cases without a subcutaneous lump or mass.

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Cryptococcus arboriformis (C. arboriformis) is a novel Cryptococcus species belonging to the genus Trichosporonales. This novel species was identified definitively in 2007 using D1/D2 26S ribosomal DNA gene sequencing. In this article, we present a rare case of central line-associated bloodstream infection caused by C. arboriformis with successful treatment of this infection.

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Tuberculous aortic aneurysm is an extremely rare disease with a high mortality rate. The clinical features of this condition are highly variable, ranging from asymptomatic with or without constitutional symptoms, abdominal pain to frank rupture, bleeding and shock. We herein report the case of a 56-year-old man with a large tuberculous mycotic aneurysm in the abdominal aorta with an initial presentation of repeated attacks of abdominal pain lasting for several months. Due to the vague nature of the initial symptoms, tuberculous aortic aneurysms may take several months to diagnose. This case highlights the importance of having a high index of suspicion and providing timely surgery for this rare but potentially lethal disease.

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Ovarian hyperstimulation syndrome (OHSS) is an iatrogenic disorder resulting from ovulation induction. Although the occurrence of this disorder is rare, it can be potentially life-threatening in its most severe forms. We herein present the case of a young nulliparous woman who presented with features of abdominal compartment syndrome and was subsequently diagnosed with severe OHSS. All physicians, in particular critical care doctors, must be aware of this rare, but potentially life-threatening iatrogenic disorder.

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