Objective To examine risk factors for coronary artery disease (CAD) and retinopathy in patients with type 2 diabetes mellitus (DM) and assess the relationship between CAD and retinopathy.
Methods A total of 1,003 outpatients with type 2 DM (578 men and 425 women) were classified into two groups according to the presence (based on ischemic findings on a resting electrocardiogram or a history of angina or myocardial infarction) or absence of CAD and four retinopathy stages based on the International Clinical Classification of Diabetic Retinopathy.
Results Stepwise multiple regression analyses showed that independent risk factors for CAD were age, the triglyceride (TG) level and smoking, while those for retinopathy included age, age of DM diagnosis, the HbA1c level and a female gender. The prevalence of CAD increased in association with the progression of retinopathy (p<0.01).
Conclusion Since it is difficult to distinguish macrovascular and microvascular diseases, diabetic vascular disorders require comprehensive approaches to assessment and treatment.

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Objective IgA nephropathy (IgAN) is widely regarded as a slowly progressive disease. However, a minor population of patients present with a rapidly progressive form of glomerulonephritis (RPGN).
Methods We studied 25 cases of IgAN who presented with RPGN. The laboratory data, histology, and five-year prognosis after diagnostic renal biopsy were evaluated. We compared the data of these patients with those of 495 patients with the non-RPGN type. In addition, we divided the patients with the RPGN type of IgAN into a group with reduced renal function and a group with maintained renal function, and compared the data between the two groups.
Results In the 'RPGN type', the serum creatinine levels and a 24-hour urinary protein excretion were significantly higher than in the non-RPGN type. Histological examinations showed that the rates of endocapillary hypercellularity and tubular atrophy/interstitial fibrosis were significantly higher in the patients with the RPGN type. In the comparison between the groups with reduced and maintained renal functions, the former group exhibited higher levels of proteinuria, serum creatinine and crescent formation than the latter group.
Conclusion The RPGN type of IgAN was significantly worse in terms of the renal survival rate at five years than the non-RPGN type. Intensive and active treatments are necessary for this minor population, according to the guideline for the management of RPGN.

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Objective To assess the efficacy of pirfenidone in patients with advanced-stage idiopathic pulmonary fibrosis (IPF), we conducted a retrospective study of patients who received pirfenidone therapy. In addition, the combined effects of inhaled N-acetylcysteine (NAC) and pirfenidone were evaluated.
Methods Eligible patients had a clinical and radiologic diagnosis of advanced-stage IPF (stages of severity III&IV). Patients who exhibited a relative decline in forced vital capacity (FVC) of 10% or more within the preceding six (±2) months were enrolled. The outcome was evaluated from the date of the 6-month follow-up PFT. Relative declines in FVC of more than 10% were defined as progressive disease (ineffective group), while those less than 10% were defined as stable disease (effective group). The clinical features were compared between the two groups. We also compared the efficacy of the combined therapy with inhaled NAC and pirfenidone (n=11) with that of pirfenidone alone (n=7).
Results Eighteen patients 59-82 years of age with IPF who received pirfenidone therapy were reviewed. Pirfenidone stabilized declines in FVC by 10% at six months in eight of the 18 cases (44%). The median changes in FVC at six months were +120 mL in the effective group and -590 mL in the ineffective group. The number of NAC users was significantly higher in the effective group (7/8=87.5%) than in the ineffective group (3/10=30%) (p=0.02). Furthermore, the use of combined NAC therapy was correlated with a favorable outcome. The median change in FVC at six months was 0 mL in the NAC group and -290 mL in the non-NAC group. The median survival period was 557±66 days in the NAC group and 196±57 days in the non-NAC group (p=0.03).
Conclusion Among the advanced-stage IPF patients with a more progressive status, pirfenidone decreased the rate of decline in FVC. In addition, patients treated with pirfenidone combined with NAC therapy exhibited favorable outcomes. Additional studies are needed to confirm the efficacy of this combined therapy for IPF.

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Objective The aim of this study was to elucidate the efficacy of cyclophosphamide (CY) in Japanese patients with antineutrophil cystoplasmic antibody (ANCA)-associated microscopic polyangiitis (MPA).
Methods Sixty-four patients, newly diagnosed with ANCA-associated MPA were included in this retrospective study. The patients were divided into two groups based on whether they received combination therapy of CY and corticosteroid (CS) (CY group, n=29) or CS alone (CS group, n=35) for remission induction. The primary outcome was all-cause mortality.
Results Most patients in the CY group were treated with oral CY. Between the two groups, there were no differences in the baseline characteristics except for a higher proportion of male patients in the CY group. The remission rate was not substantially different between the two groups (86.2% in the CY group vs. 91.4% in the CS group). The survival rate was slightly higher in the CY group than in the CS group (not statistically significant; 0.86 vs. 0.77 at 1 year and 0.73 vs. 0.64 at 5 years, p=0.648). In the CY group, the hazard ratio after adjusting for age, sex, Birmingham vasculitis activity score values, serum albumin levels and C-reactive protein (CRP) levels was 0.657 (95% CI, 0.254-1.699; p=0.386).
Conclusion We observed no increased efficacy of CY in ANCA-positive MPA in the Japanese patients, and hence, its efficacy may be limited in these patients.

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Objective We clarified the clinical characteristics of patients with pulmonary nontuberculous mycobacterial (NTM) disease complicated by pneumothorax.
Methods We retrospectively selected 220 patients who satisfied the diagnostic criteria for NTM disease proposed by the American Thoracic Society (ATS). Nine patients with pulmonary NTM disease were complicated with pneumothorax. We investigated the patients' background, laboratory findings, radiological findings, treatment and prognoses.
Results There were nine patients, including six men and three women, with a mean age of 73.2 years. Seven patients had underlying respiratory diseases such as chronic obstructive pulmonary disease (COPD) excluding pulmonary NTM disease. The causative microorganisms was Mycobacterium avium in four patients, M. intracellulare in four patients, and M. kansasii in one patient. Regarding the radiological findings, pneumothorax was recognized in the right lung in five patients, in the left lung in three patients, and in both lungs heterogeneously in one patient. Although most patients exhibited multiple cavities and extensive lesions over the unilateral lung fields, three patients were simultaneously diagnosed with pulmonary NTM disease at the onset of pneumothorax. As for treatment, thoracic drainage was performed in seven patients, while one patient was advised only to rest and one patient required both thoracic drainage and surgery. The responses to the treatment was poor in each case, and five patients died due to pneumonia or heart failure.
Conclusion In this study, the rate of pneumothorax complications in the patients with pulmonary NTM disease (4.1%) was higher than that of other reports. The responses to treatment, and prognoses were poor due to the presence of other complications.

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Objective This study was conducted to investigate the clinical characteristics of patients with Aeromonas peritonitis, particularly secondary peritonitis.
Methods Patients with Aeromonas peritonitis treated between July 2004 and December 2011 were identified from the computerized database of a regional hospital in southern Taiwan. The medical records of these patients were retrospectively reviewed.
Results A total of 50 patients with Aeromonas peritonitis were identified. Nine cases were classified as spontaneous bacterial peritonitis, and 41 cases were classified as secondary peritonitis. The most common etiology of secondary peritonitis was acute appendicitis (n=26), followed by small bowel perforation (n=7) and colon perforation (n=6). The patients with spontaneous bacterial peritonitis were more likely to be immunocompromised (p=0.0013) and more frequently had an initial presentation of shock (p=0.0129), an abnormal liver function (p<0.05) and concomitant bacteremia (p=0.0024) than the patients with secondary peritonitis. Although the patients with secondary peritonitis had higher levels of inflammatory parameters, including leukocytes and C-reactive protein, and more frequent polymicrobial infections, their survival outcome rates, such as in-hospital mortality, were significantly lower (p=0.0007). The overall in-hospital mortality rate was 20%, and initial shock was the only independent prognostic factor for mortality (p=0.012).
Conclusion The clinical characteristics, including outcomes, of patients with spontaneous and secondary Aeromonas peritonitis differ. In-hospital mortality is significantly associated with the initial presentation of shock.

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A previously a healthy 64-year-old woman complained of a two-week history of hemorrhaging upon defecation. The laboratory and urinalysis findings were normal, and no serum or urine M components were detectable on protein electrophoresis. An air contrast barium enema revealed an elevated lesion measuring -20 mm in diameter with a smooth surface and a depression in the sigmoid colon. Colonoscopy revealed a red colored and congested tumor. The exposed surface of the submucosal tumor (SMT) center was somewhat yellow in color and covered with fuzz. All other portions of the colon were normal. The endoscopy and double-contrast barium revealed a normal upper gastrointestinal tract and a normal small intestine, respectively. A histopathological evaluation of a biopsy specimen obtained from the SMT suggested amyloid deposition. However, the other biopsy specimens of the esophagus, stomach, duodenal bulb, second portion of the duodenum, terminal ileum and other portions of the colon demonstrated no amyloid deposition. Colonoscopic ultrasonography (US) revealed the hypoechoic, homogeneous SMT to be mainly localized within the submucosa. An endoscopic submucosal resection (EMR) of the solitary amyloidosis was performed and the immunohistopathology revealed the entire SMT to consist of amyloid light chain kappa amyloid deposition. We considered that the US followed by EMR contributed to the precise diagnosis of solitary amyloidosis and the treatment of hematochezia caused by a solitary area of amyloidosis within the sigmoid colon.

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Patients with myxoma normally present with cardiovascular symptoms due to mitral valve obstruction caused by the tumor. However, some cases are difficult to diagnose because the findings of auscultation are normal and there are no cardiovascular symptoms. A 62-year-old man presented at a nearby clinic with a fever. No cardiac murmurs were heard on a physical examination. Abdominal ultrasonography was conducted to evaluate the origin of the fever, and a giant left atrial myxoma was discovered incidentally. Although many myxoma cases are found on transthoracic echocardiography, we herein describe a case of a giant left atrial myxoma incidentally discovered on abdominal ultrasonography.

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A persistent left superior vena cava (PLSVC) is formed by the remains of the oblique vein of the left atrium, which is not completely degenerated during embryonic development. The incidence is approximately 0.3% in the general population. Approximately 80-92% of PLSVCs drain into the right atrium through the coronary sinus. This report describes a rare case of PLSVC in which the coronary sinus (CS) did not open into the right atrium, but rather drained into the left subclavian vein through the PLSVC.

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Two unrelated Japanese women, 41 and 27 years of age, were admitted with histories of thirst, weight loss and palpitations of a few weeks' duration. Both were diagnosed to have diabetic ketosis or ketoacidosis with acute-onset type 1 diabetes (T1D) and Graves' disease (GD) (autoimmune polyglandular syndrome type 3 variant; APS3v), and were treated with intensive insulin therapy and anti-thyroid drugs. Human leukocyte antigen examinations showed that both cases had the HLA-A2, A24, B54, and DRB1*04:05-DQA1*03:03-DQB1*04:01 haplotype, which made them susceptible not only to APS3v, but also to both acute-onset T1D and GD. The genetic background of patients strongly contributes to the simultaneous occurrence of T1D and GD.

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A 72-year-old man presented with bilateral eyelid swelling and redness. An orbital CT scan showed bilateral proptosis, extraocular muscle enlargement and swollen lacrimal glands, mimicking thyroid-associated orbitopathy (TAO) with Hashimoto's thyroiditis (HT). During the patient's clinical course, spontaneous remission of lung consolidation (35×26 mm) was seen. A diagnosis of IgG4-related disease (IgG4-RD) was made based on an elevated serum IgG4 level (1,020 mg/dL; normal, 4-108), predominance of IgG4-positive plasma cells (IgG4/IgG: 35/70 in HPF) in the lacrimal glands and typical features of Mikulicz's disease. This report provides a novel description of this unusual disease entity among HT, TAO and IgG4-RD.

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Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is becoming more common for diagnosing intrathoracic lymphadenopathy, including tuberculous lymphadenopathy. We herein report two cases of complications possibly related to EBUS-TBNA for tuberculous lymphadenopathy. The first patient was a 26-year-old woman who developed intrabronchial polypoid granulomas exclusively at puncture sites two months after undergoing EBUS-TBNA. Although endobronchial extension may occur, the risk of aggravation caused by puncture should be considered. The second patient was a 39-year-old woman with transient smear-positive bloody sputum that developed immediately after EBUS-TBNA and persisted for three days. Temporary isolation following EBUS-TBNA should be considered for possible tuberculous lymphadenopathy.

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Lymphomatosis cerebri (LC) is a rare form of primary central nervous system lymphoma (PCNSL). Little is known about cases of LC with spinal cord involvement. Among the 11 PCNSL patients treated in our hospital during a four-year period, we identified two cases of LC with spinal cord lesions. One showed a spinal cord lesion followed by leukoencephalopathy. The other showed a spinal cord lesion after LC. In both cases, the histopathology was diffuse large B-cell lymphoma. It is possible that LC may affect the entire central nerve system, and tumor infiltration to the brain and spinal cord in LC may occur more frequently than has been previously considered.

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We herein present a case of concurrent chronic myeloid leukemia (CML) and chronic lymphocytic leukemia (CLL). Two different clones, a Philadelphia (Ph) clone and a CLL clone with a 13q deletion, were identified using fluorescent in situ hybridization. Dasatinib was administered to inhibit Bcr-Abl and Lyn kinase. The patient exhibited a molecular response for CML and a partial response for CLL. To our knowledge, this is the first report to describe the occurrence of a gradual increase in the Bcr-Abl transcript level prior to the diagnosis of Ph-positive CML in an individual with CLL who was successfully treated with dasatinib as the first-line therapy.

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A 74-year-old woman visited an otolaryngology clinic with pharyngeal pain, and was diagnosed with a peritonsillar abscess. She received antibiotics and underwent incisional drainage, but displayed high white blood cell and blast cell counts, and was referred to our hospital. Gram-negative rods (Leptotrichia trevisanii) were detected in blood cultures performed on admission. She was diagnosed with bacteremia and acute myelogenous leukemia (FAB classification: M1). After antibiotic therapy, she temporarily recovered from the bacteremia, but subsequently died on day 34. Although Leptotrichia trevisanii bacteremia is extremely rare, clinicians should consider it in cases involving immunocompromised patients with oral lesions.

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A 73-year-old man with long-term food deprivation and total parenteral nutrition was diagnosed with septic pulmonary emboli (SPE) and a persistent bacteremia caused by central line-associated blood stream methicillin-resistant Staphylococcus aureus (MRSA) infection. Although daptomycin (DAP) failed to treat the persistent bacteremia, linezolid successfully controlled it. DAP is inactivated by lung surfactant, and therefore, it should not be administered for lower respiratory infections. However, SPE caused by MRSA has been reported to be treatable with DAP since it is an infection of the lung parenchyma. We herein report the lack of effect of daptomycin in SPE treatment.

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Recently, interferon gamma release assays (IGRAs) have become an important clinical tool for detecting latent tuberculosis. However, IGRA results may impede making a diagnosis. We herein present an interesting case of miliary tuberculosis with a nonspecific IGRA reaction due to hemophagocytosis.

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