Objective Although many studies have shown that cytomegalovirus (CMV) infection is an exacerbating factor in patients with ulcerative colitis (UC), there is no valid method to distinguish CMV infection requiring therapy from that disappearing without therapy. The aim of this study was to describe whether or not the endoscopic feature of a large ulcer predicts the necessity of antiviral therapy against CMV infection in active UC patients with positive mucosal viral assay.
Methods Active UC patients in whom CMV infection was detected by mucosal polymerase chain reaction (PCR) assay were enrolled in this prospective observational clinical study. Patients with a large ulcer (ulcerated group) were treated with antiviral and UC therapy. Patients without a large ulcer (non-ulcerated group) were treated with only UC therapy. We prospectively evaluated the clinical and endoscopic findings in all of the patients 2 months after starting this protocol, and observed their outcomes during one year.
Results In the ulcerated group (n=10), 3 patients still had active disease at 2 months and underwent colectomy. Although the other 7 patients achieved remission at 2 months, 4 of the 7 patients had a flare-up, and the remaining 3 patients maintained remission. All of the patients in the non-ulcerated group (n=10) attained remission without antiviral therapy at 2 months, and maintained remission.
Conclusion In active UC patients with positive CMV DNA by mucosal PCR assay, the absence of a large ulcer suggests latent CMV infection, and requires no antiviral therapy.

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Objective In Japan, percutaneous endoscopic gastrostomy (PEG) has been used mainly in patients with stroke and dementia, who undertake oral ingestion voluntarily. We have used PEG for patients with various diseases in Saga Medical School Hospital, including postoperative recovery, malignant disease, and neurodegenerative diseases. This study evaluated prognostic factors in these patients regarding long-term survival.
Methods We analyzed retrospectively all patients who received PEG at our hospital. During the period of 1998-2007, 84 patients (32 females, 52 males; mean age, 60.3 years, range 20-89 years) were followed for more than 1 year. We analyzed sex, age, total lymphocyte count, serum albumin level, presence of malignant diseases, cerebrovascular diseases, neurodegenerative disorders, poor general condition after surgical procedures, dementia before PEG, pneumonia before PEG, and complications of PEG placement.
Results As for diseases, 23 patients had malignant diseases, 27 had cerebrovascular diseases, 19 had neurodegenerative disorders, 16 were in poor general condition after surgery for nonmalignant diseases, and 12 had dementia. Multivariate analysis indicated that risk factors for 1-year survival were low serum albumin level (≤2.9 g/dL), low lymphocyte count, and complications of malignant diseases. Low serum albumin level, low lymphocyte count, and malignant diseases were risk factors, and only the albumin level was a risk factor in those without malignant diseases.
Conclusion Low serum albumin level was a risk factor for 1-year survival with PEG, which suggests that nutrient management before and during PEG placement should be monitored carefully.

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Background Inhalation of N-acetylcysteine (NAC) has been carried out in our department since 1994 for treating interstitial pneumonia such as idiopathic pulmonary fibrosis (IPF). In this study, the clinical efficacy and safety of long-term NAC inhalation monotherapy for IPF was investigated.
Methods NAC inhalation was carried out in 23 of 34 cases diagnosed as IPF by surgical lung biopsy in our department between 1994 and 2008. The treatment was continued for one year or longer in 14 cases. In these 14 cases and in 11 cases without treatment, the clinical courses, prognosis, lung function (%FVC, %DLco, and %TLC), and changes in serum markers for interstitial pneumonia (KL-6 and SP-D) were examined.
Results There were no significant differences in survival curves between the two groups. Acute exacerbation was observed in 4 of 14 cases (28.6%) receiving NAC inhalation. Compared with the results just before the beginning of NAC inhalation, Δ%FVC and Δ%DLco in the treated cases were -4.7% and -2.9% one year later and -4.0% and -5.8% two years later, respectively. In cases without treatment, Δ%FVC and Δ%DLco were -3.5% and +5.3% one year later and +0.2% and +1.0% two years later, respectively.
Conclusion Since this study is an open case-control study in a single institute and the number of cases is not large, its use in evaluating the efficacy of NAC inhalation monotherapy is limited. In addition, the role of NAC inhalation in combination with a steroid, an immunosuppressive agent, and a new anti-fibrosis drug should also be investigated.

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Objective We retrospectively evaluated the clinical usefulness of desensitization therapy for many patients showing allergic reactions to anti-mycobacterial drugs (INH and RFP) according to the proposition reported by the Japanese Society for Tuberculosis (JST).
Methods Desensitization therapy for anti-mycobacterial drugs was performed according to the propositions of JST for forty-six patients with mycobacterial disease in several hospitals partcipating in the Chugoku-Shikoku Mycobacterial Disease Committee between January 1999 and December 2009.
Results Adverse reactions occurred as drug-induced skin eruptions in 23 patients, drug-induced fever in 16, and drug-induced fever plus eruption in 7. The causative drugs suggested by the clinical course or DLST were RFP in 30 patients and INH in 16. The clinical effects of desensitization therapy for individual drugs was good in 23 of 30 patients (77%) receiving RFP, and in 13 of 16 (81%) receiving INH. Ten patients showing failure of desensitization included 5 elderly patients and 2 patients with a history of drug allergies. The interval until initiation of desensitization therapy ranged from 5 to 30 days after the disappearance of adverse reactions and the interval until the appearance of adverse reactions during desensitization therapy ranged from 3 to 18 days. A comparative study between the patient group with successful desensitization therapy and that with failure of desensitization did not show any significant differences except for the interval until initiation of desensitization therapy.
Conclusion We confirmed the clinical effectiveness of desensitization therapy for anti-mycobacterial drugs according to the propositions of JST in this multicenter study.

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A 62-year-old man presented with a two-week history of dry cough. A chest computed tomography (CT) showed three nodular masses of soft tissue density without calcification or cavitary formation in the right lung. F-18 fluorodeoxyglucose PET/CT scan revealed high FDG uptake in two out of three pulmonary nodules. Transbronchial lung biopsy specimens consisted of amorphous eosinophilic deposits that were demonstrated to be amyloid because they were positive for Congo Red staining. After oxidation with permanganate solution, the Congo Red staining disappeared, indicating that this amyloid was amyloid A protein-derived type. There was no evidence of any systemic diseases. We diagnosed the patient as having multiple nodular pulmonary AA amyloidosis. The patient was conservatively managed without treatment, and the pulmonary nodules disappeared spontaneously three months later.

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Pulmonary arterial hypertension (PAH) is a frequent complication in patients with systemic sclerosis. Bosentan is used in patients with symptomatic PAH; however, it has not been established whether or not bosentan ameliorates the progression of PAH in patients with no PAH-related symptoms.
We present a case of systemic sclerosis with no PAH-related symptoms in which bosentan ameliorated exercise-induced PAH evaluated by 6-minute walk stress echocardiography, brachial flow-mediated dilation, and skin temperature of hands and feet. The results suggest that administration of bosentan in patients with early-stage PAH ameliorates pulmonary arterial vasodilatation through improvement of endothelial function.

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A 39-year-old woman with Sanfilippo C syndrome was referred to our department for the treatment of bradycardia. An electrocardiogram revealed a second degree atrioventricular block, and pacemaker implantation was performed with the patient under general anesthesia. A transthoracic echocardiogram showed normal left ventricular systolic function, moderate mitral regurgitation due to mitral valve prolapse, and a high E/e' ratio, indicating left ventricular diastolic dysfunction. The present patient exhibited a rare case of Sanfilippo syndrome complicated with conduction disturbances, mitral regurgitation, and diastolic dysfunction.

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Multiple tumors in the liver, kidney, and on the posterior side of the urinary bladder were accidentally found when a diabetic woman visited the hospital. She refused to undergo surgery; therefore, she was only observed for 2 months. Subsequently, she was found unconscious at home and diagnosed with urinary infection, sepsis, and hyperglycemic hyperosmolar non-ketotic coma. The case followed a fulminant course, and she soon died. Postmortem computed tomography revealed emphysematous pyelonephritis (EPN), a rare, life-threatening infection. This case highlights the importance of carefully managing infection in diabetic patients, and it may contribute to clarifying the pathogenesis of EPN.

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A 48-year-old Japanese male was admitted to our hospital due to hyperosmolar hyperglycemic state (HHS), combined with rhabdomyolysis and acute kidney injury. His blood sugar levels were gradually decreased by fluid resuscitation and insulin infusion; however, his renal function worsened, and he developed bloody stools. He required continuous hemodiafiltration to improve his hemodynamics. As colonoscopy revealed longitudinal ulcers, ischemic colitis was diagnosed. We treated him conservatively at first, but when we found the ulceration of the sigmoid colon had penetrated the mesenterium, colectomy was indicated. After surgery, his general condition improved. Careful monitoring of complications related to HHS is important.

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Organizing pneumonia (OP) may be secondary to many clinical settings, including various infections, drugs, radiation therapy, malignant diseases, and connective tissue diseases. The concomitant occurrence of OP with human immunodeficiency virus infection has rarely been described. Recently, we encountered a case of OP confirmed by surgical lung biopsy in a patient with newly diagnosed AIDS. This case is the first in Korea and the second worldwide in which the diagnoses of OP and AIDS were made simultaneously. In this case, other possibilities that lead to OP, such as drug toxicity, were ruled out carefully.

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We report a case of acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) after pandemic influenza (H1N1) vaccination. A 57-year-old man, who had been diagnosed with IPF in September 2008, was admitted to our hospital in December 2009 because of aggravation of dyspnea and fever two days after H1N1 vaccination. Chest computed tomography showed diffuse bilateral ground-glass opacities superimposed on preceding reticular opacities. We diagnosed AE-IPF. Corticosteroid and cyclophosphamide were effective. Although the efficacy of influenza vaccination in patients with chronic lung diseases is well established, physicians should keep in mind that influenza vaccination has the potential to cause AE-IPF.

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An 82-year-old man was diagnosed with lymphoplasmacytic lymphoma involving multiple lymph nodes and bone marrow. On radiological examinations no involvement of the lung was seen. He was treated with rituximab. Eighteen months later he was complicated with right pneumothorax, and surgery with bullectomy was finally performed. Histological examination disclosed the proliferation of abnormal B lymphocytes near the wall of the bulla and pleura. We conclude that the pneumothorax in this patient was associated with lymphoma. Thus, radiological examination does not disclose lymphomatous lesions, it is possible that lymphoma involves the pleura, and pleural involvement can cause pneumothorax. Surgery is an effective method of treating this rare complication.

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Cytokine-induced killer (CIK) cells have been shown to be effective in the treatment of advanced cancer and minimal residual diseases. We report a multiple myeloma (MM) patient with concomitant lung cancer and paraneoplastic dermatoses, who received cellular immunotherapy with CIK cells which were derived from peripheral blood mononuclear cells (PBMCs) after being primed with anti-cluster of differentiation 3 (CD3) monoclonal antibody, interleukin-2 (IL-2), interferon-γ (IFN-γ) and IL-1. After treatment MM and lung cancer remained stable and no progression or recurrence was observed. Paraneoplastic dermatoses were obviously improved after treatment, which was first reported. No evident side effects were observed. These findings suggested that cellular immunotherapy with CIK cells was safe and effective in this patient with MM and lung cancer, and it might be a potent therapeutic option for paraneoplastic dermatoses.

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Progressive multifocal leukoencephalopathy (PML) is a rare and fatal demyelinating disease of the central nervous system caused by JC polyomavirus (JCV) reactivation in an immunocompromized host. We describe a case of PML in a 76-year-old woman with myelodysplastic syndrome, who had been treated with azathioprine for a pure red cell aplasia-like condition. PML was diagnosed based on the neurologic symptoms, the magnetic resonance imaging patterns and the detection of JCV DNA in the cerebrospinal fluid. She died ten months after the diagnosis. An autopsy confirmed the diagnosis, and JCV DNA was detected in the cerebrum. Azathioprine might have triggered PML.

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Cystic fibrosis (CF) is rare in Japan. We encountered a CF case with drug-resistant Pseudomonas aeruginosa infection and successfully performed lung transplant from living related donors. A combination of beta-lactams and aminoglycosides for drug-resistant P. aeruginosa infection was administered before lung transplantation. Intravenous colistin was also used immediately before and after transplant surgery. Gram staining of respiratory specimens was performed every day after surgery and it was useful in monitoring infection status. Strict monitoring of infections by the Gram staining and culture of respiratory specimens is considered to be effective in preventing lower respiratory infection in lung transplantation.

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