Systemic lupus erythematosus complicated with liver cirrhosis in a patient with Papillon-Lefèvre syndrome.

We report the first case of a girl who presented with Papillon-Lefèvre syndrome (PLS) and subsequently developed systemic lupus erythematosus and liver cirrhosis. This indicates that autoimmune diseases can be a complication in patients with PLS. Cathepsin C gene mutations were not found in our patient or her mother. Thus, other genetic factors may have been involved in this patient.

[The third repair for pseudoaneurysm following Bentall procedure: report of a case].

A 77-year-old male, who had undergone the Bentall procedure 27 years ago, was admitted to our hospital for the repair of postoperative pseudoaneurysm. This was the 3rd repair, and the pseudoaneurysm was close to the sternum. Total extracorporeal circulation was established with femorofemoral cannulation and sternotomy was performed under deep hypothermia. During sternotomy, we encountered massive hemorrhage due to injury of the aortic graft. We coped effectively with the situation utilizing temporary circulatory arrest. Aortic graft reimplantation was performed under continuous retrograde cerebral perfusion. Collapse of the suture line of the left coronary orifice was recognized and was reconstructed. The patient was discharged uneventfully on the 26th postoperative day.

[Treatment strategy for acute type A aortic dissection; how to manage mesenteric ischemia].

Mesenteric ischemia is a dreaded complication of acute type A aortic dissection. From January 1994 to December 2004, 134 patients with acute type A aortic dissection were operated. Eleven patients showed postoperative mesenteric ischemia. Mortality of such patients was much higher than that without mesenteric ischemia (81.8 vs. 10.6% , p < 0.0001). Preoperative mesenteric and/or lower extremity ischemia were revealed to be the risk factors of postoperative mesenteric ischemia. Our strategy to manage these patients is as follows; patients who are suffering mesenteric and/or lower extremity ischemia preoperatively, or those whose computed tomography (CT) shows stenosis, obstruction, or dissection of the superior mesenteric artery, should be recognized as high-risk patients of postoperative mesenteric ischemia. Their mesenteric circulation should be examined directly with laparotomy after the central repair. If the mesenteric circulation seems to be suboptimal, iliac artery-superior mesenteric artery bypass should be performed.

[Clinical outcome of aortic valve replacement with 19-mm prosthetic valve in elderly patients].

From 1979 to June 2005, 90 patients aged 65 or older underwent aortic valve replacement with 19-mm prosthetic valve. They were 84 women and 6 men, with a mean age of 74. The mean body surface area was 1.35 m2. Bioprosthetic valves were implanted in 77 patients (85.6%). In-hospital mortality was 2.2% (2 of 90). There were 13 late deaths. New York Heart Association (NYHA) functional class improved to class I in most of survivors. Survival rates for 5 and 10 years were 84.9 and 71.2%, respectively. The outcome of aortic valve replacement with 19-mm prosthetic valve in elderly patients was excellent.

Removals of pharmaceutical compounds from hospital wastewater in membrane bioreactor operated under short hydraulic retention time.

Pilot-scale membrane bioreactor (MBR) was operated at a short hydraulic retention time (HRT) of 3 h for the treatment of hospital wastewater. The removals of eleven pharmaceutical compounds in MBR operated at different mixed liquor suspended solids (MLSS) level were investigated during which nitrification degree was differed. The results experiments revealed the importance of immediate adsorption onto the colloidal particles in supernatant of MBR sludge and subsequently removed by membrane filtration for the recalcitrant pharmaceutical compounds. Nevertheless, the removals through biodegradation during short HRT were also found significant for some compounds. DGGE profile revealed the development of pharmaceutical degrading microorganisms in MBR.

[Successful implantation of a metal coronary angioplasty stent for bronchomalacia of the right tracheal bronchus associated with right isomerism complex in an early infant].

One-month-old boy had severe emphysema of the right upper lobe due to the stenotic tracheal bronchus compressed between the distorted right patent ductus arteriosus (PDA) and the right aortic arch associated with right isomerism complex. He underwent a left modified Blalock-Taussig shunt and a division of the PDA on cardiopulmonary bypass. Extracorporeal lung support (ECLS) was introduced because of severe hypoxemia caused by remaining bronchomalacia of the tracheal bronchus. On postoperative day 3, a metal coronary angioplasty stent was implanted at the stenotic lesion under fluoroscopic and bronchoscopic guidance. He was successfully weaned from ECLS and then respirator after the implantation. This simple stenting procedure might be an effective alternate in the treatment of bronchomalacia or bronchial stenosis in early infancy.

Dosimetric evaluation of compensator intensity modulation-based stereotactic body radiotherapy for Stage I non-small-cell lung cancer.

OBJECTIVE: To evaluate the dosimetry of compensator intensity modulation-based stereotactic body radiotherapy (SBRT) [non-coplanar intensity-modulated radiotherapy (ncIMRT)], its use was compared with that of three-dimensional conformation-based SBRT, for patients with Stage I non-small-cell lung cancer (NSCLC). METHODS: 21 consecutive patients with Stage I NSCLC were treated with ncIMRT or SBRT at Tokyo Medical University. To compare the two techniques, ncIMRT and SBRT plans for each patient were generated, where the planning target volume (PTV) coverages were adjusted to be equivalent to each other. The prescribed dose was set as 75 Gy in 30 fractions. PTV coverage, conformity index, conformation number (CN) and homogeneity index (HI) were used to compare the two strategies. RESULTS: There was no statistically significant difference between PTV coverage for the 100%, 95% and 90% dose levels in the SBRT plan and those in the ncIMRT plan. The CN values were 0.53 ± 0.13 in the SBRT plan and 0.72 ± 0.10 in the ncIMRT plan. These values were significantly better than those of the SBRT plan (p < 0.001). The HI in the ncIMRT plan was 1.04 ± 0.03%, which was also significantly better than that of SBRT. CONCLUSION: The ncIMRT plan provided superior conformity and reduced the doses to the lung for patients with Stage I NSCLC. ADVANCES IN KNOWLEDGE: The delivery technique with compensator intensity modulation-based SBRT was evaluated. Concerning target motion, this is thought to be more robust and safer than SBRT for early-stage NSCLC.

Patients with severe emphysema have a low risk of radiation pneumonitis following stereotactic body radiotherapy.

OBJECTIVE: To evaluate the risk of radiation pneumonitis (RP) after stereotactic radiotherapy (SBRT) for patients presenting with severe pulmonary emphysema. METHODS: This study included 40 patients with Stage I non-small-cell lung cancer who underwent SBRT, 75 Gy given in 30 fractions, at the Tokyo Medical University, Tokyo, Japan, between February 2010 and February 2013. The median age of the patients was 79 years (range, 49-90 years), and the male:female ratio was 24:16. There were 20 T1 and 20 T2 tumours. 17 patients had emphysema, 6 had slight interstitial changes on CT images and the remaining 17 had no underlying lung disease. The level of emphysema was classified into three groups according to the modified Goddard's criteria (severe: three patients, moderate: eight patients and mild: six patients). Changes in the irradiated lung following SBRT were evaluated by CT. RESULTS: On CT images, RP was detected in 34 (85%) patients, and not in 6 (15%) patients, during a median observation period of 313 days. Of the six patients, three had severe emphysema and three had no underlying lung disease. Patients with severe emphysema had lower risk of RP than those with moderate emphysema (p = 0.01), mild emphysema (p = 0.04) and no underlying lung disease (p = 0.01). CONCLUSION: Patients with severe emphysema had a low risk of RP following SBRT. ADVANCES IN KNOWLEDGE: Little is known about the association between RP and pulmonary emphysema. Patients with severe emphysema had lower risk of RP than those with no underlying lung disease.

Influence of suspension viscosity and colloidal particles on permeability of membrane used in membrane bioreactor (MBR).

In this study, pilot scale experiments were carried out to examine membrane fouling occurring in membrane bioreactors (MBR) with or without pre-treatment (coagulation/sedimentation). Especially, the influence of suspension viscosity and dissolved organic matter (DOM) on membrane fouling was investigated. The pre-coagulation/sedimentation process improved the performance of a MBR in terms of membrane permeability by controlling irreversible fouling and formation of thick cake layer. The upper limit of MLSS concentration for an efficient operation in MBR without pre-treatment was suggested to be around 10 g/L based on the measurement of suspension viscosity. In this study, it was difficult to directly relate membrane fouling to DOM detected in the membrane chamber. A series of laboratory scale dead-end filtration experiments was carried out to investigate which fractions in biomass suspension would be the most influential in the deterioration of membrane permeability. Based on the dead-end tests, it was shown that the deterioration of membrane permeability was mainly caused by the colloidal particle fraction in the biomass suspension.