Jarcho-Levin Syndrome Associated with Atrial Septal Defect and Partial Anomalous Pulmonary Venous Return: A Case Report

A bstract A 61-year-old woman suffering from Jarcho-Levin syndrome (JLS) was associated with atrial septal defect and partial anomalous pulmonary venous return and underwent corrective surgery. Pressure controlled postoperative ventilator therapy is preferred in patients with JLS.     

CT and MR findings in diastematomyelia, with embryogenetic consideration.

Diastemstomyelia is an extremely rare disorder that is seldom found among the Japanese. This paper presents two Japanese patients, a newborn male and a newborn female, with diastematomyelia. CT demonstrated bony spurs more clearly than plain film, and magnetic resonance images indicated split cords and associated anomalies. Although the embryogenesis of diastematomyelia has not been clearly elucidated, the coincidence of levels of associated anomalies and diastematomyelia in our cases and in the literature supports Bremer's embryogenetic explanation of persistent accessory neurenteric canal.     

Pharmacokinetics of carboplatin and etoposide in a haemodialysis patient with Merkel-cell carcinoma

We present a Merkel-cell carcinoma patient with chronic renal failure requiring haemodialysis and evaluate the pharmacokinetics of carboplatin and etoposide during haemodialysis. The area under the concentration-time curve of carboplatin was increased by prolonging the interval between administration and haemodialysis. However, that of etoposide was not changed. Carboplatin showed good membrane permeability in haemodialysis, while etoposide showed no permeability. In conclusion, the pharmacokinetics of carboplatin could be controlled by haemodialysis and the interval between chemotherapy and haemodialysis. However, the pharmacokinetics of etoposide were not affected.     

Determination of vitamin B6 derivatives in foods and biological materials by reversed-phase HPLC

Through use of a simplified analyzing system, seven vitamin B6 derivatives were determined with a satisfactory sensitivity and precision. This system consisted of a single reversed-phase ODS column with a fluorescence detector employing an isocratic solvent system. Each vitamin B6 derivative in some foods and biological materials was determined, based on the measurement of the integrated peak area. The data obtained by this method were compared with those obtained from a bioassay by Saccharomyces uvarum ATCC 9080, after acid hydrolysis of these materials.     

Radiotherapy for malignant fistula to other tract

Radiotherapy was applied to 14 esophageal carcinomas with respiratory tract fistulas using various treatment regimens. Closure of the fistulas was observed in four cases after irradiation at a daily dose of 1.5 Gy. The period of closure was transient in two cases but long-term in the other two. One long-term closure case underwent low-dose rate telecobalt therapy (LDRT)(1 Gy per hour, 7 Gy per day; a total dose of 28 Gy) as a boost. His cancer has been well controlled and he is still alive without recurrence at 74 months after fistula formation.     

VALUE OF COMPUTED TOMOGRAPHY FOR EVALUATING THE INJECTION SITE IN ENDOSONOGRAPHY‐GUIDED CELIAC PLEXUS NEUROLYSIS

Endosonography‐guided celiac plexus neurolysis (EUS‐CPN) safely and effectively relieves pain associated with intra‐abdominal malignancies when the neurolytic is accurately injected. We applied contrast medium to evaluate the ethanol injection sites in patients who received EUS‐CPN due to abdominal pain caused by malignancies. We injected, under the guidance of endoscopic ultrasonography (EUS), ethanol containing 10% contrast medium into the celiac plexus of patients with intra‐abdominal pain due to malignancies. Immediately after the endoscopic therapy, patients underwent computed tomography (CT) to confirm the injection site. Images of distribution of injected solutions were classified into three groups. Injected solution dispersed in unilateral and bilateral anterocrural space was defined as ‘unilateral injection’ or ‘bilateral injection’, respectively. Injected solution located out of the anterocrural space was defined as ‘inappropriate injection’. Pre‐ and postprocedure pain was assessed using a standard analog scale. Before and 2, 4, 8, 12, and 16 weeks after the procedure, pain scores were evaluated. From April 2003 to May 2005, 13 patients were enrolled in this study. Improvement of pain score in the ‘bilateral injection’ and ‘unilateral injection’ groups was significantly superior to the change in the ‘inappropriate injection’ group. Although EUS‐CPN was effective in eight of 13 patients (61.5%), additional EUS‐CPN to the ‘inappropriate injection group’ increased the response rate to 84.6%. Injection of ethanol to the anterocrural space by EUS‐CPN produced adequate pain relief. Immediate examination by CT for confirmation of injection sites after EUS‐CPN would increase the likelihood of induction of pain relief.     

Long-Term Outcome of Adult-to-Adult Living Donor Liver Transplantation for Post-Kasai Biliary Atresia

Our objective was to analyze problems in the perioperative management and long-term outcome of living donor liver transplantation (LDLT) for biliary atresia (BA). Many reports have described the effectiveness of liver transplantation (LT) for BA, particularly in pediatric cases, but little information is available regarding LT in adults (> or =16 years old). Between June 1990 and December 2004, 464 patients with BA underwent LDLT at Kyoto University Hospital, of whom 47 (10.1%) were older than 16 years. In this study, we compared the outcomes between adult (> or =16 years old) and pediatric (<16 years old) patients. The incidence of post-transplant intestinal perforation, intra-abdominal bleeding necessitating repeat laparotomy and biliary leakage was significantly higher (p < 0.0001, <0.001 and <0.001, respectively) in adults. Overall cumulative 1-, 5- and 10-year survival rates in pediatric patients were significantly higher (p < 0.005) than in adults. Two independent prognostic determinants of survival were identified: a MELD score over 20 and post-transplant complications requiring repeat laparotomy. Outcome of LDLT in adult BA patients was poorer than in pediatric patients. It seems likely that LT will be the radical treatment of choice for BA and that LDLT should be considered proactively at the earliest possible stage.     

A 14-year-old with pulmonary hamartomatous lymphangiomyomatosis associated with bilateral pneumothoraces]

A 14-year-old girl was admitted because of cough, chest pain and hemosputum. Chest roentgenogram on admission showed a pneumothorax and a cavitary lesion with niveau formation in the right lung and cystic lesions in the bilateral lung fields. After bed rest and intravenous administration of antibiotics for two weeks, the right lung inflated well and the niveau formation disappeared, and the patient was discharged. One week later, she was readmitted with sudden-onset severe dyspnea, caused by bilateral pneumothoraces. Emergency tube thoracostomy and wedge resection of the bullous lesion was performed. Macroscopically, multiple small cystic changes were seen on the surface of the right lung. Histological examination revealed nodular proliferations of smooth muscle cells in the interstitium and vessel walls in the lung, which contained slit-like lymphatic channels. The diagnosis of pulmonary lymphangiomyomatosis was made. In this case, we could not measure receptors for estrogen and progesterone. Recently, hormonal therapy and oophorectomy have been reported as being useful. Tamoxifen (Norvadex) was therefore initiated, and the patient has remained well with slight dyspnea on exertion. There has been no recurrence of pneumothorax. Lymphangiomyomatosis is a rare disease of unknown etiology which occurs exclusively in women, mostly in those of reproductive age. We report a 14-year-old female patient with lymphangiomyomatosis associated with repeated pneumothorax, who had been under treatment for epilepsy. We believe this case to be of importance because of the long discussed relation between pulmonary lymphangiomyomatosis and tuberous sclerosis.     

Trends in antihypertensive drugs used for elderly patients]

The admission of antihypertensive medications in the elderly (65 y.o. or more, 269 pts) was compared to that in younger cases (less than 65 y.o., 348 pts). All were outpatients who visited our clinic in 1990. The number of patients on a single therapeutic regimen was almost equal in both age groups. Once-a-day regimens were more common in the young (56.9% vs 48.3%, p less than 0.05), and three times-a-day regimens were more common in the elderly (14.7% vs 23.1%, p less than 0.01). The choice of antihypertensive drugs in patients with single therapy or combined therapy in the young was beta blockers in 49.7%, Ca blockers in 39.4%, diuretics in 30.7% and ACE inhibitors in 17.8%, and those in the elderly were Ca blockers in 46.1%, diuretics in 44.2%, beta blockers in 33.8%, and ACE inhibitors in 16.4%. The patients were subdivided into three groups according to the time of the initial visit to the clinic; initial visit during 1969-1979 (phase 1), 1980-1984 (phase 2) and 1985-1990 (phase 3). In the young, choice of beta blockers and diuretics was most popular in phase 1. However, the choice of diuretics decreased in phase 2, and in phase 3 beta blockers were used in 50.4%, Ca blockers in 43.2%, ACE inhibitors in 22.3% and diuretics in only 17.3%. In the elderly, diuretics were most popular followed by Ca blockers and beta blockers in phase 1 and phase 2. In phase 3 Ca blockers were selected in 58.2% followed by both beta blockers and ACE inhibitors in 28.4% then diuretics in 23.9%.(ABSTRACT TRUNCATED AT 250 WORDS)     

Visibility of epidermoid tumors on steady-state free precession images.

PURPOSETo determine whether steady-state free precession sequences improve the MR visibility of epidermoid tumors in comparison with spin-echo images.METHODSPatients were four women and three men with epidermoid tumors in the subarachnoid spaces. MR was performed with a 1.5-T superconductive unit. For steady-state free precession imaging, three-dimensional Fourier transform fast imaging with steady-state free precession (FISP) images were used (20-40/7 [repetition time/echo time], flip angle of 25 degrees). The visualization and contrast-to-noise ratio were compared in FISP images and spin-echo images. In one case, the images of FISP and fast low-angle shot were obtained with variable repetition times and flip angles to evaluate the best pulse sequences for the visualization of epidermoid tumors.RESULTSThe contrast-to-noise ratios between tumors and cerebrospinal fluid ranged from 7.9 to 17.5 (average was 12.9) on FISP images. The average of contrast-to-noise ratios on T1, T2, and proton density-weighted spin-echo images were 1.6, 2.0, and 4.2, respectively. Three-dimensional Fourier transform FISP images best showed central nervous system and demonstrated epidermoid tumors excellently.CONCLUSIONSEpidermoid tumors in the subarachnoid spaces were better demonstrated on steady-state free precession (three-dimensional Fourier transform FISP) than on conventional spin-echo images.