Clinical and pathologic characteristics of dilated cardiomyopathy in hemodialysis patients

BACKGROUND: Some dialysis patients have impaired left ventricular (LV) function without coronary artery disease. The pathologic changes and prognoses of these patients have not been well described. METHODS: We performed LV endomyocardial biopsies on 40 hemodialysis patients with dilated cardiomyopathy (DCM; an ejection fraction <50% and a left ventricular end-diastolic volume index >90 mL/m(2) without coronary artery disease), and on 50 nondialysis patients with idiopathic DCM as the control group. Following LV biopsies, the patients were followed-up for a mean of 3.1 +/- 2.3 years. RESULTS: The pathologic characteristics of the dialysis group were severe myocyte hypertrophy (the mean myocyte diameter across the nucleus: 37.6 +/- 10.5 mum vs. 25.6 +/- 7.7 mum, P= 0.001), myocyte disarray (30%), and extensive fibrosis (the mean percent area of left ventricular fibrosis: 22.3 +/- 18.4% vs. 21.3 +/- 14.6%, P= NS). These pathologic characteristics resembled the dilated phase of hypertrophic cardiomyopathy. In the dialysis group, a high percent area of LV fibrosis was the only significant predictor of cardiac death by multivariate analysis (P= 0.02). The 3-year cumulative event-free survival rate for cardiac death in dialysis patients with severe fibrosis (more than 30%) was 42%, while that for patients without severe fibrosis was 82% (P= 0.03). CONCLUSION: The pathologic characteristics of the heart in dialysis patients with DCM are interstitial fibrosis and severe myocyte hypertrophy with occasional disarray. The extent of LV fibrosis is a strong predictor of cardiac death. Careful follow-up and treatment are necessary for dialysis patients with a high percent area of LV fibrosis.     

Parapapillary choledochoduodenal fistula associated with cholangiocarcinoma

Parapapillary choledochoduodenal fistula is a rare disorder. We herein report a case of parapapillary choledochoduodenal fistula associated with cholangiocarcinoma. A 61-year-old woman was admitted to our hospital for further examination of a liver tumor. She had no clinical symptoms, but computed tomography scans showed an irregularly contoured liver tumor which was histologically confirmed to be adenocarcinoma, by a needle biopsy examination. Duodenal fiberscopy revealed a fistula orifice 1.0cm proximal to the orifice of the papilla of Vater, and endoscopic retrograde cholangiography through the fistula showed a communication to the common bile duct. Hypotonic duodenography demonstrated reflux of contrast material into the choledochoduodenal fistula. The bile sample collected from the common bile duct showed extremely high levels of pancreatic enzymes, including amylase, phospholipase-A2, and elastase-I. Furthermore, Helicobacter DNA was detected in bile by polymerase chain reaction (PCR) analysis. This experience suggests to us that parapapillary choledochoduodenal fistula may be a risk factor for biliary tract carcinoma, and surgical management is the treatment of choice for this rare condition, even when the patient has no significant clinical symptoms.     

Synthetic vascular prosthesis impregnated with genetically modified bone marrow cells produced recombinant proteins

The aim of this study is to develop an experimental model of small caliber expanded polytetrafluoroethylene (ePTFE) vascular prostheses that produce recombinant proteins by seeding genetically modified bone marrow mesenchymal stem cells (MSC). Beta-galactosidase (beta-gal) cDNA was transduced into rat MSC mediated by an adenovirus vector. The cells were impregnated into the ePTFE vascular prostheses measuring 2 mm in internal diameter and 90 microm in fibril length, followed by 48 h of incubation. The expressions of beta-gal were determined by X-gal staining. The luminal surface of the ePTFE vascular prostheses was covered with the MSC expressing beta-gal. Most of the gene-transduced MSC spread along the fibers forming colonies. These results suggest that small caliber vascular prostheses, in which the inner surface was seeded by genetically modified MSC, produced recombinant proteins. This may be a preliminary model to autocrine functioning vascular prostheses.     

Hearing loss in patients with enlarged vestibular aqueduct: air-bone gap and audiological Bing test

The Bing test is based on the principle that occlusion of the external auditory meatus improves the perception of bone-conducted sounds unless there is a conductive hearing impairment. An air-bone gap has been reported in patients with large vestibular aqueduct (LVA) syndrome without apparent middle ear dysfunction. We therefore performed the Bing test on nine patients with this syndrome to evaluate whether it is associated with an air-bone gap or middle ear dysfunction. Bone conduction thresholds did not change significantly during the Bing test in any patient. Because an air-bone gap is observed in patients with abnormal communication between the inner ear and cerebrospinal fluid through the LVA, dehiscent superior canal, or dilated inner ear meatus; we propose that a 'three windows' model (in which the abnormal communication provided by the enlarged endolymphatic duct and sac in LVA acts as the 'third window' for sound conductance) might explain the air-bone gap in such patients.     

Anxiety and depression of patients with digestive cancer

This study sought to characterize the psychological status of digestive cancer patients, and to investigate the relationship between psychological characteristics and clinical factors. Subjects were 85 inpatients scheduled to undergo surgery for digestive cancer and 26 control patients. The Japanese versions of Hospital Anxiety and Depression Scale (HADS) and Zung's Self Rating Depression Scale (SDS) were administered for all subjects before surgery, before discharge, and 6 months after discharge. Changes in HADS and SDS scores across the three examination days for three groups of subjects (advanced-phase, early phase, and control groups) were compared. The mean scores of anxiety and depression were significantly higher in the advanced-phase group than in the other two groups. Examination day showed a significant effect on depression; depression increased from before surgery to before discharge, and did not return to the preoperative level at 6 months after discharge, but no significant effect on anxiety. As for the relationship between psychological trends and clinical factors, anxiety in the 'middle age' and 'chemotherapy' groups was more severe than in the 'elderly' and 'no chemotherapy' groups. Depression in the 'medical treatment equipment', 'chemotherapy', and 'long-term hospitalization' groups was more severe than in the 'no equipment', 'no chemotherapy', and 'standard-term hospitalization' groups. These results suggest that we should pay careful attention to cancer patients undergoing surgery, especially young patients who are constantly at risk of anxiety, and assess their depression taking into account their disease and treatment conditions, especially after the time when their discharge is determined.     

Cognitive dysfunction in systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is an autoimmune-mediated collagen disease that results in multiorgan failure. It is the collagen disease most frequently associated with neuropsychiatric symptoms, which have been hypothesized to stem from certain types of cognitive dysfunction. Subjects were 21 patients with SLE (one man, 20 women; aged 16-55 years; mean age, 35.1+/-10.7 years) who were undergoing treatment in the rheumatology unit of a general hospital, and 17 healthy control subjects matched to the patient group with respect to age and gender (two men, 15 women; mean age, 35.9+/-6.3 years). They were administered various tests of cognitive function including verbal reasoning, non-verbal reasoning, verbal memory, non-verbal memory, attention and mental flexibility, psychomotor speed and frontal lobe function. In addition, the SLE patients were tested for antiphospholipid antibodies. The SLE patients performed worse than the control group on immediate, delayed and interference of the Rey verbal test and paired associate tests of Wechsler Memory Scale, and their reaction time was slower in Trail A and Trail B tests. Moreover, these findings were more pronounced in the group with major neuropsychiatric symptoms. However, no relationship was apparent between these deficits in cognitive function and the presence or absence of antiphospholipid antibodies. The results suggest that verbal memory and psychomotor speed underlie the neuropsychiatric symptoms seen in SLE patients.     

Abnormal regional cerebral blood flow in systemic lupus erythematosus patients with psychiatric symptoms

OBJECTIVE: Single-photon emission computed tomography (SPECT) studies have demonstrated decreased regional cerebral blood flow (rCBF) in systemic lupus erythematosus (SLE) patients. However, no study has done voxel-based analysis using statistical parametric mapping (SPM) that can evaluate rCBF objectively, and the relationship between rCBF and psychiatric symptoms has not been well investigated. Using L,L-ethyl cysteinate dimer (99mTc ECD) SPECT and SPM, we aimed to clarify the association of rCBF changes with psychiatric symptoms in SLE patients whose magnetic resonance imaging (MRI) showed no morphological abnormalities. METHOD: Twenty SLE patients and 19 healthy volunteers underwent 99mTc ECD SPECT. Data were collected from August 2000 to March 2003. SLE was diagnosed according to American College of Rheumatology criteria, and psychiatric symptoms were diagnosed according to ICD-10 criteria. On the basis of the modified Carbotte, Denburg, and Denburg method, the patients were classified into 3 groups: a group with major psychiatric symptoms (hallucinosis, delusional disorder, and mood disorder), a group with minor psychiatric symptoms (anxiety disorder, dissociative disorder, and emotionally labile disorder), and a group without psychiatric symptoms. Gross organic lesions were ruled out by brain MRI. Group comparisons of rCBF were performed with analysis using SPM99. RESULTS: SLE patients without MRI lesions showed decreased rCBF in the posterior cingulate gyrus and thalamus. The reduction in rCBF was overt in patients with major psychiatric symptoms. CONCLUSION: Our study indicated that SLE patients may have dysfunction in the posterior cingulate gyrus and thalamus and that this may be associated with the severity of psychiatric symptoms.     

Early intravenous gamma-globulin treatment for Kawasaki disease: the nationwide surveys in Japan

OBJECTIVE: To determine the optimal period of intravenous gamma-globulin (IVGG) treatment, using the database from nationwide Kawasaki disease surveys in Japan. STUDY DESIGN: We selected patients who first visited a doctor within 3 days of illness and received IVGG treatment within 9 days of illness. We divided these patients into 2 groups: an early group (treated on days 1-4: 4731 cases) and a conventional group (days 5-9: 4020 cases). We compared the rate of additional IVGG and prevalence of cardiac sequelae between these groups. RESULTS: The rate of additional IVGG in the early group was significantly higher than those of the conventional group (OR, 1.12 [95% CI, 1.10-1.16]). There were no significant differences in cardiac sequelae between the two groups. CONCLUSIONS: There is no evidence that IVGG treatment on day 4 or earlier has greater efficacy in preventing cardiac sequelae than treatment on days 5 to 9. In addition, early treatment is likely to result in a greater requirement for additional IVGG. However, there is also no evidence that early treatment increases the prevalence of cardiac sequelae in a clinical practice setting, where additional IVGG can be given to those whose initial treatment fails.