Lupus cystitis in the course of Evans syndrome

A 50-year-old woman with a 4-year history of Evans syndrome was admitted to our hospital because of progressive nausea, appetite loss, body weight loss, diarrhea and abdominal pain. Abdominal ultrasonography revealed pleural effusion, ascites, bilateral hydronephrosis, dilatation of the bilateral ureter, and irregular wall thickness of the urinary bladder. Immunological studies revealed decreased complement components (C3; 72 mg/dl, C4; 7 mg/dl, CH50; 28.8 mg/dl), a x 80 antinuclear antibody titer (homogeneous pattern), antibody against single-stranded DNA 19 U/ml, anti-SS-A antibody over 500 U/ml and negativity for antibody against double-stranded DNA (anti-dsDNA Ab). Although the patient did not fulfill the criteria for systemic lupus erythematosus (SLE), we diagnosed her as having lupus cystitis. Bolus methylprednisolone (mPSL) therapy (1,000 mg mPSL over 3 days, div) was administered, followed by 60 mg PSL, and this led to immediate improvement of the patient's symptoms and laboratory data. Later, anti-dsDNA Ab became positive, and the patient thereby fulfilled the criteria for SLE. Lupus cystitis following Evans syndrome has rarely been reported. The present such case was treated successfully with bolus mPSL therapy.     

Prostaglandin synthesis inhibitor affects humoral conditions and oxygen metabolism during normothermic cardiopulmonary bypass

We have reported that infusion of prostaglandin synthesis inhibitor (PGSI) reduced the severity of hypotension during normothermic cardiopulmonary bypass (CPB). In the present study, we investigated the effects of PGSI on humoral conditions and whole body oxygen metabolism during normothermic CPB conducted for 60 min in 8 adult goats. The PGSI group (n = 4) was administered 100 to 150 mg of flurbiprofen, a potent PGSI, before and during CPB, and the control group (n = 4) was administered noradrenaline (NA) to restore hypotension. The prostaglandin E2 (PGE2) and NA concentrations in the PGSI group were significantly lower than those of the control group (PGE2 8.8 +/- 1.0 versus 30.3 +/- 11.5 pg/ml, NA 431 +/- 197 versus 3847 +/- 2,153 pg/ml). The adrenaline concentration was not significantly different between the groups. The oxygen consumption and the oxygen extraction rate were not significantly different between the groups, but the blood lactate level in the PGSI group was lower than that of the control group (34.3 +/- 7.6 versus 43.7 +/- 3.8 mg/dl). In conclusion, PGSI improves humoral disorder and thus prevents inadequate tissue oxygen delivery.     

Brachiocephalic Vein Thrombus of Papillary Thyroid Cancer: Report of a Case

Differentiated thyroid cancer sometimes shows microscopic vascular invasion but rarely causes a tumor thrombus in a great vein. A preoperative diagnosis of the latter condition is difficult if a patient does not have any overt symptoms, but it is important for operative planning. We encountered a 26-year-old woman with papillary thyroid cancer and a tumor thrombus extending from the brachiocephalic vein to the superior vena cava. The tumor was successfully treated by surgical resection. To our knowledge only 19 such cases have so far been reported. We herein report our case, review the literature, and discuss the associated diagnostic problems and treatments including such areas as age, gender, symptoms, pathology, extension, and outcome. Received: October 20, 2000 / Accepted: July 17, 2001     

Reactive oxygen

Mammalian tissues have large amounts of available ATP which are generated by oxidative phosphorylation in mitochondria. To maintain the body, large amounts of oxygen are required to regenerate the ATP molecules. A small fraction of the inspired oxygen is converted to superoxide radicals and related metabolites even under physiological conditions. Most reactive oxygen species react rapidly with a variety of molecules, thereby interfering with cellular functions and various diseases.     

Marked thrombocytosis following relapse of acute myeloblastic leukemia associated with development of translocation (2;14) (p13;q32)

Thrombocytosis is a rare finding in acute myeloblastic leukemia (AML). Here, we describe a patient with AML who relapsed with marked thrombocytosis. The patient was initially diagnosed as having AML (M4) with a low platelet count. The patient was started on combination chemotherapy including high-dose etoposide and achieved complete remission. However, the patient relapsed six months later with an extremely high platelet count (72.5 x 10(4)/microl). Cytogenetic analysis at relapse revealed the development of t(2;14)(p13;q32). Despite the repeated combination chemotherapy, the patient died with progressive disease. This case suggests that the additional chromosomal aberration t(2;14)(p13;q32) may be related to abnormal thrombocytosis in AML.     

A network system of medical and welfare information service for the patients,their families,hospitals, local governments,and commercial companies in a medical service area

A service information system using the Internet, which connected the various people who are related to medical treatment and nursing welfare, was constructed. An intractable neurological disease patient who lives in the Onga district, Fukuoka, Japan, and the people who are related to the service were chosen as test users in an experimental model. The communicated service information was divided into open-use data (electronic bulletin board, welfare service, medical care service, and link to private company service home page) and closed-use data (the individual patient's hysterics). The open data server was installed in an Internet service provider The open data could be accessed not only by the patient, but also by the family, information center, companies, hospitals, and nursing commodity store related to patient's nursing and medical treatment. Closed data server was installed in an information center (public health center). Only patient and information center staff can access the closed data. Patients should search and collect the service information of various medical and welfare services by themselves. Therefore, services prepared for the patient are difficult to know, and they cannot be sufficiently utilized. With the use of this information system, all usable service information became accessible, and patients could easily use it. The electronic bulletin board system (BBS) was used by patients for knowing each other or each others' family, and was used as a device for exchange of wisdom. Also, the questions for the specialist, such as doctor, dentist, teacher, physical therapist, care manager, welfare office staff member, and public health nurse, and the answers were shown on the BBS. By arranging data file, a reference of various patients in question and answer, which appeared in this BBS, was made as "advisory hints" and was added to the open data. The advisory hints became the new service information for the patients and their family. This BBS discovered the possibility of becoming an important information source for companies, hospital and, administration to know the requirements of patients and their families and the kind of services to be served. Although suppliers provide medical and welfare services for the patient, there is a tendency that the service information is sent by the suppliers at their own convenience. The information system in which various people participated was constructed in order to collect information for the patient, taking a patient-oriented approach. The result of the model test showed that this information system using Internet technology is a good system for both the service supplier and its receiver.     

Distribution of pendrin in the organ of Corti of mice observed by electron immunomicroscopy

The distribution of pendrin, which is encoded by the Pendred syndrome gene, has been investigated immunohistochemically in the inner ear. In the cochlea, pendrin has been found in the spiral prominence, external sulcus cells, Hensen’s cells and Claudius cells, but its expression in the organ of Corti remains unclear. We examined whether pendrin localizes in the organ of Corti by postembedding immunogold analysis. In the organ of Corti, gold particles were clearly observed in outer and inner hair cells, including the stereocilia. The density of the particles was especially high in the cuticular plates of the hair cells. Gold particles were also detected in the external sulcus, in part of the spiral ligament adjacent to the external sulcus, in supporting cells, and in the spiral ganglion of the cochlea. Our study revealed that pendrin occurs in the organ of Corti. The role of pendrin in the organ of Corti and its association with the Cl^- or pH regulation of neurotransmission require further study.     

Regenerating the fibula with beta-tricalcium phosphate minimizes morbidity after fibula resection

The aim of this study was to assess the radiologic and clinical outcome when beta-tricalcium phosphate is used as a bone graft substitute to backfill the fibular defect that is created by harvesting the fibula. Fourteen patients who had fibula resections to be used as bone grafts for bone tumor resections were assessed radiographically. Callus formation bridging the beta-tricalcium phosphate was seen in 12 of 14 patients at an average of 1.4 months after surgery. In these 12 patients the beta-tricalcium phosphate mostly was absorbed and replaced by newly formed bone at an average of 9.3 months after surgery. In all children, beta-tricalcium phosphate was replaced by newly formed bone at an average of 3.2 months after surgery. Only one adult patient had complete regeneration of the fibula. Few patients had continuity between the regenerated fibula and the native fibula. In one patient in whom free vascularized fibula was harvested, regeneration of the fibula was not observed. Clinical functional outcome was not correlated with successful fibula regeneration and union with the native fibula, as determined using radiographs. The results of the study suggest that, in children, regeneration of the fibula by implanting beta-tricalcium phosphate into a bone defect can reduce morbidity of the fibula harvest sites.     

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.